Abstract
Hypocomplementemia with circulating C3NeF occurs in membrano-proliferative glomerulonephritis and occasionally in acute post-streptococcal and lupus nephritis. A 7 year old boy with idiopathic rapidly progressive glomerulonephritis (IRPGN), with a serum C3 level of 16 mg/dl, a circulating 7S factor splitting C3 with the classical pathway blocked (C3NeF), and circulating IgG-containing complexes reactive with Clq, is reported. Crescents were present in 50% of the glomeruli. As in normocomplementemic IRPGN, ultrastructure showed subepithelial deposits located exclusively in that part of the basement membrane in apposition to the mesangium (capillary waist) and composed, by immunofluorescence, of C3, C5, and properdin. Following the initiation of therapy with corticosteroid, renal function greatly improved with concurrent fall in the levels of C3NeF and of Clq reactive complexes and rise in C3 concentration. At 8 months, 45% of glomeruli were globally sclerotic and IgG, C3, C5 and properdin could not be demonstrated. The observations emphasize that C3NeF can be present in many types of GN and that hypocomplementemia in RPGN is not necessarily indicative of a streptococcal origin.
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Davis, C., Wyatt, R., Mcadams, A. et al. 1057 IDIOPATHIC RAPIDLY PROGRESSIVE GLOMERULONEPHRITIS WITH C3NeF AND HYPOCOMPLEMENTEMIA. Pediatr Res 12 (Suppl 4), 540 (1978). https://doi.org/10.1203/00006450-197804001-01063
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DOI: https://doi.org/10.1203/00006450-197804001-01063