Objectives: To assess the prevalence and risk factors of Adrenal crisis (AC) events in children with Adrenal insufficiency (AI) and to evaluate the effectiveness of the treatment for preventing AC.
Methods: Children diagnosed with AI between 1990 and 2017 and treated with glucocorticoids at four pediatric endocrinology units in Israel were studied. Data were retrieved retrospectively from the patients files and they included demographic factors (age, sex and ethnic origin), clinical information (age at diagnosis, specific diagnosis and clinical presentation during AC) and therapy regimen details (type, dosage and parental guidance). The collected data underwent statistical analyses.
Results: The study population consisted of 120 children (73 boys, 47 girls). Median age at study was 11.5 years (0.325) and median age at diagnosis was 0.3 years (017.5). Thirty-one AC events in 26 children took place during the study period, which is equivalent to 3.4 crises per 100 patient years (py). The prevalence of AC in children with primary AI was 4 events to 100 py compared to 1.5 events to 100 py in children with secondary AI (P=0.001). One-hundred thirteen children (94%) were treated with hydrocortisone at a mean dosage of 12.3±5.2 mg/m2/day. Sixty children also needed treatment with fludrocortisone at a mean dosage of 0.1 mg/day ±0.04. The risk factors for developing AC in children with AI were: younger age at diagnosis (P=0.003), primary AI compared with secondary AI (P=0.016), specific diagnosis of autoimmune adrenal insufficiency (Addison disease), congenital adrenal hypoplasia, adrenoleukodystrophy, salt wasting congenital adrenal hyperplasia (P<0.001), mineralocorticoid treatment (P<0.001) and hospital admissions (P>0.001). Unexpectedly, the use of an AI identification (ID) tag/card or parental use of the Solucortef kit were not negatively correlated with the development of AC. Among those who developed AC, 96% carried some ID (P=0.18) and 50% also received Solucoref treatment (P=0.82), compared to 82% and 45%, respectively, for those that did not develop AC. There was no AC-associated mortality during the study period, but there were five reports of a family history of pediatric AC-associated mortality.
Conclusions: A number of parameters were found to be risk factors for the development of AC in children with AI. Among them, age at diagnosis, etiology of AI, number of hospitalizations and mineralocorticoid treatment. The preventive measures of carrying ID and Solucoref treatment did not arrest the development of AC, calling for further investigation for effective preventive measures.
27 - 29 Sep 2018
European Society for Paediatric Endocrinology