胆道闭锁胆汁湖形成与预后关系研究

Relationship between formation and prognosis of bile lake with biliary atresia

目的 选取接受肝移植胆道闭锁患儿自体肝组织,观察胆汁湖结构,对比患儿临床指标及肝组织病理变化,探讨胆道闭锁患儿Kasai术后肝内胆汁湖形成与临床疾病进展之间的关系.方法 收集2012年1月至2013年12月16例Kasai术后接受肝移植的胆道闭锁患儿的自体肝脏标本,8例存在胆汁湖肝脏标本作为研究组,8例无胆汁湖肝脏标本作为对照组.分别从自体肝组织胆汁湖部位,肝脏左叶、右叶及肝门处取材,蜡块包埋,组织切片,应用HE染色及CK 19免疫组织化学染色方法,观察胆汁湖形态结构改变;同时收集患儿临床资料,分析胆汁湖形成对患儿Kasai术后临床疾病进展的影响.结果 研究组Kasai手术日龄（44～86 d）和肝移植年龄（216～1095 d）均低于对照组,其中肝移植年龄差异有统计学意义（P＜0.05）.胆汁湖多出现在肝右叶,肝左叶少见,发生于左、右叶的胆汁湖常靠近肝门部位.胆汁湖形状分为不规则或多角形和类圆形,研究组中多角形5例（62.5％）,直径较小（0.5～3.0） cm,内壁被覆纤维组织,胆湖内可见结石,并有较多炎细胞浸润,胆湖周围纤维组织增生较重,胆管增生不明显;类圆形3例（37.5％）,直径较大（1.3～5.0）cm,胆湖内结石少见,炎细胞浸润较重,胆湖周围胆管轻度增生.研究组中4例多角形胆湖患儿术后胆管炎反复发生,黄疸持续不退,胆汁湖形成早,肝功能恶化快,尤其是TBIL、DBIL和γ-GT升高程度明显高于其他患儿,肝移植时间都在1岁以内.结论 胆汁湖的出现是增加胆管炎发生风险的密切相关因素,出现肝内胆湖情况其肝脏功能衰竭进展较快,尤其是多角形胆汁湖,提示应尽早行肝移植手术.

Objective To explore the relationship between postoperative intrahepatic bile lake formation and clinical disease progression.Methods From January 2012 to December 2013,liver specimens were collected from liver transplantation patients after Kasai portoenterstomy.Based on the presence of bile lake,16 specimens were divided into experimental and control groups （n =8 each）.Left lobe,right lobe and porta hepatis of liver were resected respectively for paraffin-embedded sections.Hematoxylin ＆ eosin and CK19 stains were employed for observing the morphological changes of bile lake.And the clinical data were collected for analyzing the effects of bile lake on postoperative disease progression.Results Kasai procedure age （44-86 days） and liver transplantation age （216-1,095 days）in experimental group were lower than those of control group.And liver transplantation age had significant difference （P＜0.05）.Bile lake often occurred close to porta hepatis in right lobe while the involvement of left lobe was rare.The shapes of bile lake were irregular,polygonal or round.In experimental group,there were 5 polygonal cases with a smaller diameter （0.5-3.0 cm）.There was a wall covering of fibrous tissue with visible stones and an infiltration of inflammatory cells.Fibrous tissue abounded around bile lake and bile duct hyperplasia was not obvious; 3 round cases had a large diameter （1.3-5.0 cm）.Gallstones were rare and there was a greater infiltration of inflammatory cells heavier.There was a mild hyperplasia of bile duct around lake.In experimental group,4 cases of polygonal bile lake had recurrent cholangitis,persistent jaundice,early formation of bile lake and a rapid deterioration of liver function.The levels of total bilirubin,direct bilirubin andγ-glutamyl transferase were significantly higher than peers.Liver transplantation was performed under 1 year.Conclusions The emergence of bile lake is closely correlated with an elevated risk of cholangitis.Liver function failure has a quick progression,especially polygonal bile lake.And liver transplantation should be performed as soon as possible.