{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,7,4]],"date-time":"2026-07-04T00:27:18Z","timestamp":1783124838543,"version":"3.54.6"},"reference-count":33,"publisher":"Ovid Technologies (Wolters Kluwer Health)","issue":"2","license":[{"start":{"date-parts":[[2017,1,23]],"date-time":"2017-01-23T00:00:00Z","timestamp":1485129600000},"content-version":"tdm","delay-in-days":0,"URL":"http:\/\/doi.wiley.com\/10.1002\/tdm_license_1"},{"start":{"date-parts":[[2017,1,23]],"date-time":"2017-01-23T00:00:00Z","timestamp":1485129600000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Liver Transpl"],"published-print":{"date-parts":[[2017,2]]},"abstract":"<jats:p>Budd\u2010Chiari syndrome (BCS) is a rare disease resulting from obstruction of the hepatic venous outflow tract that typically presents with abdominal pain, jaundice, and ascites without frank liver failure. However, BCS may also evolve more rapidly to acute liver failure (ALF). In this study, we describe the clinical features, treatment, and outcomes of ALF due to BCS and compare our results with those in the published literature. Twenty of the 2344 patients enrolled in the Acute Liver Failure Study Group (ALFSG) registry since 1998 presented with a clinical diagnosis of BCS. An additional 19 patients of ALF\u2010BCS in the English language literature were reviewed and compared with the ALFSG cases. Most ALF\u2010BCS patients were white (84%) and female (84%) in their fourth decade. A hypercoagulable state was noted in 63% of patients. BCS was diagnosed by Doppler ultrasonography or abdominal computed tomography in all patients. Liver biopsies (n\u2009=\u20096) all had evidence of severe pericentral necrosis. Treatments used included most commonly anticoagulation (71%), but also transjugular intrahepatic portosystemic shunt (TIPS; 37%) and orthotopic liver transplantation (37%). In\u2010hospital mortality was approximately 60%. In conclusion, BCS is a rare cause of ALF and mandates prompt diagnosis and management for successful outcomes. Once the diagnosis is confirmed, prompt anticoagulation is recommended in conjunction with evaluation for malignancy or thrombophilic disorder. Mortality may have improved in recent years with use of TIPS and\/or orthotopic liver transplantation compared with prior published reports. <jats:italic toggle=\"yes\">Liver Transplantation 23 135\u2013142 2017 AASLD<\/jats:italic>\n                        <\/jats:p>","DOI":"10.1002\/lt.24643","type":"journal-article","created":{"date-parts":[[2016,9,22]],"date-time":"2016-09-22T19:19:42Z","timestamp":1474571982000},"page":"135-142","source":"Crossref","is-referenced-by-count":65,"title":["Budd\u2010chiari syndrome causing acute liver failure: A multicenter case series"],"prefix":"10.1097","volume":"23","author":[{"given":"Justin","family":"Parekh","sequence":"first","affiliation":[{"name":"Department of Surgery,University of Texas Southwestern Medical Center at Dallas,Dallas,TX"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Vlad M.","family":"Matei","sequence":"additional","affiliation":[{"name":"Department of Ophthalmology,University of Texas Southwestern Medical Center at Dallas,Dallas,TX"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Alejandro","family":"Canas\u2010Coto","sequence":"additional","affiliation":[{"name":"Gastroenterology Division HSJD,University of Costa Rica,San Pedro Montes de Oca,Costa Rica"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Daniel","family":"Friedman","sequence":"additional","affiliation":[{"name":"Texas Digestive Disease Consultants,Keller,TX"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"William M.","family":"Lee","sequence":"additional","affiliation":[{"name":"Department of Digestive and Liver Diseases Division,University of Texas Southwestern Medical Center at Dallas,Dallas,TX"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"name":"the Acute Liver Failure Study 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