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.
19 - 21 Sep 2019
European Society for Paediatric Endocrinology