Response to an llβ-Hydroxylase Inhibitor (SU-4885) in Males with Adrenal Hyperplasia and in Their Parents

SU-4885 was administered to 5 males with congenital adrenal hyperplasia and virilism. The moderate increase in urinary 17-OH-CS was due entirely to an elevation of tetrahydro S; at the same time there was a marked elevation in the excretion of pregnanetriol and 17-OH-pregnanolone. This is in contrast with the findings in six normal young adults who showed a minimal increase in urinary pregnanetriol and 17-OH-pregnanolone and a threefold to sixford increase in total 17-OH-CS. The average ratio, pregnanetriol + 17-OH-pregnanolone, 17-OH-CS in the 5 patients was 17.3 prior to SU-4885 and 14.0 on the day after SU-4885, whereas in the normal subjects the corresponding values were 0.21 and 0.13. This ratio was thought to be a sensitive index of 21-hydroxylaction, and it was expected that mild deficiency of this enzyme might be detected in presumably heterozygous parents of patients with congenital adrenal hyperplasia. However, no difference in the response to SU-4885 was found in parents when compared with normal subjects, expect for 1 parent who munifested an intermediate response.