Section of Digestive Diseases, Yale University School of Medicine, 333 Cedar Street, LMP 1080, New Haven, CT 06520, USA.
Section of Digestive Diseases, Yale Liver Center, Yale University School of Medicine, 333 Cedar Street, LMP 1080, New Haven, CT 06520, USA.
Clin Liver Dis. 2020 Aug;24(3):453-481. doi: 10.1016/j.cld.2020.04.012. Epub 2020 Jun 2.
Budd-Chiari syndrome (BCS), or hepatic venous outflow obstruction, is a rare cause of liver disease that should not be missed. Variable clinical presentation among patients with BCS necessitates a high index of suspicion to avoid missing this life-threatening diagnosis. BCS is characterized as primary or secondary, depending on etiology of venous obstruction. Most patients with primary BCS have several contributing risk factors leading to a prothrombotic state. A multidisciplinary stepwise approach is integral in treating BCS. Lifelong anticoagulation is recommended. Long-term monitoring of patients for development of cirrhosis, complications of portal hypertension, hepatocellular carcinoma, and progression of underlying diseases is important.
布加综合征（BCS），又称肝静脉流出道阻塞，是一种罕见的肝脏疾病病因，不容忽视。BCS 患者临床表现多变，需要高度怀疑以避免漏诊这一致命诊断。BCS 分为原发性和继发性，取决于静脉阻塞的病因。大多数原发性 BCS 患者存在导致血栓形成倾向的多种危险因素。多学科逐步治疗方法是治疗 BCS 的基础。建议长期抗凝治疗。长期监测患者的肝硬化、门静脉高压并发症、肝细胞癌和基础疾病的进展情况非常重要。
