title: Budd-Chiari syndrome: a single-center experience.
abstract: OBJECTIVE
  To investigate challenges, risk factors, prognostic indicators, and treatment outcomes associated with Budd-Chiari syndrome (BCS) at a tertiary care center.
  
  
  METHODS
  A retrospective cohort study was conducted at the University of Pennsylvania in patients with a diagnosis of BCS or hepatic vein thrombosis. All patients receiving care at the University of Pennsylvania, and who had at least 2 clinical encounters in the University of Pennsylvania Health system from January 1, 2008 to September 10, 2013 were eligible for study inclusion. Data were extracted from the electronic medical record of each patient, and recorded in a secure Research Electronic Data Capture database. Logistic regression analyses were applied to identify predictors of outcome of liver transplant (LT) or death.
  
  
  RESULTS
  Between January 1, 2008 and September 10, 2013, forty-seven patients were identified. Median age was 42.4 years. Thirty-one (66.0%) were women. A majority were Caucasian (68.1%). At diagnosis, 43 (91.5%) patients had ascites, 27 (57.4%) patients had a hematologic disorder associated with a hypercoagulable state and 26 (55.3%) had cirrhosis. Forty (85.1%) patients were on anticoagulation (AC), 30 (63.8%) of whom were maintained on warfarin. Two patients (4.3%) underwent thrombolytic therapy. A transjugular intrahepatic portosystemic shunt (TIPS) was placed in 21 (44.7%) patients, 19 (90.5%) of whom were also on AC. Twenty-one (44.7%) received AC alone. Over a median of 974 d, 8 (17.0%) patients received LT, and 10 (21.3%) died. The median time from listing to death was 26 mo [interquartile range (IQR) = 16, 65)]. TIPS with AC was utilized more frequently in younger patients (P = 0.02). Age, cirrhosis and chronic kidney disease (CKD) were significant predictors of LT or death.
  
  
  CONCLUSIONS
  AC alone was employed as frequently as TIPS with AC, though the latter was used more frequently in younger patients with polycythemia vera. There were no significant differences in treatment outcome regardless of the therapeutic intervention employed. Significant predictors of poor prognosis included age, cirrhosis and CKD.
authors.affiliation: Tanya M Pavri, Alan Herbst, Rajender Reddy, Kimberly A Forde, Division of Gastroenterology and Hepatology, Perelman School of Medicine at the University of Pennsylvania, Philadelphia, PA 19104, United States.
authors.forename: Tanya M
authors.initials: TM
authors.lastname: Pavri
authors.affiliation: Tanya M Pavri, Alan Herbst, Rajender Reddy, Kimberly A Forde, Division of Gastroenterology and Hepatology, Perelman School of Medicine at the University of Pennsylvania, Philadelphia, PA 19104, United States.
authors.forename: Alan
authors.initials: A
authors.lastname: Herbst
authors.affiliation: Tanya M Pavri, Alan Herbst, Rajender Reddy, Kimberly A Forde, Division of Gastroenterology and Hepatology, Perelman School of Medicine at the University of Pennsylvania, Philadelphia, PA 19104, United States.
authors.forename: Rajender
authors.initials: R
authors.lastname: Reddy
authors.affiliation: Tanya M Pavri, Alan Herbst, Rajender Reddy, Kimberly A Forde, Division of Gastroenterology and Hepatology, Perelman School of Medicine at the University of Pennsylvania, Philadelphia, PA 19104, United States.
authors.forename: Kimberly A
authors.initials: KA
authors.lastname: Forde
mesh_terms: D000328:Adult; D000367:Age Factors; D000368:Aged; D000925:Anticoagulants / Q000009:adverse effects / Q000627:therapeutic use*; D006502:Budd-Chiari Syndrome / Q000175:diagnosis / Q000209:etiology / Q000401:mortality / Q000628:therapy*; D016009:Chi-Square Distribution; D015897:Comorbidity; D005260:Female; D006801:Humans; D053208:Kaplan-Meier Estimate; D016031:Liver Transplantation / Q000009:adverse effects / Q000401:mortality; D016015:Logistic Models; D008297:Male; D008875:Middle Aged; D015143:Philadelphia; D019168:Portasystemic Shunt, Transjugular Intrahepatic; D012189:Retrospective Studies; D012307:Risk Factors; D062606:Tertiary Care Centers; D015912:Thrombolytic Therapy / Q000009:adverse effects / Q000401:mortality; D013997:Time Factors; D016896:Treatment Outcome; D014850:Waiting Lists / Q000401:mortality
chemical_list: D000925:Anticoagulants
keywords: Anticoagulation; Budd-Chiari syndrome; Hepatic vein thrombosis; Liver transplantation; Transjugular intrahepatic portosystemic shunt