Abstract
A 46-yr-old phenotypic female with XY genotype presented an unusual form of nonfamilial male pseudohermaphroditism with gynecomastia. Seemingly a normal girl until puberty, the patient developed breasts but became virilized as well at age 13 and failed to menstruate. No uterus was found above the short and blindending vagina. Serum testosterone (T) was subnormal for a male while urinary 17-ketosteroids (17KS) were elevated. The disorder was clarified when increased serum LH concentrations and a 10-fold elevation in serum androstenedione (A) were found. Serum A and estrone (E1) concentrations doubled following HCG stimulation while little change in serum T was observed. Exogenous T suppressed scrum LH by 50% and lowered serum A, E1 and estradiol (E2) to normal or subnormal levels. Dexamethasone suppression and ACTH stimulation studies revealed normal adrenal function. Blood production, metabolic clearance, and A to T conversion rates indicated that 91% of circulating T was derived from A which was produced...
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