Isolation of urinary C-20 alpha- and C-20 beta-hydroxy-C21-steroid metabolites in cases of congenital adrenal hyperplasia, postpubertal virilizing syndrome and polycystic ovary syndrome.
The proportion of 20 alpha/20 beta-epimers of the urinary C21-steroid metabolites was estimated in normal volunteers and in patients with congenital adrenal hyperplasia (CAH), postpubertal virilizing syndrome (PVS) and polycystic ovary syndrome (POS). In the normal individuals 20 alpha- and 20 beta-epimers of 5 beta-pregnane-3 alpha, 17 alpha, 20-triol (pregnanetriol) and 5-pregnene-3 beta, 17 alpha, 20-triol (5-pregnenetriol) were measured. In the other case 20 alpha- and 20 beta-epimers of the characteristic, representative metabolites were also investigated. In 26 of 27 normal persons and in all the patients with normotensive CAH, PVS and POS the 20 beta-epimer comprised 0-10% of the total pregnanetriol. The 20 beta-epimer of 5-pregnenetriol was found in only one normal case (6% of the total). The percentage of 20 beta-epimers of 3 alpha, 17 alpha, 20-trihydroxy-5 beta-pregnan-11-one, 5 beta-pregnane-3 alpha, 11 beta, 17 alpha, 20-tetrol (in the normotensive CAH, PVS and POS) and 5 alpha- or 5 beta-pregnane-3 alpha, 17 alpha, 20, 21-tetrol (in the hypertensive CAH) varied from nil to 76%. The effect of functional groups and the stereochemistry of the molecule on the direction of C-20-keto group reduction is discussed; the existence of additional factors determining this reduction in certain pathological conditions is suggested.
D000293:Adolescent; D000308:Adrenocortical Hyperfunction / Q000652:urine*; D000324:Adrenocorticotropic Hormone; D000328:Adult; D003907:Dexamethasone; D005260:Female; D006801:Humans; D006914:Hydroxysteroids / Q000652:urine*; D007664:Ketosteroids / Q000652:urine; D008297:Male; D011085:Polycystic Ovary Syndrome / Q000652:urine*; D011279:Pregnanetriol / Q000031:analogs & derivatives / Q000652:urine; D013237:Stereoisomerism; D014770:Virilism / Q000652:urine*
D006914:Hydroxysteroids; D007664:Ketosteroids; D011279:Pregnanetriol; D003907:Dexamethasone; D000324:Adrenocorticotropic Hormone