Endothelium structure and function in kidney health and disease

N Jourde-Chiche, F Fakhouri, L Dou, J Bellien… - Nature Reviews …, 2019 - nature.com
The kidney harbours different types of endothelia, each with specific structural and functional
characteristics. The glomerular endothelium, which is highly fenestrated and covered by a …

[HTML][HTML] Complement system part I–molecular mechanisms of activation and regulation

NS Merle, SE Church, V Fremeaux-Bacchi… - Frontiers in …, 2015 - frontiersin.org
Complement is a complex innate immune surveillance system, playing a key role in defense
against pathogens and in host homeostasis. The complement system is initiated by …

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] Shiga toxin-associated hemolytic uremic syndrome: A narrative review

A Joseph, A Cointe, P Mariani Kurkdjian, C Rafat… - Toxins, 2020 - mdpi.com
The severity of human infection by one of the many Shiga toxin-producing Escherichia coli
(STEC) is determined by a number of factors: the bacterial genome, the capacity of human …

[HTML][HTML] Monogenic causes of chronic kidney disease in adults

DM Connaughton, C Kennedy, S Shril, N Mann… - Kidney international, 2019 - Elsevier
Approximately 500 monogenic causes of chronic kidney disease (CKD) have been
identified, mainly in pediatric populations. The frequency of monogenic causes among …

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 …

Relative role of genetic complement abnormalities in sporadic and familial aHUS and their impact on clinical phenotype

M Noris, J Caprioli, E Bresin, C Mossali… - Clinical Journal of the …, 2010 - journals.lww.com
Background and objectives: Hemolytic uremic syndrome (HUS) is characterized by
microangiopathic hemolytic anemia, thrombocytopenia, and renal impairment. Most …

Atypical hemolytic–uremic syndrome

M Noris, G Remuzzi - New England Journal of Medicine, 2009 - Mass Medical Soc
The hemolytic–uremic syndrome, which is characterized by nonimmune hemolytic anemia,
thrombocytopenia, and renal impairment, occurs most frequently in young children. Most …