Recurrent Meningococcal Meningitis with Absence of the Sixth Component of Complement: An Evaluation of Underlying Immunologic Mechanisms
1 Cellular Immunobiology Unit of the Tumor Institute, Departments of Pediatrics and Microbiology, and Comprehensive Cancer Center, and Department of Medicine, University of Alabama in Birmingham, Birmingham, Alabama and the Department of Pediatrics, National Jewish Hospital and Research Center, University of Colorado School of Medicine, Denver, Colorado
A 5
-year-old black girl with recurrent meningococcal meningitis and absence of the sixth component of complement (C6) is reported. To explore the pathogenesis of recurrent neisserial infections in C6 deficiency, a detailed analysis of her immune competence was conducted. Her serum had normal chemotactic, opsonic, alternative complement pathway, and specific antibody activity, but lacked complement-mediated bacteriolytic activity. In addition, her C6-deficient serum was indistinguishable from normal serum in a complement-dependent assay of phagocyte bactericidal activity. Absent bacteriolysis remains the only consistent defect associated with recurrent neisserial infections and absence of one of the late-acting complement components.
Accepted on January 8, 1979
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