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。
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.
出处
《中国医科大学学报》
CAS
CSCD
北大核心
2010年第1期64-66,70,共4页
Journal of China Medical University
关键词
女性假两性畸形
荟萃分析
female pseudohermaphroditism
meta-analysis
