Search Results - "Guffroy, Aurelien"

Worldwide trends in all-cause mortality of auto-immune systemic diseases between 2001 and 2014 by Scherlinger, Marc, Mertz, Philippe, Sagez, Flora, Meyer, Alain, Felten, Renaud, Chatelus, Emmanuel, Javier, Rose-Marie, Sordet, Christelle, Martin, Thierry, Korganow, Anne-Sophie, Guffroy, Aurélien, Poindron, Vincent, Richez, Christophe, Truchetet, Marie-Elise, Blanco, Patrick, Schaeverbeke, Thierry, Sibilia, Jean, Devillers, Hervé, Arnaud, Laurent

“…To describe changes in the 2001–2014 mortality of 6 autoimmune systemic diseases (AISDs), namely Systemic Lupus Erythematosus (SLE), Systemic Sclerosis (SSc),…”
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Anti-Ku syndrome with elevated CK and anti-Ku syndrome with anti-dsDNA are two distinct entities with different outcomes by Spielmann, Lionel, Nespola, Benoit, Séverac, François, Andres, Emmanuel, Kessler, Romain, Guffroy, Aurélien, Poindron, Vincent, Martin, Thierry, Geny, Bernard, Sibilia, Jean, Meyer, Alain

“…To refine the spectrum of anti-Ku-associated disease, a condition that is equivocally described by current diagnostic criteria for connective tissue diseases…”
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Beyond Anti-viral Effects of Chloroquine/Hydroxychloroquine by Gies, Vincent, Bekaddour, Nassima, Dieudonné, Yannick, Guffroy, Aurélien, Frenger, Quentin, Gros, Frédéric, Rodero, Mathieu Paul, Herbeuval, Jean-Philippe, Korganow, Anne-Sophie

“…As the world is severely affected by COVID-19 pandemic, the use of chloroquine and hydroxychloroquine in prevention or for the treatment of patients is allowed…”
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Immune Defect in Adults With Down Syndrome: Insights Into a Complex Issue by Dieudonné, Yannick, Uring-Lambert, Beatrice, Jeljeli, Mohamed Maxime, Gies, Vincent, Alembik, Yves, Korganow, Anne-Sophie, Guffroy, Aurélien

“…Children with Down syndrome (DS) suffer from recurrent respiratory infections, which represent the leading cause of mortality during childhood. This…”
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Follow-up of COVID-19 patients: LA is transient but other aPLs are persistent by Vollmer, Olivier, Tacquard, Charles, Dieudonné, Yannick, Nespola, Benoit, Sattler, Laurent, Grunebaum, Lélia, Gies, Vincent, Radosavljevic, Mirjana, Kaeuffer, Charlotte, Hansmann, Yves, Weber, Jean-Christophe, Martin, Thierry, Arnaud, Laurent, Morel, Olivier, Guffroy, Aurélien, Collange, Olivier, Mertes, Paul Michel, Korganow, Anne-Sophie, Delabranche, Xavier, Poindron, Vincent

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IKZF1 Loss-of-Function Variant Causes Autoimmunity and Severe Familial Antiphospholipid Syndrome by Dieudonné, Yannick, Guffroy, Aurélien, Vollmer, Olivier, Carapito, Raphael, Korganow, Anne-Sophie

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Incidence and predictors of COVID-19 and flares in patients with rare autoimmune diseases: a systematic survey and serological study at a national reference center in France by Felten, Renaud, Scherlinger, Marc, Guffroy, Aurélien, Poindron, Vincent, Meyer, Alain, Giannini, Margherita, Korganow, Anne-Sophie, Sordet, Christelle, Chatelus, Emmanuel, Javier, Rose-Marie, Meyer, Aurore, Pijnenburg, Luc, Kleinmann, Jean-François, Gottenberg, Jacques-Eric, Sibilia, Jean, Martin, Thierry, Arnaud, Laurent

“…Abstract Background The risk of severe COVID-19 and its determinants remain largely unknown in patients with autoimmune and inflammatory rheumatic diseases…”
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T1 mapping cardiac magnetic resonance imaging frequently detects subclinical diffuse myocardial fibrosis in systemic sclerosis patients by Poindron, Vincent, Chatelus, Emmanuel, Canuet, Matthieu, Gottenberg, Jacques-Eric, Arnaud, Laurent, Gangi, Afshin, Gavand, Pierre-Edouard, Guffroy, Aurélien, Korganow, Anne-Sophie, Germain, Philippe, Sibilia, Jean, El Ghannudi, Soraya, Martin, Thierry

“…cardiac involvement is the second most frequent systemic sclerosis (SSc) related cause of death. It remains mostly asymptomatic in the early stage and is…”
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B cells differentiate in human thymus and express AIRE by Gies, Vincent, Guffroy, Aurélien, MD, Danion, François, MD, Billaud, Philippe, MD, Keime, Céline, PhD, Fauny, Jean-Daniel, PhD, Susini, Sandrine, PhD, Soley, Anne, LT, Martin, Thierry, MD, PhD, Pasquali, Jean-Louis, MD, PhD, Gros, Frédéric, PhD, André-Schmutz, Isabelle, PhD, Soulas-Sprauel, Pauline, PharmD, PhD, Korganow, Anne-Sophie, MD, PhD

“…If relevant, AIRE expression in thymic B cells may repress or induce the expression of other genes, as the induction of TRAs expression in medullary thymic…”
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Infection risk among adults with down syndrome: a two group series of 101 patients in a tertiary center by Guffroy, Aurélien, Dieudonné, Yannick, Uring-Lambert, Beatrice, Goetz, Joelle, Alembik, Yves, Korganow, Anne-Sophie

“…Down syndrome (DS) is the most common form of viable chromosomal abnormality. DS is associated with recurrent infections, auto-immunity and malignancies in…”
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10 most important contemporary challenges in the management of SLE by Felten, Renaud, Sagez, Flora, Gavand, Pierre-Edouard, Martin, Thierry, Korganow, Anne-Sophie, Sordet, Christelle, Javier, Rose-Marie, Soulas-Sprauel, Pauline, Rivière, Marianne, Scher, Florence, Poindron, Vincent, Guffroy, Aurélien, Arnaud, Laurent

“…From a 1-year survival of less than 50% before the discovery of glucocorticoids to over 90% at 10 years in most dedicated centres, the spectrum of SLE has…”
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Giant cell arteritis associated with scalp, tongue or lip necrosis: A French multicenter case control study by Chehem Daoud Chehem, Ferida, de Mornac, Donatienne, Feuillet, Fanny, Liozon, Eric, Samson, Maxime, Bonnotte, Bernard, de Boysson, Hubert, Guffroy, Aurélien, Balquet, Marie-Hélène, Ledoult, Emmanuel, Lavigne, Christian, Trefond, Ludovic, Smets, Perrine, Bodard, Quentin, Fenot, Marion, Richez, Christophe, Duffau, Pierre, Guillaud, Constance, Espitia, Olivier, Agard, Christian

“…Scalp, tongue and/or lip necrosis are rare complications of GCA. To describe characteristics and outcome of patients with giant cell arteritis (GCA) -related…”
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Transitional B cells in quiescent SLE: An early checkpoint imprinted by IFN by Dieudonné, Yannick, Gies, Vincent, Guffroy, Aurélien, Keime, Céline, Bird, Anna K., Liesveld, Jane, Barnas, Jennifer L., Poindron, Vincent, Douiri, Nawal, Soulas-Sprauel, Pauline, Martin, Thierry, Meffre, Eric, Anolik, Jennifer H., Korganow, Anne-Sophie

“…Systemic lupus (SLE) is characterized by a break of B cell tolerance that plays a central role in disease pathophysiology. An early checkpoint defect occurs at…”
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Allogeneic stem cell transplantation compared to conservative management in adults with inborn errors of immunity by Cheminant, Morgane, Fox, Thomas A., Alligon, Mickael, Bouaziz, Olivier, Neven, Bénédicte, Moshous, Despina, Blanche, Stéphane, Guffroy, Aurélien, Fieschi, Claire, Malphettes, Marion, Schleinitz, Nicolas, Perlat, Antoinette, Viallard, Jean-François, Dhedin, Nathalie, Sarrot-Reynauld, Françoise, Durieu, Isabelle, Humbert, Sébastien, Fouyssac, Fanny, Barlogis, Vincent, Carpenter, Benjamin, Hough, Rachael, Laurence, Arian, Marçais, Ambroise, Chakraverty, Ronjon, Hermine, Olivier, Fischer, Alain, Burns, Siobhan O., Mahlaoui, Nizar, Morris, Emma C., Suarez, Felipe

“…•Nontransplanted adults with severe IEI have an ongoing risk of severe events compared to transplanted patients.•AlloSCT prevents progressive morbidity…”
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Direct examination of Gram-negative spiral-shaped rods: what is hidden behind the Campylobacter spp.? by Boyer, Pierre H., Talagrand-Reboul, Émilie, Guffroy, Aurélien, Schramm, Fréderic, Jaulhac, Benoît, Grillon, Antoine

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Inadequate Immune Humoral Response against JC Virus in Progressive Multifocal Leukoencephalopathy Non-Survivors by Solis, Morgane, Guffroy, Aurélien, Lersy, François, Soulier, Eric, Gallais, Floriane, Renaud, Mathilde, Douiri, Nawal, Argemi, Xavier, Hansmann, Yves, De Sèze, Jérôme, Kremer, Stéphane, Fafi-Kremer, Samira

“…JC virus (JCV) causes progressive multifocal leukoencephalopathy (PML) in immunosuppressed patients. There is currently no effective specific antiviral…”
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Complex Allele with Additive Gain-of-Function STING1 Variants in a Patient with Cavitating Lung Lesions and Aspergillosis by Guffroy, Aurélien, Dieudonné, Yannick, Gies, Vincent, Danion, François

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Case Report: A New Gain-of-Function Mutation of STAT1 Identified in a Patient With Chronic Mucocutaneous Candidiasis and Rosacea-Like Demodicosis: An Emerging Association by Martinot, Martin, Korganow, Anne Sophie, Wald, Mathieu, Second, Julie, Birckel, Elodie, Mahé, Antoine, Souply, Laurent, Mohseni-Zadeh, Mahsa, Droy, Laure, Tarabeux, Julien, Okada, Satoshi, Migaud, Mélanie, Puel, Anne, Guffroy, Aurelien

“…Heterozygous missense mutations leading to a gain of function (GOF) are the most frequent genetic cause of chronic mucocutaneous candidiasis (CMC). We describe…”
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Case Report: Acquired Disseminated BCG in the Context of a Delayed Immune Reconstitution After Hematological Malignancy by Gies, Vincent, Dieudonné, Yannick, Morel, Florence, Sougakoff, Wladimir, Carapito, Raphaël, Martin, Aurélie, Weingertner, Noëlle, Jacquel, Léa, Hubele, Fabrice, Kuhnert, Cornelia, Jung, Sophie, Schramm, Frederic, Boyer, Pierre, Hansmann, Yves, Danion, François, Korganow, Anne-Sophie, Guffroy, Aurélien

“…Disseminated infections due to Bacillus Calmette-Guérin (BCG) are unusual and occur mostly in patients with inborn error of immunity (IEI) or acquired…”
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Progressive Multifocal Leukoencephalopathy in Primary Immunodeficiencies by Hadjadj, Jérôme, Guffroy, Aurélien, Delavaud, Christophe, Taieb, Guillaume, Meyts, Isabelle, Fresard, Anne, Streichenberger, Nathalie, L’Honneur, Anne-Sophie, Rozenberg, Flore, D’Aveni, Maud, Aguilar, Claire, Rosain, Jérémie, Picard, Capucine, Mahlaoui, Nizar, Lecuit, Marc, Hermine, Olivier, Lortholary, Olivier, Suarez, Felipe

“…Purpose Progressive multifocal leukoencephalopathy (PML) is a rare but severe demyelinating disease caused by the polyomavirus JC (JCV) in immunocompromised…”
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