PDF(Pubmed)
Abstract：
The expression splanchnic vein thrombosis encompasses Budd-Chiari syndrome and portal vein thrombosis. These disorders have common characteristics: they are both rare diseases which can cause portal hypertension and its complications. Budd-Chiari syndrome and portal vein thrombosis in the absence of underlying liver disease share many risk factors, among which myeloproliferative neoplasms represent the most common; a rapid comprehensive work-up for risk factors of thrombosis is needed in these patients. Long-term anticoagulation is indicated in most patients. Portal vein thrombosis can also develop in patients with cirrhosis and in those with porto-sinusoidal vascular liver disease. The presence and nature of underlying liver disease impacts the management of portal vein thrombosis. Indications for anticoagulation in patients with cirrhosis are growing, while transjugular intrahepatic portosystemic shunt is now a second-line option. Due to the rarity of these diseases, studies yielding high-grade evidence are scarce. However, collaborative studies have provided new insight into the management of these patients. This article focuses on the causes, diagnosis, and management of patients with Budd-Chiari syndrome, portal vein thrombosis without underlying liver disease, or cirrhosis with non-malignant portal vein thrombosis.
摘要：
内脏静脉血栓形成的表达包括Budd-Chiari综合征和门静脉血栓形成。这些疾病具有共同的特征：它们都是罕见的疾病，可引起门静脉高压及其并发症。Budd-Chiari综合征和门静脉血栓在没有基础肝病的情况下共有许多危险因素。其中骨髓增殖性肿瘤是最常见的;在这些患者中,需要对血栓形成的危险因素进行快速全面的检查.大多数患者需要长期抗凝治疗。肝硬化患者和门窦血管性肝病患者也可能发生门静脉血栓形成。潜在肝脏疾病的存在和性质影响门静脉血栓形成的管理。肝硬化患者的抗凝适应症越来越多，而经颈静脉肝内门体分流术现在是二线选择。由于这些疾病的罕见，产生高级证据的研究很少。然而,合作研究为这些患者的管理提供了新的见解。本文主要探讨其原因,诊断,以及布加综合征患者的治疗，无潜在肝病的门静脉血栓形成,或肝硬化合并非恶性门静脉血栓形成。
