Pediatric emergency medicine trisk 2594 2594

Obtain blood glucose, BMP, liver functions, blood gas, and ammonia levels and
admit for further management.
Congenital Adrenal Hyperplasia
Emergency providers should be familiar with the differential diagnosis of adrenal
insufficiency due to congenital adrenal hyperplasia presenting as shock in a
neonate. This diagnosis should be suspected in infants presenting with poor
feeding, vomiting, or lethargy. In the severe, salt-wasting forms, there are
characteristic metabolic derangements of hyponatremia, hyperkalemia, metabolic
acidosis, and hypoglycemia. On physical examination, female infants can exhibit
ambiguous genitalia, depending on degree of virilization, but males usually have
normal-appearing genitalia. Neonates presenting in adrenal crisis will require
urgent intervention. Initial goals include correction of hypotension and
dehydration, reversal of electrolyte and glucose abnormalities, and correction of
cortisol deficiency. Obtain bedside glucose determination. Address hypoglycemia
with 10% dextrose solutions, initial doses of 5 mL/kg. After correcting the
hypoglycemia, a continuous infusion of dextrose should be started. An initial
fluid bolus of normal saline should be given and repeated as necessary to restore
perfusion. Intravenous hydrocortisone should be administered immediately, at an
initial dose of 50 to 100 mg/m2 as an IV bolus (typically 25 mg for infants)
followed by 50 to 100 mg/m2 IV per day divided every 6 hours.
Suggested Readings and Key References
Kemper AR, Mahle WT, Martin GR, et al. Strategies for implementing screening
for critical congenital heart disease. Pediatrics 2011;128(5):e1259–e1267.
Initial Assessment
Levesque BM, Pollack P, Griffin BE, et al. Pulse oximetry: what’s normal in the
newborn nursery? Pediatr Pulmonol 2000;30(5):406–412.
Soghier L, Pham K, Rooney S, eds. Reference Range Values for Pediatric Care .
1st ed. Illinois, IL: AAP Bookstore, Ilk Groove; 2014.
Zubrow AB, Hulman S, Kushner H, et al. Determinants of blood pressure in
infants admitted to neonatal intensive care units: a prospective multicenter
study. Philadelphia Neonatal Blood Pressure Study Group. J Perinatol

1995;15(6):470–479.
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