【摘要】 目的 :探讨儿童C1q肾病临床病理特征及诊断与治疗。方法 :分析 8例C1q肾病患儿临床病理特点及激素或免疫抑制剂治疗效应 ,并与同期 77例原发性NS患儿作比较。结果 :8例C1q肾病患儿临床上大多表现为原发性NS(6例 ) ,仅 2例表现为肾炎综合征和单纯性血尿。LM主要包括MC(3例 )、MsPGN(2例 )、FSGS(2例 )和ECPGN(1例 )。IF显示明显的系膜区C1q沉积 ,伴或不伴有Ig和补体沉积。EM检查仅 1例有系膜区和内皮下电子致密物沉积。与原发性NS相比 ,6例表现为NS的C1q肾病患儿对泼尼松初次治疗产生耐药的相对危险度为 2 1(P <0 .0 0 1) ,但对免疫抑制剂治疗均敏感。结论 :儿童C1q肾病临床上以对激素耐药的NS为常见表现 ,IF是其主要诊断依据 ,使用免疫抑制剂治疗有效更多还原

【Abstract】 Objective: To evaluate the clinicopathological characteristics and the efficacy of glucocorticoids in children with C 1q nephropathy. Methods: Clinical manifestation, pathologic features and response to prednisone therapy were compared between 8 cases of C 1q nephropathy and 77 cases of idiopathic nephrotic syndrome(INS) in children. Results: 6 of 8 cases with C 1q nephropathy presented primarily with INS. Another 2 cases presented respectively with glomerulonephritis and simple hematuria. The pathologic types included MC(3 cases), MsPGN(2 cases), FSGS(2 cases) and ECPGN(1 case) under light microscopic examination. The prominent imunofluorescent features was the presence of bright mesangial deposition of C 1q , with and without other immune reactants. Only one case had mesangial and subepithelial electron-dense deposits under electron microscope. In 6 cases of C 1q nephropathy who showed INS, the relative risk of not responding to the initial course of steroid therapy was 21(P<0.001)compared with 88 cases of INS, but they were all sensitive to immunosuppressions. Conclusion: C 1q nephropathy is a distinct clinicopathologic condition with immunofluoresence as the main clue of diagnosis. It is generally presented as steroid-resistant nephrotic syndrome but has a good response to immunosuppression in children.更多还原