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A 6-week-old infant presents with recurrent episodes of unresponsiveness and seizures. Repeated bedside glucose values during episodes are 22–35 mg/dL. He requires a glucose infusion rate (GIR) of 13 mg/kg/min to maintain glucose >50 mg/dL. Critical sample during hypoglycemia shows: insulin 9.8 uU/mL, C-peptide elevated, beta-hydroxybutyrate 0.3 mmol/L, free fatty acids low, cortisol 22 mcg/dL, GH 15 ng/mL. What is the MOST appropriate initial pharmacotherapy?

A 14-month-old is brought to the ED with a 4-hour history of intermittent severe screaming with drawing-up of his knees, separated by periods of lethargy. He has vomited twice and passed one blood-tinged mucousy stool. A sausage-shaped mass is palpable in the RUQ. What is the MOST appropriate next step?

A 4-year-old with known adrenal insufficiency on hydrocortisone and fludrocortisone develops a fever of 38.9°C, has vomited three times, and is now refusing all oral medications. Her HR is 118 bpm and BP is 90/58 mmHg. Her parents call the office. What is the MOST appropriate next step?

A 16-year-old female with known Graves disease, non-adherent to methimazole for 3 months, undergoes emergency appendectomy. Five hours post-operatively, she develops fever of 40.3°C, HR 162 bpm, agitation, tremors, and severe diaphoresis. BP is 178/100 mmHg. Burch-Wartofsky score is 75. Which management sequence is CORRECT for thyroid storm?

A 3-month-old presents with profound hypotonia (floppy baby), macroglossia, and massive cardiomegaly on chest X-ray. SpO2 is 82% on room air. Echocardiogram shows severe hypertrophic cardiomyopathy (HCM) with severely reduced function. Newborn screen at 2 weeks was flagged abnormal but the family was lost to follow-up. Which enzyme is deficient and what is the MOST appropriate disease-modifying therapy?

An infant with Trisomy 21 has a murmur and signs of heart failure. ECG shows left axis deviation and first-degree AV block. Which cardiac lesion is most likely, and by what age should repair typically occur to prevent irreversible pulmonary hypertension?

A patient develops polymorphic VT with QRS complexes that appear to twist around the isoelectric baseline in the setting of a prolonged QTc and recent ondansetron administration. What is the most appropriate first-line treatment?

An 11-month-old presents with hypotonia, developmental regression (had been sitting at 6 months, now cannot), abnormal eye movements, and recurrent apneas. Brain MRI shows bilateral symmetric T2 signal hyperintensities in the basal ganglia and brainstem periaqueductal gray. CSF lactate is 5.2 mmol/L. The metabolic team is consulted and begins an evaluation for suspected mitochondrial disease. The neurology team requests a medication for seizure management. Which antiepileptic drug is CONTRAINDICATED in this patient?

You need a comma and a ____________ between two independent clauses. 

A term neonate becomes progressively cyanotic within hours of birth with minimal respiratory distress. CXR shows a narrow mediastinum with cardiomegaly (“egg-on-string”). After starting PGE1 and performing balloon atrial septostomy, what is the definitive surgical management, and by when should it ideally occur?