Abstract
Varying daily doses of a steroid inhibitor of fl-hydroxysteroid dehydrogenase, 2a-cyano-4,4,17atrimethylandrost-5-en-17a-ol-3-one, were administered to pregnant rats on the fifteenth to twentieth days of gestation to determine whether this inhibitor could produce the biologic manifestations of the human disease associated with deficient activity of this enzyme. Infants with this disorder have hyperplastic adrenal cortices deficient in activity of 3fi-HSD, incomplete penile development in males resulting in hypospadias with shortening of the anogenital distance, and clitoral hypertrophy without urethral orificial displacement in females. The external sex (anogenital distance) and gonadal sex (presence of ovaries or testes) of experimental fetuses from females receiving the inhibitor were compared to suitable controls. The experimental fetuses had adrenal cortical hyperplasia (5.9 -2_ 1.32 versus 2.2 -+ 26 rag.), deficient histochemical activity of 3fl-HSD in adrenals, testes, and placentas, and no change in histochemical activity of 3aor 17B-HSD. The anogenital distance in males was reduced from the normal of 2.88 -+ 0.12 to 1.26 ± 0.08 ram. in proportion to dose. The females had clitoral hypertrophy but no change in anogenital distance from the normal of 1 mm. This study thus demonstrates an experimental model for the production of the congenital manifestations of this inborn error of metabolism.
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