先天性肝纤维化14例临床病理特点

赵新颜; 张新; 王泰龄; 欧晓娟; 贾继东

先天性肝纤维化14例临床病理特点

首都医科大学附属北京友谊医院

详细信息

中图分类号: R575.2
计量
- 文章访问数: 3017
- HTML全文浏览量: 30
- PDF下载量: 898
- 被引次数: 0
出版历程
- 出版日期: 2010-02-20

Clinical and pathological features of patients with congenital hepatic fibrosis

摘要

摘要: 目的总结先天性肝纤维化的临床特点,描述先天性肝纤维化时产生门静脉高压的病理形态学特点。方法回顾性分析2000年～2009年9月,北京友谊医院门诊及住院诊断为先天性肝纤维化患者的临床特点,对活检肝组织标本进行特殊染色及免疫组织化学染色。结果先天性肝纤维化14例,男女比例1.33:1,平均年龄(14.78±8.52)岁,临床主要表现为门脉高压症型占57.1%,胆管炎型占14.29%,混合型占7.14%。肝脾肿大为最主要的体征占85.7%。常见的合并症为多囊肾和肝内胆管扩张,即Caroli’s病各占14.28%。病理形态学观察提示先天性肝纤维化导致门脉高压的机制包括:门静脉发育不良,门脉数量减少,纤维间隔挤压门静脉。结论先天性肝纤维化是导致非肝硬化性门脉高压症的常见原因之一,掌握其临床及病理特点,有助于提高诊治水平。
- 先天性肝纤维化 /
- 门脉高压 /
- 肝硬化 /
- 非肝硬化性门脉高压
Abstract: Objective To summarize the clinical features of congenital hepatic fibrosis (CHF) and to describe pathological features of portal hypertension (PHT) in CHF.Methods A retrospective study was conducted on 14 pathologically confirmed CHF cases from 2000 to 2009 and pathological samples were analyzed by staining.Results The male to female ratio of CHF was 1.33∶1 among the patients.The mean age was 14.78±8.52 (Range 6 to 35 years) .Variceal bleeding caused by PHT (57.1%) and cholangitis (14.3%) were major clinical manifestations, while hepatomegaly and splenomegaly occurred in 85.7% of the cases.The common associated conditions include Caroli's disease (14.28%) and polycystic kidney diseases (14.28%) .Portal hypertension in CHF was caused by the paucity and malformation of portal vein branches and compression of portal vein branches by fibrotic bands.Conclusion CHF is one of the common causes of cirrhotic PTH.Understanding the clinical and pathological features CHF will contribute towards its diagnosis and management.
- congenital hepatie fibrosis (CHF) /
- portal hypertension (PHT) /
- cirrhosis /
- noncirrhotic PHT

HTML全文

参考文献(8)

[1]Kerr DNS, Harrison CV, Sherlock S, et al.Congenital hepatic fibro-sis[J].Q J MED, 1961, 30 (1) :91-117.

[2]Poddar U, Thapa BR, Vashishta RK, et al.Congenital hepatic fi-brosis in Indian children[J].J Gastroenterol Hepatol, 1999, 14 (12) :1192-1196.

[3]赵新颜, 王宝恩, 贾继东.36例先天性肝纤维化的临床特点[J].中华消化杂志, 2005, 25 (12) :748-749.

[4]Desmet VJ.Ludwig symposium on biliary disorders-part I.Patho-genesis of ductal plate abnormalities[J].Mayo Clin Proc, 1998, 73 (1) :80-89.

[5]Gunay-Aygun M, Avner ED, Bacallao RL, et al.Autosomal reces-sive polycystic kidney disease and congenital hepatic fibrosis:summa-ry statement of a first national institutes of health/office of rare disea-ses conference[J].J Pediatr, 2006, 149 (2) :159-164.

[6]Turkbey B, Ocak I, Daryanani K, et al.Autosomal recessive polycys-tic kidney disease and congenital hepatic fibrosis (ARPKD/CHF) [J].Pediatr Radio, 2009, 39 (2) :100-111.

[7]Nakanuma Y, Harada K, Sato Y, et al.Recent progress in the etio-pathogenesis of pediatric biliary disease, particularly Caroli's disease with congenital hepatic fibrosis and biliary atresia[J].Histol His-topathol, 2010, 25 (2) :223-235.

[8]Gunay-Aygun M.Liver and kidney disease in ciliopathies[J].Am J Med Genet C Semin Med Genet, 2009, 151C (4) :296-306.

施引文献

资源附件(0)

访问统计