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目的:阐明特发性膜性肾病(IMN)的分子病理特征,探讨分子病理分型在IMN发病机制研究以及指导临床治疗和判断预后中的作用。 方法:21例经肾穿刺活检及临床诊断为IMN患者肾组织。运用免疫荧光双套色法,在激光共聚焦荧光显微镜下观察上皮侧免疫复合物沉积构象、C5b-9和WT1在肾小球基膜侧的分布,并对患者肾小球足细胞进行密度定量分析。运用免疫组化方法观察肾小球中IgG亚型的分布。 结果:①21例患者在共聚焦显微镜下可分为两类:基膜增生反应型和非增生反应型。②不同分型IgG亚型沉积不同,IgG4沉积强度在基膜增生反应型明显较非增生反应型强。③不同亚型C5b-9的沉积阳性率不同,在基膜增生反应型明显高于基膜非增生反应型,且前者足细胞密度明显降低。 结论:根据上皮侧免疫复合物的构象以及基膜变化的不同将IMN分为基膜增生反应与非增生反应两种类型。这两种类型IMN在分子病理改变上存在明显差异。这些分子标志物的不同,除了提示它们在发病机制上有所不同外,有可能为指导临床治疗和判断预后提供帮助。
Objective: To elucidate the molecular pathological features of idiopathic membranous nephropathy (IMN) and to explore the role of molecular pathological typing in studying the pathogenesis of IMN and guiding the clinical treatment and prognosis. Methods: Twenty-one cases of renal biopsy and clinical diagnosis of renal tissue in patients with IMN. Immunofluorescence double-coat method was used to observe the deposition conformation of epithelial side immunocomplex and the distribution of C5b-9 and WT1 in glomerular basement membrane by confocal laser scanning microscope. The density of glomerular podocyte was quantitatively analyzed . Immunohistochemistry was used to observe the distribution of IgG subtypes in glomeruli. Results: ①21 patients under confocal microscope can be divided into two categories: basement membrane hyperplasia and non-proliferative response. ② The deposition of different subtypes of IgG subtypes is different, IgG4 deposition intensity in the basement membrane hyperplasia reaction was significantly stronger than non-proliferative response. ③ The positive rates of C5b-9 in different subtypes were different, which were significantly higher in basement membrane hyperplasia than in non-hyperplastic basal lamina, and the former had lower podocyte density. CONCLUSION: According to the conformation of epithelial side immune complex and the change of basement membrane, IMN can be divided into two types: basement membrane hyperplasia and non-hyperplasia. These two types of IMN in molecular pathology there are significant differences. The difference of these molecular markers not only suggests that they may be different in pathogenesis, but also may help guide the clinical treatment and prognosis.