摘要
Abstract
Idiopathic non-cirrhotic portal hypertension (INCPH) is a rare disease,which is characterized by portal hypertension in the liver and the absence of liver cirrhosis,other liver diseases,or portal or hepatic vein thrombosis.At present,the research on the etiology of INCPH mainly focuses on the aspects of immune system diseases,chronic infections,exposure to drugs or toxins,genetic predisposition,and prothrombotic conditions or hypercoagulability.The main features of the clinical manifestations of INCPH are portal hypertension,splenomegaly,and hypersplenism,while liver injury,ascites,and hepatic encephalopathy are rare.Currently,there are no clear diagnostic criteria for INCPH,and a diagnosis is often made by exclusion.As for the treatment of INCPH,the therapeutic method recommended in the latest Baveno VI consensus is to follow the current guidelines for the treatment of cirrhotic portal hypertension.Since the majority of INCPH patients have good liver function,they have better prognosis compared with the patients with liver cirrhosis,but some of them may still develop liver failure and need liver transplantation.关键词
高血压,门静脉/脾大/综述Key words
hypertension, portal/splenomegaly/review分类
医药卫生
