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dc.contributor.authorHardiman, Orlaen_GB
dc.date.accessioned2012-02-01T10:02:08Z
dc.date.available2012-02-01T10:02:08Z
dc.date.issued2012-02-01T10:02:08Z
dc.identifier.citationJ Neurol. 2011 Mar;258(3):359-65. Epub 2010 Nov 17.en_GB
dc.identifier.issn1432-1459 (Electronic)en_GB
dc.identifier.issn0340-5354 (Linking)en_GB
dc.identifier.pmid21082322en_GB
dc.identifier.doi10.1007/s00415-010-5830-yen_GB
dc.identifier.urihttp://hdl.handle.net/10147/207214
dc.description.abstractRespiratory insufficiency is a frequent feature of ALS and is present in almost all cases at some stage of the illness. It is the commonest cause of death in ALS. FVC is used as important endpoint in many clinical trials, and in decision-making events for patients with ALS, although there are limitations to its predictive utility. There are multiple causes of respiratory muscle failure, all of which act to produce a progressive decline in pulmonary function. Diaphragmatic fatigue and weakness, coupled with respiratory muscle weakness, lead to reduced lung compliance and atelectasis. Increased secretions increase the risk of aspiration pneumonia, which further compromises respiratory function. Bulbar dysfunction can lead to nutritional deficiency, which in turn increases the fatigue of respiratory muscles. Early recognition of respiratory decline and symptomatic intervention, including non-invasive ventilation can significantly enhance both quality of life and life expectancy in ALS. Patients with respiratory failure should be advised to consider an advance directive to avoid emergency mechanical ventilation.
dc.language.isoengen_GB
dc.subject.meshAmyotrophic Lateral Sclerosis/*complications/physiopathology/*therapyen_GB
dc.subject.meshAnimalsen_GB
dc.subject.meshDisease Managementen_GB
dc.subject.meshHumansen_GB
dc.subject.meshPalliative Care/methodsen_GB
dc.subject.meshRespiration, Artificial/methodsen_GB
dc.subject.meshRespiratory Insufficiency/etiology/*physiopathology/*therapyen_GB
dc.subject.meshRespiratory Mechanics/physiologyen_GB
dc.subject.meshRespiratory Muscles/physiopathologyen_GB
dc.titleManagement of respiratory symptoms in ALS.en_GB
dc.contributor.departmentHRB Clinician Scientist, Trinity College and Beaumont Hospital, Dublin, Ireland. , orla@hardiman.neten_GB
dc.identifier.journalJournal of neurologyen_GB
dc.description.provinceLeinster
html.description.abstractRespiratory insufficiency is a frequent feature of ALS and is present in almost all cases at some stage of the illness. It is the commonest cause of death in ALS. FVC is used as important endpoint in many clinical trials, and in decision-making events for patients with ALS, although there are limitations to its predictive utility. There are multiple causes of respiratory muscle failure, all of which act to produce a progressive decline in pulmonary function. Diaphragmatic fatigue and weakness, coupled with respiratory muscle weakness, lead to reduced lung compliance and atelectasis. Increased secretions increase the risk of aspiration pneumonia, which further compromises respiratory function. Bulbar dysfunction can lead to nutritional deficiency, which in turn increases the fatigue of respiratory muscles. Early recognition of respiratory decline and symptomatic intervention, including non-invasive ventilation can significantly enhance both quality of life and life expectancy in ALS. Patients with respiratory failure should be advised to consider an advance directive to avoid emergency mechanical ventilation.


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