1. International practice of corticosteroid replacement therapy in congenital adrenal hyperplasia
- Author
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S. F. Ahmed, Tatjana Milenkovic, Eduardo Corrêa Costa, Ruth Krone, Berenice B Mendonca, Niels H Birkebaek, Tania A. S. S. Bachega, Andrea Luczay, Irina-Alexandra Bacila, Martijn J J Finken, Sukran Poyrazoglu, Z. Yavas Abalı, Feyza Darendeliler, Tulay Guran, Eleni Daniel, H. J. van der Kamp, Márta Korbonits, Ajay Thankamony, Ana Vieites, Oliver Blankenstein, Heba Elsedfy, Antonio Balsamo, M. Sandrk, Nils Krone, N. Freeman, Jeremy W. Tomlinson, Klaus Mohnike, Corina Lichiardopol, H.L. Claahsen-van der Grinten, Martine Cools, Walter Bonfig, Salma R Ali, Mirela C Miranda, Rita Ortolano, L. de Vries, Navoda Atapattu, Sabine E. Hannema, Silvia Einaudi, Evelien F. Gevers, J. Bryce, Claire E Higham, Ayla Guven, Richard J. Ross, Violeta Iotova, Uta Neumann, Pediatric surgery, Amsterdam Gastroenterology Endocrinology Metabolism, Amsterdam Reproduction & Development (AR&D), and Pediatrics
- Subjects
Male ,Pediatrics ,Hydrocortisone ,Endocrinology, Diabetes and Metabolism ,CHILDREN ,0302 clinical medicine ,Endocrinology ,INSUFFICIENCY ,Interquartile range ,Adrenal Cortex Hormones ,Medicine and Health Sciences ,Practice Patterns, Physicians'/statistics & numerical data ,Registries ,Practice Patterns, Physicians' ,Hydrocortisone/administration & dosage ,Child ,Age Factors ,Vascular damage Radboud Institute for Molecular Life Sciences [Radboudumc 16] ,General Medicine ,Diabetes and Metabolism ,Hormone Replacement Therapy/methods ,Transgender hormone therapy ,030220 oncology & carcinogenesis ,Fludrocortisone ,Child, Preschool ,INFANCY ,Corticosteroid ,GROWTH ,Female ,Glucocorticoid ,medicine.drug ,medicine.medical_specialty ,Fludrocortisone/administration & dosage ,Adolescent ,medicine.drug_class ,Hormone Replacement Therapy ,DISORDERS ,030209 endocrinology & metabolism ,21-HYDROXYLASE DEFICIENCY ,03 medical and health sciences ,Adrenal Cortex Hormones/administration & dosage ,SDG 3 - Good Health and Well-being ,Internal medicine ,medicine ,MANAGEMENT ,Humans ,Congenital adrenal hyperplasia ,Glucocorticoids ,Retrospective Studies ,Adrenal Hyperplasia, Congenital ,business.industry ,CORTISOL ,HYDROCORTISONE ,Infant, Newborn ,Infant ,Glucocorticoids/administration & dosage ,VELOCITY ,medicine.disease ,Mineralocorticoid ,business ,Adrenal Hyperplasia, Congenital/drug therapy - Abstract
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.
- Published
- 2021