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01-10-2010 | Review

DEAP-HUS: Deficiency of CFHR plasma proteins and autoantibody-positive form of hemolytic uremic syndrome

Authors: Peter F. Zipfel, Christoph Mache, Dominik Müller, Christoph Licht, Marianne Wigger, Christine Skerka, for the European DEAP-HUS Study Group

Published in: Pediatric Nephrology | Issue 10/2010

Abstract

DEAP-HUS [Deficiency of CFHR (complement factor H-related) plasma proteins and Autoantibody Positive form of Hemolytic Uremic Syndrome] represents a novel subtype of hemolytic uremic syndrome (HUS) with unique characteristics. It affects children and requires special clinical attention in terms of diagnosis and therapy. DEAP-HUS and other atypical forms of HUS share common features, such as microangiopathic hemolytic anemia, acute renal failure, and thrombocytopenia. However, DEAP-HUS has the unique combination of an acquired factor in the form of autoantibodies to the complement inhibitor Factor H and a genetic factor which, in most cases, is the chromosomal deletion of a 84-kbp fragment within human chromosome 1 that results in the absence of the CFHR1 and CFHR3 proteins in plasma. Special attention is required to diagnose and treat DEAP-HUS patients. Most patients show a favorable response to the reduction of autoantibody titers by either plasma therapy, steroid treatment, and/or immunosuppression. In addition, in those DEAP-HUS patients with end-stage renal disease, the reduction of autoantibody titers prior to transplantation is expected to prevent post-transplant disease recurrence by aiming for full complement control at the endothelial cell surface in order to minimize adverse complement and immune reactions.

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Title: DEAP-HUS: Deficiency of CFHR plasma proteins and autoantibody-positive form of hemolytic uremic syndrome
Authors: Peter F. Zipfel
Christoph Mache
Dominik Müller
Christoph Licht
Marianne Wigger
Christine Skerka
for the European DEAP-HUS Study Group
Publication date: 01-10-2010
Publisher: Springer-Verlag
Published in: Pediatric Nephrology / Issue 10/2010
Print ISSN: 0931-041X
Electronic ISSN: 1432-198X
DOI: https://doi.org/10.1007/s00467-010-1446-9

At a glance: The STEP trials

Springer Medicine

DEAP-HUS: Deficiency of CFHR plasma proteins and autoantibody-positive form of hemolytic uremic syndrome

Abstract

At a glance: The STEP trials

Springer Medicine

Abstract

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