RESUMO
Objective To investigate the pathogenesis of renal lesion in Fechtner syndrome . Methods Pathological characteristics of kidney tissues from Fechtner syndrome patients were explored by HE staining, immunochemistry, immunofluorescence and electron microscopy . Results Immunochemistry analysis showed that non-muscle myosin heavy chain IIA (NMMHC-IIA)was expressed in podocytes of giomeruli and distal convoluted tube, and was faintly expressed in the brush border of proximal tube . Histological examination demonstrated glomerulosclerosis and decreased expression of NMMHC-IIA in abnormal podocytes . Through standard immunofluorecence, the expression of NMMHC-IIA in patient's podocyte was higher than that in normal pedocytes . The fusion of foot process and microvillus were detected by electron microscopy . Conclusion Abnormal NMMHC-IIA aggregates in the glomeruli podocyts and foot process fusion accompanied with appearance of microvillus leads to renal lesion in Fechtuer syndrome .