In Vivoandin VitroStudies of Androgen Metabolism in a Patient with Pure Gonadal Dysgenesis and Leydig Cell Hyperplasia

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Abstract
Androgen metabolism was studied in a 33-yr-old virilized woman with pure gonadal dysgenesis and Leydig cell hyperplasia of both gonads. The plasma levels and production rates of testosterone and androstenedione and the plasma LH levels were increased. From 20 to 25% of plasma testosterone was derived from plasma androstenedione and an additional 30% was from gonadal secretion. Plasma testosterone and LH levels were not suppressed by short-term administration of androgen or estrogen, and testosterone did not increase during HCG administration. Plasma testosterone levels returned to normal without treatment even though the plasma LH remained elevated. In vitro incubation of gonadal tissue with acetate-1-14C produced a “profile” of radioactive steroids which differed from that produced by normal ovarian stroma or testis.

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