Irina Bacila1, Nicole Freeman1, Eleni Daniel1, Marija Sandrk1, Jillian Bryce2, Salma Rashid Ali2, Zehra Yavas Abali3, Navoda Atapattu4, Tania A Bachega5, Antonio Balsamo6, Niels Birkebæk7, Oliver Blankenstein8, Walter Bonfig9,10, Martine Cools11, Eduardo Correa Costa12, Feyza Darendeliler13, Silvia Einaudi14, Heba Hassan Elsedfy15, Martijn Finken16, Evelien Gevers17,18, Hedi L Claahsen-van der Grinten19, Tulay Guran3, Ayla Güven20, Sabine E Hannema21,22, Claire E Higham23, Violeta Iotova24, Hetty J van der Kamp25, Marta Korbonits17, Ruth E Krone26, Corina Lichiardopol27, Andrea Luczay28, Berenice Bilharinho Mendonca5, Tatjana Milenkovic29, Mirela C Miranda5, Klaus Mohnike30, Uta Neumann8, Rita Ortolano6, Sukran Poyrazoglu13, Ajay Thankamony31, Jeremy W Tomlinson32, Ana Vieites33, Liat de Vries34,35, S Faisal Ahmed2, Richard J Ross1, Nils P Krone1,36. 1. Department of Oncology and Metabolism, University of Sheffield, Sheffield, UK. 2. Developmental Endocrinology Research Group, University of Glasgow, Glasgow, UK. 3. Pediatric Endocrinology and Diabetes, Marmara University, Istanbul, Turkey. 4. Pediatric Endocrinology, Lady Ridgeway Hospital, Colombo, Sri Lanka. 5. Department of Internal Medicine, University of Sao Paulo, Sao Paulo, Brazil. 6. Department of Medical and Surgical Sciences, Pediatric Unit, Endo-ERN Center for Rare Endocrine Diseases, S.Orsola-Malpighi University Hospital, Bologna, Italy. 7. Department of Pediatrics, Aarhus University Hospital, Aarhus, Denmark. 8. Institute for Experimental Pediatric Endocrinology and Center for Chronically Sick Children, Charite - Universitätsmedizin Berlin, Berlin, Germany. 9. Department of Pediatrics, Technical University Munich, Munich, Germany. 10. Department of Pediatrics, Klinikum Wels-Grieskirchen, Wels, Austria. 11. Pediatric Endocrinology, Internal Medicine and Pediatric Research Unit, University Hospital Ghent, Ghent University, Ghent, Belgium. 12. Pediatric Surgery Service, Hospital de Clínicas de Porto Alegre, Porto Alegre, Brazil. 13. Paediatric Endocrinology Unit, Istanbul University, Istanbul Faculty of Medicine, Istanbul, Turkey. 14. Department of Paediatric Endocrinology, Regina Margherita Children's Hospital, University of Torino, Torino, Italy. 15. Pediatrics Department, Ain Shams University, Cairo, Egypt. 16. Department of Paediatric Endocrinology, Emma Children's Hospital, Amsterdam UMC, Vrije Universiteit Amsterdam, Amsterdam, Netherlands. 17. Centre for Endocrinology, William Harvey Research Institute, Queen Mary University London, London, UK. 18. Department of Paediatric Endocrinology, Barts Health NHS Trust - Royal London Hospital, London, UK. 19. Department of Pediatric Endocrinology, Radboud University Medical Centre, Nijmegen, Netherlands. 20. Saglik Bilimleri University, Medical Faculty Zeynep Kamil Maternity and Children Hospital, Pediatric Endocrinology Clinic, Istanbul, Turkey. 21. Department of Pediatric Endocrinology, Sophia Children's Hospital, Erasmus Medical Centre, Rotterdam, Netherlands. 22. Department of Paediatrics, Leiden University Medical Centre, Leiden, Netherlands. 23. Department of Endocrinology, Christie Hospital NHS Foundation Trust, Manchester, UK. 24. Department of Paediatrics, Medical University of Varna, Varna, Bulgaria. 25. Pediatric Endocrinology Wilhelmina Children's Hospital, University Medical Centre Utrecht, Utrecht, Netherlands. 26. Department of Endocrinology and Diabetes, Birmingham Women's and Children's Hospital, Birmingham, UK. 27. Department of Endocrinology, University of Medicine and Pharmacy Craiova, Craiova, Romania. 28. Semmelweis University, Budapest, Hungary. 29. Department of Endocrinology, Institute for Mother and Child Healthcare of Serbia 'Dr Vukan Čupić' Belgrade, Serbia. 30. Department of Pediatrics, Otto-von-Guericke University, Magdeburg, Germany. 31. Department of Pediatrics, University of Cambridge, Cambridge Biomedical Campus, Cambridge, UK. 32. Oxford Centre for Diabetes, Endocrinology & Metabolism, NIHR Oxford Biomedical Research Centre, Churchill Hospital, Oxford, UK. 33. Centro de Investigaciones Endocrinológicas (CEDIE-CONICET), Hospital de Niños Ricardo Gutiérrez, Buenos Aires, Argentina. 34. Institute for Diabetes and Endocrinology, Schneider's Children Medical Center of Israel, Petah-Tikvah, Israel. 35. Sackler School of Medicine, Tel-Aviv University, Tel-Aviv, Israel. 36. Department of Medicine III, University Hospital Carl Gustav Carus, Technische Universität Dresden, Dresden, Germany.
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.
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.
Authors: Özge Besci; İbrahim Mert Erbaş; Tuncay Küme; Kübra Yüksek Acinikli; Ayhan Abacı; Ece Böber; Korcan Demir Journal: J Clin Res Pediatr Endocrinol Date: 2021-12-06
Authors: Irina Bacila; Neil Richard Lawrence; Sundus Mahdi; Sabah Alvi; Timothy D Cheetham; Elizabeth Crowne; Urmi Das; Mehul Tulsidas Dattani; Justin H Davies; Evelien Gevers; Ruth E Krone; Andreas Kyriakou; Leena Patel; Tabitha Randell; Fiona J Ryan; Brian Keevil; S Faisal Ahmed; Nils P Krone Journal: Eur J Endocrinol Date: 2022-09-16 Impact factor: 6.558