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作者对一个遗传性肾炎家族中4个受累成员作了肾组织的光学和电子显微镜检查。该家族中4个受累成员为2男2女:2例男性均有血尿和神经性耳聋,其中1例见肾小球硬化伴有严重肾功能不全,17岁时死于尿毒症。2例女性无耳聋或肾功能不全,但有血尿,其中1例有持续蛋白尿,且每发作血尿时(每月1～2次)常伴有上呼吸道感染。她们的肾活检标本在光学显微镜下可见肾小球血管间质均有轻度非特异性增厚。在电子显微镜下,4例肾组织均见肾小球血管间质增多,毛细血管基底膜不规则增厚并分裂成几层,各层间有透亮的空隙,这些空隙中含有细小的致密颗粒。肾小管基底膜也见到这样的改变。作者后来又研究了13例遗传性肾炎的标本,其中5例见到,8例未见此改变。作者认为这种改变是遗传性肾炎的特异性
The authors performed optical and electron microscopic examinations of kidney tissue in four affected members of a hereditary nephritis family. Four affected members of the family were 2 males and 2 females: 2 males had hematuria and neurologic deafness, 1 of which had glomerulosclerosis with severe renal insufficiency and died of uremia at 17 years of age. 2 cases of women without deafness or renal insufficiency, but hematuria, of which 1 case of persistent proteinuria, and each episode of hematuria (1 or 2 times per month) often accompanied by upper respiratory tract infection. Their renal biopsy specimens showed mild nonspecific thickening of the glomerular mesenchyme in light microscopy. Under the electron microscope, glomerular vascular interstitium increased, capillary basement membrane irregularly thickened and split into several layers, and there were translucent voids among the layers. These voids contained tiny dense particles. Tubular basement membrane also saw such a change. The authors later studied 13 cases of hereditary nephritis specimens, of which 5 cases were seen, 8 cases did not change. The author considers this change is the specificity of hereditary nephritis