常染色体显性遗传多囊肾病(Autosomal dominant polycystic kidney disease, ADPKD)是一种常见的单基因遗传病。它是导致终末期肾衰的重要疾病之一，发病率约为1/1000~1/400，约占终末期肾病病因的10%。目前尚缺乏有效治疗药物。近年来，国内外对其发病机制、诊断及治疗方面进行了很多相关的研究及其论述，主要集中在囊泡的发生发展和囊液分泌等方面，对肾脏间质参与的病变过程，如间质的炎症和纤维化，探讨较少。本文主要综述ADPKD发病机制及其间质病变在ADPKD发病中的作用。<br>Autosomal dominant polycystic kidney disease is a common single-gene genetic disease. It is one of the most important causes of end-stage renal failure. Its incidence is about 1/1000-1/400, accounting for about 10% of end-stage renal disease etiology. There are no effective drugs currently. In recent years, domestic and oversea researchers have conducted a lot of studies and discussions related to the pathogenesis, diagnosis and treatment, focused on the cyst development and secretion of cyst fluid secretion. But there is little research on renal interstitial disease, including interstitial inflammation and fibrosis. In this review, we mainly discuss the pathogenesis of ADPKD and effectives of interstitial disease in ADPKD.
%K 常染色体显性遗传多囊肾病，多囊蛋白，转化生长因子-β1，间质病变