PDF(Pubmed)
Abstract：
Porto-sinusoidal vascular disease (PSVD) is defined as a vascular liver disease characterized by the absence of cirrhosis and the presence of characteristic histological features, with or without the presence of portal hypertension (PH). Half of the patients with PSVD also have associated disease that may contribute to the development of PSVD. Patients usually remain asymptomatic until complications of PH arise. Variceal bleeding and portal vein thrombosis are major complications associated with PSVD. The treatment is focused on managing complications of PH, mainly through primary prophylaxis of variceal bleeding and treatment of portal vein thrombosis. Currently, there is insufficient evidence to support the use of anticoagulants for thrombosis prevention in these patients. Despite the increase of recognition of PSVD, further research is needed to enable early disease diagnosis, establish optimal screening methods, and develop strategies to slow down disease progression.
摘要：
波窦血管疾病（PSVD）被定义为以缺乏肝硬化和特征性组织学特征为特征的血管性肝病，有或没有门静脉高压（PH）的存在。一半的PSVD患者也患有可能导致PSVD发展的相关疾病。患者通常保持无症状，直到出现PH并发症。静脉曲张破裂出血和门静脉血栓形成是与PSVD相关的主要并发症。治疗的重点是治疗PH的并发症，主要通过初级预防静脉曲张破裂出血和治疗门静脉血栓形成。目前,没有足够的证据支持在这些患者中使用抗凝剂预防血栓形成.尽管人们对PSVD的认识有所增加，需要进一步的研究来实现早期疾病诊断，建立最佳的筛选方法，并制定减缓疾病进展的策略。
