Budd-Chiari syndrome and liver transplantation

DOI 被引用文献2件 参考文献84件
  • Akamatsu Nobuhisa
    Artificial Organ and Transplantation Division, Department of Surgery, Graduate School of Medicine, The University of Tokyo
  • Sugawara Yasuhiko
    Artificial Organ and Transplantation Division, Department of Surgery, Graduate School of Medicine, The University of Tokyo
  • Kokudo Norihiro
    Artificial Organ and Transplantation Division, Department of Surgery, Graduate School of Medicine, The University of Tokyo

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Budd-Chiari syndrome involves obstruction of hepatic venous outflow tracts at various levels from small hepatic veins to the inferior vena cava and is the result of thrombosis or its fibrous sequelae. There is a conspicuous difference in its etiology in the West and the East. Myeloproliferative disease predominates in the West and obstruction of the vena cava predominates in the East. The clinical presentation and clinical manifestations are so varied that it should be suspected in any patient with acute or chronic liver dysfunction. It should be treated with step-wise management. First-line therapy should be anticoagulation with medical treatment of the underlying illness, and interventional revascularization and TIPS are indicated in the event of a lack of response to medical therapy. Liver transplantation may be indicated as a rescue treatment or for fulminant cases with promising results. This step-by-step strategy has achieved a 5-year transplant-free survival rate of 70% and a 5-year overall survival rate of 90%. Living donor liver transplantation can also be used for patients with Budd-Chiari syndrome if deceased donor livers are scarce, but it requires a difficult procedure particularly with regard to venous outflow reconstruction.

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  • Intractable & Rare Diseases Research

    Intractable & Rare Diseases Research 4 (1), 24-32, 2015

    特定非営利活動法人 バイオ＆ソーシャル・サイエンス推進国際研究交流会

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