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NDT Advance Access originally published online on May 17, 2007
Nephrology Dialysis Transplantation 2007 22(9):2452-2454; doi:10.1093/ndt/gfm193
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© The Author [2007]. Published by Oxford University Press on behalf of ERA-EDTA. All rights reserved. For Permissions, please email: journals.permissions@oxfordjournals.org

Complement hyperactivation may cause atypical haemolytic uraemic syndrome— gain-of-function mutations in factor B*

Jessica Caprioli1 and Giuseppe Remuzzi2

1Mario Negri Institute for Pharmacological Research, Clinical Research Center for Rare Diseases, Aldo e Cele Daccò, Villa Camozzi, Ranica, Bergamo and 2Division of Nephrology and Dialysis, Azienda Ospedaliera, Ospedali Riuniti di Bergamo, Italy

Correspondence and offprint requests to: Jessica Caprioli, Transplant Research Center, ‘Chiara Cucchi de Alessandri e Gilberto Crespi’, Villa Camozzi, 3 – 24020 Ranica (BG), Italy. Email: caprioli@marionegri.it

Keywords: Complement factor B; HUS; mutation

The first 150 words of the full text of this article appear below.



   Introduction
 
Non-Shiga toxin-associated haemolytic uraemic syndrome (non-Stx-HUS) is a rare disease with manifestations of haemolytic anaemia, thrombocytopenia and renal failure. The clinical outcome is unfavourable, with up to 50% of patients progressing to end-stage renal failure and 25% dying during the acute phase [1]. Genetic abnormalities in complement regulatory proteins, including complement factor H (CFH), membrane cofactor protein (MCP) and complement factor I (CFI), have been reported in about 30, 10 and 5% of patients with non-Stx-HUS, respectively [2–4], thus demonstrating that defective control of the alternative pathway of complement [5] has a pathogenetic role in at least half of the patients. The complement system consists of several plasma proteins that collectively serve to destroy pathogens Figure 1. Its activation is tightly regulated by circulating and membrane-bound inhibitors so as to prevent non-specific damage to host cells and to limit deposition of C3b to . . . [Full Text of this Article]

The role of factor B in complement cascade activation


   Results and conclusions
 
Factor B mutations in HUS


   Clinical implications
 

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