Pediatric C3 Glomerulonephritis:Six Cases Report And Literature Review

Objective: To analyze the clinical manifestations,pathological changes and prognosis of C3 glomerulonephritis(C3GN)in children.Methods: This retrospective study used the data of patients with C3 GN from the Children’ s Hospital of CQMU during Mar.2014~ Sept.2018.And a review of C3 GN children patients admitted in the Chinese literature together with our own cases has been performed.Results: The 6 patients were male and 5 of them presented as acute nephritic syndrome.2 patients had the history of preceding infection.Main clinical manifestations included macroscopic hematuria and edema.They all had the low level of serum complement C3.Light microscope showed mesangial proliferative glomerulonephritis,endocapillary proliferative glomerulonephritis,membranoproliferative glomerulonephritis and diffuse proliferative glomerulonephritis.Electron microscope showed 4 cases with electron-dense deposition in subepithelial area and 2 cases with electron-dense deposition in subendothelial area.Immunohistology revealed dominant C3 deposition in mesangial and/or capillary walls.The pathogenicity is unclear.Based on symptomatic and steroid combined with immune inhibitor treatment,and after a follow-up of 5 months to 4 years,the prognosis seems to be benign.Above all the 22 cases,the ratio of male to female was 15:7,the main manifestations were hematuria(100%),proteinuria(86%),edema(64%).And most presented with reduced complement C3(95%).Mesangial proliferative glomerulonephritis(50%)was the general pattern of injury,followed by membranoproliferative glomerulonephritis(27%).The short-term prognosis is benign.Conclusion: The dysregulation of alternative complement pathway is related to C3 GN.Streptococcal infection may play an important role in the pathogenesis of C3 GN.Sometimes it is difficult to differentiate C3 GN from post-infectious glomerulonephritis(PIGN)only by clinical and pathological findings.Complement and genetic testing are recommended to confirm the diagnosis.The clinical manifestations of pediatric C3 GN vary a lot and mesangial proliferative glomerulonephritis is the most common patterns of injury.The short-term prognosis is benign.