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目的:分析常染色体显性遗传性多囊肾病(ADPKD)患者的临床特点。方法:回顾性分析2008年8月至2018年8月收治的263例成人型ADPKD患者的临床资料，包括性别、年龄、是否合并多囊肝、泌尿系结石、泌尿系感染；有无血尿、蛋白尿；有无糖尿病、高血压病、冠心病；血脂状况等。结果:高血压[60.1%(158/263)]、蛋白尿[39.5%(104/263)]、血尿[36.5%(96/263)]、泌尿系感染[35.7%(94/263)]、合并多囊肝[27.8%(73/263)]是ADPKD患者最常见的临床表现。男女患者性别比例为1.76∶1。男女患者发病年龄比较，差异无统计学意义(n P>0.05)；女性患者蛋白尿、三酰甘油水平以及肝囊肿、泌尿系感染发生率明显高于男性(n P<0.05)，而血红蛋白明显低于男性(n P<0.05)。n 结论:成人终末期多囊肾病发病与性别、年龄无关，但与高血压、血尿、蛋白尿、贫血、肾功不全、多囊肝、泌尿系感染密切相关，及早发现并干预，对于延缓肾功能恶化，改善预后有重要意义。“,”Objective:To analyze clinical characteristics of patients with autosomal dominant polycystic kidney disease(ADPKD).Methods:Clinical data, including various common complications in 263 ADPKD patients, were retrospectively analyzed from August 2008 to August 2018.Results:Among the 263 patients, there were 168 males with a mean age of(59.2±16.4) years and 95 females with a mean age of(60.8±12.0) years, 158 cases were complicated with hypertension, 104 cases with proteinuria, 96 cases with hematuria, 94 cases of patients were complicated with urinary tract infection, 73 cases were complicated with polycystic liver.There were 49 cases with coronary heart disease, 20 cases with diabetes mellitus, 29 cases with urinary calculi, anemia, low protein, hypertension, high uric acid and proteinuria, which were negatively correlated with renal function.Conclusions:ADPKD has nothing to do with age, hypertension, hematuria, proteinuria, anemia, renal insufficiency, polycystic liver, urinary tract infection is its common clinical manifestations, early detection and intervention, to delay the deterioration of renal function, improve the prognosis has important significance.