Electrolyte abnormalities and stress dosing predict illness-related hospitalizations among infants and toddlers with congenital adrenal hyperplasia

Abstract

Objective: Infants and toddlers with classical congenital adrenal hyperplasia (CAH) are at high risk for morbidity/mortality arising from life-threatening adrenal crisis. Management of acute illnesses in CAH requires an understanding of factors leading to emergency department (ED) visits and hospitalizations in the first few years of life. We, therefore, examined adrenal crisis at prehospital and ED stages of illness in young children with CAH as they related to medical outcomes. Patients and Design: Retrospective cohort study of 39 children with CAH due to 21-hydroxylase deficiency (0–4 years of age) and 27 age-matched controls. Measurements: ED visit, acute illness symptoms (fever, vomiting, diarrhoea) and other characteristics (hospitalizations, administration of stress-dose hydrocortisone, electrolyte abnormalities). Results: CAH infants and toddlers had significantly higher rates of ED visits (0.50 [0.25–0.88] per person-year) than controls (0 [0–0] per person-year; p