肝前性非肝硬化性门静脉高压症的诊疗进展

Research advances in the diagnosis and treatment of prehepatic noncirrhotic portal hypertension

肝前性非肝硬化性门静脉高压是一组由非肝硬化因素导致的门静脉高压性疾病,目前认为这组疾病的病因主要与JAK2突变、血液高凝状态有关。临床表现主要包括静脉曲张破裂出血、脾肿大等,患者肝功能正常或仅有轻微异常。对肝前性非肝硬化性门静脉高压疾病的诊断主要依靠其影像学特点,同时还需排除肝硬化门静脉高压。治疗方面主要采取抗凝治疗和内镜下治疗,患者预后一般较好。本文系统梳理了肝前性非肝硬化性门静脉高压的主要进展。

Prehepatic noncirrhotic portal hypertension is a group of diseases caused by noncirrhotic factors,and currently it is believed that the etiology of these diseases is mainly associated with JAK2 mutation and blood hypercoagulability.Main clinical manifestations include variceal bleeding and splenomegaly,with normal or slightly abnormal liver function.The diagnosis of prehepatic noncirrhotic portal hypertension mainly depends on its imaging characteristics,and meanwhile cirrhotic portal hypertension should be excluded.Anticoagulant therapy and endoscopy are the major therapies for these diseases,and patients often have a good prognosis.This article systematically reviews the recent advances in prehepatic noncirrhotic portal hypertension.