Risk factors for hospitalization of children with congenital adrenal hyperplasia

Abstract

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 post-diagnosis 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 congenital adrenal hyperplasia (ICD-9 code 255.2).

Results

522 unique patients were coded for ICD-9 code of 255.2 (CAH) from 1999-2013; 155 patients had salt- wasting disease. 55 patients were hospitalized a total of 105 times. Patients who were hospitalized were more likely to have non-commercial insurance (RR=1.8; 95% CI [1.1-2.8]; p=0.02); this included 5 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 non-commercial 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.

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