国际妇产科学杂志

国际妇产科学杂志 ›› 2015, Vol. 42 ›› Issue (5): 508-510.

• 论著 • 上一篇    下一篇

完全型雄激素不敏感综合征4例诊治分析

王晓峰，朱颖军   

  1. 300100 天津市中心妇产科医院
  • 收稿日期:1900-01-01 修回日期:1900-01-01 出版日期:2015-10-15 发布日期:2015-10-15
  • 通讯作者: 朱颖军

Clinical Analysis of 4 Cases of Complete Androgen Insensitivity Syndrome

WANG Xiao-feng，ZHU Ying-jun   

  1. Tianjin Central Hospital of Gynecology and Obstetrics，Tianjin 300100，China
  • Received:1900-01-01 Revised:1900-01-01 Published:2015-10-15 Online:2015-10-15
  • Contact: ZHU Ying-jun

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摘要： 目的：认识完全型雄激素不敏感综合征（CAIS），阐述腹腔镜手术在其诊治中的作用。方法：回顾性研究2009年9月—2012年10月天津市中心妇产科医院普通妇科收治并行手术治疗的4例CAIS患者，根据其病史、临床表现、激素水平测定[睾酮，雌二醇（E2），黄体生成激素（LH），卵泡刺激素（FSH），孕酮，泌乳素（PRL）等]和术中所见、手术方式及病理结果，结合文献进行比较分析。结果：所有患者的染色体核型均为46，XY。激素水平测定，与正常男性相比，3例睾酮正常，1例明显低于正常；4例LH均升高；1例FSH正常，3例高于正常，其中1例明显高于正常；4例E2、孕酮、PRL均正常。1例行腹腔镜下双侧睾丸切除术，1例行腹腔镜下双侧睾丸切除术+会阴阴道成形术，1例行经外阴双侧睾丸切除术，1例行腹腔镜辅助下经腹股沟睾丸切除术。4例切除性腺后病理证实均为发育不良的睾丸组织。结论：CAIS的早期诊断非常重要，治疗宜按女性社会性别对待，切除睾丸后继续以雌激素替代治疗，腹腔镜手术在诊治CAIS中具有独特优势。

关键词: 性腺发育不全, XY, 雄激素类, 腹腔镜, 完全型雄激素不敏感综合征

Abstract: Objective：To investigate the complete androgen insensitivity syndrome （ＣＡＩＳ）, and evaluate the application of laparoscopy in the diagnosis and treatment in CAIS. Methods：Four cases of CAIS admitted to and underwent surgery in General Gynecology in Tianjin Central Hospital of Gynecology and Obstetrics from September of 2009 to October of 2012 were studied retrospectively, through analyzing and comparing medical history, clinical characteristics, hormone levels [testosterone, estradiol （Ｅ２）, luteinizing hormone （ＬＨ）, follicle stimulating hormone （ＦＳＨ）, progesterone, prolactin （ＰＲＬ） etc.], surgical findings and gonad pathological results were analysed combined with literatures. Results：All patients had a karyotype of 46, XY. Hormone levels were measured, compared with normal males, three cases had normal testosterone, one was significantly lower than the normal, four cases had elevated LH, FSH was normal in one case but higher than normal in other three cases, one in which was significantly higher than the normal; E2, progesterone, PRL were normal in four cases. One case underwent laparoscopic bilateral orchiectomy, one case underwent laparoscopic bilateral orchiectomy and perineal vaginoplasty, one case underwent bilateral orchiectomy by vulva, one case underwent laparoscopic assisted bilateral orchiectomy by inguinal. Four cases of pathologically proven after excision of the gonads were dysplasia of testicular tissue. Conclusions：Early diagnosis of CAIS is very important, patients should be treated as female social gender with gonadectomy and subsequent hormone replacement. Laparoscopy has unique advantages in the diagnosis and treatment of CAIS.

Key words: Gonadal dysgenesis, XY, Androgens, Laparoscopes, Complete androgen insensitivity syndrome