Excessive activation of the complement system in atypical hemolytic uremic syndrome: is it ready for prime time?
- PMID: 20118898
- PMCID: PMC3153079
- DOI: 10.1038/ki.2009.467
Excessive activation of the complement system in atypical hemolytic uremic syndrome: is it ready for prime time?
Abstract
Complement factor I (CFI) mutations are implicated in the pathogenesis of atypical hemolytic uremic syndrome (aHUS). Nevertheless, there is evidence that CFI deficiency is a weak effector of aHUS. Bienaime et al. report that homozygous deletion of CFHR-1 in the RCA gene cluster of chromosome 1q is a major risk factor for poor outcome for patients with CFI mutations. The basic and clinical implications of the findings are further elaborated here.
Conflict of interest statement
The author declared no competing interests.
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Comment on
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Mutations in components of complement influence the outcome of Factor I-associated atypical hemolytic uremic syndrome.Kidney Int. 2010 Feb;77(4):339-49. doi: 10.1038/ki.2009.472. Epub 2009 Dec 16. Kidney Int. 2010. PMID: 20016463
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