TY - JOUR
T1 - Treatment of congenital adrenal hyperplasia in children aged 0-3 years
T2 - A retrospective multicenter analysis of salt supplementation, glucocorticoid and mineralocorticoid medication, growth and blood pressure
AU - Neumann, Uta
AU - van der Linde, Annelieke
AU - Krone, Ruth E
AU - Krone, Nils P
AU - Güven, Ayla
AU - Güran, Tülay
AU - Elsedfy, Heba
AU - Poyrazoglu, Sukran
AU - Darendeliler, Feyza
AU - Bachega, Tania A
AU - Balsamo, Antonio
AU - Hannema, Sabine E
AU - Birkebæk, Niels
AU - Vieites, Ana
AU - Thankamony, Ajay
AU - Cools, Martin
AU - Milenkovic, Tatjana
AU - Bonfig, Walter
AU - Costa, Eduardo Correa
AU - Atapattu, Navoda
AU - de Vries, Liat
AU - Guaragna-Filho, Guilherme
AU - Korbonits, Marta
AU - Mohnike, Klaus
AU - Bryce, Jillian
AU - Ahmed, S Faisal
AU - Voet, Bernard
AU - Blankenstein, Oliver
AU - Claahsen-van der Grinten, Hedi L
N1 - Funding Information:
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
Funding Information:
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. The authors would like to acknowledge the support of the following reference centres that participate in the European Reference Network for Rare Endocrine Conditions (Endo-ERN) (list reference centres alphabetically by country – [list available at https://endo-ern.eu/about/reference-centres/, please consult with I-DSD/I-CAH for clarification]. Clinical trial registration number: EA2/128/14.
Funding Information:
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.
PY - 2022/8
Y1 - 2022/8
N2 - 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.
AB - 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.
UR - https://www.scopus.com/pages/publications/85128487416
U2 - 10.1530/EJE-21-1085
DO - 10.1530/EJE-21-1085
M3 - Article
SN - 0804-4643
VL - 187
SP - 587
EP - 596
JO - European Journal of Endocrinology
JF - European Journal of Endocrinology
IS - 2
ER -
SDG 3 Good Health and Well-being