Original article
Respiratory muscle strength and quality of life in myotonic dystrophy patients
Força muscular respiratória e qualidade de vida em pacientes com distrofia miotonica
T. Lucena Araújoa, V. Regiane Resquetib, S. Brunoc, I. Guerra Azevedod, M.E. Dourado Júniore, G. Fregonezif,
Corresponding author
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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The present study aims to investigate respiratory muscle strength and HRQoL and their relationship in MD patients.</p> <span class="elsevierStyleSectionTitle">Methods</span><p id="spar0010" class="elsevierStyleSimplePara elsevierViewall">Twenty-three patients (13 men, aged 40<span class="elsevierStyleHsp" style=""></span>±<span class="elsevierStyleHsp" style=""></span>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.</p> <span class="elsevierStyleSectionTitle">Results</span><p id="spar0015" class="elsevierStyleSimplePara elsevierViewall">Respiratory muscle strength values were 71<span class="elsevierStyleHsp" style=""></span>±<span class="elsevierStyleHsp" style=""></span>20<span class="elsevierStyleHsp" style=""></span>cmH<span class="elsevierStyleInf">2</span>O (64% predicted), 76<span class="elsevierStyleHsp" style=""></span>±<span class="elsevierStyleHsp" style=""></span>32<span class="elsevierStyleHsp" style=""></span>cmH<span class="elsevierStyleInf">2</span>O (70% predicted), and 79<span class="elsevierStyleHsp" style=""></span>±<span class="elsevierStyleHsp" style=""></span>28<span class="elsevierStyleHsp" style=""></span>cmH<span class="elsevierStyleInf">2</span>O (80% predicted) for MEP, MIP, and SNIP respectively. Significant differences were found in the SF-36 domains of physical functioning 58.7<span class="elsevierStyleHsp" style=""></span>±<span class="elsevierStyleHsp" style=""></span>31.4 <span class="elsevierStyleItalic">vs</span> 84.5<span class="elsevierStyleHsp" style=""></span>±<span class="elsevierStyleHsp" style=""></span>23 (<span class="elsevierStyleItalic">P</span><span class="elsevierStyleHsp" style=""></span><<span class="elsevierStyleHsp" style=""></span>0.01, 95% CI<span class="elsevierStyleHsp" style=""></span>=<span class="elsevierStyleHsp" style=""></span>1.6–39.9) and physical problems 43.4<span class="elsevierStyleHsp" style=""></span>±<span class="elsevierStyleHsp" style=""></span>35.2 <span class="elsevierStyleItalic">vs</span> 81.2<span class="elsevierStyleHsp" style=""></span>±<span class="elsevierStyleHsp" style=""></span>34 (<span class="elsevierStyleItalic">P</span><span class="elsevierStyleHsp" style=""></span><<span class="elsevierStyleHsp" style=""></span>0.001, 95% CI<span class="elsevierStyleHsp" style=""></span>=<span class="elsevierStyleHsp" style=""></span>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.</p> <span class="elsevierStyleSectionTitle">Conclusions</span><p id="spar0020" class="elsevierStyleSimplePara elsevierViewall">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.</p>" ] "pt" => array:2 [ "titulo" => "Resumo" "resumen" => "<span class="elsevierStyleSectionTitle">Introdução</span><p id="spar0025" class="elsevierStyleSimplePara elsevierViewall">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.</p> <span class="elsevierStyleSectionTitle">Métodos</span><p id="spar0030" class="elsevierStyleSimplePara elsevierViewall">Foi avaliada a função pulmonar, as pressões respiratórias máximas inspiratórias e expiratórias (PImáx e PEmáx, respectivamente), pressão nasal inspiratória de <span class="elsevierStyleItalic">sniff</span> (SNIP), e a QVRS através do questionário genérico SF-36 em 23 pacientes (13 homens, idade 40<span class="elsevierStyleHsp" style=""></span>±<span class="elsevierStyleHsp" style=""></span>16 anos) com DM.</p> <span class="elsevierStyleSectionTitle">Resultados</span><p id="spar0035" class="elsevierStyleSimplePara elsevierViewall">Os valores encontrados da força muscular respiratória foram de 71<span class="elsevierStyleHsp" style=""></span>±<span class="elsevierStyleHsp" style=""></span>20<span class="elsevierStyleHsp" style=""></span>cmH<span class="elsevierStyleInf">2</span>O (64% preditivo), 76<span class="elsevierStyleHsp" style=""></span>±<span class="elsevierStyleHsp" style=""></span>32<span class="elsevierStyleHsp" style=""></span>cmH<span class="elsevierStyleInf">2</span>O (70% preditivo), e 79<span class="elsevierStyleHsp" style=""></span>±<span class="elsevierStyleHsp" style=""></span>28<span class="elsevierStyleHsp" style=""></span>cmH<span class="elsevierStyleInf">2</span>O (80% preditivo) para PEmax, PImax e SNIP respectivamente. Encontramos diferenças significativas nos domínios de SF-36 de função física 58,7<span class="elsevierStyleHsp" style=""></span>±<span class="elsevierStyleHsp" style=""></span>31,4 <span class="elsevierStyleItalic">vs</span>. 84,5<span class="elsevierStyleHsp" style=""></span>±<span class="elsevierStyleHsp" style=""></span>23 (<span class="elsevierStyleItalic">p</span><span class="elsevierStyleHsp" style=""></span><<span class="elsevierStyleHsp" style=""></span>0,01, 95% CI<span class="elsevierStyleHsp" style=""></span>=<span class="elsevierStyleHsp" style=""></span>1,6 – 39,9) e problemas físicos 43,4<span class="elsevierStyleHsp" style=""></span>±<span class="elsevierStyleHsp" style=""></span>35,2 <span class="elsevierStyleItalic">vs</span>. 81,2<span class="elsevierStyleHsp" style=""></span>±<span class="elsevierStyleHsp" style=""></span>34 (<span class="elsevierStyleItalic">p</span><span class="elsevierStyleHsp" style=""></span><<span class="elsevierStyleHsp" style=""></span>0,001, 95% CI<span class="elsevierStyleHsp" style=""></span>=<span class="elsevierStyleHsp" style=""></span>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.</p> <span class="elsevierStyleSectionTitle">Conclusão</span><p id="spar0040" class="elsevierStyleSimplePara elsevierViewall">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.</p>" ] ] "bibliografia" => array:2 [ "titulo" => "References" "seccion" => array:1 [ 0 => array:2 [ "identificador" => "bibs0005" "bibliografiaReferencia" => array:31 [ 0 => array:3 [ "identificador" => "bib1" "etiqueta" => "1." "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "Maladie de Steinert" "autores" => array:1 [ 0 => array:2 [ "etal" => false "autores" => array:3 [ 0 => "F. Bouhour" 1 => "M. Bost" 2 => "C. 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