An international consensus approach to the management of atypical hemolytic uremic syndrome in children

C Loirat, F Fakhouri, G Ariceta, N Besbas, M Bitzan… - Pediatric …, 2016 - Springer
Atypical hemolytic uremic syndrome (aHUS) emerged during the last decade as a disease
largely of complement dysregulation. This advance facilitated the development of novel …

Haemolytic uraemic syndrome

M Michael, A Bagga, SE Sartain, RJH Smith - The Lancet, 2022 - thelancet.com
Haemolytic uraemic syndrome (HUS) is a heterogeneous group of diseases that result in a
common pathology, thrombotic microangiopathy, which is classically characterised by the …

[HTML][HTML] Efficacy and safety of eculizumab in atypical hemolytic uremic syndrome from 2-year extensions of phase 2 studies

C Licht, LA Greenbaum, P Muus, S Babu… - Kidney international, 2015 - Elsevier
Atypical hemolytic uremic syndrome (aHUS) is a rare, possibly life-threatening disease
characterized by platelet activation, hemolysis and thrombotic microangiopathy (TMA) …

[HTML][HTML] Terminal complement inhibitor eculizumab in adult patients with atypical hemolytic uremic syndrome: a single-arm, open-label trial

F Fakhouri, M Hourmant, JM Campistol… - American journal of …, 2016 - Elsevier
Background Atypical hemolytic uremic syndrome (aHUS) is a rare genetic life-threatening
disease of chronic uncontrolled complement activation leading to thrombotic …

[HTML][HTML] Eculizumab is a safe and effective treatment in pediatric patients with atypical hemolytic uremic syndrome

LA Greenbaum, M Fila, G Ardissino, SI Al-Akash… - Kidney international, 2016 - Elsevier
Atypical hemolytic uremic syndrome (aHUS) is caused by alternative complement pathway
dysregulation, leading to systemic thrombotic microangiopathy (TMA) and severe end-organ …

[HTML][HTML] An update for atypical haemolytic uraemic syndrome: diagnosis and treatment. A consensus document

JM Campistol, M Arias, G Ariceta, M Blasco… - Nefrología (English …, 2015 - Elsevier
Haemolytic uraemic syndrome (HUS) is a clinical entity defined as the triad of nonimmune
haemolytic anaemia, thrombocytopenia, and acute renal failure, in which the underlying …

[HTML][HTML] Eculizumab therapy in children with severe hematopoietic stem cell transplantation–associated thrombotic microangiopathy

S Jodele, T Fukuda, A Vinks, K Mizuno… - Biology of Blood and …, 2014 - Elsevier
We recently observed that dysregulation of the complement system may be involved in the
pathogenesis of hematopoietic stem cell transplantation–associated thrombotic …

Eculizumab reduces complement activation, inflammation, endothelial damage, thrombosis, and renal injury markers in aHUS

R Cofiell, A Kukreja, K Bedard, Y Yan… - Blood, The Journal …, 2015 - ashpublications.org
Atypical hemolytic uremic syndrome (aHUS) is a genetic, life-threatening disease
characterized by uncontrolled complement activation, systemic thrombotic microangiopathy …

Hemolytic uremic syndrome

C Mele, G Remuzzi, M Noris - Seminars in immunopathology, 2014 - Springer
Hemolytic uremic syndrome (HUS) is a thrombotic microangiopathy defined by
thrombocytopenia, nonimmune microangiopathic hemolytic anemia, and acute renal failure …

[HTML][HTML] The long-acting C5 inhibitor, ravulizumab, is effective and safe in pediatric patients with atypical hemolytic uremic syndrome naïve to complement inhibitor …

G Ariceta, BP Dixon, SH Kim, G Kapur, T Mauch… - Kidney international, 2021 - Elsevier
Ravulizumab, a long-acting complement C5 inhibitor engineered from eculizumab, allows
extending maintenance dosing from every 2–3 weeks to every 4–8 weeks depending on …