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Recurrent infections in partial complement factor I deficiency: evaluation of three generations of a Brazilian family.

Abstract
We report here on the evaluation of a factor I-deficient Brazilian family (three generations, 39 members) with strong consanguinity. The complete factor I-deficient patients (n = 3) presented recurrent respiratory infections, skin infections and meningitis; one of them died after sepsis. They presented an impaired total haemolytic activity (CH50), low C3, low factor H and undetectable C3dg/C3d. Partial factor I deficiency was detected in 16 family members (normal low cut-off value was 25 microg/ml). Respiratory infections were the most common clinical occurrence among partial factor I-deficient relatives. Two of them were submitted to nephrectomy following recurrent urinary tract infections. An additional two heterozygous relatives presented with arthritis and rheumatic fever. Apparently, patients with partial factor I deficiency are also at higher risk for recurrent infections. Vaccination against capsulated bacteria and the eventual use of prophylactic antibiotics should be considered individually in this patient group.
AuthorsA S Grumach, M F Leitão, V G Arruk, M Kirschfink, A Condino-Neto
JournalClinical and experimental immunology (Clin Exp Immunol) Vol. 143 Issue 2 Pg. 297-304 (Feb 2006) ISSN: 0009-9104 [Print] England
PMID16412054 (Publication Type: Journal Article, Research Support, Non-U.S. Gov't)
Chemical References
  • Complement Factor I
Topics
  • Adolescent
  • Adult
  • Aged
  • Bacterial Infections (genetics, immunology)
  • Brazil
  • Child
  • Child, Preschool
  • Complement Factor I (analysis, deficiency, immunology)
  • Family Health
  • Female
  • Humans
  • Immunity, Innate (genetics, immunology)
  • Male
  • Meningitis (immunology)
  • Pedigree
  • Recurrence
  • Respiratory Tract Infections (genetics, immunology)
  • Skin Diseases, Infectious (genetics, immunology)

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