多囊肾病(PKD)主要包括常染色体显性多囊肾病(ADPKD)及常染色体隐性多囊肾病(ARPKD)。其中ADPKD是人类最常见的遗传性肾病，占终末期肾病病因第4位。近年来发达国家已广泛建立PKD的网络注册及登记数据库，进展迅速，内容主要包括PKD患者临床和遗传信息、治疗、及标本科研信息登记等。我国亟需建立PKD患者注册和登记的网络数据库，可为进一步研究PKD的发病机制，阻断疾病遗传，延缓PKD进展提供依据。

Polycystic kidney disease (PKD) is mainly classified into autosomal dominant polycystic kidney disease (ADPKD), and autosomal recessive polycystic kidney disease (ARPKD). ADPKD is the most common human hereditary kidney disease, ranking the fourth cause for end-stage renal disease. In recent years, PKD registration databases developed rapidly in developed countries, including clinical and genetic information, registration of therapy, specimens, and research information. Now it is urgently needed for our country to establish a PKD registration database so as to provide data for further study on the pathogenesis of ADPKD, block disease heredity, and delay the progression of ADPKD.