{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,1,22]],"date-time":"2026-01-22T18:39:43Z","timestamp":1769107183730,"version":"3.49.0"},"reference-count":12,"publisher":"Wiley","issue":"7","license":[{"start":{"date-parts":[[2015,12,6]],"date-time":"2015-12-06T00:00:00Z","timestamp":1449360000000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"funder":[{"DOI":"10.13039\/100008746","name":"National Cancer Center","doi-asserted-by":"publisher","award":["NCC\u20101410860"],"award-info":[{"award-number":["NCC\u20101410860"]}],"id":[{"id":"10.13039\/100008746","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Liver International"],"published-print":{"date-parts":[[2016,7]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec><jats:title>Background &amp; Aims<\/jats:title><jats:p>The population\u2010based epidemiology of Budd\u2013Chiari syndrome (<jats:styled-content style=\"fixed-case\">BCS<\/jats:styled-content>), a rare disease of hepatic venous outflow obstruction, is largely unknown. This study aimed to elucidate the nationwide population\u2010based incidence, prevalence, complications, case fatalities and direct medical cost of <jats:styled-content style=\"fixed-case\">BCS<\/jats:styled-content> in South Korea from 2009 to 2013.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>Using two large data sources, the Health Insurance Review and Assessment Service Claims database and Rare Intractable Disease registration program database in Korea, we identified all patients with <jats:styled-content style=\"fixed-case\">BCS<\/jats:styled-content> who were registered under International Classification of Diseases 10 (code I82.0). The age\u2010 and sex\u2010adjusted incidence and prevalence of <jats:styled-content style=\"fixed-case\">BCS<\/jats:styled-content> were calculated with analysis of complications and direct medical costs.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>A total of 424 patients with <jats:styled-content style=\"fixed-case\">BCS<\/jats:styled-content> were identified in 2009\u20132013, with a female\u2010to\u2010male ratio of 1.8 and a median age of 51 years old. The average age\u2010 and sex\u2010adjusted incidence from 2011 to 2013 was 0.87 per million per year, and the average age\u2010 and sex\u2010adjusted prevalence from 2009 to 2013 was 5.29 per million population. Among them, 10.3% accompanied liver cancer and 3.3% underwent liver transplantation. Annual case\u2010fatality rate was 2.8%. Direct medical costs excluding uninsured services for <jats:styled-content style=\"fixed-case\">BCS<\/jats:styled-content> increased by year from 385 720 <jats:styled-content style=\"fixed-case\">USD<\/jats:styled-content> in 2009 to 765 983 <jats:styled-content style=\"fixed-case\">USD<\/jats:styled-content> in 2013.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusions<\/jats:title><jats:p>This is the first population\u2010based study on the epidemiology of <jats:styled-content style=\"fixed-case\">BCS<\/jats:styled-content> in an Asian country, which presented a higher prevalence than in Western studies. It suggests early diagnosis or improved prognosis of <jats:styled-content style=\"fixed-case\">BCS<\/jats:styled-content> in recent years, and clinical features of <jats:styled-content style=\"fixed-case\">BCS<\/jats:styled-content> that differ by geography.<\/jats:p><\/jats:sec>","DOI":"10.1111\/liv.13008","type":"journal-article","created":{"date-parts":[[2015,11,12]],"date-time":"2015-11-12T09:19:25Z","timestamp":1447319965000},"page":"1067-1073","source":"Crossref","is-referenced-by-count":35,"title":["Incidence, prevalence and complications of Budd\u2013Chiari syndrome in South Korea: a nationwide, population\u2010based study"],"prefix":"10.1111","volume":"36","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-8892-7104","authenticated-orcid":false,"given":"Moran","family":"Ki","sequence":"first","affiliation":[{"name":"Department of Cancer Control and Policy Graduate School of Cancer Science and Policy National Cancer Center Goyang Korea"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Hwa Young","family":"Choi","sequence":"additional","affiliation":[{"name":"Department of Cancer Control and Policy Graduate School of Cancer Science and Policy National Cancer Center Goyang Korea"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Kyung\u2010Ah","family":"Kim","sequence":"additional","affiliation":[{"name":"Departments of Internal Medicine Inje University Ilsan Paik Hospital Goyang Korea"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Bo Hyun","family":"Kim","sequence":"additional","affiliation":[{"name":"Liver Cancer Center National Cancer Center Goyang Korea"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Eun Sun","family":"Jang","sequence":"additional","affiliation":[{"name":"Department of Internal Medicine Seoul National University Bundang Hospital College of Medicine Seoul National University Seongnam Korea"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Sook\u2010Hyang","family":"Jeong","sequence":"additional","affiliation":[{"name":"Department of Internal Medicine Seoul National University Bundang Hospital College of Medicine Seoul National University Seongnam Korea"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2015,12,6]]},"reference":[{"key":"e_1_2_8_2_1","doi-asserted-by":"publisher","DOI":"10.1002\/hep.22772"},{"key":"e_1_2_8_3_1","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMra020282"},{"key":"e_1_2_8_4_1","doi-asserted-by":"publisher","DOI":"10.1055\/s-0028-1085094"},{"key":"e_1_2_8_5_1","doi-asserted-by":"publisher","DOI":"10.1093\/oxfordjournals.qjmed.a030135"},{"key":"e_1_2_8_6_1","doi-asserted-by":"publisher","DOI":"10.1016\/0168-8278(95)80252-5"},{"key":"e_1_2_8_7_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1440-1746.1996.tb00056.x"},{"key":"e_1_2_8_8_1","unstructured":"Korean Statistical Information Service.National Health Insurance Statistics.2015. 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