摘要
胆道闭锁(biliary atresia,BA)是肝内外胆管进行性纤维化引起肝内外胆管硬化和闭锁的一种常见疾病,Kasai手术(肝门空肠吻合术)改善了患儿预后。手术时肝脏的病理状态,包括肝脏纤维化程度、胆管板畸形、肝门部纤维斑块、胆道闭锁类型都对Kasai术后的效果有影响。单纯用年龄评价手术预后有欠妥当。建立胆道闭锁治疗中心以提高外科医师实施Kasai手术的经验有助于改善手术预后,而胆道闭锁脾脏畸形综合征、早产儿、CMV感染对预后有不利影响。激素、熊去氧胆酸及抗生素的预防性使用其有效性目前尚有争议。
Biliary atresia (BA) is destructive inflammatory obliterative cholangiopathy in neonates. And it affects both intra-hepatic and extra-hepatic bile ducts. If untreated, progressive liver cirrhosis becomes lethal by age 2 years. Kasai portoenterostomy is a palliative surgical procedure for establishing bile drainage by uncovering duetules and restoring bile flow. Anatomic patterns of BA and pathological conditions of liver, including grade of liver fibrosis, ductal plate malformation, fibrotic plates of portal hepatis and cholangitis impacted the postoperative prognosis after Kasai operation. It is non-suitable for evaluating the prognosis simply by operative age. Establishing BA treatment center and surgical experiences may improve the postoperative prognosis. However, the unfavorable prognostic factors include BA splenic malformation syndrome, pre-term birth and infection with cytomegalovirus. The postoperative uses of ursodeoxycholic acid, high-dose steroids and prophylactic antibiotics have remained controversial.
出处
《中华小儿外科杂志》
CSCD
2016年第5期382-386,共5页
Chinese Journal of Pediatric Surgery
基金
国家自然科学基金(81270441)