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완전형 남성호르몬 불감증후군에서 골반경하 성선제거술 2례
이성재(Seong Jae Rhee),구현진(Hyun Jin Gu),한경안(Kyung An Han),신정규(Jeong Gyu Shin),최원준(Won Jun Choi),이순애(Soon Ae Lee),이종학(Jong Hak Lee),백원영(Won Young Paik) 대한산부인과학회 1999 Obstetrics & Gynecology Science Vol.42 No.10
Androgen insensitivity syndrome is a genetic syndrome characterized by complete or partial resistance of end organs to the peripheral effect of androgen. The phenotype of this condition is female, despite the normal male karyotype 46,XY. This is the third most common cause of primary amenorrhea after gonadal dysgenesis and mullerian agenesis. There is a congenital insensitivity to androgens, transmitted by means of a maternal X-linked recessive gene responsible for the androgen intracellular receptor. Prophylactic gonadectomy in usually performed due to increased risk for development of malignancy and possible virilization. Pelviscopy provides a minimally invasive technique for the accurate diagnosis and also provides the opportunity for therapeutic management of these patients. Recently we experienced two cases of this syndrome and removed the gonad by pelviscopic surgery, so we presented them with brief review of literature.