BACKGROUND AND OBJECTIVES

Patients with congenital adrenal hyperplasia (CAH) owing to 21-hydroxylase deficiency are prone to potentially life-threatening adrenal crises. We tried to identify risk factors for postdiagnosis hospitalization for children with the salt-wasting form of CAH.

METHODS

We reviewed medical records of all children who presented to Children's Medical Center, Dallas, from 1999 to 2013 with CAH (ICD-9 code 255·2).

RESULTS

Five hundred and twenty-two unique patients were coded for ICD-9 code of 255·2 (CAH) from 1999 to 2013; 155 patients had salt-wasting disease. Fifty-five patients were hospitalized a total of 105 times. Patients who were hospitalized were more likely to have noncommercial insurance (RR = 1·8; 95% CI [1·1-2·8]; P = 0·02); this included five patients hospitalized a total of 37 times. Children younger than 2 years (RR = 3·3 [2·2-4·8]; P < 0·0001) were more likely to be hospitalized. In a nested case-control analysis, the risk of hospitalization was correlated with daily fludrocortisone dose (P ≤ 0·0001) but not hydrocortisone dose; no outpatient laboratory test predicted hospitalization. Gastroenteritis was the most frequent admitting diagnosis.

CONCLUSIONS

Younger children may be at greater risk of hospitalization owing to increased susceptibility to viral infections and decreased ability to withstand stress and dehydration. A minority of patients with noncommercial insurance may have higher risk owing to social barriers that interfere with treatment compliance. Those requiring higher daily fludrocortisone dosages likely have inherently more severe disease leading to higher rates of hospitalization.