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Eculizumab therapy for atypical haemolytic uraemic syndrome due to a gain-of-function mutation of complement factor B

Background Atypical haemolytic uraemic syndrome (aHUS) is caused by dysregulated complement activation. A humanised anti-C5 monoclonal antibody has recently become available for treatment of this condition Case-Diagnosis/Treatment We present the first description of an infant with an activating muta... Full description

Journal Title: Pediatric Nephrology 2013, Vol.28(8), pp.1315-1318
Main Author: Gilbert, Rodney
Other Authors: Fowler, Darren , Angus, Elizabeth , Hardy, Stephen , Stanley, Louise , Goodship, Timothy
Format: Electronic Article Electronic Article
Language: English
Subjects:
ID: ISSN: 0931-041X ; E-ISSN: 1432-198X ; DOI: 10.1007/s00467-013-2492-x
Link: http://dx.doi.org/10.1007/s00467-013-2492-x
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recordid: springer_jour10.1007/s00467-013-2492-x
title: Eculizumab therapy for atypical haemolytic uraemic syndrome due to a gain-of-function mutation of complement factor B
format: Article
creator:
  • Gilbert, Rodney
  • Fowler, Darren
  • Angus, Elizabeth
  • Hardy, Stephen
  • Stanley, Louise
  • Goodship, Timothy
subjects:
  • Atypical haemolytic uraemic syndrome
  • Eculizumab
  • Complement factor B
  • Gain-of-function mutation
  • Complement activation
ispartof: Pediatric Nephrology, 2013, Vol.28(8), pp.1315-1318
description: Background Atypical haemolytic uraemic syndrome (aHUS) is caused by dysregulated complement activation. A humanised anti-C5 monoclonal antibody has recently become available for treatment of this condition Case-Diagnosis/Treatment We present the first description of an infant with an activating mutation of complement factor B successfully treated with eculizumab. On standard doses she had evidence of ongoing C5 cleavage despite a good clinical response. Conclusions Eculizumab is effective therapy for aHUS associated with factor B mutations, but recommended doses may not be adequate for all patients. Keywords Atypical haemolytic uraemic syndrome * Eculizumab * Complement factor B * Gain-of-function mutation * Complement activation
language: eng
source:
identifier: ISSN: 0931-041X ; E-ISSN: 1432-198X ; DOI: 10.1007/s00467-013-2492-x
fulltext: fulltext
issn:
  • 1432-198X
  • 1432198X
  • 0931-041X
  • 0931041X
url: Link


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titleEculizumab therapy for atypical haemolytic uraemic syndrome due to a gain-of-function mutation of complement factor B
creatorGilbert, Rodney ; Fowler, Darren ; Angus, Elizabeth ; Hardy, Stephen ; Stanley, Louise ; Goodship, Timothy
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subjectAtypical haemolytic uraemic syndrome ; Eculizumab ; Complement factor B ; Gain-of-function mutation ; Complement activation
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descriptionBackground Atypical haemolytic uraemic syndrome (aHUS) is caused by dysregulated complement activation. A humanised anti-C5 monoclonal antibody has recently become available for treatment of this condition Case-Diagnosis/Treatment We present the first description of an infant with an activating mutation of complement factor B successfully treated with eculizumab. On standard doses she had evidence of ongoing C5 cleavage despite a good clinical response. Conclusions Eculizumab is effective therapy for aHUS associated with factor B mutations, but recommended doses may not be adequate for all patients. Keywords Atypical haemolytic uraemic syndrome * Eculizumab * Complement factor B * Gain-of-function mutation * Complement activation
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