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[Complement factor I deficiency revealed by repeated systemic Streptococcus pneumoniae infection].

M Monfort-Gouraud1, C Vignon, A Vincens

  • 1Service de pédiatrie, centre hospitalier intercommunal de Montfermeil-Le-Raincy, 10, avenue du Général-Leclerc, 93370 Montfermeil, France. marie.monfortgouraud@laposte.net

Archives De Pediatrie : Organe Officiel De La Societe Francaise De Pediatrie
|June 30, 2006
PubMed
Summary

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A rare Factor I deficiency was diagnosed in a 6-year-old girl with recurrent infections. Prompt diagnosis and management, including antibiotics and vaccinations, significantly improve her prognosis and quality of life.

Area of Science:

  • Immunology
  • Complement System Biology
  • Pediatric Infectious Diseases

Background:

  • Recurrent infections, including otitis media and Streptococcus pneumoniae bacteremia, can indicate underlying immune deficiencies in children.
  • The complement system plays a crucial role in innate and adaptive immunity, protecting against bacterial infections.

Observation:

  • A 6-year-old girl presented with recurrent serious otitis media and Streptococcus pneumoniae bacteremia.
  • Complement investigation revealed moderately reduced CH50 activity, normal C4 levels, low C3 levels, and undetectable Factor I.
  • These findings suggested a deficiency in the complement system, specifically Factor I.

Findings:

  • The patient was diagnosed with a rare Factor I deficiency.
  • Factor I is essential for regulating complement activation, and its absence leads to uncontrolled complement cascade and increased susceptibility to infections.

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Implications:

  • Diagnosis of Factor I deficiency has significant practical consequences for patient management and prognosis.
  • Preventive strategies, such as antibiotic prophylaxis (penicillin V) and vaccination (Neisseria meningitidis, S. pneumoniae), are vital.
  • Effective management can significantly improve the child's life expectancy and quality of life by reducing infection frequency and severity.