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1. Rapid Improvement after Starting Elexacaftor–Tezacaftor–Ivacaftor in Patients with Cystic Fibrosis and Advanced Pulmonary Disease

Author

Pierre-Régis Burgel, Isabelle Durieu, Raphaël Chiron, Sophie Ramel, Isabelle Danner-Boucher, Anne Prevotat, Dominique Grenet, Christophe Marguet, Martine Reynaud-Gaubert, Julie Macey, Laurent Mely, Annlyse Fanton, Sébastien Quetant, Lydie Lemonnier, Jean-Louis Paillasseur, Jennifer Da Silva, Clémence Martin, Claire Andrejak, Arnaud Becourt, Julie Mounard, Claire Poulet, Cinthia Rames, Marie Talleux, Marie-Chantal Chevalier, Estelle Darviot, Marie Jouvenot, Caroline Marien, Audrey Paris, Cécile Pelatan, Christine Person, Pascaline Priou, Françoise Troussier, Thierry Urban, Marie-Laure Dalphin, Jean-Charles Dalphin, Alice Ladaurade, Didier Pernet, Bénédicte Richaud-Thiriez, Pauline Roux-Claude, Nathalia Blanc, Vincent Boisserie-Lacroix, Stephanie Bui, Cyrielle Collet, Stéphane Debelleix, Emmanuel Bergot, Jacques Brouard, Karine Campbell, Muriel Laurans, Virginie Ribault, Corinne Borderon, Marie-Christine Heraud, Guillaume Labbe, Sylvie Montcouquiol, Isabelle Petit, Marc Ruivard, Céline Delestrain, Benoit Douvry, Ralph Epaud, Bernard Maitre, Natascha Remus, Guillaume Beltramo, Anne Houzel, Frédéric Huet, Stéphanie Perez, Amale Boldron-Ghaddar, Manuela Scalbert, Rabah Bouzioukh, Charles Simon, Boubou Camara, Rébecca Hamidfar, Catherine Llerena, Isabelle Pin, Antoine Deschildre, Alice Gicquello, Olivier Le Rouzic, Clara Leroy, Nicolas Paris, Thierry Perez, Caroline Thumerelle, Dominique Turck, Nathalie Wizla, Magali Dupuy-Grasset, Jane Languepin, Alexandra Masson-Rouchaud, Céline Menetrey, Stéphane Durupt, Sophie L’Excellent, Raphaele Nove-Josserand, Camille Ohlmann, Phillipe Reix, Quitterie Reynaud, Marie-Christine Werck-Gallois, Mélissandre Baravalle, Bérangère Coltey, Nadine Desmazes-Dufeu, Jean-Christophe Dubus, Clarisse Gautier, Jean-Baptiste Rey, Nathalie Stremler, Davide Caimmi, Margot Devrait, Johan Moreau, Yves Billon, Aurore Blondé, Anne Guillaumot, Sébastien Kiefer, Laura Peretti, Aurélie Tatopoulos, Angélica Tiotiu, Myriam Benhamida, Tiphaine Bihouee, Emmanuel Eschapasse, Adrien Tissot, Marie Giannantonio, Sylvie Leroy, Carole Piccini-Bailly, Johana Pradelli, Jonathan Messika, Véronique Boussaud, Nicolas Carlier, Isabelle Honoré, Dominique Hubert, Reem Kanaan, Céline Bailly-Botuha, Frédérique Chedevergne, Jacques De Blic, Christophe Delacourt, David Drummond, Brigitte Fauroux, Chantal Karila, Muriel Le Bourgeois, Isabelle Sermet, Bertrand Delaisi, Michèle Gerardin, Veronique Houdouin, Laurence Leclainche, Guillaume Aubertin, Laura Berdah, Annick Clement, Harriet Corvol, Nadia Nathan, Blandine Prevost, Nicolas Richard, Aline Tamalet, Jessica Taytard, Guillaume Thouvenin, Barbara Tourniaire, Michel Abely, Katia Bessaci-Kabouya, Sandra Dury, Bruno Ravoninjatovo, Alain Dabadie, Michel Dagorne, Eric Deneuville, Marie Jamin, Mélanie Ribault, Clémentine Vigier, Chantal Belleguic, Graziella Brinchault, Benoit Desrues, Audrey Barzic, Anne Dirou-Prigent, Jean Le Bihan, Krista Revert, Thomas Ropars, Laure Couderc, Stéphane Dominique, Hélène Morisse-Pradier, Stéphanie Pramil, Luc Thiberville, Nathalie Allou, Laurent Enaud, Elsa Gachelin, Eric Huchot, Annabelle Payet, Caroline Perisson, Saguiraly Piyaraly, Sophie Valois, Audrey Herzog, Romain Kessler, Michele Porzio, Laurence Weiss, Laurence Beaumont, Olivier Brugiere, Sylvie Colin, de Verdiere, Elise Cuquemelle, Sandra De Miranda, Adbdul Monem Hamid, François Parquin, Clément Picard, Antoine Roux, Charlotte Roy, François Bremont, Alain Didier, Marion Dupuis, Guillaume Faviez, Géraldine Labouret, Marie Mittaine, Marlène Murris-Espin, Léa Roditis, Laure Cosson, Patrice Diot, Thomas Flament, Charlotte Giraut, Julie Mankikian, Baptiste Arnouat, Gaétane Mousset, Véronique Storni, Philippe Vigneron, Emmanuelle Coirier-Duet, Asma Gabsi, Institut Cochin (IC UM3 (UMR 8104 / U1016)), Institut National de la Santé et de la Recherche Médicale (INSERM)-Centre National de la Recherche Scientifique (CNRS)-Université Paris Cité (UPCité), Hôpital Cochin [AP-HP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP), Hospices Civils de Lyon (HCL), Université de Lyon, Hôpital Arnaud de Villeneuve [CHRU Montpellier], Centre Hospitalier Régional Universitaire [Montpellier] (CHRU Montpellier), Fondation ILDYS (ILDYS), Institut du Thorax [Nantes], Centre hospitalier universitaire de Nantes (CHU Nantes), Hôpital Albert Calmette, Université de Lille, Droit et Santé-Centre Hospitalier Régional Universitaire [Lille] (CHRU Lille), Hôpital Foch [Suresnes], CHU Rouen, Normandie Université (NU), INSERM-TRANSFERT [Paris] (IT), Institut National de la Santé et de la Recherche Médicale (INSERM), Université de Rouen Normandie (UNIROUEN), Hôpital Nord [CHU - APHM], Aix Marseille Université (AMU), CHU Bordeaux [Bordeaux], Hôpital Renée Sabran [CHU - HCL], CHU Dijon, Centre Hospitalier Universitaire de Dijon - Hôpital François Mitterrand (CHU Dijon), Centre Hospitalier Universitaire [Grenoble] (CHU), Vaincre la Mucoviscidose, Association de lutte contre la Mucoviscidose, EFFI-STAT, URC/CIC Paris Descartes Necker Cochin, Hôpital Necker, Institut Desbrest de santé publique (IDESP), Institut National de la Santé et de la Recherche Médicale (INSERM)-Université de Montpellier (UM), CHU Trousseau [APHP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU), Supported by Vaincre la Mucoviscidose, Fondation Sauver la Vie, Universite Paris Descartes, Filiere Maladies Rares Muco-CFTR, and Legs PascalBonnet., Participating Investigators of the FrenchCystic Fibrosis Reference Network StudyGroup:Claire Andrejak, Arnaud Becourt, JulieMounard, Claire Poulet, Cinthia Rames, MarieTalleux (Amiens), Marie-Chantal Chevalier,Estelle Darviot, Marie Jouvenot, Caroline Marien,Audrey Paris, C ecile Pelatan, Christine Person,Pascaline Priou, Franc ̧oise Troussier, ThierryUrban (Angers-Le Mans), Marie-Laure Dalphin,Jean-Charles Dalphin, Alice Ladaurade, DidierPernet, B en edicte Richaud-Thiriez, PaulineRoux-Claude (Besanc ̧on), Nathalia Blanc,Vincent Boisserie-Lacroix, Stephanie Bui,Cyrielle Collet, St ephane Debelleix, Julie Macey(Bordeaux), Emmanuel Bergot, JacquesBrouard, Karine Campbell, Muriel Laurans,Virginie Ribault (Caen), Corinne Borderon,Marie-Christine Heraud, Guillaume Labbe,SylvieMontcouquiol, Isabelle Petit, Marc Ruivard(Clermont-Ferrand), C eline Delestrain, BenoitDouvry, Ralph Epaud, Bernard Maitre, NataschaRemus (Cr eteil), Guillaume Beltramo, AnnlyseFanton, Anne Houzel, Fr ed eric Huet, St ephaniePerez (Dijon), AmaleBoldron-Ghaddar, ManuelaScalbert (Dunkerque), Rabah Bouzioukh,Laurent Mely, Charles Simon (Giens), BoubouCamara, R ebecca Hamidfar, Catherine Llerena,Isabelle Pin, S ebastien Quetant (Grenoble), Antoine Deschildre, Alice Gicquello, Olivier LeRouzic, Clara Leroy, Nicolas Paris, Thierry Perez,Anne Prevotat, Caroline Thumerelle, DominiqueTurck, Nathalie Wizla (Lille), Magali Dupuy-Grasset, Jane Languepin, Alexandra Masson-Rouchaud, C eline Menetrey (Limoges), IsabelleDurieu, St ephane Durupt, Sophie L’Excellent,Raphaele Nove-Josserand, Camille Ohlmann,Phillipe Reix, Quitterie Reynaud, Marie-ChristineWerck-Gallois (Lyon), M elissandre Baravalle,B erang ere Coltey, Nadine Desmazes-Dufeu,Jean-Christophe Dubus, Clarisse Gautier, Jean-Baptiste Rey, Martine Reynaud-Gaubert,Nathalie Stremler (Marseille), Davide Caimmi,Rapha€el Chiron, Margot Devrait, Johan Moreau(Montpellier), Yves Billon, Aurore Blond e, AnneGuillaumot, S ebastien Kiefer, Laura Peretti,Aur elie Tatopoulos, Ang elica Tiotiu (Nancy), Myriam Benhamida, Tiphaine Bihouee, IsabelleDanner-Boucher, Emmanuel Eschapasse,Adrien Tissot (Nantes), Marie Giannantonio,Sylvie Leroy, Carole Piccini-Bailly, JohanaPradelli (Nice), Jonathan Messika (Paris, Bichat), V eronique Boussaud, Pierre-R egis Burgel,Nicolas Carlier, Isabelle Honor e, DominiqueHubert, Reem Kanaan, Cl emence Martin (Paris,Cochin), C eline Bailly-Botuha, Fr ed eriqueChedevergne, Jacques De Blic, ChristopheDelacourt, David Drummond, Brigitte Fauroux,Chantal Karila, Muriel Le Bourgeois, IsabelleSermet (Paris, Necker), Bertrand Delaisi,Mich ele Gerardin, Veronique Houdouin,Laurence Leclainche (Paris, Robert Debr e), Guillaume Aubertin, Laura Berdah, AnnickClement, Harriet Corvol, Nadia Nathan, BlandinePrevost, Nicolas Richard, Aline Tamalet, JessicaTaytard, Guillaume Thouvenin, BarbaraTourniaire (Paris, Trousseau), Michel Abely,Katia Bessaci-Kabouya, Sandra Dury, BrunoRavoninjatovo (Reims), Alain Dabadie, MichelDagorne, Eric Deneuville, Marie Jamin, M elanieRibault, Cl ementine Vigier (Rennes – SaintBrieuc), Chantal Belleguic, Graziella Brinchault,Benoit Desrues (Rennes), Audrey Barzic, AnneDirou-Prigent, Jean Le Bihan, Sophie Ramel,Krista Revert, Thomas Ropars (Roscoff), LaureCouderc, St ephane Dominique, ChristopheMarguet, H el ene Morisse-Pradier, St ephaniePramil, Luc Thiberville (Rouen), Nathalie Allou,Laurent Enaud, Elsa Gachelin, Eric Huchot,Annabelle Payet, Caroline Perisson, SaguiralyPiyaraly, Sophie Valois (La R eunion), AudreyHerzog, Romain Kessler, Michele Porzio,Laurence Weiss (Strasbourg), LaurenceBeaumont, Olivier Brugiere, Sylvie Colin deVerdiere, Elise Cuquemelle, Sandra De Miranda,Dominique Grenet, Adbdul Monem Hamid,Franc ̧ois Parquin, Cl ementPicard, Antoine Roux,Charlotte Roy (Suresnes), Franc ̧ois Bremont,Alain Didier, Marion Dupuis, Guillaume Faviez,G eraldine Labouret, Marie Mittaine, Marl eneMurris-Espin, L ea Roditis (Toulouse), LaureCosson, Patrice Diot, Thomas Flament, CharlotteGiraut, JulieMankikian(Tours), Baptiste Arnouat,Ga etane Mousset, V eronique Storni, PhilippeVigneron (Vannes-Lorient), and Emmanuelle Coirier-Duet, and Asma Gabsi (Versailles)

Subjects
Lung Diseases, Male, elexacaftor, Indoles, Cystic Fibrosis, Pyridines, medicine.medical_treatment, Regulator, Aminophenols, Critical Care and Intensive Care Medicine, [SDV.MHEP.PSR]Life Sciences [q-bio]/Human health and pathology/Pulmonology and respiratory tract, Cystic fibrosis, Gastroenterology, Membrane Potentials, Ivacaftor, 0302 clinical medicine, Prospective Studies, 030212 general & internal medicine, Chloride Channel Agonists, Aged, 80 and over, Middle Aged, Transmembrane protein, Drug Combinations, Quinolines, Female, France, medicine.drug, Adult, Pulmonary and Respiratory Medicine, congenital, hereditary, and neonatal diseases and abnormalities, medicine.medical_specialty, cystic fibrosis transmembrane conductance regulator modulators, Adolescent, Pulmonary disease, Young Adult, 03 medical and health sciences, Internal medicine, lung transplantation, medicine, Humans, Lung transplantation, In patient, Aged, [SDV.MHEP.PED]Life Sciences [q-bio]/Human health and pathology/Pediatrics, business.industry, Editorials, medicine.disease, 030228 respiratory system, Tezacaftor, Pyrazoles, business
Abstract

International audience; Rationale: Elexacaftor-tezacaftor-ivacaftor is a CFTR (cystic fibrosis [CF] transmembrane conductance regulator) modulator combination, developed for patients with CF with at least one Phe508del mutation. Objectives: To evaluate the effects of elexacaftor-tezacaftor- ivacaftor in patients with CF and advanced respiratory disease. Methods: A prospective observational study, including all patients aged ⩾12 years and with a percent-predicted FEV1 (ppFEV1)

Published
2021
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2. Chest physiotherapy enhances detection of Pseudomonas aeruginosa in nonexpectorating children with cystic fibrosis

Author

Nathalie Wizla-Derambure, Marie Mittaine, E. Deneuville, Michael Fayon, Christophe Marguet, Ludovic Lemée, Véronique Houdouin, Michel Abely, Philippe Reix, S. Ramel, Laure Couderc, Marie-Laure Dalphin, Muriel Le Bourgeois, Evelyne Leroux, Isabelle Pin, F. Huet, Harriet Corvol, Tiphaine Bihouée, Muriel Laurans, Centre d'Investigation Clinique [CHU Rouen] (CIC Rouen), Hôpital Charles Nicolle [Rouen], CHU Rouen, Normandie Université (NU)-Normandie Université (NU)-CHU Rouen, Normandie Université (NU)-Normandie Université (NU)-Université de Rouen Normandie (UNIROUEN), Normandie Université (NU)-Institut National de la Santé et de la Recherche Médicale (INSERM), Immunologie humaine, physiopathologie & immunothérapie (HIPI (UMR_S_976 / U976)), Institut National de la Santé et de la Recherche Médicale (INSERM)-Université Paris Cité (UPCité), Institute for Advanced Biosciences / Institut pour l'Avancée des Biosciences (Grenoble) (IAB), Centre Hospitalier Universitaire [Grenoble] (CHU)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Etablissement français du sang - Auvergne-Rhône-Alpes (EFS)-Centre National de la Recherche Scientifique (CNRS)-Université Grenoble Alpes (UGA), Laboratoire de Biométrie et Biologie Evolutive - UMR 5558 (LBBE), Université Claude Bernard Lyon 1 (UCBL), Université de Lyon-Université de Lyon-Institut National de Recherche en Informatique et en Automatique (Inria)-VetAgro Sup - Institut national d'enseignement supérieur et de recherche en alimentation, santé animale, sciences agronomiques et de l'environnement (VAS)-Centre National de la Recherche Scientifique (CNRS), Service de pédiatrie (CHU de Dijon), Centre Hospitalier Universitaire de Dijon - Hôpital François Mitterrand (CHU Dijon), Centre d'investigation clinique de Toulouse (CIC 1436), 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)-Pôle Santé publique et médecine publique [CHU Toulouse], Centre Hospitalier Universitaire de Toulouse (CHU Toulouse)-Centre Hospitalier Universitaire de Toulouse (CHU Toulouse), Hôpital Jeanne de Flandres, Université de Lille, Droit et Santé-Centre Hospitalier Régional Universitaire [Lille] (CHRU Lille), Centre Hospitalier Universitaire de Reims (CHU Reims), Centre Hospitalier Régional Universitaire de Besançon (CHRU Besançon), CHU de Bordeaux Pellegrin [Bordeaux], Centre hospitalier universitaire de Nantes (CHU Nantes), Service de Pneumologie et d'Allergologie Pédiatriques, 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 Pontchaillou [Rennes], Mucoviscidose: physiopathologie et phénogénomique [CRSA], Centre de Recherche Saint-Antoine (CRSA), Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Sorbonne Université (SU)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Sorbonne Université (SU), CHU Trousseau [APHP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU), Sorbonne Université - Faculté de Médecine (SU FM), Sorbonne Université (SU), CHU Caen, Normandie Université (NU)-Tumorothèque de Caen Basse-Normandie (TCBN), Vaincre la Mucoviscidose, Association de lutte contre la Mucoviscidose, Normandie Université (NU)-Normandie Université (NU), Hôpital Charles Nicolle [Rouen]-CHU Rouen, Institut National de la Santé et de la Recherche Médicale (INSERM)-Université de Paris (UP), Université Fédérale Toulouse Midi-Pyrénées-Université Fédérale Toulouse Midi-Pyrénées-CHU Toulouse [Toulouse]-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)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP), Centre de Recherche Saint-Antoine (CR Saint-Antoine), Sorbonne Université (SU)-Institut National de la Santé et de la Recherche Médicale (INSERM)-CHU Saint-Antoine [AP-HP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU), HAL-SU, Gestionnaire, Université Fédérale Toulouse Midi-Pyrénées-Université Fédérale Toulouse Midi-Pyrénées-Institut National de la Santé et de la Recherche Médicale (INSERM)-Pôle Santé publique et médecine publique [CHU Toulouse], and Service de Pneumologie pédiatrique [CHU Trousseau]

Subjects
Pulmonary and Respiratory Medicine, medicine.medical_specialty, lcsh:Medicine, Chest physiotherapy, medicine.disease_cause, Cystic fibrosis, [SDV.MHEP.PSR]Life Sciences [q-bio]/Human health and pathology/Pulmonology and respiratory tract, Haemophilus influenzae, 03 medical and health sciences, [SDV.MHEP.PED] Life Sciences [q-bio]/Human health and pathology/Pediatrics, 0302 clinical medicine, [SDV.MHEP.MI]Life Sciences [q-bio]/Human health and pathology/Infectious diseases, Internal medicine, medicine, 030212 general & internal medicine, Respiratory infections and tuberculosis, CF and non-CF bronchiectasis, [SDV.MHEP.PED]Life Sciences [q-bio]/Human health and pathology/Pediatrics, Lung, Paediatric pulmonology, Pseudomonas aeruginosa, business.industry, lcsh:R, medicine.disease, 3. Good health, medicine.anatomical_structure, 030228 respiratory system, [SDV.SPEE] Life Sciences [q-bio]/Santé publique et épidémiologie, Staphylococcus aureus, [SDV.MHEP.MI] Life Sciences [q-bio]/Human health and pathology/Infectious diseases, [SDV.MHEP.PSR] Life Sciences [q-bio]/Human health and pathology/Pulmonology and respiratory tract, Sputum, [SDV.SPEE]Life Sciences [q-bio]/Santé publique et épidémiologie, medicine.symptom, business, Airway
Abstract

Lung damage in cystic fibrosis (CF) is strongly associated with lower airway infections. Early treatment of Pseudomonas aeruginosa is recommended. Pathogen detection requires sampling of lower airway secretions, which remains a challenge in nonexpectorating patients. Our hypothesis was that chest physiotherapy would improve the quality of airway secretion samples and increase the rates of pathogens detected in nonexpectorating patients.This prospective multicentre study compared three successive methods for sampling airway secretions applied through the same session: 1) an oropharyngeal swab (OP), 2) a chest physiotherapy session followed by a provoked cough to obtain sputum (CP-SP) and 3) a second oropharyngeal swab collected after chest physiotherapy (CP-OP). Haemophilus influenzae, Staphylococcus aureus and P. aeruginosa growth cultures were assessed. Accuracy tests and an equivalence test were performed to compare the three successive methods of collection.300 nonexpectorating children with CF were included. P. aeruginosa was detected cumulatively in 56 (18.9%) children, and according to the different collection methods in 28 (9.8%), 37 (12.4%) and 44 (14.7%) children by using OP, CP-OP and CP-SP, respectively. Compared with OP, the increased detection rate was +22% for CP-OP (p=0.029) and +57% for CP-SP (p=0.003). CP-SP had the best positive predictive value (86.3%) and negative predictive value (96.0%) for P. aeruginosa compared with the overall detection.The results of this adequately powered study show differences in the rates of pathogens detected according to the sampling method used. Chest physiotherapy enhanced detection of P. aeruginosa in nonexpectorating children with CF.

Published
2021
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3. Repaglinide versus insulin for newly diagnosed diabetes in patients with cystic fibrosis: a multicentre, open-label, randomised trial

Author

Marie-Christine Vantyghem, Dominique Hubert, Andreas Claaß, Anne Munck, Doris Staab, Ute Staden, Helmut Teschler, Klaus-Michael Keller, Laurence Kessler, Horst Generlich, Guy-André Loeuille, Helen Mosnier-Pudar, Gabriela H. Thalhammer, Tanja Nickolay, Matthias V. Kopp, Nicole Prinz, Gérard Lenoir, Jürgen Hautz, Irmgard Eichler, Rüdiger Szczepanski, R Serreau, Jean-Jacques Robert, Hans-Georg Bresser, Birgit Schilling, Baroukh M. Assael, Martin Stern, Manfred Ballmann, Christina Smaczny, Egbert Herting, Matthias Wiebel, Lutz Naehrlich, Uwe Mellies, Hans-Georg Posselt, Ernst Rietschel, Thomas Biedermann, Thomas Köhnlein, Ernst-Hinrich Ballke, Wolfgang Kamin, Antje Schuster, Gerd Dockter, Holger Köster, Nathalie Wizla, Wolfram Wiebicke, Hans-Joachim Feickert, Manfred Götz, Sylvie Leroy, Marcus A Mall, Fawzia Aissat, Helge Hebestreit, Vera Wienhausen-Wilke, H.-E. Heuer, Isidor Huttegger, Alexandra Hebestreit, Raphaële Nove-Josserand, Laurence Weiss, Martin Claßen, Marguerite Honer, Reinhard W. Holl, Friedrich-Karl Tegtmeyer, Egbert Meyer, and Peter Küster

Subjects
Adult, Blood Glucose, Male, medicine.medical_specialty, Adolescent, Cystic Fibrosis, Endocrinology, Diabetes and Metabolism, medicine.medical_treatment, Population, 030209 endocrinology & metabolism, Cystic fibrosis, law.invention, Young Adult, 03 medical and health sciences, 0302 clinical medicine, Endocrinology, Piperidines, Randomized controlled trial, law, Diabetes mellitus, Internal medicine, Internal Medicine, Humans, Hypoglycemic Agents, Insulin, Medicine, 030212 general & internal medicine, Child, Adverse effect, education, Glycated Hemoglobin, education.field_of_study, business.industry, Prognosis, Repaglinide, medicine.disease, Diabetes Mellitus, Type 2, Female, Carbamates, Complication, business, Biomarkers, Follow-Up Studies, medicine.drug
Abstract

Summary Background As survival among patients with cystic fibrosis has improved in recent decades, complications have become increasingly relevant. The most frequent complication is cystic-fibrosis-related diabetes. The recommended treatment is injected insulin, but some patients are treated with oral antidiabetic drugs to ease the treatment burden. We assessed the efficacy and safety of oral antidiabetic drugs. Methods We did a multicentre, open-label, comparative, randomised trial in 49 centres in Austria, France, Germany, and Italy. Eligible patients had cystic fibrosis, were older than 10 years, and had newly diagnosed diabetes. We used a central randomisation schedule derived from a Geigy random number table to assign patients 1:1 to receive insulin or repaglinide, stratified by sex and age (10–15 years or >15 years). The primary outcome was glycaemic control assessed by mean change in HbA 1c concentration from baseline after 24 months of treatment. Differences between groups were assessed by linear models. The primary and safety analyses were done in the modified intention-to-treat population (including patients who stopped treatment early because of lack of efficacy). This trial is registered with ClinicalTrials.gov, number NCT00662714. Findings We enrolled 34 patients in the repaglinide group and 41 in the insulin group, of whom 30 and 37, respectively, were included in the analyses. At 24 months, glycaemic control was similar in the repaglinide and insulin groups (mean change in HbA 1c concentration from baseline 0·2% [SD 0·7%], 1·7 mmol/mol [8·1 mmol/mol] with repaglinide vs −0·2% [1·3%], −2·7 mmol/mol, [14·5 mmol/mol] with insulin; mean difference between groups −0·4%, (95% CI −1·1 to 0·2 [−4·4 mmol/mol, −11·5 to 2·7], p=0·15). The most frequent adverse events were pulmonary events (43 [40%] of 107 in the repaglinide group and 60 [45%] of 133 in the insulin group), and the most frequent serious adverse events were pulmonary events leading to hospital admission (five [50%] of ten and seven [54%] of 13, respectively). Interpretation Repaglinide for glycaemic control in patients with cystic-fibrosis-related diabetes is as efficacious and safe as insulin. Funding Mukoviszidose eV, Vaincre la Mucoviscidose, ABCF Association, and Novo Nordisk.

Published
2018
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4. Real-Life Safety and Effectiveness of Lumacaftor-Ivacaftor in Patients with Cystic Fibrosis

Author

Pierre-Régis Burgel, Anne Munck, Isabelle Durieu, Raphaël Chiron, Laurent Mely, Anne Prevotat, Marlene Murris-Espin, Michele Porzio, Michel Abely, Philippe Reix, Christophe Marguet, Julie Macey, Isabelle Sermet-Gaudelus, Harriet Corvol, Stéphanie Bui, Lydie Lemonnier, Clémence Dehillotte, Jennifer Da Silva, Jean-Louis Paillasseur, Dominique Hubert, Julie Mounard, Claire Poulet, Cinthia Rames, Christine Person, Françoise Troussier, Thierry Urban, Marie-Laure Dalphin, Jean-Claude Dalphin, Didier Pernet, Bénédicte Richaud-Thiriez, Mickael Fayon, Julie Macey-Caro, Karine Campbell, Muriel Laurans, Corinne Borderon, Marie-Christine Heraud, André Labbé, Sylvie Montcouquiol, Laurence Bassinet, Natascha Remus, Annlyse Fanton, Anne Houzel-Charavel, Frédéric Huet, Stéphanie Perez-Martin, Amale Boldron-Ghaddar, Manuela Scalbert, Boubou Camara, Catherine Llerena, Isabelle Pin, Sébastien Quétant, Aurélie Cottereau, Antoine Deschildre, Alice Gicquello, Thierry Perez, Lidwine Stervinou-Wemeau, Caroline Thumerelle, Benoit Wallaert, Nathalie Wizla, Jane Languepin, Céline Ménétrey, Magalie Dupuy-Grasset, Lucie Bazus, Clelia Buchs, Virginie Jubin, Marie-Christine Werck-Gallois, Catherine Mainguy, Thomas Perrin, Agnès Toutain-Rigolet, Stéphane Durupt, Quitterie Reynaud, Raphaele Nove-Josserand, Melisande Baravalle-Einaudi, Bérangère Coltey, Nadine Dufeu, Jean-Christophe Dubus, Nathalie Stremler, Davide Caimmi, Yves Billon, Jocelyne Derelle, Sébastien Kieffer, Anne-Sophie Pichon, Cyril Schweitzer, Aurélie Tatopoulos, Sarah Abbes, Tiphaine Bihouée, Isabelle Danner-Boucher, Valérie David, Alain Haloun, Adrien Tissot, Sylvie Leroy, Carole Bailly-Piccini, Annick Clément, Aline Tamalet, Isabelle Honoré, Reem Kanaan, Clémence Martin, Cécile Bailly, Frédérique Chédevergne, Jacques De Blic, Brigitte Fauroux, Murielle Le Bourgeois, Bertrand Delaisi, Michèle Gérardin, Michel Abély, Bruno Ravoninjatovo, Chantal Belleguic, Benoit Desrues, Graziella Brinchault, Michel Dagorne, Eric Deneuville, Sylvaine Lefeuvre, Anne Dirou, Jean Le Bihan, Sophie Ramel, Stéphane Dominique, Annabelle Payet, Romain Kessler, Vincent Rosner, Laurence Weiss, Sandra de Miranda, Dominique Grenet, Abdoul Hamid, Clément Picard, François Brémont, Alain Didier, Géraldine Labouret, Marie Mittaine, Marlène Murris-Espin, Laurent Têtu, Laure Cosson, Charlotte Giraut, Anne-Cécile Henriet, Julie Mankikian, Sophie Marchand, Sandrine Hugé, Véronique Storni, Emmanuelle Coirier-Duet, CHU Cochin [AP-HP], Service de Médecine Interne - Centre Hospitalier Lyon Sud, Hospices Civils de Lyon (HCL)-Centre Hospitalier Lyon Sud [CHU - HCL] (CHLS), Hospices Civils de Lyon (HCL), Centre Hospitalier Régional Universitaire [Montpellier] (CHRU Montpellier), Centre de Ressources et de Compétences en Mucoviscidose [Lyon] (CRCM [Lyon]), Hospices Civils de Lyon (HCL)-CHU Lyon-Hôpital Renée Sabran [CHU - HCL], Hôpital Albert Calmette, Université de Lille, Droit et Santé-Centre Hospitalier Régional Universitaire [Lille] (CHRU Lille), CHU Toulouse [Toulouse], Laboratoire de Biométrie et Biologie Evolutive - UMR 5558 (LBBE), Université Claude Bernard Lyon 1 (UCBL), Université de Lyon-Université de Lyon-Institut National de Recherche en Informatique et en Automatique (Inria)-VetAgro Sup - Institut national d'enseignement supérieur et de recherche en alimentation, santé animale, sciences agronomiques et de l'environnement (VAS)-Centre National de la Recherche Scientifique (CNRS), Service de pédiatrie médicale et médecine de l'adolescent [Rouen], CHU Rouen, Normandie Université (NU)-Normandie Université (NU)-Université de Rouen Normandie (UNIROUEN), Normandie Université (NU), Centre de recherche Croissance et signalisation (UMR_S 845), Université Paris Descartes - Paris 5 (UPD5)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Centre National de la Recherche Scientifique (CNRS), Service de pneumologie [CHU Trousseau], Université Pierre et Marie Curie - Paris 6 (UPMC)-Assistance publique - Hôpitaux de Paris (AP-HP) (APHP)-CHU Trousseau [APHP], Centre de Ressources et de Compétences de la Mucoviscidose (CRCM), Hôpital des Enfants, CHU Toulouse [Toulouse]-CHU Toulouse [Toulouse], Association Vaincre La Mucoviscidose, Institut Cochin (IC UM3 (UMR 8104 / U1016)), EFFI-STAT, CIC Cochin Pasteur (CIC 1417), Assistance publique - Hôpitaux de Paris (AP-HP) (APHP)-CHU Cochin [AP-HP]-Hôtel-Dieu-Université Paris Descartes - Paris 5 (UPD5)-Groupe hospitalier Broca-Institut National de la Santé et de la Recherche Médicale (INSERM), Hôpital Cochin [AP-HP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP), Centre Hospitalier Universitaire de Toulouse (CHU Toulouse), Mucoviscidose: physiopathologie et phénogénomique [CRSA], Centre de Recherche Saint-Antoine (CRSA), Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Sorbonne Université (SU)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Sorbonne Université (SU), CHU Trousseau [APHP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU), Sorbonne Université - Faculté de Médecine (SU FM), Sorbonne Université (SU), Centre de Ressources et de Compétences en Mucoviscidose [CHU Toulouse] (CRCM Toulouse), Service Pneumologie et allergologie 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), Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Hôpital Cochin [AP-HP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Hôtel-Dieu-Université Paris Descartes - Paris 5 (UPD5)-Groupe hospitalier Broca-Institut National de la Santé et de la Recherche Médicale (INSERM), Université Pierre et Marie Curie - Paris 6 (UPMC)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-CHU Trousseau [APHP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU)-Sorbonne Université (SU), Institut National de la Santé et de la Recherche Médicale (INSERM)-Groupe hospitalier Broca-Université Paris Descartes - Paris 5 (UPD5)-Hôtel-Dieu-Hôpital Cochin [AP-HP], and Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)

Subjects
Male, Cystic Fibrosis, Gastrointestinal Diseases, [SDV]Life Sciences [q-bio], Aminopyridines, Quinolones, Critical Care and Intensive Care Medicine, Logistic regression, Aminophenols, Cystic fibrosis, Body Mass Index, Ivacaftor, chemistry.chemical_compound, 0302 clinical medicine, Deprescriptions, Forced Expiratory Volume, Medicine, 030212 general & internal medicine, Fatigue, 2. Zero hunger, biology, Lumacaftor, Headache, lumacaftor–ivacaftor, Cystic fibrosis transmembrane conductance regulator, 3. Good health, Anti-Bacterial Agents, Drug Combinations, Treatment Outcome, Administration, Intravenous, Female, France, medicine.drug, Pulmonary and Respiratory Medicine, Adult, medicine.medical_specialty, Metrorrhagia, Adolescent, Nutritional Status, 03 medical and health sciences, Young Adult, Internal medicine, Product Surveillance, Postmarketing, Humans, Benzodioxoles, Adverse effect, Bronchial Spasm, business.industry, Editorials, Myalgia, medicine.disease, Discontinuation, Dyspnea, Logistic Models, 030228 respiratory system, chemistry, Cough, Multivariate Analysis, biology.protein, postmarketing study, business, Body mass index
Abstract

International audience; Rationale: Lumacaftor-ivacaftor is a CFTR (cystic fibrosis transmembrane conductance regulator) modulator combination recently approved for patients with cystic fibrosis (CF) homozygous for the Phe508del mutation.Objectives: To evaluate the safety and effectiveness of lumacaftor-ivacaftor in adolescents (≥12 yr) and adults (≥18 yr) in a real-life postapproval setting.Methods: The study was conducted in the 47 CF reference centers in France. All patients who initiated lumacaftor-ivacaftor from January 1 to December 31, 2016, were eligible. Patients were evaluated for lumacaftor-ivacaftor safety and effectiveness over the first year of treatment following the French CF Learning Society's recommendations.Measurements and Main Results: Among the 845 patients (292 adolescents and 553 adults) who initiated lumacaftor-ivacaftor, 18.2% (154 patients) discontinued treatment, often owing to respiratory (48.1%, 74 patients) or nonrespiratory (27.9%, 43 patients) adverse events. In multivariable logistic regression, factors associated with increased rates of discontinuation included adult age group, percent predicted FEV1 (ppFEV1) less than 40%, and numbers of intravenous antibiotic courses during the year before lumacaftor-ivacaftor initiation. Patients with continuous exposure to lumacaftor-ivacaftor showed an absolute increase in ppFEV1 (+3.67%), an increase in body mass index (+0.73 kg/m2), and a decrease in intravenous antibiotic courses by 35%. Patients who discontinued treatment had significant decrease in ppFEV1, without improvement in body mass index or decrease in intravenous antibiotic courses.Conclusions: Lumacaftor-ivacaftor was associated with improvement in lung disease and nutritional status in patients who tolerated treatment. Adults who discontinued lumacaftor-ivacaftor, often owing to adverse events, were found at high risk of clinical deterioration.

Published
2019
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5. Nutritional Status in the First 2 Years of Life in Cystic Fibrosis Diagnosed by Newborn Screening

Author

Anne, Munck, Rym, Boulkedid, Laurence, Weiss, Pierre, Foucaud, Nathalie, Wizla-Derambure, Philippe, Reix, François, Bremont, Jocelyne, Derelle, Julien, Schroedt, Corinne, Alberti, and Laure, Couderc

Subjects
Male, Pediatrics, medicine.medical_specialty, Staphylococcus aureus, Cystic Fibrosis, Body height, Enzyme Therapy, Nutritional Status, Cystic fibrosis, 03 medical and health sciences, 0302 clinical medicine, Neonatal Screening, 030225 pediatrics, Medicine, Humans, 030212 general & internal medicine, Longitudinal Studies, Prospective Studies, Prospective cohort study, Respiratory Tract Infections, Growth Disorders, Newborn screening, business.industry, Nutritional Support, Body Weight, Malnutrition, Gastroenterology, Infant, Newborn, Infant, Nutritional status, Avitaminosis, Proton Pump Inhibitors, Vitamins, medicine.disease, Body Height, Breast Feeding, Multicenter study, Child, Preschool, Pediatrics, Perinatology and Child Health, Cohort, Carrier State, Pseudomonas aeruginosa, Exocrine Pancreatic Insufficiency, Female, business, Breast feeding
Abstract

To evaluate nutritional status and associated factors in a cystic fibrosis (CF) cohort diagnosed by newborn screening and followed up to month 24.A prospective longitudinal multicenter study assessing nutritional status according to pancreatic status, feeding modalities, prescriptions, pulmonary outcome, and biological nutritional parameters.One hundred and five infants were recruited and 99 completed the study. Nutritional care management prevented undernutrition and stunting in those with exocrine pancreatic sufficiency (EPS), but affected (13/87) 15% and (21/86) 24%, respectively, of infants with exocrine pancreatic insufficiency (EPI). The logistic regression model found a positive association between both weight and length z scores "at risk" at month 24, and initial pulmonary symptoms (odds ratio [OR] 0.06, P 0.01 and OR 0.08, P 0.01, respectively); these symptoms were less frequent when age at first visit was earlier than 1.2 months (33% vs 67%, P = 0.02); stunting was also associated with high-calorie density intake and Staphylococcus aureus (OR 0.05, P = 0.01 and OR 0.17, P 0.01). Pulmonary outcome did not differ according to pancreatic status; breast-feeding for at least 3 months delayed first acquisition of Pseudomonas aeruginosa. Despite sodium and fat-soluble vitamin supplementation, half of both cohorts had low urinary sodium output and half of the EPI cohort had low vitamin D levels.Our data shed light on the fact that stunting was more frequent than undernutrition, while both parameters involved only patients with pancreatic insufficiency. Modalities of feeding were not associated with nutritional status; breast-feeding may provide some protection against acquisition of P aeruginosa.

Published
2018

6. Impact of Intravenous Antibiotic Therapy on Total Daily Energy Expenditure and Physical Activity in Cystic Fibrosis Children with Pseudomonas aeruginosa Pulmonary Exacerbation

Author

Dominique Guimber, G A Loeuille, Anne Sardet, Antoine Deschildre, Laurent Michaud, Dominique Turck, Frédéric Gottrand, Laurent Béghin, and Nathalie Wizla-Derambure

Subjects
Male, medicine.medical_specialty, Adolescent, Cystic Fibrosis, Physical exercise, Motor Activity, medicine.disease_cause, Cystic fibrosis, Metabolic equivalent, Internal medicine, medicine, Humans, Pseudomonas Infections, Resting energy expenditure, Respiratory system, Child, Retrospective Studies, Antibacterial agent, business.industry, Pseudomonas aeruginosa, Respiratory disease, medicine.disease, Diet Records, Anti-Bacterial Agents, Endocrinology, Child, Preschool, Pediatrics, Perinatology and Child Health, Body Composition, Female, Energy Intake, Energy Metabolism, business
Abstract

Resting energy expenditure (REE) increases during pulmonary exacerbation by Pseudomonas aeruginosa in cystic fibrosis (CF) patients, and decreases after i.v. anti-Pseudomonas aeruginosa antibiotic therapy (IVAT). However, the impact of IVAT on total energy expenditure (TEE) is unknown. The aim of this study was to assess the changes in TEE and its main components after IVAT administered at home. Body composition measured by skinfold thickness and bio-impedance analysis, energy intake (EI) assessed by a weekly diary, REE measured by indirect calorimetry (IC), TEE assessed by a technique using 24-h heart-rate monitoring method and physical activity (PA) monitored using an activity diary (AD) were assessed in 16 patients (9 boys and 7 girls) aged 12.1 +/- 2.3 y (range, 7.1-14.6 y), before and after 28 +/- 4 d including a 14-d IVAT course. After IVAT, weight increased significantly by 1.9% (32.1 +/- 7.5 versus 32.7 +/- 7.6 kg; p < 0.05), while fat mass and fat free mass increased non significantly. EI increased by 4.6% (10,797 +/- 3039 versus 11320 +/- 3074 kJ/d; p < 0.05). TEE was not affected by IVAT (7014 +/- 1929 versus 7081 +/- 1478 kJ/d) whereas REE decreased by 4.1% (5295 +/- 909 versus 5093 +/- 837 kJ/d; p < 0.05), resulting in 9.3% increase in PA assessed by AD converted to metabolic equivalent tasks (MET) (37.0 +/- 3.1 versus 40.7 +/- 4.5 MET; p < 0.05). The improvement in nutritional status after IVAT is not related to a decrease in TEE, but probably to an increase in EI and a decrease of REE after IVAT. After IVAT, the reduction in REE is probably compensated by an increase in PA in CF patients.

Published
2003
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7. Familial and Community Environmental Risk Factors for Helicobacter pylori Infection in Children and Adolescents

Author

Laurent Michaud, Serge Ganga-Zandzou, Frédéric Gottrand, Dominique Turck, Nathalie Wizla-Derambure, Simon Ategbo, and P. Vincent

Subjects
Male, Pediatrics, medicine.medical_specialty, Adolescent, Biopsy, Spirillaceae, Helicobacter Infections, Risk Factors, Surveys and Questionnaires, Epidemiology, Prevalence, medicine, Humans, Endoscopy, Digestive System, Risk factor, Child, Socioeconomic status, Family Characteristics, Helicobacter pylori, biology, medicine.diagnostic_test, business.industry, Stomach, Age Factors, Infant, Newborn, Gastroenterology, Infant, biology.organism_classification, Endoscopy, Community-Acquired Infections, Socioeconomic Factors, El Niño, Child, Preschool, Pediatrics, Perinatology and Child Health, Female, Gastritis, medicine.symptom, business
Abstract

The aim of the study was to identify familial and community environmental risk factors associated with Helicobacter pylori infection in a pediatric population.Children requiring diagnostic upper endoscopy were included in the study during a 2-year period. During endoscopy, five gastric biopsies were performed for the histologic or bacteriologic diagnosis, or both, of H. pylori infection. Epidemiologic data collected by a questionnaire were analyzed using the chi-square test or Fisher test and stepwise logistic regression.The authors included 436 patients (242 boys), aged 2 days to 17.9 years (median, 2.7 years). H. pylori prevalence was 7.3%. Univariate analysis found H. pylori was more common in older patients (P0.00001), in children who had at least one parent born in a developing country (P0.02) or with a low socioeconomic status (P0.02), and in those living in crowded conditions (P0.02). Children whose mother worked at home were more frequently infected than children whose mother worked outside the home (P0.02). Attendance at nursery or school before the age of 6 years was not associated with infection. Logistic regression showed a strong association with H. pylori only for age and number of persons at home.The source of H. pylori is intrafamilial rather than from a community, such as nursery and school attended at a young age. The number of persons in the home influences the infection status of children but not by the presence of the mother in home. These data suggest that H. pylori infection transmission occurs from siblings or the father rather than from mother.

Published
2001
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8. Effets de la rhdnase sur la fonction respiratoire et le statut nutritionnel de l'enfant et de l'adolescent atteints de mucoviscidose

Author

G Alœuille, J Dias, N. Loire, Antoine Deschildre, C. Buisine, Dominique Turck, E. Boutry, Nathalie Wizla-Derambure, E Tassin, F Hecquet, A Sardet, and Laurent Michaud

Subjects
Gynecology, medicine.medical_specialty, business.industry, Pediatrics, Perinatology and Child Health, Respiratory disease, medicine, Lung volume measurement, Nutritional status, medicine.disease, business, Lung function
Abstract

Resume Des 1994, nous avons debute la rhDNase chez tous les patients atteints de mucoviscidose âges de plus de 5 ans et ayant une CVF ≥ 40 % de la valeur theorique. Population et methodes Nous avons analyse retrospectivement les effets de la rhDNase au cours de 2 ans de traitement chez 69 enfants et adolescents. Les patients ( 35 garcons, 34 filles) ont recu une dose unique journaliere de 2,5 mg de rhDNase. Ils etaient âges initialement de 8,5 ans (5 a 16,4 ans). Les valeurs spirometriques (pourcentage de la valeur theorique) et le statut nutritionnel de base etaient les suivants: CVF = 84,8 ± 21,7, VEMS = 80,8 ± 22,2, DEP = 89,7 ± 34,2, DEM 25-75 = 71, 8 ± 32,8, Z score poids/taille = -0,41 ± 1,14, Z-score poids/âge = −0,48 ± 1,25, index de Quetelet = 15,4 ± 1,8, ration calorique = 107 ± 25 % des apports journaliers recommandes. Les donnees spirometriques et nutritionnelles ont ete analysees apres 1, 3, 6, 12, 18 et 24 mois de traitement et comparees aux valeurs de base (resultats spirometriques exprimes en pourcentage des valeurs de base moyennes). Le score de Shwachman-Kulczycki etait de 87 ± 9 avant le traitement et a ete releve 24 mois plus tard. Resultats Une amelioration de la CVF (+ 10,7 %, p p p ≤ 0,01 ). Ceci a ete note en particulier chez les enfants âges de 5 a 10 ans, chez les garcons et chez les patients dont la CVF de base etait e ( p e mois ( p ≤ 0,02), essentiellement chez les filles. Les Z scores poids/âge et poids/taille se sont ameliores respectivement pendant les 3 premiers mois et apres 2 ans de traitement. Le score de Shwachman-Kulczycki est reste stable. Conclusion La rhDNase ameliore donc la fonction respiratoire et le statut nutritionnel des jeunes patients atteints de mucoviscidose et la reponse au traitement peut etre evaluee des les 3 premiers mois.

Published
1998
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9. Application of quantitative PCR to the diagnosis and monitoring of Pseudomonas aeruginosa colonization in 5-18-year-old cystic fibrosis patients

Author

Marie-Odile Husson, René Courcol, Nathalie Wizla-Derambure, Stéphanie Herwegh, Dominique Turck, and Typhaine Billard-Pomares

Subjects
Microbiology (medical), Male, medicine.medical_specialty, Microbiological culture, Time Factors, Adolescent, Cystic Fibrosis, medicine.disease_cause, Microbiology, Group A, Cystic fibrosis, Polymerase Chain Reaction, Sensitivity and Specificity, Group B, Internal medicine, medicine, Pneumonia, Bacterial, Humans, Pseudomonas Infections, Child, Bacteriological Techniques, Pseudomonas aeruginosa, business.industry, Respiratory disease, Sputum, General Medicine, medicine.disease, Anti-Bacterial Agents, Concomitant, Child, Preschool, Immunology, Female, medicine.symptom, Drug Monitoring, business, Follow-Up Studies
Abstract

Early detection of Pseudomonas aeruginosa and early aggressive treatment are recommended to delay chronic infection in cystic fibrosis (CF) patients. The aim of this study was to assess a quantitative PCR (q-PCR) assay for the diagnosis of early P. aeruginosa colonization in 23 young CF patients (group A, age range 7–18 years) and to survey the eradication of P. aeruginosa in 10 young CF patients (group B, age range 5–18 years) after an initial antibiotic treatment. q-PCR results for consecutive sputum samples from each patient during a period of 18 months were compared with bacterial cultures during the same period plus an additional period of 12 months, and with concomitant clinical signs of pulmonary exacerbation. The q-PCR and bacterial cultures were negative for 17 of the 23 patients in group A and six of the 10 patients in group B during the study period. However, consecutive positive q-PCR results were observed for one patient in group A and three patients in group B, while the bacterial cultures for the same sputum sample remained negative. They preceded positive P. aeruginosa bacterial cultures at 7 and 8 months for two patients in group B. These positive results were associated with a worsening of the clinical status of patients, but pulmonary exacerbation appeared non-specific for the diagnosis of early P. aeruginosa colonization since pulmonary exacerbations were observed in patients in whom q-PCR or bacterial culture remained negative. In conclusion, q-PCR may be a useful additional tool to provide information on the P. aeruginosa status of CF patients.

Published
2010

10. Continuous versus Intermittent Infusions of Ceftazidime for Treating Exacerbation of Cystic Fibrosis▿

Author

Dominique Manach, Philippe Scheid, Thibaud Lavrut, E. Deneuville, Isabelle Sermet-Gaudelus, Rodolphe Garraffo, Anne Sardet, Nathalie Wizla, Evelyne Le Roux, Dominique Hubert, Benoit Wallaert, S. Ramel, Dominique Grenet, and Claire Cracowski

Subjects
Adult, Male, medicine.medical_specialty, Exacerbation, Adolescent, Cystic Fibrosis, Ceftazidime, Clinical Therapeutics, Drug Administration Schedule, Young Adult, Blood serum, Pharmacokinetics, Tobramycin, medicine, Humans, Pharmacology (medical), Antibacterial agent, Pharmacology, Cross-Over Studies, business.industry, Crossover study, Surgery, Anti-Bacterial Agents, Regimen, Infectious Diseases, Anesthesia, Female, business, medicine.drug
Abstract

The present multicenter, randomized crossover study compared the safety and efficacy of continuous infusion with those of short infusions of ceftazidime in patients with cystic fibrosis. Patients with chronic Pseudomonas aeruginosa colonization received two successive courses of intravenous tobramycin and ceftazidime (200 mg/kg of body weight/day) for pulmonary exacerbation administered as thrice-daily short infusions or as a continuous infusion. The primary endpoint was the variation in the forced expiratory volume in 1 s (FEV 1 ) during the course of antibiotic treatment. Sixty-nine of the 70 patients enrolled in the study received at least one course of antibiotic treatment. The improvement in FEV 1 at the end of therapy was not statistically different between the two treatment procedures (+7.6% after continuous infusion and +5.5% after short infusions) but was better after continuous ceftazidime treatment in patients harboring resistant isolates ( P < 0.05). The interval between the course of antibiotic treatments was longer after the continuous infusion than after the short infusion of ceftazidime ( P = 0.04). The mean serum ceftazidime concentration during the continuous infusion was 56.2 ± 23.2 μg/ml; the mean peak and trough concentrations during the short infusions were 216.3 ± 71.5 and 12.1 ± 8.7 μg/ml, respectively. The susceptibility profiles of the P. aeruginosa isolates remained unchanged and were similar for both regimens. Quality-of-life scores were similar whatever the treatment procedure, but 82% of the patients preferred the continuous-infusion regimen. Adverse events were not significantly different between the two regimens. In conclusion, the continuous infusion of ceftazidime did not increase its toxicity and appeared to be as efficient as short infusions in patients with cystic fibrosis as a whole, but it gave better results in patients harboring resistant isolates of P. aeruginosa .

Published
2009

11. Evaluation and mangement of fungal risk in cystic fibrosis: first results of a national French study

Author

V. Conseil, Annlyse Fanton, C. Person, Sylvie Leroy, Christophe Marguet, Laurence Delhaes, O. Vagnier, Frédéric Dalle, I. Accoceberry, C. Pinel, Gilles Gargala, Stéphanie Bui, P. Domblides, Stéphane Dominique, I. Durand-Joly, Jean-Philippe Bouchara, Catherine Llerena, G.-A.L. Loeuille, Yolande Lemeille, Marc Pihet, Amale Boldron, Nathalie Wizla, L. Favenne, Bérengère Coltey, J.-L. Giniès, Groupe d'Étude des Interactions Hôte-Pathogène (GEIHP), and Université d'Angers (UA)

Subjects
Pulmonary and Respiratory Medicine, medicine.medical_specialty, business.industry, Family medicine, [SDV]Life Sciences [q-bio], Pediatrics, Perinatology and Child Health, medicine, Pediatrics, Perinatology, and Child Health, medicine.disease, business, Cystic fibrosis, 3. Good health
Abstract

175 Evaluation and management of fungal risk in Cystic Fibrosis: first results of a national French study S. Leroy1, V. Conseil1, B. Coltey2, Y. Lemeille2, S. Dominique3, G. Gargala3, P. Domblides4, I. Accoceberry4, G. Loeuille5, I. Durand-Joly5, A. Fanton6, O. Vagnier6, F. Dalle6, A. Boldron5, C. Llerena2, C. Pinel2, J.L. Ginies7, M. Pihet7, C. Person7, J. Bouchara7, N. Wizla1, C. Marguet3, L. Favenne3, S. Bui4, L. Delhaes1. 1CHRU, Lille, France; 2CHU, Grenoble, France; 3CHU, Rouen, France; 4CHU, Bordeaux, France; 5CHG, Dunkerque, France; 6CHU, Dijon, France; 7CHU, Angers, France

Published
2009

12. Energetic cost of physical activity in cystic fibrosis children during Pseudomonas aeruginosa pulmonary exacerbation

Author

H. Vodougnon, Régis Hankard, M. O. Husson, Laurent Michaud, Frédéric Gottrand, Laurent Béghin, Nathalie Wizla-Derambure, and Dominique Turck

Subjects
Male, medicine.medical_specialty, Adolescent, Cystic Fibrosis, Physical exercise, Critical Care and Intensive Care Medicine, Cystic fibrosis, Predictive Value of Tests, Internal medicine, Heart rate, medicine, Humans, Resting energy expenditure, Pseudomonas Infections, Child, Exercise, Nutrition and Dietetics, business.industry, Area under the curve, medicine.disease, Intensity (physics), Anti-Bacterial Agents, Respiratory Function Tests, Basal metabolic rate, Pseudomonas aeruginosa, Breathing, Physical therapy, Cardiology, Exercise Test, Female, business, Energy Metabolism
Abstract

Chronic pulmonary infection by Pseudomonas aeruginosa is observed in 50% of patients with cystic fibrosis and requires the use of recurrent intravenous therapy. A decrease of resting energy expenditure (REE) and an increase of physical activity (PA) after intravenous anti-P. aeruginosa therapy (IVAT) is observed while total energy expenditure (TEE) does not change. A decrease in the energetic cost of physical activity (ECPA) could be hypothesized but has never been studied. Our aim was to assess the evolution of ECPA after home IVAT in both standardized condition at hospital and in free-living condition twice before and after IVAT. Sixteen CF patients (nine boys, seven girls) chronically colonized by P. aeruginosa with a mean age of 12.1+/-2.3 years (range 7.1-14.6) were studied before and after IVAT. Each patient passed throughout a visit in hospital: weight, height and fat-free mass were measured. Then, energy expenditure (EE) measured by indirect calorimetry and heart rate (HR) were simultaneously recorded at different levels of PA: REE, and at different intensity of physical activities on a cycloergometer using an incremental increase of the power brake force. Physical activity energy expenditure (PAEE) was computed in laboratory condition using PAEE=EE-BEE (basal energy expenditure). Linear regression between PAEE and power brake force was fitted for each patient before and after IVAT. ECPA in standardized conditions was compared at different range of power brake force using area under the curve (AUC). After coming back at home, 24 h TEE using the heart rate monitoring technique and PA by triaxial accelerometry were simultaneously measured in free-living condition for 24 h during a school day. ECPA in free-living conditions was compared by the ratio PAEE:PA where PAEE=DEE-REE (DEE=daily energy expenditure). After IVAT, median AUC between 60 and 90 W in standardized condition decreased significantly by -15.4% (median 14.9, range 8.8-30.3 vs. median 12.6, range 8.5-17.6; P0.05, Wilcoxon rank test) while the decrease for lower range of power work load did not reach significance. Spearman correlation was significant between variations of forced expiratory volume in 1 s and variation of AUC at 30-60 W before and after IVAT in standardized condition. In free-living conditions, ratio PAEE/PA did not vary significantly (median 3.4, range 1.6-6.4 vs. median 2.8, range 1.4-4.8; NS). Our data demonstrate a decrease of ECPA after IVAT in standardized conditions for moderate level of PA (60-90 W), but not in free-living conditions. The decrease of ECPA was probably due to a decrease in the energetic cost of breathing after IVAT, that is particularly relevant to promote PA in CF patients.

Published
2003

13. Natural outcome of Helicobacter pylori infection in asymptomatic children: a two-year follow-up study

Author

Laurent Michaud, Frédéric Gottrand, P. Vincent, Nathalie Wizla-Derambure, Marie-Odile Husson, Elisabeth Martin Delassalle, Dominique Turck, and P. S. Ganga-Zandzou

Subjects
Male, medicine.medical_specialty, Spirillaceae, Physical examination, Gastroenterology, Asymptomatic, Helicobacter Infections, Bacterial Proteins, Internal medicine, medicine, CagA, Humans, Prospective Studies, Intestinal Mucosa, Prospective cohort study, Child, Antigens, Bacterial, biology, medicine.diagnostic_test, Helicobacter pylori, business.industry, biology.organism_classification, Prognosis, Child, Preschool, Gastritis, Pediatrics, Perinatology and Child Health, Immunology, Cohort, Female, medicine.symptom, business
Abstract

Background and Objectives. It is known that Helicobacter pylori can be acquired in early childhood. There is not enough data to know whether or not infected children should be treated. A better knowledge of the natural outcome and implications of H pylori infection may provide evidence that eradication therapy is beneficial in childhood. This prospective study looks at clinical symptoms, endoscopic, microbial, and histologic changes during a 2-year period in infected asymptomatic children. It is hoped that some prognostic indicators will be found that select out the children that later need therapy. Patients and Methods. During epidemiologic study of the prevalence of H pylori infection, 18 children aged 7 ± 4 years (mean ± 1 SD) were discovered to have H pylori infection and enrolled in the 2-year follow-up study. These patients had received no eradication therapy because they were asymptomatic. The follow-up for each patient consisted of an initial assessment, a clinical examination every 6 months, and an endoscopic reevaluation at the end of the first and second years. Gastric mucosal samples were analyzed for bacteriologic and histologic changes. Various factors were initially recorded: individual factors included sex, age, and housing conditions; microbial factors included bacterial load and the presence of the CagA gene. Inflammatory changes were also noted, such as the presence of active gastritis and nodular formation, and these were correlated with the histology which was described using the Sydney classification. Typing polymerase chain reaction-restriction fragment length polymorphism was performed to check the persistence of the same strain of H pyloriin each patient. Results. All of the children were still infected after 2 years with the same strain as in the initial assessment with the exception of 1 child whose infection cleared spontaneously. The density of antral and fundal mucosal colonization with H pylorialso remained stable. There were progressive inflammatory changes in this cohort, particularly between the first and second year (histologic score, 3.5 ± 1.3 vs 5 ± 1). Active antral gastritis occurred in 3 out of 14 and 1 out of 8 children during the first and second year, respectively. Gastritis became active in the fundus in 2 out of 14 and 2 out of 8 children during the same period. Increases in the histologic score were found particularly in male children, and children colonized by cagA− strains of H pylori during the follow-up. The frequency of nodular gastritis significantly rose from 11% (2 out of 18 children) to 64% (9 out of 14 children) after 1 year, and to 80% (8 out of 10 children) after 2 years. Conclusion. These findings demonstrate a deterioration in the histologic features of the gastric mucosa of infected children despite stable H pylori colonization and the absence of symptoms.

Published
1999

14. 7 Mutation analysis of CFTR gene in a region of North of France: uncommon and novel mutations

Author

V. Desfachelles, G.-A.L. Loeuille, F. Delepoulle, M. Scalbert, E. Leroy, G. Lalau, Nathalie Wizla, and Amale Boldron

Subjects
Pulmonary and Respiratory Medicine, Genetics, business.industry, Pediatrics, Perinatology and Child Health, medicine, Mutation testing, Pediatrics, Perinatology, and Child Health, medicine.disease, business, Cystic fibrosis, Cftr gene
Published
2011
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15. A new water-soluble oral vitamin E formulation in cystic fibrosis (CF) children

Author

Frédéric Huet, Michel Roussey, E. Darviot, Nathalie Wizla, Françoise Troussier, Anne Munck, B. Delaisi, Dominique Turck, Y. Kibleur, E. Deneuville, S. Perez-Martin, J.-L. Giniès, and M. Gerardin

Subjects
Pulmonary and Respiratory Medicine, medicine.medical_specialty, Pathology, business.industry, medicine.disease, Cystic fibrosis, Gastroenterology, Water soluble, Internal medicine, Pediatrics, Perinatology and Child Health, medicine, Oral vitamin, Pediatrics, Perinatology, and Child Health, business
Published
2010
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17. NUTRITIONAL IMPACT OF RECOMBINANT HUMAN DEOXYRIBONUCLEASE (rhDNase) IN CYSTIC FIBROSIS (CF) PATIENTS

Author

Dominique Turck, A Sardet, Antoine Deschildre, E Tassin, Frédéric Gottrand, Nathalie Wizla-Derambure, C. Buisine, E. Boutry, N. Loire, Laurent Michaud, and G A Loeuille

Subjects
Pathology, medicine.medical_specialty, business.industry, Gastroenterology, Deoxyribonuclease, medicine.disease, Cystic fibrosis, law.invention, law, Pediatrics, Perinatology and Child Health, Immunology, Recombinant DNA, Medicine, business
Published
1998
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18. Gastric bacterial overgrowth is a cause of false positive diagnosis of Helicobacter pylori infection using 13C urea breath test

Author

P. Vincent, P. S. Ganga-Zandzou, Dominique Turck, Laurent Michaud, Frédéric Gottrand, and Nathalie Wizla-Derambure

Subjects
Breath test, Helicobacter pylori infection, medicine.medical_specialty, medicine.diagnostic_test, biology, Optimal test, business.industry, Stomach, Gastroenterology, Bacterial overgrowth, Helicobacter pylori, biology.organism_classification, H pylori infection, 13C urea breath test, medicine.anatomical_structure, Internal medicine, medicine, business
Abstract

Editor,—We read with interest the paper by Dominguez-Munos et al ( Gut 1997; 40 :459–62) describing an optimal test drink in the13C-urea breath test (13C UBT) for the diagnosis of Helicobacter pylori infection. In this study all H pylori negative subjects (adults with dyspeptic symptoms) had a negative result with the 13C UBT (specificity 100%) after different meals. In other studies, using13C UBT to document H pylori infection both in adults and children, the sensitivity of the test ranged from 92 to 100% whereas specificity was usually above 92%.1 2However, no explanation has been given for the occurrence of false positive tests. Methodological bias and problems in defining the cut off value are possible reasons. However, there are …

Published
1998
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