眼—耳—肾综合征又名阿尔波特氏(al—ports)综合征;遗传性出血性肾炎;出血性家族性肾炎;Diekinson综合征;慢性遗传性肾炎;遗传性家族性先天性出血性肾炎;先天遗传性血尿:特发性遗传性血尿;遗传性间质性肾盂肾炎等。本病是一种少见的遗传性疾病。临床特点:一、出血性肾炎;二、神经性耳聋;三、眼部特殊改变。眼部改变主要是:晶状体呈球形,晶状体呈前锥状或后锥状改变,有的晶状体前囊或后极部发生混浊。据文献报道还可出现眼球震颤,园锥角膜,角膜青年环,角膜色素沉着,视网膜脱离,白点状网膜病变,视网膜发育不全,黄斑变性,视乳头玻璃疣,假性视神经炎,近视等。在1927年Alports首先报告遗传性肾炎合并耳聋及眼部异常改变。此后国内外均 Eye-ear-kidney syndrome also known as al-ports syndrome; Hereditary hemorrhagic nephritis; Hemorrhagic familial nephritis; Diekinson syndrome; Chronic hereditary nephritis; Hereditary familial congenital hemorrhagic Nephritis; congenital hereditary hematuria: idiopathic hematuria; hereditary interstitial pyelonephritis. The disease is a rare genetic disease. Clinical features: First, hemorrhagic nephritis; Second, nerve deafness; Third, the special eye changes. Ocular changes are mainly: the lens was spherical, the lens was tapered or posterior conical changes, some of the anterior capsule or posterior pole opacity. According to the literature there may also be nystagmus, keratoconus, corneal young ring, corneal pigmentation, retinal detachment, retinal dysplasia, retinal dysplasia, macular degeneration, papilloma, pseudo optic neuritis, myopia, etc. . In 1927 Alports first reported hereditary nephritis with deafness and abnormal eye changes. Since then both at home and abroad