International practice of corticosteroid replacement therapy in congenital adrenal hyperplasia: Data from the I-CAH registry

Irina Bacila, Nicole Freeman, Eleni Daniel, Marija Sandrk, Jillian Bryce, Salma Rashid Ali, Zehra Yavas Abali, Navoda Atapattu, Tania A. Bachega, Antonio Balsamo, Niels Birkebæk, Oliver Blankenstein, Walter Bonfig, Martine Cools, Eduardo Correa Costa, Feyza Darendeliler, Silvia Einaudi, Heba Hassan Elsedfy, Martijn J. J. Finken, Evelien GeversHedi L. Claahsen-van der Grinten, Tulay Guran, Ayla Güven, Sabine E. Hannema, Claire E. Higham, Violeta Iotova, Hetty J. van der Kamp, Marta Korbonits, Ruth E. Krone, Corina Lichiardopol, Andrea Luczay, Berenice Bilharinho Mendonca, Tatjana Milenkovic, Mirela C. Miranda, Klaus Mohnike, Uta Neumann, Rita Ortolano, Sukran Poyrazoglu, Ajay Thankamony, Jeremy W. Tomlinson, Ana Vieites, Liat de Vries, S. Faisal Ahmed, Richard J. Ross, Nils P. Krone

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21 Citations (Scopus)


Objective: Despite published guidelines no unified approach to hormone replacement in congenital adrenal hyperplasia (CAH) exists. We aimed to explore geographical and temporal var iations in the treatment with glucocorticoids and mineralocorticoids in CAH. Design: This retrospective multi-center study, including 31 centers (1 6 countries), analyzed data from the International-CAH Registry. Methods: Data were collected from 461 patients aged 0-18 years with cla ssic 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-equival ent dose for body surface, with the lowest dose (median with interquartile range) of 12.0 (10.0-14.5) mg/m 2/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 2 010 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 countr ies 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.
Original languageEnglish
Pages (from-to)553-563
Number of pages11
JournalEuropean journal of endocrinology
Issue number4
Publication statusPublished - 1 Apr 2021


  • Adolescent
  • Adrenal Cortex Hormones/administration & dosage
  • Adrenal Hyperplasia, Congenital/drug therapy
  • Age Factors
  • Child
  • Child, Preschool
  • Female
  • Fludrocortisone/administration & dosage
  • Glucocorticoids/administration & dosage
  • Hormone Replacement Therapy/methods
  • Humans
  • Hydrocortisone/administration & dosage
  • Infant
  • Infant, Newborn
  • Male
  • Practice Patterns, Physicians'/statistics & numerical data
  • Registries
  • Retrospective Studies

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