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Redefining Budd-Chiari syndrome:A systematic review

World Journal of Hepatology 2016年第16期8卷 691-702页

作者： Naomi Shin Young H Kim Hao Xu Hai-Bin Shi Qing-Qiao Zhang Jean Paul Colon Pons Ducksoo Kim Yi Xu Fei-Yun Wu Samuel Han Byung-Boong Lee Lin-Sun Li Department of Radiology Univer-sity of Massachusetts Medical School Department of Interventional Radiology the Affiliated Hospital of Xuzhou Medical College Department of Radiology the First Affiliated Hospital of Nanjing Medical University Department of Radiology Boston University School of Medicine Department of Gastroenterology University of Massachusetts Medical School Department of Surgery George Washington University School of Medicine

AIM:To re-examine whether hepatic vein thrombosis(HVT)(classical Budd-Chiari syndrome)and hepatic vena cava-Budd Chiari syndrome(HVC-BCS)are the same ***:A systematic review of observational studies conducted in adult subjects with primary BCS,hepatic vein outflow tract obstruction,membranous obstruction of the inferior vena cava(IVC),obliterative hepatocavopathy,or HVT during the period of January2000 until February 2015 was conducted using the following databases:Cochrane Library,CINAHL,MEDLINE,Pub Med and ***:Of 1299 articles identified,26 were included in this *** BCS is more common in women with a pure hepatic vein obstruction(49%-74%).HVCBCS is more common in men with the obstruction often located in both the inferior vena cava and hepatic veins(14%-84%).Classical BCS presents with acute abdominal pain,ascites,and ***-BCS presents with chronic abdominal pain and abdominalwall *** neoplasms(MPN)are the most common etiology of classical BCS(16%-62%)with the JAK2V617-F mutation found in 26%-52%.In HVCBCS,MPN are found in 4%-5%,and the JAK2V617-F mutation in 2%-5%.Classical BCS responds well to medical management alone and 1st line management of HVC-BCS involves percutaneous recanalization,with few managed with medical management ***:Systematic review of recent data suggests that classical BCS and HVC-BCS may be two clinically different disorders that involve the disruption of hepatic venous outflow.

关键词： Budd-Chiari Hepatic vein outflow tract obstruction Membranous obstruction of the inferior vena cava obliterative hepatocavopathy Hepatic vein thrombosis

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