Hospital Admission Patterns in Children with CAH: Admission Rates and Adrenal Crises Decline with Age.

R Louise Rushworth,Ann M Maguire,David J Torpy,Henrik Falhammar,Craig F Munns

doi:10.1155/2016/5748264

Abstract

Objective. To examine patterns of hospitalisation for acute medical conditions in children with congenital adrenal hyperplasia (CAH). Design. A retrospective study of hospitalisation using administrative data. Setting. All hospitals in NSW, Australia. Patients. All patients admitted with CAH and a random sample of admissions in patients aged 0 to 18 years without adrenal insufficiency (AI). Main Outcome Measures. Admissions and comorbidities by age and sex. Results. Of 573 admissions for medical problems in CAH children, 286 (49.9%) were in males, and 236 (41.2%) had a principal diagnosis of CAH or had an adrenal crisis (AC). 37 (6.5%) ACs were recorded. An infection was found in 43.5% (n = 249) of the CAH patient admissions and 51.7% (n = 1613) of the non-AI group, p < 0.001. Children aged up to one year had the highest number of admissions (n = 149) and six ACs (four in males). There were 21 ACs recorded for children aged 1–5 years. Older CAH children had fewer admissions and fewer ACs. No in-hospital deaths were recorded. Conclusions. Admission for medical problems in CAH children declines with age. An AC was recorded in 6.5% of the admissions, with the majority of ACs occurring in the 1 to 5 years age group and there were no deaths.

Highlights

Congenital adrenal hyperplasia (CAH) is the commonest cause of adrenal insufficiency (AI) in childhood and 21hydroxylase deficiency is the most frequently occurring form, accounting for approximately 95% of cases and having an incidence of between 1 in 9,000 and 1 in 20,000 births [1,2,3]. 21-Hydroxylase deficiency is autosomal recessive, with patients inheriting inactivating mutations of varying severity in both CYP21A2 alleles, resulting in impaired synthesis and secretion of cortisol and aldosterone in combination with elevated levels of adrenal androgens [3,4,5]
The results of the present study, which covered thirteen years of admissions to both private and public hospitals in a large geographic area for medical treatment of children with CAH, demonstrated that infants had the highest admission rate among the age groups; episodes of hospitalisation decreased with increasing age; 6.5% of the admissions included a record of an adrenal crisis (AC) but if all cases of admission for hypoadrenalism were included, almost 50% were due to some degree of AI; there was a lessening of the risk of AC beyond age of five; comorbid viral infections were more common than bacterial infections; and there were no in-hospital deaths
Among all the CAH patient admissions, 149 were in infants, which corresponded to an average of 11.5 admissions in the infant age group for each year of the study

Summary

Introduction

Congenital adrenal hyperplasia (CAH) is the commonest cause of adrenal insufficiency (AI) in childhood and 21hydroxylase deficiency is the most frequently occurring form, accounting for approximately 95% of cases and having an incidence of between 1 in 9,000 and 1 in 20,000 births [1,2,3]. 21-Hydroxylase deficiency is autosomal recessive, with patients inheriting inactivating mutations of varying severity in both CYP21A2 alleles, resulting in impaired synthesis and secretion of cortisol and aldosterone in combination with elevated levels of adrenal androgens [3,4,5]. 21-Hydroxylase deficiency is autosomal recessive, with patients inheriting inactivating mutations of varying severity in both CYP21A2 alleles, resulting in impaired synthesis and secretion of cortisol and aldosterone in combination with elevated levels of adrenal androgens [3,4,5]. Patients, those with the more severe forms, are at risk of a salt-wasting crisis in infancy and an adrenal crisis (AC) at any age, when circulating cortisol levels are lower than physiologically required, especially in conditions of physiological stress such as infection [1, 3, 4]. The incidence of AC in these children is approximately 4.9 per 100 patient years [8]

Methods

Results

Conclusion