Abstract

In this review article, the author initially defines C3 glomerulopathy and provides new insights into the pathophysiology of this disease. This is followed by a description of the pathohistological and clinical presentation. Recommendations for the diagnostic work-up are reviewed as well as therapeutic options in children with C3 glomerulopathy. Finally, the prognosis in native and transplanted kidneys is discussed.

Key words: C3 glomerulopathy, dense deposit disease, C3 glomerulonephritis, complement, children.