Current knowledge in pathophysiology and management of Budd-Chiari syndrome and non-cirrhotic non-tumoral splanchnic vein thrombosis

被引:78
作者
Hernandez-Gea, Virginia [1 ]
De Gottardi, Andrea [2 ,3 ]
Leebeek, Frank W. G. [4 ]
Rautou, Pierre-Emmanuel [5 ,6 ]
Salem, Riad [7 ]
Carlos Garcia-Pagan, Juan [1 ]
机构
[1] Univ Barcelona, Barcelona Hepat Hemodynam C Lab, Liver Unit, Hosp Clin Barcelona,IDIBAPS,CIBERehd,European Ref, Barcelona, Spain
[2] Univ Bern, Hepatol, Univ Clin Visceral Med & Surg, Inselspital, Bern, Switzerland
[3] Univ Bern, Dept Biomed Res, Bern, Switzerland
[4] Erasmus Univ, Med Ctr, Dept Haematol, Rotterdam, Netherlands
[5] DHU Unity, AP HP, Serv Hepatol,Hop Beaujon, Ctr Reference Malad Vasc Foie,Rile Malad Appareil, Clichy, France
[6] PARCC, Paris Cardiovasc Res Ctr, UMR 970, INSERM, Paris, France
[7] Northwestern Univ, Dept Radiol, Sect Intervent Radiol, Chicago, IL 60611 USA
关键词
Portal vein thrombosis; Portal hypertension; Thrombophilia; Coagulopathy; Transplant; TIPS; Angioplasty; INTRAHEPATIC PORTOSYSTEMIC SHUNT; PAROXYSMAL-NOCTURNAL HEMOGLOBINURIA; TERM-FOLLOW-UP; TRANSCATHETER THROMBOLYTIC THERAPY; MESENTERIC VENOUS THROMBOSIS; ACTING ORAL ANTICOAGULANTS; V-LEIDEN MUTATION; PORTAL-VEIN; LIVER-TRANSPLANTATION; RISK-FACTORS;
D O I
10.1016/j.jhep.2019.02.015
中图分类号
R57 [消化系及腹部疾病];
学科分类号
摘要
Budd-Chiari syndrome and non-cirrhotic non-tumoral portal vein thrombosis are 2 rare disorders, with several similarities that are categorized under the term splanchnic vein thrombosis. Both disorders are frequently associated with an underlying prothrombotic disorder. They can cause severe portal hypertension and usually affect young patients, negatively influencing life expectancy when the diagnosis and treatment are not performed at an early stage. Yet, they have specific features that require individual consideration. The current review will focus on the available knowledge on pathophysiology, diagnosis and management of both entities. (C) 2019 European Association for the Study of the Liver. Published by Elsevier B.V. All rights reserved.
引用
收藏
页码:175 / 199
页数:25
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