文本框: A CLINICOPATHOLOGIC STUDY OF C1q NEPHROPATHY IN CHILDREN Liu Jianhua, He weixun, Luo Yunjiu, Zhu Guanghua, Ren Zhide Department of Nephrology, Shanghai Children’s Hospital, China Objective: To evaluate the clinicopathologic characteristics and the effect of glucocorticoids in children with C1q nephropathy. Methods: Analyzing the clinical manifestation, and comparing pathologic features and response to prednisone therapy between 8 cases of C1q nephropathy and 77 cases of idiopathic nephrotic syndrome (INS) in children. Results: 6 of 8 cases with C1q nephropathay presented primarily with INS, another 2 cases presented respectively with glomerulonephritis and single hematuria. At the light microscopic level, the pathologic types included MC (3 cases), MsPGN (2cases), FsGs (2 cases) and ECPGN (1 case); Imunofluorescence findings showed the prominent feature in each of these patients was the presence of bright mesangial depositions of C1q, with and without other immune reactants. Only one case had identified mesangial and subepithelial electron-dense deposits at electron microscopy level. In 6 cases of C1q nephropathy, who showed INS, the relative risk of not responding to the initial course of steroid therapy was 21 (p<0.001), compared with 77 cases of INS, but they were all sensitive to the immunosuppressions. Conclusion: C1q nephropathy is a distinct clinicopathologic disease, whose diagnosis depended on the immuno-fluoresence study, ordinarily resulting in steroid-resistant nephrotic syndrome, and having a good response to the immuno-suppressions in children. 0756