CAJ | 학술논문

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.
목적 총결1994-2009년국내외공개발표적이급아원2000년지금진치적녀성가량성기형(FPH)적병례,탐토기림상특점.방법 이"녀성가량성기형"적중영문단사작위검색사,검색1994-2009년국내외공개발표적문헌,병결합아원진치적10례환자,진행회췌분석.결과 공검색도332례진단명학적병례,가상아원적10례,공유342례입선병례.출생시즉취진적환자부유27례,기타병례취진년령위1개월～59세불등,315례환자취진시적중위년령위29.54세.병인이선천성신상선증생위주(70.47%).림상표현주요위골격생장교쾌(95.52%)、음체비대(84.14%)이급불동정도적남성화(77.24%).재치료방식상이약물화수술적연합치료위주.수차접수치료적시간급치료방법재예후방면기착지관중요적작용.결론 녀성가량성기형병비한견질병,아문응제고대해병적인식,제조환자접수치료적시간,견지종신용약,이개선기예후.
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.