{"id" : 3670, "question_text" : "An 8-year-old girl is being evaluated for repeated episodes of pneumonia. During these events, the girl is usually afebrile, but develops cough and sometimes spits out pink, frothy mucus. Chest radiographs have shown diffuse multifocal infiltrates. These episodes are usually treated with antibiotics and corticosteroids, and improve over several days. The girl has no history of asthma or wheezing, chronic or recurrent upper respiratory congestion with rhinorrhea, or kidney disease. Physical examination findings are normal today, and the point-of-care urinalysis is normal. Of the following, the laboratory test MOST likely to determine this girl's diagnosis is", "options" : "[\"\\u03b11-antitrypsin genotype\", \"antineutrophil cytoplasmic antibodies\", \"complete blood cell count with differential\", \"radioallergosorbent test panel for aeroallergens\"]", "explanation" : "Correct Answer: C\nThe girl in the vignette has pulmonary hemosiderosis characterized by repeated episodes of subclinical pulmonary hemorrhage that may present with cough and pink frothy sputum. Chest radiography will demonstrate multifocal infiltrates. The characteristic finding, other than the radiographic infiltrates, is anemia, which may be variable and exacerbated by episodes of hemorrhage.\n\nThe girl's presentation is not indicative of emphysema or other condition suggestive of α1-antitrypsin deficiency. α1-Antitrypsin deficiency in children most often manifests as hepatic disease rather than primary pulmonary disease. Antineutrophil cytoplasmic antibodies would be present in one of the autoimmune vasculitis pulmonary hemorrhage syndromes. This is less likely given that these are typically associated with glomerulonephritis and she has no history of renal disease or blood in the urine. Radioallergosorbent testing is not likely to be productive in this situation because the girl's history is not suggestive of allergy or asthma.\n\nIdiopathic pulmonary hemosiderosis is characterized by repeated episodes of intrapulmonary bleeding without evidence of autoimmune disease. There is a subpopulation of children with hemosiderosis associated with milk allergy. These children have neither IgE antibodies to milk nor a classic allergy phenotype, but IgG4 antibodies have sometimes been identified. For this subgroup of children, milk avoidance is paramount to treatment. In some infants with idiopathic pulmonary hemorrhage, an association with mold exposure has been identified. For most children with idiopathic pulmonary hemosiderosis, there is no identifiable trigger and treatment is targeted toward nonspecific inflammation.\n\nPREP Pearls\n• Anemia is a characteristic finding in idiopathic pulmonary hemorrhage.\n• Repeated episodes of pulmonary infiltrate in the context of variable degrees of anemia should suggest a diagnosis other than infection.\n• Pulmonary hemosiderosis is characterized by repeated episodes of subclinical pulmonary hemorrhage that may present with cough and pink frothy sputum; chest radiography will demonstrate multifocal infiltrates.\n\nABP Content Specifications(s)\n• Recognize clinical findings associated with hemosiderosis\n• Identify the risk factors associated with hemosiderosis\n\nSuggested Readings\n• Temel MT, Temel L, Coskun ME, et al. Respiratory distress and severe anemia in a child with idiopathic pulmonary hemosiderosis [published online ahead of print April 20]. J Pediatr Hematol Oncol. 2018.\n• Voter KZ, Ren CL. Pulmonary hemorrhage. In: Light MJ, ed. Pediatric Pulmonology. Itasca, IL: American Academy of Pediatrics; 2011:583-599.\n• Zhang Y, Luo F, Wang N, et al. Clinical characteristics and prognosis of idiopathic pulmonary hemosiderosis in pediatric patients [published online ahead of print October 2]. J Int Med Res. 2018. doi:10.1177/0300060518800652."}
{"id" : 1603, "question_text" : "A 14-year-old boy presents to your office for a preparticipation sports physical examination. He is a freshman in high school and would like to play for his school's hockey and soccer teams in the upcoming year. His parents report that the boy has a history of 2 concussions. The first occurred 2 years ago when he jumped off a swing. The second occurred 4 months ago while he was wrestling with his brother. He did not lose consciousness with either event, but experienced headaches and sensitivity to light for 2 weeks after the recent incident.\n\nThe boy reports that he feels \"fine\" now. However, his parents report that he seems more forgetful and has had difficulty concentrating since the second concussion. He is otherwise healthy. You counsel the family regarding the boy's current condition and the risks of sports participation based on his history.\n\nOf the following, the MOST accurate statement to include when counseling this family is that", "options" : "[\"he can be cleared for both soccer and football participation, but will have to stop contact sports permanently if he has a third concussion\", \"he can be cleared for soccer participation, but should not play hockey until his cognitive symptoms resolve\", \"neuropsychological testing is recommended to identify any cognitive deficit\", \"the boy's difficulty with concentration and forgetfulness are unrelated to his injury history\", \"the boy's recent injury does not meet the criteria for diagnosis of a concussion\"]", "explanation" : "Correct Answer: C\nThe adolescent in the vignette sustained a sports-related concussion 4 months before his preparticipation visit. His parents have concerns about persistent neurocognitive functional deficits after his injury. Neuropsychological testing could identify areas of impaired cognitive function. This would be important information to consider when making a sports participation clearance decision.\n\nA concussion is a neurologic injury that results from a rotational (side-to-side) or linear (back-and-forth) force applied to the head or from direct contact to the head. By definition, a concussion does not cause visible brain structural abnormalities, but instead can cause somatic symptoms, sleep disturbance, mood symptoms, and/or cognitive problems. Although most children recover from sports-related concussion within 6 weeks of injury, some individuals experience prolonged symptoms.\n\nThere are no gold-standard tests to identify persistent cognitive dysfunction after concussion; however, neuropsychological testing may identify memory and concentration deficits. For the boy in the vignette, in the absence of baseline neuropsychological testing, any deficits identified could not be conclusively linked to his concussion. However, low test scores that are inconsistent with prior school performance would be suggestive of concussion-related functional impairment.\n\nThe boy's parents report that he has had onset of memory and concentration difficulties since his recent concussion, and these concerns should be taken seriously. His history of headache, light sensitivity, and cognitive symptoms after a wrestling injury meet the criteria for sports concussion. He should not return to contact sports (eg, soccer, basketball) or collision sports (eg, football, hockey) without additional evaluation. A history of 3 concussions is not, by itself, an absolute contraindication to contact or collision sport participation. Withholding sports clearance should be considered when an athlete has had multiple concussions, along with a history of increasingly prolonged recovery times, a history of multiple concussions occurring after short intervals, and/or concussions that occurred with seemingly minor contact.\n\nPREP Pearls\n• Although most children recover from sports-related concussion within 6 weeks of injury, some individuals experience prolonged symptoms.\n• There are no gold-standard tests to identify persistent cognitive dysfunction after concussion, but neuropsychological testing may identify functional deficits\n\nABP Content Specifications(s)\n• Understand the importance of assessing and documenting neurocognitive function prior to sports participation\n\nSuggested Readings\n• Halstead ME, McAvoy K, Devore CD, Carl R, Lee M, Logan K. Returning to learning following a concussion. Pediatrics. 2013;132(5):948-957. http://pediatrics.aappublications.org/content/132/5/948.\n• McCrory P, Meeuwisse WH, Aubry M, et al. Consensus statement on concussion in sport: the 4th International Conference on Concussion in Sport held in Zurich, November 2012. Br J Sports Med. 2013;47(5):250-258. doi: http://dx.doi.org/10.1136/bjsports-2013-092313."}
{"id" : 1683, "question_text" : "A 6-year-old girl is brought to your office for evaluation of nonspecific periumbilical abdominal pain and loose stools. The mother reports that the girl has previously had intermittent transient abdominal pain, but the frequency of pain has increased. She has been soiling her underwear with small amounts of nonbloody loose stool for 2 weeks. Her previous stooling pattern was reported as 1 small stool every day or every other day. When the girl had a urinary tract infection at 4 years of age, a doctor recommended a stool softener, but the mother discontinued the medication after 1 month. The girl has always been a picky eater, refusing most fruits and vegetables, but her appetite is now decreased. She is voiding normally and is otherwise well. She has normal growth parameters and velocity; normoactive bowel sounds; an abdomen that is soft and nontender with a sausage-shaped mass palpable in the left lower quadrant; and deep tendon reflexes that are normal. Rectal examination reveals a normal anal wink, slight dilation of the rectal vault, and the presence of a hard stool mass. Of the following, the BEST initial step in management is to", "options" : "[\"begin an oral stimulant laxative\", \"encourage increased dietary fiber\", \"perform a mineral oil enema\", \"recommend behavioral therapy\", \"try a gluten-free diet\"]", "explanation" : "Correct Answer: C\nThe girl in this vignette exhibits many of the clinical features associated with fecal overflow incontinence. Given the hard stool mass present in a dilated rectal vault, the best initial step in management is to remove the fecal impaction by performing an enema.\n\nEncopresis is the repetitive involuntary passage of feces into inappropriate places after the age of 4 years when most children have attained continence. The most common cause of functional encopresis is chronic constipation leading to overflow incontinence. Children may not respond to the urge to defecate for a variety of reasons: fear of pain associated with prior episodes of passing large dry stools or the presence of anal fissures; fear related to prior negative or punishing parenting practices of toilet training; unsubstantiated fears related to sitting upon or flushing the toilet; timidness about stooling in a public restroom; and being too busy or distracted by enjoyable activities. When the urge to defecate is ignored, the rectal vault stretches to accommodate a greater volume. Repeated withholding causes further stretching and the retained stool becomes larger, drier, and harder. Passage of stool becomes increasingly more difficult, which leads to further avoidance of defecation, and eventually a large impacted fecal mass is formed. The pressure of this mass on the anosphincter complex makes voluntary closure of the external anal sphincter more difficult. Eventually semi-formed and liquid stool leaks around this mass. The child is often unaware of the fecal soiling because the chronic dilation impairs sensation, decreases rectal tone, and leads to pelvic floor dysfunction.\n\nThe clinical features of the girl in this vignette that are typical of chronic constipation and may be associated with fecal overflow incontinence or functional encopresis include:\n• Intermittent transient nonspecific or lower left quadrant abdominal pain that is increasing in frequency\n• Involuntary, and perhaps unrecognized, soiling of underwear with small amounts of nonbloody loose stool in the context of a prior stooling pattern of small or infrequent stools\n• History of stool softener or laxative use\n• Previous episode of urinary tract infection or enuresis\n• Decreased appetite because of early satiety\n• A diet with limited intake of natural fiber\n• Normal growth parameters\n• The presence of a fecal mass in the left lower quadrant, dilation of the rectal vault, and fecal impaction\n\nExternal indirect pressure of the fecal mass on the urethra and bladder, plus increased intra-abdominal pressure may lead to enuresis, incomplete voiding, or rectal prolapse. Dysfunctional voiding, in combination with increased urethral exposure to enteric pathogens from soiling in the underwear, leads to the greater prevalence of recurrent urinary tract infections, especially in female patients.\n\nThe management of encopresis has 4 key components: disimpaction, maintenance pharmacotherapy, behavioral modification, and education of the patient and family. Disimpaction may be performed manually. Enemas, suppositories, oral polyethylene glycol, or magnesium citrate may also be used. After disimpaction, the use of an oral stimulant laxative, stool softeners/lubricants, or osmotic laxatives is necessary to maintain regular stooling. If these treatments are initiated without prior disimpaction, the patient may experience discomfort from abdominal cramping and additional overflow incontinence. Maintenance therapy should continue until 1 or 2 soft bowel movements are passed daily, the rectal vault has returned to normal capacity, and normal sensation and anal sphincter functionality have resumed. Dietary and behavioral modifications are integral to the successful management of functional constipation. Celiac disease or gluten sensitivity may present with a range of gastrointestinal problems including constipation and diarrhea; this etiology should be given consideration in patients who do not respond to the routine management of encopresis. Family education must emphasize that fecal overflow incontinence is not intentional, relapses are common, and the corrective process is long.\n\nPREP Pearls\n• Encopresis is the repetitive involuntary passage of feces into inappropriate places after the age of 4 years when most children have attained continence.\n• The most common cause of functional encopresis is chronic constipation leading to overflow incontinence.\n• The management of encopresis has 4 components: initial disimpaction, maintenance pharmacotherapy, behavioral modification, and education of the patient and family.\n\nABP Content Specifications(s)\n• Understand the physiologic effects of stool retention\n• Recognize the clinical features associated with fecal overflow incontinence\n• Recognize co-morbidities commonly associated with encopresis\n• Plan the appropriate management of encopresis of various etiologies\n\nSuggested Readings\n• Biggs WS, Dery WH. Evaluation and treatment of constipation in infants and children. Am Fam Physician. 2006;73(3):469–477.\n• Colombo JM, Wassom MC, Rosen JM. Constipation and encopresis in childhood. Pediatr Rev. 2015;36(9):392–402. doi: http://dx.doi.org/10.1542/pir.36-9-392.\n• Har AF, Croffie JM. Encopresis. Pediatr Rev. 2010;31(9):368–374. doi: http://dx.doi.org/10.1542/pir.31-9-368."}
{"id" : 2296, "question_text" : "A 10-year-old boy is seen in the office with his maternal aunt to establish care. The aunt reports that before being permanently placed with her after his mother's parental rights were terminated owing to substance use and neglect, the boy had been moving \"back and forth\" between his mother's home and multiple foster homes for the past 5 years. The aunt has contacted his case worker to get his medical and educational records so that she can enroll him in school. The boy's mother dropped out of school in the 9th grade, after she started using drugs, and his aunt is trying to avoid \"repeating history.\" The boy takes multiple medications to help with his behavior, and his aunt is wondering if their use can be discontinued because \"he has a stable home now and does not need them.\" The boy's physical examination findings are normal. Of the following, the BEST next step in this boy's care is to", "options" : "[\"agree that the medications can be discontinued as he is now in a stable living situation with a caring family member\", \"perform developmental and mental health screening in the office and refer for further assessment and medication management\", \"recommend that the medication use be continued until medical and school records can be obtained from his case worker\", \"request that the aunt reach out to his most recent foster family to determine what each medication was treating\"]", "explanation" : "Correct answer is B\n\nCritique\nThe boy in the vignette has been placed in the care of his maternal aunt. Because this is a new placement for the boy, developmental and mental health screening should occur along with relevant referrals (eg, mental health, developmental and behavioral pediatrics) for assessment and medication management.\n\nThe American Academy of Pediatrics classifies children in the foster care system as children with special health care needs. Among those in foster care, up to 80% enter the system with a mental health condition and 40% of school-aged children have learning challenges (American Academy of Pediatrics Council on Foster Care, 2015). Children in foster care are more likely to receive special education services, change schools in the middle of the school year, experience grade retention, and drop out of school as adolescents. As adults, this population is at increased risk of experiencing low educational attainment, mental health disorders, unemployment, homelessness, and post-traumatic stress disorder.\n\nGiven the frequency of psychiatric and neurodevelopmental disorders in this population, it is recommended that a comprehensive health assessment, including developmental and mental health screening, be completed within 30 days of entry into the foster care system. Reassessment should be conducted 60 to 90 days after entry into foster care, and continued monitoring should occur semiannually to assess adjustment to placement.\n\nAlthough some studies have shown greater stability with placement in kinship care, there are associated challenges. Kinship caregivers are generally older and have limited access to services compared with nonrelative foster parents. Additionally, oversight of and subsidies provided for kinship care may be limited.\n\nGiven the boy's history of multiple placements (which, for many, results in difficulties forming a secure attachment to a caregiver) along with the history of maternal substance use and neglect, it is recommended that the boy continue to use his medications until he can be assessed by a mental health professional, a development and behavioral pediatrics professional, or both. Childhood trauma has been associated with difficulties in emotional regulation, aggression, inattention, hyperactivity, and impulsivity. Data regarding children with Medicaid insurance demonstrate that children in foster care are prescribed psychotropic medications at a much higher rate and with a higher rate of polypharmacy compared with other children.\n\nOne of the many challenges of establishing a medical home for a child in the foster care system or in kinship care is a lack of comprehensive medical and educational records (eg, immunization records, medication history, surgical history, and psychosocial/family history). Caseworkers face similar challenges, and they may not have the training needed to manage medical and educational needs with the information available to them. Thus, it is not recommended to delay medication management decisions for the boy in the vignette until records are obtained from his case worker. Requesting that the aunt reach out to his most recent foster family to determine what the boy's medications were treating is not the best next step in the boy's care, as it is unclear what the family knew or understood about his diagnoses and treatment. Foster parents often receive little education regarding the impact of trauma, risk of experiencing mental health disorders and developmental disabilities, coordinating care among medical and mental health care providers, and ensuring adequate educational support.\n\nPediatricians caring for children in the foster care system must be aware of and understand the impact of trauma, adverse childhood experiences, and neglect on early development, as well as the resulting risk of experiencing neurodevelopmental and psychiatric disorders through childhood and adolescence. Pediatricians play a key role as the child's medical home, identifying, addressing, and coordinating mental health, developmental, and educational needs. This role includes comprehensive screening for mental health and developmental disorders, referral to a specialist for further assessment when appropriate, and longitudinal monitoring. Ongoing communication between caseworkers and foster parents is also required to ensure all of a child's needs are met.\n\nContent Domain\nBehavioral/Developmental\n\nABP Content Specification(s) / Content Area(s)\n- Understand the psychosocial issues surrounding children in foster care"}
{"id" : 930, "question_text" : "A 6-month-old male infant is brought to your office for poor feeding. The mother reports that he refuses to drink from a bottle and cries when she tries to feed him. He has been seen in your office in the past for various infections, including a perirectal abscess, otitis media, and pneumonia. His axillary temperature is 38.3°C, pulse rate is 110 beats/min, respiratory rate is 24 breaths/min, and blood pressure is 100/60 mm Hg. On examination, the child is fussy but consolable. He has erythematous ulcerations in his oral mucosa, but no hepatosplenomegaly or lymphadenopathy. The remainder of the physical examination is normal. The following are the results of the infant's complete blood cell count:\n• White blood cell count, 6,000/µL (6.0 x 10'/L), with 5% polymorphonuclear leukocytes, 85% lymphocytes, 7% monocytes, and 3% eosinophils\n• Hemoglobin, 12.5 g/dL (125 g/L)\n• Mean corpuscular volume, 85/µm3 (85 fL)\n• Platelet count, 300 x 103/µL (300 x 10A)\n\nOf the following, the MOST appropriate next step in management for this patient is to", "options" : "[\"admit for bone marrow studies to rule out leukemia\", \"draw a blood culture and give parenteral antibiotics\", \"give intravenous hydration and a prescription for pain medication\", \"reassure the parents that this is most likely a self-limited viral infection\", \"repeat complete blood cell count with differential the following day\"]", "explanation" : "The infant described in the vignette with fever and severe neutropenia (absolute neutrophil count [ANC], 300/µL (0.3 x 109/L) should have a blood culture drawn and receive parenteral antibiotics including coverage for Pseudomonas. Normal values for ANC vary with age (Item C225A, page C-176). From 2 months to 1 year of age, neutropenia is defined as ANC less than 1000/4 (1.0 x 109/L), whereas in children older than 1 year and adults, the lower limit of normal for ANC is 1500/µL. (1.5 x 109/L). In addition, there is variation in normal neutrophil counts among different racial groups, with 3% to 5% of patients of African descent having ANC values less than 1500/µL. (1.5 x 109/L). The risk of infection is related to the severity (Item C225B, page C-176), duration, and mechanism of neutropenia. Long periods of neutropenia without recovery lead to an increased risk of infection. Conditions in which there is adequate bone marrow reserve to mobilize neutrophils, as in immune neutropenia, are less likely to be associated with severe infection than in disorders of decreased marrow production, such as bone marrow failure syndromes or myelosuppression secondary to chemotherapy.\n\nIn evaluating an infant or child with neutropenia, it is important to ask whether there is a history of recurrent infections in the patient or the family. A thorough physical examination for signs of neutropenia (eg, skin infections, mouth ulcers, and gingivitis) and congenital anomalies is necessary to evaluate for inherited syndromes. The presence of other cytopenias would suggest a generalized bone marrow disorder, such as aplastic anemia or leukemia.\n\nIn the pediatric population, congenital forms of neutropenia are much less common than acquired disorders. Severe congenital neutropenia (SCN) can lead to life-threatening infections and increased risk of malignant tumors. Patients with SCN often have very severe neutropenia (ANC <200/mL [0.2 x 109/L]) and present in infancy with multiple umbilical, mucosal, or skin infections. The inheritance pattern can be autosomal dominant or autosomal recessive, or it may be sporadic. It has been associated with ELANE mutations. The risk of leukemia is 5% to 10% in SCN. Kostmann syndrome is an autosomal recessive form of SCN that is associated with mutations in the HAX1 gene and has a 15% to 20% risk of leukemia. Patients at highest risk for malignant transformation are those with poor response to high doses of granulocyte colony-stimulating factor (G-CSF) and those with certain mutations in G-CSF receptor gene or ELANE gene.\n\nCyclic neutropenia is associated with different mutations within the same ELANE gene than those seen in SCN. Serial blood cell counts performed twice a week reveal a 21-day cycle of the neutropenia during which fever and oral ulcers can erupt when the ANC is at its nadir.\n\nGranulocyte-colony-stimulating factor may be required to prevent infections in patients with SCN or cyclic neutropenia and to treat infection or symptoms related to neutropenia (eg, mouth sores). Treatment with G-CSF is not generally indicated in asymptomatic patients with benign forms of neutropenia.\n\nThe child described in this vignette has a history and clinical findings concerning for SCN. A bone marrow examination would not be the first step in management because there are no other cytopenias to suggest bone marrow failure or malignant tumors. Hydration and pain medication may be useful if the oral ulcerations are prohibiting adequate oral fluid intake, but empiric treatment for an infection should be started first. It cannot be assumed that the severe neutropenia is caused by a self-limited viral infection, especially in an infant who has had multiple prior infections. Reassurance and repeating the complete blood cell count the following day is not adequate management for this infant with fever and severe neutropenia. Furthermore, if the neutropenia was thought to be transient (eg, viral mediated), the ANC is not likely to reveal a significant change in 1 day. Drawing a blood culture and giving empiric parenteral antibiotics for presumed bacteremia would be the most appropriate next step in managing this patient with suspected SCN.\n\nPREP Pearls\n• An infant with fever and severe neutropenia (ANC <500/µL [0.5 x109/L) should have a blood culture drawn and receive parenteral antibiotics including coverage for Pseudomonas.\n• Severe congenital neutropenia is associated with an increased risk of malignant tumors.\n• The risk of infection is related to the severity, duration, and mechanism of neutropenia.\n\nAmerican Board of Pediatrics Content Specification(s):\n• Recognize that congenital neutropenia may be persistent or cyclical, and manage appropriately\n\nSuggested Reading:\n• Bouma G, Ancliff PJ, Thrasher A, Burns SO. Recent advances in the understanding of genetic defects of neutrophil number and function. Br I Hernatol. 2010;151:312-326. doi:10.1111/j.1365-2141.2010.08361.x\n• Bortug K, Klein C. Genetic etiologies of severe congenital neutropenia. Curr Opin Pediatr. 2011;23:2321-2326. doi:10.1097/M0P.0b013e32834262f8\n• Segel GB, Halterman JS. Neutropenia in pediatric practice. Pediatr Rev. 2008;29:12-24. doi:10.1542/pir.29-1-12"}
{"id" : 2525, "question_text" : "A 4-month-old, full-term, developmentally normal infant is evaluated in the outpatient office for paroxysmal episodes for the past 2 weeks. During the episodes, his body crunches up repeatedly, \"as though he is doing baby sit-ups.\" Initially, it occurred once or twice per day, usually in the evening, but it now occurs in clusters, at which point he becomes difficult to console. His mother suspects that he is uncomfortable and might have gastroesophageal reflux. She is concerned because the episodes are becoming more frequent. The infant's vital signs are normal. His physical examination findings, including neurological, are normal. During the visit, the infant had a typical episode in which he stiffened, his arms extended out suddenly and he bent forward. This occurred in a short cluster during which he appeared uncomfortable and cried. Of the following, the BEST next step in this infant's management is", "options" : "[\"referral to a gastroenterologist for outpatient evaluation\", \"referral to a neurologist for outpatient evaluation\", \"reflux precautions and close follow-up in the pediatrician's office\", \"urgent neurological evaluation through the emergency department\"]", "explanation" : "Correct Answer: D\nThe infant in the vignette's episodes are suggestive of infantile spasms (IS), a condition requiring prompt diagnosis and treatment to optimize developmental outcome. Infantile spasms, the most common epilepsy syndrome in infancy, is clinically characterized by a triad of:\n• Epileptic spasms\n• Electroencephalogram background of hypsarrhythmia\n• Accompanying developmental plateau and regression\n\nWhen all 3 of these features are present the eponym, \"West syndrome,\" is used. The diagnosis requires a high index of suspicion and necessitates urgent evaluation with expedited electroencephalogram (EEG) and pediatric neurology consultation.\n\nInfantile spasms typically present during the first year after birth, with a peak age of onset between 3 and 7 months. Classification is divided into 2 etiologic categories: cryptogenic and symptomatic. Cryptogenic IS is diagnosed in 10% to 40% of affected infants, presenting in developmentally normal infants whose diagnostic evaluation does not identify an underlying cause. With prompt recognition and early initiation of treatment, this cohort of children is more likely to have a favorable developmental outcome.\n\nSymptomatic IS is diagnosed in 60% to 90% of affected infants; a greater percentage of infants are now recognized as symptomatic due to improved genetic diagnostic testing. Symptomatic IS can occur secondary to prenatal/perinatal causes including hypoxic-ischemic encephalopathy, cortical malformations, and genetic syndromes (classically, tuberous sclerosis complex and trisomy 21). Postnatal etiologies can include trauma and infection. Developmental outcome is highly dependent on the underlying etiology. Item C17A summarizes the etiologic classification of infantile spasms.\n\nOften, when symptoms of IS begin, parents bring their children to the pediatrician with concerns for colic or gastroesophageal reflux (GER). Classically, spasms involve symmetric contraction with flexion of the trunk, neck and arms lasting up to 5 seconds and occurring in clusters. However, there is a range of clinical appearance and severity of spasms, with some presenting as tonic eye rolling or neck flexion/nodding, which can be subtle and easily missed. Spasms may initially emerge while the infant is drowsy, either when falling asleep or waking from sleep, and can be accompanied by crying, grimacing, pallor, flushing, or nystagmus.\n\nSuspicion for infantile spasms should prompt urgent referral for evaluation and EEG rather than outpatient referral due to the impact early diagnosis and treatment can have on developmental outcome. While GER or colic can have a similar clinical appearance, IS should be considered and excluded first.\n\nElectroencephalography in cases of IS demonstrates a specific interictal background of hypsarrhythmia: a chaotic, disorganized, asynchronous, nonrhythmic, high-voltage spike and spike-and-slow wave pattern (Item C17B). While a routine EEG may capture hypsarrhythmia, a recording including wakefulness and sleep is helpful as hypsarrhythmia initially emerges during stage 2 and 3 of non-REM sleep; prolonged (24 hours) EEG monitoring allows exclusion of movements mimicking infantile spasms.\n\nOnce the diagnosis of IS is confirmed, evaluation is directed at identifying an underlying cause. This process typically includes magnetic resonance imaging of the brain as well as genetic and metabolic testing, with an emphasis on treatable disorders with specific therapies. Treatment of IS includes administration of adrenocorticotropic hormone (ACTH) or vigabatrin initiated as soon as possible after diagnosis. Adrenocorticotropic hormone is preferred over vigabatrin for first-line management of cryptogenic spasms. Vigabatrin can be used first-line and is the preferred initial choice in children with tuberous sclerosis complex. Treatment considerations are summarized in Item C17C. Treatment effect is often reassessed both clinically and with repeat EEG monitoring to confirm resolution of the hypsarrhythmia pattern. Children with infantile spasms often require long-term, multidisciplinary monitoring for neurodevelopmental progress and subsequent development of other seizure types.\n\nPREP Pearls\n• Infantile spasms, the most common epilepsy syndrome in infancy, are clinically characterized by a triad of: 1) epileptic spasms, 2) electroencephalogram background of hypsarrhythmia, and 3) accompanying developmental plateau and regression.\n• A high index of suspicion and urgent evaluation with expedited electroencephalography are necessary to confirm the diagnosis of infantile spasms and quickly initiate treatment.\n• Targeted evaluation for underlying causes of infantile spasms, focusing on treatable conditions, should occur concomitantly with treatment initiation and is tailored toward the individual patient. Long-term developmental outcome is impacted by time to treatment and underlying etiology.\n\nMOCA-Peds Objective\n• Recognize the clinical features of rheumatic fever.\n\nABP Content Specifications(s)\n• Understand the prognosis of infantile spasms\n• Recognize the clinical findings associated with infantile spasms\n\nSuggested Readings\n• Fine A, Wirrell E. Seizures in children. Pediatr Rev. 2020;41(7):321-347. doi:10.1542/pir.2019-0134.\n• Go CY, Mackay MT, Weiss SK, et al. Evidence based guideline update: medical treatment of infantile spasms. Report of the Guideline Development Subcommittee of the American Academy of Neurology and the Practice Committee of the Child Neurology Society. Neurology. 2012;78(24):1974-1980. doi:10.1212/WNL.0b013e318259e2cf.\n• McBride M. Seizure disorders. In: McInerny TK, Adam HM, Campbell DE, DeWitt TG, Foy JM, Kamat DM, eds. American Academy of Pediatrics Textbook of Pediatric Care. American Academy of Pediatrics; 2021:chap 327. Accessed September 1, 2022. Pediatric Care Online.\n• Whelass JW, Gibson PA, Rosbeck KL, et al. Infantile spasms (West syndrome): update and resources for pediatricians and providers to share with parents. BMC Pediatr. 2012;12:108. doi:10.1186/1471-2431-12-108."}
{"id" : 3113, "question_text" : "You are volunteering as a team physician for a high school soccer tournament. During a game, a 14-year-old adolescent girl is elbowed in the head by another player. You evaluate the injured teenager on the sideline; the girl reports dizziness, nausea, and headache, but physical examination findings are unremarkable. You hold the girl out of the remainder of the game. After the game, the girl reports that all symptoms resolved within 15 minutes after the injury. She and her parents would like to know if she could participate in a tournament game later that day. Of the following, the MOST appropriate recommendation for this family is that same-day return to play is", "options" : "[\"appropriate for this girl because physical examination was completely normal\", \"appropriate for this girl because she did not lose consciousness\", \"appropriate for this girl because symptoms resolved in less than 30 minutes\", \"not appropriate for a child with a suspected concussion\", \"not appropriate for this girl because headache was one of her symptoms\"]", "explanation" : "The soccer player in the vignette has sustained a concussion. Same day return-to-play is never appropriate for children and adolescent athletes following concussion.\n\nThe Consensus Statement on Concussion in Sport delineates the criteria for diagnosis of sports concussion. An athlete must sustain a direct blow to the head or experience linear or rotational acceleration of the skull as a result of a transmitted force. The direct or transmitted force must result in symptom development, generally within several hours following injury. Symptoms fall into l of 4 groups: somatic (eg, headache, vision changes), cognitive (eg, memory disturbance, difficulty concentrating), mood changes (eg, irritability, depression), and sleep disturbance.\n\nThis girl's injury meets the criteria for concussion diagnosis; she experienced a direct blow to her head and became symptomatic immediately after her injury.\nThe treatment of concussion includes physical rest from all exertional activities, including physical activities with minimal risk of contact. Cognitive rest should also be implemented for a child with concussion symptoms. Cognitive rest may include time off from school, school accommodations (eg, limited tests and homework), and curtailing screen time (eg, watching television, using computers or smart boards, and texting).\n\nInitiating a step-wise gradual return to physical activities is indicated once individuals with concussion have been asymptomatic for 24 hours, as long as the neurologic examination is unremarkable. Additional evaluation, such as computerized neuropsychological testing, may be used as an adjunct to determine when return to play is appropriate. Both the Clinical Report on Sports-Related Concussion from the American Academy of Pediatrics and the Consensus Statement on Concussion in Sport detail the appropriate progression for return to physical activity following sports concussion.\n\nThe first 3 responses are incorrect because same day return-to-play following concussion is contraindicated, even for individuals with short symptom duration and normal physical examination. Loss-of-consciousness is not a criterion for the diagnosis of concussion. Headache is the most common concussion symptom, though it is not exhibited in about 25% of individuals with concussion. The presence or absence of headache should not be used as a marker of concussion severity.\n\nPREP Pearls\n• Same-day return to play is never appropriate for children and adolescent athletes following a suspected concussion.\n• Loss of consciousness is not required for the diagnosis of concussion.\n• Up to 25% of children with concussion do not report headache.\n\nAmerican Board of Pediatrics Content Specification(s)\n• Understand the criteria for return to play in sports after a head injury\n\nSuggested Reading\n• Halstead ME, Walter Ka Clinical report — sport-related concussion in children and adolescents. Pediatrics. 2010;126(3):597-615. doi:10.1542/ peds.2010-2005.\n• McCrory P, Meeuwisse W, Aubry M. et at Consensus statement on concussion in sport: the 4th International Conference cm Concussion in Sport held in Zurich, November 2012. Ciin 1 Sport Med. 2013;23(2)10-117. doi:10.1097/15M.0b013e318281367cf."}
{"id" : 808, "question_text" : "A 5-year-old boy is hospitalized for hypoxia and respiratory distress associated with bacterial pneumonia. He is receiving supplemental oxygen and parenteral antibiotics. On the second hospital day, you are called to his bedside because he is noted to be pale. His oral temperature is 37.9°C, pulse rate is 110 beats/min, respiratory rate is 28 breaths/ min, and blood pressure is 100/55 min Hg. On examination, the child is alert and pale. He has mildly icteric sclerae and a grade 2/6 systolic ejection murmur. The remainder of the physical examination is normal. The following are the results of the child's laboratory tests: White blood cell count, 16,500/µL (16.5 x 109/L), with 62% polymorphonuclear leukocytes, 30% lymphocytes, 7% monocytes, and 1% eosinophils; Hemoglobin, 5.0 g/dL (50 g/L); Mean corpuscular volume, 80/µm3 (80 fl,); Platelet count, 445 x 103/uL (445 x 109/L); Reticulocyte count, 10% (0.10); Direct antiglobulin test (direct Coombs), positive for IgG, negative for C3; Indirect antiglobulin test (indirect Coombs), negative upon review of his medical record, his hemoglobin on admission was 10.5 g/dL (105 g/L). Of the following, the MOST likely cause of the acute anemia in this child is", "options" : "[\"aplastic crisis\", \"cold agglutinin disease\", \"hemolytic uremic syndrome\", \"inconclusive because of conflicting Coombs results\", \"warm agglutinin autoimmune hemolytic anemia\"]", "explanation" : "Preferred Response: E\nThe child described in the vignette has the clinical presentation and laboratory results most consistent with a warm agglutinin autoimmune hemolytic anemia (AIHA). Autoimmune hemolytic anemia in infants and toddlers often occurs after an infection, whereas in adolescents it is more likely to be associated with an underlying systemic disease. Although infections are more commonly associated, there is evidence that antibiotics, such as cephalosporins, can also lead to AIHA. The most common form of primary AIHA in children involves warm-reactive IgG autoantibodies that bind to the red blood cell (RBC) at 37°C and lead to extra-vascular hemolysis. A second form of primary AIHA in children is paroxysmal cold hemoglobinuria. This is common after viral illnesses and is caused by an IgG autoantibody that leads to intravascular hemolysis at cold temperatures but binds complement at 37°C. A third form of primary AIHA is IgM-mediated cold-agglutinin disease, which can cause extravascular or intravascular hemolysis. Although this entity is more common in adults, it can occur in children in association with Mycoplasma infections.\n\nChildren with AIHA often present with pallor and weakness due to the anemia, with hemoglobin concentrations as low as 4 to 7 g/dL (40-70 g/L). The spleen may be mildly enlarged and palpable due to an increase in red pulp. A cardiac flow murmur and tachycardia may be present as a result of the high-output state caused by the anemia. The hemolysis can cause an increase in unconjugated bilirubin, leading to jaundice, which is especially noticeable in the sclerae and palms. Dark urine may be present in patients with intravascular hemolysis. Lactate dehydrogenase or aspartate aminotransferase concentrations can be elevated because these are released from the erythrocyte during hemolysis. In warm-reactive AIHA, the peripheral blood smear reveals numerous small spherocytes formed by the ingestion of part of the erythrocyte membrane by the spleen, causing the cells to take on a spherical shape. Reticulocytosis, up to 10% to 30% of the circulating RBC count, is the bone marrow's compensatory response to the shortened erythrocyte life span in the peripheral blood, although reticulocytopenia has been reported in 10% of pediatric patients.\n\nThe prognosis for AIHA in children is encouraging, with 77% of children with AIHA having a self-limited course and most children responding well to short-term therapy. The treatment of AIHA depends on the degree of anemia and type of AIHA. Observation alone is reasonable for the patient with mild anemia (hemoglobin concentration >9 g/dL [>90 g/L]) and no evidence of cardiovascular compromise. For the child in the vignette with a severely low hemoglobin con-centration, tachycardia, and a warm IgG autoantibody, the mainstay of treatment is corticosteroids and packed RBC transfusions. Plasmapheresis and immunoglobulin intravenous may be indicated as second-line therapies if corticosteroids and packed RBC transfusions are unsuccessful.\n\nThe direct antiglobulin test (DAT, formerly known as the direct Coombs test) detects antibodies or complement proteins bound to the surface of the erythrocytes. The patient's RBCs are washed to remove plasma proteins and then incubated at 37°C with the Coombs reagent, which is an antiserum that binds to human γ-globulin and complement (eg, C3). When the Coombs reagent is added, it binds to the autoantibodies on the RBC surface, if present, and causes agglutination of the RBCs. Further testing is performed to distinguish between IgG and complement antibodies on the RBC surface. The indirect antiglobulin test (IAT) detects unbound circulating antibodies in the patient's serum. This is useful in pregnant women and before blood transfusions but is not the appropriate test to diagnose AIHA.\n\nThe patient described in the vignette does not have laboratory evidence of aplastic crisis because his reticulocyte count and leukocyte count are both elevated and his platelet count is at the high end of the normal range. Cold agglutinin disease is unlikely because it is usually IgM mediated and seen in older patients. Hemolytic uremic syndrome (HUS) can have hemolysis as a feature, but it is usually associated with a gastrointestinal infection with diarrhea, acute renal insufficiency, and thrombocytopenia. Patients with HUS typically do not have a positive DAT result. The DAT and IAT results are not conflicting and support the diagnosis of warm agglutinin AIHA in this patient.\n\nPREP Pearls\n• A positive DAT result is consistent with AIHA.\n• AIHA is a usually associated with infection or antibiotic use in young children.\n• Corticosteroids and transfusion with packed RBCs are the mainstays of treatment for AIHA in children.\n• A DAT (Coombs test) is indicated in any patient presenting with a previously undiagnosed hemolytic anemia.\n\nAmerican Board of Pediatrics Content Specifications:\n• Understand that direct and indirect Coombs tests are a necessary part of the evaluation of a child with acute-onset anemia\n\nSuggested Reading:\n• Garratty G. Drug-induced immune hemolytic anemia. ASH Education Book. 2009;2009:73-79. doi:10.1182/asheducation-2009.1.73\n• Michel M. Classification and therapeutic approaches in autoimmune hemolytic anemia: an update. Expert Rev Hematol. 2011;4:607-618. doi:10.1586/EHM.11.60\n• Ware RE. Autoimmune hemolytic anemia. In: Nathan and Oski's Hematology of Infancy and Childhood. 7th ed. Philadelphia, PA: Saunders Elsevier; 2009:613-658\n• Zantek ND, Koepsell SA, Tharp DR Jr, Cohn CS. The direct antiglobulin test: a critical step in the evaluation of hemolysis. Am I Hematol. 2012;87:707-709. doi:10.1002/ajh.23218"}
{"id" : 1552, "question_text" : "A 12-year-old girl with poorly controlled asthma is brought to the emergency department with wheezing, cough, and dyspnea. She has been seen in the emergency department 6 times in the last year and required admission to the hospital on 4 of these occasions. During 2 episodes, she required admission to the intensive care unit with oxygen or noninvasive ventilatory support. She is nonadherent with prescribed inhaled corticosteroid/long-acting β-agonist and leukotriene inhibitor medications. Her usual triggers are weather changes and viral illness. She has been using her short-acting β-agonist at a dose of 4 inhalations every 1 to 2 hours for the last 8 hours. She reports that her hands are shaking and her heart is racing. She is spontaneously breathing but tachypneic and in moderate respiratory distress. She has a heart rate of 160 beats/min, respiratory rate of 44 breaths/min, and oxygen saturation of 95% in room air. Accessory muscles are used in respiratory effort, with retractions noted at suprasternal, subcostal, and substernal sites. Auscultation of the chest reveals no wheezing, crackles, or rhonchi. A radial pulse cannot be palpated during auscultation of cardiac sounds, and systolic blood pressure decreases by 20 mm Hg during inspiration. You have initiated therapy with continuous albuterol at 15 mg/h. The patient has just received prednisone 60 mg orally and intravenous magnesium sulfate. A chest radiograph reveals bilateral hyperinflation. Arterial blood gas tests reveal a pH of 7.34 and a pCO2 of 36 mm Hg. Of the following, the MOST appropriate next step in therapy is to", "options" : "[\"administer methylprednisolone 40 mg intravenously\", \"increase continuous albuterol to 20 mg/h\", \"initiate bilevel positive airway pressure support\", \"perform endotracheal intubation\", \"place a thoracostomy tube\"]", "explanation" : "The patient in this vignette has the signs and symptoms of status asthmaticus. Therapy has appropriately been initiated with albuterol and systemic steroids. Her respiratory status and her clinical examination findings are suggestive of a moderate to severe degree of airway obstruction, and further therapy is warranted to maintain lung volume, prevent atelectasis, optimize oxygenation and ventilation, and prevent respiratory failure. The most appropriate next step in therapy is to initiate support with bilevel positive airway pressure.\n\nThis patient's asthma is poorly controlled, and she is at high risk for asthma-related morbidity and mortality. Factors that place a patient at risk for death from asthma include severe asthma, poor perception of asthmatic symptoms, previous asthma attacks with rapid deteriorations, loss of consciousness or hospital admissions, frequent use of short-acting bronchodilator medications, reliance on crisis management in the emergency department, poor adherence to controller therapies, and steroid dependence.\n\nThe first-line treatment for asthma is β-adrenergic agents for the relief of bronchospasm. β-adrenergic activation stimulates adenylate cyclase, cyclic adenosine monophosphate, and protein kinase A with resultant smooth muscle relaxation. Albuterol is relatively selective for B2 receptors and is better tolerated than epinephrine, which nonselectively activates both B1 and B2 receptors with associated B1 effects on heart rate and blood pressure. Even with albuterol, frequent or excessive use may be associated with heart rate elevation, arrhythmia, neuromotor irritability, restlessness, emotional irritability, and tremor.\n\nIn the setting of status asthmaticus, the clinician must be mindful of the signs of impending respiratory failure. These signs may include mental status changes or somnolence, decrease in respiratory effort despite worsening hypoxemia, hypercarbia, and respiratory acidosis. The absence of wheezing or a \"silent chest\" in a patient with asthma is a cause for alarm and denotes severe bronchospasm. A carbon dioxide level that is higher than expected for the degree of tachypnea may reflect worsening respiratory muscle fatigue. An arterial blood gas measurement may provide clarity with regard to acid-base status and adequacy of compensatory mechanisms; in general, a pH less than 7.35 and a PCO2 greater than 45 mm Hg are reason for concern.\n\nPulsus paradoxus is a pathologically extreme decrease in systolic blood pressure and pulse amplitude during inspiration. The normal inspiratory fall in blood pressure is less than 10 mm Hg. When the measureable decrease is more than 10 mm Hg, it is referred to as pulsus paradoxus.\n\nPulsus paradoxus is reflective of diastolic dysfunction with a decrease in left ventricular filling and stroke volume and is a sign of severe cardiopulmonary compromise. Pulsus paradoxus may be caused by cardiac, pulmonary, or other disease states. It is a valuable assessment for the severity of airway obstruction; a pulsus paradoxus greater than 20 mm Hg correlates with moderate to severe obstruction. In addition to the utility of a pulsus paradoxus measurement in status asthmaticus, this finding may be seen with cardiac tamponade, pulmonary embolism, hypovolemic shock, or anaphylaxis.\n\nThe need for endotracheal intubation and mechanical ventilation has been significantly curtailed by careful monitoring and by the availability of less invasive modes of respiratory support, including high-flow nasal cannula oxygen, continuous positive airway pressure, and bilevel positive airway pressure support. The potential benefits of noninvasive positive pressure ventilation include a reduction in work of breathing, direct bronchodilating effects, prevention of atelectasis, and improvements in ventilation/perfusion mismatch.\n\nThe patient in this vignette has already received 60 mg of prednisone. The onset of action of the steroids will be delayed by hours and a further increase in dose will not provide short-term relief of bronchospasm nor is it likely to be of physiologic benefit. Given that the patient has a significant degree of airway obstruction, a further increase in the dose of the β-agonist is not likely to provide additional relief. Other adjunctive therapies such as intravenous terbutaline or heliox may be therapeutic options, but these are not included as answer choices. In a conscious adolescent who demonstrates relative normalcy of pulse oximetry, pH, and PCO2, endotracheal intubation is not indicated at this time. Lastly, there is no evidence of differential aeration or mediastinal shift to suggest a pneumothorax or a pleural effusion. Therefore, placement of a thoracostomy tube would not be expected to be therapeutic in this patient.\n\nPREP Pearls\n• Pulsus paradoxus measurements, in addition to clinical signs and symptoms and blood gas measurements, may provide helpful adjunctive evidence of impending respiratory failure in the patient with status asthmaticus.\n• Noninvasive modalities of respiratory support may improve ventilation, prevent atelectasis, and support a patient with moderate to severe degrees of airway obstruction without the need for endotracheal intubation and invasive ventilatory support.\n• β-adrenergic side effects of albuterol include tremulousness, elevated heart rate, and anxiety. These side effects are generally mild to moderate and well tolerated in the majority of patients.\n\nMOCA-Peds Objective\n• Recognize respiratory distress and manage appropriately\n\nABP Content Specifications(s)\n• Recognize the clinical features associated with toxicity to adrenergic agonists in a patient with an acute exacerbation of asthma\n• Recognize the signs of severe obstruction during an acute exacerbation of asthma\n\nSuggested Readings\n• Galant SP, Groncy CE, Shaw KC. The value of pulsus paradoxus in assessing the child with status asthmaticus. Pediatrics. 1978;61(1):46–51.\n• National Asthma Education and Prevention Program Expert Panel Report 3: Guidelines for the Diagnosis and Management of Asthma—summary report 2007. J Allergy Clin Immunol. 2007;120(5 suppl):S94–S138. https://www.nhlbi.nih.gov/files/docs/guidelines/asthsumm.pdf.\n• Soroksky A, Klinowski E, Ilgyev E, et al. Noninvasive positive pressure ventilation in acute asthmatic attack. Eur Respir Rev. 2010;19(115):39–45. doi: http://dx.doi.org/10.1183/09059180.00006109."}
{"id" : 2250, "question_text" : "A 2-year-old girl is brought to the emergency department by her parents with a 2-day history of fever, cough, and stridor. On physical examination, she is febrile (temperature 39.2°C), tachypneic (respiratory rate 48 breaths/min), and tachycardic (heart rate 145 beats/min). She has moderate intercostal and suprasternal retractions. Auscultation reveals inspiratory stridor and diminished breath sounds bilaterally. Of the following, the MOST likely diagnosis is", "options" : "[\"bacterial tracheitis\", \"laryngotracheitis\", \"peritonsillar abscess\", \"retropharyngeal abscess\"]", "explanation" : "Stridor is a high-pitched, musical breathing sound that is characterized by its timing during respiration: inspiration, expiration, or biphasic. Inspiratory stridor originates from obstruction in the extrathoracic airways, which includes the supraglottic (nasopharynx, epiglottis), glottic, and subglottic (vocal folds, extrathoracic trachea) areas; the subglottis is the most narrow part of the trachea. Expiratory stridor originates from obstruction in the intrathoracic airways, which includes the intrathoracic trachea and mainstem bronchi. Biphasic stridor originates from a fixed airway obstruction during both inspiration and expiration.\nAge, onset, and acuity are factors that help identify the cause of the stridor. The causes of stridor can be categorized as acute and chronic. Chronic stridor may be congenital or acquired. Congenital stridor most often presents in newborns and young infants. The causes of stridor are outlined in the Table.\nThe first step in the assessment of an infant or child with stridor is to identify those who require emergency intervention. Signs and symptoms consistent with the potential for rapid respiratory deterioration include severe retractions, cyanosis, nasal flaring, drooling, muffled voice, and altered mental status. Additional history taking and diagnostic testing should be delayed until after the child is clinically stable. \nA recent history of fever suggests an infectious cause of stridor. Infants with a history of prematurity who required prolonged intubation and mechanical ventilation are at increased risk of acquired subglottic stenosis. Children with a history of surgical intervention, especially cardiac surgery, are at risk for vocal fold paralysis or vocal fold paresis secondary to damage of the recurrent laryngeal nerve.\nThe physical examination may provide clues on the etiology of the stridor. Auscultation should be performed to identify whether the stridor is inspiratory, expiratory, or biphasic. Stridor should be differentiated from wheezing or stertor. Wheezing is a high-pitched sound that is usually present on expiration; it is caused by airflow obstruction of the distal intrathoracic airways. Stertor is usually low-pitched; it occurs secondary to obstruction of the nasopharyngeal or oropharyngeal areas. Skin should be evaluated for the presence of hemangiomas or café au lait spots, that could suggest an airway hemangioma or an upper airway neurofibroma, respectively.\nDiagnostic testing may include laboratory studies, imaging, spirometry, or even formal airway evaluation. If an infectious process is suspected, a complete blood count with differential and respiratory viral polymerase chain reaction testing may be helpful. Chest radiography may identify the presence of a mass/tumor, lymphadenopathy, a vascular ring, or radiopaque foreign body. Neck radiography may identify laryngotracheitis (\"steeple\" sign), epiglottitis (\"thumb\" sign), or retropharyngeal abscess. (If epiglottitis is suspected, radiography should generally not be performed; evaluation should take place in the operating room with staff skilled in airway management). Chest computed tomography, magnetic resonance angiography, or contrast esophagography may identify abnormal findings not visible on radiography or needing further detail. Spirometry is not appropriate for evaluation in the emergency department, but may be helpful to evaluate chronic stridor in children older than 6 years. Airway examination with nasolaryngoscopy or flexible bronchoscopy allows direct visualization of the airway, often providing a definitive diagnosis.\nManagement of stridor is dependent on the underlying etiology. Laryngotracheitis may be treated with systemic steroids and/or racemic epinephrine; hospitalization may be required. Bacterial tracheitis, epiglottitis, retropharyngeal abscess, and peritonsillar abscess require emergency intervention; intubation to protect the airway or surgical intervention may also be required, along with administration of appropriate antibiotics. Foreign-body aspiration requires airway evaluation and removal of the foreign body. Laryngomalacia and tracheomalacia usually do not require any intervention; laryngomalacia typically resolves between 12 and 18 months of age, while tracheomalacia usually resolves by 6 to 12 months of age. In severe cases of airway malacia, surgical intervention or even tracheostomy placement may be required. Subglottic stenosis will require surgical intervention. Vocal fold paralysis, most often a postsurgical complication, generally requires no treatment but should be closely followed due to the risk for aspiration.\nThe child in the vignette is febrile, tachypneic, and tachycardic, but does not have evidence of severe respiratory distress. Therefore, bacterial tracheitis and epiglottitis are unlikely the cause of her stridor. An acute foreign-body aspiration generally causes biphasic stridor, and is unlikely to present with a fever. \nSuggested Reading(s)\nEscobar ML, Needleman J. Stridor. Pediatr Rev. 2015;36(3):135-137. doi:10.1542/pir.36-3-135\nZoumalan R, Maddalozzo J, Holinger LD. Etiology of stridor in infants. Ann Otol Rhinol Laryngol. 2007;116(5):329-334. doi:10.1177/000348940711600503\nBoudewyns A, Claes J, Van de Heyning P. Clinical practice: an approach to stridor in infants and children. Eur J Pediatr. 2010;169(2):135-141. doi:10.1007/s00431-009-1044-7\nAlfin G. Vicencio, MD, John P. Bent, MD, 2016. \"Stridor (Chapter 197)\", American Academy of Pediatrics Textbook of Pediatric Care, Thomas K. McInerny, MD, FAAP, Henry M. Adam, MD, FAAP, Deborah E. Campbell, MD, FAAP, Thomas G. DeWitt, MD, FAAP, Jane Meschan Foy, MD, FAAP, Deepak M. Kamat, MD, PhD, FAAP, Rebecca Baum, MD, FAAP, Kelly J. Kelleher, MD, MPH, FAAP\nContent Domain\nPulmonology\nLearning Objectives\nAssess a child with stridor\nIdentify the various causes of stridor"}
{"id" : 2079, "question_text" : "A 17-year-old adolescent girl is seen for a health supervision visit. She was diagnosed with systemic lupus erythematosus 1 year ago after developing a rash on her chest. She has no kidney involvement. She is being treated with methotrexate, prednisone, and hydroxychloroquine. During a HEADSS (home and environment; education and eating; activities; drugs; sexuality; suicide, depression, and safety) examination, with her mother out of the room, she mentions that she takes her prednisone intermittently because she does not like that she has been gaining weight and thinks her face looks more round than usual. She has not discussed this issue with her rheumatologist. There is concern that her nonadherence with the prescribed medication regimen may cause a flare of her lupus.\n\nOf the following, the BEST next step in the management of this patient is to", "options" : "[\"disclose to her mother that she is not being adherent with one of her medications\", \"discuss the benefits of being adherent and that many medication adverse effects are temporary\", \"refer her to a psychiatrist for evaluation of depression and start of antidepressant medication\", \"use motivational interviewing to engage her in becoming more adherent with taking her medication\"]", "explanation" : "Correct Answer: D\nAdherence to a medical regimen is the degree to which a person is able to follow medical advice. Children and adolescents with chronic medical conditions often have issues with adherence to medication regimens. Based on the health belief model, a patient's adherence is based on one's perception of the severity of their medical condition and the benefit of the treatment.\n\nDepending on their stage of development, it can be difficult for an adolescent to understand the severity of a medical condition. In early adolescence (10-13 years of age), teenagers become more autonomous, start separating from family, begin adjusting to pubertal changes in their bodies, and tend to have concrete thought. In middle adolescence (14-17 years of age), they become more independent, their peer group can become more important than family, and conforming with peers is a priority. They also begin to develop abstract thought. Late adolescence (18-21 years) is characterized by comfort with one's identity, making plans for the future, further development of abstract thought, and a time of transition into a more adult role in society. The ability to think abstractly and factor in consequences is an important factor in an adolescent being able to follow medical advice.\n\nAdherence to a medical regimen can be derailed by numerous factors. Adolescents who have strong support systems and parents who are actively involved in their care, check in if medication is being taken on a daily basis, and attend medical appointments have higher rates of adherence. However, if the adolescent is not experiencing any symptoms from their disease, does not understand the seriousness of their condition, has numerous medications to take multiple times per day, and has experienced negative adverse effects from medications or treatments, they are more likely to not follow medical advice. This lack of adherence can lead to a poor prognosis, increased emergency department visits/hospitalizations, and death.\n\nThe adolescent in this vignette has systemic lupus erythematosus and has not been adhering to her medication plan because of unwanted adverse effects from her medication. To provide the best care to this patient, it would be useful to get her perspective on her medical condition, why she has been prescribed certain medications, and the potential consequences if she does not follow instructions. Motivational interviewing can be a useful tool to engage this adolescent in her own care, as opposed to lecturing in a more authoritarian style. It is a nonconfrontational style of counseling that allows the patient to play a role in his or her care by deciding if changes will be made that are beneficial to their treatment, what the changes would be, and when the changes would be implemented. It forces the provider to listen so that he or she can highlight the patient's change talk. Change talk is when a patient mentions a desire, ability, reason, or need to make a change in their behavior.\n\nThe other options mentioned in this vignette are related to understanding one's disease process and support networks, but do not directly encourage the patient to take an active role in their own health care. Involving the patient's parent may help with medication compliance but does not encourage the patient to take responsibility for their actions. A psychiatrist can be useful if the patient is describing signs and symptoms suspicious for depression. Many children and adolescents with chronic disease may experience depression secondary to their medical condition. Lastly, it is always recommended to review medication dosing and assess how often a patient is missing or skipping doses. This review allows the provider an opportunity to address any information the patient might have misunderstood. However, motivational interviewing is more likely to highlight the best treatment plan to get an adolescent engaged in their care.\n\nPREP Pearls\n\nMotivational interviewing can be a useful tool in engaging adolescents with chronic illness to be more engaged in their own care.\n\nAscertain the adolescent's perspective about their medical condition, medication regimen, and support system to devise realistic plans of care.\n\nABP Content Specifications(s)/Content Area\n\nUnderstand how to improve adherence to medical regimens by adolescent patients, including those with chronic illness, and the barriers to such adherence\n\nUnderstand factors that can affect adherence to health maintenance activities by adolescents\n\nSuggested Readings\n\nArrington-Sanders A. Adherence, in brief. Pediatr Rev. 2009:30(2);e9-e10. doi: 10.1542/pir.30-2-e9.\n\nMahan JD, Betz CL, Okumura MJ, Ferris ME. Self-management and transition to adult health care in adolescents and young adults: a team process. Pediatr Rev. 2017;38(7):305-317. doi: 10.1542/pir.2016-0074.\n\nSchaefer MR, Kavookjian J. The impact of motivational interviewing on adherence and symptom severity in adolescents and young adults with chronic illness: a systematic review. Patient Educ Couns. 2017;100(12):2190-2199. doi:10.1016/j.pec.2017.05.037."}
{"id" : 2351, "question_text" : "A 16-month-old child is seen in the clinic following 3 days of nonbloody diarrhea and fever. His last urine output, a small volume 4 hours ago, was visibly concentrated. His weight today is 11.6 kg, which is decreased from 12.4 kg at his well visit 3 weeks earlier. On physical examination, his blood pressure is 70/55 mm Hg, heart rate is 135 beats/min, respiratory rate is 25 breaths/min, and temperature is 37.8 °C. He is alert but appears tired. He has tacky mucous membranes and is not making tears. The remainder of his examination findings are unremarkable. Of the following, the BEST estimate of this child's level of dehydration is", "options" : "[\"4%\", \"6%\", \"8%\", \"10%\"]", "explanation" : "PREP Pearl(s)\nDehydration is a common complication of illness in young children.\nIt is important to use physical examination and other objective findings (eg, prior weight) to determine a child's degree of dehydration to implement appropriate treatment.\nJuice, broth, and sports drinks are not appropriate fluids for the oral rehydration of children with moderate dehydration.\nCritique\nThe best estimate is that the boy in the vignette is approximately 6% dehydrated. Dehydration due to illness, particularly from diarrhea, is a common event among young children. Children in this age group are at increased risk for dehydration for multiple reasons, including the following:\nInfants' high body surface area to weight ratio results in higher fluid requirements.\nCommon childhood illnesses often include fever, vomiting, and diarrhea, each of which leads to excess fluid loss.\nYoung children are dependent on others to meet their needs and cannot independently increase their fluid intake.\nDetermination of a child's degree of dehydration, classified as mild (3%-5% volume loss), moderate (6%-9% volume loss), or severe (≥10% volume loss), is often difficult but is an important guide for management. On physical examination, children with moderate dehydration can be expected to have dry mucous membranes, decreased skin turgor, irritability, tachycardia, increased capillary reflow time, and decreased urine output and tear production. If a previous weight is available, calculation of the decrease in the child's weight makes the estimate of dehydration more accurate. The following formula is used to calculate a child's degree of dehydration:\n[Previous Weight (kg) − Current Weight (kg)] / Previous Weight (kg) × 100 = Percentage of Dehydration\nFor the child in the vignette, the calculation would be as follows:\n(12.4 − 11.6) / 12.4 × 100 = 0.8/12.4 × 100 = 6.4% (moderately dehydrated)\nWhen dehydration occurs, the body attempts to maintain water and electrolyte balance by shifting fluid from the intracellular fluid (ICF) space to the extracellular fluid (ECF) space and by secreting antidiuretic hormone to decrease the urinary excretion of fluid. In addition, receptors in the hypothalamus cause signals to be sent that increase thirst and the desire for salt.\nIsotonic (isonatremic) dehydration is the most common type; it results from a net loss of isotonic fluid containing sodium and potassium. Sodium, the primary ECF cation, is lost from the body and through a shift into the ICF compartment to balance the body's potassium loss. Sodium will shift back from the ICF space to the ECF space during rehydration as potassium levels are restored. The water deficit in dehydration comes primarily from the ECF compartment; there is minimal net loss of fluid from the ICF space.\nThe management of moderate to severe dehydration generally involves the administration of 20 mL/kg intravenous (IV) fluid boluses of normal saline followed by 1 to 2 days of IV hydration with a solution containing sodium and glucose until the child is able to take adequate oral fluids. The volume of fluid and electrolytes administered is determined by calculations that include the percentage of dehydration, predicted additional losses, plus estimated maintenance fluid requirements.\nWhen possible, the preferred method of rehydration for children with moderate, acute dehydration is to rapidly restore ECF losses and then administer oral rehydration fluid (ORF) therapy. It is important to ensure that an appropriate ORF is being used. Attempting rehydration with juice, broth, or sports drinks is not recommended because these fluids do not contain the correct sodium to glucose ratio to promote salt and water reabsorption in the gastrointestinal tract.\nSuggested Reading(s)\nChandramohan G. Management of dehydration in children: fluid and electrolyte therapy. In: Berkowitz CD, ed. Berkowitz's Pediatrics: A Primary Care Approach. 6th ed. American Academy of Pediatricians; 2021.\nMahajan P. Dehydration. In: McInerny TK, Adam HM, Campbell DE, et al, eds. American Academy of Pediatrics Textbook of Pediatric Care. American Academy of Pediatrics; 2016:chap 353. Pediatric Care Online\nMoritz ML, Ayus JC. Misconceptions in the treatment of dehydration in children. Pediatr Rev. 2016;37(7):e29–e31. doi:10.1542/pir.2016-003\nPowers KS. Dehydration: isonatremic, hyponatremic, and hypernatremic recognition and management. Pediatr Rev. 2015;36(7):274–285. doi:10.1542/pir.36-7-274\nContent Domain\nFluids and Electrolytese\nABP Content Specification(s) / Content Area(s)\nUnderstand the role of changes in extracellular fluid volume in the development of dehydration\nRecognize the laboratory abnormalities associated with isotonic dehydration, and manage appropriately\nThe correct answer is: 6%\nView Peer Results"}
{"id" : 2522, "question_text" : "A 14-year-old adolescent girl is evaluated in the emergency department for fever of 3 days' duration, sore throat, dysphagia, and a slightly muffled voice. She is otherwise healthy, fully immunized, and does not have a history of recurrent throat infections. Her family history is unremarkable. On physical examination, her temperature is 39°C, heart rate is 100 beats/min, respiratory rate is 18 breaths/min, and oxygen saturation is 99% in room air. She is in mild discomfort due to her throat pain but is speaking comfortably in complete sentences. Her left tonsil is 3+ enlarged and erythematous, her right tonsil is 1+, and her uvula is deviated to the right. She has enlargement and tenderness of her left cervical lymph nodes. The remainder of her physical examination findings are unremarkable. Of the following, the MOST appropriate next management step for this adolescent is", "options" : "[\"drainage of the abscess\", \"intravenous antibiotics\", \"observation only\", \"tonsillectomy\"]", "explanation" : "The adolescent in the vignette has a peritonsillar abscess (PTA); drainage of the abscess is the most appropriate first step in treatment. Intravenous antibiotics may be administered as the first step in treatment for individuals with certain complications resulting from PTAs (eg, septic thrombophlebitis). Observation alone is not appropriate management and may result in complications. Tonsillectomy for an acute infected PTA may be performed in certain individuals, such as those who would require general anesthesia for incision and drainage and those with a history of recurrent tonsil infections, but would not be the appropriate first-line treatment for this otherwise healthy adolescent.\n\nPeritonsillar abscesses occur when pus collects between the pharyngeal muscles and the palatine tonsil capsule. The most common inciting pathogens are Streptococcus and Fusobacterium species. Common clinical manifestations include fever, sore throat, dysphagia, muffled voice, asymmetric tonsils, and uvular deviation.\n\nThe diagnosis of a PTA is usually made clinically. If the diagnosis is uncertain, intraoral ultrasonography or computed tomography of the neck with intravenous contrast may be performed to assess for the presence of a fluid collection or neck mass, taking into consideration the risks of sedation and radiation.\n\nTreatment of an uncomplicated PTA is usually drainage under local anesthesia with a subsequent 10-day course of oral antibiotics, usually a penicillin, cephalosporin, or clindamycin. After abscess culture and sensitivities are resulted, antibiotics can be directed at the causative organism.\n\nSome children and adolescents with PTA may require hospital admission, including those who experience complications.\n\nIndications for admission in PTA\n• The need for intravenous hydration due to poor oral intake\n• pain management\n• no reliable outpatient follow-up\n• management of complications after drainage such as severe bleeding or respiratory distress secondary to aspiration of abscess contents into the patient's airway\n\nPREP Pearls\n• Peritonsillar abscesses typically present with unilateral tonsillar enlargement and uvular deviation.\n• Uncomplicated peritonsillar abscesses may be treated with drainage under local anesthesia and subsequent oral antibiotics.\n\nABP Content Specifications(s)\n• Recognize the clinical findings associated with peritonsillar abscess\n• Plan the appropriate management of a peritonsillar abscess\n• Plan the appropriate diagnostic evaluation of a peritonsillar abscess, considering commonly associated pathogens\n\nSuggested Readings\n• Bochner RE, Gangar M, Belamarich PF. A clinical approach to tonsillitis, tonsillar hypertrophy, and peritonsillar and retropharyngeal abscesses. Pediatr Rev. 2017;38(2):81-92. doi:10.1542/pir.2016-0072.\n• Conrad C, Cornfield DN. Airway obstruction. In: McInerny TK, Adam HM, Campbell DE, DeWitt TG, Foy JM, Kamat DM, eds. American Academy of Pediatrics Textbook of Pediatric Care. American Academy of Pediatrics; 2021:chap 348. Accessed September 1, 2022. Pediatric Care Online .\n• Mitchell RB, Archer SM, Ishman SL, et al. Clinical practice guideline: tonsillectomy in children (update). Otolaryngol Head Neck Surg. 2019;160(1_suppl):S1-S42. doi:10.1177/0194599818801757."}
{"id" : 117, "question_text" : "You are evaluating a 5-year-old boy who is hospitalized in the pediatric intensive care unit with findings of poor perfusion, renal failure, and respiratory compromise requiring intubation. You note an abnormality on the cardiac monitor (Item Q5). Of the following, the MOST likely cause of this patient's electrocardiographic findings is", "options" : "[\"hyperkalemia\", \"hypernatremia\", \"hypocalcemia\", \"hypokalemia\", \"hyponatremia\"]", "explanation" : "Peaked T waves on rhythm strip or electrocardiography (ECG), as shown for the boy in the vignette, are indicators of hyperkalemia. Other electrocardiographic findings might include atrioventricular block, widening of the QRS complex, and degeneration of the ECG complex into a sinusoidal shape. Elevated potassium concentrations appear to have a direct effect on the potassium channels, increasing their activity and speeding membrane repolarization. Hyperkalemia causes an overall membrane depolarization that inactivates many sodium channels. The faster repolarization of the cardiac action potential causes the tenting of the T waves, and the inactivation of sodium channels causes a sluggish conduction of the electrical wave around the heart, which leads to widening of the QRS complex. The serum potassium concentration at which electrocardiographic changes develop is somewhat variable.\n\nHyperkalemia has many causes. Hypernatremia and hyponatremia are not typically associated with rhythm irregularities or electrocardiographic disturbances. Hypocalcemia can result in prolongation of the QT interval. This type of electrical instability puts the patient at high risk for torsades de pointes, a specific type of ventricular fibrillation. Hypokalemia can cause flattened or inverted T waves, ST depression, and a prolongation of the QT interval.\n\nCritique: [As above]\n\nContent Specifications: Know the signs of hyperkalemia."}
{"id" : 2270, "question_text" : "A 17-year-old female is seen in the office for a health supervision visit. She has a history of moderate depression and type 3 Ehlers-Danlos syndrome. She had previously experienced intermittent joint pain in her knees and elbows with physical activity, which improved with physical therapy. Over the past 4 months, she has developed generalized full-body pain involving large muscle groups, and worsening fatigue. Physical examination demonstrates generalized joint hypermobility with a Beighton score of 8 out of 9. No joint effusions or abnormalities were seen. She has significant tenderness to palpation over many large muscle groups, including the pectoral, deltoid, trapezius, paraspinal, quadriceps, hamstring, and gastrocnemius muscles. Tenderness is also noted over the abdomen in all 4 quadrants and along the chest wall. Strength testing is normal in the upper and lower extremities. Laboratory tests show: White blood cell count 5,600/μL (5.6 x10⁹/L), Hemoglobin 13.8 g/dL (138.0 g/L), Platelet count 225 ×10³/µL (225 x 10⁹/L), Creatine kinase 134 U/L, Aspartate aminotransferase 21 U/L, Alanine aminotransferase 23 U/L, Thyroid stimulating hormone 2.7 µIU/mL, C-reactive protein <0.2 mg/dL (2.0 mg/L), Antinuclear antibody <1:40 titer, Anti-double–stranded DNA Negative. Of the following, the BEST intervention to address this patient's pain is (a)", "options" : "[\"bracing of the bilateral knees\", \"cognitive behavioral therapy\", \"decreasing physical activity\", \"nonsteroidal anti-inflammatory drug\"]", "explanation" : "Pediatric fibromyalgia is a condition of widespread musculoskeletal pain with associated fatigue, cognitive dysfunction, and psychiatric symptoms that can present with multiple somatic complaints.\nPediatric fibromyalgia can lead to stiffness in joints and tendons mimicking inflammatory arthritis.\nTreatment of pediatric fibromyalgia places emphasis on nonpharmacologic modalities, such as cognitive behavioral therapy, stress reduction, physical activity, and desensitization therapy.\nCritique\nThe adolescent described in the vignette has developed fibromyalgia/amplified pain disorder along with Ehlers-Danlos syndrome, which will be best addressed through cognitive behavioral therapy.\nAmplified musculoskeletal pain disorders are a group of conditions that include complex regional pain syndrome, pediatric fibromyalgia (diffuse amplified pain), and intermittent amplified pain. Clinical symptoms include excessive pain sensitivity and allodynia (ie, pain in response to stimuli that do not usually cause pain, such as light touch), which may start in a focal area before radiating to other areas of the body. Individuals with amplified musculoskeletal pain disorders may develop both localized and/or systemic features of autonomic dysfunction. Localized involvement of the skin may include vasomotor dysregulation and thermoregulatory dysfunction, leading to areas that are either erythematous and hot to the touch, or cold and mottled in the limbs. Amplified pain disorders can also present with musculoskeletal symptoms.\nFibromyalgia is characterized by widespread musculoskeletal pain associated with fatigue, cognitive dysfunction, and psychiatric symptoms, and can be associated with multiple somatic symptoms (Table 1). Although the etiology is unknown, it is considered a neurosensory disorder of dysregulated pain. Fibromyalgia occurs more commonly in females, with a prevalence of approximately 8% compared with 5% in men.\nAmong the pediatric population, fibromyalgia is mostly seen in adolescents. Although primarily associated with pain, it can also lead to stiffness in the joints and tendons and may mimic the symptoms of inflammatory arthritis. Fibromyalgia is not a life-threatening condition, nor does it cause musculoskeletal damage or deformity. However, it significantly impacts quality of life. Frequent comorbidities include anxiety and depression, and symptoms often lead to frequent absences from school or other activities.\nFibromyalgia is a diagnosis of exclusion. While there are no diagnostic or specific laboratory abnormalities associated with fibromyalgia, laboratory tests are necessary to exclude other conditions. Recommended laboratory tests include muscle enzymes, inflammatory markers, renal and hepatic function, thyroid-stimulating hormone, complete blood count, and autoimmune testing (eg, rheumatoid factor, antinuclear antibody, lupus-specific antibodies, anti–double-stranded DNA, and anti-Smith). The diagnostic criteria for fibromyalgia are outlined in Table 2.\nTreatment of fibromyalgia is often multifaceted, with a limited role for pharmacotherapies. Cognitive behavioral therapy can provide beneficial stress reduction and treatment of underlying mood disorders. Desensitization therapy through physical activity is effective in decreasing symptoms and also aids in stress management. Medication generally does not treat the underlying condition but can reduce symptoms, allowing the individual to better adhere to nonpharmacological modalities. Medications can include neuropathic pain agents (eg, gabapentin, pregabalin), selective serotonin reuptake inhibitors, muscle relaxants (eg, cyclobenzaprine), or tricyclic antidepressants. Acetaminophen and nonsteroidal anti-inflammatory drugs offer minimal to no benefit for pain symptoms.\nBracing of the knee would not be helpful in this case. The pain is more widespread and consistent with fibromyalgia; it is not associated with joint instability. A decrease in physical activity can be detrimental in the long term and worsen the child's pain. Nonsteroidal anti-inflammatory drugs provide no significant improvement in pain in individuals with fibromyalgia.\nSuggested Reading(s)\nAli A, McCarthy PL. Complementary and integrative methods in fibromyalgia. Pediatr Rev. 2014;35(12):510–518. doi:10.1542/pir.35-12-510\nGottlieb B. Connective tissue disease, CFS, fibromyalgia. In: Fisher MM, Alderman EM, Kreipe RE, Rosenfeld WD, eds. Textbook of Adolescent Health Care. American Academy of Pediatrics; 2011: https://publications.aap.org/aapbooks/book/589/chapter/5820356/Connective-Tissue-Disease-CFS-Fibromyalgia\nKashikar-Zuck S, Cunningham N, Sil S, et al. Long-term outcomes of adolescents with juvenile-onset fibromyalgia in early adulthood. Pediatrics. 2014;133(3):e592–e600. doi:10.1542/peds.2013-2220\nSabine KB, Ward C. Managing chronic pain in children. In: McInerny TK, Adam HM, Campbell DE, DeWitt TG, Foy JM, Kamat DM, eds. American Academy of Pediatrics Textbook of Pediatric Care. American Academy of Pediatrics; 2021. Accessed September 21, 2024. Pediatric Care Online\nContent Domain\nRheumatology\nLearning Objectives\nRecognize clinical findings associated with fibromyalgia"}
{"id" : 1818, "question_text" : "A 14-year-old adolescent girl is admitted to the pediatric intensive care unit for diabetic ketoacidosis. She was diagnosed with type 1 diabetes at 8 years of age. This admission is her third in the past year for diabetic ketoacidosis. Her diabetes is managed with 22 units of insulin glargine subcutaneously at bedtime and bolus doses of rapid-acting insulin. During the 12 hours prior to admission, she had diffuse abdominal pain and vomiting without fever. She weighs 45 kg. Laboratory data at the time of admission are shown: Laboratory Test Result Glucose 486 mg/dL (27 mmol/L) Serum bicarbonate 8 mEq/L (8 mmol/L) Venous pH 7.15 White blood cell count 17,000/µL (17.0 × 109/L) Of the following, the MOST likely precipitating factor of her diabetic ketoacidosis is", "options" : "[\"excessive carbohydrate intake\", \"gastroenteritis\", \"inadequate basal insulin dose\", \"insulin omission\"]", "explanation" : "The patient in this vignette has recurrent diabetic ketoacidosis (DKA) with 3 episodes in the past year. The most common cause of recurrent DKA is insulin omission. Adolescent girls are a higher risk group for recurrent DKA. Often, recurrent DKA is a symptom of an underlying psychosocial problem. Examples include intentional insulin omission for weight loss (\"diabulimia\"), depression, poor coping skills, or a mechanism of escape from an undesirable social situation. Thus, recurrent DKA should prompt a behavioral health evaluation. An interdisciplinary approach to the management of recurrent DKA includes diabetes education, psychosocial evaluation, medical treatment, and appropriate adult supervision.\n\nThe most common cause of DKA in individuals whose diabetes is managed with an insulin pump is interruption of insulin delivery, usually caused by a compromised infusion site. Insulin pumps deliver only rapid-acting insulin, so when interrupted, DKA occurs more quickly than in individuals receiving a long-acting basal insulin injection. Education is critical for the prevention of DKA in individuals on an insulin pump. Ketones should be monitored at home during times of significant hyperglycemia and on sick days. Additional insulin and oral fluid are needed if ketones are present, and the additional insulin should be administered by injection. Families should also have 24-hour access to medical advice by telephone.\n\nInfection is a rare cause of recurrent DKA. Proper education on sick-day management is essential for preventing DKA. Sick-day management includes close monitoring of blood glucose and ketone levels, as well as 24-hour telephone access to medical advice. Extra insulin is often needed because of the stress of illness. Abdominal pain and vomiting are symptoms of DKA that are often confused with gastroenteritis and resolve with treatment of the DKA. An elevated white blood cell count, as seen in the patient in this vignette, is associated with DKA due to the stress response. It should not be interpreted as a sign of infection without other indications of infection.\n\nExcessive carbohydrate intake does not cause DKA. Insulin deficiency to the extent that the body's basic fuel requirements are not met causes DKA. Excessive carbohydrate intake without adequate bolus insulin coverage causes hyperglycemia but not DKA as long as there is enough insulin to deliver enough glucose to meet the body's basic fuel requirements.\n\nInadequate basal insulin dose is not the cause of recurrent DKA for the patient in this vignette. She is prescribed 0.5 units/kg/day of long-acting basal insulin glargine, which is a typical dose for her age. Even if her prescribed dose is not keeping her blood glucose levels in the target range, it is enough to prevent DKA.\n\nPREP Pearls\n\nThe most common cause of recurrent diabetic ketoacidosis is insulin omission.\n\nRecurrent diabetic ketoacidosis is often a symptom of an underlying psychosocial problem.\n\nDiabetes education on sick-day management and insulin pump malfunction is critical for diabetic ketoacidosis prevention.\n\nABP Content Specifications(s)/Content Area\n\nDifferentiate the clinical and laboratory findings associated with adrenal insufficiency from those of the inappropriate secretion of antidiuretic hormone\n\nRecognize the clinical and laboratory manifestations of adrenal insufficiency\n\nSuggested Readings\n\nJefferies CA, Nakhla M, Derraik JG, Gunn AJ, Daneman D, Cutfield WS. Preventing diabetic ketoacidosis. Pediatr Clin North Am. 2015;62(4):857-871. doi:10.1016/j.pcl.2015.04.002.\n\nWolfsdorf JI, Allgrove J, Craig ME, et al; International Society for Pediatric and Adolescent Diabetes. ISPAD Clinical Practice Consensus Guidelines 2014. Diabetic ketoacidosis and hyperglycemic hyperosmolar state. Pediatr Diabetes. 2014;15 Suppl 20:154-179. doi:10.1111/pedi.12165."}
{"id" : 3547, "question_text" : "A critically ill child with septic shock is on vasopressor support. Regarding enteral nutrition, what does the evidence recommend?", "options" : "[\"Enteral feeding should not be withheld solely because the patient is receiving vasoactive-inotropic medications\", \"All nutrition should be withheld until vasopressor support is discontinued\", \"Parenteral nutrition should be started immediately and continued throughout the PICU stay\", \"Enteral feeding increases infection risk and should be avoided in septic patients\"]", "explanation" : "The text recommends not withholding enteral feeding solely on the basis of vasoactive-inotropic medication administration, and suggests enteral nutrition as the preferred method of feeding with parenteral nutrition withheld in the first 7 days of PICU admission."}
{"id" : 892, "question_text" : "A 15-year-old, African-American girl with systemic lupus erythematosus and lupus nephritis presents to your office for her annual health supervision visit. She has been doing very well and has not had any symptoms suggestive of active disease. Her current medications include prednisone, hydroxy-chloroquine, ranitidine, simvastatin, mycophenolate mofetil, enalapril, depot medroxyprogesterone, vitamin D with calcium, and a multivitamin. She asks you which of these medications are keeping her lupus disease under control. Of the following, the response you are MOST likely to give is", "options" : "[\"enalapril, hydroxychloroquine, mycophenolate mofetil, prednisone, and simvastatin\", \"enalapril, hydroxychloroquine, mycophenolate mofetil, and prednisone\", \"hydroxychloroquine, mycophenolate mofetil, and prednisone\", \"hydroxychloroquine, mycophenolate mofetil, prednisone, and simvastatin\", \"mycophenolate mofetil, ranitidine, and prednisone\"]", "explanation" : "Systemic lupus erythematosus (SLE) is a complex disease managed with a multisystem approach, as noted in the girl in the vignette. A listing of the various drugs used to treat SLE is shown (Item C186, page C-147) Hydroxychloroquine maintains remission in patients who have SLE and controls skin involvement and disease activity. Mycophenolate mofetil is used as induction and maintenance therapy in lupus nephritis and controls disease activity. Prednisone is also used to control lupus disease activity. Enalapril would be used to control hypertension related to lupus nephritis but would not control SLE disease activity. Simvastatin can reduce the hypercholesterolemia seen in lupus patients and reduces the risk of early atherosclerosis, but it would not control lupus activity. Ranitidine is often used in patients who have lupus for gastrointestinal prophylaxis while on steroids but would not control lupus activity.\n\nPrior to 2011, only 3 drugs-aspirin, hydroxychloroquine, and prednisone-were approved by the US Food and Drug Administration (FDA) for the treatment of SLE in adults. The FDA recently approved belimumab for adult SLE. There are no FDA-approved medications for the treatment of pediatric lupus patients. Live vaccines should be avoided while a lupus patient is on immunosuppressive therapy.\n\nPREP Pearls\n• Hydroxychloroquine helps to maintain remission and treats skin involvement in patients who have lupus.\n\nAmerican Board of Pediatrics Content Specification(s):\n• Know which drugs are useful in the treatment of systemic lupus erythematosus\n\nSuggested Reading:\n• Levy DM, Kamphuis S. Systemic lupus erythematosus in children and adolescents. Pediatr Clin N Am. 2012;59(2):345-364. doi:10.1016/j. pc1.2012.03.007\n• Weiss JE. Pediatric systemic lupus erythematosus: more than a positive antinuclear antibody. Pediatr Rev. 2012;33(2):62-73. doi:10.1542/pir.33-2-62"}
{"id" : 2049, "question_text" : "A 7-year-old boy is being evaluated for \"white spots\" on his face that developed 6 weeks ago. He has no associated symptoms and has been in good health. The physical examination findings are notable only for the lesions shown in Item Q251. Of the following, the MOST appropriate treatment is", "options" : "[\"clotrimazole topically\", \"emollient topically\", \"triamcinolone topically\", \"ultraviolet B phototherapy\"]", "explanation" : "The boy in this vignette has hypopigmented scaling macules on his face. The borders of the lesions are not sharply demarcated; rather, there is a gradual transition from normal to abnormal pigmentation. These features are most consistent with pityriasis alba and, accordingly, treatment with an emollient is appropriate. Some clinicians treat with a low-potency topical corticosteroid (eg, hydrocortisone 1% or 2.5%) for 7 days, but using a midpotency preparation like triamcinolone is not advisable because of the potential for skin atrophy. Tinea versicolor may cause hypopigmented macules that occasionally may involve the face. However, it is an uncommon infection in children, and the borders of lesions are well defined. Localized infection may be treated with clotrimazole. The lesions of vitiligo are depigmented macules or patches that have well-defined borders; scaling is not present. First-line treatment usually is with a topical corticosteroid or a topical calcineurin inhibitor. Other options include narrowband ultraviolet B phototherapy, photochemotherapy using psoralen and ultraviolet A, and excimer laser.\n\nPityriasis alba is a form of postinflammatory hypopigmentation that often occurs in children who have a history of atopic dermatitis. Lesions commonly involve the face but may occur on the trunk and extremities. Pityriasis alba often becomes apparent after sun exposure because normal skin tans but affected areas do not. The diagnosis is made clinically based on the characteristic appearance of lesions. Regardless of the treatment selected, the patient and family should be counseled that the return of normal pigmentation takes months. In addition, they should be instructed to treat new lesions (pink, scaling macules) with a low-potency topical corticosteroid to prevent new areas of hypopigmentation.\n\nPREP Pearls\n\nPityriasis alba is characterized by hypopigmented macules that may have associated scale. The borders of lesions are indistinct, with a gradual transition from normal to abnormal pigmentation.\n\nPityriasis alba is a form of postinflammatory hypopigmentation that often occurs in children who have a history of atopic dermatitis.\n\nTreatment of pityriasis alba is with an emollient or a short course (7 days) of a low-potency topical corticosteroid.\n\nABP Content Specifications(s)/Content Area\n\nRecognize the clinical features of pityriasis alba\n\nRecognize the clinical manifestations of vitiligo\n\nSuggested Readings\n\nAmerican Academy of Pediatrics Section on Dermatology. Pityriasis alba. In: Mancini AJ, Krowchuk DP, eds. Pediatric Dermatology: A Quick Reference Guide. 3rd ed. Elk Grove Village, IL: American Academy of Pediatrics; 2016:405-407."}
{"id" : 2109, "question_text" : "A 3-year-old boy is brought to the emergency department after his preschool teacher noticed bruising on his lower extremities. On the basis of the teacher's concerns about the bruising, school staff made a report to child protective services, and a social worker advised the parents to bring him to the emergency department for evaluation. The child appears well. His mother reports that he has been in good health, with normal growth and development. Aside from the bruises on his legs (Figure 1), the boy's physical examination findings are unremarkable. Of the following, the BEST next step in this child's evaluation is to", "options" : "[\"arrange for a forensic interview\", \"gather a history from the parent(s)\", \"obtain laboratory testing\", \"obtain a skeletal survey\"]", "explanation" : "The 3-year-old child in the vignette has bruising on his lower extremities. The best next step in his evaluation is to obtain a detailed history from his parent(s). The boy has no notable medical history, appears healthy, and is developing normally. His bruises involve the anterior lower extremities, a common location for minor injuries resulting from the daily activities of very young children. Although additional evaluation (eg, a forensic interview, skeletal survey, laboratory testing) may be warranted in certain cases of bruising, completing a careful history and physical examination is a critical first step and may be all that is necessary.\n\nInjuries in children are common. Bruising specifically, can pose a challenge in distinguishing accidental from nonaccidental etiologies; easy bruising can occur with some medical conditions. It is important to gather a detailed history, perform a thorough physical examination, and obtain further studies as indicated. A consistent approach to the assessment of bruising can aid in the recognition of suspicious injuries, particularly minor injuries suggestive of child abuse (sentinel injuries), and potentially prevent further abuse. Any injury that raises concern about an abusive etiology must be reported to child protective services.\n\nFor a child with bruising, an important next step after the initial assessment of injury severity and stabilization (as needed) is to obtain a thorough history. In addition to the standard elements, the history should include questions focused on determining the underlying etiology for bruising (Table 1). It is helpful to speak with parents, other caregivers, and witnesses separately; a forensic interview of the child may be indicated in certain cases. For a child with bruising or other injury, findings that may indicate abuse include the absence of a history of trauma, a change in the reported details, a delay in seeking care for observed injuries, inconsistencies between the history provided and the child's developmental abilities, the child's age, the mechanism of injury, the pattern of injury, and/or the severity of injury.\n\nAlthough bruises are a very common finding in children, they can be easily missed. Ambulatory children tend to bruise over bony prominences on the front of the body. The TEN-4-FACESp rule reminds practitioners about physical examination findings that should raise concern about physical abuse (Table 2). Any bruising in infants younger than 4 months of age should raise concern regarding abuse. It is important to take the child's skin color into consideration when evaluating bruising, as the appearance of bruises can vary with different skin tones. In addition, past guidance related to determining the age of bruises on the basis of their color is no longer supported.\n\nTable 2 outlines testing to consider when a child's injury history and physical examination findings are consistent with a high suspicion of abuse, such as patterned bruising (Figure 2). When indicated, laboratory or radiographic tests can aid in assessing for occult injuries or an underlying medical condition that may predispose a child to bruising; extensive testing is not warranted in many cases. Hematology consultation should be obtained when abnormal test results or other findings raise concern regarding an underlying bleeding disorder (Figure 3). Other conditions included in the differential diagnosis of a child with skin findings that have the appearance of bruising include congenital dermal melanocytosis (Figure 4), vasculitis (Figure 5), cancer, phytophotodermatitis (Figure 6), and lesions occurring as a result of various cultural practices (eg, cupping, coining, spooning) (Figure 7).\n\nSuggested Reading(s)\nAnderst J, Carpenter SL, Abshire TC, Killough E; AAP Section on Hematology/Oncology, AAP Section on Child Abuse and Neglect. Evaluation of children with suspected physical abuse. Pediatrics. 2022;150(3):e2022058887. doi:10.1542/peds.2022-058887\nChristian CW; Committee on Child Abuse and Neglect. The evaluation of suspected child physical abuse. Pediatrics. 2015;135(5):e20150356. doi:10.1542/peds.2015-0356\nGlick JC, Lorand MA, Bilka KR. Physical abuse of children. Pediatr Rev. 2016;37(4):146-158. doi:10.1542/pir.2015-0012\n\nContent Domain\nChild Maltreatment, Physical abuse\n\nLearning Objectives\nDevelop a systematic approach to evaluating bruising in children\nDifferentiate between accidental and inflicted bruising"}
{"id" : 3217, "question_text" : "A previously healthy 4-week-old, full-term male patient is brought to the emergency department because he stopped breathing for approximately 20 seconds and turned blue around the lips. He has also had slight nasal congestion and poor oral intake today. His mother did not report any complications during pregnancy. He was born at term and discharged home in 2 days. He usually drinks four 6-oz bottles of formula per day, and today he is only finishing 2 oz of each bottle. His 2 older siblings have been ill with cough and rhinorrhea. Vital signs show a temperature of 38.0°C, pulse rate of 150 beats/min, respiratory rate of 60 breaths/min, blood pressure of 80/40 mm Hg, and oxygen saturation of 93% on room air. Physical examination shows a tired-appearing but well-developed, well-nourished baby. Anterior fontanelle is soft, open, and flat. Eyes and oral mucous membranes are erythematous. Pupils are 2 mm, equal, and reactive. His heart has a regular rate and rhythm, and extremities are warm and well-perfused, with capillary refill time of 2 s. Respiratory examination shows tachypnea with mild intercostal retractions, nasal flaring, and coarse breath sounds bilaterally. Abdomen is soft, nontender, nondistended, and with no organomegaly. Supplemental oxygen by nasal cannula is initiated with deep suctioning of the nasopharynx and results in improvement of oxygen saturation to 100%. The infant is observed to have 2 separate periods of apnea that last 15 seconds and are associated with a drop in oxygen saturation to 80% that resolves with gentle stimulation. Of the following, the MOST appropriate diagnostic test at this time is", "options" : "[\"computed tomography of brain\", \"electroencephalogram\", \"lumbar puncture\", \"nasal secretions for respiratory syncytial virus\", \"sleep study\"]", "explanation" : "Preferred Response: D\nThe infant in this vignette most likely has apnea from a respiratory tract infection, based on the history of being full term, previously healthy, with upper respiratory tract symptoms, decreased oral intake, and the presence of sick contacts. The duration of the respiratory pause and perioral cyanosis indicates a serious event of apnea. Although several different respiratory tract infections can cause apnea, respiratory syncytial virus (RSV) is one of the most common. Evaluation of nasal secretions for respiratory viruses is the most appropriate diagnostic test.\n\nApnea is the clinical condition of the absence of respiratory air flow. The 3 general categories of apnea include central apnea from insufficient respiratory drive originating from the brainstem, obstructive apnea from upper airway obstruction, and a mixed etiology including both central and obstructive causes (Item C156). Central apnea in neonates can be caused by a relatively immature brainstem respiratory center and is a very common problem in preterm infants. It can also be caused by other illnesses that can affect the respiratory center in both newborns and older children, such as brain hemorrhage, drugs, seizures, hypoxic injury, or increased intracranial pressure. A mixed etiology of central and obstructive apnea is also common in premature neonates, from the combination of an immature brainstem as well as an underdeveloped upper airway. This problem usually resolves by 37 to 40 weeks of gestational age. Obstructive apnea, which can be caused by airway obstruction at any point from the pharynx to the distal trachea, can occur both in infants and older children. In infants, common causes include macroglossia, Pierre-Robin sequence, subglottic stenosis, and laryngotracheomalacia, leading to intermittent collapse of the airway. In older children, common causes of obstructive sleep apnea include enlarged tonsils or adenoids, nasal polyps, obesity, and insufficient upper airway tone from trisomy 21 or neuromuscular conditions. Central apnea can be distinguished from periodic breathing, which can reflect short pauses in breathing, by the presence of apnea for periods of longer than 15 to 20 seconds, cyanosis, hypotonia, bradycardia, or pallor.\n\nItem Cl 56: Differential Diagnosis of Infantile Apnea\n• Normal respiratory pauses\n  o Mild apnea associated with choking\n  o Periodic breathing during sleep\n  o Occasional 5- to 15-second apneas during sleep\n• Acute illnesses associated with apnea\n  o Sepsis\n  o Acute neurologic syndromes\n  o Infantile botulism\n  o Respiratory syncytial virus infection\n  o Pertussis\n  o Other\n• Chronic conditions associated with apnea\n  o Convulsions\n  o Gastroesophageal reflux, sensitive laryngeal chemoreceptors\n  o Cardiac dysrhythmias, \"prolonged Q-T\" syndrome\n• Abnormalities of respiratory control\n  o Immature respiratory center\n    • Apnea of prematurity\n    • Excessive periodic breathing\n  o Respiratory center dysfunction\n    • Obstructive sleep apnea\n    • Idiopathic ventilation,\n  o \"Ondine's curse\"\n    • Drug-induced or posttraumatic hypoventilation/apnea\n    • Arnold-Chiari-associated apnea\n    • Leigh's syndrome-associated apnea\n• Idiopathic apnea of infancy\n\nDiagnostic workup and treatment depends on possible causes and the age group. In infants, neuroimaging may be warranted if the condition is severe, or if hemorrhage, hydrocephalus, or other central nervous system anatomic abnormalities that could lead to central apnea are suspected. However, central apnea in most premature and full-term infants generally improves over time as the respiratory center of the brain-stem matures. Rigid or flexible endoscopy can be helpful to diagnose anatomic conditions of upper airway obstruction, such as subglottic stenosis or tracheo bronchomalacia. Apnea of prematurity can be treated with theophylline or caffeine. Continuous positive airway pressure (CPAP) or high-flow nasal cannula can be used for mixed or obstructive conditions. For older children, it should be stressed that obstructive sleep apnea is not a benign condition. It can lead to impaired development from sleep disturbances, and if severe, pulmonary hypertension and death. More than 75% of children who have obstructive sleep apnea snore. Referral to pediatric pulmonology or otolaryngology may be warranted. Possible interventions include nighttime CPAP, tonsillectomy, adenoidectomy, and weight loss, where applicable.\n\nRespiratory syncytial virus is a single-stranded RNA virus that commonly causes bronchiolitis in children younger than 1 year of age, usually in yearly epidemics. Bronchiolitis generally causes small airway obstruction from necrosis of the bronchiolar epithelium, edema, and increased mucus production. Apnea is a common manifestation of RSV infection, especially in infants who are younger than 6 weeks of age, formerly premature, or have other pre-existing chronic illnesses. The cause of apnea in RSV is not completely understood, but could be at least partially obstructive in nature because of nasopharyngeal and upper airway obstruction from increased mucous production.\n\nAlthough neuroimaging, electroencephalography, and lumbar puncture can be important in the workup of central apnea in infants to rule out hemorrhage, seizure, or meningitis, these causes are unlikely in the setting of an acute upper respiratory infection. A sleep study would be more useful in older children or in cases of suspected obstructive sleep apnea.\nThe evaluation of apnea depends on the age group and acuity of presentation. Apnea caused by respiratory infections in children generally resolves when the infection improves. Treatment of apnea is generally supportive, but could include medications in apnea of prematurity, acute or chronic positive pressure ventilatory support, or surgical correction of airway lesions.\n\nPREP Pearls\n• Causes of apnea in children can include central, obstructive, or mixed.\n• Obstructive sleep apnea is not a benign condition. It can lead to impaired development, pulmonary hypertension, cor pulmonale, and death.\n• The pathogenesis of apnea from respiratory infections is not well understood, but generally improves along with the infection. Evaluation of nasal secretions for respiratory viruses is the diagnostic study of choice.\n\nAmerican Board of Pediatrics Content Specification(s)\n• Plan the appropriate clinical and diagnostic evaluation of apnea of various etiologies\n• Plan appropriate management for apnea of various etiologies\n\nSuggested Reading\n• Carlo WA. Apnea. In: Kliegman RM, Stanton BF, St. Geme 1W III,Schor NF, Behrman RE, eds. Nelson's Textbook of Pediatrics. 19th ed.Philadelphia, PA: Saunders Elsevier; 2011:580-581.\n• Darville T, Yamauchi T. Respiratory syncyt iaI virus. Perlin& Rev.1998;19(21:55-61. doi:10.1542/pir.19-2-55.\n• Fu LY, Moon RY. Apparent life-threatening events: an update. Pediatr Rev.2012;33(8):361-369. doi:10.1542/pir.33-8-361-369.\n• Matiz A, Roman EA. Apnea. Pediatt Rev. 2003;24(032-34. doi:10.1542/ pir.24-1-32.\n• McBride JT. Infantile apnea. Pediarr Rev. 1984;5(9):275-284. doi:10.1542/"}
{"id" : 238, "question_text" : "A 15-year-old girl is recovering in the pediatric ward of a hospital after an intentional overdose of acetaminophen. She admits she wanted to die because of her shame over her first sexual activity and the conflict it has caused with her parents. Her father states he is very \"disappointed\" in her and plans to have her work with their youth minister, who \"made an example\" of her to the congregation when she attempted to date in the past. She took \"extra\" aspirin a few days before this attempt, although her family is unaware of this. She timed the current suicide attempt for when she thought no one would be able to bring her to the hospital. She denies substance use or other psychiatric disturbance. You have decided that she no longer needs inpatient medical treatment. She tells you she is very embarrassed by the incident and is asking to go home. Of the following, the MOST appropriate course of action is to", "options" : "[\"prescribe a selective serotonin reuptake inhibitor and send the girl home\", \"pursue acute inpatient psychiatric hospitalization\", \"pursue admission to a long-term residential psychiatric facility\", \"send the girl home and arrange for counseling with her youth minister\", \"send the girl home with plans for office follow-up with her primary care physician in 1 week\"]", "explanation" : "Each year, 15% to 25% of high school students seriously consider suicide. Although ideation alone may not require hospitalization, patients who have made serious suicide attempts, such as the girl described in the vignette, often benefit from acute inpatient psychiatric care to ensure safety from additional self-harm while undergoing further evaluation and treatment planning. Known risks for completing suicide can be recalled by the SAD PERSONS mnemonic adapted for children and adolescents by Juhnke: Sex (male higher risk), Age (15 to 19 years higher risk), Depression, Previous suicidal attempts, Excessive alcohol or drug use, Rational thinking loss (like psychosis), Social supports are lacking, Organized plan or serious past suicide attempt, No social support, negligent parenting, School problems (aggression or experiencing humiliation).\n\nOther known risk factors for suicide include: Significant lethality (not expecting to be rescued), Access to lethal means (firearms or drugs), Concurrent psychiatric illness (especially psychotic disorders, substance abuse, mood/anxiety disorders, or disorders involving excessive impulsivity), Persistent wish to die, Having few or inappropriate coping abilities (such as substance abuse, self-injury, or somatization), Interpersonal conflict (especially if associated with fear of abandonment or public humiliation), Family dysfunction.\n\nThis girl's serious premeditation, repeated attempts, past social humiliation, and hopelessness place her at high risk for another suicide attempt.\n\nSending the girl home without further psychiatric evaluation and treatment is not appropriate. In addition, although a selective serotonin reuptake inhibitor may be indicated for her, it is not a substitute for psychiatric hospitalization because it is unlikely to reduce her immediate risk for suicide. Such medications may not be effective for weeks and would not address her family and social risk factors. Because it is important for patients to view their individual therapists as their own advocates rather than agents of their parents, counseling by the youth minister from her parents' church is potentially harmful. Residential long-term psychiatric facilities are not used for acute management of suicidality; they are reserved for patients who have not responded to shorter term psychiatric hospital care.\n\nAAP Mental Health Competency: Know when to refer a child with suicidality for psychiatric hospitalization"}
{"id" : 1559, "question_text" : "A 10-year-old girl with type 1 diabetes diagnosed at age 7 years is brought to your office for frequent episodes of hypoglycemia over the past 2 weeks. She has required oral treatment of low blood glucose levels on 8 separate occasions during this time frame. She is on a flexible basal-bolus insulin regimen with insulin glargine and aspart. Her total daily insulin dose typically averages 0.7 units/kg per day but has averaged 0.5 units/kg per day over the past 2 weeks. There have been no recent changes in her insulin regimen, and her hemoglobin A1c 2 months ago was 7.6%. The girl has had no fever or recent illness, but has been complaining of intermittent \"stomach aches.\" She participates in dance for 1 hour, 1 evening per week. Her physical examination reveals a temperature of 37°C, blood pressure of 102/64 mm Hg, heart rate of 72 beats/min, weight of 32 kg (40th percentile), height of 143 cm (65th percentile), and body mass index of 15.6 kg/m2 (25th percentile). Her weight is unchanged from that documented 8 months ago. The remainder of her physical examination findings are within normal parameters. Of the following, the MOST likely cause of the girl's hypoglycemia is", "options" : "[\"celiac disease\", \"hypothyroidism\", \"increased physical activity\", \"insulin dosing errors\", \"puberty\"]", "explanation" : "The girl described in the vignette most likely has celiac disease, presenting with recurrent hypoglycemia in the context of type 1 diabetes. Celiac disease is an autoimmune disease with an increased incidence in those with type 1 diabetes, occurring in about 5%. Her recurrent hypoglycemia, \"stomach aches,\" and lack of weight gain over the past 8 months are all consistent with celiac disease. Celiac disease is the second most common autoimmune disease associated with type 1 diabetes. The American Diabetes Association recommends screening for celiac disease at the time of diagnosis of type 1 diabetes, and rescreening as indicated for symptoms.\n\nHypothyroidism does not generally cause hypoglycemia in those with type 1 diabetes. Increased physical activity or insulin dosing errors could cause hypoglycemia, but there is no indication in the vignette that this girl's physical activity level has changed or that insulin dosing errors are being made. Her hemoglobin A1c level measured 2 months ago suggests relatively good glycemic control at that time. Because puberty produces a natural state of insulin resistance, it is more often associated with high blood glucose levels, not hypoglycemia.\n\nAutoimmune thyroid disease, especially Hashimoto thyroiditis with associated hypothyroidism, is the most common associated autoimmune disease seen in children with type 1 diabetes. Approximately one-third of these children have detectable thyroid antibodies and 10% have abnormal thyroid function. The American Diabetes Association recommends screening for thyroid disease with thyroid antibody (thyroid peroxidase, antithyroglobulin antibodies) and thyroid-stimulating hormone (TSH) levels at the time of diagnosis of type 1 diabetes, and rescreening TSH levels every 1 to 2 years.\n\nAddison disease, due to autoimmune adrenal insufficiency, although rare, is the third most common associated autoimmune condition, occurring in less than 1% of pediatric patients with type 1 diabetes. There is no routine recommendation for screening for Addison disease in these children.\n\nHypoglycemia, in the context of type 1 diabetes, is defined as a blood glucose of less than 70 mg/dL (<3.9 mmol/L). Treatment uses the \"rule of 15s\": 15 g of fast-acting carbohydrate should be ingested and 15 minutes later the blood glucose level should be tested. Fifteen-gram \"doses\" of fast-acting carbohydrate include 1/2 cup of juice or regular soda, 1 cup of milk, 3 teaspoons of honey, or 3 to 4 glucose tablets. This treatment and glucose check should be repeated if the blood sugar remains lower than 70 mg/dL. If the next meal will not be eaten within the next 30 to 60 minutes, a small snack of complex carbohydrate, fat, and protein should be eaten to help sustain the blood glucose level. For more severe hypoglycemia, with an inability to take oral glucose, glucagon can be given intramuscularly or subcutaneously. Every patient with diabetes who takes insulin should have a glucagon emergency kit.\n\nPREP Pearls\n• Celiac disease may present with unexplained episodes of recurrent hypoglycemia in children with type 1 diabetes\n• Autoimmune thyroid disease and celiac disease are the first and second most common autoimmune diseases, respectively, associated with type 1 diabetes.\n• Hypoglycemia associated with type 1 diabetes is managed using the \"rule of 15s\": ingestion of 15 g of fast-acting carbohydrate followed by recheck of the blood glucose level after 15 minutes. This cycle is repeated until the blood glucose level is greater than 70 mg/dL.\n\nABP Content Specifications(s)\n• Recognize the association between type 1 diabetes and other autoimmune disorders\n• Plan the appropriate management of hypoglycemia in a patient with type 1 diabetes and other autoimmune disorders (eg, celiac disease, Hashimoto thyroiditis)\n\nSuggested Readings\n• American Diabetes Association. 12. Children and adolescents. Diabetes Care. 2017;40(suppl 1):S105–S113. doi: http://dx.doi.org/10.2337/dc17-S015.\n• Chase HP, Banion C. Chapter 6: Low blood sugar (hypoglycemia or insulin reaction). In: Chase HP, Maahs D, eds. Understanding Diabetes. 11th ed. Denver, CO: Paros Press; 2006:35–48. Available at: http://www.ucdenver.edu/academics/colleges/medicalschool/centers/BarbaraDavis/OnlineBooks/Pages/UnderstandingDiabetes.aspx.\n• Gregory JM, Moore DJ, Simmons JH. Type 1 diabetes mellitus. Pediatr Rev. 2013;34(5):203–215. doi: http://dx.doi.org/10.1542/pir.34-5-203.\n• Ly TT, Maahs DM, Rewers A, et al; International Society for Pediatric and Adolescent Diabetes. ISPAD Clinical Practice Consensus Guidelines 2014. Assessment and management of hypoglycemia in children and adolescents with diabetes. Pediatr Diabetes. 2014;15(suppl 20):180–192. doi: http://dx.doi.org/10.1111/pedi.12174."}
{"id" : 2691, "question_text" : "A 3-year-old girl is brought to the office for evaluation of burning with urination for 2 days. She has no urinary urgency, frequency, nighttime or daytime accidents, back pain, fever, rash, or joint pains. She has a soft bowel movement every day. She has been toilet-trained since 2 years of age. She has bubble baths 3 times a week and showers on most other days. The girl's weight is at the 50th percentile, height is at the 75th percentile, and her blood pressure is 90/60 mm Hg. Her physical examination findings, including the genitourinary examination findings, are normal. Urinalysis with microscopy results are shown: Test Result, Appearance Yellow, Specific gravity 1.015, Leukocyte esterase Negative, Nitrite Negative, Blood Positive, Protein Negative, Red blood cells 10-20/HPF, White blood cells <5/HPF. Of the following, the MOST likely diagnosis for this girl is", "options" : "[\"chemical urethritis\", \"dysfunctional voiding\", \"urethral prolapse\", \"urinary tract infection\"]", "explanation" : "The girl in the vignette most likely has chemical urethritis caused by frequent bubble baths. This diagnosis is supported by her symptom of dysuria and the urinalysis results showing microscopic hematuria without pyuria. Dysuria is defined as pain, discomfort, or burning during urination. Dysuria is a common symptom seen with urinary tract infection, inflammation, irritation, trauma, obstruction, and several systemic illnesses. The causes of dysuria in different age groups are varied.\n\nA detailed history can help focus the differential diagnosis for a child with dysuria.\n• The presence of other urinary symptoms (eg, hematuria, frequency, urgency, foul-smelling urine, and nocturnal and/or daytime enuresis) may support an infectious cause.\n• Recent use of new detergents, soaps, ointments, or frequent bubble baths may point toward chemical urethritis.\n• A history of trauma to the genital area (eg, mechanical irritation from underwear fabric, masturbation, or sexual activity), with consideration of the age group, can help differentiate the cause.\n• A history of withholding of urine, enuresis, and/or constipation may suggest dysfunctional voiding.\n• A family history of nephrolithiasis may suggest hypercalciuria.\n• A history of fever may suggest acute pyelonephritis, pelvic inflammatory disease, or systemic disease.\n• A history of conjunctival inflammation, rash, joint pain, or oral ulcer may suggest a diagnosis of reactive arthritis, Stevens-Johnson syndrome, or Behçet disease.\n\nThe physical examination of a child with dysuria should include assessment of the lower back for markers of spinal dysraphism, assessment of lower limb strength and reflexes, abdominal examination to assess for flank or suprapubic mass, and genitourinary examination.\n\nPREP Pearls\n• Chemical urethritis is a common cause of dysuria in young children and is often associated with frequent bubble baths or use of irritating soaps.\n• Dysuria without pyuria or bacteriuria suggests a non-infectious cause such as chemical urethritis.\n• A detailed history focusing on recent exposures and associated symptoms is essential in determining the etiology of dysuria.\n\nABP Content Specifications(s)\n• Recognize the clinical features associated with dysuria in children\n\nSuggested Readings\n• Goilav B, Kaskel FJ. Dysuria. In: McInerny TK, Adam HM, Campbell DE, DeWitt TG, Foy JM, Kamat DM, eds. American Academy of Pediatrics Textbook of Pediatric Care. American Academy of Pediatrics; 2021:chap 144. Accessed September 1, 2022. Pediatric Care Online."}
{"id" : 254, "question_text" : "You are seeing a 13-year-old girl, in whom you diagnosed anorexia nervosa approximately 18 months ago, for a follow-up visit. She had started to restrict her food intake about 6 months before her first visit. There was no history of binging or purging. She had become progressively more isolated from her friends and was very anxious and irritable. Currently, her mother states that she is doing well at school and has one friend. She is eating everything but still in small quantities. She has not had menarche yet but is otherwise asymptomatic. Her mother had her menarche at age 12½ years. On physical examination, the girl has normal vital signs, a body mass index of 17.4, and no focal findings. When her father comes in after your examination, he is very upset that she is not \"cured after all this time\" and that they still have to supervise her meals and eating habits. You discuss the usual course of this illness and prognosis with him. Of the following, the factor MOST likely to be associated with a poor prognosis for this girl is", "options" : "[\"absence of binging and purging\", \"comorbid psychiatric illness\", \"early onset of illness (<14 years)\", \"good family support\", \"short duration of illness\"]", "explanation" : "Although one of the diagnostic criteria for anorexia nervosa (AN) is amenorrhea, the onset of AN may predate the onset of puberty or may occur in the early pubertal stages, interrupting further development and resulting in primary rather than secondary amenorrhea, which is the case for the girl described in the vignette. Prognostic factors for eating disorders have not been reliably identified. Overall, it is estimated that about 50% of patients do well, about 30% do not do so well, and about 20% do poorly. The mortality rate of 5% to 10% is the highest among psychiatric disorders, with death resulting from either suicide or medical complications.\n\nMost longitudinal studies indicate that the onset of AN before adulthood, especially before age 14 years, along with early, intensive treatment is associated with a good prognosis. Other factors that are good prognosticators are good family support and a shorter duration of illness, suggesting that the behaviors have not become entrenched and the parents, with guidance, should be able to aid in recovery. Thus, early recognition and intensive treatment improve the prognosis. Factors associated with a less positive prognosis include the presence of binging and purging, longer duration of illness before treatment, poor family relations, and comorbid psychiatric illnesses.\n\nCritique: Preferred Response: B\n\nContent Specifications: Know the factors affecting the prognosis for adolescents with anorexia nervosa"}
{"id" : 3671, "question_text" : "A 10-month-old, full-term, male infant is seen by his pediatrician for evaluation of gross motor delay. His mother notes that he is unable to sit independently. She recalls that he was able to roll at 4 months of age and briefly sat in a tripod position around the age of 8 months. Over the last 4 weeks he is no longer able to roll or sit without support. He reaches for objects, using a raking grasp with no hand preference. He babbles, says \"mama\" and \"dada,\" and cries if approached by an unfamiliar person. He has had no preceding illness. He is alert and in no acute distress. His pupils are equal, round and reactive to light, extraocular movements are intact, and his face is symmetric. Tongue fasciculations are noted. Normal muscle bulk with diffuse axial and appendicular hypotonia is appreciated. Proximal strength is 3/5 bilaterally with more movement distally and in the arms. Deep tendon reflexes are 0/4 throughout, with toes downgoing to plantar stimulation. Fine tremors are noted in the hands. No dysmetria or ataxia are appreciated in his movements. Of the following, the MOST likely diagnosis is", "options" : "[\"congenital muscular dystrophy\", \"infantile botulism\", \"myasthenia gravis\", \"spinal muscular atrophy\"]", "explanation" : "Correct Answer: D\nThe 10-month-old infant in this vignette has signs and symptoms suggestive of spinal muscular atrophy (SMA). Spinal muscular atrophy is a neurodegenerative genetic condition of the peripheral nervous system affecting the anterior horn cells of the spinal cord and presenting with regression of motor milestones, tongue fasciculations, areflexia, diffuse hypotonia, and weakness of the extremities.\n\nInfants and children with SMA have varied clinical presentations along a continuum correlating with the genetic heterogeneity of the condition. The core clinical features of SMA are muscle weakness and hypotonia with diminished reflexes, reflecting anterior horn cell dysfunction and loss. In all but the most severe forms, there is a presymptomatic period in which the child's physical examination findings and motor development are normal, followed by regression of motor milestones and progressive weakness. Weakness is typically more proximal and affects the legs more than the arms.\n\nThree major clinical phenotypes of SMA have been described. They are distinguished by age of onset and the most advanced motor milestone obtained.\n• Type 1 (infantile-onset) SMA is the most common and has clinical presentation before 6 months of age with a severe course. These infants never attain the ability to sit and have profound hypotonia and weakness with progression to respiratory failure. Without respiratory support, patients die before the age of 2 years.\n• Type 2 SMA presents between 6 and 18 months of age. Infants attain the ability to sit independently but never walk. Clinically, affected infants have a more intermediate and varied course. In addition to the core features of the disease, a fine tremor (minipolymyoclonus) is evident in the distal limbs. With aggressive supportive care, children with SMA type 2 can survive into their 20s.\n• Type 3 SMA presents in children and teenagers who have already attained the ability to walk independently. Type 3 SMA presents with frequent falls and difficulty climbing stairs due to proximal muscle weakness. The disease course is more mild, and many affected individuals have a normal life span.\n\nSpinal muscular atrophy is an autosomal recessive disorder affecting 5q13. There are 2 survival motor neuron genes, SMN1 and SMN2, which differ by 5 base pairs resulting in a difference of a single nucleotide. SMN1 produces a full-length SMN protein whereas SMN2 produces a shortened, rapidly degraded protein and only a small percentage of full-length protein. In SMA there is a deletion in SMN1 that leads to disrupted RNA metabolism affecting motor neurons preferentially due to higher expressions in those cells. Thus, affected patients rely on the reduced protein production of SMN2. The level of protein production can vary with copy number and loosely correlates with clinical presentation and disease severity. Diagnosis is made through genetic testing showing deletion in SMN1.\n\nThe differential diagnosis of the hypotonic infant is broad and includes conditions affecting all parts of the neuroaxis. Congenital muscular dystrophy is a heterogenous group of genetic conditions presenting in the neonatal period with hypotonia, weakness, and sometimes arthrogryposis. Some subtypes can have accompanying brain malformations. Infantile botulism is an acquired disorder typically presenting with a descending paralysis and hypotonia preceded by a history of constipation and classically associated with raw honey consumption. Myasthenia gravis can present in the neonatal period as either an inherited congenital myasthenic syndrome or acquired transient autoimmune form secondary to transfer of maternal autoantibodies to the fetus.\n\nIn December 2016, the first treatment for SMA was approved for clinical use by the US Food and Drug Administration (FDA). Nusinersen, an antisense oligonucleotide drug, modifies the pre-messenger RNA splicing of SMN2, promoting increased production of full-length SMN protein. Infants in the trial who received nusinersen were more likely to be alive and have improvements on objective scales of motor function compared to infants in the control group. Infants identified earlier in the course of disease received maximum benefit of the drug, attaining motor milestones that would not have been expected in the natural history of the disease. Nusinersen is administered via intrathecal injection. The initial treatment phase consists of 4 loading doses given at intervals of 2 weeks, followed by maintenance doses given every 4 months. Nusinersen is now recommended as a standard of care for infants and children with SMA. Newborn screening for SMA has been implemented in several states and is being considered in others. Early identification of affected infants before symptom onset allows for treatment initiation before irreversible motor neuron loss occurs, potentially improving long-term outcomes.\n\nPREP Pearls\n• Spinal muscular atrophy is characterized by regression of motor milestones, tongue fasciculations, areflexia, diffuse hypotonia, and weakness of the extremities.\n• Weakness in spinal muscular atrophy is typically more proximal and affects the legs more than the arms.\n• Nusinersen, an antisense oligonucleotide drug, is the first FDA-approved treatment for spinal muscular atrophy and is now recommended as a standard of care for infants and children with SMA.\n\nABP Content Specifications(s)\n• Recognize the clinical features associated with spinal muscular atrophy\n• Understand the genetic basis of spinal muscular atrophy\n• Understand the role of nusinersen in the treatment of spinal muscular atrophy\n\nSuggested Readings\n• Kolb SJ, Kissel JT. Spinal muscular atrophy. Neurol Clin. 2015;33(4):831-846. doi:10.1016/j.ncl.2015.07.004.\n• Mercuri E, Bertini E. Spinal muscular atrophy: an update. Curr Opin Neurol. 2012;25(5):631-637. doi:10.1097/WCO.0b013e328357fa13.\n• Scoto M, Finkel RS, Mercuri E, Muntoni F. Spinal muscular atrophy. Nat Rev Dis Primers. 2017;3:17002. doi:10.1038/nrdp.2017.2.\n• Wirth B. Spinal muscular atrophy: in vivo gene therapy is within reach. Nat Med. 2015;21(4):345-346. doi:10.1038/nm.3840."}
{"id" : 3021, "question_text" : "A 6-year-old boy who is receiving maintenance chemotherapy for acute lymphocytic leukemia is brought to the emergency department after developing a burning rash over his face 2 days ago. His medications include monthly prednisone and vincristine pulse, daily oral 6-mercaptopurine, and weekly oral methotrexate. His vital signs are normal for his age. He has erythematous vesicular lesions on his upper lip, his right eyelid, and the right side of his forehead. The rest of the physical examination findings are unremarkable. He is diagnosed with herpes zoster infection and admitted for administration of intravenous acyclovir. His blood urea nitrogen level is 14 mg/dL (5.0 mmol/L), and his creatinine level is 0.5 mg/dL (44 µmol/L). Of the following, the MOST appropriate management step to prevent drug-induced nephrotoxicity in this child is", "options" : "[\"alkalinization of urine\", \"extended-interval drug dosing\", \"isotonic fluid hydration\", \"serum drug level monitoring\"]", "explanation" : "The boy in this vignette, who is receiving maintenance therapy for acute lymphocytic leukemia with herpes zoster infection, is at risk of experiencing acyclovir-induced nephrotoxicity due to precipitation of crystals in the renal tubules. This is best prevented by intravenous isotonic hydration.\n\nDrugs are a common cause of acute kidney injury. The common pathologic mechanisms of drug-induced nephrotoxicity are alteration of intraglomerular hemodynamics, renal tubular cell toxicity, acute and chronic interstitial nephritis, crystal nephropathy, and thrombotic microangiopathy. Risk factors that predispose a patient to nephrotoxicity include decreased effective intravascular volume, use of multiple nephrotoxic medications, and underlying renal insufficiency.\n\nGeneral measures to prevent drug-induced nephrotoxicity include using alternate non-nephrotoxic drugs, correcting the risk factors, assessing baseline renal function, adjusting the dose of the drug according to renal function, and avoiding nephrotoxic drug combinations. Adequate hydration is important to maintain renal perfusion, and intravascular volume should be corrected before nephrotoxic medication therapy begins. Item C224 presents specific measures to prevent the nephrotoxicity caused by different classes of drugs.\n\nThe boy in this vignette is receiving multiple chemotherapy medications, including methotrexate, which can cause nephrotoxicity when used in a high dose through the intravenous route. The child in this vignette is receiving oral methotrexate once weekly and has normal baseline renal function, so the drug is less likely to cause nephrotoxicity. Nephrotoxicity associated with high-dose methotrexate is prevented via isotonic fluid hydration, alkalinization of urine, and measurement of drug levels. Alkalinization of urine is useful in preventing contrast-induced nephropathy. Extended-interval dosing can be used to prevent nephrotoxicity related to use of aminoglycoside and vancomycin. Monitoring serum level of the drug is useful in preventing nephrotoxicity due to tacrolimus, cyclosporine, aminoglycoside, and vancomycin.\n\nPREP Pearls\n• Risk factors for drug-induced nephrotoxicity are decreased effective intravascular volume, use of multiple nephrotoxic medications, and underlying renal insufficiency.\n• The clinician should assess baseline renal function and adjust medication doses according to renal function to prevent nephrotoxicity.\n• Adequate hydration is important before initiating therapy with nephrotoxic drugs.\n• Intravenous acyclovir in high doses can induce nephrotoxicity by precipitating crystals in renal tubules. Adequate hydration is important to prevent the development of nephrotoxicity.\n\nABP Content Specifications(s)\n• Recognize the drug classes that can cause renal toxicity\n\nSuggested Readings\n• Faught LN, Greff MJ, Rieder MJ, Koren G. Drug-induced acute kidney injury in children. Br J Clin Pharmacol. 2015;80(4):901-909. doi:10.1111/bcp.12554.\n• Naughton CA. Drug-induced nephrotoxicity. Am Fam Physician. 2008;78(6):743-750. www.aafp.org/afp/2008/0915/p743.html."}
{"id" : 3535, "question_text" : "According to the 2020 Surviving Sepsis Campaign guidelines, when should empiric multi-drug antimicrobial therapy be considered in children with sepsis or septic shock?", "options" : "[\"In children with immune compromise or high risk of antimicrobial resistance (\\u226510%)\", \"Routinely in all children to ensure synergy against pathogens\", \"Only in children with documented fungal infections\", \"In all children presenting with fever of unknown origin\"]", "explanation" : "The guidelines recommend expanding spectrum of coverage with multi-drug therapy specifically for children with immune compromise or high risk of antimicrobial resistance (≥10%), while avoiding routine use for synergy in immunocompetent children."}
{"id" : 2448, "question_text" : "A 17-year-old adolescent with a history of systemic lupus erythematosus is seen for evaluation of 3 days of dysuria. She asks to be screened for sexually transmitted infections. She has had 3 lifetime sexual partners with 1 new partner in the past 3 months. She engages in vaginal and oral sex and has not used condoms consistently. She was diagnosed with gonorrhea 4 months ago; her vaginal symptoms completely resolved 2 weeks after treatment. Her partners were notified and also treated. She reports no current vaginal discharge, abdominal pain, or rash. The result of an HIV screening test was negative 4 months ago. Her only current medication is hydroxychloroquine. The adolescent's physical examination findings are normal, including vital signs and pelvic examination findings. Samples are obtained for laboratory evaluation: Vaginal and oral swabs for chlamydia, gonorrhea, and trichomonas nucleic acid amplification testing; Serum for HIV antigen/antibody concentrations and syphilis serology; Clean-catch urine for urinalysis, urine culture, and pregnancy test. Her pregnancy test result is negative. Of the following, the BEST next step in this adolescent's care is to", "options" : "[\"administer a dose of ceftriaxone intramuscularly and azithromycin orally\", \"offer treatment with HIV pre-exposure prophylaxis medication\", \"perform urine nucleic acid amplification testing for chlamydia, gonorrhea, and trichomonas\", \"prescribe ciprofloxacin for urinary tract infection\"]", "explanation" : "PREP Pearl(s)\nProviders should confidentially speak with all adolescents and young adults about sexual behaviors to provide optimal sexually transmitted infection prevention education and perform screening on samples from all involved sites.\nThe Centers for Disease Control and Prevention recommends pre-exposure HIV prophylaxis for any adolescent who asks for it.\nThe Centers for Disease Control and Prevention offers guidance for counseling on, prescribing, and managing antiretroviral medications for pre-exposure HIV prophylaxis.\nCritique\nThe adolescent in the vignette is at high risk of experiencing sexually transmitted infections (STIs). Of the response choices, the best next step in her treatment is to discuss and offer HIV pre-exposure prophylaxis (PrEP). Treatment may begin as soon as the current laboratory results, in addition to baseline tests obtained specifically for PrEP, are available.\nDysuria in the context of a recent STI and unprotected sexual intercourse increases the likelihood of an STI as the cause of this adolescent's current symptoms. However, she does not require urine nucleic acid amplification testing for chlamydia, gonorrhea, and trichomonas because a vaginal swab has already been collected for this testing. In addition, urine testing for STIs must be on a \"dirty\" urine specimen (eg, a specimen obtained with no cleansing wipe used beforehand). The urine specimen provided by this adolescent was obtained via clean catch.\nIt may be appropriate to empirically treat the adolescent in the vignette for chlamydia and gonorrhea. The 2021 Centers for Disease Control and Prevention (CDC) STI treatment guidelines recommend treatment for Chlamydia trachomatis infection with doxycycline 100 mg twice per day for 7 days. Azithromycin 1 g orally in a single dose is an alternative regimen but is not recommended as first-line therapy. Ceftriaxone 500 mg intramuscularly is a recommended treatment for gonococcal infections.\nA clean-catch urine sample was obtained for urinalysis and urine culture from the adolescent in the vignette to evaluate for a bacterial urinary tract infection (UTI). If the urinalysis is concerning for a UTI (eg, presence of leukocyte esterase, nitrites, white blood cells, or a combination of these), antibiotics should be initiated. However, ciprofloxacin is not recommended as a first-line treatment for an uncomplicated UTI. In addition, fluoroquinolones are contraindicated for this adolescent because of their interaction with hydroxychloroquine.\nAdolescents and young adults carry the highest risk of STI acquisition. All 50 states allow minors to give consent for sexual and reproductive health care (specific rules vary by state). It is imperative for pediatric providers to spend time privately and discuss confidentiality with each adolescent at every office visit. Adolescents should be asked about sexual behaviors and provided appropriate education about pregnancy and STI prevention. Providers should ask about sexual contact or interest in sexual intercourse clearly and directly (eg, \"When was the last time you had sex?\"). Vague questions (eg, \"Are you sexually active?\") should be avoided. Inquiry about all types of sexual contact (eg, vaginal, anal, oral, hand-genital) is essential to obtain STI testing from all involved sites and to provide appropriate STI prevention education. Sexually transmitted infections can occur with any type of sexual practice. Testing for HIV is recommended for all adolescents aged 15 years or older.\nPre-exposure prophylaxis with antiretroviral medication is indicated for people without HIV infection who are at risk of exposure through sex or injected drug use. The CDC PrEP guidelines include extensive algorithms and flowcharts to assist providers in counseling on, prescribing, and monitoring PrEP medications. The 2021 guidelines recommend that PrEP be discussed with all sexually active adults and adolescents and prescribed for any adolescent who asks for it.\nThree medications are approved by the US Food and Drug Administration for PrEP for individuals weighing at least 35 kg. Dosing and side effects of these medications are outlined in the Table 1.\nThe CDC recommends that renal function, hepatitis B serology, and 4th-generation HIV antigen/antibody testing results be assessed before initiation of oral PrEP (Table 2). It is appropriate to prescribe PrEP on the day of the office visit while awaiting these laboratory results. All patients should have a confirmed negative HIV test finding within 7 days of being prescribed PrEP. The antiretrovirals used for PrEP offer only partial treatment for HIV infection, making viral resistance possible if the patient has acquired HIV infection before starting PrEP. If there is concern about acute HIV infection, a viral load (HIV-1 quantitative RNA) should be obtained. Daily adherence to PrEP medications is critical for maximum prevention benefit, and providers should counsel and monitor adolescents accordingly. However, studies have shown that HIV risk reduction efficacy remains over 90% with up to 3 missed doses per week.\nSuggested Reading(s)\nAmerican Academy of Pediatrics. STIs during preventive health care of adolescents. In: Kimberlin DW, Barnett ED, Lyfield R, Sawyer MH, eds. Red Book: 2021-2024 Report of the Committee on Infectious Diseases. 32nd ed. American Academy of Pediatrics; 2021. Accessed September 1, 2023. Red Book Online\nBrundrett ME. Human immunodeficiency virus preexposure prophylaxis in adolescents and young adults. Pediatr Rev. 2022;43(1):28-36. doi:10.1542/pir.2020-002048\nEmmanuel PJ, Mansfield J, Siberry GK. Human immunodeficiency virus infection: an update for pediatricians. Pediatr Rev. 2022;43(6):335-346. doi:10.1542/pir.2020-001644\nPre-exposure prophylaxis. Centers for Disease Control and Prevention. Accessed September 1, 2023. https://www.cdc.gov/hiv/risk/prep/index.html\nPreexposure prophylaxis for the prevention of HIV infection in the United States—2021 update: a clinical practice guideline. US Public Health Service, Centers for Disease Control and Prevention. Accessed September 1, 2023. https://www.cdc.gov/hiv/pdf/risk/prep/cdc-hiv-prep-guidelines-2021.pdf\nContent Domain\nPreventative Pediatrics\nABP Content Specification(s) / Content Area(s)\nProvide appropriate counseling with regard to contraception and prevention of sexually transmitted infection for an adolescent engaging in vaginal and/or anal intercourse"}
{"id" : 1828, "question_text" : "A previously healthy 10-year-old boy is brought to the emergency department with a 2-week history of worsening exercise intolerance, orthopnea requiring 2 pillows to sleep at night, and a 2-day history of facial swelling. He is thin and in mild respiratory distress with suprasternal retractions. His face is slightly swollen with a subtle red coloration. He has a temperature of 38.8°C, heart rate of 110 beats/min, blood pressure of 104/76 mm Hg, respiratory rate of 28 breaths/min, and oxygen saturation of 97% on room air. A chest radiograph is obtained (Item Q28). Item Q28: Chest radiograph for the boy described in the vignette. Courtesy of J. Fish Of the following, the MOST appropriate next step in managing this patient is to", "options" : "[\"consult a surgeon for an emergency biopsy\", \"obtain urgent chest computed tomography with the patient lying supine\", \"secure the airway via elective intubation\", \"support the airway via noninvasive positive pressure\"]", "explanation" : "The patient in this vignette has symptoms of superior vena cava syndrome, which occurs when there is external compression of the superior vena cava, resulting in reduced blood return from the upper body and head to the heart. This leads to venous congestion in the face and arms as well as increased intracranial pressure. Over 90% of cases of superior vena cava syndrome are caused by cancer, and the chest radiograph for this patient shows a mediastinal mass. In children and adolescents, the most common cause of superior vena cava syndrome is a mediastinal mass.\n\nGiven the location of the mass on the radiograph, there should be concern not just for compression of the superior vena cava, but also of the right atrium. Should the mass completely compress the right atrium, there would be no blood return to the heart, and cardiac arrest would ensue. The factors helping to maintain the patency of the right atrium are gravity and negative intrathoracic pressure. Thus, lying the patient down on his back would worsen the compression of the right atrium, as would sedating the patient. The best management option for the patient's respiratory distress is to maintain him awake, sitting up, and with positive pressure.\n\nThe patient is a very poor candidate for general anesthesia as this would result in the loss of the negative intrathoracic pressure, increasing the risk of right atrial collapse. Consulting a surgeon for a biopsy would thus not be the next best step in management. Obtaining chest computed tomography would necessitate lying the patient supine on his back, thereby increasing the risk for right atrial collapse from the mass. As the compression in the chest is occurring distal to the larynx, the endotracheal tube would not bypass the obstruction and the sedation could potentially lead to cardiac arrest.\n\nPREP Pearls\n\nSuperior vena cava syndrome is caused by compression of the superior vena cava, most often by cancer.\n\nSigns and symptoms of superior vena cava syndrome often include edema of the face and arms, headache, and cough.\n\nFor patients with mediastinal masses, there should be concern for maintaining the patency of the right atrium. Factors contributing to the risk for collapse of the right atrium include gravity and negative intrathoracic pressure; thus, these patients should not be lying flat supine, and sedation should be avoided.\n\nABP Content Specifications(s)/Content Area\n\nRecognize the need for immediate evaluation of a child with a chest mass who is at risk of acute respiratory failure\n\nRecognize the clinical findings associated with a chest mass\n\nSuggested Readings\n\nBuhtoiarov IN. Pediatric lymphoma. Pediatr Rev. 2017;38(9):410-423. doi: http://dx.doi.org/10.1542/pir.2016-0152.\n\nPearson JK, Tan GM. Pediatric anterior mediastinal mass: a review article. Semin Cardiothorac Vasc Anesth. 2015;19(3):248-54. doi: http://dx.doi.org/10.1177/1089253215578931.\n\nPrusakowski MK, Cannone D. Pediatric oncologic emergencies. Emerg Med Clin North Am. 2014;32(3):527-548. doi: http://dx.doi.org/10.1016/j.emc.2014.04.005."}
{"id" : 2191, "question_text" : "A 16-year-old adolescent boy is evaluated for abdominal pain for the past 3 months. He has had no fever, vomiting, or diarrhea. His last stool was 7 days ago. Review of systems is positive for weight-loss of 5 kg in the previous 2 months, dry skin, dizziness on standing, and cold intolerance. There are no previous growth records to review. There is no family history of gastrointestinal or thyroid illnesses. He plays soccer and recently began training at a boxing gym 3 times a week. He reports no drug use, depressed mood, suicidal thoughts, or desire for weight loss. He mentions that he is trying to increase his water intake and is avoiding meat in order to be healthier. Physical examination reveals a temperature of 36.1 °C, heart rate of 51 beats/min, respiratory rate of 12 breaths/min, and blood pressure of 100/78 mm Hg. His weight is 63 kg (55th percentile), height is 137 cm (10th percentile), and body mass index is 84th percentile. He appears tired, his extremities are cold to the touch, capillary refill time is less than 3 seconds, and no rashes are noted. His lungs are clear with good aeration. There is no cardiac murmur or rub. There is mild diffuse abdominal tenderness to palpation and hypoactive bowel sounds, with no hepatosplenomegaly. His neurological examination is normal. An electrocardiogram is performed. Nutrition counseling is provided, and a referral is placed to a dietician. Results of laboratory testing are shown: Laboratory Test Result Sodium 133 mEq/L (133 mmol/L) Potassium 3.5 mEq/L (3.5 mmol/L) Chloride 101 mEq/L (101 mmo/L) Bicarbonate 24 mEq/L (24 mmol/L) Urea nitrogen 15 mg/dL (5.35 mmol/L) Creatinine 1.1 mg/dL (97.2 µmol/L) Albumin 4.5 g/dL (45 g/L) Hemoglobin 14.2 g/dL (142 g/L) Thyroid stimulating hormone 0.4 µIU/mL (reference range, 0.5 - 4.0 µIU/mL) Free thyroxine 0.7 ng/dL [9.01 pmol/L, (reference range, 0.8 - 2.0 ng/dL)] Of the following, the BEST next step in management is to recommend", "options" : "[\"high protein supplement drinks\", \"restriction of physical activity\", \"a stimulant laxative trial to treat constipation\", \"testing for thyroid autoantibodies\"]", "explanation" : "Critique\nThe adolescent in the vignette has signs and symptoms related to protein-calorie malnutrition, including cold intolerance, bradycardia, and constipation likely due to disordered eating. Although his body mass index is in normal range, the boy meets criteria (Golden, 2015) for malnutrition due to his severe weight loss. A normal or elevated BMI should not falsely reassure a practitioner of a child's or adolescent's nutritional status and health.\nIn addition to providing nutrition counseling and a referral to a dietician, the practitioner should advise the adolescent to limit physical activity until his test results have returned, and reassessment at a follow-up visit has occurred. The practitioner should ensure that this boy's symptoms, examination findings, weight trend, and laboratory findings are improving before recommending slow resumption of physical activity, beginning at low intensity levels.\nThe adolescent's laboratory results indicate mild hyponatremia, likely due to excessive water consumption in the setting of malnutrition. His creatinine is mildly elevated; he should not be advised to supplement with high protein drinks as these can place undue stress on the renal system if the protein load is higher than the body can absorb (typically 15-30 g). In addition, most \"high protein\" nutritional supplement drinks have low fat and/or carbohydrate content, which would not be an appropriate recommendation for an adolescent with malnutrition. Sick euthyroid syndrome is a common finding in malnutrition, with mildly low thyroid stimulating hormone and thyroxine levels. This is not an indication for thyroid autoantibody testing or thyroid hormone supplementation. Constipation is a very common finding in individuals with malnutrition. In this situation, the initial treatment of constipation is enhanced nutrition and dietary changes; an osmotic laxative is the next step in treatment. Stimulant laxatives should be used sparingly and as a last option for individuals with constipation related to disordered eating.\nEating disorders include various maladaptive behavioral patterns around food and/or the body that can lead to malnutrition and medical instability; they include anorexia nervosa, bulimia nervosa, avoidant/restrictive food intake disorder (ARFID), binge-eating disorder, and other eating disorders not specified (ie, those in which the frequency of behaviors do not meet full criteria for the other diagnoses). Although the adolescent in the vignette reports no desire for weight loss, his restrictive dietary changes and excessive exercise place him at high risk for an eating disorder.\nEating disorders affect individuals of every race, sex, gender, age, body size, sexuality, and socioeconomic status. Physicians should maintain a high index of suspicion for an eating disorder when a child or adolescent presents with weight change, pubertal concerns, growth velocity decrease, menstrual irregularity, or other associated medical symptoms (as in the vignette). For all individuals with malnutrition, a comprehensive history of eating-related behaviors should be obtained that includes questions regarding binge eating, purging (eg, by vomiting, compensatory exercise), laxative misuse, exercise routines, and fasting for weight loss. There are validated screening tools for eating disorders in adolescents, such as the SCOFF questionnaire. Limitations of this tool include that it does not identify some common disorders, including binge eating disorder. Other screening tools are available but should be used only for the age group and population for which each has been validated.\nYoung males make up one in three patients with eating disorders. Detection of eating disorders in males can be especially challenging, as they often go undiagnosed due to the common misconception that eating disorders only occur in females. In addition, males are more likely to desire leanness in an attempt to increase strength and attain a more muscular body image. Because boys and adolescent males with malnutrition due to eating disorders are underdiagnosed, they commonly present to care more medically unstable compared with females. As in the adolescent in the vignette, over-exercise is a common maladaptive behavior in males. Boys and adolescent males with eating disorders commonly have comorbid psychological conditions (eg, depression, anxiety, and substance use disorders).\nChildren and adolescents with eating disorders are at risk for a permanent negative impact on height attainment, as the normal pubertal height velocity increase typically occurs during the time when these individuals have severe malnutrition. There is a risk of low bone-mineral density associated with malnutrition, but this can be more difficult to detect in males, given the lack of menstrual cycles as an indicator of normal hormonal function. Because malnutrition causes hypothalamic hypogonadism, testosterone levels may be lower in affected males; treatment may be indicated and should be guided by an endocrinologist.\nSuggested Reading(s)\nGolden N, Katzman D, Sawyer S, et al. Update on the medical management of eating disorders in adolescents. J Adolesc Health. 2015;56,4:370-375. doi:10.1016/j.jadohealth.2014.11.020\nHornberger LL, Lane MA, et al: American Academy of Pediatrics Committee on Adolescence. Identification and management of eating disorders in children and adolescents. Pediatrics. 2021;147(1):e2020040279. doi:10.1542/peds.2020-040279\nRome E, Strandjord SE. Eating disorders. Pediatr Rev. 2016;37(8):323–336. doi:10.1542/pir.2015-0180\nSchneider M, Fisher M. Anorexia nervosa, bulimia nervosa, and other eating disorders. In: McInerny TK, Adam HM, Campbell DE, DeWitt TG, Foy JM, Kamat DM, eds. American Academy of Pediatrics Textbook of Pediatric Care. American Academy of Pediatrics; 2021:chap 216. Accessed September 21, 2024. Pediatric Care Online\nVo M, Lau J, Rubinstein M. Eating disorders in adolescent and young adult males: presenting characteristics. J Adolesc Health. 2016;59(4):397–400. doi:10.1016/j.jadohealth.2016.04.005\nContent Domain\nMental health\nLearning Objectives\nRecognize the clinical presentation of an adolescent with an eating disorder and manage appropriately\nThe correct answer is:\nrestriction of physical activity\nView Peer Results"}
{"id" : 2050, "question_text" : "A 15-year-old adolescent boy with a 5-day history of nausea, dark tarry stools, and dizziness is brought to the emergency department for a syncopal episode. He has previously had intermittent heartburn and mild abdominal pain, but not blood in his stools. He reports no recent use of nonsteroidal anti-inflammatory drugs or other over-the-counter medications. He also reports no alcohol or recreational drug use. He has a heart rate of 120 beats/min and blood pressure of 112/48 mm Hg. He is pale and fatigued. He has tachycardia and a grade 1/6 systolic murmur. His abdomen is soft and nontender without masses or hepatosplenomegaly. A digital rectal examination reveals purple, hemoccult-positive liquid stool. A nasogastric tube is placed in the emergency department and reveals dark red blood. Two large-bore intravenous catheters are placed. Laboratory results show: White blood cell count 8,000/µL (8.0 × 109/L), Hemoglobin 6.3 g/dL (63 g/L), Mean corpuscular volume 91 fL, Platelet count 167 × 103/µL (167 × 109/L), Prothrombin time 12.6 s, Partial thromboplastin time 35 s, Alanine aminotransferase 33 U/L, Aspartate aminotransferase 15 U/L, Total bilirubin < 0.5 mg/dL (8.6 µmol/L). Of the following, the BEST next step in management is to", "options" : "[\"administer packed red blood cells\", \"perform abdominal ultrasonography with Doppler\", \"perform endoscopy\", \"perform a Meckel scan\"]", "explanation" : "The patient in this vignette has evidence of an active gastrointestinal bleed with hemodynamic instability in the context of significant anemia; thus, the best next step in management is to stabilize the patient by the rapid administration of isotonic fluids and packed red blood cells. Patients with hemodynamic instability should be considered for intensive care management.\n\nGastrointestinal bleeding may manifest in various ways. Upper gastrointestinal tract bleeding (proximal to the ligament of Treitz) can present with hematemesis, melena, or even hematochezia if the bleeding is rapid. Lower gastrointestinal tract bleeding can present as melena or hematochezia. Although determining the source of the bleeding is necessary, regardless of the etiology, stabilization (with intravenous fluid, packed red blood cells, and/or other supportive measures) is the necessary first step. Confirming the presence of blood (in emesis or stool) is generally recommend with guaiac testing. Additional testing, including assessment of a complete blood cell count, coagulation studies, and liver function testing can help determine the bleeding severity and diagnosis.\n\nIf an upper gastrointestinal tract bleed is suspected (ie, hematemesis or melena), nasogastric lavage may help to confirm the presence of blood in the stomach. Causes of upper gastrointestinal tract bleeding include esophagitis, foreign body, Mallory-Weiss tear, esophageal varices, gastritis, gastric or duodenal ulcer, arteriovenous malformation, and portal hypertensive gastropathy. Endoscopy is generally recommended to diagnose the etiology of the upper gastrointestinal bleed and can be used therapeutically to provide hemostasis (through clips, coagulants, and other endoscopic tools).\n\nCauses of lower gastrointestinal tract bleeding include anal fissure, infectious enteritis/colitis, eosinophilic gastrointestinal disease, intestinal polyp, arteriovenous malformation, Meckel diverticulum, intussusception, and inflammatory bowel disease. Colonoscopy is generally indicated if infection and surgical causes of bleeding (intussusception, Meckel diverticulum) are unlikely.\n\nAlthough abdominal ultrasonography with Doppler, endoscopy, and a Meckel scan could be helpful diagnostic tests, stabilization of this hemodynamically unstable patient should occur prior to these studies. Given that blood was noted during the nasogastric lavage, a lower gastrointestinal bleed is unlikely and therefore a Meckel scan would not be initially recommended.\n\nPREP Pearls\n\nGastrointestinal bleeding can present with hematemesis, melena, and/or hematochezia.\n\nInitial assessment should include vital sign determination and laboratory testing to evaluate the severity of the gastrointestinal bleeding.\n\nInitial management of patients with gastrointestinal bleeding should include stabilization with intravenous fluids and packed red blood cells as necessary.\n\nMOCA-Peds Objective\n\nRecognize causes of gastrointestinal bleeding.\n\nABP Content Specifications(s)/Content Area\n\nPlan the appropriate evaluation of blood in vomitus or stool, including in a patient who has hemodynamically significant blood loss\n\nPlan the appropriate evaluation of upper gastrointestinal bleeding\n\nSuggested Readings\n\nNeidich GA, Cole SR. Gastrointestinal bleeding. Pediatr Rev. 2014;35(6):243-254. doi:10.1542/pir.35-6-243..\n\nSahn B, Mamula P, Friedlander J. Gastrointestinal hemorrhage. In: Wyllie R, Hyams JS, Kay M, eds. Pediatric Gastrointestinal and Liver Diseases. 5th ed. Philadelphia, PA: Elsevier; 2016:144-154."}
{"id" : 1182, "question_text" : "You are seeing a 13-year-old adolescent who has developed significant school avoidance. She has periodically missed school over the past year when she had physical complaints or reported having severe anxious feelings before school. This has worsened recently over the past week with complaints of headache, stomachache, and anxiety before school each morning, causing her to miss school each day. The mother notes that these complaints are relieved when she stays at home by herself or when she goes to work with her mother. The adolescent denies any bullying occurring at her school. She has been a good student, except for missing assignments when she is absent from school. She has a history of being \"clingy\" with her mother periodically over the years. Of the following, the management that would MOST likely produce a positive outcome is", "options" : "[\"arrange for temporary home tutoring while outpatient counseling is initiated\", \"arrange for the parent to remain with the child in the classroom for 2 hours each morning\", \"create a plan for an immediate, unaccompanied return to the classroom\", \"prescribe lorazepam and arrange for her to use it as needed during the school day\", \"set up a plan where the child knows the parent will visit her once a day at school\"]", "explanation" : "School avoidance can happen with or without a psychiatric cause, for instance, avoiding school just because the child prefers their nonschool environment, which is also known as truancy. The adolescent in this vignette might have a separation anxiety disorder (given her history of being \"clingy\" with mom), or she might have a somatic symptom disorder. Her history of experiencing headaches and stomach aches right before going to school, which are then relieved as she avoids school, is a typical way for anxiety to manifest as physical symptoms. Therefore, her school avoidance is most likely related to anxiety.\n\nSeparation anxiety disorder is a developmentally inappropriate and excessive anxiety about separating from home or from an individual with a persistence beyond 4 weeks. While as many as half of early school age children demonstrate some separation anxiety symptoms, only about 4% develop a level of dysfunction consistent with a separation anxiety disorder. There are both genetic and social origins for the development of separation anxiety disorder. There may be an inborn low threshold for experiencing anxiety that enables not just the appearance of separation anxiety disorder, but also other anxiety disorders like generalized anxiety disorder and social phobia. Even in the absence of any particular genetic predisposition for experiencing anxiety, highly anxious parenting may teach children to adopt a fearful view of their world.\n\nSchool avoidance can be a major problem when it occurs because it typically becomes increasingly difficult to resolve the longer the child remains out of school. One reason why prolonged avoidance is such a problem is that our brains interpret anxiety relief from avoidance as proof that a fear was well founded, and thus future anxious reactions to the same situation deepen. For children avoiding school, this means that their fears about school usually increase the longer their duration of avoidance, and it becomes more and more difficult to get them to return.\n\nThe hallmark of an effective school avoidance intervention involves getting the child back into school immediately without their parent sitting next to them. Supports of many forms can be provided as appropriate while the child is at school, such as homework or class work modifications, a plan for how the child will receive support by school staff, schedule modifications, etc. If any persisting anxiety is present, enrollment in psychotherapy would be appropriate. If the trigger for the avoidance was a truly aversive situation such as school bullying, then that will need to be addressed.\n\nTemporary home tutoring is counter productive for anxiety driven school avoidance because it makes it easier for the child and family to avoid a return to school. Arranging for a parent to remain in the classroom is a strategy that parents might request for a young child with separation anxiety, but this is likely to only delay the separation crisis, as it nonverbally communicates to the child that they cannot handle the situation on their own, and it is distracting to child and classroom function while the parent is present. Setting up a plan for the parent to visit the child during the day at scheduled times effectively creates additional separation experiences for the child each day, and a daily opportunity for a child to \"build their case\" as for why they need to leave mid-day. A single separation at the start of school is usually easier on both parents and children in this situation. Pharmacologic intervention is not indicated.\n\nPREP Pearls\n • School avoidance is often related to anxiety, which may manifest as headaches and stomach aches before going to school.\n • School avoidance becomes more intervention-resistant the longer the child remains out of school. An unaccompanied return to school as soon as possible is needed for these children.\n\nABP Content Specifications(s)\n • Distinguish between separation anxiety and truancy as a cause of school absence\n • Understand the relationship between separation anxiety with school phobia/refusal in patients of various ages\n • Plan the appropriate management of separation anxiety of various etiologies\n • Recognize the family dynamics associated with separation anxiety"}
{"id" : 818, "question_text" : "Parents bring in their 6-year-old daughter because her toes turn in when she walks. They are concerned because imaging studies have not been performed and treatment has not been prescribed. Examination of this appropriately grown child is notable for moderate intoeing and 90° of internal rotation of both hips when she is in the prone position (Item Q111). There is no evidence of metatarsus adductus or tibial torsion. Aside from the intoeing, she has a normal gait. Of the following, the MOST appropriate next step in the care of this child is to", "options" : "[\"counsel the family that a substantial portion of children with this condition will require surgical intervention as they grow older\", \"obtain a computed tomography scan to define precisely the femoral neck anatomy\", \"prescribe twist cables and corrective shoes\", \"reassure the parents that this child has a good prognosis for resolution as the child matures\", \"refer to orthopedics because the intoeing has not corrected by 6 years of age\"]", "explanation" : "Preferred Response: D\nFemoral anteversion occurs when the femoral neck is rotated anteriorly compared with the transcondylar axis of the knee and the long axis of the femur. It is often the result of intrauterine positioning and genetic influences and is twice as frequent in girls as in boys. As seen in the patient in the vignette, common characteristics include medially facing patellae when standing and intoeing, with the patella pointing toward the midline when walking. The running gait of a child with femoral anteversion has been described as having an \"egg-beater\" or \"windmill\" appearance with medial rotation of the thighs and outward rotation of the feet. Physical examination for femoral anteversion is conducted with the patient lying prone. With the knees flexed 90°, the lower legs are rotated out, causing the hips to rotate in (Item C111). Children with femoral anteversion have markedly increased internal hip rotation up to 90° compared with the normal 35° to 50°. Examination is sufficient to make the diagnosis, and no imaging studies are necessary unless findings are extreme.\n\nFemoral anteversion is developmentally normal at birth. Typically, the anteversion decreases by 1° to 2° per year until the adult position is achieved at skeletal maturity. Parents most commonly seek care for their child with this condition when the child is between 3 and 6 years old and the normal physiologic external rotation contracture of the hip has resolved. In these children, intoeing may continue to increase until age 6 years and then decrease. The condition is not painful and in most cases does not limit function. In about 80% of affected children, femoral anteversion resolves spontaneously, usually by age 7 years. However, in some children the final outcome is not seen until age 11 years.\n\nIn general, referral to an orthopedist is reserved for children who have persistent anteversion beyond age 11 years that causes functional or cosmetic impairment. If anteversion is unilateral, the pediatrician should conduct a thorough physical examination looking for signs of an underlying neurologic condition such as cerebral palsy. Referral to an orthopedic surgeon may be warranted in this setting. For the few patients requiring treatment (ie, those with anteversion greater than 50° as seen on radiographic study), femoral derotational osteotomy is effective but has had, in the past, a high rate of complications. Orthotics, twister cables, splinting, bracing, and physiotherapy are ineffective in changing the course or degree of intoeing.\n\nDespite reassurance by their primary care practitioner, many parents request an orthopedic evaluation for their child with less significant femoral anteversion. In one retrospective study, orthopedic specialists in Scotland found that, of 202 patients referred for intoeing, 86% were discharged after the first visit without a scheduled follow-up and another 5% were discharged within the next 2 years without intervention. Nine children were referred during the study period most commonly because of parental concern. Among parents who completed a questionnaire about their visit, 83% had wanted the referral but a minority specifically asked for it and only 22% felt their child had a major medical problem.\n\nLong-term consequences of femoral anteversion are some-what controversial. Although evidence indicates no substantive link, some practitioners believe there is an increased incidence of osteoarthritis of the hip and knee, slipped capital femoral epiphysis, and knee and patella instability.\n\nPREP Pearls\n• Femoral anteversion is diagnosed clinically in a child who has intoeing when there is increased internal hip rotation (>50°) with the child in the prone position.\n• Besides intoeing, the examiner notes that the patella points medially when these children walk.\n• The normal course of femoral anteversion is spontaneous improvement by the time of skeletal maturity.\n• Orthopedic referral is indicated when anteversion persists beyond age 11 years and causes functional or cosmetic impairment or if the anteversion is unilateral.\n\nAmerican Board of Pediatrics Content Specification(s):\n• Know that the natural history of femoral anteversion is self-correction\n• Know that x-ray studies are not necessary for the diagnosis of femoral anteversion\n• Know how to evaluate a child with femoral anteversion\n\nSuggested Reading:\n• Blackmur JP, Murray AW. Do children who in-toe need to be referred to an orthopaedic clinic. J Pediatr Orthop B. 2010;19:415-417. doi: 10.1097/ BPB.0b013e3283339067\n• Hoekelman RA, Chianese MJ. Foot and leg problems. In: Mclnerny TK, ed. Textbook of Pediatric Care. Elk Grove Village, IL: American Academy of Pediatrics; 2009;Chap 183:1541\n• Rosenfeld SB. Approach to the child with in-toeing. UptoDate. 2013. Available online only for subscription"}
{"id" : 2287, "question_text" : "A 12-year-old boy is undergoing evaluation for scrotal pain that began suddenly at school 5 hours ago and is now constant. He has vomited twice. There has been no trauma. He has not had fever, abdominal pain, dysuria, or prior testicular swelling. On physical examination, his temperature is 37.2 °C, his heart rate is 110 beats/min, his respiratory rate is 22 breaths/min, his blood pressure is 110/78 mm Hg, and his oxygen saturation is 98% in room air. The boy is alert and cooperative but appears uncomfortable. His abdomen is nondistended and nontender, with normal bowel sounds. There is moderate swelling of the right testicle, which is significantly tender and firm to palpation with overlying erythema. His right testicle is visibly higher in the scrotum as compared with the left. No cremasteric reflex can be elicited on either side. The left testicle has a vertical lie and is nontender without swelling or erythema. The closest hospital, located 1 hour away, has a general emergency department with radiologic services but no surgical or subspecialty services. There is a children's hospital 4 hours away. Air transportation is not available owing to inclement weather. Of the following, the BEST next step is", "options" : "[\"contact the children's hospital to arrange an urgent transport\", \"instruct the parent to drive directly to the children's hospital\", \"perform manual external rotation of the right testicle in the office\", \"refer him urgently to the local general emergency department\"]", "explanation" : "Correct answer is C\n\nPREP Pearl(s)\nTesticular torsion, a time-sensitive emergency, should be highly suspected when a boy has acute onset of unilateral testicular pain, nausea/vomiting, testicular tenderness, swelling, erythema/discoloration, and unilateral loss of cremasteric reflex. The affected testicle may appear \"high-riding\" and lie horizontally.\nThe likelihood of testicular survival with good outcomes is highest if testicular torsion is treated within 6 hours of symptom onset.\nManual rotation of a torsed testicle to alleviate ischemia is indicated if definitive surgical intervention is not rapidly accessible.\n\nCritique\nOf the response choices, attempting detorsion with manual external rotation of the testicle is the best next step in this boy's treatment. The child in the vignette has clinical findings that raise great concern regarding testicular torsion. In this condition, the spermatic cord (which contains the testicular artery and vein) twists, leading to reduced arterial blood flow and testicular ischemia. The likelihood of testicular survival with good function is highest when detorsion is accomplished within the first 6 hours. Salvage of a torsed testicle may be possible even after 24 hours, although the likelihood decreases steadily with the duration of torsion. The boy in the vignette has had testicular pain for 5 hours and will not be able to access pediatric surgical care for at least another 4 hours, making immediate detorsion necessary.\n\nThe Testicular Workup for Ischemia and Suspected Torsion (TWIST) score is a tool for evaluating children wiith scrotal pain for possible torsion. The elements of the score are as follows:\nTesticular swelling (2 points)\nA hard testis (2 points)\nA high-riding testis (1 point)\nAbsence of cremasteric reflex (1 point)\nNausea/vomiting (1 point)\nA score of 5 to 7 has a high specificity for testicular torsion and indicates that immediate intervention is warranted. For individuals with a score of 3 to 4, scrotal Doppler ultrasonography is recommended. A score of 0 to 2 indicates that testicular torsion is much less likely to be the cause of scrotal pain. Validation studies have demonstrated that cases of testicular torsion can be missed in individuals with lower TWIST scores; therefore, a high index of suspicion must be maintained if no alternative explanation for testicular pain is identified. The TWIST score for the boy in the vignette is 7, indicating that testicular torsion is a likely cause of his pain and that urgent intervention is necessary.\n\nManual detorsion for testicular torsion is recommended if definitive surgical intervention cannot be accomplished in a timely manner. In most cases of testicular torsion, the affected testicle twists medially; therefore, externally (or laterally) rotating the testicle is recommended. An analgesic should be administered before manual detorsion. The clinician should grasp the affected testicle with the thumb and index fingers, lift it, and then rotate it outward at least 180° and up to 360°. A successful manual detorsion maneuver will result in significant and rapid relief of scrotal pain and descent of the testis (as its twisted vascular stalk unwinds). As many as 30% of torsions require internal/medial detorsion.\n\nAlthough testicular torsion occurs most often in adolescents, this condition can affect children at any age, including in the neonatal period. Evaluation of all children with abdominal pain, genital pain, or unexplained fussiness/crying should include a chaperoned genital examination. The most common risk factor for testicular torsion is the bell clapper deformity, an embryonic malformation in which the serosal covering of the testis (tunica vaginalis) attaches high on the spermatic cord after the testis completes its descent through the inguinal ring. This high attachment causes the testis to lie more horizontally within the scrotum, which allows for easier twisting around the central vascular bundle and thus causes venous engorgement and arterial insufficiency.\n\nThe ischemia associated with testicular torsion causes significant pain, nausea/vomiting, and testicular swelling with redness or dusky discoloration. The testicle is often pulled higher in the scrotum, owing to twisting and shortening of the spermatic cord. This change in position can be difficult to assess, given the degree of discomfort affected individuals may be experiencing during physical examination. Although individuals with testicular torsion may have absence of a cremasteric reflex on the affected side, this reflex may be absent in healthy individuals; this finding is not specific for torsion. The diagnosis of testicular torsion is confirmed via color Doppler ultrasonography: this imaging study has a sensitivity of 88.9% to 96% and a specificity of 98% for testicular torsion. Findings include the \"whirlpool\" sign of the twisted vasculature and absent (or asymmetric) perfusion of the affected testis.\n\nThe differential diagnosis for acute scrotal pain includes torsion of the appendix testis, varicocele, epididymo-orchitis, and inguinal hernia. Less common causes of acute scrotal pain include urinary tract infection, nephrolithiasis, and vasculitides (eg, Henoch-Schönlein purpura).\n\nAlthough some form of transport to a children's hospital would be ideal for the patient in the vignette, this step would take several hours. Therefore, a manual detorsion maneuver should be performed before transport to maximize the likelihood of testicular survival. Referring the child to a local general emergency department without access to definitive surgical management for his condition would not be appropriate.\n\nSuggested Reading(s)\nBowlin PR, Gatti JM, Murphy JP. Pediatric testicular torsion. Surg Clin North Am. 2017;97(1):161-172. doi:10.1016/j.suc.2016.08.012\nMellick LB, Sinex JE, Gibson RW, Mears K. A systematic review of testicle survival time after a torsion event. Pediatr Emerg Care. 2019;35(12):821-825. doi:10.1097/PEC.0000000000001287\nQin KR, Qu LG. Diagnosing with a TWIST: systematic review and meta-analysis of a testicular torsion risk score. J Urol. 2022;208(1):62-70. doi:10.1097/JU.0000000000002496\nSheth KR, Keays M, Grimsby GM, et al. Diagnosing testicular torsion before urological consultation and imaging: validation of the TWIST score. J Urol. 2016;195(6):1870-1876. doi:10.1016/j.juro.2016.01.101\n\nContent Domain\nUrology, Testicular torsion\n\nLearning Objectives\nManage the emergent care of a pediatric patient with testicular torsion"}
{"id" : 2853, "question_text" : "A 10-month-old male infant is seen for follow-up. He was diagnosed with bilateral acute otitis media 3 days ago after developing fever and otalgia with abnormal tympanic membrane findings. He was prescribed a 10-day course of high-dose amoxicillin and ibuprofen as needed. He has received 6 doses of amoxicillin, but the fever persists despite administration of ibuprofen every 6 hours. He has had a poor appetite and a few episodes of nonbloody, nonbilious emesis. His parents have also noted that his eyes seem swollen and he is urinating less frequently. His history includes a previous history of otitis media treated successfully with amoxicillin at 4 months of age. He appears to be nontoxic and well hydrated. His weight and length are in the 75th percentile, and his blood pressure is in the 87th percentile. There is mild periorbital edema without conjunctival or scleral injection and no discharge. Other significant findings include erythematous, opaque, and bulging tympanic membranes bilaterally. The remainder of the physical examination findings are within normal limits. Laboratory findings reveal sterile pyuria, proteinuria, hematuria, and elevated levels of blood urea nitrogen and creatinine. Findings of a urine culture are negative. Of the following, the BEST initial step in management is to", "options" : "[\"admit for intravenous fluid administration\", \"admit for intravenous steroid administration\", \"discontinue amoxicillin\", \"discontinue ibuprofen\"]", "explanation" : "The clinical presentation of the infant in this vignette is consistent with acute interstitial nephritis (AIN) caused by the nonsteroidal anti-inflammatory drug (NSAID) ibuprofen, which can result in acute kidney dysfunction. Acute kidney injury induced by NSAIDs may be hemodynamically mediated or secondary to AIN. Nonsteroidal anti-inflammatory drugs lead to a decrease in glomerular filtration rate by causing reversible renal ischemia, even more so in a setting of decreased intravascular volume (dehydration, vomiting, and diarrhea). The child in the vignette has decreased appetite and vomiting, and is at risk of experiencing acute kidney injury secondary to NSAID-induced renal vasoconstriction. However, the presence of hematuria, proteinuria, and sterile pyuria favors a diagnosis of AIN. Acute interstitial nephritis should be included in the differential for sterile pyuria.\n\nAmoxicillin may also cause AIN, but in this vignette the infant had received amoxicillin in the past without issue. Reactions may recur with re-exposure to the same class of medications and, therefore, ibuprofen should be added to this patient's allergy list.\n\nThe mainstay of treatment for drug-induced AIN is discontinuation of the offending agent, which in this case is ibuprofen. The majority of patients improve after the offending medication is withdrawn. However, for patients with AIN-caused acute kidney injury that is severe enough to warrant dialysis, glucocorticoids may be initiated after a renal biopsy. Nonsteroidal anti-inflammatory drug–induced AIN does not typically respond to glucocorticoid therapy. Other supportive interventions may include fluid and electrolyte management, adequate hydration, and avoidance of nephrotoxic drugs. Increasing hydration alone without removal of the NSAID would not be adequate treatment for this patient.\n\nPREP Pearls\n• Drug-induced acute interstitial nephritis is commonly caused by nonsteroidal anti-inflammatory drugs, such as ibuprofen.\n• The mainstay of initial treatment for drug-induced acute interstitial nephritis is discontinuation of the offending agent. The majority of patients improve after the offending medication is withdrawn.\n• Supportive interventions for acute interstitial nephritis may include fluid and electrolyte management, adequate hydration, and avoidance of nephrotoxic drugs.\n\nABP Content Specifications(s)\n• Recognize the risks associated with the use of nonsteroidal anti-inflammatory drugs\n\nSuggested Readings\n• Joyce E, Glasner P, Ranganathan S, Swiatecka-Urban A. Tubulointerstitial nephritis: diagnosis, treatment, and monitoring. Pediatr Nephrol. 2017;32(4):577-587. doi:10.1007/s00467-016-3394-5.\n• Kodner CM, Kudrimoti A. Diagnosis and management of acute interstitial nephritis. Am Fam Physician. 2003;67(12):2527-2534. https://www.aafp.org/afp/2003/0615/p2527.html.\n• Ruebner RL, Fadrowski JJ. Tubulointerstitial nephritis. Pediatr Clin N Am. 2019;66:(1)111-119. doi:10.1016/j.pcl.2018.08.009.\n• Varade WS. Nephritis. In: McInerny TK, Adam HM, Campbell DE, DeWitt TG, Foy JM, Kamat DM, eds. American Academy of Pediatrics Textbook of Pediatric Care. 2nd ed. Elk Grove Village, IL: American Academy of Pediatrics; 2017:2358-2367. Pediatric Care Online."}
{"id" : 2602, "question_text" : "A child is admitted to the hospital with a diagnosis of chickenpox. Oral hydroxyzine is ordered for severe pruritus. A second child in the hospital room next door is undergoing therapy for essential hypertension and is prescribed parenteral hydralazine. The 2 children are being cared for by the same physician and bedside nurse. A medication error occurs when these similarly sounding medications are inadvertently placed in the wrong patient medication bin, and subsequently administered to the wrong child. Of the following, the systems-based medication safety practice that MOST likely would have prevented this medication error is", "options" : "[\"black box safety labels\", \"color-coded medication vials\", \"multi-dose medication containers\", \"tall man lettering\"]", "explanation" : "The vignette describes a \"look-alike, sound-alike\" medication error that occurred because 2 drugs with similarly sounding names, hydralazine and hydroxyzine, were confused. The systems-based medication safety practice that most likely would have prevented this medication error is the use of tall man lettering.\n\nTall man lettering uses uppercase letters, often in boldface print, to help differentiate medication names that appear or sound similar. Tall man lettering helps distinguish between similar drug names by capitalizing dissimilar letters (eg, hydrALAZINE and hydrOXYzine; DOPamine and DOBUTamine). In addition to being a specific visual clue that can assist the clinician in performing a visual double check to verify the correct medication, tall man lettering in and of itself can alert the health care professional that the drug name can be confused with another similarly sounding drug name.\n\nThe Institute for Safe Medication Practices (ISMP) coined the term \"tall man lettering\" and since 2008, has maintained a list of drug names with recommended, bolded tall man letters. In the United States, if the US Food and Drug Administration (FDA) determines that a medication name warrants tall man lettering, they will request that the manufacturer voluntarily revise their labels to include tall man lettering. Additionally, the FDA may disseminate safety communications to alert health care professionals of the potential medication name confusion. Medication errors are extremely common and are the leading cause of medical error. One in every 1,000 drugs prescribed in both inpatient and ambulatory settings have been associated with a wrong drug selection during the process of prescribing, transcribing, dispensing, or administering medications.\n\nBlack box warning labels issued by the FDA serve to warn the public and prescribers of potentially life-threatening medication side effects. The use of single-dose vials (as opposed to multi-dose vials) or single-dose drug packaging can reduce medication error, however neither directly impacts the risk of a look-alike, sound-alike medication error. Color-coded medication vials or vial tops can help distinguish medications from one another, however this practice is less likely than tall man lettering to reduce the risk of a look-alike, sound-alike error.\n\nPREP Pearls\n• Medication errors are the most common medical error, with a frequency of nearly 1 in 1,000 inpatient medication orders and ambulatory prescriptions.\n• Look-alike, sound-alike medication errors arise from confusion in the prescribing, dispensing, or administration of medications with similar sounding names.\n• Tall man lettering can help mitigate look-alike, sound-alike errors by providing a visual cue that the particular medication name carries a high risk for confusion and medication error.\n\nABP Content Specifications(s)\n• Understand the role of medical device design in prevention of medical error\n• Understand the impact of product naming and packaging on medication safety\nimpact of product naming and packaging on medication safety\n\nSuggested Readings\n• Bryan R, Aronson JK, Williams AJ, Jordan S. A systematic literature review of LASA error interventions. Br J Clin Pharmacol. 2021;87(2):336-351. doi:10.1111/bcp.14644.\n• DeHenau C, Becker MW, Bello NM, Liu S, Bix L. Tallman lettering as a strategy for differentiation in look-alike, sound-alike drug names: the role of familiarity in differentiating drug doppelgangers. Appl Ergon. 2016;52:77-84. doi:10.1016/j.apergo.2015.06.009.\n• Lambert BL, Schroeder SR, Galanter WL. Does tall man lettering prevent drug name confusion errors? Incomplete and conflicting evidence suggest need for definitive study. BMJ Qual Saf. 2016;25(4):213-217. doi:10.1136/bmjqs-2015004929."}
{"id" : 904, "question_text" : "You receive a message from the mother of a 14-year-old girl who you will be seeing in the office today. The mother requests that you test her daughter for drugs and that you not tell her daughter that she made such a request. When they arrive, both mother and daughter enter the examination room together.\n\nOf the following, the MOST appropriate way to manage this situation is to", "options" : "[\"ignore the request as the subject is not brought up during the encounter\", \"inform the patient of her mother's request and ask for a urine sample\", \"obtain a urine sample and then inform the patient you will be testing her\", \"request to speak with the patient alone before proceeding with testing\", \"send a urine drug screen without the patient's knowledge\"]", "explanation" : "In addition to speaking with parents and their adolescents together, speaking with a parent and an adolescent separately to discuss confidentiality and its limits is important to developing a trusting relationship. This will in turn allow for open communication. The history is the most important part of the visit with an adolescent and will aid in the diagnosis in most cases. This should occur irrespective of the age of the adolescent. Knowing that the adolescent has access to a trusted physician will allow the adolescent to open up, if not at a current visit then in the future, when ready to reveal details.\n\nRequests made by parents should never be ignored. In the vignette, the mother's concerns should be addressed when speaking with her alone. She needs to understand that obtaining a drug screen without her daughter's knowledge will make it more difficult to help her if there is a substance use problem because the physician-patient relationship will be compromised. After speaking with the mother alone, speaking with the adolescent alone will allow for developing a bigger picture of her functioning at home, at school, and with her peers, along with assessing her mental health status. This information will be more useful in deciding next steps. Asking for a urine sample in the mother's presence without previous discussion will not give the adolescent a chance to discuss the situation, and she may feel pressured to comply, with resultant loss of trust.\n\nPREP Pearls\n• The role of confidentiality in being able to obtain a complete psychosocial history cannot be underestimated.\n• Confidentiality should be discussed with families and provided to all adolescents of all ages.\n• Drug screens should not be obtained without an adolescent's knowledge.\n\nAmerican Board of Pediatrics Content Specification(s):\n• Know that age alone does not dictate whether parents accompany adolescents during the physical examination and history\n• Understand the importance of interviewing an adolescent without the parents present\n\nSuggested Reading:\n• American Academy of Pediatrics: Policy Statement: Substance Use Screening, Brief Intervention, and Referral to Treatment for Pediatricians Committee on Substance Abuse. Pediatrics. 2011;128:e1330-e1340. DOI: 10.1542/peds.2011-1754\n• Britto MT, Tivorsak TL, Slap GB. Adolescents' needs for health care privacy. Pediatrics. 2010;126: e1469-e1476. doi:10.1542/peds.2010-0389\n• Committee on Substance Abuse and Council on School Health. Testing for drugs of abuse in children and adolescents: addendum—testing in schools and at home. Pediatrics. 2007;119: 627-630. DOI: 10.1542/peds.2006-3688\n• Henry-Reid LM, O'Connor KB, Klein JD, Cooper E, Flynn P, Futterman DC. Current pediatrician practices in identifying high-risk behaviors of adolescents. Pediatrics. 2010;125: e741-e747. doi:10.1542/peds.2009-0271\n• Levine SB. Adolescent consent and confidentiality. Pediatr Rev. 2009;30:457-459. DOI: 10.1542/pir.30-11-457"}
{"id" : 2360, "question_text" : "A 2-year-old boy is seen for a health supervision visit. He achieved his motor milestones on time but is not saying any words yet. Physical examination findings are remarkable for 8 café au lait (CAL) macules that are each greater than 5 mm. The family history is remarkable for an older sister with multiple CAL macules and a history of optic pathway glioma; their mother with multiple CAL macules, multiple neurofibromas, and a large plexiform neurofibroma on her neck; and multiple maternal family members with similar and additional findings of this same disorder. Of the following, the MOST likely risk of this boy's parents having another child with his same disorder is", "options" : "[\"25%\", \"50%\", \"75%\", \"100%\"]", "explanation" : "PREP Pearl(s)\nAutosomal dominant inheritance is characterized by multiple affected family members in multiple generations, equal risk in males and females, and a 50% risk of passing on the condition with every offspring. Examples include neurofibromatosis type 1, achondroplasia, and tuberous sclerosis complex.\nAutosomal recessive inheritance is characterized by unaffected parents of an affected child. Parental consanguinity increases the risk. Examples include cystic fibrosis and sickle-cell anemia.\nX-linked inheritance is characterized by the absence of male-to-male transmission. Some X-linked dominant disorders have male lethality (eg, incontinentia pigmenti); a few have presentation both in males and females (eg, X-linked hypophosphatemia). X-linked recessive disorders (eg, Duchenne muscular dystrophy) are characterized by variable presentation in carrier females and a severe phenotype in males.\nMitochondrial inheritance is maternally inherited (absence of transmission of the disorder from an affected male). All children of an affected mother will be affected. Examples include Leber hereditary optic neuropathy and mitochondrial encephalomyopathy, lactic acidosis, and stroke-like episodes (MELAS).\nCritique\nThe boy in the vignette and many of his maternal family members have neurofibromatosis type 1 (NF1). Neurofibromatosis type 1 is inherited in an autosomal dominant pattern with variable expressivity. Each affected individual has a 50% risk of passing on the disorder with every offspring. An example of an autosomal dominant disorder pedigree is shown in the Figure.\nThe clinical criteria for NF1 include the following:\nIn the absence of a parent with diagnosed NF1, at least 2 of the following features are required to make a clinical diagnosis.\nCafé au lait macules (at least 6; >5-mm prepubertal, >15-mm postpubertal)\nFreckles in the axillary or inguinal region\nCutaneous neurofibromas (≥2)\nLisch nodules (≥2) or choroidal abnormalities (≥2)\nPlexiform neurofibroma (at least 1)\nOptic pathway glioma\nOsseous lesion (sphenoid dysplasia, anterolateral tibial bowing, or long bone pseudoarthrosis)\nPathogenic variant in the NF1 gene\nIn the presence of a parent diagnosed with NF1, 1 of the above features is sufficient to make a clinical diagnosis.\nThe different patterns of inheritance are outlined in the Table.\nSuggested Reading(s)\nLegius E, Messiaen L, Wolkenstein P, et al; International Consensus Group on Neurofibromatosis Diagnostic Criteria (I-NF-DC). Revised diagnostic criteria for neurofibromatosis type 1 and Legius syndrome: an international consensus recommendation. Genet Med. 2021;23(8):1506-1513. doi:10.1038/s41436-021-01170-5\nNussbaum RL, McInnes RR, Willard HF. Patterns of single gene inheritance. In: Thompson and Thompson Genetics in Medicine. 8th ed. Elsevier; 2016:107-132.\nSaul RA. Fundamentals of genetics and genomics. Medical Genetics in Pediatric Practice. American Academy of Pediatrics; 2013:1-20.\nContent Domain\nGenetics\nABP Content Specification(s) / Content Area(s)\nRecognize the inheritance pattern associated with mitochondrial inheritance\nRecognize the inheritance pattern associated with X-linked recessive disorders\nRecognize the inheritance pattern associated with X-linked dominant disorders\nThe correct answer is: 50%\nView Peer Results"}
{"id" : 3311, "question_text" : "A 2-year-old girl is brought to the emergency department with a 10-day history of crampy abdominal pain and watery diarrhea that the parents report now contains blood. The family just returned from a 3-week trip to Mexico where they traveled extensively in the countryside. On physical examination, her temperature is 38°C, heart rate is 130 beats/min, respiratory rate is 20 breaths/min, blood pressure is 100/60 mm Hg, and weight is 10.6 kg (10th percentile). The parents report her weight at a recent 2-year-old well-child check was 11 kg. The child appears ill, but nontoxic. She is interactive with the examiner. Her mucous membranes are moist, and capillary refill is less than 2 seconds. Her heart rhythm is normal without murmur, and her lungs are clear to auscultation. Her abdomen is soft, nondistended, and tender to palpation in the lower quadrants without rebound tenderness. Of the following, the MOST likely etiology of the patient's illness is", "options" : "[\"enteropathogenic Escherichia coli\", \"enterotoxigenic Escherichia roll\", \"Entamoeba histolytica\", \"Giardia intestinalis\", \"Salmonella ser enteritidis\"]", "explanation" : "Preferred Response: C\nThe girl in the vignette has crampy abdominal pain and bloody diarrhea after travel to Mexico. Given her symptoms and travel to a location that likely had poor sanitary conditions, the most likely cause of her illness is Entamoeba histolytica. This pathogen is found worldwide, most commonly in tropical regions and resource-limited countries. It is spread by the fecal-oral route. The incubation period is variable, usually 2 to 4 weeks. Only a minority (10% to 20%) of infected persons become symptomatic. Illness can be mild with bloody stools, intense abdominal pain, and fever (amebic dysentery). Severe infection can result in toxic megacolon, gastrointestinal ulcers, or intestinal perforation. Rare extraintestinal complications include amebic liver abscess or spread to other organs such as the lungs or brain.\n\nPresumptive diagnosis of intestinal amebiasis can be made by identification of trophozoites or cysts in the stool. Definitive diagnosis is made by detection of anti-E histolytica antibody by enzyme immunoassay. Nearly all (95%) patients with extraintestinal amebiasis have positive serology versus 70% of those with infection limited to the intestinal tract. Recommended treatment is metronidazole (30-40 mg/kg per day, maximum of 2 g, orally in 3 doses for 7-10 days) or tinidazole (50 mg/kg per day, maximum of 2 g, orally once daily for 3-5 days, 3 years of age and older). Either treatment is followed by a luminal amebicidal agent such as paromomycin (25-35 mg/kg per day orally in 3 doses for 7 days) or iodoquinol (30-40 mg/kg per day orally in 3 doses for 20 days). Patients who are asymptomatic, but excreting cysts can be treated with a luminal amebicidal agent alone.\n\nDiarrhea caused by enteropathogenic Escherichia coli usually is mild, watery, nonbloody, and common in resource-limited countries. It primarily affects children younger than 2 years of age. Enterotoxigenic E coil causes a brief (1- to 5-day) diarrheal illness in travelers and young infants in resource-poor settings characterized by watery, nonbloody diarrhea and abdominal cramps. Giardia intestinalis is the most common cause of parasitic diarrhea worldwide. It causes a spectrum of disease, most commonly presenting with watery, nonbloody diarrhea with abdominal pain, foul-smelling stools, and flatulence. Salmonella serotype Enteritidis can cause bloody diarrhea and fever; however, travel to resource-limited settings is not a risk factor for infection caused by nontyphoidal Salmonella species, and the incubation period is less than 72 hours.\n\nPREP Pearls\n• Travel to resource-limited settings is a risk factor for intestinal amebiasis (Entamoeba histolytica infection).\n• Entamoeba histolytica can be asymptomatic or cause severe illness with bloody stools, severe abdominal pain, and fever (amebic dysentery).\n• Rare extraintestinal complications of E histolytica infection include amebic liver abscess or spread to other organs such as the lungs or brain.\n• Presumptive diagnosis of intestinal amebiasis is by identification of trophozoites or cysts in stool; definitive diagnosis is made by detection of anti-E histolytica antibodies with an enzyme immunoassay.\n• Treatment of E histolytica infection is metronidazole or tinidazole, followed by a luminal amebicidal agent such as paromomycin or iodoquinol. Patients who are asymptomatic, but excreting cysts can be treated with a lumina' amebicidal agent alone.\n\nAmerican Board of Pediatrics Content Specification(s)\n• Understand the epidemiology of amoebiasis\n• Recognize the clinical features associated with amoebiasis, and manage appropriately\n\nSuggested Reading\n• American Academy of Pediatrics. Amebiasis. In: Pickering LK, Baker CJ, Kimberlin DW, Long SS, eds. Red Book: 2012 Report of the Committee on Infectious Diseases. 29th ed. Elk Grove Village, IL: American Academy of Pediatrics; 2012:222-225.\n• American Academy of Pediatrics. Escherichia coli diarrhea. In: Pickering LK, Baker CJ, Kimberlin DW, Long SS, eds. Red Book: 2012 Report of the Committee on Infectious Diseases. 29th ed. Elk Grove Village, IL: American Academy of Pediatrics; 2012:324-328.\n• American Academy of Pediatrics. Giardia intestinalis infections. In: Pickering LK, Baker CJ, Kimberlin DW, Long SS, eds. Red Book: 2012 Report of the Committee on Infectious Diseases. 29th ed. Elk Grove Village, IL: American Academy of Pediatrics; 2012:333-335.\n• American Academy of Pediatrics. Salmonella infections, In: Pickering LK, Baker CJ, Kimberlin DW, Long SS, eds. Red Book: 2012 Report of the Committee on Infectious Diseases. 29th ed. Elk Grove Village, IL: American Academy of Pediatrics; 2012:635-640."}
{"id" : 1864, "question_text" : "A 7-year-old boy has recurrent bright red blood per rectum. His mother reports intermittently seeing blood in the toilet for the last year. She states that she has occasionally seen a mass coming from his rectum that looks like a raspberry. He has daily, soft stools, without straining. He reports no abdominal pain, pallor, fatigue, or weight loss. There is no family history of early colon cancer. He has a weight of 25.4 kg (60th percentile), height of 122 cm (35th percentile), and body mass index of 17 kg/m2 (75th percentile). He appears healthy and in no acute distress. He has no rash or mucocutaneous hyperpigmentation. His abdomen is soft, nontender, and nondistended. On digital rectal examination, a 1- to 2-cm nontender mass is palpated. He is referred to a pediatric gastroenterologist. A colonoscopy is performed, and 8 polyps are removed. Item Q64 shows a representative lesion.\n\nItem Q64: Polyp as described in the vignette. Courtesy of J Sullivan\n\nHistopathologic examination of the polyps reveals that they are juvenile polyps.\n\nOf the following, the BEST next step in management is", "options" : "[\"referral for genetic testing\", \"referral to a colorectal surgeon for colectomy\", \"repeat colonoscopy only if rectal bleeding recurs\", \"thyroid ultrasonography\"]", "explanation" : "The boy in this vignette likely has juvenile polyposis syndrome (JPS). Although the polyps removed are classified as juvenile polyps, the presence of 5 or more polyps suggests a diagnosis of JPS. Children with a suspected polyposis syndrome should be referred for genetic testing to confirm the diagnosis and follow surveillance guidelines.\n\nJuvenile polyps are the most common type of colonic polyp in children. Typically manifesting as painless causes of rectal bleeding in young children, they are benign. Polypectomy during colonoscopy is indicated for confirmation of diagnosis and resolution of symptoms. Most commonly, simple juvenile polyps are present in the rectosigmoid colon. If less than 5 juvenile polyps are present, the diagnosis is likely simple juvenile polyps, and routine follow-up colonoscopy is not recommended. However, if symptoms recur, repeat colonoscopy is indicated.\n\nPolyposis syndromes should be suspected in children with 5 or more juvenile polyps, adenomatous polyps, and/or a family history of polyposis or early colorectal cancer. Children or adults with 5 or more juvenile polyps may have JPS. Most commonly, patients describe painless rectal bleeding; however, some patients may experience diarrhea, abdominal pain, or bowel obstruction from intussusception. Mutations in SMAD4, BMPR1A, and ENG are associated with JPS. Patients with JPS have an increased risk of colorectal and gastric cancer, and surveillance with endoscopy and colonoscopy is recommended annually if polyps are present and every 3 years if no polyps are found. Patients with SMAD4 mutations are also at increased risk for vascular malformations.\n\nOther polyposis syndromes include Peutz-Jeghers syndrome, which is caused by STK11/LKB1 mutations and characterized by mucocutaneous macules and polyps throughout the gastrointestinal tract that can result in intussusception, and familial adenomatous polyposis (FAP) syndrome, which is caused by APC mutations and characterized by numerous adenomatous polyps and significant risk for colorectal cancer. Both syndromes have increased risk for extraintestinal malignancies and have specific screening guidelines.\n\nIf a polyposis syndrome is suspected, genetic testing is recommended. Referral to a colorectal surgeon may be appropriate in cases with severe polyposis and increased risk of colorectal cancer (particularly in young adults with FAP syndrome), but it is not appropriate for the boy in this vignette because the diagnosis has not been established. Because the boy has 5 or more polyps, a polyposis syndrome is very likely; thus, surveillance colonoscopy would be indicated every 1 to 3 years as appropriate. Thyroid ultrasonography is indicated in children with FAP because of the increased risk of papillary thyroid cancer. However, the boy is this vignette did not have adenomatous polyps, thus he does not have FAP.\n\nPREP Pearls\n\nSimple juvenile polyps are the most common type of polyp in children.\n\nChildren with 5 or more juvenile polyps or any number of adenomatous intestinal polyps should be referred for genetic testing.\n\nPolyposis syndromes are associated with increased risk of intestinal and extraintestinal malignancies. Surveillance guidelines are available for each type of polyposis syndrome.\n\nABP Content Specifications(s)/Content Area\n\nRecognize the clinical features associated with juvenile polyposis\n\nRecognize the significance of a solitary juvenile polyp\n\nSuggested Readings\n\nHaidle JL, Howe JR. Juvenile polyposis syndrome. GeneReviews. https://www.ncbi.nlm.nih.gov/books/NBK1469.\n\nIglesias IM, Fernandez A, Smith-Singares E, Soyemi K, Arcia R. Abdominal pain, nausea, and vomiting in a 12-year-old girl. Pediatr Rev. 2016;37(2):83-85. doi: 10.1542/pir.2015-0106.\n\nKay M, Eng K, Wyllie R. Colonic polyps and polyposis syndromes in pediatric patients. Curr Opin Pediatr. 2015;27(5):634-641. doi: 10.1097/MOP.0000000000000265."}
{"id" : 1566, "question_text" : "A 4-month-old infant is brought to your office for a routine health supervision visit. He was born at term to a primigravida mother after an uncomplicated pregnancy. His parents report that he \"lights up\" when they enter the room, and he laughs, smiles, and coos. He holds his head steady when he is placed in a seated position and recently began to roll from his back to his abdomen. His parents report that he more consistently reaches for toys with his left hand and preferentially brings this hand in front of his face to gaze at his fingers and place them in his mouth. Of the following, the MOST appropriate assessment is that this infant's development is", "options" : "[\"concerning because he is not yet rolling from his abdomen to his back\", \"concerning because of a hand preference at this age\", \"concerning because of global developmental delay\", \"entirely appropriate for age\", \"overall advanced for age\"]", "explanation" : "The presence of a hand preference or other asymmetry in movement in a 4-month-old infant is a concerning finding that must be evaluated. Consistent handedness does not typically develop until between 4 and 6 years of age, although emerging dominance can be seen between 1 and 3 years of age. As with other asymmetrical movements, handedness that is apparent before 18 months of age may indicate a central or peripheral neurologic abnormality of the opposite side, including hemiparesis.\n\nThe remainder of the developmental milestones described for the boy in this vignette are age appropriate. Although each child progresses slightly differently, typically developing 4-month-old infants have the skills and abilities shown in Item C38. More information about milestones can be found in Bright Futures: Guidelines for Health Supervision of Infants, Children, and Adolescents published by the American Academy of Pediatrics (https://brightfutures.aap.org/materials-and-tools/guidelines-and-pocket-guide/Pages/default.aspx).\n\nPREP Pearls\n• Consistent handedness should not develop before 4 years of age; strong hand preferences at an earlier age may represent relative weakness or neurologic impairment of the \"nondominant\" side.\n• Typically developing 4-month-old infants roll from front to back, reach for objects with their hands, smile spontaneously, initiate social interactions, laugh, and vocalize when alone.\n\nABP Content Specifications(s)\n• Evaluate the cognitive and behavioral developmental progress/status of an infant at 4 months of age, including recognition of abnormalities\n• Evaluate the motor developmental progress/status of an infant at 4 months of age, including recognition of abnormalities\n\nSuggested Readings\n• Scharf RJ, Scharf GJ, Stroustrup A. Developmental milestones. Pediatr Rev. 2016;37(1):25–37. doi: http://dx.doi.org/10.1542/pir.2014-0103."}
{"id" : 1859, "question_text" : "The mother of a 5-year old girl, adopted 2 years ago from Eastern Europe, is concerned that the girl is small for her age and continues to have significant global delays despite appropriate therapy. She has behavioral problems including hyperactivity, decreased attention, and poor memory. Distinctive physical features include microcephaly, smooth philtrum, short palpebral fissures, low nasal bridge, thin upper lip, and fifth finger clinodactyly. Pregnancy and family histories are unknown.\n\nOf the following, the girl's findings are MOST consistent with", "options" : "[\"Angelman fragile X syndrome\", \"fetal alcohol syndrome\", \"Noonan syndrome\", \"Rett syndrome\"]", "explanation" : "The girl in the vignette has classic fetal alcohol syndrome (FAS). To diagnose FAS, a physician should identify 3 cardinal features: abnormal facial features, growth deficiency, and central nervous system problems. The possibility of prenatal alcohol exposure is not required to clinically make the diagnosis, but should be discussed with the child's parents. In cases of adoption or foster care, the maternal history may be unknown. Classic facial features include midfacial hypoplasia, smooth philtrum, thin upper lip, micrognathia, short palpebral fissures, epicanthal folds, small jaw, and microcephaly. Prenatal growth deficiency is typical, as are continued postnatal growth problems. Height and weight are typically less than the 10th percentile. Central nervous system findings include attention/hyperactivity problems, hypotonia, decreased impulse control, poor coordination and memory, learning disabilities (especially in math), speech and language delays, intellectual disability, impaired executive function, and sleep problems. Heart defects may occur; ventricular septal defects and atrial septal defects are most commonly seen.\n\nNo specific laboratory test is available to confirm a diagnosis of FAS; it is a clinical diagnosis. There is no reported \"safe\" level of alcohol use during pregnancy. Any form of alcohol exposure during any trimester poses a similar risk. The neurocognitive impacts are lifelong and are completely preventable if a woman abstains from alcohol use during pregnancy.\n\nSome children exposed to alcohol in utero develop alcohol-related neurodevelopmental disorder or neurobehavioral disorder associated with prenatal alcohol exposure. These children lack the physical stigmata commonly seen with FAS, but have the associated intellectual disabilities and problems with behavior and learning. Abnormalities may be noted on brain imaging. These children typically require an individualized education plan in school; speech, occupational, and physical therapy; specialized math tutoring; executive function training; and parenting and behavior management training. They often require referral to a child psychiatrist to manage the behavioral problems.\n\nAngelman syndrome is associated with intellectual disability, gait ataxia, microcephaly, severe speech delay, and a characteristic happy demeanor with inappropriate laughter and excitability. Developmental delay becomes evident at around 6 months of age; many of the characteristic features do not become apparent until after 1 year of age. Developmental regression is not a common feature of this disorder; however, seizures are quite common. Facial features can include a wide mouth with wide-spaced teeth, prognathism, and a protruding tongue.\n\nNoonan syndrome presents with characteristic facial features, short stature, varying degrees of developmental delay, and congenital heart defects. Facial features include low-set ears with fleshy helices, broad or webbed neck, and wide-spaced, downslanting eyes with epicanthal folds. Common congenital heart defects include pulmonary valve stenosis, atrial septal defect, and hypertrophic cardiomyopathy. Wide-spaced nipples and an unusual chest shape with superior pectus carinatum/inferior pectus excavatum are typical. Microcephaly is not a common feature. Multigene panel testing is available for Noonan syndrome.\n\nRett syndrome, caused by a MECP2 gene mutation, is an X-linked neurodevelopmental disorder that predominantly affects girls. Affected girls typically have normal early growth and development (6-18 months) followed by a characteristic slowing of development, loss of purposeful hand movements, distinctive hand wringing, decreased brain growth (acquired microcephaly), loss of coordination, gait abnormalities, developmental regression, autistic-like behaviors, seizures, and intellectual disability. Apraxia and breathing difficulties while awake are quite common. Diagnosis commonly is made at 2 to 3 years of age.\n\nPREP Pearls\n\nThe classic phenotype for fetal alcohol syndrome includes growth deficiency, midfacial hypoplasia, smooth philtrum, thin upper lip, micrognathia, short palpebral fissures, epicanthal folds, small jaw, and microcephaly.\n\nThere is no reported \"safe\" level of alcohol use during pregnancy; a fetus can be affected during any trimester.\n\nNeurologic problems in fetal alcohol syndrome include attention-deficit/hyperactivity problems, decreased impulse control, poor coordination and memory, learning disabilities (especially in math), speech and language delays, intellectual disability, impaired executive function, and sleep problems.\n\nABP Content Specifications(s)/Content Area\n\nRecognize the clinical and laboratory features associated with fetal alcohol syndrome, and manage appropriately\n\nSuggested Readings\n\nAmerican Academy of Pediatrics. Fetal alcohol spectrum disorders program. https://www.aap.org/en-us/advocacy-and-policy/aap-health-initiatives/fetal-alcohol-spectrum-disorders-toolkit/Pages/default.aspx.\n\nCenters for Disease Control and Prevention. Fetal alcohol syndrome disorders. https://www.cdc.gov/ncbddd/fasd/index.html.\n\nHagan JF Jr, Balachova T, Bertrand J, et al; on behalf of Neurobehavioral Disorder Associated With Prenatal Alcohol Exposure Workgroup, American Academy of Pediatrics. Neurobehavioral disorder associated with prenatal alcohol exposure. Pediatrics. 2016;138(4):pii: e20151553. doi: 10.1542/peds.2015-1553.\n\nWilliams JF, Smith VC, the Committee on Substance Abuse. Fetal alcohol spectrum disorders. Pediatrics. 2015;136(5):e1395-1406. doi: 10.1542/peds.2015-3113."}
{"id" : 2039, "question_text" : "A 6-year-old girl presents to the emergency department with a 16-day history of fever up to 38.3°C, malaise, headache, and fatigue. One week before this visit, she was evaluated by her pediatrician for these symptoms. A complete blood cell count and urinalysis at that time revealed normal values. Daily fevers and fatigue have persisted, and she has now developed right-sided abdominal pain. There are no sick contacts. The girl lives on a farm and has exposure to cats, dogs, horses, and cattle. She has not traveled internationally. There is no known tuberculosis exposure. Her immunizations are up to date. On physical examination, the girl is afebrile with a heart rate of 78 beats/min, respiratory rate of 16 breaths/min, and oxygen saturation of 92% on room air. She has right upper quadrant tenderness and hepatosplenomegaly. The remainder of her physical examination findings are normal. Laboratory data show an elevated platelet count and an erythrocyte sedimentation rate of 75 mm/hour. Results are otherwise normal, including white blood cell count, hemoglobin, lactate dehydrogenase, uric acid, and liver and renal function tests. Blood and urine cultures are negative. A purified protein derivative skin test is negative and her chest radiograph is normal. Abdominal ultrasonography shows multiple hypoechoic lesions in the liver and spleen. Of the following, the test MOST likely to establish this girl's diagnosis is", "options" : "[\"bone marrow biopsy\", \"liver biopsy\", \"serology\", \"stool testing for ova and parasites\"]", "explanation" : "Correct Answer: C\nThe girl described in the vignette presents with fever of unknown origin (FUO), abdominal pain, hepatosplenomegaly, elevated erythrocyte sedimentation rate, and abdominal ultrasound findings of hepatosplenic hypoechoic lesions. In the setting of contact with cats, this presentation is highly suggestive of hepatosplenic cat scratch disease (CSD). Thus, serology is the most likely test to confirm the diagnosis of disseminated CSD in this case. The etiologic agent of CSD is Bartonella henselae, a fastidious, slow-growing gram-negative bacillus. Domestic cats, especially kittens, are the primary reservoir and vector for transmission of CSD. More than 40% of cats have sustained, asymptomatic, B henselaebacteremia. The horizontal transmission of the disease from cat to cat is maintained by cat fleas, and on occasion, arthropod vectors (fleas or ticks) may transmit the disease to humans. Most cases of CSD are reported in the autumn and winter seasons. More than 90% of patients with CSD report a history of contact with cats.\n\nThe typical clinical presentation of CSD is regional lymphadenopathy/lymphadenitis, often in the absence of systemic symptoms. A primary papule may be noted at the presumed inoculation site approximately 7 to 12 days after a cat scratch, followed 7 to 50 days later (average 12-14 days) by lymphadenopathy in the drainage region for the inoculation site. Axillary nodes are most frequently affected, but other regional nodes in the cervical, submental, epitrochlear, or inguinal areas may be involved. The skin overlying the lymph nodes is often warm, tender, and erythematous. The lymphadenopathy regresses over 2 to 4 months; approximately 25% of nodes may suppurate spontaneously. Low-grade fever, malaise, anorexia, and headache may accompany regional adenopathy.\n\nLess common manifestations of CSD include prolonged fever/FUO because of disseminated disease, hepatosplenic abscesses, vertebral osteomyelitis, encephalopathy/encephalitis, conjunctivitis with preauricular adenopathy (Parinaud oculoglandular syndrome) and neuroretinitis. Immunocompromised patients may present with serious manifestations, such as bacillary angiomatosis and peliosis. Hepatosplenic CSD results from hematogenous spread of B henselae. Prolonged fever or FUO following contact with a cat or kitten is the most frequent manifestation. Other associated symptoms may include chills, malaise, headaches, myalgias, weight loss, and abdominal pain. Physical examination may reveal hepatomegaly and/or splenomegaly. Lymphadenopathy may be seen in some patients. Laboratory studies are significant for elevated acute-phase reactants and inflammatory markers, but liver enzymes are often normal. Abdominal imaging with ultrasonography or contrast-enhanced computed tomography may demonstrate multiple microabscesses in the liver and/or spleen similar to the case in the vignette. The diagnosis of CSD is best established with a serologic test, via indirect immunofluorescent assay performed at a reputed commercial laboratory or the CDC. High titers (>1:256) of immunoglobulin G antibody to B henselae are highly suggestive of CSD. In rare instances, fine-needle aspirate or tissue biopsy of an affected lymph node may reveal the organism via polymerase chain reaction. Detection of B henselae in tissue specimens using special stains, such as Warthin-Starry silver stain, is unusual. Tissue biopsy may demonstrate necrotizing granulomas similar to other granulomatous infections (eg, tuberculosis, brucellosis, tularemia).\n\nInvasive diagnostic procedures, such as liver biopsy and bone marrow biopsy, may be indicated in some cases of FUO to establish the diagnosis of unusual zoonotic infections (eg, brucellosis given exposure to livestock) or neoplastic disease. Cat scratch disease must be included in the differential diagnosis of FUO and hepatosplenic disease with a history of cat contact. The history and physical examination findings of the child in the vignette are consistent with the diagnosis of CSD, thus, serology is the most likely test to confirm the diagnosis. Her clinical presentation and abdominal imaging findings are not consistent with a parasitic disease.\n\nMost patients with CSD do not require antimicrobial therapy because the illness is self-limited in the majority of cases. Needle aspiration may be indicated to relieve pressure in painful suppurative nodes. Many oral antimicrobial agents (such as azithromycin, rifampin, trimethoprim-sulfamethoxazole, doxycycline, and parenteral gentamicin) have been used in patients with CSD with anecdotal reports of success. One randomized controlled trial found a significantly greater decrease in lymph node size in azithromycin-treated patients at 1 month compared with placebo, but there was no significant difference thereafter. Antimicrobial therapy may be considered to speed recovery in immunocompetent patients with severe manifestations of CSD, such as FUO associated with granulomatous hepatitis/hepatosplenic disease, vertebral osteomyelitis, and neuroretinitis. Antimicrobial therapy is always recommended for the treatment of CSD in immunocompromised patients.\n\nPREP Pearls\n\nThe typical clinical presentation of cat scratch disease (CSD) is regional lymphadenopathy/lymphadenitis, often in the absence of systemic symptoms.\n\nHepatosplenic CSD is an atypical manifestation commonly presenting as persistent fever or fever of unknown origin following cat or kitten contact.\n\nThe laboratory diagnosis of CSD is best established with a serologic test via indirect immunofluorescent assay.\n\nABP Content Specifications(s)/Content Area\n\nPlan appropriate management for a patient with cat-scratch disease\n\nRecognize the clinical features associated with cat-scratch disease\n\nUnderstand the epidemiology of cat-scratch disease\n\nSuggested Readings\n\nAmerican Academy of Pediatrics. Bartonella henselae (cat-scratch disease). In: Kimberlin DW, Brady MT, Jackson MA, Long SS, eds. Red Book: 2018 Report of the Committee on Infectious Diseases. 31st ed. Itasca, IL: American Academy of Pediatrics; 2018:244-247.\n\nArisoy ES, Correa AG, Wagner ML, Kaplan SL. Hepatosplenic cat-scratch disease in children: selected clinical features and treatment. Clin Infect Dis. 1999;28(4):778-784. doi: 10.1086/515197.\n\nFlorin TA, Zaoutis TE, Zaoutis LB. Beyond cat scratch disease: widening spectrum of Bartonella henselae infection. Pediatrics. 2008;121(5):e1413-e1425. doi: 10.1542/peds.2007-1897."}
{"id" : 210, "question_text" : "You are examining a 1-day-old term male infant during rounds in the newborn nursery and palpate a right upper quadrant abdominal mass. The remainder of the examination findings are normal. The infant's mother is an otherwise healthy 22-year-old gravida 1, para 1, woman who received no prenatal care. You note on the chart that the infant voided at 6 hours of age. Of the following, the MOST likely diagnosis in this infant is", "options" : "[\"posterior urethral valves\", \"ureterocele\", \"ureteropelvic junction obstruction\", \"urolithiasis\", \"Wilms tumor\"]", "explanation" : "The differential diagnosis of an infant who has a palpable abdominal mass begins with underlying renal causes. The most common causes of abdominal masses in the newborn period are multicystic dysplastic kidney (MCDK) and hydronephrosis due to ureteropelvic junction (UPJ) obstruction, which account for a combined 40% of all causes. Other, less common renal causes include polycystic kidney disease, renal vein thrombosis, and renal tumors (such as mesoblastic nephroma). Among the nonrenal causes of abdominal masses are gastrointestinal anomalies (duplications or obstruction) and ovarian cysts.\n\nThe abdominal mass described for the infant in the vignette most likely is due to UPJ obstruction (although MCDK would be equally likely). Posterior urethral valves are less common and should have bilateral involvement. Ureterocele could be unilateral but is less common. Urolithiasis can cause unilateral hydronephrosis but is extremely unlikely in the newborn. Wilms tumor is rare in newborns; the most common newborn renal/adrenal tumor is mesoblastic nephroma, followed by neuroblastoma or a teratoma.\n\nHydronephrosis often is related to obstruction or vesicoureteral reflux. Causes of obstruction include UPJ obstruction, ureterovesical junction obstruction, ureterocele, bladder outlet obstruction from posterior urethral valves, or functional obstruction from a neurogenic bladder. The most common cause of hydronephrosis is UPJ obstruction, which is usually unilateral and presents as hydronephrosis in utero or a palpable abdominal mass in the nursery. It is customary to perform voiding cystourethrography (VCUG) in patients who have unilateral involvement to look for possible reflux into the contralateral (seemingly uninvolved) side. Prophylactic antibiotics are recommended in cases of severe hydronephrosis because urinary stasis can predispose to urinary tract infections. A radionuclide test (MAG 3) with furosemide is recommended to measure the half-life of the radioisotope within the collecting system and determine the differential contribution to total renal function (eg, 60% contribution from uninvolved side and 40% from involved side). Delayed clearance from the upper urinary tract is consistent with UPJ obstruction (Item C102). Referral to a pediatric urologist is recommended to determine the potential need for surgical intervention. Typically, patients who have mild involvement are managed conservatively, and those whose involved kidney is contributing less than 35% to total renal function are treated surgically with a pyeloplasty. Of note, male infants who have bilateral hydronephrosis warrant urgent VCUG to rule out posterior urethral valves that require more aggressive intervention.\n\nMCDK is a nonfunctioning kidney, which occurs in an estimated 1 in 2,500 births. Bilateral involvement is incompatible with life. Ultrasonography shows multiple cysts of varying size that do not communicate and a small amount of abnormal renal parenchyma adjacent to the cysts. Distinguishing MCDK from severe hydronephrosis can be a challenge. Unlike severe hydronephrosis, the cystic lesions do not communicate and are not medially located in MCDK. In addition to the absence of renal function on the involved side of the MCDK, the contralateral kidney may be affected with vesicoureteral reflux in 30% of cases and UPJ obstruction in 15% of cases.\n\nThe natural history of MCDK is spontaneous involution of the involved side in approximately 60% of cases by 6 years of age. There is a very small likelihood of malignant potential in MCDK, including Wilms tumor in younger children and renal cell carcinoma in adolescents. Due to the high risk of vesicoureteral reflux, when MCDK is diagnosed in the neonatal period, prophylactic antibiotics should be started (10 mg/kg per day of amoxicillin) to protect the contralateral, single functional kidney from pyelonephritis. Due to the malignant potential in the involved kidney, it is standard to obtain ultrasonography every 3 to 6 months during the first postnatal year, then every 6 to 12 months until age 5. This strategy allows monitoring of the growth of the normal kidney, which often exhibits compensatory hypertrophy (because it is performing the work of two kidneys), as well as the MCDK for signs of involution or unexpected growth. In the event of unexpected growth, referral to a pediatric urologist for consideration of a nephrectomy of the MCDK is recommended to eliminate concerns of malignant transformation.\n\nContent Specifications: Know the differential diagnosis of urinary tract obstruction"}
{"id" : 1659, "question_text" : "A 10-year-old boy is brought to your office by his parents for concerns about learning. He started to struggle with his schoolwork last year, but received passing grades. This year, he has been saying that he is \"stupid\" and crying when his parents tell him it is time to leave for school. His parents requested and received an educational evaluation through his school. They have brought a copy of his school assessment to their appointment, and ask for your help in understanding the results, in preparation for their meeting at his school next week. His aptitude test scores are as follows: •verbal IQ = 85 •performance IQ = 78 •full-scale IQ = 83 His academic achievement scores are as follows: •reading = 65 •math = 75 •writing = 68 Of the following, the MOST likely diagnosis is", "options" : "[\"a learning disability in math\", \"a learning disability in reading and writing\", \"a learning disability in reading, writing, and math\", \"mild intellectual disability\", \"no learning disability\"]", "explanation" : "Correct Answer: B\nPsychoeducational tests such as IQ tests and achievement tests generally have a mean of 100 and standard deviation (SD) of 15. The average range is within 1 SD of the mean (85–115). Verbal IQ measures language-based aptitude; performance IQ measures non–language-based aptitude. One definition of a learning disability is a meaningful discrepancy between intelligence (aptitude) scores and achievement scores. A discrepancy of at least 1 SD is significant. The boy in the vignette has a greater than 1 SD discrepancy between both his verbal IQ and his reading achievement, and his verbal IQ and his writing achievement. There is no significant discrepancy between his performance IQ and his math achievement. Therefore, this boy most likely has a learning disability in reading and in writing, but not in math. He does not meet the criteria for intellectual disability, which requires IQ scores that are 2 or more SDs below the mean.\n\nAcademic underachievement affects approximately 20% of school-aged children. Children with academic problems should be evaluated for learning or intellectual disability. Psychoeducational assessments should be requested through the school district, and typically include both measures of intelligence and achievement to help determine the etiology of academic underperformance. Intelligence includes the capacity to reason, plan, solve problems, think abstractly, learn, and use appropriate judgment. These cognitive abilities (aptitude) are measured using standardized IQ tests. The Wechsler intelligence scales (eg, Wechsler Preschool and Primary Scale of Intelligence [2.5–7.5 years], Wechsler Intelligence Scale for Children [6–16 years], Wechsler Adult Intelligence Scale [16–90 years]), Kaufman Assessment Battery for Children Second Edition (3–18 years), and Differential Ability Scales-II (2.5–17 years) are commonly used IQ tests. IQ tests can evaluate verbal and nonverbal abilities, and may include assessment of visuospatial abilities, working memory, knowledge, processing speed, and problem-solving skills.\n\nAchievement, or academic proficiency, is measured using standardized tests that assess strengths and weaknesses in reading, math, written language, and oral language. Commonly used individually administered achievement tests include the Kaufman Test of Educational Achievement-II (Comprehensive Form 4.5–25 years; Brief Form 4.5–90 years), Wechsler Individual Achievement Test-III (4–50 years), Wide Range Achievement Test-4 (5–94 years), and the Woodcock-Johnson IV Tests of Achievement (2–90 years).\n\nStandardized tests compare the individual's performance to that of same-age peers. Scores greater than 2 SD below the mean (<70) in both cognitive and adaptive measures are in the intellectually disabled range. Verbal IQ scores measure language-based aptitude and performance IQ scores measure non–language-based aptitude. Achievement scores are generally within 1 SD (15 points) of cognitive scores. Depending on the state, a discrepancy of at least 1 or 2 SD between IQ and achievement test scores meets the traditional educational definition of a learning disability. A learning disability may also be defined by low achievement in the setting of at least low average intelligence. In addition, the 2004 reauthorization of the Individuals with Disabilities Education Act (IDEA) allows learning disability to be defined by a student's failure to respond to evidence-based educational interventions.\n\nPediatricians who can interpret the results of psychoeducational tests can better understand the needs of their patients and more effectively guide families advocating for appropriate educational services. Early identification of and intervention for learning or intellectual disabilities improves the educational outcomes for these children.\n\nPREP Pearls\n• Psychoeducational tests, such as IQ tests and achievement tests, generally have a mean score of 100 and standard deviation (SD) of 15. The average range is within 1 SD of the mean (85–115).\n• IQ test scores greater than 2 SD below the mean (<70) in both cognitive and adaptive measures are in the intellectually disabled range.\n• A learning disability may be defined as a meaningful discrepancy between intelligence scores and achievement scores, low achievement in the setting of at least low average intelligence, or a student's failure to respond to evidence-based educational interventions.\n\nABP Content Specifications(s)\n• Interpret the results of intelligence quotient tests, with emphasis on understanding the normal ranges\n• Plan the appropriate diagnostic evaluation of achievement and intelligence\n\nSuggested Readings\n• Aylward GP. Psychoeducational testing. In: Voigt RG, Macias MM, Myers SM, eds. Developmental and Behavioral Pediatrics. Elk Grove Village, IL: American Academy of Pediatrics; 2011:293–311.\n• Braaten EB, Norman D. Intelligence (IQ) testing. Pediatr Rev. 2006;27(11):403–408. doi: http://pedsinreview.aappublications.org/content/27/11/403 .\n• Rimrodt SL, Lipkin PH. Learning disabilities and school failure. Pediatr Rev. 2011;32(8):315–324. doi: http://dx.doi.org/10.1542/pir.32-8-315."}
{"id" : 3392, "question_text" : "A 15-year-old girl sustains a severe traumatic brain injury in a snowboarding accident. She is transported to a pediatric intensive care unit. After 3 days, her parents are told she has severe brain injury and is unlikely to recover meaningful neurologic function. Her neurologic examination shows sluggishly reactive pupils, absent corneal reflexes, and absent gag reflex. She takes intermittent spontaneous breaths but depends on the ventilator to maintain respiratory function. Her parents are considering discontinuing mechanical ventilation because of the severity of her brain injury and poor prognosis. They ask if it is possible to donate her organs in this situation. Of the following, the BEST response to the parents' question is that", "options" : "[\"brain death criteria are met and so she could be an organ donor\", \"brain death criteria are not met and so she cannot be an organ donor\", \"if cardiac death does not occur, she could be an organ donor\", \"if cardiac death occurs, she could be an organ donor\", \"organ donation requires both cardiac and brain death criteria be met\"]", "explanation" : "Preferred Response: D\nWhile the girl in the vignette is in a coma, her physical examination shows the presence of some brainstem reflexes, so she does not meet criteria for brain death. If ventilator support were discontinued and she was unable to continue effective ventilation independently, and she had a cardiac arrest and died, her organs could be donated. This is referred to as donation after cardiac death.\n\nOrgan donation after cardiac death occurs in a hospital setting, where organs can be surgically recovered as soon as possible after cardiac death and cessation of organ perfusion. It is inevitable that brain death criteria will be met after cardiac death occurs, therefore it is not necessary to perform a brain death examination in that situation.\n\nPediatric brain death criteria have been established and updated as recently as 2012. There has to be a known and irreversible cause of coma, absence of hypotension, hypothermia, metabolic disturbances, and sedating effects of medication. Two brain death examinations by different examiners, done 12 to 24 hours apart, must confirm the absence of brainstem reflexes including the reflex to breathe. Ancillary studies, such as cerebral blood flow studies or electroencephalography, are not necessary but can be used, especially in circumstances where the examination cannot be performed safely.\n\nIf neither brain death nor cardiac death has occurred, then the person cannot be an organ donor. This can have severe emotional consequences for families already devastated by the death of their child. When severe brain injury has occurred, but brain death has not, and the person does not recover significantly in 4 weeks, the patient can be described as in a persistent vegetative state. In this state, often wake and sleep cycles occur, with spontaneous eye opening and closure and even spontaneous swallowing. The person may be able to visually track, cry, or vocalize. However, they do not have purposeful interactions or meaningful communication. Case reports have described brief episodes of recovery from persistent vegetative state, but in the vast majority of cases, significant neurologic function does not return. Family members sometimes differ in their wishes for their child who is in a persistent vegetative state. Adolescents may have voiced their own desires before their injury and these should be taken into account in decision making. Consultation with an ethics committee is often very helpful.\n\nPREP Pearls\n• Organ donation can occur after either brain death or cardiac death.\n• Two brain death examinations by different examiners, performed 12 to 24 hours apart, are necessary to confirm brain death.\n\nAmerican Board of Pediatrics Content Specification(s)\n• Recognize and apply ethical principles surrounding the issue of brain death\n\nSuggested Reading\nAntommaria, AHM. Ethics for the pediatrician: conceptual and ethical issues in the declaration of death. Pediatr Rev. 2010;31(10);427-430. doi:10.1542/pir.31-10-427.\n7-430. doi:10.1542/pir.31-10-427.\nNakagawa TA, Ashwal S, Mathur M, Mysore M. Guidelines for the determination of brain death in infants and children: an update of the 1987 task force recommendations—executive summary. Ann Neural. 2012;71(4):573-585. doi:10.1002/ana.23552."}
{"id" : 1521, "question_text" : "A 40-year-old pregnant nurse presents to the occupational health clinic for annual influenza vaccination. She works at a teaching hospital and has recently been assigned to the bone marrow transplant unit. She has a history of asthma and has had hives when she consumes eggs. She inquires about appropriate influenza prevention. Of the following, the MOST appropriate preventative measure for this nurse is", "options" : "[\"amantadine orally during influenza season\", \"inactivated influenza vaccine\", \"live attenuated influenza vaccine\", \"no immunizations or oral prophylaxis\", \"oseltamivir orally during influenza season\"]", "explanation" : "The most appropriate preventative measure for the nurse in the vignette is inactivated influenza vaccine. Immunization is the best preventative measure against influenza.\n\nAs a matter of patient safety, the American Academy of Pediatrics recommends mandatory influenza vaccination for all healthcare providers. Vaccination rates need to reach at least 90% in healthcare personnel in order to prevent healthcare-associated influenza infections. Voluntary vaccination programs fail to achieve such rates, thus necessitating the recommendation for mandatory programs.\n\nThe nurse in the vignette has 3 contraindications to live attenuated influenza vaccine (LAIV): pregnancy, asthma, and egg allergy. Of note, her egg allergy alone does not preclude her from receiving inactivated influenza vaccine because her reaction is mild. For severe (anaphylactic) reactions to eggs, consultation with an allergist prior to vaccination with inactivated vaccine is recommended. Despite her inability to receive LAIV, she remains eligible for inactivated influenza vaccine.\n\nWhile antivirals, including oseltamivir, are recommended for chemoprophylaxis in outbreak settings for certain high-risk groups, they are not recommended as a substitute for vaccination. Furthermore, amantadine is no longer recommended for influenza infections for 2 reasons: high levels of resistance against the adamantanes in influenza A viruses and lack of activity against influenza B viruses.\n\nPREP Pearls\n• Immunization is the best preventative measure against influenza.\n• Mild allergic reactions to eggs are not considered a contraindication to vaccination with inactivated influenza vaccine.\n• Antivirals are not recommended as a substitute for vaccination.\n\nABP Content Specifications(s)\n• Recognize the importance of annual influenza immunizations for medical office and hospital personnel and medical staff\n\nSuggested Readings\n• American Academy of Pediatrics. Influenza. In: Kimberlin DW, Brady MT, Jackson MA, Long SS, eds. Red Book: 2015 Report Of The Committee On Infectious Diseases. 30th ed. Elk Grove Village, IL: American Academy of Pediatrics; 2015:476-493.\n• Committee on Infectious Diseases. Influenza immunization for all health care personnel: keep it mandatory. Pediatrics. 2015;136(4):809-818. doi: http://dx.doi.org/10.1542/peds.2015-2922.\n• Committee on Infectious Diseases. Recommendations for prevention and control of influenza in children, 2015-2016. Pediatrics. 2015;134(4):1-17. doi: http://dx.doi.org/10.1542/peds.2015-2920."}
{"id" : 3157, "question_text" : "A 5-year-old girl was admitted from the emergency department because of fever and lethargy. She was recently diagnosed with acute lymphoblastic leukemia and is in the middle of her induction cycle of chemotherapy. She was tachycardic and hypotensive while in the emergency department and received a total of 100 mL/kg of 0.9% saline in boluses. She also was started on a dopamine drip of 5 µg/kg Per min. Blood cultures were drawn, and she was started on empiric antimicrobial therapy, including vancomycin and cefepime. Due to respiratory distress and hypoxemia, she required intubation and mechanical ventilation. Within 24 hours, her blood cultures from both peripheral venipuncture and her indwelling central venous catheter have grown gram-negative bacilli. She is now on relatively high ventilator settings, and her arterial blood gas has a pH of 7.30, PCO2 of 50 mm Hg, and PO2 of 80 mm Hg on FiO2 of 0.60. Vital signs show a temperature of 38.5°C, heart rate of 130 beats/min, respiratory rate of 30 breaths/min, blood pressure of 90/30 mm Hg, and SpO2 of 94%. Physical examination shows an intubated and sedated girl that awakens to stimuli, with no focal neurologic findings. Cardiovascular examination shows tachycardia with regular rhythm. She has warm extremities, with capillary refill less than 1 second. Lungs have scattered crackles and decreased air movement bilaterally. Abdomen is soft, nontender, and nondistended with no palpable spleen or liver. A chest radiograph is shown in Item Q118. Of the following, the MOST likely cause of her respiratory failure is", "options" : "[\"acute respiratory distress syndrome\", \"congestive heart failure\", \"pulmonary embolism\", \"pulmonary hemorrhage\", \"pulmonary hypertension\"]", "explanation" : "Preferred Response: A\nThe child in this vignette, who has bilateral infiltrates on chest radiography, a PaO2/FiO2 ratio of 125, and a noncardiogenic cause of respiratory failure in the setting of sepsis, most likely has acute respiratory distress syndrome (ARDS). The pathogenesis of ARDS in this patient is increased pulmonary capillary permeability because of sepsis, septic shock, or systemic inflammatory response syndrome.\n\nAcute respiratory distress and failure caused by ARDS occurs in both adults and children, and is a serious cause of morbidity and mortality. According to the 1994 American-European Consensus Conference on ARDS, diagnostic criteria include respiratory failure, a ratio of arterial oxygen tension to fraction of inspired oxygen (PaO2/FiO2) of less than 200, bilateral pulmonary infiltrates on chest radiographs, and a noncardiac cause of pulmonary edema. The term acute lung injury (ALI) indicates patients with a PaO2/FiO2 ratio between 200 and 300.\n\nAn important event in the pathogenesis of ARDS is the breakdown of the alveolar-pulmonary capillary barrier, which leads to the leakage of proteinaceous fluid into the airspaces. This causes decreased lung compliance, alveolar collapse, and disordered gas exchange marked by ventilation-perfusion mismatch. Patients often breathe rapidly and shallowly and become hypoxic because of decreased compliance and intrapulmonary shunting. Surfactant production and function is also impaired by alveolar type II cell injury, which leads to further alveolar collapse. The repetitive opening and closing of lung units in the face of increased surface tension exacerbates the inflammatory cascade, leading to the secretion of proinflammatory cytokines, such as tumor necrosis factor a and interleukins, which exacerbates the cycle by increasing capillary permeability.\n\nAcute respiratory distress syndrome can be caused by direct lung injury from pneumonia, ventilator-induced lung injury, chest trauma, aspiration pneumonitis, acute chest syndrome in sickle cell disease, or indirectly from systemic causes such as sepsis, transfusion-related lung injury, pancreatitis, trauma, or systemic inflammation from other numerous causes. Landmark investigations performed by the ARDS Research Network have led to the widespread acceptance of the \"open lung\" strategy of ventilator management in ARDS. The aim is to protect the lungs from further injury by maintaining alveolar recruitment with high positive end-expiratory pressure and by limiting inflation pressures and FiO2. The clinician targets lower tidal volumes of 4 to 6 mL/kg and accepts higher level of partial pressure of carbon dioxide (PCO2), which is a strategy known as \"permissive hypercapnia.\"\n\nAlthough congestive heart failure is common in children with leukemia, it is unlikely in this patient because she has received 100 mL/kg in fluid resuscitation and has no palpable spleen or liver. Pulmonary embolism is rare in this age group and is not associated with sepsis, which is the acute condition of this patient. Pulmonary hemorrhage can occur in patients who have insufficient clotting from bone marrow suppression, but bleeding severe enough to cause respiratory failure usually coincides with hemoptysis or the presence of blood in the endotracheal tube. Pulmonary hypertension can cause hypoxia, but is not associated with sepsis, nor is it a common cause of respiratory failure in children with leukemia.\n\nAcute respiratory distress syndrome is a condition of respiratory failure in adults and children marked by decreased lung compliance and hypoxia from ventilation-perfusion mismatch and intrapulmonary shunting. It should be treated with the evidence-based guidelines of lung recruitment with positive end-expiratory pressure, and limitation of tidal volume, inflation pressures, and FiO2.\n\nPREP Pearls\n• Acute respiratory distress syndrome can be caused by direct lung injury or indirectly from nonpulmonary inflammatory conditions.\n• Acute respiratory distress syndrome is associated with pulmonary alveolar-capillary damage, decreased surfactant production and function, and proinflammatory cytokines.\n• Acute respiratory distress syndrome should be treated to protect the lungs, minimizing inflation pressures and oxygen toxicity, and accepting higher PCO2.\n\nAmerican Board of Pediatrics Content Specification(s)\n• Recognize the clinical features of acute respiratory distress syndrome, including associated sequelae\n\nSuggested Reading\n• Carlo WA, Ambalavanan N. Conventional ventilation: traditional and new strategies. Pediatr Rev. 1999;20(12):e117-e126. doi:10.1542/pir.20-12-e117. Dauger S, Durand P, Javouey E, Mercier JC. Acute respiratory distress syndrome in children. In: Fuhrman BP, Zimmerman JJ. eds. Pediatric Critical Care. 4th ed. Philadelphia, PA: Saunders Elsevier: 2011:212-218.\n• Fiser DH. Adult respiratory distress syndrome. Pediatr Rev. 1993;14(5):163166. doi:10.1542/pir.14-5-163.\n• Nitu ME, Eisen H. Respiratory failure. Pediatr Rev. 2009;30(12):470-478. doi:10.1542/pir.32-6-240."}
{"id" : 3264, "question_text" : "A 10-year-old girl presents for her routine health maintenance visit. Upon further questioning, the mother admits that the patient always snores at night and that the snoring has gotten worse this last year. She has been treated twice for throat infections this year and the mother has been told her daughter's tonsils are large. They have tried allergy medication episodically without much improvement. On physical examination, you notice darkening under the eyes, pale and large nasal turbinates, plus adenoidal facies. The tonsils are hypertrophied and the left tonsil is significantly larger than the right. Of the following, the BEST next step in management is", "options" : "[\"prescribe a nasal steroid for daily use\", \"prescribe an oral antihistamine for daily use\", \"reassure that the tonsils will get smaller with age\", \"refer for polysomnography\", \"referral to otolaryngology\"]", "explanation" : "Preferred Response: E\nAlthough excision of the tonsils and adenoids of children and adolescents has been a controversial subject for many years, there are some absolute indications for surgery. The girl in the vignette is an example of one of those indications. Significant asymmetry without signs of acute infection of the tonsils on examination should raise suspicion for malignancy, especially if the asymmetry develops rapidly. An immediate referral to otolaryngology is needed for further evaluation. Other absolute indications for surgical excision include extreme obstruction of the airway, interference with swallowing, and uncontrollable hemorrhage from tonsillar blood vessels.\n\nOther conditions that lead to the consideration of tonsillectomy or adenoidectomy include recurrent acute or chronic tonsillitis or sinusitis, recurrent acute otitis media or chronic otitis media with effusion, alteration of voice quality because of adenotonsillar hypertrophy, refractory halitosis, peritonsillar abscess, or syndrome of periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis (PFAPA syndrome). The decision to perform elective tonsillectomy or adenoidectomy should be individualized according to potential risks and benefits for each patient.\n\nThe use of daily nasal corticosteroids is considered first-line therapy for several mucosal etiologies of nasal obstruction (eg, rhinitis, nasal polyposis) and may be effective in cases of adenoidal hypertrophy. Those who respond inadequately to pharmacologic therapy should be referred to otolaryngology for evaluation and possible surgical management. Oral antihistamines can also be helpful in patients with persistent allergic rhinitis as the cause of their nasal obstruction.\n\nAdenotonsillar hypertrophy is most pronounced between 3 and 8 years of age and is usually associated with acute or chronic infections. These structures tend to involute in most children after about 10 years of age because the immunologic activity of this lymphoid tissue decreases after puberty. Therefore, snoring and sleep disordered breathing should resolve with time, rather than worsen as in this patient. Plus, one should not simply reassure the family, given the asymmetry of the tonsillar tissue in the girl in this vignette.\n\nAdenotonsillectomy is suggested for healthy children who have adenotonsillar hypertrophy and obstructive sleep apnea. It is not recommended for patients who have hypertrophy without symptoms. Although uncommon, those with chronic airway obstruction from enlarged tonsils and adenoids may develop cor pulmonale or adenoidal facies (elongation and flattening of the midface and retrognathic mandible), so evaluation is important. Tonsil and adenoid size is not predictive of the presence or severity of obstructive sleep apnea, so polysomnography should be performed. Polysomnography is considered the gold standard for assessing suspected obstructive sleep apnea, and referral for this examination before otolaryngology consultation would be appropriate if the concern for malignancy (asymmetric tonsils) was not present.\n\nPREP Pearls\n• Significant asymmetry of the tonsils without signs of acute infection should raise suspicion for malignancy and warrants prompt referral to otolaryngology.\n• Adenotonsillar hypertrophy is most pronounced between 3 and 8 years of age and is usually associated with acute or chronic infections.\n• Involution of tonsillar and adenoidal lymphoid tissue occurs in most children around the time of puberty.\n• Polysomnography is the gold standard for assessing suspected obstructive sleep apnea.\n\nAmerican Board of Pediatrics Content Specification(s)\n• Recognize conditions associated with tonsillar and/or adenoidal hyper trophy\n\nSuggested Reading\n• Bhattacharyya N. Clinical presentation, diagnosis, and treatment of nasal obstruction. UpToDate. Available online only for subscription.\n• Isaacson G. Tonsillectomy care for the pediatrician. Pediatrics. 2012;130(2)324-334. doi:10.1542/peds.2011-3857.\n• Marcus CL, Brooks LI, Draper KA, et al. Diagnosis and management of childhood obstructive sleep apnea syndrome. Pediatrics. 2012;130(3):576584. doi:10.1542/peds.2012-1671.\n• Paradise IL. Tonsillectomy and adenoidectomy in children. UpToDate. Available online only for subscription.\n• Paruthi S. Management of obstructive sleep apnea in children. UpToDate. Available online only for subscription.\n• Wetmore RF. Tonsils and adenoids. In: Kliegman RM, Stanton BE, St. Geme IW III, Schor NF, Behrman RE, eds. Nelson Textbook of Pediatrics. 19th ed. Philadelphia, PA: Saunders Elsevier; 2011:1442-1445."}
{"id" : 2251, "question_text" : "A 7-year-old girl with a 3-year history of intermittent cough, wheezing with viral respiratory infections, and repeated episodes of respiratory distress with low oxygen saturation is being evaluated. Each time she is ill, she has been treated with bronchodilators and oral corticosteroids. Inhaled corticosteroids have been prescribed as an asthma controller medication, but her parents have not given the medication consistently. Today the girl's parents report that she has a 5-day history of worsening cough and nasal congestion, but no fever or other somatic symptoms. Her parents have not heard her wheeze, but they do sense that she is having difficulty breathing. On physical examination, she is afebrile with a room air oxygen saturation of 92% on pulse oximetry, heart rate of 130 beats/min, and blood pressure of 100/70 mm Hg. Her mucous membranes are moist and there is good skin turgor. Moderate intercostal and suprasternal retractions are seen, with generally diminished breath sounds and diffuse end-expiratory wheezes. No focal adventitious sounds can be heard in the chest. After treatment with albuterol 2.5 mg with wet nebulization, she seems a bit more comfortable, but her oxygen saturation is not improved, wheezing is not resolved, and she still has retractions. Chest radiography is performed (Figure). Of the following, the MOST appropriate next management step is", "options" : "[\"endotracheal intubation and admission to the intensive care unit\", \"hospital admission for frequent inhaled bronchodilators and systemic corticosteroids\", \"outpatient management with antibiotics with next-day follow-up\", \"referral for urgent bronchoscopy and bronchoalveolar lavage\"]", "explanation" : "The girl in the vignette has an asthma exacerbation with incomplete response to inhaled bronchodilators. The most appropriate intervention for this girl is hospital admission for aggressive bronchodilator treatment and systemic steroids. She merits close observation for worsening of her clinical status despite intervention. While the findings of hyperinflation, peribronchial cuffing, and right middle lobe atelectasis seen on her chest radiographs are often misdiagnosed as pneumonia, this girl has no fever or other somatic symptoms to suggest infection. Although she does have mild respiratory distress, there is no indication of impending respiratory failure or need for intubation at this point. Acute bronchoscopy would be indicated if there were concern for foreign body aspiration, but her age and the event history do not support this as a cause of her symptoms. Mucus plugging with atelectasis may be a component of severe asthma and is appropriately addressed with attention to the asthma itself. In this situation, chest physical therapy may be beneficial.\nThe child in this vignette likely has moderate to severe asthma that has not been well managed. The history of frequent exacerbations with viral infections and need for oral steroids suggests that she is at risk for severe exacerbation. Lack of wheezing between episodes does not guarantee normal lung function. It is entirely likely that she has a current baseline of impaired airflow which would be demonstrated as decreased FEV1 on pulmonary function testing. A normal physical examination and lack of symptoms between episodes of illness can give a false indication of intermittent asthma and lead to under-recognition of severity. Per the 2020 Focused Update to the Asthma Management Guidelines, this child would be a candidate for either: 1) daily (plus as needed) use of a low to moderate dose inhaled corticosteroid with formoterol, or 2) daily medium-dose inhaled steroid use, with or without long-acting bronchodilator, plus a short-acting bronchodilator as needed. Daily asthma controller therapy should be considered for any child needing repeat courses of systemic steroids to manage episodes of wheezing. The best treatment of persistent asthma is appropriate daily controller therapy plus recognition of triggers, addressing those that can be ameliorated.\nSuggested Reading(s)\nClourier MM, SJ Teach, RF Lemanske, KV Blake. The 2020 focused updates to the NIH asthma management guidelines: key points for pediatricians. Pediatrics. 2021;147(6):e2021050286. \ndoi:10.1542/peds.2021-050286\nDinakar C. Asthma. Javed A, Schwenk WF II, Tebben P. Diabetes mellitus. In: McInerny TK, Adam HM, Campbell DE, DeWitt TG, Foy JM, Kamat DM, eds. American Academy of Pediatrics Textbook of Pediatric Care. American Academy of Pediatrics; 2021:chap 218. Pediatric Care Online\nContent Domain\nPulmonology\nLearning Objectives\nPlan  the treatment for a child with persistent asthma\nList triggers for persistent asthma"}
{"id" : 3209, "question_text" : "A 10-year-old girl presents for poor linear growth and slow weight gain. In the last year, her linear growth has stopped completely, and she continues to struggle to gain weight. Review of systems reveals frequent fatigue, anorexia, and a decreased energy level. She has also had occasional episodes of emesis in the morning that are becoming more frequent. Physical examination shows some darkening of her skin and mucous membranes. The remainder of her examination is unremarkable. Initial laboratory testing shows a serum sodium concentration of 131 mEq/L (131 mmol/L), potassium concentration of 5.8 mEq/L (5.8 mmol/L), and a glucose level of 60 mg/dL (3.3 mmol/L). The rest of her chemistry results are unremarkable. Complete blood cell count shows eosinophilia. Of the following, the BEST next test to determine her underlying diagnosis is", "options" : "[\"insulin like growth factor 1\", \"karyotype\", \"serum cortisol\", \"thyrotropin\", \"tissue transglutaminase antibody\"]", "explanation" : "Preferred Response: C\nThe child in the vignette has Addison disease, the most common cause of adrenal insufficiency in the industrialized world. Classic chronic symptoms include weakness, fatigue, anorexia, nausea, abdominal pain, and diarrhea. Poor growth can also occur, as seen in this child. Hyperpigmentation of the skin and mucous membranes can develop over time. Acute symptoms can include muscle, joint and abdominal pain, and hypotension.\n\nLaboratory evaluation may reveal hyponatremia, hyperkalemia, and hypoglycemia if the child becomes ill and develops an adrenal crisis. Sometimes the complete blood cell count (CBC) will reveal anemia and eosinophilia as well. Serum cortisol will be very low, revealing the diagnosis of adrenal insufficiency. Other supportive testing includes elevated adrenocorticotropic hormone (ACTH) levels (frequently > 100 pg/mL [22 pmol/L]), and ACTH stimulation testing can reveal a low cortisol response (< 18 µg/dL [497 nmol/L]). Mineralocorticoid deficiency is confirmed with a relatively low aldosterone value in the face of hyperreninemia. Adrenal antibodies can also be measured to confirm the diagnosis of Addison disease as the underlying cause of adrenal insufficiency.\n\nOther causes of poor growth include growth hormone deficiency, hypothyroidism, and Turner syndrome. Although insulin like growth factor 1, thyrotropin, and karyotype testing are reasonable in the evaluation of a child with poor growth, the additional symptoms shown by the child in this vignette, along with her hyperpigmentation and laboratory findings, make these diagnoses much less likely.\n\nSimilarly, tissue transglutaminase testing for celiac disease is warranted in the routine evaluation of a child with poor growth, especially with gastrointestinal symptoms. However, for girl in the vignette, her severe symptoms and classic laboratory findings fit much better with adrenal insufficiency as the most likely diagnosis.\n\nPREP Pearls\n• Most children who have Addison disease experience a constellation of ill-defined symptoms to include fatigue, generalized muscular weakness, loss of appetite, and poor weight gain. A high index of suspicion is needed to diagnose Addison disease.\n• A low serum cortisol measurement at the time of acute illness will confirm the diagnosis of adrenal insufficiency.\n\nAmerican Board of Pediatrics Content Specification(s)\n• Recognize the clinical features associated with Addison disease\n• Recognize the clinical and laboratory manifestations of adrenal insufficiency\n\nSuggested Reading\n• Antal Z, Zhou P. Addison disease. Pediatr Rev. 2009;30(12):491-493. doi:10.1542/pir.30-12-491.\n• Loraux DL. Adrenocortical insufficiency. In: Becker KL, ed. Principles and Practice of Endocrinology and Metabolism. 3rd ed. Hagerstown, MD: Lippincott Williams & Wilkins; 2001:739-742."}
{"id" : 3120, "question_text" : "You are seeing a 17-year-old adolescent girl in your office for follow-up of her asthma that now requires daily medications. She has not been adhering to her treatment regimen. She has maintained her school grades and has a clear understanding of her illness. She says she is not depressed, but does admit to being stressed with homework and college applications. You consider ways to help her comply with the treatment plans. Of the following, the intervention MOST likely to be successful for this patient is to", "options" : "[\"encourage religiosity and church attendance\", \"enroll her in a support group\", \"have her mother supervise her treatment plan\", \"mark a calendar after each dose taken\", \"set her cellphone alarm to medication use times\"]", "explanation" : "Nonadherence to a therapeutic plan is the commonest reason for failure of therapy. This is an especially big issue with an adolescent patient. They may misunderstand the instructions, forget, or choose to ignore advice and the therapeutic plan. Adherence is influenced by a number of factors including the following: attitude and beliefs about the condition being treated (eg, the severity of the condition, benefits, and adverse effects of treatment), the complexity of the regimen, perception of the ability to follow through on the plan, availability of social support, the cultural context, and emotional status, particularly if the patient is depressed. In addition, the relationship with the physician is very important, because it enables the development of a mutually agreed upon therapeutic plan.\n\nCollaborating on a plan will work better than dictating the plan and using aids that the patient is comfortable with are crucial. Most adolescents are comfortable with technology. The use of the alarm on her cellphone, for the girl in the vignette, as a cue to take medications would enhance her self-efficacy and thus her motivation to adhere with the plan. The use of a calendar is less helpful, as most adolescents do better with an active reminder. Religiosity and church attendance are a reflection of a conservative outlook and indicate that a teenager is less likely to be rebellious, but this alone does not indicate she will adhere to a regimen. No single intervention will work with all patients. Social support can also be helpful. This includes support from a group or a parent. The involvement of a parent is very important, but an adolescent who will soon be on her own needs help in achieving her independence, rather than remaining dependent on a parent as the only means to enhance adherence.\n\nPREP Pearls\n• The commonest reason for failure of therapy is lack of adherence\n• An understanding of the many factors that influence adherence is crucial.\n• Using technology to create reminders has been shown to be effective in improving adherence.\n\nAmerican Board of Pediatrics Content Specification(s)\n• Understand factors that can affect adherence to health maintenance activities by adolescents\n• Understand how to improve adherence to medical regimens by adolescent patients, including those with chronic illness, and the barriers to such adherence\n\nSuggested Reading\n• Arrington-Sanders R. Adherence. Pediatr Rev. 2009;30(2):e9-e10. doi:10.1542/pir.30-2-e9).\n• de Jongh T, Gurol-Urganci I, Vodopivec-Jamsek V, Car J, Atun R. Mobile phone messaging for facilitating self-management of long-term illnesses. Cochrane Database Syst Rev. 2012;12:CD007459. doi:10.1002/14651858. CD007459.pub2.\n• Hieftje K, Edelman EJ, Camenga DR, Fiellin LE. Electronic media-based health interventions promoting behavior change in youth: a systematic review. JAMA Pediatr. 2013;167(6):574-580. doi:10.1001/ jamapediatrics.2013.1095.\n• Horvath T, Azman H, Kennedy GE, Rutherford GW. Mobile phone text messaging for promoting adherence to antiretroviral therapy in patients with HIV infection. Cochrane Database Syst Rev. 2012;3:CD009756. doi:10.1002/14651858.CD009756.\n• Perrin JM, Gnanasekaran S, Delahaye J. Psychosocial aspects of chronic health conditions. Pediatr Rev. 2012;33(3):99-109. doi:10.1542/pir.33-3-99.\n• Salema NE, Elliott RA, Glazebrook C. A systematic review of adherence-enhancing interventions in adolescents taking long-term medicines. 1 Adolesc Health. 2011;49(5):455-466. doi:10.1016/j.jadohealth.2011.02.010.\n• Taddeo D, Egedy M, Frappier J-Y. Adherence to treatment in adolescents. Paediatr Child Health. 2008;13(1):19-24. Accessed February 21, 2014."}
{"id" : 1391, "question_text" : "A 15-year-old adolescent presents to your office for evaluation of left shoulder pain after a fall directly onto the lateral aspect of the left shoulder. On physical examination, you note mild swelling and tenderness at the distal end of the clavicle. He reports pain when he adducts his shoulder. He does not have any weakness with upper extremity muscle testing. Anteroposterior, axillary, and scapular Y radiographs of the shoulder are normal. Of the following, the MOST likely diagnosis is", "options" : "[\"acromioclavicular joint sprain\", \"occult clavicle fracture\", \"rotator cuff tear\", \"shoulder dislocation with spontaneous reduction\", \"sternoclavicular dislocation\"]", "explanation" : "The patient in the vignette has sustained an acromioclavicular (AC) joint sprain, often referred to as a shoulder separation. The most common mechanisms of injury are a blow to the AC joint or a fall onto the lateral aspect of the shoulder. The AC joint sprains typically occur in adolescents or adults who participate in collision sports such as football, rugby, and hockey. Injured athletes typically have tenderness at the AC joint and pain with adduction of the arm across the chest. Clavicle displacement is often visible with higher-grade injuries (Item C142). The distal clavicle is held in position by the AC ligament and 2 coracoclavicular (CC) ligaments. The Rockwood classification stratifies AC joint injuries into 6 types, based on whether the injury involves only the AC ligament or both the AC and CC ligaments, and the direction and degree of clavicular displacement. Lower-grade injuries are generally treated with conservative measures such as rest, a sling for comfort, and range-of-motion exercises once pain has abated. Type I injuries often heal within 1 to 2 weeks. Type II and type III injuries may take 1 to 3 months to heal. Higher-grade injuries with a large degree of clavicular displacement (eg, type IV, V, and VI) often require surgical treatment.\n\nItem C142: Clavicle displacement as seen in higher-grade injury. Courtesy of R Carl\n\nAn occult distal clavicle fracture is unlikely in this patient's case. His shoulder radiographs were normal and these films would include the distal clavicle. In addition, distal clavicle fractures are uncommon in children. The patient's history is not consistent with a shoulder joint dislocation. Rotator cuff tears are uncommon in children and cause weakness of the affected muscles. The adolescent in the vignette does not have the pain or deformity at the sternoclavicular joint that would be associated with a sternoclavicular joint dislocation.\n\nPREP Pearls\n• The most common mechanisms of acromioclavicular (AC) joint injuries are a blow to the AC joint or a fall onto the lateral aspect of the shoulder.\n• Injuries to the AC joint typically present with tenderness at the AC joint and pain with adduction of the arm across the chest.\n\nABP Content Specifications(s)\n• Recognize the clinical and radiographic findings associated with acromioclavicular separation\n\nSuggested Readings\n• Sarwark JF, LaBella CR. Pediatric Orthopaedics and Sports Injuries: A Quick Reference Guide. 2nd ed. Elk Grove Village, IL: American Academy of Pediatrics; 2014: 650 pp..\n• Stucken C, Cohen SB. Management of acromioclavicular joint injuries. Orthop Clin North Am. 2015;46(1):57-66. doi: http://dx.doi.org/10.1016/j.ocl.2014.09.003."}
{"id" : 3601, "question_text" : "A 2-year-old boy's parents are concerned about significant speech delay. He exhibits unusual dysmorphology, including malar and zygomatic hypoplasia with downslanting palpebral fissures; prominent nose; significant micrognathia and retrognathia; small, posteriorly rotated, and malformed ears; and lower eyelid colobomas with absent eyelashes. His father has similar dysmorphology. Of the following, the MOST likely reason for this boy's speech delay is", "options" : "[\"brain anomalies\", \"conductive hearing loss\", \"intellectual disability\", \"sensorineural hearing loss\"]", "explanation" : "Correct Answer: B\nThe boy in the vignette has Treacher Collins syndrome (TCS). Among patients with TCS, 40% to 50% will have conductive hearing loss because of underlying hypoplasia of the middle ear cavities and malformation of the ossicles. Inner ear anatomy is typically normal so sensorineural hearing loss is not usually seen. Less commonly, children with TCS will have a cleft palate or choanal stenosis/atresia which could also affect hearing.\n\nTreeacher Collins syndrome is typically an autosomal dominant disorder caused by gene mutations in TCOF1 (78%-93%) and POLR1C or POLR1D (8%). Sixty percent are de novo gene mutations. Fewer than 1% are inherited in an autosomal recessive pattern. There is significant inter- and intrafamilial clinical variability among affected family members. The classic findings of TCS are listed in Item C45A.\n\nIntelligence is usually normal. Craniosynostosis and brain anomalies are not features of TCS, though patients usually have brachycephaly with bitemporal narrowing. Fertility is normal. Congenital heart disease, renal anomalies, and vertebral defects are not commonly seen. Features of TCS are also seen in Nager syndrome, Miller syndrome, Goldenhar syndrome, Pierre Robin sequence, and nonsyndromic mandibular hypoplasia. With Nager syndrome and Miller syndrome, patients will have the TCS facial dysmorphology known as mandibular dysostosis, along with additional limb anomalies. Patients with Nager syndrome also have preaxial limb anomalies. Patients with Miller syndrome have postaxial limb anomalies.\n\nMajor management concerns can be stratified by age (Item C45B).\n\nPREP Pearls\n• Treacher Collins syndrome is an autosomal dominant disorder; the classic facial dysmorphology is characterized by underdevelopment of the zygomatic bones and mandible, downslanting palpebral fissures, prominent nose, micrognathia and retrognathia, external ear abnormalities, coloboma of the lower eyelid, absence of the lower eyelashes, and anterior hair displacement onto the lateral cheekbones.\n• Conductive hearing loss caused by underlying hypoplasia of the middle ear cavities and malformation of the ossicles is seen in 40% to 50% of children with Treacher Collins syndrome.\n• Careful attention to airway management and feeding difficulties in children with Treacher Collins syndrome is important in infancy and early childhood.\n\nABP Content Specifications(s)\n• Recognize the genetic and clinical features associated with various types of dysostosis, including Treacher Collins syndrome\n\nSuggested Readings\n• Katsanis SH, Jabs EW. Treacher Collins syndrome. GeneReviews. https://www.ncbi.nlm.nih.gov/books/NBK1532/.\n• Marion RW, Samanich J. Facial dysmorphism. In: McInerny TK, Adam HM, Campbell DE, DeWitt TG, Foy JM, Kamat DM, eds. American Academy of Pediatrics Textbook of Pediatric Care. 2nd ed. Elk Grove Village, IL: American Academy of Pediatrics; 2017:1326-1333. Pediatric Care Online.\n• Thompson JT, Anderson PJ, David DJ. Treacher Collins syndrome: protocol management from birth to maturity. J Craniofac Surg. 2009;20(6):2028-2035. doi:10.1097/SCS.0b013e3181be8788.\n• Trainor PA, Dixon J, Dixon MJ. Treacher Collins syndrome: etiology, pathogenesis and prevention. Eur J Hum Genet. 2009;17(3):275-283. doi:10.1038/ejhg.2008.221."}
{"id" : 3191, "question_text" : "A 4-year-old boy is referred to the hospital for the following abnormal complete blood cell count: White blood cell count, 95,000/µL (95 x 109/L), with 10% polymorphonuclear leukocytes, 25% lymphocytes, 8% monocytes, and 2% eosinophils, 55% blasts; Hemoglobin, 7.0 g/dL (70 g/L); Mean corpuscular volume, 90 µm3 (90 fL); Platelet count, 20,000 x 103/ µL (20 x 109/L). Bone marrow studies confirm the diagnosis of acute lymphoblastic leukemia. His parents give informed consent to start chemotherapy. On the second day of receiving induction chemotherapy, the child is noted to have markedly decreased urine output with an elevated serum creatinine level. Of the following, the laboratory results that would be MOST likely to be seen in this patient are", "options" : "[\"decrease in lactate dehydrogenase, potassium, and uric acid\", \"decrease in lactate dehydrogenase, uric acid, and white blood cell count\", \"increase in calcium, phosphorus, and uric acid\", \"increase in calcium, potassium, and uric acid\", \"increase in phosphorus, potassium, and uric acid\"]", "explanation" : "Preferred Response: E\nThe boy in this vignette has newly diagnosed acute leukemia and is at risk for tumor lysis syndrome (TLS), especially after he starts treatment, as a result of his high tumor burden (ie, high peripheral leukocyte count with high percentage of blasts). Tumor lysis syndrome occurs when malignant cells are rapidly lysed, typically after chemotherapy or radiation therapy has been initiated, resulting in the release of cellular components and metabolic abnormalities. Tumor lysis syndrome is more likely to occur in malignancies with a high rate of proliferation such as acute leukemia and lymphoma, or those with a high sensitivity to treatment. Tumor lysis syndrome may lead to renal insufficiency with decreased urine output and elevated serum creatinine. Brisk lysis of tumor cells can cause an increase in serum uric acid, potassium, phosphorus, and lactate dehydrogenase, with a decrease in serum calcium. Unrecognized and untreated TLS can progress to renal failure, arrhythmias, seizures, and death. Various criteria are used to diagnose clinical and laboratory TLS, all of which include elevations in uric acid, potassium, phosphate, and creatinine with hypocalcemia.\n\nEmpiric management with aggressive hydration promotes the excretion of uric acid and phosphate. The addition of sodium bicarbonate to alkalinize the urine can increase the solubility of uric acid and was historically included in TLS management strategies. However, it does not significantly increase the solubility of xanthine and hypoxanthine, the precursors of uric acid, which can lead to xanthine obstruction in the renal tubules. Therefore, alkalization of the urine is no longer universally recommended. Allopurinol, a xanthine analog, is a competitive inhibitor of xanthine oxidase that blocks the conversion of xanthine and hypoxanthine to uric acid and has been shown to decrease the incidence of obstructive uropathy caused by uric acid precipitation. Rasburicase is a recombinant urate oxidase that rapidly decreases uric acid levels by degrading uric acid to allantoin, a highly soluble substance with no known adverse effects in humans.\n\nOf note, rasburicase is contraindicated in patients with glucose-6-phosphate deficiency because of the increased risk of hemolysis.\n\nPREP Pearls\n• Tumor lysis syndrome (TLS) occurs when malignant cells are rapidly lysed, typically after chemotherapy or radiation therapy has been initiated, resulting in the release of cellular components and metabolic abnormalities. Brisk lysis of tumor cells can cause an increase in serum uric acid, potassium, phosphorus, and lactate dehydrogenase, with a decrease in serum calcium.\n• Tumor lysis syndrome is more likely to occur in malignancies with a high rate of proliferation such as acute leukemia and lymphoma, or those with a high sensitivity to treatment.\n• Tumor lysis syndrome may lead to renal insufficiency with decreased urine output and elevated serum creatinine. Unrecognized and untreated TLS can progress to renal failure, arrhythmias, seizures, and death.\n\nAmerican Board of Pediatrics Content Specification(s)\n• Plan an appropriate diagnostic evaluation to exclude tumor lysis syndrome in patients suspected of having leukemia\n\nSuggested Reading\n• Cairo MS, Coither B, Reiter A, Younes A. Recommendations for the evaluation of risk and prophylaxis of tumor lysis syndrome (TLS) in adults and children with malignant diseases: an expert TLS panel consensus. Br J Haematol. 2010;149(4):578-586. doi:10.11110365-2141.2010.08143.x.\n• Coiflier B, Altman A, Pui CH, Younes A, Cairo MS. Guidelines for the management of pediatric and adult tumor lysis syndrome: an evidence-based review. ] Gin Oncol. 2008;26(16):2767-2778. doi:10.1200/ JCO.2007.15.0177.\n• McBride A, Westervelt P. Recognizing and managing the expanded risk of tumor lysis syndrome in hematologic and solid malignancies. J Hematol Oncol. 2012;5:75. doi:10.1186/1756-8722-5-75."}
{"id" : 723, "question_text" : "A 2-year-old boy is brought to the emergency department by ambulance. Thirty minutes ago, his mother discovered him eating pills from a pillbox at his grandparents' house and called 911. The boy's mother states that the pillbox contained a 1-week supply of his grandparents' daily medications. She did not count how many tablets remained in the box before the ambulance arrived, but she states, \"I think only a couple were missing:' Both grandparents take \"blood pressure medicine\" and that the grandfather takes \"a pill for his nerves\" The boy is well-appearing and playful. His vital signs are normal for his age, and you note no abnormalities on physical examination. The mother states that she now feels \"silly for panicking over nothing. ' She asks you how soon she can take her son home. Of the following, the BEST next step in managing this patient is", "options" : "[\"administration of activated charcoal at 1 g/kg\", \"administration of intravenous normal saline at 20 mL/kg\", \"discharge the boy after educating his mother about signs and symptoms to observe for at home\", \"observe the boy in the emergency department for development of symptoms over the next 6 hours\", \"perform gastric lavage to remove ingested pill fragments from the stomach\"]", "explanation" : "The child described in this vignette was seen ingesting the contents of a pillbox thought to contain an antihypertensive agent and an antidepressant, therefore, administration of activated charcoal is warranted to decrease absorption of these toxins. Activated charcoal minimizes absorption of drugs by binding them onto its surface; it has become the gastrointestinal (GI) decontamination strategy of choice in pediatric patients and is most effective when administered within the first hour after a toxic ingestion. The dose of activated charcoal is 1 g/kg. Activated charcoal is contraindicated in patients with an unprotected airway, patients with a disrupted GI tract, or patients in whom charcoal therapy may increase the risk and severity of aspiration, such as in those ingesting a hydrocarbon. Substances that are poorly adsorbed by activated charcoal include common electrolytes, heavy metals such as iron, alcohols, cyanide, most solvents, and most water-insoluble compounds. For the asymptomatic boy described in the vignette, no contraindications for activated charcoal administration exist.\n\nPoisoning represents one of the most common medical emergencies encountered by young children and is responsible for a significant proportion of emergency department visits in the adolescent population. More than 2 million toxic exposures are reported to the American Association of Poison Control Centers Toxic Exposure Surveillance System each year. Two-thirds of these exposures occur in individuals younger than 20 years of age, with half occurring in children younger than 6 years. All physicians caring for children must be familiar with the evaluation and management of poisoning. While poisonings in young children are usually unintentional, poisonings in adolescents and young adults generally result from substance abuse, experimental risk-taking behaviors, and depression or suicidal intent.\n\nNumerous factors place young children at risk for unintentional poisonings. Between 1 and 2 years of age, most children learn to walk and develop the dexterity to use a pincer grasp; a common way that children within this age group explore their environments is by placing objects in their mouths. Young children like to mimic actions that they have seen their family members perform, such as using household products and taking medications. Furthermore, a number of household products and medications are brightly colored and may even resemble candy, making them particularly attractive to children.\n\nMost substances that young children are exposed to within their home environments, such as cosmetics and personal care items, are nontoxic. Even among the significant percentage of toxic exposures involving drugs, small quantities of most agents ingested by a child require little treatment beyond reassurance. A few medications, however, can be lethal to small children in quantities of only 1 or 2 pills or teaspoon-sized swallows.\n\nPediatric practitioners need to be familiar with the drug classes from which \"one pill can kill\" when ingested by a toddler. These classes include cardio-vascular drugs (eg, -blockers and calcium-channel antagonists), antidepressants, antipsychotics, anticonvulsants, antiarrhythmic agents, salicylates, oral hypoglycemics, and opioids, all of which are widely prescribed for adults. When drugs from these classes are involved, proper evaluation and intervention are essential for preventing severe toxic effects and even death in small children.\n\nThe first priority in managing any child who has ingested a toxic substance is to ensure stability of the airway and take any necessary steps to maintain adequate ventilation and circulation. The asymptomatic child who may have ingested only a few pills or swallows of an unknown toxin presents a clinical dilemma. A careful history, physical examination, and laboratory findings may narrow the differential diagnosis and facilitate an educated assessment of the potential severity of the exposure. In situations in which a child may have ingested a medication with potentially lethal effects, the most appropriate course of management is to decontaminate the child if no contraindications exist and to monitor closely for a period of time, depending on the poison that may have been ingested.\n\nThe boy described in the vignette is asymptomatic with normal vital signs; therefore, administration of intravenous normal saline is not warranted. Since, he may have ingested drugs with the potential to produce significant toxic effects within a few hours, GI decontamination and a period of observation are required before discharge. Although observation of the child for a period of several hours is warranted in this case, administration of activated charcoal would be the best initial step in management because activated char-coal is most efficacious within the first hour after ingestion. The clinical benefit of gastric lavage has not been confirmed in controlled studies, and its routine use in the management of poisoned patients is no longer recommended.\n\nPREP Pearls\n• Pediatric practitioners must recognize the drug classes from which \"one pill can kill\" when ingested by a toddler.\n• The first priority in managing a possible toxic ingestion is to ensure stability of the airway and maintenance of adequate ventilation and circulation.\n• Activated charcoal is the GI decontamination strategy of choice in pediatric patients with possible toxic ingestions and should be given as soon as possible provided there are no contraindications."}
{"id" : 1679, "question_text" : "A 9-month-old infant is brought to your office for evaluation of persistent watery diarrhea. You evaluated her 3 weeks ago for an episode of low-grade fever, vomiting, and diarrhea that was diagnosed as viral gastroenteritis. The mother reports that the fever and vomiting resolved after 3 to 4 days. Although the volume and frequency of diarrhea have improved, the infant's stools continue to be watery and more frequent than usual. In addition, the mother states that the infant is gassier and her stools are more malodorous than prior to this recent illness. There is no blood or mucus in her stools, and she continues to feed well. She eats a variety of puréed foods, in addition to breastfeeding. She is a well-developed, well-nourished infant with normal vital signs and weight gain. Her abdomen is nontender, but slightly distended with active bowel sounds. The remainder of the examination findings are unremarkable. A stool sample is negative for occult blood and has a pH of 5.0.\n\nOf the following, the MOST likely etiology for the infant's symptoms is", "options" : "[\"congenital lactase deficiency\", \"developmental lactase deficiency\", \"IgE-mediated milk protein allergy\", \"non-IgE\\u2013mediated milk protein allergy\", \"secondary lactase deficiency\"]", "explanation" : "Correct Answer: E\nThe persisting symptoms of frequent watery stools, flatulence, and malodorous stools in the infant in this vignette suggest malabsorption. The occurrence of these symptoms following an episode of viral gastroenteritis, plus the absence of blood and mucus in the stools or other systemic complaints in a well-nourished patient, supports the diagnosis of secondary lactase deficiency as the most likely etiology.\n\nLactose is the primary carbohydrate in mammalian milk. Lactose absorption requires lactase to hydrolyze the disaccharide into glucose and galactose in the small intestine. These monosaccharides can then be transported across the intestinal membrane. Lactose that escapes digestion and absorption by the small intestine passes into the colon where it is fermented by enteric bacteria. The byproducts of fermentation lower the stool pH to less than 6.0. The stools may also have positive test results for reducing substances. The fermentation products and the unfermented lactose cause the symptoms of lactose intolerance. The gases cause bloating, flatulence, and pain; the lactose causes osmotic diarrhea. The symptoms of lactose intolerance usually occur within a few hours of ingesting lactose, and the severity is determined by the amount consumed and the degree of lactase deficiency. Because lactase is located distally on the small intestinal villi, it is the most common disaccharidase to be affected by mucosal injury from infection or inflammation.\n\nLactose malabsorption may occur secondary to conditions that cause flattening of the villi or damage to the epithelium of the small intestine, resulting in decreased lactase levels or decreased transport across the intestinal mucosal wall. Small bowel bacterial overgrowth may also be associated with secondary lactose intolerance because of increased fermentation of ingested lactose. Acute gastroenteritis that is severe enough to cause intestinal injury (often due to rotavirus infection) is a common cause of transient lactose intolerance in young children. The symptoms are often not clinically relevant. Treatment of the underlying condition is the first step in treating secondary lactase deficiency and lactose malabsorption. If the child is symptomatic, one may consider prescribing supplemental lactase or probiotics, or recommending the temporary elimination of lactose from the diet.\n\nPrimary lactose malabsorption may be caused by congenital lactase deficiency, which is a rare autosomal recessive disorder that presents with intractable diarrhea soon after birth. Primary lactase deficiency more typically presents in childhood or adolescence and is caused by a genetically regulated reduction in lactase activity or availability. Developmental lactase deficiency is observed in premature infants born at less than 34 weeks of gestation. Lactase levels and activity increase as the gut matures.\n\nCow milk protein allergy is the most common food allergy in young children and may be IgE mediated, mixed, or related to non-IgE reactions. Exclusive breastfeeding during the first 4 to 6 months after birth reduces the risk for cow milk protein allergy. The clinical manifestations usually appear in the first few weeks to months after birth. The IgE-mediated reactions generally occur within minutes to 2 hours after ingestion and vary in severity from mild to life-threatening anaphylaxis. Mixed or non-IgE–mediated reactions may present with acute or chronic conditions and usually have a delayed onset.\n\nCow milk protein allergy may be associated with respiratory, cutaneous, and gastrointestinal reactions. Milk protein allergy that is IgE mediated may present with wheezing, stridor, otitis media with effusion, urticaria, angioedema, atopic dermatitis, vomiting, diarrhea, colic, or anaphylactic shock. Pulmonary hemosiderosis, contact or atopic dermatitis, gastroesophageal reflux, colic, constipation, failure to thrive, food protein–induced enterocolitis syndrome, eosinophilic gastrointestinal disorders, and protein-losing enteropathy are the range of clinical presentations of non-IgE–mediated milk protein allergy. Individuals with cow milk protein allergy often have stools that contain mucus and occult or frank blood, which helps to differentiate them from individuals with lactose intolerance.\n\nPREP Pearls\n• Frequent, watery, nonbloody, acidic, and malodorous stools, mild abdominal distention, and flatulence are characteristic symptoms of lactose intolerance.\n• Lactose malabsorption may occur secondary to conditions that cause flattening of the villi or damage to the epithelium of the small intestine.\n• Acute gastroenteritis is a common cause of secondary lactose intolerance that is transient in young infants and children.\n• Individuals with cow milk protein allergy often have stools that contain mucous and occult or frank blood and may experience a range of respiratory, cutaneous, and gastrointestinal reactions.\n\nABP Content Specifications(s)\n• Differentiate milk protein allergy from lactose intolerance\n\nSuggested Readings\n• American Academy of Pediatrics Committee on Nutrition. Carbohydrate and dietary fiber. In: Kleinman RE, Greer FR, eds. Pediatric Nutrition. 7th ed. Elk Grove Village, IL: American Academy of Pediatrics; 2014:387–406.\n• American Academy of Pediatrics Committee on Nutrition. Food allergy. In: Kleinman RE, Greer FR, eds. Pediatric Nutrition. 7th ed. Elk Grove Village, IL: American Academy of Pediatrics; 2014:845–862.\n• Heyman MB. Lactose intolerance in infants, children, and adolescents. Pediatrics. 2006;118(3):1279–1286. doi: http://dx.doi.org/10.1542/peds.2006-1721.\n• Vandenplas Y, Koletzko S, Isolauri E, et al. Guidelines for the diagnosis and management of cow's milk protein allergy in infants. Arch Dis Child. 2007;92(10):902–908."}
{"id" : 1786, "question_text" : "You are advising a medical student who is preparing for a rotation in Bangladesh. There is a high incidence of childhood blindness in the village she will be going to, and she asks you about potential causes of this blindness. You review the photograph from her orientation material of a child from this area (Item Q263). Of the following, this child's condition MOST likely results from a(n)", "options" : "[\"genetic disorder\", \"infection\", \"micronutrient deficiency\", \"toxic exposure\", \"traumatic incident\"]", "explanation" : "The child in this photograph has Bitot spots, which are associated with vitamin A deficiency. Bitot spots are not caused by a genetic condition, infection, toxic exposure, or trauma. Globally, vitamin A deficiency is the most common cause of preventable childhood blindness. Vitamin A is a fat-soluble vitamin found in green leafy vegetables, sweet potatoes, carrots, and liver. It is absorbed in the small intestine, converted to a stable form, and then stored in the liver. Vitamin A is essential for the normal function of epithelial cells in the eyes and the respiratory, gastrointestinal, and genitourinary tracts.\n\nVitamin A deficiency can present with:\n• Ophthalmologic symptoms\n  o Decreased visual acuity in low light (night blindness)\n  o Foamy accumulations of dead epithelial cells on the conjunctiva (Bitot spots)\n  o Corneal ulcerations or infection\n• Dry scaly skin\n• Failure to thrive\n• Impaired immune function, including increased susceptibility to diarrhea and respiratory infections\n\nOphthalmologic symptoms typically present early in the course of vitamin A deficiency and may be reversible unless corneal ulceration has occurred.\n\nVitamin A deficiency can be prevented by encouraging breastfeeding for infants and by ensuring a varied diet for older children. Patients with intestinal disease causing malabsorption are also at risk for deficiency. Treatment is with supplemental vitamin A. Hypervitaminosis A can lead to fatigue, hair loss, arthralgia, increased intracranial pressure, and carotenemia (reversible orange coloration of the skin).\n\nPREP Pearls\n• Vitamin A is essential for the normal function of the epithelial cells of the eye and the respiratory, gastrointestinal, and genitourinary tracts.\n• Vitamin A deficiency is the most common cause of preventable childhood blindness worldwide. It can cause night blindness, Bitot spots, and corneal ulcerations, as well as dry skin, failure to thrive, and increased susceptibility to infection.\n• Vitamin A is found in green leafy vegetables, sweet potatoes, carrots, and liver. Deficiency can be prevented by breastfeeding infants and ensuring a varied diet in older children.\n• Individuals with intestinal malabsorption are also at risk of vitamin A deficiency. Treatment is with supplemental vitamin A.\n\nABP Content Specifications(s)\n• Recognize the signs, symptoms, and causes of vitamin A deficiency, and manage appropriately\n\nSuggested Readings\n• Ferrari G, Viganó M. Images in clinical medicine: Bitot's spot in vitamin A deficiency. N Engl J Med. 2013;368(22):e29. http://dx.doi.org/10.1056/NEJMicm1205309.\n• Lauer B, Spector N. Vitamins. Pediatr Rev. 2012;33(8):339–351. http://dx.doi.org/10.1542/pir.33-8-339."}
{"id" : 3103, "question_text" : "You are evaluating a 12-year-old girl who was brought to the emergency department with complaints of severe headache that started 3 weeks ago and has been worsening. The headaches are not preceded by any specific symptoms, are described as throbbing in nature, and are in the back of the head and behind the eyes. They are worse at night and in the morning, and are associated with vomiting. She also has sensitivity to light and sound. Vital signs show a temperature of 37°C, heart rate of 60 beats/min, blood pressure of 140/90 mm Hg, respiratory rate of 15 breaths/min, and Spot is 98% on room air. Physical examination shows a generally well-developed and well-nourished girl who is awake, oriented, and lying still. She is complaining of a \"10 out of 10\" headache. Pupils are 6 mm, equal, and reactive to light. Her right eye cannot move laterally, but the rest of her extraocular movements and cranial nerve functions are intact. Funduscopic examination shows bilateral papilledema. She does not have any other focal neurologic deficits. She is breathing comfortably, and lungs are clear to auscultation bilaterally. Cardiovascular examination is unremarkable. Abdomen is soft, nontender, nondistended, and with no organomegaly. Extremities are warm and well perfused. Of the following, the MOST appropriate next step in management is", "options" : "[\"acetazolamide\", \"computed tomography scan of head\", \"lumbar puncture with opening and closing pressure\", \"mannitol\", \"morphine\"]", "explanation" : "Preferred Response: B\nThe child in the vignette has elevated intracranial pressure (ICP), as evidenced by symptoms of headache, vomiting that is worse when supine, and photophobia, as well as physical examination findings of hypertension, bradycardia, and papilledema. The most important next step is a computed tomography (CT) scan of the head, to evaluate for the presence or absence of hydrocephalus, and if present, whether it is obstructive or nonobstructive.\n\nIntracranial contents include blood, brain, and cerebrospinal fluid (CSF). Even in infants with open fontanelles, the calvarium is rigid and noncompliant. Thus, as indicated by the Monroe-Kellie doctrine, an increase in the volume of any of those components will cause a compensatory decrease in the other components, followed by a rapid rise in ICP when that mechanism is exhausted. Swelling of the brain component, or cerebral edema, can be caused by trauma, infection, stroke, hypoxic-ischemic encephalopathy, metabolic disturbances, or mass. The CSF component can be increased because of decreased CSF reabsorption from the arachnoid granulation system, for example, after a subarachnoid hemorrhage or from lesions obstructing CSF flow. Blood component can be increased with cerebral hemorrhage. Any of these mechanisms can cause elevated ICP.\n\nElevated ICP causes dysfunction of various parts of the brain, which may vary depending on regional variations in pressure, as well as neuronal damage and ischemia. Common symptoms of elevated ICP include lethargy, irritability, visual disturbances, vomiting, and headache. Symptoms are often worse when the patient is supine and without the benefit of gravity-driven drainage of blood and CSF. Physical findings can include macrocephaly, full or bulging fontanelle in infants, papilledema, abducens palsy, and dilated, poorly reactive, or asymmetric pupils. Late findings of elevated ICP include obtundation, decerebrate posturing, apnea, and dilated and unreactive pupils, which may indicate uncal or tonsillar herniation.\n\nAs indicated by the symptoms of headache, photophobia, and vomiting, and physical findings of elevated ICP, the most likely diagnosis in this patient is pseudotumor cerebri. Lumbar puncture with opening and closing pressure can aid in the diagnosis, and acetazolamide can be an effective treatment for pseudotumor cerebri. However, an intracranial obstructive mass lesion cannot be excluded based on this clinical picture. In that condition, a lumbar puncture can cause a large pressure gradient between the cranium and atmosphere, causing downward cerebral herniation. This phenomenon is less likely to occur in diffuse cerebral edema. Thus, a cranial CT should be performed to exclude obstructive hydrocephalus before performing a lumbar puncture. Mannitol can be an effective agent to reduce ICP, but it is not routinely used in pseudotumor cerebri, and it is more important to first establish the nature of the elevated ICP. Morphine and other medications that could potentially depress respiratory drive should be avoided at all costs. In elevated ICP, hyperventilation is a natural compensatory mechanism to decrease the blood component of the cranial vault because hypocapnia decreases cerebral blood flow Even a slight increase in PCO2 from slowed respirations because of narcotics can lead to herniation and death.\n\nElevated ICP is a serious condition that must be recognized, diagnosed, and treated promptly. Depending on age, historical findings can include irritability, photophobia, headache, vomiting, and altered sensorium. Physical examination findings can include fontanelle fullness, bradycardia, hypertension, extraocular and pupillary abnormalities, and hyperventilation. Narcotics and sedatives should be absolutely avoided. Lumbar puncture can aid in diagnosis, but only after obstructive hydrocephalus has been excluded.\n\nPREP Pearls\n• Computed tomography of the head is the first step when evaluating a patient with suspected increased intracranial pressure (ICP).\n• Narcotics and sedatives should be avoided in cases of elevated ICP.\n• Nighttime or early morning vomiting can be a sign of elevated ICP.\n• Lumbar puncture with measurement of opening and closing pressure can be helpful in elevated ICP, especially in pseudotumor cerebri or infections, but only after obstructive hydrocephalus has been excluded.\n\nAmerican Board of Pediatrics Content Specification(s)\n• Recognize the clinical findings associated with increased intracranial pressure in patients of various ages\n• Understand the indications and contraindications for examination of the cerebrospinal fluid in a patient who has increased intracranial pressure\n\nSuggested Reading\n• Kinsman SL, Johnston MV. Hydrocephalus. In: Kliegman RM, Stanton BF, St. Geme JW III, Schor NF, Behrman RE, eds. Nelson's Textbook of Pediatrics. 19th ed. Philadelphia, PA: Saunders Elsevier; 2011: 2008-2010.\n• Larsen GY, Goldstein B. Consultation with the specialist: increased intracranial pressure. Pediatr Rev. 1999;20(7):234-239. doi:10.1542/ pir.20 -7-234."}
{"id" : 2975, "question_text" : "An 11-month-old female infant is brought to the emergency department for evaluation of pallor and jaundice. She and her family immigrated to the United States from Pakistan 2 months ago. She has a temperature of 37.6°C, a heart rate of 122 beats/min, a respiratory rate of 22 breaths/min, a blood pressure of 90/68 mm Hg, and an oxygen saturation of 97% on room air. Her conjunctivae are pale and slightly icteric. Her lungs are clear to auscultation bilaterally, and her heart examination reveals a normal rhythm with tachycardia. The spleen is palpable 2.5 cm below the costal margin. Laboratory data are shown: White blood cell count 10.4 × 103/µL (10.4 × 109/L), Hemoglobin 5.1 g/dL (51 g/L), Platelet count 462 × 103/µL (462 × 109/L), Neutrophils 48%, Lymphocytes 46%, Monocytes 6%, Mean corpuscular volume 51 fL, Hemoglobin A 0%, Hemoglobin A2 0%, Hemoglobin F 100%. Of the following, the condition MOST likely to complicate this patient's disease course is", "options" : "[\"acute lymphoblastic leukemia\", \"iron deficiency\", \"iron overload\", \"vitamin B12 deficiency\"]", "explanation" : "The girl in the vignette exhibits a severe microcytic anemia with a complete absence of hemoglobin A on electrophoresis. This presentation is diagnostic of β thalassemia major. Thalassemia and thalassemia trait have an increased incidence among Middle Eastern and southeast Asian populations. Children with β thalassemia major are dependent on red cell transfusions, and the only cures are an allogeneic hematopoietic stem cell transplant or gene therapy. The chronic red cell transfusions needed to sustain these children deliver significant parenteral iron. There is no mechanism for the body to eliminate excess iron, and only a small amount of iron is lost through sloughing of the intestinal mucosa, or through menstrual bleeding in postmenarcheal girls. Iron absorption is tightly regulated through the intestine. As a consequence, children with β thalassemia major (transfusion-dependent thalassemia) will inevitably develop iron overload. The iron overload can be managed with aggressive chelation, but it remains a significant driver of morbidity and mortality in this population.\n\nHemoglobin consists of two α globin chains, two β globin chains and a heme molecule centered around an iron atom. Microcytic anemias result from the underproduction of hemoglobin, resulting in smaller erythrocytes during the hematopoietic process. A deficiency in any component of hemoglobin will result in a microcytic anemia, including iron deficiency and a defective production of the globin protein. α thalassemia occurs when there are defective α globin genes (of which there are 4). A single defective α gene results in a silent carrier, two defective genes in α thalassemia trait, three in hemoglobin H disease, and four in hydrops fetalis. As there are only two β globin genes, a single defective gene results in β thalassemia trait and two defective genes in β thalassemia major.\n\nChildren with β thalassemia major are not at higher risk of experiencing acute lymphoblastic leukemia, and they do not develop either iron deficiency or vitamin B12 deficiency.\n\nPREP Pearls\n• Microcytic anemias result from the underproduction of hemoglobin, resulting in smaller erythrocytes during the hematopoietic process. A deficiency in any component of hemoglobin will result in a microcytic anemia, including iron deficiency and a defective production of the α or β globin subchains.\n• A single defective α globin gene results in a silent carrier, two defective genes in α thalassemia trait, three in hemoglobin H disease, and four in hydrops fetalis. As there are only two β globin genes, a single defective gene results in β thalassemia trait and two defective genes in β thalassemia major.\n• Children with β thalassemia major (transfusion dependent thalassemia) will inevitably develop iron overload.\n\nABP Content Specifications(s)\n• Plan the appropriate diagnostic evaluation of suspected thalassemia\n• Recognize the clinical and laboratory findings associated with thalassemia major\n\nSuggested Readings\n• DeLoughery TG. Microcytic anemia. N Engl J Med. 2014;371(14):1324-1331. doi:10.1056/NEJMra1215361.\n• Kelly N. Thalassemia. Pediatr Rev. 2012;33(9):434-435;. doi:10.1542/pir.33-9-434.\n• Kett JC. Anemia in infancy. Pediatr Rev. 2012;33(4):186-187. doi:10.1542/pir.33-4-186.\n• McFarren AK, Levy AS. Anemia and pallor. In: McInerny TK, Adam HM, Campbell DE, DeWitt TG, Foy JM, Kamat DM, eds. American Academy of Pediatrics Textbook of Pediatric Care. 2nd ed. Elk Grove Village, IL: American Academy of Pediatrics; 2017:1199-1208. Pediatric Care Online .\n• Richardson M. Microcytic anemia. Pediatr Rev. 2007;28(1):5-14. doi:10.1542/pir.28-1-5."}
{"id" : 3751, "question_text" : "A 9-year-old boy is seen for evaluation of a rash. He was diagnosed with focal epilepsy 2 weeks ago after he was brought to the hospital for a first-time focal seizure. Evaluations at the time included an electroencephalogram that showed focal epileptiform discharges arising from the left frontal region. Magnetic resonance imaging of the brain had normal findings. Oxcarbazepine was initiated with immediate seizure control, and he was discharged home with a titration to a goal starting dose. One week ago, his mother noticed a new pruritic rash over his arms, legs, and torso. He has had temporary relief with antihistamines. No new environmental exposures have been identified. His vital signs are within normal limits. He has a fine maculopapular rash over his torso, arms, and legs with areas of excoriation but no bullae, blistering, or sloughing. His mucosal membranes and posterior pharynx have no lesions and are nonerythematous. He is breathing comfortably, in no distress with good air exchange. Neurological examination has normal findings. His neurologist is consulted, and it is determined that his anticonvulsant should be held with close outpatient monitoring and symptomatic relief with antihistamines. Within 1 week his rash has completely resolved. Of the following, the MOST likely diagnosis for this boy's rash is", "options" : "[\"drug reaction with eosinophilia and systemic symptoms\", \"exanthematous drug reaction\", \"Stevens-Johnson syndrome\", \"toxic epidermal necrolysis\"]", "explanation" : "Correct Answer: B\nThe rash described in this vignette is a hypersensitivity reaction to oxcarbazepine, a common reaction seen with antiepileptic drug (AED) use. The clinical spectrum of hypersensitivity reactions with AED use is broad, ranging from mild rashes to severe, life-threatening cutaneous reactions. Hypersensitivity reactions can include exanthematous drug eruptions, such as described in the vignette, in which a diffuse maculopapular rash develops during the initial 5 to 14 days of starting a medication. Antiepileptic medications that can commonly cause hypersensitivity reactions include phenytoin, carbamazepine, oxcarbazepine, and phenobarbital, although any AED could result in a hypersensitivity reaction. Treatment is supportive with discontinuation of the AED and symptomatic relief with antihistamines.\n\nAntiepileptic medications are a broad category of drugs with varied adverse effect profiles and adverse reactions. The 2 most common adverse drug reactions are type A and type B reactions. Type A reactions are acute, related to the pharmacologic properties of the drug, occur in a predictable, dose-dependent fashion, and are typically mild to moderate in severity. Type B reactions are idiosyncratic and unpredictable, occur unrelated to the pharmacologic properties of the medication, and can be severe.\n\nType A reactions include common adverse effects (eg, nausea, vomiting, fatigue, appetite effects, sleep-related difficulties, cognitive and behavioral changes). When selecting a specific AED, potential adverse effects should be taken into consideration in addition to the seizure type targeted. Common and severe adverse reactions or side effects should be discussed prior to the initiation of any AED, as well as any potential benefit or dual effect from a specific medication. For example, in a patient with generalized epilepsy and migraine headaches, topiramate may be chosen for dual effect as both an anticonvulsant and migraine preventative therapy.\n\nType B reactions are hypersensitivity reactions with a wide range of clinical presentation and severity. Several genetic risk factors predispose specific populations to development of these idiosyncratic reactions. Patients of Asian descent who have the HLA-B*1502 allele are at risk for serious hypersensitivity reactions, such as Stevens-Johnson syndrome or toxic epidermal necrolysis (TEN), when treated with oxcarbazepine. This risk has prompted the US Food and Drug Administration to revise the initial labeling to recommend HLA testing in at-risk populations prior to initiation of oxcarbazepine, carbamazepine, or phenytoin.\n\nLaboratory monitoring varies with AED choice. Hepatic function panels are recommended for AEDs metabolized through the liver, such as valproic acid, oxcarbazepine, and phenytoin. Complete blood cell counts are recommended for AEDs such as valproic acid that cause blood dyscrasias, commonly leukopenia or thrombocytopenia. The levels of AEDs are measured in some situations to aid in dosing, ensure therapeutic level, or evaluate for adherence. Hepatically metabolized drugs can act as either inducers or inhibitors of the cytochrome P450 system, resulting in drug-drug interactions with other hepatically metabolized AEDs, certain antibiotics, and oral contraceptive pills.\n\nDrug reaction with eosinophilia and systemic symptoms (DRESS) is a rare, potentially life-threatening condition characterized by rash, hematologic abnormalities (eosinophilia), lymphadenopathy, and internal organ involvement that develops typically 2 to 6 weeks after starting the causative medication. Antiepileptic drugs, in particular carbamazepine, lamotrigine, phenytoin, and phenobarbital, are the most common cause of DRESS. Treatment of DRESS is supportive with use of systemic corticosteroids or cyclosporine in severe cases with lung and kidney involvement.\n\nStevens-Johnson syndrome is a severe drug reaction that appears up to 3 weeks after starting a medication and is characterized by mucosal lesions at 2 or more sites and widespread targetoid or macular skin lesions preceded by a prodrome of fever, sore throat, and cough. Toxic epidermal necrolysis presents in a similar time frame, with mucosal involvement and a morbilliform rash that rapidly blisters and exfoliates involving more than 30% of the cutaneous surface. A positive Nikolsky sign, where the epidermis detaches with pressure from the finger, is consistent with TEN. Stevens-Johnson syndrome and TEN represent a disease continuum, with more extensive cutaneous involvement seen in TEN. Supportive care is provided emergently in an intensive care or burn unit setting with attention to fluids, pain control, and wound care.\n\nPotential adverse reactions and side effects of AEDs are broad. Monitoring practices, adverse effects, and drug-drug interactions vary with AED choice. Adverse cutaneous reactions can range from mild to severe and require prompt medical evaluation and discontinuation of the causative medication.\n\nPREP Pearls\n• The clinical spectrum of hypersensitivity reactions with antiepileptic drug use is broad, ranging from mild rashes, such as exanthematous cutaneous eruptions, to severe, life-threatening cutaneous reactions, such as Stevens-Johnson syndrome or toxic epidermal necrolysis.\n• Acute, dose-dependent, predictable reactions related to the pharmacologic properties of a medication include adverse effects, such as nausea, fatigue, appetite effects, and cognitive and behavioral changes, and are unique to each antiepileptic drug.\n• Laboratory monitoring for patients on antiepileptic drugs typically includes serum antiepileptic drug levels, hepatic function panels for drugs metabolized through the liver, and complete blood cell counts for medications associated with blood dyscrasias.\n\nABP Content Specifications(s)\n• Recognize laboratory abnormalities associated with anticonvulsant drug therapy\n• Recognize side effects and toxicities associated with anticonvulsant drugs\n• Plan the appropriate evaluation of serum anticonvulsant drug concentrations, including limitations and timing\n\nSuggested Readings\n• Guvenir H, Dibek Misirlioglu E, Civelek E, et al. The frequency and clinical features of hypersensitivity reactions to antiepileptic drugs in children: a prospective study. J Allergy Clin Immunol Pract. 2018;6(6):2043-2050. doi:10.1016/j.jaip.2018.02.018.\n• Henderson L, Williams JV. Drug eruptions, erythema multiforme, Stevens-Johnson syndrome. In: McInerny TK, Adam HM, Campbell DE, DeWitt TG, Foy JM, Kamat DM, eds. American Academy of Pediatrics Textbook of Pediatric Care. 2nd ed. Elk Grove Village, IL: American Academy of Pediatrics; 2017:1984-1992. Pediatric Care Online.\n• Perucca P, Gilliam FG. Adverse effects of antiepileptic drugs. Lancet Neurol. 2012;11(9):792-802. doi:10.1016/S1474-4422(12)70153-9.\n• Segal A, Doherty K, Leggott J, Zlotoff B. Cutaneous reactions to drugs in children. Pediatrics. 2007;120(4):e1082-e1096. doi:10.1542/peds.2005-2321."}
{"id" : 3673, "question_text" : "Towards the end of a busy emergency department shift, an attending physician is simultaneously caring for multiple patients in the same area. A 2-year-old girl with acute lymphoblastic leukemia, fever, and neutropenia and an 18-year-old young woman with dehydration and presumed vasovagal syncope are in adjacent beds. Orders are placed in the electronic medical record for both patients. A laboratory evaluation (including a blood culture), intravenous cefepime, and an admission order are placed for the 2-year-old patient. An intravenous bolus of normal saline, a capillary blood glucose level, and electrocardiography are ordered for the 18-year-old patient. Soon after the orders are placed, the nurse reports that she unintentionally administered the cefepime to the 18-year-old patient instead of the 2-year-old patient. A review of the electronic medical record confirms that cefepime was ordered for the correct patient. The amount of cefepime that the 18-year-old patient received was less than 11 mg/kg. Cefepime is ordered again for the 2-year-old patient and promptly administered. An hour later, the 18-year-old patient is re-examined. She is no longer dizzy and wants to go home. Her vital signs have remained stable, she has tolerated hydration by mouth, her blood glucose level and electrocardiogram are normal, and she is ambulating around the emergency department without issue. There are no apparent adverse effects from the cefepime. Her discharge papers are prepared, and the disclosure of the medical error, which resulted in the administration of a subtherapeutic dose with no adverse effects, is contemplated. Of the following, the BEST decision in regards to reporting this error is", "options" : "[\"do not report the error to the hospital's adverse event database or disclose the error to the patient\", \"report the error to the hospital's adverse event database and disclose the error to the patient\", \"report the error to the hospital's adverse event database but do not disclose the error to the patient\", \"report the error to the patient but not to the hospital's adverse event database\"]", "explanation" : "Correct Answer: B\nThe administration of cefepime to the 18-year-old patient in this vignette was a medical error. The appropriate course of action is to report the error to the hospital adverse event database (or committee) and disclose the error to the patient, even though there is unlikely to be any adverse events stemming from the error. Any medical error that occurs or almost occurs (a \"near-miss\") must be reported to the appropriate authority because these events are often indicative of a systems issue and less frequently the result of an individual error, and they must be investigated to identify the cause of the error. The creation of a central repository for medical errors allows for evaluation of error patterns and recurrences of errors and is the first step in ensuring that safeguards are ultimately put in place to prevent future medical errors.\n\nDaily medical practice is rife with medical errors. In To Err is Human, the seminal report released by the Institute of Medicine in 1999, it was estimated that up to 100,000 deaths occur each year due to medical errors. Children are at higher risk for medication errors than adults because of the need to calculate medication doses by weight or surface area, as compared to standard dosing often used for adults. Many different factors contribute to medical errors, ranging from provider fatigue and distractions to medication names that sound alike, patients with similar names, and overreliance on electronic medical records and computerized order entry. Any unanticipated event in the health care setting that puts a patient at risk of or causes serious injury or death is known as a sentinel event. Sentinel events should lead to a root-cause analysis in which every aspect leading up to the event, whether direct or indirect, is investigated in an attempt to identify the causes.\n\nStrategies can be employed by the individual and the institution to minimize the chance of a medical error. These strategies include writing orders in a quiet area, minimizing distractions, avoiding unsafe abbreviations (eg, QD for daily, QID for 4 times a day), ensuring accurate weights (in kilograms, not pounds), using leading zeros when writing numbers, and avoiding trailing zeros. Computerized order entry should be looked upon as a physician guide and not a substitute for physicians doing their own math and checking doses. Providers may be intimidated to report medical errors. This intimidation can be the result of a fear of being labeled a whistle-blower and potentially suffering repercussions from colleagues or a fear of admitting fault and exposing oneself to potential litigation. Organizations should adopt practices that encourage voluntary reporting, such as instituting anonymous methods of reporting medical errors and ensuring no retaliation or repercussions for reporting medical errors, near-misses, or unsafe conditions in good faith.\n\nPREP Pearls\n• Medical errors are typically caused by underlying systems issues and are often not the fault of the individual.\n• A sentinel event is an unanticipated event that leads to serious injury or death (or risk of serious injury or death).\n• A near-miss is a potential adverse event that does not actually occur, either due to intentional intervention or luck.\n\nABP Content Specifications(s)\n• Apply voluntary systems for reporting of adverse medical events\n• Identify barriers to reporting adverse events\n• Recognize what interventions can reduce error in situations (eg, stress, fatigue, distraction) at high risk for medical error\n• Understand and apply methodologies to prevent medication errors\n• Apply effective strategies to improve reporting of adverse events\n\nSuggested Readings\n• Kohn LT, Corrigan J, Donaldson MS, eds. To Err is Human: Building a Safer Health System. Washington, DC: National Academy Press; 2000. http://www.nationalacademies.org/hmd/~/media/Files/Report%20Files/1999/To-Err-is-Human/To%20Err%20is%20Human%201999%20%20report%20brief.pdf.\n• Leonard MS. Patient safety and quality improvement: medical errors and adverse events. Pediatr Rev. 2010;31(4):151-158. doi:10.1542/pir.31-4-151.\n• Neuspiel DR. Medical errors, adverse events, and patient safety. In: McInerny TK, Adam HM, Campbell DE, DeWitt TG, Foy JM, Kamat DM, eds. American Academy of Pediatrics Textbook of Pediatric Care. 2nd ed. Elk Grove Village, IL: American Academy of Pediatrics; 2017:2287-2295. Pediatric Care Online."}
{"id" : 1466, "question_text" : "A 2-week-old neonate with an atrioventricular canal defect presents to your office for a health supervision visit. She was asymptomatic at birth, as would be expected. The family is very conscientious about bringing their child to her scheduled appointments, and they inform you that they have decided not to pursue surgical treatment of her heart defect. They will be happy to treat her symptoms with medication, but do not want to subject her to hospitalizations or diagnostic testing that is invasive or painful. Her parents are aware that this course of action may shorten her life, but they believe that giving her a life without trauma is more important. You do not agree with this decision and hope to work toward a mutual understanding of these different perspectives. Of the following, the PRIMARY principle of ethical decision-making the parents are applying is", "options" : "[\"beneficence\", \"fidelity\", \"justice\", \"nonmaleficence\", \"veracity\"]", "explanation" : "Correct Answer: D\nThe primary principle being invoked by the parents of the neonate in the vignette is nonmaleficence.\n\nThe principle of doing no harm, or nonmaleficence, is one of the major ethical principles in medical decision-making, and was found to be the most important factor in a study of nonmedical psychology students. For these parents, this is the paramount factor in their decision-making. From the physician's point of view, the principle of autonomy (the parent's right to make decisions for their child) will likely be an area of conflict.\n\nAnother important principle in ethical decision-making is beneficence, the concept of \"doing good for others.\" In a situation in which parents and physicians or other healthcare providers are not in agreement, it is crucial to determine whether this issue is merely a difference of opinion or one of quality of care and life. The American Academy of Pediatrics (AAP) has published a policy statement that includes the core principle that \"all children are entitled to effective medical treatment likely to prevent serious harm or death.\" The question of whether the parents' decision is considered suboptimal care or likely to cause harm is a distinction with which ethics committees are often faced.\n\nThe other response choices are clearly not the most important principles guiding the parents' decision at this time. Fidelity is the principle of maintaining confidentiality. Justice is the principle of being fair. Veracity is the principle of truth telling.\n\nThe AAP maintains a comprehensive bibliography of articles and papers on the subject of ethics and medical decision-making.\n\nPREP Pearls\n• The ethical principle of nonmaleficence is that of \"do no harm.\"\n• Parents are responsible to act for their dependent children and their preferences may sometimes conflict with those of healthcare providers, especially if they are invoking different ethical principles.\n\nABP Content Specifications(s)\n• Recognize and apply ethical principles regarding the care of children and adolescents with disabilities\n\nSuggested Readings\n• Adam MB, Diekema DS, Mercurio MR. American Academy of Pediatrics bioethics resident curriculum: case-based teaching guides. American Academy of Pediatrics website. https://www2.aap.org/sections/bioethics/PDFs/BioethicsResidentCurriculum.pdf.\n• Cummings CL, Mercurio MR. Autonomy, beneficence, and the rights of parents and children—exploring the application of ethical principles in pediatrics. American Academy of Pediatrics website. https://www2.aap.org/sections/bioethics/PDFs/Curriculum_Session10.pdf.\n• Page K. The four principles: can they be measured and do they predict ethical decision making? BMC Med Ethics.2012;13:10. doi: http://dx.doi.org/10.1186/1472-6939-13-10.\n• Snyder JE, Gauthier CC. The underlying principles of ethical patient care. Evidence-Based Medical Ethics: Cases for Practice-Based Learning. Totowa, NJ: Humana Press; 2008:11-16."}
{"id" : 2913, "question_text" : "A 25-hour-old neonate with abdominal distention is being evaluated. His 34-year-old mother has gestational diabetes controlled by diet; her hemoglobin A1c level is 6.6%. Echogenic bowel was noted on prenatal ultrasonography. He was delivered vaginally with an Apgar score of 9 at 1 and 5 minutes. He has breastfed 6 times with a good latch. He has not yet had a bowel movement. On physical examination, the neonate appears comfortable. He has a prominent white forelock, barrel-shaped chest, moderate abdominal distention, and decreased bowel sounds (Item Q114A). Abdominal radiography is performed (Item Q114B). Of the following, the MOST likely diagnosis for this neonate is", "options" : "[\"Hirschsprung disease\", \"inadequate oral intake\", \"necrotizing enterocolitis\", \"small left colon syndrome\"]", "explanation" : "The neonate's clinical presentation of delayed passage of meconium and dilated bowel on abdominal radiography, in combination with a white forelock suggests the diagnosis of Hirschsprung disease. Waardenburg type 4, or Waardenburg-Shah syndrome, is known to be associated with Hirschsprung disease.\n\nHirschsprung disease is caused by arrested migration of neuroblast cells to the rectum, resulting in ineffective relaxation and increased muscle tone. In most cases, ganglion cells are absent only in the rectosigmoid area. Long-segment aganglionosis occurs in approximately 10% of affected children. Hirschsprung disease is diagnosed on suction rectal biopsy. A water-soluble contrast enema may be helpful by demonstrating a transition zone between normal colon and the narrow colon without ganglion cells. Patients with Hirschsprung disease are at risk for enterocolitis (which can progress to toxic megacolon), in which bacterial translocation occurs in areas of bowel dilation, causing a systemic inflammatory response.\n\nWaardenburg syndrome (WS) is inherited in an autosomal dominant pattern. Neonates may have a white forelock, wide nasal bridge, sensorineural deafness, heterochromia iridis, and lateral displacement of the inner canthi of the eyes. Germline mutations in the PAX3 gene are responsible for WS. There are 4 clinical subtypes; type 4 (Waardenburg-Shah syndrome) is always associated with Hirschsprung disease (Item C114).\n\nA neonate with necrotizing enterocolitis would have significant tenderness on abdominal palpation, which was not noted in the neonate in the vignette. Although associated with neonates born to mothers with diabetes, small left colon syndrome typically presents with signs and symptoms of small bowel obstruction. A neonate with inadequate intake would demonstrate feeding difficulties, unlike the neonate in the vignette.\n\nPREP Pearls\n• Hirschsprung disease typically presents in the neonatal period with delayed passage of meconium, abdominal distention, and feeding intolerance.\n• Waardenburg syndrome is characterized by a prominent white forelock, wide nasal bridge, sensorineural deafness, heterochromia iridis, and in some types, lateral displacement of the inner canthi.\n• Waardenburg syndrome type 4 (Waardenburg-Shah syndrome) is always associated with Hirschsprung disease.\n\nABP Content Specifications(s)\n• Recognize the clinical and laboratory features associated with intestinal obstruction in a newborn infant, and manage appropriately\n• Recognize the clinical and laboratory features associated with necrotizing enterocolitis in a newborn infant\n\nSuggested Readings\n• Joyce JC. Hypopigmented lesions. In: Kliegman R, St Geme JW, Blum NJ, Shah SS, Tasker RC, Wilson K, eds. Nelson Textbook of Pediatrics. Philadelphia, PA: Elsevier; 2020:3477-3480.e1.\n• Levitt M, Garza JM, Pena A, Lawal T. Colorectal disorders. In: McInerny TK, Adam HM, Campbell DE, DeWitt TG, Foy JM, Kamat DM, eds. American Academy of Pediatrics Textbook of Pediatric Care. 2nd ed. Itasca, IL: American Academy of Pediatrics; 2016;chap 232:1970-1880. Pediatric Care Online.\n• Maqbool A, Fiorino KN, Liacouras CA. Motility disorders and Hirschsprung disease. In: Kliegman R, St Geme JW, Blum NJ, Shah SS, Tasker RC, Wilson K, eds. Nelson Textbook of Pediatrics. Philadelphia, PA: Elsevier; 2020:1955-1965.e1."}
{"id" : 1342, "question_text" : "You are called to the newborn nursery to evaluate a neonate with respiratory distress. The mother is a 32-year-old gravida 4, para 3 woman with a history of obesity and type A1 gestational diabetes mellitus admitted in active labor at 38 weeks of gestation. Prenatal laboratory test results were significant for positive group B Streptococcus. Rupture of membranes was less than 4 hours and meconium-stained amniotic fluid was noted. A male neonate was born vaginally without assistance. Apgar scores were 8 and 8 and 1 and 5 min, respectively. In the nursery, his vital signs show a temperature of 37°C, heart rate of 155 beats/min, respiratory rate of 72 breaths/min, and blood pressure of 84/57 mm Hg. Pulse oximetry on the right hand reads 70% in room air. Mild respiratory distress with nasal flaring and intermittent grunting is noted on physical examination. Laboratory data are shown: Laboratory test Result White blood cell count 24,700/μL (24.7 x 109/L) Hemoglobin 13.7 g/dL (137 g/L) Platelet count 267 x 103/μL (267 x 109/L) His chest radiograph is shown in Item Q90. Of the following, the MOST likely diagnosis is", "options" : "[\"meconium aspiration syndrome\", \"pleural effusion\", \"pneumonia\", \"respiratory distress syndrome\", \"transient tachypnea of the newborn\"]", "explanation" : "Correct Answer: A\nGiven the clinical presentation and appearance on chest radiograph, meconium aspiration syndrome (MAS) is the most likely diagnosis for the neonate in this vignette. Meconium aspiration syndrome is caused by aspiration of meconium-stained amniotic fluid either before birth or during delivery. Most neonates with MAS are late term with gestational age greater than 41 weeks. The incidence of MAS has decreased due to changes in obstetric practice, with fewer mothers delivering infants after 41 weeks. Clinically, neonates with MAS will present with severe respiratory distress, grunting, and cyanosis, and will require respiratory support. On examination, there may be meconium staining of the umbilical cord, skin, and nails.\n\nMeconium aspiration syndrome causes a characteristic chest radiograph with asymmetric patchy infiltration. Meconium in the lungs inactivates surfactant. Thus, neonates with MAS may require high pressure and oxygen to maintain ventilation and oxygenation. This increases their risk of pneumothorax with positive pressure. They are also at risk of pulmonary hypertension. The diagnosis of MAS is made with clinical history and chest radiograph. While the mortality associated with MAS has decreased, infants with MAS continue to be at increased risk for reactive airway disease, which may persist into childhood.\n\nIn comparison, neonates with transient tachypnea of the newborn (TTN) also present with respiratory distress and an oxygen requirement. On examination, they classically have a hyperexpanded barrel chest. However, on chest radiograph, TTN shows increased perihilar markings and fluid in the fissure (Item C90). These findings are not seen in the neonate in this vignette.\n\nTransient tachypnea of the newborn results from delayed activation of efflux of water out of the lung by Na-K channels. Normally, these channels are activated during delivery, and possibly induced by stress hormones. There are a number of risk factors for TTN including cesarean delivery, infection, maternal diabetes, and male sex.\n\nPleural effusion may be caused by infection, congestive heart failure, or MAS. Since neonates are supine, small pleural effusions rarely cause significant respiratory distress on examination. On chest radiograph, there may be blunting of the costophrenic angle or a diffuse haziness caused by layering of pleural fluid.\n\nRespiratory distress syndrome is a disease of premature neonates born less than 37 weeks of gestation. It is characterized by inadequate surfactant production. As a consequence of poor compliance, on chest radiograph, there is poor lung expansion. Microatelectasis appears as a homogenous ground glass appearance.\n\nPneumonia in a neonate is most commonly caused by group B Streptococcus (GBS). Presentation includes respiratory distress and perinatal risk factors for infection, including prolonged rupture of membranes, chorioamnionitis, and positive maternal GBS status. On chest radiograph, neonates exhibit a bilateral diffuse infiltrate.\n\nPREP Pearls\n• Meconium aspiration syndrome is a disease of late term infants with gestational age greater than 41 weeks.\n• Meconium aspiration syndrome has a characteristic chest radiograph appearance of patchy infiltrates.\n• Transient tachypnea of the newborn on chest radiograph shows increased perihilar markings and fluid in the right horizontal fissure.\n\nABP Content Specifications(s)\n• Identify the signs and symptoms of transient tachypnea of the newborn, and manage appropriately\n• Recognize the characteristic clinical and radiographic appearance of meconium aspiration syndrome in a newborn infant, and manage appropriately\n\nSuggested Readings\n• Dargaville PA, Copnell B. The epidemiology of meconium aspiration syndrome: incidence, risk factors, therapies, and outcome. Pediatrics. 2006;117(5):1712-1721. doi: http://dx.doi.org/10.1542/peds.2005-2215.\n• Yeh TF. Core concepts: meconium aspiration syndrome: pathogenesis and current management. NeoReviews. 2010;11(9):e503-e512. doi: http://dx.doi.org/10.1542/neo.11-9-e503."}
{"id" : 2163, "question_text" : "A 2-month-old infant is seen for a health supervision visit. Her physical examination findings are remarkable for epicanthal folds, upslanted palpebral fissures, midface hypoplasia, hypotonia, and single transverse palmar crease on the right hand. Echocardiography shows a ventricular septal defect. Chromosome analysis reveals an unbalanced translocation between 2 chromosomes as the etiology of her clinical features. The parental chromosome analysis showed a mother with 45,XX,t(14:21) and a father with 46,XY. Of the following, the MOST likely risk of the parents having another liveborn child with the same disorder is", "options" : "[\"0.5%\", \"10%\", \"50%\", \"100%\"]", "explanation" : "Critique\nThe infant described in the vignette has trisomy 21 (Down syndrome). Her mother has a balanced translocation abnormality with karyotype 45,XX,t(14:21), and her father has a normal chromosome complement. Therefore, this girl has an unbalanced translocation between chromosomes 14 and 21 (46,XX,der [14;21] +21) as the etiology of her condition, and the recurrence risk (RR) is 10%.\nChromosome analysis is the appropriate test to confirm the diagnosis of trisomy 21 and provide parents with information about RR. Chromosome analysis in an individual with trisomy 21 can show either of the following:\nFreestanding extra chromosome 21 \nThis occurs in approximately 95% of affected individuals. It results from meiotic nondisjunction (maternal in 90%, paternal in 10%). Mosaicism for a freestanding extra chromosome 21 has been identified in approximately 1% of cases. Parental testing is not indicated in the case of a child with a freestanding extra chromosome, as these events are sporadic. The RR for having another child with trisomy 21 is estimated to be 1%, or the age-adjusted risk of the mother (if >1%) at the time of the next pregnancy.\nUnbalanced translocation between chromosome 21 and another acrocentric chromosome (13, 14, 15, 21, or 22) (ie, Robertsonian translocation) \nThis occurs in approximately 4% of affected individuals. It results in three copies of chromosome 21, two of which are freestanding and one of which (extra copy) is translocated onto another chromosome. Parental testing is indicated when a child has an unbalanced chromosome 21 translocation; approximately 30% of these are inherited from a parent with a balanced translocation. \nFor translocations that involve chromosomes 13, 14, 15, or 22, the RR for having another child is 10% to 15% if the mother is the carrier and <1% if the father is the carrier. For translocations between two chromosomes 21, the RR is always 100%. If neither parent is a carrier for a balanced translocation, the RR is the same as for freestanding extra chromosome 21 (1% or the age-adjusted risk of the mother [if >1%] at the time of the next pregnancy).\nThe recurrence risk of a sporadic structural or combined structural and numeric chromosome rearrangement is less than 0.5%–2%.\nSuggested Reading(s)\nBull MJ. Down syndrome: managing the child and family. In: McInerny TK, Adam HM, Campbell DE, DeWitt TG, Foy JM, Kamat DM, eds. American Academy of Pediatrics Textbook of Pediatric Care. American Academy of Pediatrics; 2021:chap 244. Accessed September 21, 2024. Pediatric Care Online\nBull MJ, Trotter T, Santoro SL, Christensen C, Grout RW; The Council on Genetics. Health supervision for children and adolescents with Down syndrome. Pediatrics. 2022;149(5):e2022057010. doi:10.1542/peds.2022-057010\nLyons MJ. Specific genetic conditions. In: Saul RA, ed. Medical Genetics in Pediatric Practice. American Academy of Pediatrics; 2013:chap 9. Accessed August 7, 2023. Medical Genetics Online\nSheets KB, Crissman BG, Feist CD, et al. Practice guidelines for communicating a prenatal or postnatal diagnosis of Down syndrome: recommendations of the National Society of Genetic Counselors. J Genet Couns. 2011;20(5):432-441. doi:10.1007/s10897-011-9375-8\nContent Domain\nGenetics\nLearning Objectives\nUnderstand the risk of having a second child with trisomy 21 when the mother is a balanced translocation carrier\nThe correct answer is: 10%\nView Peer Results"}
{"id" : 3287, "question_text" : "You were awarded a 1-year intramural grant for research and wish to conduct a study to investigate the role of intrauterine devices as a risk factor for pelvic inflammatory disease. You must decide whether to do a case-control or a cohort study. After comparing the advantages of each type of study, you determine that a case-control study would be the better option. Of the following, the BIGGEST advantage of this type of study design for this research project is that it", "options" : "[\"allows for calculation of rates of disease in exposed and unexposed\", \"allows for study of multiple potential risk factors\", \"is well suited for common conditions\", \"is well suited for conditions with a short latency\", \"relies on recall or records of past events\"]", "explanation" : "Preferred Response: B\nCase-control studies are used to determine how much more (or less) likely it is that cases with a disorder have been exposed to the factors being studied than were the controls without the disorder. An advantage of a case-control study design is that it allows for the study of multiple risk factors, is relatively quick to conduct, is relatively inexpensive, and requires fewer subjects than a cohort study. It would fit the needs of the project proposed in the vignette, allowing one to identify cases of pelvic inflammatory disease (RID) diagnosed during a specified time frame and to evaluate the subjects for several possible risk factors. Case-control design is also well suited for the study of rare diseases and conditions with a long latency period. Ideally, one should identify and enroll all incident cases in a population during a specified period, defining cases using standard diagnostic criteria that are objective rather than subjective, and noting the severity of the disease being studied. This study design has disadvantages that include the need to recall all past exposures being studied, difficulty with controlling for extraneous variables, and the inability to determine rates of disease in exposed and unexposed subjects. Rates of disease cannot be calculated in case-control, as this is a retrospective study and the population at risk is not known.\n\nCohort studies (prospective or retrospective) involve comparison of a subject group with a known exposure to that of a control group free of exposure. These studies take a long time to conduct, are relatively expensive, and loss to follow-up can be a problem. In addition, if the exposure status of the subjects or controls changes, it results in \"contamination!' Cohort study design is not well suited to conditions with a long latency and for rare conditions. However, the time interval between the exposure and outcome is clear, and it allows for rates of disease in exposed and unexposed individuals to be determined. It also allows for study of multiple outcomes. Prospective cohort design is useful for the study of rare exposures and reduces the risk of survivor bias, as subjects are followed prospectively after an exposure. A cohort design for the study described in this vignette would entail gathering data on girls in this practice who have intrauterine devices in place, which would likely be a very small number, and then following them over time to determine how many develop PID within the year of the study. Therefore, in this situation, this type of study would likely yield a limited amount of useful information.\n\nPREP Pearls\n• Case-control studies are used to determine how much more (or less) likely it is that case subjects were exposed to the factors being studied than were controls.\n• Cohort studies involve comparison of a group with a specific exposure to that of a control group free of exposure.\n\nAmerican Board of Pediatrics Content Specification(s)\n• Understand the uses and limitations of cohort studies\n• Understand the uses and limitations of case-control studies\n\nSuggested Reading\n• Jennings 1M, Sibinga E. Research and statistics: understanding and identifying bias in research studies. Pediatr Rev. 2010;31(0161-162. doi:10.1542/pir.31-4-161.\n• Johnson 51.. Research and statistics: a question of time: cross-sectional versus longitudinal study designs. Pediatr Rev. 2010;31(0:250-251. doi:10.1542/pir.31-6-250.\n• Perry-Parrish C, Dodge R. Research and statistics: validity hierarchy for study design and study type. Pediatr Rev. 2010;31(6):27-29. doi:10.1542/ pir.31-1-27.\n• Upadhya K, Rowe P. Research and statistics: case-control studies. Pediatr Rev. 2010; 31(6):70-71. doi:10.1542/pir.31-2-70."}
{"id" : 791, "question_text" : "An 8-year-old girl has a seizure at school lasting about 2 minutes. She was healthy all her life with no family history of seizures. EEG shows right and left centrotemporal spikes consistent with benign rolandic epilepsy. What is the MOST appropriate advice to provide the girl's parents?", "options" : "[\"brain damage is common even after a single seizure\", \"children who have epilepsy should not participate in contact sports\", \"children who have epilepsy should not take baths alone\", \"risk of sudden death is high in children who have epilepsy\", \"their daughter will likely need lifelong seizure medications\"]", "explanation" : "Seizure precautions should be discussed with the parents and the child, if appropriate, after a first seizure and typically at every office visit after that. Anyone with a seizure should not be in or around water by themselves. Children with seizures can shower alone because the risk of drowning in the shower is low, but they should not take a bath unattended and the bathroom door should remain unlocked. Typical school sports such as football or soccer are not restricted; however, participation in high-velocity sports, such as motocross or gymnastics should be discussed with parents and the child.\n\nMost parents fear their child will have a brain injury or die because of a seizure. There is no evidence that a single, short (<30 min) seizure causes measurable brain damage to a child. The risk of sudden, unexplained death in epilepsy is very low in adults and even lower in children. Preventing accidental drowning is the most important intervention parents can do to protect their child.\n\nIn general, there is a 45% recurrence risk after a first, unprovoked seizure. The true risk of seizure recurrence, and thus the usefulness of starting seizure medications, depends on the cause of the seizures. The history should elucidate causes of provoked seizures, such as head injury, electrolyte abnormality, hypoglycemia, infection, etc. Electroencephalography (EEG) can help identify an underlying epilepsy syndrome. If the seizure has focal onset, magnetic resonance imaging of the brain should also be performed to evaluate for a brain lesion. Unless the child has a history pointing to metabolic imbalances (vomiting, diarrhea, for example) it is not helpful to perform routine laboratory tests. Depending on the clinical situation, a seizure medication can be started to prevent further seizures while the workup is undertaken.\n\nFor the girl described in the vignette, the clinical history and EEG confirm a diagnosis of benign rolandic epilepsy (BRE). In this syndrome, seizures typically occur during sleep only and the child outgrows the seizure tendency within 2 years of diagnosis. In many cases of BRE, seizure medications are not used; seizure precautions are followed closely until the child has outgrown the seizure tendency.\n\nPREP Pearls\n• Seizure precautions including water safety should be reviewed with families of children with seizure.\n• Most patients with a new seizure can be treated by their pediatrician. Referral to a neurologist is helpful if an abnormality is identified on imaging or electroencephalography.\n\nAmerican Board of Pediatrics Content Specification(s):\n• Know how to manage a child following a first seizure\n\nSuggested Reading:\n• Camfield R Camfield C. Special considerations for a first seizure in childhood and adolescence. Epilepsia. 2008;49(suppl 1):40-44. doi: 10.1111/j.1528-1167.2008.01449.x"}
{"id" : 2285, "question_text" : "A 5-year-old girl is seen by her pediatrician in a telemedicine visit at 8 pm. For 4 days, she has had a headache, abdominal pain, decreased appetite, and throat pain with swallowing solids; she is tolerating fluids. She is taking ibuprofen as needed, which ameliorates her symptoms. She attends elementary school, where she was recently exposed to a friend with similar symptoms. The girl appears to be in no acute distress. She is able to talk without difficulty. Her temperature is 39 °C. On visual examination, there is no conjunctival injection, eye discharge, or nasal discharge. Her oral mucosa is moist. Her father assists with aspects of her physical examination. He provides a light source to inspect her mouth and pharynx. He palpates her neck and reports bilateral swollen anterior cervical lymph nodes; the girl reports tenderness on both sides of the neck. She has no pain with external manipulation of her ears and no maxillary or frontal sinus tenderness. There are no respiratory retractions. Her father reports that, palpated gently in the supine position, her abdomen feels soft without tenderness. No rash is seen on her skin examination. Of the following, the BEST next step in this girl's care is to", "options" : "[\"prescribe an antibiotic and advise an in-person visit if her symptoms persist or worsen\", \"provide reassurance and a note to return to school when she is afebrile\", \"recommend supportive care and an in-person visit the next day\", \"refer her to the emergency department for further evaluation tonight\"]", "explanation" : "The girl in the vignette has symptoms and signs that raise suspicion regarding streptococcal pharyngitis. The best next step in her treatment is to recommend supportive care for the night and an in-person visit with her primary pediatrician the next day. In this case, an in-person visit is important to help distinguish a viral from a bacterial etiology of her pharyngitis by testing for group A Streptococcus and, if the result is positive, treating her infection appropriately. It would not be appropriate to treat her presumptively with an antibiotic for a bacterial infection, nor should she return to school during this febrile illness. Because she is in no acute distress and there are no concerning history or examination findings, referral to an emergency department is not warranted at this time.\n\nThe Association of American Medical Colleges defines telehealth as 'the use of technology to deliver health care at a distance.' Telehealth is a broad term that includes both clinical and nonclinical health care; telemedicine refers to an encounter for clinical care. Both telehealth and the practice of telemedicine have increased since the COVID-19 pandemic.\n\nThere are many advantages of telehealth visits for both patients and physicians (Table 1). They provide patients with increased access to health care, especially during times of the day and week when their primary pediatrician may not be available. Consequently, this can decrease the number of visits to urgent care centers and emergency departments. Physicians have the ability to triage care and collaborate with specialists.\n\nHowever, although a wide range of conditions can be evaluated and managed via telemedicine, there are limitations to the diagnosis and treatment of specific illnesses. In addition to the limits of treatment, several factors must be considered by both families and physicians when deciding to use telehealth as a tool for care. Each family has individual needs and barriers that they may face with this modality. Although access to care is overall one of telehealth's greatest advantages, patients may face many barriers to telehealth care, including systemic inequities (eg, racism, discrimination), socioeconomic status, and educational factors (Table 2).\n\nWhen addressing barriers to telemedicine care, physicians should ask families about their financial limitations, access to technology, and ability to have private communication. Some suggested methods to overcome device and connection barriers include using a public space with wi-fi (eg, public library), using a business parking lot to access a wi-fi network, or using a regional hotspot to connect. Providing technical support, written instructions, an alternative modality to connect, and having language services readily available can reduce disparities in telehealth care.\n\nSuggested Reading(s)\nAssociation of American Medical Colleges. Telehealth Competencies Across the Learning Continuum. AAMC New and Emerging Areas in Medicine Series. Association of American Medical Colleges; 2021.\nCurfman AL, Hackell JM, Herendeen NE, et al. Telehealth: opportunities to improve access, quality, and cost in pediatric care. Pediatrics. 2022;149(3):e2021056035. doi:10.1542/peds.2021-056035\nCurfman AL, Hackell JM, Herendeen NE, et al; Section on Telehealth Care, Committee on Practice and Ambulatory Medicine, Committee on Pediatric Workforce. Telehealth: improving access to and quality of pediatric health care. Pediatrics. 2021;148(3):e2021053129. doi:10.1542/peds.2021-053129\nAmerican Academy of Pediatrics. Telehealth: practice management. American Academy of Pediatrics. Accessed September 1, 2024. www.aap.org/en/practice-management\n\nContent Domain\nTransitions of Care\n\nLearning Objectives\nUnderstand the advantages of telehealth for families and physicians\nUnderstand the barriers to utilizing telehealth for families and physicians\nBe familiar with the limitations of telemedicine when treating children"}
{"id" : 180, "question_text" : "A term infant is delivered by repeat elective cesarean section complicated by oligohydramnios. Mild bilateral pelviectasis was noted on prenatal ultrasonography. Artificial rupture of the membranes at delivery reveals scant meconium-stained fluid. The infant initially has a strong cry, but she develops escalating respiratory distress and cyanosis requiring endotracheal intubation in the delivery room. Upon arrival in the nursery, she is placed on the ventilator, with the following settings: peak inspiratory pressure of 26 mm Hg, positive end-expiratory pressure of 6 mm Hg, rate of 40 breaths/min, and FiO2 of 100%. Physical examination reveals central cyanosis, a full anterior fontanelle, II/VI systolic murmur at the left lower sternal border, and slightly diminished breath sounds bilaterally. A right radial arterial blood gas reveals:\n• pH, 7.10\n• PCO2, 70 mm Hg\n• PCO2, 26 mm Hg\n• Base excess, -7 mmol/L\n• Bicarbonate, 22 mmol/L\n\nOf the following, the MOST appropriate next study is", "options" : "[\"chest radiography\", \"echocardiography\", \"head ultrasonography\", \"renal ultrasonography\", \"tracheal aspirate\"]", "explanation" : "A term infant who experiences respiratory failure at birth requires immediate stabilization that begins with the establishment of an airway, effective ventilation, and achievement of adequate circulation. If the infant fails to ventilate or oxygenate, such as the baby described in the vignette, the most useful initial study for evaluation is the standard chest radiograph. A chest radiograph can rapidly exclude mechanical issues such as incorrect endotracheal tube placement, pneumothorax, or pleural effusion, while allowing assessment of situs, cardiac size and shape, pulmonary vascularity, and parenchymal disease.\n\nIf the chest radiograph does not reveal a mechanical issue contributing to ineffective ventilation or oxygenation, both pulmonary and nonpulmonary causes must be pursued. A complete blood count with differential count, blood culture, serum glucose, serum lactate, and tracheal aspirate may be useful in the evaluation of infectious or metabolic causes. Simultaneous preductal and postductal oximetry can allow the assessment of persistent pulmonary hypertension, with a preductal saturation of at least 10% greater than the postductal saturation suggesting right-to-left shunting of cardiac blood flow through the patent foramen ovale and patent ductus arteriosus. Conversely, a postductal saturation at least 10% greater than the preductal saturation can suggest cyanotic heart disease such as transposition of the great vessels. Echocardiography is the definitive study to evaluate for structural heart disease or pulmonary hypertension at the bedside, but it may not be immediately available.\n\nPneumonia, amniotic fluid aspiration, meconium aspiration, and respiratory distress syndrome often appear on the chest radiograph as patchy infiltrates, atelectasis, or almost complete \"white-out\". Term infants whose chest radiographs are consistent with respiratory distress syndrome and who fail to improve despite mechanical ventilation, oxygen, and repeated surfactant administration should have bronchoalveolar lavage sent for assessment of surfactant protein B deficiency. Computed tomography scan may be helpful if developmental lung abnormalities, such as congenital cystic adenomatoid malformation, are suggested by radiographic findings. A relatively clear chest radiograph may be seen with pulmonary hypertension, pulmonary hypoplasia, and congenital lymphangiectasia. Oligohydramnios and suspected pulmonary hypoplasia may be associated with renal dysplasia, prompting evaluation with renal ultrasonography.\n\nIf the infant fails to respond to aggressive respiratory management, extracorporeal membrane oxygenation (ECMO) may be considered. Head ultrasonography to evaluate for intracranial hemorrhage should be performed before cannulization because the heparinization on ECMO may lead to extension of the hemorrhage. Lung biopsy may be considered to rule out congenital lymphangiectasia and capillary alveolar dysplasia if the infant fails to improve on ECMO.\n\nAmerican Board of Pediatrics Content Specification(s): Plan the evaluation of a full-term infant who has severe respiratory failure at birth that does not respond to intubation and assisted ventilation"}
{"id" : 2252, "question_text" : "A 4-month-old infant is seen in the pediatric clinic for noisy breathing. Her parents have noticed this noisy breathing since birth, and they report that the infant has had recurrent respiratory infections. Trials of bronchodilator therapy and inhaled steroids were ineffective. The parents report that she is fussy with feeds and has intermittent episodes of emesis, for which they have tried several different formulas without improvement. A pediatric gastroenterologist recently ordered a barium swallow study; it revealed an anterior indentation of the esophagus. Computed tomography angiography is ordered for additional evaluation. Of the following, the computed tomography angiography is MOST likely to reveal a", "options" : "[\"double aortic arch\", \"left pulmonary artery arising from the right pulmonary artery\", \"right aortic arch\", \"right aortic arch with aberrant left subclavian artery and left-sided ductus arteriosus\"]", "explanation" : "The infant described in the vignette has a pulmonary artery sling (Figure 1), which is characterized by a left pulmonary artery arising from the right pulmonary artery. The barium swallow study (Figure 2) revealed an anterior indentation of the esophagus, which corresponds to the left pulmonary artery coursing posterior to the trachea and anterior to the esophagus before entering the left lung. Pulmonary artery slings are often associated with tracheal stenosis and complete tracheal rings. Therefore, a formal airway evaluation with bronchoscopy is indicated.\nVascular congenital anomalies of the aortic arch (vascular rings and slings) may result in compression of the tracheobronchial tree and esophagus, leading to both respiratory and gastrointestinal symptoms. Normal anatomy includes a left aortic arch. A right aortic arch with mirror-image branching does not cause compression of either the trachea or the esophagus. Vascular rings may be complete or incomplete. Complete vascular rings fully encircle the trachea and esophagus; incomplete vascular rings do not. Both types can cause respiratory and gastrointestinal symptoms. Respiratory symptoms which do not respond to common interventions may include stridor, cough, wheezing, respiratory distress, and recurrent respiratory infections. Intermittent episodes of cyanosis or apnea may also occur. Gastrointestinal symptoms may include feeding difficulties, emesis, dysphagia, and poor weight gain.\nDouble aortic arch and right aortic arch with an aberrant left subclavian artery and left-sided ductus arteriosus are the most common forms of complete vascular ring. In a double aortic arch, the ascending aorta bifurcates anteriorly to the trachea and esophagus and rejoins into a single descending aorta posteriorly to the trachea and esophagus. A right aortic arch with an aberrant left subclavian artery and left-sided ductus arteriosus forms a complete vascular ring with the right-sided aortic arch crossing the trachea anteriorly, the base of the left subclavian artery posteriorly, and ductus arteriosus on the left. A double aortic arch tends to cause earlier and more severe symptoms than a right aortic arch. \nThere are associated anomalies commonly found in children with vascular rings and slings. Cardiac anomalies include tetralogy of Fallot, coarctation of the aorta, patent ductus arteriosus, and ventricular septal defect. Noncardiac anomalies include tracheoesophageal fistula, subglottic stenosis, tracheal stenosis, complete tracheal rings, cleft lip, cleft palate, DiGeorge syndrome (22q11.2 deletion), or CHARGE syndrome (coloboma, heart defect, choanal atresia, restricted growth and development, genital abnormality, and ear abnormality).\nA high index of clinical suspicion is required to diagnose a vascular ring or sling. Physical examination findings vary, depending on the degree of tracheal or esophageal compression. If there is suspicion for a vascular ring or sling, the initial study to perform is a chest radiograph with posterior-anterior and lateral views to assess the location of the aortic arch. If there is still concern for a vascular ring or sling, echocardiography and computed tomography angiography (CTA) are indicated to define the vascular anatomy. Both studies are required due to their individual advantages and disadvantages. Echocardiography is more easily obtained than CTA, and there is no exposure to radiation; however, it will not evaluate the airway, and results may be limited if there are any atretic segments of the vasculature. While CTA does expose the child to radiation, it provides a better assessment of the airway and three-dimensional visualization of the vasculature. \nAirway evaluation with bronchoscopy is not usually required for diagnosis of a vascular ring or sling, but may be helpful to assess the degree and level of tracheal compression, tracheal stenosis, tracheomalacia, or bronchomalacia. This additional information may be helpful with preoperative planning. The exception is a pulmonary artery sling, for which bronchoscopy is almost always performed due to the high incidence of complete tracheal ring. A barium swallow study is not required for diagnosis of a vascular ring or sling; if performed, however, this study may reveal a posterior indentation of the esophagus with all vascular rings (with the exception of a pulmonary artery sling) which produces an anterior indentation.\nAlthough they are not true vascular rings or slings, there are other aortic arch anomalies. An aberrant right subclavian artery is the most common aortic arch anomaly and is usually asymptomatic. Innominate artery compression syndrome occurs when the innominate artery branches more distally along the course of the aortic arch, resulting in a takeoff to the left of the trachea. This causes anterior tracheal compression with associated tracheomalacia. This compression usually improves with age, but in children with severe symptoms, surgical correction is needed, which often includes aortopexy.\nFor most children, the symptoms of these vascular anomalies do not improve over time. The only definitive treatment for symptomatic patients with an aortic arch anomaly is surgery. If surgical intervention is not warranted, regular follow-up is required, as additional or worsening symptoms may occur later in life.\nSuggested Reading(s)\nBacker CL, Mongé MC, Popescu A, Eltayeb OM, Rastatter JC, Rigsby CK. Vascular rings. Sem Pediatr Surg. 2016;25(3):165-175. doi:10.1053/j.sempedsurg.2016.02.009\nConrad C, Cornfield DN. Airway obstruction. In: McInerny TK, Adam HM, Campbell DE, DeWitt TG, Foy JM, Kamat DM, eds. American Academy of Pediatrics Textbook of Pediatric Care. American Academy of Pediatrics; 2021:chap 348. Accessed September 21, 2024. Pediatric Care Online\nHumphrey C, Duncan K, Fletcher S. Decade of experience with vascular rings at a single institution. Pediatrics. 2006;117(5):e903–e908. doi:10.1542/peds.2005-1674\nLicari A, Manca E, Rispoli GA, Mannarino S, Pelizzo G, Marseglia GL. Congenital vascular rings: a clinical challenge for the pediatrician. Pediatr Pulmonol. 50:511-524. doi:10.1002/ppul.23152\nContent Domain\nPulmonology, Congenital malformations\nLearning Objectives\nBe able to identify the most common vascular congenital anomalies of the aortic arch\nLearn to identify the signs and symptoms associated with a vascular ring or sling\nUnderstand which imaging is needed to evaluate a vascular ring or sling"}
{"id" : 1743, "question_text" : "A 3-year-old boy is brought to your office for a health supervision visit. He is a new patient to your practice. Six months ago, he was diagnosed with autism by a developmental pediatrician after he was referred for evaluation of expressive language delay, repetitive stereotyped movements, and abnormal social development. He currently uses less than 10 words. His parents report that he is extremely sensitive to being touched. You are unable to perform a thorough physical examination because he screams and cries when you attempt to touch his body. The boy's previous pediatrician recommended that he have his hearing tested to determine if a hearing deficit is contributing to his developmental delays. His family moved before he was able to have this testing completed, and they are interested in testing at this time. Of the following, the MOST appropriate initial test for this patient is", "options" : "[\"auditory brainstem response testing\", \"pure-tone audiometry\", \"speech audiometry\", \"tympanometry\", \"visual reinforcement audiometry\"]", "explanation" : "Although the child in this vignette is 3 years old, he has autism, very limited verbal skills, and is resistant to examination and physical contact with others. The best initial hearing test for him would be visual reinforcement audiometry. He would likely need to be sedated to undergo auditory brainstem response testing, making this an inappropriate first test. With his limited communication and cooperation, pure-tone and speech audiometry would not be feasible. Tympanometry is not appropriate because it is not a hearing test.\n\nPediatric hearing loss is a common and often under-recognized issue. Early identification and prompt intervention are associated with improved language and developmental outcomes. All states have universal newborn hearing screening programs, which aim to identify children with permanent congenital hearing loss by 3 months of age and begin interventions by 6 months of age. Universal newborn hearing screening programs use either otoacoustic emission (OAE) or auditory brainstem response testing.\n\nDuring OAE testing, a speaker is placed in the ear canal. Sound triggers the cochlea to respond; this response can be sensed by a recorder within the ear canal insert. The OAEs serve as a surrogate marker for a normally functioning cochlea. This test is quick, inexpensive, and does not require the infant to be cooperative or sedated. Results can be affected by cerumen, fluid, or debris in the external ear canal or the middle ear. Auditory neuropathies or other neuronal problems are missed by OAE testing because the function of the auditory nerve is not measured. Auditory brainstem response testing measures electrical activity along the auditory nerve. Electrodes are placed on the scalp to record the electrical activity as speakers in the ear canal emit clicks or tones. Results can be affected by movement; newborns are best tested when sleeping, and older children may require sedation for an accurate test. Results can also be affected by outer or middle ear conditions, although less so than in OAE testing.\n\nBoth OAE and auditory brainstem response testing evaluate portions of the auditory pathway but are not true tests of hearing. Hearing cannot definitively be considered normal until a reliable audiogram can be obtained. Audiometry evaluates hearing thresholds at a variety of frequencies and can determine the degree and type of hearing loss. Ears can be tested separately and simultaneously. Various types of audiometric testing can be used with patients of different ages (Item C220A).\n\nAlthough not a hearing test per se, tympanometry can be used to assess the function of the outer and middle ear. During this test, a small probe is inserted into the ear canal, forming a seal. This probe measures pressure and can identify normal middle ear pressure, decreased tympanic membrane mobility, or tympanic membrane retraction caused by eustachian tube dysfunction. Beyond the newborn period, children with risk factors for hearing loss should have ongoing screening as well as a formal diagnostic audiology assessment by 24 to 30 months of age. All children should have periodic hearing screening, and any parental concern for hearing loss should be taken seriously. Risk factors for hearing loss are shown in Item C220B.\n\nPREP Pearls\n• Universal newborn hearing screening is performed with otoacoustic emission testing, which measures a response from the cochlea, or auditory brainstem response testing, which measures electrical activity along the acoustic nerve.\n• Otoacoustic emission testing and auditory brainstem response testing evaluate portions of the auditory pathway but are not true tests of hearing.\n• Visual reinforcement/behavioral audiometry, play audiometry, and conventional audiometry evaluate hearing thresholds at various frequencies and can determine the degree and type of hearing loss.\n\nMOCA-Peds Objective\n• Evaluate and manage the behavioral complications of autism spectrum disorder\n\nABP Content Specifications(s)\n• Understand the indications for and limitations of standard audiology tests (including acoustic emissions, tympanometry, auditory brainstem response, and behavioral audiometry) and be able to interpret their results\n• Plan the age-appropriate initial and follow-up evaluation of hearing loss of various etiologies\n\nSuggested Readings\n• Grindle CR. Pediatric hearing loss. Pediatr Rev. 2014;35(11):456–463. doi: http://dx.doi.org/10.1542/pir.35-11-456.\n• Harlor AD Jr, Bower C; Committee on Practice and Ambulatory Medicine; Section on Otolaryngology-Head and Neck Surgery. Hearing assessment in infants and children: recommendations beyond neonatal screening. Pediatrics. 2009;124(4):1252–1263. doi: http://dx.doi.org/10.1542/peds.2009-1997."}
{"id" : 919, "question_text" : "You are seeing a 3-year-old girl after hospital discharge. The girl was seen in the emergency department yesterday after she developed an erythematous, itchy, raised, blotchy rash, marked swelling of the eyes and lips, and raspy breathing. According to the mother, the child was given injectable epinephrine, diphenhydramine, and systemic corticosteroids. Although the symptoms improved after a couple of hours, the child was admitted overnight for observation. The girl's mother is shaken by this event and would like you to determine what triggered this reaction.\n\nOf the following, the MOST common triggers for events such as those experienced by the girl are", "options" : "[\"aeroallergens, contact allergens, latex, cleaning agents\", \"foods, medications, hereditary angioedema, idiopathic triggers\", \"infections (viral or bacterial), foods, medications, insect stings\", \"mast cell disorders, hereditary angioedema, urticarial vasculitis\", \"nonsteroidal anti-inflammatory drugs, antibiotics, narcotics, and radiocontrast media\"]", "explanation" : "The child described in this vignette had an episode of acute urticaria/angioedema. The most common causes of acute urticaria are infections (viral or bacterial), foods, medications, and insect sting reactions. The child had symptoms and signs of urticaria (erythematous, itchy, raised, blotchy rash) as well as angioedema (marked swelling of the eyes and lips). Her raspy breathing could have been due to pharyngolaryngeal angioedema or a sign of bronchospasm suggesting impending anaphylaxis. The epinephrine followed by diphenhydramine and corticosteroids treats both conditions simultaneously. The absence of cardiovascular (eg, hypotension) and respiratory (wheezing) decompensation and additional systemic signs helps provide reassurance against the diagnosis of anaphylaxis.\n\nAlthough no specific cause can be identified in many patients, acute urticaria is more likely to have an identifiable etiology then chronic urticaria (>6 weeks' duration). Viral or bacterial infections account for greater than 80% of cases, particularly in children. IgE-mediated, allergic reactions causing acute urticaria can be triggered by medications, stinging insects, foods and food additives, aeroallergens, contact allergens, latex, or blood products. Allergic reactions may be limited to the skin or be a part of a systemic allergic reaction (ie, anaphylaxis). Generalized urticaria or angioedema following exposure to a potential allergen should be interpreted as a systemic reaction with an increased risk of anaphylaxis with subsequent exposure. The antibiotics most frequently implicated in causing IgE-mediated urticaria include beta-lactams (penicillins and cephalosporins), although antibiotics from virtually all classes have been reported. Certain drugs cause urticaria owing to mast cell degranulation through a nonIgE-mediated mechanism. The most frequently implicated are narcotics, muscle relaxants, vancomycin, and radiocontrast medium. Nonsteroidal anti-inflammatory drugs, such as aspirin, ibuprofen, naproxen sodium, and others can trigger urticaria and/or angioedema owing to both allergic and nonallergic mechanisms.\n\nAcross all age groups, the most common triggers for anaphylaxis are ingested foods (33%), insect stings (19%), and medications (14%). Less common triggers include cats, latex, cleaning agents, environmental allergens, and exercise. For approximately one-quarter of cases, the trigger is unknown. In children, food-induced anaphylaxis is the most common trigger and accounts for 37% to 85% of cases, whereas insect bites/stings account for 5% to 13% and medications account for 5% to 12%.\n\nAcute angioedema that occurs in association with urticaria is similar in pathology to urticaria, although it takes place in the deeper levels of the dermis and subcutaneous tissues. Angioedema that is mast cell-mediated is associated with urticaria and/or pruritus in most cases. Urticarial vasculitis, on the other hand, should be suspected when the urticarial lesions are painful, last for more than 72 hours at a location, are purplish or discolored, and cause scarring. Hereditary angioedema should be considered when angioedema occurs in the absence of urticaria; occurs at sites of trauma or around the mouth and extremities; is preceded by the appearance of a transient, serpiginous rash; occurs with abdominal cramping suggesting bowel edema; requires prolonged treatment; or occurs with a family history of similar symptoms. The presence of urticaria pigmentosa, solitary reddish-brown lesions that urticate on stroking (Darier sign), or of persistently elevated tryptase should raise concern regarding underlying mast cell disorders.\n\nPREP Pearls\n• Viral or bacterial infections are commonly associated with acute urticaria in children.\n• Acute urticaria can be triggered by medications, stinging insects, foods and food additives, aeroallergens, contact allergens, latex, or blood products.\n• In children, anaphylaxis is most commonly triggered by foods, insect stings, and medications.\n\nAmerican Board of Pediatrics Content Specification(s):\n• Know the etiologic agents that commonly cause urticaria/angioedema/anaphylaxis\n\nSuggested Reading:\n• Lieberman P, Nicklas RA, Oppenheimer J, et al. The diagnosis and management of anaphylaxis practice parameter: 2010 update [published correction appears in JAllergy Clin Immunol. 2010;126(6):1104]. JAllergy Clin Immunol. 2010;126(3):477-480.e1-42. doi:10.1016/j.jaci.2010.06.022"}
{"id" : 917, "question_text" : "You are seeing a 14-year-old boy in your office for his annual health supervision visit. Over the past 6 months, he has had episodes of epistaxis that are increasing in frequency and duration. Over the past month, the episodes have occurred 3 to 4 times per week. Some have lasted as long as 30 minutes. The boy tells you he has also had increasing difficulty breathing through the left side of his nose over the past 2 months. At today's visit, the boy's height and weight are found to be at the 50th percentile for age. On physical examination, he is in no acute distress. You find no abnormalities of the anterior aspect of his nose. On inspection of the nares using a nasal speculum, you note a mass in the left posterior nasal passage.\n\nOf the following, the diagnostic study that is MOST useful in identifying the underlying cause of the boy's epistaxis is", "options" : "[\"coagulation studies\", \"complete blood cell count with differential\", \"computed tomography of the sinuses\", \"serum immunoglobulin E\", \"sweat chloride test\"]", "explanation" : "The symptoms and examination findings for the male teenager in the vignette highly suggest juvenile nasopharyngeal angiofibroma (JNA) as the etiology of his epistaxis. Computed tomography of the sinuses would be the most useful diagnostic study for this patient.\n\nJuvenile nasopharyngeal angiofibroma is a benign tumor and a rare but important cause of epistaxis in prepubertal and adolescent males, accounting for 0.05% of all head and neck tumors. It occurs almost exclusively in males in the first or second decade of life, with a mean age of onset of 15 years, and must be considered in boys with recurrent epistaxis with associated nasal obstruction. A hormonally mediated origin has been suggested for development of JNA.\n\nJuvenile nasopharyngeal angiofibroma lesions originate close to the posterior attachment of the middle turbinate, near the superior border of the sphenopalatine foramen. These lesions derive their blood supply from the internal maxillary artery. Although a histologically benign tumor, JNA is highly vascular and can result in severe epistaxis. Furthermore, the tumor can cause severe problems through local invasion of adjacent structures.\n\nThe most common presenting symptoms found in boys with JNA are nasal obstruction, recurrent unilateral epistaxis, nasal drainage, headache, and facial swelling. The most common signs identified on physical examination include unilateral nasal mass, orbital mass, and proptosis.\n\nWhen suspected, JNA can be confirmed by contrast-enhanced computed tomography or magnetic resonance imaging. These studies are indicated in children with epistaxis if a nasopharyngeal mass is visualized or suspected; in addition to confirming JNA, these diagnostic studies can identify and delineate other types of neoplasms of the nasal cavity, including rhabdomyosarcomas and nasopharyngeal carcinomas.\n\nThe differential diagnosis for epistaxis in children includes causes ranging from self-limited mucosal irritation to life-threatening neoplasms. The most common causes of epistaxis in children include mucosal dryness, local trauma, nasal foreign body, and rhinitis (allergic or infectious). Less common but extremely important underlying causes of epistaxis include bleeding disorders, medications, illicit drugs use, neoplasms, inflammatory disorders, and hypertension. Children with systemic causes of epistaxis rarely present with epistaxis as the only clinical manifestation.\n\nAlthough obtaining coagulation studies and a complete blood cell count with differential would not be unreasonable in this adolescent with recurrent epistaxis, these studies would not identify the underlying problem for this boy. The finding of a unilateral nasal mass and the patient's report of progressive nasal obstruction should raise clinical suspicion for JNA. The history of unilateral, rather than bilateral, epistaxis further indicates an isolated nasopharyngeal lesion rather than coagulopathy, thrombocytopenia, or other systemic disease. Nasal cavity neoplasms in children may include benign lesions (such as JNA, hemangioma, pyogenic granuloma, and inverting papilloma) and rare but extremely important malignant tumors, such as rhabdomyosarcomas and nasopharyngeal carcinomas.\n\nTesting for elevated serum immunoglobulin E level, which can be an indicator of allergy-related disease, is typically not included in the initial evaluation of a child with recurrent or severe nosebleeds.\n\nA sweat chloride test to confirm the diagnosis of cystic fibrosis would not be indicated in this patient. The boy's normal height and weight and absence of significant prior illnesses are inconsistent with the diagnosis of underlying cystic fibrosis.\n\nPREP Pearls\n• The diagnosis of JNA should be considered in prepubescent and adolescent males with recurrent unilateral epistaxis and nasal obstruction.\n• Systemic conditions that may result in epistaxis include bleeding disorders, medication effects, hypertension, infectious and inflammatory disorders, and malignant tumors.\n• Radiologic imagings are indicated in children presenting with epistaxis and a nasopharyngeal mass lesion.\n\nAmerican Board of Pediatrics Content Specification(s):\n• Understand the evaluation of a child with severe recurrent epistaxis\n• Know the differential diagnosis of epistaxis\n\nSuggested Reading:\n• Messner AH. Epidemiology and etiology of epistaxis in children. UPtoDate. Available online only for subscription.\n• Messner AH. Evaluation of epistaxis in children. UPtoDate. Available online only for subscription."}
{"id" : 3222, "question_text" : "You are asked to see a 3-week-old neonate who has just been hospitalized because of an apparent life-threatening event. He was delivered at term following an uncomplicated pregnancy. He weighed 2,800 g. He was started on a soy protein-based formula at birth because of a family history of allergy to cow-milk protein. At about 2 weeks of age, he developed postprandial emesis and was switched to a hydrolyzed protein-based formula. However, the vomiting episodes have persisted, and the baby spits up once or twice after each feeding. He currently consumes 6 oz of formula every 3 hours to 4 hours. Several hours ago, immediately after feeding, the baby appeared to be choking, stopped breathing, and developed perioral cyanosis. His mother slapped him on his back and formula was expelled from his nose and mouth. The parents called 911, and the infant was brought to the emergency department, where he appears active and alert. His weight is 3,700 g, temperature is 37.0°C, respiratory rate is 30 breaths/min, pulse rate is 130 beats/min, and oxygen saturation is 99% on room air. Physical examination findings are unremarkable. In the emergency department, blood, urine, and spinal fluid cultures are obtained, and he is started on intravenous antibiotics and admitted for observation and continued care. Of the following, the MOST appropriate next step is", "options" : "[\"a barium upper gastrointestinal tract series\", \"intraesophageal pH monitoring\", \"reduced feeding volumes\", \"treatment with an H2-receptor antagonist\", \"treatment with a proton-pump inhibitor\"]", "explanation" : "Preferred Response: C\nIn the vignette, a 3-week-old newborn presents with an apparent life-threatening event. This is an observer-dependent phenomenon, characterized by a combination of apnea, abnormal muscle tone (limpness, rigidity), choking, and color change (pallor, cyanosis, plethora). Apparent life-threatening events typically are reported in infants at 1 to 2 months of age, and are rarely described after 8 months of age. Although specific etiologies are poorly understood, gastroesophageal reflux (GER) has been causally implicated. However, supportive data are conflicting, and the relationship between apnea and GER remains controversial. Accordingly, in this infant whose clinical history otherwise suggests a diagnosis of uncomplicated GER, which may be exacerbated by overfeeding, current best evidence indicates that the most appropriate approach to management would include a reduction in feeding volumes.\n\nGastroesophageal reflux is defined by the passage of gastric contents into the esophagus, with or without regurgitation or vomiting. It is a normal physiologic process occurring several times per day in healthy infants, children, and adults. Most episodes are transient, lasting less than 3 minutes, occurring in the initial 2 hours postprandially, and followed by rapid esophageal clearance of the refluxate. These brief, recurring GER events are generally unassociated with clinical signs or symptoms. During the first 3 months after birth, regurgitation (defined as effortless passage of gastric contents into the mouth) or vomiting events (which may be projectile) are noted daily in 50% of infants. These GER episodes arise from several potential anatomic and physiologic mechanisms, the most prominent being repeated, transient relaxation of the lower esophageal sphincter (LES). Available reports show that 60% to 70% of infants experience more than 1 episode of emesis per day by 3 to 4 months of age. Resolution of GER symptoms occurs in most infants by 1 year of age. The emesis prevalence rate decreases to 10% by 7 to 9 months of age and to 5% by 10 to 12 months of age. As in the vignette, a diagnosis of GER in infants is based solely on a history of spitting-up gastric contents. Formal intraesophageal pH monitoring is of no value in routine evaluation. This study should be reserved for assessing equivocal reflux cases, or in an attempt to correlate symptoms with reflux episodes. A barium esophagram cannot quantify the extent or severity of GER, and is of use solely to document anatomic integrity.\n\nSymptoms and signs that have been associated with prolonged or increased GER include both respiratory and nonrespiratory events, as listed in Item C159. Although regurgitation or vomiting are not consistent findings in older patients with GER-related complications, spitting-up, with or without expulsion of gastric contents from the mouth, is considered a necessary condition for reaching a clinical diagnosis of reflux during infancy.\n\nEarly studies suggest that a potential mechanism for reflux induced apnea involved acid stimulation of pharyngeal and esophageal chemoreceptors, leading to laryngospasm. However, more recently, large case series have failed to demonstrate a consistent GER-apnea link. Where respiratory status was monitored along with both esophageal pH and bioelectrical impedance in infants presenting with a history of apnea, only 15% of apneic episodes were correlated with GER. Furthermore, these episodes were as likely to occur with nonacid as with acid GER. Additional data have failed to demonstrate any clinical efficacy of acid reduction therapy in preventing or ameliorating apnea events. Thus, acid blockade with either an H2-receptor antagonist or a proton pump inhibitor is not indicated in this clinical setting.\n\nGastroesophageal reflux has also been implicated as a causative or exacerbating factor for other respiratory disorders. When studied using esophageal pH monitoring, 60% to 70% of children with reactive airway disease demonstrate pathological reflux. However, it is unclear whether this degree of GER is a primary problem or a secondary phenomenon caused by lung hyperinflation and downward movement of the diaphragm leading to upward displacement of the LES into the chest, accompanied by a reduction in LES pressure. Several studies have attempted to assess the role of acid reduction therapy in ameliorating asthma symptoms in both children and adults, and most have failed to demonstrate consistent treatment efficacy. However, a few reports have shown improvement in asthma symptoms following acid blockade for patients with poorly controlled asthma, particularly those subjects manifesting predominantly nocturnal symptoms. Gastroesophageal reflux has been implicated in the etiology of recurrent pneumonia and interstitial lung disease. Although these complications have been reported in otherwise healthy infants and children, they are common problems in children with significant neurological impairment. In these cases, lung disease is presumably the consequence of failure of normal airway mechanisms to protect the lungs from aspirated gastric contents. Several case series have presented conflicting results regarding the efficacy of either medical or surgical GER therapy in improving lung function and reducing the risk of pneumonia in affected patients.\n\nGastroesophageal and respiratory function are closely linked, and therefore the finding of pathological GER in patients with chronic pulmonary disease is not surprising. In 1 report, 27% patients with cystic fibrosis (CF) reported symptoms of heartburn. However, when studied by esophageal pH monitoring, the prevalence of pathological GER in CF was even higher. Accordingly, GER may be considered an exacerbating factor for children and adults with a wide range of chronic respiratory disorders. Again, the effectiveness of acid blockade on respiratory symptoms in these patients has not been clearly demonstrated.\n\nPREP Pearls\n• During infancy, the presence of emesis or regurgitation is required to make a diagnosis of gastroesophageal reflux.\n• A clear relationship between gastroesophageal reflux and apnea has not been established, and acid suppression has not been shown to alter the clinical course related to apnea during infancy.\n• In patients with poorly controlled asthma, acid suppression therapy may be useful in the setting of predominantly nocturnal asthmatic symptoms.\n\nAmerican Board of Pediatrics Content Specification(s)\n• Recognize the clinical features associated with gastroesophageal reflux, including symptoms associated with other organ systems (eg, respiratory)\n• Understand the prognosis for patients who have gastroesophageal reflux\n\nSuggested Reading\n• Akinola E, Rosenkrantz TS, Pappagallo M, McKay K, Hussain N. Gastroesophagea/ reflux in infants c 32 weeks gestational age at birth: lack of relationship to chronic lung disease. Am f Perinatol. 2004;21(2):57-62. doi:10.1055/s-2004-820512.\n• American Academy of Pediatrics. EQIPP. GER or GERD? Diagnosis and management. 2014. Expires 5/1/2017.\n• Condino AA, Soridheimer 1, Pan 2, Gralla 1, Perry 0, O'Connor IA. Evaluation of gastroesophageal reflux in pediatric patients with asthma using impedance-pH monitoring. J Pediarr. 2006;149(2):216-219. doi:10.1016/j4peds.2006.03.022.\n• Mousa H, Woodley FW, Metheney M, Hayes I. Testing the association between gastroesophageal reflux and apnea in infants. f Pediarr Gastroenterol Nutt 2005;41(2):169-177.\n• Stordal K, Johannesdottir GB, Bentsen BS, et at. Acid suppression does not change respiratory symptoms in children with asthma and gastro -oesophageal reflux disease. Arch Dis Child. 2005;9G(9):956-960. doi:10.1136/adc.2004.068890.\n• Vandenplas 1/, Hauser B. Gastro-oesophageal reflux, sleep pattern, apparent life threatening event and sudden infant death: the point of view of a gastro-enterologist. Eur f Pediarr. 2000;159(10726-729. doi:10.1007/ s004310000544.\n• Vandenplas Y, Rudolph CD, Di Lorenzo C, et al. Pediatric gastroesophageal reflux clinical practice guidelines: joint recommendations of the North American Society for Pediatric Gastroenterology, Hepatology, and Nutrition (NASPGHAN) and the European Society for Pediatric Gastroenterology, Hepatology, and Nutrition (ESPGHAN). J Pediatr Gastroenteral IVutr. 2009;49(0498-547. doi:10.1097/MPG.0b013e3181b7f563."}
{"id" : 2579, "question_text" : "A 14-year-old adolescent girl is seen in the office for follow up of dysmenorrhea. Based on the results of laboratory evaluation performed at her initial visit for this condition 9 months ago, she was diagnosed with iron-deficiency anemia and type 2 diabetes (hemoglobin A1C, 6.6%). Today, she reports ongoing heavy and painful menstrual cycles as well as increasing fatigue; she is otherwise well. She has never had sexual intercourse. The girl's vital signs are normal. Her body mass index has increased over the past 3 months from 98% to 99% for age and sex. Her current prescribed medications are ferrous sulfate 325 mg every other day, naproxen sodium 440 mg twice daily at the time of her menses, and metformin 1,000 mg twice daily. Laboratory data are shown: Laboratory Test Result Hemoglobin 9.3 g/dL (93 g/L) Mean corpuscular volume 78 fL Mean corpuscular hemoglobin concentration 34.2 g/dL (342 g/L) Ferritin 2 ng/mL (2 µg/L) Hemoglobin A1C 6.7% Of the following, the BEST next step in this girl's management is", "options" : "[\"assess adherence by asking open-ended questions\", \"decrease the dose of metformin for 2 weeks\", \"increase her dose of ferrous sulfate to 325 mg daily\", \"refer her to a hematologist for possible iron infusion therapy\"]", "explanation" : "Correct Answers: A\nThe adolescent in the vignette has recently been diagnosed with chronic medical conditions that require daily medications for long-term management. Medication adherence is difficult for adolescents, especially if parents are not monitoring medications closely to increase accountability. The best next step in the management of the girl in the vignette is to assess medication adherence by asking her open-ended questions. For example, \"Are you in charge of your own medications?\" \"Do you ever accidentally forget to take them?\" \"Do any of them cause you problems?\" The physician should not increase or decrease the dosage of her medications until it is assessed if the patient is taking their medications correctly. While referral to hematology for iron infusion therapy may be needed at some point, this step should not occur before open-ended inquiry regarding her medication adherence.\n\nWhile adolescents are developing autonomy and independence and are able to take some responsibility for their actions, their struggle with peer factors and a desire to be \"normal\" may decrease adherence to medication or medical recommendations. Data show that adolescents who are solely responsible for their own medications are less adherent than those who share responsibility with their parents. Shared decision-making among adolescents, their parents, and their physicians is an important aspect of chronic medical condition management, including adherence. Physicians should work to minimize conflict between caregivers and adolescents and develop treatment plans as a team.\n\nBarriers to adherence include transition times (eg, young adults transitioning to adult services), prolonged intervals between appointments, lack of knowledge regarding medication uses, systems issues (eg, pharmacy or insurance problems), guilt regarding missed doses, and medication side effects. The World Health Organization published a report on adherence that outlines dimensions of adherence in 5 broad categories. The report highlights the importance of promoting support (strengths-based practice) instead of blame and recognition of the negative effect of poverty (and other social determinants of health) on adherence.\n\nPREP Pearls\n• Adherence to medications and medical treatment plans can be challenging for adolescents due to various factors including peer factors, striving for \"normalcy,\" forgetting the medication, physical well-being (eg, side effect concerns), and parent conflict.\n• Physicians can assess medication adherence with open-ended, nonjudgmental questions.\n• Physicians should participate as team members with parents and adolescents to problem solve how to improve adherence.\n\nABP Content Specifications(s)\n• Understand factors that can affect adherence to health maintenance activities by adolescents\n• Understand how to improve adherence to medical regimens by adolescent patients, including those with chronic illness, and the barriers to such adherence\n\nSuggested Readings\n• Hanghøj S, Boisen KA. Self-reported barriers to medication adherence among chronically ill adolescents: a systematic review. J Adol Health. 2014;54(2):121-138. doi:10.1016/j.jadohealth.2013.08.009.\n• World Health Organization. Improving adherence rates: guidance for countries. Geneva, Switzerland:2003."}
{"id" : 359, "question_text" : "The mother of an 8-year-old boy in whom you diagnosed type 1 diabetes 2 months ago calls your office for advice. There has been a gastroenteritis outbreak in the boy's school, and 2 days ago he developed fever, vomiting, and diarrhea. He no longer is interested in eating, although he is able to drink. She reports his blood glucose measurement as 205 mg/dL (11.4 mmol/L). His insulin regimen includes 15 units of glargine insulin administered daily at bedtime and aspart insulin administered before meals (1 unit per 15 g carbohydrate) and for correction of high blood glucose (1 unit for every 25 mg/dL [1.39 mmol/L] above 125 mg/dL [6.9 mmol/L]). Of the following, the MOST appropriate next step is to", "options" : "[\"administer 0.1 units/kg of regular insulin subcutaneously\", \"administer 0.1 units/kg per hour of regular insulin intravenously\", \"administer 4 units of subcutaneous aspart insulin based on his blood glucose\", \"check for urine ketones\", \"discontinue glargine until he is able to eat normally\"]", "explanation" : "The primary defect in type 1 diabetes is the complete destruction of insulin-producing beta cells. Because affected patients manufacture no endogenous insulin, they require exogenous insulin to prevent fatty acid metabolism (and the resulting ketoacidosis), regardless of their blood glucose value. Accordingly, patients who have type 1 diabetes must always take insulin, even if they are fasting. Furthermore, whenever an affected child has a vomiting illness, such as the boy described in the vignette, urine must be assessed for ketones to be sure that ketosis is not occurring. This boy has mild hyperglycemia (blood glucose of 205 mg/dL [11.4 mmol/L]) and is using a glargine-based regimen. Because glargine is a 24-hour-acting insulin with a minimal peak effect, his risk of hypoglycemia is minimal. The boy should continue on his usual glargine dose and check his urine for ketones. Regardless of blood glucose values, the presence of more than trace ketones in the urine always indicates a physiologic need for more insulin.\n\nDiscontinuing the boy's glargine insulin could result in severe ketoacidosis once his previous glargine dose is metabolized. He does not require additional regular or rapid-acting (aspart) insulin at this point because his blood glucose is only mildly elevated and he is not eating. If he does have evidence of ketones, 15% to 25% of his total daily dose of insulin should be administered as an additional subcutaneous injection of regular or rapid-acting insulin to stop further ketosis. Children who have ketosis and are unable to consume carbohydrates may need intravenous fluids and dextrose to maintain normal blood glucose concentrations while they receive additional insulin to resolve the ketosis.\n\nCritique: The primary defect in type 1 diabetes is the complete destruction of insulin-producing beta cells. Because affected patients manufacture no endogenous insulin, they require exogenous insulin to prevent fatty acid metabolism (and the resulting ketoacidosis), regardless of their blood glucose value. Accordingly, patients who have type 1 diabetes must always take insulin, even if they are fasting. Furthermore, whenever an affected child has a vomiting illness, such as the boy described in the vignette, urine must be assessed for ketones to be sure that ketosis is not occurring. This boy has mild hyperglycemia (blood glucose of 205 mg/dL [11.4 mmol/L]) and is using a glargine-based regimen. Because glargine is a 24-hour-acting insulin with a minimal peak effect, his risk of hypoglycemia is minimal. The boy should continue on his usual glargine dose and check his urine for ketones. Regardless of blood glucose values, the presence of more than trace ketones in the urine always indicates a physiologic need for more insulin.\n\nContent Specifications: Counsel patients on the self-management of type 1 diabetes (hyperglycemia, sick days)"}
{"id" : 3736, "question_text" : "An 8-year-old boy was referred to an allergist for evaluation of wheezing with viral respiratory infections and frequent cough and wheezing with exercise. He wakes up frequently at night because of his cough, requiring treatment with an albuterol inhaler before he can go back to sleep. He uses this inhaler several additional times every week. He has a long history of nasal congestion, rhinorrhea, and sneezing, especially during the spring pollen season. There is a family history of asthma and inhalant allergies. The allergist report notes that skin tests were positive for cat, dog, house dust mite, grass pollen, and maple tree pollen. The family has a dog that sleeps in the boy's bed. His mother asks if she needs to keep the dog out of the boy's bedroom. Of the following, the MOST accurate statement in response to the mother's question is", "options" : "[\"based on the boy's symptoms, his exposure to pollen is a more likely precipitant for his asthma\", \"because the boy does not get acute symptoms in response to a specific exposure, it is not likely to be causing his symptoms\", \"regardless of obvious cause and effect related to exposure, it is appropriate to avoid known allergens\", \"this change would have a detrimental psychological impact on the child\"]", "explanation" : "Correct Answer: C\nThere are multiple potential triggers for this boy's asthma, and the first treatment measure should be avoidance of known allergens. The prevalence of nocturnal symptoms and the presence of house dust mite and dog exposures in his bedroom make these highly suspect as allergy triggers for his asthma symptoms. Spring pollen exposure may be a seasonal contributor, but it is not likely the primary trigger for his persistent asthma symptoms. An acute dose/response reaction is not necessary to identify allergens in the context of daily or continual exposure, such as occurs with indoor perennial allergens. Although viral respiratory infections are the most common precipitant for acute asthma episodes, chronic allergen exposure is a primary factor in the recurrence and persistence of asthma symptoms in sensitized children. Whether or not removing the dog from the bedroom will have a detrimental psychological effect on the boy is uncertain; however, the potential positive impact of removing this allergen exposure is significant.\n\nPREP Pearls\n• Indoor allergens are common precipitants for perennial asthma symptoms in allergic children.\n• Avoidance of known allergens is the first rule in the control of allergic asthma.\n\nABP Content Specifications(s)\n• Understand the natural history of asthma and the factors that affect it\n\nSuggested Readings\n• Fitzpatrick AM, Bacharier LB, Guilbert TW, et al. Phenotypes of recurrent wheezing in preschool children: identification by latent class analysis and utility in prediction of future exacerbation. J All Clin Immunol Pract. 2018;7(3):915-924.e7. doi:10.1016/j.jaip.2018.09.016.\n• Gaffin JM, Phipatanakul W. The role of indoor allergens in the development of asthma. Curr Opin Allergy Clin Immunol. 2009;9(2):128-135. doi:10.1097/ACI.0b013e32832678b0.\n• Hill VL, Wood PR. Asthma epidemiology, pathophysiology, and initial evaluation. Pediatr Rev. 2009;30(9):331-335. doi:10.1542/pir.30-9-331.\n• Rhee H, Love T, Harrington D, Grape A. Common allergens in urban adolescents and their relationships with asthma control and healthcare utilization. Allergy Asthma Clin Immunol. 2018;14:33. doi:10.1186/s13223-018-0260-y.\n• Salo PM, Cohn RD, Zelden DC. Bedroom allergen exposure beyond house dust mites. Curr Allergy Asthma Rep. 2018;18(10):52. doi:10.1007/s11882-018-0805-7."}
{"id" : 2683, "question_text" : "An 18-month-old boy is brought to the clinic for a routine health supervision visit. He takes a few steps on his own but cannot walk without holding onto someone or something. He tries to eat with a spoon and scribbles. He points to show the physician something interesting, puts hands out to be washed, looks at a few pages in a book with the reader, and helps with dressing by pushing his arm through a sleeve. The boy tries to say 3 or more words besides \"mama\" and \"dada\" and follows 1-step directions without any gestures. Of the following, this boy most likely has developmental DELAY in", "options" : "[\"fine motor skills\", \"gross motor skills\", \"language-communication skills\", \"social-emotional skills\"]", "explanation" : "Correct Answer: B\nThe boy in the vignette is 18 months old. At this age, expected gross motor skills include walking without holding onto anyone or anything and climbing on and off a couch or chair without help. As the boy can take a few steps but cannot walk without holding onto anyone or anything, he most likely has delayed gross motor skills. His skills in the other developmental domains are appropriate for his age.\n\nThe domains of development and their relevant skills are described in Item C175A. Item C175B lists the gross motor, fine motor, cognitive, social-emotional, and language-communication developmental milestones expected for 15- to 24-month-old children.\n\nPREP Pearls\n• The 5 domains of development include fine motor, gross motor, language, cognitive, and social-emotional.\n• At 18 months of age, expected gross motor skills include creeping down stairs, walking without holding onto anything, seating oneself in a small chair, and throwing a ball while standing.\n\nABP Content Specifications(s)\n• Evaluate the motor developmental progress/status of a child at 18 months of age, including recognition of abnormalities\n• Evaluate the cognitive and behavioral developmental progress/status of a child at 18 months of age\n\nSuggested Readings\n• Feigelman S. The first year. In: Kliegman RM, Stanton BF, St Geme JW III, Schor NF, eds. Nelson Textbook of Pediatrics. 21st ed. Elsevier; 2020.\n• Scharf RJ, Scharf GJ, Stroustrup A. Developmental milestones. Pediatr Rev. 2016;37(1):25-38. doi:10.1542/pir.20140103.\n• Zubler JM, Wiggins LD, Macias MM. Evidence-informed milestones for developmental surveillance tools. Pediatrics. 2022;149(3):e2021052138. doi:10.1542/peds.2021-052138."}
{"id" : 3185, "question_text" : "A 15-year-old adolescent girl presents to your office with the complaint of periumbilical abdominal pain. This symptom has persisted for the past year, occurring several times per week at any time of day. The patient denies nocturnal symptoms, weight loss, diarrhea, constipation, and vomiting. Over the past several weeks, she feels \"bloated\" after meals and often experiences postprandial nausea. During the week, she skips breakfast, has an energy bar for lunch, and eats a large dinner at 8:30 in the evening after returning from swimming practice. She has tried her father's antacids, and these have provided minimal relief. Height and weight are at the 40th percentile for age. She is afebrile with other vital signs within the reference range. Physical examination demonstrates a well-developed, well-nourished adolescent with no abnormal physical findings. Of the following, you are MOST likely to recommend", "options" : "[\"changing her eating behavior\", \"a Helicobacter pylori antibody titer\", \"referral for an upper gastrointestinal tract endoscopy\", \"a trial with a proton-pump inhibitor\", \"an upper gastrointestinal tract series\"]", "explanation" : "Preferred Response: A\nAlthough the exact prevalence of chronic abdominal pain in children and adolescents is not known, a review of available data suggests that this complaint accounts for up to 4% of office visits to the primary care pediatrician. One study in adolescents reported that 8% of high school students sought evaluation for abdominal pain during a 1-year period. In the vignette, a 15-year-old girl presents with dyspeptic symptoms of upper abdominal discomfort, bloating, and intermittent nausea. She does not manifest any alarming signs or symptoms that would suggest an organic cause for her complaints. Considering her irregular and \"unbalanced\" dietary habits, a reasonable initial therapeutic approach would be to encourage a change in her eating behavior.\n\nChildren with functional (ie, nonorganic) abdominal pain report symptoms that include isolated abdominal pain without other physical complaints, dyspepsia, irritable bowel syndrome, or abdominal migraine. In other patients, symptom clusters include clinical features of more than 1 type of functional pain disorder (eg, Patients 12 years of age or older with symptoms for at least 12 weeks (not necessarily consecutive) during the previous 12 months.\n1. Persistent pain or discomfort in the upper abdomen.\n2. No evidence that organic disease is likely explaining symptoms.\n3. Dyspepsia is not exclusively relieved by defecation or associated with the onset of a change in stool frequency or character.\n\nFor the adolescent in the vignette, with her absence of warning signs, no evidence suggests that additional laboratory studies are of value in her evaluation. The predictive value of blood tests in functional abdominal pain (with or without warning signs) has not been adequately studied. Infection with Helicobacter pylori should be considered for patients with a positive family history or for those who have emigrated from an endemic area. The H pylori antibody titer is an unreliable and nonspecific test to evaluate H pylori-associated gastroduodenal disease, and endoscopy with biopsy remains the gold standard for diagnosis in pediatric patients. In the absence of a history (ie, family or immigration) suggesting H pylori infection or the presence of warning signs, little evidence suggests that a radiographic study or an endoscopy with biopsy has a significant yield of mucosal abnormalities. Empiric acid suppression treatment with a proton pump inhibitor has not been adequately studied in children with a diagnosis of functional dyspepsia and no evidence-based guidelines for treatment have been established.\n\nPREP Pearls\n• Dyspepsia is defined as upper abdominal discomfort with or without symptoms of nausea, bloating, or borborygmi (belching). It is usually not associated with a significant underlying acid-peptic disorder.\n• The Helicobacter pylori antibody titer is not recommended as a screening test for children with dyspepsia.\n• Blood tests have not been shown to be of value in assessing the child with functional abdominal pain.\n• Patients with functional abdominal pain may present with complaints isolated to the gastrointestinal tract, or they may describe other pain symptoms.\n\nAmerican Board of Pediatrics Content Specification(s)\n• Recognize the significance of dyspepsia in a child with recurrent abdominal pain\n\nSuggested Reading\n• American Academy of Pediatrics Subcommittee on Chronic Abdominal Pain. Chronic abdominal pain in children. Pediatrics. 2005;115(3):812-815. doi:10.1542/peds.2004-2497.\n• Di Lorenzo C, Colletti RB, Lehmann HP, et al. Chronic abdominal pain in children: a technical report of the American Academy of Pediatrics and the North American Society for Pediatric Gastroenterology, Hepatology and Nutrition. J Pediatr Gastroenterol Nutr. 2005;40(3):249-261. Huertas-Ceballos AA, Logan 5, Bennett C, Macarthur C. Dietary interventions for recurrent abdominal pain (RAP) and irritable bowel syndrome (IBS) in childhood. Cochrane Database Syst Rev. 2009;(1):CD003019. doi:10.1002/14651858.CD003019.pub3. Hyams JS, Davis P, Sylvester FA, at al. Dyspepsia in children and adolescents: a prospective study. J Pediatr Gastroenterol Nutr 2000; 30:413418.\n• Rasquin A, Di Lorenzo C, Forbes D, et al. Childhood functional gastrointestinal disorders: child/adolescent. Gastroenterology. 2006; 130(5):1527-1537. doi:10.1053/j.gastro.08.063."}
{"id" : 206, "question_text" : "The mother a 10-year-old girl whom you have been seeing since birth calls you because of concerns that her daughter might have depression. When they arrive at the office, one of your office assistants asks both the mother and child to complete a Child Depression Inventory (CDI). The mother's inventory scores are borderline significant and the child's scores are below the cutoff for depression. When you interview the girl in her mother's presence, she admits to trouble sleeping, difficulty concentrating on her schoolwork, and no longer enjoying school. Physical examination of the quiet but cooperative girl yields normal findings. Of the following, the next BEST step in care is to", "options" : "[\"administer the Patient Health Questionnaire-9 (PHQ-9)\", \"interview the child alone regarding other possible symptoms of depression\", \"prescribe a trial of fluoxetine therapy\", \"reassure the mother that her child does not have depression based on results of the CDI\", \"schedule a follow-up visit in about 1 month for reassessment\"]", "explanation" : "The girl described in the vignette exhibits some symptoms consistent with depression, but results of her CDI are negative for this diagnosis. In this ambiguous situation, more history is needed to determine if the child has depression. Mental health rating scales may assist with screening and monitoring symptoms over time, but they cannot be used to exclude a diagnosis definitively. For example, the child might want to minimize his or her symptoms or may not understand the questions. A clinical depression might be missed unless specific symptoms are elicited in an interview. Interviewing the child alone allows her to share symptoms that she may not want to reveal in her mother's presence.\n\nThe Patient Health Questionnaire-9 (PHQ-9) is a rating scale validated for use in adolescents and adults, and it is not a substitute for a more detailed history from the child. Sending the girl home without a further interview could miss the diagnosis. Initiating a trial of fluoxetine for this girl is not appropriate because neither has a diagnosis been established nor would an apparent positive response to medicine be proof of diagnosis.\n\nAAP Mental Health Competency: Know the limitations (in terms of sensitivity and specificity) of mental health rating scales"}
{"id" : 192, "question_text" : "You are examining a 6-day-old infant who was born at 36-1/7 weeks' gestation to a 19-year-old primigravida by cesarean section due to preterm labor. The mother had no prenatal care. Apgar scores were 7 and 8 at 5 and 10 minutes, respectively. The infant was stable in room air until today, when she developed apnea requiring intubation, bradycardia, and profound hypotension. On physical examination, the intubated infant appears pale. Auscultation of the lungs reveals diffuse rhonchi. The liver is palpable 4 cm below the costal margin. Laboratory findings include: White blood cell count, 5.6x103/mcL (5.6x109/L) with 20% neutrophils, 68% lymphocytes, and 12% monocytes; Hemoglobin, 10 g/dL (100 g/L); Platelet count, 60x103/mcL (60x109/L); Aspartate aminotransferase, 455 units/L; Alanine aminotransferase, 538 units/L. Cerebrospinal fluid examination reveals: White blood cells, 10/mm3 with 80% lymphocytes and 20% monocytes; Red blood cells, 300/mm3; Protein, 89 mg/dL (0.89 g/L); Glucose, 57 mg/dL (3.2 mmol/L). A chest radiograph demonstrates diffuse pneumonitis. Of the following, the test MOST likely to yield the patient's diagnosis is", "options" : "[\"blood culture for virus\", \"cerebrospinal fluid Gram stain\", \"human immunodeficiency virus Western blot\", \"Mycoplasma polymerase chain reaction\", \"rubella serology\"]", "explanation" : "The infant described in the vignette presents in the first postnatal week with clinical sepsis associated with pneumonitis, respiratory failure, hypotension, hepatitis, and leukopenia (for a newborn) accompanied by a lymphocyte predominance. The most likely cause of her illness is viral infection caused by herpes simplex virus (HSV), although adenovirus and enterovirus infections also can occur. A viral culture may grow HSV in 48 to 72 hours in patients with overwhelming sepsis. In addition, serum and cerebrospinal fluid HSV polymerase chain reaction tests should be performed.\n\nMost neonatal HSV disease is acquired in the peripartum period (85%), although postnatal (10%) and congenital (5%) infection also can occur. Peripartum and postnatal disease (together referred to as perinatal) are classified as follows: 1) Disseminated disease, which can involve multiple organ systems such as liver, lung, brain, skin, eye, and adrenal glands 2) Central nervous system (CNS) disease 3) Skin, eye, and mouth (SEM) disease\n\nDisseminated disease accounts for approximately 25% of perinatal HSV infection and occurs, on average, at postnatal days 10 through 12. Patients usually present with viral sepsis characterized by respiratory failure, pneumonitis, hepatitis (often with liver failure), and disseminated intravascular coagulation. CNS involvement is common, occurring in nearly 75% of patients. However, 20% of neonates who have disseminated disease do not have cutaneous vesicles at any point during their illness. Therefore, a high index of suspicion is required to make the diagnosis when vesicles are absent. With the use of intravenous acyclovir, the 12-month mortality for disseminated HSV disease has decreased from 85% to 30%. Patients who have lethargy and severe hepatitis are at highest risk of death. Among those who receive antiviral therapy for disseminated HSV disease, nearly 85% have normal neurologic development.\n\nCNS disease accounts for approximately 30% of perinatal HSV infection and usually presents in the third postnatal week (days 16 through 19). Common clinical manifestations of CNS disease include focal or generalized seizures, bulging fontanelle, lethargy, irritability, poor feeding, and temperature instability. Associated cutaneous vesicles occur in 60% to 70% of patients at some point during the course of illness. Mortality is associated with prematurity and seizures and usually results from severe cerebral destruction, which can involve any and multiple parts of the brain. With antiviral therapy, the mortality rate for CNS HSV disease is 4%, but only 30% of patients have normal neurologic development.\n\nInfants who have SEM HSV disease account for 45% of perinatal HSV infection, and 80% to 85% of affected patients have cutaneous vesicles or lesions (Item C83). By definition, SEM disease is limited to the skin, eyes, or mouth. Mortality associated with SEM disease is negligible, and more than 98% of infants treated with antiviral therapy have normal neurologic development.\n\nNeonatal enteroviral sepsis syndrome is uncommon and usually presents in the first 14 days after birth. Infants may have fever, lethargy, irritability, hepatomegaly, seizures, and bulging fontanelle in addition to other findings. Chest radiography can reveal pulmonary infiltrates. Transaminitis is common, and severe hepatitis can occur. Additional manifestations of severe neonatal enteroviral sepsis can include meningoencephalitis, myocarditis, pancreatitis, and adrenalitis. The mortality rate for infants who have sepsis syndrome involving the liver is greater than 80%.\n\nNeonatal adenoviral sepsis is rare. It usually involves the lung, liver, and brain. Most cases are fatal.\n\nHuman immunodeficiency virus, Mycoplasma, and rubella virus do not produce sepsis syndromes in neonates, obviating the need for associated diagnostic tests in this infant. Cerebrospinal fluid Gram stain is useful for the detection of bacteria but is not helpful for diagnosing viral infection.\n\nCritique: Preferred Response: A\n\nContent Specifications: Recognize the clinical manifestations of herpes simplex virus infection in the neonatal period, and that skin lesions are not always present"}
{"id" : 2523, "question_text" : "A 16-year-old adolescent girl is seen in the emergency department for evaluation of chest pain, shortness of breath, and nausea of 1 day's duration. She describes the pain as sharp, substernal, and constant. She feels nauseated but has not vomited, and has no interest in eating or drinking. The girl's heart rate is 120 beats/min, respiratory rate is 40 breaths/min, blood pressure is 100/70 mm Hg, and oxygen saturation is 94% in room air. Her chest pain is not reproducible by palpation. Cardiac examination reveals a normal S1, normal S2 with a gallop rhythm, and no murmur. There are crackles heard bilaterally on auscultation of her lungs. Her liver is palpated 3 cm below the right costal margin. Her extremities are pale and cool with delayed capillary refill. A chest radiograph (Item Q15A) and electrocardiogram (Item Q15B) are obtained. Of the following, the BEST next step in the adolescent's diagnostic evaluation is", "options" : "[\"cardiac catheterization\", \"cardiac magnetic resonance imaging\", \"transesophageal echocardiography\", \"transthoracic echocardiography\"]", "explanation" : "The adolescent in the vignette has findings significant for tachycardia, a gallop rhythm, bilateral crackles, and hepatomegaly. Chest radiography demonstrates cardiomegaly and pulmonary edema, and low-voltages and abnormal ST segments and T waves are noted on electrocardiography. This constellation of signs and symptoms is consistent with myocarditis, and additional diagnostic testing is warranted. Of the response choices, the best next test would be transthoracic echocardiography.\n\nTransesophageal echocardiography is not recommended for the initial evaluation of myocarditis and is typically not needed in this clinical context. Cardiac magnetic resonance imaging (MRI) can play an important role in the diagnosis of myocarditis, but it would not be recommended for the initial evaluation. Cardiac catheterization may be warranted during the evaluation of heart failure, but it would not be appropriate at this stage of diagnosis.\n\nMyocarditis can present in several ways along a spectrum of severity. Individuals with fulminant myocarditis, the most severe form, are critically ill with poor perfusion, a gallop rhythm, and hepatomegaly. They may experience life-threatening arrhythmias and cardiovascular collapse. Due to the acute onset, the left ventricle may not have had an opportunity to dilate, so chest radiography may not show cardiomegaly nor will echocardiography show a dilated left ventricle, although the cardiac function will be poor. Signs and symptoms of heart failure in the non-fulminant form may include shortness of breath, dyspnea with exertion, nausea, vomiting, and decreased appetite (poor oral intake in infants). Chest radiography will demonstrate cardiomegaly and pulmonary edema. Echocardiography will show poor function and a dilated left ventricle. Dilated cardiomyopathy, as a result of myocarditis causing a \"burned out\" myocardium, presents with a gradual progression of heart failure signs and symptoms. The muscle becomes injured by infection and chronic inflammation, resulting in a thinned out and dilated left ventricle that functions poorly.\n\nIn cases of myocarditis, chest radiography findings can range from normal to cardiomegaly and pulmonary edema, as seen in this girl's imaging study. Echocardiography will show varying degrees of ventricular dysfunction and possibly a pericardial effusion and valvular dysfunction. Electrocardiography should be obtained on all patients with suspected myocarditis. Most affected individuals will be tachycardic and some will have arrhythmias (eg, tachyarrhythmias and heart block). Relatively low voltages are commonly seen, as are abnormal T waves. Rarely, there may be changes suggestive of ischemia with ST segment elevations (Item C15). A cardiac MRI may be used to look for evidence of myocardial inflammation and scarring. Laboratory studies that may prove helpful in the evaluation of myocarditis include troponin, brain-natriuretic peptide, end-organ function studies (eg, blood urea nitrogen, creatinine, transaminases), as well as viral polymerase chain reaction studies and serologies."}
{"id" : 2152, "question_text" : "A 16-year-old adolescent boy with cystic fibrosis and a history of meconium ileus at birth is brought to the emergency department with acute-onset abdominal pain, most prominent in the right lower quadrant, and bilious emesis. He reports no fever, congestion, or increased coughing. He has been adherent to his breathing treatments and his bowel regimen, which consists of daily polyethylene glycol. He endorses that recently he has been having difficulty adhering to his pancreatic enzyme regimen due to starting a new school year and a new after-school job. He reports abdominal discomfort for 1 week. His last bowel movement was 36 hours ago. The Figure shows his abdominal radiograph. Physical examination reveals a well-developed, well-nourished adolescent who is in moderate distress. He is holding an emesis basin containing green vomitus. Pulmonary examination findings are unremarkable. His abdomen is full, tympanic, with high-pitched bowel sounds. No hepatosplenomegaly is appreciated. Of the following, the BEST next step in management of this adolescent's acute condition is", "options" : "[\"appendectomy\", \"hyperosmolar enema\", \"lysis of adhesions\", \"oral hyperosmolar solution\"]", "explanation" : "Correct answer is B\n\nPREP Pearl(s)\nThe differential diagnosis of small bowel obstruction in a patient with cystic fibrosis includes distal intestinal obstruction, constipation, appendicitis, intussusception (gastrointestinal cancer as a potential lead point in adults), adhesions or anastomotic stricture (history of bowel surgery/resection), viral gastrointestinal infection causing ileus, or small bowel bacterial overgrowth.\nDistal intestinal obstruction syndrome that presents as a complete small bowel obstruction should be managed initially with decompression via a nasogastric tube (sump) to low intermittent wall suction.\nDistal intestinal obstruction syndrome can be successfully treated with an oral (for more proximal or partial obstruction) or rectal hyperosmolar solution.\n\nCritique\nIn this patient with cystic fibrosis (CF), the clinical presentation and abdominal radiograph are most consistent with a small bowel obstruction (SBO) secondary to distal intestinal obstruction syndrome (DIOS). With evidence of bilious emesis and no air in the rectal vault, a complete obstruction is suspected. A hyperosmolar enema with diluted diatrizoate is the treatment of choice for DIOS. Diatrizoate is used to loosen mucus plugs and thickened secretions in the distal ileum allowing for the passage of stool and resolution of the obstruction.\n\nIntestinal obstruction often presents with abdominal distention, abdominal pain, and emesis that ranges from nonbilious to bilious. Bilious emesis often indicates a more complete obstruction preventing passage of bile through the gastrointestinal tract. The underlying disease process or surgical history of the patient can point to the etiology.\n\nDespite DIOS being the most common cause of obstruction in CF, it is important to consider a broader differential diagnosis for right lower quadrant pain. For this patient, who has a history of meconium ileus at birth, it is important to know whether surgical resection was required, in which case both adhesions and anastomotic strictures must be considered. In addition, with pain in the right lower quadrant, appendicitis should be on the differential and further imaging such as computed tomography with oral and intravenous contrast should be considered if there is high suspicion. Appendicitis is often missed in patients with CF, resulting in the more complicated presentation of perforation and abscess formation. Intussusception can also occur. In older patients with CF, gastrointestinal cancer should be considered as a potential lead point. Although rare, fibrosing colonopathy should be considered in the setting of a consistent ingestion of high doses of pancreatic enzymes that exceed the maximum recommended dosage of 10,000 units of lipase per kg per day.\n\nThe management of DIOS in the setting of an SBO is to relieve the obstruction and encourage passage of stool. Similar to other presentations of SBO, a large-bore nasogastric tube (sump) connected to low intermittent wall suction can provide immediate relief. Hydration is extremely important, especially if considering treatment with a hyperosmolar solution either per rectum or orally. Usually, hyperosmolar solution is administered every 24 hours until the obstruction has resolved. Often after resolution of the acute obstruction, an oral or nasogastric polyethylene glycol clean-out is performed to ensure complete passage of all thickened stool. Oral hyperosmolar solution is helpful in the setting of higher obstruction in the gastrointestinal tract or when the radiologist is having trouble getting the solution to reflux into the terminal ileum. An oral hyperosmolar enema is preferred for a distal or terminal ileal obstruction. It is important to involve the surgical team from the beginning of a patient's presentation with DIOS as the use of hyperosmolar solution increases the risk for perforation. In addition, if other etiologies such as appendicitis are noted, surgical intervention may be promptly needed.\n\nDiatrizoate must be used with caution due to high risks for perforation, hypernatremia, and massive fluid fluctuations resulting in hypovolemic shock. Most institutions use a solution of diatrizoate that is diluted to one-third or one-fourth strength to minimize the risks. Another potential option is the use of N-acetylcysteine with its mucolytic mechanism of action in which the free sulfhydryl group of the molecule binds to and dissolves the disulfide bonds found in mucoproteins, thereby decreasing viscosity; it can also be used orally or rectally. Use of N-acetylcysteine has its own risks, including elevation of liver enzymes, development of drug-induced liver injury, perforation, hypernatremia, and hypovolemic shock. Although more studies need to be done to determine the best, most efficacious, and safest agent to use for management of DIOS, having options in treatment is important when considering availability of medications at different facilities.\n\nPrevention of DIOS is challenging as even a single episode of DIOS increases the risk of repeat episodes. However, some preventive measures that can be taken include involvement of a multidisciplinary approach involving a gastroenterologist and a dietitian, with a focus on adherence to pancreatic enzyme replacement"}
{"id" : 2415, "question_text" : "A 4-day-old girl born at 36 weeks' gestation is seen in the emergency department for worsening jaundice. Maternal blood type is B positive, and her group B Streptococcus screen results were negative. This is the mother's first child. The neonate was discharged home 48 hours after birth. Her birth weight was 2,750 g; the discharge weight is unknown. Her total bilirubin level at discharge was 11 mg/dL (188.14 µmol/L). She has been breastfeeding every 2 hours at home. Her last stool was in the hospital just before discharge, and her last wet diaper was approximately 10 hours ago. Her weight in the emergency department is 12% below birth weight. On physical examination, the neonate is sleepy but arousable. She is jaundiced, her anterior fontanelle is depressed, and she has dry mucous membranes. The remainder of her examination findings are normal. Results of laboratory tests obtained in the emergency department are shown: Total bilirubin 21.2 mg/dL (362.60 µmol/L), Conjugated bilirubin 0.2 mg/dL (3.42 µmol/L), Sodium 148 mEq/L (148 mmol/L), Hemoglobin 18 g/dL (180 g/L), Reticulocyte count 2.8% (0.03), Blood type B positive, Direct Coombs test Negative. The neonate is admitted to the hospital for further management. Of the following, the BEST next step in this neonate's management is to", "options" : "[\"administer intravenous immunoglobulin\", \"discontinue breastfeeding\", \"obtain a lactation consultation\", \"perform an exchange transfusion\"]", "explanation" : "The best next step in the management of the neonate in the vignette is to obtain a lactation consultation. There are several physiologic factors that place this late preterm infant at increased risk of jaundice, including red blood cell life span (85 days in newborns compared with 120 days in adults), hemoglobin concentration, immaturity of the conjugation and excretion bilirubin enzyme pathway in the liver, and increased enterohepatic circulation. This neonate's weight loss, lack of stooling since discharge, and minimal urine output suggest that there is delayed production of breast milk, which can lead to dehydration and breastfeeding jaundice and increases the risk of significant hyperbilirubinemia.\n\nRehydration and phototherapy (based on the 2022 American Academy of Pediatrics hyperbilirubinemia guidelines) are critical steps in the initial management of this neonate. It is important to provide lactation support to help the mother with successful breastfeeding and pumping (if indicated) and facilitate adequate production of milk.\n\nThere is no indication for this dyad to discontinue breastfeeding. Bottle feeding with pumped breast milk or formula supplementation may be recommended until the neonate demonstrates weight gain. There is no evidence of a hemolytic process; therefore, intravenous immunoglobulin is not indicated. Exchange transfusion is not appropriate for this neonate; she does not have hemolytic disease, and her total bilirubin level is well below the level at which exchange transfusion is indicated.\n\nTerm neonates' bilirubin levels peak at approximately 5 days of age, whereas late preterm neonates' bilirubin levels peak at approximately 7 days of age. Jaundice in late preterm neonates is more common and more severe than in term neonates. Late preterm neonates are at increased risk for physiologic jaundice compared with those born at term, especially if they are exclusively breastfeeding. Late preterm neonates are at risk for neurotoxicity at a lower bilirubin level compared with term neonates."}
{"id" : 2843, "question_text" : "A 2-month-old female infant with poor feeding and failure to gain weight is seen for evaluation. She is fed 24 kcal/oz of appropriately prepared infant formula. She was born at 39 weeks' gestation, and her birth weight was 3 kg. She failed her newborn hearing screening. She had one hospital admission with vomiting and diarrhea at 6 weeks after birth. Her weight is currently at the third percentile, and her height is at the fifth percentile. Her cardiac examination reveals no murmur, and her chest is clear to auscultation. The rest of her physical examination findings are unremarkable. Laboratory data are shown: Laboratory Test Result Blood Sodium 134 mEq/L (134 mmol/L) Potassium 3.2 mEq/L (3.2 mmol/L) Chloride 110 mEq/L (110 mmol/L) Bicarbonate 14 mEq/L (14 mmol/L) Blood urea nitrogen 10 mg/dL (3.6 mmol/L) Creatinine 0.2 mg/dL (18 µmol/L) Glucose 90 mg/dL (5.0 mmol/L) Urine pH 7.0 Specific gravity 1.015 Urinalysis with microscopy Normal Sodium 60 mEq/L (60 mmol/L) Potassium 10 mEq/L (10 mmol/L) Chloride 55 mEq/L (55 mmol/L) Of the following, the MOST likely diagnosis for this child is", "options" : "[\"congenital adrenal hyperplasia\", \"distal renal tubular acidosis\", \"methylmalonic acidemia\", \"postinfectious diarrhea\"]", "explanation" : "Correct Answer: B\nThe infant in the vignette has failure to thrive and normal anion gap metabolic acidosis. The higher-than-expected urinary pH and positive urine anion gap favors a diagnosis of distal renal tubular acidosis.\n\nMetabolic acidosis is defined as low serum bicarbonate (HCO3−) concentration. Metabolic acidosis results from either a net gain in hydrogen (H+) ion or a net loss of HCO3−. A systematic approach is required to diagnose and treat metabolic acidosis. The first step in evaluation of metabolic acidosis is to calculate the serum anion gap (the difference between the major cation and anions).\n\nAnion gap (mEq/L) = sodium (Na+) − [chloride (Cl−) + bicarbonate (HCO3−)]\nA normal anion gap ranges from 8 mEq/L to 12 mEq/L. Metabolic acidosis can be classified as elevated anion gap acidosis or normal anion gap acidosis (hyperchloremic metabolic acidosis) (Item C43A ).\n\nNormal anion gap acidosis is most commonly due to diarrhea or renal tubular acidosis (RTA). In diarrhea, gastrointestinal loss of HCO3− occurs, whereas in RTA there is impaired renal excretion of hydrogen ion (type 1 distal) or impaired reabsorption of HCO3− (type 2 proximal). To differentiate between diarrhea and RTA, urine electrolytes should be obtained and the urine anion gap (UAG) calculated.\n\nUrine anion gap = {urine sodium (Na+) + urine potassium (K+)} - urine chloride (Cl−)\nThe UAG can be positive (urine [Na+] + urine [K+] is > urine [Cl−]) or negative (urine [Na+] + urine [K+] is < urine [Cl−]). Unlike the serum anion gap, the magnitude of the gap is not clinically relevant. The UAG serves as a surrogate marker of NH4+ production. Diarrhea leads to a negative UAG, as there is increased excretion of H+ ion in the form of ammonium chloride (NH4+Cl−) in the urine to counteract the loss of HCO3− in the gastrointestinal tract. Therefore, in diarrhea, urinary Cl− is higher than the sum of urinary Na+ and K+.\n\nRenal tubular acidosis can be further classified into type 1 (distal; failure to excrete H+ ion), type 2 (proximal; failure to reabsorb HCO3−), and type 4 (hyperkalemic, disruption of renin-angiotensin-aldosterone axis). Children with distal RTA have a distal tubular acidification defect, resulting in low NH4+ excretion in urine. Therefore, urinary Cl− is lower than the sum of urinary Na+ and K+, and the UAG is positive. Children with proximal RTA have a reduced capacity to reabsorb HCO3− in the proximal tubule. However, the distal acidification mechanism is intact; therefore, urinary acidification is appropriate (pH < 5.5) and the UAG is negative. On supplementation with HCO3−, the threshold for filtered HCO3− is exceeded, causing an alkaline urine (pH > 5.5). Further clinical and laboratory differences in various types of RTA are shown in Item C43B. Urinary alkalizing agents (sodium bicarbonate or potassium citrate) are used as first-line treatment for all types of RTA. Serum electrolytes and growth are closely monitored while the patient is receiving treatment.\n\nCongenital adrenal hyperplasia presents with failure to thrive, atypical genitalia, metabolic acidosis or alkalosis, hyponatremia, and hyperkalemia. The child in this vignette has hypokalemia and, thus, congenital adrenal hyperplasia is an unlikely diagnosis. Methylmalonic acidemia is an inborn error of metabolism associated with an elevated anion gap acidosis. The infant in this vignette has a normal anion gap acidosis, making methylmalonic acidemia unlikely. In diarrheal illness, the renal acidification mechanism is intact, urinary ammonium excretion is high, and the urine anion gap is negative, making a diarrheal illness an unlikely diagnosis for this child.\n\nPREP Pearls\n• Diarrhea and renal tubular acidosis are the most common causes of normal anion gap acidosis in children.\n• The urine anion gap helps differentiate diarrhea from renal tubular acidosis.\n• In distal renal tubular acidosis, the urine anion gap is positive and ammonium (NH4+) excretion is low.\n\nABP Content Specifications(s)\n• Understand the clinical and laboratory findings associated with renal tubular acidosis\n• Formulate a differential diagnosis of renal tubular acidosis\n\nSuggested Readings\n• Carmody JB. Urine electrolytes. Pediatr Rev. 2011;32(2):65-68. doi:10.1542/pir.32-2-65.\n• Hsu BS, Lakhani SA, Wilhelm M. Acid-base disorders. Pediatr Rev. 2016;37(9):361-369. doi:10.1542/pir.2015-0093.\n• Kallen R. Renal tubular acidosis. In: McInerny TK, Adam HM, Campbell DE, DeWitt TG, Foy JM, Kamat DM, eds. American Academy of Pediatrics Textbook of Pediatric Care. 2nd ed. Elk Grove Village, IL: American Academy of Pediatrics; 2017:2554-2571. Pediatric Care Online .\n• Pelletier J, Gbadegesin R, Staples B. Renal tubular acidosis. Pediatr Rev. 2017;38(11):537-539. doi:10.1542/pir.2016-0231."}
{"id" : 3775, "question_text" : "A 3-week-old female neonate is brought to the emergency department for poor feeding and tachypnea. Physical examination reveals a nondysmorphic neonate with nasal flaring, diffuse rales on lung examination, a quiet precordium, and mild hepatomegaly. Chest radiography demonstrates cardiomegaly and pulmonary edema. Echocardiography shows hypertrophic cardiomyopathy and pericardial effusion, with no structural defects. Electrocardiography demonstrates increased voltages throughout. Laboratory results are as follows: Complete blood cell count: Normal; C-reactive protein: Normal; Blood cultures: No growth at 48 hours; Comprehensive metabolic panel with Blood glucose: 35 mg/dL (1.9 mmol/L), Aspartate aminotransferase: 125 U/L, Alanine aminotransferase: 150 U/L; Urinalysis: Negative, no ketones noted; Newborn screening: Elevated C14:1 of >1 mmol/L; Urine organic acids: Increased dicarboxylic acids. Of the following, this neonate's MOST likely diagnosis is", "options" : "[\"Barth syndrome\", \"\\u03b1-galactosidase A deficiency\", \"Pompe disease\", \"very-long-chain acyl-coenzyme A dehydrogenase deficiency\"]", "explanation" : "The neonate in the vignette has very long-chain acyl-coenzyme A dehydrogenase (VLCAD) deficiency, an autosomal recessive inborn error of metabolism that can present in 1 of 3 scenarios. This neonate has a subtype that manifests with severe early-onset cardiac failure that can progress to multiorgan failure. Cardiac findings include hypertrophic or dilated cardiomyopathy, arrhythmias, and pericardial effusion. Affected neonates and infants will have hypotonia and hepatomegaly. Laboratory findings include hypoketotic hypoglycemia, hepatic dysfunction, elevated creatine kinase, and increased dicarboxylic acids on urine organic acid analysis. Acylcarnitine analysis, an appropriate second-tier test, would reveal elevated C14:1, C14:2, C14, and C12:1 metabolites. This disorder is typically detected via newborn screening with elevated C14:1 of more than 1 mmol/L; screening is performed because early intervention and treatment will improve morbidity and mortality in affected children.\n\nA second type of VLCAD is the hepatic or hypoketotic hypoglycemic form; this type presents in early childhood with hypotonia and hepatomegaly but typically lacks cardiomyopathy. The third type has an even later onset, manifesting with episodic myopathy and intermittent rhabdomyolysis typically provoked by muscle cramps, pain, or exercise.\n\nThe diagnosis of VLCAD is confirmed with an acylcarnitine analysis and identification of biallelic pathogenic gene mutations in ACADVL. In affected children having episodes of acute illness, management requires the administration of intravenous glucose as an energy source to stimulate insulin secretion and suppress lipolysis, as well as careful monitoring for and management of arrhythmias and rhabdomyolysis. Avoidance of triggers such as fasting, dehydration, and long-chain fats is important for preventing metabolic decompensation; therefore, patients are maintained on a low-fat, high-carbohydrate diet with supplementation of medium-chain triglyceride oil and frequent regular feeding.\n\nFats are an important source of energy and serve as the principal fuel source rather than glucose for the heart and skeletal muscle during exercise. Many tissues prefer to use fatty acids for energy, thus allowing the brain to selectively use glucose. Hepatic fatty acid oxidation is also important for ketone body synthesis. Thus, fatty acid oxidation disorders commonly lead to hypoketotic hypoglycemia, hepatomegaly, hepatic dysfunction, myopathy, rhabdomyolysis, and encephalopathy.\n\nOther fatty acid oxidation disorders include, but are not limited to, carnitine palmitoyltransferase (CPT) I deficiency, CPT II deficiency, medium-chain acyl-CoA dehydrogenase deficiency, short-chain acyl-CoA dehydrogenase deficiency, and long-chain 3-hydroxyacyl-CoA dehydrogenase deficiency.\n\nMost fatty acid oxidation disorders have been associated with significant morbidity and mortality; however, because many of these disorders are now detected via newborn screening, their outcomes have improved with early treatment, dietary interventions, and avoidance of triggers.\n\nBarth syndrome is an X-linked recessive mitochondrial disorder arising from TAZ mutations, which presents in affected males with cardiomyopathy, muscular weakness, neutropenia, distinctive facial dysmorphology, and impaired growth. Urine organic acids would reveal significantly elevated urinary 3-methylglutaconic acid and moderately increased urinary 3-methylglutaric acid and 2-ethylhydracrylic acid.\n\nα-Galactosidase A deficiency, also known as Fabry disease, is an X-linked disorder that causes increasing lysosomal deposition of globotriaosylceramide in cells. It typically presents with periodic pain crises of the distal extremities, angiokeratomas, sweating dysfunction, corneal/lenticular opacities, and proteinuria. Progressive renal deterioration ultimately leads to end-stage renal disease in untreated affected males in the 3rd to 5th decade of life. Cerebrovascular strokes and heart disease are major causes of morbidity and mortality, typically in adulthood.\n\nPompe disease, an autosomal recessive glycogen storage and lysosomal disorder caused by a deficiency of the acid α-glucosidase enzyme, can present in infancy with cardiomyopathy, left ventricular hypertrophy, hepatomegaly, poor feeding, macroglossia, failure to thrive, muscular weakness, and respiratory difficulties. Affected children will have normal cognition. It is detectable on newborn screening; the diagnosis is then confirmed by the detection of reduced acid α-glucosidase enzyme activity or biallelic GAA pathogenic gene mutations. Other laboratory abnormalities include an elevated creatine kinase and abnormal urinary oligosaccharide levels.\n\nPREP Pearls\n• Very long-chain acyl-coenzyme A dehydrogenase (VLCAD) deficiency, an autosomal recessive inborn error of metabolism, can manifest in the neonatal period or infancy with severe early-onset cardiac failure that can progress to multiorgan failure if not treated promptly. Clinical findings include hypertrophic or dilated cardiomyopathy, arrhythmias, pericardial effusions, hypotonia, and hepatomegaly.\n• Laboratory findings in very long-chain acyl-coenzyme A dehydrogenase include hypoketotic hypoglycemia, hepatic dysfunction, elevated creatine kinase, abnormal acylcarnitine analysis, and increased dicarboxylic acids on urine organic acid analysis.\n• Prevention of metabolic decompensation with very long-chain acyl-coenzyme A dehydrogenase involves avoidance of triggers such as fasting, dehydration, and long-chain fats; affected children are maintained on a low-fat diet with supplementation of medium-chain triglyceride oil and frequent regular feeding.\n\nABP Content Specifications(s)\n• Recognize the laboratory features associated with disorders of fatty acid and carnitine metabolism\n• Recognize the clinical features associated with disorders of fatty acid and carnitine metabolism\n\nSuggested Readings\n• Leslie ND, Valencia CA, Strauss AW, MD, Zhang K. Very long-chain acyl-coenzyme A dehydrogenase deficiency. GeneReviews. https://www.ncbi.nlm.nih.gov/books/NBK6816/.\n• Saronwala A, Kubendran S, Kahler SG. Screening for genetic-metabolic diseases. In: McInerny TK, Adam HM, Campbell DE, DeWitt TG, Foy JM, Kamat DM, eds. American Academy of Pediatrics Textbook of Pediatric Care. 2nd ed. Elk Grove Village, IL: American Academy of Pediatrics; 2017:210-225. Pediatric Care Online.\n• Solis JO, Singh RH. Management of fatty acid oxidation disorders: a survey of current treatment strategies. J Am Diet Assoc. 2002;102:1800-1803. doi:10.1016/S0002-8223(02)90386-X.\n• Vishwanath VA. Fatty acid beta-oxidation disorders: a brief review. Ann Neurosci. 2016;23(1):51-55."}
{"id" : 3109, "question_text" : "A 5-year-old boy is a new patient brought to your office for easy bruisability. His mother noticed an increasing number of bruises and red dots on his trunk and legs over the past few weeks without any witnessed trauma. His weight is at the tenth percentile, but his height and head circumference are below the third percentile. He has low-set ears, 3 café-au-lait spots on his chest, as well as scattered bruises and petechiae on his trunk and legs. You also notice that his thumbs are abnormal in appearance (Item Q67). The remainder of the physical examination is unremarkable. His vital signs are stable. The following are the results of the child's complete blood cell count: White blood cell count, 2,500/µL (2.5 x 109/L), with 25% polymorphonuclear leukocytes, 65% lymphocytes, 8% monocytes, and 2% eosinophils; Hemoglobin, 11 g/dL (11 g/L); Mean corpuscular volume, 100 µm3 (100 fL); Platelet count, 25,000 x 103/ µL (25 x 109/L); Prothrombin time, normal; Partial thromboplastin time, normal. You refer the patient to a specialist for further evaluation. The mother would like to know the possible diagnoses. Of the following, the MOST appropriate response would be that he likely", "options" : "[\"had a congenital infection that led to the dysmorphic features and cytopenias\", \"has acute lymphocytic leukemia\", \"has a genetic mutation that can eventually lead to bone marrow failure\", \"has a nutritional deficiency affecting his growth and bone marrow production\", \"has an X-linked condition associated with immunodeficiency\"]", "explanation" : "The child in the vignette has features consistent with Fanconi anemia (FA), a rare congenital disorder associated with bone marrow failure, congenital anomalies, chromosomal instability, and defective DNA repair. Ninety-eight percent of cases are autosomal recessive, while 2% are X-linked recessive. Most patients with FA have macrocytosis, cytopenias, and some degree of bone marrow dysfunction within the first decade after birth. The most common physical features, found in 20% to 50% of patients with FA, include café-au lait spots, short stature, abnormal thumbs and radii, and abnormal head, eyes, kidneys, and ears. It is important to detect patients with FA to recognize the predisposition to malignancies such as leukemia (acute myeloid leukemia and myelodysplastic syndrome) and squamous cell carcinoma. Laboratory testing to diagnose FA involves detection of chromosomal breakage in peripheral blood cells after culture with diepoxybutane or mitomycin C. Multiple genes involving DNA repair have been found to lead to FA.\n\nThe only curative treatment for the bone marrow failure component of FA, at this time, is hematopoietic stem cell transplantation. Patients with FA require modified conditioning regimens for bone marrow transplantation because of their defect in DNA repair mechanisms. Androgens and cytokines (eg, granulocyte-colony-stimulating factor, granulocyte macrophage-colony-stimulating factor) have also been used in the treatment of cytopenias.\n\nCongenital infections (eg, cytomegalovirus) can cause cytopenias in the neonatal period, but these usually resolve over time. Leukemia and nutritional deficiencies (eg, vitamin B12 or folate) can cause macrocytosis and cytopenias, but would not explain the dysmorphic features. Wiskott-Aldrich syndrome is an X-linked condition characterized by immunodeficiency, thrombocytopenia, and eczema, but typically does not have any congenital anomalies.\n\nPREP Pearls\n• Fanconi anemia is a rare congenital disorder, usually of autosomal recessive inheritance. Associated with high incidence of bone marrow failure, malignancy, congenital anomalies, chromosomal instability, and defective DNA repair.\n• The most common physical features in patients with Fanconi anemia, include cafe-au-lait spots, short stature, abnormal thumbs and radii, and abnormal head, eyes, kidneys, and ears.\n• The only curative treatment for the bone marrow failure component of Fanconi anemia is hematopoietic stem cell transplantation.\n\nAmerican Board of Pediatrics Content Specification(s)\n• Recognize clinical and laboratory findings associated with Fanconi anemia\n\nSuggested Reading\n• Eiler ME, Frohnmayer D, Frohnmayer L, Larsen K, Owen J, eds. Fanconi Anemia: Guidelines for Diagnosis and Management. 3rd ed. Eugene, OR: Fanconi Anemia Research Fund; 2009: 372\n• Green AM, Kupfer GM. Fanconi anemia. Hematof Disco! Clin North Am. 2009;23(2):193-214. doi:10.1016/j.hoc.2009.01.008.\n• Kee Y, D'Andrea AD. Molecular pathogenesis and clinical management of Fanconi anemia. J ain Invest. 2012;122(11):3799-3806. doi:10,1172/ JCI58321."}
{"id" : 3166, "question_text" : "A 15-year-old adolescent boy presents to your office for follow-up 3 days after a right shoulder injury. He was playing basketball when another player pulled his arm posteriorly while he reached up to rebound the ball. He had the feeling that his shoulder popped out. The boy was seen in the emergency department after the injury. Radiography confirmed that he had dislocated his shoulder and he underwent reduction under sedation. You counsel the family about this injury. Of the following, the MOST accurate statement is that", "options" : "[\"adolescent boys have lower rates of recurrent dislocation than do adolescent girls\", \"adolescents are at lower risk for recurrent dislocations compared with adults\", \"in adolescents with shoulder dislocation, the humeral head is generally posteriorly displaced\", \"rotator cuff tears are often associated with shoulder dislocation in adolescents\", \"without surgery, he is likely to experience recurrent dislocation over the next 12 months\"]", "explanation" : "Preferred Response: E\nThe boy in the vignette has suffered an acute traumatic shoulder dislocation. Given his age and gender, he is likely to suffer an additional dislocation in the subsequent year. Adolescents are particularly vulnerable to recurrent dislocations, therefore many orthopedic providers recommend surgical stabilization after an initial traumatic shoulder dislocation.\n\nShoulder dislocations can be acute, resulting from trauma, as is the case for the adolescent in the vignette, or can be a result of multidirectional instability, when lax ligaments allow the humeral head to move off the glenoid. Ligamentous laxity of the shoulder can be associated with generalized hypermobility of the joints. In athletes who engage in sports with repetitive overhead shoulder movements, such as swimming or volleyball, repetitive microtrauma can result in localized ligamentous laxity.\n\nAcute traumatic dislocations generally occur when a force is aimed at the arm while the shoulder is held in abduction and external rotation. The boy in the vignette likely had his shoulder in this position when rebounding the basketball. Most young patients have an associated Bankart lesion, a tear of the labrum, which is the soft cartilage lining of the glenoid.\n\nIndividuals with shoulder dislocations nearly always experience immediate pain and describe a sensation that the shoulder is \"out of place On physical examination, the acromion will appear to be very prominent and the proximal humeral head will not be palpable in its normal position. In the acute care setting, radiography should be performed before reduction to document bony injuries. Radiography allows a clinician to evaluate for the presence of a bony Ban-kart lesion (labrum injury with a fracture of the glenoid) or a Hill-Sachs lesion (an impaction fracture involving the humeral head).\n\nAthletes with a shoulder dislocation should be treated acutely using a reduction maneuver to restore the normal position of the humeral head. Experienced clinicians may be able to perform a shoulder reduction maneuver in the field before muscle spasm makes sedation and analgesia necessary. However, in most cases, a health care provider is not present at the time of injury, and reductions are generally performed in the emergency department using procedural sedation. Following reduction, the shoulder should be immobilized for 2 to 3 weeks. Although slings are typically used to immobilize the shoulder, there is some evidence that use of a brace that positions the shoulder in external rotation for the first 24 hours after an injury reduces the risk of recurrence. After a few weeks, patients should undergo physical therapy to improve range-of-motion and strength in the shoulder. For some patients, elective surgical repair of torn ligaments and the labrum may be indicated to stabilize the shoulder.\n\nYounger patients and males have high rates of recurrent dislocation. Male adolescents have approximately a 75% chance of redislocation in the first year after initial dislocation. Some providers feel that individuals in this demographic group should be considered surgical candidates after an initial dislocation.\n\nWith most shoulder dislocations, the humeral head is displaced anteriorly and inferiorly. Posterior dislocations make up only 5% of dislocations. Rotator cuff tears are uncommon in adolescents.\n\nPREP Pearls\n• Shoulder dislocations can be caused by acute trauma or can result from laxity of the shoulder ligaments.\n• Adolescents and boys have high rates of redislocation after initial traumatic dislocation.\n\nAmerican Board of Pediatrics Content Specification(s)\n• Understand the natural history of shoulder dislocation\n• Plan the appropriate initial management of a sports-related shoulder injury\n\nSuggested Reading\n• Hovelius L, Olofsson A, Sandstrom B, et al. Nonoperative treatment of primary anterior shoulder dislocation in patients forty years of age and younger. a prospective twenty-five-year follow-up. Bone Joint Surg Am. 2008;90(5):945-952. doi:10.21D6IJBIS.G'00070.\n• Mather RC 3rd, Orlando LA, Henderson RA, Lawrence IT, Taylor DC. A predictive model of shoulder instability after a first-time anterior shoulder dislocation. J Shoulder Elbow Surg. 2011;20(2):259-266. doi:10.1016/j. jse.2010.10.037.\n• Sarwark IF, La Bella C. Pediatric Orthopaedics and Sports Injuries: A Quick Reference Guide. Elk Grove Village, IL: American Academy of Pediatrics; 2010:650 pp."}
{"id" : 1944, "question_text" : "A healthy 6-year-old girl is being evaluated at a new patient health supervision visit. Her parents ask about the girl's growth potential, because she has always been small for her age. She is currently at the 5th percentile for height and 25th percentile for weight. Her physical examination findings are unremarkable. No previous data are available to evaluate growth velocity. The father's height is 5'7\" and the mother's height is 5'2\". After calculating the midparental height using the population-based standard deviation of 1.67 inches (4.25 cm), the girl is suspected to have familial short stature. Of the following, 95% of the time a girl with this history and physical examination is EXPECTED to have a final height that falls between", "options" : "[\"4'11.5\\\" and 5'9.5\\\"\", \"5'1.2\\\" and 5'7.8\\\"\", \"4'10.7\\\" and 5'5.3\\\"\", \"5'2.8\\\" and 5'6.2\\\"\"]", "explanation" : "Standard deviation (SD) is a measure of dispersion; it measures the variability of data around the mean. A small SD indicates that data points are clustered close to the mean; a higher SD indicates a wider range of values. In samples with a normal distribution (bell-shaped curve), 68% of the values fall within 1 SD and 95% of the values fall within 2 SD above and below the mean (Item C144).\n\nItem C144: Illustration of normal distribution. Reprinted with permission from Smith TK, Johnson SB. Research and statistics: distribution, variability, and statistical significance. Pediatr Rev. 2010;31(10):432.\n\nMidparental height is an important calculation to perform when determining normal variability in stature and it provides a good estimate of a child's genetic adult height potential. The calculation for midparental height is:\n\nFor girls, the average of (father's height - 13 cm or 5 inches) + mother's height\n\nFor boys, the average of (mother's height + 13 cm or 5 inches) + father's height\n\nThe child's projected height is then compared with the midparental height range. If a child with short stature has a projected height that falls within 2 SDs of this value (by definition, 95% of the time, the value will fall within this range from the mean), then the child's height is within the expected range, given genetic potential. If so, the child probably has familial short stature, which is considered a normal variant. If the child's projected height falls outside this range, then other causes of short stature should be considered.\n\nThe midparental height for the girl in the vignette is the average of (5'7\" - 5\") + 5'2\" = 5'2\". If she has familial short stature, given an SD of 1.67 inches, 95% of the time, a girl in this situation will have a final height between 4'10.7\" and 5'5.3\".\n\nPREP Pearls\n\nStandard deviation is a measure of dispersion; it measures the variability of data around the mean.\n\nA small standard deviation indicates that data points are clustered close to the mean; a higher standard deviation indicates a wider range of values.\n\nIn samples with a normal distribution (bell-shaped curve), 68% of the values fall within 1 standard deviation and 95% of the values fall within 2 standard deviations above and below the mean.\n\nABP Content Specifications(s)/Content Area\n\nUnderstand standard error in the interpretation of results\n\nUnderstand standard deviation in the interpretation of results\n\nUnderstand confidence interval in the interpretation of results\n\nSuggested Readings\n\nBraun LR, Marino R. Disorders of growth and stature. Pediatr Rev. 2017;38(7)293-304. doi: 10.1542/pir.2016-0178.\n\nSmith TK, Johnson SB. Research and statistics: distribution, variability and statistical significance. Pediatr Rev.2010;31(10);431-432. doi: 10.1542/pir.31-10-431.\n\nSoyemi K. Choosing the right statistical test. Pediatr Rev. 2012;33(5)e38-e44. doi: 10.1542/pir.33-5-e38.\n\nTanner JM, Goldstein H, Whitehouse RH. Standards for children's height at ages 2-9 years allowing for heights of parents. Arch Dis Child. 1970;45(244):755762."}
{"id" : 1453, "question_text" : "A 3-year-old girl presents to your office for evaluation of a \"lump\" in her right neck that was noticed by her mother while she was being bathed. She had an upper respiratory infection 3 weeks ago, with rhinorrhea and cough, which has fully resolved. She has had a normal appetite and energy level. The girl has not had persistent fevers, weight loss, or night sweats. The family has no pets, they have not been in a forest or wooded area, and have not traveled outside the country. The girl is developmentally appropriate, and is at the 40th percentile for both weight and height. On physical examination, a 1.5 × 1.0 cm freely movable and nontender mass is palpable just below the angle of her right mandible. The remainder of her physical examination is unremarkable. Of the following, the BEST next step in management is", "options" : "[\"obtain computed tomography scan of the neck with contrast\", \"perform a purified protein derivative test\", \"provide reassurance to the mother\", \"refer to a surgeon for a biopsy\", \"treat with oral amoxicillin/clavulanate\"]", "explanation" : "Correct Answer: C\nNeck masses are a common presenting problem seen by the pediatrician. The differential diagnosis for a neck mass depends on several factors including location, duration, size, and characteristics such as erythema or pain. A useful algorithm for the approach to a neck mass is shown in Item 204 . The girl in the vignette presented with a nontender, nonerythematous, small (<1.5 cm in the greatest diameter) neck mass just below the angle of the right mandible. She has been well, with normal growth and development, and has had no exposures that would raise concern for Lyme disease or cat scratch disease. The location and size of the mass, and the lack of a remarkable history other than the upper respiratory infection that occurred 3 weeks before presentation, suggest that this is a reactive lymph node. As such, no further intervention is warranted.\n\nDiagnostic computed tomography (CT) scans expose children to radiation. Several recent studies suggest that exposure to even a small radiation dose can increase a child's risk for malignancy. Unless there is a strong suspicion of malignancy, CT would not be the imaging method of choice for a child with a neck mass.\n\nWith the absence of any historical risk factors or associated symptoms, there is no reason to suspect that the girl in the vignette has tuberculosis and therefore no indication to perform a purified protein derivative test.\n\nGiven the constellation of findings suggesting that the girl in the vignette has a simple reactive lymph node, there would be no indication for referral to a surgeon for a biopsy. Bacterial lymphadenitis presents with erythema and/or pain over an enlarged lymph node, and often with fever. The girl in the vignette had none of these findings, so treatment with amoxicillin/clavulanate (or any antibiotic) would not be indicated. A child with apparent bacterial adenitis that persists despite seemingly adequate antibiotic therapy warrants evaluation by a pediatric oncologist.\n\nPREP Pearls\n• Persistent lymphadenopathy despite seemingly adequate therapy for bacterial adenitis warrants evaluation by a pediatric oncologist.\n• When evaluating a neck mass, computed tomography (CT) should be reserved for situations in which there is a high index of suspicion for malignancy. Ideally, the patient should be evaluated by a pediatric oncologist before ordering a CT scan.\n\nABP Content Specifications(s)\n• Formulate a differential diagnosis of a neck mass\n\nSuggested Readings\n• Friedmann AM. Evaluation and management of lymphadenopathy in children. Pediatr Rev. 2008;29(2):53-60. doi: http://dx.doi.org/10.1542/pir.29-2-53.\n• Geddes G, Butterly MM, Patel SM, Marra S. Pediatric neck masses. Pediatr Rev. 2013;34(3):115-125. doi: http://dx.doi.org/10.1542/pir.34-3-115.\n• Sahai S. Lymphadenopathy. Pediatr Rev. 2013;34(5):216-227. doi: http://dx.doi.org/10.1542/pir.34-5-216."}
{"id" : 744, "question_text" : "A 3-month-old female infant presents to your office for follow-up of pyelonephritis diagnosed 2 months ago. After treatment of her urinary tract infection, she had a contrast voiding cystourethrogram (VCUG) that showed narrowing of the distal urethra and a normal urinary stream upon voiding (Item Q36). Her physical examination is unremarkable. She is currently on oral amoxicillin for urinary tract infection prophylaxis. Of the following, the MOST appropriate next step in the management of this patient is", "options" : "[\"intravenous pyelography\", \"referral to urology for surgical correction\", \"repeat urine culture\", \"repeat VCUG in 1 year\", \"stop prophylactic antibiotics\"]", "explanation" : "The voiding cystourethrogram (VCUG) shown for the girl described in the vignette has the classic findings of spinning top urethral (STU) deformity. STU is caused by dilation of the proximal muscular urethra against a closed or narrow distal urethral sphincter. This is a rare variant seen in girls and young women.\n\nInitially considered to be a normal variant, it has been recently associated with bladder dysfunction (detrusor sphincter dyssynergia: contraction of the detrusor against a closed urethral sphincter). In a 3-month-old infant, this appearance could be secondary to the age-appropriate immature voiding reflexes. The overactive bladder almost always resolves, but the time to resolution is highly variable. In this patient, the detrusor- sphincter dyssynergia will most likely resolve with maturation and development of normal voiding habits. Therefore there is no indication for urology referral or surgical correction at this time. Older patients usually present with symptoms of overactive bladder such as frequency, urgency, and urge incontinence. Constipation is a commonly associated symptom in such patients. It is currently unclear if urinary tract infections (UTIs) are a cause or effect of the overactive bladder. However, the appearance of the urethra on VCUG does not correlate with either urethral caliber or episodes of lower UTI. The VCUG in the infant in the vignette shows no evidence of reflux, therefore the next most appropriate step is to stop antibiotics. Currently a repeat urine culture in the presence of a normal VCUG is not recommended before stopping antibiotics. A repeat VCUG, after an initial normal VCUG also is not indicated in this patient.\n\nIntravenous pyelography is a radiologic test for identifying the details of the urinary system including kidneys and ureters. The test has diagnostic significance for identifying upper urinary tract obstruction. The current availability of ultrasonography, computed tomography scan, and magnetic resonance urography has replaced intravenous pyelography in almost all diagnostic settings. In the patient described in the vignette, no further diagnostic imaging is indicated.\n\nPREP Pearls\n• Narrow urethra on voiding cystourethrogram (also termed spinning top urethral [STU] deformity) is caused by dilation of the proximal muscular urethra against a closed or narrow distal urethral sphincter.\n• STU has been associated with bladder dysfunction arising from contraction of the detrusor muscle of the bladder against a closed urethral sphincter.\n• Bladder dysfunction is common in infants; the development of normal voiding patterns is highly variable.\n\nAmerican Board of Pediatrics Content Specification (s):\n• Know that a girl with a narrow urethra needs no treatment\n\nSuggested Reading:\n• Elder JS. Voiding dysfunction. In: Kliegman RM, Stanton BF, St Geme JW III, Schor NF, Behrman RE, eds. Nelson Textbook of Pediatrics. 19th ed. Philadelphia, PA: Saunders Elsevier; 2011: 537:1847-1852\n• Elder JS. Obstruction of the urinary tract. In: Kliegman RM, Stanton BF, St Geme JW III, Schor NF, Behrman RE, eds. Nelson Textbook of Pediatrics. 19th ed. Philadelphia, PA: Saunders Elsevier; 2011:1838-1847\n• Saxton HM, Borzyskowski M, Mundy AR, et al: Spinning top urethra: not a normal variant. Radiology. 1988;168:147-150"}
{"id" : 2084, "question_text" : "An 8-day-old full-term neonate is being evaluated at an initial visit. She was delivered vaginally at home by her mother's friend, who is studying to be a midwife. Her 19-year-old mother has no significant medical history. She received limited prenatal care. The neonate has been breastfeeding well, with 2 to 3 wet diapers and multiple stools daily. Her mother says the girl's eyelashes on both eyes have been crusty. On physical examination, a clear discharge is noted from both eyes with mild conjunctival erythema.\n\nOf the following, this neonate's MOST likely condition could have been prevented by", "options" : "[\"avoidance of perfumed baby shampoo\", \"gentle massage of both nasal lacrimal ducts downward\", \"ocular application of 0.5% erythromycin ointment\", \"saline irrigation of the eyes\"]", "explanation" : "Correct Answer: C\nThe neonate's most likely diagnosis is ophthalmia neonatorum, or neonatal conjunctivitis, following delivery at home without ocular prophylaxis with erythromycin ointment. In 1881, German obstetrician Dr Carl Crede reduced the incidence of ophthalmia neonatorum from 10% to 0.3% among his patients with prophylactic ocular administration of 2% silver nitrate. Historically, ophthalmia neonatorum was the leading cause of neonatal blindness, caused primarily by Neisseria gonorrhoeae. With improved treatment of maternal N gonorrhoeae infection, Chlamydia trachomatis has became the most common cause of ophthalmia neonatorum affecting 8.2 in 1,000 live births. Because of the risk of chemical conjunctivitis with the use of silver nitrate, in the United States neonates receive ocular prophylaxis with 0.5% erythromycin ointment.\n\nCongenital nasolacrimal duct obstruction occurs because of abnormal formation of the nasolacrimal duct. Neonates often present with unilateral eye discharge and/or excessive tearing with normal conjunctivae. Parents may be instructed to provide gentle downward massage to help drain the duct.\n\nAlthough a perfumed shampoo could cause a chemical conjunctivitis, it is not commonly seen in neonates. Other possible eye irritants include smoke, smog, and household cleaning sprays. Saline irrigation of the eyes is recommended as part of the treatment of gonococcal ophthalmia, in conjunction with administration of ceftriaxone or cefotaxime. Saline irrigation should not cause or prevent the conjunctivitis seen in the neonate in the vignette.\n\nPREP Pearls\n\nOphthalmia neonatorum, neonatal conjunctivitis, may be caused by exposure to maternal Chlamydia trachomatis or Neisseria gonorrhoeae infection.\n\nErythromycin ocular prophylaxis after birth is indicated for all neonates.\n\nWhen used as ocular prophylaxis against ophthalmia neonatorum, silver nitrate may cause a temporary chemical conjunctivitis.\n\nABP Content Specifications(s)/Content Area\n\nPlan appropriate eye prophylaxis for a newborn infant\n\nSuggested Readings\n\nOlitsky SE, Hug D, Plummer LS, Stahl ED, Ariss MM, Lindquist TP. Disorders of the conjunctiva. In: Kliegman RM, Stanton BF, St Geme JW, Schor NF, eds. Nelson Textbook of Pediatrics. 20th ed. Philadelphia, PA: Saunders Elsevier; 2016:3036-3040.\n\nRichards A, Guzman-Cottrill, JA. Conjunctivitis. Pediatr Rev. 2010;31:196. doi: 10.1542/pir.31-5-196."}
{"id" : 170, "question_text" : "A 17-year-old sexually active girl presents for a follow-up evaluation after her third episode of a urinary tract infection. She is currently asymptomatic. The results of renal ultrasonography and voiding cystourethrography are negative. She asks you how to prevent further episodes.\n\nOf the following, you are MOST likely to advise her to", "options" : "[\"drink cranberry juice frequently\", \"increase her daily water intake\", \"make sure to void after intercourse\", \"self-medicate with antibiotics for 3 days when symptomatic\", \"use single-dose postcoital antibiotic prophylaxis\"]", "explanation" : "Acute cystitis, or an inflamed bladder mucosa, is much more common in females, who are also more likely to have recurrences. The occurrence of three infections with positive cultures in the preceding year, as described for the girl in the vignette, meets the criteria for recurrent lower urinary tract infection (UTI) or cystitis.\n\nCurrently, the best evidence supports the use of antibiotics as a preventive measure. Those who associate their symptoms with sexual intercourse should be offered postcoital antibiotic prophylaxis (taken within 2 hours of intercourse). This involves a single dose of an effective antimicrobial (eg, nitrofurantoin 50 mg, trimethoprim-sulfamethoxazole [TMP-SMX] 40/200 mg, or cephalexin 500 mg). Continuous antimicrobial prophylaxis may be required in women who fail a postcoital regimen, do not associate frequent UTIs with a modifiable cause, or are at risk for recurrent complicated UTIs. Regimens include trimethoprim (100 mg daily at bedtime or 3 times per week), TMP-SMX (40/200 mg daily at bedtime or 3 times per week), nitrofurantoin (50 to 100 mg daily at bedtime or 3 times per week), norfloxacin (200 mg daily at bedtime or 3 times per week), and cephalexin (250 mg daily). These regimens have been shown to be safe and effective, even after 5 years of use. Because one third of women experience a prolonged UTI-free period, a trial without the medication can be attempted after 6 to 12 months of continuous therapy, with prophylaxis reinstituted if the patient develops recurrent UTIs. Self-medication when symptomatic with a 3-day regimen is another option, although the evidence for this therapy is less clear than for the previous ones. Studies show that women who have a past history of at least two UTIs are capable of self-diagnosis and treatment. This regimen is best for those who have less frequent episodes (ie, one to two per year).\n\nAn important risk factor for recurrent UTIs or cystitis is sexual activity, with an odds ratio of 12.4 (95% confidence interval, 3 - 59) for symptoms developing after intercourse. Other coital behaviors that may be risk factors include coital frequency more than five times a week and use of a diaphragm and spermicides. Consideration should be given to changing the method of contraception in such patients. The infections are often closely spaced and usually have no anatomic or functional abnormalities to account for the recurrences (eg, calculus, abscess, cystic disease). Therefore, if the patient has had no history of infections before the onset of sexual activity, radiologic evaluation with ultrasonography and voiding cystourethrography is not indicated. Obtaining cultures with each episode is important to diagnose cystitis. Prompt resolution of symptoms with antibiotics differentiates it from other conditions that mimic cystitis.\n\nBehavior modifications that are often recommended but have not been well studied include use of a \"proper wiping technique from front to back,\" avoidance of tight pants, urge-initiated voiding, postcoital voiding, increased fluid intake, and the daily consumption of cranberry juice. Although evidence supports the use of cranberry juice and other products as beneficial, there are no clear directions for the amount or concentration of juice to be consumed or the duration of a regimen.\n\nAmerican Board of Pediatrics Content Specification(s): Plan the management of recurrent cystitis"}
{"id" : 3783, "question_text" : "A 2-week old neonate is having a health supervision evaluation. He was born at term to first-time parents. Prenatal ultrasonography revealed shortened femur length and macrocephaly; postnatal evaluation confirmed the diagnosis of achondroplasia. The boy's parents wish to know what the typical pattern of development is in children with this diagnosis. Of the following, the MOST accurate statement is that children with this diagnosis typically have", "options" : "[\"delayed cognitive development\", \"delayed gross motor development\", \"global developmental delay\", \"normal development\"]", "explanation" : "The neonate in the vignette has achondroplasia; children with this condition typically have delayed gross motor milestones but development in other realms, including cognition, is normal. Achondroplasia is a skeletal dysplasia caused by mutations in the fibroblast growth factor receptor 3 gene (FGFR3) and is inherited in an autosomal dominant fashion. It is the most common type of skeletal dysplasia, a group of conditions that affect bone development. Children with achondroplasia have disproportionate short stature, long bone shortening that is most prominent in the humerus and femur, brachydactyly (short fingers and toes), kyphoscoliosis, lumbar lordosis, and macrocephaly. Early gross motor delays typically exist because of atypical physical proportions; delays resolve by age 2 to 3 years. Children with achondroplasia are at increased risk of developing otitis media, obstructive sleep apnea, obesity, chronic joint pain, and cervical medullary compression.\n\nOsteogenesis imperfecta is a heterogeneous bone matrix disorder characterized by increased bone fragility. Although there are variable phenotypic presentations, all children with osteogenesis imperfecta have bone fragility, osteoporosis, bone deformities, and multiple or atypical fractures. Some also have blue sclera, hearing loss, dental abnormalities, increased ligamentous and skin laxity, and/or easy bruising. Several gene mutations have been identified, but the most common are autosomal dominant mutations in COL1A1 or COL1A2 which encode chains of type 1 collagen.\n\nArthrogryposis (also known as \"arthrogryposis multiplex congenita\") is a condition in which multiple joint contractures develop before birth. It can be associated with multiple diagnoses that decrease fetal movement in utero, including central nervous system disorders, neuromuscular diseases, connective tissue or muscular disorders, and trisomies.\n\nPREP Pearls\n• Achondroplasia is an autosomal dominant disorder that is characterized by disproportionate short stature, long bone shortening that is most prominent in the humerus and femur, brachydactyly (short fingers and toes), kyphoscoliosis, lumbar lordosis, and macrocephaly. Infants and toddlers with achondroplasia have gross motor delays but otherwise normal development.\n• Osteogenesis imperfecta is a heterogeneous disorder characterized by bone fragility, osteoporosis, bone deformities, and multiple or atypical fractures. Affected children may have blue sclera, hearing loss, dental abnormalities, increased ligamentous and skin laxity, and/or easy bruising.\n• Arthrogryposis is a condition in which multiple joint contractures develop before birth. It can be associated with multiple diagnoses that decrease fetal movement in utero, including central nervous system disorders, neuromuscular diseases, connective tissue or muscular disorders, and trisomies.\n\nABP Content Specifications(s)\n• Recognize the clinical findings associated with achondroplasia, including complications\n• Recognize the clinical findings associated with osteogenesis imperfecta\n• Recognize the clinical features associated with arthrogryposis\n\nSuggested Readings\n• Clunie G. Prenatal diagnosis. In: McInerny TK, Adam HM, Campbell DE, DeWitt TG, Foy JM, Kamat DM, eds. American Academy of Pediatrics Textbook of Pediatric Care. 2nd ed. Elk Grove Village, IL: American Academy of Pediatrics; 2017:699-710. Pediatric Care Online.\n• Hall JG. Arthrogryposis (multiple congenital contractures): diagnostic approach to etiology, classification, genetics, and general principles. Eur J Med Genet. 2014;57(8):464-472. doi:10.1016/j.ejmg.2014.03.008.\n• Pauli RM, Legare JM. Achondroplasia. GeneReviews. https://www.ncbi.nlm.nih.gov/books/NBK1152/.\n• Steiner RD, Adsit J, Basel D. COL1A1/2-related osteogenesis imperfecta. GeneReviews. https://www.ncbi.nlm.nih.gov/books/NBK1295/."}
{"id" : 1128, "question_text" : "A 12-year-old girl presents to your office for a health supervision visit prior to attending a summer gymnastics camp. The girl is premenarcheal and there is no family history of scoliosis. On physical examination, you note a right thoracic spine prominence with the Adam's Forward Bend test. Measurement of her spine using a scoliometer reveals an 8-degree angle of thoracic rotation. History and physical examination are otherwise unremarkable. Of the following, the next MOST appropriate step in management is", "options" : "[\"obtain magnetic resonance imaging of the thoracic spine\", \"obtain posteroanterior and lateral entire spine radiographs\", \"recommend use of a thoracolumbosacral spine brace\", \"repeat a clinical evaluation in 1 year\", \"withhold medical clearance for participation in gymnastics camp\"]", "explanation" : "Scoliosis is a vertebral rotation that leads to a curvature of the spine that is most pronounced in the coronal plane. Scoliosis that involves the thoracic vertebrae will also cause the ribs to rotate. A scoliometer is a tool designed to measure this rib rotation. Scoliometer measurements (expressed as angles of trunk rotation [ATR]) are an indirect measure of scoliosis. A 5- to 7-degree ATR corresponds to a scoliosis curve measuring approximately 20 degrees on radiographs using Cobb angle measurements. The girl in the vignette should undergo posteroanterior and lateral radiography of the entire spine to more accurately quantify the degree of curvature.\nScoliosis is categorized by underlying etiology. Idiopathic scoliosis is the most common type, affecting 1 in 50 individuals. Idiopathic scoliosis is generally diagnosed during preadolescence or early adolescence. With the exception of very mild cases, scoliosis shows a female predominance; girls are 10 times more likely than boys to have severe scoliosis that merits spinal fusion surgery. Idiopathic scoliosis tends to progress during years of rapid growth. If the curvature remains in the mild or moderate range by the time a teen reaches skeletal maturity, further progression of the scoliosis is unlikely.\nThe most common cause of nonidiopathic scoliosis is neuromuscular (eg, related to cerebral palsy, spinal muscular atrophy, or other neuromuscular conditions). Scoliosis is also associated with certain genetic syndromes such as neurofibromatosis and Marfan syndrome.\nOccult spinal cord pathology, such as tethered spinal cord or Chiari I malformation with syrinx, can also cause scoliosis. \"Red flags\" for occult spinal cord pathology include young age at scoliosis presentation (<10 years), atypical curve pattern (eg, a thoracic curve toward the left side of the body), foot deformities (eg, high arch, toe contractures), and other neurologic signs and symptoms on history and physical examination.\nRadiographs are the \"gold standard\" for the diagnosis of scoliosis. The curvature must measure 10 degrees or more on radiographs to meet the criteria for scoliosis. Providers taking the history of a patient with suspected scoliosis should ask about any history of worsening shoulder or back asymmetry, neurologic symptoms including bowel or bladder dysfunction, weakness or pain radiating into the extremities, and family history of scoliosis. Physical examination findings of scoliosis can include asymmetry of the shoulders or muscles of the upper back, or a shift of the pelvis. For the Adams forward bending test, the patient puts his/her palms together and attempts to touch the floor with his/her fingertips without bending at the knees. Scoliometer measurements can be performed with the patient in this position. Prominence of 1 side of the thoracic and/or lumbar spine should prompt radiographic evaluation, particularly if the ATR is 5 degrees or greater.\nThe treatment for idiopathic scoliosis depends on the severity of curvature and the years of growth remaining. Observation is the treatment of choice for mild scoliosis (10 to <25 degrees). Bracing is recommended for individuals with moderate scoliosis (25 to <45 degrees) with remaining growth potential. For children and adolescents with scoliosis curves greater than 45 to 50 degrees, spinal fusion surgery is usually required.\nThe girl in the vignette has physical examination findings consistent with scoliosis. A magnetic resonance imaging scan is not warranted because she does not exhibit atypical features suggesting occult spinal cord pathology. Bracing would be considered only if radiography showed scoliosis in the moderate range. Observation would not be appropriate because radiography could demonstrate that this girl may be a candidate for bracing without which her scoliosis could worsen over the subsequent 12 months. Individuals with idiopathic scoliosis do not need activity restriction.\nPREP Pearls\n Evaluation for occult spinal pathology is recommended for children with scoliosis presenting before age 10 years or those who present with a left-sided thoracic curvature.\n Scoliometer measures scoliosis indirectly, therefore radiographs should be obtained for individuals with a measurement of 5 degrees or greater.\n Occult neurologic disease can cause scoliosis.\nABP Content Specifications(s)\n Plan the appropriate clinical evaluation of scoliosis, and manage appropriately\n Recognize the various complications associated with scoliosis\n Understand the natural history and etiology of scoliosis"}
{"id" : 1492, "question_text" : "You are seeing a 16-year-old adolescent boy with obstructive sleep apnea and a history of recurrent sinusitis in your office. On physical examination, you note that he has a bifid uvula. You refer him to an otolaryngologist for adenoidectomy and counsel the parents about his risk of postoperative complications. Of the following, based on physical examination findings, this patient postoperatively is at INCREASED risk of", "options" : "[\"bleeding\", \"hypernasal voice\", \"nasopharyngeal stenosis\", \"surgical site infection\", \"torticollis\"]", "explanation" : "Correct Answer: B\nThe adolescent in the vignette has a bifid uvula that is likely associated with an unrecognized submucosal cleft, increasing his risk of velopharyngeal insufficiency (VPI) after an adenoidectomy. Velopharyngeal insufficiency is defined as incomplete closure between the soft palate and the pharyngeal wall during speech, which allows air to escape through the nasal cavity. Velopharyngeal insufficiency leads to a hypernasal voice and in severe cases, nasal regurgitation of fluids. Velopharyngeal insufficiency can be caused by anatomic abnormalities such as hard or soft palate or submucosal clefts, inadequate soft palate length, or paralysis of the soft palate.\n\nVelopharyngeal insufficiency is a known complication of adenoidectomy that occurs because removal of the adenoids increases the size of the nasopharyngeal airway. Individuals with preexisting palatal defects are at much higher risk for postadenoidectomy VPI. It is often a temporary postoperative occurrence, but if persistent, patients should be referred for evaluation by a speech pathologist. Sustained VPI after adenoidectomy is associated with chromosome 22q11 deletion (velocardiofacial) syndrome, and the clinician should strongly consider testing for this syndrome in these patients.\n\nA bifid uvula and possible underlying submucosal cleft do not increase the risk of postoperative bleeding, nasopharyngeal stenosis, surgical site infection, or torticollis.\n\nPREP Pearls\n• Velopharyngeal insufficiency (VPI) is the incomplete closure of the soft palate and the pharyngeal wall that allows air to escape through the nasal cavity\n• Velopharyngeal insufficiency can lead to a hypernasal voice.\n• The risk of VPI is increased after an adenoidectomy, especially in children with preexisting palatal defects or chromosome 22q11 deletion (velocardiofacial) syndrome.\n\nABP Content Specifications(s)\n• Understand the general concept of velopharyngeal insufficiency\n\nSuggested Readings\n• Gosain AK, Conley SF, Marks S, Larson DL. Submucous cleft palate: diagnostic methods and outcomes of surgical treatment. Plast Reconstr Surg. 1996;97(7):1497-1509.\n• Messner AH. Adenoidectomy in children: postoperative care and complications. UpToDate. Available online only with subscription.\n• Ruda JM, Krakovitz P, Rose AS. A review of the evaluation and management of velopharyngeal insufficiency in children. Otolaryngol Clin North Am. 2012;45(3):653-669. doi: http://dx.doi.org/10.1016/j.otc.2012.03.005."}
{"id" : 1127, "question_text" : "A full term female newborn weighing 3,900 g is in the well-baby nursery and breastfed well on day 1 of life. You are performing the discharge physical on day 2 after birth, but the routine pulse oximetry screening has a saturation reading of 91%. The baby has a heart rate of 120 beats/min, respiratory rate of 40 breaths/min, and blood pressure is 65/45 mm Hg taken in the right leg. The baby is awake, alert, and fussy. The chest examination is unremarkable. The cardiac examination shows a single S2 and a 2/6 systolic murmur at the right upper sternal border. There is no hepatosplenomegaly. The femoral pulses are easily palpable but diminished. You are awaiting a call back from the neonatologist. Of the following, the MOST appropriate next step in management is to", "options" : "[\"follow serial 4 extremity blood pressures and allow normal feedings\", \"give fluid bolus of 20 mL/kg of normal saline\", \"provide 100% O2 by facemask for 24 hours\", \"start low dose prostaglandin at 0.01 \\u00b5g/kg per min\", \"stop feedings and start dopamine at 5 \\u00b5g/kg per min\"]", "explanation" : "For the neonate in this vignette, the most appropriate next step is to start prostaglandin E (PGE) while you are awaiting the next specialty care provider's help. The patent ductus arteriosus (PDA) is a vascular structure that allows blood flow from the pulmonary artery to the aorta in utero, bypassing the lungs. After birth, there are many congenital heart disease variants that become critical when the PDA closes. The 2-day-old patient described in the vignette has physical examination findings of a single outflow tract (single S2) and intracardiac mixing with an oxygen saturation of 91%. Screening of all newborns on day 2 of life has been implemented in the United States to identify newborns with critical congenital heart disease who may be asymptomatic.\nThe decreased peripheral pulses in the neonate in the vignette point towards hypoplastic left heart syndrome with reduction in peripheral circulation. In this lesion, the PDA is functioning as if it is the aortic arch, much like it did in utero. Early recognition and initiation of PGE treatment will help to prevent the decompensation that occurs if the PDA is allowed to close. If the PDA closes, the baby will quickly become acidotic and hypotensive with resultant renal dysfunction. If you think about the entire cardiac output as the bubble in a carpenter's level, with the PDA being the level, then anything that causes the flow to shift toward one end will rob the flow to the other. Therefore, if the pulmonary vascular resistance (PVR) is acutely lowered by rapidly ventilating a newborn, the cardiac output will flow toward the lungs and leave the systemic circulation depleted. This will then cause decreased renal blood flow, decreased urine output, and poor pulses with acidosis. The lungs will become overcirculated and pulmonary edema will occur. This also happens naturally and gradually as the PVR drops during the first few days after birth and helps dictate when surgery is optimally done. If the PVR is acutely raised with a pneumothorax, for example, the baby will become quite hypoxemic.\nTwo other lesions that usually require the PDA for stabilization are pulmonary atresia and tricuspid atresia. In both of these instances where there is no or limited blood flow into the pulmonary veins, the PDA provides the pulmonary blood flow. The oxygen saturations in these lesions may be lower than the 91%, as in the patient in this vignette with hypoplastic left heart syndrome. In pulmonary atresia, a single S2 is audible. Blood flow in this lesion is from the right atrium through either an atrial septal defect to the left atrium and the left ventricle (LV), or through the tricuspid valve to the right ventricle and then through a ventricular septal defect to the LV. In either case, there is mixing in the LV and the blood leaving the LV will then get to the lungs via the aorta and PDA. Some patients may have collateral vessels that provide additional flow from the aorta to the pulmonary arteries. Prostaglandin E is needed to keep the PDA open and ensure pulmonary blood flow. In tricuspid atresia, A2 and a P2 may be audible if there is flow from the right ventricle to the pulmonary artery. In tricuspid atresia, there will need to be an adequate atrial level shunt, as well as a ventricular level shunt, to return blood to the right side of the heart and allow for blood flow into the pulmonary artery. If there is inadequate ventricular level shunting, then the PDA will be needed for pulmonary blood flow. Stabilization with PGE prior to transport for both types of patients will allow pulmonary flow to be maintained.\nIf a newborn has ductal dependent systemic circulation, they may decompensate quickly as the PDA closes. Following the 4 extremity blood pressures and allowing normal feedings may be dangerous to the baby if the mesenteric circulation has been diminished. A fluid bolus will not overcome the lack of flow in the descending aorta. One hundred percent O2 will cause the PDA to close and the pulmonary vasculature to dilate. Dopamine will increase the systemic vascular resistance, but without ductal patency in the setting of hypoplastic left heart syndrome, it will not improve distal perfusion or prevent acidosis.\nPREP Pearls\n Cardiac decompensation secondary to outflow tract atresia can be emergently managed by maintaining the patency of the ductus arteriosus with prostaglandin.\n Screening of all newborns on day 2 of life has been implemented in the United States to identify newborns with critical congenital heart disease who may be asymptomatic.\nABP Content Specifications(s)\n Understand the role of the ductus arteriosus in cyanotic congenital heart disease, and manage appropriately"}
{"id" : 925, "question_text" : "A 4-year-old boy presents with new-onset type 1 diabetes mellitus and diabetic ketoacidosis. He appears tired but, on physical examination, shows only mild signs of dehydration. Initial laboratory tests reveal the following results:\n• Serum glucose, 884 mg/dL (49.1 mmol/L)\n• Serum sodium, 131 mEq/L (131 mmol/L)\n• Serum potassium, 4.5 mEq/L (4.5 mmol/L)\n• pH, 6.92 on arterial blood gas\n\nOf the following, the MOST important first step in managing this patient is to administer", "options" : "[\"an insulin drip, beginning at 0.1 units/kg/h\", \"an intravenous insulin bolus of 0.1 units/kg\", \"lactated Ringer solution, 20 mL/kg over 15 minutes\", \"normal saline, 10 to 20 mL/kg over 1 to 2 hours\", \"sodium bicarbonate, 1 to 2 mmol/kg over 60 minutes\"]", "explanation" : "The patient described in the vignette is in diabetic ketoacidosis (DKA) with a significant acidosis. Current guidelines from the American Diabetes Association (ADA), the International Society for Pediatric and Adolescent Diabetes, and the Pediatric Endocrine Society recommend initial management with a bolus of isotonic fluids, typically 10 to 20 mL/kg over 1 to 2 hours.\n\nThe reason to give a bolus slowly is to prevent a rapid decrease in glucose concentration while restoring peripheral circulation because a rapid decrease in blood glucose may be one of many risk factors for the development of cerebral edema. A rapid bolus that could lead to osmotically mediated fluid shifts is not appropriate, although recent data suggest that vasogenic, rather than cytotoxic, cerebral edema may be the predominant finding in DKA.\n\nAll 3 guidelines recommend starting insulin therapy 1 to 2 hours after fluid resuscitation has begun because most children with DKA are 7.5% to 10% volume depleted at presentation and need volume resuscitation first. In some studies, early initiation of insulin therapy within the first hour of fluid therapy has been associated with the development of cerebral edema, although the reasons for this are not clear. Intravenous fluids and treatment with an insulin drip will serve to correct the high anion gap acidosis present in DKA. Insulin stops further ketoacid production and allows ketoacids to be metabolized. Treatment of hypovolemia improves tissue perfusion and renal function, increasing the excretion of organic acids. Alternative treatments to correct acidosis, such as treatment with intravenous sodium bicarbonate, are not routinely recommended. Controlled trials have reported no clinical benefit from sodium bicarbonate administration, and such use is also linked to an increased risk of cerebral edema. However, as noted in the ADA guidelines, for patients with an arterial pH less than 6.9, in whom decreased cardiac contractility and peripheral vasodilatation can further impair tissue perfusion, and in patients with life-threatening hyperkalemia, treatment with intravenous sodium bicarbonate can be used. Pediatricians must recognize, however, that there are well-recognized adverse effects of sodium bicarbonate therapy, including paradoxical central nervous system acidosis, hypokalemia, and an increased risk of cerebral edema.\n\nAn intravenous bolus of insulin is unnecessary, may increase the risk of cerebral edema, and should not be routinely used at the start of therapy.\n\nPREP Pearls\n• In diabetic ketoacidosis (DKA), the first therapeutic intervention should be a slow bolus infusion of normal saline.\n• Intravenous sodium bicarbonate should not be used in the routine treatment of DKA in children.\n• In children who have DKA with an arterial pH less than 6.9 and evidence of hemodynamic instability, or life-threatening hyperkalemia, treatment with intravenous sodium bicarbonate can be used, although there are well-recognized risks with this treatment.\n\nAmerican Board of Pediatrics Content Specification(s):\n• Understand the risks of using bicarbonate in diabetic ketoacidosis\n\nSuggested Reading:\n• Dunger DB, Sperling MA, Acerini CL, et al; European Society for Paediatric Endocrinology; Lawson Wilkins Pediatric Endocrine Society. European Society for Paediatric Endocrinology/Lawson Wilkins Pediatric Endocrine Society consensus statement on diabetic ketoacidosis in children and adolescents. Pediatrics. 2004;113:e133-e140. doi:10.1542/peds.113.2.e133\n• Wolfsdorf J, Glaser N, Sperling MA; American Diabetes Association. Diabetic ketoacidosis in infants, children, and adolescents: a consensus statement from the American Diabetes Association. Diabetes Care. 2006;29:1150-1159. doi:10.2337/dc06-9909\n• Wolfsdorf J, Craig ME, Daneman D, et al. Diabetic ketoacidosis in children and adolescents with diabetes. Pediatr Diabetes. 2009;10(suppl 12):118-133. doi:10.1111/j.1399-5448.2009.00569.x"}
{"id" : 1291, "question_text" : "You are supervising a resident who is caring for a 3-year old boy who presented to your urgent care center. The boy's mother states that he was healthy until 2 days ago when he developed fever, nausea, vomiting, and diarrhea. His vomiting has decreased in the past 24 hours, but his diarrhea continues with 7 to 9 large, liquid bowel movements daily. The boy's temperature is 37.8°C, heart rate is 116 beats/min, and respiratory rate is 30 breaths/min. On physical examination, he is ill appearing, pale, and has tacky mucous membranes. The resident asks about the recommended approach to management of this child's hydration. The literature shows a clear advantage to oral rehydration. You discuss the mechanism of action of this treatment with the resident. Of the following, the transporter that is MOST critical to this treatment's mechanism of action is", "options" : "[\"sodium bicarbonate\", \"sodium fructose\", \"sodium glucose\", \"sodium potassium adenosine triphosphatase\", \"sodium potassium chloride\"]", "explanation" : "Correct Answer: C\nThe mechanism of action of oral rehydration solutions (ORS) involves the sodium-glucose transporter, which cotransports one sodium with one glucose (Item C39A). Water then follows by diffusion because of the concentration gradient of the sodium. There are many versions of ORS, each varying in carbohydrate and electrolyte concentration. ORS are significantly better than other oral fluid options for rehydration because of the relatively low carbohydrate load and elevated sodium and potassium levels that maximize hydration while minimizing osmotic loads that drive diarrhea. Studies have demonstrated increased safety, more rapid recovery, and cost effectiveness with the use of ORS compared with intravenous fluid hydration. Mild to moderately dehydrated patients who are able to take in the fluid without emesis should receive ORS. Patients with diarrhea do well with ORS because of the low osmotic load and excellent absorption. Item C39B details the composition of ORS.\n\nThe sodium potassium adenosine triphosphate transporter, also known as the Na+/K+ pump, actively pumps sodium out of, and potassium into, cells. Adenosine triphosphate is used to move both electrolytes against their concentration gradients.\n\nThe sodium potassium chloride (NKCC) transporter is a membrane transport protein with 2 forms that move sodium, potassium, and chloride in and out of the cell in the same direction. NKCC1 is found in all fluid-secreting organs, such as the kidney, where it helps in the reabsorption of sodium, potassium, and chloride. NKCC2 is found in nephrons in cells of the thick ascending limb of the loop of Henle, where it aids in the reabsorption of sodium.\n\nThe sodium bicarbonate transporter mediates the coupled movement of Na+ and HCO3− across plasma membranes, and is vital in maintaining tissue pH levels.\n\nFructose transportation is typically independent of sodium, via the Glut-5 transporter.\n\nPREP Pearls\n• The sodium glucose transporter improves hydration through diffusion of water due to increased sodium concentration.\n• Oral rehydration solutions (ORS) are the best rehydration products.\n• Because of a low osmotic load and their electrolyte content, ORS help to minimize diarrhea and stabilize electrolyte levels.\n• Oral rehydration solutions with low sodium levels are less effective.\n\nABP Content Specifications(s)\n• Understand the role of oral rehydration solutions in the treatment of acute diarrheal dehydration\n• Understand the differences between and the rationale for the composition of oral rehydration solutions\n\nSuggested Readings\n• Guarino A, Dupont C, Gorelov AV, et al.The management of acute diarrhea in children in developed and developing areas: from evidence base to clinical practice. Expert Opin Pharmacother. 2012;13(1):17-26. doi: http://dx.doi.org/10.1517/14656566.2011.634800.\n• Rouhani S, Meloney L, Ahn R, Nelson BD, Burke TF. Alternative rehydration methods: a systematic review and lessons for resource-limited care. Pediatrics. 2011;127(3):e748-e757. doi: http://dx.doi.org/10.1542/peds.2010-0952.\n• Unger CC, Salam SS, Sarker MS, Black R, Cravioto A, El Arifeen S. Treating diarrhoeal disease in children under five: the global picture. Arch Dis Child. 2014;99(3):273-8. doi: http://dx.doi.org/10.1136/archdischild-2013-304765."}
{"id" : 3638, "question_text" : "A 12-year-old boy is brought to the emergency department for evaluation of 6 hours of worsening abdominal pain that began after eating a breakfast of bacon and eggs. The pain is described as sharp and is present in the epigastrium and right upper quadrant. About 4 hours ago, he had nonbilious nonbloody emesis. There has been no fever, diarrhea, or hematochezia. He is otherwise healthy. He has no surgical history and reports no medication use (including over-the-counter drugs and dietary supplements). He has a weight of 80 kg (> 99th percentile for age), height of 150 cm (50th percentile for age), and body mass index greater than the 99th percentile for age. He is afebrile and has a heart rate of 120 beats/min, respiratory rate of 16 breaths/min, and blood pressure of 122/70 mm Hg. He is uncomfortable. His sclera are icteric, his abdomen is mildly distended, there is voluntary guarding, and his right upper quadrant is tender to palpation. Bowel sounds are not appreciated. Laboratory data are shown: Laboratory Test Result White blood cell count 12,500/µL (12.5 × 109/L) Hemoglobin 13.6 g/dL (136 g/L) Platelet count 238 × 103/µL (238 × 109/L) Sodium 140 mEq/L (140 mmol/L) Potassium 3.7 mEq/L (3.7 mmol/L) Chloride 100 mEq/L (100 mmol/L) Carbon dioxide 26 mEq/L (26 mmol/L) Alkaline phosphatase 600 U/L Bilirubin 5.3 mg/dL (90.1 µmol/L) Alanine aminotransferase 230 U/L Aspartate aminotransferase 216 U/L Lipase > 10,000 U/L. Of the following, the test MOST likely to establish the diagnosis in this boy is", "options" : "[\"abdominal radiography\", \"abdominal ultrasonography\", \"upper endoscopy\", \"upper gastrointestinal series\"]", "explanation" : "The boy in this vignette has acute abdominal pain due to choledocholithiasis (common bile duct stone) and resulting pancreatitis. Signs and symptoms of choledocholithiasis can include jaundice, right upper quadrant pain and tenderness, and fever. Ultrasonography is the initial test of choice to visualize gallstones and to evaluate for biliary tract dilation. Pancreatitis can occur as a result of an obstructing common bile duct stone. Signs and symptoms of pancreatitis include epigastric abdominal pain and tenderness and vomiting in the context of hyperlipasemia.\n\nThe differential diagnosis of acute abdominal pain in children is broad and ranges from benign functional abdominal pain to surgical emergencies. Initial evaluation of acute abdominal pain should start with a thorough history that includes:\n• Location and duration of the pain\n• Quality of the pain\n• Triggering factors (ie, movement)\n• Other signs and symptoms including stool pattern and appearance of emesis (if present)\n\nThe location of the pain (Item C82A), the color of the emesis (Item C82B), and the appearance of stool (Item C82C) can narrow the differential diagnosis.\n\nItem C82B: Differential Diagnosis Based on the Color of Emesis. Reprinted with permission from Ross A, LeLeiko NS. Acute abdominal pain. Pediatr Rev. 2010;31(4):137.\n\nThe physical examination should include assessment of the child's overall appearance (ie, toxic or nontoxic). The presence of distention, tenderness, guarding, or rebound tenderness should prompt consideration for a surgical emergency.\n\nLaboratory evaluation may include complete blood cell count and differential, complete metabolic panel, ɣ-glutamyl transferase, erythrocyte sedimentation rate, C reactive protein, amylase, and lipase. Urine testing may include a urine pregnancy test (in adolescent girls) and urinalysis. Radiographic evaluation should be considered and tailored to the data accumulated from the history, physical examination, and laboratory evaluation.\n\nCauses of abdominal surgical emergencies in infants include necrotizing enterocolitis (most commonly seen in preterm infants), pyloric stenosis, malrotation with volvulus, intussusception, and incarcerated hernias. For children between 1 and 5 years of age, causes of abdominal surgical emergencies include: appendicitis (commonly perforated in this age group), intussusception, and torsed Meckel diverticulum. Appendicitis and ovarian or testicular torsion are causes of abdominal surgical emergencies more commonly seen in older children. Small bowel obstruction from other causes (eg, previous abdominal surgery or abdominal tumors) can occur at any age.\n\nThe boy in this vignette has jaundice and epigastric and right upper quadrant pain, and his laboratory data is concerning for pancreatitis and biliary tract obstruction. Abdominal radiography would be a good first test if the history was concerning for a small bowel obstruction but will not generally be helpful in identifying causes of cholestasis or pancreatitis. Upper endoscopy would be useful if there is concern for peptic ulcer disease, but it is not helpful to understand causes of pancreatitis. Finally, an upper gastrointestinal series is not diagnostic for causes of pancreatitis or biliary tract disease.\n\nPREP Pearls\n• The evaluation of acute abdominal pain should include a thorough history and physical examination, focusing on location and quality of pain, triggering symptoms, appearance of emesis (if present), and stool pattern.\n• Causes of surgical abdominal emergencies in infants include necrotizing enterocolitis, pyloric stenosis, intussusception, malrotation with midgut volvulus, and incarcerated hernias.\n\nABP Content Specifications(s)\n• Plan the appropriate evaluation of acute abdominal pain\n• Recognize the significance of dyspepsia in a child with recurrent abdominal pain\n• Formulate an age-appropriate differential diagnosis of acute abdominal pain\n\nSuggested Readings\n• Loizides AM, Atienza Orellana K, Thompson JF. Abdominal pain. In: McInerny TK, Adam HM, Campbell DE, DeWitt TG, Foy JM, Kamat DM, eds. American Academy of Pediatrics Textbook of Pediatric Care. 2nd ed. Elk Grove Village, IL: American Academy of Pediatrics; 2017:1181-1188. Pediatric Care Online.\n• McCollough M, Sharieff GQ. Abdominal surgical emergencies in infants and young children. Emerg Med Clin N Am. 2003;21:909-935. doi:10.1016/S0733-8627(03)00090-7.\n• Ross A, LeLeiko NS. Acute abdominal pain. Pediatr Rev. 2010;31(4):135-144. doi:10.1542/pir.31-4-135."}
{"id" : 2978, "question_text" : "A 1-month-old female infant is being seen for a health supervision visit. She was born at 38 weeks' gestation via cesarean delivery because of breech presentation. Her physical examination is significant for a positive Ortolani maneuver on the right. Ultrasonography of the hip confirms the suspected diagnosis. Of the following, the MOST likely mechanism for her condition is", "options" : "[\"association\", \"deformation sequence\", \"disruption sequence\", \"malformation syndrome\"]", "explanation" : "Correct Answer: B\nThe neonate in the vignette has developmental dysplasia of the right hip (DDH). Developmental dysplasia of the right hip results from abnormal alignment and movement of the head of the femur within the acetabulum in utero. When the femoral head is not correctly approximated within the acetabulum, the acetabulum does not develop properly. Deformation sequence is the best explanation for the clinical presentation of DDH. A deformity caused by a deformation sequence results from abnormal mechanical or structural external forces, rather than an intrinsic abnormality of the fetus.\n\nIn the case of disruption sequence, a normally developing fetus experiences an event or exposure that alters the normal course of development; this may have a vascular, infectious, mechanical, or metabolic cause. Amniotic band syndrome is an example of a disruption sequence. Malformation sequence is abnormal tissue development leading to congenital anomalies, such as atrioventricular canal defect in trisomy 21. An association like VACTERL (vertebral anomalies, cardiac defect, tracheoesophageal fistula, renal anomalies and limb anomalies) is an example of a collection of anomalies commonly seen together without an identified genetic mutation. Developmental dysplasia of the hip affects 1 in 1,000 live births in the United States. Risk factors for DDH include female sex, breech presentation, positive family history, and incorrect swaddling techniques (Item C180A). Swaddling should allow for hip flexion and abduction, as well as knee flexion (Item C180B and Item C180C). Surveillance for DDH should include ongoing assessment at each health supervision visit of hip mobility (range of motion) and hip stability (Ortolani and Barlow maneuvers) through age 12 weeks. Assessment for limitation of hip abduction should continue until the child is ambulating with a normal age-appropriate gait. Care should be taken during the Barlow maneuver to provide support to the hip joint and avoid posterior displacement.\n\nPREP Pearls\n• Developmental dysplasia of the hip results from a deformation sequence: improper alignment of the femoral head with the acetabulum impairs proper development of the hip joint.\n• Risk factors for developmental dysplasia include female sex, breech presentation, positive family history, and incorrect swaddling techniques.\n• Appropriate swaddling allows for hip flexion and abduction as well as knee flexion.\n\nABP Content Specifications(s)\n• Recognize the anatomic effects of amniotic bands\n• Understand how positional deformations and/or malformations develop in a fetus\n\nSuggested Readings\n• Nemeth BA, Narotam V. Developmental dysplasia of the hip. Pediatr Rev. 2012;33:553. doi: 10.1542/pir.33-12-553.\n• Shaw BA, Segal LS; Section on Orthopaedics. Evaluation and referral for developmental dysplasia of the hip in infants. Pediatrics. 2016;138:e1-e11. doi: 10.1542/peds.2016-3107.\n• Slavotinek AM. Dysmorphology. In: Kliegman RM, St Geme JW, Blum NJ, Shah SS, Tasker RC, Wilson KM. Nelson Textbook of Pediatrics. Philadelphia, PA: Elsevier; 2020:986-996."}
{"id" : 1516, "question_text" : "A 4-month-old infant with trisomy 21 is brought to your office for a health supervision visit. She was born by elective cesarean delivery at 38 weeks of gestation to a 37-year-old gravida 2 para 1 mother. A small, asymptomatic ventricular septal defect was diagnosed at birth. Her parents report a new concern of abnormal eye movements, described as occasional rhythmic beating followed by a normal focused gaze. The infant has been clinically well, with growth and development appropriate for her genetic condition. On physical examination, the infant has facial features consistent with trisomy 21. Her pupils are equal, round, and reactive to light. There is a subtle asymmetry of the brightness of the red reflexes. The corneal light reflex is centrally located. The infant is able to fix and follow past midline horizontally with conjugate eye movement. You do not appreciate any abnormal eye movements. Of the following, BEST next management step for this infant is", "options" : "[\"computed tomography of the brain and eyes\", \"thyroid function tests\", \"urgent referral to ophthalmology\", \"urine specimen for reducing substances\", \"watchful waiting and follow-up in 2 months\"]", "explanation" : "The infant in the vignette has clinical findings suggestive of congenital cataracts, for which an urgent referral to ophthalmology is warranted. The clinical presentation of congenital cataracts in infants may include:\n• Asymmetric retinal red reflexes\n• Leukocoria\n• Photophobia\n• Strabismus\n• Nystagmus\n• Decreased visual acuity\n\nParental report of abnormal eye movements should always be taken seriously, even when the findings are reported as intermittent and are not evident on physical examination.\n\nMany pediatric disorders, including trisomy 21, are associated with cataracts. A cataract is an opacification of the lens that may occur bilaterally or unilaterally, and may vary in size and location. The larger the cataract, the greater the risk is that it will negatively affect visual development. Trisomy 21 is also associated with nystagmus, which this infant's history suggests; however, nystagmus may also be a sign of poor vision. Although this infant has the ability to fix and follow past midline with conjugate eye movements, centralized corneal light reflexes, and pupils that are equal, round, and reactive, the possibility of a serious ophthalmologic disorder is not excluded. Urgent referral to a pediatric ophthalmologist is the next best step in management. Watchful waiting with follow-up in 2 months is not appropriate because early detection and prompt intervention are critical to optimize visual outcomes.\n\nThe performance of a thorough, age-appropriate eye examination is crucial at each health supervision visit, as well as at any time a concern is raised. This examination should include assessment of the external eye anatomy, ocular motility, and ability to fix and follow, as well as direct ophthalmoscopic examination of the pupil and evaluation of the retinal red reflex. The examiner should view the eyes simultaneously for comparison. The color of the normal retina varies between individuals, but should be consistent for both of an individual's eyes. If the retina appears black, white, asymmetric, or dim, then concern for an abnormal red reflex is raised. The red reflex represents the reflection of the examiner's light from the retina, so an abnormal red reflex can be caused by retinal disorders (eg, retinoblastoma) or anterior eye disorders (eg, cataract).\n\nThe infant in the vignette has been clinically well, with growth and development appropriate for her genetic condition. Therefore, evaluation for causes of cataract with computed tomography of the brain and eyes or a urine specimen for reducing substances is not appropriate at this time. Although it is important to screen routinely for hypothyroidism in patients with trisomy 21, hypothyroidism is not a cause of cataracts.\n\nPREP Pearls\n• A concern for congenital cataract warrants an urgent ophthalmologic evaluation.\n• Clinical features of congenital cataracts include asymmetric retinal red reflex, leukocoria, photophobia, strabismus, nystagmus, and decreased visual acuity.\n\nABP Content Specifications(s)\n• Recognize the clinical findings associated with congenital cataracts\n\nSuggested Readings\n• Olitsky SE, Hug D, Plummer LS, Stahl ED, Ariss MM, Lindquist TP. Abnormalities of the lens. In: Kliegman RM, Stanton BF, St Geme JW III, Schor NF, Behrman RE, eds. Nelson Textbook of Pediatrics. 20th ed. Philadelphia, PA: Saunders Elsevier; 2015:3044-3047.\n• Rogers GL, Jordan CO. Pediatric vision screening. Pediatr Rev. 2013;34(3):126-133. doi: http://dx.doi.org/10.1542/pir.34-3-126."}
{"id" : 3645, "question_text" : "A 3-year-old previously healthy, fully immunized boy is brought to the emergency department after several hours of progressive lethargy. Earlier in the day, he was in his usual state of health, without fever, decreased activity, respiratory symptoms, pain, or decreased appetite. He has not had any sick contacts or history of trauma. He has a temperature of 37°C, heart rate of 80 beats/min, respiratory rate of 12 breaths/min, blood pressure of 75/40 mm Hg, and oxygen saturation of 92% on room air. He appears lethargic. When stimulated, he opens his eyes, mumbles a few words, and moves the examiner's hands away. Pupils are pinpoint. Muscle tone is decreased, and deep tendon reflexes are 2+ throughout. Cough and gag reflexes are present. Mucous membranes are moist. He is breathing slowly but comfortably. His lungs are clear to auscultation bilaterally. His heart has a regular rate and rhythm. His abdomen is soft, nontender, and nondistended. His extremities are warm with a capillary refill time of 2 seconds. Pulses are strong throughout. Capillary blood gas data are shown:\n\nLaboratory Test: Result\npH: 7.20\nPCO2: 55 mm Hg\nPO2: 50 mm Hg\nHCO3: 24 mEq/L\nBase excess: 2 mEq/L\n\nOf the following, the MOST appropriate next step in management is", "options" : "[\"endotracheal intubation\", \"intravenous naloxone\", \"noninvasive positive-pressure ventilation\", \"oxygen, 100% nonrebreather facemask\"]", "explanation" : "The previously healthy boy in this vignette has a sudden onset of lethargy, pinpoint pupils, and hypopnea. Evaluation shows respiratory acidosis and hypoxia. These findings suggest an opioid toxidrome. The most appropriate next step in management is intravenous naloxone.\n\nThe 2 main functions of the respiratory system are providing oxygen and removing carbon dioxide from the blood. Accordingly, the 2 major types of respiratory failure are hypoxic and hypercarbic. Hypoxic respiratory failure is characterized by insufficient oxygenation of pulmonary arterial blood. Oxygenation occurs when oxygen diffuses from open, oxygen-containing alveoli to adjacent pulmonary capillaries. Pulmonary capillary blood that perfuses alveoli that are fluid filled or collapsed returns to the heart deoxygenated, contributing to hypoxia, a process called intrapulmonary shunting. Other causes of hypoxia include barriers to diffusion such as in pulmonary fibrosis and pulmonary edema, and decreased pulmonary blood flow such as that caused by pulmonary embolism, right-sided heart failure, right-to-left intracardiac shunting, and pulmonary hypertension. While oxygenation is dependent on ventilation as well as other factors, removal of carbon dioxide is solely proportional to ventilation. Hypercarbic respiratory failure is caused by disruption of ventilation from respiratory pump failure or airway obstruction anywhere in the tracheobronchial tree and the nasopharyngeal airway. Respiratory pump failure can be caused by central nervous system (CNS) depression as well as acute, chronic, or anatomic lesions affecting the function of the diaphragm or accessory respiratory muscles.\n\nAs described for the boy in this vignette, hypoxic and hypercarbic respiratory failure can coexist. Inadequate ventilation can cause decreased alveolar PO2 by several different mechanisms. Decreased air intake leads to less oxygen in the alveoli available for gas exchange. Inadequate alveolar inflation can also cause de-recruitment of alveoli, especially during exhalation, and intrapulmonary shunting. Increased PCO2 from inadequate ventilation can decrease the PO2 in accordance with the ideal gas principle.\n\nObtaining pH, PaCO2, PaO2, serum bicarbonate levels, and base deficit/excess from an arterial blood gas sample is helpful in investigating the severity and causes of respiratory failure, as well as acid-base disturbances. Since CO2 combines with H2O to produce carbonic acid (H2CO3), elevations in pCO2 decrease the pH. Chemoreceptors in the CNS respiratory center are exquisitely sensitive to the cerebrospinal fluid pH, as well as to PaO2. With acute elevations in pCO2 significant acidemia occurs due to the limited buffering by plasma proteins. Acutely, the serum bicarbonate increases only 1 mEq/L but the pH decreases by approximately 0.08 for every 10 mm Hg increase in PaCO2. In contrast, in chronic respiratory failure the pH remains closer to normal due to renal compensation, which takes several days to occur. With renal compensation the pH decreases by 0.03 and the serum bicarbonate level increases by approximately 3.5 mEq/L for every 10 mm Hg increase in PaCO2. Since obtaining an arterial blood gas sample may be more difficult in children, a capillary or venous blood gas sample will provide reliable information about pH, pCO2, base deficit/excess, and bicarbonate levels.\n\nBased on the sudden onset of lethargy and hypoventilation and the clinical signs of pinpoint pupils, hypotension, and respiratory acidosis and hypoxia, the boy in this vignette likely has an opioid ingestion. Naloxone is a competitive opioid receptor antagonist that can be used to reverse respiratory depression and altered mental status caused by opioid overdose. It is most commonly only given intravenously, although it can also be given intramuscularly, subcutaneously, or intranasally. A clinical effect from naloxone usually occurs within 1 minute. If no effect is seen within several minutes, doses can be repeated. The serum half-life is approximately 30 minutes, so repeated doses and/or continuous infusions are often required for a sustained effect. For the boy in the vignette, the mechanism of respiratory failure is from hypoventilation leading to hypercarbia and hypoxia. Respiratory failure can be managed by endotracheal intubation and mechanical ventilation, but this approach is too invasive to use prior to attempting treatment with naloxone. Intubation and mechanical ventilation may not be necessary because the boy has an intact cough and gag, and thus may be able to protect his airway. Furthermore, bag-valve mask ventilation and intubation could be risky if he has a full stomach. Noninvasive positive-pressure ventilation can be used to recruit alveoli in hypoxic respiratory failure and in some cases of upper airway obstruction, but it is less effective in CNS depression. In fact, it can lead to aspiration of stomach contents if the airway is not adequately protected. Oxygen can be harmful for the boy in this vignette because it can blunt the component of respiratory drive from hypoxia, leading to worsened hypoventilation.\n\nPREP Pearls\n• Naloxone is a useful reversal agent for opioid overdose.\n• The pH decreases significantly in acute respiratory acidosis because renal compensation for respiratory acidosis requires several days.\n• Oxygen can be a toxic therapy in patients with respiratory failure from central nervous system depression; instead, treatment of the underlying condition should be sought.\n\nABP Content Specifications(s)\n• Understand the pulmonary mechanism for regulating acid-base physiology\n• Recognize the clinical and laboratory manifestations associated with respiratory failure of various etiologies\n\nSuggested Readings\n• Abdo WF, Heunks LM. Oxygen-induced hypercapnia in COPD: myths and facts. Crit Care. 2012;16(5):323. doi:10.1186/cc11475.\n• Brinkman JE, Sharma S. Physiology, respiratory drive. StatPearls [Internet]. Treasure Island, FL: StatPearls Publishing; 2018.\n• Hsu BS, Lakhani SA, Wilhelm M. Acid-base disorders. Pediatr Rev. 2016;37(9):361-369. doi:10.1542/pir.2015-0093.\n• Vo P, Kharasch VP. Respiratory failure. Pediatr Rev. 2014;35(11):476-484. doi:10.1542/pir.35-11-476."}
{"id" : 3791, "question_text" : "A 4-month-old male infant is seen for a health supervision visit. His mother has no concerns and feels he is eating well and developing normally. His vital signs are within normal limits. He has a normal sexual maturity rating stage 1 penis. The right testicle is palpated in the scrotum, but the left testicle cannot be palpated. The remainder of his physical examination findings are normal. The patient is referred to a urologist, and the diagnosis is explained to his mother. To reduce the risk of complications, treatment for this condition should be completed by the age of", "options" : "[\"6 months\", \"12 months\", \"18 months\", \"24 months\"]", "explanation" : "Correct Answer: B\nThe most recent international guidelines state that orchiopexy to treat cryptorchidism or undescended testicle should be completed by 12 months of age. During normal development, the testes form in the abdomen and then descend into the scrotum. Having one or both testes undescended at birth is not uncommon (2%-9% of male neonates), but only 1% remain undescended at 3 months of age. Those infants with testes that remain undescended require surgery to bring the testicle into the scrotum (orchiopexy) or remove the testicle (orchiectomy) because they will not spontaneously descend from the abdomen into the scrotum after this age.\n\nIf a testicle is palpated in the scrotum at times or easily brought into the scrotum on examination, it is considered a retractile testicle and not an undescended testicle. This does not require surgical correction but does increase the risk of developing an ascending or acquired cryptorchidism later in life. A retractile testicle is likely a result of a lack of formation of attachment within the scrotum after the testes descend.\n\nAn undescended testicle that remains in the abdomen is exposed to body temperature instead of the preferred 2°F to 3°F cooler temperature of the scrotum. The increased temperature can inhibit differentiation of the germ cells as early as 4 months of age and can lead to decreased fertility and increased risk of malignancy. Due to the location in the abdominal cavity, if a malignancy develops in an undescended testis, the diagnosis is often delayed until there are systemic symptoms or the mass is large. There is also an increased risk of testicular torsion, which can be difficult to diagnose due to the intra-abdominal location.\n\nDue to the risk of complications, especially decreased fertility and malignancy, waiting until 18 or 24 months for surgical treatments is not recommended. Surgery can be completed as early as 6 months of age.\n\nPREP Pearls\n• Individuals with undescended testes are at increased risk of testicular malignancy.\n• Individuals with untreated cryptorchidism are at risk of decreased fertility.\n• Surgical treatment of undescended testes is recommended between 6 and 12 months of age.\n\nABP Content Specifications(s)\n• Differentiate the findings associated with undescended testes from those of retractile testes\n• Recognize complications associated with undescended testes\n• Plan the appropriate management of undescended testes\n\nSuggested Readings\n• Inouye B, Tourchi A, Gearhart JP. Hypospadias, epispadias, and cryptorchidism. In: McInerny TK, Adam HM, Campbell DE, DeWitt TG, Foy JM, Kamat DM, eds. American Academy of Pediatrics Textbook of Pediatric Care. 2nd ed. Elk Grove Village, IL: American Academy of Pediatrics; 2017:2180-2184. Pediatric Care Online.\n• Schneuer F, Holland A, Pereira G, Jamieson S, Bower C, Nassar N. Age at surgery and outcomes of an undescended testis. Pediatrics. 2016:137(2):e220152768. doi:10.1542/peds.2015-2768.\n• Urology Care Foundation. What are undescended testicles (cryptorchidism)? https://www.urologyhealth.org/urologic-conditions/cryptorchidism."}
{"id" : 3333, "question_text" : "A 9-year-old boy is brought to your office by his parents, who are worried about his nutritional status. The child was diagnosed with autism spectrum disorder at age 4 years. During his last office visit 14 months ago, he weighed 24 kg and his body mass index was approximately 17. Over the past year, he has frequently refused to eat meals, and he avoids solid foods. His diet is composed of mostly water, apple juice, and some mashed potatoes. His current body mass index is 15. You recommend an increase in his daily energy intake, to include a liquid nutritional supplement. He returns to the office 2 weeks later, having lost an additional kg of body weight. On physical examination, he appears cachectic. Vital signs show a resting heart rate of 55 beats/min and a blood pressure of 80/55 mm Hg. You elect to admit him to the hospital for nutritional rehabilitation. Initial laboratory studies include the following: Complete blood cell count, within normal limits; Sodium, 139 mEq/L (139 mmol/L); Potassium, 3.5 mEq/L (3.5 mmol/L); Chloride, 108 mEq/L (108 mmol/L); Bicarbonate, 22 mEq/L (22 mmol/L); Glucose, 95 mg/dL (5.3 mmol/L). After discussing the situation with his parents, you begin nasogastric feeding, which initially provides 2,100 kcal per day. Three days after commencing this regimen, he appears weak with difficulty getting out of bed. Laboratory studies are ordered, and these demonstrate the following: White blood cell count, 3,500/µL (3.5 x 109/L); Hemoglobin, 13.5 g/dL (135 g/L); Sodium, 135 mEq/L (135 mmol/L); Potassium, 2.8 mEq/L (2.8 mmol/L); Chloride, 105 inEq/L (105 mrnol/L); Bicarbonate, 22 mEq/L (22 mmol/L); Calcium, 7.5 mg/dL (1.88 mmol/L); Phosphorus, 1.8 mg/dL (0.58 mmol/dL); Magnesium, 1.0 mEq/L (0.5 mmol/L); Glucose, 115 mg/dL (6.4 mmol/dL). Of the following, you are MOST likely to recommend", "options" : "[\"continuous enteral feedings\", \"increasing calorie intake to 2,500 kcal per day\", \"reducing calorie intake to 900 kcal per day\", \"supplementing enteral feedings with calcium and electrolytes\", \"total parenteral nutrition\"]", "explanation" : "The boy described in the vignette manifests protein-energy malnutrition secondary to an autism-related behavioral eating disorder. He must be hospitalized because of continued weight loss and physical findings that indicate severe malnutrition. The boy has started receiving enteral feedings via the nasogastric route with a total intake of 2,100 kcal/per day. This level of energy consumption represents the approximate daily recommended intake for an ambulatory 50 kg individual (when using the calculation of 100 kcal/kg for the initial 10 kg body weight, 50 kcal/kg for the second 10 kg, and 20 kcal/ kg for each kg of body weight above 20 kg). However, 3 days after instituting this regimen, he develops muscle weakness and fatigue, along with hypocalcemia, hypokalemia, hypomagnesemia, and hypophosphatemia. These findings suggest the development of refeeding syndrome, which is the consequence of overly aggressive nutritional management of the malnourished patient. Feedings should be introduced more slowly in this clinical setting. Following correction of electrolyte deficits, feedings should be restarted at a rate that provides about 50% of targeted intake. Using a calculation based upon this child's \"normally nourished\" weight 14 months ago, he should receive approximately 900 kcal per day. Refeeding syndrome is a well-described phenomenon that develops as a consequence of specific metabolic events that accompany aggressive energy supplementation. Patients particularly at risk include those with the following conditions:\n• Anorexia nervosa\n• Kwashiorkor or marasmus\n• Chronic malnutrition\n• Chronic alcoholism\n• Prolonged fasting\n• Prolonged intravenous hydration\n• Metabolic stress (eg, chronic disease) and nutrient depletion\n\nDuring periods of starvation, insulin levels fall, promoting release of glucose and free fatty acids for energy. Thyroid hormone levels also are reduced resulting in a lower metabolic rate, and insulin like growth factor 1 levels fall, reducing protein synthesis. When glycogen stores are exhausted, upregulation of gluconeogenesis from protein catabolism leads to water, vitamin, and mineral depletion. A sudden, marked increase in carbohydrate intake, as with refeeding, will raise serum insulin and lower serum glucagon levels.\n\nIncreased cellular glucose uptake then promotes intracellular movement of phosphate, potassium, and magnesium, while increased utilization of vitamins (including thiamine) and adenosine triphosphate lead to a deficiency state. Clinically, multiple organ systems may be affected, resulting in muscle weakness, seizures, cardiac arrhythmias, hypotension, and ileus. To avoid the refeeding syndrome, nutritional rehabilitation of patients who are at high risk for refeeding syndrome should proceed as follows:\n1. Correct electrolyte abnormalities.\n2. Institute oral or enteral nutrition.\na. Start at a calculated intake of about 50% targeted energy consumption.\nb. Provide vitamin and trace mineral supplements to meet age-appropriate recommended daily intakes.\nc. Advance energy and volume intake slowly.\n3. Closely monitor vital signs, intake and output, and serum electrolyte levels.\n\nThe decision to institute bolus versus continuous feedings via the enteral route will depend on the patient's clinical status and gastrointestinal motility. Severely malnourished subjects may benefit from a slow, continuous infusion rate to avoid problems created by rapid hormonal changes, leading to marked fluid and electrolyte shifts. Severe malnutrition is frequently associated with electrolyte disturbances and alterations in gastrointestinal (GI) motility, including gastroparesis. Great care must be taken before instituting an oral or enteral feeding program. In severely malnourished patients before and during refeeding, correction of fluid and electrolyte abnormalities should be achieved via the intravenous route, and not by adding electrolyte to the enteral feeding regimen. A period of parenteral nutrition may be required in those situations in which GI function is significantly compromised. For the boy described in the vignette, the initial decision to employ enteral feedings was appropriate, and either the bolus or continuous route would have been an acceptable alternative. However, because this patient's gastrointestinal tract has already tolerated bolus enteral feedings, a significant reduction in energy intake should represent the initial modification in the nutrition support program.\n\nWhen deciding upon an appropriate nutritional support regimen for the hospitalized patient, a simple but dear axiom should always prevail: \"If the gut works, use it.\" Parenteral nutrition should be reserved only for those situations in which GI motility or absorptive function are compromised. Even in those cases, the availability of either partially or completely hydrolyzed formulas has greatly enhanced the ability to provide adequate nutrition via the enteral route. In general, peripheral venous nutrition is employed in patients who are unable to receive oral or enteral nutrition for periods from 3 to 7 days duration and total parenteral nutrition is instituted when patients must consume nothing by mouth (nil per os) for more than 7 days. Although enteral feedings pose fewer significant risks than parenteral nutrition, including lower risks of infection, fluid and electrolyte disturbances, and catheter malfunction, care must be taken to avoid problems related to infusion rate and composition. Item C216, page C-185, lists the most common tube feeding-related complications, including those metabolic problems associated with refeeding syndrome. Many of the mechanical tube-related complications have been obviated by the development of softer feeding tubes (polyurethane) that pose a far lower risk for perforation and mucosal irritation.\n\nItem (216). Most Common Complications of Enteral Nutrition\n• Mechanical\n- Malposition (tube placement into the trachea, pneumothorax)\n- Blockage\n- Sinusitis, otitis media\n- Laryngeal ulceration\n- Tracheoesophageal fistula - Variceal rupture\n- Duodenal perforation\n• Gastrointestinal\n- Diarrhea or constipation - Abdominal distension\n- Gastroesophageal reflux, esophagitis - Pulmonary aspiration\n• Metabolic (including refeeding syndrome)\n- Vitamin, mineral, trace element deficiencies\nn - Abdominal distension\n- Gastroesophageal reflux, esophagitis - Pulmonary aspiration\n• Metabolic (including refeeding syndrome)\n- Vitamin, mineral, trace element deficiencies\n- Essential fatty acid deficiency (with low-fat formulas)\n- Hyperglycemia\n- Hyper- or hypokalemia\n- Hypophosphatemia\n- Hypomagnesaemia\n\nPREP Pearls\n• To prevent refeeding syndrome in a malnourished patient, the initial dietary regimen should provide approximately 50% of calculated energy requirements.\n• In addition to standard serum electrolytes (sodium, potassium, calcium), magnesium and phosphorus levels should be monitored during refeeding.\n• Vitamin supplements should be given during refeeding of malnourished patients because clinical deficiency states (eg, thiamine) may develop during the refeeding period.\n\nAmerican Board of Pediatrics Content Specification(s)\n• Identify the clinical and laboratory features associated with refeeding syndrome\n\nSuggested Reading\n• ASPEN Board of Directors and the Clinical Guidelines Task Force. Guidelines for the use of parenteral and enteral nutrition in adult and pediatric patients. 'PEN 1 Pa renter Enteral Nutr. 2002;26(1 Suppl):1SA138SA. doi:10.1177/0148607102026001011.\n• Bankhead R, Boullata J, Brantley S, et al. Enteral nutrition practice recommendations. WEN J Pa renter Enteral Nutr. 2009;33(4;122-167. doi:10.1177/0148607108330314.\n• Fisher M, Simpser E, Schneider M. Hypophosphatemia secondary to oral refeeding in anorexia nervosa. Int J Eat Disord. 2000; 28 (2):181-187. 10.1002/1098-108X(200009)28:2<181::A1D-EAT7>3.0.0O2-K. Fuentebella J, Kerner JA. Refeeding syndrome. Pediatr Clin North Am. 2009;56(5)120/-1210. doi:10.1016/j.pc1.2009.06.006."}
{"id" : 2398, "question_text" : "A healthy 6-month-old infant is undergoing evaluation of 1 day of nonbilious, nonbloody vomiting followed by nonbloody diarrhea for the past 2 days. She had a fever of 38.5 °C on the first day of illness that subsequently resolved. The infant continues to have good intake and urine output. She received her routine 6-month vaccines, including the rotavirus vaccine, 1 week before her symptom onset. She attends day care but has otherwise had no infectious exposures, including travel, undercooked foods, or untreated water sources. The infant has been growing appropriately. Her vital signs are within normal limits, and she appears well-hydrated and playful. Her physical examination findings are unremarkable. Of the following, the BEST next step in this infant's care is to obtain", "options" : "[\"a blood culture\", \"a stool sample for viral testing\", \"a urinalysis and urine culture\", \"no testing at this time\"]", "explanation" : "The infant in the vignette most likely has viral gastroenteritis. She is well hydrated and her fever has resolved, so no testing is indicated at this time. If she was febrile, it would be appropriate to obtain a urinalysis and urine culture to assess for a urinary tract infection. If she was still febrile and was moderately or severely ill, requiring hospitalization, a blood culture would be appropriate. Concerning symptoms such as bloody diarrhea, prolonged diarrhea, severe dehydration, or a known exposure to a bacterial intestinal pathogen (eg, Salmonella) should prompt additional testing, including stool testing for bacterial and/or parasitic pathogens.\n\nViral gastroenteritis is common in infants and children. Before the introduction of the vaccine, rotavirus was the most common cause of pediatric, community-acquired gastroenteritis. Rotavirus usually presents with vomiting for 1 to 2 days, followed by diarrhea. High fevers occur in up to one-third of affected children. The entire illness usually lasts 3 to 7 days. Some children experience profound dehydration, especially those who have immunodeficiencies. Rarely, seizures may occur.\n\nRotavirus is transmitted via the fecal-oral route. The virus can survive for months on fomites. Rarely, it can be passed through contaminated food and water. In the United States, infections are most commonly seen in odd years in the late winter and early spring.\n\nTesting for rotavirus in a fully vaccinated child is of low yield. Additionally, the virus can be detected for at least 10 days after receiving the vaccine, and this child received a rotavirus vaccine 1 week before symptom onset. Immunoassays and polymerase chain reaction tests may detect rotavirus, but also may detect other viruses that may or may not be causing symptoms.\n\nSuggested Reading(s)\nAmerican Academy of Pediatrics. Rotavirus infections. In: Kimberlin DW, Barnett ED, Lyfield R, Sawyer MH, eds. Red Book: 2021–2024 Report of the Committee on Infectious Diseases. 32nd ed. American Academy of Pediatrics; 2021:chap 347 Accessed November 14, 2023.\nCortese MM, Haber P. Rotavirus. In: Hall E, Wodi AP, Hamborsky J, Morelli V, Schillie S, eds. Epidemiology and Prevention of Vaccine-Preventable Diseases. 14th ed. Public Health Foundation; 2021:chap 19. www.cdc.gov/vaccines/pubs/pinkbook/rota.html\n\nContent Domain\nInfectious Diseases\n\nABP Content Specification(s) / Content Area(s)\nUnderstand the epidemiology of rotavirus infection\nRecognize the clinical features associated with rotavirus infection\nPlan the appropriate diagnostic evaluation for rotavirus infection, and recognize when diagnostic evaluation may not be necessary"}
{"id" : 1597, "question_text" : "A 5-year-old previously healthy girl presents to your office for evaluation of abdominal pain that has been worsening over the past 12 hours. Three days ago, the girl began to complain of abdominal pain and had a few episodes of nonbilious vomiting, along with a low-grade fever. At that time, she was seen by one of your colleagues, who documented that on physical examination, she had a soft abdomen with active bowel sounds, along with very mild periumbilical tenderness. A rapid Strep test was negative and she was diagnosed with acute viral gastroenteritis. Your colleague advised the family to encourage the girl to drink fluids, and to follow up in 48 to 72 hours if her abdominal pain did not improve. Today, her abdominal pain is significantly worse. Her pain seemed better last evening, but when she awoke this morning, she complained of severe abdominal pain and has been refusing to walk. She has been refusing to eat or drink anything over the past day, has had intermittent episodes of nonbilious vomiting, and has continued to have fever. On physical examination, the girl's temperature is 39°C, heart rate is 150 beats/min, respiratory rate is 32 breaths/min, blood pressure is 90/60 mm Hg, and pulse oximetry is 99% on room air. She is ill-appearing and is lying very still on your examination table, with her legs drawn up in a \"fetal position.\" Her mucous membranes appear dry and her eyes are sunken. Her tympanic membranes and oropharynx are normal. Her lungs are clear to auscultation, but she is taking shallow, rapid breaths with intermittent grunting. The girl's abdomen is rigid and her bowel sounds are sparse. She cries in pain as soon as you begin to palpate her abdomen and tries to push your hand away. Her extremities are cool and clammy. You note no rashes. Urinalysis reveals 3+ ketones and 1+ leukocyte esterase, but is otherwise unremarkable. Of the following, the girl's MOST likely diagnosis is", "options" : "[\"appendicitis with perforation\", \"diabetic ketoacidosis\", \"lower lobe pneumonia\", \"malrotation with midgut volvulus\", \"mesenteric adenitis\"]", "explanation" : "Correct Answer: A\nThe constellation of signs and symptoms displayed by the 5-year-old girl in the vignette are most consistent with the diagnosis of acute appendicitis complicated by perforation. She is in need of immediate transfer to an emergency department for stabilization, emergent evaluation, and management by a pediatric surgeon.\n\nAcute appendicitis is the most common indication for emergency abdominal surgery in pediatric patients. All pediatric providers must be able to recognize the clinical features associated with appendicitis. Making this diagnosis can be challenging, because the initial signs and symptoms can be quite similar to those of many other common nonsurgical intra-abdominal processes, including self-limited viral syndromes. Furthermore, not all children with appendicitis present with the classic \"textbook\" manifestations.\n\nAppendicitis results from obstruction of the appendix due to inflammation in the appendiceal wall or a fecalith. This inflammatory process most commonly affects children 9 to 12 years of age. Although it is quite rare in children younger than 2 years, cases of infants with appendicitis have been reported. The diagnosis can be especially challenging in younger children (<5 years of age), because they often have atypical presentations, along with a decreased ability to communicate their symptoms.\n\nThe classic abdominal pain associated with acute appendicitis develops gradually, beginning as vague and poorly localized periumbilical pain, which worsens in severity and localizes to the right lower abdomen as the inflammatory process progresses. Commonly associated symptoms include nausea, anorexia, decreased activity level, and fever. Affected patients may also have vomiting (typically preceded by pain) and often experience increased abdominal pain with movement (eg, coughing, hopping, or hitting \"bumps\" during a car ride).\n\nAt the time of appendiceal perforation, there may appear to be a rapid clinical improvement, because of a sudden decrease in intraluminal pressure in the appendix, which transiently decreases the associated pain. Over the subsequent 24 hours, the child's clinical status worsens dramatically with manifestations such as peritoneal signs (abdominal rigidity with marked tenderness, rebound tenderness, and decreased or absent bowel sounds), high fever, systemic toxicity, and even findings of septic shock.\n\nBecause the anatomic position of the appendix varies in pediatric patients, localization of pain and abdominal tenderness may not always be at the classic McBurney point in the right lower abdominal quadrant as expected with appendicitis. For example, children with an appendix located in the lateral gutter may present with flank pain and lateral abdominal tenderness, whereas those with an appendix oriented toward the pubis may have tenderness near the pubis, diarrhea, and signs of bladder irritation.\n\nIt is important for pediatric providers to understand that, at this point, there is no perfect \"test\" for ruling out appendicitis, thus a thorough history and physical examination remain critical for identifying the condition. When the diagnosis is highly suspected clinically, pediatric surgical consultation should be obtained promptly. If the diagnosis of acute appendicitis is equivocal, laboratory studies, including a complete blood cell count with differential, may be useful in supporting the diagnosis; however, laboratory studies cannot be relied upon to definitively confirm or rule out this condition. Although both abdominal ultrasonography and computed tomography (CT) of the right lower quadrant (RLQ) of the abdomen have been used as tools in the evaluation of children with suspected appendicitis, ultrasonography is emerging as the primary imaging modality in many centers. RLQ abdominal ultrasonography can be used to confirm or exclude the diagnosis of appendicitis in children in whom the appendix can be clearly identified. A major advantage of ultrasonography over CT is the avoidance of exposure to unnecessary ionizing radiation.\n\nDiabetic ketoacidosis (DKA) may cause abdominal pain and vomiting in children, along with significant dehydration; however, the presence of peritoneal signs in this patient is not consistent with this diagnosis. Furthermore, the absence of glucosuria on this patient's urinalysis makes a diagnosis of DKA extremely unlikely. In a child presenting for the first time with DKA, the review of systems would typically be positive for polyuria, increased urinary frequency, polydipsia, thirst, and weight loss. Fever is not typically associated with DKA, but affected patients may have fever because of a concurrent infectious process (which may have contributed to the development of DKA).\n\nChildren with lower lobe pneumonia may present with referred abdominal pain, along with fever and vomiting. The girl in the vignette, however, has had no cough or other respiratory symptoms (which would typically be seen in patients with pneumonia), and has no pertinent findings on her lung examination. Although pneumonia can cause referred abdominal pain, patients with this diagnosis would not be expected to have focal abdominal tenderness or clinical signs of peritonitis.\n\nMalrotation of the bowel with volvulus is an emergency that requires immediate surgical intervention to avoid significant morbidity (such as bowel ischemia and short bowel syndrome) and mortality. Patients with volvulus most commonly present during the first year after birth, though the condition can present at any age. Classic symptoms include severe abdominal pain (which can be a challenge to identify in infants), along with bilious emesis and signs of abdominal obstruction. The girl in this vignette has no history of bilious vomiting, and her presentation is not consistent with that of volvulus.\n\nMesenteric adenitis is a self-limited process that occurs because of inflammation of mesenteric lymph nodes in the abdominal RLQ, and can present similarly to acute appendicitis. Patients with mesenteric adenitis would not be expected to present with pain migration, peritoneal signs, or a toxic clinical appearance.\n\nPREP Pearls\n• Appendicitis results from obstruction of the appendix due to inflammation in the appendiceal wall or from a fecalith.\n• Classic abdominal pain associated with acute appendicitis develops gradually, beginning as a vague and poorly localized periumbilical pain that worsens in severity and localizes to the right lower abdominal quadrant as the inflammatory process progresses.\n• Abdominal ultrasonography is emerging as the primary imaging modality for evaluation of suspected appendicitis in many centers.\n\nABP Content Specifications(s)\n• Recognize the clinical features associated with appendicitis\n• Plan the appropriate diagnostic evaluation when appendicitis is suspected\n\nSuggested Readings\n• Bachur RG. Abdominal emergencies. In: Shaw KN, Bachur RG, eds. Fleisher and Ludwig's Textbook of Pediatric Emergency Medicine. 7th ed. Philadelphia, PA: Lippincott Williams & Wilkins; 2016;chap 124:1313–1333.\n• Hansen LW, Dolgin SE. Trends in the diagnosis and management of pediatric appendicitis. Pediatr Rev. 2016;37(2):52. doi: http://dx.doi.org/10.1542/pir.2015-0021.\n• Ross A, LeLeiko NS. Acute abdominal pain. Pediatr Rev. 2010;31(4):135. doi: http://dx.doi.org/10.1542/pir.31-4-135."}
{"id" : 1375, "question_text" : "A 2-year-old girl is brought to your office for a health supervision visit. The mother has brought her 4-year-old sibling, and the 2 children are playing in the corner of your examination room. You confirm that the patient has approximately 50 words, is using 2-word phrases, and half of her speech is understandable. As you are talking with her mother, you hear the 2-year-old girl cry \"No!\", followed by the 4-year-old sibling saying \"Ow! She bit me!\" Her mother cries \"Stop biting!\" and then turns to you to ask why her child bites. Of the following, the MOST likely reason for this child's behavior is", "options" : "[\"autism spectrum disorder\", \"emerging behavioral disorder\", \"exposure to violence at home\", \"inability to adequately verbalize her frustration\", \"speech/language delay\"]", "explanation" : "Biting another child or adult, as seen in this vignette, is very common in older infants and young children, and probably represents a young child's inability to express herself when experiencing frustration, such as having to share a toy when she does not want to.\n\nBiting occurs frequently in childcare settings, typically starts around 9 to 13 months of age, peaks at 22 to 24 months of age, and declines after 2.5 years of age as language and emotional regulation develop. Bites from young children rarely break the skin of the victim. Recommended caregiver response to biting includes showing empathy toward the bitten, a firm and timely negative statement (eg, \"No biting! Biting hurts!\"), positive encouragement when the child is behaving well when frustrated, and avoiding those frustrating situations where a child is likely to bite.\n\nIf it is part of a larger pattern of ongoing aggression toward others and rule-breaking that is disruptive and not responsive to techniques, biting beyond a developmental age of 3 years may signify a behavioral disorder. Self-biting can be a symptom of autism spectrum disorder or obsessive-compulsive disorder.\n\nThe child's mother reports language development typical for a 2-year-old child, so she does not have a speech delay. Speech-language and social-emotional developmental delays, as well as conduct disorder, have been shown to be associated with exposure to intimate partner violence, but there is no evidence suggesting an association with biting behaviors of normal childhood.\n\nPREP Pearls\n• Biting others is a normal part of childhood development.\n• Biting incidents decrease after 2.5 years of age.\n• Biting can be part of a constellation of ongoing aggressive behaviors in older children with conduct disorder.\n\nABP Content Specifications(s)\n• Recognize factors related to biting at various developmental stages\n\nSuggested Readings\n• Garrard J, Leland N, Smith DK. Epidemiology of human bites to children in a day-care center. Am J Dis Child. 1988;142(6):643-650.\n• Zahrt DM, Melzer-Lange MD. Aggressive behavior in children and adolescents. Pediatr Rev. 2013;32(8):325-332. doi: http://dx.doi.org/10.1542/pir.32-8-325."}
{"id" : 3794, "question_text" : "A 14-year-old girl is being evaluated for concern about evolving seizures. She has a history of partial complex seizure disorder, which has been well-controlled with medication. She recently had a prolonged episode of abnormal motor movements that included backward arching of her head, neck, and back, and pelvic thrusting. Her eyes were closed tightly during the episode, but she became alert quickly when the movements subsided. The adolescent also has an anxiety disorder. She has been diligent about taking her antiepileptic and antianxiety medications as prescribed. She has otherwise been well. She has a family history of epilepsy, hypertension, anxiety, and diabetes, and a cousin who recently died of meningitis. On physical examination, her vital signs are within normal limits. The remainder of her physical and neurologic examination findings are normal. Of the following, the test or study MOST likely to reveal this girl's diagnosis is", "options" : "[\"antiepileptic medication level\", \"brain magnetic resonance imaging\", \"cerebrospinal fluid studies\", \"video electroencephalography\"]", "explanation" : "Correct Answer: D\nThe girl in the vignette is demonstrating behaviors consistent with psychogenic nonepileptic seizure (PNES). Video electroencephalography (EEG) is the gold standard test for this diagnosis and will have normal findings at the time of the apparent seizure behavior. The girl is unlikely to have low blood levels of antiepileptic medication if she has been taking her medication as prescribed. Brain magnetic resonance imaging can be used to evaluate for a structural brain abnormality causing seizures, but is not diagnostic for PNES. She is unlikely to have meningitis given her normal vital signs and physical examination findings.\n\nIn conversion disorder (functional neurologic symptom disorder), neurologic symptoms (motor or sensory) are present, which are not consistent with recognized pathophysiology of medical or neurologic conditions. These symptoms cause distress or impairment in functioning and may include weakness, paralysis, posturing, abnormal movements or gait, tremors, seizures, altered speech, visual disturbance, or altered sensation. Conversion disorder can be seen in early childhood, but is unusual before 10 years of age. Girls are more likely to be affected than boys. Associated features include symptom onset at the time of a stressor, conflict, or trauma and dissociative symptoms (eg, depersonalization, derealization). While \"la belle indifference\" (lack of concern about a serious symptom) and concerns about secondary gain have been associated with conversion disorder, they are also seen in other conditions.\n\nThe differential diagnosis of conversion symptoms includes a neurologic or medical disorder, somatic symptom disorder, factitious disorder, malingering, dissociative disorder, body dysmorphic disorder, depressive disorder, and panic disorder. Some of these conditions, such as neurologic disorder (eg, epilepsy), panic disorder, and dissociative disorder may co-occur with conversion disorder. Patients with seizures are more likely to have PNES.\n\nA comprehensive history is essential to evaluate the concerning symptoms. Neurologic symptoms should be explored in detail, paying close attention to the circumstances at the onset of the symptoms, characteristics of the symptoms, and the presence of dissociative symptoms. In conversion disorder, symptoms often start suddenly and a feeling of being disconnected from oneself or the environment may be present. Forward pelvic thrusting, side-to-side head or body movements, closed eyes resistant to opening, and a lack of postictal confusion are more often seen in PNES. The child with anesthesia due to conversion disorder may describe a pattern of sensory loss that does not correspond to known sensory nerve pathways. Family history is likely to be positive for medical and psychiatric disorders in close relatives of children with conversion disorder. Any psychiatric conditions (eg, anxiety, depression) and neurologic disorders (eg, epilepsy) in the child should be identified. Psychosocial stressors such as school problems, family disruption, interpersonal conflicts, and traumatic events should be ascertained. A psychiatric evaluation can identify mental health conditions that coexist or underlie the patient's presentation.\n\nA complete physical and neurologic examination is required to determine if the findings are consistent and the symptoms are anatomically and physiologically feasible. The child with weakness caused by conversion disorder may have varying symptoms during the visit. Laboratory and radiologic studies may be indicated, based on the presenting symptoms. Video EEG can distinguish between PNES and epileptic seizures.\n\nPREP Pearls\n• In conversion disorder (functional neurologic symptom disorder), neurologic symptoms (motor or sensory) are present, which are not consistent with recognized pathophysiology of medical or neurologic conditions.\n• Forward pelvic thrusting, side-to-side head or body movements, closed eyes resistant to opening, and a lack of postictal confusion are findings suggestive of psychogenic nonepileptic seizures rather than epileptic seizures.\n• Neurologic disorder (eg, epilepsy), panic disorder, and dissociative disorder may co-occur with conversion disorder. Patients with epileptic seizures are more likely to have psychogenic nonepileptic seizures.\n\nABP Content Specifications(s)\n• Plan an appropriate evaluation of psychosomatic disorders\n• Recognize the various features associated with conversion disorders\n• Identify the various features associated with psychosomatic disorders\n• Formulate an appropriate differential diagnosis of conversion symptoms\n\nSuggested Readings\n• American Psychiatric Association. Diagnostic and Statistical Manual of Mental Disorders, Fifth Edition. Arlington, VA: American Psychiatric Association; 2013.\n• Patel H, Dunn DW et al. Psychogenic nonepileptic seizures (pseudoseizures). Pediatr Rev. 2011;32(6):e66-e72. doi:10.1542/pir.32-6-e66.\n• Prazar GE. Conversion reactions and hysteria. In: McInerny TK, Adam HM, Campbell DE, DeWitt TG, Foy JM, Kamat DM, eds. American Academy of Pediatrics Textbook of Pediatric Care. 2nd ed. Elk Grove Village, IL: American Academy of Pediatrics; 2017:1927-1933. Pediatric Care Online."}
{"id" : 2301, "question_text" : "A 10-year-old child with no significant medical history is seen in the emergency department for an acute onset of chest pain earlier in the day. Four days ago, she was diagnosed with influenza A following 3 days of fevers and cough. These symptoms have significantly decreased, and she has been afebrile for 48 hours. She denies dyspnea or palpitations. The chest pain is constant but worsens with coughing and deep breathing. She has a heart rate of 100 beats/min, respiratory rate of 20 breaths/min, and normal blood pressure for her age. She has no reproducible chest pain to palpation. The remainder of her physical examination findings are normal. Electrocardiography results are shown (Figure).", "options" : "[\"obtain a chest radiograph and start a course of oseltamivir\", \"obtain a troponin level and consider echocardiography\", \"prescribe a 7-day course of oral steroids and follow up in 2 days\", \"prescribe a 10-day course of a nonsteroidal anti-inflammatory drug and follow up in 2 days\"]", "explanation" : "Correct answer is B\n\nCritique\nThis patient's clinical presentation and electrocardiography (ECG) findings are most suggestive of acute pericarditis, likely secondary to influenza. The child's ECG demonstrates mild sinus tachycardia, diffuse ST segment elevation, and mild PR depression, which are all classic findings of pericarditis. However, as acute myocarditis can present with similar symptoms, it is important to rule out any significant myocardial component before making decisions regarding disposition and treatment. Affected children may have myocardial dysfunction without significant symptoms. Up to one-third of patients with pericarditis have a mild elevation in troponin level and are deemed to have myopericarditis. Perimyocarditis is diagnosed when the main abnormality is myocarditis, but there is pericardial involvement demonstrated by symptoms, pericardial effusion, or typical ECG changes.\n\nAlthough pericarditis is an inflammatory condition, empiric treatment with oral steroids is not the standard of care. Data in both pediatric and adult patients suggest a higher rate of recurrence of symptoms when steroids are used as primary treatment.\n\nChest radiography would be an appropriate diagnostic test for a child with pleuritic chest pain. However, this patient's clinical symptoms and ECG findings are more consistent with pericardial inflammation. While chest radiography can be helpful in identifying a large pericardial effusion, small- and moderate-sized pericardial effusions can be missed by chest radiography and cannot be ruled out in the presence of a normal cardiac silhouette or cardiothoracic ratio.\n\nIn general, oseltamivir is effective in decreasing symptoms from influenza A when started within 48 hours of symptom onset. However, the Red Book 2021-24 Report of the Committee on Infectious Diseases recommends treatment with oseltamivir for \"any hospitalized child with suspected or confirmed influenza disease, regardless of the duration of symptoms.\" Oseltamivir would have no impact on pericarditis symptoms or prognosis.\n\nNonsteroidal anti-inflammatory drugs (NSAIDs) are often used to treat pericarditis and may be an appropriate treatment option for this patient, but it is important to rule out myocardial involvement before beginning anti-inflammatory treatment. Pericarditis is typically viral or postviral in nature. Idiopathic pericarditis is also common, mostly likely due to the inability to isolate a specific viral pathogen with routine testing such as a respiratory viral panel. The differential diagnosis of pediatric pericarditis is broad and includes many infectious and noninfectious etiologies (Table).\n\nChildren with bacterial pericarditis are typically very ill. Fungal etiologies are uncommon in immunocompetent hosts. Primary and metastatic neoplastic etiologies of pericarditis are very rare in children. However, neoplasms in the anterior mediastinum may lead to secondary pericardial inflammation. Metabolic etiologies more commonly present with pericardial effusions, which may be minimally symptomatic unless they are large. Any child who has undergone cardiac surgery or cardiac catheterization is at risk for post-pericardiotomy syndrome, which typically presents with fever, chest pain, and fatigue from one to several weeks post-surgery/intervention. Pericarditis can be the presenting abnormality of a systemic inflammatory condition. Several autoinflammatory conditions that present in childhood can lead to serositis, including pericarditis. It is important to obtain a thorough noncardiac history to assess for signs and symptoms pointing to a systemic inflammatory disease.\n\nMost children diagnosed with pericarditis are hospitalized for further evaluation, management of pain, and treatment of inflammation. All children with an elevated troponin level should be hospitalized. Evaluation of pericarditis typically includes serial ECGs and baseline echocardiography to assess for a significant pericardial effusion or evidence of decreased cardiac function, which would suggest more significant myocardial inflammation. Treatment with NSAIDs for several weeks is the mainstay of therapy. The addition of a several-month course of colchicine is being used more commonly; recent evidence shows that it may decrease recurrence. The recurrence rate of pericarditis is approximately 10%, with a higher incidence among children whose pharmacologic treatment was stopped prematurely. Participation in competitive activity after an episode of pericarditis should be restricted for a minimum of 3 months. Longer duration restrictions may be recommended if there is concomitant myocarditis or recurrence of pericarditis-type symptoms.\n\nContent Domain\nCardiology\n\nABP Content Specification(s) / Content Area(s)\n- Evaluate and manage a patient with pericarditis\n- Recognize pathogens commonly associated with pericarditis"}
{"id" : 3656, "question_text" : "A 7-year-old boy is admitted to the hospital with fever and headache. He has congenital hydrocephalus and a ventriculoperitoneal shunt. He takes phenytoin at home to prevent seizures. He is receiving intravenous vancomycin every 6 hours for suspected shunt infection. On day 2 of admission, he has decreased urine output. His vital signs are stable, and he has no swelling over the face or ankles. He is alert and oriented without any neurological deficit. The rest of the physical examination findings are unremarkable. Laboratory data are shown: Laboratory Test Result Sodium 134 mEq/L (134 mmol/L) Potassium 4.8 mEq/L (4.8 mmol/L) Chloride 100 mEq/L (100 mmol/L) Bicarbonate 20 mEq/L (20 mmol/L) Blood urea nitrogen 25 mg/dL (8.9 mmol/L) Creatinine 2 mg/dL (177 µmol/L) Vancomycin, trough level 54 µg/mL (37 µmol/L). Of the following, the additional laboratory finding MOST likely to be seen in this patient is", "options" : "[\"fractional excretion of sodium more than 2%\", \"red blood cell casts on microscopic urinalysis\", \"specific gravity of urine more than 1.020\", \"urine to plasma osmolality ratio more than 1.5\"]", "explanation" : "Correct Answer: A\nThe child in this vignette has acute kidney injury (AKI) secondary to vancomycin toxicity. A high vancomycin level causes acute renal tubular injury leading to increased fractional excretion of sodium and an elevation in serum creatinine level.\n\nAcute kidney injury is defined as a decrease in glomerular filtration rate on the basis of change in urine output or an increase in serum creatinine. The causes of AKI can be divided into those related to decreased effective renal blood flow (prerenal AKI), intrinsic renal damage (renal AKI), and obstruction to the flow of urine (postrenal AKI). Prerenal AKI is a result of decreased effective circulating blood volume as occurs in gastroenteritis, hemorrhage, sepsis, heart failure, nephrotic syndrome, or liver disease. Renal AKI can be further divided into:\n• Glomerular injury as occurs in acute glomerulonephritis\n• Tubular injury as occurs in acute tubular necrosis (ATN) resulting from medications or toxins\n• Interstitial injury as occurs in acute interstitial nephritis\n• Vascular injury as occurs in hemolytic uremic syndrome\n\nPostrenal AKI results from bilateral upper/lower urinary tract obstruction or posterior urethral valves. Prerenal AKI is the most common reason for elevation in creatinine level. However, in hospitalized children, especially in tertiary centers, the cause of AKI is often multifactorial and can be due to heart disease, sepsis, or nephrotoxic medications.\n\nThe etiology of AKI is differentiated with appropriate laboratory tests. A urinalysis with microscopic examination and urinary indices is a useful noninvasive test in a child with AKI. A normal urinalysis with no blood, protein, or cells with absence of casts is mostly seen in prerenal AKI. A urinalysis showing granular or epithelial cell casts is suggestive of ATN, whereas red blood cell casts indicate acute glomerulonephritis. In prerenal AKI, urine specific gravity is greater than 1.020, urine sodium level is less than 10 mEq/L, fractional excretion of sodium (FENa) is less than 1%, and urine to plasma osmolality is greater than 1.5. These urinary indices reflect the renal tubular reabsorption of filtered sodium as well as water in response to decreased renal perfusion. In renal AKI, the urine is dilute with specific gravity below 1.010, urine sodium level is greater than 40 mEq/L, FENa is greater than 2%, and urine to plasma osmolality is less than 1.5. The severity of AKI and renal hypoperfusion is also reflected by serum blood urea nitrogen (BUN) to creatinine ratio. A BUN to creatinine ratio of 20:1 is seen in prerenal AKI, and a ratio of 10:1 to 15:1 is seen in renal AKI. A schematic approach to a child with AKI is shown in Item C101.\n\nThe child in this vignette has vancomycin-related ATN. A high FENa is seen as a result of tubular injury and suggests a renal cause of AKI. The BUN to creatinine ratio of 12.5:1 in this vignette also favors renal AKI. A urine specific gravity of greater than 1.020 and urine to plasma osmolality greater than 1.5 are seen in prerenal AKI. Red blood cell casts are characteristically seen in acute glomerulonephritis. However, a high vancomycin trough level of 54 µg/mL (therapeutic level, 10-20 µg/mL) is suggestive of a tubular cause rather than a glomerular cause of AKI.\n\nPREP Pearls\n• Urinalysis and urinary indices are useful noninvasive tests to differentiate prerenal from renal acute kidney injury.\n• A urine specific gravity less than 1.010 and a fractional excretion of sodium of more than 2% is suggestive of a renal tubular injury.\n• Nephrotoxic medications are a common cause of acute kidney injury in hospitalized children.\n\nABP Content Specifications(s)\n• Plan the appropriate diagnostic evaluation of oliguria\n• Plan the appropriate initial management of acute renal failure, while considering the effects of various therapies on associated physiologic abnormalities\n\nSuggested Readings\n• Jain A, Mattoo TK. Oliguria and anuria. In: McInerny TK, Adam HM, Campbell DE, DeWitt TG, Foy JM, Kamat DM, eds. American Academy of Pediatrics Textbook of Pediatric Care. 2nd ed. Elk Grove Village, IL: American Academy of Pediatrics; 2017:2423-2427. Pediatric Care Online.\n• Selewski DT, Symons JM. Acute kidney injury. Pediatr Rev.2014;35(1):30-41. doi:10.1542/pir.35-1-30."}
{"id" : 2343, "question_text" : "An 8-year-old girl is admitted to the hospital with fever and dehydration. She appears ill but is responsive. Her temperature is 37 °C, her blood pressure is 98/62 mm Hg, heart rate is 130 beats/min, and respiratory rate is 20 breaths/min. Her capillary refill time is 5 seconds. The remainder of her examination findings are unremarkable. Laboratory results are shown. [Laboratory values provided]. A normal saline bolus and dextrose are ordered. The plan to place an intravenous line to administer fluid and medications is discussed with the family. The child's mother expresses understanding of and agreement with the plan. The child begins to cry and refuses treatment. Of the following, the BEST next step in this child's care is to", "options" : "[\"attempt oral therapy owing to lack of assent\", \"consult child life specialists for help in obtaining assent\", \"continue with therapy despite lack of assent\", \"seek consent from both of the child's parents\"]", "explanation" : "PREP Pearl(s)\nAdolescents' decisions regarding their sexual health (treatment of sexually transmitted illnesses, contraceptive care, and prenatal care) should be honored. Laws regarding confidentiality of this care are determined at the state level.\nConsent and assent are guided by the ethical principles of autonomy, justice, and beneficence.\nWhen a parent or guardian denies consent for medically necessary treatment, input from a team that may include members of an ethics committee, psychiatrist, chaplain, psychologist, and/or palliative care specialist should be sought before seeking legal intervention.\nCritique\nThe child in the vignette has significant dehydration, electrolyte derangements, and hypoglycemia. Not treating her would be harmful, and her mother has given consent for treatment. Oral rehydration is not sufficient given the severity of her illness. Therefore, the best next management step is to continue with intravenous therapy despite the child's lack of assent. If there were no potential harm in delaying treatment, it would be appropriate to postpone the procedure and elicit help (eg, from child life specialists) to obtain assent from the child. There is no requirement to obtain consent from both parents when there is lack of assent from a child.\nThe parent or guardian of a minor child has the right to give legal consent in most situations. If the parent is also a minor, they are able to give legal consent for their child unless there is a different predetermined legal arrangement. In all cases, if withholding emergency interventions could harm the minor child, it is not necessary to await consent (per the Emergency Medical Treatment and Active Labor Act of 1986).\nConsent and assent are guided by the ethical principles of autonomy, justice, and beneficence. Informed consent includes 3 core elements:\nDisclosure of key information\nNatural progression of the disease process\nLikelihood the test/procedure will be successful\nPotential risks and benefits\nAssessment of the patient and/or caregiver's understanding of the test/procedure\nAssessment of the patient and/or caregiver's ability to give consent\nA child gives assent when they express agreement to a procedure or testing. Children are able to give assent as early as age 7 years, when concrete thinking develops. Information, including an explanation of the testing or treatment, should be provided to the child in a developmentally appropriate manner, and the child's understanding of the procedure or treatment should be assessed. A child's wish to forgo or delay treatment or testing should not be honored if this choice would cause them harm, as in the case of the child in the vignette. However, in such a case, acknowledgment of the child's feelings and an apology can show the child respect for their wishes. For example, if a child requires a vaccine and is saying \"no,\" it is reasonable to explain, \"I know it hurts. We are doing this to help keep you healthy. I am sorry it's causing you pain.\"\nAdolescents are considered \"mature minors\" regarding decisions about their sexual health (sexually transmitted illness treatment, contraceptive care, prenatal care), mental health, and substance use; parental consent is generally not required. Levels of confidentiality of care for these issues is determined by state law. An important goal of mature minor designation is to facilitate adolescents' ability to seek care and receive proper treatment in situations when asking their parents for consent may be a barrier.\nEmancipated minor adolescents have the legal right to consent to all medical care without parental permission or notification. Although state laws vary, criteria for emancipation typically include a minor who is one of the following:\nLiving in a separate household from their parents/guardians and supporting themselves, OR\nMarried, OR\nAn active-duty armed forces service member\nWhen a parent or guardian denies consent for medically necessary treatment, consultation should be sought from a designated team that may include members of an ethics committee, psychiatrist, chaplain, psychologist, and/or palliative care specialist before seeking legal intervention. When agreement on a safe plan cannot be achieved, the state can mandate treatment. In these cases, the burden to the child should be low and the likelihood of a positive outcome high. If there is uncertainty in the outcome, the overall prognosis is poor, or the burden to the child is high, it may be reasonable for the parent or guardian to refuse treatment.\nSuggested Reading(s)\nKatz AL, Webb SA: American Academy of Pediatrics Committee on Bioethics; et al. Informed consent in decision-making in pediatric practice. Pediatrics. 2016;138(2):e20161485. doi:10.1542/peds.2016-1485\nLadd RE, Forman EN. Ethics for the pediatrician: pediatrician/patient/parent relationships. Pediatr Rev. 2010;31(9):e65-e67. doi:10.1542/pir.31-9-e65\nMaslyanskaya S, Alderman EM. Confidentiality and consent in the care of the adolescent patient. Pediatr Rev. 2019;40(10):508–516. doi:10.1542/pir.2018-0040\nWasserman JA, Navin MC, Vercler CJ. Pediatric assent and treating children over objection. Pediatrics. 2019;144(5):e20190382. doi:10.1542/peds.2019-0382\nContent Domain\nEthics\nABP Content Specification(s) / Content Area(s)\nRecognize and apply ethical principles involved in the patient-parent-pediatrician relationship regarding issues of informed consent/dissent/assent\nUnderstand the difference between informed consent and assent\nThe correct answer is: continue with therapy despite lack of assent"}
{"id" : 2686, "question_text" : "An 8-month-old boy is seen in the emergency department after falling from his crib. A cephalohematoma is noted on physical examination. His vital signs are appropriate for age. The remainder of his physical examination findings are normal. His medical history includes a 2-week neonatal intensive care unit admission for prolonged bleeding after circumcision. Laboratory data are shown: Laboratory Test Result, White blood cell count 9,800/µL (9.8 × 109/L), Hemoglobin 8.5 g/dL (85 g/L), Platelet count 90 × 103/µL (90 × 109/L), Mean corpuscular volume 85 fL, Reticulocyte 2.5%, vWF activity 34% (reference range, 54%-152%), vWF antigen 88% (reference range, 50%-150%), Factor VIII assay 95% (reference range, 50%-200%), vWF multimer assay Abnormal. Of the following, the BEST next step in management of this infant is", "options" : "[\"blood transfusion\", \"desmopressin therapy\", \"factor complex injection\", \"platelet transfusion\"]", "explanation" : "The boy in the vignette most likely has von Willebrand disease (vWD); this diagnosis is supported by his laboratory findings of an abnormal von Willebrand panel. Type 2B is associated with thrombocytopenia, including neonatal thrombocytopenia, and is the most likely diagnosis for the child in the vignette.\n\nVon Willebrand disease is an inherited bleeding disorder with qualitative or quantitative defects in von Willebrand factor (vWF). Von Willebrand factor is a plasma protein that functions as a carrier for factor VIII and helps bind platelets to damaged endothelium. Bleeding symptoms of vWD include mucosal bleeding, epistaxis, and menorrhagia. Administration of antihemophilic factor/vWF complex or another factor VIII/vWF concentrate is the treatment of choice for type 2B vWD. Acquired vWD, a decrease in vWAg in an individual without any bleeding history, may occur in some illnesses (eg, autoimmune, some cancers) or as a side effect of medications (eg, ciprofloxacin, valproic acid).\n\nDesmopressin causes an increase in factor VIII activity, von Willebrand antigen, and ristocetin cofactor activity. Desmopressin is the most common treatment for individuals with type 1 vWD, although not every affected individual has a response. Treatment with desmopressin may cause hyponatremia and therefore requires fluid restriction when used. The infant in the vignette does not have type 1 vWD and would therefore not benefit from this therapy. In addition, in individuals with type 2B vWD, desmopressin can lead to a transient decrease in platelets, which may worsen bleeding.\n\nA blood transfusion is not indicated for the infant in the vignette. Although he is anemic, there is an appropriate reticulocyte response to the anemia, and he is hemodynamically stable. Platelet transfusions in type 2B vWD may lead to increased binding between the platelets and the high-molecular-weight von Willebrand factor multimers. This binding leads to an increase in their clearance from circulation. Therefore, platelet transfusion is not recommended.\n\nPREP Pearls\n• Von Willebrand factor is a plasma protein that functions as a carrier for factor VIII and helps bind platelets to damaged endothelium.\n• Von Willebrand disease type 2B may be associated with thrombocytopenia\n• Desmopressin is not an appropriate treatment for type 2B von Willebrand disease and may worsen thrombocytopenia.\n\nABP Content Specifications(s)\n• Recognize the clinical findings associated with von Willebrand disease\n• Plan the appropriate management of von Willebrand disease\n\nSuggested Readings\n• Cooper S, Takemoto C. Von Willebrand disease. Pediatr Rev. 2014;35(3):136-137. doi:10.1542/pir.35-3-136.\n• Lanzkowsky P, Lipton J, Fish JD. Lanzkowsky's Manual of Pediatric Hematology and Oncology. 6th ed. Elsevier Inc; 2016:121-125.\n• Lichtman MA, Williams WJ, Kipps TJ, et al. Williams Manual of Hematology. 8th ed. McGraw Hill; 2010:608-613."}
{"id" : 3583, "question_text" : "A 5-year-old boy is brought to the urgent care center with a 5-day history of fever and right ear pain, with subsequent development of right facial swelling. He has no history of rhinorrhea, cough, or otorrhea. He has odynophagia resulting in decreased oral intake, but he is drinking juice well. Physical examination reveals the following: temperature of 39.1°C; tender, soft tissue swelling of the right maxilla; boggy swelling in the right preauricular area without tenderness on manipulation of right helix; clear external auditory canals and tympanic membranes; a 3-mm erythematous papule on the upper right gingiva with otherwise clear oropharynx; and a 2×2–cm tender right submandibular lymph node. Of the following, the MOST likely cause of the boy's ear pain is", "options" : "[\"dental abscess\", \"otitis media\", \"pharyngitis\", \"sinusitis\"]", "explanation" : "The boy in the vignette has referred right ear pain arising from a right upper molar dental abscess. Signs and symptoms supporting the diagnosis of dental abscess in this vignette include fever, right upper maxillary edema, the collection of purulent fluid on his right upper gingiva, right submandibular lymphadenopathy, and lack of upper respiratory symptoms or physical findings on otologic and pharyngeal examination. With this clinical picture, otitis media, pharyngitis, and sinusitis are much less likely.\n\nOtalgia has intrinsic (otogenic) (Item C27) and extrinsic (nonotogenic) causes of referred pain.\n\nNonotogenic causes of otalgia include referred pain caused by painful stimuli from one organ or structure that is misinterpreted by the sensory cortex as arising from another anatomic site because of sensory innervation from the same nerve. The boy in the vignette reports right otalgia in the setting of a dental abscess. The auriculotemporal branch of the mandibular division of the trigeminal nerve supplies sensory innervation to parts of the auricle, external auditory canal, and tympanic membrane, as well as the cranial cavity, scalp, orbit, face, nose, nasal cavity, nasal sinuses, mouth, teeth, temporomandibular joints, and parotid glands. Therefore, referred ear pain may develop due to pain arising from any of these structures. Dental abnormalities are the most common cause of referred otalgia; these may include dental caries, teething, impacted teeth, and abscesses.\n\nThe facial nerve has sensory fibers that innervate portions of the tympanic membrane, external auditory canal, posterior lobule, concha, antihelix and the overlying skin of the mastoid. Idiopathic facial paralysis (Bell palsy) typically causes referred pain in the mastoid process area. Herpes zoster oticus (Ramsay Hunt syndrome), a viral neuritis of the facial nerve, results in facial paralysis and severe otalgia with vesicular eruption on the external auditory canal and auricle.\n\nThe glossopharyngeal nerve innervates mucosal sensation of portions of the external auditory canal, the middle ear, the mastoid air cells, and the eustachian tube, in addition to the pharynx, tonsils, and posterior one-third of the tongue. As a result, conditions such as tonsillitis, recovery from a tonsillectomy, pharyngitis, peritonsillar abscess, and retropharyngeal abscess may give rise to referred otalgia.\n\nThe vagal nerve auricular branch supplies parts of the external auditory canal, tympanic membrane, and cavum conchalis in addition to the larynx, hypopharynx, trachea, thyroid gland, esophagus, lungs, and abdominal viscera. Conditions such as laryngitis, esophagitis, subacute thyroiditis, and gastroesophageal reflux may result in referred ear pain.\n\nThe cranial nerves also innervate the ear, including the greater auricular nerve and the lesser occipital nerve, which supply portions of the auricle and postauricular area along with the posterior scalp, skin, and posterior muscles of the neck and cervical spine. Conditions such as scalp infections, cervical lymphadenitis, subluxation of the atlantoaxial joint, cervical spine arthritis, cervical tumors, or cervical spine injuries may cause referred otalgia.\n\nPREP Pearls\n• Dental abnormalities are the most common cause of referred otalgia, which may include dental caries, teething, impacted teeth, and abscesses.\n• The most common otogenic etiologies of otalgia include otitis media, otitis externa, impacted cerumen, eustachian tube dysfunction, and foreign bodies in the ear canal.\n\nABP Content Specifications(s)\n• Understand the possible etiologies of ear pain, including referred pain from other anatomic sites\n\nSuggested Readings\n• Charlett SD, Coatesworth AP. Referred otalgia: a structured approach to diagnosis and treatment. Int J Clin Prac. 2007;61(6):1015-21. doi:10.1111/j.1742-1241.2006.00932.x.\n• Earwood JS, Rogers TS, Rathjen NA. Ear pain: diagnosing common and uncommon causes. Am Fam Physician. 2018:97(1):20-27. https://www.aafp.org/afp/2018/0101/p20.html.\n• Leung AK, Fong JH, Leong AG. Otalgia in children. J Natl Med Assoc. 2000;92(5):254-260. PMCID: PMC2640572."}
{"id" : 2830, "question_text" : "A 12-year-old boy with myotonic dystrophy requires nocturnal ventilator support via tracheostomy. He takes oral nutrition supplemented with feedings via gastrostomy tube. He has had intermittent episodes of fever associated with increased tracheal secretions. A modified barium swallow study demonstrates oropharyngeal dysphagia; the vocal cords are penetrated by thin liquids but not by semisolids.\n\nOf the following, the MOST appropriate next management step for this boy is", "options" : "[\"limitation of oral intake to solids and semisolids\", \"pharmacologic manipulation of oral secretions\", \"placement of a cuffed tracheostomy tube\", \"use of a Passey-Muir speaking valve\"]", "explanation" : "Correct Answer: A\nThe boy in the vignette is at risk for aspiration of thin liquids, as demonstrated by the modified barium swallow, though he does not appear to aspirate semisolids. He should have an oral diet consisting of those densities that he can safely swallow, with supplemental liquids given by gastrostomy tube. Thickening of his secretions pharmacologically will not decrease his risk of aspiration with exogenous liquids, nor will the use of a cuffed tracheostomy tube or a Passey-Muir speaking valve.\n\nAlthough some studies suggest that oral feedings are possible in children with tracheostomies and dysphagia, many studies show that just the presence of a tracheostomy promotes dysphagia and potential aspiration. Even oral secretions may be aspirated by children with a tracheostomy, both with or without a cuffed tube. Cuffed tracheostomy tubes are helpful in limiting the driving pressure and volume needed to deliver positive pressure ventilation without a large air leak, but they do not prevent aspiration. Secretions and oral liquids may pool in the valleculae and be aspirated into the lower airway. Barium and methylene blue dye studies have shown that 30% to 50% of patients with tracheostomies aspirate oral liquids, and 50% to 75% of these aspirations are silent, with no cough or attempt to clear the aspirate.\n\nPREP Pearls\n• Most patients with a tracheostomy experience at least minimal aspiration of liquids.\n• Children with a tracheostomy may safely swallow feedings of some consistencies; a modified swallow study should be performed to assess swallowing ability.\n• A cuffed tracheostomy tube does not prevent aspiration.\n\nABP Content Specifications(s)\n• Understand the effect of a tracheostomy on aspiration\n\nSuggested Readings\n• Lepainteur M, Ogne A, Claire B, et al. Risk factors for respiratory tract bacterial colonization in adults with neuromuscular or neurologic disorders and chronic tracheostomy. Respir Med. 2019;152:32-36.\n• Sanders CD, Guimbellot J, Muhlebach, MS, Lin F-C, Gilligan P, Esther CR Jr. Tracheostomy in children: epidemiology and clinical outcomes. Pediatr Pulmonol. 2018;53:1269-1275. doi: 10.1002/ppul.24071.\n• Streppel M, Veder LL, Pullens B, Joosten KFM. Swallowing problems in children with tracheostomy tubes. Int J Pediatr Otorhinolaryngol. 2019;124:30-33. doi: 10.1016/j.ijporl.2019.05.003."}
{"id" : 139, "question_text" : "A 15-year-old girl presents to your clinic for evaluation of primary amenorrhea. She has a history of mild seasonal allergies and a right radius fracture at age 9. Her mother's height is 5 ft 9 in and father's height is 6 ft 2 in. The mother reports that her menarche occurred at 11 years, and the father recalls shaving at 14 years. On physical examination, the girl has Sexual Maturity Rating (SMR) 3 pubic hair and SMR 1 breast development. Bone age radiography shows a skeletal maturity of 13 years. No other abnormalities are noted. Her growth curve is shown.\n\nOf the following, the MOST appropriate next step in this girl's evaluation and treatment is to", "options" : "[\"initiate oral conjugated estrogen therapy\", \"measure serum estradiol\", \"measure serum thyroid-stimulating hormone\", \"obtain a karyotype\", \"perform a bimanual examination\"]", "explanation" : "The differential diagnosis of primary amenorrhea includes constitutional delay, eating disorders, chronic disease (typically causing low weight for height), severe androgen resistance, Rokitansky syndrome (absence of uterus), imperforate hymen, gonadotropin deficiency, and primary ovarian failure. Based on the short stature, growth pattern, and absence of breast development in the presence of pubic hair described for the girl in the vignette, gonadal failure due to Turner syndrome should be considered and a karyotype obtained. Indeed, the most common cause of primary ovarian failure in otherwise healthy girls is Turner syndrome. Initiating estrogen therapy in a patient without establishing a clear diagnosis is unwise. Measurement of serum estradiol is unnecessary because the finding of breast Sexual Maturity Rating 1 indicates absence of pubertal estradiol concentrations. Although thyroid disease can cause menstrual irregularities, other symptoms and signs would be expected, making the measurement of thyroid-stimulating hormone unnecessary. A bimanual examination is not required in the first stages of an amenorrhea evaluation when Turner syndrome is suspected and could be distressing for this girl.\n\nClassic Turner syndrome occurs in girls who have a complete absence of one X chromosome (karyotype 45XO). However, a large percentage of affected girls have mosaic presentations, meaning they have a mix of 45XO and 46XX chromosomes when multiple cells are examined. Such girls often have less of the characteristic stigmata of Turner syndrome (eg, shield chest, webbed neck, low posterior hair line, short stature). Girls who have mosaic Turner syndrome are more likely to present with primary amenorrhea if they are not diagnosed on the basis of the more classic physical features of the condition.\n\nOf note for the girl in the vignette, her parents are both tall, providing a mid-parental height of 5 ft 9 in, which is approximately the 95th percentile for adult women, but the patient is growing at only the 5th percentile on a standard growth curve. It is important to consider parental height even when children maintain normal growth velocity. The 95th percentile for height on a Turner syndrome growth curve nearly overlaps with the 5th percentile for height in unaffected girls. Accordingly, girls who have Turner syndrome rarely reach the 5th percentile without intervention such as growth hormone or oxandrolone therapy. Growth hormone therapy is approved for short stature in girls who have Turner syndrome, even though they do not have growth hormone deficiency. In this case, the girls' above-average mid-parental height contributed to her ability to reach a height within the reference range for unaffected girls.\n\nAmerican Board of Pediatrics Content Specification(s):\n\nKnow that gonadal dysgenesis is uniformly present in Turner syndrome"}
{"id" : 1283, "question_text" : "An 8-year-old girl has been in the intensive care unit for 2 weeks after suffering a devastating neurologic injury from a ruptured arteriovenous malformation. Despite several aggressive therapies, her neurologic status has worsened. She currently has reactive pupils and breathes over the ventilator, but has no purposeful movements, response to voice or stimuli, and no cough or gag reflex. She has developed anuric renal failure, but does not yet meet criteria for emergent dialysis. You anticipate life-threatening fluid overload, hyperkalemia, and acidosis to develop within 2 or 3 days. Oxygen saturation is 100% on FiO2 of 0.4 on the ventilator. The child's parents wish to continue aggressive therapies, including dialysis, even though the brain injury is devastating and refractory to all treatments. You have brought up withdrawal of support for the first time, but the parents believe the child would have wanted to remain alive as long as possible. Critical care medicine, nephrology, neurosurgery, neurology, palliative care medicine, and religious services have all been involved in her care. Of the following, the BEST next step is to", "options" : "[\"conduct multidisciplinary family meeting\", \"consult hospital ethics committee\", \"obtain cerebral blood flow scan\", \"place a dialysis catheter\", \"remove the endotracheal tube\"]", "explanation" : "The child in this vignette has suffered an irreversible neurologic injury and withdrawal of life-sustaining medical therapies (LSMT) is reasonable. Furthermore, she would likely require dialysis, an invasive therapy, to keep her alive past the next few days. Since the family is not interested in withdrawal of support at the moment and there are several subspecialists involved, the best option is to conduct a multidisciplinary family meeting.\n\nThe American Academy of Pediatrics (AAP) released a policy statement in 1994 that was last reaffirmed in 2012 which discussed limitation of LSMT. Physicians must provide families with relevant risks and benefits of available options and to provide specific recommendations, as opposed to offering a \"menu\" of choices. For this girl, there are few benefits to continuing LSMT because of the particularly poor neurologic prognosis. Quality of life is a concept fraught with value judgment. However, society generally views those who lack the most basic cognitive functions and the capability of perceiving their surroundings to be in a persistent vegetative state and have a low quality of life. The medical team should give families adequate time to consider these risks and benefits. At the time point described in the vignette, the family would like to prolong life as long as possible. However, this is the first time withdrawal of LSMT has been introduced. For that reason, a multidisciplinary approach outlining the status and needs of the child and the family may effectively inform the medical decision makers. Physicians are not obligated to provide any treatment thought to be unlikely to benefit the patient. Even though decisions to actively withdraw LSMT should be made with the family's agreement, physicians cannot be forced to provide futile care. Children should generally be allowed to participate in their own medical decision-making when possible, and mature and emancipated minors may be able to make their own decisions. Even though the family in this vignette believes the child would have wanted to live as long as possible, she had not likely reached the cognitive status to have made that determination in an informed manner. Lastly, decisions for children who have not reached that capacity should be made based on the best interest standard, which provides that decisions should be based on the relative risks and benefits of the treatment to the child. Benefits to children can include prolongation of life beyond simple biological existence without consciousness, improved quality of life, increased physical pleasure, increased emotional enjoyment, and increased intellectual satisfaction.\n\nAlthough ethics committees can be helpful in informing hospital policies and to give guidance in unusual circumstances, the scenario in the vignette has not yet reached that point. The medical team has introduced the idea of withdrawal of LSMT for the first time. Obtaining a cerebral blood flow scan can be helpful in the diagnosis of brain death if the clinical examination is equivocal, but the child does not meet brain death criteria because breathing over the ventilator requires brainstem activity. To proceed with invasive therapies in a patient who has a poor chance of any meaningful neurologic outcome is futile care. Placing a dialysis catheter with the intention of performing dialysis may prolonging life, but it is an invasive therapy that would lead to futile care. Although removal of the endotracheal tube and withdrawal of LSMT is reasonable, the medical team should provide adequate time for the family to make informed decisions. At the moment of the vignette, the family wishes to continue aggressive therapies. Providing them more information regarding the risks and benefits of the options and adequate time to consider the options would most likely lead to decisions made in the best interest of the child and family.\n\nFor this girl who has suffered a devastating, irreversible neurologic injury whose parents are not willing to withdraw LSMT the first time the option is introduced, a multidisciplinary meeting may best inform the family and provide more time for them to make a decision in her best interest.\n\nPREP Pearls\n• Physicians are not obligated to provide futile care, which includes prolonged or invasive therapies on patients who are not likely to derive benefit from them.\n• Withdrawal of life-sustaining medical therapies should occur with agreement from the medical decision makers, after they have been allowed adequate time to make a fully informed decision.\n\nABP Content Specifications(s)\n• Recognize and apply ethical principles when caring for a patient who is in a persistent vegetative state\n• Recognize and apply ethical principles regarding the issue of medical futility\n• Recognize and apply ethical principles involving palliative care and pain management\n• Recognize and apply ethical decision-making when caring for critically ill patients\n\nSuggested Readings\n• Burns JP, Mitchell C, Outwater KM, et al. End-of-life care in the pediatric intensive care unit after the forgoing of life-sustaining treatment. Crit Care Med. 2000;28(8):3060-3066.\n• Committee on Bioethics. Guidelines on forgoing life-sustaining medical treatment. Pediatrics. 1994;93(3):532-536. http://pediatrics.aappublications.org/content/93/3/532.\n• Mercurio MR, Maxwell MA, Mears BJ, Ross LF, Silber TJ. American Academy of Pediatrics Policy Statements on Bioethics. Pediatr Rev. 2008;29(3):e15-e22. doi: http://dx.doi.org/10.1542/pir.29-3-e15.\n• Zawistowski CA, DeVita MA. A descriptive study of children dying in the pediatric intensive care unit after withdrawal of life-sustaining treatment. Pediatr Crit Care Med. 2004;5(3):216-223. doi: http://dx.doi.org/10.1097/01.PCC.0000123547.28099.44."}
{"id" : 3633, "question_text" : "A 12-year-old girl and her 15-year-old brother are seen for evaluation after several days of fever, headache, malaise, and bilateral swelling of the cheek and jaw area. The siblings are unvaccinated. The girl now has neck stiffness and demonstrates meningeal signs without other neurologic findings. Her brother reports testicular pain. He has unilateral testicular swelling and tenderness. Of the following, the BEST management for both siblings' current concerns is", "options" : "[\"antibiotics\", \"antiviral therapy\", \"immune globulin\", \"supportive care\"]", "explanation" : "The siblings in this vignette have clinical features consistent with mumps infection and its associated complications. Therefore, the best management for both siblings is supportive care. Antibiotics are not indicated because there is no evidence of a bacterial infection. Antiviral therapy specific for mumps is not available. Immune globulin therapy is not effective in the management of mumps.\n\nMumps rubulavirus is an RNA virus that exclusively infects humans and is transmitted by respiratory tract secretions. The incubation period is typically 16 to 18 days but may be 12 to 25 days. Most patients experience a prodrome of fever, headache, and malaise lasting 1 to 2 days before parotitis occurs. Parotitis usually worsens over 3 days and resolves after 7 to 10 days. Bilateral parotitis occurs in 70% of patients. Parotitis may be accompanied by ipsilateral otalgia. Although parotitis is the most classic symptom of mumps infection, one-third of infected patients may not experience parotitis or may only have symptoms of a nonspecific upper respiratory tract infection. Patients infected with mumps are contagious for up to a week prior to the onset of parotitis and should be isolated for 5 days after the onset of parotitis, although viral shedding may be detected up to 2 weeks after the onset.\n\nComplications from mumps infection can affect multiple organ systems, are more likely to occur in infected adults than in children, and are usually short lived and self-resolving. Orchitis mostly affects postpubertal males aged 15 to 29 years, may be unilateral or bilateral, and may last for several weeks. Sterility is a rare complication. Viral meningitis affects less than 10% of individuals infected with mumps and usually manifests 5 days after the onset of parotitis and resolves after 3 to 10 days. Less common complications include glomerulonephritis, pancreatitis, thyroiditis, and hearing loss. Birth defects resulting from maternal infection with mumps have not been reported.\n\nMumps is a vaccine-preventable disease with a 2-dose regimen that is 88% effective. In the United States, outbreaks often occur in vaccinated populations that reside in close quarters. In the event of an outbreak of mumps infection, local public health officials should be notified, although it is not mandatory. Vaccination after exposure to mumps virus is not effective in preventing or ameliorating mumps infection but should still be administered to protect against future exposures. After exposure, unvaccinated children 12 months of age and older should be given 1 dose, and children who have already received 1 dose of the vaccine at least 28 days prior should receive a second dose. High-risk individuals who have already received the recommended 2 doses of mumps vaccine should receive a third dose. Infants 6 to 11 months of age may also be vaccinated, although this dose would not count towards the routine vaccination schedule. Immune globulin is not effective for postexposure prophylaxis.\n\nThe differential diagnosis of mumps infection includes other viruses that can cause parotitis, including Epstein-Barr virus, influenza A virus, cytomegalovirus, parainfluenza virus, and HIV. Bacterial infection from Staphylococcus aureus may also cause parotitis, but it is usually unilateral. Sjögren syndrome, lupus, and obstruction of the Stensen duct are noninfectious etiologies of parotitis.\n\nPREP Pearls\n• Supportive care is the best management strategy for children infected with mumps, including children experiencing complications from mumps infection.\n• Complications from mumps infection, such as orchitis and meningitis, rarely result in long-term or permanent morbidity.\n• Response to outbreaks should be managed in conjunction with local public health officials and include vaccination but not administration of immune globulin.\n\nABP Content Specifications(s)\n• Recognize the clinical features and complications associated with mumps\n• Plan the appropriate management of a patient with mumps\n\nSuggested Readings\n• American Academy of Pediatrics. Mumps. In: Kimberlin DW, Brady MT, Jackson MA, Long SS, eds. Red Book: 2018 Report of the Committee on Infectious Diseases. 31st ed. Itasca, IL: American Academy of Pediatrics; 2018:567-573. https://redbook.solutions.aap.org/chapter.aspx?sectionId=189640136&bookId=2205&resultClick=1.\n• Centers for Disease Control and Prevention. Mumps. In: Hamborsky J, Kroger A, Wolfe S, eds. Epidemiology and Prevention of Vaccine-Preventable Diseases. 13th ed. Washington DC: Public Health Foundation; 2015:247-260.\n• Mason WH, Gans HA. Mumps. In: Kliegman RM, St Geme JW III, Blum NJ, Shah SS, Tasker RC, Wilson KM, eds. Nelson Textbook of Pediatrics. 21st ed. Philadelphia, PA: Elsevier; 2020:1680-1683.\n• Murray DL, Vodzak J, Cheng Immergluck L. Contagious exanthematous diseases. In: McInerny TK, Adam HM, Campbell DE, DeWitt TG, Foy JM, Kamat DM, eds. American Academy of Pediatrics Textbook of Pediatric Care. 2nd ed. Elk Grove Village, IL: American Academy of Pediatrics; 2017:1920-1927. Pediatric Care Online."}
{"id" : 2297, "question_text" : "A 14-year-old with no previous medical problems is seen by his primary care provider for evaluation of difficulty focusing. His grades have declined over the past 6 months. He has no history of attention-deficit/hyperactivity disorder (ADHD). He recently got into trouble at school for having a vape device. The adolescent privately admits to daily use of marijuana and nicotine (vaped) for the past year. The boy's mother smokes marijuana daily to help with stress relief and for perceived health benefits. She also uses nicotine products daily and takes a stimulant for ADHD. She believes that her son is old enough to make his own health decisions and is not worried about his smoking, because she also smoked at his age. Education is provided on cognitive development and the effect of substance use during adolescence on the risk of future substance use disorders and addiction. Readiness for change is assessed for both the boy and his mother. Of the following, the BEST next step in this boy's care is to", "options" : "[\"perform a urine drug screening test\", \"prescribe a stimulant medication\", \"schedule a follow-up visit in 6 months\", \"screen for comorbid psychological disorders\"]", "explanation" : "Correct answer is D\n\nCritique\nThe boy in the vignette reports difficulty focusing and daily use of marijuana and nicotine. The best next step in his care is to screen for comorbid psychological disorders that may be contributing to his substance use. Mood disorders, anxiety disorders, and attention-deficit/hyperactivity disorder (ADHD) are common comorbid conditions in adolescents who use substances. Treatment of any comorbid psychological disorders is imperative for the successful management of substance use. For diagnostic accuracy, individuals should not be under the influence of substances during psychological evaluations.\n\nUrine drug screening would not be the best next step in treating this boy. High false-positive and false-negative rates can confound assessments and delay appropriate management. An evaluation for ADHD should be performed before this boy receives any stimulant medication. A follow-up visit should be scheduled sooner than 6 months.\n\nAdolescence is commonly divided into three phases: early (ages 10 to 13 years), middle (ages 14 to 17 years), and late (ages 18 to 21 years). Early adolescents typically have concrete thinking, are egocentric, and are becoming more concerned with how they are perceived by their peers. Middle adolescents begin to develop more abstract thinking, but they allow strong emotions to drive many of their decisions. Late adolescents can better navigate decisions and begin to develop their own values and goals. The prefrontal cortex, the area of the brain that controls executive function and higher-level decision making, does not fully develop until age 21 years or later.\n\nThe boy and his mother should be counseled that at age 14 years, children lack the full capacity to make healthy decisions for themselves because the brain is still developing and forming connections. Normal adolescent brains have a reward center that responds to high levels of activity (the \"gas pedal\") with a prefrontal cortex (the \"brake pedal\") that is not fully developed. This combination can lead to risky behaviors that are commonly seen in adolescents. To be successful and safe, adolescents require structure, monitoring, support, and accountability.\n\nThe boy in the vignette has engaged in daily substance use for the past year. While the brain is developing, substance use can decrease cognitive function and alter normal development. Thus, because of his daily substance use, the boy may be functioning at a cognitive level that is lower than expected for his chronological age.\n\nContent Domain\nBehavioral/Developmental\n\nABP Content Specification(s) / Content Area(s)\n- Understand the timing of and factors influencing the development of concrete thinking and abstract reasoning in adolescents, and provide health advice accordingly\n- Recognize how thought processes in early, middle, and late adolescence influence problem solving and risk taking"}
{"id" : 146, "question_text" : "A father brings his 2-year-old son to the emergency department after they had spent several hours in the garage while the father worked on the car. The father reports that approximately 30 minutes ago he heard the child coughing and found him with an open bottle of charcoal lighter fluid in his hands. On physical examination, the awake and alert child's temperature is 37.0°C, heart rate is 120 beats/min, respiratory rate is 24 breaths/min, blood pressure is 90/60 mm Hg, and oxygen saturation is 98%. His shirt is saturated with lighter fluid. You remove the boy's shirt and decontaminate his skin.\n\nOf the following, the MOST appropriate next step is to", "options" : "[\"obtain a STAT chest radiograph\", \"obtain a urine toxicology screen\", \"perform gastric lavage\", \"place the child under observation\", \"reassure the father and discharge the patient\"]", "explanation" : "The boy described in the vignette has ingested a hydrocarbon-containing substance. In contrast to most other toxic ingestions, management does not typically involve decontamination or elimination but focuses on respiratory stabilization and observation. The asymptomatic patient who has had a hydrocarbon exposure should be observed for the development of signs or symptoms for a minimum of 6 hours. A chest radiograph should be obtained 4 to 6 hours after exposure. If the radiograph is normal and the patient does not develop any symptoms during the observation period, he or she may be safely discharged.\n\nHydrocarbons are a large family of compounds that most commonly cause toxic effects in the pulmonary and central nervous systems when ingested. Hydrocarbon ingestion does not often lead to systemic toxicity unless other toxic substances such as camphor or pesticides are admixed with the hydrocarbon. Rather, aspiration during the swallowing event deposits the hydrocarbon in the pulmonary tree, where it directly injures the respiratory mucosa. The resulting damage leads to chemical pneumonitis. Aspiration risk is highest with low-viscosity, high-volatility hydrocarbons such as kerosene, furniture polish, mineral spirits, and gasoline. Highly volatile hydrocarbons may diffuse rapidly into the central nervous system, leading to ataxia, somnolence, stupor, or coma.\n\nClinical suspicion should be high for an aspiration event in any child who presents following a hydrocarbon exposure with a history of coughing/gagging or respiratory signs and symptoms such as tachypnea, wheezing, or hypoxemia. Evaluation of symptomatic patients should include assessment of oxygen saturation or arterial blood gasses and chest radiography. Urine toxicology screening is not routinely helpful unless an illicit coingestant is suspected. A chest radiograph should be obtained acutely in a symptomatic patient, but because radiographic findings often lag behind clinical findings, the radiograph should be repeated in 4 to 6 hours if it appears initially normal. Supportive care should be provided, including oxygen therapy, beta-agonist treatment for wheezing, and intubation/mechanical ventilation for respiratory failure. Antibiotics and corticosteroids are not indicated. Gastric lavage is contraindicated because it has the potential to cause further aspiration. Patients who have any respiratory signs or symptoms on presentation should be observed for 24 to 48 hours for disease progression.\n\nAmerican Board of Pediatrics Content Specification(s):\n\nKnow how to manage a child who has ingested a substance containing a hydrocarbon"}
{"id" : 2238, "question_text" : "A 2-month-old term infant is seen for a health maintenance visit. She has no significant family history. She has been growing and developing appropriately. The parents report that she appears to see and hear well. Of the following, the MOST appropriate components of vision screening for this infant are", "options" : "[\"corneal light reflex, pupil examination, and red reflex testing\", \"external inspection, pupil examination, and red reflex testing\", \"external inspection, red reflex testing, and cover test\", \"external inspection, red reflex testing, and photoscreening\"]", "explanation" : "Infant vision screening is critical for early detection of preventable vision-threatening and life-threatening conditions. The Table summarizes vision screening recommendations for infants and children according to age. For infants up to 6 months of age, the components of vision screening should include the following: \nExternal inspection: assess for structural abnormalities (eg, ptosis or hemangioma) that are risk factors for amblyopia, the permanent uncorrectable vision loss caused by chronic visual image distortion.\nPupil examination: assess for unequal, sluggish, or abnormally shaped pupils that may signal the need for further evaluation (eg, miosis associated with ptosis would raise concern regarding Horner syndrome and neuroblastoma).\nRed reflex testing: perform as a part of the newborn examination and at every health supervision visit; both eyes should be examined simultaneously using a direct ophthalmoscope from a distance of 2 to 3 feet; abnormal findings may include an absent, asymmetric, dull, or white reflex unilaterally or bilaterally.\nAbnormalities of the red reflex can be caused by disturbances anywhere along the visual axis: the retina (retinoblastoma), vitreous (vitreous hemorrhage), lens (cataract), or cornea (scar/infection). Any abnormality of the red reflex requires referral to a pediatric ophthalmologist. A finding of leukocoria (white reflex) requires urgent ophthalmology referral for evaluation for retinoblastoma or cataract. \nInfants up to 4 months of age may exhibit intermittent strabismus as a normal finding; therefore, the corneal light reflex and cover test are not useful in this age group. Infants older than 4 months with intermittent strabismus should be referred to a pediatric ophthalmologist.\nThe American Academy of Pediatrics recommends that instrument-based vision screening (via photoscreening device or autorefractor) start at 1 year of age and continue until a child is able to cooperate with the eye chart method. \nSuggested Reading(s)\nEyeRounds.org. University of Iowa Ophthalmology and Visual Sciences. Accessed October 27, 2023. eyerounds.org\nJoint policy statement: vision screening for infants and children. American Association for Pediatric Ophthalmology and Strabismus and the American Academy of Ophthalmology. Accessed October 27, 2023. www.aao.org/education/\nLoh AR, Chiang MF. Pediatric vision screening. Pediatr Rev. 2018;39(5):225-234. doi:10.1542/pir.2016-0191\nContent Domain\nPreventive Pediatrics\nLearning Objectives\nConduct vision screening on newborn infants \nDescribe conditions that can be detected with ophthalmoscopic examinations\nThe correct answer is: external inspection, pupil examination, and red reflex testing\nView Peer Results"}
{"id" : 1769, "question_text" : "You are seeing a 17-year-old girl for a health maintenance visit. During the interview, she reports that she has been sexually active with 2 lifetime male partners and engages only in vaginal intercourse. She is taking an oral contraceptive but uses condoms inconsistently. The girl reports no abnormal vaginal discharge, dysuria, or genital lesions. Of the following, you are MOST likely to recommend that the girl undergo screening for", "options" : "[\"cervical cancer\", \"chlamydia\", \"herpes simplex virus infection\", \"syphilis\", \"trichomoniasis\"]", "explanation" : "Correct Answer: B\nOf the response choices, the girl in the vignette should undergo screening for chlamydia. In the United States, the rates of many sexually transmitted infections (STIs) are highest among adolescents and young adults. One recommended control strategy is screening for infection. The Centers for Disease Control and Prevention (CDC) recommend screening for the following adolescent groups. (Note that testing for human immunodeficiency virus [HIV] should be offered to all adolescents and repeated every 3 to 5 years. For those at very high risk of infection [young men who have sex with men, injection drug users] repeat testing may be performed annually.)\n\nSexually active young women younger than 25 years:\n• Annual testing for Chlamydia trachomatis and Neisseria gonorrhoeae using a urine or vaginal swab specimen for nucleic acid amplification testing (NAAT)\n• Cervical cancer screening by cervical cytology (ie, the Papanicolaou test) is recommended beginning at age 21 years with repeat testing every 3 years until age 29 years.\n• Routine screening for herpes simplex virus infection, syphilis, trichomoniasis, bacterial vaginosis, hepatitis A, and hepatitis B is not advised.\n\nHeterosexual adolescent men:\n• Evidence does not support routine screening for C trachomatis and N gonorrhoeae (in part because the prevalence of asymptomatic infection is low)\n• In settings where the prevalence of infection is higher (eg, adolescent clinics, correctional facilities, STI clinics) screening for C trachomatis and N gonorrhoeae may be offered.\n\nYoung men who have sex with men:\n• Annual Screening for human immunodeficiency virus and syphilis\n• Annual screening for C trachomatis and N gonorrhoea\no If practicing insertive anal intercourse: urine NAAT for both organisms\no If practicing receptive anal intercourse: rectal swab NAAT for both organisms\no If practicing receptive oral intercourse: pharyngeal swab NAAT for N gonorrhoeae only (testing for pharyngeal infection with C trachomatis is not recommended)\n\nYoung women who have sex with women:\n• Limited information exists regarding STI transmission between women, but the risk likely is related to the type of sexual practice and the STI itself. Therefore, effective screening requires a candid discussion about sexual practices (eg, digital-vaginal contact, penetrative sex item, etc) and behaviors (eg, sharing of sex items).\n\nTransgender women:\n• The prevalence of HIV infection is high in this population (27.7% for all transgender women, 56.3% for black transgender women). However, data about other STIs are limited. STI screening recommendations depend on anatomic considerations and sexual practices.\n\nTransgender men:\n• Little information exists regarding the prevalence of STIs among transgender men, though HIV infection is less common than in transgender women. STI screening recommendations depend on anatomic considerations and sexual practices.\n\nIn addition to screening practices, the CDC and other organizations have published recommendations for pre- and postexposure strategies to control STIs. These include:\n• Postexposure prophylaxis following sexual assault (CDC recommendations):\no Empiric treatment for C trachomatis, N gonorrhoeae, and trichomoniasis infection\no Emergency contraception for women\no Hepatitis B immunization without hepatitis B immunoglobulin (if the hepatitis status of the assailant is unknown and the survivor has not previously been immunized)\no Human papillomavirus immunization (if the survivor has not previously been immunized)\no HIV postexposure prophylaxis depending on risk (see: CDC Sexually Transmitted Diseases Treatment Guidelines, https://www.cdc.gov/std/tg2015/default.htm and below)\n• Preexposure prophylaxis to prevent HIV infection:\no Daily antiretroviral therapy to reduce transmission for individuals at high risk for infection.\n• Nonoccupational postexposure prophylaxis to prevent HIV infection:\no Typically, a 2- or 3-drug regimen to prevent HIV infection after injection drug use, sexual, or other nonoccupational exposure.\no Nonoccupational postexposure prophylaxis should be initiated as soon as possible after exposure (ideally within 72 hours) and continued for 28 days.\no Consultation regarding nonoccupational postexposure prophylaxis is available from the National HIV/AIDS Clinician's Consultation PEPline at 1-888-448-4911.\n\nPREP Pearls\n• Sexually active adolescent girls should be screened annually for Chlamydia trachomatis and Neisseria gonorrhoeae using a urine or vaginal swab specimen for nucleic acid amplification testing (NAAT).\n• Routine screening for C trachomatis and N gonorrhoeae in sexually active adolescent boys is not recommended. However, screening may be offered to those at high risk for infection.\n• Cervical cancer screening by cervical cytology (ie, the Papanicolaou test) is recommended beginning at age 21 years with repeat testing every 3 years until age 29 years.\n\nMOCA-Peds Objective\n• Provide guidance regarding methods of contraception\n\nABP Content Specifications(s)\n• Understand the indications for a Papanicolaou test in female adolescents\n• Plan an appropriate screening evaluation for sexually transmitted infections in various adolescent populations\n• Plan appropriate prophylaxis following possible exposure to sexually transmitted infection\n\nSuggested Readings\n• Centers for Disease Control and Prevention. Sexually transmitted diseases treatment guidelines, 2015. MMWR Recomm Rep. 2015;64(No. RR-3):1-137.\n• Centers for Disease Control and Prevention. Updated guidelines for antiretroviral postexposure prophylaxis after sexual, injection drug use, or other nonoccupational exposure to HIV – United States, 2016. http://www.cdc.gov/hiv/pdf/programresources/cdc-hiv-npep-guidelines.pdf .\n• Practice bulletin number 157 summary: cervical cancer screening and prevention. Obstet Gynecol. 2016;127:185–187. doi: http://dx.doi.org/10.1097/AOG.0000000000001256.\n• Summaries for patients: screening for HIV—US Preventive Services Task Force Recommendation Statement. Ann Intern Med. 2013;159:51–60. doi: http://dx.doi.org/10.7326/0003-4819-159-1-201307020-00647.\n• US Public Health Service. Preexposure prophylaxis for the prevention of HIV infection in the United States 2014. A Clinical Practice Guideline. http://www.cdc.gov/hiv/pdf/prepguidelines2014.pdf."}
{"id" : 143, "question_text" : "A grandmother discovers her 18-month-old grandson in the garage near an opened container of greenish fluid that she suspects to be antifreeze. She brings the child to the urgent care center for evaluation. On physical examination, the boy appears sleepy and somewhat ataxic. Initial laboratory evaluation reveals:\n• Sodium, 140 mEq/L (140 mmol/L)\n• Potassium, 4.1 mEq/L (4.1 mmol/L)\n• Chloride, 105 mEq/L (105 mmol/L)\n• Bicarbonate, 16 mEq/L (16 mmol/L)\n• Calcium, 9.0 mg/dL (2.25 mmol/L)\n• Magnesium, 2.0 mEq/L (1.0 mmol/L)\n• Phosphorus, 5.5 mg/dL (1.8 mmol/L)\n• Glucose, 90 mg/dL (5.0 mmol/L)\n• Blood urea nitrogen, 14 mg/dL (5.0 mmol/L)\n• Creatinine, 0.4 mg/dL (35.4 mcmol/L)\n• Albumin, 4.0 g/dL (40 g/L)\n• Serum osmolality, 310 mOsm/kg\n\nOf the following, the osmolar gap in this child, who has a possible ingestion, is CLOSEST to", "options" : "[\"8\", \"12\", \"16\", \"20\", \"24\"]", "explanation" : "Antifreeze can contain up to 95% ethylene glycol, and clinical suspicion that the child described in the vignette ingested antifreeze is supported by the history and physical examination findings. Laboratory evaluation reveals an elevated anion gap metabolic acidosis. The anion gap is calculated by subtracting the sum of the commonly measured anions (chloride and bicarbonate) from the most commonly measured cation (sodium):\n\nNa+ – [Cl- + HCO3-].\n\nA normal gap is 12+4; this child has an anion gap of 19. Furthermore, he has an elevated serum osmolality at 310 mOsm/kg (normal, 275 to 290 mOsmol/kg). For a patient in whom an ingestion is suspected, serum osmolality should be measured in pursuit of a possible increased osmolar gap, especially in the setting of an increased anion gap metabolic acidosis. An osmolar gap is calculated by subtracting measured plasma osmolality from a calculated plasma osmolality, with a normal value being 10.\n\nOsmolality is measured in the plasma by freezing point depression technique. The osmolality can be calculated using the following equation:\n\nCalculated Posm = 2 x plasma [Na+] + [glucose]/18 + BUN/2.8\n\nFor the patient in the vignette, the calculated osmolality is:\n\nPosm = 2 x [140] + [90]/18 + [14]/2.8 = 280 + 5 + 5 = 290 mOsmol/kg\n\nUsing this figure and the measured plasma osmolality, the osmolar gap is calculated with the equation:\n\nOsmolar gap = Measured Posm – Calculated Posm\n\nIn this patient: Osmolar gap = 310 mOsmol/kg – 290 mOsmol/kg = 20 mOsmol/kg\n\nThe rationale behind the osmolar gap is that alcohols such as methanol, ethanol, and ethylene glycol are small in size, with molecular weights ranging from 32 to 62 mg/mmol. This means that relatively small amounts of these substances can result in relatively large changes in osmolality. The relationship of molecular weight to osmolality explains why most drugs (eg, salicylates), which have much large molecular weights, have minimal effects on osmolality.\n\nThe value of calculating both the anion gap and osmolar gap in a patient who has a suspected ingestion of ethylene glycol or methanol is earlier recognition and treatment. Metabolism of these alcohols by alcohol dehydrogenase can result in production of toxic metabolites, which increase the risks of morbidity and mortality. Ethylene glycol is metabolized to glycolic acid and oxalic acid, which are associated with neurologic, cardiopulmonary, and renal toxicity. Methanol (a component of deicing solutions and varnish) is metabolized to formaldehyde and formic acid, which are associated with blindness, coma, and death.\n\nTreatment for both ethylene glycol and methanol toxicity includes ethanol or fomepizole (to block alcohol dehydrogenase metabolism of these alcohols to toxic metabolites) and possibly hemodialysis. The use of fomepizole has obviated the need for ethanol and hemodialysis in select pediatric cases of ethylene glycol toxicity. Nonetheless, the practitioner should strongly consider consultation with a medical toxicologist and possibly a nephrologist to determine if hemodialysis may be needed.\n\nAmerican Board of Pediatrics Content Specification(s):\n\nKnow that the plasma osmolality can be estimated using serum electrolytes, blood urea nitrogen, and blood glucose concentrations"}
{"id" : 1446, "question_text" : "A 16-year-old adolescent girl presents to your clinic for evaluation of a mass in her left breast, which has been present for 3 months. Her menarche occurred at age 13 years and her last menstrual period ended 2 weeks ago. She reports no change in the mass with her periods. On physical examination, you palpate a 2.5-cm diameter mobile mass in the upper outer quadrant of the patient's left breast. Of the following, the MOST likely diagnosis for the patient in the vignette is", "options" : "[\"breast abscess\", \"breast carcinoma\", \"fibroadenoma\", \"fibrocystic changes\", \"phyllodes tumor\"]", "explanation" : "Correct Answer: C\nThe most likely diagnosis for the adolescent girl in the vignette is a fibroadenoma. Breast masses among adolescent girls are typically benign. In a study of approximately 2,800 young women with breast disease, more than 98% were of benign etiology. On excisional biopsies of breast masses in adolescent girls, most are fibroadenomas. Overall, fibrocystic changes are significantly more common in adolescent girls, but biopsies are rarely performed.\n\nFibroadenomas are well-circumscribed, smooth, mobile lesions that typically do not change during the course of the menstrual cycle. The diagnosis is typically made by the combination of physical examination and the appearance of clearly defined, homogeneous, hypoechoic densities on ultrasonography. Most fibroadenomas are less than 3 cm in diameter; giant fibroadenomas are more than 5 cm. Management is usually conservative, because many fibroadenomas will spontaneously regress. Giant fibroadenomas may necessitate surgical excision because of breast distortion.\n\nBreast abscesses typically present acutely, with symptoms of inflammation such as erythema, pain, swelling, and fever. Treatment should include coverage for likely skin pathogens (eg, staphylococci, streptococci). Surgical intervention may be required.\n\nBreast cancer is uncommon among adolescents; therefore, both breast carcinoma and phyllodes tumor are unlikely in this scenario.\n\nAdolescents with fibrocystic changes in the breast often complain of breast tenderness, with peak symptoms near the time of menstruation. Physical examination will reveal nodular breasts with indistinct masses. Management includes analgesics; symptoms typically improve with oral contraceptives.\n\nAdolescent boys can also present with the complaint of breast mass. In boys, this complaint is most often secondary to gynecomastia. Physiologic gynecomastia occurs at puberty, and is likely due to a relative delay in testosterone secretion in comparison with estrogen, which stimulates breast development. Pubertal gynecomastia typically resolves in 12 to 18 months.\n\nPREP Pearls\n• Breast masses among adolescent girls are typically benign.\n• On excisional biopsies of breast masses in adolescent girls, most are fibroadenomas.\n• Fibroadenomas are well-circumscribed, smooth, mobile lesions that typically do not change during the course of the menstrual cycle. Management is usually conservative, because many fibroadenomas will spontaneously regress.\n\nABP Content Specifications(s)\n• Understand the significance of a breast mass in an adolescent girl as it relates to puberty\n\nSuggested Readings\n• Diamantopoulos S, Bao Y. Gynecomastia and premature thelarche: a guide for practitioners. Pediatr Rev. 2007;28(9):e57-e68. doi: http://dx.doi.org/10.1542/pir.28-9-e57.\n• Gooding HC, DiVasta AD. Breast disorders and gynecomasia. In: Neinstein LS, Katzman DK, Callahan T, Gordon CM, Joffe A, Rickert V, eds. Neinstein's Adolescent and Young Adult Health Care: A Practical Guide. 6th ed. Philadelphia, PA: Wolters Kluwer; 2016:461-470."}
{"id" : 320, "question_text" : "You are seeing a 14-month-old boy for a follow-up visit for anemia. When he was 12 months old, his hemoglobin value was 10 g/dL (100 g/L). Further testing at 13 months revealed a mean corpuscular volume of 62 fL on complete blood count, with the remainder of the red blood cell indices and the smear within normal limits. Reticulocyte count was 0.5% (0.005). When you review the child's diet, his mother reports that he drinks 40 oz of cow milk per day and his favorite food is macaroni and cheese. He eats little meat and few vegetables. Of the following, the BEST recommendation for management of this child's condition is to", "options" : "[\"encourage replacement of some cow milk with fruit juice\", \"prescribe a chewable multivitamin with iron tablet once daily\", \"prescribe therapeutic ferrous sulfate drops three times daily\", \"resume use of infant formula until his hemoglobin increases\", \"switch from cow milk to a 30 kcal/oz cow milk-based nutritional supplement\"]", "explanation" : "The boy described in the vignette has a low mean corpuscular volume and hemoglobin value consistent with iron deficiency. The most common cause of iron deficiency in this age group is inadequate dietary intake of iron due to a lack of iron-rich foods in the diet. Cow milk contains little bioavailable iron. Furthermore, drinking large quantities of cow milk in infancy may be associated with significant intestinal blood loss.\n\nThe most appropriate management of this boy's anemia is the administration of ferrous sulfate drops three times daily. After 2 to 4 weeks of therapy, a repeat complete blood count and reticulocyte count should be performed to determine if he is responding to therapy. If so, the ferrous sulfate should be continued for 3 months to replenish iron stores. If he does not respond to this therapy, further evaluation is indicated.\n\nA chewable multivitamin with iron is intended to provide the minimum daily requirement for dietary intake; it contains inadequate iron to treat deficiency. Similarly, infant formula and cow milk-based supplements do not contain sufficient iron to treat deficiency. Substituting juice for milk may reduce gastrointestinal blood loss from cow milk protein exposure, but it does not provide adequate protein and nutrients for a healthy diet. Indeed, excessive intake of fruit juice is associated with the development of obesity and can cause diarrhea.\n\nInfant formulas contain enough iron to meet the routine needs of growing infants younger than 6 months of age. In the past, gastrointestinal disturbances, especially constipation and colic, were mistakenly attributed to the presence of iron in infant formula, and low-iron formulas were made available. Use of such formulas may have contributed to iron deficiency in infants who required iron to recover from physiologic anemia of infancy as well as to provide adequate iron for central nervous system health. Infant cereals have been recommended as a source of extra iron for older infants, but recent concern about the relative nutritional \"emptiness\" and high simple carbohydrate content of cereals makes this recommendation somewhat obsolete. New recommendations include introduction of meat into the diet at 6 months and more attention to vegetable sources of iron.\n\nPrior recommendations to delay the introduction of meat into the infant diet in the United States also may have contributed to the risk for iron deficiency in older infants. Recent evidence points to introduction of meat as early as age 4 months, a practice common in other parts of the world, as a means of increasing iron in the diet that is associated with no additional risk for food allergy or other adverse outcomes.\n\nIn November of 2010, the American Academy of Pediatrics released new recommendations for iron supplementation of infants and children. A new emphasis is placed on ensuring adequate supplementation of exclusively breastfed infants who are not receiving complementary feedings of iron-rich foods after age 4 months. Breastfed babies should be supplemented with 1 mg/kg per day of iron starting at age 4 months.\n\nCritique: Preferred Response: C\n\nContent Specifications: Know that dietary deficiency is the most common cause of iron deficiency anemia in young children. Know that cow milk contains very little bio-available iron and that an infant with iron deficiency often drinks large amounts of cow milk. Judge the nutritional adequacy of infant formulas in relation to mineral content"}
{"id" : 3554, "question_text" : "What is the primary clinical implication of the finding that median age for pediatric sepsis is less than 3 years compared to 60-65 years in adults?", "options" : "[\"Years of life lost is three to four times higher in pediatric sepsis, making it a significant public health priority despite lower absolute mortality rates\", \"Pediatric sepsis is less severe and requires less intensive treatment\", \"Children recover more quickly than adults from sepsis complications\", \"Pediatric sepsis has a better long-term prognosis than adult sepsis\"]", "explanation" : "The text emphasizes that although pediatric ED mortality is 1-5% compared to higher adult rates, the years of life lost is three to four times higher due to the young age of affected children, underscoring the public health burden."}
{"id" : 3143, "question_text" : "A 3½-week-old neonate is seen in your office for vomiting. He was born at term and his birth weight was 3,250 g. He is breastfed. For the past 2 to 3 days, he has been exhibiting nonbilious vomiting after each feeding. After vomiting, he seems eager to resume nursing. Over the past 24 hours, his mother has noted fewer wet diapers and less stool than usual. The baby has experienced no fever, diarrhea, or upper respiratory tract symptoms. Physical examination demonstrates an alert infant sucking vigorously on a pacifier. His weight is 3,550 g, temperature is 37°C, pulse rate is 160 beats/ min, respiratory rate is 40 breaths/min, and blood pressure is 70/50 mm Hg. His skin turgor is normal. The remainder of the examination findings are unremarkable except for slight abdominal distention. You obtain the following laboratory studies:\n• Sodium, 130 mEq/L (130 mmol/L)\n• Potassium, 3.2 mEq/L (3.2 mmol/L)\n• Chloride, 95 mEq/L (95 mmol/L)\n• Bicarbonate, 30 mEq/L (30 mmol/L)\n• Blood urea nitrogen, 15 mg/dL (5.4 mmol/L)\n• Creatinine, 0.2 mg/dL (18 umol/L)\n\nOf the following, the MOST appropriate initial treatment for this infant is", "options" : "[\"continued nursing, pending results of further diagnostic tests\", \"intravenous 5% dextrose and 0.3% sodium chloride at 15 mL/h\", \"intravenous 5% dextrose and 0.45% sodium chloride at 25 mL/h\", \"intravenous 0.9% sodium chloride, 70 mL over 1 hour, followed by infusion with 5% dextrose and 0.3% sodium chloride at 15 mL/h\", \"oral rehydration with a glucose-electrolyte solution containing 70 mEq to 90 mEq (70-90 mmol/L) of sodium chloride per liter\"]", "explanation" : "The progressive nonbilious vomiting and hypochloremic metabolic alkalosis described for the infant in the vignette suggest the presence of a gastric outlet obstruction. Infantile hypertrophic pyloric stenosis (HPS) is the most likely cause of such clinical findings. The initial goals of therapy are assessment of hydration status and institution of appropriate intravenous rehydration, with gradual correction of any electrolyte disturbances. Only when hydration status has normalized and electrolyte abnormalities have been corrected should surgical pyloromyotomy be undertaken. Given this infant's birth weight of 3,250 g and an expected mean weight gain of 20 g/day, the current weight should be approximately 3,750 g. The infant's actual weight of 3,550 g likely indicates mild dehydration (~5%). Based on the serum electrolyte data, fluid therapy should be initiated with 5% dextrose and 0.45% sodium chloride at approximately 1.5 times the calculated maintenance rate (~600 mL per 24 hours, based on the infant's hydrated weight) or 25 ml/h. Oral rehydration therapy, including breast milk and glucose electrolyte solutions, is not recommended in this infant whose serum electrolyte values indicate metabolic derangements consistent with HPS.\n\nThe infant in the vignette is estimated to be only 5% dehydrated, therefore vigorous fluid management with a bolus infusion is not required. In most cases of HPS, appropriate initial intravenous fluid therapy involves a sodium chloride concentration between 0.45% and 0.9%, depending on the magnitude of the electrolyte deficits. Intravenous therapy with concentrations of less than 0.45% sodium chloride should be avoided, unless the condition is diagnosed early in the disease course and all electrolyte and acid-base values are normal. Potassium should be added to the infusate as soon as urinary output is established to prevent progressive hypokalemia. However, because the serum potassium concentration rises as the serum pH normalizes, potassium levels must be carefully monitored. Bolus infusions with 10 to 20 mL/kg 0.9% sodium chloride are reserved for infants who demonstrate clinically moderate-to-severe intravascular fluid depletion (ie, >7% dehydration).\n\nHypertrophic pyloric stenosis is the most frequent cause of metabolic alkalosis in the first few weeks after birth and the most common indication for abdominal surgery, with an estimated incidence of 1 in 250 live births. The condition occurs more commonly in white males, but greater risk of occurrence in first-born male children has not been proven. Familial clustering has been reported. Other, less common causes of gastric outlet obstruction include duodenal stenosis, gastric duplication, antral web, and annular pancreas.\n\nAlthough hypochloremic, hypokalemic metabolic alkalosis represents the classic electrolyte and acid-base imbalances of HPS, many infants have normal serum electrolyte values, therefore increasing the importance of having a \"high index of suspicion\" for this diagnosis. The magnitude of any acid-base and electrolyte disturbance is related directly to the duration of vomiting. Persistent emesis results in a progressive loss of gastric fluid and hydrochloric acid. Hypokalemia ensues as the kidneys retain hydrogen ions in favor of potassium. As extracellular fluid volume and hydrogen ions continue to be lost because of continued postprandial emesis and intravascular volume contracts, increasing the relative serum bicarbonate concentration leads to a contraction alkalosis. Serum potassium values fall further as alkalosis stimulates potassium-hydrogen exchange across cell membranes, with potassium driven intracellularly and hydrogen ions driven extracellularly.\n\nPREP Pearls\n• Hypochloremic, hypokalemic metabolic alkalosis represents the classic electrolyte and acid-base imbalances of hypertrophic pyloric stenosis (HPS).\n• In the fluid management of HPS, infused sodium concentrations of less than 0.45% should be avoided.\n• Serum potassium levels will rise in patients with alkalosis as the blood pH returns to normal.\n• Bolus intravenous fluid infusions should not be utilized in patients presenting with dehydration levels less than or equal to 5%.\n\nAmerican Board of Pediatrics Content Specification(s)\n• Recognize the acid-base changes associated with pyloric stenosis, and manage appropriately\n\nSuggested Reading\n• Dinkevich E, Ozuah PO. Pyloric stenosis. Pediatr Rev. 2000;21(7):249-250. doi:10.1542/pir.21-7-249.\n• Garcia VF, Randolph IG. Pyloric stenosis: diagnosis and management. Pediatr Rev. 1990;11(10):292-296. doi:10.1542/pir.11-10-292.\n• Rice HE, Caty MG, Glick PL. Fluid therapy for the pediatric surgical patient. Pediatr Clin North Am. 1998;45(4):719-727.\n• Roberts KB. Fluid and electrolytes: parenteral fluid therapy. Pediatr Rev. 2001;22(10:380-387. doi:10.1542/pir.22-11-380."}
{"id" : 915, "question_text" : "A 16-year-old girl presents to your office following an injury she sustained playing soccer. One week ago, another player hit her in the chest, causing her head to snap back. She had near-immediate onset of a diffuse frontal headache and mild nausea that lasted several hours after the injury. She did not lose consciousness. Her mother reports that the girl has seemed more forgetful and has been having trouble sleeping since the injury. She has not played soccer since the injury.\n\nOf the following, you are MOST likely to tell the girl and her mother that this injury was", "options" : "[\"a concussion and she can return to soccer since it has been 1 week since the injury\", \"a concussion and she can return to soccer since she no longer has headache\", \"a concussion and she should continue to rest from all physical activities\", \"not a concussion because she did not get hit in the head\", \"not a concussion because she did not have loss of consciousness\"]", "explanation" : "The girl described in the vignette sustained a concussion; she should not return to physical activities because she is still symptomatic. Among commonly played high school sports, the highest rates of sports concussion occur in American foot-ball, boys' ice hockey, boys' lacrosse, and girls' soccer. A concussion is a type of traumatic brain injury that occurs from a direct blow to the head or from a transmitted force causing linear or rotational acceleration. Individuals with concussion generally experience onset of symptoms within several hours following injury. Standard brain imaging, such as computed tomography and magnetic resonance imaging, yields normal results in concussed individuals. Symptoms can include somatic symptoms (eg, headache, nausea, or visual disturbance), cognitive symptoms (eg, difficulty concentrating or feeling confused), mood symptoms (eg, anxiety or irritability), and sleep disturbance. Over the past decade, the definition of concussion has evolved, with loss of consciousness no longer a criterion for establishing the diagnosis.\n\nChildren and adolescents who have concussion experience a 10- to 14-day average duration of symptoms. Younger individuals experience a more prolonged recovery. Treatment for concussion includes \"brain rest:' such as deferring tests at school, avoiding \"screen time\" (ie, computers, television, texting, and video games), as well as complete rest from physical activity. The current consensus guidelines for the management of sports concussion stipulate that concussed individuals can begin a progression back to physical activity if neurologic examination results are normal and athletes are asymptomatic or at symptom baseline. Use of additional evaluation, such as computerized neuropsychological testing, may be used to determine whether return to sports is appropriate.\n\nPREP Pearls\n• Loss of consciousness is not required for the diagnosis of concussion.\n• A concussion can occur with a transmitted force to the head.\n• In individuals with concussion, standard brain imaging yields normal results.\n• Generally, individuals with concussions can begin a progression back to physical activity if neurologic examination results are normal and athletes are asymptomatic or at symptom baseline.\n• Treatment for concussion includes \"brain rest\" as well as complete rest from physical activity.\n\nAmerican Board of Pediatrics Content Specification(s):\n• Know the sports most commonly associated with a head injury.\n\nSuggested Reading:\n• Guskiewicz KM, Valovich McLeod TC. Pediatric sports-related concussion. PM R. 2011;3(4):353-364. doi:10.1016/j.pmrj.2010.12.006\n• Marar M, Mcllvain NM, Fields SK, Comstock RD. Epidemiology of concussions among United States high school athletes in 20 sports. Am Sports Med. 2012;40(4):747-755. doi:10.1177/0363546511435626\n• McCrory P, Meeuwisse W, Johnston K, et al. Consensus Statement on Concussion in Sport: the 4th International Conference on Concussion in Sport held in Zurich, November 2012. Br I Sports Med. 2013;47:250-258. doi:10.1136/ bjsports-2013-092313"}
{"id" : 2071, "question_text" : "A 3-month-old male infant is brought to the emergency department for vomiting that began 10 hours ago. Initially the emesis resembled formula, however over the last few hours his parents have noticed light-brown emesis. Although he has been more irritable than usual over the last day, he is now quiet and sleepy. His medical history is significant for heterotaxy syndrome, which was diagnosed prenatally, as well as gestational diabetes and maternal tobacco smoke exposure. He was born via spontaneous vaginal delivery at 39 weeks' gestation. His postnatal course was remarkable for diagnosis of a small ventricular septal defect, and he was discharged to home 5 days after birth. The infant currently appears lethargic. He has a temperature of 39.3°C and heart rate of 190 beats/min. His abdomen is distended and tender to touch. His extremities are warm, and he has a 7-mm hemangioma on his right arm. There is concern for bowel obstruction caused by midgut volvulus. Of the following, the component of this infant's history that is MOST likely to be associated with his current condition is", "options" : "[\"gestational diabetes\", \"heterotaxy syndrome\", \"infantile hemangioma\", \"maternal tobacco smoke exposure\"]", "explanation" : "The infant in this vignette is critically ill with a midgut volvulus, which is associated with heterotaxy syndrome in which failure of normal embryological rotation results in abnormally positioned organs in the chest and abdomen. In infants and children, a midgut volvulus is most commonly associated with intestinal malrotation, a congenital abnormality caused by incomplete in utero rotation of the bowel around the superior mesenteric artery. Intestinal malrotation is often associated with other congenital anomalies including heterotaxy syndrome, omphalocele, gastroschisis, congenital diaphragmatic hernia, esophageal atresia, renal anomalies, and cardiac anomalies.\n\nWith normal in utero intestinal rotation, the third portion of the duodenum crosses the midline of the abdomen. With malrotation, the duodenum is abnormally fixed, thus the third portion of the duodenum is positioned in the right upper quadrant of the abdomen. The abnormal duodenal positioning increases the risk for the bowel to twist on itself, resulting in a midgut volvulus. The twisting and subsequent obstruction can quickly progress to bowel ischemia and necrosis.\n\nMidgut volvulus must be quickly diagnosed and treated, because sepsis and shock can rapidly ensue. Signs of midgut volvulus include bilious emesis, abdominal distention, hematochezia, and peritonitis. If the diagnosis is suspected clinically (ill- or toxic-appearing infant with bilious emesis and peritonitis), resuscitation and emergent laparotomy (without radiographic imaging) are indicated with operative correction of the volvulus and malrotation (Ladd procedure). Radiologic imaging may be used to diagnose malrotation and volvulus, particularly in an infant or child who is less toxic appearing. Plain abdominal radiographs may show signs of a small bowel obstruction; however, they may appear normal, even in the setting of malrotation with an acute volvulus. Upper gastrointestinal series is the gold standard for diagnosis of malrotation and volvulus, because this fluoroscopic study can evaluate the positioning of the duodenum. Item C273 demonstrates intestinal malrotation (the third portion of the duodenum does not cross midline) and volvulus (\"corkscrew\" sign).\n\nGestational diabetes, history of hemangiomas, and maternal tobacco smoke exposure are not associated with increased risk of midgut volvulus.\n\nPREP Pearls\n\nInfants and children with heterotaxy syndrome and/or gastrointestinal, renal, or cardiac anomalies are at risk for intestinal malrotation.\n\nMidgut volvulus is a surgical emergency that requires quick diagnosis and treatment.\n\nGold standard for diagnosis of malrotation and volvulus is an upper gastrointestinal series; however, surgical intervention may be warranted in toxic-appearing infants and children with suspected volvulus, even before radiologic testing confirms the diagnosis.\n\nABP Content Specifications(s)/Content Area\n\nRecognize the clinical features associated with volvulus, and manage appropriately\n\nSuggested Readings\n\nKapadia MR. Volvulus of the small bowel and colon. Clin Colon Rectal Surg. 2017;30(1):40-45. doi: 10.1055/s-0036-1593428.\n\nLanger JC. Intestinal rotation abnormalities and midgut volvulus. Surg Clin North Am. 2017;97(1):147-159. doi: 10.1016/j.suc.2016.08.011.\n\nShalaby MS, Kuti K, Walker G. Intestinal malrotation and volvulus in infants and children. BMJ. 2013;347:f6949. doi: 10.1136/bmj.f6949."}
{"id" : 1600, "question_text" : "You are called to admit a 36-week-gestation male newborn to the nursery who was delivered vaginally to a 27-year-old gravida 3, para 1 woman. The mother's prenatal history is significant for an Escherichia coli urinary tract infection and positive group B Streptococcus (GBS) status. Rupture of membranes occurred 8 hours before delivery. The mother received 1 dose of intravenous penicillin 3 hours before delivery, for GBS prophylaxis. The neonate is rooming in with his mother and formula feeding well. The mother has a 4-year-old son at home, and requests that her newborn be discharged 24 hours after birth. You discuss with her the current guidelines for care of neonates born to GBS-positive mothers. Of the following, the MOST appropriate management plan for this neonate would be", "options" : "[\"complete blood cell count, blood culture, and cefotaxime administration pending culture results\", \"complete blood cell count and blood culture, with possible discharge at 24 hours of age\", \"complete blood cell count and blood culture, with possible discharge at 72 hours of age\", \"observation in the newborn nursery unit, with possible discharge at 24 hours of age\", \"observation in the newborn nursery unit, with possible discharge at 48 hours of age\"]", "explanation" : "Correct Answer: E\nIn the vignette, a late preterm infant at 36 weeks' gestation was born to a mother who was group B Streptococcus (GBS)–positive and treated with 1 dose of penicillin. He should be observed for 48 hours because of premature gestation and inadequate intrapartum antibiotic prophylaxis during labor (<4 hours before delivery). Early-onset sepsis (EOS) continues to be a significant cause of morbidity and mortality for term neonates born in the United States, with a rate of 0.57 per 1,000 live births. Neonates are exposed to microorganisms from the maternal genital and anorectal tract during labor or via ascending spread after rupture of membranes. The primary organisms responsible for EOS are Streptococcus agalactiae, also known as GBS, and Escherichia coli. Most commonly, EOS due to GBS presents with respiratory distress secondary to GBS pneumonia. However, infected infants may also present with asymptomatic bacteremia.\n\nPregnant women should undergo vaginal-rectal screening for GBS colonization between 35 and 37 weeks of gestation. Women who screen positive for GBS should receive prophylactic antibiotics during labor, at least 4 hours before delivery, to decrease transfer of GBS from the mother to infant. Since the advent of maternal screening and intrapartum prophylaxis against GBS, rates of EOS due to GBS have decreased. However, intrapartum antibiotic prophylaxis does not eliminate the risk of EOS infection. Other risk factors for EOS include prematurity, prolonged rupture of membranes, and maternal intrauterine infection. Inadequate intrapartum antibiotic prophylaxis in a high-risk group may result in partial treatment and delayed onset of symptoms.\n\nNeither laboratory tests nor the use of broad-spectrum antibiotics, such as cefotaxime, is indicated in a well-appearing infant, despite maternal GBS-positive status. Discharge from the hospital 24 hours after birth should be reserved for well-appearing term neonates born to mothers without GBS colonization or mothers with GBS colonization who receive adequate intrapartum antibiotic prophylaxis, provided adequate care and follow-up can be assured.\n\nPREP Pearls\n• Premature neonates, those born before 37 weeks of gestation, are at increased risk for early-onset sepsis.\n• Group B Streptococcus (GBS) and Escherichia coli remain the most common causes of early-onset sepsis, despite the implementation of GBS screening and intrapartum prophylaxis.\n• Inadequate antibiotic prophylaxis before delivery may delay the onset of symptoms of GBS infection, necessitating observation for 48 hours before discharge.\n• Women who screen positive for GBS should receive prophylactic antibiotics during labor, at least 4 hours before delivery, to decrease transfer of GBS from the mother to neonate.\n\nABP Content Specifications(s)\n• Recognize the major clinical features associated with group B streptococcal infection, and manage appropriately\n• Understand the epidemiology of Streptococcus agalactiae\n• Plan the appropriate management of an infant born to a mother with a positive culture for group B streptococcus\n\nSuggested Readings\n• Higgins RD, Saade G, Polin RA, et al; Chorioamnionitis Workshop Participants. Evaluation and management of women and newborns with a maternal diagnosis of chorioamnionitis: summary of a workshop. Obstet Gynecol. 2016;127(3):426–436. doi: http://dx.doi.org/10.1097/AOG.0000000000001246.\n• Sass L. Group B streptococcal infections. Pediatr Rev. 2012; 33:219. doi: http://dx.doi.org/10.1542/pir.33-5-219.\n• Stoll BJ, Hansen NI, Sánchez PJ, et al. Early onset neonatal sepsis: the burden of Group B Streptococcal and E. coli disease continues. Pediatrics. 2011;127:817. doi: http://dx.doi.org/10.1542/peds.2010-2217."}
{"id" : 195, "question_text" : "An 18-month-old boy is brought to the emergency department after being found in his grandfather's room with several open pill bottles. The family reports that they removed two unidentifiable tablets from his mouth and found 23 more scattered on the floor. The medications include terazosin, simvastatin, aspirin, acetaminophen, and allopurinol. The sleepy but arousable child has a temperature of 37.0°C, heart rate of 160 beats/min, respiratory rate of 24 breaths/min, and blood pressure of 66/34 mm Hg. The remainder of his physical examination findings are normal. Of the following, the medication that is MOST likely to be the cause of this child's clinical findings is", "options" : "[\"acetaminophen\", \"allopurinol\", \"aspirin\", \"simvastatin\", \"terazosin\"]", "explanation" : "The boy described in the vignette is lethargic and hypotensive, presumably due to a toxic ingestion. Of the medications listed, only terazosin, an alpha-blocker used to treat symptoms associated with prostatic hypertrophy, causes hypotension. Many medications commonly used to treat various conditions in adults, from hypertension to insomnia, may be found in a child's environment and ingested accidentally. Hypotension following an ingestion can be an important clue to the identity of the ingested agent (Item C86).\n\nWhen evaluating a child following an unknown ingestion, it is important to collect information that will help in identifying agents that may cause life-threatening symptoms or complications and have specific treatments or antidotes. Assessment of vital signs, pupil size, skin, and neurologic status can provide useful clues. More importantly, however, is initial stabilization of the patient and correction of any vital sign derangements. The adage \"treat the patient, not the poison\" reminds the clinician that the treatment of patients who have ingested toxic substances is primarily supportive, reactive, and often not specific to the actual agent.\n\nA patient who is hypotensive following an unknown ingestion should be treated initially with fluid resuscitation. Fluid boluses of 20 mL/kg 0.9% saline should be administered rapidly, with careful reassessment following each bolus. If the patient's blood pressure does not normalize after three boluses, vasopressor agents such as dopamine or dobutamine should be considered. Electrocardiography and bedside glucose measurement also should be obtained to determine if the hypotension is related to a cardiac dysrhythmia or hypoglycemia.\n\nCritique: Preferred Response: E\n\nContent Specifications: Recognize the signs and symptoms of ingestion of medications that produce hypotension"}
{"id" : 1547, "question_text" : "A 22-month-old boy is hospitalized in a burn center after a scald injury. The injury was sustained 2 days ago after he pulled a pot of boiling water from the stove top to the floor when his mother stepped away from the kitchen. He has a temperature of 39.1°C, heart rate of 150 beats/min, respiratory rate of 38 breaths/min, blood pressure of 98/66 mm Hg, and oxygen saturation of 100% on 2 L/min of oxygen via nasal cannula. He is distressed. He has decreased breath sounds in both bases, no murmur, abdominal distention, and denuded skin over portions of his right arm, abdomen, and lower legs in a splash pattern. There are newly violaceous edges on the abdominal wound. Of the following, the BEST indication for starting parenteral antibiotics in this patient is", "options" : "[\"abnormal respiratory examination results\", \"discoloration at abdominal wound edges\", \"heart rate greater than 120 beats/min\", \"temperature greater than 38.3\\u00b0C\", \"wound involving greater than 10% of body surface area\"]", "explanation" : "The best indication for starting parenteral antibiotics in the patient in this vignette is discoloration of wound edges. Antibiotics for burn injury patients should be reserved for clinical situations with clear evidence of an active infection. A change in the appearance of the wound, including new discoloration, is the most suggestive of an infection.\n\nInfections are a major contributor to mortality associated with burns. The major infections in patients with burn injuries include pneumonia, burn wound infections, bloodstream infections, and urinary tract infections. The bloodstream infections and urinary tract infections may be associated with indwelling supportive devices.\n\nExtensive burns produce a systemic inflammatory response that results in intravascular hypovolemia from capillary leak and a hypermetabolic state. As a result, vital sign abnormalities, including fever, tachycardia, and tachypnea, are common in patients with burn injuries.\n\nAlthough pneumonia is an infectious consideration in burn patients, tachypnea and decreased basilar breath sounds are not specific findings for pneumonia. Similarly, tachycardia is not necessarily a sign of septic shock because it can be caused by various etiologies, including hypovolemia and pain. In burn injuries, fever can result from elevated core temperatures and from the systemic inflammatory response that causes a hypermetabolic state. Fever is not an indication for antibiotics because fever is not a specific marker of infection in patients with burn injuries.\n\nLastly, the extent and severity of a burn influence morbidity and mortality and factors into whether a patient requires treatment at a regional burn center. However, the extent of a burn is not an indication for antibiotics.\n\nPREP Pearls\n• Moderate to severe burn injuries create a severe systemic inflammatory response that can cause vital sign abnormalities, including fever, tachycardia, and tachypnea.\n• Antibiotics for burn injury patients should be reserved for clinical situations with clear evidence of an active infection.\n• A change in the appearance of a burn wound, including new discoloration, is suggestive of an infection.\n\nABP Content Specifications(s)\n• Recognize the major infections seen in patients with burn injuries\n\nSuggested Readings\n• Jamshidi R, Sato T. Initial assessment and management of thermal burn injuries in children. Pediatr Rev. 2013;34(9):395–403. doi: http://dx.doi.org/10.1542/pir.34-9-395.\n• Mayhall C. The epidemiology of burn wound infections: then and now. Clin Infect Dis. 2003;37(4):543–550. doi: http://dx.doi.org/10.1086/376993.\n• Sharma B. Infection in patients with severe burns: cause and prevention thereof. Infect Dis Clin North Am. 2007;21(3):745–759. doi: http://dx.doi.org/10.1016/j.idc.2007.06.003."}
{"id" : 2150, "question_text" : "An 8-year-old is seen in an urgent care center for 1 day of vomiting and diarrhea. She has no significant past medical history. She has not had fever, headache, abdominal pain, or blood in her stool. She has been unable to tolerate liquids at home and has urinated once in the past 24 hours. The girl appears tired but is fully oriented. Her heart rate is 120 beats/min, respiratory rate is 18 breaths/min, blood pressure is 96/60 mm Hg, and weight is 25 kg. Her mucous membranes are dry, capillary refill time is 3 seconds, and abdomen is soft without focal tenderness or peritoneal signs. The remainder of her examination findings are normal. Laboratory results are shown: Sodium 137 mEq/L (137 mmol/L), Potassium 4.6 mEq/L (4.6 mmol/L), Bicarbonate 20 mEq/L (20 mmol/L), Chloride 110 mEq/L (110 mmol/L), Urea nitrogen 10 mg/dL (3.57 mmol/L), Creatinine 0.6 mg/dL (53.04 µmol/L), Glucose 78 mg/dL (4.33 mmol/L). Of the following, the BEST next step is", "options" : "[\"oral rehydration with a solution containing 20 g/L of glucose and 75 mEq/L of sodium\", \"oral rehydration with a solution containing 250 g/L of glucose and 20 mEq/L of sodium\", \"parenteral rehydration with a 200 mL bolus of lactated Ringer solution\", \"parenteral rehydration with a 500 mL bolus of 0.9% sodium chloride solution\"]", "explanation" : "Correct answer is A\n\nOf the response choices, the best next step in this girl's management is oral rehydration with a solution containing 20 g/L of glucose and 75 mEq/L of sodium. The girl in the vignette has signs and symptoms of acute gastroenteritis with mild to moderate dehydration (Table). The recommended treatment for dehydration is to administer fluids using the least invasive route possible. As the child is fully oriented without signs of an abdominal emergency, fluid replacement should be initiated orally. The World Health Organization recommends use of an oral rehydration solution with a glucose concentration of ≤20 g/L and sodium concentration of 60 to 90 mEq/L. Solutions with high concentrations of glucose (eg, juice, soda) are not recommended for children with diarrhea due to their high osmotic load, which can contribute to further diarrhea.\n\nOral rehydration solutions should ideally be administered to children with dehydration in a supervised setting, such as a pediatric office, urgent care center, or emergency department. The child's condition should be reassessed frequently during the 4 to 6 hour rehydration phase. The recommended volume and timing for administration of oral hydration is 5 mL/min for infants, 10 mL/min for toddlers, and 15 mL/min for older children. Vomiting typically subsides as dehydration and acidosis resolve. Vomiting is not a contraindication to continued oral rehydration, as the entire stomach contents are not usually expelled. However, parenteral rehydration may be indicated when children are too fatigued to tolerate appropriate oral rehydration attempts, or if there is repeated emesis during the initial phase of oral rehydration.\n\nParenteral rehydration is indicated for a child with an altered level of consciousness, signs of severe dehydration or shock, severe respiratory distress, findings concerning for a surgical abdomen, or for those who fail oral rehydration. The girl in the vignette does not have any of these indications. Parenteral rehydration should begin with a bolus of 10 to 20 mL/kg of an isotonic solution (0.9% saline or lactated Ringer solution). For the 25 kg girl in the vignette, the recommended initial bolus volume for parenteral rehydration would be 250 to 500 mL of isotonic solution.\n\nSuggested Reading(s)\nSantillanes G, Rose E. Evaluation and management of dehydration in children. Emerg Med Clin N Am. 2018;36:259–273. doi:10.1016/j.emc.2017.12.004\nMahajan P. Dehydration. In: McInerny TK, Adam HM, Campbell DE, DeWitt TG, Foy JM, Kamat DM, eds. American Academy of Pediatrics Textbook of Pediatric Care. American Academy of Pediatrics; 2021:chap 58. Accessed September 21, 2024. Pediatric Care Online\n\nContent Domain\nFluids and electrolytes\n\nLearning Objectives\nPlan fluid therapy for a patient with acute gastroenteritis unresponsive to oral rehydration\n\nThe correct answer is: oral rehydration with a solution containing 20 g/L of glucose and 75 mEq/L of sodium"}
{"id" : 2080, "question_text" : "A 15-year-old, previously healthy girl presents to the emergency department (ED) with altered mental status. She had gone to sleep the previous night within her usual routine, but did not wake up for school in the morning. Attempts by her mother to arouse her were unsuccessful, so she drove her to the ED. No fever, loss of appetite, exercise intolerance, fatigue, or mental health problems are reported. The girl is fully immunized, and has no known drug allergies. She does not take any medications, and her mother does not think there are any medications in the house. She is a straight A student who plays basketball, volleyball, and the violin. To her mother's knowledge, the girl does not go to parties, has not been dating, and is not sexually active. In the ED, her vital signs are as follows: temperature, 40.2°C; heart rate, 156 beats/minute; respiratory rate, 22 breaths/min; and blood pressure, 150/90 mm Hg. On physical examination, the girl is obtunded, with episodes of agitation including thrashing of the head and all extremities. With painful stimuli, she opens her eyes, moans, and stiffens her upper and lower extremities. Cough and gag reflexes are normal. Her pupils are 8 mm and minimally reactive. Mucous membranes are dry. There is neck stiffness and markedly increased tone of the upper and lower extremities. Deep tendon reflexes are 3/4 throughout. Her breathing is slightly rapid and deep, lungs are clear to auscultation, and her heart sounds are rapid with a grade II/VI systolic ejection murmur. Her abdomen is soft, nontender, and nondistended, extremities are warm with flash capillary refill, and her skin is diffusely erythematous with no rashes. You administer empiric antibiotic therapy.\n\nOf the following, the BEST next step in management is intravenous", "options" : "[\"fomepizole\", \"haloperidol\", \"lorazepam\", \"naloxone\"]", "explanation" : "Correct Answer: C\nThe girl in the vignette has an anticholinergic toxidrome, indicated by altered mental status, fever, tachycardia, hypertension, dry mucous membranes, and rigidity. Of the answer choices, the best next step in management is intravenous lorazepam.\n\nAcetylcholine is a neurotransmitter active in the central nervous system, autonomic nervous system, and neuromuscular junction. Anticholinergic agents are competitive antagonists of acetylcholine receptors. Although acetylcholine binds to both nicotinic and muscarinic receptors, most anticholinergic agents primarily block muscarinic receptors. The wide distribution of the subtypes of muscarinic receptors contributes to the heterogenous presentation of the anticholinergic toxidrome. A convenient mnemonic for the anticholinergic toxidrome is \"mad as a hatter, blind as a bat, hot as a hare, dry as a bone, and red as a beet.\" Altered mental status manifesting as coma, delirium, or agitation can occur as a result of decreased central neurotransmission. Visual and/or auditory hallucinations are common. Impaired autonomic nervous system function from acetylcholine inhibition can cause dilated and poorly reactive pupils, anhidrosis, dry mouth, blurry vision, and photophobia. Dry skin, muscle rigidity from central disinhibition, and autonomic instability can lead to tachycardia and hyperthermia. Hypertension may also occur due to agitation. Drugs causing primarily antimuscarinic effects include atropine, scopolamine, and benztropine. Some anticholinergic agents may also affect other ion channels or receptors. For example, cardiotoxicity from tricyclic antidepressants, antihistamines, and some antipsychotics can cause hypotension, QT prolongation, and malignant arrhythmias.\n\nThe differential diagnosis for the anticholinergic toxidrome includes central nervous system infection, primary acute psychiatric conditions, and drug withdrawal. It must be distinguished from other poisonings, such as alcohol, sympathomimetics, and serotonin syndrome (Item C282). Serotonin syndrome, which can also cause muscle rigidity, hyperpyrexia, hyperreflexia, and central nervous system depression, can be distinguished from anticholinergic toxidrome by a predominance of gastrointestinal symptoms such as nausea, vomiting, and diarrhea.\n\nBenzodiazepines are a mainstay of treatment for anticholinergic poisoning. Sedation can mitigate agitation, delirium, rigidity, hypertension, and tachycardia, and secondary hyperpyrexia can improve. A toxicology screen is important to rule out concomitant poisonings. Supportive care, such as cardiopulmonary monitoring, should be provided.\n\nNaloxone, an opioid antagonist used to treat narcotic toxicity, would not be the appropriate treatment for the girl in the vignette, because she does not have symptoms consistent with narcotic toxicity, such as respiratory depression, hypotension, or constricted pupils. Fomepizole is an alcohol dehydrogenase inhibitor used to decrease the toxic metabolites of ethanol, methanol, and ethylene glycol. Haloperidol is used to treat acute psychosis. These medications would not be appropriate in this case, because the girl's symptoms of rigidity, dilated and poorly reactive pupils, and hyperpyrexia are not commonly seen in alcohol poisoning or acute psychosis.\n\nPREP Pearls\n\nA convenient mnemonic for the anticholinergic toxidrome is \"mad as a hatter, blind as a bat, hot as a hare, dry as a bone, and red as a beet.\"\n\nSedation with benzodiazepines is a mainstay of therapy for anticholinergic toxidrome, because it can mitigate agitation, delirium, rigidity, hypertension, and tachycardia, and secondarily hyperpyrexia may improve.\n\nThe differential diagnosis for the anticholinergic toxidrome includes central nervous system infection, primary acute psychiatric conditions, and drug withdrawal.\n\nABP Content Specifications(s)/Content Area\n\nRecognize the signs and symptoms of ingestion of an anticholinergic drug, and manage appropriately\n\nSuggested Readings\n\nDawson AH, Buckley NA. Pharmacological management of anticholinergic delirium—theory, evidence and practice. Br J Clin Pharmacol. 2016;81:516-524. doi: 10.1111/bcp.12839.\n\nGerardi DM, Murphy TK, Toufexis M, et al. Serotonergic or anticholinergic toxidrome case report in a 9 year-old girl. Pediatr Emerg Care. 2015;31(12):846-850. doi: 10.1097/PEC.0000000000000515.\n\nToce MS, Burns MM. The poisoned pediatric patient. Pediatr Rev. 2017;38:207. doi: 10.1542/pir.2016-0130."}
{"id" : 3218, "question_text" : "The parents of a 17-year-old college student in your practice call to ask your advice. The student awoke this morning to find a bat flying around his dormitory room. He and his roommate opened the window and the bat flew out. He feels well and on self-examination noticed no skin lesions or bite marks. His immunizations are up to date, including receipt of diphtheria-tetanus-pertussis vaccine at 11 years of age. Of the following, the BEST course of action is to", "options" : "[\"begin a 5-day course of amoxicillin-clavulanic acid\", \"begin a rabies vaccine series\", \"get a tetanus vaccine booster\", \"monitor his skin for signs of new skin lesions over the next 2 weeks\", \"provide reassurance\"]", "explanation" : "Preferred Response: B\nAlthough rabies virus infection is rare in the United States, the vast majority of indigenously acquired rabies in the United States is from bat exposure. A scratch or bite from a bat may not be readily apparent. In settings in which the contact is unknown, such as an unattended child in a room with a bat or, as in this vignette, sleeping in a room in which a bat is found, potential rabies exposure must be considered. If the animal is captured, it can be euthanized and its brain examined for rabies virus. If not (or if the brain examination is positive), institution of rabies prophylaxis is recommended. Present guidelines call for a combination of human rabies immune globulin (HRIG) and vaccine given on day 0 and subsequent doses of rabies vaccine on days 3, 7, and 14. If apparent, infiltration with as much of the HRIG as possible around the bite site is recommended. The remainder of the dose is given intramuscularly (IM). The vaccine is administered IM in the deltoid or anterior aspect of the thigh. Once signs or symptoms of rabies appear, HRIG and vaccine are not of benefit.\n\nAll mammals are potentially at risk for acquiring rabies with bats, raccoons, skunks, foxes, coyotes, and bobcats being the primary potential sources for human and animal exposure in the United States. Lagamorphs (eg, rabbits, hares) and rodents (eg, squirrels, hamsters, guinea pigs, gerbils, rats, mice) rarely transmit rabies.\n\nSecondary bacterial infection maybe a concern with larger animal bites, but is not a significant risk from a bat exposure, as reported in this vignette, making administration of antibiotics unnecessary. Similarly, assessing the need for tetanus prophylaxis may be indicated after a dirty wound from a larger animal, but not with a bat exposure with no visible lesion. Local reactions are not a hallmark of bat bites such that monitoring for skin lesions over time is of no clinical utility. Reassurance would not be appropriate here because, as noted previously, finding a bat in a room with someone who has been sleeping there is considered adequate exposure to rabies and prophylaxis as outlined above is indicated.\n\nPREP Pearls\n• Bat exposure is the leading cause of potential transmission of rabies in the United States. Bat bites are small and may not be felt in the setting of an unattended child or someone asleep in a room in which a bat is found.\n• Rabies prophylaxis consists of a dose of human rabies immune globulin and rabies vaccine on day 0 and additional doses of vaccine on days 3, 7, and 14.\n• All mammals may become infected with rabies with bats, raccoons, skunks, foxes, coyotes, and bobcats as the leading sources in the United States. Lagamorphs (eg, rabbits, hares) and rodents rarely transmit rabies.\n\nSuggested Reading\n• American Academy of Pediatrics. Rabies. In: Pickering LK, Baker CJ, Kimberlin DW, Long SS, eds. Red Book: 2012 Report of the Committee on Infectious Diseases. 29th ed. Elk Grove Village. IL: American Academy of Pediatrics; 2012:600-607.\n• Rupprecht CE, Briggs D, Brown CM, et al. Use of a reduced (4-dose) vaccine schedule for postexposure prophylaxis to prevent human rabies. Recommendations of the Advisory Committee on Immunization Practices. Mortal Morbid Weekly Rep. 2010;59(RR-2):1-12. Updated March 19, 2010. Accessed February 24, 2014."}
{"id" : 3744, "question_text" : "A 10-year-old African American boy with sickle cell anemia is admitted to the hospital for intravenous fluids and pain control because of dehydration and acute pain crisis. After 12 hours in the hospital he is found to be acutely unresponsive. Naloxone is administered with no change in his clinical status, and he is emergently transferred to the pediatric intensive care unit. He has a temperature of 38.1°C, heart rate of 160 beats/min, respiratory rate of 16 breaths/min, and blood pressure of 60/30 mm Hg. His general physical examination is notable for a rigid neck, tacky mucous membranes, and tachycardia with a flow murmur. His lungs are clear. He is obtunded and groans to painful stimuli without localizing it. His pupils are equal, round, and reactive to light, with intact extraocular movements and symmetric facies. He moves all extremities equally to noxious stimuli. Deep tendon reflexes are 2+ throughout with toes downgoing to plantar stimulation. Because of his deteriorating condition, he is intubated for airway protection, begun on fluid resuscitation, and placed on broad-spectrum antibiotics after obtaining blood cultures. Noncontrast computed tomography of the head performed as part of a stroke evaluation has normal results. Lumbar puncture is performed with an opening pressure of 61 cm H2O. The cerebrospinal fluid is cloudy, and laboratory data from its analysis are shown: White blood cell count 1,300/µL, Neutrophils 70%, Lymphocytes 25%, Monocytes 5%, Red blood cell count 0/µL, Protein 145 mg/dL, Glucose 10 mg/dL (0.6 mmol/L). Of the following, the MOST likely cause of this child's meningitis is", "options" : "[\"Haemophilus influenzae\", \"herpes simplex virus\", \"Salmonella\", \"Streptococcus pneumoniae\"]", "explanation" : "Correct Answer: D\nThe patient in this vignette has bacterial meningitis most likely caused by Streptococcus pneumoniae. Children with sickle cell disease are predisposed to invasive bacterial infections from encapsulated organisms such as S pneumoniae, Haemophilus influenzae type b (Hib), and nontyphoidal Salmonella due to functional asplenia, which results in diminished antibody-mediated phagocytosis. Of these organisms, infection with S pneumoniae occurs most frequently. Clinical manifestations of encapsulated bacteria vary in severity in both healthy and immunocompromised hosts and include bacteremia, pneumonia, otitis media, upper respiratory tract infections, and meningitis. Prophylactic antibiotics and immunization are critical aspects of preventive care in patients with sickle cell disease. However, a high index of suspicion is warranted, despite preventive measures, because these invasive bacterial infections can be rapidly progressive with significant morbidity and mortality.\n\nBacterial meningitis is a life-threatening infection that often presents with a relatively rapid onset of signs and symptoms. The condition is clinically characterized by fever, lethargy, and nuchal rigidity progressing to seizures, coma, and signs and symptoms of elevated intracranial pressure. Prompt recognition and initiation of treatment is crucial because the long-term neurological sequelae can be significant. In children suspected of having bacterial meningitis, a rapid assessment focused on stabilization and airway protection should be undertaken, especially when altered mental status is present. At the time of initial presentation, urgent head imaging is warranted if papilledema, focal neurological deficits, immune deficiency, or a pre-existing structural central nervous system condition is present, but imaging can otherwise be deferred. Lumbar puncture should be performed unless there is evidence of increased intracranial pressure, coagulopathy, or hemodynamic instability. Empiric antibiotic therapy should be initiated as quickly as possible, even prior to diagnostic testing if there will be any anticipated delay. Dexamethasone has been used as adjunct therapy to diminish the inflammatory response to the infection and reduce potential long-term sequelae of hearing loss. Although its use is controversial in the pediatric population, the American Academy of Pediatrics Committee on Infectious Disease has noted a potential benefit of dexamethasone in reducing hearing loss in Hib meningitis. If administered, steroids should be given with the initial dose of antibiotics because no benefit has been seen if given more than 1 hour after antibiotics. Caution is recommended in using corticosteroids in patients with sickle cell disease.\n\nCerebrospinal fluid (CSF) analysis is a key diagnostic study that provides critical information on the likely source of infection as well as potential isolation of the pathogen. In bacterial meningitis, the CSF will demonstrate a neutrophilic pleocytosis with an elevated protein level and low glucose level. Antibiotic pretreatment reduces the likelihood of isolating a pathogen on culture, however it will not affect the CSF cell counts or identification of the organism by molecular methods and is unlikely to obscure the diagnosis.\n\nHaemophilus influenzae is an encapsulated organism that is a common cause of bacterial meningitis in children and can have a similar presentation to S pneumoniae, making it difficult to differentiate solely on a clinical basis. While the patient in the vignette is at risk for both, S pneumoniae is the more likely bacterial pathogen to cause childhood meningitis since the introduction of Hib vaccination. Herpes simplex virus can result in a devastating meningoencephalitis with a predilection for the temporal lobes, which often presents with rapid development of headache, lethargy, refractory seizures, and personality changes. Magnetic resonance imaging can demonstrate flair hyperintensities in the bilateral temporal lobes, with electroencephalography showing periodic lateralizing epileptiform discharges. Cerebrospinal fluid analysis demonstrates a lymphocytic pleocytosis with xanthochromia and identification of the virus by polymerase chain reaction testing. Acyclovir is the antiviral agent of choice. Children with sickle cell disease remain at risk for Salmonella, which typically causes gastroenteritis and bacteremia but in severe cases can result in meningitis and other invasive bacterial infections. Invasive disease is more commonly seen in infants younger than 3 months.\n\nIn addition to early empiric broad-spectrum antibiotic treatment, management of bacterial meningitis is supportive and is frequently provided in the intensive care unit setting. Children should be closely monitored for decline in neurological status, the development of seizures, syndrome of inappropriate secretion of antidiuretic hormone, Cushing's triad, and cerebral infarction. Up to one-third of children with bacterial meningitis develop subdural effusions that do not require surgical intervention unless they develop into subdural empyema. Prognosis varies with pathogen and certain clinical features. Poorer prognosis is suggested by a younger age, large bacterial burden, decreased level of consciousness at presentation, and infection with S pneumoniae. Mortality rates range from 5% to 15%. Long-term neurological sequelae in survivors can include hearing impairment (in particular with S pneumoniae and Hib), vestibular injury, developmental delay, epilepsy, hydrocephalus, hemi- or quadriparesis, hypothalamic dysfunction, or cortical blindness. Recovery occurs over weeks to months and requires supportive rehabilitative and educational services as well as close long-term follow up.\n\nPREP Pearls\n• Children with sickle cell disease are predisposed to invasive bacterial infections from encapsulated organisms, such as Streptococcus pneumoniae, that can be rapidly progressive if unrecognized.\n• Bacterial meningitis is a life-threatening, rapidly progressive, neurological emergency that presents with fever, lethargy, nuchal rigidity, and headache. Even with early treatment, it carries high morbidity and mortality.\n\nABP Content Specifications(s)\n• Plan the appropriate diagnostic evaluation of meningitis of various etiologies\n• Recognize the clinical findings associated with meningitis and manage appropriately\n• Understand the etiology of meningitis in patients of various ages\n\nSuggested Readings\n• Curtis S, Stobart K, Vandermeer B, Simel DL, Klassen T. Clinical features suggestive of meningitis in children: a systematic review of prospective data. Pediatrics. 2010;126(5):952-960. doi:10.1542/peds.2010-0277.\n• Maraqa N. Pneumococcal infections. Pediatr Rev. 2014;35(7):299-310. doi:10.1542/pir.35-7-299.\n• Swanson D. Meningitis. Pediatr Rev. 2015;36(12):514-524. doi:10.1542/pir.36-12-514."}
{"id" : 2018, "question_text" : "A full-term female neonate is being evaluated in the well-child nursery. She was born to a 22-year-old gravida 1, para 0 woman via normal spontaneous vaginal delivery. Her Apgar scores were 8 and 9 at 1 and 5 minutes, respectively; birthweight was 2,700 g. The mother had adequate prenatal care, and there were no concerning findings on prenatal or perinatal screening tests. The neonate has been attempting to breastfeed, and she has not latched on successfully. At 48 hours after birth, her weight had decreased to 2,450 g. Physical examination findings were normal, with the exception of generalized jaundice. Her serum bilirubin level at that time was 16.2 mg/dL (277 μmol/L), and phototherapy was started. Today, 72 hours after birth, the infant appears less vigorous and is not interested in feeding. Her weight is 2,200 g, temperature is 37°C, heart rate is 170 beats/min, respiratory rate is 36 breaths/min, and blood pressure is 60/30 mm Hg. On physical examination today, the infant is sleepy and difficult to arouse; however, with noxious stimuli the infant cries normally. Her tone is normal, mucous membranes are dry, and she appears icteric. Her breathing is normal, and lungs are clear with good aeration. Heart sounds are regular, with a II/VI systolic ejection murmur. There is significant skin tenting and her extremities are cool, with 4-second capillary refill time.\n\nOf the following, the BEST next step in management is to", "options" : "[\"administer a bolus of 0.9% normal saline, 20 mL/kg, intravenously\", \"administer 0.2% normal saline at 16 mL/hour, intravenously\", \"offer formula orally\", \"perform a whole blood exchange transfusion\"]", "explanation" : "The neonate in the vignette has hypovolemic shock as evidenced by tachycardia, hypotension, and poor peripheral perfusion. The best initial step in management is a 20 mL/kg bolus of normal saline.\n\nShock is a clinical condition in which the cardiac output and oxygen delivery are insufficient to meet the metabolic demands of end organs. It is further classified as \"hypovolemic shock,\" when the primary cause is decreased stroke volume because of insufficient intravascular volume. It is important for the clinician to distinguish hypovolemic shock from simple dehydration. The neonate in the vignette presents with several important signs and symptoms consistent with hypovolemic shock including severe tachycardia, delayed capillary refilling time, and altered mental status. She is also hypotensive, which implies a higher severity of shock. It is important to realize that hypotension is not always present in shock; compensatory mechanisms for shock that can prevent hypotension include elevated circulating catecholamines, tachycardia, vasoconstriction, and diversion of blood away from the skin and splanchnic circulation.\n\nCardiac output is the product of stroke volume and heart rate. Stroke volume is dependent on preload, afterload, and contractility. Children, compared with adults, are more likely to respond with an increased heart rate in case of shock rather than an increase in contractility. Thus, tachycardia is an important compensatory mechanism for shock. Fluid administration is an important early therapy in hypovolemic shock, as an underfilled ventricle contains less blood to eject. Also, contractile elements will overlap, leading to a lower fraction of the blood ejected. Early goal-directed resuscitation has been shown to improve outcomes in neonatal, pediatric, and adult shock (Item C218A). It begins with the recognition of decreased mental status and perfusion. Intravenous or intraosseous access should be established within the first few minutes, and starting within the first 15 minutes, isotonic fluid boluses of 20 mL/kg should be pushed up to and over 60 mL/kg, until perfusion improves or rales or hepatomegaly occur. Empiric antibiotics should also be administered early. Inotropes should be initiated for fluid-refractory shock, and corticosteroids should be considered for catecholamine-resistant shock. Neonates can be less able to handle the fluid load during resuscitation compared with older children. Thus, earlier initiation of inotropes may be warranted.\n\nItem C218A: Algorithm for goal-directed management of hemodynamic support in septic shock. Adapted from the 2007 ACCM clinical practice parameters for hemodynamic support of pediatric and neonatal septic shock. Abbreviations: AI=adrenal insufficiency; BP=blood pressure; CI=cardiac index; CVP=central venous pressure; DA=dopamine; Dobut=dobutamine; ECMO=extracorporeal membrane oxygenation; Epi=epinephrine; IV=intravenous; iNO=inhaled nitric oxide; IO=intraosseous; LV=left ventricle; NL=normal; PGE1=prostaglandin E1; RV=right ventricle; ScvO2=mixed venous oxygen saturation; UVC=umbilical venous catheter. Reprinted with permission from Yager P, Noviski N. Shock. Pediatr Rev. 2010;31(8):315.\n\nOther causes of shock should be considered in neonates (Item C218B). Septic shock can be caused by early-onset sepsis. Dissociative shock can be caused by an inborn error of metabolism or a mitochondrial disorder. Cardiogenic shock is caused by inadequate myocardial function or structural heart disease; the physical examination is notable for pulmonary edema, rales, and hepatomegaly. Ductal-dependent systemic blood flow lesions such as hypoplastic left heart syndrome, critical coarctation of the aorta, and critical aortic stenosis can present with shock within the first 48 hours after birth, when the ductus arteriosus closes. All neonates in shock should be started on a prostaglandin infusion unless a definitive noncardiac diagnosis has been made.\n\nOffering formula or expressed breast milk by bottle or providing maintenance intravenous fluids are good management steps for simple dehydration, but would not be appropriate to treat a neonate in shock. Whole blood exchange transfusion has been effective in some forms of refractory septic shock, but it is not helpful in hypovolemic shock.\n\nPREP Pearls\n\nEarly goal-directed therapy aimed toward restoring cardiac output should be immediately initiated in all patients with shock.\n\nAll neonates in shock should be started on a prostaglandin infusion, unless a definitive noncardiac diagnosis has been made.\n\nHypotension is a late finding in pediatric shock; tachycardia is an early finding.\n\nABP Content Specifications(s)/Content Area\n\nRecognize the clinical signs of shock due to fluid loss, and manage appropriately\n\nSuggested Readings\n\nBhat BV, Plakkal N. Management of shock in neonates. Indian J Pediatr. 2015;82(10):923-929. doi: 10.1007/s12098-015-1758-7.\n\nDavis AL, Carcillo JA, Aneja RK, et al. American College of Critical Care Medicine clinical practice parameters for hemodynamic support of pediatric and neonatal septic shock. Crit Care Med. 2017;45(6):1061-1093. doi: 10.1097/CCM.0000000000002425.\n\nYager P, Noviski N. Shock. Pediatr Rev. 2010; 31:311-319. doi: 10.1542/pir.31-8-311."}
{"id" : 3303, "question_text" : "An 18-year-old man presents to your clinic for testicular swelling. He also reports dull lower abdominal pain for the past few weeks. He has been sexually active with 3 female partners in his lifetime and reports that he uses condoms most of the time. He denies any weight loss or fever. On physical examination, the right testicle appears noticeably larger than the left with no discoloration or tenderness. The remainder of the physical examination is unremarkable. You order testicular ultrasonography, which reveals a well-defined, noncystic solid mass in the right testicle. Of the following, the MOST appropriate next test in the evaluation of this mass is", "options" : "[\"bone radionuclide scan\", \"carcinoembryonic antigen level\", \"computed tomography scan\", \"needle biopsy\", \"positron emission tomography scan\"]", "explanation" : "Preferred Response: C\nThe patient in the vignette should have a computed tomography (CT) scan to evaluate the testicular mass seen on ultrasonography and for abnormalities in the abdominal lymph nodes. The greatest risk factor for the development of testicular carcinoma is an undescended testicle that remains uncorrected beyond 1 year of age with an estimated 10- to 50-fold higher risk of cancer. In prepubertal boys, the tumors are usually yolk sac tumors, gonadoblastomas, or teratomas, whereas in adolescents, embryonal carcinoma and teratocarcinoma are more common. Most testicular tumors present as a painless scrotal mass. The lack of symptoms may lead to a delay in seeking medical attention. A reactive hydrocele or an inguinal hernia may be present at the time of diagnosis. Fortunately, the majority of pediatric testicular tumors are localized, however, metastasis can occur most commonly to the lung and the lymph nodes of the retroperitoneum.\n\nAfter initial evaluation of the suspected mass by testicular ultrasonography, a CT scan should be performed to visualize tumor margins and to evaluate the chest and the lymph nodes in the abdomen. Bone radionuclide scan and positron emission tomography scan are used to look for areas in the bone and lymph nodes (respectively) with increased metabolic activity suggestive of inflammation, infection, or malignancy; however, this would not be indicated in the initial evaluation of suspected testicular cancer.\n\nCarcinoembryonic antigen levels are not generally elevated in the various types of testicular tumors, therefore this would not be the correct tumor maker. a-fetoprotein (elevated in yolk sac tumors and embryonal carcinomas) and 13-human choriogonadotropin (sometimes elevated in embryonal carcinomas) may be useful tumor markers for certain types of testicular cancer. A needle biopsy should not be performed due to the potential for tumor seeding, which can lead to cancer recurrence; therefore, radical inguinal orchiectomy with en bloc excision of the spermatic cord and testicles is recommended for diagnosis and treatment. Depending on the stage, multi-agent chemotherapy may be indicated.\n\nPREP Pearls\n• The greatest risk factor for the development of testicular carcinoma is an undescended testicle that remains uncorrected beyond 1 year of age with an estimated 10- to 50-fold higher risk of cancer.\n• Most testicular tumors present as a painless scrotal mass. There may be a reactive hydrocele or an inguinal hernia present at the time of diagnosis.\n• A needle biopsy of a testicular mass should not be performed due to the potential for tumor seeding, which can lead to cancer recurrence, therefore radical inguinal orchiectomy with en bloc excision of the spermatic cord and testicles is recommended for diagnosis and treatment.\n• After initial evaluation of the suspected mass by testicular ultrasonography, a computed tomography scan should be performed to visualize tumor margins and to evaluate the lungs and the lymph nodes in the abdomen.\n• α-fetoprotein and β-human choriogonadotropin may be useful tumor markers for certain types of testicular cancer.\n\nAmerican Board of Pediatrics Content Specification(s)\n• Plan the appropriate evaluation of a testicular mass\n• Recognize risk factors associated with testicular cancer\n• Recognize the laboratory findings associated with a germ cell tumor\n\nSuggested Reading\n• Chan E, Wayne C, Nasr A; FRCSC for the Canadian Association of Pediatric Surgeon Evidence-Based Resource. Ideal timing of orchiopexy: a systematic review, Pediatr Slag Int. 2014;30(1):87-97. doi:10,10071s00383- 013-34291.\n• Nallu A, Mannuel HD, Hussain A. Testicular germ cell tumors: biology and clinical update. Corr Opin Oncoe. 2013;25(3)166-272. doi:10.1097I CC0.0b013e32835fF3e3.\n• Vasdev N, Moon A, Thorpe AC. Classification, epidemiology and therapies for testicular germ cell tumours. Int f Dev Biot. 2013;57(2-0133-9. doi:10.1387/ndb.I30031nv."}
{"id" : 2220, "question_text" : "During a health supervision visit, a 12-year-old is noted to have poor growth over the past year. Since her last visit, she has started following a vegan diet due to concerns for animal cruelty. She has tried to maintain adequate calorie intake and eat a well-rounded diet. The girl is concerned about recent hair loss and diarrhea but reports she has otherwise been feeling well. Of the following, the girl's findings are are MOST likely due to a deficiency of", "options" : "[\"copper\", \"magnesium\", \"selenium\", \"Zinc\"]", "explanation" : "Symptoms of zinc deficiency include hair loss, diarrhea, and poor growth.\n\nIndividuals following a gluten-free diet are at increased risk for selenium and magnesium deficiencies.\n\nSeveral mineral deficiencies are associated with a ketogenic diet, including magnesium, copper, zinc, and selenium.\n\nCritique\nThe girl described in the vignette most likely has zinc deficiency. Symptoms of zinc deficiency include hair loss, diarrhea, and poor growth. The number of individuals following restrictive diets without medical requirements is increasing due to various personal and society goals. Among these are gluten free, vegetarian, and ketogenic diets. Some restrictive diets, especially without involvement of a dietician, can lead to mineral deficiencies. Copper deficiency is not associated with hair loss or diarrhea, and it is not common with a vegetarian diet. Magnesium deficiency does not cause diarrhea, and although hair loss can be seen, it is not associated with a vegan diet. Selenium deficiency can be associated with hair loss and diarrhea, but it is not common in those following a vegan diet.\n\nAbout 1% to 2% of the population is following a form of a vegetarian diet with restriction of meat and animal products. Some vegetables (eg, beets, sweet potatoes) have high levels of oxalates and phytates, which bind minerals including calcium, iron, and zinc. This can lead to decreased absorption of these minerals from the gastrointestinal tract. As a result, many individuals following a vegetarian diet have lower levels of zinc and iron. The evidence is unclear as to whether there is an increased risk of symptomatic zinc deficiency when following a vegetarian diet. It is also unclear if there is a link between following a vegetarian diet and microcytic anemia associated with iron deficiency.\n\nGluten-free diets are medically indicated for individuals with celiac disease. People with certain allergies (eg, wheat, barley) may require avoidance of similar foods. With a gluten-free diet, there is a known increased risk for selenium and magnesium deficiencies. There is also decreased consumption of iron and zinc; currently, there is no clear evidence regarding whether symptomatic deficiencies are common.\n\nThe ketogenic diet was initially developed in the 1920s to treat epilepsy, prior to antiepileptic medications. The diet is now used as an adjunct for individuals with refractory epilepsy, as well as for the management of individuals with pyruvate dehydrogenase deficiency and glucose transporter type 1 deficiency. Some individuals use the ketogenic diet as a weight-loss tool. The diet has a high lipid-to-nonlipid ratio, traditionally 4:1 or 3:1, with about 90% of calories from fats. Some nonmedically indicated versions of the ketogenic diet use lower ratios, such as diets with 60% fats, 10% carbohydrates, and 30% proteins. Several mineral deficiencies are associated with this restrictive diet, including magnesium, copper, zinc, and selenium. Zinc deficiency can lead to low levels of alkaline phosphatase. Copper deficiency has been associated with neutropenia. Selenium, typically found in meats and grains, is critical to forming over 25 proteins within the body and may be protective against some forms of cancer. Keshan disease, a severe form of cardiomyopathy, can be caused by selenium deficiency. Supplementation of selenium stops the progression of the inflammatory process and tissue damage, but the damage incurred is not reversible.\n\nSuggested Reading(s)\nAndrewski E, Cheng K, Vanderpool C. Nutritional deficiencies in vegetarian, gluten-free, and ketogenic diets. Pediatr Rev. 2022;43(2):61–70. doi:10.1542/pir.2020-004275\nKim JH. Providing optimal nutrition to very low birthweight infants in the NICU. Neoreviews. 2023;24(5):e271–e284. doi:10.1542/neo.24-5-e271\nLucas A, Sherman J, Fewtrell M. Postdischarge nutrition in preterm infants. Neoreviews. 2022;23(8):e541–e557. doi:10.1542/neo.23-8-e437\n\nContent Domain\nNutrition\n\nLearning Objectives\nRecognize signs and symptoms related to a zinc deficiency"}
{"id" : 1154, "question_text" : "A 3-week-old, previously healthy full-term male newborn is brought to your office for evaluation of a rash on the bottom of his feet that began a few days ago. The mother has no other concerns. On physical examination, the newborn is afebrile with normal vital signs. The anterior fontanel is soft, open, and flat. There is minimal clear rhinorrhea. The liver and spleen are palpable 2 cm and 1 cm below the right and left costal margins, respectively. There are numerous copper-colored, circular lesions, each less than 0.5 cm in diameter on the plantar surfaces of both feet (Item Q75). The remainder of the physical examination is unremarkable.\nOf the following, the MOST likely cause of the newborn's rash is", "options" : "[\"Candida albicans\", \"coxsackievirus\", \"cytomegalovirus\", \"Streptococcus agalactiae\", \"Treponema pallidum\"]", "explanation" : "The infant in the vignette has a copper-colored rash on the soles of the feet and hepatosplenomegaly consistent with congenital infection with Treponema pallidum (syphilis). Pregnant women typically are tested for syphilis with screening nontreponemal serologic tests (rapid plasma reagin [RPR]; VDRL) early in pregnancy, at the time of delivery, and occasionally, at the beginning of the third trimester. However, if this testing does not occur, an infant can present with symptomatic infection in the weeks after birth. Intrauterine infection with T pallidum can result in stillbirth, preterm birth, hydrops fetalis, or asymptomatic infection. Infants such as the child described in the vignette, can have:\n\nhepatosplenomegaly\n\ncopious nasal secretions (snuffles)\n\ncutaneous lesions\n\nedema\n\nlymphadenopathy\n\nosteochondritis\n\npneumonia\n\npseudoparalysis\n\nhemolytic anemia\n\nthrombocytopenia\n\nAn untreated intrauterine infection can affect the:\n\ncentral nervous system (eighth cranial nerve deafness)\n\neyes (interstitial keratitis)\n\nteeth (peg-shaped incisors [Hutchinson teeth], mulberry molars)\n\nbones (frontal bossing, saddle nose, tibial bowing)\n\njoints (swelling of knees [Clutton joints])\n\nskin (ulceration, desquamation, palpable lesions)\n\nAcquired syphilis occurs in 3 stages: primary, secondary, and tertiary. The primary stage of infection is characterized by 1 or more painless ulcers (chancres) on the skin or mucous membranes at the initial site of inoculation that develop approximately 3 weeks after exposure. These lesions will spontaneously heal after a few weeks and can go undetected. The secondary stage of syphilis is characterized by lymphadenopathy, mucocutaneous lesions, and rash. The rash is generalized and typically involves the palms and soles. Patients may experience flulike symptoms such as fever, headache, sore throat, arthralgias, and malaise. This stage will spontaneously resolve in 1 to 4 months without treatment. The period following the secondary stage is called the latent period during which time patients are asymptomatic and seroreactive but may suffer recurrences of secondary stage symptoms. The tertiary stage of syphilis occurs 15 to 30 years after initial infection and can include neurosyphilis, cardiovascular symptoms, and gumma formation.\nPenicillin is the treatment of choice for congenital or acquired infection. For patients with penicillin allergy and neurosyphilis, congenital syphilis, syphilis during pregnancy, or human immunodeficiency virus infection, desensitization is recommended. The recommended evaluation and treatment of neonates exposed to mothers infected with T pallidum is outlined in Item C75A. The recommended treatment for syphilis in patients older than 1 month of age is displayed in Item C75B.\nMucocutaneous infection caused by Candida in the neonate can involve the oropharynx (eg, thrush) or vagina, digits and nails, and the intertriginous areas in the groin, axillae, and neck. It also can cause a mild but diffuse cutaneous infection (Item C75C). Cutaneous infection caused by Candida typically is described as an erythematous rash with satellite lesions; it typically would not be isolated to the feet as described for the patient in the vignette. Coxsackievirus can cause a nonspecific viral exanthem or hand, foot, and mouth disease characterized by painful red blisters on the affected areas (Item C75D); it would not cause macular copper-colored lesions as described for the infant in the vignette. Similarly, acquired cytomegalovirus infection could present with a diffuse, nonspecific rash but not with localized findings. The classic \"blueberry muffin\" rash of congenital cytomegalovirus infection (Item C75E) would be present at or shortly after birth and not develop in the third week after birth as described for the patient in the vignette. Lastly, late-onset infection caused by group B Streptococcus (S agalactiae) may present as cellulitis but discrete copper-colored macular lesions are not characteristic findings.\n\nPREP Pearls\n\nIntrauterine infection with Treponema pallidum can result in stillbirth, preterm birth, hydrops fetalis, or asymptomatic infection.\n\nManifestations of congenital syphilis include\n\nhepatosplenomegaly\n\ncopious nasal secretions (snuffles)\n\ncutaneous lesions\n\nedema\n\nlymphadenopathy\n\nosteochondritis\n\npneumonia\n\npseudoparalysis\n\nhemolytic anemia\n\nthrombocytopenia\n\nUntreated intrauterine infection can affect the\n\ncentral nervous system (eighth cranial nerve deafness)\n\neyes (interstitial keratitis)\n\nteeth (peg-shaped incisors [Hutchinson teeth], mulberry molars)\n\nbones (frontal bossing, saddle nose, tibial bowing)\n\njoints (swelling of knees [Clutton joints])\n\nskin (ulceration, desquamation, palpable lesions)\n\nThe treatment of choice for all stages of syphilis is parenteral penicillin.\n\nABP Content Specifications(s)\n\nRecognize the clinical features associated with congenital and acquired Treponema pallidum infection\n\nPlan appropriate management for a patient with Treponema pallidum infection"}
{"id" : 889, "question_text" : "You are evaluating a 15-year-old boy in the emergency department who complains of a severe headache of several weeks' duration; the pain does not change throughout the day. He does not report any previous medical problems but, on review of systems, states that he has had some \"blurry vision\" over the past week. His physical examination is remarkable only for a blood pressure of 195/130 mm Hg and papilledema. You plan the boy's evaluation and elect to begin treatment. Of the following, the MOST appropriate medication to minister via continuous infusion is", "options" : "[\"diazoxide\", \"fenoldopam\", \"hydralazine\", \"labetalol\", \"phentolamine\"]", "explanation" : "Hypertension, systolic or diastolic blood pressure greater than the 95th percentile on 3 or more occasions, occurs in approximately 5% of the pediatric population. Pediatric hypertensive emergencies, defined as a severe symptomatic elevation in blood pressure with evidence of acute end organ damage, are rare (less than 1% of emergency department visits). Hypertensive emergencies can be seen in newly diagnosed patients as well as those with chronic hypertension who have an acute elevation. No specific systolic or diastolic blood pressure defines an emergency, and therefore it is imperative to recognize the end organ dysfunction to quickly treat the hypertension and prevent morbidity and mortality. The most commonly affected end organs are the brain (altered mental status, seizures, edema, increased intracranial pressure), kidneys (renal insufficiency), heart (heart failure), and eyes (papilledema, retinal hemorrhages). Hypertensive encephalopathy (altered mental status or seizures) is the most common manifestation of a hypertensive emergency and has been reported in up to 50% of cases.\n\nAssessment of hypertensive emergencies should include a thorough history and physical examination to rule out potential causes (head trauma, intracranial masses, abdominal tumors, hyperthyroidism, etc). The physical examination should include a 4-extremity blood pressure measurement to assess for a possible aortic coarctation. Risk factors for hypertensive emergencies include chronic hypertension (especially those who are not compliant with medications), chronic kidney disease, genitourinary abnormalities, renal vascular disease, acute glomerulonephritis, illicit drug use, pheochromocytoma, and pregnancy. Ancillary studies should include electrolyte and renal function measurement to assess for renal disease as well as chest radiography and electrocardiography to evaluate for cardiac hypertrophy. Other studies that might be performed based on results of the history and physical examination include complete blood count, urine toxicology screen, pregnancy test, echocardiography, abdominal ultrasonography, and computed tomography of the head.\n\nIntravenous antihypertensive medications should be started as soon as the emergency is recognized, with the goal of lowering the blood pressure to a level that will stop or at least mitigate the end organ damage. In general, lowering the blood pressure by approximately 20% to 25% over the first 8 hours will achieve this goal. Further lowering of the blood pressure may produce end organ damage because of lack of adequate perfusion secondary to altered autoregulation. The selection of the appropriate agent will depend on the suspected underlying cause but in general labetalol (a β-blocker) or nicardipine (a calcium channel blocker) are generally used as first-line agents because of their proven efficacy and their ability to be given in continuous infusions after an initial bolus.\n\nHydralazine, a direct arterial smooth muscle dilator, has been used as a first-line agent but has a slower onset of action and longer duration, and cannot be used as a continuous infusion. Phentolamine, an α-adrenergic blocker, would be indicated in the setting of excessive catecholamine levels as might occur with a pheochromocytoma or cocaine overdose. Fenoldopam, a peripheral dopamine receptor agonist, is less potent than either labetalol or nicardipine and, therefore, is not generally recommended for initial treatment. Because of its ability to improve renal perfusion, it may be a useful drug in patients with renal insufficiency. Sodium nitroprusside is no longer generally recommended as a first-line agent because of potential cyanide toxicity. Diazoxide also is no longer recommended as a first-line agent because of its unpredictable blood pressure lowering effect.\n\nPREP Pearls\n• Pediatric hypertensive emergencies, defined as a severe symptomatic elevation in blood pressure with evidence of acute end organ damage, are rare but must be recognized and treated quickly.\n• Hypertensive encephalopathy is the most common manifestation of a hypertensive emergency and has been reported in up to 50% of cases.\n• The goal in the initial treatment of hypertensive emergencies is to lower the blood pressure by approximately 20% to 25% over the first 8 hours to stop or mitigate end organ damage.\n\nAmerican Board of Pediatrics Content Specification(s):\n• Recognize and plan the therapy for a hypertensive emergency\n\nSuggested Reading:\n• Lande MB. Systemic Hypertension. In: Kliegman RM, Stanton BMD, St Geme J, Schor N, Behrman RE, eds. Nelson Textbook of Pediatrics. 19th ed. Philadelphia, PA: Elsevier Saunders; 2011:1639-1647\n• Flynn JT. Management of hypertensive emergencies and urgencies in children. UptoDate. Available online only for subscription\n• Symons J, Enriquez B. Approach to hypertensive emergencies and urgencies in children. UptoDate. Available online only for subscription"}
{"id" : 2307, "question_text" : "A 4-week-old girl is brought to the office by her mother for a scheduled weight check. Her birth history is unremarkable. The mother reported difficulty with formula feeding at her 2-week appointment and today reports that the infant continues to be fussy while feeding as well as crying intermittently throughout the day and night. The mother states, \"I thought she would be sleeping through the night by now.\" The infant lives in a home with her mother, stepfather, and twin brother. The infant's weight has increased appropriately from the last visit. There are skin changes on her left cheek (Figure). On inquiry, the mother reports no history of trauma. Of the following, the BEST next step in this infant's management is to", "options" : "[\"counsel the family regarding age-appropriate infant behavior\", \"make a report to child protective services\", \"order laboratory testing for hematologic disorders\", \"schedule a follow-up visit for the next day\"]", "explanation" : "Critique\nThe infant in the vignette has a bruise on her left cheek. Developmentally, she is preambulatory and likely cannot roll over; thus, she would not be expected to sustain an injury to a soft area of her face with no known trauma history. The bruise pattern resembles a handprint, further heightening the suspicion of abuse.\nPediatricians are mandatory reporters and can be held legally responsible if they fail to report potential child abuse. The best next step in this infant's management is to make a report to child protective services (CPS).\nA patterned slap mark on the face is a suspicious finding in an ambulatory child (Table 1) and even more concerning in a preambulatory infant. The TEN-4-FACES rule is outlined as follows:\nTorso \nEar \nNeck \nYounger than 4 years/infant younger than 4 months \nFrenulum\nAngle of jaw\nCheeks\nEyelids\nSubconjunctivae or sclera \nPatterned bruise\nThe TEN-4-FACES rule can guide pediatricians in determining which bruises are more likely to result from abuse. The infant in the vignette meets criteria for the number 4 and letters C and P. The infant's bruise can be categorized as a sentinel injury, a relatively minor injury that is suggestive of child abuse. Children who experience abuse often experience recurrent trauma; sentinel injuries may be the only indication to investigate for additional trauma and thereby prevent additional harm.\nSuspicion of abuse is all that is required to make a CPS report. Physicians do not need to prove certainty or intent, and they are not responsible for determining who committed the abuse. Consultation with subspecialists, including child abuse pediatricians, is encouraged when evaluation and management are uncertain and further guidance is needed. Depending on the severity of injury or situational safety concerns, hospitalization may be necessary. The child's medical care must be the priority. Although it may be uncomfortable to discuss concerns with the nonoffending parent, it is recommended that pediatricians do so early to provide support for the family and to remind them that physicians have ethical and legal obligations in cases of suspected abuse. When the pediatrician has concerns that the presenting parent is the alleged perpetrator, such as in the vignette, it is best to consider patient safety and ask for guidance from CPS personnel about disclosing a report. \nIt is important that the pediatrician remains a point of contact and medical home for the affected child, providing guidance, resources, assistance, and reassurance to the family. Pediatricians may need to work with investigators to describe medical findings and concerns.\nThe infant in the vignette has known risk factors and triggers for child abuse (Table 2). Her mother reported fussiness with feeding and intermittent crying, and she expressed an expectation that her child should be sleeping through the night. It is common for parents to misunderstand age-appropriate development; it is vital for the pediatrician to provide anticipatory guidance at each visit. In addition, the infant in the vignette has a twin sibling. Siblings of abused children are at increased risk for abuse, especially twin siblings, and should be evaluated for injuries.\nAlthough it is important to provide counseling and anticipatory guidance, including discussing expected behavior during each visit as children develop, the infant's safety is the critical factor at this time; therefore, this would not be the best next step in the management of the infant in the vignette. Laboratory testing for hematologic disorders might be appropriate in some cases to rule out a medical explanation for bruising; however, it would not be the best next step for the infant in the vignette with an unexplained bruise. A bleeding disorder would not explain the infant's patterned injury. Scheduling a follow-up visit the next day leaves the infant at risk for additional abuse.\nWith increased awareness and knowledge about abusive injuries and use of a multidisciplinary approach for suspected abuse cases, pediatricians will become more comfortable making CPS reports and supporting families. Physician familiarity with community resources can both assist families during an abuse investigation and, importantly, facilitate appropriate family support before abuse occurs.\nSuggested Reading(s)\nAmerican Academy of Pediatrics. Visual Diagnosis of Child Abuse [CD-ROM]. 3rd ed. American Academy of Pediatrics; 2008.\nAnderst JD, Carpenter SL, Abshire TC; Section on Hematology/Oncology and Committee on Child Abuse and Neglect; Hord J, Crouch G, Gregory Hale G, et al. Evaluation for bleeding disorders in suspected child abuse. Pediatrics. 2013;131(4):e1314-e1322. doi:10.1542/peds.2013-0195\nChristian CW; Committee on Child Abuse and Neglect, American Academy of Pediatrics. The evaluation of suspected child physical abuse. Pediatrics. 2015;135(5):e1337-e1354. doi:10.1542/peds.2015-0356\nFlaherty EG, Stirling J; The Committee on Child Abuse and Neglect. The pediatrician's role in child maltreatment prevention. Pediatrics. 2010;126(4):833-841. doi:10.1542/peds.2010-2087\nSheets LK, Leach ME, Koszewski IJ, Lessmeier AM, Nugent M, Simpson P. Sentinel injuries in infants evaluated for child physical abuse. Pediatrics. 2013;131(4):701-707. doi:10.1542/peds.2012-2780\nContent Domain\nChild maltreatment\nABP Content Specification(s) / Content Area(s)\nUnderstand the physician's duty and ethical obligation to report suspected child abuse or neglect\nProvide appropriate guidance and support to a family during an investigation of child abuse or neglect\nThe correct answer is: make a report to child protective services"}
{"id" : 2885, "question_text" : "A healthy 2-month-old male infant, born at full term, is seen for a health supervision visit. The family recently moved from another state. His mother reports persistent drainage of clear fluid from his umbilicus despite several cauterization procedures with silver nitrate by his previous pediatrician. The infant is otherwise doing well and has remained afebrile. He has had normal intake and output. His vital signs and growth parameters are normal. His physical examination findings are normal except for a 3-mm-diameter umbilical granuloma with serous drainage. There is no erythema or tenderness around the umbilicus. Of the following, the BEST next step in management is to", "options" : "[\"ligate the umbilical stump with absorbable sutures\", \"perform ultrasonography of the umbilicus\", \"prescribe oral cephalexin\", \"provide parental reassurance\"]", "explanation" : "Correct Answer: B\nThe infant in the vignette should undergo umbilical ultrasonography to assess for persistent patent umbilical structures. Ligation of the granuloma is inappropriate because of its size and shape. Antibiotics are not necessary because the child has no evidence of infection. Reassurance is inadequate because the drainage has persisted despite repeated attempts at cauterization. In utero, the umbilical cord is the sole source of nutrition for the developing fetus. After birth, the healthy neonate no longer requires this structure and the cord is usually cut, leaving a small stump. In the United States, delivering physicians usually practice delayed cord clamping to enhance neonatal iron stores and decrease the risk of intraventricular hemorrhage and necrotizing enterocolitis.\n\nCare for the umbilical stump involves practicing good hand hygiene and keeping the stump dry. Applying alcohol is no longer recommended; this practice can delay cord separation by keeping the cord moist and it does not effectively reduce bacterial colonization. In developing countries, or where delivery of the infant may be less hygienic, the application of antiseptics such as chlorhexidine may be used to reduce infection rates.\n\nThe umbilical stump usually falls off after 1 to 2 weeks, but may take up to 3 weeks. Before the umbilicus is completely healed and dried, caregivers should limit bathing to sponge baths only. If the stump is soiled with fecal matter, simple soap and water is adequate for cleaning. Causes of delayed cord separation include prematurity, antimicrobial use, or immunologic disorders. Investigation of immunologic function is recommended for neonates with delayed cord separation who also have other signs of immune system problems.\n\nAfter cord separation, a small umbilical granuloma may form, which may have serous or serosanguinous drainage. The granuloma can be treated with silver nitrate cauterization. Granulomas that are pedunculated can be ligated with absorbable sutures. Persistent granulomas should be evaluated for other abnormalities.\n\nPersistent umbilical drainage is the most common presentation for urachal anomalies such as umbilical polyps, urachal cysts, and patent urachal sinuses. Ultrasonography is the preferred imaging modality for these anomalies. Because of the risk of urachal adenocarcinoma, children who have a urachal anomaly as well as other symptoms such as infection, pain, or drainage of fecal matter should undergo surgical excision. Children who are otherwise asymptomatic can be monitored closely.\n\nWhen the umbilical cord falls off, the neonate may be found to have an umbilical hernia. Parents should be reassured that most umbilical hernias will spontaneously close by 6 years of age. Home remedies such as treatment with tape or coins are not indicated and do not accelerate closure. Referral to a surgeon is indicated if the hernia is trunk-like in appearance or becomes incarcerated. Strangulation of an umbilical hernia is rare, and should be treated as an emergency. Referral should also be considered for an umbilical hernia that has not closed by age 6 years.\n\nPREP Pearls\n• Ultrasonography is the preferred imaging modality to evaluate infants with persistent umbilical granulomas or drainage; surgical referral should be considered.\n• Dry cord care is recommended for infants delivered under hygienic conditions.\n• Most umbilical hernias close spontaneously by age 6 years.\n\nABP Content Specifications(s)\n• Plan appropriate umbilical cord care\n\nSuggested Readings\n• Marion RW, Samanich J. Umbilical anomalies. In: McInerny TK, Adam HM, Campbell DE, DeWitt TG, Foy JM, Kamat DM, eds. American Academy of Pediatrics Textbook of Pediatric Care. 2nd ed. Itasca, IL: American Academy of Pediatrics; 2016;chap 343:2745-2747. Pediatric Care Online.\n• Muniraman H, Sardesai T, Sardesai M. Disorders of the umbilical cord. Pediatr Rev. 2018;39(7):332-341. doi:10.1542/pir.2017-0202.\n• Stewart D, Benitz W; Committee on Fetus and Newborn. Umbilical cord care. Pediatrics. 2016;138(3). doi: 10.1542/peds.2016-2149."}
{"id" : 879, "question_text" : "A 9-month-old female infant develops stereotyped spells of eye rolling, head drop, and truncal flexion. They last 5 seconds each and come in clusters of 10 to 15, typically when she is falling asleep or waking up. She cries after the cluster of spells ends but then returns to normal. She is otherwise healthy. Her physical examination shows an occipital frontal circumference at the 50th percentile. She has several hypopigmented macules that are observed more clearly during a Wood lamp examination (Item Q172). The remainder of her general and neurologic examination findings is normal. Routine electroencephalogram report reveals hypsarrhythmia. Of the following, the MOST likely diagnosis is", "options" : "[\"ataxia telangiectasia\", \"hypomelanosis of Ito\", \"neurofibromatosis type 1\", \"Sturge-Weber syndrome\", \"tuberous sclerosis\"]", "explanation" : "The infant described in the vignette presents with infantile spasms. On examination, she is found to have hypopigmented skin lesions, suggesting a diagnosis of tuberous sclerosis. Other clinical manifestations of tuberous sclerosis usually do not appear until older ages. These include facial angiofibromas that can appear at about 4 years of age and periungual fibromas that appear in adolescence.\n\nThis infant should be referred to a pediatric neurologist for management of infantile spasms. She will also need a magnetic resonance image of the brain, with and without contrast, to evaluate for the typical findings of cortical tubers, radial glial bands, and subependymal nodules. Occasionally, the subependymal nodules can transform into subependymal giant cell astrocytomas, which can cause obstructive hydrocephalus. Infants and children with tuberous sclerosis should be clinically monitored for abnormal head growth and signs of increased intracranial pressure. Other manifestations of tuberous sclerosis include cardiac rhabdomyomas (especially in neonates), renal angiomyolipomas, autism spectrum disorder, and epilepsy.\n\nIt is important to recognize the characteristic skin findings in neurocutaneous syndromes. Ataxia telangiectasia presents with ataxia in young toddlers; telangiectasis of the sclera and face develop as early as 5 years of age. Hypomelanosis of Ito presents with whorls of hypopigmented and hyperpigmented skin, and some but not all patients have intellectual disability. Neurofibromatosis type 1 is characterized by café au lait spots, and later, axillary and inguinal freckling. Sturge-Weber syndrome is characterized by facial angiomatosis (also known as a port wine stain) of the forehead and upper eyelid.\n\nPREP Pearls\n• Hypopigmented macules are associated with tuberous sclerosis, and hyperpigmented café au lait spots are associated with neurofibromatosis type 1.\n• Infantile spasms may be the first recognized clinical manifestation of tuberous sclerosis. They occur in up to 65%-70% of patients with tuberous sclerosis and frequently indicate a poor neurodevelopmental outcome.\n\nAmerican Board of Pediatrics Content Specification(s):\n• Recognize the clinical manifestations of tuberous sclerosis, and manage appropriately\n\nSuggested Reading:\n• Sahin M. Neurocutaneous syndromes.. In: Kliegman RM, Stanton BMD, St Geme J, Schor N, Behrman RE, eds. Nelson Textbook of Pediatrics. 19th ed. Philadelphia, PA: Elsevier Saunders; 2011:2046-2053"}
{"id" : 2271, "question_text" : "A 16-year-old patient is seen in the office. He has a 3-year history of progressively worsening low back pain and associated morning stiffness lasting 2 to 3 hours. He denies fever, weight loss, or gastrointestinal symptoms. Over the past 6 months, he has experienced two episodes of eye redness with pain and photosensitivity. Despite his back pain, he remains active, playing baseball with no significant limitations. On physical examination, his vital signs are normal for age. There is tenderness to palpation over the left sacroiliac joint and decreased range of motion in the lumbar spine. Mild swelling and tenderness are noted over the bilateral Achilles tendons. The remainder of the musculoskeletal examination findings are unremarkable. Of the following, the laboratory finding MOST likely to be observed in this adolescent is a/an", "options" : "[\"positive antinuclear antibody (titer 1:640)\", \"positive HLA-B27\", \"positive rheumatoid factor\", \"thrombocytopenia\"]", "explanation" : "Juvenile ankylosing spondylitis, a form of inflammatory arthritis, presents with lumbar spine/sacroiliac joint pain, limited range of motion of the lumbar spine, and limited chest expansion with radiographic or magnetic resonance imaging findings of sacroiliitis.\nPositive HLA-B27 is seen in up to 90% of patients with ankylosing spondylitis; this can also be seen in 5% of the general population. A positive HLA-B27 alone is not diagnostic for ankylosing spondylitis.\nCritique\nIn this vignette, the patient's history and examination findings of sacroiliac joint tenderness, decreased lumbosacral spine mobility, symptoms of anterior uveitis, and presumed enthesitis of the bilateral Achilles tendons are most consistent with a diagnosis of ankylosing spondylitis. HLA-B27-positive status is seen in up to 90% of individuals with ankylosing spondylitis.\nJuvenile ankylosing spondylitis is a form of juvenile arthritis that belongs to a group of arthritides known as spondyloarthropathies (ie, similar forms of arthritis with frequent involvement of the sacroiliac joints and lumbar spine). Included in this subgroup are juvenile ankylosing spondylitis, psoriatic arthritis, reactive arthritis, arthropathy associated with inflammatory bowel disease, and anterior uveitis. Disease classifications within this subgroup frequently evolve and have been subject to scrutiny due to problematic classification.\nThe diagnostic classification of juvenile ankylosing spondylitis follows the adult classification criteria of ankylosing spondylitis (Table). Often, pediatric patients do not fully meet the adult criteria: radiographic evidence may not be present but instead, sacroiliitis can be detected early by magnetic resonance imaging.\nThe incidence of ankylosing spondylitis in the general population ranges from 0.5% to 2.0%; 10% of cases have onset of disease symptoms in childhood. HLA-B27 positivity itself is not diagnostic of ankylosing spondylitis or spondyloarthropathies. Ninety percent of individuals with ankylosing spondylitis are HLA-B27 positive, however, 10% of affected individuals are HLA-B27 negative. The prevalence of HLA-B27 positivity is around 5% of the general population, so interpretation of HLA-B27 status must be made in the context of the individual and their clinical presentation. Laboratory testing is nonspecific; results often show mild elevation of the C-reactive protein level and erythrocyte sedimentation rate. Other immunologic testing (eg, rheumatoid factor and cyclic citrullinated peptide) is negative. There is no correlation between antinuclear antibody positivity or thrombocytopenia and the spondyloarthropathies.\nRadiographic imaging of the axial joints (Figure 1A) may show ankylosis, erosion, sclerosis, and joint space narrowing. Peripheral joint radiography can show soft tissue swelling and periostitis. Ultrasonography may be helpful in the detection of enthesitis and synovitis of the joints. Magnetic resonance imaging is the most sensitive modality for early detection of vertebral and sacroiliac joint involvement (Figure 1B).\nPeripheral arthritis commonly involves the lower extremity, most often the knees. Involvement is often asymmetrical, commonly seen in the midfoot, ankles, and hips. Tenderness may be observed over the sternoclavicular, sternomanubrial, and costosternal joints; involvement of these areas is often associated with impairment of chest wall expansion. Anterior uveitis can manifest as acute onset of painful redness of the eyes with photophobia that is generally unilateral and recurrent (in contrast with posterior uveitis, seen in other forms of uveitis, which can be asymptomatic). Gastrointestinal symptoms should raise suspicion for inflammatory bowel disease. Cardiopulmonary manifestations (eg, aortic insufficiency or pulmonary fibrosis/bronchiectasis) may be observed in adults and are generally a result of longstanding disease; they are not typically seen in children or at disease onset. Direct neurologic involvement is not seen. Rarely, atlantoaxial subluxation or cauda equina syndrome (secondary to impingement from spinal abnormalities) can occur. Renal papillary necrosis is rare and often secondary to nonsteroidal anti-inflammatory drug usage.\nTreatment of juvenile ankylosing spondylitis and spondyloarthropathies with active sacroiliitis generally starts with nonsteroidal anti-inflammatory drug therapy but often requires biologic agents, particularly tumor necrosis factor inhibitors, for better disease control and improved long-term outcomes. Disease-modifying antirheumatic drugs have a limited role for individuals with axial joint involvement; they are usually reserved more for isolated peripheral involvement. Glucocorticoids, as short-term therapy, are used only for severely affected patients. Physical and occupational therapy play a crucial role in maintaining and regaining the range of motion of affected joints.\nSuggested Reading(s)\nAeder L, Onel KB. Update on juvenile spondyloarthritis. Pediatr Rev. 2021; 42(11):581–589. doi:10.1542/pir.2020-000810\nSiegel DM, Gewanter HL, Sahai S. Rheumatologic diseases. In: McInerny TK, Adam HM, Campbell DE, DeWitt TG, Foy JM, Kamat DM, eds. American Academy of Pediatrics Textbook of Pediatric Care. American Academy of Pediatrics; 2021:chap 324. Accessed September 21, 2024. Pediatric Care Online\nContent Domain\nRheumatology\nLearning Objectives\nDescribe the clinical and laboratory findings associated with ankylosing spondylitis"}
{"id" : 1591, "question_text" : "An 11-month-old male infant has been repeatedly seen in your practice over the last 3 months because of an unrelenting rash. The rash started in the diaper area as pink papules with a brownish crust, and then it appeared behind the ears bilaterally as pink flaky lesions. About the same time, the infant developed diffuse erythematous papular lesions on the scalp with gray scales. Topical treatments including nystatin cream and 1% hydrocortisone ointment have yielded no improvement. The application of baby oil to the scalp followed by combing out the flakes also yielded no improvement. There is no rash on the infant's arms or legs. According to his mother, there has been no recent change to his diet, brand of diapers, carpeting, bedding, skin soap, or clothing detergent. His only medication, other than the topical treatments, is a multivitamin. Until the onset of the rash, the child was healthy. His growth and development are normal. He is at the 45th percentile for height and the 55th percentile for weight, which are consistent with his prior office visits. His vital signs are normal for his age. His heart, lung, abdominal, extremity, and gonadal examination findings are normal. However, there is a clear discharge from the right ear. An otoscopic examination reveals an inflamed, occluded right external ear canal and a normal left ear canal. His skin examination findings are remarkable for the lesions shown in Item Q63. The results of a complete blood cell count and complete metabolic panel are normal. You refer the infant to a pediatric dermatologist who performs a biopsy. Of the following, the skin biopsy is MOST likely to show", "options" : "[\"allergic dermatitis, most consistent with a drug eruption\", \"candidal skin infection\", \"Langerhans cell histiocytosis\", \"Sarcoptes scabiei infection\", \"stage MS (formerly 4S) neuroblastoma\"]", "explanation" : "Correct Answer: C\nThe otherwise healthy infant in this vignette has a dermatologic process involving the diaper area, scalp, and ear canal. Treatments including antifungal and corticosteroid ointments have been tried. This presentation along with the appearance of the rash shown in Item C63 should raise concern for Langerhans cell histiocytosis (LCH), a rare disease that involves the abnormal proliferation of histiocytes.\n\nThis disease most commonly occurs in children and adolescents between 1 and 15 years of age. The LCH lesions can present in a single site (unifocal) or multiple sites (multifocal) and in single organs or multiple organs. The most commonly affected organs are the bones and skin. The most common presentations are osteolytic, painful bony lesions and eruptions on the scalp, although LCH can present with persistent fevers, weight loss, lethargy, and pancytopenia. In some children, multifocal LCH can involve the pituitary stalk and leads to diabetes insipidus. The pathogenesis of LCH is unclear. Whether LCH is a reactive process or a true malignancy has been the subject of debate; however, the recent identification of BRAF mutations in the abnormal histiocyte population suggests a clonal malignancy. Despite the prevalence of BRAF mutations within the lesions, LCH is not an inherited disease.\n\nWhen LCH is suspected, a confirmatory biopsy should be performed, and the child should undergo a complete skeletal survey, magnetic resonance imaging of the pituitary gland, and determination of urine specific gravity and serum sodium concentration, as well as a bone marrow aspirate and biopsy if a cytopenia is present. If LCH presents as a solitary bone lesion, the biopsy should be performed as a curettage, which often stimulates spontaneous regression such that no further therapy is needed. In multisystem disease or solitary bone disease in certain \"risk\" areas, systemic chemotherapy with corticosteroids and vinblastine is indicated. Children with single-focus disease have an excellent prognosis. However, up to 60% of children with multifocal or multiorgan disease will have a chronic course with a mortality rate of up to 10%.\n\nBecause the infant in this vignette has not been taking medications other than a multivitamin, a drug eruption is unlikely. Although the appearance of the rash in the picture could be consistent with a candidal rash, the lack of improvement with topical antifungal treatment and the involvement of the scalp and the ear canal makes a cutaneous candidal infection less likely. The appearance of the rash and the involvement of the scalp and ear canal are not consistent with a Sarcoptes scabiei infection. Stage MS neuroblastoma, a malignant proliferation of embryonal cells of neural crest origin, commonly presents in infants and can involve subcutaneous nodules, but it does not present with an erythematous scaly rash.\n\nPREP Pearls\n• Langerhans cell histiocytosis should be considered when skin lesions in the diaper area and scalp persist despite frontline therapy.\n• In some children, multifocal Langerhans cell histiocytosis can involve the pituitary stalk and lead to diabetes insipidus with associated increased thirst and urination.\n• Children with a confirmed Langerhans cell histiocytosis lesion should undergo a complete evaluation for distant disease, including a skeletal survey and magnetic resonance imaging of the pituitary, as well as bone marrow biopsy if a cytopenia is present.\n\nABP Content Specifications(s)\n• Recognize the clinical findings associated with histiocytosis syndromes of childhood\n\nSuggested Readings\n• Allen CE, Kelly KM, Bollard CM. Pediatric lymphomas and histiocytic disorders of childhood. Pediatr Clin North Am. 2015;62(1):139–165. doi: http://dx.doi.org/10.1016/j.pcl.2014.09.010.\n• Allen CE, Ladisch S, McClain KL. How I treat langerhans cell histiocytosis. Blood. 2015;126(1):26–35. doi: http://dx.doi.org/10.1182/blood-2014-12-569301.\n• Badalian-Very G, Vergilio JA, Degar BA, et al. Recurrent BRAF mutations in langerhans cell histiocytosis. Blood. 2010;116(11):1919–1923. doi: http://dx.doi.org/10.1182/blood-2010-04-279083."}
{"id" : 2639, "question_text" : "A 10-year-old girl is seen for a health supervision visit. The girl recently completed a formal psychoeducational evaluation through her school because of long-standing learning difficulties and was diagnosed with dyslexia. The girl starts to cry. She admits that she is worried about no longer being in the same class as her friends and that the other students will \"know I'm different because I have to go to the other classroom.\" The school has a family meeting planned to discuss how special education services will be implemented. The girl's mother received special education services for dyslexia when she was in school, which prevented her from attending her favorite class, which was art. She began investigating private school options for her daughter because she is worried that she will not have any input into determining how educational services will be provided. Of the following, the BEST next step in counseling this girl's mother is to", "options" : "[\"encourage her to continue her search for a private school so that she can be involved in decision-making\", \"have the mother request that special education instruction take place during the girl's physical education class time\", \"recommend that the girl be in a regular education setting with in-class supports and small group instruction\", \"recommend that the girl be in a self-contained classroom for her entire school day\"]", "explanation" : "In 1975 the Individuals with Disabilities Education Act (IDEA) was passed through Congress, which mandates that a free and appropriate public education be provided and that supports be implemented in the least restrictive environment allowing children with disabilities to be educated with children without disabilities as much as possible. For the girl in the vignette, in-class supports along with small group instruction would be considered the least restrictive educational environment. Pull-out instruction (having the girl leave her classroom periodically) may be considered if the girl does not meet the goals of her individualized education plan within the least restrictive setting.\n\nIt is important for parents and caregivers to be involved in the educational planning process through shared decision-making. Parents should be encouraged to provide input into the implementation of special education services. Having the girl attend a private school does not guarantee that her mother would have more involvement in decision-making. Additionally, children placed in a private school by their parents do not have the same entitlement to special education services that they would receive by attending public school. A local or state agency may place a child in private school who was previously attending public school if the public school is unable to provide a free and appropriate education.\n\nPlacement of the girl in a self-contained classroom would be the most restrictive environment and would not be an appropriate initial educational setting. For a child with more severe and global disabilities, a self-contained classroom may be an appropriate setting. However, opportunities for \"main-streaming\" during electives such as music, physical education, and recess should be encouraged to provide exposure to typical peers. The girl should not be removed from physical education as physical activity may help support self-esteem, improve mood, and provide additional opportunity for peer interaction.\n\nPediatricians play an important role in not only identifying children in need of special education services, but in the implementation and longitudinal monitoring of educational interventions. Pediatricians should encourage parents and caregivers to take an active role in their children's education plans and provide education on their right to a free and appropriate public education in the least restrictive environment.\n\nPREP Pearls\n• Children with disabilities must be educated in the least restrictive environment and, as much as possible, with children without disabilities.\n• All children are entitled to a free and appropriate public education with the school providing enough support for the child to progress.\n• Parents are key stakeholders in the design and implementation of special education services.\n\nABP Content Specifications(s)\n• Recognize appropriate educational settings for patients with learning disabilities, and the various strategies utilized in those settings to circumvent weaknesses\n\nSuggested Readings\n• Council on Early Childhood; Council on School Health. The pediatrician's role in optimizing school readiness. Pediatrics. 2016;138(3):e20162293. doi:10.1542/peds.2016-2293.\n• Frankowski BL. Learning difficulty. In: McInerny TK, Adam HM, Campbell DE, DeWitt TG, Foy JM, Kamat DM, eds. American Academy of Pediatrics Textbook of Pediatric Care. American Academy of Pediatrics; 2021:chap 172. Accessed September 1, 2022. Pediatric Care Online.\n• Lipkin PH, Okamoto J; Council on Children with Disabilities; Council on School Health. The Individuals with Disabilities Education Act (IDEA) for children with special educational needs. Pediatrics. 2015;136(6):e1650-e1662. doi:10.1542/peds.2015-3409.\n• Rey-Casserly C, McGuinn L, Lavin A; Committee on Psychosocial Aspects of Child and Family Health,section on\n• Developmental and Behavioral Pediatrics. School-aged children who are not progressing academically: considerations for pediatricians. Pediatrics. 2019;144(4):e20192520. doi:10.1542/peds.2019-2520."}
{"id" : 3580, "question_text" : "An 8-hour-old neonate who was delivered at 39 2/7 weeks' gestational age is reported to be dusky in appearance and tachycardic. Neither the maternal history nor prenatal laboratory results are of concern. The neonate's heart rate is 280 beats/min, respiratory rate is 80 breaths/min, and blood pressure is 40/20 mm Hg. The pulse oximeter will not give a reading on the right hand. Pulses are present throughout, but weak. Electrocardiography is performed (Item Q24). Of the following, the BEST next step in management is", "options" : "[\"adenosine\", \"amiodarone\", \"digoxin\", \"synchronized cardioversion\"]", "explanation" : "The neonate in the vignette is experiencing narrow complex, regular tachycardia and has poor perfusion. Therefore, the treatment of choice is synchronized cardioversion. Adenosine would often be used to diagnose and treat a narrow complex tachycardia, but in this setting of poor cardiac output (hypotension and poor perfusion), synchronized cardioversion is warranted. Antiarrhythmic medications would not be the correct choice of action, given the neonate's poor perfusion.\n\nArrhythmias can present in various ways including palpitations, chest pain, syncope, and signs of heart failure. Electrocardiography or a rhythm strip while symptomatic is instrumental in making the diagnosis. This can be aided by the use of an ambulatory cardiac rhythm monitor or event recorder if symptoms are intermittent.\n\nRhythm abnormalities can be categorized as bradyarrhythmias or tachyarrhythmias, wide or narrow complex QRS, and regular or irregular. The neonate in the vignette has a narrow complex, regular tachyarrhythmia consistent with atrial flutter. Rhythms that fall under this combination of categories include supraventricular tachycardia (SVT; including orthodromic reciprocating tachycardia and atrioventricular [AV] nodal reentrant tachycardia), atrial flutter, and atrial tachycardia. A wide complex, regular tachycardia is consistent with ventricular tachycardia though it can be SVT with aberrancy. A narrow complex, irregular tachycardia typically represents atrial fibrillation. A wide complex irregular tachycardia typically represents ventricular fibrillation. The bradyarrhythmias typically occur when either the sinus node or AV node are not functioning properly. Item C24 describes the different types of AV block.\n\nMany different medications are used to treat arrhythmias. Acutely symptomatic bradyarrhythmias may respond to epinephrine, atropine, or isoproterenol. Placement of a pacemaker will be required for severe and/or symptomatic bradyarrhythmias. Supraventricular tachycardia, a common tachyarrhythmia, can be aborted with adenosine; this drug must be administered via rapid intravenous push because of its short half-life. Categories of other antiarrhythmic medications include β-blockers, calcium channel blockers, and sodium channel blockers. Calcium channel blockers are generally avoided in neonates because of the risk of hypotension and bradycardia.\n\nIf a child with an arrhythmia becomes unstable, synchronized cardioversion or defibrillation may become necessary. More organized rhythms warrant synchronized cardioversion to avoid delivering energy during repolarization (shocking on a T wave). Less organized rhythms (pulseless ventricular tachycardia, ventricular fibrillation) necessitate defibrillation.\n\nPREP Pearls\n• Arrhythmias can present in various ways including palpitations, chest pain, syncope, and signs of heart failure.\n• Electrocardiography can help diagnose an arrhythmia; arrhythmias are categorized using characteristics such as bradycardia versus tachycardia, wide versus narrow QRS complex, and regular versus irregular.\n• Arrhythmia treatment options depend on the rhythm and patient symptoms; options include medications, pacemakers, and synchronized cardioversion or defibrillation.\n\nABP Content Specifications(s)\n• Recognize the electrocardiographic characteristics of various cardiac dysrhythmias\n• Plan the appropriate management of various cardiac dysrhythmias\n• Recognize the clinical findings associated with various cardiac dysrhythmias\n\nSuggested Readings\n• Baruteau AE, Perry JC, Sanatani S, Horie M, Dubin AM. Evaluation and management of bradycardia in neonates and children. Eur J Pediatr. 2016;175(2):151-161. doi:10.1007/s00431-015-2689-z.\n• Harris JP. Cardiac arrhythmias. In: McInerny TK, Adam HM, Campbell DE, DeWitt TG, Foy JM, Kamat DM, eds. American Academy of Pediatrics Textbook of Pediatric Care. 2nd ed. Elk Grove Village, IL: American Academy of Pediatrics; 2017:1227-1235. Pediatric Care Online.\n• Srinivasan C. Diagnosis and acute management of tachyarrhythmias in children. Indian J Pediatr. 2015;82(12):1157-1163. doi:10.1007/s12098-015-1881-5.\n• Wackel P, Cannon B. Heart rate and rhythm disorders. Pediatr Rev. 2017;38(6):243-253. doi:10.1542/pir.2016-0119."}
{"id" : 3582, "question_text" : "A 16-year-old adolescent girl is having a health supervision evaluation. She has bipolar disorder, and at the time of her last visit, was being treated with aripiprazole by her psychiatrist. She discontinued this medication 6 months ago, because she did not like the adverse effects (insomnia and weight gain). Instead, she has been smoking marijuana 3 times per day to manage her symptoms. Her mother is aware of her marijuana use and is supportive of her daughter using a \"natural\" plant-based drug to control her mood, as opposed to a psychiatric medication. The patient was recently caught with marijuana at school and expelled. She is currently enrolled in an alternative school, but has not been attending regularly because of her marijuana smoking schedule. She plans to start homeschooling and online courses. The counselor at her former school filed a report with child protective services, and the patient and her mother have been interviewed by a caseworker. The case is currently open, but the family expects it will soon be closed. The mother agrees that her daughter's marijuana use is having a negative impact on her education, but is not sure how to help her. This patient's practice of self medication with marijuana is a concern for the primary care physician. Of the following, the BEST next step in this adolescent's management is to", "options" : "[\"assess the mother's readiness for change, including the possibility of signing her daughter into a drug rehabilitation program without her assent\", \"call her psychiatrist to schedule a follow-up appointment for medication management\", \"discuss with the mother the different types of drug rehabilitation programs for adolescents in the area and provide resources\", \"schedule a follow-up appointment in 3 months to check a urine drug screen and evaluate the success of her homeschooling\"]", "explanation" : "The adolescent girl in the vignette is self-treating her bipolar disorder and has signs of cannabis use disorder based on the frequency of daily use, failure to attend school, and continued use despite the negative effects it has had on her life. She would benefit from a drug rehabilitation program to address her substance use, but may need parental intervention if she is not willing to go on her own and believes she does not have a problem.\n\nThe most common substances used by adolescents are tobacco, marijuana, and alcohol. According to the most recent Youth Risk Behavior Survey results (2018), 28.9% of high school students have ever tried a cigarette; 42.2% of students have used an electronic vaping device; 35.6% of students have smoked marijuana 1 or more times in their life; and 60.4% of students have had at least 1 alcoholic beverage in their life. Primary care physicians are often the first to suspect and manage substance abuse disorders; this can be a challenge when trying to maintain adolescent confidentiality while navigating family dynamics.\n\nMultiple factors influence adolescent substance use, including biological and genetic predisposition, as well as social influences. The prefrontal cortex, responsible for executive functioning, is one of the last portions of the brain to mature. Adolescents and young adults who experiment with and use substances may be vulnerable to drug or alcohol abuse because they are priming their award and incentive pathways, components of executive functioning, which are still developing. Adolescents and young adults with parents and friends who use substances, or are tolerant of substance use, are more likely to experiment with drugs and alcohol. In addition, adolescents and young adults with poor support systems, school failure, or a history of physical or sexual abuse are at increased risk for substance abuse.\n\nThe American Academy of Pediatrics recommends screening all adolescents for alcohol, tobacco, and drug use at yearly health supervision visits and any acute care visit. Numerous screening tools are available to providers including the HEADSSS mnemonic to evaluate the social history in adolescents and young adults (home, education, activities, drugs and alcohol, sexuality, suicide and depression, and safety/violence) and the CRAFFT screen as described in Knight et al.\n\nAs part of routine health care, the Substance Abuse and Mental Health Services Administration recommends an approach known as substance use screening, brief intervention, and referral to treatment (SBIRT). A brief intervention involves having a discussion with the adolescent about his/her substance use; the goal is to reduce, prevent, or stop unhealthy choices. This may include positive reinforcement for not using drugs or stopping use, or may involve motivational interviewing to engage an adolescent with significant substance use in making a change. Referral to treatment may be needed for the adolescent with a severe substance use disorder. The primary care provider plays an important role in helping adolescents and their family understand why a referral for treatment is deemed necessary; facilitating the referral process, including offering suggestions for outpatient versus inpatient treatment centers; and helping to identify programs that will be covered by the adolescent's health insurance. Only 10% of adolescents who need specialty care for substance abuse receive the appropriate treatment. For adolescents who participate in substance use treatment programs, the primary care provider continues to play an important role in the recovery process. Relapse is common; regular follow-up to gauge how the adolescent is doing is crucial.\n\nAddressing substance use disorders in adolescents is difficult because confidentiality must also be considered and state laws vary. Adolescents should be told in advance if their confidentiality will be broken because a provider is concerned about harm to self or others (ie, driving drunk), and it may be necessary to involve various family members to ensure that treatment is sought. In addition, it may be difficult to convince a parent who is also engaged in substance abuse that their child needs treatment.\n\nFor the adolescent girl in this vignette, medication management by the psychiatrist may not be effective until her substance abuse is under better control. Discussing available resources for treatment will be an important part of any discussion with the mother. However, a more proactive approach to getting the child treatment will be crucial because the mother has allowed her daughter to self-medicate with cannabis. Scheduling a follow-up appointment in 3 months to check-in on school and drug testing is not likely to encourage any change without an intervention.\n\nPREP Pearls\n• Adolescents with a substance abuse disorder may require a drug rehabilitation program, and may need parental intervention if unwilling to participate.\n• The American Academy of Pediatrics recommends screening all adolescents for alcohol, tobacco, and drug use at yearly health supervision visits and any acute care visit.\n• As part of routine adolescent health care, the Substance Abuse and Mental Health Services Administration recommends a substance use screening tool entitled screening, brief intervention, and referral to treatment (SBIRT).\n\nMOCA-Peds Objective\n• Recognize and apply ethical principles involving confidentiality.\n\nABP Content Specifications(s)\n• Understand the importance of periodically reassessing the progress of a patient who has been referred for substance use/abuse treatment, including reassessment for relapse\n• Understand the primary care physician's role in preparing an adolescent and his/her family for referral for substance use/abuse treatment\n\nSuggested Readings\n• Kann L, McManus T, Harris W, et al. Youth risk behavior surveillance - United States, 2017. MMWR Recomm Rep. 2018;67(8):28-51. doi:10.15585/mmwr.ss6708a1.\n• Knight J, Roberts T, Gabrielli J. Screening: adolescent alcohol and substance use and abuse. In: Tanski S, Garfunkel LC, Duncan PM, Weitzman M, eds. Performing Preventative Services: A Bright Futures Handbook. Elk Grove Village, IL: American Academy of Pediatrics; 2010:103-112. Pediatric Care Online.\n• Knight JR, Sherritt L, Shrier LA, Harris SK, Chang G. Validity of the CRAFFT substance abuse screening test among adolescent clinic patients. Arch Pediatr Adolesc Med. 2002;156(6):607-614. doi:10.1001/archpedi.156.6.607.\n• Levy SJ, Williams JF; AAP Committee on Substance Use and Prevention. Substance use screening, brief intervention, and referral to treatment. Pediatrics. 2016;138(1):e1-e15. doi:10.1542/peds.2016-1211.\n• Nackers KA, Kokotailo P, Levy SJ. Substance abuse, general principles. Pediatr Rev. 2015;36(12):535-544. doi:10.1542/pir.36-12-535."}
{"id" : 2820, "question_text" : "A mother requests early discharge of her neonate, who is 36 hours of age, so she can be home with her 2-year-old daughter and husband. Her mother is also staying at her home to help. The boy was born at 38 weeks 6 days in a normal spontaneous vaginal delivery without any complications after an uneventful pregnancy. He has been nursing well, voided four times, and passed stools three times. His temperature is 37.2°C, his heart rate is 120 beats/min, his respiratory rate is 40 breaths/min, and his oxygen saturation is 98% on room air. His physical examination findings are normal. Of the following, the BEST next management step for this neonate's care is to", "options" : "[\"discharge him at 72 hours of age\", \"discharge now with follow-up within 48 hours\", \"obtain a complete blood cell count\", \"obtain a serum bilirubin level\"]", "explanation" : "The neonate in the vignette meets all the criteria necessary for an early discharge (before 48 hours of age). Neonates discharged early should be seen by a care provider within 48 hours. If newborn testing is completed before 24 hours, a repeat newborn screening should be obtained at that follow-up visit.\n\nEstablishing a discharge plan after delivery includes many factors, including social support, ability to follow up with a pediatric provider within 48 hours of discharge, and medical stability of both the mother and infant. Parents are often less comfortable with newborn care when the infant is their firstborn child. Mothers should have support from a spouse or partner, as well as from another family member or close friend. Coordination of a follow-up appointment, including transportation, should be arranged before discharge.\n\nNeonates born to mothers with chronic medical conditions, excessive maternal bleeding, or complications from delivery, including the need for instrument assistance or cesarean delivery, should not be considered for early discharge.\n\nAs neonates transition from intrauterine to extrauterine physiology, cardiopulmonary complications can arise, usually within 12 hours of delivery. Other concerning conditions that can present in the newborn period include infection, ductal-dependent cardiac lesions, bowel obstructions, and feeding difficulties. A list of reassuring criteria, which place the neonate in a low-risk category, should be used when considering discharge for a term neonate (gestational age 37 0/7-41 6/7 weeks):\n• Normal physical examination findings, including vital signs for 12 hours before discharge\n  o Temperature 36.5°C to 37.4°C while lying in an open crib\n  o Respiratory rate less than 60 breaths/min without any increased work of breathing\n  o Heart rate 100 to 190 beats/min while awake and more than 70 beats/min while asleep, with normal circulation and appropriate response to stimuli\n• One stool and normal urination\n• Two feedings with good latch and suck/swallow pattern, with one feeding observed\n• No excessive bleeding for 2 hours after a circumcision\n• Appropriate treatment plan for hyperbilirubinemia, if present; negative result on Coombs test\n• Reassuring sepsis work-up if risk factors are identified\n• Normal maternal laboratory findings, including negative results for syphilis, hepatitis B surface antigen, and human immunodeficiency virus\n• Receipt of intramuscular vitamin K\n\nThe neonate in the vignette was delivered vaginally without complications, meets the criteria for early discharge, and can safely be discharged before 72 hours of age. A complete blood cell count is not indicated because he has no risk factors for early sepsis. This neonate does not have any risk factors for hyperbilirubinemia and his physical examination findings are normal, making it unnecessary to obtain a bilirubin level.\n\nPREP Pearls\n• Neonates at low risk of experiencing complications can be safely discharged 24 hours after birth.\n• Neonates discharged before 48 hours of age should follow up with a pediatric provider within 48 hours.\n• Social support, access to care, and maternal and newborn health should be considered when planning for discharge from the newborn nursery.\n\nABP Content Specifications(s)\n• Plan the early discharge of a newborn infant, including follow-up evaluation\n\nSuggested Readings\n• Benitz WE; Committee on Fetus and Newborn. Hospital stay for healthy term newborn infants. Pediatrics. 2015;135(5):948-953. doi:10.1542/peds.2015-0699.\n• Sullivan CK, Dela Cruz-Rivera S. Hospital discharge of the healthy term and late preterm infant. In: McInerny TK, Adam HM, Campbell DE, DeWitt TG, Foy JM, Kamat DM, eds. American Academy of Pediatrics Textbook of Pediatric Care. 2nd ed. Elk Grove Village, IL: American Academy of Pediatrics; 2016:779-789. Pediatric Care Online."}
{"id" : 893, "question_text" : "A 7-year-old girl is having difficulty establishing relation-ships with other children despite repeated opportunities to do so. The girl prefers to stay near her mother or her teacher and will avoid other children. She sometimes cries and can be difficult to calm down after being dropped off at school, so her mother frequently remains in the classroom for a few minutes before quietly leaving. On days when morning transitions to school are significantly difficult, her mother will allow her to stay home. Her mother reports that, in preschool, things were worse in that she usually \"couldn't\" leave her daughter in the classroom. The girl typically speaks little when in public, but she speaks normally when home alone with her mother. She is an only child and the parents are divorced. When the girl spends the weekend at her father's house, she often expresses worry that something bad is going to happen to her mother. Her mother frequently allows the girl to sleep with her to avoid temper tantrums or nightmares about sleeping alone. Of the following, the BEST next step in this child's care is", "options" : "[\"initiate treatment with a selective serotonin reuptake inhibitor\", \"reassure her mother that because of the improvement seen since preschool, her daughter's problems should resolve without intervention\", \"refer her for a neuropsychological evaluation to determine if she has underlying cognitive impairments\", \"refer her to a cognitive behavior therapist to work on skills for managing her distress\", \"refer her to a play therapist to assist the child in recognizing the causes of her distress\"]", "explanation" : "The child described in the vignette has many symptoms of an anxiety disorder, including characteristics of separation anxiety and generalized anxiety. The most appropriate next step would be to refer her to a mental health specialist to initiate cognitive behavior therapy (CBT). Anxiety disorders in particular are very responsive to the psychoeducation, cognitive restructuring, and graded exposure techniques used in CBT.\n\nA well-monitored low-dose trial of a selective serotonin reuptake inhibitor (SSRI) might be appropriate if she had already been engaged in appropriate psychotherapy that was failing to make progress, and she was having significant functional impairment from anxiety such as an inability to attend school. Because there are no controlled trials supporting the use of SSRIs in very young children, it would be prudent to consult a mental health specialist before prescribing an SSRI in this age group.\n\nThis child is experiencing significant distress from her current symptoms, so reassurance that this will self-resolve would not be appropriate. Her extensive anxiety symptoms are unlikely to self-resolve but rather are likely to change over time into other ways of expressing anxiety such as generalized anxiety or social phobia. Teaching both the child and her family how to appropriately address anxiety will help to manage her symptoms over the long term.\n\nThe only developmental symptom described in the vignette is difficulty making friends, and anxiety symptoms could very easily be responsible for this problem. Therefore a neuropsychological evaluation to look for cognitive impairments would not be warranted. Anxiety disorders can be present in someone with developmental impairments, so a lack of response to anxiety treatment or any other symptoms of developmental impairment could later merit neuropsychiatric testing.\n\nPlay therapy is a far less evidence-based treatment for childhood anxiety problems than CBT, and as such would not be the initial preferred approach in this case.\n\nPREP Pearls\n• CBT is the preferred treatment for childhood anxiety disorders, particularly for elementary school aged children.\n• Watchful waiting with anxiety disorders seldom leads to a full resolution of symptoms.\n\nAAP Mental Health Competency:\n• Recognize the types of evidence based psychotherapies for treating childhood anxiety\n\nSuggested Reading:\n• Connolly SD, Bernstein GA, and the American Academy of Child and Adolescent Psychiatry. Practice parameter for the assessment and treatment of children and adolescents with anxiety disorders. J Am Acad Child Adolesc Psychiatry. 2007;46(2):267-283\n• Ginsburg GS, Kendall PC, Sakolsky D. et al. Remission after acute treatment in children and adolescents with anxiety disorders: findings from the CAMS. J Consult Clin Psychol. 2011;79(6):806-813. doi:10.1037/a0025933"}
{"id" : 2498, "question_text" : "A 15-year-old female high school soccer player is seen in the office for a preparticipation physical evaluation in late August. She also participates on the school track team in the spring. Last track season she experienced severe shin splints; the symptoms have fully resolved. Her family history is significant for her father having a myocardial infarction at 48 years of age. The adolescent has been well, but today developed mild rhinorrhea. Her physical examination findings are remarkable only for a temperature of 38.5 °C and clear nasal discharge. Her hearing screening results are normal. Her vision screening results are 20/30 OD and 20/20 OS.\n\nThe girl is informed that she will need to return in 1 week for reassessment for participation clearance. She asks what made her ineligible for sports clearance today.\n\nOf the following, the BEST response to the adolescent's question is", "options" : "[\"current febrile illness\", \"family history of myocardial infarction in her father at 48 years of age\", \"severe shin splints last season\", \"uncorrected vision right eye 20/30\"]", "explanation" : "The adolescent in the vignette's febrile illness makes her currently medically ineligible for sports participation. Fever elevates the core temperature, increasing the individual's metabolism and heart rate. The altered core temperature leads to temperature dysregulation; the body inherently stores more heat. Exercise further increases heat production, which increases the risk of heat illness. Fever also increases insensible fluid losses, impairs coordination and concentration, and decreases muscle strength, aerobic power, and endurance. Viral illnesses contribute to tissue wasting, muscle catabolism, and a negative nitrogen balance.\n\nThe preparticipation physical evaluation (PPE) addresses both adolescent wellness and safe sport participation. The PPE form is a screening tool used to identify medical and musculoskeletal conditions that may affect the adolescent during physical activity. The goals of this screening are to identify risk factors for and to prevent sudden cardiac death, promote safe physical activity participation, perform injury assessment and injury prevention, and meet liability standards of sport participation for local leagues or schools.\n\nThe American Heart Association guidelines recommend the sports preparticipation evaluation as a case finding screening tool that includes a personal cardiac history (7 questions), family cardiac history (4 questions), and cardiac physical examination. Positive personal history, family history, and/or physical examination findings trigger further cardiac investigation. An isolated family history of myocardial infarction in this adolescent's father at 48 years of age is not a cause for medical ineligibility. A positive family history of sudden cardiac death at younger than 35 years, placement of a defibrillator or pacemaker at younger than 35 years, or a genetic, electrical or structural heart condition warrant further investigation and may delay or disqualify medical clearance.\n\nThe standard PPE form allows the medical practitioner to assign the child or adolescent to 1 of 4 categories:\nMedically eligible for all sports\nMedically eligible for some sports\nNot medically eligible for sports pending further evaluation\nNot medically eligible for any sports\n\nThe preparticipation evaluation also screens for musculoskeletal conditions that affect the adolescent athlete (eg, fractures, stress fracture, or ligament, tendon, muscle, or joint pathology). Unresolved musculoskeletal injuries may warrant additional evaluation or make an athlete medically ineligible. Resolved severe shin splints are not a cause for medical ineligibility. Rather, the history of shin splints should lead to counseling regarding training errors, neuromuscular training, and consideration of physical therapy in the preseason.\n\nVisual acuity should be checked during the PPE. If the corrected vision is worse than 20/20, the athlete should be referred for evaluation by an eye care specialist. If the best-corrected vision is worse than 20/40 in one eye, the individual is considered functionally 1-eyed, and eye protection should be strongly encouraged to protect the unaffected eye. Eye protection is essential for high-risk sports with small projectiles (eg, paintball, air rifle, and BB gun), hard projectiles or sticks (eg, baseball, softball, basketball, cricket, fencing, field hockey, ice hockey, lacrosse, racquetball, and squash), and combat sports (eg, boxing and martial arts). The American Academy of Ophthalmology recommends that athletes who are functionally 1-eyed should not participate in sports that do not allow athletes to wear eye protection (eg, boxing, wrestling, or full-contact martial arts).\n\nSuggested Reading(s)\nAmerican Academy of Family Physicians, American Academy of Pediatrics, American College of Sports\nMedicine, American Medical Society for Sports Medicine, American Orthopaedic Society for Sports\nMedicine and the American Osteopathic Academy of Sports Medicine. Preparticipation Physical\nEvaluation. 5th ed. American Academy of Pediatrics; 2019.\nDick NA, Diehl JJ. Febrile illness in the athlete. Sports Health. 2014;6(3):225-231.\ndoi:10.1177/1941738113508373\nHarris MD. Infectious disease in athletes. Curr Sports Med Rep. 2011;10(2):84-89.\ndoi:10.1249/JSR.0b013e3182142381\nManuel C, Feinstein R. Sports participation for young athletes with medical conditions: seizure\ndisorder, infections and single organs. Curr Probl Pediatr Adolesc Health Care. 2018;48(5-6):161-171.\ndoi:10.1016/j.cppeds.2018.06.004\n\nContent Domain\nSports Medicine\n\nABP Content Specification(s) / Content Area(s)\nRecognize the effects of a febrile illness on sports participation\n\nThe correct answer is: current febrile illness"}
{"id" : 229, "question_text" : "A 2-year-old boy is brought to the emergency department after his mother found him with an open bottle of toilet bowl cleaner. She reports that he had spilled some on his shirt and had some on his face, but she does not know if he drank any of it. The child is awake and alert, and his vital signs are normal. He is drooling slightly, but examination of his oropharynx reveals no lesions.\n\nOf the following, the MOST appropriate next step is to", "options" : "[\"administer activated charcoal\", \"administer syrup of ipecac\", \"perform gastric lavage\", \"provide no further treatment\", \"refer the boy to a gastroenterologist for urgent endoscopy\"]", "explanation" : "Ingestion of a caustic substance causes injury to mucosal and skin surfaces by liquefaction necrosis in alkali exposures and by protein coagulation in acid exposures. Substances at the extremes of the pH scale (<2 and >12) are especially damaging. Patients typically present with drooling, dysphagia, odynophagia, and in many cases, intraoral burns. In addition, they may have vomiting with hematemesis; respiratory distress with stridor or wheezing; and burns on the face, hands, or chest. Because the primary mode of injury is direct tissue corrosion and systemic symptoms are rare, decontamination of patients following caustic ingestions is focused on washing the skin and flushing the eyes, if indicated. Use of activated charcoal is not indicated because it can make subsequent endoscopic evaluation of the esophagus difficult. Syrup of ipecac and gastric lavage are contraindicated because of potential aspiration risk. Further, gastric lavage carries the risk of esophageal perforation.\n\nThe major clinical concerns with a caustic ingestion are airway or esophageal injury. Severe gastritis, perforation, or late stricture formation also may result from significant ingestions. Early airway visualization and protection are indicated in any patient who presents with stridor or respiratory distress, and evaluation of the esophagus by upper endoscopy is indicated in patients who have intraoral burns or other symptoms. In addition, some asymptomatic patients, such as the boy in the vignette, should be considered endoscopy candidates, based on history or other clinical concerns. As many as 45% of patients who do not have oral burns and 12% of asymptomatic patients have findings on endoscopy. The need for endoscopy in the asymptomatic patient in whom a significant caustic ingestion is questionable is controversial.\n\nCritique: [As above]\n\nContent Specifications: Recognize that gastric lavage is contraindicated in a caustic ingestion. Recognize that corrosive material such as hydrochloric and sulfuric acids can be transported to the stomach with few or no esophageal burns, causing severe gastritis, perforation, or late stricture formation."}
{"id" : 810, "question_text" : "You are counseling a couple whose 6-month-old son has Down syndrome and is scheduled for a ventricular septal defect (VSD) repair. He is otherwise well and has been gaining weight appropriately while taking digoxin and furosemide for mild congestive heart failure. The parents ask about complications and whether their child's Down syndrome will put him at greater risk than children who undergo VSD repair and do not have Down syndrome. Of the following, you are MOST likely to tell the parents that their son is at", "options" : "[\"greater risk for anoxic brain injury\", \"greater risk for postoperative fatal arrhythmia\", \"greater risk for postoperative respiratory complications\", \"greater risk for postoperative seizures\", \"no greater risk of perioperative complications\"]", "explanation" : "Preferred Response: C\nThe infant described in the vignette is doing well despite his clinically significant ventricular septal defect (VSD) and diagnosis of Down syndrome. Infants with Down syndrome, as well as infants with other genetic conditions, may be at increased risk for perioperative and postoperative complications, so care must be taken to anticipate potential problems. When compared to control infants who undergo VSD repair, infants with Down syndrome have significantly prolonged postoperative lengths of stay and complications, including a threefold increase in infections, a greater than twofold increase in respiratory complications, a threefold increase in pulmonary hypertension, and a threefold increase in atrioventricular block (but not fatal arrhythmias) that requires a pacemaker. There does not appear to be an increased risk for anoxic brain injury or postoperative seizures.\n\nInfants with certain cytogenetic conditions are at significant risk for concomitant cardiac defects, including Down syndrome (50% risk, often septal defects), trisomy 18, trisomy 13, 22q11 microdeletion (>80% risk of a heart defect, often conotruncal and great artery defects), and Turner syndrome (20%-40% risk, often coarctation of the aorta, aortic stenosis, or bicuspid aortic valve). Also, infants with fetal alcohol syndrome have a significant risk for cardiac anomalies, most often septal defects. Therefore, infants with these diagnoses should undergo echocardiography to rule out a critical cardiac defect.\n\nPREP Pearls\n• Infants with Down syndrome undergoing USD repair have a higher risk for postoperative respiratory complications and prolonged length of stay.\n• Infants with cytogenetic abnormalities or fetal alcohol syndrome should have echocardiography performed to assess for congenital heart defects.\n\nAmerican Board of Pediatrics Content Specification(s):\n• Recognize the increased risk and plan appropriate evaluation of congenital heart disease in a newborn infant with congenital anomalies (eg, trisomy 21, trisomy 18, fetal alcohol syndrome, 22q11 microdeletion, 45,X0)\n\nSuggested Reading:\n• Bloemers BLP, van Furth AM, Weijerman ME, et al. Down syndrome, a novel risk factor for respiratory syncytial virus bronchiolitis: a prospective birth-cohort study. Pediatrics. 2007;120(4):e1076-e1081. doi:10.1542/ peds.2007-0788\n• Botto LD, May K, Fernhoff PM, et al. A population-based study of the 22q11.2 deletion: phenotype, incidence, and contribution to major birth defects in the population. Pediatrics. 2003;112(1):101-107\n• Frias JL, Davenport ML; Committee on Genetics, Section on Endocrinology. Health supervision for children with Down syndrome. Pediatrics. 2003;111:692-702. doi:10.1542/peds.111.3.692\n• Fudge JC, Li S, Jaggers J, O'Brien SM, et al. Congenital heart surgery outcomes in down syndrome: analysis of a national clinical database. Pediatrics. 2010;126(2):315-322. doi:10.1542/peds.2009-3245\n• Health supervision for children with Down Syndrome. Pediatrics. 2011;128(2):393-406. doi:10.1542/peds.2011-1605\n• Schieve LA, Boulet SL, Boyle CB, Rasmussen SA, Schendel D. F of children 3 to 17 years of age with Down syndrome in the 19S National Health Interview Survey. Pediatrics. 2009;123(2):e253 doi:10.1542/peds.2008-1440"}
{"id" : 2476, "question_text" : "A 3-year-old with end-stage renal disease secondary to renal dysplasia is seen in the office for immunization before undergoing a kidney transplant. The surgery will occur in 8 weeks; their mother will be the donor. The child will receive long-term immunosuppressive medications (tacrolimus and mycophenolate mofetil) to prevent rejection after the transplant. They have received all of their routine age-appropriate immunizations. The child is at the 5th percentile for height and 25th percentile for weight for their age. Vital signs and physical examination findings are unremarkable.\n\nOf the following, the MOST appropriate immunization to administer to this child today is", "options" : "[\"Haemophilus influenzae type b\", \"inactivated poliovirus\", \"measles, mumps, and rubella\", \"pneumococcal conjugate\"]", "explanation" : "The child in the vignette, whose routine immunizations are up to date, should receive the measles, mumps, and rubella (MMR) vaccine at this visit. The MMR vaccine is routinely recommended at 12 to 15 months and 4 to 6 years of age. Because it is a live virus vaccine, the MMR vaccine is contraindicated after transplantation. Therefore, to enhance the child's immunity, a second dose of the MMR vaccine should be administered at least 4 weeks before the kidney transplant (as long as it has been at least 28 days since their first MMR dose).\n\nChildren and adolescents requiring kidney and other solid-organ transplants (eg, heart, liver, and lung) should receive all age-appropriate immunizations before receiving the transplant. Live virus vaccines are contraindicated after transplantation owing to the risk of a virulent vaccine strain causing severe illness in a child with immunosuppression. If indicated, live virus vaccines (eg, MMR, varicella, and live attenuated influenza virus) should be given at least 4 weeks before the transplant. In cases of deceased donor transplant, because the timing of transplantation is not planned, the child is made \"inactive\" on the transplant list for at least 4 weeks after immunization with a live virus vaccine.\n\nInactivated vaccines, if indicated, should be given at least 2 weeks before transplantation. The child in the vignette is up to date on their primary series of Haemophilus influenzae type b, inactivated poliovirus, and pneumococcal conjugate vaccine; thus, additional doses are not indicated at this time. Pneumococcal polysaccharide vaccine is indicated for transplant candidates older than 2 years for protection against additional pneumococcal strains. Inactivated virus vaccines can be given 3 to 6 months after transplantation; early administration leads to poor antibody response due to intense immunosuppression. The only exception is inactivated influenza vaccine, which can be given as early as 1 month after transplant and then annually.\n\nChildren who are not up to date with their immunizations should receive catch-up vaccinations before the transplant surgery. After transplantation, primary care providers should work closely with a transplant specialist to ensure that immunosuppressed children continue to receive age-appropriate inactivated vaccines.\n\nSuggested Reading(s)\nAmerican Academy of Pediatrics. Immunization and other considerations in immunocompromised children. In: Kimberlin DW, Barnett ED, Lyfield R, Sawyer MH, eds. Red Book: 2021–2024 Report of the Committee on Infectious Diseases, Committee on Infectious Diseases. 32nd ed. American Academy of Pediatrics; 2021. Accessed September 1, 2023. Red Book Online\nKatz DT, Torres NS, Chatani B, et al. Care of pediatric solid organ transplant recipients: an overview for primary care providers. Pediatrics. 2020;146(6):e20200696. doi:10.1542/peds.2020-0696\nSpinner JA, Denfield SW. Immunosuppressant drugs and their effects on children undergoing solid organ transplant. Pediatr Rev. 2022;43(2):71-86. doi:10.1542/pir.2020-000620\n\nContent Domain\nRenal\n\nABP Content Specification(s) / Content Area(s)\nPlan an appropriate immunization regimen for a patient who is about to undergo renal transplantation\n\nThe correct answer is: measles, mumps, and rubella"}
{"id" : 1606, "question_text" : "You are seeing a 1-day-old, full-term baby in the newborn nursery for the first time. The prenatal testing and course was unremarkable. Apgar scores were 9 at 1 minute and 9 at 5 minutes. There were no complications at birth. Vital signs are all appropriate for age. On physical examination, you note a 2/6 low-pitched, musical, midsystolic ejection murmur at the lower left sternal border. The liver edge is palpable 1 cm below the right costal margin. There is cyanosis of both feet (Item Q78). The remainder of the physical examination findings are unremarkable.\n\nOf the following, the BEST next step in management is", "options" : "[\"initiation of prostaglandin infusion\", \"performance of an echocardiogram\", \"reassurance\", \"referral to pediatric cardiologist\", \"transfer to neonatal intensive care unit\"]", "explanation" : "Correct Answer: C\nThe newborn in this vignette has completely age-appropriate examination findings. The murmur described is a classic description of a benign, innocent murmur. The liver edge is in a typical location for a newborn. The skin examination reveals acrocyanosis, which is normal in the newborn (Item C78A and Item C78B). Therefore, reassurance is all that is warranted.\nThe murmur described is a normal finding, as is the acrocyanosis, making an echocardiogram, prostaglandin infusion, or referral to a pediatric cardiologist unnecessary. There is no indication for transfer to the neonatal intensive care unit.\n\n\nItem C78A: Acrocyanosis.\nReprinted with permission from the Media Laboratory at Doernbecher.\n\n\nItem C78B: Acrocyanosis.\nCourtesy of M. LaTuga\n\nCyanosis is caused by the presence of hemoglobin that is not bound to oxygen in the blood (deoxyhemoglobin) and is typically seen when there is greater than 5 g/dL of deoxygenated hemoglobin. The ability to detect cyanosis is dependent upon hemoglobin concentration (easier to detect with a higher hemoglobin concentration, as noted in newborns, compared to a patient with severe anemia), the site of observation, and the experience of the observer. Peripheral cyanosis (acrocyanosis) is caused by the slow flow of blood through an area with a relatively large arteriovenous oxygen difference. In the newborn, acrocyanosis is commonly seen in the hands and feet and is a normal finding. Central cyanosis, however, would be seen at the lips and mucous membranes. Central cyanosis is a serious finding that warrants prompt evaluation of the respiratory, cardiac, and neurological systems.\n\nPREP Pearls\n• Peripheral cyanosis (acrocyanosis) is caused by the slow flow of blood through an area with a relative large arteriovenous oxygen difference and is a normal finding in newborns, most commonly seen in the hands and feet.\n• Central cyanosis is a serious finding that warrants prompt evaluation of the respiratory, cardiac, and neurological systems.\n• Cyanosis is caused by the presence of hemoglobin that is not bound to oxygen in the blood (deoxyhemoglobin) and is typically seen when there is greater than 5 g/dL of deoxygenated hemoglobin.\n\nABP Content Specifications(s)\n• Distinguish between central cyanosis and acrocyanosis\n\nSuggested Readings\n• Kitterman JA. Cyanosis in the newborn infant. Pediatr Rev. 1982;4(1):13–23. http://pedsinreview.aappublications.org/content/4/1/13.\n• Lees MH, King DH. Cyanosis in the newborn. Pediatr Rev. 1987;9(2):36–42. http://pedsinreview.aappublications.org/content/4/6/183.1."}
{"id" : 148, "question_text" : "A 12-year-old girl who has a history of chronic rhinitis, recurrent sinusitis, and multiple pneumonias has had a productive cough for 2 months. She has had no fever or other systemic symptoms except fatigue. Bronchodilators provide limited symptomatic relief. Two previous courses of antibiotics have produced transient but limited improvement. On physical examination, you note a slender child without clubbing. Her respiratory rate is 28 breaths/min, and faint crackles and wheezes are audible throughout her chest. She has purulent rhinitis with maxillary sinus tenderness. A chest radiograph shows areas of linear atelectasis with thickened airways. Results of sweat chloride testing and direct mutation analysis for cystic fibrosis are negative. Of the following, the MOST appropriate next test in her evaluation is", "options" : "[\"allergy skin testing\", \"echocardiography\", \"high-resolution chest computed tomography scan\", \"nasopharyngeal culture for virus\", \"sinus radiographs\"]", "explanation" : "The girl described in the vignette demonstrates clinical and radiologic signs suggestive of bronchiectasis. Bronchiectasis is a chronic disease of the conducting airways characterized by irreversible dilation of the bronchial tree and manifested clinically by chronic cough with production of thick, often purulent, sputum. Hemoptysis may occur, and fever may be present during infectious exacerbations. Children frequently have anorexia and demonstrate poor weight gain. Clubbing occurs, but it is not universal and is reported in only 2% of affected adults. With very severe disease, the child may develop dyspnea and hypoxemia. Pulmonary function tests can show an obstructive, restrictive, or mixed picture.\n\nPlain radiographs are not specific for bronchiectasis but may demonstrate a wide variety of abnormalities, including increased size and loss of definition of the bronchovascular markings, honeycomb appearance, loss of lung volume of an affected lobe, linear atelectasis, and irregular opacities representing mucus plugs. The currently accepted gold standard test for bronchiectasis is high-resolution computed tomography (HRCT) scan of the chest. Tram (parallel) lines or ring shadows (cross-section or end-on bronchial walls) may be visible on both plain radiography and HRCT. Further, HRCT can show three patterns of bronchiectasis: The cylindrical pattern shows diffuse but regular dilation of the bronchi with abruptly ending bronchial lumen; The varicose pattern also shows dilation of the bronchi, but local constriction results in an irregular outline; The saccular or cystic variation appears as progressive dilation and ballooning of the bronchi, ending in fluid/mucus-filled sacs.\n\nBecause of concern for the amount of radiation exposure with CT scans, recent studies using magnetic resonance imaging instead of HRCT have shown promising results for ongoing monitoring of disease progression.\n\nBronchiectasis is not a disease in itself but rather the end result of several different processes that can be classified as obstructive, infectious, defective host defense/immunologic, and congenital/genetic. In the developed world, the most common cause for bronchiectasis in children is cystic fibrosis. However, in the developing world and among indigenous populations, infectious causes, in particular pertussis, mycobacteria, measles, and pneumococcus, are common. Allergic bronchopulmonary aspergillosis also may lead to bronchiectasis. Airway obstruction, such as from foreign body aspiration or extraluminal compression by vascular rings, has been associated with bronchiectasis. Host defense conditions that have been associated include ciliary dyskinesia (a likely cause for the patient in the vignette), hypogammaglobulinemia, immunoglobulin G subclass deficiency, and impaired cough from neuromuscular disease. Congenital/genetic conditions include entities affecting airway development (Williams-Campbell syndrome [absence of annular cartilage] and Mounier-Kuhn syndrome [tracheobronchomegaly]) and alpha-1-antitrypsin deficiency.\n\nAlthough patients who have bronchiectasis may have asthma or allergies, particularly those who have allergic bronchopulmonary aspergillosis, allergy testing does not usually contribute to the diagnosis or management. Echocardiography may be helpful if there are concerns that airway obstruction from vascular anomalies underlies bronchiectasis, but HRCT still is necessary to confirm the presence of bronchiectasis. Nasopharyngeal cultures for virus would not help explain the chronic course of this patient's disease. Although sinus disease with abnormal-appearing sinus radiographs can be seen with cystic fibrosis, ciliary dyskinesia, and other pathogenic conditions, its presence does not explain or define the underlying pulmonary disease in this patient.\n\nCritique: The girl described in the vignette demonstrates clinical and radiologic signs suggestive of bronchiectasis. Bronchiectasis is a chronic disease of the conducting airways characterized by irreversible dilation of the bronchial tree and manifested clinically by chronic cough with production of thick, often purulent, sputum. Hemoptysis may occur, and fever may be present during infectious exacerbations. Children frequently have anorexia and demonstrate poor weight gain. Clubbing occurs, but it is not universal and is reported in only 2% of affected adults. With very severe disease, the child may develop dyspnea and hypoxemia. Pulmonary function tests can show an obstructive, restrictive, or mixed picture.\n\nPlain radiographs are not specific for bronchiectasis but may demonstrate a wide variety of abnormalities, including increased size and loss of definition of the bronchovascular markings, honeycomb appearance, loss of lung volume of an affected lobe, linear atelectasis, and irregular opacities representing mucus plugs. The currently accepted gold standard test for bronchiectasis is high-resolution computed tomography (HRCT) scan of the chest. Tram (parallel) lines or ring shadows (cross-section or end-on bronchial walls) may be visible on both plain radiography and HRCT. Further, HRCT can show three patterns of bronchiectasis: The cylindrical pattern shows diffuse but regular dilation of the bronchi with abruptly ending bronchial lumen; The varicose pattern also shows dilation of the bronchi, but local constriction results in an irregular outline; The saccular or cystic variation appears as progressive dilation and ballooning of the bronchi, ending in fluid/mucus-filled sacs.\n\nBecause of concern for the amount of radiation exposure with CT scans, recent studies using magnetic resonance imaging instead of HRCT have shown promising results for ongoing monitoring of disease progression.\n\nBronchiectasis is not a disease in itself but rather the end result of several different processes that can be classified as obstructive, infectious, defective host defense/immunologic, and congenital/genetic. In the developed world, the most common cause for bronchiectasis in children is cystic fibrosis. However, in the developing world and among indigenous populations, infectious causes, in particular pertussis, mycobacteria, measles, and pneumococcus, are common. Allergic bronchopulmonary aspergillosis also may lead to bronchiectasis. Airway obstruction, such as from foreign body aspiration or extraluminal compression by vascular rings, has been associated with bronchiectasis. Host defense conditions that have been associated include ciliary dyskinesia (a likely cause for the patient in the vignette), hypogammaglobulinemia, immunoglobulin G subclass deficiency, and impaired cough from neuromuscular disease. Congenital/genetic conditions include entities affecting airway development (Williams-Campbell syndrome [absence of annular cartilage] and Mounier-Kuhn syndrome [tracheobronchomegaly]) and alpha-1-antitrypsin deficiency.\n\nAlthough patients who have bronchiectasis may have asthma or allergies, particularly those who have allergic bronchopulmonary aspergillosis, allergy testing does not usually contribute to the diagnosis or management. Echocardiography may be helpful if there are concerns that airway obstruction from vascular anomalies underlies bronchiectasis, but HRCT still is necessary to confirm the presence of bronchiectasis. Nasopharyngeal cultures for virus would not help explain the chronic course of this patient's disease. Although sinus disease with abnormal-appearing sinus radiographs can be seen with cystic fibrosis, ciliary dyskinesia, and other pathogenic conditions, its presence does not explain or define the underlying pulmonary disease in this patient.\n\nContent Specifications: Know the differential diagnosis of bronchiectasis; Know that high-resolution CT of the chest is useful to diagnose bronchiectasis in a child"}
{"id" : 758, "question_text" : "A full-term newborn was delivered after an uneventful pregnancy to a gravida 2, para 2 woman by normal spontaneous vaginal delivery. His birth weight is 3,150 g. Findings on physical examination are unremarkable except for bilateral ear pits and a small branchial sinus on the left neck with no drainage noted. Of the following, prior to discharge, you are MOST likely to order a(n)", "options" : "[\"complete blood cell count\", \"complete metabolic profile\", \"echocardiogram\", \"head ultrasonography\", \"renal ultrasonography\"]", "explanation" : "The infant described in this vignette has clinical features suggestive of branchio-oto-renal (BOR) syndrome. This autosomal dominant disorder caused by mutations in EYA1 is associated with preauricular pits (70%-80%), branchial cysts or fistulas (30%-60%), and structural renal anomalies (12%-20%). Therefore, renal ultrasonography is recommended in light of the other 2 clinical findings. In addition to these features, individuals with BOR syndrome may have other external ear malformations, and at least 75% have some degree of hearing loss.\n\nOther syndromes typically associated with external ear malformations include CHARGE syndrome (Coloboma of the eye, Heart defects, Atresia of the choanae, Retardation of growth and development, Genital and Renal anomalies, Ear abnormalities), Townes-Brocks syndrome, oculo-auriculo-vertebral spectrum (Goldenhar syndrome), Treacher-Collins syndrome, Nager syndrome, Miller syndrome, and diabetic embryopathy (Item C50, page C-40). A large survey of patients with external ear anomalies, including preauricular pits, demonstrated that almost 30% had concomitant renal anomalies and 92% represented a multiple congenital anomaly syndrome. For individuals with apparent isolated ear anomalies, approximately 11% will be found to have a hidden renal malformation. Therefore, renal ultrasonography must be performed in infants with ear anomalies (including tags, pits, lop ear, cupped ear, microtia or anotia) in addition to any of the following findings: dysmorphic facies, facial asymmetry, ocular or eyelid colobomas, choanal atresia, micrognathia (small jaw), branchial cysts or sinuses, cardiac murmur, limb anomalies, or an imperforate or anteriorly placed anus. Renal ultrasonography should also be performed on an infant with external ear malformations if there is a family history of hearing loss and ear or renal malformations, or if there is a history of maternal diabetes during gestation.\n\nA complete blood cell count or head ultrasonography would not be indicated under these circumstances. A complete metabolic profile might uncover renal dysfunction, but most renal malformations identified in patients with BOR syndrome would not present with renal failure. An echocardiogram would only be indicated if the infant had a cardiac murmur, cyanosis, low oxygen saturation, or other signs of a cardiac defect.\n\nPREP Pearls\n• Infants with external ear malformations have an 11 % risk for also having a structural renal malformation and should be evaluated for other birth defects.\n• Renal ultrasonography should be performed if an infant has external ear malformations plus at least 1 other significant clinical finding.\n\nAmerican Board of Pediatrics Content Specification (s):\n• Recognize that malformed external and middle ears maybe associated with renal anomalies, craniofacial malformations, and inner ear malformations\n\nSuggested Reading:\n• Khoury MJ, Becerra JE, Cordero IF, Erickson JD. Clinical-epidemiological assessment of patterns of birth defects associated with human teratogens: application to diabetic embryopathy. Pediatrics. 1989;84(4):658-665\n• Roth DAE, Hildesheimer M, Bardenstein S, et al. Preauricular skin tags and ear pits are associated with permanent hearing impairment in newborns. Pediatrics. 2008;122(4):e884-e890. doi:10.1542/peds.2008-0606\n• Wang RY, Earl DL, Ruder RO, Graham JM. Syndromic ear anomalies and renal ultrasounds. Pediatrics. 2001;108(2):e32. doi:10.1542/peds.108.2.e32"}
{"id" : 1265, "question_text" : "You are performing the routine examination of a newborn 18 hours after birth. The baby was born at 37 2/7 weeks' gestation by routine vaginal delivery to a 38-year-old gravida 3 para 2 mother who had received good prenatal care since early in the first trimester. The pregnancy was unremarkable and maternal screening was negative for hepatitis B surface antigen, HIV, rubella, rapid plasma reagin, and direct antiglobulin (Coombs) test. Spontaneous rupture of membranes occurred at home 16 hours before delivery and clear fluid was noted. Since the mother had screened positive for group B Streptococcus vaginal colonization at 35 weeks of gestation, she received 5 million units of penicillin G on arrival at the hospital 6 hours before delivery and again 4 hours later. The mother had no fever and no signs of chorioamnionitis. The newborn's vital signs are within normal range and the physical examination reveals no abnormalities. The baby has latched on well for breastfeeding every 2 to 3 hours. One stool and 3 wet diapers have been documented. The parents are both present and are anxious for the newborn to be discharged from the hospital. Of the following, the BEST recommendation for early discharge and follow-up of this newborn is to discharge him", "options" : "[\"after 24 hours, with follow-up care within 24 hours\", \"after 24 hours, with follow-up care within 48 to 72 hours\", \"at 48 hours, with follow-up care within 48 to 72 hours\", \"at 72 hours, with follow-up care within 48 to 72 hours\", \"now, with follow-up care within 24 hours\"]", "explanation" : "All pediatric healthcare providers need to be familiar with the American Academy of Pediatrics (AAP) recommendations for early discharge, follow-up, and management of newborns of mothers with abnormal prenatal laboratory findings. A hospital stay of less than 48 hours after delivery may be appropriate for some healthy term newborns. This newborn with a gestation of more than 37 weeks meets criteria for discharge at or after 24 hours of age, with follow-up within 48 to 72 hours. Although she screened positive for group B Streptococcus vaginal colonization, the mother received adequate intrapartum antibiotic treatment before delivery and both she and the newborn are asymptomatic.\n\nThe newborn-mother dyad in this vignette represents a common risk, group B streptococcal disease, which must be considered in determining early discharge. The US Centers for Disease Control and Prevention and AAP Committee on Fetus and Newborn and Committee on Infectious Diseases have written recommendations addressing the prevention of perinatal group B streptococcal disease and length of hospital stay for healthy term newborns. The recommendations for prevention of group B streptococcal disease include screening, indications for maternal intrapartum antibiotic prophylaxis, and management of neonates. Adequate intrapartum antibiotic prophylaxis is defined as 5 million units of intravenous penicillin or 2 g of intravenous ampicillin or cefazolin administered at least 4 hours before delivery, then 2.5 to 3.0 million units of penicillin G or 1 g ampicillin or cefazolin every 4 hours until delivery.\n\nThe duration of hospital stay for a healthy term newborn and mother should be long enough to identify problems in either, and to ensure that the mother is able to care for herself and her newborn at home. The health of both must be considered, as well as the adequacy of support systems at home and access to follow-up care. Efforts should be made to discharge both simultaneously.\n\nMinimum criteria for discharge of a term newborn after an uncomplicated gestation, labor, and delivery include:\n\n• term gestation (between 37 0/7 and 41 6/7 weeks')\n• normal vital signs\n• no physical abnormalities requiring continued hospitalization\n• regular urination and passage of at least 1 stool spontaneously\n• completion of at least 2 successful feedings\n• no excessive bleeding from circumcision site for at least 2 hours\n• clinical significance of jaundice assessed and managed according to AAP guidelines\n• appropriate evaluation for sepsis in accordance with current guidelines\n• review of maternal and infant screening laboratory tests (syphilis, hepatitis B surface antigen, HIV status, as well as blood type and direct Coombs test if indicated)\n• hepatitis B vaccine administered as indicated by newborn's risk status\n• routine screenings, including metabolic screening, hearing, and pulse oximetry according to hospital protocol and state regulations\n• knowledgeable mother who demonstrates the ability and confidence to provide adequate care for her baby\n• an appropriate car seat (if relevant to family transportation situation)\n• addressing any social and environmental risks\n• accessible health care follow-up planned for both mother and her newborn\n\nThe neonate in this vignette has met all discharge criteria, therefore the infant and mother may be discharged as early as 24 hours after birth. If the neonate is discharged before 48 hours after delivery, examination by a healthcare practitioner should take place within 48 hours.\n\nPREP Pearls\n• Minimum criteria must be met for early discharge of a newborn (< 48 hours after delivery).\n• Adequate intrapartum antibiotic treatment for the prevention of group B streptococcal disease is required to meet early discharge criteria.\n• The hospital stay for a healthy term newborn and mother should be long enough to identify problems in either and to ensure that the mother is able to care for herself and her newborn at home.\n\nABP Content Specifications(s)\n• Plan the early discharge of a newborn infant, including follow-up evaluation\n• Plan the management of a neonate whose mother has abnormal prenatal laboratory findings\n\nSuggested Readings\n• Benitz WE, Committee on Fetus and Newborn. Hospital stay for healthy term newborn infants. Pediatrics. 2015;135(5):948-953. doi: http://dx.doi.org/10.1542/peds.2015-0699.\n• Committee on Infectious Diseases, Committee on Fetus and Newborn. Policy statement: recommendations for the prevention of perinatal group B streptococcal (GBS) disease. Pediatrics. 2011;128(3):611-616. doi: http://dx.doi.org/10.1542/peds.2011-1466.\n• Goyal NK, Fager C, Lorch SA. Adherence to discharge guidelines for late-preterm newborns. Pediatrics. 2011;128(1):62-71. doi: http://dx.doi.org/10.1542/peds.2011-0258.\n• Polin RA, Committee on Fetus and Newborn. Management of neonates with suspected or proven early-onset bacterial sepsis. Pediatrics. 2012;129(5):1006-1015. doi: http://dx.doi.org/10.1542/peds.2012-0541.\n• Shakib J, Buchi K, Smith E, Korgenski K, Young PC. Timing of initial well-child visit and readmissions of newborns. Pediatrics. 2015;135(3):469-474. doi: http://dx.doi.org/10.1542/peds.2014-2329.\n• Verani JR, McGee L, Schrag SJ. Prevention of perinatal group B streptococcal disease, revised guidelines from CDC, 2010. MMWR Morbid Mortal Wkly Rep. 2010;59(RR10):1-32. http://www.cdc.gov/mmwr/preview/mmwrhtml/rr5910a1.htm.\n• Warren JB, Phillipi CA. Care of the well newborn. Pediatr Rev. 2012;33(1):4-18. doi: http://dx.doi.org/10.1542/pir.33-1-4."}
{"id" : 3328, "question_text" : "A 15-year-old patient in your practice has a 10-month history of fatigue, headaches, and diffuse achiness. There has been no night sweats, fever, rash, or joint swellings noted. They live in an urban area in the northeastern United States, and there has been no history of travel. Physical examination shows no abnormalities. Temperature is 37.1°C, pulse rate is 76 beats/min, blood pressure is 112/68 mm Hg, and respiratory rate is 18 breaths/min. Laboratory results show the following: White blood cell count, 9,700/µL (9.7 x 109/L), with 55% neutrophils, 38% lymphocytes, 6% monocytes, and 1% eosinophils; Erythrocyte sedimentation rate, 3 mm/h; C-reactive protein, 0.10 mg/L (0.95 nmol/L); Epstein-Barr virus serology - Viral capsid antigen (VCA) Immunoglobulin G, positive; VCA Immunoglobulin M, negative; Epstein-Barr nuclear antigen, positive; Lyme enzyme immunosorbent assay, 1.27 (negative, < 0.91; equivocal, 0.91-1.09; positive, > 1.09). Of the following, the BEST next step in evaluating this patient's condition is", "options" : "[\"begin treatment with doxycycline\", \"explain that Epstein-Barr virus infection can cause a false-positive Lyme serology\", \"obtain a Western blot for Lyme disease\", \"perform a lumbar puncture to evaluate for central nervous system Lyme disease\", \"repeat the enzyme immunosorbent assay for Lyme\"]", "explanation" : "The diagnosis of Lyme disease requires an understanding of the individual's exposure to the causative agent (Borrelia burgdorferi) or epidemiological risk, the likelihood the symptoms are consistent with Lyme disease, and only then, the results of laboratory testing.\n\nLaboratory testing for Lyme disease consists of an initial screening test (Lyme enzyme immunosorbent assay [EIA) or fluorescent antibody [FA] test) that, if positive or equivocal, should be evaluated by a confirmatory test (Lyme Western blot). The advantage of a screening test is that it is relatively rapid and inexpensive and sufficiently specific that a negative test implies absence of the condition, which in this vignette is Lyme disease. A positive test is, however, not sufficiently sensitive for the condition (ie, there may be a false-positive result) and should be confirmed with the more sensitive Western blot. The Western blot for Lyme antibodies is not more specific than the screening tests. A similar sequence of testing is used in HIV testing with a screening rapid, inexpensive EIA test that, if positive or equivocal, is confirmed with a more sensitive assay (HIV Western blot or nucleic acid detection test).\n\nIn this case, the child lives in an urban area and has not described travel that would potentially expose him to ticks that can transmit Lyme disease. Additionally, the findings of fatigue, headache, and diffuse achiness in the absence of erythema migrans, migratory large joint arthritis, Bell palsy, or other objective clinical features of Lyme disease are not specific for this condition.\n\nObtaining a Western blot for Lyme antibodies would be the next step in addressing the significance of the positive Lyme EIA result. If it were negative, additional evaluation or treatment for Lyme disease would not be indicated in this case. Beginning doxycyline at this time would not be indicated given the uncertainty of the diagnosis. Acute EBV infection may cause a false-positive Lyme EIA result, but the serology in this case points to past, not active, EBV infection. Without further evidence of objective neurological findings suggestive of Lyme disease (eg, cranial neuropathy, paresthesias, aseptic meningitis) or a confirmed serology, a lumbar puncture is not indicated in this case. Repeating the same test is not likely to determine the validity of the result.\n\nIn addition to appreciating the sensitivity and specificity of a test, it is important to understand the concept of a predictive value in interpreting a test result. The positive predictive value represents the likelihood that a patient with a positive test has the condition and the negative predictive value that he does not have the disease. These findings depend on the prevalence of the condition, as well as the sensitivity and specificity of the test,\n\nPREP Pearls\n• A good screening test is a rapid, inexpensive assay and has a good specificity (ie, a negative test is reliable). A positive or equivocal test should generally be confirmed with a more sensitive confirmatory assay. Testing for Lyme or HIV antibodies employs such a system with a screening enzyme immunosorbent assay as the first line evaluation, followed by more sensitive testing of a positive or equivocal result.\n• The positive predictive value represents the likelihood that a patient with a positive test has the condition and the negative predictive value that he does not have the disease. These findings depend on the prevalence of the condition, as well as the sensitivity and specificity of the test.\n\nAmerican Board of Pediatrics Content Specification(s)\n• Understand positive and negative predictive values\n• Understand sensitivity and specificity and how to apply them to test results\n\nSuggested Reading\n• Carvajal DN, Rowe PC. Research and statistics: sensitivity, specificity, predictive values, and likelihood ratios. Pedia tr• Rev. 2010;31(14:511-513. doi:10.1542/pir.31-12-511.\n• Neuspiel DR. Weighing the evidence: positive predictive values. AAP Grand Rounds. 2003:10(3):38. http://aapgrandrounds.aappub/ications.org/ content/10/3/38.extract.\n• Moyer V. Weighing the evidence: SpPl n and SnNOut. AAP Grand Rounds. 2003;9(6):65. http://aapgrandrounds.aappublications.org/ content/9/6/65.1,full."}
{"id" : 329, "question_text" : "A 3-year-old boy presents with a temperature of 39.5°C and a first-time generalized seizure. Over the past 3 days, he has had 8 to 10 loose, liquid stools and abdominal pain. He attends child care, and several other children in the center have been reported to have diarrhea. Of the following, the MOST likely cause of this child's illness is infection with", "options" : "[\"Campylobacter jejuni\", \"rotavirus\", \"Salmonella enteritidis\", \"Salmonella typhi\", \"Shigella flexneri\"]", "explanation" : "Acute onset of fever, abdominal cramps, and diarrhea (often with blood and mucus) over 3 days, as described for the boy in the vignette, are suggestive of a bacterial gastroenteritis. The associated seizure and attendance at a child-care center support the diagnosis of Shigella infection.\n\nCampylobacter jejuni infection can present with fever and similar gastrointestinal symptoms but has not been associated with seizures. In addition, Campylobacter is typically transmitted from contaminated poultry; outbreaks in child-care centers are uncommon.\n\nNontyphoidal Salmonella infections, such as S enteritidis, are acquired from animal reservoirs, including poultry and livestock. Transmission from contaminated meats and eggs is the usual source of outbreaks, although other foods, including ice cream, fruits, and cider, have been implicated. Secondary person-to-person transmission can occur. Transmission of nontyphoidal Salmonella from contact with pet reptiles is another important source of infections. Infection may range from asymptomatic to gastroenteritis with diarrhea, abdominal cramps, and fever. Seizures are not commonly associated with these infections. Salmonella bacteremia can occur in younger children (<1 year old).\n\nS typhi is a solely human pathogen that is rare in the United States. Infection is associated with crowding and poor hygienic conditions. Most cases in the United States are acquired during international travel. Typhoid fever is a protracted illness characterized by fever, constitutional symptoms, abdominal pain and tenderness, hepatosplenomegaly, rose spots on the skin, and changes in mental status.\n\nRotavirus infection is transmitted by the fecal-oral route and can be found on multiple surfaces in child-care centers. Clinically, the illness is characterized by the acute onset of fever and vomiting, with watery diarrhea developing 24 to 48 hours later. Severe infection generally occurs in children 2 years of age and younger.\n\nAmerican Board of Pediatrics Content Specification(s):\n\nDistinguish between Salmonella and Shigella with regard to pathophysiology and symptomatology"}
{"id" : 720, "question_text" : "A 10-year-old boy is at school when his teacher notices that he is staring out the window. She can't get him to stop staring or respond to her, so he is brought to the emergency department. No other children had similar symptoms. On arrival, his physical examination reveals a temperature of 37.2°C, blood pressure of 100/60 mm Hg, heart rate of 85 beats/min, and a respiratory rate of 20 breaths/min. The boy is awake and seems restless. He follows one-step commands (eg, \"take off your shoes\"), but does not follow two-step commands. He knows his name, but not where he is. The remainder of the physical examination findings is unremarkable. Results of computed tomography of the head without contrast, serum sodium and glucose, and serum and urine toxicology testing are normal. As you are completing your examination, the boy's parents arrive and report no known ingestions at home, no history of seizures or headaches, and no similar prior events. The boy is adopted and no family history is known. After 2 hours of observation, he is alert and responding normally to commands, but complains of a headache and vomits. Of the following, the MOST likely diagnosis is", "options" : "[\"acute psychosis\", \"carbon monoxide poisoning\", \"confusional migraine\", \"postictal state\", \"pseudotumor cerebri\"]", "explanation" : "The boy described in the vignette has a confusional migraine. Confusional migraine is a subtype of migraine headache characterized by the abrupt onset of an altered level of consciousness. The child appears disoriented and is sometimes agitated or combative; the symptoms last hours before recovery. Often, the episode is followed by a headache. A prior history of headaches or a family history of migraine headaches supports this diagnosis, but other causes of altered mental status need to be considered in the acute setting.\n\nThe boy in the vignette did not have a history of a stressful trigger that may have precipitated an acute reactive psychosis. Other causes of acute psychosis in a 10-year-old child include rare inborn errors of metabolism, porphyria, or toxin/medication exposures, but the boy in the vignette had no history consistent with this. Acute psychosis in this age rarely occurs because of childhood-onset schizophrenia.\n\nThe child also had no history of carbon monoxide poisoning and no other children from the classroom were affected. Pseudotumor cerebri presents with headache but not an altered level of consciousness. He had no history of seizures that would have caused a postictal state. Other causes of altered level of consciousness such as head injury, meningitis, or encephalitis were not suggested by the clinical presentation.\n\nThe initial evaluation of altered level of consciousness depends mostly on the history and physical examination findings. Once alternate causes have been ruled out, and especially with a history of prior headaches and a family history of migraines, confusional migraine can be considered. Confusional migraines recur, and on subsequent presentations, an extensive evaluation for alternate causes is not always necessary.\n\nConfusional migraine is treated with the same approach as other migraine headaches. During the episode, acetaminophen, ibuprofen, fluids, and caffeine can help speed recovery. If episodes recur frequently or impair functioning, a prophylactic medication such as cyproheptadine can decrease the frequency and severity of both confusional migraine and migraine headache.\n\nPREP Pearls\n• Confusional migraine is characterized by an abrupt onset of altered consciousness.\n• Initial evaluation of altered level of consciousness could include serum chemistries, toxicology, infectious evaluation, or central nervous system imaging based on the history and physical examination.\n\nAmerican Board of Pediatrics Content Specification(s):\n• Know the common causes of an altered level of consciousness\n• Plan the initial phase of evaluation for an altered level of consciousness\n\nSuggested Reading:\n• Blume HK. Pediatric headache. Pediatr Rev. 2012;33(12):562-576. doi: 10.1542/pir.33-12-562\n• Bechtel K. Acute mental status change due to acute confusional migraine. Pediatr Emerg Care. 2004;20(4):238-241"}
{"id" : 3606, "question_text" : "A 2-year-old previously healthy boy was brought to the emergency department after several hours of decreased activity. He has a temperature of 37°C, heart rate of 60 beats/min, respiratory rate of 30 breaths/min, and blood pressure of 70/40 mm Hg. Oxygen saturation by pulse oximetry is 100% on room air. He is moaning and appears lethargic. He does not open his eyes and withdraws nonpurposefully to painful stimuli. His pupils are 2 mm equal and reactive. His muscle tone is decreased, and deep tendon reflexes are 2+ throughout. Cough and gag reflexes are present. Mucous membranes are moist. His extremities are cool with capillary refill time of 4 seconds. His heart rate is bradycardic with a regular rhythm. His abdomen is soft, nontender, and nondistended. Serum electrolyte levels are normal, with a serum osmolality of 290 mOsm/kg (290 mmol/kg). Of the following, the substance MOST likely ingested by the boy is", "options" : "[\"clonidine\", \"diphenhydramine\", \"ethylene glycol\", \"methylphenidate\"]", "explanation" : "Correct Answer: A\nThe boy in this vignette has lethargy, bradycardia, and hypotension. Of the response choices, the most likely substance ingested is clonidine.\n\nPediatric poisoning is an important public health problem. In 2014, the National Poison Data System of the American Association of Poison Control Centers reported more than 1 million toxic exposures and 88 poisoning fatalities in children and adolescents (younger than 20 years). Poisoning exposures have been decreasing since 2008 in children aged 5 years and younger, possibly due to safer packaging and public health campaigns. Clinicians who suspect a toxic exposure in a child should investigate the identity, timing, dose, quantity, and route of administration of the agent. A thorough history should be obtained, including information regarding other agents in the environment and any possible coexisting trauma. Many agents can cause respiratory depression and loss of airway protective reflexes, as well as hemodynamic collapse, including hypotension, hypertension, cardiac depression, arrhythmias, and cardiac arrest. Immediate management of pediatric poisoning includes assessment and treatment of airway, breathing, and circulation problems. Consultation with the poison control center and a toxicologist should be sought early. Discussion of the signs and symptoms can elucidate common toxidromes, identify co-ingestions, and direct management specific to the agent. Common pediatric toxic syndromes are shown in Item C50A.\n\nClonidine is a central α2-agonist that is administered orally or transdermally as an antihypertensive and in the treatment of opioid and alcohol withdrawal. Other reported uses include migraine headaches, anxiety disorder, and attention-deficit/hyperactivity disorder. Clonidine binds to presynaptic α2-adrenergic receptors in the central nervous system to decrease the release of norepinephrine, attenuating neurotransmission centrally and decreasing sympathetic outflow peripherally. This leads to sedation and a decrease in vascular tone, inotropy, and chronotropy. In therapeutic dosage, effects of clonidine occur relatively rapidly. The hypotensive and sedative effects occur within the first 30 to 60 minutes after ingestion. Peak plasma concentrations are reached within 3 to 5 hours, and the plasma half-life is 12 to 16 hours. Within the recommended dose range, clonidine is a generally safe medication in both adults and children. However, due to its widespread usage and relatively small pill size, it is a common source of accidental and intentional overdoses. In fact, buprenorphine and clonidine account for the most hospitalizations among poisoned children.\n\nAs described in the vignette, the toxidrome of a clonidine overdose includes lethargy, hypotension, and bradycardia. The clinical picture is similar to that of opioids and barbiturates. Pupillary constriction may be present but not in all cases. Agents causing bradycardia and hypotension are listed in Item C50B. Clonidine has some α1-agonist activity, so increased vascular tone and hypertension can be seen early after ingestion. Diphenhydramine is an antihistamine that generally causes an anticholinergic toxidrome in overdoses, which can be remembered as \"mad as a hatter, dry as a bone, hot as a hare, red as a beet, and blind as a bat.\" It can cause sedation and in severe cases hypotension, although it usually causes hypertension. Diphenhydramine overdose is not likely in this case because it causes tachycardia and pupillary dilation. Methylphenidate toxicity is an unlikely cause for the symptoms in this child because it results in tachycardia, hypertension, and pupillary dilation. Ethylene glycol can also cause altered mental status and hemodynamic instability, but in severe cases, an elevated serum osmolality and an osmolal gap is present.\n\nTreatment of clonidine overdose, as in any poisoning, starts with addressing any problems with the airway, breathing, and circulation. Respiratory depression, apnea, hypotension, and bradycardia can occur in severe overdoses. Thus, endotracheal intubation and mechanical ventilation is sometimes required, as well as fluid resuscitation, inotropes, and vasopressors. Large-bore intravenous access, continuous electrocardiogram monitoring, and pulse oximetry are required. Consultation with the poison control center should be obtained. Activated charcoal should be administered early in severe overdoses. Endotracheal intubation should be performed prior to charcoal administration if there is significant lethargy. A careful skin examination should be performed to identify any existing medication patch (children occasionally mistake medication patches for stickers). Syrup of ipecac is contraindicated. Although it is not a widely accepted therapy, naloxone can be effective in treating respiratory and central nervous system depression in clonidine overdose. Any child with a symptomatic clonidine overdose should be observed in the emergency department or the intensive care unit until symptoms have resolved and at least one half-life has elapsed. Social work consultation should also be obtained to assess the family environment.\n\nPREP Pearls\n• Clonidine and buprenorphine are the most common agents causing hospitalization due poisoning in children.\n• Clonidine is an α2-agonist that causes low blood pressure, bradycardia, and sedation.\n• Early management of the poisoned child should include assessment and treatment of problems with airway, breathing, and circulation, as well as early consultation with the poison control center and/or a toxicologist.\n\nABP Content Specifications(s)\n• Recognize the signs and symptoms of ingestion of medications that might cause hypotension, and manage appropriately\n\nSuggested Readings\n• Ahmad SA, Scolnik D, Snehal V. Use of naloxone for clonidine intoxication in the pediatric age group: case report and review of the literature. Am J Ther. 2015;22(1):e14-e16. doi:10.1097/MJT.0b013e318293b0e8.\n• Hetterich N, Lauterbach E, Stürer A. Toxicity of antihypertensives in unintentional poisoning of young children. J Emerg Med. 2014;47(2):155-162. doi:10.1016/j.jemermed.2014.02.006.\n• Lovegrove MC, Mathew J, Hampp C, Governale L, Wysowski DK, Budnitz DS. Emergency hospitalizations for unsupervised prescription medication ingestions by young children. Pediatrics. 2014;134(4):e1009-e1016. doi:10.1542/peds.2014-0840.\n• Toce MS, Burns MM. The poisoned pediatric patient. Pediatr Rev. 2017;38(5):207-220. doi:10.1542/pir.2016-0130.\n• Wang GS, Le Lait MC, Heard K. Unintentional pediatric exposures to central alpha-2 agonists reported to the National Poison Data System. J Pediatr.2014;164(1):149-152. doi:10.1016/j.jpeds.2013.08.038."}
{"id" : 2763, "question_text" : "A previously healthy, 16-month-old girl is brought to the emergency department by ambulance for burns on her face and mouth sustained from drinking a lye solution that her mother uses to make soap. The girl's mother found her crying in great pain with redness and swelling around her mouth. She rinsed the affected area with water and called 911. In the emergency department the girl's vital signs include a temperature of 37.5°C, heart rate of 165 beats/min, respiratory rate of 38 breaths/min, and oxygen saturation of 98% in room air. On physical examination she has significant facial irritation and lip edema, vocal hoarseness, drooling, nasal flaring, and audible stridor. The remainder of her physical examination findings are unremarkable. Of the following, the BEST next step in this girl's management is", "options" : "[\"chest radiography\", \"endotracheal intubation\", \"esophagogastroduodenoscopy\", \"nasogastric tube placement\"]", "explanation" : "Correct Answer: B\nThe girl in the vignette ingested lye, a caustic alkali, and is displaying signs and symptoms of upper airway edema (mouth and lip swelling, vocal hoarseness) and impending airway compromise (drooling, stridor). The best next management step is endotracheal intubation to secure her airway.\n\nCaustic ingestions immediately affect the eyes, skin, airway, and gastrointestinal tract. Evidence of a significant ingestion includes swelling of the tongue and mouth, drooling, and vomiting. Direct alkali contact with tissues during swallowing or subsequent emesis causes edema, which often involves the upper airway. Edema of the upper airway results in stridor, respiratory distress, and vocal hoarseness. Because of the risk of rapid progression of upper airway and oropharyngeal edema, when children develop an inability to handle oral secretions or voice changes, clinicians should have a high index of suspicion for impending airway compromise. Any airway compromise must be immediately addressed, as progression to complete obstruction can be life-threatening. Chest radiography may be needed to assess for air leak from an esophageal or tracheal perforation, but stabilization of the airway should occur before a chest radiograph is obtained.\n\nCaustic-induced injuries in children represent a serious public health issue in the United States. Thousands of children with caustic ingestions are hospitalized annually, with most injuries resulting from the natural exploratory behavior of young children. Rarely, pediatric ingestions are the result of intentional inflicted injury or attempted selfharm.\n\nAlkalis are commonly found in household cleaning products. Sodium hydroxide is the active ingredient in alkalinebased cleaners such as drain cleaner, dishwasher detergent, and many kitchen- or oven-cleaning products. Upon ingestion, the alkaline agent bonds with tissue proteins, leading to liquefaction necrosis. Because of a strong surface tension, alkaline fluid will stay on the tissue for a prolonged period (in contrast with acids). As such, tissue injury is ongoing until the alkali is neutralized, which can take several days. Thus, compared to acid ingestions, alkali ingestions tend to result in more significant injury and tissue damage.\n\nInitial evaluation and resuscitation efforts after caustic ingestion should focus on injuries to the oropharynx and airway. Subsequent care should address any skin and/or eye injuries from splashes or spills. Affected clothing should be removed and the skin completely exposed. Any areas of skin with suspected or confirmed alkali contact should undergo high-volume irrigation with water. If the eyes are affected, saline irrigation should begin immediately, and urgent consultation with an ophthalmologist is indicated.\n\nGastric decontamination with activated charcoal or induced vomiting are contraindicated in all caustic ingestions. Activated charcoal does not absorb alkali agents and residual charcoal is likely to obscure subsequent endoscopic visualization of esophageal or gastric erosions. Emesis can increase alkali exposure and cause additional injury. There is no evidence to support the efficacy of blind nasogastric-tube insertion to maintain esophageal patency, and there is a high risk of complications (eg, esophageal rupture).\n\nBurns on the cheeks, lips, or in the oropharynx may not accurately reflect the presence or scope of injury to the lower gastrointestinal tract. Historically, esophagogastroduodenoscopy (EGD) was performed in all children with unintentional alkali ingestion; however, current guidelines support a watchful observation period for asymptomatic or minimally symptomatic children. Children with vomiting and drooling, or with stridor alone are more likely to have severe injuries and warrant evaluation with EGD. When indicated, endoscopy is usually performed within the first 48 hours after the ingestion; identified lesions are graded by a standard scoring system reflecting the degree of tissue damage and the prognosis.\n\nThere is no evidence to support routine administration of proton-pump inhibitors or antibiotic agents in children with caustic ingestions. Antibiotics may be indicated in the event of direct lung injury, sepsis, or if perforation is suspected; however, no prospective trial has evaluated the isolated use of antibiotics (in the absence of documented infection) with respect to the prevention of perforation, or prevention of esophageal strictures. There are conflicting data regarding the use of corticosteroids after caustic ingestion with some evidence suggesting benefit in children with respiratory symptoms. The only other medications that have been studied specifically for treatment of caustic alkali ingestions are sucralfate and mitomycin C; sparse data suggest that each of these medications may have some effectiveness in the prevention of esophageal stricture formation.\n\nPREP Pearls\n• Alkali ingestions cause tissue liquefaction necrosis resulting in greater injury compared to acid ingestions.\n• Symptoms of airway injury due to alkali ingestion, including drooling, mouth pain, lip swelling, and stridor, should raise concern for impending airway compromise; a secure airway should be ensured.\n• Symptomatic, clinically stable children should have esophagogastroduodenoscopy performed within 48 hours of alkali ingestion; current guidelines support a watchful observation period for asymptomatic or minimally symptomatic children.\n\nABP Content Specifications(s)\n• Recognize the signs and symptoms of ingestion of a caustic substance, and manage appropriately\n\nSuggested Readings\n• Ali Zargar S, Kochar R, Mehta S, Mehta SK. The role of fiberoptic endoscopy in the management of corrosive ingestion and modified endoscopic classification of burns. Gastrointest Endosc. 1991;37(2):165-169. doi:10.1016/s00165107(91)70678-0.\n• Bird JH, Kumar S, Paul C, Ramsden JD. Controversies in the management of caustic ingestion injury: an evidence-based review. Clin Otolaryngol. 2017;42(3):701-708. doi:10.1111/coa.12819.\n• Hoffman RS, Burns MM, Gosselin S. Ingestion of caustic substances. N Engl J Med. 2020;382:1739-1748. doi:10.1056/NEJMra1810769.\n• Kay M, Wyllie R. Caustic ingestions in children. Curr Opin Pediatr. 2009;21(5):651-654. doi:10.1097/MOP.0b013e32832e2764.\n• Ricca, RL, Drugas GT. Esophageal caustic injury. In: McInerny TK, Adam HM, Campbell DE, DeWitt TG, Foy JM, Kamat DM, eds. American Academy of Pediatrics Textbook of Pediatric Care. American Academy of Pediatrics; 2021:chap 359. Accessed September 1, 2022. Pediatric Care Online.\n• Sheridan DC, Hughes A, Horowitz BZ. Pediatric ingestions: new high-risk household hazards. Pediatr Rev. 2021;42(1):2-10. doi:10.1542/pir.2019-0309."}
{"id" : 1912, "question_text" : "An 8-year-old boy is seen for a health supervision visit as a new patient. He feels well and is doing well in school. His parents have no concerns. His father had a myocardial infarction at the age of 45 years. The family shares that they are meticulous in maintaining a heart healthy diet and that they engage in physical activity as a family. All of the boy's growth parameters are at the 45th percentile, and the remainder of his physical examination findings are unremarkable. In addition to continuing the heart healthy diet and exercise, the BEST next step in management is to perform a comprehensive assessment of serum lipid and lipoprotein levels", "options" : "[\"now\", \"at age 18 years\", \"at age 25 years\", \"at age 40 years\"]", "explanation" : "The National Cholesterol Education Program's Expert Report (1992) made recommendations for screening and treating hypercholesterolemia in children. These guidelines were updated in 2011 by the Expert Panel on Integrated Guidelines for Cardiovascular Health and Risk Reduction in Children and Adolescents of the National Heart, Lung, and Blood Institute. Important changes have been made in concert with the increasing incidence of obesity and other cardiovascular risks in children.\n\nThe screening process for lipid disorders is based on age, other cardiovascular health risks, and family history. A positive family history is defined as early coronary heart disease in a first-degree relative. In this context \"early\" is considered to be age 55 years or younger for a man and age 65 years or younger for a woman. Diseases included are heart attack, angina, coronary intervention (either surgical or percutaneous catheterization laboratory intervention), stroke, or sudden cardiac death. Such a family history is an independent risk factor for the development of cardiovascular disease.\n\nRecent studies have demonstrated a statistically significant association between high lipid levels in later childhood and early adult cardiovascular disease. Additionally, it is normal for lipid levels to decrease during puberty. Therefore, children later in childhood but before puberty should have universal screening of their lipid levels. The guidelines now state that universal screening should take place between the ages of 9 and 11 years and then again at age 17 to 21 years. No routine screening should take place at any other age group in pediatrics. Children with a positive family history or a moderate- or high-risk medical condition (Item C112) should be screened at ages 2 to 8 years and 12 to 16 years with 2 fasting lipid profiles. (See suggested reading 1 for more information.) The average of these 2 results should be used to dictate next steps.\n\nMost people with atherosclerotic disease are asymptomatic. In more extreme cases, there can be physical examination findings suggestive of lipid deposition, such as corneal arcus and planar, tuberous, or tendinous xanthomas. Xanthomas, which are lipid deposition over tendons, are yellow-orange, nonpainful, and common on the elbows and knees.\n\nThe lipoprotein analysis for the boy in this vignette should be performed now because of his significant family history. It is not appropriate to wait to make this assessment.\n\nPREP Pearls\n\nChildren and adolescents with a family history of premature cardiovascular disease secondary to hypercholesterolemia need a lipoprotein analysis.\n\nUniversal screening for lipid disorders should occur between 9 and 11 years of age.\n\nMost patients with hypercholesterolemia are asymptomatic.\n\nMOCA-Peds Objective\n\nRecognize the risk factors for familial hyperlipidemia.\n\nABP Content Specifications(s)/Content Area\n\nKnow the risk factors associated with coronary artery disease\n\nRecognize the clinical features associated with hypercholesterolemia/hyperlipidemia, and evaluate appropriately\n\nKnow the risk factors associated with hypercholesterolemia/hyperlipidemia\n\nSuggested Readings\n\nExpert Panel on Integrated Guidelines for Cardiovascular Health and Risk Reduction in Children and Adolescents; National Heart, Lung, and Blood Institute. Expert Panel on Integrated Guidelines for Cardiovascular Health and Risk Reduction in Children and Adolescents: summary report. Pediatrics. 2011:128(suppl 5):S213-S255. doi: http://dx.doi.org/10.1542/peds.2009-2107C.\n\nNCEP Expert Panel on Blood Cholesterol Levels in Children and Adolescents. National Cholesterol Education Program (NCEP): highlights of the report of the Expert Panel on Blood Cholesterol Levels in Children and Adolescents. Pediatrics. 1992;89(3):495-501.\n\nStarc TJ, Deckelbaum RJ. Evaluation of hypercholesterolemia in childhood. Pediatr Rev. 1996;17(3):94-97. doi: http://dx.doi.org/10.1542/pir.17-3-94."}
{"id" : 1624, "question_text" : "You are reviewing the medical records of a patient who is entering your practice. This 2-week-old male newborn was born at term to a 20-year-old, gravida 1 para 1 mother who did not receive prenatal care. At delivery, the baby was found to have micrognathia, glossoptosis, and a cleft palate consistent with Pierre Robin sequence. He was cared for in the neonatal intensive care unit, and at the time of discharge he was being fed breast milk through a nasogastric tube. Notes report that he is able to breathe comfortably and maintain adequate oxygen saturation when he is positioned prone or on his side. Genetics consultation and testing during his hospital stay confirm Stickler syndrome.\n\nOf the following, the condition MOST commonly associated with this syndrome, other than those conditions already noted, is", "options" : "[\"autism spectrum disorder\", \"cardiac anomaly\", \"intestinal malrotation\", \"renal anomaly\", \"vision impairment\"]", "explanation" : "Stickler syndrome is a group of genetic conditions characterized by the Pierre Robin sequence (cleft palate, glossoptosis, and micrognathia or retrognathia) and severe myopia or other ocular abnormalities. Many patients with Stickler syndrome also have sensorineural hearing loss or skeletal abnormalities including hypermobility, scoliosis, or early arthritis. Stickler syndrome is not associated with an increased risk of autism spectrum disorder or cardiac, intestinal, and renal anomalies.\n\nMandibular abnormalities, including the micrognathia or retrognathia seen in patients with Pierre Robin sequence, can lead to airway obstruction and feeding difficulties. Treatment strategies depend on factors including individual anatomy and physiology, palatal structure, and noncraniofacial comorbidities. In some infants, airway compromise can be expected to improve as they grow; treatment may therefore include temporary interventions such as nasal airways, palatal obturators, and/or feeding through a nasogastric or gastrostomy tube. In other patients, glossopexy (surgical adhesion of the tongue to the lower lip to prevent posterior malpositioning), mandibular distraction osteogenesis (in which the mandible is surgically displaced anteriorly), or tracheostomy may be indicated.\n\nPatients with Pierre Robin sequence typically also have palatal abnormalities including orofacial clefts. Clefts can involve the lip, philtrum, nose, gums, hard or soft palate, or uvula. They can be isolated or associated with a syndrome. Genetic causes of orofacial clefts include trisomy 13, velocardiofacial syndrome (22q11.2 deletion), Smith-Lemli-Opitz syndrome, Treacher Collins syndrome, and Stickler syndrome.\n\nChildren with palatal clefts are at increased risk for chronic middle ear effusion and conductive hearing loss associated with eustachian tube dysfunction. Although infants with isolated labial clefts may be able to breastfeed, infants with palatal clefts cannot achieve effective suction and therefore typically require feeding by bottle, oftentimes with special bottle nipples. Children with orofacial clefts are at increased risk of language and articulation disorders; this outcome tends to be improved with earlier surgical correction. Lip clefts are typically repaired before 6 months of age, with palatal cleft repair occurring around 1 year of age.\n\nThe infant in this vignette has Stickler syndrome and demonstrates common complications of the associated craniofacial abnormalities, including airway obstruction and feeding difficulties. He should be assessed by a pediatric ophthalmologist for evaluation of myopia or other ocular issues. If he has myopia, corrective lenses will aid his visual development. Surgical intervention may be indicated if he has glaucoma or cataracts. The patient should also have a thorough audiologic evaluation given the increased risk of hearing impairment in children with craniofacial anomalies.\n\nPREP Pearls\n• Stickler syndrome is a group of genetic conditions characterized by Pierre Robin sequence (cleft palate, glossoptosis, and micrognathia or retrognathia) and severe myopia or other ocular abnormalities, as well as potential sensorineural hearing loss and skeletal abnormalities.\n• Children with mandibular abnormalities often have airway obstruction and feeding difficulties.\n• Children with palatal clefts are at increased risk of eustachian tube dysfunction, middle ear effusion, and conductive hearing loss. They may also experience feeding and language difficulties.\n\nABP Content Specifications(s)\n• Plan the appropriate management of a cleft palate in patients of various ages\n• Recognize conditions commonly associated with cleft palate\n• Recognize the clinical findings associated with cleft palate, including submucous cleft and ear sequelae of poor eustachian tube function\n• Recognize the clinical findings associated with mandibular abnormalities, and manage appropriately\n\nSuggested Readings\n• Abbott MA. Cleft lip and palate. Pediatr Rev. 2014;35(5):177–181. doi: http://dx.doi.org/10.1542/pir.35-5-177.\n• Butow KW, Naidoo S, Zwahlen RA, Morkel JA. Pierre Robin sequence: subdivision, data, theories, and treatment–part 4: recommended management and treatment of Pierre Robin sequence and its application. Ann Maxillofac Surg. 2016;6(1):44–49.\n• Robin NH, Moran RT, Ala-Kokko L. Stickler syndrome. GeneReviews. 2014. https://www.ncbi.nlm.nih.gov/books/NBK1302/."}
{"id" : 2404, "question_text" : "A 10-year-old boy with attention-deficit/hyperactivity disorder-combined type and tics arrives 20 minutes late for his routine follow-up visit. His mother apologizes and explains that they were late because the boy had to perform his \"leaving the house ritual.\" Whenever they leave home, the boy must tap every door 10 times and check that it is locked. He must complete the process in a particular order and, if interrupted, becomes very upset and insists on starting from the beginning. Consequently, the family has been staying home more frequently. In addition, the boy's bedtime has recently become later because he repeats the same process before going to sleep. The boy admits that he frequently worries about whether the doors are locked when he is at home, at school, or in public, and checking the doors makes him feel better temporarily. He insists that he is \"keeping his family safe.\" Before the current ritual, the boy had another bedtime routine that had to be followed precisely. He has always been particular about his belongings being touched or moved. The boy's physical examination findings are unremarkable. Of the following, the BEST next step in this boy's care management is to", "options" : "[\"advise the family to accommodate the boy's ritual and ensure they allow time for it\", \"discuss treatment with a selective serotonin reuptake inhibitor\", \"provide reassurance that these behaviors are common in children with attention-deficit/hyperactivity disorder\", \"refer him for cognitive behavioral therapy that incorporates exposure and response prevention\"]", "explanation" : "The boy in the vignette has obsessive-compulsive disorder (OCD). The best next step in his care is referral for cognitive behavioral therapy with exposure and response prevention. The boy is exhibiting recurrent and persistent thoughts, worries, and urges resulting in repetitive behaviors or mental acts aimed at reducing distress. These obsessions and compulsions are time consuming and distressing and cause significant functional impairment (eg, work, social). To diagnose OCD, the symptoms cannot be better explained by another mental disorder (eg, generalized anxiety disorder, major depressive disorder, eating disorder) and are not the result of substances such as medications or illicit drugs. Symptoms of OCD may fall under several themes, including cleaning, forbidden thoughts (sexual or religious), symmetry (repeating, ordering, counting), and hoarding. In contrast to adults with OCD, children may not recognize that these recurring obsessions and behaviors are excessive or unreasonable.\n\nThe first line of treatment for mild to moderate OCD in children is cognitive behavioral therapy. Cognitive behavioral therapy that incorporates exposure and response prevention is the most efficacious. This therapy involves exposing the individual to a trigger for their obsessive thoughts with focused practice in refraining from engaging in the associated repetitive or compulsive behavior.\n\nSelective serotonin reuptake inhibitors are effective for the treatment of children with moderate to severe OCD. Prescribers and families need to be aware of potential side effects of these medications, including suicidal ideation and development of behavioral activation or mania. The dose should be low initially and increased slowly. The physician should refer the boy in the vignette for cognitive behavioral therapy before considering treatment with a selective serotonin reuptake inhibitor.\n\nObsessive-compulsive disorder occurs in 1% to 3% of children and adolescents; the peak age of diagnosis is between the ages of 9 and 10 years. Many children have symptoms that go unrecognized for several years. The child may not be able to communicate their feeling of distress nor identify its cause, and the impairment may be seen only at home. Comorbid conditions (eg, attention-deficit/hyperactivity disorder, Tourette syndrome, and autism spectrum disorder) are frequently seen in childhood-onset OCD; these occur more often in boys. This boy's behaviors are better explained by OCD than by his attention-deficit/hyperactivity disorder, which is a separate comorbid diagnosis.\n\nThere is an increased prevalence of OCD in first-degree relatives of children diagnosed with OCD; however, the condition occurs sporadically for many children. Concordance in monozygotic twins is not 100%, indicating a combination of genetic and nongenetic etiologic factors. The rate of remission is significantly higher in cases of childhood-onset OCD than in cases of adult-onset OCD.\n\nMany children with OCD experience increased irritability and meltdowns; this often leads parents and caregivers to accommodate the child's compulsions, which reinforces the OCD behaviors. The accommodations required are dependent on the obsession. For the boy in the vignette, accommodations include providing enough time for him to complete his ritual and staying home to avoid the process. Advising the boy's mother to accommodate his compulsive behaviors would not effectively treat his OCD and may result in a worse outcome and decreased likelihood of remission. It is important to advise parents and caregivers to not make accommodations and to limit verbal reassurance regarding the compulsive behavior. Active involvement of parents/caregivers in cognitive behavioral therapy is encouraged.\n\nPediatricians play an active role in the identification and coordination of care for multiple mental health conditions, including OCD. Administering a generalized mental health screening tool is encouraged when concerns are brought up by parents or if concerning behavioral changes are reported (eg, decreased interest in friends and activities, decline in school performance).\n\nSuggested Reading(s)\nAmerican Psychiatric Association. Obsessive-compulsive disorder. In: The Diagnostic and Statistical Manual of Mental Disorders. 5th ed. American Psychiatric Publishing; 2013: 260-263.\nGeller DA, Homayoun S, Johnson G. Developmental considerations in obsessive compulsive disorder: comparing pediatric and adult-onset cases. Front Psychiatry. 2021;12:678538. doi:10.3389/fpsyt.2021.678538\nSarvet B. Childhood obsessive-compulsive disorder. Pediatr Rev. 2013;34(1):19-27. doi:10.1542/pir.34-1-19\nAdams H. Tics. Point of Care Quick Reference. Pediatric Care Online. American Academy of Pediatrics; 2022. Pediatric Care Online\n\nContent Domain\nMental health\n\nABP Content Specification(s) / Content Area(s)\nRecognize the clinical findings associated with obsessive-compulsive disorder in patients of various ages, and manage appropriately\nRecognize the various environmental and biological contributors to the development of obsessive-compulsive disorder"}
{"id" : 1987, "question_text" : "A 6-year-old girl is seen in April for evaluation of a persistent runny nose and cough. These symptoms have been present for several weeks, throughout the day and night. She reports frequent sneezing, itchy ears and throat, and cough. Her father hears her \"sniff\" frequently. The girl appears well and has no fever. Her vital signs are normal for age. Her nasal turbinates are pale and edematous, and she has cobblestoning of her posterior pharynx. Her lungs are clear and equal on auscultation bilaterally, with good air movement. Of the following, the MOST appropriate treatment for her condition is", "options" : "[\"fluticasone nasal spray\", \"oral diphenhydramine\", \"oral prednisolone\", \"phenylephrine nasal spray\"]", "explanation" : "The girl in this vignette has allergic rhinitis. First-line pharmacologic treatment for allergic rhinitis includes an intranasal corticosteroid spray or a second-generation oral antihistamine. First-generation sedating oral antihistamines, oral steroids, and intranasal decongestants should not be used.\n\nAllergic rhinitis is an IgE-mediated hypersensitivity reaction that causes inflammation of the nasal passages and manifests as nasal congestion, rhinorrhea, sneezing, nasal itching, and/or postnasal discharge. Patients may also note itching of the eyes or throat, headaches, difficulty concentrating, nighttime cough, and disrupted sleep. Allergic rhinitis occurs in sensitized individuals in response to a specific allergen. Depending on the triggering allergen(s), it may have a seasonal or perennial pattern. Allergic rhinitis is common and the incidence in the United States is rising; one study found that more than 40% of children are diagnosed with allergic rhinitis before the age of 6 years. Allergic rhinitis is most common in children with a personal or family history of atopy (eg, asthma, atopic dermatitis, or allergy).\n\nChildren with allergic rhinitis often have pale, bluish nasal mucosa, boggy and erythematous nasal turbinates, pharyngeal cobblestoning, nasal obstruction, or clear nasal discharge. Allergic shiners and a transverse nasal crease may accompany these findings. The differential diagnosis for allergic rhinitis includes infectious rhinitis, sinusitis, nasal foreign body, and structural abnormalities of the nasal passage.\n\nTreatment of allergic rhinitis begins with the avoidance or reduction of allergen exposure. Nasal saline irrigation or spray may alleviate mild symptoms. Intranasal corticosteroid sprays are effective at reducing symptoms of allergic rhinitis with limited adverse effects and are therefore recommended as first-line treatment, although some children do not tolerate intranasal administration because of fear or discomfort. Oral antihistamines are also efficacious. First-generation antihistamines (eg, diphenhydramine) should be avoided because of their sedating adverse effects. Second-generation antihistamines (eg, loratadine, cetirizine) lack these adverse effects and are therefore the preferred oral agent. Intranasal antihistamines (eg, azelastine or olopatadine) and intranasal cromolyn have minimal adverse effect profiles, but are less effective than intranasal steroids; these drugs are preferred over steroids by some families.\n\nIntranasal decongestants work by vasoconstricting the nasal mucosa and can quickly reduce symptoms of allergic rhinitis. They are not recommended, however, because they lead to downregulation of α-adrenergic receptors and can cause rebound nasal congestion. Although oral corticosteroids usually eliminate symptoms of allergic rhinitis, they have significant adverse effects and are not recommended except in extremely severe and refractory cases.\n\nPREP Pearls\n\nAllergic rhinitis is an IgE-mediated hypersensitivity reaction that causes inflammation of the nasal passages and manifests as nasal congestion, rhinorrhea, sneezing, nasal itching, and/or postnasal discharge. Patients may also note itching of the eyes or throat, headaches, difficulty concentrating, nighttime cough, and disrupted sleep.\n\nTreatment of allergic rhinitis begins with avoidance or reduction of allergen exposures. First-line pharmacologic treatment includes intranasal corticosteroid sprays and/or second-generation oral antihistamines.\n\nABP Content Specifications(s)/Content Area\n\nRecognize the common characteristics of allergic rhinitis, and manage appropriately\n\nFormulate a differential diagnosis of chronic rhinitis\n\nSuggested Readings\n\nMahr TA, Sheth K. Update on allergic rhinitis. Pediatr Rev. 2005;26(8):284-289. doi: 10.1542/pir.26-8-284.\n\nSeidman MD, Gurgel RK, Lin SY, et al. Clinical practice guideline: allergic rhinitis. Otolaryngol Head Neck Surg.2015;152(1 suppl):S1-S43. doi: 10.1177/0194599814561600.\n\nWallace DV, Dykewicz MS, Bernstein DI, et al. The diagnosis and management of rhinitis: an updated practice parameter. J Allergy Clin Immunol. 2008;122(2 suppl):S1-S84. doi: 10.1016/j.jaci.2008.06.003."}
{"id" : 3574, "question_text" : "A 16-year-old adolescent girl is seen for evaluation of excessive daytime sleepiness for the past year. Despite sleeping an average of 8 to 10 hours each night, she states that she is constantly falling asleep during the school day and sometimes feels drowsy when out with friends on the weekends. Her academic performance has been declining over the course of this school year. For the past 3 months, she has noted weakness in her neck and face with strong emotions, especially laughing with her friends. She recalls one episode of waking and being unable to move her arms and legs for several minutes, which self-resolved. Her general physical and neurological examination findings are normal. Of the following, the BEST next step in her diagnostic evaluation is", "options" : "[\"actigraphy with sleep diary\", \"electroencephalography\", \"magnetic resonance imaging of the brain with and without contrast\", \"polysomnography with multiple sleep latency test\"]", "explanation" : "Polysomnography followed by multiple sleep latency testing (MSLT) is the confirmatory test for a patient presenting with narcolepsy, such as the adolescent girl in this vignette. There are 2 purposes to this testing. An overnight sleep study provides detailed information on sleep architecture, which in narcolepsy is fragmented with an early transition to rapid eye movement (REM) sleep, and it can exclude other causes of hypersomnolence (eg, sleep apnea). Multiple sleep latency testing is performed throughout the day following the polysomnography. During MSLT, a patient is asked to fall asleep every 2 hours for 20 minutes, and their sleep onset and architecture is analyzed. In narcolepsy, patients fall asleep quickly when given the opportunity to nap and will have onset of REM sleep in at least 2 of 4 sleep periods. Both of these features on MSLT support the diagnosis of narcolepsy.\n\nNarcolepsy is a REM sleep disorder characterized by 4 core clinical features present for at least 3 months: excessive daytime sleepiness, cataplexy, hypnagogic hallucinations, and sleep paralysis. Excessive daytime sleepiness is the cardinal feature, with only 50% of patients presenting with all 4 features. Although a rare condition in the general population, narcolepsy is a common cause of excessive daytime sleepiness, which in the pediatric population can present as irritability or frequent napping. Children as young as 5 years have been described with the condition, however narcolepsy most commonly presents in adolescence or young adulthood.\n\nThe core clinical feature of narcolepsy is hypersomnolence despite adequate and restorative nighttime sleep. Hypersomnolence is usually sudden and severe in onset and typically impacts the patient's activities and academics. Accompanying clinical features reflect the underlying pathophysiology of narcolepsy as a disorder of REM sleep regulation. Cataplexy, sudden partial or complete paralysis of the voluntary muscles triggered by intense emotions, can be subtle. It usually affects the face before evolving to include trunk or limb muscles. The normal REM sleep state typically occurs later in the sleep stages and includes core clinical features of the dream state as well as muscle paralysis. In narcolepsy, REM sleep regulation is disturbed, and patients enter this state at sleep onset, resulting in hypnagogic hallucinations and sleep paralysis.\n\nTwo types of narcolepsy are recognized. Type 1 is caused by orexin (hypocretin)-producing hypothalamic neuronal loss, and type 2 has an unknown etiology. Both types have the same clinical presentation. In type 1 it is hypothesized that an autoimmune process leads to destruction of orexin-producing hypothalamic neurons. This theory is supported by development of the condition after some illnesses, such as the 2009 H1N1 influenza pandemic; however, causative antibodies have not been identified. Susceptibility for the development of narcolepsy has been found in individuals with HLA DCB1*06:02. This can be tested for but has limited diagnostic or clinical value. Cerebrospinal fluid analysis in affected individuals demonstrates low levels of orexin and is most useful in children younger than 8 years or children with inconclusive MSLT results.\n\nManagement of narcolepsy is symptomatic, targeted at the symptoms experienced by each individual patient. Stimulants can be used to address hypersomnolence, antidepressants can be used in the treatment of cataplexy and psychiatric comorbidities, and good sleep hygiene provides the foundation of care. Sodium oxybate can be used for dual therapy of excessive daytime sleepiness and cataplexy.\n\nPolysomnography with or without MSLT is used for the evaluation of sleep conditions including sleep apnea and periodic limb movement syndrome in addition to narcolepsy. Actigraphy measures limb movements through a device worn on the wrist or ankle that provides a graphical picture of sleep behavior over time. It is helpful in the evaluation of circadian rhythm sleep-related disorders but does not provide electroencephalography or electromyography data needed to confirm a diagnosis of narcolepsy. Magnetic resonance imaging may be indicated for any focal neurologic findings on examination. Findings from magnetic resonance imaging and electroencephalography are normal in the evaluation of a patient with narcolepsy.\n\nCaring for the pediatric patient with narcolepsy requires prompt identification, diagnosis, and management of the condition but also recognition of common medical and psychiatric comorbidities. Pediatric narcolepsy patients are at risk for rapid weight gain, precocious puberty, and neuropsychiatric conditions such as attention-deficit/hyperactivity disorder, anxiety, and depression. Patients can experience a significant impact on their academic performance and social lives. The ability to drive can be impacted by the excessive daytime sleepiness characteristic of the disorder. Recognition, support, and management of these conditions are a crucial component of patient care.\n\nPREP Pearls\n• Narcolepsy is a pediatric rapid eye movement sleep disorder characterized by excessive daytime sleepiness with accompanying cataplexy, hypnagogic hallucinations, and sleep paralysis.\n• Polysomnography with multiple sleep latency tests is the gold standard for confirmatory diagnostic testing for narcolepsy.\n• Management of narcolepsy is aimed at supportive care targeted at the clinical features experienced by the patient with recognition and management of common medical and neuropsychiatric comorbidities.\n\nABP Content Specifications(s)\n• Recognize the clinical features of narcolepsy and manage appropriately\n\nSuggested Readings\n• Babiker MO, Prasad M. Narcolepsy in children: a diagnostic and management approach. Pediatr Neurol. 2015;52(6):557-565. doi:10.1016/j.pediatrneurol.2015.02.020.\n• Plazzi G, Clawges HM, Owens JA. Clinical characteristics and burden of illness in pediatric patients with narcolepsy. Pediatr Neurol. 2018;85:21-32. doi:10.1016/j.pediatrneurol.2018.06.008.\n• Scammell TE. Narcolepsy. N Engl J Med. 2015;373(27):2654-2662. doi:10.1056/NEJMra1500587.\n• Splaingard ML, May A. Sleep disturbances (nonspecific). In: McInerny TK, Adam HM, Campbell DE, DeWitt TG, Foy JM, Kamat DM, eds. American Academy of Pediatrics Textbook of Pediatric Care. 2nd ed. Elk Grove Village, IL: American Academy of Pediatrics; 2017:1589-1607. Pediatric Care Online."}
{"id" : 1918, "question_text" : "A 4-year-old girl has rapid breathing. Her mother has counted her respiratory rate as 46 breaths/min. The girl has no signs of respiratory distress. She has not had fever, and there are no known ill contacts. She has an oxygen saturation of 99% in room air, a temperature of 37°C, and a respiratory rate of 48 breaths/min. She is irritable but comfortable and has no cough or wheeze. There is no increased breathing effort or use of accessory muscles. Breath sounds are clear, and the cardiac examination findings are normal. Pulses are equal and full. A chest radiograph is normal. Results of a urinalysis and a complete blood cell count and differential are normal. Of the following, the historic information that is the MOST pertinent to this girl's presentation is", "options" : "[\"her maternal aunt has type 1 diabetes\", \"an open bottle of chewable aspirin was found accessible in the home\", \"respiratory syncytial virus has been reported in her day care center\", \"she has recently been drinking more apple juice than usual\"]", "explanation" : "Correct Answer: B\nThe girl in this vignette has normal examination findings, other than tachypnea and irritability, and there is no supportive data to suggest a cardiac or respiratory source of her tachypnea. Therefore, the most likely etiology is metabolic. Of the response choices, metabolic acidosis from salicylate ingestion is the most likely cause for her tachypnea.\n\nThe girl does not have fever or primary upper respiratory signs or symptoms that might suggest respiratory syncytial virus infection. A family history of diabetes is only helpful if the child has symptoms of diabetic ketoacidosis, but the normal urinalysis results make this unlikely. Although apple juice is acidic, it is difficult to ingest enough to produce systemic acidosis. In the presence of tachypnea when there is no fever, signs of respiratory distress or acute respiratory compromise, or cardiac abnormalities, a search for a metabolic cause of the tachypnea will be the most helpful.\n\nMultiple factors affect respiratory rate, and the range of normal respiratory rate is fairly broad, particularly in younger children. Tachypnea may be one of the first signs of respiratory distress and should be evaluated. Fever is probably the most common cause of tachypnea, and the elevated respiratory rate may resolve with a decrease in body temperature. Other causes of tachypnea include airway reactivity, cardiac and intrapulmonary processes, upper airway obstruction, and metabolic derangement. For a 4-year-old, the average respiratory rate is about 25 breaths/min, and the range may be from 15 to 35 breaths/min. For newborns, a normal respiratory rate may be as high as 55 breaths/min or as low as 20 breaths/min. By one year of age, the average respiratory rate is around 30 breaths/min but the normal range is still very broad.\n\nPREP Pearls\n\nThere is a broad range in normal for respiratory rate, especially in infants and young children.\n\nIn the presence of tachypnea when there is no fever, respiratory distress, acute respiratory compromise, or cardiac abnormalities, a metabolic cause of the tachypnea should be sought.\n\nABP Content Specifications(s)/Content Area\n\nPlan the appropriate clinical and diagnostic evaluation of tachypnea of various etiologies\n\nRecognize the various factors that influence respiratory rate\n\nRecognize normal breathing patterns in patients of various ages\n\nSuggested Readings\n\nBloomfield D. Tachypnea. Pediatr Rev. 2002;23(8):294-295. doi: 10.1542/pir.23-8-294 .\n\nHarris C, Homnick DN. The pulmonary physical exam. In: Light MJ, Blaisdell CJ, Homnick DN, Schechter MS, Weinberger MM, eds. Pediatric Pulmonology. Elk Grove Village, IL: American Academy of Pediatrics; 2011:77-88."}
{"id" : 167, "question_text" : "A 6-year-old previously healthy boy presents with the recent development of nocturnal dyspnea. On questioning of his parents, you discover that the child has experienced exercise intolerance, two episodes of syncope while running, poor appetite, and a cough without congestion over the past year. His physical examination reveals a heart rate of 120 beats/min, respiratory rate of 26 breaths/min, gallop rhythm, III/VI high-pitched blowing systolic murmur at the apex, hepatomegaly, and diminished pulses. Chest radiography documents an enlarged cardiac silhouette with pulmonary vascular congestion (Item Q57), and echocardiography demonstrates a regurgitant mitral valve with a dilated left ventricle and markedly reduced systolic contractility.\n\nOf the following, the MOST likely cause for this child's dilated cardiomyopathy is", "options" : "[\"a congenital mitral valve abnormality\", \"Duchenne muscular dystrophy\", \"Friedreich ataxia\", \"rheumatic heart disease\", \"sickle cell disease\"]", "explanation" : "The causes of a dilated cardiomyopathy are diverse and include viral myocarditis, arrhythmias, metabolic conditions, muscle disorders, drug toxicities, and congenital cardiac lesions. Among the mitral valve disorders are abnormalities of the valve leaflets or chordal apparatus, which result in either acute neonatal symptoms or chronic, indolent findings that eventually degenerate. Mitral valve disorders can occur in isolation or as a part of a complex of left heart pathology that may include abnormalities of the aortic valve (eg, bicuspid aortic valve with stenosis or regurgitation), aortic arch (coarctation of the aorta), and left ventricle (hypoplastic left heart syndrome). Mitral valve disease can result in either stenosis or regurgitation. Both can be tolerated by the child for long periods of time, but eventually they worsen and result in left atrial dilation, left ventricular dilation, and elevated pressure back into the lungs (pulmonary hypertension). As the left ventricle continues to dilate, efficiency of contractility is reduced because fiber cross-linking exceeds the most optimal portion of the Starling curve, and eventually a dilated cardiomyopathy results. Accordingly, the boy in the vignette most likely has a congenital mitral valve abnormality and congestive heart failure (CHF).\n\nSymptoms of CHF in older children include exercise intolerance, fatigue, dizziness or syncope, shortness of breath, palpitations, diaphoresis, abdominal discomfort, and anorexia. Among the signs are tachycardia, rales, poor tissue perfusion, hepatomegaly, and a gallop rhythm. Auscultation may identify a blowing systolic ejection murmur at the apex with radiation to the back from mitral regurgitation. Even in the absence of an intrinsic mitral valve disorder, mitral regurgitation can develop when left ventricular dilation causes a change in geometry that results in poor leaflet coaptation. Echocardiography is the gold standard for diagnosing both mitral valve disorders and a cardiomyopathic process. In children, the mitral valve can be seen in exquisite detail, allowing identification of pathologic features, as described for this boy. Color Doppler interrogation assists with determination of mitral regurgitation or stenosis. The left ventricular cavity can be assessed for chamber dilation, and quantitative determination of contractility is the routine part of echocardiographic assessment.\n\nDuchenne muscular dystrophy often leads to the development of a dilated cardiomyopathy due to the adverse effect of the dystrophin mutation on the cardiomyocyte. However, the child in the vignette is younger than the typical child who develops left ventricular dysfunction (unusual before 10 years of age). In addition, most boys who have Duchenne muscular dystrophy and develop a dilated cardiomyopathy already manifest skeletal muscle involvement. Friedreich ataxia is associated with the development of a cardiomyopathy, but it is a hypertrophic rather than a dilated cardiomyopathy. In addition, a child who has Friedreich ataxia and advanced cardiac findings invariably exhibits ataxia. Mitral valve (as well as aortic valve) thickening and dysfunction are found in rheumatic heart disease. However, this child has none of the other cardinal features of acute rheumatic fever (dermatologic, infectious, neurologic, or joint). Sickle cell disease can result in high-output heart failure due to anemia, but this child does not have the features of a chronic disease state, which very likely would have developed by the age of 6 years.\n\nAmerican Board of Pediatrics Content Specification(s): Identify early fatigue, exercise intolerance, anorexia, and cough as symptoms of congestive heart failure in older children"}
{"id" : 2484, "question_text" : "A 2-month-old boy is seen for a routine health supervision visit. He has a unilateral undescended testis that was noted at birth. He is otherwise healthy. His growth and development are normal. His right testis is not palpable in the scrotal sac or the inguinal canal. His right hemiscrotum is underdeveloped. His left testis is easily palpable in the appropriately-developed left hemiscrotum. His penis length is normal, and the urethral meatus is normally located. The remainder of the boy's physical examination findings are normal. Of the following, this infant is at HIGHEST risk of developing", "options" : "[\"adrenal crisis\", \"malignancy\", \"short stature\", \"urinary tract infection\"]", "explanation" : "Correct answer is B\n\nThe infant in the vignette has unilateral cryptorchidism. Of the response choices, he is at highest risk of developing a testicular malignancy. The risk of testicular cancer is 2 to 8 times higher in boys with cryptorchidism. Although orchiopexy performed before puberty decreases the risk of malignancy, the risk remains higher throughout life than in those without cryptorchidism. Orchiopexy allows for easier examination and surveillance because the testis is located in the scrotum.\n\nInfertility is the other major risk associated with cryptorchidism. Orchiopexy is recommended by age 18 months to preserve the full fertility potential. Higher rates of germ cell loss and infertility occur the longer the testis remains undescended.\n\nWhen an undescended testis is associated with an inguinal hernia, there is a risk of acute strangulation. Additionally, testicular torsion is more difficult to diagnose when it occurs in an undescended testis.\n\nA testis may be nonpalpable because of arrest of descent (cryptorchidism), ectopic location, retraction into the inguinal canal (retractile testis), or intrauterine torsion (vanishing testis). Arrest of descent can occur at any location along the line of migration: intra-abdominal, the inguinal canal (internal and external rings), or the upper pole of the scrotal sac. The most common location for a cryptorchid testis is the inguinal canal, followed by the prescrotal location (distal to the external ring of the inguinal canal).\n\nCryptorchidism is the most common genital disorder identified at birth, with a prevalence of 2% to 3% in term infants. Because testicular descent occurs during the 3rd trimester (typically by 36 weeks' gestation), it has a notably higher prevalence (20% to 30%) in premature infants. The etiology of cryptorchidism is multifactorial and includes genetic predisposition, hormonal imbalances, and environmental factors. For relatives of a boy with cryptorchidism, there is a 10-fold increased risk in twins and a 3-fold increased risk in brothers. Several maternal factors (eg, obesity, smoking, diabetes) are associated with an increased risk of cryptorchidism. Low birth weight, breech presentation, and estrogen exposure are also associated with cryptorchidism.\n\nThe diagnosis of cryptorchidism is made via careful physical examination. If a testis is not palpable, techniques such as moving one hand along the inguinal canal and applying gentle pressure to close the external ring while using the other hand to palpate for the testis in the scrotum may be helpful. A retractile testis can be brought into the scrotal sac on physical examination, but the cremasteric reflex may cause it to retract back into the canal. A testis may be palpated in an ectopic location (eg, perineum, femoral triangle, contralateral scrotum, or superficial inguinal area). The presence of unilateral or bilateral cryptorchidism should be noted, as well as any other genital abnormalities (eg, hypospadias and micropenis).\n\nSpontaneous descent of a cryptorchid testis into the scrotal sac can occur up to 6 months after birth (corrected for gestational age). The likelihood of descent after age 6 months is extremely low. Therefore, infants should be referred to a urologist by age 6 to 9 months if both testes are not present in the scrotal sac. Infants with a retractile testis should be examined closely at every health supervision visit; if the condition is persistent, they should be referred to a urologist by age 6 to 9 months owing to the risk of ascent and acquired cryptorchidism.\n\nAn infant with bilateral cryptorchidism or unilateral cryptorchidism in the presence of other abnormal genital findings should be evaluated for a difference of sexual development (DSD). An infant with 46,XX chromosomes and severe virilization due to congenital adrenal hyperplasia can be mistaken for a male with bilateral cryptorchidism. Prompt recognition of a possible DSD is critical, because some DSDs are associated with adrenal insufficiency. The infant in the vignette has unilateral cryptorchidism and otherwise normal external genitalia, making a DSD and the associated risk of an adrenal crisis unlikely.\n\nThis infant has a normal penile length, making growth hormone deficiency with resulting short stature an unlikely diagnosis. Growth hormone deficiency can be associated with micropenis. Urinary tract infections are not associated with isolated cryptorchidism.\n\nSuggested Reading(s)\nInouye B, Tourchi A, Gearhart JP. Hypospadias, epispadias, and cryptorchidism. In: McInerny TK, Adam HM, Campbell DE, Foy JM, Kamat DM, eds. American Academy of Pediatrics Textbook of Pediatric Care. 2nd ed. American Academy of Pediatrics; 2016:chap 272. Accessed March 3, 2023. Pediatric Care Online\nKolon TF, Herndon CDA, Baker LA, et al. Evaluation and treatment of cryptorchidism: AUA guideline. J Urol. 2014;192(2):337-345. doi:10.1016/j.juro.2014.05.005\nWu WJ, Gitlin JS. The male genital system. Pediatr Rev. 2020;41(3):101-111. doi:10.1542/pir.2017-0316\n\nContent Domain\nReproductive Health\n\nABP Content Specification(s) / Content Area(s)\nUnderstand the natural history of cryptorchidism\nDifferentiate the findings associated with undescended testes from those of retractile testes\nRecognize complications associated with undescended testes\nPlan the appropriate management of undescended testes"}
{"id" : 3565, "question_text" : "A 17-year-old boy from North Carolina, who returned from a mission trip to Kenya 1 week ago, comes to the emergency department in December with a 3-day history of fever, chills, headache, and vomiting. He has had no exposure to sick contacts, and no known tick or mosquito bites. He has a pet dog. He reports taking a weekly regimen of antimalarial prophylaxis without missing any doses. Physical examination shows an ill-appearing boy with a temperature of 39.2°C, heart rate of 106 beats/min, and blood pressure of 116/72 mm Hg. He has scleral icterus and mild splenomegaly. The rest of the physical examination findings are normal. A complete blood cell count shows anemia and thrombocytopenia. A peripheral blood film is shown (Item Q9). Of the following, the MOST likely cause of this patient's illness is", "options" : "[\"Babesia microti\", \"Ehrlichia chaffeensis\", \"Rickettsia rickettsii\", \"Plasmodium falciparum\"]", "explanation" : "The ill-appearing adolescent described in the vignette has a history of recent travel to Kenya. He has an acute onset of febrile illness associated with chills, splenomegaly, anemia, thrombocytopenia, and a peripheral blood film depicting ring forms. This clinical picture is concerning for severe malaria infection caused by the parasite Plasmodium falciparum.\n\nTick-borne infection caused by Rickettsia rickettsia and Ehrlichia chaffeensis must be considered in children residing or traveling in southeastern, south central, and eastern United States who have nonspecific febrile illnesses with headache, emesis, and thrombocytopenia. However, for the patient described in the vignette, the presence of ring forms of P falciparum on blood films argues against the diagnosis of rickettsial sepsis. In addition, most patients with rickettsial infection develop severe illness after 4 to 5 days of fever. Other tick-borne zoonosis, such as babesiosis caused by Babesia microti, are more common in the northeast and upper midwestern states. The clinical presentation of babesiosis is similar to that of malaria with fever, hemolytic anemia, and thrombocytopenia. Detection of tetrad (Maltese-cross) forms, extracellular rings, on blood smear is pathognomonic for the diagnosis of babesiosis.\n\nMalaria is a vector-borne parasitic disease predominantly affecting tropical regions of the world. The 5 species known to infect humans are P falciparum, Plasmodium vivax, Plasmodium ovale, Plasmodium malariae, and Plasmodium knowlesi. Infection is acquired from the bite of the female Anopheles mosquito. P falciparum is the most common infection worldwide, with an estimated incidence of 207 million in 2016, and is the dominant species in sub-Saharan Africa, whereas P vivax species is prevalent in Asia and Central and South America. Infants and young children, pregnant women, and immunocompromised hosts are at risk for severe disease. In the United States, approximately 1,500 cases of malaria are reported annually, all of which occur in individuals returning from foreign travel to malaria-endemic countries. The use of appropriate malaria chemoprophylaxis and personal protective measures (eg, insect-repellant) are key to preventing malaria among travelers to endemic areas.\n\nThe incubation period of malaria can range from 7 days to several months after exposure, depending on the species. Uncomplicated malaria typically presents as a nonspecific febrile illness with chills, headache, myalgia, arthralgia, fatigue, and gastrointestinal complaints (emesis, abdominal pain, diarrhea). Altered mental status is suggestive of central nervous system involvement (cerebral malaria). Severe malaria, often caused by P falciparum infection, is characterized by at least 1 of the following manifestations: cerebral malaria, hypoglycemia, renal failure, acute respiratory distress syndrome, shock, metabolic acidosis, severe anemia, hemoglobinuria, coagulopathy, or red blood cell parasitemia greater than 5%. Severe malaria is associated with high mortality rates (>80%).\n\nThe diagnosis of malaria must be confirmed on microscopic examination of thick or thin blood films, usually with Giemsa stain. The thick blood smear is more sensitive for detecting parasites; the thin blood smear aids in species identification and quantification. If blood films are negative for parasites but malaria is strongly suspected clinically, it is recommended that smears be repeated every 12 to 24 hours over a 3-day period. The US Food and Drug Administration has approved a rapid diagnostic test (RDT) for the detection of malaria antigen. However, RDT should always be performed in parallel with microscopy because RDT may be associated with false-negative or false-positive test results. A polymerase chain reaction assay is also available for detecting Plasmodium species in certain health departments and commercial laboratories.\n\nChildren with severe P falciparum malaria must be treated in the intensive care unit with aggressive supportive care and intravenous malaria chemotherapy; intravenous quinidine gluconate plus tetracycline, clindamycin, or doxycycline is recommended. Patients must be monitored for hypotension, hypoglycemia, and cardiac dysrhythmia while receiving quinidine. If intravenous quinidine is unavailable or if patients cannot tolerate the medication, clinicians can consult the Centers for Disease Control and Prevention malaria hotline (770-488-7100) and access intravenous artesunate. The artesunate course is followed by 1 of the following other antimalarial agents: atovaquone-proguanil, doxycycline, clindamycin, or mefloquine.\n\nPREP Pearls\n• Severe malaria is often caused by Plasmodium falciparum, the dominant species in sub-Saharan Africa.\n• Severe malaria is characterized by at least 1 of the following features: cerebral malaria, hypoglycemia, renal failure, acute respiratory distress syndrome, shock, metabolic acidosis, or severe anemia.\n• The diagnosis of malaria must be confirmed by microscopic examination of thick or thin blood films.\n\nABP Content Specifications(s)\n• Understand the epidemiology of malaria\n• Recognize the clinical features of malaria, and manage appropriately\n\nSuggested Readings\n• Abdel-Haq N, Chearskul P, Rafee Y, Asmar BI. Parasitic infections. In: McInerny TK, Adam HM, Campbell DE, DeWitt TG, Foy JM, Kamat DM, eds. American Academy of Pediatrics Textbook of Pediatric Care. 2nd ed. Elk Grove Village, IL: American Academy of Pediatrics; 2017:2470-2490. Pediatric Care Online.\n• American Academy of Pediatrics. Malaria. In: Kimberlin DW, Brady MT, Jackson MA, Long SS, eds. Red Book: 2018 Report of the Committee on Infectious Diseases. 31st ed. Itasca, IL: American Academy of Pediatrics; 2018:527-536. https://redbook.solutions.aap.org/chapter.aspx?sectionid=88187185&bookid=1484#91037920.\n• Ashley EA, Pyae Phyo A, Woodrow CJ. Malaria. Lancet. 2018;391(10130):1608-1621. doi:10.1016/S0140-6736(18)30324-6.\n• Centers for Disease Control and Prevention. Malaria Surveillance – United States, 2014. MMWR Surveill Summ. 2017;66(12):1-24. doi:10.15585/mmwr.ss6612a1."}
{"id" : 3796, "question_text" : "A newborn is having a follow-up evaluation after a neonatal intensive care unit (NICU) stay. The child had tachypnea at birth, required support with oxygen and continuous positive airway pressure for several hours, and ultimately was diagnosed with transient tachypnea of the newborn. He was quickly weaned to room air over 2 days. He was breathing comfortably, latching on, and breastfeeding well before being discharged from the hospital. In the NICU, he was noted to have a cardiac murmur and echocardiography was performed, which showed a moderate ventricular septal defect (VSD) with left-to-right flow and a bicuspid aortic valve without stenosis or regurgitation. The mother reports that she was told that the VSD will likely require surgical closure, but wishes to know what needs to be done about the bicuspid aortic valve. Of the following, the MOST appropriate response to this mother is", "options" : "[\"full anticoagulation at this time\", \"long-term pediatric cardiology follow-up\", \"no further treatment\", \"valve repair or replacement at the time of VSD surgery\"]", "explanation" : "Correct Answer: B\nThe neonate in the vignette is known to have a moderate ventricular septal defect (VSD), as well as a bicuspid aortic valve (BAV) without stenosis or regurgitation. The VSD is likely to need surgical repair. There is no indication to intervene for the aortic valve at this time or to start anticoagulation. However, patients with BAV can develop regurgitation, stenosis, endocarditis, and aortopathy, and therefore need follow-up throughout life.\n\nBicuspid aortic valve is one of the most common congenital heart defects. The normal aortic valve is trileaflet; in the setting of a BAV, there are 2 leaflets. This is thought to occur because of an abnormal fusion during embryonic development. Patients with BAV are likely to have some complication during their life. Adult studies have found that a bimodal distribution of surgical intervention is required at age 30 to 40 years or after age 50 years. Some children require intervention as well. Bicuspid aortic valve can be familial or sporadic. Because of the familial inheritance risk, first-degree relatives should be screened. Bicuspid aortic valve is also associated with genetic syndromes such as Turner syndrome.\n\nVentricular septal defect (VSD) is also one of the most common congenital heart defects. It consists of a communication between the right and left ventricles via an opening in the interventricular septum. Ventricular septal defects are named based on their location along the septum (membranous, muscular, atrioventricular canal, conal septal). They are further described as small, medium, or large based on their size relative to the size of the aortic valve. A small VSD is unlikely to cause hemodynamic effects and if located in the muscular septum, may close spontaneously. Moderate and large defects will need to be surgically closed. Before closure, some children will have tachypnea and increased work of breathing because of the increased pulmonary blood flow. This can be medically treated with diuretics and higher caloric feeds.\n\nPREP Pearls\n• Patients with bicuspid aortic valve can develop regurgitation, stenosis, endocarditis, and aortopathy, and therefore need cardiology follow-up throughout life.\n• Ventricular septal defects are named based on their location (membranous, muscular, atrioventricular canal, conal septal) and size of the defect.\n• Moderate and large ventricular septal defects require surgical closure because of the impact of increased pulmonary blood flow.\n\nABP Content Specifications(s)\n• Understand the natural history of a bicuspid aortic valve\n• Understand the natural history of ventricular septal defect\n\nSuggested Readings\n• Freeze SL, Landis BJ, Ware SM, Helm BM. Bicuspid aortic valve: a review with recommendations for genetic counseling. J Genet Counsel. 2016;25(6):1171-1178. doi:10.1007/s10897-016-0002-6.\n• Masri A, Svensson LG, Griffin BP, Desai MY. Contemporary natural history of bicuspid aortic valve disease: a systematic review. Heart. 2017;103(17):1323-1330. doi:10.1136/heartjnl-2016-309916.\n• McCulloch MA, Gajarski RJ. Congenital and acquired heart disease. In: McInerny TK, Adam HM, Campbell DE, DeWitt TG, Foy JM, Kamat DM, eds. American Academy of Pediatrics Textbook of Pediatric Care. 2nd ed. Elk Grove Village, IL: American Academy of Pediatrics; 2017:1883-1917. Pediatric Care Online.\n• McDaniel NL. Ventricular and atrial septal defects. Pediatr Rev. 2001;22(8):265-270. doi:10.1542/pir.22-8-265.\n• Puri K, Allen HD, Qureshi AM. Congenital heart disease. Pediatr Rev. 2017;38(10):471-486. doi:10.1542/pir.2017-0032."}
{"id" : 3054, "question_text" : "An 18-month-old, African-American boy is brought to your clinic by his grandmother for yellowness of the eyes. The grandmother states that he has been irritable and sleeping more over the past 3 days. Last week, he had nasal congestion and a cough. Concerned that he might develop a fever, she gave him a few doses of antibiotics that were left over from his sibling's recent treatment. He is pale and quiet. His oral temperature is 37.3°C, pulse rate is 125 beats/min, respiratory rate is 22 breaths/min, and blood pressure is 100/65 mm Hg. On physical examination, he has icteric sclerae and a 2/6 systolic ejection murmur. There is no hepatosplenomegaly. The remainder of the physical examination findings are unremarkable. The following laboratory tests are obtained: White blood cell count, 10,500/µl (10.5 X 109/L), with 30% polymorphonuclear leukocytes, 61% lymphocytes, 6% monocytes, and 3% eosinophils; Hemoglobin, 6.0 g/dL (60 g/L); Hematocrit, 18%; Mean corpuscular volume, 80 um3 (80 fL); Platelet count, 250 x 103/µL (250 x 109/L); Reticulocyte count, 10% (0.10); Direct bilirubin, 0.5 mg/dL (8.6 µmol/L); Indirect bilirubin, 4.0 mg/dL (68.4 µmol/L); Direct antiglobulin (Coombs) test, negative. Of the following, the MOST likely cause of this patient's findings is", "options" : "[\"autoimmune hemolytic anemia\", \"\\u03b2-thalassemia minor\", \"Gilbert syndrome (UGT1A1 mutation)\", \"glucose-6-phosphate dehydrogenase deficiency\", \"transient erythroblastopenia of childhood\"]", "explanation" : "Preferred Response: D\nThe boy in the vignette has a clinical history and presentation most consistent with glucose-6-phosphate dehydrogenase (G6PD) deficiency. G6PD is an important enzyme in protecting the red blood cell (RBC) from oxidative stress. It is the primary step in the pentose-phosphate pathway (PPP) that produces nicotinamide adenine dinucleotide phosphate (NADPH) and keeps glutathione in its reduced state. This is the only mechanism of producing NADPH in ABCs. Certain substances, such as sulfa drugs and fava beans, interact with oxygen and hemoglobin to produce a high level of toxic oxygen peroxides. In the presence of normal levels of NADPH and reduced glutathione, the oxygen peroxides are neutralized and converted to pentose sugars via the PPP, thus protecting the RBCs against damage. In G6PD deficiency, however, these oxygen peroxides persist, causing damage to the hemoglobin and cell membrane, and ultimately leading to hemolysis.\n\nAs RBCs age, their G6PD content decreases. Glucose6-phosphate dehydrogenase activity may be 5 times higher in reticulocytes than in older cells. This leads to selective destruction of older cells with lower G6PD activity during times of hemolytic crisis. Therefore, testing for G6PD deficiency during a time of acute hemolysis and reticulocytosis may lead to false-negative results. Testing should be done when the patient is at baseline.\n\nThe gene encoding G6PD is on the long arm of the X chromosome (band X428). Male hemizygotes have only the mutated copy of this gene and therefore will have decreased G6PD levels, whereas in heterozygote females with one affected allele and one normal allele, the level of G6PD activity can vary from near normal to severely decreased.\n\nGlucose-6-phosphate dehydrogenase deficiency is the most common enzyme defect in humans and is estimated to affect more than 400 million people worldwide. Mutations in this gene are associated with resistance to Plasmodium falciparum malaria, therefore the prevalence of G6PD deficiency is higher in Mediterranean, African, Middle Eastern, and South Asian populations. Many mutations have been reported in the G6PD gene. The wild type gene most commonly found in white populations is the B allele, whereas the most common wild type polymorphism in African populations is the A+ variant. The A— variant is the most common disease-causing variant in African populations, whereas the G6PD Mediterranean variant is seen mostly in the Mediterranean and Middle Eastern regions.\n\nThe common clinical presentations for G6PD deficiency are as follows: (1) neonatal jaundice, (2) acute hemolytic anemia, and (3) chronic nonspherocytic hemolytic anemia (CNSHA). Neonatal jaundice resulting from unconjugated hyperbilirubinemia is more common in G6PD-deficient newborns than in those with normal G6PD levels. Though the exact mechanism of this is not entirely known, treatment with phototherapy or exchange transfusion is similar to neonatal hyperbilirubinemia from other causes. Acute hemolytic anemia can occur after exposure to infections, drugs, or fava beans. Infection is the most common inciting factor for acute hemolysis in G6PD deficiency. It has been reported in association with a wide variety of viral or bacterial infections. Patients who present with acute hemolytic crises usually have a baseline G6PD enzyme activity of less than 10%. This form is commonly seen in Mediterranean and Asian populations. The common presenting signs and symptoms during an acute hemolytic episode are malaise, weakness, and abdominal and back pain. Within 2 to 3 days, patients often have jaundice and dark urine from hemoglobinuria, which can lead to renal failure. Treatment is supportive with packed RBC transfusion, fluids, and hemodialysis if necessary. CNSHA is seen in a small population of G6PD-deficient patients. Baseline G6PD enzyme activity is usually less than 10%. Patients have chronic anemia with a wide range of severity. Children with CNSHA can also present with significant splenomegaly.\n\nThe classes of G6PD deficiency are summarized below.In the patient described in this vignette, the negative direct antiglobulin test makes autoimmune hemolytic anemia an incorrect answer. Although the indirect bilirubin is elevated, Gilbert syndrome is not associated with anemia as seen in this child. Beta-thalassemia is associated with a microcytic anemia, reticulocytosis, and an elevated bilirubin level, the patient in this case has a normocytic anemia. Transient erythroblastopenia of childhood is associated with a low reticulocyte count, not the elevated reticulocyte count as seen in this patient.\n\nItem CM Classes of Glucose-6-Phosphate Dehydrogenase Deficiency\nClass | Level of Deficiency | Baseline Enzyme Activity | Clinical Manifestations\nI | Severe | <10% | Chronic nonspherocytic hemolytic anemia\nII | Severe | <10% | Usually asymptomatic; Acute hemolytic anemia in response to infections, drugs, fava beans; Mediterranean and severe Asian variants\nIII | Moderate | 10-60% | Usually asymptomatic; Acute hemolytic anemia in response to infections, drugs, fava beans; African (A—) variant\n\nPREP Pearls\n• There is a high prevalence of G6PD deficiency in African, Mediterranean, and Asian populations. The African variant generally tends to be the less severe form.\n• Glucose-6-phosphate dehydrogenase (G6PD) deficiency is an X-linked disorder.\n• Higher levels of G6PD activity may be detected during acute hemolysis and reticulocytosis, and may lead to false-negative results when testing for G6PD deficiency.\n\nAmerican Board of Pediatrics Content Specification(s)\n• Recognize the inheritance pattern associated with G6PD deficiency\n• Recognize the clinical findings associated with G6PD deficiency in patients of differing ethnic backgrounds\n• Plan appropriate management of hemolysis in a patient with G6PD deficiency\n\nSuggested Reading\n• Arese P, Gallo V, Pantaleo A, Turrini F. Life and death of G6PD deficient erythrocytes- role of redox stress and band 3 modifications. Tranfus Med Hemother. 2012;39(5):328-334. doi:10.1159/000343123.\n• Glader B. Genetics and pathophysiology of glucose-6-phosphate dehydrogenase deficiency. UpToDate. Available online only for subscription.\n• Manganelli G, Amsullo U, Passarelli 5, Filosa S. Glucose-6-phosphate dehydrogenase deficiency: disadvantages and possible benefits. Cardiovasc Hematol Disord Drug Targets. 2012;10(2):143-150. doi:10.2174/18715 29X11313010008."}
{"id" : 2842, "question_text" : "A male neonate is delivered at 37 weeks' gestation because of maternal preeclampsia. His mother's history is significant for obesity and chronic hypertension. He weighs 2.2 kg at birth. At 18 hours after birth, shortly after breastfeeding and supplementing with formula, his bedside glucose level is 35 mg/dL (1.9 mmol/L). He is breathing comfortably in room air and has good perfusion. Of the following, the MOST likely explanation for this neonate's glucose level is", "options" : "[\"congenital hyperinsulinemia\", \"decreased glycogen stores\", \"maternal hyperglycemia\", \"physiologic hypoglycemia\"]", "explanation" : "Correct Answer: B\nFor the growth-restricted neonate in the vignette, limited glycogenolysis because of decreased glycogen stores is the most likely cause of hypoglycemia. In utero, a fetus relies on maternal glucose supply. After delivery, the maternal glucose is no longer available, resulting in transient neonatal hypoglycemia. In response, there is a surge in glucagon, epinephrine, and cortisol, which promotes glycogenolysis, gluconeogenesis, and lipolysis and ultimately restores serum glucose values. Increased glucagon stimulates the production of phosphoenolpyruvate carboxykinase, the rate-limiting enzyme for gluconeogenesis. At the same time, muscle tissue can use free fatty acids and ketone bodies, leaving serum glucose available for brain utilization. The causes of neonatal hypoglycemia are shown in Item C42A.\n\nThe brain relies on glucose as a primary energy source. Periods of prolonged neonatal hypoglycemia have been associated with seizures, cerebral palsy, and cognitive impairment. In particular, low serum glucose because of elevated insulin levels presents multiple challenges. Hyperinsulinemia decreases glycogenolysis, fat metabolism, and the formation of ketone bodies, limiting the availability of alternate energy sources for the brain. Congenital hyperinsulinemia because of mutations in the glucose transporter genes can cause prolonged hypoglycemia, which is associated with adverse neurodevelopmental outcomes.\n\nNeonates at risk for hypoglycemia because of growth restriction, prematurity, family history of hypoglycemia, and inherited diseases of metabolism, should be monitored closely. The definition of neonatal hypoglycemia is currently being debated (Item C42B). The American Academy of Pediatrics recommends maintaining serum glucose levels at more than 45 mg/dL (2.5 mmol/L) by 4 hours after birth. The Pediatric Endocrine Society recommends serum glucose levels greater than 50 mg/dL (2.7 mmol/L) for the first 48 hours after birth and greater than 60 mg/dL (3.3 mmol/L) by 48 hours after birth.\n\nItem C42B: Pediatric Endocrine Society (PES) and American Academy of Pediatrics (AAP) neonatal hypoglycemia guidelines in the first 48 hours after birth and beyond. Reprinted with permission from Thompson-Branch A, Havranek T. Neonatal hypoglycemia. Pediatr Rev. 2017;38(4):150\n\nPhysiologic hypoglycemia occurs in the first four hours of life and would not explain this neonate's hypoglycemia at 18 hours of life. His mother does not have diabetes making maternal hyperglycemia unlikely. While congenital hyperinsulinemia is a possible explanation, this neonate's hypoglycemia is more likely the result of inadequate glycogen stores.\n\nPREP Pearls\n• Periods of prolonged neonatal hypoglycemia have been associated with seizures, cerebral palsy, and cognitive impairment.\n• Neonates with hypoglycemia because of elevated insulin levels have less glycogenolysis, fat metabolism, and formation of ketone bodies, decreasing the available alternate sources of energy.\n• Neonates at risk for hypoglycemia because of growth restriction, prematurity, family history of hypoglycemia, or inherited diseases of metabolism should be monitored closely.\n\nABP Content Specifications(s)\n• Recognize the clinical features associated with hyperinsulinism\n• Plan the evaluation of a patient with suspected metabolic disease who has hypoglycemia, and manage appropriately\n\nSuggested Readings\n• Adamkin DH; Committee on Fetus and Newborn. Postnatal glucose homeostasis in late-preterm and term infants. Pediatrics. 2011;127:575-579. doi: 10.1542/peds.2010-3851.\n• Sperling MA. Hypoglycemia. In: Kliegman R, St Geme JW, Blum NJ, Shah SS, Tasker RC, Wilson Karen, eds. Nelson Textbook of Pediatrics. Philadelphia, PA: Elsevier; 2020:848-862.e1.\n• Thompson-Branch A, Havranek T. Neonatal hypoglycemia. Pediatr Rev. 2017;38(4):147-155. doi: 10.1542/pir.2016-0063.\n• Thornton PS, Stanley CA, De Leon DD, et al. Recommendations from the Pediatric Endocrine Society for Evaluation and Management of Persistent Hypoglycemia in Neonates, Infants, and Children. J Pediatr. 2015;167(2):238-245. doi:10.1016/j.jpeds.2015.03.057."}
{"id" : 2874, "question_text" : "A physician is conducting a follow-up visit with a 12-year-old girl who has been experiencing painless vomiting. The girl has a 6-month history of effortless, nonbloody, nonbilious regurgitation occurring 30 min after meals at least once per day. She has had a 4-kg weight loss in the last 6 months. She reports that she enjoys food and is not intentionally losing weight. She reports no nausea, abdominal pain, bloating, diarrhea, or constipation. She was prescribed a proton pump inhibitor for 6 weeks without improvement. On physical examination, her heart rate is within normal range and her dentition is normal. Of the following, the MOST likely diagnosis is", "options" : "[\"cyclic vomiting syndrome\", \"eating disorder\", \"Helicobacter pylori gastritis\", \"rumination syndrome\"]", "explanation" : "Correct Answer: D\nThe girl in this vignette has rumination syndrome, which is characterized by effortless and painless nonbilious, nonbloody regurgitation of food within minutes to hours of eating, after which partially digested food is then rechewed, re-swallowed or expulsed (see Hyams JS, Di Lorenzo C, Saps M, Shulman RJ, Staiano A, van Tilburg M). Neither nausea nor retching usually precede regurgitation in rumination syndrome. Whereas pain is not a predominant feature of rumination syndrome, dyspeptic symptoms such as epigastric or pharyngeal burning may exist. Mild weight loss is relatively common in rumination syndrome, but other features indicative of an eating disorder—such as malnutrition, dental enamel erosion, electrolyte abnormalities, and vital sign abnormalities—are absent.\n\nAlthough infections due to Helicobacter pylori may be asymptomatic, the bacterium has been associated with dyspepsia, causing epigastric abdominal pain, bloating, nausea, and vomiting; acute and chronic gastritis; and peptic ulcer disease. Complications can include gastrointestinal bleeding, bowel perforation, and gastric outlet obstruction. Cyclic vomiting syndrome is a diagnosis of exclusion characterized by recurrent, discrete stereotypical episodes of vomiting and baseline health between episodes. Cyclic vomiting syndrome is often triggered by psychological or infectious events (see Li BU, Lefevre F, Chelimsky GG, et al).\n\nMedication therapy suppressing acid production, including the use of proton pump inhibitors, provides little, if any, relief for rumination syndrome. The mainstay of treatment for rumination syndrome is behavioral modification consisting of habit reversal. Diaphragmatic or abdominal breathing techniques may be effective treatment because they compete with the urge to regurgitate.\n\nPREP Pearls\n• Rumination syndrome should be considered in patients who experience effortless and painless vomiting for 2 months.\n• Rumination syndrome can be treated with behavioral therapy.\n\nABP Content Specifications(s)\n• Recognize the clinical manifestations of rumination, and manage appropriately\n\nSuggested Readings\n• Chial HJ, Camilleri M, Williams DE, Litzinger K, Perrault J. Rumination syndrome in children and adolescents: diagnosis, treatment and prognosis. Pediatrics. 2003;111(1):158-162. doi:10.1542/peds.111.1.158.\n• Hyams JS, Di Lorenzo C, Saps M, Shulman RJ, Staiano A, van Tilburg M. Functional disorders: children and adolescents. Gastroenterology. 2016;150(6):1456-1468. doi:10.1053/j.gastro.2016.02.015.\n• Li BU, Lefevre F, Chelimsky GG, et al; North American Society for Pediatric Gastroenterology, Hepatology, and Nutrition consensus statement on the diagnosis and management of cyclic vomiting syndrome. J Pediatr Gastroenterol Nutr. 2008;47(3):379-393. doi:10.1097/MPG.0b013e318173ed39.\n• Lightdale JR. Gastroesophageal reflux disease. In: McInerny TK, Adam HM, Campbell DE, DeWitt TG, Foy JM, Kamat DM, eds. American Academy of Pediatrics Textbook of Pediatric Care. 2nd ed. Elk Grove Village, IL: American Academy of Pediatrics; 2017:2063-2075. Pediatric Care Online.\n• Stanghellini V, Chan FK, Hasler WL, et al. Gastroduodenal disorders. Gastroenterology. 2016;150(6):1380-1392. doi:10.1053/j.gastro.2016.02.011."}
{"id" : 3008, "question_text" : "A 32-year-old woman with HIV infection gives birth to a full-term boy via vaginal delivery. Rupture of membranes occurred 5 hours before delivery. The woman was taking antiretroviral medications before conception and continued the same regimen during this pregnancy. Her viral load was undetectable at her most recent obstetric visit. During labor, she did not receive intrapartum HIV prophylaxis. Of the following, the boy's risk of acquiring HIV infection is influenced MOST by", "options" : "[\"lack of intrapartum prophylaxis\", \"length of rupture of membranes\", \"maternal viral load\", \"mode of delivery\"]", "explanation" : "The risk of acquiring HIV infection by the boy in the vignette is influenced most by his mother's viral load. Mother-to-child transmission of HIV usually occurs during the process of labor and delivery. Maternal viral load is a key determinant of the likelihood that an HIV-infected mother will transmit the virus to her child.\n\nMother-to-child transmission of HIV infection in the United States has been curtailed through various measures including routine testing of mothers during pregnancy, antiretroviral prophylaxis for both mother and child, cesarean delivery before the onset of labor or rupture of membranes, and complete avoidance of breastfeeding. In general, women infected with HIV should be treated with antiretroviral drugs during pregnancy to both treat the HIV infection and minimize the potential for transmission to the child.\n\nIntrapartum prophylaxis is not indicated when a woman's HIV viral load is undetectable near or during delivery. Women should receive intrapartum prophylaxis with zidovudine when their viral load is at least 1,000 copies/mL or unknown near the time of delivery.\n\nGenerally, the interval since the rupture of membranes influences the likelihood of HIV transmission, with the risk rising with each hour since membrane rupture. However, for women whose viral load is 1,000 copies/mL or less, rupture of membranes is not associated with an increased risk of transmission and is not an indication for cesarean delivery.\n\nFor women whose viral load is undetectable, such as the mother in the vignette, cesarean delivery is not routinely recommended. In the United States, cesarean delivery at 38 weeks' gestation, before onset of labor or rupture of membranes, is recommended for HIV-infected women whose viral load is greater than 1,000 copies/mL or whose viral load is unknown.\n\nAll infants born to HIV-infected mothers should receive antiretroviral prophylaxis as soon as possible after birth, ideally within 6 to 12 hours. Infants born to mothers who were treated with antiretroviral drugs during pregnancy and had sustained viral suppression (HIV RNA level <50 copies/mL) should receive zidovudine treatment for 4 weeks. In other circumstances, infants should receive either a 2- or 3-drug antiretroviral regimen based on the perinatal guidelines available at https://aidsinfo.nih.gov/guidelines/html/3/perinatal/0.\n\nPREP Pearls\n• Mother-to-child transmission of HIV in the United States has been curtailed through various measures including routine testing of mothers during pregnancy, antiretroviral prophylaxis for HIV-positive mothers and their children, cesarean delivery before the onset of labor or rupture of membranes, and complete avoidance of breastfeeding.\n• Maternal viral load is a key determinant of the likelihood that an HIV-infected mother will transmit the virus to her child.\n• For women with viral loads less than 1,000 copies/mL, intrapartum prophylaxis and routine cesarean delivery before the onset of labor or rupture of membranes is not indicated.\n\nABP Content Specifications(s)\n• Understand the effect of a mother's positive test for human immunodeficiency virus (HIV) on the results of her infant's HIV test\n• Understand the epidemiology of human immunodeficiency virus, including the modes of transmission and how to minimize transmission risk\n\nSuggested Readings\n• American Academy of Pediatrics. Human immunodeficiency virus infection. In: Kimberlin DW, Brady MT, Jackson MA, Long SS, eds. Red Book: 2018 Report of the Committee on Infectious Diseases. Itasca, IL: American Academy of Pediatrics; 2018:459-476. Red Book Online.\n• Brady MT, Persaud D, Moss W. Human immunodeficiency virus infection and acquired immunodeficiency syndrome. In: McInerny TK, Adam HM, Campbell DE, DeWitt TG, Foy JM, Kamat DM, eds. American Academy of Pediatrics Textbook of Pediatric Care. 2nd ed. Itasca, IL: American Academy of Pediatrics; 2016;chap 268:2153-2161. Pediatric Care Online.\n• Panel on Treatment of Pregnant Women With HIV Infection and Prevention of Perinatal Transmission. Recommendations for use of antiretroviral drugs in transmission in the United States. https://aidsinfo.nih.gov/guidelines/html/3/perinatal/0.\n• Sibery G. Preventing and managing HIV infection in infants, children, and adolescents in the United States. Pediatr Rev. 2014;35(7):268-286. doi: 10.1542/pir.35-7-268."}
{"id" : 2066, "question_text" : "A healthy 5-year-old girl is seen for a health supervision visit. Her mother is 38 weeks' pregnant and mentions that she is planning to deliver the new baby at home with a midwife. Her 5-year-old daughter was born full-term in a hospital and was observed in the neonatal intensive care unit for 2 days because of hypoglycemia. The mother has another child who was born at 32 weeks' gestation and spent 6 weeks in the neonatal intensive care unit. The mother has had one urinary tract infection during this pregnancy but reports no other pregnancy-related risk factors (eg, gestational diabetes, group B Streptococcuspositivity). Her blood type is AB-positive. She inquires about post-birth follow-up care for the new baby. Of the following, the MOST appropriate plan is to", "options" : "[\"arrange for a nurse visit to check the neonate's weight and a session with the lactation consultant between 24 and 48 hours after birth\", \"arrange for serum glucose and bilirubin testing at 24 hours of age\", \"instruct the mother to have the neonate seen by her pediatrician between 24 and 48 hours after birth to perform the newborn screen and screen for cyanotic congenital heart defects\", \"instruct the mother to have the neonate seen by her pediatrician within 24 hours after birth to assess for dehydration, feeding problems, and hyperbilirubinemia\"]", "explanation" : "The mother in this vignette should call the pediatrician's office as soon as possible after the birth to arrange an evaluation within 24 hours. Because midwives and other health care professionals attending home births usually stay for only a few to several hours after the birth, an evaluation of the neonate within 24 hours of birth by a pediatric-trained clinician ensures monitoring for feeding problems and dehydration. Another evaluation within 48 hours of this initial visit is also recommended. Together, these evaluations ensure that the neonate receives screening for congenital heart defects, completes the state-required newborn screening for metabolic and other conditions, and is assessed for hyperbilirubinemia.\n\nPlanned home births represent less than 1% of all births in the United States. The American Academy of Pediatrics (AAP) agrees with the American College of Obstetricians and Gynecologists' position that it is safer to deliver in a hospital or birthing center, as compared to at home. Neonates born at home have a 2- to 3-fold increased risk of mortality in the United States. The 2013 AAP policy statement regarding newborn care during and following planned home births was, in part, an effort to reduce this risk by recommending standards for care. The AAP does not recommend a home birth over one in a health care setting.\n\nA neonate born at home should be cared for by a certified health care professional trained in neonatal resuscitation. This provider should only be responsible for the neonate. There should be 2 providers attending the birth, one to care for the mother, and one to care for the neonate. The neonate should receive care according to AAP guidelines for perinatal care, including a physical examination, assessment of growth parameters, frequent assessment of vital signs, screening for hypoglycemia, prophylaxis against gonococcal ophthalmia neonatorum, administration of vitamin K, and vaccination against hepatitis B. The provider team should remain in the home long enough to ensure the mother and neonate are stable. Any neonate requiring more intense monitoring or treatment should be transferred to a medical facility. Examples include a neonate with persistent hypoglycemia despite feeding and a neonate born to a group B Streptococcus–positive mother showing signs of chorioamnionitis. Neonates thought to be less than 37 weeks' gestational age should also be transferred for monitoring and treatment of prematurity-associated conditions.\n\nAn initial evaluation by a pediatric-trained clinician within 24 hours of birth and another evaluation within 24 to 48 hours of that initial visit are needed to ensure that further screening and monitoring procedures are followed. In addition, these 2 visits allow for assessment of feeding problems and dehydration. Home visits with a nurse and a lactation consultant in the first days after birth can augment pediatric office visits but will not provide screening for hyperbilirubinemia and congenital heart conditions. Serum glucose monitoring, if indicated, should commence shortly after birth, not at 24 hours after birth. Waiting until 24 to 48 hours after birth to have a first evaluation by a pediatric provider will fail to capture some cases of severe hyperbilirubinemia, poor feeding, and dehydration. Although this is an appropriate age to perform screening for congenital heart defects, it should not be the initial visit.\n\nPREP Pearls\n\nHospitals and birthing centers are safer locations compared to the home for delivery of all neonates.\n\nNeonates born at home should receive care that is as close to standard hospital care as possible, including having a dedicated provider trained in resuscitation who performs assessments, screening, monitoring, and treatment consistent with recommendations from the American Academy of Pediatrics.\n\nNeonates born at home should be evaluated by a pediatric-trained clinician within 24 hours of birth, and again 24 to 48 hours after this initial visit.\n\nABP Content Specifications(s)/Content Area\n\nPlan appropriate evaluation and management of an infant who was born at home\n\nSuggested Readings\n\nCommittee on Fetus and Newborn. Planned home birth. Pediatrics. 2013;131(5):1016-1020. doi: 10.1542/peds.2013-0575.\n\nRoth P. Pulse oximetry and the neonate. Pediatr Rev. 2016;37(9):402-405. doi: 10.1542/pir.2015-0174.\n\nWarren JB, Phillipi CA. Care of the well newborn. Pediatr Rev. 2012;33(1):4-18. doi: 10.1542/pir.33-1-4."}
{"id" : 2536, "question_text" : "A 3-year-old girl is brought to the emergency department after drinking an unknown amount of mouthwash. The girl's mother found her with an empty bottle of mouthwash in the bathroom. She is unsure how much had been in the bottle. The girl had 4 episodes of nonbloody, nonbilious vomiting prior to arriving at the emergency department. She has a temperature of 36.7°C, heart rate of 138 beats/min, respiratory rate of 12 breaths/min, and blood pressure of 108/66 mm Hg. She appears lethargic and is poorly responsive to painful stimuli. The remainder of the girl's physical examination findings are unremarkable. Her airway, breathing, and circulation are stabilized. Of the following, the BEST next step in this girl's management is to", "options" : "[\"administer activated charcoal\", \"administer fomepizole\", \"order an arterial blood gas analysis\", \"order a bedside blood glucose level\"]", "explanation" : "Correct Answer: D\nThe girl in the vignette has ingested an unknown amount of mouthwash, which contains ethanol (ethyl alcohol). In addition to beer, wine, and liquor, ethanol is found in a wide variety of household items including hand sanitizer, liquid cough and cold medications, cooking extracts (eg, vanilla), mouthwash, and perfumes/colognes. Because ethanol inhibits hepatic gluconeogenesis, children (especially young children) who ingest ethanol can become profoundly hypoglycemic. Therefore, the best next management step for the girl in the vignette is to obtain a bedside blood glucose level.\n\nThe girl has signs of severe mental status alteration, therefore, administration of activated charcoal is contraindicated due to aspiration risk. Although ethanol can cause anion-gap acidosis, which would be detected on an arterial blood gas analysis, it is important to first address life-threatening complications such as severe hypoglycemia and those affecting the airway, breathing, and circulation. Fomepizole is the antidote for ethylene glycol and methanol toxicity; it is not indicated for treatment of ethanol ingestion.\n\nSigns and symptoms of ethanol ingestion or overdose include:\n• Agitation\n• Altered mental status\n• Ataxia\n• Central nervous system depression or coma\n• Hypoglycemia\n• Hypothermia\n• Lethargy\n• Nausea and vomiting\n• Respiratory depression\n• Seizures\n• Slurred speech\n• Tachycardia or bradycardia\n\nThe initial management of acute alcohol toxicity is focused on stabilizing the airway and cardiopulmonary system. Respiratory depression and hypoglycemia are typically the most urgent concerns. Prompt administration of glucose is indicated if hypoglycemia is present; monitoring serial glucose levels until the child has recovered is a critical aspect of care. Hemodialysis may be considered for children with toxic ingestions displaying evidence of end-organ damage or severe acidosis that does not respond to conventional medical therapy.\n\nAlcohol is the substance most widely abused by adolescents. The signs and symptoms of acute ethanol overdose may mask those of other ingested toxic substances. It is important to consider co-ingestion in older children and adolescents with a suspected intentional ingestion.\n\nPREP Pearls\n• In addition to beer, wine, and liquor, ethanol is found in a wide variety of household products (eg, mouthwash, cough and cold medications) that are easily accessible to children of all ages.\n• Ethanol inhibits hepatic gluconeogenesis; children (especially young children) who ingest ethanol can become profoundly hypoglycemic.\n• Alcohol is the substance most widely abused by adolescents. It is important to consider coingestion in an older child or adolescent with a suspected intentional ingestion.\n\nMOCA-Peds Objective\n• Evaluate and manage a child with hypoglycemia.\n\nABP Content Specifications(s)\n• Recognize the signs and symptoms of ethanol intoxication, and manage appropriately\n• Understand that ethanol intoxication may mask toxicity caused by ingestion of other drugs\n\nSuggested Readings\n• Fein DM, Sue YJ. Methanol ingestion. Pediatr Rev. 2011;32(12):549-550. doi:10.1542/pir.32.12.549.\n• Fine JS. Poisoning. In: McInerny TK, Adam HM, Campbell DE, DeWitt TG, Foy JM, Kamat DM, eds. American Academy of Pediatrics Textbook of Pediatric Care. American Academy of Pediatrics; 2021:chap 369. Accessed September 1, 2022. Pediatric Care Online.\n• Khine H. Ethanol in over-the-counter drugs. Pediatr Rev. 1996;17(10):342-343. doi:10.1542/pir.17.10.342.\n• Sheridan DC, Hughes A, Horowitz Z. Adolescent ingestions: various reasons and medications. Pediatr Rev. 2021;42(6):279-289. doi:10.1542/pir.2019-0310.\n• Wang GS, Hoyte C. Common substances of abuse. Pediatr Rev. 2018;39(8):403-414. doi:10.1542/pir.2017-0267."}
{"id" : 1268, "question_text" : "A 15-year-old adolescent is brought to your office with concerns about poor school performance. He complains of difficulty concentrating and remembering facts needed for tests. His parents report that he seems more anxious recently and have observed that he has been intermittently eating larger meals and snacks. They are concerned about his sleep as they have noticed his eyes to appear red. There have been no significant changes at home. He has been a healthy adolescent who has done well in school in previous years. Of the following, the BEST next step in management is to", "options" : "[\"ask the family to keep a record of your patient's sleep habits\", \"direct the family to request an Individualized Education Program\", \"give the family Vanderbilt questionnaires for his teachers to complete\", \"interview your patient separately and ask about drug use\", \"provide the family with contact information for counseling services\"]", "explanation" : "The differential diagnosis for poor school performance in an adolescent includes possible illicit drug use. Although other reasons for academic underachievement are conceivable, the constellation of behavioral and physical symptoms in the patient in this vignette who is otherwise healthy and had previously done well in school is consistent with marijuana use. This includes decreased concentration, increased anxiety, increased hunger, and red eyes. Interviewing the patient separately and asking about drug use is the best next step in management for the patient in this vignette.\n\nMarijuana is the most commonly used illegal substance in the United States and worldwide. Derived from the cannabis plant, marijuana's primary active component is delta-9-tetrahydrocannabinol (THC). Selective cannabis plant breeding has increased the THC content of marijuana, making marijuana more potent today than in the past. Marijuana's street names include pot, grass, dope, ganja, MJ, and hemp. Marijuana can be smoked in a variety of forms (eg, cigarettes, pipes, cigars) or can be mixed with food and ingested (eg, brownies, candies). Marijuana's effects occur within seconds of smoking and 30 to 90 min after ingestion. The peak is 15 to 30 min after smoking and 2 to 3 hours after ingestion. Effects last up to 4 hours after smoking and up to 12 hours after ingestion.\n\nMarijuana produces various physiologic results. The cardiovascular outcomes are most consistent and include the sympathomimetic effects of tachycardia and increased blood pressure. Palpitations, abnormal orthostatic responses, and peripheral vasodilation may also occur. Regular marijuana smoking can produce respiratory problems and can decrease pulmonary function. The harmful chemicals and carcinogens in marijuana smoke increase the risk of respiratory tract cancer and lung damage. Marijuana's other physiologic effects may include conjunctival injection, nystagmus, dry mouth, slurred speech, hunger, and ataxia.\n\nNeurobehavioral consequences of marijuana use include poor executive function, decreased concentration, memory impairment, distorted perception, drowsiness, and impaired cognition. These effects interfere with learning and school performance. By impairing judgment, coordination, and reaction time, marijuana increases risk-taking behaviors (eg, unprotected sex, drug use) and injuries (eg, motor vehicle accidents). Some marijuana users may experience anxiety and panic attacks. Mental health problems such as anxiety, depression, and schizophrenia may worsen with heavy use.\n\nOther reasons for academic problems in this patient are possible. While sleep disturbance, clarified from a record of the patient's sleep habits, is possible and may coexist with marijuana use, other symptoms such as increased hunger make it unlikely as the primary cause for this patient's academic underachievement. Next, an Individualized Education Program is essential in providing for special education services for a student with learning disability or other eligible condition. Children with learning disabilities tend to present before high school, making learning disability less likely in this patient. Eligibility under \"other health impaired\" could be considered should this patient have attention-deficit/hyperactivity disorder (ADHD). Vanderbilt questionnaires are helpful in the assessment of a student with possible ADHD, which may be considered for this patient's difficulty with concentration and poor school performance. However, ADHD is also less likely, given the patient's prior academic success and that his symptoms are accompanied by other clinical features such as increased hunger and red eyes. While counseling services could be helpful in addressing the adolescent's drug use and difficulty with school, providing the family with contact information for these services would not be the next best step in management. The next step would be to interview the adolescent separately about possible drug use.\n\nIllicit drug use should be considered in the differential diagnosis of an adolescent with new onset academic underachievement. Marijuana is the most commonly used illicit drug and has negative immediate and long-term behavioral and health consequences. Early consideration of marijuana use may identify and prevent harm to adolescent users.\n\nPREP Pearls\n• Illicit drug use should be considered in the differential diagnosis of an adolescent with new onset academic underachievement.\n• Marijuana is the most commonly used illicit drug and has negative immediate and long-term behavioral and health consequences.\n• Marijuana's physiologic effects include tachycardia, increased blood pressure, decreased pulmonary function, increased risk of lung cancer, conjunctival injection, nystagmus, dry mouth, slurred speech, hunger, and ataxia.\n• Neurobehavioral consequences of marijuana use include poor executive function, decreased concentration, memory impairment, distorted perception, drowsiness; impaired cognition, judgment, and coordination; and reaction time.\n• Mental health problems such as anxiety, depression, and schizophrenia may worsen with heavy use of marijuana.\n\nABP Content Specifications(s)\n• Recognize the major behavioral consequences of marijuana use/abuse\n• Identify the major physiologic consequences associated with marijuana use/abuse\n\nSuggested Readings\n• Ammerman S, Ryan S, Adelman WP, Committee on Substance Abuse, Committee on Adolescence. Technical report: the impact of marijuana policies on youth: clinical, research, and legal update. Pediatrics. 2015;135(3):e769-e785. doi: http://dx.doi.org/10.1542/peds.2014-4147.\n• Neuspiel DR. Marijuana. Pediatr Rev. 2012;33(7):333-334. doi: http://dx.doi.org/10.1542/pir.33-7-333.\n• Wong GS. Cannabis (marijuana): acute intoxication. UpToDate. Available online only with subscription."}
{"id" : 735, "question_text" : "A 9-year-old girl who has been living in her current foster home for the past 2 months is brought to your office by the foster parents. The couple is concerned because the girl has been \"hearing and seeing things\" around the house that aren't actually there. A few nights prior to the visit, she became very distressed and afraid, stating that she saw a man looking in her second-floor bedroom window. She hears her name being called and becomes distressed about hearing doors slamming when neither has occurred. The girl occasionally wakes up from nightmares. On several occasions, the foster parents have seen her appear to \"space out\" for several minutes during the day, after which she appears scared and states that she had been thinking about \"bad things. ' She occasionally has dramatic and apparently unprovoked mood swings and often has a negative mood. At other times she can engage normally and can get along well with other children. Although they were provided no details, the foster parents were told that the girl witnessed domestic violence in her biological parents' home and that there is a family history of a mental health disorder. Results of her physical examination are within normal limits. Her interaction with you in the office is age appropriate. Of the following, the MOST likely cause of this girl's symptoms is", "options" : "[\"bipolar disorder\", \"depression\", \"posttraumatic stress disorder\", \"schizophrenia\", \"seizure disorder\"]", "explanation" : "Preferred Response: C\nThe young girl in the vignette is exhibiting symptoms commonly found in children who have experienced traumatic events, and she most likely has posttraumatic stress disorder (PTSD). Posttraumatic stress disorder is characterized by a history of exposure to a traumatic event of threatened death, injury, or sexual violence; and at least I event of each of the following 4 types of symptoms over the period of at least 1 month:\n(1) Intrusive recollection, which involves re-experiencing the traumatic event in an intrusive fashion (for her, both nightmares and spacing out episodes or flashbacks);\n(2) Hyperarousal, which may hypervigilance (exemplified by her listening for door slamming and monitoring what is outside the home's windows);\n(3) avoidance, or numbing, symptoms presenting as persistent avoidance of stimuli associated with the trauma or numbing of general responsiveness (not present in the child described in the vignette), and\n(4) negative alterations in trauma associated cognitions and mood (she does have negative moods). Because only 3 of the 4 required PTSD symptom types appear in the child described in the vignette, a diagnosis of PTSD cannot yet be confirmed, but it is the most likely diagnosis of the choices listed.\n\nYoung patients who have experienced trauma may have recurrent hallucinations that invoke the same emotional state as the child's past traumatic experience. For instance, ~ child who recurrently heard one parent striking the other may hallucinate hearing these same noises as a PTSD flashback while reexperiencing the associated emotional fear. [he sound of a door slamming shut, for instance, may be an abuse- associated noise that signaled to the child the likelihood that abuse was about to occur.\n\nOther causes of hallucinations are less likely for this child. Bipolar disorder can cause hallucinations during the manic late, but it is quite uncommon at age 9 years; the child in the vignette has no symptoms of mania. Depression can cause hallucinations when it is severe and prolonged, but no symptoms of depression were noted. Traumatized children commonly experience mood swings and irritability that are not necessarily indicative of a concurrent mood or anxiety disorder. Schizophrenia causes hallucinations, but schizophrenia is very rare in prepubertal children, and the child n the vignette has no history of a schizophrenia prodrome ie, social withdrawal or flattened personality preceding psychosis) or a pervasive loss of awareness of reality that would be characteristic of schizophrenia. Partial seizures can rarely cause hallucinations, which would typically appear during the seizure (with an abrupt onset and offset) and are less complex in experience than in this vignette (eg, a single sensation such as a particular smell).\n\nMany other possible causes of hallucinations exist, the most common of which would include substance abuse and delirium; neither of these causes would be likely for this patient.\n\nPREP Pearls\n• PTSD should be considered in pediatric patients who present with hallucinations in the absence of medical disease.\n• Psychotic disorders like schizophrenia are not a common cause of hallucinations in young children.\n\nAAP Mental Health Competency\n\nKnow the likely differential diagnosis for a child presenting with complaint of hallucinations\n\nSuggested Reading:\n\nAmerican Psychiatric Association. Diagnostic and Statistical Manual f Mental Disorders, Fifth ed. Washington, DC: American Psychiatric association; 2013:271-280\n\nArsenault L, Cannon M, Fisher HL, Polanczyk G, Moffitt T, Caspi A. Childhood trauma and children's emerging psychotic symptoms: a genetically sensitive longitudinal cohort study. Am J Psychiatry. 2011;168: 5-72. doi:10.1176/appi.ajp.2010.10040567\n\n!delson GA. Hallucinations in children and adolescents: considerations n the emergency setting. Am J Psychiatry. 2006;163:781-785. doi: 10.1176/ appi.ajp.163.5.781\n\n!lbridge D, Hillenbrand K, Serwint J. In brief: hallucinogens. Pediatr Rev. 006; 27:314-315. doi: 10.1542/pir.27-8-314"}
{"id" : 2376, "question_text" : "A 6-year-old unimmunized girl is brought to the office after sustaining a fall at home, hitting her face on the backyard coffee table. She did not lose consciousness and is acting appropriately. Her dentition is intact, and there are no loose or broken teeth. She has a small gingival abrasion near her upper frenulum and a 1 cm linear shallow laceration of her upper lip which crosses the vermillion border. She has no tenderness to palpation or swelling of her nose or cheek. Of the following, the MOST appropriate step in management of this girl's condition is to", "options" : "[\"anesthetize and suture the upper lip laceration; administer tetanus vaccine\", \"anesthetize and suture the upper lip laceration; administer tetanus vaccine and tetanus immune globulin\", \"gently irrigate the wounds and allow them to close by secondary intention\", \"order maxillofacial computed tomography to evaluate for facial fractures\"]", "explanation" : "PREP Pearl(s)\nWhen evaluating any wound, a review of the child's tetanus immunization status is important.\nAn unimmunized child with any wound other than a clean, minor wound requires administration of tetanus vaccination and tetanus immune globulin.\nFor lacerations involving the vermillion border of the lip, the first suture should be placed to exactly align the edges of the vermillion border.  \nCritique\nThe girl in the vignette has a small, simple, linear laceration of the upper lip that crosses the vermillion border. This laceration can be repaired in the office or emergency department by appropriately anesthetizing the wound and placing sutures to close the laceration. When evaluating any wound, review of the child's tetanus immunization status is important. Because this girl is unimmunized, she also requires tetanus vaccination and administration of tetanus immune globulin. Administration of the tetanus vaccine alone for any wound that is not clean and minor is not sufficient to provide protection from disease in an unimmunized individual. Table 1 shows recommendations for tetanus prophylaxis in routine wound care.  \nThe primary goals of wound closure are to restore function, achieve hemostasis, and optimize cosmesis. Placement of sutures is considered the standard method for wound closure. Table 2 lists the suggested suture type based on the location of a wound, as well as duration of time to leave sutures in place. Extra care must be taken when suturing a laceration that crosses the vermillion border. The first suture should be placed at the vermillion border to establish accurate alignment; the remainder of the sutures placed should ensure that all vermillion border edges are appropriately aligned. Allowing the wound to heal by secondary intention would leave a large scar and would not achieve the best cosmetic outcome. The girl does not have any evidence of dental or facial injury; the wound appears to be isolated to the soft tissue of the lip, therefore imaging is not indicated. Imaging studies (eg, radiographs, computed tomography) are rarely indicated for children with simple lacerations or wounds.  \nProphylactic antibiotics should not be routinely prescribed for wounds and lacerations. A few special circumstances (eg, bites from dogs, cats, or humans, or puncture wounds through the rubber sole of a shoe) warrant prophylactic antibiotics. Table 3 details suggested antibiotic choices in these circumstances.\nSuggested Reading(s)\nAmerican Academy of Pediatrics. Wound care and tetanus prophylaxis. In. Kimberlin DW, Barnett ED, Lyníeld R, Sawyer MH, eds. Red Book: 2021–2024 Report of the Committee on Infectious Diseases. 32nd ed. American Academy of Pediatrics; 2021. Red Book Online. \nBlack KD, Cico SJ, Caglar D. Wound management. Pediatr Rev. 2015;36(5):207-216. doi:10.1542/pir.36-5-207\nLease JG. Office care of wounds. Pediatr Rev. 1992;13(7):257-261. doi:10.1542/pir.13-7-257\nParlin LS. Repair of lip lacerations. Pediatr Rev. 1997;18(3):101-102. doi:10.1542/pir.18-3-101\nSchmitt B. Sutured wound care. Pediatric Care Advice. Pediatric Care Online. 2022. Accessed September 1, 2022. Pediatric Care Online\nContent Domain\nInfectious Diseases\nABP Content Specification(s) / Content Area(s)\nUnderstand the complications associated with various lacerations, including one through the vermilion border of the lip, and manage appropriately\nPlan the appropriate use of tetanus immune globulin"}
{"id" : 3729, "question_text" : "A middle school sports coach is concerned about the possibility of dehydration during afternoon practice. He seeks advice in outlining a strategy to help athletes stay well hydrated during these 60-minute practice sessions. Of the following, the BEST advice for this coach is", "options" : "[\"carbohydrate-containing fluids (sports drinks) are the beverages of choice for these young athletes\", \"dehydration in young athletes is unlikely in practices that are only 60 minutes in duration\", \"ensure that young athletes are adequately hydrated prior to the beginning of practice\", \"sodium-containing food or fluids should be encouraged in these young athletes to help maintain euhydration during exercise\"]", "explanation" : "Adequate hydration is important to assure athlete safety and optimal performance. Athletes who practice daily are at risk for cumulative fluid deficits. The coach described in this vignette should counsel his young athletes that they should recoup fluid losses after each practice and should assure that they start any training session well hydrated.\n\nYoung athletes should drink enough fluids throughout the day to keep their urine pale yellow (like lemonade), and 1 to 2 hours before training, they should prehydrate with water or a nutritive beverage of choice (eg, milk, \"nut milks,\" or vegetable juices). This timing allows for gastric emptying and absorption before exertion begins. Although fluid recommendations should accommodate individual variability in fluid loss and dietary intake, good starting points for prehydration are: 90 to 180 mL (3-6 oz) in athletes who weigh less than 40 kg, and 180 to 360 mL (6-12 oz) in athletes who weigh more than 40 kg.\n\nFluid losses during exercise are mostly from sweat and evaporative cooling, and sweat rates are highly variable. In adolescents, fluid losses typically range from 500 to 1,300 mL/h, but losses of over 3.5 L/h have been recorded. Environmental conditions and exercise intensity are dominant factors determining fluid loss during physical activity, but there is also marked interindividual variability. Adolescent athletes who are older, highly fit, or male generally have higher sweat rates than athletes who are younger, less fit, or female. Thirst is often recognized when dehydration approaches 3% to 5%, but athletic performance (particularly in endurance activity) falls off at approximately 2% dehydration. Hydration strategies should aim to keep fluid losses to less than 2% body weight. Athletes can easily determine individual rates of fluid loss by changes in pre- and post-exercise weight. Post-exercise weight should be obtained after the athlete has dried off and removed any wet clothing. Each pound of lost weight signifies an uncompensated loss of 16 oz of fluid. The athlete should replenish this volume before the next bout of exercise, and hydration strategies during subsequent workout sessions should be adapted to prevent this excess fluid loss.\n\nSample calculation: Pre-exercise weight: 100 lbs, \"Allowed\" loss of 2%: 98 lbs, Post-exercise weight: 95 lbs, Excess weight loss during exercise: 3 lbs = 48 oz of excess fluid loss. The goal for postexercise rehydration is restoration of pre-exercise weight.\n\nFor many physically active children and adolescents, water is the fluid of first choice, particularly for training sessions lasting less than an hour or of relatively low intensity. Athletes training for longer durations may benefit from ingesting added carbohydrate during their workout to help fuel working muscles. Although gastric tolerance for ingestion during intense exercise is highly variable, performance in adolescents may be enhanced by carbohydrate ingestion of up to 60 g/h. This approximates the amount of carbohydrates found in 2 medium bananas, a large bagel, or 6 fig-filled cookies. Sports drinks often contain carbohydrate concentrations of about 6% to 8% and may be a more convenient way to provide added carbohydrates. Fluids with this carbohydrate concentration appear to empty from the stomach more quickly than plain water, and may reduce stomach discomfort in some athletes. This concentration can also be obtained by diluting apple or other nonacidic fruit juices by half with water.\n\nMost young athletes do not require added sodium during sports activity. Athletes who participate in efforts lasting longer than several hours or who are participating multiple times per day (ie, tournaments or twice daily practices) in warm weather should assure that they are replenishing sodium after activity. Sodium content in adolescent sweat is typically 40 to 70 mmol/L, but some athletes appear to have high salt concentrations in their sweat and are considered \"salty sweaters.\" These athletes are often identified by salt crusting on skin and clothes after activity, and they may benefit from increasing sodium intake during and around the time of activity. Many sports drinks contain 10 to 20 mmol/L of sodium, which stimulates further drinking, but this amount of sodium is not sufficient to significantly replace sweat-related sodium losses.\n\nA clear distinction should be made between sports drinks and energy drinks. Energy drinks contain added caffeine or other herbal stimulants (eg, guarana) and are not recommended for children and adolescents. Sports drinks containing added sodium, carbohydrate, and flavoring may be a convenient way to combine fluid and carbohydrate ingestion during exercise but offer no benefit over water or other nutritive beverages before or after activity.\n\nPREP Pearls\n• Water is the fluid of choice for hydration during physical activity.\n• Fluid loss with exercise is highly variable, and therefore hydration strategies need to be individualized.\n• Many children and adolescents are hypohydrated even before onset of sports activity. Young athletes should recoup fluid losses after each practice and should start any training session well hydrated.\n\nABP Content Specifications(s)\n• Plan optimal age-appropriate replacement for fluid losses associated with athletic activity\n\nSuggested Readings\n• Bergeron MF. Reducing sports heat illness risk. Pediatr Rev. 2013;34(6):270-279. doi:10.1542/pir.34-6-270.\n• Buoite Stella A, Francescato MP, Sims ST, Morrison SA. Fluid intake behavior in athletes during typical training bouts. J Sports Med Phys Fitness. 2017;57(11):1504-1512. doi:10.23736/S0022-4707.16.06722-0.\n• Committee on Nutrition and the Council on Sports Medicine and Fitness. Sports drinks and energy drinks for children and adolescents: are they appropriate? Pediatrics. 2011;127(6):1182-1189. doi:10.1542/peds.2011-0965.\n• LaBotz M. Sports nutrition. In: Harris SS, Anderson SJ, eds. Care of the Young Athlete. 2nd ed. Elk Grove Village, IL: American Academy of Pediatrics; 2010:71-80.\n• LaBotz M. Sports nutrition. In: Kleinman RE, Greer FR, eds. Pediatric Nutrition. 7th ed. Elk Grove Village, IL: American Academy of Pediatrics; 2014:265-298."}
{"id" : 2949, "question_text" : "A 38-month-old boy is brought to clinic in July to establish care. He emigrated from El Salvador 3 months ago. He has no significant medical history, allergies, or medications. He and his mother were detained at the border in a family shelter while awaiting reunification with family members. He underwent basic medical screening, including tuberculosis screening, which had negative results, and received vaccinations. His mother brings his vaccine record from El Salvador, indicating that he received a combination vaccine of hepatitis B, DTaP, and inactivated polio at 2, 4, and 6 months of age. Two months ago at the border shelter, he received Haemophilus influenzae type B, DTaP, MMR, pneumococcal conjugate, and varicella vaccines.\n\nIn addition to hepatitis A, the most appropriate vaccine(s) for this boy at this time is (are)", "options" : "[\"DTaP\", \"Haemophilus influenzae type B and pneumococcal conjugate\", \"Haemophilus influenzae type B, pneumococcal conjugate, and DTaP\", \"none\"]", "explanation" : "The 38 month-old boy in the vignette is up-to-date on his vaccines and, with the exception of hepatitis A vaccine, is not currently due for any more. He was on the catch-up immunization schedule because he was more than 1 month behind on his vaccines when he immigrated to the United States, having only received his 2-month, 4-month, and 6-month vaccines. At the border family shelter, he received his 12-month vaccines (MMR and varicella) and his 15-month vaccines (DTaP, pneumococcal conjugate, and Haemophilus influenzae type B). He is also due to receive the influenza vaccine, which usually expires at the end of June and typically is not available until the fall. Aside from the annual influenza vaccine and the second hepatitis A vaccine in 6 months, he will not be due for additional vaccines (DTaP, inactivated polio vaccine, MMR, varicella) until he turns 4 years old (updated catch-up schedules can be found at the Centers for Disease Control and Prevention. Recommended child and adolescent immunization schedule for ages 18 years or younger.)\n\nPREP Pearls\n• Children who are more than 1 month behind on vaccinations will need catch-up vaccinations per the Centers for Disease Control and Prevention immunization schedule.\n• Hepatitis A vaccine is typically given at 1 year of age, with a second dose 6 months after.\n\nMOCA-Peds Objective\n• Evaluate an internationally adopted child for infectious diseases and appropriate immunizations.\n\nABP Content Specifications(s)\n• Plan an immunization schedule for a child or adolescent who begins receiving immunizations late or whose immunizations are delayed\n\nSuggested Readings\n• Centers for Disease Control and Prevention. Recommended child and adolescent immunization schedule for ages 18 years or younger, United States, 2020. https://www.cdc.gov/vaccines/schedules/hcp/imz/child-adolescent.html.\n• Humiston SG, Atkinson WL, Rand C, Szilagyi PG. Immunizations. In: McInerny TK, Adam HM, Campbell DE, DeWitt TG, Foy JM, Kamat DM, eds. American Academy of Pediatrics Textbook of Pediatric Care. 2nd ed. Elk Grove Village, IL: American Academy of Pediatrics; 2017:160-167. Pediatric Care Online .\n• Linton JM, Green A; Council on Community Pediatrics. Providing care for children in immigrant families. Pediatrics. 2019;144(3):e20192077. doi:10.1542/peds.2019-2077."}
{"id" : 939, "question_text" : "A 16-year-old girl reports a history of irregular menses. Her periods have become progressively less frequent since menarche at 12 years of age. Her last menstrual period was 4 months ago. She is not sexually active and has had no galactorrhea. She is taking college-level classes in school, preparing for her college-entrance exams, and helping care for her sick mother. On physical examination, her vital signs are normal and her body mass index is 31. There are inflammatory acne lesions on her face and back, hyperpigmented velvety-appearing skin at the nape of her neck, and increased body hair. Her thyroid gland is not enlarged, and the diameter of her clitoris is 4 mm.\n\nOf the following, the condition MOST likely responsible for this girl's symptoms and signs is", "options" : "[\"dysfunctional uterine bleeding\", \"emotional stress\", \"hypothyroidism\", \"pituitary adenoma\", \"polycystic ovary syndrome\"]", "explanation" : "Preferred Response: E\nSecondary amenorrhea is defined as an absence of menses for 3 or more months that occurs at least 24 months after menarche, when an ovulatory menstrual pattern should be established. The girl in the vignette underwent menarche 3 years ago and has irregular menses, along with acanthosis nigricans, obesity (body mass index >30), acne, and some increase in body hair, suggesting the diagnosis of polycystic ovary syndrome (PCOS). In addition to exclusion of related conditions (eg, Cushing syndrome or adrenal tumors), the 2003 Rotterdam criteria for the diagnosis of PCOS require the presence of 2 of the following 3 criteria: (1) oligoovulation or anovulation,(2) clinical or biochemical signs of hyperandrogenism, and (3) polycystic ovaries on ultrasonography. Other conditions with similar signs (eg, hyperprolactinemia, congenital adrenal hyperplasia, or Cushing syndrome) must be excluded.\n\nOligoovulation and anovulation present as irregular menses, and hyperandrogenism may present as acne, increased body hair, and rarely, clitoromegaly (a transverse clitoral diameter >3 mm). The extent of hirsutism can be documented using the Ferriman-Gallwey scoring system, with a score from 0 (no hair) to 4 (extensive hair growth) for each of 9 body areas most sensitive to androgens. These sites include the upper lip, chin, chest, abdomen, suprapubic region, arms, thighs, upper back, and lower back. A score of 8 or more is considered significant and suggestive of an increased androgen concentration.\n\nThe term dysfunctional uterine bleeding is reserved for postmenarchal irregularity or prolonged bleeding that occurs as a result of immaturity of the hypothalamic-pituitary axis with resultant anovulatory cycles. It lasts, on average, for 2 years. Etiologic factors for amenorrhea can be grouped under 3 anatomical compartments. The first compartment includes genital tract outlet problems. These are most frequently congenital and cause primary amenorrhea (eg, imperforate hymen). Surgical interventions causing cervical stenosis or uterine synechiae (Asherman syndrome) can result in genital tract outlet obstruction and secondary amenorrhea. The second compartment includes the ovaries. Ovarian causes may be chromosomal (eg, Turner syndrome) or nonchromosomal (eg, after chemotherapy or radiation damage). With ovarian causes, the gonadotropin (follicle-stimulating hormone and luteinizing hormone) levels are elevated, and the condition is referred to as hypergonadotropic hypogonadism. Although most commonly associated with primary amenorrhea, these patients may have some functioning ovarian tissue and present with secondary amenorrhea. The last compartment is the hypothalamic-pituitary axis and is associated with normal or lowered gonadotropin levels (ie, hypogonadotropic hypogonadism).\n\nThe most common conditions causing amenorrhea in an adolescent, after ruling out pregnancy, include stress, excessive exercise, and weight changes. Organic causes include those that are reversible, such as chronic illness associated with stress and weight loss, endocrinopathies (eg, thyroid disease and prolactin and cortisol excess), medications, and drugs. Irreversible organic causes include head trauma, radiation, and tumors. Pituitary adenomas are often microadenomas and are asymptomatic. They do not cause hirsutism or acanthosis nigricans but can be a cause of secondary amenorrhea without galactorrhea. Therefore, a prolactin level should be part of the evaluation. Extensive laboratory studies are warranted in patients who present with signs of virilization. Treatment of secondary amenorrhea requires addressing the underlying condition.\n\nPREP Pearls\n• Irregular menstrual bleeding that persists for 2 years after menarche is unlikely to be the result of physiologic anovulation.\n• PCOS, the most common endocrine condition in adolescents, is often the cause of irregular menstrual bleeding.\n• PCOS increases the risk of type 2 diabetes mellitus and cardiovascular disease in the future.\n\nAmerican Board of Pediatrics Content Specification(s):\n• Know the etiologies of secondary amenorrhea and their respective treatments\n\nSuggested Reading:\n• Bloomfield D. Secondary amenorrhea. Pediatr Rev. 2006;27:113-114. doi:10.1542/pir.27-3-113\n• Emans SJ. Amenorrhea in the adolescent. In: Emans SJ, Laufer MR, Goldstein DP, eds. Pediatric and Adolescent Gynecology. Philadelphia, PA: Lippincott Williams & Wilkins; 2005:214-269\n• Fleishman A, Gordon CM, Neinstein LS. Menstrual disorders: amenorrhea and the polycystic ovary syndrome. In: Neinstein LS, Gordon CM, Katzman DK, Rosen DS, Woods ER, eds. Adolescent Health Care: A Practical Guide. 5th ed. Philadelphia, PA: Lippincott Williams & Wilkins; 2008:691-705\n• Goldstein MA, Dechant EJ, Beresin EV. Eating disorders. Pediatr Rev. 2011;32:508-521. doi:10.1542/pir.32-12-508"}
{"id" : 3136, "question_text" : "A 7-year-old girl presents to your office for concern for precocious puberty with progressive breast development. She is overweight but otherwise healthy. Her mother had her first period at age 15 years and is concerned that if her daughter is starting puberty now, she may be at risk for early menstrual periods. On physical examination, you are able to palpate what feels like a small amount of breast tissue but also an appreciable amount of adipose tissue around her breast area. She has no pubic hair. You are concerned for precocious puberty but also consider that she may simply be overweight, and not have true breast tissue present. You need to determine if this patient needs further workup for precocious puberty by an endocrinologist. Of the following, the BEST test to help make this decision is", "options" : "[\"bone age\", \"brain magnetic resonance imaging\", \"estradiol level\", \"17-hydroxyprogesterone level\", \"luteinizing hormone level\"]", "explanation" : "Precocious puberty is defined as the development of pubertal signs before 8 years of age in girls and before 9 years of age in boys. In 1999, the Pediatric Endocrine Society recommended lower age limits for girls: before 6 years of age for blacks and before 7 years of age for whites. However, considerable controversy still exists concerning how best to define precocious puberty. A significant risk associated with precocious puberty is loss of final adult height caused by early fusion of epiphyseal growth plates from sex steroid exposure.\n\nThe best way to determine if the child in this vignette will have impaired final height is to make a height prediction based on bone age. A bone age radiograph (radiograph of the left hand and wrist) can be compared to that expected for the patient's current chronological age, and along with the patient's current height, can be used to make a height prediction. Numerous bone age atlases and standards exist to make this height prediction, which is usually done by a pediatric endocrinologist. When the bone age is delayed in an otherwise normal child, often there will be period of catch-up growth, and final height will be predicted to be higher than the current growth percentile. Similarly, when bone age is advanced, there is potentially less time for catch-up growth, and final height may be compromised.\n\nA brain magnetic resonance imaging scan is important to consider, especially if there are any neurologic symptoms or if the family ultimately opts to treat the child for precocious puberty. However, only 2% of girls with central precocious puberty starting between 6 and 8 years of age have abnormal brain imaging studies. The incidence of relevant findings is much higher for girls with central precocious puberty starting before 6 years of age. A better first test would be a bone age study.\n\nLaboratory results are also important in the evaluation of this child. A baseline luteinizing hormone (LH) level of greater than 0.3 IU/L, a gonadotropin-releasing hormone-stimulated LH level above 4 to 8 IU/L, and a random estradiol level in the pubertal range (> 20 pg/mL or > 73 pmol/L) may provide supportive evidence for progressive central precocious puberty. However, this child has evidence of puberty on physical examination and concern that breast development is progressing, so the question is not whether she has pubertal findings, but rather whether she would benefit from treatment. This is where the bone age study is most helpful. The compromised final height prediction should then prompt these additional laboratory tests, which can be sent by the consulting endocrinologist.\n\nA 17-hydroxyprogesterone level test is used to screen for 21-hydroxylase deficiency, a form of congenital adrenal hyperplasia. In this condition, signs of adrenarche (androgen exposure) such as pubic hair would be expected. As early breast development is a separate issue, it would not be appropriate to include a 17-hydroxyprogesterone level in the evaluation of a patient for central precocious puberty.\n\nPREP Pearls\n• Bone age can he used to predict final height, using one of many standard atlases and the patient's current age and height.\n• A compromised final height prediction from an advanced hone age should lead to consideration of additional workup for many conditions.\n\nAmerican Board of Pediatrics Content Specification(s)\n• Understand the relationship between bone age and chronologic age\n\nSuggested Reading\n• Chalumeau M, Chemaitilly W, Trivin C. Adan L, Brian G. Brauner R. Central precocious puberty in girls: an evidence-based diagnosis tree to predict central nervous system abnormalities. Pediatrics. 2002;109(1):6167. doi:10.1542/peds.109.1.61.\n• Greulich W, Pyle S. Radiographic Atlas of Skeletal Development of the Hand and Wrist. 2nd Edition. Redwood City, CA: Stanford University Press; 1999.\n• KapIowitz P. Treatment of central precocious puberty. Cu r r Op in Endocrinal Diabetes Obes. 2009;16(1):31-36. doi:10.1097/ MED.0b013e328320a650.\n• Kaplowitz PB, Oberfield SE. Reexamination of the age limit for defining when puberty is precocious in girls in the United States: implications for evaluation and treatment; Drug and Therapeutics and Executive Committees of the Lawson Wilkins Pediatric Endocrine Society, Pediatrics. 1999;104(4 Pt 1):936-941."}
{"id" : 1216, "question_text" : "A 14-year-old adolescent presents with emesis this morning, following 3 days of worsening abdominal pain and decreased energy. The patient has a history of pre–B cell acute lymphoblastic leukemia diagnosed 7 years ago, which was treated with chemotherapy for 3 years. His disease recurred and he underwent bone marrow transplant earlier this year. He subsequently developed graft versus host disease and was on a tapering course of prednisone over the last 2 months. He was most recently taking 5 mg orally once daily until his prednisone was discontinued 1 week ago. You order laboratory tests as a part of your evaluation. Of the following, the MOST likely laboratory abnormality will be", "options" : "[\"hypochloremia\", \"hyperglycemia\", \"hypokalemia\", \"hyponatremia\", \"metabolic alkalosis\"]", "explanation" : "The boy in the vignette has recently been weaned off glucocorticoid treatment and has developed adrenal insufficiency. Even when slowly weaned off steroid treatment, some patients may experience hypothalamic-pituitary-adrenal (HPA) axis dysfunction and subsequently develop signs of adrenal insufficiency (AI). Symptoms of AI can include weakness, fatigue, anorexia, nausea, and abdominal pain. Acute symptoms can also include muscle and joint pain and hypotension.\nThe causes of AI can be considered under 2 general headings: primary AI caused by destruction of the adrenal glands themselves, and secondary AI caused by disordered HPA axis function. In primary AI, hyponatremia and hyperkalemia occur because of mineralocorticoid deficiency. This is seen in conditions such as congenital adrenal hyperplasia, Addison disease, or other conditions leading to primary disease.\nSecondary AI can occur after exogenous steroid treatment, after cure of Cushing syndrome, and from hypothalamic or pituitary lesions. As occurred with the boy in the vignette, in some cases the HPA axis remains suppressed despite prolonged tapering of exogenous glucocorticoids. The resulting decreased adrenocorticotropic hormone secretion leads to isolated glucocorticoid deficiency, which causes retention of free water and subsequent hyponatremia. Because the renin-angiotensin system is not affected, potassium concentrations are generally normal. However, if the adrenal glands have atrophied from prolonged suppression from exogenous steroid use, then it is possible to have abnormalities in both sodium and potassium.\nOther biochemical abnormalities that can occur in both forms of AI include metabolic acidosis and hypoglycemia. Hypochloremia is not consistently associated with AI.\nPREP Pearls\n Suppression of the hypothalamic-pituitary-adrenal axis after steroid withdrawal can lead to secondary adrenal insufficiency (AI).\n In secondary AI, isolated hyponatremia without potassium abnormalities is a common finding. This is in contrast to primary AI, where hyponatremia and hyperkalemia are frequently observed.\nABP Content Specifications(s)\n Recognize"}
{"id" : 2364, "question_text" : "A 6-year-old boy is seen for a health supervision visit. His developmental milestones have been achieved on time. His physical examination findings are remarkable for downward slanting palpebral fissures, mandibular and malar hypoplasia, micrognathia, notching of the lower eyelid, and small ears bilaterally. The remainder of his examination findings are normal. The boy wears a bone-anchored hearing aid for conductive hearing loss. The family history is unremarkable. Of the following, this boy's MOST likely diagnosis is", "options" : "[\"22q11.2 deletion syndrome\", \"Noonan syndrome\", \"Pierre-Robin sequence\", \"Treacher Collins syndrome\"]", "explanation" : "PREP Pearl(s)\nTeacher Collins syndrome, a type of mandibulofacial dysostosis, is a developmental disorder of the mandible and facial bones.\nClassic craniofacial features of Treacher Collins syndrome include downward slanting palpebral fissures, mandibular and malar hypoplasia, micrognathia, notching of the lower eyelid, and ear anomalies.\nTeacher Collins syndrome is inherited in an autosomal dominant and recessive pattern with both interfamilial and intrafamilial variability. \nCritique\nThe findings of the boy in the vignette are most consistent with a diagnosis of Treacher Collins syndrome (TCS). Treacher Collins syndrome, a type of mandibulofacial dysostosis, is a developmental disorder of the mandible and facial bones. The features are symmetric and present at birth. Other examples of dysostosis include cleidocranial dysostosis, Crouzon syndrome, and Klippel-Feil syndrome. \nTeacher Collins syndrome is characterized by the following craniofacial features (Figure 1):\nEye: Downward slanting palpebral fissures, Coloboma of lower eyelid, Absent or hypoplastic lashes and nasolacrimal ducts\nEar: Microtia or anotia, Absent or hypoplastic external auditory canals, Conductive hearing loss, normal inner ear structures\nJaw: Micrognathia or retrognathia\nMidface: Hypoplasia of zygomatic arch and lateral aspect of orbits, leading to characteristic downward slant to palpebral fissures, Choanal atresia\nFacial hair: Growth extends in the preauricular region to the lateral cheekbones\nDental: Tooth agenesis, Enamel opacities\nTeacher Collins syndrome is inherited in an autosomal dominant (TCOF1, POLR1D, POLR1B) or autosomal recessive (POLR1C, POLR1D) pattern. There is variability in expression in both related and unrelated individuals. Additional evaluation recommended for a child diagnosed with TCS is outlined in the Table.\nThe 22q11.2 deletion syndrome is characterized by abnormalities of the palate (cleft palate and velopharyngeal incompetence) and/or heart (tetralogy of Fallot, ventricular septal defect, and truncus arteriosus), immune defects, characteristic facial features (bulbous nose, micrognathia, hooded eyelids, and prominent nasal bridge) (Figure 2), and developmental delay.\nClassic features of Noonan syndrome include congenital heart defect (pulmonary valve stenosis), short stature, dysmorphic features (down slanted palpebral fissure, epicanthal folds, wide-set eyes, low-set posteriorly rotated ears, webbed neck, pectus excavatum, and/or carinatum) (Figure 3), and developmental delay.\nPierre-Robin sequence (Figure 4) is characterized by micrognathia that leads to glossoptosis, with or without cleft palate. Additional craniofacial and ear anomalies with hearing loss are not seen.\nSuggested Reading(s)\nKatsanis SH, Jabs EW, Adam MP, et al, eds. Treacher Collins syndrome. In: GeneReviews [Internet]. University of Washington, Seattle; 1993.\nKruk-Marszalek BA, Wójcicki P, Dowgierd K, Śmigiel R. Treacher Collins syndrome: genetics, clinical features and management. Genes (Basel). 2021;12(9):1392. doi:10.3390/genes12091392\nMarion RW, Samanich J. Fascial dysmorphia. In: McInerny TK, Adam HM, Campbell DE, DeWitt TG, Foy JM, Kamat DM, eds. American Academy of Pediatrics Textbook of Pediatric Care (Online). 2nd ed. American Academy of Pediatrics; 2017:chap 148. Pediatric Care Online\nContent Domain\nGenetics\nABP Content Specification(s) / Content Area(s)\nRecognize the genetic and clinical features associated with various types of dysostosis, including Treacher Collins syndrome"}
{"id" : 1584, "question_text" : "A 2-year-old girl presents to your office for evaluation of refusal to bear weight. On the previous day, the girl fell sideways off a slide at the park. She cried at the time of injury, and since then, will not bear weight on her right leg. The girl was seen at an urgent care facility, where radiographs of her right hip, femur, knee, and lower leg were read as normal. Her parents are concerned because she still refuses to walk. The girl has not had any fever, change in appetite, change in energy level, or joint swelling. On physical examination, she has normal range of motion of the hip and knee. She appears to have tenderness over the distal third of the right tibia, but this is difficult to assess, because the girl was very apprehensive during the examination.\n\nOf the following, the next BEST step in evaluation and management for this girl is to", "options" : "[\"place a cast on the lower extremity and repeat radiography in 2 weeks\", \"obtain laboratory results, including complete blood cell count and inflammatory markers\", \"perform ultrasonography of the hip\", \"perform bone scintigraphy\", \"reassure the family that her examination findings and radiographs are not concerning\"]", "explanation" : "Correct Answer: A\nThe history and physical examination of the girl in the vignette suggests an occult tibia fracture. She should be treated with a cast for 2 weeks, then reassessed with clinical evaluation and repeat radiography.\n\nChildren between the ages of 1 and 5 years are especially susceptible to \"toddler fractures,\" oblique fractures of the distal tibia. These fractures can occur with seemingly minor injuries. Tenderness of the distal tibia is often the only sign of toddler fracture, which can be difficult to discern in an apprehensive, young child. Toddler fractures may be difficult to see on anteroposterior and lateral radiographs of the tibia and fibula taken immediately after an injury. The addition of oblique views increases the sensitivity of radiographic studies. Approximately 10 to 14 days after injury, radiographs typically show signs of healing at the fracture site. Therefore, in a child with a history and physical examination that point to the diagnosis of toddler fracture, the affected leg should be placed in a cast and the child should undergo reevaluation about 2 weeks later. Because young children are often unable to give a clear description of their injury and symptoms, any child refusing to bear weight should receive a complete evaluation of the spine and lower extremities.\n\nThe girl in the vignette had a witnessed fall and does not exhibit systemic symptoms or joint swelling. This history suggests an acute injury. Laboratory studies are not indicated, because the child does not have any evidence of infection or inflammatory arthritis. Joint infection and arthritis are unlikely given the absence of joint effusion and normal knee and hip motion. Reassurance alone would not be adequate management for this child, given her high likelihood of fracture. Hip ultrasonography can be used to evaluate for effusion in a child with suspected septic arthritis, which should be included in the differential diagnosis for a child with refusal to bear weight and decreased hip range-of-motion. However, the girl in the vignette has normal hip motion. Although bone scintigraphy would show a toddler fracture, this study involves a high dose of radiation and, in a young child, would require sedation; therefore, bone scintigraphy would not be recommended in this case.\n\nPREP Pearls\n• Children between the ages of about 1 and 5 years are especially susceptible to \"toddler fractures,\" oblique fractures of the distal tibia.\n• Toddler fractures can occur with seemingly minor injuries.\n• Oblique radiographs may show toddler fractures that are not seen on anteroposterior and lateral views.\n\nABP Content Specifications(s)\n• Recognize the clinical findings associated with occult fractures that may or may not affect gait in patients of various ages\n• Recognize the clinical findings associated with growth plate fractures and injuries\n\nSuggested Readings\n• Herman MJ, Martinek M. The limping child. Pediatr Rev. 2015;36(5):184-195; quiz 196-197. doi: http://dx.doi.org/10.1542/pir.36-5-184.\n• Sarwark JF, LaBella CR, eds. Pediatric Orthopaedics and Sports Injuries: A Quick Reference Guide. 2nd ed. Elk Grove Village, IL: American Academy of Pediatrics; 2014."}
{"id" : 3778, "question_text" : "Several neonates in the newborn nursery have hand or foot polydactyly. One neonate is of African descent and has a small piece of tissue adjacent to the fifth digit on the hand. A second neonate is of European descent and has an extra fully formed first digit on the hand. A third neonate has a great toe that has 2 nails. A fourth neonate has a duplicated second digit on the foot. Each family is concerned about other potential abnormalities in their newborn. The family who can be MOST reassured is the one whose newborn has the abnormality located", "options" : "[\"centrally on the foot\", \"postaxially on the hand\", \"preaxially on the foot\", \"preaxially on the hand\"]", "explanation" : "Of the families described in the vignette, the family that can be most reassured is the one whose newborn has a postaxial polydactyly on the hand, because this particular location of polydactyly is least associated with other potential abnormalities. Children with polydactyly in the preaxial and central locations are more likely to have other congenital abnormalities.\n\nPolydactyly is the condition of having more than 5 digits on the hand or foot. Postaxial polydactyly occurs on the ulnar aspect of the hand or the fibular aspect of the foot. Preaxial polydactyly occurs on the radial aspect of the hand or the tibial aspect of the foot. Central polydactyly occurs on the other digits.\n\nPostaxial polydactyly is more common in individuals of African descent with an autosomal dominant inheritance pattern, and it typically has the lowest association with other abnormalities. Postaxial polydactyly of the hand in individuals of European descent is more likely to be a recessive trait and to be associated with a syndromic condition such as Ellis-Van Creveld syndrome. Preaxial polydactyly occurs more often in individuals of European descent and is more often associated with other abnormalities including trisomy 21, Fanconi anemia, and VACTERL association. Central polydactyly is the rarest form of polydactyly and is also often associated with multiple congenital abnormalities.\n\nManagement of polydactyly depends on the examination findings. A preaxial or postaxial polydactyly that is without any bony structures and attached by a thin stalk may be ligated with a suture by the general pediatrician. A polydactyly with a thicker stalk or bony structure should be referred to a specialist for removal. A genetics referral may be prudent for individuals with postaxial polydactyly without a family history and for patients with preaxial or central polydactyly.\n\nPREP Pearls\n• Postaxial polydactyly is less likely than other polydactylies to be associated with other abnormalities.\n• A genetics referral should be considered for patients who have postaxial polydactyly without a family history and for patients with preaxial or central polydactyly.\n\nABP Content Specifications(s)\n• Plan the appropriate management of polydactyly and understand when referral is appropriate\n\nSuggested Readings\n• Chung EK, Gable EK, Golden WC, et al. Current scope of practice for newborn care in non-intensive hospital settings. Hosp Pediatr. 2017;7(8):471-482. doi:10.1542/hpeds.2016-0206.\n• Guo B, Lee SK, Paksima N. Polydactyly: a review. Bull Hosp Jt Dis. 2013;71(1):17-23. http://hjdbulletin.org/files/archive/pdfs/101.pdf.\n• Kaur H, Campbell DE. Physical examination of the newborn. In: McInerny TK, Adam HM, Campbell DE, DeWitt TG, Foy JM, Kamat DM, eds. American Academy of Pediatrics Textbook of Pediatric Care. 2nd ed. Elk Grove Village, IL: American Academy of Pediatrics; 2017:802-819. Pediatric Care Online.\n• Rosen O, Marion RW, Samanich JM. Common congenital anomalies. In: McInerny TK, Adam HM, Campbell DE, DeWitt TG, Foy JM, Kamat DM, eds. American Academy of Pediatrics Textbook of Pediatric Care. 2nd ed. Elk Grove Village, IL: American Academy of Pediatrics; 2017:828-837. Pediatric Care Online."}
{"id" : 1943, "question_text" : "A 15-year-old boy is being evaluated 1 day after his sister witnessed him having a seizure. The boy was in the bathroom getting ready for school when his sister heard a loud \"thud.\" She opened the door to the bathroom and found her brother lying on the floor, unresponsive, with his arms and legs \"jerking.\" The muscle movements lasted for less than 1 minute, after which her brother became alert and seemed \"back to normal.\" The boy does not recall the episode. He reports feeling lightheaded for several minutes after the event. His medical history is otherwise unremarkable. His mother reports that she experienced similar episodes several years ago. The boy is currently asymptomatic and his physical examination findings are normal. He would like to return to basketball practice the following day. Of the following, the MOST appropriate next steps in this boy's evaluation and management are", "options" : "[\"full clearance for sports with no further evaluation needed\", \"rest from school and sports for 48 hours, followed by gradual return to activities if he remains asymptomatic\", \"withhold clearance from sports, and perform a cardiac evaluation including electrocardiography\", \"withhold clearance from sports, and perform a neurologic evaluation including magnetic resonance imaging of the brain\"]", "explanation" : "The boy in the vignette had an episode of unresponsiveness accompanied by muscle jerking, with a very rapid recovery. He did not have a postictal phase with lethargy or confusion. His history suggests a syncopal episode with myoclonic jerks rather than a seizure. The trigger for this episode is unclear. His mother has a history of similar episodes, which could indicate an inherited ion channel disorder predisposing him to arrhythmia such as long QT syndrome or catecholaminergic polymorphic ventricular tachycardia. Although neurocardiogenic (vasovagal) and situational syncope (eg, due to micturition, defecation, or the sight of blood) are more common than cardiogenic syncope due to arrhythmia, the boy should be evaluated by a cardiologist given the unclear etiology of his syncope and his family history. If this evaluation does not reveal an underlying cardiac disorder, the boy could be cleared for basketball participation.\n\nHead injury, such as concussion, should also be included in the differential diagnosis for this child's episode of unresponsiveness. Myoclonic activity can be seen in association with concussion. Individuals with concussion can be returned to physical activity in a stepwise fashion, once asymptomatic for 24 to 48 hours, gradually increasing the intensity and difficulty of activity each day if they do not experience increased symptoms. Because the boy had a rapid recovery and is currently asymptomatic with normal physical examination findings, concussion is unlikely.\n\nIn a child with a suspected seizure, neurology evaluation, including electroencephalography, laboratory studies, and neuroimaging may be warranted. However, seizure activity would not preclude participation in basketball. Children with seizure disorders are less likely to participate in sports and physical activity than their siblings. Physicians should look for ways to promote safe sports participation for children with epilepsy. Certain sports, such as swimming, diving, archery, and powerlifting, may pose a high risk to an athlete with a poorly controlled seizure disorder (and potentially to teammates and officials). Therefore, individuals with frequent seizures, despite appropriate treatment with antiepileptic medications, should have an individualized evaluation with discussion of appropriate types of activity and ways to mitigate risk.\n\nPREP Pearls\n\nSyncope with myoclonic jerks may be mistaken for seizure activity.\n\nHaving a seizure disorder, even when poorly controlled, is not an absolute contraindication to sports participation.\n\nCertain sports, such as swimming, diving, archery, and powerlifting, may pose a high risk to an athlete with a poorly controlled seizure disorder.\n\nABP Content Specifications(s)/Content Area\n\nUnderstand the guidelines for sports participation for patients who have a seizure disorder\n\nSuggested Readings\n\nAnderson JB, Willis M, Lancaster H, Leonard K, Thomas C. The evaluation and management of pediatric syncope. Pediatr Neurol. 2016;55:6-13.\n\nBernhardt DT, Roberts WO, eds. Preparticipation Physical Evaluation. 4th ed. Elk Grove Village, IL: American Academy of Pediatrics; 2010.\n\nWong J, Wirrell E. Physical activity in children/teens with epilepsy compared with that in their siblings without epilepsy. Epilepsia. 2006;47(3):631-639. doi: 10.1111/j.1528-1167.2006.00478.x."}
{"id" : 1315, "question_text" : "A 2-week-old female newborn is brought to your office for a health supervision visit. The mother is concerned that, for the past 2 days, the baby has not been breastfeeding well. The newborn tires after feeding for only 5 minutes and had only 2 wet diapers today. She was delivered at 39 weeks of gestation to a 23-year-old gravida 1 para 0 woman. Her Apgar scores were 8 and 9 at birth and 5 minutes, respectively, and she had a birthweight of 3,500 g. The pregnancy was uncomplicated and the mother was group B Streptococcus negative.\n\nOn physical examination, the newborn appears sleepy. Her heart rate is 270 beats/min, respiratory rate is 60 breaths/min, blood pressure is 82/40 mm Hg, and oxygen saturation is 95% in room air. She is warm and well perfused. Breath sounds are clear. A 2/6 systolic murmur can be heard at the left upper sternal border, with no gallop or rub. Her liver is palpable 2 cm below the right costal margin. Capillary refill time is 2 seconds. You refer the patient to the emergency department where they perform electrocardiography.\n\nOf the following, the MOST likely finding on this test is", "options" : "[\"atrial fibrillation\", \"junctional tachycardia\", \"sinus tachycardia\", \"supraventricular tachycardia\", \"ventricular tachycardia\"]", "explanation" : "Correct Answer: D\nThe newborn in this vignette was previously well, with no history of a murmur at birth. Her oxygen saturation is minimally decreased, but is not suggestive of a mixing lesion or congenital heart disease with decreased pulmonary blood flow. The most common cause for a heart rate of 270 beats/min in this age group is an accessory pathway–mediated, reentrant supraventricular tachycardia (SVT). This may be associated with Wolff-Parkinson-White syndrome (WPW) or pre-excitation. Electrocardiography (ECG) in the usual form of reentrant SVT will be narrow complex and very regular. P waves will not be seen before the QRS complexes.\n\nAtrial fibrillation (AF) would be very unlikely in childhood. If present, it might suggest an ingestion. Other possible causes include congenital heart disease, mitral valve disease with a large dilated left atrium, and alcohol binge drinking (also known as holiday heart). Atrial fibrillation is thought to be the cause of sudden death in patients with WPW, in whom the rapid atrial rate causes a very rapid ventricular rate and ultimately ventricular fibrillation. The ECG in AF is irregular, with small, difficult-to-identify P waves. If a patient with WPW has syncope, it must be presumed that there was AF with rapid conduction, which places the child at risk for sudden death.\n\nEctopic atrial tachycardia is a more common arrhythmia in infancy, would likely have a rate of 220 to 240 beats/min, and would be regular. Premature atrial contractions may occur when the patient is in sinus rhythm. A visible change may be noted in the P wave axis when the arrhythmia starts. Since P waves cannot always be seen on telemetry, a 12-lead ECG is needed to assess for a change in P wave morphology. This rhythm may create hemodynamic instability, but may also be a rate that the infant can tolerate for several hours. In that case, the infant may become fatigued over time.\n\nAtrial flutter may be seen in newborns and often resolves spontaneously soon after birth. It is also seen in older children and adolescents with structural heart disease. The ECG will show large sawtooth P waves, especially in V1. Variable atrioventricular block may be seen with 2 to 4 P waves for each QRS. The P wave morphology may not be appreciated until the patient has been given adenosine, producing a transient atrioventricular block.\n\nPatients with congenital heart disease, who have undergone extensive surgical procedures such as the Fontan, may have P waves that are very small and difficult to see. Sinus node dysfunction and a resting bradycardia may also be seen. In such patients, who may have a baseline heart rate of 50 beats/min, a heart rate of 120 beats/min should raise suspicion that they are actually experiencing an atrial flutter.\n\nCongenital junctional ectopic tachycardia is very rare. The ECG will show a junctional rate that is more rapid than the sinus rate. Both the sinus and junctional rates will be regular, but different, causing a varying \"P-R\" interval due to atrioventricular dissociation.\n\nThe baby in the vignette has a heart rate that is too rapid for sinus tachycardia in a term newborn, and a history of fluid loss or fever would be expected. The ECG in sinus tachycardia will show P waves before each QRS and the axis will be normal, with upright P waves in II, III, and aVF.\n\nVentricular tachycardia (VT) may occur in a newborn, but a rate of 270 beats/min would likely cause more cardiovascular instability than is described in the vignette. In VT, the QRS would be wide for age, and dissociation is seen (the ventricular rate will be faster than the sinus rate, just as described for junctional rhythm). A benign form of ventricular ectopy can be seen in newborns, in which case, the ventricular and sinus rates are almost identical. When the sinus node accelerates, the ventricular rhythm becomes suppressed. This usually resolves spontaneously within a few months after birth. Children with occasional ventricular ectopy are often asymptomatic. Those with VT and some children with ventricular ectopy may present with syncope or palpitations, and often will have symptoms with exercise or high catecholamine"}
{"id" : 2915, "question_text" : "A 3-year-old boy is brought to the office by his mother for a health supervision visit. His mother is planning to enroll him in preschool, but is uncertain which educational setting would be best. The boy speaks in 3-word sentences/phrases and follows 3-step commands. He can balance on each foot for 3 seconds. He can draw lines and stack up to 3 blocks. The boy knows his sex and age. He can match letters. He enjoys playing with other children and has an easy temperament. He is toilet trained. The boy has no significant medical history and his growth and physical examination findings are within normal limits. Of the following, the MOST appropriate recommendation is a", "options" : "[\"general education program\", \"general education program with specialized academic instruction\", \"general education program with physical therapy\", \"special education program\"]", "explanation" : "The 3-year-old boy in the vignette has delayed fine motor skills but is age appropriate in all other domains of development. He does not meet the eligibility criteria for a special education program or services under any of the 13 disability categories (Item C116). Therefore, this boy would be placed in a general education program. He does not need specialized academic instruction for a learning disability or physical therapy for gross motor delay.\n\nThe Individuals with Disabilities Education Act (IDEA) is a federal law that provides for early Intervention (EI) and special education services for children with learning or physical disabilities. Early intervention programs serve children from birth to 3 years of age with developmental delays (physical, cognitive, communication, social/emotional, adaptive) or a condition with high probability of developmental delay. Early Intervention programs are family-centered and provide parents with knowledge and resources (eg, developmental therapies, family training) to improve their child's development. In the United States, EI programs serve about 2% of children from birth to age 3 years, whereas special education services serve about 13% of people 3 to 21 years old.\n\nOnce a child is determined to be eligible under any of the 13 disability categories, an Individualized Education Program is developed to provide services and accommodations to meet the child's special educational needs. Children who need intensive services may be placed in a special classroom; however, services should be delivered in the least restrictive environment, with the child supported in a \"mainstream\" standard educational setting with typical peers whenever possible. Including children with disabilities in a regular classroom promotes empathy, understanding, and appreciation of diversity in the classroom students. With educational inclusion, the student with a disability may achieve greater social, communication, and academic gains. Although the general education teacher may find it challenging to adequately meet the educational needs of the included students, ultimately educational placement should be determined based on the needs of the individual student.\n\nPREP Pearls\n• Early intervention programs serve children from birth to 3 years of age with developmental delays (physical, cognitive, communication, social/emotional, adaptive) or a condition with high probability of developmental delay.\n• The 13 disability categories for special education services are autism, deaf-blindness, deafness, emotional disturbance, hearing impairment, intellectual disability, multiple disabilities, orthopedic impairment, other health impairment, specific learning disability, speech or language impairment, traumatic brain injury, and visual impairment (including blindness).\n• Special education services should be delivered in the least restrictive environment, with the child supported in a \"mainstream\" standard educational setting with typical peers whenever possible.\n\nABP Content Specifications(s)\n• Understand the general goals of early intervention programs for children of various ages who have learning disabilities\n• Understand the educational criteria required for placement in special classrooms and the factors affecting those decisions\n• Understand the advantages and disadvantages of educational inclusion for patients of various ages who have learning or physical disabilities\n\nSuggested Readings\n• Augustyn M, Wolf M. Learning disability. In: Augustyn M, Zuckerman B, eds. Zuckerman Parker Handbook of Developmental and Behavioral Pediatrics for Primary Care. 4th ed. Philadelphia, PA: Wolters Kluwer; 2019:317-321.\n• Fogler JM, Barbaresi WJ. Learning disabilities. In: Voigt RG, Macias MM, Myers SM, Tapia CD, eds. Developmental and Behavioral Pediatrics. 2nd ed. Itasca, IL: American Academy of Pediatrics; 2018:367-381.\n• Frankowski BL. Learning difficulty. In: McInerny TK, Adam HM, Campbell DE, DeWitt TG, Foy JM, Kamat DM, eds. American Academy of Pediatrics Textbook of Pediatric Care. 2nd ed. Itasca, IL: American Academy of Pediatrics; 2016;chap 172:1484-1489. Pediatric Care Online.\n• Rose L, Herzig LD, Hussey-Gardner B. Early intervention and the role of pediatricians. Pediatr Rev. 2014;35(1):e1-e10. doi: 10.1542/pir.35-1-e1."}
{"id" : 3667, "question_text" : "A term male neonate born to a mother with limited prenatal care has a positive newborn screening result for IgM antibodies specific for Toxoplasma gondii. Further testing in conjunction with an infectious disease specialist confirms congenital toxoplasmosis. The original source of the infection is determined to be undercooked meat consumed by the boy's mother and his 7-year-old sister. The sister has no medical problems and has remained well with no symptoms but is also found to have been infected with T gondii. Of the following, the BEST advice regarding the management of the sister's infection is that she should receive", "options" : "[\"prophylaxis if she becomes immunocompromised\", \"reassurance that she does not require treatment\", \"treatment if she becomes pregnant\", \"treatment in conjunction with her brother\"]", "explanation" : "Correct Answer: A\nThe girl in this vignette is currently healthy and asymptomatic but is infected with Toxoplasma gondii; this acute infection will likely become latent. If she later becomes immunocompromised, her latent infection may reactivate and cause severe symptoms. She should therefore receive prophylaxis if she becomes immunocompromised.\n\nReassurance alone is inadequate, because she may develop symptoms in the future that would require treatment. Treatment if she becomes pregnant is unnecessary if she remains healthy, because her infection at that point would be considered latent and carries no risk to the fetus. Treatment in conjunction with her brother is also not necessary as she is currently asymptomatic.\n\nToxoplasma gondii is a globally found obligate intracellular parasite for which felines are the definitive host. After a primary infection, felines excrete T gondii oocytes for several weeks. The oocytes then take several days to sporulate, at which point they are infective. The sporulated oocysts infect various warm-blooded animals. The oocytes remain viable for the animal's entire lifetime. Risk factors for human infection in the United States include owning 3 or more kittens, eating undercooked meat or seafood products, ingesting contaminated soil (such as after gardening), and drinking unpasteurized milk or untreated water, including water used to wash fruits and vegetables.\n\nOnce infected, the incubation period is typically 1 week, but may be up to 3 weeks. Most healthy children will be asymptomatic. Symptomatic healthy children will usually have a self-limited course and commonly exhibit cervical lymphadenopathy or a mononucleosis-like syndrome. Children who have been infected by sources with high oocyte counts, with highly virulent strains, or from international travel may have a more aggressive course with severe pneumonia, myocarditis, or encephalitis. Healthy individuals who have been infected may develop chorioretinitis years after primary infection, even if they remain otherwise healthy.\n\nHealthy children who have been infected with T gondii are usually able to produce antibodies that remain present for life and control the infection. This results in latent infection that usually does not pose any problems. If, however, the child were to become immunocompromised at any age, the infection can reactivate and result in life-threatening symptoms including encephalitis, pneumonia, or myocarditis. Such individuals should receive prophylaxis, if possible, before they become immunocompromised.\n\nCongenital infection is most commonly caused when a mother contracts a primary infection 3 months prior to conception or later. Other far less common causes of congenital toxoplasmosis include reactivation of maternal latent infection if the mother becomes immunosuppressed or if the mother had a latent infection but became reinfected with a more virulent strain. The later in gestation the infection occurs, the higher the likelihood of transmission to the fetus but the less likely the fetus is to be symptomatic at birth. In neonates with congenital infection with T gondii, symptoms may appear later in life and include intellectual disability, learning disability, and visual or hearing impairment. Symptoms that may be apparent at birth include hydrocephalus, cerebral calcifications, and chorioretinitis. Other symptoms include rash, hepatosplenomegaly, cytopenia, and pneumonia.\n\nLaboratory diagnosis of T gondii involves antibody testing of the mother's serum and antibody and PCR testing of the infant's blood, urine, and cerebrospinal fluids. These tests should be performed at a reference laboratory and over several time points to rule out false-negative or false-positive results. Infants with suspected congenital toxoplasmosis should have regular eye, hearing, and neurologic examinations as well as head and abdominal imaging. Care should be coordinated by a team of specialists.\n\nTreatment of toxoplasmosis depends on symptom severity and should include specialist consultation. Healthy children with self-limiting symptoms from acquired toxoplasmosis do not require treatment. Treatment with a combination of pyrimethamine, sulfadiazine, and folinic acid should be given to healthy children who have developed chorioretinitis, children who have severe symptoms, or children who have a reactivated latent infection after becoming immunocompromised. Treatment of infants with symptomatic congenital toxoplasmosis involves the same regimen with prednisone added if there is evidence of chorioretinitis or cerebrospinal fluid infection. Treatment usually continues for 12 months. Asymptomatic infants with congenital infection should also receive similar treatment but usually for 3 months. Treatment of pregnant mothers with primary infection or reactivation of latent infection depends on gestational age.\n\nIn the event of an outbreak, local health officials are not required to be notified but physicians should screen individuals who are at higher risk of developing complications.\n\nIn the United States, not all pregnant mothers are screened for T gondii infection and only 2 states routinely screen newborns for congenital toxoplasmosis. Initial diagnosis is based on suspicion from abnormal fetal ultrasonography results or symptoms in the infant. Experts are still trying to determine if universal screening would be beneficial.\n\nPREP Pearls\n• Most healthy children who are infected with Toxoplasma gondii do not need to be treated but may need to receive prophylaxis if they become immunosuppressed at any point in their lives.\n• Symptoms of toxoplasmosis contracted congenitally or after birth may not develop until many years after primary infection.\n• Screening of pregnant mothers and newborns is not universal but should be strongly considered in individuals who are at higher risk.\n\nMOCA-Peds Objective\n• Evaluate a child with possible vertically transmitted infection.\n\nABP Content Specifications(s)\n• Understand the epidemiology of Toxoplasma gondii\n• Identify the clinical features associated with congenital and acquired Toxoplasma gondii infestation, and manage appropriately\n\nSuggested Readings\n• Abdel-Haq N, Chearskul P, Rafee Y, Asmar BI. Parasitic infections. In: McInerny TK, Adam HM, Campbell DE, DeWitt TG, Foy JM, Kamat DM, eds. American Academy of Pediatrics Textbook of Pediatric Care. 2nd ed. Elk Grove Village, IL: American Academy of Pediatrics; 2017:2470-2490. Pediatric Care Online.\n• American Academy of Pediatrics. Toxoplasma gondii infections. In: Kimberlin DW, Brady MT, Jackson MA, Long SS, eds. Red Book: 2018 Report of the Committee on Infectious Diseases. 31st ed. Itasca, IL: American Academy of Pediatrics; 2018:809-819. https://redbook.solutions.aap.org/chapter.aspx?sectionId=189640201&bookId=2205&resultClick=1.\n• Maldonado YA, Read JS; AAP Committee on Infectious Diseases. Diagnosis, treatment, and prevention of congenital toxoplasmosis in the United States. Pediatrics. 2017;139(2):e20163860. doi:10.1542/peds.2016-3860.\n• McLeod R, Boyer KM. Toxoplasmosis (Toxoplasma gondii). In: Kliegman RM, St Geme JW III, Blum NJ, Shah SS, Tasker RC, Wilson KM, eds. Nelson Textbook of Pediatrics. 21st ed. Philadelphia, PA: Elsevier; 2020:1865-1877."}
{"id" : 769, "question_text" : "A 17-year-old boy in your practice has an 18-mm induration response to a tuberculin skin test that was placed as part of a precollege physical examination. He is clinically well, with no cough, night sweats, fever, or weight loss. He is well-appearing and findings on his physical examination are unremarkable. He lives with his parents and 14-year-old sister, all of whom are reported as healthy. He immigrated to the United States from India at 3 years of age. He received a Bacillus Calmette-Guerin vaccine as an infant. He has not travelled internationally since immigrating into the United States. His grandparents visited from India 6 months ago but are reportedly healthy. Of the following, the BEST next step in management of this patient is to", "options" : "[\"begin isoniazid preventive therapy\", \"obtain a chest radiograph\", \"order an interferon-y release assay\", \"repeat the tuberculin skin test in 6 months\", \"repeat the tuberculin skin test in 2 weeks\"]", "explanation" : "Bacillus Calmette-Guerin (BCG) is an attenuated vaccine derived from a strain of Mycobacterium bovis that is administered at birth in many countries around the world to prevent tuberculosis disease. Most people who receive this vaccine develop some degree of short-term reaction to a tuberculin skin test (TST). However, more than 10 years after the receipt of BCG vaccine, an 18-mm induration as described for the boy in the vignette should not be attributed to BCG vaccine; the finding would be considered a positive TST reaction especially in a person from a region with high rates of tuberculosis.\n\nIn general, a routine TST in low-risk populations is not indicated. A history of epidemiologic risk for exposure to tuberculosis including contact with an active case of tuberculosis, incarceration, institutionalization, or living in a high-risk area of the world are indications for performing a TST.\n\nIn the face of a positive TST result in a well-appearing individual, the best next step in the evaluation would be a chest radiograph. If the chest radiograph is normal or reveals only calcification consistent with a walled-off past infection, a diagnosis of latent tuberculosis infection is made and then preventive therapy with isoniazid would be indicated.\n\nInterferon-γ release assays (IGRAs) detect reaction to Mycobacterium tuberculosis but not BCG. However, the extent of induration to the TST and the nation of origin described in this case are sufficient to define tuberculosis and additional testing is unnecessary. Although IGRAs may be useful for testing in adolescents, data to recommend their use in children younger than 5 years are insufficient. Positive IGRA and TST results do not differentiate tuberculosis infection from tuberculosis disease.\n\nIn view of the magnitude of the reaction to the TST, further repeat testing is not indicated. In addition, conducting a repeat TST 2 weeks after a prior test could boost a borderline test result to an apparent positive result, thus making interpretation difficult.\n\nPREP Pearls\n• Although prior receipt of BCG vaccine may lead to a reaction to a TST, this wanes with time and is expected to be <10 mm by 10 years after the BCG.\n• Interferon-γ release assay results are not affected by previous BCG vaccination.\n\nAmerican Board of Pediatrics Content Specification(s):\n• Understand the effect of the BCG vaccine on the tuberculin skin test\n\nSuggested Reading:\n• American Academy of Pediatrics. Tuberculosis. In: Pickering LK, Baker CI, Kimberlin DW, Long SS, eds. Red Book: 2012 Report of the Committee on Infectious Diseases. 29th ed. Elk Grove Village, IL: American Academy of Pediatrics; 2012:736-759\n• Machingaidze S, Wiysonge CS, Gonzalez-Angelo Y, et al. The utility of an interferon gamma release assay for diagnosis of latent tuberculosis infection and disease in children: a systematic review and meta-analysis. Pediatr Infect Dis J. 2011;30(8):694-700. doi:10.1097/INF.0b013e318214b915\n• Mazurek GH, Jereb J, Vernon A, et al. Updated guidelines for using interferon gamma release assays to detect Mycobacterium tuberculosis infection— United States, 2010. MMWR Recomm Rep. 2010;59 (RR-5):1- 26"}
{"id" : 3597, "question_text" : "A previously healthy 9-month-old female infant is seen in the emergency department with a 2-day history of fever and decreased appetite. She has no history of vomiting, diarrhea, cough, or nasal congestion. Her temperature is 39.5°C. She is alert and interactive. She is mildly dehydrated, and there is no focus of infection. A catheterized urine sample is collected. Urinalysis results are shown: Laboratory Test Result Leukocyte esterase 2+ Nitrite Negative White blood cells 20-50/HPF She fails an oral fluid challenge and is admitted to the hospital for intravenous antibiotics. Her urine culture grows 50,000 CFU/mL of Escherichia coli. Renal ultrasonography shows mild left hydronephrosis. Of the following, the MOST appropriate next step is to", "options" : "[\"perform DMSA (dimercaptosuccinic acid) renal scan\", \"perform voiding cystourethrography\", \"repeat renal ultrasonography after 1 month\", \"repeat urinalysis and culture\"]", "explanation" : "Correct Answer: B\nThe female infant in this vignette has high fever without a clinical focus, pyuria on urinalysis, and a catheterized urine specimen with growth of a single organism confirming the diagnosis of a first febrile urinary tract infection (UTI). An abnormal renal ultrasonography (RUS) finding of mild hydronephrosis warrants further testing with voiding cystourethrography (VCUG) to evaluate for vesicoureteral reflux.\n\nUrinary tract infections are common, accounting for 5% to 14% of pediatric emergency department visits. The prevalence of UTI in a febrile child of less than 2 years of age is approximately 7%, the risk being higher in females as compared to males. Febrile UTI (acute pyelonephritis) is more common in infants, whereas cystitis is a common presentation in school-aged children. Escherichia coli is the most common pathogen responsible for UTI across all age groups. Other common pathogens are Proteus (more so in male patients), Klebsiella, Enterococcus, and Staphylococcus species (in adolescents). In neonates, group B Streptococcus is a common causative organism for UTI, due to colonization of the mother's urinary tract. In sexually active adolescents, Neisseria gonorrhoeae and Chlamydia trachomatis are a common cause of urethritis.\n\nThe diagnosis and management of initial febrile UTI in the 2- to 24-month age group is extensively discussed in the 2011 American Academy of Pediatrics (AAP) clinical practice guideline. The guideline stresses the importance of urine specimen collection for urinalysis and urine culture in a febrile infant with no other apparent source of fever, or if the infant appears ill, and the clinician is considering starting antibiotics. For children who are not toilet trained, urine collection through catheterization or by suprapubic aspiration is recommended. Because a clean-catch specimen cannot be collected from a child who is not toilet trained, a diagnosis of UTI should not be made based on urine culture results from a bagged-urine specimen. According to the guideline, any bacterial growth in a suprapubic aspirate or 50,000 or more CFU of a single organism in a catheterized urine sample is confirmatory of UTI. In a voided urine sample (bag or clean-catch), more than 100,000 CFU is considered significant; however, growth of more than one organism on the urine culture suggests contamination.\n\nChildren younger than 24 months with UTI are often diagnosed with anatomic abnormalities like vesicoureteral reflux and obstructive uropathy. The AAP guideline recommends RUS for all children in the 2- to 24-month age group with a first febrile UTI. Renal ultrasonography can be performed after the UTI has been treated. In cases where clinical improvement is not shown in 48 hours, RUS can be performed early to evaluate for pyonephrosis or development of a renal abscess. If the results of RUS are normal, no further testing is required. However, if the results suggest hydronephrosis, scarring, or other features of urinary obstruction, VCUG is indicated to diagnose vesicoureteral reflux. The VCUG can be performed once the infection has cleared.\n\nFor the infant in the vignette, a repeat urine culture is not indicated because the first urine specimen was a catheterized collection with no suggestion of contamination. If the child is clinically improving, repeating urine culture to demonstrate cure is also not recommended. In this vignette, the infant's RUS revealed mild hydronephrosis. Repeating the study in 1 month to confirm resolution of hydronephrosis is not required. A nuclear scan using technetium-labeled DMSA (dimercaptosuccinic acid) can be used to differentiate acute pyelonephritis from lower UTI. The findings of a DMSA renal scan rarely change the acute management, and therefore it is not recommended as a routine imaging study in a child with a first febrile UTI. However, a DMSA renal scan may be helpful in children with recurrent UTI to evaluate for scarring.\n\nA systematic approach to management of young children with febrile UTI is shown in Item C41. Infants who appear ill and are unable to take oral fluids or medications will need parenteral antibiotics until they show signs of clinical improvement. Ceftriaxone is commonly used as an empiric parenteral antibiotic prior to sensitivity results being available. The empiric oral antibiotic treatment of UTI should include an oral third-generation cephalosporin (cefixime). Trimethoprim-sulfamethoxazole or amoxicillin-clavulanic acid can be used when sensitivities are known. The choice of oral antibiotics depends on local patterns of susceptibility, as wide geographic variability exists. As per the AAP guidelines, an infant with a febrile UTI should receive antibiotic therapy for a total (parenteral plus oral or oral route) of 7 to 14 days to eliminate infection and prevent renal damage.\n\nPREP Pearls\n• Pyuria and growth of a significant single organism from culture of a catheterized urine specimen in an infant with fever confirms a diagnosis of urinary tract infection.\n• Renal ultrasonography is recommended for all infants with a first febrile urinary tract infection.\n• Voiding cystourethrography is indicated if renal ultrasonography findings are abnormal or in children with recurrent urinary tract infections.\n\nABP Content Specifications(s)\n• Plan the appropriate diagnostic evaluation of a urinary tract infection in children who are and are not toilet-trained\n• Plan the appropriate initial management of acute pyelonephritis while awaiting results of diagnostic testing\n• Recognize pathogens commonly associated with urinary tract infection in children of various ages\n\nSuggested Readings\n• Balighian E, Burke M. Urinary tract infections in children. Pediatr Rev. 2018;39(1):3-12. doi:10.1542/pir.2017-0007.\n• Nanda G, Jahnukainen T, Vats A. Urinary tract infections. In: McInerny TK, Adam HM, Campbell DE, DeWitt TG, Foy JM, Kamat DM, eds. American Academy of Pediatrics Textbook of Pediatric Care. 2nd ed. Elk Grove Village, IL: American Academy of Pediatrics; 2017:2748-2757. Pediatric Care Online.\n• Subcommittee on Urinary Tract Infection, Steering Committee on Quality Improvement and Management. Urinary tract infection: clinical practice guideline for the diagnosis and management of the initial UTI in febrile infants and children 2 to 24 months. Pediatrics. 2011;128(3):595-610. doi:10.1542/peds.2011-1330."}
{"id" : 2174, "question_text" : "A 7-day-old neonate is seen in the office for a follow-up visit. He was born at term via an uncomplicated spontaneous vaginal delivery. He has been taking about 2 ounces of standard infant formula every 2 to 3 hours and producing about 5 wet diapers and 3 normal-appearing stools per day. He has a 2-year-old sibling who has been healthy. The neonate is afebrile. His weight is 3 kg, heart rate is 140 beats/min, and respiratory rate is 30 breaths/min. On physical examination, he is awake, alert, and interactive. He has scleral icterus and jaundice. On chest auscultation, his lungs are clear, and there is a 3/6 systolic ejection murmur. His abdomen is soft and nontender with no palpable hepatosplenomegaly. He has full range of motion of his extremities and no focal neurologic deficits. Results of laboratory studies are shown: White blood cell count 18,000/µL (18.0 × 10⁹/L), Neutrophils 35%, Lymphocytes 50%, Monocytes 14%, Eosinophils 1%, Platelet count 500 × 10³/µL (500 × 10⁹/L), Hemoglobin 7.9 g/dL (79 g/L), Mean corpuscular volume 103 fL, Reticulocyte 6%, Total bilirubin 20 mg/dL (342.1 µmol/L), Direct bilirubin 0.5 mg/dL (8.6 µmol/L), ABO interpretation A, Rh interpretation Positive, Antibody screen Positive, Direct Coombs IgG Positive. Of the following, the BEST next step is", "options" : "[\"close observation at home with next day follow-up\", \"referral to a cardiologist\", \"referral to an emergency department\", \"referral to a gastroenterologist\"]", "explanation" : "ABO or Rh incompatibility is a common cause of hemolytic disease of the newborn. The sequelae of hemolytic disease of the newborn can be life threatening. In first pregnancies with ABO or Rh incompatibility, there may be maternal sensitization to fetal blood groups without significant fetal hemolysis; subsequent pregnancies are typically more severely affected. The neonate described in the vignette has anemia with jaundice, indirect hyperbilirubinemia, and reticulocytosis, which should raise concern for hemolytic anemia. The severity of his anemia and hyperbilirubinemia warrant immediate evaluation in an emergency department setting. An exchange transfusion may be required based on indications defined using a neonatal bilirubin nomogram. Transfusion of red blood cells may be necessary based on the severity of his anemia. One of the most common causes of neonatal anemia is hemolytic disease of the newborn (HDN) caused by an ABO or Rh incompatibility. ABO incompatibility occurs when there is a mismatch between the maternal and fetal blood groups (eg, mother is blood type O and fetus is type A or B) leading to maternal formation of gamma immunoglobulin (IgG) antibodies. This type of mismatch usually leads to indirect hyperbilirubinemia and a mild to moderate level of anemia that is typically not as severe as that seen with Rh incompatibility. Close monitoring of this condition is required. Due to routine prenatal Rh testing and administration of anti-D immunoglobulin when indicated, cases of HDN arising from incompatibility of the RhD antigen have significantly decreased. However, many other antigens on the red blood cell surface (eg, Kell, Duffy, E, e, C, and c antigens) may lead to other forms of Rh HDN. Maternal antibodies to these minor antigens may lead to more severe hemolysis with significant anemia and unconjugated hyperbilirubinemia. Hemolytic disease of the newborn is a transient condition. The antibodies involved are produced by the mother; therefore, after delivery, the neonate's antibody titer will decrease over time. A direct antiglobulin test (DAT), previously known as the direct Coombs test, performed on the newborn's blood may reveal the presence of maternal antibodies. Neonates affected by HDN should be closely monitored for both unconjugated hyperbilirubinemia and anemia; when indicated, intervention should be prompt. Exchange transfusion may be required for neonates with severe unconjugated hyperbilirubinemia, while transfusion of packed red blood cells may be necessary for severe anemia. The use of intravenous gamma globulin in the treatment of HDN is controversial. Caregivers should be educated about concerning signs and symptoms (eg, difficulty feeding, decreased activity, increased pallor, worsening jaundice) and instructed to seek care promptly if any of these are observed. There is increased risk throughout the timeframe when physiologic anemia of infancy occurs (typically 6-9 weeks), as the decline in hemoglobin may be severe in these neonates. Investigation for additional causes of hemolysis (eg, inherited enzymopathies) may sometimes be warranted in neonates with hemolytic anemia. Maternal screening for HDN during pregnancy includes measurement of antibody titers. If detected, additional monitoring is required. While maternal antibodies associated with HDN may be formed during a first pregnancy, titers are generally low; therefore, first-born children may not be clinically affected. Antibody titers are generally higher and formation occurs earlier in subsequent pregnancies, placing these fetuses at greater risk. In the case of a pregnant individual with extremely high levels of antibodies to fetal blood groups, there is significant risk for fetal anemia; intrauterine blood transfusions may be required to avoid hydrops fetalis."}
{"id" : 126, "question_text" : "A 1-day-old male infant fails to pass urine in the nursery. He was born at 37 weeks' gestation to a 30-year-old woman who received prenatal care. On physical examination, the infant exhibits mild tachypnea, wrinkling of the skin on the anterior abdominal wall (Item Q14), and undescended testicles. Of the following, the MOST useful test to diagnose the cause of anuria in this infant is", "options" : "[\"mercaptoacetyltriglycine (MAG-3) furosemide renal scan\", \"renal/bladder ultrasonography\", \"serum creatinine measurement\", \"urinalysis obtained by urethral catheterization\", \"voiding cystourethrography\"]", "explanation" : "The infant described in the vignette has prune-belly syndrome, which is characterized by the triad of bilateral hydroureteral nephrosis, undescended testicles, and diminished/absent anterior abdominal wall musculature, resulting in a wrinkled anterior abdominal wall. In addition, he is exhibiting anuria.\n\nAssessment of anuric or oliguric renal failure begins with determination of whether it is prerenal, intrinsic renal, or postrenal renal failure. The differential diagnosis of anuria in the neonatal period includes prerenal causes (sepsis, congestive heart failure), intrinsic renal disease of the parenchyma (acute tubular necrosis, cortical necrosis, or autosomal recessive polycystic kidney disease) or vasculature (renal vein thrombosis), or postrenal failure (posterior urethral valves, prune-belly syndrome, neurogenic bladder, obstruction of a single kidney). The clinician should be familiar with this differential diagnosis to optimize evaluation and treatment of infants who have decreased urine output.\n\nPrune-belly syndrome is a form of obstructive uropathy with associated renal parenchymal damage from the high back pressure of the obstructed urinary system. Accordingly, the clinician must assess the status of the urinary tract, including the kidneys and bladder, which is accomplished best with renal/bladder ultrasonography. This evaluation allows the clinician to determine the most probable cause of renal failure as well as the status of the urinary tract. For example, the infant in the vignette would be expected to have hydronephrosis with associated hydroureter. A distended urinary bladder (containing urine) would further support the likelihood of bladder outlet obstruction.\n\nAnother test used to evaluate the urinary tract is voiding cystourethrography, which involves instillation of radiocontrast into the urinary bladder via a bladder catheter. While under fluoroscopic surveillance, the infant voids, which allows the clinician to assess bladder volume, bladder anatomy (including the absence of a ureterocele), and the presence or absence of vesicoureteral reflux (VUR). Although this is a useful test in the evaluation of an infant who has hydronephrosis to rule out VUR and in the male infant who has bilateral hydronephrosis to rule out posterior urethral valves, this would not be the appropriate initial test for the infant in the vignette.\n\nMeasurement of serum creatinine can assess renal function, but it will not determine the cause of anuria. In the immediate newborn period (first 24 to 48 hours after birth), the creatinine concentration is influenced by maternal creatinine values. However, creatinine values that exceed maternal norms warrant further assessment of neonatal renal function. Urinalysis is of limited diagnostic value for this infant, and catheterization at this age can be difficult, possibly requiring the expertise of a pediatric urologist for infants who have obstructive uropathy. This point should be conveyed to nursing staff in the neonatal setting to avoid potential complications of catheterization. A mercaptoacetyltriglycine (MAG-3) furosemide renal scan is a dynamic nuclear medicine test that is best used in the evaluation of suspected ureteropelvic junction obstruction.\n\nCritique: [As above]\n\nContent Specifications: Plan the evaluation of an anuric infant."}
{"id" : 2467, "question_text" : "A 10-year-old boy is brought to the office after coughing up blood this morning. He reports no vomiting or epistaxis. He has had cough, fatigue, malaise, and tactile fever for several weeks. His family emigrated from Somalia 1 year ago. No one else in the household has been ill recently, but his grandfather, who lives nearby, has had a chronic cough for many months.\n\nThe boy's temperature is 37.3 °C orally, his blood pressure is 105/65 mm Hg, his heart rate is 100 beats/min, his respiratory rate is 30 breaths/min, and his oxygen saturation is 97% in room air. His height and weight are at the 25th percentile. The boy appears tired but not acutely ill. He is breathing comfortably. There is no cervical tenderness or adenopathy. His nose and pharynx are clear. On lung examination, there is adequate air exchange with coarse breath sounds throughout, coarse crackles bilaterally in the mid-lung fields, and no expiratory wheezing. The remainder of the boy's physical examination findings are normal. Chest radiography is obtained, and results are pending.\n\nOf the following, the BEST next step in this boy's care is", "options" : "[\"obtain magnetic resonance imaging of his neck and chest\", \"obtain sputum evaluation with acid-fast stain and cultures\", \"refer him to an otolaryngologist\", \"wait for the chest radiography results\"]", "explanation" : "The boy in the vignette has hemoptysis with symptoms suggestive of systemic illness (fatigue, malaise, tactile fever) and abnormal pulmonary findings (coarse breath sounds, crackles). The best next step in his care is to obtain sputum evaluation with acid-fast stain and cultures.\n\nThe most common cause of hemoptysis in children without severe underlying cardiac or pulmonary disease is infection. Based on the history and his physical examination findings, tuberculosis is the most likely cause of this boy's hemoptysis. He immigrated from a country where tuberculosis is endemic and has contact with a family member with chronic cough. Other unusual infections must also be considered, as well as the more common bacterial causes of pneumonia. Although the chest radiography results should be available soon, sputum should be obtained to evaluate for a specific infectious etiology without delay. In addition to Gram stain and bacterial culture, a search for acid-fast (Mycobacterium) and fungal organisms is appropriate.\n\nNeither magnetic resonance imaging of the neck and chest nor computed tomography with contrast would be part of the initial diagnostic evaluation. These imaging studies might be indicated if there were significant concern regarding a vascular lesion or mass as a source of bleeding. This boy's history and physical examination findings are most suggestive of infection; therefore, these studies are not the best next management step.\n\nThe boy's condition is stable, and he has no history or physical examination findings suggestive of an upper airway source of bleeding. Therefore, an otolaryngology consultation is not the best next management step.\n\nAlthough hemoptysis may occur as an isolated or transient event, the physician should undertake a careful assessment of the child's hemodynamic status and stability, as well as evaluation to determine the source and clinical implications of the bleeding. For a stable child with no signs of impending cardiovascular compromise and with a clearly benign upper airway source of bleeding, reassurance, discussion of preventive strategies, and follow-up planning is appropriate. Otherwise, further evaluation is indicated to determine the etiology and direct the management of the bleeding.\n\nMost airway bleeding in children is due to mucosal irritation. The upper airway (nose, sinuses, pharynx) or lower airway (trachea, bronchi or bronchioles) mucosa may be involved. Bleeding can result from any of the following:\n- Superficial trauma to the nasal and upper airway\n- Infection of a tracheostomy site or lower airway\n- Foreign body lodged in the upper airway or aspirated into the lower airway\n\nVomiting due to esophageal or stomach bleeding may be mistaken for hemoptysis (expectoration). Infants and young children may swallow and later vomit upper-airway blood. Table 1 outlines the features differentiating hemoptysis from a gastric or upper airway blood source.\n\nCertain underlying conditions (eg, presence of a tracheostomy tube, bronchiectasis, congenital heart disease with pulmonary hypertension or collateral vessels) predispose children to airway inflammation and bleeding. The hemoptysis may be an isolated occurrence or recurrent. The associated mortality risk depends on the underlying condition. Hemoptysis associated with tracheostomy infection and local irritation is commonly recurrent but rarely fatal (an exception is erosion into a major vessel). In contrast, hemoptysis associated with severe underlying pulmonary or cardiac disease has a higher mortality risk but lower recurrence rate.\n\nThe evaluation of a child with hemoptysis associated with an underlying condition is outlined in Table 2.\n\nSystemic conditions associated with hemoptysis include bronchiectasis (cystic fibrosis-related and non–cystic fibrosis-related), collagen vascular diseases (with sinus and pulmonary foci), arteriovenous anomalies of the chest or airway, and congenital cardiopulmonary disorders. These conditions may not have been diagnosed before the episode of hemoptysis, particularly in the case of autoimmune processes with capillaritis as the source of the bleeding. Evaluation of hemoptysis in a child without a known preexisting condition is outlined in Table 3.\n\nThe initial evaluation and management of hemoptysis should focus on assessment of the child's hemodynamic status and potential for deterioration. If the child's condition is stable and not at risk of experiencing sudden decompensation, and there are no known underlying conditions, then evaluation should proceed as in Table 3. Further management depends on the specific cause of bleeding.\n\nTreatment of superficial airway irritation is directed at the identified bleeding site. Children with tracheostomies require meticulous home care routines to protect the integrity of the site and prevent superficial infections. Routine evaluation by an otorhinolaryngologist is indicated for management of the tracheostomy site and any granulomatous tissue within the airway, a potential source of bleeding. Deeper airway infections, with or without an associated underlying condition, require a more prolonged and aggressive antibiotic course targeted to the specific infection and underlying process. Bronchoscopy is indicated to determine if bleeding is from a single site or is diffuse. Management of hemoptysis associated with cardiovascular disease will require involvement of the pediatric cardiologist to provide direction regarding the state of specific cardiovascular diagnosis and potentially to address pulmonary arterial hypertension or presence of collateral circulation.\n\nSuggested Reading(s)\nChiel L, Walsh S, Andren K, et al. Pediatric hemoptysis without bronchiectasis or cardiac disease: etiology, recurrence, and mortality. J Ped. 2019;214:66-70. doi:10.1016/j.jpeds.2019.07.049\nSchroeder SA. Hemoptysis. In: McInerny TK, Adam HM, Campbell DE, DeWitt TG, Foy JM, Kamat DM, eds. American Academy of Pediatrics Textbook of Pediatric Care. American Academy of Pediatrics; 2023. Accessed September 1, 2023. Pediatric Care Online\nShnayder R, Needleman JP. Hemoptysis. Pediatr Rev. 2018;39(6):319-321. doi:10.1542/pir.2017-0157\n\nContent Domain\nPulmonology\n\nABP Content Specification(s) / Content Area(s)\nPlan the appropriate clinical and diagnostic evaluation of hemoptysis\nPlan the appropriate management of hemoptysis in patients of various ages\n\nThe correct answer is: obtain sputum evaluation with acid-fast stain and cultures"}
{"id" : 2576, "question_text" : "A 13-year-old adolescent boy with newly diagnosed type 1 diabetes is seen for follow-up. He is doing well with his insulin regimen of multiple daily subcutaneous injections. He takes 12 units of long-acting basal insulin analog once daily at bedtime and boluses of a rapid-acting insulin analog before meals, dosed based on carbohydrate counts (1 unit for every 15 g of carbohydrate) and blood glucose measurements (1 unit for every 50 mg/dL over 150 mg/dL). The boy's physical examination findings are unremarkable. His blood glucose log is shown (glucose levels are in mg/dL): Breakfast Lunch Dinner Bedtime Day 1 253 118 142 117 Day 2 322 165 133 154 Day 3 196 122 102 125 Day 4 217 131 95 111 Day 5 284 110 118 132 Of the following, the BEST next step in this boy's management is to", "options" : "[\"ask the boy to check his blood glucose at 3 AM\", \"ask the boy to check for urine ketones each morning\", \"increase the basal insulin dose to 13 units\", \"split the basal insulin dose into 6 units twice daily\"]", "explanation" : "Correct Answer: A\nThe boy in the vignette has newly diagnosed type 1 diabetes, and his blood glucose log demonstrates that he is experiencing elevated blood glucoses in the morning. This pattern is most commonly due to too little (dawn effect) or too much (Somogyi effect) bedtime basal insulin. A blood glucose level checked in the middle of the night (usually between 2-3 AM) can help distinguish between these two effects. An elevated overnight glucose level indicates too little basal insulin, and a low level indicates too much basal insulin.\n\nThe dawn effect is due to the body's circadian rhythm. As a child or adolescent is preparing to rise in the morning, regulatory hormones such as cortisol and growth hormone start to rise. In children and adolescents without diabetes, insulin works in concert with these regulatory hormones resulting in a rise in glucose level without frank hyperglycemia. However, in a child or adolescent with diabetes who has too little circulating basal insulin, the glucose level rises into the hyperglycemic range.\n\nThe Somogyi effect is due to rebound hyperglycemia after an episode of hypoglycemia. When too much circulating basal insulin results in low blood glucose levels overnight, counter-regulatory hormones such as epinephrine, cortisol, and growth hormone are secreted. This response can result in elevated blood glucose levels in the morning. The solution is to decrease the basal insulin dose to prevent episodes of hypoglycemia. This phenomenon is rare; most children and adolescents with overnight hypoglycemia due to excess basal insulin remain hypoglycemic.\n\nMost long-acting basal insulin analogs work for at least 24 hours. In rare situations, the basal insulin analog wears off sooner, and splitting the basal insulin dose can help stabilize the glucose levels. The glucose log for the boy in the vignette is not consistent with the basal insulin analog wearing off too soon. The boy takes his long-acting basal insulin analog at bedtime, so high glucose levels in the evenings, not mornings, could be a sign that it is wearing off too soon.\n\nAlthough the boy in the vignette may need an increased basal insulin dose, he should first check a middle-of-thenight blood glucose level to ensure he is not experiencing hypoglycemia and the Somogyi effect.\n\nWhen children and adolescents with type 1 diabetes have hyperglycemia, they are usually advised to check for urine or blood ketones as hyperglycemia can signify impending diabetic ketoacidosis. However, the boy in the vignette is asymptomatic and is not experiencing sustained hyperglycemia. Although the boy should check for ketones, this approach will not help determine why his morning blood glucose levels are high.\n\nPREP Pearls\n• In type 1 diabetes, elevated morning blood glucose levels may be due to either too little (dawn effect) or too much basal insulin (Somogyi effect).\n• Checking a blood glucose level in the middle of the night (2-3 AM) can help distinguish between the dawn and Somogyi effects.\n• Understanding the pharmacodynamics of insulin analogs is essential for making effective insulin dose adjustments.\n\nMOCA-Peds Objective\n• Evaluate and manage a child with hypoglycemia.\n\nABP Content Specifications(s)\n• Plan the appropriate management of type 1 diabetes to effectively achieve good control and to avoid long-term complications\n• Counsel patients regarding self-management of type 1 diabetes\n\nSuggested Readings\n• Jackson S, Creo A, Al Nofal A. Management of type 1 diabetes in children in the outpatient setting. Pediatr Rev. 2022;43(3):160-170. doi:10.1542/pir.2020-001388.\n• Javed A, Schwenk WF, II, Tebben P. Diabetes mellitus. In: McInerny TK, Adam HM, Campbell DE, DeWitt TG, Foy JM, Kamat DM, eds. American Academy of Pediatrics Textbook of Pediatric Care. American Academy of Pediatrics; 2021:chap 241. Accessed September 1, 2022. Pediatric Care Online.\n• Kliegman RM, St Geme JW, Blum NJ, Shah SS, Tasker RC, Wilson KM. Diabetes mellitus. In: Nelson Textbook of Pediatrics. 21st ed. Elsevier; 2020:3019-3052."}
{"id" : 2472, "question_text" : "A 6-month-old girl is brought to the emergency department by her maternal aunt after an unwitnessed fall from the bed to a carpeted floor. Her maternal aunt does not speak English but is able to sign the girl in at the front desk with the help of a cousin who speaks English. The family is brought to the triage area and the nurse measures the patient's vital signs. With the infant's cousin acting as translator, the attending physician obtains the child's medical history from the aunt, including information about the fall. The girl is known to be developmentally delayed and cannot yet roll. On physical examination, she has a bruise on her right cheek, which her aunt reports was from the girl laying on a stuffed animal last week. No other abnormal findings were noted on her examination. The physician documents that there is a low suspicion for nonaccidental trauma, and the girl is discharged home with her aunt.\n\nThe girl is brought to the emergency department 1 week later after having a seizure. On physical examination, she is noted to have bilateral retinal hemorrhages. Imaging reveals acute and chronic subdural hemorrhages and healing posterior rib fractures. On review of this case, the emergency department leadership determines that a systems approach to preventing a similar incident is required.\n\nOf the following, the approach MOST likely to be successful is to", "options" : "[\"implement a child abuse pathway alert in the electronic medical record\", \"implement a patient safety training session for the emergency department staff\", \"organize an interdisciplinary team to review the case\", \"present a grand rounds lecture on child abuse\"]", "explanation" : "The approach most likely to be successful in this situation is to organize an interdisciplinary team to review the case, focusing on the events that led to the unintended outcome. This approach can be accomplished by following the principles of root cause analysis (RCA).\n\nAn RCA is used to identify factors that may lead to medical errors; the goal of this analysis is prevention. This approach enables the health care organization to focus on the systems in place that may contribute to an error that reaches a patient rather than the specific event or actions of an individual. The American Academy of Pediatrics' 2019 policy statement Principles of Pediatric Patient Safety: Reducing Harm Due to Medical Care guides pediatricians to focus on \"the significance of pediatric patient safety, the science behind the culture of safety, and strategies to ensure patient safety.\" The Joint Commission requires accredited health care institutions to have a standardized process in place for situations similar to the one in the vignette.\n\nThe first step of RCA is to form a team to identify all the events that played a role in the unintended outcome. The Joint Commission provides a template to assist with this analysis. Some example questions from this template include the following:\n- What steps of an existing process have been defined by policies or procedures?\n- What steps were missed?\n- Were there human factors that contributed to the outcome?\n- In what other departments could a similar event happen?\n- What training is in place to ensure competency among staff members?\n- Was shared patient information accurate?\n- Was communication among team members adequate?\n- How does leadership support patient safety?\n- How can education and training be improved?\n- How can technology reduce risk?\n\nThe RCA process helps identify systems problems. The team then works together to identify systems solutions that will help prevent future events. Strategies and a timeline for implementation of new or modified processes and procedures are then defined. This analysis and plan are communicated to organizational leadership, who will need to provide support for the plan (eg, dissemination, education, and resources).\n\nQuality improvement methods are an important process for enhancing the delivery of health care to patients. Quality improvement initiatives approach problems at the patient level and seek to improve care, often by implementing evidence-based guidelines. This scientific method focuses on answering the question of why change is needed (global aim) and how to create change by using the SMART acronym (Specific, Measurable, Attainable, Relevant, Time-bound). A key driver diagram can assist the quality improvement team in determining the actions needed to make a change.\n\nGenerally, several rapid Plan-Do-Study-Act (PDSA) cycles are used to test proposed changes. The steps include the following:\n- Plan - defines the details of the project and determines how data will be collected\n- Do - change is implemented and observed (data collection)\n- Study - data are analyzed\n- Act - a determination is made regarding the next steps for the change\n\nThe PDSA cycle is repeated until there is enough information gathered to determine whether the change is improving the system. A run chart is helpful for visualizing the impact of the change. Once the PDSA cycles demonstrate improvement, changes can be implemented more broadly; this may include the implementation of new policies and procedures. Training for all those involved is necessary to sustain the change.\n\nAn RCA might identify several events in this infant's care that would be considered systems problems or medical errors that may have contributed to the undesired outcome. The girl was brought to the emergency department by her aunt (not her mother or father); guardianship was not confirmed. Her aunt did not speak English, and the staff relied on a family member to communicate rather than a translating service. The attending physician also failed to verify guardianship and relied on the family member to translate. The girl's history and physical examination findings should have raised suspicion for child abuse. For any infant being evaluated after an unwitnessed injury, there are recommended laboratory and imaging studies to screen for child abuse. In addition, the girl's examination revealed a bruise on her cheek, with an explanation that is not plausible (lying on a stuffed animal), and the infant's developmental level is not consistent with the story that she fell off the bed (she is unable to roll).\n\nImplementing a child abuse pathway alert in the electronic medical record, organizing a patient safety training session for the nursing staff, and planning a grand rounds lecture on child abuse for the emergency department attending physicians are all ways to potentially improve the system and decrease missed child abuse cases. However, these changes should occur after careful analysis of the events by a multidisciplinary team.\n\nSuggested Reading(s)\nJoint Commission. The framework for root cause analysis and corrective action. Accessed November 22, 2022. https://www.jointcommission.org/-/media/tjc/documents/resources/patient-safety-topics/sentinel-event/rca_framework_101017.pdf\nParikh K, Hochberg E, Cheng JJ, et al. Apparent cause analysis: a safety tool. Pediatrics. 2020;145(5):e20191819. doi:10.1542/peds.2019-1819\nSimpson B, Statile AM, Schondelmeyer AC. How to perform quality improvement projects. Pediatr Rev. 2022;43(10):549-560. doi:10.1542/pir.2021-005314\n\nContent Domain\nQuality improvement/patient safety\n\nABP Content Specification(s) / Content Area(s)\nUnderstand that quality improvement is based on applying a scientific method to improving human systems\nUnderstand and apply root cause analysis to determine the factors contributing to an error\n\nThe correct answer is: organize an interdisciplinary team to review the case"}
{"id" : 1199, "question_text" : "A 16-year-old adolescent presents to your office because she is concerned that, unlike her friends, she hasn't started menstrating. Her mother reports that she and the patient's sister had menarche around 13 years of age. The patient's past medical history is remarkable for surgery for strabismus. She denies sexual activity. On physical examination, her height is less than the fifth percentile. Her weight is at the 80th percentile. Her blood pressure and heart rate are normal. She has a soft systolic murmur. The rest of her examination is unremarkable. She has a sexual maturity rating of 2 for pubic hair and breast. Her urine pregnancy test is negative. Of the following, the MOST appropriate next step in her evaluation is", "options" : "[\"careful assessment for psychological stressors\", \"a karyotype analysis\", \"to order a prolactin level\", \"to provide reassurance\", \"to screen for nonclassical adrenal hyperplasia\"]", "explanation" : "Primary amenorrhea is defined as the absence of menses by 15 years of age or within 5 years of breast development if that occurs before 10 years of age. Primary amenorrhea is most often caused by an anatomic or genetic abnormality. Gonadal dysgenesis resulting from chromosomal abnormalities accounts for approximately 50% of cases of primary amenorrhea. Other common causes include hypothalamic hypogonadism and congenital anatomical abnormalities of the uterus, cervix, or vagina. Careful history and physical examination are important in the evaluation of the etiology for primary amenorrhea. Pubertal assessment, evaluation of reproductive anatomy, and the presence of dysmorphic features can guide the workup of primary amenorrhea.\nTurner syndrome (45 X gonadal dysgenesis) often presents during adolescence as primary amenorrhea. Other features of Turner syndrome include short stature, aortic coarctation, widely spaced nipples, webbed neck, cubitus valgus, strabismus, and congenital lymphedema. The Turner Syndrome Consensus Study Group has published guidelines for the management of girls and women with Turner syndrome. Management strategies address growth-promoting therapies and the induction of puberty.\nThe physical findings in the adolescent in this vignette suggest a diagnosis of Turner syndrome and a karyotype analysis is the most appropriate next step in her evaluation.\nWhile psychological stress can be a reason for amenorrhea, in this scenario, there are other findings that should alert the clinician to the possibility of other reasons for the patient's amenorrhea. Prolactinomas in young female adolescents can present as delayed menarche. However, the other findings in this scenario make a prolactinoma less likely as the etiology of this patient's amenorrhea. Similarly, nonclassical adrenal hyperplasia (NCAH) can present with menstrual irregularities like amenorrhea, but individuals with NCAH usually also have evidence of hyperandrogenism with hirsutism and acne, as well as premature pubarche and tall stature during adolescence.\nPREP Pearls\n• Primary amenorrhea is defined as the absence of menses by 15 years of age or within 5 years of breast development if that occurs before 10 years of age.\n• Primary amenorrhea is most often caused by an anatomic or genetic abnormality.\n• Gonadal dysgenesis resulting from chromosomal abnormalities accounts for approximately 50% of cases of primary amenorrhea.\n• Karyotype analysis should be done in the case of primary amenorrhea.\nABP Content Specifications(s)\n• Recognize the clinical findings associated with primary amenorrhea of various etiologies, and manage appropriately"}
{"id" : 2208, "question_text" : "A neonate is admitted to the newborn nursery. He was born at 37 5/7 weeks' gestation to a gravida 3 para 2 woman with type 1 diabetes mellitus, which is well controlled on 40 U of insulin per day. She was treated for a group B Streptococcus urinary tract infection at 10 weeks' gestation. The mother had routine prenatal care, including visits at 36 and 37 weeks' gestation; vaginal and rectal swabs for group B Streptococcus screening were not obtained at those visits. The membranes were ruptured 12 hours before delivery. There was no concern regarding maternal chorioamnionitis at the time of delivery. Shortly after arrival at the labor and delivery unit, the newborn's mother received 2 g of intravenous ampicillin, and another 1 g during labor. She had a spontaneous vaginal delivery about 6 hours after the first dose of ampicillin. Of the following, the MOST likely reason for administration of this medication during labor was", "options" : "[\"maternal history of type 1 diabetes\", \"maternal history of urinary tract infection\", \"no group B Streptococcus screening at 36 to 37 weeks' gestation\", \"rupture of membranes of 12 hours' duration\"]", "explanation" : "Group B Streptococcus is the most common cause of early-onset sepsis in newborns; the primary risk factor for infection is group B Streptococcus colonization of the mother.\n\nIndications for intrapartum antibiotic prophylaxis to prevent early-onset group B Streptococcus (GBS) disease in the newborn include the mother's having a previous neonate with invasive GBS disease, positive vaginal and rectal culture obtained in the late third trimester, or GBS bacteriuria at any time during the current pregnancy.\n\nAdequate intrapartum antibiotic prophylaxis to prevent early-onset group B Streptococcus disease in the newborn is 1 dose of intravenous penicillin or ampicillin at least 4 hours before delivery.\n\nIntrapartum antibiotic prophylaxis does not prevent late-onset group B Streptococcus disease.\n\nCritique\nThe most likely reason this mother received ampicillin during labor was her history of group B Streptococcus (GBS) urinary tract infection (UTI). Group B Streptococcus is the most common cause of early-onset sepsis in newborns. The primary risk factor for infection in the newborn is GBS colonization of the mother's genital tract. Transmission to neonates from mothers with GBS colonization occurs shortly before or during delivery. The prevalence of GBS colonization among pregnant individuals ranges from 15% to 35%; it can be intermittent or persistent. Approximately 50% of colonized pregnant individuals transmit the bacteria to their newborns. Without intrapartum antibiotic prophylaxis (IAP), 1% to 2% of these newborns will develop early-onset GBS disease.\n\nThe standardized practice of identification of GBS colonization during pregnancy and administration of IAP has led to an 80% reduction in early-onset GBS disease. Vaginal and rectal cultures performed within 5 weeks of delivery have the highest positive predictive value for GBS colonization at birth. The American College of Obstetricians and Gynecologists recommends universal antepartum culture-based screening for GBS at 36 0/7 to 37 6/7 weeks, regardless of planned mode of delivery. In addition, this organization recommends IAP to prevent early-onset GBS disease in the newborn for pregnant women with\nA previous neonate with invasive GBS disease\nA positive vaginal and rectal culture obtained at >36 0/7 weeks' gestation (unless a cesarean delivery is performed before rupture of membranes)\nGroup B Streptococcus bacteriuria at any time during the current pregnancy\nUnknown GBS status at the onset of labor AND any of the following:\nGestational age <37 0/7 weeks\nRupture of membranes ≥18 h\nIntrapartum temperature ≥38 °C\nKnown GBS-positive status in a previous pregnancy\n\nThe pregnant woman in the vignette appropriately received IAP with intravenous ampicillin because she had a GBS-positive UTI at 10 weeks' gestation. Because IAP for GBS was already indicated in this patient owing to her UTI, there was no need to perform vaginal and rectal GBS screening at 36 to 37 weeks' gestation.\n\nAdequate IAP prophylaxis to prevent early-onset GBS disease in the newborn involves administering 1 dose of intravenous penicillin G 5 million U, followed by 2.5 to 3.0 million U every 4 hours until delivery or ampicillin 2 g followed by 1 g every 4 hours until delivery. For maximal protection (efficacy of 80%-90%), treatment must begin at least 4 hours before delivery; a shorter interval will still provide some protection. If the pregnant individual has a penicillin allergy with a low risk of experiencing anaphylaxis, a first-generation cephalosporin should be administered; for severe allergy with a high risk of anaphylaxis, clindamycin or vancomycin should be used.\n\nAlthough late–third-trimester GBS screening has been very effective in preventing early-onset GBS disease in the newborn, approximately two-thirds of cases of early-onset GBS disease occur in neonates born to individuals with a negative GBS screening result at 35 to 37 weeks. This is due to episodes of temporary GBS colonization of the genital tract. The use of IAP has no preventive effect on late-onset GBS disease. Maternal diabetes is not an indication for IAP. Unknown GBS status with rupture of membranes for ≥18 hours is an indication for IAP, but that is not the situation described in this vignette.\n\nSuggested Reading(s)\nBriggs-Steinberg C, Roth P. Early-onset sepsis in newborns. Pediatr Rev. 2023;44 (1):14-22. doi:10.1542/pir.2020-001164\nDhudasia MB, Flannery DD, Pfeifer MR, Puopolo KM. Updated guidance: prevention and management of perinatal group B Streptococcus infection. Neoreviews. 2021;22(3):e177-e188. doi:10.1542/neo.22-3-e177\nEgge JA, Anderson RH, Schimelpfenig MD. Care of the well newborn. Pediatr Rev. 2022;43(12):676-690. doi:10.1542/pir.2022-005511\nPrevention of group B streptococcal early-onset disease in newborns: ACOG Committee Opinion No. 797. Obstet Gynecol. 2020;135:e51-e72. doi:10.1097/AOG.0000000000003668\nPuopolo KM, Lynfield R, Cummings JJ, et al; American Academy of Pediatrics, Committee on Fetus and Newborn, Committee on Infectious Diseases. Management of infants at risk for group B streptococcal disease. Pediatrics. 2019;144(2):e20191881. doi:10.1542/peds.2019-2350\n\nContent Domain\nNeonatology, Infections\n\nLearning Objectives\nPlan the management of a neonate whose birthing parent had a history of a group B Streptococcus urinary tract infection"}
{"id" : 378, "question_text" : "A 6-year-old girl presents for a health supervision visit that was scheduled as a follow-up appointment after she had an elevated blood pressure at an urgent care facility during an evaluation for abdominal pain. Her abdominal pain has resolved. Her mother recalls the blood pressure in the urgent care center as 135/90 mm Hg. The girl has had two urinary tract infections with fever in the past, and her father had hypertension diagnosed at age 45 years. On physical examination, the girl's temperature is 37.3°C, heart rate is 90 beats/min, respiratory rate is 20 breaths/min, and blood pressure is 146/86 mm Hg. A repeat blood pressure reading is 142/88 mm Hg. The four limb blood pressures are: 142/88 mm Hg in the right arm, 144/84 mm Hg in the left arm, 156/100 mm Hg in the right leg, and 160/96 mm Hg in the left leg. You find no cardiac murmurs, abdominal bruits, or edema. Femoral pulses are 2+ and symmetrical bilaterally. Renal ultrasonography shows the left kidney to be 8.5 cm with normal corticomedullary differentiation and the right kidney to be 5.5 cm with increased echogenicity. Of the following, the MOST likely cause for this patient's elevated blood pressure is", "options" : "[\"coarctation of the aorta\", \"essential hypertension\", \"renal artery stenosis\", \"renal hypoplasia/dysplasia\", \"renal scarring from prior pyelonephritis\"]", "explanation" : "The most likely cause of the hypertension in this child is renal scarring from prior pyelonephritis. Vesicoureteral reflux (VUR) is discovered in 30% to 50% of children evaluated for febrile UTI. VUR can cause renal parenchymal damage in the form of renal scarring, which is known as reflux nephropathy. A recent study revealed renal scarring in infants who had VUR that increased with increasing severity of reflux: 7%, 21%, and 43% for grades III, IV, and V reflux, respectively. Hypertension can occur in up to 20% of patients who have reflux nephropathy and may take up to 8 years to develop. Conversely, 30% to 40% of pediatric patients who have secondary hypertension have renal scars from VUR. This is one of the reasons why febrile UTIs are evaluated aggressively with renal imaging in the hopes of uncovering VUR at an early age. In addition to increased risk for hypertension, reflux nephropathy also can cause end-stage renal disease later in life. Patients usually respond well to angiotensin-converting enzyme inhibitors for the treatment of hypertension associated with renal scarring.\n\nCritique: The 6-year-old girl described in the vignette presents with hypertension that was initially observed in an urgent care facility and confirmed on two measurements in the clinic. Her past history is notable for two febrile urinary tract infections (UTIs), and her physical examination findings are essentially unremarkable. Of note, femoral pulses are present and blood pressures are higher in the legs than in the arms, observations that make coarctation of the aorta highly unlikely. Essential hypertension is a diagnosis of exclusion, often presenting with mild hypertension in adolescent patients who are overweight and have a positive family history of hypertension. The family history of hypertension in this patient's father is likely insignificant, based on his age of onset. Renal artery stenosis is a rare cause of hypertension in the pediatric patient and may have associated abdominal bruits on examination and a small kidney on ultrasonography. Although this girl does have a small kidney on ultrasonography, the increased echogenicity suggests renal parenchymal injury, which would not be expected in renal artery stenosis. Renal hypoplasia/dysplasia can be associated with an isolated small kidney, but hypertension is uncommon in this setting.\n\nContent Specifications: Recognize the association between vesicoureteral reflux and hypertension"}
{"id" : 2454, "question_text" : "A 17-year-old, new to the practice, is seen for a sports preparticipation evaluation before the start of basketball season. They are healthy and have no signiícant medical history. On a standard preparticipation evaluation form, they acknowledges having had 2 episodes of syncope during cross country meets this academic year. The írst event occurred shortly after the end of a race; they describes feeling dizzy and developing tunnel vision before losing consciousness. The other episode occurred several hundred yards before the ínish line of a race; they felt \"weird\" and had diïculty keeping their balance, resulting in a fall. It is unclear if they truly lost consciousness and they do not have signiícant recollection of the event. Evaluation by the school trainer after each incident revealed with no concerning índings. The patient did not seek further medical attention because they felt better several minutes after each episode. In the oïce today, their vital signs, growth, and physical examination índings are normal. An electrocardiogram is obtained (Figure). Of the following, the BEST next step in the adolescent's management is", "options" : "[\"clear the adolescent for sports participation and recommend vigorous hydration before and during sports activities\", \"clear the adolescent for sports participation without restrictions or further evaluation\", \"Refer the adolescent to a cardiologist and defer clearance for sports participation until after evaluation\", \"refer the adolescent to the emergency department for further evaluation and defer clearance for sports participation\"]", "explanation" : "PREP Pearl(s)\nSyncope occurs in 15% to 20% of pediatric patients; a neurocardiogenic or vasovagal origin is the most common cause.\nCardiac syncope is rare in pediatrics; it is vital to identify the circumstances of the event, as well as any concerning personal or family history to suggest a cardiac cause.\nThorough history and physical examination are key in the evaluation of syncope; a baseline electrocardiogram is an appropriate additional diagnostic tool.\nCritique\nThe best next step in this adolescent's management is referral to a cardiologist for evaluation and defer clearance for sports participation until after their evaluation. Their history of possible exertional syncope and likely postexertional syncope is concerning for a cardiac cause for her symptoms.\nSyncope occurs in 15% to 20% of children and adolescents. Presyncopal symptoms are even more common, particularly in adolescents. Syncope is deíned as a sudden and transient loss of consciousness and postural muscle tone that reverses without intervention. Presyncope or near-syncope describes symptoms of lightheadedness, dizziness, changes in vision and/or hearing, or unsteadiness or weakness without loss of consciousness. Children are often mislabeled as having had a syncopal episode where there is not a loss of postural tone, only an altered level of consciousness. This issue is more than semantic because the diìerential diagnosis is divergent.\nTrue syncope is a concerning symptom for patients, families, and pediatric practitioners. Reassuringly, most cases do not have a primary cardiac cause; rather, approximately 70% of pediatric syncope is neurocardiogenic or reîex in origin. However, because syncope can be a primary symptom of a signiícant pathologic condition, it is vital to obtain further information to assist in ruling in or out a cardiac cause. The personal and family history, a detailed description of the syncopal event, and a careful physical examination help guide clinical decision-making and the need for further testing.\nConcerning or \"red îag\" índings suggestive of a cardiac cause of syncope include the following:\nPersonal history\nCurrent febrile illness\nMultiple episodes of unexplained syncope\nCongenital heart disease or prior cardiac surgery\nPrior Kawasaki disease or other vasculitis or systemic inîammatory disease\nSigniícant decrease in exercise or activity tolerance\nSystemic disease that has known cardiac complications\nEvent history\nExertional syncope\nSyncope initiated by loud noise or emotion\nAbsence of prodromal symptoms (dizziness, lightheadedness, or vision changes)\nSyncope preceded by acute tachycardia or palpitations or signiícant chest pain\nSyncope in a child younger than 8 years\nFamily history\nCardiomyopathy\nGenetic arrhythmogenic syndromes (eg, long QT, catecholaminergic polymorphic ventricular tachycardia [CPVT])\nPacemaker or deíbrillator placement (especially at a young age)\nHeart failure at a young age\nUnexplained sudden death before 50 years of age (eg, drowning or unexplained car crash) or known sudden cardiac death\nPhysical examination índings\nPersistent tachycardia (despite intravascular volume repletion)\nIrregular heart rhythm\nUnexplained tachypnea\nPathologic murmur\nCardiac gallop\nProminent or single S2\nDistant heart sounds\nHepatom egaly\nAbnormal perfusion\nSeveral primary cardiac diseases can lead to syncope. Identiícation of concerning history and/or physical examination índings can point to speciíc causes. These cardiac conditions include but are not limited to the following:\nElectrical cardiac disease\nBrugada syndrome\nCPVT\nHeart block\nIdiopathic ventricular tachycardia\nLong QT syndrome\nWolì-Parkinson-White (WPW) syndrome\nStructural or functional cardiac disease\nCoronary artery abnormalities\nDilated cardiomyopathy (DCM)\nHypertrophic cardiomyopathy (HCM)\nMyocarditis (leading to arrhythmia or ventricular dysfunction)\nRight ventricular dysfunction secondary to pulmonary hypertension\nSevere aortic stenosis\nAfter a careful history review and physical examination, moving forward with a baseline resting electrocardiogram (ECG) is very reasonable. It is important to remember that ECG índings can aid in the diagnosis of some conditions, speciícally those with a primary electrical abnormality (eg, WPW syndrome, Brugada syndrome, and long QT) but performs less well with structural disease. Individuals with coronary abnormalities or CPVT often have a normal resting ECG. Those with HCM, DCM, myocarditis, signiícant aortic stenosis, or long-standing pulmonary hypertension can have variable ECG índings; many are abnormal.\nThe adolescent in the vignette had 1 clear syncopal episode and 1 presyncopal vs syncopal episode; both occurred during or around physical exertion, which is a concerning feature. Postexertional syncope is often multifactorial (dehydration, heat-related, or hyperventilation) and is less likely to have a primary cardiac cause than exertional syncope. However, certain arrhythmias are exacerbated by high sympathetic tone and can present in this manner. The history surrounding a true exertional syncopal event can often be vague, making the details of the event diïcult to obtain. The adolescent in the vignette has a normal ECG (sinus bradycardia and sinus arrhythmia [appropriate for an older teenage aerobic athlete]), which is reassuring, but given their desire to continue to pursue competitive, high-intensity athletics, they require further evaluation by a cardiologist.\nClearing the adolescent for sports participation without further evaluation is not appropriate, considering their history and symptoms, even with normal physical examination índings and a normal ECG. Although their symptoms may ultimately prove to have a neurocardiogenic or vasovagal mechanism, which could respond to maximized îuid intake, the adolescent still requires consultation with a cardiologist. Counseling pediatric athletes regarding vigorous hydration before, during, and after high-level or competitive individual or team activity is important and can mitigate the potential for exertional symptoms. The adolescent is clinically stable and currently asymptomatic. They do not require urgent evaluation in an emergency department.\nSuggested Reading(s)\nAdam HM. Syncope. Point-of-Care Quick Reference. Pediatric Care Online. American Academy of Pediatrics. September 30, 2020. Accessed December 7, 2021. Pediatric Care Online\nCannon B, Wackel P. Syncope. Pediatr Rev. 2016;37(4):159-167; quiz 168. doi:10.1542/pir.2014-0109\nFriedman KG, Alexander ME. Chest pain and syncope in children: a practical approach to the diagnosis of cardiac disease. J Pediatr. 2013;163(3):896-901.e1-3. doi:10.1016/j.jpeds.2013.05.001\nParis Y, Toro-Salazar OH, Gauthier NS, et al; New England Congenital Cardiology Association (NECCA). Regional implementation of a pediatric cardiology syncope algorithm using Standardized Clinical Assessment and Management Plans (SCAMPS) methodology. J Am Heart Assoc. 2016;5(2):e002931. doi:10.1161/JAHA.115.002931\nRedd C, Thomas C, Willis M, Amos M, Anderson J. Cost of unnecessary testing in the evaluation of pediatric syncope. Pediatr Cardiol. 2017;38(6):1115-1122. doi:10.1007/s00246-017-1625-6\nContent Domain\nPreventive Pediatrics\nABP Content Specication(s) / Content Area(s)\nPlan the appropriate evaluation of a syncopal or pre-syncopal episode, including episodes associated with exercise\nRecognize the cardiac causes of syncope"}
{"id" : 1373, "question_text" : "An 18-year-old young man with hemoglobin SS disease presents to the emergency department with a 24-hour history of tactile fever and new-onset shaking chills. He had been previously well. His history is remarkable for 1 admission per year for sickle cell pain crises since he started hydroxyurea 5 years ago. On physical examination, the patient appears somewhat uncomfortable. His temperature is 39.9°C, heart rate is 96 beats/min, respiratory rate is 30 breaths/min, and blood pressure is 90/70 mm Hg. His examination is remarkable for a flow murmur on cardiac examination, but is otherwise normal. Of the following, based on his presentation, the patient's MOST likely diagnosis is", "options" : "[\"acute chest syndrome\", \"Escherichia coli sepsis\", \"hydroxyurea toxicity\", \"splenic sequestration crisis\", \"Streptococcus pneumoniae sepsis\"]", "explanation" : "Hemoglobin SS disease occurs when both β-globin genes located on chromosome 11p15.5 contain a point mutation, resulting in the replacement of glutamic acid with valine at position 6. This results in a qualitatively defective hemoglobin molecule that is prone to polymerization, with resultant deformation of the red blood cell membrane (sickling). This, in turn, leads to an abbreviated red blood cell lifespan, chronic hemolysis, and frequent small vessel occlusion with resultant end-organ damage. Virtually all patients with hemoglobin SS are functionally hyposplenic or asplenic because of chronic sickling and vascular injury in the spleen, and they are prone to bacteremia with encapsulated bacteria.\n\nThe patient in the vignette has presented with signs and symptoms of sepsis, including fever, tachycardia, hypotension, and rigors. It is critical to rapidly recognize sepsis because it can quickly result in disseminated intravascular coagulation and death. Every fever in a child with sickle cell disease should be considered an emergency and treated as bacteremia until proven otherwise. A blood culture should be performed with a complete blood cell count and reticulocyte count, and a broad-spectrum antibiotic (typically a third-generation cephalosporin) should be administered as quickly as possible. Streptococcus pneumoniae is the most frequent causative agent of sepsis in patients with sickle cell disease. Prophylactic daily penicillin in children up to 5 years of age and the use of pneumococcal vaccines have been two of the most significant medical advances for reducing the morbidity and mortality of sickle cell disease. It is important to recognize that even patients who have been taking prophylactic penicillin and have received pneumococcal vaccines can still experience pneumococcal sepsis.\n\nAcute chest syndrome includes a triad of respiratory distress, hypoxemia, and an infiltrate on chest radiography or a clinical lung examination consistent with a focal pneumonia. Although the patient in the vignette is clearly quite ill, he did not present with the stigmata of acute chest syndrome.\n\nHydroxyurea increases the production of hemoglobin F, a fetal variant of hemoglobin that is not prone to polymerization. This decreases the concentration of hemoglobin S in the cell, thereby reducing polymerization, membrane deformation, and sickling. The expanding use of hydroxyurea in the sickle cell population has greatly reduced morbidity, and should be considered in a child with sickle cell disease who has had frequent hospitalizations or life-threatening crises. Hydroxyurea does not cause fever or shaking chills.\n\nSplenic sequestration occurs when sickling in the vasculature of the spleen entraps red blood cells, resulting in rapid splenic engorgement and a severe, potentially life-threatening anemia. Splenic sequestration is most common in children younger than 5 years, but can occur at any age. Affected children typically present with signs of severe anemia (tachycardia, pallor, and fatigue), thrombocytopenia, and a palpable spleen. Fever may or may not be present. Given the patient's age and rigors, splenic sequestration is unlikely.\n\nPREP Pearls\n• Patients with sickle cell disease are functionally asplenic, and thus at risk for sepsis with encapsulated organisms.\n• Streptococcus pneumoniae is the most common cause of sepsis in patients with sickle cell disease.\n\nABP Content Specifications(s)\n• Plan appropriate prophylaxis in children of various ages who have sickle cell disease\n• Recognize complications that increase the risk of death in patients who have sickle cell disease, and manage appropriately\n• Recognize the increased risk of infection with encapsulated organisms in children with asplenia\n\nSuggested Readings\n• Baskin MN, Goh XL, Heeney MM, Harper MB. Bacteremia risk and outpatient management of febrile patients with sickle cell disease. Pediatrics. 2013;131(6):1035-1041. doi: http://dx.doi.org/10.1542/peds.2012-2139.\n• Brousse V, Buffet P, Rees D. The spleen and sickle cell disease: the sick(led) spleen. Br J Haematol. 2014;166(2):165-176. doi: http://dx.doi.org/10.1111/bjh.12950.\n• Driscoll MC. Sickle cell disease. Pediatr Rev. 2007;28(7):259-268. doi: http://dx.doi.org/10.1542/pir.28-7-259.\n• McCavit TL. Sickle cell disease. Pediatr Rev. 2012;33(5):195-206. doi: http://dx.doi.org/10.1542/pir.33-5-195."}
{"id" : 1341, "question_text" : "You are supervising a resident who is seeing a 3-year-old boy for a health supervision visit. The boy's history is significant for chronic kidney disease associated with bilateral dysplastic kidneys. His temperature is 37.8°C, heart rate is 70 beats/min, respiratory rate is 16 breaths/min, and blood pressure is 115/74 mm Hg. His weight is 8 kg (< 5th percentile) and height is 85 cm (< 5th percentile). Other than pallor, the remainder of the boy's physical examination is normal. The resident asks you about associated growth issues and the management of nutrition in children with chronic kidney disease. Of the following, the MOST accurate statement about children with this condition is that", "options" : "[\"growth failure should be treated with recombinant human growth hormone\", \"malnutrition is an uncommon cause of growth failure\", \"they require more than the recommended dietary protein intake for age and sex\", \"they require restriction of fat and water soluble vitamins to less than the recommended intake for age and sex\", \"they require restriction of protein to less than the recommended intake for age and sex\"]", "explanation" : "Correct Answer: C\nIn children with chronic kidney disease (CKD), poor appetite, decreased intestinal absorption of nutrients, and metabolic acidosis lead to malnutrition. Adequate nutrition is essential for optimum growth and neurocognitive development in children. A dietician with expertise in both pediatrics and renal nutrition should collaborate with the treating physician to address the energy, protein, vitamin, mineral, and electrolyte needs of each individual patient.\n\nRestriction of protein intake is not recommended in children, in view of their unique needs for growth and neurocognitive development. Also, protein restriction has not been linked with improved outcomes in CKD. Protein intake between 100% and 140% of the dietary reference intake (DRI), based on age and sex, is recommended for children with CKD and a glomerular filtration rate (GFR) of 30 to 60 mL/min per 1.73 m2. In children with a GFR less than 30 mL/min per 1.73 m2, the recommended protein intake is between 100% and 120% of the DRI for age and sex.\n\nPoor growth is a major complication of children with CKD and a marker for disease severity. Inadequate nutrition and fluid and electrolyte abnormalities, including metabolic acidosis, osteodystrophy, and disturbances of the growth hormone/insulin-like growth factor I axis, contribute to growth impairment in children with CKD. Before initiating therapy with recombinant growth hormone, other factors contributing to growth impairment should be adequately treated. Supplemental enteral feeding via gastrostomy and nasogastric tubes is indicated in children with CKD who have inadequate spontaneous intake to meet growth requirements. Other supportive measures include treatment of (1) electrolyte and fluid losses, (2) metabolic acidosis, (3) anemia, and (4) renal osteodystrophy. Management of renal osteodystrophy includes routine measurement of calcium, phosphorus, parathyroid hormone, and vitamin D levels. Interventions for renal osteodystrophy include dietary phosphorus restriction, vitamin D supplementation, and oral phosphate binders.\n\nChildren with CKD should receive 100% of the DRI for their age and sex for both fat and water-soluble vitamins. In children with a GFR less than 15 mL/min per 1.73 m2, water-soluble vitamin supplementation is indicated. However, vitamin A supplementation is not routinely recommended because of an increased risk for hypervitaminosis A secondary to accumulation of vitamin A metabolites.\n\nPREP Pearls\n• Restriction of protein intake is not recommended in children with chronic kidney disease (CKD), in view of their unique needs for growth and neurocognitive development.\n• Protein restriction has not been linked with improved outcomes in CKD. Protein intake between 100% and 140% of the dietary reference intake (DRI), based on age and sex, is recommended for children with CKD and a glomerular filtration rate (GFR) of 30 to 60 mL/min per 1.73 m2.\n• In children with a GFR less than 30 mL/min per 1.73 m2, the recommended protein intake is between 100% and 120% of the DRI for age and sex. Children with CKD should receive 100% of the DRI for their age and sex for both fat and water-soluble vitamins.\n• Poor growth is a major complication of children with CKD, and a marker for disease severity.\n\nABP Content Specifications(s)\n• Plan the dietary management of renal insufficiency in patients of various ages\n• Recognize the nutritional deficiencies associated with renal disease\n\nSuggested Readings\n• KDOQI Work Group. KDOQI clinical practice guidelines for nutrition in chronic renal failure: 2008 update. Am J Kidney Dis. 2009;53(suppl 2):S1. doi: http://dx.doi.org/10.1053/j.ajkd.2008.11.017.\n• Massengill SF, Ferris M. Chronic kidney disease in children and adolescents. Pediatr Rev. 2014;35(1):16-29. doi: http://dx.doi.org/10.1542/pir.35-1-16.\n• Whyte DA, Fine RN. Chronic kidney disease in children. Pediatr Rev. 2008;29(10):335-341. doi: http://dx.doi.org/10.1542/pir.29-10-335."}
{"id" : 1794, "question_text" : "A 5-year-old, previously healthy boy presents to the emergency department with a 1-month history of progressive fatigue, malaise, and weight loss. He developed a cough and difficulty breathing over the past week, and has needed to be propped up on a pillow to sleep. His temperature is 37.0°C, heart rate is 130 beats/min, respiratory rate is 36 breaths/min, and blood pressure is 90/70 mm Hg. On physical examination, the boy is awake, alert, and in no distress. His breathing is rapid and shallow, but he appears comfortable. There are mild scattered rales bilaterally on lung auscultation. His heart has a regular rhythm without rubs, murmurs, or gallops. His liver is palpable 4 cm below the right costal margin. His extremities are cool, with a capillary refill time of 4 seconds. Echocardiography shows a dilated left atrium and severely decreased left and right ventricular systolic function. Laboratory results are as follows: Sodium 128 mEq/L (128 mmol/L) Potassium 4.5 mEq/L (4.5 mmol/L) Chloride 98 mEq/L (98 mmol/L) Bicarbonate 22 mEq/L (22 mmol/L) Serum urea nitrogen 20 mg/dL (7.1 mmol/L) Creatinine 0.5 mg/dL (44.2 µmol/L) Of the following, the MOST likely explanation for this child's hyponatremia is", "options" : "[\"decreased dietary intake of sodium\", \"decreased renal excretion of water\", \"hypoaldosteronism\", \"increased renal excretion of sodium\", \"pseudohyponatremia\"]", "explanation" : "The boy in the vignette has congestive heart failure, as evidenced by fatigue, weight loss, orthopnea, and suggestive echocardiographic findings. In congestive heart failure, antidiuretic hormone (ADH) release is increased, which decreases the excretion of water in the distal nephron, resulting in hyponatremia.\n\nSodium is the predominant extracellular cation, as most of the body's stores are in the extracellular compartment. Sodium is also the major determinant of plasma osmolality, which is approximated by the following formula:\n\nPlasma osmolality (mOsm/kg) = 2*Na + BUN / 2.8 + plasma glucose / 18\n\nPlasma osmolality is tightly controlled between 280 and 295 mOsm/kg by osmostatic mechanisms in the posterior pituitary gland through regulation of the thirst mechanism and ADH. Serum sodium level and serum osmolality are lowered both by water intake through the thirst mechanism and by decreased water excretion in response to the action of ADH on the collecting ducts of the nephron. ADH causes insertion of aquaporin channels into the basolateral membrane of the collecting duct cells, resulting in reabsorption of intraluminal water into the bloodstream.\n\nConditions in which hyponatremia is caused by increased ADH secretion include congestive heart failure, syndrome of inappropriate ADH (SIADH), neurologic conditions such as traumatic brain injury and after neurologic surgery, and pulmonary infections. In many of these conditions sodium stores are normal. These causes of hyponatremia, in which the water content is in excess in relation to sodium, comprise dilutional hyponatremia; this is the most common form of hyponatremia. Water retention and sodium depletion can occur with diarrhea and vomiting in the context of a normal thirst mechanism.\n\nHypertonic hyponatremia, sometimes referred to as pseudohyponatremia, can occur in the context of elevated osmotically active solutes. The most common presentation is in the setting of hyperglycemia in diabetic ketoacidosis. In response to hyperosmolality, water moves from the intracellular to the extracellular compartment, and dilutes the plasma sodium. Factitious hyponatremia occurs when severe hyperproteinemia and hyperlipidemia cause a laboratory artifact, falsely lowering the serum sodium level. Because these compounds occupy volume in the sample, the sodium distributed in the aqueous compartment of the sample is measured at a lower concentration.\n\nDecreased dietary intake of sodium rarely causes hyponatremia, as the minimum dietary requirement of sodium is easily met, outside of extreme starvation. Hypoaldosteronism can cause hyponatremia, but the aldosterone axis is usually increased in patients with congestive heart failure. Renal excretion of sodium can be increased in cases of cerebral salt wasting, renal tubular dysfunction, or diuretic use, but none of these is present in the boy in the vignette. Pseudohyponatremia can occur in certain hyperosmolar conditions, such as diabetic ketoacidosis, but no such conditions are present in the boy in the vignette."}
{"id" : 753, "question_text" : "An 8-year-old boy is seen in your office for problem behavior. He will not stay in his seat at school, does not pay attention well in class, and does not seem to be learning as expected. Last year, he was treated with methylphenidate followed by dextroamphetamine with no significant effect. He is becoming increasingly disruptive in class and refusing to do work. Notes are now being sent home repeatedly reporting aggressive behavior with others. Upon questioning, you learn that there appears to be no new social stressors, he has not been bullied, and there is no particular family disruption. His school and home behavior have worsened each year, but he is far less disruptive at home. According to his mother, he achieved his early motor milestones at the expected times. His physical examination is unremarkable, with no dysmorphic features, and normal vision and hearing. Of the following, the MOST appropriate next step is to", "options" : "[\"perform a blood lead level test\", \"recommend an electroencephalogram\", \"recommend genetic testing\", \"recommend a psychoeducational evaluation\", \"refer the boy for evaluation of possible child abuse\"]", "explanation" : "The ineffectiveness of both methylphenidate and dextroamphetamine to treat what was initially presumed to be attention-deficit/hyperactivity disorder (ADHD) suggests that something other than ADHD is causing problems for the boy described in the vignette. Behavior problems at school that are far greater than at home, overall worsening with each year, and very poor school performance are suggestive of a learning disability. Children who cannot learn as easily as others in the class often develop increasing frustration and feelings of inadequacy, which can be expressed as disruptive behavior and even aggression. Schools are required by special education law to respond to a parent's learning concerns for their child (particularly if that concern is expressed in writing rather than verbally) but are notably not required to respond to a physician's concerns about a learning disability. Therefore, encouraging the child's parent to reach out to the school to obtain a psychoeducational evaluation is the most effective next step. Other worthwhile steps might include performing screening assessments for both vision and hearing to make sure the child has no sensory problems affecting his ability to learn.\n\nChecking a lead level may be warranted in a young child who has ADHD symptoms or learning difficulties and who is living in a home, or spending time in an environment that might contain lead paint (ie, contains paint from before 1977) or other sources of lead exposure. However, the steady worsening of symptoms in an 8-year-old child is unlikely to be caused by lead toxicity unless that child is continuing to increase his exposure to toxic lead. Pica, as the primary path-way to lead toxicity, is fairly common in children younger than age 5 years but uncommon in school age children unless they have a major developmental impairment or suffer from severe neglect. Although children with seizure disorders can have comorbid learning disabilities, the absence of a seizure history makes electroencephalography unlikely to be helpful. Although genetic syndromes are often associated with learning disabilities, the absence of dysmorphic features or hallmark traits suggestive of a genetic disorder makes genetic testing unlikely to be helpful. Although inattention and behavior problems at school could result from child abuse, no information in this child's history suggests abuse. Referral for an abuse evaluation in a case where there is no reason to suspect abuse is likely to cause unnecessary distress for the family and potential mistrust of clinicians.\n\nPREP Pearls\n• Learning disabilities are common in children with ADHD.\n• For children who appear to have ADHD but fail to respond to stimulants, evaluation for a learning disability should be considered.\n\nAAP Mental Health Competency:\n• Recognize the risk factors behind chronic aggression, such as the high rate of learning disorders and academic underachievement in conduct disordered children\n\nSuggested Reading:\n• American Academy of Child and Adolescent Psychiatry. Practice parameters for the assessment and treatment of children and adolescents with language and learning disorders. J Am Acad Child Adolesc Psychiatry. 1998;37(10 suppl):465-62S.\n• American Academy of Pediatrics. Learning disabilities, dyslexia, and vision: a subject review. Pediatrics. 2009;124(2):837-844. doi: 10.1542/ peds.2009-1445"}
{"id" : 1502, "question_text" : "A 4-year-old fully vaccinated boy presents to the pediatric emergency department with fever and respiratory distress. His past medical history includes recurrent otitis media and 2 hospitalizations for pneumonia. Vital signs show a temperature of 39.4°C, respiratory rate of 40 breaths/min, heart rate of 150 beats/min, and blood pressure of 95/65 mm Hg. On physical examination, he has marked stridor and is drooling. Anesthesia is called for airway management and, on intubation, they observe the image shown in Item Q253. A blood culture is positive for Haemophilus influenzae type b.\n\nOf the following, the study that would BEST assess vaccine response in this patient is", "options" : "[\"delayed cutaneous hypersensitivity\", \"humoral immune panel\", \"lymphocyte proliferation assay\", \"phagocytic oxidative response\", \"total hemolytic complement\"]", "explanation" : "The best study to assess vaccine response is a humoral immune panel. The child in the vignette is fully immunized, but has an invasive infection, epiglottitis, and bacteremia due to Haemophilus influenzae type b. This raises concern for an inadequate vaccine response. The humoral immune panel would allow one to measure antibody titers to several vaccine antigens, allowing for an assessment of vaccine response.\n\nGenerally, immunity is divided into 2 components, the innate and adaptive immune system. The adaptive immune system is further divided into the cellular and humoral immune systems. The complement system functions in both innate and adaptive immunity. The innate immune system is comprised of proteins and cells, including monocytes, neutrophils, macrophages, and natural killer cells. The cellular and humoral immune systems is composed of T- and B-lymphocytes, respectively. However, this is a simplistic approach to the immune system because there is cross-talk between components of the various systems and defects can occur that affect several aspects of immunity simultaneously.\n\nDefects of the cellular immune system or T-lymphocyte function tend to present with failure-to-thrive, chronic diarrhea, and recurrent opportunistic infections, including cytomegalovirus, Candida, and Pneumocystis jirovicii. Disorders of T-lymphocyte function include severe combined immunodeficiency, DiGeorge syndrome, and X-linked hyperimmunoglobulin M. A complete blood cell count is the first test for assessing a defect of the cellular immune system, as some disorders are characterized by lymphopenia. If there is concern for an acquired defect of the cellular immune system, HIV testing should also be pursued. Absence of lymphopenia does not rule out a cellular defect, as qualitative or functional defects can be present. Delayed cutaneous hypersensitivity and the lymphocyte proliferation assay are 2 means of assessing T-lymphocyte function.\n\nDefects of the humoral immune system present as recurrent sinopulmonary infections with encapsulated bacteria. Disorders of the humoral immune system include X-linked aggamaglobulinemia and common variable immunodeficiency. Defects in humoral immunity can be detected by measuring serum immunoglobulins. Additionally, some disorders are characterized by low to absent B cells, detectable by flow cytometry.\n\nDefects of the innate immune system have varied presentations and include disorders such as chronic granulomatous disease (CGD), leukocyte adhesion defects, and complement deficiencies. Phagocyte oxidative response evaluates the neutrophil defect of CGD and total hemolytic complement (CH50) determines complement activity.\n\nPREP Pearls\n• The humoral immune panel assesses vaccine response by measuring antibody titers to several vaccine antigens.\n• A complete blood cell count is the first test for assessing a defect of the cellular immune system, as some disorders are characterized by lymphopenia.\n• Defects in humoral immunity can be detected by measuring serum immunoglobulins.\n\nABP Content Specifications(s)\n• Recognize the clinical findings associated with combined antibody and cellular immunodeficiency\n• Plan the laboratory evaluation of antibody function\n• Plan the laboratory evaluation of cell-mediated immunity\n\nSuggested Readings\n• Bonilla FA, Khan DA, Ballas ZK, et al. Practice parameter for the diagnosis and management of primary immunodeficiency. J Allergy Clin Immunol. 2015;136(5):1186-1205. doi: http://dx.doi.org/10.1016/j.jaci.2015.04.049.\n• Fleisher TA. Primary immune deficiencies: windows into the immune system. Pediatr Rev. 2006;27(10):363-372. doi: http://dx.doi.org/10.1542/pir.27-10-363."}
{"id" : 1160, "question_text" : "A previously healthy 12-year-old boy complains of worsening vision in the left eye over the last several weeks without other symptoms. His visual acuity is 20/80 in the left eye and 20/20 in the right eye. Fundoscopic examination of the left eye reveals a chorioretinal scar with surrounding retinitis nasal to the disk and mild overlying vitritis (Item Q95). The right eye is normal.\nOf the following, the test MOST likely to establish the cause of the patient's findings is", "options" : "[\"blood culture for Treponema pallidum\", \"microscopy of corneal scraping for Acanthamoeba\", \"serum immunoglobulin G for Toxoplasma gondii\", \"serum polymerase chain reaction for cytomegalovirus\", \"swab of ocular mucous membranes for herpes simplex virus culture\"]", "explanation" : "The boy in the vignette has isolated ocular toxoplasmosis as evidenced by the characteristic retinal lesions (chorioretinitis) in an otherwise healthy child. The diagnosis is suggested by detecting immunoglobulin G antibody to T gondii in serum, but is based largely on the characteristic appearance of the chorioretinal lesion, usually described as white focal retinitis with overlying vitreous inflammation that has the appearance of a \"headlight in the fog.\" In addition, an adjacent pigmented retinochoroidal scar also may be seen.\nIsolated ocular toxoplasmosis develops in up to 85% of adolescents and young adults after untreated congenital infection. It also can become reactivated years after initial infection in both immunocompetent and immunocompromised individuals. Patients with ocular toxoplasmosis may complain of blurred vision, decreased acuity, epiphora (excessive tear formation), eye pain, floaters, photophobia, or scotoma. Chorioretinitis caused by Toxoplasma infection may lead to vision loss.\nIn most immunocompetent individuals, Toxoplasma infection either is asymptomatic or results in a self-limited and benign flulike illness. Rare complications include pneumonia, myocarditis, pericarditis, hepatitis, and cutaneous involvement. In patients with immunodeficiency, such as that caused by infection with human immunodeficiency virus, life-threatening manifestations of T gondii infection can include encephalitis, pneumonia, or disseminated disease. Infants with congenital toxoplasmosis are asymptomatic in the majority (70%-90%) of cases, but may develop sequelae such as learning disability, mental retardation, hearing loss, or vision impairment later in life. Infants with symptomatic congenital infection can present with various signs such as lymphadenopathy, rash, hepatosplenomegaly, petechiae, thrombocytopenia, pneumonitis, meningoencephalitis, and chorioretinitis. Although rare, the classic triad of hydrocephalus, cerebral calcifications, and chorioretinitis is highly suggestive of congenital infection caused by T gondii.\nToxoplasma gondii is found worldwide, and the seroprevalence (11% in the United States) of infection varies by geographic area and socioeconomic status. Felines are definitive hosts. Intermediate hosts such as pigs, sheep, and cattle can have tissue cysts in multiple organs. Humans become infected by ingestion of oocysts from soil (or cat feces) or contaminated food or water. Congenital infection results from primary maternal infection during gestation and is estimated to occur in 1 in 1,000 to 1 in 10,000 live births.\nMost cases of acquired toxoplasmosis in immunocompetent hosts do not require antimicrobial therapy. Item C95 shows antimicrobial regimens for the treatment of toxoplasmosis, when indicated. Indications for treatment include pregnancy, immunocompromised status, severe symptoms (eg, chorioretinitis or organ damage), or persistent symptoms.\nThe duration of therapy for congenital toxoplasmosis is prolonged, usually lasting approximately 1 year. For immunocompromised patients, long-term antibiotic suppression often is indicated after the initial course of therapy to prevent recurrence.\nOcular manifestations of infection with T pallidum (syphilis) can include uveitis (anterior or posterior) that usually is granulomatous. Posterior uveitis is more common and manifests as chorioretinitis that is multifocal, retinal necrosis, or optic neuritis. Patients usually have decreased visual acuity, often in association with syphilitic meningitis. The diagnosis is made serologically and not with blood culture.\nAcanthamoeba causes keratitis that is indolent and can resemble keratitis caused by herpes simplex virus (HSV) or bacteria. Patients typically present with severe pain, photophobia, tearing, and the sensation of a foreign body in the eye. Classic findings include a stromal ring infiltrate and radial keratoneuritis. Diagnosis is made with corneal scrapings to identify the organism.\nCytomegalovirus retinitis is rare in immunocompetent individuals and usually involves the anterior chamber of the eye. The diagnosis can be made with polymerase chain reaction analysis of anterior chamber aqueous fluid. HSV can cause keratitis which presents as ocular pain, visual blurring, and discharge. Patients will have conjunctivitis, chemosis, dendritic lesions of the cornea, and decreased corneal sensation. The diagnosis usually is clinical, but viral culture from ocular mucous membranes may isolate the pathogen. HSV also can cause acute retinal necrosis, usually in immunocompromised individuals and pregnant women. Children presenting with decreased vision are reported to have bilateral acute retinal necrosis caused by HSV. Neonates can have chorioretinitis caused by HSV.\n\nPREP Pearls\n\nIn most immunocompetent individuals, Toxoplasma infection either is asymptomatic or results in a self-limited and benign flulike illness.\n\nIn ocular toxoplasmosis, the chorioretinal lesion is described as white focal retinitis with overlying vitreous inflammation that has the appearance of a \"headlight in the fog.\"\n\nIsolated ocular toxoplasmosis develops in up to 85% of adolescents and young adults after untreated congenital infection.\n\nMost cases of acquired toxoplasmosis in immunocompetent hosts do not require antimicrobial therapy.\n\nPyrimethamine plus sulfadiazine are the drugs of choice for treating toxoplasmosis.\n\nABP Content Specifications(s)\n\nIdentify the clinical features associated with congenital and acquired Toxoplasma gondii infestation, and manage appropriately\n\nUnderstand the epidemiology of Toxoplasma gondii"}
{"id" : 746, "question_text" : "The mother of a 17-year-old girl made an appointment for her daughter to see you for a gynecologic examination. She learned that her daughter had 2 Chlamydia infections in the past year and requests that her daughter be \"fully tested\" and have a Papanicolaou (Pap) smear. The girl is otherwise healthy and asymptomatic today. She has received 3 doses of quadrivalent human papillomavirus (HPV) vaccine. Of the following, the advice you are MOST likely to provide is that Pap smear testing should be", "options" : "[\"deferred until the girl reaches 18 years of age\", \"deferred until the girl reaches 21 years of age\", \"performed today because it has been requested\", \"performed today because of the girl's history of Chlamydia infection\", \"unnecessary because the girl has been immunized against HPV\"]", "explanation" : "Traditionally, screening with cervical cytologic testing for precancerous changes (ie, cervical dysplasia and cervical intraepithelial neoplasia) was routinely performed in all sexually active females using the Papanicolaou (Pap) smear. Recent recommendations from the US Preventive Services Task Force (USPSTF) have changed the timing of initiating screening to 21 years and older, and their recommendation is against screening for cervical cancer in women younger than 21 years because epidemiologic studies indicate that cervical cancer is rare in this age group. Exposure of the cervical cells to oncogenic types of human papilloma virus (HPV) during vaginal intercourse may eventually lead to cervical cancer. However, this process is not rapid; most often the virus is cleared and early lesions regress. These recommendations don't apply to those females who are immunocompromised Evidence indicates that there is more harm than benefit from Pap smear screening before 21 years of age. Complications from diagnostic procedures (eg, cervical biopsies), as documented in studies, include vaginal bleeding, pain, and infection. Abnormal results could result in short-term increase in anxiety from health concerns. Risks from the treatment procedure (eg, loop electrosurgical excision procedure [LEEP]) include the potential for adverse pregnancy outcomes, such as preterm delivery with an associated low birth-weight infant and an increased risk for perinatal death.\n\nIn their decision analyses, the USPSTF found little evidence of the influence of sexual history on the age at which to begin screening. Therefore, there is no need to screen patients earlier if they have had sexually transmitted infections. The fact that someone has been vaccinated against HPV does not negate the need for Pap smear screening starting at 21 years of age.\n\nPREP Pearls\n• Routine Pap smear testing should start at 21 years of age; a history of sexually transmitted infections does not change this recommendation.\n• Diagnostic procedures on the cervix increase the risk for future negative pregnancy outcomes.\n\nAmerican Board of Pediatrics Content Specification(s):\n• Know the indications for a Papanicolaou smear in adolescence\n\nSuggested Reading:\n• ACOG Committee on Practice Bulletins—Gynecology. ACOG Practice Bulletin no. 109: cervical cytology screening. Obstet Gynecol. 2009;114:1409-1420. doi:10.1097/A0G.0b0l3e3l81c6f8a4\n• Greydanus DE, Omar H, Patel DR. What's new: cervical cancer screening in adolescents? Pediatr Rev. 2009;30:23-25. doi:10.1542/pir.30-1-23\n• Moyer VA; on behalf of the U.S. Preventive Services Task Force. Screening for Cervical Cancer: U.S. Preventive Services Task Force Recommendation Statement. Ann Intern Med. 2012; 156:880-891, W312. doi:10.7326/0003- 4819-156-12-201206190-00424\n• Saslow D, Solomon D, Lawson HW, et al; American Cancer Society; American Society for Colposcopy and Cervical Pathology; American Society for Clinical Pathology. American Cancer Society, American Society for Colposcopy and Cervical Pathology, and American Society for Clinical Pathology screening guidelines for the prevention and early detection of cervical cancer. Am J Clin Pathol. 2012;137:516-5142. doi:10.1309/AJCPTGD94EVRSJCG"}
{"id" : 3709, "question_text" : "A 9-month-old infant is seen for a health supervision visit. Her initial visit was 5 weeks ago when her new adoptive parents brought her to the clinic, and she received her initial set of vaccinations including DTaP. At that time, she was well nourished and developing well. The infant's mother has heard a news story about pertussis and inquires about when her daughter can have her second dose of DTaP. Of the following, the BEST response to the mother is", "options" : "[\"today\", \"in 1 week\", \"at 12 months of age\", \"at 15 months of age\"]", "explanation" : "The Centers for Disease Control and Prevention catch-up vaccination schedule recommends 4 weeks between the first and second dose of DTaP. For the infant in this vignette, the first dose was given 5 weeks ago, making today the preferred response for the timing of the next dose. DTaP contains several diphtheria and tetanus toxoids as well as acellular pertussis. Children should receive 5 doses of DTaP prior to the age of 7 years. Typically the first dose is given at 2 months but can be given as early as 6 weeks. The following doses are typically given at 4 months, 6 months, 15 months, 18 months, and between 4 and 6 years of age. If a child does not receive vaccines on a typical schedule, the minimum intervals between doses are as follows: • Between first and second doses: 4 weeks • Between second and third doses: 4 weeks • Between third and fourth doses: 6 months • Between fourth and fifth doses: 6 months Tdap, which should be used for children aged 7 years and older, contains 2 tetanus toxoids, a reduced amount of diphtheria toxoid, and acellular pertussis. It is recommended that children receive 1 dose as a booster between 11 and 12 years of age. After the first 5 vaccine doses, there is 80% to 85% efficacy against severe pertussis when measured as decrease in paroxysmal cough greater than 21 days. Adverse effects such as seizures, high fever, or hypotonic hyporesponsive episodes are decreased by using the acellular pertussis vaccine. The cellular version continues to be used outside the United States. Local reactions are the most common adverse effect and increase in severity with each dose. By the fourth or fifth dose, up to 2% to 3% of children have swelling of the entire extremity. This is not an allergic reaction, and if it occurs after the fourth dose is not a contraindication to the fifth dose because most children do not have the same reaction again. It is recommended that children receive vaccinations at the earliest recommended time; therefore, waiting 1 week or until 12 or 15 months of age is not the preferred response. PREP Pearls • Children should receive 5 doses of DTaP prior to 7 years of age. • Catch-up vaccination schedules are available on the Centers for Disease Control and Prevention website. • One dose of Tdap should be given between 11 and 12 years of age. ABP Content Specifications(s) • Plan the administration of DT or dT based on the age of the patient • Know the differences in the composition of DT and dT • Plan subsequent DTaP and Tdap immunization for a patient with a prior reaction to DTaP • Know the indications, contraindications, and schedules for diphtheria, tetanus, and pertussis vaccines Suggested Readings • Centers for Disease Control and Prevention. Catch-up immunization schedule for persons aged 4 months through 18 years. https://www.cdc.gov/vaccines/schedules/hcp/imz/catchup.html. • Souder E, Long SS. Pertussis (Bordetella pertussis and Bordetella parapertussis). In: Kliegman RM, St Geme JW III, Blum NJ, Shah SS, Tasker RC, Wilson KM, eds. Nelson Textbook of Pediatrics. 21st ed. Philadelphia, PA: Elsevier; 2020:1492-1496."}
{"id" : 3756, "question_text" : "A 6-year-old girl is being evaluated for developmental delay. She is quite outgoing and talkative during the visit; however, her cognitive skills are at the level of a 3 year old. Her full-scale IQ was 50 on a recent educational psychology assessment. Her verbal IQ was significantly higher than her visual-spatial score. She has a history of idiopathic hypercalcemia and mild hypotonia. On physical examination, she has facial dysmorphology most notable for bitemporal narrowing, periorbital fullness, long philtrum, short nose, wide mouth with full lips, and stellate blue eyes. A crescendo-decrescendo low-pitched systolic murmur is noted at the cardiac base with radiation to the right carotid artery. The precordium is hyperdynamic and carotid pulses are decreased to the left upper limb. Microarray testing reveals a contiguous gene deletion of 7q11.23. Of the following, the MOST likely cardiac defect in this girl is", "options" : "[\"atrial septal defect\", \"supravalvar aortic stenosis\", \"tetralogy of Fallot\", \"ventricular septal defect\"]", "explanation" : "Correct Answer: B\nThe girl in the vignette has Williams syndrome resulting from a contiguous gene deletion in 7q11.23 that encompasses the elastin gene (ELN). Penetrance is 100% while expression is variable.\n\nThe most common cardiovascular finding in Williams syndrome is supravalvar aortic stenosis, which is present in 75% of individuals. Other cardiovascular manifestations include elastin arteriopathy, peripheral pulmonary stenosis, and hypertension. Distinctive facies include bitemporal narrowing, periorbital fullness, stellate irides, long philtrum, upper/lower lips with a thickened vermilion, wide mouth, small jaw, large ear lobes, and a broad forehead. Individuals commonly possess a specific neurocognitive profile with overfriendliness, generalized anxiety, attention-deficit/hyperactivity disorder, strengths in verbal short-term memory and language, and weakness in visual-spatial construction. Most have intellectual disability, typically mild. Growth abnormalities are characterized by prenatal growth deficiency and slow weight gain and linear growth in the first 4 years, and a brief pubertal growth spurt resulting in short stature. Endocrine abnormalities can include idiopathic hypercalciuria and hypothyroidism. The diagnosis of Williams syndrome is confirmed via chromosomal microarray or targeted deletion analysis using fluorescence in situ hybridization.\n\nA contiguous gene deletion syndrome is caused by a microdeletion that encompasses 2 or more genes in tandem position along a chromosome. By virtue of the fact that several genes are involved, contiguous gene syndromes often affect multiple systems of the body. Thus, one must assess the function of the specific genes involved within the deletion and thoroughly examine the patient for involvement for those specified regions (heart, kidney, brain, etc). Other common contiguous gene deletions include 22q11.2 deletion syndrome, 1p36 deletion, Smith-Magenis syndrome, and Cri-du-chat syndrome.\n\nVentricular septal defect and atrial septal defect are common congenital heart defects, but not the most common ones noted in an individual with Williams syndrome. Tetralogy of Fallot is typically associated with 22q11.2 deletion syndrome.\n\nPREP Pearls\n• A contiguous gene deletion syndrome is caused by a microdeletion that encompasses 2 or more genes in tandem position along a chromosome.\n• Williams syndrome is a contiguous gene deletion syndrome arising from a recurrent deletion in the 7q11.23 region encompassing the elastin gene (ELN).\n• Williams syndrome is characterized by cardiovascular disease, typical facies, connective tissue abnormalities, intellectual disability, idiopathic hypercalciuria/hypothyroidism, growth issues, and a specific neurocognitive profile; the most common cardiovascular finding is supravalvar aortic stenosis.\n\nABP Content Specifications(s)\n• Recognize the clinical and laboratory findings associated with contiguous gene syndromes\n• Recognize the inheritance pattern associated with contiguous gene syndromes\n\nSuggested Readings\n• American Academy of Pediatrics, Committee on Genetics. Health care supervision for children with Williams syndrome. Pediatrics. 2001;107(5):1192-1204. https://pediatrics.aappublications.org/content/107/5/1192.\n• Lacroix A, Famelart N, Guidetti M. Language and emotional abilities in children with Williams syndrome and children with autism spectrum disorder: similarities and differences. Pediatr Health Med Ther. 2016;7:89-97. doi:10.2147/PHMT.S66347.\n• McCulloch MA, Gajarski RJ. Congenital and acquired heart disease. In: McInerny TK, Adam HM, Campbell DE, DeWitt TG, Foy JM, Kamat DM, eds. American Academy of Pediatrics Textbook of Pediatric Care. 2nd ed. Itasca, IL: American Academy of Pediatrics; 2016:1883-1917. Pediatric Care Online.\n• Morris CA. Williams syndrome. GeneReviews. https://www.ncbi.nlm.nih.gov/books/NBK1249/."}
{"id" : 1442, "question_text" : "A 3-year-old boy with severe obstructive sleep apnea underwent a tonsillectomy and adenoidectomy. There were no reported complications in the operating room and there was minimal bleeding. Postoperatively, he is admitted to the hospital for observation. Within 6 hours after the procedure, he develops a progressive oxygen requirement and respiratory distress. Vital signs show a temperature of 37°C, pulse of 120 beats/min, respiratory rate of 40 breaths/min, and blood pressure of 100/60 mm Hg. Pulse oximetry is 85% on 100% non-rebreather facemask. Physical examination reveals a tired-appearing child in severe respiratory distress. Tonsillectomy surgical sites appear clean and intact, with minimal bleeding. He has moist mucous membranes. Heart is regular. He is breathing shallowly, with subcostal and intercostal retractions. Air entry is adequate and equal bilaterally, with scattered crackles throughout. Abdomen is soft, nontender, and non-distended with no organomegaly. Arterial blood gas analysis reveals pH of 7.5, PaCO2 of 30 mm Hg, and PaO2 of 50 mm Hg. Chest radiograph is shown in Item Q193. Of the following, the MOST likely cause of his respiratory failure is", "options" : "[\"congestive heart failure\", \"pneumonia\", \"pulmonary edema\", \"surgical bleeding\", \"upper airway obstruction\"]", "explanation" : "Correct Answer: C\nThe boy in the vignette has respiratory failure after a tonsillectomy and adenoidectomy procedure, which he underwent due to severe obstructive sleep apnea. The clinical picture of rapid, shallow breathing, crackles, and radiographic appearance indicate that he has pulmonary edema.\n\nStarling's law states that forces favoring filtration out of any capillary bed include increased capillary permeability, intraluminal hydrostatic pressure, and interstitial oncotic pressure, whereas forces preventing filtration include interstitial hydrostatic pressure and intraluminal oncotic pressure. Pulmonary edema, similar to edema in any tissue bed, occurs because of Starling forces favoring filtration out of capillary bed. Pulmonary edema is commonly caused by increased capillary permeability in sepsis and pneumonia, by increased intraluminal hydrostatic pressure in heart failure, and by decreased intraluminal oncotic pressure in hypoproteinemia.\n\nClinical signs of pulmonary edema include rapid, shallow breathing, hypoxia, retractions, and crackles. Tachypnea is caused by the effect of increased fluid on the pulmonary interstitial stretch receptors, which feed back to the brainstem respiratory center. This can cause respiratory alkalosis, as is seen in the boy in this vignette, unless respiratory failure is so profound as to cause hypercapnia. Primary cardiac etiologies can be ruled out as a cause of this child's pulmonary edema by the lack of significant tachycardia, hepatomegaly, jugular venous distention, diminished peripheral perfusion, and lower extremity edema. Treatment varies widely based on the primary etiology. Radiographic findings also vary based on etiology and can include consolidation in the case of pneumonia, increased interstitial lung markings, and prominent pulmonary vasculature in the case of heart failure.\n\nThe child in this vignette has postobstructive pulmonary edema following a tonsillectomy and adenoidectomy. Pulmonary edema can occur during or after the relief of either acute or chronic upper airway obstruction such as seen in croup, epiglottitis, postextubation subglottic edema, or obstructive sleep apnea. The etiology of postobstructive pulmonary edema is uncertain. One theory states that breathing against a severe airway obstruction requires negative intrathoracic pressure that increases venous return and decreases cardiac output, favoring filtration of fluid into the alveoli and pulmonary interstitium. Another possibility is that positive end-expiratory pressure is required to overcome an upper airway obstruction, and its sudden removal after a corrective therapy favors fluid transudation. Treatment of postobstructive pulmonary edema includes oxygen, diuretics, and in more severe cases, application of positive end-expiratory pressure either noninvasively or with intubation and mechanical ventilation.\n\nCongestive heart failure can be a cause of pulmonary edema, but it is less likely for the child in this vignette because there is no hepatomegaly or signs of decreased cardiac output. Pneumonia can occur postoperatively, but it is unlikely to cause new symptoms within 6 hours of the procedure. Surgical bleeding can be a cause of respiratory failure postoperatively after a tonsillectomy and adenoidectomy, but the surgical sites are clean and intact. Upper airway obstruction as a cause of respiratory failure presents with stridor and deep retractions, as opposed to hypoxia, tachypnea, and crackles.\n\nPREP Pearls\n• Pulmonary edema presents with rapid, shallow breathing, hypoxia, and crackles.\n• Postobstructive pulmonary edema can occur in conditions of upper airway obstruction before and after relief of obstruction.\n\nABP Content Specifications(s)\n• Recognize the clinical and laboratory manifestations associated with respiratory failure of various etiologies\n\nSuggested Readings\n• Nitu ME, Eigen H. Respiratory failure. Pediatr Rev. 2009;30(12):470-478. doi: http://dx.doi.org/10.1542/pir.30-12-470.\n• Van Kooy MA, Gargiulo RF. Postobstructive pulmonary edema. Am Fam Phys. 2000;62(2):401-404. http://www.aafp.org/afp/2000/0715/p401.html."}
{"id" : 1714, "question_text" : "A 12-year-old girl with a history of inflammatory bowel disease is brought to your office for evaluation of increasing symptoms. She has been taking drug A for years. The girl's mother mentions that she has learned of the release of a new drug (drug B) for adults with inflammatory bowel disease that is boasted to have superior outcomes. You explain that the new drug is not approved for use in children and that further studies are needed before it can be used in children. Of the following, the study design that would BEST determine if drug B produces superior outcomes in children is", "options" : "[\"an administrative database review\", \"a case-control study\", \"a cohort study\", \"a cross-sectional study\", \"a randomized controlled trial\"]", "explanation" : "Data obtained using large sample sizes, collected free from bias, and analyzed in a statistically appropriate fashion will help a clinician in the decision-making process. However, such large studies are impractical, expensive, and sometimes impossible for rare diseases. Knowing the limitations and benefits of different study designs and data sources will aid in the interpretation of study data and the evaluation of its validity.\n\nIn this vignette, the physician and the patient's mother discuss a current medication (drug A) compared to a new medication (drug B). A randomized controlled trial is the best way to minimize bias and make a comparison of these 2 drugs with minimal confounders, if the groups are formed in a truly randomized manner.\n\nAdministrative database review is helpful to provide data regarding hospitalizations and mortality in a large population over a long time. This approach is not helpful in determining the comparative efficacy of 2 drugs.\n\nCase-control studies would be useful to study individuals with a disease compared to individuals without the disease to evaluate risk factors and outcomes for the disease. As these studies are mostly retrospective analyses, data must be considered in the context of the important bias that is inherent in this design. Participants with the disease may be more likely to look for risk factors and associations as compared to participants without the disease (recall bias). Case-controlled studies are not designed to compare different treatments of a disease.\n\nCohort studies follow a population of patients with a disease over time. This approach is likely to yield important information about the natural history of a disease and associated manifestations and complications. A cohort study is not an appropriate study for the clinical question posed in the vignette.\n\nA cross-sectional study would be more appropriate in the context of comparing a new diagnostic test to the gold standard. Compared in the same population, this study could illuminate the differences in the 2 tests, and the classic 2 × 2 table could organize the data in a way to establish the sensitivity and specificity of each test.\n\nPREP Pearls\n• A randomized controlled study is the optimal way to evaluate the effect of 2 different treatments for a disease.\n• Case-control studies would be useful to study individuals with a disease compared to individuals without the disease to evaluate risk factors and outcomes for the disease.\n• Cohort studies follow a population of patients with a disease over time. This approach is likely to yield important information about the natural history of a disease\n\nMOCA-Peds Objective\n• Understand the principles and application of study design\n\nABP Content Specifications(s)\n• Assess how the data source (eg, diaries, billing data, discharge diagnostic code) may affect study results\n\nSuggested Readings\n• Palaia A. Study design and data sources. Pediatr Rev. 2013;34(8):371–372. doi: http://dx.doi.org/10.1542/pir.34-8-371.\n• Perry-Parrish C, Dodge R. Validity hierarchy for study design and study type. Pediatr Rev. 2010;31(1):27–29. doi: http://dx.doi.org/10.1542/pir.31-1-27."}
{"id" : 2928, "question_text" : "An 8-year-old boy with asthma is brought to the clinic for routine follow-up and a health supervision visit. The boy has had three exacerbations of asthma since his last visit 6 months ago. His mother has read on the Internet about an herbal supplement that can reduce exacerbations in children with asthma. She would like to start giving this herbal supplement to her son. She notes that a number of people have posted comments indicating that their own children had fewer asthma attacks when they took this supplement. Of the following, the BEST way to assess the efficacy of this herbal supplement in children with asthma is to perform a", "options" : "[\"cohort study\", \"cross-sectional study\", \"randomized controlled trial\", \"retrospective chart review\"]", "explanation" : "Designing clinical research requires a thoughtful approach to addressing the specific clinical question. Each specific type of research study design has advantages and disadvantages. The best way to assess the efficacy of the herbal supplement described in the vignette in children with asthma is to perform a randomized controlled trial.\n\nA randomized controlled trial is considered the reference standard of research design. In these studies, participants receive random assignment to either an experimental or a control group. As the study is performed, the only expected difference between the two groups is the outcome variable being studied. By randomly assigning patients to different treatment options, a randomized controlled trial limits population and investigator bias. To provide greater avoidance of bias, interventions can also be masked for both participant and researcher. Randomized controlled trials are expensive and require extensive time and effort to recruit and follow patients prospectively over time. Patients lost to follow-up often pose a challenge in randomized controlled trials.\n\nA cohort study is a prospective observational study that involves select members of a population who share a common condition or exposure and follows them over time. Cohort studies are performed to determine whether an initial exposure or risk factor is associated with the outcome measure. Randomization is not done in a cohort study; this may result in significant differences in the baseline characteristics within the cohorts. Because a cohort study follows patients over time, outcome measures cannot be obtained immediately and in some instances can take years to accomplish. For instance, a cohort study might be used to assess whether exposure to lead early in life affects intellectual performance in school-aged children. A cohort of children exposed to lead and another of children not so exposed would be followed over time and assessed for intellectual performance during the study period.\n\nA cross-sectional study evaluates the relationship between variables in a defined population at a single point in time (or limited short period). These studies are used to define the frequency of a condition in a population at a set point in time and are often used to assess the burden of a disease or health needs of a population. Cross-sectional studies are quick and easy to conduct. These studies focusing on a specific problem at a specific point in time can measure only prevalence and not incidence of a disease or condition. Risk factors and outcomes are measured simultaneously, and causation cannot be determined. A cross-sectional design might be used to assess the prevalence of asthma among children aged 6 to 10 years in a sample of US elementary schoolchildren.\n\nRetrospective studies use data that have previously been collected (such as information recorded in a medical record). In a retrospective study, the data usually were not originally collected as part of a research study. Retrospective chart review is a method of collecting data and can be used in a number of different study designs, including case-control studies. Retrospective studies are generally considered inferior to prospective studies but are inexpensive and easy to perform. Retrospective data are often used for preliminary studies or for the study of rare conditions. The results of retrospective studies frequently serve as the impetus for subsequent prospective studies.\n\nPREP Pearls\n• The specific research question determines the type of study design that should be used.\n• Randomized controlled trials are considered the reference standard for prospective analysis of a specific treatment or intervention.\n\nABP Content Specifications(s)\n• Understand the validity hierarchy for study design and study type\n• Understand the uses and limitations of controlled clinical trials\n• Understand the uses and limitations of randomized clinical trials\n\nSuggested Readings\n• Bothwell LE, Greene JA, Podolsky SH, Jones DS. Assessing the gold standard—lessons from the history of RCTs. N Engl J Med. 2016;374(22):2175-2181. doi:10.1056/NEJMms1604593.\n• Robbins BW. Evidence-based medicine. In: McInerny TK, Adam HM, Campbell DE, DeWitt TG, Foy JM, Kamat DM, eds. American Academy of Pediatrics Textbook of Pediatric Care. 2nd ed. Elk Grove Village, IL: American Academy of Pediatrics; 2017:29-32. Pediatric Care Online.\n• Sibbald B, Roland M. Understanding controlled trials. Why are randomised controlled trials important? BMJ. 1998;316(7126):201. doi:10.1136/bmj.316.7126.201."}
{"id" : 2263, "question_text" : "A 5-year-old child is brought to the office for bedwetting every night for the past 2 weeks. She has no frequency, urgency, or daytime urinary accidents. She was toilet trained, both day and night, at 3 years of age. Her parents believe she has a bowel movement every day; they do not observe her when she uses the bathroom. She sleeps deeply; her sleep is remarkable only for nightmares approximately twice per month. She recently started kindergarten. She has a 3-year-old brother and a newborn sister. The girl's weight is at the 50th percentile and height is at the 75th percentile. Her physical examination findings, including her abdomen, spine, and genitalia, are normal. A urine dipstick test performed in the office is negative. Of the following, the MOST likely cause of this child's condition is", "options" : "[\"bladder bowel dysfunction\", \"ectopic ureter\", \"psychosocial stressor\", \"sleep disordered breathing\"]", "explanation" : "The child described in the vignette has secondary nocturnal enuresis due to psychosocial stressors. She recently started kindergarten and has a newborn sibling; these events are likely contributors to her new-onset nocturnal enuresis.\nNocturnal enuresis (ie, leakage of urine during sleep) is classified as primary (never been dry at night) and secondary (dry period of 6 months or longer). Nocturnal enuresis can be further classified as monosymptomatic (without symptoms of lower urinary tract or bladder dysfunction) or nonmonosymptomatic (with symptoms of lower urinary tract or bladder dysfunction, anatomic abnormalities, or neurogenic bladder). The common conditions associated with nocturnal enuresis are shown in Table 1.\nA detailed history, including psychosocial stressors, is critical to the evaluation of nocturnal enuresis. The physical examination should include a genital, neurological, and oropharynx examination. The key history and physical examination items in the evaluation of nocturnal enuresis are shown in Table 2. Laboratory evaluation should include a complete urinalysis to evaluate for an underlying renal disease.\nThe child in the vignette has regular bowel movements and lacks urinary signs or symptoms suggestive of bladder bowel dysfunction. Ectopic ureter is an unlikely diagnosis as it usually presents with primary enuresis with continuous (day and night) leaking of urine from the vagina or perineum. The child in the vignette is a deep sleeper who has occasional nightmares, which can be normal at this age. Sleep apnea, snoring, or enlarged tonsils that could be suggestive of sleep disordered breathing are not present.\nTreatment of children with nocturnal enuresis includes management of any lower urinary tract dysfunction, constipation, underlying comorbid conditions, and psychosocial stressors. Once those issues have been addressed or ruled out, the best next steps include limiting fluid intake before bedtime, emptying the bladder before going to sleep, and waking the child to void before the parents go to bed. In children without lower urinary tract symptoms or bladder dysfunction, bedwetting alarms and desmopressin are effective treatment options.\nSuggested Reading(s)\nBayne AP, Skoog SJ. Nocturnal enuresis: an approach to assessment and treatment. Pediatr Rev. 2014;35(8);327-334. doi:10.1542/pir.35-8-327\nIorember FM. Enuresis. In: McInerny TK, Adam HM, Campbell DE, DeWitt TG, Foy JM, Kamat DM, eds. American Academy of Pediatrics Textbook of Pediatric Care. American Academy of Pediatrics; 2021:chap 249. Accessed September 21, 2024. Pediatric Care Online\nRoth EB, Austin PF. Evaluation and treatment of nonmonosymptomatic enuresis. Pediatr Rev. 2014;35(10);430-436. doi:10.1542/pir.35-10-430\nContent Domain\nRenal\nLearning Objectives\nRecognize clinical and laboratory findings associated with incontinence\nIdentify renal causes of nocturnal incontinence\nPlan the appropriate diagnostic evaluation and management of incontinence"}
{"id" : 2336, "question_text" : "A 12-year-old boy is seen in the office for evaluation of severe headaches that have occurred intermittently over the past month. His headaches are associated with blurry vision, heart palpitations, sweating, and facial flushing. There is no associated vomiting, photophobia, or phonophobia. The family history is notable for a grandparent who died of thyroid cancer and an uncle who had a cerebrovascular accident in his mid-30s. On physical examination, the boy's vital signs are a temperature of 37.1 °C, a blood pressure of 160/96 mm Hg, a heart rate of 105 beats/min, and an oxygen saturation of 100% in room air. His body mass index is at the 50th percentile for age. The remainder of his physical examination findings are unremarkable. Of the following, the BEST test to confirm this boy's diagnosis is", "options" : "[\"an aldosterone concentration\", \"fractionated metanephrine concentrations\", \"a midnight salivary cortisol concentration\", \"thyroid function testing\"]", "explanation" : "PREP Pearl(s)\nEpisodic or persistent hypertension associated with severe headaches, palpitations, and sweating should raise concern regarding pheochromocytoma.\nThe initial evaluation for suspected pheochromocytoma should include fractionated plasma metanephrine or 24-hour urinary metanephrine levels.\nMost children with pheochromocytoma have an associated genetic syndrome (eg, multiple endocrine neoplasia type 2, von Hippel-Lindau disease, neurofibromatosis type 1).\nCritique\nThe boy in the vignette has considerable hypertension (stage 2) documented in the office. The constellation of hypertension (the likely cause of his headaches and blurry vision), tachycardia, facial flushing, and palpitations is suggestive of catecholamine excess caused by a pheochromocytoma. The best test to confirm the diagnosis of pheochromocytoma is fractionated metanephrine concentrations. The boy's family history of thyroid cancer and stroke at a young age (which may have been caused by a hypertensive emergency) raises concern regarding an inherited condition, specifically multiple endocrine neoplasia type 2 (MEN2). Medullary thyroid cancer and pheochromocytoma are features of MEN2.\nConditions associated with aldosterone excess (eg, primary aldosteronism) cause hypertension owing to sodium and fluid retention. However, tachycardia, flushing, and sweating are not associated with these conditions. An elevated midnight salivary cortisol level would be consistent with Cushing syndrome, which can cause hypertension and facial flushing (plethora). However, Cushing syndrome has an indolent onset, is accompanied by weight gain and poor growth, and is not associated with palpitations and sweating. Hyperthyroidism can cause hypertension, tachycardia, palpitations, and sweating. However, in the context of the boy's findings and his family history, an inherited condition (eg, MEN2) is more likely.\nPheochromocytoma is a tumor of the adrenal medullary chromaffin cells, which produce catecholamines. These tumors may be unilateral or bilateral. They can produce any or all types of catecholamines, including epinephrine, norepinephrine, metanephrine, normetanephrine, and dopamine. Typical signs and symptoms of pheochromocytoma include episodic or chronic hypertension, tachycardia, severe headache, facial flushing, and sweating. The initial evaluation for suspected pheochromocytoma should include measurement of fractionated metanephrine concentrations (via 24-hour urine collection or spot plasma collection). The pattern of catecholamine elevation can inform the etiology of the tumor (eg, MEN2, neurofibromatosis type 1, von Hippel-Lindau disease).\nPheochromocytoma in a child is rare and often associated with a genetic syndrome. These syndromes must be considered in any child diagnosed with pheochromocytoma to avoid potential comorbidities. Multiple endocrine neoplasia type 2 is characterized by medullary thyroid cancer, pheochromocytoma, and hyperparathyroidism. It is caused by pathologic variants in the RET proto-oncogene and is inherited in an autosomal dominant fashion. Medullary thyroid carcinoma is universal in MEN2, so early detection of a RET pathologic variant is essential when there is a known family history of this condition. Pheochromocytomas caused by MEN2 typically secrete both metanephrine and normetanephrine.\nVon Hippel-Lindau disease is an autosomal dominant condition associated with multiple malignant and benign tumors, including hemangioblastomas of the retina and central nervous system, renal cell carcinoma, and pheochromocytoma. Pheochromocytomas associated with von Hippel-Lindau disease typically secrete normetanephrines, which distinguishes them from pheochromocytomas caused by MEN2.\nOther conditions associated with pheochromocytoma include succinate dehydrogenase deficiency (types B and D) and neurofibromatosis type 1. Pheochromocytomas associated with neurofibromatosis type 1 typically secrete metanephrines.\nSuggested Reading(s)\nSpeiser PW. Adrenal dysfunction. In: McInerny TK, Adam HM, Campbell DE, Foy JM, Kamat DM, eds. American Academy of Pediatrics Textbook of Pediatric Care. 2nd ed. American Academy of Pediatrics; 2016:chap 211. Accessed September 1, 2023. Pediatric Care Online\nWaguespack SG, Rich T, Grubbs E, et al. A current review of the etiology, diagnosis, and treatment of pediatric pheochromocytoma and paraganglioma. J Clin Endocrinol Metab. 2010;95(5):2023-2037. doi:10.1210/jc.2009-2830\nWeaver DJ. Hypertension in children and adolescents. Pediatr Rev. 2017;38(8):369-382. doi:10.1542/pir.2016-0106\nContent Domain\nEndocrinology\nABP Content Specification(s) / Content Area(s)\nRecognize the clinical findings associated with pheochromocytoma\nRecognize disorders commonly associated with pheochromocytoma\nThe correct answer is: fractionated metanephrine concentrations"}
{"id" : 1687, "question_text" : "A 12-year-old boy is brought to your office for a health supervision visit. His body mass index has dropped from the 50th to the 20th percentile. His mother reports that he has developed anxiety around eating and is not eating as much as he used to following an episode of food impaction several months ago. He was able to clear the impaction prior to evaluation in the emergency department. He reports no dysphagia, odynophagia, or emesis. He now takes longer than the entire family to eat and is drinking large volumes of water with meals. His medical history is positive for eczema and asthma. He has normal vital signs. He appears to be thin and is in no distress. He has no oral lesions. His abdomen is soft, nontender to palpation, and without mass. Of the following, the MOST likely diagnosis is", "options" : "[\"achalasia\", \"eosinophilic esophagitis\", \"esophageal stricture\", \"gastroesophageal reflux disease\", \"nutcracker esophagus\"]", "explanation" : "Correct Answer: B\nThe boy in this vignette has eosinophilic esophagitis. This diagnosis is based on his history of food impaction, slow eating with excessive chewing and flushing his food down with large volumes of liquids, and atopy (asthma and eczema). He reports no dysphagia or odynophagia, which would be expected with esophageal stricture, achalasia, and nutcracker esophagus. Children with gastroesophageal reflux are not likely to experience food impactions.\nEosinophilic esophagitis is an allergy/immune condition in which large numbers of eosinophils are found in the esophagus. This chronic condition is summarized in Item C163.\n\nPresentation can vary significantly but may include the following:\n• Gastroesophageal reflux that is unresponsive to acid blockade\n• Food refusal in infants and toddlers\n• Odynophagia\n• Chronic abdominal pain and vomiting in school-aged children\n• Dysphagia with food impactions in adolescents and adults\n• Prolonged chewing and food lubrication (excessive sauce use or liquid consumption with eating)\n• A history of asthma, food allergies, eczema, chronic rhinitis, or familial atopy in two-thirds of patients\n\nEosinophilic esophagitis can be a challenging diagnosis because of the broad differential diagnosis and overlap of symptoms. The gold standard for diagnosis is evaluation of esophageal biopsies. Untreated gastroesophageal reflux can appear very similar to eosinophilic esophagitis with eosinophils on biopsy. Therefore, children should be placed on proton pump inhibitors for a minimum of 6 weeks prior to endoscopy. In children with eosinophilic esophagitis, the esophagus often demonstrates longitudinal furrows, white plaques, and pallor. Biopsies should include both distal and proximal esophagus. Eosinophilic esophagitis is diagnosed if there are more than 15 eosinophils per high power field while on a proton pump inhibitor.\n\nPREP Pearls\n• Eosinophilic esophagitis is an allergy/immune condition in which large numbers of eosinophils are found in the esophagus.\n• Eosinophilic esophagitis typically presents in infants and toddlers with food refusal and in school-aged children with chronic abdominal pain and vomiting.\n• The prevalence of stricture in children with eosinophilic esophagitis is 5%.\n\nABP Content Specifications(s)\n• Recognize the clinical features associated with eosinophilic or allergic esophagitis\n\nSuggested Readings\n• Beausoleil JL, Brown-Whitehorn T. Allergic and atopic features of children with eosinophilic esophagitis. In: Liacouras CA, Markowitz JE, eds. Eosinophilic Esophagitis. New York, NY: Humana Press; 2012:239–252.\n• Markowitz JE, Liacouras CA. Eosinophilic esophagitis: treatment approach in children. In: Liacouras CA, Markowitz JE, eds. Eosinophilic Esophagitis. New York, NY: Humana Press; 2012:409–418.\n• Papadopoulou A, Koletzko S, Heuschkel R, et al. Management guidelines of eosinophilic esophagitis in childhood. J Pediatr Gastroenterol Nutr. 2014;58(1):107–118. doi: http://dx.doi.org/10.1097/MPG.0b013e3182a80be1.\n• Sorser SA, Barawi M, Hagglund K, Almojaned M, Lyons H. Eosinophilic esophagitis in children and adolescents: epidemiology, clinical presentation and seasonal variation. J Gastroenterol. 2013;48(1):81–85. doi: http://dx.doi.org/10.1007/s00535-012-0608-x.\n• Sutton AG, Mir M, Steiner MJ. Esophagitis: allergic and eosinophilic. Pediatr Rev. 2015;36(8):375–376. doi: http://dx.doi.org/10.1542/pir.36-8-375."}
{"id" : 1814, "question_text" : "A 20-month-old, previously healthy girl is preparing to go on a 5-week trip to Lagos, Nigeria, as part of an organized church group trip. Her mother is concerned about their risk of acquiring poliomyelitis in Nigeria. The child was born in the United States and received inactivated polio vaccination at 2, 4, and 15 months of age. Her mother was also born in the United States and received her complete polio vaccination series as a child. The girl's physical examination findings are normal. Of the following, the MOST appropriate management to minimize the risk of infection of both the child and mother is to administer inactivated polio vaccine in", "options" : "[\"1 dose to the child and none to the mother\", \"1 dose to the mother and none to the child\", \"1 dose to both the mother and child\", \"2 doses to both the mother and child (4 weeks apart)\"]", "explanation" : "Adults (≥18 years of age) who have been immunized against polio by completion of routine vaccine series of oral polio vaccine (OPV) or inactivated polio vaccine (IPV) are considered immune for life, but the precise duration of immunity is unknown. Fully immunized adults who intend to travel to, and remain in, a polio-endemic country for more than 4 weeks are at increased risk of exposure to wild poliovirus (WPV) or vaccine-derived poliovirus (VDPV), and should receive a dose of IPV before departure. Based on current data, no additional booster dose with IPV is recommended for adults. Unimmunized, incompletely immunized, or adult travelers with an unknown polio immunization status should receive 3 doses of IPV following the accelerated schedule and minimum intervals recommended by the Advisory Committee on Immunization Practices.\n\nRemarkable progress has been made in global polio eradication, with an over 99% estimated reduction in the number of polio cases since 1988, translating to more than 16 million people saved from paralytic disease. An estimated 350,000 cases occurred in more than 125 endemic countries in 1988 compared with 37 reported cases in 2016. Of the 3 serotypes of WPV (type 1, type 2, and type 3), type 1 poliovirus accounts for all recent polio cases caused by WPV. Eradication of WPV type 2 was certified in 1999 and the last case of WPV type 3 was reported from Nigeria in November 2012.\n\nIn the United States, the last case of indigenous acquisition of naturally occurring WPV was reported in 1979. All other cases of polio since 1986 have been vaccine-associated paralytic poliomyelitis (VAPP) in OPV recipients or their contacts. Introduction of IPV as a part of the immunization series in 1997 resulted in a steep decline in VAPP cases; in 2000, an all IPV-vaccine series was routinely implemented in the United States. There have been no reports of circulating WPV in the United States for many decades. In addition, with the recent success of the global polio eradication program, there is a very low risk of contact with imported wild-type polioviruses except in the setting of foreign travel to polio-endemic countries, such as Nigeria, Afghanistan, and Pakistan.\n\nIn the United States, a total of 4 doses of IPV at ages 2, 4, 6 to 18 months, and 4 to 6 years is currently recommended for all infants and children as part of a routine immunization series. The minimum recommended interval between IPV doses 1 and 2 and between doses 2 and 3 is 4 weeks, and the minimum interval between doses 3 and 4 is 6 months. The minimum age for dose 1 of IPV is 6 weeks. In situations of high-risk exposure, such as travel to a polio-endemic country, the minimum age and intervals should be used for administration of IPV. The fourth IPV dose should be given after age 4 years, irrespective of the previous number of doses administered, and at least 6 months after receiving the previous vaccine dose. Incompletely immunized children and adolescents should receive the complete schedule of IPV doses following the accelerated schedule and minimum intervals recommended by the American Academy of Pediatrics Committee on Infectious Diseases (https://redbook.solutions.aap.org/selfserve/ssPage.aspx?SelfServeContentId=Immunization_Schedules).\n\nThe following children and adolescents should receive an additional dose of IPV: (1) those who are up to date with their polio immunization or have completed the routine IPV series but traveling to a polio-endemic country and staying for more than 4 weeks, and (2) those who received their last IPV dose more than 12 months before the date they will be departing the country to which they are traveling. Children who receive this additional 4th IPV dose between 18 months through 4 years will still need an IPV booster dose at age 4 years or later.\n\nIn 2014, the World Health Organization (WHO) declared the international spread of WPV as a public health emergency of international concern. The risk of international spread is highest from countries with ongoing endemic circulation of WPV or VDPV, a polio outbreak, or evidence from an environmental source (eg, sewage) of WPV or VDPV circulation. In May 2017, the WHO issued updated guidance regarding the administration of polio vaccine. The Centers for Disease Control Travelers' Health website (www.cdc.gov/travel) and the Global Polio Eradication Initiative website (www.polioeradication.org/Dataandmonitoring/Poliothisweek.aspx) are good resources for up-to-date information regarding reported WPV and VDPV cases, travel notices, and vaccine recommendations. Clinicians should document all polio vaccinations on an International Certificate of Vaccination or Prophylaxis for long-term travelers and residents. The polio vaccine must be received between 4 weeks and 12 months before the date of departure from the polio-infected country to meet the departure requirement (wwwnc.cdc.gov/travel/news-announcements/polio-guidance-new-requirements(https://wwwnc.cdc.gov/travel/news-announcements/polio-guidance-new-requirements)).\n\nPREP Pearls\n\nIn the United States, a total of 4 doses of inactivated polio vaccine at ages 2, 4, and 6 to 18 months and 4 to 6 years is currently recommended for all infants and children.\n\nBefore traveling to polio-endemic areas, travelers should ensure that they have received the recommended age-appropriate polio immunization series.\n\nAdults who received a complete polio immunization series in childhood and intend to travel to and stay for more than 4 weeks in a polio-affected country should receive a single lifetime booster dose of IPV before departure.\n\nABP Content Specifications(s)/Content Area\n\nUnderstand the efficacy and safety of the poliovirus vaccine\n\nKnow the indications, contraindications, and schedules for the poliovirus vaccine, including under special circumstances (eg, unimmunized adult contacts)\n\nSuggested Readings\n\nAmerican Academy of Pediatrics. Poliovirus infections. In: Kimberlin DW, Brady MT, Jackson MA, Long SS, eds. Red Book: 2018 Report of the Committee on Infectious Diseases. 31st ed. Itasca, IL: American Academy of Pediatrics; 2018:657-664.\n\nOrenstein WA; Committee on Infectious Diseases. Eradicating polio: how the world's pediatricians can help stop this crippling illness forever. Pediatrics. 2015;135(1):196-202. doi: http://dx.doi.org/10.1542/peds.2014-3163.\n\nWorld Health Organization. Polio vaccines: WHO position paper, March 2016-recommendations. Vaccine. 2017;35(9):1197-1199. doi: http://dx.doi.org/10.1016/j.vaccine.2016.11.017."}
{"id" : 2019, "question_text" : "An 11-year-old girl presents with a 1-day history of nausea, abdominal cramping, watery diarrhea without blood or mucus, low-grade fever, malaise, and headache. Five days before presentation, she participated in a summer camp where she had contact with livestock, including preweaned calves, goats, and pigs. The campers also had access to a swimming pool and lake, and consumed meals prepared at a central kitchen. A garden provided lettuce and other produce for camp meals. Several other campers and staff members have become ill with diarrhea. The girl's temperature is 37°C, and her vital signs are normal. Her physical examination is only notable for evidence of mild dehydration. Of the following, the MOST likely cause of this girl's illness is", "options" : "[\"Bacillus cereus\", \"Brucella melitensis\", \"Cryptosporidium parvum\", \"enteropathogenic Escherichia coli\"]", "explanation" : "In the vignette, the outbreak of diarrheal illness among children and adults after visiting a summer camp where there was contact with livestock, access to a swimming pool and lake, and consumption of meals from produce grown in the local garden is highly suggestive of Cryptosporidium parvum infection. Bacillus cereus is an important toxin-mediated foodborne illness and has been linked to outbreaks of diarrheal illness, but the incubation period ranges from 10 to 16 hours. Enteropathogenic Escherichia coli causes acute and chronic watery diarrhea, primarily in children younger than 2 years of age, living in low and middle-income countries. Although Brucella melitensis, the etiologic agent of brucellosis, may be transmitted to humans via exposure to livestock, patients usually present with high fever, night sweats, and other systemic symptoms with hepatomegaly, splenomegaly, and arthritis on physical examination.\n\nCryptosporidium species are coccidian protozoal organisms comprising 2 species (Cryptosporidium hominis, which typically affects humans, and C parvum, which infects humans and animals). The infection is transmitted by the fecal-oral route after ingestion of oocysts excreted by infected hosts. Major water-associated outbreaks of cryptosporidiosis, well described in the United States, have resulted from exposure to contaminated public drinking water and treated (eg, swimming pools) or untreated (eg, lakes) recreational water locations. Person-to-person transmission and outbreaks in daycare centers, with secondary spread, have been reported. Zoonotic transmission can occur in farmers and animal handlers, as well as children via exposure to pets and animals in petting zoos, especially preweaned calves.\n\nCryptosporidiosis is characterized by nonbloody, watery diarrhea with abdominal pain, emesis, fever, loss of appetite, and weight loss. The typical incubation period is 3 to 14 days. Infection can be asymptomatic. In immunocompetent hosts, gastroenteritis is usually self-limited, with symptoms resolving by 2 to 3 weeks. Cryptosporidium infection can also result in traveler's diarrhea. In immunocompromised individuals (eg, those with human immunodeficiency virus infection, solid organ transplant recipients), severe, chronic diarrhea may persist for weeks to months, associated with weight loss and extraintestinal disease.\n\nLaboratory diagnosis of cryptosporidiosis can be made by detection of oocysts in stool by direct immunofluorescent antibody test or detection of antigen in stool by rapid point-of-care testing using enzyme immunoassay method. A modified Kinyoun acid-fast stain may detect the organism in a concentrated stool specimen. Because of the intermittent shedding of oocysts, a minimum of 3 stool specimens collected on separate days is recommended before excluding infection.\n\nAntiparasitic therapy may not be necessary in immunocompetent people with mild disease. Oral nitazoxanide administered for 3 days is the recommended therapy for all immunocompetent individuals aged 1 year and older. Immunocompromised children may require nitazoxanide therapy for 14 days or longer. Contact precautions for the duration of illness are recommended for hospitalized patients or during institutional outbreak.\n\nOther than Cryptosporidium species, frequently reported pathogens causing recreational water–associated outbreaks include Shigella, Giardia, norovirus, and Escherichia coli O157:H7. In 2011-2012, of 90 recreational water–associated outbreaks, 77% were linked to treated recreational water, with 52% due to Cryptosporidium. Twenty-three percent of outbreaks were linked to untreated recreational water, 33% of which were due to E coli O157:H7 or E coli O111. In the United States, there has been a rise in the incidence of cryptosporidiosis. Children aged 1 to 4 years are most commonly affected, followed by children aged 5 to 9 years. Seasonal peaks of cryptosporidiosis in late summer and early fall are related to the outdoor swimming season. Prevention of recreational water–associated illness warrants epidemiologic surveillance and outbreak detection, environmental hygiene maintenance, appropriate disinfection, and water quality controls. The public and staff at recreational water venues must be educated about healthy swimming behaviors to prevent the spread of recreational water–associated illness including (1) avoidance of swimming during diarrheal illness; (2) not swallowing pool water, and (3) adherence to strict hygiene measures.\n\nPREP Pearls\n\nCommon pathogens causing recreational water–associated outbreaks include Cryptosporidium, Shigella, Giardia, norovirus, and Escherichia coli O157:H7.\n\nCryptosporidium typically is transmitted via the fecal-oral route, but exposure to contaminated water, livestock, and animals in petting zoos can also result in infection.\n\nIn immunocompetent hosts, the diarrheal illness caused by Cryptosporidium species is self-limited, whereas severe, chronic diarrhea associated with weight loss occurs in immunocompromised hosts.\n\nABP Content Specifications(s)/Content Area\n\nUnderstand the epidemiology of Cryptosporidium infection\n\nRecognize the clinical features associated with Cryptosporidium infection, including Cryptosporidium diarrhea in an immunocompromised host\n\nSuggested Readings\n\nAmerican Academy of Pediatrics. Cryptosporidiosis. In: Kimberlin DW, Brady MT, Jackson MA, Long SS, eds. Red Book: 2018 Report of the Committee on Infectious Diseases. 31st ed. Itasca, IL: American Academy of Pediatrics; 2018:304-307.\n\nCenters for Disease Control and Prevention. Cryptosporidiosis surveillance --United States, 2009-2010. MMWR Surveill Summ. 2012;61(SS-5):1-12.\n\nCenters for Disease Control and Prevention. Recreational water-associated disease outbreaks --United States, 2009-2010. MMWR Morb Mortal Wkly Rep. 2014;63(1):6-10.\n\nCenters for Disease Control and Prevention. Steps of Healthy Swimming. https://www.cdc.gov/healthywater/swimming/swimmers/steps-healthy-swimming.html."}
{"id" : 805, "question_text" : "A 6-year-old boy is seen in the emergency department with fever and knee swelling of 1 day's duration. Physical examination reveals a warm, erythematous, swollen knee with decreased range of motion due to pain. His temperature is 40°C and heart rate is 110 beats/min; the remainder of his physical examination findings and vital signs are within normal limits. A complete blood cell count reveals a white blood cell count of 15,000/µL (15.0 x 109/L) and platelets of 412.0 x 109/µL (412.0 x 109/L). The erythrocyte sedimentation rate is 50 mm/h. Arthrocentesis reveals white synovial fluid, a white blood cell count of 80,000/µL (80.0 x 109/L), and a red blood cell count of 0.1 x 103/µL (0.1 x 109/L). Gram stain and culture results are pending. Of the following, the BEST antibiotic choice is", "options" : "[\"cefepime\", \"cefotaxime\", \"doxycycline\", \"nafcillin\", \"penicillin G\"]", "explanation" : "Preferred Response: D\nThe child in this vignette has pyogenic (septic) arthritis, which is a medical emergency. A delay in treatment can result in permanent joint damage. Local symptoms include joint pain, swelling, limp, and refusal to use the affected joint. Systemic features of pyogenic arthritis include fever, malaise, and fussiness. On physical examination, the patient will appear ill with a single warm, erythematous, painful joint with decreased and painful range of motion. Aspiration of joint fluid can help make the diagnosis. Synovial fluid white blood cell counts are often greater than 50,000/µL. Gram stain and culture of the synovial fluid usually yields a causative organism but can be negative in up to 50% of cases. Ultrasonography will reveal a joint effusion, and magnetic resonance imaging can reveal areas of infection and damage in the joint, bone, and adjacent soft tissue. Younger children may have coexisting osteomyelitis. Appropriate management includes evaluation by orthopedics for drainage and debridement. Antibiotic therapy should not be delayed. Treatment of pyogenic arthritis is determined by the likely causative organism, which varies with patient age (Item C98).\n\nThe best antibiotic choice in a child older than 5 years of age is nafcillin, since the infection is likely due to Staphylococcus aureus or Streptococcus pyogenes. Cefotaxime might be a good choice in a child younger than 5 years of age if Streptococcus pneumoniae is a potential pathogen, but it should only be used in combination with another antibiotic. Doxycycline would be a good choice for Brucella-induced pyogenic arthritis, which is very unlikely in this patient. Cefepime provides gram-negative coverage, and penicillin G provides group A Streptococcus coverage; therefore, neither would be a good choice for the likely organisms in this case.\n\nPREP Pearls\n• Pyogenic arthritis requires prompt treatment to prevent permanent joint damage.\n• Pyogenic arthritis should be suspected in any patient who has a single affected joint and fever.\n• The recommended antibiotic therapy for pyogenic arthritis varies with age and associated risk factors; naficillin is the best choice for a child greater than 5 years of age.\n\nAmerican Board of Pediatrics Content Specifications\n• Know the appropriate antibiotic management of pyogenic arthritis\n\nSuggested Reading:\n• Gutierrez K. Bone and joint infections in children. Pediatr Clin N Am. 2005;52(3):779-794. doi:10.1016/j.pc1.2005.02.005\n• John J Chandran L. Arthritis in children and adolescents. Pediatr Rev. 2011;32(11):470-480. doi:10.1542/pir.32-11-470"}
{"id" : 1113, "question_text" : "You are called to the neonatal intensive care unit to evaluate a 24-hour-old newborn with progressive obtundation, seizures, and tachypnea. Pregnancy, labor, and delivery were uneventful. The newborn initially did well during the first several hours after birth, but then became lethargic, hypothermic, and developed poor feeding. Results of a comprehensive metabolic panel including glucose, complete blood cell count with differential, and C-reactive protein are all normal. The anion gap is normal. Blood gas results reveal a respiratory alkalosis. Intravenous antibiotics are started and cultures are pending. Of the following, the BEST next laboratory test for diagnosis and management is", "options" : "[\"serum ammonia\", \"serum galactose-1-phosphate\", \"serum toxicology screen\", \"serum very long chain fatty acids\", \"urine organic acids\"]", "explanation" : "The neonate in the vignette has a classic presentation of a urea cycle disorder, with decompensation in the first 24 to 72 hours of life with progressive respiratory alkalosis, obtundation, and hyperammonemia, in the presence of a normal anion gap. Urea cycle disorders are caused by mutations resulting in the absence or partial functioning of 1 of the first 4 enzymes in the biochemical pathway responsible for the breakdown of nitrogen (the urea cycle). Urea is a waste byproduct of protein catabolism in the body. In a normal individual, nitrogen is broken down into urea that is excreted through the urine. If nitrogen levels build up in the body because of inefficient breakdown, it accumulates quickly in the form of ammonia. Hyperammonemia is very toxic to the brain and can cause irreversible damage without immediate intervention. Neonates will appear normal at birth, but within 24 to 72 hours, they will develop cerebral edema. This typically manifests as poor feeding, obtundation, hypothermia, seizures, hyperventilation, hyporeflexia, unusual posturing, and ultimately, coma. Classic laboratory findings include elevated ammonia levels (> 210 μg/dL [150 μmol/L]) with a normal anion gap and glucose level in the presence of respiratory alkalosis on blood gas measurement.\nTo distinguish between the specific types of urea cycle defects, one must order a plasma amino acid analysis and an urine orotic acid. Ornithine transcarbamylase deficiency (OTC) is associated with extremely high urinary orotic acid levels. Carbamoyl phosphate synthetase I (CPS I) deficiency is associated with low to undetectable urine orotic acid levels. Definitive diagnosis is dependent on either enzymatic analysis or molecular genetic testing of the genes involved. All of the urea cycle disorders are autosomal recessive, except OTC, which is transmitted by X-linked recessive inheritence. A family history of early infant death, presumably because of hyperammonemia, may also be seen. Specific urea cycle disorders include N-acetyl glutamate synthetase deficiency, carbamoyl phosphate synthetase I deficiency, OTC deficiency, citrullinemia type I, argininosuccinic aciduria, and arginase deficiency.\nUrea cycle disorders may present as a metabolic emergency, necessitating immediate recognition and treatment to avoid irreversible brain damage. Severe hyperammonemia is treated with dialysis and hemofiltration to rapidly reduce the plasma ammonia concentration, along with the intravenous administration of arginine hydrochloride and nitrogen scavenger drugs to promote the excretion of excess nitrogen through alternative pathways. Restriction of protein for 12 to 24 hours is essential, with calories provided through carbohydrates and fat. Care must be taken to stabilize the patient with intravenous fluids and inotropic drugs if necessary.\nLong term management mandates the use of specialized formulas, oral nitrogen-scavenging drugs, dietary restriction of protein, and avoidance of hyperammonemic episodes. Patients are at high risk of decompensation, necessitating hospitalization for close observation of clinical status and ammonia levels if they have gastrointestinal or respiratory illnesses. Most patients are routinely treated on a long term basis by biochemical or metabolic geneticists, in addition to their primary care provider. They should have emergency protocols in place at home, at the primary care provider's office, and at the local hospital.\nOrganic acidemias or organic acidurias are a group of disorders characterized by dysfunction of a specific step in amino acid catabolism, typically the result of a specific enzyme deficiency. Classic patterns of unusual excretion of non-amino organic acids in urine are a first line test for this group of disorders. Newborns appear well at delivery and for the first few days, followed by metabolic decompensation. Symptomatology includes vomiting, poor feeding, neurologic symptoms, and lethargy progressing to coma. Laboratory findings include metabolic acidosis (not respiratory alkalosis as in the child in this vignette), ketosis, hyperammonemia, elevated liver function tests, low blood sugar, and neutropenia. These disorders require immediate recognition to optimize long term outcome via clinical interventions.\nTyrosinemia type 1 presents in infancy with significant liver involvement, and eventually renal tubular dysfunction, growth failure, and rickets. Untreated children may present with repeated neurologic crises involving a change in mental status, peripheral neuropathy, abdominal pain, and occasionally respiratory failure. If untreated, many die before 10 years of age. Laboratory abnormalities include increased succinylacetone concentration in the blood and urine; elevated tyrosine, methionine, and phenylalanine on serum amino acids; and elevated tyrosine metabolites on urine organic acids.\nGalactosemia presents in the neonatal period with jaundice, hypotonia, scleral icterus, bruising, bleeding, and cataracts in the face of rapidly progressive liver failure. Classic laboratory abnormalities of galactosemia include positive urine-reducing substances, abnormal liver function studies, coagulation abnormalities suggestive of a progressive bleeding diathesis, elevated erythrocyte galactose-1-phosphate, and sepsis, especially due to Escherichia coli.\nPrenatal drug exposure is typically best assessed by a combination of a maternal interview, maternal hair analysis, and meconium drug testing. Withdrawal symptoms are dependent on the type of drugs abused by the mother. A history of placental abruption should be a red flag for potential cocaine exposure. Special attention should be focused on clinical signs including microcephaly, intrauterine growth retardation, prematurity, congenital malformations, and congenital infections. Neonatal abstinence syndrome can include signs of central nervous system dysfunction (high-pitched cry, restlessness, hyperreflexia, jitteriness, tremors, seizures), respiratory symptoms (nasal flaring, tachypnea, apnea), and gastrointestinal dysfunction (frantic rooting, poor feeding, vomiting, loose stools). The Finnegan scoring system is commonly used for assessing for signs of neonatal abstinence syndrome. Meconium drug analysis is currently the best method for assessing prenatal drug exposure in neonates, not a serum toxicology screen, as stated in the answers in the vignette. Babies with drug withdrawal may be obtunded, but they will not have hyperammonemia.\nPeroxisomal biogenesis disorders are typically screened with serum very long chain fatty acids. They can present in the neonatal period with hypotonia, poor feeding, dysmorphic facies, seizures, and liver cysts with hepatic dysfunction. Bony stippling may occur in the patella or long bones detectable by skeletal survey. Infants do not present with metabolic crises, but with slowly progressive neurologic deterioration, dying in the first year of life.\nPREP Pearls\n Urea cycle disorders clinically manifest with immediate decompensation in the first 24 to 72 hours of life with progressive respiratory alkalosis, obtundation, and hyperammonemia in the presence of a normal anion gap.\n Hyperammonemia is very toxic to the brain and can cause irreversible damage without immediate intervention. It is very important to check the serum ammonia level and and quickly implementation of measures to decrease the ammonia level as soon as possible (dialysis, hemofiltration, nitrogen scavenger medications, and protein restriction).\nABP Content Specifications(s)\n Plan the appropriate immediate and long-term management of urea cycle defects, while considering the long-term prognosis"}
{"id" : 3067, "question_text" : "You are caring for a 10-year-old boy who suffered a severe traumatic brain injury from a motor vehicle accident 3 days ago. He does not have trauma to any other organ system. His elevated intracranial pressure is currently being managed with mild hyperventilation, mannitol, sedation, and continuous cerebrospinal fluid drainage. Medications include fentanyl continuous infusion, cefazolin, ranitidine, and mannitol. He is receiving 30 kcal/oz formula at 50 mL/h by nasogastric tube. He has a temperature of 37°C, heart rate of 80 beats/ min, blood pressure of 120/70 mm Hg, respiratory rate of 20 breaths/min, and oxygen saturation of 98% on the ventilator. His weight is 30 kg. On physical examination, he is intubated and sedated. He does not open his eyes and withdraws all extremities equally upon painful stimuli. Pupils are 4 mm, equal, and sluggishly reactive. Mucous membranes are moist, and he has periorbital and soft tissue edema. His pulmonary and cardiovascular examinations are unremarkable. Over the last 24 hours, fluid intake was 1,200 mL of feeds and 300 mL of medications and other fluids to maintain catheter patency. Fluid output includes 500 mL of urine and 100 mL of cerebrospinal fluid drainage. Serum chemistry results include: Sodium, 125 mEq/L (125 mmol/L); Potassium, 4 mEq/L (4 mmol/L); Chloride, 90 mEq/L (90 mmol/L); Carbon dioxide, 24 mEq/L (24 mmol/L); Blood urea nitrogen, 20 mg/dL (7.1 mmol/L); Creatinine, 0.6 mg/dL (53 umol/L); Osmolality, 270 mOsm/kg (270 mmol/kg). Urine chemistry results include: Sodium, 50 mEq/L (50 mmol/L); Creatinine, 12 mg/dL (1061 µmol/L); Urine urea nitrogen, 6 mg/dL (2.1 mmol/L). The patient's urine specific gravity is 1.020. Of the following, the MOST likely physiologic mechanism for his hyponatremia is", "options" : "[\"adrenal insufficiency\", \"excessive atrial natriuretic peptide secretion\", \"excessive renal sodium loss\", \"inadequate sodium intake\", \"insufficient renal water excretion\"]", "explanation" : "Preferred Response: E\nThe child in the vignette has the syndrome of inappropriate antidiuretic hormone (SIADH) evidenced by hyponatremia, decreased urine output, a state of euvolemia or hypervolemia, a fractional excretion of sodium (FENa) of 2%, and history of traumatic brain injury. Syndrome of inappropriate antidiuretic hormone causes increased water reabsorption from the renal collecting duct, and therefore decreased water excretion.\n\nArginine vasopressin (AVP), the antidiuretic hormone (ADH) of humans, is synthesized in the hypothalamus as a response to high serum osmolality and volume depletion. In addition to other functions, AVP activates the V2 receptors in the distal nephron, which results in the insertion of the aquaporin channels in the collecting duct. This renders it more permeable to water, which leads to more water reabsorption, increased plasma volume, decreased serum osmolality, and decreased urine volume. Under normal conditions, AVP secretion is inhibited by low serum osmolality and increased intravascular volume. In SIADH, AVP secretion is not inhibited, and the patient is unable to excrete water sufficiently. Syndrome of inappropriate antidiuretic hormone (Item C23A) is usually caused by central nervous system (CNS) disorders, such as trauma, infection, tumor, or hemorrhage, or pulmonary disorders, such as pneumonia, positive pressure ventilation, and asthma. Several medications (ie, some sedatives and neuroleptics) can stimulate ADH release, and others (ie, oxytocin and vasopressin analogs) can potentiate AVP action.\n\nItem C23A: Causes of Syndrome of Inappropriate Antidiuretic Hormone\nCentral Nervous System Disorders\n• Infection: meningitis, encephalitis\n• Neoplasms\n• Vascular abnormalities\n• Psychosis\n• Hydrocephalus\n• Postpituitary surgery\nPulmonary Disorders\n• Pneumonia\n• Tuberculosis\n• Asthma\n• Positive pressure ventilation\n• Pneumothorax\nCarcinomas\n• Bronchogenic carcinomas\n• Oat cell of the lung\n• Duodenal\n• Pancreatic\n• Neuroblastoma\nMedications\n• Vincristine\n• Intravenous cyclophosphamide\n• Carbamazepine\n• Serotonin reuptake inhibitors\n\nReprinted with permission from Moritz ML, Ayus IC. Disorders in water metabolism in children: hyponatremia and hypernatremia. Pedlar Rev. 2002;23:371\n\nComplications from SIADH generally include adverse effects of hyponatremia and hypo-osmolality. In a patient with head trauma, intracranial tumor, or meningitis, the neuronal swelling from hyponatremia can lead to seizures, cerebral edema, and elevated intracranial pressures, and in some cases, even death.\n\nAs described before, SIADH constitutes an inability to excrete water, leading to decreased serum osmolality. In this setting, a net negative free water balance can be achieved if free water intake is less than the output (urine output plus insensible water loss), which will raise the osmolality. Thus, the mainstay of SIADH treatment is fluid restriction to approximately half of usual maintenance fluid requirements. Furosemide has also been used to facilitate free water excretion. If mental status deterioration, signs of impending herniation, or seizures occur, the hyponatremia should be emergently treated with hypertonic (3%) saline. However, because irreversible neurologic damage can occur with rapid correction of hyponatremia, the rate of correction should not exceed 10 to 12 mEq/L in a 24-hour period.\n\nAdrenal insufficiency can occur after traumatic brain injury and can cause hyponatremia. Cases that are significant enough to cause hyponatremia are often associated with hyperkalemia, non-anion gap metabolic acidosis, hemodynamic instability, or hypoglycemia, none of which was seen in the patient in the vignette. Excessive renal sodium loss can occur in CNS insults in the form of cerebral salt wasting. However, urine sodium levels would be higher than seen in the vignette. Excessive atrial natriuretic peptide secretion does not typically occur in head trauma, and will also be associated with increased urinary losses of both sodium and water. Decreased sodium intake is not likely in this boy who was well and taking a normal diet before the head injury. In addition, derangements in serum sodium levels usually result from imbalances between intake and output of water, as opposed to intake of sodium (Item C23B).\n\nItem C23B: Disorders of Impaired Renal Water Excretion\nEffective Circulating Volume Depletion\n• Gastrointestinal losses: vomiting, diarrhea\n• Skin losses: cystic fibrosis\n• Renal losses: salt-wasting nephropathy, diuretics, cerebral salt wasting, hypoaldosteronism\n• Edematous states: heart failure, cirrhosis, nephrosis, hypoalbuminemia\nThiazide Diuretics\nRenal Failure\n• Acute\n• Chronic\nNon hypovolemic States of Antidiuretic Hormone Excess\n• Syndrome of inappropriate secretion of antidiuretic hormone\n• Cortisol deficiency\n• Hypothyroidism\n\nReprinted with permission from Moritz ML, Ayus H. Disorders in water metabolism in children: hyponatremia and hypernatremia. Pediarr Rev. 2002;23:371\n\nSodium levels and fluid intake and output should be monitored in children with traumatic brain injury. Symptomatic hyponatremia resulting from SIADH and other conditions can be life-threatening and should be treated without delay. Once symptoms have resolved, correction should not exceed 10 to 12 mEq/L per 24 hours. Syndrome of inappropriate antidiuretic hormone frequently can be treated with fluid restriction and possibly diuretics.\n\nPREP Pearls\n• Disorders of sodium are usually from imbalances between intake and output of free water.\n• Syndrome of inappropriate antidiuretic hormone (SIADH) can be commonly seen in central nervous system disorders (trauma, infection, and tumor) and pulmonary disorders (pneumonia, asthma, pleural effusion).\n• The mainstay of treatment of SIADH is fluid restriction.\n\nAmerican Board of Pediatrics Content Specification(s)\n• Recognize the role of head trauma in the development of SIADH\n\nSuggested Reading\n• Greenbaum LA. Sodium. In: Kliegman RM, Stanton BE, St. Geme 1W Schor NE, Behrman RE, eds. Nelson's Textbook of Pediatrics. 19th ed. Philadelphia. PA: Saunders Elsevier; 2011:212-218.\n• john CA, Day MW. Central neurogenic diabetes insipidus, syndrome of inappropriate secretion of antidiuretic hormone, and cerebral salt-wasting syndrome in traumatic brain injury. Crit Care Nurse. 20 /2;32(2):e1-7. doi:10.4037/ccri2012904.\n• Moritz ML, Ayus IC. Disorders in water metabolism in children: hyponatremia and hypernatremia. Pediatr Rev. 2002:23(10:371-380. doi:10.1542/pir.23-11-371."}
{"id" : 1359, "question_text" : "A 3-month-old female infant presents to the emergency department with vomiting, decreased activity, and poor weight gain. She was born to a 36-year-old gravida 2, para 1 woman at 34 weeks of gestation via cesarean delivery because of a breech presentation. The pregnancy was complicated by gestational diabetes and maternal seizure disorder. On physical examination, the infant is fussy. Her weight is 4.1 kg (birthweight of 2.9 kg), temperature is 37.3°C, heart rate is 130 beats/min, and respiratory rate is 30 breaths/min. She has decreased skin turgor and dry mucous membranes. Her examination is otherwise unremarkable. Laboratory tests are shown: Laboratory Test Patient Result Serum Results Sodium 129 mEq/L (129 mmol/L) Potassium 3.2 mEq/L (3.2 mmol/L) Chloride 82 mEq/L (82 mmol/L) Bicarbonate 41 mEq/L (41 mmol/L) Blood urea nitrogen 27 mg/dL (9.6 mmol/L) Creatinine 0.4 mg/dL (35 μmol/L) Glucose 98 mg/dL (5.4 mmol/L) Calcium 9.6 mg/ dL (2.4 mmol/L) Phosphorus 5.2 mg/dL (1.68 mmol/L) Urine Results Sodium 41 mEq/L (41 mmol/L) Potassium 114 mEq/L (114 mmol/L) Chloride 80 mEq/L (80 mmol/L) Of the following, the MOST likely diagnosis in the patient is", "options" : "[\"Bartter syndrome\", \"cystic fibrosis\", \"distal renal tubular acidosis (RTA)\", \"proximal RTA\", \"pyloric stenosis\"]", "explanation" : "The infant in the vignette presents with inadequate weight gain, metabolic alkalosis, hypokalemia, hyponatremia, and hypochloremia. Among the response choices, her most likely diagnosis is Bartter syndrome.\n\nHypochloremia in clinical settings is usually associated with metabolic alkalosis resulting from chloride loss associated with gastrointestinal or renal losses. In patients with hypochloremia and metabolic alkalosis, chloride depletion contributes to persistent alkalosis. Evaluation and treatment for the underlying metabolic alkalosis is the preferred approach for patients with hypochloremia.\n\nThe underlying cause of metabolic alkalosis is not usually apparent based on a patient's presenting history and physical examination. Urine chloride measurement is helpful in identifying intravascular volume status and thereby the underlying etiology of metabolic alkalosis. The renal response to decreased effective circulating volume (dehydration) is to increase reabsorption of salt (sodium chloride) and water, thereby increasing effective circulatory volume. This leads to low urinary sodium and chloride (< 20 mEq/L [20 mmol/L]) concentrations. Urinary chloride is a better indicator of volume status than urinary sodium in metabolic alkalosis because sodium is the cation binding to the increased bicarbonate filtered into the tubular fluid. Thus, urinary chloride is appropriately low (< 20 mEq/L) in patients with volume contraction compared with urinary sodium, which may be increased in response to increased tubular bicarbonate. Identification of hypovolemia (low urinary chloride) versus volume repletion (urinary chloride > 40 mEq/L [40 mmol/L]) helps in identifying the underlying etiology of metabolic alkalosis.\n\nRenal tubular disorders with sodium chloride wasting present with metabolic alkalosis in association with high urinary chloride levels (> 20–40 mEq/L). Both Bartter syndrome and Gitelman syndrome are characterized by hypokalemia and metabolic alkalosis. Bartter syndrome results from a primary defect in sodium chloride reabsorption in the medullary thick ascending limb of the loop of Henle, similar to the effect of chronic furosemide therapy. Bartter syndrome often presents in childhood with growth restriction, hypokalemia, metabolic alkalosis, and polyuria or polydipsia. Gitelman syndrome results from mutations in the gene coding for the thiazide-sensitive sodium chloride transporter in the distal tubule. Gitelman syndrome generally presents in late childhood or adulthood with muscle cramps (hypokalemia), polyuria, and/or polydipsia. In contrast to Bartter syndrome, patients with Gitelman syndrome have reduced urinary calcium and hypomagnesemia (more common). Children with either Bartter or Gitelman syndrome will be volume depleted because of excessive salt and water losses secondary to the underlying renal tubular defects. This volume depletion accounts for the compensatory hyperreninemia and hyperaldosteronism seen in these patients.\n\nPatients with mineralocorticoid excess, such as primary aldosteronism, Liddle syndrome, apparent mineralocorticoid excess syndrome, or licorice ingestion (glycyrrhizic acid) typically present with hypertension, hypokalemia, and metabolic alkalosis (with high urinary chloride). Chronic therapy with loop or thiazide diuretics leads to volume depletion (contraction) and metabolic alkalosis associated with a high urinary chloride level. In patients with diuretic abuse, the urinary chloride may vary from low to high depending on diuretic use.\n\nExcessive loss of gastric secretions leading to loss of hydrochloric acid, as seen with vomiting, pyloric stenosis, or continuous nasogastric suctioning may present with metabolic alkalosis, volume depletion, and a low urinary chloride level (<20 mEq/L). Metabolic alkalosis with low urinary chloride level (< 20 mEq/L) is also seen in patients with laxative abuse, cystic fibrosis with loss of chloride-rich sweat, and congenital chloride diarrhea.\n\nThe diagnosis of renal tubular acidosis (RTA) should be considered in a young infant with failure to thrive, recurrent vomiting, rickets, episodes of dehydration, recurrent nephrolithiasis, and persistent metabolic acidosis and hypokalemia. Renal tubular acidosis is an inherited or acquired defect in the ability of the kidneys to either absorb filtered bicarbonate or excrete ammonia, and is characterized by a normal anion gap metabolic acidosis. There are 4 forms of RTA: distal or type 1, proximal or type 2, mixed or type 3 (with features of both type 1 and 2), and hypoaldosteronism or type 4. Distal RTA is associated with failure to thrive, polyuria, hypokalemia, and medullary nephrocalcinosis (due to hypercalciuria and hypocitraturia). Generalized proximal tubular dysfunction is seen in patients with Fanconi syndrome. In addition to metabolic acidosis and hypokalemia, Fanconi syndrome is associated with rickets (phosphaturia leading to hypophosphatemic rickets), dipstick-positive glucosuria with normal plasma glucose concentration, and aminoaciduria/tubular proteinuria (urine dipstick-negative for protein and quantitative urine tests positive for amino acids and protein).\n\nPREP Pearls\n• Hypochloremia is usually seen in association with metabolic alkalosis.\n• Urine chloride measurement is helpful in assessing intravascular volume status, and thereby the underlying etiology of metabolic alkalosis.\n• Mineralocorticoid excess, or renal tubular disorders with sodium chloride wasting (Bartter and Gitelman syndrome), present with metabolic alkalosis in association with high urinary chloride levels (> 20–40 mEq/L [20–40 mmol/L]).\n• Bartter syndrome is caused by a primary defect in sodium chloride reabsorption in the medullary thick ascending limb of the loop of Henle, as seen in chronic furosemide therapy.\n• Metabolic alkalosis with low urinary chloride (< 20 mEq/L) is seen in patients with gastrointestinal losses, laxative abuse, cystic fibrosis with loss of chloride-rich sweat, and congenital chloride diarrhea.\n\nABP Content Specifications(s)\n• Recognize the various etiologies of hypochloremia\n\nSuggested Readings\n• Jaffe AC. Failure to thrive: current clinical concepts. Pediatr Rev. 2011;32(3):100-108. doi: http://dx.doi.org/10.1542/pir.32-3-100.\n• Kallen RJ. Renal tubular acidosis. In: McInerny TK, Adam HM, Campbell DE, DeWitt TG, Foy JM, Kamat DM, eds, American Academy of Pediatrics Textbook of Pediatric Care. 2nd ed. Elk Grove Village, IL: American Academy of Pediatrics; 2017:2554-2571.\n• Schwaderer AL, Schwartz GJ. Back to basics: acidosis and alkalosis. Pediatr Rev. 2004;25(10):350-357. http://pedsinreview.aappublications.org/content/25/10/350.extract."}
{"id" : 3207, "question_text" : "A 7-year-old girl with a seizure disorder has been taking valproate for 1 year. She has not had any seizures during that time, nor any adverse effects from the medication. She is growing and developing well. Her mother brought the girl to your office because she has been unusually sleepy. There was no trauma and no known ingestions, and the parents have been supervising her medications closely. She has had no recent illness. You recommended checking a valproate level and liver enzymes, and now have the following results: • Valproate level, 61.00 ug/mL (423 µmol/L); reference range, 50 ug/mL to 120 ug/mL (347-832 umol/L) • Alanine aminotransferase, 34 U/L • Aspartate aminotransferase, 41 U/L Of the following, the test MOST likely to reveal the cause of her sleepiness is", "options" : "[\"blood urea nitrogen\", \"complete blood cell count\", \"free and total carnitine\", \"repeat valproate level\", \"serum ammonia\"]", "explanation" : "Preferred Response: E\nValproate can cause hyperammonemic encephalopathy without transaminitis. Of the choices listed, ammonia is the most likely to be abnormal and causing the sleepiness in the girl in the vignette. It is also possible that she is having sub-clinical seizures, also called nonconvulsive status epilepticus. If the parents have a medication to stop seizures at home, such as rectal diazepam, they should try this. If this does not cause her mental status to improve, she should be evaluated in the emergency department for other causes of encephalopathy such as infection or electrolyte abnormality. For hyperammonemia caused by valproate, the dose of valproate will need to be reduced or the medication may need to be discontinued altogether. The girl's neurologist should be consulted to help manage her seizures in this setting.\n\nValproate can cause neutropenia and thrombocytopenia, but these do not typically present with sleepiness, so a complete blood count with differential is not the best choice. Renal dysfunction is not likely to occur in this patient with a normal valproate level. Checking levels of free and total carnitine would not be helpful to find a cause of sleepiness. Although valproate has been associated with low levels of free and total carnitine, it is unclear whether this causes any symptoms in children and whether giving carnitine is indicated. The valproate level was within the normal range, so it is unlikely that this is causing the girl's sleepiness; rechecking the level is unlikely to aid in diagnosis.\n\nPREP Pearls\n• Valproate can cause hyperammonemic encephalopathy without transaminitis.\n• Valproate can cause neutropenia and thrombocytopenia.\n\nAmerican Board of Pediatrics Content Specification(s)\n• Recognize side effects and toxicities associated with anticonvulsant drugs\n• Recognize laboratory abnormalities associated with anticonvulsant drug therapy\n\nSuggested Reading\n• Lexicomp. Valproate: pediatric drug information. UpToDate. Available online only for subscription."}
{"id" : 3626, "question_text" : "A 13-year-old previously healthy adolescent girl is admitted to the hospital after 2 days of diffuse abdominal pain, nausea, and vomiting, followed by progressive fatigue, labored breathing, and altered mental status. She was in her usual state of good health prior to 2 days ago. There is no reported trauma. Her caregiver reports that she has no access to alcohol or illicit drugs. The only medications in the home are cold medicine, ibuprofen, and acetaminophen. The patient has not had weight loss, fevers, headaches, visual disturbances, sore throat, upper respiratory symptoms, changes in stool, or urinary symptoms. She has a temperature of 37°C, heart rate of 70 beats/min, respiratory rate of 24 breaths/min, and blood pressure of 100/35 mm Hg. Oxygen saturation is 100% on room air. She is confused, speaking in sentences without being oriented to the conversation, and following commands inconsistently. Her neck is supple, and her mucous membranes are moist with no petechiae. Her pupils are 3 mm, equal, and reactive. Deep tendon reflexes are brisk, and cough and gag reflexes are intact. She is breathing fast and deeply, and her lungs are clear to auscultation bilaterally. Her heart has a regular rate and rhythm, and she is warm and well perfused, with a capillary refill time of 1 second. Her abdomen is soft with her liver 2 cm below the costal margin. Laboratory data are shown:\n\nLaboratory Test | Result\nSodium | 135 mEq/L (mmol/L)\nPotassium | 4.0 mEq/L (4.0 mmol/L)\nBlood urea nitrogen | 20 mg/dL (7.1 mmol/L)\nCreatinine | 1.1 mg/dL (84 µmol/L)\nWhite blood cell count | 25,000/µL (25 × 109/L)\nHemoglobin | 12 g/dL (120 g/L)\nPlatelet count | 150 × 103/µL (150 × 109/L)\nAspartate aminotransferase | 2,500 U/L\nAlanine aminotransferase | 2,000 U/L\nPartial thromboplastin time | 44.0 s\nProthrombin time | 52.6 s\nInternational normalized ratio (INR) | 4.5\n\nOf the following, the MOST likely cause of her altered mental status is", "options" : "[\"anticholinergic toxidrome\", \"bacterial meningitis\", \"fulminant hepatic failure\", \"septic shock\"]", "explanation" : "Correct Answer: C\nThe patient in this vignette has acute onset of abdominal pain, altered mental status, and evidence of liver damage and hepatic dysfunction. The most likely cause of her altered mental status is fulminant hepatic failure.\n\nIn a patient without a previous history of chronic liver disease, the diagnosis of acute liver failure (ALF) requires elevated levels of hepatic transaminases (aspartate aminotransferase, alanine aminotransferase), hyperbilirubinemia, coagulopathy not corrected by vitamin K, and encephalopathy. If there is severe coagulopathy (international normalized ratio [INR] > 2), encephalopathy is not required to make the diagnosis. Acute liver failure can present with mild and nonspecific signs such as malaise, vomiting, diffuse abdominal pain, fatigue, and jaundice, or it can be rapidly progressive and fatal, leading to cerebral edema, respiratory failure, renal failure, coagulopathy, and shock. There is no treatment for some etiologies of ALF other than supportive care. It is important to make a timely diagnosis of ALF and an emergent referral to a liver transplant center.\n\nThe etiology of ALF in children varies by age. It is idiopathic in greater than 50% of children across all age groups. Infectious causes are also common. Herpes simplex virus, Epstein-Barr virus, adenovirus, and parvovirus are the most common infectious agents leading to ALF across all age groups. Hepatitis A, B, and C do not commonly cause ALF. Drugs and toxins are responsible for about 20% of cases of ALF in children, with acetaminophen being the most common. Other medications causing ALF include phenytoin, valproic acid, isoniazid, volatile inhalational anesthetics, and chemotherapeutic agents. Approximately 10% of cases of ALF in children are due to metabolic diseases including galactosemia, tyrosinemia, urea cycle defects, and mitochondrial disorders in infants and Wilson disease in older children. Autoimmune hepatitis can be a cause of both acute and chronic liver failure, especially in adolescents, accounting for approximately 6% of all pediatric liver failure. Disorders of perfusion including acquired or congenital cardiovascular disease, hypoxia, or Budd-Chiari syndrome can also cause ALF in all age groups.\n\nAlthough hepatic transaminases are often referred to as \"liver function tests\" it is important to know that these are markers of hepatocellular damage (not liver function per se). Functions of the liver include synthetic, detoxifying, exocrine, and excretory. Major hepatocellular synthetic functions include gluconeogenesis, the manufacture of clotting factors II, VII, IX, and X, and protein synthesis. Hepatocellular synthetic dysfunction causes coagulopathy, hypoalbuminemia, edema, hypoglycemia, and hemodynamic changes. Detoxification functions of the liver include systems of glucuronidation, reduction of oxidants, and the cytochrome P450 systems, which clear the body of drugs, toxins, depressant neurotransmitters, ammonia, and hormones. Defects in these systems in ALF cause altered mental status, cerebral edema, increased drug and toxin effects, and vasodilation. The liver also conjugates and excretes bilirubin; thus, acute and chronic liver failure cause jaundice.\n\nLiver failure often presents with mild, vague concerns such as nausea, diffuse abdominal pain, and fatigue. Signs of chronic liver failure or ALF occurring over several weeks include firm hepatomegaly, splenomegaly, jaundice, palmar erythema, and \"spider\" angiomas. However, these signs can be absent in fulminant hepatic failure, in which a child can appear relatively well, followed by a rapid deterioration manifesting with cerebral edema, circulatory collapse, and coagulopathy. Thus, it is critical to make an early diagnosis. A pediatric gastroenterologist and/or hepatologist should be emergently consulted. Any child with encephalopathy, coagulopathy, and elevated hepatic transaminase levels should be transferred to a pediatric liver transplant center due to the risk of rapid progression. Supportive care may be necessary for cerebral edema, coagulopathy, hypoglycemia, and circulatory failure. Etiology-specific treatments for ALF may be possible, such as n-acetylcysteine for acetaminophen toxicity, steroids for autoimmune hepatitis, and other therapies for metabolic disorders.\n\nAnticholinergic toxidrome can cause altered mental status and liver failure from a medication effect, but it would also likely cause tachycardia, dilated pupils, and hyperthermia. Bacterial meningitis is not likely for the patient in this vignette, because fever, neck stiffness, and headache are absent. Septic shock can also cause altered mental status, hepatocellular damage, and consumptive coagulopathy and is often on the differential diagnosis along with ALF. However, it is not as likely as ALF for the patient in this vignette, because her hemodynamics would more likely be compromised.\n\nPREP Pearls\n• In a patient without a previous history of chronic liver disease, the diagnosis of acute liver failure requires elevated hepatic transaminases (aspartate aminotransferase, alanine aminotransferase), hyperbilirubinemia, coagulopathy not corrected by vitamin K, and encephalopathy.\n• Acute liver failure can present with mild and nonspecific signs such as malaise, vomiting, diffuse abdominal pain, fatigue, and jaundice, or it can be rapidly progressive and fatal, leading to cerebral edema, respiratory failure, renal failure, coagulopathy, and shock.\n• An emergent referral to a liver transplant center is important in cases of acute liver failure.\n\nABP Content Specifications(s)\n• Recognize the signs and symptoms of impending hepatic failure\n\nSuggested Readings\n• Bhatia V, Bavdekar A, Yachha SK; Indian Academy of Pediatrics. Management of acute liver failure in infants and children: consensus statement of the Pediatric Gastroenterology Chapter, Indian Academy of Pediatrics. Indian Pediatr. 2013;50(5):477-482. https://indianpediatrics.net/may2013/may-477-482.htm.\n• Hardikar W, Schwarz KB. Hepatitis. In: McInerny TK, Adam HM, Campbell DE, DeWitt TG, Foy JM, Kamat DM, eds. American Academy of Pediatrics Textbook of Pediatric Care. 2nd ed. Elk Grove Village, IL: American Academy of Pediatrics; 2017:2131-2144. Pediatric Care Online.\n• James LP, Alonso EM, Hynan LS, et al. Detection of acetaminophen protein adducts in children with acute liver failure of indeterminate cause. Pediatrics. 2006;118(3):e676-e681. doi:10.1542/peds.2006-0069.\n• Leonis MA, Alonso EM, Im K, Belle SH, Squires RH; Pediatric Acute Liver Failure Study Group. Chronic acetaminophen exposure in pediatric acute liver failure. Pediatrics. 2013;131(3):e740-e746. doi:10.1542/peds.2011-3035.\n• Newland CD. Acute liver failure. Pediatr Ann. 2016;45(12):e433-e438. doi:10.3928/19382359-20161128-01."}
{"id" : 1542, "question_text" : "A 4-year-old boy is seen in your office for follow-up after he was evaluated and treated at a local emergency department yesterday for right scrotal pain. Two days ago, the boy began to complain of pain in his right scrotum. At that time, his right scrotum appeared swollen and red to his mother, so she took him to a local emergency department for evaluation. Results available from his emergency department visit yesterday include a urinalysis that was normal, and a urine culture that has yielded no bacterial growth. The child also underwent color Doppler ultrasonography of his scrotum, which revealed slight enlargement of his right epididymis, with increased blood flow to both his right epididymis and right testis. The emergency department physician advised the boy's family that his scrotal pain was due to acute epididymitis. The boy's medical history includes mild intermittent asthma, with no other documented medical conditions. His asthma has been relatively well-controlled, and he has no history of hospitalizations. Last week, he had an acute asthma exacerbation that seemed to have been triggered by an upper respiratory viral infection. His only current medication is albuterol, which he takes as needed for acute asthma symptoms. He has had no recent fevers, dysuria, increased urinary frequency, or urethral discharge. He is currently attending preschool and has been doing well according to his mother. In your office today, the child appears well and in no distress. He is afebrile, and his vital signs are within normal limits for his age. Genitourinary examination reveals mild swelling, erythema, and tenderness over the right hemiscrotum (which, the mother informs you, looks slightly improved from yesterday). Both of the boy's testicles lie normally, and his cremasteric reflexes are intact bilaterally. The remainder of his physical examination findings are unremarkable. Of the following, the best NEXT step in this boy's management would be", "options" : "[\"investigation for sexual abuse because sexually transmitted infections are the most likely cause of this diagnosis in prepubertal boys\", \"no further evaluation is necessary because the boy's recent upper respiratory infection most likely caused him to develop this disorder\", \"referral to a pediatric oncologist because underlying malignancies exist in more than half of prepubertal boys with this diagnosis\", \"referral to a urologist because genitourinary structural abnormalities are present in most boys with this diagnosis\", \"repeat urinalysis and urine culture because infection with Escherichia coli is identified in almost all prepubertal boys with this diagnosis\"]", "explanation" : "The 4-year old boy in the vignette has been diagnosed with acute epididymitis. The boy needs no further evaluation at this time, because his recent upper respiratory infection most likely led him to develop acute epididymitis.\n\nAll pediatric providers should recognize the clinical findings associated with epididymitis and know the most common causes in boys of various ages. Epididymitis is an infection or inflammation of the epididymis. Although it occurs more frequently in sexually active adolescents and adult men, epididymitis can affect prepubertal boys and adolescents who are not sexually active.\n\nSigns and symptoms of epididymitis include unilateral pain and swelling of the scrotum (typically gradual in onset), along with scrotal swelling and erythema. Affected boys may have dysuria, increased urinary frequency, urethral discharge, and fever (though these associated symptoms are not always present). On physical examination, testicles will be lying normally, with intact cremasteric reflexes (which help to distinguish this disorder from testicular torsion). Typically, color Doppler ultrasonography will reveal the affected testis and epididymis with an increase in size and blood flow.\n\nIn prepubertal boys, epididymitis arises most commonly as a postviral infectious phenomenon. Associated viral infections may include (but are not limited to) enterovirus and adenovirus, as well as Mycoplasma pneumoniae. Although infections with bacterial agents such as Escherichia coli can cause epididymitis in prepubertal boys (usually as a result of direct spread of a bacterial urinary tract infection to the epididymis), this etiology is relatively uncommon in this age group.\n\nIn sexually active adolescent boys, acute epididymitis is most commonly associated with sexually transmitted infections, including Chlamydia trachomatis and Neisseria gonorrhoeae. Infection with E coli, mycobacteria, and viruses are also potential causes in this age group.\n\nFor the boy in the vignette, investigation for sexual abuse is not warranted in the absence of other \"red flags\" for abuse, because sexually transmitted infections are not the most likely cause of epididymitis in his age group and he has a history of a recent viral infection.\n\nReferral to a pediatric oncologist is not indicated for this boy, because an association between underlying malignancies and epididymitis has not been demonstrated in the literature.\n\nGiven that the boy in the vignette had negative urinalysis and urine culture results, is improving clinically, and has not had recurrent episodes of epididymitis, referral to a urologist is not indicated. Referral for urologic evaluation for structural abnormalities of the genitourinary tract (such as genitourinary reflux) is indicated for prepubertal boys who are found to have bacterial epididymitis, epididymitis associated with a urinary tract infection, or recurrent epididymitis.\n\nA repeat urinalysis and urine culture is not needed for the boy in the vignette. He has had a recent history of a viral infection, has been afebrile, has had no dysuria or increased urinary frequency, and is improving clinically.\n\nPREP Pearls\n• Signs and symptoms of epididymitis include unilateral pain, scrotal swelling and erythema.\n• In prepubertal boys, epididymitis arises most commonly from a postviral infectious phenomenon.\n• In sexually active adolescent boys, acute epididymitis is most commonly associated with sexually transmitted infections.\n• Urologic referral is indicated for prepubertal boys who have bacterial epididymitis, epididymitis associated with a urinary tract infection, or recurrent epididymitis.\n\nABP Content Specifications(s)\n• Recognize the clinical findings associated with epididymitis\n• Identify common causes of epididymitis in patients of various ages\n\nSuggested Readings\n• Gkentzis A, Lee L. The aetiology and current management of prepubertal epididymitis. Ann R Coll Surg Engl. 2014;96:181–183. doi: http://dx.doi.org/10.1308/003588414X13814021679311.\n• Perron CE, Bin SS. Pain: scrotal. In: Shaw KN, Bachur RG, eds. Fleisher and Ludwig's Textbook of Pediatric Emergency Medicine. 7th ed. Philadelphia, PA: Lippincott Williams & Wilkins; 2016:chap 56:383–391.\n• Somekh E, Gorenstein A, Serour R. Acute epididymitis in boys: evidence of a post-infectious etiology. J Urol. 2004;171:391–394. doi: http://dx.doi.org/10.1097/01.ju.0000102160.55494.1f."}
{"id" : 901, "question_text" : "Your first patient of the morning is a 7-year-old boy who has been \"coughing and wheezing since dinner last night.\" He required 2 albuterol nebulizer treatments last night and has received 3 treatments since 6:00 AM. You observe that the child is sitting upright and appears breathless. His temperature is 37.1°C, oxygen saturation is 82% on room air, heart rate is 120 beats/min, and respiratory rate is 40 breaths/ min. He is using accessory muscles to breathe, and his nails and tongue are blue-tinged. On auscultation of the lungs, air entry is diminished bilaterally and there is mild expiratory wheezing. He is barely able to speak, and his peak expiratory flow is 35% of predicted. Of the following, the MOST likely initial diagnosis is", "options" : "[\"acute moderate asthma exacerbation\", \"acute severe asthma exacerbation\", \"foreign body aspiration\", \"pneumonia\", \"pneumothorax\"]", "explanation" : "The child described in the vignette has signs of severe obstruction in an acute exacerbation of asthma. He is hypoxic and cyanotic and unable to speak whole phrases. He has a markedly increased respiratory rate and uses accessory muscles of respiration. His lung findings are paradoxically and ominously mild in contrast to the other signs of respiratory distress, suggesting severe obstruction and imminent respiratory arrest.\n\nA child with moderate obstruction in contrast will typically be able to speak in phrases; he will have loud expiratory wheeze, peak expiratory flow from 49% to 60% predicted, and pulse oximetry 90% to 95% on room air, in addition to symptoms of obstruction such as increased respiratory rate and chest retractions (Item C195, page C-153). Other features include relief with use of rescue short-acting p agonists and relief in 1 to 2 days after the onset of systemic steroid burst. Note that many of the parameters, and in particular, the correlation with each other, have not been systematically studied. In general, the presence of several parameters helps classify an individual into a particular severity.\n\nThere is no history of choking or ingestion of a foreign body. He has bilateral diminished respiratory sounds making pneumothorax (and foreign body aspiration) less likely. He is afebrile and nontoxic, pushing pneumonia lower on the differential diagnosis.\n\nPREP Pearls\n• The presence of several symptoms and signs helps indicate severity of obstruction in an acute asthma exacerbation; there is no single best assessment tool or measure.\n• Diminution in breath sounds, in a child with other signs of airway obstruction, may indicate imminent respiratory failure.\n\nAmerican Board of Pediatrics Content Specification(s):\n• Recognize the signs of severe obstruction in an acute exacerbation of asthma (severe retractions, inability to speak whole phrases, cyanosis, quiet breath sounds in presence of other signs of obstruction, peak expiratory flow rates less than 30% of predicted)\n\nSuggested Reading:\n• National Asthma Education and Prevention Program. Expert Panel Report 3 (EPR-3): guidelines for the diagnosis and management of asthma—summary report 2007 [published correction appears in J Allergy Clin Immunol. 2008;121(6):1330]. I Allergy Clin Immunol. 2007;120(5 suppl):S94-S138. doi:10.1016/j.jaci.2007.09.029\n• National Heart, Lung, and Blood Institute. Expert Panel Report 3: Guidelines for the Diagnosis and Management of Asthma. National Asthma Education and Prevention Program, Third Expert Panel on the Diagnosis and Management of Asthma. Bethesda, MD: National Heart, Lung, and Blood Institute; 2007"}
{"id" : 2921, "question_text" : "A 16-year-old adolescent is brought to the emergency department by ambulance after he was found wandering outside at 2 am. He is accompanied by a friend who reports that the patient ingested an over-the-counter cough and cold medication that night in order to get high. The patient is uncooperative and agitated. He is not oriented to place or situation. He has a heart rate of 140 beats/min, blood pressure of 125/90 mm Hg, respiratory rate of 12 breaths/min, and oxygen saturation of 100% in room air. He is diaphoretic, and his pupils are dilated. Of the following, the ingredient that is MOST likely responsible for the adolescent's signs and symptoms is", "options" : "[\"acetaminophen\", \"chlorpheniramine\", \"dextromethorphan\", \"guaifenesin\"]", "explanation" : "The boy in the vignette has symptoms consistent with dextromethorphan ingestion. Dextromethorphan is an over-the-counter cough suppressant that is commonly abused. It is the D-isomer of a codeine analog, levorphanol, and also has N-methyl-D-aspartate-receptor antagonist properties. When taken in excess, it can cause euphoria, hallucinations, a feeling of dissociation, tachycardia, mydriasis, and diaphoresis. Treatment of dextromethorphan overdose is supportive. Because many cough and cold preparations that contain dextromethorphan also contain other ingredients with potential toxicities (eg, acetaminophen, antihistamines, pseudoephedrine), it is important to attempt to determine the exact medication ingested and to monitor for additional symptoms.\n\nEarly symptoms of an acetaminophen overdose are mild and nonspecific, including nausea, vomiting, diaphoresis, and lethargy. Liver function abnormalities peak between 72 and 96 hours after ingestion and may be accompanied by hepatic encephalopathy. Chlorpheniramine is an antihistamine with H1-receptor antagonist properties. Overdoses cause features of anticholinergic toxicity, including agitation, tachycardia, hypertension, elevated temperature, dry and flushed skin, and decreased pupillary responsiveness. Guaifenesin is an expectorant. Overdose may cause nausea or vomiting.\n\nPREP Pearls\n• Dextromethorphan is an over-the-counter cough suppressant that, when taken in excess, can cause euphoria, hallucinations, and a feeling of dissociation as well as tachycardia, mydriasis, and diaphoresis.\n• Chlorpheniramine is an over-the-counter antihistamine. Overdoses cause features of anticholinergic toxicity, including agitation, tachycardia, hypertension, elevated temperature, dry and flushed skin, and decreased pupillary responsiveness.\n\nABP Content Specifications(s)\n• Recognize the risk of abuse of over-the-counter cough and cold preparations\n\nSuggested Readings\n• Boyer EW. Dextromethorphan abuse. Pediatr Emerg Care. 2004;20(12):858-863. doi: 10.1097/01.pec.0000148039.14588.d0.\n• Fine JS. Poisoning. In: McInerny TK, Adam HM, Campbell DE, DeWitt TG, Foy JM, Kamat DM. Textbook of Pediatric Care. 2nd ed. Elk Grove Village, IL: American Academy of Pediatrics; 2018:2924-2949. Pediatric Care Online.\n• Wang GS, Hoyte C. Common substances of abuse. Pediatr Rev. 2018;39(8):403-414. doi: 10.1542/pir.2017-0267."}
{"id" : 3299, "question_text" : "A 13-month-old boy is brought to the office by his mother because of concerns about an unusual white reflection noted in his right eye (Item Q198) on recent photographs. She has also noted this eye intermittently turning inward. The child is otherwise healthy. Family history is remarkable for the patient's maternal grandfather requiring enucleation of his right eye as an infant because of an abnormality. A physical examination shows an abnormal red reflex in the right eye with slight esotropia. No other abnormalities are noted. You refer the child for an ophthalmology examination, which reveals a large, elevated, round, yellow mass in the right eye and a small mass in the left eye. Of the following, the MOST likely secondary tumor type that may occur with this disease is", "options" : "[\"astrocytoma\", \"leukemia\", \"neuroblastoma\", \"osteosarcoma\", \"Wilms tumor\"]", "explanation" : "Preferred Response: D\nRetinoblastoma is a malignant intraocular eye tumor that develops in the retina typically before age 5 years. It can be unilateral or bilateral. Patients with a family history of retinoblastoma (approximately 10%) or who have bilateral or multifocal retinoblastoma are more likely to have hereditary retinoblastoma, whereas unilateral and unifocal retinoblastoma tumors are more likely to be sporadic. Hereditary retinoblastoma is caused by germline heterozygous RBI gene mutations on chromosome 13q14 that are transmitted in an autosomal dominant manner, with the retinoblastoma tumor developing after a second somatic mutation or \"hit\" occurs in the RBI gene. About 40% of patients with retinoblastoma have hereditary retinoblastoma. The remainder of cases (60%) are sporadic. All patients with hereditary retinoblastoma have a 50% chance of passing the gene change on to their children.\n\nThe clinical diagnosis of retinoblastoma is based on a thorough examination of the fundus of the eye using an ophthalmoscope revealing a white pupillary reflex known as Leukocoria. Another common presentation is strabismus that can coincide with or be preceded by leukocoria. If 1 eye appears affected, then the other eye should be thoroughly assessed for evidence of a retinoblastoma as well. Staging and definitive diagnosis is supported by magnetic resonance imaging (MRI) and ultrasonography. A head MRI should be included to evaluate for the presence of a pinealoblastoma, which is a retinoblastoma in the pineal gland (if present, this is called trilateral retinoblastoma and can occur at the time of diagnosis or subsequent to the diagnosis of the retinoblastoma). All patients with retinoblastoma should have a medical genetics evaluation for germline RBI mutations.\n\nPreservation of sight is preferred, but not always possible. Treatment choices include enucleation, cryotherapy, chemotherapy, combination cryotherapy-chemotherapy, and radiation therapy. External beam radiotherapy should ONLY be used if absolutely necessary because of the increased risk of second primary cancers with this form of therapy. It is also advisable to avoid the use of computed tomography as an imaging technique because of the increased radiation exposure associated with this modality. Most patients require the involvement of multiple specialists: ophthalmology, oncology, pathology, and radiation oncology. If the tumor has not spread beyond the eye, the cure rate is greater than 90%.\n\nIf a child is at risk for hereditary retinoblastoma based on a known RBI germline mutation, a personal history of unilateral retinoblastoma, or a family history of retinoblastoma, he or she should have an eye examination every 3 to 4 weeks until 1 year of age and frequent surveillance until 3 years of age. Between 3 and 7 years of age, an eye examination should be conducted every 3 to 6 months, then annually to biannually for the remainder of the individual's life. Patients with hereditary retinoblastoma are susceptible to second primary cancers, most commonly pineal gland tumors, osteosarcomas, soft tissue sarcomas, or melanomas. These typically appear in adolescence or adulthood. More than 50% of individuals with second primary tumors have a history of external beam radiation therapy. There are not currently standardized screening protocols for patients with a history of retinoblastoma; however, careful attention should be paid to any symptoms or signs that may suggest a secondary cancer, such as bone pain or masses. Some centers advocate for total body MRIs at routine intervals, but this is not an established screening protocol.\n\nCertain genetic disorders carry a higher predisposition for specific types of cancers. Astrocytomas are more commonly seen in neurofibromatosis type 1 or tuberous sclerosis. Leukemia can be seen in Fanconi anemia, ataxia-telangiectasia, Bloom syndrome, and trisomy 21. Neuroblastoma can be seen in hereditary neuroblastoma because of ALK gene mutations, neurofibromatosis type 1, central congenital hypoventilation syndrome due to PHOX2b gene mutations, and Hirschsprung disease. Wilms tumor can be seen in Beckwith-Wiedemann syndrome, WAGR (Wilms tumor-aniridia-genitourinary anomalies-retardation) syndrome, Denys-Drash syndrome, Frasier syndrome, and isolated hemihyperplasia.\n\nPREP Pearls\n• If a child has a history of retinoblastoma, second primary cancers (the most common of which are osteosarcomas, soft tissue sarcomas, and melanomas) can occur in childhood, adolescence, or adulthood.\n• Retinoblastoma in childhood, which presents with a white pupillary reflex, highlights the importance of using the ophthalmoscope during each well-child check in infancy and childhood to look for a normal positive red reflex or any asymmetries suggestive of potential pathology.\n• if there is a family history of retinoblastoma, pay careful attention to the eye examination of the child during infancy and childhood and refer to ophthalmology to implement high-risk surveillance protocols to screen for retinoblastoma.\n\nAmerican Board of Pediatrics Content Specification(s)\n• Recognize inheritance pattern associated with retinoblastoma and the significance of the family history in planning management\n\nSuggested Reading\n• Canadian Retinoblastoma Society. National Retinoblastoma Strategy Canadian Guidelines for Care: Strategic th6rapeutique du retinablastome guide clinique canaclien. Can Ophthalmol. 2009:44(suppL2):S1-88. doi:10.3129/i09-194.\n• Lohmann DR, Gallic 81_ Retinoblastoma. GeneReviews, Accessed January 22,2014.\n• National Cancer Institute_ Retinoblastoma. National Cancer Institute t,ebsite."}
{"id" : 317, "question_text" : "An 8-month-old girl presents to your office because of poor growth. She was born at 36 weeks' gestation and had a birthweight of 2.5 kg. No problems were noted during the neonatal period, and early growth and development were normal. However, at the age of about 3 months, she was hospitalized because of purulent otitis media. Since that time, she has been treated on two more occasions for otitis media. Over the past month, her parents have noted that the baby has two to three large, foul-smelling, bulky stools per day, and she has failed to gain weight. Her developmental milestones are normal for age, and there are no findings of note on the family history. Physical examination of the alert, well-hydrated infant shows a weight of 6.4 kg, length of 66 cm, and palpable liver edge 3.0 cm below the right costal margin. Initial laboratory study results include: Hemoglobin, 10.8 g/dL (108 g/L); White blood cell count, 3.0x103/mcL (3.0x109/L) (15% neutrophils, 75% lymphocytes, 10% monocytes); Platelet count, 50x103/mcL (50x109/L); Alanine aminotransferase, 61 units/L (normal, up to 30 units/L); Albumin, 3.8 g/dL (38 g/L); Prothrombin time, 12.5 seconds; International Normalized Ratio, 1.1. Of the following, the study that is MOST likely to establish the diagnosis for this infant is", "options" : "[\"genetic testing\", \"liver biopsy\", \"serum amylase\", \"sweat chloride test\", \"tissue transglutaminase antibody assay\"]", "explanation" : "The poor weight gain; history of multiple episodes of otitis media; recent history of several large stools per day; physical finding of extremity bruising; and laboratory findings that indicate anemia as well as neutropenia, a platelet count in the low normal range, and a mild increase in alanine aminotransferase described for the infant in the vignette suggest a disorder characterized by nutrient malabsorption and abnormal hematopoiesis. The clinical condition in the pediatric age group that manifests these findings is Shwachman-Diamond syndrome (SDS), a rare autosomal recessive disorder that is the second most common cause of exocrine pancreatic insufficiency in childhood, after cystic fibrosis (CF). SDS also is characterized by bone marrow dysfunction, skeletal abnormalities, and an increased leukemia risk. Affected children typically present in early infancy with steatorrhea; growth failure; often deficiencies of fat-soluble vitamins A, D, E, and K; and symptoms arising from bone marrow dysfunction. However, SDS, unlike CF, is not associated with abnormalities in pancreatic ductular morphology or function. In fact, lipase excretion increases with age, and older patients often experience normalization of pancreatic function and fat absorption. Thus, in later childhood and adolescence, approximately 50% of patients who have SDS eventually do not require enzyme replacement therapy. Up to 90% of affected patients manifest specific genetic mutations at chromosomal locus 7q11. In infancy, the diagnosis is usually made by genetic testing for the specific mutation, although many cases may be identified by confirming extensive fatty replacement of the pancreas on imaging studies (such as abdominal magnetic resonance imaging, ultrasound, or computed tomography). Pancreatic insufficiency is defined as the loss of pancreatic exocrine function, resulting in reduced digestive enzyme output and the consequent failure of adequate intraluminal nutrient hydrolysis and absorption. Causes of pancreatic insufficiency in children include cystic fibrosis, Shwachman-Diamond syndrome, chronic pancreatitis, autosomal-dominant hereditary pancreatitis, anatomic causes, drug-induced causes, trauma, and metabolic disorders.\n\nInitial assessment of this problem must include a sweat chloride determination. A positive test (>60 mmol/L) strongly indicates a diagnosis of CF, although both false-positive and false-negative results may occur. The \"gold standard\" for evaluation of exocrine pancreatic function is the determination of enzyme output following intravenous secretin stimulation. However, this study is cumbersome, involving endoscopic intubation of the duodenum, and it is difficult to perform accurately. Determination of serum amylase levels may not be useful in this clinical setting because they may be low, normal, or elevated despite pancreatic insufficiency. Furthermore, during infancy, salivary gland production of amylase may result in normal serum levels. Accordingly, other tests are more widely employed to identify the malabsorptive state, and several screening studies have been used to assess exocrine pancreatic function. The serum immunoreactive trypsinogen value is low in most cases of pancreatic insufficiency, including SDS, but is markedly elevated in most infants who have CF, in whom exocrine pancreatic insufficiency is the consequence of pancreatic ductular obstruction, leading to \"reflux\" of enzyme into the bloodstream. For older patients who have CF, in whom progressive fibrosis and acinar destruction of the exocrine pancreas has occurred, serum trypsinogen concentrations are well below normal in 95% of cases. More recently, assay of fecal elastase has been employed to evaluate pancreatic function. Pancreatic elastase is excreted in stool, and it is not affected by bacterial degradation. Exocrine insufficiency is suggested by a fecal elastase concentration of less than 200 µg/g stool. However, although the test is highly sensitive, it has decreased specificity when evaluating exocrine pancreatic insufficiency in children.\n\nInitial estimates of steatorrhea may be made by a qualitative fecal fat determination, following a fat stain for microscopic analysis of a fecal smear. However, the fecal fat stain does not accurately assess fat output, which can only be determined from a 72-hour stool collection. This test is reserved for infants older than 6 months of age because of the normal state of relative pancreatic insufficiency in younger infants. Total stool output is collected in a dry, preweighed vessel over a 72-hour period, during which time the patient consumes a diet containing 3 g/kg fat per day to a maximum of 100 g/day. A coefficient of fat absorption (total fat excreted ÷ total fat intake) of less than 0.93 indicates malabsorption.\n\nA liver biopsy may show nonspecific changes, including steatosis, in patients who have malnutrition, but they are not specific for SDS. The sweat chloride test, which should be performed, rules out CF but does not establish a diagnosis of pancreatic insufficiency. Finally, the tissue transglutaminase antibody assay is both a sensitive and a specific screening study for celiac disease, but for an infant who likely is not consuming gluten at the time of symptom onset, celiac disease is an unlikely diagnosis.\n\nCritique: Preferred Response: A\n\nContent Specifications: Recognize and diagnose exocrine pancreatic insufficiency in infants"}
{"id" : 790, "question_text" : "A 3-year-old boy is brought to the office with a firm, smooth, nontender mass on the right side of the abdomen. He has no fever, vomiting, diarrhea, abdominal pain, or dysuria. Laboratory tests show mild anemia, elevated platelet count, and hematuria. CT scan shows a right-sided renal mass. What is the MOST likely diagnosis?", "options" : "[\"hepatoblastoma\", \"neuroblastoma\", \"renal abscess\", \"renal cell carcinoma\", \"Wilms tumor\"]", "explanation" : "The child described in the vignette with a right-sided renal mass most likely has Wilms tumor. Wilms tumor accounts for approximately 95% of renal tumors in young children, with a peak incidence at 2 to 4 years of age. In adolescents older than 15 years, renal cell carcinoma is the most common renal malignant disease. Wilms tumor usually presents with an abdominal mass (frequently painless), gross or microscopic hematuria, fever, and/or hypertension. Ninety-five percent of cases are unilateral. Approximately 10% to 12% of patients with Wilms tumor have congenital abnormalities, such as cryptorchidism, hypospadias, hemihypertrophy, or aniridia, which may be associated with syndromes such as WAGR (Wilms tumor, aniridia, genitourinary anomalies, and retardation), Beckwith-Wiedemann syndrome, Denys Drash syndrome, and hemihypertrophy. Patients diagnosed as having these syndromes may require ultrasonography screening for Wilms tumors every 4 months until 8 years of age. The initial evaluation for suspected Wilms tumor includes computed tomography with contrast or magnetic resonance imaging of the chest, abdomen, and pelvis to look for bilateral involvement, vascular extension, and metastases (most commonly to lymph nodes, lung, and liver). If the tumor appears to extend into the inferior vena cava, ultrasonography or echocardiography is recommended before surgery. Tissue evaluation at the time of nephrectomy is useful in determining whether the histologic findings are associated with a favorable or unfavorable prognosis. Wilms tumor staging is based on surgical staging and radiographic evaluation for disease spread (Item C83).\n\nOther predictors of poorer prognosis include age older than 2 years, tumor weight greater than 550 g, and loss of heterozygosity on chromosomes 1p and 16q. Treatment for Wilms tumor involves a combination of surgery, chemotherapy, and radiation therapy, depending on the risk category. Overall, the survival rate for Wilms tumor is approximately 90%; therefore, current protocols are aimed at decreasing long-term sequelae of treatment without compromising the excellent survival rate.\n\nThis child has a painless palpable abdominal mass, hematuria, and hypertension. In this age group, the most common renal tumor would be Wilms tumor. In children older than 15 years, renal cell carcinoma is the more prevalent renal malignant disease. Neuroblastoma most often occurs in the adrenal gland and presents at a median age of 19 months; 89% of cases are diagnosed by 5 years of age. Neuroblastoma in infants portends a better prognosis, whereas advanced disease is often found in children whose conditions are diagnosed when they are older than 30 months. Neuroblastoma can present as an incidentally discovered palpable abdominal mass (eg, adrenal mass with hypertension due to compression of the renal vessels), but hematuria is not common. Because of its tendency to metastasize to bone and bone marrow, neuroblastoma frequently also presents with bone pain and cytopenia. Hepatoblastoma is the most common pediatric malignant tumor of the liver; however, it still comprises only 2% of all pediatric malignant tumors. It is associated with prematurity and very low birth weight (<1000 g), hemihypertrophy, and cancer predisposition syndromes, such as Beckwith-Wiedemann, Gardner, and familial adenomatous polyposis syndromes. Although hepatoblastoma can present with a painless abdominal mass, it is not typically associated with hematuria and hypertension. A renal abscess may appear as a mass on imaging; however, there is usually enhancement around the abscess on computed tomography with contrast. Furthermore, a patient with a renal abscess would likely have pyuria, leukocytosis, and high fever as well.\n\nPREP Pearls\n• The most common renal malignant tumor in young children is Wilms tumor.\n• Wilms tumor, hepatoblastoma, and neuroblastoma can present as painless abdominal masses.\n• Wilms tumor can present with abdominal mass, hypertension, and hematuria.\n• In the presence of hemihypertrophy, screening for Wilms tumor with ultrasonography every 4 months is indicated through 8 years of age\n\nAmerican Academy of Pediatrics Content Specification:\n• Understand that Wilms' tumor usually presents as an abdominal mass and may cause hypertension and/or hematuria\n\nSuggested Reading:\n• Buckley KS. Pediatric genitourinary tumors. Curr Opin Oncol. 2012;24:291-296. doi:10.1097/CC0.0b0l3e32835265c9\n• Davenport KP, Blanco FC, Sandler AD. Pediatric malignancies: neuroblastoma, Wilm's tumor, hepatoblastoma, rhabdomyosarcoma, and sacroccygeal teratoma. SurgClin NAm. 2012;746-767. doi:10.1016/j. suc.2012.03.004\n• Fernandez C, Geller JI, Ehrlich PF, et al. Renal tumors. In: Principles and Practice of Pediatric Oncology. 6th Ed. Philadelphia, PA: Lippincott Williams & Wilkins; 2011:861-885"}
{"id" : 1822, "question_text" : "A 16-year-old boy presents for a sports preparticipation physical examination before his junior year in high school. He would like to join his school's wrestling and baseball teams this year. The boy wears glasses and contact lenses for myopia. His corrected vision is 20/20 on the left and 20/70 on the right. Of the following, the MOST appropriate recommendation for this boy is that he may participate in", "options" : "[\"baseball with appropriate sports goggles, but not wrestling\", \"baseball with appropriate sports goggles, and wrestling without special requirement\", \"neither baseball nor wrestling\", \"wrestling, but not baseball\"]", "explanation" : "The adolescent boy in the vignette has corrected vision of worse than 20/40 in 1 eye. Thus, he is considered a functionally 1-eyed athlete. If he were to sustain an eye injury resulting in vision loss in his better-seeing eye, this could result in significant disability. Baseball, basketball, hockey, lacrosse, and racket sports are among the activities typically considered high risk for eye injury, and the boy would require protective eyewear for participation in these sports.\n\nWrestling has historically been considered a sport with a low risk of eye injury. However, a recent study examining high school and college injury databases demonstrated that wrestling had the 4th highest rate of eye injury (after women's basketball, women's field hockey, and men's basketball). There is no commercially available protective eyewear for wrestling. Although custom-made eye protection may be available, the use of eye protection is typically not feasible for competitive wrestlers. Therefore, the boy in the vignette should not participate in wrestling.\n\nThe American Academy of Pediatrics (AAP) policy statement Protective Eyewear for Young Athletes outlines recommendations for eye protection for each sport. Eye injuries account for a relatively small percentage of the total number of sports injuries overall and only a small fraction of these result in severe dysfunction. However, because protective eyewear is easy to use and very effective at preventing eye injuries, protective eyewear is recommended for essentially all sports with a risk of eye injury. Eyewear used by athletes should conform to the American Society for Testing Materials (ASTM) standards for a particular sport as outlined in the AAP statement.\n\nPREP Pearls\n\nAthletes are considered functionally 1-eyed if they have corrected vision of worse than 20/40 in 1 eye.\n\nBaseball, basketball, hockey, lacrosse, and racket sports are considered high risk for eye injury.\n\nEyewear used by athletes should conform to the American Society for Testing Materials standards for a particular sport as outlined in the American Academy of Pediatrics policy statement Protective Eyewear for Young Athletes.\n\nABP Content Specifications(s)/Content Area\n\nRecognize the indications for the use of eye goggles during sports activities\n\nSuggested Readings\n\nAmerican Academy of Pediatrics Committee on Sports Medicine and Fitness. Protective eyewear for young athletes. Pediatrics. 2004;113(3 Pt 1):619-622. doi: http://dx.doi.org/10.1542/peds.113.3.619.\n\nBoden BP, Pierpoint LA, Boden RG, Comstock RD, Kerr ZY. Eye injuries in high school and collegiate athletes. Sports Health. 2017;9(5):444-449. doi: http://dx.doi.org/10.1177/1941738117712425."}
{"id" : 2157, "question_text" : "A 2-month-old term infant was seen in the clinic for a health supervision visit. He has been exclusively fed a standard cow milk protein formula. His parents reported frequent large-volume spit-ups immediately following each feed. He displayed no evidence of pain or discomfort during these episodes. At that visit, both his weight and length were at the 25th percentile for age. They were counseled regarding appropriate feeding volumes, and a recommendation was made to thicken the feeds using oatmeal, barley, or a multigrain infant cereal, which they have followed. At his 4-month visit, his parents report similar symptoms. His weight is at the 10th percentile, and his length is at the 25th percentile for age. He is meeting all developmental milestones. Of the following, the BEST next step in management is", "options" : "[\"initiation of a histamine-2 receptor antagonist\", \"initiation of a proton pump inhibitor\", \"referral to a pediatric gastroenterologist\", \"trial of an extensively hydrolyzed protein-based formula\"]", "explanation" : "Critique\nIn this vignette, the infant's symptoms meet diagnostic criteria for gastroesophageal-reflux disease (GERD); his weight percentile declined between his 2- and 4-month visits in the setting of frequent large-volume regurgitation of gastric contents. He failed the first-line interventions of overfeeding avoidance and formula thickening, therefore, the best next step in his management is a trial of extensively hydrolyzed protein-based formula.\nGastroesophageal reflux (GER) is a physiologic process of passing gastric contents into the esophagus with or without contents passing orally. This common condition peaks at age 4 months, typically self-resolves, and requires no intervention. When there is GER with complications (eg, esophagitis, refusal to eat, poor weight gain), the diagnosis of GERD is made. Symptoms of GERD (eg, excessive crying, irritability, back arching, regurgitation) vary and overlap with other conditions as well as normal infant behavior. \nDiagnostic testing is typically not recommended for investigation of GERD in infants. Initial management centers on nutritional and non-pharmacological interventions. The first-line therapy for GERD, recommended by both the North American and the European Societies for Pediatric Gastroenterology, Hepatology, and Nutrition, are overfeeding avoidance and thickening of feeds. Overfeeding avoidance and volume reduction can be accomplished through smaller volume feeds at an increased frequency and/or adjustment in the caloric fortification of formula. Feeding thickeners can include cereal-based and commercial thickeners. Cereal-based thickeners should be low or no arsenic products (eg, oatmeal, barley, multigrain; rice cereal has a significantly higher arsenic content). Cereal thickening is not recommended for use with breast milk, as the amylase in breast milk will begin to digest the cereal, decreasing its effectiveness. Commercial thickening agents (eg, carob bean thickener) may be used after 42 weeks' gestational age. Xanthan gum thickeners should be avoided until after age 1 year old due to the risk of necrotizing enterocolitis. \nDifferentiating between the symptoms of GERD and cow milk protein allergy can be challenging. Therefore, the second-line recommendation for management of GERD, also non-pharmacological, is a 2- to 4-week trial of an extensively hydrolyzed protein-based or amino acid–based formula. Soy formula is not recommended in the management of GERD. \nIf symptoms persist despite appropriate nonpharmacological intervention, the next step in management is a trial of pharmacological therapy and/or referral to a pediatric gastroenterologist. The first-line pharmacological recommendation is a 4- to 8-week trial of a proton pump inhibitor (PPI) (eg, omeprazole), or a histamine-2 receptor antagonist (H2RA) (eg, famotidine). Factors such as ease of use, availability, and cost should be considered. Gastric acid is a protective mechanism against gastrointestinal infections, and case-control studies have shown an increased risk of infection in infants taking acid suppressive therapy. Other pharmacological interventions are not recommended. There is a lack of evidence to support the use of prebiotics and probiotics. \nSuggested Reading(s)\nBerg EA, Khlevner J. Treatment of gastroesophageal reflux disease in children. Pediatr Rev. 2021;42(1):51-53. doi:10.1542/pir.2020-001602 \nRosen R, Vandenplas Y, Singendonk M, et al. Pediatric gastroesophageal reflux clinical practice guidelines: joint recommendations of the North American Society for Pediatric Gastroenterology, Hepatology, and Nutrition and the European Society for Pediatric Gastrointestinal, Hepatology, and Nutrition. J Pediatr Gastroenterol Nutr. 2018;66(3):516-554. doi:10.1097/MPG.0000000000001889\nContent Domain\nGastroenterology\nLearning Objectives\nImplement the gastroesophageal clinical practice guideline recommendations for infants\nThe correct answer is: trial of an extensively hydrolyzed protein-based formula\nView Peer Results"}
{"id" : 3283, "question_text" : "A 14-year-old adolescent girl presents for evaluation of short stature. Her height is at the fifth percentile. Her parents noticed that her growth rate slowed significantly over the last year, and they are concerned that she will be much shorter than her siblings, all of whom reached the 25th percentile in height. The girl reports that she had her first menstrual period 1 month ago. You inform the family about the average height gain after menarche. Of the following, the MOST accurate prediction for height gain after reaching this stage of puberty is", "options" : "[\"1 cm\", \"4.5 cm\", \"7.5 cm\", \"10 cm\", \"13 cm\"]", "explanation" : "Preferred Response: C\nJust before puberty, linear growth rates (growth velocity) gradually decelerate. Then, at the start of puberty, at approximately 9 years of age, growth begins to accelerate as serum gonadotropins (luteinizing hormone, follicle-stimulating hormone) gradually increase, but the exact timing of this can be variable. Luteinizing hormone levels increase 23-fold from prepuberty to late puberty. In girls, sex hormone levels increase as a consequence of ovarian and adrenal maturation, and physiologic leukorrhea (vaginal discharge) may occur secondary to rising estrogen stimulation. Production of sex hormones at puberty (both estrogen and testosterone) stimulates growth rates, with the pubertal growth spurt for girls beginning at sexual maturity rating 2. Peak height velocity of approximately 8 to 9 cm/year is achieved for girls at sexual maturity rating 3, about 1 year before menarche. After menarche, only about 7.5 cm of growth remains, and growth is 99% complete at a bone age of 15. It is expected that the child in this vignette, who is now postmenarchal, would only grow a small amount after menarche.\nThe pubertal growth spurt in boys begins an average of 2 years later than in girls. A peak height velocity of 9.5 to 10 cm/year is achieved by sexual maturity rating 4. Growth is 99% complete at a bone age of 17. The combination of a longer period of prepubertal growth and a greater pubertal height velocity results in the average adult height difference of 13 cm between men and women.\n\nAdditional physiological changes occur during puberty. Estrogens promote lipogenesis and redistribution of body fat toward the adult female body habitus. In contrast, androgens are lipolytic and promote muscular development. Thus, the increase in body mass index that occurs in both boys and girls during pubertal development is because of differences in body composition, with a higher percentage being body fat in girls and lean body mass in boys.\n\nPREP Pearls\n• Girls achieve an average peak growth velocity of 8 to 9 cm/year by sexual maturity rating 3. Boys' growth velocity peaks at an average of 10 cm/year by sexual maturity rating 4.\n• After menarche, growth slows down rapidly, with only about 7.5 cm of linear growth potential remaining.\n\nAmerican Board of Pediatrics Content Specification(s)\n• Recognize the physiologic changes that commonly precede menarche\n\nSuggested Reading\n• Bordini B, Rosenfield RL. Normal pubertal development: Part I: The endocrine basis of puberty. Pediarr Rev_ 2011;32(6):223-229. doi:10.1542/ pir.32-6-223.\n• Bordini B, Rosenfield RL. Normal pubertal development: Part II: clinical aspects of puberty. Pediatr Rev. 2011;32(7):281-292. doi:10.1542/pir.32-7-281.\n• Roche AF. The final phase of growth in stature. Growth, Genetics, and Hormones. 1989;5(4):4-6.\n• Rogol AD, Roemmich JN, Clark PA. Growth at puberty. JAdolesc Health. 2002;31(6 suppl):192-200. doi:10.1016/51054-139X(02)00485-8."}
{"id" : 1893, "question_text" : "A previously healthy 2-month-old boy was brought to the emergency department by his mother. Approximately 15 minutes after a feeding, he had an episode in which he suddenly started choking, gagging, and arching his back. His skin color turned red, then dusky. He then appeared to stop breathing for approximately 10 seconds, after which he was briefly gasping then resumed his normal breathing pattern. After about 1 more minute, his color returned to normal and he was appropriately responsive. The boy has no history of similar episodes. He was born at 35 weeks of gestation to a gravida 1, para 0 mother. There were no illnesses or infections during the pregnancy, and no concerns in the perinatal period. The boy has not had any recent illnesses, fever, or difficulty breathing. He does not breathe noisily or snore. He has been gaining weight appropriately, and takes 4 ounces of formula every 3 hours, requiring about 5 minutes to finish a feeding. When he is burped after every feed, a small amount of formula dribbles out of his mouth. The boy's vital signs are as follows: temperature, 37°C; blood pressure (right arm), 80/45 mm Hg; blood pressure (left leg), 75/40 mm Hg; respiratory rate, 25 breaths/min; and heart rate, 130 beats/min. Physical examination reveals a well-nourished infant with no external signs of trauma. While sleeping, the boy has occasional periods of apnea lasting about 6 seconds, which are followed by a few second-long periods of rapid, shallow breathing. He then resumes a normal breathing pattern. He arouses appropriately with examination. His lungs are clear, pulses are equal and strong, and his heart has a normal S1 and S2 which is variably split with breathing, with no rubs, gallops, or murmurs. His abdomen is soft, nontender, and nondistended, with no masses or organomegaly.\n\nOf the following, the MOST likely explanation for this event is", "options" : "[\"anomalous left coronary artery\", \"brief resolved unexplained event (BRUE)\", \"periodic breathing\", \"seizure\"]", "explanation" : "Correct Answer: B\nThe infant in the vignette had an episode in which he started choking, changed color, and appeared to stop breathing. The episode resolved, and the infant otherwise does not have any risk factors for sudden death. The most likely explanation for the event is a brief resolved unexplained event (BRUE), which was previously referred to as \"apparent life-threatening event\" (ALTE).\n\nThe term BRUE refers to an episode in which an infant younger than 1 year of age exhibits 1 or more of the following: (1) cyanosis or pallor; (2) apnea, hypopnea, or irregular breathing; (3) hypertonia or hypotonia; and (4) altered mental status. In 2016, the American Academy of Pediatrics published BRUE clinical practice guidelines, which provided diagnosis and management recommendations based on the likelihood of an underlying disorder or subsequent events. The guidelines also recommended replacing the term ALTE with BRUE, to decrease the subjectivity in taking the history, remove the implication of a life-threatening event, and emphasize that the event has resolved.\n\nThe diagnosis of BRUE requires that there be no known cause of the event. There are many causes for such events that would not qualify as BRUE, such as acquired or congenital disorders of the airway, cardiovascular disease, gastroesophageal reflux disease, central nervous system pathology, nonaccidental trauma, intercurrent illness, and metabolic conditions (Item C93A). Intercurrent illness may be heralded by viral symptoms such as fever, congestion, or persistent respiratory signs or symptoms. Abnormal physical findings at the time of assessment may indicate an underlying disorder. A thorough history should be taken to delineate the details of the event, and to screen for factors that would indicate an underlying disorder.\n\nSeveral characteristics differentiate infants' risks of having a recurrent BRUE event. Infants younger than 2 months, those born at less than 32 weeks' gestational age with corrected gestational age less than 45 weeks, and those who had a previous event are considered to be at higher risk. Patient factors associated with a lower risk of recurrent BRUE include age greater than 60 days, duration of the event less than 1 minute, no history of cardiopulmonary resuscitation (CPR) by a trained medical provider during the event, and no concerning historical or physical examination features. Infants classified as being at higher risk and those for whom the diagnosis of BRUE is excluded are considered outside the scope of the guidelines.\n\nManagement recommendations for patients at lower risk for BRUE are presented in 4 categories:\n1) \"should\" provide education for caregivers and resources for CPR training to caregivers.\n2) \"may\" include pertussis testing, 12-lead electrocardiography, and brief monitoring with continuous pulse oximetry and serial observations.\n3) \"should not\" obtain laboratory testing; perform radiography, electroencephalography, or echocardiography; initiate home cardiorespiratory monitoring; prescribe medications.\n4) \"need not\" obtain viral respiratory testing, urinalysis, blood glucose, serum bicarbonate, or serum lactate testing, or neuroimaging; or admit to hospital strictly for cardiorespiratory monitoring (Item C93B).\n\nItem C93B: Diagnosis, risk classification, and recommended management of a BRUE. Reprinted with permission from Tieder JS, Bonkowsky JL, Etzel RA, et al. Brief resolved unexplained events (formerly apparent life-threatening events) and evaluation of lower-risk infants. Pediatrics. 2016;137(5):e7.\n\nAnomolous left coronary artery can manifest at any age, particularly in infancy, and can cause congestive heart failure, arrhythmia, and sudden death. This diagnosis is not likely in this vignette, because the historical symptoms and physical examination findings of coronary insufficiency or congestive heart failure are not present.\n\nThe 6-second periods of apnea that the boy exhibits are consistent with periodic breathing, a normal newborn respiratory pattern that is not associated with choking, gagging, or color change. Although seizure is a possible explanation for the event, it is less likely than BRUE, given the absence of predisposing conditions, the rapid return to normal state, and the boy's otherwise low-risk stratification.\n\nPREP Pearls\n\nThe term \"brief resolved unexplained event\" has replaced \"apparent life threatening event.\" This change was implemented to decrease the subjectivity in event history taking, remove the implication that the event was life-threatening, and emphasize that the events are fully resolved.\n\nPatients presenting after a brief resolved unexplained event who meet lower-risk criteria do not require electroencephalography, neuroimaging, or laboratory testing.\n\nPatients experiencing a brief resolved unexplained event–like event with a concerning history or physical examination findings may fall outside of the definition and scope of the clinical guideline recommendations for brief resolved unexplained event.\n\nABP Content Specifications(s)/Content Area\n\nIdentify risk factors associated with sudden infant death syndrome\n\nRecognize the clinical findings of an brief resolved unexplained event (BRUE), and manage appropriately\n\nCounsel parents regarding prevention of sudden infant death syndrome\n\nSuggested Readings\n\nBommel N, Kannarkatt S. Case 5: BRUE in an infant found to have feeding difficulties. Pediatr Rev. 2017;38:535. doi: 10.1542/pir.2016-0040.\n\nTieder JS, Bonkowsky JL, Etzel RA, et al; Subcommittee on Apparent Life Threatening Events. Brief resolved unexplained events (formerly apparent life-threatening events) and evaluation of lower-risk infants. Pediatrics 2016;137(5). pii: e20160591. doi: 10.1542/peds.2016-0591.\n\nZwemer E, Claudius I, Tieder J. Update on the evaluation and management of brief resolved unexplained events (previously apparent life-threatening events). Rev Recent Clin Trials. 2017;12(4):233-239. doi: 10.2174/1574887112666170816150104."}
{"id" : 2917, "question_text" : "A 4-year-old boy is seen in the emergency department 30 min after his mother saw him swallow something shiny. Since then, the boy has been complaining of abdominal pain but is not drooling. His heart rate is 110 beats/min, his respiratory rate is 20 breaths/min, and his oxygen saturation as measured via pulse oximetry is 98% in room air. He is alert and interactive. His breath sounds are normal, with no wheezing or stridor. A chest radiograph is obtained (Item Q118). Of the following, the BEST next step in caring for this boy is", "options" : "[\"endoscopic removal of object\", \"reassurance to his mother\", \"repeat radiography in 6 hours\", \"upper gastrointestinal series\"]", "explanation" : "The boy in the vignette has swallowed a button battery that is lodged in the esophagus and should be removed promptly, ideally within 2 hours of ingestion. Because of the risk of severe injury caused by the battery, follow-up imaging and reassurance are not appropriate next steps for the boy in the vignette.\n\nChildren often swallow coins and batteries, which can become lodged in the esophagus. Symptoms of an object lodged in the esophagus include throat pain, abdominal pain, excessive drooling, and nausea. Coins and button batteries appear similar on radiography, but a button battery will show an outer rim often referred to as a \"halo effect.\" When a button battery becomes lodged in the esophagus, the battery's electrical circuit is completed; this leads to erosive electrical burns unless the battery is removed quickly via endoscopy. Endoscopy allows the clinician to visualize the extent and location of the injury. Other evidence-based interventions that can help prevent injury include giving the child honey (if older than 12 months of age) or sucralfate suspension shortly after ingestion. After battery removal, if there is no perforation, rinsing with a gentle acid such as sterile acetic acid can help neutralize residual alkali from the battery. Careful observation in the hospital is important if esophageal injury is observed during endoscopy because complications—including fistulas, erosion into blood vessels, or esophageal perforation—can occur 48 hours or longer after the removal of the battery.\n\nIf the button battery has already passed into the stomach at the time of presentation, management depends on whether the child is symptomatic. In a symptomatic child, even with mild symptoms, the battery should be removed. Asymptomatic children without any underlying conditions, who swallowed a battery smaller than 12 mm, and who have no additional foreign bodies such as a second battery or magnet, can be closely observed at home by a reliable caregiver. Imaging should be repeated in 10 to 14 days to confirm that the battery has passed in the stool.\n\nCoins are much more commonly ingested. The most concerning complication of coin ingestion is aspiration into the trachea. The location of the coin in the trachea versus the esophagus can be determined via radiography. Typically, a coin in the trachea is positioned perpendicularly on an anteroposterior radiograph, whereas the more common \"face-on\" view is seen when the coin is in the esophagus. In any child with a coin suspected to be in the trachea or who is symptomatic, the coin should be removed under direct visualization. Most coins lodged in the distal esophagus will pass into the stomach without intervention, whereas only about 15% of coins in the proximal esophagus will pass to the stomach within 24 hours. Children who are asymptomatic can be observed and undergo repeat imaging after 12 to 24 hours. Once the coin is in the stomach or more distal, it is likely to pass without complications. Additional radiography can be performed if the coin is not observed in the stool by 4 weeks.\n\nPREP Pearls\n• A button battery lodged in the esophagus should be removed promptly, ideally within 2 hours of ingestion.\n• Most coins lodged in the distal esophagus will pass into the stomach without intervention; only about 15% of coins in the proximal esophagus will pass to the stomach within 24 hours.\n• On an anteroposterior radiograph, a coin in the trachea will typically be positioned perpendicularly, whereas a coin in the esophagus will appear \"face on.\" A button battery in the esophagus will have the radiographic appearance of a halo.\n\nMOCA-Peds Objective\n• Manage foreign body ingestion.\n\nABP Content Specifications(s)\n• Plan the management of a patient who has ingested a coin\n• Plan the management of a patient who has ingested a button battery\n\nSuggested Readings\n• Clute J, Frey T, Reed J. Visual diagnosis: button versus battery: foreign body ingestions in pediatrics. Pediatr Rev. 2019;40(11):e39-e41; doi:10.1542/pir.2017-0263.\n• Green S. Ingested and aspirated foreign bodies. Pediatr Rev. 2015;36(10):430-437. doi:10.1542/pir.36-10-430.\n• National Capital Poison Center. National Capital Poison Center button battery ingestion triage and treatment guideline. https://www.poison.org/battery/guideline.\n• Skae CC, Parikh SR. Foreign bodies of the ear, nose, airway, and esophagus. In: McInerny TK, Adam HM, Campbell DE, DeWitt TG, Foy JM, Kamat DM, eds. American Academy of Pediatrics Textbook of Pediatric Care. 2nd ed. Elk Grove Village, IL: American Academy of Pediatrics; 2016:2021-2026. Pediatric Care Online."}
{"id" : 2455, "question_text" : "During a health supervision visit, a parent expresses concern that her 6-year-old is distracted at school and the teacher often calls regarding her behavior. Her mother states that bedtime is often a diïcult experience and her child may not fall asleep until 11 PM. Counseling is provided regarding the importance of a bedtime routine. Of the following, the BEST routine to address the mother's concern is", "options" : "[\"game, back rub, brush teeth, water\", \"juice, bath, game, back rub\", \"snack, brush teeth, book, cuddle\", \"snack, movie, bath, story\"]", "explanation" : "PREP Pearl(s)\nLack of sleep and excess sleep are associated with obesity and mental health disorders.\nThe ideal duration of sleep varies according to age; infants require 12 to 16 hours total (including naps), whereas adolescents require 8 to 10 hours.\nThe importance of sleep quality and duration, facilitated by consistent bedtime routines with avoidance of screen time, should be discussed at health supervision visits.\nCritique\nLack of sleep and too much sleep both have negative behavioral and health eìects. A lack of sleep can lead to inattention, poor behaviors, impulsivity, obesity, depression, and hypertension. Excess sleep is associated with obesity and mental health disorders. Bedtime routines that improve both sleep quality and duration lead to better overall health.\nConsistent bedtime routines promote healthy sleep. These routines are most successful if they consistently start at the same time each night (including weekends) rather than when children appear tired. Consistently following a bedtime activity pattern that children are able to recognize is also helpful. One approach is to choose something from each of 4 categories:\nNutrition (eg, healthy snack)\nHygiene (eg, brushing teeth)\nCommunication (eg, reading a book)\nPhysical connection (eg, cuddling)\nParticipating in these activities in the same order each night helps the child to calm down and be ready for sleep.\nScreen time within 30 minutes of bedtime signiícantly interferes with good sleep. It is recommended that there be no screen time for 30 minutes before beginning the bedtime routine. Computers, phones, tablets, television, and other screen devices should be removed from sleep spaces.\nThe ideal amount of sleep children need varies by age (Table). Bedtime rituals should take into consideration school/day care hours. For example, a 6-year-old child who begins school at 7:45 AM and needs to wake by 6:30 AM should be asleep between 6:30 and 9:30 PM\nSuggested Reading(s)\nAmerican Academy of Pediatrics. Brush, book, bed: how to structure your child's nighttime routine. Accessed December 2, 2022. https://www.healthychildren.org/English/healthy-living/oral-health/Pages/Brush-Book-Bed.aspx/\nJenco M. AAP endorses new recommendations on sleep times. AAP News; June 13, 2016. Accessed December 2, 2022. https://publications.aap.org/aapnews/news/6630?autologincheck=redirected\nKrishna J, Kalra M, McQuillan ME. Sleep disorders in childhood. Pediatr Rev. 2023;44(4):189–202. doi:10.1542/pir.2022-005521\nMindella JA, Williamson AA. Beneíts of a bedtime routine in young children: sleep, development, and beyond. Sleep Med Rev. 2018;40:93-108. doi:10.1016/j.smrv.2017.10.007\nSplaingard ML, May A. Sleep disturbances (nonspeciíc). In: McInerny TK, Adam HM, Campbell DE, Foy JM, Kamat DM, eds. American Academy of Pediatrics Textbook of Pediatric Care. 2nd ed. American Academy of Pediatrics; 2016:chap 194. Pediatric Care Online\nContent Domain\nPreventive Pediatrics\nABP Content Specication(s) / Content Area(s)\nUnderstand the normal developmental progression of sleep patterns\nPlan the appropriate management of bedtime refusal/frequent awakening\nCounsel parents regarding appropriate bedtime routines for their children"}
{"id" : 2028, "question_text" : "A 16-year-old adolescent boy has concerns of weight loss and fatigue. His family believes he has lost 4.5 kg in the last 4 to 6 weeks. He also reports abdominal bloating and diarrhea. This week, a friend whom he had not seen for several months remarked that his eyes were yellow. He has no prior history of jaundice. His family is concerned about possible drug use because he has had difficulty walking steadily. He has a history of depression diagnosed at 13 years of age and treated with psychotherapy. There is no family history of liver disease, autoimmune diseases, or emphysema. He has normal vital signs for age. He has a weight of 47.7 kg (5th percentile), height of 167 cm (25th percentile), and body mass index of 17 kg/m2 (5th percentile). He appears fatigued and jaundiced with scleral icterus. His abdomen is distended with a fluid wave. His liver edge is firm and at the right costal margin. His spleen is palpated 5 cm below the left costal margin. There is no edema. He has palmar erythema. Laboratory data are shown: White blood cell count 6,000/µL (6.0 × 109/L), Hemoglobin 11.0 g/dL (110 g/L), Platelet count 95 × 103/µL (95 × 109/L), Conjugated bilirubin 4.5 mg/dL (77.0 µmol/L), Alanine aminotransferase 245 U/L, Aspartate aminotransferase 215 U/L, Prothrombin time 18.0 s, International normalized ratio 1.6, Vitamin D, 25-hydroxy 9 ng/mL (22 nmol/L), Urine toxicology screen Negative. Of the following, the test that is MOST likely to establish the diagnosis is", "options" : "[\"\\u03b11-antitrypsin level\", \"ceruloplasmin\", \"hepatitis C virus antibody\", \"partial thromboplastin time\"]", "explanation" : "The patient in this vignette has Wilson disease, an autosomal recessive neurologic disorder associated with chronic liver disease, resulting from abnormal copper deposition in tissue. Wilson disease may present with clinical features including acute hepatitis and/or chronic hepatitis or cirrhosis, neurologic symptoms (movement disorders, tremors, ataxia, seizures), psychiatric symptoms (dementia, depression, schizophrenia, bipolar disorder), and ocular findings (Kayser-Fleischer rings). Renal tubular dysfunction, hemolysis, cardiac arrhythmias, and endocrine disorders may also occur. The patient in this vignette has evidence of chronic liver disease and portal hypertension (scleral icterus, splenomegaly, ascites, palmar erythema, thrombocytopenia, elevated aminotransferase levels, elevated conjugated bilirubin level, and mild coagulopathy) with neurologic/psychiatric symptoms (ataxia and depression), consistent with Wilson disease. Wilson disease should be considered in any older child or adolescent with liver injury of any degree and should be highly suspected in individuals with liver injury and neurologic/psychiatric symptoms.\n\nThis patient has cholestasis, or conjugated hyperbilirubinemia. Causes of cholestasis in older children and adolescents include biliary tract obstruction (cholelithiasis, choledochal cyst), viral infections (eg, infectious hepatitis A, B, and C; Epstein-Barr virus; cytomegalovirus), autoimmune disease (primary sclerosing cholangitis, autoimmune hepatitis), drug-induced cholestasis, Wilson disease, and bile transport defects. In neonates, there are many more causes of cholestasis, including biliary atresia, congenital/acquired infections, genetic/metabolic diseases (eg, α1-antitrypsin deficiency, cystic fibrosis), and endocrine disorders (eg, hypothyroidism, panhypopituitarism).\n\nIn patients with cholestasis, in addition to considering the etiology, careful attention should be paid to nutritional status. Cholestatic infants and children may be malnourished because of fat maldigestion and fat malabsorption resulting from poor bile flow from the liver to the small intestine. This maldigestion and malabsorption may result in greasy malodorous stools (steatorrhea), weight loss, poor growth, and fat-soluble vitamin deficiency. Nutritional management of these patients includes increasing caloric intake (through oral supplements or enteral supplementation with nasogastric feeding tubes; preferably with supplements that are high in medium-chain triglycerides) and using fat-soluble vitamin supplements.\n\nAlthough α1-antitrypsin deficiency is a cause of cholestasis, it most commonly presents early in life with prolonged jaundice. In addition, it is associated with emphysema in adults and is not associated with neurologic/psychiatric disease. Although chronic hepatitis C infection can result in significant chronic liver injury and portal hypertension, severe disease rarely occurs in children, and hepatitis C infection is not associated with neurologic/psychiatric disease. Partial thromboplastin time is used to assess bleeding disorders.\n\nPREP Pearls\n\nA history of neuropsychiatric symptoms in combination with any degree of liver injury in an older child or adolescent should prompt evaluation for Wilson disease.\n\nABP Content Specifications(s)/Content Area\n\nUnderstand the nutritional causes of growth failure associated with chronic cholestatic disease, including the effects of nutrient digestion and absorption\n\nRecognize the clinical features associated with alpha-1-antitrypsin deficiency\n\nRecognize the clinical features associated with Wilson disease\n\nSuggested Readings\n\nBrumbaugh D, Mack C. Conjugated hyperbilirubinemia in children. Pediatr Rev. 2012;33(7):291-302. doi:10.1542/pir.33-7-291.\n\nPerlmutter DH. Alpha-1 antitrypsin deficiency. In: Suchy FJ, Sokol RJ, Balistreri WF, eds. Liver Disease in Children. 4th ed. Cambridge, United Kingdom: Cambridge University Press; 2014:400-418.\n\nRoberts EA, Schilsky ML. Diagnosis and treatment of Wilson disease: an update. Hepatology. 2008;47(6):2089-2111. doi:10.1002/hep.22261.\n\nSokol RJ. Copper metabolism and copper storage disorders. In: Suchy FJ, Sokol RJ, Balistreri WF, eds. Liver Disease in Children. 4th ed. Cambridge, United Kingdom: Cambridge University Press; 2014:465-492."}
{"id" : 2381, "question_text" : "An 8-year-old girl with a history of well-controlled, moderate, persistent asthma is admitted to the pediatric ward for an asthma exacerbation. She is fully immunized, including the inîuenza and COVID-19 vaccines. She began to experience fever, runny nose, and cough 4 days ago. She was brought to the emergency department this morning for worsening shortness of breath. In the emergency department, the girl's temperature was 39°C and her oxygen saturation was 87% in room air. Lung examination revealed diìuse wheezing without focal índings. Oxygen supplementation by nasal cannula, oral prednisolone, and albuterol by inhalation were administered. She was admitted to the hospital for further treatment. The results of rapid testing of nasopharyngeal secretions were positive for inîuenza and negative for COVID-19. Of the following, the BEST next step in this child's management is to administer", "options" : "[\"ampicillin\", \"nirmatrelvir-ritonavir\", \"no additional medication\", \"oseltamivir\"]", "explanation" : "The best next step in management of the child in the vignette is to administer oseltamivir. Speciíc antiviral therapy (eg, neuraminidase inhibitors) is underused in the pediatric setting. Although most eìective when given early in the course of inîuenza virus infection (preferably within 2 days of symptom onset), a neuraminidase inhibitor is indicated for any patient requiring hospitalization. It is also indicated for any infected individual with a high-risk household contact (eg, family members <6 months of age or >65 years of age).\n\nThree neuraminidase inhibitors have been approved for use in children: oseltamivir (capsule or a suspension), peramivir (single intravenous dose), and zanamivir (dry-powdered inhaler). Zanamivir is not recommended for children younger than 7 years or those with asthma (due to the risk of worsening bronchospasm). Ampicillin is not indicated for this child with no evidence of pneumonia or other bacterial infection. Nirmatrelvir-ritonavir is an oral antiviral combination therapy that is only indicated for outpatient use in nonhypoxic patients older than 12 years with evidence of COVID-19. Giving no speciíc antiviral therapy would not be the best management for this child who required hospitalization for treatment of an inîuenza virus infection.\n\nSuggested Reading(s)\nAmerican Academy of Pediatrics. Inîuenza. In: Kimberlin DW, Barnett ED, Lyníeld R, Sawyer MH, eds. Red Book: 2021-2024 Report of the Committee on Infectious Diseases. 32nd ed. American Academy of Pediatrics; 2021. Red Book Online\n\nCampbell AP, Tokars JI, Reynolds S, et al. Inîuenza antiviral treatment and length of stay. Pediatrics. 2021;148(4):e2021050417. doi:10.1542/peds.2021-050417\n\nMehta K, Morris SK, Bettinger JA, et al. Antiviral use in Canadian children hospitalized for inîuenza. Pediatrics. 2021;148(4):e2020049672. doi:10.1542/peds.2020-049672\n\nMoodley A, Bradley JS, Kimberlin DW. Antiviral treatment of childhood inîuenza: an update. Curr Opin Pediatr. 2018;30(3):438-447. doi:10.1097/MOP.0000000000000618\n\nContent Domain\nInfectious Diseases\n\nABP Content Specication(s) / Content Area(s)\nPlan appropriate antiviral therapy for the treatment of inîuenza, while considering drug-resistant strains\nRecognize the risk factors for complications associated with inîuenza virus infection, including those that lead to hospitalization\n\nThe correct answer is: oseltamivir"}
{"id" : 1527, "question_text" : "It is your office's policy to ensure that a portion of each appointment with an adolescent patient is designated to interview and counsel the patient without a parent present. A premedical student shadowing you in your clinic asks if there are situations in which you would suggest that the parents accompany the adolescent during the entire visit. You explain the importance of providing confidential services to adolescents. You then discuss times when having a parent present during the entire visit is essential.\n\nOf the following, the MOST appropriate time for such involvement would be if the adolescent is", "options" : "[\"being evaluated for a life-threatening illness\", \"being evaluated for substance abuse\", \"being seen for a simple complaint like a cold\", \"hearing impaired and relies on the parent for communication\", \"intellectually disabled with an IQ of 45\"]", "explanation" : "Correct Answer: E \nWhen providing care to adolescents, 2 important concepts must be kept in mind: (1) consent¾who is authorized to give consent for care, and (2) confidentiality¾who has the right to control the release of information.\n\nAn intellectually disabled adolescent with an IQ of 45 is unlikely to be capable of providing informed consent or a reliable history; therefore, having a parent present during the entire visit would almost always be appropriate. None of the other situations would preclude the practitioner from providing an opportunity for confidential services.\n\nEmancipated minors have adult status and can provide full consent for their own health care. Criteria for emancipation typically include marriage, military service, or living apart from parents while being self-supporting. Many states have procedures for recognizing minors as emancipated.\n\nAll states have provisions that authorize minors to give consent for certain services such as contraceptive services, pregnancy care, evaluation and treatment for sexually transmitted infections, or substance abuse. In the vast majority of states, minors can receive emergency carewithout the prior consent of a parent. Furthermore, most states recognize the \"mature minor doctrine.\" This doctrine permits consent for treatment (or refusal) by minors if they are at least 15 years old, able to understand the risks and benefits of the proposed treatment, the care is not high risk, is of benefit to the minor, and is a part of established medical management.\nIn most states, minor consent laws also address confidentiality, and the disclosure of information, when a minor is authorized to give consent for care. In addition, under the Privacy Rule of the Health Insurance Portability and Accountability Act (HIPPA), when minors legally consent to health care the parent does not necessarily have the right to access that health information. Ensuring confidentiality in the delivery of health care services for adolescents is vital, because this may encourage adolescents to seek timely medical care and provide complete information when they do seek care.\n\nPREP Pearls \n• When providing care to adolescents, 2 important concepts must be kept in mind: (1) consent—who is authorized to give consent for care, and (2) confidentiality—who has the right to control the release of information. \n• Emancipated minors have adult status and can give full consent for their own health care.  \n• Criteria for emancipation typically include marriage, military service, or living apart from parents while being self‐supporting. \n\nABP Content Specifications(s) \n• Recognize factors that determine when parents may/should accompany their adolescent during medical visits \n• Recognize the circumstances that constitute an emancipated minor with regard to ability to accept or reject medical treatment \n\nSuggested Readings \n• Center for Adolescent Health and the Law. State minor consent laws: a summary, third edition. Center for Adolescent Health and the Law website. http://www.cahl.org/state-minor-consent-laws-a-summary-third-edition/. \n• English A, Gudeman R. Understanding legal aspects of care. In: Neinstein LS, Katzman DK, Callahan T, Gordon CM, Joffe A, Rickert V, eds. Neinstein's Adolescent and Young Adult Health Care: A Practical Guide. 6th ed. Philadelphia, PA: Wolters Kluwer; 2016:90-94."}
{"id" : 2516, "question_text" : "A 12-day-old male neonate in the neonatal intensive care unit develops apnea, bradycardia, and oxygen desaturation requiring an increase in continuous positive airway pressure ventilation (CPAP) from 6 to 8 cm H2O, and fraction of inspired oxygen (FiO2) from 0.23 to 0.40. His mean upper limb cuff blood pressure has ranged from 18 to 25 mm Hg for the past hour. The neonate was born at 26 weeks' gestation with a birthweight of 940 g. Delivery was by cesarean section due to maternal preeclampsia with rupture of membranes at delivery. The neonate has been tolerating nasogastric feedings of 20 calorie/oz breast milk at 80 mL/kg/day and receiving parenteral nutrition through a central intravenous line. Laboratory data are shown: Laboratory Test Result Arterial blood gas base deficit 10 White blood cell count 22,000/µL (22.0 × 109/L) Neutrophils 40% Bands 25% Lymphocytes 20% Monocytes 11% Eosinophils 4% Hemoglobin 10.8 g/dL (108 g/L) Hematocrit 30.5% Platelet count 87 × 103/µL (87 × 109/L) Blood cultures are pending. Of the following, the BEST treatment for this neonate is intravenous", "options" : "[\"ampicillin and cefotaxime\", \"ampicillin and gentamicin\", \"meropenem and oxacillin\", \"vancomycin and gentamicin\"]", "explanation" : "The preterm neonate in the vignette has signs and symptoms of late-onset sepsis with clinical deterioration and an elevated white blood cell count with a left shift. The most likely causative organisms are coagulase-negative staphylococci and gram-negative bacteria. Of the response choices, the combination of vancomycin (effective against coagulase-negative staphylococci) and gentamicin (effective against gram-negative bacteria such as Escherichia coli and Klebsiella pneumoniae) is the best treatment pending blood culture results.\n\nAmpicillin, gentamicin, cefotaxime, and meropenem are all effective against gram-negative bacteria, but they are not effective against coagulase-negative staphylococci. Oxacillin is also not effective against coagulase-negative staphylococci. Ampicillin and gentamicin is the empiric antibiotic combination of choice in early-onset sepsis and is effective against the common bacterial causes of early onset sepsis (eg, group B Streptococcus, E coli, and Listeria monocytogenes).\n\nThis neonate's risk factors for sepsis include extreme prematurity, presence of an endotracheal tube and central line, and parenteral nutrition. Extremely preterm neonates in the neonatal intensive care unit are at increased risk for infection due to immature defense barriers (eg, skin and mucous membranes), an immature immune system, invasive interventions (eg, mechanical ventilation, intravascular catheters, and parenteral nutrition), and comorbidities (eg, patent ductus arteriosus, necrotizing enterocolitis, and chronic lung disease). The more premature the neonate, the higher the risk of infection.\n\nSepsis should always be considered as a cause of clinical deterioration in a preterm neonate. Neonatal sepsis is classified as early-or late-onset. Early-onset sepsis is defined as sepsis in the first week after birth, and late-onset sepsis occurs 7 or more days after birth. Early-onset sepsis is most often caused by maternal vertical transmission of microorganisms; late-onset sepsis is most often postnatally acquired.\n\nPREP Pearls\n• Risk factors for late-onset sepsis in preterm neonates include immature defense barriers, an immature immune system, invasive interventions, and comorbidities of prematurity.\n• The choice of empiric antibiotics for late-onset sepsis in a preterm neonate should be based on the most likely causative organisms, which include coagulase-negative staphylococci and gram-negative bacteria.\n\nABP Content Specifications(s)\n• Plan appropriate antimicrobial therapy for suspected sepsis in the immediate newborn period\n\nSuggested Readings\n• Bentlin MR, Rugolo, LMSS. Late-onset sepsis: epidemiology, evaluation and outcome. NeoReviews. 2010;11(8):e426e435. doi:10.1542/neo.11-8-e426.\n• Camacho-Gonzalez A, Spearman PW, Stoll BJ. Neonatal infectious diseases: evaluation of neonatal sepsis. Pediatr Clin North Am. 2013;60(2):367-389. doi:10.1016/j.pcl.2012.12.003.\n• Chu A, Hageman JR, Schreiber M, Alexander K. Antimicrobial therapy and late-onset sepsis. NeoReviews. 2012;13(2):e94-e102. doi:10.1542/neo.13-2-e94.\n• Kojaoghlanian T. The newborn at risk of infection. In: McInerny TK, Adam HM, Campbell DE, DeWitt TG, Foy JM, Kamat DM, eds. American Academy of Pediatrics Textbook of Pediatric Care. American Academy of Pediatrics; 2021:chap 102. Accessed September 1, 2022. Pediatric Care Online.\n• Puopolo KM, Benitz WE, Zaoutis TE; Committee on Fetus and Newborn, Committee on Infectious Diseases. Management of neonates born at less than or equal to 34 6/7 weeks' gestation with suspected or proven early-onset bacterial sepsis. Pediatrics. 2018;142(6):e20182896. doi:10.1542/peds.2018-2896."}
{"id" : 2714, "question_text" : "A right orchiectomy and left orchidopexy were performed on a 1-day-old boy for in utero right testicular torsion. Postoperatively, he was returned to the neonatal intensive care unit with normal vital signs in room air. Six hours after surgery, he was minimally responsive and hypotonic, with pinpoint pupils. A narcotic overdose was suspected. A review of the medication list revealed that while in the postanesthesia care unit the neonate received 3.6 mg of intravenous morphine for pain instead of 0.36 mg. The resident gave a verbal order, documented in the medical record, for 0.1 mg/kg of morphine. The neonate's weight is 3.6 kg. Appropriate doses of naloxone were administered twice during a 4-hour period. Five hours after treatment with naloxone, he was awake, breastfeeding, and active. The medical error was disclosed to the parents by the attending physician. Of the following, the health care team's BEST next step in response to this event is to", "options" : "[\"implement a policy of no verbal orders\", \"implement an error prevention campaign\", \"notify the risk management team\", \"provide support and counseling\"]", "explanation" : "Correct Answer: D\nIn the United States, 1% to 3% of pediatric hospital admissions are complicated by medical errors. Medication error is the most common adverse event in the pediatric population, with an error present in more than 5% of pediatric medication orders, in part due to weight-based dosing. It is imperative for health care practitioners to be aware of the potential for medical error and to be prepared, when an error occurs, to disclose the error to the patient and family. Training in error disclosure methods is helpful in developing the skills to best deliver this difficult news. Practitioners have an obligation to disclose an adverse event caused by a medical error. Failure to disclose can erode trust in the patient–practitioner relationship. Disclosure also provides the opportunity to identify and address problems to prevent error recurrence, thereby leading to improved patient safety. Since the publication of the National Academy of Medicine (formerly Institute of Medicine) 1999 report, \"To Err is Human: Building a Safer Health System,\" there is not only a greater national awareness of patient safety risks but also a shift toward transparency and systems error prevention.\n\nThe emotional and psychological impact of a medical error reaches not only the patient and family but also the health care practitioners involved. A health care practitioner may become the \"second victim,\" experiencing emotional distress after a patient adverse event. Therefore, it is imperative to provide support and counseling to the patient, family, and health care practitioner; institutions should ensure that there are mechanisms in place for this. Approximately 50% of health care practitioners will experience a \"second victim\" phenomenon in their career. Although disclosure is often stressful for the practitioner, in the long term it may help alleviate guilt and anxiety surrounding the error and decrease the risk of \"second victim\"–related trauma.\n\nAlthough a no verbal orders policy might appear desirable, in certain situations a strict policy can delay urgently needed care. Therefore, most hospitals have policies that limit the use of verbal orders but permit them in specific circumstances. Notifying risk management of an error may be advisable, but providing support should be the immediate priority. An error prevention campaign may be recommended as an outcome of a root cause analysis but will happen later in the process.\n\nPREP Pearls\n• Medication errors are the most common adverse event in pediatrics.\n• Disclosure of an adverse event to a patient and family is imperative and should occur at the earliest opportunity.\n• Support and counseling should be provided to the patient, family, and health care practitioner after an adverse event. The practitioner is often the \"second victim,\" experiencing emotional distress following a patient adverse event.\n\nABP Content Specifications(s)\n• Use appropriate means to disclose medical errors to patients\n• Apply appropriate methods of support for patients and their families after an error producing medical harm occurs\n• Use appropriate methods of support for physicians and other health-care providers after an error producing medical harm occurs\n\nSuggested Readings\n• Busch IM, Moretti F, Campagna I, et al. Promoting the psychological well-being of healthcare providers facing the burden of adverse events: a systematic review of second victim support resources. Int J Environ Res Public Health. 2021;18(10):5080. doi:10.3390/ijerph18105080.\n• Edrees H, Connors C, Paine L, Norvell M, Taylor H, Wu AW. Implementing the RISE second victim support programme at the Johns Hopkins Hospital: a case study. BMJ Open. 2016;6(9):e011708. doi:10.1136/bmjopen-2016-011708.\n• McDonnell WM, Altman RL, Bondi SA, et al; Committee on Medical Liability and Risk Management, Council on Quality Improvement and Patient Safety. Disclosure of adverse events in pediatrics. Pediatrics. 2016;138 (6):e20163215. doi:10.1542/peds.2016-3215.\n• Mueller BU, Neuspiel DR, Fisher ERS; Council on Quality Improvement and Patient Safety, Committee on Hospital Care. Principles of pediatric patient safety: reducing harm due to medical care. Pediatrics. 2019;143(2):e20183649. doi:10.1542/peds.2018-3649.\n• Shaikh SK, Cohen SP. Disclosure of medical errors. Pediatr Rev. 2020;41(1):45–47. doi:10.1542/pir.2018-0228."}
{"id" : 1616, "question_text" : "You are called to the newborn nursery to evaluate a 1-day-old newborn who has developed progressive respiratory distress. There were no complications, illnesses, or toxic exposures during the pregnancy. Prenatal care was appropriate, and no abnormalities were noted on routine screening or prenatal ultrasonography at 18 weeks of gestation. Maternal screening was negative for perinatal infectious diseases. Labor and delivery were uncomplicated. The amniotic fluid was clear, and the Apgar scores were 8 and 9 at 1 and 5 minutes, respectively. By 2 hours after birth, the neonate took 15 mL of formula without difficulty. Subsequent feedings have taken longer, and by 12 hours of age, he was no longer interested in feeding. He has had occasional cough and spit-ups, but no increase in oral secretions. Since then, he has had progressive tachypnea and now is grunting. His temperature is 37°C, heart rate is 180 beats/min, respiratory rate is 60 breaths/min, blood pressure is 60/40 mm Hg, and oxygen saturation is 90% on room air. Physical examination reveals a male newborn in moderate respiratory distress. His skin has a grayish appearance. He has intercostal retractions and is grunting. His lungs have crackles bilaterally with good air entry. He has a grade 3 continuous murmur that is heard throughout the precordium. His liver edge is palpable 3 cm below the right costal margin. His extremities are cool, with capillary refill time of 4 seconds.\n\nOf the following, the diagnostic step MOST likely to reveal the boy's diagnosis is", "options" : "[\"arterial blood gas analysis\", \"chest radiography\", \"echocardiography\", \"pre- and postductal pulse oximetry\", \"urine organic acids\"]", "explanation" : "The neonate in the vignette presents with shock, hypoxia, and respiratory failure that rapidly progressed over the first day after birth. Because he does not have risk factors for or signs of sepsis, the most likely cause of his condition is congenital heart disease with ductal-dependent systemic blood flow. The diagnostic test most likely to reveal the boy's diagnosis is echocardiography.\n\nShock is the failure of circulation or cellular respiration to meet the metabolic demands of end organs. Oxygen delivery is equal to the product of oxygen content (oxygen bound to hemoglobin plus oxygen dissolved in blood) and cardiac output (stroke volume multiplied by heart rate). Stroke volume is affected by preload, afterload, and cardiac contractility.\nCardiogenic shock occurs when cardiac output is decreased because of primary pump failure. Causes include arrhythmias resulting in either inadequate preload or low heart rate, hypertension or obstructive lesions causing high afterload, and primary defects in contractility. Arrhythmias often occur in the context of family history, such as Wolff-Parkinson-White and long QT syndromes, or other conditions causing electrolyte disturbances. Pericardial tamponade can be caused by trauma or inflammatory conditions such as inflammatory arthritis and systemic lupus erythematosus.\n\nSpecific etiologies of cardiogenic shock vary by age. Myocarditis and dilated cardiomyopathy can occur in any pediatric age group. Single ventricle congenital heart disease can manifest as shock in the neonatal period, as in the case of the child in the vignette. The boy has hypoplastic left heart syndrome, in which the left ventricle does not develop, and the aortic arch and/or mitral valve annulus is often atretic. The right ventricle, therefore, supplies both the pulmonary circulation and systemic circulation via the ductus arteriosus, which inserts into the distal arch. This is known as ductal-dependent systemic blood flow. As the ductus arteriosus begins to close, systemic circulation decreases and shock ensues. Although there is mixing of deoxygenated and oxygenated blood, there is enough pulmonary blood flow such that cyanosis is not always obvious. Affected infants appear gray, poorly perfused, and tachypneic. Rales and hepatomegaly are common. The treatment for a neonate with ductal-dependent circulation is to start a prostaglandin infusion, which opens the ductus arteriosus. This therapy should be started without delay if a ductal-dependent lesion is suspected, even before echocardiography results are available. If an alternative diagnosis such as sepsis or inborn error of metabolism is certain, prostaglandins are not indicated.\n\nCongenital heart disease lesions with left-to-right shunting, such as ventricular septal defects, atrioventricular canal defects, and anomalous pulmonary venous return often manifest at approximately 2 months of age. As the pulmonary vascular resistance decreases, the amount of shunted blood increases. Because the left ventricle pumps blood through the shunt, as well as the forward systemic flow, congestive heart failure ensues.\n\nFor cases of cardiogenic shock, the most important consideration for clinicians is timely recognition. Some clinical signs and symptoms of heart failure in adults are not reliable in children, such as chest pain, orthopnea, jugular venous distention, and peripheral edema. Heart failure in children is often heralded by less concerning signs and symptoms such as poor feeding, vomiting, or difficulty breathing, masquerading as less serious gastrointestinal or respiratory illnesses. It is thus important for the clinician to maintain a high index of suspicion for cardiac illness, paying special attention to tachycardia, narrow pulse pressure, cool extremities, and hepatomegaly.\n\nAlthough arterial blood gas analysis is useful in children with shock, to detect problems with circulation, acid-base balance, and ventilation, it is not specific enough to diagnose single ventricle heart disease. Chest radiography and pre- and postductal pulse oximetry are important components of the workup of congenital heart disease, but are also not specific enough to make the diagnosis. Urine organic acids can be helpful in the workup of a neonate in shock, but the most likely diagnosis for the neonate in the vignette is congenital heart disease.\n\nPREP Pearls\n• Heart failure in children is often heralded nonspecific signs and symptoms such as poor feeding, vomiting, or difficulty breathing.\n• Congenital heart disease with left to right shunting, such as ventricular septal defects, atrioventricular canal defects, and anomalous pulmonary venous return often manifest with heart failure at approximately 2 months of age.\n• A prostaglandin infusion should be started immediately for any neonate with shock, unless ductal-dependent systemic blood flow lesions are excluded.\n\nMOCA-Peds Objective\n• Respond to abnormal results of congenital heart disease screening in a neonate\n\nABP Content Specifications(s)\n• Recognize findings associated with cardiogenic shock in children of various ages\n• Plan an appropriate diagnostic evaluation of cardiogenic shock\n\nSuggested Readings\n• Bhat BV, Plakkal N. Management of shock in neonates. Indian J Pediatr. 2015;82(10):923–929. doi: http://dx.doi.org/10.1007/s12098-015-1758-7.\n• Hsu DT, Pearson GD. Heart failure in children: part I, history, etiology, and pathophysiology. Circ Heart Fail. 2009;2:63–70. doi: http://dx.doi.org/10.1161/CIRCHEARTFAILURE.108.820217.\n• Reynolds HR, Hochman JS. Cardiogenic shock: current concepts and improving outcomes. Circulation. 2008;117(5):686–697. doi: http://dx.doi.org/10.1161/CIRCULATIONAHA.106.613596."}
{"id" : 174, "question_text" : "You are seeing a 12-year-old boy for follow-up of problematic handwashing and bathing routines. He has been receiving cognitive behavior therapy for 12 weeks and has had minimal improvement. He has always been somewhat of a perfectionist and rigid, but recently has begun to have persistent fears of germs. The routines are time-consuming and embarrassing, sometimes causing him to be late for school. He is otherwise healthy, although he is beginning to have significant skin irritation on his hands.\n\nOf the following, the BEST next course of action is to initiate", "options" : "[\"alprazolam treatment\", \"clomipramine treatment\", \"propranolol treatment\", \"risperidone treatment\", \"sertraline treatment\"]", "explanation" : "The boy described in the vignette has evidence of obsessive-compulsive disorder (OCD). Although he has not shown the expected response, cognitive behavior therapy (CBT) is considered a first-line treatment. Use of a selective serotonin reuptake inhibitor (SSRI), such as fluoxetine, fluvoxamine, or sertraline, along with CBT has been associated with a superior response to either modality alone. He should continue CBT rather than searching for other therapeutic modalities.\n\nClomipramine (a tricyclic antidepressant) has good efficacy for OCD but is usually not a first-line treatment because of less tolerance and a greater need for medical monitoring. There is no evidence that benzodiazepines such as alprazolam are effective in the treatment of pediatric OCD. In addition, significant risks are associated with the use of benzodiazepines, including disinhibition and agitation. Neuroleptics, such as risperidone, may be used as adjuncts to SSRIs for those who are refractory to attempts at treatment, but they also have worrisome adverse effects (metabolic and extrapyramidal) that require clinical and laboratory monitoring. Propranolol is sometimes used as a treatment for performance anxiety, but it is not a recommended treatment for OCD.\n\nAAP Mental Health Competency: Know how to treat obsessive-compulsive disorder (OCD) in children"}
{"id" : 716, "question_text" : "A 7-year-old boy presents to your office with a 3 -day history of fever and joint pain. His parents recall a sore throat 3 weeks ago. Findings on physical examination reveal swelling and erythema of his right knee and ankle. Yesterday, he had swelling and erythema of his right wrist and left knee that have since resolved. His temperature is 38.7°C, and the remainder of his vital signs is normal. Examination of his skin reveals a rash (Item Q8). His erythrocyte sedimentation rate is 30 mm/h and a rapid streptococcal test result is negative. Of the following, the MOST appropriate therapy for this patient is", "options" : "[\"azithromycin\", \"ceftriaxone\", \"naproxen\", \"penicillin G\", \"prednisone\"]", "explanation" : "The patient described in the vignette has fever, erythema marginatum, and a polyarticular migratory arthritis. This constellation of features is consistent with acute rheumatic fever (ARF). Acute rheumatic fever is a major cause of acquired heart disease worldwide; however the incidence of acute rheumatic fever has been declining in the United States. ARF is an autoimmune disease that follows an infection with group A streptococci (GAS) and can lead to rheumatic heart disease in patients that are not treated for streptococcal pharyngitis. ARF is diagnosed by the Jones criteria (Item C8A).\n\nThe most common features of ARF in the United States are carditis and arthritis. The cardiac manifestations of ARF present as mitral or aortic insufficiency, pericarditis, pericardial or myocardial involvement, and valvulitis. The arthritis associated with ARF is classically described as an early polyarticular arthritis that migrates. Often, the arthritis will completely resolve in one joint and appear in another joint. The arthritis is nondeforming and most commonly affects large joints such as the elbows, wrists, knees, and ankles. The arthritis is exquisitely painful and responds well to aspirin and other anti-inflammatory medications. Often, throat culture is negative by the time the patient presents with symptoms; however, it is recommended that a patient with active symptoms of ARF be treated with Penicillin G or 10 days of oral penicillin to eliminate residual GAS infection, even if the throat culture is negative. Treatment of ARF also includes anti-inflammatory medications, restriction of activity, and antibiotic prophylaxis to prevent progression of cardiac disease (Item C8B) Item C8C\n\nPenicillin G is the drug of choice to treat any residual GAS infection. Azithromycin can treat GAS infections but is a second-line therapy. Ceftriaxone does not provide good coverage for a GAS infection. While nonsteroidal anti-inflammatory drugs such as naproxen can be used to treat the arthritis associated with ARF, they are second-line agents. Aspirin would be the first-line treatment choice. Glucocorticoids such as prednisone are not recommended in the treatment of ARF except in cases of severe carditis, and even then the evidence for effectiveness is weak.\n\nItem C8A. Jones Criteria\nConfirmation of preceding Group A Streptococcus by positive throat culture, positive rapid antigen test, elevated or rising antistreptolysin O titer\nAND\n2 major manifestations\nOR\n1 major and 2 minor manifestation\n\nMajor Manifestation\n• Polyarthritis\n• Carditis\n• Subcutaneous nodules\n• Sydenham chorea\n• Erythema marginatum\n\nMinor Manifestations\n• Fever\n• Arthralgia\n• Prolonged PR interval on electrocardiogram\n• Elevated acute phase reactants (erythrocyte sedimentation rate, C-reactive protein)\n\nItem C8B. Antibiotic Prophylaxis Recommended to Prevent Recurrence of Rheumatic Fever\nAgent - Dose - Mode\nBenzathine penicillin G - 27 kg (60 Ibs), 600,000 U, >27 kg (60 Ibs) 1,200,000 U - Intramuscular every 4 weeks\nPenicillin V - 250 mg twice daily - Oral\nSulfadiazine - 27 kg (60 Ibs), 0.5 g once daily >27 kg (60 lbs) 1.0 g once daily - Oral\nMacrolide or azalide* - Varies - Oral\n* If allergic to penicillin and sulfadiazine.\nReprinted with permission from Gerber MA, Baltimore RS, Eaton CB, et al. Prevention of rheumatic fever and diagnosis and treatment of acute streptococcal pharyngitis: a scientific statement from the American Heart Association Rheumatic Fever, Endocarditis, and Kawasaki Disease Committee of the Council on Cardiovascular Disease in the Young, the Interdisciplinary Council on Functional Genomics and Translational Biology, and the Interdisciplinary Council on Quality of Care and Outcomes Research, endorsed by the American Academy of Pediatrics. Circulation. 2009;119(11):1541-1551\n\nItem C8C. Duration of Secondary Rheumatic Fever Prophylaxis\nCategory - Duration after last attack\nRheumatic fever with carditis and residual heart disease (persistent valvular disease by echocardiogram or clinical evidence) - 10 years or until 40 years of age (whichever is longer), sometimes lifelong prophylaxis\nRheumatic fever with carditis but no residual heart disease (no valvular disease on echocardiogram or clinical evidence ) - 10 years or until 21 years of age (whichever is longer)\nRheumatic fever without carditis - 5 years or until 21 years of age (whichever is longer)\nReprinted with permission from Gerber MA, Baltimore RS, Eaton CB, et al. Prevention of rheumatic fever and diagnosis and treatment of acute streptococcal pharyngitis: a scientific statement from the American Heart Association Rheumatic Fever, Endocarditis, and Kawasaki Disease Committee of the Council on Cardiovascular Disease in the Young, the Interdisciplinary Council on Functional Genomics and Translational Biology, and the Interdisciplinary Council on Quality of Care and Outcomes Research, endorsed by the American Academy of Pediatrics. Circulation. 2009;119(11):1541-1551\n\nPREP Pearls\n• Throat cultures are often negative by the time symptoms of ARF are present.\n• The arthritis of ARF is a nondeforming, migratory polyarthritis that involves the large joints.\n• Long-term antibiotic prophylaxis is used in ARF patients to prevent worsening cardiac disease.\n\nAmerican Board of Pediatrics Content Specification(s):\n• Know the characteristics of arthritis associated with rheumatic fever\n\nSuggested Reading:\n• Gerber MA, Baltimore RS, Eaton CB, et al. Prevention of rheumatic fever and diagnosis and treatment of acute streptococcal pharyngitis: a scientific statement from the American Heart Association Rheumatic Fever, Endocarditis, and Kawasaki Disease Committee of the Council on Cardiovascular Disease in the Young, the Interdisciplinary Council on Functional Genomics and Translational Biology, and the Interdisciplinary Council on Quality of Care and Outcomes Research, endorsed by the American Academy of Pediatrics. Circulation. 2009;119(11):1541-1551. doi:10.1161/CIRCULATIONAHA\n• John J, Chandran L. Arthritis in children and adolescents. Pediatr Rev. 2011;32(11):470-480. doi:10.1542/pir.32-11-470\n• Steer AC, Carapetis JR. Acute rheumatic fever and rheumatic heart disease in indigenous populations. Pediatr Clin N Am. 2009;56(6):1401-1419. doi:10.1016/j.pc1.2009.09.011"}
{"id" : 2225, "question_text" : "A 15-year-old has been having double vision for about 3 months. He was previously seen by an ophthalmologist and was prescribed glasses, which temporarily improved his vision. Since then, he has developed persistent headaches, for which acetaminophen provides mild relief. He has dizziness when standing up from sitting or lying down, followed by what he describes as a \"blackout\" and tunnel vision. He has not experienced nausea or vomiting, change in his appetite or energy level, or recent acute illnesses. His father recently noticed a change in the boy's upward gaze. Neuroimaging of the head was obtained with results concerning for a germ cell tumor. Of the following, the tumor markers that will be MOST helpful in determining this child's diagnosis are", "options" : "[\"\\u03b1-fetoprotein and anaplastic lymphoma kinase\", \"\\u03b2-human chorionic gonadotropin and \\u03b1-fetoprotein\", \"\\u03b2-human chorionic gonadotropin and cancer antigen-125\", \"carcinoembryonic antigen and cancer antigen-125\"]", "explanation" : "Germ cell tumors occur along midline structures; identification of a midline lesion should prompt evaluation for a germ cell tumor.\n\nThe tumor markers β-human chorionic gonadotropin and α-fetoprotein may be used to help diagnose and monitor for recurrence of germ cell tumors.\n\nCritique\nThe boy described in the vignette has a constellation of signs and symptoms that raise concern regarding intracranial pathology: diplopia, headache, vision changes, and abnormal upward gaze. For all children with such signs and symptoms, completing a thorough physical examination (including a complete neurologic examination) is essential. This can help localize possible central nervous system lesions, as well as inform the differential diagnosis. Table 1 summarizes signs and symptoms that may be associated with germ cell tumors in various locations. This adolescent's finding of abnormal upward gaze raises suspicion regarding a pineal lesion. Of the response choices, β-human chorionic gonadotropin and α-fetoprotein are the tumor markers that would be most helpful in determining this patient's diagnosis.\n\nCentral nervous system (CNS) germinoma, a type of germ cell tumor (GCT), is commonly found in the pineal region. Germ cell tumors arise from primordial germ cells, which ultimately develop into sperm and ova. As they migrate to their intended destinations, their travel may arrest along the path leading to tumor growth, usually occurring in midline areas: pineal gland, mediastinum, retroperitoneum, sacrococcygeal region, ovary, and testis. Central nervous system germ cell tumors fall into 2 main categories: germinomas and nongerminomatous germ cell tumors.\n\nExtracranial GCTs occur along other midline structures. They can be gonadal (in the ovary or testis) or extragonadal. These tumors may also be categorized on the basis of histology. Teratomas, the most common GCTs, may be mature or immature. Mature teratomas, the more common and benign form, are composed of tissue from all 3 germ cell layers (usually skin, adipose, and intestinal tissue). Immature teratomas are more likely to be malignant. Location and histology are used to classify malignant GCTs, which may include yolk sac tumors, germinomas, embryonal carcinomas, choriocarcinomas, or gonadoblastomas. Mixed GCTs have 2 or more types of histologies.\n\nWhen there is clinical suspicion of a CNS tumor, imaging should be obtained to confirm the diagnosis and further characterize the lesion. For the boy in the vignette, the best imaging modality would be brain magnetic resonance imaging (MRI) with and without contrast. However, it is often difficult to obtain an MRI immediately. Therefore, brain computed tomography (CT) is often the best available initial imaging study to promptly identify a lesion, its location, and associated findings requiring immediate intervention (eg, hemorrhage, herniation, or hydrocephalus). Appropriate initial imaging of the primary site for suspected tumors at various locations includes the following:\nMediastinum: CT/MRI of the chest and upper abdomen\nOvary/testis: sonogram, CT/MRI of the abdomen/pelvis\nSacrococcygeal region: CT/MRI of the abdomen/pelvis\n\nImaging to assess for evidence of metastasis may be required (eg, chest CT, chest radiography, or bone scan). Common areas of metastasis include lungs, liver, bone, CNS, and regional lymph nodes.\n\nTumor markers are useful in evaluation for potential malignancies. For GCT diagnosis and monitoring for disease recurrence, the two most common markers used are β-human chorionic gonadotropin (β-hCG) and α-fetoprotein (AFP). Beta-human chorionic gonadotropin is produced by placenta tissue, and AFP originates from the yolk sac and embryonic liver. Table 2 shows tumor marker findings seen in different types of GCTs. For CNS GCTs, β-human chorionic gonadotropin and AFP can be detected in the cerebrospinal fluid. In infants, AFP concentrations vary and should be interpreted by using an age-based nomogram. Although cancer antigen-125 is a tumor marker used in evaluation for ovarian cancer, it is not currently used in the diagnosis or monitoring of ovarian GCTs. Carcinoembryonic antigen is used as a tumor marker for colorectal or pancreatic cancer, but not for GCTs. Anaplastic lymphoma kinase is a tumor marker mainly seen in anaplastic large cell lymphoma and non–small cell lung cancer.\n\nThe definitive diagnosis of a GCT requires obtaining tissue for histologic diagnosis. Treatment consists of surgery, chemotherapy, and in some cases radiotherapy. Some disorders, mainly disorders of sexual development, may increase an individual's risk of developing a GCT. These include Swyer, Klinefelter, and Turner syndromes.\n\nSuggested Reading(s)\nAllen-Rhoades W, Whittle SB, Rainusso N. Pediatric solid tumors in children and adolescents: an overview. Pediatr Rev. 2018;39(9):444-453. doi:10.1542/pir.2017-0268\nCancers in childhood. Point-of-care Quick Reference. Pediatric Care Online. January 1, 2019. Accessed September 1, 2024. Pediatric Care Online\nFonseca A, Olson TA. Extracranial germ cell tumors. In: Lanzkowsky P, Lipton J, Fish JD, eds. Lanzkowsky's Manual of Pediatric Hematology and Oncology. 7th ed. Elsevier; 2022: 597-611.\nHanson D, Atlas MP. Central nervous system tumors. In: Lanzkowsky P, Lipton J, Fish JD, eds. Lanzkowsky's Manual of Pediatric Hematology and Oncology. 7th ed. Elsevier; 2022:485-505.\nPehlivan KC, Paul MR, Crawford JR. Central nervous system tumors in children. Pediatr Rev. 2022;43(1):3-15. doi:10.1542/pir.2020-004499\n\nContent Domain\nOncology\n\nLearning Objectives\nRecognize clinical findings and tumor marker results consistent with a germ cell tumor"}
{"id" : 325, "question_text" : "During a health supervision visit, the mother of 8-year-old girl and 10-year-old boy tells you that the children have been receiving counseling for anxiety for 3 months. The mother has a history of anxiety, and she has responded well to sertraline. She reports that the boy is not making much progress in therapy and asks you if a medication trial would be appropriate for him. Of the following, the MOST appropriate initial response is to", "options" : "[\"advise the mother to give the current therapist more time to see if an improvement occurs\", \"ask the boy if he wants a new therapist\", \"obtain consent from the mother to contact the therapist\", \"prescribe sertraline for the boy\", \"refer the family to another therapist\"]", "explanation" : "Significant advances have been made in the assessment and treatment of anxiety disorders in children and adolescents. Evidence-based practices are now available that can help to address anxiety disorders in a timely fashion. However, the specific intervention depends on the anxiety disorder that is present. For example, a child who has posttraumatic stress disorder might benefit from trauma-focused cognitive behavior therapy, but the child who has school phobia might require a different approach, such as a graded exposure plan for reintroduction and accommodation to being in school. Characterizing the type of anxiety might be assisted by the use of anxiety-specific rating scales, such as the Screen for Child Anxiety Related Emotional Disorders (SCARED). For the boy described in the vignette, the nature of the anxiety disorder has not been defined. Accordingly, the clinician should contact the therapist to clarify the diagnosis and the type and goals of therapy that is being used for this child. Recommending that a family continue with a therapist without first determining that they are receiving treatment that is appropriate for their child's issues is not fair to the patient or family.\n\n\"Liking\" a therapist can be a factor in the effectiveness of psychotherapy, but a child's feelings regarding the therapy may simply mean that the therapist is pushing for necessary changes that are uncomfortable. Doing \"exposure\" work for fears in anxiety treatment, for example, can be temporarily uncomfortable and places a new demand on the family's home time.\n\nStarting sertraline because the family perceives that the child is not making progress may not be indicated because it is unclear if this boy has an anxiety disorder and if so, whether it is being treated appropriately. Beginning a selective serotonin reuptake inhibitor simultaneously with starting therapy is appropriate for children who have moderate severe anxiety disorders and for children who have not responded to an adequate trial of appropriate counseling therapy alone.\n\nRefer the family to another therapist before communicating with the current therapist is not appropriate. Changing therapists may undercut the hard work of a competent therapist using appropriate treatments. Being familiar with community mental health resources can aid the pediatrician in making successful referrals and obtaining follow-up information on their patients.\n\nAAP Mental Health Competency:\n\nKnow how to counsel a parent/child that are doubting the effectiveness of a current counseling therapy"}
{"id" : 3724, "question_text" : "A 15-month-old boy is brought to the urgent care center after an episode during which he stopped breathing, turned blue, became limp, and stiffened. The episode lasted for about 1 minute, after which, he was very sleepy. The boy had been playing in a park when he fell, scraped his knee, and immediately cried. He then became apneic, cyanotic, and limp; lost consciousness; and developed stiffness of his upper and lower extremities. He has had no recent illness. There is no known family history of syncope, sudden death, or epilepsy. Of the following, based on this boy's history, the MOST likely diagnosis is", "options" : "[\"benign myoclonus\", \"breath-holding spell\", \"long QT syndrome\", \"seizure\"]", "explanation" : "The child in the vignette had a syncopal episode because of a breath-holding spell (BHS). Breath-holding spells are common events in children from 6 months to 4 years of age; 90% have their first episode before 18 months of age. Approximately 5% of the pediatric population may experience BHS, which are slightly more common in girls. The pathogenesis of BHS is unclear, though some data support a primary role for dysfunction of the autonomic nervous system or delayed maturation in myelination of the brainstem. A family history of BHS is present in approximately 50% of patients, and autosomal dominant inheritance has been reported in some families. Iron-deficiency anemia is more prevalent in children with BHS compared with controls and may contribute to the occurrence of BHS and underlying dysautonomia. An association has also been described between BHS and other types of anemia, including transient erythroblastopenia of childhood.\n\nBreath-holding spells can be extremely frightening for parents. While many consider BHS a form of \"attention-seeking\" behavior, these spells are an involuntary reflex that cannot be controlled and occur in developmentally normal children. There are 2 clinical types of BHS, cyanotic and pallid.\n\nBreath-holding spells may be difficult to distinguish from seizures. Although there is no diagnostic test that confirms BHS, a history of provocation (head injury, crying, etc) should be elicited, which is typical for BHS but not seizures. Prominent color change (pallor or cyanosis) is also suggestive of BHS rather than seizures. Video electroencephalography may be helpful to distinguish between seizure episodes and BHS.\n\nThe prognosis for children with BHS is excellent, with remission occuring at a mean age of 4 years; virtually all children with BHS stop having episodes by 8 years of age. Neurologic development is normal. Because iron deficiency is highly associated with BHS, evaluation with complete blood cell count and serum ferritin is warranted. Iron supplementation with ferrous sulfate, 5 to 6 mg/kg per day, seems to reduce the frequency of BHS in anemic or iron-deficient children. Antiepileptic drugs are not helpful in reducing the occurrence of BHS. Rarely, patients with severe attacks of pallid BHS associated with prolonged, severe bradycardia may need atropine or cardiac pacing to reduce spell frequency.\n\nBenign myoclonus of infancy manifests as myoclonus, spasm with brief tonic contractions, shuddering or atonia, frequently occuring at mealtime between 3 to 8 months of age and experiencing spontaneous remission at 2 to 3 years of age.\n\nOther causes of syncope should be considered if episodes are prolonged or frequent, precipitated by startle or other nontraumatic stimuli, or if a family history of syncope or sudden death exists. If these historical elements are present, one should consider a more in-depth cardiac evaluation, particularly for long QT syndrome.\n\nPREP Pearls\n• Breath-holding spells are triggered by sudden fear, pain, minor injury, frustration, or anger.\n• Breath-holding spells are an involuntary reflex that cannot be controlled and occur in developmentally normal children.\n• Iron deficiency is highly associated with breath-holding spells; evaluation with complete blood cell count and serum ferritin is warranted.\n\nABP Content Specifications(s)\n• Recognize the clinical features of breath-holding and counsel parents appropriately\n• Plan the appropriate management of breath-holding in toddlers and preschool-age children\n\nSuggested Readings\n• Breningstall GN. Breath-holding spells. Pediatr Neurol. 1996;14(2):91-97. doi:10.1016/0887-8994(96)00006-9.\n• DiMario FJ. Prospective study of children with cyanotic and pallid breath-holding spells. Pediatrics. 2001;107(2):265-269. doi:10.1542/peds.107.2.265.\n• Goldman RD. Breath-holding spells in infants. Can Fam Physician. 2015;61:149-150. http://www.cfp.ca/content/61/2/149.long.\n• Roddy SM. Nonconvulsive periodic disorders. In: McInerny TK, Adam HM, Campbell DE, DeWitt TG, Foy JM, Kamat DM, eds. American Academy of Pediatrics Textbook of Pediatric Care. 2nd ed. Elk Grove Village, IL: American Academy of Pediatrics; 2017:1517-1520. Pediatric Care Online.\n• Zehetner AA, Orr N, Buckmaster A, Williams K, Wheeler DM. Iron supplementation for breath-holding attacks in children. Cochrane Database Syst Rev. 2010;12(5):CD008132. doi:10.1002/14651858.CD008132.pub2."}
{"id" : 3587, "question_text" : "A previously healthy 5-month-old male infant is brought to the emergency department by his mother because of lethargy and fast breathing. He was in his normal state of health 12 hours prior to presentation. He has not had fevers, cough, or difficulty breathing, and he has been eating well and having normal bowel movements and urine output. The mother left the boy with another caregiver this morning. When she returned, the boy was lethargic and breathing fast. The caregiver denied any trauma and stated that these symptoms, as well as vomiting, occurred progressively over the day. The infant has a temperature of 37°C, heart rate of 95 beats/min, respiratory rate 50 breaths/min, blood pressure of 110/60 mm Hg, and oxygen saturation of 100% on room air. He appears to be well developed and well nourished. Upon painful stimuli, he does not open his eyes; he cries inconsolably and withdraws nonpurposefully. There are no external signs of trauma. The anterior fontanelle is full, and pupils are 3 mm, equal, and sluggish. He is breathing rapidly and deeply, with good air movement bilaterally and no wheezing or crackles. His abdomen is soft with no organomegaly. Computed tomography of the brain is performed (Item Q31). Of the following, the mechanism MOST likely to have led to this infant's symptoms is", "options" : "[\"cerebral venous sinus thrombosis\", \"middle cerebral artery thromboembolism\", \"rupture of the middle meningeal artery\", \"tearing of the intracranial bridging veins\"]", "explanation" : "Correct Answer: D\nThe infant in this vignette acutely developed altered mental status, increased minute ventilation, and neurologic findings of increased intracranial pressure. In the absence of an infectious prodrome of illness, abusive head trauma should be strongly suspected. Subdural hemorrhage is demonstrated on the computed tomography image shown in the vignette, and the cause is tearing of the intracranial bridging veins.\n\nFrom superficial to deep, the meningeal layers covering the brain include the dura, arachnoid, and pia. A subdural hematoma (SDH) is the accumulation of blood between the dura and the arachnoid. Since the dura straddles the sutures but does not cross the hemispheric fissure, computed tomography of the head (Item C31A) shows a crescentic pattern of density that crosses suture lines but does not cross the midline. A SDH is caused by tearing of the bridging veins, which drain the brain parenchyma into the dural cerebral venous sinuses. This disruption can be caused by blunt force trauma or acceleration-deceleration forces, either from a coup-contrecoup mechanism or from the brain's movement inside the head during shaking. This mechanism causes SDH most commonly in infants because they have less developed neck and trunk muscles to stabilize the head during the shaking, and because the head is larger relative to the rest of the body. Skull fractures may accompany SDH if there is also impact with a surface in addition to the shaking but are sometimes not present. Since these infants are less likely to be brought to medical attention, a longstanding pattern of abuse may be detected, such as healing rib or other bony fractures and brain hemorrhages of varying ages. In SDH, the blood collects under venous pressure; therefore, it often accumulates more slowly than an epidural hematoma (EDH) and can follow a more chronic clinical course. Children with increased intracranial pressure may present with respiratory distress (ie, breathing rapidly and deeply) as a result of increased minute ventilation to limit intracranial pressure. A high index of suspicion for child abuse should be maintained in infants with altered mental status, vomiting, and tachypnea. The constellation of subdural hematoma and retinal hemorrhages is pathognomonic of abusive head trauma.\n\nAn EDH is characterized by blood accumulating between the periosteum and the dura. Since the periosteum is attached to the sutures, computed tomography of the head (Item C31B) shows a lenticular pattern of density that does not cross the suture lines. A common injury mechanism for EDH is blunt force to the temporal bone, with or without skull fracture, and rupture of the middle meningeal artery. There can be a period of lucidity for a few hours after the injury while the blood is accumulating, followed by neurologic deterioration. An EDH can lead to uncal herniation and third cranial nerve palsy, clinically manifesting as an ipsilateral dilated and unreactive pupil. While other mechanisms of brain injury can certainly lead to malignant intracranial hypertension and death, in EDH, the presence of the blood itself can be fatal, because it collects under high pressure due to the arterial disruption. Thus, a high index of suspicion should be maintained in temporal skull trauma, especially with pupillary findings.\n\nFor the infant in this vignette, EDH from rupture of the middle meningeal artery was not a likely mechanism because of the absence of external signs of trauma and the radiographic appearance of the bleeding, which was crescentic shaped and crossed the suture lines. Stroke from middle cerebral artery thromboembolism can cause altered mental status and increased intracranial pressure, but there would likely be focal neurologic signs of weakness, and hemorrhagic conversion of stroke usually occurs in the brain parenchyma, not in the epidural space. Cerebral venous sinus thrombosis can also cause altered mental status but usually has a more indolent presentation and does not present with intracranial hemorrhage.\n\nPREP Pearls\n• The constellation of findings of subdural hematoma, rib fractures, and retinal hemorrhages in an infant is pathognomonic of child abuse.\n• A high index of suspicion for epidural hematoma should be maintained in temporal skull trauma, especially with pupillary findings (ipsilateral, dilated, and unreactive pupil), even in a lucid patient.\n• Subdural hematoma is caused by tearing of the intracranial bridging veins, which drain the brain parenchyma into the dural venous sinuses.\n\nABP Content Specifications(s)\n• Recognize the clinical findings associated with epidural hematoma, and manage appropriately\n• Recognize the clinical findings associated with subdural hematoma with and without skull fracture, and manage appropriately\n\nSuggested Readings\n• Atabaki SM. Pediatric head injury. Pediatr Rev. 2007;28(6):215-224. doi:10.1542/pir.28-6-215.\n• Hansen JB, Frazier T, Moffatt M, Zinkus T, Anderst JD. Evaluations for abuse in young children with subdural hemorrhages: findings based on symptom severity and benign enlargement of the subarachnoid spaces. J Neurosurg Pediatr. 2018;21(1):31-37. doi:10.3171/2017.7.PEDS17317.\n• Mahajan P, Indra S. Head injuries. In: McInerny TK, Adam HM, Campbell DE, DeWitt TG, Foy JM, Kamat DM, eds. American Academy of Pediatrics Textbook of Pediatric Care. 2nd ed. Elk Grove Village, IL: American Academy of Pediatrics; 2017:2862-2868. Pediatric Care Online.\n• Schunk JE, Schutzman SA. Pediatric head injury. Pediatr Rev. 2012;33(9):398-410. doi:10.1542/pir.33-9-398."}
{"id" : 2526, "question_text" : "A 5-year-old, previously healthy boy is seen in the clinic for evaluation of testicular pain and fever that started this morning. He had a cough and runny nose 2 weeks ago, which have resolved. He has no other symptoms and no history of trauma. On physical examination, the boy has a temperature of 38.5°C, heart rate of 117 beats/min, respiratory rate of 22 breaths/min, and oxygen saturation of 98% in room air. Both testicles are palpated in the scrotum and the cremasteric reflex is intact. The testicles appear swollen, with no abnormal coloring or masses palpated. Ultrasonography with Doppler shows normal blood flow to both testicles. Of the following, this boy's MOST likely diagnosis is", "options" : "[\"inguinal hernia\", \"postinfectious orchitis\", \"testicular torsion\", \"torsion of the appendix testis\"]", "explanation" : "Correct Answer: B\nThe boy in the vignette has fever, painful testicular swelling, and a normal cremasteric reflex. Of the response choices, these signs and symptoms are most consistent with orchitis. In this age group, orchitis is most commonly seen after viral infections. An inguinal hernia can cause scrotal pain and swelling but would be palpated during the genitourinary examination. Testicular torsion, a surgical emergency, is an unlikely diagnosis for the boy in the vignette given his normal blood flow on Doppler ultrasonography and normal cremasteric reflex. In the case of testicular torsion, the normal cremasteric reflex (retraction of the testis in response to touch on the upper thigh) is usually absent. Torsion of the appendix testis is also unlikely given the normal findings on ultrasonography and absence of a \"blue dot sign\" on physical examination.\n\nOrchitis (inflammation of the testis) and epididymitis (inflammation of the epididymis) have infectious or inflammatory etiologies. Symptoms and signs of orchitis and epididymitis include testicular swelling and tenderness, dysuria, urinary frequency and urgency, and often systemic symptoms (eg, fever). The etiology of orchitis and epididymitis varies by age. Children ages 2 to 13 years often have a postinfectious cause (most commonly Mycoplasma, enterovirus, or adenovirus). Vasculitis (eg, Henoch-Schönlein purpura) is another common cause of orchitis and epididymitis in this age group. Older children and adults are more likely to have an infectious etiology with organisms that cause urinary tract or sexually transmitted infections. A urinalysis may show the presence of nitrites and/or leukocyte esterase because the infection can start in the urine and ascend into the epididymis and testes.\n\nIt is important to differentiate testicular pain caused by orchitis and epididymitis from testicular torsion, which is an emergency. Testicular torsion can be difficult to diagnose based on physical examination findings alone. Therefore, ultrasonography with Doppler should be obtained for all children and adolescents with testicular pain. Ultrasonography with Doppler is not 100% specific; urgent surgical or urologic consultation should be obtained when there is concern for testicular torsion.\n\nPREP Pearls\n• All children and adolescents with testicular pain should have ultrasonography with Doppler performed to evaluate for testicular torsion.\n• Symptoms and signs of orchitis and epididymitis typically include testicular swelling and tenderness, dysuria, urinary frequency and urgency, and often systemic symptoms.\n\nABP Content Specifications(s)\n• Recognize the clinical findings associated with orchitis\n• Identify common causes of orchitis\n\nSuggested Readings\n• Palmer LS. Scrotal swelling and pain. In: McInerny TK, Adam HM, Campbell DE, DeWitt TG, Foy JM, Kamat DM, eds. American Academy of Pediatrics Textbook of Pediatric Care. American Academy of Pediatrics; 2021:chap 190. Accessed September 1, 2022. Pediatric Care Online.\n• Sexually Transmitted Infections Treatment Guidelines, 2021. Epididymitis. Centers for Disease Control and Prevention. Updated July 22, 2021. Accessed September 1, 2022. https://www.cdc.gov/std/treatment-guidelines/epididymitis.htm.\n• Stewart A, Ubee SS, Davies H. Epididymo-orchitis. BMJ. 2011;342:d1543. doi:10.1136/bmj.d1543.\n• Trojian TH, Lishnak TS, Heiman D. Epididymitis and orchitis: an overview. Am Fam Physician. 2009;79(7):583-587. https://pubmed.ncbi.nlm.nih.gov/19378875/.\n• Wu WJ, Gitlin JS. The male genital system. Pediatr Rev. 2020;41(3):101-111. doi:10.1542/pir.2017-0316."}
{"id" : 3301, "question_text" : "The parents of a 12-year-old boy bring him to the emergency department for evaluation of sudden onset of nausea, vomiting, headache, and slurred speech. He was well when he left for school in the morning, but when his mother returned home from work today, she found him asleep. The parents report that the patient has no significant past medical or surgical history. Two weeks ago, he had symptoms of an upper respiratory infection that have since resolved. No changes in appetite, weight, or elimination habits are noted. There is no known trauma, although he plays soccer daily. On physical examination, you note tachycardia, dilated pupils, and diaphoresis. He laughs inappropriately when questioned. You suggest performing a drug screen. Of the following, the MOST likely drug abused in this case is", "options" : "[\"atomoxetine\", \"dextromethorphan\", \"diphenhydramine\", \"methylphenidate\", \"oxycodone\"]", "explanation" : "Preferred Response: B\nThe boy in this vignette presents with sudden onset of nausea, vomiting, headache, and altered mental status (sleepiness, slurred speech, and inappropriate laughter) after being home alone. Whenever there are no physical signs of trauma or findings suggestive of an acute infectious, metabolic, or central nervous system process, one must consider accidental or purposeful ingestion of a chemical agent as the cause. Unfortunately, abuse of prescription drugs and over-the-counter medications is a significant health problem and it is increasing. Next to marijuana and alcohol, prescription medications are the most common drugs that teenagers use to get high. Medications that can be purchased over the counter are also often used inappropriately for recreational purposes. There may be several motivating factors to the nonmedical use of prescription and over-the-counter medications, such as the wrongful presumption that they are less addicting or harmful, and fewer concerns about the legal implications. In addition to the history, the findings on physical examination are key to determining the most likely drug abused. In this case, dextromethorphan is the most likely drug abused.\n\nIt has been estimated that as many as 1 million youth and young adults in the United States misuse over-the-counter cough and cold medications each year, and reports from other countries suggest this abuse is prevalent there as well. Dextromethorphan, to which the patient in the vignette had access, is commonly found in cough and cold medicines, and ingestion should be suspected when there is an abrupt change in behavior associated with findings of sympathetic stimulation (tachycardia, mydriasis, diaphoresis), gait disturbance, and euphoria or hallucinations. The signs of dextromethorphan toxicity are dose dependent, ranging from mild stimulation to euphoria and hallucinations to a dissociative state and then unresponsiveness. If co-ingested with mono-amine oxidase inhibitors or other serotonergic agents, the serotonin syndrome may be produced, which is life threatening. Common street names for dextromethorphan include Orange Crush, Triple Cs, Skittles, Vitamin D, Dex, and Robo. It can be swallowed or snorted. Of the 10% of teenagers who reported the use of cough medicine to get high, only 48% believed it was risky. Diphenhydramine is also easily purchased over the counter and is commonly used for its antihistamine and antitussive qualities. Rather than diaphoresis, dry flushed skin would be the manifestation of an overdose.\n\nAbused prescription medications most often belong to the individual or a friend. Nearly 30% of adolescents report having a friend who abuses prescription stimulants, and almost 20% report taking prescription medications that were not prescribed to them at least once. Atomoxetine and methylphenidate are both commonly prescribed for the treatment of attention-deficit/hyperactivity disorder (ADHD), so teenagers have ready access to these medications. Although most individuals with ADHD use their medications appropriately, increased availability raises the potential for use of these drugs for nonmedical purposes or by those without a proper diagnosis of ADHD. Attention-deficit/hyperactivity disorder medications may be used inappropriately to help improve concentration or to increase alertness in an effort to enhance performance on examinations or during times of high productivity demand by students without ADHD. Misuse of these drugs would present with increased alertness rather than the sleepiness and slurred speech seen in the boy in the vignette. Oxycodone is another popular substance of abuse. Estimates suggest that 10% to 20% of teenagers have abused an opioid prescription pain reliever. Oxycodone ingestion would present with miosis, bradycardia, hypotension, hypothermia, hyporeflexia, decreased respirations, and impaired mentation. Other common prescription medications abused include sleep agents and sedatives or anxiolytics.\n\nPREP Pearls\n• Dextromethorphan and other agents commonly found in cough and cold medicines are often used inappropriately for recreational purposes.\n• Dextromethorphan overdose manifests as a combination of stimulant, euphoric, and dissociative effects.\n• The nonmedical use of prescription medications is becoming increasingly common and is associated with a greater likelihood of substance abuse.\n\nAmerican Board of Pediatrics Content Specification(s)\n• Recognize the risk of abuse of over-the-counter cough and cold preparations\n• Recognize the risk of abuse of prescription medications\n\nSuggested Reading\n• Boyd CJ, McCabe SE, Cranford JA, Young A. Adolescents' motivations to abuse prescription medications. Pediatrics. 2006;118(6):2472-2480. doi:10.1542/peds.2006-1644.\n• Gunn VL, Taha SH, Liebelt EL, Serwint JR. Toxicity of over-the-counter cough and cold medications. Pediatrics. 2001;108(3)152. doi:10.1542/ peds.108.3.e52.\n• Parran TV, Wilford BB, DuPont RL. Prescription drug abuse and addiction: clinical features, epidemiology, and contributing factors. UpToDate. Available online only for subscription.\n• Rosenbaum C, Boyer EW. Dextromethorphan poisoning: epidemiology. pharmacology and clinical features. UproDate. Available online only for subscription.\n• Schwartz B, Alderman EM. Substances of Abuse. Pediatr Rev. 1997;l8(6):204-215. doi:10.1542/pir.18-6-204.\n• Setlik J. Bond GR. Ho M. Adolescent prescription ADHD medication abuse is rising along with prescriptions for these medications. Pediatrics. 2009;124(3):875 -880. doi:10.1542/peds.2008-0931.\n• Shehab N, Schaefer MK, Kegler SR, Budnitz DS. Adverse events from cough and cold medications after a market withdrawal of products labeled for infants. Pediatrics. 2010d 26(6):1100-1107. doi!10.1542/peds.2010-1839.\n• Wilford BB, Parran TV, DuPont EL. Prescription drug abuse and addiction: prevention, identification, and management. UproDate. Available online only for subscription."}
{"id" : 2882, "question_text" : "A neonate born at term is being evaluated in the newborn nursery. She was born overnight via vaginal delivery to a 27-year-old gravida 1, para 0 woman with a history of asthma. The mother's prenatal records report group B Streptococcus–negative status and a positive VDRL test (1:2) result with positive treponemal test. The neonate has unremarkable physical examination findings. She is breastfeeding well. Of the following, the BEST next diagnostic test for this neonate is", "options" : "[\"direct fluorescent antibody\", \"Treponema pallidum enzyme immunoassay\", \"rapid plasma reagin\", \"treponemal polymerase chain reaction\"]", "explanation" : "Correct Answer: C\nFor the neonate in the vignette, born to a mother with confirmed syphilis, a nontreponemal test such as rapid plasma reagin (RPR) should be performed to obtain titers on the neonate. Treponemal tests, such as Treponema pallidum enzyme immunoassay (TP-EIA) or T pallidum chemiluminescent assay (TP-CIA), are used to confirm infection with T pallidum. However, treponemal tests are not used in the diagnostic evaluation of neonates born to mothers with syphilis infection because passive maternal transfer of antibodies would lead to positive results. Direct fluorescent antibody tests are no longer available in the United States. Though treponemal polymerase chain reaction testing is available, it is not used clinically.\n\nRates of congenital syphilis, caused by the spirochete T pallidum, are rising. Detection and treatment during pregnancy reduces maternal morbidity and prevents neonatal transmission. Women should be screened for syphilis early in pregnancy. Those who test negative should undergo repeat syphilis screening at the time of delivery to identify infection later in pregnancy.\n\nConventional screening for syphilis may be performed with nontreponemal serologic tests such as the VDRL or RPR tests. These tests detect the presence of antibodies against cardiolipin-cholesterol-lecithin complex. Both RPR and VDRL may be falsely positive in the setting of viral infection, lymphoma, tuberculosis, malaria, endocarditis, connective tissue disease, or substance abuse. Both RPR and VDRL may be falsely negative early in cases of congenital syphilis, early primary syphilis, and late congenital syphilis.\n\nSome laboratories have adopted a \"reverse sequence screening\" approach; pregnant women are initially screened with a treponemal specific test such as TP-EIA or TP-CIA. Women with a positive treponemal specific test are subsequently tested using a nontreponemal serologic test such as RPR or VDRL to obtain titers. If test results are discordant, that is, the treponemal test result is positive but the nontreponemal test result is negative, a different treponemal test should be performed. The reverse sequence screening approach may have higher false-positive rates in communities with a low prevalence of syphilis. An algorithm for both conventional and reverse sequence testing is presented in Item C83.\n\nWith adequate maternal treatment of primary or secondary syphilis infection, the risk of congenital syphilis can be markedly reduced. Either RPR or VDRL titers may be followed during pregnancy to confirm adequate response to treatment. Ideally, this testing should be performed at the same laboratory. A 4-fold decrease in titers reflects adequate treatment; a 4-fold increase in titers suggests new infection or relapse.\n\nPREP Pearls\n• There are 2 approaches to screening pregnant women for syphilis:\n  o Conventional screening: Screen with rapid plasma reagin (RPR) or VDRL tests, which are nontreponemal serologic tests. Positive results are confirmed with a treponemal test such as Treponema pallidum enzyme immunoassay (TP-EIA).\n  o Reverse screening: Screen with treponemal specific tests such as TP-EIA. If positive, RPR or VDRL is sent to obtain titers.\n• A 4-fold decrease in RPR or VDRL titers suggests an appropriate response to treatment; a 4-fold increase in titers indicates inadequate treatment or new infection.\n• For a neonate born to a mother with confirmed syphilis, a nontreponemal test such as the RPR test should be performed to obtain titers on the neonate.\n\nABP Content Specifications(s)\n• Plan the management of a neonate whose mother has abnormal prenatal laboratory findings\n\nSuggested Readings\n• American Academy of Pediatrics. Summary of infectious disease. Syphilis. In: Kimberlin DW, Brady MT, Jackson MA, Long SS eds. Red Book: 2018-2021 Report of the Committee on Infectious Diseases. Elk Grove Village, IL: American Academy of Pediatrics; 2018:773-788.\n• Butterfield R. Syphilis. Pediatr Rev. 2014;35(5):212-213. doi: 10.1542/pir.35-5-212.\n• Kojaoghlanian T. The newborn at risk for infection. In: McInerny TK, Adam HM, Campbell DE, DeWitt TG, Foy JM, Kamat DM, eds. American Academy of Pediatrics Textbook of Pediatric Care. 2nd ed. Itasca, IL: American Academy of Pediatrics; 2016;chap 102:899-908. Pediatric Care Online."}
{"id" : 3545, "question_text" : "In a child with septic shock requiring vasopressor support, which agent is preferred over dopamine?", "options" : "[\"Either epinephrine or norepinephrine, guided by clinician preference and patient physiology\", \"Dopamine remains the first-line agent for all pediatric septic shock\", \"Phenylephrine is superior to all other vasopressors\", \"Vasopressin should be used as monotherapy before considering other agents\"]", "explanation" : "The text suggests using epinephrine or norepinephrine rather than dopamine in children with septic shock, and states that either epinephrine or norepinephrine may be selected as first-line based on clinician preference, individual patient physiology, and local system factors."}
{"id" : 358, "question_text" : "You are seeing a 4-month-old infant who has a 4-hour history of inconsolable crying and frequent bile-colored emesis. His mother reports that he has had no stools today. He was born at term via vaginal delivery with no prenatal or neonatal complications. Results of his 2-month health supervision visit were within normal parameters, and he received the appropriate immunizations at that time. Physical examination today reveals a well-nourished child in distress whose temperature is 37.0°C, heart rate is 160 beats/min, respiratory rate is 45 breaths/min, blood pressure is 78/50 mm Hg, and oxygen saturation is 95% in room air. His lungs are clear to auscultation. His perfusion is decreased peripherally. Abdominal examination documents a markedly distended abdomen, minimal bowel sounds, and diffuse pain. You order abdominal radiography. Of the following, the MOST likely cause of this child's symptoms is", "options" : "[\"appendicitis\", \"gastroenteritis\", \"Mallory-Weiss tear\", \"necrotizing enterocolitis\", \"volvulus\"]", "explanation" : "The infant described in the vignette has a history and physical examination findings that are consistent with an acute obstructive process that requires immediate surgical referral. His symptoms began acutely 4 hours ago and are significant for bile-covered emesis, which is concerning for an obstructive process. On physical examination, he has tachycardia, decreased perfusion, and marked abdominal distension that appears to be painful. Abdominal radiography demonstrates dilated loops of bowel, pneumatosis intestinalis (air in the bowel wall), and a paucity of intraluminal colonic bowel gas, all of which are consistent with an acute small bowel obstruction that is confirmed by subsequent ultrasonography. Volvulus associated with malrotation is the most common cause of small bowel obstruction in patients younger than 1 year of age who have not had previous bowel surgery. This is a life-threatening diagnosis, and any patient who has symptoms of acute volvulus must receive radiographic imaging (ultrasonography, upper gastrointestinal radiographic series) to rule out the diagnosis. Prompt surgical intervention is needed to prevent intestinal ischemia, and surgical fixation of the bowel (Ladd procedure) is performed to prevent subsequent episodes. In addition, an incidental appendectomy is performed because the appendix will lie in an abnormal position after repair, which would make diagnosis of appendicitis difficult.\n\nAcute abdominal pain in childhood can be a diagnostic dilemma because of the wide range of possible causes as well as the inherent difficulties in examining a preverbal or developmentally delayed child. Careful history taking and physical examination in conjunction with appropriate laboratory and radiologic testing can help guide the pediatrician in determining the likely cause, initiating appropriate management, and ensuring that a pathologic condition requiring surgical intervention, an acute \"surgical abdomen,\" is not missed. History and physical examination findings consistent with a potential need for surgical consultation and intervention include a history of abdominal trauma, pain that is made worse with movement, involuntary guarding, rebound tenderness, and tenderness with percussion. Laboratory studies such as white blood cell count, erythrocyte sedimentation rate, lipase and amylase measurement, and urinalysis can help confirm diagnostic suspicions but are not diagnostic alone. Radiologic imaging (plain film radiography, computed tomography scan, ultrasonography, or contrast enema) may be indicated to assist with diagnosis or guide subsequent treatment.\n\nAcute appendicitis in the young pediatric patient can be challenging to recognize, but the bile-covered emesis and abdominal radiography findings for this infant are not consistent with this diagnosis. Gastroenteritis is very unlikely due to the lack of stools, physical examination findings, and radiography results. Necrotizing enterocolitis also is unlikely based on the infant's age and baseline health. In addition, although pneumatosis intestinalis is classically seen in necrotizing enterocolitis, abdominal distension tends to be diffuse rather than localized. Mallory-Weiss tears (tears in the esophageal mucosa, often due to forceful vomiting or portal hypertension) typically present with hematemesis or melena in addition to abdominal pain.\n\nCritique: The infant described in the vignette has a history and physical examination findings that are consistent with an acute obstructive process that requires immediate surgical referral. His symptoms began acutely 4 hours ago and are significant for bile-covered emesis, which is concerning for an obstructive process. On physical examination, he has tachycardia, decreased perfusion, and marked abdominal distension that appears to be painful. Abdominal radiography demonstrates dilated loops of bowel, pneumatosis intestinalis (air in the bowel wall), and a paucity of intraluminal colonic bowel gas, all of which are consistent with an acute small bowel obstruction that is confirmed by subsequent ultrasonography. Volvulus associated with malrotation is the most common cause of small bowel obstruction in patients younger than 1 year of age who have not had previous bowel surgery. This is a life-threatening diagnosis, and any patient who has symptoms of acute volvulus must receive radiographic imaging (ultrasonography, upper gastrointestinal radiographic series) to rule out the diagnosis. Prompt surgical intervention is needed to prevent intestinal ischemia, and surgical fixation of the bowel (Ladd procedure) is performed to prevent subsequent episodes. In addition, an incidental appendectomy is performed because the appendix will lie in an abnormal position after repair, which would make diagnosis of appendicitis difficult.\n\nContent Specifications: Recognize an acute \"surgical abdomen\""}
{"id" : 2889, "question_text" : "A 15-year-old basketball player with hypertrophic cardiomyopathy comes for consultation regarding return to play. Three months ago, he collapsed at a basketball game and was successfully resuscitated. After additional cardiac consultation, an implantable cardioverter defibrillator (ICD) was placed. He has not received any shocks since ICD placement. The cardiologist has informed him and his family that he may consider returning to basketball at this time, as long as they all understand the potential risks. The patient is highly interested in returning to basketball, but his parents are hesitant and wish to learn more about all his options. Of the following, the principle that is MOST applicable to this decision is", "options" : "[\"autonomy\", \"beneficence\", \"justice\", \"nonmaleficence\"]", "explanation" : "Correct Answer: D\nSports participation in athletes with implantable cardiac defibrillators (ICDs) may be considered if the athlete has not received any ICD shocks from episodes of ventricular flutter or fibrillation for at least 3 months. However, sports participation increases the likelihood of both appropriate and inappropriate shocks, as well as the potential for device-related damage in contact/collision sports. These issues need to be considered when discussing the risk versus benefit ratio of return to basketball for the boy in the vignette.\n\nThe 4 basic ethical principles of modern medicine are nonmaleficence, beneficence, autonomy, and justice. Nonmaleficence requires consideration of the potential harms that may accrue to a patient in a given circumstance. In this case, the chief concerns regarding return to basketball are the potential for additional shock delivery and risk of ICD damage. Therefore, nonmaleficence is most applicable. The patient and his family need to be informed of the risks associated with a return to basketball, and to weigh these against the patient's desire to return to this activity. For example, a 2018 study looking at the consequences of sports participation in subjects with ICDs found that about 27% of subjects reported additional shocks with sports, but no other ICD-related adverse events. From a medical perspective, this is not an undue risk. However, considering the discomfort associated with ICD shock delivery, the patient may decide that he would prefer to avoid activities that increase the risk of additional shocks.\n\nBeneficence is defined as action that is in the patient's best interest. Beneficence and nonmaleficence often coexist, and potential benefits need to be considered in light of potential risks. Although there would be multiple benefits to clearing this patient for basketball participation, these are not the primary issues under consideration. Therefore, beneficence is not the most applicable ethical principle being applied in this vignette.\n\nAutonomy recognizes the right of individual patients to make decisions for themselves. Competent patients with the capacity to understand treatment risks, benefits, and alternatives are generally accorded the right to autonomy. Capacity is highly variable in the pediatric population and will depend on the patient's developmental stage, as well as the specific issue at hand. Most adolescents are considered to be developing autonomy, and should be given increasing say in their treatment options as they move toward adulthood. At 15 years of age, the adolescent in the vignette may have the capacity to fully consider the consequences of this decision. Although patient autonomy may become a dominant issue if he and his parents disagree on the decision to return to sport, or if the family disagrees with the provider's recommendation, it is not the primary issue in this vignette.\n\nThe principle of justice is based on both individual and social factors, and relies on the fair settlement of competing interests. This principle ensures that a decision for the benefit of 1 patient (or segment of patients) is not at the expense of others. The case in this vignette would not be expected to have significant medical impact on others, and therefore justice is not the primary issue in this case.\n\nPREP Pearls\n• Nonmaleficence (do no harm) and beneficence (action for the patient's benefit) are usually complementary in medical ethics decision-making.\n• The appropriate delegation of autonomy in decisions to adolescents is dependent on their stage of development and the particulars of the situation.\n• The principle of justice ensures that a decision for the benefit of 1 patient (or segment of patients) is not at the expense of others.\n\nMOCA-Peds Objective\n• Identify contraindications for sports participation.\n\nABP Content Specifications(s)\n• Recognize and apply ethical principles regarding the care of children and adolescents with disabilities\n\nSuggested Readings\n• Cummings C, Mercurio MR. Session 2: Autonomy, Beneficence, and the Rights of Parents and Children. American Academy of Pediatrics website. https://www.aap.org/en-us/Documents/Bioethics-AutonomyBeneficenceAndTheRightsofParentsandChildren.pdf .\n• Rowell EE, Fecteau A, Katz AL, Beals D, et al. Ethics. American Pediatric Surgical Association website. https://www.pedsurglibrary.com/apsa/view/Pediatric-Surgery-NaT/829247/all/Ethics.\n• Saarel EV, Law I, Berul CI, Ackerman MJ, et al. Safety of Sports for Young Patients With Implantable cardioverter-defibrillators: long-term results of the multinational ICD Sports Registry. Circ Arrhythm Electrophysiol. 2018;11:e006305. doi: 10.1161/CIRCEP.118.006305."}
{"id" : 3083, "question_text" : "A shy, 14-year-old adolescent boy presents to your office for a physical examination. He denies any concerns today and is not sexually active. His past medical history is significant only for cryptorchidism that was repaired at 1 year of age. The boy's last genital examination, at 10 years of age, was unremarkable for a prepubertal male. His height today is at the 5th percentile for his age, and weight is at the 10th percentile, and he has normal vital signs. He has no facial hair, axillary hair, or voice changes. He refuses a genital examination. The remainder of his physical examination findings are unremarkable. You discuss with your medical student the importance of performing a genital examination and how one would teach the boy about testicular self-examination.\n\nOf the following, the MOST important reason to do a genital examination in this case is to", "options" : "[\"determine that puberty is progressing normally\", \"evaluate for the presence of an indirect hernia\", \"inspect and palpate for the presence of varicoceles\", \"inspect for signs of sexually transmitted infections\", \"palpate for the presence of a testicular mass\"]", "explanation" : "It is not uncommon to have an early male adolescent refuse to undress for a genital examination. Early puberty is a time of body changes that make adolescents very self-conscious and concerned about their normality. The main value of an examination is usually to provide reassurance. The adolescent in the vignette has a history of cryptorchidism. An undescended testicle is often abnormal to start with and is at increased risk for development of masses. Therefore, this is the primary reason to teach the adolescent boy how to do a self-examination and persuade him to allow you to do one in the near future.\n\nA male adolescent begins the development of secondary sexual characteristics between 9 and 14 years of age. Testicular development is the first sign of puberty in a male adolescent and a teenager should enter sexual maturity rating 2 by 14.5 years of age at the latest. Therefore, for the boy in the vignette, it is acceptable to wait up to 6 months to confirm the onset of puberty and evaluate for testicular mass. In the meanwhile, educating the parents and the adolescent, along with gaining his trust, would be very important as most patients will allow an examination at future visits. The testicular examination can be very revealing of pathology that includes hernias, varicoceles, and signs of sexually transmitted infections; in addition, testicular examination is most important to evaluate for masses. While testicular cancer is not common, the peak incidence is during adolescence and young adulthood.\n\nPREP Pearls\n• A history of cryptorchidism requires that a male adolescent be routinely screened for the development of testicular masses.\n• A number of lesions may be found on routine genital examination in a boy, despite a negative history of symptoms.\n\nAmerican Board of Pediatrics Content Specification(s)\n• Understand the importance of testicular self-examination\n\nSuggested Reading\n• Adelman WP, Joffe A. Consultation with the specialist: testicular masses/cancer. Pediatr Rev. 2005;26(9):341-344. doi:10.1542/pir.26-9-341.\n• Brenner JS, Hergenroeder AC, Kozinetz CA, and Kelder SH. Teaching testicular self-examination: education and practices in pediatric residents. Pediatrics. 2003;111(3):e239-e244. doi:10.1542/peds.111.3.e239.\n• Cavanaugh RM Jr. Screening for genitourinary abnormalities in adolescent males. Pediatr Rev. 2009;30(11):431-438. doi:10.1542/pir.30-11-431."}
{"id" : 1500, "question_text" : "A 4-year-old boy is brought to your office for concerns about his behavior. He gets into trouble for fighting with his younger siblings and for refusing to follow directions. He gets frustrated easily and will throw things when upset. When you greet the boy, he makes good eye contact and smiles. He speaks in 2-word phrases and can follow a single step direction if given with gesture. He enjoys playing with the puzzles and cars in your office, but shows no interest in looking at books. When you praise him for solving a puzzle, he smiles and looks to his mother. His physical examination is within normal limits.\n\nOf the following, the MOST appropriate next step in management is to", "options" : "[\"engage the child in play therapy\", \"enroll the child in a preschool program\", \"order an electroencephalogram\", \"refer the child to early intervention services\", \"request an evaluation through his school district\"]", "explanation" : "The boy in this vignette has delayed language development. Since a typical 4-year-old child can speak in sentences and follow multistep commands, this child's expressive and receptive language are less than expected for his age. On the other hand, this child's social skills and play appear appropriate and his challenging behaviors may be secondary to frustration from difficulty communicating. Of the choices provided, the most appropriate next step is to request an evaluation through the child's school district to evaluate his development and learning so that appropriate educational services can be initiated. The boy in this vignette would qualify for speech and language therapy and may also qualify for specialized academic instruction (eg, placement in a special education preschool program) if cognitive or other learning concerns are identified.\n\nThe Individuals with Disabilities Education Act (IDEA) is an important federal law, which provides for early intervention (EI) and special education services for children with learning or physical disabilities. The IDEA was enacted in 1975 and most recently reauthorized in 2004. Individuals aged 3 to 21 years may qualify for special education services through part B of IDEA. An evaluation is first conducted to determine if the child meets eligibility criteria under one of the special education disability categories (eg, specific learning disability, speech or language impairment, other health impaired, emotional disturbance, intellectual disability, autistic-like behaviors, hearing impairment, visual impairment, traumatic brain injury). If the child qualifies for special education services, an Individualized Education Program (IEP) is developed, outlining the services and accommodations that will be provided to the child to meet his educational needs. About 13% of children in the United States receive special education services.\n\nEarly intervention programs are federally funded under Part C of IDEA. They provide services to children from birth to 3 years of age with delays in development (physical, cognitive, communication, social/emotional, adaptive development) or a condition that results in high probability of developmental delay. Some states also provide services to children who are at high risk for developmental delays. The EI programs are multidisciplinary, community-based, and family-centered. Early intervention starts with identification, screening, and assessment to determine eligibility and needs. Services such as special instruction, speech therapy, occupational therapy, physical therapy, family training, and counseling are offered, based on the needs identified through the assessment process. Additional services include home visits and assistance with transitioning to community or special education services as appropriate. A service coordinator assists the family with setting up the services and connecting with resources. Specific services and goals are outlined in the Individualized Family Service Plan. Early intervention serves about 2% of infants and young children.\n\nChildren younger than 3 years of age with developmental or learning concerns should be referred to their state's EI program or to their local school district if older than 3 years of age. The 4-year-old child in this vignette would benefit from an evaluation through his school district. While enrollment in a preschool program would be helpful, an evaluation of this child through his school district will help determine if his needs can be met in a typical preschool program or if a special education preschool is a better fit. The evaluation would also determine if additional therapies (eg, speech therapy) are necessary and if the child qualifies for an IEP. Play therapy, a form of psychotherapy used to allow children to express their feelings and experiences through play, would not address this child's developmental or educational needs. Landau-Kleffner syndrome, or acquired epileptic aphasia, is a neurological disorder where children develop normally and then lose language skills. An electroencephalogram is not indicated in this child who does not have a history of language regression. However, this child should be referred for audiology, speech/language, and developmental/psychological evaluations (through school, insurance, or private pay).\n\nPediatricians can be instrumental in guiding families to appropriate community services for their children with learning and behavioral problems. Children younger than 3 years of age with confirmed or suspected developmental delay should be referred to their community's EI program, and if older than 3 years of age, to their local school district for needed special education services. The pediatrician can advocate for assessment and access to appropriate instruction and services. Prompt treatment improves the outcomes of these children.\n\nPREP Pearls\n• The Individuals with Disabilities Education Act (IDEA) is an important federal law, which provides for early intervention and special education services for children with learning or physical disabilities.\n• Individuals aged 3 to 21 years may qualify for special education services through part B of IDEA.\n• Children younger than 3 years of age for whom there are developmental or learning concerns should be referred to their state's early intervention program or, if older than 3 years of age, to their local school district.\n\nABP Content Specifications(s)\n• Identify the types of community services available to families of children who have learning and behavioral problems\n\nSuggested Readings\n• Lipkin PH, Okamoto J, Council on Children with Disabilities and Council on School Health. The Individuals with Disabilities Education Act (IDEA) for children with special educational needs. Pediatrics. 2015;136(6):e1650-e1662. doi: http://dx.doi.org/10.1542/peds.2015-3409.\n• Rose L, Herzig LD, Hussey-Gardner B. Early intervention and the role of pediatricians. Pediatr Rev. 2014;35(1):e1-e10. doi: http://dx.doi.org/10.1542/pir.35-1-e1."}
{"id" : 1862, "question_text" : "A 2-week-old male infant is at a health supervision visit. He was delivered at term by vaginal delivery and was discharged from the hospital after 2 days. His mother reports that prenatal testing was normal. On physical examination in the newborn nursery, it was noted that his left foot was turned inward. The remainder of his examination findings were normal. The boy is otherwise healthy. Physical examination reveals a left foot deformity (Item Q62). The foot can be passively stretched almost to the midline, but it is not possible to dorsiflex the ankle to a neutral position.\n\nItem Q62: Findings for the neonate described in the vignette. Courtesy of R. Carl\n\nOf the following, the BEST next management step for this neonate is", "options" : "[\"bracing with reverse last shoes\", \"home stretching exercises with reevaluation at age 6 months\", \"immediate referral to orthopaedic surgery for casting\", \"referral to orthopaedic surgery for surgical reconstruction at age 6 months\"]", "explanation" : "The infant in the vignette has congenital talipes equinovarus (TEV) or clubfoot. The widely accepted treatment for TEV is known as the Ponseti method. Treatment involves 3 stages: serial long leg casting, surgery to cut the Achilles tendon (for most infants), and long-term bracing to maintain the corrected position. Most orthopaedic providers recommend beginning treatment in the first few weeks after birth, provided the child is healthy without more pressing medical issues. The boy in the vignette should be referred immediately to an orthopaedic provider to initiate casting.\n\nTalipes equinovarus deformity is the most common congenital foot deformity that requires treatment. Features include a high arch, adducted position of the forefoot, hindfoot varus, and plantar flexion contracture. Talipes equinovarus develops by 16 weeks of gestation, and can often be detected on prenatal ultrasonography. Most children treated with the Ponseti method have excellent foot appearance and function later in life.\n\nMetatarsus adductus, a flexible, adducted positioning of the forefoot, is a common foot deformity that results from intrauterine positioning. Children with metatarsus adductus have a curved appearance of the lateral edge of the foot. This diagnosis can be distinguished from TEV on physical examination; infants with metatarsus adductus have normal ankle motion, while those with TEV are fixed in a plantarflexed position. Some providers recommend stretching exercises to promote resolution of metatarsus adductus. Casting and splinting may be useful for infants with metatarsus adductus that persists beyond age 6 months, though this condition almost always resolves spontaneously by the age of 3 to 4 years. Metatarsus varus is a term used to denote a rigid adducted position of the forefoot (ie, the foot cannot be stretched into a normal position). Infants with metatarsus varus should be referred to an orthopaedic physician for treatment with casting and/or splinting.\n\nHome stretching and reverse last shoes (shoes designed to pull the forefoot into an abducted position) are not indicated for the treatment of TEV. Surgical reconstruction of the foot was historically the treatment of choice for TEV. However, multiple studies have shown that children treated with the Ponseti method have better functional outcomes than those treated with surgery, so this technique is now considered the 'gold standard'.\n\nPREP Pearls\n\nTalipes equinovarus (clubfoot) is the most common congenital foot deformity that requires treatment.\n\nThe features of talipes equinovarus include a high arch, adducted position of the forefoot, hindfoot varus, and plantar flexion contracture.\n\nThe 'gold standard' technique for the treatment of talipes equinovarus is the Ponseti method which includes serial casting, surgery to cut the Achilles tendon, and bracing.\n\nABP Content Specifications(s)/Content Area\n\nRecognize the clinical findings associated with clubfoot and the need for prompt referral\n\nSuggested Readings\n\nMosca V. The foot. In: Weinstein SL, Flynn HM, eds. Lovell & Winter's Pediatric Orthopaedics. 7th ed. Philadelphia: Lippincott Williams & Wilkins; 2013;chap 29:1389-1525."}
{"id" : 1474, "question_text" : "A previously healthy 5-year-old boy is brought to the emergency department with fever, a rapidly progressive rash, and increasing lethargy over the past 24 hours. He has not had nausea, vomiting, or sick contacts. He has no allergies or significant past medical history, and is not on any medications. On rapid assessment, he is very difficult to arouse and is moaning and mumbling on painful stimuli, with occasional eye opening. His airway is patent and he is breathing comfortably. He has cool extremities and a capillary refill time of 5 seconds. He has a disseminated purpuric rash involving face, trunk, and extremities. Liver edge is not palpable. Initial vital signs showed a temperature of 39.5°C, heart rate of 170 beats/min, respiratory rate of 24 breaths/min, blood pressure of 70/30 mm Hg, and pulse oximetry of 100% on room air. In the first 30 minutes, he is started on oxygen by non-rebreather facemask, receives 100 mL/kg of 0.9% saline boluses, and a 100 mg/kg dose of intravenous ceftriaxone. Upon re-evaluation, vital signs show a temperature of 39°C, heart rate of 160 beats/min, respiratory rate of 30 breaths/min, blood pressure of 75/40 mm Hg, and pulse oximetry of 100% on 100% non-rebreather facemask. He is still sleepy, but easier to arouse. He is in moderate respiratory distress with intercostal retractions. He has bilateral crackles on auscultation. Heart is regular, with no rubs, gallops, or murmurs. Capillary refill time is 3 seconds. Liver is 4 cm below the costal margin. Of the following, the BEST next step to stabilize the patient is", "options" : "[\"administer 0.9% saline bolus, 20 mL/kg intravenously\", \"administer 5% albumin bolus, 20 mL/kg intravenously\", \"administer hydrocortisone intravenously\", \"start double volume whole blood exchange transfusion\", \"start intravenous epinephrine infusion\"]", "explanation" : "The boy in this vignette has septic shock from meningococcemia. He has received several intravenous fluid boluses but is still in shock, evidenced by persistent tachycardia and hypotension. Since he has hepatomegaly and crackles on lung auscultation, additional fluid administration would worsen his condition, therefore the best next step is to start an epinephrine infusion.\n\nShock is the condition of oxygen and nutrient delivery insufficient to meet end-organ metabolic demands. Management includes optimizing oxygen delivery, which is the product of cardiac output and oxygen content. Cardiac output is stroke volume multiplied by heart rate, and oxygen content is mostly dependent on hemoglobin concentration and percentage of saturated hemoglobin. Although classifications change, types of shock include hypovolemic, cardiogenic, distributive, and septic. Regardless of the type of shock, fluid management can be assisted by an algorithm such as the one shown in Item C225, along with frequent clinical reassessment for hemodynamic status. Once shock is identified in a patient based on signs such as altered mental status, delayed capillary refill, tachycardia, or hypotension, airway and breathing is established, and intravenous or intraosseous access is established in the first few minutes. Rapid boluses of isotonic fluid up to and over 60 mL/kg are given in the first 15 minutes until shock is reversed or until rales or hepatomegaly develop, at which point inotropic medications are started. The 2015 Pediatric Advanced Life Support algorithm for shock should be followed. When these algorithms are followed, patients with cardiogenic shock are identified by the development of pulmonary edema, hepatomegaly, or worsened circulation with heart failure.\n\nHypovolemic shock is the most common form of shock in children. Findings include tachycardia, tachypnea, delayed capillary refill, and signs of dehydration. Extremities are usually cool because of the compensatory mechanism of vasoconstriction. Hypovolemic shock is usually reversed with fluid administration alone and is less likely to require inotropes. In contrast, septic shock is systemic inflammation caused by an infection leading to shock. The effect of the bacterial toxin, if present, and the host inflammatory cascade cause arteriolar vasodilation, cardiac depression, and increased capillary permeability. This leads to similar signs of intravascular depletion, as in hypovolemic shock. However, septic shock can be distinguished from hypovolemic shock by the presence of fever, decreased cardiac function, vasodilation with \"flash\" capillary refill, capillary leak, and inotropic requirement. Whereas hypovolemic shock is usually \"cold\" shock, septic shock may be either \"warm\" or \"cold\" depending on the degree of vascular tone.\n\nThe boy in this vignette has septic shock evidenced by signs of infection, systemic inflammation, and shock. Since he has rales and hepatomegaly after fluid resuscitation, further increase of intravascular volume, either with normal saline or 5% albumin, would worsen his condition. Hydrocortisone has been shown to reverse shock in patients at risk of adrenal insufficiency and in patients with catecholamine-resistant shock, but neither is occurring in the boy in this vignette. Double volume whole blood exchange transfusion can be effective in meningococcemia and multiple organ failure, but a more immediate need is to reverse the shock. Starting an epinephrine infusion would be the best next step in reversing the shock.\n\nPREP Pearls\n• Hypovolemic shock is usually reversed by restoration of intravascular volume, and is less likely to require inotropes\n• In addition to signs of decreased intravascular volume also seen in hypovolemic shock, septic shock can also manifest with fever, \"flash\" capillary refill, and edema\n\nABP Content Specifications(s)\n• Differentiate the findings associated with hypovolemic shock from those of septic shock, and manage appropriately\n\nSuggested Readings\n• Brierley J, Carcillo JA, Choong K, et al. Clinical practice parameters for hemodynamic support of pediatric and neonatal septic shock: 2007 update from the American College of Critical Care Medicine. Crit Care Med. 2009;37(2):666-688. doi: http://dx.doi.org/10.1097/CCM.0b013e31819323c6.\n• Brierley J, Peters MJ. Distinct hemodynamic patterns of septic shock at presentation to pediatric intensive care. Pediatrics. 2008;122(4):752-759. doi: http://dx.doi.org/10.1542/peds.2007-1979.\n• de Caen AR, Berg MD, Chameides L, et al. Part 12: pediatric advanced life support: 2015 American Heart Association guidelines update for cardiopulmonary resuscitation and emergency cardiovascular care. Circulation. 2105;132:S526-S542. doi: http://dx.doi.org/10.1161/CIR.0000000000000266."}
{"id" : 1558, "question_text" : "You are called to attend the delivery of a 40-week, large-for-gestational age male infant. The mother is a 29-year-old gravida 4, para 2 woman with an elevated glucose level on the glucose challenge test. However, she never completed a glucose tolerance test. Results of her hepatitis B and group B Streptococcus tests were negative. A right choroid plexus cyst was noted on prenatal ultrasonography. At delivery, a tight nuchal cord and shoulder dystocia are noted. The newborn was delivered vaginally with vacuum assistance. Upon delivery, the neonate had poor tone and respiratory effort, requiring positive pressure ventilation, with resulting improvement in tone, activity, and respiratory effort. His birthweight was 4.3 kg (91st percentile), head circumference 36.5 cm (95th percentile), and length 53.5 cm (95th percentile). Physical examination demonstrates a cephalohematoma, decreased abduction of his left shoulder. No movement is noted at the elbow. Sensation and grasp in the left hand remain intact. His chest radiograph is shown. A nursing student asks why the neonate is not moving his arm. Of the following, the BEST response to his question is", "options" : "[\"brachial plexus injury\", \"cephalohematoma\", \"left humeral fracture\", \"tight nuchal cord\", \"right-sided choroid plexus cyst\"]", "explanation" : "The most likely cause of this neonate's decreased arm movement is brachial plexus injury (BPI). BPI has an incidence of approximately 1 in 1,000 live births. BPI results from a combination of maternal, neonatal, and peripartum factors. It has been associated with maternal diabetes, uterine abnormalities, neonatal macrosomia, transverse lie, and failure to progress. However, most neonates born to mothers with diabetes and macrosomia do not have BPI.\n\nOn initial physical examination, neonates with BPI present with decreased movement of the affected arm and an asymmetric Moro reflex. Initial management of suspected BPI includes minimal handling of the affected arm and short-term immobilization. In the absence of fracture, continued immobilization is controversial and passive range of motion exercises are recommended beginning at 7 to 10 days after birth. Affected neonates should receive close follow-up; referral to physical therapy should be ensured to maximize long-term function.\n\nThe presentation of BPI varies with the location and degree of cervical nerve root injury. In most cases, swelling of the surrounding nerve sheath causes neuropraxia or temporary loss of motor and sensory conduction in the cervical nerve roots. Less commonly, the nerve sheath ruptures, causing a temporary interruption in nerve conduction. In the unusual case of avulsion of the cervical roots or rupture of the axon, surgical correction may be required to maximize long-term nerve function.\n\nThe location of the nerve injury affects the initial clinical presentation. Most cases of BPI result from damage to cervical nerve roots C5-C6, causing Erb-Duchenne paralysis. Affected neonates present with an asymmetric Moro reflex and decreased abduction of the shoulder, external rotation of the arm, and supination of the forearm. Of note, palmar grasp and biceps reflexes on the injured side remain intact. Neonates may also have hemidiaphragmatic paralysis on the affected side from damage to the phrenic nerve. Erb-Duchenne paralysis typically resolves between 2 to 4 weeks after birth. However, phrenic nerve injury is associated with a worse prognosis. For infants, whose symptoms persist beyond 3 months, referral for surgical intervention should be considered.\n\nKlumpke paralysis is caused by damage to cervical roots C7, C8, and T1. Affected neonates may have Horner syndrome on the affected side (ptosis and miosis), and weakness of flexor muscles of forearm and hand. Although the biceps reflex remains intact, palmar grasp will be absent. Neonates with BPI due to axonal rupture or complete nerve root avulsion present with flaccid paralysis of the arm and hand on the affected side, with no biceps reflex or palmar grasp. Long-term prognosis is poor. Magnetic resonance imaging may be helpful in assessing the extent of injury and guiding surgical repair.\n\nInitial evaluation of BPI must include radiographs of the arm and shoulder to ensure no humeral or clavicular fracture is present. In the case of the neonate in the vignette, the humerus and clavicle were intact on the radiograph. Cephalohematoma, tight nuchal cord, and choroid plexus cyst have not been associated with BPI.\n\nPREP Pearls\n• Brachial plexus injury (BPI) typically involves swelling of the nerve sheath, resulting in decreased motor and sensory conduction.\n• On physical examination, neonates with BPI most commonly have decreased abduction of the shoulder, decreased supination of the forearm, and decreased external rotation of the arm.\n• Although most cases of BPI resolve completely, close follow-up and referral to physical therapy should be ensured to maximize long-term function.\n\nMOCA-Peds Objective\n• Evaluate and manage a neonate born to a diabetic mother\n\nABP Content Specifications(s)\n• Understand the prognosis associated with brachial plexus injuries\n• Recognize the clinical features in an infant whose delivery was complicated by shoulder dystocia\n• Recognize the clinical findings associated with brachial plexus injuries, and manage appropriately\n\nSuggested Readings\n• Joyner B, Soto MA, Adam HM. Brachial plexus injury. Pediatr Rev. 2006;27:238. doi: http://dx.doi.org/10.1542/pir.27-6-238.\n• Nelson M. Birth brachial plexus palsy. In: Kliegman RM, Stanton BF, St Geme J, Schor NF eds. Nelson Textbook of Pediatrics. 20th ed. Philadelphia, PA: Saunders Elsevier; 2016; chap 713:3406-3408.e1."}
{"id" : 2160, "question_text" : "A neonate presents with poor feeding, vomiting, lethargy, and seizures on day 3 of life. Laboratory findings during an acute metabolic crisis demonstrate hyperammonemia and respiratory alkalosis (secondary to hyperventilation). Plasma amino acid testing shows high glutamine and low citrulline levels; urine organic acid testing shows a high orotic acid level. Of the following, the MOST likely diagnosis is", "options" : "[\"classic galactosemia\", \"hyperammonemia and respiratory alkalosis\", \"medium-chain acyl-CoA dehydrogenase deficiency\", \"ornithine transcarbamylase deficiency\"]", "explanation" : "The clinical features of late-onset OTC deficiency include episodes of altered mental status, recurrent vomiting, migraine-type headaches, seizures, and a history of protein avoidance. Complications of OTC deficiency may include developmental delay, intellectual disability, and cognitive function deficits. \nManagement of an acute metabolic crisis due to a UCD includes urgent intervention to accomplish the following:\nRapidly decrease the plasma ammonia concentration (hemodialysis, peritoneal dialysis, or extracorporeal membrane oxygenation)\nProvide an alternative pathway for excretion of excess nitrogen (nitrogen scavenger therapy)\nTreat the catabolic state with calories from glucose, fats, and essential amino acids\nLong-term management of a UCD includes a low-protein diet with supplementation of essential amino acids, as well as use of nitrogen scavenger medications (eg, sodium phenylacetate, sodium phenylbutyrate). \nIn addition to the clinical presentation, laboratory findings are used to differentiate various inborn errors of metabolism. Some of these are outlined in the Table.\nHyperammonemia and high anion gap acidosis are seen during the acute presentation of a neonate with an underlying organic acidemia. Common organic acid disorders include isovaleric aciduria, methylmalonic aciduria, propionic aciduria, and maple syrup urine disease. Acute management includes stopping protein intake, reversal of catabolism by administration of high-rate intravenous glucose, intravenous carnitine, and hemodialysis for hyperammonemia. Long-term management includes a low-protein diet with supplementation of essential amino acids and carnitine.\nHypoketotic hypoglycemia with a high anion gap acidosis is a hallmark of fatty acid oxidation defects. Common fatty acid oxidation disorders include very-long-chain acyl-CoA dehydrogenase deficiency, medium-chain acyl-CoA dehydrogenase deficiency, carnitine palmitoyltransferase I and II deficiency, and carnitine transporter defects. Acute intervention includes initiation of glucose-containing intravenous fluids with electrolytes to maintain normoglycemia and prevent catabolism, as well as intravenous carnitine.\nClassic galactosemia presents in the neonatal period with hypoglycemia, hyperbilirubinemia, elevated liver enzyme concentrations, and the finding of reducing substances in the urine. Affected neonates are at risk of developing gram-negative sepsis (eg, Escherichia coli) that can result in septicemic shock and death. Immediate intervention includes initiation of glucose-containing intravenous fluids and restriction of dietary galactose. Feeding must be with a soy or other lactose-free formula; classic galactosemia is an absolute contraindication to breastfeeding.\nSuggested Reading(s)\nGreene CL. Recognizing inborn errors of metabolism. In: Saul RA, ed. Medical Genetics in Pediatric Practice. American Academy of Pediatrics; 2013:chap 7. Accessed August 7, 2023. Medical Genetics Online\nLichter-Konecki U, Caldovic L, Morizono H, Simpson K, Ah Mew N, MacLeod E. Ornithine transcarbamylase deficiency. In: Adam MP, Everman DB, Mirzaa GM, Pagon RA, Wallace SE, Bean LJH, et al, eds. GeneReviews®. University of Washington;1993–2022. Updated May 1, 2022. Accessed May 3, 2023.\nRios A, Shur N. Specific congenital metabolic diseases. In: McInerny TK, Adam HM, Campbell DE, DeWitt TG, Foy JM, Kamat DM, eds. American Academy of Pediatrics Textbook of Pediatric Care. American Academy of Pediatrics; 2021:chap 106. Accessed September 21, 2024. Pediatric Care Online\nVergano SAS. Inborn errors of metabolism: becoming ready for rare. Pediatr Rev. 2022;43(7):371-383. doi:10.1542/pir.2022-005088\nContent Domain\nGenetics\nLearning Objectives\nRecognize the clinical features associated with urea cycle defects\nThe correct answer is:\nhyperammonemia and respiratory alkalosis\nView Peer Results"}
{"id" : 1497, "question_text" : "A 6-year-old girl is brought to your office for evaluation of \"swollen glands.\" For the past 2 days, she has had a low-grade fever, cough, and sore throat, and her activity level and appetite have decreased. The mother reports that, with several illnesses this year, the girl has had noticeable swelling of the glands in her neck. She was tested for strep throat several times, but the result was always negative. Each time, the swelling improved as the acute illness resolved. Her mother has a history of Hodgkin lymphoma, so she is worried that these recurring infections with lymphadenopathy may indicate something more serious. On physical examination, her vital signs are normal for age and her temperature is 37.5°C. Her tonsils are erythematous without exudate. Her bilateral jugulodigastric nodes are slightly tender to palpation and measure 1 cm in diameter. They are discrete and mobile, with no overlying erythema or warmth. The remainder of her examination is unremarkable, with no other significant lymphadenopathy or hepatosplenomegaly. You inform the mother that the girl's pattern of lymphadenopathy is not concerning and that lymphadenopathy in specific other locations would merit concern for malignancy. Of the following, the lymphadenopathy location you would find MOST concerning is", "options" : "[\"posterior cervical\", \"preauricular\", \"submandibular\", \"submental\", \"supraclavicular\"]", "explanation" : "Correct Answer: E\nLymphadenopathy, defined as an abnormality in size and consistency of lymph nodes, is common in childhood. Recurrent and transient lymphadenopathy that is related to intermittent viral illnesses, as seen in the girl in the vignette, is almost always benign. The presence of supraclavicular lymphadenopathy on physical examination should always raise concern for malignancy.\n\nIt is understandable that parents may be alarmed by the presence of enlarged lymph nodes in their child, especially when there is a family history of malignancy or immunodeficiency. It is important for pediatric healthcare providers to be aware of the broad range of disease processes, both infectious and noninfectious, that may be associated with lymphadenopathy. A thorough history and physical examination is the first step in determining a differential diagnosis of lymphadenopathy and plan for evaluation and treatment.\n\nWhen evaluating lymphadenopathy, the history and physical examination should include:\n• age and general health of the patient, including immunization status\n• location, number, and distribution of enlarged lymph nodes\n• characteristics of the lymph nodes: size, consistency, tenderness, mobility, matting\n• onset of lymphadenopathy\n• associated constitutional signs and symptoms: presence of conjunctivitis, arthritis, bone pain, rash, pallor, petechiae, fevers, night sweats, weight loss, generalized lymphadenopathy, or hepatosplenomegaly\n• progression and duration of illness: rapid progression or duration of more than 4 weeks (defined as chronic) deserves further investigation\n• exposures: contact with ill individuals, pets/animals/insects, travel, medications\n• close inspection for localized lesions that would drain directly to the involved nodes\n\nInfection is the most likely cause for lymphadenopathy in young children, with the likelihood of malignancy increasing in adolescents. The location of enlarged lymph nodes is an important factor in determining the cause. It is helpful to consider the lymphatic drainage pattern when conducting the physical examination and considering potential causes. Cervical lymphadenopathy is commonly associated with upper respiratory infections. The jugulodigastric nodes are often enlarged and tender in reaction to acute pharyngitis. Supraclavicular lymphadenopathy should always be considered abnormal and may be enlarged in cases of mycobacterial infection or sarcoidosis, as well as malignancy. Lymphadenopathy in the preauricular, submandibular or submental areas is frequently associated with infectious or inflammatory processes and do not in themselves indicate a higher likelihood of malignancy.\n\nCharacteristics that suggest benign reactive lymphadenopathy include nodes that are localized, discrete, mobile, not matted, less than 2 cm in diameter, and with no overlying erythema or warmth. Associated constitutional signs and symptoms such as bone pain, pallor, petechiae, recurrent fevers, weight loss, hepatosplenomegaly, or generalized lymphadenopathy raise the suspicion for a malignant cause."}
{"id" : 2291, "question_text" : "A 4-year-old girl is accompanied by her father to the pediatrician's office for a health supervision visit. The medical assistant obtains a brief history and takes her vital signs. On entering the room, the practitioner notes that the girl's father is sitting quietly in the corner of the room, holding his bruised wrist, and that facial bruises are visible when he removes his sunglasses, raising concern regarding intimate partner violence. Review of the social history questionnaire reveals that the girl's father immigrated to the United States from France and recently obtained his citizenship. He speaks primarily French but speaks English well enough to not need an interpreter for the visit. He lives in an affluent part of the neighborhood with his partner and their daughter. After examining the child, the practitioner steps out to gather resources for the family and ask the medical assistant to administer the immunizations. Of the following, the MOST likely barrier to the father's disclosing information at this visit about the suspected situation is", "options" : "[\"his immigration history\", \"his language skills\", \"his socioeconomic status\", \"the office environment\"]", "explanation" : "Correct answer is D\n\nPREP Pearl(s)\nIntimate partner violence may involve physical abuse, sexual abuse, psychological abuse, emotional abuse, financial abuse, or stalking.\nBarriers preventing adults from disclosing intimate partner violence to a medical practitioner include fear, language, same-sex relationships, lack of practitioner training, and lack of a safe, confidential environment.\nReporting requirements for intimate partner violence vary by state; most do not mandate a physician to report. Physicians are mandated to report any suspicions of child abuse.\n\nCritique\nThe most likely barrier to the disclosure of intimate partner violence (IPV) that the father in the vignette is facing at this visit is the office environment, including the lack of privacy. Because the practitioner was concerned about the father's bruises, they could have asked the medical assistant to take the child to another room for vaccine administration or to select a book or a sticker, providing a confidential space for a conversation with the father.\n\nIntimate partner violence is a common form of abuse that may involve violence or tactics to control another person. It is a broader term than domestic violence, which requires the people involved to reside in the same household. Intimate partner violence may include the following:\nEmotional abuse\nFinancial abuse\nPhysical abuse\nPsychological abuse\nSexual abuse\nStalking\n\nAdults experiencing IPV face many barriers to disclosing the violence. Signs may be absent or subtle. Various approaches can be used to facilitate disclosure and assist victims in getting help. The office setting can provide an important venue for conversations with patients or family members about IPV. Families can be provided with resources, and practitioners can discuss safety plans and safe contacts (universal education). The opportunity can be provided to confidentially answer screening questions for IPV, which may ask about disagreements, fear, threats, and physical force. If questions are asked face to face, the physician should ensure an environment that is safe and free from judgment and bias; this is best done alone, without children or other family members present, and an interpreter should be provided as needed.\n\nThe other response choices are not likely to be significant barriers for the father in the vignette. He is a US citizen with a low likelihood of deportation. Although not a native English speaker, he does not need a language interpreter, and he is financially stable.\n\nSurvivors of IPV may feel fear and intimidation, experience chronic medical and mental health conditions, and engage in high-risk behaviors. Violence can result in injury or death. Children exposed to IPV are at increased risk of experiencing child maltreatment and its negative effects into adulthood. Teen dating violence is IPV at the adolescent level; however, many adolescents do not realize that they are experiencing abuse. Reporting requirements for IPV vary by state and most do not mandate a physician to report; however, physicians are mandated to report any suspicions of child abuse, and this may exist in the context of IPV in the household.\n\nSuggested Reading(s)\nAmerican Academy of Pediatrics. Violence prevention. Point-of-Care Quick Reference. April 28, 2016. Accessed September 1, 2024. Pediatric Care Online\nAmerican Academy of Pediatrics. Intimate partner violence. Patient Care. Updated January 24, 2024. Accessed September 1, 2024. aap.org/en/patient-care/\nIntimate partner violence. Centers for Disease Control and Prevention. Updated October 9, 2021. Accessed September 1, 2024. cdc.gov/violenceprevention/\nThackeray J, Livingston N, Ragavan MI, Schaechter J, Sigel E; Council on Child Abuse and Neglect, Council on Injury, Violence, and Poison Prevention. Intimate partner violence: role of the pediatrician. Pediatrics. 2023;152(1):e2023062509. doi:10.1542/peds.2023-062509\n\nContent Domain\nViolence\n\nLearning Objectives\nUnderstand the definition of intimate partner violence\nRecognize barriers preventing adults from disclosing intimate partner violence\nBe familiar with laws about reporting intimate partner violence"}
{"id" : 927, "question_text" : "A 10-year-old girl presents to your office with 3 days of knee and ankle pain with swelling. There is no history of trauma. Review of systems is negative for fever, rash, or other signs of systemic illness. She was seen in your office 5 weeks ago for a diarrheal illness that resolved after 5 days. Physical examination reveals warmth, swelling, and slightly decreased range of motion of knees and ankles bilaterally. Vital signs are normal. The girl's complete blood cell count and erythrocyte sedimentation rate are within normal limits.\n\nOf the following, the BEST initial treatment is", "options" : "[\"acetaminophen\", \"amoxicillin\", \"ceftriaxone\", \"naproxen\", \"prednisone\"]", "explanation" : "This patient described in the vignette is well appearing, afebrile, with normal blood cell counts and inflammatory markers, presenting a few weeks after a diarrheal illness. She most likely has reactive arthritis. Naproxen is a nonsteroidal anti-inflammatory drug (NSAID) that is recommended as the first line treatment for reactive arthritis. The drug has both pain relieving and mild anti-inflammatory properties and would be the best initial choice for this patient. With no history of sexual activity, and a physical examination and laboratory studies not consistent with a pyogenic arthritis, no antibiotic therapy is needed. Acetaminophen has pain relieving properties, but no anti-inflammatory properties that would benefit this patient. Prednisone is not recommended as this can mask symptoms of a chronic arthritis which would require referral to a rheumatologist.\n\nReactive arthritis is associated with an infection outside the affected joint. The diagnosis of reactive arthritis is a clinical diagnosis based on mono or oligoarthritis usually of the lower extremities and exclusion of other types of arthritis, such as septic arthritis, Lyme arthritis, acute rheumatic fever, as well as trauma, neoplasm and osteomyelitis. The arthritis is usually asymmetric and affects large joints such as the knee, hip, and ankle. Sacroiliac joints and the joints of the upper extremities can be affected. Reactive arthritis is commonly associated with sexually transmitted infections such as chlamydia and gonorrhea. All patients with a history of sexually transmitted infections and arthritis should be screened for these infections. Reactive arthritis is also associated with other genitourinary, gastrointestinal and upper respiratory infections. The arthritis can appear within days or up to six weeks after the infection. After 6 weeks of joint swelling, the arthritis is considered chronic and a rheumatology referral for possible autoimmune disease should be considered. Treatment of reactive arthritis is supportive with nonsteroidal anti-inflammatory drugs (Item C222), and the use of conservative treatment such as rest and cold therapy. Activity should be limited secondary to pain and can be resumed as pain improves.\n\nPREP Pearls\n• Nonsteroidal anti-inflammatory medications are recommended as first line agents in reactive arthritis.\n• Prednisone can mask symptoms of joint swelling and make the diagnosis of chronic arthritis difficult.\n• All sexually active patients with arthritis should be screened for sexually transmitted infections.\n\nAmerican Board of Pediatrics Content Specification(s):\n• Plan the management of a patient with postinfectious arthritis\n\nSuggested Reading:\n• Berard R. Approach to the child with joint inflammation. Pediatr Clin N Am. 2012;59(2):245-262. doi:10.1016/j.pc1.2012.03.003\n• John J Chandran L. Arthritis in children and adolescents. Pediatr Rev. 2011;32(11):470-480. doi:10.1542/pir.32-11-470"}
{"id" : 3641, "question_text" : "During morning report, a third-year pediatric resident discussed the case of a 16-year-old girl who was admitted to the hospital 2 days ago for abdominal pain. The patient is sexually active and her mother is not aware. The patient's nucleic acid amplification test is positive for Chlamydia trachomatis, and her presentation is consistent with pelvic inflammatory disease. Because of resident work hour limits, this patient had 3 different residents in charge of her care over a 48-hour period. Brief handoffs were performed without using the system that was recently adopted by the hospital to help reduce errors in patient care. A key piece of information not transmitted during handoff was that the mother was not aware of the girl's sexual activity and positive finding of Chlamydia infection. This information does not have to be disclosed to the mother, because adolescents have a legal right to testing and treatment of sexually transmitted infections without parental consent.\n\nThe mother was informed by a resident that the girl tested positive for C trachomatis and pelvic inflammatory disease. The mother became irate that she was not informed that her daughter was sexually active at the time of admission, and the patient was extremely upset that her confidence had been broken. A patient advocate was contacted to assist with calming the mother. The discussion in morning report focused on what adolescents can legally consent to without parental knowledge, and that omission of important information during sign-out can lead to unfavorable situations.\n\nOf the following, the BEST next step in the management of this situation is", "options" : "[\"file a written complaint against the 3 residents involved in the patient's care\", \"initiate a root cause analysis of this sentinel event\", \"recommend that residents send a text message with important facts to the resident taking over patient care\", \"review the hospital's handoff process, highlighting the importance of both verbal and written communication\"]", "explanation" : "The pediatric residents described in this vignette did not protect the confidentiality of the patient and upset her mother, which could have been avoided with good written and verbal communication, and by following the hospital's guidelines for appropriate handoffs between providers.\n\nPediatric providers are advocates for their patients; this includes enhancing patient safety through mindful prevention of misidentification of patients, medication errors, and misdiagnosis. All health care systems have increased efforts to improve patient safety. Medical errors affect one-third of all hospitalized children. Children are more at risk for safety-related problems than are adults; many medications have weight-based dosing; children cannot always communicate when something is wrong; and there are legal issues regarding confidentiality of care of minors. Efforts to improve patient safety are mandated by state and federal agencies such as Medicaid and Medicare; certifying organizations such as The Joint Commission and the American Board of Pediatrics; and professional societies such as the American Academy of Pediatrics.\n\nPatient safety requires that all team members, the patient, and family members are informed, attentive, and communicate openly with one another. Use of a standardized handoff process has been shown to reduce medical errors. Although there are numerous handoff methods, the effectiveness of the I-PASS (© 2014 I-PASS Study Group/Boston Children's Hospital) process is the most evidence based. The I-PASS method was created to standardize verbal handoffs; studies at several pediatric institutions have shown that it reduces communication errors and preventable adverse events. I-PASS stands for: Illness severity; Patient summary; Action list; Situation awareness and contingency planning; and Synthesis by receiver. Having a structured sign-out process that involves direct communication between providers and having the receiver of the handoff, the person who is assuming care, summarize the important patient issues highlighted by the resident transferring the care of the patient, has been an excellent tool in the effort to promote patient safety.\n\nFiling a written complaint against the 3 residents does not highlight what was done incorrectly or provide constructive feedback to the involved residents, thus it is not likely to prevent recurrence in the future. The Joint Commission defines a sentinel event in relation to patient safety as an error in patient care that leads to death, permanent harm, or harm that requires life-saving measures. The event described in the vignette would not meet the criteria for a sentinel event. Performing a handoff by text message is inappropriate for several reasons. It does not allow for clarification requests by the receiver, the text may be missed or accidentally deleted, and it can potentially lead to a breach of patient confidentiality. The best option in this case would be to use this incident as a teaching opportunity and review the hospital's handoff procedures, and highlight or demonstrate how verbal and written communication is effective in improving patient safety and reducing errors.\n\nPREP Pearls\n• A structured verbal handoff process, such as the I-PASS method, is a useful tool in reducing errors arising from poor communication.\n• Patient safety requires that all team members, the patient, and family members are informed, attentive, and communicate openly with one another.\n\nMOCA-Peds Objective\n• Recognize and apply ethical principles involving confidentiality.\n\nABP Content Specifications(s)\n• Understand the importance of creating and maintaining a learning environment (eg, morning report, meetings with partners) in improving patient safety\n\nSuggested Readings\n• I-PASS website. http://www.ipasshandoffstudy.com/home. © 2014 I-PASS Study Group/Boston Children's Hospital.\n• Lautz AJ, Martin KC, Nishisaki A, et al. Focused training for the handover of critical patient information during simulated pediatric emergencies. Hosp Pediatr. 2018;8(4):227-231. doi:10.1542/hpeds.2017-0173.\n• Mueller BU, Neuspiel DR, Fisher ERS; Council on Quality Improvement and Patient Safety. Principles of pediatric patient safety: reducing harm to due to medical care. Pediatrics. 2019;143(2):e20183649. doi:10.1542/peds.2018-3649.\n• Neuspiel DR. Medical errors, adverse events, and patient safety. In: McInerny TK, Adam HM, Campbell DE, DeWitt TG, Foy JM, Kamat DM, eds. American Academy of Pediatrics Textbook of Pediatric Care. 2nd ed. Elk Grove Village, IL: American Academy of Pediatrics; 2017:2287-2295. Pediatric Care Online.\n• Starmer AJ, Spector ND, Srivastava R, Allen AD, Landrigan CP, Sectish TC. I-PASS, a mnemonic to standardize verbal handoffs. Pediatrics. 2012;129(2):201-204. doi:10.1542/peds.2011-2966."}
{"id" : 815, "question_text" : "An 18-year-old homeless girl presents to the emergency department because she \"cannot see\" in her left eye. She complains of slowly progressive decreased visual acuity and the recent development of \"floaters.\" She also reports chronic diarrhea and skin problems. On physical examination, her temperature is 36.5°C, heart rate is 65 beats/min, respiratory rate is 14 breaths/min, and blood pressure is 85/55 min Hg. The examination is notable for a cachectic girl who answers questions appropriately. Funduscopic examination reveals retinitis with extensive areas of hemorrhage and white retinal exudates (Item Q108). Visual acuity is 20/30 in the right eye and 20/400 in the left eye. The oropharyngeal examination reveals white palatal plaques. Auscultation of the lungs reveals coarse breath sounds bilaterally with normal effort. Her abdomen is mildly tender to palpation; the liver and spleen are palpable 3 cm to 4 cm below the costal margins. Neurologic examination is grossly unremarkable except for visual acuity. Examination of the skin reveals multiple erythematous, eroded papules consistent with insect bites that are not healing well. Of the following, the MOST likely cause of this patient's vision loss is infection with", "options" : "[\"Bartonella henselae\", \"Candida albicans\", \"cytomegalovirus\", \"Epstein\\u2013Barr virus\", \"Mycobacterium avium\"]", "explanation" : "Preferred Response: C\nThe patient described in the vignette presents with painless visual loss associated with cachexia, hypothermia, bradycardia, hypotension, oral thrush, hepatosplenomegaly, chronic diarrhea, and skin problems. An underlying immunodeficiency, such as human immunodeficiency virus (HIV) infection and AIDS, is suspected. Therefore, the most likely cause of this patient's vision loss is cytomegalovirus (CMV) infection.\nCMV retinitis occurs in 20% to 40% of patients with AIDS who are not receiving highly active antiretroviral therapy (HAART) and is most common in those patients with CD4 T-lymphocyte counts less than 50/4. It is the most common cause of retinitis in all patients with HIV infection and usually occurs as unilateral disease but will progress to bilateral disease if left untreated. CMV retinitis can cause loss or blurring of vision, blind spots, floaters, and flashing lights (photopsia). Floaters and photopsia are symptomatic predictors of CMV retinitis. Retinal detachment can occur. The diagnosis is made clinically by ophthalmologic examination, which reveals hemorrhage in association with white (or yellow), fluffy retinal lesions, usually close to the retinal vessels (Item C108A). CMV retinitis is associated with little inflammation of the vitreous in patients not receiving HAART.\n\nItem C108B. Opportunistic Infection in Patients with Human Immunodeficiency Virus Infection and AIDS\n• Aspergillus species\n• Candida species\n• Coccidioidomycosis\n• Cryptococcosis\n• Cryptosporidium species\n• Cytomegalovirus\n• Enteric pathogens, other (eg, Salmonella species)\n• Epstein-Barr virus\n• Herpes simplex virus\n• Histoplasmosis\n• Invasive infections due to encapsulated bacteria\n• Isospora species\n• Mycobacterium avium complex\n• Mycobacterium tuberculosis\n• Pneumocystis jirovecii\n• Toxoplasma gondii\n• Varicella-zoster virus\n\nUnlike CMV retinitis, chorioretinitis caused by Candida albicans usually progresses to involve the vitreous. Typical findings include white, infiltrative, moundlike lesions on the retina. In patients with Bartonella henselae (cat scratch disease) infection, only 1% to 2% will develop neuroretinitis. This infection is not thought to be more common in patients with HIV infection and AIDS compared with the general population. Patients usually present with fever, malaise, and acute (unilateral) vision loss from optic nerve edema associated with stellate macular exudates (macular star) on ophthalmologic examination. Other retinal findings can include hemorrhages, multiple lesions deer in the retina, and cotton wool spots. Herpes simplex virus car cause retinitis, especially acute retinal necrosis, but it is less common than CMV retinitis. Epstein-Barr virus and Mycobacterium avium complex are rare causes of retinitis.\n\nIn general, in the era of HAART, the frequency of all opportunistic infections (Item C108B) in patients with HIV infection and AIDS has substantially decreased.\n\nPREP Pearls\n• CMV is the most common cause of retinitis in patients with HIV infection and AIDS.\n• In the era of HAART, the frequency of all opportunistic infections in patients with HIV infection and AIDS has substantially decreased.\n\nAmerican Board of Pediatrics Content Specification(s):\n• Recognize the major opportunistic infections seen in patients with HIV/AIDS\n\nSuggested Reading:\n• American Academy of Pediatrics. Cytomegalovirus infection In: Pickering LK, Baker CJ, Kimberlin DW, Long SS, eds. Red Book: 2012 Report of the Committee on Infectious Diseases. 29th ed. Elk Grove Village, IL: 2012:300-305\n• American Academy of Pediatrics. Human immunodeficiency virus infection. In: Pickering LK, Baker CJ, Kimberlin DW, Long SS, eds. Red Book: 2012 Report of the Committee on Infectious Diseases. 29th ed. Elk Grove Village, IL:2012:418-439"}
{"id" : 3140, "question_text" : "You are taking care of a 10-year-old girl who is in the intensive care unit because of refractory status epilepticus. Her seizures have been difficult to control on traditional anti-epileptic therapies, so she has been placed on a pentobarbital infusion for the past week. She is intubated and on mechanical ventilation because of her coma. Over the past 48 hours, she has developed a fever as high as 38.5°C, appears to be working harder to breathe, and requires increased ventilator support to maintain her oxygen saturation and blood gases.\n\nVital signs show a temperature of 38.5°C, heart rate of 120 beats/min, respiratory rate of 20 breaths/min, and blood pressure of 100/60 mm Hg. Physical examination shows a generally sedated and intubated patient. Pupils are 4 mm equal and sluggishly reactive. Gag reflex is not present. She withdraws minimally to painful stimuli. Cardiovascular examination shows tachycardia with regular rhythm and warm, well-perfused extremities. Lung examination reveals tachypnea, bronchial breath sounds, and crackles in the right lung fields. The left lung is clear to auscultation, with good air movement and no wheezing. Abdomen is soft, nontender, and nondistended with no organomegaly. A chest radiograph is shown in Item Q99.\n\nOf the following, the MOST likely cause of her worsened respiratory status is", "options" : "[\"acute respiratory distress syndrome\", \"atelectasis\", \"pulmonary edema\", \"pulmonary embolism\", \"ventilator associated pneumonia\"]", "explanation" : "The child in this vignette received endotracheal intubation and was placed on mechanical ventilation because of refractory status epilepticus. Over the course of a week, she developed fever, tachypnea, and increased ventilator support, and chest radiography showed right middle lobe consolidation, all of which suggest ventilator-associated pneumonia (VAP).\n\nRespiratory failure is defined as inadequate function of the respiratory system to provide oxygenation or carbon dioxide removal. Ventilatory assistance should be planned to support the respiratory system to achieve both of those functions. In some conditions, this could be achieved with noninvasive treatments such as supplemental oxygen, helium-oxygen mixture, high-flow nasal cannula, or noninvasive positive pressure ventilation. If those measures are inadequate or not feasible, endotracheal intubation and mechanical ventilation may be necessary. The child in the vignette has decreased respiratory drive or apnea primarily from status epilepticus or secondary to anti-epileptic medication. Patients given pentobarbital infusions universally require intubation and mechanical ventilation. In the absence of a primary pulmonary cause of respiratory failure, ventilator settings can include a respiratory rate normal for the child's age, and a fraction of inspired oxygen (FiO2) of 0.21. If volume control ventilation is the chosen mode, a tidal volume of 8 mL/ kg can be prescribed, and in pressure control ventilation, enough pressure to achieve that tidal volume is adequate. Settings can be titrated according to arterial, venous, or capillary blood gas analysis and pulse oximetry.\n\nVentilator-associated pneumonia can be diagnosed by new onset fever, leukocytosis, positive respiratory culture, or new infiltrate on chest radiography. It can cause worsened gas exchange, increased ventilator requirement, increased hospital and intensive care unit (ICU) stay, and even death. Other potential complications of endotracheal intubation and mechanical ventilation include ventilator-induced lung injury, neuromuscular weakness from sedatives required to maintain tube placement, post-extubation upper air-way obstruction, subglottic stenosis, and VAP. Measures to attenuate some of this risk include avoidance of high inflation pressures, high FiO2, oversedation, and agitation. Weaning and early extubation should be attempted as soon as the patient is able. Also, appropriate selection of endotracheal tube size is important, and should follow the general rule that diameter (mm) = 4 + age/4. Protocols also exist to prevent VAP, including elevation of the head of the bed to 30 degrees and measures for oral decontamination, including chlorhexidine oral care. Initial treatment usually includes empiric broad-spectrum antimicrobials to cover for gram-positive and gram-negative nosocomial infections, with subsequent tailoring according to culture results.\n\nAtelectasis can occur during mechanical ventilation because of generally impaired airway clearance and mucous plugging. However, it is not usually associated with fever, and the chest radiograph shown is more indicative of an infiltrative process rather than a discrete segment of atelectasis. Similarly, pulmonary edema and pulmonary embolism do not cause fever. Acute respiratory distress syndrome can occur as a consequence of pneumonia, shock, or systemic inflammation, but 4 criteria must be satisfied, including acute onset, noncardiogenic pulmonary edema, bilateral pulmonary infiltrates, and PaO2/FiO2 ratio of less than 200.\n\nVentilator-associated pneumonia is a potential complication of endotracheal intubation and mechanical ventilation, and is usually marked by fever, impaired gas exchange, increased requirement of ventilator settings, and new infiltrate on chest radiograph. Contemporary ICU practices include protocols to prevent VAP. Daily assessment of extubation readiness to limit ventilator days is the most effective strategy.\n\nPREP Pearls\n• Ventilator-associated pneumonia is a potential complication of intubation and mechanical ventilation, and can be prevented by minimizing ventilator days.\n• In respiratory failure without primary lung pathology, initial ventilator settings can be chosen to mimic a normal breathing pattern and gas exchange.\n• Post-extubation upper airway obstruction is a potential complication of endotracheal intubation and mechanical ventilation, and can be prevented by appropriate sizing of endotracheal tube and minimizing agitation when intubated.\n\nAmerican Board of Pediatrics Content Specification(s)\n• Plan the appropriate ventilatory support for patients with various conditions\n• Understand the potential complications associated with endotracheal intubation\n\nSuggested Reading\n• Carlo WA, Ambalavanan N. Conventional ventilation: traditional and new strategies. Pediatr Rev. 1999;20(12):e117-e126. doi:10.1542/pir.20-12-e117.\n• Nitu ME, Eigen H. Respiratory failure. Pediatr Rev. 2009;30(12):470-478. doi:10.1542/pir.32-6-240.\n• Sarnaik AP, Mastropietro CM. Mechanical ventilation. In: Kliegman RM, Stanton BF, St. Geme JW III, Schor NF, Behrman RE, eds. Nelson's Textbook of Pediatrics. 19th ed. Philadelphia, PA: Saunders Elsevier; 2011:329"}
{"id" : 2989, "question_text" : "A 17-year-old adolescent boy is seen in the clinic with a several-year history of epigastric pain that has significantly worsened over the last 6 months. He was prescribed proton pump inhibitor therapy 2 months ago. Despite good medication adherence, he has continued to have abdominal pain. He has tried over-the-counter antacids with some mild and transient improvement in symptoms. He denies vomiting, regurgitation, heartburn, and weight loss. He moved from Nepal 2 years ago. The adolescent is otherwise healthy and has no known drug allergies. His physical examination is notable for a weight of 53.7 kg (11th percentile for age), height of 165 cm (10th percentile for age), and body mass index of 19.7 kg/m2 (30th percentile for age). His abdomen is soft, with epigastric tenderness. There is no hepatosplenomegaly. The remainder of his examination findings are within normal limits.\n\nRecent laboratory studies show the following:\nLaboratory Test Result\nHemoglobin 15.5 g/dL (155 g/L)\nAlanine aminotransferase 27 U/L\nAspartate aminotransferase 32 U/L\nAlbumin 4.7 g/dL\n\nEndoscopy reveals nodularity in the gastric antrum and a duodenal ulcer (Item Q191). Biopsies demonstrate chronic gastritis and erosive duodenitis, both with the presence of gram-negative, spiral-shaped bacilli. Eradication therapy is recommended to address his suspected condition. Of the following, the BEST indication for this treatment is", "options" : "[\"chronic gastritis\", \"duodenal ulcer\", \"epigastric pain\", \"regurgitation\"]", "explanation" : "Correct Answer: B\nThe adolescent in the vignette has Helicobacter pylori and a duodenal ulcer, therefore eradication treatment for H pylori is recommended.\n\nHelicobacter pylori, a gram-negative, spiral-shaped bacillus, infects more than half of the world population, and many of the affected individuals are asymptomatic. Most of those affected are adults, because the prevalence of H pylori infection increases with age. The overall prevalence of H pylori infection in North America is 37%; in Africa the prevalence is 70%. Adults are more likely than children to develop complications of H pylori infection, including peptic ulcer disease and gastric cancer. Thus, recommendations for the diagnosis and treatment of H pylori infection differ in children compared with adults.\n\nEradication treatment is recommended for all children with H pylori–associated gastric and/or duodenal ulcer disease by the 2016 European Society for Pediatric Gastroenterology, Hepatology, and Nutrition (ESPGHAN) and the North American Society for Pediatric Gastroenterology, Hepatology, and Nutrition (NASPGHAN) guidelines for the management of H pylori in children. Both the ulceration and symptoms will respond to treatment of H pylori infection. Because the presence of ulceration is a key factor considered in the management plan, making the diagnosis of H pylori infection via endoscopy is the gold standard. Noninvasive testing for H pylori infection (stool antigen testing, urease breath testing) is not indicated for diagnosis of H pylori, but is used to assess treatment response. Endoscopy should be offered as a means to diagnose the cause of the child's symptoms (abdominal pain) and not solely to diagnose H pylori infection.\n\nChildren with chronic abdominal pain (including chronic gastritis or epigastric pain) or regurgitation and histologically proven H pylori infection without a gastric or duodenal ulcer do not always experience symptom improvement with H pylori treatment. Thus, eradication therapy in such cases should be a shared decision between the gastroenterologist and the family. Eradication treatment of H pylori infection includes a proton pump inhibitor and antibiotics for 14 days, as detailed in Item C191A and Item C191B. Treatment effect should be assessed with a urease breath test or H pylori stool antigen test 4 weeks after completion."}
{"id" : 3073, "question_text" : "You are seeing a 15-year-old adolescent boy who has recently developed some academic difficulty. He is getting good grades in math and biology at school, but has just started to have significant struggles with English and social studies. The school difficulties started when expectations in those classes moved toward reflection and creative thinking, rather than just memorizing and following rules. When he is given something truly novel to work on, he has difficulty coming up with strategies to address the new problem. You note from his history that he appears to have a personal sense of invincibility, in that he does not hesitate to take significant physical risks while playing sports or skateboarding. Of the following, the predominant thought process that BEST describes what he is exhibiting is", "options" : "[\"abstract reasoning\", \"concrete thinking\", \"feelings of guilt\", \"inferiority thoughts\", \"unconscious drives\"]", "explanation" : "Preferred Response: B\nAt the beginning of adolescence, children's cognitive abilities tend to be largely \"concrete' Concrete thinking involves applying logic to interpret things objectively rather than intuitively. In concrete thinking one follows and manipulates rules, and memorizes and manipulates stored information. Concrete reasoning typically involves manipulating ideas that could be represented as tangible objects. Concrete thinking often involves a single level of situational analysis based on appearances, while missing entirely that there are other ways of viewing or analyzing a problem.\n\nAbstract thinking is the next stage of thought development, in which one uses hypothetical ideas more readily in reasoning. With abstract thinking one is able to consider several possibilities and outcomes at once, and can understand and manipulate concepts as relative rather than absolute. These are important skills in learning to solve problems in novel situations.\n\nThe 15-year-old boy in the vignette is likely developing academic difficulties in part because he has poorly developed abstract reasoning abilities. Biology and Math focus on memorization of facts and rules, whereas social studies and English high school courses require more concept reflection than fact memorization. A personal sense of invincibility is not necessarily related to concrete thinking. However, those who have well-developed abstract reasoning and reflection skills tend to better appreciate that although they feel healthy now taking a significant physical risk while doing something like skateboarding could create lasting negative consequences. In working with a patient who is still using concrete thinking, healthcare providers should avoid using strategies that require the patient to manipulate abstract concepts, such as weighing risks and benefits of behavior change, in the manner that an adult would.\n\n\"Inferiority thoughts\" refers to the Erik Erikson developmental stage of \"industry versus inferiority:' typically seen in 6 to 11 year olds. Those who struggle to develop a sense of competence in school and social interactions during play will doubt their ability to become successful and may socially withdraw. This young man is 15 years old, and we are not told that he is struggling with classwork because he doubts his ability to be successful.\n\n\"Feelings of guilt\" refers to the earlier Erikson stage of \"initiative versus guilt; typically seen during the preschool years. Children at this stage of development are learning to assert themselves as individuals through play and other social interactions. Those who struggle in this arena may feel ashamed of themselves, and become overly dependent on others.\n\n\"Unconscious drives\" refers to the Freudian theory of the unconscious mind, in which individuals make choices in their lives based on certain needs. They would only become aware of these needs if they spent a significant amount of time reflecting on why they did something that does not on its surface make sense. It is difficult to assert that this young man's school performance difficulties are the result of sexual or oral sublimated unconscious urges.\n\nPREP Pearls\n• Adolescent psychological development is marked by a transition from concrete thinking to abstract thinking.\n• Changes in academic demands requiring more abstract manipulation of concepts can lead some adolescents to suddenly start to struggle in their academics.\n\nAmerican Board of Pediatrics Content Specification(s)\n• Understand the timing of and factors influencing the development of concrete thinking and abstract reasoning in adolescents, and provide health advice accordingly\n\nSuggested Reading\n• Goldman L, Schafer Ai, ed. Goldman's Cecil Medicine. 24th ed. Philadelphia, PA: WB Saunders; 2011.\n• Hazen E, Schlozman 5, Beresin E. Adolescent psychological development: a review. Pediatr Rev. 2008;29:161-168. doi:10.1542/pir.29-5 -161."}
{"id" : 1599, "question_text" : "An 8-year-old boy is brought to your office for evaluation of a rash that was first noted 2 months ago. The rash is mildly pruritic. The boy has been otherwise well and is taking no medications. He is afebrile and has normal growth parameters. There are scaling plaques on his trunk and extremities (Item Q71). Of the following, the MOST likely diagnosis is", "options" : "[\"nummular eczema\", \"pityriasis rosea\", \"psoriasis\", \"seborrheic dermatitis\", \"tinea corporis\"]", "explanation" : "Correct Answer: C\nThe boy in this vignette has an eruption composed of erythematous plaques that have thick scale. Where scale has been removed, there are areas of hemorrhage (ie, Auspitz sign). These findings are consistent with a diagnosis of psoriasis. The lesions of nummular eczema may be round or oval but are not elevated and exhibit crust (dried fluid) rather than scale. Unlike in psoriasis, the plaques of pityriasis rosea are thin and have fine scale that is located at the trailing edges of lesions (ie, the scale does not cover the entire lesion). Especially on the scalp, seborrheic dermatitis may mimic psoriasis. However, in seborrheic dermatitis, scale is fine and often \"greasy,\" and the area of involvement is not as well defined as in psoriasis. The lesions of tinea corporis are annular with fine scale on the elevated borders and central clearing.\n\nPsoriasis is a papulosquamous (ie, elevated lesions with scale) disorder likely caused by a genetic predisposition and an environmental trigger (like infection or trauma). It is believed to be an immune-mediated inflammatory process characterized by epidermal hyperplasia. In 35% to 50% of individuals, the onset is before the age of 20 years. Recently, psoriasis has been linked to comorbidities, including metabolic syndrome and cardiovascular disease. Psoriasis may also occur in some children who have juvenile idiopathic arthritis.\n\nThe following variants of psoriasis most often affect infants, children, and adolescents:\n• Plaque psoriasis, as exhibited by the boy in this vignette, is the most common form. Lesions typically affect the extensor surfaces of the extremities, but may also occur on the scalp, face, umbilicus, and gluteal cleft. Lesions appear in areas of trauma (the Koebner phenomenon), thus explaining the commonly observed involvement of the extensor surfaces of the elbows and knees.\n• Scalp psoriasis may occur alone or accompany plaque psoriasis.\n• Nail psoriasis may be an isolated finding or may precede, coincide with, or follow the onset of disease elsewhere on the body. The most common manifestation is pitting, but individuals may develop thickening, yellowing, or roughness of the nails.\n• Guttate psoriasis (from the Latin guttātus, meaning speckled or spotted) occurs commonly in children. It is often precipitated by pharyngeal or perianal Streptococcus pyogenes infection. It begins as a generalized eruption composed of erythematous macules and papules that may mimic a viral exanthem. With time, the lesions become elevated and develop scale. Guttate psoriasis may resolve spontaneously or evolve into plaque psoriasis.\n• Diaper area psoriasis is characterized by erythematous plaques that involve the convexities and creases. Because the area is occluded, there may be little scale. Psoriasis involving the diaper area may mimic irritant contact dermatitis, seborrheic dermatitis, or candidiasis.\n\nPsoriasis is a lifelong condition that has a chronic, relapsing course. Treatment is designed to reduce inflammation and normalize epidermal proliferation. First-line therapy for the scalp, trunk, or extremities is a mid-potency topical corticosteroid (eg, triamcinolone 0.1%) applied twice daily as needed. For the face, flexures, or groin, a low-potency preparation (eg, hydrocortisone 1% or 2.5%, or desonide 0.05%) is indicated. If the disease is not controlled with a topical corticosteroid alone, a topical calcipotriene, an agent that normalizes epidermal proliferation, may be added. Other topical agents that may be beneficial are calcineurin inhibitors, retinoids, keratolytics, tars, and anthralin. Phototherapy or systemic agents (eg, methotrexate, cyclosporine A, acitretin, or biologics) are reserved for patients with severe disease that does not adequately respond to topical treatment.\n\nPREP Pearls\n• In children and adolescents, psoriasis most often presents with erythematous papules and plaques covered by a thick, adherent scale. The extensor surfaces of the elbows and knees are commonly involved.\n• First-line treatment of psoriasis is with an appropriate topical corticosteroid.\n\nABP Content Specifications(s)\n• Recognize the clinical findings associated with psoriasis\n\nSuggested Readings\n• Section on Dermatology, American Academy of Pediatrics. Psoriasis. In: Mancini AJ, Krowchuk DP, eds. Pediatric Dermatology. A Quick Reference Guide. 3rd ed. Elk Grove Village, IL: American Academy of Pediatrics; 2016:335–340.\n• Tollefson MM. Diagnosis and management of psoriasis in children. Pediatr Clin North Am. 2014;61(2):261–277. doi: http://dx.doi.org/10.1016/j.pcl.2013.11.003."}
{"id" : 788, "question_text" : "A girl with depression has stalled in her progress with cognitive behavioral therapy. She has chronic insomnia that is negatively affecting her mood and uses social media excessively overnight. Which intervention would be most appropriate?", "options" : "[\"St. John's Wort herbal supplements\", \"Dietary changes to improve mood\", \"Discontinuing all use of electronic devices in her room at night\", \"Daytime naps to improve overnight sleep\", \"Transferring psychotherapy to a different counselor\"]", "explanation" : "The girl described in the vignette is suffering from depression. She has stalled in her progress with cognitive behavioral therapy (CBT), perhaps in large part because she has chronic insomnia that is negatively affecting her mood. Insomnia could be a direct symptom of her depression, but it is likely being exacerbated by her excessive use of social media over-night. Sleep specialists recommend treating insomnia first by exploring the patient's sleep habits, and having them discontinue any sleep-interfering behaviors. Stopping all use of electronic devices in her room at night would be a key step toward improving this child's sleep hygiene.\n\nThere is no clear evidence that St John's Wort herbal supplements are helpful for treating depression, and no high-quality studies have reported its use in treating adolescent depression. Dietary changes have not been found to affect depression or insomnia. Daytime naps have a negative impact on overall sleep patterns, and clearly decrease overnight sleep time.\n\nTransferring psychotherapy to a different counselor may be warranted when a patient lacks a \"therapeutic alliance\" with the current provider, but in this vignette the girl appears to like her current provider. This is a relevant issue because research demonstrates that a positive therapeutic alliance is one of the most consistent predictors of psychotherapy treatment success. A stalling of the progress of psychotherapy is common and indicates that there may be a new issue that needs to be addressed (such as this patient's insomnia).\n\nPREP Pearls\n• Depression treatment is assisted by improving sleep hygiene.\n• Restricting access to electronic devices overnight is a simple way to improve sleep hygiene.\n• Daytime naps do not improve overnight insomnia.\n\nAAP Mental Health Competency\n• Identify depression treatments besides referring to a therapist that a pediatrician can recommend\n\nSuggested Reading:\n• Birmaher B, Brent D; AACAP Work Group on Quality Issues, et al. Practice parameter for the assessment and treatment of children and adolescents with depressive disorders. JAm Acad Child Adolesc Psychiatry. 2007;46:1503-1526. doi:10.1097/chi.Ob013e318145aelc\n• Jorm AF, Allen NB, O'Donnell CP, Parslow RA, Purcell R, Morgan Al. Effectiveness of complementary and self-help treatments for depression in children and adolescents. Med I Aust. 2006;185(7):368-372\n• Mindell J, Owens J. A Clinical Guide To Pediatric Sleep: Diagnosis and Management of Sleep Problems. 2nd ed. Philadelphia, PA: Lippincott Williams & Wilkins; 2009\n• Sherill IT, Kovacs M. Nonsomatic treatment of depression. Child Adolesc Psychiatr Clin N Am. 2002;11:579-593"}
{"id" : 3586, "question_text" : "A 13-year-old boy comes to the office for follow-up of attention-deficit/hyperactivity disorder (ADHD). He was diagnosed with ADHD at 7 years of age and has done well with behavioral therapy, school supports, and stimulant medication. His mother reports that her son's grades have been falling recently. He is no longer playing sports so that he can have more time to dedicate to his schoolwork. She is concerned about his motivation; she often finds him in his bedroom texting his friends or sleeping instead of doing his homework. When interviewed alone, the boy states that everything is \"fine\" and school is \"boring.\" His responses to the Patient Health Questionnaire 9 (PHQ-9) indicate a moderate level of depression. When discussing a management plan with his mother, warning signs for suicidal ideation and options for emergent evaluation are reviewed. Of the following, the behavior that would be of MOST concern is", "options" : "[\"conflict at school and home\", \"giving away belongings\", \"trouble falling asleep\", \"use of marijuana\"]", "explanation" : "Correct Answer: B\nBehaviors that are most concerning for suicide include distributing possessions, revealing suicidal thoughts on social media, and being preoccupied with death such as in drawings, play, music, and media. Self-injurious behaviors in young children should also raise concern for suicidal ideation because they may not fully understand what it means to be dead. Trouble falling asleep, sadness or irritable mood, anhedonia, weight or appetite change, fatigue or lack of energy, and poor concentration can be symptoms of depression. Psychosocial stressors such as conflict at school and home, and substance use, are risk factors for suicide.\n\nSuicide is a leading cause of death in adolescence; it is rare in children younger than 10 years. It has a significant impact on peers and family who may feel guilt, anger, and depression as they grieve. Suicide in an adolescent, especially when reported by the media, may be followed by a cluster of peer suicides. This contagion effect is more pronounced with more extensive coverage of the event.\n\nThe rate of suicidal behavior is higher in children with psychosis. Psychotic experiences such as hallucinations (eg, hearing voices or seeing shadows or people who are not there) and delusions (eg, falsely believing that one is being followed or has super powers) may occur with depression in adolescents. It is important to distinguish these events from a psychotic (or thought) disorder such as schizophrenia where the symptoms are accompanied by negative symptoms (eg, social withdrawal, reduced affect, slow movement, apathy) and cognitive difficulties (eg, in concentration, executive functions). A decline in self-care, grades, or job performance along with social withdrawal and the presence of hallucinations or delusions are suggestive of a psychotic disorder. Management includes making sure that the changes in thinking and behavior are not caused by other reasons such as substance use, a brain tumor or other medical condition, medication side effects, or other mental health problems. A psychiatric evaluation to clarify diagnosis and determine a treatment plan (eg, antipsychotic medication, psychological therapy) is essential if concern persists for a psychotic disorder.\n\nPREP Pearls\n• Behaviors that are most concerning for suicide include distributing possessions, revealing suicidal thoughts on social media, and being preoccupied with death such as in drawings, play, music, and media.\n• Suicide in an adolescent, especially when reported by the media, may be followed by a cluster of suicides by peers. This contagion effect is more pronounced with more extensive coverage of the event.\n• Decline in self-care, grades, or job performance along with withdrawal and the presence of hallucinations or delusions are suggestive of a psychotic disorder.\n\nABP Content Specifications(s)\n• Recognize behaviors/warning signs that indicate suicidal attempt/ideation in patients of various ages\n• Recognize behaviors suggestive of psychotic behavior/thought disorders, and manage appropriately\n• Recognize the impact of suicide on peers and family\n\nSuggested Readings\n• Abidi S. Psychosis in children and youth: focus on early-onset schizophrenia. Pediatr Rev. 2013;34(7):296-306. doi:10.1542/pir.34-7-296.\n• American Academy of Pediatrics. Promoting mental health. In: Hagan JF, Shaw JS, Duncan PM, eds. Bright Futures: Guidelines for Health Supervision of Infants, Children, and Adolescents. 4th ed. Elk Grove Village, IL: American Academy of Pediatrics; 2017:115-150. Pediatric Care Online.\n• Maslow GR, Dunlap K, Chung RJ. Depression and suicide in children and adolescents. Pediatr Rev. 2015;36(7):299-310. doi:10.1542/pir.36-7-299.\n• Shain B; Committee on Adolescence. Suicide and suicide attempts in adolescents. Pediatrics. 2016;138(1):e1-e11. doi:10.1542/peds.2016-1420."}
{"id" : 2755, "question_text" : "A 14-year-old adolescent girl is brought to the emergency department after she sustained a head injury when she collided with another player while playing soccer. She had a brief loss of consciousness at the time of the event and later developed somnolence, headache, and vomiting. In the emergency department, other than a tired appearance, the adolescent's physical examination findings are normal. Head computed tomography (CT) scan without contrast demonstrates a hyperdensity in the right frontal lobe with no mass effect. Brain magnetic resonance imaging and angiography, performed to further evaluate the frontal lobe CT finding, demonstrate a tightly packed tangle of blood vessels, with clearly visualized flow voids, within the right frontal lobe without evidence of hemorrhage. Of the following, the MOST likely diagnosis for this adolescent is", "options" : "[\"arachnoid cyst\", \"arterio-venous malformation\", \"cavernous malformation\", \"meningioma\"]", "explanation" : "The adolescent in the vignette has imaging findings consistent with a cerebral arterio-venous malformation (AVM). Arterio-venous malformations are congenital high-flow vascular malformations in which there is a direct connection between the arteries and veins and no intervening capillary bed. The majority of AVMs are caused by sporadic pathologic genetic variants resulting in dysregulated vasculogenesis. However, there are genetic syndromes associated with development of AVMs, most commonly hereditary hemorrhagic telangiectasias.\n\nThe majority of cerebral AVMs are diagnosed incidentally on imaging performed for an unrelated reason but can also be discovered during the diagnostic evaluation for seizures, headaches, or progressive focal neurological deficits or acutely, in the setting of intracranial hemorrhage. There is a bimodal peak in the age at diagnosis: childhood (age 010 years) and young to mid-adulthood (age 30 to 50 years). Manifestations of symptomatic AVMs vary based on age, location, size, and the features of the lesion itself.\n\nThe diagnosis of an AVM is made on neuroimaging. Initial identification is typically on brain magnetic resonance imaging (MRI) or head computed tomography (CT) scan. Angiographic imaging (magnetic resonance angiography or CT angiography) will provide a more detailed image of the AVM size and features. Digital subtraction angiography confirms the diagnosis and aids in treatment planning and assessing prognosis. Surgical or interventional procedures are indicated in the setting of hemorrhage due to a ruptured AVM. For non-ruptured AVMs, based on data in the adult population, conservative medical management is recommended for the prevention of death or symptomatic stroke (Mohr).\n\nAn arachnoid cyst is a fluid-filled sac, often congenital, that occurs in the arachnoid membrane. Arachnoid cysts are common and often discovered incidentally on imaging. Depending on the location of the cyst, symptomatic children can present with headache, seizures, developmental delay, increased intracranial pressure, or \"bobble-head syndrome.\"\n\nCavernous malformations (CM) are slow-flow vascular malformations consisting of a tangled, enlarged, abnormal capillary bed without intervening brain parenchyma. Cavernous malformations may be familial or sporadic. Hemorrhage may occur. Common symptoms of CM include headache, seizures, vision changes, and balance problems. On MRI, cavernous malformations have a \"popcorn\" or \"mulberry-like\" cluster of tiny blood vessels with surrounding hemosiderin deposits. Unlike AVMs, there is no intervening brain parenchyma. The imaging appearance of the child in the vignette's vascular lesion is not consistent with a CM.\n\nMeningiomas are tumors of the meninges. They are classified as benign, atypical, or malignant. Symptoms vary based on tumor location. On MRI, meningiomas appear as dural-based, extra-axial masses that are well circumscribed and homogeneous.\n\nPREP Pearls\n• Arterio-venous malformations are congenital, high-flow, vascular malformations in which there is a direct connection between the arteries and veins and no intervening capillary bed.\n• The majority of arterio-venous malformations are diagnosed incidentally, during a diagnostic evaluation for seizures, headaches, or progressive focal neurological deficits, or acutely, in the setting of intracranial hemorrhage.\n• Manifestations of symptomatic arteriovenous malformations vary based on age, location, size, and the features of the lesion itself.\n\nABP Content Specifications(s)\n• Recognize the clinical features associated with arteriovenous malformations in patients of various ages\n\nSuggested Readings\n• Lee GL, Trevino J, Andrasik W, Chen AYY. Vascular anomalies. In: McInerny TK, Adam HM, Campbell DE, DeWitt TG, Foy JM, Kamat DM, eds. American Academy of Pediatrics Textbook of Pediatric Care. American Academy of Pediatrics; 2021:chap 95. Accessed September 1, 2022. Pediatric Care Online.\n• Mohr JP, Overbey JR, Hartmann A, et al; ARUBA co-investigators. Medical management with interventional therapy versus medical management alone for unruptured brain arteriovenous malformations (ARUBA): final follow-up of a multicentre, non-blinded, randomised controlled trial. Lancet Neurol. 2020;19(7):573-581. doi:10.1016/S1474-4422(20)30181-2.\n• Solomon R, Connolly E. Arterio-venous malformations of the brain. N Engl J Med. 2017;376(19):1859-1866. doi:10.1056/NEJMra1607407."}
{"id" : 1223, "question_text" : "A 5-year-old, previously healthy boy presents to the emergency department with a 2-cm laceration above his left eyebrow, sustained after hitting his head on a granite countertop. His vital signs include a temperature of 37°C, heart rate of 140 beats/min, blood pressure of 90/50 mm Hg, respiratory rate of 30 breaths/min, and oxygen saturation of 100% on room air. He is awake, alert, and anxious appearing. The repair requires sedation because of the boy's age, anxiety level, and proximity of the laceration to the eye. He was given a dose of intravenous (IV) midazolam 0.1 mg/kg, but he remains awake, screaming, and thrashes his head when approached. He is given a second dose of IV midazolam 0.1 mg/kg and falls asleep. His vital signs after the second dose are a temperature of 37°C, heart rate of 90 beats/min, blood pressure of 68/40 mm Hg, respiratory rate of 20 breaths/min, and oxygen saturation of 95% on room air. Upon verbal stimulation, the boy opens his eyes, mumbles a few words, and falls back asleep. His extremities are warm with a capillary refill time of 1 second. Of the following, the MOST appropriate next step is the IV administration of", "options" : "[\"0.9% normal saline bolus, 20 mL/kg\", \"atropine, 0.01 mg/kg\", \"epinephrine infusion, 0.05 \\u00b5g/kg per min\", \"flumazenil, 0.01 mg/kg\", \"naloxone, 0.01 mg/kg\"]", "explanation" : "After receiving 2 doses of midazolam, the boy in the vignette became hypotensive, but is otherwise maintaining his airway and is not on the verge of hemodynamic collapse. The best choice among the options is intravenous administration of a 0.9% normal saline bolus of 20 mL/kg.\nModerate sedation is often required for laceration repairs in children from toddler to early school age, because of anxiety as well as pain. According to the American Academy of Pediatrics, moderate sedation is a state of decreased level of consciousness with appropriate response to physical stimulation or verbal commands. Monitoring should include continuous pulse oximetry, visual assessment of ventilation, and noninvasive blood pressure measurement every 5 minutes.\nFor the boy in the vignette, the intended effect was not achieved after 1 dose of midazolam, as evidenced by continued screaming and thrashing, therefore a second dose was given. Adverse effects of benzodiazepines can include hypotension, bradycardia, and respiratory depression. The following equation can be used to estimate minimum systolic blood pressure for a young child: Systolic blood pressure = 70 mm Hg + (2 × child's age in years)\nThe child in the vignette has a systolic blood pressure of 68 mm Hg, which qualifies him as hypotensive. However, because he is not profoundly hypotensive, there is no indication of significant overdose, and he has adequate clinical perfusion, the boy is not likely on the verge of cardiovascular collapse.\nAn important mechanism of hypotension caused by benzodiazepines is dilation of systemic arterioles and venules. The resulting increase in vascular capacitance can decrease preload, thereby reducing cardiac output. Administration of a fluid bolus will increase preload and therefore cardiac output, thereby increasing the blood pressure. Atropine is not recommended in this case because the child does not have bradycardia. Although epinephrine would increase blood pressure by causing vasoconstriction and increase cardiac output by its inotropic properties, it should be reserved for more profound shock states. Flumazenil is a reversal agent for benzodiazepines, but is not routinely recommended because of rebound effects and a risk of seizures. Naloxone is an opioid reversal agent, and would not be helpful in benzodiazepine overdose.\nPREP Pearls\n Benzodiazepines can cause hypotension by vasodilation, which decreases cardiac preload and therefore cardiac output.\n Flumazenil is a reversal agent for benzodiazepines, but is not routinely recommended because of rebound effects and a risk of seizures.\n Monitoring during moderate sedation should include continuous pulse oximetry, visual inspection of breathing, and noninvasive blood pressure readings.\nABP Content Specifications(s)\n Plan the appropriate pre-sedation protocol for a patient who is about to undergo moderate sedation\n Understand the indications and contraindications for moderate sedation\n Recognize the side effects associated with an overdose of commonly prescribed sedatives, and manage appropriately"}
{"id" : 1577, "question_text" : "You are working with the resident team in the neonatal intensive care unit caring for a term infant with long-segment Hirschsprung disease. The infant has had a total colectomy with an ileostomy. He is currently receiving both enteral nutrition via a gastrostomy tube and parenteral nutrition via a central venous catheter. The residents ask you how to avoid cholestasis in this infant.\n\nOf the following, the BEST answer is to", "options" : "[\"cycle the enteral nutrition over 20 hours\", \"cycle the parenteral nutrition over 20 hours\", \"increase the dose of intralipids\", \"increase the glucose infusion rate\", \"increase the trace mineral content\"]", "explanation" : "Correct Answer: B\nThe infant in this vignette with Hirschsprung disease is at risk for the development of cholestasis associated with total parenteral nutrition (TPN). This risk is limited by cycling the TPN over 20 hours or less, with a goal of 12 to 14 hours of infusion. Cycling TPN reduces insulin exposure, allowing for mobilization of fat, which reduces cholestasis risk.\n\nParenteral nutrition is used in critically ill children who require nutrition but are unable to tolerate enteral nutrition. Use of TPN is associated with many risks:\n• Catheter-related problems: sepsis, occlusion, thrombus, dysfunction\n• Cholestasis\n• Cholelithiasis/gallbladder sludge\n• Electrolyte abnormalities\n• Hepatitis\n• Hyperglycemia\n• Hypertriglyceridemia\n• Osteopenia/metabolic bone disease\n• Vitamin/micronutrient deficiency\n\nWhenever possible, enteral nutrition should be used to avoid the risks and complications associated with TPN. Enteral support is needed in children with failure to thrive or poor growth, a frequent complication of many medical conditions due to inadequate caloric intake or increased metabolic needs. Enteral support may also be indicated in children who cannot take nutrition orally because of the aspiration risk. Children must have a functional gastrointestinal tract to tolerate enteral nutrition.\n\nEnteral nutrition is associated with a healthier gastrointestinal tract with an improved gut immune system and healthier microbiota. Increasing evidence shows substantial benefit to enteral nutrition over parenteral nutrition.\n\nThere is no risk for cholestasis associated with continuous enteral feeds over 24 hours, thus there is no benefit associated with cycling enteral feeds. Cholestasis risk increases with increased lipid dosing. Both increased glucose infusion rate and increased trace mineral content make it more likely the patient will experience cholestasis.\n\nPREP Pearls\n• Parenteral nutrition is used in critically ill children who require nutrition but are unable to tolerate enteral nutrition.\n• Cycling total parenteral nutrition reduces insulin exposure allowing for mobilization of fat, which reduces cholestasis risk.\n• Whenever possible, enteral nutrition should be used to avoid the risks and complications associated with total parenteral nutrition.\n• Enteral nutrition is associated with a healthier gastrointestinal tract with an improved gut immune system.\n\nABP Content Specifications(s)\n• Understand the indications for providing enteral nutritional support\n• Judge the advantages of enteral nutrition over parenteral nutrition\n\nSuggested Readings\n• Becker P, Carney LN, Corkins MR, et al. Consensus statement of the Academy of Nutrition and Dietetics/American Society for Parenteral and Enteral Nutrition: indicators recommended for the identification and documentation of pediatric malnutrition (undernutrition). Nutr Clin Pract. 2015;30(1):147–161. doi: http://dx.doi.org/10.1177/0884533614557642.\n• ElHassan NO, Kaiser JR. Parenteral nutrition in the neonatal intensive care unit. NeoReviews. 2011;12(3):e130–e140. doi: http://dx.doi.org/10.1542/neo.12-3-e130.\n• Lauriti G, Zani A, Aufieri R, et al. Incidence, prevention, and treatment of parenteral nutrition–associated cholestasis and intestinal failure–associated liver disease in infants and children: a systematic review. JPEN J Parenter Enteral Nutr. 2014;38(1):70–85. doi: http://dx.doi.org/10.1177/0148607113496280.\n• Rangel SJ, Calkins CM, Cowles RA, et al. Parenteral nutrition–associated cholestasis: an American Pediatric Surgical Association Outcomes and Clinical Trials Committee systematic review. J Pediatr Surg. 2012;47(1):225–240. doi: http://dx.doi.org/10.1016/j.jpedsurg.2011.10.007.\n• Wales PW, Allen N, Worthington P, et al. A.S.P.E.N. clinical guidelines support of pediatric patients with intestinal failure at risk of parenteral nutrition–associated liver disease. JPEN J Parenter Enteral Nutr. 2014;38(5):538–557. doi: http://dx.doi.org/10.1177/0148607114527772."}
{"id" : 1614, "question_text" : "You are evaluating a 10-day-old term newborn who is in the emergency department because of decreased activity, poor feeding, and respiratory distress. The baby was born by normal spontaneous vaginal delivery with no pregnancy or delivery complications. Maternal history is negative for premature or prolonged rupture of membranes, group B Streptococcus colonization, genital herpes, hepatitis B surface antigen, human immunodeficiency virus, and rapid plasma reagin.\n\nThe newborn is critically ill and has a temperature of 35.3°C. He is in respiratory failure and shock. Skin examination findings are normal. Laboratory data are significant for leukopenia, thrombocytopenia, disseminated intravascular coagulation, and severe hepatitis. A chest radiograph shows bilateral pulmonary infiltrates. Blood and urine cultures were obtained, but the newborn is not stable enough for lumbar puncture.\n\nOf the following, the BEST initial antimicrobial treatment is ampicillin, cefotaxime, and", "options" : "[\"acyclovir\", \"amphotericin B\", \"oseltamivir\", \"trimethoprim-sulfamethoxazole\", \"vancomycin\"]", "explanation" : "Correct Answer: A\nThe neonate described in this vignette has a clinical picture suggestive of severe sepsis with shock, respiratory failure, pneumonitis, hepatitis, and disseminated intravascular coagulation. These findings are concerning for both viral or bacterial sepsis. Physicians must recognize that herpes simplex virus (HSV) can present in neonates with severe hepatitis, disseminated intravascular coagulation, pneumonitis, and sepsis syndrome. Thus, initiating empiric intravenous acyclovir therapy after obtaining diagnostic studies for HSV, in addition to antibacterial treatment with ampicillin and cefotaxime, is the preferred treatment for the neonate in this vignette. In addition to HSV, other viruses such as enterovirus and adenovirus can also cause severe sepsis with hepatitis and coagulopathy.\n\nThe incidence of neonatal herpes in the United States is estimated to vary from 1 in 3,000 to 20,000 live births. Approximately 70% of newborns with perinatal HSV infection are born to mothers with asymptomatic genital herpes infection near delivery. Neonatal herpes can be caused by HSV1 or HSV2; approximately 75% of cases are caused by HSV2. Approximately 85% of neonatal HSV infection is acquired at delivery by fetal exposure to the virus in the maternal genital tract. Additionally, HSV may be acquired via ascending infection with membrane rupture or with apparently intact membranes. In 5% of cases, HSV transmission occurs during pregnancy. Postnatal transmission from a parent or other caregiver (often from nongenital infection) occurs in 10% of cases. The risk of neonatal herpes is highest (25%-60%) from mothers with primary genital HSV infection as compared to mothers with recurrent genital HSV infection (< 2%).\n\nNeonatal herpes manifests as skin, eye, and mouth disease in 45% of cases; disseminated disease in 25% of cases; and central nervous system (CNS) disease with or without skin lesions in 30% of cases. Disseminated disease, which often presents around 10 to 12 days after birth, is the most severe form of neonatal HSV infection and affects many organ systems, especially the lung, liver, and brain. The clinical presentation is often characterized by sepsis syndrome with pneumonitis, hepatitis, severe coagulopathy, and encephalitis. Skin lesions may be absent at disease onset, but approximately 66% of disseminated disease cases have cutaneous vesicles. Early clinical diagnosis of disseminated neonatal HSV infection is difficult because the clinical presentation is often nonspecific and confused with bacterial infection. Skin vesicles may be absent in disseminated disease, as seen in the patient in this vignette.\n\nNeonatal HSV disease may be diagnosed by detection of virus from vesicles (if present) or surface swabs (conjunctivae, mouth, nasopharynx, or anus) by culture or polymerase chain reaction (PCR). Lumbar puncture should be performed in all cases of suspected neonatal herpes to evaluate for CNS involvement. The diagnostic evaluation of neonatal HSV infection should include HSV PCR assays of cerebrospinal fluid and whole blood and determination of serum alanine transferase levels. Positive PCR test results or viral cultures from surface swabs collected more than 12 to 24 hours after delivery are indicative of neonatal HSV infection. Neuroimaging and ophthalmologic evaluation is recommended to exclude HSV ocular disease.\n\nAll neonates with suspected or confirmed HSV infection must receive intravenous acyclovir (60 mg/kg/d in 3 divided doses). The recommended length of acyclovir therapy is 21 days for CNS or disseminated disease and 14 days for skin, eye, and mouth disease. A repeat lumbar puncture is recommended for infants with CNS disease at the completion of a 3-week acyclovir course to demonstrate negative cerebrospinal fluid HSV PCR test results. If the HSV PCR results remain positive, intravenous acyclovir is continued for 1 additional week and followed by repeated cerebrospinal fluid testing. In such cases, a pediatric infectious disease consultation is recommended.\n\nFollowing completion of intravenous acyclovir, all infants with neonatal HSV infection (disseminated disease; skin, eye, and mouth disease; or CNS disease) must receive oral acyclovir suppressive therapy for 6 months. Improvement in neurodevelopmental outcomes in babies with CNS disease and prevention of cutaneous recurrences in all 3 forms of HSV disease have been reported with oral acyclovir suppressive therapy. Infants must be closely monitored with serial complete blood cell counts for neutropenia while receiving acyclovir suppressive therapy. With receipt of intravenous acyclovir therapy, the risk of mortality in disseminated neonatal HSV infection has decreased from 85% to 30%, and neurologic development is normal in 85% of survivors. The risk of mortality is greatest when the neonatal HSV infection presents with lethargy and severe hepatitis.\n\nIn a term infant, the differential diagnosis of sepsis must include late-onset bacterial sepsis. However, the clinical presentation and laboratory findings in the neonate described in this vignette are more consistent with disseminated herpes than methicillin-resistant Staphylococcus aureus infection. Initiating acyclovir therapy in conjunction with intravenous ampicillin and cefotaxime to cover for common pathogens associated with neonatal sepsis (such as group B Streptococcus, Escherichia coli, Listeria monocytogenes, or Enterococcus) is the preferred response over vancomycin for empiric treatment of this infant.\n\nCandidiasis is a major cause of morbidity and mortality among low birth-weight infants in the neonatal intensive care unit. However, invasive fungal infection would be very unusual in an otherwise healthy term infant during the first weeks after birth. Thus, amphotericin therapy to empirically treat fungal infection would not be recommended for the neonate in this vignette. Rarely, influenza infection in infants can manifest as a sepsis-like syndrome associated with pneumonia; however, initiating empiric oseltamivir would not be a preferred response in this case. Pneumocystis jirovecii infection, an opportunistic infection in immunocompromised individuals, is rare in the first month after birth and an unlikely diagnosis in this infant given the negative maternal HIV antibody serology. Thus, trimethoprim-sulfamethoxazole therapy to empirically treat P jirovecii pneumonia would not be recommended.\n\nPREP Pearls\n• Sepsis caused by herpes simplex virus should be in the differential diagnosis of any critically ill infant with an acute presentation of sepsis syndrome, rapidly progressive pneumonitis, hepatitis, and disseminated intravascular coagulation.\n• Disseminated neonatal herpes simplex virus infection is the most severe form of neonatal herpes and is associated with high morbidity and mortality.\n• Early recognition of disseminated herpes simplex virus disease in the neonate can be difficult, because the clinical presentation often mimics bacterial sepsis with the absence of cutaneous vesicles.\n• Neonates with suspected or confirmed herpes simplex virus infection must receive intravenous acyclovir. Infants with neonatal herpes simplex virus infection must receive oral acyclovir suppressive therapy for 6 months after completion of intravenous acyclovir for acute herpes simplex virus infection.\n\nMOCA-Peds Objective\n• Recognize respiratory distress and manage appropriately\n\nABP Content Specifications(s)\n• Plan the appropriate diagnostic evaluation for herpes simplex virus infection\n• Plan the appropriate management of herpes simplex virus infection in children of various ages, taking into account appropriate timing of therapy\n• Recognize the clinical features associated with herpes simplex virus infection in children of various ages\n\nSuggested Readings\n• American Academy of Pediatrics. Herpes simplex. In: Kimberlin DW, Brady MT, Jackson MA, Long SS, eds. Red Book: 2015 Report of the Committee on Infectious Diseases. 30th ed. Elk Grove Village, IL: American Academy of Pediatrics; 2015:432–445.\n• Kimberlin DW, Lin CY, Jacobs RF, et al. Natural history of neonatal herpes simplex virus infections in the acyclovir era. Pediatrics. 2001;108(2):223–229. doi: http://dx.doi.org/10.1542/peds.108.2.223.\n• Kimberlin DW, Whitley RJ, Wan W, et al. Oral acyclovir suppression and neurodevelopment after neonatal herpes. N Engl J Med. 2011;365(14):1284–1292. doi: http://dx.doi.org/10.1056/NEJMoa1003509."}
{"id" : 2283, "question_text" : "A 17-year-old with no previous medical problems is seen for a health supervision visit at the local youth detention center. He complains of nausea, decreased appetite, diarrhea, and muscle soreness in his back and legs. On physical examination, he has a temperature of 37.8 °C, heart rate of 101 beats/min, respiratory rate of 18 breaths/min, and blood pressure of 130/78 mm Hg. He appears tired, is mildly diaphoretic, and has mild rhinorrhea. His pupils are 5 mm in diameter bilaterally and reactive to light. Heart, lung, and abdominal findings are normal. He denies suicidal ideation, thoughts of self-harm, or harm to others. He reports chronic use of a medication which he stopped 2 days ago. Of the following, the medication MOST likely discontinued by this adolescent was", "options" : "[\"alprazolam\", \"cannabis\", \"cocaine\", \"oxycodone\"]", "explanation" : "The adolescent in the vignette's signs and symptoms are consistent with opioid withdrawal: muscle soreness, nausea, vomiting, diarrhea, diaphoresis, mydriasis (pupillary dilatation), and mild elevations in blood pressure, pulse, and temperature. Oxycodone and hydrocodone are two commonly prescribed opioid medications. Other opioids include hydrocodone, tramadol, heroin, and fentanyl.\n\nAlprazolam is a short-acting benzodiazepine in the sedative/hypnotic class of substances. The signs and symptoms of benzodiazepine intoxication can appear similar to opioid intoxication (eg, somnolence, altered mental status, slurred speech, ataxia). Vital signs may be normal or exhibit a mildly decreased heart rate and blood pressure. Benzodiazepine withdrawal symptoms also may appear similar to opioid withdrawal. These may include irritability, increased anxiety, sleep disturbance, elevations in heart rate, and palpitations. Chronic, heavy benzodiazepine use may lead to life-threatening withdrawal seizures, if not treated appropriately. Pupillary changes typically do not occur with benzodiazepines. A summary of pupillary findings in common substance-related conditions is found in the Table.\n\nCannabis intoxication presents with behavioral symptoms that range from mild euphoria and disinhibition to psychosis. While cannabis can cause conjunctival injection, it typically does not lead to pupillary changes. Cocaine is a stimulant that causes mydriasis and elevations in blood pressure and heart rate with intoxication; it causes miosis and depressed mental status in the withdrawal state.\n\nThe opioid overdose triad includes pinpoint pupils, respiratory depression, and a decreased level of consciousness. When there is any suspicion for opioid overdose, the individual should be rapidly treated with naloxone, a μ-opioid receptor blocker with very high affinity for the receptor. Naloxone can be administered by intramuscular injection or intranasal spray; there are no side effects or safety concerns if given to an individual who has not used opioids. It is available for use without a prescription. Naloxone should be recommended and, if possible, provided to any patient with a personal history of opioid or prescription drug misuse or overdose, as well as any patient with a family member or friend with substance use, including but not limited to opioids. Several states now require naloxone to be co-prescribed with certain opioid prescriptions.\n\nThere has been a considerable increase in opioid overdose deaths in the United States, driven by an unprecedented increase in adolescent overdoses. Most overdose deaths among adolescents involve counterfeit pills laced with illicitly manufactured fentanyl. There is a rising incidence of adolescents taking counterfeit alprazolam, amphetamine, or other pills (bought from or provided by a friend or stranger) that are laced with a lethal dose of fentanyl, which leads to their death. Importantly, opioids are being used more frequently in combination with other drugs, most commonly a stimulant (eg, methamphetamine) or a synthetic drug with effects opposite to opioids in an attempt to counterbalance the sedative effects and thus extend or heighten the euphoric effects.\n\nMost individuals with opioid use on a daily or near-daily basis meet criteria for opioid use disorder (OUD). Most individuals with OUD first used opioids as adolescents. Those who use opioids on a daily basis for approximately 3 to 6 months or more will have various degrees of opioid withdrawal when abruptly stopping use. For adolescents and adults, the best measure of withdrawal is the Clinical Opioid Withdrawal Scale [(COWS) See Wesson 2003]. A score between 5-12 = mild withdrawal; 13-24 = moderate withdrawal; 25-36 = moderately severe withdrawal; >36 = severe withdrawal. Medication should be considered to treat opioid withdrawal for an individual with a score of 8 or more.\n\nThe best treatment for an individual with opioid withdrawal is buprenorphine. It treats withdrawal symptoms, alleviates opioid cravings, and is safe and effective. Buprenorphine is a long-acting partial μ-opioid receptor-agonist with a very high affinity for the receptor; it is higher than the full agonist drug. It is Federal Drug Administration (FDA)-approved for use in individuals aged 16 years and older and is recommended by the American Academy of Pediatrics as a medication for OUD treatment. Use of buprenorphine increases retention of adolescents and young adults in OUD treatment programs; longer treatment courses are associated with higher rates of retention. Treatment with buprenorphine is associated with decreased illicit substance use and injection drug events in adolescents. The US federal government has removed the requirement for a special Drug Enforcement Agency (DEA) \"prescribing waiver\" for buprenorphine. Now, all medical providers with a federal DEA number can prescribe buprenorphine after completing 8 hours of substance use training.\n\nBarriers to buprenorphine use include the time necessary for education on induction and the management of patients on buprenorphine, as well as the cost and availability of the medication itself. Even when covered by insurance, many plans require prior authorization, and some pharmacies do not routinely carry buprenorphine.\n\nOther medications used to treat OUD include methadone (a full agonist taken daily; it is not FDA-approved for individuals younger than 18 years ) and naltrexone (a full opioid antagonist). Naltrexone comes in both a daily pill form and an extended-release injectable form that lasts 28 to 30 days. Barriers to naltrexone use include the need for full wash-out of the opioid agonist, because of risk of withdrawal if the drug is given with any full opioid substance left in the body.\n\nAlpha-2 adrenergic receptor agonists (eg, clonidine) can assist in the treatment of opioid withdrawal. Clonidine dosing starts at 0.1 mg orally once nightly and can be given up to every 6 hours for withdrawal symptoms if pulse and blood pressure are maintained appropriately, as α-2 adrenergic receptor agonists can lower blood pressure and pulse. Other medications that target the symptoms of withdrawal include over-the-counter medications for muscle pain (eg, nonsteroidal anti-inflammatory drugs) and ondansetron for nausea and vomiting.\n\nTraining and information on buprenorphine and other treatments for opioid use disorder in adolescents are available at www.aap.org/mat, the Opioid Response Network, and the Providers Clinical Support System.\n\nSuggested Reading(s)\nAmerican Academy of Pediatrics Committee on Substance Use and Prevention. Medication-assisted treatment of adolescents with opioid use disorders. Pediatrics. 2016;138(3):e20161893. doi:10.1542/peds.2016-1893\nBagley S, Levy S. Substance use disorders. In: McInerny TK, Adam HM, Campbell DE, DeWitt TG, Foy JM, Kamat DM, eds. American Academy of Pediatrics Textbook of Pediatric Care. American Academy of Pediatrics; 2021:chap 336. Accessed September 21, 2024. Pediatric Care Online\nBuprenorphine Quick Start Guide. Substance Abuse and Mental Health Services Agency. www.samhsa.gov/sites/default/files/\nSociety for Adolescent Health and Medicine. Medication for adolescents and young adults with opioid use disorder. J Adolesc Health. 2021;68(3):632-636. doi:10.1016/j.jadohealth.2020.12.129\nTrope LA, Stemmle M, Chang A, et al. A novel inpatient buprenorphine induction program for adolescents with opioid use disorder. Hosp Pediatr. 2023;13(2):e23-e28. doi:10.1542/hpeds.2022-006864\nWesson DR, Ling W. The clinical opiate withdrawal scale (COWS). J Psychoactive Drugs. 2003;35(2):253-259. doi: 10.1080/02791072.2003.10400007.\n\nContent Domain\nSubstance Use and Addictions\n\nLearning Objectives\nRecognize clinical findings associated with opioid withdrawal"}
{"id" : 3793, "question_text" : "A 14-year-old adolescent girl is brought to the emergency department with a 1-day history of fever and left eye swelling, with an inability to open the eye. Two weeks ago, she had a mild upper respiratory tract infection. On physical examination, she is alert and interactive with a temperature of 39.2°C. Examination of the left eye reveals periorbital redness and swelling, proptosis, conjunctival edema, and limited extraocular movements. The remainder of the physical examination findings are normal. Laboratory findings are notable for a white blood cell count of 18,000/μL (18 × 10^9/L) and elevated C-reactive protein level. Of the following, the MOST likely source of this patient's infection is the", "options" : "[\"bloodstream\", \"ethmoid sinus\", \"maxillary sinus\", \"skin\"]", "explanation" : "Correct Answer: B\nThe adolescent girl in the vignette has a fever, unilateral periorbital swelling, and redness, with proptosis, restricted extraocular movements, and conjunctival chemosis after an upper respiratory tract infection. These physical examination findings and patient history are consistent with a diagnosis of orbital (postseptal) cellulitis. Orbital cellulitis is a serious infection of the orbital tissue posterior to the orbital septum, usually complicating sinusitis (especially the ethmoid sinus). The orbital septum is a protective layer of periosteal fibrous connective tissue extending to the upper and lower eyelids. The ethmoid sinus is separated from the orbit by a thin medial orbital wall (lamina papyracea). The microbiology of orbital cellulitis includes organisms associated with acute or chronic sinusitis including Streptococcus pneumoniae, Staphylococcus aureus, Streptococcus anginosus, Moraxella catarrhalis, nontypeable Haemophilus influenzae, and upper respiratory tract anaerobes.\n\nThe clinical manifestations of orbital cellulitis include proptosis, conjunctival chemosis, ophthalmoplegia, and pain with eye movement. In patients with marked eye swelling, it may be difficult to distinguish periorbital cellulitis from orbital cellulitis by physical examination alone because of difficulties in assessing visual acuity or extraocular movements. All patients with a suspected diagnosis of orbital cellulitis should undergo orbital computed tomography imaging with contrast to confirm the diagnosis and exclude orbital complications such as subperiosteal abscess and orbital abscess.\n\nManagement of orbital cellulitis warrants an urgent and multidisciplinary approach, including specialist consultations from pediatric infectious disease, otolaryngology, and ophthalmology. Empiric antimicrobial therapy of orbital cellulitis should be rapidly initiated; intravenous ampicillin-sulbactam and vancomycin are recommended to treat methicillin-resistant S aureus and other microorganisms associated with sinusitis. A 5- to 7-day course of parenteral antibiotic therapy (until the eye examination results are greatly improved), followed by 2 weeks of oral antibiotic therapy, is recommended. Surgical drainage of the affected sinus and abscess is indicated in patients with large, well-defined subperiosteal and/or orbital abscess, visual impairment, or complete ophthalmoplegia. Medical management alone is often adequate in patients with orbital cellulitis associated with phlegmon or small subperiosteal abscess, if the patient clinically improves within 48 to 72 hours.\n\nDistinguishing periorbital cellulitis from orbital cellulitis is critical to avoid potentially serious complications of the latter, including vision loss and intracranial infection. The microbiology, pathogenesis, diagnostic evaluation, and management of periorbital cellulitis and orbital cellulitis are very different. Periorbital cellulitis is an infection of the eyelid and surrounding skin and soft tissue anterior to the orbital septum. It occurs most commonly in preschool-aged children and typically begins at the site of local skin trauma (eg, an insect bite) which becomes infected. The most common etiologic agents causing periorbital cellulitis include S aureus (including methicillin-resistant S aureus) and group A Streptococcus. In the era of H influenzae type b vaccination and pneumococcal conjugate vaccination, bacteremic periorbital cellulitis is very unusual. Periorbital cellulitis may be treated with appropriate oral antibiotics for the likely causative organisms.\n\nPREP Pearls\n• Orbital cellulitis is a serious infection of the orbital tissue posterior to the orbital septum usually complicating sinusitis (especially ethmoid sinus).\n• The microbiology of orbital cellulitis includes organisms commonly associated with acute or chronic sinusitis.\n• Management of orbital cellulitis warrants an urgent and multidisciplinary approach with consultations with specialists in pediatric infectious disease, otolaryngology, and ophthalmology.\n\nABP Content Specifications(s)\n• Recognize pathogens commonly associated with orbital cellulitis\n• Recognize and differentiate the clinical and radiologic findings associated with orbital cellulitis and periorbital (preseptal) cellulitis\n• Plan the appropriate management of orbital cellulitis, including associated complications\n• Plan the appropriate diagnostic evaluation of orbital cellulitis\n\nSuggested Readings\n• Adamson J, Waterfield T. Fifteen-minute consultation: preseptal and orbital cellulitis [published online ahead of print June 22]. Arch Dis Child Educ Pract Ed. 2019;104(2):79-83. doi:10.1136/archdischild-2017-314297.\n• Hauser A, Fogarasi S. Periorbital and orbital cellulitis. Pediatr Rev. 2010;31(6):242-249. doi:10.1542/pir.31-6-242.\n• Wald ER. Preseptal and orbital cellulitis. In: McInerny TK, Adam HM, Campbell DE, DeWitt TG, Foy JM, Kamat DM, eds. American Academy of Pediatrics Textbook of Pediatric Care. 2nd ed. Elk Grove Village, IL: American Academy of Pediatrics; 2017:2537-2543. Pediatric Care Online.\n• Wong SJ, Levi J. Management of pediatric orbital cellulitis: a systematic review. Int J Pediatr Otorhinolaryngol. 2018;110:123-129. doi:10.1016/j.ijporl.2018.05.006."}
{"id" : 1941, "question_text" : "A 3-year-old girl has had fever for 6 days. Her father states that for the past 3 days her temperature has been at least 38.9°C, and it is not improving. He indicates that her eyes are red and she has a rash. He says she has been very cranky and that her appetite is decreased. He reports no vomiting, diarrhea, dysuria, cough, or trouble breathing. Her immunizations are up-to-date. The family was on vacation at a crowded amusement park for the first 3 days of her illness. She was seen at an urgent care center near the park where no etiology for her fever was found and no testing was done. Her temperature is now 38.9°C. She is irritable but interactive and cooperates with the examination. She has injected conjunctiva bilaterally without exudate, erythematous and cracked lips, and a polymorphous rash on her trunk and legs. The remainder of the physical examination findings are normal. Of the following, the MOST appropriate next steps in the diagnostic approach for this child include", "options" : "[\"chest radiography, complete blood cell count with differential, blood culture, and urine culture\", \"complete blood cell count with differential, comprehensive metabolic panel, C-reactive protein level, and urinalysis\", \"nasopharyngeal swab for influenza A and B, rapid streptococcal antigen test, and heterophile antibody test\", \"serum measles IgM antibody, complete blood cell count with differential, and nasopharyngeal swab for viral culture\"]", "explanation" : "The girl in this vignette demonstrates clinical signs and symptoms consistent with Kawasaki disease (KD). A complete blood cell count with differential, comprehensive metabolic panel, C-reactive protein level, erythrocyte sedimentation rate, and urinalysis will add to the diagnosis and guide treatment. Kawasaki disease is associated with vasculitis of coronary and other medium-sized, extraparenchymal arteries. Early initiation of treatment with intravenous immunoglobulin and high-dose aspirin can mitigate long-term effects on coronary vessels.\n\nThe etiology of KD remains unknown. There is no gold-standard diagnostic test; thus, the diagnosis is based on clinical and laboratory criteria. Kawasaki disease should be suspected in a child with a fever, typically greater than 39°C, for at least 5 days' duration. The diagnosis is made from the fever along with other signs and symptoms as described in suggested reading 1 (http://circ.ahajournals.org/content/110/17/2747). More than 90% of children with KD have bilateral, nonexudative, limbic-sparing conjunctivitis (Item C141). A diffusely erythematous oropharynx with cracked lips and a strawberry tongue are often seen, but oral ulcers and tonsillar exudate are not typical for KD. The hands and feet are often swollen early in the course, and a desquamating rash on distal fingers and toes is common. Bullae and vesicles are not typically seen.\n\nClinical features including arthritis and arthralgia, vomiting and diarrhea, abdominal pain, extreme irritability, testicular swelling, and a desquamating rash in the groin, are associated with KD and can mimic other conditions. Children with suspected KD based on clinical and/or laboratory findings should undergo echocardiography to assess for coronary artery abnormalities and overall heart function. If coronary abnormalities are seen, the child should be treated for KD, regardless of whether all criteria are met.\n\nInfants and children with some criteria for KD, but not quite meeting the clinical case definition, should be assessed for \"incomplete KD.\" Nearly all children with KD have elevated inflammatory markers. Therefore, children with a C-reactive protein level less than 3.0 mg/dL (286 nmol/L) and erythrocyte sedimentation rate less than 40 mm/h should be evaluated daily for continued fever and change in laboratory values. If peeling of the distal hands or feet is seen, echocardiography should be performed. If the C-reactive protein level is 3.0 mg/dL (286 nmol/L) or greater or the erythrocyte sedimentation rate is 40 mm/h or greater, the clinician should assess for whether the child meets other laboratory criteria as described in suggested reading 1(http://circ.ahajournals.org/content/110/17/2747). For those with fewer than 3 other criteria, echocardiography is recommended. If vascular changes are seen on echocardiography, treatment is recommended. If no changes are seen, echocardiography should be repeated if fever persists. If 3 or more other laboratory criteria are met, the child should undergo echocardiography and be treated for KD. For infants 6 months of age and younger who have had at least 7 days of fever without other clinical criteria, laboratory testing should be done to determine if laboratory criteria for KD are met.\n\nViral and bacterial infections, particularly adenovirus infection and streptococcal pharyngitis, may also present in a similar fashion to KD and should be excluded before treating for KD. Therefore, a broad viral respiratory panel and rapid antigen testing for streptococcal pharyngitis may be helpful; however, positive results alone do not exclude KD. While Epstein-Barr virus infection can present similarly to KD, a heterophile antibody test has a high false-negative rate in younger children. Because the child in this vignette has no respiratory symptoms and is fully immunized, chest radiography and blood culture are not indicated. A typically developing 3-year-old should be able to report dysuria; in the absence of dysuria, a urinary tract infection is less likely. A serum measles IgM antibody test and, in certain circumstances, viral culture from a nasopharyngeal sample can diagnose measles. However, measles prevalence is much less likely in this vaccinated child and in the absence of an epidemic; decisions around testing for measles in this context should be done in consultation with public health officials.\n\nPREP Pearls\n\nKawasaki disease should be suspected in a child with fever for several days, even when signs and symptoms less common in Kawasaki disease (eg, headache, vomiting) are present.\n\nInfants younger than 6 months with Kawasaki disease can present with fever and few or no accompanying clinical features. Echocardiography is recommended in these infants with fever for 7 or more days and elevated inflammatory marker levels.\n\nABP Content Specifications(s)/Content Area\n\nPlan the appropriate diagnostic evaluation of Kawasaki disease, and interpret the results\n\nRecognize the clinical findings associated with Kawasaki disease\n\nSuggested Readings\n\nNewburger JW, Takahashi M, Gerber MA, et al. Diagnosis, treatment, and long-term management of Kawasaki disease: a statement for health professionals from the Committee on Rheumatic Fever, Endocarditis and Kawasaki Disease, Council on Cardiovascular Disease in the Young. American Heart Association. Circulation. 2004;110(17):2751. (http://circ.ahajournals.org/content/110/17/2747) .\n\nSon MB, Newburger JW. Kawasaki disease. Pediatr Rev. 2013;34(4):151-162. doi:10.1542/pir.34-4-151."}
{"id" : 2278, "question_text" : "A 13-year-old baseball pitcher is undergoing evaluation for right shoulder pain in his dominant throwing arm. The pain, experienced with pitching and long throws from center field, has gradually worsened over the last 2 weeks. Physical examination of the shoulder reveals tenderness to palpation over the right proximal humerus laterally, decreased internal rotation, and pain with external rotation. Right shoulder radiographs demonstrate widening of the proximal humeral physis.", "options" : "[\"continue all baseball activities as tolerated\", \"discontinue pitching during competitions for 4 weeks\", \"immobilize the shoulder for 4 weeks\", \"stop all throwing for 4 weeks and complete physical therapy\"]", "explanation" : "The adolescent in the vignette has clinical and radiographic findings consistent with \"little league\" shoulder. This condition requires cessation of all throwing for approximately 4 weeks to allow physeal healing. A dedicated evaluation of throwing mechanics and physical therapy are recommended to improve mechanics, decrease the force placed across the proximal humeral physis, and increase strength.\n\n\"Little league\" shoulder was initially described as osteochondrosis of the proximal humeral epiphysis. It is also referred to as proximal humeral epiphysiolysis, physeal stress fracture, and a Salter-Harris type 1 physeal injury. This condition commonly develops during the phase of peak height velocity (ages 11-14 years). It arises from stress placed across the hypertrophic zone of the physis from repetitive overhead motion. The physis is prone to injury during this time of adolescent growth. Both intrinsic and extrinsic factors contribute to this injury. Intrinsic factors include age, height, and muscular imbalance. Extrinsic factors include overuse, repetition, poor throwing mechanics, higher velocity of throwing, higher pitch counts, and lack of adequate rest days.\n\nThere are 6 phases of throwing, defined by the position of the arm and leg and the plane of motion: wind-up, stride, cocking, acceleration, deceleration, and follow through. Biomechanical analyses suggest that maximal external rotation during the cocking and acceleration phases exerts force and torque across the physis, which becomes stressed and widens with overhead motion.\n\nChildren with proximal humeral epiphysiolysis experience shoulder pain when throwing. Other activities of daily living are largely unaffected. Physical examination reveals tenderness to palpation at the proximal humeral physis laterally. External rotation against resistance may elicit weakness and pain that localizes to the proximal humeral physis. Scapular dysfunction and a glenohumeral internal rotation deficit (asymmetry of rotation of humerus when compared to contralateral arm) may also be present. Throwing athletes with glenohumeral internal rotation deficit demonstrate greater external rotation and limited internal rotation of their throwing shoulder, a mechanical adaptation secondary to repetitive throwing. A diagnosis of glenohumeral internal rotation deficit is made when an athlete has a >20° loss of internal rotation in the throwing shoulder as compared with the contralateral, nonthrowing arm; this condition is a risk factor for injury.\n\nThe initial evaluation for \"little league\" shoulder should include standard shoulder radiography with 2 views: anteroposterior with external rotation and internal rotation. The anteroposterior view with external rotation evaluates the physis and will demonstrate an irregular, widened physis and metaphyseal sclerosis in affected children. A contralateral anteroposterior view with external rotation can be helpful in comparing the physeal appearance in the throwing shoulder versus the nonthrowing shoulder. Magnetic resonance imaging may serve as an adjunct to evaluate the proximal humeral physis if the radiographic findings are negative.\n\nTreatment of \"little league\" shoulder consists of complete rest from throwing for approximately 4 weeks, as well as rehabilitation. During this treatment phase, athletes can modify their playing position to 1st base, with activity limited to underhand toss and batting. Affected individuals should be advised to stop all repetitive hard throwing (eg, pitcher, catcher, and outfielder positions) during the rest phase. After this period of rest, a biomechanical throwing analysis and physical therapy program are recommended to guide the athlete back to activity. Young athletes are typically back to full throwing and pitching within 3 months of diagnosis. Assessment and management of extrinsic risk factors are essential to prevent recurrence of this condition.\n\nSuggested Reading(s)\nBednar ED, Kay J, Memon M, Simunovic N, Purcell L, Ayeni OR. Diagnosis and management of little league shoulder: a systematic review. Orthop J Sports Med. 2021;9(7):23259671211017563. doi:10.1177/23259671211017563\nBrenner JS; Council on Sports Medicine and Fitness. Sports specialization and intensive training in young athletes. Pediatrics. 2016;138(3):e20162148. doi:10.1542/peds.2016-2148\nColeman N. Sports injuries. Pediatr Rev. 2019;40(6):278-290. doi:10.1542/pir.2018-0221\nKannikeswaran N, Suresh S. Sports musculoskeletal injuries. In: McInerny TK, Adam HM, Campbell DE, Foy JM, Kamat DM, eds. American Academy of Pediatrics Textbook of Pediatric Care. 2nd ed. American Academy of Pediatrics; 2016:chap 334. Accessed September 1, 2024. Pediatric Care Online\nZaremski JL, Zeppieri G Jr, Tripp BL. Sport specialization and overuse injuries in adolescent throwing athletes: a narrative review. J Athl Train. 2019; 54(10):1030-1039. doi:10.4085/1062-6050-333-18\n\nContent Domain\nSports Medicine\n\nLearning Objectives\nDescribe the causes of common overuse injuries in athletes\nManage the care of a patient with an overuse injury"}
{"id" : 2469, "question_text" : "A 7-year-old boy is seen for evaluation of hoarseness that has been present for several months. He has an intermittent, nonproductive cough and has recently had difficulty keeping up with his peers when playing, becoming winded more quickly than his mother and teachers think he should. He has not had fever, recent respiratory or other illness, snoring, noisy breathing, or trauma to his neck.\n\nOn physical examination, he is comfortable at rest. His vital signs are a blood pressure of 105/55 mm Hg, a heart rate of 80 beats/min, a respiratory rate of 15 breaths/min, and an oxygen saturation of 98% in room air via pulse oximetry.\n\nHis body mass index is at the 40th percentile for age. There is a hemangioma over his left cheek and upper lip that extends to the oral surface; it has been present since birth and has not changed. He is able to breathe comfortably through his nose.\n\nChest examination reveals good aeration with no adventitious sounds, a 1:1 inspiratory-to-expiratory ratio, and a slight suprasternal tug on inspiration. The remainder of his physical examination findings are unremarkable.\n\nOf the following, the BEST next step in this boy's care is", "options" : "[\"counseling regarding voice hygiene\", \"flexible fiberoptic laryngoscopy\", \"magnetic resonance imaging of his neck\", \"referral for speech therapy\"]", "explanation" : "The boy in the vignette has persistent hoarseness with symptoms of worsening airway obstruction (exercise intolerance, abnormal inspiratory-to-expiratory ratio) in the context of a facial hemangioma that involves the mucosal surface. Although hemangiomas usually resolve in infancy or early childhood, they may persist in older children. A hemangioma that involves the mucosal surface of the oropharynx should raise concern regarding airway hemangioma. The best next step in this boy's care is visualization of the larynx with flexible fiberoptic laryngoscopy.\n\nPer the American Academy of Otolaryngology (AAO) clinical practice guidelines for hoarseness, visualization of the larynx should be performed before imaging or referral for speech therapy. In most cases of chronic hoarseness in children and adolescents, there is not an urgent need for airway visualization or imaging. However, in this case, there is a moderately urgent need for airway visualization because the boy has signs of an impaired airway.\n\nEtiologies for hoarseness vary by age. The Table outlines some of the many causes of hoarseness by category and by age. Beyond infancy, viral infection is the most common cause of hoarseness (generally benign and self-limited). Voice abuse is a common cause of laryngeal nodules, which cause hoarseness at all ages beyond infancy.\n\nMost causes of pediatric hoarseness are benign and require little or no intervention. Chronic hoarseness is defined as that which fails to improve after 3 to 4 weeks of voice rest and intervention appropriate to the suspected cause. Counseling regarding voice rest and hygiene is the best intervention for most children and adolescents, before hoarseness becomes chronic or at the first evaluation of a stable child with hoarseness that has lasted at least 3 to 4 weeks. When hoarseness persists beyond 3 or 4 weeks, evaluation for associated conditions (eg, allergies, laryngopharyngeal reflux) suggested by a careful history and physical examination may direct intervention for the vocal symptoms. The treatment for vocal cord nodules is speech therapy; however, the AAO recommends vocal cord visualization before referral to speech therapy, to provide information that will help guide the intervention.\n\nWhen stridor or other signs of airway compromise are present, the investigation should be expedited. Additionally, per the AAO guidelines, an expedited evaluation for hoarseness should occur in children who have a neck mass or who have had recent surgery (of any type) or recent airway intubation. Although laryngeal cancer related to smoking is not found in adolescents, smoking is a cause for hoarseness and is an indication for expedited evaluation. An affected adolescent whose voice is critical to a professional career should also undergo prompt evaluation.\n\nInfants with a hoarse cry and no history of surgery or airway trauma should undergo expedited evaluation for congenital anomalies of the airway and neurologic causes. Many of the etiologies for hoarseness in this age group are associated with poor feeding and aspiration. Early collaboration with specialists regarding upper- and lower-airway, esophageal, and neurologic concerns is appropriate.\n\nHoarseness in a child with a history of intubation or surgery involving the neck or chest should prompt direct visualization of the airway under controlled conditions to evaluate for laryngeal trauma, stenosis, vocal cord paralysis or dysfunction, or recurrent laryngeal nerve damage. Magnetic resonance imaging and/or other central nervous system diagnostic procedures should be pursued if there is concern about a neurological source of hoarseness or abnormal vocal cord function. In the context of cardiovascular abnormalities or dysmorphic features, referral to a geneticist is appropriate. Specific management is dictated by the diagnosis.\n\nSuggested Reading(s)\nAdam H. Hoarseness. Point-of-Care Quick Reference. Pediatric care Online. American Academy of Pediatrics. Accessed December 6, 2023. Pediatric Care Online\nParikh SR. Hoarseness. In: McInerny TK, Adam HM, Campbell DE, Foy JM, Kamat DM, eds. American Academy of Pediatrics Textbook of Pediatric Care. 2nd ed. American Academy of Pediatrics; 2016:chap 165. Accessed September 1, 2023. Pediatric Care Online\nStachler RJ, Francis DO, Schwartz SR, et al. Clinical practice guideline: hoarseness (dysphonia (update) executive summary. Otolaryngol Head Neck Surg. 2018;158(3):409-426. doi:10.1177/0194599817751031\nWorthen M, Chandran S. Hoarseness in children. Int J Head Neck Surg. 2016;7(2):130-135. doi:10.5005/jp-journals-10001-1278\nYang J, Xu W. Characteristics of functional dysphonia in children. J Voice. 2018;156.e-1-156.e-4. doi:10.1016/j.jvoice.2018.07.027\n\nContent Domain\nPulmonology\n\nABP Content Specification(s) / Content Area(s)\nRecognize the various causes of hoarseness\nFormulate a differential diagnosis of hoarseness\nPlan the appropriate evaluation of hoarseness\n\nThe correct answer is: flexible fiberoptic laryngoscopy"}
{"id" : 1921, "question_text" : "A 10-year-old boy has a 2-day history of throat pain and fever to 38.2°C. He has erythematous tonsils with petechiae on his soft palate. A rapid streptococcal antigen test result is positive. His mother states that he and his 3 siblings have had multiple throat infections over the past year, and these frequent infections are impairing her ability to keep her job. She requests a tonsillectomy to \"get this taken care of once and for all.\" Over the past 3 years, the patient has had 7 visits for pharyngitis and 5 other positive rapid streptococcal antigen test results, including 2 performed during his siblings' visits while the patient was asymptomatic. He has an allergy to amoxicillin but no reaction to cephalosporins. Regarding sleep, his mother states that he has snoring only with upper respiratory infections, and there are no concerns about his behavior or school performance. Of the following, the BEST next step in the care of this patient is", "options" : "[\"long-term prophylactic cephalosporin therapy\", \"polysomnography\", \"a referral to an otolaryngologist\", \"watchful waiting\"]", "explanation" : "Correct Answer: D\nThe child in this vignette will likely benefit most from watchful waiting, rather than referral for tonsillectomy. He does not meet the criteria for tonsillectomy based on infection frequency (Item C121A). For the child in the vignette, there is insufficient evidence to support the use of prophylactic cephalosporin. Children thought to have frequent recurrent episodes of group A Streptococcus (GAS) pharyngitis are often chronic carriers of GAS who are experiencing repeated viral infections.\n\nTonsillectomy and adenotonsillectomy are among the most common surgical procedures in children and adolescents. The most common indication for these procedures has shifted from recurrent GAS pharyngitis to obstructive sleep apnea syndrome (OSAS). The prevalence of OSAS ranges from 1.2% to 5.7%. The American Academy of Pediatrics recommends that primary care clinicians universally screen for OSAS at health supervision visits by asking caregivers if their child snores, because snoring is a nearly universally present symptom in OSAS. If affirmative, the primary care clinician should further explore the likelihood of OSAS based on signs and symptoms that are more specific to the disorder (Item C121B). Not all snoring indicates OSAS, and occasional snoring with upper respiratory infections, as in the child in this vignette, is less concerning for the syndrome.\n\nWhile eliciting associated signs and symptoms is helpful in separating snoring children at low risk of having OSAS, polysomnography remains the only reliable diagnostic tool for this condition. It is recommended that children suspected of having OSAS undergo this test prior to adenotonsillectomy. Polysomnography requires overnight testing in a specialized laboratory and is expensive. It is often inconvenient for families, and long wait times are common. If polysomnography is not available, referral to an otolaryngologist or sleep specialist for further evaluation is recommended. Alternative but less accurate strategies, such as overnight oximetry, sleep videos, daytime nap polysomnography, and clinical questionnaires, are sometimes used in lieu of polysomnography.\n\nAdenotonsillectomy is the first-line treatment for nearly all children with OSAS. Children who are obese demonstrate less improvement in symptoms, but adenotonsillectomy is still generally preferred over alternatives, including continuous positive airway pressure. Adenotonsillectomy has a complication rate of approximately 20%, with respiratory compromise and postoperative hemorrhage being the most common complications. Velopharyngeal insufficiency occurs in about 1 in 1,200 cases, and death occurs in about 1 or 2 in 30,000 cases. Children with risk factors for complications, such as craniofacial abnormalities, severe obesity, and age younger than 3 years, should be observed overnight on an inpatient unit after surgery.\n\nAdenotonsillectomy alone is usually effective for OSAS, but children should be evaluated 6 to 8 weeks after surgery to ensure resolution of symptoms. For children at high risk for persistent OSAS, including children with markedly abnormal preoperative polysomnography, obesity, or continued symptoms after surgery, a repeat polysomnogram and/or referral to a sleep specialist is recommended.\n\nTonsillectomy for recurrent severe throat infections is controversial. The 2011 American Academy of Otolaryngology-Head and Neck Surgery guideline for tonsillectomy in children recommends tonsillectomy for patients meeting the Paradise criteria (Item C121A); the child in this vignette does not meet these criteria. While tonsillectomy is very effective in reducing the frequency of episodes of severe pharyngitis, most children meeting the Paradise criteria but who did not undergo tonsillectomy in controlled trials experienced a natural reduction in the frequency and severity of throat infections. The 2012 Infectious Disease Society of America guideline for GAS infections does not recommend tonsillectomy solely to reduce the frequency of such infections, given the natural decrease in infection frequency and the difficulty identifying GAS carriers (in whom antibiotics are generally not warranted).\n\nLess common indications for tonsillectomy include recurrent peritonsillar abscess, recurrent throat infections in the setting of multiple antibiotic allergies, concern for tonsillar malignancy, and halitosis. Less common indications for adenoidectomy alone include recurrent sinusitis and otitis media with effusion where repeat tympanostomy tube insertion is required.\n\nPREP Pearls\n\nAdenotonsillectomy is the first-line therapy for obstructive sleep apnea syndrome.\n\nTonsillectomy for recurrent, severe throat infections should be limited to patients meeting strict criteria; watchful waiting is appropriate in most cases.\n\nAdenotonsillectomy has a complication rate of about 20%; however, most complications are short term and the procedure is overall well tolerated.\n\nABP Content Specifications(s)/Content Area\n\nRecognize complications associated with tonsillectomy and/or adenoidectomy, including those associated with velopharyngeal insufficiency\n\nUnderstand the indications for an adenoidectomy and the resulting effects on nasal function, sleep physiology, and eustachian tube function\n\nUnderstand the indications for a tonsillectomy\n\nSuggested Readings\n\nBaugh RF, Archer SM, Mitchell RB, et al; American Academy of Otolaryngology-Head and Neck Surgery Foundation. Clinical practice guideline: tonsillectomy in children. Otolaryngol Head Neck Surg. 2011;144(1 suppl):S1-S30. doi:10.1177/0194599810389949.\n\nMarcus CL, Brooks LJ, Draper KA, et al; American Academy of Pediatrics. Diagnosis and management of childhood obstructive sleep apnea syndrome. Pediatrics. 2012;130(3):576-584. doi:10.1542/peds.2012-1671..\n\nShulman ST, Bisno AL, Clegg HW, et al. Clinical practice guideline for the diagnosis and management of group A streptococcal pharyngitis: 2012 update by the Infectious Diseases Society of America. Clin Infect Dis. 2012;55(10):1279-1282. doi:10.1093/cid/cis847."}
{"id" : 134, "question_text" : "A 3-year-old girl presents to the emergency department with irritability and weakness that was followed by the development of nausea and vomiting and finally a seizure. Her mother reports that earlier in the day she found the girl playing in the medicine cabinet but did not see her take any pills. Physical examination reveals a toxic-appearing, febrile child who has hypertension, tachycardia, dilated pupils, hyperreflexia, reduced muscle strength, and abdominal tenderness. You order a toxicology panel.\n\nOf the following, the MOST likely cause of the child's clinical findings is an overdose of", "options" : "[\"acetaminophen\", \"amphetamines\", \"barbiturates\", \"digoxin\", \"narcotics\"]", "explanation" : "The girl described in the vignette is hypertensive and exhibits other typical signs and symptoms of an amphetamine overdose. Amphetamines are powerful central nervous system stimulants that also have peripheral adrenergic actions. The acute toxic effects due to overdose include hyperactive reflexes, dilated pupils, talkativeness, irritability, weakness, and fever. In addition, the patient may experience palpitations, tachycardia, hypertension, nausea, vomiting, diarrhea, and abdominal cramps. Severe overdose is associated with seizure, coma, and stroke. A wide variety of prescription drugs (eg, corticosteroids, combined oral contraceptives), over-the-counter drugs (eg, cough and cold medicines, nonsteroidal anti-inflammatory drugs) and supplements (eg, caffeine-containing products, ginseng) can elevate blood pressure.\n\nHepatotoxicity is the primary and most dangerous adverse effect of acetaminophen overdose. Barbiturates are central nervous system sedative-hypnotic agents that primarily depress the level of consciousness. They may cause normal or small pupils and uncommonly result in hypotension. Digoxin has a narrow therapeutic index, and overdose is not uncommon at the higher end of the prescribed dose range. Toxic effects may include palpitations due to atrial and ventricular arrhythmias, nausea and vomiting, and visual disturbances that include blurred vision and xanthopsia (a disturbance of color vision involving yellow and green). Electrocardiographic findings may include bradycardia, atrioventricular block, and a highly reproducible change that results in slurring of the upstroke of the PR interval. Narcotic ingestion causes sedation, analgesia, respiratory depression, pinpoint pupils, hypotension, nausea, and vomiting.\n\nAmerican Board of Pediatrics Content Specification(s):\n\nRecognize prescription, over-the-counter, and illicit drugs likely to elevate the blood pressure"}
{"id" : 3011, "question_text" : "A newborn is seen in a clinic for his first visit after discharge from the neonatal intensive care unit. He was born vaginally at term to first-time parents after an uncomplicated pregnancy. At delivery, he was noted to have clusters of blisters on his right hand, his posterior neck, and his left upper arm. An infectious evaluation had negative results. Skin biopsy with immunofluorescence microscopy mapping showed staining of basement membrane antigens in the floor of the blister, consistent with intraepidermal cleavage seen in epidermolysis bullosa. During his hospitalization, the neonate developed several oral blisters. His parents have been educated on skin and oral care for his condition. They have questions about his long-term prognosis and the likelihood that he will develop other medical problems. Of the following, the MOST common extracutaneous manifestation of this boy's condition is", "options" : "[\"blindness\", \"hepatic fibrosis\", \"malnutrition\", \"nephritis\"]", "explanation" : "The newborn in the vignette has epidermolysis bullosa (EB). This is suggested by his physical examination findings and confirmed on skin biopsy. Epidermolysis bullosa is a heterogeneous group of rare disorders (approximately 20 per million live births), all characterized by mechanical fragility of the epithelial tissue. The severity, age at onset of cutaneous manifestations, and prevalence of extracutaneous symptoms varies between subtypes.\n\nThe most common extracutaneous manifestations are nail, dental, mucous membrane, and eye abnormalities. Malnutrition is a common complication, occurring because of feeding difficulties; malabsorption; increased caloric requirements because of chronic inflammation, accelerated skin cell turnover, and continuous wound healing; and excess losses across the abnormal cutaneous barrier. Other complications include malnutrition-related anemia, hypoalbuminemia, and growth failure; infection; and an increased risk of skin cancer. Although patients with EB can have ocular abnormalities (eg, conjunctival irritation, corneal ulcerations, lacrimal duct obstruction) blindness is rare. Hepatic fibrosis and nephritis are not common manifestations of EB.\n\nPREP Pearls\n• Epidermolysis bullosa is a heterogenous group of disorders characterized by mechanical fragility of the epidermal tissues.\n• Common extracutaneous manifestations of epidermolysis bullosa include nail, dental, mucous membrane, and eye abnormalities. Malnutrition and infection are common complications of epidermolysis bullosa, and patients have an increased risk of skin cancer.\n\nABP Content Specifications(s)\n• Recognize the clinical findings associated with epidermolysis bullosa\n\nSuggested Readings\n• Fine JD, Bruckner-Tuderman L, Eady RA, et al. Inherited epidermolysis bullosa; updated recommendations on diagnosis and classification. J Am Acad Dermatol. 2014:70(6):1103-1126. doi: 10.1016/j.jaad.2014.01.903.\n• Fine JD. Inherited epidermolysis bullosa. Orphanet J Rare Dis. 2010;5:12. doi: 10.1186/1750-1172-5-12."}
{"id" : 2849, "question_text" : "A 2-month-old boy is seen in the office for a sick visit. His mother is concerned that he is breathing fast and eating less. Over the last few days, while breastfeeding, he has tired out sooner, breathed a little faster, and become sweaty. He now seems to be breathing faster when he is at rest and not eating. He does not have rhinorrhea, cough, congestion, fevers, vomiting, diarrhea, or rash. He is afebrile. He has a heart rate of 158 beats/min, a blood pressure of 75/45 mm Hg, and a respiratory rate of 60 breaths/min. He appears comfortable. His oxygen saturation is 95%. His weight remains at the 50th percentile for age. He has a regular heart rate and rhythm with a normal S1 and physiologically split S2. He has a grade 2/6 holosystolic murmur at the left lower sternal border. He has good perfusion and normal pulses. His liver edge is palpated 3 cm below the right costal margin. Of the following, the BEST next step(s) in treatment is (are) to", "options" : "[\"admit the infant to the hospital for a septic evaluation\", \"increase the infant's caloric density of nutrition and refer him to a pediatric cardiologist\", \"prescribe furosemide and refer the infant to a pediatric cardiologist\", \"provide reassurance to the mother and follow up in 2 weeks for a weight check\"]", "explanation" : "Correct Answer: C\nThe baby in the vignette has a clinical history and physical examination findings consistent with a hemodynamically significant ventricular septal defect. At 2 months of age, he is demonstrating that his pulmonary vascular resistance has dropped, making it far easier for blood to be shunted through the ventricular septal defect and pulmonary outflow tract instead of to the systemic circulation. Because of this, he has congested lungs with pulmonary edema and is breathing faster. The next course of action would be treatment with furosemide to help relieve some of the congestion and to refer him to a pediatric cardiologist for further care. He does not have signs or symptoms of sepsis, and he has maintained his growth trajectory and does not need increased calories at this time. Following up in 2 weeks after providing reassurance to the mother will only delay care.\n\nAcyanotic congenital heart disease can be categorized into two broad topics, left-to-right shunts and obstruction to blood flow. Left-to-right shunts include lesions such as atrial septal defects, ventricular septal defects, atrioventricular septal defects, and patent ductus arteriosus. Pulmonary and aortic valve stenosis as well as coarctation of the aorta can be categorized as obstruction to blood flow.\n\nThe cardiac lesions that result in left-to-right shunting do so through a defect that allows for oxygenated blood from the lungs (present on the left side of the heart) to mix with deoxygenated blood returning from the body to the right side of the heart. Oxygenated blood then returns to the lungs instead of going out to the body. This ineffective blood flow results in pulmonary congestion and edema, which in turn lead to tachypnea and increased work of breathing. The degree of increased pulmonary blood flow is determined by the relative resistances between the pulmonary and systemic vascular beds as well as the effective size of the ventricular septal defect (ie, whether it is large or small). An atrial level shunt will result in right ventricular enlargement, whereas a ventricular septal defect, atrioventricular septal defect, aortopulmonary window, and patent ductus arteriosus result in left heart enlargement. Findings that can aid in diagnosis include specific murmurs and hepatomegaly found on physical examination, cardiac enlargement seen on chest radiograph, specific electrocardiographic findings dependent on the lesion, and the appearance of the defect itself seen on echocardiogram. When hemodynamically significant, these lesions can be repaired through cardiac catheterization or cardiac surgery.\n\nCardiac lesions that result in outflow obstruction include aortic and pulmonary valve stenosis, as well as coarctation of the aorta. These lesions vary in severity from mild to severe. Those labeled \"critical\" require a patent ductus arteriosus to maintain adequate pulmonary or systemic cardiac output. If the ductus is inadequate, prostaglandin infusion will be required (pulmonary valve stenosis, when critical, will actually be a cyanotic lesion). Both pulmonary and aortic valve stenosis can present as an asymptomatic lesion with a murmur, ventricular hypertrophy, or both on an electrocardiogram (or echocardiogram); diagnosis is made via echocardiography. These lesions can be accompanied by fatigue and exercise intolerance. Significant aortic stenosis can also present with chest pain, signs of ischemia, or both. Both types of valve stenosis can be treated with balloon dilation in the cardiac catheterization laboratory or cardiac surgical procedure. Coarctation of the aorta can present as a ductal-dependent (ie, critical coarctation) lesion in the neonatal period with murmur, decreased femoral pulses, and tachypnea or cardiogenic shock as the ductus begins to close or closes completely. In this instance, prostaglandin infusion will need to be initiated. In some children, however, coarctation of the aorta will present later in life with murmur or hypertension. Coarctation of the aorta can be treated with cardiac catheterization or cardiac surgery.\n\nPREP Pearls\n• Cardiac lesions with left-to-right shunting can include atrial septal defects, ventricular septal defects, atrioventricular septal defects, aortopulmonary window, and patent ductus arteriosus. Hemodynamically significant lesions result in cardiac enlargement and pulmonary congestion and warrant repair.Pulmonary or aortic valvar stenosis results in varying degrees of obstruction to blood flow and ventricular hypertrophy; lesions can be treated by balloon dilation in the cardiac catheterization laboratory.\n• Pulmonary or aortic valvar stenosis results in varying degrees of obstruction to blood flow and ventricular hypertrophy; lesions can be treated by balloon dilation in the cardiac catheterization laboratory.\n• Coarctation of the aorta results in varying degrees of obstruction to blood flow and can cause cardiogenic shock in the neonate. Coarctation can be treated with cardiac surgery or in the cardiac catheterization laboratory.\n\nMOCA-Peds Objective\n• Recognize genetic causes of congenital heart disease.\n\nABP Content Specifications(s)\n• Recognize the major clinical findings associated with the various types of acyanotic congenital heart disease\n\nSuggested Readings\n• McCulloch MA, Gajarski RJ. Congenital and acquired heart disease. In: McInerny TK, Adam HM, Campbell DE, DeWitt TG, Foy JM, Kamat DM, eds. American Academy of Pediatrics Textbook of Pediatric Care. 2nd ed. Elk Grove Village, IL: American Academy of Pediatrics; 2017:1883-1916. Pediatric Care Online .\n• Price JF. Congestive heart failure in children. Pediatr Rev. 2019;40(2):60-70. doi:10.1542/pir.2016-0168.\n• Puri K, Allen HD, Qureshi AM. Congenital heart disease. Pediatr Rev. 2017;38(10):471-486. doi:10.1542/pir.2017-0032."}
{"id" : 1367, "question_text" : "You are meeting with the parents of a 15-year-old adolescent boy and 8-year-old boy for a parents-only visit. They inform you that they are getting a divorce. They are planning on selling their current home and will both find new homes within a reasonable travel distance. The children will continue to attend the same schools. They have been careful to refrain from arguing in front of the children, but state that they are having a difficult time agreeing on custody arrangements. Of the following, the BEST recommendation is to have", "options" : "[\"the children decide with whom they wish to live\", \"custody decisions determined by the courts\", \"joint custody with equal division between parents\", \"a regular schedule with flexibility for change\", \"weekdays at one home and weekends at the other home\"]", "explanation" : "Children of divorced families do best with a regular schedule with flexibility for change. These children are already going through many changes and losses; as much stability in routines and schedules as is possible is best for the children. However, there must also be flexibility to change these schedules as needed to accommodate for the changing needs of the children (eg, extracurricular activities, special events).\n\nApproximately half of first marriages end in divorce. While 85% of divorced adults remarry, 40% of these new marriages also end in divorce. Divorce affects over 1 million children per year, causing multiple changes in these children's lives. Not only does the family structure change, but the child's living circumstances also change. The child's home environment, school placement, childcare arrangements, extracurricular activities, community, routines, peer groups, and available supports may all be affected. The child's lifestyle may change due to decreased income and resources.\n\nRegardless of the developmental stage of the child, consistency in routine is key in helping the child adjust to the changes around divorce. Infants may cry more and have problems with sleep. Young children can exhibit separation anxiety, fearfulness, and aggression. Preschoolers, who are engaged in magical thinking, may believe that they caused the breakup and may attempt to control their surroundings. They may have nightmares, become clingier, or act out. Young children require consistent routines and caregivers to develop secure attachment and to address and prevent fear of abandonment. School-age children and adolescents may have academic underachievement, may become moody, and test boundaries. Adolescents may also exhibit externalizing and internalizing problems such as substance abuse, delinquency, depression, and anxiety. Consistent behavioral expectations and routines with limit setting are helpful for older children. No matter the age, these children are dealing with changes that are outside of their control, including the loss of their family unit and may respond with anger and grief. They may withdraw socially or exhibit somatic symptoms. Consistency with structure and routine provides stability and helps with both short- and long-term adjustment and leads to better outcomes. Inconsistent schedules are associated with poorer social and mental health outcomes.\n\nCustodial issues are important in how children adapt to divorce. Previously, mothers were the primary custodians with periodic visits from the fathers. However, children do better when both biological parents are available to provide care to their children and when they can continue their relationships with each parent. It is important for the child's father to have a consistent presence. Joint physical custody is common with half-time or one-thirds to two-thirds time as typical examples of how time is divided between parents. The specifics vary according to logistical considerations such as age of child, distance between homes, location of work and school, and living arrangements. Joint custody arrangements are complex and work best when both parents are able to communicate effectively with each other, work cooperatively, assist with transitions between households, and are respectful of each other and the child's wishes. When parents cannot agree on a parenting plan, mediation is preferred over using the courts as this avoids costs and conflict. The courts are generally involved in more acrimonious situations. It is also important to keep in mind that custody arrangements may need adjustment as the children mature.\n\nWhatever the custody arrangement, parental conflict is a major determinant of how children do with the divorce of their parents. Cooperative co-parenting is most helpful in children's adjustment to the divorce. Children of married or divorced parents do better if their parents do not fight in front of them. If exposed to conflict, the children are more likely to have externalizing behavioral problems and academic underachievement.\n\nIn the vignette, the children's wishes should be respected and considered, but should not be the ultimate determinant of custody arrangements, as they may not be aware of additional considerations or logistics. When parents are respectful of each other as parents and when they have their children's interests at heart, custody decisions are best made without going through the courts to avoid cost and conflict. While joint custody with equal division between parents appears fair, this may not be logistically reasonable and may be disruptive to a child who thrives on routine and who has difficulties with transitions. While weekdays at one home and weekends at the other home may assist with some of the difficulties in transitions, this may not be the ideal circumstance for all families. A regular schedule with flexibility for change is the best recommendation for families.\n\nPediatricians can assist parents in planning for their children's needs through the events before and after the divorce. They can encourage families to maintain routines, activities, contacts, discipline, and responsibilities in as normal a fashion as possible. They can guide parents in keeping the children's best interests in mind as the family adjusts to the changes in their lives.\n\nPREP Pearls\n• Children of divorced families do best with a regular schedule with flexibility for change.\n• No matter the developmental stage of the child, consistency with structure and routine provides stability, helps with both short- and long-term adjustment, and leads to better outcomes.\n• Whatever the custody arrangement, parental conflict is a major determinant of how children do with the divorce of their parents. Cooperative co-parenting is most helpful in children's adjustment to the divorce.\n\nABP Content Specifications(s)\n• Understand the custodial issues associated with divorce and the effect of those issues on patients of various ages\n\nSuggested Readings\n• Cohen GJ, American Academy of Pediatrics, Committee on Psychosocial Aspects of Child and Family Health. Helping children and families deal with divorce and separation. Pediatrics. 2002;110(5):1019-1023. http://pediatrics.aappublications.org/content/110/5/1019.\n• Kaplan-Sanoff M. Divorce. In: Augustyn M, Zuckerman B, Caronna EB, eds. The Zuckerman Parker Handbook of Developmental and Behavioral Pediatrics for Primary Care. 3rd ed. Philadelphia, PA: Lippincott Williams and Wilkins; 2011:446-449.\n• Kleinsorge C, Covitz LM. Impact of divorce on children: developmental considerations. Pediatr Rev. 2012;33(4):147-155. doi: http://dx.doi.org/10.1542/pir.33-4-147."}
{"id" : 1840, "question_text" : "An 8-year-old boy with a known history of myopia was recently diagnosed with upward dislocation of the lens. He has a tall stature, long arms, pectus excavatum, arachnodactyly, flat feet, and reduced elbow extension. His face is long and narrow with deep-set eyes and a receding chin. His mother has a similar appearance and body habitus, as well as a history of mitral valve prolapse and myopia. The maternal grandfather died in his 50s of a sudden cardiac death. Of the following, the boy's MOST likely diagnosis is", "options" : "[\"Ehlers Danlos syndrome\", \"Loeys Dietz syndrome\", \"Marfan syndrome\", \"Sotos syndrome\"]", "explanation" : "Correct Answer: C\nThe boy in the vignette has Marfan syndrome, which is an autosomal dominant connective tissue disorder with a great degree of variability in clinical presentation even among members of the same family. Classic manifestations include ocular, cardiovascular, and skeletal involvement. Cardiac involvement includes dilation of the aorta, mitral valve prolapse, tricuspid valve prolapse, and proximal pulmonary artery enlargement. Children with this syndrome are at risk for aortic rupture; therefore, yearly echocardiographic monitoring for increased aortic root diameter is recommended, with intermittent surveillance of the entire aorta with computed tomography or magnetic resonance angiography beginning in young adulthood. The most common ocular manifestation is myopia; however, upward dislocation of the lens (ectopia lentis), retinal detachment, glaucoma, and early cataract formation are also frequently noted. Annual ophthalmologic slit-lamp examination is recommended. Skeletal findings include tall stature, scoliosis, pectus excavatum, pectus carinatum, arachnodactyly, and disproportionately long extremities. Facial features consist of a long and narrow face with deep-set eyes, downslanting palpebral fissures, malar hypoplasia, and micrognathia/retrognathia.\n\nMarfan syndrome, caused by mutations in the FBN1 gene, is typically diagnosed based on family history and characteristic systemic findings. Ectopia lentis and aortic aneurysm have high specificity and clinical significance for a diagnostic consideration of Marfan syndrome. Seventy-five percent of individuals have an affected parent, while 25% of probands have a sporadic de novo pathogenic mutation.\n\nEhlers Danlos syndrome is a group of disorders with a common feature of joint hypermobility. There are multiple subtypes, including classic, kyphoscoliotic, and vascular. Clinical findings include skin hyperextensibility, abnormal wound healing, easy bruising, and atrophic scarring.\n\nLoeys Dietz syndrome is an autosomal dominant disorder that shares many features with Marfan syndrome including the pectus deformity, scoliosis, and aortic root aneurysm; however, several findings are not seen in Marfan syndrome, including hypertelorism, bifid uvula, craniosynostosis, cleft palate, and generalized arterial tortuosity along with dissections throughout the entire arterial tree.\n\nSotos syndrome is an autosomal dominant overgrowth syndrome characterized by a distinctive facial appearance with a broad forehead, sparse frontotemporal hair, downslanting palpebral fissures, long and narrow face, and malar flushing. Height and/or head circumference are greater than 2 standard deviations above the mean. Learning disability is common, varying from early developmental delay to mild, moderate, or severe intellectual deficit.\n\nPREP Pearls\n\nMarfan syndrome, caused by mutations in the FBN1 gene, is clinically diagnosed based on family history and characteristic systemic findings.\n\nMarfan syndrome is an autosomal dominant connective tissue disorder with variable expression among members of the same family. Classic manifestations include ocular, cardiovascular, and skeletal involvement.\n\nIn patients with Marfan syndrome, surveillance consists of an annual comprehensive physical and an ophthalmologic (including slit-lamp) examination, and echocardiography\n\nABP Content Specifications(s)/Content Area\n\nRecognize the clinical and laboratory findings associated with Marfan syndrome\n\nSuggested Readings\n\nDietz H. Marfan syndrome. GeneReviews. https://www.ncbi.nlm.nih.gov/books/NBK1335/.\n\nGoyal A, Keramati AR, Czarny MJ, Resar JR, Mani A. The genetics of aortopathies in clinical cardiology. Clin Med Insights Cardiol. 2017;11:1179546817709787. doi: 10.1177/1179546817709787.\n\nTinkle BT, Saal HM, the Committee on Genetics. Health supervision for children with Marfan syndrome. Pediatrics. 2013;132(4):1059-1072. doi: 10.1542/peds.2013-2063."}
{"id" : 2676, "question_text" : "A 2-hour-old neonate born at 36 4/7 weeks' gestation is examined in the newborn nursery. She was born to a 28year-old gravida 2, para 1 woman in a normal spontaneous vaginal delivery. Maternal prenatal testing is negative for group B Streptococcus, and rupture of membranes occurred 20 hours before delivery. The mother had a temperature of 39.6°C measured 8 hours before delivery and fetal tachycardia was noted at that time. The neonate has a temperature of 37.1°C, heart rate of 130 beats/min, and respiratory rate of 48 breaths/min. She appears vigorous with a good cry and normal tone. The remainder of her physical examination findings are normal. The mother requests early discharge. Of the following, the BEST next management step for this neonate is to", "options" : "[\"consider early discharge if the neonate's vital signs are stable with next day follow-up\", \"observe closely for signs of sepsis for at least 48 hours\", \"obtain a complete blood cell count and C-reactive protein level, and perform blood culture\", \"obtain a complete blood cell count, perform blood culture, and start empiric antibiotics\"]", "explanation" : "Correct Answer: D\nThe neonate in the vignette is at risk for sepsis due to a combination of factors, late preterm gestation, maternal fever, and prolonged rupture of membranes (PROM, >18 hours). The best next step in management is to obtain a complete blood cell count, perform a blood culture, and start intravenous ampicillin and gentamicin treatment. Early discharge is not the best option because signs of sepsis may be delayed until 72 hours after birth. Close observation for 48 hours without empiric antibiotics would not be a good choice given this neonate's multiple risk factors for sepsis. Although normal laboratory evaluation may seem reassuring, the white blood cell counts and inflammatory markers may not be a reliable indicator of sepsis in the first 24 to 48 hours after birth.\n\nAlthough the incidence of early-onset sepsis (EOS) has decreased over the past several years with the use of intrapartum antibiotics, the morbidity and mortality of affected neonates remains high. Clinicians must weigh the risk of delayed treatment of neonatal sepsis with that of unnecessary antibiotic use.\n\nRisk factors for EOS include prematurity, PROM, maternal colonization with group B Streptococcus (GBS), and evidence of maternal chorioamnionitis. A suspected intra-amniotic infection is defined by the American College of Obstetricians and Gynecologists as maternal fever (>39°C) and at least 1 of the following: maternal leukocytosis, purulent cervical discharge, and fetal tachycardia. If the mother meets the criteria for suspected intra-amniotic infection, laboratory evaluation and empiric treatment with antibiotics are recommended for neonates with any of the following:\n• Signs of sepsis (eg, ill appearing)\n• Gestational age <37 weeks\n• Maternal PROM\n• Inadequately treated maternal GBS colonization\n• Evidence of maternal chorioamnionitis\n\nRisk stratification algorithms are available to clinicians to assist with the at-risk neonate. The EOS calculator takes into account maternal factors as well as the clinical appearance of the neonate to guide the decision-making process. Several studies have associated use of the EOS calculator with reduction in antibiotic use, laboratory testing, and neonatal intensive care unit admissions, without any missed sepsis cases. The EOS calculator can be found at https://neonatalsepsiscalculator.kaiserpermanente.org/. For the neonate in the vignette, the EOS calculator recommends performing a blood culture and starting empiric antibiotics.\n\nPREP Pearls\n• Risk factors for early-onset neonatal sepsis include prematurity, evidence of maternal chorioamnionitis, untreated maternal colonization with group B Streptococcus, and prolonged rupture of membranes.\n• Risk stratification algorithms may guide clinicians' management of neonates at risk for sepsis and reduce the use of unnecessary antibiotics.\n• The use of intrapartum antibiotics has reduced the incidence of early-onset neonatal sepsis.\n\nABP Content Specifications(s)\n• Plan the management of a neonate whose mother is febrile at the time of delivery\n\nSuggested Readings\n• Jan AI, Ramanathan R, Cayabyab RG. Chorioamnionitis and management of asymptomatic infants =35 weeks without empiric antibiotics [erratum in: Pediatrics. 2017;140(4)]. Pediatrics. 2017;140(1):e20162744. doi:10.1542/peds.2016-\n• Klitzman MD, Jafri FW, Scott EK, Thomas AE, Engle WA. Implementation of early onset sepsis calculator safely decreases antibiotic use and laboratory testing in newborns born to mothers with chorioamnionitis. Pediatrics. 2020;146(1 MeetingAbstract):243-244. doi:10.1542/peds.146.1_MeetingAbstract.243-a.\n• Kojaoghlanian T. The newborn at risk of infection. In: McInerny TK, Adam HM, Campbell DE, DeWitt TG, Foy JM, Kamat DM, eds. American Academy of Pediatrics Textbook of Pediatric Care. American Academy of Pediatrics; 2021:chap 102. Accessed September 1, 2022. Pediatric Care Online.\n• Puopolo KM, Benitz WE, Zaoutis TE, AAP Committee on Fetus and Newborn, AAP Committee on Infectious Diseases. Management of neonates born at =35 0/7 weeks' gestation with suspected or proven early-onset bacterial sepsis. Pediatrics. 2018;142(6):e20182894. doi:10.1542/peds.2018-2894."}
{"id" : 153, "question_text" : "A 13-year-old boy presents for a routine health supervision visit. He is asymptomatic today, has no findings of significance on past medical history, and currently is not taking any medications. He appears well and has normal vital signs and a body mass index of 20.6. He is at Sexual Maturity Rating 2. You find a slightly tender, rubbery mass under his right areola that is approximately 2 cm in diameter. The remainder of his examination results, including a testicular evaluation, are normal. He tells you that one of his grandmothers has breast cancer. He was embarrassed to tell you about the lump, and he is concerned that he might have cancer. Of the following, the MOST appropriate statement to make to the boy is that", "options" : "[\"his risk of breast cancer is high because of his family history\", \"the breast mass should regress spontaneously\", \"weight loss is necessary to resolve the mass\", \"you will refer him to a plastic surgeon\", \"you will refer him to an endocrinologist\"]", "explanation" : "The presence of palpable fibroglandular breast tissue under the nipple-areolar complex in a male that measures at least 0.5 cm in diameter, as described for the boy in the vignette, is referred to as gynecomastia. In the neonatal period and early adolescence, this is most often a physiologic phenomenon that regresses spontaneously. Breast tissue has both estrogen (stimulatory) and androgen (inhibitory) receptors. In males, during early puberty, the ratio of estrogen to testosterone is increased, and up to 70% of males in Sexual Maturity Rating (SMR) 2 stage of pubertal development (testicular volume of 5 to 10 mL) have some breast enlargement on examination that may be tender as a result of edema and inflammation. Initially unilateral, the process becomes bilateral in up to 75% of cases. In most males, such breast tissue no longer is palpable in 18 months (1 to 3 years), as puberty progresses and androgen concentrations increase.\n\nIn false or pseudogynecomastia, as seen in obese males, adipose tissue does not feel like a distinct, firm, rubbery mass. The doughnut sign is occasionally elicited when lying supine, in which the areola forms a depression within the surrounding adipose tissue.\n\nPathologic conditions involving the male breast can be local masses or result from hormonal aberrations of estrogen excess or androgen insufficiency. Rapid enlargement or a size greater than 4 cm is usually pathologic. Local masses, such as hemangiomas, lymphangiomas, lipomas, and neurofibromas, are usually unilateral, not circular, and not directly beneath the areola. Breast cancer in a male adolescent is extremely rare. Such a mass is hard and fixed rather than rubbery and movable over the chest wall. Estrogen excess may result from exogenous administration (eg, various teas and creams), testicular and adrenal tumors, and chronic kidney and liver disease. Some causes of androgen insufficiency are genetic conditions (eg, Klinefelter syndrome and androgen insensitivity, an X-linked disorder) and testicular failure (eg, anorchia and primary hypogonadism). A large number of medications and street drugs have been implicated in male breast enlargement and work by either estrogenic, antiandrogenic, or other mechanisms. Some examples are: neurologic/psychiatric medications (eg, diazepam and risperidone), antiandrogens (eg, ketoconazole and spironolactone), cardiovascular medications (eg, calcium channel blockers), antiulcer drugs (eg, omeprazole), antineoplastic medications (eg, methotrexate), antimicrobials (eg, isoniazid), and a host of other exogenous hormones and products (eg, vaginal creams, licorice, and teas). Among the illicit drugs that have been implicated are anabolic steroids, marijuana, amphetamines, and alcohol.\n\nThe description of the mass in the vignette is consistent with physiologic gynecomastia and does not require a referral to endocrinology. If the mass does not regress and especially if it is causing emotional concerns, referral to a plastic surgeon may be considered at that time. Weight loss is useful only in pseudogynecomastia.\n\nCritique: The presence of palpable fibroglandular breast tissue under the nipple-areolar complex in a male that measures at least 0.5 cm in diameter, as described for the boy in the vignette, is referred to as gynecomastia. In the neonatal period and early adolescence, this is most often a physiologic phenomenon that regresses spontaneously. Breast tissue has both estrogen (stimulatory) and androgen (inhibitory) receptors. In males, during early puberty, the ratio of estrogen to testosterone is increased, and up to 70% of males in Sexual Maturity Rating (SMR) 2 stage of pubertal development (testicular volume of 5 to 10 mL) have some breast enlargement on examination that may be tender as a result of edema and inflammation. Initially unilateral, the process becomes bilateral in up to 75% of cases. In most males, such breast tissue no longer is palpable in 18 months (1 to 3 years), as puberty progresses and androgen concentrations increase.\n\nIn false or pseudogynecomastia, as seen in obese males, adipose tissue does not feel like a distinct, firm, rubbery mass. The doughnut sign is occasionally elicited when lying supine, in which the areola forms a depression within the surrounding adipose tissue.\n\nPathologic conditions involving the male breast can be local masses or result from hormonal aberrations of estrogen excess or androgen insufficiency. Rapid enlargement or a size greater than 4 cm is usually pathologic. Local masses, such as hemangiomas, lymphangiomas, lipomas, and neurofibromas, are usually unilateral, not circular, and not directly beneath the areola. Breast cancer in a male adolescent is extremely rare. Such a mass is hard and fixed rather than rubbery and movable over the chest wall. Estrogen excess may result from exogenous administration (eg, various teas and creams), testicular and adrenal tumors, and chronic kidney and liver disease. Some causes of androgen insufficiency are genetic conditions (eg, Klinefelter syndrome and androgen insensitivity, an X-linked disorder) and testicular failure (eg, anorchia and primary hypogonadism). A large number of medications and street drugs have been implicated in male breast enlargement and work by either estrogenic, antiandrogenic, or other mechanisms. Some examples are: neurologic/psychiatric medications (eg, diazepam and risperidone), antiandrogens (eg, ketoconazole and spironolactone), cardiovascular medications (eg, calcium channel blockers), antiulcer drugs (eg, omeprazole), antineoplastic medications (eg, methotrexate), antimicrobials (eg, isoniazid), and a host of other exogenous hormones and products (eg, vaginal creams, licorice, and teas). Among the illicit drugs that have been implicated are anabolic steroids, marijuana, amphetamines, and alcohol.\n\nThe description of the mass in the vignette is consistent with physiologic gynecomastia and does not require a referral to endocrinology. If the mass does not regress and especially if it is causing emotional concerns, referral to a plastic surgeon may be considered at that time. Weight loss is useful only in pseudogynecomastia.\n\nContent Specifications: Know the pathophysiology and differentiating features of normal vs abnormal gynecomastia in males"}
{"id" : 1432, "question_text" : "The mother of a 4-year-old boy with a severe peanut allergy informs you that she is pregnant. The mother is worried about the potential for allergies in the new child, and she asks for advice on how to prevent food allergies. The mother plans to feed formula to the baby. Of the following, the BEST advice for this mother is", "options" : "[\"delay the introduction of any solid foods until at least 7 to 8 months of age\", \"delay the introduction of peanuts and other allergenic foods until 2 years of age\", \"eat large amounts of peanuts and tree nuts during the pregnancy\", \"feed the baby a hydrolyzed formula\", \"feed the baby a soy-based formula\"]", "explanation" : "To reduce the risk of developing food allergies, the baby in this vignette should be fed a hydrolyzed formula. Given that this future child will have a first-degree relative with a significant food allergy, the use of a hydrolyzed formula during the first 4 months of life may help prevent the development of both atopic disease and cow milk protein allergy. An extensively hydrolyzed formula may offer more benefits than a partially hydrolyzed formula, but the data are inconclusive. No advantage has been found with using soy formulas to prevent food allergies and more studies are needed to evaluate the effects of using an amino acid-based formula.\n\nExclusive breastfeeding for the first 4 to 6 months of age is recommended and is associated with decreased atopic dermatitis during the first 2 years of life and a decreased rate of wheezing in children prior to the age of 4 years. Exclusive breastfeeding has also been associated with decreased rates of cow milk protein allergy, but not food allergies in general. There is no evidence to support maternal avoidance of eggs and cow milk (during pregnancy and lactation) to help prevent the development of food allergies, but more research is necessary to determine the results of peanut avoidance during pregnancy and lactation. The research on the effects of early maternal exposure to peanuts or the maternal avoidance of peanuts has been inconclusive. The introduction of solid foods should occur between 4 to 6 months of age. Delaying the introduction of solid foods or specifically delaying the introduction of highly allergenic foods (cow milk protein, egg, fish, shellfish, peanuts, tree nuts, soy, and wheat) does not decrease the risk of developing food allergies. The early introduction of these highly allergenic foods may actually reduce the risk of food allergy.\n\nPREP Pearls\n• Hydrolyzed formulas may prevent the development of atopic disease and the cow milk protein allergy.\n• Early introduction of highly allergenic food foods may reduce the risk of food allergy.\n\nABP Content Specifications(s)\n• Understand and apply current recommendations for feeding infants who are at risk for the development of food allergy\n\nSuggested Readings\n• Du Toit G, Roberts G, Sayre PH, et al. Randomized trial of peanut consumption in infants at risk for peanut allergy. N Engl J Med. 2015;372(9):803-813. doi: http://dx.doi.org/10.1056/NEJMoa1414850.\n• Fleischer DM, Spergel JM, Assa'ad AH, Pongracic JA. Primary prevention of allergic disease through nutritional interventions. J Allergy Clin Immunol Pract. 2013;1(1):29-36. doi: http://dx.doi.org/10.1016/j.jaip.2012.09.003.\n• Greer FR, Sicherer SH, Burks AW, Committee on Nutrition, Section on Allergy and Immunology. Effects of early nutritional interventions on the development of atopic disease in infants and children: the role of maternal dietary restriction, breastfeeding, timing of introduction of complementary foods, and hydrolyzed formulas. Pediatrics. 2008;121(1):183-191. doi: http://dx.doi.org/10.1542/peds.2007-3022.\n• Thompson RL, Miles LM, Lunn J, et al. Peanut sensitisation and allergy: influence of early life exposure to peanuts. Br J Nutr. 2010;103(9):1278-1286. doi: http://dx.doi.org/10.1017/S000711450999376X.\n• von Berg A, Filipiak-Pittroff B, Krämer U, et al. Allergies in high-risk school children after early intervention with cow's milk protein hydrolysates: 10-year results from the German Infant Nutritional Intervention (GINI) study. J Allergy Clin Immunol. 2013;131(6):1565-1573. doi: http://dx.doi.org/10.1016/j.jaci.2013.01.006."}
{"id" : 1536, "question_text" : "A previously healthy 13-year-old girl is brought to the emergency department because of altered mental status. She had been complaining of abdominal pain, nausea, and vomiting for 3 days before her presentation. For the past month, she has been drinking water and sports drinks excessively, and has been waking up in the middle of the night to urinate. During the day of admission, she became progressively somnolent and then unarousable, prompting her mother to bring her to the emergency department. On arrival, her temperature is 37.0°C; heart rate is 140 beats/min; respiratory rate is 50 breaths/min, and blood pressure is 100/60 mm Hg. Her oxygen saturation is 100% on room air. On physical examination, she is obtunded, responding to only painful stimuli with moaning and withdrawal of extremities. She has no spontaneous eye opening. Her pupils are 3 mm, equal, and reactive. Her eyes are sunken and her lips are cracked and dry. The girl's gag reflex is intact. Her extremities are cool, with a capillary refill of 3 seconds. Pulses are strong. Laboratory results are as follows: Sodium 124 mEq/L (124 mmol/L), Potassium 7.0 mEq/L (7.0 mmol/L), Chloride 100 mEq/L (100 mmol/L), Bicarbonate 5 mEq/L (5 mmol/L), Serum urea nitrogen 38 mg/dL (13.5 mmol/L), Creatinine 1.4 mg/dL (124 μmol/L), Glucose 1,350 mg/dL (74.9 mmol/L), Capillary blood gas pH 6.95, Partial pressure of carbon dioxide 14 mm Hg (1.8 kPa), Base deficit −25. Of the following, the BEST management option for this girl is", "options" : "[\"0.9% sodium chloride (NaCl) bolus, 40 mL/kg intravenously over 20 minutes\", \"3% NaCl bolus, 5 mL/kg intravenously over 20 minutes\", \"endotracheal intubation\", \"intravenous insulin infusion, 0.1 unit/kg per hour\", \"intravenous mannitol, 0.25 g/kg over 15 minutes\"]", "explanation" : "The girl in the vignette has diabetic ketoacidosis (DKA) from newly diagnosed type 1 diabetes mellitus. She is encephalopathic and dehydrated, but hemodynamically stable and maintaining her airway, oxygenation, and ventilation. The most appropriate next step in management among the choices listed is to start an intravenous insulin infusion.\n\nWith normal physiology, the release of insulin after a dextrose-rich meal leads to the uptake of glucose into fat, liver, and skeletal muscle tissue, as well as cellular glycogen and fat synthesis. In DKA, insulin deficiency causes decreased glucose uptake, and increased glycogenolysis, and gluconeogenesis. Abnormal glucose metabolism leads to the release of counterregulatory hormones including glucagon, epinephrine, and growth hormone, all of which further increase glycogenolysis and gluconeogenesis. As another counterregulatory mechanism to provide cellular energy in DKA, free fatty acids are released from adipose tissue and converted via beta-oxidation into ketoacids (acetoacetate and beta-hydroxybutyrate). Clinically, this leads to an elevated anion gap metabolic acidosis (bicarbonate level, (<15 mEq/L [<15 mmol/L]), hyperglycemia (>200 mg/dL [11.1 mmol/L]), and the presence of serum or urine ketones. Hyperglycemia causes glucosuria, which in turn leads to osmotic diuresis and dehydration. Polyuria and polydipsia are commonly seen. Hyperosmolarity is caused by ketoacidosis and hyperglycemia, as well as the hypernatremia and elevated urea nitrogen caused by dehydration from osmotic diuresis. Reversal of this physiology is achieved by administration of an insulin infusion.\n\nEncephalopathy from cerebral edema is an important potential complication of DKA. In response to elevated serum osmolarity, neurons produce organic osmolytes (also referred to as \"idiogenic osmoles\") to maintain osmolar equilibrium, thereby preventing cellular dehydration. As serum osmolarity decreases, cellular edema occurs because of shifting of water toward the higher intracellular osmolality caused by the organic osmolytes. This can occur before presentation from intake of hypotonic fluids, as well as iatrogenically because of overaggressive fluid administration. In DKA, administering 0.9% normal saline, which has an osmolality of 286 mmol/L, will lower serum osmolality. Although the girl in the vignette is dehydrated and needs correction of her fluid deficit, a fluid bolus of 40 mL/kg over 20 minutes would be excessive and could worsen cerebral edema. Most DKA treatment protocols include an initial normal saline bolus of 10 mL/kg over 1 hour, followed by correction of the free water deficit over 48 to 72 hours. For a patient in shock, more aggressive fluid administration is indicated, but should be balanced against potential neurologic complications.\n\nIf cerebral edema is severe, intracranial hypertension, herniation, and death can occur. Signs of severely elevated intracranial pressure and impending herniation include systemic hypertension, bradycardia, unreactive and/or unequal pupils, respiratory depression, and loss of cranial nerve function. Computed tomography of the brain is rarely indicated in the acute setting, because a patient's condition can deteriorate in the radiology suite where there is less monitoring, it can delay care, and the clinical examination is reliable enough to direct care.\n\nOsmotherapy, which may include 3% saline or mannitol, can be used to treat the rare patient with severe cerebral edema. However, this is not indicated for the girl in this vignette because her clinical presentation does not support this diagnosis. The rapid increase in serum sodium level that would result from treatment with 3% saline can cause central pontine myelinolysis, and osmotic diuresis from mannitol can worsen dehydration and predispose to venous sinus thrombosis.\n\nHypoventilation increases cerebral blood volume and intracranial pressure. Normal respiratory compensation for metabolic acidosis follows the Winters' Formula:\nPaCO2 (predicted) = 1.5*HCO3 + 8 ± 2\n\nIf a patient with metabolic acidosis has a partial pressure of arterial carbon dioxide (PaCO2) higher or lower than the predicted range, there is a concomitant respiratory acidosis or alkalosis, respectively. The girl in the vignette has a PaCO2 of 14 mm Hg, which falls within the range of her predicted PaCO2 of 13.5 to 17.5 mm Hg, so she does not have a respiratory disorder. In addition, because she is protecting her airway, endotracheal intubation is not indicated.\n\nPREP Pearls\n• Reversal of diabetic ketoacidosis occurs with the administration of an intravenous insulin drip.\n• Early fluid management of diabetic ketoacidosis that is either excessive or hypotonic may worsen cerebral edema and neurologic outcomes.\n• Patients with diabetic ketoacidosis rarely benefit from intubation. Indications include respiratory acidosis (which may be present even with partial pressure of arterial carbon dioxide levels below \"normal\" range), and loss of respiratory drive and airway protective reflexes.\n• Patients with diabetic ketoacidosis rarely benefit from osmotherapy. Indications include clinical findings of severe intracranial hypertension.\n\nMOCA-Peds Objective\n• Evaluate and manage a patient with metabolic acidosis\n\nABP Content Specifications(s)\n• Recognize the complications associated with diabetic ketoacidosis\n\nSuggested Readings\n• Cooke D, Plotnick L. Management of diabetic ketoacidosis in children and adolescents. Pediatr Rev. 2008;29:431-436. doi: 10.1542/pir.29-12-431.\n• Glaser NS, Wootton-Gorges SL, Buronocore MH, et al. Subclinical cerebral edema in children with diabetic ketoacidosis randomized to 2 different rehydration protocols. Pediatrics. 2013;131:e73-e80. doi: 10.1542/peds.2012-1049."}
{"id" : 228, "question_text" : "A-14 year-old girl presents with a 4-year history of recurrent infections. Her parents state that it seems she is on antibiotics almost every other month for the treatment of otitis media, sinusitis, or pneumonia. During a recent hospitalization for lobar pneumonia, the inpatient team measured serum immunoglobulins (Igs), which showed:\n• Low IgG of 54 mg/dL (0.54 g/L) (normal range, 700 to 1,500 mg/dL [7 to 15 g/L])\n• Absent IgA at <7.5 mg/dL (75 mg/L) (normal range, 15 to 200 mg/dL [150 to 2,000 mg/L])\n• Low IgM of 10 mg/dL (100 mg/L) (normal range, 50 to 300 mg/dL [500 to 3,000 mg/L])\n\nDespite the recurrent infections, the girl is otherwise growing and developing appropriately and has no other specific medical concerns.\n\nOf the following, the MOST appropriate next laboratory test is", "options" : "[\"flow cytometry for B lymphocytes, T lymphocytes, and natural killer cells\", \"genetic analysis for mutations of the Bruton tyrosine kinase (Btk) gene\", \"lymphocyte proliferation assay of peripheral blood mononuclear cells to mitogens\", \"measurement of antibody responses to protein and polysaccharide vaccines\", \"measurement of IgG subclasses (IgG1, IgG2, IgG3, IgG4)\"]", "explanation" : "An adolescent or young adult who has recurring infections and hypogammaglobulinemia, such as the girl described in this vignette, should be evaluated for common variable immunodeficiency (CVID). The diagnosis of CVID requires three criteria: a decrease of more than 2 standard deviations of one immunoglobulin below the age-adjusted mean (usually IgG, with decreased IgA or IgM), poor antibody response to protein (eg, diphtheria tetanus toxoid) and polysaccharide vaccines (eg, 23-valent pneumococcal vaccine), and exclusion of other causes of hypogammaglobulinemia (Item C121). Because the girl already meets the first criterion, the next step is to measure baseline antibody titers, vaccinate her, and repeat antibody measurements in 3 to 4 weeks. The recommended appropriate protein vaccine response in adolescents consists of a fourfold increase in titers. An appropriate pneumococcal polysaccharide response in patients 5 years and older is a titer of 1.3 µg/mL or higher in 70% of pneumococcal serotypes. Patients who demonstrate both hypogammaglobulinemia and impaired vaccine response should be referred to an immunologist for additional testing and consideration for either intravenous or subcutaneous immunoglobulin replacement therapy.\n\nFurther testing that is usually performed by the immunologist includes flow cytometry, consideration of genetic analysis for mutations in the Bruton tyrosine kinase (Btk) gene, lymphocyte proliferation assay, and assessment of IgG subclasses. Flow cytometry uses technology that can detect specific cell surface markers of T cells (CD3, CD4, CD8), B cells (CD19), and natural killer (NK) cells (CD16, CD56). Most patients who have CVID have normal B, T, and NK cell numbers, although up to 10% can have low B cell numbers, and many patients have an inverted CD4/CD8 ratio. Although flow cytometry is important, the results are not part of the current laboratory criteria for diagnosing CVID. Patients who have low-to-absent B-cell concentrations or who are younger than 2 years of age should be evaluated for X-linked recessive (Bruton) agammaglobulinemia. Flow cytometry screening detects most patients who have complete or partial expression of the Btk protein, but up to 30% of patients have abnormal function that can only be detected by Btk gene sequencing.\n\nAdditional T-cell qualitative analysis can include both anergy testing (eg, delayed hypersensitivity testing to Candida, tetanus, mumps, Trichophyton) and lymphocyte proliferation assay of peripheral blood mononuclear cells. Mitogen proliferation testing is expensive, typically yields normal results in patients who have CVID, and should be performed under the direction of an immunologist.\n\nMany clinicians assess IgG subclasses (ie, IgG1, IgG2, IgG3, and IgG4), but the clinical significance of a low IgG subclass value is unclear. Although low IgG2 concentrations have been associated with a poor polysaccharide response and low IgA concentrations in some patients, low values for one or more IgG subclasses is currently not recognized as a specific primary immunodeficiency, and the use of immunoglobulin replacement is controversial.\n\nCritique: [As above]\n\nContent Specifications: Plan the laboratory evaluation of antibody function (quantitative immunoglobulin concentrations, specific antibody to responses to both protein and polysaccharide vaccines)."}
{"id" : 2838, "question_text" : "A 3-day-old female neonate is seen in the emergency department for bleeding from the umbilical stump. Her mother had an uncomplicated pregnancy and a planned full-term delivery at home. Her mother reports that the umbilicus began bleeding 12 hours ago and has bled continuously despite pressure applied with a clean towel. There is no family history of a bleeding diathesis. The neonate is crying vigorously and appears pale. She has a weight of 3.4 kg, a temperature of 37°C, a heart rate of 178 beats/min, a respiratory rate of 28 breaths/min, a blood pressure of 66/42 mm Hg, and an oxygen saturation on room air of 95%. The lungs are clear bilaterally, and the heart rhythm is normal but tachycardic. There is no hepatomegaly or splenomegaly. There is a steady trickle of frank blood emanating from the umbilicus. There is no other evidence of bleeding. Laboratory data are shown:\n\nLaboratory Test | Result\nWhite blood cell count | 11,400/µL (11.4 × 109/L)\nHemoglobin | 11.2 g/dL (112 g/L)\nPlatelet count | 134 × 103/µL (134 × 109/L)\nNeutrophils | 53%\nLymphocytes | 42%\nMonocytes | 5%\nProthrombin time | 25.2 s\nPartial thromboplastin time | 62 s\n\nOf the following, the MOST likely condition to be contributing to this patient's presentation is", "options" : "[\"disseminated intravascular coagulation\", \"hereditary factor IX deficiency\", \"maternally derived antiplatelet antibodies\", \"a vitamin deficiency\"]", "explanation" : "Correct Answer: D\nThe neonate in the vignette has had frank bleeding from the umbilical stump that started at 3 days after birth, and it has lasted for at least 12 hours, strongly suggesting a coagulopathy. The absence of a family history of bleeding disorders decreases the likelihood of a genetic coagulopathy, and the history of a home birth is a risk factor for having not received vitamin K supplementation. Neonates who do not receive vitamin K supplementation shortly after birth are at risk of experiencing vitamin K deficiency bleeding (VKDB), also known as hemorrhagic disease of the newborn. Vitamin K is essential for the function of factors II, VII, IX, and X, affecting both the intrinsic and extrinsic coagulation pathway and thereby prolonging both the prothrombin time and the partial thromboplastin time (Item C38). Neonates are at particular risk of experiencing VKDB, as very little vitamin K crosses the placenta, the microbiome of neonatal intestines is not populated by the bacteria that produce vitamin K, and breast milk is a poor source of vitamin K. Neonatal VKDB can occur at any time from birth to 6 months of age and can be classified as early (within 24 hours), classical (1-7 days after birth) or late (2 weeks–6 months after birth). Early VKDB is typically severe and can present with hemorrhage into the intestines or head. Classical neonatal VKDB often presents with bruising and bleeding from the umbilical stump. Late VKDB can present with any type of bleeding, although intracranial bleeds are common. The infant in the vignette is exhibiting a classical presentation of neonatal VKDB.\n\nCoagulopathies can be related to platelet abnormalities (abnormal number or function) or to dysfunction of the coagulation cascade (hereditary or acquired). Disseminated intravascular coagulation is an acquired coagulopathy that can result in both thrombocytopenia and decreased levels of coagulation factors, but the neonate in the vignette is not exhibiting the stigmata of sepsis or disseminated intravascular coagulation. The female sex essentially eliminates hemophilia A (factor VIII deficiency) and hemophilia B (factor IX deficiency), because both are X-linked recessive disorders. She has mild thrombocytopenia on her complete blood count, but a platelet count of more than 100,000/µL is not low enough to permit bleeding from the umbilical stump. The mild thrombocytopenia is likely due to platelet consumption given the ongoing bleeding, which makes thrombocytopenia due to a maternally derived platelet antibody unlikely. Platelet function disorders are very rare, and the absence of a family history of a bleeding diathesis makes a platelet function disorder very unlikely in this scenario.\n\nPREP Pearls\n• Neonates who do not receive vitamin K supplementation shortly after birth are at risk of experiencing vitamin K deficiency bleeding, also known as hemorrhagic disease of the newborn.\n• Vitamin K is essential for the function of factors II, VII, IX, and X, affecting both the intrinsic and extrinsic coagulation pathways and thereby prolonging both the prothrombin time and the partial thromboplastin time.\n• Neonatal vitamin K deficiency bleeding can occur at any time from birth to 6 months of age and can be classified as early (within 24 hours), classical (1-7 days after birth), or late (2 weeks–6 months after birth).\n\nMOCA-Peds Objective\n• Evaluate and manage a patient with the new onset of thrombocytopenia.\n\nABP Content Specifications(s)\n• Plan the appropriate evaluation and management of an acquired bleeding or thrombotic disorder\n\nSuggested Readings\n• Callaghan MU, Rajpurkar M. Coagulation disorders. In: McInerny TK, Adam HM, Campbell DE, DeWitt TG, Foy JM, Kamat DM, eds. American Academy of Pediatrics Textbook of Pediatric Care. 2nd ed. Elk Grove Village, IL: American Academy of Pediatrics; 2017:1858-1867. Pediatric Care Online.\n• Lauer B, Spector N. Vitamins. Pediatr Rev. 2012;33(8):339-351. doi:10.1542/pir.33-8-339.\n• Pramanik AK. Bleeding disorders in neonates. Pediatr Rev. 1992;13(5):163-173. doi:10.1542/pir.13-5-163.\n• Stokowski LA. Reemergence of vitamin K deficiency bleeding. Adv Neonatal Care. 2014;14(2):75. doi:10.1097/ANC.0000000000000064."}
{"id" : 1396, "question_text" : "An 8-month-old female infant presents to the office with a 2-day history of fever, irritability, crying, vomiting, and blood-streaked diarrhea. She was a full term infant and has no significant past medical history. The parents report no recent travel other than to visit the maternal aunt for Thanksgiving 12 days ago. The aunt had prepared chitterlings (hog intestines), but the infant did not eat any and only took formula and boiled rice the aunt had made specifically for her. No one at dinner reported any subsequent illnesses. After returning home, she developed fever, was diagnosed with otitis media, and started on a course of amoxicillin, which is nearing completion. Her immunizations are up to date. Physical examination shows an irritable, crying female infant with a temperature of 39°C, heart rate of 120 beats/min, and respiratory rate of 30 breaths/min. Her anterior fontanelle is slightly sunken and her lips and mucosa are dry. She is making tears. Her lungs are clear to auscultation bilaterally and her heart sounds are normal. Her abdomen is soft, with no guarding or rebound tenderness. There is a large amount of urine, as well as blood-tinged diarrhea in her diaper. The baby is admitted to the local hospital for intravenous hydration and continued on amoxicillin. A routine stool culture is sent. She improves and is discharged after 48 hours. Three days later, the infant returns to your office and appears stable and well-hydrated. Her stool culture is noted to be negative for Salmonella, Shigella, and Campylobacter species. The mother reports that the patient is improving, but that the vomiting, fever, and bloody diarrhea are still present. Of the following, the MOST likely infectious etiology for this infant is", "options" : "[\"Bacillus cereus\", \"Clostridium difficile\", \"Rotavirus\", \"Trichinella\", \"Yersinia enterocolitica\"]", "explanation" : "The most likely infectious etiology in the infant in the vignette with bloody diarrhea is Yersinia enterocolitica. Yersinia species are gram-negative rods and 3 of the species are human pathogens: Yersinia pestis (spread by infected fleas infesting rats), Yersinia pseudotuberculosis, and Yersinia enterocolitica. The latter 2 species cause yersiniosis, a febrile diarrheal illness. Yersinia species are primarily zoonoses, causing disease in domestic and wild animals. Humans are not part of the natural bacterial life cycle and are inadvertent hosts. The principal reservoir of Y enterocolitica is swine, particularly domesticated pigs. Y pseudotuberculosis is rare in the United States and is found in rodents, birds, cattle, goats, sheep, deer, and other mammals. Yersiniosis primarily occurs through ingestion of contaminated food or water, especially undercooked or raw pork, and contact with animals. It has been associated with the preparation of chitterlings (also known as chitlins), which are prepared from pig intestines that must be carefully cleaned and rinsed before being boiled and stewed to prevent transmission. In 1988, an outbreak occurred in Georgia in 14 bottle-fed infants with a median age of 3 months. None of the infants had contact with the chitterlings, but the infection most likely occurred due to cross-contamination of the bottles or formula by those preparing the chitterlings.\n\nAfter an incubation period of 1 day to 2 weeks (typically 4 to 6 days), patients develop fever and diarrhea (often bloody in children), with abdominal pain, nausea, and vomiting that is indistinguishable from other acute diarrheal illnesses. Up to 20% of patients report pharyngitis (possibly from the affinity Yersinia species have for lymphoid tissue). The pharyngitis may provide an important diagnostic clue for the diarrhea's etiology, as this is not associated with other acute bacterial diarrheas. The course of illness may be more insidious than other bacterial diarrheas, with patients in one study not seeking medical attention for over 1 week, and stool cultures not requested by providers for almost 2 weeks from onset. The duration of diarrhea is typically longer than the usual acute gastroenteritis, sometimes persisting up to 3 weeks. Older children and adults may develop pseudoappendicitis, with right lower quadrant pain and elevated white blood cell counts. Abdominal pain from ileocecal mesenteric adenitis and terminal ileitis can occur. Younger children, immunocompromised patients, and individuals with iron overload syndromes are at risk of Yersinia bacteremia or sepsis. Postinfectious complications include reactive arthritis (particularly those with HLA-B27 antigen) and erythema nodosum. Y enterocolitica grows more slowly than other enterobacterial pathogens on routine laboratory media. If yersiniosis is suspected, the microbiology laboratory should be notified so that additional special media can be used to isolate the Yersinia and prevent false-negative results.\n\nThere is no good evidence that antibiotics are of any benefit in the treatment of acute uncomplicated yersiniosis. Treatment for Yersinia sepsis may be necessary for patients with severe disease or significant underlying conditions. A fluoroquinolone (ie, ciprofloxacin), doxycycline (if older than 8 years of age), and trimethoprim-sulfamethoxazole (especially for pediatric patients) would be the oral drugs of choice. Intravenous therapy would include a third-generation cephalosporin, such as ceftriaxone or a fluoroquinolone, plus gentamicin.\n\nBloody diarrhea, exposure to chitterlings, failure to respond to amoxicillin, and protracted illness in the infant in the vignette are all expected with a diagnosis of yersiniosis. While Bacillus cereus can cause severe nausea, vomiting, and diarrhea, the diarrhea is usually nonbloody, and B cereus arises from fried rice that has been sitting at room temperature for hours, not boiled rice. Rotavirus could cause prolonged fever, vomiting, and diarrhea, but the diarrhea would not be bloody, and this patient's immunizations, which would include the rotavirus vaccine series, are up to date. While Trichinella roundworms are commonly found in swine, it would come from the meat, not the intestines. Most trichinosis is subclinical, but acute trichinosis can present with diarrhea, nausea, vomiting, and abdominal pain, followed by fever, periorbital edema, and myalgias as the larvae migrate through the muscles. Clostridium difficile should be a consideration in a patient on antibiotics, who develops fever, abdominal pain, and bloody diarrhea, but pseudomembranous colitis rarely occurs before the first 12 to 24 months of life. C difficile toxin may be present with positive laboratory findings, but neonates and infants appear to lack the ability to bind and process the clostridial toxin, creating asymptomatic carriage and preventing colitis from occurring.\n\nPREP Pearls\n• Yersinia enterocolitica is associated with fever and diarrhea (often bloody in children), abdominal pain, nausea, and vomiting that is frequently indistinguishable from other acute diarrheal illnesses. Yersiniosis is associated with a slow subclinical onset and protracted duration of up to 3 weeks.\n• Pharyngitis may provide an important diagnostic clue for Yersinia as the causative pathogen because pharyngitis is not associated with other acute bacterial diarrheas.\n• Antibiotics are not beneficial in the treatment of acute uncomplicated yersiniosis.\n\nABP Content Specifications(s)\n• Recognize the clinical features associated with Yersinia enterocolitica infection\n• Plan appropriate management for a patient with Yersinia enterocolitica infection\n\nSuggested Readings\n• American Academy of Pediatrics. Yersinia enterocolitica and Yersinia pseudotuberculosis infections (enteritis and other illnesses). In: Kimberlin DW, Brady MT, Jackson MA, Long SS, eds. Red Book: 2015 Report of the Committee on Infectious Diseases. 30th ed. Elk Grove Village, IL: American Academy of Pediatrics; 2015:868-870.\n• Ong KL, Gould LH, Chen DL, et al. Changing epidemiology of Yersinia enterocolitica infections: markedly decreased rates in young black children, Foodborne Diseases Active Surveillance Network (FoodNet), 1996-2009. Clin Infect Dis. 2012;54 Suppl 5:S385-S390. doi: http://dx.doi.org/10.1093/cid/cis053."}
{"id" : 3632, "question_text" : "A 15-year-old boy is admitted to the hospital with cough, intermittent fevers, fatigue, and an abnormal chest radiograph that has not improved after treatment with oral cephalexin for 2 weeks. He complains of a sore throat, but denies joint pain or gastrointestinal symptoms. He has had transient dark urine, but no dysuria or flank pain. He has severe nodular acne for which he has been receiving doxycycline since 2 weeks before the onset of this illness. On physical examination, the boy appears moderately ill but is able to answer questions. His oxygen saturation is 85% in room air, which rises to 95% with high-flow nasal cannula at 40 L/min and FiO2 0.5. He has diffusely decreased breath sounds, more on the right than the left, with no crackles or wheezes. Cardiovascular examination findings are normal. There is no hepatosplenomegaly and no skin rashes other than nodular facial acne. A chest radiograph is obtained (Item Q76A and Item Q76B). Shortly after admission, he has an episode of bright red hemoptysis. Laboratory evaluation demonstrates the following. Laboratory Test Result Hemoglobin 7.5 g/dL (75 g/L) Hematocrit 22.3% Platelets 759,000/μL (759 × 109/L) Creatinine 1.09 mg/dL (96 μmol/L). Of the following, the MOST appropriate next step in evaluation for this boy's hemoptysis is", "options" : "[\"24-hour urine collection for protein and creatinine analysis\", \"quantitative serum immunoglobulin levels\", \"renal ultrasonography\", \"serum antineutrophil cytoplasmic antibodies\"]", "explanation" : "The combination of hemoptysis with multifocal densities on chest radiography, urinary findings (dark urine and borderline elevated creatinine), anemia, and thrombocytosis suggests a systemic vasculitis such as granulomatosis with polyangiitis (previously called Wegener syndrome). Measurement of serum antineutrophil cytoplasmic antibodies (ANCA) would be the appropriate diagnostic test.\n\nThe differential diagnosis for this boy would also include systemic lupus erythematosus, Goodpasture syndrome, and Henoch-Schonlein purpura. Infection and bronchiectasis are the most likely causes of hemoptysis in children and adults, but are not usually associated with findings of renal involvement, as seen in this child.\n\nFurther evaluation of this boy's renal function will be necessary, but neither measurement of 24-hour urine protein and creatinine levels nor renal ultrasonography will lead to a specific diagnosis and would not help to explain his hemoptysis. He is not presenting with recurrent respiratory infections or radiographic signs of bronchiectasis, therefore, serum immunoglobulins, although potentially elevated, will not assist with the diagnosis. His findings suggest a multiorgan process, for which assessment of systemic inflammation is appropriate. Other studies that would be helpful in establishing a systemic inflammatory process and evaluating for other causes of hemoptysis include erythrocyte sedimentation rate, C-reactive protein, antinuclear antibody, serum myeloperoxidase, and antiproteinase-3 antibody levels.\n\nPREP Pearls\n• Infection with or without bronchiectasis is the most common cause of hemoptysis in children.\n• When hemoptysis is associated with multisystemic findings, a diffuse inflammatory process should be the primary consideration.\n\nABP Content Specifications(s)\n• Plan the appropriate clinical and diagnostic evaluation of hemoptysis\n• Plan the appropriate management of hemoptysis in patients of various ages\n\nSuggested Readings\n• de Silva C, Mukherjee A, Jat KR, Lodha R, Kabra SK. Pulmonary hemorrhage in children: etiology, clinical profile and outcome. Indian J Pediatr. 2019;86(1):7-11. doi:10.1007/s12098-018-2725-x.\n• Greco A, Marinelli C, Fusconi M, et al. Clinic manifestations in granulomatosis with polyangiitis. Int J Immunopathol Pharmacol. 2016;29(2):151-159. doi:10.1177/0394632015617063.\n• Lutalo PM, D'Cruz DP. Diagnosis and classification of granulomatosis with polyangiitis (aka Wegener's granulomatosis). J Autoimmun. 2014;2:48-49, 94-98. doi:10.1016/j.jaut.2014.01.028.\n• Shnayder R, Needleman JP. Hemoptysis. Pediatr Rev. 2018;39(6):319-321. doi:10.1542/pir.2017-0157.\n• Simon DR, Aronoff SC, DelVecchio MT. Etiologies of hemoptysis in children: a systematic review of 171 patients. Pediatr Pulmonol. 2017;52(2):255-259. doi:10.1002/ppul.23497."}
{"id" : 3612, "question_text" : "A 6-year-old boy is being evaluated for concerns about impulsive behavior and difficulty paying attention in class. His mother states that this has been a problem since he started kindergarten 18 months ago. The boy makes friends easily and is recognized as a leader in the class. His teacher has suggested evaluation for attention-deficit/hyperactivity disorder. On further questioning, his mother reports restless sleep, nocturnal enuresis, and intermittent snoring. On physical examination, his weight is 20 kg, height is 115 cm, heart rate is 90 beats/min, and oxygen saturation in room air is 98%. The only notable findings are mildly congested nasal mucosa and 3+ noninflamed tonsils. Of the following, the evaluation MOST likely to lead to an explanation for this boy's symptoms is", "options" : "[\"allergy skin testing\", \"electroencephalography\", \"overnight sleep study\", \"Vanderbilt Rating Scales\"]", "explanation" : "Correct Answer: C\nIn the face of snoring, tonsillar hypertrophy, and nasal congestion, obstructive apnea/hypopnea must be considered as a cause for this boy's sleep disturbance and attention problems. Without witnessed apnea, the most appropriate evaluation is an overnight sleep study with recording of sleep state, nasal airflow, respiratory effort, and pulse oximetry, with or without end-tidal carbon dioxide monitoring.\n\nAlthough his behavior may be suggestive of ADHD, the constellation of symptoms is more supportive of obstructive sleep apnea/hypopnea. Obtaining the Vanderbilt Rating Scales would be an appropriate part of the evaluation, but would not lead to a diagnosis tying together his full symptom complex. It is possible that allergies may be contributing to his nasal congestion and thus to his sleep disturbance; it would be appropriate to address the nasal congestion before performing a sleep study, but that would not require formal allergy testing. His history does not suggest a seizure disorder.\n\nObstructive sleep apnea/hypopnea can have unusual presentations. In the child who is inattentive at school, with or without hyperactivity, the possibility of a sleep disorder should be considered. Nocturnal enuresis is also associated with sleep disorders, especially if it recurs after reaching overnight bladder control for some time. The boy in the vignette is not obese, but obesity is a risk factor for sleep-disordered breathing.\n\nPREP Pearls\n• Sleep-disordered breathing can have symptoms not directly related to sleep.\n• Aggressive behavior, attention deficit, and hyperactivity as well as unexpected daytime somnolence can be due to sleep apnea/hypopnea.\n• Nocturnal symptoms of enuresis and restlessness as well as frank snoring can be manifestations of sleep apnea/hypopnea.\n\nMOCA-Peds Objective\n• Recognize and evaluate a patient with sleep-disordered breathing.\n\nABP Content Specifications(s)\n• Plan appropriate management of obstructive sleep apnea\n• Plan an appropriate evaluation for obstructive sleep apnea\n• Recognize complications associated with obstructive sleep apnea\n\nSuggested Readings\n• Gipson K, Lu M, Kinane B. Sleep-disordered breathing in children. Pediatr Rev. 2019;40:3-12. doi:10.1542/pir.2018-0142.\n• Marcus CL, LJ Brooks, KA Draper, et al. Diagnosis and management of childhood obstructive sleep apnea syndrome. Pediatrics. 2012;130(3):e714-e755. doi:10.1542/peds.2012-1672.\n• Miano S, Amato N, Foderaro G, et al. Sleep phenotypes in attention deficit hyperactivity disorder. Sleep Med. 2019;60(9):123-131. doi:10.1016/j.sleep.2018.08.026.\n• Silvestri R, Gagliano A, Arico I, et al. Sleep disorders in children with attention-deficit/hyperactivity disorder recorded by overnight polysomnography. Sleep Med. 2009;10(10):1132-1138. doi:10.1016/j.sleep.2009.04.003.\n• Tsukada E, Kitamura S, Enomoto M, et al. Prevalence of childhood obstructive sleep apnea syndrome and its role in daytime sleepiness. PLoS One. 2018;13(10):00204409. doi:10.1371/journal.pone.0204409."}
{"id" : 1364, "question_text" : "You are seeing a 12-year-old girl in your clinic for a health supervision visit. She is concerned about the new appearance of \"dirt\" in her ears, which she is unable to remove. On physical examination, her vital signs, growth velocity, and body mass index are all within normal range for age. Her sexual maturity rating is 2 for breast and 3 for pubic hair development. The remainder of her examination is unremarkable, except for the skin findings on both ears (Item Q115). Of the following, the BEST description of this girl's acne is", "options" : "[\"closed comedonal\", \"nodular\", \"open comedonal\", \"popular\", \"pustular\"]", "explanation" : "The girl in the vignette has open comedonal acne, which is characterized by small dome-shaped papules with an open orifice that appears blackened. The black color is speculated to be secondary to oxidation of keratinous lipoid material at the opening, deposition of melanin, or interference of light transmission.\n\nAcne vulgaris is a very common skin disorder that can occur at any age, but primarily affects adolescents and young adults. It is important for pediatric healthcare providers to recognize the clinical findings of the different types and degrees of acne to educate patients and prescribe appropriate therapy.\n\nAcne vulgaris is a chronic inflammatory process of the pilosebaceous unit. Increased sebum production, obstruction of the follicular ostium, proliferation of Propionibacterium acnes, and secondary inflammation all contribute to the development of acne. Acne can be classified as comedonal (closed or open), inflammatory (papules, pustules, nodules, cysts), or mixed. Closed comedones are tiny yellowish-white or flesh-colored papules without surrounding erythema, commonly known as whiteheads. They are caused by obstructed follicles leading to trapped epithelial cells and sebum. Open comedones have a dilated follicular orifice that gives the blackhead appearance. As comedones rupture and become inflamed, erythematous papules or pustules are created. Pustules larger than 5 mm in diameter are termed nodules. As these lesions grow deeper and larger, sinus tracts and cysts can develop. The degree of inflammation is key to potential scar formation.\n\nAcne is often classified as mild, moderate, or severe. The number and type of lesions, distribution, and the presence or absence of scarring are typically considered in grading severity (Item C115A).\n\nEvidence-based guidelines from the American Acne and Rosacea Society, and endorsed by the American Academy of Pediatrics, were published in 2013. Effective treatment (Item C115B) must be individualized, based on the type and severity of acne, while considering the response to previously attempted therapies (both over-the-counter and prescription), cost, the likelihood of compliance, and the psychosocial impact of disease. Patient education is vital to a successful outcome.\n\nPREP Pearls\n• Acne is categorized as comedonal (closed or open), inflammatory (papules, pustules, nodules, cysts), or mixed.\n• Acne severity is classified as mild, moderate, or severe, based on the number, type, and distribution of lesions or scarring.\n• Treatment must be individualized based on the type and severity of acne, while considering cost effectiveness and likelihood of compliance\n\nABP Content Specifications(s)\n• Recognize the clinical findings associated with acne\n\nSuggested Readings\n• Basak S, Zaenglein A. Acne and its management. Pediatr Rev. 2013;34(11):479-497. doi: http://dx.doi.org/10.1542/pir.34-11-479.\n• Eichenfield LF, Krakowski AC, Piggott C, et al. Evidence-based recommendations for the diagnosis and treatment of pediatric acne. Pediatrics. 2013;131(S3):S165-S187. https://www.ncbi.nlm.nih.gov/pubmed/23637225.\n• Krowchuk DP. Managing adolescent acne: a guide for pediatricians. Pediatr Rev. 2005;26(7):250-261. doi: http://dx.doi.org/10.1542/pir.26-7-250."}
{"id" : 1682, "question_text" : "After you introduce yourself during a routine health supervision visit, your male patient is able to clearly tell you his first and last name, but not his phone number. You offer him some crayons and paper while you visit with his parents. They do not report any health concerns. He likes to dress himself, but needs some help with tying his shoes. He can brush his teeth fairly well and is toilet trained. He tells you he likes to play with blocks and can build a house. His mother adds that he will play simple board games that involve matching colors, but gets angered by games that involve a lot of rules, especially if others win. He is very inquisitive and asks a lot of \"why\" questions. He is a well-developed, well-nourished child who is able to hop on 1 foot 7 times, but is not able to skip. He tells you he is good at riding his tricycle and catching a ball. You note that he is able to copy a square, but not a triangle.\n\nOf the following, these findings are MOST typical for a child whose age is", "options" : "[\"30 months\", \"36 months\", \"48 months\", \"60 months\", \"72 months\"]", "explanation" : "Correct Answer: C\nThe child described in this vignette exhibits the cognitive-behavioral and motor milestones typically attained by 4 years of age. These milestones include:\n• Language: clearly states first and last name, speaks in paragraphs using past and present tense, is 100% intelligible to family and strangers, identifies 5 or 6 colors, asks many \"why\" questions\n• Social-emotional: dresses self but not yet able to tie shoelaces, brushes teeth and uses toilet independently, plays simple interactive board games, voices frustration about rules, displays curiosity, begins to understand the feelings of others, shows increased cooperative play\n• Gross motor: hops on 1 foot several times, catches a large ball, performs a standing broad jump\n• Fine motor: builds a house with blocks, copies a cross and square, holds a crayon well, cuts with scissors on a line\n\nA younger child would have less advanced language skills. The ability to tell a story about an experience that includes past and present tense plus the ability to relate some emotion and the perspective of others is not typical until 4 years of age. Most 4 year olds will not be able to recite their telephone number, which is a skill more typical of a child entering kindergarten at 5 years of age. By 60 months of age and beyond, a child's language will have grown to more than 2,000 words; they form sentences with 6 to 8 words and are able to answer \"why\" questions. A 6-year-old child is able to read words by site recognition and exhibits beginning phonemic awareness.\n\nSocial-emotional development progresses from the imitative and pretend parallel play found at 30 months of age to simple imaginative play and cooperative sharing with a peer at 36 months of age. Four-year-old children are usually able to distinguish between real and imaginary; they can play in groups of 3 or 4; and their imaginative play is becoming more complex. At 60 months of age and beyond, children are acquiring the social skills demanded in larger structured group settings, such as school. Self-help skills of dressing, toileting, and brushing teeth need more assistance in the younger child. The 4-year-old child is able to accomplish most of these tasks independently, but the dexterity to tie shoelaces is usually not present until 5 years of age. The ability to play games with more involved rules emerges at 6 years (72 months) of age.\n\nFor gross motor skills, a 30-month-old child is able to jump in place, and a 36-month-old child is learning to pedal a tricycle and hop a few times on 1 foot. A 5 year old is able to skip, hop on 1 foot more than 10 times, and perform a running broad jump. Coordination, balance, and speed continue to show improvement in the 6-year-old child.\n\nFine motor skills are demonstrated through building blocks. At 30 months of age, a tower of 8 cubes can be built. This skill progresses to a 3-block bridge at 36 months and to a 5-block gate or simple house at 48 months. Early literacy skills such as drawing and cutting can also be used to assess fine motor skills. Vertical and circular strokes will begin to form in the scribbling of a 24 month old. By 36 months of age a child can copy a circle. Copying a cross or square is typical for the 4 year old, copying a triangle is present at 5 years, and copying a diamond can be demonstrated by a 6 year old. A 3 year old can cut paper with child-safe scissors, but is not able to cut on a line until 4 years of age or to cut more refined shapes well until 60 months or older. A comprehensive summary of developmental milestones can be found in Table 1 of \"Developmental Milestones: Motor Development\" (Pediatr Rev. 2010;31[7]:267-277).\n\nPREP Pearls\n• Language milestones attained by 4 years of age include: clearly states first and last name, speaks in paragraphs using past and present tense, is 100% intelligible to family and strangers, identifies 5 or 6 colors, asks many \"why\" questions.\n• Social-emotional milestones attained by 4 years of age include: dresses self but not yet able to tie shoelaces, brushes teeth and uses toilet independently, plays simple interactive board games, voices frustration about rules and displays curiosity, begins to understand the feelings of others, shows increased cooperative play.\n• Gross motor milestones attained by 4 years of age include: hops on 1 foot several times, catches a large ball, performs a standing broad jump.\n• Fine motor milestones attained by 4 years of age include: builds a house with blocks, copies a cross and square, holds a crayon well, cuts with scissors on the line.\n\nABP Content Specifications(s)\n• Evaluate the motor developmental progress/status of a child at 4 years of age\n• Evaluate the cognitive and behavioral developmental progress/status of a child at 4 years of age\n\nSuggested Readings\n• Feigelman S. Middle childhood. In: Kliegman RM, Stanton BF, St Geme JW III, Schor NF, eds. Nelson Textbook of Pediatrics. Philadelphia, PA: Elsevier; 2016:79–84.\n• Feigelman S. The preschool years. In: Kliegman RM, Stanton BF, St Geme JW III, Schor NF, eds. Nelson Textbook of Pediatrics. Philadelphia, PA: Elsevier; 2016:76–79.\n• Gerber RJ, Wilks T, Erdie-Lalena C. Developmental milestones 3: social-emotional development. Pediatr Rev. 2011;32(12):533–536. doi: http://dx.doi.org/10.1542/pir.32-12-533.\n• Gerber RJ, Wilks T, Erdie-Lalena C. Developmental milestones: motor development. Pediatr Rev. 2010;31(7):267–277. doi: http://dx.doi.org/10.1542/pir.31-7-267.\n• McQuiston S, Kloczko N. Speech and language development: monitoring process and problems. Pediatr Rev. 2011;32(6):230–239. doi: http://dx.doi.org/10.1542/pir.32-6-230."}
{"id" : 308, "question_text" : "You are called to evaluate a 2-year-old girl in the emergency department who has septic shock. She has received 80 mL/kg of normal saline since her arrival 45 minutes ago as well as appropriate antibiotics. On physical examination, her heart rate is 140 beats/min, respiratory rate is 30 breaths/min, and blood pressure is 65/40 mm Hg. She appears lethargic but arouses with vigorous stimulation. Her extremities are cool, it is difficult to feel peripheral pulses, and her capillary refill time is 5 seconds. Of the following, the MOST appropriate next step is initiation of an infusion of", "options" : "[\"dobutamine\", \"dopamine\", \"milrinone\", \"norepinephrine\", \"vasopressin\"]", "explanation" : "Septic shock, the presence of sepsis and cardiovascular organ dysfunction, as described for the girl in the vignette, is a medical emergency that requires prompt recognition and treatment. One of the most important factors in lowering the mortality associated with septic shock is early and aggressive fluid resuscitation, defined as isotonic fluid boluses of 20 mL/kg titrated according to clinical assessment of adequacy of cardiac output, such as heart rate, urine output, and level of consciousness. Often, 60 mL/kg is needed for the initial resuscitation of a child who has septic shock. The choice of fluid (crystalloid versus colloid) has been the subject of much debate, with little evidence to support the superiority of one over the other.\n\nFor those who have fluid-refractory shock (evidence of persistent shock despite appropriate fluid resuscitation), such as this girl, the addition of vasoactive agents is required. Dopamine or epinephrine is considered first-line therapy for \"cold shock\" (increased heart rate, normal-to-low blood pressure, decreased pulses), and norepinephrine is first-line therapy for \"warm shock\" (increased heart rate, decreased blood pressure, and bounding pulses). Vasopressin has been used in cases of refractory warm shock, but a recent multicenter controlled study did not demonstrate any benefit with the addition of this agent to standard therapy, and, in fact, a trend toward increased mortality was seen that did not reach statistical significance. Dobutamine and milrinone are indicated for patients experiencing cardiogenic shock.\n\nCritique: Preferred Response: B\n\nContent Specifications: Know the guidelines for the initial therapy of hypovolemic or septic shock"}
{"id" : 1335, "question_text" : "You receive a call from an anxious father whose 6-year-old daughter was just diagnosed with head lice. The father has several questions regarding the etiology, treatment, and prevention of head lice. Of the following, you are MOST likely to tell the father that", "options" : "[\"any person that shares a bed with his daughter should be treated, even if no live lice are found\", \"no nit\\\" policies for return to school have been effective in reducing the transmission of lice\", \"routine lice screening at schools has been effective in reducing the transmission of lice\", \"treatment with 1% permethrin should be repeated in 3 to 5 days if live lice are seen\", \"untreated head lice has been associated with transmission of Lyme disease\"]", "explanation" : "Correct Answer: A\nFor the girl in the vignette found to have head lice, the most appropriate recommendation to the father is that any person that shares a bed with his daughter should be treated, even if no live lice are found. Despite the fact that head lice are not a health hazard and are not known to spread disease, lice continues to be a source of great anxiety for many parents, teachers, and children. The annual cost associated with lice and lice treatment (including remedies, lost wages, school system expenses) is estimated at $1 billion. Many of these expenses are due to a misunderstanding of the human lice life cycle and proper ways to screen and treat lice.\n\nThe adult head louse is typically 2 to 3 mm in length and is tan or gray in color. The female head louse can live up to 3 to 4 weeks and lays up to 10 eggs per day. These eggs firmly attach to the hair usually within 4 mm of the scalp. The eggs typically hatch after 7 to 12 days, releasing a nymph that will undergo 3 more nymph stages until becoming an adult louse 9 to 12 days later. Approximately 1.5 days after becoming an adult, the female adult louse begins to lay eggs. This cycle will repeat itself every 3 weeks unless treatment for the lice is initiated.\n\nLice can move only by crawling; transmission typically occurs through direct contact (head-to-head contact). Indirect transmission through objects such as hats and combs is a much less likely mode of transmission. Diagnosis of lice is made by the identification of an adult louse, nymph, or egg in the scalp.\n\nTreatment for lice consists not only of topical treatment of the scalp, but preventive measures to help stop the spreading of lice. All household members should be checked for lice and treated if nits within 1 cm of the scalp or live lice are found. Additionally, all family members who share a bed with the patient should be treated as well, regardless of whether any lice or nits are found on examination. Despite the fact that indirect transmission is much less likely to occur than direct transmission, it is advised to wash hair care items and bedding of the patient. Additionally, items that have come into contact with the patient's head over the past 24 to 48 hours (clothing, headgear, furniture, carpeting, and rugs) should be cleaned as well. Items such as clothing and rugs should be washed and dried at a temperature greater than 54.4°C, and furniture and carpeting should simply be vacuumed.\n\nThere are multiple topical agents used to treat head lice, with 1% permethrin or pyrethrins being the first-line agents unless significant resistance to permethrin has been reported. If live lice are still seen 7 to 10 days after application of the 1% permethrin or pyrethrins, a repeat application is recommended. Many other topical agents (including malathion 0.5%, benzyl alcohol 5%, topical ivermectin, and spinosad) are available when first-line agents do not work or if resistance is high. The safety, efficacy, and price of these agents should be examined in order to determine the best treatment for the patient.\n\nIt is important to make sure that school personnel have been properly educated on lice transmission and treatment. The routine lice screening at schools has not been shown to be effective at reducing lice transmission rates at school and is also not cost effective. A child should not be restricted from school because of lice, not on the day of diagnosis or on any day after. The \"no-nit\" policies, where patients are excluded from school until all nits have been removed, should not be enforced or tolerated.\n\nPREP Pearls\n• Pyrethrins and 1% permethrin are first-line treatment for head lice.\n• Children with lice should not be excluded from schools.\n• Lice screening in schools and \"no nit\" policies have not been shown to be effective in reducing the spread of lice in schools.\n\nABP Content Specifications(s)\n• Understand the life cycle of human lice\n\nSuggested Readings\n• American Academy of Pediatrics. In: Kimberlin DW, Brady MT, Jackson MA, Long SS, eds. Pediculosis capitis. Red Book: 2015 Report of the Committee on Infectious Diseases. 30th ed. Elk Grove Village, IL: American Academy of Pediatrics; 2015:597-601.\n• Bloomfield D. Head lice. Pediatr Rev. 2002;23(1):34-35. doi: http://dx.doi.org/10.1542/pir. 23-1-34.\n• Devore CD, Schutze GE, Council on School Health and Committee on Infectious Diseases. Head lice. Pediatrics. 2015;135(5):e1355-e1365. doi: http://dx.doi.org/10.1542/peds.2015-0746."}
{"id" : 364, "question_text" : "A 15-year-old boy was stung by a hornet and experienced anaphylaxis with urticaria, dyspnea, and throat swelling. He states that he has been stung a number of times in the past but only experienced local pain and erythema at the sting site. His parents are worried because he spends a lot of time hiking and camping with friends. Of the following, the MOST accurate statement regarding Hymenoptera allergy is that", "options" : "[\"additional Hymenoptera testing is not required if initial blood test results are negative\", \"Hymenoptera allergy testing should be performed only to the insect identified by the patient\", \"Hymenoptera immunotherapy is generally prescribed for 1 to 2 years before discontinuation\", \"immunotherapy is not recommended for children younger than 16 years of age who experience only cutaneous symptoms\", \"this boy's risk for anaphylaxis to a future Hymenoptera sting is approximately 5% to 10%\"]", "explanation" : "The teenager described in the vignette has had an anaphylactic reaction, and is at an increased risk for anaphylaxis with future stings, despite his history of only experiencing normal reactions to flying insect stings in the past. After initial management, he should be given an autoinjector containing epinephrine and a referral to an allergist. Because certain sting reactions warrant skin testing and possible venom immunotherapy (VIT), a careful history of all sting reactions is important. Interestingly, VIT is not recommended in children younger than 16 years of age who experience reactions limited to urticaria or angioedema; the risk for future reactions in these patients is 5% to 10%, which is similar to individuals experiencing large local reactions.\n\nBecause the reaction described for this boy is consistent with anaphylaxis, his future risk of a reaction is 20% to 60% without VIT and, therefore, further testing should be considered. Skin testing is recommended for individuals older than 16 years who have any type of systemic reaction, even cutaneous symptoms. Skin testing should also be considered for younger patients who have experienced more than cutaneous symptoms. Those who have negative skin testing results should also undergo venom-specific immunoglobulin (Ig)E blood testing because skin testing alone may miss the presence of IgE in 10% to 20% of patients. If serum testing is performed first and results are negative, additional venom skin testing should be performed for the same reasons.\n\nBecause insect identification can be difficult, serum or skin testing should be performed to all five flying insects: wasp, yellow jacket, honeybee, yellow hornet, white faced hornet. Rarely, bumblebee may need to be added if there is a strong history for a large, fuzzy bee and results of initial honeybee testing, which is highly cross-reactive to bumblebee, are negative. Finally, a serum tryptase should be ordered in patients experiencing anaphylaxis because 1% to 10% may have underlying mastocytosis or a clonal mast cell disease that was provoked by the insect sting.\n\nAllergen immunotherapy is beneficial in high-risk patients because it lowers the risk of future reaction to 10% for honeybees and less than 5% for the other flying insects. Current guidelines recommend continuing VIT for 3 to 5 years. However, studies have shown that even after 5 years of VIT, the risk for reaction after stopping VIT increases to 15% per sting, although the risk for a severe reaction remains low (<3%). For some patients, VIT may be recommended indefinitely.\n\nCritique: The teenager described in the vignette has had an anaphylactic reaction, and is at an increased risk for anaphylaxis with future stings, despite his history of only experiencing normal reactions to flying insect stings in the past. After initial management, he should be given an autoinjector containing epinephrine and a referral to an allergist. Because certain sting reactions warrant skin testing and possible venom immunotherapy (VIT), a careful history of all sting reactions is important. Interestingly, VIT is not recommended in children younger than 16 years of age who experience reactions limited to urticaria or angioedema; the risk for future reactions in these patients is 5% to 10%, which is similar to individuals experiencing large local reactions.\n\nContent Specifications: Know that immunotherapy with insect venom is 98% effective in preventing subsequent reactions"}
{"id" : 1189, "question_text" : "A 14-year-old adolescent is brought to the emergency department by his parents. He has complained of neck pain for 3 days since riding a roller coaster at a theme park. The day before this visit, his right arm started to tingle and became weak, and this morning, his left arm started to tingle. He has not had urinary or fecal incontinence, changes in vision, or headache. He has not been sick recently, although his sister had an upper respiratory infection 3 weeks ago. He does not recall any specific head or neck trauma. On physical examination, the patient's temperature is 36.8°C, blood pressure is 102/74 mm Hg, and his heart rate is 92 beats/min. He has no rashes and no tenderness to palpation of his vertebral spinous processes. There is a palpable mass in his lower abdomen that you suspect is a distended bladder. His neurologic examination demonstrates flaccid paralysis of his right upper extremity. His left upper extremity has only antigravity strength and he is unable to provide any resistance. Reflexes are diminished in his biceps and triceps, with normal patellar reflexes. His toes are flexor on plantar stimulation. His mental status, cranial nerves, sensation, coordination, and gait are normal. Of the following, the test MOST likely to yield the correct diagnosis is", "options" : "[\"computed tomography angiography of the neck\", \"electromyography and nerve conduction study\", \"lumbar puncture\", \"magnetic resonance imaging of the brain\", \"magnetic resonance imaging of the cervical spine\"]", "explanation" : "The boy in the vignette has had rapid onset of bilateral arm weakness, sensory symptoms, and urinary retention, suggesting an acute process involving the cervical spinal cord. Magnetic resonance imaging of the cervical spinal cord is the diagnostic test of choice, as transverse myelitis is the most likely diagnosis. Transverse myelitis is an acute onset, immune-mediated demyelination of the spinal cord, typically involving contiguous vertebral levels in the cervical and thoracic spinal cord. The most common presenting symptoms and signs are pain, weakness, and sensory changes at the level of the lesion. Sometimes sensory symptoms precede sensory deficits. The differential diagnosis of an acute process of the cervical spine includes a compressive mass lesion such as tumor or abscess, an expanding spinal cord syrinx, direct spinal cord bacterial or viral infection, or acute ischemia.\nFor the boy in the vignette, who does not have signs or symptoms of active infection, the best test to evaluate his symptoms is magnetic resonance imaging (MRI) of the cervical spinal cord. MRI of the brain would not identify a lesion in the cervical spinal cord. Computed tomography angiography of the neck would show abnormalities of the arteries but not the spinal cord itself. Electromyography and nerve conduction studies test the muscle, nerve, and neuromuscular junction, which would not be helpful in this case. Lumbar puncture would likely be performed once the MRI of the cervical spinal cord shows transverse myelitis, but it is not the best test to diagnose an acute cervical spinal cord lesion.\nPREP Pearls\n• Sudden onset of bilateral arm weakness with sensory loss is suggestive of a cervical spine lesion.\n• Magnetic resonance imaging of the spinal cord is the diagnostic test of choice when there is suspicion of an acute, nontraumatic spinal cord lesion.\nABP Content Specifications(s)\n• Plan the appropriate diagnostic evaluation of suspected transverse myelitis\n• Recognize the clinical manifestations of transverse myelitis"}
{"id" : 2445, "question_text" : "A fully immunized 4-year-old child is seen in the office because of bilateral ear pain that has lasted for 3 days. On physical examination, he appears well. His vital signs are a temperature of 39 °C, a heart rate of 90 beats/min, a respiratory rate of 20 breaths/min, and an oxygen saturation of 100% in room air. The conjunctiva of both eyes are injected and have purulent discharge, and both tympanic membranes are erythematous, opacified, and bulging. The remainder of the child's physical examination findings are normal. Of the following, the mechanism of action of the MOST appropriate antibiotic to treat this child's infection is", "options" : "[\"inhibition of protein synthesis by binding to the 30 S ribosomal subunit\", \"inhibition of protein synthesis by binding to the 50 S ribosomal subunit\", \"inhibition of the transpeptidase enzyme with a \\u03b2-lactamase inhibitor\", \"inhibition of the transpeptidase enzyme without a \\u03b2-lactamase inhibitor\"]", "explanation" : "PREP Pearl(s)\nBeta-lactam antibiotics include penicillins, cephalosporins, carbapenems, and monobactam.\nBeta-lactam antibiotics inhibit cell wall synthesis by binding to penicillin-binding protein, a transpeptidase enzyme; transpeptidation is an essential step in cross-linking peptides to form peptidoglycan, which provides stability to the bacterial cell wall. Inhibiting the transpeptidase enzyme results in autolysis of the bacterial cell wall.\nWhen treating a child with an oral antibiotic for a suspected Haemophilus influenzae infection with unknown susceptibility, the recommended first-line antibiotic is amoxicillin-clavulanic acid.\nCritique\nThe child in the vignette has bilateral acute otitis media and purulent conjunctivitis. His fever (temperature ≥39 °C) and otalgia of greater than or equal to 48 hours suggest severe disease. With this constellation of signs and symptoms, Haemophilus influenzae is the most likely causative organism. About 30% of H influenzae species produce a β-lactamase enzyme. The most appropriate antibiotic to treat this child's infection is a β-lactam agent combined with a β-lactamase inhibitor (eg, amoxicillin–clavulanic acid).\nBeta-lactam antibiotics include penicillins (eg, amoxicillin), cephalosporins, carbapenems, and monobactam (Table). The common structural feature shared by all β-lactam antibiotics is a nitro-containing 4-membered ring (1 nitro, 3 carbons). Beta-lactam antibiotics work by inhibiting bacterial cell wall synthesis. The bacterial cell wall provides structural stability. Gram-positive bacteria cell walls have a thick peptidoglycan component (≥10 layers), whereas gram-negative bacteria have a thin peptidoglycan component (2-3 layers).\nBeta-lactam antibiotics target penicillin-binding protein (PBP), a transpeptidase enzyme. Transpeptidase is responsible for the last step in peptidoglycan synthesis, the cross-linking of peptides to form peptidoglycan. Beta-lactam antibiotics interrupt this terminal transpeptidation process by acylating the PBP transpeptidase, resulting in bacterial cell death by cell lysis.\nHigh-dose amoxicillin, a β-lactam antibiotic, is the recommended initial therapy to treat otitis media caused by non-β-lactamase–producing Streptococcus pneumoniae or H influenzae species. However, a quarter of H influenzae species make β-lactamases, rendering amoxicillin therapy alone ineffective. Clavulanic acid, a β-lactamase inhibitor, binds and inhibits β-lactamases, allowing concurrently administered amoxicillin to be active against H influenzae. Therefore, when treating a child with an oral antibiotic for a suspected H influenzae infection with unknown susceptibility, the recommended first-line antibiotic is amoxicillin-clavulanic acid.\nAntibiotic classes inhibiting RNA-dependent bacterial protein synthesis by binding to the 50 S ribosomal subunit include macrolides (eg, azithromycin), clindamycin, and linezolid. Antibiotic classes inhibiting RNA-dependent bacterial protein synthesis by binding to the 30 ribosomal subunit include aminoglycosides (eg, gentamicin) and tetracyclines (eg, doxycycline).\nSuggested Reading(s)\nAmerican Academy of Pediatrics. Principles of appropriate use of antimicrobial therapy for upper respiratory tract infections. In: Kimberlin DW, Barnett ED, Lyfield R, Sawyer MH, eds. Red Book: 2021-2024 Report of the Committee on Infectious Diseases. 32nd ed. American Academy of Pediatrics; 2021. Accessed September 1, 2023. Red Book Online\nContent Domain\nPharmacology\nABP Content Specification(s) / Content Area(s)\nUnderstand the mechanism of action of beta-lactam antibiotics"}
{"id" : 3789, "question_text" : "A 15-year-old previously healthy adolescent girl is brought to the emergency department with a 1-day history of intermittent right flank pain and visible blood in the urine. Her pain is moderate intensity and radiates to the front of the abdomen and pelvis. She has bright red urine with small clots. She has no fever or burning with urination but has urgency to void. She has a heart rate of 104 beats/min, respiratory rate of 14 breaths/min, and blood pressure of 100/60 mm Hg. She has no guarding or rebound tenderness during abdominal examination. The rest of the physical examination findings are unremarkable. Results of a spot urinalysis with microscopy are shown: Laboratory test - pH: 7.0, Specific gravity: 1.020, Leukocyte esterase: Positive, Nitrite: Negative, Blood: 3+, Protein: 1+, Red blood cells: > 100/HPF, White blood cells: > 5-10/HPF. Of the following, the MOST likely diagnosis in this patient is", "options" : "[\"acute glomerulonephritis\", \"hemorrhagic cystitis\", \"nephrolithiasis\", \"urinary tract infection\"]", "explanation" : "Correct Answer: C\nThe patient in this vignette has right flank pain radiating to the suprapubic region, gross hematuria with clots, and a urinalysis showing minimal pyuria; these findings favor a diagnosis of an acute episode of nephrolithiasis or renal stone.\n\nNephrolithiasis is increasingly seen in children. The majority of children in the adolescent age group present with the classic symptom of flank pain or renal colic. Older children, like adults, have ureteral stones which can cause obstruction and present with renal colic as the stone tries to pass through the ureterovesical junction. However, children younger than 5 years may be asymptomatic; in this case, nephrolithiasis is detected when abdominal imaging is done for other clinical indications. Younger children may have stones within the kidney and can present with recurrent abdominal pain. The other presenting features of nephrolithiasis include gross hematuria, dysuria, urinary urgency, and sometimes urinary tract infection.\n\nThe evaluation of a child with nephrolithiasis includes a detailed history of urinary tract anomalies (obstruction, renal cyst), recurrent urinary tract infection (Proteus, Klebsiella), metabolic conditions (malabsorption syndrome, ketogenic diet), medications (sulfadiazine, indinavir), and family history. Physical examination includes measurement of blood pressure and growth parameters and abdominal examination to look for a mass suggestive of urinary obstruction or for other causes of abdominal pain. The initial laboratory studies are urinalysis with microscopic examination for the presence of crystals, urine culture to rule out urinary tract infection, and renal function tests.\n\nThe adolescent girl in this vignette has renal colic and favors a diagnosis of nephrolithiasis. Acute glomerulonephritis usually presents with gross hematuria without clots, proteinuria, hypertension, and azotemia. In acute glomerulonephritis, hematuria originates from the glomerulus and usually presents with cola- or tea-colored urine. Urine microscopy may show red blood cell casts that are diagnostic of acute glomerulonephritis. However, in nephrolithiasis, hematuria is from the collecting system, and the urine is bright red and has clots. Acute hemorrhagic cystitis presents with suprapubic pain or may be painless and is associated with bright red urine. The patient in this vignette has a history of flank pain, however acute urinary tract infection (pyelonephritis) is unlikely because there is no history of fever or significant pyuria on urinalysis.\n\nThe diagnosis of nephrolithiasis is confirmed by abdominal imaging. Renal ultrasonography is the initial test, but it may not detect small stones or ureteral stones. A plain abdominal radiograph helps in detecting radiopaque stones. Noncontrast helical computed tomography is the most sensitive test for detection of nephrolithiasis in children.\n\nThe acute management of nephrolithiasis includes pain control, aggressive fluid administration, and facilitating passage or removal of stone. The long-term management includes evaluation of the type of nephrolithiasis and the risk factors for stone recurrence. Accordingly, dietary interventions and medications are recommended to prevent recurrent nephrolithiasis.\n\nPREP Pearls\n• Young children with nephrolithiasis may be asymptomatic or have recurrent abdominal pain.\n• Evaluation of nephrolithiasis includes history of urinary tract abnormalities, recurrent urinary tract infections, metabolic conditions, medications, and family history\n• Noncontrast computed tomography is the most sensitive imaging modality for confirming nephrolithiasis.\n\nABP Content Specifications(s)\n• Plan the evaluation of urinary tract stones in patients of various ages\n• Recognize the signs and symptoms of urinary tract stones in patients of various ages\n\nSuggested Readings\n• Gellin CE. Urinary tract stones. Pediatr Rev. 2019;40(3):154-156. doi:10.1542/pir.2017-0235.\n• McKay CP. Renal stone disease. Pediatr Rev. 2010;31(5):179-188. doi:10.1542/pir.31-5-179.\n• Tayaba M, Kamat D. Pediatric nephrolithiasis: a review. Pediatr Ann. 2017;46(6):e242-e244. doi:10.3928/19382359-20170517-02."}
{"id" : 1120, "question_text" : "A 15-year-old adolescent collapses while playing in a high school basketball game without any preceding trauma. He is unconscious and not breathing. Of the following, the BEST sequence of actions is to", "options" : "[\"administer a precordial thump and perform a jaw-thrust maneuver\", \"ask someone to retrieve an automatic external defibrillator and start chest compressions\", \"immobilize the cervical spine and attempt to palpate the carotid pulse\", \"perform a chin-lift maneuver and start rescue breathing\", \"perform rapid assessment of circulation/airway/breathing and call 911\"]", "explanation" : "The boy in the vignette most likely has suffered a sudden cardiac arrest. A less severe cardiovascular condition with a perfusing rhythm is not as likely because he is not breathing. Although a neurologic catastrophe (eg, trauma or spontaneous hemorrhage of a cerebrovascular malformation) should be considered as a possible cause of this event, it is significantly less likely without a supporting history. The best response choice is to ask someone to retrieve an automatic external defibrillator (AED) and start chest compressions.\nAppropriate life support responses for children include the algorithms of basic life support, in which it is assumed that there is only 1 responder, and pediatric advanced life support, which takes place in an environment in which many rescuers are involved and actions can be undertaken simultaneously. Regardless of the environment, etiology, or age of the patient in cardiac arrest, the effectiveness of life support efforts depends on the quality of the cardiopulmonary resuscitation (CPR). Chest compressions and rescue breathing should be immediately started. For CPR in children it is recommended to push hard (greater than 1/3 of the anterior-posterior diameter of the chest), push fast (at least 100 compressions per minute), minimize interruptions between compressions, and rotate the person giving compressions every 2 minutes. If no advanced airway, ie, an endotracheal tube or laryngeal mask airway, is present, a 15:2 compression-ventilation ratio should be followed. If an advanced airway is in place, 8 to 10 breaths per minute should be given with continuous chest compressions. CPR recommendations for infants also include a 15:2 compression-ventilation ratio, however, newborns with cardiac arrest of cardiac origin in the delivery room or neonatal intensive care unit should receive a 3:1 compression-ventilation ratio.\nBecause the boy in the vignette presented with collapse and apnea, he is unlikely to have a perfusing rhythm. Palpation for a pulse in this setting may not be accurate, and could lead to a delay in definitive care. Even rapid assessment of circulation/airway/breathing is not necessary given the obvious gravity of the child's status and could lead to further delays in treatment. The cause of his collapse is likely cardiac, therefore jaw-thrust or chin-lift maneuvers would not be helpful. Although rescue breathing should be performed, it is not the best next step, because the therapy most likely to restore spontaneous circulation is cardioversion or defibrillation using an AED. The precordial thump is no longer recommended in the latest American Heart Association guidelines. In the absence of trauma, immobilization of the cervical spine is not recommended. Although 911 should be called, it is likely that an AED is available in the high school gymnasium and may be effective before the arrival of emergency medical services.\nPREP Pearls\n Effective cardiopulmonary resuscitation includes pushing hard (to 1/3 of the anterior-posterior diameter of the chest), pushing fast (100 compressions per minute) with minimal interruptions, and rotating personnel performing compressions every 2 minutes.\n The first steps for a child with a sudden cardiac arrest are to call for an automatic external defibrillator (AED) and to start chest compressions immediately, especially in a public place where an AED is likely to be available.\nABP Content Specifications(s)\n Understand the correct method for cardiopulmonary resuscitation in patients of various ages"}
{"id" : 2274, "question_text" : "A 5-year-old girl is seen in the office for follow-up 4 days after being treated in the emergency department for a lower extremity purpuric rash and mild arthralgia. She was diagnosed with IgA vasculitis (formerly Henoch-Schönlein purpura). Her urinalysis in the emergency department was negative for blood and protein. Conservative management with over-the-counter nonsteroidal anti-inflammatory drugs was recommended. Her joint pain has worsened since then, and she is experiencing increased difficulty with ambulation and activities of daily living. Two days ago, she developed mild abdominal pain without associated hematochezia. Physical examination reveals multiple scattered palpable purpura on the lower extremities. Her abdomen is diffusely tender without palpable masses. There is joint effusion and warmth in the ankles bilaterally, and a moderate effusion of the right knee with decreased flexion. She has an antalgic gait. A tapering course of oral prednisolone is initiated. Of the following, the MOST accurate statement regarding administration of this medication for the girl's condition is", "options" : "[\"it can reduce the severity of the joint pain\", \"it can prevent long-term complications associated with IgA vasculitis\", \"it prevents the development of renal disease\", \"it can prolong the duration of abdominal pain\"]", "explanation" : "The administration of oral corticosteroids to children with IgA vasculitis (formerly Henoch-Schönlein purpura) may reduce the intensity and duration of clinical symptoms, including joint and abdominal pain. IgA vasculitis, a prevalent childhood vasculitis, typically presents with palpable purpura, arthritis/arthralgia, and abdominal pain; one-third of those affected will experience glomerulonephritis. The 2010 European League Against Rheumatism/Paediatric Rheumatology International Trials Organisation/Paediatric Rheumatology European Society (EULAR/PRINTO/PRES) diagnostic guidelines for IgA vasculitis, outlined below, were found to have 100% sensitivity and 87% specificity in making a diagnosis of IgA vasculitis.\n\nMandatory Criterion\nPurpura or petechiae\n\nAdditional Criteria (at least 1 of the following must be present)\nAbdominal pain\nArthritis or arthralgia\nRenal involvement\nLeukocytoclastic vasculitis with predominant IgA deposits or proliferative glomerulonephritis with predominant IgA deposits\n\nIn most cases, IgA vasculitis resolves spontaneously within 4 weeks of onset. Some children may experience mild recurrences. Treatment is primarily focused on symptomatic relief. IgA vasculitis is frequently managed in an outpatient setting. However, hospital admission is recommended for patients exhibiting any of the following conditions:\nAltered mental status\nLethargy\nBlood pressure abnormalities\nSevere abdominal pain (indicative of small bowel intussusception or obstruction)\nGastrointestinal bleeding\nSignificant renal involvement (characterized by abnormal kidney function or urine output)\n\nThe initial management approach to IgA vasculitis involves reassurance and supportive care, including rest, hydration, and monitoring for renal manifestations. Nonsteroidal anti-inflammatory drugs are recommended for children with normal renal function to manage joint and abdominal pain; the presence of purpura is not a contraindication.\n\nWhile corticosteroid therapy may alter disease progression in mild to severe IgA vasculitis nephritis, it has no proven prophylactic benefit. Administration of corticosteroids has not demonstrated a clear benefit in the long-term outcomes associated with extrarenal manifestations in IgA vasculitis and should not be routinely administered. For children with severe gastrointestinal or joint symptoms, corticosteroid therapy may significantly reduce the intensity and duration. When indicated, the corticosteroid treatment regimen can vary; general recommendations are to administer 1-2 mg/kg for 1 to 2 weeks followed by a 2-week taper. Corticosteroid therapy should be strongly considered in cases of IgA vasculitis associated with pulmonary hemorrhage, cerebral vasculitis, or orchitis. Children with recurrent flares of IgA vasculitis may benefit from corticosteroids or steroid-sparing medications (eg, cyclosporine, methotrexate, azathioprine, mycophenolate, intravenous Ig).\n\nSuggested Reading(s)\nAdrogue HE, Hayde NA. Henoch-Schönlein purpura. In: McInerny TK, Adam HM, Campbell DE, DeWitt TG, Foy JM, Kamat DM, eds. American Academy of Pediatrics Textbook of Pediatric Care. American Academy of Pediatrics; 2021:chap 264. Accessed September 21, 2024. Pediatric Care Online\nKamat DM. Henoch-Schönlein purpura. Point-of-care Quick Reference. American Academy of Pediatrics. December 8, 2020. Accessed September 1, 2024. Pediatric Care Online\nLeung AKC, Barankin B, Leong KF. Henoch-Schönlein purpura in children: an updated review. Curr Pediatr Rev. 2020;16(4):265-276. doi:10.2174/1573396316666200508104708\nReid-Adam J. Henoch-Schönlein purpura. Pediatr Rev. 2014;35(10):447-449. doi:10.1542/pir.35-10-447\n\nContent Domain\nRheumatology, Vasculitis\n\nLearning Objectives\nReduce symptoms associated with IgA vasculitis"}
{"id" : 2762, "question_text" : "A 5-year-old boy is brought to the emergency department with a nosebleed. On arrival, he has active bleeding from the right naris. Pressure is applied for 10 minutes, and the bleeding resolves. The boy's mother reports that he has frequent nosebleeds. They occur at least once per month and usually resolve with applied pressure. He does pick his nose frequently, and his mother is concerned about the number of nosebleeds he is experiencing. There is no family history of bleeding disorders. The boy has a temperature of 37.4°C, heart rate of 84 beats/min, respiratory rate of 20 breaths/min, blood pressure of 108/70 mm Hg, and oxygen saturation of 99% in room air. His physical examination findings are normal except for dried blood in the anterior portion of the right naris. Of the following, the BEST next step in this boy's management is to", "options" : "[\"obtain a complete blood count and coagulation studies\", \"perform nasal cautery\", \"provide reassurance and recommend conservative measures\", \"refer to an otolaryngologist\"]", "explanation" : "Correct Answer: C\nThe boy in the vignette experienced a nosebleed that resolved after a brief period of applying pressure. The best next management step is to provide reassurance and discuss conservative measures that include applying pressure in the appropriate location for 5 to 10 minutes without interruption, frequent moisturizing of the nasal mucosa with nasal saline or ointment, humidifying the air in the home, and discouraging nose picking.\n\nNasal bleeding is common in children and frequently causes parental anxiety. The boy in the vignette has normal vital signs, and the nasal bleeding was easily controlled with appropriate pressure. No additional acute management is required. Nasal packing and nasal cautery should be considered when bleeding continues despite attempts to control bleeding with appropriate pressure. Routine laboratory evaluation (eg, complete blood count, coagulation studies) is not necessary unless the child is experiencing frequent or prolonged nasal bleeding or has skin examination findings concerning for a bleeding disorder (eg, unusual ecchymosis, petechiae). The boy in the vignette does not have abnormal skin findings or family history of a bleeding disorder, and he is experiencing infrequent nosebleeds. Referral to an otolaryngologist is generally not required unless a nosebleed cannot be stopped or there are recurrent nosebleeds requiring frequent trips to the pediatrician's office or emergency department after appropriate attempts to stop them at home.\n\nThe etiologies of epistaxis vary; a thorough history and physical examination should be performed to identify the cause. The differential diagnosis of epistaxis includes trauma, inflammation, dry air, neoplasms, intranasal drugs, structural abnormalities, and bleeding disorders.\n\nNosebleeds are divided into 2 categories, anterior and posterior, based on the location of the bleeding. Anterior nosebleeds are more common and are much easier to visualize and control. The bleeding most commonly occurs at the Kiesselbach plexus (Kiesselbach triangle), a vascular plexus located at the anteroinferior aspect of the nasal septum. The acute management of nasal bleeding includes pressure application by squeezing the nasal alae against the nasal septum and gentle elevation of the child's head. If bleeding continues, application of a topical decongestant or topical vasoconstrictor (eg, oxymetazoline or epinephrine), or nasal cauterization of the bleeding locus may be necessary. If these measures are not effective, nasal packing should be placed, and immediate evaluation by an otolaryngologist is warranted.\n\nPREP Pearls\n• Nosebleeds are common in children and most can be treated with conservative management such as applying pressure in the appropriate location for 5 to 10 minutes without interruption, moisturizing the nasal mucosa with nasal saline or ointment, providing humidified air in the home, and discouraging nose picking.\n• The differential diagnosis of epistaxis includes trauma, inflammation, dry air, neoplasms, intranasal drugs, structural abnormalities, and bleeding disorders.\n• Nosebleeds are divided into 2 categories, anterior and posterior, based on the location of the bleeding. Anterior nosebleeds are the most common type and are much easier to visualize and control than posterior nasal bleeds.\n\nABP Content Specifications(s)\n• Formulate a differential diagnosis of epistaxis and manage appropriately\n• Plan the appropriate evaluation of the various manifestations of epistaxis\n\nSuggested Readings\n• Mulbury PE. Recurrent epistaxis. Pediatr Rev. 1991;12(7):213-217. doi:10.1542/pir.12-7-213.\n• Schechter MB, Stevens DM. Epistaxis. In: McInerny TK, Adam HM, Campbell DE, DeWitt TG, Foy JM, Kamat DM, eds. American Academy of Pediatrics Textbook of Pediatric Care. American Academy of Pediatrics; 2021:chap 146. Accessed September 1, 2022. Pediatric Care Online.\n• Tunkel DE, Anne S, Payne SC, et al. Clinical practice guideline: nosebleed (epistaxis). Otolaryngol Head Neck Surg. 2020;162(1_suppl):S1-S38. doi:10.1177/0194599819890327."}
{"id" : 2958, "question_text" : "An 8-year-old girl is seen in the office for concerns about her academic performance. Her teachers note that she tends to daydream and is struggling to stay focused on her work; at times they find it difficult to get her attention. Her parents report that sometimes they must ask her a question 2 or 3 times before receiving a response. She is an otherwise healthy child and when engaged in a task she is able to complete it. The girl reports that she finds herself \"losing time\" periodically throughout the day which sometimes makes it hard to stay focused on her activity. In the office, the girl is sitting quietly next to her parents. She intermittently stares, then rapidly returns to the conversation. Her general physical examination findings are unremarkable. Neurologic findings are normal, though during the examination, she has an episode of staring, during which she is not responsive to direction for a few seconds.\n\nOf the following, the BEST next management step for this girl is", "options" : "[\"counseling\", \"electroencephalography\", \"magnetic resonance imaging of the brain\", \"neuropsychological evaluation\"]", "explanation" : "The clinical presentation of the girl in the vignette is most consistent with absence seizures. Electroencephalography (EEG) is the best next step in the diagnostic workup for this girl's staring episodes. Childhood absence epilepsy is a common childhood epilepsy syndrome presenting between the ages of 4 and 10 years. An absence seizure is a brief lapse in consciousness, clinically appearing as a staring episode, lasting seconds, and occurring up to hundreds of times in a day. Automatisms can be present and may include eye blinking, eyelid fluttering, lip smacking, or hand or face twitching. No postictal state occurs. One-third of patients develop generalized tonic-clonic seizures as part of the absence epilepsy, which may affect antiepileptic medication choice. Because the clinical presentation of absence seizures can be subtle, they can be mistaken for inattention or daydreaming; their frequency is often underestimated.\n\nThe diagnosis of absence seizures is confirmed with EEG, which will demonstrate interictal 3-Hz generalized spike and wave discharges. Seizures can be provoked in the office setting and during EEG recording with hyperventilation maneuvers. Recommended antiepileptic medication choices include ethosuximide, valproic acid, and lamotrigine. Ethosuximide is considered the first-line treatment option for absence seizures, because of its favorable side effect profile and efficacy; however, it does not provide adequate treatment for children who also have generalized tonic-clonic seizures. The prognosis for children with absence seizures is favorable, with most children outgrowing their epilepsy in adolescence.\n\nFindings on magnetic resonance imaging of the brain are generally normal in children with childhood absence epilepsy. Counseling may be beneficial in children with epilepsy who have comorbid anxiety or mood disorders, which are more common in the patient population with epilepsy. Because children with absence epilepsy have a higher rate of attention-deficit/hyperactivity disorder as well as behavioral and cognitive disorders than the general population, neuropsychological evaluation may be helpful to evaluate for these common comorbidities, but would not be the best next step in this child's evaluation.\n\nPREP Pearls\n• An absence seizure is a brief lapse in consciousness, clinically appearing as a staring episode, which can have associated automatisms or generalized tonic-clonic seizures.\n• Ethosuximide is the first-line antiepileptic medication of choice for absence seizures. Alternative options include valproic acid or lamotrigine.\n• Absence seizures are commonly mistaken for inattention or daydreaming. The presence of interictal 3-Hz spikes and slow-wave generalized discharges on electroencephalography can confirm the diagnosis.\n\nABP Content Specifications(s)\n• Recognize the clinical findings associated with absence seizures, and manage appropriately\n\nSuggested Readings\n• Glauser TA, Cnaan A, Shinnar S, et al. Ethosuximide, valproic acid and lamotrigine in childhood absence epilepsy. N Engl J Med. 2010;362:790-799. doi: 10.1056/NEJMoa0902014.\n• Park JT, Shahid AM, Jammoul A. Common pediatric epilepsy syndromes. Pediatr Ann. 2015;44(2):e30-e35. doi: 10.3928/00904481-20150203-09.\n• Roddy SM, McBride M. Seizure disorders. In: McInerny TK, Adam HM, Campbell DE, DeWitt TG, Foy JM, Kamat DM, eds. American Academy of Pediatrics Textbook of Pediatric Care. 2nd ed. Itasca, IL: American Academy of Pediatrics; 2016;chap 327:2599-2616. Pediatric Care Online.\n• Sidhu R, Velayudam K, Barnes G. Pediatric seizures. Pediatr Rev. 2013;34(8):333-342. doi: 10.1542/pir.34-8-333."}
{"id" : 159, "question_text" : "A 14-year-old previously well boy presents to your office with a 10-day history of cough and posttussive emesis. His grandmother also has a coughing illness. His 3-week-old sibling, with whom he lives, is well. On physical examination, the boy appears generally well, with a temperature of 37.0°C, heart rate of 80 beats/min, and respiratory rate of 16 breaths/min. He has mild nasal congestion, and his lungs are clear to auscultation. When you use a tongue depressor to examine his posterior oropharynx, he begins coughing and cannot stop. This cough paroxysm lasts about 30 seconds and is followed by gasping and vomiting. Of the following, the MOST appropriate intervention for the patient's 3-week-old healthy sibling is", "options" : "[\"azithromycin for 5 days\", \"clarithromycin for 7 days\", \"erythromycin for 14 days\", \"penicillin for 10 days\", \"trimethoprim-sulfamethoxazole for 14 days\"]", "explanation" : "The boy described in the vignette has a paroxysmal cough, gasping, and posttussive emesis that strongly suggest infection caused by the gram-negative bacterium Bordetella pertussis. Pertussis is a highly contagious respiratory disease, with an attack rate of approximately 90% for household contacts. Unvaccinated infants younger than 1 year of age, such as the 3-week-old in the vignette, have the highest risk of severe and life-threatening complications.\n\nPostexposure chemoprophylaxis is recommended for all household contacts, regardless of their age or immunization status. If administered promptly (within 21 days of onset of cough in index case), chemoprophylaxis may prevent secondary transmission of pertussis. For infants younger than 1 month of age, azithromycin is the preferred drug for chemoprophylaxis and therapy.\n\nAzithromycin is a macrolide antibiotic that inhibits protein synthesis by binding to the 50S subunit of the bacterial ribosome. Macrolide antibiotics (erythromycin, clarithromycin, azithromycin, and telithromycin) are inhibitory to bacteria. They are most active against gram-positive cocci and bacilli but also are active against gram-negative bacilli. Macrolide antibiotics, especially azithromycin, achieve high intracellular concentrations and, therefore, are active against susceptible intracellular pathogens. Because azithromycin provides prolonged tissue concentrations of drug at the site of infection, it can be administered for a short period of time.\n\nAzithromycin is the drug of choice for pertussis treatment and chemoprophylaxis for infants younger than 1 month of age because of the association between orally administered erythromycin and the development of infantile hypertrophic pyloric stenosis. It also is considered appropriate therapy for treatment or chemoprophylaxis in older children and adults. It is much better tolerated than erythromycin and can be dosed once daily.\n\nAzithromycin is approved for the treatment of community-acquired pneumonia, sinusitis, and acute otitis media in children, although increasingly widespread resistance of Streptococcus pneumoniae to the macrolides may limit its use. Children who have severe penicillin allergy may receive azithromycin for the treatment of streptococcal pharyngitis. Azithromycin is active against organisms that cause atypical pneumonia, such as Chlamydia, Mycoplasma, and Legionella. It also can be used to treat sexually transmitted infections such as those caused by Chlamydia trachomatis and Neisseria gonorrhoeae. Azithromycin is moderately active against Haemophilus influenzae and Moraxella catarrhalis. Compared to the other macrolides, azithromycin has better activity against gastrointestinal pathogens such as Salmonella, Shigella, Campylobacter, and Escherichia coli. It is the only antibiotic that has been prospectively evaluated for the treatment of lymphadenitis caused by Bartonella henselae.\n\nClarithromycin, like azithromycin, is better tolerated than erythromycin. It can be used to treat pertussis but is not recommended for use in children younger than 1 month of age. In addition to the previously cited indications for azithromycin, clarithromycin has an important role in the prevention and treatment of disseminated Mycobacterium avium-intracellulare complex (MAC) infections in patients who have human immunodeficiency virus infection. It also can be used for the treatment of pneumonia and cervical adenitis due to MAC. In addition, clarithromycin, in combination with amoxicillin and a proton pump inhibitor, is used for the treatment of Helicobacter pylori infections. Although clarithromycin is approved for the treatment of skin and skin structure infections, other drugs that are more active against Staphylococcus aureus are available.\n\nTrimethoprim-sulfamethoxazole also is an appropriate drug for pertussis treatment and chemoprophylaxis but is contraindicated in children younger than 2 months of age. Penicillin has no activity against B pertussis.\n\nCritique: The boy described in the vignette has a paroxysmal cough, gasping, and posttussive emesis that strongly suggest infection caused by the gram-negative bacterium Bordetella pertussis. Pertussis is a highly contagious respiratory disease, with an attack rate of approximately 90% for household contacts. Unvaccinated infants younger than 1 year of age, such as the 3-week-old in the vignette, have the highest risk of severe and life-threatening complications.\n\nPostexposure chemoprophylaxis is recommended for all household contacts, regardless of their age or immunization status. If administered promptly (within 21 days of onset of cough in index case), chemoprophylaxis may prevent secondary transmission of pertussis. For infants younger than 1 month of age, azithromycin is the preferred drug for chemoprophylaxis and therapy.\n\nAzithromycin is a macrolide antibiotic that inhibits protein synthesis by binding to the 50S subunit of the bacterial ribosome. Macrolide antibiotics (erythromycin, clarithromycin, azithromycin, and telithromycin) are inhibitory to bacteria. They are most active against gram-positive cocci and bacilli but also are active against gram-negative bacilli. Macrolide antibiotics, especially azithromycin, achieve high intracellular concentrations and, therefore, are active against susceptible intracellular pathogens. Because azithromycin provides prolonged tissue concentrations of drug at the site of infection, it can be administered for a short period of time.\n\nAzithromycin is the drug of choice for pertussis treatment and chemoprophylaxis for infants younger than 1 month of age because of the association between orally administered erythromycin and the development of infantile hypertrophic pyloric stenosis. It also is considered appropriate therapy for treatment or chemoprophylaxis in older children and adults. It is much better tolerated than erythromycin and can be dosed once daily.\n\nAzithromycin is approved for the treatment of community-acquired pneumonia, sinusitis, and acute otitis media in children, although increasingly widespread resistance of Streptococcus pneumoniae to the macrolides may limit its use. Children who have severe penicillin allergy may receive azithromycin for the treatment of streptococcal pharyngitis. Azithromycin is active against organisms that cause atypical pneumonia, such as Chlamydia, Mycoplasma, and Legionella. It also can be used to treat sexually transmitted infections such as those caused by Chlamydia trachomatis and Neisseria gonorrhoeae. Azithromycin is moderately active against Haemophilus influenzae and Moraxella catarrhalis. Compared to the other macrolides, azithromycin has better activity against gastrointestinal pathogens such as Salmonella, Shigella, Campylobacter, and Escherichia coli. It is the only antibiotic that has been prospectively evaluated for the treatment of lymphadenitis caused by Bartonella henselae.\n\nClarithromycin, like azithromycin, is better tolerated than erythromycin. It can be used to treat pertussis but is not recommended for use in children younger than 1 month of age. In addition to the previously cited indications for azithromycin, clarithromycin has an important role in the prevention and treatment of disseminated Mycobacterium avium-intracellulare complex (MAC) infections in patients who have human immunodeficiency virus infection. It also can be used for the treatment of pneumonia and cervical adenitis due to MAC. In addition, clarithromycin, in combination with amoxicillin and a proton pump inhibitor, is used for the treatment of Helicobacter pylori infections. Although clarithromycin is approved for the treatment of skin and skin structure infections, other drugs that are more active against Staphylococcus aureus are available.\n\nTrimethoprim-sulfamethoxazole also is an appropriate drug for pertussis treatment and chemoprophylaxis but is contraindicated in children younger than 2 months of age. Penicillin has no activity against B pertussis.\n\nContent Specifications: Know the appropriate use of clarithromycin and azithromycin"}
{"id" : 1478, "question_text" : "The parents of a 9-month-old infant ask your opinion about a \"spot\" that appeared on their daughter's arm several weeks ago. They have observed that when the lesion is rubbed (as when drying the skin after bathing) it may become red and swollen. The infant is well in all respects. Her temperature is 37°C and other vital signs are normal. The physical examination is notable only for an orange-pink, 2-cm oval plaque on the left arm (Item Q229A). After rubbing the lesion, it becomes erythematous (Item Q229B). Of the following, the MOST likely diagnosis is a", "options" : "[\"caf\\u00e9-au-lait macule\", \"connective tissue nevus\", \"mastocytoma\", \"melanocytic nevus\", \"nummular eczema\"]", "explanation" : "The infant in the vignette has developed a lesion with an orange-peel (peau d'orange) appearance, a finding that indicates the presence of a dermal cellular infiltrate. After rubbing or stroking, the lesion becomes erythematous, often accompanied by swelling (Darier sign). These changes are the result of mast cell degranulation and the release of mediators that cause increased blood flow and fluid leak from vessels. The appearance of the lesion and the presence of a Darier sign indicate a diagnosis of mastocytosis, a group of disorders characterized by the accumulation of mast cells in the skin and, occasionally, other organs. A café-au-lait macule, connective tissue nevus, melanocytic nevus, and nummular eczema would all lack an orange-peel appearance and would not exhibit the Darier sign.\n\nCutaneous mastocytosis is a spectrum of disease that includes mastocytomas, urticaria pigmentosa, diffuse cutaneous mastocytosis, and telangiectasia macularis eruptiva perstans. Mastocytomas and urticaria pigmentosa are the most common forms encountered in pediatrics. Both are characterized by lesions that have an orange-peel appearance and Darier sign. Mastocytosis may be present at birth or develop any time into middle age. For approximately half of patients, onset occurs in the first 2 years of life and 10% develop the disorder between 2 and 15 years of age. In infants and children, mastocytosis typically is limited to the skin, is not associated with hematologic disorders, and tends to resolve spontaneously by adolescence.\n\nMastocytomas (the diagnosis for the infant in the vignette) may be solitary or few in number. They appear as yellow to orange-brown papules or plaques (Item C229A). Lesions range in size from a few millimeters to several centimeters and may be found anywhere on the body. There is often a history of pruritus or recurrent blistering, which is the result of mast cell mediator release. Blistering may lead to confusion with disorders like recurrent herpes simplex virus infection or bullous impetigo.\n\nUrticaria pigmentosa (UP) is the most common form of cutaneous mastocytosis. It is characterized by multiple tan to brown macules or thin plaques, ranging in size from a few millimeters to several centimeters (Item C229B). The lesions of UP may be mistaken for café-au-lait macules or melanocytic nevi because of their color. Pruritus and blistering may occur. If there is sufficient mediator release, patients may experience flushing, hypotension, abdominal discomfort, diarrhea, or respiratory distress.\n\nThe diagnosis of mastocytosis is usually made clinically. If uncertainty exists, skin biopsy will demonstrate an accumulation of mast cells in the dermis. For most pediatric patients, no further testing is required. Some advocate measurement of serum tryptase concentration, a mast cell-derived protease that correlates with disease extent. This may be useful if systemic involvement is suspected. If UP is very widespread, screening with a complete blood cell count and blood chemistries is often recommended to screen for bone marrow or other systemic organ involvement.\n\nMost patients with mastocytosis are asymptomatic and require no intervention. For those experiencing pruritus, a second-generation (eg, loratadine, cetirizine) or third-generation (eg, desloratadine, fexofenadine, levocetirizine) H1 antihistamine may be prescribed. Blistering may be prevented with the application of a potent topical corticosteroid. Patients who have flushing or diarrhea may benefit from cromolyn or an H2 antagonist (eg, cimetidine, ranitidine). A premeasured epinephrine pen is indicated for those who experience hypotensive episodes. Parents should be advised that aspirin, nonsteroidal anti-inflammatory agents, and certain anesthetic agents may cause mast cell degranulation. More information and support are available from http://www.mastokids.org/index.html.\n\nPREP Pearls\n• Cutaneous mastocytosis is characterized by orange-brown macules or papules that, upon stroking, develop erythema and swelling (Darier sign).\n• Most patients with cutaneous mastocytosis require no treatment and the lesions will resolve spontaneously.\n\nABP Content Specifications(s)\n• Recognize the clinical features of the various forms of mastocytosis and manage appropriately\n\nSuggested Readings\n• Carter MC, Metcalfe DD, Komarow HD. Mastocytosis. Immunol Allergy Clin North Am. 2014;34(1):181-196. doi: http://dx.doi.org/10.1016/j.iac.2013.09.001.\n• Krowchuk DP, Mancini AJ. Cutaneous mastocytosis. Pediatric Dermatology: A Quick Reference Guide. 2nd ed. Elk Grove Village, IL: American Academy of Pediatrics; 2012:407-412."}
{"id" : 3705, "question_text" : "A 4-month-old female infant born at 36 weeks' gestation is brought to the office for a health supervision visit. Her parents are worried because she has multiple episodes of nonbloody, nonbilious emesis every day, which occur after feeding and are associated with arching and crying. At her 2-month health supervision visit, the family was counseled regarding behavioral modifications for gastroesophageal reflux symptoms, which the parents feel have not helped. She is taking a cow milk–based formula, 4 oz every 3 hours. The infant is developing normally, with no other medical history. She is growing well, and tracking along the 50th percentile for weight, height, and head circumference. Her physical examination findings are unremarkable. Of the following, the BEST next step in management is", "options" : "[\"addition of a xanthan gum product to her formula\", \"empiric treatment with a proton pump inhibitor\", \"trial of extensively hydrolyzed protein formula\", \"trial of soy formula\"]", "explanation" : "The preterm infant in the vignette has gastroesophageal reflux (GER). The next best step in the management of her symptoms would be a trial of extensively hydrolyzed protein formula. Gastroesophageal reflux is an almost universal normal physiologic phenomenon in preterm infants, which may be distinguished from pathologic GER disease, which includes troublesome symptoms or complications. The 2018 clinical report issued by the American Academy of Pediatrics (AAP) provides guidance regarding the diagnosis and management of GER in preterm infants. Elemental or extensively hydrolyzed protein-based formulas (EHPFs) reduce gastrointestinal transit time. Extensively hydrolyzed protein-based formulas frequently reduce GER symptoms in term infants, possibly because of an overlap of the signs and symptoms of GER with those of cow milk protein allergy. Therefore, a trial of EHPF may be reasonable in age-appropriate preterm infants with signs of severe reflux.\n\nThe effectiveness of thickening feedings is controversial and carries potential risks. Commercial agents such as xanthan gum have been linked to late-onset necrotizing enterocolitis; therefore, these agents are not recommended in preterm or former preterm infants in the first year after birth. One trial of starch-thickened preterm formula demonstrated that the total number of GER episodes was unchanged compared with standard formula feeding. Although esophageal acid exposure was decreased, no assessment was made about whether the reduction in acid exposure affected associated symptoms. The AAP recommends that, if formula is thickened with starch, oatmeal should be used rather than rice cereal, because of the recent concerns about arsenic levels in rice. Randomized controlled trials of thickened formulas in term infants with GER demonstrated reduced episodes of regurgitation, but a lack of efficacy in reducing acidic GER. Commercially available formula products that thicken on acidification are nutritionally inappropriate for preterm infants.\n\nBased on the lack of evidence of efficacy and the potential for harm, the AAP recommends that the use of pharmacologic agents, including proton pump inhibitors, should be minimized in preterm infants. Soy formula is nutritionally inappropriate for preterm infants because of the risk of osteopenia, aluminum toxicity, and prevention of adequate absorption of zinc, phosphorus, and iron.\n\nInfant formulas, which are regulated by the US Food and Drug Administration under the \"Infant Formula Act,\" contain 19 to 20 calories per ounce when mixed as instructed. All formulas are designed to provide sufficient amounts of vitamins and micronutrients. The major differences among the various formulas include amounts of protein, sugar, and fat sources.\n\nPREP Pearls\n• Gastroesophageal reflux is a normal, almost-universal physiologic phenomenon in preterm infants.\n• A trial of extensively hydrolyzed protein-based formula may be implemented in age-appropriate preterm infants with signs of severe reflux.\n• The use of pharmacologic agents, including proton pump inhibitors, should be minimized in preterm infants, because of the potential for harm and little evidence for efficacy.\n\nABP Content Specifications(s)\n• Understand the nutritional supplements that can be used to increase caloric density of formulas and their risks\n• Understand the qualitative and quantitative differences between human milk and various infant formulas\n• Understand the differences among categories of formula used for special nutritional support and the indications for their use\n• Know the content of various infant formulas and milk sources, the indications for their use, and possible side effects\n\nSuggested Readings\n• Chandran L. Gelfer P. Breastfeeding: the essential principles. Pediatr Rev. 2006;27(11):409-417. doi:10.1542/pir.27-11-409.\n• Corvaglia L, Aceti A, Mariani E, et al. Lack of efficacy of a starch-thickened preterm formula on gastro-oesophageal reflux in preterm infants: a pilot study. J Matern Fetal Neonatal Med. 2012;25(12):2735-2738. doi:10.3109/14767058.2012.704440.\n• DiMaggio DM, Cox A, Port AF. Updates in infant nutrition. Pediatr Rev. 2017;38(10):449-462. doi:10.1542/pir.2016-0239.\n• Eichenwald EC; Committee on Fetus and Newborn. Diagnosis and management of gastroesophageal reflux in preterm infants. Pediatrics. 2018;142(1):e20181061. doi:10.1542/peds.2018-1061.\n• Martinez JA, Ballew MP. Infant formulas. Pediatr Rev. 2011;32(5):179-189. doi:10.1542/pir.32-5-179."}
{"id" : 1874, "question_text" : "A previously healthy 6-year-old boy with a 4-day history of varicella presents to the emergency department with fever and severe pain with rapidly progressive swelling over his left upper arm. There are no sick contacts, no pets at home, and no recent travel. He is home-schooled and has not received many of his routine childhood vaccinations due to parental concerns about vaccine-related adverse effects. On arrival, the boy is ill-appearing with a temperature of 39.2°C, heart rate of 150 beats/min, respiratory rate of 46 breaths/min, and blood pressure of 73/32 mm Hg. His physical examination findings are remarkable for diffuse swelling and erythema of the left upper extremity. The overlying skin has a mottled appearance with several small bullous lesions. Laboratory data are notable for a white blood cell count of 26,000/µL ([26×109/L], 95% neutrophils), a hemoglobin of 11.1 g/dL (111 g/L), and a platelet count of 80×103/µL (80×109/L). After surgical consultation, he underwent emergency surgical debridement. Of the following, the MOST likely organism isolated from surgical tissue specimens is", "options" : "[\"Clostridium perfringens\", \"Clostridium septicum\", \"group A Streptococcus\", \"methicillin-resistant Staphylococcus aureus\"]", "explanation" : "The ill-appearing, unvaccinated boy in this vignette has a clinical picture suggestive of invasive group A streptococcal (GAS) infection after a varicella infection, and is concerning for necrotizing fasciitis (NF). Although NF can be caused by Staphylococcus aureus, including methicillin-resistant S aureus and other pathogens such as Clostridium perfringensand Clostridium septicum, the association with varicella is a known risk factor for invasive GAS infection. Primary varicella skin lesions can serve as portal of entry for GAS invasion, and accounted for approximately 15% to 30% of such infections before the routine administration of varicella vaccine in children.  Given the significant mortality and morbidity associated with streptococcal NF, emergent surgical exploration for debridement is the best next step in management.\n\nIn the United States, the burden of invasive GAS infections is significant. The Centers for Disease Control and Prevention identified 9,557 cases (3.8 cases per 100,000 persons annually) with 1,116 deaths (case-fatality rate of 11.7%) from 2005 through 2012. The highest case-fatality rate was seen in septic shock (45%) followed by streptococcal toxic shock syndrome (STSS; 38%) and NF (29%). The incidence of invasive disease is highest among individuals of age 65 years or older or less than 1 year of age and blacks. Since the addition of the varicella vaccine to the routine childhood immunization series, a steep decline has been observed in the rate of varicella-associated invasive group A streptococcal disease in resource-rich countries. Other risk factors for invasive GAS infection include exposure to other children and household crowding. In some cases, host susceptibility factors may affect the occurrence and severity of invasive GAS infection.\n\nInvasive GAS disease is typically associated with isolation of GAS from the blood or other sterile body site, and may present as STSS with evidence of local soft tissue infection such as NF. The pathogenesis of the severe systemic inflammatory response associated with STSS is related to expression of exotoxins, especially streptococcal pyrogenic exotoxin A. Streptococcal pyrogenic exotoxins represent a family of superantigens that trigger production of proinflammatory cytokines (such as tumor necrosis factor) and result in shock and multiorgan failure.\n\nThe onset and clinical course of STSS with NF can be rapidly progressive with shock and multisystem involvement. The clinical presentation of streptococcal NF is characterized by diffuse swelling of an extremity followed by the appearance of bullous lesions, which may initially contain clear fluid but rapidly develop a maroon or violaceous discoloration.  Pain, out of proportion to the apparent lesion/area of infection, is a vital early clue for NF. Distinction between cellulitis and NF may be difficult early in the disease presentation.\n\nNecrotizing fasciitis is an emergency that requires urgent surgical exploration or incisional frozen-section biopsy for definitive diagnosis and management. Evaluation by a surgeon should not be delayed for imaging studies. Tissue specimens must be sent for bacterial culture and histopathology. Surgical intervention may vary from resection of all necrotic tissue to radical débridement, or even amputation, depending on the extent and location of the disease.  Repeated resection of necrotic tissue may be required to control progressive or persistent infection. In addition to surgical management, aggressive critical care support and antimicrobial therapy with high-dose intravenous penicillin plus a protein-synthesis inhibitor (eg, clindamycin) are required. Clindamycin inhibits enzyme, bacterial toxin, or cytokine production, exhibits a long postantibiotic effect, and has an antimicrobial effect that is unaffected by inoculum size (unlike penicillin). The outcome of STSS with NF may be improved with the use of adjunctive therapy with intravenous immunoglobulin.\n\nPREP Pearls\n\nVaricella skin lesions can serve as a portal of entry for group A streptococcal infection.\n\nInvasive group A streptococcal disease may present as streptococcal toxic shock syndrome with evidence of local soft tissue infection such as necrotizing fasciitis.\n\nNecrotizing fasciitis is an emergency and requires urgent surgical exploration or incisional frozen-section biopsy for definitive diagnosis and management.\n\nABP Content Specifications(s)/Content Area\n\nRecognize the clinical findings associated with necrotizing fasciitis\n\nSuggested Readings\n\nAmerican Academy of Pediatrics. Group A streptococcal infections. In: Kimberlin DW, Brady MT, Jackson MA, Long SS, eds. Red Book: 2018 Report of the Committee on Infectious Diseases. 31st ed. Itasca, IL: American Academy of Pediatrics; 2018:748-762.\n\nFrere J, Bidet P, Tapiéro B, et al. Clinical and microbiological characteristics of invasive group A streptococcal infections before and after implementation of a universal varicella vaccine program. Clin Infect Dis. 2016;62(1):75-77. doi: 10.1093/cid/civ793.\n\nNelson GE, Pondo T, Toews KA, et al. Epidemiology of invasive group A streptococcal infections in the United States, 2005-2012. Clin Infect Dis. 2016;63(4):478-486. doi: 10.1093/cid/ciw248."}
{"id" : 1796, "question_text" : "A 10-year-old girl with autosomal recessive polycystic kidney disease is brought to your office for a health supervision visit. She is afebrile. She has a respiratory rate of 16 breaths/min, heart rate of 70 beats/min, and blood pressure of 130/80 mm Hg. Her height is 110 cm (less than fifth percentile), and her weight is 25 kg (fifth percentile). She started having menstrual periods 6 months ago. Her physical examination is significant for palpable kidneys. You note that her most recent serum creatinine level is 0.62 mg/dL (54.8 µmol/L). You discuss her underlying kidney disease, glomerular filtration rate, and the associated complications with her parents. Of the following, you are MOST likely to inform the parents that", "options" : "[\"growth failure needs treatment with recombinant human growth hormone\", \"hypertension and deterioration in glomerular filtration rate are unrelated\", \"a normal serum creatinine level indicates absence of chronic kidney disease\", \"restriction of protein to less than the recommended intake is indicated\", \"risk for deteriorating glomerular filtration rate is greatest during puberty\"]", "explanation" : "Correct Answer: E\nThe Kidney Disease: Improving Global Outcomes (KDIGO) 2012 clinical practice guidelines diagnose chronic kidney disease (CKD) in children based on the presence of 1 of the following criteria:\n• Glomerular filtration rate (GFR) of less than 60 mL/min/1.73 m2 for greater than 3 months with implications for health, regardless of whether other CKD markers are present\n• Glomerular filtration rate greater than 60 mL/min/1.73 m2 that is accompanied by evidence of structural damage or other markers of functional kidney abnormalities, including proteinuria, albuminuria, renal tubular disorders, or pathologic abnormalities detected by histology or inferred by imaging\n\nThe KDIGO guidelines also stage CKD in children (>2 years) for risk stratification into the following groups.\n• Stage G1: Normal GFR (≥ 90 mL/min/1.73 m2)\n• Stage G2: GFR between 60 and 89 mL/min/1.73 m2\n• Stage G3a: GFR between 45 and 59 mL/min/1.73 m2\n• Stage G3b: GFR between 30 and 44 mL/min/1.73 m2\n• Stage G4: GFR between 15 and 29 mL/min/1.73 m2\n• Stage G5: GFR less than 15 mL/min/1.73 m2\n\nGlomerular filtration rate is a measure of kidney function and indicative of the stage of CKD. The Schwartz formula is used to estimate the GFR from the serum creatinine level:\n          GFR = 0.413 × height (in centimeters) ÷ serum creatinine level (enzymatic method)\nThe GFR for the patient in this vignette is 73 mL/min/1.73 m2 , which corresponds to CKD stage 2.\n\nIn the majority of patients (86%), the diagnosis of CKD is associated with progression of renal disease and impairment of renal function leading to end-stage renal disease, which requires treatment with renal replacement therapy (dialysis or renal transplant). The continued decrease in renal function is caused by repeated acute and chronic insults to the renal parenchyma or by the adaptive hyperfiltration injury (increased glomerular pressure and flow) in the functioning nephrons. As a result of the adaptive hyperfiltration associated with compensatory increased function in the remaining nephrons, GFR is not a good indicator of loss of functioning nephrons in CKD. A patient with a single functioning kidney with half the number of functioning nephrons may have a normal GFR because of the compensatory hyperfiltration in the functioning nephrons. These single kidneys appear larger in size on renal ultrasonography because of compensatory hypertrophy of the functioning nephrons. As the GFR decreases with progressive glomerular injury, the rise in serum creatinine level is also counteracted by increased tubular secretion of endogenous creatinine, leading to no increase or minimal increase in serum creatinine levels. Thus, patients with a decrease in GFR from 120 to 60 mL/min/1.73m2 may have no change or minimal change in their serum creatinine level. The progression of CKD between stages G1 to G3a is accompanied by minimal initial elevation in serum creatinine but a major decrease in GFR. Subsequent progression of CKD (stage G3b to G5) is associated with a marked increase in serum creatinine levels but only a small decrease in GFR.\n\nPersistent glomerular hyperfiltration secondary to parenchymal injury leads to glomerular damage, tubulointerstitial inflammation, and fibrosis. These conditions lead to scarring of the renal glomeruli, blood vessels, and tubulointerstitium over a long-term period. Injury is histologically characterized by glomerulosclerosis, vascular sclerosis, and tubulointerstitial fibrosis. In the early stages (G1-G3a), increasing proteinuria or the onset or worsening of hypertension is indicative of progressive CKD despite stable GFR.\n\nThe rapid increase in body mass during infancy and puberty leads to increased filtration in the remaining nephrons. Risk for CKD progression is greatest during the growth spurts of infancy and puberty; and children in these growth stages should be monitored closely. According to the the Chronic Kidney Disease in Children study, among 891 children (1-16 years old) with glomerular and nonglomerular renal disease, a urine protein to creatinine ratio greater than 2 mg/mg, hypoalbuminemia, and elevated blood pressure were associated with rapid progression of CKD. In addition to increased risk of progression to end-stage renal disease with low GFR at diagnosis, the rate of decline in renal function is affected by ethnicity, primary renal disease, and genetic or familial risk factors for CKD.\n\nIn children with CKD, poor appetite, decreased intestinal absorption of nutrients, and metabolic acidosis leads to malnutrition. Provision of adequate nutrition is important to achieve optimum growth and neurocognitive development. Restriction of protein intake is not recommended in children in view of their needs for growth and neurocognitive development. Also, protein restriction has not been linked with decreased kidney function in children with CKD. Protein intake between 100% and 140% of the dietary reference intake values based on age and sex is recommended for children with CKD and a GFR of 30 to 60 mL/min/1.73m2. In children with a GFR less than 30 mL/min/1.73m2, protein intake between 100% and 120% of the dietary reference intake for age and sex is recommended.\n\nPoor growth is a major complication of children with CKD and a marker for disease severity. Inadequate nutrition, fluid and electrolyte abnormalities (including metabolic acidosis), osteodystrophy, and disturbances of the growth hormone/insulin-like growth factor 1 axis contribute to growth impairment in children with CKD. Prior to initiating therapy with recombinant growth hormone, other factors contributing to growth impairment should be adequately treated. Supplemental enteral feeding via gastrostomy and nasogastric tubes is indicated in children with CKD who have inadequate spontaneous intake to meet growth requirements. Other supportive measures include treatment of electrolyte and fluid losses, metabolic acidosis, anemia, and renal osteodystrophy. Management of renal osteodystrophy includes routine measurement of calcium, phosphorus, parathyroid hormone, and vitamin D levels. Interventions for renal osteodystrophy include dietary phosphorus restriction, vitamin D supplementation, and oral phosphate binders.\n\nPREP Pearls\n• Serum creatinine level and glomerular filtration rate are not good indicators of loss of functioning nephrons because of the compensatory increased function in the remaining nephrons.\n• Increasing proteinuria or the onset or worsening of hypertension is indicative of progressive chronic kidney disease despite a stable glomerular filtration rate.\n• A urine protein to creatinine ratio greater than 2 mg/mg, hypoalbuminemia, and elevated blood pressure were associated with rapid chronic kidney disease progression.\n• Risk for chronic kidney disease progression is greatest during the growth spurts of infancy and puberty.\n\nABP Content Specifications(s)\n• Recognize laboratory abnormalities associated with chronic kidney disease\n• Recognize complications associated with chronic kidney disease\n\nSuggested Readings\n• Kidney Disease: Improving Global Outcomes. KDIGO 2012 clinical practice guideline for the evaluation and management of chronic kidney. Kidney Int Suppl. 2013;3(1):1–150.\n• Massengill SF, Ferris M. Chronic kidney disease in children and adolescents. Pediatr Rev. 2014;35(1):16–29. doi:10.1542/pir.35-1-16.\n• National Kidney Foundation. KDOQI clinical practice guidelines for chronic kidney disease: evaluation, classification, and stratification—part 5: evaluation of laboratory measurements for clinical assessment of kidney disease. http://www2.kidney.org/professionals/kdoqi/guidelines_ckd/toc.htm."}
{"id" : 2963, "question_text" : "Three adolescents are brought to the emergency department 2 days after attending a political rally at which a small explosion occurred near them. None of the girls sustained any physical injuries, so they did not seek immediate medical care. Later that evening, all three began to have nonbloody nonbilious vomiting, diarrhea, mild headache, and fatigue, which has persisted. In the emergency department, all three adolescents appear well and have normal vital signs. On physical examination, each patient has mild diffuse abdominal tenderness and one appears to be mildly dehydrated. The remainder of their physical examination findings are normal. Intravenous lines are placed and a bolus of normal saline is administered to each adolescent. All three have a decreased absolute lymphocyte count, but laboratory findings are otherwise normal. Of the following, the MOST likely cause of the adolescents' symptoms is exposure to", "options" : "[\"anthrax\", \"phosgene gas\", \"radiation\", \"VX gas\"]", "explanation" : "The adolescents in the vignette are suffering from radiation poisoning after exposure from a \"dirty bomb\" at the political rally they attended. The constellation of symptoms after whole-body exposure to ionizing radiation is known as acute radiation syndrome (ARS). The degree of symptoms from ARS is dependent on the total-body dose of radiation.\n\nAcute radiation syndrome has four phases:\n• prodromal: the initial manifestation of symptoms; lasts 2 to 3 days after exposure\n• latent: abeyance of symptoms for several days to a month (timing is dependent on total-body dose)\n• overt illness: the recurrence of symptoms; lasts for weeks to months\n• recovery or death\n\nAcute radiation syndrome manifestations may affect the gastrointestinal system, hematopoietic system, the central nervous system, or a combination of these. The gastrointestinal syndrome includes nausea, vomiting, diarrhea, and abdominal pain. Symptoms begin at an exposure of 1 Grey (Gy); the severity is directly related to the total body dose. Time to onset of symptoms is inversely related to the total-body exposure. Severe gastrointestinal complications, including bowel necrosis and perforation, can occur with significantly higher doses of radiation.\n\nThe hematopoietic syndrome manifests at exposures of 1 Gy or higher. Affected individuals develop lymphopenia, neutropenia, thrombocytopenia, and ultimately anemia. Lymphopenia can occur as early as 6 hours after a large total-body dose of radiation. Absolute lymphocyte counts after radiation exposure follow known curves; thus, the absolute lymphocyte count at a specific interval can be used to approximate both the radiation dose and the prognosis. An absolute lymphocyte count of less than 300/µL 48 hours after radiation exposure portends a poor prognosis.\n\nCentral nervous system syndrome (or cerebrovascular syndrome) occurs with radiation doses exceeding 3 to 4 Gy; symptoms include lethargy and headache. More severe symptoms, such as cerebral edema and seizures, occur with doses greater than 10 Gy.\n\nThe cutaneous syndrome involves erythema, pruritus, pain, and blistering; there is potential for necrosis with high doses. Cutaneous symptoms usually do not occur during the prodromal phase.\n\nRadiation is categorized as either nonionizing or ionizing. Nonionizing radiation includes very-low-frequency waves, radio waves, microwaves, infrared waves, visible light waves, and ultraviolet waves. Nonionizing radiation does not have enough energy to cause acute radiation injury. Ionizing radiation includes both nonparticulate radiation (x-rays and γ-rays) and particulate radiation (α-rays, β-rays and neutrons). Ionizing radiation interacts with other atoms and molecules and causes cellular damage at the molecular level. There are background levels of ionizing radiation in nature that include cosmic radiation, solar radiation, and radon. Air travel increases ionizing radiation exposure. Additional sources of ionizing radiation include nuclear power and research facilities, medical imaging procedures, and therapeutic nuclear medicine.\n\nAs with all emergencies, care for the patient with radiation poisoning begins with assessment of the airway, breathing, and circulation; however, special care must be taken to ensure the safety of providers. Patients not requiring immediate intervention should undergo decontamination before treatment. When providing care to victims of radiation poisoning, health care workers should don a gown, a mask, a hair cover, shoe covers, and a double layer of gloves. The multiple layers of gloves allow for frequent changing of the outer layer while minimizing the risk of skin exposure. Management of radiation poisoning is mostly limited to supportive care and treatment of concomitant injuries, including thermal burns.\n\nAnthrax presents as a cutaneous, gastrointestinal, or inhalational syndrome. Cutaneous anthrax typically affects people who work with animals and handle animal products; it manifests as a localized ulcer. Gastrointestinal anthrax is transmitted through ingestion of contaminated meat. Inhalational anthrax is the most lethal form of anthrax; it can be used as a bioterrorism agent. Inhalational anthrax infection presents with acute onset of fever and respiratory symptoms and can be rapidly fatal.\n\nPhosgene gas is a chemical classified as a pulmonary agent (as is chlorine gas); it primarily affects the upper and lower respiratory tracts. VX gas is a highly toxic nerve agent that causes anticholinergic symptoms immediately on exposure.\n\nPREP Pearls\n• Acute radiation syndrome can include gastrointestinal, neurologic, cutaneous, or hematologic signs and symptoms or a combination thereof.\n• Absolute lymphocyte counts decrease following known patterns after radiation exposure and can be used to deduce approximate timing and doses.\n• Ionizing radiation is present in nature; however, medical imaging, medical therapies, and certain occupations can increase the risk of exposure.\n\nABP Content Specifications(s)\n• Recognize the clinical presentation of radiation exposure and the risk factors (including medical imaging) for such exposure\n\nSuggested Readings\n• Christensen DM, Iddins CJ, Sugarman SL. Ionizing radiation injuries and illnesses. Emerg Med Clin North Am. 2014;32(1):245-265. doi:10.1016/j.emc.2013.10.002.\n• Levin TL. Pediatric imaging: radiation exposure and how we image. Pediatr Rev. 2018;39(1):50-52. doi:10.1542/pir.2017-0181.\n• Rella JG. Radiation. In: Nelson LS, Howland MA, Lewin NW, Smith SA, Goldfrank LR, Hoffman RS, eds. Goldfrank's Toxicologic Emergencies. 11th ed. New York, NY: McGraw-Hill; 2019:1762-1773.\n• Seeyave DM, Brown KM. Environmental, emergencies, radiological emergencies, bites & stings. In: Bachur RG, Shaw KN, eds. Fleisher & Ludwig's Textbook of Pediatric Emergency Medicine. 7th ed. Philadelphia, PA: LWW; 2015:718-760.\n• US Department of Health and Human Services. Radiation emergency medical management. https://www.remm.nlm.gov/index.html#."}
{"id" : 944, "question_text" : "You walk into a mother's room to perform a newborn discharge examination at 72 hours after birth to find a mottled infant with cool extremities. The term newborn is appropriate for gestational age and was born by scheduled repeat cesarean section following an unremarkable pregnancy. Vitals signs include a temperature of 36.6°C, heart rate of 180 beats/min, respiratory rate of 74 breaths/min, blood pressure of 50/35 mm Hg (mean blood pressure, 38 mm Hg), and oxygen saturation of 90% on room air. The examination is notable for diffuse mottling, a capillary refill of 5 to 6 seconds, tachycardia with a single S2 and no murmur, liver down 2 centimeters below the right costal margin, and weak pulses. A rhythm strip is obtained.\n\nOf the following, the MOST likely cause of this infant's presentation is", "options" : "[\"cardiac tamponade\", \"cardiomyopathy\", \"hypoplastic left heart syndrome\", \"myocardial infarction\", \"supraventricular tachycardia\"]", "explanation" : "Preferred Response: C\nA neonate who presents within the first week after birth in cardiogenic shock with an echocardiogram lacking reciprocal forces in lead V1 (ie, no S wave in lead VI) most likely has hypoplastic left heart syndrome. Cardiogenic shock is caused by primary failure of the heart, which leads to a reduction in cardiac output and systemic hypotension. The clinical findings in neonates with cardiogenic shock include mottling, weak pulses, delayed capillary refill, low blood pressure, tachycardia, tachypnea, and hepatomegaly. Causes of cardiogenic shock in the neonate include left-sided critical congenital heart disease (hypoplastic left heart syndrome, critical coarctation of the aorta, interrupted aortic arch, critical aortic valve stenosis), cardiac muscle disorders (intrapartum asphyxia, myocarditis), dysrhythmias (supraventricular tachycardia, complete heart block), and rare metabolic conditions. Rapid cardiorespiratory stabilization is necessary, often before a diagnosis is made.\n\nForms of critical congenital heart disease that present with cardiogenic shock often have inadequate left ventricular development. These neonates rely on right ventricular function to eject blood through the patent ductus arteriosus to maintain systemic blood flow. The patent foramen ovale at the atrial level allows oxygenated blood returning from the lungs to shunt left to right and subsequently mix with the desaturated systemic blood returning from the body. This mixture allows relatively high oxygen saturation in the blood leaving the right ventricle and passing through the patent ductus arteriosus, preventing the infant from appearing cyanotic. Closure of the ductus decreases blood flow to the entire circulation (hypoplastic left heart syndrome, critical aortic valve stenosis) or to the lower body (critical coarctation of the aorta, interrupted aortic arch), leading to hypotension and poor perfusion. If ductal-dependent critical congenital heart disease is suspected in a neonate with cardiogenic shock, prostaglandin El should be given during the resuscitation to re-establish ductal patency.\n\nAffected neonates have very few clinical examination findings until the ductus arteriosus closes. Careful cardiac examination may reveal a single and loud S2, an active precordium, and normal femoral pulses. Because ductal closure may not occur until after the neonate has been discharged from the hospital, congenital heart disease screening is now recommended before discharge. Screening of oxygen saturations in the right hand (preductal) and a lower extremity (postductal) permits identification of neonates with hypoplastic left heart syndrome and interrupted aortic arch as well as some with coarctation of the aorta.\n\nNeonates with cardiac tamponade, cardiomyopathy, myocardial infarction, and supraventricular tachycardia can all present with cardiogenic shock. The presence of a single S2 in the neonate in the vignette suggests hypoplastic left heart syndrome. In addition, the V1 rhythm strip demonstrates a pure R wave in lead V1, with no S wave at all, which is consistent with right ventricular hypertrophy (RVH) (Item C239). Absent S wave in VI. the V1 rhythm strip demonstrates a pure R wave in lead V1, with no S wave at all, which is consistent with right ventricular hypertrophy (RVH) (Item C239). Absent S wave in VI.\n\nThis particular pattern of RVH is typical in hypoplastic left heart syndrome, unlike the upright T wave seen in patients with RVH for other reasons. The neonate described in the vignette does not have muffled heart sounds or ST elevation on the V1 rhythm strip, which makes cardiac tamponade unlikely. The clinical examination and V1 rhythm strip of the neonate in the vignette are not consistent with supraventricular tachycardia, because the heart rate of a neonate with supraventricular tachycardia is typically greater than 220 beats per minutes. The V1 rhythm strip also does not demonstrate ST wave changes suggestive of myocardial ischemia that can be seen with myocardial infarction and cardiomyopathy. Both are extremely rare in the neonatal period.\n\nPREP Pearls\n• Neonates with left-sided congenital heart disease, including hypoplastic left heart syndrome, critical coarctation of the aorta, Interrupted aortic arch, and critical aortic valve stenosis, may present with cardiogenic shock.\n\nAmerican Board of Pediatrics Content Specification(s):\n• Know that cardiogenic shock may be the initial finding in a newborn infant with congenital heart disease\n\nSuggested Reading:\n• Balakrishnan PL, Juraszek AL. Pathology of congenital heart disease. NeoReviews. 2012;13:e703-e710. doi: 10.1542/neo.13-12-e703.\n• Lees MH, King DH. Cardiogenic shock in the neonate. Pediatr Rev. 1988;9:258-266. doi: 10.1542/pir.9-8-258\n• Silberbach M, Hannon D. Presentation of congenital heart disease in the neonate and young infant. Pediatr Rev. 2007;28:123-131. doi:10.1542/pir.28-4-123\nHannon D. Presentation of congenital heart disease in the neonate and young infant. Pediatr Rev. 2007;28:123-131. doi:10.1542/pir.28-4-123"}
{"id" : 3757, "question_text" : "A 6-month-old infant who was born at 27 weeks' gestation has feeding difficulties. A gastrostomy tube has been placed through which she receives most of her nutrition. The parents seek advice on what they need to consider if tube feedings are to continue when starting daycare. Of the following, the MOST accurate statement about this infant's feeding is that", "options" : "[\"daycare centers are considered private entities, therefore they are not required to accept children with this condition\", \"in all states, this is considered a medical procedure and can only be administered by a nurse or medical professional\", \"the child should only attend a medical or special needs daycare\", \"this would be covered under the Americans with Disabilities Act; daycare centers must make reasonable accommodations\"]", "explanation" : "Correct Answer: D\nThe premature infant in the vignette receives most of her nutrition via gastrostomy tube, which would be considered a disability under federal law. The Americans with Disabilities Act (ADA) is a federal civil rights law originally passed in 1990 that, among other things, prohibits discrimination by child care centers and family child care providers against individuals of all ages with disabilities. Individuals of all ages receive protection under the ADA who have a history of, who are regarded as having, or who have a physical or mental impairment that substantially limits 1 or more major life activities (seeing, hearing, eating, etc).\n\nPrivately run child care centers are considered \"public accommodations,\" similar to private schools, recreation centers, restaurants, hotels, movie theaters, etc, and must comply with the ADA, which states that centers:\n• Cannot exclude children with disabilities from their programs unless their presence would pose a direct threat to the health or safety of others or require a fundamental alteration of the program\n• Must make reasonable modifications to policies and practices to integrate children with disabilities into their programs unless doing so would constitute a fundamental alteration\n• Must provide appropriate auxiliary aids and services needed for effective communication with children with disabilities when doing so would not constitute an undue burden\n• Must generally make facilities accessible to children with disabilities\n\nChild care services facilitated by government agencies, such as Head Start, must also comply with the ADA. Child care centers that are provided by religious entities, such as churches, mosques, or synagogues, are exempt.\n\nAlthough a day care center may make an individualized assessment about whether it can meet the particular needs of a child without fundamentally altering its program, case law has demonstrated that children who require diabetes-related care, for example, can be integrated into these settings. Furthermore, most community care licensing agencies do not consider the administration of routine gastrostomy tube feedings or administering liquid medication through gastrostomy tubes to be a medical procedure. However, day care personnel must be properly trained and instructed on gastrostomy tube care. Personnel are usually required to follow specific written instructions from the child's health care provider including, but not limited to the exact steps necessary to provide feedings or administer medications; frequency, amount, and type of formula, liquid, or medication; proper cleaning procedures; potential side effects; and emergency contact and procedures should the gastrostomy tube become dislodged. A child who feeds via gastrostomy tube does not need to attend a medical or special needs–only day care center.\n\nIn the realm of educational access, children with special needs receive additional protections under 2 federal laws, the Individuals with Disabilities Education Act (IDEA) and Section 504 of the Rehabilitation Act.\n\nRehabilitative services may be provided to children as part of IDEA (Individualized Education Program/Individualized Family Service Plan) or Section 504 (504 plan). These include occupational, physical, and speech/language therapy. Occupational therapy assists children to improve their physical, sensory, and fine motor skills. Occupational therapy addresses psychological, social, and environmental factors that can affect functioning, such as play, school performance, and daily activities. Physical therapy might assist with developing or normalizing range of motion, strength, flexibility, functional stability, proprioception, and gross motor skills. Speech and language therapy addresses disorders related to articulation, fluency, voice, reception, expression, cognitive-communication, dysphagia, and oral feeding.\n\nPREP Pearls\n• The Americans with Disabilities Act is a federal civil rights law that, among other things, prohibits discrimination by child care centers and family child care providers against individuals of all ages with disabilities, including children with gastrostomy tubes. These providers are considered public entities except for those governed by religious organizations.\n• The Individuals with Disabilities Education Act requires special educational programs for children with disabilities; provides funding for free appropriate public education for children aged 3 to 22 years with disabilities; and authorizes services for early intervention programs for infants and children younger than 3 years.\n• Section 504 of the Rehabilitation Act prohibits discrimination on the basis of disability in programs that receive federal funding; affords rights of equal access to free appropriate public education via a wide range of services in the least restrictive environment for school-aged children and reasonable accommodations for children in child care or college.\n\nABP Content Specifications(s)\n• Recognize the value and limitations of language, occupational, and physical therapy\n• Understand the provisions of current legislation for patients of various ages who have educational or physical disabilities\n\nSuggested Readings\n• Lipkin PH, Okamoto J; Council on Children with Disabilities, Council on School Health. The Individuals with Disabilities Education Act (IDEA) for children with special healthcare needs. Pediatrics. 2015;136(6):e1650-e1662. doi:10.1542/peds.2015-3409.\n• Section 504 of the Rehabilitation Act. 29 USC §794, Title 29. Labor. Chapter 16: Vocational Rehabilitation and Other Rehabilitation Services, Subchapter V; Rights and Advocacy, section 794, nondiscrimination under federal grants and programs; 2011.\n• The Americans with Disabilities Act. 42 USC §12101, Title 42. The Public Health and Welfare, Chapter 126, Equal Opportunity for Individuals with Disabilities, section 12101, findings and purpose; 1990.\n• The Individuals with Disabilities Education Act. 20 USC §1400, Title 20. Education, Education of Children with Disabilities, Subchapter I, Definitions, section 1400, short title, findings, purposes; 2011."}
{"id" : 292, "question_text" : "For the past 2 years, you have been providing medications for a 9-year-old girl in whom you diagnosed attention-deficit/hyperactivity disorder (ADHD) using parent and teacher assessments and family history. You have sequentially prescribed methylphenidate, dextroamphetamine, and currently atomoxetine. All have yielded the same benefits on her inattention and impulsivity but have not improved her occasional aggressive behaviors. Today her mother brings her in, saying that she has had a significant worsening in her aggression and is now getting into fights at school. When asked about the most recent incident, the girl replies, \"Sheryl was making faces at me for days, so I hit her, and it serves her right.\" The mother describes her daughter as being chronically vindictive at home, aggressively \"paying back\" her siblings as much as several days later when she feels she has been wronged. The mother reports no major changes in the household and no traumatic or bullying incidents for the girl. She indicates that her husband has a history of anger management difficulties. Of the following, the MOST appropriate next step in care is to", "options" : "[\"add guanfacine to the girl's regimen\", \"have the mother and teacher complete the Vanderbilt Diagnostic Rating Scale\", \"prescribe risperidone at bedtime\", \"refer the family for behavior management training\", \"wean the girl off the atomoxetine and initiate a trial of lisdexamfetamine\"]", "explanation" : "The girl described in the vignette has responded well to standard treatment for ADHD, but her persistent calculated aggressive behaviors suggest the need for further interventions. Children who exhibit aggressive behavior problems are most likely to be helped by behavior management training, which educates individuals in the world around them (parents, teachers) to reward the children for positive social behaviors and to have consistent, appropriate consequences such as physical limits and the withdrawal of positive attention for undesired behaviors. Many different types of behavior management training, including Parent Management Training, Emotion Coaching, and 123 Magic™, have been shown to accomplish this goal.\n\nThe girl's described aggression is considered a \"cold\" type of aggression, in that it is calm, planned, and calculated to obtain a goal. Medications such as risperidone and guanfacine typically do not reduce this type of aggression. \"Hot\" aggression, which is impulsive, poorly planned, and associated with fight-or-flight arousal, is much more likely to respond to medication. The situation in which medications reduce the frequency and severity of \"hot\" aggression typically involves a treatable comorbidity such as ADHD that is being addressed successfully with a medication (most commonly, stimulants for ADHD). Extreme impulsive or \"hot\" aggression that is unresponsive to behavior management might be treated with a nonspecific impulsive aggression medication (eg, risperidone or valproic acid), but such an approach should be undertaken cautiously because of potential medication adverse effects.\n\nThe Vanderbilt Diagnostic Rating Scale aids in determining the current presence/absence of ADHD symptoms, but this assessment is not indicated because the diagnosis has been confirmed and the condition has responded to appropriate therapy. In addition, there is no need to change ADHD medication to another agent such as lisdexamfetamine (prodrug of dextroamphetamine) in a child whose ADHD symptoms are already well-controlled and have responded equally well to different medications.\n\nCritique: The girl described in the vignette has responded well to standard treatment for ADHD, but her persistent calculated aggressive behaviors suggest the need for further interventions. Children who exhibit aggressive behavior problems are most likely to be helped by behavior management training, which educates individuals in the world around them (parents, teachers) to reward the children for positive social behaviors and to have consistent, appropriate consequences such as physical limits and the withdrawal of positive attention for undesired behaviors. Many different types of behavior management training, including Parent Management Training, Emotion Coaching, and 123 Magic™, have been shown to accomplish this goal.\n\nThe girl's described aggression is considered a \"cold\" type of aggression, in that it is calm, planned, and calculated to obtain a goal. Medications such as risperidone and guanfacine typically do not reduce this type of aggression. \"Hot\" aggression, which is impulsive, poorly planned, and associated with fight-or-flight arousal, is much more likely to respond to medication. The situation in which medications reduce the frequency and severity of \"hot\" aggression typically involves a treatable comorbidity such as ADHD that is being addressed successfully with a medication (most commonly, stimulants for ADHD). Extreme impulsive or \"hot\" aggression that is unresponsive to behavior management might be treated with a nonspecific impulsive aggression medication (eg, risperidone or valproic acid), but such an approach should be undertaken cautiously because of potential medication adverse effects.\n\nThe Vanderbilt Diagnostic Rating Scale aids in determining the current presence/absence of ADHD symptoms, but this assessment is not indicated because the diagnosis has been confirmed and the condition has responded to appropriate therapy. In addition, there is no need to change ADHD medication to another agent such as lisdexamfetamine (prodrug of dextroamphetamine) in a child whose ADHD symptoms are already well-controlled and have responded equally well to different medications.\n\nContent Specifications: Know the difference between \"hot\" or impulsive aggression, and \"cold\" or planned aggression"}
{"id" : 2068, "question_text" : "A 7-year-old girl sustained a tibia fracture during a family vacation. She was seen in an emergency department, placed in a hard splint, and instructed to use crutches to avoid weight bearing. She has a follow-up visit scheduled with an orthopaedic physician in 3 days. Over the past few hours, the girl has become very distressed. Her father calls the office stating that his daughter reports increased pain, despite taking the prescribed acetaminophen and hydrocodone suspension. The girl denies numbness or tingling in the foot. Her toes are visible, and the father states that they appear \"normal.\" Of the following, the MOST appropriate recommendation regarding this girl's care is to", "options" : "[\"add ibuprofen to the girl's current regimen of pain medications\", \"apply ice packs to the foot over the splint\", \"call the orthopaedic clinic and arrange for an outpatient visit in the next 24 hours\", \"loosen the splint and take the girl to an emergency department immediately\"]", "explanation" : "The girl in the vignette sustained a lower extremity fracture and is wearing a hard splint. She is experiencing a sudden increase in pain that is not responding to an opioid medication. This scenario is concerning for acute compartment syndrome. Her parents should immediately loosen the splint and bring her to the emergency department for evaluation.\n\nA 'compartment' is a group of limb muscles and their associated nerves and blood vessels, surrounded by fascia. Following an injury, bleeding and swelling can cause increased pressure in a given compartment. Acute compartment syndrome occurs when increased pressure, often as a result of injury, leads to compression of intracompartmental vasculature and ischemia, causing subsequent nerve and muscle damage. Acute compartment syndrome is a surgical emergency. Because the early signs can be subtle, and children may have difficulty articulating their symptoms, this condition can be difficult to diagnose.\n\nChildren most often present with compartment syndrome following forearm, distal humerus, or tibia fractures. The classic \"5 Ps\" of acute compartment syndrome are pain, pallor, pulselessness, paresthesia, and paralysis. With the exception of pain, these signs and symptoms are late findings. A child with a fracture who presents with agitation and increased pain that does not respond to medications should be urgently evaluated for compartment syndrome. Providers should remove casts or splints and palpate along the limb for pain and swelling. For severe fractures, a portion of the cast or splint can be left in place, but enough should be removed to allow for complete examination of the affected limb. Muscle stretch will often increase pain with acute compartment syndrome. The treatment of acute compartment syndrome is urgent fasciotomy surgery. Failure to recognize an acute increase in pain as a symptom of possible compartment syndrome can lead to delayed diagnosis and severe, permanent disability due to nerve and muscle damage. Therefore applying ice packs, ibuprofen, and evaluation in 24 hours would not be appropriate for the girl in the vignette.\n\nChronic exertional compartment syndrome (CECS) occurs when exercise leads to a mild increase in intracompartmental pressure. This condition almost always affects the muscle compartments of the lower leg. Athletes present with cramping pain, and sometimes with numbness and paresthesias, during exercise. Pain onset typically follows a predictable period of exercise, especially with running, and worsens as the athlete continues the activity. Definitive diagnosis requires measurement of compartment pressure. Because this measurement is invasive, a high index of clinical suspicion is enough to initiate conservative management of this condition. Initial treatment of CECS involves physical therapy and modification of the activity schedule. For intractable CECS, referral to orthopaedic surgery for elective fasciotomy surgery may be indicated.\n\nPREP Pearls\n\nThe classic \"5 Ps\" of acute compartment syndrome are pain, pallor, pulselessness, paresthesia, and paralysis. With the exception of pain, these signs and symptoms are late findings.\n\nAcute compartment syndrome is a surgical emergency.\n\nFailure to recognize an acute increase in pain as a symptom of possible compartment syndrome can lead to delayed diagnosis and severe, permanent disability due to nerve and muscle damage.\n\nABP Content Specifications(s)/Content Area\n\nRecognize the clinical findings associated with compartment syndrome\n\nSuggested Readings\n\nGresh M. Compartment syndrome in the pediatric patient. Pediatr Rev. 2017;38(12):560-565. doi: 10.1542/pir.2016-0114.\n\nSarwark JF, LaBella CR, ed Pediatric Orthopaedics and Sports Injuries: A Quick Reference Guide. 2nd ed. Elk Grove Village, IL: American Academy of Pediatrics; 2014.\n\nvon Keudell AG, Weaver MJ, Appleton PT, et al. Diagnosis and treatment of acute extremity compartment syndrome. Lancet. 2015;386(10000):1299-1310. doi: 10.1016/S0140-6736(15)00277-9."}
{"id" : 2895, "question_text" : "A 16-year-old adolescent girl is seen in the clinic for a pregnancy test. The limits of confidentiality are discussed. She reports that she recently met her older boyfriend on a social media site. She sneaks out of her house to see him every night; her family does not know about him. He frequently takes her to parties at which she participates in sexual activities with his friends when he asks her to. He rewards her with clothes and jewelry. Her last sexual encounter with his friend was 1 week ago and was unprotected. Physical examination findings are unremarkable. The result of her pregnancy test is negative. She reports no abdominal or genitourinary symptoms. Results of testing for sexually transmitted infections are pending. Concerns for her safety are discussed. The patient requests that her parents not be told. Of the following, the BEST next step in treatment is to contact", "options" : "[\"child protective services\", \"the local law enforcement agency\", \"a national trafficking organization hotline\", \"the patient's parents\"]", "explanation" : "Children and adolescents who experience sexual exploitation, including sex trafficking, human trafficking, and commercial sexual exploitation (CSE), seek medical attention for a variety of reasons but seldom self-identify. Unfortunately, with the rise of social media and the help of an anonymous cyberspace environment, opportunities abound for the sexual exploitation of children and adolescents by sex offenders. Many survivors perceive these sexually exploitative relationships as consensual.\n\nWhen responding to cases of suspected CSE, it is critical to avoid further harm to the patient. Ideally, a national trafficking organization (such as the National Human Trafficking Resource Center Hotline at 1-888-373-7888 sponsored by the Polaris Project) should be contacted initially for assistance. The hotline can assist in identifying sensitive law enforcement agents to ensure that the patient is treated as a victim of sexual exploitation rather than a criminal guilty of prostitution. Although it is reasonable to contact the patient's parents, state or local law enforcement, state child protective services (CPS) agency, or local child advocacy centers, health care providers, too, should be cognizant of potential harm that could occur.\n\nAlthough the health care provider is likely a mandated reporter in most states, providers must use extreme caution when making a report to CPS without first contacting a national trafficking organization. Mandated reports to CPS may not actually result in a favorable outcome because of the variability of services available and the possible lack of understanding by individual CPS workers as to the unique issues facing victims of CSE. Additionally, although federal law designates children and adolescents as victims who cannot consent to commercial sexual acts, many states still view commercial sexual exploitation of children through the lens of prostitution laws, thus treating minors as criminals rather than victims. Youth who are victims of CSE are less likely to receive critical services and protection when they enter the juvenile justice system, and they may suffer additional trauma and ultimately re-enter the world of trafficking upon release.\n\nIt is critical for health care providers to be aware of possible risk factors and indicators for CSE or trafficking because most victims will not self-identify or disclose this information (Item C96). When potential indicators are identified, the health care provider may ask more direct questions, although many barriers to disclosure exist, including fear of the exploiter, shame, hostility, and intoxication. Thus, it is critical to build rapport with the individual, discuss limits of confidentiality, and to avoid causing additional trauma.\n\nDirection questions that may be helpful to elicit a history of sexual exploitation include:\n• Have you ever been asked to exchange sex for money, food, shelter, or other items?\n• Have you ever been asked by someone to have sex with another person?\n• Have you ever had sexual photographs taken of you or posted on the internet?\n\nResearch shows that parents underestimate the likelihood that their child or teen will engage in online contact with strangers; this highlights the importance of parents' promoting Internet safety, monitoring online usage, using parental control software, and maintaining awareness of children's Internet activities from an early age. Furthermore, health care professionals should counsel patients and families and work with them to develop individualized family rules and guidelines surrounding the Internet and social media usage, such as the American Academy of Pediatrics Family Media Plan (www.healthychildren.org/MediaUsePlan). Providers should also provide anticipatory guidance to pediatric patients about protecting themselves from exploitation.\n\nPREP Pearls\n• Children and adolescents who have experienced sexual exploitation, including sex trafficking, human trafficking, and commercial sexual exploitation, seek medical attention for a variety of reasons but seldom self-identify.\n• When responding to cases of suspected commercial sexual exploitation, a national trafficking organization (such as the National Human Trafficking Resource Center Hotline at 1-888-373-7888, sponsored by the Polaris Project) should be contacted for assistance.\n• Providers should also provide anticipatory guidance to pediatric patients about protecting themselves from online exploitation.\n\nMOCA-Peds Objective\n• Respond appropriately to a child's disclosure of sexual abuse.\n\nABP Content Specifications(s)\n• Counsel patients regarding the proper use of the internet and social networking sites\n• Understand the potential effects of various media on child and adolescent behavior\n\nSuggested Readings\n• American Professional Society on the Abuse of Children Task Force. The commercial sexual exploitation of children: the medical provider's role in identification, assessment, and treatment. ASPAC Practice Guidelines. Chicago, IL: American Professional Society on the Abuse of Children; 2013. Available at: https://www.apsac.org/9235fgnl8.\n• Brown AC, Barron CE. Human trafficking. Pediatr Rev. 2018;39(2):102-103. doi:10.1542/pir.2016-0181.\n• Chassiakos YLR, Radesky K, Christakis D, Moreno MA, Cross C; American Academy of Pediatrics Council on Communications and Media. Children and adolescents and digital media. Pediatrics. 2016;138(5):e20162593. doi:10.1542/peds.2016-2593.\n• Greenbaum J, Bodrick N; Committee on Child Abuse and Neglect; Section on International Child Health. Global human trafficking and child victimization. Pediatrics. 2017;140(6):e20173138. doi:10.1542/peds.2017-3138.\n• Greenbaum J, Crawford-Jakubiak JE; Committee on Child Abuse and Neglect. Child sex trafficking and commercial sexual exploitation: health care needs of victims. Pediatrics. 2015;135(3):566-574. doi:10.1542/peds.2014-4138."}
{"id" : 1946, "question_text" : "The father of a 6-year-old girl is concerned about his daughter's learning. Although the child appears motivated at school, she has been unable to keep up with her peers. A psychoeducational evaluation has identified both cognitive and adaptive delays. The girl has qualified for an Individualized Education Program and will be placed in a special education classroom. Her father reports that the child's mother used various substances during her pregnancy and asks if any could have caused his daughter's disability. A picture of the girl is shown in Item Q146. Of the following, the substance MOST likely to have caused this child's difficulties is", "options" : "[\"cocaine\", \"ethanol\", \"marijuana\", \"nicotine\"]", "explanation" : "The girl in this vignette has intellectual disability (ID), defined by significant delays in both cognitive and adaptive functioning. The causes of ID include congenital infections (eg, cytomegalovirus, rubella, toxoplasmosis), central nervous system infections (eg, meningitis, encephalitis), trauma, malignancy, genetic abnormalities (eg, trisomy 21, fragile X syndrome), inborn errors of metabolism, and teratogens (eg, alcohol, illicit and prescription drugs, lead, radiation). Ethanol is the most common teratogen causing ID.\n\nFetal alcohol spectrum disorder encompasses a range of adverse effects associated with prenatal alcohol exposure (eg, fetal alcohol syndrome, partial fetal alcohol syndrome, alcohol-related birth defects, alcohol-related neurodevelopmental disorder, neurobehavioral disorder associated with prenatal alcohol exposure). Full fetal alcohol syndrome includes differences in growth (ie, weight and/or height ≤ 10th percentile), physical features (eg, smooth philtrum, thin upper lip, small palpebral fissures), and central nervous system abnormalities (eg, microcephaly, seizures, ID, learning disabilities, attention-deficit/hyperactivity disorder). Moderate to heavy alcohol consumption during pregnancy is associated with greater risk of fetal alcohol syndrome; however, there are no known safe limits of alcohol consumption during pregnancy.\n\nIdentifying the cause of ID can help with family planning, identifying associated medical risks, accessing support systems, and relieving families of guilt or anxiety about presumed causes of their child's ID. In the case of inborn errors of metabolism, treatment may be available.\n\nPREP Pearls\n\nThe causes of intellectual disability include congenital infections (eg, cytomegalovirus, rubella, toxoplasmosis), central nervous system infections (eg, meningitis, encephalitis), trauma, malignancy, genetic abnormalities (eg, trisomy 21, fragile X syndrome), inborn errors of metabolism, and teratogens (eg, alcohol, illicit and prescription drugs, lead, radiation).\n\nFull fetal alcohol syndrome includes differences in growth (ie, weight and/or height ≤10th percentile), physical features (eg, smooth philtrum, thin upper lip, small palpebral fissures), and central nervous system abnormalities (eg, microcephaly, seizures, intellectual disability, learning disabilities, attention-deficit/hyperactivity disorder).\n\nModerate to heavy alcohol consumption during pregnancy is associated with greater risk of fetal alcohol syndrome; however, there are no known safe limits of alcohol consumption during pregnancy.\n\nABP Content Specifications(s)/Content Area\n\nIdentify common teratogenic causes of intellectual disabilities\n\nIdentify common infectious causes of intellectual disabilities\n\nSuggested Readings\n\nOji-Mmuo CN, Corr TE, Doheny KK. Addictive disorders in women: the impact of maternal substance use on the fetus and newborn. Neoreviews. 2017;18(10):e576-e586. doi:10.1542/neo.18-10-e576.\n\nShea SE. Intellectual disability (mental retardation). Pediatr Rev. 2012;33(3):110-121. doi:10.1542/pir.33-3-110.\n\nWilliams JF, Smith VC; Committee on Substance Abuse. Fetal alcohol spectrum disorders. Pediatrics. 2015;136(5):e1395-e1406. doi:10.1542/peds.2015-3113."}
{"id" : 1800, "question_text" : "An 11-year-old boy is seen for an annual health supervision visit. He received a bone marrow transplant from an unrelated donor for relapsed acute lymphoblastic leukemia at 7 years of age. His transplant conditioning included total body irradiation (1,350 cGy) and cyclophosphamide. He was weaned off of immunosuppressive drugs over a 12-month period, and for the last 3 years he has been healthy and off all medications. As part of your evaluation, you ask about his school performance. He has been attending the fifth grade, but reports struggling in many classes, especially math and science. Although he and his parents report that he is a serious student and a hard worker, his grades have declined over the last 3 years, and he has become frustrated with his school work. Both of his parents are professionals, and he has 2 older siblings who are excellent students. The boy tells you that he wonders why he does not do as well in school as his siblings. Of the following, you counsel him and his parents that", "options" : "[\"after undergoing a traumatic medical intervention such as bone marrow transplant, he is most likely focusing on \\\"life\\\" and not giving academics his full effort\", \"because he was kept out of school for over a year for the bone marrow transplant, he should repeat the fifth grade\", \"bone marrow donors are not screened for intelligence, so his school performance likely reflects his donor's academic potential\", \"none of the therapies he received should affect his academic performance, so he should continue to work hard and over time his grades will improve\", \"the transplant conditioning, he received puts him at high risk for neurocognitive changes, so you would like to refer him for a formal neurocognitive evaluation\"]", "explanation" : "Correct Answer: E\nLong-term survivors of childhood cancer and bone marrow transplant are at high risk for chronic diseases and disorders as a consequence of their treatments. These diseases and disorders include cardiovascular disease, pulmonary dysfunction, endocrinopathies, infertility, musculoskeletal disorders, secondary malignant neoplasms, and neurocognitive deficits. Almost every long-term survivor has at least one measurable defect in organ function. The risk for specific late effects is directly related to the chemotherapy, radiation, and surgery exposures the survivor received during treatment. For example, patients who received anthracyclines such as doxorubicin are at higher risk for cardiomyopathy in a dose-dependent manner, and female survivors who received chest radiation have a 35% chance of developing breast cancer by 50 years of age. The Children's Oncology Group has created exposure-based guidelines for the screening of survivors (www.survivorshipguidelines.org). Many pediatric oncology programs have specialized survivorship programs that can assist with formulating personalized screening plans based on exposures.\n\nThe central nervous system is a sanctuary site for childhood leukemia; therefore, children treated for leukemia receive central nervous system prophylaxis with intrathecal chemotherapy, which is administered directly into the spinal fluid. Although intrathecal chemotherapy is generally well tolerated, it is often performed during brain development and can cause neurocognitive changes. Radiation therapy to the brain of a child can also impact neurocognition. The child in this vignette underwent a bone marrow transplant for relapsed acute lymphoblastic leukemia. Although the child's chemotherapy and radiation exposures during the initial treatment of the leukemia are not specified, it is very likely that he received multiple intrathecal chemotherapy administrations. In addition, he underwent total body irradiation as part of the conditioning regimen for the bone marrow transplant. Total body irradiation includes radiation to the brain, which can impact cognitive development. The boy in this vignette reports having challenges in school that make his academic experience markedly different than his family's academic achievements. These challenges must be recognized and addressed, because an appropriate education plan in school can prevent further frustration and potential education failure. The boy should have a formal neurocognitive assessment, and any resulting recommendations should be provided to the school. These recommendations can be formally discussed between the school and the family in a 504 meeting (in reference to section 504 of the Americans with Disabilities Act) and then formalized as interventions in an individualized education program.\n\nThe boy described in this vignette reports working hard at school, which has been corroborated by his parents. It is important to understand the patient's history so as not to mistake educational challenges for laziness or apathy. The intelligence of the marrow donor should have no bearing on the school performance of the recipient. Although it is likely that the boy in this vignette would benefit from some interventions in school, repeating a grade is not necessarily the best option. He should receive a formal neurocognitive assessment of his academic strengths and weaknesses prior to any recommendations being implemented.\n\nPREP Pearls\n• Survivors of childhood cancer and bone marrow transplant are at high risk for late morbidity as a consequence of chemotherapy, radiation, and surgery.\n• Childhood cancer survivors who received intrathecal chemotherapy or radiation to the brain are at risk for neurocognitive changes and educational challenges and should therefore undergo formal neurocognitive testing.\n• Many pediatric oncology programs have survivorship programs that can assist with formulating individualized, exposure-based screening plans.\n\nABP Content Specifications(s)\n• Recognize psychosocial and family issues associated with transplantation\n\nSuggested Readings\n• Landier W, Armenian S, Bhatia S. Late effects of childhood cancer and its treatment. Pediatr Clin North Am. 2015;62(1):275–300. http://dx.doi.org/10.1016/j.pcl.2014.09.017.\n• Meck MM, Leary M, Sills RH. Late effects in survivors of childhood cancer. Pediatr Rev. 2006;27(7):257–262. http://dx.doi.org/10.1542/pir.27-7-257.\n• Hudson MM, Ness KK, Gurney JG, et al. Clinical ascertainment of health outcomes among adults treated for childhood cancer. JAMA. 2013;309(22):2371–2381. http://dx.doi.org/10.1001/jama.2013.6296.\n• Children's Oncology Group. Children's Oncology Group. Long-term follow-up guidelines for survivors of childhood, adolescent, and young adult cancers. Version 4.0. 2014. http://www.survivorshipguidelines.org. Available at: http://www.survivorshipguidelines.org"}
{"id" : 3023, "question_text" : "A 31-month-old boy is seen in the office for a growth check. He was born at term. The delivery was complicated by excessive hemorrhage. He has been healthy and takes a daily multivitamin. A comprehensive review of systems is unremarkable. His adjusted midparental height is at the 40th percentile. His growth charts are shown in Item Q226A and Item Q226B. His vital signs are normal for age, and his physical examination findings are unremarkable. Laboratory data are shown: White blood cell count 8,300/μL (8.3 × 109/L), Hemoglobin 13 g/dL (130 g/L), Platelet count 281 × 103/μL (281 × 109/L), Sodium 138 mEq/L (138 mmol/L), Potassium 4.1 mEq/L (4.1 mmol/L), Bicarbonate 25 mEq/L (25 mmol/L), Creatinine 0.3 mg/dL (26.5 µmol/L), Glucose 90 mg/dL (5.0 mmol/L), Thyroid-stimulating hormone 1.4 mIU/L (reference range, 0.27-4.2 mIU/L), Free thyroxine 1.3 ng/dL (17 pmol/L) (reference range, 0.9-1.7 ng/dL [12-22 pmol/L]). Urinalysis findings are normal except for a specific gravity of 1.020 and a pH of 6. Of the following, the MOST likely diagnosis is", "options" : "[\"celiac disease\", \"growth hormone deficiency\", \"insufficient caloric intake\", \"renal tubular acidosis\"]", "explanation" : "The boy in the vignette has growth hormone deficiency. His length curve shows declining percentiles after about 12 months of age with preservation of his weight-for-length percentile. Children with congenital growth hormone deficiency are typically of normal size at birth. Linear growth deceleration occurs after the age of 6 to 12 months, when growth hormone becomes important for linear growth. Weight is not affected as much as length, so weight-for-length or body mass index percentile is preserved. There may also be evidence of altered body composition with increased fat mass and decreased lean body mass. The history of excessive hemorrhage at the time of delivery is a risk factor for growth hormone deficiency resulting from a vascular injury to the pituitary gland. Of the pituitary hormones, growth hormone is the most sensitive to pituitary insults.\n\nOther pituitary hormone deficiencies can be associated with congenital growth hormone deficiency. Hypoglycemia and prolonged jaundice may occur with isolated growth hormone deficiency but are more common with multiple pituitary hormone deficiencies. The presence of nystagmus and midline defects suggests septo-optic dysplasia. Males can have a small penis, cryptorchidism, or both, especially if concomitant gonadotropin deficiency is present. Brain magnetic resonance imaging may reveal pituitary gland or stalk abnormalities, optic nerve hypoplasia, or agenesis of the septum pellucidum/corpus callosum.\n\nAcquired growth hormone deficiency presents with linear growth deceleration after a period of normal growth. Relative weight gain with increased fat mass is common. Acquired growth hormone deficiency may be secondary to a brain tumor, cranial irradiation, or head trauma. Other pituitary hormone deficiencies may be associated. Levels of insulin-like growth hormone factor-1 and insulin-like growth factor binding protein 3 are usually low, and growth hormone levels after stimulation remain low. Bone age is delayed. Patients with growth hormone deficiency should undergo magnetic resonance imaging of the brain and sella turcica and testing for other pituitary hormone deficiencies. Children with growth hormone deficiency grow very well on relatively low doses of growth hormone therapy.\n\nCeliac disease, insufficient caloric intake, and renal tubular acidosis are less likely causes of growth failure for the boy in the vignette, given the preservation of his weight-for-length percentile and the lack of supporting findings on laboratory evaluation. Anemia may be present with celiac disease or insufficient caloric intake. Metabolic acidosis and electrolyte abnormalities are usually present with renal tubular acidosis. Although not a response choice, hypothyroidism can cause linear growth failure; however, it would not be the cause of this boy's condition, given his normal thyroid function test results.\n\nPREP Pearls\n• In those with congenital growth hormone deficiency, linear growth becomes abnormal at about the age of 6 to 12 months.\n• Patients with acquired growth hormone deficiency should undergo investigation for a brain neoplasm and other pituitary hormone deficiencies.\n• The weight-for-length or body mass index curve is important to consider in narrowing the differential diagnosis of abnormal growth.\n\nMOCA-Peds Objective\n• Recognize and plan initial evaluation of a child with a pituitary disorder.\n\nABP Content Specifications(s)\n• Recognize the clinical features associated with acquired and congenital growth hormone deficiency\n• Recognize the effects of growth hormone therapy on growth\n\nSuggested Readings\n• Braun LR, Marino R. Disorders of growth and stature. Pediatr Rev. 2017;38(7):293-304. doi:10.1542/pir.2016-0178.\n• Kaplowitz P. Short stature. In: McInerny TK, Adam HM, Campbell DE, DeWitt TG, Foy JM, Kamat DM, eds. American Academy of Pediatrics Textbook of Pediatric Care. 2nd ed. Elk Grove Village, IL: American Academy of Pediatrics; 2017:1585-1588. Pediatric Care Online.\n• Rogol AD, Hayden GF. Etiologies and early diagnosis of short stature and growth failure in children and adolescents. J Pediatr. 2014;164(5 Suppl):S1-14.e6. doi:10.1016/j.jpeds.2014.02.027.\n• Rose SR, Vogiatzi MG, Copeland KC. A general pediatric approach to evaluating a short child. Pediatr Rev. 2005;26(11):410-20. doi:10.1542/pir.26-11-410.\n• Suresh S, Santhanam I. Hypoglycemia. In: McInerny TK, Adam HM, Campbell DE, DeWitt TG, Foy JM, Kamat DM, eds. American Academy of Pediatrics Textbook of Pediatric Care. 2nd ed. Elk Grove Village, IL: American Academy of Pediatrics; 2017:2881-2887. Pediatric Care Online."}
{"id" : 1625, "question_text" : "A 16-year-old adolescent girl visits your clinic with a chief symptom of fatigue. She describes the fatigue as being present over the prior 1 or 2 weeks and increasing in severity. She has not been to soccer practice during this time and has been too fatigued to attend school over the last few days. She reports more hair loss than usual in the shower. She has a heart rate of 100 beats/min and blood pressure of 135/60 mm Hg. The remainder of her vital signs are normal. Neck examination shows an enlarged thyroid with a bruit on auscultation. Her heart rate is regular. The lungs are clear to auscultation bilaterally. Her skin is warm and moist. She has a decreased level of thyroid-stimulating hormone and an increased level of thyroid-stimulating immunoglobulin.\n\nOf the following, the BEST next step in the management of this patient is", "options" : "[\"administration of methimazole\", \"administration of nonsteroidal anti-inflammatory drugs\", \"administration of propylthiouracil\", \"radioactive iodine therapy\", \"thyroidectomy\"]", "explanation" : "The patient in this vignette has hyperthyroidism given her history, physical examination results, and laboratory evaluation results. Fatigue and hair loss, although nonspecific, can be seen with hyperthyroidism. Physical examination reveals tachycardia, hypertension with a wide pulse pressure, skin that is warm to touch and moist, and an enlarged thyroid with a bruit noted on auscultation. Her laboratory evaluation reveals a decreased thyroid-stimulating hormone level and an increased thyroid-stimulating immunoglobulin level, which are consistent with Graves disease. Most patients (90%) with Graves disease will have a positive thyroid-stimulating immunoglobulin test result. Thyroid peroxidase antibodies may be measured and are present in 10% of patients with Graves disease as well as in patients with autoimmune thyroiditis. Additionally, liver function tests and a complete blood cell count are often performed in the initial evaluation to help monitor adverse effects of therapy.\n\nTherapies for children with Graves disease include antithyroid drugs (methimazole), radioactive iodine therapy, and thyroidectomy. The first-line therapy for children with Graves disease is methimazole. Propylthiouracil has an increased risk of hepatotoxicity and is reserved for individuals with allergy or adverse reactions to methimazole or complications related to surgery or radioactive iodine therapy. Because some children experience disease remission with medication, surgery and radioactive iodine therapy are not first-line therapies, although many pediatric patients will ultimately require these interventions. Nonsteroidal anti-inflammatory medications have no specific role in the treatment of Graves disease.\n\nHyperthyroidism can cause tachycardia, increased cardiac output, and hypertension with a wide pulse pressure because of a decrease in peripheral vascular resistance. Palpitations and arrhythmias (such as atrial fibrillation) can occur, although arrhythmias are much more common in adults than children.\n\nGiven the sinus tachycardia seen with Graves disease and hyperthyroidism in general, β-blockers are often initiated at diagnosis and continued until the methimazole takes effect, which may be several weeks. β-Blockers such as propranolol are indicated in patients with a heart rate greater than 100 beats/min, palpitations, hypertension, or tremors. Neurologic symptoms associated with hyperthyroidism can also improve with β-blockers.\n\nPREP Pearls\n• The therapies for children with Graves disease include antithyroid drugs (methimazole as the first-line treatment), radioactive iodine therapy, and thyroidectomy.\n• Hyperthyroidism can cause persistent sinus tachycardia.\n• β-Blockers are often initiated at the time of diagnosis of hyperthyroidism.\n• β-Blockers such as propranolol are indicated in patients with a heart rate greater than 100 beats/min, palpitations, hypertension, or tremors.\n\nABP Content Specifications(s)\n• Recognize the role of hyperthyroidism in persistent sinus tachycardia\n\nSuggested Readings\n• Sills I. Hyperthyroidism. Pediatr Rev. 1994;15(11):417–421. doi: http://pedsinreview.aappublications.org/content/15/11/417 .\n• Srinivasan S, Misra M. Hyperthyroidism in children. Pediatr Rev. 2015;36(6):239–248. doi: http://dx.doi.org/10.1542/pir.36-6-239."}
{"id" : 846, "question_text" : "You are called in to see a female neonate born at term to a 34-year-old gravida 2, para 2 woman following a reportedly uneventful pregnancy, although the mother had only 2 prenatal visits at 30 and 36 weeks' gestation. The neonate's birth weight is 2.3 kg (below the 5th percentile), and her Apgar scores were 7 and 9 at one and five minutes, respectively. On examination, her head circumference is at the fifth percentile. Facial features are characteristic. Examination is also remarkable for bilateral radioulnar synostosis, small distal phalanges, fifth-fingernail hypoplasia, and a soft systolic cardiac murmur. Echocardiogram demonstrates a small muscular ventricular septal defect. Of the following, the MOST likely diagnosis for this infant is", "options" : "[\"Angelman syndrome\", \"fetal alcohol syndrome\", \"trisomy 21\", \"velocardiofacial syndrome\", \"Williams syndrome\"]", "explanation" : "Preferred Response: B\nThe infant described in the vignette has clinical features suggestive of fetal alcohol syndrome (FAS). In addition to the classic facial features of short palpebrae, long smooth philtrum, and thin upper lip, she also has radioulnar synostosis and a ventricular septal defect (VSD), which are consistent with the suspected diagnosis. Because these clinical features may be seen in children with no prenatal exposure to alcohol, in order to confirm this diagnosis, an accurate and honest prenatal history regarding maternal alcohol consumption needs to be obtained.\n\nOther common features of FAS include a \"hockey stick\" upper palmar crease, \"railroad track\" upper helix of the ear, ptosis, strabismus, hypoplastic nails, short fifth digits, fifth-finger clinodactyly, and camptodactyly. Relatively common birth defects include cardiac defects, radioulnar synostosis, vertebral segmentation defects, renal anomalies, optic nerve hypoplasia, hearing loss, and pectus deformities. Most children with FAS will have prenatal or postnatal growth retardation and ultimately will develop microcephaly (with head circumference <10th percentile for age). Developmental delays or cognitive deficits will be noted over time. Children with FAS commonly demonstrate marked impairment in carrying out complex tasks, higher-level receptive and expressive language delays, and behavioral difficulties.\n\nNewborns with Angelman syndrome or Williams syndrome may not exhibit any abnormalities at birth but may have mild prenatal growth deficiency. Both of these conditions have facial and clinical features that evolve over time, which makes an early diagnosis less likely. Children with Angelman syndrome typically have postnatal development of microcephaly and onset of seizures, with very limited expressive language and later appearance of prognathism. Radioulnar synostosis is not seen in any of the other syndromes listed, whereas VSDs may be seen with some frequency in children with Down syndrome and velocardiofacial syndrome. Children with Williams syndrome are much more likely to have supravalvular aortic stenosis, although VSDs may also occur. Facial features associated with Williams syndrome that become more apparent over time include medial eyebrow flare, epicanthal folds, periorbital fullness, stellate iris pattern, anteverted nares, and full lips. Individuals with velocardiofacial syndrome have a high risk for cleft palate and abnormalities of the great vessels and, over time, may be noted to have a broad nasal root, long face, postnatal microcephaly, and slender fingers. The typical facial features in children with Down syndrome include a flat facial profile, epicanthal folds, upslanting palpebrae, Brushfield spots on the iris, small ears, and a protruding tongue.\n\nPREP Pearls\n• Infants with fetal alcohol syndrome have typical facial features at birth and are at increased risk for cardiac defects (especially ventricular septal defects) as well as limb anomalies such as radioulnar synostosis.\n\nAmerican Board of Pediatrics Content Specification(s):\n• Recognize the physical features of fetal alcohol syndrome, and manage appropriately\n\nSuggested Reading:\n• Astley SJ. Comparison of the 4-Digit Diagnostic Code and the Hoyme Diagnostic Guidelines for Fetal Alcohol Spectrum Disorders. Pediatrics. 2006;118(4):1532-1545. doi:10.1542/peds.2006-0577\n• Gahagan S, Sharpe TT, Brimacombe M, et al. Pediatricians' Knowledge, Training, and Experience in the Care of Children With Fetal Alcohol Syndrome. Pediatrics. 2006;118(3):e657-e668. doi:10.1542/peds.2005-0516\n• Godel J; Canadian Paediatric Society. Canadian Paediatric Society statement: fetal alcohol syndrome. Paediatr Child Health. 2002;7(3):161-174\n• Hoyme HE, May PA, Kalberg WO, et al. A practical clinical approach to diagnosis of fetal alcohol spectrum disorders: clarification of the 1996 Institute of Medicine criteria. Pediatrics. 2005;115(1):39-47. doi:10.1542/peds.2004-0259\n• Landgren M, Svensson L, Stromland K, Gronlund MA. Prenatal alcohol exposure and neurodevelopmental disorders in children adopted from Eastern Europe. Pediatrics. 2010;125(5):e1178-e1175. doi:10.1542/peds.2009-0712"}
{"id" : 1161, "question_text" : "A 16-year-old high school football player presents to the pediatric emergency department with pain in his chest and left shoulder after an injury that he sustained during a football game, approximately 1 hour ago. While running and holding the football in his left arm, he was tackled from behind. The patient fell forward and landed forcefully on his chest, with the weight of the opposing player on top of him. At that time, he heard a \"cracking\" sound and immediately felt pain in his chest and left shoulder. In the emergency department, the boy is alert and fully oriented. He is very uncomfortable and is holding his hand over the left side of his chest. He tells you that it is difficult to breathe. His vital signs include a heart rate of 100 beats/min, respiratory rate of 24 breaths/min, blood pressure of 130/80 mm Hg, temperature of 37°C, and pulse oximetry of 96% on room air. On physical examination, his breath sounds are clear and equal bilaterally. The patient is taking shallow breaths because of pain, but is not in respiratory distress. He is tender to palpation over the left sternoclavicular junction, as well as over his left first rib, and you note bruising over these areas. He has no focal tenderness on examination of his left shoulder, but he refuses to move his left shoulder due to pain. You find no evidence of trauma to his head and his cervical spine is nontender to palpation. The remainder of his physical examination findings, including a full neurologic examination, is unremarkable. An electrocardiogram reveals sinus tachycardia with no other abnormality. You administer an intravenous analgesic and order plain radiographs of the patient's chest and left shoulder, which reveal a non-displaced fracture of his left first rib. His left shoulder radiograph reveals no fracture or dislocation. He continues to complain of severe pain over his left sternoclavicular joint and subjective dyspnea, and refuses to move his left shoulder due to pain. His vital signs are unchanged.\nOf the following, the BEST next step in his evaluation is", "options" : "[\"bone scan\", \"computed tomography of the chest\", \"echocardiography\", \"no further imaging\", \"plain radiographs of the ribs\"]", "explanation" : "The teenager in the vignette presents with pain in his left chest, subjective dyspnea, and refusal to move his left shoulder after sustaining a significant blunt traumatic force to his anterior chest wall during a football game. Plain radiography reveals that he has a fracture involving his left first rib. Computed tomography (CT) of the chest is the next best step in his evaluation.\nPhysical examination and/or plain radiography are sufficient to identify many chest wall injuries in children; however, CT of the chest may be needed to evaluate for bony injuries that are not apparent on plain radiography (eg, posterior sternoclavicular fracture or dislocation) and to investigate for associated intrathoracic injuries (eg, injury to the airway or great vessels). In general, stable children with fractures to the first rib and sternum should be evaluated with CT of the chest because of the high risk for associated intrathoracic injury.\nIt is important for all pediatric providers to know how to appropriately evaluate children who have sustained chest wall trauma. Pediatric chest wall injuries generally arise from blunt trauma to the thorax. The forceful mechanism of the trauma, such as a motor vehicle accident, can cause additional serious injuries. All children with chest wall injuries should undergo a full physical examination, beginning with assessment of their airway, breathing, circulatory, and neurologic status, and then progressing to a head-to-toe secondary survey after any life-threatening conditions have been addressed. It is important for clinicians to keep in mind that, because the rib cages of children are generally more pliable than those of adults, traumatic forces may be transmitted to their intrathoracic organs; thus, significant intrathoracic injuries such as pulmonary contusion may occur even in the absence of injury to the chest wall structures.\nChest wall injuries may include fractures of the ribs, sternum, clavicles, and scapulae, with rib fractures being the most common in children. Multiple rib fractures may result in flail chest, an uncommon but very serious injury that can cause respiratory insufficiency from a compromise to the structural integrity of the chest wall. Fractures of the upper ribs (especially the first and second), sternum, and scapulae should lead clinicians to consider more serious intrathoracic injuries such as pulmonary contusion, injury to the intrathoracic vessels, cervical spine injuries, and injuries to the trachea or esophagus. This concern is due to the large amount of force generally required to fracture these bones, given their protected anatomic positions.\nPosterior dislocation of the sternoclavicular joint or posterior displacement of a medial clavicle fracture are chest wall injuries that are relatively rare, but do occur in children. These injuries can present with very subtle physical examination findings and are often not apparent on plain radiography. Furthermore, they carry the risk of associated injuries to intrathoracic structures including the great vessels, esophagus, and trachea. Physical examination findings in children with posterior sternoclavicular joint dislocations may include pain localized to the sternoclavicular joint, a palpable gap at the joint (which can be subtle), and swelling at the medial end of the clavicle. Clinical symptoms may include difficulty swallowing, shortness of breath, hoarseness, and inability to move the shoulder on the affected side. CT of the chest is the imaging study of choice for these injuries.\nA bone scan may identify fractures that are not apparent on plain radiography, particularly stress fractures, but would not be helpful in excluding injury to intrathoracic structures.\nEchocardiography certainly has a role in the evaluation of children who have sustained chest wall trauma, as a means of excluding cardiac injury. Echocardiography is indicated for those with abnormal electrocardiograms, ectopy, abnormal cardiac enzyme levels, or other evidence of cardiac injury after thoracic trauma. The boy in the vignette does not meet any of these criteria.\nMany children with uncomplicated chest wall injuries, including those with simple clavicle fractures and fractures to the middle or lower ribs will not require further evaluation beyond plain radiography. However, the boy in the vignette has a clinical picture suggestive of a posterior sternoclavicular joint dislocation, and CT is indicated to evaluate for this injury. Furthermore, CT of the chest would be indicated in this patient because of the presence of the rib fracture.\nFinally, plain radiography of the ribs would not aid in the evaluation for associated intrathoracic injuries or dislocation of the posterior sternoclavicular joint, which is indicated for the boy in the vignette. Standard anterior-posterior and lateral radiographs of the chest are sufficient for detecting the majority of rib fractures, therefore dedicated plain radiographs of the ribs are not needed in the evaluation of most patients.\n\nPREP Pearls\n\nThe thoracic cages of children are more pliable than those of adults, resulting in the transmission of traumatic forces to the intrathoracic organs; significant intrathoracic injuries may occur even in the absence of injury to the chest wall.\n\nComputed tomography (CT) of the chest may be indicated to evaluate for injuries to chest wall structures that are not apparent on plain radiography (eg, posterior sternoclavicular fracture dislocations) and to investigate for associated intrathoracic injuries (eg, airway or great vessels).\n\nIn general, stable children with fractures to the first rib or sternum should be evaluated with CT because of the high risk for associated intrathoracic injury.\n\nABP Content Specifications(s)\n\nPlan the appropriate evaluation of a child who has experienced chest wall trauma"}
{"id" : 1781, "question_text" : "A 4-year-old girl is brought to your office for evaluation of recurrent crops of pruritic red bumps noted over the past 6 months. Individual lesions resolve in 7 to 10 days, leaving areas of hyperpigmentation. The girl has no other symptoms and takes no medications. She appears well and is afebrile. There are multiple hyperpigmented macules and papules that are concentrated on the extremities (Item Q258). Of the following, the MOST likely diagnosis is", "options" : "[\"atopic dermatitis\", \"Gianotti-Crosti disease\", \"papular urticaria\", \"scabies\", \"urticaria\"]", "explanation" : "The girl in this vignette has a recurring eruption composed of erythematous papules that last 7 to 10 days. Over time, the lesions become hyperpigmented. These findings are most consistent with a diagnosis of papular urticaria. Atopic dermatitis has a chronic course marked by intermittent flares and, in persons of color, the rash may be papular (Item C258A ). However, the presence of large individual papules, as exhibited by the girl in this vignette, would be atypical of atopic dermatitis. In atopic dermatitis, the involvement of the antecubital and popliteal fossae, not the extensor surfaces of the extremities, is expected. Gianotti-Crosti disease (papular acrodermatitis of childhood) is a unique viral exanthem composed of erythematous, edematous, monomorphous papules symmetrically distributed on the face, buttocks, and extensor surfaces of the extremities. Papules may coalesce to form large plaques (Item C258B ). The trunk is usually spared. The eruption may last 8 to 12 weeks but is not recurrent. Scabies is characterized by erythematous papules concentrated in flexural areas (interdigital webs, wrist flexures, axillae). The eruption of scabies would not be expected to recur unless the child had become reinfested. Urticaria appears as wheals, often in a variety of shapes. Although the condition may become chronic, individual lesions last less than 24 hours (usually < 2 to 3 hours).\n\nPapular urticaria is a reaction to bites from insects, especially fleas, mosquitoes, mites, and bedbugs. Although the pathophysiology is incompletely understood, it is postulated that the biting insect deposits an antigen that is disseminated hematogenously. A type 1 hypersensitivity reaction ensues, leading to the formation of lesions at locations distant from the bite. However, evidence exists supporting a component of delayed-type hypersensitivity (type IV). Papular urticaria occurs most often in children 2 to 10 years of age and is especially prevalent during spring and summer months. The eruption is characterized by grouped erythematous papules or vesicles on exposed surfaces of the extremities. The lesions are intensely pruritic. Some papules exhibit a central punctum (the site of a bite), but other papules do not have a central punctum and are caused by a hypersensitivity reaction. Lesions may become eroded and crusted from scratching or hyperpigmented as the result of the inflammatory process. The treatment of papular urticaria is symptomatic. Because lesions are pruritic, a mid- to high-potency topical corticosteroid may be applied to individual lesions twice daily. If needed, an oral antihistamine can be administered. A central component of management is prevention of insect bites. Families with pets should initiate flea-control measures. For outdoor play, children may wear protective clothing and apply insect repellent to exposed skin surfaces. The prognosis for papular urticaria is excellent. Children ultimately \"outgrow\" the condition before adolescence.\n\nPREP Pearls\n• Papular urticaria is a reaction to bites from insects, especially fleas, mosquitoes, mites, and bedbugs.\n• Papular urticaria occurs most often in children 2 to 10 years of age and is especially prevalent during spring and summer months.\n• The papular urticaria eruption is characterized by grouped erythematous papules or vesicles located on exposed surfaces of the extremities. Some papules exhibit a central punctum (the site of a bite), but other papules do not have a central punctum and are produced by a hypersensitivity reaction.\n\nABP Content Specifications(s)\n• Recognize the clinical manifestations of papular urticaria\n• Know the cause of papular urticaria\n\nSuggested Readings\n• Hernandez RG, Cohen BA. Insect bite-induced hypersensitivity and the SCRATCH principles: a new approach to papular urticaria. Pediatrics. 2006;118(1):e189–e196. http://dx.doi.org/10.1542/peds.2005-2550.\n• Section on Dermatology, American Academy of Pediatrics. Insect bites and papular urticaria. In: Mancini AJ, Krowchuk DP, eds. Pediatric Dermatology: A Quick Reference Guide. 3rd ed. Elk Grove Village, IL: American Academy of Pediatrics; 2016:295–303."}
{"id" : 1400, "question_text" : "You are seeing a 2-year-old girl with trisomy 18 and severe neurologic compromise for a health supervision visit. She has had 12 documented episodes of pneumonia, 6 of which have required hospitalization. She has a history of recurrent pneumonia with a clear recurrence pattern. A prior necrotizing pneumonia involving the right lower lobe resulted in pneumatocele formation. The girl's nutrition consists of oral feedings of milk, juice, and pureed baby foods from a bottle and spoon. You suspect that she is suffering from complications of chronic pulmonary aspiration and order a fiberoptic endoscopic evaluation of swallowing. The study reveals direct aspiration with saliva and all food consistencies without an associated cough response. On physical examination, the girl is hypotonic and in no acute distress. Her respiratory rate is 26 breaths/min. There is copious saliva in the oral cavity and posterior oropharynx. The girl frequently gags and chokes on her secretions. Auscultation of her lungs reveals coarse breath sounds and transmitted upper airway noise. Her extremities are well perfused, without cyanosis or edema. Examination of the heart and abdomen is within normal limits. You discuss the girl's care plan with her parents, including advance directives. They request that all potential care options be continued for their daughter. Of the following, the management option MOST likely to prevent pulmonary aspiration in this girl is", "options" : "[\"feeding via gastrostomy tube\", \"glycopyrrolate 3 times daily\", \"laryngotracheal separation\", \"salivary gland ligation/excision\", \"tracheostomy\"]", "explanation" : "The girl in the vignette has had multiple episodes of pneumonia complicated by respiratory failure, necrotizing pneumonia, and pneumatocele. In children with neurodevelopmental compromise and muscular weakness or discoordination, silent aspiration is common and injury may occur in the absence of overt symptoms. The risk of scarring, bronchiectasis, and loss of pulmonary function in children affected with chronic pulmonary aspiration is significant. In conversations with caregivers, the risk of continued aspiration events should be reviewed and care directives discussed.\n\nAspiration may occur directly with oral feedings, in a retrograde manner during episodes of gastroesophageal reflux, or from an inability to manage salivary secretions. A patient with aspiration may be evaluated using various methods. The study chosen in the vignette, a fiberoptic endoscopic evaluation of swallowing, uniquely allows the otolaryngologist and speech and language pathologist to directly visualize the path of oral secretions and/or feedings. During the period of inspection, pooling of material in the valleculae, effectiveness of clearance with swallowing, and any aspiration events can be documented, as well as the presence or absence of associated cough.\n\nThe most definitive preventive treatment for chronic pulmonary aspiration is a laryngotracheal separation (LTS) procedure. In appropriate cases, an LTS can prevent aspiration pneumonia, improve respiratory stability, and thereby allow a child to be cared for at home. In LTS, the digestive and respiratory tracts are separated. A tracheoesophageal diversion connects the upper trachea to the cervical segment of the esophagus, while the proximal trachea is closed with formation of a blind tracheal pouch. A tracheostomy is required, providing a connection to the mid/distal trachea. Largely dependent on the degree of antecedent airway and pulmonary injury, surgically treated patients may or may not require respiratory support in the form of supplemental oxygen or chronic mechanical ventilation. It is important to inform caregivers that children with LTS will no longer be able to phonate. Education related to tracheostomy care will also be required.\n\nSeveral alternate management options are available for patients with chronic aspiration, but none are as effective as LTS in preventing all aspiration events. If the aspiration events occur primarily with direct aspiration of oral feedings, an alternate route of feeding, such as a gastrostomy tube, may be pursued. However, this will not prevent aspiration and pulmonary damage resulting from reflux events or salivary aspiration.\n\nGlycopyrrolate or scopolamine may be effective in reducing the burden of salivary aspiration through their anticholinergic effects, but their side effect profiles must be considered and tolerance must be closely monitored. This treatment will not affect the risk of oral feeding aspiration or retrograde reflux aspiration. A complication of Nissen fundoplication may be pooling of secretions from the oral cavity in the distal esophagus, causing gagging and secondary salivary aspiration.\n\nSalivary gland ligation and excision procedures are effective in preventing episodes of pneumonia caused by aspiration of oral secretions. Injection of the submandibular or parotid glands with botulinum toxin may also be considered. With this approach, as many as 88% of patients may see a decrease in saliva production, but side effects include parotitis and viscous secretions, which may be difficult to clear from the airway. Neither treatment will prevent retrograde aspiration events.\n\nA tracheostomy allows direct access to the airway for suctioning and pulmonary toileting. However, a tracheostomy may contribute to aspiration events and can be expected to prevent a functional swallow as a result of decreased laryngeal sensation, decreased elevation of the larynx, and an inability to elevate subglottic pressure as normally occurs during swallowing and before effective coughing.\n\nPREP Pearls\n• Although multiple options exist for the management of chronic pulmonary aspiration, a laryngotracheal separation is the most definitive approach.\n• Salivary aspiration may continue to cause pulmonary disease after direct and retrograde aspiration have been optimally addressed.\n• A fiberoptic endoscopic evaluation of swallowing offers direct visualization of the airway with regard to salivary and other aspiration events.\n\nABP Content Specifications(s)\n• Understand the effect of a tracheostomy on aspiration\n\nSuggested Readings\n• Chida I, Tamura K, Nakagawa S, Ando M, et al. Clinical outcomes of tracheoesophageal diversion and laryngotracheal separation in neurologically impaired children. Auris Nasus Larynx. 2013;40(4):383-387. doi: http://dx.doi.org/10.1016/j.anl.2012.11.001.\n• Cook SP. Laryngotracheal separation in neurologically impaired children: long-term results. Laryngoscope. 2009;119(2):390-395. doi: http://dx.doi.org/10.1002/lary.20044.\n• El-Hakim H, Richards S, Thevasagayam MS. Major salivary duct clipping for control problems in developmentally challenged children. Arch Otolaryngol Head Neck Surg. 2008;134(5):470-474. doi: http://dx.doi.org/10.1001/archotol.134.5.470.\n• Hara H, Hori T, Sugahara K, Ikeda T, Kajimoto M, Yamashita H. Effectiveness of laryngotracheal separation in neurologically impaired pediatric patients. Acta Otolaryngol. 2014;134(6):626-630. doi: http://dx.doi.org/10.3109/00016489.2014.885119."}
{"id" : 202, "question_text" : "A 2-year-old boy in your practice has clinical signs of rickets; laboratory testing reveals a low phosphorus, normal calcium, elevated alkaline phosphatase, low-normal 1,25-dihydroxyvitamin D3, and high normal parathyroid hormone values. You refer him to a pediatric endocrinologist who suspects that he has X-linked dominant hypophosphatemic rickets. Of the following, the history that is MOST suggestive of the X-linked dominant form of hypophosphatemic rickets is that", "options" : "[\"his father has significant short stature, genu varum, and recurrent dental abscesses\", \"his mother and maternal aunt have mild short stature and his maternal grandfather has severe genu varum\", \"his parents are second cousins (their grandfathers are brothers)\", \"neither parent has signs of rickets, but his mother's brother and one of her maternal uncles were diagnosed with rickets\", \"the number of affected males in the extended family history is approximately twice that of affected females\"]", "explanation" : "Hypophosphatemic rickets is a relatively common condition that can be transmitted in an X-linked dominant, an autosomal dominant, or rarely an autosomal recessive pattern. A family history most suggestive of X-linked dominant inheritance includes mild short stature in the boy's mother and aunt, with more severe symptoms in his maternal grandfather (Item C94). As with a few other X-linked dominant conditions, X-linked dominant hypophosphatemic rickets (XLDHR) has variable phenotypic features, and affected men tend to have more severe findings than affected women. An affected father could not pass on this X-linked condition to his son because he contributes his Y and not his X chromosome to a boy at conception. An affected father and son would be suggestive of autosomal dominant transmission. Consanguinity (parental relatedness) would increase concerns about an autosomal recessive form of hypophosphatemic rickets but would not be seen with XLDHR. Affected males related through clinically unaffected or normal females are suggestive of X-linked recessive inheritance, which has not been described for hypophosphatemic rickets. In reviewing extensive or large pedigrees with X-linked dominant traits, the number of affected females is generally twice that of affected males because mothers can have both affected sons and daughters, and affected males can only have affected daughters.\n\nXLDHR is typically associated with short stature; frontal bossing; genu varum; recurrent dental abscesses; and low phosphate (due to renal phosphate wasting), elevated alkaline phosphatase, normal serum calcium, and normal to low-normal serum 1,25-dihydroxyvitamin D concentrations. It is caused by defects in the phosphate-regulating endopeptidase homolog, X-linked gene (PHEX), and treatment consists of oral phosphate supplements and supplemental 1,25 dihydroxy vitamin D. Serum calcium, phosphate, and alkaline phosphate should be monitored over time because some children receiving 1,25-dihydroxyvitamin D develop hypercalcemia or nephrocalcinosis. Recent studies have suggested that prepubertal use of human growth hormone for patients who have XLDHR may improve final adult height, but concerns remain about disproportionate truncal growth compared with long bone growth. Orthopedic care is also important to address leg bowing, and aggressive dental surveillance may be helpful in light of the enamel and dentin defects identified in affected patients.\n\nContent Specifications: Recognize the clinical features associated with an X-linked dominant disorder"}
{"id" : 247, "question_text" : "A 17-year-old boy is referred to you by his school because of obesity. He has been in good health, except for a rapid increase in weight for the past 2 years. During that time, his parents have noted a change in his behavior, and they are concerned about his poor school performance. Because of declining grades and some difficulty in concentrating on his studies, the young man recently was diagnosed with attention-deficit disorder and has been treated with methylphenidate. Physical examination demonstrates a well-developed, obese adolescent, whose height is 170 cm and weight is 80 kg. The only other findings of note are a firm liver edge palpated 2 cm below the right costal margin and a palpable spleen tip. Initial laboratory results include: Hemoglobin, 13.0 g/dL (130 g/L); Aspartate aminotransferase, 60 units/L (normal, 10 to 30 units/L); Alanine aminotransferase, 55 units/L (normal, 10 to 30 units/L); Alkaline phosphatase, 280 units/L (normal, 10 to 140 units/L); Bilirubin, 3.5 mg/dL (59.9 mcmol/L); Erythrocyte sedimentation rate, 30 mm/hr. Because of these results, you obtain the following additional studies: Hepatitis A antibody, negative; Hepatitis B surface antibody, positive; Hepatitis B surface antigen, negative; Hepatitis C antibody, negative; Alpha-1-antitrypsin, 220 mg/dL (40.5 mcmol/L) (normal, 150 to 350 [27.6 to 64.4 mcmol/L]); Serum ceruloplasmin, 22 mg/dL (220 mg/L) (normal, 20 to 60 [200 to 600 mg/L]). Of the following, the MOST appropriate next study is", "options" : "[\"abdominal ultrasonography\", \"Epstein-Barr virus titers\", \"liver biopsy\", \"serum copper measurement\", \"slit lamp examination\"]", "explanation" : "The adolescent boy described in the vignette presents with obesity, behavioral changes, and elevations in transaminases. Results of laboratory studies are consistent with immunity to hepatitis B and no evidence of either hepatitis A or C infection. In addition, the alpha-1-antitrypsin value is normal, serum ceruloplasmin is just above the lower limit of normal, and erythrocyte sedimentation rate (ESR) is 30 mm/hr. Based on these clinical and biochemical data and the knowledge that serum ceruloplasmin is an acute-phase reactant that may be increased in the presence of an elevated ESR, Wilson disease (WD) must be ruled out. This may be achieved by performing both a slit lamp examination to determine the presence of Kayser-Fleischer (K-F) rings, the manifestation of copper deposition in Descemet membrane of the cornea, and a 24-hour urine copper determination. The serum copper concentration is not of diagnostic significance for WD in this clinical setting, and the presence of K-F rings, in conjunction with a 24 hour urine copper value of greater than 40 µg is sufficient to diagnose WD. A liver biopsy (for copper content) may not be required. Abdominal ultrasonography may demonstrate hepatosplenomegaly in this case but will provide no further diagnostic clues. Assessment for other possible viral causes, including Epstein-Barr virus infection, is not indicated unless WD is excluded.\n\nWD, also known as hepatolenticular degeneration, was described initially in 1912 by Kinnear Wilson. If untreated, WD is a fatal disorder characterized by chronic liver disease leading to cirrhosis along with progressive neurologic deterioration. It is inherited in an autosomal recessive pattern and results from an abnormal ATP7B gene on chromosome 13. This gene encodes a metal-transporting P-type adenosine triphosphatase (ATPase), which is expressed primarily in hepatocytes and functions in the transmembrane transport of copper. Absent or reduced function of ATP7B protein resulting from this genetic abnormality leads to reduced hepatocellular excretion of copper into bile and the failure to incorporate copper into the copper-carrying protein, ceruloplasmin. Excessive hepatic copper accumulation causes hepatocellular injury that leads to inflammatory and, ultimately, cirrhotic changes. Eventually, copper is released into the bloodstream and deposited in other organs, including the brain, kidneys, and cornea (hence the appearance of K-F rings).\n\nThe clinical presentation of WD may be subtle or overt, depending upon the age at presentation and extent of copper-induced organ damage. During childhood and early adolescence, other than cases diagnosed on the basis of family history (16% in one series), the overwhelming majority of patients present with evidence of hepatocellular dysfunction without neurologic sequelae. In most pediatric series, the mean age of presentation is around the end of the first decade. Children who have WD are usually evaluated because of hepatomegaly (82% of patients in one recent series) or persistent transaminase elevations, although in one series from the United Kingdom, acute liver failure was the presenting sign in almost 50% of patients. Among older adolescents and adults, psychiatric disturbances and neurologic dysfunction become apparent, with such signs found in 10% to 40% of reported cases by the end of the second decade. With increasing age, the psychoneurologic picture tends to predominate.\n\nFor the patient who presents with signs of persistent hepatic dysfunction, irrespective of associated psychiatric or neurologic signs and symptoms, further evaluation follows an established practice guideline that is approved by the American Association for the Study of Liver Diseases. The diagnostic algorithm is based on three initial determinations: 1) K-F rings by slit lamp examination, 2) serum ceruloplasmin, and 3) 24-hour urine copper excretion. The diagnosis of WD is established in patients who present with K-F rings, a serum ceruloplasmin less than 20 mg/dL (200 mg/L), and a 24-hour urine copper excretion of greater than 40 µg. For patients who do not meet the diagnostic criteria, a WD diagnosis may be confirmed by determining hepatic copper content on a percutaneous liver biopsy sample. A hepatic copper concentration of greater than 250 µg/g dry weight is diagnostic for WD.\n\nOnce the diagnosis is confirmed, treatment with an appropriate copper-chelating agent should begin immediately. WD represents the uncommon situation of a metabolic disease for which a specific pharmacologic therapy, if begun early in the disease course, is associated with complete symptom resolution, normalization of laboratory values (except for serum ceruloplasmin), and an excellent long-term prognosis. For patients who have hepatic disease, penicillamine, the first copper chelator to be successfully used for the treatment of WD, is an effective agent. However, penicillamine has been supplanted by trientine as the drug of first choice. Trientine is also indicated as primary therapy for patients who have neurologic involvement with or without WD hepatopathy. When patients have been stabilized, with normal serum transaminase values, zinc, which interferes with intestinal copper absorption, may be used either alone or in combination with trientine, accompanied by a low copper-containing diet.\n\nAmerican Board of Pediatrics Content Specification(s): Recognize the signs and symptoms of Wilson disease"}
{"id" : 1207, "question_text" : "A 4-month-old infant presents to the emergency room for evaluation because her parents feel that she has been \"moving her legs less\" over the past 2 weeks. On physical examination, she is a happy infant in no distress who smiles at her mother and grabs her hair. The only abnormal finding is flaccidity in her legs bilaterally. A complete blood cell count, complete metabolic panel, creatine kinase, and coagulation profile are all unremarkable. Of the following, the MOST appropriate course of action is to", "options" : "[\"admit her to the hospital for overnight observation and a neurology consult\", \"arrange an outpatient appointment with a neurologist in the morning\", \"instruct her to follow-up with her pediatrician in 1 week\", \"recommend a physical therapy evaluation\", \"obtain an immediate magnetic resonance image of the thoracic and lumbar spine\"]", "explanation" : "It can be difficult for parents to identify lower extremity weakness in infants who are not yet mobile and it is imperative that examining physicians identify motor abnormalities quickly. The etiology of the weakness must be also quickly ascertained. If an infant or child is seen in an office setting and found to have new onset weakness, they should be referred to the emergency department. Once there, imaging of the spine at the level of the suspected defect must be obtained rapidly. Injury to the spinal cord may be caused by damage intrinsic to the cord (eg, myelitis) or extrinsic to the cord in the form of spinal cord compression from a hematoma, abscess, or a tumor. Spinal cord compression is a true medical emergency and requires immediate action. The longer there is compression and nerve dysfunction, the greater the likelihood that nerve damage will be permanent. As the infant in the vignette has weakness in her lower extremities but not her upper extremities, cross-sectional imaging of the thoracic and lumbar cord is required. Magnetic resonance imaging (MRI) is the imaging modality of choice when available on an immediate basis. When MRI is not immediately available, computed tomography should be performed.\nWhile many types of childhood cancer can present with spinal cord compression in early childhood, the most common include neuroblastoma and tumors of the central nervous system. Neuroblastoma is an embryonal tumor of the peripheral nervous system and can arise in the adrenal gland or in any of the sympathetic ganglia. It commonly arises in a paraspinal ganglion and tends to track into the spinal canal through the neural foramina. This results in the \"dumbbell\" sign (Item C170). While neuroblastoma rarely invades the spinal cord, it can cause severe compression (Item C170), where the cord is not visible at all in the thoracic canal.\nOnce spinal cord compression has been identified, decompression must occur quickly. Depending on the etiology of the compression, decompression can occur by surgical laminectomy or emergent chemotherapy. If the spinal cord compression is caused by a tumor, dexamethasone should be initiated immediately, as a reduction in inflammation can help reduce the pressure on the spinal cord. If a tumor is noted, a pediatric oncologist should be emergently consulted to determine the most appropriate method for cord decompression.\nAdmission for observation and a neurological evaluation are appropriate in this circumstance, but only after imaging has been performed and spinal cord compression has been ruled out. Discharge from the emergency room without imaging would not be the most appropriate management in this scenario. While a physical therapy evaluation and program would be appropriate to regain strength in the legs, it should come only after the diagnosis and management plan have been initiated.\nPREP Pearls\n New onset weakness is an emergency that requires immediate cross-sectional imaging of the spinal cord from the suspected level of injury down.\n If cord compression is found to be caused by a tumor, dexamethasone should be initiated and an emergent pediatric oncology consult should be obtained.\n Spinal cord decompression can be achieved surgically with a laminectomy, or medically with chemotherapy or radiation, depending on the suspected cause of the compression.\nABP Content Specifications(s)\n Recognize the clinical findings associated with spinal cord compression (eg, from a tumor, from myelopathy), and the need for prompt evaluation"}
{"id" : 1563, "question_text" : "You are seeing a 13-month-old boy who is new to your practice for a health supervision visit. The boy recently moved with his family to the rural area where you practice, where his parents have started a strawberry farm. They are currently living in and renovating a farmhouse that was built in 1915. Both their farm and home are supplied with well water. The entire family eats a variety of fresh fruits and vegetables that are grown on their farm. The child's parents report that he has been very healthy, with no significant medical history. He takes no medications and has no allergies. His immunizations are up to date. His growth and development have been progressing normally. He is breastfed and eats some solid foods, though his mother states that he is a \"picky\" eater. He does not attend daycare, and loves playing with his 7-year-old twin sisters and 13-year-old brother (who are all healthy and developing normally according to the parents). In your office, the boy is well-appearing and active. His growth parameters are normal for his age, and findings of a complete physical examination are unremarkable. His parents ask you to discuss the potential health consequences of exposure to environmental toxins in this boy and his older siblings and how they can reduce the risk of exposure to environmental toxins for their children. Of the following, the MOST accurate information that you can provide to address the parents' concerns is that", "options" : "[\"all well water that this 13 month old and his siblings consume should be boiled to reduce their risk of exposure to both lead and nitrites\", \"the boy's mother should discontinue breastfeeding to reduce his risk of exposure to pesticides\", \"the boy's risk of developing asthma after exposure to air contaminants on the farm is the same as the risk of asthma development in his older siblings\", \"the boy's risk of exposure to foodborne pesticide residues is lower than the risk of exposure faced by his older siblings because he consumes less food\", \"the boy's risk of ingesting lead dust in his home environment is higher than the risk faced by his older siblings\"]", "explanation" : "The boy in the vignette lives with his family in an old farmhouse on a farm that is supplied by well water. The most accurate information to provide his parents about minimizing exposure of their children to environmental toxins is that the 13-month-old boy's risk of ingesting lead dust in their home environment is higher than the risk faced by his older siblings. This is because the developmentally normal exploratory behaviors at his age (frequent oral exploratory and hand-to-mouth behaviors), his higher respiratory rate, and his closer physical proximity to the ground and contaminated surfaces (eg, window sills) place him at higher risk for exposure to lead dust than older children.\n\nPediatric health care providers should know the age- and developmentally specific effects of exposure to a toxic substance in the environment, as well as how to obtain an appropriate exposure history. Children may be exposed to various environmental toxins on a daily basis in air, food, dust, soil, and on surfaces in their home, school, play, and occupational environments. The field of pediatric environmental health is an emerging and rapidly evolving one, with a growing body of literature that is helping to shed light on the effects of various environmental toxins on human health.\n\nAccording to the World Health Organization, nearly a third of the global burden of disease in children is due to environmental factors. Children have both increased exposure and increased physiologic vulnerability to environmental toxins. Physiologically, children differ from adults in organ system functioning, metabolic capabilities, physical size, and developmental abilities/behaviors. They are particularly susceptible to adverse outcomes from toxic exposures, given their rapid growth and development. Research focusing on the impact of children's unique characteristics on the harm caused to them by environmental toxins is ongoing.\n\nA free resource available to pediatric providers about the effects of environmental toxins on children is the Pediatric Environmental Health Specialty Units (PEHSU) National Classroom, a national resource endorsed by the American Academy of Pediatrics. This resource can be accessed online at http://www.pehsu.net. In addition, the US Department of Health and Human Services Agency for Toxic Substances and Disease Registry has developed an educational module on obtaining a pediatric environmental exposure history, which is available at http://www.atsdr.cdc.gov/csem/pediatric_history/docs/pediatric_history.pdf.\n\nAdvising this boy's parents to boil all well water consumed by their children to reduce exposure to both lead and nitrates would not be appropriate. In fact, both lead and nitrates become more concentrated in water that is boiled. In homes where there is a concern for contamination of water from lead pipes or lead pipe joints, families should be advised to discard \"first-draw\" water that has stood overnight in pipes (or to use it for a purpose other than drinking/cooking).\n\nThe boy's mother should not be advised to discontinue breastfeeding to reduce his risk of exposure to pesticides. Breastfeeding is encouraged as the optimal form of nutrition for infants by the American Academy of Pediatrics and the World Health Organization. Although certain lipophilic chemicals can be transmitted through breast milk and result in exposure to nursing infants, instances of harm occurring from chemicals transmitted through breast milk are very rare. The many benefits conferred by breastfeeding, such as enhanced immune function and growth factors that enhance brain development, generally outweigh the risks of exposure to environmental toxins through breast milk.\n\nThe boy's risk of developing asthma after exposure to air contaminants on the farm is likely higher than the risk of asthma development in his older siblings. Younger children have higher respiratory rates, resulting in higher weight-adjusted exposure to air contaminants. Furthermore, there is evidence that respiratory exposure to air contaminants during the first years of life have a greater influence on the incidence and severity of asthma compared with exposure later in life.\n\nThe statement that the boy's risk of exposure to foodborne pesticide residues is lower than that of his siblings because he consumes less food is incorrect. Young children have higher metabolic rates and generally consume a greater amount of food, water, and air per kilogram than older children. As a result, they have a greater exposure per kilogram of body weight to foodborne toxins. Furthermore, many young children have limited food preferences and may consume the same foods over relatively long periods.\n\nPREP Pearls\n• Children are particularly susceptible to adverse outcomes from toxic exposures because of their rapid rate of growth and development.\n• Children are at increased risk of harm from environmental toxins because of both increased exposure and increased physiologic vulnerability.\n• Young children have higher metabolic rates and generally consume a greater amount of food, water, and air per kilogram than older children. This can result in greater exposure per kilogram of body weight to foodborne and airborne toxins.\n\nABP Content Specifications(s)\n• Understand the effects of a patient's age when exposed to a toxic substance in the environment\n• Understand how to obtain a history of exposure to toxic substances in the environment\n\nSuggested Readings\n• Etzel RA, Balk SJ, eds; The American Academy of Pediatrics Council on Environmental Health. Handbook of Pediatric Environmental Health. 3rd ed. Elk Grove Village, IL: American Academy of Pediatrics; 2011.\n• Pediatric Environmental Health Specialty Units. http://www.pehsu.net. Accessed February 14, 2017.\n• US Health and Human Services Agency for Toxic Substances and Disease Registry. Environmental health and medical education: principles of pediatric environmental health. https://www.atsdr.cdc.gov/csem/csem.asp?csem=27&po=0. Accessed April 24, 2016.\n• US Health and Human Services Agency for Toxic Substances and Disease Registry. Pediatric environmental health toolkit. https://www.atsdr.cdc.gov/emes/training/index.html. Accessed April 24, 2016.\n• World Health Organization. Principles for evaluating health risks in children associated with exposure to chemicals. Environmental health criteria 237. http://whqlibdoc.who.int/publications/2006/924157237X_eng.pdf. Accessed April 23, 2016."}
{"id" : 1967, "question_text" : "An 8-year-old boy is evaluated because of his mother's concerns of challenging behaviors. She reports that he is unpleasant and refuses to do anything that is asked of him. He loses his temper easily and is argumentative. He deliberately annoys his siblings and blames them when he gets in trouble. He is disrespectful to his teacher and the staff at school.\n\nOf the following, the BEST next step is to recommend a treatment program that teaches the parent to", "options" : "[\"apply consequences for challenging behaviors\", \"delay response to unwanted behaviors\", \"increase positive reinforcement of disruptive behaviors\", \"vary response to undesired behaviors\"]", "explanation" : "The boy in this vignette has oppositional defiant disorder (ODD) for which psychosocial intervention via parent behavioral training is indicated. Behavioral management principles include applying consequences for challenging behaviors, providing immediate (not delayed) responses to the unwanted behaviors, decreasing (not increasing) positive reinforcement of disruptive behaviors, and providing a consistent (not varied) response to the behaviors. Punishments such as time-out, which removes the child from attention, interaction, and activities, or loss of privileges can also be used to decrease undesired behaviors. Positive reinforcement of desired compliant behavior through praise is generally preferred over negative consequences.\n\nTypically developing children may tantrum as toddlers and may exhibit defiance in adolescence. Children with temperamental characteristics of low frustration tolerance, high reactivity, and high emotional intensity are generally more easily annoyed, less compliant, and more likely to respond negatively than children with comparatively \"easy\" temperaments. However, children with \"difficult\" temperaments should not be diagnosed with ODD unless they demonstrate behaviors of irritability, anger, resentfulness, defiance, and vindictiveness that are developmentally inappropriate, persistent, intense, frequent, and/or functionally impairing. A diagnosis of conduct disorder (CD) would not be warranted unless there is aggression towards people or animals, destruction of property, theft, deceit, and/or disregard for societal rules.\n\nWhen evaluating a child for possible ODD or CD, input from the child, parents, and other adults (eg, teachers, coaches, other caregivers) should be considered. The specific behaviors, their triggers and duration, and resultant functional impairment should be ascertained. Standardized tools (eg, Child Behavior Checklist, Behavior Assessment System for Children, Youth Self-Report, Conners 3) can be used to determine when behaviors exceed developmental expectations and which mental health diagnoses may be present. Since ODD and CD commonly coexist with other mental health diagnoses, particularly attention-deficit/hyperactivity disorder, these conditions (eg, anxiety, depression, learning disorder, substance use disorder) should be considered during the initial evaluation.\n\nEvidence-based treatment of ODD and CD includes individual approaches (eg, problem-solving skills training) and family approaches (eg, parent management training in effective discipline and supervision). Parent training is emphasized in younger children whereas individual approaches are more often employed in adolescents. More than one mode of treatment may be used. When behavioral strategies are insufficient, treating coexisting conditions such as attention-deficit/hyperactivity disorder or anxiety with medication may lessen the oppositional behaviors.\n\nPREP Pearls\n\nFor disruptive behavior disorders (eg, oppositional defiant disorder, conduct disorder) parent training on behavior management including applying consequences for challenging behaviors is emphasized in younger children whereas individual approaches (eg, problem-solving skills training) are more often used in adolescents.\n\nSince oppositional defiant disorder and conduct disorder commonly coexist with other mental health diagnoses, particularly attention-deficit/hyperactivity disorder, these conditions (eg, anxiety, depression, learning disorder, substance use disorder) should be considered during the initial evaluation.\n\nWhen behavioral strategies are insufficient, treating coexisting conditions, such as attention-deficit/hyperactivity disorder or anxiety, with medication may lessen the oppositional behaviors.\n\nABP Content Specifications(s)/Content Area\n\nPlan the appropriate management of oppositional defiant or conduct disorder\n\nDifferentiate the findings associated with oppositional defiant or conduct disorder from those of temperamental variations\n\nPlan the appropriate evaluation of oppositional defiant or conduct disorder\n\nSuggested Readings\n\nBlair RJ, Leibenluft E, Pine DS. Conduct disorder and callous-unemotional traits in youth. N Engl J Med. 2014;371(23):2207-2216. doi: 10.1056/NEJMra1315612.\n\nSteiner H, Remsing L; Work Group on Quality Issues. Practice parameter for the assessment and treatment of children and adolescents with oppositional defiant disorder. J Am Acad Child Adolesc Psychiatry. 2007;46(1):126-141. doi: 10.1097/01.chi.0000246060.62706.af.\n\nZahrt DM, Melzer-Lange MD. Aggressive behavior in children and adolescents. Pediatr Rev. 2011;32(8):325-331. doi: 10.1542/pir.32-8-325."}
{"id" : 1808, "question_text" : "A local Emergency Medicine Services (EMS) agency calls to request medical direction for a 2-year-old girl they are preparing to transport to the emergency department. Fifteen minutes ago, the girl was injured when her teenage brother, while giving her a \"piggyback ride\" down the front steps, lost his balance and fell, pinning the girl's left leg underneath his body. Her aunt witnessed the event and immediately called 911, because the girl's left leg appeared deformed and she was screaming in pain. The girl is alert and interactive, but has been crying incessantly. Her left femur is significantly deformed and bruised, and she refuses to move her left leg. Her neurologic status appears to be intact. The EMS team has made 4 attempts to place an intravenous line without success. They are requesting your guidance on the most appropriate pain control regimen for this girl, who is being transported an approximately 30-minute drive from her home. You advise the EMS team to immobilize the girl's left lower extremity. Of the following, the BEST next step in this girl's management is to provide", "options" : "[\"intranasal fentanyl\", \"oral ibuprofen\", \"oral 25% sucrose solution\", \"distraction techniques during transport\"]", "explanation" : "The girl in the vignette has significant pain and distress secondary to a displaced femur fracture as well as numerous unsuccessful attempts to secure intravenous access. The best next step in management is to provide intranasal fentanyl for pain.\n\nAcute pain affects many children, arising as a result of injury, illness, and/or necessary medical procedures. However, it is often inadequately assessed and treated. It is imperative that pediatric providers understand the appropriate use of pharmacologic and nonpharmacologic pain management modalities.\n\nTo provide guidance to practitioners, the American Academy of Pediatrics Committee on Pediatric Emergency Medicine and the Section on Anesthesiology and Pain Medicine published a clinical report on relieving pain and anxiety in pediatric patients in Emergency Medical Systems in 2012. This report emphasizes the importance of timely administration of analgesia to children with acute pain and anxiety, because this \"affects the entire emergency medical experience and can have a lasting effect on a child's and family's reaction to current and future medical care\" (Fein et al).\n\nFor children with severe pain who are seen in the emergency department and prehospital settings, optimal pain management requires rapid pain assessment and administration of a systemic opioid pain medication. Administration may occur through various routes, including transmucosal or intravenous. Intranasal fentanyl is a good option for relieving the severe pain experienced by the girl in the vignette. Studies have demonstrated that doses of fentanyl delivered via a transmucosal route have similar analgesic action to intravenous opioids. An advantage of transmucosal analgesics is the ability to provide rapid pain relief without the need for intravenous access.\n\nOral ibuprofen has been well-studied and shown to be effective in the management of mild to moderate pain for patients with no contraindications to receiving oral medications. However, this option would not be appropriate for the girl in the vignette because of the severity of her injury, high degree of pain and distress, and high likelihood that she will require procedural sedation and surgical intervention in the near future.\n\nOral sucrose solution has been found to decrease the response to painful stimuli, such as intravenous line insertion, heel stick, and lumbar puncture, in neonates and infants less than 6 months of age. While this option can be useful in reducing distress arising from procedure-related pain in neonates and young infants, it would not adequately address pain in a 2-year-old child with a displaced femur fracture.\n\nAlthough the use of distraction techniques and other nonpharmacologic modalities can be an important component in helping to relieve anxiety in children and enabling them to better cope with acute pain, using distraction techniques alone would be inadequate for the girl in the vignette. Administration of an appropriate analgesic to this girl should not be delayed, given the severity of her pain and distress as well as the significant transport time anticipated.\n\nPREP Pearls\n\nTimely administration of analgesia to children with acute pain and anxiety is critical, and can affect their reaction to future medical care.\n\nFor children with severe pain seen in the emergency department and prehospital settings optimal pain management requires rapid pain assessment and administration of a systemic opioid pain medication.\n\nTransmucosal fentanyl has a similar analgesic action to intravenous opioids, providing rapid pain relief without the need for intravenous access.\n\nABP Content Specifications(s)/Content Area\n\nUnderstand the appropriate use of pharmacologic pain management modalities\n\nUnderstand the appropriate use of nonpharmacologic pain management modalities\n\nUnderstand the risks associated with the use of narcotics for pain management\n\nSuggested Readings\n\nAAP Committee on Psychosocial Aspects of Child and Family Health and the Task Force on Pain in Infants, Children, and Adolescents. The assessment and management of acute pain in infants, children, and adolescents. Pediatrics. 2001;108(3):793-797. doi: http://dx.doi.org/10.1542/peds.108.3.793.\n\nFein JA, Zempsky WT, Cravero JP; Committee on Pediatric Emergency Medicine and Section of Anesthesiology and Pain Medicine. Relief of pain and anxiety in pediatric patients in Emergency Medical Services. Pediatrics. 2012;130(5):1391-1405. doi: http://dx.doi.org/10.1542/peds.2012-2536."}
{"id" : 1463, "question_text" : "A 4-hour-old neonate on the newborn nursery service was noted by nursing to have ambiguous genitalia. The neonate was born at 40 weeks of gestation by spontaneous vaginal delivery to a 38-year-old gravida 2, now para 2 mother after an uncomplicated pregnancy. Given the mother's age, noninvasive prenatal testing was performed and revealed a 46,XY fetal karyotype. Apgar scores were 8 and 9 at 1 and 5 minutes, respectively. Birthweight was 3.6 kg. The neonate is doing well and successfully breastfed during the first hour of life. On physical examination, the neonate is vigorous and without dysmorphic features. Examination of the genitalia reveals a 1-cm phallic structure with the urethral opening at the base. There are bilateral labial-scrotal folds. A gonad is palpable in the right labial-scrotal fold, but not on the left (Item Q214). The remainder of the physical examination is unremarkable. The parents want to know if their child is a boy or a girl. Of the following, the BEST statement to counsel the parents regarding gender of rearing is", "options" : "[\"appearance of the genitalia appear more female and masculinizing surgical intervention would be difficult, so recommend female gender of rearing\", \"await further multidisciplinary evaluation and assist the parents in making an informed decision about gender of rearing\", \"await a trial of testosterone, and if there is a significant increase in phallic size, recommend male gender of rearing\", \"if male genetic sex is confirmed on a repeat postnatal karyotype, recommend male gender of rearing\", \"natural history studies show that many infants born with similar-appearing genitalia ultimately identify as male, so recommend male gender of rearing\"]", "explanation" : "Correct Answer: B\nThe neonate described in the vignette has a prenatal 46,XY karyotype and was born with ambiguous genitalia. The decision regarding gender of rearing is complex and should be made ultimately by the parents after a multidisciplinary evaluation to assist them in making the most informed decision possible. Considerations when determining gender of rearing may include, but are not limited to: genetic sex, underlying diagnosis, internal and external anatomy, potential for future urologic and sexual function, response to testosterone, prognosis regarding surgical outcomes, and prediction of future gender identity based on available evidence. The nonpreferred response choices only consider one of these factors.\n\nEthical principles that can be applied to this situation are autonomy, beneficence, and nonmaleficence. Respect for autonomy is the right for individuals to make their own decisions, or in this case, the right for the parents to make decisions for their child. To assist them in making the decision they feel is best for their child, the parents should be given as much information as possible about their child's diagnosis and prognosis. Beneficence is the benefit to the patient and nonmaleficence is avoiding harm. The diagnostic approach and information provided to the parents should be in the best interest of the infant, with the goal of achieving the most benefit with the least potential for harm. The approach should be multidisciplinary and include psychosocial support for the family. As recommended in the 2006 consensus statement on management of these disorders, the multidisciplinary team should achieve consensus prior to making recommendations to the family.\n\nPREP Pearls\n• Decisions regarding gender of rearing for infants with ambiguous genitalia are complex and should ultimately be made by the parents after multidisciplinary evaluation and provision of all available information.\n• The 4 principles of medical ethics are respect for autonomy, beneficence, nonmaleficence, and justice.\n• The approach to the infant with ambiguous genitalia should be multidisciplinary and include psychological support for the family.\n\nABP Content Specifications(s)\n• Recognize and apply ethical principles involved in using new technologies for sex/gender assignment\n\nSuggested Readings\n• Karkazis K, Rossi WC. Ethics for the pediatrician: disorders of sex development: optimizing care. Pediatr Rev.2010;31(11):e82-e85. doi: http://dx.doi.org/10.1542/pir.31-11-e82.\n• Lee PA, Houk CP, Ahmed SF, Hughes IA. Consensus statement on management of intersex disorders. Pediatrics.2006;118(2):e488-e500. doi: http://dx.doi.org/10.1542/peds.2006-0738.\n• Lee PA, Wisniewski AB, Baskin L, et al. Advances in diagnosis and care of persons with DSD over the last decade. Int J Pediatr Endocrinol. 2014;2014:19. doi: http://dx.doi.org/10.1186/1687-9856-2014-19."}
{"id" : 269, "question_text" : "The mother of a 7-year-old boy brings in her son because he has been staring off into space in his second-grade classroom. The teacher is concerned that he may be having seizures. Of the following, the feature of staring spells that is MOST consistent with absence epilepsy is", "options" : "[\"body rocking\", \"greater than 1 minute duration\", \"interruption of play\", \"limb twitching\", \"preserved responsiveness\"]", "explanation" : "Descriptions of a child frequently \"staring off\" suggest the possibility of absence epilepsy. Absence epilepsy is a childhood syndrome characterized by a number of key features that differentiates it from complex partial staring seizures and other causes of staring.\n\nThe cause of absence epilepsy is unknown, although the pathophysiology involves characteristic, highly synchronized, diffuse bursts of brain electrical activity seen on electroencephalography that correspond to the period of lost awareness. These events occur unpredictably, causing an abrupt cessation of ongoing behavior, such as interruption of play. The treatment of choice for absence epilepsy is ethosuximide, based on both efficacy and adverse effect profile. Even when absence epilepsy is effectively treated, however, problems with inattention may persist. Valproic acid is an alternative but is generally reserved for atypical absence epilepsies.\n\nBody rocking may be a stereotypy, during which a child may stare off, but there is no loss of consciousness. This represents dissociation, not seizure. Limb twitching or hand flapping are also not characteristic of absence seizures. Preserved responsiveness to external stimuli is characteristic of bored staring, behavioral dissociation, or stereotypies but is not true of seizures. The duration of absence seizures is usually brief, just a few seconds, not more than 1 minute.\n\nCritique: Preferred Response: C\n\nContent Specifications: Understand the drugs used to treat absence epilepsy; Recognize the characteristics of absence epilepsy"}
{"id" : 1170, "question_text" : "You are evaluating a 4-month-old infant in the emergency room for vomiting and decreased oral intake. The baby was born to a 29-year-old primigravida woman at 39 weeks of gestation via normal vaginal delivery. The baby's birth weight was 3.6 kg (50th percentile) and had a length of 50 cm (50th percentile). The mother mentions that his pediatrician has been concerned about his weight gain for the last month. His vital signs show a weight of 5.0 kg (less than the third percentile), length is 55.2 cm (less than the third percentile), temperature of 38.0°C, heart rate of 126 beats/min, respiratory rate of 30 breaths/min, and a blood pressure of 70/60 mm Hg. His physical examination is significant for mild dehydration, excessive watering of the eyes, photophobia, frontal bossing, and wrist widening. His urinalysis today demonstrates a specific gravity of 1.005, a pH of 7.0, 3+ glucose, and no blood, protein, leukocyte esterase, or nitrites. Of the following, the MOST likely cause of the patient's findings is", "options" : "[\"Bartter syndrome\", \"Gitelman syndrome\", \"renal tubular acidosis (type 1)\", \"renal tubular acidosis (type 2)\", \"renal tubular acidosis (type 4)\"]", "explanation" : "The patient described in the vignette was at the 50th percentile for height and weight at birth and is currently less than the third percentile for both weight and height. The decline in weight gain across 2 or more major percentiles is suggestive of failure to thrive in the patient. The history of concerns for the patient's growth, wrist widening, frontal bossing (suggestive of rickets), photophobia, and excessive watering of the eyes also suggests an underlying cause for the patient's failure to thrive. The diagnosis of renal tubular acidosis (RTA) should be considered in a young infant with failure to thrive, recurrent vomiting, rickets, episodes of dehydration, and investigations showing persistent metabolic acidosis, hypokalemia, and nephrolithiasis.\n\nRenal tubular acidosis is an inherited or acquired defect in the ability of the kidneys to absorb filtered bicarbonate or excrete ammonia. Renal tubular acidosis is characterized by a normal anion gap metabolic acidosis. Distal (type 1) RTA, proximal (type 2) RTA, mixed (type 3; features of both type 1 and 2) RTA, and hypoaldosteronism (type 4) are the 4 different forms of RTA seen in patients.\n\nThe presence of glycosuria, rickets, and photophobia, along with excessive watering of the eyes, is indicative of Fanconi syndrome in a patient with RTA and is most commonly associated with proximal (type 2) RTA, as demonstrated in this infant. Fanconi syndrome, characterized by generalized proximal tubular dysfunction as evidenced by rickets (phosphaturia leading to hypophosphatemic rickets), glycosuria (dipstick positive glycosuria with normal plasma glucose concentration), and aminoaciduria or tubular proteinuria (urine dipstick negative for protein and quantitative urine tests positive for amino acids and protein). Other RTAs (type 1 or type 4) or Bartter and Gitelman syndromes are not associated with Fanconi syndrome.\n\nRenal tubular acidosis occurs from the inability of the kidneys to keep up with the excretion of the daily endogenous acid production arising from dietary proteins and amino acids. The renal response to endogenous acids leads to maintenance of normal plasma HCO3-, the extracellular buffer. Urinary ammonia (NH3) and monohydrogen phosphate (HPO42-) are the 2 principal buffers binding the endogenously produced H+ ions. Of these, increased urinary ammonia excretion is the main adaptive response (can be increased by 10-fold) to increased acid excretion, as urinary phosphate excretion is fixed. Therefore, all forms of RTA are associated with metabolic acidosis (low plasma HCO3-) and decreased renal ammonium (NH4+) excretion.\n\nAmmonia excretion by the kidneys can be explained as a 4-step process, each of which is implicated in different types of RTA. Ammonium (NH4+) is produced by the proximal tubule from glutamine and secreted by the proximal tubule into the tubular fluid via Na+-H+ exchanger by binding to the H+ site. This step primarily generates the NH4+, which is subsequently processed in the renal tubule to regeneration of new HCO3- and also contributes to restoration of the bicarbonate pool. The next step is the absorption of NH4+ by the thick ascending loop of Henle via the Na-K-2Cl channel by occupying the K site. Ammonium in the cell dissociates into NH3 and H+. The apical membrane of thick ascending loop of Henle is impermeable to NH3, while the basolateral membrane is highly permeable. This leads to increased movement of NH3 across the basolateral membrane into the medullary interstitium and a gradient of increasing concentration of NH3 in the deeper medulla similar to the countercurrent system. This is the third step in normal ammonium secretion by the kidney. The fourth step involves diffusion of NH3 into the cortical collecting duct, wherein it reacts with H+ ions secreted by the intercalated A cells. This H+ secretion (proton donated by H2CO3, which dissociates into HCO3- and H+) leads to the addition of a new HCO3- molecule to the circulation (Item C106A).\n\nThe kidneys maintain acid base balance in vivo by: 1)reclaiming the filtered preformed HCO3- in the tubular fluid 2)regeneration of new HCO3- to account for HCO3- lost in buffering of the endogenous acids. Eighty percent to 90% of the filtered bicarbonate is reclaimed by the proximal tubule and the remaining by the thick ascending loop of Henle. Reclamation is an appropriate term to describe the processes of the proximal tubule, as the HCO3- molecule returning to the circulation is different from the filtered HCO3- (Item C106B).\n\nProximal RTA is a defect in the ability of the proximal renal tubules to reclaim filtered HCO3- from the tubular fluid. Fanconi syndrome, a form of proximal RTA, has been associated with cystinosis, galactosemia, tyrosinemia, oculocerebrorenal (Lowe) syndrome, and hereditary fructose intolerance. The presence of excessive eye watering and photophobia is indicative of cystinosis as the cause of the patient's symptoms and RTA. A slit lamp examination will reveal the characteristic cysteine crystals and confirm the diagnosis. In proximal RTA, once the serum bicarbonate has dropped to a level (around 12-15 mEq/L [12-15 mmol/L]) wherein the distal renal tubules can absorb the filtered bicarbonate load, the urine can be appropriately acidified and the presenting urine pH may be less than 5.5.\n\nImpaired regeneration of new HCO3- is indicative of a decreased rate of NH4+ ammonium (predominant urinary buffer) secretion by the kidney. This may be associated with normal serum potassium (distal type 1 RTA) or elevated serum potassium (hyperkalemic type 4 RTA). Impaired H+ ion secretion (step 4 of ammonium generation) by the cortical collecting duct leads to type 1 RTA. Distal or type 1 RTA is associated with failure to thrive, polyuria, hypokalemia, and medullary nephrocalcinosis (caused by hypercalciuria and hypocitraturia).\n\nHyperkalemic RTA is most frequently seen in patients with chronic renal failure, associated with renal parenchymal injury and scarring. Mineralocorticoid deficiency (decreased production or receptor insensitivity due to genetic or acquired causes) leads to hyperkalemia, which impairs steps 1, 2, and 4 of ammonia generation, leading to type 4 RTA.\n\nType 3 RTA is rare and associated with marble bone disease. It has findings of both type 1 (impaired regeneration of HCO3-) and type 2 (bicarbonate wasting) RTA. Treatment of RTA involves correction of metabolic acidosis via sodium or potassium citrate solutions. Potassium citrate solutions are avoided in hyperkalemic RTA. As the distal tubules are responsible for regulating only 5% to 10% of the acid load, the alkali dose is higher for proximal (10-20 mEq/kg per day), as compared to distal or hyperkalemic (5-8 mEq/kg per day) RTA.\n\nBartter syndrome and Gitelman syndrome are characterized by hypokalemia and metabolic alkalosis. Bartter syndrome often presents in childhood with growth retardation, hypokalemia, metabolic alkalosis, and polyuria or polydipsia. Bartter syndrome is caused by a primary defect in sodium chloride reabsorption in the medullary thick ascending limb of the loop of Henle, similar to chronic furosemide therapy. Gitelman syndrome generally presents in late childhood or adulthood with muscle cramps (hypokalemia), polyuria, or polydipsia. Mutations in the gene encoding for the thiazide-sensitive Na-Cl transporter in the distal tubule have been identified in patients with Gitelman syndrome. In contrast to Bartter syndrome, patients with Gitelman syndrome have reduced urinary calcium and hypomagnesemia (more common).\n\nPREP Pearls\n • The diagnosis of renal tubular acidosis (RTA) should be considered in a young infant with failure to thrive, recurrent vomiting, and episodes of dehydration.\n • Proximal RTA is most commonly associated with Fanconi syndrome, characterized by generalized proximal tubular dysfunction, as evidenced by rickets, glycosuria, and aminoaciduria.\n • Patients with cystinosis develop cystine deposits in the cornea and the conjunctiva, leading to photophobia, watering, and blepharospasm.\n • Bartter syndrome and Gitelman syndrome are characterized by hypokalemia and metabolic alkalosis.\n\nABP Content Specifications(s)\n • Recognize the clinical findings associated with various anomalies of the kidneys, urinary collecting system, and urinary excretion system"}
{"id" : 3045, "question_text" : "At his health supervision visit, you note that a 15-month old child's weight has fallen to the third percentile, while his height and head circumference remain at the 25th to 50th percentile. He was born at full term with an appropriate weight for gestational age. The child has had no illnesses other than mild upper respiratory infections; he has no history of vomiting, diarrhea, or constipation; and he has had no wheezing or breathing problems. His mother reports he is a picky eater who often refuses food of various textures. There is no family history of growth problems or chronic diseases. He has lived alone with his mother since his father was deployed with the military 2 months ago. Physical examination findings are unremarkable, except for the low weight. Of the following, the MOST appropriate initial management for this child is to", "options" : "[\"begin supplemental nasogastric feedings\", \"hospitalize him to monitor weight gain\", \"obtain an occupational therapy assessment\", \"order thyroid-stimulating hormone and insulin-like growth factor 1 levels\", \"refer him to a child psychiatrist for separation issues\"]", "explanation" : "TE\n\nPreferred Response: C\nFor the child in the vignette, the first step should be a thorough assessment of the current feeding practices and optimization of oral intake. Given the stress of parental deployment, the mother may benefit from behavioral health or peer support, but the primary intervention for the child is directed at improved feeding practices.\nNew concepts stress that failure to thrive is a \"physical sign\" of under nutrition, usually with multiple contributing factors. The management of failure to thrive, therefore, is directed at optimizing feeding, for which a multidisciplinary team is often required. Occupational therapists (OT) play a vital role in evaluating and treating dysfunctional feeding practices and food aversions, and should be involved early in the evaluation of a child with failure to thrive. In addition to the OT and pediatrician, providers who may be helpful in managing these patients include a dietician to monitor energy, protein, vitamin and mineral intake; a speech and language pathologist to assist with swallowing problems; a social worker to address family needs; and a behavioral health specialist to focus on psychosocial issues. Some or all of these resources may be available in the outpatient setting, and in most cases, hospitalization is not required. Although previously children were often admitted to the hospital to observe weight gain in a controlled environment, cost issues and the recognition that the child is usually better served in his own home environment now make hospitalization a less useful approach to the management of failure to thrive in all but the most severe cases.\nThe most important aspect of the evaluation of failure to thrive is a thorough history and physical examination directed at assessing adequacy of intake, problems with nutrient absorption, or increased metabolic demand. The practitioner needs to determine both the quantity and appropriateness of the food intake (eg, incorrect formula preparation, excessive juice intake, inadequate protein), and when appropriate, the family's access to food resources. Abnormal stool patterns, frequent vomiting, personal or family history of respiratory (eg, cystic fibrosis) or gastrointestinal illness, or symptom onset with first exposure to gluten intake may all suggest malabsorption. The presence of chronic disease or genetic disorders may indicate increased metabolic needs, requiring large caloric intake to support growth. For young children in particular, it is important to note whether impaired growth started prenatally because intrauterine growth restriction often affects long-term growth prognosis.\nPhysical examination findings may indicate both causes and effects of under nutrition. The practitioner should look for signs of an underlying chronic disease or genetic syndrome, as well as signs of malnutrition such as decreased subcutaneous tissue, cheilosis, or sparse hair. While many practitioners obtain screening laboratory tests for children with failure to thrive, studies have shown that laboratory evaluation rarely detects problems that were not already indicated by the history and physical examination. For the child in the vignette with no concerning history, unremarkable physical examination, and preserved length, obtaining thyroid studies and insulin-like growth factor 1 is unlikely to add useful information to the evaluation.\nThe goal of treatment for failure to thrive is to provide adequate nutrient intake to support appropriate growth. Ideally, this is achieved by improving food choices and feeding behaviors. In some severely affected children, adequate calorie intake for catch-up growth can only be achieved with nasogastric feedings.\n\nPREP Pearls\n• A multidisciplinary team including a pediatrician, a nutritionist, an occupational therapist, a social worker, and other practitioners is useful in the evaluation and management of failure to thrive.\n• Failure to thrive is a physical sign of under nutrition that is usually multi factorial.\n• Hospitalization for evaluation and treatment of failure to thrive is reserved for severely affected children or cases in which social factors place the child at significant risk.\n• Evaluation and treatment of failure to thrive is aimed at improving nutrient quantity and quality, optimizing feeding practices, and overcoming food aversions.\n\nAmerican Board of Pediatrics Content Specification(s)\n• Plan the management of an infant with failure to thrive\n\nSuggested Reading\n• American Academy of Pediatrics. Failure to thrive. In: Kleinman RE, ed.\n• Pediatric Nutrition Handbook_ 6th ed. Elk Grove Village. IL: American Academy of Pediatrics; 2009:601-636.\n• Jaffe AC. Failure to thrive: current clinical concepts. Pediatr Rev. 2011; 32(3):100-107. doi:10.1542/pir.32-3-100."}
{"id" : 2273, "question_text" : "A 15-year-old female is seen in the clinic for a 3-week history of right-sided lumbar back pain that worsens with activity and improves with rest. She is actively involved in softball and soccer and notes that the onset of pain coincided with the start of her softball season. She has no symptoms of radiculopathy (pain, numbness, weakness, tingling), fever, or weight loss. Her family history is notable for ankylosing spondylitis in a maternal uncle and Crohn disease in her paternal grandfather. Physical examination reveals a negative FABER (flexion, abduction, external rotation) test; she has no pain in the back or groin with hip external rotation. A single-leg hyperextension test (stork test) is negative, and she has normal spinal flexibility on a modified Schober test (lumbar flexion test). There is no pain with palpation or compression of the sacroiliac joints. Mild tenderness is noted over the right lumbar paraspinal muscle. There is no point tenderness over the lumbar spine. Of the following, the BEST next step is", "options" : "[\"HLA-B27 testing\", \"magnetic resonance imaging of the lumbar spine\", \"physical therapy\", \"rheumatology referral\"]", "explanation" : "Inflammatory back pain is generally associated with insidious onset morning stiffness and improvement with physical activity.\nPhysical examination of individuals with inflammatory back pain may demonstrate evidence of sacroiliac involvement (limitation of lumbar flexion, pain in the back or groin with hip external rotation, or pain with sacroiliac joint compression or palpation).\nCritique\nThe girl in the vignette likely has a strain of the paraspinal muscles of the lumbar spine, which would be most appropriately addressed through physical therapy.\nLower back pain is a common symptom among the pediatric population. It is often associated with biomechanical abnormalities that can benefit from physical therapy. The differential diagnosis of back pain is broad (Table 1). History and physical examination findings may raise concerns for a rheumatologic condition, such as ankylosing spondylitis, an inflammatory condition primarily involving the axial joints (especially the sacroiliac joints). Not all patients with lower back pain require laboratory or radiographic imaging studies to evaluate for a rheumatologic cause; the physical examination is instrumental in determining the next steps in evaluation (Table 2).\nThis child's family history of ankylosing spondylitis and inflammatory bowel disease raises concern for an inflammatory condition of the lower spine. However, inflammatory conditions generally have an insidious onset, with symptoms of morning stiffness and pain at rest that improves with activity. The girl in the vignette has pain that started acutely once she started softball season, and her pain worsens with exercise and improves with rest."}
{"id" : 1188, "question_text" : "A mother delivers a neonate precipitously, at 25 weeks' gestation, in the labor and delivery unit of a community hospital. The membranes spontaneously rupture at the time of delivery, revealing clear amniotic fluid. The very-low-birth weight neonate has poor tone, no spontaneous cry, and rare gasping respirations. The newborn is brought over to the warmer. Of the following, the BEST next step in the management of the neonate is", "options" : "[\"dry and stimulate the newborn with warm towels\", \"initiate positive pressure ventilation with 100% oxygen\", \"insert the newborn up to the neck in a polyethylene bag\", \"place the newborn in a head down position\", \"put a portable warming pad around the newborn\"]", "explanation" : "The extremely low-birthweight (ELBW) neonate in the vignette should be placed in a polyethylene bag immediately after delivery. The immature skin and underdeveloped subcutaneous fat found in an ELBW newborn contributes to significant heat loss in the immediate neonatal period. The use of a polyethylene barrier has been shown to decrease heat loss and minimize hypothermia in neonates born at less than 28 weeks of gestation.\nMultiple strategies have been used to enhance thermoregulation of the ELBW infant in the delivery room, because both hypothermia and hyperthermia are associated with increased morbidity. The ambient temperature of the delivery room should be increased to at least 26°C to decrease radiative heat loss. To minimize conductive heat loss, a radiant warmer should be operational at delivery with an activated heated mattress (always placed under the receiving blanket to prevent thermal contact burns). The ELBW infant should immediately be placed up to the neck in a polyethylene bag or wrap, leaving the head exposed for airway management (Item C137A). The use of a polyethylene barrier, such as a bag or wrap minimizes evaporative and convective heat loss. A hat may be placed on the head to further decrease radiative heat loss. The skin of the ELBW newborn should not be wiped dry or stimulated because the fragile epidermal layer is easily damaged by friction.\nAdditional aspects of delivery management in the first minutes after birth can significantly affect the long-term outcome of the ELBW infant. The resuscitation table should be flat, because placement of the infant head down (Trendelenburg) may lead to intraventricular hemorrhage. Excessive positive pressure with assisted ventilation may cause a pneumothorax and can contribute to the development of chronic lung disease. Oxygen therapy should be administered through an oxygen blender guided by the use of a pulse oximeter. Hypoxia and hyperoxia are both associated with increased morbidity in ELBW infants. Although no agreement has been reached on the optimum oxygen saturation level for preterm infants, targeted preductal saturations are recommended immediately after delivery based on time after birth (Item C137B), as outlined in the sixth edition of the Neonatal Resuscitation Program of the American Academy of Pediatrics and American Heart Association. Many neonatal providers begin assisted ventilation of a premature newborn with 30% to 40% oxygen rather than room air because of the high likelihood of surfactant deficiency, but the use of 100% oxygen is avoided unless supported by pulse oximetry.\nPREP Pearls\n• Multiple strategies to enhance the thermoregulation of a premature infant in the delivery room should include increasing the ambient temperature of the delivery room, the use of a radiant warmer, a warming mattress, a polyethylene barrier, and a hat.\n• The delivery of oxygen therapy to a premature infant in the delivery room should be guided by the use of a pulse oximeter and administered via an oxygen blender.\nABP Content Specifications(s)\n• Plan appropriate initial care for a very-low-birth-weight infant"}
{"id" : 3714, "question_text" : "A 13-year-old adolescent girl is seen for a routine health supervision visit. She was diagnosed with Crohn disease at 10 years of age. At 12 years of age she underwent resection of an ileal stricture and had 25 cm of terminal ileum removed. Reanastomosis has since been performed, and she has done very well. She has 1 or 2 formed stools daily, reports no abdominal pain, and has been growing well. Recent endoscopic evaluation (upper endoscopy and colonoscopy) demonstrated no active Crohn disease. She receives adalimumab injections every 2 weeks, and she takes a multivitamin with additional iron and vitamin D supplements daily. She eats a regular diet. Of the following, the dietary component MOST likely to be deficient in this patient is", "options" : "[\"cobalamin\", \"folate\", \"riboflavin\", \"thiamine\"]", "explanation" : "The adolescent in this vignette has Crohn disease, a subtype of inflammatory bowel disease (IBD) and has had resection of her terminal ileum, which increases her risk for cobalamin (vitamin B12) deficiency. Cobalamin is found naturally in animal products. Once ingested and in the stomach, vitamin B12 binds to intrinsic factor. The uptake of vitamin B12 bound to intrinsic factor occurs through a specific receptor found only in the terminal ileum. Therefore, diseases affecting the stomach or terminal ileum can result in cobalamin deficiency. Nutritional deficiencies can be commonly seen in gastrointestinal or hepatic diseases. The risk for specific vitamin or micronutrient deficiencies can be determined by the location of the encountered gastrointestinal or hepatic disease. Diseases affecting the liver or biliary tract can cause disordered bile flow (cholestasis), resulting in fat malabsorption and fat-soluble vitamin (A, D, E, and K) deficiency. Pancreatic disease (exocrine pancreatic insufficiency) also results in fat malabsorption, increasing the risk for deficiencies in vitamins A, D, E, and K. The duodenum, jejunum, and proximal ileum allow for nearly all vitamin and mineral absorption, although only the proximal small bowel (duodenum and proximal jejunum) is involved in iron and folate absorption. Therefore, diseases of the small intestine (eg, celiac disease or Crohn disease) may result in a variety of vitamin deficiencies, most commonly iron, folate, and vitamin D deficiency. The terminal ileum is responsible for cobalamin and bile salt absorption. Thus, disorders (or resection) of the terminal ileum may result in cobalamin deficiency and/or bile salt malabsorption. Interestingly, the distal small bowel may adapt when portions of the proximal small bowel are resected, but the converse is not true. If a portion of the duodenum is resected, the distal jejunum/ileum will adapt and can function for iron/folate absorption. However, if the terminal ileum is resected, the jejunum or proximal small bowel will not adapt to allow cobalamin absorption. Folate is absorbed primarily in the proximal small intestine, thus a resection of the terminal ileum is not likely to result in folate deficiency. Folate deficiency can be common in children with IBD, and annual routine measurement is indicated in this population. Riboflavin (vitamin B2) and thiamin (vitamin B1) are also absorbed in the proximal small intestine, thus resection of the terminal ileum would be unlikely to result in their deficiency. Riboflavin and thiamin deficiencies are not commonly seen in pediatric patients with IBD, and routine measurement of these vitamins is not indicated. PREP Pearls • Nutritional deficiencies can be commonly associated with gastrointestinal diseases. • Diseases of the liver, pancreas, and biliary tract may result in fat-soluble vitamin deficiencies (vitamins A, D, E, and K). • Diseases of the proximal small bowel can result in a variety of vitamin and micronutrient deficiencies. The most commonly encountered deficiencies include iron, folate, and vitamin D. • The terminal ileum is responsible for cobalamin (vitamin B12) absorption, thus diseases of the terminal ileum can result in vitamin B12 deficiency. ABP Content Specifications(s) • Recognize the nutritional deficiencies associated with gastrointestinal disease Suggested Readings • Diab L, Krebs NF. Vitamin excess and deficiency. Pediatr Rev. 2018;39(4):161-180. doi:10.1542/pir.2016-0068. • Miele E, Shamir R, Aloi M, et al. Nutrition in pediatric inflammatory bowel disease: a position paper on behalf of the Porto Inflammatory Bowel Disease Group of the European Society of Pediatric Gastroenterology, Hepatology and Nutrition. J Pediatr Gastroenterol Nutr. 2018;66(4):687-708. doi:10.1097/MPG.0000000000001896. • Sevilla WMA. Nutritional considerations in pediatric chronic disease. Pediatr Rev. 2017;38(8)343-352. doi:10.1542/pir.2016-0030."}
{"id" : 3339, "question_text" : "A 13-year-old adolescent girl presents to your clinic for a routine visit. She was diagnosed with acute lymphoblastic leukemia at 5 years of age, was treated with chemotherapy, and has been in complete remission. Her chemotherapy regimen included corticosteroids, vincristine, doxorubicin, asparaginase, cytarabine, methotrexate, and 6-mercaptopurine. Her mother is concerned about the long-term effects of the treatment her daughter received. Of the following, this patient is MOST likely to be at increased risk for", "options" : "[\"cardiovascular disease\", \"deep vein thrombosis\", \"glucose intolerance\", \"infertility\", \"short stature\"]", "explanation" : "The adolescent in this vignette is most likely to have problems with cardiovascular disease as a late sequela of her exposure to chemotherapy, in particular doxorubicin.\n\nWith the overall cure rate for childhood cancer now approximately 80%, patients are experiencing the late effects of their chemotherapy. The farther out the child is from initial diagnosis and treatment, the lower the risk of recurrent or second malignancy, and the higher the likelihood of morbidity from other causes. Pediatric cancer survivors can develop cardiovascular disease after receiving chemotherapy or radiation, and it is the second leading cause of morbidity and mortality after cancer recurrence. Pediatric cancer survivors have an increased risk of premature atherosclerosis, stroke, coronary artery disease, and heart failure.\n\nAlthough asparaginase and the presence of central venous catheters can increase the risk for thrombosis around the time of treatment, it would be unlikely to cause deep venous thromboses years after treatment has ended. Glucose intolerance can be seen in patients who receive high doses of corticosteroids for long periods, but again this should be reversible once the patient has stopped taking the medication. Infertility can be a concern for cancer survivors, more frequently after treatment with alkylating agents, such as cyclophosphamide, and in older children who received treatment during puberty. Short stature and endocrine dysfunction are commonly seen in childhood cancer survivors who have received cranial, spinal, or total body radiation therapy. This was often included in treatment protocols for acute lymphoblastic leukemia before intrathecal chemotherapy became standard of care.\n\nPREP Pearls\n• Pediatric cancer survivors can develop cardiovascular disease after receiving chemotherapy or radiation, and it is the second leading cause of morbidity and mortality after cancer recurrence.\n• With the overall cure rate for childhood cancer now approximately 80%, patients are experiencing the late effects of their chemotherapy.\n\nAmerican Board of Pediatrics Content Specification(s)\n• Understand the risks, side effects, and late sequelae associated with various chemotherapeutic drugs\n\nSuggested Reading\n• Armstrong GT, Liu Q, Yasui Y, et al. Late mortality among 5-year survivors of childhood cancer: a summary from the Childhood Cancer Survivor Study. J Clin Oncol. 2009;27(14):2328-2338. doi:10.1200/ JC0.2008.21.1425.\n• Chow EJ, Liu W, Srivastava K, et al. Differential effects of radiotherapy on growth and endocrine function among acute leukemia survivors: a childhood cancer survivor study report. Pediatr Blood Cancer. 2013;60(1):110-5. doi:10.1002/pbc.24198.\n• Dengel DR, Kelly AS, Zhang L, Hodges JS, Baker KS, Steinberger J. Signs of early sub-clinical atherosclerosis in childhood cancer survivors. Pediatr Blood Cancer. 2013;61(3):532-537. doi:10.1002/pbc.24829.\n• Leisenring WM, Mertens AC, Armstrong GT, et al. Pediatric cancer survivorship research: experience of the Childhood Cancer Survivor Study. J Clin Oncol. 2009;27(14):2319-2327. doi:10.1200/)C0.2008.21.1813.\n• Lipshultz SE, Cochran TR, Franco VI, Miller TL. Treatment-related cardiotoxicity in survivors of childhood cancer. Nat Rev Clin Oncol. 2013;10(12):697-710. doi:10.1038/nrclinonc.2013.195."}
{"id" : 2815, "question_text" : "A 6-month-old male infant who is limp and exhibiting obtundation in the morning is brought to the emergency department. Bedside blood analysis reveals a significant metabolic acidosis, including an elevated lactate level and a capillary blood glucose level of 35 mg/dL (1.9 mmol/L) and 3+ ketones in the urine. His newborn screening results were normal. His parents report that he only recently has been able to sleep through the night without waking for a feeding and that he has had a few episodes of morning lethargy that improved after his first morning feeding. The infant appears small and nearly comatose. He has chubby cheeks, a short nose, and a relatively small chin. He has a temperature of 37.8°C, a heart rate of 155 beats/min, a respiratory rate of 15 breaths/min, and a blood pressure of 75/45 mm Hg. Cardiac examination reveals no murmur and a normal S1 and S2. The liver edge is palpable 3 cm below the right costal margin. The remainder of the physical examination findings are unremarkable. Of the following, the MOST likely diagnosis is", "options" : "[\"fatty acid oxidation disorder\", \"glycogen storage disorder\", \"mitochondrial disorder\", \"organic acid disorder\"]", "explanation" : "The infant in the vignette, with obtundation after prolonged overnight fasting and hypoglycemia with lactic acidosis and ketosis, most likely has a glycogen storage disorder (GSD). Glycogen storage disease type 1a (also known as von Gierke disease) is caused by a deficiency in glucose-6-phosphatase. This type often presents in infancy and results in hepatomegaly, growth failure, and recurrent episodes of hypoglycemia with ketosis. Although some neonates with glycogen storage disease present with severe hypoglycemia, it is more common for infants aged 3 to 4 months to manifest hepatomegaly, lactic acidosis, hypoglycemic seizures, or a combination of these. Additional laboratory abnormalities include elevated uric acid levels and hyperlipidemia. The physical findings of chubby cheeks, a short nose, and a relatively small chin are nonspecific but can be found in children with GSD. The diagnosis is made by means of genetic analysis identifying pathologic variants in specific genes, including G6PC, SLC37A4, or both. These genes encode for glucose-6-phosphatase activity and glucose-6-phosphate exchanger SLC37A4 activity, respectively.\n\nInborn errors of metabolism, in general, are insidious and involve abnormalities in the processing of carbohydrate, lipid, or protein that result in a toxic accumulation of energy precursors or metabolites or in a deficiency of nutritional elements. Because early intervention and appropriate therapy are required to ensure optimal outcomes, clinicians must remain aware of the physical findings and characteristic histories of different inborn errors of metabolism.\n\nMetabolic disorders involving protein include amino acid, organic acid, and urea cycle disorders. When amino acids are metabolized, deamination removes the amine group and leaves ammonia and an organic acid. Disorders of the first step in amino acid metabolism result in accumulation of unmetabolized precursor amino acids. If the abnormality occurs further along the processing pathway, organic acids that are unable to be metabolized will accumulate. Finally, if the child has a urea cycle defect, ammonia will not be metabolized to urea, resulting in hyperammonemia. Patients with organic and amino acid disorders often experience neonatal lethargy, vomiting, coma, strokes, and, if unrecognized, death. Patients may have elevations of specific amino acids, organic acids, or ammonia in the serum or urine.\n\nDisorders of lipid metabolism, or fatty acid oxidation disorders, result from abnormal β-oxidation of fatty acids. A hallmark of fatty acid oxidation disorders is the development of hypoglycemia without the concurrent development of ketones, which normally results from the breakdown of fats. Hypoglycemia generally occurs during a period of fasting when glycogen and gluconeogenesis normally ensure maintenance of serum glucose levels. To produce glucose from gluconeogenesis, fats must be oxidized; thus, patients with fatty acid oxidation defects cannot metabolize fatty acids to produce ketone bodies and cannot release stored energy from glycogen. Presenting signs of fatty acid oxidation disorders may include nonketotic hypoglycemia, seizures, rhabdomyolysis, cardiomyopathy, liver dysfunction, and sudden infant death.\n\nMitochondrial disorders result from mutations in the mitochondrial genome required for mitochondrial function. This leads to decreased production of energy in the form of adenosine triphosphate and intracellular acidosis. Metabolically active tissues, including the brain, skeletal muscles, and cardiac muscles, are most affected. Diagnosis is made via fresh muscle biopsy microscopic analysis revealing ragged red fibers. Patients develop nonspecific lactic acidosis and concurrent organ-specific findings such as strokes, seizures, cardiac conduction abnormalities, hypotonia, or weakness.\n\nThe 6-month-old in the vignette does not have characteristic neurological features of a mitochondrial disorder, including hypotonia. Although lactic acidosis is present in mitochondrial disorders, the other laboratory findings and clinical history are more consistent with GSD.\n\nThe infant in the vignette has ketonuria, indicating that the β-oxidation of fatty acids is normal, making a fatty acid oxidation disorder an incorrect choice.\n\nOrganic acid disorders generally present in the neonatal period.\n\nPREP Pearls\n• Inborn errors of metabolism are generally insidious; they involve abnormalities of carbohydrate, lipid, or protein processing that result in a toxic accumulation of energy precursors or metabolites or in a deficiency of nutritional elements.\n• Glycogen storage disease often presents in infancy and results in hepatomegaly, growth failure, and recurrent episodes of hypoglycemia with ketosis.\n• Children with organic and amino acid disorders often experience neonatal lethargy, vomiting, coma, strokes, and, if unrecognized, even death.\n• A hallmark of fatty acid oxidation disorders is the development of hypoglycemia without the concurrent development of ketones.\n\nABP Content Specifications(s)\n• Plan the evaluation of a patient with suspected metabolic disease who is acidotic\n• Plan the evaluation of a patient with suspected metabolic disease who is comatose\n• Plan the evaluation of a patient with suspected metabolic disease who has hypoglycemia, and manage appropriately\n\nSuggested Readings\n• Bali DS, Chen YT, Austin S, Goldstein JL. Glycogen storage disease type I. In: Adam MP, Ardinger HH, Pagon RA, et al, eds. GeneReviews. Seattle, WA: University of Washington, Seattle; 1993–2016. 2006 Apr 19 (updated 2016 Aug 25).\n• Rice GM, Steiner RD. Inborn errors of metabolism (metabolic disorders). Pediatr Rev. 2016;37(1):3-15. doi:10.1542/pir.2014-0122.\n• Rios A, Adams DJ. Specific congenital metabolic diseases. In: McInerny TK, Adam HM, Campbell DE, DeWitt TG, Foy JM, Kamat DM, eds. American Academy of Pediatrics Textbook of Pediatric Care. 2nd ed. Elk Grove Village, IL: American Academy of Pediatrics; 2017:938-962. Pediatric Care Online."}
{"id" : 3160, "question_text" : "You are charged with helping your hospital's pharmacy and therapeutic committee to formulate a policy for recommending drug choices to treat pediatric pneumonia. In preparing for the meeting, you review the literature on the topic. Of the following, the MOST reliable of available analyses would be a", "options" : "[\"case-control study\", \"double-blind, placebo-controlled trial\", \"meta-analysis of published studies\", \"published editorial on the subject\", \"review of the product insert of relevant drugs\"]", "explanation" : "Preferred Response: C\nA systematic review of a clinical question entails a comprehensive review of the existing literature on the topic. In performing a systematic review of the literature on a treatment regimen, a meta-analysis offers the advantage of combining the results of multiple studies meeting specified criteria on the subject using statistical methods that then provides a quantified estimate of the benefits or adverse effects of the therapeutic intervention. A high quality meta-analysis requires a comprehensive search of the literature and a critical evaluation of studies to be included. A meta-analysis may be limited by the quality and number of studies analyzed.\n\nCase control studies are evaluations of patients with a given condition (in this case pneumonia) who received a specific treatment and comparing their outcomes to those receiving another regimen. Such studies are limited by being retrospective in nature and generally involve only a single specific population.\n\nA double-blind, placebo-controlled trial, if randomized, is a study design in which a group of patients are randomly assigned to receive the drug being studied or a control drug (placebo or present standard treatment) and then assessed for prespecified outcomes. Limitations may include the nature of the study population and the inability to compare multiple treatment regimens.\n\nA published editorial likely gives expert opinion based on existing evidence, but is limited by the review and opinions of the authors.\n\nThe product insert of a drug describes the US Food and Drug Administration (FDA) approved features of a given agent and includes studies submitted to the FDA, but may not provide a comparison of alternative agents.\n\nPREP Pearls\n• A systematic review includes a comprehensive review of the literature on a clinical question.\n• A meta-analysis uses statistical methods to combine results of multiple studies on a given topic.\n\nAmerican Board of Pediatrics Content Specification(s)\n• Understand the uses and limitations of systematic review and meta-analysis\n\nSuggested Reading\n• Bair-Merritt MH. Systematic reviews and meta-analysis. Pediatr Rev. 2009;30(10):409-410. doi:10.1542/pir.30-10-409.\n• Cochrane Collaboration. What is meta-analysis? The Cochrane Collaboration website. Accessed December 12, 2013.\n• Stroup DF. Berlin JA, Morton SC, et al. Meta-analysis of observational studies in epidemiology: a proposal for reporting: meta-analysis of observational studies in epidemiology (MOOSE) group. JAMA. 2000;283(15):2008-2012. doi:10.1001/jama.283.15.2008."}
{"id" : 2444, "question_text" : "A 4-month-old infant is brought to the emergency department for concerns about dehydration. She has been congested for the past 3 days. Her oral intake has decreased and today she has had only 1 damp diaper. Her temperature is 37.4 °C, her heart rate is 190 beats/min, her respiratory rate is 55 breaths/min, and her oxygen saturation is 94% in room air. She is awake and alert, sitting in her mother's lap. She has nasal congestion. Pulmonary examination reveals mild retractions; diffuse, scattered rhonchi; no wheezing; and good air entry throughout. Her capillary refill time is 4 seconds. The remainder of her examination findings are unremarkable. Intravenous hydration with isotonic fluid is ordered. Of the following, the BEST next step in this child's care is to prescribe", "options" : "[\"a topical anesthetic\", \"albuterol\", \"ibuprofen\", \"oxygen\"]", "explanation" : "PREP Pearl(s)\nChildren of all ages undergoing painful procedures should receive appropriate pain management.\nNeonates and infants who undergo painful procedures without adequate pain management may develop sleep disturbances, difficulty bonding with caregivers, an increased stress response, and increased pain perception in response to repeated noxious stimuli.\nAppropriate methods of procedural pain management vary according to age and developmental stage.\nCritique\nThe infant in the vignette has signs and symptoms of dehydration (prolonged capillary refill time, tachycardia, decreased urine output) and requires intravenous fluids. Managing pain during intravenous catheter insertion, a painful procedure, is important. Of the response choices, a topical anesthetic is the best next step in her care. These should be used with caution in infants <3 months of age owing to the risk of methemoglobinemia. Instructions vary by manufacturer.\nIbuprofen may help control pain during painful procedures, but this infant is too young (age <6 months). Supplemental oxygen is not indicated for this child, who has mild retractions and an oxygen saturation of >90% in room air. She has only mild retractions, no wheezing, and good lung aeration, so treatment with albuterol is not indicated.\nPain management should be considered for children of all ages during painful procedures. The appropriate management varies according to age and developmental stage.\nNeonates feel pain as early as 25 weeks' gestation. Neonates born prematurely are at increased risk of experiencing long-term sequelae if pain is not managed well because of their developing brain and exposure to recurrent painful interventions. Sequelae include sleep disturbances, difficulty bonding with caregivers, an increased stress response, and increased pain perception in response to repeated noxious stimuli. Infants as young as 6 months who have experienced pain in the medical setting display anticipation, fear, and avoidance of painful procedures. Nonpharmacological pain management strategies for neonates and infants include positioning (eg, swaddling, skin-to-skin contact), sucking, and breastfeeding.\nPain management is especially important for toddler-aged children. Toddlers explore their world using physical touch and sensory stimulation, which places them at increased risk of experiencing trauma resulting from painful procedures. They are not able to understand why a painful intervention is required but are likely to retain the memory of painful procedures long term. Nonpharmacologic pain management strategies for toddlers include using positions of comfort (eg, sitting on a caregiver's lap in a chest-to-chest position), comfort objects (eg, stuffed animal or blanket), and distraction (eg, light-up toys, electronic games, videos). It is important to avoid an overwhelming environment for toddlers. Identifying one person (a caregiver or medical professional) to be the voice the child hears during the procedure helps minimize stimulation. Supplies should be set up before the child enters the room and placed to the side, so as not to be a focal point. If the child has undergone procedures in the past, it is helpful to ask caregivers what has worked well to ease the experience for the child.\nPreschoolers are developing the ability to differentiate between pretend and reality. It is important to ensure they understand that painful procedures are not punishment. Their ability to understand time is still developing. As the procedure is discussed, the child may not understand when it will start and the expected duration of the pain. Appropriate pain-control interventions for preschoolers include positions of comfort (eg, sitting on or with a caregiver and using a pillow as support under the arm) and the one-voice method. Keeping discussions simple and avoiding use of medical terms is important at this age.\nSchool-aged children are concrete in their thinking. Preparing a child for a painful procedure with pictures, videos, and examples of equipment they can see and touch, as well as explaining that pain-control therapies (eg, topical anesthetics) will be used, can help decrease anxiety. Positions of comfort, comfort objects, and methods that have previously made them feel safe also help decrease stress.\nAdolescents vary in their response to painful procedures. Those who have had previous painful experiences may feel more stress than expected for their age. In response to this stress, adolescents may regress to a prior developmental stage. It is important to use easy-to-understand language to discuss what to expect and how methods to decrease pain work.\nPharmacologic pain-control interventions commonly used include the following:\nOral sucrose or glucose for neonates and infants\nTopical or subcutaneous lidocaine (eg, for intravenous catheter placement)\nAcetaminophen or ibuprofen administered at least 30 min before the procedure\nOpiates for more invasive procedures (eg, wound dressing change)\nModerate sedation provided by trained practitioners should be considered for more invasive or painful procedures.\nSuggested Reading(s)\nCommittee on Fetus and Newborn and Section on Anesthesiology and Pain Medicine. Prevention and management of procedural pain in the neonate: an update. Pediatrics. 2016;137(2):e20154271. doi:10.1542/peds.2015-4271\nKennedy RM, Luhmann J, Zempsky WT. Clinical implications of unmanaged needle-insertion pain and distress in children. Pediatrics. 2008;122(suppl 3):S130-S133. doi:10.1542/peds.2008-1055e\nUman LS, Birnie KA, Noel M, et al. Psychological interventions for needle-related procedural pain and distress in children and adolescents. Cochrane Database Syst Rev. 2013;(10):CD005179. doi:10.1002/14651858.CD005179.pub3\nContent Domain\nPharmacology\nABP Content Specification(s) / Content Area(s)\nUnderstand the effects of a patient's developmental stage on tolerating and dealing with pain\nPlan the appropriate management of pain in patients of various ages"}
{"id" : 1460, "question_text" : "A 3-week-old neonate born at 28 weeks of gestation develops episodes of apnea and bradycardia in the neonatal intensive care unit. Vital signs show a temperature of 37°C, respiratory rate of 25 breaths/min, heart rate of 110 beats/min, blood pressure of 55/35 mm Hg, and his weight is 1,425 g. On physical examination, he is pale and breathing irregularly. Laboratory data are shown: White blood cell count 3,200/µL (3.20 x 109/L), Segmented neutrophils 59%, Bands 10%, Lymphocytes 20%, Monocytes 11%, Hemoglobin 8.8 g/dL (88 g/L), Platelet count 87 x 103/µL (87 x 109/L), Blood culture Candida glabrata, Cerebrospinal fluid (CSF) white blood cells 88/µL, CSF red blood cells 5/µL, CSF glucose 50 mg/dL, CSF protein 150 mg/dL. Of the following, the BEST next study to evaluate for dissemination of this infection is", "options" : "[\"abdominal ultrasonography\", \"dilated retinal examination\", \"echocardiogram\", \"head ultrasonography\", \"long bone films\"]", "explanation" : "Correct Answer: B\nThe patient in the vignette has candidemia and meningitis. As in the vignette, lumbar puncture is recommended for all neonates with candidemia. The best next step to evaluate for disseminated infection is a dilated retinal examination. Ophthalmologic evaluation is recommended for all patients with candidemia.\n\nInfections due to Candida species have a wide spectrum of presentation. In immunocompetent hosts, Candida species cause superficial infections, such as thrush, dermatitis, and onychia. However, very low birthweight neonates and immunocompromised individuals (especially those with neutropenia, neutrophil defects, or T-cell defects) can develop invasive candidiasis, which can lead to infection in nearly any anatomic site. Additionally, individuals with indwelling devices, such as central vascular catheters, peritoneal catheters, and urinary catheters are at risk for invasive Candida infections.\n\nAbdominal ultrasonography and echocardiography are recommended in the setting of multiple positive cultures. Abdominal ultrasonography can assess genitourinary tract involvement, as well as fungal nodules in the liver and spleen. Echocardiogram helps assess for endocarditis or an endovascular focus. Head ultrasonography would be needed if a central nervous system complication, such as hydrocephalus, is suspected. While osteomyelitis can occur in the setting of candidemia, plain films would be recommended only if this diagnosis is suggested by physical examination.\n\nThrush is usually treated with nystatin oral suspension and skin infections with topical antifungals including nystatin and several azole drugs. Refractory mucocutaneous infections are treated with oral agents, usually fluconazole. Invasive infections are treated with parenteral antifungals including the azoles, echinocandins, or amphotericin. Certain Candida species have variable susceptibilities to the parenteral drugs and this must be considered when choosing empiric agents. In addition to pharmacologic therapy, management of a patient with an invasive Candida infection associated with an indwelling device usually involves removal of the involved device.\n\nPREP Pearls\n• Very low birthweight neonates and immunocompromised individuals (especially those with neutropenia, neutrophil defects, or T-cell defects) can develop invasive candidiasis.\n• Lumbar puncture is recommended for all neonates and ophthalmologic evaluation for all individuals with candidemia.\n• Management of a patient with an invasive Candida infection associated with an indwelling device usually includes removal of the involved device.\n\nABP Content Specifications(s)\n• Recognize the clinical features associated with Candida infection\n• Identify the risk factors for candidiasis in patients of various ages\n• Plan appropriate management for a patient with Candida infection\n\nSuggested Readings\n• American Academy of Pediatrics. Candidiasis. In: Kimberlin DW, Brady MT, Jackson MA, Long SS, eds. Red Book: 2015 Report Of The Committee On Infectious Diseases. 30th ed. Elk Grove Village, IL: American Academy of Pediatrics; 2015:275-280.\n• Pappas PG, Kauffman CA, Andres D, et al. Clinical practice guideline for the management of candidiasis: 2009 update by the Infectious Diseases Society of America. Clin Infect Dis. 2009;48(5):502-535. doi: http://dx.doi.org/10.1086/596757."}
{"id" : 2204, "question_text" : "A 36-year-old woman with a history of insulin-dependent diabetes mellitus, chronic hypertension, and previous intrauterine fetal demise at 36 weeks' gestation is undergoing a biophysical profile evaluation at 35 weeks' gestation. The fetal nonstress test shows >2 accelerations in fetal heart rate of 10 beats/min above baseline. Fetal breathing is observed, and the single deepest vertical pocket of amniotic fluid is >2 cm. Multiple fetal body movements, including rolling, are observed, with at least one kick with return to flexion. Of the following, the biophysical profile score of this fetus is", "options" : "[\"4\", \"6\", \"8\", \"10\"]", "explanation" : "The primary objective of antepartum fetal surveillance is to detect fetal hypoxia early to prevent hypoxic ischemic encephalopathy and fetal demise. The nonstress test is based on evidence that the heart rate of the healthy fetus will temporarily accelerate with fetal movement.\n\nThe biophysical profile is used to evaluate fetal well being, based on the concept that the fetal brain centers that control fetal biophysical activities are sensitive to varying degrees of hypoxia.\n\nThe 5 components of the biophysical profile consist of the nonstress test (fetal heart rate assessment), fetal movement, fetal tone, fetal respiratory movement, and amniotic fluid volume, with each parameter score being either 2 (present) or 0 (not present); the maximum possible score is 10.\n\nCritique\nThe primary objective of antepartum fetal surveillance is early detection of fetal hypoxia to prevent hypoxic ischemic encephalopathy and fetal demise. The nonstress test (NST) is a commonly used test for fetal surveillance. The NST is based on evidence that the heart rate of the healthy fetus will temporarily accelerate with fetal movement. A reactive (normal) NST test result is defined as ≥2 fetal heart rate accelerations that peak at least 15 beats/min above the baseline and last 15 seconds from baseline to baseline within a 20-min period. Most NSTs are reactive within 20 min. For those nonreactive at 20 min, the test can be extended to 40 min to account for the fetal sleep-wake cycle. A NST is nonreactive (abnormal) if there are <2 accelerations in 40 min.\n\nThe fetus in the vignette has a biophysical profile (BPP) score of 8. The BPP is also used to evaluate fetal well being. This test is based on the concept that the fetal brain centers that control fetal biophysical activities are sensitive to varying degrees of hypoxia. The BPP parameters can be affected by factors other than hypoxemia, including maternal medications, fetal sleep cycles, oligohydramnios, congenital anomalies, and prematurity. The BPP involves the use of ultrasonography and electronic fetal heart rate monitoring for evaluation of fetal well being. The 5 components of the BPP are as follows:\nnonstress test (fetal heart rate assessment)\nFetal movement\nFetal tone\nFetal respiratory movement\nAmniotic fluid volume\n\nEach parameter is scored as either 2 (present) or 0 (not present), with a maximum possible score of 10 (Table).\n\nA total BPP score of score ≥8 is predictive of normal fetal oxygenation, and no further testing is required for at least 1 week.\n\nA BPP score of 6 is an equivocal test and requires follow-up testing within 24 hours to determine if delivery is required.\n\nA total BPP score of ≤4 can be a sign of fetal compromise and suggests that fetal delivery is required to prevent adverse fetal and/or neonatal outcome.\n\nA BPP of 8 of 8, using only the ultrasonography assessment, has the same high predictive accuracy of fetal well being as a BPP of 10 of 10 (including the NST). The fetus in the vignette receives 2 points each for amniotic fluid volume, movement, tone, and breathing (a total of 8 points). No points are received for the NST result because the fetus is not reactive (15 beat accelerations are required at 32 weeks).\n\nIn contrast to the NST, which evaluates fetal condition, the contraction stress test evaluates uteroplacental function. It is a method of fetal surveillance that evaluates fetal heart rate in response to induced mild uterine contractions. Three contractions of ≥40 seconds' duration in a 10-min period are required for a negative (normal) test. If this does not occur spontaneously, nipple stimulation or intravenous oxytocin administration is used. A contraction stress test result is negative if there are no late or significant variable decelerations; it is positive if late decelerations follow ≥50% of contractions (even if the contraction frequency is <3 in 10 min).\n\nContent Domain\nNeonatology, Maternal-fetal care\n\nLearning Objectives\nKnow how to evaluate fetal well being using the stress and nonstress tests."}
{"id" : 1823, "question_text" : "A parent needs to administer 125 mg of oral amoxicillin liquid (250 mg/5 mL) to their young child. Of the following, the method MOST likely to reduce the likelihood of a dosing error is to administer the medication using a", "options" : "[\"medication cup labeled in 2.5-mL increments\", \"medication cup labeled in \\u00bd-teaspoon increments\", \"medication cup labeled in \\u00bd-teaspoon and 2.5-mL increments\", \"syringe labeled in 0.5-mL increments\"]", "explanation" : "The type of measuring device used to administer liquid medications and the units marked on the device influence parents' ability to deliver an accurate dose. A syringe labeled in 0.5-mL increments is the best dosing device to provide this parent to ensure 2.5 mL of amoxicillin will be correctly administered. Although medication cups may be more accessible and perceived as more acceptable to parents, evidence shows there is an increased likelihood of dosing errors when liquid medications are given by cup compared with syringe. This occurs across health literacy and language groups, and is especially true for smaller amounts of medication. Medication errors by parents are more likely when teaspoon or tablespoon units are used compared with milliliter units. Also the use of dosing tools that are labeled with both milliliter and teaspoon units leads to more errors.\n\nParents commonly administer the incorrect dose of medication to their children. The ability of parents to understand medication dosing is related to their literacy and numeracy skills. The dose, formulation, and frequency of prescribed medications must be clearly explained. Provision of dosing tools closely matched to the prescribed dose volume is an important strategy to help reduce medication dosing errors. Prescribing easy-to-dose volumes when selecting the formulation of medications is another strategy. Pictographic depiction of dosage is also an effective means to ensure understanding and reduce medical errors at home. The use of household spoons as measuring devices should always be discouraged because of inaccuracy and inconsistency in volume. Over-the-counter products should provide clear directions and a concordant measuring device for parents to use.\n\nPediatric health care providers need to understand the impact of product packaging on medication safety, plus the role medical device design plays in the prevention of medical errors. Several medical organizations have issued statements that recognize the importance of clarity and precision for dosing orally administered liquid medications.\n\nThe American Academy of Pediatrics Committee on Drugs policy statement from April 2015 includes the following recommendations:\n1. Metric-based dosing with milliliters should be used exclusively.\n2. Medications should be dosed to the nearest 0.1, 0.5, or 1 mL.\n3. The concentration of the medication and the frequency of administration should be clearly noted on prescriptions.\n4. Appropriate-volume milliliter-based dosing devices should be distributed with the medication.\n5. Syringes are the preferred dosing device. Measuring cups and spoons calibrated and marked in milliliters are acceptable alternatives.\n6. Advanced counseling strategies to ensure parental understanding and adequate health literacy and numeracy should be offered.\n\nPREP Pearls\n\nTo decrease the risk of medication administration errors by parents, liquid medications should be dosed in milliliters, not teaspoons or tablespoons.\n\nEvidence shows there is an increased likelihood of dosing errors when liquid medications are measured by cup or spoon compared with syringe.\n\nThe use of dosing tools that are labeled with both milliliter and teaspoon units, rather than with milliliter labels only, leads to more medical errors by parents.\n\nABP Content Specifications(s)/Content Area\n\nUnderstand the impact of product naming and packaging on medication safety\n\nUnderstand the role of medical device design in prevention of medical error\n\nSuggested Readings\n\nCommittee on Drugs. Metric units and the preferred dosing of orally administered liquid medications. Pediatrics. 2015;135(4);784-787. doi: http://dx.doi.org/10.1542/peds.2015-0072.\n\nLeonard MS. Patient safety and quality improvement: reducing risk of harm. Pediatr Rev. 2015;36;448-458. doi: http://dx.doi.org/10.1542/pir.36-10-448.\n\nShonna Yin H, Parker RM, Sanders LM, et al. Liquid medication errors and dosing tools: a randomized controlled experiment. Pediatrics. 2016;138(4)e20160357. doi: http://dx.doi.org/10.1542/peds.2016-0357.\n\nShonna Yin H, Parker RM, Sanders LM, et al. Pictograms, units and dosing tools, and parent medication errors: a randomized study. Pediatrics. 2017;140(1):e20163237. doi: http://dx.doi.org/10.1542/peds.2016-3237."}
{"id" : 3581, "question_text" : "A 13-year-old Native American boy is receiving a health supervision evaluation. He and his parents have no concerns or questions. The boy's height is 162 cm (75th percentile), weight is 71 kg (97th percentile), and body mass index is 27 kg/m2 (97th percentile). His blood pressure is 110/70 mm Hg. The skin on his posterior neck and bilateral axillae is hyperpigmented and velvety. The remainder of his physical examination findings are normal. Of the following, the MOST appropriate screening test to perform in this patient is a(n)", "options" : "[\"fasting insulin\", \"hemoglobin A1c\", \"insulin-like growth factor\", \"random glucose\"]", "explanation" : "The boy in the vignette has obesity, signs of insulin-resistance (acanthosis nigricans), and is from a high-risk ethnic group (Native American); therefore he should be screened for type 2 diabetes mellitus. Screening can be done by measuring a patient's hemoglobin A1c or fasting glucose level, or performing an oral glucose-tolerance test. A fasting insulin level, insulin-like growth factor, or random glucose would not be appropriate screening for type 2 diabetes mellitus. Although insulin levels are often elevated in patients with prediabetes and type 2 diabetes mellitus, there is no standardized universal insulin assay and there is considerable overlap in levels between those with and without insulin resistance. Insulin-like growth factor is a hormone that functions as a mediator of growth hormone (GH)–stimulated somatic growth and has no role in the diagnosis of diabetes mellitus. Glucose levels are highly variable and are tied to food intake; they are not appropriate for screening in high-risk groups for asymptomatic individuals.\n\nObesity is an increasingly common condition; about one-third of American children and adolescents have obesity. Obesity is defined as a body mass index (BMI; weight in kilograms divided by height in meters squared) over the 95th percentile for age, and overweight is defined as a BMI between the 85th and 95th percentile for age. Environmental factors, including sedentary lifestyle and excess caloric intake, are by far the main contributors to obesity. There are rare genetic disorders that cause obesity (eg, Beckwith-Wiedemann, Prader-Willi, and Bardet-Biedl syndromes) as well as endocrinologic disorders (eg, hypothyroidism, cortisol excess, growth hormone deficiency) that contribute to a small percentage of children with obesity.\n\nChildren with obesity are likely to remain obese into adulthood, and the risk of adult obesity increases with age; adolescents have a 90% chance that their obesity will persist into adulthood. Obesity is associated with comorbidities in childhood, including:\n• Depression\n• Hepatic steatosis\n• Hyperlipidemia\n• Hypertension\n• Obstructive sleep apnea\n• Orthopedic conditions and joint pain\n• Type 2 diabetes mellitus\n\nChildren with obesity should be screened for hypertension, hyperlipidemia, and fatty liver disease. Beginning at age 10 years or the onset of puberty (whichever occurs earlier), they should also be screened for type 2 diabetes mellitus if they have at least 1 of the following risk factors:\n• Family history of type 2 diabetes mellitus in a first- or second-degree relative\n• High-risk race/ethnicity (Native American, African-American, Latino, Asian American, or Pacific Islander)\n• Signs of insulin resistance on physical examination\n• Maternal history of diabetes or gestational diabetes during the child's gestation\n\nPREP Pearls\n• Nearly all obesity is related to excess caloric intake and sedentary lifestyles; genetic syndromes and endocrinologic disorders account for a very small percentage of cases.\n• Obesity in children is associated with an increased risk of hypertension, hyperlipidemia, type 2 diabetes mellitus, obstructive sleep apnea, hepatic steatosis, orthopedic conditions, and depression.\n• Children with obesity should be screened for hypertension and hyperlipidemia. Beginning at age 10 years or the onset of puberty (whichever occurs earlier), they should also be screened for type 2 diabetes mellitus with a fasting glucose, hemoglobin A1c, or oral glucose tolerance test if they have any additional risk factors:\n  o family history of type 2 diabetes mellitus in a first- or second-degree relative\n  o high-risk race/ethnicity (Native American, African-American, Latino, Asian American, or Pacific Islander)\n  o signs of insulin resistance on physical examination\n  o maternal history of diabetes or gestational diabetes during the child's gestation\n\nMOCA-Peds Objective\n• Evaluate and manage a patient with obesity.\n\nABP Content Specifications(s)\n• Recognize the various complications associated with obesity\n• Formulate a differential diagnosis in a patient who is obese\n\nSuggested Readings\n• American Diabetes Association. Classification and diagnosis of diabetes: Standards of Medical Care in Diabetes-2019. Diabetes Care. 2019;42(suppl 1):S13-S28. doi:10.2337/dc19-S002.\n• Chiehyu Wong H, Gahagan S. Obesity and metabolic syndrome. In: McInerny TK, Adam HM, Campbell DE, DeWitt TG, Foy JM, Kamat DM, eds. American Academy of Pediatrics Textbook of Pediatric Care. 2nd ed. Elk Grove Village, IL: American Academy of Pediatrics; 2017:2396-2406. Pediatric Care Online.\n• Rome ES. Obesity prevention and treatment. Pediatr Rev. 2011;32(9):363-372. doi:10.1542/pir.32-9-363.\n• Skinner AC, Ravanbakht SN, Skelton JA, Perrin EM, Armstrong SC. Prevalence of obesity and severe obesity in US children, 1999-2016. Pediatrics. 2018;142(3):e20181916. doi:10.1542/peds.2018-1916.\n• Styne DM, Arslanian SA, Connor EL, et al. Pediatric obesity-assessment, treatment, and prevention: an Endocrine Society clinical practice guideline. J Clin Endocrinol Metab. 2017;102(3):709-757. doi:10.1210/jc.2016-2573."}
{"id" : 1524, "question_text" : "A 3-month-old infant is brought to your office because he has been very irritable, and feeding poorly over the past 2 days. He vomited once this morning. The infant has had no fever or symptoms of an upper respiratory infection. He is a full-term infant with no significant past medical history and he has been growing and developing appropriately. His mother just returned to work last week and his father, who is unemployed, has been caring for the infant during the day. She thinks that the baby's fussiness may be due to the recent change in his routine. In your office, the baby's vital signs include a temperature of 37.2°C, heart rate of 160 beats/min, respiratory rate of 36 breaths/min, and pulse oximetry of 96% on room air. On physical examination, the baby is very fussy and the mother is having difficulty consoling him. There are several circular bruises on his right forehead and left forearm. The remainder of his physical examination is unremarkable. His mother denies any history of trauma. You advise the baby's mother that he should undergo further evaluation in the emergency department because of his concerning symptoms of irritability, poor feeding, vomiting, and bruising. Your differential diagnosis includes child abuse. At this point, the MOST appropriate manner to proceed given your concern for abuse is to", "options" : "[\"discuss with the mother, but do not report to child protective services unless additional injuries are identified\", \"discuss with the mother, but leave reporting to child protective services to emergency department providers to avoid conflicting information\", \"report to child protective services and discuss with the mother during the current office visit\", \"report to child protective services, but do not discuss with the mother until the emergency department evaluation has been completed\", \"report to child protective services only if the mother grants consent due to federal privacy legislation\"]", "explanation" : "The infant in the vignette presents with a clinical picture that is concerning for abusive head trauma, along with unexplained bruising that should raise suspicion for child abuse. Further evaluation in the emergency department is indicated at this time. The most appropriate manner to proceed given the concerns for abuse is to report the concerns to Child Protective Services and discuss them with the mother during the current office visit.\n\nChild abuse is unfortunately quite common and can result in significant morbidity and even mortality. For this reason, prompt reporting and appropriate management of cases of suspected child abuse is essential to the health and safety of children.\n\nAll physicians should understand their duty and ethical obligation to report suspected child abuse and neglect and to provide appropriate guidance and support to families during an investigation. Identifying and reporting suspected child abuse to Child Protective Services can be one of the most challenging responsibilities for pediatricians who have a unique and important opportunity to recognize the signs and symptoms of abuse and intervene in order to protect victims.\n\nWhen pediatricians have a reasonable suspicion that a child is a victim of abuse, the law mandates reporting to Child Protective Services. Failure to report suspected abuse can result in further injury to the patient involved (and to other children in the same environment) and can result in civil or criminal penalties for the physician. It is important to discuss the concern and requirement to report suspected child abuse with the child's parents whenever a pediatrician reports a concern to Child Protective Services. While such a discussion may be very difficult, it will enable more honest, open dialogue with parents during and following the ensuing investigation. In discussing suspected child abuse with parents, it is helpful to explain concern about an injury while not placing blame and to inform the parents that a report to Child Protective Services is required by law. Preparing the family for what will happen next is important. Though some families may choose not to return to a pediatrician's practice once a report is made, pediatricians should continue to offer support to parents throughout the entire process. It is important for pediatricians to realize that the parents bringing the child to medical attention may or may not be the perpetrators responsible for inflicting the identified injuries.\n\nFor the infant in the vignette, deferring reporting of suspected abuse to Child Protective Services until additional injuries are identified is not an appropriate approach. The presence of unexplained forearm and forehead bruising in this nonambulatory 3-month-old infant should prompt reporting to Child Protective Services, whether or not any additional injuries are identified on further evaluation. Any injury to a young preambulatory infant, including bruises, mouth injuries, fractures, and intracranial or abdominal injury, is suggestive of abuse and should be reported to Child Protective Services. Bruises are the most common and apparent injuries due to physical abuse, but are missed as a \"sentinel injury\" in nearly half of fatal and near-fatal abusive injuries. Even if the infant's further evaluation in the emergency department is negative for additional occult injuries, this should not be interpreted as false reassurance that child abuse can be ruled out.\n\nLeaving it up to emergency department providers is not the most appropriate course of action, even if concerns are discussed with the mother during the office visit. If any physician suspects that a patient is a victim of abuse, transferring the child to another physician or facility does not release him or her from the requirement to report suspected abuse. As an advocate for children, the pediatrician must report suspected abuse to the appropriate Child Protective Services and law enforcement authorities, regardless of the decision to transfer the child for further care. Reporting suspected abuse without discussing with the mother is also not the most appropriate course of action. As explained previously, it is important for pediatricians to discuss the need to report suspected child abuse to Child Protective Services with the child's parents whenever possible.\n\nFinally, obtaining consent from the patient's mother to report suspected child abuse is unnecessary and does not need to occur before making a report. The Health Insurance Portability and Accountability Act rules allow disclosure of protected health information to Child Protective Services without legal guardian authorization when the physician makes a mandatory report.\n\nPREP Pearls\n• Reporting to Child Protective Services is mandated by law whenever pediatricians have a reasonable suspicion that a child is a victim of abuse. Failure to report suspected abuse can result in further injury to the patient involved and civil or criminal penalties for the physician.\n• It is important to discuss the concern and requirement to report child abuse to Child Protective Services with the child's parents.\n• Any injury to a young preambulatory infant, including bruises, mouth injuries, fractures, and intracranial or abdominal injury are suggestive of abuse and should be reported to Child Protective Services.\n• If any physician suspects that a patient is a victim of abuse, transferring the child to another physician or facility for further care does not release him or her from the requirement to report suspected abuse.\n\nABP Content Specifications(s)\n• Understand the physician's duty and ethical obligation to report suspected child abuse or neglect\n• Provide apporpriate guidance and support to a family during an investigation of child abuse or neglect\n\nSuggested Readings\n• Christian CW, American Academy of Pediatrics Committee on Child Abuse and Neglect. The evaluation of suspected child physical abuse. Pediatrics. 2015;135(5):e1337-e1354. doi: http://dx.doi.org/10.1542/peds.2015-0356.\n• Wood JN, Callahan JM, Christian CW. Child abuse/assault. In: Shaw KN, Bachur RG, Chamberlain J, Lavelle J, Nagler J, Shook JE, eds. Textbook of Pediatric Emergency Medicine. 7th ed. Philadelphia, PA: Lippincott Williams and Wilkins; 2015:657-670."}
{"id" : 2074, "question_text" : "A 4-month-old girl is being evaluated at a health supervision visit. She was born at term, and had no medical problems. Her parents report that the girl's head \"wobbles\" a lot, and she does not roll or push herself up when prone. She babbles, smiles at her parents, and tracks objects closely with her eyes. Her growth has been tracking along the 10th percentile in length, weight, and occipital frontal head circumference. Physical examination shows an alert infant who is lying on her back with a frog-leg posture. With inspiration her abdomen is distended. She smiles responsively, tracks faces, and her facial movements are symmetric and appear to be full strength. Her tongue has a trembling appearance. Her arms and legs are hypotonic, with a small amount of spontaneous movement. No deep tendon reflexes are elicited. Of the following, the girl's MOST likely diagnosis is", "options" : "[\"cerebral palsy\", \"congenital muscular dystrophy\", \"infant botulism\", \"spinal muscular atrophy\"]", "explanation" : "The girl in the vignette has spinal muscular atrophy (SMA). She has weak, hypotonic limbs, areflexia, and paradoxical breathing, which implies weakness of muscles of respiration. The muscles of her face are spared, but she has tongue fasciculations. An additional finding in SMA can be finger trembling. The girl's physical examination findings suggest a disorder of the peripheral nervous system. Electromyography/nerve conduction study would confirm dysfunction of the motor neurons. Genetic testing for SMA would confirm the diagnosis.\n\nCongenital muscular dystrophy is a group of disorders that can also present with weakness, hypotonia, and hyporeflexia or areflexia. Affected infants may have bulbar weakness that causes poor feeding or choking. A high arched palate, joint contractures, or hyperflexibility may be noted on physical examination. Depending on the specific disorder, associated brain malformations, ophthalmologic abnormalities, or cardiomyopathy may be seen. The clinical presentation can have elements of both a central nervous system disorder and a peripheral nervous system disorder. Electromyography/nerve conduction study can indicate that there is a primary disorder of the muscles. Serum creatine kinase is sometimes elevated. A specific diagnosis can be made with muscle biopsy or genetic testing. Although the girl in the vignette has profound limb weakness and hypotonia, she does not have other findings seen in congenital muscular dystrophies, and she has the typical findings of SMA, so congenital muscular dystrophy is not the best answer.\n\nCerebral palsy can cause hypotonia, in which case, it is usually referred to as \"ataxic cerebral palsy.\" Ataxic cerebral palsy can be caused by prenatal or perinatal brain injury, especially to the cerebellum, or by cerebellar hypoplasia. There may be associated speech or language disorders and intellectual disability. Diagnosis is made based on history, physical examination, and abnormalities seen on brain imaging. Tongue fasciculations would not be present, so for the girl in the vignette, cerebral palsy is not the best answer.\n\nInfant botulism presents with a prodrome of constipation, followed by ptosis, decreased facial movements, head lag, and choking or aspiration. Limb weakness and hypotonia are less prominent early in the course. Deep tendon reflexes are usually diminished. The infant can appear to be asleep or encephalopathic due to bilateral ptosis, but careful observation will show normal limb movements even when the eyes are closed. If the examiner opens the eyelids, the infant will appear alert and interactive. These findings suggest a disorder of the peripheral nervous system. Electromyography/nerve conduction study can confirm a disorder of the neuromuscular junction but can be technically difficult in an infant. Diagnosis is made by identification of botulinum toxin in the stool. The girl in the vignette has limb weakness and hypotonia, but her face is spared, so infant botulism is not the best answer.\n\nPREP Pearls\n\nSpinal muscular atrophy presents in infancy with head lag; weak, hypotonic limbs; areflexia; paradoxical breathing; tongue fasciculations; and finger trembling. The muscles of facial expression are spared.\n\nInfant botulism presents with a prodrome of constipation, followed by ptosis, decreased facial movements, head lag, and choking or aspiration. Limb weakness is less prominent early in the course.\n\nMOCA-Peds Objective\n\nEvaluate a hypotonic infant.\n\nABP Content Specifications(s)/Content Area\n\nDifferentiate the findings associated with central nervous system causes of hypotonia from those of peripheral nervous system causes\n\nPlan the appropriate evaluation of hypotonia in patients of various ages\n\nSuggested Readings\n\nBeinvogl BC, Rosman NP, Baumer FM, et al. A 10-month-old with intermittent hypotonia and paralysis. Pediatrics. 2016;138(1):e20151896. doi: 10.1542/peds.2015-1896.\n\nLisi EC, Cohn RD. Genetic evaluation of the pediatric patient with hypotonia: perspective from a hypotonia specialty clinic and review of the literature. Dev Med Child Neurol. 2011;53(7):586-599. doi: 10.1111/j.1469-8749.2011.03918.x."}
{"id" : 1362, "question_text" : "A 2-month-old male infant presents to the emergency department in late winter with a 2-day history of cough, rhinorrhea, decreased feeding, and progressive difficulty breathing. Based on the infant's clinical picture and the case mix you have seen recently, you estimate an approximately 50% chance that he is infected with respiratory syncytial virus (RSV). You send a rapid RSV antigen test, which is positive. It is known that patients who have RSV are 5 times more likely to have a positive test than are patients who do not have the disease. Applying the nomogram shown in Item Q113, the post-test probability that he has RSV is", "options" : "[\"1%\", \"20%\", \"50%\", \"80%\", \"99%\"]", "explanation" : "The infant in the vignette has symptoms of bronchiolitis. The pre-test probability that he has a respiratory syncytial virus (RSV) infection is approximately 50%, based on your estimate of the prevalence of RSV and taking into account the entire clinical picture, including demographics, presentation, location, and season.\n\nTo best determine the post-test probability of whether he has RSV infection, the clinician should take into account:\n1. The probability that he has the disease prior to sending the test, which is known as the pre-test probability\n2. The degree to which the results of the test changes the probability, which is known as the likelihood ratio (LR)\n3. The result of the test\n\nThe likelihood ratio (LR) is the percentage of people with an illness with a given test result divided by the percentage of people without the illness with the same test result. Sensitivity, specificity, LR for a positive test, and LR for a negative test can all be calculated with a 2 x 2 table as shown in Item C113A. The Fagan nomogram (Item C113B) can be used to determine how a test with a known LR can predict the post-test probability if the pretest probability is known. This is done by drawing a straight line starting from the pretest probability through the likelihood ratio and recording the result of the post-test probability.\n\nIncidence is the rate of disease occurrence and prevalence is the number of cases of disease existing in a given population. Incidence and prevalence are directly proportional to pretest probability and therefore impact post-test probability when a test with a given LR is applied. For example, if one is considering whether a patient with fever and toxic appearance has Ebola in the United States, post-test probability with a positive test will still be very low, even if the test were relatively sensitive and specific, because the prevalence of Ebola is almost zero. Conversely, if one is clinically certain that a patient has a disease, the post-test probability would still be high even after a negative test. In both scenarios, the clinician should consider not testing because the result would not likely change management.\n\nFor the infant in the vignette, you have assigned a pretest probability of 50%, so a positive test must lead to a post-test probability of greater than 50%. Since patients with RSV are 5 times more likely to have a positive RSV test than patients without RSV, the LR is 5. To conclude an approximate post-test probability of 80%, a line is drawn starting from 50% through the LR of 5.\n\nPREP Pearls\n• The Fagan nomogram can be used to ascertain post-test probability when a likelihood ratio is applied to a given pretest probability.\n• The pretest probability takes into account the clinical picture and incidence and prevalence of a disease in the selected population.\n\nABP Content Specifications(s)\n• Understand pre-test and post-test probability\n• Understand prevalence and incidence\n\nSuggested Readings\n• Carvajal DN, Rowe PC. Research and statistics: sensitivity, specificity, predictive values, and likelihood ratios. Pediatr Rev. 2010;31(12):511-513. doi: http://dx.doi.org/10.1542/pir.31-12-511.\n• Crewe S, Rowe PC. Research and statistics: likelihood ratio in diagnosis. Pediatr Rev. 2011;32(7):296-298. doi: http://dx.doi.org/10.1542/pir.32-7-296."}
{"id" : 1511, "question_text" : "The father of a 3-year-old patient mentions at his son's health supervision visit that the family recently purchased a motorboat to use on a local lake. In addition to your typical safety recommendations, you counsel the family regarding safe boat use.\n\nOf the following, the intervention MOST likely to reduce this child's risk of drowning is", "options" : "[\"cardiopulmonary resuscitation training for the supervising adults\", \"inflatable arm bands (\\\"water wings\\\")\", \"personal flotation device (\\\"life jackets\\\")\", \"supervision by a responsible adult\", \"swimming lessons\"]", "explanation" : "The intervention most likely to reduce the risk of drowning for the boy in the vignette is a personal flotation device (PFD). Drowning is the second most common cause of death in children ages 1 to 4 years, surpassed only by congenital anomalies. After motor vehicle crashes, drowning is the second leading cause of injury-related death in all children younger than 14 years. Boys, young children, adolescents, African-Americans, and those with a history of seizure are at higher risk of drowning than the general pediatric population. Among adolescents and adults, alcohol use is involved in up to 70% of deaths associated with water recreation.\n\nPersonal flotation devices or \"life jackets\" are key to preventing drowning, especially in open water or boating-related incidents. One matched cohort study analyzing US Coast Guard data showed that wearing a PFD reduced the risk of drowning in a boating accident by nearly 50%. Children should wear PFDs that are approved by the US Coast Guard and are the appropriate size for the user.\n\nCardiopulmonary resuscitation (CPR) training for supervising adults is important because effective and timely CPR is associated with improved outcomes in drowning victims. However, primary prevention of drowning is the most important intervention. Inflatable arm bands (\"water wings\"), pool toys, and other foam or inflatable objects are not effective in reducing the risk of drowning. Personal flotation devices alone cannot prevent drowning; constant, focused adult supervision is also important. There are no data demonstrating that swimming lessons decrease the risk of drowning in children younger than 4 years of age.\n\nPREP Pearls\n• Drowning is a common cause of death in children.\n• Appropriately fitted personal flotation devices can significantly reduce the risk of drowning in open water and boating-related incidents.\n• Swimming lessons have not been shown to decrease the risk of drowning in children younger than 4 years of age.\n\nABP Content Specifications(s)\n• Counsel parents and children regarding safe boat use (eg, flotation devices, supervision)\n\nSuggested Readings\n• Cummings P, Mueller BA, Quan L. Association between wearing a personal floatation device and death by drowning among recreational boaters: a matched cohort analysis of United States Coast Guard data. Inj Prev. 2011;17(3):156-159. doi: http://dx.doi.org/10.1136/ip.2010.028688.\n• Meyer RJ, Theodorou AA, Berg RA. Childhood drowning. Pediatr Rev. 2006;27(5):163-168. doi: http://dx.doi.org/10.1542/pir.27-5-163.\n• US Centers for Disease Control and Prevention. Unintentional drowning: get the facts. US Centers for Disease Control and Prevention website. http://www.cdc.gov/HomeandRecreationalSafety/Water-Safety/waterinjuries-factsheet.html. Updated October 24, 2014.\n• Weiss J, American Academy of Pediatrics Committee on Injury, Violence, and Poison Prevention. Prevention of drowning. Pediatrics. 2010;126(1):e253-e262. doi: http://dx.doi.org/10.1542/peds.2010-1265.\n• Yuma P, Carroll J, Morgan M. A guide to personal flotation devices and basic open water safety for pediatric health care practitioners. J Pediatr Health Care. 2006;20(3):214-218. doi: http://dx.doi.org/10.1016/j.pedhc.2006.02.008."}
{"id" : 2008, "question_text" : "During a health supervision visit, the parents of a healthy 4-year-old boy raise concerns about his behavior. A review of the medical record shows that the boy has had normal growth and development. The boy entered a preschool program this year. His teacher reports that he does not always share well or follow the rules, and sometimes he is aggressive toward other students to \"get his way.\" The boy's parents deny behavior issues at home. He is their first child, so they have many questions about the most effective way to change his behavior.\n\nOf the following, the BEST intervention to recommend to this boy's parents is to", "options" : "[\"discuss with him alternative acceptable means to \\\"get his way\\\"\", \"initiate time-outs at home after bad conduct days\", \"provide positive praise and rewards after good behavior days\", \"take away a favorite toy or activity after bad conduct days\"]", "explanation" : "Providing positive praise and rewards after good behavior days is the best discipline technique parents can use to effectively change behaviors, particularly in preschool- and elementary school–aged children. Because this 4-year old just entered preschool and has no siblings, one should not assume he will automatically share well or follow rules. His occasional aggression to \"get his way\" likely reflects his limited understanding of how to be part of a group. By giving him specific and timely praise, he will learn desired actions and feel valued. Offering rewards after good behavior days through a graduated token economy strategy can help sustain behavior change.\n\nDiscipline is meant to teach and nurture correct behavior. It should involve the use of age- and developmentally appropriate methods that encourage progress toward independence, greater responsibility, and self-regulation. Effective discipline requires a positive, attentive, supportive, respectful, and consistent learning environment as a foundation. Good behaviors should be identified and recognized, along with intentional modeling of new skills, in an effort to teach and strengthen these behaviors. When undesirable behavior occurs, discipline strategies such as selectively ignoring the misbehavior, explaining limits, redirecting toward a correct behavior, allowing natural consequences, implementing time out, and withdrawing privileges may be used. These techniques are most successful when a positive relationship exists between the parent or caregiver and the child, and should be used in combination with strategies to stimulate more desirable behaviors. The child should not be encouraged to use alternative means to \"get his way\"; rather he should gain an understanding of group play and empathy for others.\n\nDiscipline does not equate to punishment. Punishment is the application of a negative stimulus, such as verbal reprimand or corporal punishment, to reduce or eliminate an undesirable behavior. The American Academy of Pediatrics \"Guidance for Effective Discipline\" policy statement notes that corporal punishment is ineffective, unnecessary, and potentially psychologically harmful. Pediatric health care providers can serve as valuable resources to parents in improving parenting skills by offering various discipline strategies.\n\nPREP Pearls\n\nDiscipline should be achieved through the use of age- and developmentally appropriate methods that nurture independence, progressive responsibility, and self-regulation.\n\nThrough specific and timely praise, children will learn desired actions and feel valued.\n\nA graduated token economy strategy can help sustain behavior change.\n\nABP Content Specifications(s)/Content Area\n\nAdvise parents regarding appropriate discipline and limit-setting for children of various ages\n\nSuggested Readings\n\nCommittee on Psychosocial Aspects of Child and Family Health. Guidance for effective discipline. Pediatrics. 1998;101(4):723728.\n\nLaptops J. The patient-parent-pediatrician relationship: everyday ethics in the office. Pediatr Rev. 2015;36(1):22-30. doi: 10.1542/pir.36-1-22.\n\nPipan ME, Blum NJ. Basics of child behavior and primary care management of common behavioral problems. In: Voigt RG, Macias MM, Myers SM, eds. American Academy of Pediatrics Developmental and Behavioral Pediatrics. Elk Grove, IL: American Academy of Pediatrics; 2011:37-58.\n\nSchooler SJ. Parental monitoring and discipline in middle childhood. Pediatr Rev. 2009:30(9):366-367. doi: 10.1542/pir.30-9-366."}
{"id" : 2290, "question_text" : "A 2-month-old uncircumcised boy is being seen by his pediatrician for painless debris on his penis that has been present for 1 week. He has otherwise been well, afebrile, and asymptomatic. Physical examination reveals the findings shown in the Figure. The remainder of the examination findings are normal. Of the following, the MOST appropriate next step is", "options" : "[\"prescribe mupirocin ointment\", \"prescribe nystatin cream\", \"provide reassurance\", \"refer to a urologist\"]", "explanation" : "Correct answer is C\n\nPREP Pearl(s)\nSmegma is a normal accumulation of epithelial debris under the penile foreskin, which is usually white or cream in color.\nSmegma is a benign and self-limiting condition. The foreskin will eventually become naturally retractile, and the epithelial debris will be released.\nForcible retraction of the penile foreskin can cause bleeding and lead to the development of adhesions.\n\nCritique\nThe physical examination findings of the infant in the vignette are most consistent with smegma, a white or cream-colored accumulation of epithelial debris under the penile foreskin. These findings are benign and self-limiting; therefore, reassurance is the best management option. Neither mupirocin ointment nor nystatin cream is indicated because there is no evidence of a fungal or bacterial infection. Referral to a urologist might be appropriate if the infant were experiencing pain or swelling, which are not present in this case.\n\nNo special care is required when smegma is noted on an infant's uncircumcised penis. The penile foreskin should not be forcibly retracted to release the smegma; forcible retraction can cause bleeding and lead to the development of adhesions. Over time, the foreskin will become naturally retractile and release any trapped smegma.\n\nSuggested Reading(s)\nAmerican Academy of Pediatrics. Care of the uncircumcised penis. In: Pediatric Patient Education; 2022. Updated December 15, 2022. Accessed February 26, 2024. Pediatric Patient Education Online\nAmerican Academy of Pediatrics. Foreskin care questions. In: Pediatric Patient Education; 2023. Updated July 13, 2023. Accessed February 26, 2024. Pediatric Patient Education Online\nLawless MR. The foreskin. Pediatr Rev. 2006;27(12):477-488. doi:10.1542/pir.27-12-477\nOettgen AB. Phimosis. In: McInerny TK, Adam HM, Campbell DE, DeWitt TG, Foy JM, Kamat DM, eds. American Academy of Pediatrics Textbook of Pediatric Care. American Academy of Pediatrics; 2021:chap 312. Accessed September 21, 2024. Pediatric Care Online\n\nContent Domain\nUrology\n\nLearning Objectives\nUnderstand that smegma is a normal accumulation of epithelial debris under the penile foreskin and no action is typically needed to treat this condition"}
{"id" : 2367, "question_text" : "A 3-year-old girl is undergoing a routine health supervision visit. She has a history of abdominal distension since birth. She has no vomiting, diarrhea, constipation, headaches, or easy fatigability. Her 5-year-old sibling has a history of autosomal recessive polycystic kidney disease. The girl's weight and height are at the 25th percentile for age. Her blood pressure is 122/82 mm Hg, her heart rate is 92 beats/min, and her respiratory rate is 16 breaths/min. Her abdomen is distended and soft, with mild hepatosplenomegaly, normal bowel sounds, and no free fluid. The remainder of her physical examination findings are unremarkable. Renal ultrasonography shows bilateral enlarged kidneys, poor corticomedullary differentiation, and multiple microcysts. Of the following, the condition MOST likely to occur in this child is", "options" : "[\"a cerebral aneurysm\", \"colonic diverticula\", \"a hepatic cyst\", \"portal fibrosis\"]", "explanation" : "PREP Pearl(s)\nNeonates with autosomal recessive polycystic kidney disease may exhibit nephromegaly and respiratory distress.\nChildren and adolescents with autosomal recessive polycystic kidney disease exhibit hypertension, urinary tract infection, nephrolithiasis, hematuria, proteinuria, and/or chronic kidney disease. \nAutosomal recessive polycystic kidney disease is always associated with portal and interlobular fibrosis of the liver and dilation of the intrahepatic bile ducts.\nCritique\nThe girl in the vignette has autosomal recessive polycystic kidney disease (ARPKD). Her abdominal distension (secondary to nephromegaly), hypertension, family history of ARPKD, and renal ultrasonographic findings of enlarged kidneys with microcysts favor this diagnosis. The most common condition associated with ARPKD in children is portal fibrosis, which may result in gastroesophageal varices.\nAutosomal recessive polycystic kidney disease is a rare genetic disorder with an incidence of 1 in 10,000 to 40,000 live births. One-third of cases present in infancy, one-third present between ages 1 and 20 years, and the remainder present in adulthood. Infantile forms are detected when prenatal ultrasonography shows enlarged hyperechoic kidneys. Oligohydramnios may be present in severe cases. At birth, abdominal masses may be palpated and the neonate may develop respiratory distress owing to diaphragmatic elevation, lung hypoplasia, or both. Infants can have difficult-to-treat hypertension. Affected children and adolescents exhibit hypertension, urinary tract infection, nephrolithiasis, hematuria, proteinuria, and/or chronic kidney disease. \nRenal ultrasonography in ARPKD shows large echogenic kidneys with poor corticomedullary differentiation and microcysts (<2 mm in diameter). Macrocysts are seen in autosomal dominant polycystic kidney disease; they are not usually present in infants with ARPKD. \nChildren with ARPKD always have associated portal and interlobular fibrosis of the liver and dilation of the intrahepatic bile ducts. Portal hypertension develops and may result in gastrointestinal bleeding, esophageal varices, hepatosplenomegaly, and hypersplenism. Anemia, thrombocytopenia, and leukopenia may occur owing to splenic sequestration. Children with ARPKD are at risk of developing acute cholangitis. \nHepatic cysts, colonic diverticula, and cerebral aneurysms are extrarenal manifestations seen in autosomal dominant polycystic kidney disease; they are not usually seen in children with ARPKD.\nChildren with ARPKD should be closely monitored for worsening kidney function, hypertension, and infections. Periodic endoscopy to monitor for gastroesophageal varices and portal hypertension is recommended. Once progressive renal insufficiency ensues, dialysis or kidney transplant is required. \nSuggested Reading(s)\nBenun J, Lewis C. Polycystic kidney disease. Pediatr Rev. 2009;30(10):e78-e79. doi:10.1542/pir.30-10-e78\nJanjua HS, Lam KS, Gupta V, Krishna S. Congenital anomalies of the kidneys, collecting system, bladder, and urethra. Pediatr Rev. 2019;40(12):619-626. doi:10.1542/pir.2018-0242\nContent Domain\nGenetics\nABP Content Specification(s) / Content Area(s)\nRecognize the clinical findings associated with autosomal-recessive polycystic kidney disease in patients of various ages"}
{"id" : 1569, "question_text" : "A 15-year-old adolescent girl is seen in your office for her routine health supervision visit. Her mother reports no concerns, other than her daughter's tendency to be moody and withdrawn, which the family attributes to being a teenager. During the confidential psychosocial interview, the patient reports that she often feels sad. Upon further questioning about her home environment, she tells you that she lives with her mother, stepfather, and younger stepsister. She rarely sees her biological father. Although her stepfather has been present in the home since she was 3 years of age, he is not very involved with her. He often spends time with her stepsister, but leaves her out. He never buys her gifts and has refused to pay for essential items, such as clothing and hygiene products. She hears him state that her own father should be paying for these items. She feels he disciplines her more harshly than her stepsister and reports that he has called her names, but he has never physically harmed her or threatened to injure her. Of the following, the MOST appropriate management plan is to", "options" : "[\"acknowledge that differential treatment by the nonbiological parent is understandable and not harmful\", \"agree to keep the patient's conversation confidential and re-evaluate at her next visit\", \"encourage the patient to seek alternative living arrangements\", \"reassure the mother that emotional lability and the desire to spend time alone is typical of teenagers\", \"recommend individual and family counseling\"]", "explanation" : "The teenager in this vignette is the victim of psychological or emotional abuse. Individual and family counseling is needed to minimize the negative impact on the girl and to hopefully improve the relationships within the family.\n\nChild maltreatment includes physical abuse, sexual abuse, emotional abuse, and neglect of an individual younger than 18 years by a parent or other custodial caregiver. Physical abuse is the intentional use of physical force, such as hitting, kicking, choking, shaking, and burning. Sexual abuse includes fondling and any other form of sexual activity with a child. Emotional or psychological abuse/maltreatment refers to any behavior that negatively affects one's feelings of self-worth or emotional well-being. Neglect is the failure to have one's basic needs met for shelter, food, clothing, education, and health care.\n\nPsychological maltreatment is the most prevalent form of abuse, yet it is often difficult to identify because it is hidden. Patients and families may rationalize or minimize the abusive behaviors. Often the maladaptive behaviors of the victim, such as the moodiness, sadness, and desire to withdraw, as seen in the girl in this vignette, are mischaracterized as the child's fault or misperceived as related to the child's temperament rather than understood to be a consequence of abuse. Caregiver behaviors that constitute maltreatment of the girl in this vignette are her biological father's abandonment and her stepfather's passive detachment, unresponsiveness, active omission from father-daughter relationships, commission of verbal belittling and name-calling, and refusal to provide for essential needs. This differential treatment is psychologically harmful and requires the physician to address the concern with the family in a sensitive and supportive manner on behalf of the teen. This discussion should occur at the time of the visit. Simply offering reassurance and re-evaluation is not appropriate. In some cases, seeking alternative living arrangements may be in the best interest of the patient, but it would not be the first step in management.\n\nAll forms of child maltreatment can negatively affect the cognitive, social, emotional, and physical development of the abused individual. The impact of psychological maltreatment in early childhood is particularly profound; it interferes with the development of secure attachments and healthy peer and intimate relationships. Adults who were abused as children are at greater risk of addiction (alcohol, drugs, or tobacco), mental health problems (including suicide and eating disorders), high-risk sexual behaviors, and other chronic conditions and diseases.\n\nPREP Pearls\n• Child maltreatment includes physical abuse, sexual abuse, emotional abuse, and neglect of an individual younger than 18 years by a parent or other custodial caregiver.\n• Emotional or psychological abuse/maltreatment refers to any behavior that negatively affects one's feelings of self-worth or emotional well-being.\n• Psychological abuse/maltreatment, the most prevalent form of abuse, is difficult to identify and is often under-reported.\n• The impact of psychological abuse/maltreatment in early childhood is particularly profound; it interferes with the development of secure attachments and healthy relationships and increases the risk of some chronic conditions in adulthood.\n\nABP Content Specifications(s)\n• Recognize the history, signs, and symptoms indicative of psychological abuse\n• Understand the behavioral and emotional consequences of psychological abuse\n\nSuggested Readings\n• Hibbard R, Barlow J, MacMillan H; Committee on Child Abuse and Neglect and American Academy of Child and Adolescent Psychiatry, Child Maltreatment and Violence Committee. Psychological maltreatment. Pediatrics. 2012;130(2):372–378. doi: http://dx.doi.org/10.1542/peds.2012-1552.\n• National Center for Injury Prevention and Control Division of Violence Prevention. Understanding child maltreatment. 2016. https://www.cdc.gov/violenceprevention/pdf/understanding-cm-factsheet.pdf."}
{"id" : 3738, "question_text" : "A 16-year-old adolescent boy undergoes a follow-up evaluation after a recent hospital stay for a deep venous thrombosis of his right lower extremity. He is taking anticoagulation medication. He has a history of mild intellectual disability, severe myopia, and downward dislocation of the lens. He has tall stature, long limbs, pectus excavatum, moderate levothoracic scoliosis, high-arched palate, and arachnodactyly. He has no joint hypermobility. Complete blood cell count is unremarkable. Findings of a coagulation evaluation were unremarkable, other than an abnormal plasma amino acid analysis that showed a methionine level of 70 μmol/L (reference range 10-40 μmol/L). A genetic disorder is suspected. Of the following, the BEST test to determine this patient's diagnosis is", "options" : "[\"FBN1 analysis\", \"NSD1 analysis\", \"total plasma homocysteine\", \"urine S-sulfocysteine\"]", "explanation" : "Correct Answer: C\nThe history, physical examination, and laboratory findings for the patient in the vignette suggest that he has homocystinuria, an amino acid disorder. A markedly increased total plasma homocysteine level, in conjunction with an elevated methionine level on serum amino acid analysis, would be biochemically consistent with a diagnosis of homocystinuria, also known as cystathionine β-synthase (CBS) deficiency. The diagnosis can be further confirmed with the detection of biallelic pathogenic mutations in CBS because this is an autosomal recessive disorder. It is identified on newborn screening via tandem mass spectrometry which detects most but not all cases.\n\nHomocystinuria characteristically presents with a marfanoid habitus (tall stature, pectus excavatum, long limbs, and scoliosis), eye findings (severe myopia and risk of downward ectopia lentis), intellectual disability/developmental delay, and vascular thromboembolic events. Downward dislocation of the eye lens is typical rather than the upward dislocation seen in Marfan syndrome. Vascular events are the primary cause of morbidity and mortality in CBS deficiency.\n\nManagement of homocystinuria involves prevention of thromboembolic events and normalization or near-normalization of the plasma homocysteine concentration. Treatment includes vitamin B6 (pyridoxine) supplementation for those who are B6 responsive, a methionine-restricted diet, betaine therapy, and folate and vitamin B12 supplementation. Betaine therapy yields an alternate remethylation pathway for the conversion of homocysteine to methionine and is helpful in preventing thrombosis.\n\nAmino acid disorders typically involve impairment in the breakdown of amino acids or in the transport of amino acids into the cells, leading to a buildup of harmful substances in the body that can lead to serious or life-threatening clinical presentations. Other amino acid disorders include isovaleric acidemia, propionic acidemia, maple syrup urine disease, phenylketonuria, methylmalonic acidemia, and tyrosinemia. Many of these conditions present with neonatal onset of irritability, poor feeding, seizures, and worsening metabolic encephalopathy; others, such as phenylketonuria, present with progressive and irreversible intellectual disability because of the accumulation of a specific harmful amino acid. Most amino acid disorders are included in statewide newborn screening programs because of the importance of early intervention and treatment in optimizing clinical outcomes.\n\nFBN1 analysis would be an appropriate test if Marfan syndrome were highly suspected; however, the elevated methionine and homocysteine combined with the downward ectopia lentis and mild intellectual disability would make homocystinuria more likely in this clinical vignette. NSD1 mutations cause Sotos syndrome, which presents as an overgrowth syndrome with learning disability/developmental delay and a distinctive facial appearance (sparse frontotemporal hair, downslanting palpebral fissures, prominent forehead, and elongated face). Isolated sulfite oxidase deficiency (ISOD) manifests with elevated urine S-sulfocysteine levels and reduced levels of total plasma homocysteine. Clinically ISOD presents from birth to 18 months of age with ectopia lentis, seizures, feeding difficulties, episodic encephalopathy, developmental delay/regression, and an evolving movement disorder (dystonia, choreoathetosis, and ataxia) without evidence of overgrowth or tall stature.\n\nPREP Pearls\n• Homocystinuria, also known as cystathionine β-synthase deficiency, characteristically presents with a marfanoid habitus (tall stature, pectus excavatum, long limbs, and scoliosis), eye findings (severe myopia and risk of downward ectopia lentis), intellectual disability/developmental delay, and vascular thromboembolic events.\n• Cardinal biochemical features of homocystinuria include a markedly elevated total plasma homocysteine and elevated methionine level on serum amino acid analysis.\n• Many amino acid disorders are detectable on newborn screening via tandem mass spectrometry; early intervention, both dietary and via medical therapies, will optimize patient outcomes and save lives.\n\nABP Content Specifications(s)\n• Recognize the clinical features associated with a disorder of amino acid metabolism other than phenylketonuria\n\nSuggested Readings\n• Baric I, Staufner C, Augoustides-Savvopoulou P, et al. Consensus recommendations for the diagnosis, treatment and follow-up of inherited methylation disorders. J Inherit Metab Dis. 2017;40:5-20. doi:10.1007/s10545-016-9972-7.\n• Morris AA, Kožich V, Santra S, et al. Guidelines for the diagnosis and management of cystathionine ß-synthase deficiency. J Inherit Metab Dis. 2017;40:49-74. doi:10.1007/s10545-016-9979-0.\n• Rios A, Adams DJ. Specific congenital metabolic diseases. In: McInerny TK, Adam HM, Campbell DE, DeWitt TG, Foy JM, Kamat DM, eds. American Academy of Pediatrics Textbook of Pediatric Care. 2nd ed. Elk Grove Village, IL: American Academy of Pediatrics; 2017:938-963. Pediatric Care Online.\n• Sacharow SJ, Picker JD, Levy HL. Homocystinuria caused by cystathionine beta-synthase deficiency. GeneReviews. https://www.ncbi.nlm.nih.gov/books/NBK1524/.\n• Saronwala A, Kubendran S, Kahler SG. Screening for genetic-metabolic diseases. In: McInerny TK, Adam HM, Campbell DE, DeWitt TG, Foy JM, Kamat DM, eds. American Academy of Pediatrics Textbook of Pediatric Care. 2nd ed. Elk Grove Village, IL: American Academy of Pediatrics; 2017:210-225. Pediatric Care Online."}
{"id" : 2254, "question_text" : "An 8-year-old girl is seen in the emergency department due to an episode of hemoptysis. Her parents report a several month history of occasional dyspnea and cough. On physical examination, she appears pale. Her temperature is 37.2 °C, her heart rate is 90 beats/min, her respiratory rate is 36 breaths/min at rest, and her oxygen saturation is 94% in room air. On auscultation, crackles are heard throughout her lung fields. The remainder of her examination findings are normal. Laboratory studies are obtained, including complete blood count with differential, coagulation studies, complete metabolic panel, urinalysis, serum iron, serum ferritin, rapid respiratory viral panel, blood culture, and screening for several autoimmune disorders. Other than evidence of iron deficiency anemia, the girl's laboratory results are within normal limits. Chest radiograph is shown (Figure). The girl is admitted to the hospital for observation and further evaluation. Of the following, the BEST next step in evaluation is", "options" : "[\"bronchoscopy with bronchoalveolar lavage\", \"high-resolution chest computed tomography\", \"lung biopsy\", \"serologic testing for IgA anti-tissue transglutaminase antibodies\"]", "explanation" : "The afebrile child in the vignette has been hospitalized due to hemoptysis. Her findings of pallor, tachypnea, and diffuse crackles on lung examination are suggestive of alveolar hemorrhage. She is currently stable. The results of her laboratory studies were reassuring with the exception of evidence of iron deficiency anemia. Therefore, the best next step in her evaluation is to obtain high resolution chest computed tomography to evaluate her for idiopathic pulmonary hemosiderosis (IPH).\nIdiopathic pulmonary hemosiderosis is a rare disease that primarily occurs in the pediatric population. It is characterized by recurrent episodes of diffuse alveolar hemorrhage with no identifiable cause. The etiology of IPH is unknown. However, an immunologic mechanism has been suggested. A link has been identified between IPH and certain ingested protein antigens. There is a rare association of IPH with celiac disease, known as Lane-Hamilton syndrome. Affected individuals have improvement of pulmonary symptoms when adapting a gluten-free diet. Screening for celiac disease with IgA anti-tissue transglutaminase antibodies is recommended for individuals with a diagnosis of IPH. There appears to be a genetic predisposition for IPH; there may be multiple family members affected. Down syndrome has been identified as a possible risk factor. Environmental exposure to secondhand smoke is an additional risk factor for IPH.\nThe clinical manifestations of IPH in children vary; they may include recurrent episodes of alveolar hemorrhage, dyspnea, cough, fever, faltering growth, and anemia. Onset of symptoms may be acute or progress slowly. Children with suspected IPH should be monitored closely due to the potential for rapid respiratory decompensation. The differential diagnosis of alveolar hemorrhage is extensive and includes infectious processes, immune mediated diseases, thromboembolic disease, bleeding disorders, and rheumatic diseases. A substantial workup is required to rule out alternative diagnoses before a diagnosis of IPH can be made. Factors considered when making the diagnosis include clinical presentation, results of laboratory testing, high-resolution computed tomography findings, and bronchoalveolar lavage findings. Positive testing for celiac disease with negative immune-mediated disease serologies is also consistent with IPH.\nHigh-resolution chest computed tomography is the imaging of choice when making a diagnosis of IPH. Common findings include ground glass opacities, and with recurrent hemorrhage, hemosiderin is deposited in the interstitium, causing interlobular septal thickening. While chest radiography may reveal patchy opacities, depending on the severity of alveolar hemorrhage, the detail provided by computed tomography scan has a higher diagnostic yield. Bronchoscopy with bronchoalveolar lavage with cytology is required for diagnosis of IPH, however, airway evaluation is best performed after appropriate imaging, to better focus on areas of concern. Hemosiderin-laden macrophages are consistent with chronic alveolar hemorrhage. Lung biopsy is only required for the diagnosis of IPH when uncertainty remains after performing appropriate laboratory and imaging studies and bronchoalveolar lavage.\nWhile no laboratory studies are diagnostic, evidence of iron deficiency anemia with a normal or high serum ferritin level are consistent with IPH. Iron deficiency anemia occurs secondary to alveolar hemorrhage with depletion of iron stores. The serum ferritin level is normal or even high in children with IPH, reflecting the hemosiderin iron present in the alveolar macrophages after each episode of hemorrhage.\nIn addition to supportive care, treatment for IPH includes respiratory support, which may range from supplemental oxygen to full ventilatory support. Systemic glucocorticoids are the primary form of therapy for acute episodes of alveolar hemorrhage and to prevent recurrence. Due to the association between IPH and celiac disease, a gluten-free diet may also be implemented.\nDue to recurrent alveolar hemorrhage, free iron accumulates in pulmonary tissue, which may lead to free radicals, subsequent tissue remodeling, and resulting pulmonary fibrosis. Children with IPH must be monitored closely; this includes chest imaging, pulse oximetry, and spirometry with diffusing capacity for carbon monoxide.\nSuggested Reading(s)\nCrowe O, Maiorella RM, Tanimoto A, Patel SJ. Anemia and a new supplemental oxygen requirement in a 2-year-old boy. Pediatr Rev. 2023;44(S1):S81–S84. doi:10.1542/pir.2022-005663\nIoachimescu OC, Sieber S, Kotch A. Idiopathic pulmonary haemosiderosis revisited. Eur Respir J. 2004;24(1):162-170. doi:10.1183/09031936.04.00116302\nSaha BK. Idiopathic pulmonary hemosiderosis: a state of the art review. Respir Med. 2021;176:106234. doi:10.1016/j.rmed.2020.106234\nSchroeder S. Hemoptysis. In: McInerny TK, Adam HM, Campbell DE, DeWitt TG, Foy JM, Kamat DM, eds. American Academy of Pediatrics Textbook of Pediatric Care. American Academy of Pediatrics; 2021:chap 161. Accessed September 21, 2024. Pediatric Care Online\nTarchichi T, Lupo K. Case 1: hemoptysis, hypoxia, and anemia in a 10-year-old girl. Pediatr Rev. 2018;39(3):140. doi:10.1542/pir.2016-0144\nContent Domain\nPulmonology\nLearning Objectives\nDescribe clinical findings and risk factors associated with idiopathic pulmonary hemosiderosis"}
{"id" : 1543, "question_text" : "You are seeing a 15-year-old boy in your office for a sports preparticipation evaluation and annual health supervision visit. The boy is a competitive basketball player and his parents ask if their son should use a mouthguard during sports participation. Of the following, the MOST accurate statement to include in your discussion with the family is that", "options" : "[\"basketball has one of the highest rates of dental injuries among high school sports\", \"most dental injuries occur in athletes who were wearing a mouthguard at the time of injury\", \"the rate of dental injuries is higher during sports practice sessions than during competition\", \"the risk of dental injury is much lower if the boy uses a professionally fitted rather than a self-fitted mouthguard\", \"the use of a mouthguard will decrease the boy's risk of sports concussion\"]", "explanation" : "The boy in the vignette should use a mouthguard during sports participation because of the high rate of dental injuries during basketball. Dental injuries are common during youth sports. In boys' high school sports, the highest rates of dental injuries occur during basketball, baseball, wrestling, and soccer. Among girls' sports, field hockey, softball, basketball, and lacrosse have the highest rates of dental trauma. Even athletes participating in noncontact sports, such as swimming and track, are at risk for dental injuries. These injuries typically occur as a result of player-to-player contact, getting hit by a ball or sports equipment, or when a player hits the ground.\n\nThe American Dental Association recommends that athletes use mouthguards for 29 common sports and physical activities. Use of a mouthguard decreases the risk of various oral injuries, including tooth avulsions, tooth fractures, and lacerations. In sports that do not require the use of a mouthguard by league rules, most dental injuries occur in athletes who were not wearing mouthguards.\n\nAlthough the rate of dental injury is highest during competition, athletes generally spend many more hours practicing than competing; thus, the overall risk of injury may be higher during practice. Mouthguards should therefore be worn during both practice and competition.\n\nCustom mouthguards, made for an individual by a dental health professional, may fit better and be more comfortable than less expensive \"boil and bite\" or off-the-shelf mouthguards. However, these have not consistently been shown to decrease injury rates compared with off-the-shelf models. Although mouthguards decrease the risk of dental injury, they do not provide protection against sports concussions.\n\nPREP Pearls\n• Use of a mouthguard during sports activities decreases oral injuries, including tooth avulsions, tooth fractures, and lacerations.\n• Professionally fitted mouthguards have not consistently been shown to decrease oral injury rates compared with \"boil and bite\" or off-the-shelf mouthguards.\n\nABP Content Specifications(s)\n• Recognize the indications for the use of a mouth guard during sports activities\n\nSuggested Readings\n• ADA Council on Access, Prevention and Interprofessional Relations; ADA Council on Scientific Affairs. Using mouthguards to reduce the incidence and severity of sports-related oral injuries. J Am Dent Assoc. 2006;137(12):1712–1720.\n• Collins CL, McKenzie LB, Ferketich AK, Andridge R, Xiang H, Comstock RD. Dental injuries sustained by high school athletes in the United States, from 2008/2009 through 2013/2014 academic years. Dent Traumatol. 2016;32(2):121–127. doi: http://dx.doi.org/10.1111/edt.12228.\n• McGuine TA, Hetzel S, McCrea M, Brooks MA. Protective equipment and player characteristics associated with the incidence of sport-related concussion in high school football players: a multifactorial prospective study. Am J Sports Med. 2014;42(10):2470–2478. doi: http://dx.doi.org/10.1177/0363546514541926."}
{"id" : 1876, "question_text" : "A 5-year-old boy is brought to the emergency department for wheezing with a viral respiratory infection that has been present for the last 4 days. This is his third visit this year to emergency or urgent care services for the same symptoms. He uses an albuterol inhaler without a holding chamber when he has audible wheezing, but his mother says it never completely stops his symptoms. He has had 3 courses of oral prednisolone in the last year. He wheezes only with viral illnesses, but does cough at night several times weekly. His teacher has said that he frequently seems short of breath when playing at recess or in gym class. He has a temperature of 37.2°C, heart rate of 135 beats/min, respiratory rate of 30 breaths/min, and oxygen saturation of 94% on room air. He looks uncomfortable and has intercostal and suprasternal retractions. Breath sounds are generally diminished, and there is a faint end-expiratory wheeze heard throughout his chest. Cardiovascular examination findings are normal, and his skin is warm and well perfused. There is concern that the boy has previously unrecognized persistent asthma triggered by viral respiratory infections. His acute symptoms are managed with bronchodilators and systemic steroids. Of the following, the BEST long-term approach to his care would be to begin an", "options" : "[\"inhaled anticholinergic agent\", \"inhaled corticosteroid\", \"oral antihistamine\", \"oral leukotriene receptor antagonist\"]", "explanation" : "The boy in this vignette has persistent rather than intermittent asthma and needs daily controller therapy in addition to bronchodilators given for acute symptoms. Inhaled corticosteroids are the most effective controller therapy for persistent asthma of any severity. This patient likely has moderate persistent asthma, based on his symptoms of frequent exercise-induced dyspnea and night cough. Low- to medium-dose inhaled steroids would be the first intervention, with escalation of steroid dose or addition of a second controller therapy as the next intervention. The most appropriate second controller therapy would be a leukotriene modifier. Pharmacotherapy should be accompanied by education of the patient and his caregivers about asthma pathophysiology, identification of triggers and recommendations for avoidance, and a written asthma action plan for escalation of therapy with mild and more severe exacerbations.\n\nAn oral leukotriene antagonist as primary therapy might be appropriate for mild persistent asthma, but inhaled corticosteroids are the most effective controller therapy and are a better choice in the context of moderate persistent asthma.\n\nAn inhaled anticholinergic agent (eg, ipratropium bromide) is appropriate for second-line rescue therapy as a supplement to albuterol for patients in the emergency department with a severe asthma exacerbation or for home therapy in a limited number of patients demonstrated to respond better to ipratropium combined with albuterol than to albuterol alone. Anticholinergic agents are not appropriate for primary controller therapy of asthma.\n\nOral antihistamines would be beneficial for allergies that may be hidden triggers for this patient, but they are not an effective primary treatment for persistent asthma.\n\nThe boy in this vignette has recognized asthma symptoms with viral infections, but likely has other triggers given his subtle symptoms between acute wheezing episodes. Environmental tobacco smoke exposure, allergies, and sources of air pollution (indoors and outdoors) should be addressed as possible unrecognized triggers.\n\nPREP Pearls\n\nInhaled corticosteroids are the most effective controller therapy for persistent asthma of any severity.\n\nIntermittent wheezing symptoms may be associated with persistent asthma and airway reactivity that is often underappreciated.\n\nABP Content Specifications(s)/Content Area\n\nPlan appropriate management for wheezing of various etiologies\n\nPlan the appropriate clinical and diagnostic evaluation of wheezing of various etiologies\n\nSuggested Readings\n\nNational Asthma Education and Prevention Program. Expert Panel Report 3: Guidelines for the Diagnosis and Management of Asthma, Summary Report 2007. Bethesda, MD: National Institutes of Health; 2007. NIH publication 08-5846. https://www.nhlbi.nih.gov/files/docs/guidelines/asthsumm.pdf.\n\nRachelefsky G. Inhaled corticosteroids and asthma control in children: assessing impairment and risk. Pediatrics. 2009;123(1):353-366. doi: 10.1542/peds.2007-3273.\n\nWood PR, Hill VL. Practical management of asthma. Pediatr Rev. 2009;30(10):375-385. doi: 10.1542/pir.30-10-375."}
{"id" : 3009, "question_text" : "A 4-year-old girl is seen for worsening of a persistent cough and wheezing that has been present for the last 3 years. She has been treated with inhaled budesonide and albuterol without resolution of her symptoms. She has intermittent pain in the right lateral abdomen that does not interfere with sleep or any activities. On physical examination she is healthy appearing, and her vital signs and growth parameters are normal. The oxygen saturation on pulse oximetry is 96% on room air. She has nasal congestion but otherwise normal upper airway examination findings. There is no increased work of breathing. There are no adventitious sounds in the chest, but breath sounds are diminished on the right. Cardiac examination findings are normal. The liver is palpable 5 cm below the right costal margin, but the spleen is not palpable. There is no digital clubbing. A chest radiograph is obtained (Item Q212). Of the following, the MOST appropriate next step in making a diagnosis is", "options" : "[\"computed tomography of the chest with intravenous contrast\", \"flexible bronchoscopy and bronchoalveolar lavage\", \"modified barium swallow with speech therapist\", \"thoracoscopic biopsy of right lower lobe\"]", "explanation" : "The girl in the vignette has abnormal radiographic findings in the right lung; areas of hyperinflation and cyst formation suggest a congenital pulmonary airway malformation that needs further anatomic definition. Computed tomography of the chest with intravenous contrast will provide additional definition of lung structure.\n\nFlexible bronchoscopy will provide evaluation of airway anatomy, but will not help evaluate parenchymal abnormalities or airway abnormalities beyond the segmental bronchus. A modified barium swallow would assist in evaluating the integrity of this girl's swallow and her potential for aspiration, but neither the history nor the radiographic findings is suggestive of aspiration. A thoracoscopic biopsy would help evaluate the child's pulmonary microanatomy, but not with the overall structural abnormality.\n\nThe computed tomography findings in this patient (Item C212A, Item C212B, Item C212C) confirm a large malformation occupying most of the right chest composed of multiple cysts with atelectasis of innate normal lung. Item C212C shows a truncated right lower lobe posterior segment bronchus indicating the source of the lesion. The multicystic lesion in that segment fills the entire right chest, with the remaining lobes and segments being almost completely atelectatic.\n\nCongenital pulmonary airway malformation is part of the spectrum of cystic lesions of the lower airway, which also includes congenital lobar emphysema. Many lesions are diagnosed with prenatal ultrasonography; 70% of those resolve or decrease in size before birth, 10% remain unchanged, and 20% increase in size. Those presenting in the newborn period with respiratory compromise may require surgical resection. Some have no early symptoms or their symptoms are mild enough that they are not diagnosed until much later.\n\nThe presence of persistent or unusual cough, wheezing not responding to appropriate treatment for asthma, or recurrent respiratory infections indicates a need for additional investigation. Plain radiography of the chest is recommended as the first imaging modality. Results may suggest the need for additional testing. In most cases, computed tomography of the chest is the best additional imaging modality to further investigate anomalies of the pulmonary parenchyma or airways identified on chest radiography.\n\nPREP Pearls\n• Most congenital pulmonary airway malformations are diagnosed prenatally; 70% of those diminish or resolve before birth.\n• Rarely, congenital pulmonary airway malformation may present later in life with chronic respiratory symptoms.\n• Congenital pulmonary airway malformation and congenital lobar emphysema represent a spectrum of airway anomalies present at birth.\n\nABP Content Specifications(s)\n• Recognize the clinical findings associated with congenital malformations of the lower airway\n\nSuggested Readings\n• Cook J, Chitty LS, Decoppi P. The natural history of prenatally diagnosed congenital cystic lung lesion: long term follow up of 119 cases. Arch Dis Child. 2017;102(9):798-803. doi: 10.1136/archdischild-2016-311233.\n• McBride W. Congenital lesions of the lung. NeoReviews. 2016;17(5):e263-e270. doi: 10.1542/neo.17-5-e263.\n• Nafday SM, Long C. Respiratory distress and breathing disorders in the newborn. In: McInerny TK, Adam HM, Campbell DE, DeWitt TG, Foy JM, Kamat DM, eds. American Academy of Pediatrics Textbook of Pediatric Care. 2nd ed. Itasca, IL: American Academy of Pediatrics; 2016; chap 100:867-887. Pediatric Care Online.\n• O'Sullivan B, Kinane TB. Congenital lung anomalies. In: Light MJ, Blaisdell CJ, Homnick DN, eds. Pediatric Pulmonology. Itasca, IL. American Academy of Pediatrics; 2011;chap 14: 277-307."}
{"id" : 2902, "question_text" : "A 6-year-old girl with a 1-day history of fever, limp, and pain in the right lower extremity is brought to the emergency department. Two weeks ago, she had a mild upper respiratory tract infection. There has been no recent trauma. She is alert and interactive and has a temperature of 39.5°C. She is lying with her right lower extremity abducted and externally rotated. Examination of the musculoskeletal system reveals discomfort with internal and external rotation of the right hip. The remainder of the physical examination findings are normal. Laboratory findings are notable for a white blood cell count of 18,000/μL (18 × 109/L) with 70% segmented neutrophils and an elevated C-reactive protein level. Plain radiography of the right hip has normal findings, and a blood culture is obtained. Of the following, the BEST next step in management is to", "options" : "[\"administer ibuprofen\", \"administer intravenous clindamycin\", \"perform ultrasonography of the right hip\", \"schedule orthopedic consultation for the next day\"]", "explanation" : "For the child described in the vignette, the clinical presentation of acute onset of fever, limp, and limited range of motion of the right hip associated with leukocytosis and high C-reactive protein (CRP) raises concern regarding septic arthritis of the hip. The best next step in management is to obtain an ultrasonogram of the hip to help in excluding the diagnosis of septic arthritis. Detection of a hip effusion requires a diagnostic aspiration of the joint space and analysis of the synovial fluid. If the diagnosis of septic arthritis is suspected or confirmed, prompt surgical drainage and irrigation of the joint space are recommended. Delay in surgical drainage can result in serious complications (eg, aseptic necrosis of the femoral head) because increased intra-articular pressure resulting from hip joint space infection compromises vascular flow. Other management options after hip aspiration include obtaining a blood culture, initiating empiric antibiotic therapy, and instituting pain management.\n\nSeptic arthritis in childhood most frequently results from hematogenous spread of bacteria, although infection can also occur owing to local spread from a contiguous infection, as well as because of traumatic or surgical infection.The bacterial etiology of septic arthritis varies with age. In neonates, Staphylococcus aureus, group B Streptococcus, and gram-negative enteric bacilli are the usual pathogens. Beyond the neonatal age group, S aureus is the most common infecting organism in all age groups. Kingella kingae is a common cause of septic arthritis in children younger than 5 years. Other important pathogens include group A streptococci and Streptococcus pneumoniae. In unimmunized children, Haemophilus influenzae type b must be considered. Neisseria gonorrhoeae must be considered in neonates and sexually active adolescents. In addition to S aureus, Salmonella spp frequently cause osteomyelitis and septic arthritis in patients with sickle cell anemia. Immunocompromised hosts are at high risk of developing gram-negative bacterial infections. In chronic septic arthritis, mycobacteria and fungi should be considered.\n\nSeptic arthritis is usually accompanied by fever, malaise, poor appetite, and irritability. In the infant or neonate, there may be fever, poor feeding, lethargy, and pseudoparalysis of the extremity. In the older child, the signs are more localized. Approximately 75% of cases of septic arthritis involve the joints of the lower extremities. Limp or refusal to walk is the most frequent presentation. The knee is the most commonly involved site, followed by the hip and ankle, although the elbow and shoulder also may be affected. Examination of the infected joint will reveal local erythema, warmth, swelling, pain, and decreased range of motion. Joint dislocation may be observed. Smaller distal joints are less likely to be affected than are larger proximal joints. Over 90% of children with septic arthritis have monoarticular joint infections. Polyarticular infections may occur with certain pathogens (eg, N gonorrhoeae, Neisseria meningitidis, Salmonella spp, and, rarely, S aureus).\n\nClinical suspicion of septic arthritis should lead to the examination of the joint fluid. Synovial fluid aspirated should be sent for Gram staining, aerobic and anaerobic bacterial cultures, and white blood cell (WBC) count with differential. Synovial fluid in septic arthritis is typically turbid or grossly purulent. A synovial fluid WBC count of greater than 50,000 cells/mm3, with a predominance of polymorphonuclear leukocytes is strongly suggestive of septic arthritis even if the joint fluid culture finding is negative. The yield of organisms from joint fluid culture is about 50% to 60%. Enhanced culture techniques or polymerase chain reaction of joint fluid may be necessary to detect fastidious organisms such as K kingae.\n\nThe results of plain radiography may be normal, or they may show periarticular soft-tissue swelling and widening of the joint space owing to a large joint effusion. Ultrasonography of the hips is the modality of choice to detect joint effusion in a case of suspected septic arthritis of the hip joint. Magnetic resonance imaging is highly sensitive for detection of inflammatory joint fluid and is superior to computed tomography in delineation of adjacent bone and soft-tissue structures.\n\nThe WBC count may be elevated, with a predominance of polymorphonuclear leukocytes. Blood cultures are positive in 40% of patients with septic arthritis. Markers of systemic inflammation including erythrocyte sedimentation rate (ESR) or CRP have been used as an adjunct to culture for the diagnosis of bone and joint infections and for monitoring response to treatment. Levels of CRP or ESR are elevated in most patients with septic arthritis. The ESR usually rises 3 to 5 days after initiation of therapy and then slowly returns to normal within about 4 weeks. In contrast, CRP peaks at day 2 of therapy and can normalize within 1 week in uncomplicated cases.\n\nThe initial empiric antimicrobial therapy in cases of septic arthritis beyond the neonatal period should include an antistaphylococcal agent, either a β-lactamase–resistant penicillin (eg, nafcillin, oxacillin), a first-generation cephalosporin (eg, cefazolin), or clindamycin or vancomycin. In children aged 5 years or younger, in addition to S aureus, empiric coverage for K kingae with ampicillin-sulbactam or second-generation or third-generation cephalosporin must be considered. Empiric coverage for H influenzae type b with ceftriaxone is also warranted for unimmunized patients. In sexually active adolescents, empiric coverage should include agents active against N gonorrhoeae (such as ceftriaxone).\n\nThere is considerable experience in the sequential parenteral-oral regimen for antibiotic therapy for osteomyelitis and septic arthritis. Oral antibiotic therapy can be instituted only when a patient's clinical condition has improved (eg, resolution of fever, decrease in pain, increase in mobility), when CRP levels are decreased, and when medication compliance and close monitoring can be ensured. For oral therapy with β-lactam antibiotics, a dosage two to three times that used for mild infections should be used to achieve high synovial fluid:serum ratios. Besides being convenient for patients and families, oral therapy decreases the risk of complications of long-term intravenous therapy. Erythrocyte sedimentation rate and CRP are useful in monitoring response to therapy. The appropriate duration of therapy for septic arthritis is still controversial and depends on the infecting pathogen, the joint involved, and the host. However, it is advised to treat for at least 3 to 4 weeks in uncomplicated cases. Longer duration of therapy (eg, 4 weeks) may be necessary for septic arthritis of the hip or shoulder; it may also be necessary if the septic arthritis is caused by S aureus or gram-negative bacteria. Infection of a small to medium joint such as the knee that is caused by S pneumoniae, H influenzae, or Neisseria spp requires treatment of about 2 or 3 weeks.\n\nIn septic arthritis of the hip (or shoulder) joint, open surgical drainage should be performed immediately; waiting until the next day for orthopedic consultation is not appropriate. In the treatment of septic arthritis of joints other than the hip, open surgical drainage is not necessary and therapy can be individualized. Sequelae of septic arthritis in children are not uncommon and include cartilage damage, stiffness of joint with poor mobility, abnormal bone growth if the epiphysis is involved, unstable joint, and joint dislocation.\n\nPREP Pearls\n• Staphylococcus aureus is the most common cause of joint infections in all age groups; microbial invasion of the synovial space typically results from hematogenous seeding.\n• Early diagnosis via needle aspiration of the affected joint and prompt initiation of appropriate antimicrobial therapy, in conjunction with drainage of the affected joint, are critical to avoid destruction of the articular cartilage and prevent disability.\n• Empiric antibiotic regimens should always include adequate antistaphylococcal coverage.\n• Ultrasonography is the initial step in management of suspected septic arthritis of the hip.\n\nABP Content Specifications(s)\n• Differentiate the clinical findings of pyogenic arthritis from those of toxic synovitis and arthralgia\n• Plan the appropriate diagnostic evaluation of synovitis\n• Understand the natural history of pyogenic arthritis\n\nSuggested Readings\n• Brown DW, Sheffer BW. Pediatric septic arthritis: an update. Orthop Clin North Am. 2019;50(4):461-470. doi:10.1016/j.ocl.2019.05.003.\n• Herman MJ, Martinek M. The limping child. Pediatr Rev. 2015;36(5):184-195. doi:10.1542/pir.36-5-184.\n• Wolf M. Knee pain in children, part II: limp and life-threatening conditions, hip pathology and effusion. Pediatr Rev. 2016;37(2):72-76. doi:10.1542/pir.2015-0041.\n• Yee-Guardino S, Goldfarb J. Septic arthritis. In: McInerny TK, Adam HM, Campbell DE, DeWitt TG, Foy JM, Kamat DM, eds. American Academy of Pediatrics Textbook of Pediatric Care. 2nd ed. Elk Grove Village, IL: American Academy of Pediatric; 2017:2617-2619. Pediatric Care Online."}
{"id" : 160, "question_text" : "A 4-year-old boy presents with a 3-day history of vomiting followed by diarrhea. The vomiting resolved after 24 hours, and for the past 2 days, he has been keeping down clear liquids. On physical examination, the afebrile boy has a heart rate of 90 beats/min, respiratory rate of 18 breaths/min, and blood pressure of 106/58 mm Hg. He has tacky mucous membranes and a capillary refill time of 3 seconds. Other findings are within normal parameters. Laboratory evaluation reveals: Sodium, 130 mEq/L (130 mmol/L); Potassium, 3.7 mEq/L (3.7 mmol/L); Chloride, 102 mEq/L (102 mmol/L); Bicarbonate, 16 mEq/L (16 mmol/L); Glucose, 100 mg/dL (5.6 mmol/L); Blood urea nitrogen, 34 mg/dL (12.1 mmol/L); Creatinine, 0.6 mg/dL (53 mcmol/L). Urinalysis reveals a specific gravity of 1.030, pH of 5.5, 1+ ketones, and otherwise negative findings. Of the following, the MOST likely urine sodium concentration and urine osmolality for this patient are", "options" : "[\"Urine Sodium: 6 mEq/L, Urine Osmolality: 1,100 mOsm/kg\", \"Urine Sodium: 28 mEq/L, Urine Osmolality: 300 mOsm/kg\", \"Urine Sodium: 60 mEq/L, Urine Osmolality: 450 mOsm/kg\", \"Urine Sodium: 90 mEq/L, Urine Osmolality: 900 mOsm/kg\", \"Urine Sodium: 130 mEq/L, Urine Osmolality: 1,100 mOsm/kg\"]", "explanation" : "The boy described in the vignette presents with signs and symptoms consistent with acute gastroenteritis. During his recovery period, he is rehydrating with oral fluids. His physical examination is noteworthy for features of reduced effective circulating blood volume (tachycardia, absence of moist mucous membranes, and delayed capillary refill time), and the laboratory evaluation documents hyponatremia, a normal anion gap metabolic acidosis, and azotemia (elevation of blood urea nitrogen and creatinine). The urinalysis is essentially normal, except for a high urine specific gravity and ketonuria.\n\nThe challenge to clinicians evaluating children who have hyponatremia is determining the underlying cause because the appropriate treatment is related to the cause. Most pediatric disorders of serum sodium concentration (hyponatremia or hypernatremia) are caused by derangements of water balance: excess water to sodium in cases of hyponatremia and insufficient water to sodium for hypernatremia. Because water balance is critical to maintaining a normal serum sodium concentration, antidiuretic hormone (ADH) and the renal response to this hormone are of paramount importance. For water excretion to occur via the kidneys, the patient must have normal renal perfusion, normal renal function, and normal ability to inhibit the response to ADH.\n\nAnother step in the evaluation is to classify patients as hypovolemic, euvolemic, or hypervolemic, based on the history and physical examination findings. Hypovolemic disorders are characterized by losses of sodium and water. In some cases, sodium and fluid losses through the kidneys or skin result in hypovolemia. The hyponatremia can be aggravated by fluid replacement of hypotonic fluids, which are avidly retained by the body in the setting of hypovolemia due to appropriate ADH secretion, sometimes known as syndrome of appropriate ADH secretion (SAADH). Euvolemic or borderline hypervolemic states associated with hyponatremia include hypothyroidism, glucocorticoid deficiency, water intoxication, and syndrome of inappropriate ADH secretion (SIADH). Hypervolemic states are characterized by volume overload (eg, edema) and hyponatremia.\n\nOne test that is particularly useful in the evaluation of hyponatremia is measurement of the urine sodium concentration. Patients who have hyponatremia and hypovolemia of extrarenal origin (eg, acute gastroenteritis) can be expected to have an activated renin-angiotensin-aldosterone axis and increased ADH secretion. These compensatory mechanisms cause avid sodium and water retention in an effort to restore the intravascular volume to normal. As a result, the urine sodium is low (<20 mEq/L) and the urine osmolality is elevated (>600 mOsm/kg). In contrast, conditions of hyponatremia and hypovolemia associated with renal dysplasia or a tubulopathy feature elevated urine sodium (>40 mEq/L) with an inability to concentrate the urine (<300 mOsm/kg). Hypervolemic states associated with decreased effective circulating blood volume (eg, congestive heart failure, cirrhosis, or nephrotic syndrome) generally behave like hypovolemia from a renal perspective and are characterized by a low urine sodium and high urine osmolality. Patients who have SIADH often have elevated urine sodium concentrations (>40 mEq/L) and a high urine osmolality (often >600 mOsm/kg).\n\nBecause the boy in the vignette has hyponatremia and hypovolemia associated with decreased effective circulating blood volume and gastroenteritis, his urine sodium should be low (6 mEq/L) and urine osmolality should be high (1,100 mOsm/kg). His clinical condition and high urine osmolality should not be mistaken for SIADH, which would lead to the erroneous treatment approach of fluid/free water restriction. Rather, his ADH secretion is appropriate (SAADH), and once this diagnosis is established, he should receive fluid resuscitation with isotonic fluids to minimize the addition of free water, which could result in worsening hyponatremia.\n\nCritique: The boy described in the vignette presents with signs and symptoms consistent with acute gastroenteritis. During his recovery period, he is rehydrating with oral fluids. His physical examination is noteworthy for features of reduced effective circulating blood volume (tachycardia, absence of moist mucous membranes, and delayed capillary refill time), and the laboratory evaluation documents hyponatremia, a normal anion gap metabolic acidosis, and azotemia (elevation of blood urea nitrogen and creatinine). The urinalysis is essentially normal, except for a high urine specific gravity and ketonuria.\n\nThe challenge to clinicians evaluating children who have hyponatremia is determining the underlying cause because the appropriate treatment is related to the cause. Most pediatric disorders of serum sodium concentration (hyponatremia or hypernatremia) are caused by derangements of water balance: excess water to sodium in cases of hyponatremia and insufficient water to sodium for hypernatremia. Because water balance is critical to maintaining a normal serum sodium concentration, antidiuretic hormone (ADH) and the renal response to this hormone are of paramount importance. For water excretion to occur via the kidneys, the patient must have normal renal perfusion, normal renal function, and normal ability to inhibit the response to ADH.\n\nAnother step in the evaluation is to classify patients as hypovolemic, euvolemic, or hypervolemic, based on the history and physical examination findings. Hypovolemic disorders are characterized by losses of sodium and water. In some cases, sodium and fluid losses through the kidneys or skin result in hypovolemia. The hyponatremia can be aggravated by fluid replacement of hypotonic fluids, which are avidly retained by the body in the setting of hypovolemia due to appropriate ADH secretion, sometimes known as syndrome of appropriate ADH secretion (SAADH). Euvolemic or borderline hypervolemic states associated with hyponatremia include hypothyroidism, glucocorticoid deficiency, water intoxication, and syndrome of inappropriate ADH secretion (SIADH). Hypervolemic states are characterized by volume overload (eg, edema) and hyponatremia.\n\nOne test that is particularly useful in the evaluation of hyponatremia is measurement of the urine sodium concentration. Patients who have hyponatremia and hypovolemia of extrarenal origin (eg, acute gastroenteritis) can be expected to have an activated renin-angiotensin-aldosterone axis and increased ADH secretion. These compensatory mechanisms cause avid sodium and water retention in an effort to restore the intravascular volume to normal. As a result, the urine sodium is low (<20 mEq/L) and the urine osmolality is elevated (>600 mOsm/kg). In contrast, conditions of hyponatremia and hypovolemia associated with renal dysplasia or a tubulopathy feature elevated urine sodium (>40 mEq/L) with an inability to concentrate the urine (<300 mOsm/kg). Hypervolemic states associated with decreased effective circulating blood volume (eg, congestive heart failure, cirrhosis, or nephrotic syndrome) generally behave like hypovolemia from a renal perspective and are characterized by a low urine sodium and high urine osmolality. Patients who have SIADH often have elevated urine sodium concentrations (>40 mEq/L) and a high urine osmolality (often >600 mOsm/kg).\n\nBecause the boy in the vignette has hyponatremia and hypovolemia associated with decreased effective circulating blood volume and gastroenteritis, his urine sodium should be low (6 mEq/L) and urine osmolality should be high (1,100 mOsm/kg). His clinical condition and high urine osmolality should not be mistaken for SIADH, which would lead to the erroneous treatment approach of fluid/free water restriction. Rather, his ADH secretion is appropriate (SAADH), and once this diagnosis is established, he should receive fluid resuscitation with isotonic fluids to minimize the addition of free water, which could result in worsening hyponatremia.\n\nContent Specifications: Recognize the importance of urinary sodium concentration and urinary osmolality in the differential diagnosis of hyponatremia"}
{"id" : 3195, "question_text" : "A 15-year-old adolescent girl comes for evaluation with complaints of intermittent shoulder pain that has waxed and waned for the past 3 to 4 months. She has been on multiple courses of nonsteroidal anti-inflammatory medications, such as ibuprofen, has had improvement with each course for 1 to 2 weeks, and then the shoulder pain recurs. Over the last few days, she has had worsening shoulder pain and shortness of breath. She was unable to sleep last night. She is able to speak, but is most comfortable sitting upright on the stretcher. Her heart rate is 130 beats/min, respiratory rate is 32 breaths/ min, and blood pressure is 90/60 mm Hg. She is anxious and has increased work of breathing. On physical examination, her neck veins are visible. Chest is clear with equal breath sounds. Her cardiac examination is difficult because of her body habitus, but her heart sounds are quite soft. There is no murmur, rub, or gallop. Her abdominal examination is not reliable because she cannot lie flat. You obtain a chest radiograph. She is admitted to the hospital and an echocardiogram shows a large pericardial effusion. Of the following, the intervention MOST likely to worsen this patient's clinical status is", "options" : "[\"ceftriaxone\", \"dopamine\", \"furosemide\", \"normal saline bolus\", \"supplemental oxygen\"]", "explanation" : "Preferred Response: C\nThe patient described in the vignette has symptoms that began several months ago, but have acutely worsened in the last few days. With the additional information that her chest radiograph and her echocardiogram show a large pericardial effusion, it is important to decide if she has tamponade physiology. She has several signs that she is now experiencing tamponade: her neck veins are distended, her blood pressure is low for her level of anxiety and distress, and her heart sounds are soft. These findings suggest, in order, that she is unable to fill the right side of her heart easily, that her cardiac output is decreased, and that her heart is further away from her chest wall than would be expected. In this situation, administration of furosemide would be dangerous. It would lower her circulating blood volume, make compression of her right ventricle more severe, and acutely decrease her systolic output. Of the choices given, this is most likely to worsen the patient's status.\n\nAdministration of ceftriaxone would be helpful if this was a bacterial pericarditis and the bacteria were sensitive to that antibiotic. If this were bacterial pericarditis, a much more fulminant course would be expected. Antibiotics would not likely cause an adverse effect. Dopamine may be needed if the patient became hypotensive and would not make the effusion worse. A normal saline bolus would be the best intervention while more definitive treatment is being arranged, including draining the effusion. Supplemental oxygen would not hurt the patient in this setting of decreased cardiac output; it would be helpful to keep tissue and myocardial oxygenation as high as possible.\n\nThe causes of pericardial effusions are many. The major categories are metabolic (uremia, hypothyroid states), infectious (viral, bacterial, fungal) with or without myocarditis, and autoimmune (lupus, juvenile rheumatoid arthritis, acute rheumatic fever). Presentation can be acute or chronic or a combination, as in this case. The most likely cause in this patient is an autoimmune process of several months' duration, which responded to anti-inflammatory medications. Her shoulder pain is referred from diaphragmatic irritation. She now exhibits tamponade physiology because her pericardium cannot stretch any further. As her effusion accumulates, her right atrium and right ventricle are collapsing. Her heart sounds are soft because of fluid encasing the heart. The flow to her lungs, and therefore to her left ventricle and her systemic circulation, is not adequate, thus making her blood pressure low.\n\nPREP Pearls\n• Pericardial effusions can present with positional chest pain, abdominal pain, or shoulder pain.\n• A slowly accumulating pericardial effusion can suddenly become dangerous when tamponade physiology develops.\n\nAmerican Board of Pediatrics Content Specification(s)\n• Understand the natural history of pericarditis\n• Recognize the clinical findings associated with pericarditis and plan appropriate initial management\n• Recognize pathogens commonly associated with pericarditis\n\nSuggested Reading\n• LeWinter MM, Tischler MD. Pericardial diseases. In: Bonow RO, Mann DL, Zipes DP, Libby P, eds. Braunwald's Heart Disease: A Textbook of Cardiovascular Medicine. 9th ed. Philadelphia, PA: Saunders Elsevier; 2012:1651-1671.\n• Maisch B, Ristic AC. Practical aspects of management of pericardial disease. Heart. 2003;89(9):1096-1103. doi:10.1136/heart.89.9.1096.\n• Spicer R, Ware S. Diseases of the pericardium. In: Kliegman RM, Stanton BF, St Geme J W Ill, Schor NF, eds. Nelson Textbook of Pediatrics. 19th ed. Philadelphia, PA: Saunders Elsevier; 2011:1635-1637."}
{"id" : 1494, "question_text" : "A 10-year-old girl is brought to your office by her parents for evaluation of clumsiness. She had met all the early developmental milestones on time, but over the past year, her parents have noticed that she falls over small obstacles like steps or curbs. They have seen occasional quick jerks of her eyes, especially when she turns her head. Over the past month, she has been coughing when she drinks fluids. The mother reports that she had a cousin who died at 20 years of age of a progressive neurological disorder. Her neurological examination shows an alert girl with upper extremity dysmetria, diffuse areflexia, lower extremity weakness, and an ataxic gait. A brain magnetic resonance image is performed and is normal. Of the following, the MOST likely test to show the correct diagnosis is", "options" : "[\"15q11 methylation study (Angelman syndrome)\", \"frataxin gene sequencing (Friedreich ataxia)\", \"leukocyte lysosomal enzyme panel (leukodystrophy)\", \"neurofibromin gene sequencing (neurofibromatosis type 1)\", \"serum \\u03b1-fetoprotein level (ataxia telangiectasia)\"]", "explanation" : "Correct Answer: B\nThe girl in the vignette has Friedreich ataxia. Friedreich ataxia is an autosomal recessive disorder caused by GAA repeat expansion in the frataxin gene on chromosome 9. Of the choices listed, the test most likely to make the diagnosis in this girl is frataxin gene sequencing. In Friedreich ataxia, birth and early developmental milestones are almost always normal. The first signs are gait and limb ataxia, which typically appear in childhood as in the girl in the vignette, but can be earlier or later. Other clinical findings are areflexia, lower extremity weakness, dysarthria, and dysphagia. Eye movement abnormalities such as abnormal saccades (rapid jerky movements of both eyes) may be seen by observant parents. Once the diagnosis is established by genetic testing, the girl will need monitoring for complications of Friedreich ataxia, including cardiomyopathy, diabetes mellitus, and bladder dysfunction, as well as supportive treatment for progressive ataxia, weakness, and dysphagia. Treatment trials are ongoing, but there is currently no known treatment or cure. If symptoms start at an earlier age, life expectancy is shorter.\nThe evaluation of ataxia should start with chronicity. Chronic, progressive ataxias are more likely to be due to genetic syndromes. In these cases, it is important to obtain a family history and include assessment of eye movements, strength, and reflexes during the neurologic examination. Evaluation typically includes brain imaging, although in genetic ataxias, this is likely to be normal. If a particular genetic syndrome is not identified based on the clinical presentation, a gene panel for hereditary ataxias can be helpful.\n\nAcute ataxias are more likely due to an acute process such as infection, stroke, intracranial mass, or toxicity. In acute ataxias, the history should focus on infections, injuries, and exposures to toxins, and the examination should evaluate mental status and signs of increased intracranial pressure. If there is abnormal mental status or signs of increased intracranial pressure, computed tomography of the head is the quickest test to make a diagnosis. If this does not show signs of edema or mass lesion, lumbar puncture is often needed to obtain cerebral spinal fluid for studies to evaluate for viral and bacterial infection. If there is a clear history of a toxic ingestion, lumbar puncture may not be necessary.\n\nAngelman syndrome is a genetic disorder usually caused by abnormal imprinting on chromosome 15. Symptoms start in infancy or early childhood ages and include gait ataxia, lack of expressive language, microcephaly, and seizures. When Angelman syndrome is suspected, 15q11 methylation study is the best initial test. The girl in the vignette has a later onset of ataxia, so this is not the most likely diagnosis.\n\nLeukocyte lysosomal enzyme panels test for a variety of disorders in which there is an abnormal accumulation of a substance in the lysosomes. Examples include Tay-Sachs disease, metachromatic leukodystrophy, and mucopolysaccharidosis. The clinical presentation of these diseases varies significantly. The girl in the vignette has a very typical presentation of the most common cause of hereditary ataxia, which clinicians should recognize. In her case, the best test is for the most likely disorder, instead of testing for a panel of disorders.\n\nNeurofibromatosis type 1 is an autosomal dominant disorder caused by mutations in the neurofibromin 1 gene on chromosome 17; about half the cases are due to de novo mutations (not inherited). Symptoms include cutaneous neurofibromas, café-au-lait spots, axillary and inguinal freckling, Lisch nodules, optic nerve gliomas, bone dysplasia, and learning disorders. The girl in the vignette does not have these findings, so this is not the best test.\n\nAtaxia telangiectasia is an autosomal recessive disorder due to mutations in the ATM gene on chromosome 11. In most cases, serum α-fetoprotein is elevated. In ataxia telangiectasia, ataxia typically presents in early childhood. Additional signs and symptoms include recurrent respiratory infections, oculomotor apraxia, choreoathetosis, dysarthria, and ocular telangiectasias. The girl in the vignette is older than the typical age at which ataxia telangiectasia presents, and although there is overlap in the signs and symptoms of these 2 disorders, her clinical presentation points more towards Friedreich ataxia than ataxia telangiectasia.\n\nPREP Pearls\n• Friedreich ataxia is the most common hereditary ataxia.\n• Chronic, progressive ataxias are more likely due to genetic syndromes, whereas acute ataxias are more likely due to infection, stroke, or toxicity.\n\nABP Content Specifications(s)\n• Plan the appropriate evaluation of ataxia\n\nSuggested Readings\n• Bidichandani S. Friedreich ataxia. GeneReviews. http://www.ncbi.nlm.nih.gov/books/NBK1281/.\n• Kruer MC. Pediatric movement disorders. Pediatr Rev. 2015;36(3):104-116. doi: http://dx.doi.org/10.1542/pir.36-3-104."}
{"id" : 1598, "question_text" : "A 15-month-old previously healthy boy is brought to the emergency department with fever, rash, and increasing lethargy. He was in his usual state of health until he developed a runny nose and cough the day before presentation. On the day of presentation, he has felt warm to the touch, had decreased oral intake, and became progressively lethargic and listless. When he woke up in the morning, his mother noticed a few red spots on his skin. Throughout the day, the rash progressed, significantly covering his trunk, arms, and legs. He has no known allergies. The boy's temperature is 39.5°C, heart rate is 180 beats/min, respiratory rate is 50 breaths/min, blood pressure is 70/40 mm Hg, and oxygen saturation is 100% on room air. His physical examination reveals a well-nourished but toxic-appearing, lethargic child. He has a nonblanching, purpuric rash evenly distributed over his face, trunk, and upper and lower extremities. His mucous membranes are dry. He is in moderate respiratory distress, with clear lung fields and good bilateral air exchange. His heartbeat is regular, with no murmur. The boy's extremities are cold, with a capillary refill time of 5 seconds. His abdomen is soft with no organomegaly. The nurse places 2 large-bore intravenous catheters, and fluid resuscitation is rapidly initiated. Of the following, the MOST appropriate antimicrobial therapy in this case is intravenous", "options" : "[\"acyclovir\", \"amphotericin B\", \"ceftriaxone\", \"chloramphenicol\", \"vancomycin\"]", "explanation" : "Correct Answer: C\nThe boy in this vignette has septic shock from disseminated meningococcemia, evidenced by tachycardia, tachypnea, hypotension, lethargy, and toxic appearance combined with a characteristic purpuric rash. The best antimicrobial choice is ceftriaxone.\n\nBecause of its capacity to cause rapidly progressive septic shock and meningitis in healthy children, Neisseria meningitidis is one of the most feared bacterial pathogens. It is a gram-negative encapsulated diplococcus that colonizes the nasopharynx. Rates of carriage range from less than 2% in children younger than 2 years of age to as high as 40% in adolescents and young adults. Transmission occurs via respiratory droplets. Individuals in crowded living conditions, such as military barracks and college dormitories, are at higher risk of infection. Younger children are more likely to become ill with meningococcal disease because of less developed innate immune defense mechanisms. Children with acquired or congenital immune defects, such as complement deficiency or functional asplenia, are predisposed to invasive meningococcal disease.\n\nChildren with meningococcal disease can deteriorate quickly. Meningococcemia can initially masquerade as a viral syndrome, with presenting signs and symptoms including high fever, rash, chills, and body aches. Within hours, the rash, which can initially be confused with a viral exanthem, will become purpuric (purplish, blotchy, and nonblanching). Endotoxin from the bacterial capsule causes a severe host inflammatory response that can lead to cardiovascular collapse because of myocardial depression and vasodilation, disseminated intravascular coagulation, lethargy, respiratory failure, and death. Pediatricians should be on the alert for signs or symptoms that indicate severe illness. Tachycardia out of proportion to the degree of fever should raise suspicion for shock. A rule of thumb is that the heart rate may increase by 10 beats/min for every degree Celsius above 37 without causing additional concern. A rash that does not blanch (ie, petechiae or purpura) is less likely to be a viral exanthem, rather, it raises concern for thrombocytopenia, sepsis, or disseminated intravascular coagulation. Lastly, signs of meningitis such as vomiting without diarrhea, lethargy, or a stiff neck help differentiate meningococcemia from a viral illness.\n\nTreatment of a child with meningococcemia should focus on the circulation, airway, and breathing, according to guidelines based on pediatric septic shock and pediatric advanced life support (PALS) guidelines. Artificial ventilation should be provided to any child with oxygenation, ventilation, or airway protective reflexes, and should be considered in children who are obtunded or in shock. Intravenous or intraosseous access should be established within the first 2 minutes and aggressive fluid resuscitation should be initiated. Inotropes, vasopressors, and steroids should be considered if the shock is refractory to fluids.\n\nThe importance of timely administration of antibiotics cannot be overstated. Although a blood culture and lumbar puncture are important to make the diagnosis, antibiotics should not be delayed. While N meningitidis is sensitive to penicillin, ceftriaxone is the appropriate initial therapy when the diagnosis may be uncertain, because it also covers resistant streptococcal disease. Acyclovir and vancomycin could be considered for the broad treatment of meningitis, but neither is effective for meningococcal disease. Chloramphenicol is an excellent bactericidal agent for meningococcemia and penetrates the blood-brain barrier, but it has an unfavorable side effect profile. Amphotericin B is an effective antifungal agent for use in immunocompromised patients, but would not be appropriate treatment for the boy in this vignette.\n\nPREP Pearls\n• Meningococcemia should be considered in cases of sudden-onset fever and rash.\n• Tachycardia out of proportion to the degree of fever should raise suspicion for shock. A rule of thumb is to expect an increase of up to 10 beats/min for every degree Celsius above 37 without additional concern.\n• Younger children are more likely to become ill from meningococcal disease; however, rates of carriage are higher among adolescents and young adults, and those in crowded living conditions.\n• Children with acquired or congenital immune defects, such as complement deficiency or functional asplenia, are predisposed to invasive meningococcal disease.\n\nABP Content Specifications(s)\n• Understand the epidemiology of Neisseria meningitidis\n• Plan appropriate management for a patient with meningococcal disease\n• Understand the significance of purpura in a febrile child\n• Understand which patients are at increased risk of invasive and recurrent meningococcal disease (eg, asplenia, terminal complement component)\n• Recognize the major clinical features associated with Neisseria meningitidis infection\n\nSuggested Readings\n• Brayer AF, Humiston SG. Invasive meningococcal disease in childhood. Pediatr Rev. 2011;32:152–161. doi: http://dx.doi.org/10.1542/pir.32-4-152.\n• Brierley J, Carcillo JA, Choong K, et al. Clinical practice parameters for hemodynamic support of pediatric and neonatal septic shock: 2007 update from the American College of Critical Care Medicine. Crit Care Med. 2009;37(2):666–688. doi: http://dx.doi.org/10.1097/CCM.0b013e31819323c6.\n• MacNeil JR, Bennett N, Farley MM, et al. Epidemiology of infant meningococcal disease in the United States, 2006-2012. Pediatrics 2015;135:e305–e311. doi: http://dx.doi.org/10.1542/peds.2014-2035."}
{"id" : 1747, "question_text" : "A 10-year-old girl is brought to your office for a new patient health supervision visit. Her family moved into the area a few months ago. When you ask the girl how she likes school, she tells you that it is \"okay,\" except for a group of girls in her class who encourage others to exclude her from activities. They also frequently make fun of her accent and lack of athletic ability. She attempts to avoid these girls, but they follow her around on the playground. The girl's mother asks you if there is anything that can be done at school to address the situation. Of the following, the BEST intervention you can recommend for implementation at the girl's school is to", "options" : "[\"allow the girl to stay in her classroom at recess\", \"assign the involved girls to different classrooms\", \"increase adult supervision of the school grounds\", \"implement an anti-bullying intervention in the girl's class\", \"suspend the students who are bullying\"]", "explanation" : "Increasing adult supervision of school grounds (eg, playgrounds, restrooms, hallways) helps to make the environment safer for all students, and serves as a deterrent to bullies. As is typical, the problematic behaviors seen in this vignette are occurring outside the classroom.\n\nDuring normal development, children may exhibit limited periods of aggressive behaviors such as hitting, biting, pushing, and making threats. Typical aggressive behaviors may present in children as tantrums in toddlers or defiance in adolescents. When aggressive behaviors persist, escalate, impair functioning, and/or are accompanied by delinquent acts (eg, vandalism, theft, assault), a child may meet criteria for a mental health condition such as oppositional defiant disorder or conduct disorder. Children with conditions such as attention-deficit/hyperactivity disorder (ADHD) or anxiety disorder may demonstrate aggressive behaviors in response to stressors. Standardized rating scales (eg, Child Behavior Checklist, Behavior Assessment System for Children) can help determine if a child's behaviors are outside the normal range for age.\n\nBullying is defined as repeated aggression (eg, verbal harassment, physical harm or threats, social exclusion) toward an apparently less powerful peer. Cyberbullying involves the use of electronic devices (eg, cell phones, computers) to threaten or harass peers. Bullying is common in children, with a prevalence rate between 15% and 50%. Bullying most commonly occurs at school, particularly during less supervised activities (eg, breaks, recess, lunch) and in less supervised settings (eg, playground, hallways).\n\nParents can teach their children appropriate social behaviors by role modeling, talking to them about empathy and compassion, and by avoiding the use of physical discipline. They can help their children understand that aggressive behaviors are not acceptable, and coach them on preferred approaches. Children should be encouraged to form positive relationships with their peers, and to use nonviolent means to resolve disagreements. They should be praised when demonstrating appropriate social behaviors. For toddlers demonstrating aggressive behavior, a simple statement (eg, \"No hitting\") followed by distraction or redirection can be effective. Older children and adolescents having difficulty with conflict resolution may benefit from social skills and/or anger management training. When behavioral strategies are not sufficiently effective in managing an aggressive child with ADHD or anxiety, psychopharmacologic agents (eg, stimulants or alpha-agonists for ADHD; selective serotonin reuptake inhibitors for anxiety) can be helpful.\n\nVictims of bullying can be taught strategies to avoid situations in which they are vulnerable. These include avoidance of less supervised areas of the school, participation in structured activities during break times, and remaining near friends at school. They should be taught that reporting a student's inappropriate behavior to seek help is different from tattling to get that person in trouble. Because children who appear insecure are more likely to be bullied, these children can be taught how to look confident through role-modeling or participation in drama clubs. Activities that build self-esteem (eg, athletics, clubs) should be encouraged.\n\nEffective interventions to address bullying are typically school-based and work to improve the school environment. They often include antiviolence or conflict resolution curricula. Successful programs aim to change the school culture with participation by the entire school, rather than a single classroom. Effective programs incorporate improved student supervision, school-wide rules and antibullying policies, parent training, and collaboration between schools and families. Zero-tolerance policies are ineffective in decreasing bullying; removing the bully from school (eg, suspension, expulsion) can unintentionally reward and thereby reinforce bullying behavior.\n\nAllowing the girl in the vignette to stay in her classroom at recess does not address the problem, and isolating her decreases her opportunities for developing healthy relationships and friendships with her classmates. Assigning the girls to different classrooms does not address the behaviors that are occurring on the playground. The pediatrician can assist families dealing with aggressive behaviors in children by validating their concerns, providing strategies and resources for managing these behaviors, and advocating for violence prevention. Helpful antibullying resources include the American Academy of Pediatrics Connected Kids program (Connected Kids Clinical Guide) and www.stopbullying.gov.\n\nPREP Pearls\n• Bullying most commonly occurs at school and in less supervised settings (eg, playground, hallways).\n• Effective antibullying programs incorporate improved student supervision, school-wide rules and antibullying policies, parent training, and collaboration between schools and families.\n• Zero-tolerance policies are ineffective in decreasing bullying; removing the bully from school (eg, suspension, expulsion) can unintentionally reward and thereby reinforce bullying behavior.\n\nABP Content Specifications(s)\n• Plan the appropriate management of aggressive or intimidating (bullying) behavior in patients of various ages, including those who are victims of such behavior\n• Differentiate the findings associated with aggressive behavior from those of normal variants\n\nSuggested Readings\n• Glew GM, Frey KS, Walker WO. Bullying update: are we making any progress? Pediatr Rev. 2010;31(9):e68–e74. doi: http://dx.doi.org/10.1542/pir.31-9-e68.\n• Shetgiri R. Bullying and victimization among children. Adv Pediatr. 2013;60(1):33–51. doi: http://dx.doi.org/10.1016/j.yapd.2013.04.004.\n• Zahrt DM, Melzer-Lange MD. Aggressive behavior in children and adolescents. Pediatr Rev. 2011;32(8):325–331. doi: http://dx.doi.org/10.1542/pir.32-8-325."}
{"id" : 145, "question_text" : "A 13-year-old girl who has moderate persistent asthma complains of daily ocular and nasal allergy symptoms. She states that her symptoms are worse at home and bother her, despite a regimen of an oral antihistamine and a nasal corticosteroid. On further questioning, she states that she has five dogs and two cats, sleeps in a finished room in the basement, and enjoys collecting stuffed animals. She asks how she can reduce her symptoms at home after learning that her serum immunoglobulin (Ig) E tests were positive to cat and dust mite.\n\nOf the following, you are MOST likely to advise her that she should", "options" : "[\"decrease indoor humidity to less than 50%\", \"install a high-efficiency particulate air (HEPA) filter\", \"use an impermeable mattress cover\", \"vacuum carpets twice a week\", \"wash the cats once a month\"]", "explanation" : "Identification and avoidance of indoor allergens is an important step in the management of immunoglobulin (Ig)E-mediated diseases such as allergic rhinitis and allergic asthma. Dust mite is one of the most common indoor allergens, except in locations where humidity is consistently less than 50%. When dust mite allergen is significantly reduced, patients have demonstrated improvement in allergic rhinitis symptoms and bronchial hyperresponsiveness.\n\nOther allergens that have been identified as potential causes of IgE-mediated symptoms include animal dander (eg, cat, dog, rodent), cockroach, and mold. Some of the recommended avoidance measures for the bedroom include covering the mattress, box spring, and pillows with impermeable covers; washing bedding regularly at 130.0°F; removing stuffed animals and carpeting; and vacuuming regularly. A recent Cochrane review, however, demonstrated that no single intervention alone, such as impermeable covers, is likely to have a significant overall effect. Vacuums or high-efficiency particulate air (HEPA) filters can collect dust mite allergen, but most allergen exposure located in the bedding is not affected by these interventions.\n\nStudies over the past 20 years have demonstrated interesting findings about cat allergen. Fel d 1, the major allergen, initially was believed to be produced in saliva, but studies in which cats were prevented from licking themselves demonstrated that this allergen is made in the skin. Because washing the animals reduces allergen found in the skin and fur, studies have been performed to evaluate regular washing as a possible intervention because most pet owners are unwilling to remove their pets from the home. Two studies demonstrated that immersion up to the neck for 3 minutes followed by towel drying does significantly reduce allergen, but the reduction is short-lived; concentrations of the allergen return to baseline values by the next day. Patients who remove the cat(s) from the home find that Fel d 1 concentrations do decrease, although significant reduction may take 2 to 3 months.\n\nAvoidance continues to be the recommendation for patients who are allergic to cats, but they often describe a rapid onset of ocular and nasal symptoms when entering a home that contains a cat. In addition, concentrations of cat dander that can result in IgE-mediated sensitization can be found in buildings in which a cat never has been, illustrating the ease of transporting cat dander inadvertently by people who own cats. More recently, studies have shown that children exposed to pet dander since birth (ie, the pet is already in the home) have a lower incidence of rhinitis and asthma to these allergens compared with children exposed later in life.\n\nAmerican Board of Pediatrics Content Specification(s):\n\nKnow that indoor pets may contribute to allergic disease"}
{"id" : 2504, "question_text" : "An 18-month-old girl with a history of sickle cell disease is evaluated for 2 days of fever, cough, and vomiting. A rapid influenza diagnostic test is performed due to her symptoms and the high incidence of influenza A in the community. The test has 55% sensitivity and 95% specificity for influenza.\n\nOf the following, the MOST likely error type to occur when using this test is", "options" : "[\"type I\", \"type II\", \"type III\", \"type IV\"]", "explanation" : "The most likely error type to occur when using the rapid influenza diagnostic test is a type II error. A type II error occurs when the diagnostic test result is negative but the patient has the disease being tested for (ie, false negative). When the sensitivity of a test is low, as in this case, any negative test result has a high probability of being a false negative or type II error. In this situation, the practitioner must consider both the child's risk of complications from influenza (eg, increased risk for this child with sickle cell disease) and the likelihood of a false-negative test result and decide whether to believe the test result, administer antiviral therapy based on clinical suspicion, or perform further influenza testing of respiratory specimens by molecular assays.\n\nA type I error occurs when a diagnostic test result is positive but the patient does not have the disease being tested for (ie, false positive). Tests with a low specificity have a higher likelihood of a false-positive result (Table).\n\nA type III error occurs in statistics when you correctly reject the null hypothesis, but it is rejected for the wrong reason. One arrives at the correct conclusion but for the wrong reason.\n\nA type IV error occurs when you correctly reject the null hypothesis but make a mistake interpreting the results.\n\nSuggested Reading(s)\nCenters for Disease Control and Prevention. Rapid influenza diagnostic tests. Accessed October 22,\n2022. https://www.cdc.gov/flu/professionals/diagnosis/clinician_guidance_ridt.htm\nJennings JM, Sibinga E. Demystifying type I and type II errors. Pediatr Rev. 2010;37(11):209-210.\ndoi:10.1542/pir.31-5-209\n\nContent Domain\nStatistics\n\nABP Content Specification(s) / Content Area(s)\nDistinguish between type I and type II statistical errors\nUnderstand standard deviation in the interpretation of results\nUnderstand standard error in the interpretation of results\nUnderstand confidence interval in the interpretation of results\n\nThe correct answer is: type II"}
{"id" : 1583, "question_text" : "You are seeing a 6-year-old girl in the emergency department. Her parents are concerned that she has developed dehydration due to food poisoning. The girl attended a neighborhood picnic, along with the rest of her family, where she consumed various foods including chicken, potato salad, watermelon, and strawberry pie. Within half an hour of eating, the girl told her mother that \"her belly hurt really badly,\" and then proceeded to have multiple episodes of vomiting and diarrhea. These symptoms have continued for 2 hours; she feels very dizzy and cannot stand up. Her mother remarks that the girl's face has appeared flushed since her symptoms began. The girl's 10-year-old brother had an isolated episode of watery diarrhea approximately 1 hour after eating the same food, so the parents are worried that both children were exposed to \"bad food\" at the picnic.\n\nOther than a history of mild eczema, the girl has been healthy. She takes no medications, and has no known allergies. Her immunizations are up to date.\n\nHer vital signs are as follows: temperature of 36.9°C, heart rate of 145 beats/min, respiratory rate of 30 breaths/min, and blood pressure of 70/45 mm Hg. The girl is awake and responds to your questions and instructions, but seems fatigued and uncomfortable. She retches several times as you are talking with her parents. On physical examination, her entire face and neck appear flushed. She has profuse clear rhinorrhea and watery discharge from both of her eyes. The girl's airway is intact, and you note no angioedema. She has clear lungs bilaterally, and no other signs of respiratory distress. Her abdomen is soft and diffusely tender with hyperactive bowel sounds, but no peritoneal signs. There is no swelling of her extremities. Her hands and feet are cool, with a capillary refill time of 3 seconds. Neurologic examination reveals no focal deficits; however, you cannot assess the girl's gait because she complains of dizziness with any attempt to stand up and prefers to remain lying in bed. You order the administration of a 20 mL/kg intravenous bolus of normal saline.\n\nOf the following, the BEST next step in this girl's management is administration of", "options" : "[\"diphenhydramine, intravenously\", \"dopamine, intravenously\", \"epinephrine, intramuscularly\", \"methylprednisolone, intravenously\", \"ondansetron, intravenously\"]", "explanation" : "Correct Answer: C\nThe girl in the vignette displays signs and symptoms that meet the diagnostic criteria for acute anaphylaxis. Administration of intramuscular epinephrine is the best next step in her management.\n\nAcute anaphylaxis is a life-threatening medical emergency that typically affects 2 or more organ systems. The gastrointestinal symptoms (acute nausea, vomiting, diarrhea, and crampy abdominal pain) may be mistakenly attributed to more common etiologies, such as foodborne illnesses. It is imperative for pediatric providers to distinguish between anaphylaxis and food poisoning, because the 2 disorders are managed very differently.\n\nSeveral important factors can be helpful in distinguishing anaphylaxis from food poisoning. Anaphylaxis is an immunoglobulin E (IgE)–mediated process that generally occurs within minutes (almost always within the first hour) after ingesting a specific food (or after contact with an offending substance). Foodborne illnesses are not IgE-mediated, and tend to manifest within a few hours to as long as 24 hours after ingestion of contaminated food. Most cases of food-related anaphylaxis present with skin manifestations, including hives, flushing, and/or angioedema, whereas these findings are not typical in food poisoning.\n\nThe clinical diagnosis of anaphylaxis depends on recognition of its signs and symptoms, which may include urticaria, skin flushing, pruritus, angioedema, rhinorrhea, wheezing, shortness of breath, abdominal pain, vomiting, diarrhea, light-headedness, and even frank syncope. This clinical syndrome is highly likely in patients meeting any 1 of 3 diagnostic criteria established by the World Allergy Organization (Item C55).\n\nSymptoms of food poisoning typically include abdominal pain, nausea, vomiting, and diarrhea, which develop within 1 to 6 hours of eating contaminated food (although symptom onset can be delayed for up to 24 hours). In the United States, staphylococcal food poisoning is one of the most common causes. Food poisoning is typically a self-limited illness, though severe cases may require treatment with antiemetics and intravenous fluids to correct dehydration.\n\nEarly intramuscular administration of epinephrine has been clearly shown to decrease both hospitalizations and death among patients with anaphylaxis. Although administration of intravenous ondansetron would be appropriate for patients with both anaphylaxis and foodborne illnesses to help alleviate vomiting, intramuscular epinephrine is the first-line treatment recommended for anaphylaxis and should be administered as quickly as possible to all children presenting with the characteristic signs and symptoms.\n\nIntravenous diphenhydramine is commonly used as an adjunctive therapy in the management of anaphylaxis, but there is a lack of evidence supporting its efficacy. Although diphenhydramine may be beneficial for specific anaphylaxis-related symptoms (such as itching), it is not a replacement for epinephrine therapy. Epinephrine should never be deferred or delayed due to administration of antihistamines, such as diphenhydramine, in patients with anaphylaxis.\n\nThe girl in the vignette is hypotensive with decreased peripheral perfusion, due to acute anaphylaxis, therefore administration of intramuscular epinephrine rather than intravenous dopamine is the best next management step for her. At least 2 to 3 additional 20-mL/kg boluses of crystalloid fluid would be indicated to address this patient's hemodynamic instability before initiating vasopressor therapy. For patients with persistent hypotension secondary to acute anaphylaxis, intravenous epinephrine would be the pressor of choice.\n\nIntravenous methylprednisolone is sometimes used for treating patients with acute anaphylaxis, though the medical literature does not strongly support its benefit. As with antihistamines, corticosteroids may be used as adjunct treatment for anaphylaxis, but intramuscular epinephrine is always recommended as the initial treatment.\n\nPREP Pearls\n• Food-associated anaphylaxis is an immunoglobulin E (IgE)–mediated process that generally occurs within minutes (almost always within the first hour) after ingesting a specific food.\n• Foodborne poisoning is not IgE-mediated and manifests within a few hours, up to as long as 24 hours, after ingestion of contaminated food.\n• Most food-related anaphylaxis cases present with skin manifestations including hives, flushing, and/or angioedema, whereas these findings are not typical in food poisoning.\n• The clinical diagnosis of anaphylaxis depends on recognition of its signs and symptoms, which may include urticaria, skin flushing, pruritus, angioedema, rhinorrhea, wheezing, shortness of breath, abdominal pain, vomiting, diarrhea, light-headedness, and even frank syncope.\n• Intramuscular epinephrine is the first-line treatment for anaphylaxis, and should be given as quickly as possible to children presenting with the characteristic signs and symptoms.\n\nABP Content Specifications(s)\n• Distinguish between anaphylaxis and food poisoning\n\nSuggested Readings\n• Chipps BE. Update in pediatric anaphylaxis: a systematic review. Clin Pediatr. 2013;52(5):451–-461. doi: http://dx.doi.org/10.1177/0009922812474683.\n• Langley EW, Gigante J. Anaphylaxis, urticaria, and angioedema. Pediatr Rev. 2013;34:247. doi: http://dx.doi.org/10.1542/pir.34-6-247.\n• Simons FE, Ardusso LR, Bilò MB, et al; World Allergy Organization. World Allergy Organization guidelines for the assessment and management of anaphylaxis. World Allergy Organ J. 2011;4:13–37. doi: http://dx.doi.org/10.1097/WOX.0b013e318211496c."}
{"id" : 2960, "question_text" : "A 6-week-old infant girl is seen in the office for evaluation of a 4-day history of cough, posttussive emesis, and fussiness. The mother reports that the infant became apneic and limp for a brief period after a coughing episode at home. Her grandmother has had a chronic cough for more than 1 month. The infant has a temperature of 37.2°C, a heart rate of 148 beats/min, a respiratory rate of 37 breaths/min, a blood pressure of 82/44 mm Hg, and an oxygen saturation of 96% on room air. Findings of a respiratory examination are notable for bilateral coarse breath sounds. Several episodes of transient apnea are observed. Findings from the rest of the physical examination are unremarkable. Rapid viral antigen testing via direct fluorescent antibody is negative for respiratory syncytial virus.\n\nOf the following, the BEST next diagnostic step in the evaluation of this infant's illness is", "options" : "[\"chest radiography\", \"complete blood cell count\", \"nasopharyngeal swab for polymerase chain reaction\", \"serology\"]", "explanation" : "The infant described in this vignette has a clinical picture suggestive of pertussis (ie, whooping cough). Pertussis is a highly contagious acute respiratory tract infection caused by Bordetella pertussis, a fastidious gram-negative coccobacillus. The diagnosis of pertussis may be challenging in infants younger than 6 months. Pertussis in young infants can have an atypical presentation during the early stages of illness characterized by gagging, gasping, apnea, or bradycardia without a \"whoop.\"\n\nNucleic acid amplification tests using polymerase chain reaction assay from nasopharyngeal (NP) wash, aspirate, or Dacron swab is the diagnostic modality of choice for diagnosis of pertussis. Compared to culture, polymerase chain reaction assays are highly sensitive and specific, and results are rapidly available. Commercially available serologic assay using IgG antibody to pertussis toxin (PT) may be used for presumptive diagnosis of pertussis. Elevated IgG antibody to PT is detected approximately 2 to 8 weeks after a coughing illness and is indicative of recent pertussis disease (only in the absence of recent pertussis vaccination). A single positive IgG level to PT (> 100 IU/mL) is indicative of diagnosis. Complete blood count may show marked leukocytosis (15,000 to 100,000 cells/µL) owing to an increase in absolute lymphocytes in approximately 75% of unimmunized children. A chest radiograph may show perihilar infiltrate or interstitial edema with or without atelectasis. Consolidation is noted in pertussis cases complicated by secondary bacterial infection.\n\nPertussis cases declined in the United States after the introduction of whole-cell pertussis (DTP) vaccine in the 1940s. A gradual increase in pertussis cases was noted during the 1980s. By 2017, pertussis cases peaked at more than 18,000. Acellular pertussis (DTaP) vaccine replaced DTP for all doses by 1997. Immunity to pertussis wanes over time after receipt of acellular pertussis vaccine. The epidemiology of pertussis has changed in the United States owing to many contributing factors, including the exclusive use of acellular pertussis vaccines, waning immunity, changes to the microorganism, vaccine refusal, and undetected cases. Adults are reservoirs of the infection. Children younger than 1 year have the highest reported rate of pertussis; infants younger than 6 months are at risk of experiencing severe disease and death. The incubation period of pertussis is 7 to 10 days but may be prolonged up to 21 days. Clinical illness is categorized into three stages, each lasting approximately 2 weeks; the total duration of illness ranges from 6 to 10 weeks. In the initial catarrhal stage, patients exhibit symptoms of cough and rhinorrhea. This is followed by the paroxysmal stage, consisting of repeated coughing spells during the same breath followed by a \"whoop\" with associated emesis. Fever is typically low grade or absent. The illness ends with the convalescent stage, during which symptoms wane over weeks to months.\n\nPertussis must be included in the differential diagnosis of patients who have perioral cyanosis during coughing spells, posttussive emesis, and prolonged coughing illness.\n\nYoung infants are at risk of experiencing complications such as pneumonia, pulmonary hypertension, hypoxia, seizures, and encephalopathy. The disease is fatal in approximately 1% of infants younger than 2 months and 0.5% in affected infants aged 2 to 11 months. Complications of pertussis in adults include syncope, rib fractures, pneumonia, and weight loss. The systemic manifestations and laboratory findings of marked leukocytosis and lymphocytosis noted in infants with pertussis may be related to pertussis toxin, a key virulence factor of B pertussis. Other Bordetella species include Bordetella parapertussis, which can result in a less severe pertussis-like illness. A number of other infections due to community respiratory viruses can mimic pertussis, among them adenovirus, respiratory syncytial virus, Mycoplasma pneumoniae, and Chlamydia trachomatis.\n\nThe treatment and postexposure prophylaxis antimicrobial agent of choice for pertussis is a 5-day course of azithromycin, regardless of age. Trimethoprim-sulfamethoxazole can be used as an alternative agent in patients older than 2 months who are not able to tolerate azithromycin or are infected with a macrolide-resistant strain (Item C162). In addition to standard precautions, droplet isolation for at least 5 days after initiation of azithromycin therapy is recommended for hospitalized patients with pertussis.\n\nImmunization remains the key strategy to prevent pertussis. In 2012, universal maternal immunization with TdaP during each pregnancy, preferably at 27 to 36 weeks of gestation, was recommended to prevent pertussis-associated deaths in young infants. Early diagnosis of pertussis in conjunction with initiation of appropriate antimicrobial therapy may be associated with lower case fatality in infants.\n\nPREP Pearls\n• Pertussis in young infants can have an atypical presentation characterized by gagging, gasping, apnea, or bradycardia without the characteristic \"whoop.\"\n• Children younger than 1 year have the highest reported rate of pertussis; infants younger than 6 months are at risk of experiencing severe disease and death.\n• Immunization is the key strategy to prevent pertussis.\n\nABP Content Specifications(s)\n• Recognize the clinical features associated with pertussis in children of various ages\n• Understand the epidemiology of Bordetella pertussis\n• Plan the appropriate diagnostic evaluation of a patient in whom pertussis is suspected\n• Plan the appropriate management of pertussis in its various stages, including treatment for contacts of infected patients\n\nSuggested Readings\n• American Academy of Pediatrics. Pertussis (whooping cough). In: Kimberlin DW, Brady MT, Jackson MA, Long SS, eds. Red Book: 2018-2021 Report of the Committee on Infectious Diseases. 31st ed. Elk Grove Village, IL: American Academy of Pediatrics; 2018:620-634. Red Book Online.\n• Daniels HL, Sabella C. Bordetella pertussis (pertussis). Pediatr Rev. 2018;39(5):247-257. doi:10.1542/pir.2017-0229.\n• Sabella C. Pertussis (whooping cough). In: McInerny TK, Adam HM, Campbell DE, DeWitt TG, Foy JM, Kamat DM, eds. American Academy of Pediatrics Textbook of Pediatric Care. 2nd ed. Elk Grove Village, IL: American Academy of Pediatrics; 2017:2493-2497. Pediatric Care Online .\n• Tiwari T, Murphy TV, Moran J; National Immunization Program, CDC. Recommended antimicrobial agents for the treatment and postexposure prophylaxis of pertussis: 2005 CDC guidelines. MMWR Recomm Rep. 2005;54(RR-14):1-16."}
{"id" : 278, "question_text" : "An 8-year-old boy presents with cola-colored urine without blood clots. He was well until 2 days ago, when he developed a sore throat with upper respiratory tract infection symptoms. He denies any dysuria, frequency, urgency, flank pain, or trauma. On physical examination, his temperature is 37.8°C, heart rate is 84 beats/min, respiratory rate is 18 breaths/min, and blood pressure is 118/78 mm Hg. He has no costovertebral tenderness, abdominal tenderness, or edema. The urinalysis reveals: Specific gravity, 1.025; pH, 6.0; 3+ blood; 3+ protein; 1+ leukocyte esterase; Nitrite, negative. Microscopy shows more than 100 red blood cells/high-power field (hpf) and 5 to 10 white blood cells/hpf. Other laboratory findings include: Blood urea nitrogen, 24 mg/dL (8.6 mmol/L); Creatinine, 0.9 mg/dL (79.6 mcmol/L); Complement component 3 (C3), 140 mg/dL (normal, 80 to 200 mg/dL); Complement component 4 (C4), 30 mg/dL (normal, 16 to 40 mg/dL); Antinuclear antibody, negative. Of the following, the MOST likely diagnosis is", "options" : "[\"acute pyelonephritis\", \"immunoglobulin A glomerulonephritis\", \"postinfectious glomerulonephritis\", \"urolithiasis\", \"viral cystitis\"]", "explanation" : "The boy described in the vignette presents with the clinical picture of acute glomerulonephritis (cola-colored urine, hematuria/proteinuria, and mild azotemia). Lower tract bleeding, such as from viral hemorrhagic cystitis or stones within the urinary tract, is characterized by bright red urine, often accompanied by clots. Cola-colored urine without clots points to the upper urinary tract, typically glomerular hematuria. The boy's history reveals a close temporal relationship between the onset of pharyngitis and cola-colored urine. This 2-day lag period between the upper respiratory symptoms and the nephritis is sometimes described as synpharyngitic, which is characteristic of but not specific for immunoglobulin A (IgA) glomerulonephritis. Such a temporal relationship differs from that seen in postinfectious or poststreptococcal glomerulonephritis, in which a 7- to 21-day lag period is seen between the onset of pharyngitis and the development of gross hematuria.\n\nThe evaluation of a child in whom acute glomerulonephritis (AGN) is suspected includes determining the blood pressure and the renal function. If the patient is normotensive or has only mildly elevated blood pressure, the focus turns to the serum creatinine. Mild elevation of the creatinine rules out severe nephritis at that moment, but the creatinine measurement may need to be repeated in a day or two to assess whether it is rising, remaining the same, or returning toward normal. Children who have AGN and a rising serum creatinine require urgent nephrology consultation for possible renal biopsy. Following assessment of serum creatinine, a serologic evaluation should be undertaken, including complement factor 3 and 4 (C3 and C4), antinuclear antibody, and anti-double-stranded DNA. The boy in the vignette has normal C3 and C4 values. The combination of a normocomplementemic GN and a synpharyngitic pattern to the onset of glomerulonephritis makes IgA glomerulonephritis likely. IgA glomerulonephritis can also present with asymptomatic hematuria or hematuria/proteinuria. Patients who have gross hematuria have a better prognosis. Definitive diagnosis requires a renal biopsy, which also allows assessment of the severity of IgA glomerulonephritis. Treatment usually consists of immunosuppression such as with corticosteroids and angiotensin-converting enzyme inhibitors, depending on the renal biopsy findings and the severity of azotemia and proteinuria.\n\nAlternative diagnostic explanations for the patient in the vignette include AGN due to Alport nephritis and membranoproliferative glomerulonephritis (hypocomplementemic in two thirds of cases at disease onset), both of which are less common than IgA glomerulonephritis. The patient lacks any clinical features of acute pyelonephritis. It should be noted that the mild pyuria (5 to 10 white blood cells/hpf) is consistent with the inflammation accompanying the glomerulonephritis. Patients who have pyelonephritis would have more pyuria, and the white blood cell count would be greater than the red blood cell count.\n\nAmerican Board of Pediatrics Content Specification(s):\n\nRecognize the signs and symptoms of IgA nephropathy"}
{"id" : 2495, "question_text" : "A 16-year-old male soccer player is evaluated for right knee pain. He recalls no trauma. The medial knee has gradually become more painful during activity in the past several weeks. He now limps with activity and reports locking of the right knee. Physical examination reveals a limp, mild effusion of the right knee, and limited extension of the right leg. The remainder of his physical examination findings are unremarkable, including testing of the ligaments and meniscus. Four-view knee radiographs are obtained. The anterior posterior knee image is shown in Figure 1.\n\nOf the following, the adolescent's MOST likely diagnosis is", "options" : "[\"anterior cruciate ligament tear\", \"bone contusion\", \"meniscus tear\", \"osteochondritis dissecans\"]", "explanation" : "Reprinted with permission from Atanda A Jr, Reddy D, Rice JA, Terry MA. Injuries and chronic conditions of the knee in young athletes. Pediatr Rev. 2009;30(11):424.\n\nFigure 1. Knee radiographs of the adolescent described in the vignette.\n\nCritique\nThe adolescent in the vignette's history and physical examination findings are most consistent with osteochondritis dissecans (OCD) (Figure 2). The joint effusion is concerning and could be secondary to an anterior cruciate ligament or meniscus tear; however, the lack of trauma and the insidious onset of symptoms are inconsistent with those diagnoses. Additionally, the findings on ligament and meniscus examination are normal. The limited extension of the right knee is likely caused by a loose body in an unstable joint with OCD. A bone bruise would not have the gradual, insidious onset of signs and symptoms seen in this adolescent.\n\nReprinted with permission from Atanda A Jr, Reddy D, Rice JA, Terry MA. Injuries and chronic conditions of the knee in young athletes. Pediatr Rev. 2009;30(11):424\n\nFigure 2. Osteochondral dissecans lesion seen on the medial femoral condyle on A. plain radiograph and B. magnetic resonance T1-weighted coronal image.\n\nOsteochondritis dissecans is a focal abnormality in the subchondral bone of the knee, elbow, or ankle joint, which can progress to joint instability. Joint instability can lead to bone and overlying articular cartilage detachment, resulting in a loose body within the joint space. A loose body or irregular bony surface leads to pain, swelling, locking of the affected joint, and limited joint range of motion. The most common location of knee OCD is the medial femoral condyle, although OCD of the lateral femoral condyle, trochlea, patella, and tibia have been reported. Osteochondritis dissecans is best visualized by 4-view knee radiographs that include anterior-posterior, lateral, tunnel, and sunrise projections. The tunnel view will best demonstrate OCD in the posterior femoral condyles. The sunrise view may identify a patellar or trochlear OCD.\n\nOsteochondritis dissecans in the knee may be asymptomatic or may present with symptoms such as pain, swelling, locking, and limited range of motion. During adolescence, OCD is more common in boys than girls. Notably, children 6 to 10 years of age have normal baseline irregularity (ossification variant) of the posterior femoral condyles, which can mimic OCD on radiographs; age and development should always be taken into account when interpreting imaging studies. In children older than 10 years, irregularity of the femoral condyles warrants further evaluation. Magnetic resonance imaging (MRI) is typically used to characterize the size and stability of OCD.\n\nBoth nonoperative and operative options exist for treatment of OCD; orthopedic surgery consultation is recommended regarding management. The ultimate goal is to achieve bony healing and prevent breakdown of the joint. Nonoperative management may be appropriate in the case of an open femoral physis (indicating healing potential) if the OCD appears stable on MRI. When a radiographic diagnosis of OCD is made incidentally, an asymptomatic OCD may be followed with serial radiographs until full ossification or healing is seen. A symptomatic OCD that is stable on imaging with an open femoral physis may also be followed with serial radiographs every 3 to 6 months. In such a case, the patient and family should be counseled to restrict high-impact exercise, such as running, jumping, and pivoting, while the OCD is being monitored. If after 3 to 6 months, there is persistent pain, joint instability, or no radiographic evidence of healing, surgery should be considered. Adolescents with a closed femoral physis typically require surgery to drill, fix, or graft the OCD lesion. Operative treatment is also recommended for all unstable OCD lesions seen initially on MRI.\n\nSuggested Reading(s)\nAtanda A Jr, Reddy D, Rice JA, Terry MA. Injuries and chronic conditions of the knee in young athletes.\nPediatr Rev. 2009;30(11):419-428; quiz 429-430. doi:10.1542/pir.30-11-419\nCruz AI Jr, Shea KG, Ganley TJ. Pediatric knee osteochondritis dissecans lesions. Orthop Clin North Am.\n2016;47(4):763-775. doi:10.1016/j.ocl.2016.05.001\nKannikeswaran N, Srinivasan Suresh S. Sports musculoskeletal injuries. In: McInerny TK, Adam HM,\nCampbell DE, DeWitt TG, Foy JM, Kamat DM, eds. American Academy of Pediatrics Textbook of Pediatric\nCare. American Academy of Pediatrics; 2021:chap 334. Pediatric Care Online\nUppstrom TJ, Gausden EB, Green DW. Classification and assessment of juvenile osteochondritis\ndissecans knee lesions. Curr Opin Pediatr. 2016;28(1):60-67. doi:10.1097/MOP.0000000000000308\nWall EJ, Brtko K. The nonoperative treatment of osteochondritis dissecans of the knee. Curr Opin\nPediatr. 2021;33(1):59-64. doi:10.1097/MOP.0000000000000976\n\nContent Domain\nSports Medicine\n\nABP Content Specification(s) / Content Area(s)\nRecognize the clinical findings associated with sports-related internal derangement of the knee\nUnderstand when orthopedic consultation is required for a sports-related knee injury\n\nThe correct answer is: osteochondritis dissecans"}
{"id" : 1440, "question_text" : "You are seeing a 16-year-old adolescent boy with cystic fibrosis in your office. Over the last 5 years, he has had poor adherence to medical recommendations, with frequent hospital admissions and marked losses in weight and lung function. In addition, he smokes cigarettes, frequently drinks beer, and has dropped out of school. His parents recently divorced. His weight and body mass index are at the fifth percentile. His forced expiratory volume in 1 second is 45% of predicted. His sputum cultures are growing a pan-resistant, mucoid Pseudomonas aeruginosa and methicillin-resistant Staphylococcus aureus. Computed tomography of the chest reveals bilateral bronchiectasis, most notably at the upper lobes. The adolescent and his parents have repeatedly declined gastrostomy tube placement for supplemental nutrition. On multiple occasions, you and the cystic fibrosis team have counseled the adolescent regarding the importance of managing his chronic disease. He has told at least 1 team member: \"everything will be fine once I qualify for lung transplantation.\" Of the following, the MOST appropriate next step in management of this patient is to", "options" : "[\"refer him for lung transplantation\", \"refer him for palliative care\", \"refer him for psychological/psychiatric support services\", \"seek a court order for directly observed therapies\", \"transfer his care to an adult cystic fibrosis center\"]", "explanation" : "Correct Answer: C\nThe most appropriate next step in management of this adolescent is referral for psychological/psychiatric support services. The 16-year-old patient in the vignette is experiencing many of the complications of late-stage cystic fibrosis (CF). A comprehensive approach to care across the life spectrum is advocated for patients with CF and many other chronic diseases in which increased survival has led to a concomitant increase in complications and comorbidities. The complex care regimen required in CF includes multiple medications, nutritional support, and airway clearance therapies. Difficulties families experience with managing care and nonadherence with various treatments may reflect the chronic stress of coping with a life-threatening illness; these patients often present with depression and/or anxiety.\n\nCystic fibrosis care has evolved over the years. Newborn screening for CF, now implemented in all 50 US states, has facilitated optimization of nutrition and respiratory health in affected children from infancy. Increasingly effective management with nutritional advancements, airway clearance options, inhaled antibiotics, and the newest CF transmembrane regulator modification agents has led to increased disease survival. The median age of survival for a child born and diagnosed with CF in 2010 is now estimated at 39 years.\n\nIncreased survivability allows patients to pursue life goals that were previously less available such as higher education, marriage, and parenthood. Accompanying the increased lifespan, however, has been an increase in disease and treatment-associated complications and comorbidities. These include, but are not limited to, an increased prevalence of CF-related liver disease, CF-related diabetes with attendant microvascular and renal complications, as well as vestibular and renal sequelae from years of aminoglycoside treatment for chronic pseudomonal infection. Lung transplantation may be indicated for end-stage pulmonary disease and frequent infections, giving further hope for disease survival. However, availability of organs is limited and patients may succumb to disease while awaiting transplantation. As children become adolescents and young adults, transition of care may be an additional stressor for the patient and their family; care providers may change and the primary responsibility for disease management shifts from the parent to the affected individual.\n\nAdherence to CF treatment has historically been greater with respiratory and gastrointestinal medications compared with nutritional supplementation and chest physiotherapy. Treatment adherence worsens with age and disease severity. Quality of life may suffer with the burden of chronic disease management.\n\nReported rates of depression in patients with CF range from 9% to 29% in children and from 13% to 33% in adults. Similarly, rates of anxiety range from 30% to 33%. Parents and caregivers also demonstrate increased rates of depression and anxiety. High levels of depression have been associated with less positive beliefs regarding medications, which may then affect treatment adherence. Furthermore, depressed patients with CF require hospitalization at a rate more than 3 times that of their nondepressed counterparts. As seen with any patient with depression, those affected with CF are at increased risk for substance abuse and school failure.\n\nFor the patient in the vignette, a court order for directly observed therapies (DOTs) is not likely to afford a feasible or long-term option for chronic disease management. The DOTs are generally considered for treatment regimens of defined lengths, such as in the treatment of tuberculosis. Transfer of care to an adult center is not likely to result in improved adherence. Although the age recommended for transition to adult care is highly variable across centers, the boy in the vignette is somewhat young for this change. Most patients will transition to adult care between 17 and 23 years of age. Patients often have difficulty with care transitions, and every attempt should be made, ideally before implementation, to engage the patient in his or her own disease management.\n\nLung transplantation referral is recommended when the forced expiratory volume in 1 second (FEV1) is 30% or less of that predicted. Referral is also appropriate when the FEV1 is greater than 30%, but is demonstrating rapid and progressive decline. Psychosocial problems that cannot be resolved are a relative contraindication for transplantation. Candidates for a lung transplant must be free of substance addiction for at least 6 months. Nutritional concerns are also critically important, and have been identified as a negative predictor for surgical outcome.\n\nIn general, patients with CF and their caregivers should be engaged in care directive and end-of-life discussions. Palliative care focuses on the management of symptoms and improving quality of life, regardless of prognosis. In a survey of adults with CF, nearly 80% reported feeling comfortable talking to their care provider about advance directives. Therefore, palliation may be viewed as an option in those patients who wish to forego life-extending options such as transplantation. Patient age, disease severity, and mental health concerns should be considered and addressed; a unilateral referral to palliative care without addressing reasons for nonadherence is not advocated.\n\nPREP Pearls\n• Individuals with cystic fibrosis and other chronic diseases often have a significant burden of disease management.\n• Depression and anxiety are frequently overlooked comorbid conditions in chronic disease.\n• Depression and anxiety may adversely affect medication adherence and may be modifiable factors in disease progression.\n\nABP Content Specifications(s)\n• Recognize the importance of planning for survival into adulthood for patients with cystic fibrosis\n\nSuggested Readings\n• Arias Llorente RP, Bousoño García C, Díaz Martín JJ. Treatment compliance in children and adults with cystic fibrosis. J Cyst Fibros. 2008;7(5):359-367. doi: http://dx.doi.org/10.1016/j.jcf.2008.01.003.\n• MacKenzie T, Gifford AH, Sabadosa KA, et al. Longevity of patients with cystic fibrosis in 2000 to 2010 and beyond: survival analysis of the cystic fibrosis foundation patient registry. Ann Intern Med. 2014;161(4):233-241. doi: http://dx.doi.org/10.7326/M13-0636.\n• Quittner AL, Saez-Flores E, Barton JD. The psychological burden of cystic fibrosis. Curr Opin Pulm Med. 2016;22(2):187-191. doi: http://dx.doi.org/10.1097/MCP.0000000000000244.\n• Robinson WM. Palliative and end-of-life care in cystic fibrosis: what we know and what we need to know. Curr Opin Pulm Med. 2009;15(6):621-625. doi: http://dx.doi.org/10.1097/MCP.0b013e3283304c29."}
{"id" : 2611, "question_text" : "A 10-month-old male infant with sickle cell disease is seen in the emergency department for evaluation of fever and fussiness of 3 days' duration. He has reduced his formula intake and had a few episodes of nonbloody, nonbilious emesis. This morning it was hard to wake him up. On physical examination, the infant is irritable. His temperature is 40°C, heart rate is 150 beats/min, respiratory rate is 25 breaths/min, and oxygen saturation is 100% in room air. Laboratory data are shown: Laboratory Test Result Serum White blood cell count 25,000/µL (25.0 × 109/L) Hemoglobin 10 g/dL (100 g/L) Platelet count 90 × 103/µL (90 × 109/L) Cerebrospinal fluid White blood cell count 550/µL (0.55 × 109/L) Protein 190 mg/dL Glucose 15 mg/dL (0.83 mmol/L) Gram stain gram-negative coccobacilli Polymerase chain reaction Haemophilus species detected Three days later, the cerebrospinal fluid culture grew Haemophilus influenzae serotype b. His parents are concerned and ask how to prevent transmission to his 5-year-old sister who is currently undergoing chemotherapy for acute lymphoblastic leukemia. Of the following, the MOST appropriate chemoprophylaxis regimen for this child's household members is", "options" : "[\"amoxicillin\", \"azithromycin\", \"rifampin\", \"trimethoprim-sulfamethoxazole\"]", "explanation" : "The infant in the vignette has meningitis from Haemophilus influenzae type b. Secondary disease accounts for less than 2% of invasive Haemophilus influenzae b infections. The highest-risk individuals are unimmunized household contacts younger than 4 years. Children with sickle cell disease, asplenia, HIV infection, cancer, or a primary immunodeficiency are more susceptible to invasive infection. Chemoprophylaxis for close contacts protects susceptible individuals from acquiring infection by eliminating colonization. Rifampin achieves high concentrations in the respiratory secretions. At a dose of 20 mg/kg, orally, once a day for 4 days, rifampin is one of the most effective antimicrobial agents to eradicate nasopharyngeal carriage of H influenzae b. Amoxicillin, trimethoprim sulfamethoxazole, and azithromycin are ineffective antimicrobial prophylaxis agents in this situation.\n\nHaemophilus influenzae is a gram-negative coccobacillus, typically considered normal flora of the upper respiratory tract in humans. Encapsulated strains (a, b, c, d, e, and f) cause invasive infections, and the unencapsulated strains are commonly associated with mucosal infections but are also responsible for invasive infections.\n\nBefore the widespread use of the conjugate Haemophilus influenzae b vaccine (pre-1987), invasive disease was the leading infectious diseases problem in young children, with those between ages 6 and 18 months at highest risk. Haemophilus influenzae was responsible for more than 95% of invasive infections in children (meningitis, bacteremia, epiglottitis, pneumonia, septic arthritis). The incidence of invasive disease decreased by 99% in the post-vaccine era (1989-2000). Non–type b capsular strains (a, c, d, e, and f) and nontypeable strains are responsible for most cases of invasive infection in infants and young children since the universal use of Haemophilus influenzae b conjugate vaccination. The non-b encapsulated H influenzae serotypes cause invasive disease similar to that seen with H influenzae b. Vaccination against H influenzae b does not confer immunity against other encapsulated or unencapsulated strains. Nontypeable H influenzae is currently responsible for half of the cases of mucosal infections such as otitis media, sinusitis, conjunctivitis, and bronchitis.\n\nSecondary cases after H influenzae b meningitis are rare. Rifampin prophylaxis is recommended for all household members, including adults, if the household has a contact younger than 4 years who is not fully immunized. Rifampin prophylaxis is also recommended for all household members if anyone younger than 18 years has an immunocompromised condition, irrespective of vaccination status. Rifampin prophylaxis for the index case is suggested if an antibiotic other than ceftriaxone or cefotaxime is used to treat invasive infection. Chemoprophylaxis in a daycare or school setting is advised if there are 2 or more cases among the attendees exposed within 60 days.\n\nClinical manifestations and management of Haemophilus influenzae infections are described in Item C103.\n\nPREP Pearls\n• Haemophilus influenzae is a gram-negative coccobacillus. Infections in children are caused by encapsulated and unencapsulated strains.\n• Type b Haemophilus influenzae was a common cause of invasive infections before the widespread use of the conjugated vaccine. Invasive infections are now caused by other encapsulated strains (a, c, d, e, and f) and nontypeable strains. The nontypeable strains account for 50% of mucosal infections such as otitis media and sinusitis.\n• In cases of Haemophilus influenzae type b meningitis, rifampin prophylaxis is recommended for all household members, including adults, if the household has a child younger than 4 years who is not fully immunized.\n\nABP Content Specifications(s)\n• Plan the appropriate management of a typable and nontypable Haemophilus influenzae infection\n• Recognize the clinical features associated with typable and nontypable Haemophilus influenzae infection\n• Plan appropriate prophylaxis for individuals exposed to invasive Haemophilus influenzae type B\n• Understand the epidemiology of Haemophilus influenzae infection\n\nSuggested Readings\n• American Academy of Pediatrics. Haemophilus influenzae infections. In: Kimberlin DW, Barnett ED, Lynfield R, Sawyer MH, eds. Red Book: 2021–2024 Report of the Committee on Infectious Diseases. 32nd ed. American Academy of Pediatrics; 2021. Accessed September 1, 2022. Red Book Online.\n• Briere EC, Rubin L, Moro PL, et al. Prevention and control of Haemophilus influenzae type b Disease Recommendations of the Advisory Committee on Immunization Practices (ACIP). Centers for Disease Control and Prevention. MMWR Recomm Rep. 2014;63(RR-1):1-14. https://pubmed.ncbi.nlm.nih.gov/24572654/.\n• Murphy TF. Haemophilus species, including H. influenzae and H. ducreyi (chancroid). In: Bennett JE, Dolin R, Blaser MJ, eds. Mandell, Douglas and Bennett's Principles and Practice of Infectious Diseases. 8th ed. Saunders; 2015:25752583.e2.\n• St. Geme JW, Rempe KA. Haemophilus influenzae. In: Long SS, Prober CG, Fischer M, eds. Principles and Practice of Pediatric Infectious Diseases. 5th ed. Elsevier; 2018:926-931.e3.\n• Weinberg GA, Buchanan AM. Meningitis. In: McInerny TK, Adam HM, Campbell DE, DeWitt TG, Foy JM, Kamat DM, eds. American Academy of Pediatrics Textbook of Pediatric Care. American Academy of Pediatrics; 2021:chap 289. Accessed September 1, 2022. Pediatric Care Online."}
{"id" : 366, "question_text" : "A 10-month-old girl presents to your office because of diarrhea, irritability, and a fall-off in her weight gain. She was born at term to a 28-year-old gravida 1, para 1, woman following an uncomplicated pregnancy and delivery and had a birthweight of 2,900 g. She was exclusively breastfed for the first 2 months after birth. At 2 months of age, human milk was supplemented with cow milk protein-based formula. Because of increasing problems with postprandial emesis and \"colicky\" symptoms, human milk feeding was discontinued and several formula changes were attempted. At 6 months of age, she was started on a soy protein formula thickened with rice cereal because of symptoms suggesting gastroesophageal reflux. Since that time, her mother has noted an increased frequency and reduced consistency of the baby's stools. The infant now passes six to eight loose-to-watery bowel movements per day. Physical examination of the alert, well-developed, somewhat thin-appearing infant shows a length of 72 cm and a weight of 8.0 kg. The only additional finding of note is an erythematous and eroded diaper dermatitis. Of the following, the MOST appropriate diagnostic test for this infant is a", "options" : "[\"breath hydrogen test\", \"d-xylose absorption study\", \"lactose tolerance test\", \"tissue transglutaminase antibody\", \"zinc measurement\"]", "explanation" : "The infant described in the vignette has experienced a fall-off in weight gain associated with the passage of multiple loose, watery stools per day. Coincidentally, diarrhea began soon after the baby's diet was changed to a soy formula thickened with rice cereal. The erythematous, eroded diaper dermatitis is consistent with the passage of watery, acidic stools, reflecting an osmotic diarrhea accompanied by bacterial fermentation of unabsorbed carbohydrate. Because proprietary soy formulas contain sucrose or glucose polymers as their constituent carbohydrates and rice cereal is composed largely of complex glucose polymers as starch molecules, the infant's dietary history and clinical findings strongly suggest a diagnosis of sucrase-isomaltase (SI) deficiency, the most common congenital disaccharidase deficiency. The most appropriate and noninvasive study to confirm this diagnosis is a breath hydrogen test following oral administration of sucrose as the carbohydrate challenge. A definitive diagnosis of SI deficiency requires upper gastrointestinal tract endoscopy and duodenal biopsy for direct measurement of SI activity.\n\nIntestinal malabsorption is usually suspected on the basis of clinical findings that include bulky, malodorous stools; chronic diarrhea; and growth and weight gain disturbances. In many patients, even those who do not have a prominent history of diarrhea, other signs of malnutrition suggest a problem in nutrient absorption or utilization. Disturbed digestive-absorptive function may result in anemia, bruising, edema, osteopenia, and reduced muscle mass. The differential diagnosis is a long one and includes both common and rare disorders, several of which may present during early infancy. The major causes of malabsorption during infancy and childhood include digestive abnormalities, alterations in the intraluminal milieu, infectious processes, mucosal changes leading to a reduced villus absorptive surface area, shortened bowel length, and nutrient transport defects.\n\nSpecific screening tests are used to document the malabsorptive state. Reaching a definitive diagnosis often requires referral to a pediatric gastroenterologist for the performance of more invasive testing, including upper endoscopy and small bowel biopsy. Initial screening should include an examination of the stool. An acidic fecal pH indicates bacterial fermentation, and a stool reducing substances test can identify any unfermented reducing sugars. The fecal fat stain shows the presence of excreted lipid and should be a precursor to a formal 72-hour quantitative fecal fat determination. Fat malabsorption often is manifested by the passage of bulky, malodorous stools, but it may also be accompanied by a complaint of constipation. In any case, when fat malabsorption is suspected, either because of stool characteristics or signs of malnutrition, pancreatic insufficiency must be considered. For the general pediatrician, this problem should be evaluated initially with a sweat chloride determination to rule out cystic fibrosis, by far the most common cause of pancreatic insufficiency in childhood. Other useful screening tests in the evaluation of pancreatic excretory function include fecal elastase and trypsin assessment. Formal pancreatic function testing involves endoscopy and duodenal intubation and should be carried out only in a specialized referral center.\n\nAn important screening study for any patient presenting with a suspicion of malabsorption, signs of malnutrition, or undiagnosed gastrointestinal symptoms is the tissue transglutaminase antibody (TTG) assessment, a highly sensitive and specific screening test for celiac disease. This test is useful as a diagnostic tool only if the patient is consuming a source of gluten. Because TTG is an immunoglobulin A (IgA) antibody, the test should always be accompanied by assessment of total IgA to identify patients who may be IgA-deficient. Other studies may be used to assess overall small bowel absorptive function as a measure of villus surface area and integrity in patients who have suspected diffuse microvillus abnormalities. These tests, however, do not provide specific diagnostic information about the primary disease. The d-xylose absorption study takes advantage of this five-carbon sugar's property to be excreted in the urine following proximal jejunal absorption without undergoing intraluminal hydrolysis or endogenous metabolism.\n\nVitamin and trace minerals may be measured as an indicator of fat absorption (vitamins A, D, E, K) as well as ileal (vitamin B12) and proximal small bowel absorptive function (iron, zinc, folate). Although serum zinc concentrations are not accurate predictors of total body zinc nutriture, low values have been reported in malnourished states associated with underlying bowel disorders, including Crohn disease and celiac disease. The lactose tolerance test has largely been supplanted by the lactose breath hydrogen test to identify patients who have lactose intolerance, a common problem in those who have bowel disease, malnutrition, and chronic diarrhea. Inflammatory bowel (IBD) serologies may also be obtained, particularly when there is a family history of IBD, or in the presence of other signs and symptoms of Crohn disease. However, negative studies do not rule out IBD, especially in pediatric patients.\n\nCritique: The infant described in the vignette has experienced a fall-off in weight gain associated with the passage of multiple loose, watery stools per day. Coincidentally, diarrhea began soon after the baby's diet was changed to a soy formula thickened with rice cereal. The erythematous, eroded diaper dermatitis is consistent with the passage of watery, acidic stools, reflecting an osmotic diarrhea accompanied by bacterial fermentation of unabsorbed carbohydrate. Because proprietary soy formulas contain sucrose or glucose polymers as their constituent carbohydrates and rice cereal is composed largely of complex glucose polymers as starch molecules, the infant's dietary history and clinical findings strongly suggest a diagnosis of sucrase-isomaltase (SI) deficiency, the most common congenital disaccharidase deficiency. The most appropriate and noninvasive study to confirm this diagnosis is a breath hydrogen test following oral administration of sucrose as the carbohydrate challenge. A definitive diagnosis of SI deficiency requires upper gastrointestinal tract endoscopy and duodenal biopsy for direct measurement of SI activity.\n\nContent Specifications: Know the appropriate laboratory tests for malabsorption"}
{"id" : 1576, "question_text" : "An 11-year-old boy recently adopted from an orphanage in Burma is brought to your office for a health supervision visit. The child reports no symptoms and is feeling well. No information is known about his biological parents. The results of his physical examination are normal. Laboratory data are shown:\n\nLaboratory Test Result\nWhite blood cell count 8,000/µL (8.0 x 109/L)\nPlatelet count 185 x 103/µL (185 x109/L)\nAbsolute eosinophil count 3,720/µL (3.72 × 109/L) (normal, < 0.45× 109/L)\nHemoglobin 11.5 g/dL (115 g/L) (normal, 13.5-17.5 g/dL)\nHuman immunodeficiency virus Negative\n\nA purified protein derivative skin test for tuberculosis is negative. Three stool samples are negative for ova and parasites.\n\nOf the following, the BEST next test to determine the cause of this child's eosinophilia is serology for", "options" : "[\"Chagas disease\", \"hepatitis B virus\", \"hydatid disease\", \"Strongyloides stercoralis\", \"Toxocara canis\"]", "explanation" : "Correct Answer: D\nEach year, more than 12,000 children from Asia, Latin America, Eastern Europe, and Africa are adopted by parents in the United States. Infectious diseases are the most frequently diagnosed medical problem among immigrant children on arrival to the United States. The American Academy of Pediatrics has published guidelines related to providing care for immigrant children including initial medical evaluation and screening tests for infectious diseases. The guidelines also address immunizations, cultural adjustment, nutrition, growth and development, and psychosocial needs.\n\nThe recently immigrated boy from Burma described in this vignette is clinically asymptomatic but has eosinophilia defined as an absolute eosinophil count greater than 450 cells/µl. Stool test results were negative for ova and parasites. In such cases, serologic testing must be considered for tissue-invasive parasitic infections such as strongyloidiasis, schistosomiasis, and lymphatic filariasis. Irrespective of country of origin, all international adoptees and refugees coming to the United States with eosinophilia must undergo serologic testing for Strongyloides stercoralis after exclusion of common pathogens associated with eosinophilia. Serologic testing for Schistosoma species is recommended for children with eosinophilia immigrating from sub-Saharan Africa, Southeast Asia, or certain regions of Latin America after exclusion of common pathogens associated with eosinophilia. Likewise, serologic testing for lymphatic filariasis should be considered for children aged 2 years and older with eosinophilia immigrating from countries endemic for lymphatic filariasis. Immigrant children should also be screened for hepatitis B and C, syphilis, HIV types 1 and 2, and tuberculosis (Item C48).\n\nStrongyloidiasis is an intestinal helminth infection that is endemic in Africa, Asia, areas of Latin America (Argentina, Ecuador, Venezuela, Peru, and Brazil), the Caribbean, and the southeast United States. Humans acquire infection after contact with contaminated soil. Following skin penetration, the infective filariform larvae migrate to the lungs via a hematogenous route, penetrate the alveoli, ascend the bronchial tree, and are then swallowed. After reaching the duodenum, the larvae mature into adult females that release eggs; the eggs hatch into first-stage larvae that are released in feces. The life cycle is maintained when first-stage larvae molt and develop into infective filariform larvae, which penetrate the intestinal mucosa and cause autoinfection. Autoinfection may persist for decades. Chronic infections with S stercoralis are usually asymptomatic but eosinophilia is frequently noted. The drug of choice for treating strongyloidiasis is usually ivermectin. However, immigrants from countries endemic for loiasis (a filarial infection caused by Loa loa) should be treated with albendazole because ivermectin treatment is associated with toxic encephalopathy in individuals with high blood concentrations of L loa microfilariae.\n\nChagas disease (American trypanosomiasis) is endemic in Latin America, and serologic testing for Trypanosoma cruzi should be considered in immigrant children from Chagas disease–endemic nations. Serologic testing for hepatitis B virus is recommended as part of the routine evaluation of internationally adopted or immigrant children but would not be the appropriate test to determine the cause of eosinophilia for the boy is this vignette. Eosinophilia may be noted in other tissue-invasive parasitic infections such as hydatid disease and toxocariasis; however, these infections are less common than intestinal helminth infections (or soil-transmitted helminth infections, such as ascariasis, trichuriasis, and hookworm infection) and schistosomiasis in internationally adopted and refugee children.\n\nPREP Pearls\n• All international adoptees and refugees coming to the United States with unexplained eosinophilia must undergo serologic testing for Strongyloides stercoralis after exclusion of common pathogens associated with eosinophilia.\n• Serologic testing for Schistosoma species and lymphatic filariasis should be considered for children with eosinophilia immigrating from endemic countries after exclusion of common pathogens associated with eosinophilia.\n• Immigrant children should also be screened for hepatitis B and C, syphilis, HIV types 1 and 2, and tuberculosis.\n\nABP Content Specifications(s)\n• Plan the appropriate infectious disease screening evaluation of an internationally adopted child\n• Plan the appropriate immunizations for an internationally adopted child\n\nSuggested Readings\n• American Academy of Pediatrics, Council on Community Pediatrics. Providing care for immigrant, migrant, and refugee children. Pediatrics. 2013; 131(6):e2028–e2034. doi: http://dx.doi.org/10.1542/peds.2013-1099.\n• American Academy of Pediatrics. Medical evaluation for infectious diseases for internationally adopted refugee, and immigrant children. In: Kimberlin DW, Brady MT, Jackson MA, Long SS, eds. Red Book: 2015 Report of the Committee on Infectious Diseases. 30th ed. Elk Grove Village, IL: American Academy of Pediatrics; 2015:194–201.\n• Weatherhead JE, Hotez PJ. Worm infection in children. Pediatr Rev. 2015;36(8):341–354. doi: http://dx.doi.org/10.1542/pir.36-8-341."}
{"id" : 1433, "question_text" : "A 9-day-old Jewish newborn is brought to your office for evaluation of bleeding at his circumcision site. He was born at term via a cesarean delivery for failure to progress after an uncomplicated pregnancy. He was discharged from the hospital on the second day after birth. On the eighth day after birth, a ritual circumcision was performed by a certified mohel (expert in the Jewish rite of circumcision). Since that time, there has been continuous and significant bleeding at the circumcision site, requiring a change of blood-soaked bandages every 2 hours. There is no family history of bleeding disorders. The newborn's physical examination is remarkable only for mild pallor and a continuously oozing, circumferential circumcision wound around the glans of the penis. A complete blood cell count is normal. His prothrombin time is 12 seconds and partial thromboplastin time is 85 seconds. His von Willebrand antigen is 90% (normal range, 50%–150%) and von Willebrand activity is 95% (normal range, 50%–150%). Of the following, the MOST likely diagnosis for the newborn in the vignette is", "options" : "[\"factor XIII deficiency\", \"Glanzmann thrombasthenia\", \"hemophilia A\", \"protein C deficiency\", \"type III von Willebrand disease\"]", "explanation" : "The prolonged bleeding after circumcision in the newborn in the vignette suggests a congenital bleeding disorder. The formation of a functional clot requires 2 components, fibrin and platelets. Fibrin is the end-product of the coagulation cascade. The absence of any of the coagulation cascade factors can result in failure to form a clot and is associated with a prolonged prothrombin time (PT) or partial thromboplastin time (PTT). Prothrombin time and PTT are effective measures of the functionality and presence of the components of coagulation cascade, except for the conversion of fibrinogen to fibrin. The patient's prolonged PTT suggests an absence or dysfunction of a factor in the coagulation cascade. The gene for factor VIII is found on the X chromosome and is the subject of frequent spontaneous mutations. The deficiency of factor VIII is known as hemophilia A. Given that the child is male and therefore subject to X-linked disorders, factor VIII deficiency is the most likely diagnosis.\n\nFactor XIII is responsible for clot stabilization. Since it plays a role after the formation of a clot, an absence of factor XIII would not prolong the PT or PTT; those are measures of the time to clot formation, not clot stabilization.\n\nThe most common congenital platelet function disorders are Bernard-Soulier syndrome (a disorder of platelet adhesion) and Glanzmann thrombasthenia (a disorder of platelet aggregation). Although both these disorders result in a bleeding phenotype, neither affects the clotting factor cascade, and therefore neither would result in a prolonged PTT.\n\nProtein C is an anticoagulant protein. A deficiency of protein C would result in an increased risk of thrombosis, not an increased risk of bleeding.\n\nVon Willebrand disease (vWD) is the most common congenital bleeding disorder. Von Willebrand factor is a linking factor that allows functional platelets to bind to fibrin to form a clot. Von Willebrand disease is the result of decreased function or absence of von Willebrand factor. Von Willebrand disease has multiple phenotypes ranging from mild to severe bleeding disorders that mirror the degree of dysfunction or absence of von Willebrand factor. There are several different types of vWD, including types 1, 2A, 2B, 2M, 2N, and 3. Although severe vWD could explain the neonate's presentation, his normal von Willebrand levels rule that out as the cause of his bleeding.\n\nPREP Pearls\n• Factor VIII deficiency, or hemophilia A, should be suspected in a male infant who presents with prolonged bleeding and a prolonged partial thromboplastin time.\n• Hemophilia A is an X-linked disorder.\n\nABP Content Specifications(s)\n• Recognize clinical findings associated with congenital coagulation factor deficiency\n\nSuggested Readings\n• Zimmerman B, Valentino LA. Hemophilia: in review. Pediatr Rev. 2013;34(7):289-295. doi: http://dx.doi.org/10.1542/pir.34-7-289.\n• Doering CB, Spencer HT. Replacing bad (F)actors: hemophilia. Hematol Am Soc Hematol Educ Program. 2014;2014(1):461-467. doi: http://dx.doi.org/10.1182/asheducation-2014.1.461.\n• Journeycake JM, Buchanan GR. Coagulation disorders. Pediatr Rev. 2003;24(3):83-91. doi: http://dx.doi.org/10.1542/pir.24-3-83.\n• Sharathkumar AA, Pipe SW. Bleeding disorders. Pediatr Rev. 2008;29(4):121-130. doi: http://dx.doi.org/10.1542/pir.29-4-121."}
{"id" : 794, "question_text" : "A 4-year-old girl has a 1-cm vertical laceration to her upper lip extending approximately 4 mm beyond the vermillion border from a fall into a bookshelf corner. The laceration is not through and through and not actively bleeding. What is the MOST appropriate response regarding treatment?", "options" : "[\"absorbable suture material should be used for the best possible outcome\", \"consultation with a plastic surgeon may be required to achieve an acceptable outcome\", \"lidocaine should be infiltrated liberally around the wound edges to provide adequate analgesia\", \"suture placement is not needed since the laceration primarily involves a mucosal surface\", \"use of a cyanoacrylate tissue adhesive would be a good option for laceration repair given the patient's young age\"]", "explanation" : "The girl in the vignette presents with a laceration to her upper lip with extension through the vermilion border. Lip lacerations require special attention for closure because they can result in significant cosmetic defects if not repaired properly. The vermilion border, the junction of the dry oral mucosa of the lip and the facial skin, serves as an important landmark for proper repair of a lip laceration when involved. Misalignment of the vermilion border by as little as 0.5 mm is easily noticeable. Although pediatric practitioners with prior training and experience in management of lip lacerations may possess the skill needed to properly repair lip injuries involving the vermilion border, consultation with an orofacial or plastic surgeon is indicated for most practitioners. When involved, the vermilion border should be the first area approximated in repair of a lip laceration. It is essential to identify and mark the vermilion border before initiating infiltration anesthesia or wound debridement. Infiltration of local anesthetic around the wound edges may lead to soft tissue swelling and tissue distortion, which can interfere with proper tissue apposition. This can be avoided through the use of regional nerve blocks to anesthetize the wound.\n\nWhen parted, the vermilion border should be reapproximated precisely using a 6-0 nonabsorbable nylon suture. In general, lip lacerations should be closed in layers, depending on the depth of the wound. Full-thickness lip lacerations require a 3-layer repair.\n\nAlthough small lip lacerations that involve only the inner mucosal surfaces of the lip may not require suture placement, the vermilion border must be precisely approximated whenever involved to avoid an unacceptable cosmetic outcome. Cyanoacrylate tissue adhesives are an acceptable option for repair of some uncomplicated facial lacerations, but they are not recommended for repair of injuries that involve the oral mucosa and would not facilitate exact alignment of the vermilion border.\n\nPREP Pearls\n• Lip lacerations that involve the vermilion border require special attention for closure. Misalignment of the vermilion border by as little as 0.5 mm is easily noticeable and can result in permanent cosmetic defects.\n• Consultation with an orofacial or plastic surgeon may be indicated for lip lacerations that involve the vermilion border.\n• In managing a child with a lip laceration, it is essential to identify and mark the vermilion border before initiating infiltration anesthesia or wound debridement.\n\nAmerican Board of Pediatrics Content Specification(s):\n• Recognize the problem with a laceration through the vermilion border of the lip\n\nSuggested Reading\n• Attia MW, Loiselle J. Management of soft-tissue injuries of the mouth. In: King C, Henretig FM, eds. Textbook of Pediatric Emergency Procedures. 2nd ed. Philadelphia, PA: Lippincott Williams & Wilkins; 2008:680-687\n• Harter DA, Miller S. Management of specific soft tissue injuries. In: Reichman EF, Simon RR, eds. Emergency Medicine Procedures. New York, NY: McGraw-Hill; 2004\n• Sagerman PL Wounds. Pediatr Rev. 2005;26:43-49. doi:10.1542/pir.26-2-43\n• Selbst SM, Attia MW. Minor trauma—lacerations. In: Fleisher GR, Ludwig S, eds. Textbook of Pediatric Emergency Medicine. 6th ed. Philadelphia, PA: Lippincott Williams & Wilkins; 2010:1256-1270"}
{"id" : 905, "question_text" : "You are called to the nursery to see a 6-hour-old, full-term neonate who has just vomited a large amount of clear, yellow fluid. The baby was the 2,500-g product of a full-term, uncomplicated pregnancy and was born to a gravida 2, para 2 mother. You ask the resident on call to pass a nasogastric tube and obtain an abdominal radiograph (Item Q199).\n\nITEM Q199: Abdominal radiograph for the infant in the vignette.\n\nOf the following, this radiographic finding is MOST likely to be associated with", "options" : "[\"annular pancreas\", \"congenital Ladd bands\", \"Peutz\\u2013Jegher syndrome\", \"trisomy 18\", \"trisomy 21\"]", "explanation" : "When bilious emesis is suspected in any patient, especially when bile-tinged fluid appears with the initial episode of vomiting (in contrast to protracted vomiting and retching), management must include assessment for functional or mechanical bowel obstruction. In the vignette, a 6-hour-old infant has vomited a large amount of bilious fluid. The emergency response should be to temporarily relieve obstructive symptoms by passing a nasogastric tube, followed by a diagnostic evaluation. In the newborn, this scenario suggests mechanical obstruction as the consequence of one of the following gut malformations: intestinal malrotation with midgut volvulus, intestinal atresia, or intestinal stenosis. The abdominal radiograph for the infant in the vignette demonstrates the presence of a typical \"double bubble\" sign characteristic of duodenal atresia (Item C199). This anomaly represents the most common form of fetal intestinal atresia, occurring in 1 in 10,000 births. Importantly, duodenal atresia is most commonly encountered in patients who have trisomy 21; such infants account for 25% to 40% of cases. In trisomy 21, the incidence of duodenal atresia has been reported to be as high as 1 in 12 fetuses. Duodenal atresia is not only the most common intestinal atresia but is also the most important cause of complete duodenal obstruction. Although the causative factors are not fully understood, duodenal atresia is thought to arise from a failure of duodenal recanalization between the 9th and 11th weeks of gestation. This is in contrast to jejunal and ileal atresias, which are thought to be related to intrauterine vascular accidents. The postpartum, double bubble radiographic appearance corresponds, respectively, to a gas- and fluid-filled stomach and duodenum proximal to the atresia. Polyhydramnios occurs as the result of impaired absorption of amniotic fluid by the fetal intestine in approximately half of the cases.\n\nFifty percent of cases of duodenal obstruction (stenosis or atresia) occur in the presence of other anomalies, including the following:\n• Intestinal malrotation\n• Esophageal atresia\n• Ectopic or imperforate anus\n• Annular pancreas\n• Biliary atresia\n• Renal anomalies\n• Vertebral anomalies\n\nOther, less frequent diagnoses include partial or complete obstruction caused by a duodenal web, Ladd bands, or a preduodenal portal vein. Another chromosomal disorder, trisomy 18 (Edwards syndrome), may be associated with various gastrointestinal anomalies, including malrotation. In any newborn with suspected duodenal obstruction, midgut volvulus remains the most important differential diagnosis to consider. When present at birth, midgut volvulus typically appears as partial duodenal obstruction on a small bowel radiographic series. However, complete obstruction may also be present, making the distinction between midgut volvulus and duodenal atresia difficult. When duodenal obstruction occurs after the immediate newborn period, a diagnosis of midgut volvulus must be assumed until ruled out.\n\nPREP Pearls\n• Bilious vomiting in the newborn is always a sign of intestinal obstruction\n• Trisomy 21 represents the most common genetic abnormality associated with intestinal atresias\n• In the newborn with Bilious vomiting, the upper GI series to the ligament of Treitz is indicated to rule out malrotation\n\nAmerican Board of Pediatrics Content Specification(s):\n• Know the clinical situations in which duodenal atresia may occur\n\nSuggested Reading:\n• Applebaum H, Lee SL, Puapong DP. Duodenal atresia and stenosis: annular pancreas. In: Grosfeld JL, O'Neill JA, Fonkalsrud E, Coran AG. Pediatric Surgery. Philadelphia, PA: Mosby Elsevier; 2006:1260-1268\n• Choudhry MS, Rahman N, Boyd P, Lakhoo K. Duodenal atresia: associated anomalies, prenatal diagnosis and outcome. Pediatr Surg Int. 2009;25:727-730. doi:10.1007/s00383-009-2406-y\n• Escobar MA, Ladd AP, Grosfeld JL, et al. Duodenal atresia and stenosis: long-term follow-up over 30 years. I Pediatr Surg. 2004;39:867-871. doi:10.1016/j.jpedsurg.2004.02.025\n• Freeman SB, Torfs CP, Romitti PA, et al. Congenital gastrointestinal defects in Down syndrome: a report from the Atlanta and National Down Syndrome Projects. Clin Genet. 2009;75:180-184. doi:10.1111/j.1399- 0004.2008.01110.x\n• Piper HG, Alesbury J, Waterford SD, Zurakowski D, Jaksic T. Intestinal atresias: factors affecting clinical outcomes. J Pediatr Surg. 2008;43:12441248. doi:10.1016/j.jpedsurg.2007.09.053"}
{"id" : 2901, "question_text" : "A 17-year-old adolescent boy is seen for evaluation of lower back pain that has been present for several months. He has no history of trauma or back injury. He reports that his back is stiff and painful when he wakes in the morning and that he has intermittent bilateral hip pain. His symptoms improve over the course of the day. His medical history is significant for plantar fasciitis and anterior uveitis. He appears well and has age-appropriate vital signs. He has tenderness over his sacroiliac joints and limited back flexion and extension. The remainder of his physical examination, including joint and skin examinations, has normal findings. Of the following, the laboratory result MOST commonly associated with the adolescent's condition is the presence of", "options" : "[\"anti\\u2013double-stranded DNA antibody\", \"antinuclear antibody\", \"human leukocyte antigen-B27\", \"rheumatoid factor\"]", "explanation" : "The boy in the vignette has signs and symptoms consistent with enthesitis-related arthritis (ERA), which is most commonly associated with the presence of human leukocyte antigen (HLA)-B27. Juvenile ankylosing spondylitis is a type of ERA, with diagnostic criteria requiring radiologic evidence of bilateral sacroiliitis\n\nAlthough not all children with arthritis satisfy criteria for any specific category (and some meet criteria for more than 1), the International League of Associations for Rheumatology recommends the following categories of juvenile idiopathic arthritis:\n• Systemic arthritis\n• Polyarthritis (rheumatoid factor + and -)\n• Oligoarthritis\n• ERA\n• Psoriatic arthritis\n• Undifferentiated\n\nApproximately 10% to 20% of children with juvenile idiopathic arthritis have ERA, which is defined as:\n• Arthritis with enthesitis or\n• Arthritis and at least 2 of the following:\n  o Presence of or history of sacroiliac joint tenderness and/or inflammatory lumbosacral pain\n  o HLA-B27 antigen\n  o Onset of arthritis in a boy older than 6 years\n  o Anterior uveitis\n  o Positive family history of ankylosing spondylitis, ERA, sacroiliitis with inflammatory bowel disease, reactive arthritis, or acute anterior uveitis in a first-degree relative\n\nEnthesitis-related arthritis occurs more often in males, and the mean age at diagnosis is 12 years. Enthesitis occurs most commonly at the patella or Achilles tendon insertion sites, and arthritis and axial symptoms are typically worse in the morning and responsive to heat and nonsteroidal anti-inflammatory medications. Between 60% and 90% of children with ERA have HLA-B27 antigens. Rheumatoid factor is usually negative, and anti–double-stranded DNA antibodies and antinuclear antibodies are uncommon in patients with ERA.\n\nPREP Pearls\n• Enthesitis-related arthritis is a subtype of juvenile idiopathic arthritis defined as arthritis with enthesitis or arthritis and at least 2 of the following: presence or history of sacroiliac joint tenderness and/or inflammatory lumbosacral pain; human leukocyte antigen-B27 antigen; onset of arthritis in a boy older than 6 years; anterior uveitis; and positive family history of ankylosing spondylitis, enthesitis-related arthritis, sacroiliitis with inflammatory bowel disease, reactive arthritis, or acute anterior uveitis.\n• The majority of children with enthesitis-related arthritis have human leukocyte antigen-B27 antigens.\n• In patients with enthesitis-related arthritis, rheumatoid factor is usually negative, and anti–double-stranded DNA antibodies and antinuclear antibodies are uncommon.\n\nABP Content Specifications(s)\n• Recognize the clinical and laboratory findings associated with ankylosing spondylitis\n\nSuggested Readings\n• Martini A, Ravelli A, Avcin T, et al; fPediatric Rheumatology International Trials Organization (PRINTO). Toward new classification criteria for juvenile idiopathic arthritis: first steps, Pediatric Rheumatology International Trials Organization International Consensus. J Rheumatol. 2019;46(2):190-97. doi: 10.3899/jrheum.180168.\n• Petty RE, Southwood TR, Manners P, et al; International League of Associations for Rheumatology classification of juvenile idiopathic arthritis; second revision, Edmonton, 2001. J Rheumatol. 2004;31(2):390-392. PMID: 14760812.\n• Rosenthal A, Janow G. Enthesitis-related juvenile idiopathic arthritis. Pediatr Rev. 2019;40(5): 256-258. doi: 10.1542/pir.2017-0177.\n• Siegel DM, Gewanter HL, Sahai S. Rheumatologic diseases. In: McInerny TK, Adam HM, Campbell DE, DeWitt TG, Foy JM, Kamat DM. Textbook of Pediatric Care. 2nd ed. Elk Grove Village, IL: American Academy of Pediatrics; 2018:2578-2591. Pediatric Care Online."}
{"id" : 303, "question_text" : "You are seeing a 10-year-old girl for follow-up evaluation of behavior problems and recurrent abdominal pain. Her mother reports that she continues to be irritable most of the time, even with friends, she has problems falling asleep, and she \"talks back\" when pressed to complete tasks such as getting dressed in the morning. She adds that symptoms have been ongoing for a couple of months, stating \"last summer she wasn't like this.\" The girl's teacher reports that she has been quiet and withdrawn in class. The girl's abdominal pain is daily and periumbilical in location. She admits to decreased appetite, denies nausea, and reports one soft bowel movement per day. Physical examination, including neurologic evaluation, shows no findings of note other than periumbilical tenderness to deep palpation. The girl exhibits no guarding, masses, or organomegaly. Of the following, the MOST appropriate next diagnostic step is to", "options" : "[\"collect a stool sample for bacterial culture\", \"complete a Child Depression Inventory\", \"complete a Connors Parent Rating Scale short form\", \"measure her ceruloplasmin\", \"obtain abdominal radiography\"]", "explanation" : "The constellation of symptoms described for the girl in the vignette best represents the presentation of depression in a school-age child. Unlike in adults, who predominantly present with depressed mood, children who have depression often exhibit irritability, anxiety, and psychosomatic symptoms. Other symptoms of depression can include weight gain or loss, fatigue, sleep disturbance, and difficulty concentrating. The most appropriate next step is to administer a screening tool to better characterize her symptoms. The Child Depression Inventory is one of several validated rating scales available for assessing childhood depression.\n\nThe Connors Parent Rating Scale short form is used to aid in the diagnosis of attention-deficit/hyperactivity disorder (ADHD). The short form of this scale focuses only on ADHD symptoms. This girl's symptoms have been present for only 2 months, and patients who have ADHD typically display chronic symptoms starting at a younger age. Furthermore, although ADHD can present with a chief complaint of behavior problems, it is more often characterized by impulsivity and inattention. Abdominal radiography can help characterize constipation, which can contribute to abdominal pain, but this child reports one soft bowel movement per day, making constipation unlikely. Bacterial culture of stool is appropriate for the child who has acute or chronic diarrhea, which this girl does not report. Assessment of ceruloplasmin to detect Wilson disease may be appropriate if the girl had neurologic signs, hepatomegaly, or other stigmata of liver disease.\n\nCritique: The constellation of symptoms described for the girl in the vignette best represents the presentation of depression in a school-age child. Unlike in adults, who predominantly present with depressed mood, children who have depression often exhibit irritability, anxiety, and psychosomatic symptoms. Other symptoms of depression can include weight gain or loss, fatigue, sleep disturbance, and difficulty concentrating. The most appropriate next step is to administer a screening tool to better characterize her symptoms. The Child Depression Inventory is one of several validated rating scales available for assessing childhood depression.\n\nThe Connors Parent Rating Scale short form is used to aid in the diagnosis of attention-deficit/hyperactivity disorder (ADHD). The short form of this scale focuses only on ADHD symptoms. This girl's symptoms have been present for only 2 months, and patients who have ADHD typically display chronic symptoms starting at a younger age. Furthermore, although ADHD can present with a chief complaint of behavior problems, it is more often characterized by impulsivity and inattention. Abdominal radiography can help characterize constipation, which can contribute to abdominal pain, but this child reports one soft bowel movement per day, making constipation unlikely. Bacterial culture of stool is appropriate for the child who has acute or chronic diarrhea, which this girl does not report. Assessment of ceruloplasmin to detect Wilson disease may be appropriate if the girl had neurologic signs, hepatomegaly, or other stigmata of liver disease.\n\nContent Specifications: Recognize that childhood depression can present with predominantly irritable mood rather than predominantly depressed mood"}
{"id" : 2187, "question_text" : "A 2-year-old child is undergoing evaluation for upper respiratory symptoms that began 4 days ago. The child initially had a fever, which resolved 2 days ago. For the past 2 days, the mother noticed discharge from both of the child's eyes on waking up in the morning. The child appears well and is afebrile; the vital signs are within normal limits. The physical examination findings are remarkable only for clear rhinorrhea and bilateral scleral injection without active discharge. The mother asks when the child can return to daycare.", "options" : "[\"after treatment with a topical antibiotic for at least 24 hours\", \"once the rhinorrhea has resolved\", \"once the scleral injection has resolved\", \"24 hours after resolution of fever\"]", "explanation" : "Critique\nThe child in the vignette has a resolving viral infection with viral conjunctivitis. Because there has been no fever for >24 hours, they would be cleared to return to child care. None of the other response options aligns with current recommendations for return to child care.\nExclusion from child care and school programs owing to childhood illnesses results in missed opportunities for learning and socialization with peers for children and missed work days for parents. There is little evidence that, in nonpandemic times, excluding children from these environments prevents the spread of illness; many illnesses are contagious before signs and symptoms occur. Determining when a child should be excluded from child care or school should be based on the risk of illness exposure for other children, the ability of school staff to provide any necessary care, and the child's ability to participate in planned activities.\nCurrent recommendations support allowing well-appearing children with the following conditions to participate in child care and school activities:\nConjunctivitis without fever\nRash without fever\nThrush\nHead lice\nScabies\nChildren with more than 2 episodes of emesis and/or diarrhea that cannot be contained in a diaper should be excluded from child care settings until they are evaluated by a health care professional and/or these symptoms have resolved. Children with oral lesions should be excluded if their symptoms include drooling that cannot be contained. Skin lesions or sores must be covered for a child to remain in child care.\nRecommendations for exclusion from child care or school have been determined for a limited number of specific conditions, which are summarized in the Red Book.\nImmunization and infection control practices are highly effective and essential for preventing the spread of communicable illnesses in day care and school settings. Immunization is the most effective of these strategies. Most states require a minimum level of vaccination for school entry, and some states have disallowed personal belief exemptions, further increasing the percentage of fully vaccinated children within their schools. All day care and school settings should have evidence-based infection control policies and practices in place to help reduce the spread of communicable illnesses among participating children and staff members. Examples include the following:\nHand hygiene policies\nRespiratory etiquette practices\nDiaper-changing procedures\nSurface disinfection\nAdequate ventilation\nPolicies regarding the handling of animals in the classroom (eg, exclusion of reptiles, turtles, amphibians, birds, primates, live poultry, ferrets, or rodents from settings with children younger than 5 years)\nSuggested Reading(s)\nAmerican Academy of Pediatrics. Management and prevention of infectious diseases. In: Kimberlin DW, Banerjee R, Barnett ED, Lynfield R, Sawyer MH, eds. Red Book: 2024-2027 Report of the Committee on Infectious Diseases. 33nd ed. American Academy of Pediatrics; 2024. Accessed September 1, 2024. Red Book Online\nAmerican Academy of Pediatrics. Reduce the risk of infection: practice prevention. In: Shope TR, Hashikawa AN, eds. Managing Infectious Diseases in Child Care and Schools: A Quick Reference Guide. 6th ed. American Academy of Pediatrics; 2023:chap 2\nContent Domain\nInfectious Diseases, Infection Prevention and Control\nLearning Objectives\nUnderstand when a child can return to day care after an episode of illness\nThe correct answer is: 24 hours after resolution of fever\nView Peer Results"}
{"id" : 938, "question_text" : "A 4-year-old girl is seen in the emergency department with a 5-hour history of fever and vomiting. She tells you she \"hurts all over.\" On physical examination, her temperature is 39°C, heart rate is 130 beats/min, respiratory rate is 40 breaths/min, and blood pressure is 90/45 mm Hg. The girl is ill-appearing and tired but answers questions appropriately. Her mucous membranes are dry, but her nares and oropharynx are normal. She is tachycardic but has no murmur, and her capillary refill is 2 to 3 seconds. Examination of the lungs, abdomen, and extremities is unremarkable. You note a rash. The peripheral white blood cell count is 25,000/pL (25.0 x 109/L), with 70% polymorphonuclear neutrophils, 20% lymphocytes, and 10% monocytes. The girl's hemoglobin level is 10 g/dL (100 g/L) and platelet count is 160 x 101/pL (160 x 109/L).\n\nOf the following, the factor MOST closely associated with death caused by this infection is", "options" : "[\"leukocytosis\", \"meningitis\", \"pericarditis\", \"serotype\", \"young age\"]", "explanation" : "Preferred Response: E\nThe ill-appearing girl in the vignette presents with fever, myalgias, rash, tachycardia, tachypnea, hypotension, and delayed capillary refill time concerning for evolving shock. The papular skin lesions and the polymorphonuclear leukocytosis suggest a bacterial infection. Given the rapid onset of illness and lack of focal findings besides the rash, early meningococcemia is likely. Mortality from meningococcemia occurs in 10% of cases and is associated with young age, hypotension, leukopenia, absence of meningitis, thrombocytopenia, and coma. Pericarditis can occur but is not associated with death caused by Neisseria meningitidis. There is no association between serotype and mortality. Invasive infection due to N meningitidis is rapid in onset and characterized by fever, chills, malaise, myalgias, and a rash that initially can be macular, maculopapular, petechial, or purpuric. Many patients are initially thought to have a viral infection. Invasive pneumococcal infection also may present in a clinically similar manner, but its incidence has notably decreased with the widespread use of pneumococcal conjugate vaccine in infants and young children.\n\nCultures of blood and cerebrospinal fluid (CSF) are recommended for patients with suspected invasive meningococcal disease. A Gram stain from cultures of blood or CSF typically reveals gram-negative (pink) diplococci. Empirical therapy with cefotaxime or ceftriaxone is recommended for patients with presumed sepsis due to meningococcemia because coverage for Streptococcus pneumoniae is warranted, pending culture results. Once the diagnosis of meningococcemia is established, intravenous penicillin G (250,000- 300,000 U/ kg per day divided every 4-6 hours) is the drug of choice. Meningococcal isolates with decreased susceptibility to penicillin have been identified in the United States but mostly occur in Spain. Because these isolates remain moderately susceptible to penicillin, penicillin at high doses for 5 to 7 days is adequate for the treatment of invasive meningococcal disease. Prompt treatment of shock with intravenous fluids, and inotropic or ventilatory support when needed, may be critical in improving survival.\n\nPREP Pearls\n• Invasive infection due to N meningitidis is rapid in onset and characterized by fever, chills, malaise, myalgias, and a rash that initially can be macular, maculopapular, petechial, or purpuric.\n• Mortality from meningococcemia occurs in 10% of cases and is associated with young age, hypotension, leukopenia, absence of meningitis, thrombocytopenia, and coma.\n• High-dose intravenous penicillin G for 5 to 7 days is the preferred treatment for infection due to N meningitidis.\n\nAmerican Board of Pediatrics Content Specification(s):\n• Plan the treatment of a Neisseria meningitidis infection\n\nSuggested Reading:\n• American Academy of Pediatrics. Meningococcal infections. In: Pickering LK, Baker CJ, Kimberlin DW, Long SS, eds. Red Book: 2012 Report of the Committee on Infectious Diseases. 29th ed. Elk Grove Village, IL: American Academy of Pediatrics; 2012:500-509\n• Centers for Disease Control and Prevention. Meningococcal disease. www.cdc.gov/meningococcal"}
{"id" : 1372, "question_text" : "You are working in the emergency department when a cyanotic 4-month-old infant arrives by ambulance. She has no significant medical history, and on her physical examination, she looks well other than cyanosis of her mucous membranes. She is not tachypneic and her pulse oximetry is 96% on room air. The cyanosis does not improve with administration of oxygen and you suspect methemoglobinemia. In talking with her mother, you learn that the patient lives in a farmhouse built in 1920, which the parents have been renovating. The baby drinks formula prepared using well water from the farm. Of the following, the MOST likely contaminant leading to this patient's cyanotic episode is", "options" : "[\"arsenic\", \"lead\", \"mercury\", \"nitrates\", \"trichloroethylene\"]", "explanation" : "The infant in this vignette has methemoglobinemia caused by exposure to high levels of nitrates, a chemical often found in well water. Nitrate contamination often results from sewage or use of fertilizer. The nitrates are converted to nitrites in the infant's stomach. Nitrites oxidize the iron in hemoglobin to the ferric (Fe+++) state, forming methemoglobin that binds oxygen poorly. Methemoglobinemia typically presents only with cyanosis. This may first be noted as the percentage of methemoglobin reaches 3%, but symptoms become more obvious as the methemoglobin level reaches 20%. The cyanosis is typically the only symptom, but as the percentage of methemoglobin increases, other symptoms including irritability, tachypnea, and changes in mental status may be noted. In the presence of methemoglobinemia, the pulse oximeter does not give accurate readings, especially at methemoglobin levels above 30%. Poison control or a toxicologist should be consulted if methemoglobinemia is suspected. No treatment is typically required for patients with a percentage of methemoglobin less than 20%, other than identification and removal of the source of nitrates.\n\nApproximately 15% to 20% of homes in the United States obtain their drinking water from wells. Well water is not regulated by the US Environmental Protection Agency and wells are minimally regulated by states. Well water may contain chemical, microbiologic, and radioactive contaminants. The most common contaminants include Cryptosporidium, Escherichia coli, lead, arsenic, nitrates/nitrites, and trichloroethylene/perchloroethylene. Of the contaminants listed, only nitrates are associated with the cyanosis described for the infant in the vignette.\n\nIt is also important to be aware of toxic substances that may contaminate food sources. The most common food contaminants are microbes, especially Salmonella, Campylobacter jejuni, Toxoplasma gondii, Norovirus, Listeria monocytogenes, and E coli 0157:H7. Other contaminants that may be found in food sources include pesticide residues and chemicals that may be found in some fish (including mercury and chlorinated hydrocarbons). High levels of pesticides raise concern for effects on the immune system, endocrine system, and neurodevelopment.\n\nRecently, bisphenol A (BPA) has been noted to be a contaminant of both water and food due to storage in plastic bottles and containers. The BPA contaminant has been associated with early puberty, neurotoxicity, and increased weight gain.\n\nPREP Pearls\n• Well water contains many contaminants including lead, arsenic, nitrates, trichloroethylene/perchloroethylene, Cryptosporidium, and Escherichia coli.\n• The most common food contaminants are microbes, especially Salmonella, Campylobacter jejuni, Toxoplasma gondii, Norovirus, Listeria monocytogenes, and E coli 0157:H7.\n• Bisphenol A is an emerging contaminant of food and water due to storage in plastic containers.\n\nABP Content Specifications(s)\n• Know the toxic substances that may contaminate food sources (eg, mercury, Escherichia coli)\n• Know the contaminants potentially found in drinking water (eg, mercury, Escherichia coli)\n\nSuggested Readings\n• Greer FR, Shannon M, Committee on Nutrition, Committee on Environmental Health. Infant methemoglobinemia: the role of dietary nitrate in food and water. Pediatrics. 2005;116(3):784-786. doi: http://dx.doi.org/10.1542/peds.2005-1497.\n• Karr C. Addressing environmental contaminants in pediatric practice. Pediatr Rev. 2011;32(5):190-200. doi: http://dx.doi.org/10.1542/pir.32-5-190.\n• Rogan WJ, Brady MT, Committee on Environmental Health, Committee on Infectious Diseases. Drinking water from private wells and risks to children. Pediatrics. 2009;123(6):e1123-e1137. doi: http://dx.doi.org/10.1542/peds.2009-0752."}
{"id" : 1575, "question_text" : "A 13-year-old adolescent boy with Duchenne muscular dystrophy, who is cared for in your practice, was recently hospitalized due to newly symptomatic cardiomyopathy. He is currently stable with medical management and frequent cardiology follow-up. Yesterday he told his older sister that he does not want to undergo procedures and blood tests if he is not going to get better. His parents, however, desire that all available medical care be provided. The family seeks guidance regarding adolescents and personal medical decision making.\n\nOf the following, the MOST appropriate recommendation for this family is to", "options" : "[\"apply for a mature minor exception available through most state court systems\", \"delay discussion of end-of-life decisions until the boy's death is imminent\", \"participate in a structured interview to promote family agreement about the boy's medical decisions\", \"permit the boy to assent/dissent for each medical procedure and treatment\", \"permit the boy to have input into his own end-of-life decisions starting at age 16 years\"]", "explanation" : "Correct Answer: C\nShared medical decision making is an important component of patient and family-centered care and a key concept of the medical home. The best approach for this family would be to begin open communication early and to participate in a structured process using available resources and tools to promote family agreement about the boy's medical decisions.\n\nThis approach is supported by evidence from a study of chronically ill adolescents infected with the human immunodeficiency virus (HIV). The HIV-infected adolescents in this study reported a preference for initiating conversations about their wishes earlier, rather than later, in the course of their life-threatening illness. Electronic media-based tools may be particularly useful in helping some children understand and express their preferences. Based on the results of this study, the adolescent in the vignette would likely benefit from a structured or semi structured interview approach to elicit his preferences, inform him and his family about end-of-life decisions, improve communication, and increase agreement among all involved parties regarding medical decisions, particularly end-of-life care.\n\nShared medical decision making relies on the concept of autonomy, a major developmental task of adolescence. Many pediatricians believe that, beginning at age 12 to 13 years, children may be developmentally able to understand the consequences of their medical decisions and so should be involved in making them. There is no uniform age at which all children are ready to participate in their medical decisions, such as 16 years. Rather than using age as the determining factor for involving a child in decision making, it is critical to evaluate each individual child's understanding of the information provided and ability to recognize the consequences of his/her decisions.\n\nAlthough parents or their surrogates are required to give informed permission for medical care, children who are capable should be given the right to assent. However, the limits of assent must be made clear to the child. If a treatment or procedure will proceed despite his/her objection, efforts should be made to help the child to understand what will happen and why, and that the procedure will occur despite his/her objection. It is not practical or even safe to allow a child to overrule each and every medication, blood draw, or medical procedure in the course of treatment. Some ethicists have proposed a model of \"constrained parental autonomy,\" in which parents make decisions, but their decision making is based on respect for the child and his/her perspective.\n\nMost legal standards for consent are based on supporting individual rights of adults, including the right (and obligation) of parents to make decisions for their children. The legal and political support for underage adolescent medical decision making is highly variable. Many states give adolescents the right to consent to care in specialized circumstances, such as the provision of contraception, or the diagnosis and treatment of sexually transmitted infections, drug abuse, and/or mental illness. These rules are based on public health concerns that make this specific medical care a \"compelling state interest.\" In the United States, only 10 states have \"mature minor exceptions,\" granting minors the legal right to consent to general medical care. In states that have a mature minor doctrine, the state often legislates a specific age at which the doctrine can take effect, ranging from 14 to 18 years of age.\n\nEmancipated minors fall under a different regulation, which also varies by jurisdiction. A minor may be considered emancipated, and thus able to provide informed consent for medical care, if he/she is married, in the military, a parent, self-supporting while not living with parents, a high school graduate, or various other criteria.\n\nPREP Pearls\n• The \"mature minor doctrine\" is a developmental concept that many children, often beginning at age 12 to 13 years, may be able to understand the consequences of their medical decisions and so should be involved in making them.\n• The legal standard for consent is based on the right of adults to provide consent for themselves or their children. In the United States, only 10 states have \"mature minor exceptions,\" granting minors the legal right to consent to general medical care.\n• An \"emancipated minor\" has the right to provide informed consent for medical care. The criteria vary by jurisdiction and may include marriage, military service, parenthood, being self-supporting while not living with parents, and high school graduation.\n• Structured interviews and electronic media–based programs may help support communication and understanding between a child and his/her guardian regarding the child's desires about medical care.\n\nABP Content Specifications(s)\n• Understand when it is appropriate to have a minor involved in making decisions about his or her medical care\n\nSuggested Readings\n• American Academy of Pediatrics, Committee on Bioethics. Informed consent, parental permission, and assent in pediatric practice. Pediatrics. 1995;95:314-317.\n• Berlinger N, Barfield R, Fleischman AR. Facing persistent challenges in pediatric decision-making: new Hastings Center guidelines. Pediatrics. 2013;132:789-791. doi: http://dx.doi.org/10.1542/peds.2013-1378.\n• Coleman DL, Rosoff PM. The legal authority of mature minors to consent to general medical treatment. Pediatrics. 2013;131:786-793. doi: http://dx.doi.org/10.1542/peds.2012-2470.\n• Lee KJ, Havens PL, et al. Assent for treatment: clinician knowledge, attitudes, and practice. Pediatrics. 2006;118:723-730. doi: http://dx.doi.org/10.1542/peds.2005-2830.\n• Lyon ME, Garvie PA, McCarter R, Briggs L, He J, D'Angelo LJ. Who will speak for me? improving end-of life-decision-making for adolescents with HIV and their families. Pediatrics. 2009;123:e199-e206. doi: http://dx.doi.org/10.1542/peds.2008-2379."}
{"id" : 1231, "question_text" : "A 10-month-old male infant is brought to the office with 6 days of fever and a new rash. His mother states that he has been difficult to feed for 2 days because he is so irritable. He usually breastfeeds for 20 min every 3 hours, but has only been feeding for 5 min every 2 hours. He has had 2 wet diapers in the last 24 hours. He has not been given any medication. No one else is ill at home. The infant's family is Asian American and he is in day care 3 days a week. Vital signs show a temperature of 39.5°C rectally, respiratory rate of 30 breaths/min, heart rate of 160 beats/min, and a blood pressure of 90/65 mm Hg. Physical examination shows an alert, but very fussy infant. His conjunctiva are injected, but there is no purulent drainage. The lips are red and cracked. There are no mouth ulcers. His tongue is red with white papilla. The neck, chest, and cardiac examinations are unremarkable. The liver edge is at the right coastal margin. No spleen is palpated. His hands and feet have mild edema. There is an erythematous maculopapular rash on his trunk and arms. Laboratory results are as follows: White blood cells, 10,500/µL (10.5 x 109/L) with 65% neutrophils, 25% lymphocytes, 10% atypical lymphocytes, Hemoglobin, 9.5 g/dL (95 g/L), Hematocrit, 30.1%, Erythrocyte sedimentation rate, 60 mm/h, C-reactive protein, 4.5 mg/L, Urinalysis shows white blood cells, Alanine aminotransferase, 50 U/L, aspartate aminotransferase, 45 U/L. The patient is admitted to the hospitalist service. Of the following, the BEST next combination of diagnostic and therapeutic maneuvers is", "options" : "[\"blood culture and intravenous azithromycin\", \"echocardiogram and intravenous immunoglobulin\", \"lumbar puncture and intravenous ceftriaxone\", \"throat culture and intravenous penicillin\", \"urine culture and intravenous ampicillin\"]", "explanation" : "The patient described in this vignette has the symptoms of Kawasaki disease (KD) and is in a high risk demographic group for development of complications. The laboratory data is consistent with KD including pyuria, elevated inflammatory makers, and evidence of hepatic involvement. For the boy in this vignette, the best next step in diagnosis and therapy is to obtain an echocardiogram and treat with intravenous immunoglobulin (IVIG).\nAs the etiology is still not known, there is no definitive diagnostic test available for KD and the diagnosis must be made on clinical grounds with supporting laboratory tests. Kawasaki disease is more common in boys. The major symptom of KD is fever for at least 5 days. Four additional clinical signs and symptoms are needed to make the diagnosis of typical KD, including cervical lymphadenopathy, red and cracked lips or strawberry tongue, nonpurulent conjunctivitis, a polymorphous rash, and redness and edema of hands and feet, with peeling of the fingers and toes in the later part of the illness. If more than 4 of the principal symptoms are present along with fever, the diagnosis may be made before the fifth day of the illness.\nThe differential diagnosis of KD includes scarlet fever secondary to group A streptococcal infection or a viral infection such as adenovirus or enterovirus. In KD, one would not expect to see evidence of purulent pharyngitis. If less than 5 clinical criteria are met, the diagnosis may be especially challenging, and the patient may have atypical or incomplete KD (Item C212A). In this situation, an echocardiogram is helpful in making the diagnosis.\nEchocardiogram is not essential to make the diagnosis of typical KD when there are adequate criteria. The urgency in making the diagnosis of KD is related to the possible development of an inflammatory process that includes the coronary arteries. In KD, there is a 25% risk of development of coronary artery aneurysms (CAA) (Item C212B), which can be decreased to 4% by the administration of IVIG and high dose aspirin (ASA).\nThe highest risk for development of CAA is in the first 2 weeks of the illness, and administration of IVIG is needed within the first 10 days of febrile illness to help prevent that sequelae. Coronary artery (CA) findings are not required to make the diagnosis of KD, although this may be helpful in making a diagnosis in patients with atypical KD. Extreme irritability, sterile pyuria, and thrombocytosis are frequently seen in KD, but are not 1 of the 5 principle findings used for the diagnosis of KD. Prior to the use of IVIG, thrombocytosis with platelet counts up to 1 million/μL were seen and thought to contribute to the damaging effects of coronary artery vasculitis. Patients with persistent fever despite treatment with IVIG are at a higher risk of CAA development. A second dose of IVIG, infliximab, or steroids, as well as continuation of high dose ASA, are among the recommendations in this situation. In less complicated cases where the patient becomes afebrile after initial IVIG administration, the high dose ASA is decreased to low dose ASA.\nRecommendations for further treatment and follow-up depend on the presence or absence of CAA or the less severe finding of CA ectasia. Follow-up at 2 weeks and 6 weeks after discharge is recommended, as a minimum, even if no coronary involvement was seen initially.\nIf CAA have been identified, more intense follow-up will be needed. Cardiac stress testing and angiography may be required in these cases. If the CAA are large, anticoagulation will be needed. Over time, CAA can cause CA stenosis and myocardial ischemia.\nA rapid diagnosis of KD must be made because of the risk of CAA. Diagnosis and treatment of KD with IVIG should not be delayed while waiting for an echocardiogram. Treatment with IVIG will help prevent development of CAA. A very high risk group for development of CAA are younger male children of Asian heritage, such as described in the vignette. With or without CAA, patients require cardiology follow-up.\nFor uncomplicated KD, an echocardiogram is recommended at 2 weeks and again at 6 to 8 weeks. Echocardiograms after this period of time, if initially normal, are not likely to show any new coronary artery abnormalities. Long term planning includes delaying live virus vaccines, such as measles and varicella, for 11 months after treatment with IVIG.\nThe infant in this vignette does not have respiratory symptoms, therefore azithromycin for pneumonia would not be the ideal treatment for him. Irritability would create concern for meningitis, but the other clinical findings would not make that the most likely diagnosis. A urinary tract infection would not cause the combination of findings described. Kawasaki disease does share several similar clinical findings with scarlet fever. The rash in that case is more likely to be \"sand paper\" in appearance. This and the additional findings of pyuria without bacteria in this infant would make a throat culture and penicillin not the next step in this patient.\nPREP Pearls\nKawasaki disease (KD) is a clinical diagnosis that needs to be made as soon as possible to allow initiation of intravenous immunoglobulin treatment and prevent development of coronary artery aneurysms.\nAn echocardiogram is needed to evaluate for coronary artery involvement, but not to make the diagnosis, except in cases that do not meet criteria for typical KD.\nABP Content Specifications(s)\nIdentify cardiac complications associated with Kawasaki disease and how to prevent their occurrence"}
{"id" : 2647, "question_text" : "A 14-year-old adolescent boy is seen for concerns about a worsening chest deformity. He is self-conscious about his appearance and has experienced difficulty exercising. He has no other known health problems. On physical examination, his oxygen saturation in room air is 100% by pulse oximetry. There is a moderately severe depression of his sternum with some molding of the anterior ribs (Item Q139). Breath sounds are equal with no stridor or wheeze; there is good air exchange. Cardiac sounds are displaced slightly to the left. The remainder of the adolescent's physical examination findings are normal. A chest radiograph shows a long, narrow thoracic cavity with the heart slightly displaced to the left on anteriorposterior view and the sternum displaced posteriorly on the lateral view. Complete lung function testing including spirometry and lung volumes is normal for age and height. Of the following, the BEST test to evaluate the impact of this adolescent's condition on his respiratory function is", "options" : "[\"cardiopulmonary exercise testing\", \"computed tomography scan of the chest\", \"echocardiography\", \"a lung ventilation-perfusion scan\"]", "explanation" : "The adolescent in the vignette has a pectus excavatum chest deformity that is causing him concern about his appearance and exercise capacity. The most common chest wall deformity, pectus excavatum often has a significant negative impact on body image and perceived exercise capacity. Static pulmonary function testing results are rarely abnormal, as is the case for the boy in the vignette. Cardiopulmonary exercise testing may uncover subtle deficits in exercise capacity and is the best test to further evaluate his respiratory function.\n\nSurgical repair of pectus excavatum has been shown to increase lung volumes, air flow, and exercise capacity to within the normal range on pulmonary function and cardiopulmonary exercise testing, but the greatest improvement is in body image perception and subjective assessment of exercise performance.\n\nThe degree of pectus excavatum can be quantitated on computed tomography (CT) of the chest using the Haller index: the ratio of the internal transverse rib-to-rib diameter to the distance between the spine and sternum on a cross-sectional CT view (Item C139). An index of 2.5 or less is normal; the higher the index, the worse the pectus deformity. However, the Haller index is a poor biomarker of functional limitation; there is no direct correlation between severity as determined by Haller index and any pulmonary function abnormalities.\n\nMore severe pectus deformities can cause displacement of the heart to the left and cardiac conduction abnormalities. Exercise intolerance may have both cardiac and pulmonary components. While echocardiography is indicated in the evaluation of exercise intolerance in children and adolescents with pectus excavatum, this test will not define any functional respiratory deficits caused by the chest wall deformity.\n\nA lung ventilation-perfusion scan is not indicated for the adolescent in the vignette. Chest wall deformities do not cause ventilation-perfusion mismatch.\n\nThe second most common chest wall abnormality is scoliosis, which has many etiologies. Idiopathic scoliosis is rarely associated with objective pulmonary function changes until it is very severe. A restrictive pulmonary physiology is expected in any individual with scoliosis, with loss of lung volume on the side of the concavity and a hyperexpanded lung associated with stretched and poorly compliant intercostal muscles on the convex side. A rotational component and/or kyphosis worsens chest wall compliance and function, further restricting lung volume and chest wall expansion with respiration. When scoliosis is secondary to neuromuscular conditions, the underlying poor function of the intercostal muscles and diaphragm compounds the restrictive pulmonary physiology. There is a direct relationship between the severity and complexity of scoliosis and restrictive pulmonary physiology. Surgical management of severe or progressive scoliosis can stabilize lung function and prevent further decline; however, it is rarely associated with improvement in lung function even when the chest wall abnormalities are corrected.\n\nEarly severe scoliosis is associated with structural abnormalities of the spine (eg, hemivertebrae, bar vertebrae) with or without rib fusions or absent ribs. Affected children may have life-threatening pulmonary restriction from birth and require ventilator support until intervention can be provided to stabilize and expand the chest wall. This constellation of anatomic anomalies and severe restrictive pulmonary physiology (thoracic insufficiency syndrome) requires surgical intervention to preserve linear growth. If linear growth is not preserved, pulmonary restriction will worsen as the child ages. Placement of expanding rods in the spine for chest/rib stabilization promotes ongoing skeletal growth through interval extension.\n\nPREP Pearls\n• Severe chest wall deformities can cause restrictive pulmonary physiology. The severity of the restrictive lung disease is directly related to the complexity of the chest deformity.\n• Pectus excavatum can have a significant negative impact on self-image and perceived exercise capacity; static pulmonary function testing is usually normal.\n• Surgical repair of scoliosis is not likely to improve lung function but can prevent it from worsening.\n\nABP Content Specifications(s)\n• Recognize the association of thoracic deformities with restrictive pulmonary disease\n\nSuggested Readings\n• Alapati D, Shaffer TH. Skeletal dysplasia: respiratory management during infancy. Respir Med. 2017;131:18-26. doi:10.1016/j.rmed.2017.07.063 .\n• Kelly RE Jr, Cash TF, Shamburger RC, et al. Surgical repair of pectus excavatum markedly improves body image and perceived ability for physical activity: multicenter study. Pediatrics. 2008;122(6):1218-1222. doi:10.1542/peds.20072723 .\n• Mayer OH, Allen JL. Chest wall and spinal deformities. In: Light MJ, ed. Pediatric Pulmonology. American Academy of Pediatrics; 2011:309-345.\n• Mino J, Stallion A, Monteiro R. Pectus excavatum and pectus carinatum. In: McInerny TK, Adam HM, Campbell DE, DeWitt TG, Foy JM, Kamat DM, eds. American Academy of Pediatrics Textbook of Pediatric Care. American Academy of Pediatrics; 2021:chap 309. Accessed September 1, 2022. Pediatric Care Online.\n• Redding GJ. Clinical issues for pediatric pulmonologists managing children with thoracic insufficiency syndrome. Front Pediatr. 2020;8:392. doi:10.3389/fped.2020.00392.\n• Yang S, Andras L, Redding G, Skaggs D. Early-onset scoliosis: a review of history, current treatment and future directions. Pediatrics. 2016;137(1). doi:10.1542/peds.2015-0709."}
{"id" : 3169, "question_text" : "You are assessing a newborn with hypoglycemia 8 hours after birth. She was born at 37 weeks' gestation to a 40-year old primigravida woman whose pregnancy was notable only for the use of assisted reproductive technology. The newborn required admission to the special care nursery shortly after birth for intravenous glucose therapy to maintain her blood sugar values over 45 mg/dL (2.5 mmol/L). Your physical examination reveals a large for gestational age newborn with an abdominal wall defect. Of the following, the additional clinical finding this patient is MOST likely to have is", "options" : "[\"anorectal malformation\", \"hypospadias\", \"large tongue\", \"patent urachus\", \"single umbilical artery\"]", "explanation" : "Preferred Response: C\nThe newborn in the vignette has the clinical findings of Beckwith-Wiedemann syndrome (BWS), which is associated with macroglossia (large tongue). Beckwith-Wiedemann syndrome is related to alterations on chromosome 11p15.5, with a familial transmission rate of 15%. There is an increased incidence seen in pregnancies requiring assisted reproductive technology. Beckwith-Wiedemann syndrome is an overgrowth syndrome with affected infants often being large for gestational age at birth. Other manifestations of somatic overgrowth include macroglossia, visceromegaly, and hemihypertrophy. Islet cell hyperplasia leads to the hypoglycemia seen in up to 50% of affected infants. Abdominal-intestinal wall defects including omphalocele, umbilical hernia, and diastasis recti are commonly seen with BWS.\n\nAn omphalocele is an abdominal-intestinal wall defect formed by the protrusion of bowel into the base of the umbilical cord, whereas a gastroschisis is formed by loops of bowel extending through a defect in the abdominal wall to the right of the umbilical cord. Both require immediate surgical evaluation and management in the neonatal period. The initial management in the delivery room includes covering the exposed bowel with saline-soaked sterile dressings, inserting a gastric tube to decompress the bowel, and placing the newborn in a bowel bag to the level of the axilla to minimize fluid and heat losses. Parenteral fluid resuscitation is often needed because of increased insensible losses associated with the defects.\n\nThe type of abdominal-intestinal wall defect guides further evaluation. Gastroschisis is an isolated defect seen in infants with intrauterine growth restriction: approximately 10% will have coexisting intestinal atresia or stenosis. Omphalocele is associated with other anomalies in up to 70% of cases. Beckwith--Wiedemann syndrome may be seen in 10% of affected infants, whereas karyotype anomalies, including trisomy 13, 18, and 21, may be found in up to 30% of cases. Echocardiography should be performed in any infant with an omphalocele because of the 50% risk of congenital heart disease.\n\nAnorectal malformations, hypospadias, patent urachus, and single umbilical artery are not associated with BWS. They are unlikely to be found in the newborn in the vignette\n\nPREP Pearls\n• An omphalocele is an abdominal wall defect formed by the protrusion of bowel into the base of the umbilical cord, whereas a gastroschisis is formed by loops of bowel extending through a defect in the abdominal wall to the right of the umbilical cord.\n• The initial management of an abdominal-intestinal wall defect in the delivery room includes covering the exposed bowel with saline-soaked sterile dressings, inserting a gastric tube to decompress the bowel, and placing the newborn in a bowel bag to the level of the axilla to minimize fluid and heat losses.\n\nAmerican Board of Pediatrics Content Specification(s)\n• Plan the appropriate evaluation and management of a newborn infant who has abdominal-intestinal wall defect\n\nSuggested Reading\n• Chabra S. Management of gastroschisis: prenatal, perinatal, and neonatal. NeoReviews. 2006;7(8):e419-e427. doi:10.1542/neo.7-8-e419.\n• Hwang P, Kousseff BG. Omphalocele and gastroschisis: an 18-year review study. Genet Med. 2004;6(4):232-236. do1:10.109701. G1M.0000133919.68912.A3.\n• Kastenberg Z1, Dutta S. Ventral abdominal wall defects. NeoReviews. 2013;14(8):e402-e411. doi:10.1542/nen.14-8-e402.\n• Weksberg 12, Shuman C. Beckwith M. Beckwith-Wiedemann syndrome. Eu r J Hum Genet. 2010;18:8-14. doi:10.1038/ejhg.2009.106."}
{"id" : 2945, "question_text" : "A 2-hour-old male neonate is being seen in the well-child nursery for feeding difficulties. He was born via normal spontaneous vaginal delivery at 40 weeks' gestation to a primigravida mother who has been healthy and had an uncomplicated pregnancy. His mother is reporting difficulty with latching and notes that his tongue seems to be anchored to the bottom of his mouth.\n\nOf the following, the BEST management approach for this infant-mother dyad is to", "options" : "[\"offer lactation support\", \"perform a frenulotomy\", \"provide a breast pump\", \"provide donor breast milk\"]", "explanation" : "The infant-mother dyad in the vignette should be offered lactation support to enforce good breastfeeding habits as early as possible. Performing a frenulotomy is inadvisable at this time because other noninvasive techniques have not been attempted. Providing a breast pump or donor milk are also inappropriate; the infant should be given the opportunity to breastfeed directly from his mother's breast as often as possible.\n\nThe terms \"ankyloglossia\" and \"tongue-tie\" most commonly refer to a short anterior lingual frenulum that limits tongue movement. Prevalence rates range from 4% to 10%, depending on the definition used.\n\nVery little evidence exists to inform patients and medical providers as to whether or not ankyloglossia in healthy children should be corrected at any age. This is especially true in older children, for whom speech problems or social problems such as licking ice cream must be considered. Most available studies are focused on breastfeeding outcomes, but the quality of these studies are at best mediocre. Any decision to perform a frenulotomy in a healthy neonate or infant should be made in conjunction with the patient's parents, primary care physician, lactation consultant, and other specialists. The procedure should be performed by a trained provider; simple frenulotomies are preferred over laser therapy or more complex interventions.\n\nRelease of posterior tongue-tie or lip tie in healthy infants have also become popular. Evidence regarding benefits are even more lacking. Open and nonjudgmental communication between the parent(s) and provider is key in all such discussions.\n\nPREP Pearls\n• For feeding problems in healthy breastfeeding infant-mother dyads, lactation support should be offered before any other intervention.\n• Evidence for procedural correction of shortened oral frenulum in healthy neonates, infants, and children is lacking; the decision to perform such a procedure should be made with open communication among the patient's parents, primary care physician, and specialists.\n\nABP Content Specifications(s)\n• Plan the most appropriate management of a short lingual frenulum\n\nSuggested Readings\n• Bunik M. The pediatrician's role in encouraging exclusive breastfeeding. Pediatr Rev. 2017;38(8):353-368. doi: 10.1542/pir.2016-0109.\n• Chinnadurai S, Francis DO, Epstein RA, Morad A, Kohanim S, McPheeters M. Treatment of ankyloglossia for reasons other than breastfeeding: a systematic review. Pediatrics. 2015;135(6):e1467-e1474. doi: 10.1542/peds.2015-0660.\n• Francis DO, Krishnaswami S, McPheeters M. Treatment of ankyloglossia and breastfeeding outcomes: a systematic review. Pediatrics. 2015;135(6):1458-e1466. doi: 10.1542/peds.2015-0658.\n• Younger Meek J, Hatcher AJ; Section on Breastfeeding. The breastfeeding-friendly pediatric office practice. Pediatrics. 2017;139(5):e20170647. doi: 10.1542/peds.2017-0647."}
{"id" : 3327, "question_text" : "A 1-week-old patient is brought to the pediatric clinic by her mother for concerns that, since birth, the newborn is breathing too fast and intermittently stops breathing. The mother states that her newborn does not seem to be in any distress between the episodes of fast breathing. These periods last as long as 5 seconds, at which point she will resume breathing spontaneously at a rapid rate and then slow to a more normal rate. These episodes occur more often when she is asleep and are not associated with any color change. The newborn is a product of a full-term, uncomplicated pregnancy and delivery. She went home with the mother on day 2 after birth. She takes a 3-oz bottle of formula every 3 hours and usually makes 4 wet and 2 dirty diapers every day. She does not have any vomiting. Her growth parameters are normal. Vital signs show a temperature of 37°C, heart rate of 140 beats/min, respiratory rate of 45 breaths/min, and blood pressure of 80/40 mm Hg. Pulse oximetry shows oxygen saturation of 100% on room air. Physical examination shows a well-developed, well-nourished baby. Anterior fontanelle is open, soft, and flat. Pupils are equal and reactive. The newborn has moist and nonerythematous eye, oral, and nasal mucous membranes. Cardiovascular examination reveals a regular rate and rhythm and a soft, grade 1/6 systolic ejection murmur. She has warm and well-perfused extremities, with strong and equal femoral pulses. She is breathing comfortably at a timed rate of 45 breaths/min. She has no notable thoracic deformities. During inspiration, her chest moves in, while her abdomen protrudes. Lungs are clear to auscultation with good air entry bilaterally. Abdomen is soft, non-tender, and nondistended with no enlarged organs. Of the following, the MOST appropriate course of action is", "options" : "[\"admit to hospital for observation\", \"obtain capillary blood gas\", \"obtain chest radiograph\", \"reassure the mother\", \"refer to pediatric cardiologist\"]", "explanation" : "The newborn in the vignette is exhibiting periodic breathing, which is a normal respiratory pattern in healthy full-term newborns in the first month after birth and the MOST appropriate course of action is to reassure the mother. Periodic breathing is characterized by normal rhythmic breathing interrupted by brief (< 10 seconds) intermittent periods of respiratory pause, alternating with fast breathing. These episodes, which resolve in the first few months after birth, are not associated with distress or altered gas exchange, and thus require no further evaluation or treatment.\n\nRegulation of respiration is a complex process involving central respiratory controllers, sensors, receptors, and effectors. Voluntary control of respiration, which requires some level of consciousness, originates in the cerebral motor cortex. Subconscious control of respiration occurs in the pre-Botzinger complex (preBotC) in the brainstem, and controls the automaticity of breathing much in the same way as does the pacemaker of the heart. This group of neurons is also responsible for various patterns of breathing, including sighing and gasping. Other nuclei in the pons, including the apneustic center and the pneumotaxic center, are responsible for regulating the preBotC. Derangements in the fine tuning of these mechanisms of respiratory regulation can result in (A) Cheyne-Stokes respiration, which are periods of apnea alternating with hyperventilation, (B) apneustic breathing, characterized by prolonged inspiratory gasps, or (C) generally shallow respirations and hyperventilation, as is seen with global central nervous system dysfunction. The stimulus to breathe is also dependent on central chemoreceptors, which respond with exquisite sensitivity to changes in cerebrospinal pH, and peripheral chemoreceptors in the carotid bodies, which respond to changes in the arterial pressure of oxygen (PaO2). These receptors lead to an increase in respiratory stimuli in metabolic acidosis and hypoxia, and prevent hypoventilation. Lastly, stretch receptors in the lungs feed back to the controllers in the brain to breathe more shallowly and rapidly in conditions of decreased compliance, such as pulmonary edema, interstitial lung diseases, and pneumonia.\n\nThe clinical sign of tachypnea carries a wide differential diagnosis, and depends greatly on age, premorbid conditions, and scenario. Tachypnea has both pulmonary and nonpulmonary causes. Cardiac disease in children commonly presents with respiratory signs and symptoms. Left ventricular failure and resulting left atrial hypertension and dilation can lead to pulmonary edema and increased alveolar and interstitial lung water. This leads to decreased lung compliance and rapid shallow breathing, and sometimes \"cardiac wheezing\" caused by the extrinsic compression of small airways by interstitial edema. Metabolic acidosis from decreased cardiac output can decrease the pH of the cerebrospinal fluid, which causes further tachypnea. If a cardiac cause of tachypnea is suspected, the evaluation can include chest radiography, echocardiography, or blood gas measurement where indicated. Metabolic acidosis from other causes, for example diabetic ketoacidosis or severe diarrhea, can lead to so-called Kussmaul breathing, which is rapid and deep.\n\nEarly manifestations of sepsis and other inflammatory conditions include tachycardia and tachypnea. Other toxic, metabolic, and CNS conditions can also cause tachypnea, either through metabolic acidosis or derangement of regulation of respiration, such as aspirin ingestion, hyperammonemia, liver disease, or global CNS dysfunction. These conditions can be investigated with measurement of electrolytes, ammonia level, and liver function, or neuroimaging where indicated. Pulmonary conditions of decreased lung compliance, such as pneumonia, alveolar hemorrhage, pulmonary edema, interstitial fibrosis, and acute respiratory distress syndrome, usually cause rapid shallow breathing. Plain radiography can be performed to elucidate lung pathology in these conditions. Lastly, tachypnea is an early sign of pulmonary embolism, which can be a life-threatening condition. In older children and others with risk factors for thrombosis, a high index of suspicion must be maintained. Rapid sequence computed tomography with contrast is the best initial test if pulmonary embolism is suspected.\n\nThe newborn in this vignette has periodic breathing, as opposed to one of the aforementioned disorders because she was born at full term, and is otherwise healthy and neurologically normal. In addition, the breathing is not associated with distress or any change in color that would indicate abnormal gas exchange. A soft-grade 1/6 systolic ejection murmur is likely normal in the newborn period, as is the breathing pattern of the chest moving in while the abdomen protrudes. Thus, pulmonary conditions such as pneumonia, pulmonary hemorrhage, or pulmonary edema are not likely, so chest radiography or a capillary blood gas measurement is not indicated. Cardiac conditions are not likely because the extremities are well perfused and pulses are equal, so neither observation in the hospital nor referral to a cardiologist is warranted. The complicated mechanisms of respiratory regulation are not yet mature in normal, full-term newborns, and asymptomatic periodic breathing does not require evaluation or treatment.\n\nPREP Pearls\n• Periodic breathing occurs in normal newborns, and consists of periods of respiratory pause lasting 10 seconds or less, followed by episodes of tachypnea.\n• Tachypnea can be caused by pulmonary conditions or nonpulmonary conditions, such as central nervous system, cardiac, inflammatory, or metabolic conditions.\n• Cardiac disease in children often manifests with respiratory signs and symptoms.\n\nAmerican Board of Pediatrics Content Specification(s)\n• Recognize normal breathing patterns in patients of various ages\n• Recognize the various factors that influence respiratory rate\n• Plan the appropriate clinical and diagnostic evaluation of tachypnea of various etiologies\n\nSuggested Reading\n• Bloomfield D. Tachypnea. Pediatr Rev. 2002;23(8):294-295. doi:10.1542/ pir.23-8-294.\n• Carlo WA. Respiratory tract disorders. In: Kliegman RM, Stanton BF, St. Geme JW III, Schor NF, Behrman RE, eds. Nelson's Textbook of Pediatrics. 19th ed. Philadelphia, PA: Saunders Elsevier; 2011:579-580_\n• Carroll JL, Marcus CL, Loughlin GM. Disordered control of breathing in infants and children. Pediatr Rev. 1993;14(2):51-65. doi:10.1542/pir.14-2-51.\n• Sarnaik AP, Heidemann SM. Regulation of respiration. In: Kliegman RM, Stanton BF, St. Geme JW III, Schor NF, Behrman RE, eds. Nelson's Textbook of Pediatrics. 19th ed. Philadelphia, PA: Saunders Elsevier; 2011:1421."}
{"id" : 3006, "question_text" : "A 9-year-old boy is seen in the office for evaluation of right eye swelling of 3 weeks' duration. He reports a painless bump on his right upper eyelid. He reports no fever, eye redness, discharge, or vision changes. He has a 7-mm, firm, nonmobile nodule on his right upper eyelid without overlying erythema or discharge (Item Q209). His conjunctiva are clear, and extraocular movements are intact. The red reflex is present and symmetrical bilaterally. Of the following, the NEXT best step in management is", "options" : "[\"referral to an ophthalmologist\", \"use of topical antibiotics\", \"use of topical glucocorticoids\", \"warm compresses\"]", "explanation" : "The boy in this vignette has a chalazion of the right upper eyelid. A chalazion results from an obstruction and inflammation of the meibomian glands of the upper or lower eyelid, causing a lipogranuloma. Clinically, it presents as a firm, painless, nonmobile, rubbery nodule in the lower or upper eyelid, which varies in size between 3 and 10 mm. The skin overlying a chalazion has minimal, if any, inflammatory changes. Larger lesions tend to be chronic and slow-growing, lasting for several weeks to months. The primary treatment for a chalazion is application of warm compresses to the eyelid multiple times per day. Instructions should include the avoidance of microwaving, boiling, or placing compresses in the oven to heat, given the association of these measures with burns.\n\nBecause chalazia are not caused by an infectious process, neither topical nor oral antibiotics are indicated unless there is a secondary infection present, such as blepharitis or cellulitis. A secondary infection will typically present with the onset of pain, erythema, edema, tenderness, and possibly even fever. Most chalazia resolve spontaneously with the use of warm compresses. Referral to an ophthalmologist should be considered for lesions that fail to resolve after 4 to 6 weeks of conservative management or for recurrent lesions. Excision may be necessary if the chalazion distorts vision or leads to a cosmetic issue. Glucocorticoid injections, not topical therapy, may be a treatment initiated by an ophthalmologist for lesions that fail to resolve spontaneously.\n\nIn contrast to a chalazion, an external hordeolum (sty) represents an acute infectious process of the glands of the eyelid (the meibomian glands or the glands of Zeis or Moll) that are associated with hair follicles. An internal hordeolum results from an infection of the meibomian gland, the large sebaceous gland with an opening at the lid margin. The typical infectious agent is Staphylococcus aureus. Clinically, a sty presents as a tender, edematous, and erythematous abscess of the eyelid. Internal hordeola tend to have more diffuse edema just under the conjunctival side of the eyelid. Most hordeola resolve without intervention over several days. The primary management for a hordeolum consists of warm compresses to help relieve the obstruction and promote drainage. Use of eye makeup should be avoided. A topical antibiotic ointment may be applied if significant blepharitis is present, but there is little evidence that topical antibiotics result in rapid healing. Surgical excision and drainage by an ophthalmologist may be required for lesions that fail to decrease in size within 1 to 2 weeks.\n\nPREP Pearls\n• A chalazion is a painless lipogranuloma that tends to be chronic in nature, lasting weeks to months, whereas a hordeolum (sty) is an acute infectious process.\n• The primary treatment for a chalazion is application of warm compresses to the eyelid multiple times per day.\n\nABP Content Specifications(s)\n• Plan the appropriate management of a stye\n• Plan the appropriate management of a chalazion\n• Differentiate the clinical findings associated with a stye from those of a chalazion\n\nSuggested Readings\n• Aguilera ZP. Chen PL. Eye pain in children. Pediatr Rev. 2016;37(10):418-425. doi:10.1542/pir.2015-0096.\n• Lederman C, Miller M. Hordeola and chalazia. Pediatr Rev. 1999;20(8):283-284. doi:10.1542/pir.20-8-283.\n• Otlisky SE, Hug D, Plummer LS, Stahl ED, Ariss MM, Lindquist TP. Abnormalities of the lids. In: Kliegman RM, Stanton BF, St Geme JW III, Schor NF, Behrman RE, eds. Nelson Textbook of Pediatrics. 20th ed. Philadelphia, PA: Saunders Elsevier; 2016:3033-3034.\n• Rosales T. Infections of the eye. In: Berkowitz, CD, ed. Berkowitz's Pediatrics: A Primary Care Approach. 5th ed. Elk Grove Village, IL: American Academy of Pediatrics; 2014:495-500.\n• Wald ER. Periorbital and orbital infections. Pediatr Rev. 2004;25(9):312-320. doi:10.1542/pir.25-9-312."}
{"id" : 3540, "question_text" : "What was the primary contribution of Edward Frank's 1964 publication on sepsis management?", "options" : "[\"He published management strategies including continuous monitoring of hemodynamics, cardiac output, urine output, electrolytes, and pH in addition to antibiotics\", \"He first identified the causative organisms of sepsis\", \"He developed the first antibiotic effective against gram-negative bacteria\", \"He established the first sepsis screening protocol in emergency departments\"]", "explanation" : "The text states that Edward Frank, a surgeon from Boston, published management strategies for sepsis in 1964 that included continuous monitoring of blood pressure, cardiac output, urine output, correct electrolytes and pH in addition to antibiotics."}
{"id" : 2269, "question_text" : "A 14-year-old girl is seen in the emergency department for abdominal pain. She has had cycles of abdominal and back pain over the past few months, but her pain has significantly worsened this week. She reports that she is not sexually active, and that she has not yet started having menstrual cycles. She appears uncomfortable and is afebrile with a heart rate of 120 beats/min, a respiratory rate of 18 breaths/min, and a blood pressure of 120/80 mm Hg. Physical examination reveals a palpable lower abdominal mass and a breast and pubic hair sexual maturity rating of 4. The remainder of her examination findings are normal. The result of a urine β-human chorionic gonadotropin test is negative. Of the following, the MOST likely cause of the girl's abdominal mass is", "options" : "[\"ectopic pregnancy\", \"hydrometrocolpos\", \"stool retention\", \"suprarenal tumor\"]", "explanation" : "An imperforate hymen can be diagnosed in the newborn period by examination of the external genitalia, which will show a thin bulging membrane across the vaginal opening.\nThe diagnosis of imperforate hymen is typically delayed until puberty when girls with a sexual maturity rating of 3 to 5 experience abdominal pain and primary amenorrhea and are found to have a membrane obstructing the vaginal opening.\nUnrecognized hydrometrocolpos may lead to constipation, urinary frequency, and rarely, renal failure secondary to urinary retention.\nCritique\nThe adolescent in the vignette has chronic abdominal pain that is worsening and a sexual maturity rating that is inconsistent with her premenarchal status. Imperforate hymen with hydrometrocolpos—a collection of retained fluid or menstrual blood resulting from obstruction of the vaginal outflow tract—is the most likely cause of this girl's abdominal mass. Ectopic pregnancy has a more acute presentation; it would not present with a several-month history of abdominal pain with a lower abdominal mass, and the β-human chorionic gonadotropin result would be positive. Although this adolescent's symptoms could also be seen with chronic constipation, the constellation of sexual maturity stage 4, primary amenorrhea, and cyclic abdominal pain makes imperforate hymen a more likely diagnosis. A suprarenal tumor large enough to present as a mass would be palpable in the upper quadrants of the abdomen, rather than in the lower abdomen or pelvic regions.\nChildren and adolescents with imperforate hymen will develop hydrometrocolpos. The incidence of imperforate hymen is in up to 0.1% of term neonates. It generally occurs sporadically, with no known associated genetic markers, and is usually not associated with other anomalies.\nAn imperforate hymen can be diagnosed in the newborn period through close examination of the external genitalia, which will show a thin bulging membrane across the vaginal opening. The bulging is due to a collection of vaginal secretions stimulated by maternal estrogen. The diagnosis is typically delayed until puberty when girls with a sexual maturity rating of 3 to 5 experience primary amenorrhea and abdominal pain. Affected individuals may experience months of cyclic abdominal pain, along with back pain, constipation, and urinary complaints. A delayed diagnosis can lead to renal failure secondary to urinary retention resulting from obstruction caused by hydrometrocolpos. Physical examination in the adolescent with imperforate hymen will reveal an abdominal or pelvic mass and mature sexual development. An external genital examination is crucial to this diagnosis. The vaginal opening will appear as a bulging membrane with a bluish discoloration (Figure).\nAbdominal ultrasonography can confirm the diagnosis. A fluid collection will be visualized in the vaginal canal and uterus. Magnetic resonance imaging is required to accurately define the reproductive tract anatomy and determine the level and thickness of the obstructing membrane. Once the genitourinary tract anatomy has been clearly defined, treatment for imperforate hymen involves a surgical hymenectomy performed in an operating room.\nContent Domain\nReproductive Health\nLearning Objectives\nIdentify clinical findings associated with an imperforate hymen\nPreform test to confirm a diagnosis of an imperforate hymen"}
{"id" : 1228, "question_text" : "A 1-month-old male infant is brought to your office by his mother for his first health supervision visit. Pregnancy, labor, and delivery were uncomplicated. He was born appropriate for gestational age at 39 weeks of gestation and was discharged at 3 days of age. He stayed 1 extra day in the hospital for mild jaundice and delayed passage of his first stool. He is primarily breastfed and has been gaining weight along the 25th percentile for his age. He passes stools every 3 days and his mother thinks he may have constipation, but states that another pediatrician assured her that this was normal for breastfed babies. The mother denies any significant family history, though you note that she has iris heterochromia. On physical examination, the infant has mild abdominal fullness, but no organomegaly or palpable masses. On digital rectal examination, the anal canal is tight and a small amount of stool squirts out as you withdraw your finger from the anus. The remainder of his physical examination is unremarkable. Of the following, the BEST test to confirm the suspected diagnosis for this infant is", "options" : "[\"celiac panel\", \"immunoreactive trypsinogen level\", \"rectal suction biopsy\", \"sweat chloride test\", \"thyroid panel\"]", "explanation" : "The newborn in the vignette has the characteristic history and physical examination findings of congenital aganglionic megacolon or Hirschsprung disease. Although findings on abdominal radiography, contrast enema, or anorectal manometry support the diagnosis, rectal suction biopsy is the diagnostic gold standard for Hirschsprung disease. The history and physical examination findings that suggest the diagnosis in this case include delayed passage of meconium stool, decreased frequency of stools during the first month after birth, mild abdominal fullness, and the \"squirt sign\" on digital rectal examination. In addition, the patient's mother has heterochromia of her irises, which should lead the practitioner to consider the association of Hirschsprung disease and Waardenburg syndrome.\nDelayed passage of meconium, defined as beyond 48 hours after birth, can be indicative of a serious problem such as bowel atresia or obstruction, imperforate anus, meconium plug, or Hirschsprung disease. It is important for pediatric health care providers to recognize the potential significance of this delay. Neonates who pass their first meconium stool after 48 hours but before 72 hours, and are otherwise well appearing, should undergo a thorough physical examination. The conditions that may be commonly associated with delayed passage of meconium should be carefully considered and excluded. These infants should be followed closely and evaluated promptly if they develop symptoms of abdominal distention, bilious vomiting, or constipation. Any neonate who fails to pass meconium within the first 72 hours after birth should be evaluated for Hirschsprung disease.\nHirschsprung disease is a motility disorder caused by the absence of parasympathetic ganglion cells, because of the failure of neural crest cells to migrate completely during intestinal development. Hirschsprung disease is more prevalent in several genetic syndromes, including Down syndrome, Bardet-Biedl syndrome, multiple endocrine neoplasia type 2, Smith-Lemli-Opitz, and Waardenburg syndrome. Type IV Waardenburg syndrome (also known as Waardenburg-Shah syndrome) has signs and symptoms of both Waardenburg syndrome and Hirschsprung disease. Mutations in the SOX10,EDN3, or EDNRB genes cause type IV Waardenburg syndrome. These genes are important for the development of nerve cells in the large intestine in addition to melanocyte development. Mutation in any of these genes results in hearing loss; changes in the pigmentation of skin, hair, and eyes; and intestinal problems related to Hirschsprung disease. Often there is an autosomal dominant pattern of inheritance, but an autosomal recessive pattern may occur as well.\nCeliac disease or gluten-sensitive enteropathy typically presents in infants between 6 and 24 months of age, after the introduction of gluten into the diet. The signs and symptoms suggestive of gluten sensitivity are chronic diarrhea (or rarely, constipation), anorexia, abdominal distention, chronic abdominal pain, and failure to thrive.\nMeconium ileus in the newborn is almost always caused by cystic fibrosis. These infants fail to pass meconium stools, and often have marked abdominal distention with bilious emesis soon after birth. Occasionally, infants with cystic fibrosis may have lesser degrees of meconium impaction and a presentation that is more benign. The diagnosis of cystic fibrosis is made through immunoreactive trypsinogen levels on the newborn screening test or sweat chloride levels in the older infant or child.\nCongenital hypothyroidism should be considered in infants who present with prolonged jaundice and constipation. However, these infants will have additional signs and symptoms, which the infant in this vignette did not have, such as lethargy, hypothermia, feeding problems, poor weight gain, macroglossia, umbilical hernia, large fontanels, hypotonia, and dry skin.\nPREP Pearls\nPassage of meconium occurring beyond the first 48 hours after birth is considered delayed.\nDelayed or absent passage of meconium can indicate a serious problem, such as bowel atresia or obstruction, imperforate anus, meconium plug, or Hirschsprung disease.\nNeonates who pass their first meconium stool between 48 and 72 hours after birth, but are otherwise well-appearing, should undergo a thorough physical examination, with close follow-up and further evaluation performed as indicated.\nAny neonate who fails to pass meconium within the first 72 hours after birth should be evaluated for Hirschsprung disease.\nABP Content Specifications(s)\nRecognize disorders associated with delayed or absent passage of meconium"}
{"id" : 322, "question_text" : "A 6-month-old girl presents to the urgent care clinic with vomiting, fussiness, and head tilt. She had a similar episode 1 month ago. She was born at term with no complications and has been otherwise healthy. She is a good eater and has no diarrhea or other gastrointestinal problems. Developmentally she has been on track and currently she sits independently. On physical examination, her head shape is normal and the circumference is 43 cm. Growth parameters are otherwise normal. The pale, fussy infant has a soft abdomen with normal bowel sounds. When placed in a sitting position, her head tilts to the left. Sternocleidomastoid muscles feel symmetric in size. After you straighten her head and neck, she resumes the tilted posture. Her eyes are normally aligned, visual tracking is normal and full, there is no nystagmus, and facial movements are symmetric during crying. Tone in vertical suspension is normal, as are reflexes. Muscle bulk is also normal, with no distal muscle wasting or fasciculations. Of the following, the condition that BEST explains this constellation of findings is", "options" : "[\"brainstem glioma\", \"congenital muscular torticollis\", \"dystonia musculorum deformans\", \"left fourth nerve palsy\", \"paroxysmal torticollis of infancy\"]", "explanation" : "The girl described in the vignette presents with head tilt that developed in infancy. The appearance of this phenomenon, termed torticollis, is generally both head tilting to one side and rotation of the chin toward the other side. In this case, the episodic history (the torticollis comes and goes) and normal comprehensive physical and neurological examination findings are reassuring. The most likely explanation is paroxysmal torticollis of infancy, an idiopathic neurologic condition of infancy characterized by bouts of torticollis that last for hours or days. The cause is unknown, and the prognosis is for continued episodes, with waning after age 2 and resolution by age 3 years. Mild delays in fine and gross motor skills are common, as is a family history of migraine. There is no accepted medical treatment or therapy.\n\nA fixed torticollis in infancy is also generally benign. However, because a head tilt or torticollis can result from a variety of serious and even life-threatening conditions, the evaluation needs to be systematic. Congenital muscular torticollis presents in early infancy with a consistent, not paroxysmal head tilt. Generally, there is an imbalance in the tone or activation of the sternocleidomastoid muscle, leading to palpable hypertrophy or contracture of this muscle in about 50% of cases. The head tilt directs toward and the chin rotation directs away from the affected sternocleidomastoid muscle. Sometimes such a persistent head tilt results in some positional molding of the skull bones. Treatment involves physical therapy. Congenital muscular torticollis is also found in a variety of congenital spinal malformations.\n\nA tumor infiltrating the brainstem can affect multiple cranial nerves as well as motor and sensory pathways. The paroxysmal history and absence of any abnormalities in eye movement for this girl is reassuring and makes brainstem neoplasms unlikely. Dystonia musculorum deformans is an autosomal dominant form of dystonia (DYT 1: MIM ID #128100) that can include cervical dystonia. When it presents in childhood, it begins in older children, typically localizing in a limb before generalizing to other body areas, and the time course is not paroxysmal. Lesions of the fourth cranial nerve (the trochlear nerve) can present with head tilt. The fourth cranial nerve innervates the inferior oblique eye muscle. Activating this nerve rotates the eye inward (intorsion). A palsy in the left fourth nerve results in external rotation of the left eye that is not paroxysmal. In addition, to compensate for this, a head tilt to the right, not left, would be observed.\n\nAmerican Board of Pediatrics Content Specification(s):\n\nRecognize that the differential diagnosis of torticollis includes head tilt secondary to malformation of the cervical spine, visual disturbance, posterior fossa tumor, etc.\n\nDifferentiate between congenital and paroxysmal torticollis"}
{"id" : 1992, "question_text" : "A newborn is seen for a first health supervision visit. There is a family history of retinoblastoma, and his mother carries one wild-type RB1 allele and one mutated RB1 allele. Of the following, this newborn's risk for developing retinoblastoma is", "options" : "[\"between 25% and 50%\", \"between 75% and 100%\", \"dependent on the father's genotype\", \"no different than the general population\"]", "explanation" : "Retinoblastoma is a tumor of the retinal cells that occurs in young children, with two-thirds of the cases diagnosed by 2 years of age and 95% diagnosed by 5 years of age. The development of retinoblastoma requires the inactivation of both copies of RB1 on band 13q14 through mutation, deletion, or epigenetic silencing. Hereditary retinoblastoma occurs when there is a germline mutation of one of the RB1 alleles and a somatic event silencing the other. Nonhereditary or sporadic retinoblastoma occurs when there are 2 independent somatic events silencing both RB1 loci. Despite having a pathophysiology resembling an autosomal recessive disease (the development of retinoblastoma requires the inactivation of both copies of RB1), most germline RB1 mutations have a penetrance of approximately 90%, meaning that hereditary retinoblastoma behaves in a manner approximating an autosomal dominant Mendelian disease. Approximately 40% of cases are hereditary. Of these, 80% arise from de novo mutations in the father's germ cells with no known family history.\n\nThe mother of the neonate in this vignette is a carrier of an abnormal RB1 allele. There is therefore a 50% chance that the neonate inherited this abnormal allele. As germline mutations are approximately 90% penetrant, his chance of developing retinoblastoma is calculated as: (chance of inheriting the abnormal allele) × penetrance = 0.5 × 0.9 = 0.45\n\nTherefore, the chance that this neonate will develop retinoblastoma is less than 50% but more than 25%. If it was certain that the neonate had inherited the abnormal RB1 allele from his mother, then his chances of developing retinoblastoma would be 75% to 100%. However, it is not know if he inherited the mutated gene. Retinoblastoma as a whole is very rare with a prevalence of 11 cases per million children under 5 years, and 80% of heritable retinoblastoma is caused by de novo mutations; therefore, it is reasonable to assume that the father does not carry a germline RB1mutation.\n\nPREP Pearls\n\nThe development of retinoblastoma requires the inactivation of both copies of RB1 on band 13q14 through mutation, deletion, or epigenetic silencing.\n\nRetinoblastoma can be heritable or nonheritable. Heritable retinoblastoma has a penetrance of approximately 90%.\n\nApproximately 40% of cases of retinoblastoma are hereditary, of which approximately 80% arise from de novo mutations in the father's germ cells with no known family history.\n\nABP Content Specifications(s)/Content Area\n\nRecognize the inheritance pattern associated with retinoblastoma and the significance of the family history in planning management\n\nRecognize the clinical and laboratory findings associated with retinoblastoma\n\nDifferentiate the historical and clinical findings associated with hereditary retinoblastoma from those of sporadic retinoblastoma\n\nSuggested Readings\n\nGill J. Inherited hematologic and oncologic syndromes. Pediatr Rev. 2011;32(9):401-404. doi:10.1542/pir.32-9-401.\n\nPDQ Pediatric Treatment Editorial Board, National Cancer Institute. PDQ cancer information summaries: retinoblastoma treatment. https://www.ncbi.nlm.nih.gov/books/NBK66006/.\n\nWilson WG. Retinoblastoma. Pediatr Rev. 2007;28(1):37-38. doi:10.1542/pir.28-1-37."}
{"id" : 2126, "question_text" : "A 2-year-old girl pulled a cup of hot tea off the counter, spilling the liquid onto herself. Her parents immediately removed the wet clothes and brought her to the office. On physical examination, the child is crying but consolable. She is afebrile. There are denuded blisters across her forehead and down her entire right cheek, with extension onto her neck. She has no lip or tongue swelling, stridor, drooling, or obvious injuries to her eyes. An appropriate dose of acetaminophen is given. Of the following, the BEST next step in managing this child's injury is to", "options" : "[\"advise the parents to clean daily and apply a thin layer of petroleum-based ointment\", \"apply long-acting silver-impregnated foam dressing and reassess in 2 to 3 days\", \"apply a sterile dressing and reassess in 1 day\", \"refer the child to a pediatric burn center\"]", "explanation" : "Critique\nManagement of a child's burn depends on the location, depth, and extent of the burns. The girl in the vignette, with partial-thickness burns to her face, should be referred to a pediatric burn center. Pediatric burns are categorized according to severity and body surface area. Burn severity is defined by the depth of structures injured: \nSuperficial: Injury to the epidermal layer of the skin (eg, sunburn) \nPartial thickness: Extends into the dermis \nCharacterized by blisters, significant pain, and fluid loss in areas of denudation \nFurther classified as superficial and deep partial-thickness burns, with implications for healing with deeper injury\nFull thickness: Injury through the dermis \nLeathery, dry appearance \nThe extent of body surface involvement is important to quantify. There are various tools and techniques available for quantification, including the Rule of 9s (must be adapted for younger children), the technique of measuring and equating the child's palm and fingers to 1% of the child's body surface area, and internet-based apps into which the burns can be drawn for surface area calculation.\nAccording to the current American Burn Association guidelines, this child, with burns to her face, should be referred to a pediatric burn center. The American Burn Association guidelines state the following:\nAll superficial burns can be treated at home, regardless of extent. \nPartial-thickness burns involving more than 10% of a child's body surface area require hospitalization for management of fluids, pain, infection, and calorie intake. \nFull-thickness burns of any degree should be referred for evaluation at a pediatric burn center. \nHigher-risk areas of the body are better treated at a specialized burn center, including partial- or full-thickness burns to the face, hands, feet, genitalia, and perineum, as well as burns that cross joints. \nSuperficial burns (in which the skin is intact) or partial-thickness burns of small body surface area can be treated at home. Management in the office includes treatment of pain with acetaminophen or ibuprofen. Small blisters are left intact. For larger blisters or denuded areas, gentle debridement with gauze, water, and mild cleanser is suggested before dressing. Options for dressing include the following:\nApplication of a thin layer of petroleum-based ointment with or without antibiotic so that the dressing does not adhere to the burn wound, coverage with a sterile dressing, and taping or wrapping in place; this is changed 1 to 2 times per day until follow-up \nApplication of a long-acting dressing made of absorbing foam and containing silver or other antibiotic material; this is kept on continuously for 2 to 3 days\nFor the face and neck, petroleum-based moisturizers are the mainstay of burn care after the initial cleaning and debridement\n\nSuggested Reading(s)\nGrote AC, Lacey AM, Garner WL, Gillenwater TJ, Maniago E, Yenikomshian HA. Small pediatric burns can be safely managed on an outpatient basis. J Burn Care Res. 2020;41(5):1029-1032. doi:10.1093/jbcr/iraa115\nGuidelines for Burn Patient Referral. American Burn Association. Accessed September 26, 2023.ameriburn.org\nMoniruzzaman M, Khan AR, Haq MA, Naznin RA, Haque M. Pediatric first-degree burn management with honey and 1% silver sulfadiazine (Ag-SD): comparison and contrast. Cureus. 2022;14(12):e32842. doi:10.7759/cureus.32842 \nSheridan RL. Thermal injuries. In: Kimberlin DW, Banerjee R, Barnett ED, Lynfield R, Sawyer MH, eds. Red Book: 2024-2027 Report of the Committee on Infectious Diseases. 33nd ed. American Academy of Pediatrics; 2024. Accessed September 1, 2024. Red Book Online\nTran S, Jacques MA, Holland AJA. Assessment and management of minor burns in children. Aust J Gen Pract. 2019;48(9):590-594. doi:10.31128/AJGP-04-19-4919\n\nContent Domain\nEmergency Medicine\n\nLearning Objectives\nIdentify burns that require management at a burn center\nDistinguish the severity of burns\n\nThe correct answer is: refer the child to a pediatric burn center"}
{"id" : 3360, "question_text" : "A 3-year-old boy visits your office for a new patient visit. His mother reports that he has had very dry, scaly skin since infancy that has not improved with trials of various over-the-counter moisturizing creams. His mother reports that her brother also had very scaly skin, and both she and her brother have small cataracts that do not interfere with vision. On physical examination, the boy's skin is remarkable for a widespread fine brown scale, especially prominent in flexor joint creases, on the sides of the neck, the flanks, and in front of the ears. The palms and soles are not affected. You also detect a small corneal opacity in his right eye. Of the following, in addition to referral to ophthalmology, the BEST next treatment to prescribe for this child is", "options" : "[\"calcineurin inhibitor\", \"high-potency topical corticosteroid\", \"topical antibiotic ointment\", \"topical retinoid\", \"urea-containing emollient\"]", "explanation" : "Ichthyosis occurs in various forms, all involving abnormal skin keratinization, barrier function, and desquamation that produce the characteristic scaly skin of these disorders. Treatment is aimed at moisturizing and softening the skin and promoting desquamation. Urea cream is one of the most effective products to treat ichthyosis and therefore would be the most appropriate initial treatment for the child in the vignette.\n\nIchthyosis is often categorized genetically and can be autosomal dominant, autosomal recessive, X-linked, syndromic, or sporadic. Recent evidence implicates mutations of the filaggrin gene, also important in atopic dermatitis, with autosomally inherited ichthyosis. The most common form is ichthyosis vulgaris, an autosomal dominant condition with onset after the newborn period (often during the first year of life or early childhood). It is characterized by fine white or tan scales most prominent on the lower legs, while sparing the antecubital and popliteal fossae. Patients also demonstrate hyperlinearity of the palms and soles. They frequently have comorbid atopic dermatitis and keratosis pilaris.\n\nThe patient in the vignette demonstrates characteristic findings of X-linked ichthyosis, which is associated with an absence of cholesterol sulfate sulfhydrolase. The onset of this form of ichthyosis can occur at birth with widespread erythema and desquamation. As the patient matures, he develops brown, polygonal scales in the flexures, preauricular, lateral neck and flank areas. There is no palmar or plantar involvement. Corneal opacities are common both among patients (50%) and carriers (30%), and patients also have an increased risk of testicular cancer. Lamellar ichthyosis is a rare condition (incidence of about 1:300,000), but is among the most common autosomal recessive forms of ichthyosis. It is one of the causes of \"collodion baby; in which the newborn is covered by a membrane and exhibits ectropion and small, misshapen ears. Hair may be sparse and palms and soles are thickened; teeth and mucosal surfaces are normal. Scales are large, dark, rectangular, and adherent to the entire surface, including flexor creases. The other common type of collodion baby occurs with congenital ichthyosiform erythroderma. These infants demonstrate erythroderma, which is not seen in lamellar ichthyosis. Scales are finer and whiter in color. There is hyperkeratosis of the knees, elbows, ankles, palms, and soles. Hair is sparse and nails are dystrophic.\n\nIn addition to these more common forms of ichthyosis, a vast array of syndromes exist in which ichthyosis is a component. In these syndromes, ichthyosis can be associated with disorders of the eyes, bones, central nervous system, growth, and development. Affected children may also have sensorineural hearing loss and may be born prematurely.\n\nThe goal of treatment for most children suffering from ichthyosis is to support skin barrier function and reduce pruritus by use of moisturizing agents. Among the most effective classes of products to achieve moisturization, as well as keratolysis, are urea and lactic acid creams. Topical retinoids have been useful for some patients, but are irritating and expensive to apply over a large area; they are not first-line therapy for ichthyosis. Topical steroids or calcineurin inhibitors may be useful, especially for patients who also have atopic dermatitis, but would be second-line therapy for ichthyosis. Steroids should be used at the lowest potency that is effective. Topical antibiotics are not indicated unless there is a secondary bacterial infection.\n\nPREP Pearls\n• The ichthyoses are a set of disorders with abnormal skin keratinization and barrier function, characterized by scaling.\n• Ichthyoses may be autosomal dominant, recessive, X-linked, sporadic, or syndromic.\n• Urea and lactic acid creams are effective first-line treatments for ichthyoses.\n• The most common form of ichthyosis is the autosomal dominant ichthyosis vulgaris, characterized by white scale prominent on the lower extremities.\n• X-linked ichthyosis presents with brown adherent scale on the neck, flank, face, and flexor creases, and is often associated with lens opacities.\n\nAmerican Board of Pediatrics Content Specification(s)\n• Recognize the clinical findings associated with ichthyosis\n\nSuggested Reading\n• Krowchuk DP, Mancini Al. Ichthyosis. In: Krowchuk DP, Mancini AJ, eds. Pediatric Dermatology: A Quick Reference Guide. 2nd ed. Elk Grove Village IL: American Academy of Pediatrics; 2011:441-448.\n• Epps RE. Atopic dermatitis and ichthyosis. Pediatr Rev. 2010;131(7):278285. doi:10.1542/pir.31-7-278.\n• Fleckman P, Newell BD, van Steensel MA, Yan AC. Topical treatment of ichthyoses. Dermatol Then 2013;2611):16-25. dal: 10.1111/j.1529-8019.2012.01526.x.\n• Morelli JG. Disorders of keratinization. In: Kliegman RM, Stanton BMD, St. Geme JW Ill, Schor NF, Behrman RE, eds. Nelson Textbook of Pediatrics. 19th ed. Philadelphia, PA: Saunders Elsevier; 2011:2267-2273."}
{"id" : 2123, "question_text" : "A 5-year-old girl is undergoing evaluation for a persistently itchy scalp. She was prescribed permethrin 1% cream 4 weeks ago and has received 2 applications 10 days apart. Her physical examination is remarkable for live lice and nits (Figure). Of the following, the BEST next step in this girl's care is", "options" : "[\"lindane shampoo\", \"oral ivermectin\", \"pyrethrin + piperonyl butoxide shampoo\", \"spinosad 0.9% topical suspension\"]", "explanation" : "Critique\nMultiple nits and live lice are seen on this child's hair and scalp in the figure. Treatment with permethrin 1% lotion, with a repeat application 10 days later, is a correct first step for treating pediculosis capitis or head lice. The recommended next step for persistent head lice in children older than 6 months is treatment with spinosad shampoo or topical ivermectin. \nOral ivermectin may be considered for treating head lice in children that persist after all recommended topical options have been tried. This would be an off-label use, because oral ivermectin is approved for this indication by the US Food and Drug Administration only in adults. Topical malathion is also approved for treatment of resistant head lice in children older than 6 years. Lindane shampoo is no longer recommended in children owing to its neurotoxicity.\nHead lice infestation is common in school-aged children in the United States. It affects children in all socioeconomic groups and with all hair lengths and textures. It is not a sign of poor hygiene; nor is it a health hazard, because head lice are not a vector of disease transmission. Lice are transmitted mostly via head-to-head contact; transmission via contact with personal items, such as hair brushes and head wear, is rare. Lice may also be found in pubic hair (pediculosis pubis). This is most commonly seen in adolescents and young adults and transmitted via sexual contact.\nThe diagnosis of pediculosis capitis or pubis is made clinically. Live lice can be visualized with the naked eye. Finding nits (empty shell casings or eggs) on the hair shaft within ¼ inch of the base of the shaft that are hard to remove suggests that there has been a lice infestation; however, because nits can remain firmly attached after death or hatching, this finding does not confirm an active infestation. \nOnly children with confirmation of an active lice infestation should be treated; treatment should not be implemented if only nits are seen. In the United States, the first-line treatments for lice are 1% permethrin lotion (approved for children aged 2 months or older) or pyrethrin + piperonyl butoxide shampoo (approved for children aged 24 months or older). A second application of either of these products is recommended 9 to 10 days after the initial treatment to treat lice that may have hatched after the first treatment, because these treatments are not fully ovicidal. In some communities, the clinical effectiveness of these products is only 25%. Options for treating resistant cases of lice include 0.9% topical spinosad suspension or topical ivermectin for children older than 6 months. Topical malathion is an option for children older than 6 years. Family members of a person with a lice infestation should be treated if they have lice or nits within 1 cm of the scalp. It also may be prudent to treat anyone who shares a bed with an infested person. All Food and Drug Administration–approved topical lice treatments are considered safe for pregnant and lactating women. Because lice generally do not survive >48 hours on fomites, cleaning should be focused on items that have been in contact with an infested person's head in the last 2 days.\nRoutine screening for lice or nits in schools is discouraged. Screening for nits is not an accurate way to determine active infestation, and screening for head lice has not been shown to decrease the incidence of cases in a school. Children with active lice are likely to have had the infestation for many weeks, as the pruritus often takes 4 to 6 weeks to manifest. Children with lice should not be restricted from attending school, and school-wide \"no-nit\" policies should no longer be considered acceptable.\n\nSuggested Reading(s)\nAmerican Academy of Pediatrics. Pediculosis capitis (head lice). In: Kimberlin DW, Banerjee R, Barnett ED, Lynfield R, Sawyer MH, eds. Red Book: 2024-2027 Report of the Committee on Infectious Diseases. 33nd ed. American Academy of Pediatrics; 2024. Accessed September 1, 2024. Red Book Online\nNolt D, Moore S, Yan AC, Melnick L; Committee on Infectious Diseases, Committee on Practice and Ambulatory Medicine, Section on Dermatology. Head lice. Pediatrics. 2022;150(4):e2022059282. doi:10.1542/peds.2022-059282\n\nContent Domain\nDermatology\n\nLearning Objectives\nPlan the appropriate management for a patient with pediculosis capitis or pediculosis pubis\nRecognize that children with lice should not be restricted from attending school\n\nThe correct answer is: spinosad 0.9% topical suspension"}
{"id" : 1920, "question_text" : "A 5-month-old male infant is being evaluated for growth failure, severe muscle weakness, delayed motor development, and global hypotonia. The mother reports a recent concern with his suck and swallow capabilities as well as 2 episodes of aspiration pneumonia. He was the product of a normal pregnancy and delivery. Development of symptoms, with progression, was noted after 2 months of age. Physical examination reveals a small, nondysmorphic infant with severe muscular weakness which spares the face. He is able to smile, laugh, and coo. He has mild contractures at the knees, postural tremor of the fingers, areflexia, and tongue fasciculations. He has normal male genitalia, with bilaterally descended testicles of normal size. Family history is unremarkable. Of the following, the infant's MOST likely diagnosis is", "options" : "[\"Duchenne muscular dystrophy\", \"Prader Willi syndrome\", \"spinal muscular atrophy\", \"Zellweger syndrome\"]", "explanation" : "Correct Answer: C\nThe infant in the vignette has spinal muscular atrophy (SMA), which results from progressive degeneration and loss of anterior horn cells in the spinal cord and the brain stem nuclei. Children present with symmetric muscle weakness beginning proximally then moving distally, hypotonia, areflexia/hyporeflexia, and tongue fasciculations. The facial muscles are relatively spared; however, suck and swallow capabilities are typically affected. Fasciculations of the tongue are seen in most children, which is a defining physical examination finding not commonly seen in pediatric neuromuscular disorders. As the disease advances, the child develops a bell-shaped chest and paradoxical respirations because of intercostal muscle weakness with relative preservation of the diaphragm musculature. This is known as \"abdominal breathing.\" Diaphragmatic involvement will occur later in the disease course, resulting in full-time ventilator dependency or death.\n\nSpinal muscular atrophy is a predominantly autosomal recessive neuromuscular disorder with 5 subtypes:\n\nSMA 0: Prenatal onset with infantile death\n\nSMA I: Onset <6 months of age with lifespan of ≥2 years\n\nSMA II: Onset at 6-18 months, 70% alive at 25 years of age\n\nSMA III: Onset >18 months, normal lifespan\n\nSMA IV: Adulthood onset, normal lifespan\n\nThere is one X-linked form of SMA.\n\nThe infant in the vignette likely has SMA type I, given the age at symptom onset and clinical findings. Diagnosis of SMA is established by history, physical examination, and the presence of biallelic pathogenic gene variants in SMN1 on molecular analysis, most commonly an exon 7 deletion. The number of copies, ranging from 0 to 5, of the SMN2 gene, can modify the phenotype. The more copies of SMN2, the milder the phenotype.\n\nTreatment is supportive at this time; there is no cure. Close monitoring of the child's nutritional state, respiratory function, and orthopedic status, with reassessment at least every 6 months is recommended, with appropriate interventions such as gastrostomy tube or noninvasive ventilator support, as the child's condition worsens. New approaches and treatments are being investigated, including upregulating the SMN2 gene protein product to alter the natural history of the motor neuron degeneration via antisense oligonucleotides which are single-stranded RNA molecules that target complementary sequences in the SMN2 transcript that lead to inclusion of exon 7 increasing the full-length of the SMN protein. This has demonstrated substantial promise for the treatment of this disorder.\n\nDuchenne muscular dystrophy is an X-linked recessive disorder presenting with progressive proximal muscular weakness and calf hypertrophy in boys. Creatine kinase levels are 10 times that of normal. Symptoms present in early childhood, typically between 3 and 5 years of age, with wheelchair dependency occurring before age 13 years. Dilated cardiomyopathy occurs during the second decade of life, and is the leading cause of morbidity and mortality. Heterozygous female carriers are also at risk for dilated cardiomyopathy.\n\nPrader Willi syndrome presents in the neonate with global hypotonia, poor suck, hypogonadism, and characteristic facial features that include bitemporal narrowing of the head, almond-shaped eyes, elongated face, and thin upper lip. It is characterized by feeding difficulties and poor weight gain in early infancy, with the transition to excessive eating and morbid obesity in early to late childhood. Diagnosis is made via DNA methylation testing of the parent-specific imprinting critical region for Prader Willi (PWCR) on chromosome 15.\n\nZellweger syndrome is an autosomal recessive peroxisomal biogenesis disorder that presents in the newborn period with global hypotonia, poor feeding, seizures, liver cysts with dysfunction, and distinctive facies. Typical facial dysmorphology includes flattened facies, large anterior fontanelle, broad nasal bridge, and widely spaced sutures. Affected infants typically die in the first year after birth.\n\nAn infant with Duchenne muscular dystrophy, Prader Willi syndrome, or Zellweger syndrome will not have tongue fasciculations.\n\nPREP Pearls\n\nSpinal muscular atrophy (SMA) is an autosomal recessive neuromuscular disorder with 5 subtypes, which results from progressive degeneration and loss of anterior horn cells in the spinal cord and the brain stem nuclei.\n\nChildren with SMA present with symmetric muscle weakness beginning proximally then moving distally, hypotonia, areflexia/hyporeflexia, and tongue fasciculations with sparing of the facial musculature.\n\nDuchenne muscular dystrophy is an X-linked recessive disorder presenting in boys typically between 3 and 5 years of age with progressive proximal muscular weakness, calf hypertrophy, and creatine kinase levels 10 times normal. Wheelchair dependency typically occurs before age 13 years.\n\nABP Content Specifications(s)/Content Area\n\nUnderstand the inheritance pattern in a patient who has a neuromuscular disorder (eg, muscular dystrophy, spinal muscular atrophy\n\nSuggested Readings\n\nBowerman M, Becker CG, Yáñez-Muñoz RJ, et al; UK SMA Research Consortium. Therapeutic strategies for spinal muscular atrophy: SMN and beyond. Dis Model Mech. 2017;10(8):943-954. doi: 10.1242/dmm.030148 .\n\nParente V, Corti S. Advances in spinal muscular atrophy therapeutics. Ther Adv Neurol Disord. 2018 Feb 5;11:1756285618754501. doi: 10.1177/1756285618754501.\n\nPrior TW, Finanger E. Spinal muscular atrophy. GeneReviews. https://www.ncbi.nlm.nih.gov/books/NBK1352 .\n\nWang CH, Finkel RS, Bertini ES, et al; Participants of the International Conference on SMA Standard of Care. Consensus statement for standard of care in spinal muscular atrophy. J Child Neurol. 2007;22(8)1027-1049. doi: 10.1177/0883073807305788 ."}
{"id" : 1277, "question_text" : "An 18-year-old young woman presents to your office with a 4-day history of left ear pain and swelling. She reports no recent trauma or insect bites to the ear, but did have a new piercing along the upper pinna about 1 week ago. Her past medical history is significant only for seasonal allergies. Physical examination shows an uncomfortable young woman complaining of 8 out of 10 pain in her left ear and left lateral neck. Vital signs show a temperature of 37°C, respiratory rate of 18 breaths/min, heart rate of 95 beats/min, and blood pressure of 125/65 mm Hg. Her left ear is impressively swollen, hot, erythematous, and tender along the helix; there is fluctuance with an earring embedded in the swelling. Left postauricular swelling is also seen. There is no lymphadenopathy and she has full range of motion in her neck. The left tympanic membrane and external auditory canal appeared normal. Laboratory data are shown: White blood cell count: 10,400/μL (10.4 x 109/L); Lymphocytes: 30%; Neutrophils: 66%; Monocytes: 1%; Hemoglobin: 13 g/dL (130 g/L); Hematocrit: 40%; Platelet count: 369 x 103/μL (369 x 109/L). When the earring is removed, about 3 mL of pus is expressed. The pus is sent for culture and Gram stain (Item Q25). Item Q25: Gram stain of the pus described in the vignette. Courtesy of P Lee. Of the following, the BEST choice for initial therapy is", "options" : "[\"ceftriaxone\", \"ciprofloxacin\", \"mupirocin\", \"trimethoprim-sulfamethoxazole\", \"vancomycin\"]", "explanation" : "The best choice for initial therapy for the young woman in the vignette is ciprofloxacin. At first glance, she has what may appear to be a straightforward ear abscess, but due to location and association with piercing of the ear helix (ie, a \"high piercing\"), this is more consistent with a suppurative auricular perichondritis. The perichondrium is a layer of connective tissues surrounding all of the body's cartilage except the cartilage in joints. Since cartilage is avascular, it is dependent on the perichondrium for its nutrients and oxygen. When an infection from a piercing through cartilage occurs, the subsequent inflammation and pus that results can separate the perichondrium from the cartilage and lead to aseptic and/or septic necrosis, resulting in permanent loss or deformity of the outer ear. While Staphylococcus aureus remains an important cause of perichondritis, Pseudomonas aeruginosa is the usual pathogen involving piercing of the helix of the ear. Pseudomonas from the external ear canal, where it is commonly found, or from nonsterile water may inadvertently get into the piercing, creating an insidious, progressively destructive infection.\n\nThe Gram stain in this example shows gram-negative rods, confirming that Pseudomonas, not the less common Staphylococcus, is the pathogen that needs to be treated. Vancomycin, which has activity only against gram-positive organisms, would be the antibiotic of choice for S aureus, but would not be appropriate for Pseudomonas. Mupirocin and trimethoprim-sulfamethoxazole, which are also used against S aureus, have some gram-negative activity, but not against Pseudomonas. Also, mupirocin is topical and systemic intravenous antibiotics are necessary to treat perichondritis. Third-generation cephalosporins have good gram-negative activity, but many of them, including ceftriaxone, are ineffective against Pseudomonas. Of the choices listed, ciprofloxacin, a fluoroquinolone, is the only antibiotic with excellent Pseudomonas coverage. It can penetrate into the cartilage, making it a frequent first-line agent for perichondritis.\n\nA multidisciplinary team, including otorhinolaryngology, surgery, and plastic surgery, may be necessary for incision and drainage of the abscess, removal of necrotic tissue, and reconstruction and revision of the deformed ear that often results.\n\nPREP Pearls\n• Piercings of the upper ear helix may cause a suppurative perichondritis, frequently caused by Pseudomonas aeruginosa, instead of Staphylococcus aureus.\n• Systemic treatment with an antipseudomonal agent like ciprofloxacin is required.\n• Surgical drainage, debridement, and reconstruction of the ear may be necessary.\n\nABP Content Specifications(s)\n• Recognize the risk factors for the development of pseudomonal infections\n• Recognize the clinical manifestations of pseudomonal infections and manage appropriately\n\nSuggested Readings\n• American Academy of Pediatrics. Escherichia coli and other gram-negative bacilli (septicemia and meningitis in neonates). In: Kimberlin DW, Brady MT, Jackson MA, Long SS, eds. Red Book: 2015 Report of the Committee on Infectious Diseases. 30th ed. Elk Grove Village, IL: American Academy of Pediatrics; 2015:340-342 .\n• Davidi E, Paz A, Duchman H, Luntz M, Potasman I. Perichondritis of the auricle: analysis of 114 cases. Isr Med Assoc J. 2011;13(1):21-24. http://www.ima.org.il/FilesUpload/IMAJ/0/38/19428.pdf."}
{"id" : 2478, "question_text" : "A 5-year-old boy is brought to the emergency room for evaluation of body swelling and weight gain over the past 3 days. The swelling started over his eyelids and now involves his legs. He has normal urine output. There is no change in urine color, dysuria, fever, or rash. His vital signs are a blood pressure of 105/65 mm Hg, a heart rate of 92 beats/min, and a respiratory rate of 16 breaths/min.\n\nThe boy's weight is at the 85th percentile and height is at the 50th percentile for age. He is alert.\n\nHe has swelling of the upper eyelids, abdominal distension, and pitting edema of the lower legs. The remainder of the boy's physical examination findings are unremarkable.\n\nLaboratory results are shown:\nSodium: 131 mEq/L (131 mmol/L)\nPotassium: 3.9 mEq/L (3.90 mmol/L)\nChloride: 102 mEq/L (102 mmol/L)\nBicarbonate: 24 mEq/L (24 mmol/L)\nBlood urea nitrogen: 14 mg/dL (5.0 mmol/L)\nCreatinine: 0.3 mg/dL (26.52 µmol/L)\nCalcium: 7.5 mg/dL (1.88 mmol/L)\nAlbumin: 2.0 g/dL (20.00 g/L)\n\nUrine:\npH: 5.7\nSpecific gravity: 1.020\nProtein: 4+\nBlood: Negative\nRed blood cells: < 2/high-power field\nWhite blood cells: < 5/high-power field\n\nOf the following, the MOST likely cause of this boy's hyponatremia is", "options" : "[\"decreased glomerular filtration rate\", \"pseudohyponatremia\", \"urinary sodium loss\", \"water retention\"]", "explanation" : "The boy in the vignette has nephrotic syndrome (NS), evidenced by his eyelid and leg edema, hypoalbuminemia, and 4+ proteinuria. His hyponatremia is dilutional secondary to water retention. The low effective circulatory volume secondary to hypoalbuminemia in NS causes a decrease in urine sodium.\n\nNephrotic syndrome is characterized by the following:\n- Heavy proteinuria (urine protein >40 mg/m²/hr or urine-protein-to-creatinine ratio of >2 mg/mg)\n- Hypoalbuminemia (serum albumin <2.5 g/dL [25 g/L])\n- Edema\n- Hyperlipidemia\n\nNephrotic syndrome usually presents with periorbital edema and a progressive increase in swelling that leads to pedal edema, ascites, pleural effusion, vulvar or scrotal edema, and anasarca. The typical age of NS onset is between 2 and 10 years. Most cases of NS in children are idiopathic (primary), but some have secondary causes (underlying systemic disease such as vasculitis or systemic lupus erythematosus). Minimal change disease is the most common histology in children with idiopathic NS.\n\nLaboratory evaluation of suspected NS typically begins with a urinalysis that shows proteinuria. Microscopic hematuria (3-5 red blood cells/high-power field) occurs in 25% of children with idiopathic NS. The serum albumin concentration is low because of increased urinary losses. The serum cholesterol concentration is elevated because of low oncotic pressure from loss of serum albumin, increased synthesis, and decreased degradation of products in the cholesterol pathway. The serum calcium concentration may be low in NS as a result of hypoalbuminemia. However, ionized calcium concentrations are normal.\n\nChildren with NS have mild dilutional hyponatremia because of water retention, which is a result of lower effective intravascular volume from hypoalbuminemia in addition to inappropriate secretion of antidiuretic hormone. A normal amount of water intake causes edema and hyponatremia in NS. Hyponatremia can also result from diuretic therapy used in the treatment of NS. Hyponatremia in NS is not due to the urinary loss of sodium. Urine sodium excretion is decreased due to low effective intravascular volume. The hyponatremia in NS is true hyponatremia and is not a factitious or pseudohyponatremia.\n\nThe glomerular filtration rate (indicated by blood urea nitrogen and serum creatinine concentrations) is usually normal in NS, as seen with the child in the vignette. Mildly increased blood urea nitrogen and serum creatinine concentrations may occur in NS owing to decreased effective circulatory blood volume, but this is not the cause of hyponatremia in NS. A decrease in glomerular filtration rate (azotemia), hypertension, and macroscopic hematuria are commonly seen in nephritic syndrome or acute glomerulonephritis.\n\nPrednisone is the first-line treatment for idiopathic NS. Hyponatremia in NS is treated with water and sodium restriction and by addressing the hypoalbuminemia.\n\nSuggested Reading(s)\nRodriguez-Ballestas E, Reid-Adam J. Nephrotic syndrome. Pediatr Rev. 2022;43(2):87-99. doi:10.1542/pir.2020-001230\nVarade WS. Nephrotic syndrome. In: McInerny TK, Adam HM, Campbell DE, DeWitt TG, Foy JM, Kamat DM, eds. American Academy of Pediatrics Textbook of Pediatric Care. 2nd ed. American Academy of Pediatrics; 2016:chap 295. Accessed September 1, 2023. Pediatric Care Online\n\nContent Domain\nRenal\n\nABP Content Specification(s) / Content Area(s)\nFormulate a differential diagnosis of nephrotic syndrome with and without hematuria\nIdentify the etiology of hyponatremia in nephrotic syndrome\n\nThe correct answer is: water retention"}
{"id" : 907, "question_text" : "A 2-year-old boy is admitted to the pediatric inpatient service 4 hours after swallowing a small amount of kerosene. On physical examination, the boy has a temperature of 38.0°C, heart rate of 130 beats/min, blood pressure of 85/50 min Hg, and respiratory rate of 40 breaths/min. He is awake and alert with mild nasal flaring; he has bilateral breath sounds with mild wheezing and mild intercostal retractions. His oxygen saturation on room air as measured by pulse oximetry is 90%. A chest radiograph is obtained (Item Q201).\n\nITEM Q201: Radiographic findings for the boy in the vignette.\n\nOf the following, the MOST appropriate next step in the management of this boy is administration of", "options" : "[\"activated charcoal\", \"albuterol\", \"antibiotics\", \"corticosteroids\", \"isoproterenol\"]", "explanation" : "Hydrocarbons represent about 2% of all pediatric non-pharmaceutical ingestions in the United States. Hydrocarbons generally are ingested in small amounts because of the foul taste, but can produce significant morbidity and mortality as evidenced by the fact that hydrocarbons comprise 10% of pediatric ingestion fatalities in the United States. Symptoms of hydrocarbon ingestion can be limited to the respiratory system (direct effect of the hydrocarbon on pulmonary tissue) or they can be systemic (due to gastrointestinal and respiratory absorption). Respiratory symptoms include coughing, choking, tachypnea, and cyanosis. Systemic symptoms and clinical findings include central nervous system depression, cardiac dysrhythmias, fever, leukocytosis, hemolysis, and hemoglobinuria. Hydrocarbons produce both destruction of the respiratory components (airway epithelium, alveolar septae, and pulmonary capillaries) and dissolution of the lipid surfactant layer. These processes can result in chemical pneumonitis, pneumothorax, necrotizing pneumonia, respiratory failure, and death.\n\nManagement is primarily supportive. Patients often develop bronchospasm and should be treated with a 82 selective agent such as albuterol. Isoproterenol should be avoided because of myocardial sensitization and subsequent risk of fatal dysrhythmias. The pneumonitis associated with hydrocarbon ingestion is chemical and, therefore, antibiotics are indicated only if there are signs of secondary infection (fever or progression of infiltrates on chest radiography after 48 hours). Corticosteroids have not been shown to reduce respiratory symptoms or damage. External decontamination (removal of contaminated clothes and cleansing of skin) should be performed. Gastric decontamination (administration of syrup of ipecac or nasogastric lavage) should be avoided because of the risk of aspiration. The use of activated charcoal is contraindicated in single-agent hydrocarbon ingestion and when the patient presents with pulmonary symptoms, as seen in the patient in the vignette. Activated charcoal might be recommended by the poison control center in a multiple substance ingestion with systemic toxicity.\n\nPREP Pearls\n• Ingestion of hydrocarbons represents about 2% of all pediatric nonpharmaceutical ingestions in the United States but cause 10% of pediatric ingestion fatalities.\n• Hydrocarbon ingestion can manifest in respiratory symptoms or systemic symptoms if gastrointestinal or respiratory absorption occurs.\n• Gastric decontamination (administration of syrup of ipecac or nasogastric lavage) should be avoided in cases of hydrocarbon ingestion because of the risk of aspiration.\n\nAmerican Board of Pediatrics Content Specification(s):\n• Understand that hydrocarbon pneumonitis may cause acute and chronic lung disease\n\nSuggested Reading:\n• Colombo JL. Aspiration syndromes. In: Kliegman RM, Stanton BMD, St Geme J, Schor N, Behrman RE, eds. Nelson Textbook of Pediatrics. 19th ed. Philadelphia, PA: Elsevier Saunders; 2011:1469-1471\n• Lewander WJ, Aleguas A. Hydrocarbon poisoning. UptoDate. Available online only for subscription"}
{"id" : 3628, "question_text" : "A mother reports to the nurse that her newborn is not feeding well. He was born 28 hours ago at 39 weeks' gestation via spontaneous vaginal delivery after an uncomplicated pregnancy. Her group B Streptococcus status was positive and she received 3 doses of penicillin before delivery. The neonate's Apgar scores were 8 and 9 at 1 and 5 minutes, respectively. The neonate latched on and fed well yesterday, but has not been interested in breastfeeding today. On physical examination, his temperature is 36°C, heart rate is 145 beats/min, respiratory rate is 65 breaths/min, and blood pressure is 65/43 mm Hg. He is unresponsive. His pupils are reactive to light; anterior fontanelle is flat; heart has a regular rate and rhythm, with no murmur noted; lungs are clear with normal work of breathing. He has no hepatomegaly; +2 peripheral pulses; capillary refill 2 to 3 seconds; generalized decreased tone; limited active movement; no clonus; and decreased reflexes throughout. His glucose level on dextrose stick is 65 mg/dL (3.6 mmol/L). He is brought to the neonatal intensive care unit, where a blood culture, cerebrospinal fluid culture, arterial blood gas, and ammonia level are obtained. He is started on treatment with intravenous ampicillin, gentamicin, and acyclovir. Of the following, the BEST next test to confirm this neonate's diagnosis is", "options" : "[\"chest radiography\", \"echocardiography\", \"electroencephalography\", \"head ultrasonography\"]", "explanation" : "The most likely cause of this neonate's condition is seizures, which can be diagnosed with electroencephalography. Neonatal seizures affect 1 to 3 per 1,000 neonates born at term gestation. The most common cause is hypoxia resulting from perinatal events or cardiac dysfunction. Hypoglycemia, intracranial hemorrhage, and intracerebral infection are other important reasons for neonatal seizures.\n\nNeonates are at increased risk for seizures compared with older children and adults for several reasons. In the first weeks after birth, neonates have decreased inhibition of cortical activity. The neonatal neuron has a relatively high chloride concentration, dampening the inhibitory neurotransmitter effect of γ-aminobutyric acid (GABA). In addition, mechanisms of inhibition within the substantia nigra are not fully developed. There is also a predominance of excitatory activity via glutamate and N-methyl D-aspartic acid (NMDA) receptors.\n\nNeonatal seizures are often subtle. Focal tonic clonic movement of the extremities or facial muscles is the most common presentation. Eye deviation, tongue thrusting, and other facial movements are other presenting signs. The initial evaluation of neonatal seizures should include the measurement of serum glucose and electrolyte levels. Diagnosis is confirmed with continuous electroencephalographic findings correlated with clinical movements concerning for seizure. Because meningitis, viral or bacterial, can present with new-onset seizures, a lumbar puncture must be performed. Radiologic imaging should include head ultrasonography and magnetic resonance imaging as needed. Pharmacologic treatment typically begins with phenobarbital. Seizures that are difficult to control may require the addition of a second agent such as fosphenytoin. The long-term complications of neonatal seizure vary based on the underlying etiology. Approximately half of all neonates with seizures caused by hypoxic injury at birth will have long-term neurodevelopmental delays.\n\nThe differential diagnosis for lethargy in a neonate includes early-onset sepsis, hypoglycemia, inherited diseases of metabolism, and seizures. Initial evaluation must include blood culture and empiric treatment for group B Streptococcus (GBS), Escherichia coli, and herpes simplex virus. Group B Streptococcus is the most common cause of early-onset sepsis among term neonates. Infection with GBS has a range of presentations, including respiratory distress and hypoglycemia. Neonatal hypoglycemia is defined by a serum glucose of less than 45 mg/dL (2.5 mmol/L) in the first 24 hours after birth, and may be associated with decreased activity. Although neonatal seizures are a more likely diagnosis in this scenario, inherited diseases of metabolism should be considered in a neonate with this presentation and investigated with an arterial blood gas, lactate, serum electrolytes, and ammonia at the minimum. Additional workup may include serum and urine amino acids, urine organic acids, urinalysis, and urine-reducing substances.\n\nIn the absence of respiratory symptoms, chest radiography is unlikely to identify this neonate's diagnosis. Echocardiography is not indicated given his normal cardiac examination, pulses, and perfusion. Head ultrasonography should be included in the evaluation of neonatal seizures, but will not confirm the diagnosis.\n\nPREP Pearls\n• Neonates are at increased risk for seizures because of decreased inhibition of excitatory signals and underdeveloped mechanisms of inhibition.\n• The most common cause of neonatal seizure is hypoxia resulting from perinatal events or cardiac dysfunction.\n• The differential diagnosis for decreased activity in a neonate includes seizures, early-onset sepsis, hypoglycemia, and inherited diseases of metabolism.\n\nMOCA-Peds Objective\n• Evaluate an infant with suspected neonatal seizures.\n\nABP Content Specifications(s)\n• Formulate a differential diagnosis of lethargy and coma in a neonate\n• Formulate a differential diagnosis of neonatal seizures\n\nSuggested Readings\n• Burton BK. Inborn errors of metabolism in infancy: a guide to diagnosis. Pediatrics. 1998;102(6):e69. doi:10.1542/peds.102.6.e69.\n• Gillam-Krakauer M, Carter BS. Neonatal hypoxia and seizures. Pediatr Rev. 2012;33(9):387-396. doi:10.1542/pir.33-9-387.\n• Mosley M. Neonatal seizures. Pediatr Rev. 2010;31(3):127-128. doi:10.1542/pir.31-3-127.\n• Roddy SM, McBride MC. Seizure disorders. In: McInerny TK, Adam HM, Campbell DE, DeWitt TG, Foy JM, Kamat DM, eds. American Academy of Pediatrics Textbook of Pediatric Care. 2nd ed. Elk Grove Village, IL: American Academy of Pediatrics; 2017:2599-2617. Pediatric Care Online."}
{"id" : 1136, "question_text" : "A 21-year-old woman presents to the clinic for her first routine gynecologic examination. She states that her last menstrual period was about 3 weeks ago. She reports mild cramping during her cycle. She denies sexual activity. She has no other concerns or complaints. Upon physical examination, she is afebrile and has normal vital signs. Her pelvic examination is significant for a left-sided adnexal mass, but the rest of her examination is unremarkable. She is sent for a pelvic ultrasonography, which reveals a 4 cm anechoic fluid-filled mass of the left ovary. Of the following, the MOST appropriate next step in her management is", "options" : "[\"\\u03b1-fetoprotein testing\", \"CA-125 testing\", \"culdocentesis\", \"cystectomy\", \"oral contraceptives\"]", "explanation" : "Functional ovarian cysts are the most common type of ovarian mass in the postpubertal female and are the result of ovulation. These cysts are often asymptomatic and found on routine pelvic examination or incidentally on imaging. Symptomatic patients may complain of pelvic pain or menstrual irregularity. Two types of functional cysts include simple or follicular cysts and corpus luteum cysts. Follicular cysts develop when the growing follicle does not open to release an egg. The corpus luteum is the natural result of ovulation. However, the area of the corpus luteum can fill with fluid and develop into an ovarian cyst.\nFunctional cysts that are less than 5 cm usually self-resolve within 2 to 3 menstrual cycles. Oral contraceptives can be used to prevent future cysts, but are not thought to aid in the resolution of a cyst.\nα-fetoprotein is a tumor marker that is frequently elevated in patients with malignant ovarian germ cell tumors (MOGCT). On ultrasonography, an MOGCT would have a more complex and heterogeneous appearance than a simple cyst.\nThe tumor marker CA-125 is associated with epithelial ovarian cancer. However, it can also be elevated in young women with endometriosis. It is not indicated in the woman in this vignette because the ultrasonographic features of the mass suggest a benign process.\nA simple cyst of less than 5 cm in a premenopausal woman can be followed for resolution and often does not require surgical intervention. Oral contraceptives can be used for prevention of additional cysts. Larger cysts, symptomatic cysts, or cysts that are increasing in size may require aspiration or cystectomy. Large cysts increase the risk of ovarian torsion because of their weight.\nPREP Pearls\n Functional ovarian cysts are the most common type of ovarian mass in the postpubertal female and are the result of ovulation.\n Hormonal contraceptives may help prevent future ovarian cysts, but they are not thought to help with the resolution of existing cysts.\nABP Content Specifications(s)\n Recognize the association of small ovarian cysts with normal development\n Plan the appropriate diagnostic evaluation and management of ovarian cyst"}
{"id" : 1328, "question_text" : "You are seeing a 12-year-old transgender girl for a health supervision visit. The mother asks to speak with you privately. During the discussion, the mother expresses concern that her child has no friends because she is transgender. She asks you how she can best support her child. Of the following, your BEST advice for the mother is that she should", "options" : "[\"be aware of the increased risk of depression in transgender children\", \"encourage the child to delay gender expression until she is older\", \"encourage gender conformation surgery as soon as possible\", \"have the child treated for gender dysphoria with reparative therapy\", \"recognize that the child's transgender identification is likely a fad\"]", "explanation" : "Children who do not identify with any peers are at risk for psychological difficulty. Children may feel isolated for a multitude of reasons, including sexual orientation or gender identity. Sexual orientation refers to an individual's pattern of arousal by and attraction toward others, whereas gender identity refers to an individual's sense of being male, female, or neither. Since the existence of societal stigma resulting from homophobia and heterosexism, transgender youth have experienced higher rates of depression and suicidal ideation than their gender-conforming peers.\n\nMany children will experiment with gender expression and roles at a young age, but a pervasive, consistent, persistent, and insistent sense of being another gender is characteristic of transgender youth. Gender dysphoria is defined as a marked difference between an individual's experienced gender and his or her natal sex. The difference must be present for at least 6 months and cause clinically significant distress. More detailed interviewing of the adolescent in this vignette would be required to appropriately assess for gender dysphoria (Item C76 ).\n\nEncouraging the child in the vignette to delay gender expression may be interpreted as rejection. Rejection has been associated with depressive symptoms, self-harm, and suicidality.\n\nThe Endocrine Society clinical practice guideline for the treatment of transsexual persons recommends that the suppression of pubertal hormones start when the child first exhibits physical changes of puberty, but no earlier than sexual maturity ratings of 2 to 3. Initiation of cross-sex steroids for pubertal development of the desired opposite sex should begin at approximately 16 years of age. The Endocrine Society recommends deferring surgery until the individual is at least 18 years of age.\n\nReparative therapy involves techniques designed to convert one's sexual orientation from homosexual to heterosexual. The American Psychological Association and the American Psychiatric Association oppose such treatment.\n\nPREP Pearls\n• Adolescents who do not identify with any peers are at risk for psychological difficulty.\n• Gender dysphoria is defined as a marked difference between an individual's experienced gender and his or her natal sex. The difference must be present for at least 6 months and cause clinically significant distress.\n• The Endocrine Society clinical practice guideline for the treatment of transsexual persons recommends that the suppression of pubertal hormones start when the adolescent first exhibits physical changes of puberty, followed by initiation of cross-sex steroids for pubertal development of the desired opposite sex at approximately 16 years of age.\n\nABP Content Specifications(s)\n• Recognize the risks associated with adolescents who do not identify with any peers (\"loners\")\n\nSuggested Readings\n• American Academy of Pediatrics. Section on Lesbian, Gay, Bisexual, and Transgender Health and Wellness webinars. American Academy of Pediatrics website. https://www.aap.org/en-us/about-the-aap/Committees-Councils-Sections/solgbt/Pages/Education.aspx.\n• Committee on Adolescence. Policy statement: office-based care for lesbian, gay, bisexual, transgender, and questioning youth. Pediatrics. 2013;132(1):198-203. doi: http://dx.doi.org/10.1542/peds.2013-1282.\n• Hembree WC, Cohen-Kettenis P, Delemarre-van de Waal HA, et al. Endocrine treatment of transsexual persons: An Endocrine Society clinical practice guideline. J Clin Endocrinol Metab. 2009;94(9):3132-3154. doi: http://dx.doi.org/10.1210/jc.2009-0345.\n• Lopez X, Stewart S, Jacobson-Dickman E. Approach to children and adolescents with gender dysphoria. Pediatr Rev. 2016;37(3):89-98. doi: http://dx.doi.org/10.1542/pir.2015-0032."}
{"id" : 3733, "question_text" : "A 12-month-old girl is seen for evaluation of early hand preference. She was born at 39 weeks' gestation after an uncomplicated pregnancy to a gravida 1 para 1 mother via normal spontaneous vaginal delivery. The Apgar scores were 8 and 9 with a normal neonatal course. Her mother has noticed that she always reaches for toys with her right hand and that since shortly after birth she has had a tendency to hold her left hand in a closed fist with the arm tense against her side. She rolled at 5 months of age, sat independently at 7 months, and has pulled to stand since 10 months. Her mother has noticed that her left foot does not rest flat on the floor when she stands and that she tends to drag the left leg when cruising along furniture or crawling. She has a well-developed pincer grasp with her right hand but predominantly grasps objects with a raking motion with her left hand. Social-emotional and language development have been normal. No developmental regression has been appreciated. Her general physical examination findings are normal. Neurological examination is significant for hypertonia and hyperreflexia in the left arm and leg with normal cranial nerve and sensory examination findings. She tends to hold her left hand in a fist with the thumb adducted across the palm. She only reaches for objects with her left hand when her right arm is restrained; in this case, she uses a raking motion associated with some tremulousness. When cruising she tends to hold her left foot in a tip-toe position and drag the leg. She is referred to a pediatric neurology clinic where magnetic resonance imaging of the brain reveals right hemispheric closed-lip schizencephaly. Her mother returns for follow-up, tearful, because the neurologist diagnosed her daughter with a form of cerebral palsy. Of the following, this child's cerebral palsy is classified as", "options" : "[\"dyskinetic\", \"mixed\", \"spastic diplegia\", \"spastic hemiplegia\"]", "explanation" : "The patient in this vignette has a spastic hemiplegia secondary to schizencephaly (a congenital disorder of cortical migration). Malformations of cortical development have a broad range of clinical manifestations and severities including epilepsy, hemiparesis, early hand preference, global developmental delay, learning disability, or attention issues. In children suspected of having cerebral palsy (CP), magnetic resonance imaging (MRI) is recommended to evaluate for an underlying brain abnormality as part of the initial diagnostic evaluations. Abnormal MRI findings are seen in a majority of children with CP. Term infants are more likely to have cortical and grey matter lesions in comparison to preterm infants who are more likely to have periventricular white matter lesions. The presence of a brain malformation or normal MRI findings should prompt second-tier diagnostic testing including genetic and metabolic studies aimed at identifying an underlying etiology for the child's CP.\n\nCerebral palsy is a common group of conditions defined as a nonprogressive (static) genetic or acquired disorder of posture, tone, and/or movement secondary to injury or abnormality of the developing brain. The term encompasses a broad range of clinical conditions with variable presentations and severity. While there has been debate around the nomenclature and definition of CP, clinically the condition is classified by tone and limb involvement, which can allow for localization and guide diagnostic investigations aimed at identifying etiology and tailoring symptomatic management.\n\nIn patients with bilateral spasticity, there are 2 types of CP conditions: spastic diplegia, in which there is predominately spasticity of the legs, and spastic quadriparesis, in which spasticity affects all extremities. Spastic diplegia is most commonly seen in premature infants due to periventricular leukomalacia and often presents with gross motor delay. Spastic quadriparesis can result from a broad range of injuries including periventricular leukomalacia, brain malformations, prenatal and postnatal infections, traumatic brain injury, or anoxic injury and presents with prominent gross motor delay and often global developmental delay. Unilateral spasticity is seen in spastic hemiplegia and is often secondary to structural abnormalities, such as brain malformations, stroke, or vascular malformations. Patients often achieve the ability to ambulate independently and have normal intelligence with increased risk for seizures. A third category of CP is the extrapyramidal or dyskinetic type, typically affecting the arms more than the legs with various abnormal movements such as bradykinesia, choreoathetosis, hemiballismus, or dystonia. Although there are a variety of causes for dyskinetic CP, the 2 most common are neonatal and include hypoxic-ischemic encephalopathy and kernicterus. The least common types of CP, hypotonic and ataxic, are a result of a heterogenous group of disorders, most commonly nonprogressive genetic-metabolic disorders such as Prader-Willi syndrome or Angelman syndrome.\n\nClassification and identification of the etiology for a child's CP is critical for accurate diagnosis and treatment. The majority of clinicians will wait to establish the diagnosis until the child is aged 1 to 2 years to ensure the nonprogressive nature of the motor abnormality and account for normal variation in infant and child development. An accurate and complete history including a detailed developmental history and bedside assessment coupled with a thorough and complete neurological examination are useful in identifying prenatal and perinatal factors suggestive of a specific etiology. Failure to identify a specific etiology for the child's CP from the history, physical examination findings, or progression of symptoms should prompt investigations for CP mimics. These mimics include inborn errors of metabolism, neurodegenerative conditions, movement disorders, neoplasm, or hydrocephalus. These conditions share features at presentation with CP but may have specific treatments. For example, Segawa disease, an inherited dopamine responsive dystonia, presents with a diurnal variation predominantly affecting the lower extremities, thus mimicking spastic diplegia; however, Segawa disease is treatable with levodopa, resulting in rapid improvement in tone and return to normal motor function.\n\nPediatricians are uniquely positioned to assume a central role in the care of children with CP during both the diagnostic evaluation and long-term management. Through routine developmental surveillance and screening, early detection of tone abnormalities or delays in motor development can be identified, prompting further diagnostic evaluation that typically includes neuroimaging followed by genetic/metabolic testing. Once a diagnosis is established, focus shifts to initiation of intervention services such as therapies, educational services, and symptom management, allowing promotion of development. Children with CP can have associated difficulties with communication, swallowing/feeding, intellectual disability, seizures, mental health conditions, and respiratory disease. Part of their comprehensive care includes screening and identification of these common comorbidities to allow for initiation of appropriate care.\n\nPREP Pearls\n• Cerebral palsy is a common group of conditions defined as a nonprogressive (static) genetic or acquired disorder of posture, tone, and/or movement secondary to injury or abnormality of the developing brain.\n• Cerebral palsy is classified by the tone abnormality and limbs involved. Types of cerebral palsy include spastic (diplegia, quadriplegia, hemiplegia), dyskinetic, and hypotonic-ataxic.\n• Care of children with cerebral palsy includes spasticity management, establishment of intervention and educational services, and screening and treatment of common comorbidities including communication difficulties, intellectual disability, seizures, feeding difficulties, and respiratory disorders.\n\nABP Content Specifications(s)\n• Recognize the clinical features associated with cerebral palsy\n• Understand the prenatal risk factors associated with cerebral palsy\n\nSuggested Readings\n• Ashwal S, Russman BS, Blasco PA, et al. Practice parameter: diagnostic assessment of the child with cerebral palsy: report of the Quality Standards Subcommittee of the American Academy of Neurology and the Practice Committee of the Child Neurology Society. Neurology. 2004;62(6):851-863. doi:10.1212/01.WNL.0000117981.35364.1B.\n• Leach E, Shevell M, Bowden K, Stockler-Ipsiroglu S, van Karnebeek C. Treatable inborn errors of metabolism presenting as cerebral palsy mimics: systematic literature review. Orphanet J Rare Dis. 2014;9:197. doi:10.1186/s13023-014-0197-2.\n• Liptak G, Murphy N; Council on Children with Disabilities. Clinical report: providing a primary care medical home for children and youth with cerebral palsy. Pediatrics. 2011;128(5):e1321-e1329. doi:10.1542/peds.2011-1468.\n• Murphy N. Cerebral palsy. In: McInerny TK, Adam HM, Campbell DE, DeWitt TG, Foy JM, Kamat DM, eds. American Academy of Pediatrics Textbook of Pediatric Care. 2nd ed. Elk Grove Village, IL: American Academy of Pediatrics; 2017:1829-1835. Pediatric Care Online.\n• Noritz GH, Murphy NA; Neuromotor Screening Expert Panel. Motor delays: early identification and evaluation. Pediatrics. 2013;131(6):e2016-e2027. doi:10.1542/peds.2013-1056."}
{"id" : 3737, "question_text" : "A 6-year-old boy is seen for evaluation of ear pain and fever. He has had pain in the right ear for 2 days and fever as high as 39.5°C. He has had 3 previous episodes of otitis media. In the medical record, penicillin is listed as an allergy with a description of a rash for the allergic reaction. He has a temperature of 38.8°C, blood pressure of 97/57 mm Hg, heart rate of 110 beats/min, and respiratory rate of 20 breaths/min. Examination of the right ear is shown in Item Q184. The remainder of the physical examination findings are normal. Of the following, the antibiotic that is BEST suited for this boy's infection is", "options" : "[\"amoxicillin-clavulanate\", \"azithromycin\", \"cefdinir\", \"trimethoprim-sulfamethoxazole\"]", "explanation" : "Correct Answer: C\nThe antibiotic that is best suited for the boy in this vignette is cefdinir. The American Academy of Pediatrics guidelines for the management of otitis media list several cephalosporins, including cefdinir, as acceptable alternatives in the setting of penicillin allergy. Only 2% of individuals with a penicillin allergy are expected to react to cephalosporins, and less than 1% will react to carbapenems.\n\nIn the United States, up to 10% of the population is labeled as penicillin allergic. However, after allergy testing or challenge, less than 10% of individuals that report an antibiotic allergy are confirmed to be allergic. When a reported allergy is encountered, it is prudent to solicit detailed information of the purported reaction. Most rashes attributed to antibiotics are in fact viral in origin or caused by a drug-virus interaction. Patients that are labelled with drug allergies have higher drug prescriptions costs and higher rates of antibiotic-resistant infections as a result of being exposed to more broad-spectrum agents.\n\nPenicillins and cephalosporins are broadly characterized as β-lactam antibiotics. Other β-lactam antibiotics include carbapenems, monobactams, and β-lactamase inhibitors. β-Lactam refers to a structural feature, a 4-membered β-lactam ring, shared by all antibiotics in the class. There is a side chain that rises from the β-lactam ring called the R1 side chain. Penicillins and cephalosporins have R1 side chains, and the R1 side chain is a major determinant of cross-reactivity. In the setting of a penicillin allergy, as in the vignette, the risk of cross-reacting is minimized by choosing a cephalosporin with an R1 side chain that is different from the R1 side chain on the penicillin drug to which there is an allergy.\n\nβ-Lactam antibiotics inhibit enzymes used by bacteria for cell wall synthesis. Adverse effects of β-lactam antibiotics include gastrointestinal upset, diarrhea, and rashes. IgE-mediated hypersensitivity can occur, as well as delayed-onset reactions including Stevens-Johnson syndrome, acute interstitial nephritis, serum sickness, and drug-induced cytopenias. Amoxicillin-clavulanate is in the penicillin class and should be avoided if a drug allergy is suspected. Because of decreased susceptibility in Streptococcus pneumoniae isolates, azithromycin and trimethoprim-sulfamethoxazole should not be used to treat otitis media. Additionally, azithromycin has limited activity against Haemophilus influenzae, which is the causative agent in a significant number of otitis media cases.\n\nPREP Pearls\n• β-Lactam antibiotics, which include penicillins, cephalosporins, and carbapenems, inhibit enzymes used by bacteria for cell wall synthesis.\n• β-Lactam antibiotics can cause gastrointestinal upset, diarrhea, rashes, delayed-onset reactions (Stevens-Johnson syndrome, acute interstitial nephritis, serum sickness, and drug-induced cytopenias), and IgE-mediated hypersensitivity.\n• For a patient with penicillin allergy, use of a cephalosporin with a different R1 side chain minimizes the risk of a cross-reaction.\n\nABP Content Specifications(s)\n• Know the mechanism of action of penicillin and other beta-lactam antibiotics\n• Recognize the adverse effects associated with the use of various antibiotic drugs\n\nSuggested Readings\n• Lieberthal A, Carroll A, Chonmaitree T, et al. The diagnosis and management of acute otitis media. Pediatrics. 2013;131(3):e964-e999. doi:10.1542/peds.2012-3488.\n• Norton A, Konvinse K, Phillips E, Broyles A. Antibiotic allergy in pediatrics. Pediatrics. 2018;141(5):e20172497. doi:10.1542/peds.2017-2497.\n• Rosa-Olivares J, Porro A, Rodriguez-Varela M, Riefkohl G, Niroomand-Rad I. Otitis media: to treat, to refer, to do nothing: a review for the practitioner. Pediatr Rev. 2015;36(11):480-488. doi:10.1542/pir.36-11-480."}
{"id" : 1973, "question_text" : "The mother of a 6-month-old female infant is concerned that the infant has caught an upper respiratory tract infection from her older siblings. She describes that the infant is not eating well and is breathing fast. The infant is tachypneic with intermittent grunting and an absence of nasal congestion. She is afebrile with a heart rate of 180 beats/min, blood pressure of 80/60 mm Hg, respiratory rate of 60 beats/min, and an oxygen saturation as measured by pulse oximetry of 94%. Her lungs have diffuse crackles. A gallop is noted on auscultation of the heart. Her liver is palpable 4 cm below the costal margin. She is sent to the emergency department for monitoring. Of the following, the BEST next step in the management of this infant is", "options" : "[\"administer hypertonic saline per nebulizer\", \"administer normal saline (20 mL/kg)\", \"perform abdominal ultrasonography\", \"perform echocardiography\"]", "explanation" : "Correct Answer: D\nThe infant in this vignette has symptoms and physical examination findings that are consistent with myocardial dysfunction and congestive heart failure (CHF). Echocardiography should be the next step in evaluation and management to assess myocardial function and to evaluate for structural cardiac defects.\n\nThe etiologies of CHF can be divided into causes that occur with structural heart disease (Item C173A) and causes that occur with a structurally normal heart (Item C173B).\n\nThe clinical manifestations of CHF depend somewhat on the age of the patient and their level of activities. Infants, for example, \"exercise\" when eating. Heart failure in infancy frequently presents with feeding difficulties, distress with eating, or refusal to eat. Infants and young children often present with failure to thrive and can also have respiratory symptoms such as tachypnea, retractions, and grunting. Tachycardia, a murmur or gallop, and hepatomegaly are often seen. In older children, CHF can often present with exercise intolerance, manifest typically by difficulty keeping up with their peers. Respiratory symptoms and vomiting, which is reflective of decreased gut perfusion, are also common in this age group.\n\nThe other response choices would not be appropriate next steps in the management of this patient. Hypertonic saline nebulizer treatments are often used in the context of airway clearance of secretions. The infant in this vignette is not noted to have an important secretion burden. Although an infant or child with CHF may be dehydrated from vomiting or decreased oral intake, fluid must be given judiciously because these patients are in a volume-overloaded state. Thus, giving a 20-mL/kg normal saline bolus could be harmful. The hepatomegaly in this infant is caused by the CHF from volume overload of the right ventricle; thus, liver ultrasonography is unlikely to prove useful.\n\nPREP Pearls\n\nHeart failure can be caused by structural heart disease or occur with a normally structured heart.\n\nExercise intolerance, vomiting, and respiratory signs and symptoms are common presentations of heart failure.\n\nABP Content Specifications(s)/Content Area\n\nIdentify the causes of congestive heart failure in children of various ages\n\nRecognize the clinical findings associated with congestive heart failure in children of various ages\n\nSuggested Readings\n\nMadriago E, Silberbach M. Heart failure in infants and children. Pediatr Rev. 2010:31(1):4-12. doi: 10.1542/pir.31-1-4.\n\nSubramaniam S, Rutman M. Cardiogenic shock. Pediatr Rev. 2015:36(5):225-226. doi: 10.1542/pir.36-5-225."}
{"id" : 1958, "question_text" : "A 14-year-old adolescent boy is seen for a new concern about difficulty with exercise. His history is notable for seasonal allergic rhinitis with the recent development of perennial nasal symptoms. There is a strong family history of asthma, as well as a health and fitness orientation for the entire family. He has problems with cough and difficulty catching his breath after running more than a mile. He has been practicing for track competition season and running up to 5 miles at a time. His breathing problems start after 1 to 2 miles and persist for the rest of his run. He often has to stop running and walk for a while. One of his teammates has allowed him to use an albuterol inhaler before running, which has greatly improved his ability to run without discomfort. Results of pulmonary function testing are shown (Item Q158).\n\nOf the following, the BEST approach to the management of this patient's problem is", "options" : "[\"daily inhaled corticosteroid\", \"daily inhaled long-acting \\u03b2-agonist\", \"no medication\", \"pre-exercise inhaled bronchodilator\"]", "explanation" : "The patient in this vignette has exercise-related dyspnea that most likely is caused by bronchospasm. Onset of symptoms after several minutes of aerobic exercise in the context of a family history of asthma is strongly suggestive of exercise-induced asthma. In the face of normal baseline spirometry and no symptoms from other triggers, the appropriate therapy is pretreatment with inhaled bronchodilator. At least a therapeutic trial of albuterol is needed, with formal exercise testing recommended if he does not get adequate prevention of symptoms from albuterol administered before running. Because there is a clear association of symptoms with exercise and a reported response to albuterol, a lack of drug therapy would be inappropriate.\n\nThe patient does not appear to have persistent asthma as he has no symptoms other than with exercise and a normal baseline spirogram. Without a diagnosis or suspicion of persistent asthma, daily controller therapy is unnecessary at this point. However, because he appears to have allergies based on his perennial nasal symptoms superimposed on seasonal allergies, he is at risk for having persistent asthma and that should be an ongoing consideration.\n\nIt is not appropriate to use inhaled long-acting β-agonists as sole therapy for asthma. The only accepted use for long-acting β-agonists in the pediatric population is in combination with inhaled corticosteroids as step-up therapy for moderate or severe persistent asthma.\n\nPREP Pearls\n\nExercise may be one of many triggers in the child predisposed to asthma.\n\nExercise may be the only trigger for wheezing in some children.\n\nABP Content Specifications(s)/Content Area\n\nRecognize the clinical features associated with exercise-induced asthma\n\nSuggested Readings\n\nBoulet L-P, O'Byrne PM. Asthma and exercise-induced bronchoconstriction in athletes. N Engl J Med. 2015;372(7):641-648. doi: 10.1056/NEJMra1407552.\n\nTilles SA. Exercise-induced respiratory symptoms: an epidemic among adolescents. Ann Allergy Asthma Immunol. 2010;104(5):361-367. doi: 10.1016/j.anai.2009.12.008.\n\nWood PR, Hill VL. Practical management of asthma. Pediatr Rev. 2009;30(10):375-385. doi: 10.1542/pir.30-10-375."}
{"id" : 1785, "question_text" : "An 8-year-old girl is brought to the emergency department with a 2-day history of worsening nausea, abdominal pain, and vomiting. Her mother also reports significant fatigue and weight loss. She denies fever, headache, respiratory symptoms, or diarrhea. The girl's medical history is significant for severe persistent asthma, treated with fluticasone 220 μg 2 puffs inhaled twice daily and albuterol 4 puffs inhaled every 4 hours as needed. She recently completed a 2-week course of oral prednisolone for an asthma exacerbation. Her temperature is 37°C, blood pressure is 90/46 mm Hg, heart rate is 110 beats/min, respiratory rate is 16 breaths/min, and oxygen saturation is 98% on room air. She is tired-appearing. Her physical examination is significant for diffuse abdominal tenderness without rebound. Laboratory evaluation reveals the following: •White blood cell count, 8,000/μL (8 × 109/L) •Hemoglobin 12 g/dL (12 g/L) •Hematocrit, 38% •Platelets, 253 × 103/μL (253 × 109/L) •Sodium, 130 mEq/L (130 mmol/L) •Potassium, 3.9 mEq/L (3.9 mmol/L) •Chloride, 98 mEq/L (98 mmol/L) •Bicarbonate, 18 mEq/L (18 mmol/L) •Blood urea nitrogen, 25 mg/dL (8.9 mmol/L) •Creatinine, 0.9 mg/dL (80 μmol/L) •Glucose, 52 mg/dL (2.9 mmol/L) •Urinalysis: Specific gravity 1.025, pH 5, small ketones, no white blood cells, no red blood cells, no glucose Of the following, the MOST likely diagnosis for this girl is", "options" : "[\"adrenal insufficiency\", \"gastroenteritis\", \"hypothyroidism\", \"inappropriate secretion of antidiuretic hormone\", \"urinary tract infection\"]", "explanation" : "The girl in the vignette has secondary adrenal insufficiency due to hypothalamic-pituitary-adrenal (HPA) suppression by exogenous steroids. Chronic use of high-dose inhaled corticosteroids can cause HPA suppression. Furthermore, this girl was recently treated with 2 weeks of oral prednisolone. She has become symptomatic after steroid withdrawal, as the axis takes time to recover. The girl's fatigue, weight loss, nausea, vomiting, abdominal pain, hypotension, and tachycardia are characteristic of adrenal insufficiency.\n\nSalt craving and generalized skin hyperpigmentation can occur in primary adrenal insufficiency (pathology of the adrenal gland itself), because of mineralocorticoid deficiency and high adrenocorticotropic hormone (ACTH) levels, respectively. In contrast, with ACTH insufficiency, as is the case for the girl in the vignette, adrenal mineralocorticoid production remains sufficient because it is under control of the renin-angiotensin-aldosterone system.\n\nThe girl's hyponatremia, hypoglycemia, metabolic acidosis, and laboratory indicators of dehydration are also consistent with adrenal insufficiency. Hyperkalemia occurs in primary adrenal insufficiency because of mineralocorticoid deficiency. An ACTH level in this girl with secondary adrenal insufficiency would be low but would be high in the context of primary adrenal insufficiency. A cortisol level would be low in both cases.\n\nHypothyroidism and inappropriate secretion of antidiuretic hormone are potential causes of hyponatremia, but volume status would not be depleted in these conditions, as seen in the girl in the vignette. Gastroenteritis and urinary tract infection can present with nausea, vomiting, abdominal pain, and signs of dehydration, but the girl's history of steroid use makes adrenal insufficiency a more likely cause. The findings on complete blood count and urinalysis also make a urinary tract infection less likely.\n\nPREP Pearls\n• Long-term use of high-dose inhaled corticosteroids can cause hypothalamic-pituitary-adrenal axis suppression and significant signs and symptoms of adrenal insufficiency.\n• Hyponatremia, hypoglycemia, metabolic acidosis, and laboratory indicators of dehydration are consistent with adrenal insufficiency.\n• Hyperkalemia occurs in primary adrenal insufficiency because of mineralocorticoid deficiency.\n\nMOCA-Peds Objective\n• Recognize the complications of chronic corticosteroid therapy\n\nABP Content Specifications(s)\n• Differentiate the clinical and laboratory findings associated with adrenal insufficiency from those of the inappropriate secretion of antidiuretic hormone\n• Recognize the clinical and laboratory manifestations of adrenal insufficiency\n\nSuggested Readings\n• Auron M, Raissouni N. Adrenal insufficiency. Pediatr Rev. 2015;36(3):92–102; quiz 103, 129. http://dx.doi.org/10.1542/pir.36-3-92.\n• Kapadia CR, Nebesio TD, Myers SE, et al; Drugs and Therapeutics Committee of the Pediatric Endocrine Society. Endocrine effects of inhaled corticosteroids in children. JAMA Pediatr. 2016;170(2):163-170."}
{"id" : 369, "question_text" : "A 14-year-old boy presents for a preparticipation sports evaluation for baseball. He plays shortstop. His mother is very concerned about his playing because of the injuries she has heard about in professional and collegiate athletes. You explain to her that appropriate equipment, including a batting helmet, is needed to provide protection for her son. Of the following, the LARGEST percentage of baseball injuries can be prevented by also using", "options" : "[\"a mouth guard\", \"a protective cup\", \"elbow pads\", \"knee pads\", \"polycarbonate goggles\"]", "explanation" : "More than 50% of all high school students participate in athletics, and injury prevention should be a mainstay of sports participation for youth. Both parents and coaches should provide and insist on the use of equipment and safety rules to prevent injury in young athletes. Mouth injuries, along with other head injuries, account for the majority of injuries (48%) sustained by youth in baseball. Injuries generally are caused by contact with sports equipment (eg, the bat, the ball, and the base). Most serious injuries result from being struck by a batted ball and are more common among infield players. Other injuries include those to the leg and groin as well as the chest. Although these are uncommon, use of a protective cup in all sports is recommended to prevent testicular injury.\n\nDental and facial injuries may be prevented best by using a mouth guard both in the field and at the plate in baseball to avoid injury by pitched and batted balls. Plastic and metal helmets with face protection have been available for batters for several years but are used uncommonly in high school athletics. Clearly, use of a helmet with face protection is important to prevent cranial injuries for the catcher. Even with the use of a helmet, mouth guards protect further against injuries of teeth and from teeth to the oral mucosa. The American Association of Orthodontists recommends that mouth guards be used for the following sports: baseball, football, soccer, basketball, wrestling, softball, ice and field hockey, volleyball, and lacrosse.\n\nElbow and knee pads may be helpful in prevention of abrasions and other minor injuries, but they are unlikely to prevent serious injury. Polycarbonate goggles are recommended for batting, but evidence for their routine use in fielding is lacking. Eye protection is afforded by most helmets with face protection.\n\nCritique: More than 50% of all high school students participate in athletics, and injury prevention should be a mainstay of sports participation for youth. Both parents and coaches should provide and insist on the use of equipment and safety rules to prevent injury in young athletes. Mouth injuries, along with other head injuries, account for the majority of injuries (48%) sustained by youth in baseball. Injuries generally are caused by contact with sports equipment (eg, the bat, the ball, and the base). Most serious injuries result from being struck by a batted ball and are more common among infield players. Other injuries include those to the leg and groin as well as the chest. Although these are uncommon, use of a protective cup in all sports is recommended to prevent testicular injury.\n\nContent Specifications: Know the indications for the use of mouth guards in athletics"}
{"id" : 1995, "question_text" : "A medical student rotating on the inpatient pediatric ward is learning about medical decision-making, including balancing risks and benefits and cost effective care. The roles of the physician's experiences, parental preferences, and study data in medical decision-making are discussed. The student asks how the risk-benefit ratio for treatment of any individual patient might be determined. Of the following, the statistical concept that BEST addresses the student's question is", "options" : "[\"number needed to treat\", \"paired t test\", \"power\", \"sample size\"]", "explanation" : "The number needed to treat (NNT) analysis helps providers determine the risk-benefit ratio for an individual patient for a specific therapy. The paired t test, power, and sample size do not specifically address this concept.\n\nThe NNT is the number of patients needed to be treated to prevent 1 additional adverse event. The formula used to calculate NNT is:\n\nNNT = 1 ÷ Absolute Risk Reduction\n\nAbsolute risk reduction is the difference in the risk of adverse outcomes between the study population and a control group. The solution to this formula should be rounded to a whole number.\n\nData from studies can lead an investigator to an incorrect conclusion, whether due to a random sample not actually being reflective of the general population or the presence of bias. When the sample is not representative of the general population, type 1 or type 2 errors can occur. A type 1 error or false-positive, occurs when an investigator rejects the null hypothesis when it is actually true. A type 2 error, or false-negative, occurs when an investigator fails to reject the null hypothesis that is actually false. Type 2 error is inversely related to the power of a study. The larger the sample size, the less likely type 1 or type 2 errors will occur.\n\nPREP Pearls\n\nThe number needed to treat analysis helps providers determine the risk-benefit ratio for an individual patient for a specific therapy.\n\nThe number needed to treat, which is calculated as 1 ÷ Absolute Risk Reduction, is the number of patients needed to be treated to prevent 1 additional adverse event.\n\nABP Content Specifications(s)/Content Area\n\nDistinguish between type I and type II statistical errors\n\nUnderstand the concept of number-needed-to-treat when utilized to describe therapeutic interventions\n\nSuggested Readings\n\nBanerjee A, Chitnis UB, Jadhav SL, Bhawalkar JS, Chaudhury S. Hypothesis testing, type 1 and type 2 errors. Ind Psychiatry J. 2009;18(2):127-131. doi: 10.4103/0972-6748.62274.\n\nNuovo J, Melnikow J, Chang D. Reporting number needed to treat and absolute risk reduction in randomized controlled trials. JAMA. 2002:287(21):2813-2814.\n\nTschudy MM, Rowe PC. Research and statistics: Number needed to treat and intention to treat analysis. Pediatr Rev. 2010;31(9):380-382. doi: 10.1542/pir.31-9-380."}
{"id" : 2085, "question_text" : "A 15-year-old adolescent boy is seen for a sports preparticipation examination prior to starting cross-country track. He is concerned that his puberty is not progressing normally. His medical history is significant only for right orchidopexy at the age of 10 months. There is no family history of delayed puberty. A comprehensive review of systems is significant for the inability to smell strong odors. He runs an average of 10 miles per week. He has a blood pressure of 102/64 mm Hg and a heart rate of 68 beats/min. His weight is at the 25th percentile, height is at the 50th percentile, and body mass index is at the 20th percentile. His sexual maturity rating is grade 3 for pubic hair and grade 1 for genital development. He does not have axillary hair or facial hair. The remainder of his physical examination findings are unremarkable.\n\nOf the following, the MOST likely diagnosis is", "options" : "[\"constitutional delay of puberty\", \"functional hypogonadism\", \"Kallmann syndrome\", \"Klinefelter syndrome\"]", "explanation" : "Correct Answer: C\nThe patient in this vignette has delayed puberty caused by Kallmann syndrome, which is hypogonadotropic hypogonadism associated with anosmia or hyposmia, the lack of or reduced ability to smell. The association results from disrupted migration of gonadotropin-releasing hormone and olfactory neurons in the developing brain. The patient's history of cryptorchidism was an early indicator of hypogonadism. Gonadotropin (luteinizing hormone [LH], follicle-stimulating hormone [FSH]) and testosterone levels are low in Kallmann syndrome. The patient's testes are prepubertal in size (sexual maturity rating 1) because of the lack of gonadotropin stimulation. His pubic hair development is a result of adrenal androgen production (adrenarche), which occurs normally in Kallmann syndrome.\n\nConstitutional delay of puberty is the most common cause of delayed puberty, especially in boys. Short stature is commonly associated with constitutional delay, and adrenarche is also delayed. The pubertal growth spurt occurs later, duration of growth is longer, and final adult height is usually in the normal range. There is often a family history of delayed puberty in individuals who are affected. The adolescent boy in this vignette has normal stature, normal timing of adrenarche, and no family history of constitutional delay, making this diagnosis less likely.\n\nFunctional hypogonadism caused by excessive exercise may be considered for the patient in this vignette. However, his body mass index is normal and his clinical features are more consistent with Kallmann syndrome. Other common causes of functional hypogonadism include eating disorders and chronic systemic disease.\n\nKlinefelter syndrome (47,XXY karyotype) is another cause of delayed puberty in boys. The delayed puberty is caused by primary testicular failure (primary hypogonadism). Small, firm testes on physical examination are characteristic. Adrenarche occurs normally, so pubic hair is usually present at the expected time. Other features of Klinefelter syndrome include language delay, learning problems, and tall stature with disproportionately long legs. Although the physical examination findings of the patient in this vignette can be consistent with Klinefelter syndrome, his history of anosmia makes Kallmann syndrome more likely.\n\nPuberty is considered delayed if there is no breast development by age 13 years in girls or if there is a lack of testicular growth to at least 4 mL in volume or 2.5 cm in length by age 14 years in boys. The etiologies of delayed puberty can be classified based on gonadotropin (LH, FSH) levels. In constitutional delay, gonadotropin levels are low or early pubertal. Gonadotropin levels are also low in functional hypogonadism and hypogonadotropic hypogonadism. Hypogonadotropic hypogonadism may be isolated, as in Kallmann syndrome, or associated with other pituitary hormone deficiencies. Hypogonadotropic hypogonadism can be acquired when associated with a central nervous system abnormality, such as a craniopharyngioma.\n\nGonadotropin levels are elevated in hypergonadotropic hypogonadism. Etiologies of hypergonadotropic hypogonadism include Klinefelter syndrome (47,XXY) in male individuals, Turner syndrome (45,X and variants) and autoimmune ovarian failure in female individuals, and gonadal toxicity (eg, from chemotherapy or radiation).\n\nThe evaluation of an adolescent with delayed puberty should include a complete history and physical examination with special attention to signs and symptoms of underlying systemic disease, sense of smell, nutrition, exercise, history of cryptorchidism, and family history of pubertal timing. Examination of the growth charts, body mass index percentile, sexual maturity ratings, and testicular size and consistency should be performed. Features associated with Turner or Klinefelter syndrome should be identified. A bone age radiograph can be helpful because bone age often correlates better with pubertal status than with chronologic age. In constitutional delay, bone age can help predict catch-up growth, which is often a concern. Initial laboratory evaluation should include gonadotropins (LH, FSH) and either estradiol for girls or testosterone for boys. Other laboratory testing should be done as indicated by history and physical examination findings. Brain magnetic resonance imaging is indicated for unexplained hypogonadotropic hypogonadism or if a central nervous system abnormality is suspected.\n\nDelayed puberty can cause significant psychosocial stress. For adolescents with constitutional delay and significant stress, a short course of sex steroid may trigger pubertal onset. Individuals with permanent hypogonadism require long-term treatment with sex steroid. For individuals with functional hypogonadism, correction of the underlying problem results in pubertal progression.\n\nPREP Pearls\n\nPuberty is considered delayed if there is no breast development prior to age 13 years in girls or if there is a lack of testicular growth to at least 4 mL in volume or 2.5 cm in length prior to age 14 years in boys.\n\nMajor categories of delayed puberty include constitutional delay, functional hypogonadism, hypogonadotropic hypogonadism, and hypergonadotropic hypogonadism.\n\nGonadotropin (luteinizing hormone, follicle-stimulating hormone) levels are important for narrowing the differential diagnosis of delayed puberty.\n\nABP Content Specifications(s)/Content Area\n\nRecognize the clinical features associated with a delay in sexual maturation of various causes\n\nIdentify the causes of delayed puberty\n\nUnderstand the relationship between bone age and chronologic age\n\nRecognize the psychosocial risks associated with delayed puberty\n\nPlan the appropriate evaluation of an adolescent boy or girl who has no signs of the onset of puberty\n\nSuggested Readings\n\nKaplowitz PB. Delayed puberty. Pediatr Rev. 2010;31(5):189-195. doi: 10.1542/pir.31-5-189.\n\nUS National Library of Medicine. Kallmann syndrome. Genetics Home Reference website. https://ghr.nlm.nih.gov/condition/kallmann-syndrome.\n\nWolf RM, Long D. Pubertal development. Pediatr Rev. 2016;37(7):292-300. doi:10.1542/pir.2015-0065."}
{"id" : 1326, "question_text" : "A male infant is brought to your office for a health supervision visit. He was born at term and has been healthy since birth. While performing the physical examination, you assess the developmental milestones he has achieved. The infant smiles responsively and vocalizes with vowel sounds, but does not squeal or laugh. He can track an object horizontally to midline and sometimes past midline. You note a mild head lag when you pull him to the sitting position from supine, and he makes an effort to hold his head midline when held upright. When placed in the prone position, he is able to lift his head up to 45 degrees. The infant's hands are open and relaxed much of the time, and he will hold an object placed in his hand. He is not yet reaching for objects, nor bringing his hands to the midline. Of the following, the age that BEST matches this infant's developmental abilities is", "options" : "[\"1 month\", \"2 months\", \"3 months\", \"4 months\", \"2 weeks\"]", "explanation" : "The infant described in the vignette exhibits the cognitive/behavioral and motor milestones typically attained by 2 months of age: social smile, cooing, visually tracking an object past midline, diminishing head lag, and disappearance of the grasp reflex. Smiling responsively rather than smiling spontaneously is a key milestone to observe at 2 months of age.\n\nMost infants can vocalize with vowels or coo by 2 months of age. Language then progresses with laughing beginning closer to 3 months of age and squealing at 4 months of age. The addition of consonant sounds, or babbling, is typical of a 6-month-old infant, followed by polysyllabic babbling at 9 months of age.\n\nVisual receptive skills progress from the newborn's ability to fix on the mother's face to following objects to midline at 1 month of age, past midline at 2 months of age, and 180 degrees at 3 to 4 months of age.\n\nThe progression of head control is a clinical measure of early gross motor skills. A 2-week-old neonate will exhibit poor head control. Mild head lag when pulled to sitting position from supine, the ability to lift the head to 45 degrees when prone, and the attempt to hold the head erect when upright is typical of a 2-month-old infant. By 3 months of age, the head lag should be minimal when pulled to sitting and most infants will be able to lift the head up to 90 degrees when prone. A 4-month-old infant can lift both the head and chest when prone.\n\nThe attainment of fine motor skills is exhibited through the progression of hand skills. Newborns' hands are flexed and fisted most of the time. The involuntary grasp reflex disappears at 2 months of age, allowing the infant to hold an object placed in the hand. Reaching for and swiping at a toy occurs at 3 months of age and is followed by voluntary grasp at 4 months. Bringing hands together in the midline is noted at 3 to 4 months of age, with progression to transferring objects from hand to hand at 6 months of age.\n\nThe infant described in the vignette has more advanced milestones than would be expected at 2 weeks or 1 month of age. At 1 month of age, the infant would demonstrate the ability to lift his head only slightly, track visually to midline but not beyond, and smile spontaneously but not socially. At 3 months of age, the infant should have more advanced visual receptive skills with the ability to follow for a full 180 degrees and in a circular motion; plus, reaching for objects and stronger head control should be evident. By 4 months of age, an infant should be laughing, squealing, grasping objects voluntarily, and lifting both the chest and head when prone.\n\nPREP Pearls\n• Most 2-month-old infants will smile responsively, vocalize with vowels (cooing), visually track an object past midline, hold an object placed in their hand, lift their head to 45 degrees when prone, and attempt to hold their head erect when held upright.\n\nABP Content Specifications(s)\n• Evaluate the cognitive and behavioral developmental progress/status of an infant at 2 months of age, including recognition of abnormalities\n• Evaluate the motor developmental progress/status of an infant at 2 months of age, including recognition of abnormalities\n\nSuggested Readings\n• Bickley LS. Bates' Guide to Physical Examination and History Taking. 11th ed. Philadelphia, PA: Lippincott Williams and Wilkins; 2012:1024 pp.\n• Feigelman S. The first year. In: Kliegman RM, Stanton BF, St Geme JW III, Schor NF, Behrman RE, eds. Nelson Textbook of Pediatrics. 20th ed. Philadelphia, PA: Saunders Elsevier; 2015:65-69.\n• Feigelman S. The second year. In: Kliegman RM, Stanton BF, St Geme JW III, Schor NF, Behrman RE, eds. Nelson Textbook of Pediatrics. 20th ed. Philadelphia, PA: Saunders Elsevier; 2015:70-75.\n• Schonhaut L, Armijo I, Pérez M. Gestational age and developmental risk in moderately and late preterm and early term infants. Pediatrics. 2015;135(4):e835-e841. doi: http://dx.doi.org/10.1542/peds.2014-1957.\n• Stein REK. Are we on the right track? Examining the role of developmental behavioral pediatrics. Pediatrics. 2015;135(4):589-591. doi: http://dx.doi.org/10.1542/peds.2014-3274.\n• Weitzman C, Wegner L, Section on Developmental and Behavioral Pediatrics, Committee on Psychosocial Aspects of Child and Family Health, Council on Early Childhood, Society for Developmental and Behavioral Pediatrics. Promoting optimal development: screening for behavioral and emotional problems. Pediatrics. 2015;135(2):384-395. doi: http://dx.doi.org/10.1542/peds.2014-3716."}
{"id" : 3172, "question_text" : "A 13-year-old adolescent girl who was treated successfully for leukemia with chemotherapy and cranial irradiation at 2 years of age now presents for evaluation of a \"lump\" on the side of her neck. She also reports an increase in fatigue over the last few months, as well as a 3.2-kg weight gain. She denies any skin changes, temperature intolerance, or changes in school performance. On physical examination, you note a firm, hard, fixed mass over the left thyroid. Her thyroid gland also appears to be enlarged. Of the following, the BEST test to determine the underlying cause of this girl's abnormal physical finding is", "options" : "[\"fine-needle aspiration thyroid biopsy\", \"iodine uptake scan\", \"thyroid peroxidase antibody\", \"thyroid ultrasonography\", \"thyrotropin\"]", "explanation" : "Preferred Response: A\nThe child in the vignette has a history of radiation exposure and a thyroid nodule. The best test to determine if the nodule is benign or malignant is fine-needle aspiration (FNA) biopsy. Sometimes FNA results are inconclusive, and surgical removal of part or all of the thyroid becomes necessary. Fine-needle aspiration is most often performed under ultrasonographic guidance.\n\nAll patients with a thyroid nodule require ultrasonography to assess for features of thyroid malignancy to determine if there are nonpalpable lesions that may also warrant FNA and to assess the lymph nodes in the central and lateral neck_ Given this patient's history of radiation exposure to the neck and the presence of a palpable nodule, FNA is still needed and is the only test offered that can give a definitive diagnosis.\n\nA '\"iodine uptake scan can be used to determine if the nodule is hot (hyperactive) or cold (inactive). In this case, '\"iodine uptake should only be performed if the child's thyrotropin (TSH) level is at the bottom of the normal range or actually suppressed. Given the patient had no signs of hyperthyroidism, this is unlikely.\n\nRadiation exposure can also lead to autoimmune disease (Hashimoto disease), and given the child's thyroid enlargement, weight gain, and fatigue, checking thyroid peroxidase antibodies to look for autoimmune disease is reasonable. In addition, measurement of thyrotropin levels is recommended. It is important to verify that thyrotropin levels are not suppressed. Thyroid nodules and thyroid cancer are also more common in patients with high-normal or elevated thyrotropin, especially in those with a history of prior radiation exposure. However, an abnormal thyrotropin level would not diagnose the underlying cause of this patient's thyroid nodule. For that diagnosis, FNA is needed.\n\nFor low-risk patients (those with no history of radiation therapy to the neck and no family history of thyroid cancer) in whom a thyroid nodule is found incidentally, ultrasonography should be done first and FNA would be warranted for nodules greater than or equal to 1 cm in size or for any concerning characteristics seen in the nodule on imaging studies.\n\nPREP Pearls\n• A history of radiation exposure increases the risk of thyroid malignancy later in life.\n• The best test to assess for malignancy in the evaluation of a discrete thyroid nodule in a high-risk patient is fine-needle aspiration.\n\nAmerican Board of Pediatrics Content Specification(s)\n• Recognize the clinical features associated with a thyroid cyst/tumor\n• Plan the appropriate evaluation and management of a thyroid mass/ nodule\n\nSuggested Reading\n• American Thyroid Association (ATA) Guidelines Taskforce on Thyroid Nodules and Differentiated Thyroid Cancer, Cooper DS, Doherty GM, et al. Revised American Thyroid Association management guidelines for patients with thyroid nodules and differentiated thyroid cancer. Thyroid. 2009;19(11):1167-1214. doi:10.1089/thy.2009.0110.\n• Rosenfeld RG, Cohen P. Disorders of growth hormone/insulin-like growth factor secretion and action. In: Sperling MA, ed. Pediatric Endocrinology. 3rd ed. Philadelphia. PA: Saunders Elsevier; 2008:254-334.\n• Waguespack SG, Francis G. Initial management and follow-up of differentiated thyroid cancer in children. J Natl Compr Canc Netw. 2010;(8)11:1289-1300."}
{"id" : 2082, "question_text" : "A 4-year-old previously healthy, unimmunized Alaskan native girl is brought to the emergency department with a 1-day history of fever and a 4-hour history of drooling and respiratory distress. There are no sick contacts, no pets at home, and no recent travel. She has not received routine childhood vaccinations for philosophical reasons. She appears ill and has a temperature of 40°C, heart rate of 126 beats/min, and a respiratory rate of 32 breaths/min. Her blood pressure is normal. There is inspiratory stridor. The remainder of the physical examination findings are unremarkable. She is evaluated by an otolaryngologist and then undergoes urgent intubation in the operating room (Item Q284).\n\nOf the following, the BEST initial antimicrobial treatment is", "options" : "[\"ampicillin\", \"ceftriaxone\", \"clindamycin\", \"vancomycin\"]", "explanation" : "Correct Answer: B\nThe toxic-appearing, unimmunized young girl in this vignette, with acute onset of fever, inspiratory stridor, drooling, and severe respiratory distress, and evidence of markedly swollen epiglottis during endotracheal intubation, has the classic presentation of epiglottitis caused by Haemophilus influenzae type b (Hib) infection. Of the choices listed, the best initial antimicrobial treatment for this patient is ceftriaxone, a third-generation cephalosporin.\n\nAcute epiglottitis is a medical emergency, and diagnosis is based on clinical criteria. For a toxic-appearing child with respiratory distress, securing the airway is a key priority. Interventions such as examination of the oropharynx, blood collection, intravenous line initiation, or neck radiography are not recommended, given the risk of potential respiratory arrest. Collaboration between the emergency department physician or pediatrician with the otolaryngologist and anesthesiologist is vital. The child should be transported to the operating room under the direct supervision of health care providers with expertise in airway management. Establishment of a secure airway as the first priority in cases of epiglottitis with impending airway obstruction is the standard of care.\n\nAntimicrobial therapy is recommended for acute epiglottitis, but should be started only after securing the airway. Empiric antimicrobial therapy must cover Hib (especially in unimmunized children) and other potential pathogens (GAS, pneumococcus, and S aureus). Approximately 30% to 40% of H influenzae strains seen in the United States produce β-lactamase. Therefore, a third-generation cephalosporin (eg, cefotaxime or ceftriaxone) would be the preferred initial therapy for the suspected case of Hib epiglottitis illustrated in this vignette. A third-generation cephalosporin would also treat infection caused by GAS and pneumococcus.\n\nAntistaphylococcal agents, such as vancomycin and clindamycin, may be added if S aureus epiglottitis is suspected (eg, trauma to the epiglottis). Ampicillin alone would not be an appropriate empiric antibiotic choice. The antibiotic regimen may be tailored based on isolation of an organism on culture (obtained at the time of direct laryngoscopy and endotracheal intubation) and antimicrobial susceptibility. The recommended duration of intravenous antibiotic treatment for Hib epiglottitis ranges from 7 to 10 days. Droplet precautions are indicated for hospitalized patients with invasive Hib disease until 24 hours after starting appropriate antibiotic therapy.\n\nRifampin chemoprophylaxis is recommended for all household contacts of children with invasive Hib disease, in the setting of at least 1 unimmunized or incompletely immunized contact younger than 48 months of age. In contrast, rifampin chemoprophylaxis is recommended for all household contacts of children with invasive Hib disease in the setting of at least 1 household contact who is immunocompromised, regardless of that contact's age or immunization status. Chemoprophylaxis is also recommended for preschool and child care center contacts of children with invasive Hib disease if 2 or more cases of invasive Hib disease occur at that site within 60 days. Chemoprophylaxis is not recommended for index patients with invasive Hib disease treated with cefotaxime or ceftriaxone. Chemoprophylaxis is also not recommended for contacts of children with invasive non–type b or nontypeable H influenzae disease, 24 hours after treatment with cefotaxime or ceftriaxone.\n\nPREP Pearls\n\nAcute epiglottitis results from inflammation and swelling of the supraglottis, with a potential to cause serious, life-threatening illness due to upper airway obstruction.\n\nHaemophilus influenzae type b is the most frequent cause of epiglottitis in unimmunized children; a third-generation cephalosporin (eg, ceftriaxone or cefotaxime) is the treatment of choice.\n\nAcute epiglottitis is a medical emergency. In a toxic-appearing child with respiratory distress, securing the airway is a key priority.\n\nABP Content Specifications(s)/Content Area\n\nPlan appropriate prophylaxis for individuals exposed to invasive Haemophilus influenzae type B\n\nUnderstand the epidemiology of Haemophilus influenzae infection\n\nPlan the appropriate management of a typable and nontypable Haemophilus influenzae infection\n\nRecognize the clinical features associated with typable and nontypable Haemophilus influenzae infection\n\nSuggested Readings\n\nAmerican Academy of Pediatrics. Haemophilus influenzae infections. In: Kimberlin DW, Brady MT, Jackson MA, Long SS, eds. Red Book: 2018 Report of the Committee on Infectious Diseases. 31st ed. Itasca, IL: American Academy of Pediatrics; 2018:367-375.\n\nButler DF, Myers AL. Changing epidemiology of Haemophilus influenzae in children. Infect Dis Clin North Am. 2018;32(1):119-128. doi:10.1016/j.idc.2017.10.005.\n\nD'Agostino J. Pediatric airway nightmares. Emerg Med Clin North Am. 2010;28(1):119-126. doi:10.1016/j.emc.2009.09.005."}
{"id" : 2662, "question_text" : "A 1-month-old infant is seen for a health supervision visit. Her parents are concerned that she breathes faster while eating. She takes 1 to 2 ounces of standard infant formula every 2 to 3 hours and is sleeping well between feedings. The infant was born at term. There were no complications during pregnancy or the delivery, and she was discharged from the hospital 24 hours after birth. She has had consistent growth along the 50th percentile for weight and length. On physical examination, she has a heart rate of 150 beats/min, respiratory rate of 60 breaths/min, holosystolic murmur at the lower left sternal border, and liver edge palpable 3 cm below the right costal margin. The remainder of her physical examination findings are normal. Of the following, this infant's MOST likely diagnosis is", "options" : "[\"aortic stenosis\", \"atrial septal defect\", \"pulmonary stenosis\", \"ventricular septal defect\"]", "explanation" : "The infant in the vignette has tachypnea while feeding, hepatomegaly, and a holosystolic murmur at the lower left sternal border. These findings are consistent with a ventricular septal defect (VSD). The flow across an atrial septal defect (ASD) itself does not create a murmur, though the increased flow can result in a fixed split S2 and produce a murmur across the pulmonary valve, which would be heard in mid-systole at the left upper sternal border. Pulmonary valve stenosis would produce a systolic ejection murmur in the pulmonic location. An aortic stenosis murmur would be heard in mid-systole, loudest at the right upper sternal border.\n\nAs the pulmonary vascular resistance decreases through the first 1 to 2 months after birth, the communication between the right and left ventricles from a VSD results in a left-to-right shunt. Pulmonary blood flow increases as blood leaves the left ventricle, enters the right ventricle, and then enters the lungs. Symptoms and signs of this increased pulmonary blood flow include tachypnea, sweating with feedings, decreased oral intake, poor weight gain, tachycardia, and hepatomegaly. On cardiac auscultation, a VSD murmur is heard throughout all of systole (holosystolic) at the lower left sternal border. Its pitch is determined by the size of the defect, with larger defects creating a lower pitch.\n\nA VSD large enough to cause symptoms (typically moderate to large) requires closure. Ventricular septal defects are usually closed surgically, though some are closed via a transcatheter approach. Diuretics can provide symptom improvement while children are awaiting surgical repair. If the VSD is left open, over time, the pulmonary vascular resistance increases due to the excess pulmonary blood flow leading to irreversible pulmonary hypertension (Eisenmenger syndrome).\n\nPREP Pearls\n• A ventricular septal defect creates a holosystolic murmur heard best at the left lower sternal border.\n• Symptoms and signs of a moderate to large ventricular septal defect include tachypnea, sweating while feeding, tachycardia, and hepatomegaly.\n• A moderate to large ventricular septal defect requires surgical closure and if left open can lead to irreversible pulmonary hypertension (Eisenmenger syndrome).\n\nABP Content Specifications(s)\n• Understand the natural history of ventricular septal defect\n\nSuggested Readings\n• Backer CL, Eltayeb O, Monge MC, Mazwi ML, Costello JC. Shunt lesions part I: patent ductus arteriosus, atrial septal defect, ventricular septal defect, and atrioventricular septal defect. Pediatr Crit Care Med. 2016;17(8 suppl 1):S302S309. doi:10.1097/PCC.0000000000000786.\n• Madriago E, Silberbach M. Heart failure in infants and children. Pediatr Rev. 2010;31(1):4-12. doi:10.1542/pir.31-1-4.\n• McCulloch MA, Gajarski RJ. Congenital and acquired heart disease. In: McInerny TK, Adam HM, Campbell DE, DeWitt TG, Foy JM, Kamat DM, eds. American Academy of Pediatrics Textbook of Pediatric Care. American Academy of Pediatrics; 2021:chap 234. Pediatric Care Online.\n• McDaniel NL. Ventricular and atrial septal defect. Pediatr Rev. 2001;22(8):265-270. doi:10.1542/pir.22-8-265."}
{"id" : 310, "question_text" : "A 15-month-old boy bangs his head when he gets upset and does not get his way as well as when he falls asleep in his crib. He currently says about five words and is ambulating independently. His parents are concerned that he will seriously hurt himself and are puzzled about how to decrease this behavior. Of the following, the MOST appropriate next step is to", "options" : "[\"have the child fitted for a soft helmet\", \"have the parents hold him when he begins to bang his head\", \"instruct the parents to ignore the behavior\", \"monitor the child for possible autism\", \"refer the boy for an early intervention evaluation\"]", "explanation" : "The boy described in the vignette is banging his head because of frustration and possibly as a relaxation or comfort measure at bedtime. As a result, his parents should be instructed to ignore the behavior. They should not pick him up and hold him, which could reinforce the behavior. The use of a soft helmet may be protective for a child who bangs his or her head continuously, but it does not diminish the behavior. Children who have autism or other developmental delays may bang their heads or display other self-stimulatory behaviors, but because this boy has no signs of developmental delay, there is no indication to either refer him for an early intervention evaluation or monitor him for autism.\n\nHead banging may occur in about 25% of infants and toddlers, tends to be more common in boys, and peaks by about 18 to 24 months of age. Most normally developing children outgrow the habit by age 3 years. Head banging may relax or comfort the child and, therefore, may be performed as a toddler falls asleep or wakes during the night. Children who are teething may also bang their heads, possibly to distract themselves from the pain. Head banging also may occur when a child is frustrated or during a tantrum. Some children may bang their heads to gain attention, particularly if the parents have a strong reaction to the behavior.\n\nCritique: Preferred Response: C\n\nContent Specifications: Recognize that head banging does not indicate a sensory deficit"}
{"id" : 2974, "question_text" : "An otherwise healthy, 4-month-old male infant with a hemangioma is scheduled to start propranolol treatment. Possible complications of treatment are discussed with his parents. Of the following, the MOST likely complication is", "options" : "[\"apnea\", \"hypertension\", \"hypoglycemia\", \"tachycardia\"]", "explanation" : "The infant in the vignette is about to start receiving propranolol for treatment of a hemangioma. Of the response choices listed, only hypoglycemia is a known adverse effect of β-blocking drugs. β-Blockers work on β receptors to inhibit the binding of the neurotransmitters norepinephrine and epinephrine. There are three different kinds of β receptors: β1 receptors are on the heart and kidneys; β2 receptors are on the lungs, gastrointestinal tract, liver, uterus, vascular smooth muscle, and skeletal muscle; and β3 receptors are in fat cells.\n\nTypes of β-blocking drugs differ according to the β receptors that they block. Some are nonselective (eg, propranolol) and block both β1 and β2 receptors, whereas others are selective (eg, metoprolol), blocking only β1. Mechanism of action and adverse effects of β-blockers are based on the locations of these receptors and the type of β-blocker administered (nonselective versus selective). Adverse effects could include bradycardia, conduction disturbances (eg, atrioventricular block), hypotension, bronchospasm, and hypoglycemia.\n\nBefore initiating β-blocker therapy, the clinician should obtain a thorough cardiopulmonary history and ask the child's parents or caregivers about poor feeding, exercise intolerance, dyspnea, tachypnea, diaphoresis, wheezing, heart murmur, and family history of heart block or arrhythmia. Additionally, the clinician should perform a thorough cardiopulmonary examination, paying specific attention to heart rate, blood pressure, and auscultation. Some have advocated for routine pretreatment electrocardiography, although the consensus statement by Drolet et al recommends obtaining an electrocardiogram only for those with a lower-than-normal heart rate for age; a family history of congenital heart conditions, arrhythmias, or maternal connective tissue disease; or a personal history of arrhythmia or an arrhythmia noted during physical examination. This same publication notes the following as contraindications to propranolol (specifically): cardiogenic shock, sinus bradycardia, hypotension, greater-than-first-degree heart block, heart failure (although carvedilol is often used in this setting), bronchial asthma, or hypersensitivity to the medication.\n\nThere are no specific guidelines for monitoring after β-blocker administration. Some centers will admit young infants to an inpatient setting, whereas older children are more routinely monitored in an outpatient setting. Some centers may choose to monitor patients for 1 to 4 hours on an outpatient basis. Studies failed to demonstrate asymptomatic hypoglycemia and, therefore, caregivers are instructed to give β-blockers during daytime hours with a feeding shortly after administration. β-Blockers may need to be withheld during intercurrent illnesses, and parents and caregivers should be educated about the signs and symptoms of hypoglycemia.\n\nPREP Pearls\n• β-Blocking agents block binding of the neurotransmitters norepinephrine and epinephrine to β1, β2, and/or β3 receptors. Selective β-blockers act preferentially on β1 receptors.\n• Adverse effects of ß-blocking drugs include hypotension, bradycardia, conduction delays, bronchospasm, and hypoglycemia.\n• A thorough cardiopulmonary history and examination should be performed before initiation of ß-blocker therapy, and parents and caregivers should be educated about the signs and symptoms of hypoglycemia.\n\nABP Content Specifications(s)\n• Recognize the adverse effects associated with beta-blocking drugs\n\nSuggested Readings\n• Arneja JS, Benson A, Gilardino MS. Hemangiomas. In: McInerny TK, Adam HM, Campbell DE, DeWitt TG, Foy JM, Kamat DM, eds. American Academy of Pediatrics Textbook of Pediatric Care. 2nd ed. Elk Grove Village, IL: American Academy of Pediatrics; 2017:2110-2116. Pediatric Care Online .\n• Baselga E, Debowska-Baginska B, Przewratil P, et al. Efficacy of propranolol between 6 and 12 months of age in high-risk infantile hemangioma. Pediatrics. 2018;142(3):e20173866. doi:10.1542/peds.2017-3866.\n• Droitcourt C, Kerbrat S, Rault C, et al. Safety of oral propranolol for infantile hemangioma. Pediatrics. 2018;141(6):e20173783. doi:10.1542/peds.2017-3783.\n• Drolet BA, Frommelt PC, Chamlin SL, et al. Initiation and use of propranolol for infantile hemangioma: report of a consensus conference. Pediatrics. 2013;131(1):128-140. doi:10.1542/peds.2012-1691.\n• Krowchuk DP, Frieden IJ, Mancini AJ, et al; Subcommittee on the Management of Infantile Hemangiomas. Clinical practice guideline for the management of infantile hemangiomas. Pediatrics. 2019;143(1):e20183475. doi:10.1542/peds.2018-3475."}
{"id" : 748, "question_text" : "A 6-week-old infant is being discharged after a hospitalization for an apparent life-threatening event (ALTE). He was born at term and had no medical problems before this event. During the hospitalization, no underlying cause for the ALTE was found, and he remained well, had a normal physical examination, and had no further events. His parents ask about home apnea monitoring. Of the following, the MOST accurate information to give these parents is", "options" : "[\"the benefits of home monitoring to the infant outweigh the negative psychosocial effects on parents\", \"the psychosocial changes the parents will experience are likely to remain constant during the entire time the infant is monitored\", \"the parents are likely to experience early increases in depression and hostility once home monitoring is instituted\", \"the parents are likely to experience increases in depression starting about 6 months after home monitoring is instituted\", \"the parents are likely to report feeling that home monitoring of their infant is not helpful\"]", "explanation" : "Evidence does not indicate that home apnea monitors have saved lives or had any effect on the incidence of sudden infant death syndrome. Despite the lack of consensus on the indications, timing, and duration of monitoring, home cardiorespiratory monitoring is still an intervention that many pediatricians and neonatologists consider for individual patients. The psychological effects on families by the presence of a home apnea monitor, both good and bad, should be one factor that the practitioner considers when contemplating prescribing this intervention.\n\nReports show family psychosocial responses to be some-times conflicted when a home apnea monitor is prescribed for an infant. Any family with a newborn infant experiences stress, sleep deprivation, and fatigue, but one expects that these effects would be magnified if the infant has a medical problem that requires home monitoring. Studies have demonstrated increased parental anxiety, increased mood disturbances in mothers, and social isolation especially if the family does not have access to respite care. However, in other studies, parents report that the presence of a monitor is a source of comfort, especially if they had previously lost a child or if the current infant had a cyanotic episode before the monitor was instituted. Parents have frequently described the monitor as helpful to them.\n\nA 1999 study compared 2 groups of parents of infants discharged from a neonatal intensive care unit: 1 group discharged with monitors and the other without monitors. The most striking finding was that depression and hostility increased in the first 2 weeks after hospital discharge for the monitor group in contrast to the no-monitor group in whom depression decreased and hostility stayed constant during the same period. In the monitor group, depression and hostility steadily decreased between 2 weeks and 6 months after discharge. Interestingly, the no-monitor group had increased feelings of hostility by 3 to 6 months after hospital discharge. The authors hypothesized that this may be because mothers returned to work outside the home by that time and were having difficulty juggling multiple roles. This, however, was speculation and was not studied. There was no change in family functioning scores in either group.\n\nPREP Pearls\n• There is no evidence that home apnea monitors are effective in decreasing mortality or preventing sudden infant death syndrome.\n• Use of apnea monitors in the home may increase parental anxiety, mood disturbance, and social isolation, especially immediately after hospital discharge.\n• Parents often describe home apnea monitors as helpful and a source of comfort.\n\nAmerican Board of Pediatrics Content Specification(s):\n• Recognize the psychosocial issues surrounding the use of home monitors\n\nSuggested Reading:\n• Abendroth D, Moser DK, Dracup, K, Doering LV. Do apnea monitors decrease emotional distress in parents of infants at high risk for cardiopulmonary arrest? JPediatr Health Care. 1999;13:50-57. doi:10.1016/ S0891-5245(99)90053-6\n• American Academy of Pediatrics, Committee on Fetus and Newborn. Apnea, sudden infant death syndrome and home monitoring. Pediatrics. 2003;111(4 pt 0:914-916\n• Silvestri JM. Indications for home apnea monitoring (or not). Clin Perinatol. 2009;36:87-99"}
{"id" : 2168, "question_text" : "A 6-week-old infant born at 30 weeks' gestation is in the neonatal intensive care unit. He is currently in room air maintaining adequate oxygen saturation, and has been weaned to a crib. His diet is 160 mL/kg/day of expressed breast milk with human milk fortifier (24 kcal/oz). He takes about 60% of his feedings orally; the remainder is provided via nasogastric tube. The infant has never received a blood transfusion. The infant's weight gain over the last 7 days has been approximately 25 g/day; his current weight is 1.9 kg. He has occasional episodes of bradycardia and desaturation that mainly occur during feedings and are self resolving. His heart rate is 140 beats/min, his blood pressure is 62/32 mm Hg, and his mean arterial blood pressure is 40 mm Hg. His current medications are ferrous sulfate 3.75 mg once per day and a multivitamin 1 mL per day. His recent laboratory findings are as follows: Hemoglobin 8.2 g/dL (82 g/L), Hematocrit 25%, Reticulocyte count 5.4%. Of the following, the BEST next step in this infant's care is", "options" : "[\"administer a blood transfusion\", \"continue his current dose of ferrous sulfate\", \"increase the caloric value of his feedings to 26 kcal/oz\", \"prescribe a multivitamin with iron\"]", "explanation" : "Preterm infants are at risk of experiencing iron deficiency anemia owing to several factors, including inadequate iron stores, phlebotomy-associated blood loss, and an exaggerated and earlier physiologic anemia. The hematocrit concentration increases by 5% to 10% a few hours after birth and subsequently declines steadily for 8 to 10 weeks. The physiologic nadir in hematocrit occurs earlier and may be more clinically significant in preterm infants than in term infants. The infant described in the vignette was born at 30 weeks' gestation. He has inadequate iron stores, as most iron stores are acquired in the third trimester of pregnancy. The fetal hematocrit (HCT) level gradually increases from about 22 to 23 weeks' gestation until term; at 23 weeks, the mean HCT is 40%, and at term the mean HCT is 52%. This infant has anemia of prematurity (an exaggerated and early physiologic anemia), which has been exacerbated by phlebotomy-associated blood loss. He is currently hemodynamically stable, growing well, and receiving an appropriate dose of iron for his weight. His reticulocyte count suggests an adequate bone marrow response. His occasional self-resolved episodes of bradycardia and desaturation are within normal expectations for his age and gestation. Because the infant is hemodynamically stable, has a normal oxygen saturation level in room air, and adequate weight gain, there is no indication for blood transfusion or increasing the caloric value of his feedings. A multivitamin with iron preparation is usually indicated after an infant's weight is >2.5 kg. Iron supplementation is indicated for the following: Preterm infants born at <37 weeks' gestation and/or weighing <2.5 kg; Infants with anemia born at ≥37 weeks' gestation and/or weighing ≥2.5 kg with a history of iatrogenic blood loss; Term infants >4 months of age who are exclusively or partially breastfed and not receiving iron-containing foods/formula. The American Academy of Pediatrics recommends 2 to 4 mg/kg/day of iron supplementation for preterm infants, which should be started when the infant is on full enteral feedings (usually by 2-4 weeks of age). The maximum daily iron dose for infants is 15 mg/day; the total iron intake should be calculated to include iron provided through supplementation and enteral feedings. In all newborns, the hemoglobin and HCT typically increase by 5% to 10% a few hours after birth. This increase is attributed to placental transfusion during labor and delivery, followed by normal neonatal diuresis. Over the subsequent 8 to 10 weeks, there is a progressive decrease in HCT. This decline is the most significant normal change in HCT for any period of life. In healthy term and late-preterm infants, this decline in HCT is usually asymptomatic, even in cases in which the HCT reaches a level as low as 25% to 30% (hemoglobin 8.3 to 10.0 g/dL [83-100 g/L]) at 8 to 10 weeks of age (an approximately 50% decrease from the level at birth). After reaching this nadir, the levels gradually increase to adult levels by around age 2 years. The postnatal decline in HCT occurs earlier in preterm infants and can be more severe, with HCT levels of 20% to 24% (hemoglobin 6.7-8.0 g/dL [67-80 g/L]). This decline can occur without a history of phlebotomy-associated blood loss. These infants may experience clinical symptoms and, according to clinical status, may require blood transfusion; former extremely low-birth-weight infants are at the greatest risk. The decrease in postnatal HCT results from several factors, including reduced erythropoiesis, shorter red blood-cell life span, and dilution due to the infant's increase in body weight and blood volume. Gestational age plays an important role in newborn HCT concentration. These physiologic characteristics may be further influenced by events such as frequent phlebotomy (eg, sick preterm infants cared for in the neonatal intensive care unit), blood loss from other reasons (eg, fetomaternal hemorrhage, placental abruption), and administration of intravenous fluids. Suggested Reading(s): Christensen RD, Bahr TM, Tweddell SM, Ohls RK, Henry E. Diagnosing anemia in neonates: an evidence-based approach. Neoreviews. 2023; 24(6):e343-e355. doi:10.1542/neo.24-6-e343; Pabón-Rivera S, Flores RR, Frei-Jones M. The complete blood count: a practical tool for the pediatrician. Pediatr Rev. 2023;44(7):363-382. doi:10.1542/pir.2021-005273; Strauss RG. Transfusion approach to neonatal anemia. Neoreviews. 2000;1(4):e74-e80. doi:10.1542/neo.1-4-e74; Widness JA. Pathophysiology, diagnosis, and prevention of neonatal anemia. Neoreviews. 2000;1(4):e61-e68. doi:10.1542/neo.1-4-e61. Content Domain: Hematology. Learning Objectives: Describe how hematocrit values differ in pre- and full-term infants."}
{"id" : 141, "question_text" : "An 8-year-old boy has been hospitalized for 2 days with Neisseria meningitidis meningitis that has been confirmed by culture. He is responding well to intravenous antibiotic therapy. He has a healthy 8-year-old twin brother.\n\nOf the following, the BEST choice for prophylaxis for his twin brother is", "options" : "[\"azithromycin\", \"ciprofloxacin\", \"penicillin VK\", \"rifampin\", \"trimethoprim-sulfamethoxazole\"]", "explanation" : "Prophylaxis is recommended for close contacts of a child who has invasive meningococcal infection. Rifampin administered orally in four doses over 2 days is an appropriate prophylactic regimen for the twin brother described in the vignette. Other prophylactic regimens include a single intramuscular dose of ceftriaxone or a single oral dose of ciprofloxacin, although fluoroquinolones are not routinely indicated for children if safer alternatives exist.\n\nIntravenous penicillin G is effective for treatment of invasive meningococcal infections, but oral penicillin VK has not been shown to be effective for prophylaxis of this infection. Azithromycin and trimethoprim-sulfamethoxazole have not demonstrated effectiveness as prophylactic agents for exposure to meningococcal infections.\n\nRifampin is also indicated for prophylaxis after exposure to invasive Haemophilus influenzae type b infection if there is a susceptible child (eg, inadequately immunized or immunocompromised) younger than 4 years of age in the household but with a different dosing schedule (once daily for 4 days).\n\nOther indications for rifampin in pediatrics include use:\n1. as a part of standard tuberculosis treatment regimens — may be used as an alternative regimen for preventive therapy when isoniazid is not an option.\n2. in combination with vancomycin or a beta-lactam antibiotic in certain staphylococcal infections (eg, ventriculoperitoneal shunt infections, osteomyelitis, endocarditis).\n3. in combination with a beta-lactam antibiotic in an attempt to clear persistent group A streptococcal pharyngitis.\n4. in select instances in an attempt to eradicate methicillin-resistant Staphylococcus aureus carriage.\n\nAmerican Board of Pediatrics Content Specification(s):\n\nKnow the appropriate use of rifampin"}
{"id" : 1847, "question_text" : "A clinic has received feedback that patients are frustrated because providers are \"always running late\" for their appointments. Clinicians are working with the outpatient clinic manager to identify ways to improve the clinic workflow, so that patients can see their providers more quickly. Based on the Langley Model for Improvement, the following aim, measurement, and possible changes are defined: Aim: Decrease average time from check-in to ready-to-be-seen from 15 minutes to 10 minutes over the next 6 months. Measurement: Time from patient check-in at the front desk to the time the medical assistant enters their vital signs (which is when the patient is ready to be seen) as recorded in the electronic medical record. Changes that may lead to an improvement: Patients will verify their demographics and insurance online before the appointment. Medical assistants will obtain vital signs in the clinic room instead of at the medical assistant's station, where there is space for only 1 patient at a time. The number of medical assistants will be increased. Before permanently implementing any of the changes for the whole clinic, a pilot test will be run on a smaller scale. The clinic uses the Plan-Do-Study-Act model, and arranges for a few of the medical assistants to obtain vital signs in the clinic rooms for the next 2 weeks. At the end of the 2-week period, the clinician and the office manager review the time data collected, which demonstrates that the average time from check-in to entry of vital signs has increased from 15 to 17 minutes. The office manager notes that it is the beginning of winter, and many patients are now wearing coats and boots. More time is required for them to take the extra clothes off. Of the following, the MOST accurate statement regarding this project is that", "options" : "[\"implementation on a large scale would have been more effective in creating change leading to improvement\", \"obtaining vital signs in the clinic room was an ineffective method of creating change leading to improvement\", \"the best next step is to change the Aim statement\", \"the best next step is to do another Plan-Do-Study-Act cycle\"]", "explanation" : "The Langley Model for Improvement, often called the Model for Improvement, is a framework used to create improvement in many systems, including healthcare. It starts with 3 questions that generate corresponding statements: Question: What are we trying to accomplish? Statement: Aim. Question: How will we know that a change is an improvement? Statement: Measurement. Question: What changes can we make that will lead to an improvement? Statement: Changes that may lead to an improvement.\n\nOnce these statements are defined, the changes are tested in a Plan-Do-Study-Act (PDSA) cycle (Item C47). The results of the PDSA cycle may or may not demonstrate whether the aim was achieved, and may be used to generate new changes, which are then followed by a new PDSA cycle. In the project in the vignette, the first PDSA cycle was completed but the results showed that the average time actually increased instead of decreasing. Discussion among the team members revealed a possible reason for this result. Of the response choices, the best next step is to do another PDSA cycle, for instance, in the same season as the baseline data are gathered.\n\nItem C47: Model for improvement. This model addresses 3 questions and uses rapid cycles of plan (P), do (D), study (S), and act (A) to test change concepts for improvement. Reprinted with permission from Langley GJ, Moen RD, Nolan KM, Nolan TW, Norman CL, Provost LP. The Improvement Guide: A Practical Approach to Enhancing Organizational Performance. 2nd ed. San Francisco, CA: Jossey-Bass; 2009:24.\n\nChanging the Aim statement would only be helpful if the goal for improvement has changed. Unexpected PDSA results should prompt changes in the improvement plan and additional PDSA cycles, not a change in the Aim.\n\nCorrect interpretation of PDSA cycle results is important to achieving the Aim. In the vignette, the time the patient was ready-to-be-seen actually increased after the changes were implemented, but a reason for this increase was found to be unrelated to the change. It would be incorrect to assume that the changes were ineffective after this 1 PDSA cycle. Quality improvement requires looking at data or processes (ie, trends) over time.\n\nThe scale of the project does not necessarily correlate to the achievement of quality improvement. Smaller scale projects can often be completed more quickly than larger scale projects. An advantage of the Langley Model for Improvement framework is that it allows for multiple, rapid PDSA cycles that can ultimately lead to quality improvement in a relatively brief time.\n\nPREP Pearls\n\nThe Langley Model of Improvement is a framework for quality improvement that addresses 3 questions: 1. What are we trying to accomplish? 2. How will we know that a change is an improvement? 3. What changes can we make that will lead to an improvement using multiple, rapid Plan-Do-Study-Act cycles.\n\nQuality improvement requires looking at data or processes (ie, trends) over time.\n\nABP Content Specifications(s)/Content Area\n\nIdentify the components of the Langley Model for Improvement\n\nRecognize that quality improvement requires looking at data or processes (ie, trends) over time\n\nRecognize that analysis of variation in data is critical in quality improvement to understand whether the variation is actually improvement\n\nSuggested Readings\n\nEQIPP. eqipp.aap.org.\n\nHorbar JD, Plsek PE, Leahy K, NIC/Q 2000. Establishing habits for improvement in neonatal intensive care units. Pediatrics. 2003;111(suppl E1):e397-e410. http://pediatrics.aappublications.org/content/111/Supplement_E1/e397.\n\nInstitute for Healthcare Improvement. www.ihi.org/resources/Pages/HowtoImprove.\n\nLangley GJ. The Improvement Guide: A Practical Approach to Enhancing Organizational Performance. 2nd ed. San Francisco, CA: Jossey-Bass; 2009."}
{"id" : 3782, "question_text" : "The pediatric clinic supervising attending physician and pediatric intern discuss the management of otitis media in a 12-month-old female patient. They agree on treatment with amoxicillin 80 to 90 mg/kg per day divided twice daily for 10 days. The intern writes the prescription using the electronic medical record. Later that day, the patient's mother calls the clinic asking for clarification of the amoxicillin dose, because the dose seems too high. The girl's 3-year-old sister recently took amoxicillin for otitis media at a lower dose. Upon review of the prescription order, it was found that a weight of 20 kg was used to calculate the medication dose. The girl's weight was recorded in the electronic medical record as 20 lbs. Of the following, the BEST way to prevent similar errors from occurring is to", "options" : "[\"counsel the intern on ensuring that the correct weight is used to calculate medication doses\", \"have the clinic supervising attending physician review all prescriptions before the patient leaves the clinic\", \"review medication dose calculations with the parent(s)\", \"standardize the use of kilograms when recording weight for all clinic patients\"]", "explanation" : "The vignette describes a situation in which a medication error was made because the dose was calculated using kilograms, but the weight was recorded in the medical record in pounds. Presumably, the units for which weight is recorded in the medical record was not standardized, thus causing the confusion. The best way to prevent similar errors from occurring is to standardize the use of kilograms when recording the weight of all clinic patients. This solution is systems-based versus focusing on individual improvements. Systems-based solutions help prevent similar errors from occurring again and improve the overall system. They help to reduce error caused by human factors. In the vignette, if all weights were recorded in kilograms, the human factors of deciding to record weight in kilograms versus pounds, realizing the weight was recorded in pounds, and the need to convert pounds to kilograms when calculating the medication dose would be eliminated. A culture of safety that encourages health care team members to report errors for the purpose of improving systems facilitates systems-based solutions.\n\nCounseling the intern on ensuring that the correct weight is used to calculate medication doses is an individual-based improvement. It does nothing to improve the system or prevent a similar error from being made by another individual. Having the clinic supervising attending physician review all prescriptions before the patient leaves the clinic would add an additional check on the medication dose but would not be the most efficient way to improve the system. Similarly, reviewing the medication dose calculations with the parent(s) would not be the most efficient way to improve the system.\n\nPREP Pearls\n• Systems-based rather than individual improvements should be sought for health care systems problems.\n• A culture of safety that encourages health care team members to report errors for the purpose of improving systems facilitates systems-based solutions.\n\nABP Content Specifications(s)\n• Apply the psychology of change (eg, motivating people to improve) to improve health-care systems\n• Understand that quality improvement is based on applying a scientific method to improving human systems\n• Understand what a system is (eg, people, procedures, equipment) and how each component of that system affects outcome\n\nSuggested Readings\n• Bartman T, McClead RE. Core principles of quality improvement and patient safety. Pediatr Rev. 2016;37(10):407-417. doi:10.1542/pir.2015-0091.\n• Leonard MS. Patient Safety and quality improvement: reducing risk of harm. Pediatr Rev. 2015;36(10):448-456. doi:10.1542/pir.36-10-448.\n• Neuspiel DR. Medical errors, adverse events, and patient safety. In: McInerny TK, Adam HM, Campbell DE, DeWitt TG, Foy JM, Kamat DM, eds. American Academy of Pediatrics Textbook of Pediatric Care. 2nd ed. Elk Grove Village, IL: American Academy of Pediatrics; 2017:2287-2295. Pediatric Care Online."}
{"id" : 2403, "question_text" : "A 15-year-old is seen in the office for evaluation of new-onset auditory hallucinations. Their mother reports that over the past 6 months the adolescent has become irritable, is sleeping more, is no longer interested in extracurricular activities, and spends most of his time in his room. They still attends school full time, and their grades have not declined. In private, the adolescent reports no history of trauma, bullying, substance use, or physical or emotional abuse. During the interview, they become tearful and admit to feeling scared because for the past 2 months they have heard a voice saying they are \"worthless\" and that everything would be better if the adolescent was not around. The adolescent is otherwise able to stay on topic during the interview and answers all questions appropriately. The adolescent insists, \"I know the voice is not real—I just want it to stop.\" Their physical examination findings are unremarkable. Of the following, the BEST next step in this adolescent's care is to", "options" : "[\"inquire about thoughts of self-harm/suicidal ideation, plan, and intent\", \"obtain a comprehensive metabolic panel, complete blood cell count, and urine toxicology screening\", \"provide resources for cognitive behavioral therapy and social skills programming\", \"refer to a psychiatrist for further evaluation and initiation of antipsychotic medication\"]", "explanation" : "The adolescent in the vignette is experiencing auditory hallucinations as a symptom of a major depressive disorder. These symptoms are sometimes referred to as psychotic experiences and can also be seen in other psychiatric conditions (eg, anxiety and obsessive-compulsive disorder). Given the content of the adolescent's hallucinations, suicide screening via a standardized instrument should take priority. If there is concern regarding the adolescent's safety, they should be referred to the emergency department for evaluation. The other response choices may be appropriate but only after screening for suicidal ideation, plan, and intent.\n\nPsychosis is defined as the inability to distinguish between reality and nonreality. Features of psychotic disorders as defined by Diagnostic and Statistical Manual of Mental Disorders, 5th edition, include delusions, hallucinations, disorganized thinking/speech or motor behavior (eg, catatonia), and negative symptoms. Delusions can be further classified as bizarre (eg, an outside force is controlling one's thoughts and actions) or nonbizarre (thoughts are plausible, but have no supporting evidence). Hallucinations occur more frequently than do delusions among children and adolescents diagnosed with psychosis. Hallucinations are vivid perceptions that can involve any of the senses (sight, sound, smell, touch). Individuals with disorganized thinking may be unable to stay on topic, have difficulty answering questions, or have incoherent speech. Disorganized motor behaviors may include catatonia, ranging from a decreased response to environmental stimuli to increased/excessive motor movement (eg, catatonic excitement). The adolescent in the vignette is not exhibiting disorganized thinking or motor behaviors and appears to have insight into his auditory hallucinations. Negative symptoms include decreased emotional expression, lack of speech, anhedonia, and difficulty forming relationships. The adolescent in the vignette is not experiencing negative symptoms.\n\nLaboratory studies may be considered after a detailed medical history—including birth history, family psychiatric history, history of trauma or abuse, and substance use—is obtained. This evaluation may include a comprehensive metabolic panel, complete blood cell count, thyroid studies, HIV screening, antinuclear antibodies test, and a urine toxicology screen. Urine toxicology screening should be considered because some substances (including hallucinogens and cannabis) can cause psychotic experiences that would be classified as a drug-induced psychotic disorder.\n\nThe prevalence of schizophrenia of early onset (before age 18 years) has been reported to be 0.5%. Very-early-onset schizophrenia (onset of symptoms before age 13 years) has a prevalence of 1 per 10,000 children. Prodromal symptoms of schizophrenia may include social withdrawal, worsening academic performance, unusual behavior, and poor personal hygiene. The etiology of schizophrenia is multifactorial, with both genetic and environmental risk factors. Environmental risk factors include prenatal substance exposure or maternal infection, and advanced paternal age. Childhood trauma resulting from emotional or physical abuse, domestic violence, and bullying can increase the risk of psychotic experiences and psychotic disorders, especially when genetic risk is present.\n\nThe treatment of schizophrenia is multimodal; first-line treatment includes second-generation antipsychotic medication (including for children with very-early-onset schizophrenia). First-generation antipsychotic medications (eg, haloperidol, chlorpromazine) target dopamine receptors in all areas of the brain, resulting in an increased risk of experiencing extrapyramidal adverse effects (eg, hypertonia, tremor, slowed movements, restlessness [akathisia], and dystonic or dyskinetic movements). Second-generation antipsychotic medications (eg, risperidone, aripiprazole) have a decreased risk of extrapyramidal side effects but can result in weight gain, hypercholesterolemia, dyslipidemia, and glucose intolerance. When starting a second-generation antipsychotic medication, metabolic monitoring (fasting blood glucose, hemoglobin A1c, fasting lipid levels) and monitoring of body mass index/waist circumference is recommended for the first 3 months, at 6 months, and then annually. Additional treatment modalities for psychotic symptoms or a psychotic disorder include cognitive behavioral therapy (to address distressing thoughts, delusions, and hallucinations) and psychosocial interventions focused on the family and building social skills. Measures to support children and adolescents educationally should also be implemented.\n\nSuggested Reading(s)\nAbidi S. Psychosis in children and youth: focus on early-onset schizophrenia. Pediatr Rev. 2013 Jul;34(7):296-305. doi:10.1542/pir.34-7-296\nHua LL; Committee on Adolescence. Collaborative care in the identification and management of psychosis in adolescents and young adults. Pediatrics. 2021;147(6):e2021051486. doi:10.1542/peds.2021-051486\nMcClellan J. Psychosis in children and adolescents. J Am Acad Child Adolesc Psychiatry. 2018;57(5):308-312. doi:10.1016/j.jaac.2018.01.021\nWalter HJ, DeMaso DR. Psychiatric emergencies: suicidality, agitation, psychosis, and disaster exposure. In: McInerny TK, Adam HM, Campbell DE, Foy JM, Kamat DM, eds. American Academy of Pediatrics Textbook of Pediatric Care. 2nd ed. American Academy of Pediatrics; 2023. Accessed September 1, 2023. Pediatric Care Online\n\nContent Domain\nMental health\n\nABP Content Specification(s) / Content Area(s)\nRecognize behaviors suggestive of psychotic behavior/thought disorders, and manage appropriately"}
{"id" : 189, "question_text" : "An 8-year-old girl presents for evaluation of \"neck swelling.\" Her mother reports that the swelling began approximately 1 year ago and has progressed with time. The swelling is not causing any pain. On physical examination, the girl's thyroid gland is diffusely enlarged, has a cobblestone texture, but has no discrete nodules (Item Q80). Findings for all other systems are within normal parameters. Free thyroxine and thyroid-stimulating hormone measurements are normal. Of the following, the BEST next step in the evaluation of this patient is to", "options" : "[\"assess thyroglobulin\", \"assess thyroid peroxidase antibodies\", \"assess thyroid-stimulating immunoglobulins\", \"order neck ultrasonography\", \"perform a thyroid biopsy\"]", "explanation" : "The girl described in the vignette has classic signs and symptoms of chronic lymphocytic thyroiditis (Hashimoto thyroiditis). Patients who have chronic lymphocytic thyroiditis are commonly euthyroid (have normal free thyroxine and thyroid-stimulating hormone values) for many years despite having other physical (goiter) or laboratory (autoantibody) signs of autoimmune thyroid disease. Although this girl has thyromegaly, as many as two thirds of patients who have Hashimoto thyroiditis have atrophic disease that results in small or even nonpalpable glands. The most sensitive laboratory test to confirm the presence of autoimmune thyroid disease is measurement of autoantibodies specific to thyroid antigens. In the case of Hashimoto thyroiditis, antibodies to thyroid peroxidase alone have greater than 85% sensitivity. Sensitivity increases to more than 90% if thyroglobulin autoantibodies are also measured. Thyroglobulin concentrations (not to be confused with thyroglobulin antibody titers) simply reflect the total volume of thyroid tissue and are expected to be elevated in patients who have thyromegaly. Thus, measuring thyroglobulin is not helpful in determining the cause of thyromegaly. Thyroid-stimulating immunoglobulins are an excellent tool for assessing risk for Graves disease (which should result in a large, smooth, and firm gland) but are not specific for Hashimoto disease. On rare occasions, patients who have negative antibodies can still be proven to have Hashimoto thyroiditis by virtue of a biopsy and histologic review of thyroid tissue. However, unless the patient has a discrete nodule that is palpable on physical examination, neither ultrasonography nor thyroid biopsy is indicated during the initial evaluation.\n\nOf note, patients who have any type of autoimmune thyroid disease are at increased lifetime risk for thyroid nodules and thyroid cancers compared with the general population. Pain on palpation of the thyroid could be indicative of acute or subacute thyroiditis but is rarely noted in those who have Hashimoto thyroiditis.\n\nCritique: Preferred Response: B\n\nContent Specifications: Recognize the signs and symptoms of Hashimoto thyroiditis"}
{"id" : 2385, "question_text" : "A 6-year-old is being evaluated in the clinic for a 2-day history of sore throat, fever, and headache. He has not had congestion or cough. On physical examination, his temperature is 38.5 °C. His uvula is midline, there are bilateral tonsillar exudates, and he has tender anterior cervical lymph nodes. The remainder of his examination índings are normal. A point-of-care rapid test is performed that conírms the diagnosis. On review of the boy's allergies, before the physician prescribes antibiotic treatment, the family reports that the boy had an anaphylactic reaction to amoxicillin. Of the following, the BEST treatment for this boy's condition is a 10-day course of", "options" : "[\"azithromycin\", \"cefdinir\", \"clindamycin\", \"trimethoprim-sulfamethoxazole\"]", "explanation" : "The child in the vignette has symptoms and signs consistent with group A streptococcal (GAS) pharyngitis. The diagnosis is best conírmed with a point-of-care rapid antigen test or culture performed on a throat swab specimen. The typical treatment is a 10-day course of oral amoxicillin (50 mg/kg/dose once per day). However, because this child has a history of an anaphylactic reaction to amoxicillin, of the response choices, the best treatment option is clindamycin. Azithromycin is an appropriate treatment in a child allergic to amoxicillin or penicillin, but with a treatment duration of 5 days (12 mg/kg/dose once on day 1, then 6 mg/kg/day once per day for 4 days). There is a risk of cross reaction to cephalosporins in a child with an anaphylactic amoxicillin allergy. A 1st-generation cephalosporin (eg, cephalexin) can be considered to treat GAS in cases where amoxicillin is contraindicated. Cefdinir is a 3rd-generation cephalosporin, which provides much broader coverage than is needed.\n\nPharyngitis is the most common manifestation of a Streptococcus pyogenes infection. The highest incidence is in children aged 5 to 15 years; infection is most prevalent during the winter. Symptoms and signs of streptococcal pharyngitis may include sore throat, vomiting, headache, fever, tender anterior cervical chain lymphadenopathy, and tonsillar and posterior pharyngeal exudates. Cough and congestion make the diagnosis of streptococcal pharyngitis less likely.\n\nFor children older than 3 years, a throat-swab rapid antigen test is recommended to conírm the diagnosis. If the result of the rapid test is negative, a throat culture should be performed. The US Food and Drug Administration has approved a few nucleic acid ampliícation tests as stand-alone tests, not requiring culture conírmation of a negative rapid test. For children younger than 3 years, testing is not recommended given the low risk of complications of disease, but it can be considered for a symptomatic child with an infected close household contact.\n\nTreatment of streptococcal pharyngitis should be started within 9 days of illness onset to decrease the risk of rheumatic fever. Low-dose (50 mg/kg/day) amoxicillin given orally once per day for 10 days or one dose of intramuscular benzathine penicillin G is recommended. Repeat testing after completing appropriate therapy is not routinely recommended.\n\nAcute rheumatic fever (ARF) is a leading cause of death worldwide, especially in developing countries. ARF is a sequela of untreated or inadequately treated GAS pharyngitis. The diagnosis of ARF is based on the revised Jones criteria. Manifestations of ARF include carditis, polyarthritis, chorea, erythema marginatum, and subcutaneous nodules. Carditis is the most serious manifestation and can result in permanent cardiac damage and valvular disease. Recurrent GAS pharyngitis increases the risk of ARF. Children who have had ARF may develop symptom recurrence with subsequent GAS pharyngitis. Penicillin prophylaxis is recommended for 5 years if there is no evidence of carditis and for 10 years or until age 21 years (whichever is later) if there is carditis that improves. If carditis with permanent cardiac damage is present, lifelong penicillin prophylaxis is recommended.\n\nPost-streptococcal glomerulonephritis (PSGN) is another sequela of GAS infection. PSGN can occur after either pharyngitis or skin infection (impetigo). The incidence of PSGN is lower than that of ARF. PSGN typically presents 1 to 3 weeks after the onset of GAS infection with hematuria, proteinuria, hypertension, and edema. Most children with PSGN recover completely; however, a small percentage may develop chronic kidney disease.\n\nSuggested Reading(s)\nAmerican Academy of Pediatrics. Group A Streptococcus. In: Kimberlin DW, Barnett ED, Lyníeld R, Sawyer MH, eds. Red Book: 2021–2024 Report of the Committee on Infectious Diseases. 32nd ed. American Academy of Pediatrics; 2021. Accessed September 1, 2023. Red Book Online\n\nBernardes LS, Katz G, Rehder CW, et al. Acute rheumatic fever: an update on diagnosis, management, and prevention. Pediatr Rev. 2020;41(12):599-609. doi:10.1542/pir.2019-0149\n\nCampbell KM. Diagnosis and management of streptococcal pharyngitis. Pediatr Rev. 2021;42(1):3-14. doi:10.1542/pir.2020-0003\n\nContent Domain\nInfectious Diseases\n\nABP Content Specication(s) / Content Area(s)\nRecognize the clinical features associated with group A Streptococcus pharyngitis\nPlan appropriate management for a patient with group A Streptococcus pharyngitis\nRecognize the complications associated with group A Streptococcus infection\n\nThe correct answer is: clindamycin"}
{"id" : 1310, "question_text" : "A 10-year-old boy with spina bifida is brought to his pediatrician for evaluation of cloudy urine. He has a history of neurogenic bladder requiring catheterization. He has had multiple urinary tract infections in the past. Vital signs show a temperature of 38.1°C, respiratory rate of 20 breaths/min, heart rate of 88 beats/min, and blood pressure of 110/60 mm Hg. On physical examination, he has no motor function of his lower extremities. Laboratory data show:\n\nUrinalysis, 3+ leukocytes, nitrite negative\nUrine Gram stain, gram-positive cocci in pairs and chains\n\nOf the following, the BEST therapy for this infection is", "options" : "[\"ampicillin\", \"cefixime\", \"cephalexin\", \"nitrofurantoin\", \"trimethoprim-sulfamethoxazole\"]", "explanation" : "Correct Answer: A\nThe best choice for the treatment of the urinary tract infection in the boy in this vignette is ampicillin. The Gram stain reveals gram-positive cocci in pairs and chains. The most likely pathogen in this vignette is Enterococcus, therefore ampicillin is the preferred choice.\n\nEnterococci are normal flora of the gastrointestinal tract of humans and other animals. They are widely recognized as a cause of urinary tract infections, as well as bacteremia, endocarditis, and wound infections. Since they reside in the human gastrointestinal tract, they must be considered in the presence of intra-abdominal infections. Rarely, enterococci can cause meningitis. Enterococci are opportunists and their rise to prominence has been attributed to a growing population of patients that are immunocompromised or severely ill and necessitate medical devices such as central venous or urinary catheters. Enterococci should be considered in any child who requires chronic bladder catheterization and develops an urinary tract infection. In addition, enterococci frequently develop resistance to antibiotics.\n\nEnterococci are intrinsically resistant to cephalosporins, therefore cefixime and cephalexin would not be correct choices. Cephalosporins are appropriate antimicrobials to use for urinary tract infections caused by gram-negative enteric bacteria. The vignette, however, reveals that the etiology of the infection is due to a gram-positive organism.\n\nNitrofurantoin can be used for the treatment of cystitis caused by a susceptible gram-negative or gram-positive organism or for prophylaxis of urinary tract infections. Nitrfurantoin has activity against susceptible enterococci, but either penicillin or ampicillin are preferred agents for uncomplicated urinary tract infections.\n\nThe use of trimethoprim-sulfamethoxazole for enterococcal urinary tract infections is controversial. While there are some in vitro data to suggest susceptibility, this may not correlate with clinical outcomes and thus is not the best treatment for this patient.\n\nPREP Pearls\n• Enterococci are widely recognized as a cause of urinary tract infections, as well as bacteremia, endocarditis, and wound infections, and must be considered in intra-abdominal infections.\n• Enterococci are opportunists and their rise to prominence has been attributed to a growing population of patients that are immunocompromised or severely ill and necessitate medical devices such as central venous or urinary catheters.\n• Enterococci are intrinsically resistant to cephalosporins.\n\nABP Content Specifications(s)\n• Recognize the clinical syndromes associated with enterococcal infections\n\nSuggested Readings\n• American Academy of Pediatrics. Non-group A or B streptococcal and enterococcal infections. In: Kimberlin DW, Brady MT, Jackson MA, Long SS, eds. Red Book: 2015 Report of the Committee on Infectious Diseases. 30th ed. Elk Grove Village, IL: American Academy of Pediatrics; 2015:750-753.\n• Butler KM. Enterococcal infection in children. Semin Pediatr Infect Dis. 2011;17(3):128-139. doi: http://dx.doi.org/10.1053/j.spid.2006.06.006."}
{"id" : 3771, "question_text" : "A 16-year-old sexually active girl is seen at the school-based health clinic. She is concerned that she might have genital herpes, though she has never been diagnosed with herpes simplex virus. At her recent health supervision visit 2 months ago, screening for Neisseria gonorrhoeae, Chlamydia trachomatis, syphilis, and HIV were all negative. She had coitarche at age 16 years, and is currently receiving medroxyprogesterone injections every 3 months as a birth control method. She has had 1 lifetime male partner whom she has been dating for the past year. They use condoms intermittently. Genital lesions are noted (Item Q219). Of the following, the MOST likely diagnosis for this girl is", "options" : "[\"condyloma acuminata\", \"condyloma lata\", \"genital herpes simplex virus\", \"molluscum contagiosum\"]", "explanation" : "The most likely diagnosis for the girl in the vignette is condyloma acuminata, which is caused by human papillomavirus (HPV). Human papillomavirus is the most common sexually transmitted infection in the United States. According to the Centers for Disease Control and Prevention, there are approximately 14 million new cases of HPV per year and 4,000 deaths per year in women with cervical cancer related to HPV. There are approximately 120 serotypes of HPV, and at least 40 are associated with infection in the genital tract. Almost all sexually active people have acquired HPV at some point in their lifetime, but may not be aware because they are asymptomatic. The strains of HPV are divided into categories of high risk (oncogenic) and low risk (nononcogenic). Risk factors for acquiring HPV include: 1) multiple sexual partners; 2) having a partner who has had multiple sexual partners; 3) early sexual debut; and 4) inconsistent condom use.\n\nHuman papillomavirus can affect the anogenital area as well as the mouth and throat. Infection with high-risk strains of HPV can cause changes in cervical cells, which may lead to abnormal results on a Papanicolaou smear (cervical cancer screen). However, most cellular changes induced by HPV are transient. Current cervical cancer screening guidelines recommend that screening begin at age 21 years, which is older than previous recommendations. This change was made in an effort to reduce identification of low-grade lesions that will most likely self-resolve with no significant health consequences, reduce unnecessary anxiety in patients, and avoid the costs and risks of unnecessary diagnostic and therapeutic medical procedures such as colposcopy and loop electrosurgical excision procedure.\n\nInfections with the low-risk HPV strains tend to cause genital warts (condyloma acuminata) in boys and girls. Boys develop lesions on their penis, scrotum, and perianal area; less commonly, boys can have lesions in the urethral meatus. Girls develop lesions on the vulva, perineum, and perianal area, and less commonly, in the vagina or cervix. The lesions are usually flesh colored and described as cauliflower-like, painless, and nonpruritic. The differential diagnosis of genital warts includes molluscum contagiosum, condyloma lata associated with secondary syphilis, pearly penile papules (boys), and vestibular papillae (girls).\n\nPrepubertal children can present with condylomata acuminata. There is no consensus regarding whether genital or anal warts are specific for sexual abuse. The presence of warts should always prompt the provider to inquire about sexual abuse. Children can develop condyloma acuminata from vertical transmission.\n\nGenital warts may spontaneously resolve. Treatment can be patient or physician applied. With treatment, warts usually resolve within 3 months. Treatment does not eradicate the virus, and the genital warts may return. As a preventive measure, the 9-valent HPV vaccine is approved for use in the United States in males and females between the ages of 9 and 45 years. The vaccine has been shown to prevent genital warts and cancers associated with HPV strains 6, 11, 16, 18, 31, 33, 45, 52, and 58. For boys and girls between 9 and 14 years of age, a 2-dose regimen is recommended, with the second dose given 6 to 12 months after the first. For men and women between 15 and 45 years of age, a 3-dose regimen is recommended, with doses repeated at 1 to 2 months, and 6 months after the first.\n\nThe physical examination findings for the girl in the vignette are not consistent with the other response diagnoses. Condyloma lata are smooth flat warts that are associated with secondary syphilis. Genital herpes simplex virus generally presents as painful vesicles or ulcers. Molluscum contagiosum are pearly lesions with a central umbilication.\n\nPREP Pearls\n• Human papillomavirus is the most common sexually transmitted infection in the United States.\n• Almost all sexually active people acquire human papillomavirus at some point in their lifetime.\n• The 9-valent human papillomavirus vaccine is approved for use in the United States in males and females between the ages of 9 and 26 years, and was recently approved for men and women between 27 and 45 years, to prevent cancers caused by high-risk human papillomavirus strains.\n\nABP Content Specifications(s)\n• Recognize the clinical findings associated with condylomata acuminata\n• Understand the significance of condylomata acuminata in patients of various ages, including their association with sexual abuse\n• Plan the appropriate management of condylomata acuminata\n\nSuggested Readings\n• Eliscu A. Human papillomavirus and HPV vaccines. Pediatr Rev. 2017;38(9):443-445. doi:10.1542/pir.2016-0018.\n• Kaskowitz A, Quint E. A practical overview of managing adolescent gynecologic conditions in the pediatric office. Pediatr Rev. 2014:35(9):371-381. doi:10.1542/pir.35-9-371.\n• Workowski KA, Bolan, GA. Sexually transmitted diseases treatment guidelines, 2015. MMWR Recomm Rep. 2015;64(3):84-93. https://www.cdc.gov/mmwr/preview/mmwrhtml/rr6403a1.htm?s_cid=rr6403a1."}
{"id" : 1757, "question_text" : "You are reviewing discharge plans for a 2-day-old neonate born at 35 weeks of gestation with a birthweight of 2.62 kg. His mother, a recent immigrant from Sri Lanka, has blood type O positive. The neonate's blood type is O positive. He has been breastfeeding well, with 4 wet diapers and 2 stools in the past 24 hours. He received 1 feeding of formula. On physical examination, his weight is 2.58 kg and he has mild facial jaundice. His total bilirubin is 10.2 mg/dL (174.5 μmol/L) at 48 hours of age. Using the Bhutani nomogram, the pediatric resident labeled his bilirubin risk level as low intermediate with no recommendation for a repeat bilirubin level on discharge. Of the following, the factor that places this neonate at INCREASED risk of hyperbilirubinemia is", "options" : "[\"east Asian race\", \"excess weight loss\", \"hemolytic disease\", \"jaundice\", \"prematurity\"]", "explanation" : "For the neonate in the vignette, prematurity is the risk factor that places him at increased risk for hyperbilirubinemia. As a result, a repeat serum bilirubin level should be performed after discharge. All neonates have physiologic jaundice in the first days after birth because of a relatively high bilirubin load, decreased uptake of bilirubin in the liver, increased enterohepatic circulation, and lower activity of uridine diphosphoglucuronosyl transferase. In addition, immediately after birth, the blood-brain barrier is relatively permeable, allowing passage of bilirubin into the brain where it can damage neurons. The blood-brain barrier strengthens quickly, allowing neonates to tolerate increasingly higher serum bilirubin levels over the first few days after birth. It is recommended that providers use hour- and risk factor–specific total serum bilirubin level nomograms to estimate a neonate's risk of bilirubin-associated neurologic damage (Item C234). Serum bilirubin level may be accurately predicted with transcutaneous measurement. All neonates should be screened for pathologic jaundice with total bilirubin measurement (serum or transcutaneous) prior to discharge.\n\nItem C234: Nomogram for designation of risk in 2840 well newborns at 36 weeks' gestational age with birth weight of 2000 g or 35 weeks' gestational age and birth weight of 2500 g based on the hour-specific serum bilirubin values.\nReproduced with permission from Bhutani VK, Johnson L, Sivieri EM. Pediatrics. 1999;103[1]:6 –14.\n\nPremature neonates, born at less than 37 weeks of gestation, are at risk for acute bilirubin encephalopathy at lower bilirubin levels than term neonates. They have relatively slower clearance of bilirubin. The risk is even greater if enteral feeds cannot be started immediately after birth because of respiratory distress, leading to increased enterohepatic circulation. East Asian race is associated with an increased risk of pathologic jaundice. Those at highest risk include those born to Chinese, Japanese, or Filipino parents.\n\nExclusively breastfed neonates who have lost more than 10% of their birthweight within the first 3 days after birth are at higher risk for jaundice, presumably due to increased enterohepatic circulation. This likely occurs because the mother's milk supply takes time to be established. The increased risk of jaundice in exclusively breastfed neonates is called \"breastfeeding jaundice.\" The 2-day-old neonate in the vignette has lost only 2% of his birthweight, and has had 4 wet diapers in the past 24 hours, suggesting adequate enteral intake. Therefore, excess weight loss is not a risk factor for pathologic jaundice for this neonate.\n\nHemolytic disease in neonates may occur due to Rh or ABO isoimmunization. In the United States, the use of Rho (D) immune globulin for mothers who are negative for the Rh antigen has markedly decreased the incidence of Rh isoimmunization. ABO isoimmunization occurs when a mother with type O blood delivers a neonate with type A, B, or AB blood. Mothers with type O blood have anti-A and anti-B antibodies because of environmental exposure to these epitopes. However, in this vignette, both the mother and neonate are blood type O positive.\n\nTypically, jaundice moves from the face to the thorax and downward, as bilirubin levels increase. Visibly yellow skin can be used as an initial screen for pathologic jaundice. However, it has been shown that clinicians are not able to reliably diagnose pathologic jaundice based solely on visual appearance, particularly in neonates with darker skin pigmentation. In addition, jaundice is visible on the skin at levels that may not be pathologic, therefore, jaundice alone does not necessitate a repeat bilirubin test.\n\nPREP Pearls\n• Serum bilirubin levels in neonates should be monitored based on hour- and risk factor–specific nomograms.\n• Neonates born before 37 weeks of gestation are at increased risk for acute bilirubin encephalopathy.\n• All neonates should be screened for pathologic jaundice with a total serum bilirubin or transcutaneous bilirubin level prior to discharge.\n\nMOCA-Peds Objective\n• Manage breast-feeding difficulties\n\nABP Content Specifications(s)\n• Understand the mechanism of breast-milk jaundice and manage appropriately\n• Plan the appropriate diagnostic evaluation of jaundice in a full-term infant\n• Recognize the association between breast-feeding and physiologic jaundice in the neonatal period\n• Understand the differences between physiologic jaundice in pre-term and full-term infants\n\nSuggested Readings\n• Lauer BJ, Spector ND. Hyperbilirubinemia in the newborn. Pediatr Rev. 2011;32:341. doi: http://dx.doi.org/10.1542/pir.32-8-341.\n• Maisels MJ, Bhutani VK, Bogen D, Newman TB, Stark AR. Hyperbilirubinemia in the newborn infant =35 weeks' gestation: an update with clarifications. Pediatrics. 2009;124;1193; originally published online September 28, 2009. doi: http://dx.doi.org/10.1542/peds.2009-0329."}
{"id" : 1858, "question_text" : "A 9-year-old girl is evaluated for concerns about her lack of growth. The girl has not been outgrowing her clothes, and her sister, who is 2 years younger, is catching up to her in height. She has no significant medical history and takes no medication. A review of systems reveals worsening vision over the past month. She has also been having intermittent headaches at school that her mother attributes to her vision problems. Her adjusted midparental height is at the 60th percentile. Her mother had menarche at 12 years of age, and her father had delayed puberty. She has a temperature of 37°C, blood pressure of 100/65 mm Hg, and heart rate of 72 beats/min. Her visual acuity is 20/100 bilaterally. Her growth curve is shown (Item Q58). The remainder of the physical examination findings are unremarkable. Of the following, the test MOST likely to reveal the diagnosis is", "options" : "[\"bone age radiography\", \"brain magnetic resonance imaging\", \"a karyotype\", \"a thyroid-stimulating hormone level\"]", "explanation" : "The girl in this vignette has growth failure secondary to a craniopharyngioma. Brain magnetic resonance imaging (MRI) is the test most likely to reveal the diagnosis. Poor linear growth, headaches, and vision problems are common presenting symptoms of craniopharyngioma. Growth hormone deficiency is the most common anterior pituitary hormone deficiency at the time of diagnosis, manifesting as growth problems. Other pituitary hormone deficiencies may also be present and can contribute to abnormal growth. The other symptoms of craniopharyngioma are caused by increased intracranial pressure and mass effect on the optic chiasm.\n\nCraniopharyngioma is the most common suprasellar tumor in childhood. These tumors originate from the remnants of Rathke's pouch and are benign, but they can cause significant problems because of their location and mass effect. The peak incidence in childhood is between 5 and 14 years of age. The standard imaging modality is MRI with and without contrast.\n\nCraniopharyngioma appears as a cystic mass on MRI (Item C58). Characteristic intratumoral calcifications can be seen on computed tomography. Treatment is associated with significant morbidity, specifically panhypopituitarism, diabetes insipidus, hypothalamic obesity, and vision loss."}
{"id" : 3271, "question_text" : "You are meeting with the parents of a child who was diagnosed with leukemia. The parents have researched the drugs the oncologist recommended to treat their child's leukemia and are concerned about the potential adverse effects and how \"toxic\" they are to the body. They have also researched alternative treatments for leukemia. You believe in incorporating the principles of autonomy and beneficence into your patient care. Of the following, the statement that BEST represents those principles is", "options" : "[\"alternative therapies to treat leukemia do not have the research to support their use\", \"alternative treatments could be considered for use in conjunction with the standard medical therapy\", \"the cost of unproven treatments can take away resources from other patients\", \"declining standard treatment for leukemia is a family decision that physicians should respect\", \"the risk of treatment with potentially harmful medications should be compared only with the risk of no treatment\"]", "explanation" : "Preferred Response: B\nThe response that best represents autonomy and beneficence is that alternative treatments could be considered for use in conjunction with the standard medical therapy. This approach incorporates the principles of autonomy by allowing the family to make decisions about their child's medical care and beneficence, ensuring that no harm will be done to the patient. Leukemia is a life-threatening condition and conventional medicine has a high rate of cure. The family in the vignette is exploring complementary and alternative medicine (CAM) treatment for their child. It is important to discuss with the family the reasons why they are exploring these options and to make an effort to understand the family's needs.\n\nIt is not uncommon for families with chronic or life-threatening conditions to explore alternative therapies and to try unconventional therapies. Clinicians should be willing to discuss these treatments with patients and their families. It is possible that some natural or alternative treatments could be used along with conventional treatment. It is important that the clinician open up a dialogue with the family to learn what types of treatments the family wants to include. The patient should be monitored for side effects of CAM treatment.\n\nComplementary and alternative medicine is defined as a group of diverse medical and health care systems and practices that are not generally considered part of conventional medicine. CAM can include acupuncture, homeopathy, naturopathy, meditation, prayer, yoga, biofeedback, hypnosis, guided imagery, art therapy, music therapy, herbs, vitamins, nutritional therapy, massage, chiropractic care, and biofield and bioelectromagnetic therapies. Several of these modalities are now accepted as beneficial in conjunction with conventional medicine. It has been reported that up to 40% of children use dietary supplements. In regional surveys of families that use CAM, 45% report using an herbal product. The widespread use of CAM makes it an important subject for clinicians to address in patient interactions.\n\nThe clinician should always be guided by the ethical principles of autonomy, beneficence, nonmaleficence, and justice. Autonomy is the right of the patient to make decisions about personal medical care consistent with personal values. It is important that the families using CAM be able to discuss this use with the physician and make informed decisions. Beneficence is the duty of the physician to act in the best interest of the patient. The physician should listen to each family's concerns and desire to use CAM, and recommend or not recommend the best options for the patient. Nonmaleficence is the duty to do no harm. The physician should advise the family against any CAM that can do harm or interfere with other treatments. Justice is fairness, and the clinician should treat any conflicting interest of individuals in a fair manner. The physician should work to ensure fair access to care for their patient.\n\nCompared with conventional medicine, alternative treatments to treat leukemia do not have evidence-based research data to support their use, but ignoring interest in CAM does not promote autonomy for the patient and their family. Although the cost of unproven treatments may take away resources from others and this could violate the principle of justice, the family's resources and access to care should not be limited by this alone. In the case described in the vignette, the physician should not accept the family's decision to decline treatment. This would go against the principle of beneficence, because leukemia is a life-threatening condition with a standard of care that has good outcomes. The ethical principle of nonmaleficence would guide against comparing the risk of potentially harmful medications only with the risk of no treatment at all.\n\nPREP Pearls\n• Complementary and alternative medicine (CAM) can include acupuncture, homeopathy, naturopathy, meditation, prayer, yoga, biofeedback, hypnosis, guided imagery, art therapy, music therapy, herbs, vitamins, nutritional therapy, massage, chiropractic care, and biofield and bioelectromagnetic therapies.\n• Clinicians should provide an environment where families can openly discuss the use of CAM.\n• Respecting patient autonomy requires the clinician to consider the use of CAM.\n\nAmerican Board of Pediatrics Content Specification(s)\n• Recognize and apply ethical principles regarding the use of complementary and alternative medicine\n\nSuggested Reading\n• Gardiner P, Riley D. Herbs to homeopathy-medicinal products for children. Pediatr Chin North Am. 2007;54(6):859-874. doi:10.1016/j. pc1.2007.10.005.\n• Mears BJ. Ethics for the pediatrician: the ethics of complementary and alternative medicine. Pediatr Rev. 2010;31(7):e49-e51. doi:10.1542/pir.31-7-e49."}
{"id" : 2233, "question_text" : "A 5-year-old child with severe autism is seen for a health supervision visit. He has great difficulty with emotional regulation in both home and school and frequently manifests his emotions physically. His parents have been taking him to a chiropractor and an acupuncturist weekly in an effort to help with his emotional outbursts, to some subjective benefit. The parents have now started giving the child a supplement that contains fish oil, folic acid, and zinc but have found it to be cost prohibitive and request a prescription. Of the following, the BEST response to the parents' request is to", "options" : "[\"advise discontinuing this therapy immediately\", \"discuss the risks and benefits of this therapy\", \"refer the family to an alternative medicine practitioner\", \"report the family to child protective services\"]", "explanation" : "The parents of the child in the vignette are seeking complementary and alternative medicine (CAM) to treat a chronic condition for which no complete cure is known. Empathy and respect are imperative when discussing CAM. Thus, the best response to this family's request is to discuss the risks and benefits of their requested therapy. Advising them to discontinue this therapy immediately, without proper consideration of not only the tangible but also the intangible consequences, would be ill advised. Referring the family to an alternative medicine practitioner could be considered, but only after a thorough discussion with the family about treatment goals and setting realistic expectations. Reporting the family to child protective services would be inappropriate, because there is no indication of abuse and/or neglect.\nThe use of CAM is a common practice for conditions as minor as the common cold to more serious illnesses such as cancer, and use of CAM often goes unreported to the health care team unless specifically elicited. Discussion regarding the use of CAM must be culturally sensitive and respectful, using basic ethical principles, especially autonomy, beneficence, nonmaleficence, and justice. \nAutonomy refers to the ability to make informed decisions freely. \nBeneficence refers to the obligation to prioritize the families' best interests over other competing interests.\nNonmaleficence refers to proactively doing no harm.\nJustice refers to providing care in a fair, equitable manner.\nOne approach to applying these principles in conversations about CAM is to use the acronym ARMED: asking, respecting, monitoring, educating, and distributing quality information. The ARMED approach is aimed at helping health care practitioners address the topic of complementary approaches with more confidence and ease. These actions are further detailed in the Table. \nSuggested Reading(s)\nBioethics case based teaching guides for resident training. American Academy of Pediatrics, Section on Bioethics and Committee on Bioethics. Accessed March 11, 2024. https://www.aap.org/en/pedialink/bioethics-case-based-teaching-guides-for-resident-training/\nMcClafferty H, Vohra S, Bailey M, et al; Section on Integrative Medicine. Pediatric integrative medicine. Pediatrics. 2017;140(3):e20171961. doi:10.1542/peds.2017-1961\nContent Domain\nPharmacology\nLearning Objectives\nAsk caregivers about their goals when considering the use of complementary and alternative medicines\nThe correct answer is: discuss the risks and benefits of this therapy\nView Peer Results"}
{"id" : 1853, "question_text" : "A 16-year-old adolescent girl has recently become sexually active. Her mother would like to discuss contraceptive options. The patient reports that she does not want to become pregnant, but does not want to start a birth control method because her friends have experienced adverse effects. She and her boyfriend have been using the withdrawal method, and she has not become pregnant. Of the following, the BEST next step in management is to", "options" : "[\"discuss all forms of birth control and their side effects\", \"provide her with condoms\", \"schedule a follow-up visit in 3 months to reassess her thoughts about birth control\", \"test for gonorrhea, chlamydia, HIV, and syphilis\"]", "explanation" : "Adolescence is a time of physical, psychological, and social maturity as individuals transition from childhood to adulthood. Neuroimaging studies have demonstrated dramatic development of the brain during adolescence and into young adulthood, with the development of higher cognitive functioning such as decision making, organization, hypothetical thought, and reasoning skills occuring later. Throughout adolescence there is progression from concrete thought to abstract thought. Individuals who engage in concrete thought tend to focus on physical objects and literal meanings, whereas individuals who engage in abstract thought can think about experiences they have not had or think through potential consequences of behavior. Not everyone has a smooth transition from concrete to abstract thought, and many adults remain concrete in their thought processes. This transition period can often be difficult for the adolescent as well as parents, teachers, and health care providers.\n\nThe developmental phases of adolescence are divided into the early stage (10-13 years of age), middle stage (14-17 years of age), and late stage (18-21 years of age). There are psychosocial processes associated with each stage. Young adulthood is defined as 18 to 25 years of age.\n\nEarly adolescence (10-13 years of age) is characterized by the initiation of puberty, self-absorption, impulsivity, and concrete thought. Children in this stage are trying to establish independence from their parents and are less interested in family related activities. They start to rely on their friendships for support. These youth are adjusting to their new bodies. They can be preoccupied with themselves and may develop dissatisfaction with how they look. This is a time when adolescents may start to diet, compare themselves to others, and develop eating disorders. Youth in early adolescence often think that everyone else is looking at or thinking about them (ie, \"the imaginary audience\"). They also start to develop emotional feelings and may have opposite- or same-sex attractions. Youth in this stage often have a need for privacy. They might start writing in a diary or closing the door to their room. They will also test the limits of their parents and other authority figures to see what they can get away with. Because they can also be impulsive, they are prone to taking more risks. As providers, it is important that we consider what we say to youth in this stage of development because our recommendations, comments, and advice may be misconstrued with these concrete thinkers.\n\nMiddle adolescence (14-17 years of age) is characterized by the peer group becoming even more important, greater independence, development of personal identity, and movement from concrete to abstract thought. School becomes more academically challenging and encourages these youth to develop better organizational skills and think more abstractly. This is often the most trying period of time for parents. Peer groups or \"cliques\" become a primary focus, with a great need for youth to conform in terms of thoughts, values, and clothing. Peer pressure often becomes an issue. Romantic relationships develop, and during this stage teenagers often experiment and become sexually active. There is also planning for the future in regard to career goals. This stage of development is known for the \"personal fable.\" For example, a youth may believe someone else can get pregnant if they have unprotected sex, but it would not be an issue for themself. Adolescents in this stage often feel invincible and will engage in high-risk behaviors, such as drug and alcohol use, risky sexual behavior, and dangerous driving. The leading cause of death for individuals 15 to 19 years of age are accidents (unintentional injuries). Adolescents between 15 and 19 years of age also have the highest incidence rates of sexually transmitted infections (STIs). Youth in this stage are also able to appreciate and learn from their own experiences.\n\nLate adolescence (18-21 years of age) is characterized by more intense planning for the future, the ability to think abstractly, comfort with one's identity and self, and taking on more responsibility and functioning independently of one's parents. During this stage, pubertal changes are complete, but youth may still be focused on improving how their body looks by diet and exercise. There is often more focus on individual relationships rather than peer group interactions. One's values and beliefs are more clearly defined. Youth in this stage will often start to appreciate that their parents do have valuable advice to share and are knowledgeable.\n\nThe adolescent girl in this vignette is an example of a youth in middle adolescence. She is aware she does not want to become pregnant but is engaging in high-risk sexual behaviors by having unprotected sex. This alludes to the personal fable. She is hesitant to start a birth control method based of the experiences of her friends. It would not be useful to discredit her friends, as their opinions carry weight. But as her provider, you can provide information (risks, benefits, and side effects) on all of the available methods so that she can make an independent and informed decision on her own. It would be helpful to assess her readiness to incorporate this information into her decision-making process via motivational interviewing techniques.\n\nThe provision of condoms and STI screening need to be addressed, but are not the best way to reduce this teenager's risk for pregnancy. For a sexually active teenager, STI screening should be done at a minimum of once per year. Offering condoms and encouraging use is a fundamental component of safe sex counseling. Waiting 3 months to let this adolescent contemplate if she would like a birth control method increases her chance of pregnancy.\n\nPREP Pearls\n\nAdolescence is a time of biopsychosocial development, and there are significant cognitive shifts from concrete to abstract thought.\n\nThe goal of moving through adolescence is to become an independent and productive individual in society.\n\nConsider what stage of development an adolescent is in when deciding the best way to counsel about a medical-related issue.\n\nABP Content Specifications(s)/Content Area\n\nUnderstand the timing of and factors influencing the development of concrete thinking and abstract reasoning in adolescents, and provide health advice accordingly\n\nSuggested Readings\n\nHazen E, Schlozman S, Beresin E. Adolescent psychological development: a review. Pediatr Rev. 2008:29(5):161-168. doi: 10.1542/pir.29-5-161.\n\nSanders RA. Adolescent psychosocial, social, and cognitive development. Pediatr Rev. 2013:34(8):354-359. doi: 10.1542/pir.34-8-354.\n\nSherer S, Radzik M. Psychosocial development in normal adolescents and young adults. In: Neinstein LS, Katzman DK, Callahan ST, Gordon CM, Joffe A, Rickert VI, eds. Neinstein's Adolescent and Young Adult Health Care: a Practical Guide. 6th ed. Philadelphia, PA: Wolters Kluwer; 2016: 38-43."}
{"id" : 179, "question_text" : "You are seeing a 15 month-old boy who has been placed in foster care. He was born to a 22-year-old human immunodeficiency virus (HIV)-positive woman who had a history of intravenous drug use. His birthweight was 2,850 g. The child experienced no neonatal complications and, at 13 months of age, was found to be HIV-negative and hepatitis C antibody-positive. At the time of his office visit today, the boy appears well and demonstrates no abnormal physical findings. Based upon his perinatal exposure, you obtain the following laboratory studies:\n• Hemoglobin, 12.5 g/dL (125 g/L)\n• White blood cell count, 6.5x103/mcL (6.5x109/L) (40% neutrophils, 56% lymphocytes, 4% eosinophils)\n• Aspartate aminotransferase, 30 units/L (normal, 5 to 30 units/L)\n• Alanine aminotransferase, 35 units/L (normal, 10 to 30 units/L)\n• Hepatitis C RNA (PCR), 1x104 copies/mL\n\nOf the following, the MOST appropriate next management step includes", "options" : "[\"follow-up in 6 months\", \"interferon therapy\", \"lamivudine therapy\", \"liver biopsy\", \"pegylated interferon and ribavirin therapy\"]", "explanation" : "As demonstrated by the boy described in the vignette, maternal coinfection with human immunodeficiency virus (HIV) is a major risk factor for vertical transmission of the hepatitis C virus (HCV). Therapy for HCV is generally not recommended for children younger than 2 years of age in the absence of signs of hepatic decompensation, which is extremely rare during childhood. Furthermore, even in the absence of detectable HCV RNA, an infant may demonstrate HCV antibody in the serum until 18 months of age as a consequence of passively transferred maternal antibody. For children who have active infection, hepatitis C tends to follow an indolent course, which makes the timing of therapy controversial and dependent, in part, upon evidence of liver damage on biopsy as well as viral genotype. In chronic hepatitis C infection, serum transaminase values alone are poor predictors of disease severity and (in contrast to hepatitis B infection) should not be used to predict the clinical course or response to treatment. A prudent approach for the asymptomatic toddler in the vignette, who has HCV viremia and minimal elevation in liver enzymes, is to monitor both liver functions and hepatitis C profile (including quantitative assessment of viral load) in 6 months to confirm active infection and plan further follow-up. Other diagnostic studies or therapeutic interventions are not indicated.\n\nHCV is an enveloped, single-stranded RNA virus, first identified in 1989. Recently, significant new information has been gathered about the molecular biology, pathology, and treatment of HCV liver disease. Based on sequence analysis, six major HCV genotypes are recognized, with genotype I being the most prevalent in the United States (74%). The primary mode of acquisition during infancy and childhood is via vertical transmission. However, unlike the high transmission rate seen for hepatitis B virus, vertical transmission of HCV occurs in only 5% to 10% of deliveries. Factors associated with higher HCV vertical transmission rates include a maternal serum viral load of greater than 106 copies/mL, coinfection with HIV, prolonged or difficult delivery, and the use of internal fetal monitoring during delivery. Typically, HCV RNA reaches detectable concentrations several weeks after birth, but a positive antibody titer is not sufficient to indicate active infection in children younger than 18 months of age. Chronic infection is defined as the persistence of HCV RNA for at least 6 months after detection.\n\nTreatment guidelines have undergone significant modifications in recent years, owing to the discovery of therapeutic options that offer improved rates of viral eradication. The American Association for the Study of Liver Diseases currently recommends treatment with a combination of pegylated interferon plus ribavirin, and recent evidence in pediatric patients demonstrates the therapeutic efficacy of this therapy. In adult studies, a sustained virologic response (SVR) previously has been related directly to HCV genotype, with most data indicating that a durable response is achieved in only 40% to 50% of patients who have viral genotype I. However, one recent report describes a mean SVR greater than 80% in these patients. Individuals exhibiting viral genotypes II and III (common in Australia and Asia) demonstrate an SVR of approximately 80%, in response to pegylated interferon/ribavirin treatment. Recent adult studies have also shown that the addition of the protease inhibitor telaprevir to this regimen is effective in treating all three major genotypes and further may permit reducing the duration of therapy from 48 to 24 weeks. This treatment also appears to offer a greater likelihood of achieving an SVR in patients who have failed prior therapy without telaprevir.\n\nBecause HCV infection often exhibits a benign, nonprogressive course during childhood, clear pediatric management criteria have not yet been established. However, most centers recommend periodic monitoring of viral load and liver function profile before considering antiviral therapy. Children who have newly diagnosed HCV infection should undergo a thorough medical evaluation. Although uncommon, progressive liver disease, including hepatocellular carcinoma and cirrhosis necessitating liver transplantation, has been reported during childhood. Accordingly, periodic monitoring of all children who manifest chronic HCV infection should include ultrasonographic evaluation and measurement of serum alpha-fetoprotein. The requirement for liver biopsy in children is controversial, particularly for patients who have genotypes II and III, in whom earlier treatment (even in the absence of overt histologic evidence of liver disease) should be considered because of the high likelihood of achieving an SVR. However, particularly in patients who have HCV genotype I, which is the most likely genotype for the infant in the vignette, a liver biopsy may be useful in guiding therapy by evaluating the degree of liver injury in the setting of normal liver function test results.\n\nWhen combination therapy with pegylated interferon and ribavirin is used, current data indicate improved SVR rates in older patients, particularly in those who have HCV genotype II and III. Importantly, virologic response rates are independent of pretreatment serum transaminase values. Despite these advances in HCV therapy, the decision to treat the asymptomatic child who has little or no evidence of liver disease remains a difficult one.\n\nAmerican Board of Pediatrics Content Specification(s): Know the long-term outcome of hepatitis C infection: chronic carriers, chronic hepatitis, cirrhosis, hepatocellular carcinoma; Recognize that children with chronic hepatitis C infection should undergo periodic screening tests for hepatic complications, and that treatment regiments are available"}
{"id" : 3772, "question_text" : "A 6-year-old boy with ear drainage is brought to the otolaryngology clinic. Four weeks ago he underwent bilateral tympanostomy tube placement for recurrent otitis media. For the last 2 weeks, he has had persistent foul-smelling drainage from the right ear. He has a temperature of 37.2°C, blood pressure of 100/60 mm Hg, heart rate of 90 beats/min, and respiratory rate of 22 breaths/min. There is purulent drainage in the right ear canal, and the right tympanic membrane cannot be visualized. A patent tympanostomy tube is seen in the left tympanic membrane. Of the following, the BEST management of this boy's infection is", "options" : "[\"oral ciprofloxacin\", \"oral clindamycin\", \"topical neomycin\", \"topical ofloxacin\"]", "explanation" : "The best management for the infection of the boy in this vignette is topical ofloxacin. Certain pathogens, including Pseudomonas aeruginosa and Staphylococcus aureus, must be considered as the causative agents of tympanostomy tube otorrhea in older children when water penetration could have contributed to developing otorrhea. Thus, empiric therapy should have broad-spectrum activity. For tympanostomy tube otorrhea, topical therapy is more efficacious than systemic therapy. Therefore, of the agents and routes presented as therapeutic options, topical ofloxacin is preferred.\n\nThe clinical manifestations of Pseudomonas infections are varied as many clinical syndromes have been associated with this pathogen. Infections associated with Pseudomonas species include chronic otitis media, hot tub folliculitis, foot osteomyelitis associated with nail puncture trauma, and pneumonia in patients with cystic fibrosis. Additionally, nosocomial infections associated with Pseudomonas species include superinfection of burn wounds, ventilator-associated pneumonia, catheter-associated bloodstream infections, catheter-associated urinary tract infections, and surgical site infections. In immunocompromised hosts, bacteremia can lead to ecthyma gangrenosum, skin lesions that evolve from hemorrhagic pustules to black eschars.\n\nThe fluoroquinolones are the only antibiotic class that offer oral options for treatment of Pseudomonas infections. Other parenteral drugs with activity include higher-generation cephalosporins, such as ceftazidime and cefepime, and β-lactamase inhibitor combinations, such as piperacillin-tazobactam, carbapenems, and aminoglycosides. However, Pseudomonas species can have multiple mechanisms of drug resistance, and therapy should be guided by results of antimicrobial susceptibilities.\n\nOral ciprofloxacin has broad-spectrum activity, however, topical therapy is preferred for tympanostomy tube otorrhea. Oral clindamycin has activity against susceptible S aureus but not Pseudomonas species. Neomycin is an aminoglycoside antibiotic that has a narrower spectrum of antimicrobial activity compared to fluoroquinolone antibiotics and more potential for adverse effects including ototoxicity and contact dermatitis. Thus, neomycin is not recommended for tympanostomy tube otorrhea.\n\nPREP Pearls\n• Pseudomonas infections include tympanostomy tube otorrhea, chronic otitis media, hot tub folliculitis, foot osteomyelitis associated with nail puncture trauma, pneumonia in patients with cystic fibrosis, and multiple nosocomial infections.\n• Although select β-lactam antibiotics, fluoroquinolones, and aminoglycosides can have activity against Pseudomonas species, therapy should be guided by results of antimicrobial susceptibilities given the potential for drug resistance.\n• Topical ofloxacin is the treatment of choice for tympanostomy tube otorrhea.\n\nABP Content Specifications(s)\n• Recognize the clinical manifestations of pseudomonal infections and manage appropriately\n• Recognize the risk factors for the development of pseudomonal infections\n\nSuggested Readings\n• American Academy of Pediatrics. Serious bacterial infections caused by Enterobacteriaceae. In: Kimberlin DW, Brady MT, Jackson MA, Long SS, eds. Red Book: 2018 Report of the Committee on Infectious Diseases. 31st ed. Itasca, IL: American Academy of Pediatrics; 2018:328-331. https://redbook.solutions.aap.org/chapter.aspx?sectionId=189640085&bookId=2205&resultClick=1.\n• Light M. Pneumonia. In: McInerny TK, Adam HM, Campbell DE, DeWitt TG, Foy JM, Kamat DM, eds. American Academy of Pediatrics Textbook of Pediatric Care. 2nd ed. Elk Grove Village, IL: American Academy of Pediatrics; 2017:2510-2522. Pediatric Care Online.\n• Steele D, Adam G, Di M, Halladay C, Balk E, Trikalinos T. Prevention and treatment of tympanostomy tube otorrhea: a meta-analysis. Pediatrics. 2017;139(6):e20170667. doi:10.1542/peds.2017-0125."}
{"id" : 1318, "question_text" : "An 8-year-old boy presents to your office for a health supervision visit. The family recently moved to the state and the boy has had difficulty adjusting. A review of systems is significant for longtime daytime and nocturnal enuresis. His family history is significant for nocturnal enuresis in the dad until age 12 years. Physical examination reveals a pale and visibly anxious patient with a temperature of 37.8°C, heart rate of 110 beats/min, respiratory rate of 16 breaths/min, and blood pressure of 125/80 mm Hg. His weight is 22 kg (10th percentile) and height is 115 cm (< 5th percentile). The boy's physical examination is otherwise normal. His urinalysis demonstrates a specific gravity of 1.005, pH of 6.0, and 3+ protein, with no blood, leukocyte esterase, or nitrates. Of the following, the MOST likely cause of this boy's enuresis is", "options" : "[\"chronic kidney disease\", \"genetic\", \"psychogenic polydipsia\", \"stress\", \"urinary tract infection\"]", "explanation" : "Enuresis is diagnosed in children aged 5 years or older who void in bed or their clothes twice or more per week for 3 consecutive months. Primary enuresis occurs in children with no interval of sustained dryness. Secondary enuresis is diagnosed in children with sustained dryness for a period of 6 months (for nocturnal enuresis) or 3 months (for diurnal enuresis).\n\nPolyuria is characterized by increased total urine volume resulting from an underlying defect in water balance. This presents with the excretion of large volumes of dilute urine, as seen in patients with chronic kidney disease, diabetes mellitus (osmotic diuresis), diabetes insipidus (antidiuretic hormone disorders), and psychogenic polydipsia. It is important to note that the symptoms of frequency, nocturia, or enuresis are often not associated with increased urinary volume.\n\nThe 8-year-old boy in the vignette has an abnormal voiding pattern of primary daytime and nocturnal enuresis, which needs further evaluation. His growth restriction (height < 5th percentile), pallor, and elevated blood pressure suggest an underlying chronic kidney disease (CKD). Furthermore, symptoms of enuresis and a specific gravity of 1.005 on urinalysis point to an underlying urine concentration defect associated with increased urine volume. Proteinuria (3+) on urine dipstick analysis is also an indicator of underlying kidney disease in this patient.\n\nCongenital anomalies of the kidney and urinary tract (CAKUT) and cystic kidney diseases (nonglomerular CKD) account for nearly 60% of pediatric CKD. Tubulointerstitial injury associated with CAKUT leads to reduced urinary concentration (acquired nephrogenic diabetes insipidus) and these patients usually present with polyuria with or without enuresis. Proteinuria can be seen in patients with underlying glomerular disease or tubulointerstitial injury. Persistent proteinuria may be the only indicator of renal disease in asymptomatic patients. Persistent dipstick-positive proteinuria or a urine protein-creatinine ratio higher than 0.2, is considered abnormal. Children with CKD usually have poor growth. In the North American Pediatric Renal Trials and Collaborative Studies, the mean height and weight of children with CKD were 1.44 and 0.88 standard deviations below age- and sex-specific normal values. Reduced renal erythropoietin production with CKD also leads to a normocytic normochromic anemia, consistent with anemia of chronic inflammation. These children are also at increased risk for iron and vitamin B12 deficient anemia because of the poor nutritional status associated with advanced stages of CKD.\n\nFurther evaluation in children suspected of having CKD should include urinalysis, serum creatinine, serum electrolytes, complete blood count, iron profile, and lipid profile, as well as ultrasonography of the kidneys. Glomerular filtration rate (GFR) is estimated from serum creatinine using the Schwartz formula (GFR = 0.413 ´ height [in centimeters]/serum creatinine [enzymatic method]) and is then used for diagnosing and stratifying risk in CKD.\n\nIn the early stages of CKD (GFR > 60 mL/min per 1.73 m2), patients are often asymptomatic. Symptoms of abnormal voiding patterns (enuresis or polyuria), poor growth, and pallor can be subtle and may be missed in the early stages of CKD. Patients with advanced stages of CKD associated with lower GFR are increasingly symptomatic.\n\nPsychogenic polydipsia presents with increased water intake. Laboratory testing would demonstrate a low urine osmolality, consistent with water overload. Maximal urine concentration is usually impaired (500-600 mOsm/kg) compared with that in normal patients (≥ 800 mOsm/kg). A parental history of nocturnal enuresis is associated with an increased risk for nocturnal enuresis in children. One or both the parents with a history of prolonged nighttime wetting have been reported, respectively, in nearly 50% and 75% of the children with nocturnal enuresis. Recent-onset stress, such as moving to a new home and school, can sometimes lead to secondary nocturnal enuresis in children, but is unlikely for the boy in the vignette who has poor growth and abnormal findings. In this case, urinary tract infection is an unlikely diagnosis in the absence of fever; urinary symptoms such as dysuria, flank pain, or burning micturition; and the absence of pyuria, nitrates, and bacteria on urinalysis. None of the response choices, other than CKD, explains the additional findings of poor growth, pallor, increased blood pressure, and proteinuria seen in this patient.\n\nPREP Pearls\n• Congenital anomalies of the kidney and urinary tract (CAKUT) and cystic kidney diseases (nonglomerular chronic kidney disease [CKD]) account for nearly 60% of pediatric CKD.\n• Tubulointerstitial injury associated with CAKUT leads to reduced urinary concentration (acquired nephrogenic diabetes insipidus) and usually presents with polyuria with or without enuresis.\n• In the early stages of CKD (GFR >60 mL/min per 1.73 m2), patients are often asymptomatic.\n• Symptoms of abnormal voiding patterns (enuresis or polyuria), poor growth, and pallor may be subtle and thereby missed in the early stages of CKD.\n\nABP Content Specifications(s)\n• Recognize the clinical and laboratory findings associated with voiding dysfunction\n• Identify the possible renal causes of nocturnal incontinence\n\nSuggested Readings\n• Kidney Disease Improving Global Outcomes, National Kidney Foundation. KDIGO 2012: Clinical practice guideline for the evaluation and management of chronic kidney disease. Kidney Int Suppl. 2013;3(1):1-163. http://www.kdigo.org/clinical_practice_guidelines/pdf/CKD/KDIGO_2012_CKD_GL.pdf.\n• Massengill SF, Ferris M. Chronic kidney disease in children and adolescents. Pediatr Rev. 2014;35(1):16-29. doi: http://dx.doi.org/10.1542/pir.35-1-16."}
{"id" : 3631, "question_text" : "A 16-year-old boy is brought to the emergency department by his parents after they had trouble awakening him this morning. They report that he has become increasingly fatigued over the past month and that he has been getting up multiple times at night to use the bathroom. His medical history is significant for autism spectrum disorder, and he was started on risperidone 3 months ago for aggressive behavior. The boy is somnolent but answers questions appropriately. His temperature is 37°C, blood pressure is 90/50 mm Hg, heart rate is 120 beats/min, respiratory rate is 18 breaths/min, and oxygen saturation is 97% in room air. His weight is 110 kg. He has acanthosis nigricans over the nape of his neck, dry mucous membranes, and a capillary refill time of 4 seconds. The remainder of his physical examination findings are unremarkable. Laboratory evaluation shows the following. Laboratory Test Result Plasma glucose 1,060 mg/dL (58.27 mmol/L) Sodium 154 mg/dL (154 mmol/L) Bicarbonate 22 mEq/L (22 mmol/L) Blood urea nitrogen 60 mg/dL (21 mmol/L) Creatinine 1.8 mg/dL (159 μmol/L) Serum osmolality 388 mOsm/kg Venous pH 7.34 Serum ketones Negative Urine ketones Negative. Of the following, the BEST next management step for this boy is to administer intravenous", "options" : "[\"half-normal saline with potassium at twice-maintenance rate\", \"insulin at 0.1 unit/kg bolus\", \"insulin at 0.1 units/kg per hour\", \"normal saline, 20 mL/kg bolus; repeat as needed to restore perfusion\"]", "explanation" : "The boy in the vignette has hyperglycemic hyperosmolar state (HHS), most likely associated with type 2 diabetes mellitus (DM) and risperidone therapy. He has severe hyperglycemia, hyperosmolality, dehydration, and mental status change without ketosis or significant acidosis. The best next management step for this boy is to administer a 20-mL/kg bolus of intravenous normal saline. The bolus should be repeated as needed to restore perfusion. Because dehydration and electrolyte loss are often profound in HHS, fluid therapy is the first step in treatment. In contrast to diabetic ketoacidosis (DKA), large volumes of fluid are usually indicated. \n\nPotassium should be replaced aggressively. Phosphate and magnesium levels should be monitored closely and deficits replaced as indicated. Half-normal saline with potassium (40 mEq/L) at twice-maintenance rate may be an appropriate fluid regimen after peripheral perfusion is restored, but would not be the best next management step.\n\nInsulin is indicated for the treatment of HHS, but at lower doses and later in the course of treatment compared with the treatment of DKA. Guidelines recommend starting a continuous infusion of insulin at 0.025 to 0.05 units/kg per hour after glucose levels stop declining by at least 50 mg/dL per hour with fluid administration alone. Insulin boluses are not recommended.\n\nHyperglycemic hyperosmolar state is defined as:\n• Significant hyperglycemia: Plasma glucose concentration >600 mg/dL (33.3 mmol/L)\n• Lack of acidosis: Arterial pH >7.30 or venous pH >7.25, serum bicarbonate >15 mmol/L\n• Absent to small ketonemia, small ketonuria\n• Hyperosmolarity: Serum osmolality >320 mOsm/kg\n• Mental status changes: Obtundation, combativeness, or seizures\n\nHyperglycemic hyperosmolar state is associated with type 2 DM as well as treatment with antipsychotic medications (eg, risperidone). The boy in the vignette has obesity and acanthosis nigricans. Both are risk factors for type 2 DM. Although rare, HHS is associated with significant risk of mortality related to the severity of dehydration.\n\nPREP Pearls\n• Hyperglycemic hyperosmolar state is associated with severe hyperglycemia, hyperosmolality, dehydration, and mental status change without significant ketosis or acidosis.\n• In contrast to diabetic ketoacidosis, large volumes of fluid are usually indicated for the treatment of hyperglycemic hyperosmolar state.\n• Hyperglycemic hyperosmolar state is associated with type 2 diabetes and antipsychotic medications (eg, risperidone).\n\nABP Content Specifications(s)\n• Plan appropriate fluid therapy for a patient with hyperosmolar non-ketotic coma\n\nSuggested Readings\n• Fayfman M, Pasquel FJ, Umpierrez GE. Management of hyperglycemic crises: diabetic ketoacidosis and hyperglycemic hyperosmolar state. Med Clin North Am. 2017;101(3):587-606. doi:10.1016/j.mcna.2016.12.011.\n• Maletkovic J, Drexler A. Diabetic ketoacidosis and hyperglycemic hyperosmolar state. Endocrinol Metab Clin North Am. 2013;42(4):677-695. doi:10.1016/j.ecl.2013.07.001.\n• Wolfsdorf JI, Glaser N, Agus M, et al. ISPAD Clinical practice consensus guidelines 2018: diabetic ketoacidosis and the hyperglycemic hyperosmolar state. Pediatr Diabetes. 2018;19(suppl 27):155-177. doi:10.1111/pedi.12701.\n• Zeitler P, Haqq A, Rosenbloom A, Glaser N; Drugs and Therapeutics Committee of the Lawson Wilkins Pediatric Endocrine Society. Hyperglycemic hyperosmolar syndrome in children: pathophysiological considerations and suggested guidelines for treatment. J Pediatr. 2011;158(1):9-14. doi:10.1016/j.jpeds.2010.09.048."}
{"id" : 2886, "question_text" : "An 11-year-old who was assigned female at birth is seen for a health supervision visit. He has been diagnosed with gender dysphoria by a psychologist who has followed him for several years. He asks to be called \"Michael\" and uses the pronouns \"he\" and \"him.\" His mother states that he displays typical male play behaviors; prefers typical male clothing, including underwear; and has insisted that he is a boy since about age 3 years. He became very distressed when he recently began showing signs of typical female puberty. An interdisciplinary gender team has recommended that he start receiving a gonadotropin-releasing hormone agonist to suppress puberty. His parents have consented, and he has provided assent for this therapy. They understand the potential adverse effects, including that on bone health. He is wearing typical male clothing and has a typical male hair style. His sexual maturity rating is 2 for breast and pubic hair development. The remainder of his physical examination findings are normal. Of the following, the team's action that BEST represents the ethical principle of beneficence is", "options" : "[\"ensuring that the parents have consented to therapy\", \"ensuring that the child and family understand potential adverse effects of therapy\", \"making the recommendation to initiate therapy\", \"obtaining assent of the child before the initiation of therapy\"]", "explanation" : "Correct Answer: C\nThe child described in the vignette is a transgender male who is showing worsening gender dysphoria with the onset of typical female puberty. The interdisciplinary gender team's action that best represents the ethical principle of beneficence is making the recommendation to initiate therapy with a gonadotropin-releasing hormone (GnRH) agonist to suppress puberty.\n\nBeneficence is the ethical principle concerned with benefiting others. Therapy with a GnRH agonist benefits the child by suppressing pubertal progression, an unwanted outcome from the child's perspective, with the goal of improving gender dysphoria. This therapy also facilitates full gender transition in the future, because the effects of normal female puberty would not need to be reversed. Although GnRH agonists are not formally approved by the United States Food and Drug Administration for gender-affirming care at this time, clinical practice guidelines support their use in the treatment of gender dysphoria.\n\nNonmaleficence is the ethical principle concerned with minimizing harm. Applied to this vignette, the effects of GnRH agonist are reversible if the decision to progress through female puberty is made in the future. Guidelines recommend waiting until the child has reached sexual maturity rating 2 to initiate GnRH agonist. This timing allows for confirmation of the gender dysphoria diagnosis (gender dysphoria worsens with the onset of puberty), and prevents unnecessary treatment in a prepubertal child.\n\nEnsuring that the child and family understand the potential adverse effects of therapy, ensuring that the parents have consented to therapy, and obtaining the child's assent before the initiation of therapy all relate to the ethical principle of autonomy. Autonomy is the principle concerned with allowing people to make decisions for themselves. These decisions should be informed and should be made with parents' consent and the child's assent.\n\nThe fourth ethical principle, justice, is concerned with fairness and equality. This principle relates to youth undergoing gender-affirming care in the potential inequalities in access to and insurance coverage of therapy.\n\nPREP Pearls\n• Therapy with a gonadotropin-releasing hormone agonist for youth undergoing gender-affirming care shows beneficence by suppressing pubertal progression with the goal of improving gender dysphoria.\n• Guidelines recommend waiting until youth undergoing gender-affirming care achieve sexual maturity rating 2 to initiate gonadotropin-releasing hormone agonist. This timing allows confirmation of the gender dysphoria diagnosis (gender dysphoria worsens with the onset of puberty), and prevents unnecessary treatment in a prepubertal child.\n\nABP Content Specifications(s)\n• Recognize and apply ethical principles involved in using new technologies for sex/gender assignment\n\nSuggested Readings\n• Hembree WC, Cohen-Kettenis PT, Gooren L, et al. Endocrine treatment of gender-dysphoric/gender-incongruent persons: an Endocrine Society clinical practice guideline (published corrections appear in J Clin Endocrinol Metab. 2018;103(2):699 and J Clin Endocrinol Metab. 2018;103(7):2758-2759). J Clin Endocrinol Metab. 2017;102(11):3869-3903. doi:10.1210/jc.2017-01658.\n• Jennings J, Jennings J. Trans teen shares her story. Pediatr Rev. 2016;37(3):99-100. doi:10.1542/pir.2016-002.\n• Kimberly LL, Folkers KM, Friesen P, et al. Ethical issues in gender-affirming care for youth. Pediatrics. 2018;142(6):e20181537. doi:10.1542/peds.2018-1537.\n• Lopez X, Stewart S, Jacobson-Dickman E. Approach to children and adolescents with gender dysphoria. Pediatr Rev. 2016;37(3):89-96. doi:10.1542/pir.2015-0032.\n• Shumer DE, Nokoff NJ, Spack NP. Advances in the care of transgender children and adolescents. Adv Pediatr. 2016;63(1):79-102. doi:10.1016/j.yapd.2016.04.018."}
{"id" : 756, "question_text" : "You are evaluating a 4-year-old boy who complains of \"bouncing eyes:' His mother reports seeing his eyes jiggle for a few seconds at a time. There are no abnormal head, neck, trunk, or limb movements. He is awake during these episodes and complains that his eyes are \"bouncing. \" On physical examination, the pupils are round and equally reactive to light, and eye movements are conjugate and intact in all directions. On downward gaze, there is downward nystagmus. The remainder of his physical examination findings, including hair and skin, are unremarkable. Of the following, the MOST likely diagnosis is", "options" : "[\"Chiari I malformation\", \"congenital nystagmus\", \"neurofibromatosis type 1\", \"phenytoin ingestion\", \"spasmus nutans\"]", "explanation" : "The boy described in the vignette has downbeat nystagmus, a subtype of vertical nystagmus. Vertical nystagmus is never normal and is usually the result of a brainstem abnormality. (Horizontal nystagmus can sometimes be normal, but a clinical evaluation is necessary, if it is a new finding.)\n\nIn this case the most likely cause is a Chiari I malformation. Chiari I malformation is defined as the descent of the cerebellar tonsils at least 5 mm below the foramen magnum. Although Chiari I malformation is often an incidental finding, it can cause neurologic problems. Symptomatic Chiari I malformation can cause brainstem compression, which presents with dysphagia, dysarthria, upbeat or downbeat nystagmus, or limb weakness with hyperreflexia. Headache with Valsalva. maneuver (coughing, straining, or laughing, for example) can also be a symptom. Chiari I malformation can also be associated with spinal cord syrinx. In a clinically unstable patient, computed tomography of the head is the best test to evaluate for structural brain abnormality; in stable patients for whom magnetic resonance imaging is safe, this modality will yield greater information. The treatment for symptomatic Chiari I malformation is surgical decompression.\n\nCongenital nystagmus is present at birth and persists throughout life. The nystagmus is most often in the horizontal direction but can be vertical. No oscillopsia—the subjective sensation of objects moving in the visual field—is reported. Congenital nystagmus is often an isolated, benign finding, but can be associated with rare neurogenetic disorders such as Pelizaeus-Merzbacher disease or brain malformations.\n\nNeurofibromatosis type 1 is associated with optic nerve gliomas and asymptomatic brain lesions, termed focal areas of signal abnormality, but neither of these cause downbeat nystagmus.\n\nPhenytoin can cause horizontal nystagmus especially when given in high or loading doses. It does not cause vertical nystagmus.\n\nSpasmus nutans, an idiopathic condition of infancy, is characterized by horizontal nystagmus, head tilting, and head nodding. It does not cause downbeat nystagmus. If the clinical diagnosis is uncertain, head and neck imaging should be performed to evaluate for structural abnormalities of the eye, brain, or neck.\n\nPREP Pearls\n• Vertical nystagmus can be caused by Chiari I malformation and is never normal; evaluation for brainstem abnormality is required.\n• In a clinically unstable patient, computed tomography of the head is the best test to evaluate for structural brain abnormality; in stable patients for whom magnetic resonance imaging is safe, this modality will yield greater information.\n\nAmerican Board of Pediatrics Content Specification(s):\n• Recognize that nystagmus may signify important eye or central nervous system pathology"}
{"id" : 2871, "question_text" : "An 18-year-old patient is seen in the office to request sexually transmitted infection screening. He noticed that his 19-year-old male partner, whom he has been dating for the past 10 months, has a rash on the palms of his hands. He did an online search and is worried he may have been exposed to an infection. He has engaged in oral and anal sex (insertive and receptive) and uses condoms infrequently. He has never been screened for sexually transmitted infections. He reports no dysuria, penile discharge, lesions, recent fever, or viral illnesses. His physical examination findings are normal, and he does not have any skin lesions. Of the following, the BEST initial step in treatment is to", "options" : "[\"administer azithromycin 1 g orally once and ceftriaxone 250 mg intramuscularly once\", \"administer penicillin G benzathine 2.4 million units intramuscularly once\", \"discuss pre-exposure prophylaxis and safer sex practices to reduce risk of sexually transmitted infections\", \"order a rapid plasma reagin test and tests for Neisseria gonorrh\\u0153ae, Chlamydia trachomatis, and HIV\"]", "explanation" : "Correct Answer: D\nThe asymptomatic adolescent boy described in the vignette is at risk of acquiring sexually transmitted infections (STIs). The first step in his treatment would be to order screening tests for gonorrhea, chlamydia, HIV, and syphilis. He does not have a partner who has been confirmed to have an STI, and he has not yet been screened, so he would not be treated presumptively for syphilis, gonorrhea, or chlamydia infections. Although he would benefit from a discussion about his options for reducing his risk of acquiring STIs, he has never been screened and is engaging in high-risk behaviors.\n\nSyphilis is caused by the spirochete Treponema pallidum and transmitted through sexual contact. Syphilis infections can be congenital or acquired. Congenital syphilis occurs as a result of intrauterine infection and can be spread transplacentally. Early congenital syphilis is defined as diagnosis before age 2 years; late congenital syphilis is diagnosed at age 2 years or older. Infants with early congenital syphilis may present with a rash, snuffles, hepatosplenomegaly, and thrombocytopenia. Infants diagnosed with late congenital syphilis may present with developmental delay, anterior bowing of the shins on plain radiographs, and Hutchinson teeth.\n\nAcquired syphilis infections in childhood and adulthood are divided into primary, secondary, latent, and tertiary-stage syphilis. Primary syphilis is characterized by a painless ulcer or chancre that occurs at the site of inoculation and spontaneously resolves in 3 to 6 weeks without any treatment. Secondary syphilis can occur weeks to months after inoculation and may present with a diffuse maculopapular rash that may affect the palms and soles, condyloma lata, fever, malaise, and lymphadenopathy. These symptoms can also spontaneously resolve in 3 weeks to 3 months. Latent syphilis is asymptomatic; however, if affected patients are screened, they will be seropositive. Latent syphilis is further divided into early (acquired within the preceding 12 months) and late (acquired more than 12 months prior) types. Tertiary-stage syphilis occurs decades after initial infection and is characterized by dementia, cardiovascular involvement such as aortic aneurysms, and development of granulomas or gummas.\n\nSyphilis is diagnosed in a stepwise fashion, first with a nontreponemal screening test (rapid plasmin reagin or Venereal Disease Research Laboratory test) and, if that first test result is positive, a treponema-specific test (fluorescent treponemal antibody absorption test). A second-step confirmatory test is necessary because false-positive nontreponemal test results can occur with pregnancy, tuberculosis, other viral infections, and autoimmune disease. The treatment for primary, secondary, and early latent syphilis is one dose of penicillin G benzathine 2.4 million units intramuscularly; the treatment for late latent syphilis and tertiary syphilis is penicillin G benzathine 2.4 million units intramuscularly once per week for 3 doses.\n\nThere has been an increase in the number of cases of syphilis in the United States in all stages. In 2018, there were more than 35,000 cases of primary and secondary syphilis reported to the Centers for Disease Control and Prevention, and 64% of the cases were among men who have sex with men.\n\nPREP Pearls\n• There has been an increase in syphilis cases in the United States, with the majority among men who have sex with men.\n• The four stages of syphilis are primary, secondary, latent, and tertiary, each with varying clinical manifestations.\n• Syphilis is diagnosed in a stepwise fashion, first with a non treponemal screening test (rapid plasmin reagin or Venereal Disease Research Laboratory test); if that result is positive, a treponema-specific test (fluorescent treponemal antibody absorption test).\n\nMOCA-Peds Objective\n• Screen an adolescent for sexually transmitted diseases and manage appropriately.\n\nABP Content Specifications(s)\n• Recognize the clinical features associated with congenital and acquired Treponema pallidum infection\n• Plan appropriate management for a patient with Treponema pallidum infection\n\nSuggested Readings\n• American Academy of Pediatrics. Syphilis. In: Kimberlin DW, Brady MT, Jackson MA, Long SS, eds. Red Book: 2018 Report of the Committee on Infectious Diseases. 31st ed. Itasca, IL; American Academy of Pediatrics; 2018:773-788. Red Book Online.\n• Centers for Disease Control and Prevention. Sexually Transmitted Disease Surveillance 2018. Atlanta, GA: U.S. Department of Health and Human Services; 2019. doi:10.15620/cdc.79370.\n• Peeling RW, Mabey D, Kamb ML, Chen XS, Radolf JD, Benzaken AS. Syphilis. Nat Rev Dis Primers. 2017;12(3):1-21. doi:10.1038/nrdp.2017.73."}
{"id" : 1421, "question_text" : "A 4-year-old boy is brought to your clinic for follow-up after a recent hospitalization 1 week ago. Discharge summary shows that he presented with cough and tachypnea and a temperature of 35.8°C. His chest radiograph showed a lobar pneumonia. He received intravenous antibiotics and required oxygen in the hospital for 2 days, but he never had fever. In clinic, his mother says he is improved. On physical examination, his temperature is 35°C, heart rate is 98 beats/min, respiratory rate is 20 breaths/min, and body mass index is 18 kg/m2 (93rd percentile). He is well-appearing and has no respiratory distress.\n\nOf the following, the test MOST likely to reveal the cause of the hypothermia is", "options" : "[\"blood pressure\", \"magnetic resonance imaging of the head\", \"repeat chest radiograph\", \"serum glucose\", \"serum immunoglobulins\"]", "explanation" : "The boy in the vignette has persistently low temperature, even during a lobar pneumonia. This implies a disorder of the thermoregulatory system, which is controlled in the hypothalamus. The hypothalamus interacts closely with the endocrine system and the autonomic nervous system to maintain thermostasis. Of the answers, the best test to diagnose a disorder of the thermoregulatory system is magnetic resonance imaging of the head. Lesions of the hypothalamus can include tumors such as gliomas or craniopharyngiomas, cysts, and granulomas as in Langerhans cell histiocytosis. Other symptoms of hypothalamic dysfunction include polyphagia and obesity (as seen in the boy in the vignette), hypersomnolence, precocious puberty, short stature, polydipsia, polyuria, and symptoms of adrenal insufficiency. Hypothalamic dysfunction is increasingly recognized in children, for example, as Rapid-Onset Obesity With Hypothalamic Dysfunction, Hypoventilation, and Autonomic Dysregulation (ROHHAD syndrome).\n\nHypothermia can have many causes. Imbalance between the ability to produce heat and the ability to prevent heat loss is more common than an abnormal thermoregulatory control system (due to a hypothalamic tumor or injury, for instance). In neonates, the inability to prevent heat loss contributes more to hypothermia than an immature thermoregulatory control system. Inability to produce heat, as in severe malnutrition, can contribute to the development of hypothermia. Other causes of hypothermia include sepsis, hypothyroidism, and hypoglycemia. Although no blood pressure is reported, hypertension or hypotension are unlikely to cause persistent hypothermia in this child. Chest radiograph is not indicated because the boy's respiratory symptoms have improved. Serum glucose and serum immunoglobulins are not indicated for this boy's current clinical presentation and would not be helpful in finding a cause of his hypothermia.\n\nPREP Pearls\n• Persistent hypothermia can be a sign of hypothalamic dysfunction.\n• Hypothalamic tumors can impair temperature regulation.\n\nABP Content Specifications(s)\n• Understand the effects of the immature/abnormal hypothalamic thermoregulatory system on the development of fever in infants and children who have diseases of the central nervous system"}
{"id" : 332, "question_text" : "A 6-year-old boy who has moderate persistent asthma has experienced more frequent asthma symptoms as well as nasal congestion and headaches for the past 4 weeks. Recently, he went to the dentist because of upper tooth pain, but the dentist stated his examination findings were normal, and there was no evidence of dental caries. Of the following, the MOST likely cause for the boy's symptoms is", "options" : "[\"allergic rhinitis\", \"bacterial sinusitis\", \"migraine headache\", \"nonallergic rhinitis\", \"viral upper respiratory tract infection\"]", "explanation" : "The combination of nasal congestion, headaches, worsening asthma control, and upper tooth pain described for the boy in the vignette is concerning for acute bacterial sinusitis. General expert consensus recommends that symptoms should persist longer than 7 to 10 days to make this diagnosis. Other signs and symptoms of sinusitis include purulent nasal discharge, fever, facial pressure or congestion, anosmia, halitosis, cough, otalgia, and fatigue.\n\nAllergic rhinitis can be a risk factor for an asthma exacerbation or acute sinusitis, but discolored rhinorrhea and tooth pain are not consistent with uncomplicated allergic rhinitis. Primary headache disorders such as migraines often result in head and sinus pain. If initial antibiotic treatment does not improve suspected sinus symptoms in a patient whose complaint is headache or pressure, consideration should be given to sinus imaging such as computed tomography scan. Nonallergic rhinitis is more common than allergic rhinitis in children and may result in symptoms that are similar to allergic rhinitis. Common causes of nonallergic rhinitis include gustatory rhinitis, vasomotor rhinitis (due to irritants such as cold air and strong odors), and nonallergic rhinitis with eosinophilia. The lack of a specific irritant associated with nonallergic rhinitis makes this unlikely for this boy. Viral upper respiratory tract infections can result in the symptoms described in this vignette, but symptoms persisting past 1 to 2 weeks are unlikely in these infections.\n\nAmerican Board of Pediatrics Content Specification(s):\n\nKnow that sinusitis should be included in the differential diagnosis of toothache, sore throat, persistent cough, and poorly controlled asthma"}
{"id" : 2538, "question_text" : "A chief medical officer (CMO) discovers that a physician at the hospital is receiving compensation for recommending a medical device to his patients. The CMO informs the physician about the institution's conflict of interest policies. Of the following, the BEST next step for the CMO is", "options" : "[\"require creation of a consulting agreement that stipulates that the physician must disclose the financial arrangement to his patients\", \"require creation of a document that discloses the financial relationship between the physician and the company to his employer\", \"require the physician and his staff to perform remedial training on the institution's relevant policies\", \"terminate the physician from practice immediately for unethical behavior\"]", "explanation" : "The physician in the vignette has a conflict of interest (COI). The Institute of Medicine in 2009 defined COI as \"a set of circumstances that create a risk that professional judgment or actions regarding a primary interest will be unduly influenced by a secondary interest.\" Receiving compensation for recommending a medical device to patients is a COI because medical judgment could be influenced by secondary financial gain. The physician might be more inclined to recommend the medical device even if it is not the best choice for the patient.\n\nThe best next step for the chief medical officer to mitigate the COI is to require creation of a consulting agreement that stipulates that the physician must disclose the arrangement to his patients. The agreement would require the physician to inform patients of the financial relationship before prescribing the medical device. Those already using the medical device must also be informed.\n\nCreation of a document that discloses the financial relationship to his employer is also important. However, the physician still must disclose the arrangement to his patients. Creation of a disclosure document alone would not suffice. Education regarding institutional policies that pertain to COIs is also important. However, education alone would not mitigate the COI that is occurring in the vignette. Depending on institutional policy, disciplinary action for not appropriately disclosing a COI may include termination. Disclosing the financial relationship to patients who have already been prescribed the medical device, however, is still required to help mitigate the COI.\n\nOther examples of COIs include acceptance of gifts from industry (eg, meals and drug samples), writing for or promoting an industrial product, and financial interest in a medical product that the physician prescribes, uses, or recommends. Conflicts of interest can be managed by instituting COI policies that address disclosure content, restricting participation of researchers with COIs, and providing ongoing education at all levels of participation (faculty, students, residents, and fellows).\n\nPREP Pearls\n• The Institute of Medicine in 2009 defined conflict of interest as \"a set of circumstances that create a risk that\n• professional judgment or actions regarding a primary interest will be unduly influenced by a secondary interest.\"\n• Examples of conflict of interest include acceptance of gifts from industry (eg, meals and drug samples), writing for or promoting an industrial product, and financial interest in a medical product that the physician prescribes, uses, or recommends.\n\nABP Content Specifications(s)\n• Recognize and apply ethical principles regarding conflicts of interest\n\nSuggested Readings\n• Muth CC. Conflict of interest in medicine. JAMA. 2017;317(17):1812. doi:10.1001/jama.2017.4044.\n• US Institute of Medicine Committee on Conflict of Interest in Medical Research, Education, and Practice; Lo B, Field MJ, eds. Conflicts of interest and medical practice. In: Conflict of Interest in Medical Research, Education, and Practice. National Academies Press; 2009:6.\n• Wilfond BS. Duenas DM, Johnson L-M. Conflicts of interest and recommendations for clinical treatments that benefit researchers. Am J Bioethics. 2020;20(10):90-91. doi:10.1080/15265161.2020.1806380."}
{"id" : 2808, "question_text" : "An 8-year-old girl is referred to her primary care provider by her dentist for evaluation of tongue and mouth lesions (Item Q7A and Item Q7B). Her mother recalls the lesions as being present for at least the past 3 years. They have not changed and are asymptomatic. The girl has no known medical problems. A review of systems is significant for intermittent constipation and diarrhea. No one in her family has similar lesions. Her weight is at the 25th percentile, and her height is at the 90th percentile. Her face is long and thin, and she has full lips. Her joints are hyperextensible, and pes planus is present. The remainder of her physical examination findings are unremarkable. Of the following, the BEST next step is to evaluate for", "options" : "[\"an insulinoma\", \"a medullary thyroid carcinoma\", \"an optic glioma\", \"a pheochromocytoma\"]", "explanation" : "Correct Answer: B\nThe girl in the vignette has multiple endocrine neoplasia type 2B (MEN2B). Her mouth lesions are mucosal neuromas. The mucosal neuromas; tall, thin body habitus; full lips; and joint laxity are all phenotypic features of MEN2B. Her intermittent constipation and diarrhea are typical symptoms of associated gastrointestinal tract ganglioneuromas. Although not mentioned for the girl in the vignette, alacrima from birth is also a feature of MEN2B.\n\nThe best next step is to evaluate for medullary thyroid carcinoma (MTC). Medullary thyroid carcinoma occurs in 100% of individuals with MEN2B, is aggressive, and metastasizes early. Other than resection, effective treatment options for MTC are limited. The American Thyroid Association recommends prophylactic thyroidectomy in the first year after birth for those with MEN2B. It is important to recognize the clinical features of MEN2B for timely prevention, detection, and treatment of the associated MTC.\n\nEvaluation for MTC includes thyroid ultrasonography and a serum calcitonin level test. Calcitonin is produced by the neuroendocrine C cells of the thyroid and is elevated in MTC. Pheochromocytoma is also associated with MEN2B, but the onset is later and the prognosis is significantly better than for MTC. Insulinoma and optic glioma are not associated with MEN2B. Insulinoma is associated with MEN type 1. Optic glioma is associated with neurofibromatosis type 1.\n\nMultiple endocrine neoplasia type 2 (2A and 2B) is caused by heterozygous pathogenic variants in the RET proto-oncogene. They are inherited in an autosomal dominant fashion. At-risk family members of those with a pathogenic RET proto-oncogene variant should also be tested. Most of those with MEN2B, however, harbor a de novo pathogenic variant.\n\nThere is significant genotype-phenotype correlation with RET pathogenic variants, which are stratified according to risk level. Although the penetrance of MTC in MEN type 2 (2A and 2B) is essentially 100%, the age of onset varies according to risk level. Multiple endocrine neoplasia type 2B is associated with the highest risk variants. Multiple endocrine neoplasia type 2A and familial medullary thyroid carcinoma are associated with less high, moderate, and lower risk variants. Features of MEN type 2A include hyperparathyroidism in addition to MTC and pheochromocytoma, but not the physical phenotype. The American Thyroid Association recommends timing of prophylactic thyroidectomy based on the risk level of the specific RET proto-oncogene pathogenic variant. Item C7 summarizes the clinical features of the conditions associated with RET proto-oncogene pathogenic variants.\n\nRisk for multiple endocrine neoplasia type 2A is often identified in children after genetic testing is performed because of family history. Papillary thyroid carcinoma is the most common type of thyroid cancer occurring in children and commonly presents as a thyroid nodule, cervical lymphadenopathy, or both.\n\nPREP Pearls\n• Phenotypic features of multiple endocrine neoplasia type 2B include mucosal neuromas; a tall, thin body habitus; full lips; joint laxity; and alacrima. Gastrointestinal tract ganglioneuromas may cause intermittent constipation and diarrhea.\n• Multiple endocrine neoplasia type 2B is associated with early-onset and aggressive medullary thyroid carcinoma, in addition to pheochromocytoma and the physical phenotype.\n• It is important to recognize the clinical features of multiple endocrine neoplasia type 2B for timely prevention (prophylactic thyroidectomy), detection, and treatment of the associated medullary thyroid carcinoma.\n\nABP Content Specifications(s)\n• Recognize the clinical features associated with thyroid carcinoma\n\nSuggested Readings\n• Anisowicz SK, McIver H, Pedersen AM. Visual diagnosis: exophytic lesions on tongue and oral mucosa. Pediatr Rev. 2018;39(9):e43-e46. doi:10.1542/pir.2017-0184.\n• Francis GL, Waguespack SG, Bauer AJ, et al; American Thyroid Association Guidelines Task Force. Management guidelines for children with thyroid nodules and differentiated thyroid cancer. Thyroid. 2015;25(7):716-759. doi:10.1089/thy.2014.0460.\n• Kochmann M, Bansal S, Umpaichitra V, Perez-Colon S. Visual diagnosis: an 11-year-old girl with swollen lips and oral bumps. Pediatr Rev. 2017;38(10):e38-e40. doi:10.1542/pir.2016-0059.\n• Nagaoka R, Sugitani I, Sanada M, et al. The reality of multiple endocrine neoplasia type 2B diagnosis: awareness of unique physical appearance is important. J Nippon Med Sch. 2018;85(3):178-182. doi:10.1272/jnms.JNMS.2018_85-26.\n• Osipoff JN, Wilson TA. Consultation with the specialist: thyroid nodules. Pediatr Rev. 2012;33(2):75-81. doi:10.1542/pir.33-2-75."}
{"id" : 2836, "question_text" : "A 5-year-old boy is brought to the office by his mother who is concerned that her son has been intentionally banging his head. When he is watching videos, he may bang his head several times against the back of his chair. He may bang his head against the wall when he does not want to do something or when he is upset. At bedtime, the boy bangs his head on his pillow until he falls asleep. His mother is worried that he is having headaches or may cause himself serious injury.\n\nOf the following, the MOST appropriate next step is", "options" : "[\"applied behavioral analysis therapy\", \"magnetic resonance imaging of the brain\", \"reassurance that these behaviors will resolve with time\", \"screening for a developmental disability\"]", "explanation" : "Correct Answer: D\nHead-banging is a repetitive motor behavior (stereotypy) that usually resolves by age 4 years in typically developing children. Persistence after age 5 years is associated with developmental disorders such as autism or intellectual disability. Thus, the most appropriate next step would be to screen for those conditions in this 5-year-old boy.\n\nHead-banging occurs in 5% to 15% of typically developing children. It usually begins at 9 to 18 months of age and stops by age 4 years. It occurs more commonly in boys. Some children will bang their forehead on walls, the floor, a mattress, or pillow; others will bang their occiput on a carseat. The behavior can last for less than 15 minutes to more than an hour. Head-banging may occur at bedtime or when the child is upset. It can result in bruising, abrasions, calluses, or hair loss. Significant head injury is rare.\n\nThe pediatric provider should provide reassurance about the head-banging to the parents of the toddler or preschooler, who is otherwise healthy and typically developing. Parents should be instructed to redirect their child's behavior and to avoid giving undue attention to the head-banging because they may inadvertently reinforce it. Providing reassurance would not be the appropriate next step for the 5-year-old child in the vignette.\n\nHead-banging can be more persistent and severe in a child with developmental disability or severe sensory impairment (ie, vision, hearing). In the context of a child with significantly impaired communication skills, self-injurious behaviors such as head banging, should prompt evaluation for painful medical conditions such as otitis media, sinusitis, or constipation, particularly when the behavior is acute. Applied behavioral analysis therapy may be an appropriate future step if this child were confirmed to have an autism spectrum disorder. Magnetic resonance imaging of the brain is not warranted at this time; it is rare for children with head-banging behavior to cause themselves significant injury.\n\nPREP Pearls\n• Head-banging is a repetitive motor behavior (stereotypy) that usually resolves by age 4 years in typically developing children. Persistence after age 5 years is associated with developmental disorders such as autism or intellectual disability.\n• Parents of typically developing toddlers or preschoolers who bang their heads should be instructed to redirect their child's behavior and to avoid giving undue attention to the head-banging because they may inadvertently reinforce it.\n• In the context of a child with significantly impaired communication skills, self-injurious behaviors such as head-banging should prompt evaluation for painful medical conditions such as otitis media, sinusitis, or constipation, particularly when the behavior is acute.\n\nABP Content Specifications(s)\n• Plan the appropriate management of head banging in toddlers and preschool-age children\n\nSuggested Readings\n• Blum NJ, Pipan ME. Basics of child behavior and primary care management of common behavioral problems. In: Voight RG, Macias MM, Myers SM, Tapia CD, eds. Developmental and Behavioral Pediatrics. Itasca, IL: American Academy of Pediatrics; 2018:91-110.\n• Sarles RM, Edwards S. Self-stimulating behaviors. In: McInerny TK, Adam HM, Campbell DE, DeWitt TG, Foy JM, Kamat DM, eds. American Academy of Pediatrics Textbook of Pediatric Care. 2nd ed. Itasca, IL: American Academy of Pediatrics; 2016;chap 192:1582-1584. Pediatric Care Online.\n• Senturias YSN. Behavior patterns in infancy and preschool. In: Feld LG, Mahan JD, eds. Succinct Pediatrics Book 3: Evaluation and Management for Newborn, Genetic, Neurologic, and Developmental-Behavioral Disorders. Elk Grove Village, IL: American Academy of Pediatrics; 2018:435-446.\n• Stein MS, Blum NJ, Lukasik MK. Self-injury and mental retardation in a 7-year-old boy. J Dev Behav Pediatr. 2005;26(3):241-245. doi: 0.1097/00004703-200506000-00013."}
{"id" : 1379, "question_text" : "You are called to the nursery to see a male neonate born at 38 weeks of gestation to a 24-year-old gravida 1 para 0 woman who received routine prenatal care. Ultrasonography performed at 30 weeks of gestation because of poor fetal growth revealed hyperechogenic bowel and periventricular intracranial calcifications. At delivery, the small-for–gestational age neonate was noted to have a diffuse petechial rash and hepatosplenomegaly. Of the following, the condition MOST commonly associated with this neonate's condition is", "options" : "[\"cerebral palsy\", \"learning disability\", \"seizures\", \"sensorineural hearing loss\", \"vision impairment\"]", "explanation" : "The newborn in the vignette has congenital cytomegalovirus (CMV) infection. Congenital CMV is the most common cause of nonhereditary sensorineural hearing loss. In utero manifestations of congenital CMV disease include the following:\n• Growth restriction\n• Hepatosplenomegaly\n• Hydrops\n• Hyperechogenic fetal bowel\n• Microcephaly\n• Oligo- or polyhydramnios\n• Periventricular intracranial calcifications\n\nMany neonates are asymptomatic. In those with symptoms, frequent findings include the following:\n• Hemolytic anemia\n• Hepatosplenomegaly\n• Hypotonia\n• Jaundice\n• Microcephaly\n• Petechiae\n• Seizures\n\nAlthough children with a history of congenital CMV infection can have cerebral palsy, intellectual disability, learning disability, or seizures, sensorineural hearing loss is the most common clinical sequela of congenital CMV infection. Hearing loss is identified in one-third to one-half of infants with symptomatic disease.\n\nHearing loss is a common pediatric condition. One in 1,000 newborns and 2 in 1,000 young children experience hearing loss; by age 18 years, 17 in 1,000 will have some degree of permanent hearing loss. Hearing loss in early childhood can lead to delays in speech, language, social, and cognitive development. Some children with hearing loss also experience delays in gross motor development. Untreated hearing loss can have dramatic effects on educational attainment and mental health. Although young infants with profound hearing loss will develop prelingual language skills at a normal pace, after age 6 to 9 months, they will lose these skills and will not progress. Parents may note that children with hearing loss do not make eye contact or turn to sound, although even those with profound hearing loss may react to shouts or other loud sounds because they can feel vibrations. Once hearing loss is identified, treatment aims to minimize the duration or degree of hearing loss, maximize remaining hearing, and provide appropriate strategies to optimize communication and development.\n\nHearing loss is classified as conductive, sensorineural, mixed, or central.\n\nPREP Pearls\n• Conductive hearing loss includes disruption of sound traveling to the cochlea or middle ear. Etiologies include congenital anomalies, blockages in the external canal, and conditions that affect the integrity and function of the tympanic membrane and ossicles.\n• Sensorineural hearing loss occurs at the cochlea, inner ear, or auditory nerve and includes hereditary hearing loss, congenital and postnatal infections, trauma, and exposure to drugs and noise.\n• Unrecognized and untreated hearing loss can lead to developmental delays and learning problems.\n\nABP Content Specifications(s)\n• Recognize conditions that contribute to hearing loss/impairment in patients of various ages, and the effects of that hearing loss on language development and learning\n• Understand the natural history and etiologies of conductive hearing loss\n• Understand the etiologies (eg, infectious, genetic, traumatic) of sensorineural hearing loss\n• Recognize age-related clinical findings associated with hearing loss of various etiologies\n\nSuggested Readings\n• Boppana SB, Ross SA, Fowler KB. Congenital cytomegalovirus infection: clinical outcome. Clin Infect Dis. 2013;57(suppl 4):S178-S181. doi: http://dx.doi.org/10.1093/cid/cit629.\n• Grindle C. Pediatric hearing loss. Pediatr Rev. 2014;35(11):456-464. doi: http://dx.doi.org/10.1542/pir.35-11-456.\n• Kral A, O'Donoghue GM. Profound deafness in childhood. N Engl J Med. 2010;363(15):1438-1450. doi: http://dx.doi.org/10.1056/NEJMra0911225."}
{"id" : 1832, "question_text" : "A 16-year-old adolescent girl with morbid obesity is seen for a health supervision visit. Her mother is concerned that she is failing 3 classes. During a HEADSS (home and environment; education, employment, and eating; activities; drugs; sexuality; suicide, depression, and safety) assessment, with her mother out of the room, the patient discloses that she is teased at school about her weight and that she feels alone because she does not have many friends. She reports that she has no suicidal thoughts. She has acanthosis nigricans on her neck and both axillae and several superficial well-healed scars on her left wrist. Of the following, the BEST next step in this patient's evaluation and management would be to", "options" : "[\"order hemoglobin A1C and fasting plasma glucose tests\", \"provide information on adolescent weight loss programs\", \"refer her to a dietitian for nutrition counseling\", \"refer her to a psychologist for counseling\"]", "explanation" : "Correct Answer: D\nSelf-esteem is defined as confidence in one's abilities and self-worth. Low self-esteem can influence an adolescent's psychological and physical development. The adolescent girl in this vignette is displaying signs of low self-esteem in that she is bullied about her appearance, does not have a support network at school, and has failing grades and evidence of nonsuicidal self-injury (NSSI) via cutting. She would benefit from counseling to address her thoughts about herself, identify areas that can be modified to improve her outlook, and develop coping skills to deal with comments from her peers at school.\n\nPoor self-esteem has been associated with anxiety, depression, and suicide. According to the fifth edition of the Diagnostic and Statistical Manual of Mental Disorders, major depressive episodes in adolescents usually last between 4 to 9 months and often go unrecognized. The key features of depression in adolescents are a depressed or irritable mood for the majority of the day, sadness, and lack of interest in activities. Other diagnostic criteria include changes in weight (> 5% loss or gain), insomnia or hypersomnia, fatigue or loss of energy, psychomotor agitation or slowing, feelings of worthlessness or guilt, poor concentration, and suicidal thoughts or attempts. The diagnosis requires 5 of the 9 criteria to be present for at least 2 weeks and must include a depressed or irritable mood and lack of interest in activities.\n\nChildren who are overweight and obese are more likely to be bullied. Bullying includes verbal commentary, physical threats, spreading rumors, and social isolation. Children who are bullied often have low self-esteem and higher rates of depression and other mental health disorders, are more prone to substance abuse, and may have limited opportunities because of dropping out of school. Schools that use anti-bullying interventions tend to have less violence overall. However, many schools do not get involved and leave it up to the child and their parents to identify solutions. Schools that do not provide a safe learning environment and fail to enforce no-bullying policies are conveying a message for students to tolerate and accept abusive behavior.\n\nNonsuicidal self-injury is inflicting harm to one's own body without intent of committing suicide. It may include cutting, burning, biting, or punching. There is an 18% lifetime prevalence of NSSI amongst adolescents. Adolescents that repeatedly engage in self-injurious behaviors have a greater risk of committing suicide. Individuals who self-injure often report engaging in these behaviors for short-term relief of stress, to punish themselves, to deal with feelings of emptiness, and to communicate their feelings to others. Pediatricians and other primary care providers are often the first to identify that an adolescent is engaging in NSSI. The SOARS (suicidal ideation, onset, aftercare, reasons, and stage of change) model can be used to screen for NSSI and determine the appropriate type of brief intervention.\n\nThe adolescent in this vignette is morbidly obese with acanthosis nigricans. She should be screened for type 2 diabetes mellitus with a hemoglobin A1C test and fasting plasma glucose test. She would also benefit from nutrition counseling by a dietitian and a discussion regarding adolescent weight loss programs in an effort to prevent long-term consequences such as cardiovascular disease, type 2 diabetes mellitus, hyperlipidemia, and nonalcoholic fatty liver disease. However, if her thoughts about herself and mood are not addressed first with therapy, it is unlikely she will be motivated to make other types of positive lifestyle changes toward improving her health.\n\nPREP Pearls\n\nAdolescents should be asked about factors that may influence their self-esteem, such as support networks at home and school, bullying, and self-injury.\n\nPsychotherapy can be used to help adolescents process their feelings and develop healthy coping mechanisms to address negative interactions with peers.\n\nABP Content Specifications(s)/Content Area\n\nIdentify outcomes and plan the management of a poor self-image in adolescence\n\nSuggested Readings\n\nGlew GM, Frey KS, Walker WO. Bullying update: are we making any progress? Pediatr Rev. 2010;31(9):e68-e74. doi: 10.1542/pir.31-9-e68.\n\nTrzesniewski KH, Donnellan MB, Moffitt TE, Robins RW, Poulton R, Caspi A. Low self-esteem during adolescence predicts poor health, criminal behavior, and limited economic prospects during adulthood. Dev Psychol. 2006;42(2):381-390. doi: 10.1037/0012-1649.42.2.381.\n\nWesters NJ, Muehlenkamp JJ, Lau M. SOARS model: risk assessment of nonsuicidal self-injury. Contemp Pediatr. 2016:33(7):25-31."}
{"id" : 1565, "question_text" : "A 14-year-old girl comes to your office for her routine health supervision visit. During the visit, her mother asks about the human papillomavirus (HPV) vaccine. She states that she has heard that the vaccine causes infertility and increases sexual activity in vaccinated girls. She is concerned about vaccinating her daughter and wants to know more about the vaccine before making a decision. You provide the mother with evidence-based information about the HPV vaccine. Of the following, the statement you are most likely to include in your discussion is that", "options" : "[\"the vaccine provides protection against noncervical HPV-associated cancers\", \"the vaccine is only effective if given before any sexual activity\", \"the vaccine should not be given to girls who have already initiated sexual activity\", \"vaccinated girls are more likely to engage in sexual activity than unvaccinated girls\", \"vaccination eliminates the need for cervical cancer screening\"]", "explanation" : "The statement you are most likely to include in your discussion is that the vaccine provides protection against noncervical HPV-associated cancers. Human papillomavirus (HPV) is the most common sexually transmitted infection in the United States. In a study conducted before HPV vaccine licensure, 25% of persons aged 14 to 19 years and 45% of those aged 20 to 24 years were infected. It is estimated that more than 80% of men and women in the United States will be infected with HPV during their lifetime.\n\nMost HPV infections are asymptomatic and resolve spontaneously. Infection with low-risk HPV types (eg, 6 and 11) may cause anogenital warts or mild forms of intraepithelial neoplasia affecting the vulva, vagina, cervix, anus, or penis. Persistent infection with 1 of the 13 oncogenic (high-risk) types may lead to precancerous or cancerous lesions. In the United States, types 16 and 18 are responsible for 63% of HPV-associated cancers (70% of cervical cancers); types 31, 33, 45, 52 and 58 cause an additional 10%. In addition to cervical carcinoma, HPV is associated with other malignancies. In 1 study, HPV DNA was found in 90% of cervical, 75% of vaginal, 69% of vulvar, 63% of penile, and 91% of anal cancers. Approximately 70% of oropharyngeal cancers are believed to result from HPV infection, especially type 16.\n\nCurrently, 2 HPV vaccines are licensed and available in the United States: 4-valent (types 6, 11, 16, 18) and 9-valent (types 6, 11, 16, 18, 31, 33, 45, 52, 58). Each of the vaccines is approved for the prevention of HPV-associated cervical, vaginal, vulvar, and anal cancers, and precancerous lesions. The additional coverage provided by the 9-valent vaccine could increase protection against invasive cervical cancer from 70% to 90%.\n\nThe 4- and 9-valent vaccines are approved for the prevention of anogenital warts, 90% of which are caused by types 6 and 11. Both vaccines are approved for use in both girls and boys.\n\nThe Advisory Committee on Immunization Practices offers the following recommendations regarding HPV immunization:\n• Vaccination of girls and boys should begin routinely at 11 or 12 years, but may be initiated as early as 9 years.\n  o The vaccine is also recommended for females of ages 13 to 26 years and males of ages 13 to 21 years who were not previously immunized.\n  o For men who have sex with men and for persons who are immunocompromised, vaccination is recommended through age 26 years.\n• The vaccines are administered with 1 of 2 schedules:\n  o For those 9 to 14 years of age, a 2-dose schedule is recommended: the second dose is given 6 or 12 months after the first (if the series is initiated before the 15th birthday, 2 doses are administered)\n  o For those 15 to 26 years of age, a 3-dose schedule is recommended: the second dose is administered 1 to 2 months after the first; the third dose is given at least 6 months after the first.\n• If the vaccine series is interrupted it may be resumed (ie, the series does not need to be restarted).\n• If possible, the same vaccine form should be used to complete the immunization series. However, if this is not possible, another vaccine may be substituted.\n\nThe most common adverse effects of HPV vaccination are injection site pain, erythema, and/or edema. Headache, dizziness, fever, nausea, fatigue, or syncope may occur. The vaccine is not associated with an increased risk of developing central nervous system demyelinating disease. Although it is a concern expressed by some parents, studies indicate that girls who receive the vaccine are not more likely than those who do not to be sexually active or to have an increased number of sexual partners.\n\nIn the United States, only 39.7% of girls and 21.6% of boys aged 13 to 17 years receive 3 or more doses of HPV vaccine (ie, complete the series). The rate of immunization with 1 or more doses of HPV has increased, but remains lower than for 1 or more doses of tetanus, diphtheria, and pertussis (Tdap) or meningococcal bacteria A, C, W and Y (MenACWY). Although the reasons for this are not fully understood, the lack of a strong provider recommendation is 1 factor. In 1 study, 55% of parents who received a physician's recommendation for HPV immunization had their sons vaccinated compared with only 1% of parents who received no such recommendation. Similarly, in a study published in 2014, the most common reason parents did not vaccinate their daughters against HPV was the lack of a physician recommendation. Resources that may assist clinicians in communicating the importance of HPV vaccine to parents and patients are available at: http://www.cdc.gov/hpv/index.html.\n\nPREP Pearls\n• The 9-valent HPV vaccine may protect against HPV types that cause 90% of anogenital warts and 90% of invasive cervical carcinomas.\n• A strong provider recommendation is an important factor in a parent's decision to allow the administration of the HPV vaccine to their child.\n\nABP Content Specifications(s)\n• Recognize the clinical features associated with human papillomavirus infection\n• Understand the epidemiology of human papillomavirus infection\n• Know the recommendations, limitations, and schedule for the human papillomavirus vaccine\n\nSuggested Readings\n• Ben-Harush Negari S, Kahn JA. Human papillomavirus infection and anogenital warts. In Neinstein LS, Katzman DK, Callahan ST, Gordon CM, Joffe A, Rickert VI, eds. Neinstein's Adolescent and Young Adult Medicine. 6th ed. Philadelphia, PA: Wolters Kluwer; 2016.\n• Petrosky E, Bocchini JA Jr, Hairi S, et al. Use of 9-valent human papillomavirus (HPV) vaccine: updated HPV vaccination recommendations of the Advisory Committee on Immunization Practices. MMWR Morb Mortal Wkly Rep. 2015;64:300–304.\n• Reagan-Steiner S, Yankey D, Jeyarajah J, et al. National, regional, state, and selected local area vaccination coverage among adolescents aged 13-17 years – United States, 2015. MMWR Morb Mortal Wkly Rep. 2016;65:850–858.\n• Viens LJ, Henley SJ, Watson M, et al. Human papillomavirus-associated cancers – United States, 2008-2012. MMWR Morb Mortal Wkly Rep. 2016;65:661–666."}
{"id" : 2735, "question_text" : "A 17-year-old, previously healthy adolescent boy is brought to the emergency department with lower extremity weakness. He was walking into his house after checking the mailbox when he experienced the onset of bilateral lower extremity weakness, which he describes as a heaviness. His weakness progressively worsened over several hours to the point that he could not stand up from the dinner table. The boy recalls a self-limited episode of blurred vision 6 months ago, but no other episodes of focal neurological deficits. On physical examination, the adolescent's vital signs are normal. His mental status and cranial nerve examination findings are normal. Motor examination reveals normal upper extremity strength, tone, and reflexes; lower extremities are flaccid with 0/5 strength, absent reflexes, and hypotonia. His toes are upgoing to plantar stimulation. He has a sensory level at his mid-thorax, below which he is unable to reliably feel light touch, pin prick, temperature, or vibration. He has normal finger to nose testing but cannot perform heel-to-shin testing due to weakness. He is unable to ambulate. The remainder of his physical examination findings are normal. Of the following, the BEST next step in this adolescent's management is", "options" : "[\"electromyography and nerve conduction studies\", \"lumbar puncture with cerebrospinal fluid analysis\", \"spine computed tomography\", \"spine magnetic resonance imaging\"]", "explanation" : "Correct Answer: D\nThe adolescent in the vignette is experiencing an acute myelopathy, a neurological emergency requiring urgent neuroimaging to evaluate for spinal cord compression or inflammation. Magnetic resonance imaging (MRI) of the spine with and without contrast is the best test to evaluate the spinal cord and associated structures. The presence of a compressive lesion requires neurosurgical intervention.\n\nCauses of acute and subacute myelopathies are shown in Item C228.\n\nRecognition of acute spinal cord dysfunction is critical to timely diagnosis and treatment.\n\nCommon symptoms and signs of an acute myelopathy include:\n• Bilateral motor and/or sensory symptoms\n• Bladder and/or bowel dysfunction\n• Neck and back pain\n\nIn the acute phase, children and adolescents with myelopathy can present with a flaccid paralysis and areflexia below the level of the lesion, leading to diagnostic confusion due to the absence of upper motor neuron signs. Careful history and physical examination can aid in determining the appropriate evaluation to distinguish a peripheral nervous system condition, such as Guillain-Barré syndrome, from myelopathy, and guide the management plan.\n\nIn the setting of acute-subacute myelopathy, MRI of the spine is the most precise diagnostic test. Computed tomography of the spine is helpful to look at bony structures but does not provide sufficient information about the cord and soft tissues. Lumbar puncture is a useful ancillary test, in particular in inflammatory disorders, and is often part of a tiered evaluation; however, it should not be prioritized over imaging in a patient with acute spinal cord dysfunction. Electromyography and nerve conduction studies can be useful in prognostication and monitoring progression of peripheral nervous system disorder. They provide ancillary data in the subacute phase but are often normal in the acute phase.\n\nManagement of myelopathy is aimed at the underlying cause. In early inflammatory myelitis, MRI of the spine can be normal; empiric treatment should be initiated if there is a high index of suspicion and alternative etiologies have been excluded. To prevent complications, monitoring and supportive care should include:\n• Frequent bladder scans with intermittent catheterization\n• Monitoring for the development of autonomic dysfunction\n• Prevention of deep venous thrombosis\n• Prevention of decubitus ulcer formation Psychological support\n\nPREP Pearls\n• Acute spinal cord dysfunction is a neurological emergency requiring careful history, physical examination, and urgent neuroimaging to evaluate for cord compression. Magnetic resonance imaging of the spine with and without contrast is the best test to evaluate the spinal cord and associated structures.\n• Common symptoms and signs of an acute myelopathy include: bilateral motor and/or sensory symptoms, bladder and/or bowel dysfunction, and neck/back pain.\n• In the acute phase, children and adolescents with myelopathy can present with a flaccid paralysis and areflexia below the level of the lesion; this may lead to diagnostic confusion due to the absence of upper motor neuron signs.\n\nABP Content Specifications(s)\n• Plan the initial neurodiagnostic evaluation of acute spinal cord dysfunction\n\nSuggested Readings\n• Absoud M, Greenberg BM, Lim M, Lotze T, Thomas T, Deiva K. Pediatric transverse myelitis. Neurology. 2016;87(9 suppl 2):S46-S52. doi:10.1212/WNL.0000000000002820.\n• Huh Y, Park EJ, Jung JW, Oh S, Choi SC. Clinical insights for early detection of acute transverse myelitis in the emergency department. Clin Exp Emerg Med. 2015;2(1):44-50. doi:10.15441/ceem.14.034.\n• Lu V, Niazi T. Pediatric spinal cord diseases. Pediatr Rev. 2021 42(9):486-499. doi:10.1542/pir.2020-000661."}
{"id" : 775, "question_text" : "While examining a 2-year-old for a routine health supervision visit at least 18 hyperpigmented macules greater than 0.5 cm in diameter are noted on her trunk and extremities. No axillary or inguinal freckles are noted and no subcutaneous or cutaneous lesions suggestive of neurofibromas are observed. Findings on examination by a pediatric ophthalmologist are normal, with good visual acuity in both eyes and no Lisch nodules noted. The history is negative for anyone else in the family with cafe au lait spots or other signs of neurofibromatosis. Of the following, you are MOST likely to tell her parents that", "options" : "[\"she has neurofibromatosis because she has more than 6 cafe au lait spots\", \"she possibly has neurofibromatosis because she is only 2 years old and other findings are likely to appear over time\", \"she probably does not have neurofibromatosis because the family history is negative for neurofibromatosis\", \"she probably does not have neurofibromatosis because she has no cutaneous findings other than the spots\", \"she probably has neurofibromatosis because she has 18 spots, which is significantly greater than the minimum number of 6\"]", "explanation" : "The young girl described in the vignette has 18 or more cafe au lait spots (CLSs) greater than 0.5 cm in diameter. While this is a significant finding suggestive of neurofibromatosis type 1 (NFI), this diagnosis cannot be confirmed without a second clinical finding. Because she is only 2 years old, if she has NF1, there is a good chance that additional clinical features will appear over time. For example, axillary and inguinal freckles may appear for the first time in older children and neurofibromas may not appear until preadolescence through adulthood. However, there are individuals with many CLSs who never develop a second sign of NF1 and are eventually cleared after appropriate investigations. While 6 is the minimum number to count as a clue for the diagnosis of NF1, greater than 6 CLSs does not increase the likelihood of an eventual diagnosis. A second sign, including: axillary or inguinal freckles (Item C68A, page C-55), 1 plexiform or 2 simple neurofibromas, an optic nerve glioma, Lisch nodules (Item C68B, page C-55), sphenoid bone dysplasia, a bony pseudarthrosis, or a first-degree relative with NF 1; must be present for a diagnosis of NF I to be made in any patient who has 6 or more CLSs. A family history that is positive for NF1 is quite helpful, especially in confirming a diagnosis in an infant or young child (eg, the girl in the vignette) who has only CLSs. However, approximately 50% of individuals with NF1 are the first person in their family with this condition. In such cases, the gene mutation occurs as a result of a de novo event at the time of conception. Lisch nodules (ie, benign iris freckles) are almost never present in young children but are seen in most patients who have NF1 after 10 years of age.\n\nChildren, such as this girl, must be monitored closely for other signs of NF1, including axillary or inguinal freckles, optic nerve gliomas, and bony malformations such as thinning or curving of a long bone (eg, the tibia). Because clinically significant optic nerve gliomas most often present before age 5 years, a careful eye examination by an experienced pediatric ophthalmologist may be critical to assess the optic nerves and visual acuity. Regular eye examinations may permit earlier identification of optic nerve gliomas in young children who cannot adequately describe visual difficulties, thereby allowing for prompt treatment and improved visual outcomes. Macrocephaly is also quite common in infants, children, and adults who have NF1 but may not necessitate head imaging studies unless measurements are sequentially crossing percentiles or are associated with neurologic symptoms. Younger children should also be assessed for the need for services, such as speech, occupational, or physical therapies. Children and teenagers who have NF1 must be monitored closely for developmental problems because of an increased risk for learning disabilities and attention difficulties. Children and teenagers must also be monitored closely for scoliosis, since the incidence in this population is quite high and scoliosis may be rapidly progressive in patients who have NF1.\n\nMore recently, molecular diagnostic testing has proven helpful in confirming a diagnosis of NF1 in patients with only a single finding and for family planning purposes. Neurofibromin gene sequencing, however, is not generally available through large commercial laboratories and must be performed at one of several genetic specialty laboratories. As opposed to older methodologies, gene sequencing has a mutation detection rate of about 95%. However, a negative molecular genetic test does not exclude a diagnosis of NFI. Patients should still be monitored for additional features of NF1. If by the age of 10 years an individual has no eye findings (including Lisch nodules), axillary or inguinal freckles, neurofibromas, or other characteristic features, the diagnosis of NF 1 is unlikely.\n\nPREP Pearls\n• In order to confirm a diagnosis of NF1, at least 2 of the following clinical features must be present: axillary or inguinal freckles, 6 or more cafe au lait spots, neurofibromas, an optic nerve glioma, Lisch nodules, specific bony lesions, or a first-degree relative with NF1.\n• Molecular testing can be helpful in confirming a diagnosis of NF1 in some cases and may be useful for family planning purposes.\n\nAmerican Board of Pediatrics Content Specification(s):\n• Know the clinical features of neurofibromatosis\n\nSuggested Reading:\n• Hersh JH. Health supervision for children with neurofibromatosis. Pediatrics. 2008;121(3):633-642. doi:10.1542/peds.2007-3364"}
{"id" : 2671, "question_text" : "A 7-year-old girl is brought to the clinic for concerns about her balance. For the past few days, the girl has complained of the room spinning and nausea. She has 5 to 6 episodes per day, each lasting about 1 minute, during which she grabs onto a chair or the sofa. Between episodes she acts and feels normal. She is otherwise healthy with no history of trauma or recent viral symptoms. Her physical examination findings are unremarkable, including her tympanic membranes and a detailed neurologic examination. Of the following, the BEST next step in this girl's management is", "options" : "[\"an antihistamine prescription\", \"head and neck imaging\", \"observation\", \"referral to neurology\"]", "explanation" : "Correct Answer: C\nThe girl in the vignette most likely has benign paroxysmal vertigo of childhood (BPVC), given her intermittent symptoms and unremarkable physical examination findings. Benign paroxysmal vertigo of childhood is typically a self-limited condition for which observation is recommended. Diagnostic evaluation and referral to neurology are only recommended if symptoms worsen or there is impairment of function. Medication management with antiemetics or antihistamines may be warranted if episodes are long lasting, which is not yet the case for the girl in the vignette. Although the cause of BPVC is unknown, there is usually a family history of migraine headaches, and children with BPVC may subsequently develop migraines.\n\nYoung children may have difficulty describing vertigo, defined as a sensation of rotational movement that usually involves the vestibular system, either peripheral (inner ear) or central (brainstem, cerebellum, or cranial nerve VIII). History and physical examination findings provide etiologic clues. Middle ear disease is the most common cause of vertigo in children. Benign paroxysmal vertigo of childhood is the second most common cause of vertigo in children aged 2 to 12 years. The diagnosis of BPVC is clinical and based the following criteria:\n• Brief episodes of vertigo associated with vomiting, pallor, nystagmus, ataxia, or fearfulness\n• Normal neurologic examination, audiometric, and vestibular findings between episodes\n• Symptom-free intervals\n• Not attributable to another disorder\n\nBenign paroxysmal vertigo of childhood may be considered a migraine equivalent. The prognosis is good, usually with spontaneous resolution before adolescence.\nBenign paroxysmal vertigo of childhood is a distinct entity from benign paroxysmal positional vertigo (BPPV), which is associated with position change, can be elicited by the Dix-Hallpike maneuver (neck is extended and turned to one side followed by quick placement in the supine position), and is caused by calcium deposits in the posterior semicircular canal. Benign paroxysmal positional vertigo is rare in children. Vestibular migraines may also cause vertigo.\n\nPREP Pearls\n• Benign paroxysmal vertigo of childhood is the second most common cause of vertigo in children aged 2 to 12 years; the most common cause is middle ear disease.\n• The diagnosis of benign paroxysmal vertigo is based on a history of brief episodes of vertigo associated with vomiting, pallor, nystagmus, ataxia or fearfulness, normal neurologic examination findings, audiometric, and vestibular findings between episodes, symptom-free intervals, and no other attributable disorder.\n• Benign paroxysmal vertigo is a self-limited condition for which the treatment is observation. Referral to neurology for further evaluation and management may be considered for worsening symptoms or impairment of function.\n\nABP Content Specifications(s)\n• Recognize the clinical findings associated with benign paroxysmal vertigo\n\nSuggested Readings\n• Batson G. Benign paroxysmal vertigo of childhood: a review of the literature. Paediatr Child Health. 2004;9(1):31-34. doi:10.1093/pch/9.1.31.\n• Benun J. Balance and vertigo in children. Pediatr Rev. 2011;32(2):84-85. doi:10.1542/pir.32.2.84.\n• Rivera RF, Sellinger CR. Dizziness and vertigo. In: McInerny TK, Adam HM, Campbell DE, DeWitt TG, Foy JM, Kamat DM, eds. American Academy of Pediatrics Textbook of Pediatric Care. American Academy of Pediatrics; 2021:chap 140. Accessed September 1, 2022. Pediatric Care Online.\n• van de Berg R, Widdershoven J, Bisdorff A, et al. Vestibular migraine of childhood and recurrent vertigo of childhood: diagnostic criteria consensus document of the Committee for the Classification of Vestibular Disorders of the Bárány Society and the International Headache Society. J Vestib Res. 2021;31(1):1-9. doi:10.3233/VES-200003."}
{"id" : 3718, "question_text" : "A 35-week-gestation neonate with tachypnea is being evaluated 2 hours after birth. Her mother has a history of asthma. Maternal prenatal serologic test results were negative. Group B Streptococcus status is unknown. The neonate was born via cesarean section with meconium-stained amniotic fluid noted at delivery. She cried immediately and received routine initial steps of care. Apgar scores were 6 and 8 at 1 and 5 minutes, respectively. Vital signs include a heart rate of 186 beats/min, respiratory rate of 67 breaths/min, blood pressure of 76/45 mm Hg, temperature of 37.6°C, and room air oxygen saturation of 87% on the left foot. Physical examination reveals nasal flaring with intermittent grunting, no cardiac murmur, +1 peripheral pulses throughout, and no hepatosplenomegaly. Her chest radiograph is shown (Item Q164). Of the following, this neonate's MOST likely diagnosis is", "options" : "[\"meconium aspiration syndrome\", \"pneumonia\", \"pneumothorax\", \"respiratory distress syndrome\"]", "explanation" : "The chest radiograph of the neonate in the vignette, in combination with meconium-stained amniotic fluid and respiratory distress, is most consistent with a diagnosis of meconium aspiration syndrome (MAS). Meconium aspiration syndrome affects 0.4 to 1.6 neonates per 1,000 live births, with higher rates noted among late term (41 to 41 6/7 weeks') and post-term (42 to 42 6/7 weeks') gestation neonates. Risk factors for MAS include cesarean delivery and fetal compromise. It may be associated with neonatal respiratory failure and pulmonary hypertension. Exposure to meconium in utero may cause remodeling of vascular endothelium, increasing vascular resistance, resulting in severe pulmonary hypertension. After delivery, meconium acts via a ball valve mechanism, obstructing air flow and causing patchy areas of atelectasis and overdistention which can be seen on chest radiography. The incidence of MAS has decreased over the past 20 years, likely because of changes in obstetric management. Because of changes in the neonatal resuscitation program in 2015, vigorous neonates born through meconium-stained amniotic fluid no longer undergo elective intubation. This change in practice may be associated with an increase in admissions to the neonatal intensive care unit. Group B streptococcal (GBS) pneumonia typically has a similar clinical presentation to respiratory distress syndrome (RDS) and MAS. Neonates exhibit tachypnea, grunting, flaring, and poor lung expansion. Maternal status may be GBS negative or positive with inadequate prophylaxis before delivery. Chest radiograph (Item C164) has a diffuse reticulogranular appearance. Neonates with GBS pneumonia should receive intravenous antibiotic therapy with ampicillin plus an aminoglycoside. A pneumothorax may occur as part of an air leak syndrome. Neonates requiring positive pressure ventilation after delivery have an increased risk of pneumothorax. In addition, both RDS and MAS are associated with an increased risk of pneumothorax. A large pneumothorax may compress the heart, requiring evacuation with a needle thoracostomy. Less often, a thoracotomy may be required. Respiratory distress syndrome is a disease of premature neonates caused by inadequate surfactant production. The severity of RDS is proportional to the degree of prematurity. Affected neonates present with tachypnea, grunting, flaring, and poor lung expansion. Chest radiography shows decreased lung volumes and a homogenous ground glass appearance, identical to that seen in GBS. Surfactant replacement therapy may be required in neonates for severe hypoxic respiratory failure. Respiratory distress syndrome typically resolves within 48 to 72 hours after birth with endogenous surfactant production. PREP Pearls • Meconium aspiration syndrome in a neonate is characterized by respiratory distress, meconium-stained amniotic fluid, and chest radiography with hyperexpansion and patchy opacities. • Meconium aspiration syndrome may be associated with pulmonary hypertension and respiratory failure. • Pneumothorax is more common among neonates who require positive pressure ventilation. ABP Content Specifications(s) • Recognize the characteristic clinical and radiographic appearance of pneumothorax in a newborn infant, and manage appropriately • Recognize the characteristic clinical and radiographic appearance of meconium aspiration syndrome in a newborn infant, and manage appropriately • Identify the signs and symptoms of transient tachypnea of the newborn, and manage appropriately Suggested Readings • Carbine DN. Meconium aspiration. Pediatr Rev. 2008;29:212. doi:10.1542/pir.29-6-212. • Chiruvolu A, Miklis KK, Chen E, Petrey B, Desai S. Delivery room management of meconium-stained newborns and respiratory support. Pediatrics. 2018;142(6):e20181485. doi:10.1542/peds.2018-1485. • Dargaville PA, Copnell B. The epidemiology of meconium aspiration syndrome: incidence, risk factors,therapies, and outcome. Pediatrics. 2006;117:1712. doi:10.1542/peds.2005-2215. • Nafday SM, Long C. Respiratory distress and breathing disorders in the newborn. In: McInerny TK, Adam HM, Campbell DE, DeWitt TG, Foy JM, Kamat DM, eds. American Academy of Pediatrics Textbook of Pediatric Care. 2nd ed. Elk Grove Village, IL: American Academy of Pediatrics; 2017:867-888. Pediatric Care Online."}
{"id" : 2128, "question_text" : "A previously healthy 2-year-old is brought to the emergency department after his mother had difficulty waking him. At his home, emergency medical services personnel measured the child's glucose at 125 mg/dL (6.94 mmol/L). In the emergency department, the child has a temperature of 37 C, his heart rate is 115 beats/min, his respiratory rate is 42 breaths/min, his blood pressure is 100/74 mm Hg, and his oxygen saturation is 100% in room air. The child is sleepy but arouses to touch and responds to questions with a few words. He moves all of his extremities symmetrically in response to pain. Both pupils are 6 mm in size and sluggishly reactive. Physical examination findings are otherwise unremarkable. Laboratory findings are shown: Glucose 94 mg/dL (5.22 mmol/L), Venous blood gas pH 7.01, PaO2 40 mm Hg, PaCO2 20 mm Hg, Bicarbonate 6 mEq/L (6 mmol/L), Oxygen saturation 100%, Sodium 136 mEq/L (136 mmol/L), Potassium 3.4 mEq/L (3.4 mmol/L), Chloride 103 mEq/L (103 mmol/L), Ionized calcium 5.0 mg/dL (1.25 mmol/L), Lactate 27.03 mg/dL (3 mmol/L). Of the following, the BEST test to confirm this child's most likely diagnosis is a serum", "options" : "[\"aspirin concentration\", \"\\u03b2-hydroxybutyrate concentration\", \"methanol concentration\", \"osmolar gap\"]", "explanation" : "Critique\nThe child in the vignette has vomiting and an acute change in mental status that developed shortly after he was playing in a garage. He is also experiencing vision changes. His clinical and laboratory findings are consistent with a diagnosis of methanol ingestion. A serum methanol level is the most specific test to confirm this diagnosis.\nThe differential diagnosis for altered mental status in children is broad. Important causes to consider include toxic ingestion, intracranial pathology (eg, hemorrhage, trauma, mass, infection, stroke), metabolic derangement, seizure activity, and systemic infection. A number of diagnostic studies may be necessary to identify the cause of the altered mental status. A blood glucose concentration and blood gas panel with electrolytes should be obtained urgently; these can provide rapid information to guide further evaluation and management. \nThe blood gas results for the child in the vignette demonstrate severe metabolic acidosis with partial compensatory respiratory alkalosis. The results of a serum electrolyte panel will differentiate whether this is a normal or an increased anion gap metabolic acidosis. Causes of normal anion gap acidosis are relatively few; these typically occur in children with ongoing health concerns (eg, renal tubular acidoses, profound diarrhea, ongoing hyperalimentation, gastrointestinal fistulae). For increased anion gap metabolic acidosis, the mnemonic \"MUDPILES\" lists the differential diagnosis: \nMethanol/metformin ingestion \nUremia\nDiabetic ketoacidosis\nParaldehyde ingestion\nIsoniazid or iron ingestion\nLactic acidosis (including carbon monoxide or cyanide) \nEthylene glycol ingestion \nSalicylate ingestion \nEvaluation of children with increased anion gap metabolic acidosis should focus on differentiating between these potential underlying causes.\nThe rapid onset of symptoms in this child makes a toxic ingestion likely. For suspected ingestions, focused questions regarding activities before symptom onset and access to medications and other toxins should be included in the history. Given that this child was playing in a garage shortly before becoming ill, there is a high likelihood of access to substances containing toxic alcohols, such as windshield wiper fluid (methanol) and antifreeze (ethylene glycol and methanol). \nInitial symptoms most commonly arising from ingestion of toxic alcohols include mental status changes, sleepiness, and vomiting. Methanol converts in the body to formaldehyde and then to formic acid, leading to profound metabolic acidosis. Vision changes can result from formic acid's acting on the optic nerve and retina, with the potential for permanent vision loss. Treatment of methanol toxicity includes airway and breathing support, administration of intravenous fluids, an intravenous bicarbonate drip (to counteract the metabolic acidosis), and administration of intravenous fomepizole (an antidote that blocks methanol's conversion to its toxic metabolites). Hemodialysis is required if signs of end-organ damage are present and/or improvement in acidosis is not observed with initial treatment steps. Ethylene glycol is an alcohol also found in automotive fluids, as the sweet-tasting ingredient of antifreeze. Similar to methanol, ingestion of ethylene glycol presents as confusion, sleepiness, and vomiting. It causes an anion gap metabolic acidosis through its stepwise conversion to glycolic acid. The downstream product of oxalic acid crystals leads to renal injury (with increasing blood urea nitrogen) and hypocalcemia through binding of serum calcium. The normal calcium concentration in this vignette makes an ethylene glycol ingestion less likely than a methanol ingestion.\nThe definitive diagnosis of acute methanol toxicity is made by measuring a serum methanol concentration. The results of this test are not rapidly available in many practice settings; therefore, empiric treatment for methanol poisoning should be initiated if there is strong suspicion of this condition. \nIngestion of methanol can result in an elevated osmolar gap (osmoles that are not accounted for by serum sodium, glucose, and urea), calculated as follows: \nosmolar gap = 2[Na+] + glucose (mg/dL)/18 + blood urea nitrogen (mg/dL)/2.8 \nAn osmolar gap >10 mOsm/kg indicates the presence of substances that may include toxic alcohols (eg, ethylene glycol, methanol, propylene glycol), sugars (eg, mannitol, sorbitol), hyperlipidemia, and/or hyperproteinemia. The serum osmolar gap may be transient, increasing soon after ingestion of toxic alcohols and declining over the course of several hours after ingestion as the alcohol is converted to the toxic metabolite(s). Some alcohol metabolites cause metabolic acidosis that worsens over time while the osmolar gap decreases. For this reason, a serum osmolar gap may be normal in individuals with significant toxicity resulting from ingestion of toxic alcohols. \nAlthough salicylate toxicity caused by aspirin ingestion could cause the altered mental status, tachypnea, and elevated anion gap metabolic acidosis in the vignette, vision changes are not commonly associated with salicylate poisoning. Furthermore, children with salicylate toxicity complain of ringing in the ears (tinnitus), which this child does not have. However, it is still important to obtain salicylate and acetaminophen concentrations for any child with altered mental status resulting from a suspected ingestion. \nAn elevated serum β-hydroxybutyrate concentration would support the diagnosis of diabetic ketoacidosis (DKA). Although DKA can cause altered mental status, tachypnea, and an elevated anion gap metabolic acidosis in children, this child's normal glucose concentration and acute onset of symptoms make DKA unlikely.\n\nSuggested Reading(s)\nBarceloux DG, Bond GR, Krenzelok EP, Cooper H, Vale JA, et al; American Academy of Clinical Toxicology Ad Hoc Committee on the Treatment Guidelines for Methanol Poisoning. American Academy of Clinical Toxicology practice guidelines on the treatment of methanol poisoning. J Toxicol Clin Toxicol. 2002;40(4):415-46. doi:10.1081/clt-120006745\nDay R, Eddleston M, Thomas SH, Thompson JP, Bradberry SM, Vale JA. Toxicity from automotive screenwashes reported to the United Kingdom National Poisons Information Service (NPIS) from 2012 to 2015. Clin Toxicol (Phila). 2017;55(3):221-226. doi:10.1080/15563650.2016.1271130\nVohra V, Lelak K, Neuman MI, et al. Pediatric hand sanitizer exposures reported to United States poison centers, 2017-2021. Clin Toxicol (Phila). 2023;61(6):463-469. doi:10.1080/15563650.2023.2221816\n\nContent Domain\nEmergency Medicine, Ingestions\n\nLearning Objectives\nEvaluate and treat a patient with a methanol ingestion\n\nThe correct answer is: methanol concentration"}
{"id" : 2224, "question_text" : "A 6-month-old exclusively breastfed infant is undergoing a health supervision visit. His mother has allergies to milk, soy, and seafood and has restricted these foods from her diet for many years. She does not take any medications or supplements. Because the mother is concerned that the infant may also develop food allergies, she has not yet introduced any complementary foods. She is giving the infant 400 IU of vitamin D daily. Of the following, the infant is MOST at risk for experiencing a deficiency of", "options" : "[\"calcium\", \"iodine\", \"iron\", \"zinc\"]", "explanation" : "Infants that are exclusively breastfed are at risk for experiencing iodine deficiency when the breastfeeding parent has dietary restrictions that limit intake of iodine-rich foods (eg, milk, soy, and seafood)\n\nThe American Academy of Pediatrics recommends that all breastfeeding parents take a supplemental vitamin that includes 150 µg of iodine.\n\nThe levels of calcium, iron, and zinc in breast milk are not significantly affected by the breastfeeding parent's dietary intake.\n\nCritique\nOf the response choices, the infant in the vignette is at greatest risk for experiencing iodine deficiency. Because of his mother's allergies to milk, soy, and seafood (3 major sources of dietary iodine), she likely had a deficiency of iodine during pregnancy. This deficiency will also affect the concentration of iodine in her breast milk. Iodine is an essential component of the thyroid hormones triiodothyronine and thyroxine, which are critical for skeletal and central nervous system development in fetuses and infants. Infants that are exclusively breastfed rely entirely on parental iodine intake for optimal neurodevelopment. Chronic iodine deficiency can have a negative impact on long-term cognitive development. The American Academy of Pediatrics and the American Thyroid Association recommend that all breastfeeding parents take a vitamin supplement with 150 µg of iodine daily. Testing to assess for iodine deficiency is difficult; urine testing analyzes an individual's present state of iodine sufficiency according to recent intake, but it is not helpful in understanding chronic iodine deficits. Urine testing is more useful for assessing iodine sufficiency across a population.\n\nHuman milk is a good source of other micronutrients, including calcium, iron, and zinc. The amounts of these micronutrients in human milk are less affected by maternal dietary intake than is the amount of iodine. Maternal calcium intake has a minimal effect on the calcium concentrations in breast milk. Although the total calcium level in human milk will decrease over time, the ionized calcium concentration remains stable throughout lactation. Infants accumulate hepatic iron stores during the final trimester of gestation. After 6 months of age, infants should receive complementary foods that are good sources of iron (eg, iron-enriched cereals). Parental iron supplementation does not affect iron availability in breast milk. Zinc is a key component of many biochemical pathways and is highly bioavailable in breast milk, owing to the presence of a zinc-binding ligand.\n\nSuggested Reading(s)\nCosme-Blanco W, Arroyo-Flores E, Ale H. Food allergies. Pediatr Rev. 2020;41(8):403-415. doi:10.1542/pir.2019-0037\nDror DK, Allen LH. Overview of nutrients in human milk. Adv Nutr. 2018;9(suppl 1):278S-294S. doi:10.1093/advances/nmy022\nWright K, Feeney M, Yerlett N, Meyer R. Nutritional management of children with food allergies. Curr Treat Options Allergy. 2022;9:375-393. doi:10.1007/s40521-022-00320-7\n\nContent Domain\nNutrition, Deficiencies\n\nLearning Objectives\nRecognize how a restricted diet may impact the nutrition of a breastfed infant"}
{"id" : 1110, "question_text" : "An 8-month-old infant with asplenia presents to the emergency department for evaluation of irritability, fever, and rash. A physical examination reveals multiple purpuric lesions. Lumbar puncture reveals a cerebrospinal fluid white blood cell count of 1,800/µL. He is treated with vancomycin and cefotaxime, and admitted to the pediatric intensive care unit. His mother asks if anything could have been done to prevent this illness. Of the following, the BEST response to the mother's question is that meningococcal conjugate vaccine is", "options" : "[\"indicated for travelers starting at 6 months of age\", \"indicated for patients with human immunodeficiency virus infection\", \"indicated starting at 2 months of age\", \"licensed for children only older than 9 months of age\", \"protective against the most common serogroup in infants\"]", "explanation" : "The infant in the vignette with asplenia should have been vaccinated with meningococcal conjugate vaccine beginning at 2 months of age. Groups considered to have increased risk include those with anatomic or functional asplenia, such as the infant in this vignette, or those with complement component deficiencies. Of note, HIV infection is not an indication for infant immunization.\nIn the United States, there are 2 meningococcal quadrivalent polysaccharide protein conjugate vaccines licensed for young children that offer protection against serogroups A, C, W, and Y. An additional bivalent conjugate vaccine combined with Haemophilus influenzae vaccine protects against serogroups C and Y. Since 2005, vaccination has been routinely recommended in adolescents. In 2010, the recommendation for a booster dose at 16 years of age was made. Although 60% of meningococcal disease occurs in children younger than 5 years of age, the two licensed novel meningococcal serogroup B-specific vaccines available in the US are approved for use in persons 10 to 25 years of age. Serogroup B-specific vaccines have been used successfully in outbreak settings.\nMeningococcal vaccination is recommended for travelers to endemic regions, such as the \"meningitis belt\" in sub-Saharan Africa or during the Hajj in Saudi Arabia. For children younger than 9 months of age who are travelling to endemic areas, a 3-dose primary series of conjugate vaccine at 2, 4, and 6 months of age should be completed prior to travel. Children 9 months to 23 months of age require 2 doses and those 24 months or older require a single dose.\nIn the United States, one of the commercially available quadrivalent vaccines is licensed for persons as young as 2 months of age and the bivalent vaccine that is combined with Haemophilus influenzae is licensed for infants starting at 6 weeks of age.\nPREP Pearls\n High risk groups should be vaccinated with meningococcal vaccine starting at 2 months of age.\n Commercially available serogroup B-specific vaccines are licensed for persons 10 to 25 years of age\n Serogroup B is responsible for most infections in young children.\n For travel or residence in a hyperendemic region, meningococcal vaccination can begin at 2 months of age.\nABP Content Specifications(s)\n Know the indications and schedule for the meningococcal vaccine\n Know which serotypes are included in the meningococcal vaccine"}
{"id" : 2823, "question_text" : "A male neonate, born at 34 weeks' gestation, is being evaluated for respiratory distress shortly after delivery. The mother reports no medical problems during the pregnancy; prenatal care records are not available. Maternal group B Streptococcus status is unknown. The mother was febrile, with a maximum temperature of 39°C, for 6 hours before delivery. The neonate was delivered vaginally through green amniotic fluid. On physical examination, he is noted to have grunting and tachypnea. The remainder of his examination findings are unremarkable. He is started on continuous positive airway pressure +5 cm H2O and 30% oxygen in the delivery room. Chest radiography shows bilateral streaky infiltrates. Of the following, the pathogen MOST likely to be responsible for this neonate's findings is", "options" : "[\"Chlamydia trachomatis\", \"Escherichia coli\", \"Listeria monocytogenes\", \"Streptococcus agalactiae\"]", "explanation" : "For the late preterm neonate in the vignette, the most likely pathogen is Listeria monocytogenes. Listeria is an uncommon cause of neonatal infection, with mortality ranging from 14% to 56%. Because immunity against Listeria is dependent on T-cell–mediated immunity and granuloma formation, pregnant women and other patients with immunosuppression are at increased risk of infection.\n\nTypically, pregnant women acquire Listeria via contaminated food such as ready-to-eat deli meat, unpasteurized milk, soft cheese, ice cream, or frozen fruits and vegetables. Affected pregnant women may report a flu-like illness with fever before delivery. Up to 22% of affected pregnancies will result in a stillbirth.\n\nNeonatal Listeria may present as early-onset disease in the first week after birth or as late-onset disease. In early-onset disease, following maternal bacteremia, Listeria crosses the placenta into the fetal circulation. More than 70% of women with Listeria deliver before 35 weeks' gestation. Among premature infants, meconium-stained amniotic fluid is uncommon. However, green or brown-stained amniotic fluid is often noted with Listeria infection. Affected neonates may present with respiratory distress because of pneumonia; chest radiographic findings show bilateral streaky infiltrates or a miliary pattern. With severe infection, neonates may have an erythematous rash with small, pale papules called granulomatosis infantisepticum, which is caused by disseminated granulomas. Pustular lesions may also be noted on the skin and pharynx.\n\nIn late-onset Listeria infection, neonates are more often of term gestation. Two weeks after birth, these neonates develop nonspecific symptoms such as poor feeding. Transmission is thought to be perinatal or nosocomial. Transmission via breast milk has also been reported. Meningitis is more common among neonates with late-onset disease. Antibiotic therapy for Listeria should include ampicillin plus an aminoglycoside, typically gentamicin.\n\nPneumonia caused by Streptococcus agalactaie has a reticulogranular pattern on chest radiography. Neonatal chlamydial infection presents later, typically between 4 and 12 weeks after birth, not in the immediate postnatal period. Neonatal infection with Escherichia coli most commonly manifests as bloodstream infection rather than pneumonia.\n\nPREP Pearls\n• Neonates born to mothers with Listeria infection are often delivered prematurely with brown- or green-stained amniotic fluid. Among premature infants, meconium-stained amniotic fluid is uncommon.\n• Early-onset neonatal Listeria is uncommon, with a high rate of mortality. These neonates may present with respiratory distress and an erythematous papular rash.\n• Late-onset neonatal Listeria typically presents with nonspecific findings among term infants at 14 days after birth. Meningitis is more common with late-onset neonatal Listeria.\n\nABP Content Specifications(s)\n• Differentiate respiratory distress syndrome from congenital pneumonia in a newborn infant\n• Recognize the characteristic clinical and radiographic appearance of respiratory distress syndrome in a newborn infant, and manage appropriately\n\nSuggested Readings\n• American Academy of Pediatrics. Listeria monocytogenes infections. In: Kimberlin DW, Brady MT, Jackson MA, Long SS, eds. Red Book: 2018 Report of the Committee on Infectious Diseases. Itasca, IL: American Academy of Pediatrics; 2018:511-515 Red Book Online.\n• Lee PJ, Krilov LR. Listeria. Pediatr Rev. 2018;39;153-155. doi: 10.1542/pir.2017-0062.\n• Posfay-Barbe KM, Wald ER. Listeriosis. Pediatr Rev. 2004;25;151-159. doi: 10.1542/pir.25-5-151."}
{"id" : 1181, "question_text" : "A 15-year-old adolescent boy presents to your office with a 1-month history of progressive fatigue and exercise intolerance. His physical examination is remarkable only for pallor. His weight is 60 kg. He specifically denies any history of hematuria, hematochezia, epistaxis, or unusual bruising. He has had a normal diet. The results of a complete blood cell count are shown in Item 130. Of the following, the MOST appropriate next step is to advise him to start", "options" : "[\"325 mg of iron sulfate twice daily on an empty stomach with a glass of orange juice and perform a stool guaiac\", \"325 mg of iron sulfate twice daily on an empty stomach with a glass of orange juice and repeat the blood cell counts in 4 weeks\", \"325 mg of iron sulfate twice daily with meals and perform a stool guaiac\", \"325 mg of iron sulfate twice daily with meals and repeat the blood cell counts in 4 weeks\", \"325 mg of iron sulfate twice daily with milk and perform a stool guaiac\"]", "explanation" : "This patient's presentation with new symptoms of anemia at 15 years of age suggests that his anemia is of new onset and therefore not hereditary. It is most likely that he has an iron deficiency anemia. A complete evaluation for iron deficiency includes a serum iron level, total iron binding capacity, reticulocyte count, and a ferritin level.\n\nThe size of red blood cells (RBC), as measured by the mean corpuscular volume (MCV) is in large part determined by the content of hemoglobin within the cell. Any deficiency in the components of hemoglobin will therefore result in a low MCV. The 2 primary components of hemoglobin that can be deficient are iron (deficiency of which causes decreased heme production) or the globin protein. Hemoglobin A, the normal adult hemoglobin variant, consists of 2 ß- and 2 a-globin chains, with the ß-globin gene located on chromosome 11 and the a-globin gene on chromosome 16. Mutations resulting in reduced production of either a- globin or ß-globin result in various thalassemia phenotypes, and present with a microcytic anemia. In order to form hemoglobin, the 4 globin subunits must bind to a molecule of heme, which is dependent on iron. Iron deficiency will therefore also result in a microcytic anemia. Although the patient in the vignette could theoretically have a variant of thalassemia, his acute findings are more consistent with iron deficiency.\n\nThe human body has a tight regulatory system for the absorption of iron, but no mechanism of iron excretion. It would be highly unusual for a male adolescent with a relatively normal diet to develop iron deficiency from dietary restriction. It is therefore very important that any time a male adolescent presents with iron deficiency anemia, a source of iron loss must be sought. Causes of iron loss in this population would include gastrointestinal bleeding, paroxysmal nocturnal hemoglobinuria, and pulmonary hemosiderosis. Paroxysmal nocturnal hemoglobinuria can be evaluated through flow cytometry on a blood sample, and pulmonary hemosiderosis can be initially screened through a chest radiograph. If suspicion of hemosiderosis is strong, sputum analysis for hemosiderin-laden macrophages is recommended. While all 3 should be evaluated in the patient in the vignette, the most common cause would be gastrointestinal bleeding. It would therefore be most appropriate to perform a stool guaiac.\n\nIron therapy for iron deficiency should include 2 mg to 4 mg of elemental iron per kg of weight daily. Elemental iron is 20% of iron sulfate. For the patient in the vignette, the most appropriate dose of elemental iron would be 120 mg to 240 mg daily, which would be 600 mg to 1,200 mg of iron sulfate daily. Iron is best absorbed on an empty stomach with an acidic or neutral fluid. It is therefore most appropriate to take iron with water or orange juice. The high concentration of casein and whey proteins in milk inhibits iron absorption, and tea contains chelators that will bind the iron and prevent its absorption. Medications that decrease the acidic environment of the upper gastrointestinal tract may also impair absorption of iron.\n\nPREP Pearls\n • Iron deficiency in a male adolescent is rarely dietary and needs further evaluation to rule out occult bleeding.\n • The nondietary causes of iron deficiency include blood loss (typically gastrointestinal), paroxysmal nocturnal hemoglobinuria, and pulmonary hemosiderosis.\n • Iron should be administered on an empty stomach with water or an acidic drink such as orange juice.\n\nABP Content Specifications(s)\n • Plan the appropriate diagnostic evaluation of iron deficiency\n • Recognize the clinical findings associated with iron deficiency in patients of various ages"}
{"id" : 302, "question_text" : "The mother of an 11-year-old girl brings in her daughter because she noted white-to-pale yellow vaginal discharge on the girl's cotton underwear when doing the laundry. The child denies discomfort or itching. The mother notes that the girl likes to take bubble baths. Results of a careful interview raise no concerns about sexual abuse or activity. Physical examination reveals normal vital signs with no fever and normal head, eyes, ears, nose, and throat findings without pharyngitis or conjunctivitis. She has Sexual Maturity Rating 3 breast development and normal genitourinary examination findings, with normal hymenal tissue, pink vaginal mucosa, and scant white discharge. No tenderness is elicited on abdominal examination. Of the following, the MOST likely cause of this girl's discharge is", "options" : "[\"chlamydial infection\", \"nonspecific vulvovaginitis\", \"physiologic leukorrhea\", \"streptococcal vaginitis\", \"vaginal candidiasis\"]", "explanation" : "The girl described in the vignette has a painless, scant white vaginal discharge. Because she has a Sexual Maturity Rating of 3, it is most likely that the discharge represents the estrogenic effect on the vaginal mucosa as a precursor of menarche, usually occurring 4 to 6 months after the onset of such physiologic leukorrhea.\n\nThe differential diagnosis of vaginal discharge associated with complaints of discomfort or foul smell include foreign body (as trivial as retained toilet paper or as significant as foreign objects deliberately inserted), sexual abuse and sexually transmitted infection (eg, chlamydial infection), pinworms, vaginal candidiasis, and group A beta-hemolytic streptococcal infection. In addition, especially in younger girls, structural abnormalities such as ectopic ureter, urethral prolapse, rectovaginal fistula, and neoplasms (including sarcomas) must be considered as rare causes.\n\nThe most common cause of vulvar itching or thin vaginal discharge, especially in the prepubertal girl, is nonspecific vulvovaginitis, which is usually caused by irritation of this sensitive area by perfumes, deodorant soaps, shampoos, or bubble baths. The condition also may be caused by inadequate genital hygiene (eg, inadequate wiping or wiping from \"back to front\") or overly aggressive genital hygiene after voiding or stooling, which causes excessive friction.\n\nStreptococcal vaginitis is usually accompanied by pain, severe erythema of the vulva and often the perianal area, and constitutional signs and symptoms (eg, fever, nausea, and malaise). It may occur with group A streptococcal pharyngitis and is treated with penicillins or cephalosporins.\n\nSexual abuse or other sexual contact must be considered, even in the absence of signs of penetration, in the girl who presents with vulvar and vaginal complaints, especially discharge, regardless of age. Sexually transmitted infections may be spread not only by genital-to-genital contact but also by oral-genital contact. Gonococcal infections generally present with purulent discharge, but signs of chlamydial infection may be minimal. Trichomonas vaginalis infection and bacterial vaginosis are uncommon in prepubertal children.\n\nCritique: The girl described in the vignette has a painless, scant white vaginal discharge. Because she has a Sexual Maturity Rating of 3, it is most likely that the discharge represents the estrogenic effect on the vaginal mucosa as a precursor of menarche, usually occurring 4 to 6 months after the onset of such physiologic leukorrhea.\n\nThe differential diagnosis of vaginal discharge associated with complaints of discomfort or foul smell include foreign body (as trivial as retained toilet paper or as significant as foreign objects deliberately inserted), sexual abuse and sexually transmitted infection (eg, chlamydial infection), pinworms, vaginal candidiasis, and group A beta-hemolytic streptococcal infection. In addition, especially in younger girls, structural abnormalities such as ectopic ureter, urethral prolapse, rectovaginal fistula, and neoplasms (including sarcomas) must be considered as rare causes.\n\nThe most common cause of vulvar itching or thin vaginal discharge, especially in the prepubertal girl, is nonspecific vulvovaginitis, which is usually caused by irritation of this sensitive area by perfumes, deodorant soaps, shampoos, or bubble baths. The condition also may be caused by inadequate genital hygiene (eg, inadequate wiping or wiping from \"back to front\") or overly aggressive genital hygiene after voiding or stooling, which causes excessive friction.\n\nStreptococcal vaginitis is usually accompanied by pain, severe erythema of the vulva and often the perianal area, and constitutional signs and symptoms (eg, fever, nausea, and malaise). It may occur with group A streptococcal pharyngitis and is treated with penicillins or cephalosporins.\n\nSexual abuse or other sexual contact must be considered, even in the absence of signs of penetration, in the girl who presents with vulvar and vaginal complaints, especially discharge, regardless of age. Sexually transmitted infections may be spread not only by genital-to-genital contact but also by oral-genital contact. Gonococcal infections generally present with purulent discharge, but signs of chlamydial infection may be minimal. Trichomonas vaginalis infection and bacterial vaginosis are uncommon in prepubertal children.\n\nContent Specifications: Evaluate and recognize the cause of a vaginal discharge in a preadolescent girl"}
{"id" : 2698, "question_text" : "A previously healthy, 16-year-old adolescent boy is seen in the emergency department. Two days ago, he fell from his bicycle and sustained a minor injury to his left arm. For 1 day he has had pain, redness, and swelling of his left upper extremity and fever up to 39.4°C. The pain and swelling have increased in severity over the past few hours, with spread of the redness to his shoulder. He has no sick contacts, exposure to pets or animals, or recent travel. The adolescent appears toxic, with a temperature of 39.1°C, heart rate of 154 beats/min, respiratory rate of 42 breaths/min, and blood pressure of 83/36 mm Hg. Musculoskeletal examination is significant for exquisite tenderness and tense swelling of his left arm with a purplish discoloration of the skin. The remainder of his physical examination findings are unremarkable. A complete blood count shows a white blood cell count of 23,000/µL (23 × 109/L) with 97% neutrophils, a hemoglobin level of 10.9 g/dL (109 g/L), and a platelet count of 76 × 103/µL (76 × 109/L). After a blood sample is drawn for culture, intravenous vancomycin and ceftazidime are administered. Of the following, the BEST next step in this adolescent's management is", "options" : "[\"administration of clindamycin, intravenously\", \"magnetic resonance imaging of the left arm\", \"plain radiography of the left arm\", \"urgent surgical debridement\"]", "explanation" : "The ill-appearing adolescent in the vignette has a clinical picture suggestive of necrotizing fasciitis (NF) following a history of recent trauma. Given the significant mortality and morbidity associated with NF, surgical exploration for debridement is urgently indicated. Necrotizing fasciitis is a life-threatening pyogenic infection of the subcutaneous tissues and fascia characterized by rapid spread along the fascial planes and thrombosis of the nutrient vessels, leading to ischemia and necrosis. The trunk and lower limbs are most commonly affected in children. Pain disproportionate to local findings is the classical presentation and should alert the physician to consider the diagnosis of NF; signs of systemic toxicity are often noted.\n\nNecrotizing fasciitis is unusual in children; the incidence is 0.08 to 0.13 per 100,000 children per year. Case-fatality rates are high (10%). Group A Streptococcus (GAS) is a well-recognized causative pathogen. Escherichia coli and other gram-negative rods, and Staphylococcus aureus, including methicillin-resistant S aureus (MRSA), have also been implicated. Infection can be polymicrobial and include anaerobes (eg, Peptostreptococcus and Bacteroides fragilis), especially when the perineum is involved. In immunocompromised hosts with neutropenia, NF due to Pseudomonas aeruginosa and Clostridium septicum has been reported. Myonecrosis caused by Clostridium perfringens may complicate contaminated surgical or traumatic wounds. While affected individuals often report a history of trauma, other risk factors for NF include eczema, surgery, immune deficiency, and diabetes. Prior to the availability of varicella vaccine, NF and other forms of invasive group A streptococcal infection (eg, toxic shock syndrome, bacteremia without a source, pneumonia) occurred in 15% to 30% of children with varicella disease. In cases of NF associated with streptococcal toxic shock syndrome, pathogen virulence (eg, pyrogenic exotoxin A) and host susceptibility factors may contribute to the disease severity.\n\nThe pathogenesis of NF involves entry of bacteria into the subcutaneous tissue via a breach in the skin barrier or by hematogenous spread. Early clinical findings include severe pain and tenderness on palpation of the affected area. There may be associated swelling and erythema. Fever and other non-specific complaints (eg, vomiting, myalgia, and diarrhea) may be noted. Early diagnosis of NF may be challenging as the disease can mimic cellulitis or a viral illness when there is no local evidence of skin injury or infection. However, pain in the affected area disproportionate to local findings is a crucial, early clue for NF; systemic toxicity is another red flag, if present. As the disease progresses, the affected area may become markedly swollen followed by the appearance of hemorrhagic bullae (or blisters), crepitus, and gangrene. A rapidly progressive clinical course with sepsis, septic shock, and multisystem involvement is a frequent occurrence in cases of NF caused by GAS strains.\n\nThe clinical suspicion of NF is a surgical emergency. Surgical intervention may range from aggressive debridement of all devitalized tissue to amputation, based on the operative findings of disease severity. In many cases, repeated resection of necrotic tissue is necessary for source control and removal of devitalized tissue. Tissue specimens must be submitted to the laboratory for bacterial cultures and histopathology. An incisional frozen-section biopsy may be considered in cases in which the diagnosis is uncertain.\n\nPerformance of imaging studies should never delay surgical exploration when NF is suspected. Imaging studies may be warranted when the clinical diagnosis is in doubt. Plain radiographs are not recommended, as they may appear normal in cases of NF. Contrast-enhanced computed tomography (CT) is very sensitive and specific, and is the imaging modality of choice to confirm the diagnosis of NF. Extensive soft tissue involvement and presence of gas may be noted on CT. Magnetic resonance imaging is very sensitive and specific, but challenging to obtain in the emergency setting.\n\nManagement of NF requires a multidisciplinary team involving infectious disease specialists, surgeons, and critical care specialists. In addition to early surgical debridement, aggressive critical care support and empiric broad-spectrum antimicrobial therapy is recommended. Antibiotic coverage should include gram-positive cocci (including MRSA), gram-negative rods, and anaerobic pathogens. Culture with sensitivities is used to guide specific antibiotic choice once results are available. Treatment of GAS infection includes high-dose intravenous penicillin plus a protein-synthesis inhibitor (eg, clindamycin). Clindamycin is recommended due to its anti-toxin activity and other properties (eg, long postantibiotic effect unaffected by inoculum size compared to penicillin). Adjunctive immunomodulatory therapy with intravenous immunoglobulin may improve outcomes in cases of streptococcal toxic shock syndrome associated with NF.\n\nPREP Pearls\n• Necrotizing fasciitis is a severe, life-threatening pyogenic infection of the subcutaneous tissues and fascia characterized by rapidly progressive spread of infection along the fascial planes, resulting in tissue necrosis and often sepsis.\n• Recent trauma, burns, surgery, and varicella infection are common risk factors for necrotizing fasciitis in children.\n• Necrotizing fasciitis is a surgical emergency. Urgent surgical exploration with aggressive debridement of devitalized tissue is required.\n\nABP Content Specifications(s)\n• Recognize the clinical findings associated with necrotizing fasciitis\n\nSuggested Readings\n• American Academy of Pediatrics. Group A streptococcal infections. In: Kimberlin DW, Barnett ED, Lynfield R, Sawyer MH, eds. Red Book: 2021–2024 Report of the Committee on Infectious Diseases. 32nd ed. American Academy of Pediatrics; 2021. Accessed September 1, 2022. Red Book Online.\n• Diab J, Bannan A, Polity T. Necrotizing fasciitis. BMJ. 2021;369:m1428 doi:10.1136/bmj.m1428.\n• Marathe K, Williams JV. Bacterial skin infections. In: McInerny TK, Adam HM, Campbell DE, DeWitt TG, Foy JM, Kamat DM, eds. American Academy of Pediatrics Textbook of Pediatric Care. American Academy of Pediatrics; 2021:chap 222. Accessed September 1, 2022. Pediatric Care Online.\n• Nelson GE, Pondo T, Toews KA, et al. Epidemiology of invasive group A Streptococcal infections in the United States, 2005-2012. Clin Infect Dis. 2016;63(4):478-486. doi:10.1093/cid/ciw248.\n• Noor A, Krilov LR. Necrotizing fasciitis. Pediatr Rev. 2021;42(10):573-575. doi:10.1542/pir.2020-003871.\n• Schröder A, Gerin A, Firth GB, et al. A systematic review of necrotising fasciitis in children from its first description in 1930 to 2018. BMC Infect Dis. 2019;19(1):317. doi:10.1186/s12879-019-3941-3."}
{"id" : 1850, "question_text" : "A 3-year-old girl is being seen at a new patient visit. One week ago, she was removed from her mother's custody and placed into the custody of her aunt (who brought her to the pediatrician to establish a new medical home). The patient's mother, who has a history of substance abuse, has been charged with child neglect and endangerment. The girl was born full-term. She has a history of failure to thrive, an elevated blood lead level, and speech delay. She was hospitalized 6 months ago after ingesting a household substance that resulted in extensive esophageal burns. She has had 2 subsequent hospital stays for management of esophageal strictures that developed as a complication of this injury. Of the following, the household substance the girl MOST likely ingested is", "options" : "[\"drain cleaner\", \"household bleach\", \"insecticide\", \"lamp oil\"]", "explanation" : "The 3-year-old girl in the vignette sustained extensive esophageal burns and later developed esophageal strictures by ingesting a household cleaning substance. She most likely ingested an alkaline product, such as drain cleaner.\n\nMost caustic ingestions in young children occur in their homes, where both toxic alkaline and acidic products are often readily available. These products may cause significant toxicity if ingested. Caustics—also known as corrosives—are concentrated acidic, alkaline, or oxidizing agents. Many are found in common household products including drain cleaners, toilet bowl cleaners, laundry detergents, stain and mildew removers, floor cleaners, oven cleaners, swimming pool cleaners, rust removers, phenol-based disinfectants, swimming pool products, and batteries.\n\nAll household chemical products should be stored in their original labeled containers and kept out of the reach of children at all times to prevent accidental exposure. The US Centers for Disease Control and Prevention estimate that more than a half million children are treated emergently each year for acute poisoning with corrosives. Ingestion of even small amounts of a corrosive alkaline substance, such as drain cleaners, toilet bowl cleaners, or rust remover, can result in serious penetrating injuries to mucosal and skin surfaces by liquefaction necrosis. Acidic substances tend to cause injury via coagulation necrosis; acids carry a relatively lower risk of esophageal perforation, but are still capable of producing serious injury.\n\nPatients ingesting corrosive substances typically present with odynophagia, dysphagia, drooling, intraoral injury, and esophageal burns and/or ulcerations. There is a significant potential for later development of strictures. Additional signs and symptoms may include vomiting with hematemesis, respiratory difficulty with stridor or wheezing, hoarseness, retrosternal chest pain, dyspnea, and burns on the face, hands, or chest. Significant burns to the eyes may occur with any ocular exposure. Because the primary mode of injury is direct tissue corrosion, systemic symptoms are rare. Apart from distress caused by pain, mental status is usually normal in affected children.\n\nThe most pressing clinical concern after a caustic ingestion is the potential for airway and/or esophageal injury. The presence or absence of intraoral injuries does not always correlate with the presence of injury to the lower airway or esophagus. Early airway visualization and protection are indicated in any child presenting with a suspected caustic ingestion and stridor or respiratory distress. Evaluation of the esophagus with upper endoscopy is indicated in children with a history strongly suggesting ingestion of a corrosive product and in those with intraoral burns or other symptoms related to the ingestion.\n\nThe ingestion of household bleach, insecticide, or lamp oil would not typically cause the esophageal injury seen in this child.\n\nAlthough household bleach (sodium hypochlorite) is a substance with an alkaline pH (pH ~12), retrospective studies of children ingesting liquid household bleach have found that accidental ingestion is typically associated with a benign clinical course and does not require hospitalization or pharmacologic interventions.\n\nIngestion of an insecticide could result in organophosphate toxicity, characterized by clinical signs and symptoms related to the overactivation of cholinergic receptors by excess acetylcholine. The classic features of cholinergic toxicity can be recalled with the mnemonic SLUDGE: salivation, lacrimation, urination, defecation/diarrhea, gastrointestinal upset, and emesis. Although tachypnea, bradycardia, and pupillary constriction are among the expected abnormalities resulting from insecticide ingestion, this ingestion would not typically result in esophageal injury.\n\nLamp oil is a hydrocarbon-based product, and ingestion would primarily cause respiratory distress due to pulmonary aspiration. In acute situations, patients who ingest/aspirate a hydrocarbon present with coughing, gagging and choking. Physical examination findings may include fever, tachypnea, cyanosis, and abnormal lung sounds which may include crackles and wheezing. Direct central nervous system effects of the hydrocarbon may lead to lethargy, seizures, or even coma.\n\nPREP Pearls\n\nMost caustic ingestions in young children occur in their homes. Common products include: drain, toilet bowl, floor, and oven cleaners; laundry detergents; stain, mildew, and rust removers; phenol-based disinfectants; swimming pool products; and batteries.\n\nIngestion of even small amounts of a corrosive alkaline substance can result in serious penetrating injuries to mucosal and skin surfaces due to liquefaction necrosis. The most pressing clinical concern is the potential for airway and/or esophageal injury.\n\nAll household chemical products should be stored in their original labeled containers and kept out of the reach of children at all times to prevent accidental exposure.\n\nABP Content Specifications(s)/Content Area\n\nKnow the common household sources of acids and alkali\n\nRecognize the signs and symptoms of ingestion of a caustic substance, and manage appropriately\n\nSuggested Readings\n\nHarley EH, Collins MD. Liquid household bleach ingestion in children: a retrospective review. Laryngoscope. 1997;107(1):122-125. doi: 10.1097/00005537-199701000-00023.\n\nKay M, Wyllie R. Caustic ingestions in children. Curr Opin Pediatr 2009;21(5):651-654. doi: 10.1097/MOP.0b013e32832e2764.\n\nTurner A, Robinson P. Respiratory and gastrointestinal complications of caustic ingestion in children. Emerg Med J.2005;22:359-361. doi: 10.1136/emj.2004.015610."}
{"id" : 2127, "question_text" : "Emergency department staff receive notice that a \"dirty\" bomb, containing explosive material and radioactive nuclear waste, has been detonated near a school. Thirty injured children are being transported to the emergency department. The hospital's disaster response and trauma teams have been notified. Of the following, the MOST essential patient care item to have available is", "options" : "[\"bleach for cleaning patients' skin after removing clothing\", \"potassium iodide for oral administration\", \"single-use patient monitoring equipment\", \"staff personal protective equipment with goggles and masks\"]", "explanation" : "Critique\nThe term \"dirty bomb\" refers to an explosive device containing radioactive material. The principal risk from a dirty bomb is the explosion itself, which results in injuries caused both by the device's exploding and by damage to nearby structures and objects. Once the radioactive material settles to the ground, it poses a risk only to individuals within a limited radius of the explosion. There is a health risk if the radioactive material is then inhaled or absorbed through open skin. By contrast, a nuclear device can spread radioactive material for hundreds of miles. \nHospital preparations for providing care for victims of a dirty bomb explosion should include alerting hospital leadership to establish an incident command center and activating the trauma response team. It is essential for all staff members to wear personal protective equipment (gowns, gloves, goggles, and masks) until all victims have been decontaminated through the removal of clothing and irrigation/showering to remove any radioactive material and prevent its transfer to health care personnel.\nThe other preparatory measures listed in the response choices are not essential for responding to the scenario in the vignette. Although bleach may be used to wipe down surfaces and objects used by victims, it should not be applied to the skin of victims after a dirty bomb explosion. Bleach may irritate the skin and potentially enhance penetration of radioactive powder into open wounds. Monitors used in the care of individuals affected by a dirty bomb explosion will need to be carefully cleaned in accordance with appropriate protocols, but it is not necessary to discard monitors used in this situation. Although this practice has been recommended after care of individuals affected by infectious agents such as Ebola virus, the same measure has not been recommended for situations involving dirty bomb explosions/exposures. The use of potassium iodide is recommended for reducing thyroid cancer risk after exposure to ionizing radiation. However, dirty bombs do not contain this type of radioactive material. Therefore, the children in the vignette would not require treatment with potassium iodide.\n\nSuggested Reading(s)\nChecklist: essential pediatric domains and considerations for every hospital's disaster preparedness policies. Emergency Medical Services for Children Innovation and Improvement Center. Accessed March 5, 2024. media.emscimprovement.center/documents/Checklist_HospitalDisasterPrepared2125.pdf\nDart RC, Goldfrank LR, Erstad BL, et al. Expert consensus guidelines for stocking of antidotes in hospitals that provide emergency care. Ann Emerg Med. 2018;71(3):314-325.e1. doi:10.1016/j.annemergmed.2017.05.021\nGilchrist N, Simpson JN. Pediatric disaster preparedness: identifying challenges and opportunities for emergency department planning. Curr Opin Pediatr. 2019;31(3):306-311. doi:10.1097/MOP.0000000000000750\nKutanzi KR, Lumen A, Koturbash I, Miousse IR. Pediatric exposures to ionizing radiation: carcinogenic considerations. Int J Environ Res Public Health. 2016;13(11):1057. doi:10.3390/ijerph13111057\n\nContent Domain\nEmergency Medicine\n\nLearning Objectives\nDemonstrate preparatory measures for care of patients from dirty bomb exposure\nDefine the risks from a \"dirty bomb.\"\n\nThe correct answer is: staff personal protective equipment with goggles and masks"}
{"id" : 3569, "question_text" : "A policy recommending a 60-second delay in cord clamping for all neonates was recently implemented. As part of a Plan-Do-Study-Act cycle, the initial hematocrit values for all neonates delivered over the past year are being evaluated. The mean hematocrit value ranges from 42% to 52%. Of the following, the MOST important factor explaining this variability is the", "options" : "[\"gestational age\", \"maternal age\", \"maternal body mass index\", \"sex of the neonate\"]", "explanation" : "Hematocrit is the ratio of volume of red blood cells per volume of blood. Many factors influence neonatal hematocrit values. In a population-based sample, such as in this vignette, gestational age is the best explanation for the range of hematocrit values seen in this neonatal cohort (Item C13).\n\nBoth fetal and neonatal erythropoiesis are stimulated by hypoxia. However, red blood cell production differs between the developmental periods. Fetal hemoglobin has a higher affinity for oxygen than adult hemoglobin, which may contribute to relative hypoxia in utero. Fetal erythropoietin (EPO), produced by the liver, is less sensitive to hypoxia than is the kidney (which is the main source of erythropoietin in older children and adults), resulting in slower production of EPO. During the third trimester, maternal iron is transferred to the fetus, resulting in increased red blood cell production. Neonates born prematurely do not benefit from these additional iron stores. Those born at earlier gestational ages are most severely affected. Hematocrit values do not vary by neonatal sex. Maternal age and body mass index, independent of vascular disease, do not affect neonatal hematocrit values. Hypoxia caused by placental insufficiency, congenital heart disease, and altitude above sea level also increases intrauterine red blood cell production, resulting in increased hematocrit values.\n\nPREP Pearls\n• At birth, term neonates have a higher hematocrit than preterm neonates.\n• Preterm neonates have a lower hematocrit than term neonates because of decreased sensitivity of hepatic sensors to hypoxia and reduced iron stores.\n• Hypoxia caused by placental insufficiency, congenital heart disease, and altitude above sea level may lead to increased intrauterine red blood cell production.\n\nABP Content Specifications(s)\n• Recognize the differences in hematocrit values in pre- and full-term infants, and the normal ranges for both\n\nSuggested Readings\n• Jopling J, Henry E, Wiedmeier SE, Christensen RD. Reference ranges for hematocrit and blood hemoglobin concentration during the neonatal period: data from a multihospital health care system. Pediatrics. 2009;123(2):e333-e337. doi:10.1542/peds.2008-2654.\n• Kates EH, Kates JS. Anemia and polycythemia in the newborn. Pediatr Rev. 2007;28(1):33-34. doi:10.1542/pir.28-1-33.\n• McGann PT, Ware RE. Anemia in the newborn infant. In: Kliegman RM, St Geme JW III, Blum NJ, Shah SS, Tasker RC, Wilson KM, eds. Nelson Textbook of Pediatrics. 21st ed. Philadelphia, PA: Elsevier; 2020:961-966."}
{"id" : 3679, "question_text" : "A 6-month-old infant is seen for a health supervision visit in the spring. She is developing normally, and her mother has no concerns. During anticipatory guidance, outdoor safety is reviewed. The mother asks about how to best protect her infant from insects. Of the following, the BEST option to address the mother's concern is", "options" : "[\"an aerosolized spray with 30% DEET\", \"a backyard bug zapper\", \"a citronella oil and lemon eucalyptus wristband\", \"a combination product with 30 SPF sunscreen and 30% DEET\"]", "explanation" : "Correct Answer: A\nQuestions regarding tick and mosquito repellents, including what methods are safe and most effective, are common in pediatric practice. Repellents with DEET (N,N-diethyl-3-methylbenzamide) are commonly used and are the most effective in protecting infants and children from ticks and mosquitos. The duration of protection depends on the concentration of DEET. Products containing 10% DEET typically last about 2 hours, and products with 24% DEET typically last around 5 hours. There is no known benefit in duration of protection with concentrations of DEET greater than 30% to 50%. Of the response choices, an aerosol of 30% DEET is the best option to protect a 6-month-old infant from ticks and mosquitos.\n\nThe American Academy of Pediatrics states that it is safe to use DEET-containing products on children as young as 2 months of age. The product should be applied to clothing and only exposed skin. Children should be bathed at the end of the day to remove repellant from the skin, and their clothes should be washed before wearing again. Other safety precautions include avoiding direct contact to the face or any wounds, encouraging the use of products to be supervised by an adult, and only reapplying if needed.\n\nA backyard bug zapper would not be recommended because it can actually attract more bugs to the area. Wristbands soaked in repellents such as citronella or eucalyptus are likely safe but do not offer adequate protection. There are topical repellents made from eucalyptus or oil of lemon that appear to repel mosquitos for about 1 to 2 hours. Testing for the ability to repel ticks is still needed, and allergic reactions to the essential oils can occur. Combination products with sunscreen and DEET are not recommended because sunscreen needs to be reapplied every 1 to 2 hours but DEET-containing products should be reapplied infrequently. The DEET can also make the sunscreen less effective.\n\nPREP Pearls\n• Products containing DEET are safe in children 2 months of age and older.\n• Higher concentrations of DEET provide longer protection.\n• Concentrations of DEET greater than 30% provide little added benefit for protection from ticks and mosquitos.\n\nABP Content Specifications(s)\n• Identify the measures to prevent tick- and mosquito-borne infections\n• Advise parents regarding the appropriate use of topical insect repellants in children\n\nSuggested Readings\n• American Academy of Pediatrics. Choosing an insect repellent for your child. HealthyChildren.org. https://www.healthychildren.org/English/safety-prevention/at-play/Pages/Insect-Repellents.aspx.\n• American Academy of Pediatrics. Patient education handouts: parent's guide to insect repellents, A. Pediatric Patient Education.\n• Mutebi J-P, Hawley WA, Brogdon WG. Protection against mosquitoes, ticks, and arthropods. In: Brunette GW, ed. CDC Health Information for International Travel 2018 (Yellow Book 2018). New York, NY: Oxford University Press; 2017:81-87. https://wwwnc.cdc.gov/travel/yellowbook/2018/the-pre-travel-consultation/protection-against-mosquitoes-ticks-other-arthropods."}
{"id" : 1211, "question_text" : "A 9-year-old girl presents to your office for evaluation of right wrist pain 2 days after falling onto an outstretched arm during a soccer game. On physical examination, the girl is tender over the right ulnar mid shaft with limited pronation and supination. Neurovascular examination of the hand and wrist is unremarkable. Radiographs of the wrist demonstrate anterior bowing of the shaft of the ulna. Of the following, the MOST appropriate next step would be to", "options" : "[\"allow return to activities without immobilization once she regains pronation and supination\", \"obtain dedicated radiographs of the elbow\", \"obtain magnetic resonance imaging of the forearm\", \"recommend cast immobilization of the wrist and forearm\", \"refer to orthopedic surgery for operative fixation of the ulna\"]", "explanation" : "The girl in the vignette has restricted pronation and supination, which should prompt evaluation for an associated elbow injury. Examination of the elbow after an acute injury should include inspection for deformity, swelling, bruising, and evaluation of motion. Range of motion is often restricted with both acute and chronic injuries.\nThe medial border of the ulna should be perfectly straight on lateral radiography; curvature suggests plastic deformity, bowing of the bone on radiographs without evidence of cortical dysfunction. Similar to greenstick fractures, plastic deformity is only seen in pediatric patients because of the increased flexibility of young bones. The presence of an ulnar deformity with limited pronation and supination raises concern for a Monteggia lesion—fracture or deformation of the ulna associated with a radiocapitellar dislocation. Although Monteggia lesions are rare, failure to diagnose this condition can lead to subsequent disability. Therefore, any patient with a midshaft or proximal ulnar injury, even patients with plastic deformity or mild greenstick fracture, should undergo dedicated elbow radiography to examine the radiocapitellar joint. Item C174 shows a greenstick fracture of the ulna with an associated radiocapitellar dislocation.\nMonteggia lesions were classified by Bado into 4 types. The most common is a type 1 injury, with fracture of the proximal or midshaft of the ulna and associated anterior dislocation of the radial head. There are also Monteggia lesion variants, ulnar injuries with radial head subluxation that were not included in Bado's classification system. Monteggia injuries represent fewer than 1% of pediatric fractures and typically occur in children younger than 12 years. A Monteggia lesion identified within 3 weeks of injury often can be treated with closed reduction. Monteggia lesions with delayed diagnosis and fracture healing, or those unsuccessfully treated by closed reduction, require open reduction with ulnar osteotomy to restore normal radiocapitellar joint anatomy.\nElbow dislocations at the ulnar-trochlear joint are rare in children, with the incidence rising during adolescence. Dislocations most often occur in the posterolateral direction. When an elbow dislocation is suspected, radiographs should always be obtained before performing a reduction maneuver, both to confirm the diagnosis and to look for associated fractures.\nFor the girl in the vignette, a return to sports without imaging to evaluate the elbow and immobilization for the ulnar deformity would not be appropriate. Magnetic resonance imaging is not indicated because a Monteggia lesion, if present, should be visible on plain radiography. Cast immobilization of the wrist and forearm would be appropriate if no radiocapitellar disruption is seen on elbow radiography. The patient should have close follow-up because of the risk for radiocapitellar dislocation in the first few weeks after injury. If a Monteggia lesion is seen on radiography, referral to orthopedic surgery would be indicated. Because the girl in the vignette was injured only 2 days before presentation, the appropriate first step would be to attempt closed reduction under sedation.\nPREP Pearls\n For children with any type of acute ulnar deformity or fracture, dedicated elbow radiographs should be obtained to assess for possible Monteggia lesion (radiocapitellar joint dislocation).\n Elbow dislocation at the ulna/trochlear joint is uncommon in children.\nABP Content Specifications(s)\n Recognize the clinical findings associated with sports-related dislocation of the elbow, including associated complications, and manage appropriately\n Recognize the clinical findings associated with sports-related elbow pain, and manage appropriately"}
{"id" : 1123, "question_text" : "You are caring for a 16-year-old adolescent diagnosed 1 year ago with Crohn disease, with fistulas affecting the ileum and colon. Despite aggressive medical management, she required resection of her ileum, including her ileocecal valve 4 months ago. Since her surgery, she has had persistent diarrhea. She is currently on a regular, low fiber diet without supplementation. Stool studies demonstrate normal bacterial flora, negative reducing substances, and are heme negative. Of the following, the patient's diarrhea is MOST likely caused by malabsorption of", "options" : "[\"bile\", \"fat\", \"fructose\", \"lactose\", \"sucrose\"]", "explanation" : "Crohn disease is a chronic inflammatory condition of the gastrointestinal tract. In addition to having a chronic disease, the child in this vignette has lost most of her ileum. Absorption of bile occurs in the ileum and malabsorption results in chronic diarrhea, as in the case in the girl in this vignette. Disaccharides (lactose, fructose, sucrose, etc) are digested and absorbed in the duodenum and jejunum. Small bowel inflammation may result in injury to villi, causing a secondary disaccharidase deficiency resulting in diarrhea. Malabsorption of carbohydrates results in increased stool reducing substances, not seen in the child in the vignette. Fat is digested and absorbed in the proximal intestine, and can be malabsorbed in severe chronic inflammation, however, this is fairly uncommon. Elevated fecal fat levels would identify this as a possible etiology.\nCrohn disease may affect the small bowel and the colon. Malnutrition can occur in Crohn disease caused by chronic inflammation, as an adverse effect of medication or due to surgical resection. The most common cause of nutritional issues in Crohn disease is inadequate intake of calories or protein. Patients with active disease have a significant increase in their nutritional needs that exceed their ability to ingest sufficient calories. Additional nutritional deficiency concerns resulting from gastrointestinal disorders are listed in Item C27.\nPREP Pearls\n Patients with Crohn disease are at increased risk for malnutrition and nutritional deficiency.\n Understanding the anatomy following gastrointestinal resection will help to identify nutrients that may require additional supplementation.\n Malabsorption of bile, disacharrides, and fats can result in worsening diarrhea.\nABP Content Specifications(s)\n Recognize the nutritional deficiencies associated with gastrointestinal disease"}
{"id" : 2310, "question_text" : "A previously healthy 8-month-old girl is brought to the emergency department shortly after falling out of her high chair. Her mother saw the girl stand in the chair, then fall from approximately 3 feet onto a hardwood floor, landing head first. The girl appeared dazed and did not cry immediately, but then started to cry vigorously. Her mother notified the pediatrician, who instructed her to bring the child to the emergency department. On physical examination, the girl's vital signs are normal for age except for a heart rate of 160 beats/min and a blood pressure of 125/80 mm Hg. There is noticeable swelling over the right side of her head. She becomes progressively somnolent but wakes when she is examined and to noxious stimulation. Her Glasgow Coma Scale score is 11 (eye opening 3, verbal 3, motor 5). She moves all extremities; however, her left side moves less than her right. The remainder of the girl's physical examination findings are normal. Results of noncontrast computed tomography of the head are shown (Figure 1). Of the following, this girl's MOST likely diagnosis is", "options" : "[\"concussion\", \"diffuse axonal injury\", \"epidural hematoma\", \"subdural hematoma\"]", "explanation" : "Critique\nThe girl in the vignette sustained blunt head trauma after falling onto a hard surface, resulting in a moderate traumatic brain injury (TBI). Children with TBI that is moderate (Glasgow Coma Scale [GCS] score of 9-13) to severe (GCS score of 3-8) often have distinct intracranial injuries. The most common manifestations of intracranial hemorrhage caused by TBI in children are epidural and subdural hematomas. The hyperdense lens-shaped collection of blood seen on the girl's computed tomographic (CT) scan is characteristic of an epidural hematoma (Figure 1). \nEpidural hematomas result from arterial bleeding that fills the potential space between the dura mater and the skull; they are often associated with skull fractures. As a result of arterial disruption, epidural hemorrhages can quickly create a mass effect on the brain, leading to rapid neurologic deterioration.\nIn contrast, subdural hemorrhage results from disruption of the subarachnoid bridging veins that leads to accumulation of blood in the virtual space between the dura and arachnoid layer. Subdural hematomas (Figure 2) have a characteristic concave or crescent-shaped hyperdense appearance on CT. Because of cerebral compliance, subdural hemorrhage is generally well tolerated in young children and rarely leads to rapid neurologic deterioration. However, if the hematoma evolves quickly and exceeds the ability to displace other intracranial components, neurologic deterioration can ensue. The most catastrophic consequence of any intracranial hemorrhage with cerebral displacement is cerebral herniation.\nDiffuse axonal injury is caused by significant acceleration and deceleration forces. The stretching and tearing of neurons results in diffuse edema that may not be apparent on initial head imaging. Concussion occurs when the brain rapidly shifts inside the skull, often from a blow to the head. Concussion symptoms may or may not include altered mental status, headache, confusion, nausea, dizziness, tinnitus, and alterations in memory. Although a coexisting concussion is possible for the girl in the vignette, the findings of an epidural hematoma on CT make concussion unlikely to be the primary cause of her evolving symptoms.\nTraumatic brain injury is classified as mild, moderate, or severe, depending on the degree of neurologic dysfunction as measured by the Glasgow Coma Scale:\nMild—GCS score of 14 to 15 \nModerate—GCS score of 9 to 13 \nSevere—GCS score of 3 to 8 \nThe GCS requires modification for infants to account for child development. The score is the sum of the points from each of 3 clinical neurological testing domains (Table).\nChildren with minor head injuries may not require radiographic imaging. Recent clinical guidelines suggest that children at low to intermediate risk of experiencing intracranial hemorrhage can be observed for 4 to 6 hours in the emergency department and do not require CT imaging if they remain asymptomatic. Guidelines from the Pediatric Emergency Care Applied Research Network (Schonfeld 2014) recommend judicious observation of children who are seen in the emergency department within 24 hours of injury and who have GCS scores of 14 to 15. The Canadian Assessment of Tomography for Childhood Head Injury guideline contains similar recommendations.\nTreatment of more severe head injuries consists of stopping the progression of the primary insult and preventing or reducing secondary brain injury; this requires early recognition and management. Intracranial hemorrhage may require surgical intervention to address the primary injury. Neurological protection measures aim to prevent secondary injury and require attention to normal physiological parameters. In severe traumatic brain injury, intracranial pressure monitoring with targeted interventions focused on preservation of normal cerebral perfusion may be required. \nSuggested Reading(s)\nGelineau-Morel RN, Zinkus TP, Le Pichon JB. Pediatric head trauma: a review and update. Pediatr Rev. 2019;40(9):468-481. doi:10.1542/pir.2018-0257\nHolmes JF, Palchak MJ, MacFarlane T, Kuppermann N. Performance of the pediatric Glasgow Coma Scale in children with blunt head trauma. Acad Emerg Med. 2005;12(9):814-819. doi:10.1197/j.aem.2005.04.019\nKochanek PM, Tasker RC, Carney N, et al. Guidelines for the management of pediatric severe traumatic brain injury: update of the Brain Trauma Foundation guidelines, executive summary. Neurosurgery. 2019;84(6):1169-1178. doi:10.1093/neuros/nyz051\nNigrovic LE, Kuppermann N. Children with minor blunt head trauma presenting to the emergency department. Pediatrics. 2019;144(6):e20191495. doi:10.1542/peds.2019-1495\nSchonfeld D, Bressan S, Da Dalt L, Henien MN, Winnett JA, Nigrovic LE. Pediatric Emergency Care Applied Research Network head injury clinical prediction rules are reliable in practice. Arch Dis Child. 2014 May;99(5):427-431. doi:10.1136/archdischild-2013-305004\nContent Domain\nCritical Care\nABP Content Specification(s) / Content Area(s)\nRecognize the clinical findings associated with epidural hematoma, and manage appropriately\nRecognize the clinical findings associated with subdural hematoma with and without skull fracture, and manage appropriately\nThe correct answer is: epidural hematoma"}
{"id" : 2092, "question_text" : "A 10-year-old girl is brought by her parents for a health supervision visit. The parents ask for a few minutes to speak to the pediatrician alone. In private, the parents disclose that they will be finalizing their divorce after a 6 month separation. Since her parents separated, the girl's grades have declined, and she is acting out more at home when she does not get her way. Her parents have not agreed on how to discipline the girl or address her concerns during this time. The mother is considering moving so that the girl can attend a new school and \"make a new start.\" Of the following, the BEST recommendation to offer the parents is", "options" : "[\"advise them both to listen to the girl's concerns and discuss the emotions she is feeling\", \"recommend that both parents be more flexible with rules and routines\", \"suggest that the parents ask the girl her preference for living arrangements\", \"support the mother's plan to move and change the girl's school\"]", "explanation" : "PREP Pearl(s)\nThe existence of close and secure relationships with adults helps support children's adjustment during major life transitions.\nMaintaining routines, rules, and parental expectations through times of transition provides children with stability.\nChildren should be allowed to express their concerns and encouraged to discuss their emotions when working through a life transition.\nCritique\nThe best recommendation is to advise both parents to listen to their daughter's concerns and discuss the emotions she is feeling. Major life transitions for children can include divorce, loss of a parent or death in the family, moving to a new location, starting school, and the birth of a sibling. While each of these events comes with its own unique set of challenges, there are ways that parents and caregivers can help their child adjust. The types of adjustment support will depend on the child's age, their temperament, and the strength of existing relationships. For school-age children, parents and caregivers should be straightforward when discussing the situation; they should listen to the child's concerns and discuss the emotions that the child is feeling. When possible, parents should allow their child time to prepare and process the change that is coming. For the girl in the vignette, this might have included discussion of the possibility of divorce at the time of her parents separation.\nHaving the girl change schools would not be recommended. When a child is going through a major life transition, routines and consistency are important. Moving to a new school and community may create additional stressors during an already difficult time. Keeping to a schedule, including mealtimes and bedtime, can provide a sense of security. It would not be recommended that the parents change their parenting techniques or level of discipline. The girl's behavior at home and at school should be addressed, and appropriate support should be provided, including counseling. It is important to recognize the influence a child's temperament has on their adjustment to a major transition. Some children will have a more intense emotional reaction than others, or need more time to process the changes that are taking place.\nThe existence of close supportive relationships with adults directly impacts how a child will handle a major transition; the child will depend on those adults to support them through the transition and provide security and comfort. Asking the girl to pick who she wants to live with may strain her relationship with her parents, as well as increase the tension between her parents. A child's exposure to parental conflict is predictive of poor emotional adjustment to a transition. It is important for pediatricians caring for a child whose parents are going through a divorce to remain objective and not take sides, in order to provide the best support for the family.\nSuggested Reading(s)\nArthur JD. Helping children cope with divorce, death, and deployment. Pediatr Rev. 2020;41(2):93–95. doi:10.1542/pir.2018-0215\nCohen G, Weitzman C, Yogman M, et al; Committee on Psychosocial Aspects of Child and Family Health, Section on Developmental and Behavioral Pediatrics. Helping children and families deal with divorce and separation. Pediatrics. 2016;138(6):e20163020. doi:10.1542/peds.2016-3020\nKleinsorge C, Covitz LM. Impact of divorce on children: developmental considerations. Pediatr Rev. 2012;33(4):147–155. doi:10.1542/pir.33-4-147\nUS Department of Human Health and Services. Supporting transitions: using child development as a guide. Early Head Start Early Learning and Knowledge. Updated December 4, 2023. Accessed April 30, 2024. Supporting Transitions\nContent Domain\nBehavioral/Developmental\nLearning Objectives\nCounsel families regarding their childrens' adjustment to major life transitions"}
{"id" : 2268, "question_text" : "A 14-year-old with no significant medical history is seen in the clinic for prolonged and irregular menses that have been interfering with her daily life. She had menarche at age 13 years. Her menses have occurred every 2 to 3 weeks since that time, each event lasting 10 to 14 days and sometimes associated with dime-size clots. She complains of fatigue and lightheadedness on standing. Her menstrual pain is well controlled by nonsteroidal anti-inflammatory medication. The family history is significant for ovarian cysts in her mother. There is no family history of clotting disorders. Physical examination findings are unremarkable, including normal vital signs for age and normal abdominal and external genitourinary findings. Laboratory test results show: Urine beta-human chorionic gonadotropin Negative, Ferritin 12.4 ng/mL (12.40 µg/L), Hemoglobin 9.9 g/dL (99.0 g/L), Thyroid stimulating hormone 0.9 μIU/mL, Free testosterone 1.4 pg/mL (reference range 1.1-6.3 pg/mL), Prolactin 14.8 ng/mL (14.80 µg/L) (reference range 2.8-29.2 ng/mL), von Willebrand antigen normal, von Willebrand activity normal, Factor VIII normal. Transabdominal pelvic ultrasonography reveals a simple left ovarian cyst measuring 4 cm x 4.1 cm x 4.9 cm. The right ovary and uterus findings were normal, and blood flow was normal to both ovaries. Of the following, the BEST next step is", "options" : "[\"obtain pelvic computed tomography\", \"prescribe a combination oral contraceptive pill\", \"provide reassurance and follow up in 3 months\", \"refer to a gynecologist for surgical treatment\"]", "explanation" : "Ovarian cysts are common in adolescents and are typically a result of ovulation.\nPatients with simple ovarian cysts that are 5 cm in diameter or less should be prescribed ovulation suppression, and ultrasonography should be performed after 4 to 6 weeks; precautions around symptoms of ovarian torsion should be discussed.\nFor adolescents with simple ovarian cysts, ovulation suppression (with combined estrogen and progesterone contraceptive pills) or depot medroxyprogesterone may be used to prevent future physiologic cysts from developing.\nCritique\nThe patient described in the vignette has irregular (frequent) and heavy menstrual bleeding that has led to iron deficiency anemia. Imaging revealed a simple ovarian cyst (thin, smooth walls with no solid components and no internal blood flow) that is less than 5 cm in diameter. She has no symptoms that suggest ovarian torsion. The best next step in her management is to prescribe a monophasic combination estrogen/progestin oral contraceptive pill to both suppress ovulation (decreasing the risk of development of additional cysts) and treat her heavy menstrual bleeding. This patient should also receive oral iron supplementation.\nAdolescents commonly have simple ovarian cysts that resolve spontaneously over 6 to 8 weeks. While reassurance regarding resolution of the cyst is appropriate, follow up in 3 months without treatment would not be the best next step. She should be prescribed medication to provide ovulation suppression (eg, combined oral contraceptive pills, depot medroxyprogesterone) and iron supplementation to treat her iron deficiency anemia. Transvaginal ultrasonography may be helpful in the evaluation of adnexal masses in adolescents who have been sexually active; computed tomography is not recommended for the initial evaluation. Referral to a gynecologist may be necessary if the cyst is persistent or enlarging on repeat ultrasonography after 6 weeks, or if new symptoms arise. Surgical treatment (eg, cystectomy) is not recommended in this case, since there is no concern for ovarian torsion and the diameter of the cyst is less than 5 cm.\nOvarian cysts occur commonly in postmenarchal and premenopausal women as a result of normal ovulation. Simple cysts have no solid or papillary components; they are typically less than 5 cm in diameter. The most common type of ovarian cyst is a follicular cyst, which results from a lack of ovulation and involution of the follicle. Follicular cysts are commonly 2 to 3 cm in diameter. Corpus luteum cysts occur after ovulation and can be 5 to 12 cm in diameter.\nSimple cysts are almost always benign, even those up to 10 cm in diameter. There is a greater concern for neoplasm if cysts greater than 5 cm in diameter persist after 6 to 8 weeks despite ovulation suppression, are increasing in size, or have solid components on imaging. The most common benign ovarian neoplasm seen in adolescents is a benign teratoma. The most common ovarian malignancy seen in children and adolescents is a germ cell tumor. If a germ cell tumor is suspected, alpha fetoprotein, human chorionic gonadotropin, and lactate dehydrogenase levels should be obtained.\nAn adolescent diagnosed with ovarian cysts should be counseled on ovarian torsion precautions, including the importance of seeking medical care immediately if they develop sudden onset intermittent and non-radiating abdominal pain, nausea, or vomiting. The risk of torsion increases for ovarian masses greater than 5 cm in diameter. Pelvic ultrasonography should be repeated in 6 weeks. At every encounter, the practitioner should interview the adolescent privately, obtain a sexual history, and perform testing for pregnancy and sexually transmitted illnesses, if indicated. Ovulation suppression with monophasic combination estrogen/progestin oral contraceptive pills or depot medroxyprogesterone can prevent development of new physiologic cysts, but will not treat current cysts. Oral contraceptive pills with 35 μg of ethinyl estradiol are more effective for ovulation suppression than those with 20 μg.\nSuggested Reading(s)\nAdnexal torsion in adolescents. Committee Opinion No. 783. American College of Obstetricians and Gynecologists. Obstet Gynecol. 2019;134(2):e56-e63. doi:10.1097/AOG.0000000000003373\nEskander R, Berman M, Keder L. Committee on Practice Bulletins. Evaluation and management of adnexal masses. Practice Bulletin No. 174. American College of Obstetricians and Gynecologists. Obstet Gynecol. 2016;128:e210–26. DOI:10.1097/AOG.0000000000001768\nContent Domain\nReproductive Health, Ovarian cysts and torsion\nLearning Objectives\nPlan the care for an adolescent with small simple ovarian cysts"}
{"id" : 2870, "question_text" : "A 15-year-old adolescent girl is evaluated in the office for a 2-month history of chronic nonproductive cough associated with fatigue, 5- to 10-lb weight loss, low-grade fever, and mildly painful nodules on her arms and legs. She has not traveled out of the state or had any known sick exposures. On pulse oximetry, her oxygen saturation is 98% on room air. On physical examination, the girl has no respiratory distress, and no crackles or wheezes are heard. She has several 1- to 3-cm diameter, tender, nonerythematous, nonexcoriated nodules on the extensor surfaces of both arms and legs. Chest radiography suggests hilar adenopathy. Computed tomography additionally demonstrates interstitial changes in the pulmonary parenchyma. Pulmonary function testing shows a mild restrictive pattern with a decrease in forced vital capacity and total lung capacity. Carbon monoxide diffusion is slightly decreased for age and hemoglobin concentration. A tuberculin skin test has a negative result 60 hours after placement. Of the following, the BEST next step in the evaluation of this adolescent is", "options" : "[\"biopsy of a skin nodule\", \"bronchoscopy and bronchoalveolar lavage\", \"repeat spirometry after administration of albuterol\", \"6-minute walk test\"]", "explanation" : "Correct Answer: A\nThe girl in the vignette likely has sarcoidosis, with both pulmonary and nonpulmonary manifestations. The presence of cough and fatigue with hilar adenopathy and restrictive lung disease may be nonspecific, but for this adolescent with tender peripheral nodules, sarcoidosis is the most likely diagnosis. Of the response choices listed, the most appropriate next step in evaluation is biopsy of one of the nodules to demonstrate noncaseating granulomas. Bronchoscopy will not produce a diagnosis, though the bronchoalveolar lavage findings may show lymphocytosis with a reduced number of CD8 lymphocytes. However, this is not specific for sarcoidosis and not necessary for the diagnosis.\n\nBronchodilator responsiveness on pulmonary function testing would be most helpful in a patient with an obstructive rather than restrictive pattern of findings. The pathophysiology of sarcoidosis does not involve airway reactivity, rather, interstitial inflammation can be seen, which is not sensitive to bronchodilators. The girl in the vignette likely would have reduced endurance in a 6-minute walk test, but that finding is nonspecific and would not help determine her diagnosis.\n\nSarcoidosis is a multisystem disease that usually presents between 20 and 60 years of age. Sarcoidosis is 4 times more common in black than white populations, and more common in women than men. Black women are most commonly affected; they may develop disease up to 10 years earlier than white patients. It is estimated that the lifetime risk for sarcoidosis in blacks in the United States is 2.4% compared with a lifetime risk of 0.85% in whites. The most specific diagnostic test for sarcoidosis is biopsy of an affected organ; for the adolescent in the vignette, biopsy of a skin nodule is the least invasive approach to obtaining a tissue diagnosis. Biopsy of a hilar lymph node would be a more invasive approach than necessary.\n\nSarcoidosis can affect all organ systems; the lungs are involved in most cases. Extrapulmonary sites may be affected exclusively or may be involved before pulmonary findings. After the lungs, the skin, eyes, and lymph nodes are the most commonly involved organs. The liver, spleen, parotid glands, and bone marrow may also be sites of noncaseating granulomas characteristic of sarcoidosis.\n\nPREP Pearls\n• Sarcoidosis is diagnosed based on the presence of noncaseating granulomas in affected tissues.\n• The lungs, skin, eyes, and lymph nodes are the organs most commonly affected by sarcoidosis.\n• Sarcoidosis is 4 times more common in black than white populations, and more common in women than men.\n\nABP Content Specifications(s)\n• Recognize the clinical findings associated with sarcoidosis\n\nSuggested Readings\n• Iannuzzi MC, Rybicki BA, Teirstein AS. Sarcoidosis. N Engl J Med. 2007;357:2153-2165. doi: 10.1056/NEJMra071714.\n• Nathan N, Marcelo P, Houdouin V, et al. Lung sarcoidosis in children: update on disease expression and management. Thorax. 2015;70:537-542. doi: 10.1136/thoraxjnl-2015-206825.\n• Shetty AD, Gedalia A. Sarcoidosis in children. Curr Prob Pediatr. 2000;30:149-176. doi: 10.1067/mps.2000.105929.\n• Sileo C, Epaud R, Mahloul M, et al. Sarcoidosis in children: HRCT findings and correlation with pulmonary function tests. Pediatr Pulmonol. 2014;49:1223. doi: 10.1002/ppul.22956."}
{"id" : 2984, "question_text" : "A 14-year-old adolescent boy is brought to the emergency department for evaluation of difficult and painful swallowing. His symptoms began 5 hours ago after he ate ramen noodles and chicken sausage. He noticed a feeling that something \"was stuck\" in his chest; he has been retching and vomiting since then. He has a 2-year history of dysphagia but has never experienced symptoms to this degree. He has a history of multiple environmental allergies, for which he receives immunotherapy, and a history of anaphylaxis to eggs. On physical examination, the boy is uncomfortable appearing and frequently retching and spitting clear liquid into the emesis basin near his bed. His examination findings are otherwise normal. Chest radiograph is normal. Of the following, the BEST next management step for this adolescent is", "options" : "[\"abdominal ultrasonography\", \"endoscopy\", \"epinephrine injection\", \"esophagography\"]", "explanation" : "Correct Answer: B\nThe adolescent in the vignette has acute esophageal obstruction, evidenced by chest pain, retching, and vomiting. He is unable to tolerate oral secretions based on the frequent spitting of clear liquid observed during the physical examination. Acute esophageal obstruction necessitates endoscopic management to remove the foreign body (in this case, likely food, given his history and negative chest radiography findings). During this boy's endoscopic evaluation, the food bolus was removed; findings concerning for eosinophilic esophagitis, such as linear ridging and white plaques, were seen (Item C186).\n\nEosinophilic esophagitis (EoE) is an immune-mediated inflammatory condition of the esophagus, most commonly associated with non-IgE allergic response to food allergens (including milk, egg, wheat, soy, nuts/peanuts, and fish/shellfish). Eosinophilic esophagitis often presents with dysphagia and/or chronic reflux-like symptoms in older children. It is not uncommon for older children to have chronic dysphagia to which they have acclimated by chewing and eating slowly, cutting foods (meats in particular) into very small bites, and drinking significant amounts of liquids while eating. A sensation of \"food sticking\" in the chest while eating may be reported. The initial presentation may be an acute esophageal obstruction, as seen in the vignette. Up to 88% of children with food impaction requiring therapeutic endoscopy have EoE. Younger children with EoE often have more generalized symptoms, including vomiting, feeding difficulties, and even failure to thrive. A history of atopy is common in children with EoE.\n\nHistologically EoE is defined as 15 or more eosinophils present in an esophageal biopsy sample, after excluding other esophageal pathologies (generally gastroesophageal reflux disease [GERD]). To decrease the risk that GERD will be misdiagnosed as EoE, children with suspected EoE should be treated with a proton pump inhibitor for a minimum of 6 to 8 weeks before diagnostic endoscopy. Following removal of the food impaction, the adolescent in the vignette underwent proton pump inhibitor therapy for 12 weeks and then underwent repeat endoscopy, which confirmed the suspected diagnosis of EoE.\n\nManagement of EoE can include dietary and/or medical therapies and should involve a multidisciplinary team including a gastroenterologist, allergist, and dietitian. Dietary modification includes removal of the foods most commonly associated with EoE. Unfortunately, traditional food allergy testing does not always correlate with the offending food antigen in EoE. Thus, empiric 3-, 4-, or 6-food elimination diets, or guided food elimination diets in conjunction with an allergist, may be prescribed. Endoscopic surveillance is used to ensure histologic remission, because clinical improvement in symptoms does not always correlate with histologic remission. It is important to note that even the 3-food elimination diet (milk-, egg-, and wheat-free diet) may be difficult to follow, and it is critical to involve a dietitian. Medical management of EoE may include the use of swallowed topical steroids (fluticasone or oral viscous budesonide) and proton pump inhibitors which may be used alone or in conjunction with dietary modification. Rarely, children may not respond to elimination diets and/or medical therapies; in such cases, an exclusive amino acid–based elemental formula may be necessary to induce remission (administered orally, via nasogastric, or gastrostomy tube).\n\nAbdominal ultrasonography would not be useful for the adolescent in this vignette. Because this adolescent is not experiencing symptoms of anaphylaxis, epinephrine injection is not indicated. Esophagography may be useful when there is a possible or partial esophageal obstruction (vomiting, difficulty swallowing), but when the obstruction is complete (when the patient is unable to swallow oral secretions), a contrast study may pose additional aspiration risk and could coat the foreign body and esophageal mucosa, compromising the necessary endoscopy.\n\nPREP Pearls\n• Esophageal obstruction presents clinically with chest pain, dysphagia, and the inability to swallow fluids or oral secretions.\n• Emergent consultation with a pediatric gastroenterologist and a therapeutic endoscopy are indicated for esophageal obstruction.\n• Older children with esophageal food impaction are likely to have eosinophilic esophagitis.\n\nMOCA-Peds Objective\n• Manage foreign body ingestion.\n\nABP Content Specifications(s)\n• Recognize the clinical features associated with eosinophilic or allergic esophagitis\n\nSuggested Readings\n• Adamiak T, Plati KF. Pediatric esophageal disorders: diagnosis and treatment of reflux and eosinophilic esophagitis. Pediatr Rev. 2018;39(8):392-402. doi: 10.1542/pir.2017-0266.\n• Le-Carlson M, Kerner JA. Gastrointestinal allergy. In: McInerny TK, Adam HM, Campbell DE, DeWitt TG, Foy JM, Kamat DM, eds. American Academy of Pediatrics Textbook of Pediatric Care. 2nd ed. Itasca, IL: American Academy of Pediatrics; 2016; chap 256:2076-2080. Pediatric Care Online."}
{"id" : 761, "question_text" : "A 9-year-old girl presents with symptoms of an itchy, raised rash. The rash started 2 weeks ago, but she has had similar episodes intermittently for the previous 4 months. The rash consists of multiple erythematous, slightly elevated lesions ranging in size from a dime to a golf ball (Item Q53). The lesions resolve in a few hours without bruising or discoloration. Diphenhydramine as needed and daily cetirizine help minimize the frequency and severity of her symptoms, but she still gets breakthrough episodes. Her parents have not found any association of the eruption with foods, cosmetics, or medications. At times, the rash occurs after the girl becomes hot and sweaty. The parents have heard that food allergies might be a trigger for their daughter's episodes and wonder if she should undergo testing. Of the following, the MOST appropriate response is to", "options" : "[\"order allergen- specific IgE tests to milk, egg, soy, wheat, fish, shellfish, peanuts, nuts, and food additives\", \"order allergen- specific IgG4 tests to milk, egg, soy, wheat, fish, shellfish, peanuts, nuts, and food additives\", \"reassure the parents that allergy testing is not needed; an undiscovered allergy to foods or food additives is unlikely to be the cause\", \"recommend an elimination diet of home-cooked rice or oats, chicken or turkey, and vegetables for a trial period of 2 weeks\", \"refer them to an allergist for skin testing to milk, egg, soy, wheat, fish, shellfish, peanuts, nuts, and food additives\"]", "explanation" : "The child described in the vignette is unlikely to have a new-onset allergy to food/food additives as the cause of the chronic urticaria (CU). Chronic urticaria is defined by the presence of urticaria (hives) on most days of the week for a period of 6 weeks or longer. While acute urticaria and angioedema may be manifestations of IgE-mediated allergic reactions, no external cause can be identified in 80% to 90% of people affected by CU. While milk, egg, soy, wheat, fish, shellfish, peanuts, and nuts are the most common triggers of food-allergic reactions, IgE-mediated reactions to foods and food additives are not a cause of CU and allergy testing is not warranted. Nonstandardized tests such as allergen-specific IgG4 are not recommended for the routine evaluation of IgE mediated food allergic disorders and do not have a role in the evaluation of chronic urticaria.\n\nPatients with CU may perceive food-associated reactions; but their perceptions have not been validated in studies using placebo-controlled challenges. They may also often report that rich meals, fermented foods, and alcohol worsen the condition transiently. This may be related to the histamine content or innate histamine-releasing properties of these foods, as well as the vasodilatory effects of alcohol and certain spices. Food additives are rarely, if ever, confirmed to cause or contribute to flares of CU in carefully performed studies. While temporary avoidance of the offending foods discussed above may be considered, elimination diets have not been shown to help and are not recommended.\n\nNevertheless, every effort should be made to determine the etiology of these symptoms. A detailed history should be taken and repeated periodically if the CU persists. The history should be geared toward (1) differentiating between vasculitic (duration of lesions >72 hours, pain, discoloration, or residual scarring) and nonvasculitic urticaria (duration <72 hours and absence of above signs), (2) identifying possible underlying cause, and (3) ensuring that the patient does not have evidence of a more serious systemic disease (fever, weight loss, arthralgias, and other constitutional symptoms). It is helpful to approach patients on the basis of broad categories of mechanisms such as IgE-dependent mechanisms (eg, drug, food, insect venom, and latex exposure) and complement-mediated mechanisms (eg, hereditary angioedema and serum sickness). Other factors to consider include (1) physical urticarias; (2) underlying infection; (3) autoimmune etiology; (4) possible hormonal effects, especially when hives in women occur on a cyclic basis; (5) association with malignant tumor; (6) pertinent occupational exposure; (7) multiple/repetitive or late onset reactions to insect stings/bites; (8) direct contact of skin or oropharynx with foods, chemicals, animal saliva, and other substances; (9) familial pattern/ hereditary syndromes; and (10) psychologic stresses.\n\nInitial screening tests for disorders most commonly associated with urticaria include: a complete blood count with differential, markers of inflammation (C-reactive protein or erythrocyte sedimentation rate), liver function tests, urine analysis, and a thyroid-stimulating hormone level. Further testing should be based upon the results of these tests or as dictated by the history, physical examination, and review of systems. Biopsy of a fresh lesion may be indicated if urticaria) vasculitis is suspected. Skin or serum-specific IgE allergy testing is typically not indicated unless a specific trigger is suspected on the basis of history. At this stage of the evaluation it is reasonable to define chronic urticaria angioedema as idiopathic because this is a diagnosis by exclusion of underlying etiologies. If treatment is ineffective at this point, referral to an allergist/ immunologist or dermatologist might be considered.\n\nPREP Pearls\n• Chronic urticaria is defined by the presence of hives on most days of the week for a period of 6 weeks or longer.\n• Typically, unless a specific trigger is suspected, chronic urticaria does not warrant allergy testing.\n\nAmerican Board of Pediatrics Content Specification (s):\n• Recognize that chronic urticaria does not warrant allergy testing\n\nSuggested Reading:\n• Joint Task Force on Practice Parameters. The diagnosis and management of urticaria: a practice parameter, part II: chronic urticaria/angioedema. Ann Allergy Asthma Immunol. 2000; 85(6 pt 2):S521-S544\n• Khan DA. Chronic urticaria: diagnosis and management. Allergy Asthma Proc. 2008;29(5):439-446. doi:10.2500/aap.2008.29.3151\n• NIAID-Sponsored Expert Panel; Boyce JA, Assa'ad A, Burks AW, et\n• al. Guidelines for the diagnosis and management of food allergy in the United States: report of the NIAID-Sponsored Expert Panel. I Allergy Clin Immunol. 2010;126(6 suppt):SI-S58. doi:10.1016/j.jaci.2010.10.007"}
{"id" : 1744, "question_text" : "An 18-month-old girl presents to the emergency department with diarrhea and vomiting. Over the past several days, she has vomited approximately 5 times per day, and has had 8 to 10 stools per day. She refuses to eat solids, but has been drinking fluids adequately, including milk, juice, and water. She continues to urinate approximately 5 times per day. Her vital signs are as follows: temperature, 37.8°C; heart rate, 150 beats/min; respiratory rate, 40 breaths/min; and blood pressure, 90/50 mm Hg. On physical examination, she is awake and alert, and has slightly dry mucous membranes. She is warm and well-perfused, with a capillary refill time of 2 seconds. She is breathing slightly rapidly and deeply. Her lungs are clear to auscultation bilaterally. Laboratory results show sodium 130 mEq/L, potassium 5.0 mEq/L, chloride 108 mEq/L, bicarbonate 14 mEq/L, serum urea nitrogen 20 mg/dL, creatinine 0.3 mg/dL, glucose 80 mg/dL, capillary blood gas pH 7.25, and partial pressure of carbon dioxide 28 mm Hg. Of the following, the MOST likely contributor to this girl's acidemia is", "options" : "[\"decreased renal excretion of hydrogen ion\", \"end-organ production of lactic acid\", \"gastric production of hydrochloric acid\", \"intestinal losses of bicarbonate\", \"respiratory accumulation of carbon dioxide\"]", "explanation" : "The girl in the vignette has metabolic acidosis caused by acute gastroenteritis with appropriate respiratory compensation. Evidenced by a normal anion gap, the most important contributor to her acidemia is intestinal losses of bicarbonate.\n\nAcute gastroenteritis is a very common cause of emergency department visits for children. Denuding of epithelium and dysfunction of small intestinal villi can result in malabsorption, and if severe, can impair intestinal absorption of bicarbonate causing metabolic acidosis. Electrolyte and serum bicarbonate levels can provide useful information about the severity of illness and guide management. Children with bicarbonate levels higher than 15 mEq/L (15 mmol/L) are unlikely to be more than 10% dehydrated, and those with bicarbonate levels less than 14 mEq/L (14 mmol/L) are less likely to tolerate an oral fluid challenge. Other electrolyte disturbances seen in acute gastroenteritis can include hypernatremia, hyponatremia, and hypokalemia.\n\nBlood gas analysis can provide helpful information in cases of severe gastroenteritis and other causes of acid-base imbalances. It is important to characterize both the respiratory and metabolic contributions to acidosis or alkalosis, if present. This can be accomplished by evaluating the PH, Pᴄᴏ2, and the calculated bicarbonate level from blood gas data. The normal serum bicarbonate level is 22 to 26 mEq/L (22–26 mmol/L); a value below or above this range constitutes metabolic acidosis or alkalosis, respectively. In cases of metabolic acidosis, such as with the girl in this vignette, it is important to determine whether the anion gap is increased or normal. The anion gap is the mathematical difference between serum values of the predominantly measured cations (sodium and potassium) and the predominantly measured anions (chloride and bicarbonate). The normal anion gap is between 3 and 11 mEq/L. Because serum is electroneutral, an elevated anion gap indicates the presence of unmeasured anions in the serum. The possibilities for the identity of these unmeasured anions comprise the differential diagnosis of an elevated anion gap metabolic acidosis, which is represented by the mnemonic \"MUDPILES\": methanol, uremia, diabetic ketoacidosis, paraldehyde, iron/isoniazid, lactate, ethanol/ethylene glycol, and salicylates. A more broad differential diagnosis includes toxic ingestions, lactic acidosis, renal failure, and ketoacidosis. In contrast, normal anion gap metabolic acidosis is not caused by unmeasured anions. Rather, it is caused by the loss of bicarbonate, either from the urine or the gastrointestinal tract, most commonly because of renal tubular acidosis or gastroenteritis.\n\nIn cases of metabolic acidosis, it is important to recognize whether there is a concomitant respiratory alkalosis or acidosis. Appropriate respiratory compensation for a metabolic acidosis can be assessed by using the Winters formula:\n\nExpected Pᴄᴏ2 = 1.5 × (Serum bicarbonate) + 8 ± 2\n\nIf the Pco2 is above or below the expected level, there is a concomitant respiratory acidosis or alkalosis, respectively. It should be noted that appropriate respiratory compensation does not usually restore the PH to the normal level, and mathematically, the Pco2 can be lower than the reference range but still be classified as respiratory acidosis (ie, inadequate compensation). The girl in the vignette has a normal anion gap metabolic acidosis with appropriate respiratory compensation, therefore respiratory accumulation of carbon dioxide is not the correct answer. The etiology of her acidosis is intestinal losses of bicarbonate, not increased gastric production of hydrochloric acid. Although lactic acidosis can occur with hypovolemic shock from severe gastroenteritis, it usually presents with an elevated anion gap. In response to acidosis, there is increased reabsorption of bicarbonate by the kidneys as opposed to decreased renal excretion of hydrogen ion.\n\nPREP Pearls\n• The anion gap is the mathematical difference between serum values of the predominantly measured cations (sodium and potassium) and the predominantly measured anions (chloride and bicarbonate).\n• Normal anion gap metabolic acidosis is caused by the loss of bicarbonate from the urine or stool.\n• Elevated anion gap acidosis is caused by lactic acidosis, toxic ingestions, ketoacidosis, or renal failure.\n\nMOCA-Peds Objective\n• Evaluate and manage a patient with metabolic acidosis\n\nABP Content Specifications(s)\n• Formulate a differential diagnosis of acidosis associated with various anion gap values\n• Identify the arterial blood gas abnormalities associated with an acid-base imbalance\n\nSuggested Readings\n• Kappy MS, Morrow G. A diagnostic approach to metabolic acidosis in children. Pediatrics. 1980;65(2):351–356. http://pediatrics.aappublications.org/content/65/2/351.\n• Nagler J, Wright RO, Krauss B. End-tidal carbon dioxide as a measure of acidosis among children with gastroenteritis. Pediatrics. 2006;118:260–267. doi: http://dx.doi.org/10.1542/peds.2005-2723.\n• Schwaderer AL, Schwartz GJ. Back to basics: acidosis and alkalosis. Pediatr Rev. 2004;25:350. http://pedsinreview.aappublications.org/content/25/10/350."}
{"id" : 754, "question_text" : "During morning rounds, the nurse informs you that a 3.8-kg neonate has just been delivered by caesarean section due to breech presentation. The pregnancy was notable for well-controlled maternal type I diabetes. The maternal glycated hemoglobin A,, values ranged between 5% to 6% before conception and during pregnancy. Level II screening ultrasonography done at 18 weeks of gestation was normal. Your assessment at 40 minutes after birth reveals a pink, well-perfused neonate with normal tone, strong suck, and good color. Cardiac examination reveals a grade 1/6 systolic murmur at the left lower sternal border with a preductal oxygen saturation of 97% on room air. The mother is anxious to breastfeed. Of the following, the MOST appropriate next step in management is to immediately", "options" : "[\"determine blood glucose concentration\", \"initiate breastfeeding\", \"obtain a chest radiograph\", \"perform an echocardiogram\", \"supplement with formula\"]", "explanation" : "An infant of a diabetic mother (IDM) who has no symptoms of hypoglycemia should be breastfed within the first hour after birth. The Committee on Fetus and Newborn of the American Academy of Pediatrics published a clinical report in 2011 outlining screening and management strategies for hypoglycemia in the late-preterm and full-term infant (Item C46). Early feeding, within the first hour after birth, with a screening glucose value obtained 30 minutes after the first feeding, is the first step in the management of the asymptomatic infant described in this vignette. Glucose concentrations normally decline in the first 1 to 2 hours after birth following the discontinuation of maternal glucose influx with umbilical cord clamping. Plasma glucose values as low as 30 mg/dL (1.7 mmol/L) may be seen transiently during this period. The plasma glucose concentrations then routinely increase above 45 mg/dL (2.5 mmol/L) by 12 hours after birth. Infants at risk for neonatal hypoglycemia, including late-preterm, small for gestational age, large for gestational age, and IDM, should be monitored closely during this period for clinical symptoms of hypoglycemia. These signs include jitteriness, tremors, high-pitched cry, irritability, lethargy, floppiness, poor suck, apnea, cyanosis, exaggerated Moro reflex, and seizure activity. Screening glucose values should be obtained in infants with symptoms and intravenous glucose initiated if the value is less than 40 mg/dL (2.2 mmol/L).\n\nMaternal hyperglycemia is hypothesized to produce hyperglycemia in the developing fetus, leading to fetal pancreatic stimulation, islet cell hyperplasia, and hyperinsulinemia in the IDM. While contributing to the hypoglycemia seen in the IDM at birth, the elevated insulin levels also cause increased growth in insulin-sensitive tissues such as the heart, liver, and muscle leading to fetal macrosomia. The cardiomyopathies associated with IDMs, which include thickening of the intraventricular septum and ventricular walls, are believed to be related to the direct effect of fetal insulin on cardiac muscle growth. Fetal hyperinsulinemia is also linked to decreased surfactant production, with respiratory distress syndrome (RDS) more common in IDMs. Well-controlled maternal diabetes in pregnancy is associated with less severe hypoglycemia and a decreased risk of RDS in the IDM, but no effect has been reported on macrosomia. Polycythemia and hypocalcemia are associated with IDMs, but the mechanisms underlying these findings are not fully known.\n\nPreconception counseling and optimization of metabolic control is essential for women with type 1 diabetes who are considering pregnancy. Well-managed maternal diabetes before conception has been demonstrated to decrease the frequency of congenital anomalies associated with the IDM including congenital heart disease (transposition of the great vessels, ventricular septal defect), caudal regression syndrome, and neural tube defects (anencephaly, spina bifida). In spite of preconceptual management, up to 5% of IDMs may have small left colon syndrome.\n\nThe IDM in the vignette is at risk for hypoglycemia. Because the infant is asymptomatic, he should be allowed to breastfeed within 1 hour of birth with a screening glucose value obtained 30 minutes after feeding. The infant does not require formula supplementation at this time, with further glucose management tailored to meet the needs of the infant while supporting the mother-infant dyad and breastfeeding. A low rapid bedside screening glucose value should be confirmed in the laboratory by determining the serum glucose concentration, but treatment should not be delayed while awaiting the results. Although IDMs are at an increased risk for respiratory problems at birth, the infant in the vignette is not demonstrating any respiratory symptoms and does not require chest radiography immediately. If the murmur persists or respiratory distress develops, a chest radiograph and/or echocardiogram can be obtained to screen for cardiomyopathy.\n\nPREP Pearls\n• An infant of a diabetic mother who has no symptoms of hypoglycemia should be fed, preferably by breast, within the first hour after birth and undergo a screening glucose measurement 30 minutes after the feeding.\n\nAmerican Board of Pediatrics Content Specification (s):\n• Understand the management of a newborn whose mother has type 1 diabetes\n\nSuggested Reading:\n• American Academy of Pediatrics Committee on Fetus and Newborn. Postnatal glucose homeostasis in late-preterm and term infants. Pediatrics. 2011;127:575-579. doi: 10.1542/peds.2010-3851\n• Carlo W. Infants of diabetic mothers. In: Kleigman RM, Stanton BF St Genre JW III, Schor NF, Behrman RE, eds. Nelson Textbook of Pediatrics. 19th ed. Philadelphia, PA: Saunders Elsevier; 2011:627-629\n• Ogata ES. Problems of the infant of the diabetic mother. NeoReviews. 2010;11:e627-e631. doi: 10.1542/neo.11-11-e627\n• Srinivason G, Pildes RS, Cattamanchi G, Voora S, Lilien LD. Plasma glucose values in normal neonates: a new look. I Pediatr. 1986;109:114-117"}
{"id" : 2365, "question_text" : "An 8-year-old boy is seen for a health supervision visit. His developmental milestones were delayed for motor skills, and he required physical therapy till 4 years of age. Physical examination findings are remarkable only for macrocephaly, short stature, and rhizomelic (proximal) shortening of extremities. The family history is significant for his mother and maternal family members with similar findings; the family pedigree is shown in the Figure. Of the following, the inheritance pattern seen in the boy's family is", "options" : "[\"autosomal dominant\", \"autosomal recessive\", \"mitochondrial\", \"X-linked dominant\"]", "explanation" : "PREP Pearl(s)\nAchondroplasia is a skeletal dysplasia inherited in an autosomal dominant pattern.\nClinical features of achondroplasia include disproportionate short stature, rhizomelic shortening of extremities, macrocephaly, motor delay, frontal bossing with midface retrusion, bowed legs, and trident configuration of the hands.\nComplications seen in achondroplasia include middle ear dysfunction, spinal stenosis, obstructive sleep apnea, obstructive hydrocephalus (secondary to craniocervical junction compression), and kyphosis.\nCritique\nThe boy and his affected family members have achondroplasia, which is inherited in an autosomal dominant pattern. Achondroplasia is a skeletal dysplasia characterized by the following:\nDisproportionate short stature: rhizomelic (proximal) shortening of extremities\nBowed legs, trident configuration of the hands\nMacrocephaly\nFacial features: frontal bossing, midface retrusion\nDevelopment: motor delay secondary to hypotonia\nNormal intelligence\nComplications: Kyphosis, Middle ear dysfunction, Obstructive sleep apnea, Obstructive hydrocephalus (secondary to craniocervical junction compression), Spinal stenosis\nThe different inheritance patterns are outlined in the Table.\nAn autosomal recessive inheritance pattern would be incorrect for the child in the vignette, as multiple family members are affected across multiple generations and all affected individuals have inherited the disorder from their affected parent. Mitochondrial inheritance would be incorrect as mitochondrial conditions are maternally inherited and there is transmission from males shown in this family's pedigree (Note: There are rare occurrences of mitochondrial disorder transmission from an affected father reported in the literature). An X-linked inheritance pattern would not explain the male-to-male transmission seen in this boy's disorder.\nSuggested Reading(s)\nLegare JM, ed. Achondroplasia. GeneReviews [Internet]. University of Washington, Seattle; 2022. Accessed September 1, 2023. https://www.ncbi.nlm.nih.gov/books/NBK1152/\nOverview of genetics. In: American Academy of Pediatrics Committee on Genetics; Saul RA, eds. Medical Genetics in Pediatric Practice. American Academy of Pediatrics; 2014:chap 1.\nPatterns of single-gene inheritance.. In: Willard HF, Nussbaum RL, McInnes RR, eds. Thompson and Thompson Genetics in Medicine. 8th ed. Elsevier; 2016:107-132.\nContent Domain\nGenetics\nABP Content Specification(s) / Content Area(s)\nRecognize the inheritance pattern of achondroplasia"}
{"id" : 1385, "question_text" : "You are called to the newborn nursery to evaluate a 3-day-old female newborn with worsening neurologic status. The full-term newborn was delivered via repeat cesarean delivery after an unremarkable pregnancy. She was initially doing well after birth. At approximately 12 to 24 hours of age, the mother noted a sweet, caramel-like odor. At 2 days of age, the newborn was feeding poorly, becoming irritable, and then developed drowsiness that progressed to lethargy, intermittent apnea, opisthotonus, and hypertonia. On day 3 after birth, the newborn developed \"bicycling\" movements of the legs. On physical examination, the newborn appears mildly dehydrated, lethargic, and hypertonic. Initial laboratory workup shows mild metabolic acidosis (pH = 7.3) and a blood ammonia level of 280 μg/dL (200 μmol/L). A complete blood cell count, lactate, calcium, and glucose are normal. A sepsis workup is initiated with cultures pending. You suspect an inborn error of metabolism. Of the following, the BEST next test to confirm your suspected diagnosis is", "options" : "[\"biotinidase level\", \"ceruloplasmin level\", \"lysosomal enzyme screen\", \"serum amino acids\", \"very-long-chain fatty acids\"]", "explanation" : "The newborn in the vignette has maple syrup urine disease (MSUD). By 12 to 24 hours of age, neonates with classic MSUD will have a maple syrup odor that is evident, especially in the cerumen. Plasma concentrations of branched-chain amino acids (isoleucine, leucine, and valine) and alloleucine will be elevated on serum amino acid analysis. Branched-chain hydroxyacids and ketoacids are evident on urine organic acid analysis. By 2 to 3 days of age, affected infants experience ketonuria, fussiness, and poor feeding. By 4 to 7 days of age, encephalopathy ensues, with opisthotonus, intermittent apnea, and lethargy progressing to respiratory failure and coma.\n\nMaple syrup urine disease is an inborn error of metabolism belonging to the subtype known as organic acidemias. Organic acidemias are characterized by the excretion of non-amino organic acids in the urine, caused by an enzymatic deficiency in specific steps involved in amino acid catabolism. Other organic acidemias include:\n• Glutaric acidemia type I\n• Isovaleric acidemia, homocystinuria\n• Methylmalonic acidemia\n• Propionic acidemia\n• 3-hydroxy-3-methylglutaryl-Coenzyme A (HMG-CoA) lyase deficiency\n\nAll are inherited in an autosomal recessive manner. Typically, newborns with these disorders appear well during the first few days after birth, with rapid decompensation to an encephalopathic state if not quickly identified. Common laboratory abnormalities include:\n• Acidosis\n• Elevated liver function tests\n• Hyperammonemia\n• Ketosis\n• Low blood glucose\n• Neutropenia\n\nRecommended laboratory tests in the setting of a suspected organic acidemia include serum amino acids, urine organic acids, ammonia level, and a plasma acylcarnitine profile. Patients have improved outcomes if the disorder is identified in the first 10 days after birth and appropriate treatment and dietary restrictions are implemented.\n\nWith the advent of newborn screening programs using tandem mass spectrometry, MSUD is commonly identified shortly after birth, thus allowing for life-saving early treatment. Management includes dietary leucine restriction, specially manufactured branched chain amino acid-free foods, supplementation with isoleucine and valine, and intermittent biochemical monitoring. Care also includes clinical evaluations by a team specializing in metabolic disorders, including a biochemical geneticist, genetic counselor, and a metabolic dietitian. These patients are at increased risk for metabolic decompensation during periods of catabolic stress, such as intercurrent illness, and may require frequent hospitalization to manage the metabolic disorder appropriately and prevent the serious complication of brain edema.\n\nBiotinidase levels screen for biotinidase deficiency. This disorder presents in young children with the slow evolution of neurologic abnormalities, including seizures, hypotonia, ataxia, developmental delays, vision problems, hearing loss, alopecia, and a skin rash.\n\nCeruloplasmin levels, in conjunction with copper levels, screen for Menkes disease. This disorder presents with a period of normal development in early infancy, followed by developmental regression, coarse, kinky hair (pili torti), and tortuosity of the carotid arteries and vasculature of the brain.\n\nMucopolysaccharidoses are identified through lysosomal enzyme screening and urine glycosaminoglycans. These disorders typically present with a slowly progressive coarsening of facial features, joint stiffness, and developmental regression.\n\nVery-long-chain fatty acids screen for peroxisomal disorders, which present with a slow progression of hypotonia, poor feeding, dysmorphic facies, seizures, hepatic dysfunction, retinal dystrophy, and sensorineural hearing loss.\n\nPREP Pearls\n• Neonates with maple syrup urine disease (MSUD) appear well for the first 1 to 2 days after birth, and then experience a rapid progression to an encephalopathic state. Symptoms typically start with ketonuria, a maple syrup odor, poor feeding, and opisthotonus.\n• Neonates with classic MSUD will have elevated plasma concentrations of branched-chain amino acids (isoleucine, leucine, and valine) and alloleucine on plasma amino acid analysis.\n• Organic acidemias are caused by enzymatic deficiencies in specific steps of amino acid catabolism.\n• Organic acidemias are inherited in an autosomal recessive pattern.\n\nABP Content Specifications(s)\n• Plan the appropriate immediate and long-term management of organic acidemias, while considering the long-term prognosis\n• Recognize the clinical features associated with organic acidemias\n\nSuggested Readings\n• Häberle J. Clinical and biochemical aspects of primary and secondary hyperammonemic disorders. Arch Biochem Biophys. 2013;536(2):101-108. doi: http://dx.doi.org/10.1016/j.abb.2013.04.009.\n• Seashore MR. The organic acidemias: an overview. GeneReviews. http://www.ncbi.nlm.nih.gov/books/NBK1134/. Updated December 22, 2009.\n• Strauss KA, Puffenberger EG, Morton DH. Maple syrup urine disease. GeneReviews.\n• van Karnebeek CD, Shevell M, Zschocke J, Moeschler JB, Stockler S. The metabolic evaluation of the child with an intellectual developmental disorder: diagnostic algorithm for identification of treatable causes and new digital resource. Mol Genet Metab. 2014;111(4):428-438. doi: http://dx.doi.org/10.1016/j.ymgme.2014.01.011."}
{"id" : 3556, "question_text" : "What is the rationale for using a multi-disciplinary approach in managing children with hyperinflammatory sepsis according to the study conclusions?", "options" : "[\"A multi-disciplinary approach is beneficial to timely recognition and safe treatment of both the underlying trigger and the cytokine storm\", \"Multi-disciplinary teams are required only for adult sepsis management\", \"A single specialist is sufficient to manage all aspects of hyperinflammatory sepsis\", \"Multi-disciplinary approaches delay diagnosis and should be avoided\"]", "explanation" : "The text concludes that in patients with hyperinflammatory sepsis/sHLH, targeting the underlying trigger plus cytokine storm may be beneficial, and that a multi-disciplinary approach is beneficial to timely recognition and safe treatment."}
{"id" : 738, "question_text" : "A 5-year-old boy complains of headache and neck pain and lies down for a nap. An hour later, his father tries to rouse him, but the boy can only mumble. He is brought to the emergency department, where on physical examination his blood pressure is 125/90 mm Hg, heart rate is 78 beats/ min, respiratory rate is 14 breaths/min, and temperature is 37.4°C. The boy briefly opens his eyes but otherwise does not respond during the examination. There is no sign of head injury, no nuchal rigidity, and no rashes. Neurologic examination shows that the pupils are round and equally reactive to light, the limbs are flaccid, the deep tendon reflexes are brisk, and the toes are upgoing on plantar stimulation. The parents report that all medications in the home are secured and deny any ingestions. Computed tomography of the head without contrast is obtained (Item Q30). Of the following, the MOST likely cause of his symptoms is", "options" : "[\"arterial ischemic stroke\", \"arteriovenous malformation\", \"brain abscess\", \"choroid plexus carcinoma\", \"vein of Galen aneurysmal malformation\"]", "explanation" : "Preferred Response: B\nThe boy in the vignette has a ruptured arteriovenous mal-formation (AVM). His slightly elevated blood pressure and coma are consistent with increased intracranial pressure. The computed tomography image shows intraventricular hemorrhage, and the ventricles are enlarged from early, non-obstructive hydrocephalus (Item C30A). As hydrocephalus progresses and intracranial pressure increases, the brain will begin to herniate. A neurosurgeon should be consulted immediately.\n\nAn AVM is an abnormally formed connection between arteries and veins. AVMs are congenital, not acquired, and are commonly found in the brain. Clinically, some AVMs can be detected by auscultation of an intracranial bruit through the orbit or an open fontanelle. Rarely, AVMs can present with high output cardiac failure because of abnormal shunting between the arterial and venous systems. On contrasted imaging, AVMs appear as a tangled clump of vessels (Item C30B). These can occur anywhere in the brain, including inside the ventricles as in this case.\n\nUnruptured AVMs can cause local brain ischemic. Because there is no capillary bed in the area of the AVM, the local brain tissue is ischemic. This can present with focal neuro-logic deficits, seizures, or headaches. Unruptured AVMs in children have a 2% rate of hemorrhage per year. When AVMs rupture, they produce a sudden, severe headache and loss of consciousness. The intracranial hemorrhage causes increasing intracranial pressure, which quickly leads to coma and death if no intervention occurs.\n\nArterial ischemic stroke typically presents with an acute focal neurologic deficit such as hemiparesis. In children, an acute ischemic stroke often presents with seizure. If the area of the stroke is large enough, consciousness can be impaired, leading to signs of increased intracranial pressure because of brain swelling. Computed tomography shows decreased attenuation in the brain parenchyma (Item C30C).\n\nA brain abscess presents with signs of infection, such as fever, altered mental status, or nuchal rigidity. Seizures can also be a presenting symptom especially if the abscess is near the cerebral cortex. Bacteremia, cardiac defects with right to left shunt, recent neurosurgery, or dental procedures are risk factors for brain abscess. Computed tomography with contrast typically shows a round, enhancing mass (Item C30D, page C-26).choroid plexus carcinoma presents with persistent headache, vomiting, and lethargy arising from gradually progressive hydrocephalus. Altered mental status worsens over days, unlike the sudden loss of consciousness resulting from a ruptured AVM. On noncontrasted computed tomography, a mass is apparent in the region of the choroid plexus. Ventricles are often enlarged, reflecting nonobstructive hydrocephalus (ItemC30E, page C-26).\n\nVein of Galen aneurysmal malformation (VGAM) is a type of arteriovenous malformation that typically presents with high output cardiac failure in the neonatal period or infancy. Hydrocephalus, signs of increased intracranial pressure, seizures, and developmental delay can be present in infants and older children. Hemorrhage on presentation is rare. On imaging the dilated vein is visible (ItemC30F, page C-26). Treatment for VGAM is endovascular embolization of the malformation and management of cardiac symptoms.\n\nPREP Pearls\n• When central nervous system arteriovenous malformations (AVMs) rupture, they cause sudden, severe headache and loss of consciousness.\n• Central nervous system AVM rupture is a neurosurgical emergency.\n\nAmerican Board of Pediatrics Content Specification(s):\n\nIdentify the clinical features of CNS arteriovenous malformations of childhood\n\nSuggested Reading:\n\nFullerton HI, Achrol AS, Johnston SC, et al. Long-term hemorrhage risk in children versus adults with brain arteriovenous malformations. Stroke. 2005;36(10):2099-2104. doi: 10.1161/01.STR.0000181746.77149.2b\n\nGetzoff M, Goldstein B. Spontaneous subarachnoid hemorrhage in children. Pediatr Rev. 1999;20(4):141. doi 10.1542/pir.20-12-422\n\nKochanek PM, Bell MJ. Neurologic emergencies and stabilization. In: Kliegman RM, Stanton BF, St Geme JW III, Schor NF, Behrman RE, eds. Nelson Textbook of Pediatrics. 19th ed. Philadelphia, PA: Saunders Elsevier; 2011:296-304"}
{"id" : 3617, "question_text" : "A 9-year-old boy is seen for concerns of fecal incontinence. As a toddler, he often had large, hard stools with withholding. Over the last 3 years, he has continued to have large, hard stools once to twice weekly as well as multiple fecal accidents daily. He receives polyethylene glycol every 3 to 4 days as needed. He has no enuresis. He takes atomoxetine for attention-deficit/hyperactivity disorder. He was born at 41 weeks' gestation and passed meconium within the first 24 hours after birth. He has no surgical history. His parents are divorced, and his father has recently gained shared custody. There is a feculent odor in the examination room. Physical examination is notable for a well-appearing child in no apparent distress. His abdomen is distended but nontender. There is a large amount of palpable stool in the left lower quadrant. Rectal examination reveals a normal-appearing anus with brown soiling present, normal anal wink, and dilated rectal vault with palpable hard stool. Normal lower extremity reflexes are present.\n\nOf the following, the MOST likely diagnosis is", "options" : "[\"anal stenosis\", \"functional constipation\", \"Hirschsprung disease\", \"tethered cord syndrome\"]", "explanation" : "Correct Answer: B\nThe boy in this vignette has functional constipation and fecal incontinence. His history of normal passage of meconium and physical examination findings of significant stool burden, dilated rectum, and otherwise normal neurologic examination findings are consistent with a diagnosis of functional constipation. The Rome III diagnostic criteria for functional constipation require the presence of at least 2 of the following: 2 or less defecations in the toilet per week, at least 1 episode of fecal incontinence per week, history of stool retention, history of painful/hard bowel movements, rectal fecal mass, and/or history of large-diameter stools that may obstruct the toilet. Thus, the boy in this vignette meets the diagnostic criteria for functional constipation.\n\nConstipation is defined as the infrequent passage of hard, painful stools that are distressing to the child. While there are many causes of constipation (Item C61), functional constipation is the most common cause in children. Constipation occurring in young infants, particularly in association with delayed passage of meconium, abdominal distention, and/or failure to thrive, should prompt consideration for pathologic causes of constipation, including Hirschsprung disease, anorectal malformations, and cystic fibrosis. In older infants or children with constipation, pathologic causes of constipation should be considered if concerning signs are present, including abdominal distention, bloody stools, poor growth, and/or abnormal anorectal examination findings (fistula, absent anal wink, tight anorectal tone, or sacral dimple), or if constipation continues despite appropriate therapy.\n\nManagement of functional constipation includes education of the child and family, medication administration (for \"clean out\"/disimpaction in addition to daily, maintenance medications), and behavioral modification. Education is critical as the first step in management, as often families assume the child is \"being lazy\" or soiling on purpose. An understanding of normal colonic physiology and anatomy in addition to the pathophysiology of chronic constipation, including overflow incontinence, can be helpful for children and families. The next step, particularly in children with fecal incontinence, is disimpaction/\"clean out.\" If a child has a large stool burden, prescribing maintenance laxative dosing may exacerbate soiling and further frustrate the child and/or family. Generally, polyethylene glycol solution (1.0-1.5 g/kg/day for up to 3 days) is suggested. Rectal therapies are discouraged if possible. Maintenance programs should include daily medication administration and behavioral modification. Polyethylene glycol, an osmotic laxative, is recommended as first-line maintenance therapy (0.4 g/kg/day, adjusted to achieve daily stooling) for at least 2 months. All symptoms should be resolved for at least 1 month prior to stopping treatment. For children who are toilet training, medications should not be stopped until successful toilet training is achieved. Other medications that may be used to treat constipation include other osmotic laxatives (lactulose), stool softeners/lubricants (mineral oil), and stimulant laxatives (bisacodyl, senna). Behavioral modifications should include scheduled toilet sitting after meals for 5 to 10 minutes (with rewards given for completing the task of sitting on the toilet and not necessarily for defecating on the toilet), appropriate toileting position (using a child insert seat if appropriate and a stepstool to keep the feet securely planted), and keeping a diary of bowel movements/episodes of incontinence to track the child's progress.\n\nAnal stenosis presents in infancy, and examination would demonstrate a tight anal sphincter. Hirschsprung disease is unlikely in this patient with a dilated rectum and normal history of meconium passage. Hirschsprung disease presenting in childhood may occur, however it is usually associated with abdominal distention, poor weight gain, and severe, refractory constipation. Tethered cord syndrome and other spinal cord abnormalities may present with bladder dysfunction and abnormal neurologic examination findings, including an absent anal wink.\n\nPREP Pearls\n• Functional constipation is the most common cause of constipation in children.\n• Management of functional constipation includes family education, medication administration (for \"clean out\" and maintenance), and behavior modification.\n• First-line therapy for functional constipation is daily polyethylene glycol; this should continue for at least 2 months and should not stop unless symptoms have resolved for at least 1 month.\n• Red flags for pathologic causes of constipation include: abdominal distention, failure to thrive, delayed passage of meconium, and constipation refractory to medical management.\n\nMOCA-Peds Objective\n• Evaluate and manage a patient with constipation.\n\nABP Content Specifications(s)\n• Understand the action of laxatives, stool softeners, and lubricants in a patient with constipation\n• Formulate an age-appropriate differential diagnosis in a patient with constipation\n• Plan the appropriate management of a patient with constipation\n\nSuggested Readings\n• Belamarich PF. Constipation. In: McInerny TK, Adam HM, Campbell DE, DeWitt TG, Foy JM, Kamat DM, eds. American Academy of Pediatrics Textbook of Pediatric Care. 2nd ed. Elk Grove Village, IL: American Academy of Pediatrics; 2017:1240-1247. Pediatric Care Online.\n• Colombo JM, Wassom MC, Rosen JM. Constipation and encopresis in childhood. Pediatr Rev. 2015;36(9):392-402. doi:10.1542/pir.36-9-392.\n• Tabbers MM, DiLorenzo C, Berger MY, et al. Evaluation and treatment of functional constipation in infants and children: evidence-based recommendations from ESPGHAN and NASPGHAN. J Pediatr Gastroenterol Nutr. 2014;58(2):258-274. https://www.naspghan.org/files/documents/pdfs/cme/jpgn/evaluation_and_treatment_of_functional.24.pdf."}
{"id" : 3548, "question_text" : "A child with septic shock has been stabilized hemodynamically. What hemoglobin threshold should guide red blood cell transfusion decisions?", "options" : "[\"Transfusion should be avoided if hemoglobin is \\u22657 g/dL in hemodynamically stabilized children\", \"All children with septic shock require transfusion to maintain hemoglobin >10 g/dL\", \"Transfusion is indicated whenever hemoglobin drops below 9.5 g/dL\", \"Hemoglobin levels do not influence transfusion decisions in septic shock\"]", "explanation" : "The text recommends against RBC transfusion if blood hemoglobin concentration is ≥7 g/dL in hemodynamically stabilized children with septic shock, based on the TRIPICU study showing no differences in outcomes between restrictive and liberal transfusion strategies."}
{"id" : 1408, "question_text" : "A 16-year-old healthy adolescent girl is brought to your office for a follow-up visit, accompanied by her mother. She was seen and treated at a local emergency department yesterday after having multiple episodes of vomiting with inability to tolerate oral fluids. Her mother tells you that the adolescent was diagnosed with \"a stomach flu\" and dehydration, received 2 bags of intravenous fluids, and was discharged home after she drank a few ounces of ginger ale. Her urine pregnancy test was negative.\n\nThe mother brought her daughter to your office for re-evaluation this morning because \"she still seems really run down,\" and has been complaining of worsening abdominal pain. She has had no vomiting since last evening. She has had no fever, diarrhea, cough, or congestion. The mother tells you that her daughter has also seemed very \"stressed out\" over the past 5 days since her boyfriend ended their relationship. The adolescent denies any vaginal discharge or bleeding; her last menstrual period was 2 weeks ago.\n\nThe patient's vital signs include a temperature of 37.1°C, heart rate of 100 beats/min, blood pressure of 118/70 mm Hg, respiratory rate of 16 breaths/min, and pulse oximetry of 100% on room air. On physical examination, she is tearful and seems to be in moderate distress due to pain. Her abdomen is soft with tenderness over her right upper quadrant. Her liver edge is palpable about 3 cm below her right costal margin. She has active bowel sounds and no peritoneal signs. A complete neurologic examination reveals no focal abnormalities. Genitourinary examination reveals no vaginal discharge or bleeding, and a bimanual examination reveals no adnexal or cervical motion tenderness.\n\nFollowing the physical examination, you ask the patient's mother to give you an opportunity to talk with her daughter alone. When you ask the patient why she is tearful, she discloses to you that, over a 2-day period that ended approximately 36 hours ago, she took more than fifty 500 mg acetaminophen tablets because she was \"in so much pain\" after her boyfriend ended their relationship. She denies suicidal ideation and states that she took the medicine because \"I just wanted something to take away my pain.\" She did not share this information with her mother or any staff in the emergency department yesterday because she felt embarrassed about taking the medication.\n\nOf the following, the BEST next step in the management of this patient is to", "options" : "[\"arrange to see her again in your office within 24 hours for re-evaluation\", \"obtain a serum acetaminophen level to determine her need for further therapy\", \"obtain urgent child psychiatric consultation due to concern for suicidality\", \"transfer her to the emergency department for activated charcoal\", \"transfer her to the emergency department for N-acetylcysteine therapy\"]", "explanation" : "The adolescent girl in the vignette presents with fatigue and right upper quadrant tenderness, which have developed since she intentionally ingested more than 25 g of acetaminophen over a 48-hour period. Her abdominal pain was preceded by multiple episodes of vomiting, which have now resolved. This patient's symptoms and clinical findings are highly suggestive of the development of hepatotoxicity as a result of acetaminophen poisoning. The best next step in her management is to transfer her to the emergency department for N-acetylcysteine (NAC) therapy.\n\nAcetaminophen is the most commonly used analgesic and antipyretic in the United States. It is one of the most common pharmaceutical products ingested by young children, and is one of the top drugs taken by adolescents and adults in intentional drug overdoses. Clinical outcomes for children and adolescents presenting with acetaminophen poisoning are nearly always positive if appropriate management, including prompt (within 8 to 10 hours following ingestion) administration of NAC, is instituted. All pediatric providers should be able to recognize the signs and symptoms of acetaminophen toxicity and to manage them appropriately.\n\nThe risk for toxicity from acetaminophen ingestion in children and adolescents depends largely upon the situation surrounding exposure. Among adolescents, intentional ingestion due to suicidal intent is more prevalent. These ingestions tend to involve higher doses of acetaminophen, often taken as a single overdose. Intentional ingestion of other substances, in addition to acetaminophen, is not uncommon in these situations. Adolescents who ingest acetaminophen in an attempt to self-harm often underestimate its toxicity and develop hepatocellular injury more frequently than younger children with lower-dose, exploratory ingestions. These ingestions may be disclosed by the adolescent to friends, family members, and healthcare providers following ingestion, or they may be discovered only as a result of toxicological screening.\n\nUnintentional ingestions of acetaminophen, often due to developmentally-normal exploratory behaviors, are more commonly observed among young children. Most of these ingestions involve small doses, and can often be managed in the primary care setting or even at home with appropriate anticipatory guidance.\n\nCases of significant acetaminophen toxicity due to unintentional repeated administration of supratherapeutic doses to children by well-meaning caregivers have been reported. Diagnosis of these cases may be delayed because symptoms and signs are nonspecific and may be mistaken for symptoms of the illness for which acetaminophen was being given in the first place. Deliberate poisoning of infants with acetaminophen as a form of child abuse has also been reported.\n\nThe primary toxicity of acetaminophen is severe hepatic injury. Acetaminophen is metabolized in the liver to a highly reactive metabolite by the cytochrome P450 pathway. Its toxic intermediate is normally inactivated by conjugation with hepatic glutathione. In massive acetaminophen overdoses (or in situations of chronic acetaminophen ingestion), glutathione becomes depleted, allowing the toxic intermediate to bind to liver cells and cause cellular injury. This injury can lead to elevation of liver enzymes, hepatic dysfunction, and even hepatic failure and death.\n\nAn acute acetaminophen ingestion of greater than 150 to 200 mg/kg in children or greater than 7.5 g total in adults is potentially hepatotoxic. Patients who have taken supratherapeutic doses over the course of consecutive days are also at risk for hepatocellular injury.\n\nEarly signs and symptoms of acute acetaminophen poisoning in children are nonspecific and can often be mild. The expected clinical course of acute acetaminophen toxicity is often divided into 4 characteristic stages:\n• Stage 1 (up to 24 hours post-ingestion): Patients may be entirely asymptomatic, or display nonspecific symptoms such as nausea, vomiting, malaise, and fatigue/lethargy\n• Stage 2 (24 to 72 hours post-ingestion): Findings may include right upper quadrant pain, elevated hepatic enzymes, elevated prothrombin time (PT) and international normalized ratio of PT, and hepatic enlargement. In severe cases, evidence of nephrotoxicity (elevated blood urea nitrogen, creatinine, decreased urine output) and pancreatitis may be apparent.\n• Stage 3 (72 to 96 hours post-ingestion): Progression to hepatic failure, and even renal failure and multisystem organ failure in severe cases. Death from acetaminophen poisoning occurs most commonly in this clinical stage.\n• Stage 4 (4 to 14 days post-ingestion): Recovery\n\nClinical findings for the adolescent girl in the vignette indicate that she has progressed to \"Stage 2\" of acetaminophen toxicity. Transfer to an emergency department for immediate treatment with NAC and further management to help prevent progression to hepatic failure is crucial to her management.\n\nInitial management of acute acetaminophen exposures in children should involve aggressive supportive care, decontamination with activated charcoal in those children presenting within 4 hours of ingestion, and administration of NAC when indicated. N-acetylcysteine is most beneficial if administered within 8 hours of an acute acetaminophen ingestion. When acute acetaminophen toxicity is suspected, a serum acetaminophen level should be obtained (ideally at 4 hours post-ingestion) and plotted on the Rumack-Matthew nomogram, which can be found in many standard references on acetaminophen toxicity. Given the serum acetaminophen level and the number of hours post-ingestion, this nomogram can be used to classify the patent as at no risk, possible risk, or probable risk of hepatotoxicity. If the serum acetaminophen level falls above the possible risk threshold, treatment with NAC should be initiated. Hepatic enzyme levels, a coagulation profile, serum electrolytes, and a complete blood cell count should be obtained before initiating treatment, as well as following treatment.\n\nRecommending that the adolescent follow up in the office within 24 hours would be an inappropriate choice related to her management. Her clinical findings indicate that she has progressed to \"Stage 2\" of acetaminophen toxicity (hepatocellular injury), and she is at high risk for progressing to complete hepatic failure and even death without immediate clinical intervention, including institution of NAC therapy.\n\nDelaying referral to the emergency department in order to obtain a serum acetaminophen level would not be appropriate in the management of this patient. While obtaining a serum acetaminophen level (ideally 4 hours post-ingestion) to help determine the need for further therapy would be recommended for children presenting acutely after a known or suspected acetaminophen ingestion, this patient's presentation is delayed (approximately 72 hours after ingestion) and she is presenting with current findings that indicate hepatotoxicity arising after excessive acetaminophen ingestion. Immediate institution of NAC therapy would be indicated in her situation, regardless of her serum acetaminophen level.\n\nAlthough assessment by a child psychiatric specialist is certainly indicated for the adolescent in the vignette due to her recent depressive symptoms and concern for suicidality, the more immediate priority in her management should involve emergent clinical interventions to prevent her from developing hepatic failure and failure of other organ systems. Child psychiatric consultation may be obtained after she has been medically stabilized.\n\nWhile the administration of activated charcoal for gastrointestinal decontamination is recommended for children and adolescents presenting very soon after an acute acetaminophen overdose (provided that the airway is protected), the patient in the vignette is presenting days(not hours) after ingestion of a large amount of acetaminophen. She has unfortunately already had complete gastrointestinal absorption of the acetaminophen she ingested and is now displaying signs of hepatic toxicity. Activated charcoal would not have a clinical benefit at this point in her clinical course. For children presenting within 4 hours of a significant known or suspected acetaminophen ingestion, treatment with activated charcoal (1 g/kg) is recommended, provided that there are no contraindications to its administration, such as a concern that the child's airway is not adequately protected or gastrointestinal obstruction.\n\nPREP Pearls\n• Among adolescents, intentional ingestions of acetaminophen due to suicidal intent tend to involve high doses of acetaminophen, taken as a single overdose.\n• An acute acetaminophen ingestion of greater than 150 to 200 mg/kg in children, or greater than 7.5 g total in adults is potentially hepatotoxic.\n• Initial management of acute acetaminophen exposures in children should involve aggressive supportive care, decontamination with activated charcoal in those children presenting within 4 hours of ingestion, and administration of N-acetylcysteine when indicated.\n\nABP Content Specifications(s)\n• Recognize the signs and symptoms of acetaminophen toxicity, and manage appropriately\n\nSuggested Readings\n• Argentieri J, Morrone K, Pollack Y. Acetaminophen and ibuprofen overdosage. Pediatr Rev. 2012;33(4):188-189. doi: http://dx.doi.org/10.1542/pir.33-4-188.\n• Feng S-Y, Goto CS. Toxic ingestions and exposures. In: Stone CK, Humphries RL, Drigalla D, Stephan M, eds. Current Diagnosis and Treatment: Pediatric Emergency Medicine. New York, NY: McGraw-Hill; 2015:631-662.\n• O'Donnell KA, Osterhoudt KC, Burns MM, Caiello, Henretig FM. Toxicologic emergencies. In: Shaw KN, Bachur RG, Chamberlain J, Lavelle J, Nagler J, Shook JE, eds. Textbook of Pediatric Emergency Medicine. 7th ed. Philadelphia, PA: Lippincott Williams and Wilkins; 2015:1061-1114."}
{"id" : 346, "question_text" : "A 4-year-old boy presents with a 4-day history of worsening right eyelid swelling and redness after a mosquito bite. On physical examination, his temperature is 38.0°C, heart rate is 100 beats/min, and respiratory rate is 25 breaths/min. His right eyelid is markedly swollen, red, and tender, and he is unable to open it fully. His conjunctivae are clear, and extraocular movements are not limited. There is no proptosis. Visual acuity is difficult to assess fully but appears normal. There are no other physical findings of note. The white blood cell count is 19.0x103/mcL (19.0x109/L), with 55% polymorphonuclear leukocytes, 20% band forms, 20% lymphocytes, and 5% monocytes. Of the following, the MOST appropriate antibiotic for this patient is", "options" : "[\"ampicillin-sulbactam\", \"cefazolin\", \"clindamycin\", \"doxycycline\", \"trimethoprim-sulfamethoxazole\"]", "explanation" : "The boy described in the vignette has periorbital (preseptal) cellulitis characterized by eyelid edema and erythema, normal extraocular movements, apparent normal visual acuity, and absence of proptosis and chemosis. Staphylococcus aureus and Streptococcus pyogenes (group A Streptococcus) (GAS) are the most common causes of periorbital cellulitis in cases in which cultures are obtained. In addition, community-associated methicillin-resistant S aureus (CA-MRSA) has emerged as a significant pathogen. Clindamycin can treat GAS and susceptible strains of CA-MRSA and is appropriate therapy for this boy. The use of a semisynthetic penicillin (eg, dicloxacillin, nafcillin, oxacillin) or a first-generation cephalosporin (eg, cephalexin, cefazolin) is appropriate in communities where MRSA is not prevalent. Doxycycline can be used to treat uncomplicated soft-tissue infections caused by susceptible strains of S aureus in children older than 7 years of age, but it has variable activity against GAS. Trimethoprim-sulfamethoxazole is effective against susceptible strains of S aureus but is not useful for treating GAS. Vancomycin should be used in patients who have severe infection and in communities where clindamycin resistance is prevalent.\n\nPeriorbital cellulitis occurs most commonly in children younger than 5 years of age. It can be due to localized infection or inflammation of the eyelid, conjunctivae, or adjacent structures (hordeolum, dacrocystitis, dacroadenitis, trauma), such as occurred in the patient in the vignette, who developed infection following an insect bite. In such cases, GAS and S aureus are the most common causes of infection, but S pneumoniae and nontypable Haemophilus influenzae also can cause infection in patients who have dacryoadenitis and dacrocystitis. Antimicrobial therapy can be guided based on Gram stain of an aspirate from the infected area.\n\nPeriorbital cellulitis also can be caused by inflammatory edema of rhinosinusitis, most often involving the ethmoids. In such cases, S pneumoniae, nontypable H influenzae, and Moraxella catarrhalis are the usual pathogens. Appropriate antibiotic therapy in such cases can include amoxicillin-clavulanate, a second-generation cephalosporin (cefuroxime), or a third-generation cephalosporin (cefdinir, cefpodoxime, cefotaxime, ceftriaxone).\n\nOccasionally, periorbital cellulitis is caused by hematogenous seeding in children younger than 18 months of age, with an antecedent viral upper respiratory tract infection. S pneumoniae, nontypable H influenzae, GAS, and S aureus are likely pathogens. Cerebrospinal fluid analysis should be performed in young infants or children who are severely ill. Parenteral therapy with a third-generation cephalosporin and clindamycin or vancomycin is appropriate.\n\nFinally, periorbital cellulitis caused by a mixed aerobic and anaerobic infection should be considered in patients who have dental abscesses. Ampicillin-sulbactam or clindamycin can be used in those who have suspected anaerobic infections and require intravenous antibiotics. Appropriate oral therapy includes amoxicillin-clavulanate or clindamycin.\n\nA complete history and physical examination are necessary for all children who have suspected periorbital cellulitis. A history of sinusitis, dental problems, and trauma, including eye surgery, should be elicited. Physical examination focusing on the presence or absence of eyelid fluctuance, proptosis, chemosis, and normal extraocular movements and visual acuity is important. Patients who have severe eyelid swelling that prevents eye examination or those in whom orbital abscess (eg, orbital cellulitis) is suspected should undergo computed tomography scan with contrast of the sinuses and orbits. Eyelid (or other) abscesses should be drained and purulent material sent for culture.\n\nThe decision to treat a patient with intravenous versus oral therapy should be based on the severity of disease and clinical response. A 7- to 10-day course of treatment may be appropriate for those who have simple periorbital cellulitis. Duration of therapy is guided by clinical response. Children who have bacteremia should receive 10 days of parenteral therapy. Patients who have complicated disease may require longer courses of antibiotics.\n\nCritique: Preferred Response: C\n\nContent Specifications: Know the diagnostic approach for periorbital (preseptal) cellulitis; Know the treatment of periorbital (preseptal) cellulitis"}
{"id" : 2562, "question_text" : "A 6-month-old infant, born at 27 weeks' gestation, with grade 2 vesicoureteral reflux, gastroesophageal reflux, and chronic lung disease is seen in the emergency department for lethargy. His daily medications include fluticasone, furosemide, lansoprazole, and nitrofurantoin. His temperature is 36.8°C, heart rate is 120 beats/min, respiratory rate is 30 breaths/min, blood pressure is 85/65 mm Hg, and oxygen saturation is 99% on his home oxygen support of 0.5 L/min via nasal cannula. The infant's anterior fontanelle is slightly sunken, and his mucous membranes are moist and pink. On lung auscultation, fine rhonchi are heard throughout without wheezing or rales. There are no retractions or other signs of increased work of breathing. The remainder of his physical examination findings are normal. Laboratory data are shown: Laboratory Test Result Sodium 130 mEq/L (130 mmol/L) Potassium 2.8 mEq/L (2.8 mmol/L) Chloride 89 mEq/L (89 mmol/L) Carbon dioxide 38 mEq/L (38 mmol/L) Blood urea nitrogen 10 mg/dL (3.6 mmol/L) Creatinine 0.3 mg/dL (26.5 µmol/L) Of the following, the medication MOST likely to have led to this infant's findings is", "options" : "[\"fluticasone\", \"furosemide\", \"lansoprazole\", \"nitrofurantoin\"]", "explanation" : "Correct Answers: B\nThe infant in the vignette, with chronic lung disease related to prematurity, has signs of mild dehydration (mildly sunken anterior fontanelle), and electrolyte abnormalities including hyponatremia, hypokalemia, hypochloremia, and an elevated bicarbonate level. These findings are consistent with a contraction metabolic alkalosis induced by chronic diuretic therapy. Thus, of the response choices, furosemide (used to control excess fluid in the lungs) is the most likely medication to have contributed to this infant's condition.\n\nFluticasone (an inhaled corticosteroid used to manage pulmonary inflammation), lansoprazole (a proton-pump inhibitor used to treat gastroesophageal reflux), and nitrofurantoin (a bactericidal antibiotic used for urinary tract infection prophylaxis), commonly used to treat complications of prematurity, are not associated with the electrolyte abnormalities seen in the infant in the vignette.\n\nFurosemide is a loop diuretic that inhibits reabsorption of sodium and chloride in the ascending loop of Henle and the proximal and distal renal tubules by interfering with the chloride-binding co-transport system. Both natriuresis (sodium loss) and diuresis (water loss) result. Diuresis decreases extracellular fluid volume and concentrates extracellular serum bicarbonate, contributing to metabolic alkalosis. Historically, this process was known as \"contraction alkalosis,\" and the primary mechanism was thought to be due to decreased fluid volume and the resulting concentration of serum bicarbonate. However, newer studies have demonstrated that chloride plays a major role in the development of metabolic alkalosis, and some have suggested that \"chloride depletion alkalosis\" replace the term \"contraction alkalosis\" to more accurately reflect the underlying mechanism.\n\nMetabolic alkalosis is often associated with chloride anion loss, either through gastric fluid or excessive urinary excretion. Infants with severe emesis syndromes (eg, pyloric stenosis) classically present with a hypochloremic metabolic alkalosis. Additionally, chloride-wasting diuretics can induce a metabolic alkalosis. Infants and children with chronic lung disease can have a chronic respiratory acidosis from retention of carbon dioxide, and may develop a compensatory metabolic alkalosis to maintain a normal acid-base balance.\n\nGitelman and Bartter syndromes are associated with metabolic alkalosis, a high urinary excretion of serum potassium, and hypokalemia. Bartter syndrome is an autosomal recessive condition characterized by hypokalemia, excessive renal wasting of sodium chloride, and metabolic alkalosis due to reduced activity of one of several electrolyte transporters in the ascending loop of Henle. In addition to the expected electrolyte abnormalities associated with salt-wasting, impaired sodium chloride reabsorption leads to volume depletion and increased activity of the renin-angiotensin-aldosterone system. Secondary hyperaldosteronism and increased sodium excretion enhances potassium and hydrogen ion loss in the urine leading to hypokalemia and metabolic alkalosis. Children with Bartter syndrome are often seen with failure to thrive, lethargy, polydipsia, polyuria, dehydration, hypotonia, and developmental delay.\n\nPREP Pearls\n• Metabolic alkalosis is often caused by hypochloremia.\n• Infants and children on chronic diuretic therapy with loop diuretics (eg, furosemide) are at risk for developing a chloride depletion alkalosis.\n• Bartter syndrome can cause hypokalemic, hypochloremic, metabolic alkalosis; this diagnosis should be suspected in infants with hyponatremia, hypokalemia, failure to thrive, and dehydration.\n\nABP Content Specifications(s)\n• Identify factors contributing to metabolic alkalosis\n\nSuggested Readings\n• Hsu BS, Lakhani SA, Wilhelm M. Acid-base disorders. Pediatr Rev. 2016;37(9):361-369. doi:10.1542/pir.2015-0093.\n• Mahajan P. Fluids, electrolytes, and acid-base composition. In: McInerny TK, Adam HM, Campbell DE, DeWitt TG, Foy JM, Kamat DM, eds. American Academy of Pediatrics Textbook of Pediatric Care. American Academy of Pediatrics; 2021:chap 58. Accessed September 1, 2022. Pediatric Care Online.\n• Schwaderer AL, Schwartz GJ. Back to basics: acidosis and alkalosis. Pediatr Rev. 2004;25(10):350-357. doi:10.1542/pir.25-10-350.\n• Tergestina M, Chandran S, Kumar M. Case 2: Metabolic alkalosis in a neonate 12 hours after birth. NeoReviews. 2018;19(10):e613-e615. doi:10.1542/neo.19-10-e613.\n• Thakore P, Anderson M, Yosypiv IV. Classic Bartter syndrome: a cause of severe hypokalemic metabolic alkalosis. Clin Pediatr (Phila). 2019;58(14):1557-1561. doi:10.1177/0009922819857535."}
{"id" : 2332, "question_text" : "A 12-year-old girl is seen for evaluation of absent menstrual periods. Her mother had menarche at age 11 years and her older sister at age 12 years. The girl has no relevant medical history, and results of a review of systems are unremarkable. She is a dancer and practices 5 days per week. Her mother reports that she has a good appetite. The girl's vital signs are normal for age. Her growth curves are shown in Figure 1 and Figure 2. Her sexual maturity rating is 1 for breast development and pubic hair. The remainder of the girl's physical examination findings are unremarkable. Of the following, this girl's MOST likely diagnosis is", "options" : "[\"anorexia nervosa\", \"constitutional delay of growth and development\", \"Mayer-Rokitansky-K\\u00fcster-Hauser syndrome\", \"Turner syndrome\"]", "explanation" : "PREP Pearl(s)\nThe first sign of puberty is breast development in girls and testicular growth in boys.\nNormal pubertal onset occurs between ages 8 to 13 years in girls and 9 to 14 years in boys.\nThe most common cause of delayed puberty is constitutional delay of growth and development.\nCritique\nThe girl in the vignette has no signs of puberty. She has short stature compared with her peers and a low body mass index. She is otherwise healthy, is very physically active, and has normal physical examination findings and vital signs. At age 12 years, she does not yet have abnormally delayed puberty. The most likely cause of her lack of pubertal development and short stature is constitutional delay of growth and development, a normal variant of growth and puberty.\nPuberty is defined as the appearance of breast tissue in girls or testicular growth in boys. The normal range for puberty onset is between ages 8 and 13 years for girls and ages 9 and 14 years for boys. True puberty should be distinguished from isolated pubic or axillary hair growth (premature adrenarche). Adolescents who do not show any signs of pubertal development by age 13 years (girls) or 14 years (boys) should undergo evaluation for primary (gonadal) or secondary (pituitary or hypothalamic) causes of pubertal failure. This evaluation may include measurement of concentrations of gonadotropins (luteinizing hormone, follicle-stimulating hormone) and sex steroids (testosterone for boys, estradiol for girls), which normally rise during puberty. Pubertal development is affected by genetic factors, general health status, and body weight. Adolescents who have low weight will often have delayed puberty.\nAnorexia nervosa can result in delayed puberty owing to low body weight and overall energy deficiency. Anorexia nervosa is often accompanied by abnormal vital signs (hypothermia, bradycardia), physical signs such as lanugo, and laboratory indicators of malnutrition. The girl in the vignette is healthy, with no concerning findings other than her lack of puberty. Additionally, there is not a concern about restrictive eating or excess exercise.\nMayer-Rokitansky-Küster-Hauser syndrome is characterized by lack of development of the Müllerian structures (uterus, fallopian tubes, proximal vagina). This syndrome typically manifests in affected girls during adolescence with primary amenorrhea. The hormone changes of puberty, and therefore secondary sex characteristics (except menarche), are normal. The girl in the vignette has no signs of puberty, so Mayer-Rokitansky-Küster-Hauser syndrome is not the most likely diagnosis.\nGirls with Turner syndrome (45,X karyotype or variant) typically have abnormal puberty caused by primary ovarian failure. Classically, there are physical findings that suggest the diagnosis (eg, significant short stature, increased carrying angle of the elbows, wide-spaced nipples, low-lying hairline). The girl in the vignette does not have any features suggestive of Turner syndrome.\nSuggested Reading(s)\nBakhtiani P, Geffner M. Delayed puberty. Pediatr Rev. 2022;43(8):426-435. doi:10.1542/pir.2020-005291\nHarrington J, Palmert MR. An approach to the patient with delayed puberty. J Clin Endocrinol Metab. 2022;107(6):1739-1750. doi:10.1210/clinem/dgac054\nKritzler RK, Long D, Plotnick L. Puberty: normal and abnormal. In: McInerny TK, Adam HM, Campbell DE, Foy JM, Kamat DM, eds. American Academy of Pediatrics Textbook of Pediatric Care. 2nd ed. American Academy of Pediatrics; 2023. Accessed September 1, 2023. Pediatric Care Online\nWolf RM, Long D. Pubertal development. Pediatr Rev. 2016;37(7):292-300. doi:10.1542/pir.2015-0065\nContent Domain\nEndocrinology\nABP Content Specification(s) / Content Area(s)\nRecognize the stages of sexual development and the range of age of onset of each\nUnderstand factors that influence the timing of puberty\nRecognize laboratory values that change in girls and boys during puberty\nThe correct answer is: constitutional delay of growth and development"}
{"id" : 2865, "question_text" : "A 9-year-old boy is seen for a routine follow-up for asthma that was diagnosed at age 3 years. He has been treated with inhaled corticosteroids since the time of diagnosis. His current medications include mometasone furoate 100 μg with formoterol fumarate 5 μg two inhalations twice daily, fluticasone propionate one spray in each nostril daily, and albuterol two puffs inhaled every 4 hours as needed for cough, shortness of breath, or wheeze. His asthma has been well controlled since his inhaled corticosteroid therapy was intensified 3 years ago. His last asthma exacerbation and last course of oral corticosteroids was more than 1 year ago. His vital signs are normal for his age. His growth chart is shown. His body mass index is 15.9 kg/m2 (40th percentile). Examination of his respiratory system yields normal findings. The remainder of his physical examination findings are unremarkable. Of the following, the MOST likely cause of this boy's physical findings is", "options" : "[\"constitutional delay of growth\", \"exogenous glucocorticoid\", \"growth hormone deficiency\", \"pituitary adenoma\"]", "explanation" : "The boy in the vignette has linear growth failure that coincides with intensification of his inhaled glucocorticoid therapy regimen. The most likely cause of his linear growth failure is exogenous glucocorticoid. Excess glucocorticoid by any route can have a profound effect on linear growth. In this case, the route is inhalation.\n\nThe boy's growth curve is not consistent with constitutional delay of growth. In constitutional delay of growth, growth velocity at this age should be normal. His growth curve could be consistent with growth hormone deficiency, but given his history, exogenous glucocorticoid is more likely. An adrenocorticotropic hormone (ACTH)-producing pituitary adenoma can cause growth failure due to excess endogenous glucocorticoid, but the boy has a history of exogenous inhaled glucocorticoid exposure, making the exogenous form the more likely culprit. Excess inhaled glucocorticoid, as with excess glucocorticoid acquired by any route, can also suppress the endogenous hypothalamic-pituitary-adrenal (HPA) axis. This HPA axis suppression becomes a problem when the axis cannot respond appropriately to stress and symptoms of adrenal insufficiency ensue.\n\nFrank Cushingoid features can also occur with excess inhaled corticosteroids. Cushing syndrome is characterized by weight gain, centripetal obesity, linear growth failure, violaceous striae, and osteopenia. The most common cause of Cushing syndrome is exogenous glucocorticoid. Cushing syndrome caused by endogenous glucocorticoid excess is rare.\n\nEndogenous Cushing syndrome can be divided into adrenocorticotropic hormone (ACTH)-dependent and ACTH-independent etiologies. Adrenocorticotropic hormone–dependent causes include an ACTH-secreting pituitary adenoma and ectopic ACTH secretion. Adrenocorticotropic hormone–independent causes are due to adrenal tumors or other adrenal hyperfunction. After the clinician excludes exogenous glucocorticoid exposure, the first step in the evaluation of Cushing syndrome is to confirm hypercortisolism. Options for initial testing for hypercortisolism include a 24-hour urine free cortisol test, a low-dose (1-mg) overnight dexamethasone suppression test, or a midnight salivary cortisol test. Positive test findings should be confirmed with a second measurement. A morning cortisol level that fails to suppress after administration of low-dose dexamethasone the night before is consistent with hypercortisolism. Similarly, an elevated midnight salivary cortisol level is consistent with hypercortisolism and disruption of the normal circadian rhythm.\n\nOnce hypercortisolism is confirmed, the next step is to distinguish an ACTH-dependent versus ACTH-independent etiology and further determine the source. These second-line tests include an ACTH level, a high-dose dexamethasone suppression test, and pituitary or adrenal imaging as indicated. Cortisol levels are suppressed after administration of high-dose dexamethasone with an ACTH-dependent pituitary source of Cushing but not with adrenal sources. Pituitary imaging may show an adenoma. Adrenal imaging may show an adrenal tumor or nodular adrenal disease.\n\nPREP Pearls\n• The most common cause of Cushing syndrome is exogenous glucocorticoid.\n• Excess glucocorticoid acquired by any route can have a profound effect on linear growth.\n• Suppression of the hypothalamic-pituitary-adrenal axis by excess glucocorticoid acquired by any route can cause an adrenal crisis during times of stress.\n\nABP Content Specifications(s)\n• Plan appropriate diagnostic evaluation of Cushing syndrome\n• Identify the clinical features associated with Cushing syndrome, including that associated with exogenous corticosteroid therapy\n\nSuggested Readings\n• Kapadia CR, Nebesio TD, Myers SE, et al; Drugs and Therapeutics Committee of the Pediatric Endocrine Society. Endocrine effects of inhaled corticosteroids in children. JAMA Pediatr. 2016;170(2):163-170. doi:10.1001/jamapediatrics.2015.3526.\n• Klein J, Vuguin P, Hyman S. Cushing syndrome. Pediatr Rev. 2014;35(9):405-407. doi:10.1542/pir.35-9-405.\n• Smith A, Doan ML, Roy D, Pinsker JE. Adrenal insufficiency and growth failure secondary to inhaled corticosteroids: a paradoxical complication. Clin Pediatr (Phila). 2012;51(12):1194-1196. doi:10.1177/0009922812437932.\n• Speiser PW. Adrenal dysfunction. In: McInerny TK, Adam HM, Campbell DE, DeWitt TG, Foy JM, Kamat DM, eds. American Academy of Pediatrics Textbook of Pediatric Care. 2nd ed. Elk Grove Village, IL: American Academy of Pediatrics; 2017:1692-1700. Pediatric Care Online."}
{"id" : 3593, "question_text" : "A 13-year-old adolescent girl was a passenger in a multi-car collision and is brought to the emergency department by emergency medical services. She was appropriately restrained by a shoulder and lap belt in the rear passenger seat when the car was struck on the driver's side by a sports utility vehicle that ran a red light. The airbags deployed, and there was minor difficulty opening the driver's side doors. The patient never lost consciousness, but reported mild abdominal, back, and right upper extremity pain. She was placed in a hard cervical collar, secured to a spine board, and transported to the emergency department. Upon arrival, the patient is awake, alert, and oriented, although visibly anxious. She answers all questions without any difficulty. Her vital signs are unremarkable aside from mild tachycardia. She is attached to a cardiorespiratory monitor, removed from the spine board, and placed onto a bed in the trauma bay. Two intravenous lines are placed, and 20 mL/kg of normal saline is infused. The primary and secondary surveys are significant for mild tachycardia, right thoracic and lumbar paraspinal muscle tenderness, and mild lower abdominal tenderness. There is no bony tenderness and no visible bruising. Urinalysis is negative for blood, and a urine pregnancy test has negative results. Of the following, the MOST appropriate initial imaging study for this patient's abdomen and pelvis is", "options" : "[\"computed tomography\", \"focused sonography\", \"magnetic resonance imaging\", \"radiography\"]", "explanation" : "Correct Answer: B\nThe adolescent in this vignette has been in a serious car collision, as evident by the airbag deployment and difficulty opening the car door. She was appropriately placed in a hard cervical collar and transported to the emergency department for further evaluation. Given the abdominal tenderness, there is concern for an intra-abdominal injury, which warrants imaging. The first imaging test that should be considered for evaluation of intra-abdominal pathology caused by blunt trauma is focused abdominal sonography in trauma (FAST). The FAST examination consists of sonographic views of the suprapubic area, the hepatorenal recess, and the splenorenal recess to look for free fluid in the abdomen and a subxiphoid view of the heart to look for a pericardial effusion. Extended-FAST (e-FAST) adds sonographic views of the lungs to evaluate for of a pneumothorax. Computed tomography of the abdomen and pelvis is indicated if fluid is seen in the abdomen by FAST.\n\nThe initial approach to a patient who has sustained trauma is called the primary survey and consists of evaluation of the airway (with cervical spine control), breathing, circulation, disability, and exposure of the patient. Item C37 lists life-threatening emergencies that can be found (and treated) during the primary survey. During the primary survey, gastric and urinary catheters should be placed. However, if there is blood at the urethral meatus or a perineal hematoma, a urinary catheter should not be placed. Electrocardiography, chest and pelvic radiography, and FAST are adjuncts to the primary survey. After completing the primary survey and ensuring the patient's stability, the secondary survey is done. It includes a comprehensive history and physical examination to evaluate for information that may facilitate treatment and an assessment for additional injuries. During the secondary survey, laboratory evaluation, additional radiography, and computed tomography are performed as necessary.\n\nChildren who sustain blunt abdominal trauma and are hemodynamically unstable require a diagnostic laparotomy irrespective of the findings of the FAST examination. On the other hand, positive FAST findings do not always lead to a laparotomy because most solid organ injuries in children are managed nonoperatively. Laboratory studies that are helpful in a child who has sustained significant blunt abdominal trauma include a complete blood cell count, electrolytes, creatinine, liver function tests, amylase, lipase, coagulation studies, blood type and screen, urinalysis, and β-human chorionic gonadotropin if appropriate.\n\nPREP Pearls\n• The primary survey for trauma evaluates a patient's airway, breathing, circulation, disability, and exposure.\n• Focused abdominal sonography in trauma should be used to evaluate for abdominal free fluid in the setting of abdominal trauma.\n\nABP Content Specifications(s)\n• Plan the appropriate evaluation of abdominal trauma, with and without hematuria\n\nSuggested Readings\n• American College of Surgeons. Abdominal and pelvic trauma. In: Advanced Trauma Life Support ATLS Student Course Manual. 9th ed. Chicago, IL: American College of Surgeons; 2012:122-140.\n• American College of Surgeons. Initial assessment and management. In: Advanced Trauma Life Support ATLS Student Course Manual. 9th ed. Chicago, IL: American College of Surgeons; 2012:2-28.\n• Fein DM, Fagan MJ. Overall approach to trauma in the emergency department. Pediatr Rev. 2018;39(10):479-489. doi:10.1542/pir.2017-0246."}
{"id" : 1886, "question_text" : "A 5-year-old boy with myelomeningocele has a ventriculoperitoneal shunt that was last revised when he was 1 year old. He has epilepsy that is well-controlled with levetiracetam. He ambulates with forearm crutches. Over the past 2 months, the boy has been complaining that his legs and back hurt, and he refuses to walk longer distances. On physical examination, he has weak and atrophic lower extremities with diminished reflexes, and bilateral pes cavus. The remainder of his examination findings are normal. Computed tomography of the head without contrast shows his shunt tubing in appropriate position and ventricles unchanged in size from the previous imaging 18 months ago. Of the following, the MOST likely cause of his new symptoms is", "options" : "[\"chronic urinary tract infection\", \"expected progression of myelomeningocele\", \"shunt failure\", \"tethered cord\"]", "explanation" : "The boy in the vignette, with myelomeningocele, has new symptoms of back and leg pain and decreased ambulation. Myelomeningocele is a static problem, so new symptoms suggest a new process. The 2 most likely causes for his symptoms are tethered cord and increased intracranial pressure due to shunt failure. Because computed tomography of his head showed no change from previous imaging, the most likely cause is tethered cord. The best diagnostic test for suspected tethered cord is magnetic resonance imaging of the spine. Chronic urinary tract infection may cause back pain but would not cause limitations in ambulation.\n\nMyelomeningocele is a neural tube defect in which the spinal cord and the meninges protrude out from the spinal canal to the surface of the skin. It is typically repaired at birth, and the defect does not worsen or change over time. Myelomeningocele is commonly associated with Chiari II malformation and hydrocephalus, strabismus, learning disabilities, seizures, bowel and bladder dysfunction, latex allergy, and tethered cord. Myelomeningocele and Chiari II malformation are generally diagnosed antenatally, but tethered cord may not present until years later, especially during periods of growth. Symptoms of tethered cord include back or leg pain and worsening of gait, as in the boy in the vignette. There may also be a change in bowel or bladder function, progressive scoliosis, new contractures, or worsening limb atrophy.\n\nPREP Pearls\n\nMyelomeningocele is a static problem, so new symptoms suggest a new process.\n\nIn children with myelomeningocele, tethered cord may not present until the child is older, particularly during periods of growth.\n\nSymptoms of tethered cord may include back or leg pain, worsening of gait, change in bowel or bladder function, progressive scoliosis, new contractures, or worsening limb atrophy.\n\nABP Content Specifications(s)/Content Area\n\nRecognize the clinical manifestations of and complications associated with spinal dysraphism, and manage appropriately\n\nRecognize other abnormalities commonly associated with myelomeningocele\n\nSuggested Readings\n\nBui CJ, Tubbs RS, Oakes WJ. Tethered cord syndrome in children: a review. Neurosurg Focus. 2007;23(2):E2. doi: 10.3171/foc.2007.23.2.2.\n\nLiptak GS, Dosa NP. Myelomeningocele. Pediatr Rev. 2010;31(11):443-450. doi: 10.1542/pir.31-11-443. doi: 10.1542/pir.31-11-443."}
{"id" : 225, "question_text" : "A healthy term newborn has done well in the nursery and is ready for discharge. While speaking with the new mother, you learn that her brother has a coughing illness accompanied by fever, weight loss, and hemoptysis. He often stays at his sister's home. No other family members, including the infant's mother, are ill.\n\nOf the following, the next BEST management step for the newborn is to", "options" : "[\"isolate the infant and the mother\", \"obtain a chest radiograph\", \"place a tuberculin skin test\", \"separate the infant from the uncle\", \"start isoniazid therapy\"]", "explanation" : "The infant described in the vignette, whose maternal uncle may have active tuberculosis, should be separated from the uncle. Cases of suspected or proven tuberculosis require immediate reporting to the local health department to initiate a contact investigation. If the infant's mother is asymptomatic and tuberculosis is not suspected, separating the infant from the mother is not necessary. An interferon-gamma release assay (IGRA) or a tuberculin skin test (TST) should be performed on the mother, and if results are positive, a chest radiograph is necessary. Because the infant has never been in contact with the uncle, it is unnecessary to place a TST or obtain a chest radiograph for the infant unless evaluation of the mother raises concerns for tuberculosis. In addition, the infant does not require isoniazid therapy.\n\nIf tuberculosis is suspected in the mother or in a household member with whom the infant has been in contact, the infant should be separated from the mother (or household contact) until the mother (or household contact) has been evaluated and the mother (contact) and infant are receiving appropriate antituberculosis therapy. The mother also must comply with proper infection control measures, including wearing a mask. Infants of mothers who have tuberculosis at the time of delivery require an evaluation for congenital infection, including a TST, chest radiograph, and lumbar puncture for cerebrospinal fluid analysis that involves stain and culture for acid-fast bacilli. IGRAs should not be used in infants. Congenital infection is rare and is unlikely in cases of maternal pulmonary disease but can occur with maternal bacillary tuberculosis. Because TSTs often are negative in infants who have congenital or perinatally acquired tuberculosis, antituberculosis therapy (isoniazid, rifampin, pyrazinamide, amikacin) should be initiated in all infants undergoing evaluation for congenital infection. If congenital infection is excluded, exposed infants should receive isoniazid until a repeat TST is performed at 3 to 4 months of age. If the TST is positive, the infant should undergo repeat evaluation for tuberculosis disease. If the TST is negative and the adult contact who has tuberculosis is culture-negative (ie, not contagious) and has good antituberculosis medication compliance, isoniazid can be discontinued.\n\nCritique: [As above]\n\nContent Specifications: Plan the management of a child who has an adult household contact with active tuberculosis."}
{"id" : 860, "question_text" : "You are called by a family who has just learned that an infant will become available for adoption. The mother is a late entrant into prenatal care. Results of maternal screening studies, including hepatitis B, human immunodeficiency virus, and rapid plasma reagin, are negative. Urine toxicology result is positive for marijuana and cocaine at the most recent prenatal visit at 30 weeks of gestation. The family is delighted about the upcoming adoption but asks if the illicit substances used by the mother during pregnancy may affect the infant. Of the following, the MOST appropriate response is that the infant is at increased risk of", "options" : "[\"congenital heart disease\", \"failure to thrive\", \"moderate intellectual disability\", \"neonatal drug withdrawal\", \"preterm birth\"]", "explanation" : "Preferred Response: E\nFetal cocaine exposure is associated with preterm birth, low birthweight, and small-for-gestational age size. This relationship exists after controlling for maternal confounders, including cigarette smoking, other drug exposures, lower socioeconomic status, and inadequate prenatal care. Cocaine exposure has been associated with a decrease in all fetal growth measurements, which worsens with advancing gestational age. Maternal cocaine use is postulated to have vasoconstrictive effects that contribute to placental insufficiency, infarction, and/or abruption. These events may contribute to the preterm birth, low birthweight, and small-for-gestational age size associated with infants exposed to cocaine prenatally.\n\nNo drug withdrawal syndrome has been formally described for cocaine. Cocaine is a central nervous system stimulant and may be found in the urine sample of an exposed infant up to 1 week after delivery. Although some infants exposed to prenatal cocaine will exhibit tremors and irritability 2 to 3 days after delivery, studies have not supported either a drug withdrawal syndrome or drug toxicity syndrome.\n\nThe effects of fetal cocaine exposure on the developing brain remain unclear. A recent review by Ackerman, et al, summarized existing data on school-aged children prenatally exposed to cocaine. After adjusting for environmental influences, exposed children have sustained attention and behavioral self-regulation deficits. Growth, cognitive ability, academic achievement, and language appear to be minimally affected. Brain imaging studies suggest minor effects on structure and function, but the study numbers are limited.\n\nCocaine has not been associated with major teratogenic malformations of the cardiac or genitourinary system. Although infants exposed to cocaine may be small for gestational age or of low birthweight, they typically catch up within 6 months and demonstrate small to no growth differences at school age. When environmental risk factors are removed, cognitive ability appears to be normal. Prenatal identification and support of cocaine-dependent mothers, with continued postnatal education and support may have a positive effect on the long-term development of at-risk infants.\n\nPREP Pearls\n• Fetal cocaine exposure is associated with preterm birth, low birthweight, and small-for-gestational age size.\n• No drug withdrawal syndrome has been formally described for cocaine.\n\nAmerican Board of Pediatrics Content Specification(s):\n• Know the association between the maternal use of cocaine and any fetal abnormalities and/or neonatal withdrawal syndrome\n\nSuggested Reading\n• Ackerman JP, Riggins T, Black MM. A review of the effects of prenatal cocaine exposure among school-aged children. Pediatrics. 2010;125:554565. doi:10.1542/peds.2009-0637. http://pediatrics.aappublications.org/ content/125/3/554.full\n• Behnke M, Smith VC; Committee on Substance Abuse, Committee on Fetus and Newborn. Prenatal substance abuse: short- and long-term effects on the exposed fetus. Pediatrics. 2013;131(3):e1009-e1024. doi:10.1542/peds.2012- 3931\n• Gouin K, Murphy K, Shah PS, et al. Effects of cocaine use during pregnancy on low birthweight and preterm birth: systematic review and metaanalyses. Am J Obstet Gynecol. 2011;204:340e1-e12. doi:10.1016/j. ajog.2010.11.013\n• Hudak ML, Tan RC, the Committee on Drugs, the Committee on Fetus and Newborn. Neonatal drug withdrawal. Pediatrics. 2012;129:e540-e560. doi:10.1542/peds.2011-3212"}
{"id" : 2515, "question_text" : "A 3-month-old male infant born at term is brought to the emergency department via ambulance for a seizure. Initial evaluation shows an ionized calcium level of 3.3 mg/dL (0.8 mmol/L) (reference range, 4.5-5.3 mg/dL [1.1-1.3 mmol/L]). He is treated with intravenous calcium, which aborts the seizure. The infant's parents report that he has been jittery and irritable for the past few days. He exclusively breastfed for the first 2 months after birth. Due to fussiness, his parents recently started making formula using a recipe found on the internet containing hemp seed hearts, coconut water, dates, and sea moss. The infant was recently diagnosed with laryngomalacia after an evaluation for noisy breathing. His physical examination findings are normal for age. His parents are of normal stature. Laboratory evaluation drawn prior to treatment reveals the following: Laboratory Test Result Total calcium 4.5 mg/dL (1.1 mmol/L) (reference range, 9-11 mg/dL [2.2-2.8 mmol/L]) Phosphorous 1.2 mg/dL (reference range, 2.7-4.5 mg/dL) Magnesium 2.1 mg/dL (0.9 mmol/L) (1.6-2.6 mg/dL [0.7-1.1 mmol/L]) Parathyroid hormone 534 pg/mL (reference range, 10-65 pg/mL) Alkaline phosphatase 1,021 U/L (reference range, 146-477 U/L) 25-hydroxyvitamin D Pending 1,25-dihydroxyvitamin D Pending Of the following, the BEST next step in this infant's management is oral administration of", "options" : "[\"calcium, cholecalciferol, and calcitriol\", \"calcium and phosphorus\", \"magnesium\", \"phosphorus and calcitriol\"]", "explanation" : "The infant in the vignette has hypocalcemia due to severe vitamin D deficiency. His noisy breathing and seizure are manifestations of hypocalcemia. After treating his symptomatic hypocalcemia with intravenous calcium, the infant should be treated with oral calcium and cholecalciferol (dietary vitamin D3). Calcitriol (1,25-dihydroxyvitamin D, the active form of vitamin D) should be added given the severity of his hypocalcemia. Calcitriol acts immediately to absorb calcium from the intestine while vitamin D stores are replenished by cholecalciferol. The infant should also be switched to a cow milk–based formula, given its higher vitamin D content, once he can safely drink from a bottle.\n\nBreast milk does not provide adequate vitamin D; infants who are exclusively or predominantly breastfed must receive supplementation with this vitamin. Although commercial formulas are fortified with vitamin D, homemade formulas, including the one described in the vignette, do not contain additional vitamin D. The American Academy of Pediatrics recommends that breastfed infants receive at least 400 IU of supplemental vitamin D daily.\n\nVitamin D is a steroid hormone that is integral in the regulation of calcium and phosphorus homeostasis. Item C7A outlines vitamin D metabolism and action. Laboratory findings depend on the severity and duration of the vitamin D deficiency (Item C7B). In mild vitamin D deficiency, relative hypocalcemia will result in a rise in parathyroid hormone (PTH) to maintain serum calcium in the normal range. Calcium is reabsorbed from the kidney, absorbed from the gut (via conversion of remaining 25-hydroxyvitamin D stores to 1,25-dihydroxyvitamin D), and released from the bones. As vitamin D deficiency becomes more severe and PTH rises higher, calcium is depleted and levels begin to fall. Increased PTH levels cause phosphate wasting in the kidney, resulting in low serum phosphorus levels. Parathyroid hormone has an indirect effect on osteoclasts, resulting in increased bone resorption. Alkaline phosphatase levels rise as bone turnover is increased. When 25-hydroxyvitamin D stores are extremely low, there is inadequate vitamin D available for conversion to 1,25-dihydroxyvitamin D, which results in low levels of this hormone. The infant in the vignette is expected to have low 25-hydroxyvitamin D and 1,25-dihydroxyvitamin D levels.\n\nItem C7B\n\nItem C7A\n\nManifestations of severe vitamin D deficiency include rickets (widening of the wrists, rachitic rosary, craniotabes) and symptoms of hypocalcemia. Infants may display irritability, constipation, noisy breathing due to laryngospasm, and seizures. Older children may experience paresthesias.\n\nTreatment with phosphorus and calcitriol is indicated for hypophosphatemia due to defects in the fibroblast growth factor 23 (FGF23) pathway, usually due to mutations in the PHEX gene. Fibroblast growth factor 23 is the main hormone responsible for phosphorus wasting in the kidney. Burosumab, a monoclonal antibody that inhibits FGF23, is approved by the US Food and Drug Administration for the treatment of children aged 6 months and older with X-linked hypophosphatemia. Although the infant in the vignette is hypophosphatemic, this presentation is due to secondary hyperparathyroidism and the phosphate wasting effect that the elevated PTH level has on the kidney. Additionally, both parents are of normal stature, which is evidence against familial hypophosphatemia, an X-linked dominant disorder that negatively impacts growth.\n\nMagnesium is required for a normal PTH response to low calcium levels. In children with hypomagnesemia, hypocalcemia may develop due to inadequate PTH action. In this case, the PTH level would be inappropriately low. This situation commonly occurs in infants of diabetic mothers.\n\nCalcium and phosphorus is the treatment for metabolic bone disease of prematurity, which occurs due to a deficiency of these minerals. The infant in the vignette was born at term, so this would be an unlikely diagnosis. In addition, severe hypocalcemia is an unlikely finding in metabolic bone disease of prematurity.\n\nPREP Pearls\n• The American Academy of Pediatrics recommends supplementation of 400 IU daily of vitamin D for exclusively breastfed infants.\n• Manifestations of severe vitamin D deficiency may include rickets (widening of the wrists, rachitic rosary, craniotabes) and hypocalcemia with secondary hyperparathyroidism.\n• Homemade infant formulas may contain or lack ingredients that may affect mineral homeostasis.\n\nABP Content Specifications(s)\n• Recognize the effects of vitamin D deficiency in patients of various ages, including those who are breast-fed\n\nSuggested Readings\n• Dawodu A, Wagner CL. Vitamin D inadequacy. In: McInerny TK, Adam HM, Campbell DE, DeWitt TG, Foy JM, Kamat DM, eds. American Academy of Pediatrics Textbook of Pediatric Care. American Academy of Pediatrics; 2021:chap 346. Accessed September 1, 2022. Pediatric Care Online.\n• Holick MF, Binkley NC, Bischoff-Ferrari HA, et al; Endocrine Society. Evaluation, treatment, and prevention of vitamin D deficiency: an Endocrine Society clinical practice guideline. J Clin Endocrinol Metab. 2011;96(7):1911-30. doi:10.1210/jc.2011-0385.\n• Misra M, Pacaud D, Petryk A, Collett-Solberg PF, Kappy M. Vitamin D deficiency in children and its management: review of current knowledge and recommendations. Pediatrics. 2008; 122(2):398-417. doi:10.1542/peds.2007-1894.\n• Munns CF, Shaw N, Kiely M, et al. Global consensus recommendations on prevention and management of nutritional rickets. J Clin Endocrinol Metab. 2016;101(2):394-415. doi:10.1210/jc.2015-2175.\n• Vieira MA, Kube PK, van Helmond JL, et al. Recipe for disaster: homemade formula leading to severe complications in 2 infants. Pediatrics. 2021;148(3):e2021050947. doi:10.1542/peds.2021-050947.\n• Wagner CL, Greer FR; American Academy of Pediatrics Section on Breastfeeding; American Academy of Pediatrics Committee on Nutrition. Prevention of rickets and vitamin D deficiency in infants, children, and adolescents. Pediatrics. 2008;122(5):1142-1152. doi:10.1542/peds.2008-1862."}
{"id" : 2632, "question_text" : "A 14-year-old adolescent boy is seen for a preparticipation physical examination to play soccer. He is healthy with no concerns voiced by him or his parents. His physical examination findings are normal except for a systolic ejection click heard best at the right upper sternal border. Further inquiry reveals that the boy's paternal grandfather had aortic valve surgery at age 55 years. The boy is referred to a pediatric cardiologist. Of the following, the boy's MOST likely diagnosis is", "options" : "[\"bicuspid aortic valve\", \"partial anomalous pulmonary venous return\", \"pulmonary valve stenosis\", \"ventricular septal defect\"]", "explanation" : "The boy in the vignette has a systolic ejection click heard at the right upper sternal border, suggestive of aortic valve pathology, and a family history (paternal grandfather) of aortic valve pathology. Of the response choices, bicuspid aortic valve (BAV) is the most likely diagnosis. He should be evaluated by a pediatric cardiologist; echocardiography is needed to confirm the diagnosis. If there is no significant valve dysfunction or aortic dilation, children with BAV can often play sports and exercise without restriction.\n\nThe boy's physical examination findings and family history are not suggestive of the other congenital heart diseases listed as response choices. Children with partial anomalous pulmonary venous return have clinical evidence of increased pulmonary blood flow; the acyanotic child may be tachypneic with hepatomegaly and a pulmonary murmur due to the relatively increased flow. The murmur is a systolic ejection murmur best heard at the left upper sternal border. A child with pulmonary stenosis may have few symptoms, but may have exercise intolerance and a systolic ejection murmur best heard at the left upper sternal border. A ventricular septal defect should present in infancy with tachypnea and symptoms of pulmonary overcirculation, depending on the size of the defect, with a holosystolic murmur at the left sternal border.\n\nBicuspid aortic valve is a congenital heart disease in which the aortic valve develops with 2 leaflets instead of the normal 3. It is one of the most common congenital anomalies, affecting ~1% to 2% of the population. It is more common in males than females with a 3:1 predominance. Twenty percent to 30% of affected individuals have a family member with BAV or an associated aortopathy. Individuals with BAV can develop aortic regurgitation, aortic stenosis, or both. Roughly one-third will develop aortic aneurysms. Abnormalities of the aorta itself can occur independent of valve function. While most individuals with BAV develop these sequelae during adulthood, ~10% to 15% will do so in childhood. Life-long serial imaging is indicated to monitor aortic valve function, ascending aortic size, and for aneurysm formation.\n\nIndividuals with BAV and significant aortic valve dysfunction, aortic dilation, or aortic aneurysm require medical and sometimes surgical intervention. Medication to prevent high blood pressure (eg, β-blocker) is often prescribed when there is aortic dilation; surgical intervention may ultimately be indicated. Surgical or interventional (cardiac catheterization) procedures may be required to relieve obstruction caused by aortic valve stenosis. When the aortic valve is significantly regurgitant, sometimes surgical procedures to repair the valve are successful, but if not, the valve needs to be replaced with a prosthetic or mechanical valve. Anticoagulation is needed after valve replacement.\n\nPREP Pearls\n• Bicuspid aortic valve, in which the aortic valve develops with only 2 leaflets instead of the normal 3, is one of the most common congenital heart defects.\n• Bicuspid aortic valve occurs in males more than females and can be familial.\n• Individuals with bicuspid aortic valve can develop valve dysfunction (stenosis, regurgitation, or both), aortic dilation, or aortic aneurysm.\n\nABP Content Specifications(s)\n• Understand the natural history of a bicuspid aortic valve\n\nSuggested Readings\n• Baleilevuka-Hart M, Teng BJ, Carson KA, Ravekes WJ, Holmes KW. Sports participation and exercise restriction in children with isolated bicuspid aortic valve. Am J Cardiol. 2020;125(11):1673-1677. doi:10.1016/j.amjcard.2020.02.039.\n• Niaz T, Fernandes S, Sanders SP, Michelena H, Hagler DJ. Clinical history and management of bicuspid aortic valve in children and adolescents. Prog Cardiovasc Dis. 2020;63:425-433. doi:10.1016/j.pcad.2020.05.012.\n• Niaz T, Johnson JN, Cetta F, Olson TM, Hagler DJ. Bicuspid aortic valve in infants, children, and adolescents: a review for primary care providers. Pediatr Rev. 2021;42(5):233-244. doi:10.1542/pir.2019-0307.\n• Otto CM, Nishimura RA, Borrow RO, et al. 2020 ACC/AHA Guideline for the Management of Patients with Valvular Heart Disease: A Report of the American College of Cardiology/American Heart Association Joint Committee on Clinical Practice Guidelines. J Circulation. 2021;143(5):e72-e227. doi:10.1161/CIR.0000000000000923."}
{"id" : 2315, "question_text" : "A 15-year-old adolescent is being evaluated for acne. They tried an over-the-counter salicylic acid wash without benefit. They has been in good health and take no medications. There are approximately 8 eight active inflammatory lesions on each cheek and their chin. There are several open comedones (Figure 1), but no scarring. The forehead, chest, and back are free of acne lesions. Of the following, the MOST appropriate treatment is", "options" : "[\"benzoyl peroxide administered topically\", \"benzoyl peroxide/clindamycin administered topically\", \"benzoyl peroxide/clindamycin and a retinoid administered topically\", \"doxycycline administered orally and benzoyl peroxide administered topically\"]", "explanation" : "Critique\nThis patient has moderate inflammatory and comedonal acne involving the face (Figure 2). There are several inflammatory papules (red arrow) and open comedones (yellow arrow). No scarring is evident, but there are several resolving inflammatory lesions (blue arrow). These lesions may remain erythematous or violaceous for months and are often confused with scars. The most appropriate treatment, therefore, is benzoyl peroxide (BPO)/clindamycin applied to the face each morning and a retinoid (eg, tretinoin or adapalene) applied at bedtime. Therapy with BPO, BPO/clindamycin, or doxycycline orally combined with BPO topically would address the inflammatory component of the disease, but not follicular obstruction. In addition, because the adolescent has moderate acne that is limited to the face with no scarring, and is using no medication, an attempt to manage the inflammatory component of the disease with topical agents is reasonable.\nSeveral factors contribute to the development of acne. Key among these are disordered keratinization (leading to obstruction within pilosebaceous follicles), increased sebum production (which contributes to obstruction), and inflammation (caused, in large part, by activation of the immune system by the bacterium Cutibacterium acnes). As obstruction increases, follicles may rupture, contributing to the inflammatory process. In some patients, the inflammatory process results in scarring. On the face, scars appear as small pits, whereas on the trunk they are hypopigmented macules. \nTreatment plans should be designed to affect as many aspects of disease pathophysiology as possible. The inflammatory process can be addressed with BPO, antibiotics (topical or oral), and dapsone. Topical retinoids improve follicular obstruction; oral contraceptives, in girls, lessen the impact of androgens (reducing sebum production and follicular obstruction). It is important to note that particularly in moderate or severe acne, follicular obstruction is present and should be addressed therapeutically, even if blackheads (open comedones) and whiteheads (closed comedones) are not observed. \nGuidelines for acne management exist, but treatment should be individualized according to the patient's perception of disease severity, past experiences with medications, cost (insurance coverage may vary), and the ability to adhere to therapy. Suggested treatment plans for mild, moderate, and severe acne are presented in Table 1, Table 2, and Table 3.\nSuggested Reading(s)\nChen C, Williams JV. Acne. In: McInerny TK, Adam HM, Campbell DE, DeWitt TG, Foy JM, Kamat DM, eds. American Academy of Pediatrics Textbook of Pediatric Care. American Academy of Pediatrics; 2023. Accessed September 1, 2023. Pediatric Care Online\nEichenfield LF, Krakowski AC, Piggott C, et al; American Acne and Rosacea Society. Evidence-based recommendations for the diagnosis and treatment of pediatric acne. Pediatrics. 2013;131(suppl 3):S163-S186. doi:10.1542/peds.2013-0490B\nMancini AJ, Krowchuk DP, eds. Pediatric Dermatology: A Quick Reference Guide. 3rd ed. Elk Grove Village, IL: American Academy of Pediatrics; 2016.\nThiboutot DM, Dréno B, Abanmi A, et al. Practical management of acne for clinicians: an international consensus from the Global Alliance to Improve Outcomes in Acne. J Am Acad Dermatol. 2018;78(2 suppl 1):S1-S23.e1. doi:10.1016/j.jaad.2017.09.078\nContent Domain\nDermatology\nABP Content Specification(s) / Content Area(s)\nRecognize the clinical findings associated with acne\nPlan the appropriate management of acne\nThe correct answer is: benzoyl peroxide/clindamycin and a retinoid administered topically"}