Budd-Chiari syndrome epidemiology and demographics

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief:

Overview

It occurs in 1 out of 100,000 individuals and is more common in females. Some 10-20% also have obstruction of the portal vein.

Epidemiology and Demographics

Prevalence

• In 2009-2013, the prevalence of Budd-Chiari syndrome was estimated to be 424 patients cases per 5.29 per million (100,000) individuals in South Korea.

Incidence

• Budd-Chiari syndrome is a rare disease.
• The incidence of Budd-Chiari syndrome was estimated to be about 1 case per million population per year 100,000 individuals in Sweden.

Case Fatality Rate

• The annual case fatality rate of Budd-Chiari syndrome is approximately 2.8%.

Age

• Budd-Chiari syndrome commonly presents in the third or fourth decade of life.
• The median age at diagnosis is 51 years.
• Budd-Chiari syndrome is rare in children.

Gender

• Females are more commonly affected with Budd-Chiari syndrome than males. The female-to-male ratio is approximately 1.8 to 1.

Developed Countries

• Budd-Chiari syndrome in the United States is predominantly seen in women and is commonly associated with hematologic disorders.

Developing Countries

• Congenital membranous forms is the most common cause of Budd-Chiari syndrome worldwide and particularly in Asia, is due to.