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Eculizumab for Atypical Hemolytic–Uremic Syndrome [PDF]
To the Editor: Atypical hemolytic–uremic syndrome is a disease of uncontrolled complement activation associated with a high mortality rate, and most cases progress to end-stage renal disease.1 About 50% of patients with this syndrome carry mutations in genes encoding complement proteins.2 Complement inhibition has been suggested for the treatment of ...
Michael Kirschfink+10 more
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Atypical hemolytic uremic syndrome [PDF]
AbstractAtypical hemolytic uremic syndrome (aHUS) is a thrombotic microangiopathy (TMA) that affects multiple organs and the kidneys in particular. aHUS can be sporadic or familial and is most commonly caused by dysregulation of the alternative complement pathway.
Arif Asif, Ali Nayer
openaire +6 more sources
Hemolytic uremic syndrome (HUS) is a thrombotic microangiopathy (TMA) defined by the triad of hemolytic anemia, thrombocytopenia, and acute kidney injury.
Keval Yerigeri+7 more
semanticscholar +1 more source
Hemolytic Uremic Syndrome: A Case Report
Thrombotic microangiopathy is a pathological condition comprised of microvascular thrombosis involving any body organ leading to thrombocytopenia, coombs-negative hemolytic anemia, and end-organ damage.
Sandesh Gaire+5 more
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Introduction Atypical hemolytic uremic syndrome (aHUS) is a rare disease that manifests with microangiopathic hemolytic anemia, thrombocytopenia, and acute renal failure, and is associated with dysregulation of the alternative complement pathway.
R. Piras+11 more
semanticscholar +1 more source
Atypical Hemolytic Uremic Syndrome [PDF]
Hemolytic uremic syndrome (HUS) is a triad of microangiopathic hemolytic anemia, thrombocytopenia, and acute renal failure. The atypical form of HUS is a disease characterized by complement overactivation. Inherited defects in complement genes and acquired autoantibodies against complement regulatory proteins have been described.
Kavanagh D, Goodship TH, Richards A
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Background Atypical hemolytic uremic syndrome is an exceedingly rare thrombotic microangiopathy caused by accelerated activation of the alternative complement pathway.
Christine J. Kurian+7 more
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Key Points • We prove in genotype-matched complement-mediated aHUS cohorts that C5 inhibition results in a statistical improvement in ESKD-free survival.• We demonstrate that biallelic pathogenic mutations in EXOSC3 cause eculizumab nonresponsive aHUS.
V. Brocklebank+136 more
semanticscholar +1 more source
A 34-year-old Japanese man presented with blurred vision, headache, nausea, anemia, thrombocytopenia, and severe renal dysfunction. Thrombotic microangiopathy was initially suspected to have been caused by malignant hypertension.
Kosuke Osawa+13 more
semanticscholar +1 more source
Hemolytic uremic syndrome (HUS) is classically described as a triad of nonimmune hemolytic anemia, thrombocytopenia, and acute kidney injury. Atypical HUS (aHUS) is a rare variant of the disease, and it accounts for 5–10% of the cases.
Mohammed Tawhari+4 more
semanticscholar +1 more source