[Androgen insensitivity syndrome--testicular feminization--Morris syndrome. A surgical procedure in cases when a testis is found during herniotomy in a girl].
The androgen insensitivity syndrome is uncommon, but still the most frequent male pseudohermaphroditism. It is an X-linked recessive disorder characterized by androgen receptor defectiveness of various intensity. In the state of complete insensitivity, the male karyotype 46-XY develops a female gender with bilateral testes. Inguinal hernia is a common clinical presentation for a young child with complete androgen insensitivity. Thirty-five to forty percent of those with Morris syndrome come out with incarcerated hernias containing a testis, some even pass unnoticed at elective herniorraphy on apparently normal infant girls. It is recommended to perform orchiectomy when a testis is found during surgery. Subsequently the patient should be referred to major centres for further and thorough surveys.
Arstad
C
C
Hillerød Sygehus, organkirurgisk afdeling A.
Lendorf
A
A
D013734:Androgen-Insensitivity Syndrome / Q000175:diagnosis / Q000235:genetics / Q000601:surgery; D005260:Female; D006552:Hernia, Inguinal / Q000175:diagnosis; D006801:Humans; D007223:Infant; D008297:Male; D009919:Orchiectomy
Androgent insensitivitetssyndrom--testikulaer feminisering--Morris' syndrom. Kirurgisk procedure ved fund af testis i broksoek hos piger under herniotomi.