17 α-HYDROXYPROGESTERONE AND 21-DESOXYHYDROCORTISONE; THEIR METABOLISM AND POSSIBLE ROLE IN CONGENITAL ADRENAL VIRILISM 1

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Abstract
An attempt to reproduce the urinary steroidal pattern characteristic of congenital adrenal virilism by administration of 17[alpha]-hydroxy-progesterone and 21-desoxyhydrocortisone was made. Urinary metabolites of these steroids were identified as: etiocholanolone; androsterone; 11-keto-etiocholanolone; 11-hydroxy-etiocholanolone; 11[beta]-hydroxy-androsterone; pregnanolone; 17-hydroxy-pregnanolone and 11-keto-pregnanolone. The presence of pregnanediol, pregnanetriol and pregnanetriol-11-one were indicated by means of chromo-genic reactions. The following hypothesis is presented: Con-genital adrenal hyperplasia is accompanied by enzymatic defects in the hydroxylation mechanisms, which results in the piling up of partially hydroxylated hydrocortisone precursors. These escape into the circulation and are metabolized along well-known pathways resulting in the production of androgens.