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1. Echinocandins Susceptibility Patterns of 2,787 Yeast Isolates: Importance of the Thresholds for the Detection of FKS Mutations

Author: Desnos-Ollivier, Marie, Bretagne, Stéphane, Lortholary, Olivier, Dromer, Françoise, French Mycoses Study Group, The, Mycologie moléculaire - Molecular Mycology, Institut Pasteur [Paris] (IP)-Centre National de la Recherche Scientifique (CNRS)-Université Paris Cité (UPCité), Centre National de Référence Mycoses Invasives et Antifongiques - National Reference Center Invasive Mycoses & Antifungals (CNRMA), Institut Pasteur [Paris] (IP)-Université Paris Cité (UPCité), Laboratoire de Parasitologie-Mycologie [CHU Saint Louis, Paris], Groupe Hospitalier Saint Louis - Lariboisière - Fernand Widal [Paris], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP), Centre d'infectiologie Necker-Pasteur [CHU Necker], Institut Pasteur [Paris] (IP)-CHU Necker - Enfants Malades [AP-HP], Imagine - Institut des maladies génétiques (IHU) (Imagine - U1163), Institut National de la Santé et de la Recherche Médicale (INSERM)-Université Paris Cité (UPCité), This work was supported by Santé Publique France and Institut Pasteur., Members of the French Mycoses Study Group who contributed to the data are, in alphabetical order of the cities, all the French microbiologists and mycologists who sent isolates for characterization of unusual antifungal susceptibility profiles or to contribute to the ongoing surveillance program on the epidemiology of invasive fungal infections in France (YEASTS and RESSIF programs): N. Brieu (CH Aix), T. Chouaki, C. Damiani, A. Totet (CHU Amiens), J. P. Bouchara, D. Chabasse, M. Pihet (CHU Angers), S. Bland (CH Annecy), V. Blanc (CH Antibes), S. Branger (CH Avignon), A. P. Bellanger, L. Millon (CHU Besançon), C. Plassart (CH Beauvais), I. Poilane (Hôpital Jean Verdier, Bondy), I. Accoceberry, L. Delhaes, B. Couprie, F. Gabriel (CH Bordeaux), J. Dunand, A. L. Roux, V. Sivadon-Tardy (Hôpital Ambroise Paré, Boulogne Billancourt), F. Laurent (CH, Bourg en Bresse), S. Legal, E. Moalic, G. Nevez, D. Quinio (CHU Brest), M. Cariou (CH Bretagne Sud), J. Bonhomme, C. Duhamel (CHU, Caen), B. Podac (CH, Chalon sur Saône), S. Lechatch (CH, Charleville-Mézières), C. Soler (Hopital d’Instruction des armées, Clamart), M. Cambon, C. Nourrisson, P. Poirier, D. Pons (CHU, Clermont Ferrand), O. Augereau, I. Grawey (CH, Colmar), N. Fauchet (CHIC, Créteil), A. Bonnin, F. Dalle (CHU, Dijon), P. Cahen, P. Honderlick (CMC, Foch), N. Desbois, C. Miossec (CHU, Fort de France), J. L. Hermann (Hôpital Raymond Poincaré, Garches), M. Cornet, R. Grillot, B. Lebeau, D. Maubon, H. Pelloux (CHU, Grenoble), M. Nicolas (CHU, Guadeloupe), C. Aznar, D. Blanchet, J. F. Carod, M. Demar, (CHU, Guyane), A. Angoulvant (Hôpital Bicêtre, le Kremlin Bicêtre), C. Ciupek (CH, Le Mans), A. Gigandon (Hôpital Marie Lannelongue, Le Plessis Robinson), B. Bouteille (CH Limoges), E. Frealle, D. Poulain, B. Sendid (CHU Lille), D. Dupont, J. Menotti, F. Persat, M.-A. Piens, M. Wallon (CHU, Lyon), C. Cassagne, S. Ranque (CHU, Marseille), T. Benoit-Cattin, L. Collet (CH Mayotte), A. Fiacre (CH Meaux), N. Bourgeois, L. Lachaud, P. Rispail, Y. Sterkers (CHU, Montpellier), M. Machouart (CHU, Nancy), F. Gay-Andrieu, P. Lepape, F. Morio (CHU, Nantes), O. Moquet (CH, Nevers), S. Lefrançois (Hôpital Américain, Neuilly), M. Sasso (CHU, Nimes), F. Reibel (GH, Nord-Essone), M. Gari-Toussaint, L. Hasseine (CHU Nice), L. Bret, D. Poisson (CHR Orléans), S. Brun (Hôpital Avicenne, Paris), C. Bonnal, C. Chochillon, S. Houzé (Hôpital Bichat, Paris), A. Paugam (Hôpital Cochin, Paris), N. Ait-Ammar, F. Botterel, R. Chouk (CHU Henri Mondor, Paris), M. E. Bougnoux, E. Sitterle (Hôpital Necker, Paris), A. Fekkar, R. Piarroux (Hôpital Pitié Salpêtrière, Paris), J. Guitard, C. Hennequin, J.-L. Poirot (Hôpital St Antoine, Paris), M. Gits-Muselli, S. Hamane, C. Lacroix (Hôpital Saint Louis, Paris), S. Bonacorsi, P. Mariani (Hôpital Robert Debré, Paris), D. Moissenet (Hôpital Trousseau, Paris), C. Kauffmann-Lacroix, A. Minoza, E. Perraud, M. H. Rodier (CHU Poitiers), G. Colonna (CH, Porto Vecchio), A. Huguenin, D. Toubas (CHU Reims), S. Chevrier, J. P. Gangneux, F. Robert-Gangneux, C. Guigen (CHU Rennes), O. Belmonte, G. Hoarau, M. C. Jaffar Bandjee, J. Jaubert, S. Picot, N. Traversier (CHU Réunion), L. Favennec, G. Gargala (CHU, Rouen), N. Godineau, C. Tournus (CH, St Denis), C. Mahinc, H. Raberin (CHU, St Etienne), V. Letscher Bru (CHU, Strasbourg), S. Cassaing (CHU, Toulouse), P. Patoz (CH Tourcoing), E. Bailly, J. Chandenier, G. Desoubeaux (CHU Tours), F. Moreau (CH Troyes), P. Munier (CH Valence), E. Mazars (CH Valenciennes), O. Eloy (CH Versailles), E. Chachaty (Institut Gustave Roussy, Villejuif), and and members of the NRCMA (Institut Pasteur, Paris): A. Bertho, C. Blanc, A. Boullié, C. Gautier, V. Geolier, D. Hoinard, and D. Raoux-Barbot for technical help, and K. Boukris-Sitbon, F. Lanternier, A. Alanio, and D. Garcia-Hermoso for their expertise and contribution to the surveillance programs.
Subjects: Pharmacology, Antifungal Agents, [SDV]Life Sciences [q-bio], yeasts, Microbial Sensitivity Tests, antifungal resistance, Anidulafungin, bacterial infections and mycoses, rare yeast, common yeast, Echinocandins, Lipopeptides, Infectious Diseases, Susceptibility, Caspofungin, Drug Resistance, Fungal, Mutation, Micafungin, polycyclic compounds, FKS mutation, Humans, Candidiasis, Invasive, Pharmacology (medical), MIC distribution
Abstract: International audience; Since echinocandins are recommended as first line therapy for invasive candidiasis, detection of resistance, mainly due to alteration in FKS protein, is of main interest. EUCAST AFST recommends testing both MIC of anidulafungin and micafungin, and breakpoints (BPs) have been proposed to detect echinocandin-resistant isolates. We analyzed MIC distribution for all three available echinocandins of 2,787 clinical yeast isolates corresponding to 5 common and 16 rare yeast species, using the standardized EUCAST method for anidulafungin and modified for caspofungin and micafungin (AM3-MIC). In our database, 64 isolates of common pathogenic species were resistant to anidulafungin, according to the EUCAST BP, and/or to caspofungin, using our previously published threshold (AM3-MIC ≥ 0.5 mg/L). Among these 64 isolates, 50 exhibited 21 different FKS mutations. We analyzed the capacity of caspofungin AM3-MIC and anidulafungin MIC determination in detecting isolates with FKS mutation. They were always identified using caspofungin AM3-MIC and the local threshold while some isolates were misclassified using anidulafungin MIC and EUCAST threshold. However, both methods misclassified four wild-type C. glabrata as resistant. Based on a large data set from a single center, the use of AM3-MIC testing for caspofungin looks promising in identifying non-wild-type C. albicans, C. tropicalis and P. kudiravzevii isolates, but additional multicenter comparison is mandatory to conclude on the possible superiority of AM3-MIC testing compared to the EUCAST method.
Published: 2022
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2. Azoles susceptibility profiles of more than 9,000 clinical yeast isolates belonging to 40 common and rare species

Author: Olivier Lortholary, Françoise Dromer, Stéphane Bretagne, Marie Desnos-Ollivier, Mycologie moléculaire - Molecular Mycology, Institut Pasteur [Paris] (IP)-Centre National de la Recherche Scientifique (CNRS), Service des Maladies infectieuses et tropicales [CHU Necker], CHU Necker - Enfants Malades [AP-HP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP), Laboratoire de Parasitologie-Mycologie [CHU Saint Louis, Paris], Groupe Hospitalier Saint Louis - Lariboisière - Fernand Widal [Paris], Université Paris Cité (UPCité), Institut Pasteur and Santé Publique France., Members of the French Mycoses Study group who contributed to the data are in alphabetical order of the cities, all the French microbiologists and mycologists who sent isolates for characterization of unusual antifungal susceptibility profiles or to contribute to the ongoing surveillance program on the epidemiology of invasive fungal infections in France (YEASTS and RESSIF programs): N. Brieu (CH Aix), T. Chouaki, C. Damiani, A. Totet (CHU Amiens, J. P. Bouchara, M. Pihet (CHU Angers), S. Bland (CH Annecy), V. Blanc (CH Antibes), S. Branger (CH Avignon), A. P. Bellanger, L. Million (CHU Besançon), C. Plassart (CH Beauvais), I. Poilane (hôpital Jean Verdier, Bondy), I. Accoceberry, L. Delhaes, F. Gabriel (CH Bordeaux), A. L. Roux, V. Sivadon-Tardy (hôpital Ambroise Paré, Boulogne Billancourt), F. Laurent (CH, Bourg en Bresse), S. Legal, E. Moalic, G. Nevez, D. Quinio (CHU Brest), M. Cariou (CH Bretagne Sud), J. Bonhomme (CHU, Caen), B. Podac (CH, Chalon sur Saône), S. Lechatch (CH, Charleville-Mézières), C. Soler (hopital d’Instruction des armées, Clamart), P. Poirier, C. Nourrisson (CHU, Clermont Ferrand), O. Augereau (CH, Colmar), N. Fauchet (CHIC, Créteil), F. Dalles (CHU, Dijon), P. Cahen (CMC, Foch), N. Desbois, C. Miossec (CHU, Fort de France), J. L. Hermann (hôpital Raymond Poincaré, Garches), M. Cornet, D. Maubon, H. Pelloux (CHU, Grenoble), M. Nicolas (CHU,Guadeloupe), C. Aznar, D. Blanchet, J. F. Carod, M. Demar, (CHU, Guyane), A. Angoulvant (hôpital Bicêtre, le Kremlin Bicêtre), C. Ciupek (CH, Le Mans), A. Gigandon (hôpital Marie Lannelongue, Le Plessis Robinson), B. Bouteille (CH Limoges), E. Frealle, B. Sendid (CHU Lille), D. Dupont, J. Menotti, F. Persat, M. Wallon (CHU, Lyon), C. Cassagne, S. Ranque (CHU, Marseille), T. Benoit-Cattin, L. Collet (CH Mayotte), A. Fiacre (CH Meaux), N. Bourgeois, L. Lachaud (CHU, Montpellier), M. Machouart (CHU, Nancy), P. Lepape, F. Morio (CHU, Nantes), O. Moquet (CH, Nevers), S. Lefrançois (hôpital Américain, Neuilly), M. Sasso (CHU, Nimes), F. Reibel (GH, Nord-Essone), M. Gari-Toussaint, L. Hasseine (CHU Nice), L. Bret, D. Poisson (CHR Orléans), S. Brun (hôpital Avicenne, Paris), C. Bonnal, S. Houze (hôpital Bichat, Paris), A. Paugam (hôpital Cochin, Paris), E. Dannaoui (HEGP, Paris), N. Ait-Ammar, F. Botterel, R. Chouk (CHU Henri Mondor, Paris), M. E. Bougnoux, E. Sitterle (hôpital Necker, Paris), A. Fekkar, R. Piarroux (hôpital Pitié Salpêtrière, Paris), J. Guitard, C. Hennequin (hôpital St Antoine, Paris), M. Gits-Muselli, S. Hamane (hôpital Saint Louis, Paris), S. Bonacorsi, P. Mariani (hôpital Robert Debré, Paris), D. Moissenet (hôpital Trousseau, Paris), A. Minoza, E. Perraud, M. H. Rodier (CHU Poitiers), G. Colonna (CH, Porto Vecchio), D. Toubas (CHU Reims), J. P. Gangneux, F. Robert-Gangneux (CHU Rennes), O. Belmonte, G. Hoarau, M. C. Jaffar Bandjee, J. Jaubert, S. Picot, N. Traversier (CHU Réunion), L. Favennec, G. Gargala (CHU, Rouen), C. Tournus (CH, St Denis), H. Raberin (CHU, St Etienne), V. Letscher Bru (CHU, Strasbourg), S. Cassaing (CHU, Toulouse), P. Patoz (CH Tourcoing), E. Bailly, G. Desoubeaux (CHU Tours), F. Moreau (CH Troyes), P. Munier (CH Valence), E. Mazars (CH Valenciennes), O. Eloy (CH Versailles), E. Chachaty (Institut Gustave Roussy, Villejuif), and members of the NRCMA (Institut Pasteur, Paris): A. Boullié, C. Gautier, V. Geolier, C. Blanc, D. Hoinard and D. Raoux-Barbot for technical help, and K. Boukris-Sitbon, F. Lanternier, A. Alanio, D. Garcia-Hermoso for their expertise and contribution to the surveillance programs., Centre National de la Recherche Scientifique (CNRS)-Institut Pasteur [Paris], and Université de Paris (UP)
Subjects: Serotype, Azoles, Antifungal Agents, Microbial Sensitivity Tests, Candida parapsilosis, Rhodotorula mucilaginosa, Pichia, Microbiology, Candida tropicalis, 03 medical and health sciences, 0302 clinical medicine, Drug Resistance, Fungal, Pharmacology (medical), 030212 general & internal medicine, Candida albicans, [SDV.MP.MYC]Life Sciences [q-bio]/Microbiology and Parasitology/Mycology, Pharmacology, chemistry.chemical_classification, 0303 health sciences, biology, Candida glabrata, 030306 microbiology, Broth microdilution, Rhodotorula, biology.organism_classification, 3. Good health, Infectious Diseases, chemistry, Susceptibility, Saccharomycetales, Azole, France
Abstract: Invasive yeast infections represent a major global public health issue, and only few antifungal agents are available. Azoles are one of the classes of antifungals used for treatment of invasive candidiasis. The determination of antifungal susceptibility profiles using standardized methods is important to identify resistant isolates and to uncover the potential emergence of intrinsically resistant species. Here, we report data on 9,319 clinical isolates belonging to 40 pathogenic yeast species recovered in France over 17 years. The antifungal susceptibility profiles were all determined at the National Reference Center for Invasive Mycoses and Antifungals based on the EUCAST broth microdilution method. The centralized collection and analysis allowed us to describe the trends of azole susceptibility of isolates belonging to common species, confirming the high susceptibility for Candida albicans (n = 3,295), Candida tropicalis (n = 641), and Candida parapsilosis (n = 820) and decreased susceptibility for Candida glabrata (n = 1,274) and Pichia kudriavzevii (n = 343). These profiles also provide interesting data concerning azole susceptibility of Cryptococcus neoformans species complex, showing comparable MIC distributions for the three species but lower MIC(50)s and MIC(90)s for serotype D (n = 208) compared to serotype A (n = 949) and AD hybrids (n = 177). Finally, these data provide useful information for rare and/or emerging species, such as Clavispora lusitaniae (n = 221), Saprochaete clavata (n = 184), Meyerozyma guilliermondii complex (n = 150), Candida haemulonii complex (n = 87), Rhodotorula mucilaginosa (n = 55), and Wickerhamomyces anomalus (n = 36).
Published: 2021
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3. Randomized Clinical Trial of Sevelamer Carbonate on Serum Klotho and Fibroblast Growth Factor 23 in CKD

Author: Bertrand Dussol, Sophie Liabeuf, Eric Thervet, Pablo Ureña, Najeh El Esper, Christian Combe, Jean-Philippe Ryckelynck, Gabriel Choukroun, Dominique Prié, Luc Frimat, Romuald Mentaverri, Philippe Vanhille, Denis Fouque, Centre de recherche en épidémiologie et santé des populations (CESP), Université de Versailles Saint-Quentin-en-Yvelines (UVSQ)-Université Paris-Sud - Paris 11 (UP11)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Hôpital Paul Brousse-Institut National de la Santé et de la Recherche Médicale (INSERM), CHU Amiens-Picardie, Mécanismes physiopathologiques et conséquences des calcifications vasculaires - UR UPJV 7517 (MP3CV), Université de Picardie Jules Verne (UPJV)-CHU Amiens-Picardie, AURA [Paris], Bioingénierie tissulaire (BIOTIS), Université de Bordeaux (UB)-Institut National de la Santé et de la Recherche Médicale (INSERM), CHU Bordeaux [Bordeaux], Centre de néphrologie et transplantation rénale [Hôpital de la Conception - APHM], Assistance Publique - Hôpitaux de Marseille (APHM)-Hôpital de la Conception [CHU - APHM] (LA CONCEPTION), Vascular research center of Marseille (VRCM), Aix Marseille Université (AMU)-Institut National de la Santé et de la Recherche Médicale (INSERM), Cardiovasculaire, métabolisme, diabétologie et nutrition (CarMeN), Institut National de la Recherche Agronomique (INRA)-Université Claude Bernard Lyon 1 (UCBL), Université de Lyon-Université de Lyon-Institut National des Sciences Appliquées de Lyon (INSA Lyon), Université de Lyon-Institut National des Sciences Appliquées (INSA)-Institut National des Sciences Appliquées (INSA)-Hospices Civils de Lyon (HCL)-Institut National de la Santé et de la Recherche Médicale (INSERM), Centre Hospitalier Lyon Sud [CHU - HCL] (CHLS), Hospices Civils de Lyon (HCL), Service de médecine interne et néphrologie, CH Valenciennes, Service de Néphrologie [CHRU Nancy], Centre Hospitalier Régional Universitaire de Nancy (CHRU Nancy), Service de Néphrologie et Hémodialyse [CHU HEGP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Hôpital Européen Georges Pompidou [APHP] (HEGP), Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Hôpitaux Universitaires Paris Ouest - Hôpitaux Universitaires Île de France Ouest (HUPO)-Hôpitaux Universitaires Paris Ouest - Hôpitaux Universitaires Île de France Ouest (HUPO), Paris-Centre de Recherche Cardiovasculaire (PARCC - UMR-S U970), Hôpital Européen Georges Pompidou [APHP] (HEGP), Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Hôpitaux Universitaires Paris Ouest - Hôpitaux Universitaires Île de France Ouest (HUPO)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Hôpitaux Universitaires Paris Ouest - Hôpitaux Universitaires Île de France Ouest (HUPO)-Université Paris Descartes - Paris 5 (UPD5)-Institut National de la Santé et de la Recherche Médicale (INSERM), Département Hospitalo-Universitaire Paris Ouest (Paris Descartes), Université Paris Descartes - Paris 5 (UPD5), Centre de recherche Croissance et signalisation (UMR_S 845), Université Paris Descartes - Paris 5 (UPD5)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Centre National de la Recherche Scientifique (CNRS), and DESSAIVRE, Louise
Subjects: Fibroblast growth factor 23, medicine.medical_specialty, Epidemiology, medicine.drug_class, [SDV]Life Sciences [q-bio], 030232 urology & nephrology, Urology, Renal function, 030204 cardiovascular system & hematology, Critical Care and Intensive Care Medicine, Fibroblast growth factor, Sevelamer, Placebo, 03 medical and health sciences, chemistry.chemical_compound, 0302 clinical medicine, Interquartile range, Medicine, Transplantation, Creatinine, business.industry, Phosphate binder, [SDV] Life Sciences [q-bio], chemistry, Nephrology, business, medicine.drug
Abstract: International audience; Background and objectives: Epidemiologic studies suggest that higher serum phosphaturic hormone fibroblast growth factor 23 levels are associated with increase morbidity and mortality. The aim of the FGF23 Reduction Efficacy of a New Phosphate Binder in CKD Trial was to evaluate the effect of sevelamer carbonate on serum C-terminal fibroblast growth factor 23 levels in normophosphatemic patients with CKD stage 3b/4. Design, setting, participants, & measurements: Patients with CKD, eGFR between 45 and 15 ml/min per 1.73 m(2), fasting serum phosphate concentration >3.1 mg/dl, and serum C-terminal fibroblast growth factor 23 >80 relative units/ml were included in our double-blind, placebo-controlled, randomized multicenter study. All patients received 100,000 IU cholecalciferol at time of randomization. Participants received either placebo or sevelamer carbonate 4.8 g daily during a 12-week period. Biologic parameters, including serum C-terminal fibroblast growth factor 23, intact fibroblast growth factor 23, and a-klotho, were evaluated at baseline and 12 weeks after inclusion. Results: Of 96 screened patients, 78 (mean +/- SD age: 63 +/- 13 years old; 70% men; mean eGFR: 27 +/- 9 ml/min per 1.73 m(2)) met the inclusion criteria. At baseline, mean eGFR was 27 +/- 9 ml/min per 1.73 m(2), mean serum phosphate level was 3.8 +/- 0.5 mg/dl, and median (interquartile range) serum C-terminal fibroblast growth factor 23 level was 157 (120-241) relative units/ml. After 12 weeks of treatment, urinary phosphate-to-creatinine ratio fell significantly in the sevelamer group. The sevelamer and placebo groups did not differ significantly in terms of median change in serum C-terminal fibroblast growth factor 23 levels: the median (interquartile range) change was 38 (-13-114) relative units/ml in the placebo group and 37 (-1-101) relative units/ml in the sevelamer group (P=0.77). There was no significant difference in serum intact fibroblast growth factor 23, alpha-klotho, or phosphate levels changes between the two groups. Serum total and LDL cholesterol levels fell significantly in the sevelamer group. Conclusions: In our double-blind, placebo-controlled, randomized study performed in normophosphatemic patients with CKD, a 12-week course of sevelamer carbonate significantly reduced phosphaturia without changing serum phosphorus but did not significantly modify serum C-terminal fibroblast growth factor 23 and intact fibroblast growth factor 23 or alpha-klotho levels.
Published: 2017
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4. Intravenous immunoglobulins in systemic sclerosis: Data from a French nationwide cohort of 46 patients and review of the literature

Author: Camille Francès, Christophe Deligny, Bernard Imbert, Emmanuel Chatelus, L. Boukari, Noémie Le Gouellec, Claire Cazalets, Patricia Senet, Arsène Mekinian, Thierry Martin, Patrick Jego, Christian Agard, Thomas Quemeneur, David Launay, Pierre-Yves Hatron, Alexis Mathian, Sébastien Rivière, Alain Le Quellec, Alban Deroux, Grégory Pugnet, Alain Lescoat, Thomas Sené, Silvia Speca, Olivier Fain, Sébastien Sanges, Eric Hachulla, Sylvain Dubucquoi, Lille Inflammation Research International Center - U 995 (LIRIC), Institut Pasteur de Lille, Réseau International des Instituts Pasteur (RIIP)-Réseau International des Instituts Pasteur (RIIP)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Université de Lille-Centre Hospitalier Régional Universitaire [Lille] (CHRU Lille), Service de médecine interne [Lille], Centre Hospitalier Régional Universitaire [Lille] (CHRU Lille), CHU Saint-Antoine [AP-HP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU), Université Pierre et Marie Curie - Paris 6 - UFR de Médecine Pierre et Marie Curie (UPMC), Université Pierre et Marie Curie - Paris 6 (UPMC), CHU Strasbourg, Immunopathologie et chimie thérapeutique (ICT), Institut de biologie moléculaire et cellulaire (IBMC), Université de Strasbourg (UNISTRA)-Centre National de la Recherche Scientifique (CNRS)-Université de Strasbourg (UNISTRA)-Centre National de la Recherche Scientifique (CNRS)-Centre National de la Recherche Scientifique (CNRS), Centre Hospitalier Régional Universitaire [Montpellier] (CHRU Montpellier), Service de rhumatologie [Strasbourg], CHU Strasbourg-Hôpital de Hautepierre [Strasbourg], Immuno-Rhumatologie Moléculaire, Université de Strasbourg (UNISTRA)-Institut National de la Santé et de la Recherche Médicale (INSERM), CHU Pontchaillou [Rennes], CH Valenciennes, CHU Tenon [AP-HP], CHU Grenoble, Hôpital Jean Verdier [AP-HP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP), Groupe Hospitalier Diaconesses Croix Saint-Simon, Hôpital Pierre Zobda-Quitman [CHU de la Martinique], CHU de la Martinique [Fort de France], CHU Pitié-Salpêtrière [AP-HP], Service de médecine interne [Nantes], Université de Nantes (UN)-Hôtel-Dieu-Centre hospitalier universitaire de Nantes (CHU Nantes), Centre Hospitalier Universitaire de Toulouse (CHU Toulouse), Epidémiologie et analyses en santé publique : risques, maladies chroniques et handicaps (LEASP), Université Toulouse III - Paul Sabatier (UT3), Université de Toulouse (UT)-Université de Toulouse (UT)-Institut National de la Santé et de la Recherche Médicale (INSERM), Centre Hospitalier Régional Universitaire [Lille] (CHRU Lille)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Université de Lille-Institut Pasteur de Lille, Réseau International des Instituts Pasteur (RIIP)-Réseau International des Instituts Pasteur (RIIP), Service de Médecine Interne [CHU Saint-Antoine], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU), Centre National de la Recherche Scientifique (CNRS)-Institut de biologie moléculaire et cellulaire (IBMC), Université de Strasbourg (UNISTRA)-Centre National de la Recherche Scientifique (CNRS)-Université de Strasbourg (UNISTRA)-Centre National de la Recherche Scientifique (CNRS), Service de dermatologie et allergologie [CHU Tenon], Sorbonne Université (SU)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP), Centre National de Référence du Lupus Systémique, Syndrome des Anticorps Anti-phospholipides et Maladies Auto-immunes Systémiques Rares [CHU Pitié Salpêtrière], Service de Médecine Interne 2, maladies auto-immunes et systémiques [CHU Pitié-Salpêtrière], Institut E3M [CHU Pitié-Salpêtrière], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU)-CHU Pitié-Salpêtrière [AP-HP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU)-Institut E3M [CHU Pitié-Salpêtrière], CHU Toulouse [Toulouse], Université Fédérale Toulouse Midi-Pyrénées-Université Fédérale Toulouse Midi-Pyrénées-Institut National de la Santé et de la Recherche Médicale (INSERM), Lille Inflammation Research International Center (LIRIC), Assistance publique - Hôpitaux de Paris (AP-HP) (APHP)-CHU Saint-Antoine [APHP], Service de Dermatologie [CHU Tenon], Assistance publique - Hôpitaux de Paris (AP-HP) (APHP)-CHU Tenon [APHP], Hôpital Jean Verdier [Bondy], Assistance publique - Hôpitaux de Paris (AP-HP) (APHP), Centre National de Référence du Lupus Systémique, Syndrome des Anticorps Anti-phospholipides et Maladies Auto-immunes Systémiques Rares[CHU Pitié Salpêtrière], Assistance publique - Hôpitaux de Paris (AP-HP) (APHP)-CHU Pitié-Salpêtrière [APHP], Sorbonne Université (SU)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-CHU Pitié-Salpêtrière [AP-HP], and Sorbonne Université (SU)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Institut E3M [CHU Pitié-Salpêtrière]
Subjects: 0301 basic medicine, medicine.medical_specialty, medicine.drug_class, Deep vein, Immunology, Population, Intravenous immunoglobulins, Systemic inflammation, Cohort Studies, 03 medical and health sciences, 0302 clinical medicine, Internal medicine, medicine, Humans, Immunology and Allergy, education, Adverse effect, Retrospective Studies, 030203 arthritis & rheumatology, education.field_of_study, Scleroderma, Systemic, business.industry, Immunoglobulins, Intravenous, Middle Aged, medicine.disease, Fibrosis, Thrombosis, 3. Good health, Surgery, Discontinuation, Treatment Outcome, 030104 developmental biology, medicine.anatomical_structure, Inflammatory myopathies, Joint pain, Systemic sclerosis, [SDV.IMM]Life Sciences [q-bio]/Immunology, Corticosteroid, Female, France, medicine.symptom, business
Abstract: International audience; BACKGROUND:As intravenous immunoglobulins (IVIG) exhibit immunomodulatory and antifibrotic properties, they may be a relevant treatment for systemic sclerosis (SSc). The objectives of this work were thus to report on the efficacy and safety of IVIG in a population of SSc patients and to review the available literature.METHODS:46 patients from 19 French centers were retrospectively recruited. They were included if they had a diagnosis of SSc and received at least 1 IVIG infusion at a dosage >1g/kg/cycle. Relevant data collected at IVIG discontinuation were compared to those collected at IVIG initiation. A comprehensive literature review was performed.RESULTS:We observed a significant improvement of muscle pain (74% vs. 20%, p
Published: 2017
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5. Metformin-related lactic acidosis with acute kidney injury: results of a French observational multicenter study

Author: Anthony Corchia, Pascal Reboul, Alexandre Lautrette, Julie Moussi Frances, Julien Journet, Zoubir Djerada, Alain Wynckel, Laurence Vrigneaud, Elisabeth Lewandowski, Philippe Rieu, Nihel Skandrani, Lara Zafrani, Centre Hospitalier Universitaire de Reims (CHU Reims), Centre Hospitalier Chalon-sur-Saône William Morey, CHU Marseille, CH Belfort-Montbéliard, Unité de soins intensifs [Clermont Ferrand], CHU Clermont-Ferrand-CHU Gabriel Montpied [Clermont-Ferrand], CHU Clermont-Ferrand, Service de Réanimation Médicale, Hôpital Saint Louis, Paris, France, CHU Amiens-Picardie, Centre Hospitalier Universitaire de Nîmes (CHU Nîmes), Service de Néphrologie [Valenciennes], Centre Hospitalier de Valenciennes, Service de Néphrologie et Médecine interne [CH Valenciennes], and Centre hospitalier [Valenciennes, Nord]
Subjects: Male, medicine.medical_specialty, endocrine system diseases, medicine.medical_treatment, Toxicology, Gastroenterology, [SDV.MP.PRO]Life Sciences [q-bio]/Microbiology and Parasitology/Protistology, 03 medical and health sciences, 0302 clinical medicine, Internal medicine, Diabetes mellitus, Humans, Hypoglycemic Agents, Medicine, 030212 general & internal medicine, Renal replacement therapy, ComputingMilieux_MISCELLANEOUS, Aged, Retrospective Studies, Aged, 80 and over, business.industry, digestive, oral, and skin physiology, Acute kidney injury, food and beverages, nutritional and metabolic diseases, 030208 emergency & critical care medicine, General Medicine, Acute Kidney Injury, Hydrogen-Ion Concentration, Middle Aged, medicine.disease, [SDV.MP.BAC]Life Sciences [q-bio]/Microbiology and Parasitology/Bacteriology, Metformin, 3. Good health, Renal Replacement Therapy, Multicenter study, Lactic acidosis, Acidosis, Lactic, Female, Observational study, business, [SDV.MHEP]Life Sciences [q-bio]/Human health and pathology, medicine.drug
Abstract: Background: Metformin-associated lactic acidosis (MALA) and metformin-induced lactic acidosis (MILA) remain controversial entities. Metformin toxic effect depends on accumulation to lead to lactic ...
Published: 2019
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6. Early predictors of one-year mortality in patients over 65 presenting with ANCA-associated renal vasculitis: a retrospective, multicentre study

Author: Thierry Lobbedez, Dominique Guerrot, Boris Bienvenu, Alexis Francois, Eric Hachulla, Dimitri Titeca-Beauport, Laurence Vrigneaud, Maité Daroux, David Launay, Momar Diouf, C. Lebas, Gabriel Choukroun, CHU Amiens-Picardie, Service de Néphrologie-Dialyse-Transplantation rénale [CHU Caen], Université de Caen Normandie (UNICAEN), Normandie Université (NU)-Normandie Université (NU)-CHU Caen, Normandie Université (NU)-Tumorothèque de Caen Basse-Normandie (TCBN)-Tumorothèque de Caen Basse-Normandie (TCBN), Registre de Dialyse Péritonéale de Langue Française et Hémodialyse à Domicile [Pontoise] (RDPLF-HDD), Service de Néphrologie [Rouen], CHU Rouen, Normandie Université (NU)-Normandie Université (NU)-Université de Rouen Normandie (UNIROUEN), Normandie Université (NU), Endothélium, valvulopathies et insuffisance cardiaque (EnVI), Université de Rouen Normandie (UNIROUEN), Normandie Université (NU)-Normandie Université (NU)-Institut National de la Santé et de la Recherche Médicale (INSERM), Lille Inflammation Research International Center - U 995 (LIRIC), Institut Pasteur de Lille, Réseau International des Instituts Pasteur (RIIP)-Réseau International des Instituts Pasteur (RIIP)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Université de Lille-Centre Hospitalier Régional Universitaire [Lille] (CHRU Lille), Service de médecine interne [Lille], Centre Hospitalier Régional Universitaire [Lille] (CHRU Lille), Centre hospitalier [Valenciennes, Nord], Centre Hospitalier Boulogne-sur-mer, Service de Néphrologie et Transplantation rénale [CHRU-lille], Mobilités : Vieillissement, Pathologie, Santé (COMETE), Service de médecine interne [CHU Caen], Direction de la recherche clinique et de l'innovation [CHU Amiens], The authors thank Joelle Gracia, Audrey Sultan and Magaly Parsy for the help with collecting the data., Bodescot, Myriam, Service de Néphrologie-Dialyse-Transplantation [CHU Amiens-Picardie], Registre de Dialyse Péritonéale de Langue Française (RDPLF), Société savante (Pontoise), Département de Médecine interne et d'Immunologie clinique [CHRU Lille], Centre national de référence des maladies auto-immunes systémiques rares (sclérodermie systémique) [Lille], Service de Néphrologie et Médecine interne [CH Valenciennes], Service de Néphrologie [CH Boulogne-sur-Mer], Service de Néphrologie [CHRU Lille], Institut National de la Santé et de la Recherche Médicale (INSERM)-Université de Caen Normandie (UNICAEN), Normandie Université (NU)-Normandie Université (NU), Service de Médecine interne [CHRU Caen], CHU Caen, and Normandie Université (NU)-Tumorothèque de Caen Basse-Normandie (TCBN)-Normandie Université (NU)-Tumorothèque de Caen Basse-Normandie (TCBN)
Subjects: Male, Nephrology, 030232 urology & nephrology, lcsh:RC870-923, [SDV.MHEP.UN]Life Sciences [q-bio]/Human health and pathology/Urology and Nephrology, Elderly, Glomerulonephritis, MESH: Aged, 80 and over, 0302 clinical medicine, Interquartile range, Aged, 80 and over, MESH: Aged, education.field_of_study, Univariate analysis, MESH: France/epidemiology, ANCA, Mortality rate, Age Factors, MESH: Follow-Up Studies, MESH: Predictive Value of Tests, 3. Good health, Female, France, Infection, Vasculitis, Research Article, medicine.medical_specialty, Population, Renal function, Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis, 03 medical and health sciences, Predictive Value of Tests, Internal medicine, medicine, Humans, Mortality, education, Aged, Retrospective Studies, MESH: Age Factors, 030203 arthritis & rheumatology, MESH: Mortality/trends, MESH: Humans, MESH: Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis/blood, MESH: Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis/mortality, business.industry, MESH: Retrospective Studies, Retrospective cohort study, lcsh:Diseases of the genitourinary system. Urology, medicine.disease, [SDV.MHEP.UN] Life Sciences [q-bio]/Human health and pathology/Urology and Nephrology, MESH: Male, MESH: Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis/diagnosis, business, MESH: Female, Follow-Up Studies
Abstract: Background The risk of early death is particularly high in patients over the age of 65 presenting with antineutrophil cytoplasmic antibody (ANCA)-associated renal vasculitis. We hypothesized that by combining disease severity markers, a comorbidity index and serious adverse event reports, we would be able to identify early predictors of one-year mortality in this population. Methods We performed a multicentre, retrospective study in the nephrology and internal medicine departments of six tertiary hospitals in northern France. A total of 149 patients (median [interquartile range (IQR)] age: 72.7 [68.5–76.8] years) presenting with ANCA-associated vasculitis and renal involvement were included between January 2002 and June 2015. The primary endpoint was the one-year mortality rate. Results Renal function was severely impaired at presentation (median [IQR] peak serum creatinine (SCr): 337 [211–522] μmol/l), and 45 patients required dialysis. The Five-Factor Score (FFS, scored as + 1 point for each poor prognostic factor (age > 65 years, cardiac symptoms, gastrointestinal involvement, SCr ≥150 μmol/L, and the absence of ear, nose, and throat involvement)) was ≥3 in 120 cases. The one-year mortality rate was 19.5%. Most of the deaths occurred before month 6, and most of these were related to severe infections. In a univariate analysis, age, a high comorbidity index, a performance status of 3 or 4, a lack of co-trimoxazole prophylaxis, early severe infection, and disease activity parameters (such as the albumin level, haemoglobin level, peak SCr level, dialysis status, and high FFS) were significantly associated with one-year mortality. In a multivariable analysis, the best predictors were a high FFS (relative risk (RR) [95% confidence interval (CI)] = 2.57 [1.30–5.09]; p = 0.006) and the occurrence of a severe infection during the first month (RR [95%CI] = 2.74 [1.27–5.92]; p = 0.01). Conclusions When considering various disease severity markers in over-65 patients with ANCA-associated renal vasculitis, we found that an early, severe infection (which occurred in about a quarter of the patients) is a strong predictor of one-year mortality. A reduction in immunosuppression, the early detection of infections, and co-trimoxazole prophylaxis might help to reduce mortality in this population. Electronic supplementary material The online version of this article (10.1186/s12882-018-1102-3) contains supplementary material, which is available to authorized users.
Published: 2018
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7. Abnormal T-cell phenotype in episodic angioedema with hypereosinophilia (Gleich's syndrome): frequency, clinical implication and prognosis

Author: Isabelle Lamaury, Christian Agard, Noémie Abisror, Olivier Fain, Jean-François Dufour, Chafika Morati, Matthieu Groh, Mathilde Hunault-Berger, Guillaume Lefèvre, Arsène Mekinian, Felix Ackermann, Agnès Dechartres, Jean Emmanuel Kahn, Sébastien Trouillier, Pierre-Yves Jeandel, Nicolas Noel, Thomas Quemeneur, Alice Bérezné, François Lhote, Julien Haroche, Loïk Geffray, Bernardo, Elizabeth, Service de rhumatologie [CHU Saint-Antoine], CHU Saint-Antoine [AP-HP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU), Département de Biostatistique, Santé Publique et Information Médicale [CHU Pitié-Salpêtrière] (BIOSPIM ), CHU Pitié-Salpêtrière [AP-HP], Hôpital Foch [Suresnes], Hôpital Cochin [AP-HP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP), UNIROUEN - UFR Santé (UNIROUEN UFR Santé), Université de Rouen Normandie (UNIROUEN), Normandie Université (NU)-Normandie Université (NU), Centre National de Référence du Lupus Systémique, Syndrome des Anticorps Anti-phospholipides et Maladies Auto-immunes Systémiques Rares [CHU Pitié Salpêtrière], Service de Médecine Interne 2, maladies auto-immunes et systémiques [CHU Pitié-Salpêtrière], Institut E3M [CHU Pitié-Salpêtrière], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU)-CHU Pitié-Salpêtrière [AP-HP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU)-Institut E3M [CHU Pitié-Salpêtrière], Innate Immunity and Immunotherapy (CRCINA-ÉQUIPE 7), Centre de Recherche en Cancérologie et Immunologie Nantes-Angers (CRCINA), Université d'Angers (UA)-Université de Nantes (UN)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Centre National de la Recherche Scientifique (CNRS)-Centre hospitalier universitaire de Nantes (CHU Nantes)-Université d'Angers (UA)-Université de Nantes (UN)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Centre National de la Recherche Scientifique (CNRS)-Centre hospitalier universitaire de Nantes (CHU Nantes), Service de médecine interne [Nantes], Université de Nantes (UN)-Hôtel-Dieu-Centre hospitalier universitaire de Nantes (CHU Nantes), Centre Hospitalier Robert Bisson (CH Robert Bisson), Service de médecine interne [Nice], Université Nice Sophia Antipolis (1965 - 2019) (UNS), COMUE Université Côte d'Azur (2015-2019) (COMUE UCA)-COMUE Université Côte d'Azur (2015-2019) (COMUE UCA)-Centre Hospitalier Universitaire de Nice (CHU Nice)-Hôpital l'Archet, CHU Clermont-Ferrand, Département de Médecine Interne (VALENCIENNES - Med Int), CH Valenciennes, Centre Hospitalier de Bourg-en-Bresse, CHU Pointe-à-Pitre/Abymes [Guadeloupe], Centre d'Investigation Clinique Antilles-Guyane (CIC - Antilles Guyane), Université des Antilles et de la Guyane (UAG)-Institut National de la Santé et de la Recherche Médicale (INSERM)-CHU Pointe-à-Pitre/Abymes [Guadeloupe] -CHU de Fort de France-Centre Hospitalier Andrée Rosemon [Cayenne, Guyane Française], Centre Hospitalier de Saint-Denis [Ile-de-France], Lille Inflammation Research International Center - U 995 (LIRIC), Institut Pasteur de Lille, Réseau International des Instituts Pasteur (RIIP)-Réseau International des Instituts Pasteur (RIIP)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Université de Lille-Centre Hospitalier Régional Universitaire [Lille] (CHRU Lille), Service de médecine interne [CHU Saint-Antoine], Sorbonne Université (SU)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP), Sorbonne Université (SU)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-CHU Pitié-Salpêtrière [AP-HP], Sorbonne Université (SU)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Institut E3M [CHU Pitié-Salpêtrière], Institut National de la Santé et de la Recherche Médicale (INSERM)-Université de Nantes - UFR de Médecine et des Techniques Médicales (UFR MEDECINE), Université de Nantes (UN)-Université de Nantes (UN)-Centre hospitalier universitaire de Nantes (CHU Nantes)-Centre National de la Recherche Scientifique (CNRS)-Université d'Angers (UA)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Université de Nantes - UFR de Médecine et des Techniques Médicales (UFR MEDECINE), Université de Nantes (UN)-Université de Nantes (UN)-Centre hospitalier universitaire de Nantes (CHU Nantes)-Centre National de la Recherche Scientifique (CNRS)-Université d'Angers (UA), Université Nice Sophia Antipolis (... - 2019) (UNS), CHU de Fort de France-Centre Hospitalier Andrée Rosemon [Cayenne, Guyane Française]-CHU Pointe-à-Pitre/Abymes [Guadeloupe] -Institut National de la Santé et de la Recherche Médicale (INSERM)-Université des Antilles et de la Guyane (UAG), Centre Hospitalier Régional Universitaire [Lille] (CHRU Lille)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Université de Lille-Institut Pasteur de Lille, Réseau International des Instituts Pasteur (RIIP)-Réseau International des Instituts Pasteur (RIIP), Service de Médecine Interne [CHU Saint-Antoine], and Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU)
Subjects: medicine.medical_specialty, Population, [SDV.CAN]Life Sciences [q-bio]/Cancer, Hypereosinophilia, Dermatology, Hypereosinophilic syndrome, Gastroenterology, 030207 dermatology & venereal diseases, 03 medical and health sciences, 0302 clinical medicine, [SDV.CAN] Life Sciences [q-bio]/Cancer, Recurrence, Prednisone, Internal medicine, Peripheral), medicine, Eosinophilia, Angioedema, education, education.field_of_study, business.industry, Gene rearrangement, medicine.disease, 3. Good health, 030220 oncology & carcinogenesis, Lymphoma (T-cell, Gleich's syndrome, Treatment Outcome, medicine.symptom, business, medicine.drug
Abstract: International audience; Background: Episodic Angioedema with eosinophilia (EAE, Gleich's syndrome) is a rare disorder consisting of recurrent episodes of angioedema, hypereosinophilia and frequent elevated serum Immunoglobin M.Methods: We conducted a retrospective multicenter nationwide study regarding the clinical spectrum and therapeutic management of patients with EAE in France.Results: Thirty patients were included with a median age at diagnosis of 41 years [5-84]. The median duration of each crisis was 5.5 days [1-90] with swelling affecting mainly the face and the upper limbs. Total serum IgM levels were increased in 20 patients (67%). Abnormal T-cell immunophenotypes were detected in 12 patients (40%) among which 5 (17%) showed evidence of clonal TCR γ gene rearrangement. Median follow-up duration was 53 months [31-99]. The presence of an abnormal T-cell population was the sole factor associated with a shorter time to flare (hazard ratio 4.15 [CI 95% 1.18-14.66; p=0.02). At last follow-up, 3 patients (10%) were able to withdraw all treatments and 11 (37%) were in clinical and biological remission with less than 10 mg of daily prednisone.Conclusion: EAE is a heterogeneous condition that encompasses several disease forms. Although patients usually respond well to glucocorticoids, those with evidence of abnormal T-cell phenotype have a shorter time to flare.
Published: 2018
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8. Practical implementation, education and interpretation guidelines for continuous glucose monitoring: A French position statement

Author: Borot , S., Benhamou , P., Atlan , C., Bismuth , E., Bonnemaison , E., Catargi , B., Charpentier , G., Farret , A., Filhol , N., Franc , S., Gouet , D., Guerci , B., Guilhem , I., Guillot , C., Jeandidier , N., Joubert , M., Melki , V., Merlen , E., Penfornis , A., Picard , S., Renard , E., Reznik , Y., Riveline , JP., Rudoni , S., Schaepelynck , P., Sola-Gazagnes , A., Tubiana-Rufi , N., Verier-Mine , O., Hanaire , H., du diabète (SFD) , société francophone, d'endocrinologie (SFE) , Société française, Group (EVADIAC) , Evaluation dans le diabète des implants actifs, Service de diabétologie - endocrinologie, Centre Hospitalier Régional Universitaire [Besançon] ( CHRU Besançon ) -Hôpital Jean Minjoz, Université de Franche-Comté ( UFC ), CHU Grenoble, Université Joseph Fourier - Grenoble 1 ( UJF ) -CHU Grenoble, Service d'endocrinologie, Assistance publique - Hôpitaux de Paris (AP-HP)-Hôpital Robert Debré-Université Paris Diderot - Paris 7 ( UPD7 ), CHU Trousseau [Tours], CHRU Tours, CHU Bordeaux [Bordeaux], Centre d'études et de recherches pour l'intensification du traitement du diabète ( CERITD ), Centre Hospitalier Régional Universitaire [Montpellier] ( CHRU Montpellier ), CHU Marseille, Centre Hospitalier Sud Francilien, CH Evry-Corbeil, Centre Hospitalier Régional Universitaire de Nancy ( CHRU Nancy ), Service d'endocrinologie diabétologie et nutrition [Rennes], Université de Rennes 1 ( UR1 ), Université de Rennes ( UNIV-RENNES ) -Université de Rennes ( UNIV-RENNES ) -CHU Pontchaillou [Rennes]-Hôpital Anne-de-Bretagne, CHU Strasbourg, Service d'endocrinologie [Caen], Université de Caen Normandie ( UNICAEN ), Normandie Université ( NU ) -Normandie Université ( NU ) -CHU Caen, Service de Diabétologie - Maladies Métaboliques - Nutrition, CHU Toulouse [Toulouse]-Hôpital de Rangueil, Centre Hospitalier Régional Universitaire [Lille] ( CHRU Lille ), Point médical (Dijon), Hôpital Lariboisière, Assistance publique - Hôpitaux de Paris (AP-HP)-Hôpital Lariboisière-Université Paris Diderot - Paris 7 ( UPD7 ), Service d'Endocrinologie, Diabétologie et Maladies Métaboliques (CHU de Dijon), Centre Hospitalier Universitaire de Dijon - Hôpital François Mitterrand ( CHU Dijon ), Service de diabétologie [CHU Cochin], Assistance publique - Hôpitaux de Paris (AP-HP)-CHU Cochin [AP-HP], CH Valenciennes, Service de Diabétologie - Endocrinologie [CHRU Besançon], Centre Hospitalier Régional Universitaire de Besançon (CHRU Besançon), Université de Franche-Comté (UFC), Université Bourgogne Franche-Comté [COMUE] (UBFC), Université Joseph Fourier - Grenoble 1 (UJF)-CHU Grenoble, Laboratory of Fundamental and Applied Bioenergetics = Laboratoire de bioénergétique fondamentale et appliquée (LBFA), Institut National de la Santé et de la Recherche Médicale (INSERM)-Université Grenoble Alpes [2016-2019] (UGA [2016-2019]), Université Paris Diderot - Paris 7 (UPD7)-Hôpital Robert Debré-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP), Centre Hospitalier Régional Universitaire de Tours (CHRU Tours), Centre d'études et de recherches pour l'intensification du traitement du diabète (CERITD), Centre Hospitalier Régional Universitaire [Montpellier] (CHRU Montpellier), Centre Hospitalier Régional Universitaire de Nancy (CHRU Nancy), Université de Rennes 1 (UR1), Université de Rennes (UNIV-RENNES)-Université de Rennes (UNIV-RENNES)-CHU Pontchaillou [Rennes]-Hôpital Anne-de-Bretagne, Service d'endocrinologie [CHU Caen], Université de Caen Normandie (UNICAEN), Normandie Université (NU)-Normandie Université (NU)-CHU Caen, Normandie Université (NU)-Tumorothèque de Caen Basse-Normandie (TCBN)-Tumorothèque de Caen Basse-Normandie (TCBN), CHU Toulouse [Toulouse], Centre Hospitalier Régional Universitaire [Lille] (CHRU Lille), Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Hôpital Lariboisière-Fernand-Widal [APHP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Université Paris Diderot - Paris 7 (UPD7), Centre Hospitalier Universitaire de Dijon - Hôpital François Mitterrand (CHU Dijon), Université de Montpellier (UM), Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Hôpital Cochin [AP-HP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP), Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Hôpital Robert Debré-Université Paris Diderot - Paris 7 (UPD7), Centre Hospitalier Régional Universitaire [Besançon] (CHRU Besançon)-Hôpital Jean Minjoz, Laboratoire de bioénergétique fondamentale et appliquée (LBFA), Université Joseph Fourier - Grenoble 1 (UJF)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Université Grenoble Alpes (UGA), Assistance publique - Hôpitaux de Paris (AP-HP) (APHP)-Hôpital Robert Debré-Université Paris Diderot - Paris 7 (UPD7), Assistance publique - Hôpitaux de Paris (AP-HP) (APHP)-Hôpital Lariboisière-Université Paris Diderot - Paris 7 (UPD7), Assistance publique - Hôpitaux de Paris (AP-HP) (APHP)-CHU Cochin [AP-HP], Université de Rennes (UR)-CHU Pontchaillou [Rennes]-Hôpital Anne-de-Bretagne, Service Diabétologie [CHU Toulouse], Pôle Cardiovasculaire et Métabolique [CHU Toulouse], and Centre Hospitalier Universitaire de Toulouse (CHU Toulouse)-Centre Hospitalier Universitaire de Toulouse (CHU Toulouse)
Subjects: Position statement, Process management, Standardization, endocrine system diseases, Endocrinology, Diabetes and Metabolism, 030209 endocrinology & metabolism, Guidelines, Artificial pancreas, Subcutaneous insulin infusion, 03 medical and health sciences, Flash glucose monitoring, 0302 clinical medicine, Endocrinology, Patient Education as Topic, Internal Medicine, Medicine, Humans, 030212 general & internal medicine, Good practice, Continuous glucose monitoring, Reimbursement, Retrospective Studies, business.industry, Blood Glucose Self-Monitoring, nutritional and metabolic diseases, Type 2 diabetes, General Medicine, Patient education, [SDV.MHEP.EM]Life Sciences [q-bio]/Human health and pathology/Endocrinology and metabolism, [ SDV.MHEP.EM ] Life Sciences [q-bio]/Human health and pathology/Endocrinology and metabolism, 3. Good health, Diabetes Mellitus, Type 1, Type 1 diabetes, Diabetes Mellitus, Type 2, Practice Guidelines as Topic, Interstitial glucose, France, business
Abstract: International audience; The use by diabetes patients of real-time continuous interstitial glucose monitoring (CGM) or the FreeStyle Libre®(FSL) flash glucose monitoring (FGM) system is becoming widespread and has changed diabetic practice. The working group bringing together a number of French experts has proposed the present practical consensus. Training of professionals and patient education are crucial for the success of CGM. Also, institutional recommendations must pay particular attention to the indications for and reimbursement of CGM devices in populations at risk of hypoglycaemia. The rules of good practice for CGM are the precursors of those that need to be enacted, given the oncoming emergence of artificial pancreas devices. It is necessary to have software combining user-friendliness, multiplatform usage and average glucose profile (AGP) presentation, while integrating glucose and insulin data as well as events. Expression of CGM data must strive for standardization that facilitates patient phenotyping and their follow-up, while integrating indicators of variability. The introduction of CGM involves a transformation of treatment support, rendering it longer and more complex as it also includes specific educational and technical dimensions. This complexity must be taken into account in discussions of organization of diabetes care.
Published: 2018
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9. Antibiotic treatment of infections caused by carbapenem-resistant Gram-negative bacilli: an international ESCMID cross-sectional survey among infectious diseases specialists practicing in large hospitals

Author: Papst, Lea, Beović, Bojana, Pulcini, Céline, Durante-Mangoni, Emanuele, Rodríguez-Baño, Jesús, Kaye, Keith S, Daikos, George L, Raka, Lul, Paul, Mical, Esgap, Esgbis, ESGIE and the CRGNB treatment survey study group collaborators: Abbo, L, Abgueguen, P, Almirante, B, Azzini, Am, Bani-Sadr, F, Bassetti, M, Ben-Ami, R, Beović, B, Béraud, G, Botelho-Nevers, E, Bou, G, Boutoille, D, Cabié, A, Cacopardo, B, Cascio, A, Cassir, N, Castelli, F, Cecala, M, Charmillon, A, Chirouze, C, Cisneros, Jm, Colmenero, Jd, Coppola, N, Corcione, S, Daikos, Gl, Dalla Gasperina, D, De la Calle Cabrera, C, Delobel, P, Di Caprio, D, Durante Mangoni, E, Dupon, M, Ettahar, N, Falagas, Me, Falcone, M, Fariñas, Mc, Faure, E, Forestier, E, Foti, G, Gallagher, J, Gattuso, G, Gendrin, V, Gentile, I, Giacobbe, Dr, Gogos, Ca, Grandiere Perez, L, Hansmann, Y, Horcajada, Jp, Iacobello, C, Jacob, Jt, Justo, Ja, Kernéis, S, Komnos, A, Kotnik Kevorkijan, B, Lebeaux, D, Le Berre, R, Lechiche, C, Le Moing, V, Lescure, Fx, Libanore, M, Martinot, M, Merino de Lucas, E, Mondain, V, Mondello, P, Montejo, M, Mootien, J, Muñoz, P, Nir-Paz, R, Pan, A, Paño-Pardo, Jr, Patel, G, Paul, M, Pérez Rodríguez MT, Piroth, L, Pogue, J, Potoski, Ba, Pourcher, V, Pyrpasopoulou, A, Rahav, G, Rizzi, M, Rodríguez-Baño, J, Salavert, M, Scheetz, M, Sims, M, Spahija, G, Stefani, S, Stefos, A, Tamma, Pd, Tattevin, P, Tedesco, A, Torre-Cisneros, J, Tripolitsioti, P, Tsiodras, S, Uomo, G, Verdon, R, Viale, P, Vitrat, V, Weinberger, M, Wiener-Well, Y, Papst L., Beovic B., Pulcini C., Durante-Mangoni E., Rodriguez-Bano J., Kaye K.S., Daikos G.L., Raka L., Paul M., Abbo L., Abgueguen P., Almirante B., Azzini A.M., Bani-Sadr F., Bassetti M., Ben-Ami R., Beraud G., Botelho-Nevers E., Bou G., Boutoille D., Cabie A., Cacopardo B., Cascio A., Cassir N., Castelli F., Cecala M., Charmillon A., Chirouze C., Cisneros J.M., Colmenero J.D., Coppola N., Corcione S., Dalla Gasperina D., De la Calle Cabrera C., Delobel P., Di Caprio D., Durante Mangoni E., Dupon M., Ettahar N., Falagas M.E., Falcone M., Farinas M.C., Faure E., Forestier E., Foti G., Gallagher J., Gattuso G., Gendrin V., Gentile I., Giacobbe D.R., Gogos C.A., Grandiere Perez L., Hansmann Y., Horcajada J.P., Iacobello C., Jacob J.T., Justo J.A., Kerneis S., Komnos A., Kotnik Kevorkijan B., Lebeaux D., Le Berre R., Lechiche C., Le Moxing V., Lescure F.X., Libanore M., Martinot M., Merino de Lucas E., Mondain V., Mondello P., Montejo M., Mootien J., Munoz P., Nir-Paz R., Pan A., Pano-Pardo J.R., Patel G., Perez Rodriguez M.T., Piroth L., Pogue J., Potoski B.A., Pourcher V., Pyrpasopoulou A., Rahav G., Rizzi M., Salavert M., Scheetz M., Sims M., Spahija G., Stefani S., Stefos A., Tamma P.D., Tattevin P., Tedesco A., Torre-Cisneros J., Tripolitsioti P., Tsiodras S., Uomo G., Verdon R., Viale P., Vitrat V., Weinberger M., Wiener-Well Y., University Medical Centre Ljubljana [Ljubljana, Slovenia] (UMCL), Maladies chroniques, santé perçue, et processus d'adaptation (APEMAC), Université de Lorraine (UL), Monaldi Hospital, Hospital Virgen Macarena, University of Michigan Medical School [Ann Arbor], University of Michigan [Ann Arbor], University of Michigan System-University of Michigan System, National and Kapodistrian University of Athens (NKUA), University Clinical Center of Kosova, Rambam Health Care Campus, Jackson Memorial Hospital, Centre Hospitalier Universitaire d'Angers (CHU Angers), PRES Université Nantes Angers Le Mans (UNAM), Vall d'Hebron University Hospital [Barcelona], University of Verona (UNIVR), Centre Hospitalier Universitaire de Reims (CHU Reims), Ospedale 'Santa Maria della Misericordia' = University Hospital 'Santa Maria della Misericordia', Tel Aviv Sourasky Medical Centre, Centre hospitalier universitaire de Poitiers (CHU Poitiers), Centre Hospitalier Universitaire de Saint-Etienne (CHU de Saint-Etienne), Hospital Universitario, A Coruña, Centre hospitalier universitaire de Nantes (CHU Nantes), CHU de la Martinique [Fort de France], ARNAS 'Garibaldi, S. Luigi-Currò, Ascoli-Tomaselli', Università degli studi di Palermo - University of Palermo, Assistance Publique-Hôpitaux de Marseille (AP-HM), ASST Spedali Civili of Brescia, ARNAS Civico Palermo, Centre Hospitalier Régional Universitaire de Nancy (CHRU Nancy), Centre Hospitalier Régional Universitaire de Besançon (CHRU Besançon), Hospital Universitario Virgen del Rocío [Sevilla], Hospital Regional Universitario de Málaga [Spain], Università degli studi della Campania 'Luigi Vanvitelli', University of Turin, University of Insubria, Varese, Hospital Clínic de Barcelona, Hôpital Purpan [Toulouse], CHU Toulouse [Toulouse], AO San Sebastiano, CHU Bordeaux [Bordeaux], CH Valenciennes, Henry Dunant Hospital, Università degli Studi di Roma 'La Sapienza' = Sapienza University [Rome], Hospital Marques de Valdecillas, Centre Hospitalier Régional Universitaire [Lille] (CHRU Lille), Centre Hospitalier Métropole Savoie [Chambéry], Ospedale di Reggio Calabria, Children’s Hospital of Philadelphia (CHOP ), Carlo Poma Hospital Mantova (ASST Mantova ), CH Belfort-Montbéliard, University of Naples Federico II, AUO San Martino IST Ist Nazl Ric Canc, I-16132 Genoa, Italy, University of Patras [Patras], Centre Hospitalier Le Mans (CH Le Mans), CHU Strasbourg, IMIM-Hospital del Mar, Generalitat de Catalunya, Cannizzaro Hospital, Emory University School of Medicine, Emory University [Atlanta, GA], University of South Carolina [Columbia], AP-HP - Hôpital Cochin Broca Hôtel Dieu [Paris], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP), General Hospital of Larissa, University medical centre Maribor (UKC Maribor), Hôpital Européen Georges Pompidou [APHP] (HEGP), Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Hôpitaux Universitaires Paris Ouest - Hôpitaux Universitaires Île de France Ouest (HUPO), Centre Hospitalier Régional Universitaire de Brest (CHRU Brest), Centre Hospitalier Universitaire de Nîmes (CHU Nîmes), CHU Montpellier, Centre Hospitalier Régional Universitaire [Montpellier] (CHRU Montpellier), AP-HP - Hôpital Bichat - Claude Bernard [Paris], University of Ferrara at St. Anna Hospital, CH Colmar, Hospital General Universitario de Alicante, CHU Nice [Cimiez], Hôpital Cimiez [Nice] (CHU), AOU Policlinico 'G. Martino', Messina, Italy, Hospital Universitario Cruces = Cruces University Hospital, Centre Hospitalier Emile Muller [Mulhouse] (CH E.Muller Mulhouse), Groupe Hospitalier de Territoire Haute Alsace (GHTHA), Hospital General Universitario 'Gregorio Marañón' [Madrid], Hadassah Hebrew University Medical Center [Jerusalem], Azienda Istituti Ospitalieri di Cremona, Lozano Blesa Clinical Hospital [Zaragoza, Spain], Mount Sinai Hospital [Toronto, Canada] (MSH), Complejo Hospitalario de Vigo, CHU Dijon, Centre Hospitalier Universitaire de Dijon - Hôpital François Mitterrand (CHU Dijon), Detroit Medical Center, University of Pittsburgh Medical Center [Pittsburgh, PA, États-Unis] (UPMC), CHU Pitié-Salpêtrière [AP-HP], Sorbonne Université (SU)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP), Hippokration General Hospital, Sheba Medical Centre, Ramat Gan, Israel, ASST Papa Giovanni XXIII [Bergamo, Italy], Hospital Universitario La Fe, Valencia, Northwestern Hospital Chicago, Beaumont Hospital, Lagjia e Universitetit, Rruga 1, nr.32, 10000 Prishtina, Kosovo, parent, Università degli studi di Catania [Catania], Larissa University Hospital, Johns Hopkins University School of Medicine [Baltimore], CHU Pontchaillou [Rennes], Ospedale Fracastoro San Bonifacio [Verona], Hospital Reina Sofia, Cordoba, Agioi Anargiroi Hospital, Attikon University Hospital, Ospedale Cardarelli, CHU Caen, Normandie Université (NU)-Tumorothèque de Caen Basse-Normandie (TCBN), Alma Mater Studiorum Università di Bologna [Bologna] (UNIBO), CH Annecy Genevois, Assah Harofeh Medical Centre, Zerifin, Israel, Shaare Zedek Medical Centre, Jerusalem, Israel, National Institutes of Health (US), Instituto de Salud Carlos III, Ministerio de Economía, Industria y Competitividad (España), Red Española de Investigación en Patología Infecciosa, European Commission, Papst, Lea, Beović, Bojana, Pulcini, Céline, Durante-Mangoni, Emanuele, Rodríguez-Baño, Jesú, Kaye, Keith S, Daikos, George L, Raka, Lul, Paul, Mical, Papst, L., Beovic, B., Pulcini, C., Durante-Mangoni, E., Rodriguez-Bano, J., Kaye, K. S., Daikos, G. L., Raka, L., Paul, M., Abbo, L., Abgueguen, P., Almirante, B., Azzini, A. M., Bani-Sadr, F., Bassetti, M., Ben-Ami, R., Beraud, G., Botelho-Nevers, E., Bou, G., Boutoille, D., Cabie, A., Cacopardo, B., Cascio, A., Cassir, N., Castelli, F., Cecala, M., Charmillon, A., Chirouze, C., Cisneros, J. M., Colmenero, J. D., Coppola, N., Corcione, S., Dalla Gasperina, D., De la Calle Cabrera, C., Delobel, P., Di Caprio, D., Dupon, M., Ettahar, N., Falagas, M. E., Falcone, M., Farinas, M. C., Faure, E., Forestier, E., Foti, G., Gallagher, J., Gattuso, G., Gendrin, V., Gentile, I., Giacobbe, D. R., Gogos, C. A., Grandiere Perez, L., Hansmann, Y., Horcajada, J. P., Iacobello, C., Jacob, J. T., Justo, J. A., Kerneis, S., Komnos, A., Kotnik Kevorkijan, B., Lebeaux, D., Le Berre, R., Lechiche, C., Le Moxing, V., Lescure, F. X., Libanore, M., Martinot, M., Merino de Lucas, E., Mondain, V., Mondello, P., Montejo, M., Mootien, J., Munoz, P., Nir-Paz, R., Pan, A., Pano-Pardo, J. R., Patel, G., Perez Rodriguez, M. T., Piroth, L., Pogue, J., Potoski, B. A., Pourcher, V., Pyrpasopoulou, A., Rahav, G., Rizzi, M., Salavert, M., Scheetz, M., Sims, M., Spahija, G., Stefani, S., Stefos, A., Tamma, P. D., Tattevin, P., Tedesco, A., Torre-Cisneros, J., Tripolitsioti, P., Tsiodras, S., Uomo, G., Verdon, R., Viale, P., Vitrat, V., Weinberger, M., Wiener-Well, Y., Università degli studi di Verona = University of Verona (UNIVR), Assistance Publique - Hôpitaux de Marseille (APHM), Hospital Regional Universitario de Málaga = Regional University Hospital of Malaga [Spain], and Università degli studi di Torino = University of Turin (UNITO)
Subjects: 0301 basic medicine, Acinetobacter baumannii, Carbapenem, Antibiotics, Drug Resistance, Drug resistance, Tigecycline, Carbapenem-resistant Gram-negative bacilli, Combination therapy, Enterobacteriaceae, Polymyxin, Pseudomonas aeruginosa, Survey, 0302 clinical medicine, Surveys and Questionnaires, polycyclic compounds, 030212 general & internal medicine, Anti-Bacterial Agents, Carbapenems, Cross Infection, Cross-Sectional Studies, Drug Resistance, Bacterial, Gram-Negative Bacteria, Gram-Negative Bacterial Infections, Hospitals, Humans, Microbial Sensitivity Tests, Microbiology (medical), Infectious Diseases, biology, Microbial Sensitivity Test, Bacterial, antibiotic management, carbapenem-resistant Gram-negative bacteria, General Medicine, 3. Good health, medicine.drug, Human, medicine.medical_specialty, medicine.drug_class, 030106 microbiology, Fosfomycin, carbapenem-resistant Gram-negative bacteria, 03 medical and health sciences, Hospital, Internal medicine, Anti-Bacterial Agent, medicine, Gram-Negative Bacterial Infection, Cross-Sectional Studie, business.industry, biochemical phenomena, metabolism, and nutrition, biology.organism_classification, bacterial infections and mycoses, Infectious disease (medical specialty), Carbapenem-resistant gram-negative bacilli, [SDV.SPEE]Life Sciences [q-bio]/Santé publique et épidémiologie, antibiotic management, business, Rifampicin
Abstract: ESGAP, ESGBIS, ESGIE and the CRGNB treatment survey study group., [Objectives] To explore contemporary antibiotic management of infections caused by carbapenem-resistant Gram-negative bacteria in hospitals., [Methods] Cross-sectional, internet-based questionnaire survey. We contacted representatives of all hospitals with more than 800 acute-care hospital beds in France, Greece, Israel, Italy, Kosovo, Slovenia, Spain and selected hospitals in the USA. We asked respondents to describe the most common actual practice at their hospital regarding management of carbapenem-resistant Enterobacteriaceae, Acinetobacter baumannii and Pseudomonas aeruginosa through close-ended questions., [Results] Between January and June 2017, 115 of 141 eligible hospitals participated (overall response rate 81.6%, country-specific rates 66.7%–100%). Most were tertiary-care (99/114, 86.8%), university-affiliated (110/115, 89.1%) hospitals and most representatives were infectious disease specialists (99/115, 86.1%). Combination therapy was prescribed in 114/115 (99.1%) hospitals at least occasionally. Respondents were more likely to consider combination therapy when treating bacteraemia, pneumonia and central nervous system infections and for Enterobacteriaceae, P. aeruginosa and A. baumannii similarly. Combination of a polymyxin with a carbapenem was used in most cases, whereas combinations of a polymyxin with tigecycline, an aminoglycoside, fosfomycin or rifampicin were also common. Monotherapy was used for treatment of complicated urinary tract infections, usually with an aminoglycoside or a polymyxin. The intended goal of combination therapy was to improve the effectiveness of the treatment and to prevent development of resistance. In general, respondents shared the misconception that combination therapy is supported by strong scientific evidence., [Conclusions] Combination therapy was the preferred treatment strategy for infections caused by carbapenem-resistant Gram-negative bacteria among hospital representatives, even though high-quality evidence for carbapenem-based combination therapy is lacking., EDM received funding by NIH for project HHSN272201000039C. JRB received funding for research from Plan Nacional de I + D + i 2013–2016 and Instituto de Salud Carlos III, Subdirección General de Redes y Centros de Investigación Cooperativa, Ministerio de Economía, Industria y Competitividad, Spanish Network for Research in Infectious Diseases (REIPI RD16/0016/0001)—co-financed by European Development Regional Fund A way to achieve Europe, Operative Programme Intelligent Growth 2014–2020.
Published: 2018
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10. Cogan syndrome: Characteristics, outcome and treatment in a French nationwide retrospective study and literature review

Author: Robin Dhote, Christophe Deligny, Charlotte Durtette, Edouard Pertuiset, Christian Lavigne, Anne Grasland, Thomas Quemeneur, Eric Hachulla, Thomas Papo, Pascal Sève, Matthieu Resche-Rigon, Pierre-Yves Hatron, Thierry Zenone, Marc Lambert, Arsène Mekinian, Thomas Le Gallou, Benoit De Wazieres, Mohamed Hamidou, Guillaume Gondran, Cédric Landron, Bertrand Lioger, Jacques Pouchot, Jean Emmanuel Kahn, Snfmi, Olivier Fain, Service de Médecine Interne [CHU Saint-Antoine], Assistance publique - Hôpitaux de Paris (AP-HP) (APHP)-CHU Saint-Antoine [APHP], Service de rhumatologie, inflammation-immunopathologie- biothérapie [CHU Saint-Antoine] (DHU i2B ), CHU Saint-Antoine [APHP]-Assistance publique - Hôpitaux de Paris (AP-HP) (APHP), Sorbonne Université - Faculté de Médecine (SU FM), Sorbonne Université (SU), Service de médecine interne [Lille], Centre Hospitalier Régional Universitaire [Lille] (CHRU Lille), Service de biostatistiques et information médicale [Saint-Louis], Université Paris Diderot - Paris 7 (UPD7)-Groupe Hospitalier Saint Louis - Lariboisière - Fernand Widal [Paris], Assistance publique - Hôpitaux de Paris (AP-HP) (APHP)-Assistance publique - Hôpitaux de Paris (AP-HP) (APHP), AP-HP - Hôpital Bichat - Claude Bernard [Paris], Centre hospitalier de Valence, Centre Hospitalier Régional Universitaire de Tours (CHRU TOURS), Service de Médecine interne [CHU de Fort-de France], CHU de Fort de France, Centre hospitalier universitaire de Poitiers (CHU Poitiers), Service de Médecine Interne [CHU HEGP], Assistance publique - Hôpitaux de Paris (AP-HP) (APHP)-Hôpital Européen Georges Pompidou [APHP] (HEGP), Hôpitaux Universitaires Paris Ouest - Hôpitaux Universitaires Île de France Ouest (HUPO)-Assistance publique - Hôpitaux de Paris (AP-HP) (APHP)-Hôpitaux Universitaires Paris Ouest - Hôpitaux Universitaires Île de France Ouest (HUPO)-Centre de référence des syndromes drépanocytaires majeurs, Université Paris Descartes - Paris 5 (UPD5), Université Sorbonne Paris Cité (USPC), Assistance publique - Hôpitaux de Paris (AP-HP) (APHP), Hôpital Foch [Suresnes], Centre Hospitalier Universitaire d'Angers (CHU Angers), PRES Université Nantes Angers Le Mans (UNAM), Centre Hospitalier Régional Universitaire de Nîmes (CHRU Nîmes), Université de Montpellier (UM), Service de médecine interne [Avicenne], Hôpital Avicenne, Service de Médecine interne A et polyclinique médicale [CHU Limoges], CHU Limoges, Centre Hospitalier René Dubos [Pontoise], CH Valenciennes, Service de médecine interne [Nantes], Université de Nantes (UN)-Hôtel-Dieu-Centre hospitalier universitaire de Nantes (CHU Nantes), Hôpital de la Croix-Rousse [CHU - HCL], Hospices Civils de Lyon (HCL), Hopital Louis Mourier - AP-HP [Colombes], CHU Saint-Antoine [AP-HP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU), Service de rhumatologie [CHU Saint-Antoine], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Université Paris Diderot - Paris 7 (UPD7)-Groupe Hospitalier Saint Louis - Lariboisière - Fernand Widal [Paris], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP), Centre de référence des syndromes drépanocytaires majeurs-Hôpital Européen Georges Pompidou [APHP] (HEGP), Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Hôpitaux Universitaires Paris Ouest - Hôpitaux Universitaires Île de France Ouest (HUPO)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Hôpitaux Universitaires Paris Ouest - Hôpitaux Universitaires Île de France Ouest (HUPO), Centre Hospitalier Universitaire de Nîmes (CHU Nîmes), Hôpital Avicenne [AP-HP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP), Hôpital Louis Mourier - AP-HP [Colombes], and Centre Hospitalier Régional Universitaire de Tours (CHRU Tours)
Subjects: Adult, Male, medicine.medical_specialty, Cogan's syndrome, Adolescent, Interstitial keratitis, Cogan syndrome, Immunology, 03 medical and health sciences, Young Adult, 0302 clinical medicine, Internal medicine, medicine, Immunology and Allergy, Cogan Syndrome, Humans, 030223 otorhinolaryngology, Prospective cohort study, Child, Aged, Retrospective Studies, Outcome, 030203 arthritis & rheumatology, Keratitis, business.industry, Tumor Necrosis Factor-alpha, Bilateral hearing loss, Retrospective cohort study, Odds ratio, Middle Aged, medicine.disease, Confidence interval, Infliximab, 3. Good health, Surgery, Treatment, Treatment Outcome, Antirheumatic Agents, Child, Preschool, [SDV.IMM]Life Sciences [q-bio]/Immunology, Female, business, medicine.drug
Abstract: Background Cogan syndrome is mainly treated with steroids. We aimed to determine the place of DMARDs and biologic-targeted treatments. Patients and methods We conducted a French nationwide retrospective study of patients with Cogan syndrome (n = 40) and a literature review of cases (n = 22) and analyzed the efficacy of disease-modifying anti-rheumatic drugs (DMARDs) and tumor necrosis factor α (TNF-α) antagonists. Results We included 62 patients (31 females) (median age 37 years [range 2–76]. At diagnosis, 61 patients (98%) had vestibulo-auditory symptoms, particularly bilateral hearing loss in 41% and deafness in 31%. Ocular signs were present in 57 patients (92%), with interstitial keratitis in 31 (51%). The first-line treatment consisted of steroids alone (n = 43; 70%) or associated with other immunosuppressive drugs (n = 18; 30%). Overall, 13/43 (30%) and 4/18 (22%) patients with steroids alone and with associated immunosuppressive drugs, respectively (p = 0.8), showed vestibulo-auditory response; 32/39 (82%) and 15/19 (79%) ocular response; and 23/28 (82%) and 10/14 (71%) general response. Overall 61 patients had used a total of 126 lines of treatment, consisting of steroids alone (n = 51 lines), steroids with DMARDs (n = 65) and infliximab (n = 10). Vestibulo-auditory response was significantly more frequent with infliximab than DMARDs or steroids alone (80% vs 39% and 35%, respectively), whereas ocular, systemic and acute-phase reactant response rates were similar. Infliximab was the only significant predictor of vestibulo-auditory improvement (odds ratio 20.7 [95% confidence interval 1.65; 260], p = 0.019). Conclusion Infliximab could lead to vestibulo-auditory response in DMARDS and steroid-refractory Cogan syndrome, but prospective studies are necessary.
Published: 2017
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11. Value of biomarkers for predicting immunoglobulin A vasculitis nephritis outcome in an adult prospective cohort

Author: Claire Demongeot, Manuelle Viguier, Jean-Jacques Boffa, Stéphanie Tellier, Margarita Hurtado-Nedelec, Vincent Pestre, Gérard Cheron, Guillaume Burda, Martin Flamant, Héloïse Flament, Jérôme Verine, Alexandre Seidowsky, E Bourrat, Stéphane Decramer, Mathilde de Menthon, Karine Brochard, Karim Bouchireb, Maud Bezier, Evangeline Pillebout, Fabrice Mihout, Martine Bagot, Olivier Fain, Maya Halabi-Tawil, Agnès Jamin, Philippe Remy, Sonia Azib, Vincent Audard, Monique Dehoux, Jacqueline Rivet, Quentin Raimbourg, Denis Viglietti, Alexis Mathian, Laure Champion, Guillaume Bussone, Bertrand Godeau, Eric Daugas, Anne Saussine-Hickman, Antoine Froissart, Eric Thervet, Elisa Funck-Brentano, Alain Robert, Laureline Berthelot, Nathalie Vittoz, Nicolas Limal, Denis Glotz, Georges Deschênes, Isabelle Halphen, Theresa Kwon, Laurence Vrigneaud, Pierre Housset, Sophie Georgin-Lavialle, Florence Cordoliani, Arsene Mekinian, Virginia Sauvaget, Philippe Grimbert, Jean Christophe Mercier, Philippe Vanhille, Véronique Baudouin, François Vrtovsnik, Agathe Raynaud-Simon, Renato C. Monteiro, Jean-David Bouaziz, Leila Tricot, Laurène Dehoux, Melissa Pierre, Alexandre Karras, Laure Dehen, Céline Lebas, Nathalie Bocquet, Marc Fila, Claire Dossier, Thomas Quemeneur, Maryam Piram, Coralie Bloch-Queyrat, Antoine Dossier, David Verhelst, Anne Maisin, Olivier Benveniste, Dominique Farge, Charlotte Fite, Loïc Guillevin, Rémi Salomon, Emmanuelle Vidal-Petiot, Zahir Amoura, Valérie Caudwell, Assia Smail, Philippe Bouvier, Michel Delahousse, Gentiane Monsel, Jonathan M. Chemouny, Hamza Ayari, Anne-Sophie Verhoeven, Centre de Recherche en Transplantation et Immunologie (U1064 Inserm - CRTI), Institut National de la Santé et de la Recherche Médicale (INSERM)-Université de Nantes - UFR de Médecine et des Techniques Médicales (UFR MEDECINE), Université de Nantes (UN)-Université de Nantes (UN), Centre de recherche sur l'Inflammation (CRI (UMR_S_1149 / ERL_8252 / U1149)), Université Paris Diderot - Paris 7 (UPD7)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Centre National de la Recherche Scientifique (CNRS), Service de Néphrologie [Saint-Louis], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Groupe Hospitalier Saint Louis - Lariboisière - Fernand Widal [Paris], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP), Service de Néphrologie [Bichat - Claude Bernard], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-AP-HP - Hôpital Bichat - Claude Bernard [Paris], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Université Paris Diderot - Paris 7 (UPD7), UMR 1599, Centre National de la Recherche Scientifique (CNRS), Service de médecine interne [CHU Pitié-Salpétrière], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-CHU Pitié-Salpêtrière [AP-HP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU)-Sorbonne Université (SU), Service de néphrologie [CHU Henri Mondor], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-CHU Henri Mondor, Service de néphrologie pédiatrique, Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Hôpital Robert Debré, Institut Mondor de recherche biomédicale (IMRB), Institut National de la Santé et de la Recherche Médicale (INSERM)-Université Paris-Est Créteil Val-de-Marne - Paris 12 (UPEC UP12), Service de neuropédiatrie et maladies métaboliques [CHU Robert-Debré], Sorbonne Université (SU), Hôpital Saint-Louis, Institut National de l'Environnement Industriel et des Risques (INERIS), Remodelage et Reparation du Tissu Renal, Université Pierre et Marie Curie - Paris 6 (UPMC)-Institut National de la Santé et de la Recherche Médicale (INSERM), Service de Dermatologie [AP-HP Hôpital Saint-Louis], Hopital Saint-Louis [AP-HP] (AP-HP), Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP), Department of dermatology, Groupe Hospitalier Saint Louis - Lariboisière - Fernand Widal [Paris], Service Pédiatrique [Toulouse], CHU Toulouse [Toulouse], Institut Cochin (IC UM3 (UMR 8104 / U1016)), Université Paris Descartes - Paris 5 (UPD5)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Centre National de la Recherche Scientifique (CNRS), Centre Hospitalier Sud Francilien, Department of Dermatology, Institut de médecine moléculaire de Rangueil (I2MR), Université Toulouse III - Paul Sabatier (UT3), Université Fédérale Toulouse Midi-Pyrénées-Université Fédérale Toulouse Midi-Pyrénées- Institut Fédératif de Recherche Bio-médicale Institution (IFR150)-Institut National de la Santé et de la Recherche Médicale (INSERM), Service de dermatologie [Paris], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Université Paris Diderot - Paris 7 (UPD7)-Groupe Hospitalier Saint Louis - Lariboisière - Fernand Widal [Paris], Service de transplantation rénale, Hôpital Foch [Suresnes], AP-HP, Service de Néphrologie Pédiatrique, Hôpital Robert Debré, Paris, Hôpital Bicêtre, Service de Médecine Interne, Centre de Recherche Saint-Antoine (UMRS893), Physiopathologie et Epidemiologie de l'Insuffisance Respiratoire, Université Paris Diderot - Paris 7 (UPD7)-Institut National de la Santé et de la Recherche Médicale (INSERM), Service de médecine interne, Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Hôpital Jean Verdier [AP-HP], Centre Hospitalier Régional Universitaire [Montpellier] (CHRU Montpellier), Service de dermatologie, Service de Physiologie [Bichat-Claude Bernard], Université Pierre et Marie Curie - Paris 6 (UPMC)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-AP-HP - Hôpital Bichat - Claude Bernard [Paris], Immunité et cancer (U932), Université Paris Descartes - Paris 5 (UPD5)-Institut Curie [Paris]-Institut National de la Santé et de la Recherche Médicale (INSERM), Biomarqueurs et essais cliniques en Cancérologie et Onco-Hématologie (BECCOH), Université de Versailles Saint-Quentin-en-Yvelines (UVSQ)-Université Paris-Saclay, Physiopathologie des maladies génétiques d'expression pédiatrique, Service de médecine interne [Mondor], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Hôpital Henri Mondor-Université Paris-Est Créteil Val-de-Marne - Paris 12 (UPEC UP12), Service de médecine interne et centre de référence des maladies rares [CHU Cochin], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Hôpital Cochin [AP-HP], Hôpital Européen Georges Pompidou [APHP] (HEGP), Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Hôpitaux Universitaires Paris Ouest - Hôpitaux Universitaires Île de France Ouest (HUPO), AP-HP Hôpital universitaire Robert-Debré [Paris], Service de Néphrologie [CHRU Lille], Centre Hospitalier Régional Universitaire [Lille] (CHRU Lille), Service de rhumatologie [CHU Saint-Antoine], CHU Saint-Antoine [AP-HP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU), Service de Département de Néphrologie = Service de Néphrologie et Dialyses [CHU Tenon], CHU Tenon [AP-HP], Service de Pédiatrie Générale et Rhumatologie Pédiatrique, Université Paris-Sud - Paris 11 (UP11)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Hôpital Bicêtre, CH Valenciennes, Université Paris Descartes - Paris 5 (UPD5), Gvh et Gvl : Physiopathologie Chez l'Homme et Chez l'Animal, Incidence et Role Therapeutique, Développement et Communication Chimique chez les Insectes (DCCI), Université de Bourgogne (UB)-Centre National de la Recherche Scientifique (CNRS), Service de néphrologie pédiatrique [CHU Necker], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-CHU Necker - Enfants Malades [AP-HP], Centre de Référence du Sud Ouest des Maladies Rénales Rares, Service de néphrologie adultes [CHU Necker], Service de médecine interne et néphrologie, Centre Hospitalier Henri Duffaut (Avignon), Service de Néphrologie [Valenciennes], Centre Hospitalier de Valenciennes, Service d'anatomo-pathologie [Paris], Paris-Centre de Recherche Cardiovasculaire (PARCC - UMR-S U970), Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Hôpitaux Universitaires Paris Ouest - Hôpitaux Universitaires Île de France Ouest (HUPO)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Hôpitaux Universitaires Paris Ouest - Hôpitaux Universitaires Île de France Ouest (HUPO)-Université Paris Descartes - Paris 5 (UPD5)-Institut National de la Santé et de la Recherche Médicale (INSERM), CHU Pitié-Salpêtrière [AP-HP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU), Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-CHU Henri Mondor [Créteil], Pôle Enfants [CHU Toulouse], Centre Hospitalier Universitaire de Toulouse (CHU Toulouse), Université de Toulouse (UT)-Université de Toulouse (UT)- Institut Fédératif de Recherche Bio-médicale Institution (IFR150)-Institut National de la Santé et de la Recherche Médicale (INSERM), Université Fédérale Toulouse Midi-Pyrénées-Université Fédérale Toulouse Midi-Pyrénées-IFR150-Institut National de la Santé et de la Recherche Médicale (INSERM), Service de Néphrologie et Dialyses [CHU Tenon], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-CHU Tenon [AP-HP], Institut National de la Santé et de la Recherche Médicale (INSERM)-Centre National de la Recherche Scientifique (CNRS)-Université Paris Diderot - Paris 7 (UPD7), CHU Toulouse [Toulouse]-Hôpital des Enfants, Centre National de la Recherche Scientifique (CNRS)-Université Paris Descartes - Paris 5 (UPD5)-Institut National de la Santé et de la Recherche Médicale (INSERM), CH Evry-Corbeil, Université Paris Diderot - Paris 7 (UPD7)-Groupe Hospitalier Saint Louis - Lariboisière - Fernand Widal [Paris], Sorbonne Université (SU)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU), Centre National de la Recherche Scientifique (CNRS)-Université de Bourgogne (UB)-AgroSup Dijon - Institut National Supérieur des Sciences Agronomiques, de l'Alimentation et de l'Environnement, Université Paris Descartes - Paris 5 (UPD5)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Hôpital Européen Georges Pompidou [APHP] (HEGP), Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Hôpitaux Universitaires Paris Ouest - Hôpitaux Universitaires Île de France Ouest (HUPO)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Hôpitaux Universitaires Paris Ouest - Hôpitaux Universitaires Île de France Ouest (HUPO), Université Paris Diderot - Paris 7 (UPD7)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP), Université Paris Diderot - Paris 7 (UPD7)-AP-HP - Hôpital Bichat - Claude Bernard [Paris], Institut National de la Santé et de la Recherche Médicale (INSERM)-Université Pierre et Marie Curie - Paris 6 (UPMC), and Hôpital Cochin [AP-HP]
Subjects: 0301 basic medicine, Immunoglobulin A, Adult, Male, medicine.medical_specialty, IgA Vasculitis, Urinary system, 030232 urology & nephrology, Antigen-Antibody Complex, Gastroenterology, [SDV.MHEP.UN]Life Sciences [q-bio]/Human health and pathology/Urology and Nephrology, 03 medical and health sciences, Young Adult, 0302 clinical medicine, Internal medicine, medicine, Humans, Prospective Studies, 10. No inequality, Prospective cohort study, ComputingMilieux_MISCELLANEOUS, Aged, Transplantation, Proteinuria, Nephritis, biology, business.industry, Middle Aged, medicine.disease, Prognosis, 3. Good health, 030104 developmental biology, IgA vasculitis, ROC Curve, Nephrology, biology.protein, Female, medicine.symptom, Vasculitis, business, Biomarkers, Systemic vasculitis, Glomerular Filtration Rate
Abstract: Background Henoch-Schonlein purpura, more recently renamed immunoglobulin A vasculitis (IgAV), is a systemic vasculitis characterized by IgA deposits. The current markers used to assess IgAV inaccurately evaluate the risk of nephritis occurrence and its long-term outcomes. The current study assessed biomarkers of nephritis outcomes. Methods This French multicentre prospective study enrolled 85 adult patients at the time of disease onset. Patients were assessed for clinical and biological parameters and re-examined after 1 year. Immunoglobulins, cytokines, IgA glycosylation, IgA complexes and neutrophil gelatinase-associated lipocalin (NGAL) concentrations were assessed in blood and urine. Results We identified 60 patients with IgAV-related nephritis (IgAV-N) and 25 patients without nephritis (IgAV-woN). At the time of inclusion (Day 1), the serum levels of galactose-deficient IgA1 (Gd-IgA1) and urinary concentrations of IgA, IgG, IgM, NGAL, interleukin (IL)-1β, IL-6, IL-8, IL-10, IgA-IgG and IgA-sCD89 complexes were higher in the IgAV-N patients than in the IgAV-woN patients (P
Published: 2017
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12. Adding Azathioprine to Remission-Induction Glucocorticoids for Eosinophilic Granulomatosis With Polyangiitis (Churg-Strauss), Microscopic Polyangiitis, or Polyarteritis Nodosa Without Poor Prognosis Factors

Author: Puechal, Xavier, Pagnoux, Christian, Baron, Gabriel, Quemeneur, Thomas, Néel, Antoine, Agard, Christian, Lifermann, François, Liozon, Eric, Ruivard, Marc, Godmer, Pascal, Limal, Nicolas, Mekinian, Arsène, Papo, Thomas, Ruppert, Anne, Bourgarit, Anne, Bienvenu, Boris, Geffray, Loïc, Saraux, Luc, Diot, Elisabeth, Crestani, Bruno, Delbrel, Xavier, Sailler, Laurent, Cohen, Pascal, Le Guern, Véronique, Terrier, Benjamin, Groh, Matthieu, Le Jeunne, Claire, Mouthon, Luc, Ravaud, Philippe, Guillevin, Loïc, Pineton de Chambrun, Marc, Luyt, Charles-Edouard, Beloncle, Francois, Gousseff, Marie, Mauhin, Wladimir, Argaud, Laurent, Ledochowski, Stanislas, Moreau, Anne-Sophie, Sonneville, Romain, Verdière, Bruno, Merceron, Sybille, Zappella, Nathalie, Landais, Mickael, Contou, Damien, Demoule, Alexandre, Paulus, Sylvie, Souweine, Bertrand, Lecomte, Bernard, Vieillard-Baron, Antoine, Terzi, Nicolas, Azoulay, Elie, Friolet, Raymond, Puidupin, Marc, Devaquet, Jérôme, Mazou, Jean-Marc, Fédun, Yannick, Mira, Jean-Paul, Raphalen, Jean-Herlé, Combes, Alain, Amoura, Zahir, Lega, Jean-Christophe, Lambert, Marc, Rivière, Sophie, Dossier, Antoine, Lhote, François, Blaison, Gilles, Alric, Laurent, Saadoun, David, Graveleau, Julie, Soubrier, Martin, Lecomte, Marie-Josée, Christides, Christine, Bosseray, Annick, Lévesque, Hervé, Viallard, François, Tieulie, Nathalie, Lovey, Pierre-Yves, Le Moal, Sylvie, Bibes, Béatrice, Malizia, Giuseppe, Abgueguen, Pierre, Liferman, François, Ninet, Jacques, Hatron, Pierre-Yves, Hot, Arnaud, Hernu, Romain, de La Salle, Sylvie, Similowski, Thomas, Haroche, Julien, Boileau, Julien, Hanslik, Thomas, Bulte, Caroline, Talasczka, Aline, Hachulla, Eric, Decaux, Olivier, Ibouanga, Florent, Arnulf, Bertrand, Benedit, Marc, Maalouf, Assaad, Moulin, Bruno, Cohen-Aubart, Fleur, Pha, Micheline, Rivard, Georges-Etienne, Rondeau, Eric, Debourdeau, Philippe, Presne, Claire, Andrieu, Maude, Len Abad, Oscar, Devilliers, Hervé, Rogers, Alister, Hie, Miguel, Mathian, Alexis, Heidelberger, Valentine, Ingen-Housz-Oro, Saskia, Marquet, Alicia, Mahevas, Matthieu, Bessis, Didier, Bouillet, Laurence, Caux, Frédéric, Chapelon-Abric, Catherine, Debarbieux, Sebastien, Delaporte, Emmanuel, Duval-Modeste, Anne-Bénédicte, Fain, Olivier, Joly, Pascal, Marchand-Adam, Marc, Monfort, Jean-Benoît, Noel, Nicolas, Passeron, Thierry, Sarrot-Reynauld, Françoise, Verrot, Denis, Bouvry, Diane, Fardet, Laurence, Chosidow, Olivier, Sève, Pascal, Valeyre, Dominique, Moreau, Sophie, Centre Hospitalier Le Mans (CH Le Mans), Service de médecine interne et centre de référence des maladies rares [CHU Cochin], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Hôpital Cochin [AP-HP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP), Modèles et méthodes de l'évaluation thérapeutique des maladies chroniques (U738 / UMR_S738), Institut National de la Santé et de la Recherche Médicale (INSERM)-Université Paris Diderot - Paris 7 (UPD7), Département de Médecine Interne (VALENCIENNES - Med Int), CH Valenciennes, Service de médecine interne [Nantes], Université de Nantes (UN)-Hôtel-Dieu-Centre hospitalier universitaire de Nantes (CHU Nantes), Service de Médecine Interne [Dax], Centre Hospitalier de Dax, Service de Médecine interne A et polyclinique médicale [CHU Limoges], CHU Limoges, CHU Estaing [Clermont-Ferrand], CHU Clermont-Ferrand, Service de médecine interne [CHU Bretagnes Atlantique], CHU Bretagnes Atlantique, Service de rhumatologie [CHU Saint-Antoine], CHU Saint-Antoine [AP-HP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU), Department of Internal Medicine, Université Paris Diderot - Paris 7 (UPD7), Centre d'Immunologie et de Maladies Infectieuses (CIMI), Centre National de la Recherche Scientifique (CNRS)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Université Pierre et Marie Curie - Paris 6 (UPMC), CHU Caen, Normandie Université (NU)-Tumorothèque de Caen Basse-Normandie (TCBN), Médecine interne, Centre Hospitalier Régional Universitaire de Tours (CHRU Tours), Physiopathologie et Epidemiologie de l'Insuffisance Respiratoire, Université Paris Diderot - Paris 7 (UPD7)-Institut National de la Santé et de la Recherche Médicale (INSERM), Service de Médecine Interne [Pau], Centre hospitalier de Pau, Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU), Immunologie - Immunopathologie - Immunothérapeutique (I3), Université Pierre et Marie Curie - Paris 6 (UPMC)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Centre National de la Recherche Scientifique (CNRS), Service des maladies infectieuses et tropicales, Université Paris Diderot - Paris 7 (UPD7)-AP-HP - Hôpital Bichat - Claude Bernard [Paris], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP), Institut Cochin (IC UM3 (UMR 8104 / U1016)), Centre National de la Recherche Scientifique (CNRS)-Université Paris Descartes - Paris 5 (UPD5)-Institut National de la Santé et de la Recherche Médicale (INSERM), Service de Chirurgie Orthopédique et traumatique, Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-AP-HP - Hôpital Bichat - Claude Bernard [Paris], Centre de référence pour les vascularites nécrosantes et la sclérodermie systémique, Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Université Paris Descartes - Paris 5 (UPD5)-Faculté de Médecine-pôle de Médecine Interne, CHU Tenon [AP-HP], Sorbonne Université (SU)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP), Unité de Recherche sur les Maladies Cardiovasculaires, du Métabolisme et de la Nutrition = Institute of cardiometabolism and nutrition (ICAN), Université Pierre et Marie Curie - Paris 6 (UPMC)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Institut National de la Santé et de la Recherche Médicale (INSERM)-CHU Pitié-Salpêtrière [AP-HP], Sorbonne Université (SU)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU), Service de Réanimation Médicale et de Médecine Hyperbare [Angers], Centre Hospitalier Universitaire d'Angers (CHU Angers), PRES Université Nantes Angers Le Mans (UNAM)-PRES Université Nantes Angers Le Mans (UNAM), Institut Pasteur de Madagascar, Réseau International des Instituts Pasteur (RIIP), Centre de recherche en myologie, Université Pierre et Marie Curie - Paris 6 (UPMC)-Association française contre les myopathies (AFM-Téléthon)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Centre National de la Recherche Scientifique (CNRS), Hôpital Edouard Herriot [CHU - HCL], Hospices Civils de Lyon (HCL), Ecole Polytechnique Fédérale de Lausanne (EPFL), Hôpital Bichat - Claude Bernard, Service de soins intensifs, Centre Hospitalier de Versailles André Mignot (CHV), Service de réanimation médicale, Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Hôpital Henri Mondor-Université Paris-Est Créteil Val-de-Marne - Paris 12 (UPEC UP12), Neurophysiologie Respiratoire Expérimentale et Clinique, Université Pierre et Marie Curie - Paris 6 (UPMC)-Institut National de la Santé et de la Recherche Médicale (INSERM), Laboratoire Microorganismes : Génome et Environnement (LMGE), Université Blaise Pascal - Clermont-Ferrand 2 (UBP)-Centre National de la Recherche Scientifique (CNRS)-Université d'Auvergne - Clermont-Ferrand I (UdA), Médecine générale, Service de réanimation medico-chirurgicale [CHU Raymond-Poincaré], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Hôpital Ambroise Paré [AP-HP], Service de réanimation médicale [CHU Caen], Normandie Université (NU)-Tumorothèque de Caen Basse-Normandie (TCBN)-Normandie Université (NU)-Tumorothèque de Caen Basse-Normandie (TCBN)-Université de Caen Normandie (UNICAEN), Normandie Université (NU), Medical ICU, Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Groupe Hospitalier Saint Louis - Lariboisière - Fernand Widal [Paris], Service d'anesthésie-réanimation SAMU94-SMUR94 [Mondor], Institut Cochin (UMR_S567 / UMR 8104), Université Paris Descartes - Paris 5 (UPD5)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Centre National de la Recherche Scientifique (CNRS), Immunité et Infection, Université Pierre et Marie Curie - Paris 6 (UPMC)-IFR113-Institut National de la Santé et de la Recherche Médicale (INSERM), Centre Hospitalier Universitaire de Montpellier (CHU Montpellier ), Centre National de la Recherche Scientifique (CNRS), Hôpital pasteur [Colmar], Service de Medecine Interne, CHU Toulouse [Toulouse]-Hôpital Purpan [Toulouse], CHU Toulouse [Toulouse], Centre hospitalier de Saint-Nazaire, Clinique de médecine interne, Université Joseph Fourier - Grenoble 1 (UJF)-CHU Grenoble, Service de Médecine Interne [CHU Rouen], CHU Rouen, Normandie Université (NU)-Normandie Université (NU)-Université de Rouen Normandie (UNIROUEN), Hôpital l'Archet, Service des maladies infectieuses et tropicales [CHU Angers], Médecine Interne Dax (MEDECINE INTERNE), Hopital, Service de Médecine Interne, Hospices Civils de Lyon (HCL)-Hospices Civils de Lyon (HCL), Service de médecine interne [Lille], Centre Hospitalier Régional Universitaire [Lille] (CHRU Lille), Service de Pneumologie et Réanimation Médicale [CHU Pitié-Salpêtrière] (Département ' R3S '), CHU Pitié-Salpêtrière [AP-HP], Sorbonne Université (SU)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP), Service de médecine interne [CHU Pitié-Salpétrière], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-CHU Pitié-Salpêtrière [AP-HP], Department of Clinical Immunology, CHU Strasbourg, CHU Pontchaillou [Rennes], Service d'hématologie biologique, Service de néphrologie, Service de médecine interne et d'immunologie clinique [CHU Pitié-Salpêtrière], Service d'Hématologie-Oncologie, Hôpital Ste-Justine, Département d'Oncologie (Dep Oncol - AVIGNON), Institut Ste Catherine, Service de Néphrologie - Médecine Interne, CHU Amiens-Picardie-hôpital Sud, Service de Médecine Interne (SOC 1 et SOC 2) [CHU de Dijon], Centre Hospitalier Universitaire de Dijon - Hôpital François Mitterrand (CHU Dijon), CHU Henri Mondor, Service de Médecine Interne [Henri Mondor], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Hôpital Henri Mondor-Centre de référence maladie rare des cytopénies auto-immunes de l'adulte-Université Paris-Est Créteil Val-de-Marne - Paris 12 (UPEC UP12), Service de Dermatologie, Centre Hospitalier Régional Universitaire [Montpellier] (CHRU Montpellier), Service de dermatologie [Avicenne], Hôpital Avicenne [AP-HP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Université Paris 13 (UP13), Service de Dermatologie, Centre Hospitalier Sud, Hospices Civils, Lyon, Service de dermatologie (CHRU de Lille), Service de dermatologie [Rouen], Service de médecine interne, Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Hôpital Jean Verdier [AP-HP], Commissariat à l'énergie atomique et aux énergies alternatives - Laboratoire d'Electronique et de Technologie de l'Information (CEA-LETI), Direction de Recherche Technologique (CEA) (DRT (CEA)), Commissariat à l'énergie atomique et aux énergies alternatives (CEA)-Commissariat à l'énergie atomique et aux énergies alternatives (CEA), Université Paris Descartes - Faculté de Médecine (UPD5 Médecine), Université Paris Descartes - Paris 5 (UPD5), UNIROUEN - UFR Santé (UNIROUEN UFR Santé), Université de Rouen Normandie (UNIROUEN), Normandie Université (NU)-Normandie Université (NU), Equipe 12, Centre Hospitalier Universitaire de Nice (CHU Nice)-Hôpital l'Archet-Centre Hospitalier Universitaire de Nice (CHU Nice)-Hôpital l'Archet-Centre méditerranéen de médecine moléculaire (C3M), Université Nice Sophia Antipolis (... - 2019) (UNS), COMUE Université Côte d'Azur (2015-2019) (COMUE UCA)-COMUE Université Côte d'Azur (2015-2019) (COMUE UCA)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Université Côte d'Azur (UCA)-Université Nice Sophia Antipolis (... - 2019) (UNS), COMUE Université Côte d'Azur (2015-2019) (COMUE UCA)-COMUE Université Côte d'Azur (2015-2019) (COMUE UCA)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Université Côte d'Azur (UCA), Service de pneumologie [Avicenne], Service de médecine interne [Saint-Antoine], Université Pierre et Marie Curie - Paris 6 (UPMC)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-CHU Saint-Antoine [AP-HP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU)-Sorbonne Université (SU), Epidemiology in Dermatology and Evaluation in Therapeutics (EpiDermE), Université Paris-Est Créteil Val-de-Marne - Paris 12 (UPEC UP12), Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Université Paris Diderot - Paris 7 (UPD7), Unité de Recherche sur les Maladies Cardiovasculaires, du Métabolisme et de la Nutrition = Research Unit on Cardiovascular and Metabolic Diseases (ICAN), Centre de recherche en Myologie – U974 SU-INSERM, Institut National de la Santé et de la Recherche Médicale (INSERM)-Sorbonne Université (SU), Université Blaise Pascal - Clermont-Ferrand 2 (UBP)-Université d'Auvergne - Clermont-Ferrand I (UdA)-Centre National de la Recherche Scientifique (CNRS), Université de Caen Normandie (UNICAEN), Normandie Université (NU)-Normandie Université (NU)-CHU Caen, Normandie Université (NU)-Tumorothèque de Caen Basse-Normandie (TCBN)-Tumorothèque de Caen Basse-Normandie (TCBN), Service Médecine interne [CHU Toulouse], Pôle Inflammation, infection, immunologie et loco-moteur [CHU Toulouse] (Pôle I3LM Toulouse), Centre Hospitalier Universitaire de Toulouse (CHU Toulouse)-Centre Hospitalier Universitaire de Toulouse (CHU Toulouse), CHU Henri Mondor [Créteil], Université Paris 13 (UP13)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Hôpital Avicenne [AP-HP], Université Nice Sophia Antipolis (1965 - 2019) (UNS), COMUE Université Côte d'Azur (2015-2019) (COMUE UCA)-COMUE Université Côte d'Azur (2015-2019) (COMUE UCA)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Université Côte d'Azur (UCA)-Université Nice Sophia Antipolis (1965 - 2019) (UNS), Hôpital Ambroise Paré [AP-HP]-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP), Institut National de la Santé et de la Recherche Médicale (INSERM)-Centre National de la Recherche Scientifique (CNRS)-Université Paris Descartes - Paris 5 (UPD5), Institut National de la Santé et de la Recherche Médicale (INSERM)-IFR113-Université Pierre et Marie Curie - Paris 6 (UPMC), Service de Département de médecine interne et immunologie clinique [CHU Pitié-Salpêtrière] (DMIIC), COMUE Université Côte d'Azur (2015-2019) (COMUE UCA)-COMUE Université Côte d'Azur (2015-2019) (COMUE UCA)-Université Côte d'Azur (UCA)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Université Nice Sophia Antipolis (... - 2019) (UNS), COMUE Université Côte d'Azur (2015-2019) (COMUE UCA)-COMUE Université Côte d'Azur (2015-2019) (COMUE UCA)-Université Côte d'Azur (UCA)-Institut National de la Santé et de la Recherche Médicale (INSERM), and Service de médecine interne [CHU Saint-Antoine]
Subjects: [SDV.MHEP.RSOA]Life Sciences [q-bio]/Human health and pathology/Rhumatology and musculoskeletal system, [SDV]Life Sciences [q-bio], ComputingMilieux_MISCELLANEOUS, [SDV.MHEP]Life Sciences [q-bio]/Human health and pathology
Abstract: International audience
Published: 2017
Full Text: View/download PDF

13. Large α-aminonitrilase activity screening of nitrilase superfamily members: Access to conversion and enantiospecificity by LC–MS

Author: David Vallenet, Virginie Pellouin, Jean-Louis Petit, Jean Weissenbach, Marcel Salanoubat, Franck Bordier, Carine Vergne-Vaxelaire, Aurélie Fossey, Johanna Rohault, Adrien Debard, Véronique de Berardinis, Alain Perret, Ekaterina Darii, Sabine Tricot, Mark Stam, Aline Mariage, Anne Zaparucha, Génomique métabolique (UMR 8030), Genoscope - Centre national de séquençage [Evry] (GENOSCOPE), Direction de Recherche Fondamentale (CEA) (DRF (CEA)), Commissariat à l'énergie atomique et aux énergies alternatives (CEA)-Commissariat à l'énergie atomique et aux énergies alternatives (CEA)-Université Paris-Saclay-Direction de Recherche Fondamentale (CEA) (DRF (CEA)), Commissariat à l'énergie atomique et aux énergies alternatives (CEA)-Commissariat à l'énergie atomique et aux énergies alternatives (CEA)-Université Paris-Saclay-Centre National de la Recherche Scientifique (CNRS)-Université d'Évry-Val-d'Essonne (UEVE), CH Valenciennes, Structure et Instabilité des Génomes (STRING), Muséum national d'Histoire naturelle (MNHN)-Université Pierre et Marie Curie - Paris 6 (UPMC)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Centre National de la Recherche Scientifique (CNRS), Commissariat à l'énergie atomique et aux énergies alternatives (CEA)-Commissariat à l'énergie atomique et aux énergies alternatives (CEA)-Université Paris-Saclay, Institut de Chimie des Substances Naturelles (ICSN), Centre National de la Recherche Scientifique (CNRS), Université Paris-Saclay-Direction de Recherche Fondamentale (CEA) (DRF (CEA)), Commissariat à l'énergie atomique et aux énergies alternatives (CEA)-Commissariat à l'énergie atomique et aux énergies alternatives (CEA)-Université d'Évry-Val-d'Essonne (UEVE)-Centre National de la Recherche Scientifique (CNRS), Commissariat à l'énergie atomique et aux énergies alternatives (CEA)-Commissariat à l'énergie atomique et aux énergies alternatives (CEA)-Centre National de la Recherche Scientifique (CNRS)-Université d'Évry-Val-d'Essonne (UEVE), Muséum national d'Histoire naturelle (MNHN)-Université Pierre et Marie Curie - Paris 6 (UPMC)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Institut de Chimie du CNRS (INC)-Centre National de la Recherche Scientifique (CNRS), Commissariat à l'énergie atomique et aux énergies alternatives (CEA)-Commissariat à l'énergie atomique et aux énergies alternatives (CEA), and Centre National de la Recherche Scientifique (CNRS)-Institut de Chimie du CNRS (INC)
Subjects: chemistry.chemical_classification, [SDV.BIO]Life Sciences [q-bio]/Biotechnology, biology, Chemistry, Stereochemistry, Process Chemistry and Technology, Burkholderia xenovorans, Substrate (chemistry), Bioengineering, SUPERFAMILY, [CHIM.CATA]Chemical Sciences/Catalysis, biology.organism_classification, Biochemistry, Nitrilase, Catalysis, Hydrolysis, Enzyme, Liquid chromatography–mass spectrometry, [CHIM]Chemical Sciences, Enantiomeric excess, ComputingMilieux_MISCELLANEOUS
Abstract: A high-throughput screening for the identification of nitrilases demonstrating activity towards alpha-aminonitriles is reported. A LC–MS assay giving access to both conversion and enantiospecificity was developed. 588 candidate enzymes were screened as cell lysates against six alpha-aminonitriles in 96-well microplates. The candidate enzymes were selected following two criteria, their sequence identity with a set of known nitrilases or their phylogenetic position among the nitrilase superfamily. Five enzymes were identified and found to hydrolyse alpha-aminonitrile into the corresponding alpha-aminoacid. The substrate range was found to be very narrow as only two different alpha-aminonitriles, 2-aminovaleronitrile and 2-amino-2-phenylacetonitrile, were found to be substrates. The biocatalytic capabilities of three enzymes were further investigated and the best result was obtained with an enzyme from Burkholderia xenovorans catalysing the enantiospecific hydrolysis of 2-aminovaleronitrile into (S)-norvaline with excellent conversion and enantiomeric excess.
Published: 2014
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14. Grams ME, Sang Y, Ballew SH, et al, for the Chronic Kidney Disease Prognosis Consortium. Predicting timing of clinical outcomes in patients with chronic kidney disease and severely decreased glomerular filtration rate. Kidney Int. 2018;93:1442–1451

Author: Remy, Philippe, Audard, Vincent, Natella, Pierre André, Pelle, Gaëlle, Dussol, Bertrand, Leray-Moragues, Hélène, Bouachi, Khedidja, Dantal, Jacques, Vrigneaud, Laurence, Karras, Alexandre, Pourcine, Frank, Gatault, Philippe, Grimbert, Philippe, Ait Sahlia, Nawelle, Moktefi, Anissa, Daugas, Eric, Rigothier, Claire, Bastuji-Garin, Sylvie, Sahali, Dil, Aldigier, Jean Claude, Bataille, Pierre, Canaud, Bernard, Chauveau, Dominique, Combe, Christian, Choukroun, Gabriel, Cornec-Legall, Emilie, Dahan, Karine, Delahousse, Michel, Desvaux, Dominique, Deteix, Patrice, Durrbach, Antoine, Esnault, Vincent, Essig, Marie, Fievet, Patrick, Frouget, Thierry, Guerrot, Dominique, Godin, Michel, Gontiers-Picard, Annie, Gosselin, Morgane, Hanrotel-Saliou, Catherine, Heng, Anne-Elisabeth, Huart, Antoine, Humbert, Antoine, Kofman, Tomek, Hummel, Aurélie, Lang, Philippe, Laville, Maurice, Lemeur, Yannick, Malvezzi, Paolo, Matignon, Marie, Mesbah, Rafik, Moulin, Bruno, Muller, Sandrine, Olagne, Jerome, Pardon, Agathe, Provôt, François, Queffeulou, Guillaume, Plaisier, Emmanuelle, Raimbourg, Quentin, Rieu, Philippe, Stehlé, Thomas, Vanhille, Philippe, Astor, Brad, Levin, Adeera, Tang, Mila, Djurdjev, Ognjenka, Navaneethan, Sankar, Jolly, Stacey, Schold, Jesse, Nally, Joseph, Emberson, Jonathan, Townend, John, Landray, Martin, Feldman, Harold, Hsu, Chi-Yuan, Lash, James, Appel, Lawrence, Kalra, Philip, Ritchie, James, Maharajan, Raman, Middleton, Rachel, O’donoghue, Donal, Schneider, Markus, Kronenberg, Florian, Bärthlein, Barbara, Green, Jamie, Kirchner, H. Lester, Ho, Kevin, Marks, Angharad, Black, Corri, Prescott, Gordon, Fluck, Nick, Nakayama, Masaaki, Miyazaki, Mariko, Yamamoto, Tae, Yamada, Gen, Wang, Angela Yee-Moon, Cheung, Sharon, Wong, Sharon, Chu, Jessie, Wu, Henry, Garg, Amit, Mcarthur, Eric, Nash, Danielle, Shalev, Varda, Chodick, Gabriel, Blankestijn, Peter, Wetzels, Jack F.M., van Zuilen, Arjan, van den Brand, Jan, Inker, Lesley, Sarnak, Mark, Tighiouart, Hocine, Zhang, Haitao, Stengel, Bénédicte, Metzger, Marie, Flamant, Martin, Houillier, Pascal, Haymann, Jean-Philippe, Rios, Pablo, Mazzuchi, Nelson, Gadola, Liliana, Lamadrid, Verónica, Solá, Laura, Collins, John, Elley, C. Raina, Kenealy, Timothy, Moranne, Olivier, Couchoud, Cécile, Vigneau, Cécile, Brunskill, Nigel, Major, Rupert, Shepherd, David, Medcalf, James, Kovesdy, Csaba, Kalantar-Zadeh, Kamyar, Sumida, Keiichi, Potukuchi, Praveen, Heerspink, Hiddo J.L., de Zeeuw, Dick, Brenner, Barry, Carrero, Juan Jesus, Gasparini, Alessandro, Qureshi, Abdul Rashid, Elinder, Carl-Gustaf, Visseren, Frank L.J., van Der Graaf, Yolanda, Evans, Marie, Stendahl, Maria, Schön, Staffan, Segelmark, Mårten, Prütz, Karl-Göran, Naimark, David, Tangri, Navdeep, Mark, Patrick, Traynor, Jamie, Geddes, Colin, Thomson, Peter, Chang, Alex, Gansevoort, Ron, Köttgen, Anna, Levey, Andrew, Zhang, Luxia, Ballew, Shoshana, Chen, Jingsha, Coresh, Josef, Grams, Morgan, Kwak, Lucia, Matsushita, Kunihiro, Sang, Yingying, Surapaneni, Aditya, Woodward, Mark, Eckardt, Kai-Uwe, Hemmelgarn, Brenda, Wheeler, David, Winkelmayer, Wolfgang, Davis, John, Green, Danielle, Cheung, Michael, Green, Tanya, Mcmahan, Melissa, Molnar, Miklos, Service de néphrologie [CHU Henri Mondor], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-CHU Henri Mondor, Assistance Publique-Hôpitaux de Paris, Department of Pathology, Centre hospitalier universitaire Henri Mondor, F-94000 Créteil, France, Department of Nephrology and Transplantation, Foch Hospital, Chirurgie urologique et transplantation rénale [Hôpital de la Conception - APHM], Aix Marseille Université (AMU)-Assistance Publique - Hôpitaux de Marseille (APHM)-Hôpital de la Conception [CHU - APHM] (LA CONCEPTION), Centre recherche en CardioVasculaire et Nutrition = Center for CardioVascular and Nutrition research (C2VN), Aix Marseille Université (AMU)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Institut National de Recherche pour l’Agriculture, l’Alimentation et l’Environnement (INRAE), Centre Hospitalier Régional Universitaire [Montpellier] (CHRU Montpellier), Service de néphrologie et transplantation, Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Hôpital Henri Mondor-Université Paris-Est Créteil Val-de-Marne - Paris 12 (UPEC UP12), Institut Transplantation Urologie et Néphrologie, Centre hospitalier universitaire de Nantes (CHU Nantes), Service de Néphrologie [Valenciennes], Centre Hospitalier de Valenciennes, Hôpital Européen Georges Pompidou [APHP] (HEGP), Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Hôpitaux Universitaires Paris Ouest - Hôpitaux Universitaires Île de France Ouest (HUPO), Service de Néphrologie CHU TOURS, INSERM U955, équipe 21, Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Hôpital Henri Mondor-Université Paris-Est Créteil Val-de-Marne - Paris 12 (UPEC UP12)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Hôpital Henri Mondor-Université Paris-Est Créteil Val-de-Marne - Paris 12 (UPEC UP12)-Institut Mondor de Recherche Biomédicale (IMRB), Institut National de la Santé et de la Recherche Médicale (INSERM)-IFR10-Université Paris-Est Créteil Val-de-Marne - Paris 12 (UPEC UP12)-Institut National de la Santé et de la Recherche Médicale (INSERM)-IFR10-Université Paris-Est Créteil Val-de-Marne - Paris 12 (UPEC UP12), UMR 1599, Centre National de la Recherche Scientifique (CNRS), Department of Nephrology, Transplantation and Dialysis, Bordeaux, Laboratoire d'Investigation Clinique (LIC), Université Paris-Est Créteil Val-de-Marne - Paris 12 (UPEC UP12), Hôpital Henri Mondor, Université de Lausanne = University of Lausanne (UNIL), Service de Néphrologie et Immunopathologie Clinique, Centre Hospitalier Universitaire de Toulouse, PRES Université de Toulouse, CHU Bordeaux [Bordeaux], Unité Médicale de Soins Intensifs, CHU Amiens-Picardie, CHRU Brest - Service de Nephrologie (CHU - BREST - Nephrologie), Centre Hospitalier Régional Universitaire de Brest (CHRU Brest), Service de Département de Néphrologie = Service de Néphrologie et Dialyses [CHU Tenon], CHU Tenon [AP-HP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU), Service de transplantation rénale, Hôpital Foch [Suresnes], Institut Mondor de Recherche Biomédicale (IMRB), Institut National de la Santé et de la Recherche Médicale (INSERM)-IFR10-Université Paris-Est Créteil Val-de-Marne - Paris 12 (UPEC UP12), Service Néphrologie, Pôle REUNNIRH, CHU Clermont-Ferrand, U542, Institut National de la Santé et de la Recherche Médicale (INSERM), Service de néphrologie, Centre Hospitalier Universitaire de Nice (CHU Nice), Service de Néphrologie, Dialyse, Transplantations [CHU Limoges], CHU Limoges, Univers, Transport, Interfaces, Nanostructures, Atmosphère et environnement, Molécules (UMR 6213) (UTINAM), Institut national des sciences de l'Univers (INSU - CNRS)-Centre National de la Recherche Scientifique (CNRS)-Université de Franche-Comté (UFC), Université Bourgogne Franche-Comté [COMUE] (UBFC)-Université Bourgogne Franche-Comté [COMUE] (UBFC), CHU Pontchaillou [Rennes], Service de Néphrologie [Rouen], CHU Rouen, Normandie Université (NU)-Normandie Université (NU)-Université de Rouen Normandie (UNIROUEN), Normandie Université (NU), Nouvelles Cibles Pharmacologiques de la Protection Endothéliale et de l'Insuffisance Cardiaque (EnVI), Normandie Université (NU)-Institut National de la Santé et de la Recherche Médicale (INSERM), Service de Néphrologie, Centre Hospitalier Régional Universitaire de Brest (CHRU Brest)-Hôpital de la Cavale Blanche - CHRU Brest (CHU - BREST ), Unité de Nutrition Humaine (UNH), Institut National de la Recherche Agronomique (INRA)-Université Clermont Auvergne [2017-2020] (UCA [2017-2020]), CHU Necker - Enfants Malades [AP-HP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP), Centre Hospitalier Lyon Sud [CHU - HCL] (CHLS), Hospices Civils de Lyon (HCL)-Hospices Civils de Lyon (HCL), Service de néphrologie, dialyse, aphérèses et transplantation, CHU Grenoble-Université Grenoble Alpes [2016-2019] (UGA [2016-2019]), CH Boulogne sur Mer, Service de Néphrologie et Transplantation, CHU Strasbourg, CHU Saint-Antoine [AP-HP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU), Service de Néphrologie [CHPC - Site Louis Pasteur], Site Louis Pasteur [CHPC], CH Centre Hospitalier Public du Cotentin (CHPC)-CH Centre Hospitalier Public du Cotentin (CHPC), Remodelage et Reparation du Tissu Renal, Université Pierre et Marie Curie - Paris 6 (UPMC)-Institut National de la Santé et de la Recherche Médicale (INSERM), Service de Néphrologie [Bichat - Claude Bernard], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-AP-HP - Hôpital Bichat - Claude Bernard [Paris], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Université Paris Diderot - Paris 7 (UPD7), Centre Hospitalier Universitaire de Reims (CHU Reims), Université Paris Descartes - Faculté de Médecine (UPD5 Médecine), Université Paris Descartes - Paris 5 (UPD5), Service de médecine interne et néphrologie, CH Valenciennes, Division of Nephrology [Vancouver, BC, Canada], University of British Columbia (UBC), Aberdeen University, Fraunhofer Institute for Secure Information Technology [Darmstadt] (Fraunhofer SIT), Fraunhofer (Fraunhofer-Gesellschaft), Division of genetic epidemiology, HMNC Brain Health-Molecular and Clinical Pharmacology-Innsbruck Medical University [Austria] (IMU), Department of electronic engineering, Chang Gung University, Institute of Applied Health Sciences, University of Aberdeen, Department of Medicine, The University of Hong Kong (HKU), Department of Nephrology [Utrecht, Pays-Bas], University Medical Center [Utrecht], Department of Nephrology [Nijmegen, The Netherlands], Radboud University Medical Center [Nijmegen], Department of nephrology [Nimègue, Pays-Bas], Radboud Institute for Health Sciences [Nimègue, Pays-Bas], Radboud University Medical Center [Nijmegen]-Radboud University Medical Center [Nijmegen], Tufts Medical Center, Nanjing University of Aeronautics and Astronautics [Nanjing] (NUAA), Centre de recherche en épidémiologie et santé des populations (CESP), Université de Versailles Saint-Quentin-en-Yvelines (UVSQ)-Université Paris-Sud - Paris 11 (UP11)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Hôpital Paul Brousse-Institut National de la Santé et de la Recherche Médicale (INSERM), Service de Physiologie [Bichat-Claude Bernard], Université Pierre et Marie Curie - Paris 6 (UPMC)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-AP-HP - Hôpital Bichat - Claude Bernard [Paris], Service de Physiologie [Georges-Pompidou], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Hôpital Européen Georges Pompidou [APHP] (HEGP), Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Hôpitaux Universitaires Paris Ouest - Hôpitaux Universitaires Île de France Ouest (HUPO)-Hôpitaux Universitaires Paris Ouest - Hôpitaux Universitaires Île de France Ouest (HUPO)-Université Paris Descartes - Paris 5 (UPD5), Des Maladies Rénales Rares aux Maladies Fréquentes, Remodelage et Réparation, Center for Research in Agricultural Genomics, Directora Division Epidemiologia [Montevideo, Uruguay], Ministerio de Salud Publica, Hôpital Pasteur [Nice] (CHU)-Centre Hospitalier Universitaire de Nice (CHU Nice), Agence de la biomédecine [Saint-Denis la Plaine], Institut de recherche en santé, environnement et travail (Irset), Université d'Angers (UA)-Université de Rennes 1 (UR1), Université de Rennes (UNIV-RENNES)-Université de Rennes (UNIV-RENNES)-École des Hautes Études en Santé Publique [EHESP] (EHESP)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Structure Fédérative de Recherche en Biologie et Santé de Rennes ( Biosit : Biologie - Santé - Innovation Technologique ), Division of Nephrology, Department of Medicine, Vanderbilt University School of Medicine [Nashville], Divisions of Renal Medicine and Baxter Novum, Department of Clinical Science, Intervention and Technology, Karolinska Institutet [Stockholm], Julius Center for Health Sciences and Primary Care, Division of Drug Research/Clinical Pharmacology, Department of Medicine and Health, Faculty of Health Sciences, Linköping University (LIU), Department of Nephrology [Groningue, Pays-Bas], University Medical Center Groningen [Groningen] (UMCG), Renal Division [Beijing, China], Peking University [Beijing], The Georges Institute for International Health, The University of Sydney, Department of Nephrology and Hypertension [Erlangen, Germany], Friedrich-Alexander Universität Erlangen-Nürnberg (FAU), Baylor College of Medicine (BCM), Baylor University, Université de Lausanne (UNIL), Service de Néphrologie et Dialyses [CHU Tenon], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-CHU Tenon [AP-HP], Sorbonne Université (SU)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU), Institut National de la Santé et de la Recherche Médicale (INSERM)-Université de Rouen Normandie (UNIROUEN), Normandie Université (NU)-Normandie Université (NU)-CHU Rouen, Université Joseph Fourier - Grenoble 1 (UJF)-CHU Grenoble, Service de néphrologie [CHPC, Cherbourg-en-Cotentin], CHPC - Site Louis Pasteur, Centre Hospitalier Public du Cotentin (CHPC)-Centre Hospitalier Public du Cotentin (CHPC), Innsbruck Medical University [Austria] (IMU)-HMNC Brain Health-Molecular and Clinical Pharmacology, Radboud University Medical Centre [Nijmegen, The Netherlands], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Université Paris-Sud - Paris 11 (UP11)-Hôpital Paul Brousse-Institut National de la Santé et de la Recherche Médicale (INSERM)-Université de Versailles Saint-Quentin-en-Yvelines (UVSQ), Structure Fédérative de Recherche en Biologie et Santé de Rennes ( Biosit : Biologie - Santé - Innovation Technologique )-Institut National de la Santé et de la Recherche Médicale (INSERM)-École des Hautes Études en Santé Publique [EHESP] (EHESP)-Université de Rennes 1 (UR1), Université de Rennes (UNIV-RENNES)-Université de Rennes (UNIV-RENNES)-Université d'Angers (UA), Peking University First Hospital [China]-Peking University Institute of Nephrology [China], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-CHU Henri Mondor [Créteil], Service Médecine interne et immunopathologie clinique [CHU Toulouse], Pôle IUCT [CHU Toulouse], Centre Hospitalier Universitaire de Toulouse (CHU Toulouse)-Centre Hospitalier Universitaire de Toulouse (CHU Toulouse), HMNC Brain Health-Molecular and Clinical Pharmacology-Innsbruck Medical University = Medizinische Universität Innsbruck (IMU), Université d'Angers (UA)-Université de Rennes (UR)-École des Hautes Études en Santé Publique [EHESP] (EHESP)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Structure Fédérative de Recherche en Biologie et Santé de Rennes ( Biosit : Biologie - Santé - Innovation Technologique ), Assistance publique - Hôpitaux de Paris (AP-HP) (APHP)-CHU Henri Mondor, Aix Marseille Université (AMU)-Assistance Publique - Hôpitaux de Marseille (APHM)-Hôpital de la Conception [CHU - APHM] (LA CONCEPTION ), Assistance publique - Hôpitaux de Paris (AP-HP) (APHP)-Hôpital Henri Mondor-Université Paris-Est Créteil Val-de-Marne - Paris 12 (UPEC UP12), Assistance publique - Hôpitaux de Paris (AP-HP) (APHP)-Hôpitaux Universitaires Paris Ouest - Hôpitaux Universitaires Île de France Ouest (HUPO), Assistance publique - Hôpitaux de Paris (AP-HP) (APHP)-Hôpital Henri Mondor-Université Paris-Est Créteil Val-de-Marne - Paris 12 (UPEC UP12)-Assistance publique - Hôpitaux de Paris (AP-HP) (APHP)-Hôpital Henri Mondor-Université Paris-Est Créteil Val-de-Marne - Paris 12 (UPEC UP12)-Institut Mondor de Recherche Biomédicale (IMRB), Assistance publique - Hôpitaux de Paris (AP-HP) (APHP)-CHU Tenon [APHP], Centre Hospitalier Universitaire de Clermont-Ferrand, Université de Rouen Normandie (UNIROUEN), Unité de Nutrition Humaine - Clermont Auvergne (UNH), Institut National de la Recherche Agronomique (INRA)-Université Clermont Auvergne (UCA), Service de Néphrologie, Dialyse, Aphérèses et Transplantation [CHU Grenoble], Centre Hospitalier Universitaire [Grenoble] (CHU), CHU Saint-Antoine [APHP], Centre Hospitalier du Cotentin, Assistance publique - Hôpitaux de Paris (AP-HP) (APHP)-AP-HP - Hôpital Bichat - Claude Bernard [Paris]-Université Paris Diderot - Paris 7 (UPD7), Fraunhofer Institute for Secure Information Technology [Darmstadt] (SIT), Université de Versailles Saint-Quentin-en-Yvelines (UVSQ)-Université Paris-Sud - Paris 11 (UP11)-Assistance publique - Hôpitaux de Paris (AP-HP) (APHP)-Hôpital Paul Brousse-Institut National de la Santé et de la Recherche Médicale (INSERM), Université Pierre et Marie Curie - Paris 6 (UPMC)-Assistance publique - Hôpitaux de Paris (AP-HP) (APHP)-AP-HP - Hôpital Bichat - Claude Bernard [Paris], Assistance publique - Hôpitaux de Paris (AP-HP) (APHP)-Hôpital Européen Georges Pompidou [APHP] (HEGP), Assistance publique - Hôpitaux de Paris (AP-HP) (APHP)-Hôpitaux Universitaires Paris Ouest - Hôpitaux Universitaires Île de France Ouest (HUPO)-Hôpitaux Universitaires Paris Ouest - Hôpitaux Universitaires Île de France Ouest (HUPO)-Université Paris Descartes - Paris 5 (UPD5), Institut National de la Santé et de la Recherche Médicale (INSERM)-Université Pierre et Marie Curie - Paris 6 (UPMC), Méthodes Algorithmes pour l'Ordonnancement et les Réseaux (MAORE), Laboratoire d'Informatique de Robotique et de Microélectronique de Montpellier (LIRMM), and Université de Montpellier (UM)-Centre National de la Recherche Scientifique (CNRS)-Université de Montpellier (UM)-Centre National de la Recherche Scientifique (CNRS)
Subjects: Nephrology, medicine.medical_specialty, [SDV]Life Sciences [q-bio], media_common.quotation_subject, 030232 urology & nephrology, 03 medical and health sciences, 0302 clinical medicine, Internal medicine, medicine, In patient, 030212 general & internal medicine, mycophenolate, media_common, clinical trial, Art, medicine.disease, Optimal management, Treatment efficacy, 3. Good health, Transplantation, Cohort, idiopathic nephrotic syndrome, Humanities, [SDV.MHEP]Life Sciences [q-bio]/Human health and pathology, steroids, Kidney disease, Cohort study
Abstract: The Chronic Kidney Disease (CKD) Prognosis Consortium is a collaborative author of the above-mentioned article. The CKD Prognosis Consortium investigators/collaborators are as follows: • African American Study of Kidney Disease and Hypertension (AASK): Brad Astor, Lawrence J. Appel; Canadian Study of Prediction of Death, Dialysis and Interim Cardiovascular Events (CanPREDDICT): Adeera Levin, Mila Tang, Ognjenka Djurdjev; Cleveland Clinic CKD Registry Study (CCF): Sankar D. Navaneethan, Stacey E. Jolly, Jesse D. Schold, Joseph V. Nally Jr.; Chronic Renal Impairment in Birmingham (CRIB): David C. Wheeler, Jonathan Emberson, John Townend, Martin Landray; Chronic Renal Insufficiency Cohort Study (CRIC): Harold I. Feldman, Chi-yuan Hsu, James P. Lash, Lawrence J. Appel; Chronic Renal Insufficiency Standards Implementation Study (CRISIS): Philip A. Kalra, James P. Ritchie, Raman Maharajan, Rachel J. Middleton, Donal J. O'Donoghue; German Chronic Kidney Disease Study (GCKD): Kai-Uwe Eckardt, Markus P. Schneider, Anna Kottgen, Florian Kronenberg, Barbara Barthlein; Geisinger Health System: Alex R. Chang, Jamie A. Green, H. Lester Kirchner, Kevin Ho; Grampian Laboratory Outcomes, Morbidity and Mortality Studies – 2 (GLOMMS2): Angharad Marks, Corri Black, Gordon J. Prescott, Nick Fluck; Gonryo Study: Masaaki Nakayama, Mariko Miyazaki, Tae Yamamoto, Gen Yamada; Hong Kong CKD Studies: Angela Yee-Moon Wang, Sharon Cheung, Sharon Wong, Jessie Chu, Henry Wu; Ontario Institute for Clinical Evaluative Sciences, Provincial Kidney, Dialysis and Transplantation program (ICES KDT): Amit X. Garg, Eric McArthur, Danielle M. Nash; Maccabi Health System: Varda Shalev, Gabriel Chodick; Multifactorial Approach and Superior Treatment Efficacy in Renal Patients with the Aid of a Nurse Practitioner (MASTERPLAN): Peter J. Blankestijn, Jack F.M. Wetzels, Arjan D. van Zuilen, Jan A. van den Brand; Modification of Diet in Renal Disease Study (MDRD): Andrew S. Levey, Lesley A. Inker, Mark J. Sarnak, Hocine Tighiouart; Nanjing CKD Network Cohort Study (Nanjing CKD): Haitao Zhang; NephroTest Study (NephroTest): Benedicte Stengel, Marie Metzger, Martin Flamant, Pascal Houillier, Jean-Philippe Haymann; National Renal Healthcare Program – Uruguay (NRHP-URU): Pablo G. Rios, Nelson Mazzuchi, Liliana Gadola, Veronica Lamadrid, Laura Sola; New Zealand Diabetes Cohort Study (NZDCS): John F. Collins, C. Raina Elley, Timothy Kenealy; Parcours de Soins des Personnes Agees (PSPA): Olivier Moranne, Cecile Couchoud, Cecile Vigneau; Primary-Secondary Care Partnership to Prevent Adverse Outcomes in Chronic Kidney Disease (PSP CKD): Nigel J. Brunskill, Rupert W. Major, David Shepherd, James F. Medcalf; Racial and Cardiovascular Risk Anomalies in CKD Cohort (RCAV): Csaba P. Kovesdy, Kamyar Kalantar-Zadeh, Miklos Z. Molnar, Keiichi Sumida, Praveen K. Potukuchi; Reduction of Endpoints in Non-insulin Dependent Diabetes Mellitus with the Angiotensin II Antagonist Losartan (RENAAL): Hiddo J.L. Heerspink, Dick de Zeeuw, Barry Brenner; Stockholm CREAtinine Measurements Cohort (SCREAM): Juan Jesus Carrero, Alessandro Gasparini, Abdul Rashid Qureshi, Carl-Gustaf Elinder; Second Manifestations of ARTerial Disease Study (SMART): Frank L.J. Visseren, Yolanda van der Graaf; Swedish Renal Registry CKD Cohort (SRR CKD): Marie Evans, Maria Stendahl, Staffan Schon, Marten Segelmark, Karl-Goran Prutz; Sunnybrook Cohort: David M. Naimark, Navdeep Tangri; West of Scotland CKD Study: Patrick B. Mark, Jamie P. Traynor, Colin C. Geddes, Peter C. Thomson.• CKD Prognosis Consortium Steering Committee: Alex R. Chang, Josef Coresh (Chair), Ron T. Gansevoort, Morgan E. Grams, Anna Kottgen, Andrew S. Levey, Kunihiro Matsushita, Mark Woodward, Luxia Zhang.• CKD Prognosis Consortium Data Coordinating Center: Shoshana H. Ballew (Assistant Project Director), Jingsha Chen (Programmer), Josef Coresh (Principal Investigator), Morgan E. Grams (Director of Nephrology Initiatives), Lucia Kwak (Programmer), Kunihiro Matsushita (Director), Yingying Sang (Lead Programmer), Aditya Surapaneni (Programmer), Mark Woodward (Senior Statistician).• Kidney Disease Improving Global Outcomes (KDIGO) Controversies Conference on Prognosis and Optimal Management of Patients with Advanced CKD: Kai-Uwe Eckardt (Conference Co-Chair), Brenda R. Hemmelgarn (Conference Co-Chair), David C. Wheeler (KDIGO Co-Chair), Wolfgang C. Winkelmayer (KDIGO Co-Chair), John Davis (CEO), Danielle Green (Managing Director), Michael Cheung (Chief Scientific Officer), Tanya Green (Communications Director), Melissa McMahan (Programs Director).
Published: 2018
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15. Characteristics and Management of IgA Vasculitis (Henoch-Schonlein) in Adults Data From 260 Patients Included in a French Multicenter Retrospective Survey

Author: Audemard-Verger, Alexandra, Terrier, Benjamin, Dechartres, Agnès, Chanal, Johan, Amoura, Zahir, Le Gouellec, Noémie, Cacoub, Patrice, Jourde-Chiche, Noemie, Urbanski, Geoffrey, Augusto, François, Moulis, Guillaume, Raffray, Loïc, Deroux, Alban, Hummel, Aurélie, Lioger, Bertrand, Catroux, Melanie, Faguer, Stanislas, Goutte, Julie, Martis, Nihal, Maurier, François, Rivière, Etienne, Sanges, Sébastien, Baldolli, Aurélie, Costedoat-Chalumeau, Nathalie, Roriz, Mélanie, Puéchal, Xavier, André, Marc, Lavigne, Christian, Bienvenu, Boris, Mekinian, Arsene, Zagdoun, Elie, Girard, Charlotte, Bérezné, Alice, Guillevin, Loïc, Thervet, Eric, Pillebout, Evangeline, CHU Cochin [AP-HP], Immunologie - Immunopathologie - Immunothérapeutique ( I3 ), Université Pierre et Marie Curie - Paris 6 ( UPMC ) -Institut National de la Santé et de la Recherche Médicale ( INSERM ) -Centre National de la Recherche Scientifique ( CNRS ), Centre d'Epidémiologie Clinique, Assistance publique - Hôpitaux de Paris (AP-HP)-Hôtel-Dieu-Université Paris Descartes - Paris 5 ( UPD5 ) -PRES Sorbonne Paris Cité-French Cochrane Centre, Institut Cochin ( UM3 (UMR 8104 / U1016) ), Université Paris Descartes - Paris 5 ( UPD5 ) -Institut National de la Santé et de la Recherche Médicale ( INSERM ) -Centre National de la Recherche Scientifique ( CNRS ), Service d'immunologie [CHU Pitié-Salpétrière], Assistance publique - Hôpitaux de Paris (AP-HP)-CHU Pitié-Salpêtrière [APHP], CH Valenciennes, Immunologie - Immunopathologie - Immunothérapie ( I3 ), Aix Marseille Université ( AMU ), Vascular research center of Marseille ( VRCM ), Aix Marseille Université ( AMU ) -Institut National de la Santé et de la Recherche Médicale ( INSERM ), CHU Angers, CHU Toulouse [Toulouse], Processus Infectieux en Milieu Insulaire Tropical ( PIMIT ), Centre National de la Recherche Scientifique ( CNRS ) -IRD-Institut National de la Santé et de la Recherche Médicale ( INSERM ) -Université de la Réunion ( UR ), Clinique de médecine interne, Université Joseph Fourier - Grenoble 1 ( UJF ) -CHU Grenoble, CHU Necker - Enfants Malades [AP-HP], Service de Médecine Interne, Centre Hospitalier Régional Universitaire de Tours ( CHRU TOURS ), CHU de Poitiers, Département de Néphrologie et Transplantation d'organes, CHU Toulouse [Toulouse]-Hôpital de Rangueil, Centre Hospitalier Universitaire de Saint-Etienne ( CHU de Saint-Etienne ), Université Nice Sophia Antipolis - Faculté de Médecine ( UNS UFR Médecine ), Université Nice Sophia Antipolis ( UNS ), Université Côte d'Azur ( UCA ) -Université Côte d'Azur ( UCA ), Service de médecine interne, Hôpital Sainte-Blandine-Centre hospitalier régional Metz-Thionville ( CHR Metz-Thionville ), Université de Neuchâtel, Centre Hospitalier Universitaire de Lille ( CHU de Lille ), CHU Caen, Nutrition, obésité et risque thrombotique ( NORT ), Institut National de la Recherche Agronomique ( INRA ) -Aix Marseille Université ( AMU ) -Institut National de la Santé et de la Recherche Médicale ( INSERM ), Centre Hospitalier Universitaire de Caen, Service de rhumatologie, inflammation-immunopathologie- biothérapie [CHU Saint-Antoine] ( DHU i2B ), Assistance publique - Hôpitaux de Paris (AP-HP)-CHU Saint-Antoine [APHP], UFR des Sciences et Technologies, Université de la Réunion ( UR ), Service de médecine interne et centre de référence des maladies rares [CHU Cochin], Assistance publique - Hôpitaux de Paris (AP-HP)-CHU Cochin [AP-HP], Service de néphrologie adultes [CHU Necker], Assistance publique - Hôpitaux de Paris (AP-HP)-CHU Necker - Enfants Malades [AP-HP], Service de rhumatologie, CHU Bordeaux [Bordeaux], Hôpital Cochin [AP-HP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP), Immunologie - Immunopathologie - Immunothérapeutique (I3), Université Pierre et Marie Curie - Paris 6 (UPMC)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Centre National de la Recherche Scientifique (CNRS), Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Hôtel-Dieu-Université Paris Descartes - Paris 5 (UPD5)-PRES Sorbonne Paris Cité-French Cochrane Centre, Institut Cochin (IC UM3 (UMR 8104 / U1016)), Université Paris Descartes - Paris 5 (UPD5)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Centre National de la Recherche Scientifique (CNRS), Service d'Immunologie [CHU Pitié-Salpétrière], CHU Pitié-Salpêtrière [AP-HP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU), Immunologie - Immunopathologie - Immunothérapie (I3), Aix Marseille Université (AMU), Vascular research center of Marseille (VRCM), Aix Marseille Université (AMU)-Institut National de la Santé et de la Recherche Médicale (INSERM), Centre Hospitalier Universitaire d'Angers (CHU Angers), PRES Université Nantes Angers Le Mans (UNAM), Centre Hospitalier Universitaire de Toulouse (CHU Toulouse), Processus Infectieux en Milieu Insulaire Tropical (PIMIT), Université de La Réunion (UR)-Institut National de la Santé et de la Recherche Médicale (INSERM)-IRD-Centre National de la Recherche Scientifique (CNRS), Université Joseph Fourier - Grenoble 1 (UJF)-CHU Grenoble, Centre hospitalier universitaire de Poitiers (CHU Poitiers), Département de Néphrologie et Transplantation d'organes [CHU Toulouse], Pôle Urologie - Néphrologie - Dialyse - Transplantations - Brûlés - Chirurgie plastique - Explorations fonctionnelles et physiologiques [CHU Toulouse], Centre Hospitalier Universitaire de Toulouse (CHU Toulouse)-Centre Hospitalier Universitaire de Toulouse (CHU Toulouse), Centre Hospitalier Universitaire de Saint-Etienne (CHU de Saint-Etienne), Université Nice Sophia Antipolis - Faculté de Médecine (UNS UFR Médecine), Université Nice Sophia Antipolis (1965 - 2019) (UNS), COMUE Université Côte d'Azur (2015-2019) (COMUE UCA)-COMUE Université Côte d'Azur (2015-2019) (COMUE UCA), Hôpital Sainte-Blandine-Centre hospitalier régional Metz-Thionville (CHR Metz-Thionville), Université de Neuchâtel (UNINE), Centre Hospitalier Universitaire de Lille (CHU de Lille), Normandie Université (NU)-Tumorothèque de Caen Basse-Normandie (TCBN), Centre Hospitalier Régional Universitaire de Tours (CHRU Tours), Nutrition, obésité et risque thrombotique (NORT), Institut National de la Recherche Agronomique (INRA)-Aix Marseille Université (AMU)-Institut National de la Santé et de la Recherche Médicale (INSERM), Service de rhumatologie [CHU Saint-Antoine], CHU Saint-Antoine [AP-HP], Université de La Réunion (UR), Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Hôpital Cochin [AP-HP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-CHU Necker - Enfants Malades [AP-HP], DIGNAT-GEORGE, Françoise, Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU), Centre Hospitalier Universitaire de Saint-Etienne [CHU Saint-Etienne] (CHU ST-E), Assistance publique - Hôpitaux de Paris (AP-HP) (APHP)-Hôtel-Dieu-Université Paris Descartes - Paris 5 (UPD5)-PRES Sorbonne Paris Cité-French Cochrane Centre, Assistance publique - Hôpitaux de Paris (AP-HP) (APHP)-CHU Pitié-Salpêtrière [APHP], Centre Hospitalier Régional Universitaire de Tours (CHRU TOURS), Hôpital de Rangueil, CHU Toulouse [Toulouse]-CHU Toulouse [Toulouse], Université Nice Sophia Antipolis (... - 2019) (UNS), Université Côte d'Azur (UCA)-Université Côte d'Azur (UCA), Aix Marseille Université (AMU)-Institut National de la Recherche Agronomique (INRA)-Institut National de la Santé et de la Recherche Médicale (INSERM), Service de rhumatologie, inflammation-immunopathologie- biothérapie [CHU Saint-Antoine] (DHU i2B ), CHU Saint-Antoine [APHP]-Assistance publique - Hôpitaux de Paris (AP-HP) (APHP), Assistance publique - Hôpitaux de Paris (AP-HP) (APHP)-CHU Cochin [AP-HP], Assistance publique - Hôpitaux de Paris (AP-HP) (APHP)-CHU Necker - Enfants Malades [AP-HP], Centre National de la Recherche Scientifique (CNRS)-Université Paris Descartes - Paris 5 (UPD5)-Institut National de la Santé et de la Recherche Médicale (INSERM), Institut National de la Santé et de la Recherche Médicale (INSERM)-Aix Marseille Université (AMU), Centre National de la Recherche Scientifique (CNRS)-IRD-Institut National de la Santé et de la Recherche Médicale (INSERM)-Université de La Réunion (UR), Centre hospitalier régional Metz-Thionville (CHR Metz-Thionville)-Hôpital Sainte-Blandine, Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP), and Sorbonne Université (SU)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)
Subjects: Adult, Male, Gastrointestinal Diseases, characteristics, [ SDV.MHEP.RSOA ] Life Sciences [q-bio]/Human health and pathology/Rhumatology and musculoskeletal system, IgA vasculitis, Adrenal Cortex Hormones, Surveys and Questionnaires, Humans, Propensity Score, Cyclophosphamide, Aged, Retrospective Studies, [SDV.MHEP.RSOA] Life Sciences [q-bio]/Human health and pathology/Rhumatology and musculoskeletal system, treatment, Glomerulonephritis, IGA, [ SDV.SPEE ] Life Sciences [q-bio]/Santé publique et épidémiologie, Middle Aged, Arthralgia, Henoch-Schönlein purpura, Logistic Models, Treatment Outcome, [SDV.MHEP.RSOA]Life Sciences [q-bio]/Human health and pathology/Rhumatology and musculoskeletal system, [SDV.SPEE] Life Sciences [q-bio]/Santé publique et épidémiologie, Antirheumatic Agents, Multivariate Analysis, Drug Therapy, Combination, Female, [SDV.SPEE]Life Sciences [q-bio]/Santé publique et épidémiologie, France, prognosis, Colchicine
Abstract: Data on adult IgA vasculitis (Henoch-Schönlein) (IgAV) are scarce. This survey was designed to better define the clinical spectrum of IgAV and efficacy of treatments in a French patient population.Data on clinical characteristics, histologic features, and treatment response from 260 patients with IgAV included in a French multicenter retrospective survey were analyzed. Efficacy data were compared using different statistical models.The mean ± SD age of the patients with IgAV at diagnosis was 50.1 ± 18 years, and 63% of patients were male. Baseline manifestations included purpura (100%), arthralgias/arthritis/myalgia (61%), glomerulonephritis (70%), and/or gastrointestinal involvement (53%). Thirty percent of patients showed renal failure at baseline. In univariate analysis, the response to therapy was 80% (64 of 80) in patients treated with corticosteroids (CS) alone, compared to 77% (23 of 30) in patients treated with CS plus cyclophosphamide (CYC) and 59% (10 of 17) in patients treated with colchicine (P = 0.17). Multivariable analysis showed that treatment with CS or CS plus CYC was more effective than colchicine in achieving a response. Efficacy differences were demonstrated using different statistical models: in the multivariable logistic regression model, odds ratio (OR) 3.68, 95% confidence interval (95% CI) 1.10-12.33 (P = 0.03); in the inverse probability weighting on propensity score model, OR 3.75, 95% CI 1.28-10.99 (P = 0.02). The efficacy of CS plus CYC as compared to CS alone was discordant according to the analytic method used. Analysis with the multivariable logistic regression model did not demonstrate a difference between CS plus CYC and CS alone (OR 0.88, 95% CI 0.29-2.67; P = 0.82). In contrast, inverse probability weighting on propensity score showed that CS plus CYC was more effective than CS alone (OR 1.79, 95% CI 1.00-3.20; P = 0.049).This series constitutes the largest series of adults with IgAV reported in the literature so far. It provides data on clinical and histologic presentation and therapeutic efficacy, suggesting that CS alone appears to be a reasonable first-line therapy in patients with IgAV, while the benefit of adding CYC to CS remains uncertain.
Published: 2017
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16. A multicentre study of 95 biopsy-proven cases of renal disease in primary Sjögren’s syndrome

Author: Stanislas Faguer, H. François, Alain Le Quellec, Guillaume Moulis, Laurent Chiche, Alexandre Karras, Philippe Remy, Xavier Mariette, Aurélie Hummel, Evguenia Krastinova, Perrine Jullien, Anne-Laure Fauchais, J.B. Fraison, Véronique Le Guern, Estibaliz Lazaro, R. Mesbah, Magali Jasiek, Noémie Jourde-Chiche, Emmanuelle Dernis, Benjamin Terrier, Sophie Ferlicot, Carole Cordonnier, Philippe Vanhille, Pierre Ronco, Eric Thervet, Raphaèle Seror, Nathalie Costedoat-Chalumeau, Eric Hachulla, Vannary Meas-Yedid, Laurent Daniel, AP-HP Hôpital Bicêtre (Le Kremlin-Bicêtre), Hôpital Européen Georges Pompidou [APHP] (HEGP), Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Hôpitaux Universitaires Paris Ouest - Hôpitaux Universitaires Île de France Ouest (HUPO), Centre National de Référence Maladies Systémiques et Autoimmunes Rares, Hôpital Cochin [AP-HP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP), CH Boulogne sur Mer, Hôpital de Rangueil, CHU Toulouse [Toulouse], Hôpital de la Conception [CHU - APHM] (LA CONCEPTION), CHU Dupuytren, Hôpital Européen [Fondation Ambroise Paré - Marseille], Centre Hospitalier Le Mans (CH Le Mans), Hôpital Purpan [Toulouse], CH Béziers, Hôpital Haut-Lévêque [CHU Bordeaux], CHU Bordeaux [Bordeaux], CHU Saint-Etienne, Hôpital Claude Huriez [Lille], CHU Lille, CHU Saint-Eloi, CHU Henri Mondor, CHU Necker - Enfants Malades [AP-HP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP), CHU Tenon [AP-HP], Sorbonne Université (SU)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP), CH Valenciennes, Analyse d'images biologiques - Biological Image Analysis (BIA), Institut Pasteur [Paris]-Centre National de la Recherche Scientifique (CNRS), CHU Amiens-Picardie, Hôpital Bicêtre, No specific funding was received from any bodies in the public, commercial or not-for-profit sectors to carry out the work described in this manuscript., The authors thank all the clinicians and pathologists who participated in the REINSS study: C. Agard, A. Audemard, S. Bally, J.J. Boffa, D. Le Thi Huong-Boutin, P. Cacoub, P. Cathebras, G. Choukroun, E. Daugas, J.G. Fuzibet, C. Garrouste, M. Hamidou, P.Y. Hatron, D. Joly, J.E. Kahn, B. Knebelmann, T. Kofman, B. Laurent-Pilonchery, C. Lavigne, G. Le Guenno, T. Lobbedez, J. Schmidt, C. Sordet, A. Barbier-Dupas, A. Guillaudeau, A. Moreau, B. Ambrosetti, B. Mougenot, B. Mac-Gregor, C. Deminiere, C. Guilbeau-Frugier, C. Mussini, D. Droz, D. Desvaux-Belghiti, F. Comoz, H. Perrochia, H. Beaufils, J. Verine, L.H. Noel, J.P. Duong Van Huyen, A. Modesto, L. Marcellin, M. Mignon-Conte, D. Nochy, N. Patey-Mariaud De Serre, P. Rouvier, S. Lepreux, V. Algagnac-Oskman, M.-C. Copin, B. Gosselin, B. Delisle, F. Paraf, I. Brocheriou, J.-P. Saint-Are, J.-L. Kermeny, P. Callard., Centre Hospitalier Universitaire de Toulouse (CHU Toulouse), Centre Hospitalier Universitaire de Saint-Etienne [CHU Saint-Etienne] (CHU ST-E), Hôpital Saint Eloi (CHRU Montpellier), Centre Hospitalier Régional Universitaire [Montpellier] (CHRU Montpellier), CHU Henri Mondor [Créteil], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU), and Institut Pasteur [Paris] (IP)-Centre National de la Recherche Scientifique (CNRS)
Subjects: Male, Biopsy, T-Lymphocytes, Kidney Glomerulus, Plasma cell, Gastroenterology, [SDV.MHEP.UN]Life Sciences [q-bio]/Human health and pathology/Urology and Nephrology, 0302 clinical medicine, Adrenal Cortex Hormones, Medicine, Pharmacology (medical), tubulointerstitial nephritis, 030212 general & internal medicine, Renal Insufficiency, Cryoglobulins, Aged, 80 and over, B-Lymphocytes, medicine.diagnostic_test, biology, Middle Aged, 3. Good health, medicine.anatomical_structure, Sjogren's Syndrome, [SDV.MHEP.RSOA]Life Sciences [q-bio]/Human health and pathology/Rhumatology and musculoskeletal system, Antibodies, Antinuclear, Rituximab, Female, Renal biopsy, France, Antibody, Immunosuppressive Agents, medicine.drug, Adult, medicine.medical_specialty, Adolescent, Plasma Cells, primary Sjögren’s syndrome, Renal function, cryoglobulinaemia, Nephropathy, 03 medical and health sciences, Young Adult, Rheumatology, Internal medicine, Humans, Aged, Retrospective Studies, 030203 arthritis & rheumatology, business.industry, Plasmacytosis, medicine.disease, biology.protein, Nephritis, Interstitial, business
Abstract: International audience; Objective. Renal involvement is a rare event during primary SS (pSS). We aimed to describe the clinico-biological and histopathological characteristics of pSS-related nephropathy and its response to treatment. Methods. We conducted a French nationwide, retrospective, multicentre study including pSS patients fulfilling AmericanEuropean Consensus Group criteria or enlarged AmericanEuropean Consensus Group criteria, and with biopsy-proven renal involvement. Results. A total of 95 patients were included (median age 49 years). An estimated glomerular filtration rate (eGFR) of
Published: 2017
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17. Underestimation Rate at MR Imaging–guided Vacuum-assisted Breast Biopsy: A Multi-Institutional Retrospective Study of 1509 Breast Biopsies

Author: Isabelle Doutriaux, Domenico Lepori, Edouard Poncelet, Guillaume Laffargue, Patrice Taourel, Aurélie Jalaguier, Pascal Cherel, Ingrid Millet, Corinne Balleyguier, Emma Pages-Bouic, Cécile Verheyden, Isabelle Thomassin-Naggara, Centre Hospitalier Régional Universitaire [Montpellier] (CHRU Montpellier), Département d'imagerie médicale [Gustave Roussy], Institut Gustave Roussy (IGR), CRLCC René Huguenin, UNICANCER - Institut régional du Cancer Montpellier Val d'Aurelle (ICM), CRLCC Val d'Aurelle - Paul Lamarque, Institut de Cancérologie de l'Ouest [Angers/Nantes] (UNICANCER/ICO), UNICANCER, Institut Paoli-Calmettes, Fédération nationale des Centres de lutte contre le Cancer (FNCLCC), CH Valenciennes, Service de Radiologie [CHU Tenon], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-CHU Tenon [AP-HP], and Sorbonne Université (SU)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU)
Subjects: Image-Guided Biopsy, medicine.medical_specialty, Vacuum, [SDV.IB.IMA]Life Sciences [q-bio]/Bioengineering/Imaging, Breast Neoplasms, [SDV.IB.MN]Life Sciences [q-bio]/Bioengineering/Nuclear medicine, 030218 nuclear medicine & medical imaging, 03 medical and health sciences, 0302 clinical medicine, Biopsy, medicine, Humans, Radiology, Nuclear Medicine and imaging, Ductal Hyperplasia, Breast, skin and connective tissue diseases, Retrospective Studies, medicine.diagnostic_test, business.industry, Carcinoma, Ductal, Breast, Retrospective cohort study, Ductal carcinoma, Middle Aged, Mr imaging, Magnetic Resonance Imaging, 3. Good health, Carcinoma, Intraductal, Noninfiltrating, 030220 oncology & carcinogenesis, Vacuum-assisted breast biopsy, Female, Radiology, business, [SDV.MHEP]Life Sciences [q-bio]/Human health and pathology
Abstract: International audience; Purpose To assess the rate of underestimation of atypical ductal hyperplasia (ADH) and ductal carcinoma in situ (DCIS) at magnetic resonance (MR) imaging-guided vacuum-assisted breast biopsy and to explore the imaging, demographic, and histologic characteristics associated with lesion upgrade after surgery. Materials and Methods This retrospective study had institutional review board approval, and the need to obtain informed patient consent was waived. A total of 1509 MR imaging-guided vacuum-assisted biopsy procedures were performed in nine centers. A diagnosis of ADH was obtained after biopsy in 72 cases, and a diagnosis of DCIS was obtained in 118 cases. Pearson χ2 and Fisher tests were used to assess the association between demographic, MR imaging, and biopsy features and lesion upgrade. Univariate statistical analyses were performed, and each significant parameter was entered into a multivariate logistic regression analysis. Results Surgical excision was performed in 66 of the 72 ADH cases and in 117 of 118 DCIS cases. The ADH and DCIS underestimation rates were 25.8% (17 of 66) and 23.1% (27 of 117), respectively. Underestimation was 5.6-fold (odds ratio [OR] = 5.6; 95% confidence interval [CI]: 1.7, 18.3) and 3.6-fold (OR = 3.6; 95% CI: 1.2, 10) more likely in mass (n = 20 for ADH and n = 20 for DCIS) than in non-mass (n = 46 for ADH and n = 97 for DCIS), compared with nonunderestimation, in ADH and DCIS respectively. At multivariate analysis, the use of a 9- or 10-gauge needle versus a 7- or 8-gauge needle was also an independently associated with underestimation when a diagnosis of ADH was made at MR imaging-guided biopsy. No other parameters were associated with of ADH or DCIS upgrade at surgery. Conclusion The rates of underestimation in ADH and DCIS diagnosed at MR imaging-guided vacuum-assisted biopsy were high, at around 25%, and were significantly associated with the presence of a mass at MR imaging. © RSNA, 2016.
Published: 2016
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18. Bullous pemphigoid and dipeptidyl peptidase IV inhibitors: a case-noncase study in the French Pharmacovigilance Database

Author: Béné, J., Moulis, G., Bennani, I., Auffret, Michel, Coupe, P., Babai, S., Hillaire-Buys, D., Micallef, J., Gautier, S., Regional Pharmacovigilance Centres, French Association Of, Centre Hospitalier Régional Universitaire [Lille] (CHRU Lille), CHU Toulouse [Toulouse], Epidémiologie et analyses en santé publique : risques, maladies chroniques et handicaps (LEASP), Université Toulouse III - Paul Sabatier (UT3), Université Fédérale Toulouse Midi-Pyrénées-Université Fédérale Toulouse Midi-Pyrénées-Institut National de la Santé et de la Recherche Médicale (INSERM), Centre d'investigation clinique de Toulouse (CIC 1436), Université Fédérale Toulouse Midi-Pyrénées-Université Fédérale Toulouse Midi-Pyrénées-CHU Toulouse [Toulouse]-Institut National de la Santé et de la Recherche Médicale (INSERM), CH Valenciennes, Hôpital Henri Mondor, Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Hôpital Henri Mondor-Université Paris-Est Créteil Val-de-Marne - Paris 12 (UPEC UP12), Centre Hospitalier Régional Universitaire [Montpellier] (CHRU Montpellier), Pathogénèse et contrôle des infections chroniques (PCCI), Institut National de la Santé et de la Recherche Médicale (INSERM)-Université de Montpellier (UM)-Centre Hospitalier Universitaire de Montpellier (CHU Montpellier ), Service de Pharmacologie Clinique [Hôpital de la Timone - APHM], and Assistance Publique - Hôpitaux de Marseille (APHM)- Hôpital de la Timone [CHU - APHM] (TIMONE)
Subjects: Adult, Male, Pemphigoid, medicine.medical_specialty, animal structures, Dermatology, Saxagliptin, Dipeptidyl peptidase, Pharmacovigilance, Safety-Based Drug Withdrawals, 030207 dermatology & venereal diseases, 03 medical and health sciences, chemistry.chemical_compound, 0302 clinical medicine, Risk Factors, Pemphigoid, Bullous, medicine, Humans, Vildagliptin, 030212 general & internal medicine, skin and connective tissue diseases, Aged, Aged, 80 and over, Dipeptidyl-Peptidase IV Inhibitors, integumentary system, business.industry, Middle Aged, medicine.disease, eye diseases, 3. Good health, chemistry, Sitagliptin, Immunology, Female, Drug Eruptions, France, Bullous pemphigoid, business, Adverse drug reaction, [SDV.MHEP.DERM]Life Sciences [q-bio]/Human health and pathology/Dermatology, medicine.drug
Abstract: SummaryBackground Inhibitors of dipeptidyl peptidase (DPP)-IV have been suspected in the onset of bullous pemphigoid for several years now. However, comparative studies assessing the link between DPP-IV inhibitor exposure and bullous pemphigoid have not yet been performed. Objectives To detect, from the French Pharmacovigilance Database (FPVD), a signal of risk of bullous pemphigoid during DPP-IV inhibitor exposure by comparative study. Methods All spontaneous reports of DPP-IV inhibitor-related bullous pemphigoid recorded in the FPVD between April 2008 and August 2014 were described. We conducted disproportionality analyses (case–noncase method) to assess the link between DPP-IV inhibitors and bullous pemphigoid, calculating reporting odds ratios (RORs). We also compared DPP-IV inhibitor-induced bullous pemphigoid reports rated per million defined daily doses dispensed during the study period. Results Among 217 331 spontaneous adverse drug reaction reports registered in the FPVD, 1297 involved DPP-IV inhibitors. Among these observations, 42 were bullous pemphigoid (vildagliptin, n = 31; sitagliptin, n = 10; saxagliptin, n = 1). The ROR for pooled DPP-IV inhibitors was 67·5 [95% confidence interval (CI) 47·1–96·9]. Disproportionality was also observed for each DPP-IV inhibitor: vildagliptin (ROR 225·3, 95% CI 148·9–340·9), sitagliptin (ROR 17·0, 95% CI 8·9–32·5) and saxagliptin (ROR 16·5, 95% CI 2·3–119·1). Analyses adjusted on dispensing data led to similar results. Conclusions These data confirm a strong signal for an increased risk of bullous pemphigoid during DPP-IV inhibitor exposure. This adverse drug reaction is observed for each DPP-IV inhibitor, suggesting a class effect. The signal was higher with vildagliptin than with the other DPP-IV inhibitors.
Published: 2016
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19. Ileal or Anastomotic Location of Lesions Does Not Impact Rate of Postoperative Recurrence in Crohn's Disease Patients Classified i2 on the Rutgeerts Score

Author: O. Manolache, P. Bayart, Vincent Maunoury, Jean-Eric Laberenne, Benjamin Pariente, Julien Branche, Pierre Desreumaux, Boruchowicz A, Romain Gerard, Nicolas Duveau, P. Zerbib, G Pineton de Chambrun, Medina Boualit, Pauline Wils, Maria Nachury, Hôpital Claude Huriez [Lille], CHU Lille, Lille Inflammation Research International Center - U 995 (LIRIC), Centre Hospitalier Régional Universitaire [Lille] (CHRU Lille)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Université de Lille-Institut Pasteur de Lille, Réseau International des Instituts Pasteur (RIIP)-Réseau International des Instituts Pasteur (RIIP), CH Valenciennes, Service d'Hépato-Gastroenterologie [Seclin], Centre Hospitalier de Seclin, Centre Hospitalier Régional Universitaire [Montpellier] (CHRU Montpellier), Institut Pasteur de Lille, and Réseau International des Instituts Pasteur (RIIP)-Réseau International des Instituts Pasteur (RIIP)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Université de Lille-Centre Hospitalier Régional Universitaire [Lille] (CHRU Lille)
Subjects: Crohn’s disease, Adult, Male, medicine.medical_specialty, Therapeutic management, Physiology, Colon, Postoperative recurrence, Constriction, Pathologic, Anastomosis, Gastroenterology, 03 medical and health sciences, 0302 clinical medicine, Secondary outcome, Crohn Disease, Ileocolonic anastomosis, Ileum, Recurrence, Internal medicine, medicine, Humans, In patient, Postoperative Period, Retrospective Studies, Crohn's disease, business.industry, Anastomosis, Surgical, Retrospective cohort study, [SDV.MHEP.HEG]Life Sciences [q-bio]/Human health and pathology/Hépatology and Gastroenterology, Rutgeerts score, Colonoscopy, Hepatology, medicine.disease, Prognosis, Ileocolonoscopy, 3. Good health, Surgery, Intestinal Perforation, 030220 oncology & carcinogenesis, Clinical recurrence, 030211 gastroenterology & hepatology, Female, business, Intestinal Obstruction
Abstract: International audience; BACKGROUND:The Rutgeerts score with 5 grades of severity (i0-i4) is a suitable endoscopic model to predict clinical recurrence following ileocolonic resection in Crohn's disease (CD). Definition of grade i2 includes lesions confined to the ileocolonic anastomosis (i2a) or moderate lesions on the neo-terminal ileum (i2b). The aim of the present study was to evaluate the probability of clinical recurrence in i2a and i2b patients.METHODS:This multicenter retrospective study included all CD patients classified i2 at the first postoperative ileocolonoscopy. The primary outcome was to evaluate the probability of clinical recurrence in patients classified i2a and i2b. The secondary outcome was to compare the rate of global recurrence of CD.RESULTS:Fifty patients were included: 23 were classified i2a and 27 were classified i2b. The median duration of follow-up was 40 (18.0-80.4) months in the i2a group and 53.5 (25.0-69.0) months in the i2b group (p = 0.9). The probability of clinical recurrence was not significantly different between patients classified i2a and i2b (p = 0.64). Median time to clinical recurrence after the first ileocolonoscopy and probability of global CD recurrence were not different between the two groups (p ≥ 0.19).CONCLUSIONS:The rate of clinical postoperative recurrence is not different in i2a and i2b patients. These results suggest that the same therapeutic strategy should be used in all patients classified i2 on the Rutgeerts score whatever the location of postoperative CD recurrence.
Published: 2016
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20. Long-term outcomes of the WEGENT trial on remission-maintenance for granulomatosis with polyangiitis or microscopic polyangiitis

Author: Puéchal, Xavier, Pagnoux, Christian, Perrodeau, Elodie, Hamidou, Mohamed, Boffa, Jean-Jacques, Kyndt, Xavier, Lifermann, François, Papo, Thomas, Merrien, Dominique, Smail, Amar, Delaval, Philippe, Hanrotel-Saliou, Catherine, Imbert, Bernard, Khouatra, Chahéra, Lambert, Marc, Leské, Charles, Ly, Kim, Pertuiset, Edouard, Roblot, Pascal, Ruivard, Marc, Subra, Jean-François, Viallard, Jean-François, Terrier, Benjamin, Cohen, Pascal, Mouthon, Luc, Le Jeunne, Claire, Ravaud, Philippe, Guillevin, Loïc, Institut Cochin ( UM3 (UMR 8104 / U1016) ), Université Paris Descartes - Paris 5 ( UPD5 ) -Institut National de la Santé et de la Recherche Médicale ( INSERM ) -Centre National de la Recherche Scientifique ( CNRS ), Service de médecine interne et centre de référence des maladies rares [CHU Cochin], Assistance publique - Hôpitaux de Paris (AP-HP)-CHU Cochin [AP-HP], Equipe 5 : METHODS - Méthodes de l’évaluation thérapeutique des maladies chroniques ( CRESS - U1153 ), Université Paris Descartes - Paris 5 ( UPD5 ) -Centre de Recherche Épidémiologie et Statistique Sorbonne Paris Cité ( CRESS (U1153 / UMR_A 1125) ), Institut National de la Recherche Agronomique ( INRA ) -Université Sorbonne Paris Cité ( USPC ) -Institut National de la Santé et de la Recherche Médicale ( INSERM ) -Institut National de la Recherche Agronomique ( INRA ) -Université Sorbonne Paris Cité ( USPC ) -Institut National de la Santé et de la Recherche Médicale ( INSERM ), Service de médecine interne [Nantes], Université de Nantes ( UN ) -Hôtel-Dieu-Centre hospitalier universitaire de Nantes ( CHU Nantes ), Remodelage et Reparation du Tissu Renal, Université Pierre et Marie Curie - Paris 6 ( UPMC ) -Institut National de la Santé et de la Recherche Médicale ( INSERM ), Néphrologie, Assistance publique - Hôpitaux de Paris (AP-HP)-CHU Tenon [APHP], Service de médecine interne et néphrologie, CH Valenciennes, Service de Médecine Interne [Dax], Hôpital Dax, Hôpital Bichat - Claude Bernard, Centre Hospitalier Compiègne-Noyon, Service de Néphrologie - Médecine Interne, CHU Amiens-Picardie-Hôpital Sud, Institut de recherche, santé, environnement et travail ( Irset ), Université d'Angers ( UA ) -Université de Rennes 1 ( UR1 ), Université de Rennes ( UNIV-RENNES ) -Université de Rennes ( UNIV-RENNES ) -École des Hautes Études en Santé Publique [EHESP] ( EHESP ) -Institut National de la Santé et de la Recherche Médicale ( INSERM ) -Structure Fédérative de Recherche en Biologie et Santé de Rennes ( Biosit : Biologie - Santé - Innovation Technologique ) -Université des Antilles ( UA ), Service de pneumologie, CHU Pontchaillou [Rennes], Immunologie et Pathologie ( EA2216 ), Université de Brest ( UBO ) -IFR148, CHRU - Service de néphrologie, dialyse et transplantation rénale, Centre Hospitalier Régional Universitaire de Brest ( CHRU Brest ), CHU Grenoble, Hôpital Louis Pradel [CHU - HCL], Hospices Civils de Lyon ( HCL ), Centre Hospitalier Régional Universitaire [Lille] ( CHRU Lille ), CH Cholet, CHU Limoges, Hopital Réné Dubos, Service de Médecine Interne, Centre hospitalier universitaire de Poitiers ( CHU Poitiers ), CHU Estaing [Clermont-Ferrand], Service de Néphrologie [Angers], Université d'Angers ( UA ) -CHU Angers, Service de Médecine Interne et Maladies Infectieuses [Pessac], CHU Bordeaux [Bordeaux]-Hôpital Haut-Lévêque [CHU Bordeaux], CHU Bordeaux [Bordeaux], CHU Cochin [AP-HP], Club Rhumatismes et Inflammation, Institut Cochin (IC UM3 (UMR 8104 / U1016)), Université Paris Descartes - Paris 5 (UPD5)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Centre National de la Recherche Scientifique (CNRS), Assistance publique - Hôpitaux de Paris (AP-HP) (APHP)-CHU Cochin [AP-HP], Equipe 5 : METHODS - Méthodes de l’évaluation thérapeutique des maladies chroniques (CRESS - U1153), Université Paris Descartes - Paris 5 (UPD5)-Centre de Recherche Épidémiologie et Statistique Sorbonne Paris Cité (CRESS (U1153 / UMR_A_1125 / UMR_S_1153)), Université Paris Diderot - Paris 7 (UPD7)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Institut National de la Recherche Agronomique (INRA)-Université Paris Descartes - Paris 5 (UPD5)-Université Sorbonne Paris Cité (USPC)-Université Paris Diderot - Paris 7 (UPD7)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Institut National de la Recherche Agronomique (INRA)-Université Sorbonne Paris Cité (USPC), Université de Nantes (UN)-Hôtel-Dieu-Centre hospitalier universitaire de Nantes (CHU Nantes), Université Pierre et Marie Curie - Paris 6 (UPMC)-Institut National de la Santé et de la Recherche Médicale (INSERM), CHU Tenon [APHP], Centre Hospitalier de Dax, Institut de recherche en santé, environnement et travail (Irset), Université d'Angers (UA)-Université de Rennes 1 (UR1), Université de Rennes (UNIV-RENNES)-Université de Rennes (UNIV-RENNES)-École des Hautes Études en Santé Publique [EHESP] (EHESP)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Structure Fédérative de Recherche en Biologie et Santé de Rennes ( Biosit : Biologie - Santé - Innovation Technologique ), Immunologie et Pathologie (EA2216), Université de Brest (UBO)-IFR148, Centre Hospitalier Régional Universitaire de Brest (CHRU Brest), Hospices Civils de Lyon (HCL), Centre Hospitalier Régional Universitaire [Lille] (CHRU Lille), Centre hospitalier universitaire de Poitiers (CHU Poitiers), Université d'Angers (UA)-Centre Hospitalier Universitaire d'Angers (CHU Angers), PRES Université Nantes Angers Le Mans (UNAM)-PRES Université Nantes Angers Le Mans (UNAM), Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Hôpital Cochin [AP-HP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP), Institut National de la Recherche Agronomique (INRA)-Université Paris Diderot - Paris 7 (UPD7)-Université Paris Descartes - Paris 5 (UPD5)-Université Sorbonne Paris Cité (USPC)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Institut National de la Recherche Agronomique (INRA)-Université Paris Diderot - Paris 7 (UPD7)-Université Sorbonne Paris Cité (USPC)-Institut National de la Santé et de la Recherche Médicale (INSERM), CHU Tenon [AP-HP], Sorbonne Université (SU)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP), CHU Amiens-Picardie-hôpital Sud, Structure Fédérative de Recherche en Biologie et Santé de Rennes ( Biosit : Biologie - Santé - Innovation Technologique )-Institut National de la Santé et de la Recherche Médicale (INSERM)-École des Hautes Études en Santé Publique [EHESP] (EHESP)-Université de Rennes 1 (UR1), Université de Rennes (UNIV-RENNES)-Université de Rennes (UNIV-RENNES)-Université d'Angers (UA), CHU Clermont-Ferrand, Hôpital Cochin [AP-HP], Hospices Civils de Lyon (Délégation à la Recherche Clinique, Lyon, France, Trial no. 97.129), Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU), Université d'Angers (UA)-Université de Rennes (UR)-École des Hautes Études en Santé Publique [EHESP] (EHESP)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Structure Fédérative de Recherche en Biologie et Santé de Rennes ( Biosit : Biologie - Santé - Innovation Technologique ), and Jonchère, Laurent
Subjects: [SDV] Life Sciences [q-bio], [ SDV ] Life Sciences [q-bio], [SDV]Life Sciences [q-bio]
Abstract: International audience; Objective - Findings from the WEGENT trial and other short-term studies have suggested that azathioprine (AZA) or methotrexate (MTX) could effectively maintain remission of granulomatosis with polyangiitis (Wegener's) (GPA) or microscopic polyangiitis (MPA). This study was undertaken to examine whether differences in rates of relapse or adverse events would appear after discontinuation of these 2 maintenance regimens, when assessed over a longer followup period. Methods - Long-term outcomes in patients enrolled in the WEGENT trial were analyzed according to their randomized treatment group (AZA or MTX). Parameters at trial entry were evaluated as potential prognostic factors for death, relapse, or damage in multivariate models. Results - Data from 10 years of followup were available for 112 (88.8%) of the 126 original trial participants. The median followup time was 11.9 years (95% confidence interval [95% CI] 11.3-12.5 years). In patients receiving AZA and those receiving MTX, the 10-year overall survival rates were 75.1% (95% CI 64.8-86.9%) and 79.9% (95% CI 70.3-90.8%) (P = 0.56), respectively, and relapse-free survival rates were 26.3% (95% CI 17.3-40.1%) and 33.5% (95% CI 23.5-47.7%) (P = 0.29), respectively. No between-treatment differences were observed with regard to rates of relapse, adverse events, damage, survival without severe side effects, and survival without relapse and severe side effects. In analyses limited to the 97 patients with GPA, no between-treatment differences in survival rates were observed. The 10-year relapse-free survival rate was lower in patients with GPA than in patients with MPA. However, in the multivariate analysis, anti-proteinase 3 antineutrophil cytoplasmic antibody (ANCA) positivity, and not GPA, was retained as being independently associated with the relapse rate. Conclusion - The results of this long-term analysis confirm that AZA and MTX are comparable treatment options for maintaining remission of GPA or MPA. Despite achieving good overall survival with these treatments, relapse rates, adverse events, and damage remain matters of concern and further studies are needed to reduce their frequency in these ANCA-associated vasculitides.
Published: 2016
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21. BACH2 promotes indolent clinical presentation in Waldenström macroglobulinemia

Author: Catherine Roche-Lestienne, Sylvie Galiègue-Zouitina, Eileen M Boyle, Sabine Tricot, Stéphanie Poulain, Aline Renneville, Guillemette Marot, Nicolas Poret, Bruno Quesnel, Christophe Roumier, Claude Preudhomme, Guillemette Fouquet, Xavier Leleu, Natacha Broucqsault, Olivier Nibourel, Elisabeth Bertrand, Charles Herbaux, Franck Morschhauser, Valérie Soenen, CHU Lille, Inserm, Université de Lille, Evaluation des technologies de santé et des pratiques médicales - ULR 2694 [METRICS], METRICS : Evaluation des technologies de santé et des pratiques médicales - ULR 2694, Centre de Recherche Jean-Pierre AUBERT Neurosciences et Cancer (JPArc) - U1172, Groupe de Recherche sur les formes Injectables et les Technologies Associées - ULR 7365 [GRITA], Centre Hospitalier Régional Universitaire [Lille] (CHRU Lille), Centre de Recherche Jean-Pierre AUBERT Neurosciences et Cancer - U837 (JPArc), Université Lille Nord de France (COMUE)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Université de Lille, Evaluation des technologies de santé et des pratiques médicales - ULR 2694 (METRICS), Université de Lille-Centre Hospitalier Régional Universitaire [Lille] (CHRU Lille), MOdel for Data Analysis and Learning (MODAL), Laboratoire Paul Painlevé (LPP), Université de Lille-Centre National de la Recherche Scientifique (CNRS)-Université de Lille-Centre National de la Recherche Scientifique (CNRS)-Université de Lille, Sciences et Technologies-Inria Lille - Nord Europe, Institut National de Recherche en Informatique et en Automatique (Inria)-Institut National de Recherche en Informatique et en Automatique (Inria)-Evaluation des technologies de santé et des pratiques médicales - ULR 2694 (METRICS), Université de Lille-Centre Hospitalier Régional Universitaire [Lille] (CHRU Lille)-Université de Lille-Centre Hospitalier Régional Universitaire [Lille] (CHRU Lille)-École polytechnique universitaire de Lille (Polytech Lille), Service d'Hématologie Cellulaire [Lille], CH Valenciennes, Centre hospitalier universitaire de Poitiers (CHU Poitiers), Centre de Recherche Jean-Pierre AUBERT Neurosciences et Cancer - U1172 Inserm - U837 (JPArc), Institut National de la Santé et de la Recherche Médicale (INSERM)-Université Lille Nord de France (COMUE)-Université de Lille, Laboratoire Paul Painlevé - UMR 8524 (LPP), Centre National de la Recherche Scientifique (CNRS)-Université de Lille-Centre National de la Recherche Scientifique (CNRS)-Université de Lille-Université de Lille, Sciences et Technologies-Inria Lille - Nord Europe, and Marot, Guillemette
Subjects: 0301 basic medicine, Oncology, Candidate gene, medicine.medical_specialty, Pathology, Tumor suppressor gene, [SDV]Life Sciences [q-bio], BACH2, Asymptomatic, 03 medical and health sciences, [MATH.MATH-ST]Mathematics [math]/Statistics [math.ST], Progression, Waldenström macroglobulinemia, Indolent, Physiopathology, Internal medicine, indolent, medicine, Clonogenic assay, [MATH.MATH-ST] Mathematics [math]/Statistics [math.ST], business.industry, Waldenstrom macroglobulinemia, Macroglobulinemia, Gene signature, medicine.disease, Pathophysiology, 3. Good health, [SDV] Life Sciences [q-bio], 030104 developmental biology, progression, medicine.symptom, Clinical Research Paper, physiopathology, business
Abstract: International audience; Approximately 30% of the patients who fulfil the criteria of Waldenström's macroglobulinemia (WM) are diagnosed while asymptomatic (indolent), and will not require immediate therapy. Conversely, patients with a disease-related event will be considered for therapy. The physiopathology of these 2 groups remains unclear, and the mechanisms of progression from indolent to symptomatic WM have yet to be fully understood. Seventeen patients diagnosed with WM were included in this study, 8 asymptomatic WM (A-WM) and 9 symptomatic WM (S-WM). A differential analysis was performed on a first series of 11 patients and identified 48 genes whose expression separated samples from A-to S-WM. This gene signature was then confirmed on a second independent validation set of 6 WM. Within this expression profile, BACH2, a B-cell transcription factor known to be a tumor suppressor gene, was found to be over-expressed in A-MW relatively to S-MW. We specifically over-expressed BACH2 in a WM-related cell line and observed a significant reduction of the clonogenic activity. To the best of our knowledge, we report for the first time a specific gene expression signature that differentiates A-WM and S-WM. Within this expression profile, BACH2 was identified as a candidate gene that may help to understand better the behavior of tumor cells in indolent WM.
Published: 2016

22. Non HCV-related infectious cryoglobulinemia vasculitis: Results from the French nationwide CryoVas survey and systematic review of the literature

Author: Emmanuelle Plaisier, Bruno Graffin, Arnaud Hot, Luc de Saint-Martin, Catherine Michel, Patrice Cacoub, Patricia Senet, P. Belenotti, Olivier Hermine, Adeline Lacraz, Jean-Emmanuel Kahn, Benjamin Terrier, Xavier Mariette, Isabelle Marie, Jean-Marc Léger, Thomas Quemeneur, Fabrice Bonnet, Laurent Chiche, Service de médecine interne et centre de référence des maladies rares [CHU Cochin], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Hôpital Cochin [AP-HP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP), Service de Médecine Interne [CHU Rouen], CHU Rouen, Normandie Université (NU)-Normandie Université (NU)-Université de Rouen Normandie (UNIROUEN), Normandie Université (NU), Service de Néphrologie-Transplantation-Dialyse, CHU Bordeaux [Bordeaux]-Groupe hospitalier Pellegrin, Service de Médecine Interne (MARSEILLE - Med Int), Assistance Publique - Hôpitaux de Marseille (APHM), CHU Pontchaillou [Rennes], Centre d'Immunologie de Marseille - Luminy (CIML), Aix Marseille Université (AMU)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Centre National de la Recherche Scientifique (CNRS), Service de Médecine Interne, Hôpital Edouard Herriot [CHU - HCL], Hospices Civils de Lyon (HCL)-Hospices Civils de Lyon (HCL), Laboratoire d'Immunologie (EA 2686), Université de Lille, Droit et Santé, Physiologie des Adaptations Nutritionnelles (PhAN), Institut National de la Recherche Agronomique (INRA)-Université de Nantes (UN), CH Valenciennes, Département de Médecine Interne et Pneumologie [Brest] (DMIP - Brest), Centre Hospitalier Régional Universitaire de Brest (CHRU Brest), Groupe d'Etude de la Thrombose de Bretagne Occidentale (GETBO), Université de Brest (UBO)-Institut Brestois Santé Agro Matière (IBSAM), Université de Brest (UBO)-Université de Brest (UBO), AB Science SA, Centre d'Immunologie et de Maladies Infectieuses (CIMI), Université Pierre et Marie Curie - Paris 6 (UPMC)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Centre National de la Recherche Scientifique (CNRS), Institut National des Sciences Appliquées - Toulouse (INSA Toulouse), Institut National des Sciences Appliquées (INSA), Service de Dermatologie [CHU Tenon], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-CHU Tenon [APHP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université-Sorbonne Université, Service de Néphrologie et Dialyses [CHU Tenon], Immunologie - Immunopathologie - Immunothérapie (I3), Assistance publique - Hôpitaux de Paris (AP-HP) (APHP)-CHU Cochin [AP-HP], Service de Médecine Interne [Rouen], Université de Rouen Normandie (UNIROUEN), Normandie Université (NU)-Normandie Université (NU)-CHU Rouen, Institut National de la Santé et de la Recherche Médicale (INSERM)-Centre National de la Recherche Scientifique (CNRS)-Aix Marseille Université (AMU), UMR 1280 Physiologie des Adaptations Nutritionnelles, Institut National de la Recherche Agronomique (INRA)-Université de Nantes (UN)-Alimentation Humaine (ALIM.H), PRES Université Nantes Angers Le Mans (UNAM)-PRES Université Nantes Angers Le Mans (UNAM)-Physiologie des Adaptations Nutritionnelles (PhAN), Université de Brest (UBO), Assistance publique - Hôpitaux de Paris (AP-HP) (APHP)-CHU Tenon [APHP], Institut Cochin (IC UM3 (UMR 8104 / U1016)), Université Paris Descartes - Paris 5 (UPD5)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Centre National de la Recherche Scientifique (CNRS), Physiopathologie, Autoimmunité, maladies Neuromusculaires et THErapies Régénératrices (PANTHER), Normandie Université (NU)-Normandie Université (NU)-Institut National de la Santé et de la Recherche Médicale (INSERM), Hôpital Européen [Fondation Ambroise Paré - Marseille], Service de Médecine Interne [HI des Armées Legouest, Metz], Hôpital d’Instruction des Armées Legouest [Metz], Physiopathologie des Adaptations Nutritionnelles (PhAN), CHU Valenciennes, Service d'immuno-hématologie pédiatrique [CHU Necker], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-CHU Necker - Enfants Malades [AP-HP], Service de Neurologie [CHU Pitié-Salpêtrière], IFR70-CHU Pitié-Salpêtrière [AP-HP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP), Régulation de la réponse immune, infection VIH-1 et autoimmunité, Université Paris-Sud - Paris 11 (UP11)-Institut National de la Santé et de la Recherche Médicale (INSERM), Service de dermatologie et allergologie [CHU Tenon], CHU Tenon [AP-HP], Remodelage et Reparation du Tissu Renal, Université Pierre et Marie Curie - Paris 6 (UPMC)-Institut National de la Santé et de la Recherche Médicale (INSERM), Physiologie des Adaptations Nutritionnelles [UMR_A1280] (PhAN), Assistance publique - Hôpitaux de Paris (AP-HP) (APHP)-CHU Necker - Enfants Malades [AP-HP], Service de neurologie 1 [CHU Pitié-Salpétrière], Assistance publique - Hôpitaux de Paris (AP-HP) (APHP)-CHU Pitié-Salpêtrière [APHP], CHU Pitié-Salpêtrière [AP-HP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU), Sorbonne Université (SU)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP), Centre National de la Recherche Scientifique (CNRS)-Université Paris Descartes - Paris 5 (UPD5)-Institut National de la Santé et de la Recherche Médicale (INSERM), Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-CHU Pitié-Salpêtrière [AP-HP], Sorbonne Université (SU)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU), and Centre National de la Recherche Scientifique (CNRS)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Université Pierre et Marie Curie - Paris 6 (UPMC)
Subjects: Adult, Male, medicine.medical_specialty, Epstein-Barr Virus Infections, Hepatitis C virus, [SDV]Life Sciences [q-bio], Immunology, medicine.disease_cause, Anti-Infective Agents, Adrenal Cortex Hormones, Recurrence, Surveys and Questionnaires, medicine, Immunology and Allergy, Humans, Epstein–Barr virus infection, ComputingMilieux_MISCELLANEOUS, Aged, Hepatitis B virus, business.industry, Remission Induction, Systemic Vasculitis, Bacterial Infections, Hepatitis B, Middle Aged, medicine.disease, Prognosis, Cryoglobulinemia, Dermatology, 3. Good health, Treatment Outcome, Cytomegalovirus Infections, [SDV.IMM]Life Sciences [q-bio]/Immunology, Rituximab, Female, France, Vasculitis, business, Immunosuppressive Agents, [SDV.MHEP]Life Sciences [q-bio]/Human health and pathology, medicine.drug, Systemic vasculitis
Abstract: In patients with infectious cryoglobulinemia vasculitis (CryoVas) in the absence of hepatitis C virus infection, data on presentation, therapeutic management and outcome are lacking. We conducted a nationwide survey that included patients with HCV-negative CryoVas. We describe here the presentation, therapeutic management and outcome of 18 patients with non-HCV infectious CryoVas and 27 additional patients identified form a systematic review of the literature. We included 18 patients, mean age 57.9±13.5 years. Infectious causes were viral infections in 8 patients [hepatitis B virus (HBV) in 4, and cytomegalovirus, Epstein Barr virus, parvovirus B19 and human immunodeficiency virus in one case each], pyogenic bacterial infection in 6 patients, parasitic infection in 2 patients, and leprosy and candidiasis in one case each. Baseline manifestations were purpura (78%), glomerulonephritis (28%), arthralgia (28%), peripheral neuropathy (22%), skin necrosis (22%), cutaneous ulcers (17%), and myalgia (11%). Cryoglobulinemia was type II in 2/3 of cases. Most cases received specific anti-infectious therapy as first-line therapy, sometimes associated with corticosteroids, achieving sustained remission in the majority of cases. Refractory or relapsing patients, frequently related to HBV infection, showed a complete remission after rituximab in addition to antiviral therapy. In contrast, corticosteroids and/or immunosuppressive agents used in the absence of anti-infectious agents were frequently associated with refractory CryoVas. Viral and pyogenic bacterial infections represent the main causes of non-HCV infectious CryoVas. Antimicrobial therapy is commonly associated with sustained remission. Immunosuppressive agents should be considered only as a second-line option in patients with refractory vasculitis.
Published: 2015
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23. Clinical reappraisal of SHORT syndrome with PIK3R1 mutations: toward recommendation for molecular testing and management

Author: M, Avila, D A, Dyment, J V, Sagen, J, St-Onge, U, Moog, B H Y, Chung, S, Mo, S, Mansour, A, Albanese, S, Garcia, D O, Martin, A A, Lopez, T, Claudi, R, König, S M, White, S L, Sawyer, J A, Bernstein, L, Slattery, R K, Jobling, G, Yoon, C J, Curry, M L, Merrer, B L, Luyer, D, Héron, M, Mathieu-Dramard, P, Bitoun, S, Odent, J, Amiel, P, Kuentz, J, Thevenon, M, Laville, Y, Reznik, C, Fagour, M-L, Nunes, D, Delesalle, S, Manouvrier, O, Lascols, F, Huet, C, Binquet, L, Faivre, J-B, Rivière, C, Vigouroux, P R, Njølstad, A M, Innes, C, Thauvin-Robinet, CHU Dijon, Centre Hospitalier Universitaire de Dijon - Hôpital François Mitterrand (CHU Dijon), Génétique des Anomalies du Développement (GAD), IFR100 - Structure fédérative de recherche Santé-STIC-Université de Bourgogne (UB), Children's hospital of Eastern Ontario Research Institute, University of Bergen (UiB), Haukeland University Hospital, Human Genetics Institute, Heidelberg University, Department of Paediatrics and Adolescent Medicine [HKU], The University of Hong Kong (HKU), SW Thames Regional Genetics Service, St Georgeâ™s University of London, London, St George's Hospital, INGEMM, Instituto de Genética Médica y Molecular, IDIPAZ-Hospital Universitario La Paz, Instituto de Salud Carlos III [Madrid] (ISC), Hospital Central de la Cruz Roja San Jose y Santa Adela, University Hospital Puerta de Hierro, Madrid, Bodø University College, Humangenetik, Universitätsklinikum Frankfurt, University of Frankfurt, University of Melbourne, Victorian Clinical Genetics Services, Stanford University, The Hospital for sick children [Toronto] (SickKids), University of California [San Francisco] (UCSF), University of California, CHU Necker - Enfants Malades [AP-HP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP), CH Le Havre, Service de Génétique Cytogénétique et Embryologie [CHU Pitié-Salpêtrière], CHU Pitié-Salpêtrière [AP-HP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU), Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU), Groupe de Recherche Clinique : Déficience Intellectuelle et Autisme (GRC), Université Pierre et Marie Curie - Paris 6 (UPMC), CHU Trousseau [APHP], CHU Amiens-Picardie, Service de Pédiatrie [Jean Verdier], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Université Paris 13 (UP13)-Hôpital Jean Verdier [AP-HP], Institut de Génétique et Développement de Rennes (IGDR), Structure Fédérative de Recherche en Biologie et Santé de Rennes ( Biosit : Biologie - Santé - Innovation Technologique )-Centre National de la Recherche Scientifique (CNRS)-Université de Rennes 1 (UR1), Université de Rennes (UNIV-RENNES)-Université de Rennes (UNIV-RENNES), CHU Pontchaillou [Rennes], Génétique et épigénétique des maladies métaboliques, neurosensorielles et du développement (Inserm U781), Université Paris Descartes - Paris 5 (UPD5)-Institut National de la Santé et de la Recherche Médicale (INSERM), Centre de génétique - Centre de référence des maladies rares, anomalies du développement et syndromes malformatifs (CHU de Dijon), Cardiovasculaire, métabolisme, diabétologie et nutrition (CarMeN), Hospices Civils de Lyon (HCL)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Institut National des Sciences Appliquées de Lyon (INSA Lyon), Institut National des Sciences Appliquées (INSA)-Université de Lyon-Institut National des Sciences Appliquées (INSA)-Université de Lyon-Université Claude Bernard Lyon 1 (UCBL), Université de Lyon-Institut National de la Recherche Agronomique (INRA), Centre de Recherche en Nutrition Humaine Rhône-Alpes (CRNH-RA), Université Grenoble Alpes [2016-2019] (UGA [2016-2019])-Université Claude Bernard Lyon 1 (UCBL), Université de Lyon-Université de Lyon-Institut National de la Recherche Agronomique (INRA)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Université Jean Monnet [Saint-Étienne] (UJM)-CHU Saint-Etienne-Hospices Civils de Lyon (HCL)-CHU Grenoble, Hôpital Côte de Nacre [CHU Caen], CHU Caen, Normandie Université (NU)-Tumorothèque de Caen Basse-Normandie (TCBN)-Normandie Université (NU)-Tumorothèque de Caen Basse-Normandie (TCBN), CHU Bordeaux [Bordeaux], CH Valenciennes, Hôpital Jeanne de Flandres, Université de Lille, Droit et Santé-Centre Hospitalier Régional Universitaire [Lille] (CHRU Lille), Université Pierre et Marie Curie - Paris 6 - UFR de Médecine Pierre et Marie Curie (UPMC), Unité de Recherche sur les Maladies Cardiovasculaires, du Métabolisme et de la Nutrition = Institute of cardiometabolism and nutrition (ICAN), Université Pierre et Marie Curie - Paris 6 (UPMC)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Institut National de la Santé et de la Recherche Médicale (INSERM)-CHU Pitié-Salpêtrière [AP-HP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU)-Sorbonne Université (SU), Centre de Recherche Saint-Antoine (UMRS893), Université Pierre et Marie Curie - Paris 6 (UPMC)-Institut National de la Santé et de la Recherche Médicale (INSERM), Centre d'Investigation Clinique 1432 (Dijon) - Epidemiologie Clinique/Essais Cliniques (CIC-EC), Université de Bourgogne (UB)-Centre Hospitalier Universitaire de Dijon - Hôpital François Mitterrand (CHU Dijon)-Institut National de la Santé et de la Recherche Médicale (INSERM), University of Calgary, Financial support was from the Regional Council of Burgundy and the Care4Rare Canada Consortium was funded by Genome Canada, the Canadian Institutes of Health Research, the Ontario Genomics Institute, Ontario Research Fund, Genome Quebec, and Children’sHospital of Eastern Ontario Foundation. DAD is the recipient of a CIHR Clinical Investigator award from the Institute of Genetics. PRN was supported in part by grants from the Research Council of Norway, The University of Bergen, an ERC Advanced Grant, Helse Vest and the KG Jebsen Foundation., SW Thames Regional Genetics Service, St Georgeâ™s University of London, London, Université Paris 13 (UP13)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Hôpital Jean Verdier [AP-HP], Université de Rennes 1 (UR1), Université de Rennes (UNIV-RENNES)-Université de Rennes (UNIV-RENNES)-Centre National de la Recherche Scientifique (CNRS)-Structure Fédérative de Recherche en Biologie et Santé de Rennes ( Biosit : Biologie - Santé - Innovation Technologique ), Université de Lyon-Institut National des Sciences Appliquées (INSA)-Université de Lyon-Institut National des Sciences Appliquées (INSA)-Université Claude Bernard Lyon 1 (UCBL), Université de Bourgogne (UB)-IFR100 - Structure fédérative de recherche Santé-STIC, University of Bergen (UIB), Instituto de Salud Carlos III (ISC), Stanford University [Stanford], The Hospital for Sick Children, University of Toronto, Toronto M5G 1X8, Canada, Assistance publique - Hôpitaux de Paris (AP-HP) (APHP)-CHU Pitié-Salpêtrière [APHP], CHU Pitié-Salpêtrière [APHP], Service de neuropédiatrie [Trousseau], Centre hospitalier universitaire d'Amiens (CHU Amiens-Picardie), Assistance publique - Hôpitaux de Paris (AP-HP) (APHP)-Université Paris 13 (UP13)-Hôpital Jean Verdier [Bondy], Assistance publique - Hôpitaux de Paris (AP-HP) (APHP), Institut National de la Recherche Agronomique (INRA)-Université Claude Bernard Lyon 1 (UCBL), Université de Lyon-Université de Lyon-Institut National des Sciences Appliquées de Lyon (INSA Lyon), Université de Lyon-Institut National des Sciences Appliquées (INSA)-Institut National des Sciences Appliquées (INSA)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Hospices Civils de Lyon (HCL), Recherche Clinique, Université Joseph Fourier - Grenoble 1 (UJF)-Centre de Recherche en Nutrition Humaine Rhône - Alpes, Université Pierre et Marie Curie - Paris 6 (UPMC)-Assistance publique - Hôpitaux de Paris (AP-HP) (APHP)-Institut National de la Santé et de la Recherche Médicale (INSERM)-CHU Pitié-Salpêtrière [APHP], Centre de Recherche Saint-Antoine (CR Saint-Antoine), Institut National de la Santé et de la Recherche Médicale (INSERM)-Université Pierre et Marie Curie - Paris 6 (UPMC), Centre Hospitalier Universitaire de Dijon - Hôpital François Mitterrand ( CHU Dijon ), Génétique des Anomalies du Développement ( GAD ), Université de Bourgogne ( UB ) -IFR100 - Structure fédérative de recherche Santé-STIC, University of Bergen ( UIB ), The University of Hong Kong ( HKU ), Instituto de Salud Carlos III (ISCIII) - Spain RDR - CIBERER, University of California [San Francisco] ( UCSF ), Assistance publique - Hôpitaux de Paris (AP-HP)-CHU Pitié-Salpêtrière [APHP], Centre de Référence des Déficiences Intellectuelles de Causes Rares, Groupe de Recherche Clinique : Déficience Intellectuelle et Autisme ( GRC ), Université Pierre et Marie Curie - Paris 6 ( UPMC ), Centre hospitalier universitaire d'Amiens ( CHU Amiens-Picardie ), Assistance publique - Hôpitaux de Paris (AP-HP)-Université Paris 13 ( UP13 ) -Hôpital Jean Verdier, Institut de Génétique et Développement de Rennes ( IGDR ), Université de Rennes 1 ( UR1 ), Université de Rennes ( UNIV-RENNES ) -Université de Rennes ( UNIV-RENNES ) -Centre National de la Recherche Scientifique ( CNRS ) -Structure Fédérative de Recherche en Biologie et Santé de Rennes ( Biosit : Biologie - Santé - Innovation Technologique ), Génétique et épigénétique des maladies métaboliques, neurosensorielles et du développement ( Inserm U781 ), Université Paris Descartes - Paris 5 ( UPD5 ) -Institut National de la Santé et de la Recherche Médicale ( INSERM ), Cardiovasculaire, métabolisme, diabétologie et nutrition ( CarMeN ), Institut National de la Santé et de la Recherche Médicale ( INSERM ) -Hospices Civils de Lyon ( HCL ) -Institut National des Sciences Appliquées de Lyon ( INSA Lyon ), Université de Lyon-Institut National des Sciences Appliquées ( INSA ) -Université de Lyon-Institut National des Sciences Appliquées ( INSA ) -Université Claude Bernard Lyon 1 ( UCBL ), Université de Lyon-Institut National de la Recherche Agronomique ( INRA ), Université Joseph Fourier - Grenoble 1 ( UJF ) -Centre de Recherche en Nutrition Humaine Rhône - Alpes, Cancers et préventions, Université de Caen Normandie ( UNICAEN ), Normandie Université ( NU ) -Normandie Université ( NU ) -Institut National de la Santé et de la Recherche Médicale ( INSERM ), Centre Hospitalier Universitaire de Bordeaux, Service de Génétique Clinique et Université Lille 2, Centre Hospitalier Régional Universitaire [Lille] ( CHRU Lille ), Université Pierre et Marie Curie - Paris 6 - UFR de Médecine Pierre et Marie Curie ( UPMC ), Unité de Recherche sur les Maladies Cardiovasculaires, du Métabolisme et de la Nutrition = Institute of cardiometabolism and nutrition ( ICAN ), Université Pierre et Marie Curie - Paris 6 ( UPMC ) -Assistance publique - Hôpitaux de Paris (AP-HP)-Institut National de la Santé et de la Recherche Médicale ( INSERM ) -CHU Pitié-Salpêtrière [APHP], Centre de Recherche Saint-Antoine ( CR Saint-Antoine ), Institut National de la Santé et de la Recherche Médicale ( INSERM ) -Université Pierre et Marie Curie - Paris 6 ( UPMC ), Centre d'Investigation Clinique 1432 (Dijon) - Epidemiologie Clinique/Essais Cliniques ( CIC-EC ), Université de Bourgogne ( UB ) -Centre Hospitalier Universitaire de Dijon - Hôpital François Mitterrand ( CHU Dijon ) -Institut National de la Santé et de la Recherche Médicale ( INSERM ), University of California [San Francisco] (UC San Francisco), University of California (UC), Université de Rennes (UR)-Centre National de la Recherche Scientifique (CNRS)-Structure Fédérative de Recherche en Biologie et Santé de Rennes ( Biosit : Biologie - Santé - Innovation Technologique ), Université de Lyon-Institut National des Sciences Appliquées (INSA)-Institut National des Sciences Appliquées (INSA)-Hospices Civils de Lyon (HCL)-Institut National de la Santé et de la Recherche Médicale (INSERM), Centre Hospitalier Universitaire de Saint-Etienne [CHU Saint-Etienne] (CHU ST-E)-Institut National de la Recherche Agronomique (INRA)-Université Claude Bernard Lyon 1 (UCBL), Université de Lyon-Université de Lyon-CHU Grenoble-Hospices Civils de Lyon (HCL)-Université Jean Monnet - Saint-Étienne (UJM)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Université Grenoble Alpes [2016-2019] (UGA [2016-2019]), and Unité de Recherche sur les Maladies Cardiovasculaires, du Métabolisme et de la Nutrition = Research Unit on Cardiovascular and Metabolic Diseases (ICAN)
Subjects: short stature, lipoatrophy, intrauterine growth restriction, [ SDV ] Life Sciences [q-bio], [SDV.GEN.GH]Life Sciences [q-bio]/Genetics/Human genetics, [SDV]Life Sciences [q-bio], Diabetes, PIK3R1 gene, Insulin Resistance, [ SDV.GEN.GH ] Life Sciences [q-bio]/Genetics/Human genetics, SHORT syndrome
Abstract: International audience; SHORT syndrome has historically been defined by its acronym: short stature (S), hyperextensibility of joints and/or inguinal hernia (H), ocular depression (O), Rieger abnormality (R) and teething delay (T). More recently several research groups have identified PIK3R1 mutations as responsible for SHORT syndrome. Knowledge of the molecular etiology of SHORT syndrome has permitted a reassessment of the clinical phenotype. The detailed phenotypes of 32 individuals with SHORT syndrome and PIK3R1 mutation, including eight newly ascertained individuals, were studied to fully define the syndrome and the indications for PIK3R1 testing. The major features described in the SHORT acronym were not universally seen and only half (52%) had 4 or more of the classic features. The commonly observed clinical features of SHORT syndrome seen in the cohort included IUGR \textless 10(th) percentile, postnatal growth restriction, lipoatrophy and the characteristic facial gestalt. Anterior chamber defects and insulin resistance or diabetes were also observed but were not as prevalent. The less specific, or minor features of SHORT syndrome include teething delay, thin wrinkled skin, speech delay, sensorineural deafness, hyperextensibility of joints and inguinal hernia. Given the high risk of diabetes mellitus, regular monitoring of glucose metabolism is warranted. An echocardiogram, ophthalmological and hearing assessments are also recommended.
Published: 2015
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24. Mutation Update of the CLCN5 Gene Responsible for Dent Disease 1

Author: Patrick Niaudet, François Nobili, Guylhène Bourdat-Michel, Estelle Colin, Karin Dahan, Brigitte Gilbert-Dussardier, Claire Rigothier, Robert Novo, Dominique Martin-Coignard, Justine Bacchetta, Karim Bouchireb, Rémi Salomon, Thierry Hannedouche, Ariela Vergara-Jaque, Chantal Loirat, Dominique Chauveau, Pascal Houillier, Stéphane Burtey, Nicole Van Regemorter, Lamisse Mansour-Hendili, Sandrine Lemoine, Xavier Jeunemaitre, Muriel Holder-Espinasse, Michel Fischbach, Denis Morin, François-Guillaume Debray, S. Benoit, Valérie Leroy, Gilles Morin, Marie Alice Macher, Andreas Schleich, Mathilde Cailliez, Hassan Izzedine, Isabelle Roncelin, Gwenaëlle Roussey-Kesler, Cyrielle Treard, Claire Cartery, Hubert Nivet, Stéphane Lourdel, Véronique Baudouin, Pierre Cochat, Bertrand Knebelmann, Anne Blanchard, Wendy González, Estelle Desport, Tim Ulinski, Stella Dieguez, Marco Janner, Rosa Vargas-Poussou, Laurence Faivre, Alexandre Karras, Nelly Le Pottier, Francesco Emma, Philippe Vanhille, Renaud de la Faille, Anne Laure Sellier-Leclerc, J. Fourcade, Olivier Devuyst, Denis Fouque, Kenza Soulami, Aurélien Tiple, Etienne Bérard, Laurenne Dehoux, Marie Pierre Lavocat, Hélène François, Gérard Champion, Gerard Cardon, Georges Deschênes, Université Paris Descartes - Faculté de Médecine (UPD5 Médecine), Université Paris Descartes - Paris 5 (UPD5), Centre de Recherche des Cordeliers (CRC (UMR_S 872)), Université Paris Descartes - Paris 5 (UPD5)-Université Pierre et Marie Curie - Paris 6 (UPMC)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Centre National de la Recherche Scientifique (CNRS), Paris-Centre de Recherche Cardiovasculaire (PARCC - UMR-S U970), Hôpital Européen Georges Pompidou [APHP] (HEGP), Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Hôpitaux Universitaires Paris Ouest - Hôpitaux Universitaires Île de France Ouest (HUPO)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Hôpitaux Universitaires Paris Ouest - Hôpitaux Universitaires Île de France Ouest (HUPO)-Université Paris Descartes - Paris 5 (UPD5)-Institut National de la Santé et de la Recherche Médicale (INSERM), Centro de Bioinformática y Simulación Molecular, Universidad de Talca, Univ Talca, Escuela Ingn Bioinfomat, Talca, Chile, Institut d'histoire du temps présent (IHTP), Centre National de la Recherche Scientifique (CNRS), Service de génétique [Angers], Université d'Angers (UA)-Centre Hospitalier Universitaire d'Angers (CHU Angers), PRES Université Nantes Angers Le Mans (UNAM)-PRES Université Nantes Angers Le Mans (UNAM), Centre Hospitalier Régional Universitaire [Lille] (CHRU Lille), Centre de référence des maladies rénales rares Néphrogones [CHU-HCL, Lyon], Hôpital Femme Mère Enfant [CHU - HCL] (HFME), Hospices Civils de Lyon (HCL)-Hospices Civils de Lyon (HCL), Service de neuropédiatrie et maladies métaboliques [CHU Robert-Debré], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Hôpital Robert Debré, Centre de Référence des Maladies Rénales Héréditaires de l'Enfant et de l'Adulte (MARHEA), CHU Necker - Enfants Malades [AP-HP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP), Service de Néphrologie Pédiatrique, Centre Hospitalier Universitaire de Nice (CHU Nice)-Fondation LENVAL-Hopital pour Enfants, Vascular research center of Marseille (VRCM), Institut National de la Santé et de la Recherche Médicale (INSERM)-Aix Marseille Université (AMU), Hôpital de la Timone [CHU - APHM] (TIMONE), Département de Néphrologie et Transplantation d'organes, Hôpital de Rangueil, CHU Toulouse [Toulouse]-CHU Toulouse [Toulouse], Service de Néphrologie et Immunopathologie Clinique, Centre Hospitalier Universitaire de Toulouse, PRES Université de Toulouse, Département de Pédiatrie, Hôpital Edouard Herriot [CHU - HCL], Service de Néphrologie-Transplantation-Dialyse, CHU Bordeaux [Bordeaux]-Groupe hospitalier Pellegrin, Centre Hospitalier Universitaire de Liège (CHU-Liège), Unité de Néphrologie Pédiatrique, Centre hospitalier universitaire de Poitiers (CHU Poitiers), Division of Nephrology, Université catholique de Louvain Medical school, Université Catholique de Louvain = Catholic University of Louvain (UCL), Department of Nephrology and Urology, IRCCS Ospedale Pediatrico Bambino Gesù [Roma]-IRCCS, Service de Néphrologie pédiatrique [Hôpital de Hautepierre, Strasbourg], CHU Strasbourg-Hôpital de Hautepierre [Strasbourg], Cardiovasculaire, métabolisme, diabétologie et nutrition (CarMeN), Hospices Civils de Lyon (HCL)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Institut National des Sciences Appliquées de Lyon (INSA Lyon), Université de Lyon-Institut National des Sciences Appliquées (INSA)-Université de Lyon-Institut National des Sciences Appliquées (INSA)-Université Claude Bernard Lyon 1 (UCBL), Université de Lyon-Institut National de la Recherche Agronomique (INRA), Human-machine spoken dialogue (CORDIAL), Institut de Recherche en Informatique et Systèmes Aléatoires (IRISA), Université de Rennes 1 (UR1), Université de Rennes (UNIV-RENNES)-Université de Rennes (UNIV-RENNES)-Institut National des Sciences Appliquées - Rennes (INSA Rennes), Institut National des Sciences Appliquées (INSA)-Université de Rennes (UNIV-RENNES)-Institut National des Sciences Appliquées (INSA)-Institut National de Recherche en Informatique et en Automatique (Inria)-Centre National de la Recherche Scientifique (CNRS)-Université de Rennes 1 (UR1), Institut National des Sciences Appliquées (INSA)-Université de Rennes (UNIV-RENNES)-Institut National des Sciences Appliquées (INSA)-Institut National de Recherche en Informatique et en Automatique (Inria)-Centre National de la Recherche Scientifique (CNRS)-INRIA Rennes, Institut National de Recherche en Informatique et en Automatique (Inria)-École Nationale Supérieure des Sciences Appliquées et de Technologie (ENSSAT), Génétique Médicale, Centre hospitalier universitaire de Poitiers (CHU Poitiers)-Centre de Référence Anomalies du Développement Ouest, Service de néphrologie et hémodialyse [CHU de Strasbourg], CHU Strasbourg, Service de Physiologie [Georges-Pompidou], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Hôpital Européen Georges Pompidou [APHP] (HEGP), Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Hôpitaux Universitaires Paris Ouest - Hôpitaux Universitaires Île de France Ouest (HUPO)-Hôpitaux Universitaires Paris Ouest - Hôpitaux Universitaires Île de France Ouest (HUPO)-Université Paris Descartes - Paris 5 (UPD5), Service de Néphrologie [CHU Pitié-Salpêtrière], CHU Pitié-Salpêtrière [AP-HP], Sorbonne Université (SU)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP), Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Hôpitaux Universitaires Paris Ouest - Hôpitaux Universitaires Île de France Ouest (HUPO), Service de néphrologie adultes [CHU Necker], Sciences pour l'environnement (SPE), Centre National de la Recherche Scientifique (CNRS)-Université Pascal Paoli (UPP), Service de néphrologie pédiatrique, Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Hôpital Robert Debré-Université Paris Diderot - Paris 7 (UPD7), Laboratoire de génétique médicale et cytogénétique [Le Mans], Centre Hospitalier Le Mans (CH Le Mans), Métal, Travaux et recherches archéologiques sur les cultures, les espaces et les sociétés (TRACES), École des hautes études en sciences sociales (EHESS)-Université Toulouse - Jean Jaurès (UT2J)-Ministère de la Culture et de la Communication (MCC)-Centre National de la Recherche Scientifique (CNRS)-École des hautes études en sciences sociales (EHESS)-Université Toulouse - Jean Jaurès (UT2J)-Ministère de la Culture et de la Communication (MCC)-Centre National de la Recherche Scientifique (CNRS), Service de néphrologie pédiatrique [CHU Necker], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-CHU Necker - Enfants Malades [AP-HP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP), Service de Néphrologie, Hôpital de Clocheville, Service de néphrologie pédiatrique [CHRU Besançon], Centre Hospitalier Régional Universitaire de Besançon (CHRU Besançon), Unité Néphrologie Pédiatrique [CHRU Lille], Génétique des Anomalies du Développement (GAD), Université de Bourgogne (UB)-IFR100 - Structure fédérative de recherche Santé-STIC, Department of Nephrology, Transplantation and Dialysis, Bordeaux, Hospices Civils de Lyon (HCL), Service de Néphrologie Dialyse Transplantation, CHU Ibn Rochd [Casablanca], CHU Clermont-Ferrand, Service de néphrologie et pédiatrie générale [CHU Trousseau], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU)-CHU Trousseau [APHP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU), Service de médecine interne et néphrologie, CH Valenciennes, Centre de Génétique de Bruxelles, Université libre de Bruxelles (ULB), Service de Génétique [CHU HEGP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Hôpitaux Universitaires Paris Ouest - Hôpitaux Universitaires Île de France Ouest (HUPO)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Hôpitaux Universitaires Paris Ouest - Hôpitaux Universitaires Île de France Ouest (HUPO), French Ministry of Health (Plan Maladies Rares), European Community FP7EUNEFRON 201590 and EURenOmics 2012‐305608, The Chilean PIA‐Conycit program (grant ACT‐1104), The Swiss National Science Foundation project (grant 310030_146490), RADIZ, Rare Diseases Initiative Zürich, a KFSP of the University of Zurich., Université Pierre et Marie Curie - Paris 6 (UPMC)-Université Paris Descartes - Paris 5 (UPD5)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Centre National de la Recherche Scientifique (CNRS), Centre de Référence des Maladies Rénales Héréditaires de l'Enfant et de l'Adulte [CHU-Necker] (MARHEA), Aix Marseille Université (AMU)-Institut National de la Santé et de la Recherche Médicale (INSERM), Département de Néphrologie et Transplantation d'organes [CHU Toulouse], Pôle Urologie - Néphrologie - Dialyse - Transplantations - Brûlés - Chirurgie plastique - Explorations fonctionnelles et physiologiques [CHU Toulouse], Centre Hospitalier Universitaire de Toulouse (CHU Toulouse)-Centre Hospitalier Universitaire de Toulouse (CHU Toulouse), Service Médecine interne et immunopathologie clinique [CHU Toulouse], Pôle IUCT [CHU Toulouse], Institut National de la Recherche Agronomique (INRA)-Université Claude Bernard Lyon 1 (UCBL), Université de Lyon-Université de Lyon-Institut National des Sciences Appliquées de Lyon (INSA Lyon), Université de Lyon-Institut National des Sciences Appliquées (INSA)-Institut National des Sciences Appliquées (INSA)-Hospices Civils de Lyon (HCL)-Institut National de la Santé et de la Recherche Médicale (INSERM), Université de Rennes (UR)-Institut National des Sciences Appliquées - Rennes (INSA Rennes), Institut National des Sciences Appliquées (INSA)-Institut National des Sciences Appliquées (INSA)-Institut National de Recherche en Informatique et en Automatique (Inria)-Centre National de la Recherche Scientifique (CNRS)-Université de Rennes (UR)-Institut National des Sciences Appliquées - Rennes (INSA Rennes), Institut National des Sciences Appliquées (INSA)-Institut National des Sciences Appliquées (INSA)-Institut National de Recherche en Informatique et en Automatique (Inria)-Centre National de la Recherche Scientifique (CNRS)-INRIA Rennes, Université Pascal Paoli (UPP)-Centre National de la Recherche Scientifique (CNRS), École des hautes études en sciences sociales (EHESS)-Université Toulouse - Jean Jaurès (UT2J), Université de Toulouse (UT)-Université de Toulouse (UT)-Ministère de la Culture et de la Communication (MCC)-Centre National de la Recherche Scientifique (CNRS)-École des hautes études en sciences sociales (EHESS)-Université Toulouse - Jean Jaurès (UT2J), Université de Toulouse (UT)-Université de Toulouse (UT)-Ministère de la Culture et de la Communication (MCC)-Centre National de la Recherche Scientifique (CNRS), CHU Trousseau [APHP], Centre de Recherche des Cordeliers (CRC), Université Paris Diderot - Paris 7 (UPD7)-École pratique des hautes études (EPHE)-Université Pierre et Marie Curie - Paris 6 (UPMC)-Université Paris Descartes - Paris 5 (UPD5)-Institut National de la Santé et de la Recherche Médicale (INSERM), Centre de Recherche des Cordeliers (CRC (UMR_S_1138 / U1138)), École pratique des hautes études (EPHE)-Université Paris Diderot - Paris 7 (UPD7)-Université Paris Descartes - Paris 5 (UPD5)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Sorbonne Université (SU), Max Planck Institute for Biophysical Chemistry (MPI-BPC), Max-Planck-Gesellschaft, Service de Génétique [AP-HP Hôpital Européen Georges Pompidou, Paris], Hôpitaux Universitaires Paris Ouest - Hôpitaux Universitaires Île de France Ouest (HUPO)-Assistance publique - Hôpitaux de Paris (AP-HP) (APHP)-Hôpitaux Universitaires Paris Ouest - Hôpitaux Universitaires Île de France Ouest (HUPO)-Assistance publique - Hôpitaux de Paris (AP-HP) (APHP), Hôpitaux Universitaires Paris Ouest - Hôpitaux Universitaires Île de France Ouest (HUPO)-Assistance publique - Hôpitaux de Paris (AP-HP) (APHP)-Hôpitaux Universitaires Paris Ouest - Hôpitaux Universitaires Île de France Ouest (HUPO)-Assistance publique - Hôpitaux de Paris (AP-HP) (APHP)-Université Paris Descartes - Paris 5 (UPD5)-Institut National de la Santé et de la Recherche Médicale (INSERM), Assistance publique - Hôpitaux de Paris (AP-HP) (APHP)-Hôpital Robert Debré, Université Catholique de Louvain (UCL), IRCCS-IRCCS Ospedale Pediatrico Bambino Gesù [Roma], Université de Lyon-Institut National des Sciences Appliquées (INSA)-Institut National des Sciences Appliquées (INSA)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Hospices Civils de Lyon (HCL), Assistance publique - Hôpitaux de Paris (AP-HP) (APHP)-Hôpital Européen Georges Pompidou [APHP] (HEGP), Hôpitaux Universitaires Paris Ouest - Hôpitaux Universitaires Île de France Ouest (HUPO)-Assistance publique - Hôpitaux de Paris (AP-HP) (APHP)-Hôpitaux Universitaires Paris Ouest - Hôpitaux Universitaires Île de France Ouest (HUPO)-Université Paris Descartes - Paris 5 (UPD5), Service de néphrologie [CHU Pitié-Salpêtrière], Assistance publique - Hôpitaux de Paris (AP-HP) (APHP)-CHU Pitié-Salpêtrière [APHP], Hôpitaux Universitaires Paris Ouest - Hôpitaux Universitaires Île de France Ouest (HUPO)-Assistance publique - Hôpitaux de Paris (AP-HP) (APHP), Assistance publique - Hôpitaux de Paris (AP-HP) (APHP)-CHU Necker - Enfants Malades [AP-HP], Assistance publique - Hôpitaux de Paris (AP-HP) (APHP)-Hôpital Robert Debré-Université Paris Diderot - Paris 7 (UPD7), CHU Necker - Enfants Malades [AP-HP]-Assistance publique - Hôpitaux de Paris (AP-HP) (APHP), Service de nephrologie pédiatrique, Centre Hospitalier Régional Universitaire [Besançon] (CHRU Besançon), Assistance publique - Hôpitaux de Paris (AP-HP) (APHP)-CHU Trousseau [APHP]-Sorbonne Université (SU), Free University of Brussels (BELGIUM), Université Paris Descartes - Paris 5 (UPD5)-Hôpital Européen Georges Pompidou [APHP] (HEGP), Hôpitaux Universitaires Paris Ouest - Hôpitaux Universitaires Île de France Ouest (HUPO)-Assistance publique - Hôpitaux de Paris (AP-HP) (APHP)-Hôpitaux Universitaires Paris Ouest - Hôpitaux Universitaires Île de France Ouest (HUPO)-Assistance publique - Hôpitaux de Paris (AP-HP) (APHP)-Institut National de la Santé et de la Recherche Médicale (INSERM), Physiopathologie Cardiovasculaire et Mitochondriale (MITOVASC), Université d'Angers (UA)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Centre National de la Recherche Scientifique (CNRS), Centre Hospitalier Lyon Sud [CHU - HCL] (CHLS), IFR100 - Structure fédérative de recherche Santé-STIC-Université de Bourgogne (UB), Centre de génétique - Centre de référence des maladies rares, anomalies du développement et syndromes malformatifs (CHU de Dijon), Centre Hospitalier Universitaire de Dijon - Hôpital François Mitterrand (CHU Dijon), Néphrologie, Centre Hospitalier Universitaire (CHU), Institut National des Sciences Appliquées (INSA)-Université de Lyon-Institut National des Sciences Appliquées (INSA)-Université de Lyon-Université Claude Bernard Lyon 1 (UCBL), Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-CHU Pitié-Salpêtrière [AP-HP], Sorbonne Université (SU)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU), Université Pierre et Marie Curie - Paris 6 (UPMC)-École pratique des hautes études (EPHE), Université Paris sciences et lettres (PSL)-Université Paris sciences et lettres (PSL)-Université Paris Diderot - Paris 7 (UPD7)-Université Paris Descartes - Paris 5 (UPD5)-Institut National de la Santé et de la Recherche Médicale (INSERM), École pratique des hautes études (EPHE), Université Paris sciences et lettres (PSL)-Université Paris sciences et lettres (PSL)-Université Paris Diderot - Paris 7 (UPD7)-Université Paris Descartes - Paris 5 (UPD5)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Sorbonne Université (SU), Université Paris Descartes - Paris 5 (UPD5)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Hôpital Européen Georges Pompidou [APHP] (HEGP), Assistance publique - Hôpitaux de Paris (AP-HP) (APHP)-Hôpitaux Universitaires Paris Ouest - Hôpitaux Universitaires Île de France Ouest (HUPO)-Assistance publique - Hôpitaux de Paris (AP-HP) (APHP)-Hôpitaux Universitaires Paris Ouest - Hôpitaux Universitaires Île de France Ouest (HUPO), Institut de biologie de Lille - IBL (IBLI), Université de Lille, Sciences et Technologies-Institut Pasteur de Lille, Réseau International des Instituts Pasteur (RIIP)-Réseau International des Instituts Pasteur (RIIP)-Centre National de la Recherche Scientifique (CNRS)-Université de Lille, Droit et Santé, Assistance publique - Hôpitaux de Paris (AP-HP) (APHP)-Hôpitaux Universitaires Paris Ouest - Hôpitaux Universitaires Île de France Ouest (HUPO)-Hôpitaux Universitaires Paris Ouest - Hôpitaux Universitaires Île de France Ouest (HUPO)-Université Paris Descartes - Paris 5 (UPD5), and Assistance publique - Hôpitaux de Paris (AP-HP) (APHP)-Hôpitaux Universitaires Paris Ouest - Hôpitaux Universitaires Île de France Ouest (HUPO)
Subjects: Male, chloride channel, renal failure, [SDV]Life Sciences [q-bio], 030232 urology & nephrology, Dent Disease, ClC-5, medicine.disease_cause, Cohort Studies, Mice, 0302 clinical medicine, Missense mutation, MESH: Animals, MESH: DNA Mutational Analysis, low molecular weight proteinuria, Genetics (clinical), Genetics, Mice, Knockout, 0303 health sciences, Mutation, biology, MESH: Genetic Predisposition to Disease, MESH: Anion Transport Proteins, Phenotype, 3. Good health, Pedigree, Chloride Channels/chemistry/*genetics/metabolism, Dent Disease/*genetics/metabolism, Nephrocalcinosis, MESH: Bartter Syndrome, MESH: Mutation, Knockout, [SDV.BC]Life Sciences [q-bio]/Cellular Biology, 03 medical and health sciences, Tubulopathy, Chloride Channels, medicine, Animals, Humans, Genetic Association Studies, 030304 developmental biology, MESH: Humans, CLCN5, MESH: Chloride Channels, Dent disease 1, medicine.disease, Bartter syndrome, biology.protein, OCRL, CLC family of chloride transporters and channels
Abstract: International audience; Dent disease is a rare X-linked tubulopathy characterized by low molecular weight proteinuria, hypercalciuria, nephrocalcinosis and/or nephrolithiasis, progressive renal failure, and variable manifestations of other proximal tubule dysfunctions. It often progresses over a few decades to chronic renal insufficiency, and therefore molecular characterization is important to allow appropriate genetic counseling. Two genetic subtypes have been described to date: Dent disease 1 is caused by mutations of the CLCN5 gene, coding for the chloride/proton exchanger ClC-5; and Dent disease 2 by mutations of the OCRL gene, coding for the inositol polyphosphate 5-phosphatase OCRL-1. Herein, we review previously reported mutations (n = 192) and their associated phenotype in 377 male patients with Dent disease 1 and describe phenotype and novel (n = 42) and recurrent mutations (n = 24) in a large cohort of 117 Dent disease 1 patients belonging to 90 families. The novel missense and in-frame mutations described were mapped onto a three-dimensional homology model of the ClC-5 protein. This analysis suggests that these mutations affect the dimerization process, helix stability, or transport. The phenotype of our cohort patients supports and extends the phenotype that has been reported in smaller studies.
Published: 2015
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25. Treatment of Systemic Necrotizing Vasculitides in Patients Aged Sixty-Five Years or Older: Results of a Multicenter, Open-Label, Randomized Controlled Trial of Corticosteroid and Cyclophosphamide-Based Induction Therapy

Author: Pagnoux, Christian, Quéméneur, Thomas, Ninet, Jacques, Diot, Elisabeth, Kyndt, Xavier, de Wazières, Benoît, Reny, Jean-Luc, Puéchal, Xavier, le Berruyer, Pierre-Yves, Lidove, Olivier, Vanhille, Philippe, Godmer, Pascal, Fain, Olivier, Blockmans, Daniel, Bienvenu, Boris, Rollot, Florence, Aït el Ghaz-Poignant, Séverine, Mahr, Alfred, Cohen, Pascal, Mouthon, Luc, Perrodeau, Elodie, Ravaud, Philippe, Guillevin, Loïc, French Vasculitis Study Group, Service de médecine interne et centre de référence des maladies rares [CHU Cochin], CHU Cochin [AP-HP]-Assistance publique - Hôpitaux de Paris (AP-HP) (APHP), Université Paris Descartes - Paris 5 (UPD5), CH Valenciennes, Hôpital Edouard Herriot [CHU - HCL], Hospices Civils de Lyon (HCL), Centre Hospitalier Régional Universitaire de Tours (CHRU TOURS), Centre Hospitalier Régional Universitaire de Nîmes (CHRU Nîmes), Université de Montpellier (UM), Centre Hospitalier de Béziers, CHU Cochin [AP-HP], Hôpital Robert Debré, Hôpital Robert Debré-Centre Hospitalier Universitaire de Reims (CHU Reims), AP-HP - Hôpital Bichat - Claude Bernard [Paris], Centre Hospitalier de Bretagne Atlantique, Hôpital Jean Verdier [Bondy], Assistance publique - Hôpitaux de Paris (AP-HP) (APHP), University Hospital Gasthuisberg, Centre d'épidémiologie Clinique [Hôtel-Dieu], Assistance publique - Hôpitaux de Paris (AP-HP) (APHP)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Hôpital Hôtel Dieu, Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Hôpital Cochin [AP-HP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP), Centre Hospitalier Universitaire de Nîmes (CHU Nîmes), Hôpital Cochin [AP-HP], Centre hospitalier Bretagne Atlantique (Morbihan) (CHBA), Hôpital Jean Verdier [AP-HP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Hôpital Hôtel Dieu, and Centre Hospitalier Régional Universitaire de Tours (CHRU Tours)
Subjects: Male, Methotrexate/therapeutic use, macromolecular substances, Adrenal Cortex Hormones/therapeutic use, Drug Administration Schedule, stomatognathic system, Adrenal Cortex Hormones, hemic and lymphatic diseases, Azathioprine, Humans, cardiovascular diseases, Cyclophosphamide, Aged, Aged, 80 and over, Remission Induction/methods, Remission Induction, Systemic Vasculitis, Systemic Vasculitis/drug therapy, Methotrexate, Treatment Outcome, [SDV.MHEP.RSOA]Life Sciences [q-bio]/Human health and pathology/Rhumatology and musculoskeletal system, Cyclophosphamide/therapeutic use, ddc:618.97, Drug Therapy, Combination, Female, Azathioprine/therapeutic use
Abstract: International audience; OBJECTIVE:To investigate a new therapeutic strategy, with rapid corticosteroid dose tapering and limited cyclophosphamide (CYC) exposure, for older patients with systemic necrotizing vasculitides (SNVs; polyarteritis nodosa [PAN], granulomatosis with polyangiitis [Wegnener's] [GPA], microscopic polyangiitis [MPA], or eosinophilic GPA [Churg-Strauss] [EGPA]).METHODS:A multicenter, open-label, randomized controlled trial comprising patients ≥65 years old and newly diagnosed as having SNV was conducted. The experimental treatment consisted of corticosteroids for ∼9 months and a maximum of six 500-mg fixed-dose intravenous (IV) CYC pulses, every 2-3 weeks, then maintenance azathioprine or methotrexate. The control treatment included ∼26 months of corticosteroids for all patients, combined with 500 mg/m(2) IV CYC pulses, every 2-3 weeks until remission, then maintenance for all patients with GPA or MPA and for those with EGPA or PAN with a Five-Factors Score (FFS) of ≥1. Randomization used a 1:1 ratio computer-generated list and was performed centrally with sealed opaque envelopes. The primary outcome measure was ≥1 serious adverse event (SAE) occurring within 3 years of followup. Secondary outcome measures included remission and relapse rates.RESULTS:Among the 108 patients randomized, 4 were excluded (early consent withdrawal or protocol violation). Mean ± SD age at diagnosis was 75.2 ± 6.3 years. Analysis at 3 years included 53 patients (21 GPA, 21 MPA, 8 EGPA, and 3 PAN) in the experimental arm and 51 patients (15 GPA, 23 MPA, 6 EGPA, and 7 PAN) in the conventional arm. In total, 32 (60%) versus 40 (78%) had ≥1 SAE (P = 0.04), most frequently infections; 6 (11%) versus 7 (14%) failed to achieve remission (P = 0.71); 9 (17%) versus 12 (24%) died (P = 0.41); and 20 (44%) of 45 versus 12 (29%) of 41 survivors in remission experienced a relapse (P = 0.15).CONCLUSION:For older SNV patients, an induction regimen limiting corticosteroid exposure and with fixed low-dose IV CYC pulses reduces SAEs in comparison to conventional therapy, and does not affect the remission rate. Three-year relapse rates remain high for both arms.TRIAL REGISTRATION:ClinicalTrials.gov NCT00307671.
Published: 2015
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26. Bullous pemphigoid induced by vildagliptin: a report of three cases

Author: Aurélie Jacobsoone, Annie Vermersch, Johana Béné, Anne-Fleur Tronquoy, Marlène Vonarx, Marine Auffret, Dominique Hillaire-Buys, Patrick Coupe, Samy Babai, Sophie Gautier, Marie-Josèphe Jean-Pastor, Centre Hospitalier Régional Universitaire [Lille] (CHRU Lille), Hôpital Claude Huriez [Lille], CHU Lille, CH Valenciennes, Hôpital Henri Mondor, Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Hôpital Henri Mondor-Université Paris-Est Créteil Val-de-Marne - Paris 12 (UPEC UP12), Centre Hospitalier Régional Universitaire [Montpellier] (CHRU Montpellier), Pathogénèse et contrôle des infections chroniques (PCCI), Institut National de la Santé et de la Recherche Médicale (INSERM)-Université de Montpellier (UM)-Centre Hospitalier Universitaire de Montpellier (CHU Montpellier ), Hôpital Salvator [CHU - APHM] (Hôpitaux Sud), and Herrada, Anthony
Subjects: Male, bullous pemphigoid, medicine.medical_specialty, Pyrrolidines, 030209 endocrinology & metabolism, Adamantane, 030207 dermatology & venereal diseases, 03 medical and health sciences, 0302 clinical medicine, Nitriles, Pemphigoid, Bullous, medicine, Humans, Pharmacology (medical), Vildagliptin, Gliclazide, adverse reactions, skin and connective tissue diseases, Aged, Pharmacology, Aged, 80 and over, Dipeptidyl-Peptidase IV Inhibitors, integumentary system, business.industry, Type 2 Diabetes Mellitus, medicine.disease, Dermatology, eye diseases, 3. Good health, Discontinuation, Metformin, [SDV.SP.PHARMA] Life Sciences [q-bio]/Pharmaceutical sciences/Pharmacology, Clobetasol, Sitagliptin, drug-induced skin disorder, gliptins, [SDV.SP.PHARMA]Life Sciences [q-bio]/Pharmaceutical sciences/Pharmacology, Female, Bullous pemphigoid, business, medicine.drug
Abstract: International audience; To report three cases of bullous pemphigoid in patients treated with vildagliptin. Case 1: An 86-year-old woman presented with bullous pemphigoid after 1 month of treatment with vildagliptin and metformin. After introduction of clobetasol, the symptoms resolved although vildagliptin was continued. However, the skin lesions reappeared 3 months later. Sustained remission was achieved only after definitive withdrawal of vildagliptin. Case 2: A 79-year-old man presented with bullous pemphigoid after 37-month treatment with gliclazide, vildagliptin and metformin. The disease at first responded to clobetasol but 3 months later the lesions reappeared. They finally regressed when the gliptin was discontinued. Case 3: A 77-year-old woman, treated with gliclazide and vildagliptin for 26 months, presented with bullous pemphigoid, which responded well to discontinuation of the gliptin and topical clobetasol. Gliptins are new molecules for treatment of type 2 diabetes mellitus, which have been suspected of implication in bullous pemphigoid. Such cases have been described in the literature (seven with vildagliptin and three with sitagliptin). In nine of these cases, the gliptin was associated with metformin, but the latter had never been considered responsible. The mechanism implicated in the development of bullous pemphigoid has not yet been clearly identified, but may involve a modified immune response or alteration of the antigenic properties of the epidermal basement membrane. These reports support the risk of bullous pemphigoid in patients exposed to gliptins.
Published: 2015
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27. Spectrum and Prognosis of Noninfectious Renal Mixed Cryoglobulinemic GN

Author: Mohamad, Zaidan, Benjamin, Terrier, Agnieszka, Pozdzik, Thierry, Frouget, Nathalie, Rioux-Leclercq, Christian, Combe, Sébastien, Lepreux, Aurélie, Hummel, Laure-Hélène, Noël, Isabelle, Marie, Bruno, Legallicier, Arnaud, François, Antoine, Huart, David, Launay, Gilles, Kaplanski, Frank, Bridoux, Philippe, Vanhille, Raifah, Makdassi, Jean-François, Augusto, Philippe, Rouvier, Alexandre, Karras, Chantal, Jouanneau, Marie-Christine, Verpont, Patrice, Callard, Fabrice, Carrat, Olivier, Hermine, Jean-Marc, Léger, Xavier, Mariette, Patricia, Senet, David, Saadoun, Pierre, Ronco, Isabelle, Brochériou, Patrice, Cacoub, Emmanuelle, Plaisier, Thierry, Zénone, CHU Necker - Enfants Malades [AP-HP], Departement Hospitalo- Universitaire - Inflammation, Immunopathologie, Biothérapie [Paris] (DHU - I2B), Université Pierre et Marie Curie - Paris 6 (UPMC)-CHU Pitié-Salpêtrière [APHP], Service de médecine interne et centre de référence des maladies rares [CHU Cochin], CHU Cochin [AP-HP]-Assistance publique - Hôpitaux de Paris (AP-HP) (APHP), Department of Nephrology - Dialysis and Renal Transplantation, Hôpital Erasmes, Laboratory of Experimental Nephrology - Department of Biochimie, Université Libre de Bruxelles [Bruxelles] (ULB), Service de néphrologie, Université de Rennes 1 (UR1), Université de Rennes (UNIV-RENNES)-Université de Rennes (UNIV-RENNES)-Hôpital Pontchaillou-CHU Pontchaillou [Rennes], Institut de Génétique et Développement de Rennes (IGDR), Université de Rennes (UNIV-RENNES)-Université de Rennes (UNIV-RENNES)-Centre National de la Recherche Scientifique (CNRS)-Structure Fédérative de Recherche en Biologie et Santé de Rennes ( Biosit : Biologie - Santé - Innovation Technologique ), Service de Néphrologie-transplantation-dialyse [Bordeaux], CHU Bordeaux [Bordeaux], Centre de compétences des microangiopathies thrombotiques, Université Bordeaux Segalen - Bordeaux 2, Centre de recherche Croissance et signalisation (UMR_S 845), Université Paris Descartes - Paris 5 (UPD5)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Centre National de la Recherche Scientifique (CNRS), Service d'anatomie pathologique [CHU Necker], Assistance publique - Hôpitaux de Paris (AP-HP) (APHP)-CHU Necker - Enfants Malades [AP-HP], Service de Médecine Interne [Rouen], CHU Rouen, Normandie Université (NU)-Normandie Université (NU)-Université de Rouen Normandie (UNIROUEN), Normandie Université (NU), Service d'Anatomie et Cytologie Pathologique [Rouen], Service de Néphrologie et Immunopathologie Clinique, Centre Hospitalier Universitaire de Toulouse, PRES Université de Toulouse, Laboratoire d'Immunologie (EA 2686), Université de Lille, Droit et Santé, Inflammation: mécanismes et régulation et interactions avec la nutrition et les candidoses, Institut National de la Santé et de la Recherche Médicale (INSERM)-Université de Lille, Droit et Santé, Service de Médecine Interne et d'Immunologie clinique, Centre National de Référence Maladies rares-Centre Hospitalier Régional Universitaire [Lille] (CHRU Lille), Service de néphrologie - hémodialyse et transplantation rénale, Centre hospitalier universitaire de Poitiers (CHU Poitiers), Service de médecine interne et néphrologie, CH Valenciennes, Université d'Angers - Faculté de médecine (UA UFR Médecine), Centre Hospitalier Universitaire d'Angers (CHU Angers), PRES Université Nantes Angers Le Mans (UNAM)-PRES Université Nantes Angers Le Mans (UNAM)-Université d'Angers (UA), Service de Néphrologie et Hémodialyse [CHU HEGP], Assistance publique - Hôpitaux de Paris (AP-HP) (APHP)-Hôpital Européen Georges Pompidou [APHP] (HEGP), Hôpitaux Universitaires Paris Ouest - Hôpitaux Universitaires Île de France Ouest (HUPO)-Assistance publique - Hôpitaux de Paris (AP-HP) (APHP)-Hôpitaux Universitaires Paris Ouest - Hôpitaux Universitaires Île de France Ouest (HUPO), Des Maladies Rénales Rares aux Maladies Fréquentes, Remodelage et Réparation, Université Pierre et Marie Curie - Paris 6 (UPMC)-Institut National de la Santé et de la Recherche Médicale (INSERM), CHU Tenon [APHP], Institut Pierre Louis d'Epidémiologie et de Santé Publique (iPLESP), CHU Saint-Antoine [APHP], Centre de référence des mastocytoses, Service d'Hématologie Adulte, Imagine - Institut des maladies génétiques (IMAGINE - U1163), Centre d'Immunologie et de Maladies Infectieuses (CIMI), Université Pierre et Marie Curie - Paris 6 (UPMC)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Centre National de la Recherche Scientifique (CNRS), Régulation de la réponse immune, infection VIH-1 et autoimmunité, Université Paris-Sud - Paris 11 (UP11)-Institut National de la Santé et de la Recherche Médicale (INSERM), Service de Dermatologie [CHU Tenon], Assistance publique - Hôpitaux de Paris (AP-HP) (APHP)-CHU Tenon [APHP], Immunologie - Immunopathologie - Immunothérapie (I3), Centre National de la Recherche Scientifique (CNRS)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Université Pierre et Marie Curie - Paris 6 (UPMC), Service d'immunologie [CHU Pitié-Salpétrière], Assistance publique - Hôpitaux de Paris (AP-HP) (APHP)-CHU Pitié-Salpêtrière [APHP], Sorbonne Universités, Service de Néphrologie et Dialyses [CHU Tenon], Departement Hospitalo- Universitaire - Inflammation, Immunopathologie, Biothérapie [Paris] ( DHU - I2B ), Université Pierre et Marie Curie - Paris 6 ( UPMC ) -CHU Pitié-Salpêtrière [APHP], Assistance publique - Hôpitaux de Paris (AP-HP)-CHU Cochin [AP-HP], Université Libre de Bruxelles [Bruxelles] ( ULB ), Université de Rennes 1 ( UR1 ), Université de Rennes ( UNIV-RENNES ) -Université de Rennes ( UNIV-RENNES ) -Hôpital Pontchaillou-CHU Pontchaillou [Rennes], Institut de Génétique et Développement de Rennes ( IGDR ), Université de Rennes ( UNIV-RENNES ) -Université de Rennes ( UNIV-RENNES ) -Centre National de la Recherche Scientifique ( CNRS ) -Structure Fédérative de Recherche en Biologie et Santé de Rennes ( Biosit : Biologie - Santé - Innovation Technologique ), Centre de recherche Croissance et signalisation ( UMR_S 845 ), Université Paris Descartes - Paris 5 ( UPD5 ) -Institut National de la Santé et de la Recherche Médicale ( INSERM ) -Centre National de la Recherche Scientifique ( CNRS ), CHU Necker - Enfants Malades [AP-HP]-Assistance publique - Hôpitaux de Paris (AP-HP), CHU Rouen-Université de Rouen Normandie ( UNIROUEN ), Normandie Université ( NU ) -Normandie Université ( NU ), Laboratoire d'Immunologie ( EA 2686 ), Institut National de la Santé et de la Recherche Médicale ( INSERM ) -Université de Lille, Droit et Santé, Centre National de Référence Maladies rares-Centre Hospitalier Régional Universitaire [Lille] ( CHRU Lille ), Université de Poitiers-Centre hospitalier universitaire de Poitiers ( CHU Poitiers ), Centre de Recherche en Cancérologie / Nantes - Angers ( CRCNA ), CHU Angers-Centre hospitalier universitaire de Nantes ( CHU Nantes ) -Hôtel-Dieu de Nantes-Institut National de la Santé et de la Recherche Médicale ( INSERM ) -Hôpital Laennec-Centre National de la Recherche Scientifique ( CNRS ) -Faculté de Médecine d'Angers, Université d'Angers - Faculté de médecine ( UA UFR Médecine ), Université d'Angers ( UA ) -CHU Angers, Assistance publique - Hôpitaux de Paris (AP-HP)-Hôpital Européen Georges Pompidou [APHP] ( HEGP ), Université Pierre et Marie Curie - Paris 6 ( UPMC ) -Institut National de la Santé et de la Recherche Médicale ( INSERM ), Service de Pathologie, Assistance publique - Hôpitaux de Paris (AP-HP)-CHU Tenon [APHP], Institut Pierre Louis d'Epidémiologie et de Santé Publique ( iPLESP ), Institut National de la Santé et de la Recherche Médicale ( INSERM ) -Université Pierre et Marie Curie - Paris 6 ( UPMC ), Département de santé publique, Assistance publique - Hôpitaux de Paris (AP-HP)-CHU Necker - Enfants Malades [AP-HP], Imagine - Institut des maladies génétiques ( IMAGINE - U1163 ), Centre d'Immunologie et de Maladies Infectieuses ( CIMI ), Université Pierre et Marie Curie - Paris 6 ( UPMC ) -Institut National de la Santé et de la Recherche Médicale ( INSERM ) -Centre National de la Recherche Scientifique ( CNRS ), Université Paris-Sud - Paris 11 ( UP11 ) -Institut National de la Santé et de la Recherche Médicale ( INSERM ), Immunologie - Immunopathologie - Immunothérapie ( I3 ), Assistance publique - Hôpitaux de Paris (AP-HP)-CHU Pitié-Salpêtrière [APHP], Anatomopathologie, Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP), Université Pierre et Marie Curie - Paris 6 (UPMC)-CHU Pitié-Salpêtrière [AP-HP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP), Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Hôpital Cochin [AP-HP], Université libre de Bruxelles (ULB), Service de néphrologie [Rennes], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-CHU Necker - Enfants Malades [AP-HP], Service de Médecine Interne [CHU Rouen], Service d'Anatomie et Cytologie Pathologique [CHU Rouen], Université d'Angers (UA)-Centre Hospitalier Universitaire d'Angers (CHU Angers), PRES Université Nantes Angers Le Mans (UNAM)-PRES Université Nantes Angers Le Mans (UNAM), Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Hôpital Européen Georges Pompidou [APHP] (HEGP), Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Hôpitaux Universitaires Paris Ouest - Hôpitaux Universitaires Île de France Ouest (HUPO)-Hôpitaux Universitaires Paris Ouest - Hôpitaux Universitaires Île de France Ouest (HUPO), CHU Tenon [AP-HP], CHU Saint-Antoine [AP-HP], Centre de référence des mastocytoses (CEREMAST), Université Paris Descartes - Paris 5 (UPD5)-Institut National de la Santé et de la Recherche Médicale (INSERM), Service de dermatologie et allergologie [CHU Tenon], Service d'Immunologie [CHU Pitié-Salpétrière], CHU Pitié-Salpêtrière [AP-HP], Sorbonne Université (SU), Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-CHU Tenon [AP-HP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU)-Université Pierre et Marie Curie - Paris 6 (UPMC), Université de Rouen Normandie (UNIROUEN), Normandie Université (NU)-Normandie Université (NU)-CHU Rouen, Institut National de la Santé et de la Recherche Médicale (INSERM)-Université Pierre et Marie Curie - Paris 6 (UPMC), Sorbonne Université (SU)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP), Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU), Sorbonne Université (SU)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP), Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU), Sorbonne Université (SU)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU), Université de Rennes (UR)-Hôpital Pontchaillou-CHU Pontchaillou [Rennes], Université de Rennes (UR)-Centre National de la Recherche Scientifique (CNRS)-Structure Fédérative de Recherche en Biologie et Santé de Rennes ( Biosit : Biologie - Santé - Innovation Technologique ), Service Médecine interne et immunopathologie clinique [CHU Toulouse], Pôle IUCT [CHU Toulouse], Centre Hospitalier Universitaire de Toulouse (CHU Toulouse)-Centre Hospitalier Universitaire de Toulouse (CHU Toulouse), CHU Pitié-Salpêtrière [APHP]-Université Pierre et Marie Curie - Paris 6 (UPMC), Assistance publique - Hôpitaux de Paris (AP-HP) (APHP)-CHU Cochin [AP-HP], Université de Lille, Droit et Santé-Institut National de la Santé et de la Recherche Médicale (INSERM), and Assistance publique - Hôpitaux de Paris (AP-HP) (APHP)-Hôpitaux Universitaires Paris Ouest - Hôpitaux Universitaires Île de France Ouest (HUPO)-Hôpitaux Universitaires Paris Ouest - Hôpitaux Universitaires Île de France Ouest (HUPO)
Subjects: Male, medicine.medical_specialty, Pathology, Cyclophosphamide, Glomerulonephritis, Membranoproliferative, [SDV]Life Sciences [q-bio], 030232 urology & nephrology, Lymphoproliferative disorders, Gastroenterology, 03 medical and health sciences, 0302 clinical medicine, Clinical Research, Internal medicine, medicine, Humans, Cause of death, Retrospective Studies, 030203 arthritis & rheumatology, Proteinuria, [ SDV ] Life Sciences [q-bio], business.industry, General Medicine, membranoproliferative GN, Middle Aged, medicine.disease, Prognosis, Cryoglobulinemia, 3. Good health, Lymphoma, Regimen, Nephrology, histopathology, Rituximab, Female, medicine.symptom, business, Immunosuppressive Agents, medicine.drug
Abstract: International audience; Noninfectious mixed cryoglobulinemic GN (MCGN) has been poorly investigated. We analyzed presentation and outcome of 80 patients with biopsy-proven MCGN, which were identified in the retrospective French CryoVas survey. MCGN was related to primary Sjögren's syndrome in 22.5% of patients and to lymphoproliferative disorders in 28.7% of patients, and was defined as essential in 48.8% of patients. At presentation, hematuria, proteinuria ≥1 g/d, hypertension, and renal failure were observed in 97.4%, 84.8%, 85.3%, and 82.3% of cases, respectively. Mean±eGFR was 39.5±20.4 ml/min per 1.73 m2. Membranoproliferative GN was the predominant histologic pattern, observed in 89.6% of cases. Renal interstitium inflammatory infiltrates were observed in 50% of cases. First-line treatment consisted of steroids alone (27.6%) or in association with rituximab (21.1%), alkylating agents (36.8%) or a combination of cyclophosphamide and rituximab (10.5%). After a mean follow-up of 49.9±45.5 months, 42.7% of patients relapsed with a renal flare in 75% of cases. At last follow-up, mean eGFR was 50.2±26.1 ml/min per 1.73 m2 with 9% of patients having reached ESRD; 59% and 50% of patients achieved complete clinical and renal remission, respectively. A rituximab+steroids regimen prevented relapses more effectively than steroids alone or a cyclophosphamide+steroids combination did, but was associated with a higher rate of early death when used as first-line therapy. Severe infections and new-onset B-cell lymphoma occurred in 29.1% and 8.9% of cases, respectively; 24% of patients died. In conclusion, noninfectious MCGN has a poor long-term outcome with severe infections as the main cause of death
Published: 2015
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28. Long-term effectiveness and safety of interleukin-1 receptor antagonist (anakinra) in Schnitzler's syndrome: A French multicenter study

Author: Adrien Lorleac'h, J.M. Brisseau, Olivier Decaux, Eric Hachulla, Fabrice Bonnet, Jacques Serratrice, Guillaume Moulis, Robin Dhote, Pierre-Jean Weiller, Pierre Pottier, Fabienne Closs-Prophette, Jean-Dominique de Korwin, Jean-Marie Berthelot, Renato Fior, Bernard Grosbois, Benoit Henry, Antoine Néel, Achille Aouba, Bérangère Cador, Thomas Dejoie, Amar Smail, Xavier Puéchal, F. Perrin, Agathe Masseau, Arnaud Hot, Claire Bernier, Xavier Kyndt, Sébastien Barbarot, Mohamed Hamidou, H. Jardel, Jacques Ninet, Pierre-Yves Hatron, Leonardo Astudillo, David Launay, Service de médecine interne [Nantes], Université de Nantes (UN)-Hôtel-Dieu-Centre hospitalier universitaire de Nantes (CHU Nantes), Service de dermatologie [Nantes], Université de Nantes (UN)-Centre hospitalier universitaire de Nantes (CHU Nantes), Service de médecine interne et néphrologie, CH Valenciennes, Service de médecine interne et centre de référence des maladies rares [CHU Cochin], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Hôpital Cochin [AP-HP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP), Service de Médecine Interne, Hôpital de la Timone [CHU - APHM] (TIMONE), Service de Médecine interne et immunologie clinique [Rennes] = internal medicine and clinical immunology [Rennes], CHU Pontchaillou [Rennes], Institut de Génétique et Développement de Rennes (IGDR), Université de Rennes (UR)-Centre National de la Recherche Scientifique (CNRS)-Structure Fédérative de Recherche en Biologie et Santé de Rennes ( Biosit : Biologie - Santé - Innovation Technologique ), Hôpital Edouard Herriot [CHU - HCL], Hospices Civils de Lyon (HCL)-Hospices Civils de Lyon (HCL), Service de médecine interne, immunologie clinique [Béclère], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-AP-HP - Hôpital Antoine Béclère [Clamart], Service Médecine Interne et immunologie clinique [CHU Toulouse], Pôle Maladies de l'appareil digestif [CHU Toulouse], Centre Hospitalier Universitaire de Toulouse (CHU Toulouse)-Centre Hospitalier Universitaire de Toulouse (CHU Toulouse), Centre d'Investigation Clinique [Rennes] (CIC), Université de Rennes (UR)-Hôpital Pontchaillou-Institut National de la Santé et de la Recherche Médicale (INSERM), Foie, métabolismes et cancer, Université de Rennes (UR)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Structure Fédérative de Recherche en Biologie et Santé de Rennes ( Biosit : Biologie - Santé - Innovation Technologique ), Service de rhumatologie [Nantes], Service de médecine polyvalente et de médecine interne [CH Le Mans], Centre Hospitalier Le Mans (CH Le Mans), Laboratoire de Biochimie [Nantes], Centre hospitalier universitaire de Nantes (CHU Nantes), Service de Médecine Interne et Médecine Générale [CHRU Nancy], Centre Hospitalier Régional Universitaire de Nancy (CHRU Nancy), CHU Avicenne, Service de médecine interne, Centre Hospitalier Régional Universitaire [Lille] (CHRU Lille), Laboratoire d'Immunologie (EA 2686), Université de Lille, Droit et Santé, Faculté de Médecine Henri Warembourg - Université de Lille, CH Bretagne Atlantique, Service de Médecine Interne et Maladies Infectieuses, CH Bretagne Sud, Service de Néphrologie - Médecine Interne, CHU Amiens-Picardie-hôpital Sud, AN received a grant from Groupama Loire Bretagne--Fondation Groupama pour la Santé., Jonchère, Laurent, Université de Rennes 1 (UR1), Université de Rennes (UNIV-RENNES)-Université de Rennes (UNIV-RENNES)-Centre National de la Recherche Scientifique (CNRS)-Structure Fédérative de Recherche en Biologie et Santé de Rennes ( Biosit : Biologie - Santé - Innovation Technologique ), Service de médecine interne, hôpital Purpan, CHU Toulouse [Toulouse], Université de Rennes (UNIV-RENNES)-Université de Rennes (UNIV-RENNES)-Hôpital Pontchaillou-Institut National de la Santé et de la Recherche Médicale (INSERM), Université de Rennes (UNIV-RENNES)-Université de Rennes (UNIV-RENNES)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Structure Fédérative de Recherche en Biologie et Santé de Rennes ( Biosit : Biologie - Santé - Innovation Technologique ), Faculté de Médecine, PRES Université Lille Nord de France, Université de Nantes ( UN ) -Hôtel-Dieu-Centre hospitalier universitaire de Nantes ( CHU Nantes ), Université de Nantes ( UN ) -Centre hospitalier universitaire de Nantes ( CHU Nantes ), Assistance publique - Hôpitaux de Paris (AP-HP)-CHU Cochin [AP-HP], Hôpital de la Timone [CHU - APHM] ( TIMONE ), Service de Médecine Interne [Rennes], Institut de Génétique et Développement de Rennes ( IGDR ), Université de Rennes 1 ( UR1 ), Université de Rennes ( UNIV-RENNES ) -Université de Rennes ( UNIV-RENNES ) -Centre National de la Recherche Scientifique ( CNRS ) -Structure Fédérative de Recherche en Biologie et Santé de Rennes ( Biosit : Biologie - Santé - Innovation Technologique ), Hospices Civils de Lyon ( HCL ) -Hospices Civils de Lyon ( HCL ), Université Paris-Sud - Paris 11 ( UP11 ) -Assistance publique - Hôpitaux de Paris (AP-HP)-Hôpital Antoine Béclère, Centre d'Investigation Clinique [Rennes] ( CIC ), Université de Rennes ( UNIV-RENNES ) -Université de Rennes ( UNIV-RENNES ) -Hôpital Pontchaillou-Institut National de la Santé et de la Recherche Médicale ( INSERM ), Université de Rennes ( UNIV-RENNES ) -Université de Rennes ( UNIV-RENNES ) -Institut National de la Santé et de la Recherche Médicale ( INSERM ) -Structure Fédérative de Recherche en Biologie et Santé de Rennes ( Biosit : Biologie - Santé - Innovation Technologique ), Service de Médecine Polyvalente, CH du Mans, Centre hospitalier universitaire de Nantes ( CHU Nantes ), Centre Hospitalier Régional Universitaire de Nancy ( CHRU Nancy ), Centre Hospitalier Régional Universitaire [Lille] ( CHRU Lille ), Laboratoire d'Immunologie ( EA 2686 ), and CHU Amiens-Picardie-Hôpital Sud
Subjects: Male, medicine.medical_specialty, Autoinflammatory disease, IL-1 receptor antagonist, Urticaria, Anemia, [SDV]Life Sciences [q-bio], Immunology, Schnitzler's syndrome, Neutropenia, AA amyloidosis, Gammopathy, Internal medicine, medicine, Humans, Immunology and Allergy, Schnitzler Syndrome, Retrospective Studies, Anakinra, [ SDV ] Life Sciences [q-bio], business.industry, Remission Induction, Macroglobulinemia, medicine.disease, 3. Good health, Surgery, [SDV] Life Sciences [q-bio], Interleukin 1 Receptor Antagonist Protein, Schnitzler syndrome, Joint pain, Quality of Life, Female, medicine.symptom, business, medicine.drug
Abstract: Conflicts of interest: DL received a grant from Sobi (< 10,000 USD). EH received lecture fees from Sobi (< 10,000 USD). The other authors have no conflict of interest to disclose. Ethics approval: According to French legislation regarding retrospective observational studies using anonymous data, Ethics Committee approval was not required, which was confirmed by the chair of our Institutional Ethics Committee. The study was performed in accordance with the Declaration of Helsinki's guidelines for research in humans.; International audience; The aim of this study is to assess the long-term effectiveness and safety of IL1Ra in Schnitzler syndrome (SchS). Between 2010 and 2012, we performed a nationwide survey among French internal medicine departments to identify SchS patients. We retrospectively analyzed the long-term efficacy and safety of IL1Ra and the outcome of patients that did not receive this treatment. Forty-two patients were included in the study, 29 of whom received IL1Ra. The mean age at disease onset was 59.9years. Disease manifestations included urticaria (100%), fever (76%), bone/joint pain (86%), bone lesions (76%), anemia (67%), and weight loss (60%). The monoclonal gammopathy was overwhelmingly IgM kappa (83%). The mean follow-up was 9.5years (range: 1.6-35). Two patients developed Waldenström's macroglobulinemia and one developed AA amyloidosis. All of the 29 patients who received IL1Ra responded dramatically. After a median follow-up of 36months (range: 2-79), the effectiveness remained unchanged. All patients remained on anti-IL-1 therapy. Twenty-four patients (83%) went into complete remission and five (17%) into partial remission. Three patients experienced grade 3-4 neutropenia. Six patients developed severe infections. No lymphoproliferative diseases occurred while on IL1Ra. When last seen, all patients without anakinra had an active disease with variable impact on their quality of life. Their median corticosteroids dosage was 6mg/d (range: 5-25). IL1Ra is effective in SchS, with a sharp corticosteroid-sparing effect. Treatment failures should lead to reconsider the diagnosis. Long-term follow-up revealed no loss of effectiveness and a favorable tolerance profile. The long-term effects on the risk of hemopathy remain unknown.
Published: 2014
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29. High prevalence of infectious events in thrombotic thrombocytopenic purpura and genetic relationship with toll-like receptor 9 polymorphisms: experience of the French Thrombotic Microangiopathies Reference Center

Author: Morgand , Marjolaine, Buffet , Marc, Busson , Marc, Loiseau , Pascale, Malot , Sandrine, Amokrane , Kahina, Fortier , Catherine, London , Jonathan, Bonmarchand , Guy, Wynckel , Alain, Provôt , François, Poullin , Pascale, Vanhille , Philippe, Presne , Claire, Bordessoule , Dominique, Girault , Stéphane, Delmas , Yahsou, Hamidou , Mohamed, Mousson , Christiane, Vigneau , Cécile, Lautrette , Alexandre, Pourrat , Jacques, Galicier , Lionel, Azoulay , Elie, Pène , Frédéric, Mira , Jean-Paul, Rondeau , Eric, Ojeda-Uribe , Mario, Charron , Dominique, Maury , Eric, Guidet , Bertrand, Veyradier , Agnès, Tamouza , Ryad, Coppo , Paul, Center , Thrombotic Microangiopathies Reference, Service d'immunologie et hématologies biologiques, Université Pierre et Marie Curie - Paris 6 ( UPMC ) -Assistance publique - Hôpitaux de Paris (AP-HP)-CHU Saint-Antoine [APHP], Hématologie -Immunologie -Cibles thérapeutiques, Université Paris Diderot - Paris 7 ( UPD7 ) -Institut National de la Santé et de la Recherche Médicale ( INSERM ), Laboratoire Jean Dausset, Assistance publique - Hôpitaux de Paris (AP-HP)-Groupe Hospitalier Saint Louis - Lariboisière - Fernand Widal [Paris], Immunogénétique humaine, Assistance publique - Hôpitaux de Paris (AP-HP)-Institut National de la Santé et de la Recherche Médicale ( INSERM ), Heuristique et Diagnostic des Systèmes Complexes [Compiègne] ( Heudiasyc ), Université de Technologie de Compiègne ( UTC ) -Centre National de la Recherche Scientifique ( CNRS ), Service de réanimation médicale [Rouen], Hôpital Charles Nicolle [Rouen]-CHU Rouen-Université de Rouen Normandie ( UNIROUEN ), Normandie Université ( NU ) -Normandie Université ( NU ), Service de Néphrologie, Centre Hospitalier Universitaire de Reims ( CHU Reims ) -Hôpital Maison Blanche, Service de néphrologie, Centre Hospitalier Régional Universitaire [Lille] ( CHRU Lille ), Centre de Référence des microangiopathies thrombotiques ( CNR-MAT ), service d'hématologie-CHU Saint-Antoine [APHP], Service d'Hémaphérèse - Médecine Interne, Assistance Publique - Hôpitaux de Marseille ( APHM ) -Hôpital de la Conception [CHU - APHM] ( LA CONCEPTION ), Service de médecine interne et néphrologie, CH Valenciennes, Service de Néphrologie - Médecine Interne, CHU Amiens-Picardie-Hôpital Sud, Service d'Hématologie clinique et thérapie cellulaire [CHU Limoges], CHU Limoges, Contrôle de la Réponse Immune B et des Lymphoproliférations ( CRIBL ), Université de Limoges ( UNILIM ) -Génomique, Environnement, Immunité, Santé, Thérapeutique ( GEIST FR CNRS 3503 ) -Centre National de la Recherche Scientifique ( CNRS ), Fibrose hépatique et cancer du foie, Université Bordeaux Segalen - Bordeaux 2-IFR66-Institut National de la Santé et de la Recherche Médicale ( INSERM ), Service de médecine interne [Nantes], Université de Nantes ( UN ) -Hôtel-Dieu-Centre hospitalier universitaire de Nantes ( CHU Nantes ), Service de néphrologie (CHU de Dijon), Centre Hospitalier Universitaire de Dijon - Hôpital François Mitterrand ( CHU Dijon ), Université de Rennes 1 ( UR1 ), Université de Rennes ( UNIV-RENNES ) -Université de Rennes ( UNIV-RENNES ) -Hôpital Pontchaillou-CHU Pontchaillou [Rennes], Institut de Génétique et Développement de Rennes ( IGDR ), Université de Rennes ( UNIV-RENNES ) -Université de Rennes ( UNIV-RENNES ) -Centre National de la Recherche Scientifique ( CNRS ) -Structure Fédérative de Recherche en Biologie et Santé de Rennes ( Biosit : Biologie - Santé - Innovation Technologique ), Unité de soins intensifs [Clermont Ferrand], CHU Clermont-Ferrand-Hôpital Gabriel Montpied, Service de Néphrologie - Immunologie Clinique [Toulouse], CHU Toulouse [Toulouse]-Hôpital de Rangueil, CHU Toulouse [Toulouse]-PRES Université de Toulouse, Service d'Immunopathologie [Hôpital Saint-Louis, Paris], Université Paris Diderot - Paris 7 ( UPD7 ) -CHU Saint Louis [APHP], Outcomerea Organization, Outcomerea, Unité de Soins Intensifs [Saint-Louis], Université Paris Descartes - Faculté de Médecine ( UPD5 Médecine ), Université Paris Descartes - Paris 5 ( UPD5 ), Institut Cochin ( UM3 (UMR 8104 / U1016) ), Université Paris Descartes - Paris 5 ( UPD5 ) -Institut National de la Santé et de la Recherche Médicale ( INSERM ) -Centre National de la Recherche Scientifique ( CNRS ), Unité Médicale de Soins Intensifs, Assistance publique - Hôpitaux de Paris (AP-HP)-CHU Cochin [AP-HP], Service de Réanimation polyvalente, Assistance publique - Hôpitaux de Paris (AP-HP)-CHU Cochin [AP-HP]-Université Paris Descartes - Paris 5 ( UPD5 ), Remodelage et Reparation du Tissu Renal, Université Pierre et Marie Curie - Paris 6 ( UPMC ) -Institut National de la Santé et de la Recherche Médicale ( INSERM ), Unité de Soins Intensifs et Transplantation Rénale, Assistance publique - Hôpitaux de Paris (AP-HP)-CHU Tenon [APHP], Fondation FondaMental, Assistance publique - Hôpitaux de Paris (AP-HP)-Hôpital Henri Mondor, Epidémiologie des maladies infectieuses et modélisation ( ESIM ), Service de Réanimation Médicale [CHU Saint-Antoine], Assistance publique - Hôpitaux de Paris (AP-HP)-CHU Saint-Antoine [APHP], Service d'Hématologie Biologique [Béclère], Université Paris-Sud - Paris 11 ( UP11 ) -Assistance publique - Hôpitaux de Paris (AP-HP)-Hôpital Antoine Béclère, Service d'hématologie clinique et de thérapie cellulaire [CHU Saint-Antoine], CHU Saint-Antoine [AP-HP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU), Université Paris Diderot - Paris 7 (UPD7)-Institut National de la Santé et de la Recherche Médicale (INSERM), Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Groupe Hospitalier Saint Louis - Lariboisière - Fernand Widal [Paris], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP), Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Institut National de la Santé et de la Recherche Médicale (INSERM), Heuristique et Diagnostic des Systèmes Complexes [Compiègne] (Heudiasyc), Université de Technologie de Compiègne (UTC)-Centre National de la Recherche Scientifique (CNRS), Service de réanimation médicale [CHU Rouen], Hôpital Charles Nicolle [Rouen], CHU Rouen, Normandie Université (NU)-Normandie Université (NU)-CHU Rouen, Normandie Université (NU)-Normandie Université (NU)-Université de Rouen Normandie (UNIROUEN), Normandie Université (NU), Centre Hospitalier Universitaire de Reims (CHU Reims)-Hôpital Maison Blanche, Centre Hospitalier Régional Universitaire [Lille] (CHRU Lille), Assistance Publique - Hôpitaux de Marseille (APHM)-Hôpital de la Conception [CHU - APHM] (LA CONCEPTION), CHU Amiens-Picardie-hôpital Sud, Contrôle de la Réponse Immune B et des Lymphoproliférations (CRIBL), Université de Limoges (UNILIM)-Génomique, Environnement, Immunité, Santé, Thérapeutique (GEIST FR CNRS 3503)-Centre National de la Recherche Scientifique (CNRS), Université Bordeaux Segalen - Bordeaux 2-IFR66-Institut National de la Santé et de la Recherche Médicale (INSERM), Université de Nantes (UN)-Hôtel-Dieu-Centre hospitalier universitaire de Nantes (CHU Nantes), Centre Hospitalier Universitaire de Dijon - Hôpital François Mitterrand (CHU Dijon), Service de néphrologie [Rennes], Université de Rennes (UR)-Hôpital Pontchaillou-CHU Pontchaillou [Rennes], Institut de Génétique et Développement de Rennes (IGDR), Université de Rennes (UR)-Centre National de la Recherche Scientifique (CNRS)-Structure Fédérative de Recherche en Biologie et Santé de Rennes ( Biosit : Biologie - Santé - Innovation Technologique ), CHU Clermont-Ferrand-CHU Gabriel Montpied [Clermont-Ferrand], CHU Clermont-Ferrand, Centre Hospitalier Universitaire de Toulouse (CHU Toulouse)-PRES Université de Toulouse, Université Paris Diderot - Paris 7 (UPD7)-Hopital Saint-Louis [AP-HP] (AP-HP), Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP), Université Paris Descartes - Faculté de Médecine (UPD5 Médecine), Université Paris Descartes - Paris 5 (UPD5), Institut Cochin (IC UM3 (UMR 8104 / U1016)), Université Paris Descartes - Paris 5 (UPD5)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Centre National de la Recherche Scientifique (CNRS), Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Hôpital Cochin [AP-HP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Université Paris Descartes - Paris 5 (UPD5), Université Pierre et Marie Curie - Paris 6 (UPMC)-Institut National de la Santé et de la Recherche Médicale (INSERM), CHU Tenon [AP-HP], Fondation FondaMental [Créteil], Epidémiologie des maladies infectieuses et modélisation (ESIM), Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-AP-HP - Hôpital Antoine Béclère [Clamart], Service d'immunologie et hématologies biologiques [Saint-Antoine], Université Pierre et Marie Curie - Paris 6 (UPMC)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-CHU Saint-Antoine [AP-HP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU)-Sorbonne Université (SU), Hôpital Charles Nicolle [Rouen]-CHU Rouen, Centre National de la Recherche Scientifique (CNRS)-Université de Limoges (UNILIM)-Génomique, Environnement, Immunité, Santé, Thérapeutique (GEIST FR CNRS 3503), Université de Rennes 1 (UR1), Université de Rennes (UNIV-RENNES)-Université de Rennes (UNIV-RENNES)-Hôpital Pontchaillou-CHU Pontchaillou [Rennes], Structure Fédérative de Recherche en Biologie et Santé de Rennes ( Biosit : Biologie - Santé - Innovation Technologique )-Centre National de la Recherche Scientifique (CNRS)-Université de Rennes 1 (UR1), Université de Rennes (UNIV-RENNES)-Université de Rennes (UNIV-RENNES), Sorbonne Université (SU)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP), Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-CHU Saint-Antoine [AP-HP], AP-HP - Hôpital Antoine Béclère [Clamart], Université Pierre et Marie Curie - Paris 6 (UPMC)-Assistance publique - Hôpitaux de Paris (AP-HP) (APHP)-CHU Saint-Antoine [APHP], Assistance publique - Hôpitaux de Paris (AP-HP) (APHP)-Groupe Hospitalier Saint Louis - Lariboisière - Fernand Widal [Paris], Assistance publique - Hôpitaux de Paris (AP-HP) (APHP), Assistance publique - Hôpitaux de Paris (AP-HP) (APHP)-Institut National de la Santé et de la Recherche Médicale (INSERM), Université de Rouen Normandie (UNIROUEN), Normandie Université (NU)-Hôpital Charles Nicolle [Rouen], CHU Saint-Antoine [APHP], Assistance Publique - Hôpitaux de Marseille (APHM)-Hôpital de la Conception [CHU - APHM] (LA CONCEPTION ), Université de Rennes (UNIV-RENNES)-Université de Rennes (UNIV-RENNES)-Centre National de la Recherche Scientifique (CNRS)-Structure Fédérative de Recherche en Biologie et Santé de Rennes ( Biosit : Biologie - Santé - Innovation Technologique ), Université Paris Diderot - Paris 7 (UPD7)-CHU Saint Louis [APHP], Assistance publique - Hôpitaux de Paris (AP-HP) (APHP)-CHU Cochin [AP-HP], Assistance publique - Hôpitaux de Paris (AP-HP) (APHP)-CHU Cochin [AP-HP]-Université Paris Descartes - Paris 5 (UPD5), CHU Tenon [APHP], Assistance publique - Hôpitaux de Paris (AP-HP) (APHP)-CHU Saint-Antoine [APHP], and Université Paris-Sud - Paris 11 (UP11)-Assistance publique - Hôpitaux de Paris (AP-HP) (APHP)-Hôpital Antoine Béclère
Subjects: Male, Adult, Exacerbation, Immunology, Thrombotic thrombocytopenic purpura, Human leukocyte antigen, 030204 cardiovascular system & hematology, Infections, 03 medical and health sciences, 0302 clinical medicine, Genetic, Risk Factors, hemic and lymphatic diseases, Genotype, medicine, Prevalence, Immunology and Allergy, Humans, Genetic Predisposition to Disease, Prospective Studies, Registries, Polymorphism, Prospective cohort study, Purpura, [SDV.GEN]Life Sciences [q-bio]/Genetics, Polymorphism, Genetic, Thrombotic Thrombocytopenic, Purpura, Thrombotic Thrombocytopenic, business.industry, Thrombotic Microangiopathies, Haplotype, Hematology, Middle Aged, medicine.disease, 3. Good health, Haplotypes, Toll-Like Receptor 9, Cohort, Rituximab, Female, France, business, Infection, [ SDV.GEN ] Life Sciences [q-bio]/Genetics, 030215 immunology, medicine.drug
Abstract: National audience; BACKGROUND: Infectious events have been reported as major environmental triggers of thrombotic thrombocytopenic purpura (TTP). We detail here the potential association between infections and TTP. STUDY DESIGN AND METHODS: We recruited randomly and prospectively a cohort of 280 consecutive TTP patients during a 9-year period. Features of infection were systematically recorded. RESULTS: Features consistent with an infectious event were observed in 114 patients (41%) at time of TTP diagnosis. Infectious agents were documented in 34 cases and were mainly Gram-negative bacilli. At time of diagnosis infected patients more frequently had fever (p \textless 0.001). Infections at diagnosis did not impact prognosis and outcome. Thirty-six percent of patients experienced an infectious event during hospitalization, which resulted in more exacerbation of TTP (p = 0.02). Infections were not overrepresented during treatment in patients who received steroids and/or rituximab. Further genetic analysis of toll-like receptor (TLR)-9 functionally relevant polymorphisms revealed that TLR-9 +2848 G and TLR-9 +1174 A genotypes were more frequent in TTP patients than in controls (p = 0.04 and p = 0.026, respectively) and more particularly in patients negative for the Class II human leukocyte antigen system susceptibility allele DRB1*11 (p = 0.001 and p = 0.002, respectively). Haplotypes estimation showed that 1174A-2848G haplotype was significantly more frequent in TTP (p = 0.004), suggesting a primary role for this haplotype variation in conferring a predisposition for acquired TTP. CONCLUSION: Infections should be considered as an aggravating factor during the course of TTP. Particular polymorphisms in TLR-9 gene may represent risk factors for TTP.
Published: 2014
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30. First cases of Middle East Respiratory Syndrome Coronavirus (MERS-CoV) infections in France, investigations and implications for the prevention of human-to-human transmission, France, May 2013

Author: A Mailles, K Blanckaert, P Chaud, S van der Werf, B Lina, V Caro, C Campese, B Guéry, H Prouvost, X Lemaire, M C Paty, S Haeghebaert, D Antoine, N Ettahar, H Noel, S Behillil, S Hendricx, J C Manuguerra, V Enouf, G La Ruche, C Semaille, B Coignard, D Lévy-Bruhl, F Weber, C Saura, D Che, Collective The investigation team, Institut de Veille Sanitaire (INVS), Antenne Régionale de Lutte contre les Infections Nosocomiales [Lille] (ARLIN), Génétique Moléculaire des Virus à ARN - Molecular Genetics of RNA Viruses (GMV-ARN (UMR_3569 / U-Pasteur_2)), Institut Pasteur [Paris] (IP)-Université Paris Diderot - Paris 7 (UPD7)-Centre National de la Recherche Scientifique (CNRS), Centre National de Référence des virus influenzae (Grippe) (CNR), Institut Pasteur [Paris] (IP), Virologie et pathogenèse virale (VPV), Université Claude Bernard Lyon 1 (UCBL), Université de Lyon-Université de Lyon-Centre National de la Recherche Scientifique (CNRS), Cellule d'Intervention Biologique d'Urgence - Laboratory for Urgent Response to Biological Threats (CIBU), Université de Lille, Service maladies infectieuses [CH Douai], Centre Hospitalier [Douai, Nord], CH Valenciennes, Cellule d'Intervention Biologique d'Urgence (Centre National de Référence) - Laboratory for Urgent Response to Biological Threats (National Reference Center) (CIBU), Environnement et Risques infectieux - Environment and Infectious Risks (ERI), Institut Pasteur [Paris] (IP)-Institut Pasteur [Paris] (IP), Virologie et pathologie humaine (VirPath), Université de Lyon-Université de Lyon, and Investigation team : InVS, Saint Maurice, France: Sandrine Barquin, Fatima Belghiti, Alexandre Blake, Gabriel Bedubourg, Isabelle Bonmarin, Jérémie Carré, Carole Debreyer, Gilles Delmas, Anne Gallay, Claire Gourier-Fréry, Etienne Lucas, Roselyne Pinget, Mathieu Tourdjmann, Sarah Tubiana, Dieter Van Cauteren, Annie Velter, Manuel Zubaran. InVS, Lille, France: Audrey Andrieu, Emile Baro, Guillaume Clement. National Reference Center for influenza viruses, Hospices Civils de Lyon and Virpath, Université Claude Bernard Lyon1, Lyon, France: Maude Bouscambert, Martine Valette. Centre Hospitalier Régional et Universitaire, Université de Lille 2, Lille, France: Karine Faure, Hugues Melliez, Fanny Vuotto. Centre hospitalier, Douai, France: Loubna El-Mansouf. Centre hospitalier, Valenciennes, France: Anne-Adélaide Cracco. 5www.eurosurveillance.org Regional Health Agency, Lille, France: Jean-Philippe Legendre, Sandrine Segovia-Kueny. Centre hospitalier universitaire, Tourcoing, France: Eric Senneville.
Subjects: Male, Pediatrics, medicine.medical_specialty, MESH: Fatal Outcome, Adult male, Epidemiology, Middle East respiratory syndrome coronavirus, MESH: Coronavirus Infections, viruses, [SDV]Life Sciences [q-bio], United Arab Emirates, MESH: Disease Transmission, Infectious, medicine.disease_cause, MESH: Contact Tracing, Fatal Outcome, Virology, MESH: Reverse Transcriptase Polymerase Chain Reaction, Disease Transmission, Infectious, medicine, MESH: Coronavirus, Humans, Symptom onset, Respiratory Tract Infections, Travel, Respiratory illness, MESH: Middle Aged, MESH: Humans, Reverse Transcriptase Polymerase Chain Reaction, business.industry, Transmission (medicine), Public Health, Environmental and Occupational Health, virus diseases, Middle Aged, MESH: Male, Coronavirus, MESH: France, MESH: United Arab Emirates, MESH: Respiratory Tract Infections, France, Contact Tracing, Coronavirus Infections, business
Abstract: International audience; In May 2013, Middle East Respiratory Syndrome Coronavirus (MERS-CoV) infection was diagnosed in an adult male in France with severe respiratory illness, who had travelled to the United Arab Emirates before symptom onset. Contact tracing identified a secondary case in a patient hospitalised in the same hospital room. No other cases of MERS-CoV infection were identified among the index case’s 123 contacts, nor among 39 contacts of the secondary case, during the 10-day follow-up period.
Published: 2013
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31. Prognostic factors of survival in patients with non-infectious mixed cryoglobulinaemia vasculitis: data from 242 cases included in the CryoVas survey

Author: Isabelle Marie, Jean-Marc Léger, Estibaliz Lzaro, Xavier Mariette, Adeline Lacraz, Thomas Quemeneur, Olivier Hermine, Franck Bridoux, Olivier Hinschberger, P. Rullier, Jean-Emmanuel Kahn, Fabrice Carrat, Patrice Cacoub, Thierry Zenone, Christian Pagnoux, P. Belenotti, Fabrice Bonnet, Patricia Senet, Luc de Saint Martin, Guillaume Le Guenno, Antoine Huart, Aurélie Hummel, Evguenia Krastinova, Elisabeth Diot, Benjamin Terrier, Emmanuelle Plaisier, David Launay, Immunologie - Immunopathologie - Immunothérapeutique (I3), Université Pierre et Marie Curie - Paris 6 (UPMC)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Centre National de la Recherche Scientifique (CNRS), Service de Médecine Interne, Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Hôpital Cochin [AP-HP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Université Paris Descartes - Paris 5 (UPD5), Epidémiologie des maladies infectieuses et modélisation (ESIM), Université Pierre et Marie Curie - Paris 6 (UPMC)-Institut National de la Santé et de la Recherche Médicale (INSERM), Centre de recherche en épidémiologie et santé des populations (CESP), Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Université Paris-Sud - Paris 11 (UP11)-Hôpital Paul Brousse-Institut National de la Santé et de la Recherche Médicale (INSERM)-Université de Versailles Saint-Quentin-en-Yvelines (UVSQ), Service de Médecine Interne [CHU Rouen], CHU Rouen, Normandie Université (NU)-Normandie Université (NU)-Université de Rouen Normandie (UNIROUEN), Normandie Université (NU), Physiopathologie et biothérapies des maladies inflammatoires et autoimmunes, Université de Rouen Normandie (UNIROUEN), Normandie Université (NU)-Normandie Université (NU)-Institut National de la Santé et de la Recherche Médicale (INSERM), Department of Internal Medicine, Centre Hospitalier Régional Universitaire [Lille] (CHRU Lille), Service de Néphrologie-Transplantation-Dialyse, CHU Bordeaux [Bordeaux]-Groupe hospitalier Pellegrin, Service de Médecine Interne (MARSEILLE - Med Int), Assistance Publique - Hôpitaux de Marseille (APHM), Département de Médecine Interne et Pneumologie [Brest] (DMIP - Brest), Centre Hospitalier Régional Universitaire de Brest (CHRU Brest), Groupe d'Etude de la Thrombose de Bretagne Occidentale (GETBO), Université de Brest (UBO)-Institut Brestois Santé Agro Matière (IBSAM), Université de Brest (UBO)-Université de Brest (UBO), Département de Médecine Interne (VALENCIENNES - Med Int), CH Valenciennes, Service de Néphrologie et Immunopathologie Clinique, Centre Hospitalier Universitaire de Toulouse, PRES Université de Toulouse, Foie, métabolismes et cancer, Université de Rennes 1 (UR1), Université de Rennes (UNIV-RENNES)-Université de Rennes (UNIV-RENNES)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Structure Fédérative de Recherche en Biologie et Santé de Rennes ( Biosit : Biologie - Santé - Innovation Technologique ), CHU Clermont-Ferrand, Laboratoire d'Immunologie (EA 2686), Université de Lille, Droit et Santé, Département de Médecine Interne (MULHOUSE - Med Int), Centre Hospitalier Emile Muller [Mulhouse] (CH E.Muller Mulhouse), Groupe Hospitalier de Territoire Haute Alsace (GHTHA)-Groupe Hospitalier de Territoire Haute Alsace (GHTHA), Service de Médecine Interne B, Centre Hospitalier Régional Universitaire [Montpellier] (CHRU Montpellier), Hôpital Bretonneau, Service de médecine interne et centre de référence des maladies rares [CHU Cochin], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP), Contrôle de la Réponse Immune B et des Lymphoproliférations (CRIBL), Université de Limoges (UNILIM)-Génomique, Environnement, Immunité, Santé, Thérapeutique (GEIST FR CNRS 3503)-Centre National de la Recherche Scientifique (CNRS), Service de néphrologie - hémodialyse et transplantation rénale, Centre hospitalier universitaire de Poitiers (CHU Poitiers), Médecine Interne (VALENCE - Med Int), Centre hospitalier de Valence, Cytokines, hématopoïèse et réponse immune (CHRI), Université Paris Descartes - Paris 5 (UPD5)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Centre National de la Recherche Scientifique (CNRS), Service de Neurologie [CHU Pitié-Salpêtrière], IFR70-CHU Pitié-Salpêtrière [AP-HP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU), Régulation de la réponse immune, infection VIH-1 et autoimmunité, Université Paris-Sud - Paris 11 (UP11)-Institut National de la Santé et de la Recherche Médicale (INSERM), Service de dermatologie et allergologie [CHU Tenon], CHU Tenon [AP-HP], Sorbonne Université (SU)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP), Remodelage et Reparation du Tissu Renal, Service de médecine interne [CHU Pitié-Salpétrière], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-CHU Pitié-Salpêtrière [AP-HP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU)-Sorbonne Université (SU), Biologie et thérapeutique des pathologies immunitaires (BTPI), Université Pierre et Marie Curie - Paris 6 (UPMC)-Centre National de la Recherche Scientifique (CNRS), Assistance publique - Hôpitaux de Paris (AP-HP) (APHP)-CHU Cochin [AP-HP]-Université Paris Descartes - Paris 5 (UPD5), Université de Versailles Saint-Quentin-en-Yvelines (UVSQ)-Université Paris-Sud - Paris 11 (UP11)-Assistance publique - Hôpitaux de Paris (AP-HP) (APHP)-Hôpital Paul Brousse-Institut National de la Santé et de la Recherche Médicale (INSERM), Service de Médecine Interne [Rouen], Normandie Université (NU)-Normandie Université (NU)-CHU Rouen, Université de Brest (UBO), Centre Hospitalier Universitaire Estaing, CHU Estaing, Assistance publique - Hôpitaux de Paris (AP-HP) (APHP)-CHU Cochin [AP-HP], Service de neurologie 1 [CHU Pitié-Salpétrière], Assistance publique - Hôpitaux de Paris (AP-HP) (APHP)-CHU Pitié-Salpêtrière [APHP], Service de Dermatologie [CHU Tenon], Assistance publique - Hôpitaux de Paris (AP-HP) (APHP)-CHU Tenon [APHP], Centre National de la Recherche Scientifique (CNRS)-Université Pierre et Marie Curie - Paris 6 (UPMC), Immunologie - Immunopathologie - Immunothérapeutique ( I3 ), Université Pierre et Marie Curie - Paris 6 ( UPMC ) -Institut National de la Santé et de la Recherche Médicale ( INSERM ) -Centre National de la Recherche Scientifique ( CNRS ), Assistance publique - Hôpitaux de Paris (AP-HP)-CHU Cochin [AP-HP]-Université Paris Descartes - Paris 5 ( UPD5 ), Epidémiologie des maladies infectieuses et modélisation ( ESIM ), Université Pierre et Marie Curie - Paris 6 ( UPMC ) -Institut National de la Santé et de la Recherche Médicale ( INSERM ), Centre de recherche en épidémiologie et santé des populations ( CESP ), Université de Versailles Saint-Quentin-en-Yvelines ( UVSQ ) -Université Paris-Sud - Paris 11 ( UP11 ) -Assistance publique - Hôpitaux de Paris (AP-HP)-Hôpital Paul Brousse-Institut National de la Santé et de la Recherche Médicale ( INSERM ), Université de Rouen Normandie ( UNIROUEN ), Normandie Université ( NU ) -Normandie Université ( NU ) -CHU Rouen, Normandie Université ( NU ) -Normandie Université ( NU ) -Institut National de la Santé et de la Recherche Médicale ( INSERM ), Centre Hospitalier Régional Universitaire [Lille] ( CHRU Lille ), Service de Médecine Interne ( MARSEILLE - Med Int ), Assistance Publique - Hôpitaux de Marseille ( APHM ), Département de Médecine Interne et Pneumologie [Brest] ( DMIP - Brest ), Centre Hospitalier Régional Universitaire de Brest ( CHRU Brest ), Groupe d'Etude de la Thrombose de Bretagne Occidentale ( GETBO ), Université de Brest ( UBO ), Département de Médecine Interne ( VALENCIENNES - Med Int ), Université de Rennes 1 ( UR1 ), Université de Rennes ( UNIV-RENNES ) -Université de Rennes ( UNIV-RENNES ) -Institut National de la Santé et de la Recherche Médicale ( INSERM ) -Structure Fédérative de Recherche en Biologie et Santé de Rennes ( Biosit : Biologie - Santé - Innovation Technologique ), Laboratoire d'Immunologie ( EA 2686 ), Département de Médecine Interne ( MULHOUSE - Med Int ), Centre Hospitalier Emile Muller [Mulhouse] ( CH E.Muller Mulhouse ), Groupe Hospitalier de Territoire Haute Alsace ( GHTHA ) -Groupe Hospitalier de Territoire Haute Alsace ( GHTHA ), Centre Hospitalier Régional Universitaire [Montpellier] ( CHRU Montpellier ), Assistance publique - Hôpitaux de Paris (AP-HP)-CHU Cochin [AP-HP], Contrôle de la Réponse Immune B et des Lymphoproliférations ( CRIBL ), Université de Limoges ( UNILIM ) -Génomique, Environnement, Immunité, Santé, Thérapeutique ( GEIST FR CNRS 3503 ) -Centre National de la Recherche Scientifique ( CNRS ), Université de Poitiers-Centre hospitalier universitaire de Poitiers ( CHU Poitiers ), Médecine Interne ( VALENCE - Med Int ), Cytokines, hématopoïèse et réponse immune ( CHRI ), Université Paris Descartes - Paris 5 ( UPD5 ) -Institut National de la Santé et de la Recherche Médicale ( INSERM ) -Centre National de la Recherche Scientifique ( CNRS ), Assistance publique - Hôpitaux de Paris (AP-HP)-CHU Pitié-Salpêtrière [APHP], Université Paris-Sud - Paris 11 ( UP11 ) -Institut National de la Santé et de la Recherche Médicale ( INSERM ), Assistance publique - Hôpitaux de Paris (AP-HP)-CHU Tenon [APHP], Biologie et thérapeutique des pathologies immunitaires ( BTPI ), Université Pierre et Marie Curie - Paris 6 ( UPMC ) -Centre National de la Recherche Scientifique ( CNRS ), Université de Versailles Saint-Quentin-en-Yvelines (UVSQ)-Université Paris-Sud - Paris 11 (UP11)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Hôpital Paul Brousse-Institut National de la Santé et de la Recherche Médicale (INSERM), Université de Brest (UBO)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Institut Brestois Santé Agro Matière (IBSAM), Service Médecine interne et immunopathologie clinique [CHU Toulouse], Pôle IUCT [CHU Toulouse], Centre Hospitalier Universitaire de Toulouse (CHU Toulouse)-Centre Hospitalier Universitaire de Toulouse (CHU Toulouse), Université de Rennes (UR)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Structure Fédérative de Recherche en Biologie et Santé de Rennes ( Biosit : Biologie - Santé - Innovation Technologique ), CHU Pitié-Salpêtrière [AP-HP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU), Institut Brestois Santé Agro Matière (IBSAM), Université de Brest (UBO)-Université de Brest (UBO)-Université de Brest (UBO), Centre National de la Recherche Scientifique (CNRS)-Université de Limoges (UNILIM)-Génomique, Environnement, Immunité, Santé, Thérapeutique (GEIST FR CNRS 3503), and Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)
Subjects: Male, MESH : Retrospective Studies, [SDV]Life Sciences [q-bio], MESH : Aged, 030232 urology & nephrology, Kaplan-Meier Estimate, medicine.disease_cause, Severity of Illness Index, 0302 clinical medicine, Epidemiology, Immunology and Allergy, MESH : Female, MESH: Data Collection, MESH: Aged, MESH : Prognosis, MESH: Middle Aged, Data Collection, Mortality rate, MESH: Vasculitis, Middle Aged, Prognosis, 3. Good health, MESH : Vasculitis, Cryoglobulinemia, Area Under Curve, Female, MESH : Severity of Illness Index, MESH : Data Collection, Vasculitis, medicine.medical_specialty, MESH: Genetic Diseases, Inborn, MESH: Cryoglobulinemia, MESH : Male, Hepatitis C virus, Immunology, Renal function, General Biochemistry, Genetics and Molecular Biology, MESH: Prognosis, MESH : Kaplan-Meier Estimate, 03 medical and health sciences, MESH : Genetic Diseases, Inborn, Rheumatology, Internal medicine, MESH: Severity of Illness Index, Severity of illness, medicine, Humans, MESH : Middle Aged, In patient, MESH: Kaplan-Meier Estimate, Aged, Retrospective Studies, 030203 arthritis & rheumatology, MESH: Humans, [ SDV ] Life Sciences [q-bio], business.industry, MESH : Humans, Genetic Diseases, Inborn, Retrospective cohort study, MESH: Retrospective Studies, medicine.disease, MESH : Cryoglobulinemia, MESH: Male, Surgery, MESH: Area Under Curve, MESH : Area Under Curve, business, MESH: Female
Abstract: International audience; BACKGROUND: Data on the prognosis of non-infectious mixed cryoglobulinaemia vasculitis (CryoVas) in the era of hepatitis C virus screening are lacking. METHODS: The French multicentre and retrospective CryoVas survey included 242 patients with non-infectious mixed CryoVas. Causes of death and prognostic factors of survival were assessed and a prognostic score was determined to predict survival at 5 years. RESULTS: After a median follow-up of 35 months, 42 patients (17%) died. Causes of death were mainly serious infections (50%) and vasculitis flare (19%). One-, 2-, 5- and 10-year overall survival rates were 91%, 89%, 79% and 65%, respectively. A prognostic score, the CryoVas score (CVS), for the prediction of survival at 5 years was devised. Pulmonary and gastrointestinal involvement, glomerular filtration rate 65 years were independently associated with death. At 5 years the death rates were 2.6%, 13.1%, 29.6% and 38.5% for a CVS of 0, 1, 2 and ≥3, respectively. At 1 year the death rates were 0%, 3.2%, 18.5% and 30.8% for a CVS of 0, 1, 2 and ≥3, respectively. The CVS was strongly correlated with the Five Factor Score (FFS) 2009, another prognostic score validated in primary necrotising vasculitis (r=0.82; p65 years, pulmonary and gastrointestinal involvement and renal failure. A score including these variables is significantly associated with the prognosis.
Published: 2013
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32. Management of noninfectious mixed cryoglobulinemia vasculitis: data from 242 cases included in the CryoVas survey

Author: Antoine Huart, Estibaliz Lazaro, P. Belenotti, Olivier Hermine, David Launay, Guillaume Le Guenno, Thierry Zenone, Adeline Lacraz, Patrice Cacoub, Evguenia Krastinova, Franck Bridoux, Patricia Senet, P. Rullier, Elisabeth Diot, Xavier Mariette, Luc de Saint-Martin, Thomas Quemeneur, Isabelle Marie, Emmanuelle Plaisier, Benjamin Terrier, Fabrice Carrat, Fabrice Bonnet, Olivier Hinschberger, Jean-Emmanuel Kahn, Jean-Marc Léger, Immunologie - Immunopathologie - Immunothérapeutique (I3), Université Pierre et Marie Curie - Paris 6 (UPMC)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Centre National de la Recherche Scientifique (CNRS), Centre de recherche en épidémiologie et santé des populations (CESP), Université de Versailles Saint-Quentin-en-Yvelines (UVSQ)-Université Paris-Sud - Paris 11 (UP11)-Assistance publique - Hôpitaux de Paris (AP-HP) (APHP)-Hôpital Paul Brousse-Institut National de la Santé et de la Recherche Médicale (INSERM), Service de Médecine Interne [Rouen], Université de Rouen Normandie (UNIROUEN), Normandie Université (NU)-Normandie Université (NU)-CHU Rouen, Normandie Université (NU), Physiopathologie et biothérapies des maladies inflammatoires et autoimmunes, Normandie Université (NU)-Normandie Université (NU)-Institut National de la Santé et de la Recherche Médicale (INSERM), Department of Internal Medicine, Centre Hospitalier Régional Universitaire [Lille] (CHRU Lille), Service de Néphrologie-Transplantation-Dialyse, CHU Bordeaux [Bordeaux]-Groupe hospitalier Pellegrin, Service de Médecine Interne (MARSEILLE - Med Int), Assistance Publique - Hôpitaux de Marseille (APHM), Département de Médecine Interne et Pneumologie [Brest] (DMIP - Brest), Centre Hospitalier Régional Universitaire de Brest (CHRU Brest), Groupe d'Etude de la Thrombose de Bretagne Occidentale (GETBO), Université de Brest (UBO)-Institut Brestois Santé Agro Matière (IBSAM), Université de Brest (UBO), Département de Médecine Interne (VALENCIENNES - Med Int), CH Valenciennes, Service de Néphrologie et Immunopathologie Clinique, Centre Hospitalier Universitaire de Toulouse, PRES Université de Toulouse, Foie, métabolismes et cancer, Université de Rennes 1 (UR1), Université de Rennes (UNIV-RENNES)-Université de Rennes (UNIV-RENNES)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Structure Fédérative de Recherche en Biologie et Santé de Rennes ( Biosit : Biologie - Santé - Innovation Technologique ), Centre Hospitalier Universitaire Estaing, CHU Estaing, Laboratoire d'Immunologie (EA 2686), Université de Lille, Droit et Santé, Service de Médecine Interne B, Centre Hospitalier Régional Universitaire [Montpellier] (CHRU Montpellier), Service de Médecine Interne, Hôpital Bretonneau, Service de médecine interne et maladies tropicales, CHU Bordeaux [Bordeaux]-Groupe hospitalier Saint-André, Contrôle de la Réponse Immune B et des Lymphoproliférations (CRIBL), Centre National de la Recherche Scientifique (CNRS)-Université de Limoges (UNILIM)-Génomique, Environnement, Immunité, Santé, Thérapeutique (GEIST FR CNRS 3503), Service de néphrologie - hémodialyse et transplantation rénale, Centre hospitalier universitaire de Poitiers (CHU Poitiers), Médecine Interne (VALENCE - Med Int), Centre hospitalier de Valence, Epidémiologie des maladies infectieuses et modélisation (ESIM), Université Pierre et Marie Curie - Paris 6 (UPMC)-Institut National de la Santé et de la Recherche Médicale (INSERM), Cytokines, hématopoïèse et réponse immune (CHRI), Université Paris Descartes - Paris 5 (UPD5)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Centre National de la Recherche Scientifique (CNRS), Service de neurologie 1 [CHU Pitié-Salpétrière], Assistance publique - Hôpitaux de Paris (AP-HP) (APHP)-CHU Pitié-Salpêtrière [APHP], Régulation de la réponse immune, infection VIH-1 et autoimmunité, Université Paris-Sud - Paris 11 (UP11)-Institut National de la Santé et de la Recherche Médicale (INSERM), Service de Dermatologie [CHU Tenon], Assistance publique - Hôpitaux de Paris (AP-HP) (APHP)-CHU Tenon [APHP], Remodelage et Reparation du Tissu Renal, Service de médecine interne [CHU Pitié-Salpétrière], Biologie et thérapeutique des pathologies immunitaires (BTPI), Centre National de la Recherche Scientifique (CNRS)-Université Pierre et Marie Curie - Paris 6 (UPMC), Immunologie - Immunopathologie - Immunothérapeutique ( I3 ), Université Pierre et Marie Curie - Paris 6 ( UPMC ) -Institut National de la Santé et de la Recherche Médicale ( INSERM ) -Centre National de la Recherche Scientifique ( CNRS ), Centre de recherche en épidémiologie et santé des populations ( CESP ), Université de Versailles Saint-Quentin-en-Yvelines ( UVSQ ) -Université Paris-Sud - Paris 11 ( UP11 ) -Assistance publique - Hôpitaux de Paris (AP-HP)-Hôpital Paul Brousse-Institut National de la Santé et de la Recherche Médicale ( INSERM ), CHU Rouen-Université de Rouen Normandie ( UNIROUEN ), Normandie Université ( NU ) -Normandie Université ( NU ), Université de Rouen Normandie ( UNIROUEN ), Normandie Université ( NU ) -Normandie Université ( NU ) -Institut National de la Santé et de la Recherche Médicale ( INSERM ), Centre Hospitalier Régional Universitaire [Lille] ( CHRU Lille ), Service de Médecine Interne ( MARSEILLE - Med Int ), Assistance Publique - Hôpitaux de Marseille ( APHM ), Département de Médecine Interne et Pneumologie [Brest] ( DMIP - Brest ), Centre Hospitalier Régional Universitaire de Brest ( CHRU Brest ), Groupe d'Etude de la Thrombose de Bretagne Occidentale ( GETBO ), Université de Brest ( UBO ), Département de Médecine Interne ( VALENCIENNES - Med Int ), Université de Rennes 1 ( UR1 ), Université de Rennes ( UNIV-RENNES ) -Université de Rennes ( UNIV-RENNES ) -Institut National de la Santé et de la Recherche Médicale ( INSERM ) -Structure Fédérative de Recherche en Biologie et Santé de Rennes ( Biosit : Biologie - Santé - Innovation Technologique ), Laboratoire d'Immunologie ( EA 2686 ), Centre Hospitalier Régional Universitaire [Montpellier] ( CHRU Montpellier ), Contrôle de la Réponse Immune B et des Lymphoproliférations ( CRIBL ), Centre National de la Recherche Scientifique ( CNRS ) -Université de Limoges ( UNILIM ) -Génomique, Environnement, Immunité, Santé, Thérapeutique ( GEIST FR CNRS 3503 ), Université de Poitiers-Centre hospitalier universitaire de Poitiers ( CHU Poitiers ), Médecine Interne ( VALENCE - Med Int ), Epidémiologie des maladies infectieuses et modélisation ( ESIM ), Université Pierre et Marie Curie - Paris 6 ( UPMC ) -Institut National de la Santé et de la Recherche Médicale ( INSERM ), Cytokines, hématopoïèse et réponse immune ( CHRI ), Université Paris Descartes - Paris 5 ( UPD5 ) -Institut National de la Santé et de la Recherche Médicale ( INSERM ) -Centre National de la Recherche Scientifique ( CNRS ), Assistance publique - Hôpitaux de Paris (AP-HP)-CHU Pitié-Salpêtrière [APHP], Université Paris-Sud - Paris 11 ( UP11 ) -Institut National de la Santé et de la Recherche Médicale ( INSERM ), Assistance publique - Hôpitaux de Paris (AP-HP)-CHU Tenon [APHP], Biologie et thérapeutique des pathologies immunitaires ( BTPI ), Université Pierre et Marie Curie - Paris 6 ( UPMC ) -Centre National de la Recherche Scientifique ( CNRS ), Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Université Paris-Sud - Paris 11 (UP11)-Hôpital Paul Brousse-Institut National de la Santé et de la Recherche Médicale (INSERM)-Université de Versailles Saint-Quentin-en-Yvelines (UVSQ), Service de Médecine Interne [CHU Rouen], CHU Rouen, Normandie Université (NU)-Normandie Université (NU)-Université de Rouen Normandie (UNIROUEN), Université de Brest (UBO)-Université de Brest (UBO), CHU Clermont-Ferrand, Université de Limoges (UNILIM)-Génomique, Environnement, Immunité, Santé, Thérapeutique (GEIST FR CNRS 3503)-Centre National de la Recherche Scientifique (CNRS), Service de Neurologie [CHU Pitié-Salpêtrière], IFR70-CHU Pitié-Salpêtrière [AP-HP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU), Service de dermatologie et allergologie [CHU Tenon], CHU Tenon [AP-HP], Sorbonne Université (SU)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP), Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-CHU Pitié-Salpêtrière [AP-HP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU)-Sorbonne Université (SU), Université Pierre et Marie Curie - Paris 6 (UPMC)-Centre National de la Recherche Scientifique (CNRS), Université de Versailles Saint-Quentin-en-Yvelines (UVSQ)-Université Paris-Sud - Paris 11 (UP11)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Hôpital Paul Brousse-Institut National de la Santé et de la Recherche Médicale (INSERM), Université de Brest (UBO)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Institut Brestois Santé Agro Matière (IBSAM), Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP), Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP), Service Médecine interne et immunopathologie clinique [CHU Toulouse], Pôle IUCT [CHU Toulouse], Centre Hospitalier Universitaire de Toulouse (CHU Toulouse)-Centre Hospitalier Universitaire de Toulouse (CHU Toulouse), Université de Rennes (UR)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Structure Fédérative de Recherche en Biologie et Santé de Rennes ( Biosit : Biologie - Santé - Innovation Technologique ), CHU Pitié-Salpêtrière [AP-HP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU), Institut Brestois Santé Agro Matière (IBSAM), and Université de Brest (UBO)-Université de Brest (UBO)-Université de Brest (UBO)
Subjects: Male, MESH: Combined Modality Therapy, MESH : Retrospective Studies, [SDV]Life Sciences [q-bio], MESH : Aged, Arthritis, 030204 cardiovascular system & hematology, Biochemistry, Gastroenterology, MESH: Antibodies, Monoclonal, Cohort Studies, Antibodies, Monoclonal, Murine-Derived, 0302 clinical medicine, Prednisone, Adrenal Cortex Hormones, MESH : Female, MESH: Data Collection, MESH: Cohort Studies, MESH : Algorithms, MESH: Treatment Outcome, MESH: Aged, MESH: Middle Aged, Data Collection, MESH: Vasculitis, Antibodies, Monoclonal, Hematology, Middle Aged, Connective tissue disease, Combined Modality Therapy, 3. Good health, MESH : Vasculitis, MESH : Infection, Treatment Outcome, Cryoglobulinemia, MESH : Antibodies, Monoclonal, Rituximab, Female, medicine.symptom, MESH : Data Collection, Vasculitis, MESH: Infection, Algorithms, medicine.drug, medicine.medical_specialty, MESH: Cryoglobulinemia, MESH : Male, Immunology, MESH : Cohort Studies, MESH: Algorithms, MESH : Treatment Outcome, Infections, MESH : Adrenal Cortex Hormones, MESH: Adrenal Cortex Hormones, 03 medical and health sciences, Internal medicine, medicine, Humans, MESH : Middle Aged, Aged, Retrospective Studies, 030203 arthritis & rheumatology, MESH: Humans, [ SDV ] Life Sciences [q-bio], business.industry, MESH : Humans, MESH: Retrospective Studies, Cell Biology, Skin ulcer, medicine.disease, MESH : Cryoglobulinemia, MESH: Male, MESH : Antibodies, Monoclonal, Murine-Derived, Regimen, Peripheral neuropathy, MESH: Antibodies, Monoclonal, Murine-Derived, business, MESH : Combined Modality Therapy, MESH: Female
Abstract: Data on the clinical spectrum and therapeutic management of noninfectious mixed cryoglobulinemia vasculitis (CryoVas) in the era of hepatitis C virus screening are lacking. We analyzed data from 242 patients with noninfectious mixed CryoVas included in the French multicenter CryoVas survey. Baseline manifestations were purpura (75%), peripheral neuropathy (52%), arthralgia or arthritis (44%), glomerulonephritis (35%), cutaneous ulcers (16%), and cutaneous necrosis (14%). A connective tissue disease was diagnosed in 30% and B-cell non-Hodgkin lymphoma in 22%, whereas the CryoVas was considered to be essential in 48%. With the use of Cox-marginal structural models, rituximab plus corticosteroids showed the greater therapeutic efficacy compared with corticosteroids alone and alkylating agents plus corticosteroids to achieve complete clinical, renal, and immunologic responses and a prednisone dosage < 10 mg/d at 6 months. However, this regimen was also associated with severe infections, particularly when high doses of corticosteroids were used, whereas death rates did not differ between the therapeutic regimens. The role of each of these strategies remains to be defined in well-designed randomized controlled trials.
Published: 2012
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33. Development and validation of a predictive model for death in acquired severe ADAMTS13 deficiency-associated idiopathic thrombotic thrombocytopenic purpura: the French TMA Reference Center experience

Author: Jean-Bernard Palcoux, Bertrand Guidet, Hanspeter Rottensteiner, Dominique Bordessoule, Didier Gruson, Paul Coppo, Claire Pouteil-Noble, Jacques Pourrat, Jean-Paul Mira, Alain Wynckel, Cécile Vigneau, Agnès Veyradier, Claire Presne, Pierre-Yves Boëlle, Cyrielle Assie, Sandrine Malot, Cédric Daubin, Rana Grillberger, Lionel Galicier, Philippe Vanhille, Marc Buffet, Véronique Le Guern, François Provôt, Gabriel Choukroun, Guy Bonmarchand, Mohamed Hamidou, Christiane Mousson, Ygal Benhamou, Pascale Poullin, Elie Azoulay, Eric Rondeau, Service de Médecine Interne [CHU Rouen], CHU Rouen, Normandie Université (NU)-Normandie Université (NU)-Université de Rouen Normandie (UNIROUEN), Normandie Université (NU), CHU Saint-Antoine [AP-HP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU), Epidémiologie des maladies infectieuses et modélisation (ESIM), Université Pierre et Marie Curie - Paris 6 (UPMC)-Institut National de la Santé et de la Recherche Médicale (INSERM), Service d'immunologie et hématologies biologiques [Saint-Antoine], Université Pierre et Marie Curie - Paris 6 (UPMC)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-CHU Saint-Antoine [AP-HP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU)-Sorbonne Université (SU), Baxter Innovations GmbH, Campus Vienna, Service de Néphrologie, Centre Hospitalier Universitaire de Reims (CHU Reims)-Hôpital Maison Blanche, Service de Néphrologie - Médecine Interne, CHU Amiens-Picardie-hôpital Sud, Service d'Hémaphérèse - Médecine Interne, Assistance Publique - Hôpitaux de Marseille (APHM)-Hôpital de la Conception [CHU - APHM] (LA CONCEPTION), Service de néphrologie, Centre Hospitalier Régional Universitaire [Lille] (CHRU Lille), Service de Réanimation, CHU Bordeaux [Bordeaux]-Hôpital Pellegrin-Tripode, Service de médecine interne [Nantes], Université de Nantes (UN)-Hôtel-Dieu-Centre hospitalier universitaire de Nantes (CHU Nantes), Service d'Hématologie clinique et thérapie cellulaire [CHU Limoges], CHU Limoges, Service de Néphrologie - Immunologie Clinique [Toulouse], CHU Toulouse [Toulouse]-Hôpital de Rangueil, CHU Toulouse [Toulouse]-PRES Université de Toulouse, Service de Réanimation polyvalente, Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Hôpital Cochin [AP-HP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Université Paris Descartes - Paris 5 (UPD5), Service de Médecine Interne, Service de néphrologie et transplantation, Hôpital Edouard Herriot [CHU - HCL], Hospices Civils de Lyon (HCL)-Hospices Civils de Lyon (HCL), Service de réanimation médicale [CHU Caen], CHU Caen, Normandie Université (NU)-Tumorothèque de Caen Basse-Normandie (TCBN)-Normandie Université (NU)-Tumorothèque de Caen Basse-Normandie (TCBN)-Université de Caen Normandie (UNICAEN), Service de médecine interne et néphrologie, CH Valenciennes, CHU Tenon [AP-HP], Sorbonne Université (SU)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP), Service de Néphrologie pédiatrique, CHU Clermont-Ferrand, Service de néphrologie (CHU de Dijon), Centre Hospitalier Universitaire de Dijon - Hôpital François Mitterrand (CHU Dijon), Service de néphrologie [Rennes], Université de Rennes 1 (UR1), Université de Rennes (UNIV-RENNES)-Université de Rennes (UNIV-RENNES)-Hôpital Pontchaillou-CHU Pontchaillou [Rennes], Service de réanimation médicale [CHU Rouen], Hôpital Charles Nicolle [Rouen]-CHU Rouen, Service de Réanimation Médicale [CHU Saint-Antoine], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-CHU Saint-Antoine [AP-HP], Service d'immunologie clinique, Université Paris Diderot - Paris 7 (UPD7)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Groupe Hospitalier Saint Louis - Lariboisière - Fernand Widal [Paris], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP), Service de réanimation médicale, Université Paris Diderot - Paris 7 (UPD7)-Groupe Hospitalier Saint Louis - Lariboisière - Fernand Widal [Paris], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP), Service d'Hématologie Biologique [Béclère], AP-HP - Hôpital Antoine Béclère [Clamart], Etablissement Français du Sang (CS/2002/009), GIS-Institut des Maladies Rares (GIS MR0428)., Centre Hospitalier Universitaire de Toulouse (CHU Toulouse)-PRES Université de Toulouse, Université de Caen Normandie (UNICAEN), Normandie Université (NU)-Normandie Université (NU)-CHU Caen, Normandie Université (NU)-Tumorothèque de Caen Basse-Normandie (TCBN)-Tumorothèque de Caen Basse-Normandie (TCBN), Université de Rennes (UR)-Hôpital Pontchaillou-CHU Pontchaillou [Rennes], Hôpital Charles Nicolle [Rouen], Normandie Université (NU)-Normandie Université (NU)-CHU Rouen, Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Université Paris Diderot - Paris 7 (UPD7)-Groupe Hospitalier Saint Louis - Lariboisière - Fernand Widal [Paris], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-AP-HP - Hôpital Antoine Béclère [Clamart], Service de Médecine Interne [Rouen], CHU Rouen-Université de Rouen Normandie ( UNIROUEN ), Normandie Université ( NU ) -Normandie Université ( NU ), Centre de Référence des microangiopathies thrombotiques ( CNR-MAT ), service d'hématologie-CHU Saint-Antoine [APHP], Epidémiologie des maladies infectieuses et modélisation ( ESIM ), Université Pierre et Marie Curie - Paris 6 ( UPMC ) -Institut National de la Santé et de la Recherche Médicale ( INSERM ), Service d'immunologie et hématologies biologiques, CHU Saint-Antoine [APHP]-Assistance publique - Hôpitaux de Paris (AP-HP)-Université Pierre et Marie Curie - Paris 6 ( UPMC ), Centre Hospitalier Universitaire de Reims ( CHU Reims ) -Hôpital Maison Blanche, CHU Amiens-Picardie-Hôpital Sud, Assistance Publique - Hôpitaux de Marseille ( APHM ) -Hôpital de la Conception [CHU - APHM] ( LA CONCEPTION ), Centre Hospitalier Régional Universitaire [Lille] ( CHRU Lille ), Université de Nantes ( UN ) -Hôtel-Dieu-Centre hospitalier universitaire de Nantes ( CHU Nantes ), Assistance publique - Hôpitaux de Paris (AP-HP)-CHU Cochin [AP-HP]-Université Paris Descartes - Paris 5 ( UPD5 ), Université Claude Bernard Lyon 1 ( UCBL ), Université de Lyon-Université de Lyon-Hospices Civils de Lyon ( HCL ) -Centre Hospitalier Lyon Sud [CHU - HCL] ( CHLS ), Hospices Civils de Lyon ( HCL ), Service de Réanimation Médicale, Université Pierre et Marie Curie - Paris 6 ( UPMC ) -Assistance publique - Hôpitaux de Paris (AP-HP)-CHU Tenon [APHP], Centre Hospitalier Universitaire de Dijon - Hôpital François Mitterrand ( CHU Dijon ), Université de Rennes 1 ( UR1 ), Université de Rennes ( UNIV-RENNES ) -Université de Rennes ( UNIV-RENNES ) -Hôpital Pontchaillou-CHU Pontchaillou [Rennes], Service de réanimation médicale [Rouen], Hôpital Charles Nicolle [Rouen]-CHU Rouen-Université de Rouen Normandie ( UNIROUEN ), Assistance publique - Hôpitaux de Paris (AP-HP)-CHU Saint-Antoine [APHP], Assistance publique - Hôpitaux de Paris (AP-HP)-Université Paris Diderot - Paris 7 ( UPD7 ) -Groupe Hospitalier Saint Louis - Lariboisière - Fernand Widal [Paris], Université Paris-Sud - Paris 11 ( UP11 ) -Assistance publique - Hôpitaux de Paris (AP-HP)-Hôpital Antoine Béclère, De Villemeur, Hervé, and Service d'immunologie et hématologies biologiques [CHU Saint-Antoine]
Subjects: [SDV.MHEP.HEM] Life Sciences [q-bio]/Human health and pathology/Hematology, Adult, Male, medicine.medical_specialty, Thrombotic thrombocytopenic purpura, ADAMTS13 Protein, 030204 cardiovascular system & hematology, Cohort Studies, 03 medical and health sciences, 0302 clinical medicine, rituximab, Internal medicine, plasma exchange, medicine, Humans, [ SDV.MHEP.HEM ] Life Sciences [q-bio]/Human health and pathology/Hematology, Registries, thrombotic thrombocytopenic purpura, prognostic factor, Aged, Purpura, Thrombocytopenic, Idiopathic, Framingham Risk Score, Acquired Thrombotic Thrombocytopenic Purpura, Models, Statistical, business.industry, Reproducibility of Results, [SDV.MHEP.HEM]Life Sciences [q-bio]/Human health and pathology/Hematology, Hematology, Middle Aged, medicine.disease, Prognosis, ADAMTS13, 3. Good health, Surgery, ADAM Proteins, ROC Curve, Cohort, Rituximab, Female, Caplacizumab, Original Articles and Brief Reports, business, 030215 immunology, medicine.drug, Cohort study
Abstract: Acquired thrombotic thrombocytopenic purpura is still associated with a 10-20% death rate. It has still not been possible to clearly identify early prognostic factors of death. This study involved thrombotic thrombocytopenic purpura patients with acquired severe (10% of normal activity) ADAMTS13 deficiency and aimed to identify prognostic factors associated with 30-day death.The study involved a prospective cohort of patients and was carried out between October 2000 and August 2010. A validation cohort of patients was set up from September 2010 to August 2011. Altogether, 281 (analysis cohort) and 66 (validation cohort) consecutive adult thrombotic thrombocytopenic purpura patients with acquired severe ADAMTS13 deficiency were enrolled. The study evaluated 30-day mortality after treatment initiation according to characteristics at inclusion.Non-survivors (11%) were older (P=10(-6)) and more frequently presented arterial hypertension (P=5.10(-4)) and ischemic heart disease (P=0.013). Prognosis was increasingly poor with age (P=0.004). On presentation, cerebral manifestations were more frequent in non-survivors (P=0.018) and serum creatinine level was higher (P=0.008). The most significant independent variables determining death were age, severe cerebral involvement and LDH level 10 N or over. A 3-level risk score for early death was defined and confirmed in the validation cohort using these variables, with higher values corresponding to increased risk of early death.A risk score for early death was defined in patients with thrombotic thrombocytopenic purpura and validated on an independent cohort. This score should help to stratify early treatment and identify patients with a worse prognosis.
Published: 2012
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34. Renal lesions associated with IgM-secreting monoclonal proliferations: revisiting the disease spectrum

Author: Benoit Georges, Cécile Toly, René Cuvelier, Xavier Belenfant, Vincent Audard, Benjamin Deroure, Pierre Ronco, Philippe Vanhille, Brigitte Surin, Béatrice Mougenot, Fadi Fakhouri, Pierre Aucouturier, Service de néphrologie et transplantation, Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Hôpital Henri Mondor-Université Paris-Est Créteil Val-de-Marne - Paris 12 (UPEC UP12), Institut Mondor de recherche biomédicale (IMRB), Institut National de la Santé et de la Recherche Médicale (INSERM)-Université Paris-Est Créteil Val-de-Marne - Paris 12 (UPEC UP12), Département de néphrologie, Centre hospitalier de Namur, Service de médecine interne et néphrologie, CH Valenciennes, CHU Tenon [AP-HP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU), Maladies à prions et système immunitaire, IFR65-Institut National de la Santé et de la Recherche Médicale (INSERM), Service de néphrologie et de transplantation rénale, Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-AP-HP Hôpital Bicêtre (Le Kremlin-Bicêtre), Service de néphrologie pédiatrique [CHU Necker], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-CHU Necker - Enfants Malades [AP-HP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP), Service de néphrologie, hôpital de Mouscron, hôpital de Montreuil, Remodelage et Reparation du Tissu Renal, Université Pierre et Marie Curie - Paris 6 (UPMC)-Institut National de la Santé et de la Recherche Médicale (INSERM), Service d'Anatomie et cytologie pathologiques [CHU Tenon], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU), Guellaen, Georges, Sorbonne Université (SU)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP), Service d'anatomie pathologique [CHU Tenon], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-CHU Tenon [AP-HP], Sorbonne Université (SU)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU), Assistance publique - Hôpitaux de Paris (AP-HP)-Hôpital Henri Mondor-Université Paris-Est Créteil Val-de-Marne - Paris 12 ( UPEC UP12 ), Institut Mondor de recherche biomédicale ( IMRB ), Institut National de la Santé et de la Recherche Médicale ( INSERM ) -Université Paris-Est Créteil Val-de-Marne - Paris 12 ( UPEC UP12 ), Service d'hématologie et d'immunologie, Université Pierre et Marie Curie - Paris 6 ( UPMC ) -Assistance publique - Hôpitaux de Paris (AP-HP)-CHU Tenon [APHP], IFR65-Institut National de la Santé et de la Recherche Médicale ( INSERM ), Assistance publique - Hôpitaux de Paris (AP-HP)-AP-HP Hôpital Bicêtre (Le Kremlin-Bicêtre), CHU Necker - Enfants Malades [AP-HP]-Assistance publique - Hôpitaux de Paris (AP-HP), Université Pierre et Marie Curie - Paris 6 ( UPMC ) -Institut National de la Santé et de la Recherche Médicale ( INSERM ), and Assistance publique - Hôpitaux de Paris (AP-HP)-CHU Tenon [APHP]
Subjects: Male, Pathology, medicine.medical_specialty, Lymphoma, B-Cell, Nephrotic Syndrome, Epidemiology, Glomerulonephritis, Membranoproliferative, 030232 urology & nephrology, 030204 cardiovascular system & hematology, Critical Care and Intensive Care Medicine, Nephropathy, 03 medical and health sciences, 0302 clinical medicine, medicine, [SDV.BBM] Life Sciences [q-bio]/Biochemistry, Molecular Biology, Humans, [SDV.BBM]Life Sciences [q-bio]/Biochemistry, Molecular Biology, [ SDV.BBM ] Life Sciences [q-bio]/Biochemistry, Molecular Biology, Aged, Cell Proliferation, Retrospective Studies, Transplantation, B-Lymphocytes, biology, business.industry, Waldenstrom macroglobulinemia, Fanconi syndrome, Antibodies, Monoclonal, Amyloidosis, Lymphoma, B-Cell, Marginal Zone, Middle Aged, medicine.disease, Cryoglobulinemia, Lymphoproliferative Disorders, 3. Good health, Treatment Outcome, Immunoglobulin M, Nephrology, Clinical Nephrology, Monoclonal, biology.protein, Neoplastic cell, Female, Kidney Diseases, Nephrocalcinosis, Waldenstrom Macroglobulinemia, business, Multiple Myeloma
Abstract: The spectrum of renal diseases associated with proliferations of the B cell lineage is continuously expanding. Most of them are caused by deposition of a monoclonal Ig or a fragment thereof, whereas others include neoplastic cell infiltration, nephrocalcinosis, and infection-related complications. Solomon et al. (1) first demonstrated that human renal lesions associated with deposition of monoclonal lights chains (LC) could be reproduced in mice by intraperitoneal injection. Subsequent studies have shown that the pattern of renal lesions associated with the deposition of monoclonal Ig mostly depends on biochemical and structural abnormalities of Ig (2). Most renal complications occur in patients who produce free LC with or without complete IgG molecules. Renal complications of malignant IgM-secreting proliferations are rare. Their incidence seems to have decreased, mostly because of improved therapy of Waldenstrom macroglobulinemia (WM), the major cause of those nephropathies. First reviewed by Morel-Maroger et al. (3), WM-related nephropathies include characteristic intracapillary deposits of IgM with or without cryoglobulinemia, AL-amyloidosis, and infiltration of the interstitium by neoplastic lymphoplasmacytic cells. Subsequent case reports included immunotactoid and nonamyloid fibrillary glomerulopathy (4,5), cryoglobulinemia-related glomerulonephritis (6), and crescentic glomerulonephritis (7,8). Cases of cast nephropathy (9), Fanconi syndrome (10), and LC deposition disease (11) as a result of free LC toxicity have also been reported in patients who presented with a circulating monoclonal IgM. In this study, we retrospectively analyzed 14 patients with renal lesions associated with an IgM-secreting proliferation with the aims of reappraising the relative prevalence of the various renal lesions and underlying hematologic disorders, the characteristics of the nephropathies, and the patients’ outcome. We included both renal lesions that were caused by cell infiltration and those that were caused by the deposition of the monoclonal component (complete IgM and/or isolated LC) to cover the full disease spectrum.
Published: 2008
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35. Clinical relevance of soluble HLA class I molecules in Waldenstrom Macroglobulinemia

Author: Moreau, Anne-Sophie, Sebti, Yasmine, Duhamel, Alain, Roccaro, Aldo M., Coiteux, Valérie, Gastinne, Thomas, Le Friec, Gaëlle, Burwick, Nicholas, Amiot, Laurence, Ho, Allen W., Poulain, Stephanie, Hennache, Bernadette, Hunter, Zachary R., Dessaint, Jean-Paul, Ghobrial, Irene M., Treon, Steven P., Facon, Thierry, Zorn, Emmanuel, Leleu, Xavier, Institut Mondor de Recherche Biomédicale (IMRB), Institut National de la Santé et de la Recherche Médicale (INSERM)-IFR10-Université Paris-Est Créteil Val-de-Marne - Paris 12 (UPEC UP12), Department of Medical oncology, Harvard Medical School [Boston] (HMS)-Dana-Farber Cancer Institute [Boston], Récepteurs nucléaires, maladies cardiovasculaires et diabète - U 1011 (RNMCD), Institut Pasteur de Lille, Réseau International des Instituts Pasteur (RIIP)-Réseau International des Instituts Pasteur (RIIP)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Université de Lille-Centre Hospitalier Régional Universitaire [Lille] (CHRU Lille), Service de Biostatistiques [CHRU Lille], Centre Hospitalier Régional Universitaire [Lille] (CHRU Lille), MRC Centre for Transplantation, Service d'hématologie clinique, Université de Rennes (UR)-Hôpital Pontchaillou, Groupe d'Etude de la Reproduction Chez l'Homme et les Mammiferes (GERHM), Université de Rennes (UR)-Institut National de la Santé et de la Recherche Médicale (INSERM), Laboratoire de Biologie Moleculaire, CH Valenciennes, Laboratoire de Biochimie, Laboratoire d'Immunologie (EA 2686), Université de Lille, Droit et Santé, Hôpital Claude Huriez [Lille], CHU Lille, Transplantation Biology Research Center, Massachusetts General Hospital [Boston], Franco-American Fulbright Foundation, Fondation pour la Recherche Medicale, Association pour la Recherche contre le Cancer, Institut National de la Santé et de la Recherche Médicale (INSERM)-Université Paris-Est Créteil Val-de-Marne - Paris 12 (UPEC UP12)-IFR10, Dana-Farber Cancer Institute [Boston]-Harvard Medical School [Boston] (HMS), Réseau International des Instituts Pasteur (RIIP)-Réseau International des Instituts Pasteur (RIIP)-Université de Lille-Centre Hospitalier Régional Universitaire [Lille] (CHRU Lille)-Institut National de la Santé et de la Recherche Médicale (INSERM), Laboratoire de Biostatistique et Biomathématiques, Université de Rennes 1 (UR1), Université de Rennes (UNIV-RENNES)-Université de Rennes (UNIV-RENNES)-Hôpital Pontchaillou, Institut National de la Santé et de la Recherche Médicale (INSERM)-Université de Rennes 1 (UR1), Université de Rennes (UNIV-RENNES)-Université de Rennes (UNIV-RENNES), and Université de Rennes (UNIV-RENNES)-Université de Rennes (UNIV-RENNES)-Institut National de la Santé et de la Recherche Médicale (INSERM)
Subjects: Male, MESH: Humans, MESH: Middle Aged, MESH: Waldenstrom Macroglobulinemia, IgM-monoclonal gammopathy of undetermined significance, Histocompatibility Antigens Class I, [SDV.MHEP.HEM]Life Sciences [q-bio]/Human health and pathology/Hematology, serum HLA-Gs molecule, Middle Aged, serum total soluble HLA-I molecule, Prognosis, MESH: Male, MESH: Prognosis, MESH: Histocompatibility Antigens Class I, hemic and lymphatic diseases, Humans, Female, Waldenstrom Macroglobulinemia, MESH: Female
Abstract: International audience; OBJECTIVES: Waldenstrom Macroglobulinemia (WM) is a B-cell neoplasm characterised by secretion of IgM by lymphoplasmacytic bone marrow cells and by cytopenias and hypogammaglobulinemia in a subset of patients. Beta-2 microglobulin (b2m) is a major prognostic factor in WM and the heavy chain of HLA class I molecules, which are known to have immunosuppressive properties and have been implicated in the pathogeny of several malignancies. METHODS: We assessed the serum levels of the total soluble HLA-I molecules and the HLA-Gs molecules in 105 patients with IgM-related disorders [WM (n = 42) and IgM MGUS (n = 63)], and compared the results to 41 healthy subjects. RESULTS: We found higher levels of HLA-Is in WM, compared to IgM MGUS and healthy donors. HLA-Gs levels were similar in WM and in IgM MGUS, but higher than in healthy donors. The association between HLA-Is at the cut-off of 1.8 microg/mL and known markers of poor prognosis was then evaluated among WM patients using univariate and multivariate methods. Based on this, high HLA-Is level was strongly associated with high serum beta2M level >3 mg/L [OR = 2, (CI 95% 1.1-5.7); P = 0.04], age > 65 yrs [OR = 1.5, (CI 95% 0.5-4.1), P = 0.06] and haemoglobin < or =11.5 g/dL [OR = 3.3, (CI 95% 1.2-9.7); P = 0.03]. High levels of serum HLA-Is were also found in patients with cryoglobulinemia, however irrespectively of WM or IgM-MGUS status. CONCLUSION: Together our results suggest a possible role for soluble MHC class I molecules in WM disease. Further investigations are necessary to further demonstrate the prognostic impact of soluble MHC class I molecules in Waldenstrom Macroglobulinemia.
Published: 2008
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36. Clinical and histological characteristics of renal AA amyloidosis: a retrospective study of 68 cases with a special interest to amyloid-associated inflammatory response. : Clinical and histological characteristics of renal AA amyloidosis

Author: Verine, Jérôme, Mourad, Nathalie, Desseaux, Kristell, Vanhille, Philippe, Noël, Laure-Hélène, Beaufils, Hélène, Grateau, Gilles, Janin, Anne, Droz, Dominique, Service d'anatomo-pathologie [Paris], Assistance publique - Hôpitaux de Paris (AP-HP)-Université Paris Diderot - Paris 7 ( UPD7 ) -Groupe Hospitalier Saint Louis - Lariboisière - Fernand Widal [Paris], Gvh et Gvl : Physiopathologie Chez l'Homme et Chez l'Animal, Incidence et Role Therapeutique, Université Paris Diderot - Paris 7 ( UPD7 ) -Institut National de la Santé et de la Recherche Médicale ( INSERM ), Service d'informatique médicale, Service de médecine interne et néphrologie, CH Valenciennes, Mecanismes de l'Inflammation et de l'Adherence Cellulaire Dans les Maladies Renales, Université Paris Descartes - Paris 5 ( UPD5 ) -Institut National de la Santé et de la Recherche Médicale ( INSERM ), Service d'anatomie pathologique neurologique, Université Pierre et Marie Curie - Paris 6 ( UPMC ) -Assistance publique - Hôpitaux de Paris (AP-HP)-CHU Pitié-Salpêtrière [APHP], Service de médecine interne, Assistance publique - Hôpitaux de Paris (AP-HP)-CHU Tenon [APHP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Université Paris Diderot - Paris 7 (UPD7)-Groupe Hospitalier Saint Louis - Lariboisière - Fernand Widal [Paris], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP), Université Paris Diderot - Paris 7 (UPD7)-Institut National de la Santé et de la Recherche Médicale (INSERM), Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Groupe Hospitalier Saint Louis - Lariboisière - Fernand Widal [Paris], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Université Paris Diderot - Paris 7 (UPD7), Université Paris Descartes - Paris 5 (UPD5)-Institut National de la Santé et de la Recherche Médicale (INSERM), Service d'anatomie pathologique neurologique [CHU Pitié-Salpêtrière], Université Pierre et Marie Curie - Paris 6 (UPMC)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-CHU Pitié-Salpêtrière [AP-HP], Sorbonne Université (SU)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU), CHU Tenon [AP-HP], and Sorbonne Université (SU)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)
Subjects: MESH: Inflammation, [ SDV.MHEP.UN ] Life Sciences [q-bio]/Human health and pathology/Urology and Nephrology, MESH: Serum Amyloid A Protein, MESH : Retrospective Studies, MESH : Hematuria, Inflammatory reaction, MESH : Male, Histological forms, MESH : Aged, urologic and male genital diseases, Kidney, [SDV.MHEP.UN]Life Sciences [q-bio]/Human health and pathology/Urology and Nephrology, MESH: Hypertension, MESH: Proteinuria, AA amyloidosis, MESH: Amyloidosis, MESH : Middle Aged, MESH : Female, MESH: Hematuria, MESH: Aged, MESH: Kidney Diseases, MESH: Middle Aged, MESH: Humans, MESH : Proteinuria, MESH : Serum Amyloid A Protein, MESH : Inflammation, urogenital system, MESH : Humans, MESH : Hypertension, MESH: Adult, MESH: Retrospective Studies, MESH : Adult, MESH: Male, Glomerular crescents, MESH : Amyloidosis, MESH : Kidney Diseases, MESH: Female
Abstract: International audience; We retrospectively reviewed the clinicopathological features of a series of 68 renal AA amyloidosis observations collected between 1990 and 2005. The amyloidogenic disease was a chronic infection (40.8%), a chronic inflammation (38%), a tumor (9.9%), a hereditary disease (9.9%), or was undetermined in 1.4% of cases. Nephrotic syndrome and renal insufficiency were noted in 63.1% and 75% of patients, respectively. The distribution pattern of glomerular amyloid deposits was mesangial segmental (14.7%), mesangial nodular (26.5%), mesangiocapillary (32.3%), and hilar (26.5%). Glomerular form was observed in 80.9% of cases and vascular form in 19.1%. AA amyloidosis-related inflammation was noted in 30 patients (44.1%) and appeared as a multinucleated giant cell reaction (27.9%) or a glomerular inflammatory infiltrate (25%), including glomerular crescents (17.6%). At the end of follow-up, 26 patients (38.2%) showed end-stage renal disease. The clinical presentation of glomerular and vascular forms was distinct with a clear predominance of proteinuria in glomerular form. Inflammatory reaction was preferentially observed in biopsies with a codeposition of immunoglobulin chains and/or complement factors in AA amyloid deposits. The distribution pattern of glomerular amyloid deposits and glomerular inflammatory reaction were independent factors influencing proteinuria level. Tubular atrophy, abundance, and distribution pattern of glomerular amyloid deposits at the time of biopsy were independent predictors of renal outcome. In conclusion, the glomerular involvement appeared as the determining histological factor for clinical manifestations and outcome of renal AA amyloidosis. AA amyloidosis-related inflammation could partly result from an immune response directed against AA fibrils and could induce amyloid resolution and crescents.
Published: 2007
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37. Rituximab versus azathioprine for ANCA-associated vasculitis maintenance therapy: Impact on global disability and health-related quality of life

Author: Pugnet, G., Pagnoux, C., Terrier, B., Elodie Perrodeau, Puéchal, X., Karras, A., Khouatra, C., Aumaître, O., Cohen, P., Maurier, F., Decaux, O., Ninet, J., Gobert, P., Quemeneur, T., Blanchard-Delaunay, C., Godmer, P., Carron, P. -L, Hatron, P. -Y, Limal, N., Hamidou, M., Ducret, M., Daugas, E., Papo, T., Bonnotte, B., Mahr, A., Ravaud, P., Mouthon, L., Guillevin, L., Service de Médecine Interne, Centre Hospitalier Universitaire de Toulouse, PRES Université de Toulouse, Service de Néphrologie et Immunopathologie Clinique, Centre Hospitalier Universitaire de Toulouse, Service de médecine interne et centre de référence des maladies rares [CHU Cochin], Assistance publique - Hôpitaux de Paris (AP-HP)-CHU Cochin [AP-HP], Departement Hospitalo- Universitaire - Inflammation, Immunopathologie, Biothérapie [Paris] ( DHU - I2B ), Université Pierre et Marie Curie - Paris 6 ( UPMC ) -CHU Pitié-Salpêtrière [APHP], Equipe 5 : METHODS - Méthodes de l’évaluation thérapeutique des maladies chroniques ( CRESS - U1153 ), Centre de Recherche Épidémiologie et Statistique Sorbonne Paris Cité ( CRESS (U1153 / UMR_A 1125) ), Institut National de la Recherche Agronomique ( INRA ) -Université Sorbonne Paris Cité ( USPC ) -Institut National de la Santé et de la Recherche Médicale ( INSERM ) -Institut National de la Recherche Agronomique ( INRA ) -Université Sorbonne Paris Cité ( USPC ) -Institut National de la Santé et de la Recherche Médicale ( INSERM ) -Université Paris Descartes - Paris 5 ( UPD5 ), Institut National de la Recherche Agronomique ( INRA ) -Université Sorbonne Paris Cité ( USPC ) -Institut National de la Santé et de la Recherche Médicale ( INSERM ), Service de Néphrologie et Hémodialyse [CHU HEGP], Assistance publique - Hôpitaux de Paris (AP-HP)-Hôpital Européen Georges Pompidou [APHP] ( HEGP ), Service de médecine interne, hôpital Gabriel-Montpied, CHU Clermont-Ferrand, Service de médecine interne, Hôpital Sainte-Blandine-Centre hospitalier régional Metz-Thionville ( CHR Metz-Thionville ), Institut de Génétique et Développement de Rennes ( IGDR ), Université de Rennes 1 ( UR1 ), Université de Rennes ( UNIV-RENNES ) -Université de Rennes ( UNIV-RENNES ) -Centre National de la Recherche Scientifique ( CNRS ) -Structure Fédérative de Recherche en Biologie et Santé de Rennes ( Biosit : Biologie - Santé - Innovation Technologique ), Service de Médecine Interne, Hôpital Edouard Herriot [CHU - HCL], Hospices Civils de Lyon ( HCL ) -Hospices Civils de Lyon ( HCL ), Département de Médecine Interne ( VALENCIENNES - Med Int ), CH Valenciennes, Service de médecine interne [Lille], Centre Hospitalier Régional Universitaire [Lille] ( CHRU Lille ), Service de médecine interne [Nantes], Université de Nantes ( UN ) -Hôtel-Dieu-Centre hospitalier universitaire de Nantes ( CHU Nantes ), Immunopathologie rénale, récepteurs et inflammation, Université Paris Diderot - Paris 7 ( UPD7 ) -Institut National de la Santé et de la Recherche Médicale ( INSERM ), Hôpital Bichat - Claude Bernard, Lipides - Nutrition - Cancer (U866) ( LNC ), Université de Bourgogne ( UB ) -Institut National de la Santé et de la Recherche Médicale ( INSERM ) -AgroSup Dijon - Institut National Supérieur des Sciences Agronomiques, de l'Alimentation et de l'Environnement-Ecole Nationale Supérieure de Biologie Appliquée à la Nutrition et à l'Alimentation de Dijon ( ENSBANA ), Hôpital Saint-Louis, Assistance publique - Hôpitaux de Paris (AP-HP)-Université Paris Diderot - Paris 7 ( UPD7 ), Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Hôpital Cochin [AP-HP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP), Departement Hospitalo- Universitaire - Inflammation, Immunopathologie, Biothérapie [Paris] (DHU - I2B), CHU Pitié-Salpêtrière [AP-HP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU)-Université Pierre et Marie Curie - Paris 6 (UPMC), Equipe 5 : METHODS - Méthodes de l’évaluation thérapeutique des maladies chroniques (CRESS - U1153), Université Paris Descartes - Paris 5 (UPD5)-Centre de Recherche Épidémiologie et Statistique Sorbonne Paris Cité (CRESS (U1153 / UMR_A_1125 / UMR_S_1153)), Institut National de la Recherche Agronomique (INRA)-Université Paris Diderot - Paris 7 (UPD7)-Université Paris Descartes - Paris 5 (UPD5)-Université Sorbonne Paris Cité (USPC)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Institut National de la Recherche Agronomique (INRA)-Université Paris Diderot - Paris 7 (UPD7)-Université Sorbonne Paris Cité (USPC)-Institut National de la Santé et de la Recherche Médicale (INSERM), Centre de Recherche Épidémiologie et Statistique Sorbonne Paris Cité (CRESS (U1153 / UMR_A_1125 / UMR_S_1153)), Institut National de la Recherche Agronomique (INRA)-Université Paris Diderot - Paris 7 (UPD7)-Université Paris Descartes - Paris 5 (UPD5)-Université Sorbonne Paris Cité (USPC)-Institut National de la Santé et de la Recherche Médicale (INSERM), Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Hôpital Européen Georges Pompidou [APHP] (HEGP), Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Hôpitaux Universitaires Paris Ouest - Hôpitaux Universitaires Île de France Ouest (HUPO)-Hôpitaux Universitaires Paris Ouest - Hôpitaux Universitaires Île de France Ouest (HUPO), Service de Médecine Interne [CHU Clermont-Ferrand], CHU Gabriel Montpied [Clermont-Ferrand], CHU Clermont-Ferrand-CHU Clermont-Ferrand, Hôpital Sainte-Blandine-Centre hospitalier régional Metz-Thionville (CHR Metz-Thionville), Institut de Génétique et Développement de Rennes (IGDR), Structure Fédérative de Recherche en Biologie et Santé de Rennes ( Biosit : Biologie - Santé - Innovation Technologique )-Centre National de la Recherche Scientifique (CNRS)-Université de Rennes 1 (UR1), Université de Rennes (UNIV-RENNES)-Université de Rennes (UNIV-RENNES), Hospices Civils de Lyon (HCL)-Hospices Civils de Lyon (HCL), Département de Médecine Interne (VALENCIENNES - Med Int), Centre Hospitalier Régional Universitaire [Lille] (CHRU Lille), Université de Nantes (UN)-Hôtel-Dieu-Centre hospitalier universitaire de Nantes (CHU Nantes), Université Paris Diderot - Paris 7 (UPD7)-Institut National de la Santé et de la Recherche Médicale (INSERM), Lipides - Nutrition - Cancer (U866) (LNC), Université de Bourgogne (UB)-Institut National de la Santé et de la Recherche Médicale (INSERM)-AgroSup Dijon - Institut National Supérieur des Sciences Agronomiques, de l'Alimentation et de l'Environnement-Ecole Nationale Supérieure de Biologie Appliquée à la Nutrition et à l'Alimentation de Dijon (ENSBANA), Université Paris Diderot - Paris 7 (UPD7)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP), CHU Cochin [AP-HP]-Assistance publique - Hôpitaux de Paris (AP-HP) (APHP), Université Pierre et Marie Curie - Paris 6 (UPMC)-CHU Pitié-Salpêtrière [APHP], Assistance publique - Hôpitaux de Paris (AP-HP) (APHP)-Hôpital Européen Georges Pompidou [APHP] (HEGP), Hôpitaux Universitaires Paris Ouest - Hôpitaux Universitaires Île de France Ouest (HUPO)-Assistance publique - Hôpitaux de Paris (AP-HP) (APHP)-Hôpitaux Universitaires Paris Ouest - Hôpitaux Universitaires Île de France Ouest (HUPO), Université de Rennes 1 (UR1), Université de Rennes (UNIV-RENNES)-Université de Rennes (UNIV-RENNES)-Centre National de la Recherche Scientifique (CNRS)-Structure Fédérative de Recherche en Biologie et Santé de Rennes ( Biosit : Biologie - Santé - Innovation Technologique ), Assistance publique - Hôpitaux de Paris (AP-HP) (APHP)-Université Paris Diderot - Paris 7 (UPD7), Jonchère, Laurent, Centre hospitalier régional Metz-Thionville (CHR Metz-Thionville)-Hôpital Sainte-Blandine, Université Paris Diderot - Paris 7 (UPD7)-Assistance publique - Hôpitaux de Paris (AP-HP) (APHP), Assistance publique - Hôpitaux de Paris (AP-HP) (APHP)-CHU Cochin [AP-HP], Université Paris Diderot - Paris 7 (UPD7)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Institut National de la Recherche Agronomique (INRA)-Université Paris Descartes - Paris 5 (UPD5)-Université Sorbonne Paris Cité (USPC)-Université Paris Diderot - Paris 7 (UPD7)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Institut National de la Recherche Agronomique (INRA)-Université Sorbonne Paris Cité (USPC), Université Paris Diderot - Paris 7 (UPD7)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Institut National de la Recherche Agronomique (INRA)-Université Paris Descartes - Paris 5 (UPD5)-Université Sorbonne Paris Cité (USPC), Assistance publique - Hôpitaux de Paris (AP-HP) (APHP)-Hôpitaux Universitaires Paris Ouest - Hôpitaux Universitaires Île de France Ouest (HUPO)-Hôpitaux Universitaires Paris Ouest - Hôpitaux Universitaires Île de France Ouest (HUPO), Service Médecine Interne et immunologie clinique [CHU Toulouse], Pôle Maladies de l'appareil digestif [CHU Toulouse], Centre Hospitalier Universitaire de Toulouse (CHU Toulouse)-Centre Hospitalier Universitaire de Toulouse (CHU Toulouse), Service Médecine interne et immunopathologie clinique [CHU Toulouse], Pôle IUCT [CHU Toulouse], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU), Université de Rennes (UR)-Centre National de la Recherche Scientifique (CNRS)-Structure Fédérative de Recherche en Biologie et Santé de Rennes ( Biosit : Biologie - Santé - Innovation Technologique ), and Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Université Paris Diderot - Paris 7 (UPD7)
Subjects: Adult, Male, [ SDV ] Life Sciences [q-bio], [SDV]Life Sciences [q-bio], Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis, Middle Aged, [SDV] Life Sciences [q-bio], Azathioprine, Quality of Life, Humans, Disabled Persons, Female, Rituximab, Aged
Abstract: IF 2.724 (2014); International audience; OBJECTIVES: To investigate the effects on health-related quality of life (HRQOL) and functional capability of rituximab vs azathioprine for ANCA-associated vasculitis (AAV) maintenance therapy. METHODS: In a 24-month phase III randomised-controlled trial, 115 patients over time received rituximab or azathioprine for AAV maintenance therapy. Mean changes of 36-item Short-form Health Survey (SF-36) and Health Assessment Questionnaire (HAQ) scores from baseline were analysed. RESULTS: Mean improvements of HAQ scores, from baseline to month 24 were significantly better for the rituximab (0.16 points lower) than the azathioprine group (p=0.038). As demonstrated by SF-36, study patients' baseline HRQOL was significantly impaired compared with age- and sex-matched US norms. At month 24, mean changes from baseline of SF-36 physical component score tended to be better for the rituximab group (+3.95 points, p=0.067) whereas mean changes from baseline of the SF-36 mental component score were significantly better for the azathioprine group (+4.23 points, p=0.041). CONCLUSIONS: Azathioprine-treated patients' for AAV maintenance therapy showed a decline in physical abilities when compared to RTX at M24 in the MAINRITSAN trial. TRIAL REGISTRATION: ClinicalTrials.gov, http://clinicaltrials.gov/, NCT00748644

38. Top-down infliximab plus azathioprine versus azathioprine alone in patients with acute severe ulcerative colitis responsive to intravenous steroids: a parallel, open-label randomised controlled trial, the ACTIVE trial.

Author: Amiot A, Seksik P, Meyer A, Stefanescu C, Wils P, Altwegg R, Vuitton L, Plastaras L, Nicolau A, Pereira B, Duveau N, Laharie D, Mboup B, Boualit M, Allez M, Rajca S, Chanteloup E, Bouguen G, Bazin T, Goutorbe F, Richard N, Moussata D, Vicaut E, and Peyrin-Biroulet L
Abstract: Background: It is unknown which maintenance therapy is the most effective option for patients admitted for an acute severe ulcerative colitis (ASUC) episode responding to intravenous steroids., Methods: We conducted a multicentre, parallel-group, open-label randomised controlled trial among 23 French centres in thiopurine and biologics-naïve adults admitted for ASUC responding to intravenous steroids. Eligible patients were randomly assigned to receive infliximab (IFX) and azathioprine (AZA) with a 7-day steroid tapering scheme (IFX+AZA arm) or AZA and conventional standardised steroid tapering regimen (AZA arm). The primary composite endpoint was treatment failure at week 52, defined as the absence of steroid-free clinical remission, the absence of endoscopic response, the use of a prohibited treatment for relapse, severe adverse event leading to treatment interruption, colectomy or death. Multiple imputation for missing data was performed., Findings: Among the 64 patients randomised (Lichtiger score 13.5±2.0; median age of 34.5 (P25-P75 26.3-50.3) years, median C reactive protein of 29.0 (12.8-96.8) mg/L at baseline): 32 were assigned to the IFX+AZA arm and 32 to the AZA arm. In the ITT population, treatment failure at week 52 was observed in 22/27 (81.5%) in the AZA arm and 16/30 (53.3%) in the IFX+AZA arm (risk ratio 3.85, 95% CI (1.15 to 12.88), p=0.03). 29 adverse events were severe, including 13 disease exacerbations, 6 severe infections without any difference between both arms., Interpretation: Combination therapy with IFX+AZA was more effective at 1 year than AZA alone to avoid treatment failure in patients with ASUC responding to intravenous steroids., Trial Registration Number: NCT02425852., Competing Interests: Competing interests: AA received consulting fees from Abbvie, Pfizer, Takeda, Tillotts Pharma, Janssen and Sandoz as well as lecture fees and travel accommodations from Abbvie, Janssen, Pfizer, Takeda, Biogen, Fresenius Kabi, Amgen and Celltrion. PS reports consulting fees from Pfizer, Astellas, Janssen, Fresenius Kabi, Takeda, Abbvie, Merck-MSD, Pilège, Lilly, Celltrion and Biocodex; and grants from Biocodex and Janssen. CS declare lecture and/or consulting fees from Abbvie, Amgen, Celltrion, Janssen, Lilly, Takeda, Tillots. PW received board membership, consultancy, or lecture fees from Abbvie, Amgen, Celltrion, Ferring, Janssen, and Takeda. LV received fees from Abbvie, Amgen, MSD, Ferring, Takeda, Pfizer, Celltrion, Janssen, Galapagos, Dr Falk. RA declares lecture fees from MSD, Abbvie, Pfizer, Takeda, and Janssen. GB received lecture fees from Abbvie, Ferring, Takeda and Pfizer and consultant fees from Takeda, Janssen, Lilly, Celltrion, Sandoz, Abbvie. MB has received lecture fees and travel accommodation from Abbvie, Fresenius Kabi, Janssen, Pfizer and Takeda. DL has received counseling, boards, transports and/or fees from Abbvie, Amgen, Biogen, Ferring, Galapagos, Janssen, Lilly, MSD, Novartis, Pfizer, Prometheus, Roche, Takeda, Tillots. MA has served as a speaker, consultant, and/or advisory board member for Abbvie, Amgen, Biogen, Boehringer-Ingelheim, Celgene, Celltrion, Ferring, Galapagos, Genentech, IQVIA, Janssen, Lilly, MSD, Pfizer, Roche, Takeda, Tillotts. FG declares counseling, boards, transports and lectures fees from Abbvie, Amgen, Biogen, Celltrion, Janssen, Lilly, MSD, Pfizer, Takeda. NR has received lecture fees from AbbVie and Takeda. LP-B received consulting fees from Merck, Abbvie, Janssen, Genentech, Ferring, Norgine, Tillots, Vifor, Shire, Therakos, Pharmacosmos, Pilège, BMS, UCB-Pharma, Hospira, Celltrion, Takeda, Biogaran, Boerhinger-Ingelheim, Lilly, Pfizer and HAC-Pharma. This author also received lecture fees from Merck, Abbvie, Takeda, Janssen Cilag, Ferring, Norgine, Tillots, Vifor, Therakos, HAC-Pharma and Mitsubishi. No conflicts of interest are claimed by the remaining authors., (© Author(s) (or their employer(s)) 2024. No commercial re-use. See rights and permissions. Published by BMJ.)
Published: 2024
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39. Integrating automated dispensing cabinets into the medication dispensing process: feedback from the practice in European hospitals.

Author: Francois O, Hufschmid Thurnherr E, Blatrie C, Cousein E, Herranz A, Sadeghipour F, and Bonnabry P
Abstract: Objectives: Automated dispensing cabinets (ADCs) offer improved medication safety, greater efficiency and return on investment. However, integrating ADCs into medication dispensing processes can be challenging in complex hospital environments. This study aimed to draft suggestions to help hospitals adopt ADCs., Methods: Two-day visits were organised in seven European hospitals operating ADCs. Investigators used an observational grid, a questionnaire and interviews, each divided into the themes of medication processes before and after the introduction of ADCs, the major steps followed and the resources involved, ergonomics and staff perceptions., Results: ADCs were integrated into four global hospital medication dispensing systems (packs of drugs are distributed from the central pharmacy to wards for dispensing) and three nominative systems-that is, patient-specific ones (drug doses prescribed for individuals are distributed from the central pharmacy to wards with ADC as supplementary stock). A general ADC project implementation timeline was shaped: main drivers of automation to initiate the project, visit of other sites, pilot test (with IT integration and staff training), and evaluation phase (satisfaction, safety, efficiency) to justify a possible expansion. Users (7 pharmacists, 21 nurses, 7 data engineers) identified facilitators (such as a dedicated project manager, a pilot phase, an intuitive device), barriers and any improvements needed (training for incoming staff, reorganisation of ward workflow, dynamic inventories)., Conclusions: Despite their diverse pharmacy organisations, each hospital raised similar challenges and reported analogous major steps in project implementation. Although integration processes are complex, ADCs rapidly provide users with benefits. By following the practical advice and recommendations from these hospitals, new adopters might reduce the risks of failed ADC projects and accelerate their integration., Competing Interests: Competing interests: None declared., (© European Association of Hospital Pharmacists 2024. No commercial re-use. See rights and permissions. Published by BMJ.)
Published: 2024
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40. Unravelling a KMT2A::ARHGEF12 fusion within chromoanagenesis in acute myeloid leukemia using Optical Genome Mapping.

Author: Klos C, Roynard P, Berthon C, Soenen V, Marceau A, Fournier E, Fenwarth L, Daudignon A, Roche C, and Guermouche H
Published: 2024
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41. Outpatient balloon catheter versus expectant management for post-term labor induction in nulliparous women: A randomized trial.

Author: Garabedian C, Tillouche N, Drumez E, Labreuche J, Dreyfus M, and Deruelle P
Subjects: Humans, Female, Pregnancy, Adult, Ambulatory Care methods, Labor, Induced methods, Parity, Cervical Ripening, Watchful Waiting methods, Pregnancy, Prolonged therapy
Abstract: Background: Increased use of labor induction has renewed interest in outpatient cervical ripening. Post-term pregnancy (i.e., ≥41 weeks) is a specific situation of increased neonatal risk, including greater risk of perinatal death and adverse perinatal outcomes. While a high proportion of these patients will need induction, outpatient management of this specific population has never been studied. Therefore, our objective was to compare two policies of management of post term pregnancies: the use of a transcervical Foley catheter for outpatient cervical ripening compared with expectant management., Methods: Multicenter, randomized controlled open-label study comparing home induction with a Foley catheter versus expectant management. Inclusion criteria were nulliparous, live singleton fetus in a vertex position, post-term (at 41 + 4 days), requiring cervical ripening (Bishop score <6), intact membranes, and distance home-hospital within 40 min. The primary endpoint was change in Bishop score beetween randomization (41 + 4 days) and consultation (41 + 5 days)., Results: Forty-five women were included: 21 in the home induction group and 24 in the control group. The study was stopped due to low recruitment. The difference in Bishop score increases one day after randomization approached significance (p = 0.055), with home induction showing a larger change compared with expectant management (Cohen's d = 0.60; 95 % confidence interval [CI] -0.002 to 1.21). Regarding change in Bishop score, 81 % of home induction group patients had a better score at 41 + 5 days versus 52.2 % in the control group (relative risk = 1.55; 95 %CI 0.99 to 2.15)., Conclusion: By specifically evaluating home induction in nulliparous women with post term pregnancies, we observed a Bishop score improvement in the home induction group. These data support further evaluation of induction methods and birth experiences in a larger cohort of this population., Trial Registration: The study was registered under European policy (number EudraCT 2015-A01298-41) and on www.clinitrials.gov (number NCT02932319). Date of registration: 13/10/2016, Date of initial participant enrollment: 31/03/2017., (Copyright © 2024 The Author(s). Published by Elsevier Masson SAS.. All rights reserved.)
Published: 2024
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42. Assessment of chronical total occlusions management in France: The ENCOCHE Registry, a prospective, multicentric study.

Author: Cornillet L, Lefèvre T, Lemoine J, Zuffi A, Avran A, Gervasoni R, La Scala E, Teiger E, Godin M, Staat P, Mangin L, Philippart R, Blanchart K, Hovasse T, Brunel P, Bressollette E, Letocart V, Bataille V, and Boudou N
Abstract: Background: Coronary chronic total occlusions (CTO) are frequent, and coronary angioplasty has been increasingly used in recent years for lesion revascularisation. However, to date, no dedicated multicentric prospective study is available in France., Aim: To describe the characteristics of CTO patients and to assess current treatment strategies in French catheterisation laboratory practice., Methods: Patients presenting with CTOs were included from 16/09/2021 to 13/12/2021 over two consecutive prospective phases. In phase I (one month), data were collected to include all patients presenting CTO at diagnostic angiography. In phase II (two months), data were collected focusing on patients who underwent CTO-PCI., Results: A total of 1303 patients (1460 CTOs) were included in 68 French centres. The mean age was 67.7±10.7 years and 84.3% of the patients were men. The prevalence of prior PCI (44.6%), and diabetes mellitus (35.6%) was high. In phase I, multivessel coronary artery disease was detected in two-thirds of cases, and most of them (88.5%) had a single CTO. The mean J-CTO score was 1.9±1.2, with a proportion of difficult and very difficult CTO (J CTO score ≥2) of 61.1%. The selected treatment was medical therapy in 57% of cases, coronary angioplasty in 30% and bypass surgery in 13%. In phase II, 528 patients were included with a mean J-CTO score of 1.8±1.2. Successful guidewire crossing through CTO lesion was obtained with an antegrade access in 89% of patients. Procedural success rate of CTO-PCI was 80%, with a rate of major in-hospital complications of 1% (death: 0.4%, MI: 0.2%, stroke: 0.2%, emergency CABG: 0.2%)., Conclusion: This prospective study provides a snapshot of CTOs prevalence and CTO treatment strategies in France in 2021., (Copyright © 2024 Elsevier Masson SAS. All rights reserved.)
Published: 2024
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43. Optimization of Automated Radiosynthesis of Gallium-68-Labeled PSMA11 with Two [ 68 Ge]Ge/[ 68 Ga]Ga Generators: Fractional Elution or Prepurification?

Author: Durieux F, Dekyndt B, Legrand JF, Rogeau A, Malek E, Semah F, and Odou P
Abstract: Prostate cancer is one of the most common forms of cancer in men. An imaging technique for its diagnosis is [ 68 Ga]-prostate-specific membrane antigen ([ 68 Ga]Ga-PSMA-11) positron emission tomography (PET). To address the increasing demand for [ 68 Ga]-labeled peptides and reduce the cost of radiosynthesis, it is therefore necessary to optimize the elution process of [ 68 Ge]Ge/[ 68 Ga]Ga generators. This study aims to identify the most effective approach for optimizing radiosynthesis using double elution in parallel of two [ 68 Ge]Ge/[ 68 Ga]Ga generators. Two methods have been tested: one using prepurification, and the other using fractionated elution. Five synthesis sequences were conducted using each method. The mean labeling yields for double elution with prepurification were 45.8 ± 29.4 (mean ± standard deviation) and none met the required criteria. The mean labeling yields for the fractionated double elution were 97.5 ± 1.9 (mean ± standard deviation) meeting the criteria, significantly superior to the prepurification method ( p = 0.012), and similar to those of simple elution. This study showed that fractionated double elution from [ 68 Ge]Ge/[ 68 Ga]Ga generators produced a significantly higher labeling yield than double elution with prepurification, resulting in a larger activity recovered via radiosynthesis, thereby allowing more diagnostic tests to be performed.
Published: 2023
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44. Clinical and Prognostic Factors in Patients with IgG4-Related Kidney Disease.

Author: Chaba A, Devresse A, Audard V, Boffa JJ, Karras A, Cartery C, Deltombe C, Chemouny J, Contamin C, Courivaud C, Duquennoy S, Garcia H, Joly D, Goumri N, Hanouna G, Halimi JM, Plaisier E, Hamidou M, Landron C, Launay D, Lebas C, Legendre M, Masseau A, Mathian A, Mercadal L, Morel N, Mutinelli-Szymanski P, Palat S, Pennaforte JL, Peraldi MN, Pozdzik A, Schleinitz N, Thaunat O, Titeca-Beauport D, Mussini C, Touati S, Prinz E, Faller AL, Richter S, Vilaine E, Ferlicot S, Von-Kotze C, Belliere J, Olagne J, Mesbah R, Snanoudj R, Nouvier M, Ebbo M, and Zaidan M
Subjects: Adult, Middle Aged, Humans, Male, Aged, Female, Rituximab adverse effects, Cohort Studies, Prognosis, Kidney pathology, Immunoglobulin G, Recurrence, Retrospective Studies, Immunoglobulin G4-Related Disease complications, Immunoglobulin G4-Related Disease diagnosis, Immunoglobulin G4-Related Disease drug therapy, Nephritis, Interstitial pathology
Abstract: Background: IgG4-related kidney disease is a major manifestation of IgG4-related disease, a systemic fibroinflammatory disorder. However, the clinical and prognostic kidney-related factors in patients with IgG4-related kidney disease are insufficiently defined., Methods: We conducted an observational cohort study using data from 35 sites in two European countries. Clinical, biologic, imaging, and histopathologic data; treatment modalities; and outcomes were collected from medical records. Logistic regression was performed to identify the possible factors related to an eGFR ≤30 ml/min per 1.73 m 2 at the last follow-up. Cox proportional hazards model was performed to assess the factors associated with the risk of relapse., Results: We studied 101 adult patients with IgG4-related disease with a median follow-up of 24 (11-58) months. Of these, 87 (86%) patients were male, and the median age was 68 (57-76) years. Eighty-three (82%) patients had IgG4-related kidney disease confirmed by kidney biopsy, with all biopsies showing tubulointerstitial involvement and 16 showing glomerular lesions. Ninety (89%) patients were treated with corticosteroids, and 18 (18%) patients received rituximab as first-line therapy. At the last follow-up, the eGFR was below 30 ml/min per 1.73 m 2 in 32% of patients; 34 (34%) patients experienced a relapse, while 12 (13%) patients had died. By Cox survival analysis, the number of organs involved (hazard ratio [HR], 1.26; 95% confidence interval [CI], 1.01 to 1.55) and low C3 and C4 concentrations (HR, 2.31; 95% CI, 1.10 to 4.85) were independently associated with a higher risk of relapse, whereas first-line therapy with rituximab was protective (HR, 0.22; 95% CI, 0.06 to 0.78). At their last follow-up, 19 (19%) patients had an eGFR ≤30 ml/min per 1.73 m 2 . Age (odd ratio [OR], 1.11; 95% CI, 1.03 to 1.20), peak serum creatinine (OR, 2.74; 95% CI, 1.71 to 5.47), and serum IgG4 level ≥5 g/L (OR, 4.46; 95% CI, 1.23 to 19.40) were independently predictive for severe CKD., Conclusions: IgG4-related kidney disease predominantly affected middle-aged men and manifested as tubulointerstitial nephritis with potential glomerular involvement. Complement consumption and the number of organs involved were associated with a higher relapse rate, whereas first-line therapy with rituximab was associated with lower relapse rate. Patients with high serum IgG4 concentrations (≥5 g/L) had more severe kidney disease., (Copyright © 2023 by the American Society of Nephrology.)
Published: 2023
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45. Correction: Role, Cost, and Availably of Urinary pH Monitoring for Kidney Stone Disease-A Systematic Review of the Literature.

Author: Sanz-Gómez I, Angerri O, Baboudjian M, Kanashiro A, Gracia S, Millán F, Sánchez-Martín F, Somani B, Galan-Llopis JA, Barghouthy Y, and Emiliani E
Published: 2023
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46. Role, Cost, and Availably of Urinary pH Monitoring for Kidney Stone Disease-A Systematic Review of the Literature.

Author: Sanz-Gómez I, Angerri O, Baboudjian M, Kanashiro A, Gracia S, Millán F, Sánchez-Martín F, Somani B, Galan-Llopis JA, Barghouthy Y, and Emiliani E
Subjects: Humans, Hydrogen-Ion Concentration, Forecasting, Kidney Calculi diagnosis, Urolithiasis, Urinary Tract
Abstract: Purpose of Review: Urinary pH is an important factor related to renal stone disease, and it plays an essential role in stone prevention. Monitoring of urinary pH by patients at home provides information that can help to assess the treatment needed by each patient. We conducted a systematic review is to assess the available evidence concerning urinary pH monitoring methods along with their accuracy, cost, and usefulness by patients with urolithiasis., Recent Findings: A total of 9 articles were included (1886 urinary pH measurements). They reported information about urinary dipsticks, portable electronic pH meters and electronic strip readers, amongst other methods. Accuracy was compared with a laboratory pH meter (gold standard). Urinary dipsticks were found to be not accurate enough to guide clinical decision making and portable electronic pH meters showed promising results. Urinary dipsticks are neither precise nor accurate enough. Portable electronic pH meters seem to be more accurate, easy to use, and cost-effective. They are a reliable source for patients to use at home in order to prevent future episodes of nephrolithiasis., (© 2023. The Author(s), under exclusive licence to Springer Science+Business Media, LLC, part of Springer Nature.)
Published: 2023
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47. A randomised trial of anti-GM-CSF otilimab in severe COVID-19 pneumonia (OSCAR).

Author: Patel J, Bass D, Beishuizen A, Bocca Ruiz X, Boughanmi H, Cahn A, Colombo H, Criner GJ, Davy K, de-Miguel-Díez J, Doreski PA, Fernandes S, François B, Gupta A, Hanrott K, Hatlen T, Inman D, Isaacs JD, Jarvis E, Kostina N, Kropotina T, Lacherade JC, Lakshminarayanan D, Martinez-Ayala P, McEvoy C, Meziani F, Monchi M, Mukherjee S, Muñoz-Bermúdez R, Neisen J, O'Shea C, Plantefeve G, Schifano L, Schwab LE, Shahid Z, Shirano M, Smith JE, Sprinz E, Summers C, Terzi N, Tidswell MA, Trefilova Y, Williamson R, Wyncoll D, and Layton M
Subjects: Adult, Humans, Granulocyte-Macrophage Colony-Stimulating Factor, Antibodies, Monoclonal, Humanized, Double-Blind Method, Treatment Outcome, COVID-19, Respiratory Insufficiency
Abstract: Background: Granulocyte-macrophage colony-stimulating factor (GM-CSF) and dysregulated myeloid cell responses are implicated in the pathophysiology and severity of COVID-19., Methods: In this randomised, sequential, multicentre, placebo-controlled, double-blind study, adults aged 18-79 years (Part 1) or ≥70 years (Part 2) with severe COVID-19, respiratory failure and systemic inflammation (elevated C-reactive protein/ferritin) received a single intravenous infusion of otilimab 90 mg (human anti-GM-CSF monoclonal antibody) plus standard care (NCT04376684). The primary outcome was the proportion of patients alive and free of respiratory failure at Day 28., Results: In Part 1 (n=806 randomised 1:1 otilimab:placebo), 71% of otilimab-treated patients were alive and free of respiratory failure at Day 28 versus 67% who received placebo; the model-adjusted difference of 5.3% was not statistically significant (95% CI -0.8-11.4%, p=0.09). A nominally significant model-adjusted difference of 19.1% (95% CI 5.2-33.1%, p=0.009) was observed in the predefined 70-79 years subgroup, but this was not confirmed in Part 2 (n=350 randomised) where the model-adjusted difference was 0.9% (95% CI -9.3-11.2%, p=0.86). Compared with placebo, otilimab resulted in lower serum concentrations of key inflammatory markers, including the putative pharmacodynamic biomarker CC chemokine ligand 17, indicative of GM-CSF pathway blockade. Adverse events were comparable between groups and consistent with severe COVID-19., Conclusions: There was no significant difference in the proportion of patients alive and free of respiratory failure at Day 28. However, despite the lack of clinical benefit, a reduction in inflammatory markers was observed with otilimab, in addition to an acceptable safety profile., Competing Interests: Conflict of interest: J. Patel, D. Bass, A. Cahn, K. Davy, S. Fernandes, A. Gupta, K. Hanrott, D. Inman, E. Jarvis, D. Lakshminarayanan, S. Mukherjee, C. O'Shea, L. Schifano, J.E. Smith, R. Williamson and M. Layton are shareholders and/or employees of GSK. A. Beishuizen, X. Bocca Ruiz, H. Boughanmi, H. Colombo, G.J. Criner, J. de-Miguel-Díez, P.A. Doreski, B. François, T. Hatlen, J.D. Isaacs, N. Kostina, T. Kropotina, J-C. Lacherade, P. Martinez-Ayala, C. McEvoy, F. Meziani, M. Monchi, R. Muñoz-Bermúdez, G. Plantefeve, L.E. Schwab, Z. Shahid, M. Shirano, E. Sprinz, C. Summers, N. Terzi and Y. Trefilova were investigators in the OSCAR trial, which was funded by GSK. X. Bocca Ruiz has served as a clinical trial investigator for AstraZeneca and Zambon. B. François reports consultancy fees with GSK, Enlivex, Inotrem, Takeda, Aridis, Transgene, AM-Pharma, Asahi-Kasai and Biomérieux within the past 36 months. R. Muñoz-Bermúdez has participated on an advisory board for GSK. J.D. Isaacs has received research funding from GSK, Janssen and Pfizer, and personal fees from AbbVie, BMS, Gilead, Roche and UCB, all outside the submitted work, as well as support for event attendance from Eli Lilly and Gilead. A. Beishuizen has received consultancy fees from GSK. C. McEvoy has received research funding from the National Institutes of Health, US Department of Defense, Patient-Centered Outcomes Research Institute, GSK and AstraZeneca. C. Summers’ institution has received research funding from GSK, AstraZeneca, the Wellcome Trust, The Medical Research Council and National Institute for Health Research to support her work outside the area of the submitted manuscript. C. Summers has received personal fees from AbbVie, Roche and GSK. G.J. Criner has received research grants from ALung Technologies Inc., American College of Radiology, American Lung Associations, AstraZeneca, BioScale Inc., Boehringer Ingelheim, BREATH Therapeutics Inc., COPD Foundation, Coridea/ZIDAN, Corvus, Dr Karen Burns of St Michael's Hospital, Fisher & Paykel Healthcare Ltd, Galapagos NV, GSK, Kinevent, Lungpacer Medical Inc., National Heart Lung and Blood Institute, Nurvaira Inc., Patient-Centered Outcomes Research Institute, Pulmonary Fibrosis Foundation, PulmonX, Respironics Inc., Respivant Sciences, Spiration Inc., Steward St Elizabeth's Medical Center of Boston Inc. and Veracyte Inc.; and received personal fees from Amgen, AstraZeneca, Boehringer Ingelheim, Broncus Medical, CSA Medical, EOLO Medical, Gala Therapeutics, GSK, Helios Medical, Ion, Merck, Medtronic, Mereo BioPharma, NGM Biopharmaceuticals, Novartis, Olympus, PulmonX, Respironics Inc., Respivant Sciences, The Implementation Group and Verona Pharma. J. Neisen is an employee and shareholder of AstraZeneca, and a shareholder and former employee of GSK. L.E. Schwab reports holding shares in Johnson & Johnson and BMS, and participated on the Holy Cross Health Institutional Review Board. Z. Shahid has received research funding from Karyopharm. M. Shirano is an investigator in separate trials funded by Roche and AstraZeneca, and has received payment for lectures from Gilead Sciences. E. Sprinz participates on a data safety monitoring board/advisory board for, and has received consulting fees and honoraria from, GSK, Janssen and Gilead. M.A. Tidswell received a fee for serving on the independent data monitoring committee for this study, as well as for serving on a data safety monitoring board/advisory board for Spectral Diagnostics Inc., ReAlta Life Sciences Inc., Celltrion Inc., AstraZeneca and Molecular Partners AG. Additionally, M.A. Tidswell has held a research contract with Edesa Biotech Research Inc., RevImmune SAS, Spectral Diagnostics, Beyond Air Inc., National Institutes of Health and National Heart, Lung, and Blood Institute. D. Wyncoll received a fee for serving on the independent data monitoring committee for this study, has served as a study adjudicator for AstraZeneca, and reports consulting fees and/or honoraria from Gilead and Shionogi. J. de-Miguel-Díez, H. Boughanmi, J-C. Lacherade, P. Martinez-Ayala, T. Hatlen, G. Plantefeve, N. Terzi, M. Shirano, N. Kostina, T. Kropotina, Y. Trefilova and M. Monchi have no other conflicts of interest to declare., (Copyright ©The authors 2023.)
Published: 2023
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48. The Versatile Role of miR-21 in Renal Homeostasis and Diseases.

Author: Larrue R, Fellah S, Van der Hauwaert C, Hennino MF, Perrais M, Lionet A, Glowacki F, Pottier N, and Cauffiez C
Subjects: Humans, Fibrosis, Kidney pathology, Homeostasis, MicroRNAs metabolism, Renal Insufficiency, Chronic pathology
Abstract: MicroRNAs (miRNAs) are small, non-coding RNA species that control gene expression and confer robustness to biological processes. Over the last two decades, their important roles during kidney development, homeostasis and the treatment of diseases have been established, in particular during the onset and progression of various forms of acute and chronic renal disorders. In recent years, miR-21, one of the best-characterized miRNAs to date, has received much attention in renal physiology in particular given its high degree of conservation and expression in kidneys, as well as its potent pathogenic role in various debilitating renal diseases. This review summarizes the current knowledge on miR-21's involvement in both renal homeostasis and diseases, in particular its double-edged-sword role in acute versus chronic kidney injuries. Finally, we also discuss the potential of miR-21 as a biomarker and therapeutic target in renal diseases.
Published: 2022
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49. Localized versus systemic granulomatosis with polyangiitis: data from the French Vasculitis Study Group Registry.

Author: Iudici M, Pagnoux C, Courvoisier DS, Cohen P, Néel A, Aouba A, Lifermann F, Ruivard M, Aumaître O, Bonnotte B, Maurier F, Le Gallou T, Hachulla E, Karras A, Khouatra C, Jourde-Chiche N, Viallard JF, Blanchard-Delaunay C, Godmer P, Le Quellec A, Quéméneur T, de Moreuil C, Régent A, Terrier B, Mouthon L, Guillevin L, and Puéchal X
Subjects: Antibodies, Antineutrophil Cytoplasmic, Humans, Recurrence, Registries, Retrospective Studies, Granulomatosis with Polyangiitis diagnosis
Abstract: Objective: To describe the main features at diagnosis and evolution over time of patients with localized granulomatosis with polyangiitis (L-GPA) compared with those of systemic GPA (S-GPA)., Methods: EULAR definitions of L-GPA, i.e. upper and/or lower respiratory tract involvement, and S-GPA were applied to patients from the French Vasculitis Study Group Registry. L-GPA and S-GPA patients' characteristics at diagnosis and long-term outcomes were analysed and compared., Results: Among the 795 Registry patients, 79 (10%) had L-GPA. Their main clinical manifestations were rhinitis, lung nodules, sinusitis and otitis. L-GPA vs S-GPA patients at diagnosis, respectively, were younger, more frequently had saddle nose deformity or subglottic stenosis and were less often PR3-ANCA-positive. L-GPA vs S-GPA induction therapy less frequently included CYC but more often a combination of MTX and glucocorticoids; 64% of MTX-treated patients experienced disease progression within 18 months post-diagnosis. L- and S-GPA patients' estimated relapse-free-survival probabilities, relapse rates and refractory disease rates at each time point were comparable, but L-GPA patients had more frequent ENT and lung relapses, and higher overall survival rates (P<0.02). Over a median follow-up of 3.5 years, 18 (22.8%) L-GPA progressed to S-GPA, either as a relapse after a period in remission or more frequently in the context of refractory disease. L-GPA patients experienced more ENT-related damage., Conclusions: The relapse risks of L-GPA and S-GPA were similar, but relapse patterns differed and L-GPA overall survival rate was higher. About one-quarter of L-GPA patients developed S-GPA over time, but without end-stage organ involvement., (© The Author(s) 2021. Published by Oxford University Press on behalf of the British Society for Rheumatology. All rights reserved. For permissions, please email: journals.permissions@oup.com.)
Published: 2022
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50. Classification of pedicle ossification after maxillofacial reconstruction with bony free flap: An observational study.

Author: Barry F, Schlund M, Raoul G, Myon L, Ferri J, and Nicot R
Subjects: Humans, Osteogenesis, Postoperative Complications diagnosis, Postoperative Complications etiology, Postoperative Complications surgery, Free Tissue Flaps adverse effects, Free Tissue Flaps surgery, Ossification, Heterotopic diagnosis, Ossification, Heterotopic etiology, Ossification, Heterotopic surgery, Plastic Surgery Procedures adverse effects, Plastic Surgery Procedures methods
Abstract: Introduction: Maxillofacial reconstruction with bony free flap is a classical technique. However, pedicle ossification after such reconstruction is a misunderstood complication that is rarely reported in the literature. It is usually manifested as trismus, neck pain, and hard swelling, but it is predominantly asymptomatic and, thus, mainly incidentally discovered at a later stage. The aim of our study is to propose a classification for pedicle ossification based on both radiological features and vascular calcification progression. We also describe a case of metachronous ossification after two fibula free flap procedures., Material & Methods: Our observational study includes all patients from our unit who underwent maxillofacial reconstruction with bony free flap from 2003 to 2018. We collected all cases of pedicle ossifications identified during the follow-up and described the radiological status of each one to categorise them in different groups and propose a classification scheme for the same., Results: Radiological and histological analysis showed a progressive three-step evolution of pedicle ossification, starting from the media, progressing into the lumen, and then reaching completion in the extravascular region. The final stage was observed in all symptomatic patients., Conclusion: Pedicle ossification is a progressive process that passes through three successive histological stages that may be associated with factors such as smooth muscle cell phenotype modification [1]. This complication may lead to more severe clinical symptoms and may require surgery for removal of the calcification., (Copyright © 2021 Elsevier Masson SAS. All rights reserved.)
Published: 2022
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