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진성 혈소판증가증에 동반한 Budd - Chiari 증후군 1 예
서봉근(Bong Geun Seo),한상영(Sang Young Han),이종훈(Jong Hun Lee),장진석(Jin Seok Jang),이현수(Hyun Soo Lee),노명환(Myung Hwan Roh),최석렬(Seok Ryeol Choi),신우원(Woo Won Shin) 대한소화기학회 2002 대한소화기학회지 Vol.39 No.1
Budd-Chiari syndrome is an acute or subacute obstruction of hepatic venous outflow which causes post-sinusoidal portal hypertension, esophageal varix, and cirrhotic course. Its prognosis is usually poor. The cause and pathogenesis are unclear, but the primary cause is generally thought to be congenital. The main secondary causes are chronic myeloproliferative disorders, paroxysmal nocturnal hemoglobinuria, presence of lupus anticoagulant, protein C or protein S deficiency, hypercoagulability due to oral pills, tumor, trauma, and infection. It can be treated by shunt operation, balloon dilatation, stent insertion, or transjugular intrahepatic portosystemic shunt. We report one case of Budd-Chiari syndrome with essential thrombocythemia which has never been reported in Korea. (Korean J Gastroenterol 2002;39:64-67)