遗传性进行性肾炎,又名Alport’s综合征。其临床特点为持续性血尿,神经性耳聋和进行性肾功能衰竭(下称肾衰)。本病于19世纪末国外文献早有报导。1902年由Guthrie首先提出遗传性肾疾病的名称,1927年Alport’s随访了Guthrie组病人的家系,发现部分病人除肾脏损害外尚有神经性耳聋。既往曾把不伴耳聋者称Guthrie病,伴耳聋者称Alport’s综合征,这是毫无意义的,因为两者可以在同一家庭中出现。近年来,本病在世界各地均有报导,据不完全统计,截止80年国外文献已有近百个家系报告,国内北京医学院附属一院内科报导11家系和儿科16家系综合报告,北京儿童医院近年共收治10个家系。 Hereditary progressive nephritis, also known as Alport’s syndrome. The clinical features of persistent hematuria, neurological deafness and progressive renal failure (hereinafter referred to as renal failure). The disease in the late 19th century foreign literature has long been reported. In 1902, Guthrie first proposed the name of hereditary kidney disease. In 1927, Alport’s followed up the pedigree of Guthrie patients and found that some patients had neurodegeneration other than kidney damage. It has been meaningless to include Guthrie disease with deafness and Alport’s syndrome with deafness in the past, because both can occur in the same family. In recent years, the disease has been reported around the world, according to incomplete statistics, as of 80 years, nearly 100 pedigrees reported in foreign literature, the Beijing Medical College Affiliated Hospital reported 11 pediatrics and 16 pediatrics comprehensive report, Beijing Children In recent years, the hospital admitted a total of 10 pedigrees.