BCS 是一种罕见但可能危及生命的疾病,在全球总人口中的发生率为 1/100,000。[12]Valla DC. The diagnosis and management of the Budd-Chiari syndrome: consensus and controversies. Hepatology. 2003;38:793-803.http://www.ncbi.nlm.nih.gov/pubmed/14512865?tool=bestpractice.com在西方国家,估计年发生率在 0.005% 至 0.061% 之间。[13]Parker RG. Occlusion of the hepatic veins in man. Medicine (Baltimore). 1959;38:369-402.http://www.ncbi.nlm.nih.gov/pubmed/14430507?tool=bestpractice.com[14]Armstrong CD, Carnes WH. Obstruction of the hepatic veins (Chiari's disease): report of five cases. Am J Med Sci. 1944;208:470-478.[15]Sequeira FW, Weber TR, Smith WL, et al. Budd-Chiari syndrome caused by hepatic torsion. Am J Roentgenol. 1981;137:393-394.http://www.ajronline.org/doi/pdf/10.2214/ajr.137.2.393http://www.ncbi.nlm.nih.gov/pubmed/6789650?tool=bestpractice.com在 20 至 29 岁或 30 至 39 岁女性中更为常见。[16]Mahmoud AE, Mendoza A, Meshikhes AN, et al. Clinical spectrum, investigations and treatment of Budd-Chiari syndrome. QJM. 1996;89:37-43.http://qjmed.oxfordjournals.org/content/89/1/37.full.pdf+htmlhttp://www.ncbi.nlm.nih.gov/pubmed/8730341?tool=bestpractice.com肝静脉梗阻在西方更为常见,而下腔静脉 (IVC) 原发性膜性梗阻在南非和亚洲是最常见病因。[17]Okuda K. Inferior vena cava thrombosis at its hepatic portion (obliterative hepatocavopathy). Semin Liver Dis. 2002;22:15-26.http://www.ncbi.nlm.nih.gov/pubmed/11928076?tool=bestpractice.comBCS 在具有高生活标准的人群中是一种罕见疾病。相比之下,在生活标准较低的人群中是因肝脏相关入院病例的主要病因。[1]DeLeve LD, Valla DC, Garcia-Tsao G; American Association for the Study Liver Diseases. Vascular disorders of the liver. Hepatology. 2009;49:1729-1764.http://onlinelibrary.wiley.com/doi/10.1002/hep.22772/fullhttp://www.ncbi.nlm.nih.gov/pubmed/19399912?tool=bestpractice.com