Perrin D, Jungers P, Grünfeld J P, Delons S, Noël L H, Zenatti C
Clin Nephrol. 1980 Apr;13(4):163-7.
From 1962 to 1977, 79 patients with Alport's syndrome underwent serial fundoscopic examination. In 29 patients (24 males, 5 females), symmetric bilateral perimacular changes were found, consisting of bright, whitish or yellowish dense granulations, surrounding the foveal area. Fluorescein angiography was normal in 8/8 studied cases. All 29 patients had bilateral lenticonus and neural hearing loss; ultrastructural lesion of glomerular basement membrane was present in 11/11 studied patients. Chronic renal failure developed in 26 patients, with 20 treated by maintenance hemodialysis. In contrast, perimacular lesions were absent in the other 50 patients (27 males, 23 females). Anterior lenticonus was detected in none; perceptive deafness was demonstrated in only 20; kidney ultrastructural lesions in 16 of 30 studied cases. Patients with perimacular changes had significantly earlier renal failure (P less than 0.001) than patients without. In conclusion, perimacular changes appear as a simple and reliable indicator of Alport's syndrome, often associated with early renal failure.
1962年至1977年，对79例阿尔波特综合征患者进行了系列眼底检查。在29例患者（24例男性，5例女性）中，发现双侧黄斑周围对称性改变，表现为围绕黄斑区的明亮、白色或淡黄色致密颗粒。8例接受荧光素血管造影检查的患者结果均正常。所有29例患者均有双侧圆锥形晶状体和神经性听力损失；11例接受检查的患者肾小球基底膜有超微结构病变。26例患者出现慢性肾衰竭，其中20例接受维持性血液透析治疗。相比之下，另外50例患者（27例男性，23例女性）未出现黄斑周围病变。无一例患者有前圆锥形晶状体；仅20例患者有感觉神经性耳聋；30例接受检查的患者中有16例有肾脏超微结构病变。有黄斑周围改变的患者肾衰竭出现时间明显早于无黄斑周围改变的患者（P<0.001）。总之，黄斑周围改变是阿尔波特综合征的一个简单可靠指标，常与早期肾衰竭相关。
