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dc.contributor.authorHardiman, Orla
dc.date.accessioned2011-03-29T14:44:14Z
dc.date.available2011-03-29T14:44:14Z
dc.date.issued2011-03
dc.identifier.citationManagement of respiratory symptoms in ALS. 2011, 258 (3):359-65 J. Neurol.en
dc.identifier.issn1432-1459
dc.identifier.pmid21082322
dc.identifier.doi10.1007/s00415-010-5830-y
dc.identifier.urihttp://hdl.handle.net/10147/126128
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.isoenen
dc.titleManagement of respiratory symptoms in ALS.en
dc.typeArticleen
dc.contributor.departmentHRB Clinician Scientist, Trinity College and Beaumont Hospital, Dublin, Ireland, orla@hardiman.net.en
dc.identifier.journalJournal of neurologyen
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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