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골반경으로 성선제거술을 시행한 남성호르몬 불감증후군 (완전형) 1 례
이정호,조남규,김판조,이은우 대한산부인과학회 1996 Obstetrics & Gynecology Science Vol.39 No.8
Androgen insensitivity syndrome is a genetic syndrome characterized by complete or partial resistance of end organs to the peripheral effect of androgen. patients have a male karyotype(46,XY) and bilateral testes. Appearance of the external genitalia depend on the degree of androgen insensitivity. In the complete from(Complete AIS), the external genitalia is normla female. In the incomplete from(Incomplete AIS), the external genitalia appearance varies from that of a virilized female to that of an undervirilized male with a short penis penis and hypospadia. The usual presenting complaint associated with complete AIS is an inguinal hernia in a female infant or primary amenorrhea. In incomplete AIS, the patient present with ambiguous genitalia in a neonate or later because of insufficient virilization during puberty. Recently we experienced one case of this syndrome and removed the gonad by pelviscopic surgery, so we report it with brief review of literature.