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Successful treatment of DEAP-HUS with eculizumab

Deficiency of complement factor H-related (CFHR) proteins and CFH autoantibody-positive hemolytic uremic syndrome (DEAP-HUS) represents a unique subgroup of complement-mediated atypical HUS (aHUS). Autoantibodies to the C-terminus of CFH block CFH surface recognition and mimic mutations found in the...

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Bibliographic Details
Main Authors: Noone, Damien (Author) , Waters, Aoife (Author) , Pluthero, Fred G. (Author) , Geary, Denis F. (Author) , Kirschfink, Michael (Author) , Zipfel, Peter F. (Author) , Licht, Christoph (Author)
Format: Article (Journal)
Language:English
Published: 2014
In: Pediatric nephrology
Year: 2013, Volume: 29, Issue: 5, Pages: 841-851
ISSN:1432-198X
DOI:10.1007/s00467-013-2654-x
Online Access:Verlag, lizenzpflichtig, Volltext: https://doi.org/10.1007/s00467-013-2654-x
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Author Notes:Damien Noone, Aoife Waters, Fred G. Pluthero, Denis F. Geary, Michael Kirschfink, Peter F. Zipfel, Christoph Licht
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Summary:Deficiency of complement factor H-related (CFHR) proteins and CFH autoantibody-positive hemolytic uremic syndrome (DEAP-HUS) represents a unique subgroup of complement-mediated atypical HUS (aHUS). Autoantibodies to the C-terminus of CFH block CFH surface recognition and mimic mutations found in the genetic form of (CFH-mediated) aHUS. CFH autoantibodies are found in 10-15 % of aHUS patients and occur—so far unexplained—almost exclusively in the background of CFHR1 or CFHR3/CFHR1 deletions.
Item Description:Gesehen am 14.09.2020
First published: 20 November 2013
Physical Description:Online Resource
ISSN:1432-198X
DOI:10.1007/s00467-013-2654-x

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