遗传性进行性肾炎又名Alport氏综合征。为遗传性家族性疾病。其临床主要特点为反复血尿、进行性慢性肾功能衰竭,部分病例合并耳聋及眼疾。现报告一家二代共10人,其中4人发病,1人死亡。例1 肖姓,男 10岁。为本文的先证者。于1984年10月4日因全身浮肿、尿少一周,门诊以急性肾炎介绍入院。自幼聋哑。病前无感 Hereditary progressive nephritis, also known as Alport’s syndrome. Hereditary familial disease. The main clinical features of hematuria repeated, progressive chronic renal failure, some cases of deafness and eye diseases. It is reported that a second generation a total of 10 people, of which 4 were ill and 1 died. Example 1 Xiao surname, male 10 years old. Proof of this article. On October 4, 1984 due to systemic edema, less urine for a week, outpatient clinic introduced acute nephritis. Since childhood, deaf and dumb. No sense of illness before