Abstract
In fourteen cases of C21-hydroxylation deficient cases, ratios of etiocholanolone to androsterone were lower than normal, and 11β-hydroxyandrosterone was predominant of 11-oxygenated-17-KS. A ratio of pregnanetriols to 17α-hydroxypregnanolone was more than unity in almost all cases. A ratio of 5β-pregnane-3α, 17α, 20α-triol to its 5α-isomer did not seem parallel to a ratio of etiocholanolone to androsterone in individual cases. A urinary output of THE was much higher than that of THF. Estrogens were excreted in higher amount and in decreased amount after oral administration of corticoid. A case of hypertensive form, with chief complaints of progressive virilization and hypertension showed a high urinary excretion of tetrahydro-11-deoxycortisol (41mg/day) and 11-deoxy-17-ketosteroids with small amounts of 11-oxy-17-ketosteroids. Allotetrahydro-11-deoxycortisol was detected and quantitatively determined (250μg/day).