遗传性肾炎（Ａｌｐｏｒｔ综合征）是一种表现于肾小球基底膜弥漫性病变的遗传性疾病。神经性耳聋、肾小球肾炎、进行性肾功能衰竭是其特征性表现。我院为２例患有此病的同胞兄弟作了肾移植术，术后肾功能恢复良好，分别随访３～５年，移植肾存活率１００％。提示遗传性肾炎肾功能衰竭接受肾移植手术是一种有效的治疗方法。 Hereditary nephritis (Alport syndrome) is a manifestation of the glomerular basement membrane diffuse lesions of the genetic disease. Neurological deafness, glomerulonephritis, progressive renal failure is its characteristic manifestation. In our hospital, 2 cases of siblings who had this disease were treated with kidney transplantation. The postoperative renal function recovered well. All cases were followed up for 3 to 5 years and the survival rate of graft was 100%. Prompt hereditary nephritis renal failure renal transplant surgery is an effective treatment.