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...

Full description

Saved in:
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
Get full text
Author Notes:Damien Noone, Aoife Waters, Fred G. Pluthero, Denis F. Geary, Michael Kirschfink, Peter F. Zipfel, Christoph Licht

MARC

LEADER 00000caa a2200000 c 4500
001 1731815212
003 DE-627
005 20240406193253.0
007 cr uuu---uuuuu
008 200914r20142013xx |||||o 00| ||eng c
024 7 |a 10.1007/s00467-013-2654-x  |2 doi 
035 |a (DE-627)1731815212 
035 |a (DE-599)KXP1731815212 
035 |a (OCoLC)1341359673 
040 |a DE-627  |b ger  |c DE-627  |e rda 
041 |a eng 
084 |a 33  |2 sdnb 
100 1 |a Noone, Damien  |e VerfasserIn  |0 (DE-588)1217624759  |0 (DE-627)1731814003  |4 aut 
245 1 0 |a Successful treatment of DEAP-HUS with eculizumab  |c Damien Noone, Aoife Waters, Fred G. Pluthero, Denis F. Geary, Michael Kirschfink, Peter F. Zipfel, Christoph Licht 
264 1 |c 2014 
300 |a 11 
336 |a Text  |b txt  |2 rdacontent 
337 |a Computermedien  |b c  |2 rdamedia 
338 |a Online-Ressource  |b cr  |2 rdacarrier 
500 |a Gesehen am 14.09.2020 
500 |a First published: 20 November 2013 
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. 
534 |c 2013 
700 1 |a Waters, Aoife  |e VerfasserIn  |4 aut 
700 1 |a Pluthero, Fred G.  |e VerfasserIn  |4 aut 
700 1 |a Geary, Denis F.  |e VerfasserIn  |0 (DE-588)113061056X  |0 (DE-627)88482439X  |0 (DE-576)48686751X  |4 aut 
700 1 |a Kirschfink, Michael  |e VerfasserIn  |0 (DE-588)120927152  |0 (DE-627)080974457  |0 (DE-576)292452411  |4 aut 
700 1 |a Zipfel, Peter F.  |d 1954-  |e VerfasserIn  |0 (DE-588)110935810  |0 (DE-627)691099006  |0 (DE-576)289687810  |4 aut 
700 1 |a Licht, Christoph  |d 1964-  |e VerfasserIn  |0 (DE-588)1089083289  |0 (DE-627)850995280  |0 (DE-576)459952781  |4 aut 
773 0 8 |i Enthalten in  |t Pediatric nephrology  |d Berlin : Springer, 1987  |g 29(2014), 5, Seite 841-851  |h Online-Ressource  |w (DE-627)254638872  |w (DE-600)1463004-7  |w (DE-576)074531689  |x 1432-198X  |7 nnas  |a Successful treatment of DEAP-HUS with eculizumab 
773 1 8 |g volume:29  |g year:2014  |g number:5  |g pages:841-851  |g extent:11  |a Successful treatment of DEAP-HUS with eculizumab 
856 4 0 |u https://doi.org/10.1007/s00467-013-2654-x  |x Verlag  |x Resolving-System  |z lizenzpflichtig  |3 Volltext 
951 |a AR 
992 |a 20200914 
993 |a Article 
994 |a 2014 
998 |g 120927152  |a Kirschfink, Michael  |m 120927152:Kirschfink, Michael  |d 910000  |d 911600  |d 50000  |e 910000PK120927152  |e 911600PK120927152  |e 50000PK120927152  |k 0/910000/  |k 1/910000/911600/  |k 0/50000/  |p 5 
999 |a KXP-PPN1731815212  |e 3752968648 
BIB |a Y 
SER |a journal 
JSO |a {"person":[{"given":"Damien","family":"Noone","role":"aut","display":"Noone, Damien","roleDisplay":"VerfasserIn"},{"roleDisplay":"VerfasserIn","display":"Waters, Aoife","role":"aut","family":"Waters","given":"Aoife"},{"role":"aut","roleDisplay":"VerfasserIn","display":"Pluthero, Fred G.","given":"Fred G.","family":"Pluthero"},{"role":"aut","roleDisplay":"VerfasserIn","display":"Geary, Denis F.","given":"Denis F.","family":"Geary"},{"given":"Michael","family":"Kirschfink","role":"aut","display":"Kirschfink, Michael","roleDisplay":"VerfasserIn"},{"given":"Peter F.","family":"Zipfel","role":"aut","roleDisplay":"VerfasserIn","display":"Zipfel, Peter F."},{"roleDisplay":"VerfasserIn","display":"Licht, Christoph","role":"aut","family":"Licht","given":"Christoph"}],"title":[{"title":"Successful treatment of DEAP-HUS with eculizumab","title_sort":"Successful treatment of DEAP-HUS with eculizumab"}],"recId":"1731815212","language":["eng"],"note":["Gesehen am 14.09.2020","First published: 20 November 2013"],"type":{"bibl":"article-journal","media":"Online-Ressource"},"name":{"displayForm":["Damien Noone, Aoife Waters, Fred G. Pluthero, Denis F. Geary, Michael Kirschfink, Peter F. Zipfel, Christoph Licht"]},"id":{"eki":["1731815212"],"doi":["10.1007/s00467-013-2654-x"]},"origin":[{"dateIssuedKey":"2014","dateIssuedDisp":"2014"}],"relHost":[{"title":[{"title_sort":"Pediatric nephrology","title":"Pediatric nephrology","subtitle":"journal of the International Pediatric Nephrology Association"}],"note":["Gesehen am 06.12.05"],"disp":"Successful treatment of DEAP-HUS with eculizumabPediatric nephrology","type":{"bibl":"periodical","media":"Online-Ressource"},"language":["eng"],"recId":"254638872","pubHistory":["1.1987 -"],"part":{"issue":"5","pages":"841-851","year":"2014","extent":"11","text":"29(2014), 5, Seite 841-851","volume":"29"},"origin":[{"dateIssuedKey":"1987","publisher":"Springer ; Springer","dateIssuedDisp":"1987-","publisherPlace":"Berlin ; Heidelberg ; Berlin ; Heidelberg ; New York, NY"}],"id":{"eki":["254638872"],"zdb":["1463004-7"],"issn":["1432-198X"]},"physDesc":[{"extent":"Online-Ressource"}]}],"physDesc":[{"extent":"11 S."}]} 
SRT |a NOONEDAMIESUCCESSFUL2014