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Variations in the management of acute illness in children with congenital adrenal hyperplasia: An audit of three paediatric hospitals
Author(s) -
Chrisp Georgina L.,
Maguire Ann M.,
Quartararo Maria,
Falhammar Henrik,
King Bruce R.,
Munns Craig F.,
Torpy David J.,
Hameed Shihab,
Rushworth R. Louise
Publication year - 2018
Publication title -
clinical endocrinology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.055
H-Index - 147
eISSN - 1365-2265
pISSN - 0300-0664
DOI - 10.1111/cen.13826
Subject(s) - medicine , congenital adrenal hyperplasia , dosing , hydrocortisone , context (archaeology) , pediatrics , adrenal insufficiency , fludrocortisone , adrenal crisis , vomiting , emergency medicine , paleontology , biology
Summary Objective Episodes of acute adrenal insufficiency ( AI )/adrenal crises ( AC ) are a serious consequence of congenital adrenal hyperplasia ( CAH ). This study aimed to assess morbidity from acute illness in CAH and identify factors associated with use of IV hydrocortisone, admission and diagnosis of an AC . Method An audit of acute illness presentations among children with CAH to paediatric hospitals in New South Wales, Australia, between 2000 and 2015. Results There were 321 acute presentations among 75 children with CAH . Two‐thirds (66.7%, n = 214) of these resulted in admission and 49.2% (n = 158) of the patients received intravenous ( IV ) hydrocortisone. An AC was diagnosed in (9.0%). Prior to presentation, 64.2% (n = 206) had used oral stress dosing and 22.1% (n = 71) had been given intramuscular ( IM ) hydrocortisone. Vomiting was recorded in 61.1% (n = 196), 32.7% (n = 64) of whom had used IM hydrocortisone. Admission, AC diagnosis and use of stress dosing varied significantly between hospitals. IM use varied from 7.0% in one metropolitan hospital to 45.8% in the regional hospital. Children aged up to 12 months had the lowest levels of stress dosing and IV hydrocortisone administration. Higher numbers of prior hospital attendances for acute illness were associated with increased use of IM hydrocortisone. Conclusion Prehospital and in‐hospital management of children with CAH can vary between health services. Children under 12 months have lower levels of stress dosing prior to hospital than other age groups. Experience with acute episodes improves self‐management of CAH in the context of acute illness in educated patient populations.