Recurrent bacterial meningitis in patients with genetic defects of terminal complement components.
Isolated genetic deficiencies of complement components in man are rare. We describe two kindreds with inborn deficiencies of either C5 or C6 in which both propositi presented with recurrent bacterial meningitis. Neisseria meningitidis was isolated from the cerebrospinal fluid of the C5-deficient patient and bactericidal activity against his autologous meningococcus was absent from whole fresh patients' serum despite a rising titre of complement-fixing antibody. The stimulated movement of normal leucocytes was impaired in the presence of C5-deficient serum but not in the presence of C6-deficient serum; neither deficiency reduced significantly the complement-dependent opsonization of Saccharomyces cerevisiae. HLA typing and complement component phenotyping showed no segregation with the complement defect in either the C5- or C6-deficient families. Normal individuals and apparent heterozygotes with approximately half the normal levels of the relevant component were found in both families, in keeping with an autosomal codominant inheritance of the defects.
D000328:Adult; D000907:Antibodies, Bacterial / Q000032:analysis; D002465:Cell Movement; D003182:Complement C5 / Q000172:deficiency*; D003183:Complement C6 / Q000172:deficiency*; D003170:Complement Pathway, Alternative; D006801:Humans; D008297:Male; D008585:Meningitis, Meningococcal / Q000276:immunology*; D009504:Neutrophils / Q000276:immunology; D010375:Pedigree
D000907:Antibodies, Bacterial; D003182:Complement C5; D003183:Complement C6