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

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 presen...

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Hauptverfasser: Grumach, Anete (VerfasserIn) , Leitão, M F (VerfasserIn) , Arruk, V G (VerfasserIn) , Kirschfink, Michael (VerfasserIn) , Condino-Neto, A (VerfasserIn)
Dokumenttyp: Article (Journal)
Sprache:Englisch
Veröffentlicht: 2006
In: Clinical & experimental immunology
Year: 2006, Jahrgang: 143, Heft: 2, Pages: 297-304
ISSN:1365-2249
DOI:10.1111/j.1365-2249.2005.02988.x
Online-Zugang:Verlag, lizenzpflichtig, Volltext: https://dx.doi.org/10.1111/j.1365-2249.2005.02988.x
Verlag, lizenzpflichtig, Volltext: https://www.ncbi.nlm.nih.gov/pmc/articles/PMC1809586/
Volltext
Verfasserangaben:A.S. Grumach, M.F. Leitão, V.G. Arruk, M. Kirschfink and A. Condino-Neto
Beschreibung
Zusammenfassung: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 µg/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.
Beschreibung:Gesehen am 15.02.2021
Beschreibung:Online Resource
ISSN:1365-2249
DOI:10.1111/j.1365-2249.2005.02988.x

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