Korean J Urol.  1999 Mar;40(3):398-401.

A Case of Pseudovaginal Perineoscrotal Hypospadias with 5alpha-reductase Deficiency

Affiliations
  • 1Department of Urology, College of Medicine, Chosun University, Kwangju, Korea.

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.

Keyword

Pseudohermaphroditism; Testosterone; 5alpha-reductase; Intersex

MeSH Terms

46, XY Disorders of Sex Development
Androgen-Insensitivity Syndrome
Dihydrotestosterone
Disorders of Sex Development
Female
Humans
Hypospadias*
Male
Steroids
Testosterone
Dihydrotestosterone
Steroids
Testosterone
Full Text Links
  • KJU
Actions
Cited
CITED
export Copy
Close
Share
  • Twitter
  • Facebook
Similar articles
    DB Error: unknown error