Association of Homozygous C4B Deficiency with Bacterial Meningitis
- 1 September 1989
- journal article
- research article
- Published by Oxford University Press (OUP) in The Journal of Infectious Diseases
- Vol. 160 (3), 448-451
- https://doi.org/10.1093/infdis/160.3.448
Abstract
The fourth component of complement (C4) is encoded by two separate but linked loci (C4A and C4B), each of which produces functionally active C4. Although C4A and C4B share certain antigenic and functional characteristics that identify them as C4, they differ with respect to other structural and functional properties. For example, C4B possesses four times the functional hemolytic activity of C4A. This suggests that homozygous deficiency of C4B might be associated with an increased susceptibility to infection. Forty-six children with bacterial meningitis were examined. Of these, 5 (10.9%) were homozygous deficient for C4B versus 7 (3.1%) of 223 controls (P = 0.038). There was no relation between the prevalence of heterozygous C4B deficiency and meningitis or between the prevalence of either homozygous or heterozygous C4A deficiency and meningitis. These results suggest that homozygous C4B deficiency is a relatively common immunodeficiency disorder that is clinically significant and predisposes children to bacterial meningitis.Keywords
This publication has 10 references indexed in Scilit:
- SCREENING FOR COMPLEMENT DEFICIENCIES IN UNSELECTED PATIENTS WITH MENINGITIS1987
- COMBINED FAMILIAL C7 AND C4B DEFICIENCY IN AN ADULT WITH MENINGOCOCCAL DISEASE1987
- Complement phenotypes in glomerulonephritis: Increased frequency of homozygous null C4 phenotypes in IgA nephropathy and Henoch-Schönlein purpuraKidney International, 1984
- Factor I Deficiency and C3 Nephritic Factor: Immunochemical Findings and Association with Neisseria meningitidis Infection in Two PatientsScandinavian Journal of Immunology, 1984
- A comparison of the properties of two classes, C4A and C4B, of the human complement component C4.The EMBO Journal, 1984
- The molecular basis for the difference in immune hemolysis activity of the Chido and Rodgers isotypes of human complement component C4.The Journal of Immunology, 1984
- Inherited structural polymorphism of the fourth component of human complement.Proceedings of the National Academy of Sciences, 1980
- Two HLA-linked loci controlling the fourth component of human complement.Proceedings of the National Academy of Sciences, 1978
- The biochemistry of complementNature, 1978
- Inherited structural polymorphism in human C2: evidence for genetic linkage between C2 and Bf.The Journal of Experimental Medicine, 1976