Isolation, Identification and Measurement of 3α,17α-Dihydroxypregnane-11,20-dione in Congenital Adrenal Hyperplasia1

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Abstract
11-Keto-17-hydroxypregnanolone was isolated from the urine of patients with congenital virilizing adrenal hyperplasia. The identification was based on its mobility on paper chromatograms and the pink spot obtained when treated with the Zim-mermann reagent; the product of oxidation with sodium bismuthate had an Rf value and a color reaction with the Zimmermann reagent similar to that of 11-ketoetiocholanolone; its ultraviolet spectrum as a sulfuric acid chromogen, and its infrared spectrum. A method for the measurement of this steroid based on its ultravioletabsorption as a sulfuric acid chromogen is described. In the simple virilizing form, patients of 5 to 39 years of age excreted 1.1-4.9 mg/24 hr of 11-keto-17-hydroxypregnanolone, while 2 infants under 1 year excreted 0.8 and 0.9 mg. In the salt-losing form, 5 patients under 1 year of age excreted 0.3-0.8 mg/24 hr. In subjects with the hypertensive form this steroid could not be detected.