Terminal complement inhibitor eculizumab in atypical hemolytic–uremic syndrome

CM Legendre, C Licht, P Muus… - … England Journal of …, 2013 - Mass Medical Soc
Background Atypical hemolytic–uremic syndrome is a genetic, life-threatening, chronic
disease of complement-mediated thrombotic microangiopathy. Plasma exchange or infusion …

[HTML][HTML] Overview of complement activation and regulation

M Noris, G Remuzzi - Seminars in nephrology, 2013 - Elsevier
Complement is an important component of the innate immune system that is crucial for
defense from microbial infections and for clearance of immune complexes and injured cells …

Complement: a key system for immune surveillance and homeostasis

D Ricklin, G Hajishengallis, K Yang, JD Lambris - Nature immunology, 2010 - nature.com
Nearly a century after the significance of the human complement system was recognized, we
have come to realize that its functions extend far beyond the elimination of microbes …

Genetics and outcome of atypical hemolytic uremic syndrome: a nationwide French series comparing children and adults

V Fremeaux-Bacchi, F Fakhouri, A Garnier… - Clinical Journal of the …, 2013 - journals.lww.com
Results Onset of aHUS occurred as frequently during adulthood (58.4%) as during
childhood (41.6%). The percentages of patients who developed the disease were 23 …

Complement activation, regulation, and molecular basis for complement‐related diseases

G Bajic, SE Degn, S Thiel, GR Andersen - The EMBO journal, 2015 - embopress.org
The complement system is an essential element of the innate immune response that
becomes activated upon recognition of molecular patterns associated with microorganisms …

[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) …

Management of thrombotic microangiopathy in pregnancy and postpartum: report from an international working group

F Fakhouri, M Scully, F Provôt, M Blasco… - Blood, The Journal …, 2020 - ashpublications.org
Pregnancy and postpartum are high-risk periods for different forms of thrombotic
microangiopathy (TMA). However, the management of pregnancy-associated TMA remains …

HUS and atypical HUS

TS Jokiranta - Blood, The Journal of the American Society of …, 2017 - ashpublications.org
Hemolytic uremic syndrome (HUS) is a thrombotic microangiopathy characterized by
intravascular hemolysis, thrombocytopenia, and acute kidney failure. HUS is usually …

Thrombotic thrombocytopenic purpura

BS Joly, P Coppo, A Veyradier - Blood, The Journal of the …, 2017 - ashpublications.org
Thrombotic thrombocytopenic purpura (TTP) is a rare and life-threatening thrombotic
microangiopathy characterized by microangiopathic hemolytic anemia, severe …

Genetic testing in the diagnosis of chronic kidney disease: recommendations for clinical practice

N Knoers, C Antignac, C Bergmann… - Nephrology Dialysis …, 2022 - academic.oup.com
The overall diagnostic yield of massively parallel sequencing–based tests in patients with
chronic kidney disease (CKD) is 30% for paediatric cases and 6–30% for adult cases. These …