المؤلفون: Gilbert Desmarais, Frédéric Lofaso, Dante Brasil Santos, Sandrine Cognet, Bruno Louis, L. Falaize, Hélène Prigent, Adam Ogna, David Orlikowski

المصدر: Neuromuscular Disorders. 27:518-525

مصطلحات موضوعية: Adult, Male, medicine.medical_specialty, Weakness, Neuromuscular disease, Diaphragm, Diaphragmatic breathing, Stimulation, Sensitivity and Specificity, 03 medical and health sciences, Esophagus, 0302 clinical medicine, Internal medicine, Pressure, medicine, Humans, Respiratory muscle weakness, Genetics (clinical), Mouth, Muscle Weakness, business.industry, Muscle weakness, Neuromuscular Diseases, Middle Aged, musculoskeletal system, medicine.disease, Nasal pressure, Respiratory Muscles, Respiratory Function Tests, Surgery, Phrenic Nerve, Inhalation, 030228 respiratory system, Neurology, Mouth pressure, Pediatrics, Perinatology and Child Health, Cardiology, Female, Neurology (clinical), medicine.symptom, business, 030217 neurology & neurosurgery

الوصف: Twitch mouth pressure using magnetic stimulation of the phrenic nerves and an automated inspiratory trigger is a noninvasive, non-volitional assessment of diaphragmatic strength. Our aims were to validate this method in patients with suspected neuromuscular disease, to determine the best inspiratory-trigger pressure threshold, and to evaluate whether twitch mouth pressure decreased the overdiagnosis of muscle weakness frequently observed with noninvasive volitional tests. Maximal inspiratory pressure, sniff nasal pressure, and twitch mouth pressure were measured in 112 patients with restrictive disease and suspected neuromuscular disorder. Esophageal and transdiaphragmatic pressures were measured in 64 of these patients to confirm or infirm inspiratory muscle weakness. Magnetic stimulation was triggered by inspiratory pressures of −1 and −5 cmH2O. The −5 cmH2O trigger produced the best correlation between twitch mouth pressure and twitch esophageal pressure (R2 = 0.86; P

الوصول الحر: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::e478c38ee86be07bebba5ffba6e3c629Test
https://doi.org/10.1016/j.nmd.2017.01.022Test

المؤلفون: Sandra Pottier, David Orlikowski, Dante Brasil Santos, Ghilas Boussaïd, Stéphane Bahrami, Isabelle Vaugier, Frédéric Lofaso, Hélène Prigent

المصدر: Respiratory Medicine. 115:26-32

مصطلحات موضوعية: Adult, Male, Pulmonary and Respiratory Medicine, medicine.medical_specialty, Adolescent, medicine.medical_treatment, Duchenne muscular dystrophy, Young Adult, 03 medical and health sciences, Tracheostomy, 0302 clinical medicine, Internal medicine, medicine, Humans, Non-invasive ventilation, In patient, Prospective Studies, Child, Prospective cohort study, Heart Failure, Mechanical ventilation, Noninvasive Ventilation, business.industry, Hazard ratio, medicine.disease, Respiration, Artificial, Survival Analysis, Surgery, Muscular Dystrophy, Duchenne, 030228 respiratory system, Child, Preschool, Breathing, France, Respiratory Insufficiency, business, 030217 neurology & neurosurgery, Respiratory care

الوصف: Many patients with DMD undergo tracheostomy. Tracheostomy is associated with certain complications, however its effect on prognosis is not known.The relationship between type of mechanical ventilation and survival at 12 years was evaluated in a prospective cohort of patients with Duchenne muscular dystrophy followed in a French reference center for Neuromuscular Diseases. Cox proportional-hazards regressions were used to estimate the hazard ratios associated with risk of switching from non-invasive to invasive ventilation, and with risk of death.One hundred and fifty patients were included. Initial use of invasive ventilation was associated with an episode of acute respiratory failure (p 0.0001) and with a severe clinical status (p 0.05). Risk of death was associated with swallowing disorders (2.51, IC [1.12-5.66], p 0.03) and cardiac failure (p 0.05) but not with type of mechanical ventilation.Switching to invasive ventilation is appropriate when non-invasive ventilation is ineffective.

الوصول الحر: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::36040a89e19e8fe1b90d18ca594586d1Test
https://doi.org/10.1016/j.rmed.2016.04.009Test

المؤلفون: Constantinos Papadopoulos, David Orlikowski, Hélène Prigent, Arnaud Lacour, Céline Tard, Alain Furby, Julien Praline, Guilhem Solé, Jean-Yves Hogrel, Marie De Antonio, Claudio Semplicini, Joelle Deibener-Kaminsky, Pierre Kaminsky, Bruno Eymard, Nadjib Taouagh, Barbara Perniconi, Dalil Hamroun, Pascal Laforêt, G. Bassez, A.-L. Bedat-Millet, A. Behin, B. Eymard, S. Leonard-Louis, T. Stojkovic, A. Canal, V. Decostre, F. Bouhour, F. Boyer, C. Caillaud, Y. Castaing, F. Chapon, P. Cintas, I. Durieu, A. Echaniz-Laguna, L. Feasson, X. Ferrer, R. Froissart, M. Piraud, D. Germain, K. Benistan, N. Guffon-Fouilhoux, H. Journel, P. Labauge, A. Levy, A. Magot, Y. Péréon, M.-C. Minot-Myhié, A. Nadaj-Pakleza, C. Nathier, N. Pellegrini, P. Petiot, F. Lofaso, A. Dutry, D. Renard, S. Sacconi, C. Desnuelle, E. Salort-Campana, J. Pouget, V. Tiffreau, D. Vincent, F. Zagnoli

المساهمون: Centre Hospitalier Régional Universitaire [Lille] (CHRU Lille), Troubles cognitifs dégénératifs et vasculaires - U 1171 - EA 1046 (TCDV), Institut National de la Santé et de la Recherche Médicale (INSERM)-Université de Lille, Droit et Santé-Centre Hospitalier Régional Universitaire [Lille] (CHRU Lille), Unité de Recherche Pluridisciplinaire Sport, Santé, Société (URePSSS) - ULR 7369 - ULR 4488 (URePSSS), Université d'Artois (UA)-Université du Littoral Côte d'Opale (ULCO)-Université de Lille, Troubles cognitifs dégénératifs et vasculaires - U 1171 (TCDV), Université de Lille-Centre Hospitalier Régional Universitaire [Lille] (CHRU Lille)-Institut National de la Santé et de la Recherche Médicale (INSERM), Université d'Artois (UA)-Université de Lille-Université du Littoral Côte d'Opale (ULCO)

المصدر: Molecular Genetics and Metabolism
Molecular Genetics and Metabolism, 2017, Molecular genetics and metabolism, 122 (1-2), pp.80-85. ⟨10.1016/j.ymgme.2017.06.007⟩
Molecular Genetics and Metabolism, Elsevier, 2017, Molecular genetics and metabolism, 122 (1-2), pp.80-85. ⟨10.1016/j.ymgme.2017.06.007⟩

مصطلحات موضوعية: 0301 basic medicine, Male, Pediatrics, Endocrinology, Diabetes and Metabolism, [SDV]Life Sciences [q-bio], Mesh:Walking, Walking, Mesh:Adult, Respiratory failure, Biochemistry, Late Onset Disorders, Cohort Studies, 0302 clinical medicine, Endocrinology, Mesh:alpha-Glucosidases/therapeutic use, Glycogen Storage Disease Type II, Respiration, Pompe disease, Enzyme replacement therapy, Middle Aged, 3. Good health, Diabetes and Metabolism, Mesh:Late Onset Disorders/drug therapy, Cohort, Breathing, Female, France, Mesh:Respiration, Mesh:alpha-Glucosidases/administration & dosage, Mesh:Enzyme Replacement Therapy*/adverse effects, medicine.drug, Adult, Mesh:Female, medicine.medical_specialty, congenital, hereditary, and neonatal diseases and abnormalities, Mesh:Glycogen Storage Disease Type II/drug therapy, Mesh:Male, Late onset, 03 medical and health sciences, FEV1/FVC ratio, Mesh:alpha-Glucosidases/adverse effects, Mesh:Middle Aged, Mesh:Glycogen Storage Disease Type II/physiopathology, Genetics, medicine, Humans, Mesh:Cohort Studies, Adverse effect, Molecular Biology, Alglucosidase alfa, Mesh:France, Advanced, business.industry, nutritional and metabolic diseases, alpha-Glucosidases, Surgery, Mesh:Humans, 030104 developmental biology, business, 030217 neurology & neurosurgery

الوصف: Background The efficacy of enzyme replacement therapy (ERT) in patients at an advanced stage of Pompe disease has only been addressed in a few studies. Our objective was to assess the long term effects of ERT in a cohort of patients with severe Pompe disease. Methods We identified patients from the French Pompe Registry with severe respiratory failure and permanent wheelchair use (assisted walk for a few meters was allowed) when starting ERT. Patients' medical records were collected and reviewed and respiratory and motor functions, before ERT initiation and upon last evaluation were compared. Results Twelve patients (7 males) were identified. Median age at symptom onset was 24 years [IQR = 15.5; 36.0]. At baseline ventilation was invasive in 11 patients and noninvasive in one, with a median ventilation time of 24 h [IQR = 21.88; 24.00] (min 20; max 24). ERT was initiated at a median age of 52.5 years [IQR = 35.75; 66.50]. Median treatment duration was 55 months [IQR = 39.5; 81.0]. During observational period no adverse reaction to ERT was recorded, five patients (41.67%) died, three decreased their ventilation time by 30, 60 and 90 min and two increased their assisted walking distance, by 80 and 20 m. Conclusion Some patients at a very advanced stage of Pompe disease may show a mild benefit from ERT, in terms of increased time of autonomous ventilation and of enlarged distance in assisted walk. ERT can be initiated in these patients in order to retain their current level of independence and ability to perform daily life activities.

الوصول الحر: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::2eca040ef3e520953509b66aeb672813Test
https://hal.univ-lille.fr/hal-02369159Test

المؤلفون: Djillali Annane, Tarek Sharshar, M. Lejaille, Adam Ogna, David Orlikowski, Frédéric Lofaso, Hélène Prigent, Patricia Samb

المصدر: Brain and Behavior

مصطلحات موضوعية: Adult, Male, medicine.medical_specialty, dysphagia, medicine.medical_treatment, Guillain-Barre Syndrome, intubation, 03 medical and health sciences, Behavioral Neuroscience, 0302 clinical medicine, Swallowing, Tongue, medicine, Respiratory muscle, otorhinolaryngologic diseases, Intubation, Humans, Respiratory function, Original Research, Aged, Guillain-Barre syndrome, business.industry, digestive, oral, and skin physiology, vital capacity, Pneumonia, Ventilator-Associated, tongue strength, Middle Aged, medicine.disease, Prognosis, Dysphagia, Surgery, Pneumonia, Intensive Care Units, medicine.anatomical_structure, 030228 respiratory system, Anesthesia, ICU, Female, Guillain‐Barré syndrome, medicine.symptom, business, Deglutition Disorders, Respiratory Insufficiency, 030217 neurology & neurosurgery

الوصف: Background Bulbar weakness and respiratory impairment have been associated with increased morbidity in retrospective studies of Guillain-Barre syndrome (GBS) patients. The aim of this study was to prospectively explore the relationship between subclinical swallowing impairment, respiratory function parameters, the necessity to intubate patients and the development of early postintubation pneumonia in patients with GBS in the intensive care unit (ICU). Methods Respiratory, swallowing, and tongue strength parameters were measured in 30 consecutive adults (51.7 ± 18.1 years old), hospitalized for GBS in the ICU of a teaching hospital. Twenty healthy volunteers were recruited as a control group. The primary outcomes were intubation and pneumonia during the ICU stay. Results Nineteen patients (65.5%) had piecemeal swallowing, and 19 (65.5%) had impaired breathing-swallowing interaction, of which, respectively, 47.4% and 52.6% had a clinically apparent swallowing impairment. Swallowing impairment was associated with lower values of respiratory function, but not with peripheral motor weakness. Tongue protrusion strength was correlated with respiratory parameters and swallowing impairment. Ten patients were intubated and six developed pneumonia. Age, BMI, severe axial involvement, respiratory parameters (vital capacity and respiratory muscle strength), tongue protrusion strength, and clinical swallowing impairment were predictors of intubation. Conclusions Swallowing impairment was present early after ICU admission in over 80% of patients and was an important predictor of intubation. A systematic clinical evaluation of swallowing should be carried out, eventually combined with an evaluation of tongue protrusion strength, along with the usual assessment of neurological and respiratory function, to determine the severity of the GBS.

الوصول الحر: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::8b40f683dfd29497914719066641065cTest
http://europepmc.org/articles/PMC5318364Test

المؤلفون: Frédéric Lofaso, Nicholas Heming, Cendrine Chaffaut, David Orlikowski, Maria-Antonia Quera Salva, Adam Ogna, Sylvie Chevret, Djillali Annane, Hélène Prigent

المصدر: Neuromuscular disorders : NMD. 27(4)

مصطلحات موضوعية: Adult, Male, medicine.medical_specialty, Vital capacity, Neuromuscular disease, medicine.medical_treatment, Nocturnal, Hypercapnia, 03 medical and health sciences, 0302 clinical medicine, Internal medicine, medicine, Humans, Genetics (clinical), Mechanical ventilation, business.industry, Hazard ratio, Hypoventilation, Neuromuscular Diseases, Middle Aged, medicine.disease, Prognosis, Respiration, Artificial, Surgery, 030228 respiratory system, Neurology, Pediatrics, Perinatology and Child Health, Cohort, Cardiology, Female, Neurology (clinical), medicine.symptom, business, 030217 neurology & neurosurgery, Follow-Up Studies

الوصف: In neuromuscular disease (NMD) patients, current guidelines recommend the initiation of home mechanical ventilation (HMV) in case of daytime hypercapnia or nocturnal desaturation as an indirect sign of hypoventilation. Transcutaneous capno-oximetry (TcCO 2 ) enables the direct assessment of nocturnal hypercapnia; however the best cut-off value remains to be defined. We aimed to compare the prognostic value of several published definitions of nocturnal hypercapnia in a cohort of NMD patients. All consecutive TcCO 2 recordings performed between 2010 and 2014 in unventilated adult NMD patients in a tertiary reference centre were retrospectively collected. Initiation of HMV and mortality were collected as outcomes of interest. 124 patients with normal daytime blood gazes were analysed (median age 39 [IQR 31–55] years; vital capacity 61% [43–82] of predicted). The prevalence of nocturnal hypercapnia ranged from 3% to 44%, depending on the definition. Over a median follow-up duration of 2.5 years [IQR 1.6–4.1], HMV was initiated for 51 patients, whilst 4 patients died. Nocturnal peak TcCO 2 ≥49 mmHg was the best predictor of HMV initiation in the follow-up, being associated with a hazard ratio of 2.6 [95% CI 1.4–4.6] in a multivariate analysis adjusting for lung function parameters. Nocturnal TcCO 2 identifies NMD patients at risk for subsequent need for HMV in the following few years, who were not identified by daytime blood gases or nocturnal oximetry. As a consequence, peak nocturnal TcCO 2 ≥49 mmHg should be considered as one of the criteria to start HMV in patients with NMDs, along with symptoms of hypoventilation, daytime hypercapnia, abnormal nocturnal oximetry results, and a diminished level of forced vital capacity.

الوصول الحر: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::c1b7f29db3b91b2e5bcb9b3870b6ca72Test
https://pubmed.ncbi.nlm.nih.gov/28153460Test

المؤلفون: Thomas Similowski, Valérie Attali, Jesus Gonzalez Bermejo, Vincent Meininger, Capucine Morélot-Panzini, Nathalie Guy, Tanguy Marie Laure, Christophe Perrin, Claude Desnuelle, François Salachas, Hélène Prigent, Nierat Marie-Cecile, Philippe Couratier

المصدر: 1.2 Rehabilitation and Chronic Care.

مصطلحات موضوعية: medicine.medical_specialty, Vital capacity, Randomization, business.industry, medicine.disease, Surgery, law.invention, Diaphragm pacing, Respiratory failure, Randomized controlled trial, law, Medicine, Respiratory system, Amyotrophic lateral sclerosis, business, Phrenic nerve

الوصف: Background. Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disorder associated with respiratory muscle weakness and respiratory failure. Non invasive ventilation (NIV) alleviates respiratory symptoms and prolongs life but delaying NIV in ALS is therefore desirable. We aimed to establish whether early diaphragm pacing could achieve this objective. Methods. Multicenter randomized controlled trial involving 12 French multidisciplinary ALS centers. Eligible patients had moderate ALS-related respiratory involvement (forced vital capacity 60-80 % predicted). All patients were operated laparoscopically and received an intradiaphragmatic phrenic nerve stimulator (NeuRxRA4®, Synapse Biomedical, USA). They were then randomly assigned to active or sham stimulation. The primary outcome was NIV free survival. Investigators and patients were blinded to treatment. NIV was decided by an external allocation panel (triple blind design). Results. we randomly assigned 74 participants to active (n=37) or sham (n=37) stimulation. The median NIV free survival since randomization was 6.0 months in the active stimulation group, vs. 8.8 months in the sham stimulation group (p = 0.02). Sixty-five percent of the patients in the active group experienced severe adverse events, vs. 59%. Median overall survival from randomization was 15.6 months (95%CI 9-27) in the active group, vs. more than 33 months (p = 0.007). Conclusions. Early diaphragm pacing in ALS patients with incipient respiratory involvement did not delay NIV and was associated with decreased survival.

الوصول الحر: https://explore.openaire.eu/search/publication?articleId=doi_________::5da6c5903439c6d8789bf20eaf1899d2Test
https://doi.org/10.1183/13993003.congress-2016.oa270Test

المؤلفون: Christophe Perrin, Marie-Cécile Niérat, Thomas Similowski, Jésus Gonzalez-Bermejo, Philippe Couratier, Catherine Fargeot, Claude Desnuelle, Capucine Morélot-Panzini, Marie-Laure Tanguy, Timothée Lenglet, Gaëlle Bruneteau, Catherine Royer, Valérie Attali, Vincent Meininger, François Salachas, Nathalie Guy, Nadine Le Forestier, Pierre-François Pradat, Hélène Prigent, Fabrice Menegaux

المصدر: The Lancet. Neurology. 15(12)

مصطلحات موضوعية: Male, medicine.medical_specialty, Population, Diaphragm, Electric Stimulation Therapy, law.invention, 03 medical and health sciences, 0302 clinical medicine, Randomized controlled trial, Double-Blind Method, law, medicine, Humans, Respiratory system, education, Phrenic nerve, Aged, education.field_of_study, business.industry, Amyotrophic Lateral Sclerosis, Middle Aged, Respiration Disorders, Respiration, Artificial, Hypoventilation, Diaphragm (structural system), Surgery, Phrenic Nerve, Diaphragm pacing, 030228 respiratory system, Respiratory failure, Anesthesia, Early Termination of Clinical Trials, Female, Laparoscopy, Neurology (clinical), medicine.symptom, business, Respiratory Insufficiency, 030217 neurology & neurosurgery

الوصف: Summary Background Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disorder associated with respiratory muscle weakness and respiratory failure. Non-invasive ventilation alleviates respiratory symptoms and prolongs life, but is a palliative intervention. Slowing the deterioration of diaphragm function before respiratory failure would be desirable. We aimed to assess whether early diaphragm pacing could slow down diaphragm deterioration and would therefore delay the need for non-invasive ventilation. Methods We did a multicentre, randomised, controlled, triple-blind trial in patients with probable or definite ALS in 12 ALS centres in France. The main inclusion criterion was moderate respiratory involvement (forced vital capacity 60–80% predicted). Other key eligibility criteria were age older than 18 years and bilateral responses of the diaphragm to diagnostic phrenic stimulation. All patients were operated laparoscopically and received phrenic stimulators. Clinicians randomly assigned patients (1:1) to receive either active or sham stimulation with a central web-based randomisation system (computer-generated list). Investigators, patients, and an external outcome allocation committee were masked to treatment. The primary outcome was non-invasive ventilation-free survival, analysed in the intention-to-treat population. Safety outcomes were also assessed in the intention-to-treat population. This trial is registered with ClinicalTrials.gov, number NCT01583088. Findings Between Sept 27, 2012, and July 8, 2015, 74 participants were randomly assigned to receive either active (n=37) or sham (n=37) stimulation. On July 16, 2015, an unplanned masked analysis was done after another trial showed excess mortality with diaphragm pacing in patients with hypoventilation (DiPALS, ISRCTN 53817913). In view of this finding, we analysed mortality in our study and found excess mortality (death from any cause) in our active stimulation group. We therefore terminated the study on July, 16, 2015. Median non-invasive ventilation-free survival was 6·0 months (95% CI 3·6–8·7) in the active stimulation group versus 8·8 months (4·2–not reached) in the control (sham stimulation) group (hazard ratio 1·96 [95% CI 1·08–3·56], p=0·02). Serious adverse events (mainly capnothorax or pneumothorax, acute respiratory failure, venous thromboembolism, and gastrostomy) were frequent (24 [65%] patients in the active stimulation group vs 22 [59%] patients in the control group). No treatment-related death was reported. Interpretation Early diaphragm pacing in patients with ALS and incipient respiratory involvement did not delay non-invasive ventilation and was associated with decreased survival. Diaphragm pacing is not indicated at the early stage of the ALS-related respiratory involvement. Funding Hospital Program for Clinical Research, French Ministry of Health; French Patients' Association for ALS Research (Association pour la Recherche sur la Sclerose Laterale Amyotrophique); and Thierry de Latran Foundation.

الوصول الحر: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::19a260bb434838c0442a1dfe0bf80b1bTest
https://pubmed.ncbi.nlm.nih.gov/27751535Test

المؤلفون: Bernard Clair, Sylvie Chevret, Djillali Annane, Raquel Guimarães-Costa, Abdallah Fayssoil, Tanya Stojkovic, Cendrine Chaffaut, Olivier Nardi, David Orlikowski, Adam Ogna, Pascal Laforêt, Karim Wahbi, Anthony Behin, Hélène Prigent, Frédéric Lofaso

المساهمون: HAL UPMC, Gestionnaire, Institut de Myologie, Université Pierre et Marie Curie - Paris 6 (UPMC)-Commissariat à l'énergie atomique et aux énergies alternatives (CEA)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Association française contre les myopathies (AFM-Téléthon)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Centre National de la Recherche Scientifique (CNRS), Service de réanimation médicale[Poincaré], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Hôpital Raymond Poincaré [AP-HP], Centre d’Investigation Clinique 1429 [Garches] (CIC 1429), Hôpital Raymond Poincaré [AP-HP]-Institut National de la Santé et de la Recherche Médicale (INSERM), Service de biostatistique et information médicale de l’hôpital Saint Louis (Equipe ECSTRA) (SBIM), Université Paris Diderot - Paris 7 (UPD7)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Institut national du cancer [Boulogne] (INCA)-Hopital Saint-Louis [AP-HP] (AP-HP), Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP), Service de biochimie et de génétique moléculaire [CHU Cochin], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Hôpital Cochin [AP-HP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP), Service de Cardiologie [CHU Cochin], Service de physiologie et d'explorations fonctionnelles [CHU Raymond-Poincaré], Centre National de la Recherche Scientifique (CNRS)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Association française contre les myopathies (AFM-Téléthon)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Commissariat à l'énergie atomique et aux énergies alternatives (CEA)-Université Pierre et Marie Curie - Paris 6 (UPMC), Hopital Saint-Louis [AP-HP] (AP-HP), Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Institut national du cancer [Boulogne] (INCA)-Université Paris Diderot - Paris 7 (UPD7)-Institut National de la Santé et de la Recherche Médicale (INSERM), Hôpital Cochin [AP-HP]

المصدر: PLoS ONE
PLoS ONE, 2016, 11 (4), pp.e0153095. ⟨10.1371/journal.pone.0153095⟩
PLoS ONE, Vol 11, Iss 4, p e0153095 (2016)
PLoS ONE, Public Library of Science, 2016, 11 (4), pp.e0153095. ⟨10.1371/journal.pone.0153095⟩

مصطلحات موضوعية: Male, Heredity, Pulmonology, Genetic Linkage, Physiology, Vital Capacity, Cardiomyopathy, lcsh:Medicine, Duchenne Muscular Dystrophy, Cardiovascular Physiology, Muscular Dystrophies, 0302 clinical medicine, Medicine and Health Sciences, Medicine, Respiratory function, Respiratory system, lcsh:Science, Multidisciplinary, Ejection fraction, [SDV.MHEP] Life Sciences [q-bio]/Human health and pathology, Heart, Prognosis, Respiratory Muscles, 3. Good health, Neurology, X-Linked Traits, Sex Linkage, Cardiology, Female, Cardiomyopathies, Respiratory Insufficiency, Research Article, Adult, medicine.medical_specialty, Death Rates, Diastole, 03 medical and health sciences, Population Metrics, Respiratory Failure, Diagnostic Medicine, Internal medicine, Respiratory muscle, Sarcoglycanopathies, Genetics, Humans, Demography, Clinical Genetics, Population Biology, business.industry, lcsh:R, Biology and Life Sciences, Arrhythmias, Cardiac, medicine.disease, Surgery, 030228 respiratory system, Respiratory failure, Muscular Dystrophies, Limb-Girdle, Heart failure, People and Places, lcsh:Q, business, Physiological Processes, Sleep, 030217 neurology & neurosurgery, [SDV.MHEP]Life Sciences [q-bio]/Human health and pathology, Ejection Fraction

الوصف: BACKGROUND:Type 2C and 2D limb girdle muscular dystrophies (LGMD) are a group of autosomal recessive limb girdle muscular dystrophies manifested by proximal myopathy, impaired respiratory muscle function and cardiomyopathy. The correlation and the prognostic impact of respiratory and heart impairment are poorly described. We aimed to describe the long-term cardiac and respiratory follow-up of these patients and to determine predictive factors of cardio-respiratory events and mortality in LGMD 2C and 2D. METHODS:We reviewed the charts of 34 LGMD patients, followed from 2005 to 2015, to obtain echocardiographic, respiratory function and sleep recording data. We considered respiratory events (acute respiratory failure, pulmonary sepsis, atelectasis or pneumothorax), cardiac events (acute heart failure, significant cardiac arrhythmia or conduction block, ischemic stroke) and mortality as outcomes of interest for the present analysis. RESULTS:A total of 21 patients had type 2C LGMD and 13 patients had type 2D. Median age was 30 years [IQR 24-38]. At baseline, median pulmonary vital capacity (VC) was 31% of predicted value [20-40]. Median maximal inspiratory pressure (MIP) was 31 cmH2O [IQR 20.25-39.75]. Median maximal expiratory pressure (MEP) was 30 cm H2O [20-36]. Median left ventricular ejection fraction (LVEF) was 55% [45-64] with 38% of patients with LVEF

وصف الملف: application/pdf

الوصول الحر: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::be31212f679c614f55c1df3526b92aebTest
https://hal.sorbonne-universite.fr/hal-01323875Test

المؤلفون: Valérie Gounant, Jean-Jacques Boffa, Adoracion Esteso, Jean-Pierre Fulgencio, Valeria Loi, Charlotte Verdet, Eric Bouvard, Annabelle Stoclin, Antoine Parrot, Christophe Ridel, Muriel Fartoukh, Jonathan Messika, Ioan-Paul Muresan, Hélène Prigent, Claude Bachmeyer, Michel Denis

المصدر: Respiratory care. 61(2)

مصطلحات موضوعية: 0301 basic medicine, Pulmonary and Respiratory Medicine, Male, Methicillin-Resistant Staphylococcus aureus, medicine.medical_specialty, medicine.drug_class, 030106 microbiology, Antibiotics, Respiratory System, Critical Care and Intensive Care Medicine, medicine.disease_cause, Hospital-acquired pneumonia, 03 medical and health sciences, 0302 clinical medicine, Community-acquired pneumonia, Enterobacteriaceae, Internal medicine, Drug Resistance, Bacterial, medicine, Pneumonia, Bacterial, Humans, In patient, 030212 general & internal medicine, Prospective Studies, Diagnostic Errors, Aged, Aged, 80 and over, Cross Infection, Gram-Negative Aerobic Bacteria, business.industry, General Medicine, University hospital, medicine.disease, Surgery, Anti-Bacterial Agents, Bacterial Typing Techniques, Pneumonia, Staphylococcus aureus, Etiology, Female, France, business

الوصف: BACKGROUND: Early recognition and an attempt at obtaining microbiological documentation are recommended in patients with non-community-acquired pneumonia (NCAP), whether hospital-acquired (HAP) or health care-associated (HCAP). We aimed to characterize the clinical features and microbial etiologies of NCAP to assess the impact of microbiological investigation on their management. METHODS: This was a prospective 1-y study in a university hospital with 141 non-mechanically ventilated subjects suspected of having HAP ( n = 110) or HCAP ( n = 31). RESULTS: Clinical criteria alone poorly identified pneumonia (misdiagnosis in 50% of cases). Microbiological confirmation was achievable in 80 subjects (57%). Among 79 microorganisms isolated, 28 were multidrug-resistant aerobic Gram-negative bacilli and group III Enterobacteriaceae and 6 were methicillin-resistant Staphylococcus aureus . Multidrug-resistant aerobic Gram-negative bacilli accounted for one third of the microorganisms in early-onset HAP and for 50% in late-onset HAP. Methicillin-resistant S. aureus was most often recovered from subjects with HCAP. Inappropriate empirical antibiotics were administered to 36% of subjects with confirmed pneumonia. Forty subjects were admitted to the ICU, 13 (33%) of whom died. Overall, 39 subjects (28%) died in the hospital. CONCLUSIONS: Integrating the microbiological investigation in the complex clinical diagnostic workup of patients suspected of having NCAP is mandatory. Respiratory tract specimens should be obtained whenever possible for appropriate management.

الوصول الحر: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::a9b07b8f75e639dd9d0f20f495e57876Test
https://pubmed.ncbi.nlm.nih.gov/26647452Test

المؤلفون: Hélène Prigent, David Orlikowski, Djillali Annane, Frédéric Lofaso, Line Falaize, Nicolas Terzi, Michèle Lejaille

المصدر: Neuromuscular Disorders. 20:493-498

مصطلحات موضوعية: Adult, Male, medicine.medical_specialty, medicine.medical_treatment, Duchenne muscular dystrophy, Electromyography, Intermittent Positive-Pressure Ventilation, Young Adult, 03 medical and health sciences, Tracheostomy, 0302 clinical medicine, Bolus (medicine), stomatognathic system, Swallowing, otorhinolaryngologic diseases, medicine, Humans, Plethysmograph, Prospective Studies, Muscular dystrophy, Genetics (clinical), Mechanical ventilation, medicine.diagnostic_test, business.industry, digestive, oral, and skin physiology, medicine.disease, Deglutition, Surgery, Muscular Dystrophy, Duchenne, Plethysmography, 030228 respiratory system, Neurology, Anesthesia, Pediatrics, Perinatology and Child Health, Respiratory Mechanics, Female, Neurology (clinical), Deglutition Disorders, business, Airway, 030217 neurology & neurosurgery

الوصف: Mechanical ventilation has improved survival in patients with Duchenne muscular dystrophy (DMD). Over time, these patients experience upper airway dysfunction, swallowing impairments, and dependency on the ventilator that may require invasive mechanical ventilation via a tracheostomy. Tracheostomy is traditionally believed to further impair swallowing. We assessed swallowing performance and breathing-swallowing interactions before and after tracheostomy in 7 consecutive wheelchair-bound DMD patients, aged 25+/-4 years, over a 4-year period. Chin electromyography, laryngeal motion, and inductive respiratory plethysmography recordings were obtained during swallowing of three water-bolus sizes in random order. Piecemeal deglutition occurred in all patients over several breathing cycles. Half the swallows were followed by inspiration before tracheostomy. Total bolus swallowing time was significantly shorter (P=0.009), and the number of swallows per bolus significantly smaller (P=0.01), after than before tracheostomy. Invasive ventilation via a tracheostomy may improve swallowing.

الوصول الحر: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::c4c71f7d2c17e0deeae10380e891acccTest
https://doi.org/10.1016/j.nmd.2010.05.009Test