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Complement factor I deficiency associated with recurrent infections, vasculitis and immune complex glomerulonephritis

Författare

Summary, in English

Here we report complement factor I deficiency in an 11-y-old girl from a consanguineous Turkish family, who presented with recurrent pyogenic infections, vasculitic eruptions and immune complex glomerulonephritis. A moderately low C3 level together with the clinical picture suggested a deficiency affecting regulation of complement activation. Analysis of haemolytic activity revealed absence of alternative pathway activity and subsequent analysis showed no detectable factor I (<2%) together with a low level of factor B and a moderately low level of factor H, indicating consumption secondary to the factor I deficiency. Factor I inhibits complement activation beyond C3 by cleavage of C3b in the presence of cofactors. Complement factor I deficiency is frequently associated with recurrent pyogenic infections mainly affecting the upper and lower respiratory tract, or presenting as meningitis or septicaemia, while rheumatic disorders have not been a prominent feature. The patient's sister also suffered from recurrent pyogenic infections and had a low C3 level clearly suggesting the same deficiency.

Publiceringsår

2005

Språk

Engelska

Sidor

615-618

Publikation/Tidskrift/Serie

Scandinavian Journal of Infectious Diseases

Volym

37

Issue

8

Dokumenttyp

Artikel i tidskrift

Förlag

Informa Healthcare

Ämne

  • Infectious Medicine

Status

Published

ISBN/ISSN/Övrigt

  • ISSN: 1651-1980