Haemolytic uraemic syndrome

F Fakhouri, J Zuber, V Frémeaux-Bacchi, C Loirat - The Lancet, 2017 - thelancet.com
Haemolytic uraemic syndrome is a form of thrombotic microangiopathy affecting
predominantly the kidney and characterised by a triad of thrombocytopenia, mechanical …

[HTML][HTML] Complement system part II: role in immunity

NS Merle, R Noe, L Halbwachs-Mecarelli… - Frontiers in …, 2015 - frontiersin.org
The complement system has been considered for a long time as a simple lytic cascade,
aimed to kill bacteria infecting the host organism. Nowadays, this vision has changed and it …

[HTML][HTML] Atypical hemolytic uremic syndrome and C3 glomerulopathy: conclusions from a “Kidney Disease: Improving Global Outcomes”(KDIGO) Controversies …

THJ Goodship, HT Cook, F Fakhouri, FC Fervenza… - Kidney international, 2017 - Elsevier
In both atypical hemolytic uremic syndrome (aHUS) and C3 glomerulopathy (C3G)
complement plays a primary role in disease pathogenesis. Herein we report the outcome of …

Eculizumab discontinuation in children and adults with atypical hemolytic-uremic syndrome: a prospective multicenter study

F Fakhouri, M Fila, A Hummel, D Ribes… - Blood, The Journal …, 2021 - ashpublications.org
The optimal duration of eculizumab treatment in patients with atypical hemolytic uremic
syndrome (aHUS) remains poorly defined. We conducted a prospective national multicenter …

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 microangiopathy in aHUS and beyond: clinical clues from complement genetics

F Fakhouri, V Frémeaux-Bacchi - Nature Reviews Nephrology, 2021 - nature.com
Studies of complement genetics have changed the landscape of thrombotic
microangiopathies (TMAs), particularly atypical haemolytic uraemic syndrome (aHUS) …

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 …

[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 …

Atypical aHUS: state of the art

CM Nester, T Barbour, SR de Cordoba… - Molecular …, 2015 - Elsevier
Tremendous advances in our understanding of the thrombotic microangiopathies (TMAs)
have revealed distinct disease mechanisms within this heterogeneous group of diseases. As …

STEC-HUS, atypical HUS and TTP are all diseases of complement activation

M Noris, F Mescia, G Remuzzi - Nature Reviews Nephrology, 2012 - nature.com
Haemolytic uraemic syndrome (HUS) and thrombotic thrombocytopaenic purpura (TTP) are
diseases characterized by microvascular thrombosis, with consequent thrombocytopaenia …