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目的 :探讨小儿遗传性肾炎 (Alport’ssyndrome,AS)的临床病理尤其是超微病理特征和鉴别诊断。 方法 :收集我科小儿肾活检标本 82 0例 ,对其中诊断为AS的 7例肾活检进行了光镜、特殊染色 (PAS、PASM和Masson染色 )、免疫组化 (IgA、IgM、IgG和补体C3 )和电镜的系统观察 ,着重对其超微病理进行了详尽的观测 ,并结合临床病史进行了分析。结果 :7例小儿AS的肾活检光镜下主要表现为局灶性系膜细胞增生 ,间质有泡沫细胞形成。电镜下小球基膜可见增厚、变薄及两者相间改变 ,见特征性的基膜板层状分裂、蓝编状及花纹状改变 ,其内常有圆形的微粒 ,小球内未见电子致密物沉着 ,免疫组化小球内未见明确的免疫复合物沉着。结论 :电镜是诊断AS的重要手段 ,AS的诊断主要通过电镜 ,但必须强调要与常规的病理组织学、免疫组化 ,尤其是临床病史紧密结合方可确诊。
Objective: To investigate the clinicopathological features and differential diagnosis of children with hereditary nephritis (Alport’ssyndrome, AS). Methods: 82 cases of pediatric renal biopsy specimens collected from our department were examined by light microscopy, special staining (PAS, PASM and Masson staining), immunohistochemistry (IgA, IgM, IgG and complement C3) and electron microscopy of the system observation, focusing on its superficial pathology conducted a detailed observation, combined with clinical history were analyzed. Results: Kidney biopsy in 7 pediatric AS patients showed focal mesangial cell proliferation and foam cell formation in the stroma. Under the electron microscope, the basement membrane of the globules shows thickening, thinning and the change between the two. See the characteristic basement membrane lamellar division, the blue-knit pattern and the pattern change, which usually have round particles, See electronic dense sedimentation, no clear immunocomplex immune complex within the immune complex complex. Conclusion: Electron microscopy is an important method for the diagnosis of AS. The diagnosis of AS is mainly through electron microscopy. However, it must be emphasized that it should be confirmed with routine histopathology, immunohistochemistry, especially the clinical history.