C3 glomerulonephritis in 7 children

Abstract

Objective To analyze the clinical characteristics of 7 pediatric patients with C3 glomerulonephritis(C3-GN). Methods The clinical manifestations, pathological features, therapies, prognosis of patients from Jun.2006 to Nov.2011 were analyzed retrospectively. Results All of the patients were presented as acute nephritic syndrome, and 4 patients with macroscopic hematuria, other 3 cases with severe proteinuria.Five patients with eyelid edema.All the patients showed decreased level of serum complement C3, while serum complement C4 was normal.Investigations showed elevation of 24 h urine protein, 5 patients with elevated antistreptolysin O titers.Three patients with renal dysfunction.Isolated C3 deposition in mesangial and endothelial areas(+ + -+ + + ) was confirmed by immunofluorescence.Light microscope revealed membrane proliferative glomerulonephritis and mesangial proliferative glomerulonephritis.Electron microscope showed swelling and hyperplasia of endothelial cells without electron-dense deposition or podocyte foot fusion.Based on conventional treatment, administration of immunosuppressant was performed in 3 patients with severe pathological changes.After a follow-up of 2 months to 5 years, the prognosis seems to be benign. Conclusions Children with C3-GN are usually presented as acute nephritic syndrome, characterized by isolate C3 deposition in immunofluorescence. Electron microscope showed lesion of endothelial cells, while no electron-dense deposits in mesangial and endothelial areas. The mechanism may be associated with dysregulation of alternative complement pathway, and seems had a good short-term prognosis. Key words: C3 glomerulonephritis; Clinicopathologic feature; Prognosis; Child