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首页> 美国卫生研究院文献>The Korean Journal of Internal Medicine >Rapidly Progressing Budd-Chiari Syndrome Complicated by Hepatocellular Carcinoma
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Rapidly Progressing Budd-Chiari Syndrome Complicated by Hepatocellular Carcinoma

机译：迅速发展的布加氏综合征并发肝细胞癌

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摘要

Budd-Chiari syndrome (BCS) is a disorder caused by occlusion of the hepatic vein or inferior vena cava. The clinical presentation include abdominal pain, hepatomegaly, ascites, leg edema, collateral venous dilatation of the body trunk, and portal hypertension. In addition, BCS can cause hepatocellular carcinoma (HCC) in some patients, although its pathogenesis is not yet completely understood. The average reported time lag from diagnosis of BCS to full-blown HCC ranges from several years to several decades. Hepatic carcinogenesis in patients with BCS perhaps reflects a prolonged and persistent liver injury in that it occurs in the primary inferior vena cava obstruction rather than the primary hepatic vein thrombosis. Among patients with BCS, membranous obstruction of the vena cava (MOVC) usually presents an insidious and chronic illness, whereas primary hepatic vein thrombosis presents an acute or subacute illness. We experienced a case pf a patient with BCS, which progressed rapidly that HCC developed only nine months after the diagnosis of BCS. The factors causing this rapid progression are still unclear and remain to be investigated.
机译：Budd-Chiari综合征（BCS）是由肝静脉或下腔静脉闭塞引起的疾病。临床表现包括腹痛，肝肿大，腹水，腿水肿，躯干侧支静脉扩张和门静脉高压症。此外，BCS可能在某些患者中引起肝细胞癌（HCC），尽管其发病机理尚未完全明了。从诊断出BCS到成熟的HCC的平均报告时间间隔为数年到数十年不等。 BCS患者的肝癌发生可能反映了长期持续的肝损伤，因为它发生在原发性下腔静脉阻塞而不是原发性肝静脉血栓形成中。在BCS患者中，腔静脉膜阻塞（MOVC）通常表现为隐匿性和慢性疾病，而原发性肝静脉血栓形成则表现为急性或亚急性疾病。我们遇到了一起BCS患者的病例，该病例进展迅速，肝癌在诊断BCS后仅9个月就发展了。导致这种快速进展的因素仍不清楚，有待研究。

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