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        "resumen" => "<span class="elsevierStyleSectionTitle">Objective</span><p class="elsevierStyleSimplePara elsevierViewall">To describe our experience in managing patients with Duchenne muscular dystrophy&#46;</p> <span class="elsevierStyleSectionTitle">PATIENTS AND METHODS</span><p class="elsevierStyleSimplePara elsevierViewall">We analyzed the following variables in a group of 27 patients with Duchenne muscular dystrophy&#58; arterial blood gases&#44; lung function before and after mechanical ventilation&#44; oxygen saturation &#40;measured by pulse oximetry&#41;&#44; nocturnal PaCO<span class="elsevierStyleInf">2</span> &#40;measured transcutaneously by capnography&#41;&#44; heart function&#44; and dysphagia&#46;</p> <span class="elsevierStyleSectionTitle">RESULTS</span><p class="elsevierStyleSimplePara elsevierViewall">The mean &#40;SD&#41; age was 26 &#40;6&#41; years&#44; and the mean age at which mechanical ventilation had been initiated in the 27 patients was 21 &#40;5&#41; years&#46; Sixty-two percent had undergone tracheostomy and invasive mechanical ventilation&#46; Arterial blood gas levels returned to normal once mechanical ventilation was administered and remained so for the entire treatment period &#40;mean duration of follow- up&#44; 56 &#91;49&#93; months&#41;&#46; Thirteen patients had cardiac symptoms and they all presented abnormal electrocardiograms and echocardiograms indicating dilated cardiomyopathy&#44; left ventricular dysfunction&#44; and posterior hypokinesis&#46; Only 9 patients were receiving enteral nutrition &#40;7 through a gastrostomy tube and 2 through a nasogastric tube&#41;&#46; The videofluoroscopic swallowing study confirmed that dysphagia was related to neuromuscular disease rather than the presence or not of a tracheostomy&#46; Five patients &#40;18&#37;&#41;&#44; 4 of whom were receiving invasive mechanical ventilation&#44; died during the follow-up period&#46; Three patients had serious heart disease&#46;</p> <span class="elsevierStyleSectionTitle">CONCLUSIONS</span><p class="elsevierStyleSimplePara elsevierViewall">Mechanical ventilation confers clinical benefits and prolongs life expectancy in patients with Duchenne muscular dystrophy&#46; Heart disease and feeding difficulties are determining factors in the prognosis of these patients&#46;</p>"
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        "resumen" => "<span class="elsevierStyleSectionTitle">Objetivo</span><p class="elsevierStyleSimplePara elsevierViewall">Describir nuestra experiencia en el manejo de pacientes con distrofia muscular de Duchenne &#40;DMD&#41;&#46;</p> <span class="elsevierStyleSectionTitle">PACIENTES Y M&#201;TODOS</span><p class="elsevierStyleSimplePara elsevierViewall">En 27 pacientes con DMD analizamos los gases arteriales y la funci&#243;n pulmonar antes y despu&#233;s de la ventilaci&#243;n mec&#225;nica &#40;VM&#41;&#59; la pulsioximetr&#237;a &#40;saturaci&#243;n de oxihemoglobina&#41; y la capnograf&#237;a &#40;presi&#243;n arterial de anh&#237;drido carb&#243;nico por determinaci&#243;n transcut&#225;nea&#41; nocturna&#59; la funci&#243;n card&#237;aca y la evaluaci&#243;n de la disfagia&#46;</p> <span class="elsevierStyleSectionTitle">RESULTADOS</span><p class="elsevierStyleSimplePara elsevierViewall">Se incluy&#243; en el estudio a 27 pacientes con una edad media &#177; desviaci&#243;n est&#225;ndar de 26 &#177; 6 a&#241;os&#44; 24 de los cuales recib&#237;an VM&#44; que se hab&#237;a iniciado cuando contaban 21 &#177; 5 a&#241;os&#46; El 62&#37; eran portadores de traqueostom&#237;a y VM invasiva&#46; Una vez iniciada la VM&#44; se observ&#243; la normalizaci&#243;n de los gases arteriales&#44; que se mantuvo durante todo el tiempo de tratamiento &#40;seguimiento medio&#58; 56 &#177; 49 meses&#41;&#46; Trece pacientes presentaban s&#237;ntomas card&#237;acos y en todos ellos se observaban anormalidades en el electrocardiograma y ecocardiograma&#58; miocardiopat&#237;a dilatada&#44; disfunci&#243;n ventricular izquierda o hipocinesia de la pared posterior&#46; S&#243;lo 7 pacientes llevaban una sonda de gastrostom&#237;a para alimentaci&#243;n y 2 una sonda nasog&#225;strica&#46; El estudio con videofluoroscopia permiti&#243; afirmar que los problemas de disfagia estaban relacionados con la enfermedad neuro- muscular y no con la presencia o no de traqueostom&#237;a&#46; Durante el per&#237;odo de seguimiento&#44; 5 pacientes fallecieron &#40;18&#37;&#41;&#44; 4 de ellos con VM invasiva&#59; 3 pacientes presentaban una enfermedad card&#237;aca grave&#46;</p> <span class="elsevierStyleSectionTitle">CONCLUSIONES</span><p class="elsevierStyleSimplePara elsevierViewall">La VM proporciona beneficios cl&#237;nicos y prolonga la vida de los pacientes con DMD&#46; Las alteraciones card&#237;acas y nutricionales son factores determinantes en el pron&#243;stico de estos pacientes&#46;</p>"
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Original Articles
Pulmonary and Nonpulmonary Alterations in Duchenne Muscular Dystrophy
María Rosa Güella,
Corresponding author
mguellr@santpau.es

Correspondence: Dra M.R. Güell. Departament de Pneumologia. Hospital de la Santa Creu i de Sant Pau. Sant Antoni M. Claret, 167. 08025 Barcelona. España
, Mónica Avendanob, Janet Fraserb, Roger Goldsteinb
a Departament de Pneumologia, Hospital de la Santa Creu i Sant Pau, Barcelona, Spain
b Respiratory Medicine, West Park Hospital, Toronto, Canada
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        "resumen" => "<span class="elsevierStyleSectionTitle">Objective</span><p class="elsevierStyleSimplePara elsevierViewall">To describe our experience in managing patients with Duchenne muscular dystrophy&#46;</p> <span class="elsevierStyleSectionTitle">PATIENTS AND METHODS</span><p class="elsevierStyleSimplePara elsevierViewall">We analyzed the following variables in a group of 27 patients with Duchenne muscular dystrophy&#58; arterial blood gases&#44; lung function before and after mechanical ventilation&#44; oxygen saturation &#40;measured by pulse oximetry&#41;&#44; nocturnal PaCO<span class="elsevierStyleInf">2</span> &#40;measured transcutaneously by capnography&#41;&#44; heart function&#44; and dysphagia&#46;</p> <span class="elsevierStyleSectionTitle">RESULTS</span><p class="elsevierStyleSimplePara elsevierViewall">The mean &#40;SD&#41; age was 26 &#40;6&#41; years&#44; and the mean age at which mechanical ventilation had been initiated in the 27 patients was 21 &#40;5&#41; years&#46; Sixty-two percent had undergone tracheostomy and invasive mechanical ventilation&#46; Arterial blood gas levels returned to normal once mechanical ventilation was administered and remained so for the entire treatment period &#40;mean duration of follow- up&#44; 56 &#91;49&#93; months&#41;&#46; Thirteen patients had cardiac symptoms and they all presented abnormal electrocardiograms and echocardiograms indicating dilated cardiomyopathy&#44; left ventricular dysfunction&#44; and posterior hypokinesis&#46; Only 9 patients were receiving enteral nutrition &#40;7 through a gastrostomy tube and 2 through a nasogastric tube&#41;&#46; The videofluoroscopic swallowing study confirmed that dysphagia was related to neuromuscular disease rather than the presence or not of a tracheostomy&#46; Five patients &#40;18&#37;&#41;&#44; 4 of whom were receiving invasive mechanical ventilation&#44; died during the follow-up period&#46; Three patients had serious heart disease&#46;</p> <span class="elsevierStyleSectionTitle">CONCLUSIONS</span><p class="elsevierStyleSimplePara elsevierViewall">Mechanical ventilation confers clinical benefits and prolongs life expectancy in patients with Duchenne muscular dystrophy&#46; Heart disease and feeding difficulties are determining factors in the prognosis of these patients&#46;</p>"
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        "resumen" => "<span class="elsevierStyleSectionTitle">Objetivo</span><p class="elsevierStyleSimplePara elsevierViewall">Describir nuestra experiencia en el manejo de pacientes con distrofia muscular de Duchenne &#40;DMD&#41;&#46;</p> <span class="elsevierStyleSectionTitle">PACIENTES Y M&#201;TODOS</span><p class="elsevierStyleSimplePara elsevierViewall">En 27 pacientes con DMD analizamos los gases arteriales y la funci&#243;n pulmonar antes y despu&#233;s de la ventilaci&#243;n mec&#225;nica &#40;VM&#41;&#59; la pulsioximetr&#237;a &#40;saturaci&#243;n de oxihemoglobina&#41; y la capnograf&#237;a &#40;presi&#243;n arterial de anh&#237;drido carb&#243;nico por determinaci&#243;n transcut&#225;nea&#41; nocturna&#59; la funci&#243;n card&#237;aca y la evaluaci&#243;n de la disfagia&#46;</p> <span class="elsevierStyleSectionTitle">RESULTADOS</span><p class="elsevierStyleSimplePara elsevierViewall">Se incluy&#243; en el estudio a 27 pacientes con una edad media &#177; desviaci&#243;n est&#225;ndar de 26 &#177; 6 a&#241;os&#44; 24 de los cuales recib&#237;an VM&#44; que se hab&#237;a iniciado cuando contaban 21 &#177; 5 a&#241;os&#46; El 62&#37; eran portadores de traqueostom&#237;a y VM invasiva&#46; Una vez iniciada la VM&#44; se observ&#243; la normalizaci&#243;n de los gases arteriales&#44; que se mantuvo durante todo el tiempo de tratamiento &#40;seguimiento medio&#58; 56 &#177; 49 meses&#41;&#46; Trece pacientes presentaban s&#237;ntomas card&#237;acos y en todos ellos se observaban anormalidades en el electrocardiograma y ecocardiograma&#58; miocardiopat&#237;a dilatada&#44; disfunci&#243;n ventricular izquierda o hipocinesia de la pared posterior&#46; S&#243;lo 7 pacientes llevaban una sonda de gastrostom&#237;a para alimentaci&#243;n y 2 una sonda nasog&#225;strica&#46; El estudio con videofluoroscopia permiti&#243; afirmar que los problemas de disfagia estaban relacionados con la enfermedad neuro- muscular y no con la presencia o no de traqueostom&#237;a&#46; Durante el per&#237;odo de seguimiento&#44; 5 pacientes fallecieron &#40;18&#37;&#41;&#44; 4 de ellos con VM invasiva&#59; 3 pacientes presentaban una enfermedad card&#237;aca grave&#46;</p> <span class="elsevierStyleSectionTitle">CONCLUSIONES</span><p class="elsevierStyleSimplePara elsevierViewall">La VM proporciona beneficios cl&#237;nicos y prolonga la vida de los pacientes con DMD&#46; Las alteraciones card&#237;acas y nutricionales son factores determinantes en el pron&#243;stico de estos pacientes&#46;</p>"
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