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1. 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

2. Lessons from a French collaborative case–control study in cystic fibrosis patients during the 2009 A/H1N1 influenza pandemy

Author: Julie Bucher, M. Lebourgeois, Dominique Hubert, Isabelle Sermet-Gaudelus, Odile Launay, Marianne Leruez-Ville, V. Boussaud, E. Deneuville, N. Remus, Isabelle Durieu, François Bremont, Marie Véronique Vodoff, Laurence Bassinet, Nathalie Stremler, Françoise Troussier, Pierre-Yves Boëlle, Dominique Grenet, Bertrand Delaisi, Jean-Marc Tréluyer, Harriet Corvol, CHU Necker - Enfants Malades [AP-HP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP), Institut Pierre Louis d'Epidémiologie et de Santé Publique (iPLESP), Université Pierre et Marie Curie - Paris 6 (UPMC)-Institut National de la Santé et de la Recherche Médicale (INSERM), Mucoviscidose et bronchopathies chroniques : biopathologie et phénotypes cliniques (EA 2511), Hôpital Cochin [AP-HP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Université Paris Descartes - Paris 5 (UPD5), Service de Pneumologie - CRCM adultes, Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Hôpital Cochin [AP-HP], Hôpital de la Timone [CHU - APHM] (TIMONE), 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), 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), Centre de Ressources et de Compétences de la Mucoviscidose, hôpital Sud, Service de gastro-entérologie, mucoviscidose, pneumologie et nutrition[Debré], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Hôpital Robert Debré-Université Paris Diderot - Paris 7 (UPD7), CHU Trousseau [APHP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU), Centre de Recherche Saint-Antoine (UMRS893), Service de Pneumologie [Créteil], Centre Hospitalier Intercommunal Créteil (CHIC), Hôpital Foch [Suresnes], Service de Pédiatrie [Créteil], CHI Créteil, Service de pédiatrie, Centre Hospitalier Universitaire d'Angers (CHU Angers), PRES Université Nantes Angers Le Mans (UNAM)-PRES Université Nantes Angers Le Mans (UNAM), Laboratoire de Virologie [CHU Necker], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-CHU Necker - Enfants Malades [AP-HP], Fédération pour la recherche en explorations et thérapeutiques innovantes in utéro (FETUS - EA 7328), Université Paris Descartes - Paris 5 (UPD5), Infections à Vih, Réservoirs, Pharmacologie des Antirétroviraux et Prévention de la Transmission Mère Enfant, 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) (AP-HP)-CHU Trousseau [APHP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU)-Sorbonne Université (SU), CHU Cochin [AP-HP]-Université Paris Descartes - Paris 5 (UPD5), CHU Cochin [AP-HP]-AP-HP, Pédiatrie - Pneumologie, Allergologie, Hôpital des Enfants, CHU Toulouse [Toulouse]-CHU Toulouse [Toulouse], Hôpital Sud, Assistance publique - Hôpitaux de Paris (AP-HP) (APHP)-Hôpital Robert Debré-Université Paris Diderot - Paris 7 (UPD7), Université Pierre et Marie Curie - Paris 6 (UPMC)-Assistance publique - Hôpitaux de Paris (AP-HP) (APHP)-CHU Trousseau [APHP], Centre de Recherche Saint-Antoine (CR Saint-Antoine), CHU Necker - Enfants Malades [AP-HP]-Assistance publique - Hôpitaux de Paris (AP-HP) (APHP), CHU Cochin [AP-HP], Institut Pierre Louis d'Epidémiologie et de Santé Publique ( iPLESP ), Institut National de la Santé et de la Recherche Médicale ( INSERM ) -Université Pierre et Marie Curie - Paris 6 ( UPMC ), Mucoviscidose et bronchopathies chroniques : biopathologie et phénotypes cliniques ( EA 2511 ), Université Paris Descartes - Paris 5 ( UPD5 ), Hôpital de la Timone [CHU - APHM] ( TIMONE ), Hospices Civils de Lyon ( HCL ) -Centre Hospitalier Lyon Sud [CHU - HCL] ( CHLS ), Hospices Civils de Lyon ( HCL ), Assistance publique - Hôpitaux de Paris (AP-HP)-Hôpital Robert Debré-Université Paris Diderot - Paris 7 ( UPD7 ), Service de pneumologie, CHU Trousseau [APHP]-Assistance publique - Hôpitaux de Paris (AP-HP)-Université Pierre et Marie Curie - Paris 6 ( UPMC ), Centre de Recherche Saint-Antoine ( CR Saint-Antoine ), CHU Angers, Assistance publique - Hôpitaux de Paris (AP-HP)-CHU Necker - Enfants Malades [AP-HP], Fédération pour la recherche en explorations et thérapeutiques innovantes in utéro ( FETUS - EA 7328 ), 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 ), Institut National de la Santé et de la Recherche Médicale (INSERM)-Université Pierre et Marie Curie - Paris 6 (UPMC), and Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)
Subjects: Male, medicine.medical_treatment, [SDV]Life Sciences [q-bio], Antibiotics, Cystic Fibrosis Transmembrane Conductance Regulator, Cystic fibrosis, chemistry.chemical_compound, 0302 clinical medicine, Medical microbiology, Influenza A Virus, H1N1 Subtype, Pandemic, Medicine, 030212 general & internal medicine, Prospective Studies, Child, virus diseases, 3. Good health, Anti-Bacterial Agents, Infectious Diseases, Child, Preschool, Pseudomonas aeruginosa, Female, medicine.symptom, Research Article, Adult, Oseltamivir, medicine.medical_specialty, Adolescent, medicine.drug_class, Antiviral Agents, 03 medical and health sciences, Young Adult, Internal medicine, Influenza, Human, A/H1N1 influenza, Lung transplantation, Humans, Pseudomonas Infections, Pandemics, [ SDV ] Life Sciences [q-bio], business.industry, Case-control study, Sputum, medicine.disease, respiratory tract diseases, 030228 respiratory system, chemistry, Case-Control Studies, Immunology, Mutation, business
Abstract: Background Viral infections such as influenza are thought to impact respiratory parameters and to promote infection with Pseudomonas aeruginosa in patients with cystic fibrosis (CF). However, the real morbidity of the influenza virus in CF needs to be further investigated because previous studies were only observational. Methods CF patients were included in a case–control study (n = 44 cases and n = 371 controls) during the 2009 pandemic A/H1N1 influenza. Cases were patients with polymerase reaction chain-confirmed influenza A/H1N1 infection. Controls did not report any influenza symptoms during the same period. Sputum colonization and lung function were monitored during 1 year after inclusion. Results Cases were significantly younger than controls (mean(SD) 14.9 years(11) versus 20.1 years (13.2) and significantly less frequently colonized with P. aeruginosa (34 % versus 53 %). During influenza infection, 74 % of cases had pulmonary exacerbation, 92 % had antibiotics adapted to their usual sputum colonization and 82 % were treated with oseltamivir. Two cases required lung transplantation after A/H1N1 infection (one had not received oseltamivir and the other one had been treated late). The cases received a mean number of antibiotic treatments significantly higher during the year after the influenza infection (mean(SD) 2.8 (2.4) for cases versus 1.8(2.1) for controls; p = 0.002). An age-matched comparison did not demonstrate any significant modification of bronchopulmonary bacterial colonization during the year after influenza infection nor any significant change in FEV1 at months 1, 3 and 12 after A/H1N1 infection. Conclusions Our results do not demonstrate any change in sputum colonization nor significant lung disease progression after pandemic A/H1N1 influenza. Trial registration Clinical Trials.gov registration number: NCT01499914
Published: 2016

3. Analysis of the upper airway by the acoustic reflection method in children with mucopolysaccharidosis

Author: Bénédicte Heron, Raphaël Vialle, Brigitte Fauroux, Nicolas Leboulanger, Bruno Louis, CHU Trousseau [APHP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU), 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 de Recherche Saint-Antoine (UMRS893), Université Pierre et Marie Curie - Paris 6 (UPMC)-Institut National de la Santé et de la Recherche Médicale (INSERM), Service d'ORL pédiatrique et Chirurgie Cervico-faciale [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), Service de pneumologie [CHU Trousseau], Service de chirurgie orthopédique et reconstructive pédiatrique [CHU Trousseau], and Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-CHU Trousseau [APHP]
Subjects: Pulmonary and Respiratory Medicine, medicine.medical_specialty, Mucopolysaccharidosis, Single Center, 03 medical and health sciences, 0302 clinical medicine, Airway resistance, children, Internal medicine, Medicine, Mucopolysaccharidosis type II, 030223 otorhinolaryngology, Prospective cohort study, upper airways, acoustic pharyngometry, business.industry, mucopolysaccharidosis, respiratory system, Airway obstruction, medicine.disease, Surgery, acoustic reflection method, El Niño, Pediatrics, Perinatology and Child Health, Cardiology, Airway, business, 030217 neurology & neurosurgery
Abstract: Background Upper airway obstruction is common in children with mucopolysaccharidosis. The acoustic reflection method is a noninvasive technique that can analyze the caliber of the upper airways. The aim of the study was to evaluate the feasibility of the acoustic reflection method in children with mucopolysaccharidosis, and to compare the characteristics of the upper airways evaluated by the acoustic reflection method in patients with mucopolysaccharidosis to matched healthy counterparts. Methods Open, single center, prospective, study. Results Accurate acoustic reflection measurements could be obtained in 7 of 10 patients (mean age: 10.4 ± 3.9 years; mucopolysaccharidosis type II (n = 3); type IV (n = 2), type VI (n = 1), and fucosidosis (n = 1)). The mean minimum cross-sectional area was lower in mucopolysaccharidosis patients (1.6 ± 0.3 cm2) as compared to 14 healthy counterparts (1.8 ± 0.3 cm2; P = 0.03). The mean resistance of the airways was significantly higher in the MPS group (7.9 ± 1.8 cmH2O l−1 sec) as compared to the controls (5.5 ± 1.2 cmH2O l−1 sec; P = 0.006). Conclusion This study is the first to analyze the upper airways by the noninvasive acoustic reflection method in children with mucopolysaccharidosis. Due to a lack of cooperation, reliable measurements could only be obtained in 70% of a selected group of patients. Children with mucopolysaccharidosis have significant upper airway obstruction as assessed by the reduction of the minimal cross-sectional area of the upper airways and the increase in airway resistance. Pediatr. Pulmonol. 2011; 46:587–594. © 2011 Wiley-Liss, Inc.
Published: 2011

4. Normal and Cystic Fibrosis Human Bronchial Epithelial Cells Infected with Pseudomonas aeruginosa Exhibit Distinct Gene Activation Patterns

Author: Viviane Balloy, Hugo Varet, Marie-Agnès Dillies, Caroline Proux, Bernd Jagla, Jean-Yves Coppée, Olivier Tabary, Harriet Corvol, Michel Chignard, Loïc Guillot, Centre de Recherche Saint-Antoine (UMRS893), Université Pierre et Marie Curie - Paris 6 (UPMC)-Institut National de la Santé et de la Recherche Médicale (INSERM), Transcriptome et Epigénome (PF2), Institut Pasteur [Paris], Service de pneumologie [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), 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), Institut Pasteur [Paris] (IP), CHU Trousseau [APHP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU), Sorbonne Université - Faculté de Médecine (SU FM), and Sorbonne Université (SU)
Subjects: Adult, Male, Transcriptional Activation, Cystic Fibrosis, lcsh:R, lcsh:Medicine, Bronchi, Epithelial Cells, Young Adult, Gene Expression Regulation, Pseudomonas aeruginosa, Humans, lcsh:Q, Female, Pseudomonas Infections, lcsh:Science, [SDV.MHEP]Life Sciences [q-bio]/Human health and pathology, Research Article
Abstract: International audience; Background and AimsIn cystic fibrosis (CF), Pseudomonas aeruginosa is not eradicated from the lower respiratory tract and is associated with epithelial inflammation that eventually causes tissue damage. To identify the molecular determinants of an effective response to P. aeruginosa infection, we performed a transcriptomic analysis of primary human bronchial epithelial cells from healthy donors (CTRL) 2, 4, and 6 h after induced P. aeruginosa infection. Compared to noninfected cells, infected cells showed changes in gene activity, which were most marked 6 h postinfection and usually consisted in upregulation.ResultsBy comparing for each time point of infection, the transcriptomic response of epithelial cells from CF patients and healthy donors, we identified 851, 638, 667, and 980 differentially expressed genes 0, 2, 4, and 6 h postinfection, respectively. Gene selection followed by bioinformatic analysis showed that most of the differentially expressed genes, either up- or downregulated, were in the protein-binding and catalytic gene-ontology categories. Finally, we established that the protein products of the genes exhibiting the greatest differential upregulation (CSF2, CCL2, TNF, CSF3, MMP1, and MMP10) between CF patients and CTRL were produced in higher amounts by infected cells from CF patients versus CTRL.ConclusionsThe differentially expressed genes in CF patients may constitute a signature for a detrimental inflammatory response and for an inefficient P. aeruginosa host-cell response.
Published: 2015

5. Idiopathic eosinophilic pneumonia in children: the French experience

Author: Jacques de Blic, Virginie Feret, François Bremont, Nadia Nathan, Jean Christophe Dubus, Thierry Leblanc, M. Albertini, Véronique Houdouin, Lisa Giovannini-Chami, Michèle Berlioz-Baudoin, Karine Morelle, Michael Fayon, Annick Clement, Alice Hadchouel, Service de Pneumologie Allergologie pédiatriques, Centre Hospitalier Universitaire de Nice (CHU Nice)-Hôpitaux pédiatriques de Nice CHU-Lenval, Université Nice Sophia Antipolis (... - 2019) (UNS), COMUE Université Côte d'Azur (2015-2019) (COMUE UCA), Service de Pneumologie Allergologie [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), Centre de Recherche Saint-Antoine (UMRS893), Université Pierre et Marie Curie - Paris 6 (UPMC)-Institut National de la Santé et de la Recherche Médicale (INSERM), Service de pneumologie [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), Pédiatrie - Pneumologie, Allergologie, Hôpital des Enfants, CHU Toulouse [Toulouse]-CHU Toulouse [Toulouse], Service de pneumologie pédiatrique, Assistance Publique - Hôpitaux de Marseille (APHM)- Hôpital de la Timone [CHU - APHM] (TIMONE)-CHU Marseille, CHU Bordeaux [Bordeaux], CIC - Bordeaux, Université Bordeaux Segalen - Bordeaux 2-CHU Bordeaux [Bordeaux]-Institut National de la Santé et de la Recherche Médicale (INSERM), Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Hôpital Robert Debré-Université Paris Diderot - Paris 7 (UPD7), Service d'hématologie et immunologie pédiatrique, the French Respirare® Group, CHU Nice-Hôpitaux pédiatriques de Nice CHU-Lenval, Université Nice Sophia Antipolis ( UNS ), Université Côte d'Azur ( UCA ), Assistance publique - Hôpitaux de Paris (AP-HP)-CHU Necker - Enfants Malades [AP-HP], Centre de Recherche Saint-Antoine ( CR Saint-Antoine ), Université Pierre et Marie Curie - Paris 6 ( UPMC ) -Institut National de la Santé et de la Recherche Médicale ( INSERM ), Service de pneumologie, Université Pierre et Marie Curie - Paris 6 ( UPMC ) -Assistance publique - Hôpitaux de Paris (AP-HP)-CHU Trousseau [APHP], Assistance Publique - Hôpitaux de Marseille ( APHM ) - Hôpital de la Timone [CHU - APHM] ( TIMONE ) -CHU Marseille, Université Bordeaux Segalen - Bordeaux 2-CHU Bordeaux [Bordeaux]-Institut National de la Santé et de la Recherche Médicale ( INSERM ), Assistance publique - Hôpitaux de Paris (AP-HP)-Hôpital Robert Debré-Université Paris Diderot - Paris 7 ( UPD7 ), BMC, Ed., Université Nice Sophia Antipolis (1965 - 2019) (UNS), 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), CHU Trousseau [APHP], and Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU)
Subjects: Male, Pediatrics, medicine.medical_specialty, Adolescent, medicine.medical_treatment, Idiopathic acute eosinophilic pneumonia, Eosinophilic lung disease, Interstitial lung disease, [SDV.GEN] Life Sciences [q-bio]/Genetics, Disease, medicine, Extracorporeal membrane oxygenation, Eosinophilic pneumonia, Humans, Genetics(clinical), Pharmacology (medical), Idiopathic chronic eosinophilic pneumonia, Pulmonary Eosinophilia, Child, Idiopathic interstitial pneumonia, Genetics (clinical), Retrospective Studies, Medicine(all), [SDV.GEN]Life Sciences [q-bio]/Genetics, business.industry, Research, Retrospective cohort study, General Medicine, medicine.disease, 3. Good health, Child, Preschool, Immunology, Female, France, [ SDV.GEN ] Life Sciences [q-bio]/Genetics, business, Tomography, X-Ray Computed, Bronchoalveolar Lavage Fluid, Rare disease
Abstract: International audience; BACKGROUND: Idiopathic eosinophilic pneumonia is extremely rare in children and adults. We present herein the first series describing the specificities of idiopathic chronic (ICEP) and acute (IAEP) eosinophilic pneumonia in children. METHODS: We retrospectively analyzed all cases of ICEP and IAEP in children that were retrieved from French Reference Centers for rare pediatric lung diseases. RESULTS: Five cases of pediatric ICEP were identified. Corticosteroid or immunosuppressive therapy dramatically improved the outcome in three cases. The remaining two cases had a persistent interstitial pattern with progressive development of cystic airspace lesions. Three cases of IAEP in adolescents were reported, with one requiring four days of extracorporeal membrane oxygenation. CONCLUSION: ICEP is a rare disease with a polymorphic clinical presentation in children. We identified patients with persistent interstitial patterns progressing to cystic airspace regions, for which the boundaries with idiopathic interstitial pneumonias are difficult to establish. We therefore propose a specific pediatric definition and classification algorithm. IAEP in children remains an inflammatory reaction of the lung to an acute toxic exposure, mainly tobacco, as in adults. International studies are required to comprehensively assess the various clinical forms of the disease as well as the appropriate therapeutic regimens.
Published: 2014

6. Evaluation of new minimally invasive methods of exploration of pediatric upper airways

Author: Leboulanger, Nicolas, Bupmc, Theses, Service d'ORL pédiatrique et Chirurgie Cervico-faciale [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), 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 de Recherche Saint-Antoine (UMRS893), Université Pierre et Marie Curie - Paris 6 (UPMC)-Institut National de la Santé et de la Recherche Médicale (INSERM), Service de pneumologie [CHU Trousseau], Université Pierre et Marie Curie - Paris VI, Brigitte FAUROUX, and Bruno LOUIS
Subjects: Enfant, Travail respiratoire, Obstruction, Exploration non-invasive, Méthode de réflexion acoustique, [SDV.MHEP.PSR] Life Sciences [q-bio]/Human health and pathology/Pulmonology and respiratory tract, Voies aériennes supérieures, [SDV.MHEP.PSR]Life Sciences [q-bio]/Human health and pathology/Pulmonology and respiratory tract
Abstract: The management of obstructive diseases of the pediatric upper airway (UA) is currently based on the evaluation of the obstruction during an endoscopy with general anesthesia. This procedure has a significant morbidity. The difficulty of quantifying UA obstruction and its impact with simple and accurate tools emphasizes the importance of evaluating new techniques. The objective of this work was to study new methods of exploration, minimally or non- invasive, of these obstructive diseases: the acoustic reflection method (ARM) and measurement of work of breathing. The ARM is based on the analysis of the reflection of a pressure wave in the UA. The work of breathing studies the load imposed on the respiratory muscles during breathing. We demonstrated that the MRA was applicable in children over 2 years, with a standardized technique. Measurements on children with cystic fibrosis and mucopolysaccharidosis were conducted, to assess the UA resistance and its evolution during the course of the disease. In younger children, when the ARM is possible, we demonstrated in infants with a Robin sequence having an increase in the work of breathing that noninvasive ventilation was able to reduce the load imposed on respiratory muscles and to improve the clinical course. Non- or minimally invasive techniques exploring the UA in children are promising because simple, safe, and potentially complementary to other more invasive tests. Their achievement is routinely possible in many cases, La prise en charge des pathologies obstructives des voies aériennes supérieures (VAS) de l'enfant est actuellement basée sur l'évaluation de l'obstruction lors d'une endoscopie sous anesthésie générale. Cet examen a une morbidité non négligeable et les contraintes pesant sur un enfant atteint d'une pathologie obstructive des VAS sont lourdes. La difficulté de quantifier de manière simple et précise l'obstruction des VAS et son retentissement justifie l'évaluation de nouvelles techniques. L'objectif de ce travail a été d'étudier des méthodes d'explorations, non ou peu invasives, de ces pathologies obstructives : la méthode de réflexion acoustique (MRA) et la mesure du travail respiratoire. La MRA est basée sur l'analyse de la réflexion d'une onde sonore dans les VAS. Le travail respiratoire est la mesure de l'effort fourni par les muscles respiratoires au cours de la respiration. Nous avons démontré que la MRA était applicable chez l'enfant de plus de 2 ans. Des mesures sur des enfants atteints de mucoviscidose et de mucopolysaccharidose ont permis d'étudier les résistances des VAS et leur évolution au cours de la maladie. Chez l'enfant plus jeune, quand la MRA est impossible, nous avons mis en évidence chez des nourrissons atteints d'une séquence de Robin et présentant un travail respiratoire très augmenté qu'une ventilation non invasive était capable de diminuer l'effort fourni et d'améliorer l'évolution clinique Les méthodes d'explorations peu ou non invasives des VAS de l'enfant sont prometteuses car simples, sans danger, et potentiellement complémentaires d'autres examens plus invasifs. Leur réalisation en routine est envisageable dans de nombreux cas de figure
Published: 2012

7. Evaluation of new minimally invasive methods of exploration of pediatric upper airways

Author: Leboulanger, Nicolas, Bupmc, Theses, Service d'ORL pédiatrique et Chirurgie Cervico-faciale [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), 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 de Recherche Saint-Antoine (UMRS893), Université Pierre et Marie Curie - Paris 6 (UPMC)-Institut National de la Santé et de la Recherche Médicale (INSERM), Service de pneumologie [CHU Trousseau], Université Pierre et Marie Curie - Paris VI, Brigitte FAUROUX, Bruno LOUIS, CHU Trousseau [APHP], and Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU)
Subjects: Enfant, Travail respiratoire, Obstruction, Exploration non-invasive, Méthode de réflexion acoustique, [SDV.MHEP.PSR] Life Sciences [q-bio]/Human health and pathology/Pulmonology and respiratory tract, Voies aériennes supérieures, [SDV.MHEP.PSR]Life Sciences [q-bio]/Human health and pathology/Pulmonology and respiratory tract
Abstract: The management of obstructive diseases of the pediatric upper airway (UA) is currently based on the evaluation of the obstruction during an endoscopy with general anesthesia. This procedure has a significant morbidity. The difficulty of quantifying UA obstruction and its impact with simple and accurate tools emphasizes the importance of evaluating new techniques. The objective of this work was to study new methods of exploration, minimally or non- invasive, of these obstructive diseases: the acoustic reflection method (ARM) and measurement of work of breathing. The ARM is based on the analysis of the reflection of a pressure wave in the UA. The work of breathing studies the load imposed on the respiratory muscles during breathing. We demonstrated that the MRA was applicable in children over 2 years, with a standardized technique. Measurements on children with cystic fibrosis and mucopolysaccharidosis were conducted, to assess the UA resistance and its evolution during the course of the disease. In younger children, when the ARM is possible, we demonstrated in infants with a Robin sequence having an increase in the work of breathing that noninvasive ventilation was able to reduce the load imposed on respiratory muscles and to improve the clinical course. Non- or minimally invasive techniques exploring the UA in children are promising because simple, safe, and potentially complementary to other more invasive tests. Their achievement is routinely possible in many cases, La prise en charge des pathologies obstructives des voies aériennes supérieures (VAS) de l'enfant est actuellement basée sur l'évaluation de l'obstruction lors d'une endoscopie sous anesthésie générale. Cet examen a une morbidité non négligeable et les contraintes pesant sur un enfant atteint d'une pathologie obstructive des VAS sont lourdes. La difficulté de quantifier de manière simple et précise l'obstruction des VAS et son retentissement justifie l'évaluation de nouvelles techniques. L'objectif de ce travail a été d'étudier des méthodes d'explorations, non ou peu invasives, de ces pathologies obstructives : la méthode de réflexion acoustique (MRA) et la mesure du travail respiratoire. La MRA est basée sur l'analyse de la réflexion d'une onde sonore dans les VAS. Le travail respiratoire est la mesure de l'effort fourni par les muscles respiratoires au cours de la respiration. Nous avons démontré que la MRA était applicable chez l'enfant de plus de 2 ans. Des mesures sur des enfants atteints de mucoviscidose et de mucopolysaccharidose ont permis d'étudier les résistances des VAS et leur évolution au cours de la maladie. Chez l'enfant plus jeune, quand la MRA est impossible, nous avons mis en évidence chez des nourrissons atteints d'une séquence de Robin et présentant un travail respiratoire très augmenté qu'une ventilation non invasive était capable de diminuer l'effort fourni et d'améliorer l'évolution clinique Les méthodes d'explorations peu ou non invasives des VAS de l'enfant sont prometteuses car simples, sans danger, et potentiellement complémentaires d'autres examens plus invasifs. Leur réalisation en routine est envisageable dans de nombreux cas de figure
Published: 2012

8. Value of gas exchange recording at home in children receiving noninvasive ventilation: Gas exchange during noninvasive ventilation

Author: Osama, Felemban, Karl, Leroux, Guillaume, Aubertin, Fabrice, Miandy, Frédéric, Damagnez, Bruno, Amorim, Adriana, Ramirez, Brigitte, Fauroux, Service de pneumologie [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), Pediatric department, Faculty of Medicine, King Abdulaziz University, ADEP Assistance, ADEP assistance, Centre de Recherche Saint-Antoine (UMRS893), Université Pierre et Marie Curie - Paris 6 (UPMC)-Institut National de la Santé et de la Recherche Médicale (INSERM), The research of Brigitte Fauroux is supported by the Association Française contre les Myopathies (AFM), Assistance Publique-Hôpitaux de Paris, Inserm, ADEP Assistance, and Université Pierre et Marie Curie-Paris 6., CHU Trousseau [APHP], and Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU)
Subjects: Male, child, Adolescent, Pulmonary Gas Exchange, carbon dioxide, Neuromuscular Diseases, gas exchange, home, pulse oximetry, Oxygen, Positive-Pressure Respiration, Young Adult, Child, Preschool, Humans, Female, Oximetry, noninvasive positive pressure ventilation, Blood Gas Monitoring, Transcutaneous
Abstract: International audience; Noninvasive positive pressure ventilation (NPPV) is preferentially performed at home. The objectives of the study were to evaluate the feasibility of an overnight gas exchange recording at home and to compare recordings performed in the hospital and at home. Twenty nine pairs of overnight gas exchange recordings during NPPV were performed at home and in the hospital in 11 children with neuromuscular disease and 13 children with other disorders treated with long term NPPV. No technical problem occurred during the recordings performed at home and one pulse oximetry (SpO2) recording failed in the hospital. For the two groups, SpO2 and transcutaneous carbon dioxide (PtcCO2) values did not differ significantly between the hospital and the home. However, correlations between SpO2 and PtcCO2 values obtained in the hospital and at home were better for mean values than for minimal and maximal values, and in patients with other disorders as compared to patients with neuromuscular disease. Overnight gas exchange recordings with NPPV by a combined PtcCO2/SpO2 monitor are feasible at home and show results comparable to hospital recordings. Home PtcCO2/SpO2 recordings may be integrated in the care of children treated with domiciliary NPPV and are associated with less disruption of family life and decreased health care costs.
Published: 2011

9. Value of gas exchange recording at home in children receiving noninvasive ventilation

Author: Felemban, Osama, Leroux, Karl, Aubertin, Guillaume, Miandy, Fabrice, Damagnez, Frederic, Amorim, Bruno, Ramirez, Adriana, Fauroux, Brigitte, Peer, Hal, Service de pneumologie [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), Pediatric department, Faculty of Medicine, King Abdulaziz University, ADEP Assistance, ADEP assistance, Centre de Recherche Saint-Antoine (UMRS893), Université Pierre et Marie Curie - Paris 6 (UPMC)-Institut National de la Santé et de la Recherche Médicale (INSERM), and The research of Brigitte Fauroux is supported by the Association Française contre les Myopathies (AFM), Assistance Publique-Hôpitaux de Paris, Inserm, ADEP Assistance, and Université Pierre et Marie Curie-Paris 6.
Subjects: child, carbon dioxide, gas exchange, home, noninvasive positive pressure ventilation, pulse oximetry
Abstract: International audience; Noninvasive positive pressure ventilation (NPPV) is preferentially performed at home. The objectives of the study were to evaluate the feasibility of an overnight gas exchange recording at home and to compare recordings performed in the hospital and at home. Twenty nine pairs of overnight gas exchange recordings during NPPV were performed at home and in the hospital in 11 children with neuromuscular disease and 13 children with other disorders treated with long term NPPV. No technical problem occurred during the recordings performed at home and one pulse oximetry (SpO2) recording failed in the hospital. For the two groups, SpO2 and transcutaneous carbon dioxide (PtcCO2) values did not differ significantly between the hospital and the home. However, correlations between SpO2 and PtcCO2 values obtained in the hospital and at home were better for mean values than for minimal and maximal values, and in patients with other disorders as compared to patients with neuromuscular disease. Overnight gas exchange recordings with NPPV by a combined PtcCO2/SpO2 monitor are feasible at home and show results comparable to hospital recordings. Home PtcCO2/SpO2 recordings may be integrated in the care of children treated with domiciliary NPPV and are associated with less disruption of family life and decreased health care costs.
Published: 2011

10. Analysis of the upper airway by the acoustic reflection method in children with mucopolysaccharidosis: Acoustic reflection in mucopolysaccharidosis

Author: Leboulanger, Nicolas, Louis, Bruno, Vialle, Raphael, Heron, Benedicte, Fauroux, Brigitte, Peer, Hal, Service d'ORL pédiatrique et Chirurgie Cervico-faciale [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), 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 de Recherche Saint-Antoine (UMRS893), Université Pierre et Marie Curie - Paris 6 (UPMC)-Institut National de la Santé et de la Recherche Médicale (INSERM), Service de pneumologie [CHU Trousseau], Service de chirurgie orthopédique et reconstructive pédiatrique [CHU Trousseau], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-CHU Trousseau [APHP], CHU Trousseau [APHP], and Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU)
Subjects: acoustic reflection method, upper airways, acoustic pharyngometry, children, mucopolysaccharidosis, respiratory system
Abstract: International audience; Background Upper airway obstruction is common in children with mucopolysaccharidosis. The acoustic reflection method is a noninvasive technique that can analyse the calibre of the upper airways. The aim of the study was to evaluate the feasibility of the acoustic reflection method in children with mucopolysaccharidosis, and to compare the characteristics of the upper airways evaluated by the acoustic reflection method in patients with mucopolysaccharidosis to matched healthy counterparts. Methods Open, single centre, prospective, study Results Accurate acoustic reflection measurements could be obtained in 7 of 10 patients (mean age: 10.4 ± 3.9 years; mucopolysaccharidosis type II (n=3); type IV (n=2), type VI (n=1), and fucosidosis (n=1)). The mean minimum cross-sectional area was lower in mucopolysaccharidosis patients (1.6 ± 0.3 cm²) as compared to 14 healthy counterparts (1.8 ± 0.3 cm²; p=0.03). The mean resistance of the airways was significantly higher in the MPS group (7.9 ± 1.8 cmH2O.l-1.s) as compared to the controls (5.5 ± 1.2 cmH2O.l-1.s; p=0.006). Conclusion This study is the first to analyze the upper airways by the noninvasive acoustic reflection method in children with mucopolysaccharidosis. Due to a lack of cooperation, reliable measurements could only be obtained in 70% of a selected group of patients. Children with mucopolysaccharidosis have significant upper airway obstruction as assessed by the reduction of the minimal cross-sectional area of the upper airways and the increase in airway resistance.
Published: 2011

11. Otologic features in children with primary ciliary dyskinesia

Author: Prulière-Escabasse, Virginie, Coste, André, Chauvin, Pierre, Fauroux, Brigitte, Tamalet, Aline, Garabedian, Erea-Noel, Escudier, Estelle, Roger, Gilles, INSERM U955, équipe 13, Service d'ORL et de chirurgie cervico-faciale, Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Hôpital Henri Mondor-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Hôpital Henri Mondor-Institut Mondor de Recherche Biomédicale (IMRB), Institut National de la Santé et de la Recherche Médicale (INSERM)-IFR10-Université Paris-Est Créteil Val-de-Marne - Paris 12 (UPEC UP12)-Institut National de la Santé et de la Recherche Médicale (INSERM)-IFR10-Université Paris-Est Créteil Val-de-Marne - Paris 12 (UPEC UP12), ESIM - Déterminants Sociaux de la Santé et du Recours aux Soins (DS3), Université Pierre et Marie Curie - Paris 6 (UPMC)-Institut National de la Santé et de la Recherche Médicale (INSERM), Service de pneumologie [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), Centre de Recherche Saint-Antoine (UMRS893), Service d'ORL pédiatrique et Chirurgie Cervico-faciale [CHU Trousseau], Service de génétique et embryologie médicales [CHU Trousseau], 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)-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)-Physiopathologie des maladies génétiques d'expression pédiatrique, Université Pierre et Marie Curie - Paris 6 (UPMC)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Université Pierre et Marie Curie - Paris 6 (UPMC)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Institut Mondor de Recherche Biomédicale (IMRB), Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU), 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), and Institut National de la Santé et de la Recherche Médicale (INSERM)-Université Paris-Est Créteil Val-de-Marne - Paris 12 (UPEC UP12)-IFR10-Institut National de la Santé et de la Recherche Médicale (INSERM)-Université Paris-Est Créteil Val-de-Marne - Paris 12 (UPEC UP12)-IFR10
Subjects: MESH: Adolescent, MESH: Bronchiectasis, Kartagener syndrome, MESH: Chi-Square Distribution, MESH: Humans, MESH: Child, Preschool, cilia, serous otitis media, MESH: Retrospective Studies, macromolecular substances, MESH: Infant, MESH: Male, MESH: Otitis Media, MESH: Audiometry, MESH: Anti-Bacterial Agents, MESH: Child, otorhinolaryngologic diseases, MESH: Kartagener Syndrome, [SDV.BBM]Life Sciences [q-bio]/Biochemistry, Molecular Biology, sense organs, MESH: Hearing Loss, conductive hearing loss, MESH: Female
Abstract: International audience; Despite continuous antibiotic therapy, the middle ear condition in PCD remained severe throughout childhood, with improvement only after age 18 years. Armstrong grommet placement did not improve the middle ear condition. Central complex defect is a marker of severity.
Published: 2010

12. Effect of manufacturer-inserted mask leaks on ventilator performance

Author: Karl Leroux, Bruno Louis, Brigitte Fauroux, Frédéric Lofaso, Daniel Isabey, INSERM U955, équipe 13, Institut Mondor de Recherche Biomédicale (IMRB), Institut National de la Santé et de la Recherche Médicale (INSERM)-IFR10-Université Paris-Est Créteil Val-de-Marne - Paris 12 (UPEC UP12)-Institut National de la Santé et de la Recherche Médicale (INSERM)-IFR10-Université Paris-Est Créteil Val-de-Marne - Paris 12 (UPEC UP12), ADEP assistance, CHU Trousseau [APHP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU), Centre de Recherche Saint-Antoine (UMRS893), Université Pierre et Marie Curie - Paris 6 (UPMC)-Institut National de la Santé et de la Recherche Médicale (INSERM), Institut National de la Santé et de la Recherche Médicale (INSERM)-IFR10-Université Paris-Est Créteil Val-de-Marne - Paris 12 (UPEC UP12)-Institut National de la Santé et de la Recherche Médicale (INSERM)-IFR10-Université Paris-Est Créteil Val-de-Marne - Paris 12 (UPEC UP12)-Centre d’Investigation Clinique 1429 [Garches] (CIC 1429), Hôpital Raymond Poincaré [AP-HP]-Institut National de la Santé et de la Recherche Médicale (INSERM)-Hôpital Raymond Poincaré [AP-HP]-Institut National de la Santé et de la Recherche Médicale (INSERM), Association d'Entraide des Polios et Handicapés (ADEP), Service de pneumologie [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), and Guellaen, Georges
Subjects: Pulmonary and Respiratory Medicine, Adult, Leak, medicine.medical_specialty, Patient-ventilator synchrony, Pulmonary disease, MESH: Equipment Failure Analysis, Mascara, MESH: Ventilators, Mechanical, law.invention, Positive-Pressure Respiration, 03 medical and health sciences, MESH: Positive-Pressure Respiration, 0302 clinical medicine, law, [SDV.BBM] Life Sciences [q-bio]/Biochemistry, Molecular Biology, Medicine, Humans, [SDV.BBM]Life Sciences [q-bio]/Biochemistry, Molecular Biology, 030212 general & internal medicine, Simulation, Ventilators, Mechanical, MESH: Humans, MESH: Middle Aged, business.industry, Masks, MESH: Adult, Equipment Design, Middle Aged, Leaks, Interface, Surgery, Equipment Failure Analysis, On ventilator, Trigger, 030228 respiratory system, MESH: Masks, Ventilation (architecture), Noninvasive ventilation, business, MESH: Equipment Design
Abstract: International audience; Most pressure-support devices use a single circuit with an exhalation port integrated in the mask. The aim of the current study was to compare the effects of masks having different manufacturer-inserted leaks on ventilator performance. We simulated chronic obstructive pulmonary disease and restrictive disease. Four ventilators (VENTImotion (Weinmann, Hamburg, Germany), VPAP III STA (ResMed, Saint Priest, France), Synchrony 2 (Respironics, Nantes, France) and Vivo 40 (Breas, Saint Priest)) were tested with the recommended masks and with the masks having the largest and smallest leaks. Tests were performed with pressure support levels of 10, 15 and 20 cmH(2)O. The in vivo evaluation compared two ventilators using recommended masks opposed in terms of exhaled port resistance. The ventilators were tested with their recommended mask, and after mask exchange. The mask with the largest leak induced auto-triggering and/or increased inspiratory-trigger sensitivity was the VENTImotion under both simulated conditions and VPAP III STA under the simulated obstructive-disease condition. The mask with the smallest leak-increased inspiratory-trigger delay was Synchrony 2 in the simulated obstructive-disease condition and increased rebreathing. The in vivo study confirmed the bench results. When switching to a mask that has a different leak, evaluation is needed to adjust trigger sensitivity and pressurisation level and to check the absence of rebreathing.
Published: 2010

13. Retrospective French nationwide survey of childhood aggressive vascular anomalies of bone, 1988-2009

Author: Jean-Claude Léonard, Pierre Mary, Michèle Bigorre, Jacques de Blic, Ralph Epaud, Makram Ziade, Marion Gillibert-Yvert, Isabelle Pin, Martine Le Merrer, Sébastien Héritier, Benoit de Courtivron, Marie Ouache, Véronique Houdoin, Brigitte Fauroux, Jeanne Wagnon, Pascal Chastagner, Francis Jaubert, Daniel Wendling, Christian Carrie, Liliane Boccon-Gibod, Jean Donadieu, J. Languepin, Véronique Forin, Yann Revillon, Yves Bertrand, Cécile Bost-Bru, Hubert Ducou Lepointe, Service de Génétique Médicale [CHU Necker], Assistance publique - Hôpitaux de Paris (AP-HP) (APHP)-CHU Necker - Enfants Malades [AP-HP], Service d'AnatomoPathologie, Assistance publique - Hôpitaux de Paris (AP-HP) (APHP)-Université Paris Descartes - Paris 5 (UPD5)-CHU Necker - Enfants Malades [AP-HP], Conception et Test de Systèmes MICroélectroniques (SysMIC), Laboratoire d'Informatique de Robotique et de Microélectronique de Montpellier (LIRMM), Université de Montpellier (UM)-Centre National de la Recherche Scientifique (CNRS)-Université de Montpellier (UM)-Centre National de la Recherche Scientifique (CNRS), Centre Hospitalier Régional Universitaire de Nancy (CHRU Nancy), CHU Necker - Enfants Malades [AP-HP], Département de Pédiatrie - CHRU de Grenoble, CHU Grenoble, Agents pathogènes et inflammation - UFC (EA 4266) (API), Université de Franche-Comté (UFC), Physiologie, biologie des organismes, populations, interactions, Université Pierre et Marie Curie - Paris 6 (UPMC)-Institut National de la Santé et de la Recherche Médicale (INSERM), 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 Recherche Saint-Antoine (CR Saint-Antoine), Service d'hématologie-immunologie-oncologie pédiatrique [CHU Trousseau], Centre de référence des Histiocytoses, Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-CHU Necker - Enfants Malades [AP-HP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP), Institut d'hématologie et d'oncologie pédiatrique [CHU - HCL] (IHOPe), Hospices Civils de Lyon (HCL), Centre Hospitalier Universitaire [Grenoble] (CHU), Université Bourgogne Franche-Comté [COMUE] (UBFC)-Université Bourgogne Franche-Comté [COMUE] (UBFC), CHU Trousseau [APHP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU), Centre de Recherche Saint-Antoine (UMRS893), Assistance publique - Hôpitaux de Paris (AP-HP)-CHU Necker - Enfants Malades [AP-HP], Assistance publique - Hôpitaux de Paris (AP-HP)-Université Paris Descartes - Paris 5 ( UPD5 ) -CHU Necker - Enfants Malades [AP-HP], Conception et Test de Systèmes MICroélectroniques ( SysMIC ), Laboratoire d'Informatique de Robotique et de Microélectronique de Montpellier ( LIRMM ), Université de Montpellier ( UM ) -Centre National de la Recherche Scientifique ( CNRS ) -Université de Montpellier ( UM ) -Centre National de la Recherche Scientifique ( CNRS ), Centre Hospitalier Régional Universitaire de Nancy ( CHRU Nancy ), Agents pathogènes et inflammation - UFC (EA 4266) ( API ), Université de Franche-Comté ( UFC ), Université Pierre et Marie Curie - Paris 6 ( UPMC ) -Institut National de la Santé et de la Recherche Médicale ( INSERM ), Service de pneumologie, Université Pierre et Marie Curie - Paris 6 ( UPMC ) -Assistance publique - Hôpitaux de Paris (AP-HP)-CHU Trousseau [APHP], Centre de Recherche Saint-Antoine ( CR Saint-Antoine ), Service d'hématologie-immunologie-oncologie pédiatrique, Université Pierre et Marie Curie - Paris 6 (UPMC)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-CHU Trousseau [APHP], and Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU)-Sorbonne Université (SU)
Subjects: Male, medicine.medical_specialty, Pediatrics, Pleural effusion, Vascular Malformations, medicine.medical_treatment, Alpha interferon, lcsh:Medicine, Kaplan-Meier Estimate, 030218 nuclear medicine & medical imaging, [ SDV.MHEP.RSOA ] Life Sciences [q-bio]/Human health and pathology/Rhumatology and musculoskeletal system, 03 medical and health sciences, 0302 clinical medicine, Epidemiology, medicine, Humans, Genetics(clinical), Pharmacology (medical), Child, Genetics (clinical), Retrospective Studies, Medicine(all), Respiratory distress, Diphosphonates, business.industry, Mortality rate, Research, lcsh:R, Interferon-alpha, General Medicine, Bone fracture, medicine.disease, 3. Good health, Radiation therapy, Thalidomide, [SDV.MHEP.RSOA]Life Sciences [q-bio]/Human health and pathology/Rhumatology and musculoskeletal system, 030220 oncology & carcinogenesis, Female, France, Bone Diseases, business, medicine.drug
Abstract: Objective To document the epidemiological, clinical, histological and radiological characteristics of aggressive vascular abnormalities of bone in children. Study design Correspondents of the French Society of Childhood Malignancies were asked to notify all cases of aggressive vascular abnormalities of bone diagnosed between January 1988 and September 2009. Results 21 cases were identified; 62% of the patients were boys. No familial cases were observed, and the disease appeared to be sporadic. Mean age at diagnosis was 8.0 years [0.8-16.9 years]. Median follow-up was 3 years [0.3-17 years]. The main presenting signs were bone fracture (n = 4) and respiratory distress (n = 7), but more indolent onset was observed in 8 cases. Lung involvement, with lymphangiectasies and pleural effusion, was the most frequent form of extraosseous involvement (10/21). Bisphosphonates, alpha interferon and radiotherapy were used as potentially curative treatments. High-dose radiotherapy appeared to be effective on pleural effusion but caused major late sequelae, whereas antiangiogenic drugs like alpha interferon and zoledrenate have had a limited impact on the course of pulmonary complications. The impact of bisphosphonates and alpha interferon on bone lesions was also difficult to assess, owing to insufficient follow-up in most cases, but it was occasionally positive. Six deaths were observed and the overall 10-year mortality rate was about 30%. The prognosis depended mainly on pulmonary and spinal complications. Conclusion Aggressive vascular abnormalities of bone are extremely rare in childhood but are lifethreatening. The impact of anti-angiogenic drugs on pulmonary complications seems to be limited, but they may improve bone lesions.
Published: 2010

14. Longitudinal survey of Staphylococcus aureus in cystic fibrosis patients using a multiple-locus variable-number of tandem-repeats analysis method

Author: Hoang Vu-Thien, Brigitte Fauroux, Katia Hormigos, Gaëlle Corbineau, Gilles Vergnaud, Christine Pourcel, Harriet Corvol, Didier Moissenet, Service de pneumologie [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), Centre de Recherche Saint-Antoine (UMRS893), Université Pierre et Marie Curie - Paris 6 (UPMC)-Institut National de la Santé et de la Recherche Médicale (INSERM), Institut de génétique et microbiologie [Orsay] (IGM), Université Paris-Sud - Paris 11 (UP11)-Centre National de la Recherche Scientifique (CNRS), CHU Trousseau [APHP], and Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU)
Subjects: Cystic Fibrosis, Minisatellite Repeat, lcsh:QR1-502, Minisatellite Repeats, medicine.disease_cause, Cystic fibrosis, lcsh:Microbiology, MESH: Genotype, MESH: Child, MESH: Staphylococcus aureus, Cluster Analysis, Longitudinal Studies, MESH: Longitudinal Studies, Child, 0303 health sciences, Staphylococcal Infections, MESH: Infant, Bacterial Typing Techniques, 3. Good health, Variable number tandem repeat, [SDV.MP]Life Sciences [q-bio]/Microbiology and Parasitology, MESH: Young Adult, Staphylococcus aureus, Child, Preschool, DNA, Bacterial, Microbiology (medical), MESH: Cystic Fibrosis, Adolescent, Genotype, MESH: Staphylococcal Infections, Multiple Loci VNTR Analysis, Biology, Staphylococcal infections, Microbiology, MESH: Bacterial Typing Techniques, Young Adult, 03 medical and health sciences, Tandem repeat, Research article, medicine, Humans, [SDV.BBM]Life Sciences [q-bio]/Biochemistry, Molecular Biology, Genotyping, 030304 developmental biology, MESH: Adolescent, [SDV.GEN]Life Sciences [q-bio]/Genetics, MESH: Humans, 030306 microbiology, MESH: Child, Preschool, Infant, bacterial infections and mycoses, medicine.disease, MESH: Cluster Analysis, MESH: DNA, Bacterial, MESH: Minisatellite Repeats
Abstract: Background Staphylococcus aureus infection in patients with cystic fibrosis (CF) is frequent and may be due to colonization by a few pathogenic lineages. Systematic genotyping of all isolates, methicillin-susceptible S. aureus (MSSA) as well as methicillin-resistant S. aureus (MRSA) is necessary to identify such lineages and follow their evolution in patients. Multiple-locus variable-number tandem repeat analysis (MLVA/VNTR) was used to survey S. aureus clinical isolates in a French paediatric CF centre. Results During a 30 months period, 108 patients, aged 2 to 21 years, regularly followed up at the centre, provided sputum for culture. From 79 patients, a total of 278 isolates were genotyped by MLVA, resolving into 110 genotypes and 19 clonal complexes (CC) composed of similar or closely related isolates. 71% of the strains were distributed into four main CCs, in term of number of isolates and number of genotypes. Spa (Staphylococcus protein A) typing was performed on representative samples, showing an excellent concordance with MLVA. In 17 patients, strains from two to four different CCs were recovered over time. On six occasions, S. aureus isolates with the same genotype were shared by 2 different patients and they belonged to one of the four main clusters. Methicillin-resistance was observed in 60% of the isolates, 90% of which belonged to the main clonal complexes CC8, CC45 and CC5. In 5 patients, methicillin-resistance of S. aureus isolates was not associated with the mecA gene: for four patients, it was due to overproduction of β-lactamase, leading to BOR-SA (borderline S. aureus) isolates, while a strain showing probably a new modified penicillin-binding capacity (MOD-SA) was observed from one patient. Conclusion Systematic genotyping of S. aureus isolates recovered from sputum of CF children allows a thorough analysis of the strains responsible for sporadic as well as chronic colonization and the follow up of their evolution over time. We show here that more than 70% of these strains belong to 4 major CCs. MSSA as well as MRSA, BOR-SA and MOD-SA isolates can persist over several years, despite antibiotic treatments.
Published: 2010

15. Otologic features in children with primary ciliary dyskinesia.: Otologic features in children with PCD

Author: Prulière-Escabasse, Virginie, Coste, André, Chauvin, Pierre, Fauroux, Brigitte, Tamalet, Aline, Garabedian, Erea-Noel, Escudier, Estelle, Roger, Gilles, Guellaen, Georges, INSERM U955, équipe 13, Service d'ORL et de chirurgie cervico-faciale, Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Hôpital Henri Mondor-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Hôpital Henri Mondor-Institut Mondor de Recherche Biomédicale (IMRB), Institut National de la Santé et de la Recherche Médicale (INSERM)-IFR10-Université Paris-Est Créteil Val-de-Marne - Paris 12 (UPEC UP12)-Institut National de la Santé et de la Recherche Médicale (INSERM)-IFR10-Université Paris-Est Créteil Val-de-Marne - Paris 12 (UPEC UP12), ESIM - Déterminants Sociaux de la Santé et du Recours aux Soins (DS3), Université Pierre et Marie Curie - Paris 6 (UPMC)-Institut National de la Santé et de la Recherche Médicale (INSERM), Service de pneumologie [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), Centre de Recherche Saint-Antoine (UMRS893), Service d'ORL pédiatrique et Chirurgie Cervico-faciale [CHU Trousseau], Service de génétique et embryologie médicales [CHU Trousseau], 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)-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)-Physiopathologie des maladies génétiques d'expression pédiatrique, and Université Pierre et Marie Curie - Paris 6 (UPMC)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Université Pierre et Marie Curie - Paris 6 (UPMC)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Institut Mondor de Recherche Biomédicale (IMRB)
Subjects: MESH: Adolescent, MESH: Bronchiectasis, Kartagener syndrome, MESH: Chi-Square Distribution, MESH: Humans, MESH: Child, Preschool, cilia, serous otitis media, MESH: Retrospective Studies, macromolecular substances, MESH: Infant, MESH: Male, MESH: Otitis Media, MESH: Audiometry, MESH: Anti-Bacterial Agents, MESH: Child, otorhinolaryngologic diseases, [SDV.BBM] Life Sciences [q-bio]/Biochemistry, Molecular Biology, MESH: Kartagener Syndrome, [SDV.BBM]Life Sciences [q-bio]/Biochemistry, Molecular Biology, sense organs, MESH: Hearing Loss, conductive hearing loss, MESH: Female
Abstract: International audience; Despite continuous antibiotic therapy, the middle ear condition in PCD remained severe throughout childhood, with improvement only after age 18 years. Armstrong grommet placement did not improve the middle ear condition. Central complex defect is a marker of severity.
Published: 2010

16. Pressure stability with CPAP devices: A bench evaluation

Author: Frédéric Lofaso, Véronique Grillier-Lanoir, Karl Leroux, Bruno Louis, Brigitte Fauroux, Maud Boucherie, Daniel Isabey, 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), ADEP assistance, Respiratory division, Covidien, Service de pneumologie [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), Centre de Recherche Saint-Antoine (UMRS893), Université Pierre et Marie Curie - Paris 6 (UPMC)-Institut National de la Santé et de la Recherche Médicale (INSERM), Centre d’Investigation Clinique 1429 [Garches] (CIC 1429), Hôpital Raymond Poincaré [AP-HP]-Institut National de la Santé et de la Recherche Médicale (INSERM), Guellaen, Georges, CHU Trousseau [APHP], and Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU)
Subjects: Leak, MESH: Continuous Positive Airway Pressure, obstructive sleep apnoea, medicine.medical_treatment, MESH: Equipment Failure Analysis, Pulmonary compliance, MESH: Pulmonary Ventilation, leak effects, Positive-Pressure Respiration, 03 medical and health sciences, Work of breathing, MESH: Positive-Pressure Respiration, 0302 clinical medicine, Airway resistance, MESH: Computer Simulation, MESH: Airway Resistance, work of breathing, MESH: Humidity, [SDV.BBM] Life Sciences [q-bio]/Biochemistry, Molecular Biology, medicine, Humans, Computer Simulation, [SDV.BBM]Life Sciences [q-bio]/Biochemistry, Molecular Biology, Continuous positive airway pressure, Lung Compliance, bench test, Pressure drop, MESH: Humans, business.industry, Airway Resistance, Humidity, General Medicine, respiratory tract diseases, Equipment Failure Analysis, Compliance (physiology), MESH: Work of Breathing, 030228 respiratory system, Constant pressure, Anesthesia, Pulmonary Ventilation, business, MESH: Lung Compliance, 030217 neurology & neurosurgery, continuous positive airway pressure
Abstract: International audience; BACKGROUND: Continuous positive airway pressure (CPAP) maintains a constant pressure to reduce the patient's work of breathing (WOB). The aim of this study was to measure the additional WOB imposed by four current CPAP devices during simulation of a difficult but commonly encountered clinical situation. METHOD: Flow contour, respiratory system compliance and total lung-airway resistance of a patient under CPAP were simulated. The devices were tested at a CPAP of 15cm H(2)O with a heated humidifier and a nasal pillow, which increased circuitry resistance and with and without a simulated unintentional leak. RESULTS: With no leak, positive end-expiratory pressure (PEEP) at the interface varied across devices from 14.0 to 15.3cm H(2)O. With a leak of 1L/s, PEEP varied from 11.5 to 17.1cm H(2)O. Imposed inspiratory WOB ranged from less than 0.1J/min to 0.45J/min with no leak, and the range broadened with leaking. Findings were similar for the imposed expiratory WOB. CONCLUSION: The performances of CPAP devices are variable. The device that calibrated for the pressure loss in the circuitry under dynamic conditions and made appropriate pressure adjustments outperformed the other devices.
Published: 2010

17. Interstitial lung diseases in children

Author: Harriet Corvol, Brigitte Fauroux, Nadia Nathan, Annick Clement, Ralph Epaud, Centre de Recherche Saint-Antoine (UMRS893), Université Pierre et Marie Curie - Paris 6 (UPMC)-Institut National de la Santé et de la Recherche Médicale (INSERM), Service de pneumologie [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), CHU Trousseau [APHP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU), This work was supported by Inserm, Université Pierre et Marie Curie-Paris6, Paris, Assistance Publique-Hopitaux de Paris, Ministère de la Santé (Centre de Référence des Maladies Respiratoires Rares), and Comité de Soutien de Belleherbe, and BMC, Ed.
Subjects: Male, MESH: Pulmonary Alveoli, medicine.medical_treatment, Anti-Inflammatory Agents, lcsh:Medicine, MESH: Respiratory Function Tests, Review, [SDV.GEN] Life Sciences [q-bio]/Genetics, Endoplasmic Reticulum, Pulmonary function testing, 0302 clinical medicine, Oxygen therapy, Genetics(clinical), Pharmacology (medical), 030212 general & internal medicine, Genetics (clinical), Medicine(all), Mortality rate, Interstitial lung disease, General Medicine, respiratory system, MESH: Infant, Respiratory Function Tests, 3. Good health, medicine.anatomical_structure, Child, Preschool, Female, Pulmonary alveolar proteinosis, Bronchoalveolar Lavage Fluid, Hypersensitivity pneumonitis, medicine.medical_specialty, behavioral disciplines and activities, 03 medical and health sciences, MESH: Endoplasmic Reticulum, medicine, Humans, Intensive care medicine, [SDV.GEN]Life Sciences [q-bio]/Genetics, Lung, MESH: Humans, business.industry, MESH: Bronchoalveolar Lavage Fluid, lcsh:R, MESH: Child, Preschool, Infant, medicine.disease, MESH: Male, respiratory tract diseases, Pulmonary Alveoli, 030228 respiratory system, MESH: Anti-Inflammatory Agents, Immunology, Etiology, Lung Diseases, Interstitial, business, MESH: Female, MESH: Lung Diseases, Interstitial
Abstract: International audience; Interstitial lung disease (ILD) in infants and children comprises a large spectrum of rare respiratory disorders that are mostly chronic and associated with high morbidity and mortality. These disorders are characterized by inflammatory and fibrotic changes that affect alveolar walls. Typical features of ILD include dyspnea, diffuse infiltrates on chest radiographs, and abnormal pulmonary function tests with restrictive ventilatory defect and/or impaired gas exchange. Many pathological situations can impair gas exchange and, therefore, may contribute to progressive lung damage and ILD. Consequently, diagnosis approach needs to be structured with a clinical evaluation requiring a careful history paying attention to exposures and systemic diseases. Several classifications for ILD have been proposed but none is entirely satisfactory especially in children. The present article reviews current concepts of pathophysiological mechanisms, etiology and diagnostic approaches, as well as therapeutic strategies. The following diagnostic grouping is used to discuss the various causes of pediatric ILD: 1) exposure-related ILD; 2) systemic disease-associated ILD; 3) alveolar structure disorder-associated ILD; and 4) ILD specific to infancy. Therapeutic options include mainly anti-inflammatory, immunosuppressive, and/or anti-fibrotic drugs. The outcome is highly variable with a mortality rate around 15%. An overall favorable response to corticosteroid therapy is observed in around 50% of cases, often associated with sequelae such as limited exercise tolerance or the need for long-term oxygen therapy.
Published: 2010

18. New surfactant protein C gene mutations associated with diffuse lung disease

Author: Laurence Jonard, F. Counil, J. de Blic, Annick Clement, Delphine Feldmann, Rola Abou Taam, Philippe Reix, Florence Flamein, A Mohsni, Ralph Epaud, Guillaume Thouvenin, M. Le Bourgeois, Loïc Guillot, Rémy Couderc, Centre de Recherche Saint-Antoine (UMRS893), Université Pierre et Marie Curie - Paris 6 (UPMC)-Institut National de la Santé et de la Recherche Médicale (INSERM), Service de pneumologie [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), Service de Biochimie et de Biologie Moléculaire [CHU Trousseau], 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 Arnaud de Villeneuve [CHRU Montpellier], Centre Hospitalier Régional Universitaire [Montpellier] (CHRU Montpellier), Service de Pneumologie Allergologie [CHU Necker], CHU Necker - Enfants Malades [AP-HP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP), Service de Pédiatrie, Pneumologie, Allergologie et Mucoviscidose, Hôpital Femme Mère Enfant, Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU), Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-CHU Necker - Enfants Malades [AP-HP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP), Centre de Recherche Saint-Antoine ( CR Saint-Antoine ), Université Pierre et Marie Curie - Paris 6 ( UPMC ) -Institut National de la Santé et de la Recherche Médicale ( INSERM ), Service de pneumologie, CHU Trousseau [APHP]-Assistance publique - Hôpitaux de Paris (AP-HP)-Université Pierre et Marie Curie - Paris 6 ( UPMC ), Service de biochimie [CHU Trousseau], Assistance publique - Hôpitaux de Paris (AP-HP)-CHU Trousseau [APHP], Hôpital Arnaud de Villeneuve, Centre Hospitalier Régional Universitaire [Montpellier] ( CHRU Montpellier ), CHU Necker - Enfants Malades [AP-HP]-Assistance publique - Hôpitaux de Paris (AP-HP), and Peer, Hal
Subjects: Lung Diseases, Male, medicine.medical_specialty, DNA Mutational Analysis, Biology, Gene mutation, medicine.disease_cause, Cohort Studies, 03 medical and health sciences, 0302 clinical medicine, Molecular genetics, Genetics, medicine, Humans, Child, Gene, Genetics (clinical), 030304 developmental biology, 0303 health sciences, Mutation, Respiratory disease, Interstitial lung disease, Infant, Surfactant protein C, medicine.disease, Molecular biology, Pulmonary Surfactant-Associated Protein C, 3. Good health, Pedigree, 030228 respiratory system, Child, Preschool, Diffuse parenchymal lung disease, Immunology, Female, Protein C, medicine.drug
Abstract: Background: Mutations in the surfactant protein C gene ( SFTPC ) have been recently associated with the development of diffuse lung disease, particularly sporadic and familial interstitial lung disease (ILD). Objective: We have investigated the prevalence and the spectrum of SFTPC mutations in a large cohort of infants and children with diffuse lung disease and suspected with surfactant dysfunction. Method and results: 121 children were first screened for the common SFTPC mutation, p.Ile73Thr (I73T). Ten unrelated patients were shown to carry this mutation. The I73T mutation was inherited in six cases, and appeared de novo in four. The 111 patients without the I73T mutation were screened for the entire coding sequence of SFTPC . Of these, eight (seven unrelated) subjects were shown to carry a novel mutant allele of SFTPC . All these seven new mutations are located in the BRICHOS domain except the p.Val39Ala (V39A) mutation, which is in the surfactant protein C (SP-C) mature peptide. Conclusions: Our results confirm that SFTPC mutations are a frequent cause of diffuse lung disease, and that I73T is the most frequent SFTPC mutation associated with diffuse lung disease.
Published: 2009

19. ventilators for noninvasive ventilation

Author: Annick Clement, Frédéric Lofaso, Karl Leroux, Bruno Louis, Gilbert Desmarais, Brigitte Fauroux, Daniel Isabey, Service de pneumologie [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), Physiologie, biologie des organismes, populations, interactions, Université Pierre et Marie Curie - Paris 6 (UPMC)-Institut National de la Santé et de la Recherche Médicale (INSERM), ADEP assistance, Institut Mondor de recherche biomédicale (IMRB), Institut National de la Santé et de la Recherche Médicale (INSERM)-Université Paris-Est Créteil Val-de-Marne - Paris 12 (UPEC UP12), Service de physiologie et d'explorations fonctionnelles [CHU Raymond-Poincaré], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Hôpital Raymond Poincaré [AP-HP], The research of BF is supported by the Association Française contre les Myopathies (AFM), Assistance Publique-Hôpitaux de Paris, Inserm, Legs Poix, and Université Pierre et Marie Curie-Paris6., CHU Trousseau [APHP], and Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU)
Subjects: Pulmonary and Respiratory Medicine, Artificial ventilation, Adult, medicine.medical_specialty, Adolescent, Home Nursing, medicine.medical_treatment, Positive pressure, law.invention, Home ventilation, Positive-Pressure Respiration, 03 medical and health sciences, 0302 clinical medicine, law, Medicine, Humans, [SDV.BBM]Life Sciences [q-bio]/Biochemistry, Molecular Biology, 030212 general & internal medicine, Positive pressure ventilation, Intensive care medicine, Tidal volume, Mechanical ventilation, Ventilators, Mechanical, business.industry, Infant, Equipment Design, 030228 respiratory system, Anesthesia, Child, Preschool, Ventilation (architecture), Equipment Failure, business, Airway
Abstract: International audience; The aim of the present study was to evaluate the performance characteristics of all the ventilators proposed for home noninvasive positive-pressure ventilation in children in France. The ventilators (one volume-targeted, 12 pressure-targeted and four dual) were evaluated on a bench which simulated six different paediatric ventilatory patterns. For each ventilator, the quality of the inspiratory and expiratory trigger and the ability to reach and maintain the preset pressures and volumes were evaluated with the six patient profiles. The performance of the ventilators showed great variability, and depended upon the type of trigger (flow or pressure), type of circuit and patient profile. Differences were observed between the preset and measured airway pressure and between the tidal volume measured by the ventilator and on the bench. Leaks were associated with an inability to detect the patient's inspiratory effort or autotriggering. No single ventilator was able to adequately ventilate the six paediatric profiles. Only a few ventilators were able to ventilate the profiles simulating the youngest patients. A systematic paediatric bench evaluation is recommended for every ventilator proposed for home ventilation, in order to detect any dysfunction and guide the choice of the appropriate ventilator for a specific patient.
Published: 2008

20. Performance of ventilators for noninvasive positive-pressure ventilation in children

Author: Fauroux, Brigitte, Leroux, Karl, Desmarais, Gilbert, Isabey, Daniel, Clément, Annick, Lofaso, Frédéric, Louis, Bruno, Guellaen, Georges, Service de pneumologie [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), Physiologie, biologie des organismes, populations, interactions, Université Pierre et Marie Curie - Paris 6 (UPMC)-Institut National de la Santé et de la Recherche Médicale (INSERM), ADEP assistance, Institut Mondor de recherche biomédicale (IMRB), Institut National de la Santé et de la Recherche Médicale (INSERM)-Université Paris-Est Créteil Val-de-Marne - Paris 12 (UPEC UP12), Service de physiologie et d'explorations fonctionnelles [CHU Raymond-Poincaré], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Hôpital Raymond Poincaré [AP-HP], and The research of BF is supported by the Association Française contre les Myopathies (AFM), Assistance Publique-Hôpitaux de Paris, Inserm, Legs Poix, and Université Pierre et Marie Curie-Paris6.
Subjects: [SDV.BBM] Life Sciences [q-bio]/Biochemistry, Molecular Biology, [SDV.BBM]Life Sciences [q-bio]/Biochemistry, Molecular Biology
Abstract: International audience; The aim of the present study was to evaluate the performance characteristics of all the ventilators proposed for home noninvasive positive-pressure ventilation in children in France. The ventilators (one volume-targeted, 12 pressure-targeted and four dual) were evaluated on a bench which simulated six different paediatric ventilatory patterns. For each ventilator, the quality of the inspiratory and expiratory trigger and the ability to reach and maintain the preset pressures and volumes were evaluated with the six patient profiles. The performance of the ventilators showed great variability, and depended upon the type of trigger (flow or pressure), type of circuit and patient profile. Differences were observed between the preset and measured airway pressure and between the tidal volume measured by the ventilator and on the bench. Leaks were associated with an inability to detect the patient's inspiratory effort or autotriggering. No single ventilator was able to adequately ventilate the six paediatric profiles. Only a few ventilators were able to ventilate the profiles simulating the youngest patients. A systematic paediatric bench evaluation is recommended for every ventilator proposed for home ventilation, in order to detect any dysfunction and guide the choice of the appropriate ventilator for a specific patient.
Published: 2008

21. Asthme : dépistage et prévention chez l'enfant

Author: Bousquet, Jean, Demenais, Florence, Grimfeld, Alain, Neukirch, Françoise, Scheinmann, Pierre, Service des Maladies Respiratoires, Hôpital Arnaud de Villeneuve-Institut National de la Santé et de la Recherche Médicale (INSERM), Méthodologie statistique et épidémiologie génétique des maladies multifactorielles, Institut National de la Santé et de la Recherche Médicale (INSERM), Service de Pneumologie pédiatrique [CHU Trousseau], 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), Service de Pneumologie Allergologie [CHU Necker], CHU Necker - Enfants Malades [AP-HP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP), Institut national de la santé et de la recherche médicale(INSERM), ORANGE, Colette, Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-CHU Necker - Enfants Malades [AP-HP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP), Hôpital Arnaud de Villeneuve - Institut National de la Santé et de la Recherche Médicale (INSERM), Service de Pneumologie [CHU Trousseau], Assistance publique - Hôpitaux de Paris (AP-HP) - CHU Trousseau [APHP], and Assistance publique - Hôpitaux de Paris (AP-HP) - CHU Necker - Enfants Malades [AP-HP]
Subjects: Pathologie chronique, Asthme bronchique, Inflammation chronique, Crises, [SDV.SPEE] Life Sciences [q-bio]/Santé publique et épidémiologie, Etat atopique, Allergènes, Hérédité, Système immunitaire, Rhinite allergique, [SDV.SPEE]Life Sciences [q-bio]/Santé publique et épidémiologie, Génétique
Abstract: Les manifestations allergiques, l’asthme en particulier, sont de plus en plusfréquentes dans tous les pays industrialisés. Il faut également remarquer quenon seulement la prévalence mais aussi la sévérité de l’asthme se sont fortementaccrues au cours des dernières années. Plusieurs études effectuées àtravers le monde montrent que, depuis 1960, la fréquence de l’asthme s’accroîtd’environ 6 % à 10 % par an chez l’enfant, quels que soient le pays ou l’ethnieétudiés. Elle est la première maladie chronique de l’enfant dans les paysdéveloppés. Cette augmentation ne semble pas procéder d’une modificationdes moyens de diagnostic ou d’une meilleure connaissance de l’affection.Si la prise en charge de l’asthme a nettement progressé depuis une vingtained’années, du fait d’une meilleure connaissance des mécanismes physiopathologiquesde la maladie et de l’arrivée sur le marché de nouvelles thérapeutiques,il reste cependant essentiel d’appréhender les facteurs à l’origine de saprogression foudroyante.Les facteurs à l’origine des maladies allergiques, et de l’asthme en particulier,sont de deux ordres, génétiques et environnementaux, d’importantes interactionsexistant entre eux. Si un certain nombre de gènes candidats pourl’immunopathologie de l’asthme sont en cours d’identification, des modificationsde ces gènes ne peuvent constituer une explication cohérente pourrendre compte de l’augmentation de la prévalence de l’asthme observée cesvingt dernières années. Les facteurs environnementaux apparaissent commel’explication la plus plausible : la pollution de l’air et les modifications dumode de vie, d’une part, la disparition de facteurs de résistance (infections aucours de la petite enfance), d’autre part, sont en première ligne des responsablespossibles.L’asthme est défini comme une obstruction bronchique réversible, spontanémentou sous l’effet de traitements bronchodilatateurs. Sur le plan physiopathologique,l’asthme est caractérisé par une inflammation de la muqueusebronchique. Cette inflammation est constante, plus ou moins importanteselon le stade de gravité de la maladie.La prise en charge des asthmatiques a largement bénéficié d’une meilleurecompréhension des mécanismes physiopathologiques impliqués dans le développementde la maladie. Cette meilleure connaissance a permis d’élaborerune stratégie thérapeutique cohérente et de proposer des schémas de traitementsappropriés aux différentes formes d’asthme.Cependant, si nous avons largement progressé dans la compréhension del’histoire naturelle et la prise en charge de l’asthme, il n’existe pas encore de traitement curateur de la maladie. C’est dire l’importance des mesures préventives,qui reposent avant tout sur l’éducation des patients, mais aussi desmédecins. La prévention primaire implique un processus dans lequel l’interventionprécède le développement de la maladie. La prévention secondaire apour objectif de prévenir le développement de la maladie chez un sujetprédisposé, par exemple atopique. Pour les patients chez qui le diagnosticd’asthme a été posé, la prévention tertiaire consiste en une modification del’environnement et la prescription d’un traitement pharmacologique adapté.Ce document constitue une bonne synthèse des travaux les plus représentatifsen épidémiologie, physiopathologie et clinique. Il indique les principalesrecommandations en prévention et éducation à la santé, à l’intention desdifférents acteurs impliqués dans la prise en charge de cette pathologie, etpropose quelques pistes de recherche qui mériteraient d’être développées.
Published: 2002

22. New insights into pediatric idiopathic pulmonary hemosiderosis: the French RespiRare® cohort

Author: Marc Lubrano, Pierrick Cros, Malika Mahloul, Antoine Deschildre, Nadia Nathan, M. Fayon, Harriet Corvol, Jacques de Blic, Philippe Reix, Françoise Troussier, Ralph Epaud, Annick Clement, Jessica Taytard, Raphaël Chiron, Delphine Michon, CHU Trousseau [APHP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU), Centre de Recherche Saint-Antoine (UMRS893), Université Pierre et Marie Curie - Paris 6 (UPMC)-Institut National de la Santé et de la Recherche Médicale (INSERM), Service de Pneumologie Allergologie [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), CIC - Bordeaux, Université Bordeaux Segalen - Bordeaux 2-CHU Bordeaux [Bordeaux]-Institut National de la Santé et de la Recherche Médicale (INSERM), Service de pédiatrie, CHI Créteil, Centre Hospitalier Régional Universitaire [Lille] (CHRU Lille), Centre Hospitalier Universitaire d'Angers (CHU Angers), PRES Université Nantes Angers Le Mans (UNAM)-PRES Université Nantes Angers Le Mans (UNAM), 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 Hospitalier Régional Universitaire [Montpellier] (CHRU Montpellier), Service de pneumologie [Centre Hospitalier Lyon Sud - HCL], Centre Hospitalier Lyon Sud [CHU - HCL] (CHLS), Hospices Civils de Lyon (HCL)-Hospices Civils de Lyon (HCL), Centre Hospitalier Régional Universitaire de Brest (CHRU Brest), The French RespiRare® group, BMC, Ed., Service de pneumologie [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), Service de pneumologie, Université Pierre et Marie Curie - Paris 6 ( UPMC ) -Assistance publique - Hôpitaux de Paris (AP-HP)-CHU Trousseau [APHP], Centre de Recherche Saint-Antoine ( CR Saint-Antoine ), Université Pierre et Marie Curie - Paris 6 ( UPMC ) -Institut National de la Santé et de la Recherche Médicale ( INSERM ), Assistance publique - Hôpitaux de Paris (AP-HP)-CHU Necker - Enfants Malades [AP-HP], Université Bordeaux Segalen - Bordeaux 2-CHU Bordeaux [Bordeaux]-Institut National de la Santé et de la Recherche Médicale ( INSERM ), Centre Hospitalier Régional Universitaire [Lille] ( CHRU Lille ), CHU Angers, CHU Rouen-Université de Rouen Normandie ( UNIROUEN ), Normandie Université ( NU ) -Normandie Université ( NU ), Centre Hospitalier Régional Universitaire [Montpellier] ( CHRU Montpellier ), Centre Hospitalier Lyon Sud [CHU - HCL] ( CHLS ), Hospices Civils de Lyon ( HCL ) -Hospices Civils de Lyon ( HCL ), Centre Hospitalier Régional Universitaire de Brest ( CHRU Brest ), CHU Necker - Enfants Malades [AP-HP], and Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)
Subjects: Lung Diseases, Male, Down syndrome, Pediatrics, medicine.medical_specialty, Hemosiderosis, Adolescent, Anemia, Idiopathic pulmonary hemosiderosis, Interstitial lung disease, Pulmonary hemosiderosis, [SDV.GEN] Life Sciences [q-bio]/Genetics, Coeliac disease, 03 medical and health sciences, Autoimmune lung disease, 0302 clinical medicine, 030225 pediatrics, Humans, Medicine, Genetics(clinical), Pharmacology (medical), Child, Genetics (clinical), Medicine(all), [SDV.GEN]Life Sciences [q-bio]/Genetics, Lung, business.industry, Research, Infant, General Medicine, medicine.disease, 3. Good health, medicine.anatomical_structure, 030228 respiratory system, Child, Preschool, Cohort, Immunology, Female, [ SDV.GEN ] Life Sciences [q-bio]/Genetics, business, Immunosuppressive Agents
Abstract: Background Idiopathic pulmonary hemosiderosis (IPH) is a rare cause of alveolar hemorrhage in children and its pathophysiology remains obscure. Classically, diagnosis is based on a triad including hemoptysis, diffuse parenchymal infiltrates on chest X-rays, and iron-deficiency anemia. We present the French pediatric cohort of IPH collected through the French Reference Center for Rare Lung Diseases (RespiRare®, http://www.respirare.fr). Methods Since 2008, a national network/web-linked RespiRare® database has been set up in 12 French pediatric respiratory centres. It is structured as a medical recording tool with extended disease-specific datasets containing clinical information relevant to all forms of rare lung diseases including IPH. Results We identified 25 reported cases of IPH in children from the database (20 females and 5 males). Among them, 5 presented with Down syndrome. Upon diagnosis, median age was 4.3 [0.8-14.0] yrs, and the main manifestations were: dyspnea (n = 17, 68%), anemia (n = 16, 64%), cough (n = 12, 48%), febrile pneumonia (n = 11, 44%) and hemoptysis (n = 11, 44%). Half of the patients demonstrated diffuse parenchymal infiltrates on chest imaging, and diagnosis was ascertained either by broncho-alveolar lavage indicating the presence of hemosiderin-laden macrophages (19/25 cases), or lung biopsy (6/25). In screened patients, initial auto-immune screening revealed positive antineutrophilic cytoplasmic antibodies (ANCA) (n = 6, 40%), antinuclear antibodies (ANA) (n = 5, 45%) and specific coeliac disease antibodies (n = 4, 28%). All the patients were initially treated by corticosteroids. In 13 cases, immunosuppressants were introduced due to corticoresistance and/or major side effects. Median length of follow-up was 5.5 yrs, with a satisfactory respiratory outcome in 23/25 patients. One patient developed severe pulmonary fibrosis, and another with Down syndrome died as a result of severe pulmonary hemorrhage. Conclusion The present cohort provides substantial information on clinical expression and outcomes of pediatric IPH. Analysis of potential contributors supports a role of auto-immunity in disease development and highlights the importance of genetic factors.
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22 results on '"Service de pneumologie [CHU Trousseau]"'

1. Real-Life Safety and Effectiveness of Lumacaftor-Ivacaftor in Patients with Cystic Fibrosis

2. Lessons from a French collaborative case–control study in cystic fibrosis patients during the 2009 A/H1N1 influenza pandemy

3. Analysis of the upper airway by the acoustic reflection method in children with mucopolysaccharidosis

4. Normal and Cystic Fibrosis Human Bronchial Epithelial Cells Infected with Pseudomonas aeruginosa Exhibit Distinct Gene Activation Patterns

5. Idiopathic eosinophilic pneumonia in children: the French experience

6. Evaluation of new minimally invasive methods of exploration of pediatric upper airways

7. Evaluation of new minimally invasive methods of exploration of pediatric upper airways

8. Value of gas exchange recording at home in children receiving noninvasive ventilation: Gas exchange during noninvasive ventilation

9. Value of gas exchange recording at home in children receiving noninvasive ventilation

10. Analysis of the upper airway by the acoustic reflection method in children with mucopolysaccharidosis: Acoustic reflection in mucopolysaccharidosis

11. Otologic features in children with primary ciliary dyskinesia

12. Effect of manufacturer-inserted mask leaks on ventilator performance

13. Retrospective French nationwide survey of childhood aggressive vascular anomalies of bone, 1988-2009

14. Longitudinal survey of Staphylococcus aureus in cystic fibrosis patients using a multiple-locus variable-number of tandem-repeats analysis method

15. Otologic features in children with primary ciliary dyskinesia.: Otologic features in children with PCD

16. Pressure stability with CPAP devices: A bench evaluation

17. Interstitial lung diseases in children

18. New surfactant protein C gene mutations associated with diffuse lung disease

19. ventilators for noninvasive ventilation

20. Performance of ventilators for noninvasive positive-pressure ventilation in children

21. Asthme : dépistage et prévention chez l'enfant

22. New insights into pediatric idiopathic pulmonary hemosiderosis: the French RespiRare® cohort

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