Søreide Kjetil
Hepatogastroenterology. 2014 Mar-Apr;61(130):388-90.
Budd-Chiari syndrome is an eponym for "hepatic venous outflow tract obstruction". BCS left untreated has a high mortality rate. Diagnosis can be difficult because of the wide spectrum of presentation of the disease and the varying severity of liver damage. The onset of disease may be insidious, with a chronic, asymptomatic course, or also present on other occasions as an acute, life-threatening condition. In rare instances, BCS is associated with hepatocellular carcinoma (HCC), which may be both a cause and a consequence of BCS. Presented here is a case of acute, rapidly fatal severe BCS associated with HCC. The association between BCS and HCC in 'the chicken or the egg' analogy is revisited.
布加综合征是“肝静脉流出道梗阻”的一个专属名称。未经治疗的布加综合征死亡率很高。由于该疾病表现形式多样且肝损伤严重程度各异,诊断可能会很困难。疾病的起病可能隐匿,呈慢性、无症状病程,也可能在其他情况下表现为急性、危及生命的病症。在罕见情况下,布加综合征与肝细胞癌(HCC)相关,肝细胞癌可能既是布加综合征的病因,也是其结果。本文介绍了一例与肝细胞癌相关的急性、迅速致命的严重布加综合征病例。文中重新探讨了布加综合征与肝细胞癌之间类似于“先有鸡还是先有蛋”的关联。