MALE PSEUDOHERMAPHRODITISM SECONDARY TO 17-BETA-HYDROXYSTEROID DEHYDROGENASE-DEFICIENCY - GENDER ROLE CHANGE WITH PUBERTY

被引:60
作者
IMPERATOMCGINLEY, J
PETERSON, RE
STOLLER, R
GOODWIN, WE
机构
[1] UNIV CALIF LOS ANGELES, DEPT PSYCHIAT, DIV NEUROPSYCHIAT, LOS ANGELES, CA 90024 USA
[2] UNIV CALIF LOS ANGELES, DEPT SURG, DIV UROL, LOS ANGELES, CA 90024 USA
关键词
D O I
10.1210/jcem-49-3-391
中图分类号
R5 [内科学];
学科分类号
1002 ; 100201 ;
摘要
A 31-yr-old male pseudohermaphrodite is reported with 17β-hydroxysteroid dehydrogenase deficiency. Laboratory data revealed a plasma testosterone of 228 ng/100 ml, a plasma androstenedione of 620 ng/100 ml, and an abnormal androstenedione to testosterone 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. © 1979 by The Endocrine Society.
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收藏
页码:391 / 395
页数:5
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