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本文首次报告上海儿科所见6例与抗中性粒细胞胞浆抗体(ANCA)相关的肾小球疾病。其中原发性肾小球疾病为急进性肾炎、肾炎综合征,总性肾小球肾炎和肾病综合征各1例。继发性为狼疮性肾炎和紫癜性肾炎各1例。血浆ANCA测定均为C-ANCA阳性。结合病例对与ANCA相关的肾小球疾病发病机理、诊断及治疗进行探讨。其中1例急进性肾炎在血液透析基础上使用激素及CTX冲击等治疗获愈。
This is the first report of 6 cases of glomerular diseases associated with anti-neutrophil cytoplasmic antibodies (ANCA) seen in Shanghai Pediatric. The primary glomerular diseases were acute nephritis, nephritic syndrome, total glomerulonephritis and nephrotic syndrome in 1 case. Secondary to lupus nephritis and purpuric nephritis in 1 case. Plasma ANCA measurements were positive for C-ANCA. Combined with cases of ANCA-related glomerular disease pathogenesis, diagnosis and treatment are discussed. One case of acute nephritis hemodialysis on the basis of the use of hormones and CTX impact treatment and more.