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

Author:

Bacila Irina1,Freeman Nicole1,Daniel Eleni1,Sandrk Marija1,Bryce Jillian2,Ali Salma Rashid2ORCID,Yavas Abali Zehra3,Atapattu Navoda4,Bachega Tania A5,Balsamo Antonio6,Birkebæk Niels7,Blankenstein Oliver8,Bonfig Walter910,Cools Martine11,Costa Eduardo Correa12,Darendeliler Feyza13,Einaudi Silvia14,Elsedfy Heba Hassan15,Finken Martijn16,Gevers Evelien1718,Claahsen-van der Grinten Hedi L19,Guran Tulay3ORCID,Güven Ayla20,Hannema Sabine E2122,Higham Claire E23,Iotova Violeta24,van der Kamp Hetty J25,Korbonits Marta17,Krone Ruth E26,Lichiardopol Corina27,Luczay Andrea28,Mendonca Berenice Bilharinho5,Milenkovic Tatjana29,Miranda Mirela C5,Mohnike Klaus30,Neumann Uta8,Ortolano Rita6,Poyrazoglu Sukran13,Thankamony Ajay31,Tomlinson Jeremy W32,Vieites Ana33,de Vries Liat3435,Ahmed S Faisal2ORCID,Ross Richard J1,Krone Nils P136

Affiliation:

1. 1Department of Oncology and Metabolism, University of Sheffield, Sheffield, UK

2. 2Developmental Endocrinology Research Group, University of Glasgow, Glasgow, UK

3. 3Pediatric Endocrinology and Diabetes, Marmara University, Istanbul, Turkey

4. 4Pediatric Endocrinology, Lady Ridgeway Hospital, Colombo, Sri Lanka

5. 5Department of Internal Medicine, University of Sao Paulo, Sao Paulo, Brazil

6. 6Department of Medical and Surgical Sciences, Pediatric Unit, Endo-ERN Center for Rare Endocrine Diseases, S.Orsola-Malpighi University Hospital, Bologna, Italy

7. 7Department of Pediatrics, Aarhus University Hospital, Aarhus, Denmark

8. 8Institute for Experimental Pediatric Endocrinology and Center for Chronically Sick Children, Charite – Universitätsmedizin Berlin, Berlin, Germany

9. 9Department of Pediatrics, Technical University Munich, Munich, Germany

10. 10Department of Pediatrics, Klinikum Wels-Grieskirchen, Wels, Austria

11. 11Pediatric Endocrinology, Internal Medicine and Pediatric Research Unit, University Hospital Ghent, Ghent University, Ghent, Belgium

12. 12Pediatric Surgery Service, Hospital de Clínicas de Porto Alegre, Porto Alegre, Brazil

13. 13Paediatric Endocrinology Unit, Istanbul University, Istanbul Faculty of Medicine, Istanbul, Turkey

14. 14Department of Paediatric Endocrinology, Regina Margherita Children’s Hospital, University of Torino, Torino, Italy

15. 15Pediatrics Department, Ain Shams University, Cairo, Egypt

16. 16Department of Paediatric Endocrinology, Emma Children’s Hospital, Amsterdam UMC, Vrije Universiteit Amsterdam, Amsterdam, Netherlands

17. 17Centre for Endocrinology, William Harvey Research Institute, Queen Mary University London, London, UK

18. 18Department of Paediatric Endocrinology, Barts Health NHS Trust – Royal London Hospital, London, UK

19. 19Department of Pediatric Endocrinology, Radboud University Medical Centre, Nijmegen, Netherlands

20. 20Saglik Bilimleri University, Medical Faculty Zeynep Kamil Maternity and Children Hospital, Pediatric Endocrinology Clinic, Istanbul, Turkey

21. 21Department of Pediatric Endocrinology, Sophia Children’s Hospital, Erasmus Medical Centre, Rotterdam, Netherlands

22. 22Department of Paediatrics, Leiden University Medical Centre, Leiden, Netherlands

23. 23Department of Endocrinology, Christie Hospital NHS Foundation Trust, Manchester, UK

24. 24Department of Paediatrics, Medical University of Varna, Varna, Bulgaria

25. 25Pediatric Endocrinology Wilhelmina Children’s Hospital, University Medical Centre Utrecht, Utrecht, Netherlands

26. 26Department of Endocrinology and Diabetes, Birmingham Women’s and Children’s Hospital, Birmingham, UK

27. 27Department of Endocrinology, University of Medicine and Pharmacy Craiova, Craiova, Romania

28. 28Semmelweis University, Budapest, Hungary

29. 29Department of Endocrinology, Institute for Mother and Child Healthcare of Serbia ‘Dr Vukan Čupić’ Belgrade, Serbia

30. 30Department of Pediatrics, Otto-von-Guericke University, Magdeburg, Germany

31. 31Department of Pediatrics, University of Cambridge, Cambridge Biomedical Campus, Cambridge, UK

32. 32Oxford Centre for Diabetes, Endocrinology & Metabolism, NIHR Oxford Biomedical Research Centre, Churchill Hospital, Oxford, UK

33. 33Centro de Investigaciones Endocrinológicas (CEDIE-CONICET), Hospital de Niños Ricardo Gutiérrez, Buenos Aires, Argentina

34. 34Institute for Diabetes and Endocrinology, Schneider’s Children Medical Center of Israel, Petah-Tikvah, Israel

35. 35Sackler School of Medicine, Tel-Aviv University, Tel-Aviv, Israel

36. 36Department 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.

Publisher

Bioscientifica

Subject

Endocrinology,General Medicine,Endocrinology, Diabetes and Metabolism

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