Atypical hemolytic uremic syndrome is a rare and progressive disease caused by uncontrolled alternative complement activation. Dysregulatıon of the complement activation results in thrombotic microangiopathy and multiorgan damage.
Serife Solmaz Medeni +7 more
doaj +1 more source
When the environment and mutations affect organ systems [PDF]
Atypical hemolytic uremic syndrome (aHUS) is a rare thrombotic microangiopathy (TMA) with a genetic predisposition. Like other TMAs, it presents clinically with thrombocytopenia and microangiopathic hemolytic anemia, which is accompanied by disruption of
Abu Ghanimeh, Mouhanna +3 more
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Complement regulator CD46: Genetic variants and disease associations [PDF]
Membrane cofactor protein (MCP; CD46) is an ubiquitously expressed complement regulatory protein that protects host cells from injury by complement.
Atkinson, John P, Liszewski, M. Kathryn
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Atypical Hemolytic Uremic Syndrome
Kaan GÜLLEROĞLU +2 more
+8 more sources
The major autoantibody epitope on factor H in atypical hemolytic uremic syndrome is structurally different from its homologous site in factor H-related protein 1, supporting a novel model for induction of autoimmunity in this disease [PDF]
Atypical hemolytic uremic syndrome (aHUS) is characterized by complement attack against host cells due to mutations in complement proteins or autoantibodies against complement factor H (CFH).
Abarrategui-Garrido +59 more
core +1 more source
Atypical hemolytic uremic syndrome (aHUS) is a clinical diagnosis characterized by a triad of microangiopathic hemolytic anemia, thrombocytopenia, and renal dysfunction. Postoperative aHUS in nontransplant patients is a rare entity.
Anil Khetarpal +2 more
doaj +1 more source
FHR-1 binds to C-reactive protein and enhances rather than inhibits complement activation [PDF]
Factor H (FH)-related protein 1 (FHR-1) is one of the five human factor H-related proteins, which share sequence and structural homology with the alternative pathway complement inhibitor FH.
Bánlaki, Zsófia +7 more
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Atypical hemolytic-uremic syndrome, manifesting without thrombocytopenia (clinical case)
Background. Atypical hemolytic uremic syndrome is an ultra-rare (orphan) disease from the group of thrombotic microangiopathies of progressive course, which is caused by uncontrolled activation of the alternative complement pathway of hereditary or ...
A.A. Dyachkova +3 more
doaj +1 more source
Concurrent Atypical Hemolytic Uremic Syndrome and Autoimmune Hemolytic Anemia: a case report
Background: Atypical hemolytic uremic syndrome (aHUS) is a life-threatening and scarce disorder characterized by acute renal failure and disease, non-immune microangiopathic hemolytic anemia and thrombocytopenia, leading to end-stage renal failure or ...
Sayed Yousef Mojtahedi +1 more
doaj
Successful long-term outcome after renal transplantation in a patient with atypical haemolytic uremic syndrome with combined membrane cofactor protein CD46 and complement factor I mutations [PDF]
Background: Atypical haemolytic uremic syndrome (aHUS) is often associated with a high risk of disease recurrence and subsequent graft loss after isolated renal transplantation.
Bresin, Elena +5 more
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