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        "resumen" => "<span class="elsevierStyleSectionTitle">Introduction</span><p id="spar0005" class="elsevierStyleSimplePara elsevierViewall">Studies on quality of life in myotonic dystrophy &#40;MD&#41; are scarce and the relationship between respiratory muscle strength and health-related quality of life &#40;HRQoL&#41; has yet to be determined&#46; The present study aims to investigate respiratory muscle strength and HRQoL and their relationship in MD patients&#46;</p> <span class="elsevierStyleSectionTitle">Methods</span><p id="spar0010" class="elsevierStyleSimplePara elsevierViewall">Twenty-three patients &#40;13 men&#44; aged 40<span class="elsevierStyleHsp" style=""></span>&#177;<span class="elsevierStyleHsp" style=""></span>16 years&#41; with MD were evaluated for pulmonary function&#44; maximal inspiratory and expiratory pressure &#40;MIP and MEP&#44; respectively&#41;&#44; sniff nasal inspiratory pressure &#40;SNIP&#41; and HRQoL using the Short Form &#40;SF-36&#41; quality of life questionnaire&#46;</p> <span class="elsevierStyleSectionTitle">Results</span><p id="spar0015" class="elsevierStyleSimplePara elsevierViewall">Respiratory muscle strength values were 71<span class="elsevierStyleHsp" style=""></span>&#177;<span class="elsevierStyleHsp" style=""></span>20<span class="elsevierStyleHsp" style=""></span>cmH<span class="elsevierStyleInf">2</span>O &#40;64&#37; predicted&#41;&#44; 76<span class="elsevierStyleHsp" style=""></span>&#177;<span class="elsevierStyleHsp" style=""></span>32<span class="elsevierStyleHsp" style=""></span>cmH<span class="elsevierStyleInf">2</span>O &#40;70&#37; predicted&#41;&#44; and 79<span class="elsevierStyleHsp" style=""></span>&#177;<span class="elsevierStyleHsp" style=""></span>28<span class="elsevierStyleHsp" style=""></span>cmH<span class="elsevierStyleInf">2</span>O &#40;80&#37; predicted&#41; for MEP&#44; MIP&#44; and SNIP respectively&#46; Significant differences were found in the SF-36 domains of physical functioning 58&#46;7<span class="elsevierStyleHsp" style=""></span>&#177;<span class="elsevierStyleHsp" style=""></span>31&#46;4 <span class="elsevierStyleItalic">vs</span> 84&#46;5<span class="elsevierStyleHsp" style=""></span>&#177;<span class="elsevierStyleHsp" style=""></span>23 &#40;<span class="elsevierStyleItalic">P</span><span class="elsevierStyleHsp" style=""></span>&#60;<span class="elsevierStyleHsp" style=""></span>0&#46;01&#44; 95&#37; CI<span class="elsevierStyleHsp" style=""></span>&#61;<span class="elsevierStyleHsp" style=""></span>1&#46;6&#8211;39&#46;9&#41; and physical problems 43&#46;4<span class="elsevierStyleHsp" style=""></span>&#177;<span class="elsevierStyleHsp" style=""></span>35&#46;2 <span class="elsevierStyleItalic">vs</span> 81&#46;2<span class="elsevierStyleHsp" style=""></span>&#177;<span class="elsevierStyleHsp" style=""></span>34 &#40;<span class="elsevierStyleItalic">P</span><span class="elsevierStyleHsp" style=""></span>&#60;<span class="elsevierStyleHsp" style=""></span>0&#46;001&#44; 95&#37; CI<span class="elsevierStyleHsp" style=""></span>&#61;<span class="elsevierStyleHsp" style=""></span>19&#46;4&#8211;6&#46;1&#41; when compared with the reference values&#46; According to single linear regression analysis&#44; MIP explains 29&#37; of the variance in physical functioning&#44; 18&#37; of physical problems and 20&#37; of vitality&#46;</p> <span class="elsevierStyleSectionTitle">Conclusions</span><p id="spar0020" class="elsevierStyleSimplePara elsevierViewall">Individuals with MD have reduced expiratory muscle strength&#46; HRQoL may be more impaired in some physical domains&#44; which might be influenced by variations in inspiratory muscle strength&#46;</p>"
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Vol. 16. Issue 6.
Pages 892-898 (November - December 2010)
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Vol. 16. Issue 6.
Pages 892-898 (November - December 2010)
Original article
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Respiratory muscle strength and quality of life in myotonic dystrophy patients
Força muscular respiratória e qualidade de vida em pacientes com distrofia miotonica
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T. Lucena Araújoa, V. Regiane Resquetib, S. Brunoc, I. Guerra Azevedod, M.E. Dourado Júniore, G. Fregonezif,
Corresponding author
fregonezi@ufrnet.br

Corresponding autor.
a Physiotherapy, Fellow in PneumoCardioVascular Physical Therapy Laboratory, Department of Physical Therapy, Universidade Federal do Rio Grande do Norte, Natal, Brazil
b Physiotherapy, Fellow in PneumoCardioVascular Physical Therapy Laboratory, Department of Physical Therapy, Universidade Federal do Rio Grande do Norte, Natal, Brazil
c Physiotherapy, Master Degree Physical Therapy Program, PneumoCardioVascular Physical Therapy Laboratory, Department of Physical Therapy, Universidade Federal do Rio Grande do Norte, Natal, Brazil
d Physiotherapy, Master Degree Physical Therapy Program, PneumoCardioVascular Physical Therapy Laboratory, Department of Physical Therapy, Universidade Federal do Rio Grande do Norte, Natal, Brazil
e Physician Neurology, Electroneuromyography Service and Neuromuscular Disease Ambulatory, Onofre Lopes University Hospital, Universidade Federal do Rio Grande do Norte, Natal, Brazil
f Physiotherapy, Master Degree Physical Therapy Program, PneumoCardioVascular Physical Therapy Laboratory, Department of Physical Therapy, Universidade Federal do Rio Grande do Norte, Natal, Brazil
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Abstract
Introduction

Studies on quality of life in myotonic dystrophy (MD) are scarce and the relationship between respiratory muscle strength and health-related quality of life (HRQoL) has yet to be determined. The present study aims to investigate respiratory muscle strength and HRQoL and their relationship in MD patients.

Methods

Twenty-three patients (13 men, aged 40±16 years) with MD were evaluated for pulmonary function, maximal inspiratory and expiratory pressure (MIP and MEP, respectively), sniff nasal inspiratory pressure (SNIP) and HRQoL using the Short Form (SF-36) quality of life questionnaire.

Results

Respiratory muscle strength values were 71±20cmH2O (64% predicted), 76±32cmH2O (70% predicted), and 79±28cmH2O (80% predicted) for MEP, MIP, and SNIP respectively. Significant differences were found in the SF-36 domains of physical functioning 58.7±31.4 vs 84.5±23 (P<0.01, 95% CI=1.6–39.9) and physical problems 43.4±35.2 vs 81.2±34 (P<0.001, 95% CI=19.4–6.1) when compared with the reference values. According to single linear regression analysis, MIP explains 29% of the variance in physical functioning, 18% of physical problems and 20% of vitality.

Conclusions

Individuals with MD have reduced expiratory muscle strength. HRQoL may be more impaired in some physical domains, which might be influenced by variations in inspiratory muscle strength.

Keywords:
Maximal respiratory pressures
Sniff test
Neuromuscular disease
SF-36
Respiratory muscles
Resumo
Introdução

Na distrofia miotônica (DM) estudos sobre qualidade de vida relacionada à saúde (QVRS) ainda são escassos e a sua relação com a força muscular respiratória não foi determinada. Este estudo teve como objetivo a avaliação da força muscular respiratória e da QVRS, além de determinar as relações entre estas variáveis na DM.

Métodos

Foi avaliada a função pulmonar, as pressões respiratórias máximas inspiratórias e expiratórias (PImáx e PEmáx, respectivamente), pressão nasal inspiratória de sniff (SNIP), e a QVRS através do questionário genérico SF-36 em 23 pacientes (13 homens, idade 40±16 anos) com DM.

Resultados

Os valores encontrados da força muscular respiratória foram de 71±20cmH2O (64% preditivo), 76±32cmH2O (70% preditivo), e 79±28cmH2O (80% preditivo) para PEmax, PImax e SNIP respectivamente. Encontramos diferenças significativas nos domínios de SF-36 de função física 58,7±31,4 vs. 84,5±23 (p<0,01, 95% CI=1,6 – 39,9) e problemas físicos 43,4±35,2 vs. 81,2±34 (p<0,001, 95% CI=19,4 – 6,1) comparado com os valores de referência. A analise de regressão linear demonstrou que a PImax explica 29% da variação da função física, 18% dos problemas físicos e 20% da vitalidade.

Conclusão

Indivíduos com DM têm uma redução da força muscular expiratoria. A QVRS pode ser mais prejudicada em alguns domínios da atividade física, o que pode sofrer influência das variações da força muscular inspiratória.

Palavras chave:
Pressões respiratórias máximas
Sniff teste
Doença neuromuscular
SF-36
Músculos respiratórios
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