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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
Author(s) -
Miyake Takeshi,
Ohta Kuniaki,
Kawamori Juzou,
Hirao Takao,
Akagaki Youji,
Inai Shinya
Publication year - 1986
Publication title -
microbiology and immunology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.664
H-Index - 70
eISSN - 1348-0421
pISSN - 0385-5600
DOI - 10.1111/j.1348-0421.1986.tb00953.x
Subject(s) - neisseria meningitidis , biology , complement deficiency , microbiology and biotechnology , complement system , immunology , alternative complement pathway , classical complement pathway , neisseria , meningitis , neisseriaceae , antibody , bacteria , medicine , genetics , antibiotics , psychiatry
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 Mg 2+ 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.