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 …

Alternative complement pathway assessment in patients with atypical HUS

LT Roumenina, C Loirat, MA Dragon-Durey… - Journal of …, 2011 - Elsevier
The atypical Hemolytic Uremic Syndrome (aHUS) is a rare thrombotic microangiopathy
leading to end stage renal disease in approximately 60% of patients. Over the last decade, a …

[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 …

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] Acquired and genetic complement abnormalities play a critical role in dense deposit disease and other C3 glomerulopathies

A Servais, LH Noël, LT Roumenina, M Le Quintrec… - Kidney international, 2012 - Elsevier
Dense deposit disease and glomerulonephritis with isolated C3 deposits are
glomerulopathies characterized by deposits of C3 within or along the glomerular basement …

Pregnancy-associated hemolytic uremic syndrome revisited in the era of complement gene mutations

F Fakhouri, L Roumenina, F Provot… - Journal of the …, 2010 - journals.lww.com
In contrast to pregnancy-associated thrombotic thrombocytopenic purpura, the pathogenesis
and presentation of pregnancy-associated atypical hemolytic uremic syndrome (P-aHUS) …

Differential impact of complement mutations on clinical characteristics in atypical hemolytic uremic syndrome

AL Sellier-Leclerc, V Fremeaux-Bacchi… - Journal of the …, 2007 - journals.lww.com
Mutations in factor H (CFH), factor I (IF), and membrane cofactor protein (MCP) genes have
been described as risk factors for atypical hemolytic uremic syndrome (aHUS). This study …

Atypical haemolytic uraemic syndrome and mutations in complement regulator genes

MA Dragon-Durey, V Frémeaux-Bacchi - Springer seminars in …, 2005 - Springer
Haemolytic uraemic syndrome (HUS) is a thrombotic microangiopathy (TMA) disorder
characterised by the association of haemolytic anaemia, thrombocytopenia and acute renal …

Mutations in complement C3 predispose to development of atypical hemolytic uremic syndrome

V Frémeaux-Bacchi, EC Miller… - Blood, The Journal …, 2008 - ashpublications.org
Atypical hemolytic uremic syndrome (aHUS) is a disease of complement dysregulation. In
approximately 50% of patients, mutations have been described in the genes encoding the …

Heterozygous and homozygous factor h deficiencies associated with hemolytic uremic syndrome or membranoproliferative glomerulonephritis: report and genetic …

MA Dragon-Durey, V Fremeaux-Bacchi… - Journal of the …, 2004 - journals.lww.com
Factor H (FH) is the major regulatory protein of the complement alternative pathway, with a
structure consisting of a tandem array of 20 homologous units, called short consensus …