Effects of Inspiratory Muscle Training on Lung Volumes, Respiratory Muscle Strength, and Quality of Life in Patients With Ataxia Telangiectasia

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dc.contributor.author Félix, Erika [UNIFESP]
dc.contributor.author Gimenes, Ana Cristina [UNIFESP]
dc.contributor.author Costa-Carvalho, Beatriz Tavares [UNIFESP]
dc.date.accessioned 2016-01-24T14:35:21Z
dc.date.available 2016-01-24T14:35:21Z
dc.date.issued 2014-03-01
dc.identifier http://dx.doi.org/10.1002/ppul.22828
dc.identifier.citation Pediatric Pulmonology. Hoboken: Wiley-Blackwell, v. 49, n. 3, p. 238-244, 2014.
dc.identifier.issn 8755-6863
dc.identifier.uri http://repositorio.unifesp.br/handle/11600/37474
dc.description.abstract BackgroundAtaxia telangiectasia (AT) is a genetic syndrome caused by a mutation of chromosome 11. the clinical features are cerebellar ataxia, telangiectasia, and progressive loss of muscular coordination, including an inefficient cough secondary to progression of neurological disease.ObjectiveTo evaluate the effects of inspiratory muscle training (IMT) on ventilation, lung volume, dyspnoea, respiratory muscle strength, and quality of life in patients with AT.MethodsA longitudinal study was conducted with 11 AT patients and nine healthy volunteers. Ventilometry, subjective sensation of dyspnoea, maximal inspiratory pressure (MIP), maximal expiratory pressure (MEP), and quality of life were assessed before and after a 24-week IMT program. the IMT load used was set at 60% of the MIP, and the training was performed for 20min daily.ResultsPatients with AT had lower height and weight and also had lower respiratory muscle strength and lung volume compared with healthy volunteers. Furthermore, patients with AT showed a significant improvement when pre- and post-IMT were compared for ventilatory pattern: Vt (476.5135ml vs. 583.3 +/- 66ml, P=0.015) and f (23.3 +/- 6rpm vs. 20.4 +/- 4rpm, P=0.018), and VC (1,664 +/- 463ml/kg vs. 2,145 +/- 750ml/kg, P=0.002). IMT also significantly improved the sensation of dyspnoea (median 0.5; minimum 0; maximum 1.0; P=0.022) and respiratory muscle strength: MIP (-22.2 +/- 2cmH(2)O vs. -38 +/- 9cmH(2)O, P<0.001) and MEP (29 +/- 7cmH(2)O vs. 40 +/- 8cmH(2)O, P=0.001). the health and vitality domains of the SF-36 also showed significant improvement (P=0.009 and P=0.014, respectively) post-IMT.ConclusionIMT was effective in improving ventilatory pattern, lung volume, respiratory muscle strength, and the health and vitality domains for quality of life in patients with AT. IMT may be an effective adjunct therapy to drug treatment for patients with AT. Pediatr Pulmonol. 2014; 49:238-244. (c) 2013 Wiley Periodicals, Inc. en
dc.description.sponsorship Coordenação de Aperfeiçoamento de Pessoal de Nível Superior (CAPES)
dc.format.extent 238-244
dc.language.iso eng
dc.publisher Wiley-Blackwell
dc.relation.ispartof Pediatric Pulmonology
dc.rights Acesso restrito
dc.subject ataxia telangiectasia en
dc.subject immunodeficiency syndromes en
dc.subject physical therapy modalities en
dc.subject inspiratory muscle training en
dc.subject respiratory function tests en
dc.title Effects of Inspiratory Muscle Training on Lung Volumes, Respiratory Muscle Strength, and Quality of Life in Patients With Ataxia Telangiectasia en
dc.type Artigo
dc.rights.license http://olabout.wiley.com/WileyCDA/Section/id-406071.html
dc.contributor.institution Universidade Federal de São Paulo (UNIFESP)
dc.description.affiliation Fed Univ São Paulo UNIFESP, Sch Med, Dept Pediat, São Paulo, Brazil
dc.description.affiliation Fed Univ São Paulo UNIFESP, Dept Med, Div Resp Dis, Pulm Funct & Clin Exercise Physiol Unit, São Paulo, Brazil
dc.description.affiliationUnifesp Fed Univ São Paulo UNIFESP, Sch Med, Dept Pediat, São Paulo, Brazil
dc.description.affiliationUnifesp Fed Univ São Paulo UNIFESP, Dept Med, Div Resp Dis, Pulm Funct & Clin Exercise Physiol Unit, São Paulo, Brazil
dc.identifier.doi 10.1002/ppul.22828
dc.description.source Web of Science
dc.identifier.wos WOS:000331354900031



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