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 …

Thrombotic thrombocytopenic purpura

JA Kremer Hovinga, P Coppo, B Lämmle… - Nature reviews Disease …, 2017 - nature.com
Thrombotic thrombocytopenic purpura (TTP; also known as Moschcowitz disease) is
characterized by the concomitant occurrence of often severe thrombocytopenia …

[HTML][HTML] Sepsis and septic shock: endothelial molecular pathogenesis associated with vascular microthrombotic disease

JC Chang - Thrombosis journal, 2019 - Springer
In addition to protective “immune response”, sepsis is characterized by destructive
“endothelial response” of the host, leading to endotheliopathy and its molecular dysfunction …

The genetic fingerprint of susceptibility for transplant-associated thrombotic microangiopathy

S Jodele, K Zhang, F Zou, B Laskin… - Blood, The Journal …, 2016 - ashpublications.org
Transplant-associated thrombotic microangiopathy (TA-TMA) occurs frequently after
hematopoietic stem cell transplantation (HSCT) and can lead to significant morbidity and …

[HTML][HTML] Variable eculizumab clearance requires pharmacodynamic monitoring to optimize therapy for thrombotic microangiopathy after hematopoietic stem cell …

S Jodele, T Fukuda, K Mizuno, AA Vinks… - Biology of blood and …, 2016 - Elsevier
Thrombotic microangiopathy (TMA) after hematopoietic stem cell transplantation (HSCT)
associated with terminal complement activation, as measured by elevated plasma terminal …

Spectrum of complement-mediated thrombotic microangiopathies: pathogenetic insights identifying novel treatment approaches

M Riedl, F Fakhouri, M Le Quintrec… - … in thrombosis and …, 2014 - thieme-connect.com
Thrombotic microangiopathy (TMA) is a rare but severe disorder characterized by
endothelial cell activation and thrombus formation. It manifests with the triad of hemolytic …

Reincarnation of ancient links between coagulation and complement

EM Conway - Journal of Thrombosis and Haemostasis, 2015 - Wiley Online Library
Throughout evolution, organisms have developed means to contain wounds by
simultaneously limiting bleeding and eliminating pathogens and damaged host cells via the …

[HTML][HTML] Diagnosis of complement alternative pathway disorders

A Angioi, FC Fervenza, S Sethi, Y Zhang, RJ Smith… - Kidney international, 2016 - Elsevier
Kidney diseases resulting from abnormal control of the complement alternative pathway
include atypical hemolytic uremic syndrome, C3 glomerulonephritis, and dense-deposit …

Complement factor B mutations in atypical hemolytic uremic syndrome—disease-relevant or benign?

MC Marinozzi, L Vergoz, T Rybkine, S Ngo… - Journal of the …, 2014 - journals.lww.com
Atypical hemolytic uremic syndrome (aHUS) is a genetic ultrarare renal disease associated
with overactivation of the alternative pathway of complement. Four gain-of-function …

Modified Ham test for atypical hemolytic uremic syndrome

E Gavriilaki, X Yuan, Z Ye, AJ Ambinder… - Blood, The Journal …, 2015 - ashpublications.org
Atypical hemolytic uremic syndrome (aHUS) is a thrombotic microangiopathy (TMA)
characterized by excessive activation of the alternative pathway of complement (APC) …