遗传性肾炎又名Alport氏综合征,国内报导甚少。我们发现5例,其中1例做了局部解剖,除本病外尚合并肾结石,特报告如下: 临床资料先证者同胞8人,3例夭折,1例未确诊,余4例及其母均患本病,其中2例已死于尿毒症。家系调查如下图: 例1:先证病例介绍男性,13岁,住院号90103,1980年10月9日入院。发现蛋白尿已1个月,除有时头痛外无其它不适。大哥、三哥均因尿毒症于近期死亡,其弟亦有蛋白尿。体检正常,唯视力右0.6左0.5,双侧晶体轻微混浊,中心凹反射消失,黄斑区混浊粗糙。双耳鼓膜正常,电测听有中度神经性耳聋。血小板15.4万/毫米~3,长形、弯曲杆状占9％。血肌酐、血尿素氮、 Hereditary nephritis, also known as Alport’s syndrome, little domestic coverage. We found 5 cases, including 1 case of local anatomy, in addition to this disease is still associated with kidney stones, special report is as follows: 8 cases of proband siblings clinical data, 3 cases died, 1 case was not diagnosed, the remaining 4 cases and their mother All had this disease, of which 2 had died of uremia. Family survey as shown below: Example 1: Prevalence cases introduced male, 13 years old, hospital number 90,103, October 9, 1980 admission. Proteinuria has been found for 1 month, except for occasional headache and no other discomfort. Big Brother, Third Brother are due to uremia in the near future, his brother also has proteinuria. Physical examination was normal, only the right visual acuity 0.6 left 0.5, slightly cloudy on both sides of the crystal, the foveal reflex disappeared, the macular area cloudy and rough. Binaural eardrum normal, electrical audiometry with moderate nerve deafness. Platelet 154,000 / mm ~ 3, long, curved rod accounted for 9%. Serum creatinine, blood urea nitrogen,