import json NEW = {} NEW[136] = [ {"title": "Clinical paths", "content": """**Recognition and Initial Assessment** CAH is suspected in any infant presenting with: - Ambiguous genitalia in a 46,XX infant - Salt-wasting crisis in a male infant aged 5–14 days: vomiting, weight loss, lethargy, poor feeding, [[99|dehydration]], tachycardia, or hypothermia - Abnormal newborn screening result (elevated 17-hydroxyprogesterone)"""}, {"title": "Diagnosis", "content": """**Immediate Investigations** Urgent blood tests are obtained: - Electrolytes (looking for [[170|hyponatremia]] and hyperkalemia) - Glucose - Venous or arterial blood gas (to assess for [[387|metabolic acidosis]]) - 17-hydroxyprogesterone, renin, cortisol, and aldosterone levels - ECG is performed to assess for peaked T waves or arrhythmias from hyperkalemia"""}, {"title": "Management", "content": """**Acute Management of Salt-Wasting Crisis** 1. **Fluid resuscitation:** Intravenous normal saline is administered to correct [[161|severe dehydration]] and [[170|hyponatremia]] 2. **Glucocorticoid replacement:** Initiated immediately 3. **Mineralocorticoid replacement:** Required in salt-wasting forms 4. **Electrolyte monitoring:** Electrolytes are rechecked frequently during the acute phase 5. **Cardiac monitoring:** Arrhythmias related to hyperkalemia are watched for **Ongoing Glucocorticoid Dosing** - Classic CAH (salt-wasting and simple virilizing forms): 10–15 mg/m²/day - Nonclassical CAH: 6–10 mg/m²/day **Specialist Referral** Urgent referral to a pediatric endocrinologist is arranged. Management involves a multidisciplinary team including endocrinology, genetics, urology, and psychosocial support, particularly for discussion of sex assignment and long-term management in virilized females."""}, ] NEW[137] = [ {"title": "Clinical paths", "content": """**Initial Assessment** 1. A complete menstrual history is taken, including timing of pain, duration and severity 2. HEEADSSS psychosocial screening and detailed sexual history are performed 3. Hidden agendas are explored (school avoidance, abuse history, secret sexual activity) 4. Abdominal and pelvic examination is performed (per vaginum in sexually active adolescents) 5. Red flags for secondary dysmenorrhea are identified: pain at menarche, intercycle pain, dyspareunia, abnormal discharge, pelvic mass or tenderness"""}, {"title": "Diagnosis", "content": """**Investigation for Secondary Causes** If pain persists despite appropriate NSAIDs and hormonal management for 6 months, or if red flags are present: - Pelvic ultrasound is arranged - Pelvic MRI is considered - Endometriosis is specifically screened for (most common secondary cause) - Müllerian anomalies, obstructive reproductive tract anomalies, myomas and ovarian cysts are evaluated for"""}, {"title": "Management", "content": """**First-Line Treatment: NSAIDs** Treatment is started as soon as the patient anticipates menstruation will begin: - **Naproxen**: twice daily (age and weight-appropriate dose) - **Ibuprofen**: every 6–8 hours (age and weight-appropriate dose) Treatment continues on schedule throughout menstruation. Reassessment occurs after 2–3 menstrual cycles. **Second-Line Treatment: Hormonal Suppression** For patients not responding to NSAIDs: - **Oral contraceptive pills (OCPs)**: used as for contraception. The patient is informed that 2–3 cycles may elapse before maximal effect - **Levonorgestrel-releasing intrauterine device (LNG-IUD)**: effective for both primary and secondary dysmenorrhea - **Subdermal implant**: effective for both primary and secondary dysmenorrhea For non-sexually active teenagers on OCPs, therapy is reassessed at 6–12 month intervals. **Note**: Acetaminophen-containing products are not recommended as they are less effective than NSAIDs for dysmenorrhea."""}, ] NEW[138] = [ {"title": "Clinical paths", "content": """**Initial Assessment** 1. **History and observation**: A complete feeding session is observed. Feeding duration, volumes consumed, fatigue, respiratory symptoms, and vomiting or gagging are documented. Feeding schedule consistency, mealtime environment, and caregiver concerns are asked about. 2. **Physical examination**: Neurologic signs, craniofacial abnormalities, oral anatomy (lip closure, tongue position, ankyloglossia), and signs of aspiration (recurrent respiratory infections, chronic lung disease) are assessed. The growth chart is examined for recent decline or weight below 5th centile. 3. **Determination of organic versus nonorganic etiology**: This distinction guides further workup. Organic causes (neurologic impairment, cardiac disease, pulmonary disease, anatomic abnormality) require specific investigation. Nonorganic causes (schedule inconsistency, environmental factors, caregiver practices) require behavioral and environmental intervention."""}, {"title": "Diagnosis", "content": """**Diagnostic Considerations** - **Swallowing assessment**: If concerns exist regarding pharyngeal phase physiology or aspiration risk, instrumental assessment of swallowing is considered. - **Specific conditions**: [[171|Hypothyroidism]] is diagnosed through blood sample (low T3 and T4, high TSH). Prader-Willi syndrome presents with hypotonia in an otherwise normal child. - **Referral for specialist evaluation**: Occupational therapy or speech-language pathology is considered for assessment of oral-motor difficulties and aspiration risk, particularly in preterm infants or those with [[82|developmental delay]]."""}, {"title": "Management", "content": """**Management Principles** - **Correctable organic causes are addressed first**: Underlying conditions are treated (e.g., [[245|gastroesophageal reflux]], cardiac disease, [[171|hypothyroidism]]). - **Environmental optimization**: A consistent feeding schedule is established. The child is positioned in a high chair when available. Distractions (television) are removed. Ineffective positioning such as the hammerlock hold in the parent's lap is avoided. - **Feeding therapy**: Ongoing therapy for oral-motor difficulties may be required. Feeding evaluation and therapy may benefit infants with difficulty transitioning to solid foods or aspiration risk. - **Caregiver education**: Nutritional knowledge, appropriate feeding techniques, and cultural food practices are addressed. - **Nutritional support**: If oral intake remains insufficient despite intervention, tube feeding is considered."""}, ] NEW[139] = [ {"title": "Clinical paths", "content": """3. **Disability**: Consciousness level is assessed, and pupil size and reactivity are examined. Shock does not occur from isolated head injury beyond infancy. In children with [[209|hemophilia]] and major head trauma, immediate treatment with replacement therapy is initiated to achieve 100% factor activity level. For minor trauma without symptoms or external evidence of injury in mild hemophilia, replacement therapy may not be required; the physician must assess the type of trauma and bleeding history to determine need."""}, {"title": "Diagnosis", "content": """Blood is obtained for full blood count and group and cross-match. If unequal pupils or concerns regarding head injury are present, neuroprotection measures are initiated, CT head imaging is arranged, and neurosurgical consultation is obtained. Once the child's condition is stabilized, a complete secondary survey is performed to identify all injuries. X-rays of chest and pelvis are considered."""}, {"title": "Management", "content": """**Primary Survey** 1. **Airway and Breathing**: The airway is secured. Respiratory support is provided if Glasgow Coma Scale is less than 8 or the child is responsive only to pain. 2. **Circulation**: Vital signs are assessed. Crystalloid 20 ml/kg is administered and the patient is reassessed. 4. **Exposure and Temperature Control**: All clothing is removed. All parts of the body are examined. Hypothermia is avoided. Analgesia is considered. A gastric tube is placed (not a nasal tube in head injury). **Secondary Survey** Surgical opinion is sought if ruptured liver/spleen, fractured pelvis, or long bone fracture is suspected."""}, ] NEW[140] = [ {"title": "Clinical paths", "content": """**Initial assessment:** - Detailed history is obtained, including maternal illness, delivery circumstances, and symptom onset - Thorough physical examination is performed, repeated if necessary - Danger signs are assessed for: abnormal temperature (hypothermia <36°C or fever ≥38°C), tachypnea >60 breaths/min, [[151|respiratory distress]], apnea, tachycardia >180 beats/min, delayed capillary refill >3 seconds, weak or bounding pulses, abnormal abdominal or neurologic findings"""}, {"title": "Diagnosis", "content": """**Investigations (all febrile neonates):** - Complete blood count - Blood culture - Urine culture (via catheterization or suprapubic aspiration) - Cerebrospinal fluid study and culture (lumbar puncture) - Chest X-ray if respiratory cause suspected"""}, {"title": "Management", "content": """**Empiric antibiotic therapy:** Intravenous antibiotics are started immediately after investigations are sent, without awaiting results. *Neonates 0–7 days of age:* - Ampicillin PLUS Gentamicin - Some experts add a third or fourth generation cephalosporin if cerebrospinal fluid Gram stain shows gram-negative organisms *Neonates 8–28 days of age:* - Ampicillin PLUS Cefotaxime (or Ceftazidime or Cefepime if Cefotaxime unavailable) - Some experts add an aminoglycoside if cerebrospinal fluid Gram stain shows gram-negative organisms **Important considerations:** - Ceftriaxone is avoided (risk of bilirubin displacement and hyperbilirubinemia) - All febrile neonates are admitted regardless of clinical appearance or initial laboratory results - Toxic-appearing neonates are treated aggressively - For well-appearing febrile neonates with suspected non-infectious cause (over-bundling), observation for [[226|sepsis]] signs is maintained - Specific antibiotic choice and duration are guided by culture results and organism susceptibility once available - Some experts recommend repeat lumbar puncture to document cerebrospinal fluid sterility - Empiric acyclovir with surface, blood, and cerebrospinal fluid HSV sampling is considered for infants at increased risk **Follow-up:** - Parents are counselled on danger signs requiring immediate return - Outpatient follow-up is arranged for infants with no identified focus on initial evaluation"""}, ] NEW[141] = [ {"title": "Clinical paths", "content": """**History and Examination** Evaluation begins with a systematic history addressing the onset, distribution, and progression of the rash. Associated symptoms, triggers, and any treatments already attempted are documented. The lesions are examined carefully, noting their morphology, distribution pattern, and any secondary changes. **Lesion Morphology** Lesions are classified by type (papules, vesicles, pustules, plaques, erythema), as this guides the differential diagnosis. Papulosquamous and vesiculopustular eruptions are the most common presentations in children. For neonatal presentations, the age of onset and associated systemic symptoms are considered."""}, {"title": "Diagnosis", "content": """**Special Considerations** For pigmented lesions, particularly those involving the nail unit, size, color uniformity, and any changes over time are documented. Specialist evaluation is sought if there is diagnostic uncertainty or concerning features."""}, {"title": "Management", "content": """Diaper dermatitis, seborrheic dermatitis, and vascular lesions require different management approaches. **Documentation** Findings are recorded with clear description of morphology and distribution. This documentation supports accurate diagnosis and guides appropriate management or referral."""}, ] NEW[142] = [ {"title": "Clinical paths", "content": """**Initial Assessment** Evaluation begins with rapid assessment of consciousness level and vital signs. Arousal is assessed by observing spontaneous activity and response to stimulation. Awareness is evaluated by testing orientation to person, place, and time where age-appropriate. **Focused Neurologic Examination** Pupils are examined for size, symmetry, and reactivity. Extraocular movements are tested for impairment. Motor function is assessed for focal weakness or asymmetry. Reflexes are checked for asymmetry. Meningeal signs (nuchal rigidity, Kernig sign) are sought. Abnormal posturing, seizure activity, or involuntary movements are observed for. **Red Flag Findings Suggesting Structural Pathology** - Asymmetric or dilated pupils - Cranial nerve VI palsy - Focal weakness or asymmetrical reflexes - Abnormal posturing - Cushing triad (bradycardia, [[92|hypertension]], Cheyne-Stokes breathing) **Red Flag Findings Suggesting Infection** - Fever with headache, photophobia, and nuchal rigidity - Poor perfusion with fever ([[226|sepsis]]) **Red Flag Findings Suggesting Trauma** - History of head injury - Scalp or skull hematoma or ecchymoses - Retinal hemorrhages (concerning for nonaccidental trauma)"""}, {"title": "Diagnosis", "content": """**Next Steps** Based on clinical presentation, appropriate investigations are obtained: laboratory tests for metabolic causes (uremia, sepsis), lumbar puncture for CNS infection, neuroimaging (CT or MRI) for structural lesions, and EEG if seizure is suspected."""}, {"title": "Management", "content": """Management is directed at the underlying cause once identified."""}, ] NEW[143] = [ {"title": "Clinical paths", "content": """**Immediate Actions** 1. Airway, breathing, and circulation are assessed 2. Emergency help is called for (911/EMS in community settings; resuscitation team in healthcare settings) 3. The patient is positioned on their back or in a position of comfort if [[151|respiratory distress]] or vomiting is present 4. The lower extremities are elevated 5. Standing, walking, or running is not allowed"""}, {"title": "Diagnosis", "content": """The severity of the reaction is determined by the initial assessment of airway, breathing, and circulation."""}, {"title": "Management", "content": """**Medication** **Epinephrine (adrenaline)** is the primary initial treatment: - Route: Intramuscular injection in the mid-outer aspect of the thigh - Administration: An epinephrine auto-injector (EA) is used - Second dose: May be given 5 to 15 minutes after the first injection if needed **Additional Supportive Care** - Supplemental oxygen - Intravenous fluids - Corticosteroids - H1 antihistamines - H2 antihistamines **Transport** The patient is transported to an emergency department, preferably by EMS vehicle, for further assessment and monitoring. Continued observation is essential because biphasic reactions can occur."""}, ] NEW[144] = [ {"title": "Clinical paths", "content": """**Step 1: Initial Recognition and Stabilization** Feeding intolerance is identified by the presence of increased gastric residual volume, bilious or blood-stained stomach contents, abdominal distension, vomiting, diarrhea, bloating, or signs of microaspiration. **Step 3: Decision Point Based on Clinical Course** *If no worrying symptoms and intolerance resolves:* - Enteral feeding is restarted - Step 4 follows *If worrying symptoms develop or clinical condition worsens:* - Enteral nutrition is withdrawn - Observation continues for at least 48–72 hours - Once resolved, Step 5 follows"""}, {"title": "Diagnosis", "content": """When worrying symptoms develop during feeding intolerance, abdominal radiography and ultrasound are performed, with imaging repeated every 6 hours."""}, {"title": "Management", "content": """**Step 2: Withhold and Observe** Enteral nutrition is withheld for 6–12 hours. Close observation is maintained for worrying symptoms (signs of necrotizing enterocolitis, sepsis, or acute deterioration). **Step 4: Minimal Enteral Feeding (if intolerance persists without worrying symptoms)** - Enteral feeding is restarted at 50% of the previous volume - Observation continues for at least 24 hours - If intolerance persists or reappears without worrying symptoms, Step 5 follows - If no intolerance, feeding advances **Step 5: Feeding Regimen Modifications** One or more of the following are adjusted: - **Schedule:** Bolus feeding is changed to continuous feeding - **Rate:** The infusion rate is decreased - **Concentration:** The formula is concentrated to decrease volume - **Formula:** Changing to a different formula is considered - **Fluid intake:** Adequate hydration is ensured **Step 6: Medical Management** Considered: - Acid-suppressing therapy for [[245|gastroesophageal reflux]] - Prokinetic agents to enhance gastric motility **Step 7: Advancement After Resolution** Once feeding intolerance resolves, enteral nutrition is increased by 20–30 ml/kg/day. **Ongoing Monitoring** A multidisciplinary team (physician and registered dietitian nutritionist): - Monitors enteral feeding tolerance at each assessment - Tracks growth trends - Reviews relevant laboratory values - If the child is also eating by mouth, evaluates the combination of tube and oral feedings to ensure adequate nutrient intake"""}, ] NEW[145] = [ {"title": "Clinical paths", "content": """**Initial Assessment** 1. A detailed history is taken and a complete physical examination is performed 2. Red flag symptoms and signs are identified: unintentional weight loss, blood in stools, persistent right upper or lower abdominal pain, persistent vomiting, dysphagia, odynophagia, arthritis, family history of [[254|inflammatory bowel disease]], or nocturnal diarrhea"""}, {"title": "Diagnosis", "content": """Directed laboratory studies and imaging are arranged as indicated when red flags are present. When no red flags are present: 1. Limited screening laboratory studies are performed"""}, {"title": "Management", "content": """**If Red Flags Are Present** If symptoms are consistent with [[193|acute abdomen]]: - The patient is kept nothing by mouth - Surgical consultation is requested - Antibiotics are started: - **Piperacillin/tazobactam**: 350 mg/kg/day divided into doses every 6 hours (maximum 12 g/day), OR - **IV meropenem**: 20 mg/kg every 8 hours (maximum 6 g/day), OR - **Cefoxitin**: 30 mg/kg every 6 hours (maximum 12 g/day), OR - **Cefotetan**: 30 mg/kg every 12 hours (maximum 12 g/day) **If No Red Flags Are Present** 2. Parents are reassured that no organic basis for pain exists 3. Placebo medications are not prescribed 4. Behavioral and psychological approaches are counselled: - Relaxation techniques (deep breathing exercises) - Distraction strategies (conversation, television, games) - Identification and management of psychosocial stressors 7. Referral and consultation are considered as indicated by clinical context"""}, {"title": "Prognosis and outcome", "content": """5. Symptoms typically improve on weekends and vacations 6. Most children improve with coping strategies alone"""}, ] NEW[146] = [ {"title": "Clinical paths", "content": """[[334|Syncope]] (especially exertional) is evaluated for as a major risk factor for sudden cardiac death. Patients at increased risk for sudden cardiac death are identified based on risk stratification."""}, {"title": "Diagnosis", "content": """**Initial Assessment** - ECG and echocardiography are obtained to confirm diagnosis and assess for LVOT obstruction - Exercise stress testing is performed as part of risk stratification - Family history is obtained and genetic counseling is arranged"""}, {"title": "Management", "content": """**Pharmacological Management** - Negative inotropic agents are initiated: - Beta-blockers (first-line) - Calcium channel blockers (alternative) - Adequate hydration is maintained; HCM is preload dependent and patients may benefit from higher rates of fluid administration - Positive inotropes, medications that increase heart rate, and afterload-reducing agents are avoided **Activity and Lifestyle** - Moderate restriction of physical activity is implemented - Competitive athletics are counselled against - Genetic counseling is provided and family member evaluation is recommended **Risk Stratification and Intervention** - Implantable cardioverter-defibrillator placement is considered in high-risk patients - Symptomatic patients with subaortic obstruction are referred for possible myectomy **Follow-up** - Ongoing [[121|pediatric cardiology]] follow-up is arranged - Risk stratification is reassessed periodically"""}, ] NEW[147] = [ {"title": "Clinical paths", "content": """Any febrile child with a purpuric rash receives intramuscular benzylpenicillin immediately and is transferred urgently to hospital without waiting for confirmatory tests."""}, {"title": "Diagnosis", "content": """Imaging (CT or MRI) is obtained before lumbar puncture to exclude hydrocephalus or mass lesion. Lumbar puncture is performed and cerebrospinal fluid is sent for Gram stain, culture, and analysis. In patients with ventriculoperitoneal shunts, cerebrospinal fluid is obtained via shunt tap if present. **Antibiotic Susceptibility Considerations** For *Streptococcus pneumoniae* meningitis, penicillin and cephalosporin susceptibility are interpreted using meningitis-specific breakpoints: - Penicillin (intravenous): susceptible ≤0.06 mcg/mL; resistant ≥0.12 mcg/mL (no intermediate category) - Cefotaxime or ceftriaxone (intravenous): susceptible ≤0.5 mcg/mL; intermediate 1 mcg/mL; resistant ≥2 mcg/mL"""}, {"title": "Management", "content": """**Antibiotic Therapy** For shunt-related meningitis in patients who are seriously ill, vancomycin and a third-generation cephalosporin (cefotaxime or ceftriaxone) are initiated to cover Staph aureus, coagulase-negative staphylococci (including methicillin-resistant strains), and gram-negative rods. In less seriously ill patients without shunt infection, therapy may be postponed while awaiting Gram stain and culture results. **Supportive Care** - General and supportive measures are provided as indicated - Monitoring occurs for syndrome of inappropriate antidiuretic hormone, which may accompany meningitis - Increased intracranial pressure from cerebral edema or hydrocephalus is managed"""}, ] NEW[148] = [ {"title": "Clinical paths", "content": """**Initial Assessment** 1. A detailed menstrual history is obtained, including age at menarche, cycle regularity, and duration of amenorrhea 2. Pubertal development is assessed (breast and pubic hair staging) 3. Confidential sexual history is taken and pregnancy is screened for 4. Medications, hormonal contraceptive use, exercise intensity, dietary habits, stress, and systemic illness are inquired about"""}, {"title": "Diagnosis", "content": """**First-Line Investigations** The following tests are ordered: - Thyroid-stimulating hormone (TSH) - Prolactin - Follicle-stimulating hormone (FSH) - Human chorionic gonadotropin (HCG) - Pelvic ultrasound (for [[394|primary amenorrhea]]) **Interpretation and Next Steps** **If FSH is low or normal:** - Suggests hypothalamic or pituitary dysfunction - Eating disorders, excessive exercise, stress, systemic illness, and constitutional delay are evaluated for - Screening for celiac disease and other autoimmune disorders is considered **If FSH is elevated:** - Suggests ovarian insufficiency - FSH testing is repeated - Celiac and other autoimmune disorders are evaluated for - Karyotype analysis is considered - Pelvic and adrenal imaging are ordered **Bone Health Assessment** After 6 months or more of amenorrhea, bone densitometry is performed to assess for low bone density, particularly in patients with functional hypothalamic amenorrhea or relative energy deficiency in sport."""}, {"title": "Management", "content": """When FSH is elevated, suggesting ovarian insufficiency, specialist referral is arranged. **Management of Hormonal Contraceptive-Related Amenorrhea** Amenorrhea occurring during hormonal contraceptive use does not require immediate investigation. However, if amenorrhea persists for 12 months after the last injection of medroxyprogesterone or for 6 months after discontinuation of birth control pills, rings, or patches, standard evaluation as outlined above is undertaken."""}, ] NEW[149] = [ {"title": "Clinical paths", "content": """Detailed dietary history is taken and risk factors are assessed."""}, {"title": "Diagnosis", "content": """**Diagnosis and confirmation:** - 24-hour urine niacin measurement is obtained - RBC NAD/NADP number is measured"""}, {"title": "Management", "content": """**Treatment:** - Niacin: 50–100 mg per dose, orally, three times daily for several weeks - Dietary sources are ensured to be adequate: beef, liver, fish, pork, wheat flour, eggs - Underlying causes are addressed (e.g., medication review for isoniazid, anticonvulsants, antidepressants, 5-fluorouracil, 6-mercaptopurine, chloramphenicol, sulfonamides) **Monitoring:** - Resolution of gastrointestinal symptoms (diarrhea, anorexia) is assessed - Skin changes (dermatitis, sun-exposed areas) are monitored - Neurological improvement (dementia, glossitis, angular stomatitis) is evaluated"""}, ] NEW[150] = [ {"title": "Clinical paths", "content": """Staphylococcal pneumonia requires prompt hospitalization. Pertussis requires hospitalization for young infants or any child with significant paroxysms."""}, {"title": "Diagnosis", "content": """**Diagnostic approach:** Chest radiography should be performed to evaluate for complications such as empyema, lung abscess, [[396|pneumothorax]], or pneumomediastinum. Blood cultures should be obtained, particularly in severe or bacteremic cases. In neonates, ultrasonography may show absent, irregular, disrupted, or coarse pleural line; parenchymal hepatization with air bronchograms; or > 3 B-lines or areas of white lung."""}, {"title": "Management", "content": """**Neonatal pneumonia (< 3 weeks):** Treatment includes supportive care and specific antibiotic therapy. Standard regimens are: - Ampicillin or cloxacillin with gentamicin - For hospital-acquired infection: cephalosporins with amikacin **Older children with suspected bacterial pneumonia:** Inpatient therapy for pneumococcal pneumonia is generally ceftriaxone or cefotaxime. **Staphylococcal pneumonia:** Treatment options are: - Nafcillin (for methicillin-sensitive S. aureus) - Vancomycin (if high community prevalence of MRSA, recent hospitalization, indwelling catheter, or tracheostomy) Pneumothoraces require decompression. **[[125|Pertussis]]:** Antibiotics are required for organism eradication."""}, ] NEW[151] = [ {"title": "Clinical paths", "content": """**Initial Assessment and Stabilization** 1. **Airway, Breathing, Circulation (ABC)** — This is the first priority - Airway patency is assessed urgently - Breathing adequacy is determined - Circulation is evaluated **Concurrent Assessment** Severity and type of respiratory problem are determined simultaneously."""}, {"title": "Diagnosis", "content": """Laboratory studies (CBC, arterial blood gas, serum electrolytes, blood culture) are obtained as indicated. Chest radiography is performed. Detailed evaluation is pursued to determine underlying etiology."""}, {"title": "Management", "content": """2. **Positioning and Airway Clearance** - The child is positioned appropriately - Oronasal suctioning is performed if needed to clear secretions 3. **Oxygen Delivery** - Supplementary oxygen is provided as needed - Oxygen hood or nasal prongs are used - Oxygen saturation (SpO₂) is monitored 4. **Intubation** - Intubation is performed if airway patency cannot be maintained - Note: Children have significant anatomical variation with age—shorter trachea, smaller tracheal diameter, and technically difficult intubation; risk of right mainstem intubation, tube dislodgement, or esophageal intubation **Supportive Measures** 5. **Temperature Management** - Normal temperature is maintained - Hypothermia or hyperthermia is corrected 6. **Fluid and Metabolic Support** - Intravenous fluid boluses are administered - Hypoglycemia is corrected 7. **Respiratory Support** - CPAP (continuous positive airway pressure) is considered - Nebulization with bronchodilators is given as indicated - Positive pressure ventilation is used for severe cases 8. **Specific Therapy for RDS** - Surfactant replacement therapy is given for confirmed RDS - Positive pressure ventilation is used for severe cases 9. **Drainage** - Intercostal tube drainage is placed if air or fluid is present"""}, ] NEW[152] = [ {"title": "Clinical paths", "content": """**Tuberculosis Meningitis** Lumbar puncture is performed in all children <12 months with suspected TB disease; in children ≥12 months only if neurological signs or symptoms are present."""}, {"title": "Diagnosis", "content": """**Tuberculosis Meningitis** Chest radiography and TST/IGRA are obtained. Cerebrospinal fluid is sent for culture and acid-fast bacilli examination. **Drug Susceptibility Testing** Performed on organism recovered from patient or mother. **Congenital Tuberculosis** **Immediate Evaluation** - Tuberculin skin test - Interferon-gamma release assay - Chest radiography - Lumbar puncture - Appropriate cultures - Placental histology for granulomata and acid-fast bacilli - Placental culture for *M. tuberculosis* complex **Maternal Evaluation** Pulmonary and extrapulmonary tuberculosis, including genitourinary disease, are assessed for. HIV testing is performed (essential). Drug susceptibility testing is performed on maternal isolate."""}, {"title": "Management", "content": """**Tuberculosis Meningitis** **Drug-Susceptible *M. tuberculosis* Meningitis** *Intensive phase (2 months):* - Isoniazid, once daily - Rifampin, once daily - Pyrazinamide, once daily - Aminoglycoside OR ethionamide, once daily *Continuation phase (7–10 months):* - Isoniazid, once daily or twice weekly - Rifampin, once daily or twice weekly **Total duration:** 9–12 months **Drug-Susceptible *M. bovis* Meningitis** - At least 12 months of therapy without pyrazinamide **Geographic Considerations** - In areas with common streptomycin resistance, kanamycin, amikacin, or capreomycin is substituted for aminoglycoside **Adjunctive Therapy** - Corticosteroids are added if [[147|meningitis]] is confirmed **Congenital Tuberculosis** **Treatment (initiated promptly regardless of TST/IGRA results)** - Rifampin, once daily - Isoniazid, once daily - Pyrazinamide, once daily - Either ethambutol (RIPE regimen) OR aminoglycoside (streptomycin, kanamycin, or amikacin) OR capreomycin, once daily **If Meningitis Confirmed** - Corticosteroids are added"""}, ] NEW[153] = [ {"title": "Clinical paths", "content": """**Suspicion and History** Anorexia nervosa is suspected when an adolescent presents with: - Restricted energy intake and fear of weight gain - Unwillingness or inability to maintain body weight above minimally normal BMI - Distorted attitudes and behaviors about eating or body image - Infrequent or absent menses - Compulsive exercising - Refusal to eat in front of others **Physical Examination** The following are assessed: - Vital signs: bradycardia, hypotension, hypothermia, orthostasis (pulse increase >20 beats on standing or systolic BP drop >20 mm Hg on standing) - Skin: dryness, lanugo - Hair: thinning or alopecia - Head and neck: parotid or submandibular gland swelling - Hands: calluses on dorsum (if purging) - Teeth: erosion (if purging) - Breasts: atrophy (in females) **Hospitalization Indications** Hospitalization is indicated if any of the following are present: - Severe [[214|malnutrition]]: BMI <14–15 kg/m² or BMI z-score ≤−3 - Severe bradycardia - Hypotension - Hypothermia - Prolonged QTc interval - Electrolyte abnormalities - Acute food refusal - Failure of outpatient management"""}, {"title": "Diagnosis", "content": """**Investigations** - Metabolic panel - Urinalysis - Pregnancy test (females of childbearing age) - Erythrocyte sedimentation rate, celiac panel, or other tests to exclude systemic disease - Electrocardiogram (assess QTc interval)"""}, {"title": "Management", "content": """**Interdisciplinary Team** Care should be provided by an interdisciplinary treatment team including a pediatrician or medical provider. **Refeeding Syndrome Monitoring** Patients with severe malnutrition are at risk for refeeding syndrome during nutritional rehabilitation. Although initial serum magnesium, potassium, and phosphorus concentrations may be normal, these electrolytes can fall to life-threateningly low levels during refeeding, potentially causing serious complications such as arrhythmias or heart failure. **Electrolyte Monitoring** Close monitoring of electrolytes is required in patients at risk for refeeding syndrome. Specific monitoring intervals and target ranges are not detailed in the available passages."""}, ] NEW[154] = [ {"title": "Clinical paths", "content": """Weight status is assessed at routine visits, and recommendations are discussed with patients and families. Note that BMI has limited utility in assessing body fatness in athletes with high muscle mass or muscular habitus."""}, {"title": "Diagnosis", "content": """**Initial Assessment** BMI is calculated and plotted on age- and sex-specific CDC growth charts to determine percentile classification: - BMI 85th–94th percentile: overweight - BMI ≥95th percentile: [[257|obesity]] - BMI ≥120% of 95th percentile: severe obesity For children with obesity, severity is further classified: - Class 1: BMI 95th percentile to <120% of 95th percentile - Class 2: BMI 120–140% of 95th percentile or BMI ≥35 - Class 3: BMI ≥140% of 95th percentile or BMI ≥40"""}, {"title": "Management", "content": """Guidance is provided on: - Dietary practices, including promotion of breastfeeding in early infancy - Physical activity - Growth chart monitoring **Treatment Approach** Nutritional interventions form the cornerstone of obesity management. In most cases, treatment of obesity and its associated health conditions (hypercholesterolemia, hypertension, nonalcoholic fatty liver disease, prediabetes, type 2 diabetes) involves similar nutritional best practices. **Prevention Focus** Early identification and intervention are emphasized, particularly during ages 2–6 years when the most excessive weight gain typically occurs. Close tracking of growth charts enables recognition of risk factors and allows for timely, tailored anticipatory guidance before obesity becomes established."""}, ] NEW[155] = [ {"title": "Clinical paths", "content": """**Initial Assessment** When congenital infection is suspected, a detailed maternal history is obtained, including primary infection timing, serologic status, and exposure risks. Thorough physical examination is performed, documenting growth parameters, hepatosplenomegaly, rash characteristics, jaundice, and neurologic findings."""}, {"title": "Diagnosis", "content": """**Diagnostic Workup** For suspected [[341|toxoplasmosis]]: - Newborn and maternal serology are obtained: toxoplasma-specific IgG, IgM, IgA, and IgE - Infant blood, cerebrospinal fluid, and amniotic fluid are sent to a reference laboratory for PCR detection of *Toxoplasma gondii* - Lumbar puncture is performed For suspected CMV: - Cord blood or infant urine is tested for CMV by PCR or culture - CMV-specific serology is obtained if indicated For suspected HHV-6: - HHV-6A or HHV-6B DNA is detected in cord blood by PCR **Interpretation of Serologic Results** Congenital infection is confirmed serologically by: - Persistent or increasing IgG antibody levels in infant compared to mother - Persistently positive IgG antibodies beyond the first year of life - Positive *Toxoplasma*-specific IgM or IgA antibody in infant - Persistence of other antibody types also indicates infant infection **Imaging and Additional Studies** For symptomatic congenital CMV, neuroimaging is obtained to assess for periventricular calcifications, cerebral atrophy, and hydrocephalus. Ophthalmologic examination is performed to detect chorioretinitis."""}, {"title": "Management", "content": """For suspected toxoplasmosis, thorough ophthalmologic, auditory, and neurologic evaluation is arranged. For symptomatic congenital CMV, auditory assessment including formal audiometry is arranged."""}, ] NEW[156] = [ {"title": "Clinical paths", "content": """**Initial Assessment** The hemangioma is identified by location (superficial vs. deep), size, and appearance. Superficial lesions are bright red; deep lesions appear bluish. The age of onset and growth pattern are documented. Any functional impairment or complications are assessed for. Parents are educated about the potential for complications or permanent disfigurement and the rationale for watchful waiting versus intervention."""}, {"title": "Diagnosis", "content": """Imaging is typically reserved for deeper subcutaneous lesions without typical skin findings or when diagnosis is unclear."""}, {"title": "Management", "content": """**Treatment Options** **Topical therapy:** Topical timolol maleate may be prescribed for thin and/or superficial infantile hemangiomas. **Surgical and laser therapy:** Considered for selected hemangiomas causing functional impairment or significant disfigurement. Surgery is avoided during the proliferating phase due to high vascularity and risk of blood loss. The optimal timing for surgical resection is between 3 and 4 years of age, when the lesion has stabilized and before the child's long-term memory and self-esteem formation begins. **Follow-up** Lesions are monitored clinically during the proliferating and involuting phases."""}, {"title": "Prognosis and outcome", "content": """Parents are educated about the expected natural history: rapid growth over the first 3–4 months, plateau by 9–12 months, then gradual involution beginning after 12 months and typically completing by 4 years of age."""}, ] NEW[157] = [ {"title": "Clinical paths", "content": """**Assessment** Worrisome lymphadenopathy features are evaluated for: systemic symptoms (fever, night sweats, weight loss), fixed nontender nodes, and supraclavicular involvement. Lymphoma is considered in patients older than 3 years presenting with intussusception or abdominal symptoms (crampy pain, vomiting, obstruction, bleeding, palpable mass)."""}, {"title": "Diagnosis", "content": """Risk stratification includes consideration of bone marrow and central nervous system involvement at presentation."""}, {"title": "Management", "content": """**Treatment by Disease Extent** *Localized disease with complete surgical resection:* - Multiagent chemotherapy over 6 weeks - COPAD regimen: two cycles of cyclophosphamide, vincristine, prednisone, and doxorubicin *Advanced disease:* - Multiagent chemoimmunotherapy regimen - Duration: 4 to 6 months - Examples: FAB/LMB 96 protocol therapy or COG ANHL01P1 (which includes rituximab) - Treatment phases typically include reduction, induction, intensification, and maintenance therapy *Gastrointestinal lymphoma:* - Combination of surgical resection and chemotherapy **Risk Stratification** Treatment intensity and specific protocols are adjusted based on risk stratification."""}, {"title": "Prognosis and outcome", "content": """For localized disease with complete surgical resection treated with the COPAD regimen, expected 4-year overall survival is 99%. For advanced disease treated with multiagent chemoimmunotherapy, 4-year overall survival is 95%."""}, ] NEW[158] = [ {"title": "Clinical paths", "content": """Secondary bacterial infections ([[160|otitis media]], bronchopneumonia, croup, diarrhea) occur commonly in young children and immunocompromised hosts."""}, {"title": "Diagnosis", "content": """**Diagnosis and Confirmation** - IgM assay is obtained for serologic confirmation - PCR or virus isolation from urine, blood, or throat/nasopharyngeal secretions is considered if IgM is unavailable"""}, {"title": "Management", "content": """**Isolation Precautions** - Standard and airborne precautions are implemented for all hospitalized patients - Isolation is maintained from 7 days after exposure through 4–6 days after rash onset - For immunocompromised patients: isolation continues throughout the entire illness as viral shedding is prolonged - Exposed hospitalized patients: isolation occurs for 5–21 days following exposure **Supportive Care** - Fever and symptoms are managed with supportive measures - Secondary bacterial infections are monitored for - Complications including encephalitis and hemorrhagic manifestations are assessed for **Exposure Management** - Susceptible individuals should avoid contact with infected patients during the infectious period - Unvaccinated or inadequately vaccinated close contacts require post-exposure evaluation and vaccination consideration"""}, ] NEW[159] = [ {"title": "Clinical paths", "content": """When a neonate presents with suspected seizure activity, immediate evaluation is essential. True seizures are distinguished from neonatal mimics such as jitteriness or benign sleep myoclonus (which occurs only during sleep and resolves on awakening)."""}, {"title": "Diagnosis", "content": """**Identification of Reversible Causes** Before antiseizure medication is started, electrolyte abnormalities and hypoglycemia are identified, as these are rapidly reversible causes: - **[[321|Hypoglycemia]]**: Thresholds vary by postnatal age: <25–40 mg/dL in first 4 hours, <35–45 mg/dL from 4–24 hours, <45 mg/dL from 24–48 hours, <60 mg/dL thereafter - **[[368|Hypocalcemia]]**: Defined as calcium <8 mg/dL in infants >1,500 g birthweight; <7 mg/dL in very low birthweight infants - **Hypomagnesemia**: Assessed as indicated **Diagnostic Workup** Concurrently with seizure management, investigation of underlying cause is pursued based on clinical presentation and timing of seizure onset. This may include neuroimaging, cerebrospinal fluid analysis, metabolic screening, and infectious disease evaluation as clinically indicated."""}, {"title": "Management", "content": """Adequate oxygenation and ventilation are ensured. Electrolyte abnormalities and hypoglycemia are treated urgently, as these are rapidly reversible causes; hypomagnesemia is corrected as indicated. **Pharmacological Management** For infrequent or transient seizures: - **Diazepam**: 0.1–0.5 mg/kg IV - **Lorazepam**: 0.1 mg/kg IV If seizures do not stop, occur more frequently, or become more severe, therapy is escalated. Respiratory support (ventilator in ICU setting) must be available whenever IV benzodiazepines or phenobarbital are administered. For ongoing or repeated seizures requiring sustained control, antiseizure medications are used, though their efficacy in suppressing seizures is considerably poorer in neonates than in older children. For refractory seizures, general anesthesia with endotracheal intubation is required. Agents used in this setting include continuous infusion of midazolam, propofol, ketamine, or pentobarbital."""}, ] NEW[160] = [ {"title": "Clinical paths", "content": """Supporting features include rapid symptom onset, fever, otalgia or ear tugging, and recent upper respiratory tract infection history."""}, {"title": "Diagnosis", "content": """**Diagnosis at the bedside** The diagnosis is confirmed using pneumatic otoscopy. Findings sought include: - Moderate to severe bulging of the tympanic membrane, OR - New-onset otorrhea not due to acute [[316|otitis externa]] **Special note** Tympanocentesis is not performed routinely; treatment is empirical. Clinical features cannot reliably predict causative organism in individual children."""}, {"title": "Management", "content": """**First-line treatment** Amoxicillin (oral): **80–90 mg/kg/day** for at least 7 days Alternative: Ceftriaxone (single dose) in certain cases **Second-line agents** (if first-line unsuitable): - Coamoxiclav - Cefaclor - Cefuroxime - Newer-generation cephalosporins - Macrolides (for penicillin and/or cephalosporin allergy) **Follow-up** - Otoscopic examination is repeated at 3–4 days - Otoscopic examination is repeated at 3 weeks **Not prescribed** - Oral or topical decongestants (not necessary) - Antihistamines (ineffective and may precipitate sinus infection) - Antibiotics for [[258|otitis media with effusion]]"""}, ] NEW[161] = [ {"title": "Clinical paths", "content": """## Escalation Any child requiring more than 40 mL/kg as a bolus should be urgently reviewed for need of vasopressor or inotropic support and consideration of intensive care. ## Poor response to resuscitation Lack of response to initial resuscitation or persistently poor perfusion despite multiple boluses suggests an underlying problem such as [[178|septic shock]], toxic shock syndrome, [[100|myocarditis]], myocardiopathy, or pericarditis."""}, {"title": "Diagnosis", "content": """## Laboratory assessment **Electrolytes:** Serum electrolyte levels, BUN, and creatinine are determined."""}, {"title": "Management", "content": """## Immediate resuscitation phase **Vascular access:** A large-bore intravenous catheter is established. If IV access cannot be obtained, intraosseous access is used. **Initial fluid bolus:** 20 mL/kg of isotonic fluid (normal saline or lactated Ringer solution) is administered over approximately 20 minutes. **Fluid choice:** - **Normal saline or lactated Ringer solution** is used for initial resuscitation - In children with isolated vomiting and probable metabolic alkalosis, **normal saline** is used (lactated Ringer solution or Plasma-Lyte is avoided) - Lactated Ringer solution or Plasma-Lyte may be preferable to normal saline in shock as balanced solutions **Reassessment and repeat boluses:** Vital signs are monitored closely. Repeat boluses of 20 mL/kg may be needed. The child may require multiple boluses administered as rapidly as possible. Up to 20–100 mL/kg total may be required to restore plasma volume and pulses. **Monitoring:** Return of normal pulse and state of consciousness is assessed. Hypotension and orthostatic vital sign changes are monitored. Peripheral perfusion and mental status are evaluated. **Urine output:** Urine output is monitored; normal output is approximately 1 mL/kg/h. If the patient does not void after 3 fluid boluses, a bladder catheter is inserted. **Potassium:** Intravenous potassium is withheld until urine output is established. ## Deficit replacement phase Once shock is reversed and mental status is satisfactory, the remaining fluid deficit is replaced over 12–24 hours using isotonic fluid. Dextrose and potassium are added to ongoing fluids as appropriate. ## Transition to oral rehydration When the child's condition has stabilized and mental status is satisfactory, oral rehydration therapy is initiated. The intravenous line is maintained until oral intake is adequate. **Alternative:** Enteral hydration via nasogastric tube is considered for patients who refuse oral intake but do not have ongoing vomiting. Broad-spectrum antibiotics and vasopressors are given, and the patient is transferred to an intensive care unit."""}, ] NEW[162] = [ {"title": "Clinical paths", "content": """**Assessment and Initial Evaluation** Children presenting with suspected or known sickle cell disease require rapid assessment for acute complications, as life-threatening conditions can develop quickly. A detailed history of pain location and severity, fever, respiratory symptoms, and any recent infections or triggers is obtained. A thorough physical examination is performed, including vital signs, assessment for acute chest syndrome (chest pain, dyspnea, cough with pulmonary consolidation), splenic enlargement, and signs of sepsis. **Acute Presentations** Acute presentations may include: - Vaso-occlusive pain crises (musculoskeletal and abdominal) - Acute chest syndrome - Splenic sequestration - Bacterial [[226|sepsis]] or [[147|meningitis]] - Stroke - Priapism Rapid assessment and intervention are essential as complications can become life-threatening within hours."""}, {"title": "Diagnosis", "content": """**Diagnostic Approach** For newly identified cases or confirmatory diagnosis: - Hemoglobin electrophoresis - High-performance liquid chromatography (HPLC) - Immunologic tests - Molecular genetic testing Note: If the child has received a blood transfusion, testing is delayed until more than 90 days after transfusion."""}, {"title": "Management", "content": """**Comprehensive Care Framework** All children with sickle cell disease require coordinated comprehensive care through a medical home with appropriate expertise. This includes: - **Ongoing education** for patient and family regarding disease management, pain recognition, and when to seek emergency care - **Periodic comprehensive evaluations** to monitor for complications - **Disease-specific health maintenance services** tailored to prevent and detect complications early - **Psychosocial support** to address the emotional and social impact of chronic illness - **Genetic counseling** for family members **Acute Illness Management** Timely and appropriate treatment of acute complications is critical."""}, ] NEW[163] = [ {"title": "Clinical paths", "content": """**Prenatal Recognition** Increased nuchal translucency on early prenatal ultrasound may raise suspicion for trisomy 18. Combined screening protocols incorporating maternal age, nuchal translucency, and maternal serum markers enable early detection. **Postnatal Assessment** At birth, examination is performed for characteristic dysmorphic features: overlapping fingers in clenched fist, short sternum, rocker-bottom feet, prominent occiput, and low-set ears. Reduced birth weight and small placenta are documented. Signs of fetal distress are assessed."""}, {"title": "Diagnosis", "content": """**Cardiac Evaluation** Congenital heart disease is present in the majority of cases. Echocardiography is obtained to identify structural lesions (VSD, PDA, ASD, polyvalvular disease). **Diagnostic Confirmation** G-banded karyotype analysis is the study of choice to confirm diagnosis and determine recurrence risk. This is particularly important if translocation is suspected, as recurrence risk is higher for translocation carriers and depends on the chromosomes involved and the sex of the carrier parent."""}, {"title": "Management", "content": """Signs of heart failure and pulmonary hypertension are monitored. **Feeding and Nutrition** Poor feeding is common. Nasogastric tube feeding is often necessary. Growth charts specific to trisomy 18 should be used for monitoring. **Respiratory Support** Respiratory monitoring and support are provided as clinically indicated."""}, {"title": "Prognosis and outcome", "content": """Central apnea and hypoventilation are major causes of mortality."""}, ] NEW[164] = [ {"title": "Clinical paths", "content": """**Initial Assessment** A detailed physical examination is performed, including: - Assessment of muscle tone (increased, variable, or decreased) - Evaluation of primitive reflexes (persistence suggests abnormality) - Observation of posture, gait, and spontaneous movements - Testing of reflexes and coordination - Screening for associated features: visual function, hearing, speech, feeding ability, seizure history"""}, {"title": "Diagnosis", "content": """**Early Identification** Standardized assessments are recommended for early detection: - **General Movements Assessment (GMA)**: assess spontaneous motor patterns in infants - **Hammersmith Infant Neurological Examination (HINE)**: comprehensive neurological evaluation - These tools enable identification within the first few months to one year of life **Neuroimaging** Neuroimaging may be performed to support diagnosis and identify structural abnormalities or evidence of prior injury (such as [[299|intraventricular hemorrhage]] or periventricular leukomalacia in premature infants). **Functional Classification** Motor severity is classified using: - **Gross Motor Function Classification Scale (GMFCS)**: ranges 1–5, valid and reliable particularly in children >2 years; guides therapy planning and prognostic counseling - **Manual Ability Classification Scale (MACS)**: assesses hand function"""}, {"title": "Management", "content": """**Multidisciplinary Management** A coordinated care approach is implemented: - **Early intervention services**: access as soon as diagnosis is confirmed to leverage neuroplasticity - **Physiotherapy**: goal-directed and functional training tailored to GMFCS level - **Assistive technology and orthoses**: splints and orthoses to support positioning and prevent contractures - **Feeding and swallowing support**: assessment and intervention for feeding difficulties - **Seizure management**: antiepileptic therapy if [[315|seizures]] present - **Family education**: key messages are provided regarding realistic expectations, functional goals, and quality of life **Monitoring for Complications** Regularly assessed for: - Development of contractures (initially dynamic, may become fixed) - Orthopedic deformities - Nutritional status - Sleep disturbances - Behavioral and cognitive concerns - Sensory impairments (vision, hearing) - Autonomic dysfunction or incontinence"""}, ] NEW[165] = [ {"title": "Clinical paths", "content": """Infants at risk are identified through newborn screening programs. Congenital [[171|hypothyroidism]] should be considered in any infant presenting with significant constipation, prolonged jaundice, hypotonia, or hypothermia."""}, {"title": "Diagnosis", "content": """Diagnosis is confirmed with TSH measurement ideally after 48 hours of age (minimum 24 hours). TSH elevation above a threshold of 20 μL indicates congenital hypothyroidism. In suspected central hypothyroidism, clinical suspicion is required as TSH-based screening may miss these cases."""}, {"title": "Management", "content": """**Treatment Initiation** Levothyroxine replacement therapy is begun as quickly as possible after diagnosis, with the goal of initiating treatment by 2 weeks of age. The passages do not provide specific dosing information for levothyroxine. **Monitoring and Follow-up** Resolution of clinical manifestations is monitored. Neonatal cholestasis, if present, typically resolves with appropriate hormone supplementation. Associated [[115|congenital anomalies]], particularly cardiac anomalies, are assessed, and hearing is evaluated. In infants with central hypothyroidism or pan-hypopituitarism, adrenal insufficiency is screened for, as delayed treatment may lead to severe consequences including death."""}, ] NEW[166] = [ {"title": "Clinical paths", "content": """**Initial assessment:** Rapid clinical evaluation is performed to determine if congenital heart disease is the cause. Careful neurological examination is performed to identify complications (hypoxemic spells, stroke, brain abscess). **If signs of [[285|congestive heart failure]] or cardiogenic shock are present:** - Survival depends on maintaining ductus arteriosus patency - Early infusion of prostaglandin E1 (alprostadil) is initiated under carefully controlled monitoring - Emergent cardiology consultation is arranged - Inhaled nitrous oxide or extracorporeal membrane oxygenation is considered in severe cases **If well-appearing but investigations abnormal:** - Urgent referral to a pediatric cardiologist is made - Admission for therapy and cardiac evaluation occurs if the infant is acutely ill"""}, {"title": "Diagnosis", "content": """Pulse oximetry, chest radiography, and electrocardiography are obtained. Echocardiography is arranged for further evaluation."""}, {"title": "Management", "content": """**Ongoing management of cyanotic patients:** - Polycythemia is monitored for - [[99|Dehydration]] is avoided; decreasing or temporarily discontinuing diuretics is considered during [[273|acute gastroenteritis]] or excessively hot weather - High altitudes and sudden thermal environmental changes are avoided - Iron deficiency is treated - Rhythm disturbances and sudden cardiac death risk are monitored for"""}, ] NEW[167] = [ {"title": "Clinical paths", "content": """**Screening and Diagnosis** 1. Candidates are identified: children with family history of FH, family history of premature coronary heart disease, or severely elevated cholesterol in a parent"""}, {"title": "Diagnosis", "content": """2. Fasting lipid profile is measured, including total cholesterol and LDL cholesterol 3. Diagnosis is confirmed if LDL cholesterol is persistently >160 mg/dL (>4.144 mmol/L) or >190 mg/dL (>4.921 mmol/L) with supporting family or clinical history 4. Genetic testing is performed to confirm pathogenic variant in *LDLR*, *APOB*, or *PCSK9*"""}, {"title": "Management", "content": """**Initial Management** 1. Patients are counselled on dietary modification: reduction of total and saturated fat consumption 2. At least 1 hour of physical activity daily is recommended 3. The diet provides appropriate energy for normal growth and sufficient micronutrients 4. Weight management is encouraged in obese patients 5. Dietary changes alone are expected to lower LDL cholesterol by 5–15% **Pharmacological Treatment** 1. Statin therapy is initiated when dietary measures are insufficient 2. Statins are the preferred agents for LDL cholesterol reduction 3. Treatment target: LDL cholesterol reduced by 50% or levels achieved below 130 mg/dL (3.367 mmol/L) 4. Statin therapy is considered starting at age 10 years in children with confirmed FH to achieve long-term cardiovascular risk reduction comparable to unaffected siblings"""}, ] NEW[168] = [ {"title": "Clinical paths", "content": """**Assessment** A clinical history is obtained and examination is performed to identify severity and complications."""}, {"title": "Diagnosis", "content": """Differential diagnoses are considered, including surgical emergencies (intussusception, appendicitis), systemic infections (septicemia, meningitis), and metabolic disorders."""}, {"title": "Management", "content": """**Rehydration** Oral rehydration is the mainstay of treatment for most cases. This is typically sufficient for mild to moderate gastroenteritis. **Antimicrobial Therapy** Antimicrobials are indicated only for: - Severe diarrhea - Wound infection - Septicemia For suspected Vibrio species infection causing severe diarrhea: - Doxycycline or ciprofloxacin - Doxycycline can be used for short durations (21 days or less) without regard to patient age For Vibrio septicemia with or without hemorrhagic bullae, or wound infections: - Third-generation cephalosporin plus either doxycycline or ciprofloxacin - Alternative: trimethoprim-sulfamethoxazole plus an aminoglycoside Wound infections may also require surgical débridement of necrotic tissue if present. **Monitoring** Complications, including reactive arthritis and neurological symptoms (particularly with Campylobacter infection), are monitored for."""}, {"title": "Prognosis and outcome", "content": """Most cases resolve within 4–5 days."""}, ] NEW[169] = [ {"title": "Clinical paths", "content": """Birth mates of a multiple birth index case with early- or late-onset GBS disease should be observed carefully."""}, {"title": "Diagnosis", "content": """**Diagnosis and Initial Assessment** - Blood culture and cerebrospinal fluid (CSF) culture are obtained from any neonate or infant with suspected GBS infection - Gram stain and culture on 5% sheep blood agar are performed; narrow-zone beta-hemolysis is sought - Diagnosis is confirmed by Lancefield group B antigen detection (latex agglutination) or biochemical testing (bacitracin resistance, CAMP factor production, bile esculin negative, trimethoprim-sulfamethoxazole resistance) - White blood cell count and immature-to-total neutrophil ratio are checked"""}, {"title": "Management", "content": """**Treatment** - Parenteral antibiotics are administered for a full 10-day course - Duration is not shortened, nor is treatment switched to oral antibiotics, in uncomplicated cases, as outcome data supporting such approaches are lacking **Special Considerations** - Birth mates are evaluated and treated empirically for suspected systemic infection if signs of illness develop - Full-course treatment is continued in birth mates with confirmed GBS infection"""}, ] NEW[170] = [ {"title": "Clinical paths", "content": """**Step 1: Volume Status Assessment** Volume status is determined clinically — hypovolemic, euvolemic, or hypervolemic."""}, {"title": "Diagnosis", "content": """Volume status is determined using history, physical examination (including weight changes), vital signs, and laboratory data (serum electrolytes, blood urea nitrogen, creatinine, uric acid, urine sodium, specific gravity, osmolality)."""}, {"title": "Management", "content": """**Step 2: Correction Rate** The rise in serum sodium should not exceed 0.5 mEq/L per hour or 6–8 mEq/L per 24 hours unless the patient demonstrates central nervous system symptoms (seizures, altered mental status, coma) that warrant more rapid initial correction. **Step 3: Deficit Calculation and Replacement Plan** Half of the sodium deficit is replenished in the first 8 hours of therapy, and the remainder is given over the following 16 hours. Maintenance and replacement fluids are also provided. Deficit plus maintenance calculations often approximate 5% dextrose with 0.45% or higher saline. **Step 4: Management by Volume Status** **Hypovolemic hyponatremia:** Intravascular volume is first restored with normal saline boluses of 20 mL/kg as needed to correct the volume deficit. Hypotonic fluids are then provided to further replace the water deficit. Note that even normal saline (osmolality 308 mEq/L) is hypotonic relative to hypertonic serum and allows gradual sodium reduction. **Hypervolemic hyponatremia:** Both sodium and water intake are restricted, and the underlying disorder is corrected. If due to water intoxication (characterized by maximally dilute urine with specific gravity <1.003), water is restricted. **Step 5: Monitoring and Avoidance of Complications** Overly rapid correction is avoided, as it can cause osmotic demyelination syndrome, cerebral dehydration, and seizures. Serum sodium is monitored regularly to ensure correction remains within safe limits."""}, ] NEW[171] = [ {"title": "Clinical paths", "content": """**Diagnostic Approach** 1. Serum TSH and free T4 levels are measured - Elevated TSH with low/low-normal free T4 indicates primary hypothyroidism - Normal or low TSH with low free T4 suggests central hypothyroidism"""}, {"title": "Diagnosis", "content": """2. Anti-thyroid antibodies (anti-thyroperoxidase and anti-thyroglobulin) are obtained if autoimmune thyroiditis is suspected 3. Thyroid ultrasound is considered to confirm presence of thyroid tissue, particularly in congenital cases 4. In central hypothyroidism, additional pituitary hormone deficiencies are assessed for and midline defects are evaluated **Neonatal Screening** All newborns require thyroid function testing. If not performed at birth, screening should occur at the first clinical encounter (e.g., vaccination visit). Standard neonatal screening programs typically measure TSH to detect primary hypothyroidism; many do not detect central hypothyroidism."""}, {"title": "Management", "content": """**Monitoring After Treatment Initiation** Growth velocity is followed as a key indicator of adequate thyroid hormone replacement. Normal growth velocity should resume following initiation of therapy."""}, ] NEW[172] = [ {"title": "Clinical paths", "content": """**Initial Assessment** 1. A detailed history of mechanism of injury is obtained, as this guides diagnosis 2. Timing of swelling is assessed: rapid swelling within hours suggests hemarthrosis and serious internal injury 3. Physical examination is performed, recognizing that reliable assessment of ligamentous stability may not be possible with significant swelling"""}, {"title": "Diagnosis", "content": """**Imaging** - Radiographs are obtained to exclude fractures, particularly physeal injuries in younger children - Magnetic resonance imaging is arranged for evaluation of soft tissue and bony injuries when available - Outpatient orthopedic referral is planned for repeat clinical evaluation once swelling has improved"""}, {"title": "Management", "content": """**Immobilization** - Immobilizers are used for patients with significant pain with movement - Prolonged immobilization without repeat evaluation is avoided due to risk of quadriceps atrophy **Bracing and Rehabilitation** - A functional knee brace is applied to enhance proprioception and control terminal extension - Full knee motion within the brace is permitted within a few days - Weight bearing is allowed - A strengthening program is initiated - Bracing is continued until pain and range of motion improve and subjective instability resolves - Functional brace required for return to competition"""}, {"title": "Prognosis and outcome", "content": """**Return to Activity** Most isolated, low-grade medial collateral ligament injuries: return to play in 3–5 weeks. Return to sports for other injuries is variable and depends on severity of tear and associated injuries."""}, ] NEW[173] = [ {"title": "Clinical paths", "content": """Timing of symptom onset and character of discharge are documented."""}, {"title": "Diagnosis", "content": """**Immediate assessment:** Emergency Gram stain is performed to rule out [[185|gonorrhea]]. Aerobic, anaerobic, and viral cultures are obtained. **Diagnostic testing:** - Giemsa staining from conjunctival scrapings for chlamydia - Direct immunofluorescence antibody testing for chlamydia"""}, {"title": "Management", "content": """All cases are referred to ophthalmology. **Treatment by organism:** | Organism | Treatment | |----------|----------| | *Neisseria gonorrhoeae* | Intravenous penicillin or third-generation cephalosporin + topical antibiotics + saline irrigation | | *Chlamydia trachomatis* | Oral azithromycin (topical drops not effective as monotherapy) | | *Pseudomonas* | Systemic aminoglycoside + saline irrigation + gentamicin ophthalmic ointment | | *Staphylococcus* | Parenteral methicillin + saline irrigation | | Chemical (prophylaxis-related) | Supportive care; resolves by 48 hours | **Important notes:** - Topical treatment alone is insufficient for infectious conjunctivitis because it does not clear nasopharyngeal carriage - Treatment of maternal contacts is required for chlamydial cases: mothers of infected infants and mothers' sexual partners should be treated for *C. trachomatis*"""}, ] NEW[174] = [ {"title": "Clinical paths", "content": """**Initial Assessment** High clinical suspicion for neonatal infection is maintained in any symptomatic newborn. Detailed maternal history is obtained, including: - Maternal genital infections (herpes simplex virus, [[185|gonorrhea]]) - Maternal fever or [[355|chorioamnionitis]] - Duration of rupture of membranes - Maternal antibiotic prophylaxis status **General Principles** Because 60–80% of mothers transmitting herpes simplex virus have no prior genital infection history, clinical suspicion cannot rely on maternal history alone. Enteroviral infections are more severe in neonates than older children and may rapidly progress to meningitis, hepatitis, or [[100|myocarditis]]; a low threshold for investigation and treatment is maintained."""}, {"title": "Diagnosis", "content": """**Diagnostic Evaluation** For suspected systemic infection: - Blood culture (before antibiotics) - Complete blood count - Liver function tests (abnormalities suggest herpes simplex virus or enteroviral infection) - Cerebrospinal fluid examination and culture if [[147|meningitis]] suspected - Enteroviral polymerase chain reaction from cerebrospinal fluid in neonates with [[226|sepsis]] signs and elevated liver enzymes For skin lesions: - Potassium hydroxide preparation (candidiasis shows budding yeast with pseudohyphae) - Bacterial culture as indicated For suspected herpes simplex virus: - Considered in any neonate with abnormal liver function tests - Viral culture and polymerase chain reaction from affected sites"""}, {"title": "Management", "content": """**Specific Infection Management** **Neonatal Candidiasis (skin):** Topical antifungal medications are effective for diaper dermatitis and oral thrush. If difficult to treat, immunosuppression is evaluated for. **Gonococcal Ophthalmia Neonatorum:** In-hospital evaluation and treatment are recommended. **Impetigo:** Treated as indicated for *Staphylococcus aureus* or group A streptococcus infection."""}, ] NEW[175] = [ {"title": "Clinical paths", "content": """**Recognition and Initial Assessment** The goal of treatment is prompt recognition of osteomyelitis in the febrile child presenting with bone pain. A focused history is obtained, including trauma, preceding infection, and risk factors ([[162|sickle cell disease]], prior surgery, immunocompromise). Examination is performed for point tenderness over the metaphysis, swelling, erythema, warmth, and range of motion limitation."""}, {"title": "Diagnosis", "content": """**Diagnostic Testing** 1. **Blood cultures** — obtained before antibiotics (positive in ~50% of cases) 2. **Complete blood count** — assessed for elevated white blood cell count 3. **Acute phase reactants** — erythrocyte sedimentation rate, C-reactive protein, or procalcitonin 4. **Plain radiographs** — of the suspected site; may be negative early but should be obtained 5. **Bone biopsy or aspiration** — attempted prior to antibiotics if the child is nontoxic and immunocompetent; provides organism identification and susceptibility testing 6. **MRI** — the preferred imaging modality; demonstrates early edema, subperiosteal abscess, and contiguous infections; should guide surgical drainage if abscess is suspected"""}, {"title": "Management", "content": """**Antibiotic Therapy** Empiric antibiotics are initiated promptly after cultures are obtained. The passages do not provide specific antibiotic regimens, dosing, or routes. Therapy is tailored based on: - Organism identification and susceptibility - Local resistance patterns - Patient age and renal function - Presence of risk factors ([[162|sickle cell disease]], immunocompromise, puncture wounds, bites) Macrolides are less effective than other antibiotics and are recommended only for patients who cannot tolerate cephalosporins, penicillins, or tetracyclines. **Monitoring** Acute phase reactants (erythrocyte sedimentation rate or C-reactive protein) are repeated to monitor treatment effectiveness."""}, ] NEW[176] = [ {"title": "Clinical paths", "content": """No presentation details are available."""}, {"title": "Diagnosis", "content": """No diagnostic protocols are available."""}, {"title": "Management", "content": """The passages provided do not contain specific clinical management protocols, drug dosages, routes of administration, or step-by-step bedside procedures. To provide accurate clinical guidance with specific doses and management sequences, additional reference material containing treatment protocols and pharmacological data would be required."""}, ] NEW[177] = [ {"title": "Clinical paths", "content": """**Assessment** Detailed menstrual and sexual history is obtained with HEEADSSS psychosocial screening. Pelvic examination may be deferred in non-sexually active adolescents with a presentation consistent with primary [[137|dysmenorrhea]]. If examination is performed, it should be normal."""}, {"title": "Diagnosis", "content": """**When to suspect secondary dysmenorrhea** If symptoms do not improve after 6 months of appropriate NSAIDs and/or hormonal management, secondary dysmenorrhea caused by endometriosis or other pelvic pathology is suspected and investigated further. Red flags include pain at menarche, intercycle pain, severe dysmenorrhea, or family history of endometriosis."""}, {"title": "Management", "content": """**First-line treatment: NSAIDs** NSAIDs are started 1–2 days before the expected onset of menses and continued through day 2–3 of bleeding. Age and weight-appropriate doses are used: - Ibuprofen: every 6–8 hours - Naproxen: twice daily - Mefenamic acid: as an alternative Acetaminophen-containing products are not as effective as NSAIDs for dysmenorrhea. **Second-line treatment: Hormonal contraception** For adolescents who do not respond to NSAIDs after appropriate use, or who prefer hormonal management: - Combination oral contraceptive pills improve symptoms in 90% of young women - Maximum therapeutic benefit may take 3 cycles to achieve - NSAIDs and oral contraceptives used together can provide enhanced relief **Adjunctive measures** Patients are counselled to avoid smoking and caffeine. Alternative treatments with evidence of effectiveness are considered: omega-3 polyunsaturated fatty acids, vitamin E, vitamin B1, and magnesium."""}, ] NEW[178] = [ {"title": "Clinical paths", "content": """Signs of fluid overload (increased work of breathing, rales, cardiac gallop, hepatomegaly) are observed for; fluids are stopped if these develop. **Resuscitation endpoints to achieve:** - Heart rate: age-appropriate threshold - Blood pressure: age-appropriate normal - Capillary refill: ≤2 seconds - No difference between peripheral and central pulses - Warm extremities - Urine output: >1 mL/kg per hour - Normal mental status - Cardiac index: 3.3–6.0 L/min/m² - Superior vena cava oxygen saturation: ≥70% - Perfusion pressure (MAP − CVP): 55 ± 1.5 × age in years Vasoactive agents are started as needed if resuscitation endpoints are not achieved with fluids alone."""}, {"title": "Diagnosis", "content": """[[321|Hypoglycemia]] is diagnosed. [[368|Hypocalcemia]] is diagnosed. Blood cultures and laboratory studies are drawn."""}, {"title": "Management", "content": """**Immediate actions (0–5 minutes):** - Intravenous access is established; if unsuccessful, an intraosseous line is established - Airway and breathing are maintained or restored - Continuous cardiac monitoring is initiated **Within 15 minutes:** - Fluid bolus is administered: **10–20 mL/kg isotonic crystalloid or balanced/buffered solution** - Airway is attended to and intravascular access is established if not already done - Hypoglycemia is corrected - Hypocalcemia is corrected - Empiric broad-spectrum antibiotics are administered **Fluid resuscitation strategy:** - Repeat fluid boluses are delivered as needed up to **40–60 mL/kg total** in the first 15–60 minutes **Ongoing assessment:** - Patients responsive to fluid may be observed in the pediatric intensive care unit - Cardiac output measurement can be performed invasively or noninvasively to guide therapy - Oxygen and glucose delivery continue to be maximized"""}, ] NEW[179] = [ {"title": "Clinical paths", "content": """**Initial Assessment** A detailed sleep history is obtained from the child and parent or caregiver, including sleep onset time, nighttime awakenings, morning wake time, daytime sleepiness, snoring, witnessed apneas, and movements during sleep. Behavioral and environmental factors are documented, such as bedtime routine, bedroom environment (presence of electronics), caffeine intake, and consistency of sleep schedule. Associated medical, psychiatric, or neurodevelopmental conditions are identified. In children with ADHD, depression, anxiety, or [[117|autism spectrum disorder]], sleep disturbance is specifically assessed for as part of the clinical evaluation."""}, {"title": "Diagnosis", "content": """**Diagnostic Considerations** For suspected [[311|obstructive sleep apnea]], polysomnography may be indicated based on clinical presentation and risk factors. Videography can document excessive nocturnal movements in suspected restless sleep disorder."""}, {"title": "Management", "content": """**Management Approach** Management begins with behavioral interventions addressing sleep hygiene: consistent bedtimes and wake times are established, a structured bedtime routine is implemented, electronics are removed from the bedroom, and caffeine intake is eliminated. For children with autism spectrum disorder or depression presenting with [[377|insomnia]], cognitive behavioral therapy is an evidence-based treatment option. Melatonin or medications may be considered in autism-associated insomnia when behavioral interventions are insufficient. In restless sleep disorder associated with low serum iron levels, iron supplementation should be considered, with symptomatic improvement expected following treatment. For medication-related sleep disturbance in ADHD, timing and type of stimulant or nonstimulant medication are reviewed, with consideration for dose adjustment or alternative agents in consultation with the prescribing clinician. Underlying psychiatric conditions (depression, anxiety, bipolar disorder) are addressed with appropriate pharmacotherapy and psychotherapy as indicated, which may improve associated sleep disturbance."""}, ] NEW[180] = [ {"title": "Clinical paths", "content": """**Prenatal screening:** Maternal serum screening (quad screen) during the second trimester assesses risk. A concerning result shows low maternal serum alpha-fetoprotein, low unconjugated estriol, elevated human chorionic gonadotropin, and elevated inhibin levels."""}, {"title": "Diagnosis", "content": """**Postnatal diagnosis:** Karyotype analysis or microarray analysis is obtained to confirm trisomy 21. If microarray shows extra chromosome 21 material, routine karyotype is performed to distinguish full trisomy from translocation forms. If translocation is identified, parental karyotyping is performed to determine if inherited or de novo. **Cardiac evaluation:** Echocardiography is indicated given the high frequency of cardiac defects including endocardial cushion defects, ventricular septal defects, and atrial septal defects."""}, {"title": "Management", "content": """**Ongoing surveillance:** - Complete blood count by day 3 of life, then annually - Thyroid-stimulating hormone screening in newborns, at 6 and 12 months of age, then annually - Vision screening using developmentally appropriate methods at routine pediatrician visits, then formally at 4 years of age and annually thereafter - Cervical spine x-ray for atlantoaxial subluxation only in symptomatic children showing signs of myelopathy; asymptomatic screening is not currently supported by evidence, though discussion about cervical spine positioning and contact sports risks is recommended"""}, ] NEW[181] = [ {"title": "Clinical paths", "content": """**Initial Assessment** 1. History of discharge characteristics, duration, associated symptoms, and hygiene practices is obtained 2. Examination is performed for perianal redness, introital inflammation, and discharge appearance"""}, {"title": "Diagnosis", "content": """3. Vaginal discharge is collected for wet preparation and KOH microscopy using a cotton swab inserted into the vagina while avoiding hymenal contact 4. In sexually active adolescents or suspected sexual abuse: urine is obtained for NAATs (*Chlamydia*, *Neisseria gonorrhoeae*); VDRL/RPR, HIV testing, and HSV testing are considered if lesions are present **Diagnostic Interpretation** | Finding | Likely Diagnosis | |---------|------------------| | Clue cells, few leukocytes, *Lactobacillus* outnumbered by mixed flora | Bacterial vaginosis | | Leukocytes, yeast, mycelia, or pseudomycelia (40–80% of cases) | Candida vulvovaginitis | | Motile trichomonds (50–70% of symptomatic patients) | *Trichomonas* vaginitis | | Normal epithelial cells, *Lactobacillus* predominates | Physiologic discharge |"""}, {"title": "Management", "content": """**Treatment by Organism** **[[239|Bacterial vaginosis]]:** - Metronidazole 500 mg orally twice daily for 7 days, OR - Metronidazole gel 0.75% one full applicator intravaginally daily for 5 days, OR - Clindamycin 300 mg orally twice daily for 7 days **Vulvovaginal candidiasis:** - Fluconazole 150 mg orally once, OR - Intravaginal azole cream (multiple formulations available) **[[402|Trichomoniasis]]:** - Metronidazole 2 g orally once, OR - Tinidazole 2 g orally once **Supportive Care (All Cases)** - Sitz baths are recommended - Front-to-back wiping technique is advised - Frequent urination opportunities are encouraged - Regular washing with warm water without excessive scrubbing is instructed - Loose-fitting clothes and white cotton underwear, well rinsed after washing, are recommended - Avoidance of bubble baths and irritant products is advised **Follow-up** - Adherence to hygiene measures is reviewed at follow-up visit - If no improvement despite appropriate treatment and adherence, diagnosis is reassessed and sexual abuse, foreign body, or referral to pediatric gynecology is considered"""}, ] NEW[182] = [ {"title": "Clinical paths", "content": """1. **History and examination**: Timing of symptom onset, preceding viral illness, and current neurological status are documented. Normal mental status, normal strength, normal sensation, and normal reflexes are confirmed to support the diagnosis. **Red Flags Requiring Escalation** If the child develops signs of increased intracranial pressure, focal neurological signs, meningismus with fever, or progressive encephalopathy, alternative diagnoses are considered (cerebellitis with MRI abnormalities, posterior fossa tumor, cerebellar abscess, or acute disseminated encephalomyelitis). These conditions may require more aggressive intervention including steroids or neurosurgical consultation."""}, {"title": "Diagnosis", "content": """2. **Lumbar puncture**: Performed to exclude [[147|meningitis]] and other CNS infections. Normal or near-normal opening pressure, protein, and glucose are expected. Mild lymphocytic pleocytosis (10–30/mm³) is acceptable; significant elevation in white blood cell count or protein suggests alternative diagnosis. 3. **Brain MRI**: Obtained as imaging modality of choice. Normal MRI supports diagnosis of acute cerebellar ataxia and indicates more favorable prognosis. T2 hyperintensities suggest cerebellitis rather than simple ataxia."""}, {"title": "Management", "content": """**Treatment** There is no evidence that corticosteroids or other immune therapy alters outcome. Management is supportive: - Reassurance and observation - Management of nausea and vomiting as needed - Safety precautions during the acute phase - Monitoring for improvement is maintained"""}, {"title": "Prognosis and outcome", "content": """Acute cerebellar ataxia is self-limiting. Improvement typically begins within weeks."""}, ] NEW[183] = [ {"title": "Clinical paths", "content": """1. Airway and respiratory status are assessed immediately. Laryngeal edema is life-threatening; preparation for airway intervention is made if stridor, hoarseness, or voice changes are present."""}, {"title": "Diagnosis", "content": """7. Follow-up testing for suspected hereditary angioedema is arranged: C4 level is obtained; if low, C1-inhibitor level and functional assay follow for confirmation."""}, {"title": "Management", "content": """2. Subcutaneous epinephrine is administered for rapid relief: - Dose: 0.01 mL/kg of 1:1000 concentration - Route: subcutaneous 3. This is followed by an antihistamine for longer-term control: - Diphenhydramine or other second-generation antihistamines - Route and dose per institutional protocol 4. Systemic corticosteroids are considered if angioedema is severe or not responding to initial treatment. 5. For ACE inhibitor-associated angioedema, the offending medication is discontinued. 6. Gastrointestinal involvement is monitored for: abdominal pain, distension, vomiting, or diarrhea are assessed for, as these may indicate bowel involvement."""}, ] NEW[184] = [ {"title": "Clinical paths", "content": """### Assessment Decline in growth is monitored for, which may indicate worsening pulmonary status, onset of cystic fibrosis-related diabetes, or cystic fibrosis-related liver disease. ### Management of Pulmonary Exacerbations Pulmonary exacerbations present with cough, chest congestion, dyspnea, tachypnea, and may include fever. Rhonchi and/or rales may be diffuse or localized. Sputum viscosity or color may change. ### Management of Acute Complications **Distal ileal obstruction syndrome:** Presents with abdominal pain, distention, and emesis without fever. **[[396|Pneumothorax]]:** Presents with acute onset ipsilateral chest pain, shortness of breath, and ipsilateral decreased or absent breath sounds. **Cystic fibrosis-related diabetes:** Weight loss or poor weight gain, polydipsia, polyuria, and glycosuria are screened for."""}, {"title": "Diagnosis", "content": """Nutritional parameters are routinely assessed as part of standard care. Lung function testing (FEV₁) and chest radiography are used to assess for worsening pulmonary exacerbations. For distal ileal obstruction syndrome, abdominal radiography is obtained to confirm diagnosis. For pneumothorax, diagnosis is confirmed with chest radiography. For cystic fibrosis-related diabetes, glucose and hemoglobin A₁c levels are measured; glucose tolerance testing is performed if indicated."""}, {"title": "Management", "content": """### Nutritional Support Children over 2 years of age (including adolescents) should achieve energy intake at levels of **110% to 200% above usual requirements** in healthy children to achieve age-appropriate weight gain. A combination of approaches is implemented: - Calorie boosting - Nutritional supplements - Behavioral interventions The goal is to maintain normal weight and height for age. ### Coordination of Care When possible, management of a patient with cystic fibrosis is coordinated with the staff of the cystic fibrosis center where the child receives routine care."""}, {"title": "Prognosis and outcome", "content": """Normal growth is associated with better lung function and survival."""}, ] NEW[185] = [ {"title": "Clinical paths", "content": """Careful sexual history is obtained, including pharyngeal and anorectal exposure. In prepubertal children with genital, rectal, or pharyngeal infection, thorough epidemiologic investigation is conducted and sexual abuse is considered. Infants with clinical evidence of ophthalmia neonatorum or scalp abscess are hospitalized."""}, {"title": "Diagnosis", "content": """Blood cultures are sent in neonatal cases to evaluate for disseminated infection ([[226|sepsis]], arthritis, [[147|meningitis]]). All patients are tested for concurrent syphilis, HIV, and *Chlamydia trachomatis* infection. In neonatal disease, disseminated infection is evaluated for."""}, {"title": "Management", "content": """**Treatment of Uncomplicated Infections (Adolescents)** Dual therapy is recommended for uncomplicated gonococcal infections of the cervix, urethra, rectum, and pharynx: - **Ceftriaxone** intramuscularly, once - **Azithromycin** orally, once **Neonatal Disease** Treatment is provided in consultation with an infectious disease specialist. **Post-Treatment Counseling** - Patients are instructed to abstain from sexual activity for 7 days after treatment and until all sexual partners are adequately treated - Same-day treatment linkage is provided if medications are unavailable at initial visit - Sexual contacts are identified and treated; expedited partner treatment (prescriptions without examination) increases success - Medication is administered on-site with direct observation when possible"""}, ] NEW[186] = [ {"title": "Clinical paths", "content": """**Diagnosis and Initial Assessment** GAS pharyngitis is confirmed clinically based on sudden sore throat, tonsillar inflammation, and cervical lymphadenopathy, particularly in children aged 5–15 years."""}, {"title": "Diagnosis", "content": """Throat culture may be negative if infection has localized to cervical lymph nodes."""}, {"title": "Management", "content": """**Antimicrobial Therapy** A standard pharyngitis antibiotic regimen is initiated. The passages do not specify drug names, doses, or routes, so these should be determined from current local guidelines and formularies. **Timing** Treatment is begun promptly to reduce acute morbidity and prevent complications. Patients become non-contagious 24 hours after starting appropriate antimicrobial therapy. **Management of [[194|Acute Rheumatic Fever]]** If ARF develops: - GAS is eradicated with the standard pharyngitis antibiotic regimen - Acute manifestations (arthritis, valvulitis, heart failure) are treated according to clinical presentation - Education is provided to parents and patient - Secondary prophylaxis is initiated to prevent future GAS infections **Note on [[369|Glomerulonephritis]]** Antimicrobial therapy does not prevent acute glomerulonephritis after pharyngitis or pyoderma, so treatment decisions should not be based on this goal."""}, ] NEW[187] = [ {"title": "Clinical paths", "content": """2. Focused history is obtained: painless vs painful, intermittent vs persistent, gross vs microscopic; trauma history; family history of hematuria or renal disease 3. Examination is performed for signs of systemic disease: [[92|hypertension]], edema, flank mass, perineal bruising (abuse is considered), signs of heart failure Glomerular hematuria is suggested by brown or tea-colored urine, deformed red cells, casts, and proteinuria."""}, {"title": "Diagnosis", "content": """1. Hematuria is confirmed with urine microscopy of fresh, spun urine showing red blood cells; dipstick alone is insufficient **Investigations for all children with hematuria** - Urine microscopy with phase contrast and culture - Urine protein and calcium excretion - Kidney and urinary tract ultrasound - Blood tests: urea, electrolytes, creatinine, calcium, phosphate, albumin, full blood count, platelets, coagulation screen, sickle cell screen **Additional investigations if glomerular hematuria suspected** - Erythrocyte sedimentation rate - Complement levels (C3, C4) - Anti-DNA antibodies"""}, {"title": "Management", "content": """**Admission indications** Admission occurs if hematuria is associated with: - Severe abdominal or flank pain - [[285|Congestive heart failure]] or fluid overload with oliguria or anuria - [[92|Hypertension]] - Renal insufficiency - Generalized edema (anasarca)"""}, ] NEW[188] = [ {"title": "Clinical paths", "content": """Hemolytic anemia is recognized in any child presenting with unexplained low hemoglobin. Typical features include pallor, jaundice, dark urine, fatigue, and splenomegaly. Symptoms may develop suddenly or evolve over days to weeks."""}, {"title": "Diagnosis", "content": """Hemolytic anemia is confirmed by reticulocytosis accompanying the low hemoglobin."""}, {"title": "Management", "content": """**Immediate Stabilization** The goal is to stabilize the hemoglobin level and maintain sufficient oxygen-carrying capacity and cardiac output. Early recognition and intervention for uncompensated anemia is critical. **Transfusion Indications** - **Severe [[349|anemia]] with cardiovascular compromise**: Transfusion is given when hemoglobin <5 g/dL - **Reticulocytopenia**: Transfusion is given in the presence of reticulocytopenia **Warm Autoimmune Hemolytic Anemia** 1. **First-line treatment**: Corticosteroids (prednisone) 2. **Second-line options**: Splenectomy or rituximab 3. **Transfusion**: Reserved for severe [[349|anemia]] with cardiovascular compromise or reticulocytopenia **Cold Autoimmune Hemolytic Anemia** - Cold avoidance is advised **Life-Threatening Autoimmune Hemolysis** - Hematology is urgently consulted - High-dose steroids, intravenous immunoglobulin, plasmapheresis, or exchange transfusion are considered as indicated - Long-term management may include splenectomy or immunosuppressant medications **Neonatal Hemolytic Disease** - Intensive phototherapy - Exchange transfusion **Nonimmune Hemolytic Anemia** - Observation and supportive care - The offending agent is removed if identified - Renal damage from significant hemolysis is prevented - For small-vessel disease (e.g., thrombotic thrombocytopenic purpura): prompt plasma exchange can be lifesaving - The underlying disorder is treated (e.g., collagen vascular disease, renal failure in hemolytic-uremic syndrome) **Inherited Hemolytic Anemias** - Careful long-term monitoring - Occasional transfusions as needed - Hematology consultation for management planning"""}, ] NEW[189] = [ {"title": "Clinical paths", "content": """5. Signs of hemolysis are monitored for: jaundice within first 6 hours, pallor, hepatosplenomegaly"""}, {"title": "Diagnosis", "content": """1. Blood group and direct Coombs test are obtained on cord blood or infant blood 2. Peripheral blood smear is performed to identify spherocytes, schistocytes, and nucleated red blood cells 3. Hemoglobin, hematocrit, and reticulocyte count are measured 4. Baseline total serum bilirubin level is established **Ongoing Monitoring** Infants with Rh disease require surveillance for: - [[400|Thrombocytopenia]] (from liver dysfunction or disseminated intravascular coagulation) - [[321|Hypoglycemia]] (secondary to pancreatic islet cell hyperplasia) - Direct [[130|hyperbilirubinemia]] (from hepatocellular damage)"""}, {"title": "Management", "content": """**Therapeutic Interventions** **Exchange Transfusion** - Removes antibody-coated red blood cells - Replaces with uncoated donor red blood cells lacking the sensitizing antigen - Prolongs intravascular red blood cell survival - Reduces bilirubin concentration **Intravenous Immunoglobulin (IVIG)** - Indicated if total serum bilirubin is rising despite intensive phototherapy - Indicated if total serum bilirubin is within 2 to 3 mg/dL of the exchange transfusion threshold - Decreases the need for exchange transfusion in Rh and ABO incompatibility - Specific dosing not provided in available guidelines"""}, ] def main(): src_path = "/tmp/claude-0/-home-danvics-docker-quiz/c1e0577a-e42c-4a3d-b1ea-3edd61103a4e/scratchpad/mdm/mdm-02.json" out_path = "/tmp/claude-0/-home-danvics-docker-quiz/c1e0577a-e42c-4a3d-b1ea-3edd61103a4e/scratchpad/mdm/mdm-02.done.json" with open(src_path) as f: data = json.load(f) missing = [] for article in data: aid = article["article_id"] if aid not in NEW: missing.append(aid) continue article["sections"] = NEW[aid] if missing: print("MISSING article ids (not replaced):", missing) else: print("All", len(data), "articles replaced.") with open(out_path, "w") as f: json.dump(data, f, ensure_ascii=False, indent=2) print("Wrote", out_path) if __name__ == "__main__": main()