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Type: Artigo de periódico
Title: Recurrent infections in partial complement factor I deficiency: evaluation of three generations of a Brazilian family
Author: Grumach, AS
Leitao, MF
Arruk, VG
Kirschfink, M
Condino-Neto, A
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 mu g/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.
Subject: complement deficiency
complement regulatory proteins
factor I
innate immunity
primary immunodeficiency
recurrent infections
Country: Inglaterra
Editor: Blackwell Publishing
Rights: fechado
Identifier DOI: 10.1111/j.1365-2249.2005.02988.x
Date Issue: 2006
Appears in Collections:Unicamp - Artigos e Outros Documentos

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