Reproductive Pathology, Pathology, USMLE Step 1 - Full Vignette with Extended Explanations

Описание: A 7-day-old term male neonate presents with poor feeding, repeated non-bilious vomiting, dehydration, severe hyponatremia, hyperkalemia, hypoglycemia, and hypotension. The newborn screen shows elevated 17-hydroxyprogesterone, with confirmatory tests pending. What critical considerations should guide your next steps in stabilizing this infant? How do the clinical and laboratory features help prioritize immediate interventions in life-threatening neonatal emergencies?

VIDEO INFO
Category: Reproductive Pathology, Pathology, USMLE Step 1
Difficulty: Expert - Expert level - For those seeking deep understanding
Question Type: Emergency Priorities
Case Type: Common Scenario

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QUESTION
A 7-day-old term male neonate is brought to the emergency department for poor feeding, repeated non-bilious emesis, decreased urine output, and progressive sleepiness over 12 hours. Pregnancy and delivery were uncomplicated. The state newborn screen reported earlier today is positive on the two-tier assay for 17-hydroxyprogesterone by LC-MS/MS (confirmatory steroid profile pending)....

OPTIONS
A. Administer 0.9% sodium chloride 10 mL/kg IV as a rapid bolus (repeat as needed while reassessing perfusion), give hydrocortisone 25 mg IV immediately followed by 5-10 mg IV every 6 hours, and treat hypoglycemia with 10% dextrose 2 mL/kg IV while preparing standard hyperkalemia measures if ECG cha...
B. Administer 0.9% sodium chloride 20 mL/kg IV as the first step and give dexamethasone 0.5 mg/kg IV instead of hydrocortisone to avoid assay interference, then delay fludrocortisone initiation until confirmatory results return from the laboratory after endocrine consultation.
C. Obtain a cosyntropin stimulation test before any steroid dosing, start continuous hydrocortisone infusion at 50 mg/m2/day without an initial isotonic crystalloid bolus, and run 5% dextrose in 0.45% sodium chloride at 1.5x maintenance in lieu of early volume resuscitation.
D. Prioritize correction of hyponatremia with 3% hypertonic saline 6 mL/kg IV bolus and insulin with dextrose for hyperkalemia, delay glucocorticoids until cortisol and ACTH levels return, and begin broad-spectrum antibiotics before addressing adrenal hormone replacement.

CORRECT ANSWER
A. Administer 0.9% sodium chloride 10 mL/kg IV as a rapid bolus (repeat as needed while reassessing perfusion), give hydrocortisone 25 mg IV immediately followed by 5-10 mg IV every 6 hours, and treat hypoglycemia with 10% dextrose 2 mL/kg IV while preparing standard hyperkalemia measures if ECG changes persist; proceed without delaying for additional testing in the resuscitation bay.

EXPLANATION
This critically ill neonate has hypotension, hypoglycemia, hyponatremia, hyperkalemia with peaked T waves, metabolic acidosis, and diffuse hyperpigmentation shortly after a positive newborn screen for 21-hydroxylase deficiency. The immediate priority in suspected adrenal crisis is to restore intravascular volume and perfusion with isotonic saline, rapidly replace glucocorticoids with a stress dose that also provides mineralocorticoid activity, and correct life-threatening hypoglycemia-without delaying for additional testing. Hydrocortisone is preferred because it treats cortisol deficiency and, at stress doses, provides sufficient mineralocorticoid effect for the acute phase....


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