Absence of the sixth component of complement in a patient with repeated episodes of meningococcal meningitis

• Published in: The Journal of pediatrics Vol. 89; no. 1; p. 42
• Main Authors: Lim, D, Gewurz, A, Lint, T F, Ghaze, M, Sepheri, B, Gewurz, H
• Format: Journal Article
• Language: English
• Published: United States 01-07-1976
• Subjects: Child; Complement C6 - deficiency; Complement System Proteins - deficiency; Hemolysis; Humans; Male; Meningitis, Meningococcal - immunology; Neisseria meningitidis - immunology
• ISSN: 0022-3476
• DOI: 10.1016/S0022-3476(76)80924-9

A previously healthy 6-year-old boy who presented with meningococcal meningitis responded favorably to ampicillin, but suffered two and possibly three repeat attacks in the ensuing month. No abnormality of the otolarynx, skin, or neuroskeleton was found. The infecting strain, Neisseria meningitidis, Group Y, Type IV, was sensitive to the therapeutic agents used, and antibiotic levels were adequate. Serum bactericidal antibody titers against autologous meningococci were high. Serum complement hemolytic bactericidal activities, however, were entirely lacking, and this was attributable to a complete deficiency of C6. Measurements of the remaining complement components, C-dependent chemotaxis and opsonization, neutrophil function, specific immunity, and the coagulation system, were normal. The parents had half-normal C6 levels. Recurrence of meningitis in this patient supports the concept that complement plays a role in resistance to certain microorganisms and emphasizes the need for complete evaluation of the complement system in individuals with unexplained repeated infections.