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Syndrome, Complete Androgen Insensitivity

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Definition

A genetic disorder that makes XY fetuses insensitive (unresponsive) to androgens (male hormones) because of a lack of a receptor for testosterone on the cells. Instead, they are born looking externally like normal girls. Internally there is a short blind-pouch vagina and no uterus, Fallopian tubes, or ovaries. There are testes in the abdomen or the inguinal canal. The complete androgen insensitivity syndrome is usually detected at puberty when a girl should, but does not begin to menstruate. The gene for the syndrome is on the X chromosome and codes for the androgen receptor (also called the dihydrotestosterone receptor). There is also partial androgen insensitivity syndrome.


 

 

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