title: Long-term follow-up study in Budd-Chiari syndrome: single-center experience in 22 years.
abstract: OBJECTIVE
  We aimed to present our long-term surveillance experience in patients with Budd-Chiari syndrome (BCS), and we retrospectively evaluated the natural history, results of thrombophilia studies, and the factors related to mortality.
  
  
  BACKGROUND
  Primary BCS is a rare form of vascular disease, secondary to underlying thrombophilia. Because of its rarity and heterogeneous nature, there is a scarcity of knowledge about the natural history of the disease.
  In 22 years, a total of 62 patients with primary BCS were followed in our tertiary hospital. We identified an acquired cause of BCS in 40 out of 62 patients (64.5%), whereas in 6 patients (9.7%), we found no identifiable cause. One or more thrombophilia causes were identified in 56 patients (90.3%). In 19 patients with myeloproliferative disease, 15 had Janus tyrosine kinase 2 mutation analysis and Janus tyrosine kinase 2 positivity was found in 10 patients. In regression analysis, portal vein thrombosis was found to be the only indicator of mortality, with an estimated instantaneous risk of 8.4.
  
  
  CONCLUSIONS
  In this study, we present one of the largest series of BCS in the English literature. We have shown that the multifactorial nature of underlying thrombophilia should be thoroughly investigated. In a patient with BCS, a clinician should be alert for the development or coexistence of portal vein thrombosis due to its deleterious effect on mortality.
authors.affiliation: Departments of Gastroenterology, Hacettepe University School of Medicine, Ankara, Turkey. ozgurmd@hacettepe.edu.tr
authors.forename: Ozgur
authors.initials: O
authors.lastname: Harmanci
authors.forename: Taylan
authors.initials: T
authors.lastname: Kav
authors.forename: Bora
authors.initials: B
authors.lastname: Peynircioglu
authors.forename: Yahya
authors.initials: Y
authors.lastname: Buyukasik
authors.forename: Cenk
authors.initials: C
authors.lastname: Sokmensuer
authors.forename: Yusuf
authors.initials: Y
authors.lastname: Bayraktar
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D006502:Budd-Chiari Syndrome / Q000235:genetics / Q000401:mortality / Q000503:physiopathology*; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D053614:Janus Kinase 2 / Q000235:genetics*; D008297:Male; D008875:Middle Aged; D009154:Mutation; D011169:Portal Vein; D012044:Regression Analysis; D012189:Retrospective Studies; D019851:Thrombophilia / Q000150:complications*; D013997:Time Factors; D020246:Venous Thrombosis / Q000209:etiology / Q000473:pathology*; D055815:Young Adult
chemical_list: C507924:JAK2 protein, human; D053614:Janus Kinase 2