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原发性布加综合征与窦状隙阻塞综合征:综述。

Primary Budd-Chiari syndrome versus sinusoidal obstruction syndrome: a review.

机构信息

College of Medicine and Biological Information Engineering, Northeastern University, Shenyang, China.

Liver Cirrhosis Study Group, Department of Gastroenterology, General Hospital of Northern Theater Command, Shenyang, China.

出版信息

Curr Med Res Opin. 2024 Feb;40(2):303-313. doi: 10.1080/03007995.2023.2288909. Epub 2024 Jan 24.

Abstract

Budd-Chiari syndrome (BCS) and sinusoidal obstruction syndrome (SOS) are two major vascular disorders of the liver, of which both can cause portal hypertension related complications, but their locations of obstruction are different. BCS refers to the obstruction from the hepatic vein to the junction between the inferior vena cava and right atrium, which is the major etiology of post-sinusoidal portal hypertension; by comparison, SOS is characterized as the obstruction at the level of hepatic sinusoids and terminal venulae, which is a cause of sinusoidal portal hypertension. Both of them can cause hepatic congestion with life-threatening complications, especially acute liver failure and chronic portal hypertension, and share some similar features in terms of imaging and clinical presentations, but they have heterogeneous risk factors, management strategy, and prognosis. Herein, this paper reviews the current evidence and then summarizes the difference between primary BCS and SOS in terms of risk factors, clinical features, diagnosis, and treatment.

摘要

布加综合征(BCS)和窦状隙阻塞综合征(SOS)是肝脏的两种主要血管疾病,两者均可导致门静脉高压相关并发症,但阻塞部位不同。BCS 是指肝静脉至下腔静脉和右心房交界处的阻塞,是窦后性门静脉高压的主要病因;相比之下,SOS 的特征是肝窦和终末小静脉水平的阻塞,是窦性门静脉高压的一个原因。两者均可导致肝淤血,危及生命,尤其是急性肝衰竭和慢性门静脉高压,并在影像学和临床表现方面具有一些相似特征,但它们具有不同的危险因素、管理策略和预后。本文回顾了目前的证据,然后总结了原发性 BCS 和 SOS 在危险因素、临床特征、诊断和治疗方面的差异。

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