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Rare Diseases

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

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Pages 303-313 | Received 05 Sep 2023, Accepted 24 Nov 2023, Published online: 13 Dec 2023
 

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.

Transparency

Declaration of funding

This study was partially supported by the grant from the National Natural Science Foundation of China (no. 81500474) for Dr. Xingshun Qi.

Declaration of financial/other relationships

The authors have no relevant affiliations or financial involvement with any organization or entity with a financial interest in or financial conflict with the subject matter or materials discussed in the manuscript. This includes employment, consultancies, honoraria, stock ownership or options, expert testimony, grants or patents received or pending, or royalties.

Peer reviewers on this manuscript have no relevant financial or other relationships to disclose.

Author contributions

Feifei Lu, Siqi Jia, Huiyuan Lu, Haonan Zhao, Zhe Li, and Xingshun Qi participated in writing the first draft. Yuzheng Zhuge, Fernando Gomes Romeiro, Nahum Mendez-Sanchez, and Xingshun Qi revised the paper and provided critical comments. All authors approved the final version and its submission.

Acknowledgements

None.

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