Steroid excretion in urine during suppression and stimulation of adrenals in the 17 alpha-hydroxylase deficiency syndrome.

Twenty-three steroids were determined by capillary column gas chromatography in urine specimens from a patient with the 17 alpha-hydroxylase deficiency syndrome during dexamethasone suppression and ACTH stimulation tests. These steroids included pregnanediols and pregnenediols, 21-deoxy metabolites of corticosterone (e.g. 3 alpha, 20 alpha-dihydroxy-5 alpha-pregnan-11-one), and 21-hydroxy metabolites [e.g. allo-tetrahydrocorticosterone (3 alpha, 11 beta, 21-trihydroxy-5 alpha-pregnan-20-one)]. During dexamethasone suppression or ACTH stimulation, the excretion of all steroids decreased and increased, respectively, indicating the pituitary-dependent adrenal origin of each compound. The changes in excretion of 21-deoxy steroids responded slowly (compared with the 21-hydroxylated metabolites) to these maneuvers, thereby providing further evidence for extensive enterohepatic circulation of corticosterone metabolites. 21-Dehydroxylated corticosterone metabolites accounted for approximately 40% the total steroids excreted during the control period, and this percentage probably approximates the proportion of corticosterone excreted in bile.