Abstract
Aminoglutethimide, a selective inhibitor of cholesterol desmolase, produces in rats the fetal adrenal and testicular defects and hypospadias in male fetuses characteristic of the human disease postulated to be due to a genetic defect in this enzyme, but unlike the disease, virilizes female fetuses. The effects of testosterone (1 mg/kg) and corticosterone (10 mg/kg) administered to pregnant rats treated with aminoglutethimide (100 mg/kg) from days 13 to 20 were studied. In the mothers, testosterone did not significantly affect the marked adrenal enlargement or increase of adrenal size and cholesterol content, but corticosterone reduced the increased in adrenal size and cholesterol content produced by this drug. Testosterone prevented the aminoglutethimide-induced production of hypospadias and the increase of testicular cholesterol content. Corticosterone reduced fetal adrenal enlargement, increase in fetal adrenal cholesterol content, and the increase of the anourethral distance in female fetuses produced by aminoglutethimide. These experiments provide evidence that the production of hypospadias by this drug is due to inhibition of fetal testicular cholesterol desmolase, and that the production of the paradoxic virilization of females by aminoglutethimide is due to androgens of aerenal origin.
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Goldman, A. Experimental Congenital Lipoid Adrenal Hyperplasia: Prevention of Defects Produced by Aminoglutethimide. Pediatr Res 4, 455 (1970). https://doi.org/10.1203/00006450-197009000-00084
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DOI: https://doi.org/10.1203/00006450-197009000-00084