LD2A.4

46,XY disorder of sex development due to androgen resistance

Transtorno 46, XY do desenvolvimento sexual devido à resistência a androgênios

Category

Definition

Androgen insensitivity syndrome (AIS) is a disorder of sex development (DSD) characterised by the presence of female external genitalia, ambiguous genitalia or variable defects in virilization in a 46,XY individual with absent or partial responsiveness to age-appropriate levels of androgens. It comprises two clinical subgroups: complete AIS (CAIS) and partial AIS (PAIS).

Index Terms

46,XY disorder of sex development due to androgen resistanceAndrogen resistance syndromeTesticular feminization syndromeAndrogen insensitivity syndromeGoldberg-Maxwell syndromeMorris syndromeMale pseudohermaphroditism with androgen resistanceperipheral androgen receptor disorderGoldberg-Morris syndromesyndrome of feminising testessyndrome of feminizing testestesticular feminisationtesticular feminisation syndromemale pseudohermaphroditism with feminizing testisTFM - [testicular feminisation syndrome]Complete peripheral androgen resistance syndromeComplete androgen insensitivity syndrome46,XY disorder of sex development due to complete androgen insensitivity46,XY DSD due to complete androgen insensitivitycomplete androgen resistance syndromePartial peripheral androgen resistance syndrome46,XY disorder of sex development due to partial androgen insensitivity46,XY DSD due to partial androgen insensitivityReifenstein syndromePartial androgen insensitivitypartial androgen resistance syndrome