Korean J Obstet Gynecol.  1999 Mar;42(3):632-636.

A Case of Complete Androgen Insensitivity Syndrome with Bilateral Inguinal Gonads

Abstract

Androgen insensitivity syndrome is a genetic syndrome characterized by complete or partial resistance of end organ to the peripheral effect of androgen. Patient have a male karyotype(46,XY) and bilateral testes. Appearance of external genitalia depend on the degree of androgen insensitivity. In the complete form, external genitalia is normal female but in the incomplete form, external genitalia figure varies hom that of a virilized female to that of an undervirilized male with a short penis and hypospadia. The gonads are able to be located along the descending course of the testis during development. The usual presenting symptom in complete type is primary amenorrhea or inguinal mass, but in incomplete type, the patient mainly present with ambiguous genitalia. Recently we experienced one case of this syndmme and removed the bilateral gonads, so we report it with brief review of literatures.

Keyword

Androgen Insensitivity Syndrome

MeSH Terms

Amenorrhea
Androgen-Insensitivity Syndrome*
Disorders of Sex Development
Female
Genitalia
Gonads*
Humans
Hypospadias
Male
Penis
Testis
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