Department of Gastroenterology, King Edward Memorial Hospital and Seth Gordhandas Sunderdas Medical College, Mumbai, India.
Alka Hospital, Kathmandu, Nepal.
Hepatol Int. 2021 Jun;15(3):531-567. doi: 10.1007/s12072-021-10189-4. Epub 2021 Jul 8.
Budd Chiari syndrome (BCS) is a diverse disease with regard to the site of obstruction, the predisposing thrombophilic disorders and clinical presentation across the Asia-Pacific region. The hepatic vein ostial stenosis and short segment thrombosis are common in some parts of Asia-Pacific region, while membranous obstruction of the vena cava is common in some and complete thrombosis of hepatic veins in others. Prevalence of myeloproliferative neoplasms and other thrombophilic disorders in BCS varies from region to region and with different sites of obstruction. This heterogeneity also raises several issues and dilemmas in evaluation and approach to management of a patient with BCS. The opportunity to recanalize hepatic vein in patients with hepatic vein ostial stenosis or inferior vena cava stenting or pasty among those membranous obstruction of the vena cava is a unique opportunity in the Asia-Pacific region to restore hepatic outflow closely mimicking physiology. In order to address these issues arising out of the diversity as well as the unique features in the region, the Asia Pacific Association for Study of Liver has formulated these guidelines for clinicians.
布加综合征（BCS）是一种具有多种表现的疾病，其特征为阻塞部位、易栓倾向及临床表现存在多样性。在亚太地区，肝静脉开口狭窄和短节段血栓形成较为常见，而膜性腔静脉阻塞在某些地区较为常见，另一些地区则表现为肝静脉完全血栓形成。BCS 患者中骨髓增生性肿瘤和其他易栓倾向的疾病的患病率在不同地区和不同阻塞部位存在差异。这种异质性也在评估和处理 BCS 患者时引发了一些问题和困境。在亚太地区，对于肝静脉开口狭窄或下腔静脉支架置入或膜性腔静脉阻塞患者再通肝静脉的机会是一种独特的机会，可以恢复接近生理的肝流出道。为了解决这些由多样性和该地区独特特征所带来的问题，亚太肝脏研究学会为临床医生制定了这些指南。
