Patients with Classic Congenital Adrenal Hyperplasia due to 21-Hydroxylase Deficiency Can Achieve Their Target Height: The Leipzig Experience

Abstract

Background: Despite treatment, the mean final height (FH) of patients with classic congenital adrenal hyperplasia (CAH) is below the mean height of a normal population. Aims:To show that CAH patients can achieve their target height (TH), 39 adult subjects, whose therapy had started in infancy, were studied in a retrospective analysis. All height SDS were corrected so that they related to TH SDS. Patients: Group 1: patients born before 1975 (n = 13) had received prednisolone, at doses equivalent to hydrocortisone 39.4 ± 15.6 mg/m² BSA daily, together with DOCA in the first 2 years of life. Group 2: patients born from 1975 to 1986 (n = 26) received at this age lower hydrocortisone doses (16.4 ± 6.9 mg/m² BSA daily, divided 8 hourly; p < 0.001) combined with fludrocortisone, had outpatient visits every 3 months and bone age (BA) estimation every 6 months. Results: Patients of group 1 (FH SDS –1.2 ± 1.0) had a poor outcome, whereas patients of group 2 (FH SDS 0.1 ± 0.9; p = 0.01) achieved their TH. Conclusion: Combined corticoid administration adjusted quarterly to keep height, BMI, blood pressure and BA within normal limits resulted in FH close to TH in patients with classic CAH.