BCS is a rare but potentially life-threatening condition affecting 1/100,000 of the general population worldwide. The estimated annual incidence in Western countries is between 0.005% and 0.061%. It is more common in women in their third or fourth decade of life. 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. 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.
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