胆道闭锁的诊断及治疗新进展  

New advances in the diagnosis and treatment of biliary atresia

作　　者：王衍夫(综述) 富建华[2] 张志波(审校)[1] WANG Yan-Fu;FU Jian-Hua;ZHANG Zhi-Bo(Department of Neonatal Surgery/Pediatric Surgery,Shengjing Hospital of China Medical University,Shenyang 110004,China)

机构地区：[1]中国医科大学附属盛京医院新生儿外科/小儿外科,辽宁沈阳110004 [2]中国医科大学附属盛京医院新生儿内科,辽宁沈阳110004

出　　处：《中国当代儿科杂志》2022年第11期1269-1274,共6页Chinese Journal of Contemporary Pediatrics

摘　　要：胆道闭锁的诊断基于临床表现、筛查及相关化验检查。近年来血液标志物的研发和超声检查手段的提高使得胆道闭锁的就诊年龄普遍提前,其中最具代表性的突破为血清基质金属蛋白酶-7,以其高灵敏度和特异度超越了现有血生化指标的诊断效能,并正在向临床推广应用;肝弹性成像技术在诊断及预后判断方面也能在很大程度上提高诊断效能。手术仍是治疗胆道闭锁的唯一方法,当前序贯手术治疗方案首先是Kasai肝门空肠吻合术,加以抗感染、减轻炎症等药物治疗方案,而Kasai手术失败及肝功能丧失的患者,最终需要肝移植挽救生命。因此胆道闭锁目前的研究重点内容为早期诊断标志物,以及特异性靶向性、防止进行性肝纤维化药物的研发。该文将综述目前胆道闭锁的诊断方法和治疗措施,并探讨潜在的研究方向。The diagnosis of biliary atresia(BA)is mainly based on clinical manifestations,screening,and related biochemistry tests.In recent years,the development of blood biomarkers and the improvement in ultrasound examination have made it possible for BA to be diagnosed at a younger age.In particular,matrix metalloproteinase-7 shows high sensitivity and specificity and has a higher diagnostic efficiency than existing biochemical parameters,thereby holding a promise for clinical application.Sound touch elastography can increase the diagnostic efficiency for BA in terms of diagnosis and prognostic evaluation.Surgery is still the only method for the treatment of BA at present,with the preferred surgical treatment regimen of Kasai portoenterostomy combined with pharmacotherapies for alleviating infection and inflammation,and the patients who fail Kasai portoenterostomy or have liver dysfunction may require liver transplantation to save their lives.Therefore,the current research on BA should focus on the biomarkers for early diagnosis,specifically targeted drugs,and drugs for preventing progressive liver fibrosis.This article reviews the current diagnosis and treatment methods for BA and discusses the potential research directions.

关 键 词：胆道闭锁 诊断 标志物 治疗 新生儿 

分 类 号：R726.5[医药卫生—儿科]