A Case of Pseudovaginal Perineoscrotal Hypospadias with 5alpha-reductase Deficiency.
- Author:
Hee Su PARK
1
;
Hee Cheol YOON
;
Yong Ho JEONG
;
Joon RHO
;
Chul Sung KIM
;
Dae Soo JANG
Author Information
1. Department of Urology, College of Medicine, Chosun University, Kwangju, Korea.
- Publication Type:Original Article
- Keywords:
Pseudohermaphroditism;
Testosterone;
5alpha-reductase;
Intersex
- MeSH:
46, XY Disorders of Sex Development;
Androgen-Insensitivity Syndrome;
Dihydrotestosterone;
Disorders of Sex Development;
Female;
Humans;
Hypospadias*;
Male;
Steroids;
Testosterone
- From:Korean Journal of Urology
1999;40(3):398-401
- CountryRepublic of Korea
- Language:Korean
-
Abstract:
Male pseudohermaphroditism can be caused by absent m llerian regression, inadequate synthesis of testosterone, inadequate synthesis of dihydrotestosterone, or androgen receptor deficiency. Defects in either the production or the action of androgenic steroids have been demonstrated to cause pseudovaginal perineoscrotal hypospadias, a syndrome of male pseudohermaphroditism. This is mostly caused by a deficiency of 5alpha-reductase, which controls the conversion of testosterone to 5alpha-dihydrotestosterone. We report a case of male pseudohermaphroditism due to 5alpha-reductase deficiency who was born with ambiguous genitalia and was reared as female.
