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Research advances in idiopathic non- cirrhotic portal hypertension
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摘要:
特发性非肝硬化门静脉高压症(INCPH)是一种罕见疾病,其特点是肝内门静脉高压,且无肝硬化或者其他肝脏疾病以及门静脉、肝静脉血栓的表现。目前对于INCPH的病因学研究主要集中在免疫系统疾病、慢性感染、接触药物或毒物、遗传性倾向、促血栓或高凝状态几个方面。INCPH临床表现的主要特点为门静脉高压、脾大或巨脾以及脾功能亢进,而肝损伤、腹水以及肝性脑病较少见。目前,对INCPH的诊断尚无统一标准,还是以排除性诊断为主。治疗方面,最新的BavenoⅥ共识对于INCPH的推荐治疗方法还是参照肝硬化门静脉高压的治疗指南。由于大部分INCPH患者的肝功能良好,所以其预后比相同门静脉压力的肝硬化患者好,但也有少部分患者可进展至肝衰竭,并需要肝移植治疗。
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.
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Key words:
- hypertension /
- portal /
- splenomegaly
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