Dexamethasone Treatment of Virilizing Congenital Adrenal Hyperplasia: The Ability to Achieve Normal Growth

Abstract

Objective.: To assess whether treatment of virilizing congenital adrenal hyperplasia (CAH) with long-acting glucocorticoids is associated with favorable growth outcomes. Method.: We examined the long-term growth of 17 boys and 9 girls with CAH treated with dexamethasone (.27 ± .01 mg/m2/day). Results.: For individuals with comparable bone age (BA) and chronological age (CA) at the onset of dexamethasone therapy, males were 2.8 ± .8 years (mean ± standard error of the mean;n = 13) and females were 2.4 ± 1.0 years (n = 6). Males were treated for 7.3 ± 1.1 years (ΔCA) over which time the change in BA (ΔBA) was 7.0 ± 1.3 years, and the change in height age (ΔHA) was 6.9 ± 1.1 years. Females were treated for 6.8 ± 1.3 years, over which time the ΔBA was 6.5 ± 1.0 years, and the ΔHA was 6.3 ± .8 years. During treatment 17 ketosteroid excretion rates were normal for age and 17-hydroxyprogesterone values were 69.6 ± 18 ng/dL. Testicular enlargement was first detected at 10.7 ± .8 years and breast tissue at 9.9 ± 1.2 years. Three boys and 1 girl had final heights of 171.8 ± 6 cm and 161 cm, respectively, compared with midparental heights of 176.1 ± 4.1 cm and 160 cm. Predicted adult heights for 6 other boys and 5 girls were 176.8 ± 2.0 cm and 161.4 ± 2.8 cm, respectively, compared with midparental heights of 174.6 ± 1.4 cm and 158.2 ± 2.0 cm. Statural outcomes were less favorable for 7 children started on dexamethasone when BAs were considerably advanced, although height predictions increased during therapy. Conclusions.: These observations show that children treated with dexamethasone for CAH can achieve normal growth with the convenience of once-a-day dosing in most cases. congenital adrenal hyperplasia, dexamethasone, growth.