Literature DB >> 7389212

Recurrent bacterial meningitis in patients with genetic defects of terminal complement components.

M R Haeney, R A Thompson, J Faulkner, P Mackintosh, A P Ball.   

Abstract

Isolated genetic deficiencies of complement components in man are rare. We describe two kindreds with inborn deficiencies of either C5 or C6 in which both propositi presented with recurrent bacterial meningitis. Neisseria meningitidis was isolated from the cerebrospinal fluid of the C5-deficient patient and bactericidal activity against his autologous meningococcus was absent from whole fresh patients' serum despite a rising titre of complement-fixing antibody. The stimulated movement of normal leucocytes was impaired in the presence of C5-deficient serum but not in the presence of C6-deficient serum; neither deficiency reduced significantly the complement-dependent opsonization of Saccharomyces cerevisiae. HLA typing and complement component phenotyping showed no segregation with the complement defect in either the C5- or C6-deficient families. Normal individuals and apparent heterozygotes with approximately half the normal levels of the relevant component were found in both families, in keeping with an autosomal codominant inheritance of the defects.

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Year:  1980        PMID: 7389212      PMCID: PMC1536945     

Source DB:  PubMed          Journal:  Clin Exp Immunol        ISSN: 0009-9104            Impact factor:   4.330


  29 in total

1.  Hereditary deficiency of the second component of complement (C'2) in man.

Authors:  M R Klemperer; H C Woodworth; F S Rosen; K F Austen
Journal:  J Clin Invest       Date:  1966-06       Impact factor: 14.808

2.  Homozygous deficiency of C3 in a patient with repeated infections.

Authors:  C A Alper; H R Colten; F S Rosen; A R Rabson; G M Macnab; J S Gear
Journal:  Lancet       Date:  1972-12-02       Impact factor: 79.321

3.  Radioactive single radial diffusion: a method for increasing the sensitivity of immunochemical quantification of proteins in agar gel.

Authors:  D S Rowe
Journal:  Bull World Health Organ       Date:  1969-04       Impact factor: 9.408

4.  C3 inactivating factor in the serum of a patient with chronic hypocomplementaemic proliferative glomerulo-nephritis.

Authors:  R A Thompson
Journal:  Immunology       Date:  1972-01       Impact factor: 7.397

5.  Quantitative leukocyte iodination.

Authors:  S H Pincus; S J Klebanoff
Journal:  N Engl J Med       Date:  1971-04-08       Impact factor: 91.245

6.  Hereditary deficiency of the sixth component of complement in man. I. Immunochemical, biologic, and family studies.

Authors:  J P Leddy; M M Frank; T Gaither; J Baum; M R Klemperer
Journal:  J Clin Invest       Date:  1974-02       Impact factor: 14.808

7.  Genetically determined heterogeneity of the C1 esterase inhibitor in patients with hereditary angioneurotic edema.

Authors:  F S Rosen; C A Alper; J Pensky; M R Klemperer; V H Donaldson
Journal:  J Clin Invest       Date:  1971-10       Impact factor: 14.808

8.  Reactive lysis: the complement-mediated lysis of unsensitized cells. I. The characterization of the indicator factor and its identification as C7.

Authors:  R A Thompson; P J Lachmann
Journal:  J Exp Med       Date:  1970-04-01       Impact factor: 14.307

9.  Deficiency of C1r in human serum. Effects on the structure and function of macromolecular C1.

Authors:  R J Pickering; G B Naff; R M Stroud; R A Good; H Gewurz
Journal:  J Exp Med       Date:  1970-04-01       Impact factor: 14.307

10.  Genetic polymorphism in human glycine-rich beta-glycoprotein.

Authors:  C A Alper; T Boenisch; L Watson
Journal:  J Exp Med       Date:  1972-01       Impact factor: 14.307

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  8 in total

Review 1.  Bactericidal and bacteriolytic activity of serum against gram-negative bacteria.

Authors:  P W Taylor
Journal:  Microbiol Rev       Date:  1983-03

2.  Bactericidal versus bacteriostatic antibiotic therapy of experimental pneumococcal meningitis in rabbits.

Authors:  W M Scheld; M A Sande
Journal:  J Clin Invest       Date:  1983-03       Impact factor: 14.808

3.  Bacterial meningitis--ten years experience.

Authors:  P G Davey; J K Cruikshank; I C McManus; B Mahood; M H Snow; A M Geddes
Journal:  J Hyg (Lond)       Date:  1982-06

4.  [Recurrent meningitis in familial deficiency of the 8th component of the complement system].

Authors:  H Keller; S Löke; G M Hänsch; D Jentschura; H Gerhard; D L Heene
Journal:  Klin Wochenschr       Date:  1987-04-15

5.  Immunogenicity of meningococcal antigens as detected in patient sera.

Authors:  J T Poolman; C T Hopman; H C Zanen
Journal:  Infect Immun       Date:  1983-04       Impact factor: 3.441

6.  Molecular organization of C9 within the membrane attack complex of complement. Induction of circular C9 polymerization by the C5b-8 assembly.

Authors:  E R Podack; J Tschoop; H J Müller-Eberhard
Journal:  J Exp Med       Date:  1982-07-01       Impact factor: 14.307

7.  A simple PCR-based method for the rapid genotyping of inherited fifth complement component (C5)-deficient mice.

Authors:  Qingkai Wang; Na Wang; Xin Zhang; Weiguo Hu
Journal:  Exp Anim       Date:  2015-03-10

Review 8.  Monoclonal antibody therapy and renal transplantation: focus on adverse effects.

Authors:  Gianluigi Zaza; Paola Tomei; Simona Granata; Luigino Boschiero; Antonio Lupo
Journal:  Toxins (Basel)       Date:  2014-02-28       Impact factor: 4.546

  8 in total

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