Zheltova O I, Batorov E V, Starostina N M
Ter Arkh. 2010;82(2):56-9.
The Budd-Chiari syndrome is a rare disease associated with occlusion of the hepatic vein by a tumor or a thrombus. It develops due to progressive narrowing or occlusion of the hepatic veins and may occasionally proceed through the chronic disease within months, rarely years as individual recurrences, with pains, enlarged liver, and mild jaundice. These patients generally have partial hepatic vein occlusion. The paper describes a long (more than 20 years) course of the Budd-Chiari syndrome in which only a special angiographic study could verify the presumptive diagnosis and reveal the cause of evolving liver cirrhosis.
布加综合征是一种罕见疾病,与肿瘤或血栓导致肝静脉闭塞有关。它是由于肝静脉逐渐狭窄或闭塞而发展形成的,偶尔可能在数月内进展为慢性病,个别复发情况很少会持续数年,伴有疼痛、肝脏肿大和轻度黄疸。这些患者通常存在部分肝静脉闭塞。本文描述了一例病程长达20多年的布加综合征病例,其中只有通过特殊的血管造影研究才能证实初步诊断并揭示肝硬化进展的原因。