Atypical Hemolytic Uremic Syndrome
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"Atypical Hemolytic Uremic Syndrome" refers background in this paper
...The prognosis of aHUS with CFB mutations also is poor.(51,55,56) A proportion of patients will have combined mutations....
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...Only 30% to 40% of individuals with CFI and C3 mutations will be alive with native kidney function at 3 to 5 years.(49,51) The prognosis of aHUS with CFB mutations also is poor....
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...In patients with a CFH mutation the initial mortality rate was 30% in children and 4% in adults,(49) and evolution to ESRD at first episode in survivors was 19% to 33% in children and 48% in adults.(49,51) At 3 to 5 years of follow-up evaluation, up to 77% of patients with CFH mutations had developed ESRD or had died....
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...Infectious events, mainly upper respiratory tract infections or diarrhea/gastroenteritis, trigger onset of aHUS in at least half of patients.(49,51) Likewise, in pregnancy-associated aHUS, Fakhouri et al(98) showed that 86% of patients for whom this was a trigger carried a complement gene mutation....
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...diarrhea preceded aHUS in 23% of a French cohort(94) and in 28% of an Italian cohort.(51) Infectious events, mainly upper respiratory tract infections or diarrhea/gastroenteritis, trigger onset of aHUS in at least half of patients....
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"Atypical Hemolytic Uremic Syndrome" refers background in this paper
...Eculizumab, a recombinant humanized monoclonal antibody directed against C5, blocks the cleavage of C5 into its effector components C5a and C5b.(106) Since the initial use of this complement inhibitor in aHUS in 2009,(107) the effectiveness of eculizumab has been communicated in many case reports, meeting abstracts, and a recently published prospective trial....
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