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肝静脉流出道梗阻:三种相似综合征。

Hepatic venous outflow obstruction: three similar syndromes.

作者信息

Bayraktar Ulas-Darda, Seren Soley, Bayraktar Yusuf

机构信息

Department of Internal Medicine, Interfaith Medical Center, 229 Parkville Ave Apt# 4B, Brooklyn, NY 11230, United States.

出版信息

World J Gastroenterol. 2007 Apr 7;13(13):1912-27. doi: 10.3748/wjg.v13.i13.1912.

Abstract

Our goal is to provide a detailed review of veno-occlusive disease (VOD), Budd-Chiari syndrome (BCS), and congestive hepatopathy (CH), all of which results in hepatic venous outflow obstruction. This is the first article in which all three syndromes have been reviewed, enabling the reader to compare the characteristics of these disorders. The histological findings in VOD, BCS, and CH are almost identical: sinusoidal congestion and cell necrosis mostly in perivenular areas of hepatic acini which eventually leads to bridging fibrosis between adjacent central veins. Tender hepatomegaly with jaundice and ascites is common to all three conditions. However, the clinical presentation depends mostly on the extent and rapidity of the outflow obstruction. Although the etiology and treatment are completely different in VOD, BCS, and CH; the similarities in clinical manifestations and liver histology may suggest a common mechanism of hepatic injury and adaptation in response to increased sinusoidal pressure.

摘要

我们的目标是对静脉闭塞性疾病(VOD)、布加综合征(BCS)和充血性肝病(CH)进行详细综述,所有这些疾病都会导致肝静脉流出道梗阻。这是第一篇对所有这三种综合征进行综述的文章,使读者能够比较这些疾病的特征。VOD、BCS和CH的组织学表现几乎相同:窦状隙充血和细胞坏死主要发生在肝腺泡的小叶中央静脉周围区域,最终导致相邻中央静脉之间的桥接纤维化。三种情况都常见肝肿大伴压痛、黄疸和腹水。然而,临床表现主要取决于流出道梗阻的程度和速度。尽管VOD、BCS和CH的病因和治疗方法完全不同;但临床表现和肝脏组织学的相似性可能提示存在一种共同的肝损伤机制以及对窦状隙压力升高的适应性反应。

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