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首页> 外文期刊>World Journal of Gastroenterology >Is portal vein cavernous transformation a component of congenital hepatic fibrosis?
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Is portal vein cavernous transformation a component of congenital hepatic fibrosis?

机译：门静脉海绵状转化是否是先天性肝纤维化的组成部分？

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摘要

Congenital hepatic fibrosis (CHF) is an autosomal recessive disorder that belongs to the family of fibropolycystic liver diseases. This family includes a spectrum of disorders which are usually found in combination with each other and are usually inherited. Clinically fibropolycystic diseases have three effects being present in different proportions, those of a space occupying lesion, of portal hypertension and of cholangitis. In most patients, the first manifestations of CHF are signs and symptoms related to portal hypertension such as splenomegaly and varices. Portal hypertension in these patients has been attributed to the hypoplasia or compression of the portal vein radicles in the fibrous bands. Cavernous transformation of the portal vein (CTPV) is a relatively rare condition resulting from extrahepatic portal vein obstruction with recanalization or collateral vein formation to bypass the obstruction. It has been found that patients with CHF having an accompanying CTPV have relatively large splenomegaly and suffers more frequent episodes of bleeding from esophageal varices.We believe that CTPV is a congenital component of CHF and also one of the important causative factors of portal hypertension in these patients.
机译：先天性肝纤维化（CHF）是一种常染色体隐性遗传疾病，属于纤维性多囊性肝病家族。该家族包括一系列疾病，这些疾病通常相互结合并通常遗传。临床上，纤维性多囊性疾病具有三种不同比例的作用，即占位性病变，门脉高压和胆管炎。在大多数患者中，CHF的最初表现是与门脉高压相关的体征和症状，如脾肿大和静脉曲张。这些患者的门静脉高压症可归因于纤维带中门静脉小径根系发育不全或受压。门静脉海绵状变（CTPV）是由肝外门静脉阻塞伴再通或侧支静脉旁路形成的一种相对罕见的疾病。已发现伴有CTPV的CHF患者脾肿大相对较大，食管静脉曲张破裂出血频率更高。我们认为CTPV是CHF的先天性成分，也是门静脉高压的重要原因之一。耐心。

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