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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| Main Authors: | , , , , , , |
|---|---|
| Format: | Article (Journal) |
| Language: | English |
| Published: |
2014
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| 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 |
| Author Notes: | Damien Noone, Aoife Waters, Fred G. Pluthero, Denis F. Geary, Michael Kirschfink, Peter F. Zipfel, Christoph Licht |
MARC
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| 520 | |a 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. | ||
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