What is the prevalence of Budd-Chiari syndrome?

Updated: Oct 10, 2018
  • Author: Praveen K Roy, MD, AGAF; Chief Editor: BS Anand, MD  more...
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Budd-Chiari syndrome is extremely rare, and the incidence is not well reported in the literature, although a study by Rajani et al found an incidence of about 1 case per million population per year in Sweden. Congenital membranous forms of Budd-Chiari syndrome are the most common cause of the disease worldwide, particularly in Asia. [13]

A retrospective (2009-2013) nation-wide, population-based study in South Korea found a total of 424 patients with Budd-Chiari syndrome, with an average age- and sex-adjusted prevalence of 5.29 per million population. [14] The female-to-male ratio was 1.8, the median age was 51 years, and the annual case-fatality rate was 2.8%. [14]

The prevalence of Budd-Chiari syndrome in France appears to 4.04 per million inhabitants, with myeloproliferative neoplasms (48%), use of oral contraceptives (35%), and factor V Leiden (16%) the highest risk factors. [52]

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