title: Etiology and portal vein thrombosis in Budd-Chiari syndrome.
abstract: OBJECTIVE
  To research the etiology, portal vein thrombosis and other features of Budd-Chiari syndrome (BCS) patients prospectively.
  
  
  METHODS
  A total of 75 patients (40 female, 35 male) who were diagnosed between January 2002 and July 2004 as having BCS were studied prospectively. Findings from on physical examination, ultrasonography, duplex ultrasonography and venography were analyzed. Hemogram and blood chemistry were studied at the time of diagnosis and on each hospital visit. Bone marrow examination and immune phenotyping were performed by a hematologist when necessary. Protein C, S, antithrombin III, activated protein C resistance, and anticardiolipin antibodies, antinuclear antibodies, and anti ds-DNA were studied twice. The presence of ascite, esophageal varices, and portal thrombosis were evaluated at admission and on every visit.
  
  
  RESULTS
  At least one etiological factor was determined in 54 (72%) of the patients. The etiology could not be defined in 21 (28%) patients. One etiological factor was found in 39, 2 factors in 14 and 3 factors in 1 patient. The most common cause was the web (16%), the second was Hydatid disease (11%), the third was Behcet's disease (9%). Portal vein thrombosis was present in 11 patients and at least one etiology was identified in 9 of them (82%).
  
  
  CONCLUSIONS
  Behcet's disease and hydatid disease are more prominent etiological factors in Turkey than in other countries. Patients with web have an excellent response to treatment without signs of portal vein thrombosis while patients having thrombofilic factors more than one are prone to develop portal vein thrombosis with worse clinical outcome.
authors.affiliation: Yayla Mah. 1. cadde no: 123/3, Kecioren, Ankara 06010, Turkey. ouskudar@hotmail.com
authors.forename: Oguz
authors.initials: O
authors.lastname: Uskudar
authors.forename: Meral
authors.initials: M
authors.lastname: Akdogan
authors.forename: Nurgul
authors.initials: N
authors.lastname: Sasmaz
authors.forename: Sevinc
authors.initials: S
authors.lastname: Yilmaz
authors.forename: Muharrem
authors.initials: M
authors.lastname: Tola
authors.forename: Burhan
authors.initials: B
authors.lastname: Sahin
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D001528:Behcet Syndrome / Q000150:complications / Q000453:epidemiology / Q000503:physiopathology; D006502:Budd-Chiari Syndrome / Q000453:epidemiology / Q000209:etiology* / Q000503:physiopathology; D004444:Echinococcosis, Hepatic / Q000150:complications / Q000453:epidemiology / Q000503:physiopathology; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D011169:Portal Vein / Q000000981:diagnostic imaging / Q000503:physiopathology; D011446:Prospective Studies; D012189:Retrospective Studies; D013927:Thrombosis / Q000453:epidemiology / Q000209:etiology* / Q000503:physiopathology; D014421:Turkey; D014463:Ultrasonography