Treatment of congenital adrenal hyperplasia in children aged 0–3 years: a retrospective multicenter analysis of salt supplementation, glucocorticoid and mineralocorticoid medication, growth and blood pressure

Neumann, U. et al. (2022) Treatment of congenital adrenal hyperplasia in children aged 0–3 years: a retrospective multicenter analysis of salt supplementation, glucocorticoid and mineralocorticoid medication, growth and blood pressure. European Journal of Endocrinology, 186(5), pp. 587-596. (doi: 10.1530/eje-21-1085) (PMID:35290211) (PMCID:PMC9066592)

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Abstract

Objectives: International guidelines recommend additional salt supplementation during infancy in classic congenital adrenal hyperplasia (CAH) due to 21-hydroxylase deficiency. The influence of corticoid medication and growth has not been assessed. Aim: To investigate the current use of salt supplementation, fludrocortisone (FC) and hydrocortisone (HC) dosage as well as weight, height, BMI and blood pressure (BP) in CAH children aged 0–3 years. Methods: Retrospective multicentre analysis using data from the I-CAH registry. Salt-treated (ST) and non-salt-treated (NST) children were compared regarding FC and HC dosage, weight, height and BP at 0, 3, 6, 9, 12, 18, 24, 30, and 36 months. Results: We analysed 2483 visits of 331 patients born after year 2000 in 13 countries (male, n  = 145) with 203 ST patients (61%). NST children had significantly higher FC dosages at 1.5–4.5 months and higher HC dosages until 1.5 months of age. No differences in weight, length and BP between subgroups were observed. Children of the whole cohort showed increased BMI-SDS during the study period and about half of the reported BP readings were >P95. Conclusion: In children treated with additional salt supplementation, FC and HC dosages are lower during the first months of life but without differences in weight, length and BP until 3 years of age compared to NST children. All children showed an increase in BMI-SDS and a high rate of BP readings >P95 until 3 years, indicating the start of weight gain and negative effects on blood pressure already in very early life.

Item Type:Articles
Additional Information:Funding: This project has received support from the I-CAH Registry project that has received unrestricted education grants from Diurnal Ltd and Neurocrine Biosciences. The initial development of the Registry was supported by the Medical Research Council (G1100236), the Seventh European Union Framework Program (201444) and the European Society for Paediatric Endocrinology Research Unit.
Keywords:Endocrinology, General Medicine, Endocrinology, Diabetes and Metabolism
Status:Published
Refereed:Yes
Glasgow Author(s) Enlighten ID:Ahmed, Professor Syed Faisal and Bryce, Dr Jillian
Authors: Neumann, U., van der Linde, A., Krone, R. E., Krone, N. P., Güven, A., Güran, T., Elsedfy, H., Poyrazoglu, S., Darendeliler, F., Bachega, T. A., Balsamo, A., Hannema, S. E., Birkebæk, N., Vieites, A., Thankamony, A., Cools, M., Milenkovic, T., Bonfig, W., Costa, E. C., Atapattu, N., de Vries, L., Guaragna-Filho, G., Korbonits, M., Mohnike, K., Bryce, J., Ahmed, S. F., Voet, B., Blankenstein, O., and Claahsen-van der Grinten, H. L.
College/School:College of Medical Veterinary and Life Sciences > School of Medicine, Dentistry & Nursing
Journal Name:European Journal of Endocrinology
Publisher:Bioscientifica
ISSN:0804-4643
ISSN (Online):1479-683X
Published Online:11 April 2022
Copyright Holders:Copyright © 2022 The Authors
First Published:First published in European Journal of Endocrinology 186(5): 587-596
Publisher Policy:Reproduced under a Creative Commons License

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Project CodeAward NoProject NamePrincipal InvestigatorFunder's NameFunder RefLead Dept
190419The International DSD Network (I-DSD)Syed Faisal AhmedMedical Research Council (MRC)G1100236/1Med - Child Health
160901European Disorders of Sex DevelopmentRichard SinnottEuropean Commission (EC)201444US - E-Science