Acidosis metabólica por laxantes en paciente con distrofia muscular de Duchenne y ventilación mecánica no invasiva / Metabolic Acidosis Caused by Laxatives in a Patient with Duchenne Muscular Dystrophy Receiving Non-Invasive Mechanical Ventilation

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Benefits of Long-Term Pulmonary Rehabilitation Maintenance Program in Patients with Severe Chronic Obstructive Pulmonary Disease. Three-Year Follow-up.

RATIONALE: In chronic obstructive pulmonary disease (COPD), the benefits of pulmonary rehabilitation (PR) tend to wane over time. Whether maintenance techniques may help sustain the benefits achieved after completion of the initial PR program remains controversial. OBJECTIVES: To determine whether a long-term (3-yr) maintenance program after PR preserves the short-term effects on outcomes in patients with COPD. METHODS: This was a multicenter prospective randomized trial including 143 patients with moderate-severe COPD, with 3 years of PR maintenance following an 8-week outpatient PR program. Patients were randomized to maintenance intervention group (IG) and standard monitoring program or control group (CG). The effects on BODE index, 6-minute-walk test distance (6MWD), and health-related quality of life were compared at 12, 24, and 36 months. MEASUREMENTS AND MAIN RESULTS: A total of 138 (96.5%) completed the 8-week program. At this time, all outcomes (BODE, 6MWD, and health-related quality of life) showed clinically and statistically significant improvements (P ≤ 0.001). During the follow-up period, the magnitude of change in 6MWD differed between IG and CG (P = 0.042), with a slight initial increase in the IG during the first year and smaller decline afterward. The BODE index changes differed between baseline and measurements at Month 24 (P = 0.043). At 3 years, the adherence rate of IG patients was 66% and 17% for the CG group (P < 0.001). CONCLUSIONS: This study shows a 2-year beneficial effect of a program of rehabilitation maintenance on the BODE index and 6MWD when compared with a standard strategy. This effect vanishes after the second year of follow-up. Clinical trial registered with www.clinicaltrials.gov (NCT 01090999).

Atención integral a pacientes con esclerosis lateral amiotrófica: un modelo asistencial / Comprehensive Care of Amyotrophic Lateral Sclerosis Patients: A Care Model

La esclerosis lateral amiotrófica (ELA) es una enfermedad neurodegenerativa devastadora que se manifiesta por debilidad muscular y produce dificultades progresivas de movilización, comunicación, alimentación y, en última instancia, respiración, creando una dependencia creciente de familiares y de otros cuidadores. La manera ideal de afrontar los problemas derivados de la enfermedad y las decisiones necesarias es a través de equipos multidisciplinarios. Los objetivos fundamentales de estos equipos son optimizar la atención médica, facilitar la comunicación entre los miembros del equipo y consecuentemente mejorar la calidad asistencial. En nuestro centro tenemos una larga experiencia en la atención a pacientes con ELA a través de un equipo interdisciplinario cuya pretensión es asegurar la correcta asistencia del paciente desde el hospital hasta el propio domicilio. En este artículo mostramos los componentes del equipo, sus funciones y nuestro modo de trabajo (AU)

Amyotrophic lateral sclerosis (ALS) is a devastating neurodegenerative disease that presents with muscle weakness, causing progressive difficulty in movement, communication, eating and ultimately, breathing, creating a growing dependence on family members and other carers. The ideal way to address the problems associated with the disease, and the decisions that must be taken, is through multidisciplinary teams. The key objectives of these teams are to optimise medical care, facilitate communication between team members, and thus to improve the quality of care. In our centre, we have extensive experience in the care of patients with ALS through an interdisciplinary team whose aim is to ensure proper patient care from the hospital to the home setting. In this article, we describe the components of the team, their roles and our way of working (AU)

Comprehensive care of amyotrophic lateral sclerosis patients: a care model.

Amyotrophic lateral sclerosis (ALS) is a devastating neurodegenerative disease that presents with muscle weakness, causing progressive difficulty in movement, communication, eating and ultimately, breathing, creating a growing dependence on family members and other carers. The ideal way to address the problems associated with the disease, and the decisions that must be taken, is through multidisciplinary teams. The key objectives of these teams are to optimise medical care, facilitate communication between team members, and thus to improve the quality of care. In our centre, we have extensive experience in the care of patients with ALS through an interdisciplinary team whose aim is to ensure proper patient care from the hospital to the home setting. In this article, we describe the components of the team, their roles and our way of working.

Are oxygen-conserving devices effective for correcting exercise hypoxemia?

BACKGROUND: Correction of exercise hypoxemia in advanced lung diseases is crucial and often challenging. However, oxygen-conserving devices have been introduced in the market with limited evidence of effectiveness. In the present study the efficacy of 2 oxygen-conserving devices, a pulse demand oxygen delivery (DOD) system and pendant reservoir cannula (PRC), were evaluated in subjects with COPD and interstitial lung disease (ILD). METHODS: A cross-sectional, crossover study included 28 COPD and 31 ILD subjects with oxygen desaturation on the 6-min walk test (average S(pO2) < 88%). Each subject underwent 3 walk tests with DOD, PRC, and continuous oxygen flow by standard nasal cannula (CFNC), in random order, taking average S(pO2) ≥ 90% as the resaturation criterion. RESULTS: Exercise desaturation was corrected in 79%, 79%, and 86% of COPD subjects with CFNC, DOD, and PRC, respectively, and in 77%, 61%, and 81% of ILD subjects with CFNC, DOD, and PRC, respectively. When compared to CFNC, the oxygen-conserving devices showed similar efficacy, except a lower performance for the DOD in the ILD subjects (P = .01). CONCLUSIONS: Although these oxygen-conserving devices corrected exercise hypoxemia in most COPD and ILD subjects, correction was not achieved in about 20% of the severe COPD subjects, regardless of the device, and in nearly 40% of the ILD subjects with the DOD device. These findings underscore that individualized adjustment of oxygen flow is needed for optimal correction of exercise hypoxemia, especially with a DOD in an ILD patient. (ClinicalTrials.gov NCT01086891).

Estándares de calidad asistencial en rehabilitación respiratoria en pacientes con enfermedad pulmonar crónica / Standards for Quality Care in Respiratory Rehabilitation in Patients With Chronic Pulmonary Disease

La rehabilitación respiratoria (RR) ha demostrado ser eficaz con un alto nivel de evidencia en términos de mejora de los síntomas, la capacidad de esfuerzo y la calidad de vida relacionada con la salud (CVRS) en los pacientes con enfermedad pulmonar obstructiva crónica (EPOC) y en algunos pacientes con enfermedades distintas de la EPOC. De acuerdo con las guías internacionales, la RR está indicada fundamentalmente en todo paciente con síntomas respiratorios crónicos. Dependiendo de los mismos se le ofrecerá un tipo u otro de programa. Por encargo de la Sociedad Española de Neumología y Cirugía Torácica (SEPAR) hemos realizado este documento con el objetivo de unificar los criterios de calidad asistencial en RR. El documento esta organizado en 5 apartados que incluyen: las indicaciones de la RR, la evaluación de los candidatos, los componentes de los programas, las características de los programas de RR y el papel de la administración en la implantación de la RR. En cada apartado hemos distinguido 5 grandes grupos de enfermedades: EPOC, enfermedades respiratorias crónicas distintas de la EPOC con disnea limitante (ERCDL), enfermedades hipersecretoras, enfermedades neuromusculares con síntomas respiratorios y pacientes candidatos a cirugía torácica para una resección pulmonar(AU)

Respiratory rehabilitation (RR) has been shown to be effective with a high level of evidence in terms of improving symptoms, exertion capacity and health-related quality of life (HRQL) in patients with COPD and in some patients with diseases other than COPD. According to international guidelines, RR is basically indicated in all patients with chronic respiratory symptoms, and the type of program offered depends on the symptoms themselves. As requested by the Spanish Society of Pneumology and Thoracic Surgery (SEPAR), we have created this document with the aim to unify the criteria for quality care in RR. The document is organized into sections: indications for RR, evaluation of candidates, program components, characteristics of RR programs and the role of the administration in the implementation of RR. In each section, we have distinguished 5 large disease groups: COPD, chronic respiratory diseases other than COPD with limiting dyspnea, hypersecretory diseases, neuromuscular diseases with respiratory symptoms and patients who are candidates for thoracic surgery for lung resection(AU)

Standards for quality care in respiratory rehabilitation in patients with chronic pulmonary disease. Quality Healthcare Committee. Spanish Society of Pneumology and Thoracic Surgery (SEPAR).

Respiratory rehabilitation (RR) has been shown to be effective with a high level of evidence in terms of improving symptoms, exertion capacity and health-related quality of life (HRQL) in patients with COPD and in some patients with diseases other than COPD. According to international guidelines, RR is basically indicated in all patients with chronic respiratory symptoms, and the type of program offered depends on the symptoms themselves. As requested by the Spanish Society of Pneumology and Thoracic Surgery (SEPAR), we have created this document with the aim to unify the criteria for quality care in RR. The document is organized into sections: indications for RR, evaluation of candidates, program components, characteristics of RR programs and the role of the administration in the implementation of RR. In each section, we have distinguished 5 large disease groups: COPD, chronic respiratory diseases other than COPD with limiting dyspnea, hypersecretory diseases, neuromuscular diseases with respiratory symptoms and patients who are candidates for thoracic surgery for lung resection.

[Home vs hospital-based pulmonary rehabilitation for patients with chronic obstructive pulmonary disease: a Spanish multicenter trial]. / Comparación de un programa de rehabilitación domiciliario con uno hospitalario en pacientes con EPOC: estudio multicéntrico español.

OBJECTIVE: To compare the effects of a simple home pulmonary rehabilitation program and an intensive hospital-based program in terms of the exercise tolerance and health-related quality of life (HRQL) of patients with severe chronic obstructive pulmonary disease (COPD). PATIENTS AND METHODS: Patients in this prospective, multicenter trial were randomized to 2 groups to receive hospital or home pulmonary rehabilitation. Patients in both groups attended 2 informative sessions about the disease and 4 physical therapy sessions. Patients in the hospital group then carried out a structured exercise program while home group patients performed low intensity exercises at home without supervision. RESULTS: Twenty-eight patients were randomized to the hospital rehabilitation group and 23 to the home group. Both groups showed a similar improvement on the 6-minute walk test (mean difference, 8.7 m; P=.61). HRQOL measured with the Chronic Respiratory Questionnaire also improved in both groups, but the change was greater on the emotional function domain in the hospital rehabilitation group (mean difference between groups, 0.58 on a scale for which the smallest clinically relevant difference is 0.5 points). The benefits were maintained in both groups 6 months after the programs ended. CONCLUSIONS: This study demonstrates that the improvement in exercise tolerance achieved by COPD patients with an unsupervised home pulmonary rehabilitation program is similar to the gains of patients in an intensive hospital-based program. However, the hospital program afforded greater benefit on the HRQOL emotional function domain.

Comparación de un programa de rehabilitación domiciliario con uno hospitalario en pacientes con EPOC: estudio multicéntrico español / Home vs hospital-based pulmonary rehabilitation for patients with Chronic Obstructive Pulmonary Disease: a spanish multicenter trial

OBJETIVO: Estudiar el impacto de un programa simple de rehabilitación respiratoria domiciliario, comparado con uno intensivo hospitalario, sobre la capacidad de esfuerzo y la calidad de vida relacionada con la salud (CVRS) de los pacientes con enfermedad pulmonar obstructiva crónica. PACIENTES Y MÉTODOS: Se ha realizado un estudio prospectivo y multicéntrico en el que se aleatorizó a pacientes con EPOC grave en 2 grupos: hospital (GH) o domicilio (GD). En ambos los pacientes recibieron 2 sesiones de educación y 4 de fisioterapia. Los del GH realizaron un programa estructurado de ejercicio en el hospital y los del GD, un programa de ejercicio de baja intensidad en el domicilio, sin supervisión. RESULTADOS: Se aleatorizó a 28 pacientes en el GH y a 23 en el GD. Ambos grupos mostraron una mejoría similar en la prueba de la marcha de 6 min (diferencia media de 8,7 m; p = 0,61). A pesar de que en ambos grupos mejoró la CVRS, medida con el Cuestionario de Enfermedad Respiratoria Crónica (CRQ), se objetivó un incremento mayor en el área de función emocional para el GH (diferencia media entre grupos: 0,58, en una escala donde un valor de 0,5 representa la diferencia mínima importante). Los beneficios del programa se mantuvieron en ambos grupos hasta 6 meses después de finalizarlo. CONCLUSIONES: El estudio muestra que un programa domiciliario de rehabilitación respiratoria sin supervisión produce una mejoría similar en la capacidad de esfuerzo de los pacientes con EPOC que un programa intensivo hospitalario. Sin embargo, éste alcanza mayores beneficios en el área de la función emocional de la CVRS

OBJECTIVE: To compare the effects of a simple homepulmonary rehabilitation program and an intensivehospital-based program in terms of the exercise toleranceand health-related quality of life (HRQL) of patients withsevere chronic obstructive pulmonary disease (COPD).PATIENTS AND METHODS: Patients in this prospective,multicenter trial were randomized to 2 groups to receivehospital or home pulmonary rehabilitation. Patients in bothgroups attended 2 informative sessions about the disease and4 physical therapy sessions. Patients in the hospital groupthen carried out a structured exercise program while homegroup patients performed low intensity exercises at homewithout supervision.RESULTS: Twenty-eight patients were randomized to thehospital rehabilitation group and 23 to the home group. Bothgroups showed a similar improvement on the 6-minute walktest (mean difference, 8.7 m; P=.61). HRQOL measured withthe Chronic Respiratory Questionnaire also improved in bothgroups, but the change was greater on the emotional functiondomain in the hospital rehabilitation group (mean differencebetween groups, 0.58 on a scale for which the smallestclinically relevant difference is 0.5 points). The benefits weremaintained in both groups 6 months after the programs ended.CONCLUSIONS: This study demonstrates that theimprovement in exercise tolerance achieved by COPD patientswith an unsupervised home pulmonary rehabilitation programis similar to the gains of patients in an intensive hospital-basedprogram. However, the hospital program afforded greaterbenefit on the HRQOL emotional function domain

[Pulmonary and nonpulmonary alterations in Duchenne muscular dystrophy]. / Alteraciones pulmonares y no pulmonares en la distrofia muscular de Duchenne.

OBJECTIVE: To describe our experience in managing patients with Duchenne muscular dystrophy. PATIENTS AND METHODS: We analyzed the following variables in a group of 27 patients with Duchenne muscular dystrophy: arterial blood gases, lung function before and after mechanical ventilation, oxygen saturation (measured by pulse oximetry), nocturnal PaCO2 (measured transcutaneously by capnography), heart function, and dysphagia. RESULTS: The mean (SD) age was 26 (6) years and the mean age at which mechanical ventilation had initiated in the patients was 21 (5) years. Sixty-two percent had undergone tracheostomy and invasive mechanical ventilation. Arterial blood gas levels returned to normal once mechanical ventilation was administered and remained so for the entire treatment period (mean duration of follow-up, 56 [49] months). Thirteen patients had cardiac symptoms and they all presented abnormal electrocardiograms and echocardiograms indicating dilated cardiomyopathy, left ventricular dysfunction, and posterior hypokinesis. Only 9 patients were receiving enteral nutrition (7 through a gastrostomy tube and 2 through a nasogastric tube). The videofluoroscopic swallowing study confirmed that dysphagia was related to neuromuscular disease rather than the presence or not of a tracheostomy. Five patients (18%), 4 of whom were receiving invasive mechanical ventilation, died during the follow-up period. Three patients had serious heart disease. CONCLUSIONS: Mechanical ventilation confers clinical benefits and prolongs life expectancy in patients with Duchenne muscular dystrophy. Heart disease and feeding difficulties are determining factors in the prognosis of these patients.

Alteraciones pulmonares y no pulmonares en la distrofia muscular de Duchenne / Pulmonary and nonpulmonary alterations in Duchenne muscular dystrophy

Objetivo: Describir nuestra experiencia en el manejo de pacientes con distrofia muscular de Duchenne (DMD). Pacientes y métodos: En 27 pacientes con DMD analizamos los gases arteriales y la función pulmonar antes y después de la ventilación mecánica (VM); la pulsioximetría (saturación de oxihemoglobina) y la capnografía (presión arterial de anhídrido carbónico por determinación transcutánea) nocturna; la función cardíaca y la evaluación de la disfagia. Resultados: Se incluyó en el estudio a 27 pacientes con una edad media ± desviación estándar de 26 ± 6 años. Todos recibían VM, que se había iniciado cuando contaban 21 ± 5 años. El 62% eran portadores de traqueostomía y VM invasiva. Una vez iniciada la VM, se observó la normalización de los gases arteriales, que se mantuvo durante todo el tiempo de tratamiento (seguimiento medio: 56 ± 49 meses). Trece pacientes presentaban síntomas cardíacos y en todos ellos se observaban anormalidades en el electrocardiograma y ecocardiograma: miocardiopatía dilatada, disfunción ventricular izquierda o hipocinesia de la pared posterior. Sólo 7 pacientes llevaban una sonda de gastrostomía para alimentación y 2 una sonda nasogástrica. El estudio con videofluoroscopia permitió afirmar que los problemas de disfagia estaban relacionados con la enfermedad neuromuscular y no con la presencia o no de traqueostomía. Durante el período de seguimiento, 5 pacientes fallecieron (18%), 4 de ellos con VM invasiva; 3 pacientes presentaban una enfermedad cardíaca grave. Conclusiones: La VM proporciona beneficios clínicos y prolonga la vida de los pacientes con DMD. Las alteraciones cardíacas y nutricionales son factores determinantes en el pronóstico de estos pacientes

Objective: To describe our experience in managing patients with Duchenne muscular dystrophy. Patients and Methods: We analyzed the following variables in a group of 27 patients with Duchenne muscular dystrophy: arterial blood gases, lung function before and after mechanical ventilation, oxygen saturation (measured by pulse oximetry), nocturnal PaCO2 (measured transcutaneously by capnography), heart function, and dysphagia. Results: The mean (SD) age was 26 (6) years and the mean age at wich mechanical ventilation had initiatid in the patients was 21 (5) years. Sixty-two percent had undergone tracheostomy and invasive mechanical ventilation. Arterial blood gas levels returned to normal once mechanical ventilation was administered and remained so for the entire treatment period (mean duration of follow-up, 56 [49] months). Thirteen patients had cardiac symptoms and they all presented abnormal electrocardiograms and echocardiograms indicating dilated cardiomyopathy, left ventricular dysfunction, and posterior hypokinesis. Only 9 patients were receiving enteral nutrition (7 through a gastrostomy tube and 2 through a nasogastric tube). The videofluoroscopic swallowing study confirmed that dysphagia was related to neuromuscular disease rather than the presence or not of a tracheostomy. Five patients (18%), 4 of whom were receiving invasive mechanical ventilation, died during the follow-up period. Three patients had serious heart disease. Conclusions: Mechanical ventilation confers clinical benefits and prolongs life expectancy in patients with Duchenne muscular dystrophy. Heart disease and feeding difficulties are determining factors in the prognosis of these patients