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作 者:乔奇(综述) 张志波(审校)[1] Qiao Qi;Zhang Zhibo(Department of Neonatal Surgery,Affiliated Shengjing Hospital,China Medical University,Shenyang 110004.)
机构地区:[1]中国医科大学附属盛京医院新生儿外科,辽宁省沈阳市110003
出 处:《临床小儿外科杂志》2021年第2期133-139,共7页Journal of Clinical Pediatric Surgery
基 金:国家自然科学基金(编号:81270437);辽宁科技厅项目(编号:2017225014)。
摘 要:胆道闭锁(biliary atresia,BA)是婴儿期肝内外胆管进行性炎症及肝纤维化的疾病,其病因及疾病进展机制尚不清楚。Kasai手术(Kasai portoenterostomy,KPE)是治疗胆道闭锁的首选术式,成功的Kasai手术能够重建胆汁引流,但Kasai手术后长期自体肝生存(native liver survival,NLS)率较低,患者常合并各种并发症,因而Kasai手术后大部分患者在成年前需行肝移植手术,而Kasai手术后综合管理对NLS及患者的长期生存质量有着极大的影响,如何改善Kasai手术后综合管理值得深入探讨。Biliary atresia(BA)is a disease of progressive inflammation and fibrosis of intra/extrahepatic bile ducts in infants.Its etiology and mechanism of progression have remained ill elucidated.Kasai portoenterostomy(KPE)is a first choice for biliary atresia.Successful KPE operation may reconstruct bile drainage.However,the long-term native liver survival(NLS)after KPE operation is relatively low and children often suffer from various complications.Most children require liver transplantation before adulthood.Comprehensive managements after KPE have great impact on NLS and long-term quality of life of children.And how to improve postoperative managements is vital for the prognosis of BA.
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