Health10 Jul 2009 03:27 pm

Similar to the longstanding urban legend claiming that Michael Jackson’s prosthetic nose flew off and struck an astonished audience member during a concert, actress Jamie Lee Curtis has been plagued by the decades-old rumor that she was actually born a hermaphrodite and/or suffers from Testicular feminization syndrome. Testicular feminization syndrome, also known as complete Androgen insensitivity syndrome (AIS), is a genetic disorder that that renders XY fetuses unresponsive to androgens (male hormones, including testosterone). This insensitivity to androgens prevents a genetically male fetus from developing male body parts and other physical characteristics. Thus, people with this disorder are born looking female, even though they are genetically male: internally, they have testes (located either in the abdomen or the inguinal canal), but externally they have a vaginal opening (with no uterus). It is estimated that complete AIS occurs in 1 in 20,000 live births.
Because a person with complete AIS externally appears female, the disorder usually remains undetected until puberty, when a girl would normally begin to menstruate. A person with AIS might have little or no pubic or armpit hair; however, they frequently have luxurious scalp hair (and do not develop male pattern baldness later in life). Upon the commencement of puberty, a person with AIS will develop breasts, but do not menstruate and are infertile, as they lack a uterus.
AIS can be diagnosed with a pelvic sonogram, genetic testing and/or blood work. Once diagnosed with the syndrome, a person will usually begin estrogen replacement therapy, as the syndrome sharply increases the likelihood of developing osteoporosis later in life. Moreover, the testicular tissue is usually removed after puberty, because of an increased risk of developing cancer.
Despite the widespread dissemination of this urban legend, only circumstantial evidence has been put forward in support of the claim that Jamie Lee Curtis actually has AIS. Proponents of this rumor cite her “androgynous” appearance, “gender-bending” name and the fact that she and her husband, actor Christopher Guest, adopted their two children. Interestingly, the longevity of the rumor is often attributed to the fact that it is spoken of as a given in medical school classrooms and textbooks, despite the fact that she has never been mentioned in any medical textbooks or journals to date.
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