电镜检查对小儿遗传性进行性肾炎诊断的意义(附8例报告)

The significance of electron-microscopy in the diagnosis of children Alport syndrome.

目的 :评价电镜检查对小儿遗传性进行性肾炎的诊断意义。方法 :对 8例遗传性进行性肾炎患儿进行肾活检组织电镜检查结合临床表现及随访观察。结果 :该病在小儿时期常表现为复发性血尿 ,很少有眼和耳异常及肾功能不全。部分患儿可无家族史。肾活检组织电镜检查肾小球基底膜(GBM)变薄或增厚和分层。结论 :电镜检查对遗传性进行性肾炎具有确诊意义。

Objective:To evaluate the significance of electron-microscopy in the diagnosis of children Alport syndrome.Method:Renal biopsy detected by electron-mcroscopy combining clinical presentations and followed up in 8 children with Alport syndrome.Results:The disease in childhood often presented with hematuria.hearing imparment,ocular lesions and renal failure were uncommon.Some patients had a negative faminaly history.Electron-microscopy finding of renal biopsy showed thinning or thickening and splitting of glomerular basement membrane.Conclusion:Electron-microscopy is a very useful means in the diagnosis of children Alport syndrome.