作者: Ashwani Kumar , Raja Ramachandran , Amit Rawat , Reena Das , Charan S Rayat
DOI: 10.1093/CKJ/SFZ135
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摘要: Background Complement 3 glomerulopathy (C3G) results from dysfunction of the alternative complement pathway (ACP). No data are available on post-transplant C3G in South Asia. Methods In this study, renal allograft biopsies patients performed 2012 to 2017 were analysed for ACP functional assay (APFA), serum levels, factor H (CFH), B (CFB) and autoantibodies CFH CFB. Limited genetic screening CFH/CFHR5 genes was carried out. All study also followed up. Results A total 21 cases included, which 11 had native disease (that is, recurrent C3G). Of these cases, 7 presented with 4 proteinuria dysfunction. Early recurrence (<1 month) noted six patients, whereas five occurred within 8-17 months transplant. Biopsies showed mild focal mesangial expansion or without endocapillary proliferation thrombotic microangiopathy. Rejection patients. APFA/C3 levels low all cases. Serum [dense deposit (DDD), 44%; C3 glomerulonephritis (C3GN), 25%], CFB normal. Autoantibodies CFH, nephritic present 11, 0 44% DDD respectively, 17, 17 33% C3GN respectively. Genetic analysis revealed only non-pathogenic gene variants (93%). novel mutation found. At follow-up (140 months), stable graft 28% progressive failure 19%, loss 34%, 19% died. Conclusion Post-transplant can and/or proteinuria. Subtle histological findings demand careful interpretation immunofluorescence results. regulatory proteins common, no has been found limited workup. Clinical outcome is poor.