Menarche and subsequent ovarian function in girls with congenital adrenal hyperplasia (CAH)

Abstract

Chronological age, body weight and plasma steroid concentrations at menarche, and subsequent ovarian function in relation to plasma/saliva steroid concentrations and glucocorticoid therapy were reviewed in 6 girls with 21-hydroxylase deficiency (3 salt-losers). Mean age at menarche was 13.6 yr (12.6-14.5); body weight varied from 45 to 66 kg. Menarche did not occur with plasma testosterone (T) levels of 5-6 nmol/L. Normal female plasma T levels occurred on changing from hydrocortisone to single dose dexamethasone (dex) given as 0.25 to 0.75 mg/day. The potency of dex relative to cortisol was 80:1 based on adrenal suppression effect. Regular menses occurred with plasma T levels in the normal female range. Ovulatory cycles were documented using menstrual profiles of saliva progesterone (P) performed at a post-menarchal age of 3.0-3.4 yr. Some showed the characteristic rise in luteal levels of saliva 17P indicating previous ovulation; one 16 yr old girl became pregnant. Anovulatory cycles also occurred in well-controlled girls. Preliminary data on controls showed absence of ovulatory cycles for at least 2 yr post-menarche. Conclusions: in CAH, 1) Menarche is not usually delayed. 2) Ovulatory cycles may be delayed but further control data is needed. 3) Normal plasma T levels are required for regular menses. 4) This can be achieved using single dose dex. therapy.