المؤلفون: Pascal Crenn, Tanya Stojkovic, Dominique Mompoint, Hélène Prigent, David Orlikowski, Helge Amthor, Cendrine Chaffaut, Laure Lamothe, Susana Quijano Roy, Guillaume Bassez, Sylvie Chevret, Djillali Annane, Bernard Clair, Pascal Laforêt, Frédéric Lofaso, Robert Carlier, Paris Meng, Adam Ogna, Anthony Behin, Abdallah Fayssoil, Bruno Eymard

المصدر: Journal of Neuromuscular Diseases. 6:55-64

مصطلحات موضوعية: Adult, Male, 0301 basic medicine, Vital capacity, medicine.medical_specialty, Adolescent, Duchenne muscular dystrophy, Diaphragm, Diaphragmatic breathing, Muscle disorder, Pulmonary function testing, Young Adult, 03 medical and health sciences, 0302 clinical medicine, Internal medicine, Respiratory muscle, Humans, Medicine, Child, Retrospective Studies, Ultrasonography, business.industry, Respiration, Organ Size, Middle Aged, musculoskeletal system, medicine.disease, Respiration, Artificial, Diaphragm (structural system), Muscular Dystrophy, Duchenne, Cross-Sectional Studies, 030104 developmental biology, Neurology, Cardiology, Median body, Neurology (clinical), business, 030217 neurology & neurosurgery

الوصف: BACKGROUND Duchenne muscular dystrophy (DMD) is an X-linked recessive genetic muscle disorder. Respiratory muscle function is classically affected in this disease. Ultrasound recently emerged as a non-invasive tool to assess diaphragm function. However, there are only a few studies using diaphragm ultrasound (US) in DMD. PURPOSE We aimed to assess diaphragm ultrasound patterns in DMD, their relationship with age and their association with home mechanical ventilation (HMV). METHODS We included DMD patients followed at Raymond Poincare Hospital who benefited from diaphragm ultrasound and pulmonary function tests. RESULTS There were 110 DMD patients and 17 male sex-matched healthy subjects included. In all, 94% of patients were permanent wheelchair users. Median body mass index (BMI) was 18 kg/m2. DMD patients disclosed a reduced forced vital capacity (VC) (12% of predicted value), and 78% of patients were on HMV. In patients, right and left diaphragmatic motions on deep inspiration were reduced and end expiratory diaphragm thickness was borderline normal. In patients, right and left diaphragmatic thickening fractions (TF) were reduced 12.7% and 15.5%, respectively. Age and end expiratory thickness were significantly inversely associated (p = 0.005 for the right diaphragm, p = 0.018 for the left diaphragm). Diaphragm TF was significantly inversely associated with age (p = 0.001 for the right side, p

الوصول الحر: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::54576aaa09a075dc125c0779852f4c1bTest
https://doi.org/10.3233/jnd-180326Test

المؤلفون: Tanya Stojkovic, Helge Amthor, David Orlikowski, Hélène Prigent, Djillali Annane, Frédéric Lofaso, Stéphane Vinit, Bernard Clair, Anthony Behin, Arnaud Mansart, Dominique Mompoint, Abdallah Fayssoil, Bruno Eymard, Robert Carlier, Pascal Laforêt, Adam Ogna

المصدر: Journal of Neuromuscular Diseases

مصطلحات موضوعية: medicine.medical_specialty, Diaphragm, Respiratory pattern, Review, neuromuscular disorders, 03 medical and health sciences, 0302 clinical medicine, Respiratory muscle, Medicine, Humans, Respiratory system, Ultrasonography, business.industry, ultrasound, Ultrasound, Neuromuscular Diseases, musculoskeletal system, Pathophysiology, Diaphragm (structural system), 030228 respiratory system, Neurology, Ultrasound imaging, Neurology (clinical), Radiology, business, 030217 neurology & neurosurgery

الوصف: Respiratory muscles are classically involved in neuromuscular disorders, leading to a restrictive respiratory pattern. The diaphragm is the main respiratory muscle involved during inspiration. Ultrasound imaging is a noninvasive, radiation-free, accurate and safe technique allowing assessment of diaphragm anatomy and function. The authors review the pathophysiology of diaphragm in neuromuscular disorders, the methodology and indications of diaphragm ultrasound imaging as well as possible pitfalls in the interpretation of results.

الوصول الحر: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::13c8b6d88488a2d0a3d8a4aaf8e04296Test
http://europepmc.org/articles/PMC5836400Test

المؤلفون: Hélène Prigent, Christian Devaux, Frédéric Lofaso, David Orlikowski, Gilbert Caranhac, Ghilas Boussaïd, Djillali Annane

المصدر: Journal of Neuromuscular Diseases. 4:165-168

مصطلحات موضوعية: Adult, Male, Emergency Medical Services, medicine.medical_specialty, Referral, medicine.medical_treatment, Muscular Dystrophies, 03 medical and health sciences, 0302 clinical medicine, Oxygen therapy, Health care, Emergency medical services, Humans, Medicine, Hospital Mortality, Muscular dystrophy, Hospitals, Teaching, Retrospective Studies, Cause of death, business.industry, Retrospective cohort study, medicine.disease, Hospitalization, Treatment Outcome, 030228 respiratory system, Neurology, Respiratory failure, Emergency medicine, Female, France, Neurology (clinical), Respiratory Insufficiency, business, 030217 neurology & neurosurgery, Preliminary Data

الوصف: BACKGROUND In France, referral centres in teaching hospitals were created 10 years ago to provide MD patients with treatments and follow-up designed to prevent complications and improve outcomes. Respiratory failure is a major cause of death among subjects with MD, and its prevention and treatment can serve as a touchstone for assessing the effectiveness of MD care pathways. OBJECTIVE We report data from a preliminary study of admissions of MD patients in France and of factors associated with mortality, with special emphasis on respiratory failure [RF]. METHODS Retrospective analysis of the data from the French nationwide hospital database [Programme de M e dicalisation des syst e mes d'information, PMSI] for 2009 and 2010. RESULTS 7187 admissions of patients with MD were included in the study. Most admissions were to teaching hospitals [5913/7187, 82.3%]. 302 [4.2%] patients were admitted for RF requiring invasive ventilation, 924 [12.9%] for RF requiring only NIV or high-flow oxygen therapy, and 5961 [82.9%] required no respiratory assistance. 494 [6.9%] admissions occurred on an emergency basis. 77/7187 [1.1%] patients died while hospitalised. Teaching-hospital admission was associated with lower frequencies of emergency admission [3.08% vs. 24.5%, p

الوصول الحر: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::6c0a880250c23a998397d1df5a693f0bTest
https://doi.org/10.3233/jnd-170212Test