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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146%,GBM厚度(12921±2921)nm。局灶节段变薄组(FTBM)占333%,GBM厚度(17910±4980)nm。非薄GBM组(NTGBM)占521%,GBM厚度(35661±9673)nm,分别与前两组相比,均有显著性差异(P均<001,t=673)。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 To further investigate the relationship between clinical and pathological changes of IgA nephropathy. Methods According to the range of glomerular basement membrane (GBM) thinning, 48 cases of primary IgA nephropathy in children were divided into three groups. The clinical, pathological and ultrastructural changes were systematically observed and the thickness of GBM was measured . Results Pediatric IgA nephropathy has significant ultrastructural features. Diffuse GBM thinning group (DTBM) accounted for 14 6%, GBM thickness (129 21 ± 29 21) nm. Focal segmental thinning group (FTBM) accounted for 333%, GBM thickness (17910 ± 4980) nm. NTGBM accounted for 52.1% and GBM thickness 356.61 ± 96.73 nm, respectively, which were significantly different from those of the former two groups (P <001, t = 6 73). Conclusion IgA nephropathy may have different degrees of GBM thinning. The thickness of the IgA nephropathy is below 230 nm with a range of more than 50%, which can be defined as DTBM type. The range of 20% ~ 50% is defined as FTBM type. Hematuria may be related to GBM thinning, but does not affect the prognosis; proteinuria has nothing to do with the GBM thinning, and GBM damage and segment foot process fusion may be an important factor affecting the prognosis.