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目的分析孤立性C3沉积系膜增生性肾小球肾炎的临床特点。方法对5例孤立性C3沉积系膜增生性肾小球肾炎患儿进行回顾性分析。结果临床表现:均有浮肿,镜下或肉眼血尿,伴蛋白尿,且血压增高;血清补体C3均有明显下降,且恢复较慢;4例24 h尿蛋白定量大于2 g/d,但未达到肾病综合征标准;3例患儿出现肾功能不全;病理改变:(1)光镜:表现为系膜增生性改变,伴有毛细血管内增生;(2)免疫荧光:仅见C3沿毛细血管袢及系膜区沉积,强度为(+~);(3)电镜:2例基底膜见电子致密物的沉积。结论孤立性C3沉积系膜增生性肾小球肾炎临床表现为急性肾炎综合征,免疫荧光仅见C3沉积为其特点,该病短期预后较好,免疫抑制剂有一定的疗效。
Objective To analyze the clinical features of isolated C3 sedimentary mesangial proliferative glomerulonephritis. Methods A retrospective analysis was performed on 5 children with isolated C3 sedimentary mesangial proliferative glomerulonephritis. Results The clinical manifestations were edema, microscopic or gross hematuria, with proteinuria, and elevated blood pressure. Serum complement C3 was significantly decreased and recovered slowly; 4 cases of 24 h urinary protein was greater than 2 g / d, but not 3 cases of renal insufficiency; pathological changes: (1) light microscopy: showed mesangial proliferative changes, accompanied by capillary proliferation; (2) immunofluorescence: only see C3 along the capillaries袢 and mesangial deposition, intensity (+ ~); (3) electron microscopy: 2 cases of basement membrane deposition of electron dense material. Conclusion The clinical manifestations of solitary glomerulonephritis with isolated mesangial proliferative glomerulonephritis are acute nephritic syndrome. The immunofluorescence is characterized by C3 deposition only. The short-term prognosis of the disease is good, and the immunosuppressive agents have a certain curative effect.