Medical Definition of Complete androgen insensitivity syndrome

  • Medical Author:
    Melissa Conrad Stöppler, MD

    Melissa Conrad Stöppler, MD, is a U.S. board-certified Anatomic Pathologist with subspecialty training in the fields of Experimental and Molecular Pathology. Dr. Stöppler's educational background includes a BA with Highest Distinction from the University of Virginia and an MD from the University of North Carolina. She completed residency training in Anatomic Pathology at Georgetown University followed by subspecialty fellowship training in molecular diagnostics and experimental pathology.

Reviewed on 9/7/2018

Complete androgen insensitivity syndrome: An older term for the complete androgen insensitivity syndrome, a genetic disorder that makes XY fetuses insensitive (unresponsive) to androgens (male hormones). 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 are also partial androgen insensitivity syndromes.

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Reviewed on 9/7/2018