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Familial late complement component (C6, C7) deficiency with chronic meningococcemia
Archives of Internal Medicine
|July 1, 1980
Insights
Two patients with chronic meningococcemia lacked hemolytic complement due to C6 or C7 deficiency. These autosomal co-dominant traits did not affect their recovery after antibiotic treatment.
Area of Science:
- Immunology
- Complement System Biology
- Molecular Immunology
Background:
- Chronic meningococcemia is a rare but serious bacterial infection.
- The complement system plays a crucial role in host defense against Neisseria meningitidis.
- Deficiencies in terminal complement components are associated with increased susceptibility to meningococcal disease.
Observation:
- Two patients with chronic meningococcemia presented with a complete lack of hemolytic complement activity.
- Genetic analysis revealed C6 deficiency in one patient and C7 deficiency in the other.
- Family studies indicated autosomal co-dominant inheritance patterns for both C6 and C7 deficiencies, independent of HLA linkage.
Findings:
- Complement-deficient sera from these patients supported monocyte chemotaxis.
- However, these sera failed to mediate phagocytosis or lysis of meningococci.
- Antibiotic treatment led to successful recovery in both individuals.
Implications:
- This study highlights the critical role of terminal complement components (C6 and C7) in protecting against Neisseria meningitidis infections.
- Understanding the genetic basis and functional consequences of complement deficiencies is vital for managing recurrent meningococcal disease.
- Despite complement deficiencies, effective antibiotic therapy can resolve chronic meningococcemia, suggesting a multifaceted approach to treatment.
Abstract:
Two patients with chronic meningococcemia were found to lack hemolytic complement, one because of C6 deficiency, the other because of C7 deficiency. In both cases family studies were consistent with inheritance of the deficiencies as non-HLA-linked, autosomal co-dominant traits. Functional studies showed the deficient sera to support monocyte chemotaxis but not phagocytosis or lysis of meningococci. Both patients have remained well following antibiotic treatment.