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Budd-Chiari Syndrome/Hepatic Venous Outflow Tract Obstruction: East vs West

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Portal Hypertension VI

Abstract

Features of Budd-Chiari syndrome/hepatic venous outflow tract obstruction [BCS/HVOTO] have been contrasted according to world area. Western cases would be very rare and characterized by hepatic vein thrombosis related to underlying prothrombotic conditions, whereas Eastern cases would be relatively common and characterized by inferior vena cava [IVC] involvement in the absence of prothrombotic conditions. Available data now question this view. Convincing evidence for a different incidence in the West and in the East is lacking. Epidemiological data are needed for China and India. Whatever the area, virtually all cases of BCS/HVOTO appear to be caused by hepatic venous obstruction with or without involvement of a variable part of IVC. Progress in skill and availability of noninvasive imaging techniques has probably been instrumental in the change of paradigm. India, Pakistan, Middle East, Mediterranean area, and Europe appear to share many characteristics with respect to level of obstruction and individual causal factors. In China however, associated IVC involvement might be more common. Furthermore, the major causal factor in Europe, JAK2-V617F-positive myeloproliferative neoplasms, accounts for relatively low proportion of cases in China. There is a strong suggestion that a factor related to a low socioeconomic status and rural living is causing BSC/HVOTO in Nepal, probably in India and in China as well. Preliminary data indicate that the stepwise approach to treatment that has been implemented in Europe appears to apply to Asian patients and result in similar outcomes. Hepatocellular carcinoma complicating BCS/HVOTO is equally challenging the East and the West.

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  1. Service d’hépatologie, DHU UNITY, APHP Hôpital Beaujon, Clichy la Garenne, Clichy, France

    Dominique-Charles Valla MD

  2. Centre de recherche de l’inflammation, UMR 1149, Université Paris Diderot and Inserm, Paris, France

    Dominique-Charles Valla MD

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  1. Gastroenterology Unit, Luigi Sacco University Hospital, Milan, Italy

    Roberto de Franchis

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Valla, DC. (2016). Budd-Chiari Syndrome/Hepatic Venous Outflow Tract Obstruction: East vs West. In: de Franchis, R. (eds) Portal Hypertension VI. Springer, Cham. https://doi.org/10.1007/978-3-319-23018-4_39

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