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[Update on the respiratory management of patients with chronic neuromuscular disease].

Authors
  • Priou, P1
  • Trzepizur, W2
  • Meslier, N3
  • Gagnadoux, F2
  • 1 Département de pneumologie, CHU d'Angers, 4, rue Larrey, 49933 Angers, France; Centre de référence des maladies neuromusculaires, CHU d'Angers, 4, rue Larrey, 49933 Angers, France. Electronic address: [email protected] , (France)
  • 2 Département de pneumologie, CHU d'Angers, 4, rue Larrey, 49933 Angers, France. , (France)
  • 3 Département de pneumologie, CHU d'Angers, 4, rue Larrey, 49933 Angers, France; Centre de référence sur la sclérose latérale amyotrophique, CHU d'Angers, 4, rue Larrey, 49933 Angers, France. , (France)
Type
Published Article
Journal
Revue de pneumologie clinique
Publication Date
Dec 01, 2017
Volume
73
Issue
6
Pages
316–322
Identifiers
DOI: 10.1016/j.pneumo.2017.10.001
PMID: 29174288
Source
Medline
Keywords
Language
French
License
Unknown

Abstract

Neuromuscular diseases include a wide range of conditions that may involve potentially life-threatening respiratory complications (infection, respiratory failure). For patients with neuromuscular diseases, clinical assessment of respiratory function and regular pulmonary function tests are needed to screen for nocturnal respiratory disorders, weakness of the diaphragm and potential restrictive disorders and/or chronic hypercapnic respiratory insufficiency, possibly with couch deficiency. MANAGEMENT OF NOCTURNAL RESPIRATORY DISORDERS AND CHRONIC RESPIRATORY FAILURE: Nocturnal respiratory assistance is an important phase of care for nocturnal respiratory disorders and chronic respiratory failure. This may involve continuous positive airway pressure, adaptative servo-ventilation or non-invasive ventilation with a facial or nasal mask. As needed, diurnal assistance may be proposed by mouthpiece ventilation. Should non-invasive ventilation prove insufficient, or if significant swallowing disorders or recurrent bronchial obstruction develop, or in case of prolonged intubation, tracheotomy may be required. In case of lower airway infection with ineffective cough, physical therapy, associated with air stacking, intermittent positive pressure breathing or mechanical in-exsufflation may be proposed. Care for swallowing disorders, nutritional counseling (cachexia, obesity), vaccinations and therapeutic education are integral elements of patient-centered management aiming to prevent the negative impact of infection and to manage respiratory failure of chronic neuromuscular disease. Copyright © 2017 Elsevier Masson SAS. All rights reserved.

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