CAJ | 학술논문

目的总结分析进行性家族性肝内胆汁淤积(PFIC)的非移植外科治疗的不同手术方式及其治疗现状.方法检索PubMed、EMBASE、Scopus和中国生物医学文献数据库(CBM)非移植外科治疗PFIC的所有相关文献,提取临床资料,总结分析.结果 20项个案或病例系列报道纳入本研究,共144例患儿接受非移植外科治疗,其中108例(75％)预后良好.疾病进展,脏纤维化或硬化,往往提示预后不良.结论从某种意义上讲,虽然本荟萃分析所纳入研究存在一定的不全面性和不一致性,但是非移植外科治疗确实能明显缓解PFIC患儿症状,遏制疾病进展.
목적 총결분석진행성가족성간내담즙어적(PFIC)적비이식외과치료적불동수술방식급기치료현상.방법 검색PubMed、EMBASE、Scopus화중국생물의학문헌수거고(CBM)비이식외과치료PFIC적소유상관문헌,제취림상자료,총결분석.결과 20항개안혹병례계렬보도납입본연구,공144례환인접수비이식외과치료,기중108례(75％)예후량호.질병진전,장섬유화혹경화,왕왕제시예후불량.결론 종모충의의상강,수연본회췌분석소납입연구존재일정적불전면성화불일치성,단시비이식외과치료학실능명현완해PFIC환인증상,알제질병진전.
Objective To review the outcome of the different choices of non-transplant surgical treatment for progress familial intrahepatic cholestasis (PFIC) by performing meta-analysis.Methods A systematic literature search of PubMed,Embase,Scopus and Chinese Biomedical Literature Database (CBM) was performed.All the articles regarding the outcome of non-transplant surgery in PFIC patients were recruited in this study.Results A total of 20 case series or case reports were included in this study.Of the 144 patients who underwent a non-transplant surgery,108 (75％) patients had favorable outcomes.Unfavorable outcomes were often associated with more advanced stage of liver fibrosis or cirrhosis.Conclusions Non-transplant surgical interventions in PFIC patients can effectively delay progression of liver cirrhosis.