Results 41 to 50 of about 25,762 (279)
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
Atypical Hemolytic Uremic Syndrome
Atypical hemolytic uremic syndrome is a rare life-threatening disease of unregulated complement activation. Untreated, the prognosis is generally poor; more than one-half of patients die or develop end-stage renal disease within 1 year. Atypical hemolytic uremic syndrome is characterized by thrombotic microangiopathy with evidence of hemolysis ...
Bradley P. Dixon, Ralph A. Gruppo
+7 more sources
Atypical hemolytic uremic syndrome (aHUS) is a type of thrombotic microangiopathy and is characterized by microangiopathic hemolytic anemia, thrombocytopenia, and acute kidney failure. The complement cascade plays an integral role in aHUS.
Kamran Shahid, S. Qayyum
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
Influenza B-Associated Atypical Hemolytic Uremic Syndrome [PDF]
Hemolytic uremic syndrome (HUS) triggered by influenza virus (iHUS) is rare. Influenza A infections have been described to trigger atypical HUS (aHUS) in individuals with an underlying genetic complement dysregulation. To date there are only few reports
Lia Mano +5 more
doaj +1 more source
Pregnancy-associated atypical hemolytic uremic syndrome (P-aHUS) is a rare condition characterized by microangiopathic hemolytic anemia and kidney injury from thrombotic microangiopathy.
Alex Domínguez-Vargas +6 more
semanticscholar +1 more source
Hyperfunctional complement C3 promotes C5-dependent atypical hemolytic uremic syndrome in mice [PDF]
Atypical hemolytic uremic syndrome (aHUS) is frequently associated in humans with loss-of-function mutations in complement-regulating proteins or gain-of-function mutations in complement-activating proteins.
Atkinson, John P +11 more
core +3 more sources
How I diagnose and treat atypical hemolytic uremic syndrome.
Our understanding and management of atypical haemolytic uremic syndrome (HUS) has dramatically improved in the last decade. Atypical HUS has been established as a prototypic disease resulting from a dysregulation of the complement alternative C3 ...
F. Fakhouri +2 more
semanticscholar +1 more source
Anti-factor H antibody and its role in atypical hemolytic uremic syndrome
Atypical hemolytic uremic syndrome (aHUS) an important form of a thrombotic microangiopathy (TMA) that can frequently lead to acute kidney injury (AKI). An important subset of aHUS is the anti-factor H associated aHUS.
R. Raina +7 more
semanticscholar +1 more source
How I treat thrombotic thrombocytopenic purpura and atypical haemolytic uraemic syndrome [PDF]
Thrombotic thrombocytopenic purpura (TTP) and atypical haemolytic uraemic syndrome (aHUS) are acute, rare life-threatening thrombotic microangiopathies that require rapid diagnosis and treatment.
Goodship, T, Scully, M
core +1 more source

