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目的探讨电子致密物沉积病(dense deposit disease,DDD)临床、肾脏病理及治疗方法。方法回顾10年中经肾穿刺活检术确诊为DDD的5例患儿病案资料,同时复习国内外相关文献,整合分析。结果 5例DDD患儿占同期肾活检的0.3%。2例临床表现为肾病综合征,2例为血尿和蛋白尿,1例为肾炎综合征;3例C3降低;4例光镜表现系膜增生性肾小球肾炎,1例表现为膜增生性肾小球肾炎。所有病例显示C3免疫荧光沉积,3例伴有免疫球蛋白成分。电镜下5例患儿均可见特征性飘带样电子致密物沉积在肾小球基底膜,部分患儿伴系膜区、内皮下、上皮下沉积。结论 DDD临床和光镜病理表现多样,无特异性。即使蛋白尿程度不重、血清C3正常者也需警愓DDD。诊断依赖电镜检查。
Objective To investigate the clinical, renal pathology and treatment of dense deposit disease (DDD). Methods The data of 5 cases diagnosed as DDD by renal biopsy during the past 10 years were retrospectively reviewed. At the same time, relevant literature at home and abroad was reviewed and analyzed. Results 5 DDD children accounted for 0.3% of the same period of renal biopsy. 2 cases of nephrotic syndrome, 2 cases of hematuria and proteinuria, 1 case of nephritic syndrome; 3 cases of C3 decreased; 4 cases of mesangial proliferative glomerulonephritis, 1 case of membrane proliferative glomerulus nephritis. All cases showed C3 immunofluorescence and 3 cases had immunoglobulin components. Electron microscope, 5 cases of children can be seen characteristic streamers like electron density deposited in the glomerular basement membrane, some children with mesangial area, subcutaneous, subepithelial deposition. Conclusion DDD clinical and light microscope pathological performance of diverse, non-specific. Even if the degree of proteinuria is not heavy, normal serum C3 also need to police DDD. Diagnosis depends on electron microscopy.