Farboody G H, Valenzuela R, McCormack L J, Kallen R, Osborne D G
Hum Pathol. 1979 Nov;10(6):655-68. doi: 10.1016/s0046-8177(79)80110-0.
Thirty-three patients with chronic hereditary nephritis, obtained from 23 unrelated families, were evaluated with respect to clinicopathologic features. Renal tissue was examined by light microscopy in 25 cases, immunofluorescence in 19 cases, and electron microscopy in 16 cases. The light microscopic findings varied, and foam cells were present in only four cases. Immunofluorescence was negative in all but four cases, and in these the immunomicroscopic pattern was compatible with the findings of end stage glomeruli and hyaline arteriolar sclerosis. Although electron microscopy uniformly showed marked thinning or splitting of the glomerular basement membrane, parallel splitting of the glomerular basement membrane with interposition of electron dense granular particles was seen in only eight cases. Association of glomerular basement membrane splitting with granular particles was observed in four of six patients with IgA nephropathy, in two patients with benign familial hematuria, and in a normal kidney donor. Eleven patients, seven men and four women, had chronic renal failure requiring dialysis. Of five patients who received renal allografts, three are alive, with post-transplant survival ranging from 24 to 70 months. The other two died of septicemia.
对来自23个非亲缘家庭的33例慢性遗传性肾炎患者的临床病理特征进行了评估。25例患者的肾组织进行了光镜检查，19例进行了免疫荧光检查，16例进行了电镜检查。光镜检查结果各异，仅4例可见泡沫细胞。除4例以外，免疫荧光检查均为阴性，这4例的免疫显微镜表现与终末期肾小球及透明小动脉性硬化的表现相符。虽然电镜检查均显示肾小球基底膜明显变薄或分层，但仅8例可见肾小球基底膜平行分层并伴有电子致密颗粒状物质插入。在6例IgA肾病患者中的4例、2例良性家族性血尿患者及1例正常肾供体中观察到肾小球基底膜分层与颗粒状物质相关。11例患者（7例男性，4例女性）出现慢性肾衰竭需要透析治疗。接受肾移植的5例患者中，3例存活，移植后存活时间为24至70个月。另外2例死于败血症。
