多囊肝病的治疗及进展

多囊肝病(polycystic liver diseases,PLD)是一种罕见的遗传性疾病,囊肿可单独出现于肝脏(常染色体显性遗传性多囊肝病)或者合并肾脏囊肿(常染色体显性遗传性多囊肾病)。PLD是良性疾病,囊肿的体积和数量会持续不断的增长但肝脏功能不受影响。大部分患者无临床症状,无需治疗或仅需保守治疗。少数患者会因肝肿大占位效应或囊肿并发症而产生严重的临床症状时需要治疗,目的主要是减小肝脏的体积,治疗方法包括抽吸硬化术、开窗术、部分肝切除术、肝动脉栓塞术、肝脏移植术及药物治疗等。本文对PLD的治疗及进展进行综述。

Progress in Treatment of Polycystic Liver Disease

Polycystic liver diseases (PLD) represent a group of rare genetic disorders in which cysts occur in the liver (autosomal dominant polycystic liver disease) or in combination with cysts in the kidneys (autosomal dominant polycystic kidney disease). PLD is a benign disease, characterized by a continuous increase in the volume and the number of cysts while no influence on liver function, and most patients are asymptomatic. For these cases, it is no need of treatment or only conservative treatment. However, a minority of patients with severe symptoms including massive hepatomegaly with compression of the surrounding organs or cysts complications need treatments. The aim of treatments is to reduce liver volume for relieving symptoms. The means of therapies included aspiration-sclerotherapy, fenestration, segmental hepatic resection, hepatic artery embolization, liver transplantation and medical treatment. This paper reviewed the progess in the treatment of PLD.