Jayanthi V, Udayakumar N
Department of Gastroenterology, Stanley Medical College, Chennai, India.
Minerva Gastroenterol Dietol. 2010 Mar;56(1):71-80.
Budd-Chiari Syndrome (BCS) is a rare cause of portal hypertension. Geographical variations occur, particularly in the clinical presentation, where there are distinct differences between West and South Asia. Idiopathic forms are common in south Asia, while hypercoagulable disorders are common causes of BCS in the West. The site of thrombosis is also different, with patients from South Asia presenting with combined obstruction of the hepatic veins and the inferior vena cava in contrast to isolated obstruction of the hepatic veins, common in the West. Ultrasound-Doppler studies confirm the diagnosis in the majority. Early radiological interventions, including transjugular intrahepatic portosystemic shunt, can cure the majority of cases with idiopathic forms. Prothrombotic forms are treated with long-term anticoagulants. Surgery is reserved to a selected few with long segment obstruction. Liver transplantation is indicated in patients with worsening clinical functional status not responding to medical and/or interventional management.
布加综合征（BCS）是门静脉高压的一种罕见病因。存在地域差异，尤其是在临床表现方面，西亚和南亚之间存在明显差异。特发性形式在南亚很常见，而高凝性疾病是西方BCS的常见病因。血栓形成部位也有所不同，南亚患者表现为肝静脉和下腔静脉联合阻塞，而西方常见的是孤立的肝静脉阻塞。超声多普勒检查可确诊大多数病例。早期放射介入治疗，包括经颈静脉肝内门体分流术，可治愈大多数特发性形式的病例。血栓前状态形式采用长期抗凝治疗。手术仅适用于少数长节段阻塞的患者。对于临床功能状态恶化且对药物和/或介入治疗无反应的患者，应考虑肝移植。
