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Articles

Title: Diagnosis and management of the Budd-Chiari syndrome

Author:
Joanna Raszeja-Wyszomirska
Type:
Review articles
Language:
EN
Journal:
Polish Gastroenterology
Year:
2010
Volume:
17
Number:
4
Start page:
317
Final page:
321
ISSN:
1232-9886
Keywords:
hepatic vein thrombosis, prothrombotic conditions, anticoagulation, TIPS, liver transplantation
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The Budd-Chiari syndrome (BCS) is the blockade of hepatic venous outflow from the small veins to the inferior vena cava (IVC). This rare disease affects mainly young adults. BCS once considered the primary entity, currently is recognized as a clinical manifestation of many prothrombotic conditions. Presentation and manifestation vary and BCS should always be considered in a young/middle age patient with portal hypertension of unknown origin. The diagnostic algorithm should cover wide spectrum of hereditary and acquired prothrombotic conditions supported by Doppler-ultrasound, computed tomography or magnetic resonance imaging of hepatic veins and IVC. The disease may be lethal within 3 years of symptom occurrence in majority of untreated patients, however, modern treatment allows to achieve almost 90% 5-year survival rate. In this paper we review diagnostic, clinical and therapeutic issues related to the primary BCS.