ESPE2019 Poster Category 1 Adrenals and HPA Axis (1) (12 abstracts)
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 Neumann 1 , Annelieke van der Linde 2 , Ruth Krone 3 , Ayla Guven 4 , Tülay Güran 5 , Heba Elsedfy 6 , Feyza Darendeliler 7 , Tania Bachega 8 , Antonio Balsamo 9 , Sabine Hannema 10,11 , Niels Birkebaek 12 , Ana Vieites 13 , Carlo Acerini 14 , Martine Cools 15 , Tatjana Milenkovic 16 , Walter Bonfig 17 , Eduardo Costa 18 , Navoda Atapattu 19 , Liat de Vries 20 , Guilherme Filho 21 , Marta Korbonits 22 , Klaus Mohnike 23 , Jillian Bryce 24 , Faisal Ahmed 24 , Bernard Voet 25 , Oliver Blankenstein 1 & Hedi Claahsen van der Grinten 2
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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