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Background Noninvasive ventilation (NIV) is an effective form of treatment in obesity hypoventilation syndrome (OHS) with severe OSA. However, there is paucity of evidence in patients with OHS without severe OSA phenotype. Research Question Is NIV effective in OHS without severe OSA phenotype? Study Design and Methods In this multicenter, open-label parallel group clinical trial performed at 16 sites in Spain, we randomly assigned 98 stable ambulatory patients with untreated OHS and apnea-hypopnea index Results Forty-nine patients in the NIV group and 49 in the control group were randomized, and 48 patients in each group were analyzed. During a median follow-up of 4.98 years (interquartile range, 2.98-6.62), the mean hospitalization days per year ± SD was 2.60 ± 5.31 in the control group and 2.71 ± 4.52 in the NIV group (adjusted rate ratio, 1.07; 95% CI, 0.44-2.59; P = .882). NIV therapy, in contrast with the control group, produced significant longitudinal improvement in Paco2, pH, bicarbonate, quality of life (Medical Outcome Survey Short Form 36 physical component), and daytime sleepiness. Moreover, per-protocol analysis showed a statistically significant difference for the time until the first ED visit favoring NIV. In the subgroup with high NIV adherence, the time until the first event of hospital admission, ED visit, and mortality was longer than in the low adherence subgroup. Adverse events were similar between arms. Interpretation In stable ambulatory patients with OHS without severe OSA, NIV and lifestyle modification had similar long-term hospitalization days per year. A more intensive program aimed at improving NIV adherence may lead to better outcomes. Larger studies are necessary to better determine the long-term benefit of NIV in this subgroup of OHS. Trial Registry ClinicalTrials.gov; No.: NCT01405976; URL: www.clinicaltrials.gov

Spanish Sleep Network., [Rationale] Obesity hypoventilation syndrome (OHS) has been associated with cardiac dysfunction. However, randomized trials assessing the impact of long-term noninvasive ventilation (NIV) or continuous positive airway pressure (CPAP) on cardiac structure and function assessed by echocardiography are lacking. Rationale: Obesity hypoventilation syndrome (OHS) has been associated with cardiac dysfunction. However, randomized trials assessing the impact of long-term noninvasive ventilation (NIV) or continuous positive airway pressure (CPAP) on cardiac structure and function assessed by echocardiography are lacking., [Objectives] In a prespecified secondary analysis of the largest multicenter randomized controlled trial of OHS (Pickwick Project; N = 221 patients with OHS and coexistent severe obstructive sleep apnea), we compared the effectiveness of three years of NIV and CPAP on structural and functional echocardiographic changes., [Methods] At baseline and annually during three sequential years, patients underwent transthoracic two-dimensional and Doppler echocardiography. Echocardiographers at each site were blinded to the treatment allocation. Statistical analysis was performed using a linear mixed-effects model with a treatment group and repeated measures interaction to determine the differential effect between CPAP and NIV. Measurements and Main Results: A total of 196 patients were analyzed: 102 were treated with CPAP and 94 were treated with NIV. Systolic pulmonary artery pressure decreased from 40.5 ± 1.47 mm Hg at baseline to 35.3 ± 1.33 mm Hg at three years with CPAP, and from 41.5 ± 1.56 mm Hg to 35.5 ± 1.42 with NIV (P, [Conclusions] In patients with OHS who have concomitant severe obstructive sleep apnea, long-term treatment with NIV and CPAP led to similar degrees of improvement in pulmonary hypertension and left ventricular diastolic dysfunction.

[Rationale] Obesity hypoventilation syndrome (OHS) with concomitant severe obstructive sleep apnea (OSA) is treated with CPAP or noninvasive ventilation (NIV) during sleep. NIV is costlier, but may be advantageous because it provides ventilatory support. However, there are no long-term trials comparing these treatment modalities based on OHS severity., [Objective] To determine if CPAP have similar effectiveness when compared to NIV according to OHS severity subgroups., [Methods] Post hoc analysis of the Pickwick randomized clinical trial in which 215 ambulatory patients with untreated OHS and concomitant severe OSA, defined as apnoea-hypopnea index (AHI) ≥ 30 events/h, were allocated to NIV or CPAP. In the present analysis, the Pickwick cohort was divided in severity subgroups based on the degree of baseline daytime hypercapnia (PaCO2 of 45–49.9 or ≥50 mmHg). Repeated measures of PaCO2 and PaO2 during the subsequent 3 years were compared between CPAP and NIV in the two severity subgroups. Statistical analysis was performed using linear mixed-effects model., [Results] 204 patients, 97 in the NIV group and 107 in the CPAP group were analyzed. The longitudinal improvements of PaCO2 and PaO2 were similar between CPAP and NIV based on the PaCO2 severity subgroups., [Conclusion] In ambulatory patients with OHS and concomitant severe OSA who were treated with NIV or CPAP, long-term NIV therapy was similar to CPAP in improving awake hypercapnia, regardless of the severity of baseline hypercapnia. Therefore, in this patient population, the decision to prescribe CPAP or NIV cannot be solely based on the presenting level of PaCO2., [Introducción] El síndrome de hipoventilación-obesidad (SHO) con apnea obstructiva del sueño (AOS) grave concomitante se trata con CPAPo ventilación no invasiva (VNI) durante el sueño. La VNI es más costosa, pero puede ser beneficiosa porque proporciona soporte ventilatorio; sin embargo, no existen estudios a largo plazo que comparen estas modalidades de tratamiento basándose en la gravedad del SHO., [Objetivo] Determinar si la CPAP tiene una eficacia similar a la VNI según los subgrupos de gravedad del SHO., [Métodos] Análisis a posteriori del ensayo clínico aleatorizado Pickwick en el que 215 pacientes ambulatorios con SHO sin tratar y con AOS grave concomitante (definida como un índice de apnea-hipopnea [IAH] ≥ 30 episodios/hora) recibieron tratamiento con VNI o CPAP. En el presente análisis, la cohorte Pickwick se dividió en subgrupos según la gravedad basándose en el grado de hipercapnia diurna al inicio del estudio (PaCO2 de 45-49.9 mm Hg o ≥ 50 mm Hg). Se compararon las mediciones periódicas de PaCO2 y PaO2 durante los 3 años siguientes entre la CPAP y la VNI entre los dos subgrupos de gravedad. Se realizó un análisis estadístico utilizando un modelo lineal mixto., [Resultados] Se analizaron 204 pacientes, 97 en el grupo de VNI y 107 en el grupo de CPAP. Las mejoras lineales de PaCO2 y PaO2 fueron similares entre la CPAP y la NIV según los subgrupos de gravedad en función de la PaCO2., [Conclusión] En los pacientes ambulatorios con SHO y AOS grave concomitante a los que se trató con VNI o CPAP, el tratamiento a largo plazo con VNI resultó similar a la CPAP, en cuanto a la mejora de la hipercapnia en vigilia, independientemente de la gravedad de la hipercapnia de inicio. Por lo tanto, en esta población de pacientes la decisión de prescribir CPAP o VNI no puede basarse exclusivamente en el nivel de partida de PaCO2., Instituto de Salud Carlos III (Fondo de Investigaciones Sanitarias, Ministerio de Sanidad y Consumo, Madrid, Spain) PI050402, Spanish Respiratory Foundation 2005 (FEPAR) and Air Liquide Spain.

Background Emergency Department (ED) overcrowding mainly due to the lack of access to inpatient beds negatively affects safety and quality of care. Implementation of ED short-stay units (EDSSUs) may help to mitigate this situation. Objectives To describe the general characteristics and evaluate the activity of EDSSUs in Spanish hospitals. Methods This is a cross-sectional study. A questionnaire was sent to coordinators responsible for the EDSSUs identified among Spanish hospitals appearing on the Ministry of Health Web page. Data regarding structure, caseloads, and clinical management practices were collected. Results Among the 591 hospitals surveyed, 35 EDSSUs (5.9%) were identified and 23 participated in the study. Admissions to EDSSUs over different periods in 2011 were assessed: 12-month activity in 17 EDSSUs and between 5 and 10.5 months in six EDSSUs. A total of 25,568 patients with a mean age of 67.2 ± 9.8 years were admitted, representing between 6% and 16.3% of hospital admissions from the ED. The most frequent diagnoses were acute heart failure, chronic obstructive pulmonary disease exacerbation, and urinary and respiratory tract infections. The average length of stay (LOS) was 2.6 ± 1.1 days (range 1.2–5.3), in-hospital mortality 0.59% (range 0–2.68), and the 30-day readmission rate after discharge was 6.7% (range 0–14.6). Conclusions To date, only a few Spanish hospitals have implemented EDSSUs. Prevalent infections and exacerbation of chronic conditions are the most frequent causes for admission. Considering LOS, 30-day readmission rate and mortality, EDSSUs appear to be safe and effective and might be considered a tool to alleviate ED overcrowding.

Pompe disease is an autosomal-recessive lysosomal storage disorder characterized by progressive myopathy with proximal muscle weakness, respiratory muscle dysfunction, and cardiomyopathy (in infants only). In patients with juvenile or adult disease onset, respiratory muscle weakness may decline more rapidly than overall neurological disability. Sleep-disordered breathing, daytime hypercapnia, and the need for nocturnal ventilation eventually evolve in most patients. Additionally, respiratory muscle weakness leads to decreased cough and impaired airway clearance, increasing the risk of acute respiratory illness. Progressive respiratory muscle weakness is a major cause of morbidity and mortality in late-onset Pompe disease even if enzyme replacement therapy has been established. Practical knowledge of how to detect, monitor and manage respiratory muscle involvement is crucial for optimal patient care. A multidisciplinary approach combining the expertise of neurologists, pulmonologists, and intensive care specialists is needed. Based on the authors' own experience in over 200 patients, this article conveys expert recommendations for the diagnosis and management of respiratory muscle weakness and its sequelae in late-onset Pompe disease.

BACKGROUND: Research has provided evidence for the safety, feasibility, and efficacy of exercise training in patients with COPD. However, little is known about the impact of exercise training in patients with chronic respiratory failure due to kyphoscoliosis. We evaluated the effect of an exercise training program on exercise capacity, muscle strength, dyspnea, and quality-of-life indices in subjects with chronic respiratory failure due to kyphoscoliosis. METHODS: The 34 subjects were clinically stable, had been receiving nighttime home mechanical ventilation for ≥ 6 months, and were randomly assigned to the exercise group (n = 17) or the control group (n = 17). The exercise group conducted cycle and strength training on 3 non-consecutive days per week for 12 weeks. We measured pulmonary function, exercise capacity, peripheral muscle strength, dyspnea scores, and quality of life. RESULTS: Statistical analysis was carried out on the data from 16 subjects in the exercise group and in 11 subjects in the control group. Three of the lung-function parameters in the exercise group significantly changed: PaCO2 (P = .04), inspiratory pressure (P = .03), and expiratory pressure (P = .04); and endurance time (P = .002) and shuttle walk distance (P = .001) increased significantly. The exercise group had significantly greater improvements in peripheral muscle strength, dyspnea, and quality of life. CONCLUSIONS: In patients with chronic respiratory failure due to kyphoscoliosis, exercise training improved exercise capacity, peripheral muscle strength, dyspnea, and quality of life. (Deutschen Register Klinischer Studien DRKS00000443)

Unidad Funcional Interdisciplinaria Sociosanitaria (UFISS) Respiratoria, Hospital de Bellvitge, L’Hospitalet de Llobregat, Barcelona, Spain Servicio de Respiratorio, Hospital de la Santa Creu i Sant Pau, Barcelona, Spain Unidad Sueno y Ventilacion, S. Respiratorio, Hospital Universitario de Alava, Alava, Spain Equipo de Soporte de Cuidados Paliativos, Hospital Universitario de Alava, Alava, Spain Servicio de Neumologia, Hospital San Pedro de Alcantara, Caceres, CIBERES, ISCIII, Madrid, Spain Servicio de Respiratorio, Hospital de San Carlos, Madrid, Spain Complejo Hospitalario Universitario A Coruna, CHUAC, A Coruna, Spain Unidad de Cuidados Respiratorios, Servicio de Neumologia, Hospital Clinico Universitario de Valencia, Valencia, Spain Unidad Medico-Quirurgica de Enfermedades Respiratorias, Hospital Universitario Virgen del Rocio, Sevilla, Spain downloaded from http://www.archbronconeumol.org, day 16/05/2016. This copy is for personal use. Any transmission of this document by any media or format is strictly prohibited.

We conducted a pilot study of the effectiveness of home telehealth for patients with advanced chronic obstructive pulmonary disease treated with long-term oxygen therapy. Patients were randomized into a telehealth group (n = 24) and a control group (n = 21) who received usual care. Patients in the telehealth group measured their vital signs on weekdays and performed spirometry on two days per week. The data were transmitted automatically to a clinical call centre. After four months of monitoring the mean number of accident and emergency department visits in the telehealth group was slightly lower than in the control group (0.29 versus 0.43, P = 0.25). The mean number of hospital admissions was 0.38 in the telehealth group and 0.14 in the control group ( P = 0.47). During the study a total of 40 alerts were detected. The clinical triage process detected eight clinical exacerbations which were escalated by the case manager for a specialist consultation. There were clinically important differences in health-related quality of life in both groups. The mean score on the SGRQ was 10.9 versus 4.5 in the control group (P = 0.53). The EuroQol-5D score improved by 0.036 in the telehealth group and by 0.003 in the control group ( P = 0.68). Both patients and healthcare professionals showed a high level of satisfaction with the telehealth programme.

Background Obesity hypoventilation syndrome (OHS) is associated with a high burden of cardiovascular morbidity (CVM) and mortality. The majority of patients with OHS have concomitant OSA, but there is a paucity of data on the association between CVM and OSA severity in patients with OHS. The objective of our study was to assess the association between CVM and OSA severity in a large cohort of patients with OHS. Methods In a cross-sectional analysis, we examined the association between OSA severity based on tertiles of oxygen desaturation index (ODI) and CVM in 302 patients with OHS. Logistic regression models were constructed to quantify the independent association between OSA severity and prevalent CVM after adjusting for various important confounders. Results The prevalence of CVM decreased significantly with increasing severity of OSA based on ODI as a continuous variable or ODI tertiles. This inverse relationship between OSA severity and prevalence of CVM was seen in the highest ODI tertile and it persisted despite adjustment for multiple confounders. Chronic heart failure had the strongest negative association with the highest ODI tertile. No significant CVM risk change was observed between the first and second ODI tertiles. Patients in the highest ODI tertile were younger, predominantly male, more obese, more hypersomnolent, had worse nocturnal and daytime gas exchange, lower prevalence of hypertension, better exercise tolerance, and fewer days hospitalized than patients in the lowest ODI tertile. Conclusions In patients with OHS, the highest OSA severity phenotype was associated with reduced risk of CVM. This finding should guide the design of future clinical trials assessing the impact of interventions aimed at decreasing cardiovascular morbidity and mortality in patients with OHS. Trial Registry Clinicaltrial.gov; No.: NCT01405976 ; URL: www.clinicaltrials.gov

Patients with neuromuscular diseases usually require mechanical ventilation because of progression of their disease or after a hospitalization. In the latter situation, it is essential to optimize long-term care and to be able to transfer the patient in the best condition from the intensive care unit to home, ensuring their safety and comfort and avoiding invasive ventilation forms such as tracheostomy.

Pompe disease is an autosomal-recessive lysosomal storage disorder characterized by progressive myopathy with proximal muscle weakness, respiratory muscle dysfunction, and cardiomyopathy (in infants only). In patients with juvenile or adult disease onset, respiratory muscle weakness may decline more rapidly than overall neurological disability. Sleep-disordered breathing, daytime hypercapnia, and the need for nocturnal ventilation eventually evolve in most patients. Additionally, respiratory muscle weakness leads to decreased cough and impaired airway clearance, increasing the risk of acute respiratory illness. Progressive respiratory muscle weakness is a major cause of morbidity and mortality in late-onset Pompe disease even if enzyme replacement therapy has been established. Practical knowledge of how to detect, monitor and manage respiratory muscle involvement is crucial for optimal patient care. A multidisciplinary approach combining the expertise of neurologists, pulmonologists, and intensive care specialists is needed. Based on the authors' own experience in over 200 patients, this article conveys expert recommendations for the diagnosis and management of respiratory muscle weakness and its sequelae in late-onset Pompe disease. OA gold

Background: Severe acidosis can cause noninvasive ventilation (NIV) failure in chronic obstructive pulmonary disease (COPD) patients with acute hypercapnic respiratory failure (AHRF). NIV is therefore contraindicated outside of intensive care units (ICUs) in these patients. Less is known about NIV failure in patients with acute cardiogenic pulmonary edema (ACPE) and obesity hypoventilation syndrome (OHS). Therefore, the objective of the present study was to compare NIV failure rates between patients with severe and non-severe acidosis admitted to a respiratory intermediate care unit (RICU) with AHRF resulting from ACPE, COPD or OHS. Methods: We prospectively included acidotic patients admitted to seven RICUs, where they were provided NIV as an initial ventilatory support measure. The clinical characteristics, pH evolutions, hospitalization or RICU stay durations and NIV failure rates were compared between patients with a pH >= 7.25 and a pH < 7.25. Logistic regression analysis was performed to determine the independent risk factors contributing to NIV failure. (Continued on next page) Results: We included 969 patients (240 with ACPE, 540 with COPD and 189 with OHS). The baseline rates of severe acidosis were similar among the groups (45 % in the ACPE group, 41 % in the COPD group, and 38 % in the OHS group). Most of the patients with severe acidosis had increased disease severity compared with those with non-severe acidosis: the APACHE II scores were 21 +/- 7.2 and 19 +/- 5.8 for the ACPE patients (p < 0.05), 20 +/- 5.7 and 19 +/- 5.1 for the COPD patients (p < 0.01) and 18 +/- 5.9 and 17 +/- 4.7 for the OHS patients, respectively (NS). The patients with severe acidosis also exhibited worse arterial blood gas parameters: the PaCO2 levels were 87 +/- 22 and 70 +/- 15 in the ACPE patients (p < 0.001), 87 +/- 21 and 76 +/- 14 in the COPD patients, and 83 +/- 17 and 74 +/- 14 in the OHS patients (NS)., respectively Further, the patients with severe acidosis required a longer duration to achieve pH normalization than those with non-severe acidosis (patients with a normalized pH after the first hour: ACPE, 8 % vs. 43 %, p < 0.001; COPD, 11 % vs. 43 %, p < 0.001; and OHS, 13 % vs. 51 %, p < 0.001), and they had longer RICU stays, particularly those in the COPD group (ACPE, 4 +/- 3.1 vs. 3.6 +/- 2.5, NS; COPD, 5.1 +/- 3 vs. 3.6 +/- 2.1, p < 0.001; and OHS, 4.3 +/- 2.6 vs. 3.7 +/- 3.2, NS). The NIV failure rates were similar between the patients with severe and non-severe acidosis in the three disease groups (ACPE, 16 % vs. 12 %; COPD, 7 % vs. 7 %; and OHS, 11 % vs. 4 %). No common predictive factor for NIV failure was identified among the groups. Conclusions: ACPE, COPD and OHS patients with AHRF and severe acidosis (pH, Spanish Respiratory Foundation, 2011 (FEPAR)

Resumen Antecedentes y objetivo En los algoritmos diagnosticos de pacientes ambulatorios con sospecha de trombosis venosa profunda (TVP), se incluye la determinacion del dimero D (DD). Un valor elevado del DD no es diagnostico de TVP, pero una cifra normal contribuye a excluir una TVP. Desconocemos el mejor metodo para determinar el DD. Hemos analizado la utilidad clinica de 3 metodos cuantitativos para determinar el DD en pacientes ambulatorios con sospecha de TVP. Pacientes y metodos Incluimos a pacientes consecutivos, ambulatorios, con sospecha de TVP que fueron remitidos a la consulta de enfermedad tromboembolica venosa. Aplicamos un algoritmo diagnostico que incluia la determinacion de la probabilidad clinica de padecer una TVP (escala de Wells) y la determinacion del DD mediante 3 metodos cuantitativos (ELISA mini-VIDAS ® , Acute-care DDMR y DD-Plus). El diagnostico de TVP se confirmo mediante ecografia compresiva seriada de miembros inferiores. Analizamos la concordancia entre los 3 metodos analiticos para cuantificar el DD, asi como sus caracteristicas. Resultados Incluimos 306 pacientes (edad media: 60 anos; mujeres: 62%) con sospecha de TVP. La ecografia compresiva confirmo el diagnostico de TVP en el 23,8% de los casos. Los pacientes en los que se descarto TVP no fueron anticoagulados y no se produjo ningun evento tromboembolico en 3 meses de seguimiento. Los metodos de determinacion de DD que mejor se correlacionaron fueron el ELISA mini-VIDAS ® y el Acute-care DDMR. Ambos metodos mostraron una elevada sensibilidad y un valor predictivo negativo. El mejor metodo analitico para el subgrupo de pacientes con probabilidad clinica baja de TVP fue el ELISA mini-VIDAS ® . Conclusiones El metodo ELISA mini-VIDAS ® para la determinacion del DD descarta con seguridad una TVP en pacientes con baja probabilidad clinica.

Resumen Fundamento y objetivo Realizamos un estudio sobre seguridad, costes comparados y analisis de impacto presupuestario del tratamiento ambulatorio de pacientes con cancer y trombosis venosa profunda (TVP) frente al tratamiento hospitalario. Material y metodos Estudio observacional y prospectivo de pacientes con neoplasia conocida y diagnosticados de TVP desde 2003 hasta 2007. Las variables de resultados fueron mortalidad, recidivas y hemorragias a un mes. Realizamos un analisis economico para evaluar el coste comparado de los pacientes tratados ambulatoriamente frente al procedimiento habitual. Resultados Se incluyeron 317 pacientes, 55 (17%) con cancer. La edad media (DE) de los pacientes fue de 63 (11) anos. Las complicaciones a un mes fueron: 2 sangrados, 2 recidivas y 6 muertes. De todos los pacientes, solo 7 (13,7%) necesitaron ingreso hospitalario. Todas las muertes excepto una fueron debidas a la progresion de su enfermedad de base. Cuando realizamos el analisis economico observamos que el tratamiento ambulatorio es 6 veces menos costoso que el hospitalario, lo que implicaria una reduccion de costes de un 85%. Conclusiones El tratamiento ambulatorio a traves de una consulta monografica de los pacientes con cancer y TVP es seguro y podria ahorrar importantes recursos economicos.

There have been limited studies regarding the characteristics of subjects who have decided to initiate a cessation program to quit tobacco smoking. Identifying such characteristics is important because it provides information regarding recruiting and tailoring strategies, as well as intervention methods. This observational trial with cross-sectional data analysis sought to identify the characteristics of subjects who have decided to initiate a smoking cessation program compared with those who did not. All subjects who asked for information about our cessation program between January 2000 and December 2005 were eligible for participation. All subjects were interviewed regarding their tobacco consumption practices. We also collected information on medical history and psychological conditions. Multivariate analysis was used to identify variables associated with the decision to initiate the cessation program. A total of 1,681 subjects asked for information about smoking cessation at our center, and 1,132 (67.3%) decided to enter our program. Independent factors associated with the decision to initiate the cessation program included female gender (OR: 1.54; 95% CI:1.23-1.93), having a university degree (OR: 1.46; 95% CI: 1.12-1.88), bronchial asthma (OR: 0.41; 95% CI: 0.24-0.71), nonpsychotic psychiatric disorders (OR: 0.52; 95% CI: 0.40-0.66), psychotic disorders (OR: 6.00; 95% CI: 2.74-13.14), and a history of insomnia (OR: 6.22; 95% CI: 1.85-20.87). Although all smokers should be counseled to quit smoking, our data may help to concentrate efforts on some subjects in order to increase the participation in smoking cessation programs.

Resumen Introduccion Analizamos la eficacia de una intervencion conductual-cognitiva de alta intensidad frente a una intervencion minima iniciada durante un ingreso hospitalario, y si la combinacion con tratamiento sustitutivo con nicotina (TSN) puede aumentar las tasas de abstinencia a los 12 meses de seguimiento. Metodo Se estudiaron 2.560 fumadores activos durante un ingreso hospitalario. De ellos, 717 fumadores rehusaron entrar en el estudio y tras una intervencion minima se les solicitaba poder telefonearlos al ano para preguntar si continuaban fumando. El resto, 1.843 fumadores recibieron tratamiento cognitivo de alta intensidad y fueron aleatorizados para recibir o no TSN. El seguimiento tras el alta se realizaba en consultas externas o con sesiones telefonicas. Resultados Al ano de seguimiento, el 7% de los que rehusaron entrar en el estudio se mantenian sin fumar frente al 27% de los que entraron en el estudio (p Conclusiones Una intervencion de orientacion cognitiva iniciada en fumadores ingresados aumenta las tasas de abstinencia a los 12 meses frente a una intervencion minima, y aun aumenta de forma mas significativa dichas tasas si se le anade TSN.

We analysed the effectiveness of a high intensity behavioural-cognitive intervention compared to minimal intervention started during a hospital stay, to see if the combination of nicotine replacement therapy (NRT) can increase the quitting rate at 12 months of follow up.A total of 2560 active smokers were studied during their hospital stay. Of these, 717 smokers refused to enter the study and after a minimal intervention they were asked if we could telephone them after one year to ask if they still smoked. The remaining 1843 smokers who received high intensity cognitive therapy were randomised to receive or not receive NRT. The follow up after discharge was carried out by outpatient visits or with telephone sessions.At one year of follow up, 7% of those who declined to enter the study had stopped smoking compared to 27% of those who entered the study (p0.001). There were significant differences between the group that only had behavioural therapy (21% stopped) compared to the group that also had NRT (33% stopped; p=0.002). In this latter group there were significant differences (p=0.03) between those who had follow up in clinics (39% stopped) compared to those who were followed up telephone sessions (30%). In the multivariate analysis, the predictors of quitting at 12 months were: to have used NRT (OR 12.2; 95% CI, 5.2-32; p=0.002) and a higher score in the Richmond Test (OR 10.1; 95% CI, 3.9-24.2; p=0.01).A cognitive type intervention started on smokers when admitted to hospital increases quitting rates at 12 months, compared to a minimal intervention, and these rates increase even more significantly if NRT is added.

Summary Background Cryptogenic organizing pneumonia (COP) is a rare disease, and its diagnosis requires histological confirmation. The objective of our study was to describe the findings of the thoracic high-resolution computed tomography (HR-CT) and bronchoalveolar lavage (BAL) in patients with confirmed COP and evaluate the complementary diagnostic use of BAL and thoracic HR-CT. Methods Patients recorded in the registry of interstitial pulmonary diseases between 1991 and 2008 were located and the COP patients selected. Results We identified 21 patients with histological confirmation of COP. The median age was 58.0 ± 15.9 years, and 61.9% of patients were female. The most frequent thoracic HR-CT profile was patchy infiltrate (71.4%), followed by parenchymatous consolidation (42.9%). The most frequent BAL profile was mixed alveolitis (62%) with lymphocyte predominance, a CD4/CD8 index of 0.4 and foamy macrophages. The effectiveness of transbronchial biopsy was 66.6%. The diagnostic utility of Poletti’s BAL criteria gives us a specificity of 88.8%, although the sensitivity obtained was low. The specificity of certain HR-CT profiles is 99%. In addition, we observed a complementary use of the HR-CT and the BAL. Conclusions The imaging findings and BAL could be useful for patients with appropriate clinical presentation and for those whose transbronchial biopsy is negative or for whom a confirmatory biopsy cannot be performed.

Introduction An association between pulmonary embolism (PE) and a subsequent diagnosis of cancer has been repeatedly reported. Although screening and early detection might play a pivotal part in reducing mortality from cancer, there are currently no definite data to suggest that cancer screening may improve survival rates in patients with PE. We hereby present the results of a screening program and a two-year follow-up survey for detecting occult cancer in this patient population. Materials and methods A total of 107 patients with PE were consecutively enrolled. All subjects underwent an initial screening program followed by a two-year follow-up survey. We calculated the sensitivity of our screening program, and identified risk factors associated with occult cancer by means of logistic regression. Results The initial screening program yielded positive results in five patients (4.7%), and four additional cases were identified during the 2-year follow-up. The overall sensitivity of our screening program in idiopathic PE was 55.5%. In the entire study cohort, the number necessary for screening was 12.1 (6.1 in idiopathic PE, and 58 in secondary PE). Logistic regression analysis revealed that a shock index ≥ 1 (odds ratio: 5.467; p = 0.007) and idiopathic PE (odds ratio: 12.82; p = 0.03) were independent risk factors for occult cancer in our PE patients. Conclusions A simple and noninvasive screening program yields an acceptable sensitivity for detecting occult cancer in idiopathic PE patients. These results highlight the importance of screening for occult cancer in patients diagnosed with PE, especially in idiopathic forms.

Summary Objectives The objective of this study was to evaluate factors associated with health-related quality of life (HRQOL) among patients receiving home mechanical ventilation (HMV). Methods Observational, cross-sectional study. Patients receiving HMV were recruited from hospital outpatients facilities at five participating centers; a single follow-up visit was scheduled. The Spanish version of the Severe Respiratory Insufficiency (SRI) Questionnaire was used and the following variables were collected: socio-demographic status, previous medical history (Charlson-Age Comorbidity Index), current symptoms, administration of questionnaires, pulmonary function tests (PFT), current ventilatory support, and oxygen therapy. Results One hundred and fifteen patients (57 males and 58 females, mean age 62±13 years) were investigated. The reasons for HMV were as follows: thoracic cage abnormalities (33 patients), obesity hypoventilation syndrome (37 patients), neuromuscular disorders (18 patients), sequelae of tuberculosis (12 patients), and chronic obstructive pulmonary disease (15 patients). In a bivariate approach, dyspnea, the number of hospitalizations, and the number of emergency room admissions in last year were the main predictors of each HRQOL dimension. Multivariate analysis showed that dyspnea, FEV 1 /FVC, and the number of hospitalizations in the previous year were independently associated with HRQOL. Conclusions HRQOL of patients receiving HMV is influenced by many factors, especially by dyspnea and the number of admissions. An obstructive pattern in the PFT also influences HRQOL. These findings may have therapeutic implications.

Although treatment with alglucosidase alfa has helped improve the prognosis of patients with late-onset Pompe disease, both the development of the disease and the effectiveness of the treatment need to be monitored on a regular basis. This is the reason that has led a committee of Spanish experts to draw up a series of guidelines on how to follow up these patients. The committee proposes a model of follow-up tests for late-onset Pompe disease. First of all, the nutritional status and swallowing function must be evaluated. Second, and due to the variability of the clinical features, the committee recommends the simultaneous use of several scales to measure different functions and parameters. Thus, muscular force is assessed with the Medical Research Council scale; motor functioning, with the six-minute walk test and timed tests; disability, with the Rasch-built Pompe-specific Activity scale; respiratory functioning, with measurement of the forced vital capacity and oxygen saturation; and fatigue, with the fatigue intensity scale. Lastly, the safety and tolerability of enzyme replacement therapy are controlled by registering and treating the potential side effects and measurement of the anti-alglucosidase alfa antibodies. A number of different general recommendations are also included.Guia para el seguimiento de la enfermedad de Pompe de inicio tardio.Aunque el tratamiento con alglucosidasa alfa ha contribuido a mejorar el pronostico de los pacientes con enfermedad de Pompe de inicio tardio, es necesario hacer un seguimiento periodico de la evolucion de la enfermedad y de la eficacia del tratamiento. Por este motivo, un comite de expertos españoles ha elaborado una guia para el seguimiento de estos pacientes. El comite propone un modelo de pruebas de seguimiento para la enfermedad de Pompe de inicio tardio. En primer lugar, ha de valorarse el estado nutricional y la funcion deglutoria. En segundo lugar, y debido a la variabilidad del cuadro clinico, el comite recomienda el uso simultaneo de varias escalas que midan distintas funciones y parametros. De este modo, la fuerza muscular se evalua con la escala del Medical Research Council; la funcion motora, con la prueba de la marcha en seis minutos y pruebas cronometradas; la discapacidad, con la escala de actividad especifica de la enfermedad de Pompe construida segun el analisis de Rasch; la funcion respiratoria, con la medida de la capacidad vital forzada y la saturacion de oxigeno; y la fatiga, con la escala de intensidad de la fatiga. Por ultimo, la seguridad y la tolerabilidad del tratamiento enzimatico sustitutivo se controlan con el registro y tratamiento de los potenciales efectos adversos y la medicion de los anticuerpos antialglucosidasa alfa. Se incluyen tambien diversas recomendaciones generales.

Objetivo La ventilacion mecanica domiciliaria se aplica a pacientes con insuficiencia respiratoria cronica y grave, lo que tiene un impacto considerable en su calidad de vida. El objetivo de este trabajo ha sido realizar una traduccion y adaptacion transcultural para la poblacion espanola del cuestionario Severe Respiratory Insuficiency (SRI), el primer cuestionario de calidad de vida relacionada con la salud especifico para estos pacientes. Metodos Intervinieron 4 traductores bilingues aleman-espanol siguiendo el metodo de la traduccion y retrotraduccion. Despues de cada paso se realizaron reuniones con los traductores para obtener una version unica antes de continuar. Al final del proceso se realizo una prueba piloto para valorar su comprensibilidad. El proceso de traduccion se evaluo en dificultad y naturalidad, mediante una escala entre 1 (minimo) y 10 (maximo), asi como en equivalencia de los items con la version original. Resultados Se obtuvieron 3 versiones en espanol. La dificultad de la traduccion fue considerablemente baja para la traduccion (media ± desviacion estandar 1,4 ± 0,6) y la retrotraduccion (2,2 ± 1,1). La naturalidad de los items fue muy elevada y mejoro con las sucesivas versiones (version 1: 8,4; version 2: 8,7; version 3: 9,1; p Conclusiones La traduccion del cuestionario siguiendo el metodo de la traduccion-retrotraduccion ha dado una version espanola equiparable a la original y asequible para los pacientes. Actualmente se esta llevando a cabo la validacion del cuestionario mediante un estudio multicentrico.

Fundamento y objetivo En pacientes con enfermedad pulmonar obstructiva cronica (EPOC) eninsuficiencia respiratoria aguda hipercapnica se ha demostrado que la ventilacion mecanica noinvasiva (VMNI) con soporte de presion (mediante el dispositivo BiPAP) puede mejorar los parametrosclinicos y fisiologicos. El objetivo de nuestro estudio ha sido evaluar, en un trabajo prospectivoy aleatorizado, los beneficios del tratamiento con VMNI aplicada a los pacientes ingresadosen una planta de neumologia por descompensacion hipercapnica de su EPOC frente altratamiento estandar en un hospital de tercer nivel. Pacientes y metodo Se incluyo en el estudio a 41 pacientes que se asignaron de forma aleatoria algrupo de tratamiento medico convencional (grupo control) o la VMNI con soporte de presion (Bi-PAP ST, Respironics Inc., Murrysville, PA, EE.UU.) ademas del tratamiento estandar. No hubo diferenciasentre los dos grupos en los parametros clinicos o fisiologicos en el momento del ingreso. Resultados En el grupo de tratamiento con VMNI se observo una reduccion de la frecuencia respiratoriay mejoria del nivel de conciencia en las primeras 2 h respecto al grupo control (p Conclusiones La aplicacion temprana de VMNI en los pacientes con descompensacion hipercapnicade su EPOC consigue una mejoria clinica y fisiologica mas rapida frente al tratamiento estandar.El uso de esta modalidad terapeutica en una planta convencional de neumologia es factible.

Summary Background Non-invasive ventilation and exercise training might prove beneficial in the management of COPD patients. Objetive to compare the combined use of exercise training and non-invasive ventilation with the two interventions separately in chronic respiratory failure due to chronic obstructive pulmonary disease. As primary objective exercise capacity and secondary objectives gas exchange, peripheral muscle strength, BODE index, quality of life and systemic inflammatory response. Methods Forty-five patients with severe chronic obstructive pulmonary disease were randomized into three groups for an intervention of 12 weeks: exercise training alone, ventilation alone and combined treatment. We assessed exercise capacity, pulmonary function, BODE index, perception of dyspnoea, quality of life and several biomarkers. Results All exercise capacity parameters improved after training and the combined treatment. In addition, peripheral muscle strength and six-minute walk distance increased after ventilation. We found differences between the combined group and the ventilation group in submaximal effort and in oxygen consumption. Changes in respiratory function were observed in blood gases that improved after ventilation and the combined treatment, with differences between these groups. BODE index, perception of dyspnoea and quality of life improved in all three groups without differences between groups. Levels of interleukin 8 and tumour necrosis factor α decreased after ventilation, and interleukin 8, C-reactive protein and surfactant protein D decreased after training, while all four of these markers fell after the combined treatment. No differences between groups were found. Conclusions The combination of ventilation and exercise training had greater benefits than the separate treatments: improvements were observed in both blood gases and the levels of more biomarkers decreased. In addition, submaximal exercise capacity increased in all groups. The improvements seen in BODE index, perception of dyspnoea and quality of life were similar in all groups.

We conducted a cost-utility analysis of a telehealth programme for patients with severe chronic obstructive pulmonary disease (COPD) compared with usual care. A randomized controlled trial was carried out over four months with 45 patients treated with long-term oxygen therapy, 24 in the telehealth group (TG) and 21 in the control group (CG). The analysis took into account whether the severity of comorbidity (defined as the presence of additional chronic diseases co-occurring with COPD) was associated with differences in costs and/or quality-adjusted life years (QALYs). Results of cost-utility analysis were expressed in terms of the incremental cost-effectiveness ratio (ICER). The average total cost was €2300 for the TG and €1103 for the CG, and the average QALY gain was 0.0059 for the TG and 0.0006 for the CG (resulting an ICER of 223,726 €/QALY). For patients without comorbidity, the average total cost was €855 for the TG and €1354 for the CG, and the average QALY gain was 0.0288 for the TG and 0.0082 for the CG (resulting in the telehealth programme being the dominant strategy). For patients with comorbidity, the average total cost was €2782 for the TG and €949 for the CG, and the average QALY gain was −0.0017 for the TG and −0.0041 for the CG (resulting an ICER of 754,592 €/QALY). The telehealth programme may not have been cost-effective compared to usual care, although it could be considered cost-effective for patients without comorbidity.

Introduction Randomized clinical trials have demonstrated non-inferiority of rivaroxaban compared with vitamin K antagonists (VKAs) in the treatment of venous thromboembolism (VTE). Our objective was to analyze in real life, tolerance, recurrence, bleeding and adverse events of rivaroxaban in patients with acute symptomatic VTE. Material and Methods Open follow-up study of a cohort of patients aged 18 and over diagnosed with deep vein thrombosis (DVT) and/or pulmonary embolism (PE) treated with rivaroxaban from December 2011 to January 2014. Results The total number of patients treated with rivaroxaban was 103. The mean age was 58 +/-17 years. The most frequent co-morbidities were: hypertension (30.0%), dyslipidemia (23.3%) and respiratory disease (25.2%). The type of thromboembolic event treated was: DVT (64.1%), PE (18.4%), DVT + PE (17.5%). Of the rivaroxaban-treated patients, 30% did so from the initial anticoagulant therapy and the other 70% in long-term or extended anticoagulant therapy. The median time of treatment with rivaroxaban was 5 months. There was one recurrence and no deaths occurred. Six patients had bleeding, one of which was severe. Conclusions Rivaroxaban provides a therapeutic alternative in a group of patients with VTE with advantages over VKAs, because of the convenience in dosing, lack of requirements for periodic monitoring and limited interaction with other drugs.

Background: Bronchial variability in COPD patients may be a phenotypic feature associated with clinical characteristics and differential treatment response. Objectives: We analysed whether symptoms, quality of life and exercise capacity varied in COPD patients as a function of bronchodilator test results. Further, we compared response to an exercise programme in the groups. Methods: A positive bronchodilator test result was defined as FVC and/or FEV1>12% plus >200 ml improvement after 400 μg salbutamol. We studied 198 COPD patients, 94 with positive reversibility and 104 with negative reversibility. Training sessions were carried out on three non-consecutive days each week for 12 weeks, and consisted of a combination of resistance and strength training. Subjects were evaluated on two consecutive days at baseline, and at the end of the 12-week training programme. Results: Those with positive reversibility had shorter time—to-exhaustion on the endurance test (19.1±12.6 min versus 24.5±14.5 min in negative reversibility patients; p

Our aim is to assess the impact on respiratory function and exercise capacity of a respiratory rehabilitation program (RR) compared with a program of home mechanical ventilation (HMV) and the summation of both interventions. Methods: 45 COPD patients with severe functional impairment and in situations of hypercapnic respiratory failure were prospectively randomized to 3 groups: RR (12 weeks in 40-minute sessions that included strength training and resistance), HMV (12 weeks BiPAP mode) and RR-HMV (combination of the two interventions). Patients underwent pulmonary function tests. Exercise capacity was measured with a measurement of peripheral muscle strenght (PMS) using the one repetition maximum (1 RM) and maximal cardiopulmonary exercise testing performed on a cycle ergometer. Submaximal test of endurance was measure with cycle ergometer at 70% load. Results: Neither HMV or RR produced changes in respiratory function tests, except for the blood gases that improved after HMV (PaCO2 significant improvement with a decrease of 4 mmHg, p = 0.008) and in the combined with improvement in PaO2 (increase of more than 3 mmHg, p = 0.02) and PaCO2 (decrease of 5 mmHg, p

Our aim is to assess the impact on BODE index, quality of live and inflammatory parameters (IP) of a respiratory rehabilitation program (RR) compared with a program of home mechanical ventilation (HMV) and the summation of both interventions. Methods: 45 COPD patients with severe functional impairment and in situations of hypercapnic respiratory failure were prospectively randomized to 3 groups: RR (12 weeks in 40-minute sessions that included strength training and resistance), HMV (12 weeks BiPAP mode) and RR-HMV (combination of the two interventions). The patients underwent diferents components of BODE index, quality of live (CRDQ) and IP: C Reactive Protein (CRP), Tumor Necrosis Factor-α (TNF-α), Interleucin-6 (IL-6), IL-8 and Surfanctan D Protein (SDP). Results: HMV group improved BODE index significantly by decreasing median 2 points (p = 0.003). Also get statistically significant improvement in quality of life index CRDQ (in total and its four components). IP improved significantly by decreasing TNF-α and IL-8. RR group obtained the same results in BODE index, and CRDQ. IP significantly improved by lowering CRP, IL-8, SDP and C3. RR-HMV group improves BODE index significantly when going from 5 to 3.5 (p = 0.001). CRDQ in total and components of dyspnea, fatigue and disease control. Significantly improved IP by lowering CRP, TNF-α, SDP and prealbumin. Conclusions: HMV and the RR produced improvement in the BODE index and quality of life as well as when the two interventions are given together without finding differences when applied independently. The combination of both intervention control more IP than each other separately.

Aquaporin-1 (AQP1) is the main water channel responsible for water transport through many epithelia and endothelia. The latest evidence pointed toward an important role of this protein also in gas permeation, angiogenesis, cell proliferation and migration. In the present work we studied the expression of AQP1 by immunohistochemical staining of 92 lung biopsies from patients diagnosed with a pleuro-pulmonary tumor (71 lung and 21 pleural neoplasms). AQP1 expression was analyzed comparing the results among the different histological patterns and against 9 control cases (5 parenchyma and 4 healthy pleura). Clear staining of AQP1 was detected in 39 of the 92 tumors analyzed. In parenchyma, AQP1 was more frequently detected in primary lung adenocarcinomas (55%, P0.001); in contrast, small cell carcinomas were the least AQP1 expressive tumors studied (93% of negative staining, P0.05). Carcinomas analyzed in pleura (mesotheliomas and metastatic adenocarcinomas) also revealed strong expression of AQP1. High expression of this protein was detected in small capillaries in areas near or surrounding the tumor, and novel intense AQP1 immunostaining was detected over thicker alveolar walls in alveoli inside or next to the tumoral tissue regardless of the tumor type. An important role of AQP1 in tumor angiogenesis is sustained by the abundant expression of this protein in the endothelia of tumor capillaries. Further studies are necessary to elucidate the potential pathophysiological role of this protein in pleuro-pulmonary neoplasms.

This is a safety and cost comparison study with an analysis of budgetary impact of ambulatory management of patients with cancer and deep vein thrombosis (DVT) compared with hospital management.Prospective observational study of patients with known malignancy and diagnosed with DVT from 2003 to 2007. The outcome variables were mortality, relapse and bleeding in one month. We conducted an economic analysis to evaluate the comparative cost of ambulatory patients.Three hundred and seventeen patients, 55 (17%) had cancer. The mean age of patients was 63 ± 11 years. There were 2 hemorrhagic events, 2 recurrences and 6 deaths in one month of follow-up. Of all patients, only 7 (13,7%) required hospitalization. All but one deaths were due to progression of the underlying disease. Economic analysis concluded that outpatient management is 6 times less expensive than hospital management, which would imply a cost reduction of 85%.Specialized outpatient treatment of cancer patients with DVT is safe and could save significant financial resources.

Objectives: To analyse survival rate after 24 months in consecutive patients with a diagnosis of PE as well as associated factors. Methods: Prospective cohort study during a follow-up period of two years in a series of consecutive patients with PE. Results: During the follow-up period, 34 out of 148 patients died (23%). Factors independently associated with reduced survival rate were: creatinine levels > 2 (OR, 8.8; 95% CI, 1.1 - 70.87), previous neoplasm (OR, 8.8; 95% CI, 3.69 - 20.98), dementia (OR, 6.85; 95% CI, 2.1 - 22.33) and dyslipidemia (OR, 5.07; 95% CI, 1.92 - 13.44). Forty four percent of the patients with dyslipidemia died vs. 20.8% of patients without this condition. Conclusions: In our study dyslipidemia shows as a long-term negative prognostic marker for survival in patients with EP. Resumo: Objetivos: Analisar a taxa de sobrevivência após 24 meses, em pacientes consecutivos com diagnóstico de PE, bem como fatores associados. Métodos: Estudo prospectivo durante um período de seguimento de dois anos em uma série consecutiva de pacientes com PE. Resultados: Durante o período de acompanhamento, 34 dos 148 pacientes morreram (23%). Fatores independentemente associados à reduzida taxa de sobrevivência foram: os níveis de creatinina> 2 (OR, 8,8; 95% CI, 1,1-70,87), neoplasia anterior (OR, 8,8; IC 95%, 3,69-20,98), demência (OR, 6,85; 95% CI, 2,1-22,33) e dislipidemia (OR, 5,07; IC 95%, 1,92-13,44). Quarenta e quatro por cento dos pacientes com dislipidemia morreram contra 20,8% dos pacientes sem essa condição. Conclusões: No nosso estudo, a dislipidemia mostra-se um marcador prognóstico negativo de longo prazo na sobrevida de pacientes com EP. Keywords: Dyslipidemia, Lipid metabolic disorders, Pulmonary embolism, Survival analysis, Venous thromboembolism, Palavras-chave: Dislipidemia, Doenças metabólicas lipídicas, Embolia pulmonar, Análise de sobrevivência, Tromboembolismo venoso

We have developed a system for computer-assisted surgical planning of tracheal surgeries. The system allows to plan the intervention based on CT images of the patient, and includes a virtual database of commercially available prostheses. Automatic segmentation of the trachea and apparent pathological structures is obtained using a modified region growing algorithm. A method for automatic adaptation of a finite element mesh allows to build a patient-specific biomechanical model for simulation of the expected performance of the implant under physiological movement (swallowing, sneezing). Laboratory experiments were performed to characterise the tissues present in the trachea, and movement models were obtained from fluoroscopic images of a patient. Results are reported on the planning and biomechanical simulation of two patients that underwent surgery at our hospital.

The education and training of medical personnel in noninvasive ventilation (NIV) is essential in improving outcomes, reducing failures, and avoiding complications associated with the use of ventilation [1]. Furthermore, adequate training of staff provides a cost reduction in patient care. In this regard, a proper training period should be part of the education of respiratory health care professions who are part of the multidisciplinary respiratory team dealing with the NIV.