{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,7]],"date-time":"2026-10-07T01:52:28Z","timestamp":1791337948219,"version":"4.3.1"},"reference-count":0,"publisher":"Oxford University Press (OUP)","issue":"4","license":[{"start":{"date-parts":[[2021,4,1]],"date-time":"2021-04-01T00:00:00Z","timestamp":1617235200000},"content-version":"unspecified","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":["eje.bioscientifica.com"],"crossmark-restriction":true},"short-container-title":[],"published-print":{"date-parts":[[2021,4]]},"abstract":"<jats:sec>\n<jats:title>Objective<\/jats:title>\n<jats:p>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.<\/jats:p>\n<\/jats:sec>\n<jats:sec>\n<jats:title>Design<\/jats:title>\n<jats:p>This retrospective multi-center study, including 31 centers (16 countries), analyzed data from the International-CAH Registry.<\/jats:p>\n<\/jats:sec>\n<jats:sec>\n<jats:title>Methods<\/jats:title>\n<jats:p>Data were collected from 461 patients aged 0\u201318 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.<\/jats:p>\n<\/jats:sec>\n<jats:sec>\n<jats:title>Results<\/jats:title>\n<jats:p>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\u201314.5) mg\/m<jats:sup>2<\/jats:sup>\/day at age 1\u20138 years and the highest dose of 14.0 (11.6\u201317.4) mg\/m<jats:sup>2<\/jats:sup>\/day at age 12\u201318 years. Glucocorticoid doses decreased after 2010 in patients 0\u20138 years (P &lt; 0.001) and remained unchanged in patients aged 8\u201318 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.<\/jats:p>\n<\/jats:sec>\n<jats:sec>\n<jats:title>Conclusions<\/jats:title>\n<jats:p>Data from the I-CAH Registry suggests international variations in hormone replacement therapy, with a tendency to treatment with high doses in children.<\/jats:p>\n<\/jats:sec>","DOI":"10.1530\/eje-20-1249","type":"journal-article","created":{"date-parts":[[2021,1,18]],"date-time":"2021-01-18T18:28:57Z","timestamp":1610994537000},"page":"553-563","update-policy":"https:\/\/doi.org\/10.1530\/crossmarkpolicy-7","source":"Crossref","is-referenced-by-count":38,"title":["International practice of corticosteroid replacement therapy in congenital adrenal hyperplasia: data from the I-CAH registry"],"prefix":"10.1093","volume":"184","author":[{"given":"Irina","family":"Bacila","sequence":"first","affiliation":[{"name":"1Department of Oncology and Metabolism, University of Sheffield, Sheffield, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Nicole","family":"Freeman","sequence":"additional","affiliation":[{"name":"1Department of Oncology and Metabolism, University of Sheffield, Sheffield, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Eleni","family":"Daniel","sequence":"additional","affiliation":[{"name":"1Department of Oncology and Metabolism, University of Sheffield, Sheffield, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Marija","family":"Sandrk","sequence":"additional","affiliation":[{"name":"1Department of Oncology and Metabolism, University of Sheffield, Sheffield, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jillian","family":"Bryce","sequence":"additional","affiliation":[{"name":"2Developmental Endocrinology Research Group, University of Glasgow, Glasgow, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-2178-269X","authenticated-orcid":true,"given":"Salma Rashid","family":"Ali","sequence":"additional","affiliation":[{"name":"2Developmental Endocrinology Research Group, University of Glasgow, Glasgow, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Zehra","family":"Yavas Abali","sequence":"additional","affiliation":[{"name":"3Pediatric Endocrinology and Diabetes, Marmara University, Istanbul, Turkey"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Navoda","family":"Atapattu","sequence":"additional","affiliation":[{"name":"4Pediatric Endocrinology, Lady Ridgeway Hospital, Colombo, Sri Lanka"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Tania A","family":"Bachega","sequence":"additional","affiliation":[{"name":"5Department of Internal Medicine, University of Sao Paulo, Sao Paulo, Brazil"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Antonio","family":"Balsamo","sequence":"additional","affiliation":[{"name":"6Department of Medical and Surgical Sciences, Pediatric Unit, Endo-ERN Center for Rare Endocrine Diseases, S.Orsola-Malpighi University Hospital, Bologna, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Niels","family":"Birkeb\u00e6k","sequence":"additional","affiliation":[{"name":"7Department of Pediatrics, Aarhus University Hospital, Aarhus, Denmark"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Oliver","family":"Blankenstein","sequence":"additional","affiliation":[{"name":"8Institute for Experimental Pediatric Endocrinology and Center for Chronically Sick Children, Charite \u2013 Universit\u00e4tsmedizin Berlin, Berlin, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Walter","family":"Bonfig","sequence":"additional","affiliation":[{"name":"9Department of Pediatrics, Technical University Munich, Munich, Germany"},{"name":"10Department of Pediatrics, Klinikum Wels-Grieskirchen, Wels, Austria"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Martine","family":"Cools","sequence":"additional","affiliation":[{"name":"11Pediatric Endocrinology, Internal Medicine and Pediatric Research Unit, University Hospital Ghent, Ghent University, Ghent, Belgium"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Eduardo Correa","family":"Costa","sequence":"additional","affiliation":[{"name":"12Pediatric Surgery Service, Hospital de Cl\u00ednicas de Porto Alegre, Porto Alegre, Brazil"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Feyza","family":"Darendeliler","sequence":"additional","affiliation":[{"name":"13Paediatric Endocrinology Unit, Istanbul University, Istanbul Faculty of Medicine, Istanbul, Turkey"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Silvia","family":"Einaudi","sequence":"additional","affiliation":[{"name":"14Department of Paediatric Endocrinology, Regina Margherita Children\u2019s Hospital, University of Torino, Torino, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Heba Hassan","family":"Elsedfy","sequence":"additional","affiliation":[{"name":"15Pediatrics Department, Ain Shams University, Cairo, Egypt"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Martijn","family":"Finken","sequence":"additional","affiliation":[{"name":"16Department of Paediatric Endocrinology, Emma Children\u2019s Hospital, Amsterdam UMC, Vrije Universiteit Amsterdam, Amsterdam, Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Evelien","family":"Gevers","sequence":"additional","affiliation":[{"name":"17Centre for Endocrinology, William Harvey Research Institute, Queen Mary University London, London, UK"},{"name":"18Department of Paediatric Endocrinology, Barts Health NHS Trust \u2013 Royal London Hospital, London, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Hedi L","family":"Claahsen-van der Grinten","sequence":"additional","affiliation":[{"name":"19Department of Pediatric Endocrinology, Radboud University Medical Centre, Nijmegen, Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-2658-6866","authenticated-orcid":true,"given":"Tulay","family":"Guran","sequence":"additional","affiliation":[{"name":"3Pediatric Endocrinology and Diabetes, Marmara University, Istanbul, Turkey"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ayla","family":"G\u00fcven","sequence":"additional","affiliation":[{"name":"20Saglik Bilimleri University, Medical Faculty Zeynep Kamil Maternity and Children Hospital, Pediatric Endocrinology Clinic, Istanbul, Turkey"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sabine E","family":"Hannema","sequence":"additional","affiliation":[{"name":"21Department of Pediatric Endocrinology, Sophia Children\u2019s Hospital, Erasmus Medical Centre, Rotterdam, Netherlands"},{"name":"22Department of Paediatrics, Leiden University Medical Centre, Leiden, Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Claire E","family":"Higham","sequence":"additional","affiliation":[{"name":"23Department of Endocrinology, Christie Hospital NHS Foundation Trust, Manchester, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Violeta","family":"Iotova","sequence":"additional","affiliation":[{"name":"24Department of Paediatrics, Medical University of Varna, Varna, Bulgaria"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Hetty J","family":"van der Kamp","sequence":"additional","affiliation":[{"name":"25Pediatric Endocrinology Wilhelmina Children\u2019s Hospital, University Medical Centre Utrecht, Utrecht, Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Marta","family":"Korbonits","sequence":"additional","affiliation":[{"name":"17Centre for Endocrinology, William Harvey Research Institute, Queen Mary University London, London, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ruth E","family":"Krone","sequence":"additional","affiliation":[{"name":"26Department of Endocrinology and Diabetes, Birmingham Women\u2019s and Children\u2019s Hospital, Birmingham, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Corina","family":"Lichiardopol","sequence":"additional","affiliation":[{"name":"27Department of Endocrinology, University of Medicine and Pharmacy Craiova, Craiova, Romania"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Andrea","family":"Luczay","sequence":"additional","affiliation":[{"name":"28Semmelweis University, Budapest, Hungary"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Berenice Bilharinho","family":"Mendonca","sequence":"additional","affiliation":[{"name":"5Department of Internal Medicine, University of Sao Paulo, Sao Paulo, Brazil"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Tatjana","family":"Milenkovic","sequence":"additional","affiliation":[{"name":"29Department of Endocrinology, Institute for Mother and Child Healthcare of Serbia \u2018Dr Vukan \u010cupi\u0107\u2019 Belgrade, Serbia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mirela C","family":"Miranda","sequence":"additional","affiliation":[{"name":"5Department of Internal Medicine, University of Sao Paulo, Sao Paulo, Brazil"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Klaus","family":"Mohnike","sequence":"additional","affiliation":[{"name":"30Department of Pediatrics, Otto-von-Guericke University, Magdeburg, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Uta","family":"Neumann","sequence":"additional","affiliation":[{"name":"8Institute for Experimental Pediatric Endocrinology and Center for Chronically Sick Children, Charite \u2013 Universit\u00e4tsmedizin Berlin, Berlin, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Rita","family":"Ortolano","sequence":"additional","affiliation":[{"name":"6Department of Medical and Surgical Sciences, Pediatric Unit, Endo-ERN Center for Rare Endocrine Diseases, S.Orsola-Malpighi University Hospital, Bologna, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sukran","family":"Poyrazoglu","sequence":"additional","affiliation":[{"name":"13Paediatric Endocrinology Unit, Istanbul University, Istanbul Faculty of Medicine, Istanbul, Turkey"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ajay","family":"Thankamony","sequence":"additional","affiliation":[{"name":"31Department of Pediatrics, University of Cambridge, Cambridge Biomedical Campus, Cambridge, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jeremy W","family":"Tomlinson","sequence":"additional","affiliation":[{"name":"32Oxford Centre for Diabetes, Endocrinology & Metabolism, NIHR Oxford Biomedical Research Centre, Churchill Hospital, Oxford, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ana","family":"Vieites","sequence":"additional","affiliation":[{"name":"33Centro de Investigaciones Endocrinol\u00f3gicas (CEDIE-CONICET), Hospital de Ni\u00f1os Ricardo Guti\u00e9rrez, Buenos Aires, Argentina"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Liat","family":"de Vries","sequence":"additional","affiliation":[{"name":"34Institute for Diabetes and Endocrinology, Schneider\u2019s Children Medical Center of Israel, Petah-Tikvah, Israel"},{"name":"35Sackler School of Medicine, Tel-Aviv University, Tel-Aviv, Israel"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-0689-5549","authenticated-orcid":true,"given":"S Faisal","family":"Ahmed","sequence":"additional","affiliation":[{"name":"2Developmental Endocrinology Research Group, University of Glasgow, Glasgow, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Richard J","family":"Ross","sequence":"additional","affiliation":[{"name":"1Department of Oncology and Metabolism, University of Sheffield, Sheffield, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Nils P","family":"Krone","sequence":"additional","affiliation":[{"name":"1Department of Oncology and Metabolism, University of Sheffield, Sheffield, UK"},{"name":"36Department of Medicine III, University Hospital Carl Gustav Carus, Technische Universit\u00e4t Dresden, Dresden, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"286","container-title":["European Journal of 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