Caroli病Ⅰ、Ⅱ型的临床特征—78例分析

2016-08-30欣吴孟晋罗生强杜霄壤冯兴中

胃肠病学 2016年7期

关键词：特征水平

吴　欣吴孟晋罗生强杜霄壤冯兴中*

首都医科大学附属北京世纪坛医院中医科1(100038)　解放军第302医院中西医结合肝病中心2

Caroli病Ⅰ、Ⅱ型的临床特征—78例分析

吴欣1吴孟晋1罗生强2杜霄壤1冯兴中1*

首都医科大学附属北京世纪坛医院中医科1(100038)解放军第302医院中西医结合肝病中心2

背景：Caroli病是一种以肝内大胆管节段性囊状扩张为特征的罕见先天性遗传性肝病。典型的Caroli病(Caroli病Ⅰ型)仅涉及胆管畸形，Caroli综合征(Caroli病Ⅱ型)指Caroli病合并先天性肝纤维化和门静脉高压。目的：探讨Caroli病Ⅰ、Ⅱ型的临床特征。方法：纳入2005年9月—2015年11月于首都医科大学附属北京世纪坛医院和解放军第302医院确诊Caroli病的患者78例，分为Ⅰ型组和Ⅱ型组，对两组性别、年龄、临床表现、体征、实验室指标、影像学和组织病理学表现等进行回顾性分析。结果：Ⅰ型组患者21例，中位年龄49.00 (31.00,57.25)岁，食欲减退和细菌性胆管炎的发生率显著高于Ⅱ型组(P<0.05)，无呕血、黑便，血常规正常，ALT、AST、碱性磷酸酶(ALP)、γ谷氨酰转移酶(GGT)和亮氨酸氨肽酶(LAP)水平升高；Ⅱ型组患者57例，中位年龄29.00 (14.50,40.00)岁，呕血、黑便、肝脾肿大、腹水和食管静脉曲张发生率显著高于Ⅰ型组(P<0.05)，WBC、HGB、血细胞比容(HCT)和PLT水平均低于正常值下限，肝功能基本正常。两组间年龄、凝血酶原时间(PT)、凝血酶原活动度(PTA)、国际标准化比率(INR)、纤维蛋白原(FIB)、WBC、中性粒细胞、RBC、HGB、HCT、PLT、ALT、ALP、GGT和LAP水平差异有统计学意义(P<0.05)。Caroli病Ⅰ型病理以肝内胆管节段性囊状扩张伴炎性细胞浸润为主要特征，Caroli病Ⅱ型病理以肝内胆管扩张伴门静脉周围纤维化为主要特征。结论：Caroli病Ⅰ型以肝内胆管节段性囊状扩张为主要特征，主要表现为肝功能异常，Caroli病Ⅱ型以门静脉高压症为主要表现，肝功能基本正常。

Caroli病；临床特征；肝功能试验；门静脉高压；回顾性研究

Correspondence to:FENG Xingzhong,Email:wux1@163.com

Background:Caroli’s disease is a rare congenital liver disease characterized by segmental saccular dilation of intrahepatic bile duct.Classic Caroli’s disease (type Ⅰ) involves the malformation of biliary tract,whereas Caroli’s syndrome (type Ⅱ) refers to the additional presence of associated congenital hepatic fibrosis and portal hypertension.Aims:To investigate the clinical features of type Ⅰ and type Ⅱ Caroli’s disease.Methods:Seventy-eight patients with the diagnosis of Caroli’s disease from Sept.2005 to Nov.2015 at Beijing Shijitan Hospital Affiliated to Capital Medical University and 302 Military Hospital of China were enrolled,and were assigned into type Ⅰ Caroli’s disease group and type Ⅱ Caroli’s disease group.Clinical data including gender,age,clinical manifestations,physical sign,laboratory test,imaging examination and histopathologic characteristics were retrospectively analyzed.Results:A total of 21 cases were assigned into type Ⅰ Caroli’s disease group and 57 cases were assigned into type Ⅱ Caroli’s disease group.In type Ⅰ group,the median age was 49.00 (31.00,57.25) years old,the incidences of poor appetite and bacterial cholangitis were significantly higher than those in type Ⅱ group (P<0.05),and no case with haematemesis and melena was found; blood routine tests were normal; levels of ALT,AST,alkaline phosphatase (ALP),γ-glutamyl transferase (GGT) and leucine aminopeptidase (LAP) were increased.In type Ⅱ group,the median age was 29.00 (14.50,40.00) years old,and the incidences of haematemesis,melena,hepatosplenomegaly,ascites and esophageal varices were significantly higher than those in type Ⅰ group (P<0.05),and levels of WBC,HGB,hematocrit (HCT) and PLT were below the lower limits of normal,while liver biochemical tests were essentially normal.Significant differences in age,prothrombin time (PT),prothrombin time activity (PTA),international normalized ratio (INR),fibrinogen (FIB),WBC,neutrophil,RBC,HGB,HCT,PLT,ALT,ALP,GGT and LAP were found between the two groups (P<0.05).Type Ⅰ Caroli’s disease was characterized by segmental saccular dilation of intrahepatic bile ducts with inflammatory cells infiltration,and type Ⅱ Caroli’s disease was characterized by dilated intrahepatic bile ducts along with portal fibrosis.Conclusions:Type Ⅰ Caroli’s disease is characterized by segmental saccular dilation of intrahepatic bile ducts with manifestations of abnormal liver function.Type Ⅱ Caroli’s disease is characterized by portal hypertension while liver function is essentially normal.

Caroli病由法国学者Jacques Caroli于1958年首次报道，主要表现为肝内胆管节段性交通性囊状扩张[1]。Caroli病分为2型，Ⅰ型为单纯型Caroli病，仅涉及胆管畸形；Ⅱ型为混合型，胆管畸形伴门静脉周围纤维化(先天性肝纤维化)和门静脉高压，称为Caroli综合征[1]。以往对Caroli病的研究局限于小样本病例研究或个案报道，缺乏对其临床特征的系统研究。本研究通过回顾性分析78例Caroli病患者的临床表现，旨在比较Caroli病Ⅰ型与Ⅱ型的临床特点。