Endocrine Abstracts (2017) 49 EP206 | DOI: 10.1530/endoabs.49.EP206

Congenital adrenal hyperplasia: impact of therapy on growth and sexual maturation - a 5-year retrospective study of a Tertiary Pediatric Endocrinology Center

Camelia Procopiuc, Madalina Vintila, Iuliana Gherlan, Amel Cirla, Andreea Brehar, Andra Caragheorgheopol, Suzana Vladoiu Oana Popa & Cristina Dumitrescu

CI Parhon National Institute of Endocrinology, Bucharest, Romania.

Objective: To evaluate the comparative effects of different glucocorticoid treatments on growth and sexual maturation in patients with congenital adrenal hyperplasia.

Patients and methods: We conducted a retrospective observational cohort study in 78 patients (60 girls, 18 boys) diagnosed with congenital adrenal hyperplasia (CAH), followed-up for a period of 5 years. The majority had 21-hydroxylase deficiency (75 patients), 2 had 11-β hydroxylase deficiency and 1 patient had 3-β hydroxysteroid dehydrogenase deficiency. Patients received either Prednisone (PDN), Hydrocortisone (HC) or Hydrocortisone plus Dexamethasone (DEX). Data on growth, bone maturation, puberty onset, predicted adult height, and parental height were collected. Height gain was calculated as the difference between parentally determined target height and final or predicted height at the last evaluation.

Results: Height gain with therapy was 0.42 S.D. over the initial estimated height deficit. Height gain correlated with the clinical form of CAH, with better outcome in non-classical vs classic forms (P<0.01). There were no significantly differences between groups in regard to type of therapy (P=0.164). There was no difference in change of body mass index regarding the type of treatment (P=0.99). Girls over 12 years of age treated with HC had higher rates of spontaneous menarche versus PDN treated girls (P=0.006). Twelve children developed precocious puberty, which correlated with older age at treatment initiation (P=0.048).

Conclusions: The type of therapy did not influenced height gain. HC facilitated spontaneous menarche onset in normal age range. Late diagnosis and therapy led to higher rates of precocious puberty in children with CAH.