Evidence for Reduced 3β-ol-Hydroxysteroid Dehydrogenase Activity in Some Hirsute Women Thought to Have Poly cystic Ovary Syndrome*

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Abstract
Congenital adrenal hyperplasia due to 3β-ol-hydroxysteroid dehydrogenase (3β-ol) deficiency is usually lethal. A partial deficiency in 3β-ol has been suggested in some women presenting with androgen excess. In this study, 24 women were investigated who had hirsutism and oligomenorrhea and high serum Δ5 androgens compared to Δ4 androgens. Of these women, 9 had significantly elevated 17-hydroxypregnenolone (17 Preg) to 17-hydroxyprogesterone (17 Prog) ratios when compared to controls under basal conditions. On further testing of 9 women with ACTH, 4 had significantly elevated 17 Preg to 17 Prog ratios. Eight women had elevated ratios of dehydroepiandrosterone sulfate to androstenedione, 4 had elevations of androstenediol (Adiol) to testosterone (T), and 4 had abnormal 17 Preg to cortisol ratios. Only 3 women out of the original 24 selected for study had elevated ratios for all 4 different steroid pairs measured. 17 Prog was normal in these women with 3β-ol deficiency in contradistinction to the high levels normally observed in women with congenital adrenal hyperplasia due to 21- or 11-hydroxylase deficiency. It is suggested that the cause of androgen excess in these women is high circulating levels of Adiol and, in part, the slightly elevated unbound T levels in these women. In conclusion, a subtle, incomplete form of 3β-ol deficiency may exist in adult women and is manifest by high Δ5 androgens such as dehydroepiandrosterone sulfate and Adiol and normal Δ4 androgens such as androstenedione and T.

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