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.
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