Fulminant Meningococcemia in a Child With Hereditary Deficiency of the Seventh Component of Complement and Proteinuria

Loirat, C; Buriot, D; Peltier, A; Berche, Patrick; Aujard, Y; Griscelli, C; Mathieu, H

doi:10.1111/j.1651-2227.1980.tb07132.x

Cited by 10 publications

(2 citation statements)

References 16 publications

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“…This mode of inheritance is consistent with an autosomal codominant trait as reported for the other cases of C7D. To date, genetic analysis of the pedigrees of the C7D patients has failed to show a linkage of the HLA-A, -B loci with C7D (7,10,17,19,21). Family studies on C7 deficiency and the HLA system revealed no genetic linkage of C7 deficiency either with HLA-A and -B loci or with HLA-C and -DR loci (Fig.…”

Section: Complementsupporting

confidence: 86%

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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

Miyake

Ohta

Kawamori

et al. 1986

Microbiology and Immunology

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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.The biological significance of the complement system has been fully elucidated with the discovery of complement deficiencies associated with disease (9, 25). Deficiencies of the early-acting complement components (Cl, C4, and C2) are frequently associated with systemic lupus erythematosus (SLE) or other rheumatic diseases (2). In patients with C3 deficiency, recurrent bacterial infection has been shown to be the predominant complication (24) and lupus-like symptoms have also been reported as complications (26). It has been reported that the deficiencies Hamamatsu 430.

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Section: Complementsupporting

confidence: 86%

“…(25). Association between neisserial meningitis and C7D has been reported in many cases (1,7,8,(17)(18)(19)23). Some individuals with C7D have been healthy (7,28) and others have had an associated chronic pyelonephritis (21), bacteremia, systemic lupus erythematosus (29) or ankylosing spondylitis (10).…”

Section: Complementmentioning

confidence: 99%