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

Uta Neumann*, Annelieke van der Linde, Ruth E. Krone, Nils P. Krone, Ayla Güven, Tülay Güran, Heba Elsedfy, Sukran Poyrazoglu, Feyza Darendeliler, Tania A.S.S. Bachega, Antonio Balsamo, Sabine E. Hannema, Niels Birkebaek, Ana Vieites, Ajay Thankamony, Martine Cools, Tatjana Milenkovic, Walter Bonfig, Eduardo Correa Costa, Navoda AtapattuLiat de Vries, Guilherme Guaragna-Filho, Marta Korbonits, Klaus Mohnike, Jillian Bryce, S. Faisal Ahmed, Bernard Voet, Oliver Blankenstein, Hedi L. Claahsen-Van der Grinten

*Corresponding author for this work

Research output: Contribution to journalArticleAcademicpeer-review

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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.

Original languageEnglish
Pages (from-to)587-596
Number of pages10
JournalEuropean Journal of Endocrinology
Volume186
Issue number5
Early online date11 Apr 2022
DOIs
Publication statusPublished - May 2022

Bibliographical note

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.

Publisher Copyright:
© 2022 The authors Published by Bioscientifica Ltd.

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