中国医科大学学报

中国医科大学学报

中国医科大学学报 ›› 2010, Vol. 39 ›› Issue (1): 64–.

• 临床医学 • 上一篇    下一篇

342例女性假两性畸形的荟萃分析

李全荣; 林蓓; 伊喜苓; 刘水策; 王圣坦; 张淑兰   

  1. 中国医科大学附属盛京医院妇产科
  • 出版日期:2010-01-20 发布日期:2013-06-07
  • 通讯作者: 林蓓 E-mail:linbei88@hotmail.com

Meta-analysis of 342 Female Pseudohermaphroditism

  • Online:2010-01-20 Published:2013-06-07

摘要:

目的总结1994-2009年国内外公开发表的以及我院2000年至今诊治的女性假两性畸形(FPH)的病例,探讨其临床特点。方法以"女性假两性畸形"的中英文单词作为检索词,检索1994-2009年国内外公开发表的文献,并结合我院诊治的10例患者,进行荟萃分析。结果共检索到332例诊断明确的病例,加上我院的10例,共有342例入选病例。出生时即就诊的患者仅有27例,其他病例就诊年龄为1个月59岁不等,315例患者就诊时的中位年龄为29.54岁。病因以先天性肾上腺增生为主(70.47%)。临床表现主要为骨骼生长较快(95.52%)、阴蒂肥大(84.14%)以及不同程度的男性化(77.24%)。在治疗方式上以药物和手术的联合治疗为主。首次接受治疗的时间及治疗方法在预后方面起着至关重要的作用。结论女性假两性畸形并非罕见疾病,我们应提高对该病的认识,提早患者接受治疗的时间,坚持终身用药,以改善其预后。

关键词: 女性假两性畸形, 荟萃分析, 女性假两性畸形, 荟萃分析

Abstract:

Objective To summarize the clinical characteristics of female
      pseudohermaphroditism(FPH)in the publication from 1994 to 2009 and the
      cases diagnosed and treated in our hospital from 2000. Methods We employed
      Chinese and English name of"female pseudohermaphroditism"as a key word
      separately to retrieve published articles of FPH during 1994 to 2009. A
      meta-analysis was performed together with the ten cases in our hospital.
      Results Among 342 cases involved in this study,only twenty-seven children
      were diagnosed immediately after birth and the other 315 cases at the age
      of 1 month to 59 years with median age of 29.54 years. The etiological
      factors were mostly congenital adrenal hyperplasia(70.47%). Clinical
      manifestations were advanced bone age(95.52%),clitoromegaly (84.14%)and
      early virilization (77.24%). The treatment mainly included drug and
      operation. Early diagnosis and available therapy were very important for
      the prognosis. Conclusion FPH is not rare,so we should improve our
      knowledge for its early diagnosis,good intervention and final better
      prognosis.

Key words: female pseudohermaphroditism, meta-analysis

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