良性家族性血尿是一种比较少见的遗传性肾脏疾患。在1986年全国21省市地区儿科224291名儿童尿过筛检查中共发现本病18人,占总受检人数的0.08‰。本病多有家族史,表现为持续性镜下血尿伴间歇发作性肉眼血尿,肾功能多良好。因此,称为良性家族性血尿(Benign familial hematuria)。但近年来有的作者指出本病是否预后良好,需在长期观察后方可确定。遗传方式本病多表现为常染色体显性遗传,偶 Benign familial hematuria is a relatively rare hereditary kidney disease. In 1986, 224,91 children in 21 provinces and municipalities in China were examined by urinalysis, and 18 people were found, accounting for 0.08 ‰ of the total number of people examined. The disease is more family history, manifested as persistent microscopic hematuria with intermittent onset of gross hematuria, renal function and more good. Therefore, it is called benign familial hematuria. However, some authors pointed out in recent years whether the prognosis of this disease is good and needs to be confirmed after long-term observation. The way the disease manifested as autosomal dominant, even