METABOLISM OF RADIOLABELED CORTICOSTERONE IN AN ADULT WITH THE 17-ALPHA-HYDROXYLASE DEFICIENCY SYNDROME

[4-14C]Corticosterone was administered to a woman with the 17α-hydroxylase deficiency syndrome and urine was collected for 72 h. Sixty-three percent of the radioactivity was eliminated on the first day, 10.3% on the second, and 3.8% on the third, making a total recovery of 77%. On the first day, 85% of the recovered radioactivity was in the glucuronide conjugates of corticosterone, 10.6% was in the sulfate form of this steroid, and 3.9% was in the free forms of the steroid. On the following 2 days, the proportion of labeled glucuronides and free steroids decreased and that of labeled sulfates increased. On the first day of collection, the major radiolabeled metabolites were 21-hydroxylated steroids (e.g. allo-tetrahydrocorticosterone and 5α- and β-pregnane-3α,11β,20α,21-tetrol), but by the third day, at least 75% of the excreted activity was associated with 21- deoxysteroids, such as 3α,20α-dihydroxy-5α (and β)-pregnan-llone and 5α-and β-pregnane-3α,11β,20α-triol. Bacterial metabolism in the intestinal tract is responsible for the dehydroxylation. 6α-Hydroxytetrahydrocorticosterone was tentatively identified among several new metabolites of corticosterone. © 1979 by The Endocrine Society.