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Juan Carlos Garcia-Pagán, M.D., Ph.D., and Dominique-Charles Valla, M.D.
Primary Budd–Chiari syndrome is a spontaneously fatal disease that results from obstruction of the hepatic venous outflow tract due to thrombosis or primary disease of the venous wall. The condition is extremely rare, with a prevalence of approximately 1 case per 1 million persons per year. The article emphasizes the importance of evaluating underlying prothrombotic disorders, as they are found in a significant percentage of patients. Recent diagnostic advancements have improved the understanding and management of this condition.
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