International practice of corticosteroid replacement therapy in congenital adrenal hyperplasia: data from the I-CAH registry
Publication year
2021Author(s)
Source
European Journal of Endocrinology, 184, 4, (2021), pp. 553-563ISSN
Publication type
Article / Letter to editor

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Organization
Paediatrics
Journal title
European Journal of Endocrinology
Volume
vol. 184
Issue
iss. 4
Page start
p. 553
Page end
p. 563
Subject
Radboudumc 16: Vascular damage RIMLS: Radboud Institute for Molecular Life SciencesAbstract
OBJECTIVE: Despite published guidelines no unified approach to hormone replacement in congenital adrenal hyperplasia (CAH) exists. We aimed to explore geographical and temporal variations in the treatment with glucocorticoids and mineralocorticoids in CAH. DESIGN: This retrospective multi-center study, including 31 centers (16 countries), analyzed data from the International-CAH Registry. METHODS: Data were collected from 461 patients aged 0-18 years with classic 21-hydroxylase deficiency (54.9% females) under follow-up between 1982 and 2018. Type, dose and timing of glucocorticoid and mineralocorticoid replacement were analyzed from 4174 patient visits. RESULTS: The most frequently used glucocorticoid was hydrocortisone (87.6%). Overall, there were significant differences between age groups with regards to daily hydrocortisone-equivalent dose for body surface, with the lowest dose (median with interquartile range) of 12.0 (10.0-14.5) mg/m2/day at age 1-8 years and the highest dose of 14.0 (11.6-17.4) mg/m2/day at age 12-18 years. Glucocorticoid doses decreased after 2010 in patients 0-8 years (P < 0.001) and remained unchanged in patients aged 8-18 years. Fludrocortisone was used in 92% of patients, with relative doses decreasing with age. A wide variation was observed among countries with regards to all aspects of steroid hormone replacement. CONCLUSIONS: Data from the I-CAH Registry suggests international variations in hormone replacement therapy, with a tendency to treatment with high doses in children.
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- Academic publications [227883]
- Electronic publications [107344]
- Faculty of Medical Sciences [86219]
- Open Access publications [76470]
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