Real world estimates of adrenal insufficiency related adverse events in children with congenital adrenal hyperplasia
AuthorsAli, S. R.
Lewsey, J. D.
Tan, L. E.
Birkebaek, N. H.
Claahsen-van der Grinten, H. L.
Costa, E. C.
Finken, M. J. J.
Fluck, C. E.
Hannema, S. E.
Higham, Claire E
Hughes, I. A.
Krone, N. P.
Mendonca, B. B.
Miranda, M. C.
van der Kamp, H.
de Vries, L.
Ross, R. J. M.
Ahmed, S. F.
AffiliationDevelopmental Endocrinology Research Group, School of Medicine, Dentistry & Nursing, University of Glasgow, UK.
MetadataShow full item record
AbstractBackground: Although congenital adrenal hyperplasia (CAH) is known to be associated with adrenal crises (AC), its association with patient or clinician reported sick day episodes (SDE) is less clear. Methods: Data on children with classic 21-hydroxylase deficiency CAH from 34 centers in 18 countries, of which 7 were Low or Middle Income (LMIC) and 11 were High Income (HIC), were collected from the International CAH Registry and analysed to examine the clinical factors associated with SDE and AC. Results: 518 children with a median of 11 children (range 1, 53) per center had 5,388 visits evaluated over a total of 2,300 patient years. The median number of AC and SDE per patient year per center was 0 (0, 3) and 0.4 (0.0, 13.3), respectively. Of the 1,544 SDE, an AC was reported in 62 (4%), with no fatalities. Infectious illness was the most frequent precipitating event, reported in 1,105 (72%) and 29 (47%) of SDE and AC, respectively. On comparing cases from LMIC and HIC, the median SDE per patient year was 0.75 (0, 13.3) vs 0.11 (0, 12.0) (p<0.001), respectively and the median AC per patient year was 0 (0, 2.2) vs 0 (0, 3.0) (p=0.43), respectively. Conclusions: The real-world data that are collected within the I-CAH Registry show wide variability in the reported occurrence of adrenal insufficiency related adverse events. As these data become increasingly used as a clinical benchmark in CAH care, there is a need for further research to improve and standardise the definition of SDE.
CitationAli SR, Bryce J, Haghpanahan H, Lewsey JD, Tan LE, Atapattu N, et al. Real World Estimates Of Adrenal Insufficiency Related Adverse Events In Children With Congenital Adrenal Hyperplasia. J Clin Endocrinol Metab. 2020.
JournalJournal of Clinical Endocrinology and Metabolism
- Children with classic congenital adrenal hyperplasia experience salt loss and hypoglycemia: evaluation of adrenal crises during the first 6 years of life.
- Authors: Odenwald B, Nennstiel-Ratzel U, Dörr HG, Schmidt H, Wildner M, Bonfig W
- Issue date: 2016 Feb
- Adrenal crises in children with adrenal insufficiency: epidemiology and risk factors.
- Authors: Eyal O, Levin Y, Oren A, Zung A, Rachmiel M, Landau Z, Schachter-Davidov A, Segev-Becker A, Weintrob N
- Issue date: 2019 May
- Variations in the management of acute illness in children with congenital adrenal hyperplasia: An audit of three paediatric hospitals.
- Authors: Chrisp GL, Maguire AM, Quartararo M, Falhammar H, King BR, Munns CF, Torpy DJ, Hameed S, Rushworth RL
- Issue date: 2018 Nov
- Etiology of primary adrenal insufficiency in children: a 29-year single-center experience.
- Authors: Wijaya M, Huamei M, Jun Z, Du M, Li Y, Chen Q, Chen H, Song G
- Issue date: 2019 Jun 26
- Glucocorticoid replacement regimens for treating congenital adrenal hyperplasia.
- Authors: Ng SM, Stepien KM, Krishan A
- Issue date: 2020 Mar 19