完全正常女性外觀之雄性素阻抗症：病例報告及文獻回顧
歐詠豪1、林宜佳1,2、蔡德甫1,2、黃一勝1,2
新光吳火獅紀念醫院 外科部 泌尿科1
輔仁大學 醫學系2
Case report: The clinical features and management of complete androgen insensitivity syndrome in a 16-year-old female
AO WENG HOU1, Yi-Chia Lin 1,2, Te-Fu Tsai 1,2 , Thomas I.S. Hwang 1,2
Division of Urology, Department of Surgery, Shin Kong WHS Memorial Hospital1
School of Medicine, Fu-Jen Catholic University2
Abstract:
Androgen play a pivotal role in the development of male reproductive organ and function. Disorders of androgen receptor function can cause hermaphroditism. We present a 16-year-old female who was diagnosed with complete androgen insensitivity syndrome. She experienced amenorrhea after puberty with unequivocal female external genitalia. Chromosome analysis showed the karyotype was 46, XY. Elevated level of testosterone was also found. Computed tomography revealed possible bilateral intra-abdominal testes without evidence of female genital organs. Prophylactic laparoscopic bilateral orchiectomy was performed to prevent tumor formation in cryptorchid testes. For patients with this disorder, the timing of gonadectomy, gender assignment and psychological support should initially be focused.