Male Pseudohermaphroditism Secondary to 17β-Hydroxysteroid Dehydrogenase Deficiency: Gender Role Change with Puberty*

Publisher Website
Google Scholar
Abstract
A 31 yr old male pseudohermaphrodite is reported with 17.beta.-hydroxysteroid dehydrogenase deficiency. Laboratory data revealed a plasma testosterone [T] of 228 ng/100 ml, a plasma androstenedione [DIONE] of 620 ng/100 ml, and an abnormal DIONE to T ratio. Plasma estradiol was 4.6 ng/100 ml and plasma estrone was 22 ng/100 ml. This subject was born in a hospital, incontrovertibly declared to be a female and unambiguously raised as a girl by his parents for the first 14 yr of his life. At age 14 yr, he was able to change to a male gender role with ease. As an adult, he is a well adjusted, happily married man with a successful professional career. Surgical correction of bilateral cryptorchidism and hypospadias was carried out at age 14 yr. At age 30 yr, he developed a teratocarcinoma-seminoma of the right testis with retroperitoneal node metastases. After orchiectomy and retroperitoneal node dissection, he was placed on chemotherapy and is presently free of metastases.

This publication has 0 references indexed in Scilit: