{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,24]],"date-time":"2026-09-24T11:24:59Z","timestamp":1790249099542,"version":"4.1.0"},"reference-count":53,"publisher":"Elsevier BV","issue":"4","license":[{"start":{"date-parts":[[2009,10,1]],"date-time":"2009-10-01T00:00:00Z","timestamp":1254355200000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.elsevier.com\/tdm\/userlicense\/1.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Journal of Hepatology"],"published-print":{"date-parts":[[2009,10]]},"DOI":"10.1016\/j.jhep.2009.06.019","type":"journal-article","created":{"date-parts":[[2009,7,24]],"date-time":"2009-07-24T05:00:06Z","timestamp":1248411606000},"page":"696-706","source":"Crossref","is-referenced-by-count":98,"title":["Paroxysmal nocturnal hemoglobinuria in Budd-Chiari Syndrome: Findings from a cohort study"],"prefix":"10.1016","volume":"51","author":[{"given":"Jildou","family":"Hoekstra","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Frank W.G.","family":"Leebeek","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Aurelie","family":"Plessier","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sebastian","family":"Raffa","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sarwa Darwish","family":"Murad","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"J\u00f6rg","family":"Heller","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Antoine","family":"Hadengue","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Carine","family":"Chagneau","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Elwyn","family":"Elias","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Massimo","family":"Primignani","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Juan-Carlos","family":"Garcia-Pagan","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Dominique C.","family":"Valla","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Harry L.A.","family":"Janssen","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"78","reference":[{"key":"10.1016\/j.jhep.2009.06.019_bib1","doi-asserted-by":"crossref","first-page":"364","DOI":"10.1016\/S0168-8278(02)00434-8","article-title":"Budd-Chiari syndrome: a review by an expert panel","volume":"38","author":"Janssen","year":"2003","journal-title":"J Hepatol"},{"key":"10.1016\/j.jhep.2009.06.019_bib2","doi-asserted-by":"crossref","first-page":"5","DOI":"10.1055\/s-2002-23202","article-title":"Hepatic vein thrombosis (Budd-Chiari syndrome)","volume":"22","author":"Valla","year":"2002","journal-title":"Semin Liver Dis"},{"key":"10.1016\/j.jhep.2009.06.019_bib3","doi-asserted-by":"crossref","first-page":"264","DOI":"10.1136\/gut.48.2.264","article-title":"Factor V Leiden related Budd-Chiari syndrome","volume":"48","author":"Deltenre","year":"2001","journal-title":"Gut"},{"key":"10.1016\/j.jhep.2009.06.019_bib4","first-page":"2364","article-title":"Factor V Leiden mutation, prothrombin gene mutation, and deficiencies in coagulation inhibitors associated with Budd-Chiari syndrome and portal vein thrombosis: results of a case\u2013control study","volume":"96","author":"Janssen","year":"2000","journal-title":"Blood"},{"key":"10.1016\/j.jhep.2009.06.019_bib5","doi-asserted-by":"crossref","first-page":"587","DOI":"10.1002\/hep.510310307","article-title":"Cause of portal or hepatic venous thrombosis in adults: the role of multiple concurrent factors","volume":"31","author":"Denninger","year":"2000","journal-title":"Hepatology"},{"key":"10.1016\/j.jhep.2009.06.019_bib6","first-page":"1712","article-title":"Myeloproliferative disease in the pathogenesis and survival of Budd-Chiari syndrome","volume":"91","author":"Smalberg","year":"2006","journal-title":"Haematologica"},{"key":"10.1016\/j.jhep.2009.06.019_bib7","doi-asserted-by":"crossref","first-page":"329","DOI":"10.7326\/0003-4819-103-3-329","article-title":"Primary myeloproliferative disorder and hepatic vein thrombosis. A prospective study of erythroid colony formation in vitro in 20 patients with Budd-Chiari syndrome","volume":"103","author":"Valla","year":"1985","journal-title":"Ann Intern Med"},{"key":"10.1016\/j.jhep.2009.06.019_bib8","doi-asserted-by":"crossref","first-page":"1253","DOI":"10.1056\/NEJM199511093331904","article-title":"Natural history of paroxysmal nocturnal hemoglobinuria","volume":"333","author":"Hillmen","year":"1995","journal-title":"N Engl J Med"},{"key":"10.1016\/j.jhep.2009.06.019_bib9","doi-asserted-by":"crossref","first-page":"573","DOI":"10.1016\/S0140-6736(95)12360-1","article-title":"Paroxysmal nocturnal haemoglobinuria: long-term follow-up and prognostic factors. French Society of Haematology","volume":"348","author":"Socie","year":"1996","journal-title":"Lancet"},{"key":"10.1016\/j.jhep.2009.06.019_bib10","doi-asserted-by":"crossref","first-page":"249","DOI":"10.1056\/NEJM199401273300404","article-title":"Abnormalities of PIG-A transcripts in granulocytes from patients with paroxysmal nocturnal hemoglobinuria","volume":"330","author":"Miyata","year":"1994","journal-title":"N Engl J Med"},{"key":"10.1016\/j.jhep.2009.06.019_bib11","doi-asserted-by":"crossref","first-page":"703","DOI":"10.1016\/0092-8674(93)90250-T","article-title":"Deficiency of the GPI anchor caused by a somatic mutation of the PIG-A gene in paroxysmal nocturnal hemoglobinuria","volume":"73","author":"Takeda","year":"1993","journal-title":"Cell"},{"key":"10.1016\/j.jhep.2009.06.019_bib12","doi-asserted-by":"crossref","first-page":"5272","DOI":"10.1073\/pnas.90.11.5272","article-title":"Specific defect in N-acetylglucosamine incorporation in the biosynthesis of the glycosylphosphatidylinositol anchor in cloned cell lines from patients with paroxysmal nocturnal hemoglobinuria","volume":"90","author":"Hillmen","year":"1993","journal-title":"Proc Natl Acad Sci USA"},{"key":"10.1016\/j.jhep.2009.06.019_bib13","doi-asserted-by":"crossref","first-page":"517","DOI":"10.1084\/jem.177.2.517","article-title":"Deficient biosynthesis of N-acetylglucosaminyl-phosphatidylinositol, the first intermediate of glycosyl phosphatidylinositol anchor biosynthesis, in cell lines established from patients with paroxysmal nocturnal hemoglobinuria","volume":"177","author":"Takahashi","year":"1993","journal-title":"J Exp Med"},{"key":"10.1016\/j.jhep.2009.06.019_bib14","doi-asserted-by":"crossref","first-page":"1184","DOI":"10.1056\/NEJM199010253231707","article-title":"Inherited complete deficiency of 20-kilodalton homologous restriction factor (CD59) as a cause of paroxysmal nocturnal hemoglobinuria","volume":"323","author":"Yamashina","year":"1990","journal-title":"N Engl J Med"},{"key":"10.1016\/j.jhep.2009.06.019_bib15","doi-asserted-by":"crossref","first-page":"133","DOI":"10.1111\/j.1365-2141.2004.04992.x","article-title":"Natural history of paroxysmal nocturnal haemoglobinuria using modern diagnostic assays","volume":"126","author":"Moyo","year":"2004","journal-title":"Br J Haematol"},{"key":"10.1016\/j.jhep.2009.06.019_bib16","first-page":"103","article-title":"Paroxysmal nocturnal hemoglobinuria and the risk of venous thrombosis: review and recommendations for management of the pregnant and nonpregnant patient","volume":"30","author":"Ray","year":"2000","journal-title":"Haemostasis"},{"key":"10.1016\/j.jhep.2009.06.019_bib17","doi-asserted-by":"crossref","first-page":"642","DOI":"10.1111\/j.1538-7836.2007.02379.x","article-title":"Thrombosis in paroxysmal nocturnal hemoglobinuria: sites, risks, outcome. An overview","volume":"5","author":"Ziakas","year":"2007","journal-title":"J Thromb Haemost"},{"key":"10.1016\/j.jhep.2009.06.019_bib18","doi-asserted-by":"crossref","first-page":"S4","DOI":"10.1016\/S0168-8278(07)61601-8","article-title":"Prospective follow-up study on 163 patients with Budd-Chiari syndrome: results from the European network for vascular disorders of the liver (EN-VIE)","volume":"46","author":"Murad","year":"2007","journal-title":"J Hepatol"},{"key":"10.1016\/j.jhep.2009.06.019_bib19","doi-asserted-by":"crossref","first-page":"569","DOI":"10.1016\/0016-5085(87)90920-6","article-title":"Hepatic vein thrombosis in paroxysmal nocturnal hemoglobinuria. A spectrum from asymptomatic occlusion of hepatic venules to fatal Budd-Chiari syndrome","volume":"93","author":"Valla","year":"1987","journal-title":"Gastroenterology"},{"key":"10.1016\/j.jhep.2009.06.019_bib20","doi-asserted-by":"crossref","first-page":"1042","DOI":"10.1016\/0016-5085(94)90765-X","article-title":"The changing scene of hepatic vein thrombosis: recognition of asymptomatic cases","volume":"106","author":"Hadengue","year":"1994","journal-title":"Gastroenterology"},{"key":"10.1016\/j.jhep.2009.06.019_bib21","doi-asserted-by":"crossref","first-page":"500","DOI":"10.1002\/hep.20064","article-title":"Determinants of survival and the effect of portosystemic shunting in patients with Budd-Chiari syndrome","volume":"39","author":"Murad","year":"2004","journal-title":"Hepatology"},{"key":"10.1016\/j.jhep.2009.06.019_bib22","doi-asserted-by":"crossref","first-page":"1308","DOI":"10.1002\/hep.21354","article-title":"Aiming at minimal invasiveness as a therapeutic strategy for Budd-Chiari syndrome","volume":"44","author":"Plessier","year":"2006","journal-title":"Hepatology"},{"key":"10.1016\/j.jhep.2009.06.019_bib23","doi-asserted-by":"crossref","first-page":"193","DOI":"10.1097\/01.md.0000126763.68170.46","article-title":"Clinical course and flow cytometric analysis of paroxysmal nocturnal hemoglobinuria in the United States and Japan","volume":"83","author":"Nishimura","year":"2004","journal-title":"Medicine (Baltimore)"},{"key":"10.1016\/j.jhep.2009.06.019_bib24","doi-asserted-by":"crossref","first-page":"3587","DOI":"10.1182\/blood-2003-01-0009","article-title":"Primary prophylaxis with warfarin prevents thrombosis in paroxysmal nocturnal hemoglobinuria (PNH)","volume":"102","author":"Hall","year":"2003","journal-title":"Blood"},{"key":"10.1016\/j.jhep.2009.06.019_bib25","doi-asserted-by":"crossref","first-page":"392","DOI":"10.1046\/j.1365-2141.1999.01195.x","article-title":"Bone marrow transplants for paroxysmal nocturnal haemoglobinuria","volume":"104","author":"Saso","year":"1999","journal-title":"Br J Haematol"},{"key":"10.1016\/j.jhep.2009.06.019_bib26","doi-asserted-by":"crossref","first-page":"1840","DOI":"10.1182\/blood-2007-06-094136","article-title":"Multicenter phase 3 study of the complement inhibitor eculizumab for the treatment of patients with paroxysmal nocturnal hemoglobinuria","volume":"111","author":"Brodsky","year":"2008","journal-title":"Blood"},{"key":"10.1016\/j.jhep.2009.06.019_bib27","doi-asserted-by":"crossref","first-page":"1233","DOI":"10.1056\/NEJMoa061648","article-title":"The complement inhibitor eculizumab in paroxysmal nocturnal hemoglobinuria","volume":"355","author":"Hillmen","year":"2006","journal-title":"N Engl J Med"},{"key":"10.1016\/j.jhep.2009.06.019_bib28","doi-asserted-by":"crossref","first-page":"4123","DOI":"10.1182\/blood-2007-06-095646","article-title":"Effect of the complement inhibitor eculizumab on thromboembolism in patients with paroxysmal nocturnal hemoglobinuria","volume":"110","author":"Hillmen","year":"2007","journal-title":"Blood"},{"key":"10.1016\/j.jhep.2009.06.019_bib29","doi-asserted-by":"crossref","first-page":"767","DOI":"10.1111\/j.1365-2036.2006.02820.x","article-title":"Transjugular intrahepatic portosystemic shunt for portal vein thrombosis with and without cavernous transformation","volume":"23","author":"Senzolo","year":"2006","journal-title":"Aliment Pharmacol Ther"},{"key":"10.1016\/j.jhep.2009.06.019_bib30","doi-asserted-by":"crossref","first-page":"785","DOI":"10.1007\/s00270-005-0090-4","article-title":"Transjugular intrahepatic portosystemic shunt placement in patients with cirrhosis and concomitant portal vein thrombosis","volume":"29","author":"Van Ha","year":"2006","journal-title":"Cardiovasc Intervent Radiol"},{"key":"10.1016\/j.jhep.2009.06.019_bib31","doi-asserted-by":"crossref","first-page":"890","DOI":"10.1111\/j.1432-2277.2003.tb00260.x","article-title":"Recurrence of Budd-Chiari syndrome after liver transplantation in paroxysmal nocturnal hemoglobinuria","volume":"16","author":"Bahr","year":"2003","journal-title":"Transpl Int"},{"key":"10.1016\/j.jhep.2009.06.019_bib32","first-page":"867","article-title":"Recurrence of the Budd-Chiari syndrome after orthotopic liver transplantation","volume":"46","author":"Ruckert","year":"1999","journal-title":"Hepatogastroenterology"},{"key":"10.1016\/j.jhep.2009.06.019_bib33","doi-asserted-by":"crossref","first-page":"354","DOI":"10.1007\/BF00335976","article-title":"Liver transplantation for PNH with Budd-Chiari syndrome. A case report","volume":"6","author":"Schattenfroh","year":"1993","journal-title":"Transpl Int"},{"key":"10.1016\/j.jhep.2009.06.019_bib34","doi-asserted-by":"crossref","first-page":"421","DOI":"10.1016\/S1051-0443(92)72056-9","article-title":"Budd-Chiari syndrome with long segmental inferior vena cava obstruction: treatment with thrombolysis, angioplasty, and intravascular stents","volume":"3","author":"Ishiguchi","year":"1992","journal-title":"J Vasc Interv Radiol"},{"key":"10.1016\/j.jhep.2009.06.019_bib35","first-page":"1188","article-title":"Hepatic and portal veins thrombosis in a pregnant woman with paroxysmal-nocturnal hemoglobinuria (Pnh) and severe thrombocytopenia \u2013 efficacy of tissue plasminogen-activator in the treatment of the Budd-Chiari syndrome and successful outcome of pregnancy","volume":"65","author":"Destefano","year":"1991","journal-title":"Thromb Haemost"},{"key":"10.1016\/j.jhep.2009.06.019_bib36","first-page":"109","article-title":"Recombinant tissue-plasminogen activator for acute Budd-Chiari syndrome secondary to paroxysmal nocturnal hemoglobinuria","volume":"92","author":"Kwan","year":"1992","journal-title":"NY State J Med"},{"key":"10.1016\/j.jhep.2009.06.019_bib37","doi-asserted-by":"crossref","first-page":"505","DOI":"10.1002\/ajh.21141","article-title":"Lysis and thrombosis: manifestation of the same disease","volume":"83","author":"Abou Antoun","year":"2008","journal-title":"Am J Hematol"},{"key":"10.1016\/j.jhep.2009.06.019_bib38","doi-asserted-by":"crossref","first-page":"211","DOI":"10.1016\/0028-2243(94)90121-X","article-title":"Pregnancy and paroxysmal nocturnal hemoglobinuria","volume":"53","author":"Bais","year":"1994","journal-title":"Eur J Obstet Gynecol Reprod Biol"},{"key":"10.1016\/j.jhep.2009.06.019_bib39","doi-asserted-by":"crossref","first-page":"1067","DOI":"10.1046\/j.1600-0412.2003.00337.x","article-title":"Paroxysmal nocturnal hemoglobinuria in pregnancy","volume":"82","author":"Bjorge","year":"2003","journal-title":"Acta Obstet Gynecol Scand"},{"key":"10.1016\/j.jhep.2009.06.019_bib40","doi-asserted-by":"crossref","first-page":"1532","DOI":"10.1002\/lt.21317","article-title":"Use of splenic artery embolization to relieve tense ascites following liver transplantation in a patient with paroxysmal nocturnal hemoglobinuria","volume":"13","author":"Chang","year":"2007","journal-title":"Liver Transpl"},{"key":"10.1016\/j.jhep.2009.06.019_bib41","doi-asserted-by":"crossref","first-page":"124","DOI":"10.1046\/j.1365-2141.2002.03543.x","article-title":"Stem cell transplantation for paroxysmal nocturnal haemoglobinuria in childhood","volume":"118","author":"Flotho","year":"2002","journal-title":"Br J Haematol"},{"key":"10.1016\/j.jhep.2009.06.019_bib42","doi-asserted-by":"crossref","first-page":"1157","DOI":"10.1002\/hep.510270434","article-title":"Budd-Chiari syndrome in patients with hematological disease: a therapeutic challenge","volume":"27","author":"Ganguli","year":"1998","journal-title":"Hepatology"},{"key":"10.1016\/j.jhep.2009.06.019_bib43","doi-asserted-by":"crossref","first-page":"707","DOI":"10.1046\/j.1365-2141.1996.365901.x","article-title":"Resolution of Budd-Chiari syndrome following bone marrow transplantation for paroxysmal nocturnal haemoglobinuria","volume":"92","author":"Graham","year":"1996","journal-title":"Br J Haematol"},{"key":"10.1016\/j.jhep.2009.06.019_bib44","doi-asserted-by":"crossref","first-page":"851","DOI":"10.1016\/S0730-725X(98)00081-2","article-title":"Active intrahepatic gadolinium extravasation following TIPS","volume":"16","author":"Hasegawa","year":"1998","journal-title":"Magn Reson Imaging"},{"key":"10.1016\/j.jhep.2009.06.019_bib45","doi-asserted-by":"crossref","first-page":"299","DOI":"10.1007\/s00277-003-0639-8","article-title":"Fibrinolytic therapy with rt-PA in a patient with paroxysmal nocturnal hemoglobinuria and Budd-Chiari syndrome","volume":"82","author":"Hauser","year":"2003","journal-title":"Ann Hematol"},{"key":"10.1016\/j.jhep.2009.06.019_bib46","doi-asserted-by":"crossref","first-page":"383","DOI":"10.1097\/01.RVI.0000196338.87954.CE","article-title":"Catheter-directed thrombolysis and thrombectomy for the Budd-Chiari syndrome in paroxysmal nocturnal hemoglobinuria in three patients","volume":"17","author":"Kuo","year":"2006","journal-title":"J Vasc Interv Radiol"},{"key":"10.1016\/j.jhep.2009.06.019_bib47","doi-asserted-by":"crossref","first-page":"85","DOI":"10.1111\/j.1365-2796.1994.tb01037.x","article-title":"Tissue plasminogen activator for hepatic vein thrombosis in paroxysmal nocturnal haemoglobinuria","volume":"235","author":"McMullin","year":"1994","journal-title":"J Intern Med"},{"key":"10.1016\/j.jhep.2009.06.019_bib48","doi-asserted-by":"crossref","unstructured":"Shindo S, Motohashi S, Kaga S, Inoue H, Matsumoto M, Shindo H. Paroxysmal nocturnal hemoglobinuria: complete resolution of an occluding inferior vena caval thrombus. Abdom Imaging 2007 [Epub ahead of print].","DOI":"10.1007\/s00261-007-9184-6"},{"key":"10.1016\/j.jhep.2009.06.019_bib49","doi-asserted-by":"crossref","first-page":"1065","DOI":"10.1007\/s00270-007-9067-9","article-title":"Late intrahepatic hematoma complicating transjugular intrahepatic portosystemic shunt for Budd-Chiari syndrome","volume":"30","author":"Terreni","year":"2007","journal-title":"Cardiovasc Intervent Radiol"},{"key":"10.1016\/j.jhep.2009.06.019_bib50","doi-asserted-by":"crossref","first-page":"75","DOI":"10.1007\/s11739-008-0182-7","article-title":"Budd-Chiari syndrome in a paroxysmal nocturnal hemoglobinuria patient with previous cerebral venous thrombosis","volume":"4","author":"Tufano","year":"2009","journal-title":"Intern Emerg Med"},{"key":"10.1016\/j.jhep.2009.06.019_bib51","doi-asserted-by":"crossref","first-page":"453","DOI":"10.1097\/00042737-200504000-00011","article-title":"Paroxysmal nocturnal hemoglobinuria and Budd-Chiari syndrome: therapeutic challenge with bone marrow transplantation, transjugular intrahepatic portosystemic shunt, and vena cava stent","volume":"17","author":"Vergniol","year":"2005","journal-title":"Eur J Gastroenterol Hepatol"},{"key":"10.1016\/j.jhep.2009.06.019_bib52","first-page":"180","article-title":"Liver transplant for Budd-Chiari syndrome caused by paroxysmal nocturnal hemoglobinuria","volume":"6","author":"Yedibela","year":"2008","journal-title":"Exp Clin Transplant"},{"key":"10.1016\/j.jhep.2009.06.019_bib53","doi-asserted-by":"crossref","first-page":"642","DOI":"10.1160\/TH07-11-0664","article-title":"Temporal trends in mortality rates from visceral vein thrombosis in paroxysmal nocturnal haemoglobinuria: an optimistic view","volume":"99","author":"Poulou","year":"2008","journal-title":"Thromb Haemost"}],"container-title":["Journal of Hepatology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S0168827809004632?httpAccept=text\/xml","content-type":"text\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S0168827809004632?httpAccept=text\/plain","content-type":"text\/plain","content-version":"vor","intended-application":"text-mining"}],"deposited":{"date-parts":[[2020,5,20]],"date-time":"2020-05-20T19:25:02Z","timestamp":1590002702000},"score":1,"resource":{"primary":{"URL":"https:\/\/linkinghub.elsevier.com\/retrieve\/pii\/S0168827809004632"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2009,10]]},"references-count":53,"journal-issue":{"issue":"4","published-print":{"date-parts":[[2009,10]]}},"alternative-id":["S0168827809004632"],"URL":"https:\/\/doi.org\/10.1016\/j.jhep.2009.06.019","relation":{},"ISSN":["0168-8278"],"issn-type":[{"value":"0168-8278","type":"print"}],"subject":[],"published":{"date-parts":[[2009,10]]}}}