Gender related differences in glucocorticoid therapy and growth outcomes among pubertal children with 21-hydroxylase deficiency congenital adrenal hyperplasia (CAH)

摘要

Twenty-one-hydroxylase deficient congenital adrenal hyperplasia (CAH) causes glucocorticoid and mineralocorticoid deficiency, hyperandrogenism and short stature. Management of the pubertal CAH patient is particularly challenging. The purpose of this retrospective chart review study was to determine if pubertal males and females with simple virilizing CAH (SVCAH) required different glucocorticoid dosages at progressive Tanner stages. The relationship between hydrocortisone dose and height was also assessed. Twenty females and seventeen males with SVCAH were identified and followed throughout all stages of pubertal development. Males received an average hydrocortisone dose of 16.4 ±4.8 mg/m 2/ day and for females, 13.7 ±4.6 mg/m 2/day. The glucocorticoid dosage in males was significantly higher than in females at Tanner stages 3 through 5. Higher doses were associated with a shorter (9.6 cm) achieved than anticipated adult height.