Case Reports
Domiciliary Respiratory Muscle Training in Myotonic Dystrophy
Guilherme Augusto de Freitas Fregonezi1,
, Vanessa Regiane Resqueti, Pere Casan
Corresponding author
gfregonezi@msn.com
Correspondence: Dr. G.A. de Freitas Fregonezi Departamento de Neumología. Hospital de la santa Creu i saint Pau Antoni Maria Claret, 167. 08025 Barcelona. España
Correspondence: Dr. G.A. de Freitas Fregonezi Departamento de Neumología. Hospital de la santa Creu i saint Pau Antoni Maria Claret, 167. 08025 Barcelona. España
Departamento de Neumología, Hospital de la Santa Creu i Sant Pau, Facultad de Medicina, Universitat Autònoma de Barcelona, Barcelona, Spain
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Hospital de la santa Creu i saint Pau Antoni Maria Claret, 167. 08025 Barcelona. España" ] ] ] ] "pdfFichero" => "main.pdf" "tienePdf" => true "fechaRecibido" => "2006-01-24" "fechaAceptado" => "2006-03-16" "PalabrasClave" => array:2 [ "en" => array:1 [ 0 => array:4 [ "clase" => "keyword" "titulo" => "Key words" "identificador" => "xpalclavsec154217" "palabras" => array:3 [ 0 => "Myotonic dystrophy" 1 => "Respiratory muscle traning" 2 => "Maximal respiratory pressures" ] ] ] "es" => array:1 [ 0 => array:4 [ "clase" => "keyword" "titulo" => "Palabras claves" "identificador" => "xpalclavsec154218" "palabras" => array:3 [ 0 => "Distrofia muscular miotónica" 1 => "Entrenamiento muscular respiratorio" 2 => "Presiones respiratorias máximas" ] ] ] ] "tieneResumen" => true "resumen" => array:2 [ "en" => array:1 [ "resumen" => "<p class="elsevierStyleSimplePara elsevierViewall">A 42-year-old man diagnosed with myotonic dystrophy experienced loss of respiratory muscle strength over a period of 6 months. We report the application of a domiciliary training program targeting both inspiratory and expiratory muscles. Maximal inspiratory and expiratory pressures, forced vital capacity, and forced midexpiratory flow rate were measured 6 months before start of training, just before commencement of the program, and immediately after 12 weeks of training. Adherence to the program was satisfactory. inspiratory muscle training was efficacious in increasing respiratory muscle strength. Expiratory muscle training, which made use of the Threshold PEP bronchial hygiene device incorporating an adapted flutter valve, was not efficacious in increasing maximal expiratory pressure or halting its loss. However, decreased obstruction of medium-caliber airways was observed with use of the device.</p>" ] "es" => array:1 [ "resumen" => "<p class="elsevierStyleSimplePara elsevierViewall">Se describe el caso de un varón de 42 años de edad con diagnóstico de distrofia muscular miotónica e historia de disminución de la fuerza muscular respiratoria en los últimos 6 meses. Relatamos la aplicación de un programa de entrenamiento muscular respiratorio (inspiratorio e espira torio) domiciliario. En los 6 meses previos al entrenamiento, justo antes del comienzo del programa e inmediatamente después de 12 semanas de su inicio se estudiaron la presión inspiratoria máxima, la presión espiratoria máxima, la Capacidad vital forzada y el flujo mesoespiratorio forzado.</p><p class="elsevierStyleSimplePara elsevierViewall">El cumplimento del programa fue satisfactorio. El entrenamiento muscular inspiratorio se mostró efectivo para aumentar la fuerza muscular respiratoria. El entrenamiento muscular espiratorio utilizando la válvula adaptada de higiene bronquial Threshold PEP®, adaptada para el entrenamiento, no fue eficiente para aumentar la presión espiratoria máxima y/o frenar su disminución. Sin embargo, con la utilización de la válvula Threshold PEP® se observó la disminución de la obstrucción de las vías aéreas de medio calibre.</p>" ] ] "NotaPie" => array:1 [ 0 => array:3 [ "etiqueta" => "1" "nota" => "<p class="elsevierStyleNotepara">GA de Freitas Fregonezi is a doctoral fellow of CNPq-Brasil (process 2000-05/01-4).</p>" "identificador" => "fn1" ] ] "bibliografia" => array:2 [ "titulo" => "REFERENCES" "seccion" => array:1 [ 0 => array:1 [ "bibliografiaReferencia" => array:15 [ 0 => array:3 [ "identificador" => "bib1" "etiqueta" => "1" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "Profiles of neuromuscular diseases. 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| 2024 August | 55 | 41 | 96 |
| 2024 July | 24 | 20 | 44 |
| 2024 June | 31 | 32 | 63 |
| 2024 May | 55 | 39 | 94 |
| 2024 April | 28 | 23 | 51 |
| 2024 March | 26 | 11 | 37 |
| 2024 February | 20 | 20 | 40 |
| 2023 March | 2 | 1 | 3 |
| 2023 February | 15 | 18 | 33 |
| 2023 January | 18 | 34 | 52 |
| 2022 December | 18 | 34 | 52 |
| 2022 November | 16 | 20 | 36 |
| 2022 October | 22 | 35 | 57 |
| 2022 September | 12 | 20 | 32 |
| 2022 August | 18 | 26 | 44 |
| 2022 July | 19 | 40 | 59 |
| 2022 June | 13 | 26 | 39 |
| 2022 May | 20 | 28 | 48 |
| 2022 April | 18 | 27 | 45 |
| 2022 March | 22 | 31 | 53 |
| 2022 February | 18 | 30 | 48 |
| 2022 January | 12 | 31 | 43 |
| 2021 December | 13 | 47 | 60 |
| 2021 November | 17 | 48 | 65 |
| 2021 October | 18 | 42 | 60 |
| 2021 September | 20 | 58 | 78 |
| 2021 August | 7 | 21 | 28 |
| 2021 July | 11 | 25 | 36 |
| 2021 June | 12 | 27 | 39 |
| 2021 May | 13 | 27 | 40 |
| 2021 April | 23 | 50 | 73 |
| 2021 March | 15 | 12 | 27 |
| 2021 February | 8 | 16 | 24 |
| 2021 January | 5 | 10 | 15 |
| 2020 December | 14 | 11 | 25 |
| 2020 November | 10 | 11 | 21 |
| 2020 October | 8 | 11 | 19 |
| 2020 September | 7 | 7 | 14 |
| 2020 August | 12 | 11 | 23 |
| 2020 July | 9 | 13 | 22 |
| 2020 June | 8 | 1 | 9 |
| 2020 May | 14 | 13 | 27 |
| 2020 April | 11 | 11 | 22 |
| 2020 March | 11 | 16 | 27 |
| 2020 February | 15 | 13 | 28 |
| 2020 January | 15 | 12 | 27 |
| 2019 December | 16 | 9 | 25 |
| 2019 November | 6 | 7 | 13 |
| 2019 October | 2 | 8 | 10 |
| 2019 September | 9 | 5 | 14 |
| 2019 August | 7 | 9 | 16 |
| 2019 July | 7 | 12 | 19 |
| 2019 June | 6 | 5 | 11 |
| 2019 May | 12 | 5 | 17 |
| 2019 April | 31 | 20 | 51 |
| 2019 March | 16 | 13 | 29 |
| 2019 February | 9 | 14 | 23 |
| 2019 January | 6 | 10 | 16 |
| 2018 December | 10 | 20 | 30 |
| 2018 November | 19 | 6 | 25 |
| 2018 October | 23 | 16 | 39 |
| 2018 September | 9 | 10 | 19 |
| 2018 May | 8 | 0 | 8 |
| 2018 April | 14 | 5 | 19 |
| 2018 March | 4 | 2 | 6 |
| 2018 February | 5 | 8 | 13 |
| 2018 January | 6 | 3 | 9 |
| 2017 December | 5 | 6 | 11 |
| 2017 November | 3 | 6 | 9 |
| 2017 October | 3 | 6 | 9 |
| 2017 September | 5 | 9 | 14 |
| 2017 August | 6 | 9 | 15 |
| 2017 July | 2 | 6 | 8 |
| 2017 June | 11 | 13 | 24 |
| 2017 May | 4 | 10 | 14 |
| 2017 April | 2 | 4 | 6 |
| 2017 March | 2 | 15 | 17 |
| 2017 February | 0 | 2 | 2 |
| 2017 January | 1 | 3 | 4 |
| 2016 December | 15 | 12 | 27 |
| 2016 November | 11 | 10 | 21 |
| 2016 October | 13 | 11 | 24 |
| 2016 September | 19 | 14 | 33 |
| 2016 August | 15 | 4 | 19 |
| 2016 July | 12 | 9 | 21 |
| 2016 March | 2 | 0 | 2 |
| 2016 February | 1 | 0 | 1 |
| 2015 December | 2 | 0 | 2 |
| 2015 October | 51 | 5 | 56 |
| 2015 September | 44 | 6 | 50 |
| 2015 August | 26 | 8 | 34 |
| 2015 July | 47 | 5 | 52 |
| 2015 June | 40 | 6 | 46 |
| 2015 May | 43 | 2 | 45 |
| 2015 April | 51 | 10 | 61 |
| 2015 March | 36 | 3 | 39 |
| 2015 February | 40 | 3 | 43 |
| 2015 January | 34 | 11 | 45 |
| 2014 December | 26 | 3 | 29 |
| 2014 November | 62 | 10 | 72 |
| 2014 October | 51 | 7 | 58 |
| 2014 September | 45 | 10 | 55 |
| 2014 August | 38 | 7 | 45 |
| 2014 July | 39 | 5 | 44 |
| 2014 June | 53 | 8 | 61 |
| 2014 May | 43 | 13 | 56 |
| 2014 April | 58 | 4 | 62 |
| 2014 March | 56 | 10 | 66 |
| 2014 February | 58 | 9 | 67 |
| 2014 January | 52 | 12 | 64 |
| 2013 December | 49 | 16 | 65 |
| 2013 November | 40 | 7 | 47 |
| 2013 October | 41 | 9 | 50 |
| 2013 September | 29 | 10 | 39 |
| 2013 August | 37 | 7 | 44 |
| 2013 July | 39 | 10 | 49 |
| 2013 June | 42 | 7 | 49 |
| 2013 May | 39 | 5 | 44 |
| 2013 April | 27 | 4 | 31 |
| 2013 March | 8 | 4 | 12 |
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