Autor: |
Landen, Serge, Doniga, Sebastian, Bollars, Peter, Delugeau, Veronique, Merlin, Jean |
Zdroj: |
Gynecological Surgery; Nov2009, Vol. 6 Issue 4, p375-379, 5p |
Abstrakt: |
Two elderly identical twin sisters with inguinal hernias and malignant gonadic tumors were diagnosed during surgery as having complete androgen insensitivity syndrome. Androgen insensitivity syndrome, also referred to as testicular feminization, is an X-chromosome-linked genetic condition in which the tissues of a genotypic male are unresponsive to androgens because of an anomaly of the androgen receptor. This results in feminization of the external genitalia. The internal genital organs including the cranial part of the vagina, cervix, uterus, and fallopian tubes are absent because of testicular inhibition during embryonic development. Patients most frequently present as young females with asymptomatic bilateral inguinal hernias. They may undergo normal puberty but have primary amenorrhea. Rarely, the diagnosis may follow a workup for infertility. As in cryptorchidia, the testicles are prone to malignant transformation and require that orchidectomy be performed, usually after puberty. [ABSTRACT FROM AUTHOR] |
Databáze: |
Complementary Index |
Externí odkaz: |
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