46XY单纯性腺发育不全2例病例报道及文献回顾  

46XY simple gonadal hypoplasia:2 case reports and literature review

作　　者：李雪[1] 丁梦帆 滕飞[1] 张艳芳[1] 罗营[1] 郑荣秀[1] 崔瑾[1] 张慧英[1] Li Xue;Ding Mengfan;Teng Fei;Zhang Yanfang;Luo Ying;Zheng Yongxiu;Cui Jin;Zhang Huiying(Department of Obstetrics and Gynecology,Tianjin Medical University General Hospital,Tianjin 300052,China)

机构地区：[1]天津医科大学总医院妇产科,天津300052

出　　处：《中国综合临床》2024年第1期65-69,共5页Clinical Medicine of China

摘　　要：46XY单纯性腺发育不全,又称Swyer综合征,患者往往因原发性闭经或青春期第二性征不发育就诊,其合并性腺肿瘤可能性较高,在治疗过程中往往推荐预防性切除性腺,术后予激素替代治疗。我们描述两例诊断为Swyer综合征的患者,1例合并性腺母细胞瘤及成熟畸胎瘤,1例为合并结节状Leydig细胞增生及异位的肾上腺组织,并进行文献复习。46XY simple gonadal hypoplasia,also known as Sweyer syndrome,patients often due to primary amenorrhea or pubertal secondary sex characteristics do not develop the doctor,its combined gonadal tumor is more likely,in the treatment process is often recommended prophylactic removal of gonads,postoperative hormone replacement therapy.We describe two patients diagnosed with Sweyer syndrome,one with gonadowlastoma and mature teratoma,and one with nodular Leydig cell hyperplasia and ectopic adrenal tissue,and reviews the literature.

关 键 词：46XY单纯性腺发育不全 Swyer综合征 原发性闭经 

分 类 号：R711.2[医药卫生—妇产科学]