遗传性肾炎是一种伴染色体显性遗传的慢性肾脏病,又名耳—眼—肾综合征,家族性肾炎、Alport’s综合征等。常伴有耳部疾患(耳聋、中耳炎)和视力缺陷(白内障、近视眼、视网膜炎和眼球震颤等)。本病虽不多见,但若能充分认识它,并在特发性血尿、慢性肾炎、尿毒症及复发性间质性肾盂肾炎的患者中注意鉴别诊断,则并非罕见。 [概念和历史] 遗传性肾炎早就为人们所知。1874年Samuelson首次叙述了本病,次年Dickinson报告了一个三代16人的家族中,有蛋白尿者11人。1902年Guthrie、1912年Keodall等、1923年Hurst相继报导了特发性、先天遗传性、家族性血尿性肾炎的调查, Hereditary nephritis is a chromosomally inherited chronic kidney disease, also known as otorhimenal syndrome, familial nephritis, Alport’s syndrome and so on. Often accompanied by ear disease (deafness, otitis media) and vision defects (cataracts, myopia, retinitis and nystagmus, etc.). Although the rare disease, but if fully aware of it, and in idiopathic hematuria, chronic nephritis, uremia and recurrent interstitial pyelonephritis in patients with differential diagnosis is not uncommon. [Concept and history] Hereditary nephritis has long been known. Samuelson first described the disease in 1874, and the following year Dickinson reported 11 cases of proteinuria in a three-generation, 16-person family. Guthrie in 1902, Keodall et al. In 1912, Hurst in 1923 successively reported the investigation of idiopathic, congenital, familial hematuria,