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Androgen Insensitivity Syndrome (AIS) is a collection of sex development disorders caused by mutations of the genes encoding the androgen receptor. The resulting disorders vary according to the structure and sensitivity of the abnormal receptor. Common forms of AIS involve a variable degree of undervirilization and/or infertility in XY persons of any gender. A person with complete androgen insensitivity syndrome (CAIS) has a female external appearance despite a 46XY karyotype and undescended testes.

Since the early 90's, scientific advances have expanded the medical understanding and management of the molecular mechanisms of the clinical features of AIS. The value of accurate and scientifically detailed information for patients is now emphasized, with physicians no longer automatically recommending surgical corrections, with an elective option now viewed as a possible but no longer necessary intervention for ambiguous conditions.

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