Abstract
The serum of a 26-year-old black man with a recent episode of meningococcemia complicated by meningitis and arthritis was found to lack hemolytic complement activity. The sixth component of complement was not detected by functional or immunochemical assays whereas other components were normal by hemolytic assay. His fresh acute-phase serum lacked complement-mediated bactericidal activity against the homologous strain of Neisseria meningitidis, but the addition of fresh normal serum or purified C6 restored bactericidal activity as well as hemolytic activity. The absence of C6 activity could not be accounted for on the basis of an inhibitor. Opsonization and chemotaxis functioned normally. Histocompatibility typing of family members did not demonstrate evidence for genetic linkage of C6 deficiency with the major histocompatibility loci. This report represents the first published case of C6 deficiency associated with bacteremic Neisseria infections in which antimeningococcal bactericidal antibodies have been definitively demonstrated against the homologous strain in the acute phase of the illness.
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- Blatt P. M., Yount W. J., Utsinger P. D., Korn J. H., Hadler N. M., Roberts H. R. Factor XI deficiency, juvenile rheumatoid arthritis and systemic lupus erythematosus. Report of the first case. Am J Med. 1977 Aug;63(2):289–297. doi: 10.1016/0002-9343(77)90244-3. [DOI] [PubMed] [Google Scholar]
- Böyum A. Isolation of mononuclear cells and granulocytes from human blood. Isolation of monuclear cells by one centrifugation, and of granulocytes by combining centrifugation and sedimentation at 1 g. Scand J Clin Lab Invest Suppl. 1968;97:77–89. [PubMed] [Google Scholar]
- Glass D., Raum D., Balavitch D., Kagan E., Rabson A., Schur P. H., Alper C. A. Inherited deficiency of the sixth component of complement: a silent or null gene. J Immunol. 1978 Feb;120(2):538–541. [PubMed] [Google Scholar]
- Goldschneider I., Gotschlich E. C., Artenstein M. S. Human immunity to the meningococcus. I. The role of humoral antibodies. J Exp Med. 1969 Jun 1;129(6):1307–1326. doi: 10.1084/jem.129.6.1307. [DOI] [PMC free article] [PubMed] [Google Scholar]
- HAESSIG A., BOREL J. F., AMMANN P., THOENI M., BUETLER R. ESSENTIELLE HYPOKOMPLEMENTAEMIE. Pathol Microbiol (Basel) 1964;27:542–547. [PubMed] [Google Scholar]
- Jersild C., Rubinstein P., Day N. K. The HLA system and inherited deficiencies of the complement system. Transplant Rev. 1976;32:43–71. doi: 10.1111/j.1600-065x.1976.tb00228.x. [DOI] [PubMed] [Google Scholar]
- Leddy J. P., Frank M. M., Gaither T., Baum J., Klemperer M. R. Hereditary deficiency of the sixth component of complement in man. I. Immunochemical, biologic, and family studies. J Clin Invest. 1974 Feb;53(2):544–553. doi: 10.1172/JCI107588. [DOI] [PMC free article] [PubMed] [Google Scholar]
- Lee T. J., Utsinger P. D., Snyderman R., Yount W. J., Sparling P. F. Familial deficiency of the seventh component of complement associated with recurrent bacteremic infections due to Neisseria. J Infect Dis. 1978 Sep;138(3):359–368. doi: 10.1093/infdis/138.3.359. [DOI] [PubMed] [Google Scholar]
- Lim D., Gewurz A., Lint T. F., Ghaze M., Sepheri B., Gewurz H. Absence of the sixth component of complement in a patient with repeated episodes of meningococcal meningitis. J Pediatr. 1976 Jul;89(1):42–47. doi: 10.1016/s0022-3476(76)80924-9. [DOI] [PubMed] [Google Scholar]
- Mancini G., Carbonara A. O., Heremans J. F. Immunochemical quantitation of antigens by single radial immunodiffusion. Immunochemistry. 1965 Sep;2(3):235–254. doi: 10.1016/0019-2791(65)90004-2. [DOI] [PubMed] [Google Scholar]
- Mittal K. K., Wolski K. P., Lim D., Gewurz A., Gewurz H., Schmid F. R. Genetic independence between the HL--A system and deficits in the first and sixth components of complement. Tissue Antigens. 1976 Feb;7(2):97–104. doi: 10.1111/j.1399-0039.1976.tb01038.x. [DOI] [PubMed] [Google Scholar]
- Nelson R. A., Jr, Jensen J., Gigli I., Tamura N. Methods for the separation, purification and measurement of nine components of hemolytic complement in guinea-pig serum. Immunochemistry. 1966 Mar;3(2):111–135. doi: 10.1016/0019-2791(66)90292-8. [DOI] [PubMed] [Google Scholar]
- Petersen B. H., Graham J. A., Brooks G. F. Human deficiency of the eighth component of complement. The requirement of C8 for serum Neisseria gonorrhoeae bactericidal activity. J Clin Invest. 1976 Feb;57(2):283–290. doi: 10.1172/JCI108279. [DOI] [PMC free article] [PubMed] [Google Scholar]
- Pincus S. H., Klebanoff S. J. Quantitative leukocyte iodination. N Engl J Med. 1971 Apr 8;284(14):744–750. doi: 10.1056/NEJM197104082841402. [DOI] [PubMed] [Google Scholar]