Medizinische Universität Graz Austria/Österreich - Forschungsportal - Medical University of Graz
Gewählte Publikation:
SHR Neuro Krebs Kardio Lipid Stoffw Microb
Zipfel, PF; Mache, C; Müller, D; Licht, C; Wigger, M; Skerka, C; for the European DEAP-HUS Study Group.
DEAP-HUS: Deficiency of CFHR plasma proteins and autoantibody-positive form of hemolytic uremic syndrome.
Pediatr Nephrol. 2010; 25(10): 2009-2019.
Doi: 10.1007/s00467-010-1446-9
Web of Science
PubMed
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- Co-Autor*innen der Med Uni Graz
- Mache Christoph
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- 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.
- Find related publications in this database (using NLM MeSH Indexing)
- Autoantibodies - blood
- Autoantigens - immunology
- Blood Proteins - deficiency
- Child -
- Complement C3b Inactivator Proteins - deficiency
- Complement Factor H - immunology
- Hemolytic-Uremic Syndrome -
- Humans -
- Find related publications in this database (Keywords)
- CFHR1/CFHR3 deficiency
- DEAP-HUS
- Factor H autoantibodies
- Immunosuppression
- Plasma therapy
- Transplantation