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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
Objective: To investigate the clinicopathological features, diagnosis and treatment of C1q nephropathy in children. Methods: The clinical and pathological characteristics of 8 children with C1q nephropathy and the therapeutic effect of hormone or immunosuppressive agents were analyzed and compared with 77 children with primary NS. Results: Most of 8 children with C1q nephropathy showed primary NS (6 cases), only 2 cases showed nephritic syndrome and simple hematuria. LM mainly includes MC (3 cases), MsPGN (2 cases), FSGS (2 cases) and ECPGN (1 case). IF showed significant mesangial Clq deposition, with or without Ig and complement deposition. In the EM examination, only 1 patient had a mesangial area and a subendothelial electron dense deposit. Compared with primary NS, the relative risk of resistance to primary prednisone in 21 children with C1q nephropathy who were NS was 21 (P <0.001), but the immunosuppressive therapy Are sensitive. Conclusion: Children with C1q nephropathy clinically hormone-resistant NS is a common manifestation, IF is the main basis for diagnosis, the use of immunosuppressive agents is effective