Neutrophil Function in a Patient With Meningococcal Meningitis and C7 Deficiency

Davis, Charles A.

doi:10.1001/archpedi.1983.02140300082026

Cited by 4 publications

(2 citation statements)

References 14 publications

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“…These sera did not lyse type 0 erythrocytes. C7-deficient human serum was obtained from a patient with no detectable C7 (19), and absorbed with H. influenzae type b at 00C for 60 min twice prior to use. All samples were separated within 90 min of collection, and stored at -90'C in aliquots.…”

Section: Methodsmentioning

confidence: 99%

Pathophysiology of hemolysis in infections with Hemophilus influenzae type b.

Shurin¹,

Anderson²,

Zollinger³

et al. 1986

J. Clin. Invest.

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The capsular polysaccharide of Hemophilus influenzae type b, polyribosyl ribitol phosphate (PRP), is released from growing organisms during human infection and can be found in body fluids. It binds to untreated erythrocytes. Many patients with invasive infections with this organism develop significant hemolysis, but the mechanism has been unclear. We have found that PRP binds to human erythrocytes in vivo. PRP-coated erythrocytes have a shortened circulation time in mice, but do not lyse spontaneously or fix complement. PRP-coated erythrocytes exposed to antiserum to H. influenzae type b are undamaged in the absence of complement, but are rapidly and effectively lysed in the presence of an intact complement system both in vitro and in vivo in mice. PRP-coated red cells are taken up by liver and spleen. Antiserum to PRP increases hepatic uptake of PRP-coated red cells more than splenic, and appears to induce intravascular, complementmediated hemolysis, as well as extravascular hemolysis. Patients with invasive infection develop hemolysis when circulating PRP and antibody to PRP are present simultaneously. PRP can sometimes be detected on patient erythrocytes when free PRP is present in serum, but this is an inconsistent finding. The hemolytic anemia that occurs during human infection with H. influenzae type b may be due to absorption of PRP to red cells and immune destruction of sensitized erythrocytes. The process requires an intact complement system; both complement-mediated cell lysis and extravascular hemolysis contribute to red cell destruction.

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Section: Methodsmentioning

confidence: 99%

Pathophysiology of hemolysis in infections with Hemophilus influenzae type b.

Shurin¹,

Anderson²,

Zollinger³

et al. 1986

J. Clin. Invest.

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“…(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%

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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Familial deficiency of the seventh component of complement associated with recurrent meningococcal infections

et al. 1989

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We describe an 11-year-old girl suffering from recurrent meningitis with a complete absence of the seventh component of complement (C7). Diagnosis was established by haemolytic titration and western blotting. The patient's serum lacked the 85 kDa C7 chain. Haemolytic activity of serum was reconstituted with either pooled normal human serum or with purified C7. The relatives (parents and one sister) had half-normal levels of both immunochemically and functionally determined C7, indicating a heterozygous state for C7 deficiency.

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Neutrophil Function in a Patient With Meningococcal Meningitis and C7 Deficiency

Cited by 4 publications

References 14 publications

Pathophysiology of hemolysis in infections with Hemophilus influenzae type b.

Pathophysiology of hemolysis in infections with Hemophilus influenzae type b.

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

Familial deficiency of the seventh component of complement associated with recurrent meningococcal infections

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