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

in European Journal of Endocrinology
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  • 1 I Bacila, Oncology and Metabolism, The University of Sheffield, Sheffield, S10 2TN, United Kingdom of Great Britain and Northern Ireland
  • 2 N Freeman, Medical School, The University of Sheffield, Sheffield, United Kingdom of Great Britain and Northern Ireland
  • 3 E Daniel, Department of Oncology and Metabolism, The Medical School, University of Sheffield, Sheffield, S10 2RX, United Kingdom of Great Britain and Northern Ireland
  • 4 M Sandrk, Oncology and Metabolism, The University of Sheffield, Sheffield, United Kingdom of Great Britain and Northern Ireland
  • 5 J Bryce, Developmental Endocrinology Research Group, University of Glasgow School of Medicine, Glasgow, G51 4TF, United Kingdom of Great Britain and Northern Ireland
  • 6 S Ali, Developmental Endocrinology Research Group, University of Glasgow, Glasgow, G51 4TF, United Kingdom of Great Britain and Northern Ireland
  • 7 Z Yavas Abalı, Pediatric Endocrinology, Istanbul Universitesi, Fatih, Turkey
  • 8 N Atapattu, University Paediatric Unit, Lady Ridgeway Hospital for Children, Colombo, Sri Lanka
  • 9 T Bachega, Endocrinology and Development Unit, Hospital das Clinicas, Sao Paulo, 05403900, Brazil
  • 10 A Balsamo, Pediatrics, University of Bologna Hospital of Bologna Sant'Orsola-Malpighi Polyclinic, Bologna, Italy
  • 11 N Birkebæk, Department of Pediatrics, Aarhus Universitetshospital, Aarhus, Denmark
  • 12 O Blankenstein, Center for Chronic Sick Children, Charité-Universitätsmedizin Berlin, Berlin, 13353, Germany
  • 13 W Bonfig, Department of Pediatrics, Klinikum Wels - Grieskirchen GmbH, Wels, 4600, Austria
  • 14 M Cools, Pediatrics, University Hospital Ghent, Ghent, 9000, Belgium
  • 15 E Costa, Pediatric Surgery Service, Hospital de Clínicas de Porto Alegre, Porto Alegre, Brazil
  • 16 F Darendeliler, Department of Pediatrics, Pediatric Endocrinology Unit, Istanbul University, Istanbul Faculty of Medicine, Istanbul, 34390, Turkey
  • 17 S Einaudi, Department of Public Health and Pediatric Sciences, Universita degli Studi di Torino, Torino, Italy
  • 18 H Elsedfy, Pediatrics, Ain Shams University, Cairo, 11511, Egypt
  • 19 M Finken, Department of Paediatric Endocrinology, Emma Children’s Hospital, Amsterdam, Amsterdam, Netherlands
  • 20 E Gevers, Centre for Endocrinology, William Harvey Research Institute, Queen Mary University of London, London, United Kingdom of Great Britain and Northern Ireland
  • 21 H Claahsen-van der Grinten, Pediatrics, Radboud University Medical Center, Nijmegen, Netherlands
  • 22 T Guran, Department of Pediatric Endocrinology, Marmara University, Istanbul, Turkey
  • 23 A Güven, Pediatric Endocrinology, Marmara Universitesi, Istanbul, Turkey
  • 24 S Hannema, Dept of Pediatrics, Leiden University Medical Center, Leiden, Netherlands
  • 25 C Higham, Department of Endocrinology, The Christie NHS Foundation Trust, Manchester, United Kingdom of Great Britain and Northern Ireland
  • 26 V Iotova, Department of Paediatrics, Medical University of Varna, Varna, Bulgaria
  • 27 H van der Kamp, Pediatric Endocrinology, UMC Utrecht - Locatie WKZ, Utrecht, Netherlands
  • 28 M Korbonits, Centre for Endocrinology, William Harvey Research Institute, Queen Mary University of London, London, United Kingdom of Great Britain and Northern Ireland
  • 29 R Krone, Department of Endocrinology and Diabetes , Birmingham Women's and Children's NHS Foundation Trust, Birmingham, United Kingdom of Great Britain and Northern Ireland
  • 30 C Lichiardopol, Endocrinology, University of Medicine and Pharmacy Craiova, Craiova, 200340, Romania
  • 31 A Luczay, 2nd Department of Pediatrics, Semmelweis Egyetem, Budapest, Hungary
  • 32 B Mendonca, Internal Medicine, University of Sao Paulo, Sao Paulo, Brazil
  • 33 T Milenkovic, Endocrinology, Institute for Mother and Child Healthcare of Serbia, Belgrade, Serbia
  • 34 M Miranda, Department of Internal Medicine, University of Sao Paulo, Sao Paulo, Brazil
  • 35 K Mohnike, Department of Pediatrics, Otto von Guericke Universitat Magdeburg, Magdeburg, 39120, Germany
  • 36 U Neumann, Institute for experimental pediatric endocrinology, Charité University medicine Berlin, Campus Virchow Klinikum, Berlin, 13353, United Kingdom of Great Britain and Northern Ireland
  • 37 R Ortolano, Pediatric Unit, University of Bologna Hospital of Bologna Sant'Orsola-Malpighi Polyclinic, Bologna, Italy
  • 38 S Poyrazoglu, Department of Pediatrics, Pediatric Endocrinology Unit, Istanbul University, Istanbul Faculty of Medicine, Istanbul, 34390, Turkey
  • 39 A Thankamony, Department of Paediatrics, University of Cambridge, Cambridge, United Kingdom of Great Britain and Northern Ireland
  • 40 J Tomlinson, Centre for Endocrinology, Diabetes and Metabolism, NIHR Oxford Biomedical Research Centre, Oxford, United Kingdom of Great Britain and Northern Ireland
  • 41 A Vieites, Centro de Investigaciones Endocrinológicas (CEDIE-CONICET), Hospital de Niños Ricardo Gutiérrez, Buenos Aires, Argentina
  • 42 L de Vries, The Jesse Z and Sara Lea Shafer Institute for Endocrinology and Diabetes, Schneider Children’s Medical Center of Israel, Petah-Tikva, 49202, Israel
  • 43 S Ahmed, Developmental Endocrinology Research Group, University of Glasgow, Glasgow, G51 4TF, United Kingdom of Great Britain and Northern Ireland
  • 44 R Ross, Department of Oncology and Metabolism, The University of Sheffield, Sheffield, United Kingdom of Great Britain and Northern Ireland
  • 45 N Krone, Academic Unit of Child Health, Department of Oncology and Metabolism, University of Sheffield, Sheffield, S10 2TG, United Kingdom of Great Britain and Northern Ireland

Correspondence: Nils Krone, Email: n.krone@sheffield.ac.uk

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 was collected from 461 patients aged 0-18 years with classic 21-hydroxylase deficiency (54.9% females) under follow-up between 1982 – 2018. Type, dose and timing of glucocorticoid and mineralocorticoid replacement was 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.

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