Epidemiology
Budd-Chiari syndrome (BCS) is a rare but potentially life-threatening condition affecting 1/100,000 of the general population worldwide.[13] BCS has a reported incidence between 0.168 and 4.09 cases per million people, and a prevalence of 2.40 to 33.10 per million.[14]
It is more common in women in their third or fourth decade of life.[15] 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.[16][17] One population-based study in the US showed an increase of 4.41% (95% CI 4.23% to 4.59%, P = 0.0001) in the annual percentage of hospitalisation from BCS between 1998 and 2017.[18]
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