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.%Objectives To discuss the clinical, pathological features and treatment in 5 dense deposit disease (DDD) patients. Methods The data of 5 biopsy-confirmed DDD patients were analyzed and related literatures were reviewed. Results Five patients (3 males and 2 females) were diagnosed with DDD and it accounted for 0.3% of renal biopsy performed in the same period. Two cases manifested as nephrotic syndrome, 2 cases as hematuria andproteinuria and 1 case as nephritic syndrome. Serum C3 was reduced in 3 cases. Four cases had mesangial proliferative glomerulonephritis and only 1 case had membrane proliferative glomerulonephritis. Immunofluorescence showed predominant C3 deposition in all cases and 3 patients also had immunoglobulin deposits. All 5 cases had typical ribbon-like dense deposits in glomerular basement menbrane (GBM) under electron microscopy and some cases had mesangial, endothelial and subepithelial deposition. Conclusions The clinical and microscopic pathological manifestations of DDD are variable and non-specific, and may include minor proteinuria and even normal level of serum C3. The diagnosis of DDD still relies on the examination by electron microscopy.
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