A complicated case of atypical hemolytic uremic syndrome with frequent relapses under eculizumab

Atypical hemolytic uremic syndrome (aHUS) is a form of thrombotic microangiopathy characterized by uncontrolled activation of the alternative complement pathway with consecutive generation of the terminal complement complex. Mortality is increased, particularly in the first year of the disease. Ther...

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Main Authors: Schalk, Gesa (Author) , Kirschfink, Michael (Author) , Wehling, Cyrill (Author) , Gastoldi, Sara (Author) , Bergmann, Carsten (Author) , Hoppe, Bernd (Author) , Weber, Lutz T. (Author)
Format: Article (Journal)
Language:English
Published: 10 March 2015
In: Pediatric nephrology
Year: 2015, Volume: 30, Issue: 6, Pages: 1039-1042
ISSN:1432-198X
DOI:10.1007/s00467-015-3078-6
Online Access:Verlag, lizenzpflichtig, Volltext: https://doi.org/10.1007/s00467-015-3078-6
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Author Notes:Gesa Schalk, Michael Kirschfink, Cyrill Wehling, Sara Gastoldi, Carsten Bergmann, Bernd Hoppe, Lutz T. Weber
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Summary:Atypical hemolytic uremic syndrome (aHUS) is a form of thrombotic microangiopathy characterized by uncontrolled activation of the alternative complement pathway with consecutive generation of the terminal complement complex. Mortality is increased, particularly in the first year of the disease. Therapeutic options include plasma therapy and terminal complement blockade using the anti-C5 monoclonal antibody eculizumab. Eculizumab prevents activation of the terminal sequence of the complement cascade and formation of the potentially lytic terminal complement complex (C5b-9).
Item Description:Gesehen am 17.06.2020
Physical Description:Online Resource
ISSN:1432-198X
DOI:10.1007/s00467-015-3078-6