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

Autor:	Bacila I; Department of Oncology and Metabolism, University of Sheffield, Sheffield, UK., Freeman N; Department of Oncology and Metabolism, University of Sheffield, Sheffield, UK., Daniel E; Department of Oncology and Metabolism, University of Sheffield, Sheffield, UK., Sandrk M; Department of Oncology and Metabolism, University of Sheffield, Sheffield, UK., Bryce J; Developmental Endocrinology Research Group, University of Glasgow, Glasgow, UK., Ali SR; Developmental Endocrinology Research Group, University of Glasgow, Glasgow, UK., Yavas Abali Z; Pediatric Endocrinology and Diabetes, Marmara University, Istanbul, Turkey., Atapattu N; Pediatric Endocrinology, Lady Ridgeway Hospital, Colombo, Sri Lanka., Bachega TA; Department of Internal Medicine, University of Sao Paulo, Sao Paulo, Brazil., Balsamo A; Department of Medical and Surgical Sciences, Pediatric Unit, Endo-ERN Center for Rare Endocrine Diseases, S.Orsola-Malpighi University Hospital, Bologna, Italy., Birkebæk N; Department of Pediatrics, Aarhus University Hospital, Aarhus, Denmark., Blankenstein O; Institute for Experimental Pediatric Endocrinology and Center for Chronically Sick Children, Charite - Universitätsmedizin Berlin, Berlin, Germany., Bonfig W; Department of Pediatrics, Technical University Munich, Munich, Germany.; Department of Pediatrics, Klinikum Wels-Grieskirchen, Wels, Austria., Cools M; Pediatric Endocrinology, Internal Medicine and Pediatric Research Unit, University Hospital Ghent, Ghent University, Ghent, Belgium., Costa EC; Pediatric Surgery Service, Hospital de Clínicas de Porto Alegre, Porto Alegre, Brazil., Darendeliler F; Paediatric Endocrinology Unit, Istanbul University, Istanbul Faculty of Medicine, Istanbul, Turkey., Einaudi S; Department of Paediatric Endocrinology, Regina Margherita Children's Hospital, University of Torino, Torino, Italy., Elsedfy HH; Pediatrics Department, Ain Shams University, Cairo, Egypt., Finken M; Department of Paediatric Endocrinology, Emma Children's Hospital, Amsterdam UMC, Vrije Universiteit Amsterdam, Amsterdam, Netherlands., Gevers E; Centre for Endocrinology, William Harvey Research Institute, Queen Mary University London, London, UK.; Department of Paediatric Endocrinology, Barts Health NHS Trust - Royal London Hospital, London, UK., Claahsen-van der Grinten HL; Department of Pediatric Endocrinology, Radboud University Medical Centre, Nijmegen, Netherlands., Guran T; Pediatric Endocrinology and Diabetes, Marmara University, Istanbul, Turkey., Güven A; Saglik Bilimleri University, Medical Faculty Zeynep Kamil Maternity and Children Hospital, Pediatric Endocrinology Clinic, Istanbul, Turkey., Hannema SE; Department of Pediatric Endocrinology, Sophia Children's Hospital, Erasmus Medical Centre, Rotterdam, Netherlands.; Department of Paediatrics, Leiden University Medical Centre, Leiden, Netherlands., Higham CE; Department of Endocrinology, Christie Hospital NHS Foundation Trust, Manchester, UK., Iotova V; Department of Paediatrics, Medical University of Varna, Varna, Bulgaria., van der Kamp HJ; Pediatric Endocrinology Wilhelmina Children's Hospital, University Medical Centre Utrecht, Utrecht, Netherlands., Korbonits M; Centre for Endocrinology, William Harvey Research Institute, Queen Mary University London, London, UK., Krone RE; Department of Endocrinology and Diabetes, Birmingham Women's and Children's Hospital, Birmingham, UK., Lichiardopol C; Department of Endocrinology, University of Medicine and Pharmacy Craiova, Craiova, Romania., Luczay A; Semmelweis University, Budapest, Hungary., Mendonca BB; Department of Internal Medicine, University of Sao Paulo, Sao Paulo, Brazil., Milenkovic T; Department of Endocrinology, Institute for Mother and Child Healthcare of Serbia 'Dr Vukan Čupić' Belgrade, Serbia., Miranda MC; Department of Internal Medicine, University of Sao Paulo, Sao Paulo, Brazil., Mohnike K; Department of Pediatrics, Otto-von-Guericke University, Magdeburg, Germany., Neumann U; Institute for Experimental Pediatric Endocrinology and Center for Chronically Sick Children, Charite - Universitätsmedizin Berlin, Berlin, Germany., Ortolano R; Department of Medical and Surgical Sciences, Pediatric Unit, Endo-ERN Center for Rare Endocrine Diseases, S.Orsola-Malpighi University Hospital, Bologna, Italy., Poyrazoglu S; Paediatric Endocrinology Unit, Istanbul University, Istanbul Faculty of Medicine, Istanbul, Turkey., Thankamony A; Department of Pediatrics, University of Cambridge, Cambridge Biomedical Campus, Cambridge, UK., Tomlinson JW; Oxford Centre for Diabetes, Endocrinology & Metabolism, NIHR Oxford Biomedical Research Centre, Churchill Hospital, Oxford, UK., Vieites A; Centro de Investigaciones Endocrinológicas (CEDIE-CONICET), Hospital de Niños Ricardo Gutiérrez, Buenos Aires, Argentina., de Vries L; Institute for Diabetes and Endocrinology, Schneider's Children Medical Center of Israel, Petah-Tikvah, Israel.; Sackler School of Medicine, Tel-Aviv University, Tel-Aviv, Israel., Ahmed SF; Developmental Endocrinology Research Group, University of Glasgow, Glasgow, UK., Ross RJ; Department of Oncology and Metabolism, University of Sheffield, Sheffield, UK., Krone NP; Department of Oncology and Metabolism, University of Sheffield, Sheffield, UK.; Department of Medicine III, University Hospital Carl Gustav Carus, Technische Universität Dresden, Dresden, Germany.
Jazyk:	angličtina
Zdroj:	European journal of endocrinology [Eur J Endocrinol] 2021 Apr; Vol. 184 (4), pp. 553-563.
DOI:	10.1530/EJE-20-1249
Abstrakt:	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.
Databáze:	MEDLINE
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