ESPE Abstracts (2019) 92 P1-157

Influence of salt supplementation on drug therapy in children with congenital adrenal hyperplasia (CAH) due to 21-hydroxylase deficiency aged 0-3 years: Update on a retrospective multicentre analysis using the I-CAH registry

Uta Neumann1, Annelieke van der Linde2, Ruth Krone3, Ayla Guven4, Tülay Güran5, Heba Elsedfy6, Feyza Darendeliler7, Tania Bachega8, Antonio Balsamo9, Sabine Hannema10,11, Niels Birkebaek12, Ana Vieites13, Carlo Acerini14, Martine Cools15, Tatjana Milenkovic16, Walter Bonfig17, Eduardo Costa18, Navoda Atapattu19, Liat de Vries20, Guilherme Filho21, Marta Korbonits22, Klaus Mohnike23, Jillian Bryce24, Faisal Ahmed24, Bernard Voet25, Oliver Blankenstein1, Hedi Claahsen van der Grinten2


1Charité Universitätsmedizin Berlin, Berlin, Germany. 2Radboud University Medical Centre, Nijmegen, Netherlands. 3Birmingham Women's and Children's Hospital, Birmingham, United Kingdom. 4Saglik Bilimleri University, Istanbul, Turkey. 5Marmara University, Istanbul, Turkey. 6Ain Shams University, Cairo, Egypt. 7Istanbul University, Istanbul, Turkey. 8University of Sao Paulo, Sao Paulo, Brazil. 9S.Orsola-Malpighi University Hospital, Bologna, Italy. 10Leiden University Medical Centre, Leiden, Netherlands. 11Erasmus Medical Centre Rotterdam, Rotterdam, Netherlands. 12Aarhus University Hospital, Aarhus, Denmark. 13Hospital de Niños Ricardo Gutiérrez, Buenos Aires, Argentina. 14University of Cambridge and Addenbrooke's Hospital, Cambridge, United Kingdom. 15University Hospital Ghent, Ghent, Belgium. 16Institute for Mother and Child Healthcare of Serbia "Dr Vukan Čupic", Belgrade, Serbia. 17Technical University of Munich, Munich, Germany. 18Hospital de Clínicas de Porto Alegre, Porto Alegre, Brazil. 19Lady Ridgeway Hospital, Colombo, Sri Lanka. 20Tel Aviv University, Tel Aviv, Israel. 21Catholic University Porto Alegre, Porto Alegre, Brazil. 22Queen Mary University of London Barts, London, United Kingdom. 23Otto-von-Guericke University, Magdeburg, Germany. 24University of Glasgow, Glasgow, United Kingdom. 25Bernard Voet consulting, Berlin, Germany


Introduction: Classic congenital adrenal hyperplasia (CAH) caused by 21-hydroxylase deficiency results in impaired synthesis of gluco- and often also of mineralocorticoids. Early treatment with glucocorticoids and mineralocorticoids prevents life-threatening crises. In some centres, additional salt is prescribed in the first year. However, until now the use of salt is controversial and not proved to result in better outcome in studies.

Objectives: Primary aim: to evaluate the effect of additional salt supplementation on the fludrocortisone (FC) dosage in young children (0 – 3 yrs). Secondary aim: to evaluate effect of additional salt supplementation on hydrocortisone (HC) dosage, height and blood pressure

Methods: Retrospective analysis of patients born after 2000. Longitudinal data of the I-CAH (International congenital adrenal hyperplasia)-registry were extracted and divided in a salt treated (ST) (at least between two visits between 46 and 136 days of life) and a non-salt treated (NST) group. HC and FC dosage, weight, height and blood pressure were analysed at birth, 3, 6, 9, 12, 18, 24, 30, 36 month of age.

Results: A total of 2672 visits of 355 patients born after year 2000 and treated in 23 centres from 13 countries were analysed (actual assigned sex male – n=155 and female – n=200) with 114 patients (32.1%) in the ST group. Mean dose of FC was reduced in the ST group from birth until 18 months compared to the NST group. Dose of FC at 3 month of age: ST 362.98+/-220.91 µg/m² BSA vs. NST 386.46+/-186.72 µg/m² BSA. Mean dose of HC was not different between the groups. There were no clear differences in body weight and body length between the groups at the different time points. Although, in the ST group height and weight development were closer to the normal population and the systolic blood pressure was slightly lower.

Conclusion: Salt supplementation in newborns and infants suffering from classic CAH might reduce FC-dose and therefore reduce side effects on blood pressure in these children. Further longitudinal follow up is necessary to examine the effect on long-term parameters.

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