| Literature DB >> 7817416 |
Abstract
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.Entities:
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Year: 1994 PMID: 7817416
Source DB: PubMed Journal: Ugeskr Laeger ISSN: 0041-5782