Hydrocortisone dosing in children with classic congenital adrenal hyperplasia: results of the German/Austrian registry

in Endocrine Connections
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  • 1 H Hoyer-Kuhn, Department of Paediatrics, Faculty of Medicine and University Hospital Cologne, University of Cologne, Cologne, 50937, Germany
  • 2 A Huebner, Department of Paediatrics, University Hospital Carl Gustav Carus Dresden, Medizinische Fakultät Carl Gustav Carus, Technische Universität Dresden, Dresden, Germany
  • 3 A Richter-Unruh, Sektion Kinderendokrinologie und Diabetologie, Klinik für Kinder- und Jugendmedizin der Ruhr-Universität Bochum im St. Josef-Hospital, Bochum, Germany
  • 4 M Bettendorf, University Children’s Hospital Heidelberg, University Heidelberg, Germany, Heidelberg, Germany
  • 5 T Rohrer, Division of Paediatric Endocrinology, Saarland University Medical Centre, Homburg, 66424, Germany
  • 6 K Kapelari, Pediatric and Adolescent Medicine, Medical University Innsbruck, Innsbruck, 6020, Austria
  • 7 S Riedl, Pediatric Department, Medical University of Vienna, Vienna, Austria, Austria
  • 8 K Mohnike, Department of Pediatrics, Otto von Guericke Universitat Magdeburg, Magdeburg, 39120, Germany
  • 9 H Dörr, Paediatrics, University Hospital Erlangen, Erlangen, 91054, Germany
  • 10 F Roehl, Department of Biometrics, Otto von Guericke Universität Magdeburg, Germany, Otto von Guericke Universität Magdeburg, Germany, Magdeburg, Germany
  • 11 K Fink, Institute of Epidemiology and Medical Biometry, ZIBMT, German Center for Diabetes Research (DZD), University of Ulm, Ulm, Germany
  • 12 R Holl, Institute of Epidemiology and Medical Biometry, ZIBMT, German Center for Diabetes Research (DZD) , University of Ulm, Ulm, Germany
  • 13 J Woelfle, Children’s Hospital, University of Erlangen, Erlangen, Germany

Correspondence: Heike Hoyer-Kuhn, Email: heike-katharina.hoyer-kuhn@uk-koeln.de

Objective: Treatment in classic congenital adrenal hyperplasia (CAH) is necessary to compensate for glucocorticoid/mineralocorticoid deficiencies and to suppress androgen excess. Hydrocortisone (HC) is preferred in growing children with classic CAH, but recommendations regarding dosage/administration are inconsistent. Aim of the study was to evaluate HC dosing in children with CAH in relation to chronological age, sex, and phenotype based on a multicentre CAH registry.

Design: The CAH registry was initiated in 1997 by the AQUAPE in Germany. On December 31st 2018, data from 1571 patients were included.

Methods: A custom-made electronic health record software is used at the participating centres. Pseudonymized data are transferred for central analysis. Parameters were selected based on current guidelines. Descriptive analyses and linear regression models were implemented with SAS 9.4.

Results: We identified 1288 patients on exclusive treatment with hydrocortisone three times daily (604 boys; median age 7.2 years; 817 salt-wasting phenotype, 471 simple-virilizing phenotype). The mean [lower-upper quartiles] daily HC dose [mg/m² body surface area] was 19.4 [18.9-19.8] for patients <3 months (n=329), 15.0 [14.6-15.3] age ≥3-12 months (n=463), 14.0 [13.7-14.3] age 1-5.9 years (n=745), 14.2 [14.0-14.5] age 6 years-puberty entry (n=669), and 14.9 [14.6-15.2] during puberty-18 years (n=801). Fludrocortisone was administered in 74.1% of patients (median daily dosage 88.8 µg).

Conclusion: Our analyses demonstrated still a high proportion of children with HC doses higher than recommended. This evaluation provides comprehensive information on nationwide hydrocortisone substitution dosages in children with CAH underlining the benefit of systematic data within a registry to assess daily practice.