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Pediatr Pulmonol . 2021 May 3. doi: 10.1002/ppul.25430. Online ahead of print.

Nutritional status, swallowing disorders, and respiratory prognosis in adult Duchenne muscular dystrophy patients

Abdallah Fayssoil 1 2 3 4 , Cendrine Chaffaut 5 , Helene Prigent 2 4 6 , Pascal Laforet 2 4 7 , Bernard Clair 1 2 , David Orlikowski 1 2 4 , Adam Ogna 1 , Sylvie Chevret 5 , Paris Meng 1 , Djillali Annane 1 2 4 , Frederic Lofaso 2 4 6 , Pascal Crenn 2 4 8

1 Service de Médecine intensive et Réanimation/Unité de ventilation à domicile, Hôpital Raymond Poincaré, APHP, Université Paris Saclay/UFR Simone Veil-Santé-Université de Versailles Saint Quentin en Yvelines, Garches, France.

2 CRMR des maladies neuromusculaires Nord-Est-Ile de France, Hôpital Raymond Poincaré, APHP, Université Paris Saclay/UFR Simone Veil-Santé-Université de Versailles Saint Quentin en Yvelines, Garches, France.

Bibliographie sur les maladies neuromusculaires Bibliography of neuromuscular disorders

n° 2021-05-1 du 23 avril 2021 au 06 mai 2021 (April 23 to May 6, 2021)

3 Service de cardiologie, Centre de référence des cardiomyopathies et des troubles du rythme cardiaque héréditaires ou rares, Hôpital Ambroise Paré, AP-HP, Université Paris Saclay/UFR Simone Veil-Santé-Université de Versailles Saint Quentin en Yvelines, Boulogne-Billancourt, France.

4 Centre d'Investigation clinique (CIC) 1429 INSERM, Hôpital Raymond Poincaré, APHP, Université Paris Saclay/UFR Simone Veil-Santé-Université de Versailles Saint Quentin en Yvelines, Garches, France.

5 Service de Biostatistiques et Information Médicale (SBIM), Hôpital Saint Louis, APHP, Université Paris, Paris, France.

6 Service de Physiologie-Explorations fonctionnelles, Hôpital Raymond Poincaré, APHP, Université Paris Saclay/UFR Simone Veil-Santé-Université de Versailles Saint Quentin en Yvelines, Garches, France.

7 Service de Neurologie/Hôpital Raymond Poincaré, APHP, Université Paris Saclay/UFR Simone Veil-Santé-Université de Versailles Saint Quentin en Yvelines, Garches, France.

8 Unité de Nutrition clinique transversale, Hôpital Raymond Poincaré, APHP Université Paris Saclay/UFR Simone Veil-Santé-Université de Versailles Saint Quentin en Yvelines, Garches, France.

• PMID: 33939888

• DOI: 10.1002/ppul.25430

Keywords: Duchenne muscular dystrophy; albumin; home mechanical ventilation; malnutrition; prognosis.

Review

Respiration. 2021 May 5;1-10. doi: 10.1159/000515607. Online ahead of print.

Antifibrotic Therapy: Is There a Role in Myositis-Interstitial Lung Disease?

Alyssa Soskis 1 2, Robert Hallowell 1 2

1Division of Pulmonary and Critical Care Medicine, Department of Medicine, Massachusetts General Hospital, Boston, Massachusetts, USA.

2Harvard Medical School, Boston, Massachusetts, USA.

• PMID: 33951665

• DOI: 10.1159/000515607

Keywords: Antifibrotic; Autoimmune disease; Interstitial lung disease; Myositis; Progressive-fibrosing interstitial lung disease.

J Clin Sleep Med . 2021 May 4. doi: 10.5664/jcsm.9400. Online ahead of print.

Determinants of usage and non-adherence to noninvasive ventilation in children and adults with Duchenne muscular dystrophy

Manju S Hurvitz 1 , Rakesh Bhattacharjee 1 , Daniel J Lesser 1 , Andrew J Skalsky 2 , Jeremy E Orr 3

1 Department of Pediatrics, Division of Respiratory Medicine, Rady Children's Hospital San Diego University of California San Diego, San Diego, CA.

2 Department of Orthopedics, Division of Rehabilitation Medicine, Rady Children's Hospital San Diego, University of California San Diego, San Diego, CA.

3 Department of Medicine, Division of Pulmonary and Critical Care Medicine, University of California San Diego, San Diego, CA.

• PMID: 33949945

• DOI: 10.5664/jcsm.9400

Keywords: Duchenne muscular dystrophy; adherence; bilevel positive airway pressure; children; noninvasive ventilation;

usage.

Front Neurol . 2021 Apr 13;12:658532. doi: 10.3389/fneur.2021.658532. eCollection 2021.

Factors Influencing the Severity and Progression of Respiratory Muscle Dysfunction in Myotonic Dystrophy Type 1

Leigh Hartog 1 , Jing Zhao 2 , Jerry Reynolds 3 , Gabrielle Brokamp 1 , Ferdinand Vilson 1 , W David Arnold 3 , Samantha LoRusso 3

1 Department of Neurology, The Ohio State University College of Medicine, Columbus, OH, United States.

2 Department of Biomedical Informatics, The Ohio State University College of Medicine, Columbus, OH, United States.

3 Department of Neurology, Ohio State University Medical Center, Columbus, OH, United States.

• PMID: 33927684

• PMCID: PMC8076608

• DOI: 10.3389/fneur.2021.658532

Keywords: FVC; PFT; impairment; myotonic dystrophy; respiratory.

Bibliographie sur les maladies neuromusculaires Bibliography of neuromuscular disorders

n° 2021-05-1 du 23 avril 2021 au 06 mai 2021 (April 23 to May 6, 2021)

ERJ Open Res. 2021 Apr 19;7(2):00911-2020. doi: 10.1183/23120541.00911-2020. eCollection 2021 Apr.

REINVENT: ERS International survey on REstrictive thoracic diseases IN long term home noninvasive VENTilation

Paola Pierucci 1 2, Claudia Crimi 3, Annalisa Carlucci 4 5, Giovanna E Carpagnano 1 2, Jean-Paul Janssens 6, Manel Lujan 7, Alberto Noto 8 9, Peter J Wijkstra 10 11, Wolfram Windisch 12, Raffaele Scala 13

1Cardiothoracic Dept, Respiratory and Critical Care Unit Bari Policlinic University Hospital, Bari, Italy.

2"Aldo Moro" Bari University School of Medicine, Bari, Italy.

3Respiratory Medicine Unit, "Policlinico-Vittorio Emanuele San Marco" University Hospital, Catania, Italy.

4Reparto Pneumologia Riabilitativa Istituti Clinici Scientifici Maugeri Pavia, Pavia, Italy.

5Dipartimento di Medicina e Chirurgia, Università Insubria Varese-Como, Varese, Italy.

6Division of Pulmonary Diseases, Geneva University Hospitals, Geneva, Switzerland.

7Pneumology Service, Hospital Universitari Parc Taulí de Sabadell, Universitat Autònoma de Barcelona, CIBERES, Sabadell, Spain.

8Dept of Human Pathology of the Adult and Evolutive Age "Gaetano Barresi", Division of Anesthesia and Intensive Care, University of Messina, Policlinico "G. Martino", Messina, Italy.

9IPCF-CNR, Institute for Chemical and Physical Processes, National Research Council, Messina, Italy.

10Dept of Pulmonary Diseases/Home Mechanical Ventilation, University of Groningen, University Medical Center Groningen, Groningen, the Netherlands.

11Groningen Research Institute for Asthma and COPD (GRIAC), University of Groningen, University Medical Center Groningen, Groningen, the Netherlands.

12Cologne Merheim Hospital, Dept of Pneumology, Kliniken der Stadt Köln; gGmbH Witten/Herdecke University, Faculty of Health/School of Medicine, Cologne, Germany.

13Pulmonology and Respiratory Intensive Care Unit, S Donato Hospital, Arezzo, Italy.

• PMID: 33898619

• PMCID: PMC8053911

• DOI: 10.1183/23120541.00911-2020 Case Reports

Turk J Pediatr . 2021;63(2):314-318.doi: 10.24953/turkjped.2021.02.016.

Brown Vialetto Van Laere syndrome: presenting with left ventricular non-compaction and mimicking mitochondrial disorders

Berna Şeker Yılmaz, Serdar Ceylaner 1 , Neslihan Önenli Mungan 2

1 Department of Genetics, Intergen Genetics Centre, Ankara.

2 Department of Pediatric Metabolism, Çukurova University Faculty of Medicine, Adana, Turkey.

• PMID: 33929122

• DOI: 10.24953/turkjped.2021.02.016

Keywords: Brown-Vialetto-Van Laere syndrome; left ventricle-non compaction; mitochondrial disorders; riboflavin.

Case Reports

Clin Case Rep . 2021 Feb 12;9(4):1925-1928. doi: 10.1002/ccr3.3905. eCollection 2021 Apr.

Tailoring NIV by dynamic laryngoscopy in a child with spinal muscular atrophy type I Maria Vollsæter 1 2 3 , Anett Skjoldmo 4 , Ola Røksund 1 5 6 , Magnus Hilland 6 , Tiina Andersen 3 4 5

1 Department of Pediatrics Haukeland University Hospital Bergen Norway.

2 Department of Clinical Science University of Bergen Bergen Norway.

3 Norwegian Advisory Unit for Home Mechanical Ventilation Thoracic Department Haukeland University Hospital Bergen Norway.

4 Department of Physiotherapy Haukeland University Hospital Bergen Norway.

5 Faculty of Health and Social Sciences Western Norway University of Applied Sciences Bergen Norway.

6 Department of Head and Neck surgery ENT Haukeland University Hospital Bergen Norway.

• PMID: 33936616

• PMCID: PMC8077329

• DOI: 10.1002/ccr3.3905

Keywords: ear nose and throat; neurology; pediatrics; respiratory medicine.

Bibliographie sur les maladies neuromusculaires Bibliography of neuromuscular disorders

n° 2021-05-1 du 23 avril 2021 au 06 mai 2021 (April 23 to May 6, 2021) Review

Biomedicines . 2021 Apr 29;9(5):492. doi: 10.3390/biomedicines9050492.

20-Hydroxyecdysone, from Plant Extracts to Clinical Use: Therapeutic Potential for the Treatment of Neuromuscular, Cardio-Metabolic and Respiratory Diseases

Laurence Dinan 1 , Waly Dioh 1 , Stanislas Veillet 1 , Rene Lafont 1 2

1 Biophytis, Sorbonne Université, BC9, 4 place Jussieu, 75005 Paris, France.

2 BIOSIPE, IBPS, Sorbonne Université, UPMC, 75005 Paris, France.

• PMID: 33947076

• DOI: 10.3390/biomedicines9050492

Keywords: COVID-19; Duchenne muscular dystrophy; Mas1; anabolic; cardiometabolic diseases; diabetes; ecdysteroid;

ecdysterone; osteoporosis; respiratory diseases; sarcopenia; β-ecdysone.