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        "titulo" => "Abstract"
        "resumen" => "<span class="elsevierStyleSectionTitle">Introduction</span><p id="spar0005" class="elsevierStyleSimplePara elsevierViewall">Neuromuscular disease &#40;NMD&#41; patients frequently have impaired cough&#46; Mechanical insufflation-exsufflation &#40;MI-E&#41; has proven efficacy in improving airway clearance&#44; however data related to its long-term home use is lacking&#46; The purpose of this study was to describe indications&#44; safety and compliance of home MI-E in NMD patients&#46;</p> <span class="elsevierStyleSectionTitle">Methods</span><p id="spar0010" class="elsevierStyleSimplePara elsevierViewall">Four years observational analysis of 21 NMD patients on home MI-E&#46; Diagnosis included bulbar and non-bulbar Amyotrophic Lateral Sclerosis &#40;ALS&#41; and other NMD&#46; Median age was 58 years&#46; Only cooperative patients with unassisted baseline Peak Cough Flow &#40;PCF&#41; &#60; 270 L&#47;min were included&#46; All patients were under continuous mechanical ventilation &#40;6 by tracheostomy&#41;&#46; Pulmonary function before initiation of MI-E &#40;median&#41;&#58; FVC &#61; 0&#46;81 L&#44; MIP &#61; 28 cmH<span class="elsevierStyleInf">2</span>O&#44; MEP &#61; 22 cmH<span class="elsevierStyleInf">2</span>O and PCF &#61; 60 L&#47;min&#46; MI-E was performed by previously trained non-professional caregivers&#44; with an on-call support of a trained health care professional&#46; Patients had pulse oximetry monitorization and applied MI-E whenever SpO<span class="elsevierStyleInf">2</span> &#60; 95 &#37;&#46; Median follow-up was 12 months &#40;3-41 months&#41;&#46;</p> <span class="elsevierStyleSectionTitle">Results</span><p id="spar0015" class="elsevierStyleSimplePara elsevierViewall">Ten patients &#40;9 ALS&#41; used MI-E daily&#46; Eleven patients used MI-E intermittently&#44; during exacerbations&#44; and in 8 patients early application of MI-E &#40;guided by oximetry feed-back&#41; avoided hospitalization&#46; All tracheostomized patients used MI-E daily and more times a day than patients under NIV&#46; Four patients &#40;3 bulbar ALS&#41;&#44; were hospitalized due to secretion encumbrance&#46; MI-E was well-tolerated and there were no complications&#46; In general&#44; caregivers considered MI-E effective&#46; During this period&#44; 4 patients died&#44; related to disease progression&#46;</p> <span class="elsevierStyleSectionTitle">Conclusions</span><p id="spar0020" class="elsevierStyleSimplePara elsevierViewall">Home MI-E is well tolerated&#44; effective and safe if used by well trained caregivers&#46; MI-E should be considered as a complement to mechanical ventilation&#46;</p>"
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        "resumen" => "<span class="elsevierStyleSectionTitle">Introducci&#243;n</span><p id="spar0025" class="elsevierStyleSimplePara elsevierViewall">Con frecuencia&#44; los pacientes con enfermedades neuromusculares &#40;ENM&#41; presentan un deterioro del mecanismo de la tos&#46; Se ha demostrado la eficacia de la insuflaci&#243;n-exuflaci&#243;n mec&#225;nica &#40;IEM&#41; en la mejora del aclaramiento de las v&#237;as respiratorias aunque no se dispone de datos relacionados con su utilizaci&#243;n domiciliaria a largo plazo&#46; El objetivo del presente estudio fue describir las indicaciones&#44; tolerabilidad y cumplimiento con la IEM domiciliaria en pacientes con ENM&#46;</p> <span class="elsevierStyleSectionTitle">M&#233;todos</span><p id="spar0030" class="elsevierStyleSimplePara elsevierViewall">An&#225;lisis observacional de 4 a&#241;os de duraci&#243;n de 21 pacientes ENM tratados con IEM domiciliaria&#46; El diagn&#243;stico incluy&#243; esclerosis lateral amiotr&#243;fica &#40;ELA&#41; bulbar y no bulbar y otras ENM&#46; La edad mediana fue de 58 a&#241;os&#46; S&#243;lo se incluyeron pacientes cooperadores con valores de flujo pico de tos basal &#40;FTM&#41; no asistido &#60; 270 l&#47;min&#46; Todos los pacientes estaban sometidos a ventilaci&#243;n mec&#225;nica continua &#40;seis mediante traqueostom&#237;a&#41;&#46; La funci&#243;n pulmonar previa al inicio de la IEM &#40;mediana&#41; era&#58; FVC&#44; 0&#44;81 l&#44; MIP&#44; 28 cmH<span class="elsevierStyleInf">2</span>O&#44; MEP &#61; 22 cmH<span class="elsevierStyleInf">2</span>O y PCF &#61; 60 l&#47;min&#46; Cuidadores no profesionales adiestrados previamente con el apoyo permanente de un profesional sanitario experto efectuaron la IEM&#46; En los pacientes se monitoriz&#243; la pulsioximetr&#237;a y se aplic&#243; IEM siempre que la SpO<span class="elsevierStyleInf">2</span> fue &#60; 95 &#37;&#46; El seguimiento mediano fue de 12 meses &#40;3-41 meses&#41;&#46;</p> <span class="elsevierStyleSectionTitle">Resultados</span><p id="spar0035" class="elsevierStyleSimplePara elsevierViewall">Utilizaron diariamente IEM 10 pacientes &#40;nueve con ELA&#41;&#46; La utilizaron de forma intermitente 11 pacientes durante las exacerbaciones y en 8 la aplicaci&#243;n precoz de IEM &#40;guiada por la informaci&#243;n de la oximetr&#237;a&#41; evit&#243; la hospitalizaci&#243;n&#46; Todos los pacientes traqueostomizados utilizaron la IEM a diario y un mayor n&#250;mero de veces al d&#237;a que los pacientes sometidos a ventilaci&#243;n mec&#225;nica no invasiva &#40;VNI&#41;&#46; Requirieron ingreso hospitalario 4 pacientes &#40;3 ELA bulbar&#41; debido a la acumulaci&#243;n de secreciones&#46; La IEM fue bien tolerada y no se asoci&#243; a complicaciones&#46; En general&#44; los cuidadores la consideraron eficaz&#46; Durante este per&#237;odo&#44; 4 pacientes fallecieron&#44; en relaci&#243;n con la progresi&#243;n de la enfermedad&#46;</p> <span class="elsevierStyleSectionTitle">Conclusiones</span><p id="spar0040" class="elsevierStyleSimplePara elsevierViewall">La IEM domiciliaria es bien tolerada&#44; eficaz y segura cuando la utilizan cuidadores adiestrados apropiadamente&#46; Debe considerarse un complemento de la ventilaci&#243;n mec&#225;nica&#46;</p>"
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Original Article
Indications and Compliance of Home Mechanical Insufflation-Exsufflation in Patients with Neuromuscular Diseases
Indicaciones y cumplimiento con la insuflación-exuflación mecánica domiciliaria en pacientes con enfermedades neuromusculares
João Bentoa,
Corresponding author
joao.a.p.bento@gmail.com

Corresponding author.
, Miguel Gonçalvesa,b, Nuno Silvac, Tiago Pintoa, Anabela Marinhoa, João Carlos Wincka,b
a Pulmonology Department, HS João, Oporto, Portugal
b Faculty of Medicine, University of Porto, Oporto, Portugal
c Linde homecare, Oporto, Portugal
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          "titulo" => "Palabras clave"
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          "palabras" => array:5 [
            0 => "Insuflaci&#243;n-exuflaci&#243;n mec&#225;nica"
            1 => "Domicilio"
            2 => "Seguridad"
            3 => "Eficacia"
            4 => "Enfermedad neuromuscular"
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      "en" => array:2 [
        "titulo" => "Abstract"
        "resumen" => "<span class="elsevierStyleSectionTitle">Introduction</span><p id="spar0005" class="elsevierStyleSimplePara elsevierViewall">Neuromuscular disease &#40;NMD&#41; patients frequently have impaired cough&#46; Mechanical insufflation-exsufflation &#40;MI-E&#41; has proven efficacy in improving airway clearance&#44; however data related to its long-term home use is lacking&#46; The purpose of this study was to describe indications&#44; safety and compliance of home MI-E in NMD patients&#46;</p> <span class="elsevierStyleSectionTitle">Methods</span><p id="spar0010" class="elsevierStyleSimplePara elsevierViewall">Four years observational analysis of 21 NMD patients on home MI-E&#46; Diagnosis included bulbar and non-bulbar Amyotrophic Lateral Sclerosis &#40;ALS&#41; and other NMD&#46; Median age was 58 years&#46; Only cooperative patients with unassisted baseline Peak Cough Flow &#40;PCF&#41; &#60; 270 L&#47;min were included&#46; All patients were under continuous mechanical ventilation &#40;6 by tracheostomy&#41;&#46; Pulmonary function before initiation of MI-E &#40;median&#41;&#58; FVC &#61; 0&#46;81 L&#44; MIP &#61; 28 cmH<span class="elsevierStyleInf">2</span>O&#44; MEP &#61; 22 cmH<span class="elsevierStyleInf">2</span>O and PCF &#61; 60 L&#47;min&#46; MI-E was performed by previously trained non-professional caregivers&#44; with an on-call support of a trained health care professional&#46; Patients had pulse oximetry monitorization and applied MI-E whenever SpO<span class="elsevierStyleInf">2</span> &#60; 95 &#37;&#46; Median follow-up was 12 months &#40;3-41 months&#41;&#46;</p> <span class="elsevierStyleSectionTitle">Results</span><p id="spar0015" class="elsevierStyleSimplePara elsevierViewall">Ten patients &#40;9 ALS&#41; used MI-E daily&#46; Eleven patients used MI-E intermittently&#44; during exacerbations&#44; and in 8 patients early application of MI-E &#40;guided by oximetry feed-back&#41; avoided hospitalization&#46; All tracheostomized patients used MI-E daily and more times a day than patients under NIV&#46; Four patients &#40;3 bulbar ALS&#41;&#44; were hospitalized due to secretion encumbrance&#46; MI-E was well-tolerated and there were no complications&#46; In general&#44; caregivers considered MI-E effective&#46; During this period&#44; 4 patients died&#44; related to disease progression&#46;</p> <span class="elsevierStyleSectionTitle">Conclusions</span><p id="spar0020" class="elsevierStyleSimplePara elsevierViewall">Home MI-E is well tolerated&#44; effective and safe if used by well trained caregivers&#46; MI-E should be considered as a complement to mechanical ventilation&#46;</p>"
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      "es" => array:2 [
        "titulo" => "Resumen"
        "resumen" => "<span class="elsevierStyleSectionTitle">Introducci&#243;n</span><p id="spar0025" class="elsevierStyleSimplePara elsevierViewall">Con frecuencia&#44; los pacientes con enfermedades neuromusculares &#40;ENM&#41; presentan un deterioro del mecanismo de la tos&#46; Se ha demostrado la eficacia de la insuflaci&#243;n-exuflaci&#243;n mec&#225;nica &#40;IEM&#41; en la mejora del aclaramiento de las v&#237;as respiratorias aunque no se dispone de datos relacionados con su utilizaci&#243;n domiciliaria a largo plazo&#46; El objetivo del presente estudio fue describir las indicaciones&#44; tolerabilidad y cumplimiento con la IEM domiciliaria en pacientes con ENM&#46;</p> <span class="elsevierStyleSectionTitle">M&#233;todos</span><p id="spar0030" class="elsevierStyleSimplePara elsevierViewall">An&#225;lisis observacional de 4 a&#241;os de duraci&#243;n de 21 pacientes ENM tratados con IEM domiciliaria&#46; El diagn&#243;stico incluy&#243; esclerosis lateral amiotr&#243;fica &#40;ELA&#41; bulbar y no bulbar y otras ENM&#46; La edad mediana fue de 58 a&#241;os&#46; S&#243;lo se incluyeron pacientes cooperadores con valores de flujo pico de tos basal &#40;FTM&#41; no asistido &#60; 270 l&#47;min&#46; Todos los pacientes estaban sometidos a ventilaci&#243;n mec&#225;nica continua &#40;seis mediante traqueostom&#237;a&#41;&#46; La funci&#243;n pulmonar previa al inicio de la IEM &#40;mediana&#41; era&#58; FVC&#44; 0&#44;81 l&#44; MIP&#44; 28 cmH<span class="elsevierStyleInf">2</span>O&#44; MEP &#61; 22 cmH<span class="elsevierStyleInf">2</span>O y PCF &#61; 60 l&#47;min&#46; Cuidadores no profesionales adiestrados previamente con el apoyo permanente de un profesional sanitario experto efectuaron la IEM&#46; En los pacientes se monitoriz&#243; la pulsioximetr&#237;a y se aplic&#243; IEM siempre que la SpO<span class="elsevierStyleInf">2</span> fue &#60; 95 &#37;&#46; El seguimiento mediano fue de 12 meses &#40;3-41 meses&#41;&#46;</p> <span class="elsevierStyleSectionTitle">Resultados</span><p id="spar0035" class="elsevierStyleSimplePara elsevierViewall">Utilizaron diariamente IEM 10 pacientes &#40;nueve con ELA&#41;&#46; La utilizaron de forma intermitente 11 pacientes durante las exacerbaciones y en 8 la aplicaci&#243;n precoz de IEM &#40;guiada por la informaci&#243;n de la oximetr&#237;a&#41; evit&#243; la hospitalizaci&#243;n&#46; Todos los pacientes traqueostomizados utilizaron la IEM a diario y un mayor n&#250;mero de veces al d&#237;a que los pacientes sometidos a ventilaci&#243;n mec&#225;nica no invasiva &#40;VNI&#41;&#46; Requirieron ingreso hospitalario 4 pacientes &#40;3 ELA bulbar&#41; debido a la acumulaci&#243;n de secreciones&#46; La IEM fue bien tolerada y no se asoci&#243; a complicaciones&#46; En general&#44; los cuidadores la consideraron eficaz&#46; Durante este per&#237;odo&#44; 4 pacientes fallecieron&#44; en relaci&#243;n con la progresi&#243;n de la enfermedad&#46;</p> <span class="elsevierStyleSectionTitle">Conclusiones</span><p id="spar0040" class="elsevierStyleSimplePara elsevierViewall">La IEM domiciliaria es bien tolerada&#44; eficaz y segura cuando la utilizan cuidadores adiestrados apropiadamente&#46; Debe considerarse un complemento de la ventilaci&#243;n mec&#225;nica&#46;</p>"
      ]
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ISSN: 15792129
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