CONCURRENT 3β-HYDROXYSTEROID DEHYDROGENASE DEFICIENCY IN ADRENAL AND SCLEROCYSTIC OVARY

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Abstract
In vivo and in vitro studies were performed in a virilized patient with enlarged sclerocystic ovaries, in whom urinary corticoid excretion was not suppressed by dexamethasone. Both ovarian and adrenal tissues were incubated with 5-pregnenlone-4-C14 and the metabolites isolated and definitively identified. Both tissues showed a relative deficiency of 3[beta]-hydroxysteroid dehydrogenase. The ovarian aromatizing mechanism was intact. 5-Andro-stene-3[beta],17[beta]-diol was the major adrenal biosynthetic product, and its metabolites were identified in the urine. The abnormality of the hypo-thalamic-pituitary-adrenal regulatory system resembled that seen in Cushing''s syndrome, but the clinical manifestations were altered by the steroid enzyme abnormality.