Document Detail


Good long-term outcome of Budd-Chiari syndrome with a step-wise management.
MedLine Citation:
PMID:  23389867     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
Budd-Chiari syndrome (BCS) is a rare, life-threatening disease caused by obstruction of hepatic venous outflow. The aim of the study was to assess long-term outcome and identify prognostic factors in BCS patients managed by a step-wise approach using anticoagulation, angioplasty/thrombolysis, transjugular intrahepatic portosystemic shunting (TIPS), and orthotopic liver transplantation (OLT). We reviewed long-term data on 157 patients previously included by the European Network for Vascular Disorders of the Liver, a multicenter prospective study of newly diagnosed BCS patients in nine European countries. Patients were followed for a median of 50 months (range, 0.1-74.0). During the study, 88 patients (56%) received at least one invasive intervention (22 patients angioplasty/thrombolysis, 62 TIPS, and 20 OLT) and 36 (22.9%) died. Most interventions and/or deaths occurred in the first 2 years after diagnosis. The Rotterdam score was excellent in predicting intervention-free survival, and no other variable could significantly improve its prognostic ability. Moreover, BCS-TIPS prognostic index (PI) score (based on international normalized ratio, bilirubin, and age) was strongly associated with survival and had a discriminative capacity, which was superior to the Rotterdam score. Conclusions: The current study confirms, in a large cohort of patients with BCS recruited over a short period, that a step-wise treatment approach provides good long-term survival. In addition, the study validates the Rotterdam score for predicting intervention-free survival and the BCS-TIPS PI score for predicting survival.
Authors:
Susana Seijo; Aurelie Plessier; Jildou Hoekstra; Alessandra Dell'era; Dalvinder Mandair; Kinan Rifai; Jonel Trebicka; Isabelle Morard; Luc Lasser; Juan G Abraldes; Sarwa Darwish Murad; Jörg Heller; Antoine Hadengue; Massimo Primignani; Elwyn Elias; Harry L A Janssen; Dominique C Valla; Juan-Carlos Garcia-Pagan;
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Publication Detail:
Type:  Journal Article; Multicenter Study; Research Support, Non-U.S. Gov't    
Journal Detail:
Title:  Hepatology (Baltimore, Md.)     Volume:  57     ISSN:  1527-3350     ISO Abbreviation:  Hepatology     Publication Date:  2013 May 
Date Detail:
Created Date:  2013-04-23     Completed Date:  2013-07-05     Revised Date:  2014-04-08    
Medline Journal Info:
Nlm Unique ID:  8302946     Medline TA:  Hepatology     Country:  United States    
Other Details:
Languages:  eng     Pagination:  1962-8     Citation Subset:  IM    
Copyright Information:
Copyright © 2013 American Association for the Study of Liver Diseases.
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MeSH Terms
Descriptor/Qualifier:
Adolescent
Adult
Aged
Aged, 80 and over
Anticoagulants / therapeutic use
Budd-Chiari Syndrome / diagnosis*,  mortality,  therapy*
Cohort Studies
Disease Management*
Europe
Female
Humans
Longitudinal Studies
Male
Middle Aged
Portasystemic Shunt, Transjugular Intrahepatic
Prognosis
Prospective Studies
Retrospective Studies
Survival Rate
Thrombolytic Therapy
Young Adult
Chemical
Reg. No./Substance:
0/Anticoagulants
Comments/Corrections
Comment In:
Hepatology. 2014 Mar;59(3):1213   [PMID:  23857382 ]

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine


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