C3 NEPHRITIC FACTOR IN A PATIENT WITH RECURRENT NEISSERIA MENINGITIDIS INFECTIONS
- 15 August 2009
- journal article
- research article
- Published by Wiley in Acta Pathologica Microbiologica Scandinavica Series C: Immunology
- Vol. 92C (1-6) , 341-349
- https://doi.org/10.1111/j.1699-0463.1984.tb00098.x
Abstract
A 15 yr old female experienced 2 systemic infections with N. meningitidis (group C and B) within a 2 mo. period. Classical and alternative pathway total hemolytic complement determinations on her serum showed no lysis. All individual complement factor concentrations, except for C3, were within the reference area. Crossed immunoelectrophoretic analysis of C3 revealed no demonstrable native C3. The patient had normal levels of C3c and a markedly elevated C3d concentration. Serum from the patient converted all native C3 in normal sera within 10 min at 37.degree. C. The active converting principle, present in the IgG fraction, activated C3 in C4-depleted serum and had a dose-dependent stabilizing effect on the erythrocyte-antibody-C3bBb complex. The isolated factor showing the characteristics of C3 nephritis factor was unchanged in her serum over a 10 mo. observation period. Circulating immune complexes (IC) could not be demonstrated by a C1q-dependent assay but the patient''s capacity to solubilize performed IC in vitro was virtually abolished. The patient had no signs of renal disease of lipodystrophy.Keywords
This publication has 32 references indexed in Scilit:
- Meningococcal meningitis in a woman with inherited deficiency of the ninth component of complementClinical Immunology and Immunopathology, 1983
- Recurrent Bacterial Infections Associated with C3 Nephritic Factor and HypocomplementemiaNew England Journal of Medicine, 1983
- A standardized method for quantitating the complement-mediated immune complex solubilizing capacity of human serumJournal of Immunological Methods, 1983
- Prevalence of Congenital or Acquired Complement Deficiency in Patients with Sporadic Meningococcal DiseaseNew England Journal of Medicine, 1983
- The normal occurrence of two molecular forms of the eight complement component (C8) and their concentrations in a family with C8 deficiencyEuropean Journal of Clinical Investigation, 1983
- Double-decker rocket immunoelectrophoresis for direct quantitation of complement C3 split products with C3d specificities in plasmaJournal of Immunological Methods, 1981
- Metabolic Studies of the Third Component of Complement and the Glycine-Rich Beta Glycoprotein in Patients with HypocomplementemiaJournal of Clinical Investigation, 1974
- Increased Susceptibility to Infection in a Patient with Type II Essential Hypercatabolism of C3New England Journal of Medicine, 1973
- HOMOZYGOUS DEFICIENCY OF C3 IN A PATIENT WITH REPEATED INFECTIONSThe Lancet, 1972
- Inactivator of the Third Component of Complement as an Inhibitor in the Properdin PathwayProceedings of the National Academy of Sciences, 1972