小儿IgA肾病与肾小球基底膜变薄

The children IgA nephropathy with the GBM thinning

目的为了深入研究ＩｇＡ肾病的临床与病理改变的关系。方法根据肾小球基底膜（ＧＢＭ）变薄的范围，将４８例小儿原发性ＩｇＡ肾病分为三组，对其临床、病理及其超微结构进行了系统观察，对ＧＢＭ厚度进行了测量。结果小儿ＩｇＡ肾病有显著的超微结构特点。弥漫性ＧＢＭ变薄组（ＤＴＢＭ）占１４６％，ＧＢＭ厚度（１２９２１±２９２１）ｎｍ。局灶节段变薄组（ＦＴＢＭ）占３３３％，ＧＢＭ厚度（１７９１０±４９８０）ｎｍ。非薄ＧＢＭ组（ＮＴＧＢＭ）占５２１％，ＧＢＭ厚度（３５６６１±９６７３）ｎｍ，分别与前两组相比，均有显著性差异（Ｐ均＜００１，ｔ＝６７３）。结论ＩｇＡ肾病可有不同程度的ＧＢＭ变薄，其厚度在２３０ｎｍ以下，范围在５０％以上，可定义为ＤＴＢＭ型；范围在２０％～５０％之间，可定义为ＦＴＢＭ型。血尿可能与ＧＢＭ变薄有关，但不影响预后；蛋白尿与ＧＢＭ变薄无关，而与ＧＢＭ病损破坏及节段足突融合有关，可能为影响预后的重要因素。

Objective In order to study the relationship between clinic and pathology. Methods 48 children with IgA nephropathy (IgAN) were observed clinically and pathologically,meanwhile thickness of the GBM was measured.According to the degrees and ranges of GBM thinning all patients were divided into three groups. Results GBM thinning was found in children with primary IgAN.If the thickness of GBM was less than 230nm and the range of GBM thinning was more than 50%,defined as diffuse thin GBM type (DTBM).If the range of GBM thinning was between 20%50%,it was defined as focal and segmental GBM thinning type (FTBM). Conclusion5BZIn children with primary IgAN there might be GBM thinning.The occurrence of hematuria in IgAN may relate to GBM thinning,but not effect the prognosis.Proteinuria might be in no relation to GBM thining,but the feetfusion and GBM structure damage may be the important factor of effect on prognosis.