{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,2,5]],"date-time":"2026-02-05T23:50:50Z","timestamp":1770335450778,"version":"3.49.0"},"reference-count":15,"publisher":"Ovid Technologies (Wolters Kluwer Health)","issue":"5","license":[{"start":{"date-parts":[[2009,5,1]],"date-time":"2009-05-01T00:00:00Z","timestamp":1241136000000},"content-version":"tdm","delay-in-days":0,"URL":"http:\/\/doi.wiley.com\/10.1002\/tdm_license_1.1"},{"start":{"date-parts":[[2009,4,27]],"date-time":"2009-04-27T00:00:00Z","timestamp":1240790400000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":["lww.com","ovid.com"],"crossmark-restriction":true},"short-container-title":[],"published-print":{"date-parts":[[2009,5]]},"abstract":"<jats:sec>\n            <jats:title\/>\n            <jats:p>Paroxysmal nocturnal hemoglobinuria (PNH) is a rare, acquired hemolytic anemia caused by somatic mutations in the phosphatidylinositol glycan-complementation class A gene and the resulting absence of a key complement regulatory protein, CD59. Affected red blood cells in patients with PNH undergo intravascular complement-mediated lysis with resulting anemia, hemoglobinuria, and venous thromboses. Hepatic venous outflow thrombosis [Budd-Chiari syndrome (BCS)] is especially common in PNH patients and often fatal. The few case reports of outcomes in patients undergoing liver transplant for BCS secondary to PNH detail instances of recurrent BCS as well as early thrombotic portal vein occlusion and hepatic artery thrombosis requiring retransplantation. PNH is therefore generally considered a contraindication to liver transplantation. Here we present the first report of a patient with PNH and BCS undergoing successful liver transplantation while receiving eculizumab, a humanized monoclonal antibody that blocks the activation of the terminal complement at C5.<\/jats:p>\n          <\/jats:sec>","DOI":"10.1002\/lt.21714","type":"journal-article","created":{"date-parts":[[2009,4,27]],"date-time":"2009-04-27T23:20:22Z","timestamp":1240874422000},"page":"540-543","update-policy":"https:\/\/doi.org\/10.1097\/lww.0000000000001000","source":"Crossref","is-referenced-by-count":25,"title":["Successful liver transplantation for Budd-Chiari syndrome in a patient with paroxysmal nocturnal hemoglobinuria treated with the anti-complement antibody eculizumab"],"prefix":"10.1097","volume":"15","author":[{"given":"Andrew L.","family":"Singer","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Jamye E.","family":"Locke","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Zoe A.","family":"Stewart","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Bonnie E.","family":"Lonze","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"James P.","family":"Hamilton","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Jennifer R.","family":"Scudiere","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Robert A.","family":"Anders","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Russell P.","family":"Rother","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Robert A.","family":"Brodsky","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Andrew M.","family":"Cameron","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"276","reference":[{"key":"R1-12-20250330","doi-asserted-by":"crossref","first-page":"587","DOI":"10.7326\/0003-4819-148-8-200804150-00003","article-title":"Paroxysmal nocturnal hemoglobinuria: the physiology of complementrelated hemolytic anemia.","volume":"148","author":"Brodsky","year":"2008","journal-title":"Ann Intern Med"},{"key":"R2-12-20250330","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":"R3-12-20250330","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":"R4-12-20250330","doi-asserted-by":"crossref","first-page":"573","DOI":"10.1016\/S0140-6736(95)12360-1","article-title":"Paroxysmal nocturnal haemoglobinuria: longterm followup and prognostic factors. French Society of Haematology.","volume":"348","author":"Socie","year":"1996","journal-title":"Lancet"},{"key":"R5-12-20250330","doi-asserted-by":"crossref","first-page":"43","DOI":"10.1097\/00000658-199001000-00007","article-title":"Liver transplantation for the BuddChiari syndrome.","volume":"211","author":"Halff","year":"1990","journal-title":"Ann Surg"},{"key":"R6-12-20250330","doi-asserted-by":"crossref","first-page":"354","DOI":"10.1007\/BF00335976","article-title":"Liver transplantation for PNH with BuddChiari syndrome. A case report.","volume":"6","author":"Schattenfroh","year":"1993","journal-title":"Transpl Int"},{"key":"R7-12-20250330","doi-asserted-by":"crossref","first-page":"890","DOI":"10.1111\/j.1432-2277.2003.tb00260.x","article-title":"Recurrence of BuddChiari syndrome after liver transplantation in paroxysmal nocturnal hemoglobinuria.","volume":"16","author":"Bahr","year":"2003","journal-title":"Transpl Int"},{"key":"R8-12-20250330","first-page":"867","article-title":"Recurrence of the BuddChiari syndrome after orthotopic liver transplantation.","volume":"46","author":"Ruckert","year":"1999","journal-title":"Hepatogastroenterology"},{"key":"R9-12-20250330","doi-asserted-by":"crossref","first-page":"629","DOI":"10.1053\/jlts.2003.50082","article-title":"Repeated graft loss caused by recurrent hepatic artery thrombosis after liver transplantation.","volume":"9","author":"Vivarelli","year":"2003","journal-title":"Liver Transpl"},{"key":"R10-12-20250330","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":"R11-12-20250330","doi-asserted-by":"crossref","first-page":"1256","DOI":"10.1038\/nbt1344","article-title":"Discovery and development of the complement inhibitor eculizumab for the treatment of paroxysmal nocturnal hemoglobinuria.","volume":"25","author":"Rother","year":"2007","journal-title":"Nat Biotechnol"},{"key":"R12-12-20250330","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":"R13-12-20250330","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":"R14-12-20250330","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":"R15-12-20250330","doi-asserted-by":"crossref","first-page":"459","DOI":"10.1093\/ajcp\/114.3.459","article-title":"Improved detection and characterization of paroxysmal nocturnal hemoglobinuria using fluorescent aerolysin.","volume":"114","author":"Brodsky","year":"2000","journal-title":"Am J Clin Pathol"}],"container-title":["Liver Transplantation"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.wiley.com\/onlinelibrary\/tdm\/v1\/articles\/10.1002%2Flt.21714","content-type":"unspecified","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/api.wiley.com\/onlinelibrary\/tdm\/v1\/articles\/10.1002%2Flt.21714","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/journals.lww.com\/01445473-200905000-00012","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,3,30]],"date-time":"2025-03-30T23:51:29Z","timestamp":1743378689000},"score":1,"resource":{"primary":{"URL":"https:\/\/journals.lww.com\/01445473-200905000-00012"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2009,5]]},"references-count":15,"journal-issue":{"issue":"5","published-print":{"date-parts":[[2009]]}},"URL":"https:\/\/doi.org\/10.1002\/lt.21714","relation":{},"ISSN":["1527-6465"],"issn-type":[{"value":"1527-6465","type":"print"}],"subject":[],"published":{"date-parts":[[2009,5]]}}}