Epidemiology

BCS is a rare but potentially life-threatening condition affecting 1/100,000 of the general population worldwide.[12] The estimated annual incidence in Western countries is between 0.005% and 0.061%.[13][14][15] It is more common in women in their third or fourth decade of life.[16] Hepatic venous obstruction is more common in the West, while primary membranous obstruction of the inferior vena cava is the most common cause in South Africa and Asia.[17] BCS is a rare disease in population groups with a high standard of living. By contrast, it is a leading cause of liver-related hospital admission in populations with a lower standard of living.[1]

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