布-加综合征、肝血窦阻塞综合征与肝硬化的鉴别

Identification of Budd-Chiari syndrome,hepatic sinusoidal obstruction syndrome and hepatic cirrhosis

布-加综合征是由肝静脉或其开口以上的下腔静脉阻塞性病变引起的以下腔静脉阻塞、门静脉高压为特点的综合征,诊断依靠影像学检查,治疗的关键是解除梗阻。肝血窦阻塞综合征是指肝窦内皮完整性破坏和肝窦内充血阻塞而产生的肝内窦后性门静脉高压症,临床表现类似布-加综合征,诊断依靠肝组织活检。这两种疾病的治疗和预后均与肝实质病变导致的肝硬化不同,所以临床上需要注意这三种疾病的鉴别。

The Budd-Chiari syndrome（BCS） is caused by obstruction of the hepatic venous or the inferior vena cava which results in hepatic sinusoidal pressure increased and portal hypertension.The diagnosis of BCS depends on imageology.The key point of the therapy is the relief of the obstruction.The pathomechanism of hepatic sinusoidal obstruction syndrome（SOS）is intrahepatic portal vein hypertension result from damaged of the integrity of sinusoidal endothelial,which causes sinusoidal congestion.Its clinical manifestation is similar to BCS.Its diagnosis depends on pathology.The therapy and prognosis of the two diseases is different from liver cirrhosis caused by the liver parenchyma disease,so it is important to distinguish the three diseases.