中国常染色体显性多囊肾病临床实践指南

<正>常染色体显性多囊肾病(autosomal dominant polycystic kidney disease,ADPKD)是最常见的遗传性肾病,发病率为1/400~1/1000。ADPKD主要致病基因有两个,分别称为PKD1和PKD2。该病代代发病,子代发病机率为50%。患者多在成年后出现双侧肾脏囊肿,随年龄增长,逐渐损害肾脏结构和功能;至60岁时,约半数患者进展至终末期肾脏疾病(end stage renal disease,ESRD),占ESRD病因的第四位。ADPKD还可合并肝囊肿、心脏瓣膜异常、颅内动脉瘤等肾外病变。迄今,ADPKD尚无...

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Bibliographic Details
Published inLinchuang Shenzangbing Zazhi pp. 580 - 588
Main Authors 李林, 梅长林
Format Journal Article
LanguageChinese
Published Editorial Department of Journal of Clinical Nephrology 01.01.2016
Online AccessGet full text
ISSN1671-2390
DOI10.3969/j.issn.1671-2390.2016.10.001

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Summary:<正>常染色体显性多囊肾病(autosomal dominant polycystic kidney disease,ADPKD)是最常见的遗传性肾病,发病率为1/400~1/1000。ADPKD主要致病基因有两个,分别称为PKD1和PKD2。该病代代发病,子代发病机率为50%。患者多在成年后出现双侧肾脏囊肿,随年龄增长,逐渐损害肾脏结构和功能;至60岁时,约半数患者进展至终末期肾脏疾病(end stage renal disease,ESRD),占ESRD病因的第四位。ADPKD还可合并肝囊肿、心脏瓣膜异常、颅内动脉瘤等肾外病变。迄今,ADPKD尚无
ISSN:1671-2390
DOI:10.3969/j.issn.1671-2390.2016.10.001