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  • Title: Membranoproliferative glomerulonephritis with C3NeF and genetic complement dysregulation.
    Author: Leroy V, Fremeaux-Bacchi V, Peuchmaur M, Baudouin V, Deschênes G, Macher MA, Loirat C.
    Journal: Pediatr Nephrol; 2011 Mar; 26(3):419-24. PubMed ID: 21188423.
    Abstract:
    The development of membranoproliferative glomerulonephritis (MPGN) is associated with uncontrolled activation of the complement alternative pathway. This dysregulation is related either to C3 nephritic factor (C3NeF), an auto-antibody directed against the alternative C3 convertase, or to homozygous loss-of-function mutation of the complement regulatory protein factor H. Heterozygous mutations in the genes coding for factor H, or for the other alternative pathway inhibitory proteins factor I and membrane cofactor protein, have recently been identified in a small number of patients with MPGN with exclusive C3 deposits. We report three hypocomplementemic children with dense deposit disease (n=1) or immune-complex-mediated MPGN type I (n=2), associated with both C3NeF activity and heterozygous mutation of factor H or factor I. These observations highlight the possible combination of genetic and acquired defect in complement control in various subtypes of MPGN, a finding that may influence the treatment strategy in some patients.
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