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 Bachega, Antonio Balsamo, Sabine E Hannema, Niels Birkebæk, Ana Vieites, Ajay Thankamony, Martin 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

Research output: Contribution to journalArticleAcademicpeer-review


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
Issue number2
Early online date1 Mar 2022
Publication statusPublished - Aug 2022

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