尽管他们的患病率相对较低,肝脏的血管疾病代表了肝脏疾病领域的重大健康问题。许多此类疾病共有的共同特征是它们倾向于引起门静脉高压以及增加的发病率和死亡率。这些疾病通常在年轻患者中被诊断出来,其延迟诊断和/或不适当的治疗会大大降低预期寿命。本文回顾了当前有关Budd-Chiari综合征的证据,非肝硬化门静脉血栓形成,特发性门静脉高压症,正弦阻塞综合征,遗传性出血性毛细血管扩张症的肝血管畸形,肝硬化门静脉血栓形成和其他罕见的血管疾病,包括动静脉瘘。它还包括关于肝脏血管疾病的诊断成像及其从血液学角度的治疗的部分(血栓素质和抗凝治疗的研究)。所有建议均基于从PubMed中提取的已发表研究。证据的质量和建议的强度按照分级系统(建议的分级,评估开发和评估)。在缺乏充分证据的情况下,建议是根据编写指南的委员会的意见提出的。
Despite their relatively low prevalence, vascular diseases of the liver represent a significant health problem in the field of liver disease. A common characteristic shared by many such diseases is their propensity to cause portal hypertension together with increased morbidity and mortality. These diseases are often diagnosed in young patients and their delayed diagnosis and/or inappropriate treatment can greatly reduce life expectancy. This article reviews the current body of evidence concerning Budd-Chiari syndrome, non-cirrhotic portal vein thrombosis, idiopathic portal hypertension, sinusoidal obstruction syndrome, hepatic vascular malformations in hereditary haemorrhagic telangiectasia, cirrhotic portal vein thrombosis and other rarer vascular diseases including arterioportal fistulas. It also includes a section on the diagnostic imaging of vascular diseases of the liver and their treatment from a haematological standpoint (study of thrombotic diathesis and anticoagulation therapy). All recommendations are based on published studies extracted from PubMed. The quality of evidence and strength of recommendations were rated in accordance with the GRADE system (Grading of Recommendations, Assessment Development and Evaluation). In the absence of sufficient evidence, recommendations were based on the opinion of the committee that produced the guide.