Inherited Deficiency of the Seventh Component of Complement Associated with Meningococcal Meningitis: Lack of Serum Bactericidal Activity against Neisseria meningitidis in a Girl with C7 Deficiency and HLA Studies of a C7-Deficient Japanese Family

An 8-year-old girl with meningococcal meningitis lacked serum complement activity. The seventh component of complement (C7) could not be detected in her serum by either functional or immunochemical analysis. The levels of the other components were within the normal range. Her serum complement activi...

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Bibliographic Details
Published in:MICROBIOLOGY and IMMUNOLOGY 1986, Vol.30(4), pp.363-372
Main Authors: Miyake, Takeshi, Ohta, Kuniaki, Kawamori, Juzou, Hirao, Takao, Akagaki, Youji, Inai, Shinya
Format: Article
Language:English
Subjects:
Bacteria
Biological and medical sciences
Blood Bactericidal Activity
Chemotaxis, Leukocyte
Child
Complement C7 - deficiency
Complement C7 - genetics
Female
HLA Antigens - genetics
Humans
Immunodeficiencies
Immunodeficiencies. Immunoglobulinopathies
Immunopathology
Japan
Male
Medical sciences
Meningitis, Meningococcal - genetics
Meningitis, Meningococcal - immunology
Neisseria meningitidis
Neisseria meningitidis - immunology
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Summary:An 8-year-old girl with meningococcal meningitis lacked serum complement activity. The seventh component of complement (C7) could not be detected in her serum by either functional or immunochemical analysis. The levels of the other components were within the normal range. Her serum complement activity was restored by the addition of purified C7. Her fresh serum showed a total absence of bactericidal activity against Neisseria meningitidis, group Y, but her serum bactericidal activity was restored by the addition of purified C7. The restoration of her serum bactericidal activity was completely inhibited in the presence of Mg2+ EGTA. These findings suggest that restoration of the bactericidal activity of her serum against N. meningitidis might be mediated by the specific antibody against N. meningitidis and the reconstituted complement system in her serum. Heterozygous deficiency of C7 was found in 10 of her family members. Genetic studies showed that the mode of inheritance might be an autosomal codominant trait. No genetic linkage between deficiency of C7 and the HLA system was found.
ISSN:0385-5600
1348-0421
DOI:10.1111/j.1348-0421.1986.tb00953.x