C3 glomerulopathy—understanding a rare complement-driven renal disease
The C3 glomerulopathies are a group of rare kidney diseases characterized by complement
dysregulation occurring in the fluid phase and in the glomerular microenvironment, which …
dysregulation occurring in the fluid phase and in the glomerular microenvironment, which …
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) …
microangiopathies (TMAs), particularly atypical haemolytic uraemic syndrome (aHUS) …
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 …
syndrome (aHUS) remains poorly defined. We conducted a prospective national multicenter …
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 …
chronic kidney disease (CKD) is 30% for paediatric cases and 6–30% for adult cases. These …
The role of complement in kidney disease
V Petr, JM Thurman - Nature Reviews Nephrology, 2023 - nature.com
The complement cascade comprises soluble and cell surface proteins and is an important
arm of the innate immune system. Once activated, the complement system rapidly generates …
arm of the innate immune system. Once activated, the complement system rapidly generates …
[HTML][HTML] Complement in secondary thrombotic microangiopathy
LMP Palma, M Sridharan, S Sethi - Kidney international reports, 2021 - Elsevier
Thrombotic microangiopathy (TMA) is a condition characterized by thrombocytopenia and
microangiopathic hemolytic anemia (MAHA) with varying degrees of organ damage in the …
microangiopathic hemolytic anemia (MAHA) with varying degrees of organ damage in the …
Genetic justification of severe COVID-19 using a rigorous algorithm
E Gavriilaki, PG Asteris, T Touloumenidou… - Clinical …, 2021 - Elsevier
Recent studies suggest excessive complement activation in severe coronavirus disease-19
(COVID-19). The latter shares common characteristics with complement-mediated …
(COVID-19). The latter shares common characteristics with complement-mediated …
Genetic variability shapes the alternative pathway complement activity and predisposition to complement‐related diseases
S Rodríguez de Córdoba - Immunological Reviews, 2023 - Wiley Online Library
The implementation of next‐generation sequencing technologies has provided a sharp
picture of the genetic variability in the components and regulators of the alternative pathway …
picture of the genetic variability in the components and regulators of the alternative pathway …
[HTML][HTML] Complementopathies and precision medicine
E Gavriilaki, RA Brodsky - The Journal of clinical …, 2020 - Am Soc Clin Investig
The renaissance of complement diagnostics and therapeutics has introduced precision
medicine into a widened field of complement-mediated diseases. In particular, complement …
medicine into a widened field of complement-mediated diseases. In particular, complement …
[HTML][HTML] Regulation of regulators: Role of the complement factor H-related proteins
M Cserhalmi, A Papp, B Brandus, B Uzonyi… - Seminars in …, 2019 - Elsevier
The complement system, while being an essential and very efficient effector component of
innate immunity, may cause damage to the host and result in various inflammatory …
innate immunity, may cause damage to the host and result in various inflammatory …