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Inherited deficiency of C8 in a patient with recurrent meningococcal infections: Further evidence for a dysfunctional C8 molecule and nonlinkage to the HLA system

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Abstract

An adult male with recurrent meningococcal infections is reported whose serum lacked functional C8 activity but possessed antigenic C8. The addition of 1500 U of purified C8/ml of serum restored hemolytic activity to normal. Four to five times more C8 was required to restore bactericidal activity than to restore hemolytic activity. Bactericidal activity could also be restored by mixing the patient's serum with a second C8-deficient serum that lacked detectable antigenic or functional C8. The patient's serum contained bactericidal antibody for groups A, B, C, and Y meningococci and specific antibody to group Y capsular polysaccharide. There was two to three times more bactericidal antibody activity in the serum than in a pool of normal sera for the infecting strain. Family studies disclosed a sibling who was HLA identical to the patient but whose serum contained normal amounts of total hemolytic and C8 functional activity.

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Authors and Affiliations

  1. Evans Memorial Department of Clinical Research and Department of Medicine, Boston University Medical Center, 02118, Boston, Massachusetts

    P. Densen & R. A. Clark

  2. Laboratory of Clinical Investigation, National Institute of Allergy and Infectious Diseases, National Institutes of Health, 20205, Bethesda, Maryland

    E. J. Brown & M. M. Frank

  3. Memorial Sloan-Kettering Cancer Center, 10021, New York, New York

    G. J. O'Neill

  4. Centro Trasfusionale e di Immunologia dei Trapianti, Ospedale Policlinico, Milano, Italy

    F. Tedesco

  5. Department of Medicine, U.S. Public Health Service Hospital, 02135, Boston, Massachusetts

    D. Webb

  6. Department of Medicine, Mountainside Hospital, 07042, Montclair, New Jersey

    J. Myers

Authors
  1. P. Densen
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  2. E. J. Brown
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  3. G. J. O'Neill
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  4. F. Tedesco
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  5. R. A. Clark
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  6. M. M. Frank
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  7. D. Webb
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  8. J. Myers
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Densen, P., Brown, E.J., O'Neill, G.J. et al. Inherited deficiency of C8 in a patient with recurrent meningococcal infections: Further evidence for a dysfunctional C8 molecule and nonlinkage to the HLA system. J Clin Immunol 3, 90–99 (1983). https://doi.org/10.1007/BF00919144

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  • DOI: https://doi.org/10.1007/BF00919144

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