特发性非硬化性门静脉高压诊治进展

Progress in the diagnosis and treatment of idiopathic non-cirrhotic portal hypertension

特发性非硬化性门静脉高压是一组少见的临床综合征,它的特征是没有肝硬化的组织学表现但有门静脉高压的临床表现,并且排除已知的可以引起门静脉高压的肝内外原因。其病因和发病机制尚不明确,但是可以确定与肝内血管病变的发展有关。目前认为可能的致病机制包括:免疫紊乱、慢性感染、毒物或药物损伤、微血栓形成、基因异常等。最常见的临床表现为食管胃底静脉曲张和脾肿大,腹水和肝性脑病则较少见。实验室检查表现为脾功能亢进引起的贫血、血小板减少及白细胞减少,而肝功能正常或轻度异常。此外,有一部分患者处于临床前期,尚未出现门静脉高压的表现,仅仅在肝活组织检查时发现有门静脉血管异常。诊断以排除性诊断为主,肝活组织检查是必不可少的。总体预后较肝硬化患者好,但目前仍以控制胃食管静脉曲张出血、防治血栓形成等对症治疗为主。

Idiopathic non-cirrhotic portal hypertension is a rare group of clinical syndromes characterized by clinical manifestations of portal hypertension in the absence of histological manifestations of liver cirrhosis,and with the exclusion of known intrahepatic and extrahepatic causes of portal hypertension.Importantly,its etiology and pathogenesis are unclear,but it has been determined to be related to the development of intrahepatic vascular disease.It is currently believed that possible pathogenic mechanisms include immune disorders,chronic infections,drug-related poisoning or injury,microthrombosis,genetic abnormalities,etc.The most common clinical manifestations are esophagogastric varices and splenomegaly.Ascites and hepatic encephalopathy are the least common.Laboratory test demonstrates anemia,leukopenia,and thrombocytopenia due to hypersplenism,and normal or mild liver function abnormality.In addition,in the preclinical stage,despite the presence of abnormal portal vein during liver biopsy,no signs of portal hypertension can be detected in some patients.Therefore,the diagnosis is based on the diagnosis of exclusion and mandatory liver biopsy.The overall prognosis of idiopathic non-cirrhotic portal hypertension is better than that of patients with liver cirrhosis,but symptomatic treatment(controlling gastroesophageal varices bleeding and preventing thrombosis)is still the main treatment.