{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,12]],"date-time":"2026-08-12T15:49:02Z","timestamp":1786549742804,"version":"build-2736575974"},"reference-count":41,"publisher":"Wiley","issue":"1","license":[{"start":{"date-parts":[[2026,8,12]],"date-time":"2026-08-12T00:00:00Z","timestamp":1786492800000},"content-version":"vor","delay-in-days":223,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"},{"start":{"date-parts":[[2026,1,1]],"date-time":"2026-01-01T00:00:00Z","timestamp":1767225600000},"content-version":"tdm","delay-in-days":0,"URL":"http:\/\/doi.wiley.com\/10.1002\/tdm_license_1.1"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["International Journal of Hepatology"],"published-print":{"date-parts":[[2026,1]]},"abstract":"<jats:sec>\n                    <jats:title>Background\/Aims<\/jats:title>\n                    <jats:p>Budd\u2013Chiari Syndrome (BCS) is a vascular disease of the liver characterized by venous outflow obstruction most often due to a myeloproliferative neoplasm (MPN). Restoration of hepatic venous outflow and long\u2010term anticoagulation remain the mainstay of treatment. We examined our experiences and treatment outcomes of BCS, including comprehensive testing for underlying MPN.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods<\/jats:title>\n                    <jats:p>A retrospective study was conducted to assess overall and transplant\u2010free survival in patients with primary BCS. All cases of primary BCS that presented to our institution over the past 3 decades were included. Details relating to their hematological work\u2010up and radiological interventions were recorded, as well as their longer term outcomes, including the need for liver transplantation.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>Forty\u2010three primary BCS patients were identified over a 28\u2010year period, with a median follow\u2010up time of 134 months. MPN was detected in 25 (62.5%) of cases who had comprehensive testing. All patients received primary anticoagulation. Seventeen (39.5%) patients underwent primary transjugular intrahepatic portosystemic shunt (TIPS). Twenty (46.5%) received anticoagulation alone as primary therapy, of whom 14 (70%) failed therapy and ultimately needed a rescue TIPS. Four (9.3%) patients underwent liver transplantation. Overall survival was 100% and 92.9% at 1 and 5 years, respectively, with transplant\u2010free survival of 97.7% and 88.1% at 1 and 5 years, respectively.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusion<\/jats:title>\n                    <jats:p>MPN (particularly JAK2V617F\u2010positive) is an important underlying cause of primary BCS. Anticoagulation alone without radiological intervention can lead to disease progression. In this single\u2010center cohort, early TIPS for rapid hepatic decompression appeared safe and was associated with excellent long\u2010term outcomes.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1155\/ijh\/2141081","type":"journal-article","created":{"date-parts":[[2026,8,12]],"date-time":"2026-08-12T14:58:11Z","timestamp":1786546691000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":0,"title":["Early Radiological Intervention in Budd\u2013Chiari Syndrome: Clinical Outcomes From a Single\u2010Center Study"],"prefix":"10.1155","volume":"2026","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-2210-5773","authenticated-orcid":false,"given":"Tai","family":"Ermongkonchai","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0009-0009-0306-3940","authenticated-orcid":false,"given":"Alysia","family":"Bastas","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0009-0005-9840-0612","authenticated-orcid":false,"given":"Katrina","family":"Tan","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Adam","family":"Testro","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2026,8,12]]},"reference":[{"key":"e_1_2_9_1_2","doi-asserted-by":"publisher","DOI":"10.1111\/j.1478-3231.2008.01838.x"},{"key":"e_1_2_9_2_2","doi-asserted-by":"publisher","DOI":"10.1016\/j.clinre.2018.10.014"},{"key":"e_1_2_9_3_2","doi-asserted-by":"publisher","DOI":"10.1002\/hep.22772"},{"key":"e_1_2_9_4_2","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2011-09-376517"},{"key":"e_1_2_9_5_2","doi-asserted-by":"publisher","DOI":"10.1016\/S0140-6736(05)71142-9"},{"key":"e_1_2_9_6_2","doi-asserted-by":"publisher","DOI":"10.1002\/hep.21354"},{"key":"e_1_2_9_7_2","doi-asserted-by":"publisher","DOI":"10.1016\/S0168-8278(12)60004-X"},{"key":"e_1_2_9_8_2","doi-asserted-by":"publisher","DOI":"10.1002\/hep.26306"},{"key":"e_1_2_9_9_2","doi-asserted-by":"publisher","DOI":"10.1002\/hep.31646"},{"key":"e_1_2_9_10_2","doi-asserted-by":"publisher","DOI":"10.1016\/j.jhep.2015.07.040"},{"key":"e_1_2_9_11_2","doi-asserted-by":"publisher","DOI":"10.1111\/imj.13544"},{"key":"e_1_2_9_12_2","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMoa1311347"},{"key":"e_1_2_9_13_2","doi-asserted-by":"publisher","DOI":"10.1053\/jhep.2001.22172"},{"key":"e_1_2_9_14_2","doi-asserted-by":"publisher","DOI":"10.1053\/gast.2003.50016"},{"key":"e_1_2_9_15_2","doi-asserted-by":"publisher","DOI":"10.1007\/s00270-004-0145-y"},{"key":"e_1_2_9_16_2","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-2036.2009.04134.x"},{"key":"e_1_2_9_17_2","doi-asserted-by":"publisher","DOI":"10.1002\/hep.20064"},{"key":"e_1_2_9_18_2","doi-asserted-by":"publisher","DOI":"10.1016\/j.jceh.2018.02.008"},{"key":"e_1_2_9_19_2","doi-asserted-by":"publisher","DOI":"10.1053\/j.gastro.2008.05.051"},{"key":"e_1_2_9_20_2","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-2036.2011.04627.x"},{"key":"e_1_2_9_21_2","doi-asserted-by":"publisher","DOI":"10.1016\/j.thromres.2018.05.007"},{"key":"e_1_2_9_22_2","doi-asserted-by":"publisher","DOI":"10.1111\/bjh.13121"},{"key":"e_1_2_9_23_2","doi-asserted-by":"publisher","DOI":"10.1111\/bjh.13235"},{"key":"e_1_2_9_24_2","doi-asserted-by":"publisher","DOI":"10.1097\/MEG.0000000000000526"},{"key":"e_1_2_9_25_2","doi-asserted-by":"publisher","DOI":"10.1016\/j.jhep.2017.04.021"},{"key":"e_1_2_9_26_2","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2007-11-125328"},{"key":"e_1_2_9_27_2","doi-asserted-by":"publisher","DOI":"10.1002\/lt.23443"},{"key":"e_1_2_9_28_2","doi-asserted-by":"publisher","DOI":"10.1111\/j.1751-553X.2007.00973.x"},{"key":"e_1_2_9_29_2","doi-asserted-by":"publisher","DOI":"10.1002\/hep.510310307"},{"key":"e_1_2_9_30_2","doi-asserted-by":"publisher","DOI":"10.21037\/hbsn-22-584"},{"key":"e_1_2_9_31_2","doi-asserted-by":"publisher","DOI":"10.1007\/s12072-021-10189-4"},{"key":"e_1_2_9_32_2","volume-title":"Budd-Chiari Syndrome BMJ Best Practice","author":"Saoud Abdel Moneim S.","year":"2024"},{"key":"e_1_2_9_33_2","doi-asserted-by":"publisher","DOI":"10.1016\/j.jhep.2019.02.015"},{"key":"e_1_2_9_34_2","doi-asserted-by":"publisher","DOI":"10.1172\/JCI105380"},{"key":"e_1_2_9_35_2","doi-asserted-by":"publisher","DOI":"10.1016\/j.cgh.2022.04.024"},{"key":"e_1_2_9_36_2","doi-asserted-by":"publisher","DOI":"10.1111\/jgh.14843"},{"key":"e_1_2_9_37_2","doi-asserted-by":"publisher","DOI":"10.1111\/liv.13285"},{"key":"e_1_2_9_38_2","doi-asserted-by":"publisher","DOI":"10.1111\/ejh.14363"},{"key":"e_1_2_9_39_2","doi-asserted-by":"publisher","DOI":"10.1016\/j.jhepr.2022.100667"},{"key":"e_1_2_9_40_2","doi-asserted-by":"publisher","DOI":"10.1097\/MPG.0000000000003860"},{"key":"e_1_2_9_41_2","doi-asserted-by":"publisher","DOI":"10.1186\/1471-230X-10-25"}],"container-title":["International Journal of Hepatology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1155\/ijh\/2141081","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/full-xml\/10.1155\/ijh\/2141081","content-type":"application\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1155\/ijh\/2141081","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2026,8,12]],"date-time":"2026-08-12T14:58:12Z","timestamp":1786546692000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1155\/ijh\/2141081"}},"subtitle":[],"editor":[{"given":"Fortofoiu","family":"Mircea-Catalin","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"editor"}]}],"short-title":[],"issued":{"date-parts":[[2026,1]]},"references-count":41,"journal-issue":{"issue":"1","published-print":{"date-parts":[[2026,1]]}},"alternative-id":["10.1155\/ijh\/2141081"],"URL":"https:\/\/doi.org\/10.1155\/ijh\/2141081","archive":["Portico"],"relation":{},"ISSN":["2090-3448","2090-3456"],"issn-type":[{"value":"2090-3448","type":"print"},{"value":"2090-3456","type":"electronic"}],"subject":[],"published":{"date-parts":[[2026,1]]},"assertion":[{"value":"2025-06-19","order":0,"name":"received","label":"Received","group":{"name":"publication_history","label":"Publication History"}},{"value":"2026-07-28","order":2,"name":"accepted","label":"Accepted","group":{"name":"publication_history","label":"Publication History"}},{"value":"2026-08-12","order":3,"name":"published","label":"Published","group":{"name":"publication_history","label":"Publication History"}}],"article-number":"2141081"}}