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2,454 results on '"Hôpital des Enfants"'

1. Cas grave d’eczéma et d’asthme chez le nourrisson

Author: Académie interuniversitaire de Pneumologie pédiatrique (18 septembre 1999: Hôpital des Enfants), Casimir, Georges, Académie interuniversitaire de Pneumologie pédiatrique (18 septembre 1999: Hôpital des Enfants), and Casimir, Georges
Abstract: Séance d’enseignement à l’Hôpital des Enfants sur le thème de cas cliniques interactifs (82 inscrits), info:eu-repo/semantics/nonPublished
Published: 1999

2. Estat présent de l'Hospital des enfans trouvez

Author: Hôpital des enfants trouvés (Paris) and Hôpital des enfants trouvés (Paris)
Abstract: https://patrimoniodigital.ucm.es/r/thumbnail/743198, https://patrimoniodigital.ucm.es/r/item/5323862983

3. Use of the surgical glue in the cutaneous closure of cheiloplasties for cleft lip

Author: A Ramon, C. François, L Pouzet, L. Jayyosi, Marie-Laurence Poli-Merol, Chirurgie Pédiatrique [Reims] (CHU de Reims - American Memorial Hospital (Hôpital des enfants)), Centre Hospitalier Universitaire de Reims (CHU Reims)-American Memorial Hospital (Hôpital des enfants) [Reims], Hémostase et Remodelage Vasculaire Post-Ischémie (HERVI - EA 3801), and Université de Reims Champagne-Ardenne (URCA)
Subjects: [SDV.MHEP.PED]Life Sciences [q-bio]/Human health and pathology/Pediatrics, medicine.medical_specialty, business.industry, Cleft Lip, Closure (topology), Infant, [SDV.MHEP.CHI]Life Sciences [q-bio]/Human health and pathology/Surgery, 030206 dentistry, Plastic Surgery Procedures, 030230 surgery, Surgery, Surgical glue, 03 medical and health sciences, 0302 clinical medicine, medicine, Humans, Tissue Adhesives, business, ComputingMilieux_MISCELLANEOUS
Abstract: International audience
Published: 2019
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4. Amniotic fluid peptides predict postnatal kidney survival in developmental kidney disease

Author: Romain Favre, Marie-Pierre Lavocat, Bernard Boudailliez, Charlotte Lucas, Camille Fédou, Jean-Sebastien Saulnier Blache, Anne-Sophie Weingertner, Blandine Hougas, Joost P. Schanstra, Pascal Gaucherand, Sylvie Cloarec, Julie Batut, Catherine Noel, J. Gondry, Philippe Eckart, Norbert Winer, Benjamin Breuil, Gérard Champion, Jean-Baptiste Benevent, Franck Perrotin, Christophe Vayssière, Florence Biquard, Harald Mischak, Gwenaelle Le Bouar, Jérôme Massardier, Françoise Conte Auriol, Pedro Magalhães, Sophie Martin, Jean-Paul Bory, Sophie Collardeau-Frachon, Eve Mousty, Lucie Bessenay, Corinne Floch, Julie Klein, Amelie Ryckewaert, Elisabeth Simon, Alain Martin, Guylène Bourdat-Michel, Marie-Françoise Froute, Franz Schaefer, Pascale Marcorelles, Stéphane Decramer, Nabila Moussaoui, Franck Boizard, Marie-Christine Manca-Pellissier, Mariannick Maupin-Hyvonnet, Marion Groussolles, Jean-Marie Delbosc, Guylène Feuillet, Anke Raaijmakers, François Nobili, Sophie Taque, Petra Zürbig, Vincent Guigonis, Audrey Casemayou, Patrick Blader, An Hindryckx, Luc Decatte, Karel Allegaert, Ophélie Lescat, Eric Neau, Odile Basmaison, Emma Allain-Launay, Agnes Sartor, Jean-Loup Bascands, Claudine Le Vaillant, Hélène Laurichesse Delmas, Bénédicte Buffin-Meyer, Nadia Lounis, Anne-Hélène Saliou, Véronique Baudouin, Elena Levtchenko, Maryse Fiorenza, Christine Pietrement, Valérie Goua, Marina Merveille, Laurent Bidat, Yves Aubard, Alexandra Benachi, Sylvie Kessler, Loic De Parscau, Jean-François Oury, Fabienne Prieur, Centre de biologie du développement (CBD), Centre de Biologie Intégrative (CBI), Université Toulouse III - Paul Sabatier (UT3), Université Fédérale Toulouse Midi-Pyrénées-Université Fédérale Toulouse Midi-Pyrénées-Centre National de la Recherche Scientifique (CNRS)-Université Toulouse III - Paul Sabatier (UT3), Université Fédérale Toulouse Midi-Pyrénées-Université Fédérale Toulouse Midi-Pyrénées-Centre National de la Recherche Scientifique (CNRS)-Centre National de la Recherche Scientifique (CNRS), Equipe 7 Inserm U1048, Institut des Maladies Métaboliques et Cardiovasculaires (I2MC), Institut National de la Santé et de la Recherche Médicale (INSERM)-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)-Université Toulouse III - Paul Sabatier (UT3), Université Fédérale Toulouse Midi-Pyrénées-Université Fédérale Toulouse Midi-Pyrénées, Université Fédérale Toulouse Midi-Pyrénées, University Hospitals Leuven [Leuven], Catholic University of Leuven - Katholieke Universiteit Leuven (KU Leuven), Laboratoire de Gérontechnologie [Hôpital La Grave-CHU de Toulouse], Université Fédérale Toulouse Midi-Pyrénées-Université Fédérale Toulouse Midi-Pyrénées-CHU Toulouse [Toulouse]-Institut National de la Santé et de la Recherche Médicale (INSERM)-Gérontopôle, Centre hospitalier universitaire de Poitiers (CHU Poitiers), Imagerie et cerveau (iBrain - Inserm U1253 - UNIV Tours ), Université de Tours-Institut National de la Santé et de la Recherche Médicale (INSERM), Hôpital Gatien de Clocheville [Tours] (CHRU Tours), Centre Hospitalier Régional Universitaire de Tours (CHRU Tours), AP-HP - Hôpital Antoine Béclère [Clamart], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP), Hôpital de la Timone [CHU - APHM] (TIMONE), CHU Clermont-Ferrand, Centre hospitalier universitaire de Nantes (CHU Nantes), Groupe de Recherche sur l'Analyse Multimodale de la Fonction Cérébrale - UMR INSERM_S 1105 (GRAMFC), Université de Picardie Jules Verne (UPJV)-CHU Amiens-Picardie-Institut National de la Santé et de la Recherche Médicale (INSERM), CHU Amiens-Picardie, Centre Hospitalier Universitaire de Nice (CHU Nice), Centre Hospitalier Universitaire de Saint-Etienne (CHU de Saint-Etienne), Centre Hospitalier Régional Universitaire de Brest (CHRU Brest), Hôpital Morvan - CHRU de Brest (CHU - BREST ), Centre Hospitalier René Dubos [Pontoise], Hôpital Louis Mourier - AP-HP [Colombes], Centre Hospitalier Universitaire [Grenoble] (CHU), Les Hôpitaux Universitaires de Strasbourg (HUS), AP-HP Hôpital universitaire Robert-Debré [Paris], Centre Hospitalier Universitaire de Reims (CHU Reims), Hospices Civils de Lyon (HCL), Hôpital Femme Mère Enfant [CHU - HCL] (HFME), Centre de dépistage des Carmes [Toulouse] (CDC), Hôpital des Enfants, CHU Toulouse [Toulouse], Laboratoire sur les interactions Epithéliums Neurones (LIEN), Université de Brest (UBO), Département de Pathologie [CHU Lyon-Sud - HCL], Centre Hospitalier Lyon Sud [CHU - HCL] (CHLS), Hospices Civils de Lyon (HCL)-Hospices Civils de Lyon (HCL), Mosaiques Diagnostics & Therapeutics AG [Hannover, Germany], University of Glasgow, Hannover Medical School [Hannover] (MHH), Centre National de la Recherche Scientifique (CNRS)-Université Toulouse III - Paul Sabatier (UT3), Université Fédérale Toulouse Midi-Pyrénées-Université Fédérale Toulouse Midi-Pyrénées-Centre de Biologie Intégrative (CBI), Diabète athérothrombose et thérapies Réunion Océan Indien (DéTROI), Institut National de la Santé et de la Recherche Médicale (INSERM)-Université de La Réunion (UR), Heidelberg University, Centre De Référence des Maladies Rénales Rares du Sud Ouest (SORARE), Centre De Référence des Maladies Rénales Rares du Sud Ouest, BIOMAN consortium: Karel Allegaert, Yves Aubard, Odile Basmaison, Jean-Baptiste Benevent, Florence Biquard, Gérard Champion, Jean-Marie Delbosc, Philippe Eckart, Marie-Françoise Froute, Pascal Gaucherand, Marion Groussolles, Vincent Guigonis, Blandine Hougas, Gwenaelle Le Bouar, Alain Martin, Sophie Martin, Mariannick Maupin-Hyvonnet, Marina Merveille, Eve Mousty, François Nobili, Amelie Ryckewaert, Agnes Sartor, Sophie Taque, Norbert Winer, Université Fédérale Toulouse Midi-Pyrénées-Université Fédérale Toulouse Midi-Pyrénées-Institut National de la Santé et de la Recherche Médicale (INSERM), Department of Development and Regeneration, Université Fédérale Toulouse Midi-Pyrénées-Université Fédérale Toulouse Midi-Pyrénées-Gérontopôle-Institut National de la Santé et de la Recherche Médicale (INSERM)-CHU Toulouse [Toulouse], Université de Tours (UT)-Institut National de la Santé et de la Recherche Médicale (INSERM), Clocheville, Department of Pediatric and Prenatal Radiology, Timone's Children-Hospital (APHM), Hôpital Dupuytren [CHU Limoges], CHU Toulouse, Hôpital des Enfants, Unité de Gastroentérologie, Hépatologie et Nutrition, Département de Pédiatrie, Hôpital Purpan [Toulouse], CHU Toulouse [Toulouse]-CHU Toulouse [Toulouse], Cardiovasculaire, métabolisme, diabétologie et nutrition (CarMeN), Institut National de Recherche pour l’Agriculture, l’Alimentation et l’Environnement (INRAE)-Université Claude Bernard Lyon 1 (UCBL), Université de Lyon-Université de Lyon-Institut National des Sciences Appliquées de Lyon (INSA Lyon), Institut National des Sciences Appliquées (INSA)-Université de Lyon-Institut National des Sciences Appliquées (INSA)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Hospices Civils de Lyon (HCL), Groupement Hospitalier Est [Bron], University Medical Center Heidelberg, Centre Hospitalier Régional Universitaire de Tours (CHRU TOURS), 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), Saulnier-Blache, Jean Sébastien, Pôle Gériatrie [CHU Toulouse], Centre Hospitalier Universitaire de Toulouse (CHU Toulouse), Université Fédérale Toulouse Midi-Pyrénées-Université Fédérale Toulouse Midi-Pyrénées-Centre National de la Recherche Scientifique (CNRS)-Centre de Biologie Intégrative (CBI), and Université de La Réunion (UR)-Institut National de la Santé et de la Recherche Médicale (INSERM)
Subjects: 0301 basic medicine, Pathology, medicine.medical_specialty, Amniotic fluid, Urinary system, [SDV]Life Sciences [q-bio], congenital anomalies of the kidney and the urinary tract, 030232 urology & nephrology, Kidney, Fetal Kidney, 03 medical and health sciences, 0302 clinical medicine, Pregnancy, medicine, Animals, Humans, termination of pregnancy, Prospective Studies, Child, Urinary Tract, Zebrafish, ComputingMilieux_MISCELLANEOUS, Fetus, business.industry, infants, Area under the curve, amniotic fluid, prediction, medicine.disease, 3. Good health, Pronephros, [SDV] Life Sciences [q-bio], 030104 developmental biology, medicine.anatomical_structure, Nephrology, Urogenital Abnormalities, peptides, Female, Kidney Diseases, business, management, Kidney disease
Abstract: Although a rare disease, bilateral congenital anomalies of the kidney and urinary tract (CAKUT) are the leading cause of end stage kidney disease in children. Ultrasound-based prenatal prediction of postnatal kidney survival in CAKUT pregnancies is far from accurate. To improve prediction, we conducted a prospective multicenter peptidome analysis of amniotic fluid spanning 140 evaluable fetuses with CAKUT. We identified a signature of 98 endogenous amniotic fluid peptides, mainly composed of fragments from extracellular matrix proteins and from the actin binding protein thymosin-β4. The peptide signature predicted postnatal kidney outcome with an area under the curve of 0.96 in the holdout validation set of patients with CAKUT with definite endpoint data. Additionally, this peptide signature was validated in a geographically independent sub-cohort of 12 patients (area under the curve 1.00) and displayed high specificity in non-CAKUT pregnancies (82 and 94% in 22 healthy fetuses and in 47 fetuses with congenital cytomegalovirus infection respectively). Change in amniotic fluid thymosin-β4 abundance was confirmed with ELISA. Knockout of thymosin-β4 in zebrafish altered proximal and distal tubule pronephros growth suggesting a possible role of thymosin β4 in fetal kidney development. Thus, recognition of the 98-peptide signature in amniotic fluid during diagnostic workup of prenatally detected fetuses with CAKUT can provide a long-sought evidence base for accurate management of the CAKUT disorder that is currently unavailable. ispartof: KIDNEY INTERNATIONAL vol:99 issue:3 pages:737-749 ispartof: location:United States status: published
Published: 2020
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5. Les soins palliatifs en néonatologie : une revue de littérature

Author: Flora Koliouli, Charlotte Casper, Chantal Zaouche Gaudron, Laurence Berdot-Talmier, Laboratoire Interdisciplinaire Solidarités, Sociétés, Territoires (LISST), École des hautes études en sciences sociales (EHESS)-Université Toulouse - Jean Jaurès (UT2J)-École Nationale Supérieure de Formation de l'Enseignement Agricole de Toulouse-Auzeville (ENSFEA)-Centre National de la Recherche Scientifique (CNRS), Unité de Néonatalogie [Hôpital des enfants Toulouse], CHU Toulouse [Toulouse], École des hautes études en sciences sociales (EHESS)-Université Toulouse - Jean Jaurès (UT2J)-Centre National de la Recherche Scientifique (CNRS)-École Nationale Supérieure de Formation de l'Enseignement Agricole de Toulouse-Auzeville (ENSFEA), Hôpital des Enfants, and CHU Toulouse [Toulouse]-CHU Toulouse [Toulouse]
Subjects: Advanced and Specialized Nursing, 03 medical and health sciences, 0302 clinical medicine, 030504 nursing, 030225 pediatrics, [SHS.PSY]Humanities and Social Sciences/Psychology, Medicine (miscellaneous), Fundamentals and skills, 0305 other medical science, 3. Good health
Abstract: International audience; The aim of this article is to establish the extent of knowledge in neonatal palliative care in order to reflect on new professional and research possibilities. We have used literature published between 2005 and 2016 available on electronic databases (Francis, Medline, PsychInfo) in English and/or French. This revealed two main trends: studies focused on professional practice in palliative care and those centered on the parents and their experience. The emphasis in the studies representing the carers’ points of view is on the need for training and the formalization of interventions with the medical professionals. The latter relate to medical aspects of pain management in newborn babies in palliative care and also to communication and relational skills. The subject of burnout in the profession is also raised. In the studies related to the parents, two major aspects are highlighted: their role as parents in the decision of when to end life and their own personal experience. As a result of our analysis of this research at a national and international level, we have exposed material for future research and improvements in professional practice; L’objectif de cet article est d’établir un état des connaissances sur les soins palliatifs en néonatologie afin de réfléchir sur de nouvelles perspectives tant professionnelles que de recherche. Nous avons utilisé la littérature publiée de 2005 à 2016, disponible sur les bases de données électroniques (Francis, Medline, PsychInfo) en langue anglaise et/ou française. Deux pôles importants sont relevés : les études axées sur les pratiques professionnelles en soins palliatifs et celles focalisées sur les parents et leur vécu ; les études portant sur le point de vue des soignants mettent l’accent sur la nécessité d’une formation et la formalisation des interventions auprès des professionnels de santé. Ces dernières portent sur des aspects médicaux de la gestion de la douleur chez le nouveau-né en soins palliatifs mais également sur ses compétences communicatives et relationnelles. Le burnout des professionnels est également mis en avant. Deux aspects principaux sont soulignés dans les études qui portent sur les parents : leur rôle en tant que parents vis-à-vis de la prise de décision quant à la fin de vie, et, leur propre vécu. À la suite de notre analyse sur ces recherches tant nationales qu’internationales, nous avons pu dégager pour de futures recherches et l’amélioration des pratiques professionnelles.
Published: 2017
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6. Glutamate controls vessel-associated migration of GABA interneurons from the pial migratory route via NMDA receptors and endothelial protease activation

Author: Léger, Cécile, Dupré, Nicolas, Aligny, Caroline, Bénard, Magalie, Lebon, Alexis, Henry, Vincent, Hauchecorne, Michelle, Galas, Ludovic, Frebourg, Thierry, Leroux, Philippe, Vivien, Denis, Lecointre, Maryline, Marret, Stéphane, Gonzalez, Bruno J., Team 4 'NeoVasc' - INSERM U1245, Génomique et Médecine Personnalisée du Cancer et des Maladies Neuropsychiatriques (GPMCND), Université de Rouen Normandie (UNIROUEN), Normandie Université (NU)-Normandie Université (NU)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Université de Rouen Normandie (UNIROUEN), Normandie Université (NU)-Normandie Université (NU)-Institut National de la Santé et de la Recherche Médicale (INSERM), Endothélium microcirculatoire cérébral et lésions du système nerveux central au cours du développement (Néovasc), Normandie Université (NU)-Normandie Université (NU)-Institute for Research and Innovation in Biomedicine (IRIB), Normandie Université (NU)-Normandie Université (NU)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Centre National de la Recherche Scientifique (CNRS)-Université de Rouen Normandie (UNIROUEN), Normandie Université (NU)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Centre National de la Recherche Scientifique (CNRS)-Institut National de la Santé et de la Recherche Médicale (INSERM), Unité de Néonatalogie [Hôpital des enfants Toulouse], Hôpital des Enfants, CHU Toulouse [Toulouse]-CHU Toulouse [Toulouse], Lab Phys Collis Electron & Atom, Université de Brest (UBO), Neuroendocrinologie cellulaire et moléculaire, GIP Cyceron (Cyceron), Université de Caen Normandie (UNICAEN), Normandie Université (NU)-Normandie Université (NU)-Commissariat à l'énergie atomique et aux énergies alternatives (CEA)-CHU Caen, Normandie Université (NU)-Tumorothèque de Caen Basse-Normandie (TCBN)-Tumorothèque de Caen Basse-Normandie (TCBN)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Centre National de la Recherche Scientifique (CNRS), Team 4 NeoVasc - Region Team ERI 28 INSERM (Neovasc), Service Obstétrique [CHU Toulouse], Pôle Femme-Mère-Couple [CHU Toulouse], and Centre Hospitalier Universitaire de Toulouse (CHU Toulouse)-Centre Hospitalier Universitaire de Toulouse (CHU Toulouse)
Subjects: Endothelial cells, Neurogenesis, [SDV]Life Sciences [q-bio], Glutamic Acid, Mice, Transgenic, Receptors, N-Methyl-D-Aspartate, GABA interneuron, Mice, Cell Movement, Interneurons, Animals, Humans, GABAergic Neurons, Migration, gamma-Aminobutyric Acid, ComputingMilieux_MISCELLANEOUS, Brain Mapping, Glutamate Decarboxylase, Somatosensory Cortex, NMDAR, nervous system, Animals, Newborn, Gene Expression Regulation, Matrix Metalloproteinase 9, t-PA, Tissue Plasminogen Activator, Blood Vessels, Original Article, MMP-9
Abstract: During cortex development, fine interactions between pyramidal cells and migrating GABA neurons are required to orchestrate correct positioning of interneurons, but cellular and molecular mechanisms are not yet clearly understood. Functional and age-specific expression of NMDA receptors by neonate endothelial cells suggests a vascular contribution to the trophic role of glutamate during cortical development. Associating functional and loss-of-function approaches, we found that glutamate stimulates activity of the endothelial proteases MMP-9 and t-PA along the pial migratory route (PMR) and radial cortical microvessels. Activation of MMP-9 was NMDAR-dependent and abrogated in t-PA−/− mice. Time-lapse recordings revealed that glutamate stimulated migration of GABA interneurons along vessels through an NMDAR-dependent mechanism. In Gad67-GFP mice, t-PA invalidation and in vivo administration of an MMP inhibitor impaired positioning of GABA interneurons in superficial cortical layers, whereas Grin1 endothelial invalidation resulted in a strong reduction of the thickness of the pial migratory route, a marked decrease of the glutamate-induced MMP-9-like activity along the PMR and a depopulation of interneurons in superficial cortical layers. This study supports that glutamate controls the vessel-associated migration of GABA interneurons by regulating the activity of endothelial proteases. This effect requires endothelial NMDAR and is t-PA-dependent. These neurodevelopmental data reinforce the debate regarding safety of molecules with NMDA-antagonist properties administered to preterm and term neonates. Electronic supplementary material The online version of this article (10.1007/s00018-019-03248-5) contains supplementary material, which is available to authorized users.
Published: 2019
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7. Feasibility, Safety and Accuracy of Echocardiography-Fluoroscopy Imaging Fusion During Percutaneous Atrial Septal Defect Closure in Children

Author: Clément Karsenty, Alain Fraisse, Khaled Hadeed, Francoise Auriol, Nicolas Combes, Philippe Acar, François Heitz, Gerald Chausseray, Xavier Alacoque, Yves Dulac, Pascal Amedro, Sébastien Hascoët, Université Paris-Sud - Paris 11 - Faculté de médecine (UP11 UFR Médecine), Université Paris-Sud - Paris 11 (UP11), Université Toulouse III - Paul Sabatier (UT3), Université Fédérale Toulouse Midi-Pyrénées, CHU Toulouse [Toulouse], Service pédiatrie-cardiologie, CHU Toulouse [Toulouse]-Hôpital des Enfants, Laboratoire des sciences de l'ingénieur, de l'informatique et de l'imagerie (ICube), Institut National des Sciences Appliquées - Strasbourg (INSA Strasbourg), Institut National des Sciences Appliquées (INSA)-Institut National des Sciences Appliquées (INSA)-Université de Strasbourg (UNISTRA)-Centre National de la Recherche Scientifique (CNRS)-École Nationale du Génie de l'Eau et de l'Environnement de Strasbourg (ENGEES)-Réseau nanophotonique et optique, Centre National de la Recherche Scientifique (CNRS)-Université de Strasbourg (UNISTRA)-Université de Haute-Alsace (UHA) Mulhouse - Colmar (Université de Haute-Alsace (UHA))-Centre National de la Recherche Scientifique (CNRS)-Université de Strasbourg (UNISTRA)-Université de Haute-Alsace (UHA) Mulhouse - Colmar (Université de Haute-Alsace (UHA))-Matériaux et nanosciences d'Alsace (FMNGE), Institut de Chimie du CNRS (INC)-Université de Strasbourg (UNISTRA)-Université de Haute-Alsace (UHA) Mulhouse - Colmar (Université de Haute-Alsace (UHA))-Institut National de la Santé et de la Recherche Médicale (INSERM)-Centre National de la Recherche Scientifique (CNRS)-Institut de Chimie du CNRS (INC)-Université de Strasbourg (UNISTRA)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Centre National de la Recherche Scientifique (CNRS), Clinique Pasteur [Toulouse], CHU Toulouse, Hôpital des Enfants, Unité de Gastroentérologie, Hépatologie et Nutrition, Département de Pédiatrie, Hôpital Purpan [Toulouse], CHU Toulouse [Toulouse]-CHU Toulouse [Toulouse], Physiologie & médecine expérimentale du Cœur et des Muscles [U 1046] (PhyMedExp), Institut National de la Santé et de la Recherche Médicale (INSERM)-Université de Montpellier (UM)-Centre National de la Recherche Scientifique (CNRS), Centre Hospitalier Régional Universitaire [Montpellier] (CHRU Montpellier), Aix Marseille Université (AMU), Université de Toulouse (UT), Centre Hospitalier Universitaire de Toulouse (CHU Toulouse), Service Cardiologie pédiatrique [CHU Toulouse], Pôle Enfants [CHU Toulouse], Centre Hospitalier Universitaire de Toulouse (CHU Toulouse)-Centre Hospitalier Universitaire de Toulouse (CHU Toulouse), École Nationale du Génie de l'Eau et de l'Environnement de Strasbourg (ENGEES)-Université de Strasbourg (UNISTRA)-Institut National des Sciences Appliquées - Strasbourg (INSA Strasbourg), Institut National des Sciences Appliquées (INSA)-Institut National des Sciences Appliquées (INSA)-Institut National de Recherche en Informatique et en Automatique (Inria)-Les Hôpitaux Universitaires de Strasbourg (HUS)-Centre National de la Recherche Scientifique (CNRS)-Matériaux et Nanosciences Grand-Est (MNGE), Université de Strasbourg (UNISTRA)-Université de Haute-Alsace (UHA) Mulhouse - Colmar (Université de Haute-Alsace (UHA))-Institut National de la Santé et de la Recherche Médicale (INSERM)-Institut de Chimie du CNRS (INC)-Centre National de la Recherche Scientifique (CNRS)-Université de Strasbourg (UNISTRA)-Université de Haute-Alsace (UHA) Mulhouse - Colmar (Université de Haute-Alsace (UHA))-Institut National de la Santé et de la Recherche Médicale (INSERM)-Institut de Chimie du CNRS (INC)-Centre National de la Recherche Scientifique (CNRS)-Réseau nanophotonique et optique, Université de Strasbourg (UNISTRA)-Université de Haute-Alsace (UHA) Mulhouse - Colmar (Université de Haute-Alsace (UHA))-Centre National de la Recherche Scientifique (CNRS)-Université de Strasbourg (UNISTRA)-Centre National de la Recherche Scientifique (CNRS), and Service Gastroentérologie, hépatologie nutrition, diabétologie et maladies héréditaires du métabolisme pédiatrique [CHU Toulouse]
Subjects: Male, Cardiac Catheterization, Percutaneous, Adolescent, Septal Occluder Device, Pediatric cardiology, medicine.medical_treatment, [SDV]Life Sciences [q-bio], education, Echocardiography, Three-Dimensional, 030204 cardiovascular system & hematology, Multimodal Imaging, Heart Septal Defects, Atrial, Congenital heart diseases, 03 medical and health sciences, 0302 clinical medicine, Interquartile range, medicine, Fluoroscopy, Humans, Radiology, Nuclear Medicine and imaging, 030212 general & internal medicine, Prospective Studies, Cardiac Surgical Procedures, Child, Fluoroscopic imaging, Cardiac catheterization, 3D echocardiography, medicine.diagnostic_test, business.industry, Imaging guidance, Reproducibility of Results, Atrial septal defect closure, Echocardiography, Doppler, Color, Treatment Outcome, Surgery, Computer-Assisted, Child, Preschool, Feasibility Studies, Female, Cardiology and Cardiovascular Medicine, Fluoroscopic image, business, Nuclear medicine, EchoNavigator, Echocardiography, Transesophageal, Imaging fusion
Abstract: Imaging fusion between echocardiography and fluoroscopy was recently developed. The aim of this study was to assess its feasibility and accuracy during pediatric cardiac catheterization.Thirty-one patients (median weight, 26 kg; interquartile range [IQR], 21-37 kg) who underwent percutaneous atrial septal defect closure were prospectively included. The feasibility and accuracy of various imaging fusion modalities (live two-dimensional, live color two-dimensional, live three-dimensional and markers) with EchoNavigator software were assessed. To assess the accuracy of spatial registration of the echocardiogram on the fluoroscopic image, the occluder screw, an object that appeared on each image, was used as a reference tool, and the distance between the two when fused was measured. A distance was measured on the fusion screen between a marker positioned on the screw from the echocardiography screen and from the fluoroscopy screen (distance 1). Another distance was measured on the fusion screen between the screw visualized by three-dimensional echocardiography and by fluoroscopy (distance 2). The two distances were measured on four C-arm orientations in end-systolic and end-diastolic frames.Fusion and marker positioning were feasible in real time in all cases. On the fusion screen, median systolic and diastolic distance 1 were 0.5 mm (IQR, 0.3-1 mm) and 2 mm (IQR, 1.5-2.5 mm; P .0001), respectively. The marker positioned from the echocardiography screen was fixed on the fusion screen and did not follow the movement of the screw. Median systolic and diastolic distance 2 were 0.5 mm (IQR, 0-0.5 mm) and 2 mm (IQR, 1.5-2.5 mm; P .0001), respectively.Echocardiographic fluoroscopic imaging fusion is feasible, safe, and accurate in children weighting20 kg. This technique offers a new method of imaging guidance in the catheterization laboratory for complex procedures and training.
Published: 2018
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8. Sodium bicarbonate therapy for patients with severe metabolic acidaemia in the intensive care unit (BICAR-ICU): a multicentre, open-label, randomised controlled, phase 3 trial

Author: Samir Jaber, Catherine Paugam, Emmanuel Futier, Jean-Yves Lefrant, Sigismond Lasocki, Thomas Lescot, Julien Pottecher, Alexandre Demoule, Martine Ferrandière, Karim Asehnoune, Jean Dellamonica, Lionel Velly, Paër-Sélim Abback, Audrey de Jong, Vincent Brunot, Fouad Belafia, Antoine Roquilly, Gérald Chanques, Laurent Muller, Jean-Michel Constantin, Helena Bertet, Kada Klouche, Nicolas Molinari, Boris Jung, Marion Monnin, Jean-Marc Delay, Moussa Cissé, Marie Geniez, Matthieu Conseil, Bruno Souche, Jean Michel Constantin, Eric Noll, Elise Morawiec, Alexandre Robert, Thibaut Triglia, Malika Mechati, Jean-Michel Arnal, Jacques Durand-Gasselin, Didier Demoly, Sami Hraiech, Laurent Papazian, Vincent Gilles, Thomas Rimmelé, Béatrice Riu, Pierre Cougot, Olivier Fourcade, Philippe Seguin, Jonathan Charbit, Xavier Capdevila, Marc Leone, Laurent Zieleskiewicz, Carole Ichai, Jean Christophe Orban, Michael Darmon, Elie Azoulay, Virginie Lemiale, Lara Zafrani, Karim Debbat, Oliver Mimoz, Claude Guérin, Eric Kipnis, Physiologie & médecine expérimentale du Cœur et des Muscles [U 1046] (PhyMedExp), Institut National de la Santé et de la Recherche Médicale (INSERM)-Université de Montpellier (UM)-Centre National de la Recherche Scientifique (CNRS), Centre Hospitalier Régional Universitaire [Montpellier] (CHRU Montpellier), Hôpital Beaujon [AP-HP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP), Génétique, Reproduction et Développement (GReD), Institut National de la Santé et de la Recherche Médicale (INSERM)-Université Clermont Auvergne [2017-2020] (UCA [2017-2020])-Centre National de la Recherche Scientifique (CNRS), Centre Hospitalier Universitaire de Nîmes (CHU Nîmes), Service d'anesthésie et réanimation chirurgicale, Centre Hospitalier Universitaire d'Angers (CHU Angers), PRES Université Nantes Angers Le Mans (UNAM)-PRES Université Nantes Angers Le Mans (UNAM), Institut du Cerveau = Paris Brain Institute (ICM), 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)-Sorbonne Université (SU)-Centre National de la Recherche Scientifique (CNRS), Université de Strasbourg (UNISTRA), CHU Pitié-Salpêtrière [AP-HP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU), Centre Hospitalier Régional Universitaire de Tours (CHRU Tours), Thérapeutiques cliniques et expérimentales des infections (EA 3826) (EA 3826), Université de Nantes - UFR de Médecine et des Techniques Médicales (UFR MEDECINE), Université de Nantes (UN)-Université de Nantes (UN), 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), Assistance Publique - Hôpitaux de Marseille (APHM), Hôpital Beaujon, Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Université Paris Diderot - Paris 7 (UPD7)-Hôpital Beaujon [AP-HP], Centre hospitalier universitaire de Nantes (CHU Nantes), CHU Clermont-Ferrand, Génétique, Reproduction et Développement - Clermont Auvergne (GReD), Institut National de la Santé et de la Recherche Médicale (INSERM)-Université Clermont Auvergne (UCA)-Centre National de la Recherche Scientifique (CNRS), Centre Hospitalier Régional Universitaire de Nîmes (CHRU Nîmes), Institut du Cerveau et de la Moëlle Epinière = Brain and Spine Institute (ICM), Institut National de la Santé et de la Recherche Médicale (INSERM)-CHU Pitié-Salpêtrière [APHP]-Sorbonne Université (SU)-Centre National de la Recherche Scientifique (CNRS), CHU Pitié-Salpêtrière [APHP], Assistance publique - Hôpitaux de Paris (AP-HP) (APHP)-Université Paris Diderot - Paris 7 (UPD7)-Hôpital Beaujon, Hôpital des Enfants Albert Royer, Laboratoire de Bactériologie, Hôpital des Enfants Albert Royer, Laboratoire de Bactériologie, Avenue Cheikh Anta Diop, Dakar, Senegal., Anesthesiology, Robert Debré Hospital, PRES Université Nantes Angers Le Mans (UNAM), Service des Soins Intensifs [CHU Saint-Antoine], Assistance publique - Hôpitaux de Paris (AP-HP) (APHP)-CHU Saint-Antoine [APHP], Mitochondries, stress oxydant et protection musculaire (Strasbourg), Mitochondrie, stress oxydant et protection musculaire (MSP), Université de Strasbourg (UNISTRA)-Université de Strasbourg (UNISTRA), Sorbonne Université (SU), Service de Pneumologie et Réanimation Médicale [CHU Pitié-Salpêtrière] (Département ' R3S '), Assistance publique - Hôpitaux de Paris (AP-HP) (APHP)-CHU Pitié-Salpêtrière [APHP], 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), Conservation des espèces, restauration et suivi des populations (CERSP), Université Pierre et Marie Curie - Paris 6 (UPMC)-Centre National de la Recherche Scientifique (CNRS), Service d'anesthésie et de réanimation [Hôpital de la Timone - APHM], Hôpital de la Timone [CHU - APHM] (TIMONE), sans affiliation, Centre Hospitalier de Toulon-La Seyne, Service de réanimation-Détresses Respiratoires et Infections Sévères [Hôpital Nord - APHM] (DRIS), Aix Marseille Université (AMU)-Assistance Publique - Hôpitaux de Marseille (APHM)- Hôpital Nord [CHU - APHM], Unité de Recherche sur les Maladies Infectieuses et Tropicales Emergentes (URMITE), Institut de Recherche pour le Développement (IRD)-Aix Marseille Université (AMU)-Institut National de la Santé et de la Recherche Médicale (INSERM)-IFR48, INSB-INSB-Centre National de la Recherche Scientifique (CNRS), Agressions vasculaires et réponses tissulaires, Université Claude Bernard Lyon 1 (UCBL), Université de Lyon-Université de Lyon-Institut National de la Santé et de la Recherche Médicale (INSERM), CHU Toulouse [Toulouse], Service de cardiologie [Toulouse], Université Toulouse III - Paul Sabatier (UT3), Université Fédérale Toulouse Midi-Pyrénées-Université Fédérale Toulouse Midi-Pyrénées-CHU Toulouse [Toulouse]-Hôpital de Rangueil, CHU Pontchaillou [Rennes], Institut des Neurosciences de Montpellier - Déficits sensoriels et moteurs (INM), Institut National de la Santé et de la Recherche Médicale (INSERM)-Université de Montpellier (UM), Service Anesthésie et Réanimation [Hôpital Nord - APHM], Service de réanimation, Centre Hospitalier Universitaire de Nice (CHU Nice)-Hôpital St Roch, Département Imagerie et Simulation pour le Contrôle (DISC), Laboratoire d'Intégration des Systèmes et des Technologies (LIST), 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-Saclay-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-Saclay, Medical ICU, Assistance publique - Hôpitaux de Paris (AP-HP) (APHP)-Groupe Hospitalier Saint Louis - Lariboisière - Fernand Widal [Paris], Assistance publique - Hôpitaux de Paris (AP-HP) (APHP), Service de réanimation médicale, Assistance publique - Hôpitaux de Paris (AP-HP) (APHP)-Université Paris Diderot - Paris 7 (UPD7)-Groupe Hospitalier Saint Louis - Lariboisière - Fernand Widal [Paris], Service de Réanimation Médicale, Hôpital Saint Louis, Paris, France, Laboratoire de Géologie de Lyon - Terre, Planètes, Environnement [Lyon] (LGL-TPE), École normale supérieure - Lyon (ENS Lyon)-Université Claude Bernard Lyon 1 (UCBL), Université de Lyon-Université de Lyon-Institut national des sciences de l'Univers (INSU - CNRS)-Centre National de la Recherche Scientifique (CNRS), Université Lille 2 - Faculté de Médecine, 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)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU)-Centre National de la Recherche Scientifique (CNRS), Sorbonne Université (SU)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP), Université de Montpellier (UM)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Centre National de la Recherche Scientifique (CNRS), Centre National de la Recherche Scientifique (CNRS)-Université Clermont Auvergne [2017-2020] (UCA [2017-2020])-Institut National de la Santé et de la Recherche Médicale (INSERM), 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)-Centre National de la Recherche Scientifique (CNRS), and Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Université Paris Diderot - Paris 7 (UPD7)
Subjects: Bicarbonate, medicine.medical_treatment, [SDV]Life Sciences [q-bio], Population, Metabolic alkalosis, law.invention, Cohort Studies, 03 medical and health sciences, chemistry.chemical_compound, 0302 clinical medicine, law, Intensive care, Medicine, Humans, 030212 general & internal medicine, Renal replacement therapy, education, Infusions, Intravenous, ComputingMilieux_MISCELLANEOUS, education.field_of_study, Sodium bicarbonate, business.industry, 030208 emergency & critical care medicine, General Medicine, [SDV.MHEP.EM]Life Sciences [q-bio]/Human health and pathology/Endocrinology and metabolism, Hydrogen-Ion Concentration, medicine.disease, Intensive care unit, Survival Analysis, 3. Good health, Renal Replacement Therapy, Intensive Care Units, Intravenous sodium bicarbonate, Sodium Bicarbonate, chemistry, Anesthesia, [SDV.SPEE]Life Sciences [q-bio]/Santé publique et épidémiologie, business, Acidosis
Abstract: Summary Background Acute acidaemia is frequently observed during critical illness. Sodium bicarbonate infusion for the treatment of severe metabolic acidaemia is a possible treatment option but remains controversial, as no studies to date have examined its effect on clinical outcomes. Therefore, we aimed to evaluate whether sodium bicarbonate infusion would improve these outcomes in critically ill patients. Methods We did a multicentre, open-label, randomised controlled, phase 3 trial. Local investigators screened eligible patients from 26 intensive care units (ICUs) in France. We included adult patients (aged ≥18 years) who were admitted within 48 h to the ICU with severe acidaemia (pH ≤7·20, PaCO2 ≤45 mm Hg, and sodium bicarbonate concentration ≤20 mmol/L) and with a total Sequential Organ Failure Assessment score of 4 or more or an arterial lactate concentration of 2 mmol/L or more. We randomly assigned patients (1:1), by stratified randomisation with minimisation via a restricted web platform, to receive either no sodium bicarbonate (control group) or 4·2% of intravenous sodium bicarbonate infusion (bicarbonate group) to maintain the arterial pH above 7·30. Our protocol recommended that the volume of each infusion should be within the range of 125–250 mL in 30 min, with a maximum of 1000 mL within 24 h after inclusion. Randomisation criteria were stratified among three prespecified strata: age, sepsis status, and the Acute Kidney Injury Network (AKIN) score. The primary outcome was a composite of death from any cause by day 28 and the presence of at least one organ failure at day 7. All analyses were done on data from the intention-to-treat population, which included all patients who underwent randomisation. This study is registered with ClinicalTrials.gov, number NCT02476253. Findings Between May 5, 2015, and May 7, 2017, we enrolled 389 patients into the intention-to-treat analysis in the overall population (194 in the control group and 195 in the bicarbonate group). The primary outcome occurred in 138 (71%) of 194 patients in the control group and 128 (66%) of 195 in the bicarbonate group (absolute difference estimate −5·5%, 95% CI −15·2 to 4·2; p=0·24). The Kaplan-Meier method estimate of the probability of survival at day 28 between the control group and bicarbonate group was not significant (46% [95% CI 40–54] vs 55% [49–63]; p=0·09. In the prespecified AKIN stratum of patients with a score of 2 or 3, the Kaplan-Meier method estimate of survival by day 28 between the control group and bicarbonate group was significant (37% [95% CI 28–48] vs 54% [45–65]; p=0·0283). Metabolic alkalosis, hypernatraemia, and hypocalcaemia were observed more frequently in the bicarbonate group than in the control group, with no life-threatening complications reported. Interpretation In patients with severe metabolic acidaemia, sodium bicarbonate had no effect on the primary composite outcome. However, sodium bicarbonate decreased the primary composite outcome and day 28 mortality in the a-priori defined stratum of patients with acute kidney injury. Funding French Ministry of Health and the Societe Francaise d'Anesthesie Reanimation.
Published: 2018
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9. One-Year Outcome for Congenital Diaphragmatic Hernia: Results From the French National Register

Author: Charles Muszynski, Jean Breaud, Quentin Ballouhey, Elsa Kermorvant, Sylvain Samperiz, Valérie Datin-Dorrière, Laurent Storme, Anne Schneider, Thierry Blanc, Alexis Arnaud, Odile Pidoux, François Barrière, Norbert Winer, Julia Boubnova, Elisabeth Carricaburu, Fabrice Michel, Armelle Garenne, Nicolas Berte, Frederic Lavrand, Yann Chaussy, Aurélien Binet, Amélie Desrumaux, Sébastien Blanc, Anderson Loundou, Guillaume Levard, Virginie Fouquet, Marie-Odile Marcoux, Isabelle Rayet, Urgences pédiatriques [Hôpital de la Timone - APHM], Aix Marseille Université (AMU)-Assistance Publique - Hôpitaux de Marseille (APHM)- Hôpital de la Timone [CHU - APHM] (TIMONE), Centre d'études et de recherche sur les services de santé et la qualité de vie (CEReSS), Aix Marseille Université (AMU), Hôpital Bicètre, Service de chirurgie pédiatrique, Hôpital Bicêtre, unité de soins intensifs néonatals, Université Paris Descartes - Paris 5 (UPD5)-CHU Necker - Enfants Malades [AP-HP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP), Unité de soins intensifs néonatals, Hôpital Femme Mère Enfant [CHU - HCL] (HFME), Hospices Civils de Lyon (HCL)-Hospices Civils de Lyon (HCL), Service de chirurgie pédiatrique, AP-HP Hôpital universitaire Robert-Debré [Paris], unité de soins intensifs pédiatriques, hôpital couple-enfant [CHU Grenoble Alpes], département de néonatologie [Centre Hospitalier Régional Universitaire [Montpellier], Centre Hospitalier Régional Universitaire [Montpellier] (CHRU Montpellier), Service de chirurgie pédiatrique [Rennes] = Paediatric / Pediatric surgery [Rennes], CHU Pontchaillou [Rennes], Département de chriurgie, Centre Hospitalier Régional Universitaire de Nancy (CHRU Nancy), Service de pédiatrie néonatale et réanimation - neuropédiatrie [CHU Rouen], CHU Rouen, Normandie Université (NU)-Normandie Université (NU)-Hôpital Charles Nicolle [Rouen]-Université de Rouen Normandie (UNIROUEN), Normandie Université (NU), Service de chirurgie pédiatrique [CHU Bordeaux], Hôpital des Enfants - Groupe hospitalier Pellegrin - CHU de Bordeaux, service de chirurgie pédiatrique, Centre hospitalier universitaire de Poitiers (CHU Poitiers), Hôpital Nord (Saint Etienne), Centre hospitalier Félix-Guyon [Saint-Denis, La Réunion], Département de chirurgie pédiatrique, Hôpital de Hautepierre [Strasbourg], Unité de soins intensifs néonatals [Hôpital des Enfants, Toulouse], Hôpital des Enfants, CHU Toulouse [Toulouse]-CHU Toulouse [Toulouse], Département d'obstétrique et Gynécologie [Hôtel-Dieu, Nantes], Hôtel-Dieu de Nantes, Département de chirurgie pédiatrique [Hôspital Universitaire Jean Minjoz , Besançon], Hôpital JeanMinjoz, 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), Service de chirurgie pédiatrique viscérale, orthopédique et plastique [CHU Limoges], CHU Limoges, Département de chirurgie pédiatrique [Clocheville University Hospital, Tours], CHRU Clocheville, Département d'obstétrique Gynécologie [, Amiens University Hospital, Amiens], CHU Amiens-Picardie, Département de chirurgie pédiatrique [Nice Pediatric Hospital, University of Nice-Sophia Antipolis, Nice], Université Nice Sophia Antipolis (... - 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 de Nice), CHRU de Brest - Département de Pédiatrie (CHU BREST Pédiatrie), Centre Hospitalier Régional Universitaire de Brest (CHRU Brest), Service de médecine néonatale [Lille University Hospital, Lille], Centre Hospitalier Régional Universitaire [Lille] (CHRU Lille), Chirurgie orthopédique et pédiatrique [Hôpital de la Timone - APHM], Groupe Hospitalier Sud, Service de pédiatrie néonatale et réanimation - neuropédiatrie [Rouen], Normandie Université (NU)-Normandie Université (NU)-Université de Rouen Normandie (UNIROUEN), Normandie Université (NU)-Hôpital Charles Nicolle [Rouen], Hôpital Universitaire d'Amiens, Université Côte d'Azur (UCA)-Université Côte d'Azur (UCA)-Centre Hospitalier Universitaire de Nice (CHU de Nice), Ingénierie Moléculaire et Physiopathologie Articulaire (IMoPA), Université de Lorraine (UL)-Centre National de la Recherche Scientifique (CNRS), Hôpital Charles Nicolle [Rouen], Normandie Université (NU)-Normandie Université (NU)-CHU Rouen, Centre Hospitalier Universitaire de Saint-Etienne [CHU Saint-Etienne] (CHU ST-E), Service Réanimation néonatale et pédiatrique spécialisée [CHU Toulouse], Pôle Enfants [CHU Toulouse], Centre Hospitalier Universitaire de Toulouse (CHU Toulouse)-Centre Hospitalier Universitaire de Toulouse (CHU Toulouse), 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), Service de chirurgie pédiatrique [Rennes], Université de Rennes 1 (UR1), Université de Rennes (UNIV-RENNES)-Université de Rennes (UNIV-RENNES)-CHU Pontchaillou [Rennes], Hôpital Charles Nicolle [Rouen]-CHU Rouen, and COMUE Université Côte d'Azur (2015 - 2019) (COMUE UCA)-COMUE Université Côte d'Azur (2015 - 2019) (COMUE UCA)-Centre Hospitalier Universitaire de Nice (CHU de Nice)
Subjects: Male, Pediatrics, medicine.medical_specialty, Adverse outcomes, education, congenital diaphragmatic hernia, 03 medical and health sciences, 0302 clinical medicine, Pregnancy, Risk Factors, 030225 pediatrics, Prenatal Diagnosis, Infant Mortality, medicine, Hospital discharge, Humans, 030212 general & internal medicine, Fetal loss, Prospective Studies, Registries, health care economics and organizations, business.industry, Mortality rate, Persistent pulmonary hypertension, Infant, Newborn, Congenital diaphragmatic hernia, Infant, Prenatal Care, medicine.disease, mortality, 3. Good health, Survival Rate, Treatment Outcome, Prenatal risk, Pediatrics, Perinatology and Child Health, outcome, Female, France, prognosis, business, Hernias, Diaphragmatic, Congenital, [SDV.MHEP]Life Sciences [q-bio]/Human health and pathology
Abstract: To evaluate the status of congenital diaphragmatic hernia (CDH) management in France and to assess predictors of adverse outcomes.We reviewed the first-year outcome of all cases of CDH reported to the French National Register in 2011.A total of 158 cases were included. Of these, 83% (131) were prenatally diagnosed, with a mortality rate of 39% (44 of 112) for live born infants with a known outcome at hospital discharge. Mortality increased to 47% (60 of 128) including those with termination of pregnancy and fetal loss. This contrasts with the 7% (2 of 27) mortality rate of the patients diagnosed postnatally (P = .002). Mortality worsened with 1 prenatal marker of CDH severity (OR 3.38 [1.30-8.83] P = .013) and worsened further with 2 markers (OR 20.64 [5.29-80.62] P .001). Classic postnatal risk factors of mortality such as side of hernia (nonleft P = .001), prematurity (P .001), low birth weight (P = .002), and size of the defect (P .001) were confirmed. Of the 141 live births (114 prenatal and 27 postnatal diagnosis) with known outcomes, 93 (67%) survived to hospital discharge, 68 (60%) with a prenatal diagnosis and 25 (93%) with a postnatal diagnosis. The median time to hospital discharge was 34 days (IQR, 19.25-62). Of these survivors, 71 (76%) were followed up for 1 year.Despite advances in management of CDH, mortality was high and associated with prenatal risk factors. Postnatally, severe persistent pulmonary hypertension was difficult to predict and presented persistent challenges in management.
Published: 2018
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10. Renal parenchyma impairment characterization in partial unilateral ureteral obstruction in mice with intravoxel incoherent motion-MRI

Author: Ulrich Blank, Michel Peuchmaur, Simon A. Lambert, Miguel Albuquerque, Marianne Alison, Maguelonne Pons, Liza Ali, Benjamin Leporq, Alaa El Ghoneimi, Marie-Laurence Poli Merol, 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), Laboratoire d'Excellence Inflamex [Paris] (Faculté de Médecine Xavier Bichat), Université Paris Diderot - Paris 7 (UPD7)-Université Sorbonne Paris Cité (COMUE) (USPC), Centre de Recherche en Acquisition et Traitement de l'Image pour la Santé (CREATIS), Université Jean Monnet [Saint-Étienne] (UJM)-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-Centre National de la Recherche Scientifique (CNRS)-Institut National de la Santé et de la Recherche Médicale (INSERM), RMN et optique : De la mesure au biomarqueur, Université de Lyon-Centre National de la Recherche Scientifique (CNRS)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Université Jean Monnet [Saint-Étienne] (UJM)-Hospices Civils de Lyon (HCL)-Institut National des Sciences Appliquées de Lyon (INSA Lyon), Chirurgie pédiatrique et urologie [Hôpital Robert Debré - APHP], Hôpital Robert Debré-Université Paris Diderot - Paris 7 (UPD7)-Université Sorbonne Paris Cité (COMUE) (USPC), Neuroprotection du Cerveau en Développement / Promoting Research Oriented Towards Early Cns Therapies (PROTECT), Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Hôpital Robert Debré-Université Paris Diderot - Paris 7 (UPD7)-Institut National de la Santé et de la Recherche Médicale (INSERM), Département de Radiologie Pédiatrique [AP-HP Hôpital Robert-Debré], Université Paris Diderot - Paris 7 (UPD7)-AP-HP Hôpital universitaire Robert-Debré [Paris], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP), Service de Pathologie [Hôpital Beaujon - APHP], Hôpital Beaujon [AP-HP], Service de Pathologie [Hôpital Robert Debré - APHP], Sorbonne Paris Cité-Hôpital Robert Debré-Université Paris Diderot - Paris 7 (UPD7), Chirurgie Pédiatrique [Reims] (CHU de Reims - American Memorial Hospital (Hôpital des enfants)), Centre Hospitalier Universitaire de Reims (CHU Reims)-American Memorial Hospital (Hôpital des enfants) [Reims], Institut National de la Santé et de la Recherche Médicale, Centre National de la Recherche Scientifique, and Paris‐Diderot University, French National Research Agency, Investissements d'Avenir programme, Grant/Award Number: ANR‐11‐IDEX‐0005‐02, Sorbonne Paris Cite, Laboratoire d'excellence INFLAMEX, Association des amis de l'American Memorial Hospital Reims France, Section Française d'Urologie Pédiatrique et de l'Adolescent (SFUPA), ANR-11-IDEX-0005,USPC,Université Sorbonne Paris Cité(2011), 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)-Université Jean Monnet - Saint-Étienne (UJM)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Centre National de la Recherche Scientifique (CNRS), Université de Lyon-Institut National des Sciences Appliquées (INSA)-Institut National des Sciences Appliquées (INSA)-Hospices Civils de Lyon (HCL)-Université Jean Monnet - Saint-Étienne (UJM)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Centre National de la Recherche Scientifique (CNRS)-Université Claude Bernard Lyon 1 (UCBL), Blank, Ulrich, Université Sorbonne Paris Cité - - USPC2011 - ANR-11-IDEX-0005 - IDEX - VALID, Institut National des Sciences Appliquées (INSA)-Université de Lyon-Institut National des Sciences Appliquées (INSA)-Hospices Civils de Lyon (HCL)-Université Jean Monnet [Saint-Étienne] (UJM)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Centre National de la Recherche Scientifique (CNRS)-Université Claude Bernard Lyon 1 (UCBL), Institut National des Sciences Appliquées (INSA)-Université de Lyon-Institut National des Sciences Appliquées (INSA)-Hospices Civils de Lyon (HCL)-Université Jean Monnet [Saint-Étienne] (UJM)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Centre National de la Recherche Scientifique (CNRS), Centre de recherche biomédicale Bichat-Beaujon (CRB3), Université Paris Diderot - Paris 7 (UPD7)-Institut National de la Santé et de la Recherche Médicale (INSERM), Pathologie pédiatrique (EA_3102), Hôpital Robert Debré-Université Paris Diderot - Paris 7 (UPD7)-IFR02, Immunopathologie rénale, récepteurs et inflammation, and Hôpital Robert Debré
Subjects: medicine.medical_specialty, kidney, 030232 urology & nephrology, Urology, Renal function, Scintigraphy, urologic and male genital diseases, [SDV.MHEP.UN]Life Sciences [q-bio]/Human health and pathology/Urology and Nephrology, 030218 nuclear medicine & medical imaging, Motion, 03 medical and health sciences, 0302 clinical medicine, hydronephrosis, Fibrosis, partial unilateral ureteral obstruction, Parenchyma, [INFO.INFO-IM]Computer Science [cs]/Medical Imaging, [SDV.MHEP.AHA]Life Sciences [q-bio]/Human health and pathology/Tissues and Organs [q-bio.TO], medicine, Animals, Radiology, Nuclear Medicine and imaging, Hydronephrosis, ComputingMilieux_MISCELLANEOUS, Spectroscopy, Intravoxel incoherent motion, intravoxel incoherent motion, Kidney, medicine.diagnostic_test, business.industry, diffusion, medicine.disease, Magnetic Resonance Imaging, [SDV.MHEP.UN] Life Sciences [q-bio]/Human health and pathology/Urology and Nephrology, 3. Good health, Mice, Inbred C57BL, Perfusion, medicine.anatomical_structure, Molecular Medicine, business, Ureteral Obstruction
Abstract: International audience; Ureteropelvic junction obstruction constitutes a major cause of progressive pediatric renal disease. The biological mechanisms underlying the renal response to obstruction can be investigated using a clinically relevant mouse model of partial unilateral ureteral obstruction (pUUO). Renal function and kidney morphology data can be evaluated using renal ultrasound, scintigraphy and uro-magnetic resonance imaging (uro-MRI), but these methods are poorly linked to histological change and not all are quantitative. Here, we propose to investigate pUUO for the first time using an intravoxel incoherent motion diffusion sequence. The aim of this study was to quantitatively characterize impairment of the kidney parenchyma in the pUUO model. This quantitative MRI method was able to assess the perfusion and microstructure of the kidney without requiring the injection of a contrast agent. The results suggest that a perfusion fraction (f) reduction is associated with a decrease in the volume of the renal parenchyma, which could be related to decreased renal vascularization. The latter may occur before impairment by fibrosis and the findings are in accordance with the literature using the UUO mice model and, more specifically, on pUUO. Further investigation is required before this technique can be made available for the diagnosis and management of children with antenatal hydronephrosis and to select the optimal timing of surgery if required.Copyright © 2017 John Wiley & Sons, Ltd.
Published: 2018
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11. Marfan Sartan: a randomized, double-blind, placebo-controlled trial

Author: H. Plauchu, Francois Sassolas, F. Arnoult, G. Delorme, Florence Tubach, Sophie Naudion, Guillaume Jondeau, Jacques Ropers, Olivier Milleron, Sylvie Odent, Martine Barthelet, Delphine Detaint, Philippe Aegerter, Nicolas Pangaud, Catherine Boileau, Thomas Edouard, Sophie Dupuis-Girod, Gilbert Habib, Jean-Eric Wolf, David Attias, Laurence Faivre, Adeline Basquin, Patrick Collignon, Yves Dulac, Julie Thomas-Chabaneix, Service de cardiologie, 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), Service d'explorations fonctionnelles, 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), Agence Française de Sécurité Sanitaire des Produits de Santé (AFSSAPS), AFSSAPS, Vieillissement et Maladies chroniques : approches épidémiologique et de santé publique (VIMA), Institut National de la Santé et de la Recherche Médicale (INSERM)-Université de Versailles Saint-Quentin-en-Yvelines (UVSQ), Département de santé publique, Hôpital Ambroise Paré [AP-HP], Consultation Marfan, Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Hôpital Bichat, Université Paris Diderot - Paris 7 (UPD7), CIC epidémiologie clinique/ essais cliniques, Hôpital Bichat - Claude Bernard, Institut National de la Santé et de la Recherche Médicale (INSERM), Service de Génétique, Hospices Civils de Lyon (HCL)-Hôpital Louis Pradel [CHU - HCL], Hospices Civils de Lyon (HCL)-Groupe Hospitalier Est, Hôtel Dieu, Service pédiatrie-cardiologie, CHU Toulouse [Toulouse]-Hôpital des Enfants, CHU Toulouse [Toulouse], Centre d'Endocrinologie, Maladies Osseuses, Génétique et Gynécologie Médicale, Hôpital des Enfants, CHU Toulouse [Toulouse]-CHU Toulouse [Toulouse], Service de Cardiologie [CHU de Dijon], Centre Hospitalier Universitaire de Dijon - Hôpital François Mitterrand (CHU Dijon), Centre de génétique - Centre de référence des maladies rares, anomalies du développement et syndromes malformatifs (CHU de Dijon), 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 cardiologie et maladies vasculaires [Rennes] = Cardiac, Thoracic, and Vascular Surgery [Rennes], CHU Pontchaillou [Rennes], Laboratoire Traitement du Signal et de l'Image (LTSI), Université de Rennes (UNIV-RENNES)-Université de Rennes (UNIV-RENNES)-Institut National de la Santé et de la Recherche Médicale (INSERM), Université de la Méditerranée - Aix-Marseille 2-Assistance Publique - Hôpitaux de Marseille (APHM)- Hôpital de la Timone [CHU - APHM] (TIMONE), Service de Génétique Médicale, Centre Hospitalier Intercommunal, Toulon, Laboratoire de Recherche Vasculaire Translationnelle (LVTS (UMR_S_1148 / U1148)), Université Paris 13 (UP13)-Université Paris Diderot - Paris 7 (UPD7)-Institut National de la Santé et de la Recherche Médicale (INSERM), Centre de référence MARFAN, Hôpital Bichat, Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Université Paris Diderot - Paris 7 (UPD7), Université de Versailles Saint-Quentin-en-Yvelines (UVSQ)-Institut National de la Santé et de la Recherche Médicale (INSERM), Service Cardiologie pédiatrique [CHU Toulouse], Pôle Enfants [CHU Toulouse], Centre Hospitalier Universitaire de Toulouse (CHU Toulouse)-Centre Hospitalier Universitaire de Toulouse (CHU Toulouse), Centre Hospitalier Universitaire de Toulouse (CHU Toulouse), 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 Rennes (UR)-Institut National de la Santé et de la Recherche Médicale (INSERM), Université Paris Diderot - Paris 7 (UPD7)-AP-HP - Hôpital Bichat - Claude Bernard [Paris]-Assistance publique - Hôpitaux de Paris (AP-HP) (APHP), Assistance publique - Hôpitaux de Paris (AP-HP) (APHP)-AP-HP - Hôpital Bichat - Claude Bernard [Paris], AP-HP, Hôpital Ambroise Paré, Assistance publique - Hôpitaux de Paris (AP-HP) (APHP)-Hôpital Bichat, Service de cardiologie et maladies vasculaires, Université de Rennes (UNIV-RENNES)-Université de Rennes (UNIV-RENNES)-Hôpital Pontchaillou-CHU Pontchaillou [Rennes], Dispositifs, diagnostics et thérapeutiques, and Hôpital Pontchaillou-CHU Pontchaillou [Rennes]-Institut National de la Santé et de la Recherche Médicale (INSERM)
Subjects: Adult, Male, Marfan syndrome, medicine.medical_specialty, Adolescent, [SDV]Life Sciences [q-bio], Adrenergic beta-Antagonists, Aortic Diseases, Placebo-controlled study, Blood Pressure, Placebo, Sudden death, Drug Administration Schedule, Losartan, Marfan Syndrome, Young Adult, Double-Blind Method, Heart Rate, medicine.artery, Internal medicine, Marfan, Humans, Medicine, Prospective Studies, Systole, Aorta, Aged, Sartan, business.industry, Middle Aged, medicine.disease, 3. Good health, Blood pressure, Echocardiography, Hypertension, cardiovascular system, Cardiology, Female, Cardiology and Cardiovascular Medicine, business, Angiotensin II Type 1 Receptor Blockers, Dilatation, Pathologic, medicine.drug
Abstract: AIMS: To evaluate the benefit of adding Losartan to baseline therapy in patients with Marfan syndrome (MFS). METHODS AND RESULTS: A double-blind, randomized, multi-centre, placebo-controlled, add on trial comparing Losartan (50 mg when \textless50 kg, 100 mg otherwise) vs. placebo in patients with MFS according to Ghent criteria, age \textgreater10 years old, and receiving standard therapy. 303 patients, mean age 29.9 years old, were randomized. The two groups were similar at baseline, 86% receiving β-blocker therapy. The median follow-up was 3.5 years. The evolution of aortic diameter at the level of the sinuses of Valsalva was not modified by the adjunction of Losartan, with a mean increase in aortic diameter at the level of the sinuses of Valsalva of 0.44 mm/year (s.e. = 0.07) (-0.043 z/year, s.e. = 0.04) in patients receiving Losartan and 0.51 mm/year (s.e. = 0.06) (-0.01 z/year, s.e. = 0.03) in those receiving placebo (P = 0.36 for the comparison on slopes in millimeter per year and P = 0.69 for the comparison on slopes on z-scores). Patients receiving Losartan had a slight but significant decrease in systolic and diastolic blood pressure throughout the study (5 mmHg). During the study period, aortic surgery was performed in 28 patients (15 Losartan, 13 placebo), death occurred in 3 patients [0 Losartan, 3 placebo, sudden death (1) suicide (1) oesophagus cancer (1)]. CONCLUSION: Losartan was able to decrease blood pressure in patients with MFS but not to limit aortic dilatation during a 3-year period in patients \textgreater10 years old. β-Blocker therapy alone should therefore remain the standard first line therapy in these patients
Published: 2015
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12. The Use of Oxytocin to Improve Feeding and Social Skills in Infants With Prader-Willi Syndrome

Author: Jeanne Pourrinet, Catie Cessans, Kader Boulanouar, Catherine Molinas, Sylvie Viaux-Sauvelon, Gwenaelle Diene, David Cohen, Maithé Tauber, Pierre Payoux, Sandy Faye, Sophie Çabal-Berthoumieu, Jean-Pierre Salles, Henri Martens, Virginie Ehlinger, Pascale Fichaux-Bourin, Françoise Muscatelli, Antoine Guedeney, Catherine Arnaud, Vincent Geenen, Patric J.D. Delhanty, Céline Bascoul, Marion Valette, Angèle Consoli, CHU Toulouse, Hôpital des Enfants, Unité de Gastroentérologie, Hépatologie et Nutrition, Département de Pédiatrie, Hôpital Purpan [Toulouse], CHU Toulouse [Toulouse]-CHU Toulouse [Toulouse], Institut des sciences du cerveau de Toulouse. (ISCT), Université Toulouse - Jean Jaurès (UT2J)-Université Toulouse III - Paul Sabatier (UT3), Université Fédérale Toulouse Midi-Pyrénées-Université Fédérale Toulouse Midi-Pyrénées-CHU Toulouse [Toulouse]-Institut National de la Santé et de la Recherche Médicale (INSERM)-Centre National de la Recherche Scientifique (CNRS), 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), CHU Toulouse [Toulouse], Centre de Référence du Syndrome de Prader-Willi, 161 Av. Churchill, Institut National de la Santé et de la Recherche Médicale (INSERM), Centre d'Endocrinologie, Maladies Osseuses, Génétique et Gynécologie Médicale, Hôpital des Enfants, and Internal Medicine
Subjects: Male, 0301 basic medicine, Pediatrics, medicine.medical_specialty, [SDV]Life Sciences [q-bio], Oxytocin, Drug Administration Schedule, Social Skills, 03 medical and health sciences, Animal data, 0302 clinical medicine, Swallowing, Social skills, medicine, Humans, Adverse effect, Administration, Intranasal, ComputingMilieux_MISCELLANEOUS, Dose-Response Relationship, Drug, business.industry, Infant, Feeding Behavior, Mother-Child Relations, Poor Feeding, Clinical trial, 030104 developmental biology, Child, Preschool, Sucking Behavior, Pediatrics, Perinatology and Child Health, Commentary, Female, Ghrelin, business, Prader-Willi Syndrome, 030217 neurology & neurosurgery, Follow-Up Studies, medicine.drug
Abstract: BACKGROUND AND OBJECTIVES: Patients with Prader–Willi syndrome (PWS) display poor feeding and social skills as infants and fewer hypothalamic oxytocin (OXT)-producing neurons were documented in adults. Animal data demonstrated that early treatment with OXT restores sucking after birth. Our aim is to reproduce these data in infants with PWS. METHODS: We conducted a phase 2 escalating dose study of a short course (7 days) of intranasal OXT administration. We enrolled 18 infants with PWS under 6 months old (6 infants in each step) who received 4 IU of OXT either every other day, daily, or twice daily. We investigated the tolerance and the effects on feeding and social skills and changes in circulating ghrelin and brain connectivity by functional MRI. RESULTS: No adverse events were reported. No dose effect was observed. Sucking assessed by the Neonatal Oral-Motor Scale was abnormal in all infants at baseline and normalized in 88% after treatment. The scores of Neonatal Oral-Motor Scale and videofluoroscopy of swallowing significantly decreased from 16 to 9 (P < .001) and from 18 to 12.5 (P < .001), respectively. Significant improvements in Clinical Global Impression scale scores, social withdrawal behavior, and mother–infant interactions were observed. We documented a significant increase in acylated ghrelin and connectivity of the right superior orbitofrontal network that correlated with changes in sucking and behavior. CONCLUSIONS: OXT is well tolerated in infants with PWS and improves feeding and social skills. These results open perspectives for early treatment in neurodevelopment diseases with feeding problems.
Published: 2017
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13. Characterization of fluorescent synthetic epicocconone-based dye through advanced light microscopies for live cell imaging applications

Author: Alexis Lebon, Stéphane Leleu, Ludovic Galas, Xavier Franck, Agathe Boulangé, Magalie Bénard, Thibault Gallavardin, Caroline Perraudeau, Damien Schapman, Chimie Organique et Bioorganique : Réactivité et Analyse (COBRA), Institut Normand de Chimie Moléculaire Médicinale et Macromoléculaire (INC3M), Institut de Chimie du CNRS (INC)-École Nationale Supérieure d'Ingénieurs de Caen (ENSICAEN), Normandie Université (NU)-Normandie Université (NU)-Institut national des sciences appliquées Rouen Normandie (INSA Rouen Normandie), Institut National des Sciences Appliquées (INSA)-Normandie Université (NU)-Institut National des Sciences Appliquées (INSA)-Université Le Havre Normandie (ULH), Normandie Université (NU)-Université de Rouen Normandie (UNIROUEN), Normandie Université (NU)-Centre National de la Recherche Scientifique (CNRS)-Université de Caen Normandie (UNICAEN), Normandie Université (NU)-Institut de Chimie du CNRS (INC)-École Nationale Supérieure d'Ingénieurs de Caen (ENSICAEN), Normandie Université (NU)-Centre National de la Recherche Scientifique (CNRS)-Institut de Chimie Organique Fine (IRCOF), Université de Rouen Normandie (UNIROUEN), Institut National des Sciences Appliquées (INSA)-Normandie Université (NU)-Institut National des Sciences Appliquées (INSA)-Centre National de la Recherche Scientifique (CNRS)-Centre National de la Recherche Scientifique (CNRS), Unité de Néonatalogie [Hôpital des enfants Toulouse], Hôpital des Enfants, CHU Toulouse [Toulouse]-CHU Toulouse [Toulouse], Institute for Research and Innovation in Biomedicine (IRIB), Normandie Université (NU)-Normandie Université (NU)-Centre National de la Recherche Scientifique (CNRS)-Institut National de la Santé et de la Recherche Médicale (INSERM), Lab Phys Collis Electron & Atom, Université de Brest (UBO), Neuroendocrinologie cellulaire et moléculaire, Normandie Université (NU)-Normandie Université (NU)-Institut National de la Santé et de la Recherche Médicale (INSERM), and Normandie Université (NU)-Normandie Université (NU)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Centre National de la Recherche Scientifique (CNRS)
Subjects: Dye, General Chemical Engineering, Analytical chemistry, Epicocconone, Organic synthesis, 02 engineering and technology, Fluorophore, 010402 general chemistry, 01 natural sciences, law.invention, Live cell imaging, Confocal microscopy, law, Microscopy, Organelle, [CHIM]Chemical Sciences, Cellular biology, Chemistry, Process Chemistry and Technology, Advanced light microscopies, 021001 nanoscience & nanotechnology, Fluorescence, 0104 chemical sciences, Membrane, Covalent bond, Biophysics, 0210 nano-technology
Abstract: International audience; Identification of new fluorescent biomarkers is a key issue to decipher mechanisms in living cells. Isolated from the fungus Epicoccum nigrum, natural epicocconone (NE) establishes a covalent and reversible link with primary amines and has been identified as a new fluorescent dye for cell imaging. In the present study, the properties of a synthetic and multifunctional analogue of epicocconone (SE) have been compared to those of NE through 1-photon (1P) and 2-photon (2P) advanced light microscopies. 1P Λλ confocal microscopy analyses revealed that, in cell culture medium, SE and NE displayed two peaks of excitation (ex) at 480 and 530 nm and a large band of emission (em) with a maximum at 610 nm. In living cells, SE presented sharper bands compared to NE with maxima at 545 nm (ex) and 605 nm (em). In 2P microscopy, SE and NE presented maxima around 790 nm (ex) and 595 nm (em) when diluted in cell culture medium. In living cells, SE did not present any large disturbing blue-shift for emission that was observed for NE. In addition, 2P bands for SE were sharper than the NE ones. SE was 2–3 times more fluorescent than NE as determined through 1P and 2P approaches. SE did not induce any cytotoxicity and was adapted for time-lapse acquisition. In PC12 cells, SE broadly labeled nuclear and plasma membranes, vesicles-like structures and organelles including the Golgi apparatus and mitochondria. Taken together, these data indicate that SE has appropriate properties for in vitro and in vivo cell biology studies and presents many advantages compared to NE for 1P and 2P microscopies.
Published: 2017
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14. Doxapram Dosing for Apnea of Prematurity Based on Postmenstrual Age and Gender: A Randomized Controlled Trial

Author: F. E. Haddad, M. Benard, Jean-Michel Hascoet, Isabelle Hamon, M.-J. Boutroy, Charlotte Casper, E. Greze, Développement, Adaptation et Handicap. Régulations cardio-respiratoires et de la motricité (DevAH), Université de Lorraine (UL), Unité de Néonatalogie [Hôpital des enfants Toulouse], Hôpital des Enfants, and CHU Toulouse [Toulouse]-CHU Toulouse [Toulouse]
Subjects: Male, Apnea, medicine.medical_treatment, Respiratory System Agents, Infant, Premature, Diseases, law.invention, 03 medical and health sciences, 0302 clinical medicine, Randomized controlled trial, Double-Blind Method, law, 030225 pediatrics, medicine, Humans, Pharmacology (medical), 030212 general & internal medicine, Dosing, Continuous positive airway pressure, Prospective Studies, Prospective cohort study, Apnea of prematurity, business.industry, Postmenstrual Age, Infant, Newborn, Infant, Doxapram, medicine.disease, respiratory tract diseases, 3. Good health, Anesthesia, Pediatrics, Perinatology and Child Health, Female, medicine.symptom, business, Infant, Premature, [SDV.MHEP]Life Sciences [q-bio]/Human health and pathology, medicine.drug
Abstract: International audience; IntroductionDoxapram is used as a third-line treatment for apnea unresponsive to caffeine and continuous positive airway pressure (CPAP) in preterm infants.ObjectivesThe objectives of this study were to compare the effects of dosing adjusted for gender and postmenstrual age (PMA) (GrA) versus infants’ weight alone (GrW) on doxapram plasma levels, clinical efficacy, and side effects.MethodsThis was a randomized, double-blind study, including premature infants for whom optimized caffeine and CPAP therapy for apnea of prematurity had failed. Failure was defined as the persistence of more than one significant apnea per hour over an 8-h period. Plasma levels of doxapram and ketodoxapram were measured with high-performance liquid chromatography (HPLC) 48 h after the onset of treatment. Dosing aimed to maintain the combined doxapram and ketodoxapram plasma level in the therapeutic range of 1.5–4 mg/l. Infants were followed-up for 4 days after the onset of treatment.ResultsA total of 85 infants were included: 46 in GrW (27.7 ± 1.9 weeks’ gestational age [GA]), 39 in GrA (27.9 ± 1.4 weeks’ GA); available plasma levels showed that 25 of 40 in the GrW group and 27 of 37 in the GrA group had levels within the therapeutic range (p = 0.344). Of note, plasma level variance was significantly higher in GrW for doxapram + ketodoxapram (1.87 vs. 0.89; p = 0.028). Clinical efficacy was better in the GrA group, with a reduction from 32 to 3 of 38 (76 %) infants with significant apnea versus 30 to 5 of 45 (56 %) in the GrW group (p < 0.001). No adverse effects were observed during the study.ConclusionsTaking gender and PMA into account for doxapram dosing did not significantly increase the number of infants with a plasma level in the therapeutic range. However, it improved plasma level stability and clinical efficacy without adverse effects.
Published: 2016
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15. High third-generation cephalosporin resistant Enterobacteriaceae prevalence rate among neonatal infections in Dakar, Senegal

Author: Olivier Moquet, Benoit Garin, Haby Signate Sy, M. Cisse, Sidy Ka, Helene Salord, Jean-Marie Sire, Raymond Bercion, R. Michel, Philippe Glaser, Sébastien Breurec, Abdoulaye Seck, Ousmane Ndiaye, Coralie Bouchiat, Institut Pasteur de la Guadeloupe, Réseau International des Instituts Pasteur (RIIP), Université des Antilles et de la Guyane (UAG), Institut Pasteur de Dakar, Institut Pasteur de Bangui, Hôpital des Enfants Albert Royer, Laboratoire de Bactériologie, Hôpital des Enfants Albert Royer, Laboratoire de Bactériologie, Avenue Cheikh Anta Diop, Dakar, Senegal., Biologie des Bactéries Pathogènes à Gram-positif, Institut Pasteur [Paris] (IP)-Centre National de la Recherche Scientifique (CNRS), Université Cheikh Anta Diop [Dakar, Sénégal] (UCAD), Département de Pédiatrie, Hôpital Principal de Dakar, Laboratoire de Microbiologie Croix-Rousse, Hôpital de la Croix-Rousse [CHU - HCL], Hospices Civils de Lyon (HCL)-Hospices Civils de Lyon (HCL), Hôpital des Enfants Albert Royer, Unité d’Epidémiologie, Réseau International des Instituts Pasteur (RIIP)-Réseau International des Instituts Pasteur (RIIP), and Institut Pasteur [Paris]-Centre National de la Recherche Scientifique (CNRS)
Subjects: Male, 0301 basic medicine, CTX-M-15, Klebsiella pneumoniae, 030106 microbiology, Prevalence, Microbial Sensitivity Tests, Antimicrobial resistance, beta-Lactam Resistance, beta-Lactamases, lcsh:Infectious and parasitic diseases, Microbiology, Tertiary Care Centers, 03 medical and health sciences, 0302 clinical medicine, Antibiotic resistance, Enterobacteriaceae, [SDV.MHEP.MI]Life Sciences [q-bio]/Human health and pathology/Infectious diseases, 030225 pediatrics, Intensive care, Enterobacter cloacae, Case fatality rate, Humans, Medicine, lcsh:RC109-216, biology, business.industry, Mortality rate, Enterobacteriaceae Infections, Infant, Newborn, biology.organism_classification, [SDV.MP.BAC]Life Sciences [q-bio]/Microbiology and Parasitology/Bacteriology, Senegal, Cephalosporins, 3. Good health, Neonatal infection, Treatment Outcome, Infectious Diseases, Africa, Third-generation cephalosporin-resistant Enterobacteriaceae, Neonatal infections, Female, business, Research Article
Abstract: International audience; Background: Neonatal infection constitutes one of Senegal’s most important public health problems, with amortality rate of 41 deaths per 1,000 live births.Methods: Between January 2007 and March 2008, 242 neonates with suspected infection were recruited at threeneonatal intensive care units in three major tertiary care centers in Dakar, the capital of Senegal. Neonatal infections wereconfirmed by positive bacterial blood or cerebrospinal fluid culture. The microbiological pattern of neonatal infectionsand the antibiotic susceptibility of the isolates were characterized. In addition, the genetic basis for antibiotic resistanceand the genetic background of third-generation cephalosporin-resistant (3GC-R) Enterobacteriaceae were studied.Results: A bacteriological infection was confirmed in 36.4 % (88/242) of neonates: 22.7 % (30/132) during the early-onsetand 52.7 % (58/110) during the late-onset periods (p > 0.20). Group B streptococci accounted for 6.8 % of the 88 collectedbacterial isolates, while most of them were Enterobacteriaceae (n = 69, 78.4 %). Of these, 55/69 (79.7 %) were 3GC-R. TheblaCTX-M-15 allele, the blaSHV and the blaTEM were highly prevalent (63.5, 65.4 and 53.8 %, respectively), usually associatedwith qnr genes (65.4 %). Clonally related strains of 3GC-R Klebsiella pneumoniae and 3GC-R Enterobacter cloacae, the twomost commonly recovered 3GC-R Enterobacteriaceae (48/55), were detected at the three hospitals, underlining the roleof cross-transmission in their spread. The overall case fatality rate was 18.6 %.Conclusions:Measures should be taken to prevent nosocomial infections and the selection of resistant bacteria
Published: 2016
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16. Recombinant Bile Salt-Stimulated Lipase in Preterm Infant Feeding: A Randomized Phase 3 Study

Author: Ingrid Palmgren, María L. Couce, Jacques Rigo, Tibor Ertl, Charlotte Casper, Alexandre Lapillonne, Jean-Michel Hascoet, Janusz Gadzinowski, Mårten Vagerö, Kristina Timdahl, Virgilio P. Carnielli, Olle Hernell, Unité de Néonatalogie [Hôpital des enfants Toulouse], Hôpital des Enfants, CHU Toulouse [Toulouse]-CHU Toulouse [Toulouse], Service de Néonatalogie, Centre Hospitalier Régional Universitaire de Nancy (CHRU Nancy), University of Pecs, Poznan University of Medical Sciences [Poland] (PUMS), Polytechnic University of Marche — Ospedali Riuniti Ancona [Italy], Université de Liège, CHU Necker - Enfants Malades [AP-HP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP), Universidade de Santiago de Compostela [Spain] (USC ), Swedish Orphan Biovitrum (SOBI), Umeå University, Service de Médecine Néonatale [CHRU Nancy], Développement, Adaptation et Handicap. Régulations cardio-respiratoires et de la motricité. (DevAH), Université de Lorraine (UL), Poznan University of Medical Sciences, Fédération pour la recherche en explorations et thérapeutiques innovantes in utéro (FETUS - EA 7328), Université Paris Descartes - Paris 5 (UPD5), Hospital Clínico Universitario de Santiago, and Complejo Hospitalario Universitario de Santiago de Compostela [Saint-Jacques-de-Compostelle, Espagne] (CHUS)
Subjects: Male, Physiology, Hydrolases, lcsh:Medicine, Drug research and development, Weight Gain, Pediatrics, Biochemistry, Fats, Placebos, Families, 0302 clinical medicine, Clinical trials, Sterol esterase, Medicine and Health Sciences, Bile, Birth Weight, 030212 general & internal medicine, Lipases, lcsh:Science, Children, Breast Milk, 2. Zero hunger, Multidisciplinary, biology, Pediatrik, Lipids, Infant Formula, Recombinant Proteins, 3. Good health, Body Fluids, Enzymes, Milk, Physiological Parameters, Research Design, Pasteurization, Female, medicine.symptom, Anatomy, Digestion, Infants, Phase II clinical investigation, Infant, Premature, Research Article, medicine.medical_specialty, Clinical Research Design, Triacylglycerol lipase, Gestational Age, Breast milk, Research and Analysis Methods, 03 medical and health sciences, Enteral Nutrition, Double-Blind Method, 030225 pediatrics, Internal medicine, medicine, Humans, Lipase, Pharmacology, [SDV.MHEP.PED]Life Sciences [q-bio]/Human health and pathology/Pediatrics, Milk, Human, business.industry, Body Weight, lcsh:R, Infant, Newborn, Biology and Life Sciences, Proteins, Infant, Sterol Esterase, Bottle Feeding, Endocrinology, Parenteral nutrition, Infant formula, Age Groups, Clinical medicine, People and Places, Dietary Supplements, biology.protein, Enzymology, Population Groupings, lcsh:Q, Adverse Events, business, Weight gain, [SDV.AEN]Life Sciences [q-bio]/Food and Nutrition
Abstract: INTRODUCTION:Feeding strategies are critical for healthy growth in preterm infants. Bile salt-stimulated lipase (BSSL), present in human milk, is important for fat digestion and absorption but is inactivated during pasteurization and absent in formula. This study evaluated if recombinant human BSSL (rhBSSL) improves growth in preterm infants when added to formula or pasteurized breast milk. PATIENTS AND METHODS:LAIF (Lipase Added to Infant Feeding) was a randomized, double-blind, placebo-controlled phase 3 study in infants born before 32 weeks of gestation. The primary efficacy variable was growth velocity (g/kg/day) during 4 weeks intervention. Follow-up visits were at 3 and 12 months. The study was performed at 54 centers in 10 European countries. RESULTS:In total 415 patients were randomized (rhBSSL n = 207, placebo n = 208), 410 patients were analyzed (rhBSSL n = 206, placebo n = 204) and 365 patients were followed until 12 months. Overall, there was no significantly improved growth velocity during rhBSSL treatment compared to placebo (16.77 vs. 16.56 g/kg/day, estimated difference 0.21 g/kg/day, 95% CI [-0.40; 0.83]), nor were secondary endpoints met. However, in a predefined subgroup, small for gestational age infants, there was a significant effect on growth in favor of rhBSSL during treatment. The incidence of adverse events was higher in the rhBSSL group during treatment. CONCLUSIONS:Although this study did not meet its primary endpoint, except in a subgroup of infants small for gestational age, and there was an imbalance in short-term safety, these data provide insights in nutrition, growth and development in preterm infants. TRIAL REGISTRATION:ClinicalTrials.gov NCT01413581.
Published: 2016
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17. 0362 : Marfan syndrome diagnosed during childhood: focus on cardiac events in the French database

Author: Bruno Leheup, Jean Ferrière, Guillaume Jondeau, Thomas Edouard, Sébastien Hascoët, Nicole Philip, Bertrand Chevallier, Olivier Milleron, Sylvie Odent, Yves Dulac, Chantal Stheneur, Laurence Olivier-Faivre, Sophie Dupuis-Girod, Philippe Acar, F. Arnoult, Cécile Zordan, Nathalie Blot-Souletie, Service Cardiologie pédiatrique [CHU Toulouse], Pôle Enfants [CHU Toulouse], Centre Hospitalier Universitaire de Toulouse (CHU Toulouse)-Centre Hospitalier Universitaire de Toulouse (CHU Toulouse), Centre d'Endocrinologie, Maladies Osseuses, Génétique et Gynécologie Médicale, Centre Hospitalier Universitaire de Toulouse (CHU Toulouse), Service de Génétique, Hospices Civils de Lyon (HCL)-Hôpital Louis Pradel [CHU - HCL], Hospices Civils de Lyon (HCL)-Groupe Hospitalier Est, Service de cardiologie, 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), Service d'explorations fonctionnelles, Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP), Department of Pediatrics, Service de Médecine Infantile III et Génétique Clinique [CHRU Nancy], Centre Hospitalier Régional Universitaire de Nancy (CHRU Nancy), Consultation Marfan, Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Hôpital Bichat, Service de pédiatrie, urgences enfants [CHU Ambroise-Paré], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Hôpital Ambroise Paré [AP-HP], Assistance Publique - Hôpitaux de Marseille (APHM)- Hôpital de la Timone [CHU - APHM] (TIMONE), 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 ), Centre de référence MARFAN, Hôpital Bichat, Service pédiatrie-cardiologie, CHU Toulouse [Toulouse]-Hôpital des Enfants, CHU Toulouse [Toulouse], Hôpital des Enfants, CHU Toulouse [Toulouse]-CHU Toulouse [Toulouse], Université Paris Diderot - Paris 7 (UPD7)-AP-HP - Hôpital Bichat - Claude Bernard [Paris]-Assistance publique - Hôpitaux de Paris (AP-HP) (APHP), Assistance publique - Hôpitaux de Paris (AP-HP) (APHP)-AP-HP - Hôpital Bichat - Claude Bernard [Paris], Assistance publique - Hôpitaux de Paris (AP-HP) (APHP), Assistance publique - Hôpitaux de Paris (AP-HP) (APHP)-Hôpital Bichat, Service de pédiatrie, urgences enfants, Université de Versailles Saint-Quentin-en-Yvelines (UVSQ)-Assistance publique - Hôpitaux de Paris (AP-HP) (APHP)-Hôpital Ambroise Paré, 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 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), Hôpital Ambroise Paré [AP-HP]-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP), 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), and Université de Rennes (UNIV-RENNES)-Université de Rennes (UNIV-RENNES)
Subjects: Marfan syndrome, Aortic dissection, medicine.medical_specialty, Aorta, business.industry, Incidence (epidemiology), [SDV]Life Sciences [q-bio], Bentall procedure, Mechanical Aortic Valve, 030204 cardiovascular system & hematology, medicine.disease, Surgery, 03 medical and health sciences, Dissection, 0302 clinical medicine, Interquartile range, medicine.artery, medicine, 030212 general & internal medicine, business, Cardiology and Cardiovascular Medicine
Abstract: International audience; Objectives Life expectancy of patients with Marfan syndrome has increased, due to earlier diagnosis, better familial screening, regular follow-up (FU) and prophylactic aortic surgery (PASu). Incidence of events in affected patients recognized during childhood is unknown. Methods 465 patients with Marfan syndrome, diagnosed before 18y.o. were included in the French multicenter database. Cardio-vascular events (death, aortic dissection or PASu) were recorded Results A cardio-vascular event occurred in 25 patients (5.4% 95CI 3.5- 7.8%), including PASu (n=20, 4.3% 95CI 2.5-6.2%), aortic dissection (n=3, 0.6% 95CI 0.0-1.4%) and deaths (n=2, 0.4% 95CI 0.0-1.0%). 16 events (64%) occurred before 19 year-old (Median 15.0, min 2.8, interquartile 11.7-16.3; PASu n=12, deaths n=2 and dissection n= 2). An aortic surgery was performed in 23 patients (4.9%, 95CI 3.0-6.9%), including a Bentall procedure with mechanical aortic valve in 10 (43.5%), a valve sparing surgery in the remaining 13 (56.5%) and a supra-coronary graft in 4 (17.4%, dissection: n=2 and PASu: n=2). Mean age at the date of PASu was 17.1±6.5 year-old. Events occurred before or at inclusion in the database in 8 patients (32.0%) (PASu n=5, dissection n=2, death n=1). Dissection was observed before inclusion in 2 patients and during pregnancy in 1 patient. Kaplan-Meier survival estimate indicates that 95% of patients remained free from events at eighteen and 78% at thirty year-old. Conclusion Prophylactic surgery for enlarged aorta is the main cause of cardiac events in patients with Marfan syndrome diagnosed during childhood. A quarter of them have a cardiac event before thirty year-old.
Published: 2015
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18. L’alliance thérapeutique est associée à l’adhésion au traitement chez les enfants atteints d’arthrite juvénile idiopathique

Author: Valérie Devauchelle-Pensec, Anaïs Arbault, Elisabeth Solau-Gervais, Irène Lemelle, L. Sparsa, Agnès Duquesne, Tu-Anh Tran, Anne Lohse, Camille Alleyrat, Francis Guillemin, Pascal Pillet, Linda Rossi, Laurence Goumy, Claire Ballot, Héloïse Reumaux, Centre Hospitalier de Belfort (Service de Rhumatologie), CH Belfort-Montbéliard, Service d'Onco-Hématologie Pédiatrique, Centre Hospitalier Universitaire de Nancy, hôpital des Enfants [CHU Bordeaux], Pédiatrie hospices civils de Lyon, hôpital Femme-Mère-Enfant, Service de pédiatrie [CHRU Besançon], Centre Hospitalier Régional Universitaire de Besançon (CHRU Besançon), Service d'Hématologie Pédiatrique, CHU Nîmes, France, Centre Hospitalier de Mulhouse (Service de Rhumatologie), 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 Pédiatrie Générale [CHU Angers], Centre Hospitalier Universitaire d'Angers (CHU Angers), PRES Université Nantes Angers Le Mans (UNAM)-PRES Université Nantes Angers Le Mans (UNAM), Service de Rhumatologie Pédiatrique, Centre Universitaire de Lille, Pediatric Rheumatology [Le Kremlin-Bicêtre], Centre de Référence des Maladies Auto-Inflammatoires et des Amyloses [Le Kremlin-Bicêtre] (CeRéMAIA), Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Hôpital Bicêtre-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Hôpital Bicêtre, Service de rhumatologie [Poitiers], Centre hospitalier universitaire de Poitiers (CHU Poitiers), Service de Rhumatologie (CHU de Dijon), Centre Hospitalier Universitaire de Dijon - Hôpital François Mitterrand (CHU Dijon), Centre d'investigation clinique - Epidémiologie clinique [Nancy] (CIC-EC), Centre d'investigation clinique [Nancy] (CIC), Centre Hospitalier Régional Universitaire de Nancy (CHRU Nancy)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Université de Lorraine (UL)-Centre Hospitalier Régional Universitaire de Nancy (CHRU Nancy)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Université de Lorraine (UL), CHRU Brest - Service de Rhumatologie (CHU - BREST - Rhumato), Centre Hospitalier Régional Universitaire de Brest (CHRU Brest), Lymphocyte B et Auto-immunité (LBAI), Université de Brest (UBO)-Institut Brestois Santé Agro Matière (IBSAM), and Université de Brest (UBO)-Institut National de la Santé et de la Recherche Médicale (INSERM)
Subjects: 030203 arthritis & rheumatology, 03 medical and health sciences, 0302 clinical medicine, Rheumatology, [SDV.IMM]Life Sciences [q-bio]/Immunology, 030212 general & internal medicine, ComputingMilieux_MISCELLANEOUS
Abstract: Objectifs L’alliance therapeutique (AT) se definit comme l’engagement mutuel entre le soignant et le patient au cours du processus therapeutique. L’adhesion therapeutique est un enjeu majeur dans la prise en charge de l’arthrite juvenile idiopathique (AJI) et repose sur la forte capacite de l’enfant a suivre les traitements. Cette etude avait pour objectif d’evaluer l’association entre l’AT et l’adhesion therapeutique chez des patients atteints d’AJI. Methodes Etude observationnelle, transversale, multicentrique. Des enfants atteints d’AJI, âges de 8 a 16 ans ont ete inclus. Les enfants, leurs parents et les medecins ont complete le Helping Alliance Questionnaire (HAq-CP) pour mesurer l’AT. L’observance aux traitements a ete mesuree a l’aide du Child/Parent Adherence Report Questionnaire (CARQ & PARQ). Les donnees demographiques, les caracteristiques de la maladie, les traitements en cours et l’environnement social ont ete recueillis. La relation univariee entre l’AT et l’adhesion a ete mesuree a l’aide du coefficient de correlation de Pearson. L’analyse multivariee a utilise un modele de regression lineaire multiple. Resultats 119 patients ont ete inclus : 68,9 % de filles, l’âge moyen (ET) etait de 12,4 (2,9) ans, avec une duree de la maladie de 73,1 (48,2) mois. L’AJI etait en remission (52 %), de faible activite (32 %) et active (16 %). Les scores de l’AT etaient eleves (≥ 80/100) chez les enfants, les parents et les medecins. Le HAQ-CP a montre une forte correlation avec le CARQ (r = 0,31 ; p Conclusion L’AT exerce une forte influence sur l’adhesion therapeutique et pourrait jouer un role majeur dans l’efficacite du traitement.
Published: 2022
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19. Childhood acute lymphoblastic leukaemia and indicators of early immune stimulation: the Estelle study (SFCE)

Author: Anne Lambilliotte, Yves Bertrand, J. Rudant, R. Ajrouche, Catherine Paillard, Virginie Gandemer, André Baruchel, Arnaud Petit, Nicolas Blin, Laure Saumet, Denis Hémon, Marion Gambart, Laurent Orsi, Jacqueline Clavel, Stéphane Ducassou, G. Michel, Lecoupe-Grainville, Marie, Université Paris-Sud - Paris 11 (UP11), Université Paris Descartes - Paris 5 (UPD5), Université Pierre et Marie Curie - Paris 6 (UPMC), CHU Trousseau [APHP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU), Service d'hématologie et immunologie pédiatrique, Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Hôpital Robert Debré-Université Paris Diderot - Paris 7 (UPD7), Service d'hématologie pédiatrique-oncologie, Hôpital Jeanne de Flandre [Lille]-Centre Hospitalier Régional Universitaire [Lille] (CHRU Lille), Centre Hospitalier Universitaire de Toulouse (CHU Toulouse), Assistance Publique - Hôpitaux de Marseille (APHM), Institut d'hématologie et d'oncologie pédiatrique [CHU - HCL] (IHOPe), Hospices Civils de Lyon (HCL), CHU de Bordeaux Pellegrin [Bordeaux], 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 de Hautepierre [Strasbourg], Hôpital Arnaud de Villeneuve [CHRU Montpellier], Centre Hospitalier Régional Universitaire [Montpellier] (CHRU Montpellier), Hôpital Mère-Enfant, CHU de Nantes, This work was supported by grants from the Ligue Nationale Contre le Cancer (LNCC), the PNREST Anses, the Cancer TMOI AVIESAN, 2013/1/248, the Institut National du Cancer (INCa), and the association Enfants et sante., Université Paris-Sud - Paris 11 ( UP11 ), Université Paris Descartes - Paris 5 ( UPD5 ), Université Pierre et Marie Curie - Paris 6 ( UPMC ), Assistance publique - Hôpitaux de Paris (AP-HP)-Hôpital Robert Debré-Université Paris Diderot - Paris 7 ( UPD7 ), Hôpital Jeanne de Flandre [Lille]-Centre Hospitalier Régional Universitaire [Lille] ( CHRU Lille ), Hôpital des enfants Toulouse, Assistance Publique - Hôpitaux de Marseille ( APHM ), Institut d'hématologie et d'oncologie pédiatrique [CHU - HCL] ( IHOPe ), Hospices Civils de Lyon ( HCL ), 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 ), CHRU, Hopital A. de Villeneuve, Montpellier, Université Paris Diderot - Paris 7 (UPD7)-Hôpital Robert Debré-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP), Hôpital des Enfants, CHU Toulouse [Toulouse], 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), and Université de Rennes (UNIV-RENNES)-Université de Rennes (UNIV-RENNES)
Subjects: Male, Cancer Research, Pediatrics, medicine.medical_treatment, Breastfeeding, [ SDV.CAN ] Life Sciences [q-bio]/Cancer, 0302 clinical medicine, Risk Factors, Surveys and Questionnaires, Medicine, 030212 general & internal medicine, Registries, infections, Child, education.field_of_study, Confounding, Precursor Cell Lymphoblastic Leukemia-Lymphoma, day-care, 3. Good health, animals, Oncology, breast feeding, 030220 oncology & carcinogenesis, Child, Preschool, Female, France, pets, Adult, Risk, medicine.medical_specialty, Adolescent, Population, Mothers, [SDV.CAN]Life Sciences [q-bio]/Cancer, 03 medical and health sciences, childhood leukaemia, [SDV.CAN] Life Sciences [q-bio]/Cancer, Humans, Caesarean section, education, business.industry, Case-control study, Infant, Odds ratio, Child Day Care Centers, Confidence interval, Case-Control Studies, caesarean section, Clinical Study, business, Breast feeding
Abstract: International audience; Background: Factors related to early stimulation of the immune system (breastfeeding, proxies for exposure to infectious agents, normal delivery, and exposure to animals in early life) have been suggested to decrease the risk of childhood acute lymphoblastic leukaemia (ALL). Methods: The national registry-based case-control study, ESTELLE, was carried out in France in 2010-2011. Population controls were frequency matched with cases on age and gender. The participation rates were 93% for cases and 86% for controls. Data were obtained from structured telephone questionnaires administered to mothers. Odds ratios (OR) were estimated using unconditional regression models adjusted for age, gender, and potential confounders. Results: In all, 617 ALL and 1225 controls aged >= 1 year were included. Inverse associations between ALL and early common infections (OR = 0.8, 95% confidence interval (CI): 0.6, 1.0), non-first born (>= 3 vs 1; OR = 0.7, 95% CI: 0.5, 1.0), attendance of a day-care centre before age 1 year (OR = 0.7, 95% CI: 0.5, 1.0), breastfeeding (OR = 0.8, 95% CI: 0.7, 1.0), and regular contact with pets (OR = 0.8, 95% CI: 0.7, 1.0) in infancy were observed. Conclusions: The results support the hypothesis that conditions promoting the maturation of the immune system in infancy have a protective role with respect to ALL.
Published: 2015
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20. Long-term side effects of radiotherapy for pediatric localized neuroblastoma

Author: Ducassou , Anne, Gambart , Marion, Munzer , Caroline, Padovani , Laetitia, Carrie , Christian, Haas-Kogan , Daphne, Bernier-Chastagner , Valérie, Demoor , Charlotte, Claude , Line, Helfre , Sylvie, Bolle , Stéphanie, Leseur , Julie, Huchet , Aymeri, Rubié , Hervé, Valteau-Couanet , Dominique, Schleiermacher , Gudrun, Coze , Carole, Defachelles , Anne-Sophie, Marabelle , Aurelien, Ducassou , Stéphane, Devalck , Christine, Gandemer , Virginie, Munzer , Martine, Laprie , Anne, Centre Hospitalier Universitaire de Toulouse (CHU Toulouse), Centre de Recherches en Oncologie biologique et Oncopharmacologie (CRO2), Aix Marseille Université (AMU)- Hôpital de la Timone [CHU - APHM] (TIMONE)-Institut National de la Santé et de la Recherche Médicale (INSERM), Service de Radiothérapie, Assistance Publique - Hôpitaux de Marseille (APHM)- Hôpital de la Timone [CHU - APHM] (TIMONE), Centre Léon Bérard [Lyon], Dept. of Radiation Oncology, UCSF, University of California [San Francisco] (UC San Francisco), University of California (UC)-University of California (UC), Institut de Cancérologie de Lorraine - Alexis Vautrin [Nancy] (UNICANCER/ICL), UNICANCER, CRLCC Eugène Marquis (CRLCC), CHU Bordeaux [Bordeaux], Sercice Hématologie, immunologie et oncologie pédiatrique [CHU Toulouse], Pôle Enfants [CHU Toulouse], Centre Hospitalier Universitaire de Toulouse (CHU Toulouse)-Centre Hospitalier Universitaire de Toulouse (CHU Toulouse), Département de cancérologie de l'enfant et de l'adolescent [Gustave Roussy], Institut Gustave Roussy (IGR), Unité de génétique et biologie des cancers (U830), Université Paris Descartes - Paris 5 (UPD5)-Institut Curie [Paris]-Institut National de la Santé et de la Recherche Médicale (INSERM), Hôpital de la Timone [CHU - APHM] (TIMONE), Institut de Génétique et Développement de Rennes (IGDR), Université de Rennes (UR)-Centre National de la Recherche Scientifique (CNRS), Hématogoie pédiatrique, Institut Jean Godinot [Reims], Imagerie cérébrale et handicaps neurologiques (ICHN), Institut des sciences du cerveau de Toulouse. (ISCT), Université Toulouse - Jean Jaurès (UT2J), Université de Toulouse (UT)-Université de Toulouse (UT)-Université Toulouse III - Paul Sabatier (UT3), Université de Toulouse (UT)-Centre Hospitalier Universitaire de Toulouse (CHU Toulouse)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Centre National de la Recherche Scientifique (CNRS)-Université Toulouse - Jean Jaurès (UT2J), Université de Toulouse (UT)-Centre Hospitalier Universitaire de Toulouse (CHU Toulouse)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Centre National de la Recherche Scientifique (CNRS)-Institut National de la Santé et de la Recherche Médicale (INSERM), Hôpital des Enfants, CHU Toulouse [Toulouse], University of California [San Francisco] (UCSF), University of California-University of California, Institut de Cancérologie de Lorraine - Alexis Vautrin (ICL), Unité d'Hémato-Oncologie, Hôpital des Enfants, Université Paris Descartes - Paris 5 (UPD5)-Institut Curie-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)-IFR140-Centre National de la Recherche Scientifique (CNRS), Institut Jean Godinot, CRLCC Jean Godinot, Imagerie cérébrale et handicaps neurologiques, Université Toulouse - Jean Jaurès (UT2J)-Université Toulouse III - Paul Sabatier (UT3), Université Fédérale Toulouse Midi-Pyrénées-Université Fédérale Toulouse Midi-Pyrénées-CHU Toulouse [Toulouse]-Institut National de la Santé et de la Recherche Médicale (INSERM)-Centre National de la Recherche Scientifique (CNRS)-Université Toulouse - Jean Jaurès (UT2J)-Université Toulouse III - Paul Sabatier (UT3), Université Fédérale Toulouse Midi-Pyrénées-Université Fédérale Toulouse Midi-Pyrénées-CHU Toulouse [Toulouse]-Institut National de la Santé et de la Recherche Médicale (INSERM)-Centre National de la Recherche Scientifique (CNRS)-Institut National de la Santé et de la Recherche Médicale (INSERM), Centre National de la Recherche Scientifique (CNRS)-Université de Rennes 1 (UR1), Université de Rennes (UNIV-RENNES)-Université de Rennes (UNIV-RENNES), Institut Curie [Paris]-Institut National de la Santé et de la Recherche Médicale (INSERM)-Université Paris Descartes - Paris 5 (UPD5), Hôpital des enfants, Centre de Recherches en Oncologie biologique et Oncopharmacologie ( CRO2 ), Aix Marseille Université ( AMU ) - Hôpital de la Timone [CHU - APHM] ( TIMONE ) -Institut National de la Santé et de la Recherche Médicale ( INSERM ), Assistance Publique - Hôpitaux de Marseille ( APHM ) - Hôpital de la Timone [CHU - APHM] ( TIMONE ), University of California [San Francisco] ( UCSF ), Institut de Cancérologie de Lorraine - Alexis Vautrin ( ICL ), CRLCC Eugène Marquis ( CRLCC ), Institut Gustave Roussy ( IGR ), Unité de génétique et biologie des cancers ( U830 ), Université Paris Descartes - Paris 5 ( UPD5 ) -Institut Curie-Institut National de la Santé et de la Recherche Médicale ( INSERM ), Hôpital de la Timone [CHU - APHM] ( TIMONE ), Institut de Génétique et Développement de Rennes ( IGDR ), Université de Rennes 1 ( UR1 ), Université de Rennes ( UNIV-RENNES ) -Université de Rennes ( UNIV-RENNES ) -IFR140-Centre National de la Recherche Scientifique ( CNRS ), Université Toulouse III - Paul Sabatier ( UPS ), and Université Fédérale Toulouse Midi-Pyrénées-Université Fédérale Toulouse Midi-Pyrénées-Institut National de la Santé et de la Recherche Médicale ( INSERM )
Subjects: [ SDV ] Life Sciences [q-bio], Radiotherapy, Neoplasien, Late effects, [SDV]Life Sciences [q-bio], Musculoskeletal abnormalities, Skoliose, Strahlentherapie, Radiation therapy, second primary, Scoliosis, Oncology, Spätfolgen, Neoplasms, Muskel-Skelett-Anomalien, zweite primäre, ComputingMilieux_MISCELLANEOUS
Abstract: International audience; Introduction - Neuroblastoma (NB) is the most frequent indication for extracranial pediatric radiotherapy. As long-term survival of high-risk localized NB has greatly improved, we reviewed treatment-related late toxicities in pediatric patients who received postoperative radiotherapy (RT) for localized NB within two French prospective clinical trials: NB90 and NB94. Patients and methods - From 1990-2000, 610 children were enrolled. Among these, 35 were treated with induction chemotherapy, surgery, and RT. The recommended RT dose was 24 Gy at ≤ 2 years, 34 Gy at > 2 years, ± a 5 Gy boost in both age groups. Results - The 22 patients still alive after 5 years were analyzed. The median follow-up time was 14 years (range 5-21 years). Late effects after therapy occurred in 73 % of patients (16/22), within the RT field for 50 % (11/22). The most frequent in-field effects were musculoskeletal abnormalities (n = 7) that occurred only with doses > 31 Gy/1.5 Gy fraction (p = 0.037). Other effects were endocrine in 3 patients and second malignancies in 2 patients. Four patients presented with multiple in-field late effects only with doses > 31 Gy. Conclusion - After a median follow-up of 14 years, late effects with multimodality treatment were frequent. The most frequent effects were musculoskeletal abnormalities and the threshold for their occurrence was 31 Gy.
Published: 2015
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21. Pharmacokinetics of mycophenolate mofetil in children with lupus and clinical findings in favour of therapeutic drug monitoring

Author: Woillard , Jean-Baptiste, Bader-Meunier , Brigitte, Salomon , Rémi, Ranchin , Bruno, Decramer , Stéphane, Fischbach , Michel, Berard , Etienne, Guigonis , Vincent, Harambat , Jérôme, Dunand , Olivier, Tenenbaum , Julie, Marquet , Pierre, Saint-Marcoux , Franck, Pharmacologie des Immunosuppresseurs et de la Transplantation (PIST), Université de Limoges (UNILIM)-CHU Limoges-Génomique, Environnement, Immunité, Santé, Thérapeutique (GEIST FR CNRS 3503)-Institut National de la Santé et de la Recherche Médicale (INSERM), Developpement Normal et Pathologique du Système Immunitaire, 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 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 Rhumatologie Dermatologie, Hôpital Femme Mère Enfant [CHU - HCL] (HFME), Hospices Civils de Lyon (HCL)-Hospices Civils de Lyon (HCL)-HCL Groupement Hospitalier Est-Centre de référence Maladies Rénales Rares, Service Pédiatrie - Néphrologie, CHU Toulouse [Toulouse]-Hôpital des Enfants, CHU Toulouse [Toulouse], Service Néphrologie Pédiatrique, CHU Strasbourg-Hôpital de Hautepierre [Strasbourg], Service de Néphrologie Pédiatrique, Centre Hospitalier Universitaire de Nice (CHU Nice)-Fondation LENVAL-Hopital pour Enfants, Service de Pédiatrie médicale - Spécialités médicales [CHU Limoges], CHU Limoges, Service Pédiatrie, CHU Bordeaux [Bordeaux]-Centre de référence des maladies rénales rares (SORARE), Service de Néphrologie, Centre Hospitalier Universitaire de La Réunion (CHU La Réunion), Service de Pédiatrie spécialisée, Centre Hospitalier Régional Universitaire [Montpellier] (CHRU Montpellier)-Hôpital Lapeyronie, Institut National de la Santé et de la Recherche Médicale (INSERM)-Génomique, Environnement, Immunité, Santé, Thérapeutique (GEIST FR CNRS 3503)-Université de Limoges (UNILIM)-CHU Limoges, HCL Groupement Hospitalier Est-Hôpital Femme Mère Enfant [CHU - HCL] (HFME), Hospices Civils de Lyon (HCL)-Hospices Civils de Lyon (HCL)-Centre de référence Maladies Rénales Rares, Marquet, Pierre, Service Néphrologie, médecine interne et hypertension pédiatrique [CHU Toulouse], Pôle Enfants [CHU Toulouse], Centre Hospitalier Universitaire de Toulouse (CHU Toulouse)-Centre Hospitalier Universitaire de Toulouse (CHU Toulouse), Pharmacologie des Immunosuppresseurs et de la Transplantation ( PIST ), Université de Limoges ( UNILIM ) -CHU Limoges-Génomique, Environnement, Immunité, Santé, Thérapeutique ( GEIST FR CNRS 3503 ) -Institut National de la Santé et de la Recherche Médicale ( INSERM ), 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 Necker - Enfants Malades [AP-HP], Hôpital Femme Mère Enfant [CHU - HCL] ( HFME ), Hospices Civils de Lyon ( HCL ) -Hospices Civils de Lyon ( HCL ) -HCL Groupement Hospitalier Est-Centre de référence Maladies Rénales Rares, CHU Toulouse [Toulouse]-Hôpital des enfants Toulouse, CHU Nice-Fondation LENVAL-Hopital pour Enfants, Centre Hospitalier Universitaire de La Réunion ( CHU La Réunion ), and Centre Hospitalier Régional Universitaire [Montpellier] ( CHRU Montpellier ) -Hôpital Lapeyronie
Subjects: Male, Adolescent, Pharmacokinetic Dynamic Relationships, PK/PD, Bayes Theorem, [SDV.SP]Life Sciences [q-bio]/Pharmaceutical sciences, Systemic Lupus Erythematosus, [SDV.SP] Life Sciences [q-bio]/Pharmaceutical sciences, [ SDV.SP ] Life Sciences [q-bio]/Pharmaceutical sciences, Logistic Models, Area Under Curve, Child, Preschool, Humans, Lupus Erythematosus, Systemic, Female, Pharmacokinetics, Mycophenolic acid, Drug Monitoring, Child, Immunosuppressive Agents, Retrospective Studies
Abstract: International audience; AIMS: The use of mycophenolate mofetil (MMF) in children with Systemic Lupus Erythematosus (SLE) is increasing. However, the clinical benefit of its monitoring has been scarcely studied, and little is known about its pharmacokinetics in this context. The objectives of the present study were: (i) to describe mycophenolic acid (MPA; the active moiety of MMF) pharmacokinetics; (ii) to develop a Bayesian estimator (BE) allowing the determination AUC (Area under the curve) from a limited number of blood samples; and (ii) to explore the relationships between exposure indices to MPA and the clinical status in children with SLE. METHODS: This was a retrospective study including 36 children with SLE, extracted from the expert system ISBA, for whom full- pharmacokinetic profiles of MPA were collected together with clinical data. A pharmacokinetic model and a BE were developed using an iterative two-stage Bayesian approach. ROC curve analyses and logistic regressions was used to investigate the association of exposure and active disease. RESULTS: A pharmacokinetic model and a BE were developed that allowed good AUC estimation performance (bias±SD=-0.02±0.15). ROC curve analyses showed that AUC/dose
Published: 2014
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22. Severe Global Inflammatory Involvement of Ocular Segments and Optic Disc Swelling in a 12-Year-Old Girl with Kawasaki Disease

Author: C. Debuisson, Christine Pajot, Cécile Lesage Beaudon, E. Grouteau, Karine Brochard, Soizic Paranon, Isabelle Claudet, Centre de Référence du Sud Ouest des Maladies Rénales Rares, CHU Toulouse [Toulouse]-Hôpital des Enfants, CHU Toulouse [Toulouse], Service Pédiatrique [Toulouse], Hôpital des Enfants, Embodiment, social ineQualities, lifecoUrse epidemiology, cancer and chronIc diseases, intervenTions, methodologY (Equipe 5 - EQUITY), 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)-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), Institut National de la Santé et de la Recherche Médicale (INSERM)-Université Toulouse III - Paul Sabatier (UT3), and Université Fédérale Toulouse Midi-Pyrénées-Université Fédérale Toulouse Midi-Pyrénées
Subjects: medicine.medical_specialty, Visual acuity, Eye Diseases, media_common.quotation_subject, Optic Disk, Vision Disorders, Visual Acuity, Mucocutaneous Lymph Node Syndrome, Keratitis, 03 medical and health sciences, 0302 clinical medicine, 030225 pediatrics, Ophthalmology, Humans, Medicine, Girl, Child, ComputingMilieux_MISCELLANEOUS, Subclinical infection, media_common, [SDV.MHEP.PED]Life Sciences [q-bio]/Human health and pathology/Pediatrics, Aspirin, business.industry, General Medicine, medicine.disease, Uveitis, Anterior, 3. Good health, Surgery, Vitreous Body, Posterior segment of eyeball, 030221 ophthalmology & optometry, [SDV.SPEE]Life Sciences [q-bio]/Santé publique et épidémiologie, Female, Kawasaki disease, medicine.symptom, business, Uveitis, Papilledema, medicine.drug
Abstract: Purpose Pediatric Kawasaki ocular involvement is dominated by bulbar conjunctival injection and mild, self-limited anterior uveitis. Posterior segment involvement is rare. Methods/Results Case report. Despite early efficient treatment including aspirin and intravenous immunoglobulins, a 12-year-old girl developed a severe bilateral global inflammatory ocular involvement including punctuated keratitis, retrodescemetic precipitates, anterior uveitis, vitritis, and bilateral optic disc swelling with papillitis. This is the first description of severe bilateral global inflammatory involvement of the eyes in Kawasaki disease (KD). Usually subclinical and self-limited, eye involvement in KD can lead to severe visual impairment. Conclusions Inflammation of both anterior and posterior segments does not seem to respond to KD-specific treatment and could justify a specific ophthalmologic therapeutic approach.
Published: 2010
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23. A universal predictive and mechanistic urinary peptide signature in acute kidney injury

Author: Piedrafita, Alexis, Siwy, Justyna, Klein, Julie, Akkari, Amal, Amaya-Garrido, Ana, Mebazaa, Alexandre, Sanz, Anna Belen, Breuil, Benjamin, Montero Herrero, Laura, Marcheix, Bertrand, Depret, François, Fernandez, Lucie, Tardif, Elsa, Minville, Vincent, Alves, Melinda, Metzger, Jochen, Grunenwald, Etienne, Feuillet, Guylène, Buléon, Marie, Brunet, Manon, Mayeur, Nicolas, Casemayou, Audrey, Labaste, François, Grossac, Julia, Mischak, Harald, Ortiz, Alberto, Gazut, Stéphane, Schanstra, Joost, Faguer, Stanislas, 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), Institut des Maladies Métaboliques et Cardiovasculaires, INSERM, Université Paul Sabatier, UMR 1297-I2MC, Toulouse, France, Université Toulouse III - Paul Sabatier (UT3), Université de Toulouse (UT), Laboratoire Sciences des Données et de la Décision (LS2D), Département Métrologie Instrumentation & Information (DM2I), Laboratoire d'Intégration des Systèmes et des Technologies (LIST (CEA)), 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)-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-Saclay-Laboratoire d'Intégration des Systèmes et des Technologies (LIST (CEA)), Commissariat à l'énergie atomique et aux énergies alternatives (CEA)-Commissariat à l'énergie atomique et aux énergies alternatives (CEA)-Université Paris-Saclay, Institut des Humanités numériques (IDHN), Equipes Traitement de l'Information et Systèmes (ETIS - UMR 8051), Ecole Nationale Supérieure de l'Electronique et de ses Applications (ENSEA)-Centre National de la Recherche Scientifique (CNRS)-CY Cergy Paris Université (CY)-Ecole Nationale Supérieure de l'Electronique et de ses Applications (ENSEA)-Centre National de la Recherche Scientifique (CNRS)-CY Cergy Paris Université (CY)-Lexiques, Textes, Discours, Dictionnaire - Centre Jean Pruvost (LT2D), CY Cergy Paris Université (CY)-CY Cergy Paris Université (CY)-Laboratoire Mobilités, Réseaux, Territoires, Environnements (MRTE - EA 4112), CY Cergy Paris Université (CY)-CY Cergy Paris Université (CY)-Laboratoire AGORA (AGORA - EA 7392), CY Cergy Paris Université (CY)-CY Cergy Paris Université (CY), Hôpitaux Universitaires Saint-Louis, Lariboisière, Fernand-Widal, Marqueurs cardiovasculaires en situation de stress (MASCOT (UMR_S_942 / U942)), Institut National de la Santé et de la Recherche Médicale (INSERM)-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 National de la Recherche Scientifique (CNRS)-Université Paris Cité (UPCité)-Université Sorbonne Paris Nord, Service Anesthésie - Hôpital des enfants [CHU Toulouse], Pôle Anesthésie Réanimation [CHU de Toulouse], Mosaiques Diagnostics (GmbH), Mosaique Diagnostics, Service Chirurgie Cardio-Vasculaire [CHU Toulouse] (CCV), Pôle Cardiovasculaire et Métabolique [CHU Toulouse], French National Institute of Health and Medical Research, Centre Hospitalier Universitaire de Toulouse (CHU Toulouse), ANR‐18‐PERM‐0003, and ANR-21-CE14-0013,CALPROTECT,Rôle de la calprotectine dans la calcification vasculaire associée à l'insuffisance rénale(2021)
Subjects: kidney, classification, peptide signature, statistical analysis, [INFO.INFO-TS]Computer Science [cs]/Signal and Image Processing, [SDV.BBM.GTP]Life Sciences [q-bio]/Biochemistry, Molecular Biology/Genomics [q-bio.GN], biomarker, acute kidney injury (AKI), signal processing, Critical Care and Intensive Care Medicine
Abstract: Background The delayed diagnosis of acute kidney injury (AKI) episodes and the lack of specificity of current single AKI biomarkers hamper its management. Urinary peptidome analysis may help to identify early molecular changes in AKI and grasp its complexity to identify potential targetable molecular pathways. Methods In derivation and validation cohorts totalizing 1170 major cardiac bypass surgery patients and in an external cohort of 1569 intensive care unit (ICU) patients, a peptide-based score predictive of AKI (7-day KDIGO classification) was developed, validated, and compared to the reference biomarker urinary NGAL and NephroCheck and clinical scores. Results A set of 204 urinary peptides derived from 48 proteins related to hemolysis, inflammation, immune cells trafficking, innate immunity, and cell growth and survival was identified and validated for the early discrimination (p p p Conclusions An overarching AKI physiopathology-driven urinary peptide signature shows significant promise for identifying, at an early stage, patients who will progress to AKI and thus to develop tailored treatments for this frequent and life-threatening condition. Performance of the urine peptide signature is as high as or higher than that of single biomarkers but adds mechanistic information that may help to discriminate sub-phenotypes of AKI offering new therapeutic avenues.
Published: 2022
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24. Results from the French National Esophageal Atresia register: one-year outcome

Author: Frédéric Gottrand, J.-L. Lemelle, Laurent Michaud, Rony Sfeir, Josephine Borgnon, J. Gaudin, Cécile Pelatan, Virginie Fouquet, Frédéric Auber, Christophe Laplace, J. Languepin, Frederic Lavrand, Jean-Luc Michel, Hubert Lardy, Thierry Petit, Guillaume Podevin, Allal Hossein, Anne Breton, P. Buisson, M.L. Poli-Merol, Arnaud Bonnard, Pascal de Lagausie, Corine Borderon, Olivier Jaby, Dominique Weil, Anis Echaieb, Manuel Lopez, Anne Schneider, F Elbaz, Myriam Pouzac, Thierry Merrot, François Becmeur, Jean Breaud, S. Geiss, François Lefebvre, Sébastien Blanc, Naziha Khen-Dunlop, Didier Aubert, Catherine Jacquier, E. Habonimana, Philine De Vries, Terres Inovia, PremUp Foundation, Institut de Recherche pour le Développement (IRD)-Université Paris-Sud - Paris 11 (UP11)-Université Pierre et Marie Curie - Paris 6 (UPMC)-Université Paris Diderot - Paris 7 (UPD7)-CHI Créteil-Université Paris Descartes - Paris 5 (UPD5)-Institut National de la Santé et de la Recherche Médicale (INSERM), Service de Chirurgie Viscérale Pédiatrique, Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-CHU Necker - Enfants Malades [AP-HP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP), CHU Trousseau [APHP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU), Service Diabétologie [CHU Toulouse], Pôle Cardiovasculaire et Métabolique [CHU Toulouse], Centre Hospitalier Universitaire de Toulouse (CHU Toulouse)-Centre Hospitalier Universitaire de Toulouse (CHU Toulouse), Hémodynamique, Interaction Fibrose et Invasivité tumorales Hépatiques (HIFIH), Université d'Angers (UA), Hôpital Jeanne de Flandre [Lille], 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, Hôpital Bicètre, Service de chirurgie pédiatrique, Hôpital Bicêtre, service de chirurgie viscérale pédiatrique [CHRU Nancy], Centre Hospitalier Régional Universitaire de Nancy (CHRU Nancy), Service de chirurgie pédiatrique [CHU Bordeaux], Hôpital des Enfants - Groupe hospitalier Pellegrin - CHU de Bordeaux, Service de chirurgie pédiatrique [CHU Strasbourg], Les Hôpitaux Universitaires de Strasbourg (HUS), Mines Nantes (Mines Nantes), Theory, Algorithms and Systems for Constraints (TASC), Laboratoire d'Informatique de Nantes Atlantique (LINA), Mines Nantes (Mines Nantes)-Université de Nantes (UN)-Centre National de la Recherche Scientifique (CNRS)-Mines Nantes (Mines Nantes)-Université de Nantes (UN)-Centre National de la Recherche Scientifique (CNRS)-Département informatique - EMN, Mines Nantes (Mines Nantes)-Inria Rennes – Bretagne Atlantique, Institut National de Recherche en Informatique et en Automatique (Inria)-Institut National de Recherche en Informatique et en Automatique (Inria), Service de Pédiatrie, Centre Hospitalier Universitaire de Reims (CHU Reims)-American Memorial Hospital (Reims), Université de Reims Champagne-Ardenne (URCA), UMR 6578 : Anthropologie Bio-Culturelle (UAABC), Université de la Méditerranée - Aix-Marseille 2-Centre National de la Recherche Scientifique (CNRS), Service de Chirurgie et Radiologie Pédiatrique, Université de la Méditerranée - Aix-Marseille 2, Laboratoire de recherche sur la gouvernance publique, territoire et communication (LARGOTEC/CECCOPOP), Université Paris-Est Créteil Val-de-Marne - Paris 12 (UPEC UP12), Système Nerveux Autonome - Epidémiologie, Physiologie, Ingénierie, Santé (SNA - EPIS), Centre Hospitalier Universitaire de Saint-Etienne [CHU Saint-Etienne] (CHU ST-E)-Université Jean Monnet - Saint-Étienne (UJM), CHU Pontchaillou [Rennes], Centre Hospitalier Le Mans (CH Le Mans), Centre Hospitalier Régional Universitaire de Brest (CHRU Brest), Centre Hospitalier Régional Universitaire de Tours (CHRU de Tours), Service Pédiatrie, Centre Hospitalier Universitaire [Rennes], Service de chirurgie pédiatrique [CHU Besançon], Centre Hospitalier Régional Universitaire de Besançon (CHRU Besançon), Centre Hospitalier Universitaire de Nice (CHU Nice), Service de Chirurgie infantile, CHU Fort de France-Hôpital Pierre Zobda Quitman, CHU Clermont-Ferrand, CHU Strasbourg, Hôpital Jeanne de Flandres, Université de Lille, Droit et Santé-Centre Hospitalier Régional Universitaire [Lille] (CHRU Lille), Union Nationale Interprofessionnelle des plantes riches en Protéines (UNIP), Service de chirurgie viscérale pédiatrique [CHU Trousseau], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-CHU Trousseau [APHP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU)-Sorbonne Université (SU), Gastro-entérologie, Hépatologie, Nutrition, Diabétologie, Système Nerveux Autonome - Epidémiologie, Physiologie, Ingénierie, Santé (SNA-EPIS), Université Jean Monnet [Saint-Étienne] (UJM)-Centre Hospitalier Universitaire de Saint-Etienne, Institut de Recherche pour le Développement (IRD)-Université Paris-Sud - Paris 11 (UP11)-Université Pierre et Marie Curie - Paris 6 (UPMC)-Université Paris Diderot - Paris 7 (UPD7)-CHI Créteil-Université Paris Descartes - Paris 5 (UPD5)-Sorbonne Université (SU)-Institut National de la Santé et de la Recherche Médicale (INSERM), Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU), Hôpital des Enfants, CHU Toulouse [Toulouse]-CHU Toulouse [Toulouse], Université de Lille, Droit et Santé-Institut National de la Santé et de la Recherche Médicale (INSERM), Inria Rennes – Bretagne Atlantique, Institut National de Recherche en Informatique et en Automatique (Inria)-Institut National de Recherche en Informatique et en Automatique (Inria)-Département informatique - EMN, Mines Nantes (Mines Nantes)-Mines Nantes (Mines Nantes)-Laboratoire d'Informatique de Nantes Atlantique (LINA), and Mines Nantes (Mines Nantes)-Université de Nantes (UN)-Centre National de la Recherche Scientifique (CNRS)-Université de Nantes (UN)-Centre National de la Recherche Scientifique (CNRS)
Subjects: Male, congenital, hereditary, and neonatal diseases and abnormalities, Pediatrics, medicine.medical_specialty, Time Factors, Long term follow up, One-year outcome, [SDV]Life Sciences [q-bio], Pharmacology toxicology, Population, Treatment outcome, Outcome (game theory), Population-based registry, 03 medical and health sciences, 0302 clinical medicine, 030225 pediatrics, medicine, Humans, Genetics(clinical), Pharmacology (medical), Registries, 030212 general & internal medicine, education, Esophageal Atresia, Long-term follow-up, Genetics (clinical), Medicine(all), 2. Zero hunger, education.field_of_study, business.industry, Research, Infant, Newborn, Infant, General Medicine, medicine.disease, 3. Good health, Hospitalization, Treatment Outcome, Register (music), Population Surveillance, Atresia, Female, France, business, Population-Based Registry, Follow-Up Studies
Abstract: Background The aim of the present national prospective population-based study was to assess the early morbidity of esophageal atresia (EA). Methods All 38 multidisciplinary French centers that care for patients with EA returned a specific questionnaire about the 1-year outcome for each patient. This information was centralized, checked, and entered into a database. Results From the total population of 307 EA patients born in 2008 and 2009, data about the 1-year outcome were obtained from 301 (98%) patients, of whom 4% were lost to follow-up and 5% died. Medical complications occurred in 34% of the patients: anastomotic leaks (8%), recurrent tracheoesophageal fistula (4%), and anastomotic stenosis (22%); all of the latter group needed dilation (median, 2 dilations/patient). A new hospitalization was required for 59% of patients (2.5 hospitalizations/patient) for digestive (52%) or respiratory (48%) reasons. Twelve percent of patients required antireflux surgery at a median age of 164 days (range, 33–398 days), and 1% underwent an aortopexy for severe tracheomalacia. The weight/age Z-score was −0.8 (range, −5.5 to 3.7 months) at 12 months. Fifteen percent of patients were undernourished at 12 months of age, whereas 37% presented with respiratory symptoms and 15% had dysphagia at the last follow-up. Significant independent factors associated with medical complications were anastomotic esophageal tension (p = .0009) and presence of a gastrostomy (p = .0002); exclusive oral feeding at discharge was associated with a decreased risk of complications (p = .007). Conclusions Digestive and respiratory morbidities remain frequent during the first year of life and are associated with difficult anastomosis and lack of full oral feeding. Electronic supplementary material The online version of this article (doi:10.1186/s13023-014-0206-5) contains supplementary material, which is available to authorized users.
Published: 2014
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25. rhBSSL Improves Growth and LCPUFA Absorption in Preterm Infants Fed Formula or Pasteurized Breast Milk

Author: Mårten Vagerö, Luca Maggio, Virgilio P. Carnielli, Kristina Timdahl, Birgitta Olsson, Charlotte Casper, Jean-Michel Hascoet, Olle Hernell, Alexandre Lapillonne, Unité de Néonatalogie [Hôpital des enfants Toulouse], Hôpital des Enfants, CHU Toulouse [Toulouse]-CHU Toulouse [Toulouse], Polytechnic University of Marche — Ospedali Riuniti Ancona [Italy], Service de Médecine Néonatale [CHRU Nancy], Centre Hospitalier Régional Universitaire de Nancy (CHRU Nancy), Développement, Adaptation et Handicap. Régulations cardio-respiratoires et de la motricité (DevAH), Université de Lorraine (UL), CHU Necker - Enfants Malades [AP-HP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP), Università cattolica del Sacro Cuore [Roma] (Unicatt), Swedish Orphan Biovitrum (SOBI), and Umeå University
Subjects: Male, Pediatrics, medicine.medical_specialty, Docosahexaenoic Acids, Physiology, Pasteurization, Absorption (skin), Organ development, Breast milk, Intestinal absorption, law.invention, preterm infant, Child Development, Double-Blind Method, law, Humans, Infant, Very Low Birth Weight, Medicine, recombinant human bile-salt-stimulated lipase, Arachidonic Acid, Cross-Over Studies, Milk, Human, business.industry, Infant, Newborn, Gastroenterology, Infant, Original Articles: Hepatology and Nutrition, food and beverages, fat absorption, Sterol Esterase, clinical study, Crossover study, Infant Formula, Recombinant Proteins, 3. Good health, Intestinal Absorption, Infant formula, Docosahexaenoic acid, Pediatrics, Perinatology and Child Health, growth velocity, Female, business, Infant, Premature, [SDV.MHEP]Life Sciences [q-bio]/Human health and pathology
Abstract: International audience; Objectives:Preterm infants often experience suboptimal growth, which can affect organ development. The aim of this study was to improve growth by treatment with bile salt–stimulated lipase (BSSL), naturally present in breast milk, but lost after pasteurization, and absent in formula.Methods:Two clinical trials were performed with a predefined analysis of combined data to investigate the effects of recombinant human BSSL (rhBSSL) treatment on growth velocity and fat absorption in preterm infants. The studies were randomized and double-blinded comparing 7-day treatment with rhBSSL and placebo, administered in pasteurized breast milk or formula, using a crossover design.Results:Sixty-three infants were evaluated for safety. At randomization, the mean (standard deviation) weight was 1467 (193) g and mean postmenstrual age was 32.6 (0.5) weeks. Sixty and 46 infants were evaluated for growth velocity and fat absorption, respectively. rhBSSL treatment significantly improved mean growth velocity by 2.93 g · kg−1 · day−1 (P
Published: 2014
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26. Simultaneous detection of gastrointestinal pathogens with a multiplex Luminex-based molecular assay in stool samples from diarrhoeic patients

Author: Jacques Izopet, Isabelle Claudet, M.-F. Prère, Alexis Valentin, Melinda Benard, G. Plat, Nassim Kamar, N. Marty, Catherine Mengelle, Anne Huynh, E. Grouteau, Antoine Berry, Jean-Michel Mansuy, CHU Purpan, Hôpital Purpan [Toulouse], CHU Toulouse [Toulouse]-CHU Toulouse [Toulouse], Laboratoire de microbiologie et génétique moléculaires (LMGM), Centre de Biologie Intégrative (CBI), Université Toulouse III - Paul Sabatier (UT3), Université Fédérale Toulouse Midi-Pyrénées-Université Fédérale Toulouse Midi-Pyrénées-Centre National de la Recherche Scientifique (CNRS)-Université Toulouse III - Paul Sabatier (UT3), Université Fédérale Toulouse Midi-Pyrénées-Université Fédérale Toulouse Midi-Pyrénées-Centre National de la Recherche Scientifique (CNRS)-Centre National de la Recherche Scientifique (CNRS), CHU Toulouse [Toulouse], Embodiment, social ineQualities, lifecoUrse epidemiology, cancer and chronIc diseases, intervenTions, methodologY (Equipe 5 - EQUITY), Epidémiologie et analyses en santé publique : risques, maladies chroniques et handicaps (LEASP), Université Fédérale Toulouse Midi-Pyrénées-Université Fédérale Toulouse Midi-Pyrénées-Institut National de la Santé et de la Recherche Médicale (INSERM)-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), Institut de médecine moléculaire de Rangueil (I2MR), Université Fédérale Toulouse Midi-Pyrénées-Université Fédérale Toulouse Midi-Pyrénées-IFR150-Institut National de la Santé et de la Recherche Médicale (INSERM), Unité de Néonatalogie [Hôpital des enfants Toulouse], Hôpital des Enfants, Laboratoire de Virologie [Toulouse], Institut National de la Santé et de la Recherche Médicale (INSERM)-Université Toulouse III - Paul Sabatier (UT3), Université Fédérale Toulouse Midi-Pyrénées-Université Fédérale Toulouse Midi-Pyrénées, Pharmacochimie et Biologie pour le Développement (PHARMA-DEV), Université Fédérale Toulouse Midi-Pyrénées-Université Fédérale Toulouse Midi-Pyrénées-Institut de Chimie de Toulouse (ICT-FR 2599), Institut National Polytechnique (Toulouse) (Toulouse INP), Université Fédérale Toulouse Midi-Pyrénées-Université Fédérale Toulouse Midi-Pyrénées-Centre National de la Recherche Scientifique (CNRS)-Institut de Recherche pour le Développement (IRD)-Université Toulouse III - Paul Sabatier (UT3), Université Fédérale Toulouse Midi-Pyrénées-Institut de Chimie du CNRS (INC)-Institut National Polytechnique (Toulouse) (Toulouse INP), Université Fédérale Toulouse Midi-Pyrénées-Centre National de la Recherche Scientifique (CNRS)-Institut de Recherche pour le Développement (IRD)-Institut de Chimie du CNRS (INC)-Institut de Recherche pour le Développement (IRD), Centre de Physiopathologie de Toulouse-Purpan (INSERM U563 - CNRS UMR1037), CHU Toulouse [Toulouse]-Institut Claudius Regaud-Hôpital Purpan [Toulouse], and CHU Toulouse [Toulouse]-Institut National de la Santé et de la Recherche Médicale (INSERM)-Centre de lutte contre le cancer (CLCC)-Centre National de la Recherche Scientifique (CNRS)
Subjects: Adult, Diarrhea, Microbiological Techniques, Microbiology (medical), Salmonella, Microbiological culture, Adolescent, medicine.drug_class, [SDV]Life Sciences [q-bio], 030231 tropical medicine, Antibiotics, Biology, medicine.disease_cause, Microbiology, Feces, 03 medical and health sciences, 0302 clinical medicine, Rotavirus, medicine, Humans, Multiplex, Prospective Studies, Child, ComputingMilieux_MISCELLANEOUS, Aged, 0303 health sciences, microbiological, [SDV.MHEP.PED]Life Sciences [q-bio]/Human health and pathology/Pediatrics, Chi-Square Distribution, 030306 microbiology, Campylobacter, Infant, Cryptosporidium, General Medicine, Middle Aged, Clostridium difficile, biology.organism_classification, Diarrhoea, 3. Good health, Molecular Typing, multiplex, Infectious Diseases, Child, Preschool, luminex, [SDV.SPEE]Life Sciences [q-bio]/Santé publique et épidémiologie, stools
Abstract: International audience; We have evaluated the multiplex molecular method xTAG(®) Gastrointestinal Panel (GPP) for detecting pathogens in stool samples of diarrhoeic patients. We collected 440 samples from 329 patients (male:female ratio of 1.2:1), including 102 immunosuppressed adults, 50 immunosuppressed children, 56 children attending the neonatal unit and 121 children attending the emergency unit. Of these, 176 samples from 162 patients were xTAG(®) GPP positive (102 viruses, 61 bacteria and 13 parasites) and the assay was more sensitive than the conventional test for detecting rotavirus (p
Published: 2013
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27. Differences in Caco-2 cell attachment, migration on collagen and fibronectin coated polyelectrolyte surfaces

Author: N. Zanina, A.C. Duncan, L. Mora, David Vaudry, Magalie Bénard, Mina Souiri, Thierry Jouenne, Ali Othmane, Institut Galilée (IG), Université Paris 13 (UP13), Polymères Biopolymères Surfaces (PBS), Institut national des sciences appliquées Rouen Normandie (INSA Rouen Normandie), Institut National des Sciences Appliquées (INSA)-Normandie Université (NU)-Institut National des Sciences Appliquées (INSA)-Normandie Université (NU)-Institut de Chimie du CNRS (INC)-Institut Normand de Chimie Moléculaire Médicinale et Macromoléculaire (INC3M), Institut de Chimie du CNRS (INC)-École Nationale Supérieure d'Ingénieurs de Caen (ENSICAEN), Normandie Université (NU)-Normandie Université (NU)-Institut national des sciences appliquées Rouen Normandie (INSA Rouen Normandie), Institut National des Sciences Appliquées (INSA)-Normandie Université (NU)-Institut National des Sciences Appliquées (INSA)-Université Le Havre Normandie (ULH), Normandie Université (NU)-Université de Rouen Normandie (UNIROUEN), Normandie Université (NU)-Centre National de la Recherche Scientifique (CNRS)-Université de Caen Normandie (UNICAEN), Normandie Université (NU)-École Nationale Supérieure d'Ingénieurs de Caen (ENSICAEN), Normandie Université (NU)-Université Le Havre Normandie (ULH), Normandie Université (NU)-Centre National de la Recherche Scientifique (CNRS), Unité de Néonatalogie [Hôpital des enfants Toulouse], Hôpital des Enfants, CHU Toulouse [Toulouse]-CHU Toulouse [Toulouse], International Livestock Research Institute [CGIAR, Ethiopie] (ILRI), Consultative Group on International Agricultural Research [CGIAR] (CGIAR), Institut Fédératif de Recherches Multidisciplinaires sur les Peptides (IFRMP 23), Institut National de la Santé et de la Recherche Médicale (INSERM)-Centre de Lutte Contre le Cancer Henri Becquerel Normandie Rouen (CLCC Henri Becquerel)-Université de Rouen Normandie (UNIROUEN), Normandie Université (NU)-Normandie Université (NU)-Université Le Havre Normandie (ULH), Normandie Université (NU)-CHU Rouen, Différenciation et communication neuronale et neuroendocrine (DC2N), Université de Rouen Normandie (UNIROUEN), and Normandie Université (NU)-Normandie Université (NU)-Institut National de la Santé et de la Recherche Médicale (INSERM)
Subjects: [SDV]Life Sciences [q-bio], Cell, Biomedical Engineering, Motility, Bioengineering, 02 engineering and technology, 010402 general chemistry, 01 natural sciences, Applied Microbiology and Biotechnology, Allylamine, chemistry.chemical_compound, medicine, Viability assay, Cell adhesion, ComputingMilieux_MISCELLANEOUS, biology, Adhesion, 021001 nanoscience & nanotechnology, Polyelectrolyte, 0104 chemical sciences, Fibronectin, medicine.anatomical_structure, Biochemistry, chemistry, Biophysics, biology.protein, 0210 nano-technology, Biotechnology
Abstract: Metastasis mechanisms depend on cell metabolism changes, migration and adhesion to different tissues. To understand their choice of interaction site, the tumoral cell adhesion to model surfaces was studied. The response of Caco-2 tumoral cells cultured on polyelectrolyte film-functionalized surfaces with or without adhesion proteins (fibronectin or collagen IV) was analyzed. Using the layer-by- layer method, multilayer films were prepared with cationic poly(allylamine hydrochloride) and anionic poly(sodium 4-styrenesulfonate) polyelectrolytes. Film surface wettability was evaluated. The electrochemical impedance spectroscopy analyses were carried out to control the elaborated surfaces on which Caco-2 tumoral cells were cultured. The cell velocity was studied by video-microscopy and a cell colorimetric assay (WST-1) was used to quantify cell viability. The film surface parameters as well as the protein nature and localization in the film were found to modulate cell response. Results demonstrated that the cancer cell motility and proliferation were higher when cultured onto pure collagen located above the polyelectrolyte film and that the reverse surprisingly was observed when proteins were inserted into the polyelectrolyte film. Data also showed that cell motility was correlated with a high charge transfer resistance (Rct) and a low surface free energy (SFE) polar component (electron donor character). This relationship was valid only for pure external proteins. Thus, fibronectin exhibited a low Rct and a high SFE polar component, which decreased cell motility and proliferation.
Published: 2013
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28. Natural history of Barth syndrome: a national cohort study of 22 patients

Author: Damien Bonnet, Marlène Rio, Pascale de Lonlay, Jean Donadieu, Helene Ansquer, Géraldine Viot, François Rivier, Blandine Beaupain, Philippe Charron, Charlotte Rigaud, Renaud Touraine, Sylvie Di Filippo, Chris Ottolenghi, Alice Goldenberg, Anne-Sophie Lebre, Betina Borm, Allel Chabli, Michèle Mathieu-Dramard, Hulya Ozsahin, Marie-Catherine Vaillant, Service d'Hémato-oncologie Pédiatrique, Assistance publique - Hôpitaux de Paris (AP-HP)-CHU Trousseau [APHP], 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 ), Service de Génétique Clinique Chromosomique et Moléculaire, CHU Saint-Etienne-Hôpital Nord - Saint-Etienne, Laboratoire de Biochimie, Assistance publique - Hôpitaux de Paris (AP-HP)-Université Paris Descartes - Paris 5 ( UPD5 ) -CHU Necker - Enfants Malades [AP-HP], Service de Néonatalogie, Hôpital Morvan [Brest]-Centre Hospitalier Régional Universitaire de Brest ( CHRU Brest ), Service d'Onco-Hématologie Pédiatrique, Hôpitaux Universitaires de Genève ( HUG ) -Hôpital des Enfants, Service de Cardiologie Pédiatrique, Hospices Civils de Lyon ( HCL ) -Hôpital Louis Pradel [CHU - HCL], Hospices Civils de Lyon ( HCL ), Centre de référence des maladies métaboliques, Service de Pédiatrie, CH Béziers, Service de Neuropédiatrie, Centre Hospitalier Régional Universitaire [Montpellier] ( CHRU Montpellier ) -Hôpital Gui de Chauliac, Service de Médecine Pédiatrique, CHU Toulouse [Toulouse]-Centre Hospitalo-Universitaire, Service de Génétique [Amiens], CHU Amiens-Picardie, Service de génétique [Rouen], CHU Rouen-Université de Rouen Normandie ( UNIROUEN ), Normandie Université ( NU ) -Normandie Université ( NU ), Service de Génétique [Cochin], Assistance publique - Hôpitaux de Paris (AP-HP)-CHU Cochin [AP-HP], Centre de référence pour les maladies cardiaques héréditaires, Assistance publique - Hôpitaux de Paris (AP-HP)-CHU Pitié-Salpêtrière [APHP], Assistance Publique-Hôpitaux de Paris, Centre de Référence M3C Malformations Cardiaques Congénitales Complexes, Assistance publique - Hôpitaux de Paris (AP-HP)-Université Paris Descartes - Paris 5 ( UPD5 ) -PRES Sorbonne Paris Cité-CHU Necker - Enfants Malades [AP-HP], BMC, Ed., CHU Trousseau [APHP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU), 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), Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Université Paris Descartes - Paris 5 (UPD5)-CHU Necker - Enfants Malades [AP-HP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP), Hôpital Morvan [Brest]-Centre Hospitalier Régional Universitaire de Brest (CHRU Brest), Hôpitaux Universitaires de Genève (HUG), Hospices Civils de Lyon (HCL)-Hôpital Louis Pradel [CHU - HCL], Hospices Civils de Lyon (HCL), Centre Hospitalier Régional Universitaire [Montpellier] (CHRU Montpellier)-Hôpital Gui de Chauliac [Montpellier], Pôle Enfants [CHU Toulouse], Centre Hospitalier Universitaire de Toulouse (CHU Toulouse), CHU Rouen, Normandie Université (NU)-Normandie Université (NU)-Université de Rouen Normandie (UNIROUEN), Normandie Université (NU), Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Hôpital Cochin [AP-HP], CHU Pitié-Salpêtrière [AP-HP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Université Paris Descartes - Paris 5 (UPD5)-PRES Sorbonne Paris Cité-CHU Necker - Enfants Malades [AP-HP], Hôpitaux Universitaires de Genève (HUG)-Hôpital des Enfants, CHU Toulouse [Toulouse]-CHU Toulouse [Toulouse], and Sorbonne Université (SU)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)
Subjects: Male, Pediatrics, TAZ gene, Cardiomyopathy, [SDV.GEN] Life Sciences [q-bio]/Genetics, 030204 cardiovascular system & hematology, Cohort Studies, 0302 clinical medicine, Genetics(clinical), Pharmacology (medical), Child, Genetics (clinical), Medicine(all), 0303 health sciences, Neutropenia/complications, ddc:618, Incidence (epidemiology), Cohort, Barth syndrome, General Medicine, 3. Good health, Pedigree, Natural history, Survival Rate, Child, Preschool, Cardiomyopathies/complications, Female, France, Cardiomyopathies, Cohort study, medicine.medical_specialty, Neutropenia, Adolescent, Transcription Factors/genetics, 03 medical and health sciences, medicine, Humans, Barth Syndrome/complications/genetics/mortality/physiopathology, Survival rate, 030304 developmental biology, [SDV.GEN]Life Sciences [q-bio]/Genetics, business.industry, Research, medicine.disease, Barth Syndrome, Mutation, [ SDV.GEN ] Life Sciences [q-bio]/Genetics, business, Acyltransferases, Transcription Factors
Abstract: International audience; BACKGROUND: This study describes the natural history of Barth syndrome (BTHS). METHODS: The medical records of all patients with BTHS living in France were identified in multiple sources and reviewed. RESULTS: We identified 16 BTHS pedigrees that included 22 patients. TAZ mutations were observed in 15 pedigrees. The estimated incidence of BTHS was 1.5 cases per million births (95%CI: 0.2--2.3). The median age at presentation was 3.1 weeks (range, 0--1.4 years), and the median age at last follow-up was 4.75 years (range, 3--15 years). Eleven patients died at a median age of 5.1 months; 9 deaths were related to cardiomyopathy and 2 to sepsis. The 5-year survival rate was 51%, and no deaths were observed in patients >=3 years. Fourteen patients presented with cardiomyopathy, and cardiomyopathy was documented in 20 during follow-up. Left ventricular systolic function was very poor during the first year of life and tended to normalize over time. Nineteen patients had neutropenia. Metabolic investigations revealed inconstant moderate 3-methylglutaconic aciduria and plasma arginine levels that were reduced or in the low-normal range. Survival correlated with two prognostic factors: severe neutropenia at diagnosis (
Published: 2013
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29. Association between Initial Treatment Strategy and Long-Term Survival in Pulmonary Arterial Hypertension

Author: Vincent Cottin, David Montani, Jérémie Pichon, Martine Reynaud-Gaubert, Xavier Jaïs, Pascal Magro, Gérald Simonneau, Florence Parent, Fabrice Bauer, Marianne Riou, Laurent Bertoletti, Pamela Moceri, Ari Chaouat, Andrei Seferian, Antoine Beurnier, Sébastien Renard, Pierre Mauran, Delphine Horeau-Langlard, Pascal de Groote, Laurent Savale, Mitja Jevnikar, Sophie Bulifon, Pascal Roblot, Hélène Bouvaist, Yuanchao Feng, Patrice Poubeau, Sylvain Palat, Zhiying Liang, Emmanuel Bergot, François Picard, Etienne-Marie Jutant, C. Chabanne, Olivier Sitbon, Athénaïs Boucly, Grégoire Prévot, Jean-François Mornex, Cécile Tromeur, Marc Humbert, Bruno Degano, Claire Dauphin, Arnaud Bourdin, Olivier Sanchez, Nicolas Favrolt, Jason Weatherald, Hypertension arterielle pulmonaire physiopathologie et innovation thérapeutique, Centre chirurgical Marie Lannelongue-Institut National de la Santé et de la Recherche Médicale (INSERM), Université Paris-Saclay, AP-HP Hôpital Bicêtre (Le Kremlin-Bicêtre), 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), CHU Rouen, Normandie Université (NU), CHU Caen, Normandie Université (NU)-Tumorothèque de Caen Basse-Normandie (TCBN), Université de Caen Normandie (UNICAEN), Biologie intégrative du tissu osseux, Université Jean Monnet [Saint-Étienne] (UJM)-Institut National de la Santé et de la Recherche Médicale (INSERM), Centre Hospitalier Universitaire de Saint-Etienne (CHU de Saint-Etienne), Physiologie & médecine expérimentale du Cœur et des Muscles [U 1046] (PhyMedExp), Université de Montpellier (UM)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Centre National de la Recherche Scientifique (CNRS), Centre Hospitalier Régional Universitaire [Montpellier] (CHRU Montpellier), Centre Hospitalier Universitaire [Grenoble] (CHU), Laboratoire Traitement du Signal et de l'Image (LTSI), 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), CHU Pontchaillou [Rennes], Défaillance Cardiovasculaire Aiguë et Chronique (DCAC), Centre Hospitalier Régional Universitaire de Nancy (CHRU Nancy)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Université de Lorraine (UL), Service de Pneumologie [CHRU Nancy], Centre Hospitalier Régional Universitaire de Nancy (CHRU Nancy), Infections Virales et Pathologie Comparée - UMR 754 (IVPC), École pratique des hautes études (EPHE), Université Paris sciences et lettres (PSL)-Université Paris sciences et lettres (PSL)-Université Claude Bernard Lyon 1 (UCBL), Université de Lyon-Université de Lyon-Institut National de Recherche pour l’Agriculture, l’Alimentation et l’Environnement (INRAE), Hospices Civils de Lyon (HCL), CHU Gabriel Montpied [Clermont-Ferrand], CHU Clermont-Ferrand, Facteurs de Risque et Déterminants Moléculaires des Maladies liées au Vieillissement - U 1167 (RID-AGE), 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), Centre Hospitalier Universitaire de Dijon - Hôpital François Mitterrand (CHU Dijon), University of Calgary, Centre hospitalier universitaire de Nantes (CHU Nantes), Hôpital Nord Laennec [CHU Nantes], CHU Trousseau [Tours], Centre Hospitalier Régional Universitaire de Tours (CHRU Tours), Centre Hospitalier Universitaire de Reims (CHU Reims), American Memorial Hospital (Hôpital des enfants) [Reims], Centre Hospitalier Universitaire de Nice (CHU Nice), Université Côte d'Azur (UCA), Hôpital Dupuytren [CHU Limoges], Hôpital Haut-Lévêque [CHU Bordeaux], CHU Bordeaux [Bordeaux], Université de Bordeaux (UB), Centre Hospitalier Universitaire de La Réunion (CHU La Réunion), Hôpital Larrey [Toulouse], CHU Toulouse [Toulouse], Hôpital de la Timone [CHU - APHM] (TIMONE), Assistance Publique - Hôpitaux de Marseille (APHM), Aix Marseille Université (AMU), Nouvel Hôpital Civil de Strasbourg, Centre hospitalier universitaire de Poitiers (CHU Poitiers), 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), CIC Brest, Université de Brest (UBO)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Hôpital de la Cavale Blanche, Centre Chirurgical Marie Lannelongue (CCML)-Institut National de la Santé et de la Recherche Médicale (INSERM), Biologie Intégrative du Tissu Osseux (LBTO), Université Jean Monnet - Saint-Étienne (UJM)-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 Santé et de la Recherche Médicale (INSERM)-Université de Montpellier (UM)-Centre National de la Recherche Scientifique (CNRS), Université de Rennes (UR)-Institut National de la Santé et de la Recherche Médicale (INSERM), École Pratique des Hautes Études (EPHE), Centre Hospitalier Universitaire de Toulouse (CHU Toulouse), Institut National de la Santé et de la Recherche Médicale (INSERM)-Centre chirurgical Marie Lannelongue, Centre Hospitalier Régional Universitaire [Lille] (CHRU Lille)-Université de Lille-Institut National de la Santé et de la Recherche Médicale (INSERM)-Institut Pasteur de Lille, Réseau International des Instituts Pasteur (RIIP)-Réseau International des Instituts Pasteur (RIIP), and MORNET, Dominique
Subjects: Pulmonary and Respiratory Medicine, medicine.medical_specialty, business.industry, [SDV]Life Sciences [q-bio], Critical Care and Intensive Care Medicine, medicine.disease, survival, Pulmonary hypertension, 3. Good health, [SDV] Life Sciences [q-bio], pulmonary arterial hypertension, Internal medicine, pulmonary hypertension, Long term survival, therapeutics, Cardiology, Medicine, Initial treatment, business
Abstract: International audience; Rationale: The relationship between the initial treatment strategy and survival in pulmonary arterial hypertension (PAH) remains uncertain. Objectives: To evaluate the long-term survival of patients with PAH categorized according to the initial treatment strategy. Methods: A retrospective analysis of incident patients with idiopathic, heritable, or anorexigen-induced PAH enrolled in the French Pulmonary Hypertension Registry (January 2006 to December 2018) was conducted. Survival was assessed according to the initial strategy: monotherapy, dual therapy, or triple-combination therapy (two oral medications and a parenteral prostacyclin). Measurements and Main Results: Among 1,611 enrolled patients, 984 were initiated on monotherapy, 551 were initiated on dual therapy, and 76 were initiated on triple therapy. The triple-combination group was younger and had fewer comorbidities but had a higher mortality risk. The survival rate was higher with the use of triple therapy (91% at 5 yr) as compared with dual therapy or monotherapy (both 61% at 5 yr) (P < 0.001). Propensity score matching of age, sex, and pulmonary vascular resistance also showed significant differences between triple therapy and dual therapy (10-yr survival, 85% vs. 65%). In high-risk patients (n = 243), the survival rate was higher with triple therapy than with monotherapy or dual therapy, whereas there was no difference between monotherapy and double therapy. In intermediate-risk patients (n = 1,134), survival improved with an increasing number of therapies. In multivariable Cox regression, triple therapy was independently associated with a lower risk of death (hazard ratio, 0.29; 95% confidence interval, 0.11-0.80; P = 0.017). Among the 148 patients initiated on a parenteral prostacyclin, those on triple therapy had a higher survival rate than those on monotherapy or dual therapy. Conclusions: Initial triple-combination therapy that includes parenteral prostacyclin seems to be associated with a higher survival rate in PAH, particularly in the youngest high-risk patients.
Published: 2021
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30. Low influenza vaccination coverage in asthmatic children in France in 2006-7

Author: F. Rancé, Antoine Deschildre, M. Fayon, Isabelle Pin, Jean-Christophe Dubus, L. Donato, G. Le Manach, C. Santos, Caroline Thumerelle, André Labbé, M. Le Bourgeois, C. Llerena, J. de Blic, Catherine Weil-Olivier, M. Aubert, C. Chave, Hôpital des Enfants, CHU Toulouse [Toulouse], Sanofi Pasteur MSD, CHU Necker - Enfants Malades [AP-HP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP), Centre Hospitalier Régional Universitaire [Lille] (CHRU Lille), Centre Hospitalier Universitaire Strasbourg, CHU Strasbourg, Centre Hospitalier Universitaire Marseille, CHU Marseille, CHU Bordeaux [Bordeaux], CHU Clermont-Ferrand, Service de pédiatrie générale et maladies infectieuses, CHU Grenoble, Université Paris Diderot - Paris 7 (UPD7), Hôpital des enfants, Centre Hospitalier Régional Universitaire [Lille] ( CHRU Lille ), Centre Hospitalier Universitaire Clermont Ferrand, Université Paris Diderot - Paris 7 ( UPD7 ), Vesin, Aurélien, and Centre Hospitalier Universitaire de Toulouse (CHU Toulouse)
Subjects: Male, Pediatrics, MESH: Asthma, Epidemiology, MESH: Immunization Programs, Seasonal influenza, MESH: Influenza Vaccines, 0302 clinical medicine, MESH : Physician's Practice Patterns, MESH : Child, Surveys and Questionnaires, MESH: Child, MESH : Female, 030212 general & internal medicine, MESH : Influenza, Human, Practice Patterns, Physicians', Child, MESH: Influenza, Human, Respiratory disease, MESH : Questionnaires, [ SDV.SPEE ] Life Sciences [q-bio]/Santé publique et épidémiologie, 3. Good health, Asthmatic children, Vaccination, Influenza Vaccines, Vaccination coverage, Female, France, medicine.medical_specialty, Adolescent, MESH : Male, MESH : Asthma, MESH : Immunization Programs, 03 medical and health sciences, 030225 pediatrics, Virology, MESH : Adolescent, Influenza, Human, medicine, Asthmatic patient, Humans, MESH : France, MESH: Physician's Practice Patterns, Asthma, MESH: Adolescent, MESH: Humans, business.industry, Immunization Programs, Public health, MESH: Questionnaires, MESH : Humans, Public Health, Environmental and Occupational Health, medicine.disease, MESH : Influenza Vaccines, MESH: Male, MESH: France, [SDV.SPEE] Life Sciences [q-bio]/Santé publique et épidémiologie, Immunology, [SDV.SPEE]Life Sciences [q-bio]/Santé publique et épidémiologie, business, MESH: Female
Abstract: International audience; In France, annual seasonal influenza vaccination has been recommended since 2000 for patients suffering from chronic respiratory diseases, including asthma. Since 1988, each year from September to December, a free influenza vaccination voucher is sent by the French Public Health Insurance authorities to patients with chronic respiratory disease, including severe asthma. In November 2006, this measure was extended to all asthmatic patients, irrespective of asthma severity. The present paper examines the 2006-7 influenza vaccination coverage rate (VCR) in 433 asthmatic children aged 6 to 17 years (mean age: 9.5 years; male: 61%) who consulted a paediatric pulmonologist between March and September 2007 in eight hospitals throughout France. The influenza VCR was 15.7% for the 2006-7 season (13.9% for the 2005-6 season and 10.9% for the 2004-5 season). General practitioners vaccinated 72.1% of the children. "Lack of information" (42%) was the most frequently reported reason for non-vaccination. Vouchers (received by 39.6% of the children) significantly increased the VCR (31% versus 5.9%; p
Published: 2008

31. Parental smoking, maternal alcohol, coffee and tea consumption during pregnancy and childhood malignant central nervous system tumours: the ESCALE study (SFCE)

Author: Dominique Plantaz, Didier Frappaz, Denis Hémon, Jacqueline Clavel, Brigitte Lacour, Xavier Rialland, Celine Icher, François Doz, Olivier Hartmann, Anne-Isabelle Bertozzi, Pascal Chastagner, Anne-Sophie Defaschelles, Matthieu Plichart, Alain Pierre-Kahn, Florence Menegaux, Secretariat, U754, Epidémiologie environnementale des cancers, Université Paris-Sud - Paris 11 (UP11)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Institut National de la Santé et de la Recherche Médicale (INSERM), Registre National des Tumeurs Solides de l'Enfant (RNTSE), Centre Hospitalier Régional Universitaire de Nancy (CHRU Nancy)-Cancéropôle du Grand Est, Département de Pédiatrie, Institut Gustave Roussy (IGR), Centre Léon Bérard [Lyon], Institut Curie [Paris], Centre Hospitalier Universitaire de Toulouse (CHU Toulouse), Institut de médecine predictive et de recherche thérapeutique (IMPRT), Institut Pasteur de Lille, Réseau International des Instituts Pasteur (RIIP)-Réseau International des Instituts Pasteur (RIIP)-Centre Régional de Lutte contre le Cancer Oscar Lambret [Lille] (UNICANCER/Lille), Université de Lille-UNICANCER-Université de Lille-UNICANCER-Institut National de la Santé et de la Recherche Médicale (INSERM)-Université de Lille, Droit et Santé-Centre Hospitalier Régional Universitaire [Lille] (CHRU Lille), CHU Necker - Enfants Malades [AP-HP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP), Hôpital Pellegrin, CHU Bordeaux [Bordeaux]-Groupe hospitalier Pellegrin, Centre Hospitalier Régional Universitaire de Nancy (CHRU Nancy), Département de pédiatrie, CHU Grenoble-Hôpital Michallon, Centre Robert Debré, Centre Hospitalier Universitaire d'Angers (CHU Angers), PRES Université Nantes Angers Le Mans (UNAM)-PRES Université Nantes Angers Le Mans (UNAM), Registre National des Hémopathies malignes de l'Enfant (RNHE), Institut de Veille Sanitaire (INVS)-Institut National de la Santé et de la Recherche Médicale (INSERM), This work was supported by grants from INSERM, the Fondation de France, the Association pour la Recherche contre le Cancer (ARC), the Agence Française de Sécurité Sanitaire des Produits de Santé (AFSSAPS), the Agence Française de Sécurité Sanitaire de l'Environnement et du Travail (AFSSET) and the association 'Cent pour sang la vie'., Hôpital des Enfants, CHU Toulouse [Toulouse], Université Lille Nord de France (COMUE)-UNICANCER-Université Lille Nord de France (COMUE)-UNICANCER-Institut National de la Santé et de la Recherche Médicale (INSERM)-Université de Lille, Droit et Santé-Centre Hospitalier Régional Universitaire [Lille] (CHRU Lille), Université Paris-Sud - Paris 11 ( UP11 ) -Institut National de la Santé et de la Recherche Médicale ( INSERM ) -Institut National de la Santé et de la Recherche Médicale ( INSERM ), Registre National des Tumeurs Solides de l'Enfant ( RNTSE ), Centre Hospitalier Régional Universitaire de Nancy ( CHRU Nancy ) -Cancéropôle du Grand Est, Institut Gustave Roussy ( IGR ), Institut Curie, Hôpital des enfants, Institut de médecine predictive et de recherche thérapeutique ( IMPRT ), Réseau International des Instituts Pasteur ( RIIP ) -Réseau International des Instituts Pasteur ( RIIP ) -CRLCC Oscar Lambret-Institut National de la Santé et de la Recherche Médicale ( INSERM ) -Université de Lille, Droit et Santé-Centre Hospitalier Régional Universitaire [Lille] ( CHRU Lille ), Centre Hospitalier Régional Universitaire de Nancy ( CHRU Nancy ), CHU Angers, Registre National des Hémopathies malignes de l'Enfant ( RNHE ), and Institut de Veille Sanitaire (INVS)-Institut National de la Santé et de la Recherche Médicale ( INSERM )
Subjects: Male, Pathology, MESH : Tea, MESH: Chi-Square Distribution, Physiology, MESH: Logistic Models, MESH : Central Nervous System Neoplasms, MESH: Risk Assessment, alcohols, MESH: Central Nervous System Neoplasms, Central Nervous System Neoplasms, 0302 clinical medicine, MESH: Pregnancy, MESH : Child, Pregnancy, Reference Values, MESH: Child, MESH: Incidence, Child, education.field_of_study, Incidence, MESH: Sex Distribution, MESH: Reference Values, MESH : Infant, MESH : Maternal Exposure, [ SDV.SPEE ] Life Sciences [q-bio]/Santé publique et épidémiologie, MESH: Follow-Up Studies, MESH: Infant, MESH: Case-Control Studies, MESH : Incidence, MESH : Paternal Exposure, 3. Good health, Oncology, risk factor, Maternal Exposure, Child, Preschool, 030220 oncology & carcinogenesis, Paternal Exposure, MESH : Case-Control Studies, medicine.medical_specialty, Alcohol Drinking, MESH: Probability, Risk Assessment, Article, MESH : Tobacco Smoke Pollution, 03 medical and health sciences, MESH : Adolescent, Humans, Risk factor, education, MESH: Age Distribution, Survival analysis, MESH: Adolescent, Chi-Square Distribution, MESH: Humans, MESH : Chi-Square Distribution, MESH : Humans, MESH: Child, Preschool, Public Health, Environmental and Occupational Health, Case-control study, Infant, MESH: Coffee, MESH : Follow-Up Studies, MESH: Adult, Odds ratio, medicine.disease, MESH : Coffee, Survival Analysis, MESH : Pregnancy, MESH : Alcohol Drinking, Logistic Models, Brain neoplasms, Case-Control Studies, Tobacco Smoke Pollution, MESH: Female, MESH: Alcohol Drinking, MESH : Logistic Models, Cancer Research, tea, MESH : Age Distribution, MESH : Child, Preschool, Epidemiology, MESH : Female, 030212 general & internal medicine, MESH : Risk Assessment, MESH: Maternal Exposure, MESH : Sex Distribution, Incidence (epidemiology), MESH: Tea, MESH : Adult, MESH: Survival Analysis, MESH: Tobacco Smoke Pollution, Female, epidemiology, France, Adult, Adolescent, MESH : Male, MESH : Probability, Population, coffee, MESH : Reference Values, smoking, Age Distribution, medicine, Sex Distribution, MESH : France, Probability, MESH: Paternal Exposure, business.industry, MESH: Male, MESH: France, [SDV.SPEE] Life Sciences [q-bio]/Santé publique et épidémiologie, [SDV.SPEE]Life Sciences [q-bio]/Santé publique et épidémiologie, MESH : Survival Analysis, business, Follow-Up Studies
Abstract: International audience; Parental smoking and maternal alcohol and caffeinated beverage consumption are prevalent exposures which may play a role, either directly or through their influence on metabolism, in the aetiology of childhood malignant central nervous system (CNS) tumours. The hypothesis was investigated in the Epidemiological Study on childhood Cancer and Leukemia ESCALE study, a national population-based case-control study carried out in France in 2003-2004. The study included 209 incident cases of CNS tumours and 1681 population-based controls, frequency matched with the cases by age and sex. The data were collected through a standardized telephone interview of the biological mothers. No association between maternal smoking during pregnancy and CNS tumours [odds ratio (OR): 1.1 (0.8-1.6)] was observed. Paternal smoking during the year before birth was associated with CNS tumours (P for trend=0.04), particularly astrocytomas [OR: 3.1 (1.3-7.6)]. Maternal alcohol consumption during pregnancy was not associated with CNS tumours. Associations between ependymomas and the highest consumption of coffee [OR: 2.7 (0.9-8.1)] and tea [OR: 2.5 (1.1-5.9)] were observed. A strong association between CNS tumours and the highest maternal consumption of both coffee and tea during pregnancy was observed [OR: 4.4 (1.5-13)]. The results constitute additional evidence for a role of paternal smoking and suggest that maternal coffee and tea consumption during pregnancy may also increase the risk of CNS tumours. The study does not suggest an increased risk of CNS tumours related to alcohol consumption during pregnancy.
Published: 2008
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32. Optimization of biologics to reduce treatment failure in inflammatory bowel diseases

Author: Anne Breton, Frédérick Barreau, Emmanuel Mas, Cyrielle Gilletta De Saint-Joseph, Aurélie Bourchany, Unité de Gastroentérologie, Hépatologie, Nutrition, Diabétologie et Maladies Héréditaires du Métabolisme, Hôpital des Enfants, CHU de Toulouse, Département de Gastroentérologie, Hôpital Rangueil, CHU de Toulouse, Institut de Recherche en Santé Digestive (IRSD ), Université Toulouse III - Paul Sabatier (UT3), Université Fédérale Toulouse Midi-Pyrénées-Université Fédérale Toulouse Midi-Pyrénées-Ecole Nationale Vétérinaire de Toulouse (ENVT), Institut National Polytechnique (Toulouse) (Toulouse INP), Université Fédérale Toulouse Midi-Pyrénées-Université Fédérale Toulouse Midi-Pyrénées-Institut National Polytechnique (Toulouse) (Toulouse INP), Université Fédérale Toulouse Midi-Pyrénées-Institut National de la Santé et de la Recherche Médicale (INSERM)-Institut National de Recherche pour l’Agriculture, l’Alimentation et l’Environnement (INRAE), CCSD, Accord Elsevier, Service Diabétologie [CHU Toulouse], Pôle Cardiovasculaire et Métabolique [CHU Toulouse], Centre Hospitalier Universitaire de Toulouse (CHU Toulouse)-Centre Hospitalier Universitaire de Toulouse (CHU Toulouse), Service de Gastroentérologie et pancréatologie [CHU Toulouse], and Pôle Maladies de l'appareil digestif [CHU Toulouse]
Subjects: 0301 basic medicine, Drug, medicine.medical_specialty, media_common.quotation_subject, Drug Resistance, Drug resistance, Antibodies, Monoclonal, Humanized, 030226 pharmacology & pharmacy, Inflammatory bowel disease, law.invention, Efficacy, 03 medical and health sciences, 0302 clinical medicine, Gastrointestinal Agents, Randomized controlled trial, law, Drug Discovery, medicine, Humans, Treatment Failure, Intensive care medicine, media_common, Pharmacology, Biological Products, [SDV.MHEP] Life Sciences [q-bio]/Human health and pathology, medicine.diagnostic_test, Tumor Necrosis Factor-alpha, business.industry, Inflammatory Bowel Diseases, medicine.disease, 3. Good health, 030104 developmental biology, Therapeutic drug monitoring, Monoclonal, Trough level, Ustekinumab, Drug Monitoring, business, [SDV.MHEP]Life Sciences [q-bio]/Human health and pathology
Abstract: International audience; Moderate to severe inflammatory bowel disease patients can fail to respond to conventional therapy and/or to biologic treatment. In the era of TNFα antagonists and other non-anti-TNF biologic drugs, it is important to review the literature on biologic treatment failure, which could be defined as primary non-response, secondary loss of response and intolerance. Therapeutic drug monitoring (TDM), that is, drug trough level and antidrug antibodies, should enable to determine the mechanisms of treatment failure and to optimize drug efficacy. There is a consensus on reactive TDM at the time of loss of response. Proactive TDM could be of interest during induction and/or maintenance, but randomized controlled trials are required.
Published: 2020
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33. Etiological assessment of status epilepticus

Author: Jonathan Curot, C. Hein, Alain Viguier, C. Hachon Le Camus, Marie Denuelle, Marie Benaiteau, Luc Valton, F. Rulquin, Centre Hospitalier Universitaire de Toulouse (CHU Toulouse), Centre de recherche cerveau et cognition (CERCO), Institut des sciences du cerveau de Toulouse. (ISCT), Université Toulouse - Jean Jaurès (UT2J)-Université Toulouse III - Paul Sabatier (UT3), Université Fédérale Toulouse Midi-Pyrénées-Université Fédérale Toulouse Midi-Pyrénées-Centre Hospitalier Universitaire de Toulouse (CHU Toulouse)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Centre National de la Recherche Scientifique (CNRS)-Université Toulouse - Jean Jaurès (UT2J)-Université Toulouse III - Paul Sabatier (UT3), Université Fédérale Toulouse Midi-Pyrénées-Université Fédérale Toulouse Midi-Pyrénées-Centre Hospitalier Universitaire de Toulouse (CHU Toulouse)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Centre National de la Recherche Scientifique (CNRS)-Centre National de la Recherche Scientifique (CNRS), CCSD, Accord Elsevier, Hôpital Pierre-Paul Riquet [Toulouse], CHU Toulouse [Toulouse], Hôpital Purpan [Toulouse], Université Fédérale Toulouse Midi-Pyrénées-Université Fédérale Toulouse Midi-Pyrénées-CHU Toulouse [Toulouse]-Institut National de la Santé et de la Recherche Médicale (INSERM)-Centre National de la Recherche Scientifique (CNRS)-Université Toulouse - Jean Jaurès (UT2J)-Université Toulouse III - Paul Sabatier (UT3), Université Fédérale Toulouse Midi-Pyrénées-Université Fédérale Toulouse Midi-Pyrénées-CHU Toulouse [Toulouse]-Institut National de la Santé et de la Recherche Médicale (INSERM)-Centre National de la Recherche Scientifique (CNRS)-Centre National de la Recherche Scientifique (CNRS), and Hôpital des Enfants
Subjects: Adult, medicine.medical_specialty, [SDV]Life Sciences [q-bio], Diagnostic Techniques, Neurological, Status epilepticus, Diagnosis, Differential, 03 medical and health sciences, Epilepsy, Status Epilepticus, 0302 clinical medicine, Pregnancy, Orientation (mental), medicine, Humans, 030212 general & internal medicine, Child, Intensive care medicine, Pathological, Aged, Autoimmune encephalitis, business.industry, medicine.disease, 3. Good health, Discontinuation, [SDV] Life Sciences [q-bio], Neurology, Etiology, Female, Neurology (clinical), medicine.symptom, business, 030217 neurology & neurosurgery, Encephalitis
Abstract: Status epilepticus (SE) is a potentially serious condition that can affect vital and functional prognosis and requires urgent treatment. Etiology is a determining factor in the patient's functional outcome and in almost half of all cases justifies specific treatment to stop progression. Therefore, identifying and addressing the cause of SE is a key priority in SE management. However, the etiology can be difficult to identify among acute and remote causes, which can also be multiple and interrelated. The most common etiologies are the discontinuation of antiepileptic medication in patients with a prior history of epilepsy, and acute brain aggression in cases of new onset SE (cerebrovascular pathologies are the most common). The list of remaining possible etiologies includes heterogeneous pathological contexts. Refractory SE and especially New-Onset Refractory Status Epilepticus (NORSE) lead to an extension of the etiological assessment in the search for encephalitis of autoimmune or infectious origin in adults and in children, as well as a genetic pathology in children in particular. This is an overview of current knowledge of SE etiologies and a pragmatic approach for carrying out an etiological assessment based on the following steps: – Which etiological orientation is identified according to the field and clinical presentation?; – Which etiologies to look for in an inaugural SE?; – Which first-line assessment should be carried out? The place of the biological, EEG and imaging assessment is discussed; – Which etiologies to look for in case of refractory SE?
Published: 2020
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34. Recessive PRDM13 mutations cause fatal perinatal brainstem dysfunction with cerebellar hypoplasia and disrupt Purkinje cells differentiation

Author: Marion Coolen, Nami Altin, Karthyayani Rajamani, Eva Pereira, Karine Siquier-Pernet, Emilia Puig Lombardi, Nadjeda Moreno, Giulia Barcia, Marianne Yvert, Annie Laquerrière, Aurore Pouliet, Patrick Nitschké, Nathalie Boddaert, Antonio Rausell, Féréchté Razavi, Alexandra Afenjar, Thierry Billette de Villemeur, Almundher Al-Maawali, Khalid Al-Thihli, Julia Baptista, Ana Beleza-Meireles, Catherine Garel, Marine Legendre, Antoinette Gelot, Lydie Burglen, Sébastien Moutton, Vincent Cantagrel, Imagine - Institut des maladies génétiques (IHU) (Imagine - U1163), Institut National de la Santé et de la Recherche Médicale (INSERM)-Université Paris Cité (UPCité), Great Ormond Street Institute of Child Health (UCL), University College of London [London] (UCL), Maison de Santé Protestante de Bordeaux-Bagatelle (MSPB), Université de Rouen Normandie (UNIROUEN), Normandie Université (NU), URC, hôpital Necker Enfants Maladies, Paris, CHU Trousseau [APHP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU), Sorbonne Université (SU), Sultan Qaboos University (SQU), Peninsula Medical School, University Hospitals Bristol, Hôpital des Enfants - Groupe hospitalier Pellegrin - CHU de Bordeaux, Institut de Neurobiologie de la Méditerranée [Aix-Marseille Université] (INMED - INSERM U1249), Aix Marseille Université (AMU)-Institut National de la Santé et de la Recherche Médicale (INSERM), and ANR-16-CE12-0005,SCD-Mec,Mécanismes développementaux des anomalies structurelles cérébelleuses(2016)
Subjects: Brain Diseases, Developmental Disabilities, Neurogenesis, Histone-Lysine N-Methyltransferase, Nervous System Malformations, Article, Mice, Purkinje Cells, Cerebellum, Mutation, Genetics, Animals, Humans, [SDV.NEU]Life Sciences [q-bio]/Neurons and Cognition [q-bio.NC], Genetics (clinical), Zebrafish, ComputingMilieux_MISCELLANEOUS, Brain Stem, Transcription Factors
Abstract: Pontocerebellar hypoplasias (PCHs) are congenital disorders characterized by hypoplasia or early atrophy of the cerebellum and brainstem, leading to a very limited motor and cognitive development. Although over 20 genes have been shown to be mutated in PCHs, a large proportion of affected individuals remains undiagnosed. We describe four families with children presenting with severe neonatal brainstem dysfunction and pronounced deficits in cognitive and motor development associated with four different bi-allelic mutations in PRDM13, including homozygous truncating variants in the most severely affected individuals. Brain MRI and fetopathological examination revealed a PCH-like phenotype, associated with major hypoplasia of inferior olive nuclei and dysplasia of the dentate nucleus. Notably, histopathological examinations highlighted a sparse and disorganized Purkinje cell layer in the cerebellum. PRDM13 encodes a transcriptional repressor known to be critical for neuronal subtypes specification in the mouse retina and spinal cord but had not been implicated, so far, in hindbrain development. snRNA-seq data mining and in situ hybridization in humans show that PRDM13 is expressed at early stages in the progenitors of the cerebellar ventricular zone, which gives rise to cerebellar GABAergic neurons, including Purkinje cells. We also show that loss of function of prdm13 in zebrafish leads to a reduction in Purkinje cells numbers and a complete absence of the inferior olive nuclei. Altogether our data identified bi-allelic mutations in PRDM13 as causing a olivopontocerebellar hypoplasia syndrome and suggest that early deregulations of the transcriptional control of neuronal fate specification could contribute to a significant number of cases.
Published: 2022
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35. Diagnosing undernutrition children and adults: new French criteria. Why, for what and for whom? A joint statement of the French National Authority for Health and French Federation of Nutrition – Corrigendum

Author: Delarue, Jacques, Desport, Jean-Claude, Dubern, Béatrice, Joly, Francisca, Mas, Emmanuel, Pitard, Alexandre, Fontaine, Eric, Département de Nutrition (Dep Nutrition - BREST), Centre Hospitalier Régional Universitaire de Brest (CHRU Brest), Epidémiologie des Maladies Chroniques en zone tropicale (EpiMaCT), CHU Limoges-Institut d'Epidémiologie Neurologique et de Neurologie Tropicale-Institut National de la Santé et de la Recherche Médicale (INSERM)-OmégaHealth (ΩHealth), Université de Limoges (UNILIM)-Université de Limoges (UNILIM), Faculté de Médecine [Limoges], Université de Limoges (UNILIM), Department of Nutrition, Pitie-Salpetrière hospital (AP-HP), Sorbonne University, CRNH-Ile de France, Service de Gastroentérologie et d’Assistance nutritive [AP-HP Hôpital Beaujon], Université Paris Diderot - Paris 7 (UPD7)-Hôpital Beaujon [AP-HP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP), Unité de Gastroentérologie, Hépatologie, Nutrition, Diabétologie et Maladies Héréditaires du Métabolisme, Hôpital des Enfants, CHU de Toulouse, and Université Grenoble Alpes (UGA)
Subjects: [SDV.SPEE]Life Sciences [q-bio]/Santé publique et épidémiologie, ComputingMilieux_MISCELLANEOUS
Abstract: International audience
Published: 2022
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36. Anti-reflux surgery in children with congenital diaphragmatic hernia: A prospective cohort study on a controversial practice

Author: Louise Montalva, Elisabeth Carricaburu, Rony Sfeir, Virginie Fouquet, Naziha Khen-Dunlop, Frederic Hameury, Nicoleta Panait, Alexis Arnaud, Hubert Lardy, Françoise Schmitt, Christian Piolat, Frederic Lavrand, Quentin Ballouhey, Aurélien Scalabre, Erik Hervieux, Jean-Luc Michel, Isabelle Germouty, Philippe Buisson, Frederic Elbaz, Jean-Francois Lecompte, Thierry Petit, Audrey Guinot, Olivier Abbo, Emmanuel Sapin, François Becmeur, Dominique Forgues, Maguelonne Pons, Arnaud Fotso Kamdem, Nicolas Berte, Marie Auger-Hunault, Alexandra Benachi, Arnaud Bonnard, Centre de Référence des Maladies Endocriniennes Rares de la Croissance [APHP Robert Debré], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Hôpital Robert Debré-Université Paris Cité (UPCité), Service de chirurgie pédiatrique, AP-HP Hôpital universitaire Robert-Debré [Paris], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP), Institute for Translational Research in Inflammation - U 1286 (INFINITE (Ex-Liric)), Institut National de la Santé et de la Recherche Médicale (INSERM)-Université de Lille-Centre Hospitalier Régional Universitaire [Lille] (CHRU Lille), Hôpital Bicêtre, Service de chirurgie et urologie pédiatrique [CHU-Necker], CHU Necker - Enfants Malades [AP-HP], Hospices Civils de Lyon (HCL), Service de chirurgie pédiatrique [Rennes] = Paediatric / Pediatric surgery [Rennes], CHU Pontchaillou [Rennes], Unité de recherche Nutrition et Sécurité Alimentaire (LNSA), Institut National de la Recherche Agronomique (INRA), Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP), Service de chirurgie pédiatrique [CHU Angers], Centre Hospitalier Universitaire d'Angers (CHU Angers), PRES Université Nantes Angers Le Mans (UNAM)-PRES Université Nantes Angers Le Mans (UNAM), Hémodynamique, Interaction Fibrose et Invasivité tumorales Hépatiques (HIFIH), Université d'Angers (UA), Service de chirurgie pédiatrique [CHU Grenoble], Centre Hospitalier Universitaire [Grenoble] (CHU), Service de chirurgie pédiatrique [CHU Bordeaux], Hôpital des Enfants - Groupe hospitalier Pellegrin - CHU de Bordeaux, Service de Pédiatrie médicale [CHU Limoges], CHU Limoges, Centre Hospitalier Universitaire de Saint-Etienne [CHU Saint-Etienne] (CHU ST-E), CHU Trousseau [APHP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU), Centre Hospitalier Universitaire de Dijon - Hôpital François Mitterrand (CHU Dijon), CHU Amiens-Picardie, Hôpitaux Pédiatriques de Nice Lenval (CHU-Lenval), and Centre Hospitalier Universitaire de Nice (CHU Nice)
Subjects: Cohort Studies, [SDV]Life Sciences [q-bio], Pediatrics, Perinatology and Child Health, Infant, Newborn, Humans, Infant, Fundoplication, Surgery, General Medicine, Prospective Studies, Child, Hernias, Diaphragmatic, Congenital, Failure to Thrive
Abstract: Gastro-esophageal reflux disease (GERD) is the most frequent long-term morbidity of congenital diaphragmatic hernia (CDH) survivors. Performing a preventive fundoplication during CDH repair remains controversial. This study aimed to: (1) Analyze the variability in practices regarding preventive fundoplication; (2) Identify predictive factors for fundoplication. (3) Evaluate the impact of preventive fundoplication on gastro-intestinal outcomes in children with a CDH patch repair; METHODS: This prospective multi-institutional cohort study (French CDH Registry) included CDH neonates born in France between January 1st, 2010-December 31st, 2018. Patch CDH was defined as need for synthetic patch or muscle flap repair. Main outcome measures included need for curative fundoplication, tube feed supplementation, failure to thrive, and oral aversion.Of 762 CDH neonates included, 81 underwent fundoplication (10.6%), either preventive or curative. Median follow-up was 3.0 years (IQR: 1.0-5.0). (1) Preventive fundoplication is considered in only 31% of centers. The rates of both curative fundoplication (9% vs 3%, p = 0.01) and overall fundoplication (20% vs 3%, p 0.0001) are higher in centers that perform preventive fundoplication compared to those that do not. (2) Predictive factors for preventive fundoplication were: prenatal diagnosis (p = 0.006), intra-thoracic liver (p = 0.005), fetal tracheal occlusion (p = 0.002), CDH-grade C-D (p 0.0001), patch repair (p 0.0001). After CDH repair, 8% (n = 51) required curative fundoplication (median age: 101 days), for which a patch repair was the only independent predictive factors identified upon multivariate analysis. (3) In neonates with patch CDH, preventive fundoplication did not decrease the need for curative fundoplication (15% vs 11%, p = 0.53), and was associated with higher rates of failure to thrive (discharge: 81% vs 51%, p = 0.03; 6-months: 81% vs 45%, p = 0.008), tube feeds (6-months: 50% vs 21%, p = 0.02; 2-years: 65% vs 26%, p = 0.004), and oral aversion (6-months: 67% vs 37%, p = 0.02; 1-year: 71% vs 40%, p = 0.03).Children undergoing a CDH patch repair are at high risk of requiring a curative fundoplication. However, preventive fundoplication during a patch repair does not decrease the need for curative fundoplication and is associated with worse gastro-intestinal outcomes in children.II - Prospective Study.
Published: 2022
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37. Joint involvement in Noonan syndrome. A retrospective paediatric descriptive study

Author: Quellec, Aurore Le, Edouard, Thomas, Audebert-Bellanger, Séverine, Pouzet, Antoine, Bourdet, Karine, Colson, Cindy, Oriot, Charlotte, Poignant, Sylvaine, Saraux, Alain, Devauchelle-Pensec, Valérie, Le Quellec, Aurore, CHRU Brest - Service de Rhumatologie (CHU - BREST - Rhumato), Centre Hospitalier Régional Universitaire de Brest (CHRU Brest), Service d'endocrinologie pédiatrique [CHU Hôpital des Enfants, Toulouse], CHU Toulouse [Toulouse], Département de génétique médicale en pédiatrie [CHRU Brest], CHRU de Brest - Département de Pédiatrie (CHU BREST Pédiatrie), Caen University Hospital, Department of Genetics, F 14000, Normandy Center for Genomic and Personalized Medicine, Caen, France, Département de pédiatrie [CHU Nantes], Centre hospitalier universitaire de Nantes (CHU Nantes), Département de génétique médicale en pédiatrie [CHRU Nantes], Lymphocyte B et Auto-immunité (LBAI), Université de Brest (UBO)-Institut Brestois Santé Agro Matière (IBSAM), and Université de Brest (UBO)-Institut National de la Santé et de la Recherche Médicale (INSERM)
Subjects: musculoskeletal diseases, Pediatrics, medicine.medical_specialty, Heart disease, Synovitis, Pigmented Villonodular, Joint involvement, Central giant cell granuloma, 03 medical and health sciences, 0302 clinical medicine, Rheumatology, Intellectual disability, medicine, Humans, Lupus Erythematosus, Systemic, Child, Retrospective Studies, 0303 health sciences, Synovitis, Pigmented villonodular synovitis, business.industry, 030305 genetics & heredity, Noonan Syndrome, Genetic disorder, Retrospective cohort study, medicine.disease, 3. Good health, RASopathy, Cohort, Noonan syndrome, [SDV.IMM]Life Sciences [q-bio]/Immunology, Villonodular synovitis, business, 030217 neurology & neurosurgery
Abstract: Noonan syndrome is a rare genetic disorder characterized mainly by congenital heart disease, occasional intellectual disability, and varied orthopaedic, rheumatological and haematologic anomalies. Despite potentially serious functional consequences, joint involvement has been rarely studied in the literature. Our objective was to perform a retrospective study evaluating the prevalence and characteristics of joint involvement in Noonan syndrome.We recorded articular symptoms, including their type and frequency, in patients with Noonan syndrome followed up in French hospitals. Patients were included if the diagnosis was confirmed before the age of 20 based on the van der Burgt criteria or genetic analysis. Data are presented as frequencies or medians (ranges), and patient groups were compared using chi-square or Fisher tests.Seventy-one patients were included from 4 centres. The average age was 12.5 years (range: 2-36). Musculoskeletal pain was found in 18 patients (25%) and joint stiffness in 10 (14%) located in the wrists, elbows, ankles, knees and hips, which was usually bilateral. Only one destructive form was described (multiple villonodular synovitis and a giant cell lesion of the jaw). There were no cases of systemic lupus erythaematosus (SLE) or other autoimmune arthritis. Raynaud's phenomenon was observed in 3 patients. Only 50% of joint complaints led to additional exploration. SOS1 mutations (P0.05) and treatment with growth hormone (GH) (P0.05) were the only factors significantly related to musculoskeletal pain. Patients treated with GH did not have more SOS1 mutations. Patients experiencing pain were not more likely to experience stiffness, joint hypermobility, or coagulation abnormalities.Joint manifestations were frequent in Noonan syndrome, predominant in large joints, and rarely explored. Multiple villonodular synovitis is characteristic but rare. Auto-immune disorders were not described in this cohort. A more multidisciplinary approach could be recommended for the early detection of possibly disabling rheumatologic manifestations.
Published: 2022
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38. A novel LARGE1-AFF2 fusion expanding the molecular alterations associated with the methylation class of neuroepithelial tumors with PATZ1 fusions

Author: Tauziède-Espariat, Arnault, Chotard, Guillaume, Le Loarer, François, Baud, Jessica, Azmani, Rihab, Dangouloff-Ros, Volodia, Boddaert, Nathalie, Icher-De-Bouyn, Céline, Gimbert, Edouard, Hasty, Lauren, Métais, Alice, Chrétien, Fabrice, Varlet, Pascale, GHU Paris Psychiatrie et Neurosciences, Institut de psychiatrie et neurosciences de Paris (IPNP - U1266 Inserm), Institut National de la Santé et de la Recherche Médicale (INSERM)-Université Paris Cité (UPCité), Service de pathologie [Bordeaux], Université Bordeaux Segalen - Bordeaux 2-CHU Bordeaux [Bordeaux]-Groupe hospitalier Pellegrin, Actions for OnCogenesis understanding and Target Identification in ONcology (ACTION), Institut Bergonié [Bordeaux], UNICANCER-UNICANCER-Université Bordeaux Segalen - Bordeaux 2-Institut National de la Santé et de la Recherche Médicale (INSERM), Département de Biopathologie (Institut Bergonié - CRLCC Bordeaux), UNICANCER-UNICANCER, Imagine - Institut des maladies génétiques (IHU) (Imagine - U1163), Département d'oncologie pédiatrique [CHU Bordeaux], Hôpital des Enfants - Groupe hospitalier Pellegrin - CHU de Bordeaux, CHU Bordeaux [Bordeaux], the RENOCLIP-LOC, and Martinez Rico, Clara
Subjects: Oncogene Proteins, Fusion, Brain Neoplasms, PATZ1, Kruppel-Like Transcription Factors, Nuclear Proteins, [SDV.CAN]Life Sciences [q-bio]/Cancer, Case Report, AFF2, N-Acetylglucosaminyltransferases, Neoplasms, Neuroepithelial, Neuroepithelial tumor, Repressor Proteins, [SDV.CAN] Life Sciences [q-bio]/Cancer, Child, Preschool, Humans, [SDV.NEU]Life Sciences [q-bio]/Neurons and Cognition [q-bio.NC], Female, [SDV.NEU] Life Sciences [q-bio]/Neurons and Cognition [q-bio.NC], LARGE1
Abstract: A novel DNA methylation class of tumor within the central nervous system, the "neuroepithelial tumor (NET), PATZ1 fusion-positive” has recently been identified in the literature, characterized by EWSR1- and MN1-PATZ1 fusions. The cellular origin of this tumor type remains unknown, wavering between glioneuronal or mesenchymal (as round cell sarcomas with EWSR1-PATZ1 of the soft tissue). Because of the low number of reported cases, this tumor type will not be added to the 2021 World Health Organization Classification of Tumors of the Central Nervous System (CNS). Herein, we report one case of a CNS tumor classified by DNA methylation analysis as NET-PATZ1 but harboring a novel LARGE1-AFF2 fusion which has until now never been described in soft tissue or the CNS. We compare its clinical, histopathological, immunophenotypical, and genetic features with those previously described in NET-PATZ1. Interestingly, the current case presented histopathological (astroblastoma-like features, glioneuronal phenotype), clinical (with a favorable course), genetic (1p loss), and epigenetic (DNA-methylation profiling) similarities to previously reported cases of NET-PATZ1. Our results added data suggesting that different histomolecular tumor subtypes seem to be included within the methylation class “NET, PATZ1 fusion-positive”, including non PATZ1 fusions, and that further cases are needed to better characterize them. Supplementary Information The online version contains supplementary material available at 10.1186/s40478-022-01317-8.
Published: 2021
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39. Multi-Omics Analysis of Gut Microbiota in Inflammatory Bowel Diseases: What Benefits for Diagnostic, Prognostic and Therapeutic Tools?

Author: Frédérick Barreau, Emmanuel Mas, Alexis Cassard, Vickie Lacroix, CHU Toulouse, Hôpital des Enfants, Unité de Gastroentérologie, Hépatologie et Nutrition, Département de Pédiatrie, Hôpital Purpan [Toulouse], CHU Toulouse [Toulouse]-CHU Toulouse [Toulouse], CHU Toulouse [Toulouse], Institut de Recherche en Santé Digestive (IRSD ), Université Toulouse III - Paul Sabatier (UT3), Université Fédérale Toulouse Midi-Pyrénées-Université Fédérale Toulouse Midi-Pyrénées-Ecole Nationale Vétérinaire de Toulouse (ENVT), Institut National Polytechnique (Toulouse) (Toulouse INP), Université Fédérale Toulouse Midi-Pyrénées-Université Fédérale Toulouse Midi-Pyrénées-Institut National Polytechnique (Toulouse) (Toulouse INP), Université Fédérale Toulouse Midi-Pyrénées-Institut National de la Santé et de la Recherche Médicale (INSERM)-Institut National de Recherche pour l’Agriculture, l’Alimentation et l’Environnement (INRAE), DGOS (Reference Center of Rare Digestive Diseases of Toulouse University Hospital), the patient association François Aupetit, Service Gastroentérologie, hépatologie nutrition, diabétologie et maladies héréditaires du métabolisme pédiatrique [CHU Toulouse], Pôle Enfants [CHU Toulouse], Centre Hospitalier Universitaire de Toulouse (CHU Toulouse)-Centre Hospitalier Universitaire de Toulouse (CHU Toulouse), Centre Hospitalier Universitaire de Toulouse (CHU Toulouse), and SEGUIN, Nathalie
Subjects: Crohn’s disease, [SDV.BIO]Life Sciences [q-bio]/Biotechnology, [SDV.MHEP.PHY] Life Sciences [q-bio]/Human health and pathology/Tissues and Organs [q-bio.TO], Review, Disease, Gut flora, Bioinformatics, Inflammatory bowel disease, 0302 clinical medicine, A, E, Precision Medicine, Biology (General), Phylogeny, Spectroscopy, 0303 health sciences, Crohn's disease, [SDV.MHEP] Life Sciences [q-bio]/Human health and pathology, biology, Microbiota, General Medicine, Ulcerative colitis, 3. Good health, Computer Science Applications, Chemistry, Mas, Disease Progression, 030211 gastroenterology & hepatology, F. Multi-Omics Analysis of Gut Microbiota in Inflammatory Bowel Diseases: What Benefits for Diagnostic, QH301-705.5, Omics, digestive system, eulcerative colitis, Catalysis, Inorganic Chemistry, 03 medical and health sciences, medicine, [SDV.MHEP.PHY]Life Sciences [q-bio]/Human health and pathology/Tissues and Organs [q-bio.TO], Humans, Physical and Theoretical Chemistry, QD1-999, Molecular Biology, Barreau, 030304 developmental biology, Bacteria, business.industry, V, Organic Chemistry, Cassard, Lacroix, Inflammatory Bowel Diseases, medicine.disease, biology.organism_classification, [SDV.MP.BAC]Life Sciences [q-bio]/Microbiology and Parasitology/Bacteriology, digestive system diseases, Prognostic inflammatory bowel disease, [SDV.BIO] Life Sciences [q-bio]/Biotechnology, Gastrointestinal Microbiome, Crohn's diseas, Metagenomics, Personalized medicine, [SDV.MP.BAC] Life Sciences [q-bio]/Microbiology and Parasitology/Bacteriology, business, Dysbiosis, [SDV.MHEP]Life Sciences [q-bio]/Human health and pathology
Abstract: International audience; Inflammatory bowel diseases (IBDs), which include Crohn’s disease and ulcerative colitis, are multifactorial diseases that involve in particular a modification of the gut microbiota, known as dysbiosis. The initial sets of metataxonomic and metagenomic data first made it possible to approximate the microbiota profile in IBD. In addition, today the new ‘omics’ techniques have enabled us to draw up a functional and integrative map of the microbiota. The key concern in IBD is to develop biomarkers that allow us to assess the activity of the disease and predict the complications and progression, while also guiding the therapeutic care so as to develop personalized medicine. In this review, we present all of the latest discoveries on the microbiota provided by “omics” and we outline the benefits of these techniques in developing new diagnostic, prognostic and therapeutic tools.
Published: 2021
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40. Lessons from a large nationwide cohort of 350 children with ovarian mature teratoma: A study in favor of ovarian-sparing surgery

Author: Hortense Alliot, Sabine Irtan, Fanny Delehaye, Jérémie Rouger, Julien Rod, Claude Borionne, Anne Croue, Guillaume Podevin, Cécile Faure-Conter, Aurélien Scalabre, Agnès Wacrenier, Matthieu Peycelon, Solène Joseph, Quentin Ballouhey, Yann Chaussy, Louise Galmiche, Camille Duchesne, Frederic Lavrand, Jean-Jacques Parienti, Estelle Aubry, Sabine Sarnacki, Frédéric Hameury, Daniel Orbach, Isabelle Pommepuy, Marie Pierre Guibal, Alaa Cheikhelard, Florent Guérin, E. Habonimana, Elodie Haraux, Anne Dariel, Frédérique Dijoud, CHU Caen, Normandie Université (NU)-Tumorothèque de Caen Basse-Normandie (TCBN), CHU Necker - Enfants Malades [AP-HP], Assistance publique - Hôpitaux de Paris (AP-HP) (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), Service de pédiatrie, adolescents, jeunes adultes [Institut Curie], Institut Curie [Paris], Groupe de Recherche sur l'Adaptation Microbienne (GRAM 2.0), Université de Caen Normandie (UNICAEN), Normandie Université (NU)-Normandie Université (NU)-Université de Rouen Normandie (UNIROUEN), Normandie Université (NU), Service d'hémato-immuno-oncologie pédiatrique [Rouen], CHU Rouen, Hôpital Femme Mère Enfant [CHU - HCL] (HFME), Hospices Civils de Lyon (HCL), Environnement périnatal et croissance - EA 4489 (EPS), Université de Lille-Centre Hospitalier Régional Universitaire [Lille] (CHRU Lille), Institut de Pathologie [CHU Lille], Pôle de Biologie Pathologie Génétique [CHU Lille], Centre Hospitalier Régional Universitaire [Lille] (CHRU Lille)-Centre Hospitalier Régional Universitaire [Lille] (CHRU Lille), CHU Pontchaillou [Rennes], Immunogenic Cell Death and Mesothelioma Therapy (CRCINA-ÉQUIPE 4), 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), Centre Hospitalier Universitaire de Saint-Etienne [CHU Saint-Etienne] (CHU ST-E), Centre Hospitalier Régional Universitaire de Besançon (CHRU Besançon), Centre Hospitalier Universitaire d'Angers (CHU Angers), PRES Université Nantes Angers Le Mans (UNAM), Département de Pathologie Cellulaire et Tissulaire [CHU Angers] (Laboratoire d’Histopathologie-Cytopathologie), 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), CHU Amiens-Picardie, Périnatalité et Risques Toxiques - UMR INERIS_I 1 (PERITOX), Institut National de l'Environnement Industriel et des Risques (INERIS)-Université de Picardie Jules Verne (UPJV)-CHU Amiens-Picardie, Service d'Hématologie biologique [CHU Limoges], CHU Limoges, Service de Pédiatrie médicale [CHU Limoges], Service de chirurgie pédiatrique [CHU Bordeaux], Hôpital des Enfants - Groupe hospitalier Pellegrin - CHU de Bordeaux, Service de Chirurgie Viscerale et Urologie Pediatriques, Hôpital Robert Debré, Université Sorbonne Paris Cité (USPC), Maladies Endocriniennes Rares de la Croissance (CRMERC), Maladies génétiques d'expression pédiatrique (U933), Institut National de la Santé et de la Recherche Médicale (INSERM)-CHU Trousseau [APHP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU), Riley Children's Hospital at Indiana University Health, Indiana University School of Medicine, Indiana University System, Université Paris Diderot - Paris 7 (UPD7), CHU Trousseau [APHP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU), AP-HP Hôpital Bicêtre (Le Kremlin-Bicêtre), Centre hospitalier universitaire de Nantes (CHU Nantes), Service d'anatomie pathologique [CHU Necker], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-CHU Necker - Enfants Malades [AP-HP], Unité de recherche interdisciplinaire pour la prévention et le traitement des cancers (ANTICIPE), Normandie Université (NU)-Normandie Université (NU)-CHU Caen, Normandie Université (NU)-Tumorothèque de Caen Basse-Normandie (TCBN)-Tumorothèque de Caen Basse-Normandie (TCBN)-Centre Régional de Lutte contre le Cancer François Baclesse [Caen] (UNICANCER/CRLC), Normandie Université (NU)-UNICANCER-Tumorothèque de Caen Basse-Normandie (TCBN)-UNICANCER-Institut National de la Santé et de la Recherche Médicale (INSERM), Service de Pédiatrie Médicale [Caen], and Normandie Université (NU)-Tumorothèque de Caen Basse-Normandie (TCBN)-Tumorothèque de Caen Basse-Normandie (TCBN)
Subjects: medicine.medical_specialty, conservative surgery, Adolescent, media_common.quotation_subject, medicine.medical_treatment, [SDV]Life Sciences [q-bio], Ovariectomy, Fertility, Ovarian tumor, children, Laparotomy, medicine, Humans, mature teratoma, germ cell tumors, Laparoscopy, Child, media_common, Retrospective Studies, Ovarian Neoplasms, medicine.diagnostic_test, business.industry, Teratoma, Oophorectomy, Hematology, Perioperative, medicine.disease, Surgery, Oncology, Pediatrics, Perinatology and Child Health, Cohort, Female, Germ cell tumors, ovarian tumors, business
Abstract: International audience; Background Ovarian mature teratoma (OMT) is a common ovarian tumor found in the pediatric population. In 10%-20% of cases, OMT occurs as multiple synchronous or metachronous lesions on ipsi- or contralateral ovaries. Ovarian-sparing surgery (OSS) is recommended to preserve fertility, but total oophorectomy (TO) is still performed. Design This study reviews the clinical data of patients with OMT, and analyzes risk factors for second events. A national retrospective review of girls under 18 years of age with OMTs was performed. Data on clinical features, imaging, laboratory studies, surgical reports, second events and their management were retrieved. Results Overall, 350 children were included. Eighteen patients (5%) presented with a synchronous bilateral form at diagnosis. Surgery was performed by laparotomy (85%) and laparoscopy (15%). OSS and TO were performed in 59% and 41% of cases, respectively. Perioperative tumor rupture occurred in 23 cases, independently of the surgical approach. Twenty-nine second events occurred (8.3%) in a median time of 30.5 months from diagnosis (ipsilateral: eight cases including one malignant tumor; contralateral: 18 cases; both ovaries: three cases). A large palpable mass, bilateral forms, at diagnosis and perioperative rupture had a statistical impact on the risk of second event, whereas the type of surgery or approach did not. Conclusion This study is a plea in favor of OSS as the first-choice treatment of OMT when possible. Close follow-up during the first 5 years is mandatory considering the risk of 8.3% of second events, especially in cases with risk factors.
Published: 2021
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41. Recurrent bacterial infections, but not fungal infections, characterise patients with ELANE-related neutropenia: a French Severe Chronic Neutropenia Registry study

Author: Aude Marie-Cardine, Flore Sicre de Fontbrune, Stéphane Blanche, Despina Moushous, Thierry Leblanc, Christine Bellanné-Chantelot, Fanny Rialland, Nathalie Aladjidi, Claire Freycon, Virginie Gandemer, Catherine Paillard, Blandine Beaupain, Marlène Pasquet, French Severe Chronic Neutropenia Registry, Marie-Gabrielle Vigue, Wadih Abou-Chahla, Christophe Piguet, Frédéric Millot, Martin Biosse-Duplan, Pacifique Lévy, Cecile Renard, Jean Donadieu, Gioacchino Andrea Rotulo, Geneviève Plat, Vincent Barlogis, Claire Fieschi, Therapeia Rehabilitation Center, CHU Trousseau [APHP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU), Registre des neutropénies chroniques [CHU Trousseau], Service d'hématologie-immunologie-oncologie pédiatrique [CHU Trousseau], 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 Trousseau [APHP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU), Hôpital des Enfants, CHU Toulouse [Toulouse], CHU Necker - Enfants Malades [AP-HP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP), Hôpital Robert Debré, Assistance Publique - Hôpitaux de Marseille (APHM), Centre Hospitalier Universitaire [Grenoble] (CHU), CHU Pontchaillou [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 ), Centre hospitalier universitaire de Nantes (CHU Nantes), Centre hospitalier universitaire de Poitiers (CHU Poitiers), CHU Rouen, Normandie Université (NU), CHU Strasbourg, CHU Bordeaux [Bordeaux], Hôpital Bretonneau, Amgen SAS, Chugai SA, Inserm, Association Sportive de Saint-Quentin–Fallavier, Société d’Hémato-Immunologie Pédiatrique, 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), Centre Hospitalier Universitaire de Toulouse (CHU Toulouse), and 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 )
Subjects: Adult, medicine.medical_specialty, Neutropenia, Adolescent, severe congenital neutropenia, [SDV]Life Sciences [q-bio], Disease, medicine.disease_cause, 03 medical and health sciences, Cyclic neutropenia, Young Adult, 0302 clinical medicine, Recurrence, Internal medicine, medicine, Humans, Registries, Child, 030304 developmental biology, 0303 health sciences, ELANE-related neutropenia, business.industry, Elastase, Hematopoietic Stem Cell Transplantation, Genetic Variation, Infant, Hematology, Bacterial Infections, medicine.disease, opportunistic infections, 3. Good health, Transplantation, Pneumonia, Mycoses, Staphylococcus aureus, 030220 oncology & carcinogenesis, Cellulitis, France, business, Leukocyte Elastase, Follow-Up Studies
Abstract: International audience; Among 143 patients with elastase, neutrophil-expressed (ELANE)-related neutropenia enrolled in the French Severe Chronic Neutropenia Registry, 94 were classified as having severe chronic neutropenia (SCN) and 49 with cyclic neutropenia (CyN). Their infectious episodes were classified as severe, mild or oral, and analysed according to their natural occurrence without granulocyte-colony stimulating factor (G-CSF), on G-CSF, after myelodysplasia/acute leukaemia or after haematopoietic stem-cell transplantation. During the disease’s natural history period (without G-CSF; 1913 person-years), 302, 957 and 754 severe, mild and oral infectious events, respectively, occurred. Among severe infections, cellulitis (48%) and pneumonia (38%) were the most common. Only 38% of episodes were microbiologically documented. The most frequent pathogens were Staphylococcus aureus (37·4%), Escherichia coli (20%) and Pseudomonas aeruginosa (16%), while fungal infections accounted for 1%. Profound neutropenia (3000/mm(3) ) and neutropenia subtype were associated with high risk of infection. Only the p.Gly214Arg variant (5% of the patients) was associated with infections but not the overall genotype. The first year of life was associated with the highest infection risk throughout life. G-CSF therapy achieved lower ratios of serious or oral infectious event numbers per period but was less protective for patients requiring >10 µg/kg/day. Infections had permanent consequences in 33% of patients, most frequently edentulism.
Published: 2021
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42. Exposure to low-dose ionising radiation from cardiac catheterisation and risk of cancer: the COCCINELLE study cohort profile

Author: Abalo, Kossi Dovene, Malekzadeh-Milani, Sophie, Hascoët, Sébastien, Dreuil, Serge, Feuillet, Tiphaine, Cohen, Sarah, Dauphin, Claire, Filippo, Sylvie Di, Douchin, Stéphanie, Godart, François, Guérin, Patrice, Helms, Pauline, Karsenty, Clement, Lefort, Bruno, Mauran, Pierre, Ovaert, Caroline, Piéchaud, Jean-François, Thambo, Jean-Benoît, Leuraud, Klervi, Bonnet, Damien, Bernier, Marie-Odile, Rage, Estelle, Service d'épidémiologie [Fontenay aux Roses], Institut de Radioprotection et de Sûreté Nucléaire (IRSN), CHU Necker - Enfants Malades [AP-HP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP), Centre chirurgical Marie Lannelongue, CHU Gabriel Montpied [Clermont-Ferrand], CHU Clermont-Ferrand, Hôpital Louis Pradel [CHU - HCL], Hospices Civils de Lyon (HCL), Université Claude Bernard Lyon 1 (UCBL), Université de Lyon, Centre Hospitalier Universitaire [Grenoble] (CHU), Institut Coeur Poumon [CHU Lille], Centre Hospitalier Régional Universitaire [Lille] (CHRU Lille), Centre d’Investigation Clinique de Nantes (CIC Nantes), Université de Nantes (UN)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Centre hospitalier universitaire de Nantes (CHU Nantes), Centre hospitalier universitaire de Nantes (CHU Nantes), Université de Nantes (UN), CHU Strasbourg, Equipe 7 Inserm U1048, Institut des Maladies Métaboliques et Cardiovasculaires (I2MC), Institut National de la Santé et de la Recherche Médicale (INSERM)-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)-Université Toulouse III - Paul Sabatier (UT3), Université Fédérale Toulouse Midi-Pyrénées-Université Fédérale Toulouse Midi-Pyrénées, Université Fédérale Toulouse Midi-Pyrénées, Centre Hospitalier Régional Universitaire de Tours (CHRU TOURS), American Memorial Hospital (Hôpital des enfants) [Reims], Centre Hospitalier Universitaire de Reims (CHU Reims), Marseille medical genetics - Centre de génétique médicale de Marseille (MMG), Aix Marseille Université (AMU)-Institut National de la Santé et de la Recherche Médicale (INSERM), Hôpital de la Timone [CHU - APHM] (TIMONE), Aix Marseille Université (AMU), Hôpital Privé Jacques Cartier [Massy], CHU Bordeaux [Bordeaux], 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 Hospitalier Régional Universitaire de Tours (CHRU Tours), Institut National de la Santé et de la Recherche Médicale (INSERM)-Aix Marseille Université (AMU), Laboratoire d épidémiologie des rayonnements ionisants (IRSN/PSE-SANTE/SESANE/LEPID), Service de recherche sur les effets biologiques et Sanitaires des rayonnements ionisants (IRSN/PSE-SANTE/SESANE), Institut de Radioprotection et de Sûreté Nucléaire (IRSN)-Institut de Radioprotection et de Sûreté Nucléaire (IRSN), Centre Chirurgical Marie Lannelongue (CCML), Université de Toulouse (UT)-Université de Toulouse (UT)-Institut National de la Santé et de la Recherche Médicale (INSERM)-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), Université de Toulouse (UT), and dormoy, valerian
Subjects: Cardiac Catheterization, cancer pain, Epidemiology, paediatric cardiology, Incidence, [SDV]Life Sciences [q-bio], congenital heart disease, [SDV] Life Sciences [q-bio], Risk Factors, paediatric oncology, Neoplasms, Radiation, Ionizing, cardiology, Medicine, Humans, France, Child, Retrospective Studies
Abstract: International audience; Purpose The COCCINELLE study is a nationwide retrospective French cohort set up to evaluate the risk of cancer in patients who undergone cardiac catheterisation (CC) procedures for diagnosis or treatment of congenital heart disease during childhood. Participants Children who undergone CC procedures from 1 January 2000 to 31 December 2013, before the age of 16 in one of the 15 paediatric cardiology departments which perform paediatric CC in mainland France were included. The follow-up started at the date of the first recorded CC procedure until the exit date, that is, the date of death, the date of first cancer diagnosis, the date of the 18th birthday or the 31 December 2015, whichever occurred first. The cohort was linked to the National Childhood Cancer Registry to identify patients diagnosed with cancer and with the French National Directory for the Identification of Natural Persons to retrieve the patients’ vital status. Findings to date A total of 17 104 children were included in the cohort and followed for 110 335 person-years, with 22 227 CC procedures collected. Among the patients, 81.6% received only one procedure. Fifty-nine cancer cases were observed in the cohort. Standardised incidence ratios (SIRs) were increased for all-cancer (SIR=3.8, 95% CI: 2.9 to 4.9), leukaemia (SIR=3.3, 95% CI: 2.0 to 5.4), lymphoma (SIR=14.9, 95% CI: 9.9 to 22.5) and solid cancers excluding central nervous system (CNS) tumours (SIR=3.3, 95% CI: 2.0 to 5.5) compared with the general population. Future plans Dose reconstruction is currently underway to estimate individual cumulative doses absorbed to relevant organs, including red bone marrow and brain for respectively haematologic disorders and CNS tumours risk estimation. A dose–response analysis will be conducted with consideration to confounding factors such as age at exposure, gender, predisposing factors to cancer and other sources of medical diagnostic low-dose ionising radiation.
Published: 2021
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43. Pediatric Evans syndrome is associated with a high frequency of potentially damaging variants in immune genes

Author: Helder Fernandes, Guy Leverger, Vincent Barlogis, Yves Bertrand, Mohammed Zarhrate, Corinne Pondarré, E. Dore, Nathalie Cheikh, Elodie Colomb Bottollier, Caroline Thomas, Eric Jeziorski, Frédéric Rieux-Laucat, Fabienne Mazerolles, Y Perel, Capucine Picard, Pascale Blouin, Cécile Fourrage, Nicolas Garcelon, Aude Magerus-Chatinet, Marlène Pasquet, Sylvain Hanein, Benedicte Neven, Dominique Plantaz, Nathalie Aladjidi, Fanny Fouyssac, Thierry Leblanc, Jérémie Rosain, Alain Fischer, Marie-Claude Stolzenberg, Stéphane Ducassou, Sidonie Jacques, Frédéric Millot, Jérôme Hadjadj, Wadih Abou Chahla, Isabelle Pellier, Immunogenetics of pediatric autoimmune diseases (Equipe Inserm U1163), Imagine - Institut des maladies génétiques (IMAGINE - U1163), Université Paris Descartes - Paris 5 (UPD5)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Université Paris Descartes - Paris 5 (UPD5)-Institut National de la Santé et de la Recherche Médicale (INSERM), CIC Bordeaux, Université Bordeaux Segalen - Bordeaux 2-Institut National de la Santé et de la Recherche Médicale (INSERM), Centre de Référence National des Cytopénies Auto-immunes de l'Enfant (CEREVANCE), Service d'hématologie-immunologie-oncologie pédiatrique [CHU Trousseau], Université Pierre et Marie Curie - Paris 6 (UPMC)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-CHU Trousseau [APHP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU)-Sorbonne Université (SU), Université Paris Descartes - Paris 5 (UPD5)-Institut National de la Santé et de la Recherche Médicale (INSERM), CHU Necker - Enfants Malades [AP-HP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP), The Clinical Bioinformatics laboratory (Equipe Inserm U1163), Structure Fédérative de Recherche Necker (SFR Necker - UMS 3633 / US24), Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-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 Toulouse [Toulouse], Centre Hospitalier Régional Universitaire [Lille] (CHRU Lille), Service d'hématologie pédiatrique, Université de la Méditerranée - Aix-Marseille 2-Assistance Publique - Hôpitaux de Marseille (APHM)- Hôpital de la Timone [CHU - APHM] (TIMONE), Amélioration génétique et adaptation des plantes méditerranéennes et tropicales (UMR AGAP), Centre de Coopération Internationale en Recherche Agronomique pour le Développement (Cirad)-Institut National de la Recherche Agronomique (INRA)-Centre international d'études supérieures en sciences agronomiques (Montpellier SupAgro)-Institut national d’études supérieures agronomiques de Montpellier (Montpellier SupAgro), Hospices Civils de Lyon, Departement de Neurologie (HCL), Service d'Immuno-Hémato-Oncologie Pédiatrique, Centre Hospitalier Universitaire d'Angers (CHU Angers), PRES Université Nantes Angers Le Mans (UNAM)-PRES Université Nantes Angers Le Mans (UNAM), CHU Dijon, Centre Hospitalier Universitaire de Dijon - Hôpital François Mitterrand (CHU Dijon), Centre Hospitalier Régional Universitaire de Nancy (CHRU Nancy), Centre Hospitalier Régional Universitaire de Tours (CHRU Tours), Centre hospitalier universitaire de Nantes (CHU Nantes), Centre Hospitalier Régional Universitaire de Besançon (CHRU Besançon), CHU Clermont-Ferrand, 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), Centre Hospitalier Universitaire [Grenoble] (CHU), Hôpital Arnaud de Villeneuve [CHRU Montpellier], Centre Hospitalier Régional Universitaire [Montpellier] (CHRU Montpellier), Centre hospitalier universitaire de Poitiers (CHU Poitiers), Centre de Recherche des Cordeliers (CRC (UMR_S_1138 / U1138)), 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), Unité d'Hémato-Immunologie pédiatrique [Hôpital Robert Debré, Paris], Service d'Immuno-hématologie pédiatrique [Hôpital Robert Debré, Paris], Hôpital Robert Debré-Hôpital Robert Debré, Chaire Médecine expérimentale (A. Fischer), Collège de France (CdF (institution)), Rieux-Laucat, Frédéric, Centre Hospitalier Universitaire de Toulouse (CHU Toulouse), Collège de France - Chaire Médecine expérimentale (A. Fischer), Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Université Paris Descartes - Paris 5 (UPD5)-Centre National de la Recherche Scientifique (CNRS), Hôpital des Enfants, Institut National de la Recherche Agronomique (INRA)-Institut national d’études supérieures agronomiques de Montpellier (Montpellier SupAgro), Institut national d'enseignement supérieur pour l'agriculture, l'alimentation et l'environnement (Institut Agro)-Institut national d'enseignement supérieur pour l'agriculture, l'alimentation et l'environnement (Institut Agro)-Centre international d'études supérieures en sciences agronomiques (Montpellier SupAgro)-Centre de Coopération Internationale en Recherche Agronomique pour le Développement (Cirad), Institut national d'enseignement supérieur pour l'agriculture, l'alimentation et l'environnement (Institut Agro)-Institut national d'enseignement supérieur pour l'agriculture, l'alimentation et l'environnement (Institut Agro), Centre Hospitalier Régional Universitaire de Tours (CHRU TOURS), CHU Trousseau [APHP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU), École Pratique des Hautes Études (EPHE), Université Paris Descartes - Paris 5 (UPD5)-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)-Centre National de la Recherche Scientifique (CNRS), Université Pierre et Marie Curie - Paris 6 (UPMC)-Assistance publique - Hôpitaux de Paris (AP-HP) (APHP)-CHU Trousseau [APHP], 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)-Université Paris Descartes - Paris 5 (UPD5)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Centre National de la Recherche Scientifique (CNRS), Institut national d’études supérieures agronomiques de Montpellier (Montpellier SupAgro)-Institut National de la Recherche Agronomique (INRA)-Centre de Coopération Internationale en Recherche Agronomique pour le Développement (Cirad)-Centre international d'études supérieures en sciences agronomiques (Montpellier SupAgro), Centre Hospitalier Régional Universitaire [Besançon] (CHRU Besançon), Hôpital Arnaud de Villeneuve, 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), Hôpital Robert Debré, Hôpital Robert Debré-Centre Hospitalier Universitaire de Reims (CHU Reims), Collège de France (CDF), and Collège de France (CdF)
Subjects: Adult, Male, Evans syndrome, Adolescent, [SDV.IMM] Life Sciences [q-bio]/Immunology, [SDV]Life Sciences [q-bio], Immunology, [SDV.GEN.GH] Life Sciences [q-bio]/Genetics/Human genetics, medicine.disease_cause, Biochemistry, Genetic determinism, LRBA, Cohort Studies, Young Adult, 03 medical and health sciences, 0302 clinical medicine, Germline mutation, Humans, Medicine, Child, 030304 developmental biology, Genetic testing, Autoimmune disease, 0303 health sciences, Mutation, medicine.diagnostic_test, business.industry, Infant, Cell Biology, Hematology, medicine.disease, Thrombocytopenia, 3. Good health, [SDV] Life Sciences [q-bio], [SDV.GEN.GH]Life Sciences [q-bio]/Genetics/Human genetics, [SDV.IMM.IA]Life Sciences [q-bio]/Immunology/Adaptive immunology, Child, Preschool, [SDV.IMM.IA] Life Sciences [q-bio]/Immunology/Adaptive immunology, 030220 oncology & carcinogenesis, [SDV.IMM]Life Sciences [q-bio]/Immunology, Female, Anemia, Hemolytic, Autoimmune, Autoimmune hemolytic anemia, business
Abstract: Evans syndrome (ES) is a rare severe autoimmune disorder characterized by the combination of autoimmune hemolytic anemia and immune thrombocytopenia. In most cases, the underlying cause is unknown. We sought to identify genetic defects in pediatric ES (pES), based on a hypothesis of strong genetic determinism. In a national, prospective cohort of 203 patients with early-onset ES (median [range] age at last follow-up: 16.3 years ([1.2-41.0 years]) initiated in 2004, 80 nonselected consecutive individuals underwent genetic testing. The clinical data were analyzed as a function of the genetic findings. Fifty-two patients (65%) received a genetic diagnosis (the M+ group): 49 carried germline mutations and 3 carried somatic variants. Thirty-two (40%) had pathogenic mutations in 1 of 9 genes known to be involved in primary immunodeficiencies (TNFRSF6, CTLA4, STAT3, PIK3CD, CBL, ADAR1, LRBA, RAG1, and KRAS), whereas 20 patients (25%) carried probable pathogenic variants in 16 genes that had not previously been reported in the context of autoimmune disease. Lastly, no genetic abnormalities were found in the remaining 28 patients (35%, the M− group). The M+ group displayed more severe disease than the M− group, with a greater frequency of additional immunopathologic manifestations and a greater median number of lines of treatment. Six patients (all from the M+ group) died during the study. In conclusion, pES was potentially genetically determined in at least 65% of cases. Systematic, wide-ranging genetic screening should be offered in pES; the genetic findings have prognostic significance and may guide the choice of a targeted treatment.
Published: 2019
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44. The ESC-EORP EURO-ENDO (European Infective Endocarditis) registry

Author: Habib G., Lancellotti P., Erba P. -A., Sadeghpour A., Meshaal M., Sambola A., Furnaz S., Citro R., Ternacle J., Donal E., Cosyns B., Popescu B., Iung B., Prendergast B., Laroche C., Tornos P., Pazdernik M., Maggioni A., Gale C. P., Ali Tatar-Chentir N. N., Al-Mallah M., Astrom Aneq M., Athanassopoulos G., Badano L, Benyoussef S., Calderon Aranda E., Cardim N. M., Chan K. -L., Cruz I., Edvardsen T., Goliasch G., Hagendorff A., Hristova K., Kamp O., Kang D. -H., Kong W., Matskeplishvili S., Mirocevic M., Neskovic A. N., Plonska-Gosciniak E., Popescu B. A., Raissouni M., Ronderos R., Sade L. E., Sengupta S., Separovic-Hanzevacki J., Takeuchi M., Tucay E., Tude Rodrigues A. C., Varga A., Vaskelyte J., Yamagata K., Yiangou K., Zaky H., Granada I., Mahia M., Ressi S., Nacinovich F., Iribarren A., Fernandez Oses P., Avegliano G., Filipini E., Obregon R., Bangher M., Dho J., Cartasegna L., Plastino M. L., Novas V., Shigel C., Reyes G., De Santos M., Gastaldello N., Granillo Fernandez M., Potito M., Streitenberger G., Velazco P., Casabe J. H., Cortes C., Guevara E., Salmo F., Seijo M., Weidinger F., Heger M., Brooks R., Stollberger C., Ho C. -Y., Perschy L., Puskas L., Binder C., Rosenhek R., Schneider M., Winter M. -P., Hoffer E., Melissopoulou M., Lecoq E., Legrand D., Jacquet S., Massoz M., Pierard L., Marchetta S., Dulgheru R., Emal C. D., Oury C., Droogmans S., Kerkhove D., Plein D., Soens L., Weytjens C., Motoc A., Roosens B., Lemoine I., Rodrigus I., Paelinck B., Amsel B., Unger P., Konopnicki D., Beauloye C., Pasquet A., Vanoverschelde J. L., Pierard S., Vancraeynest D., Sinnaeve F., Andrade J. L., Staszko K., Dos Santos Monteiro R., Miglioranza M. H., Shuha D. L., Alcantara M., Cravo V., Fazzio L., Felix A., Iso M., Musa C., Siciliano A. P., Villaca Filho F., Rodrigues A., Vilela F., Braga J., Silva R., Rodrigues D., Silva L., Morhy S., Fischer C., Vieira M., Afonso T., Abreu J., Falcao S. N., Moises V. A., Gouvea A., Mancuso F. J., Souza A. C., Silva C. Y., Joao G., Abboud C. S., Bellio De Mattos Barretto R., Ramos A., Arnoni R., Assef J. E., Della Togna D. J., Bihan D. L., Miglioli L., Romero Oliveira A. P., Tadeu Magro Kroll R., Cortez D., Gelape C. L., Peirira Nunes M. D. C., De Abreu Ferrari T. C., Hay K., Le V., Page M., Poulin F., Sauve C., Serri K., Mercure C., Beaudoin J., Pibarot P., Sebag I. A., Rudski L. G., Ricafort G., Barsic B., Krajinovic V., Vargovic M., Lovric D., Reskovic-Luksic V., Vincelj J., Jaksic Jurinjak S., Yiannikourides V., Ioannides M., Pofaides C., Masoura V., Pudich J., Linhart A., Siranec M., Marek J., Blechova K., Kamenik M., Pelouch R., Coufal Z., Mikulica M., Griva M., Jancova E., Mikulcova M., Taborsky M., Precek J., Jecmenova M., Latal J., Widimsky J., Butta T., MacHacek S., Vancata R., Spinar J., Holicka M., Pow Chon Long F., Anzules N., Bajana Carpio A., Largacha G., Penaherrera E., Moreira D., Mahfouz E., Elsafty E., Soliman A., Zayed Y., Aboulenein J., Abdel-Hay M., Almaghraby A., Abdelnaby M., Ahmed M., Hammad B., Saleh Y., Zahran H., Elgebaly O., Saad A., Ali M., Zeid A., El Sharkawy R., Al Kholy A., Doss R., Osama D., Rizk H., Elmogy A., Mishriky M., Assayag P., El Hatimi S., Hubert S., Casalta J. -P., Gouriet F., Arregle F., Cammilleri S., Tessonnier L., Riberi A., Botelho-Nevers E., Gagneux-Brunon A., Pierrard R., Tulane C., Campisi S., Fuzellier J. -F., Detoc M., Mehalla T., Boutoille D., Lecompte A. S., Lefebvre M., Pattier S., Al Habash O., Asseray-Madani N., Biron C., Brochard J., Caillon J., Cueff C., Tourneau T. L., Lecomte R., Magali Michel M. M., Orain J., Delarue S., Bras M. L., Faucher J. -F., Aboyans V., Beeharry A., Durox H., Lacoste M., Magne J., Mohty D., David A., Pradel V., Sierra V., Neykova A., Bettayeb B., Elkentaoui S., Tzvetkov B., Landry G., Strady C., Ainine K., Baumard S., Brasselet C., Tassigny C., Valente-Pires V., Lefranc M., Hoen B., Lefevre B., Curlier E., Callier C., Fourcade N., Jobic Y., Ansard S., Berre R. L., Ven F. L., Pouliquen M. -C., Prat G., Roux P. L., Bouchart F., Savoure A., Alarcon C., Chapuzet C., Gueit I., Tribouilloy C., Bohbot Y., Peugnet F., Gun M., Duval X., Lescure X., Ilic-Habensus E., Sadoul N., Selton-Suty C., Alla F., Goehringer F., Huttin O., Chevalier E., Garcia R., Marcis V. L., Tattevin P., Flecher E., Revest M., Chirouze C., Bouiller K., Hustache-Mathieu L., Klopfenstein T., Moreau J., Fournier D., Brunel A. -S., Lim P., Oliver L., Moussafeur A., Chavanet P., Piroth L., Salmon-Rousseau A., Buisson M., Mahy S., Martins C., Gohier S., Axler O., Baumann F., Lebras S., Piper C., Guckel D., Borgermann J., Horstkotte D., Winkelmann E., Brockmeier B., Grey D., Nickenig G., Schueler R., Ozturk C., Stohr E., Hamm C., Walther T., Brandt R., Fruhauf A. -C., Hartung C. T., Hellner C., Wild C., Becker M., Hamada S., Kaestner W., Stangl K., Knebel F., Baldenhofer G., Brecht A., Dreger H., Isner C., Pfafflin F., Stegemann M., Zahn R., Fraiture B., Kilkowski C., Karcher A. -K., Klinger S., Tolksdorf H., Tousoulis D., Aggeli C., Sideris S., Venieri E., Sarri G., Tsiapras D., Armenis I., Koutsiari A., Floros G., Grassos C., Dragasis S., Rallidis L., Varlamos C., Michalis L., Naka K., Bechlioulis A., Kotsia A., Lakkas L., Pappas K., Papadopoulos C., Kiokas S., Lioni A., Misailidou S., Barbetseas J., Bonou M., Kapelios C., Tomprou I., Zerva K., Manolis A., Hamodraka E., Athanasiou D., Haralambidis G., Samaras H., Poulimenos L., Nagy A., Bartykowszki A., Gara E., Mungulmare K., Kasliwal R., Bansal M., Ranjan S., Bhan A., Kyavar M., Maleki M., Noohi Bezanjani F., Alizadehasl A., Boudagh S., Ghavidel A., Moradnejad P., Pasha H. R., Ghadrdoost B., Gilon D., Strahilevitz J., Wanounou M., Israel S., D'Agostino C., Colonna P., Michele L. D., Fumarola F., Stante M., Marchionni N., Scheggi V., Alterini B., Del Pace S., Stefano P., Sparano C., Ruozi N., Tenaglia R., Muraru D., Limbruno U., Cresti A., Baratta P., Solari M., Giannattasio C., Moreo A., Chiara B. D., Lopez Montero B., Musca F., Orcese C. A., Panzeri F., Spano F., Russo C. F., Alfieri O., Bonis M. D., Chiappetta S., Del Forno B., Ripa M., Scarpellini P., Tassan Din C., Castiglioni B., Pasciuta R., Carletti S., Ferrara D., Guffanti M., Iaci G., Lapenna E., Nisi T., Oltolini C., Busnardo E., Pajoro U., Agricola E., Meneghin R., Schiavi D., Piscione F., Benvenga R. M., Greco L., Soriente L., Radano I., Prota C., Bellino M., Vece D. D., Santini F., Salsano A., Olivieri G. M., Turrini F., Messora R., Tondi S., Olaru A., Agnoletto V., Grassi L., Leonardi C., Sansoni S., Del Ponte S., Actis Dato G. M., Martino A. D., Ohte N., Kikuchi S., Wakami K., Aonuma K., Seo Y., Ishizu T., MacHino-Ohtsuka T., Yamamoto M., Iida N., Nakajima H., Nakagawa Y., Izumi C., Amano M., Miyake M., Takahashi K., Shiojima I., Miyasaka Y., Maeba H., Suwa Y., Taniguchi N., Tsujimoto S., Kitai T., Ota M., Yuda S., Sasaki S., Hagiwara N., Yamazaki K., Ashihara K., Arai K., Saitou C., Saitou S., Suzuki G., Shibata Y., Watanabe N., Nishino S., Ashikaga K., Kuriyama N., Mahara K., Okubo T., Fujimaki H., Shitan H., Yamamoto H., Abe K., Terada M., Takanashi S., Sata M., Yamada H., Kusunose K., Saijo Y., Seno H., Yuichiro O., Onishi T., Sera F., Nakatani S., Mizuno H., Sengoku K., Park S. W., Eun Kyoung K., Ga Yeon L., Hwang J. -W., Jin-Oh C., Park S. -J., Sang-Chol L., Sung-A C., Jang S. Y., Heo R., Lee S., Song J. -M., Jung E., Plisiene J., Dambrauskaite A., Gruodyte G., Jonkaitiene R., Mizariene V., Atkocaityte J., Zvirblyte R., Sow R., Codreanu A., Staub T., Michaux C., De La Vega E. C. 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M., Zemedkun M., Alyavi B., Uzokov J., Cardiovascular Centre (CVC), Universidad de Cantabria, Habib, G, Lancellotti, P, Erba, P, Sadeghpour, A, Meshaal, M, Sambola, A, Furnaz, S, Citro, R, Ternacle, J, Donal, E, Cosyns, B, Popescu, B, Iung, B, Prendergast, B, Laroche, C, Tornos, P, Pazdernik, M, Maggioni, A, Gale, C, Ali Tatar-Chentir, N, Al-Mallah, M, Astrom Aneq, M, Athanassopoulos, G, Badano, L, Benyoussef, S, Calderon Aranda, E, Cardim, N, Chan, K, Cruz, I, Edvardsen, T, Goliasch, G, Hagendorff, A, Hristova, K, Kamp, O, Kang, D, Kong, W, Matskeplishvili, S, Mirocevic, M, Neskovic, A, Plonska-Gosciniak, E, Raissouni, M, Ronderos, R, Sade, L, Sengupta, S, Separovic-Hanzevacki, J, Takeuchi, M, Tucay, E, Tude Rodrigues, A, Varga, A, Vaskelyte, J, Yamagata, K, Yiangou, K, Zaky, H, Granada, I, Mahia, M, Ressi, S, Nacinovich, F, Iribarren, A, Fernandez Oses, P, Avegliano, G, Filipini, E, Obregon, R, Bangher, M, Dho, J, Cartasegna, L, Plastino, M, Novas, V, Shigel, C, Reyes, G, De Santos, M, Gastaldello, N, Granillo Fernandez, M, Potito, M, Streitenberger, G, Velazco, P, Casabe, J, Cortes, C, Guevara, E, Salmo, F, Seijo, M, Weidinger, F, Heger, M, Brooks, R, Stollberger, C, Ho, C, Perschy, L, Puskas, L, Binder, C, Rosenhek, R, Schneider, M, Winter, M, Hoffer, E, Melissopoulou, M, Lecoq, E, Legrand, D, Jacquet, S, Massoz, M, Pierard, L, Marchetta, S, Dulgheru, R, Emal, C, Oury, C, Droogmans, S, Kerkhove, D, Plein, D, Soens, L, Weytjens, C, Motoc, A, Roosens, B, Lemoine, I, Rodrigus, I, Paelinck, B, Amsel, B, Unger, P, Konopnicki, D, Beauloye, C, Pasquet, A, Vanoverschelde, J, Pierard, S, Vancraeynest, D, Sinnaeve, F, Andrade, J, Staszko, K, Dos Santos Monteiro, R, Miglioranza, M, Shuha, D, Alcantara, M, Cravo, V, Fazzio, L, Felix, A, Iso, M, Musa, C, Siciliano, A, Villaca Filho, F, Rodrigues, A, Vilela, F, Braga, J, Silva, R, Rodrigues, D, Silva, L, Morhy, S, Fischer, C, Vieira, M, Afonso, T, Abreu, J, Falcao, S, Moises, V, Gouvea, A, Mancuso, F, Souza, A, Silva, C, Joao, G, Abboud, C, Bellio De Mattos Barretto, R, Ramos, A, Arnoni, R, Assef, J, Della Togna, D, Bihan, D, Miglioli, L, Romero Oliveira, A, Tadeu Magro Kroll, R, Cortez, D, Gelape, C, Peirira Nunes, M, De Abreu Ferrari, T, Hay, K, Le, V, Page, M, Poulin, F, Sauve, C, Serri, K, Mercure, C, Beaudoin, J, Pibarot, P, Sebag, I, Rudski, L, Ricafort, G, Barsic, B, Krajinovic, V, Vargovic, M, Lovric, D, Reskovic-Luksic, V, Vincelj, J, Jaksic Jurinjak, S, Yiannikourides, V, Ioannides, M, Pofaides, C, Masoura, V, Pudich, J, Linhart, A, Siranec, M, Marek, J, Blechova, K, Kamenik, M, Pelouch, R, Coufal, Z, Mikulica, M, Griva, M, Jancova, E, Mikulcova, M, Taborsky, M, Precek, J, Jecmenova, M, Latal, J, Widimsky, J, Butta, T, Machacek, S, Vancata, R, Spinar, J, Holicka, M, Pow Chon Long, F, Anzules, N, Bajana Carpio, A, Largacha, G, Penaherrera, E, Moreira, D, Mahfouz, E, Elsafty, E, Soliman, A, Zayed, Y, Aboulenein, J, Abdel-Hay, M, Almaghraby, A, Abdelnaby, M, Ahmed, M, Hammad, B, Saleh, Y, Zahran, H, Elgebaly, O, Saad, A, Ali, M, Zeid, A, El Sharkawy, R, Al Kholy, A, Doss, R, Osama, D, Rizk, H, Elmogy, A, Mishriky, M, Assayag, P, El Hatimi, S, Hubert, S, Casalta, J, Gouriet, F, Arregle, F, Cammilleri, S, Tessonnier, L, Riberi, A, Botelho-Nevers, E, Gagneux-Brunon, A, Pierrard, R, Tulane, C, Campisi, S, Fuzellier, J, Detoc, M, Mehalla, T, Boutoille, D, Lecompte, A, Lefebvre, M, Pattier, S, Al Habash, O, Asseray-Madani, N, Biron, C, Brochard, J, Caillon, J, Cueff, C, Tourneau, T, Lecomte, R, Magali Michel, M, Orain, J, Delarue, S, Bras, M, Faucher, J, Aboyans, V, Beeharry, A, Durox, H, Lacoste, M, Magne, J, Mohty, D, David, A, Pradel, V, Sierra, V, Neykova, A, Bettayeb, B, Elkentaoui, S, Tzvetkov, B, Landry, G, Strady, C, Ainine, K, Baumard, S, Brasselet, C, Tassigny, C, Valente-Pires, V, Lefranc, M, Hoen, B, Lefevre, B, Curlier, E, Callier, C, Fourcade, N, Jobic, Y, Ansard, S, Berre, R, Ven, F, Pouliquen, M, Prat, G, Roux, P, Bouchart, F, Savoure, A, Alarcon, C, Chapuzet, 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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)-Institut National de la Santé et de la Recherche Médicale (INSERM), Service de Chirurgie Cardiovasculaire, University Hospital of Cruces, Geneva University Hospital (HUG), Institut d'Investigacions Biomèdiques August Pi i Sunyer (IDIBAPS), Universitat de Barcelona (UB), Preventive Medicine Unit, University Hospital Joan XXIII, IISPV, Rovira and Virgili University, Habib, G., Lancellotti, P., Erba, P. -A., Sadeghpour, A., Meshaal, M., Sambola, A., Furnaz, S., Citro, R., Ternacle, J., Donal, E., Cosyns, B., Popescu, B., Iung, B., Prendergast, B., Laroche, C., Tornos, P., Pazdernik, M., Maggioni, A., Gale, C. 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H., Cortes, C., Guevara, E., Salmo, F., Seijo, M., Weidinger, F., Heger, M., Brooks, R., Stollberger, C., Ho, C. -Y., Perschy, L., Puskas, L., Binder, C., Rosenhek, R., Schneider, M., Winter, M. -P., Hoffer, E., Melissopoulou, M., Lecoq, E., Legrand, D., Jacquet, S., Massoz, M., Pierard, L., Marchetta, S., Dulgheru, R., Emal, C. D., Oury, C., Droogmans, S., Kerkhove, D., Plein, D., Soens, L., Weytjens, C., Motoc, A., Roosens, B., Lemoine, I., Rodrigus, I., Paelinck, B., Amsel, B., Unger, P., Konopnicki, D., Beauloye, C., Pasquet, A., Vanoverschelde, J. L., Pierard, S., Vancraeynest, D., Sinnaeve, F., Andrade, J. L., Staszko, K., Dos Santos Monteiro, R., Miglioranza, M. H., Shuha, D. L., Alcantara, M., Cravo, V., Fazzio, L., Felix, A., Iso, M., Musa, C., Siciliano, A. P., Villaca Filho, F., Rodrigues, A., Vilela, F., Braga, J., Silva, R., Rodrigues, D., Silva, L., Morhy, S., Fischer, C., Vieira, M., Afonso, T., Abreu, J., Falcao, S. N., Moises, V. A., Gouvea, A., Mancuso, F. J., Souza, A. C., Silva, C. Y., Joao, G., Abboud, C. S., Bellio De Mattos Barretto, R., Ramos, A., Arnoni, R., Assef, J. E., Della Togna, D. J., Bihan, D. L., Miglioli, L., Romero Oliveira, A. P., Tadeu Magro Kroll, R., Cortez, D., Gelape, C. L., Peirira Nunes, M. D. C., De Abreu Ferrari, T. C., Hay, K., Le, V., Page, M., Poulin, F., Sauve, C., Serri, K., Mercure, C., Beaudoin, J., Pibarot, P., Sebag, I. A., Rudski, L. 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L., Tattevin, P., Flecher, E., Revest, M., Chirouze, C., Bouiller, K., Hustache-Mathieu, L., Klopfenstein, T., Moreau, J., Fournier, D., Brunel, A. -S., Lim, P., Oliver, L., Moussafeur, A., Chavanet, P., Piroth, L., Salmon-Rousseau, A., Buisson, M., Mahy, S., Martins, C., Gohier, S., Axler, O., Baumann, F., Lebras, S., Piper, C., Guckel, D., Borgermann, J., Horstkotte, D., Winkelmann, E., Brockmeier, B., Grey, D., Nickenig, G., Schueler, R., Ozturk, C., Stohr, E., Hamm, C., Walther, T., Brandt, R., Fruhauf, A. -C., Hartung, C. 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R., Ghadrdoost, B., Gilon, D., Strahilevitz, J., Wanounou, M., Israel, S., D'Agostino, C., Colonna, P., Michele, L. D., Fumarola, F., Stante, M., Marchionni, N., Scheggi, V., Alterini, B., Del Pace, S., Stefano, P., Sparano, C., Ruozi, N., Tenaglia, R., Muraru, D., Limbruno, U., Cresti, A., Baratta, P., Solari, M., Giannattasio, C., Moreo, A., Chiara, B. D., Lopez Montero, B., Musca, F., Orcese, C. A., Panzeri, F., Spano, F., Russo, C. F., Alfieri, O., De Bonis, M., Chiappetta, S., Del Forno, B., Ripa, M., Scarpellini, P., Tassan Din, C., Castiglioni, B., Pasciuta, R., Carletti, S., Ferrara, D., Guffanti, M., Iaci, G., Lapenna, E., Nisi, T., Oltolini, C., Busnardo, E., Pajoro, U., Agricola, E., Meneghin, R., Schiavi, D., Piscione, F., Benvenga, R. M., Greco, L., Soriente, L., Radano, I., Prota, C., Bellino, M., Vece, D. D., Santini, F., Salsano, A., Olivieri, G. M., Turrini, F., Messora, R., Tondi, S., Olaru, A., Agnoletto, V., Grassi, L., Leonardi, C., Sansoni, S., Del Ponte, S., Actis Dato, G. M., Martino, A. D., Ohte, N., Kikuchi, S., Wakami, K., Aonuma, K., Seo, Y., Ishizu, T., MacHino-Ohtsuka, T., Yamamoto, M., Iida, N., Nakajima, H., Nakagawa, Y., Izumi, C., Amano, M., Miyake, M., Takahashi, K., Shiojima, I., Miyasaka, Y., Maeba, H., Suwa, Y., Taniguchi, N., Tsujimoto, S., Kitai, T., Ota, M., Yuda, S., Sasaki, S., Hagiwara, N., Yamazaki, K., Ashihara, K., Arai, K., Saitou, C., Saitou, S., Suzuki, G., Shibata, Y., Watanabe, N., Nishino, S., Ashikaga, K., Kuriyama, N., Mahara, K., Okubo, T., Fujimaki, H., Shitan, H., Yamamoto, H., Abe, K., Terada, M., Takanashi, S., Sata, M., Yamada, H., Kusunose, K., Saijo, Y., Seno, H., Yuichiro, O., Onishi, T., Sera, F., Nakatani, S., Mizuno, H., Sengoku, K., Park, S. W., Eun Kyoung, K., Ga Yeon, L., Hwang, J. -W., Jin-Oh, C., Park, S. -J., Sang-Chol, L., Sung-A, C., Jang, S. Y., Heo, R., Lee, S., Song, J. -M., Jung, E., Plisiene, J., Dambrauskaite, A., Gruodyte, G., Jonkaitiene, R., Mizariene, V., Atkocaityte, J., Zvirblyte, R., Sow, R., Codreanu, A., Staub, T., Michaux, C., De La Vega, E. C. L., Jacobs-Orazi, L., Mallia Azzopardi, C., Xuereb, R. G., Piscopo, T., Farrugia, J., Fenech, M., Pllaha, E., Vella, C., Borg, D., Casha, R., Grib, L., Raevschi, E., Grejdieru, A., Kravcenco, D., Prisacari, E., Samohvalov, E., Samohvalov, S., Sceglova, N., Panfile, E., Cardaniuc, L., Corcea, V., Feodorovici, A., Gaina, V., Girbu, L., Jimbei, P., Balan, G., Cardaniuc, I., Benesco, I., Marian, V., Sumarga, N., Bozovic, B., Bulatovic, N., Lakovic, P., Music, L., Budde, R., Wahadat, A., Gamela, T., Meijers, T., Van Melle, J. P., Deursen, V. M., Crijns, H. J., Bekkers, S. C., Cheriex, E. C., Gilbers, M., Kietselaer, B. L., Knackstedt, C., Lorusso, R., Schalla, S., Streukens, S. A., Chamuleau, S., Cramer, M. -J., Teske, A., Van Der Spoel, T., Wind, A., Lokhorst, J., Liesbek, O., Van Heusden, H., Tanis, W., Van Der Bilt, I., Vriend, J., Lange-Van Bruggen, H. D., Karijodikoro, E., Riezebos, R., Van Dongen, E., Schoep, J., Stolk, V., Offstad, J. T., Beitnes, J. O., Helle-Valle, T., Skulstad, H., Skardal, R., Qamar, N., Ahmed, B., Butt, M. H., Khanzada, M. F., Saghir, T., Wahid, A., Hryniewiecki, T., Szymanski, P., Marzec, K., Misztal-Ogonowska, M., Kosmala, W., Przewlocka-Kosmala, M., Rojek, A., Woznicka, K., Zachwyc, J., Lisowska, A., Kaminska, M., Kasprzak, J. D., Kowalczyk, E., Strzecka, D. F., Wejner-Mik, P., Trabulo, M., Freitas, P., Ranchordas, S., Rodrigues, G., Pinto, P., Queiros, C., Azevedo, J., Marques, L., Seabra, D., Branco, L., Cruz, M., Galrinho, A., Moreira, R., Rio, P., Timoteo, A. T., Selas, M., Carmelo, V., Duque Neves, B., Pereira, H., Guerra, A., Marques, A., Pintassilgo, I., Tomescu, M. C., Trofenciuc, N. -M., Andor, M., Bordejevic, A., Branea, H. S., Caruntu, F., Velcean, L. A., Mavrea, A., Onel, M. F., Parvanescu, T., Pop, D., Pop-Moldovan, A. L., Puticiu, M. I., Cirin, L., Citu, I. M., Cotoraci, C. A., Darabantiu, D., Farcas, R., Marincu, I., Ionac, A., Cozma, D., Mornos, C., Goanta, F., Popescu, I., Beyer, R., Mada, R., Rancea, R., Tomoaia, R., Rosianu, H., Stanescu, C., Kobalava, Z., Karaulova, J., Kotova, E., Milto, A., Pisaryuk, A., Povalyaev, N., Sorokina, M., Alrahimi, J., Elshiekh, A., Jamiel, A., Ahmed, A., Attia, N., Putnikovic, B., Dimic, A., Ivanovic, B., Matic, S., Trifunovic, D., Petrovic, J., Kosevic, D., Stojanovic, I., Petrovic, I., Dabic, P., Milojevic, P., Srdanovic, I., Susak, S., Velicki, L., Vulin, A., Kovacevic, M., Redzek, A., Stefanovic, M., Yeo, T. C., Kong, W. K. F., Poh, K. K., Vilacosta, I., Ferrera, C., Olmos, C., Abd El-Nasser, M., Calvo Iglesias, F., Blanco-Gonzalez, E., Bravo Amaro, M., Lopez-Rodriguez, E., Lugo Adan, J., Germinas, A. N., Pazos-Lopez, P., Pereira Loureiro, M., Perez, M. T., Raposeiras-Roubin, S., Rasheed Yas, S., Suarez-Varela, M. -M., Vasallo Vidal, F., Garcia-Dorado, D., Fernandez-Hidalgo, N., Gonzalez-Alujas, T., Lozano, J., Maisterra, O., Pizzi, N., Rios, R., Bayes-Genis, A., Pedro Botet, L., Vallejo, N., Llibre, C., Mateu, L., Nunez, R., Quesada, D., Berastegui, E., Bosch Portell, D., Aboal Vinas, J., Albert Bertran, X., Brugada Tarradellas, R., Loma-Osorio Ricon, P., Tiron De Llano, C., Arnau, M. A., Bel, A., Blanes, M., Osa, A., Anguita, M., Carrasco, F., Castillo, J. C., Zamorano, J. L., Moya Mur, J. L., Alvaro, M., Fernandez-Golfin, C., Monteagudo, J. M., Navas Elorza, E., Farinas Alvarez, M. C., Aguero Balbin, J., Zarauza, J., Gutierrez-Diez, J. F., Arminanzas, C., Arnaiz De Las Revillas, F., Arnaiz Garcia, A., Cobo Belaustegui, M., Fernandez Sampedro, M., Gutierrez Cuadra, M., Garcia Cuello, L., Gonzalez Rico, C., Rodriguez-Alvarez, R., Goikoetxea, J., Montejo, M., Miro, J. M., Almela, M., Ambrosioni, J., Moreno, A., Quintana, E., Sandoval, E., Tellez, A., Tolosana, J. M., Vidal, B., Falces, C., Fuster, D., Garcia-De-La-Maria, C., Hernandez-Meneses, M., Llopis, J., Marco, F., Ruiz-Zamora, I., Bardaji Ruiz, A., Sanz Girgas, E., Garcia-Pardo, G., Guillen Marzo, M., Rodriguez Oviedo, A., Villares Jimenez, A., Abid, L., Hammami, R., Kammoun, S., Mourali, M. S., Ben Hlima, M., Boudiche, S., Ouali, S., Zakhama, L., Antit, S., Slama, I., Gulel, O., Sahin, M., Karacaglar, E., Kucukoglu, S., Cetinarslan, O., Yasar, U. S., Canpolat, U., Mutlu, B., Atas, H., Dervishova, R., Ileri, C., Alhashmi, J., Tahir, J., Zarger, P., Baslib, F., Woldman, S., Menezes, L., Primus, C., Uppal, R., Bvekerwa, I., Chandrasekaran, B., Kopanska, A., Chambers, J., Hancock, J., Klein, J., Rajani, R., Ursi, M. P., Cannata, S., Dworakowski, R., Fife, A., Breeze, J., Browne-Morgan, M., Gunning, M., Streather, S., Asch, F. M., Zemedkun, M., Alyavi, B., Uzokov, J., RS: Carim - H01 Clinical atrial fibrillation, MUMC+: MA Cardiologie (9), Cardiologie, RS: CARIM - R3.11 - Imaging, RS: CARIM - R2.01 - Clinical atrial fibrillation, MUMC+: MA Med Staf Artsass CTC (9), Promovendi CD, Fysiologie, RS: CARIM - R1.06 - Genetic Epidemiology and Genomics of cardiovascular diseases, MUMC+: MA Med Staf Spec Cardiologie (9), RS: Carim - V04 Surgical intervention, CTC, MUMC+: MA Med Staf Spec CTC (9), RS: CARIM - R2.12 - Surgical intervention, RS: Carim - H02 Cardiomyopathy, and RS: CARIM - R2.02 - Cardiomyopathy
Subjects: medicine.medical_specialty, Registry, MEDLINE, Infective endocarditi, infective endocarditis, registry, valve disease, Disease, Infective endocarditis, Valve disease, 030204 cardiovascular system & hematology, 03 medical and health sciences, 0302 clinical medicine, Epidemiology, medicine, Journal Article, MANAGEMENT, Endocarditis, Humans, 030212 general & internal medicine, Registries, Intensive care medicine, health care economics and organizations, Surrogate endpoint, business.industry, Health Policy, Patient data, MED/11 - MALATTIE DELL'APPARATO CARDIOVASCOLARE, medicine.disease, 3. Good health, Cardiac surgery, Europe, Practice Guidelines as Topic, business, Cardiology and Cardiovascular Medicine, [SDV.MHEP]Life Sciences [q-bio]/Human health and pathology
Abstract: Aims The European Society of Cardiology (ESC) EURObservational Research Programme (EORP) European Endocarditis (EURO-ENDO) registry aims to study the care and outcomes of patients diagnosed with infective endocarditis (IE) and compare findings with recommendations from the 2015 ESC Clinical Practice Guidelines for the management of IE and data from the 2001 Euro Heart Survey. Methods and results Patients (n = 3116) aged over 18 years with a diagnosis of IE based on the ESC 2015 IE diagnostic criteria were prospectively identified between 1 January 2016 and 31 March 2018. Individual patient data were collected across 156 centres and 40 countries. The primary endpoint is all-cause mortality in hospital and at 1 year. Secondary endpoints are 1-year morbidity (all-cause hospitalization, any cardiac surgery, and IE relapse), the clinical, epidemiological, microbiological, and therapeutic characteristics of patients, the number and timing of non-invasive imaging techniques, and adherence to recommendations as stated in the 2015 ESC Clinical Practice Guidelines for the management of IE. Conclusion EURO-ENDO is an international registry of care and outcomes of patients hospitalized with IE which will provide insights into the contemporary profile and management of patients with this challenging disease.
Published: 2019
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45. ETP par application mobile auprès d'adolescent.es diabétiques : perceptions des professionnel.les de santé des effets sur la relation soignant.e-adolescent.e

Author: Charles, Anne-Elodie, Vansimaeys, Camille, Balagué, Christine, Colmel, Corinne, Le Tallec, Claire, Centre d’Etudes et de Recherches en Psychopathologie et Psychologie de la Santé (CERPPS), Université Toulouse - Jean Jaurès (UT2J), Laboratoire de Psychopathologie et Processus de Santé (LPPS (URP_4057)), Université de Paris (UP), Laboratoire en Innovation, Technologies, Economie et Management (EA 7363) (LITEM), Université d'Évry-Val-d'Essonne (UEVE)-Université Paris-Saclay-Institut Mines-Télécom Business School (IMT-BS), Institut Mines-Télécom [Paris] (IMT)-Institut Mines-Télécom [Paris] (IMT), Consommateur Connecté dans la Société Numérique (CONNECT), Département Droit, Economie et Finances (DEFI), Télécom Ecole de Management (TEM)-Institut Mines-Télécom [Paris] (IMT)-Institut Mines-Télécom Business School (IMT-BS), Institut Mines-Télécom [Paris] (IMT)-Télécom Ecole de Management (TEM)-Institut Mines-Télécom [Paris] (IMT)-Institut Mines-Télécom Business School (IMT-BS), Institut Mines-Télécom [Paris] (IMT)-Département Management, Marketing et Stratégie (MMS), Institut Mines-Télécom [Paris] (IMT), Département Management, Marketing et Stratégie (MMS), Enfance Adolescence & Diabète, Hôpital des Enfants, CHU Toulouse [Toulouse]-CHU Toulouse [Toulouse], LITEM-NPR, balague, Christine, Université de Toulouse (UT)-Université de Toulouse (UT)-Toulouse Mind & Brain Institut (TMBI), Université de Toulouse (UT)-Université de Toulouse (UT)-Université Toulouse III - Paul Sabatier (UT3), Université de Toulouse (UT)-Université Toulouse - Jean Jaurès (UT2J), Université de Toulouse (UT)-Université Toulouse III - Paul Sabatier (UT3), Université de Toulouse (UT), Université Paris Cité (UPCité), Département Droit, Economie et Finances (IMT-BS - DEFI), Institut Mines-Télécom [Paris] (IMT)-Département Management, Marketing et Stratégie (IMT-BS - MMS), Département Management, Marketing et Stratégie (IMT-BS - MMS), Association Enfance Adolescence & Diabète [Toulouse], Service Gastroentérologie, hépatologie nutrition, diabétologie et maladies héréditaires du métabolisme pédiatrique [CHU Toulouse], Pôle Enfants [CHU Toulouse], Centre Hospitalier Universitaire de Toulouse (CHU Toulouse)-Centre Hospitalier Universitaire de Toulouse (CHU Toulouse)-Pôle Enfants [CHU Toulouse], Centre Hospitalier Universitaire de Toulouse (CHU Toulouse)-Centre Hospitalier Universitaire de Toulouse (CHU Toulouse), Association Francophone de Psychologie de la Santé (AFPSA), and CHU Toulouse [Toulouse]
Subjects: Professionnel de santé, [STAT.AP]Statistics [stat]/Applications [stat.AP], [STAT.ME] Statistics [stat]/Methodology [stat.ME], Mhealth, Recherche qualitative, Diabète de type 1, [SHS]Humanities and Social Sciences, Relation soignant-soigné, [STAT.AP] Statistics [stat]/Applications [stat.AP], [SHS.GESTION]Humanities and Social Sciences/Business administration, [SHS] Humanities and Social Sciences, [SHS.GESTION] Humanities and Social Sciences/Business administration, application, [STAT.ME]Statistics [stat]/Methodology [stat.ME]
Abstract: International audience; Contexte : Il est peu abordé dans la littérature l’impact des technologies mobiles et applicatives sur la relation soignant.e-soigné.e, et la perception des professionnel.les de santé travaillant avec des adolescent.es vivant avec un de diabète de type 1. L’objectif de cette étude est de présenter le point de vue des professionnel.les de santé concernant les effets sur la relation soignant.e-soigné.e de l’usage d’une application mobile d’ETP auprès d’adolescents diabétiques. Méthode : Nous avons eu recours à un design d’étude qualitative afin de réaliser des entretiens semi-directifs auprès de 14 professionnel.les de santé (médecins, infirmières, diététiciennes, psychologue). Ces entretiens ont été enregistrés et retranscrits ad verbatim, puis analysés selon une méthode inductive. Résultats : L’analyse des entretiens fait ressortir trois thèmes : 1) les applications favorisent la construction et le renforcement de la relation soignant.e-soigné.e, 2) la conscience et la prise en compte du risque que peut engendrer la digitalisation sur la relation, 3) la démarche importante d’intégration du numérique tout en préservant la relation. Discussion : Les résultats viennent nourrir une réflexion plus générale au sujet du risque de déshumanisation du soin lié à la digitalisation des pratiques de soin et des prises en charge. La perspective des professionnel.les de santé, converge avec celle des patient.es décrite dans la littérature, notamment en ce qui concerne l’amélioration de la communication et la prise de décision partagée. Par conséquent, la digitalisation de l’ETP par l’usage d’application mobile semble compatible avec la préservation de la relation humaine essentielle au soin.
Published: 2021

46. Separation of the Ca V 1.2‐Ca V 1.3 calcium channel duo prevents type 2 allergic airway inflammation

Author: Joerg Striessnig, Marion Mars, Brice Ronsin, Magali Savignac, Marc Moreau, Jean-Charles Guéry, Lucette Pelletier, Marine Michelet, Nicolas Giang, Antoine Magnan, Geoffrey G. Murphy, José E. Mejía, Grégory Bouchaud, Institut Toulousain des Maladies Infectieuses et Inflammatoires (Infinity), 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 National de la Recherche Scientifique (CNRS), Centre de Physiopathologie Toulouse Purpan (CPTP), Centre de biologie du développement (CBD), Centre National de la Recherche Scientifique (CNRS)-Université Toulouse III - Paul Sabatier (UT3), Université Fédérale Toulouse Midi-Pyrénées-Université Fédérale Toulouse Midi-Pyrénées-Centre de Biologie Intégrative (CBI), Université Fédérale Toulouse Midi-Pyrénées-Université Fédérale Toulouse Midi-Pyrénées-Centre National de la Recherche Scientifique (CNRS)-Centre National de la Recherche Scientifique (CNRS), Unité de recherche sur les Biopolymères, Interactions Assemblages (BIA), Institut National de Recherche pour l’Agriculture, l’Alimentation et l’Environnement (INRAE), Institut du thorax, Université de Nantes (UN)-IFR26-Institut National de la Santé et de la Recherche Médicale (INSERM), CHU Toulouse, Hôpital des Enfants, Unité de Gastroentérologie, Hépatologie et Nutrition, Département de Pédiatrie, Hôpital Purpan [Toulouse], CHU Toulouse [Toulouse]-CHU Toulouse [Toulouse], Eastern Michigan University, Universität Innsbruck [Innsbruck], Leopold Franzens Universität Innsbruck - University of Innsbruck, Austrian Science Fund (FWF) : P27809, Foundation for Medical Research : DEQ20180339187, and French Society of Allergology : A11013BS.
Subjects: Calcium metabolism, Th2 lymphocytes, Voltage-dependent calcium channel, Calcium channel, T cell, Immunology, chemistry.chemical_element, Tyrosine phosphorylation, [SDV.MHEP.HEM]Life Sciences [q-bio]/Human health and pathology/Hematology, Calcium, asthma, Ca(v)1, cytokines, Cell biology, chemistry.chemical_compound, Calcium imaging, medicine.anatomical_structure, chemistry, Transcription (biology), calcium channels, cardiovascular system, medicine, Immunology and Allergy, [SDV.IMM.ALL]Life Sciences [q-bio]/Immunology/Allergology, signaling
Abstract: International audience; Background Voltage-gated calcium (Ca(v)1) channels contribute to T-lymphocyte activation. Ca(v)1.2 and Ca(v)1.3 channels are expressed in Th2 cells but their respective roles are unknown, which is investigated herein. Methods We generated mice deleted for Ca(v)1.2 in T cells or Ca(v)1.3 and analyzed TCR-driven signaling. In this line, we developed original fast calcium imaging to measure early elementary calcium events (ECE). We also tested the impact of Ca(v)1.2 or Ca(v)1.3 deletion in models of type 2 airway inflammation. Finally, we checked whether the expression of both Ca(v)1.2 and Ca(v)1.3 in T cells from asthmatic children correlates with Th2-cytokine expression. Results We demonstrated non-redundant and synergistic functions of Ca(v)1.2 and Ca(v)1.3 in Th2 cells. Indeed, the deficiency of only one channel in Th2 cells triggers TCR-driven hyporesponsiveness with weakened tyrosine phosphorylation profile, a strong decrease in initial ECE and subsequent reduction in the global calcium response. Moreover, Ca(v)1.3 has a particular role in calcium homeostasis. In accordance with the singular roles of Ca(v)1.2 and Ca(v)1.3 in Th2 cells, deficiency in either one of these channels was sufficient to inhibit cardinal features of type 2 airway inflammation. Furthermore, Ca(v)1.2 and Ca(v)1.3 must be co-expressed within the same CD4(+) T cell to trigger allergic airway inflammation. Accordingly with the concerted roles of Ca(v)1.2 and Ca(v)1.3, the expression of both channels by activated CD4(+) T cells from asthmatic children was associated with increased Th2-cytokine transcription. Conclusions Thus, Ca(v)1.2 and Ca(v)1.3 act as a duo, and targeting only one of these channels would be efficient in allergy treatment.
Published: 2021
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47. Does a combined screw and dowel construct improve tibial fixation during anterior cruciate ligament reconstruction?

Author: Meagan E. Tibbo, Dominique Barbier, Gregoire Laumond, Pauline Assemat, Franck Accadbled, Pierre Laumonerie, Pascal Swider, Centre National de la Recherche Scientifique - CNRS (FRANCE), Institut National Polytechnique de Toulouse - Toulouse INP (FRANCE), Université Toulouse III - Paul Sabatier - UT3 (FRANCE), Centre Hospitalier Universitaire de Bordeaux - CHU (FRANCE), Centre Hospitalier Universitaire de Toulouse - CHU Toulouse (FRANCE), Mayo Clinic (USA), Institut de Mécanique des Fluides de Toulouse - IMFT (Toulouse, France), CHU de Bordeaux Pellegrin [Bordeaux], Mayo Clinic [Rochester], CHU Toulouse, Hôpital des Enfants, Unité de Gastroentérologie, Hépatologie et Nutrition, Département de Pédiatrie, Hôpital Purpan [Toulouse], CHU Toulouse [Toulouse]-CHU Toulouse [Toulouse], Institut de mécanique des fluides de Toulouse (IMFT), Université Toulouse III - Paul Sabatier (UT3), Université Fédérale Toulouse Midi-Pyrénées-Université Fédérale Toulouse Midi-Pyrénées-Centre National de la Recherche Scientifique (CNRS)-Institut National Polytechnique (Toulouse) (Toulouse INP), and Université Fédérale Toulouse Midi-Pyrénées
Subjects: musculoskeletal diseases, Anterior cruciate ligament reconstruction, medicine.medical_treatment, Anterior cruciate ligament, Mécanique des fluides, Tibial fixation, Dowel, [SPI.MECA.MEFL]Engineering Sciences [physics]/Mechanics [physics.med-ph]/Fluids mechanics [physics.class-ph], 03 medical and health sciences, 0302 clinical medicine, Interference screw, medicine, Orthopedics and Sports Medicine, Displacement (orthopedic surgery), 030212 general & internal medicine, Fixation (histology), Orthodontics, 030222 orthopedics, business.industry, Pullout strength, Interference screws, equipment and supplies, musculoskeletal system, Biomechanical strength, medicine.anatomical_structure, surgical procedures, operative, Surgery, business
Abstract: International audience; Purpose: The aims of the present study were to compare the biomechanical properties of tibial fixation in hamstring-graft ACL reconstruction using interference screw and a novel combination interference screw and dowel construct. Material and methods: We compared the fixation of 30 (2- and 4-stranded gracilis and semitendinosis tendons) in 15 fresh-frozen porcine tibiae with a biocomposite resorbable interference screw (Group 1) and a screw and dowel construct (Group 2). Each graft was subjected to load-to-failure testing (50 mm/min) to determine maximum load, displacement at failure and pullout strength.Results: There were no significant differences between the biomechanical properties of the constructs. Multivariate analysis demonstrated that combination constructs (β = 140.20, p = 0.043), screw diameter (β = 185, p = 0.006) and 4-strand grafts (β = 51, p = 0.050) were associated with a significant increase in load at failure. Larger screw diameter was associated with increased construct stiffness (β = 20.15, p = 0.020).Conclusion: The screw and dowel construct led to significantly increased fixation properties compared to interference screws alone in a porcine model. Increased screw diameter and utilization of 4-strand ACL grafts also led to improvement in load-to-failure of the construct. However, this is an in vitro study and additional investigations are needed to determine whether the results are reproducible in vivo.Level of evidence: Level V; Biomechanical study.
Published: 2021
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48. How Can a Polymeric Formula Induce Remission in Crohn’s Disease Patients ?

Author: kawthar Boumessid, Frédérick Barreau, Emmanuel Mas, Institut de Recherche en Santé Digestive (IRSD ), Université Toulouse III - Paul Sabatier (UT3), Université Fédérale Toulouse Midi-Pyrénées-Université Fédérale Toulouse Midi-Pyrénées-Ecole Nationale Vétérinaire de Toulouse (ENVT), Institut National Polytechnique (Toulouse) (Toulouse INP), Université Fédérale Toulouse Midi-Pyrénées-Université Fédérale Toulouse Midi-Pyrénées-Institut National Polytechnique (Toulouse) (Toulouse INP), Université Fédérale Toulouse Midi-Pyrénées-Institut National de la Santé et de la Recherche Médicale (INSERM)-Institut National de Recherche pour l’Agriculture, l’Alimentation et l’Environnement (INRAE), Institut National de la Santé et de la Recherche Médicale (INSERM), CHU Toulouse, Hôpital des Enfants, Unité de Gastroentérologie, Hépatologie et Nutrition, Département de Pédiatrie, Hôpital Purpan [Toulouse], CHU Toulouse [Toulouse]-CHU Toulouse [Toulouse], Service Gastroentérologie, hépatologie nutrition, diabétologie et maladies héréditaires du métabolisme pédiatrique [CHU Toulouse], Pôle Enfants [CHU Toulouse], Centre Hospitalier Universitaire de Toulouse (CHU Toulouse)-Centre Hospitalier Universitaire de Toulouse (CHU Toulouse), and Raynaud, Christelle
Subjects: 0301 basic medicine, Crohn’s disease, medicine.medical_specialty, Crohn&#8217, Polymers, Lipid composition, Disease, Review, Inflammatory bowel disease, Gastroenterology, Models, Biological, Catalysis, Inorganic Chemistry, lcsh:Chemistry, s disease, mucosal healing, 03 medical and health sciences, Transforming Growth Factor beta2, 0302 clinical medicine, Crohn Disease, inflammatory bowel disease, Internal medicine, medicine, [SDV.BBM] Life Sciences [q-bio]/Biochemistry, Molecular Biology, Humans, [SDV.BBM]Life Sciences [q-bio]/Biochemistry, Molecular Biology, Dietary therapy, Palatability, Physical and Theoretical Chemistry, Intestinal Mucosa, Molecular Biology, lcsh:QH301-705.5, Spectroscopy, Crohn's disease, business.industry, exclusive enteral nutrition, Organic Chemistry, Remission Induction, General Medicine, medicine.disease, 3. Good health, Computer Science Applications, 030104 developmental biology, Parenteral nutrition, lcsh:Biology (General), lcsh:QD1-999, Mucosal healing, 030211 gastroenterology & hepatology, business
Abstract: International audience; Crohn’s disease is an inflammatory bowel disease whose prevalence is increasing worldwide. Among medical strategies, dietary therapy with exclusive enteral nutrition is recommended as a first-line option, at least for children, because it induces clinical remission and mucosal healing. Modulen®, a polymeric TGF-β2 enriched formula, has good palatability and is widely used. For the first time in the literature, this review outlines and discusses the clinical outcomes obtained with this therapy, as well as the potential mechanisms of action of its compounds. It can be explained by its TGF-β2 content, but also by its protein and lipid composition. Further well-designed studies are required to improve our knowledge and to optimize therapeutic strategies.
Published: 2021
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49. « Il n’y a pas de distance, il n’y a pas de virtuel » : regard des professionnels de santé sur les effets des applications mobiles d’ETP sur leur relation avec les adolescent.es vivant avec un diabète de type 1

Author: Vansimayes, Camille, Chomel, Corinne, Vansimaeys, Camille, Balagué, Christine, Charles, Anne-Elodie, Colmel, Corinne, Le Tallec, Claire, Laboratoire en Innovation, Technologies, Economie et Management (EA 7363) (LITEM), Université d'Évry-Val-d'Essonne (UEVE)-Université Paris-Saclay-Institut Mines-Télécom Business School (IMT-BS), Institut Mines-Télécom [Paris] (IMT)-Institut Mines-Télécom [Paris] (IMT), Laboratoire de Psychopathologie et Processus de Santé (LPPS (URP_4057)), Université Paris Cité (UPCité), Centre Hospitalier Universitaire de Toulouse (CHU Toulouse), Consommateur Connecté dans la Société Numérique (CONNECT), Département Droit, Economie et Finances (IMT-BS - DEFI), Télécom Ecole de Management (TEM)-Institut Mines-Télécom [Paris] (IMT)-Institut Mines-Télécom Business School (IMT-BS), Institut Mines-Télécom [Paris] (IMT)-Télécom Ecole de Management (TEM)-Institut Mines-Télécom [Paris] (IMT)-Institut Mines-Télécom Business School (IMT-BS), Institut Mines-Télécom [Paris] (IMT)-Département Management, Marketing et Stratégie (IMT-BS - MMS), Institut Mines-Télécom [Paris] (IMT), Département Management, Marketing et Stratégie (IMT-BS - MMS), Centre d’Etudes et de Recherches en Psychopathologie et Psychologie de la Santé (CERPPS), Université Toulouse - Jean Jaurès (UT2J), Université de Toulouse (UT)-Université de Toulouse (UT)-Toulouse Mind & Brain Institut (TMBI), Université de Toulouse (UT)-Université de Toulouse (UT)-Université Toulouse III - Paul Sabatier (UT3), Université de Toulouse (UT)-Université Toulouse - Jean Jaurès (UT2J), Université de Toulouse (UT)-Université Toulouse III - Paul Sabatier (UT3), Université de Toulouse (UT), LITEM-NPR, Université de Paris (UP), CHU Toulouse [Toulouse], Département Droit, Economie et Finances (DEFI), Institut Mines-Télécom [Paris] (IMT)-Département Management, Marketing et Stratégie (MMS), Département Management, Marketing et Stratégie (MMS), Hôpital des Enfants, Institut Mines-Télécom [Paris] (IMT)-Télécom Ecole de Management (TEM)-Institut Mines-Télécom Business School (IMT-BS), Institut Mines-Télécom [Paris] (IMT)-Institut Mines-Télécom [Paris] (IMT)-Télécom Ecole de Management (TEM)-Institut Mines-Télécom Business School (IMT-BS), and balague, Christine
Subjects: [STAT.ME] Statistics [stat]/Methodology [stat.ME], [SHS.GESTION]Humanities and Social Sciences/Business administration, [SHS] Humanities and Social Sciences, [SHS.GESTION] Humanities and Social Sciences/Business administration, [STAT.ME]Statistics [stat]/Methodology [stat.ME], [SHS]Humanities and Social Sciences
Abstract: International audience; Titre : « Il n'y a pas de distance, il n'y a pas de virtuel » : perceptions par les professionnel.les de santé des effets sur la relation soignant.e-soigné.e de l'utilisation d'une application mobile d'ETP auprès d'adolescents diabétiques.
Published: 2021

50. Association of Intravenous Immunoglobulins Plus Methylprednisolone vs Immunoglobulins Alone With Course of Fever in Multisystem Inflammatory Syndrome in Children

Author: Fanny Bajolle, Mathie Lorrot, Arnaud Wiedemann, Marion Grimaud, Slimane Allali, Fouad Madhi, Mehdi Oualha, Ulrich Meinzer, Noémie Lachaume, Corinne Levy, Jeanne Bordet, Pierre-Louis Leger, Alexandre Belot, Caroline Ovaert, Etienne Javouhey, Julie Toubiana, Marie-Laure Girardin, Alexis Mandelcwajg, Robert Cohen, Blandine Biot, François Angoulvant, Denise Antona, Caroline Galeotti, David D. Yang, Ana-Maria Paulet, Naim Ouldali, Jean-Emmanuel Kahn, Michael Levy, Caroline Claude, Morgane Dumortier, Lucas Percheron, Constance Beyler, Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP), Association Clinique et Thérapeutique Infantile du Val de Marne (ACTIV), Epidémiologie Clinique et Evaluation Economique Appliquées aux Populations Vulnérables (ECEVE (U1123 / UMR_S_1123)), Institut National de la Santé et de la Recherche Médicale (INSERM)-AP-HP Hôpital universitaire Robert-Debré [Paris], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Université Paris Cité (UPCité), Biodiversité et Epidémiologie des Bactéries pathogènes - Biodiversity and Epidemiology of Bacterial Pathogens, Institut Pasteur [Paris] (IP), Santé publique France - French National Public Health Agency [Saint-Maurice, France], Hospices Civils de Lyon (HCL), Physiopathologie de l'immunodépression associée aux réponses inflammatoires systémiques / Pathophysiology of Injury-induced Immunosuppression (PI3), Université Claude Bernard Lyon 1 (UCBL), Université de Lyon-Université de Lyon, Centre Hospitalier Intercommunal de Créteil (CHIC), Centre Hospitalier Universitaire de Nancy (CHU Nancy), Nutrition-Génétique et Exposition aux Risques Environnementaux (NGERE), Institut National de la Santé et de la Recherche Médicale (INSERM)-Université de Lorraine (UL), Assistance Publique - Hôpitaux de Marseille (APHM), Marseille medical genetics - Centre de génétique médicale de Marseille (MMG), Aix Marseille Université (AMU)-Institut National de la Santé et de la Recherche Médicale (INSERM), Centre hospitalier universitaire de Nantes (CHU Nantes), Université de Versailles Saint-Quentin-en-Yvelines (UVSQ), Hôpital Delafontaine, Centre Hospitalier de Saint-Denis [Ile-de-France], Centre Hospitalier Universitaire de Toulouse (CHU Toulouse), Centre hospitalier de Valence, CHU Strasbourg, Centre de recherche sur l'Inflammation (CRI (UMR_S_1149 / ERL_8252 / U1149)), Institut National de la Santé et de la Recherche Médicale (INSERM)-Centre National de la Recherche Scientifique (CNRS)-Université Paris Cité (UPCité), Hôpital Nord Franche-Comté [Hôpital de Trévenans] (HNFC), Centre de Recherche des Cordeliers (CRC (UMR_S_1138 / U1138)), École Pratique des Hautes Études (EPHE), Université Paris sciences et lettres (PSL)-Université Paris sciences et lettres (PSL)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Sorbonne Université (SU)-Université Paris Cité (UPCité), Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Université de Paris (UP), Institut Pasteur [Paris], Physiopathologie de l'immunodépression associée aux réponses inflammatoires systémiques - EA 7426 (PI3), Hôpital des Enfants, CHU Toulouse [Toulouse], Institut National de la Santé et de la Recherche Médicale (INSERM)-Centre National de la Recherche Scientifique (CNRS)-Université de Paris (UP), École pratique des hautes études (EPHE), and Université Paris sciences et lettres (PSL)-Université Paris sciences et lettres (PSL)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Sorbonne Université (SU)-Université de Paris (UP)
Subjects: Male, medicine.medical_specialty, Adolescent, Fever, [SDV]Life Sciences [q-bio], Lower risk, Intensive Care Units, Pediatric, Methylprednisolone, 03 medical and health sciences, 0302 clinical medicine, Pharmacotherapy, Interquartile range, Recurrence, 030225 pediatrics, Internal medicine, Medicine, Humans, 030212 general & internal medicine, Child, Propensity Score, Glucocorticoids, Retrospective Studies, Original Investigation, Pediatric intensive care unit, business.industry, Absolute risk reduction, COVID-19, Immunoglobulins, Intravenous, Correction, Retrospective cohort study, Odds ratio, General Medicine, Length of Stay, Combined Modality Therapy, Systemic Inflammatory Response Syndrome, 3. Good health, COVID-19 Drug Treatment, Treatment Outcome, Child, Preschool, Female, France, business, medicine.drug
Abstract: International audience; Importance: Multisystem inflammatory syndrome in children (MIS-C) is the most severe pediatric disease associated with severe acute respiratory syndrome coronavirus 2 infection, potentially life-threatening, but the optimal therapeutic strategy remains unknown. Objective: To compare intravenous immunoglobulins (IVIG) plus methylprednisolone vs IVIG alone as initial therapy in MIS-C. Design, Setting, and Participants: Retrospective cohort study drawn from a national surveillance system with propensity score-matched analysis. All cases with suspected MIS-C were reported to the French National Public Health Agency. Confirmed MIS-C cases fulfilling the World Health Organization definition were included. The study started on April 1, 2020, and follow-up ended on January 6, 2021. Exposures: IVIG and methylprednisolone vs IVIG alone. Main Outcomes and Measures: The primary outcome was persistence of fever 2 days after the introduction of initial therapy or recrudescence of fever within 7 days, which defined treatment failure. Secondary outcomes included a second-line therapy, hemodynamic support, acute left ventricular dysfunction after first-line therapy, and length of stay in the pediatric intensive care unit. The primary analysis involved propensity score matching with a minimum caliper of 0.1. Results: Among 181 children with suspected MIS-C, 111 fulfilled the World Health Organization definition (58 females [52%]; median age, 8.6 years [interquartile range, 4.7 to 12.1]). Five children did not receive either treatment. Overall, 3 of 34 children (9%) in the IVIG and methylprednisolone group and 37 of 72 (51%) in the IVIG alone group did not respond to treatment. Treatment with IVIG and methylprednisolone vs IVIG alone was associated with lower risk of treatment failure (absolute risk difference, -0.28 [95% CI, -0.48 to -0.08]; odds ratio [OR], 0.25 [95% CI, 0.09 to 0.70]; P =.008). IVIG and methylprednisolone therapy vs IVIG alone was also significantly associated with lower risk of use of second-line therapy (absolute risk difference, -0.22 [95% CI, -0.40 to -0.04]; OR, 0.19 [95% CI, 0.06 to 0.61]; P =.004), hemodynamic support (absolute risk difference, -0.17 [95% CI, -0.34 to -0.004]; OR, 0.21 [95% CI, 0.06 to 0.76]), acute left ventricular dysfunction occurring after initial therapy (absolute risk difference, -0.18 [95% CI, -0.35 to -0.01]; OR, 0.20 [95% CI, 0.06 to 0.66]), and duration of stay in the pediatric intensive care unit (median, 4 vs 6 days; difference in days, -2.4 [95% CI, -4.0 to -0.7]). Conclusions and Relevance: Among children with MIS-C, treatment with IVIG and methylprednisolone vs IVIG alone was associated with a more favorable fever course. Study interpretation is limited by the observational design..
Published: 2021
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