Background: An adrenal crisis (AC) is a life-threatening complication of congenital adrenal hyperplasia (CAH). Despite modern therapies, children with CAH still present with symptomatic adrenal insufficiency (AI) and AC.
Objective and hypotheses: The aim of the study was to determine the spectrum of symptoms and signs of AI in children with diagnosed CAH who were admitted to hospital for an acute illness, as well as to evaluate the use of stress dosing and parenteral hydrocortisone (HC) in these children.
Method: We audited the records of all patients with a diagnosis of CAH who were admitted to one of two specialist paediatric referral hospitals for an acute illness in Sydney, Australia, between 2000 and 2015. All patients in the study were receiving glucocorticoid replacement therapy. Information on symptoms and signs of acute AI including hypotension, abnormal electrolytes and hypoglycaemia was collected, together with information on all diagnoses (including AC) and the use of stress dosing and parenteral HC.
Results: There were 113 admissions between 2000 and 2015. Of these, 49 (43.4%) were in males; 20 (17.7%) were aged under 1 year; 45 (39.8%) were aged 15 years, and 48 (42.5%) were over 6 years of age. hypotension was recorded in 9 (8%) children; hyperkalaemia was reported in 8 (7.1%); hyponatraemia in 14 (12.4%); hypoglycaemia in 13 (11.5%); reduced consciousness in 12 (10.6%); vomiting in 53 (46.9%); diarrhoea in 24 (21.2%); lethargy in 27 (23.9%); fever in 37 (32.7%). Oral stress dosing was used prior to admission in 53 (46.9%) cases. Intravenous HC was given in hospital to 56 (49.6%) children. Five admissions (4.4%) were identified as an AC.
Conclusion: In children with treated CAH, admission to hospital with symptoms consistent with gastroenteritis and acute AI were common. The diagnosis of AC was rare in this group.
10 - 12 Sep 2016
European Society for Paediatric Endocrinology