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Acute primary Budd-Chiari syndrome complicated with severe portal vein thrombosis - serious complication of delayed diagnosis and treatment Ostry zespół Budda-Chariego powikłany masywną zakrzepicą żyły wrotnej - poważne powikłanie spóźnionej diagnostyki i leczenia

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Author(s): Joanna Raszeja-Wyszomirska | Michał Wasilewicz

Journal: Polish Gastroenterology
ISSN 1232-9886

Volume: 18;
Issue: 2;
Start page: 91;
Date: 2011;
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Keywords: primary Budd-Chiari syndrome | portal vein thrombosis | prothrombotic conditions | myeloproliferative syndrome | essential thrombocythemia | pierwotny zespół Budda-Chiariego | zakrzepica żyły wrotnej | czynniki prozakrzepowe

ABSTRACT
Primary Budd-Chiari Syndrome (BCS) is an obstruction of the hepatic venous outflow related to a primary venous disease, in the absence of right heart failure, with a potentially dismal outcome if not treated optimally. BCS is a rare disease in population with a high standard of living. This syndrome affects young/middle age patients with the prevalence of about 2 per million inhabitants. The male to female ratio is closer to 1 and mean age is about 45 years at presentation. Complex therapeutic strategy has achieved 5-year survival rates approaching 90%, although long-term outcome might be jeopardized by other complications, e.g. extrahepatic portal vein thrombosis affecting about 15% of BCS patients. The disease is usually more severe in such a case. In this paper we described two cases of combined hepatic venous outflow tract obstruction and portal vein thrombosis in the course of essential thrombocythemia, not diagnosed and not treated despite haematological observation. Because of the clinical severity of the disease, the only therapeutic option was liver transplantation in both patients.

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