title: Treatment of Budd-Chiari syndrome with a focus on transjugular intrahepatic portosystemic shunt.
abstract: OBJECTIVE
  To evaluate long-term complications and survival in patients with Budd-Chiari syndrome (BCS) referred to a Danish transjugular intrahepatic portosystemic shunt (TIPS) centre.
  
  
  METHODS
  Twenty-one consecutive patients from 1997-2008 were retrospectively included [15 women and 6 men, median age 40 years (range 17-66 years)]. Eighteen Danish patients came from the 1.8 million catchment population of Aarhus University Hospital and three patients were referred from Scandinavian hospitals. Management consisted of tests for underlying haematological, endocrinological, or hypercoagulative disorders parallel to initiation of specific treatment of BCS.
  
  
  RESULTS
  BCS was mainly caused by thrombophilic (33%) or myeloproliferative (19%) disorders. Forty-three percents had symptoms for less than one week with ascites as the most prevalent finding. Fourteen (67%) were treated with TIPS and 7 (33%) were manageable with treatment of the underlying condition and diuretics. The median follow-up time for the TIPS-treated patients was 50 mo (range 15-117 mo), and none required subsequent liver transplantation. Ascites control was achieved in all TIPS patients with a marked reduction in the dose of diuretics. A total of 14 TIPS revisions were needed, mostly of uncovered stents. Two died during follow-up: One non-TIPS patient worsened after 6 mo and died in relation to transplantation, and one TIPS patient died 4 years after the TIPS-procedure, unrelated to BCS.
  
  
  CONCLUSIONS
  In our BCS cohort TIPS-treated patients have near-complete survival, reduced need for diuretics and compared to historical data a reduced need for liver transplantation.
authors: Anders Bay Neumann, Stine Degn Andersen, Peter Holland-Fischer, Hendrik Vilstrup, Henning Grønbæk, Department of Medicine V (Gastroenterology and Hepatology), Aarhus University Hospital, DK-8000 Aarhus C, Denmark.
authors: Anders Bay
authors: AB
authors: Neumann
authors: Stine Degn
authors: SD
authors: Andersen
authors: Dennis Tønner
authors: DT
authors: Nielsen
authors: Peter
authors: P
authors: Holland-Fischer
authors: Hendrik
authors: H
authors: Vilstrup
authors: Henning
authors: H
authors: Grønbæk
keywords: Ascites; Budd-Chiari syndrome; Myeloproliferative disorder; Thrombophilia; Thrombosis; Transjugular intrahepatic portosystemic shunt
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