C3 nephritic factor in a patient with recurrent Neisseria meningitidis infections.
A 15-year-old female experienced two systemic infections with N.meningitidis (group C and B) within a two months period. Classical as well as alternative pathway CH50 determinations on the patients serum showed no lysis. All individual complement factor concentrations, except for C3, were found to be 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 was found to convert all native C3 in normal sera within 10 minutes at 37 degrees 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 EA-C3bBb complex. The isolated factor showing the characteristics of C3 nephritic factor (C3 NeF), was unchanged in the patients serum over a ten months observation period. Circulating immune complexes (IC) could not be demonstrated by a C1q-dependent assay but the patients capacity to solubilize preformed IC in vitro was virtually abolished. The patient had no signs of renal disease or lipodystrophy.
D000293:Adolescent; D003176:Complement C3 / Q000172:deficiency / Q000378:metabolism; D003178:Complement C3 Nephritic Factor / Q000097:blood*; D050577:Complement C3-C5 Convertases / Q000097:blood; D011415:Complement Factor B / Q000097:blood; D003169:Complement Inactivator Proteins / Q000097:blood*; D003170:Complement Pathway, Alternative; D005260:Female; D006801:Humans; D007074:Immunoglobulin G; D008585:Meningitis, Meningococcal / Q000097:blood / Q000276:immunology*
D003176:Complement C3; D003178:Complement C3 Nephritic Factor; D003169:Complement Inactivator Proteins; D007074:Immunoglobulin G; D050577:Complement C3-C5 Convertases; D011415:Complement Factor B