先天性肝纤维化47例临床及病理特点分析  

Clinical and Pathological Features Analysis of 47 Cases of Congenital Hepatic Fibrosis

作　　者：马雪梅[1] 任辉[2] 郭晓东[3] 吴勤[1] 金波[1] 文凤[1] 孟繁平[1] 申力军[1] 刘博[1] 李捍卫[1] 

机构地区：[1]解放军302医院肝硬化诊疗中心,北京100039 [2]解放军302医院肝胆外科二中心,北京100039 [3]解放军302医院病理诊断与研究中心,北京100039

出　　处：《现代生物医学进展》2012年第34期6667-6669,共3页Progress in Modern Biomedicine

摘　　要：目的:分析先天性肝纤维化的临床及病理特点。方法:回顾性分析2008年1月-2012年8月在解放军第302医院住院治疗、资料完整的先天性肝纤维化患者的临床及病理资料。结果:先天性肝纤维化47例,男女比例25:22,平均发病年龄17.85±12.49岁,临床以门脉高压症表现为主,肝功能无明显减退,主要并发症为腹水及上消化道出血,45例肝组织病理主要表现为肝细胞板排列基本正常,汇管区增宽,炎细胞浸润不明显,较致密的纤维间隔穿插、包绕大致正常的肝实质,但不形成典型的假小叶,可伴有小胆管增生、扩张和畸形,其中35.6%伴有calori病。结论:先天性肝纤维化多以青少年时期起病,无明显性别差异,门脉高压与肝功能损害不一致,可伴有calori病,确诊需经肝组织病理学检查。Objective： To analyze the clinical and pathological features of congenital hepatic fibrosis（CHF）.Methods： Clinical and pathological features of 47 cases of congenital hepatic fibrosis in the 302 hospital of PLA from January 2008 to August 2012 were retrospectively studied.Results： There were 25 male and 22 female patients with mean age of 17.85±12.49 years old.The main clinical manifestations of CHF was portal hypertension and liver function test had no obvious decline.The main complications were ascites and hemorrhage of upper digestive tract.The main pathologic features of 45 cases of CHF showed that normally arranged hepatocytes were encompassed or intruded by the densely and non-inflammation collagenized septa,or hepatic plates were arrayed by fiber bundles with broaded periportal zone.No typical pseudolobule structure were found.Some cases showed small bile duct proliferation,expansion and deformity,35.6% of them with Calori disease.Conclusions：Congenital hepatic fibrosis usually presents in adolescent or young adulthood without significant gender difference.Portal hypertension and liver function damage are inconsistent in CHF patients,some of them with Calori disease.Liver biopsy is required for diagnosis.

关 键 词：先天性肝纤维化 门脉高压 病理 

分 类 号：R575.2[医药卫生—消化系统]