Full Text Journal Articles by
Author Veronique Fremeaux Bacchi

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Atypical hemolytic and uremic syndrome due to C3 mutation in pancreatic islet transplantation: a case report.

Thibault Bahougne, Jérome Olagne, Marion Munch, Laura Braun-Parvez, Marie-Pierrette Chenard, Véronique Frémeaux-Bacchi, Sophie Caillard, Philippe Baltzinger, Michel Greget, Laurence Kessler, Bruno Moulin,

BACKGROUND:We here report on the first observation of a C3 mutation that is related to atypical hemolytic and uremic syndrome (aHUS), which occurred in a pancreatic islet transplant patient. Immunosuppressive treatments, such as calcineurin inhibitors, have been linked to undesirable effects like nephrotoxicity. CASE PRESENTATION:A 40-year-old man with brittle diabetes, ... Read more >>

BMC Nephrol (BMC nephrology)
[2020, 21(1):405]

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Post-partum acute kidney injury: sorting placental and non-placental thrombotic microangiopathies using the trajectory of biomarkers.

Fleuria Meibody, Matthieu Jamme, Vassilis Tsatsaris, François Provot, Jérôme Lambert, Véronique Frémeaux-Bacchi, Anne-Sophie Ducloy-Bouthors, Mercédès Jourdain, Yahsou Delmas, Pierre Perez, Julien Darmian, Alain Wynckel, Jean-Michel Rebibou, Paul Coppo, Cédric Rafat, Eric Rondeau, Luc Frimat, Alexandre Hertig,

BACKGROUND:Among the severe complications of preeclampsia (PE), acute kidney injury (AKI) is problematic if features of thrombotic microangiopathy (TMA) are present. Although a haemolysis enzyme liver low-platelets syndrome is considerably more frequent, it is vital to rule out a flare of atypical haemolytic and uraemic syndrome (aHUS). Our objective was ... Read more >>

Nephrol. Dial. Transplant. (Nephrology, dialysis, transplantation : official publication of the European Dialysis and Transplant Association - European Renal Association)
[2020, 35(9):1538-1546]

Cited: 1 time

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Practical management of C3 glomerulopathy and immunoglobulin-mediated MPGN: facts and uncertainties.

Fadi Fakhouri, Moglie Le Quintrec, Véronique Frémeaux-Bacchi,

In recent years, a substantial body of experimental and clinical work has been devoted to C3 glomerulopathy and immunoglobulin-mediated membranoproliferative glomerulonephritis. Despite the rapid accumulation of data, several uncertainties regarding these two rare forms of nephropathies persist. They concern their pathophysiology, classification, clinical course, relevance of biomarkers and of pathology ... Read more >>

Kidney Int. (Kidney international)
[2020, :]

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Anti-C5 antibody treatment for delayed hemolytic transfusion reactions in sickle cell disease.

Aline Floch, Alexandre Morel, Fabian Zanchetta-Balint, Catherine Cordonnier-Jourdin, Slimane Allali, Maximilien Grall, Ghislaine Ithier, Benjamin Carpentier, Sadaf Pakdaman, Jean-Claude Merle, Radjiv Goulabchand, Tackwa Khalifeh, Ana Berceanu, Cécile Helmer, Christelle Chantalat-Auger, Véronique Frémeaux-Bacchi, Marc Michel, Mariane de Montalembert, Armand Mekontso-Dessap, France Pirenne, Anoosha Habibi, Pablo Bartolucci,

Haematologica (Haematologica)
[2020, :]

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Anti-Factor B Antibodies and Acute Postinfectious GN in Children.

Sophie Chauvet, Romain Berthaud, Magali Devriese, Morgane Mignotet, Paula Vieira Martins, Tania Robe-Rybkine, Maria A Miteva, Aram Gyulkhandanyan, Amélie Ryckewaert, Ferielle Louillet, Elodie Merieau, Guillaume Mestrallet, Caroline Rousset-Rouvière, Eric Thervet, Julien Hogan, Tim Ulinski, Bruno O Villoutreix, Lubka Roumenina, Olivia Boyer, Véronique Frémeaux-Bacchi,

BACKGROUND:The pathophysiology of the leading cause of pediatric acute nephritis, acute postinfectious GN, including mechanisms of the pathognomonic transient complement activation, remains uncertain. It shares clinicopathologic features with C3 glomerulopathy, a complement-mediated glomerulopathy that, unlike acute postinfectious GN, has a poor prognosis. METHODS:This retrospective study investigated mechanisms of complement activation ... Read more >>

J. Am. Soc. Nephrol. (Journal of the American Society of Nephrology : JASN)
[2020, 31(4):829-840]

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Complement activation in sickle cell disease: Dependence on cell density, hemolysis and modulation by hydroxyurea therapy.

Lubka T Roumenina, Philippe Chadebech, Gwellaouen Bodivit, Paula Vieira-Martins, Anne Grunenwald, Idris Boudhabhay, Victoria Poillerat, Sadaf Pakdaman, Laurent Kiger, Alicia Jouard, Etienne Audureau, France Pirenne, Frédéric Galactéros, Véronique Frémeaux-Bacchi, Pablo Bartolucci,

The complement system is an innate immune defense cascade that can cause tissue damage when inappropriately activated. Evidence for complement over activation has been reported in small cohorts of patients with sickle cell disease (SCD). However, the mechanism governing complement activation in SCD has not been elucidated. Here, we observe ... Read more >>

Am. J. Hematol. (American journal of hematology)
[2020, 95(5):456-464]

Cited: 1 time

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Atypical hemolytic and uremic syndrome due to C3 mutation in pancreatic islet transplantation: a case report

Thibault Bahougne, Jérome Olagne, Marion Munch, Laura Braun-Parvez, Marie-Pierrette Chenard, Véronique Frémeaux-Bacchi, Sophie Caillard, Philippe Baltzinger, Michel Greget, Laurence Kessler, Bruno Moulin,

Background We here report on the first observation of a C3 mutation that is related to atypical hemolytic and uremic syndrome (aHUS), which occurred in a pancreatic islet transplant patient. Immunosuppressive treatments, such as calcineurin inhibitors, have been linked to undesirable effects like nephrotoxicity. Case presentation A 40-year-old man with ... Read more >>

BMC Nephrol (BMC nephrology)
[2020, 21(1):]

Cited: 0 times

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C3 glomerulonephritis in a patient treated with anti-PD-1 antibody.

Simon Ville, Christine Kandel-Aznar, Véronique Frémeaux-Bacchi, Fadi Fakhouri,

Eur. J. Cancer (European journal of cancer (Oxford, England : 1990))
[2020, 125:46-48]

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Shiga toxin-producing Escherichia coli-associated hemolytic uremic syndrome in solid organ transplant recipients.

Simon Ville, Amandine Ydee, Claire Garandeau, Emmanuel Canet, Adrien Tissot, Diego Cantarovich, Véronique Frémeaux-Bacchi, Patricia Mariani-Kurkdjian, François Provôt, Fadi Fakhouri,

Kidney Int. (Kidney international)
[2019, 96(6):1423-1424]

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

Fadi Fakhouri, Marie Scully, François Provôt, Miquel Blasco, Paul Coppo, Marina Noris, Kathy Paizis, David Kavanagh, Frederic Pene, Sol Quezada, Alexandre Hertig, Sébastien Kissling, Patrick O'Brien, Yahsou Delmas, Lorenzo Alberio, Norbert Winer, Agnès Veyradier, Spero R Cataland, Veronique Fremeaux-Bacchi, Chantal Loirat, Giuseppe Remuzzi, Vassilis Tsatsaris,

Pregnancy and postpartum are high-risk periods for different forms of thrombotic microangiopathy (TMA). However, the management of pregnancy-associated TMA remains ill-defined. This report by an international multidisciplinary (obstetricians, nephrologists, hematologists, intensivists, neonatologists and complement biologists) working group summarizes the current knowledge of these potentially severe disorders and proposes a practical ... Read more >>

Blood (Blood)
[2020, :]

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Circulating FH Protects Kidneys From Tubular Injury During Systemic Hemolysis.

Nicolas S Merle, Juliette Leon, Victoria Poillerat, Anne Grunenwald, Idris Boudhabhay, Samantha Knockaert, Tania Robe-Rybkine, Carine Torset, Matthew C Pickering, Sophie Chauvet, Veronique Fremeaux-Bacchi, Lubka T Roumenina,

Intravascular hemolysis of any cause can induce acute kidney injury (AKI). Hemolysis-derived product heme activates the innate immune complement system and contributes to renal damage. Therefore, we explored the role of the master complement regulator Factor H (FH) in the kidney's resistance to hemolysis-mediated AKI. Acute systemic hemolysis was induced ... Read more >>

Front Immunol (Frontiers in immunology)
[2020, 11:1772]

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Monitoring Complement Activation: The New Conundrum in Thrombotic Microangiopathies.

Fadi Fakhouri, Véronique Frémeaux-Bacchi,

Clin J Am Soc Nephrol (Clinical journal of the American Society of Nephrology : CJASN)
[2019, 14(12):1682-1683]

Cited: 0 times

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Atypical hemolytic uremic syndrome and hypertensive crisis.

Khalil El Karoui, Idris Boudhabhay, Véronique Frémeaux-Bacchi,

Kidney Int. (Kidney international)
[2019, 96(5):1239]

Cited: 0 times

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Use of Highly Individualized Complement Blockade Has Revolutionized Clinical Outcomes after Kidney Transplantation and Renal Epidemiology of Atypical Hemolytic Uremic Syndrome.

Julien Zuber, Marie Frimat, Sophie Caillard, Nassim Kamar, Philippe Gatault, Florent Petitprez, Lionel Couzi, Noemie Jourde-Chiche, Valérie Chatelet, Raphael Gaisne, Dominique Bertrand, Jamal Bamoulid, Magali Louis, Rebecca Sberro Soussan, David Navarro, Pierre-Francois Westeel, Luc Frimat, Charlotte Colosio, Antoine Thierry, Joseph Rivalan, Laetitia Albano, Nadia Arzouk, Emilie Cornec-Le Gall, Guillaume Claisse, Michelle Elias, Khalil El Karoui, Sophie Chauvet, Jean-Philippe Coindre, Jean-Philippe Rerolle, Leila Tricot, Johnny Sayegh, Cyril Garrouste, Christophe Charasse, Yahsou Delmas, Ziad Massy, Maryvonne Hourmant, Aude Servais, Chantal Loirat, Fadi Fakhouri, Claire Pouteil-Noble, Marie-Noelle Peraldi, Christophe Legendre, Eric Rondeau, Moglie Le Quintrec, Véronique Frémeaux-Bacchi,

BACKGROUND:Atypical hemolytic uremic syndrome (HUS) is associated with high recurrence rates after kidney transplant, with devastating outcomes. In late 2011, experts in France recommended the use of highly individualized complement blockade-based prophylaxis with eculizumab to prevent post-transplant atypical HUS recurrence throughout the country. METHODS:To evaluate this strategy's effect on kidney ... Read more >>

J. Am. Soc. Nephrol. (Journal of the American Society of Nephrology : JASN)
[2019, 30(12):2449-2463]

Cited: 3 times

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Neisseria meningitidis inside neutrophils, revealing properdin deficiency.

Benjamin Gillet, Nicolas Joram, Véronique Frémeaux Bacchi, Caroline Thomas, Marie C Béné, Soraya Wuillème,

Int. J. Infect. Dis. (International journal of infectious diseases : IJID : official publication of the International Society for Infectious Diseases)
[2020, 99:117-118]

Cited: 0 times

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Complement C5 inhibition in patients with COVID-19 - a promising target?

Regis Peffault de Latour, Anne Bergeron, Etienne Lengline, Thibault Dupont, Armance Marchal, Lionel Galicier, Nathalie de Castro, Louise Bondeelle, Michael Darmon, Clairelyne Dupin, Guillaume Dumas, Pierre Leguen, Isabelle Madelaine, Sylvie Chevret, Jean-Michel Molina, Elie Azoulay, Veronique Fremeaux-Bacchi, ,

Haematologica (Haematologica)
[2020, :]

Cited: 0 times

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Clinical and Genetic Spectrum of a Large Cohort With Total and Sub-total Complement Deficiencies.

Carine El Sissy, Jérémie Rosain, Paula Vieira-Martins, Pauline Bordereau, Aurélia Gruber, Magali Devriese, Loïc de Pontual, Muhamed-Kheir Taha, Claire Fieschi, Capucine Picard, Véronique Frémeaux-Bacchi,

The complement system is crucial for defense against pathogens and the removal of dying cells or immune complexes. Thus, clinical indications for possible complete complement deficiencies include, among others, recurrent mild or serious bacterial infections as well as autoimmune diseases (AID). The diagnostic approach includes functional activity measurements of the ... Read more >>

Front Immunol (Frontiers in immunology)
[2019, 10:1936]

Cited: 2 times

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The authors reply.

Alice Le Clech, Véronique Frémeaux-Bacchi, Fadi Fakhouri,

Kidney Int. (Kidney international)
[2019, 96(2):517-518]

Cited: 0 times

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Glomerulonephritis With Isolated C3 Deposits as a Manifestation of Subtotal Factor I Deficiency.

Idris Boudhabhay, Véronique Frémeaux-Bacchi, Lubka T Roumenina, Anissa Moktefi, Jean-Michel Goujon, Marie Matignon, Valérie Caudwell, Vincent Audard, Khalil El Karoui,

Kidney Int Rep (Kidney International Reports)
[2019, 4(9):1354-1358]

Cited: 0 times

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Haemolytic uraemic syndrome associated with non shiga toxin-producing Escherichia coli bacteraemia: a case report.

Stéphane Bally, Jacques Fourcade, Véronique Frémeaux-Bacchi,

BACKGROUND:Haemolytic uraemic syndrome (HUS) is a thrombotic microangiopathy (TMA) characterized by predominant renal involvement. Several types of HUS can be distinguished: the most frequent « typical » HUS, due to shiga toxin producing Escherichia coli (STEC), "atypical" HUS due to complement alternative pathway dysregulation and "secondary" HUS associated with various ... Read more >>

BMC Nephrol (BMC nephrology)
[2019, 20(1):157]

Cited: 0 times

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Impact of hypertensive emergency and rare complement variants on the presentation and outcome of atypical hemolytic uremic syndrome.

Khalil El Karoui, Idris Boudhabhay, Florent Petitprez, Paula Vieira-Martins, Fadi Fakhouri, Julien Zuber, Florence Aulagnon, Marie Matignon, Eric Rondeau, Laurent Mesnard, Jean-Michel Halimi, Véronique Frémeaux-Bacchi,

A typical hemolytic uremic syndrome (aHUS) is a prototypic thrombotic microangiopathy attributable to complement dysregulation. Hypertensive emergency, characterized by elevation of systolic (>180 mmHg) or diastolic (>120 mmHg) blood pressure together with end-organ damage, can cause thrombotic microangiopathy which may mimic aHUS. We retrospectively evaluated the clinical, biological and complement ... Read more >>

Haematologica (Haematologica)
[2019, 104(12):2501-2511]

Cited: 2 times

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Atypical and secondary hemolytic uremic syndromes have a distinct presentation and no common genetic risk factors.

Alice Le Clech, Noémie Simon-Tillaux, François Provôt, Yahsou Delmas, Paula Vieira-Martins, Sophie Limou, Jean-Michel Halimi, Moglie Le Quintrec, Ludivine Lebourg, Steven Grangé, Alexandre Karras, David Ribes, Noémie Jourde-Chiche, Eric Rondeau, Véronique Frémeaux-Bacchi, Fadi Fakhouri,

Secondary hemolytic uremic syndrome (HUS) is a heterogeneous group of thrombotic microangiopathies associated with various underlying conditions. Whether it belongs to the spectrum of complement-mediated HUS remains controversial. We analysed the presentation, outcome, and frequency of complement gene rare variants in a cohort of 110 patients with secondary HUS attributed ... Read more >>

Kidney Int. (Kidney international)
[2019, 95(6):1443-1452]

Cited: 4 times

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C5b9 Deposition in Glomerular Capillaries Is Associated With Poor Kidney Allograft Survival in Antibody-Mediated Rejection.

Valentin Goutaudier, Hélène Perrochia, Simon Mucha, Marie Bonnet, Sylvie Delmas, Florian Garo, Valérie Garrigue, Sébastien Lepreux, Vincent Pernin, Jean-Emmanuel Serre, Ilan Szwarc, Pierre Merville, Annie Ramounau-Pigot, Céline René, Jonathan Visentin, Bryan Paul Morgan, Véronique Frémeaux-Bacchi, Georges Mourad, Lionel Couzi, Moglie Le Quintrec,

C4d deposition in peritubular capillaries (PTC) reflects complement activation in antibody-mediated rejection (ABMR) of kidney allograft. However, its association with allograft survival is controversial. We hypothesized that capillary deposition of C5b9-indicative of complement-mediated injury-is a severity marker of ABMR. This pilot study aimed to determine the frequency, location and prognostic ... Read more >>

Front Immunol (Frontiers in immunology)
[2019, 10:235]

Cited: 0 times

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P-selectin drives complement attack on endothelium during intravascular hemolysis in TLR-4/heme-dependent manner.

Nicolas S Merle, Romain Paule, Juliette Leon, Marie Daugan, Tania Robe-Rybkine, Victoria Poillerat, Carine Torset, Véronique Frémeaux-Bacchi, Jordan D Dimitrov, Lubka T Roumenina,

Hemolytic diseases are frequently linked to multiorgan failure subsequent to vascular damage. Deciphering the mechanisms leading to organ injury upon hemolytic event could bring out therapeutic approaches. Complement system activation occurs in hemolytic disorders, such as sickle cell disease, but the pathological relevance and the acquisition of a complement-activating phenotype ... Read more >>

Proc. Natl. Acad. Sci. U.S.A. (Proceedings of the National Academy of Sciences of the United States of America)
[2019, 116(13):6280-6285]

Cited: 8 times

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Analysis of protein missense alterations by combining sequence- and structure-based methods.

Aram Gyulkhandanyan, Alireza R Rezaie, Lubka Roumenina, Nathalie Lagarde, Veronique Fremeaux-Bacchi, Maria A Miteva, Bruno O Villoutreix,

BACKGROUND:Different types of in silico approaches can be used to predict the phenotypic consequence of missense variants. Such algorithms are often categorized as sequence based or structure based, when they necessitate 3D structural information. In addition, many other in silico tools, not dedicated to the analysis of variants, can be ... Read more >>

Mol Genet Genomic Med (Molecular genetics & genomic medicine)
[2020, 8(4):e1166]

Cited: 1 time

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