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CF-COVID19-Spain Registry Group., [Background] Given the high incidence of confirmed infection by SARS-CoV-2 and mortality by COVID-19 in the Spanish population, its impact was analysed among persons with Cystic Fibrosis (CF) as a group at risk of a worse evolution. The possible causes of the incidence observed in them are explained and how CF Units have faced this health challenge is detailed., [Methods] Retrospective descriptive observational study, for which a Spanish CF Patients with Confirmed COVID-19 Registry is created, requesting information on number of people affected between 8 March–16 May 2020 and their clinical-demographic characteristics from the CF Units participating in the European Cystic Fibrosis Society Patient Registry (ECFSPR). The accumulated incidence is calculated, compared with that of the general population. Additionally, a survey (CF-COVID19-Spain) is carried out on prevention of SARS-CoV-2 infection, workings of CF Units and possible reasons for the incidence observed., [Results] COVID-19 was diagnosed in eight CF patients, one of whom had received a lung transplant. The accumulated incidence was 32/10000 in CF patients and 49/10000 in the general population. General death rate was 5.85/10000 while no CF patients included in the ECFSPR died. The characteristics of those affected and the results of the survey are described., [Conclusions] Despite being considered a disease at high risk of severe COVID-19, the low incidence and mortality in CF patients in Spain contrasts with the figures for the general population. The possible factors that would explain such findings are discussed, with the help of the results of the CF-COVID19-Spain survey.

Introduction: The criteria of Murray and Washington to rule out sputum culture may not be suitable for patients with non-cystic fibrosis bronchiectasis, due to their high pre-test probability of isolating potential pathogenic microorganisms (PPM) and the prognostic and therapeutic implications of these isolations. Objectives: Analyze the profitability of growing sputum samples classified as “not valid” by Gram stain in patients with non-cystic fibrosis bronchiectasis and describe the PPM isolated. Methods: We collected sputum samples from non-cystic fibrosis bronchiectasis patients from November 2016 to May 2017. We performed Gram stain and culture on plates of blood agar, chocolate agar and McConkey, incubated at 37oC during 72 hours, in all the sputum samples. The predominant growth of a PPM was considered as a positive culture. Results: We achieved 372 sputum samples. 112 of them were classified as “not valid” by Gram stain. The 112 “not valid” samples were obtained from 80 patients (40% ♀ 60% ♂) with an average age of 70 S.D. 12 years. 71% were collected in stable phase and 29% during exacerbation. PPM were isolated in 42% of the 112 samples. The most frequent was P. aeruginosa (40% of the isolated PPM). There were no differences during exacerbation or stable phase. Conclusions: The profitability of sputum culture of “not valid” samples by Gram stain in patients with non-cystic fibrosis bronchiectasis was 42%. The most frequently isolated PPM was P. aeruginosa. The Murray and Washington criteria may not be an appropiate method to refuse the sample, since early detection of P. aeruginosa has an impact on the management.

The persistent isolation of Pseudomonas aeruginosa in the airways of non-cystic fibrosis bronchiectasis (NCFB) patients is associated with a worsening of the symptoms, increase of exacerbations, poor quality of life and functional impairment. The objective of this study was the analysis of the eradication rate of P. aeruginosa in the sputum of patients with NCFB treated with inhaled colistin and the effects of the treatment in the exacerbations. This was a prospective, cohort, study of 67 NCFB patients treated with inhaled colistin at the Hospital of A Coruña (Spain). We recorded dyspnoea, exacerbations, lung function and sputum cultures of P. aeruginosa in the patients. The mean age of the patients was 67.25 ± 14.6 years (59.7% male). The percentages of eradication of P. aeruginosa in sputum at 3, 6, 9 and 12 months were 61.2%, 50.7%, 43.3% and 40.3%, respectively. We observed a significant decrease in exacerbations after 1 year of colistin treatment (1.98 ± 3.62) versus the previous year (3.40 ± 4.21, p < 0.001). We conclude that treatment with inhaled colistin in patients with NCFB and P. aeruginosa in sputum can achieve high rates of eradication even in patients with several previous positive cultures, as well as a significant decrease of exacerbations and hospital admissions.

[Abstract] The persistent isolation of Pseudomonas aeruginosa in the airways of non-cystic fibrosis bronchiectasis (NCFB) patients is associated with a worsening of the symptoms, increase of exacerbations, poor quality of life and functional impairment. The objective of this study was the analysis of the eradication rate of P. aeruginosa in the sputum of patients with NCFB treated with inhaled colistin and the effects of the treatment in the exacerbations. This was a prospective, cohort, study of 67 NCFB patients treated with inhaled colistin at the Hospital of A Corun˜a (Spain).We recorded dyspnoea, exacerbations, lung function and sputum cultures of P. aeruginosa in the patients. The mean age of the patients was 67.25+14.6 years (59.7% male). The percentages of eradication of P. aeruginosa in sputum at 3, 6, 9 and 12 months were 61.2%, 50.7%, 43.3% and 40.3%, respectively.We observed a significant decrease in exacerbations after 1 year of colistin treatment (1.98+3.62) versus the previous year (3.40 + 4.21, p < 0.001). We conclude that treatment with inhaled colistin in patients with NCFB and P. aeruginosa in sputum can achieve high rates of eradication even in patients with several previous positive cultures, as well as a significant decrease of exacerbations and hospital admissions.

Background The appropriate identification of asthma phenotypes of responders to omalizumab would optimize the selection of treatment. Objective To describe the most frequent clinical phenotypes in patients with severe asthma responding to omalizumab and their clinical and pulmonary function improvement. Methods This was an observational, retrospective, multicenter study. Adult patients with severe asthma, who achieved good control after the first year of treatment with omalizumab were included. Omalizumab was prescribed according to clinical routine practice. Responders were assigned to one pre-established phenotype based on the most predominant one before they had started treatment with omalizumab, all according to the physician's criteria. Data about asthma symptoms, number of non-severe asthma exacerbations, medication intake (inhaled and oral corticosteroids and rescue medication), lung function, high fractional exhaled nitric oxide (FeNO) and peripheral eosinophils counts were recorded. Results Among the 345 patients included, the main phenotypes were severe asthma with frequent exacerbations (29.9%), early-onset allergic asthma (23.8%), severe steroid-dependent asthma (18.8%), and severe eosinophilic asthma (13.6%). Clinical and respiratory changes observed after first year of treatment with omalizumab included: reduction in asthma symptoms, reduction in the use and dose of corticosteroids and need for rescue therapy, improvement of pulmonary function, reduction in the number of episodes of non-severe asthma exacerbations regardless of the duration of severe disease since the diagnosis. Increased blood levels of peripheral eosinophils and high FeNO levels were found at baseline. Conclusion Several heterogeneous severe asthma phenotypes were observed as good responders to omalizumab.

Resumen: El asma es una de las enfermedades más prevalentes en España y, a pesar de los grandes avances diagnósticos y terapéuticos, los resultados en salud indican que hasta el 80% de los pacientes no tendrían su asma bien controlada. Aunque las causas de esta situación son diversas, existen algunas prácticas entre los profesionales que atienden el asma que no contribuyen a la mejora de los resultados en salud de esta enfermedad. Nuestro grupo de trabajo ha elaborado este documento para recordar una serie de pautas básicas, alineadas con las guías actuales y la literatura reciente, y consensuadas mediante metodología Delphi por un panel experto multidisciplinar. Estas recomendaciones sobre qué no se debe hacer en el manejo del asma en el adulto quedan articuladas en 4 ámbitos: el diagnóstico, el seguimiento, el tratamiento y la prevención de exacerbaciones. Abstract: Asthma is one of the most prevalent diseases in Spain, yet despite the great diagnostic and therapeutic advances made in this field, health outcomes suggest that up to 80% of patients do not have their asthma well controlled. Although the causes of this situation are diverse, there are some practices among the professionals who treat asthma that are not conducive to improving health outcomes in this disease. Our working group has prepared this document in order to remind clinicians of a series of basic guidelines, aligned with current guidelines and recent literature, and agreed by a multidisciplinary expert panel using Delphi methodology. These recommendations about what not to do in the management of asthma in the adult patient are structured into four areas: diagnosis, monitoring, treatment and prevention of exacerbations.

Resumen: A pesar de los avances terapéuticos disponibles actualmente, el grado de control del asma es escaso. Dicho control se basa en evaluar al paciente, ajustar el tratamiento y revisar la respuesta al mismo. En situaciones normales, el seguimiento y el control del asma se realizan mediante visitas presenciales secuenciales. Sin embargo, debido a las medidas de bioseguridad y distanciamiento para evitar la transmisión de la enfermedad durante una pandemia, ese seguimiento y control se ven limitados. Así es como ha surgido la teleasistencia, la cual dispone de una amplia evidencia publicada en asma. Aun así, no ha de entenderse como una forma de sustituir a las consultas presenciales, sino como una alternativa complementaria a las mismas, en las que se permite el seguimiento de los pacientes cuando no sea necesario o no se pueda realizar una consulta presencial. A través del proyecto COntrol como Meta en la Era de la Telemedicina en el Asma (COMETA), un grupo de expertos abordó en profundidad la enfermedad asmática, analizando de forma detallada los problemas existentes para poder alcanzar el control, y proponer soluciones ante situaciones como las que estamos viviendo actualmente con la pandemia de la COVID-19. Abstract: Despite the therapeutic advances currently available, asthma control is poor. Such control is based on assessing the patient, adjusting treatment, and reviewing the response to treatment. In normal situations, asthma is monitored and controlled by sequential face-to-face visits. However, due to biosecurity and distancing measures to avoid disease transmission during a pandemic, such monitoring and control is limited. This is how tele-assistance, which is available from extensive published evidence in asthma, has emerged. Even so, it should not be understood as a substitute for face-to-face consultations, but as a complementary alternative to them, in which patients can be monitored when a face-to-face consultation is not necessary or cannot be carried out. Through the COMETA project (COntrol como Meta en la Era de la Telemedicina en el Asma, Control as a Goal in the Age of Telemedicine in Asthma), a group of experts addressed in depth the asthmatic pathology, analyzing in detail the existing problems in order to achieve control and propose solutions to situations such as those we are currently experiencing with the COVID-19 pandemic.

Resumen: La Sociedad Española de Neumología y Cirugía Torácica (SEPAR) ha elaborado este documento de recomendaciones sobre la vacuna para la COVID-19 en las enfermedades respiratorias, con el objetivo de ayudar al personal sanitario en la toma de decisiones sobre cómo actuar en la vacunación de estos pacientes.Las recomendaciones han sido elaboradas por un grupo de expertos en la materia, tras la revisión de la literatura recopilada hasta el 7 de marzo del 2021, y de la información aportada por distintas sociedades científicas, agencias del medicamento y estrategias de organismos gubernamentales hasta esa fecha.Podemos concluir que las vacunas para la COVID-19 no solo son seguras y eficaces, sino que, en aquellos pacientes vulnerables con enfermedades respiratorias crónicas, son prioritarias. Además, la implicación activa de los profesionales sanitarios que manejan estas patologías en la estrategia de vacunación es clave para lograr una buena adherencia y coberturas vacunales elevadas. Abstract: The Spanish Society of Pneumonology and Thoracic Surgery (SEPAR) has elaborated this document of recommendations for COVID-19 vaccination in patients with respiratory diseases aimed to help healthcare personnel make decisions about how to act in case of COVID-19 vaccination in these patients.The recommendations have been developed by a group of experts in this field after reviewing the materials published up to March 7, 2021, the information provided by different scientific societies, drug agencies and the strategies of the governmental bodies up to this date.We can conclude that COVID-19 vaccines are not only safe and effective, but also prior in vulnerable patients with chronic respiratory diseases. In addition, an active involvement of healthcare professionals, who manage these diseases, in the vaccination strategy is the key to achieve good adherence and high vaccination coverage.

BACKGROUND: The minimum controlling dose of treatment must be established in patients with asthma, but the outcome of step-down is unpredictable. OBJECTIVE: To identify factors associated with risk of control loss when stepping down asthma treatment and to develop a score to predict this risk. METHODS: A prospective, multicenter study including adults with well-controlled asthma was performed. Treatment was stepped up or stepped down over a 12-month period to maintain asthma control. We determined associations between clinical and functional variables and step-down failure. Finally, we derived a score to predict loss of control in 1 cohort and validated it in an independent cohort. RESULTS: The derivation cohort consisted of 228 patients; 218 completed at least 1 step-down episode and a total of 495 step-down episodes were evaluated. A medical-record documented postbronchodilator spirometry result of = 1 severe exacerbation in the previous 12 months (OR = 2.43; 95% CI: 1.48-4.01), and Asthma Control Test score < 25 (OR = 2.30; 95% CI: 1.35-3.92) were independently associated with failure. The score showed an area under the curve of 0.690 (95% CI: 0.633-0.747; P < .05) in the derivation cohort and 0.76 (95% CI: 0.643-0.882; P < .001) in a validation cohort of 114 patients. A score 8 implies a high risk (>40%). CONCLUSION: This score can facilitate the prediction of step-down failure before medication taper in patients with well-controlled asthma. (C) 2018 American Academy of Allergy, Asthma & Immunology

Fibromyalgia can affect the control of asthma when both diseases are present in a single patient.To characterize asthma in patients with concomitant fibromyalgia to assess whether fibromyalgia is an independent factor of asthma severity that influences poor asthma control. We also evaluated how dyspnea is perceived by patients in order to demonstrate that alterations in the perception of airway obstruction may be responsible for poor asthma control.This was a cross-sectional case-control multicenter study, in which 56 patients in the asthma and fibromyalgia group were matched to 36 asthmatics by sex, approximate age, and asthma severity level. All patients were women. Study variables included the Asthma Control Test (ACT), the Mini Asthma Quality of Life Questionnaire (MiniAQLQ), the Nijmegen hyperventilation syndrome questionnaire, the Hospital Anxiety and Depression Scale, and perception of dyspnea after acute bronchoconstriction.Although patients in both study groups showed similar asthma severity and use of anti-asthmatic drugs, patients in the asthma and fibromyalgia group showed lower scores on the ACT and MiniAQLQ questionnaires, and higher scores of anxiety and depression as well as hyperventilation compared to asthma patients without fibromyalgia. All these differences were statistically significant.Fibromyalgia in patients with asthma influences poor control of the respiratory disease and is associated with altered perception of dyspnea, hyperventilation syndrome, high prevalence of depression and anxiety, and impaired quality of life.Fibromyalgia may be considered a risk factor for uncontrolled asthma in patients suffering from asthma and fibromyalgia concomitantly.

Summary Introduction Aerosolized tobramycin is a standard of care for chronic Pseudomonas aeruginosa (Pa) infection in patients with cystic fibrosis (CF). Objectives The long-term safety and efficacy of intermittent (28-day “on”/“off” cycles) inhaled tobramycin nebulization solution 300 mg/4 ml (TNS4, Bramitob®/Bethkis®) was assessed over 56 weeks in CF patients aged ≥6 years having baseline 1 sec forced expiratory volume (FEV1) 40–80% predicted. Methods Patients were initially randomized in an 8-week open-label trial (core phase) to compare TNS4 (N = 159) and tobramycin 300 mg/5 ml (TNS5, TOBI®) (N = 165). A subset of patients continued in a 48-week, single-arm extension receiving TNS4 only. The primary endpoint of the core phase was to demonstrate the non-inferiority of TNS4 compared to TNS5 in terms of absolute change from baseline to week 4 in FEV1 % predicted. The assessment of long-term safety was the primary purpose of the extension phase. Throughout all phases of the study, microbiological assessments, adverse events, and audiometry findings were also evaluated. Results In the core phase (N = 321), FEV1 (% predicted) increased from baseline (absolute change) following a single on-treatment cycle for both TNS4 (7.0%) and TNS5 (7.5%) and the non-inferiority between treatments was met [difference between treatments of −0.5 (95% CI: −2.6; 1.6)]. These improvements were maintained throughout the extension phase (N = 209), ranging throughout the study between 5.1% (95% CI: 3.2; 6.9) and 8.1% (95% CI: 6.8; 9.4) compared to baseline. Pa sputum count reductions ranged between 0.6 (95% CI: 0.2; 0.9) to 2.3 (95% CI: 2.0; 2.6) log10 CFU/g throughout the 56 weeks. No remarkable safety issues were identified throughout both study phases, with similar percentages of patients reporting adverse events in the two treatment groups during the 8-week core phase [TNS4 (31.4%); TNS5 (28.0%)]. Conclusions Overall, TNS4 demonstrated short-term clinical benefits similar to TNS5 which were maintained during the long-term use of TNS4 and was also associated with a favorable tolerability profile. Pediatr Pulmonol. 2014; 49:1076–1089. © 2014 Wiley Periodicals, Inc.

[Introduction] It is not known whether clinical practice guidelines for the treatment of COPD exacerbations with short coursesofsystemic corticosteroids(SC-SCS) are followed in clinical practice. [Method] Prospective, observational cohort study in patients admitted due to severe COPD exacerbation. The primary endpoint was the percentage of patients who received SC-SCS as treatment for severe exacerbation (doses of 200-300 mg for 5-6 days). Secondary variables were percentageof patients with duration or reduced dose, dose in the first 24 hours, days of intravenous systemic corticosteroids (SCS), and duration of hospital length of stay (LOS). Simple linear regression was performed with LOS as a dependent variable and multivariate analysis with factors associated with LOS. [Results] 158 patients were evaluated. 4.4% (7) patients received SC-SCS, 8.7% received a reduced dose and duration was reduced in 15.8%. The median dose and duration of SCS were 602.5 mg (200-1625) and 14 (4-36) days, respectively. We observed an association between days of SCSand LOS (P < .001) and doses of intrahospitalSCSand LOS (P < .001). Factors associated with LOS were doses of intrahospitalSCS received (.01 [95% CI:.007-.013]; P < 0.001), days of steroid treatment (.14 [95% CI .03-.25], P = .009) and PAFI (pO2/FiO2 ratio) at admission (-.012 [95% CI: -.012 to -.002], P = .015). [Conclusions] The SCS schedules used in routine clinical practice are longer and administered at a higher dose than recommended, leading toa longer hospital stay. [Introducción] Se desconoce si en la práctica clínica habitual se siguen las recomendaciones de las guías de práctica clínica con respecto al tratamiento de las exacerbaciones de la EPOC con pautas cortas (PC) de corticoesteroides sistémicos (CS). [Método] Estudio de cohortes, prospectivo y observacional en pacientes que ingresan por una agudización grave de su EPOC. La variable principal fue porcentaje de pacientes que recibían PC de CS como tratamiento en la exacerbación grave (dosis acumulada total de 200 a 300 mg y una duración de 5-6 días). Las variables secundarias fueron porcentaje de pacientes con duración o dosis corta, dosis en las primeras 24 horas, días de CS intravenosos y duración de la estancia hospitalaria (EH). Se realizó regresión lineal simple con días de estancia hospitalaria como variable dependiente y análisis multivariante con factores asociados a estancia hospitalaria. [Resultados] Se evaluaron 158 pacientes; 4,4% (7) pacientes recibieron una PC de CS. El 8,7% recibió un tratamiento corto y el 15,8% una duración reducida. La mediana de dosis y duración de CS fue 602,5 mg (rango intercuartílico: 430-850) y 14 (rango intercuartílico: 4-36) días respectivamente. Observamos asociación entre más días de CS y una mayor EH (p < 0,001) y una mayor dosis de CS intrahospitalaria e incremento de EH (p < 0,001).Los factores asociados con EH fueron dosis de CS intrahospitalaria recibida (0,01 [IC 95%: 0,007-0,013]; p < 0,001), días de tratamiento esteroideo (0,14 [IC 95%: 0,03-0,25]; p = 0,009) y PAFI (cociente pO2/FiO2) al ingreso (–0,012 [IC 95%: –0,012 a –0,002]; p = 0,015). [Conclusiones] Las pautas de CS empleadas en la práctica clínica habitual son más prolongadas y a una mayor dosis que las recomendadas, asociando una mayor estancia hospitalaria.

Introduction: There is limited data regarding the impact of telemonitoring over the outcomes in COPD patients after hospital discharge. Methods: Prospective, cohort study in a tertiary hospital between 7/1/2013- 1/8/2015.Inclusion:1)age > 40 ,2)smoker/former,3)FEV1/FVC Main outcome :% of patients readmitted at 30-day. Secondary outcomes :% patients readmitted at 90-day and time to readmission at 90-day. X 2 for categoric variables comparison among groups.Kaplan-Meier curves and log-rank for comparing time to readmission.Logistic regression and Cox proportional hazard ratio for multivariate analysis. Results: 181 were analysed (TMON=61 [33.7%]).Less patients from TMON were readmitted at 30-days:4(6.6%) vs.29(24.2%);OR=0.22 (IQR: 0.07-0.66;p=0.007).TMON patients had less % of readmission at 90-days (18% vs. 43.3%; p

To describe the psychometric properties of the Spanish version of the Airways Questionnaire 20 (AQ20S) for asthma and COPD separately. Two hundred and eight patients (108 asthma, 100 COPD) filled in the AQ20S, the St. George′s Respiratory Questionnaire (SGRQ), the Asthma Quality of Life Questionnaire (AQLQ) and the Chronic Respiratory Disease Questionnaire (CRQ). Clinical and physiological measurements were assessed concurrently. Clinically stable patients were asked to fill in again the AQ20S after 14 days. The same assessment as on baseline visit was repeated after 3 months. The AQ20S showed a satisfactory score distribution, with minimal floor and ceiling effects, high internal consistency (Cronbach’s α > 0.80), and good test–retest reliability (ICC ≥ 0.87). The AQ20S showed a high correlation with the SGRQ in both asthma and COPD (r s > 0.75; P

Resumen Introduccion y objetivos el uso habitual de cuestionarios de calidad de vida relacionada con la salud en enfermedades respiratorias cronicas esta limitado por el tiempo que requiere su cumplimentacion. El Airways Questionnaire 20 (AQ20) consta de 20 items con un formato de respuesta sencillo, idoneo para aplicar en la practica asistencial, pero aun no se dispone de una version para su uso en Espana. Asi pues, nuestro objetivo ha sido elaborar una version del AQ20 en lengua espanola equivalente al original ingles. Metodo para la adaptacion del cuestionario utilizamos el metodo de traduccion-retrotraduccion. Evaluamos la equivalencia de cada uno de los items con los de la version original clasificandolos como totalmente equivalentes (tipo A), con alguna expresion dudosa (tipo B) o de equivalencia dudosa (tipo C). Se volvieron a evaluar los items de tipo B y C mediante la discusion entre investigadores y traductores, con las aportaciones del autor del cuestionario y la consulta a un grupo de pacientes. Con la version final se realizo un estudio piloto en 30 pacientes. Resultados las puntuaciones de dificultad de traduccion y retrotraduccion fueron bajas (3,45 y 3,52, respectivamente). En cuanto a la equivalencia conceptual entre la version retrotraducida y el cuestionario original, el 65% de los items se califico de tipo A, el 15% de tipo B y el 20% de tipo C. Tras la discusion de los items B y C se elaboro la version espanola final del AQ20, que se aplico a 15 pacientes con asma (un 66% mujeres; edad media±desviacion estandar: 53,13±19,6 anos) y a otros 15 con enfermedad pulmonar obstructiva cronica (un 13% mujeres; edad media: 67,8±8,94 anos), y se obtuvo un alfa de Cronbach de 0,92. Conclusion el proceso de adaptacion ha permitido obtener un instrumento en espanol conceptualmente semejante al original ingles, de facil comprension y con una consistencia interna adecuada.

Objetivo Valorar la eficacia del tratamiento con sildenafilo en monoterapia en pacientes con hipertension pulmonar (HP). Pacientes y metodos Se ha realizado un estudio observacional de 11 pacientes con HP en clases funcionales II y III, a quienes se administro tratamiento con sildenafilo (150 mg/dia). Siete presentaban HP tromboembolica cronica y no quirurgica y 4, hipertension arterial pulmonar. Para valorar la respuesta analizamos a los 3, 6 y 12 meses los siguientes parametros: tolerancia al esfuerzo mediante el test de la marcha de 6 min, cambio de clase funcional y ecocardiograma para valorar la presion sistolica en la arteria pulmonar. Resultados Encontramos una mejoria significativa de la tolerancia al esfuerzo, con un incremento de la distancia caminada en 6 min, a los 3, 6 y 12 meses de tratamiento (+20, +67 y +95 m, respectivamente). Todos los pacientes mejoraron de clase funcional. El seguimiento ecocardiografico no mostro diferencias estadisticamente significativas entre los valores basal y a los 6 y 12 meses de tratamiento de la presion sistolica en la arteria pulmonar. No observamos efectos adversos significativos, aun cuando se retiro el tratamiento con sildenafilo en un paciente por cefalea persistente. Conclusiones Los hallazgos de este estudio confirman que el sildenafilo es un farmaco eficaz para el manejo de la hipertension arterial pulmonar y la HP tromboembolica cronica y no quirurgica, tanto a corto como a medio-largo plazo, con buena tolerancia y escasos efectos secundarios.

Introduccion y objetivos La hipertension arterial pulmonar tiene mal pronostico en pacientes adultos y pediatricos. El conocimiento actual de los mecanismos que condicionan la hipertension arterial pulmonar ha permitido descubrir medicamentos como los antagonistas de los receptores de la endotelina e inhibidores de la 5-fosfodiesterasa, administrables ambos por via oral y generalmente bien tolerados. En este estudio se valora la capacidad funcional y la tolerancia al ejercicio en el tratamiento a largo plazo con sildenafilo o bosentan en pacientes con hipertension arterial pulmonar idiopatica y sindrome de Eisenmenger, y se comparan los resultados en ambos grupos de pacientes. Metodos Siete pacientes fueron incluidos en el protocolo de estudio de la hipertension arterial pulmonar, y en ellos se confirmo el diagnostico de hipertension arterial pulmonar idiopatica. Se trato con sildenafilo a 5 pacientes y con bosentan a 2. Cinco pacientes con comunicacion interventricular no restrictiva e hipertension arterial pulmonar fueron tratados con sildenafilo. En uno de ellos se anadio bosentan al tratamiento con sildenafilo. Resultados El sildenafilo y el bosentan mejoraron significativamente la capacidad de ejercicio en pacientes con hipertension arterial pulmonar idiopatica. El efecto fue menor en los pacientes con fisiologia de sindrome de Eisenmenger. La mejoria en la clase funcional de la Organizacion Mundial de la Salud fue mayor en los pacientes con hipertension arterial pulmonar idiopatica, aunque fue significativa en ambos grupos. Conclusiones El tratamiento a largo plazo con sildenafilo y bosentan mejora la capacidad de ejercicio y la clase funcional en la hipertension arterial pulmonar idiopatica y debida a cardiopatias congenitas. Los cambios son mas llamativos en los pacientes con hipertension arterial pulmonar idiopatica.

Objetivo Realizamos el estudio con el proposito de conocer, a traves de un cuestionario especifico, el impacto de la enfermedad pulmonar obstructiva cronica (EPOC) sobre las actividades de la vida diaria en un grupo amplio de pacientes en Espana. Asimismo estudiamos su relacion con parametros clinicos, funcionales y socioeconomicos, y con escalas validadas de calidad de vida, para evaluar su utilidad en la practica habitual y determinar que variables pueden definir a los pacientes “fragiles” o que requieren una mayor atencion. Metodos Se ha realizado un estudio observacional, descriptivo, multicentrico y transversal, con la participacion de 227 neumologos de toda Espana, que incluyo a pacientes con el diagnostico de EPOC. A todos estos se les paso un cuestionario especifico con 7 preguntas que median el impacto de la enfermedad sobre aspectos de la vida diaria Las respuestas se valoraron de 0 a 2, segun el grado de afectacion, lo que supone un intervalo entre 0 y 14 unidades. Definimos al paciente como “fragil” si presentaba valores iguales o mayores de 9 en el cuestionario de impacto. Se evaluo la relacion entre la escala de impacto y variables clinicas, situacion socioeconomica, espirometria y calidad de vida medida con el cuestionario especifico St. George's Respiratory Questionnaire (SGRQ). Resultados Estudiamos a 1.057 pacientes (un 95,2% varones), con una edad media (± desviacion estandar) de 67 ± 9 anos y volumen espiratorio forzado en el primer segundo (FEV1), expresado en porcentaje, del 41,8 ± 13,3%. La puntuacion media del cuestionario de impacto fue de 6,3 ± 3,1. Las actividades con mayor afectacion fueron deporte y ocio, actividad fisica habitual y vida sexual (mucha afectacion en el 52,5, el 30,3 y el 20,2% de los pacientes, respectivamente). Encontramos relacion entre la puntuacion del cuestionario y parametros conocidos de gravedad (SGRQ, disnea, numero de agudizaciones y FEV1 expresado en litros). Los pacientes definidos como fragiles tenian mayor edad y menor nivel tanto cultural como economico. Conclusiones El cuestionario de impacto de la EPOC se correlaciona bien con los clasicos parametros clinicos y funcionales de valoracion de la gravedad de la enfermedad (disnea, FEV1 y exacerbaciones) y con el cuestionario de calidad de vida relacionada con la salud SGRQ. Asi pues, podria ser un instrumento util para identificar un perfil de paciente fragil, que presenta, ademas de una peor situacion clinica y funcional, una situacion socioeconomica mas desfavorecida.

Introduction and objectives. Pulmonary arterial hypertension carries a poor prognosis in both adult and pediatric patients. Current understanding of the mechanisms underlying pulmonary arterial hypertension has enabled the rapid development of appropriate drugs, such as endothelin receptor antagonists and 5-phosphodiesterase inhibitors, that can be administered orally and which are generally well tolerated. The aims of the present study were to evaluate functional class and exercise capacity following long-term treatment with sildenafil or bosentan in patients with idiopathic pulmonary arterial hypertension and Eisenmenger’s syndrome and to compare results in the 2 groups. Methods. Seven patients were included in the pulmonary arterial hypertension study, and diagnoses of idiopathic pulmonary arterial hypertension were confirmed. Five patients were treated with sildenafil, while 2 received bosentan. The 5 patients with a non-restrictive ventricular septal defect and pulmonary arterial hypertension were treated with sildenafil. In 1 patient, bosentan was added to the sildenafil. Results. Both sildenafil and bosentan significantly improved exercise capacity in patients with idiopathic pulmonary arterial hypertension. The treatment effect was less in those with Eisenmenger physiology. Although the improvement in World Health Organization functional class was greater in patients with idiopathic pulmonary arterial hypertension, it was significant in both groups. Conclusions. Long-term treatment with sildenafil and bosentan improved both exercise capacity and functional class in patients with idiopathic pulmonary arterial hypertension and in those with hypertension due to congenital heart disease. The changes were more marked in patients with idiopathic pulmonary arterial hypertension.

The aim of this study was to assess the relationships between depressive symptoms and symptom-centered panic-fear (P-F), and reports of health-related quality of life (HRQOL), in persons with near-fatal asthma (NFA), controlling for age, gender, and pulmonary function. Forty persons with NFA were interviewed. Sociodemographic and clinical asthma-related information were collected. HRQOL was assessed by the SF-36 Health Questionnaire, depressive symptoms were assessed by the Beck Depression Inventory, and P-F was assessed by the P-F scale of the Asthma Symptom Checklist. Multiple regression analyses were carried out with the Physical Component Summary (PCS) and the Mental Component Summary (MCS) of the SF-36 as a dependent variable, and depressive symptoms, P-F, age, gender, and pulmonary function as independent variables. Results showed that P-F and age were associated with PCS, whereas depressive symptoms accounted for a marked percentage of the variance explained in MCS. Neither gender nor pulmonary function were related to HRQOL. In conclusion, depressive symptoms and P-F play an important role in how persons with NFA assess their HRQOL. These patient-centered variables are potentially modifiable and may offer new ways to intervene in order to improve HRQOL in persons with NFA.

Muscular training is the corner stone of pulmonary rehabilitation programs.To evaluate the effects of a muscular training program - carried out on chronic obstructive pulmonary disease (COPD) subjects with antecedents of moderate or severe exacerbation - on exercise tolerance, Health Related Quality of Life (HRQoL) and illness prognosis.A quasi-experimental study.University Hospital.Twenty-five subjects with COPD (Global Initiative for Chronic Obstructive Lung Disease (GOLD) degrees II, III and IV); with moderate or severe exacerbations and functional deterioration due to respiratory disability; with commitment and capacity to participate in the program. Subjects were selected by consecutive sampling.Subjects underwent 20 muscular training sessions consisting of 30 minutes of inspiratory muscle training, 15 minutes of warm-up protocol of upper limb exercises, 30 minutes of muscle training in ergometric cycle, 5 minutes of stretching protocol of lower limbs plus illness awareness. The main outcome measures were six minute walking test (6MWT), specific HRQoL questionnaires (St. Georges Respiratory Questionnaire (SGRQ), Chronic Respiratory Disease Questionnaire (CRDQ) and Airways Questionnaire 20 (AQ20)) and the BODE Index.All subjects improved significantly (P0.001) their HRQoL in the SGRQ, the CRDQ and the AQ20, and this was demonstrated in each one of the evaluated dimensions. A positive response in relation to exercise tolerance and illness prognosis was observed. Following the program subjects walked an average of 56 meters more (P0.001) and the BODE index was a mean of 1.5 less regarding the initial value (P0.001).A 20-session muscular training program contributes to an improvement in HRQoL, exercise tolerance and illness prognosis in COPD subjects with moderate or severe exacerbations.The intervention program could be easily implemented since it needs a minimum of human and technological resources.

Resumen La neuralgia amiotrofica es una neuropatia inflamatoria e idiopatica que se caracteriza por dolor neuropatico. Se describio por primera vez en 1948 como una afectacion solo del plexo braquial y se denomino sindrome de Parsonage-Turner. Aunque este sindrome es mas frecuente en el plexo braquial, puede afectar de forma concomitante o aislada al nervio frenico, y en esta circunstancia el diagnostico es muy dificil si no hay alta sospecha clinica. Presentamos el caso de un paciente con neuralgia amiotrofica cuya unica manifestacion fue la afectacion frenica izquierda, y destacamos la refractariedad del dolor a los analgesicos, asi como la persistencia de los sintomas y de la alteracion diafragmatica durante mas de 6 meses.

Amyotrophic neuralgia is an inflammatory and idiopathic neuropathy which is characterised by neuropathic pain. It was described for the first time in 1948 as condition that only affected the brachial plexus and was called Parsonage-Turner syndrome. Although this syndrome is more common in the brachial plexus, it can concomitantly, or in isolation affect the phrenic nerve, and in this case the diagnosis is very difficult if there is no high clinical suspicion. We present a case of a patient with amyotrophic neuralgia in which the only sign was left phrenic involvement, and we highlight the resistance of the pain to analgesics, as well as the persistence of the symptoms and diaphragm problems for over 6 months.

We evaluated a Spanish version of the Asthma Opinion Survey (AOS) in a sample of 90 adult Spanish asthma patients. The Spanish AOS showed acceptable internal consistency, and a factorial structure similar to that reported by others, i.e., vulnerability, perceived quality of care, and recognition and control, although we additional ly obtained a fourth factor, attitudes toward patient knowledge. Vulnerability was significantly higher among older patients, and among patients with lower educational level, lower income, and more severe asthma. Perceived quality of care, and recognition and control were positively correlated with frequency of attacks, and recognition and control was significantly higher in older patients. The factor attitudes toward patient knowledge was significantly higher in younger patients and in patients with relatively high income. These results support the cross-cultural validity of this questionnaire.

Aerosolized tobramycin is a standard of care for chronic Pseudomonas aeruginosa (Pa) infection in patients with cystic fibrosis (CF).The long-term safety and efficacy of intermittent (28-day "on"/"off" cycles) inhaled tobramycin nebulization solution 300 mg/4 ml (TNS4, Bramitob(®)/Bethkis(®)) was assessed over 56 weeks in CF patients aged ≥6 years having baseline 1 sec forced expiratory volume (FEV(1)) 40-80% predicted.Patients were initially randomized in an 8-week open-label trial (core phase) to compare TNS4 (N = 159) and tobramycin 300 mg/5 ml (TNS5, TOBI(®)) (N = 165). A subset of patients continued in a 48-week, single-arm extension receiving TNS4 only. The primary endpoint of the core phase was to demonstrate the non-inferiority of TNS4 compared to TNS5 in terms of absolute change from baseline to week 4 in FEV(1) % predicted. The assessment of long-term safety was the primary purpose of the extension phase. Throughout all phases of the study, microbiological assessments, adverse events, and audiometry findings were also evaluated.In the core phase (N = 321), FEV(1) (% predicted) increased from baseline (absolute change) following a single on-treatment cycle for both TNS4 (7.0%) and TNS5 (7.5%) and the non-inferiority between treatments was met [difference between treatments of -0.5 (95% CI: -2.6; 1.6)]. These improvements were maintained throughout the extension phase (N = 209), ranging throughout the study between 5.1% (95% CI: 3.2; 6.9) and 8.1% (95% CI: 6.8; 9.4) compared to baseline. Pa sputum count reductions ranged between 0.6 (95% CI: 0.2; 0.9) to 2.3 (95% CI: 2.0; 2.6) log10 CFU/g throughout the 56 weeks. No remarkable safety issues were identified throughout both study phases, with similar percentages of patients reporting adverse events in the two treatment groups during the 8-week core phase [TNS4 (31.4%); TNS5 (28.0%)].Overall, TNS4 demonstrated short-term clinical benefits similar to TNS5 which were maintained during the long-term use of TNS4 and was also associated with a favorable tolerability profile.

Background Several studies that have analyzed differences in psychological and self-management variables between patients with a near-fatal asthma (NFA) attack and asthmatics without a NFA attack (non-NFA) have shown conflicting results, probably due to the heterogeneity of the events studied and the selection of comparison groups. Objective To determine whether NFA patients, in stable situation, have greater psychological morbidity and worse self-management behavior than non-NFA patients with similar sociodemographic and clinical characteristics. Methods A sample of 44 NFA patients (mean=5.65 years after the NFA episode) and 44 non-NFA patients matched for age, sex, and asthma severity was assessed. All patients were in clinical stable situation. Information about sociodemographic, clinical, functional, and morbidity variables was collected for each patient, and the Cognitive Depression Inventory, the Trait-Anxiety Scale, the Toronto Alexithymia Scale, the Practical Knowledge of Self-management questionnaire, and the Medication Adherence scale were administered. Results In comparison with non-NFA patients, NFA patients showed higher levels of trait-anxiety (23.84 vs. 16.86; P =.001) and more difficulties describing and communicating feelings (11.36 vs. 8.90; P =.002). NFA and non-NFA patients did not differ in self-management variables. After adjustment in multivariate logistic regression analysis for age, sex, and asthma severity, significant differences were observed between NFA and control group patients in marital status [odds ratio (OR)=0.26; P =.017; 95% confidence interval (CI)=0.09–0.78], prescribed dose of inhaled corticoids (OR=4.48; P =.006;95% CI=1.53–13.09), and trait-anxiety (OR=1.071; P =.025;95%CI=1.01–1.14). Conclusions NFA patients show higher psychological morbidity than non-NFA, even years after the NFA episode.

Background and Objective The routine use of health-related quality-of-life questionnaires in patients with chronic respiratory disease is limited due to the time required to complete them. The Airways Questionnaire 20 (AQ20) contains 20 easy-to-answer questions, making it ideal for use in routine practice. However, a Spanish version is not available. Our aim was to create a version of the AQ20 for use in Spain that would be equivalent to the original English questionnaire. Method The original questionnaire was adapted using the translation-backtranslation method. We evaluated the conceptual equivalence of the translation to the original questionnaire, classifying items as type A if they were fully equivalent, type B if they contained questionable wording, and type C if there were doubts about their equivalence. The items in categories B and C were re-examined by the researchers and translators, with input from the author of the original questionnaire and a group of patients. The final version was piloted among 30 patients. Results Low difficulty ratings were given to both the translation (3.45) and backtranslation (3.52). Sixty-five percent of the backtranslated items were considered to be fully equivalent to the original English items (type A), 15% were classified as type B, and 20% as type C. The final Spanish version, produced after discussion of items B and C, was administered to 15 patients with asthma (66% women; mean [SD] age, 53.13 [19.6] y) and 15 patients with chronic obstructive pulmonary disease (13% women; mean age, 67.8 [8.94] y). A Cronbach α of 0.92 was obtained. Conclusion The adaptation process produced a Spanish questionnaire that is conceptually equivalent to the English original, easy to understand, and satisfactory in terms of internal consistency.

Objective To assess the efficacy of treatment with sildenafil monotherapy in patients with pulmonary hypertension. Patients and methods An observational study was undertaken in 11 patients with pulmonary hypertension in functional class II or III who received treatment with sildenafil (150 mg/day). Seven of the patients had inoperable chronic thromboembolic pulmonary hypertension and 4 had pulmonary arterial hypertension. To assess treatment response, the following parameters were assessed during follow-up at 3, 6, and 12 months: exercise tolerance in the 6-minute walk test, change in functional class, and systolic pulmonary arterial pressure measured by echocardiography. Results We observed a significant improvement in exercise tolerance, as shown by increased 6-minute walk distance after 3, 6, and 12 months of treatment (increases of 20, 67, and 95 m, respectively). All patients showed an improvement in functional class. The results of echocardiography did not reveal statistically significant differences in systolic pulmonary arterial pressure between baseline and 6 or 12 months of treatment. No significant adverse effects were observed, although sildenafil treatment was suspended in 1 patient due to persistent headache. Conclusions The results of this study confirm that sildenafil is an effective drug for the management of pulmonary arterial hypertension and inoperable chronic thromboembolic pulmonary hypertension both in the short term and medium to long term, and that the drug is well tolerated and shows few side effects.

Oral corticosteroids are the most effective drugs for illness management, especially in near-fatal asthma (NFA) patients. Prescription of oral corticosteroids in other groups of asthmatic patients has been shown to be related not only to clinical features of the illness, but also to psychological variables, what suggests the need to investigate this relationship specifically in NFA patients. Forty-two NFA patients aged 18--83 were interviewed to collect data on sociodemographics, clinical features and psychological variables, specifically panic-fear, anxiety and depression; 45.2% had been prescribed oral corticosteroids and 54.8% had not been prescribed this type of medication. Only frequency and severity of symptoms (odds ratio=3.14; 95% confidence interval=1.27-7.79; p=0.013) and state-anxiety (odds ratio=1.03; 95% confidence interval=1.03-1.29; p=0.015) emerged as significant risk indicators for the prescription of oral corticosteroids. These preliminary results in NFA patients confirm previous reports about the relationship between prescription of oral corticosteroids and psychological variables in asthmatic patients. State-anxiety could increase the magnitude of breathing difficulties, and thus imply the need for more effective medications for its management, but further research is necessary to reject other possible explanations.

We describe a 41-year-old patient with adult-onset dermatomyositis who developed persistent pneumomediastinum and severe subcutaneous emphysema due to end-stage interstitial lung disease. The diagnosis of dermatomyositis was based on proximal muscle weakness, electromyographic findings of inflammatory myopathy, and positive findings on muscle biopsy. Low levels of creatine kinase elevation were found at the time of diagnosis (a form of dermatomyositis which has been associated with a poor prognosis). The patient had no signs of cutaneous vasculitis. Despite treatment with prednisone and azathioprine, she died of intercurrent gram-negative sepsis 15 months after the diagnosis of dermatomyositis.

Background There is some evidence that quality of life measured by long disease-specific questionnaires may predict exacerbations in asthma and COPD, however brief quality of life tools, such as the Airways Questionnaire 20 (AQ20) or the Clinical COPD Questionnaire (CCQ), have not yet been evaluated as predictors of hospital exacerbations. Objectives To determine the ability of brief specific health-related quality of life (HRQoL) questionnaires (AQ20 and CCQ) to predict emergency department visits (ED) and hospitalizations in patients with asthma and COPD, and to compare them to longer disease-specific questionnaires, such as the St George´s Respiratory Questionnaire (SGRQ), the Chronic Respiratory Disease Questionnaire (CRQ) and the Asthma Quality of Life Questionnaire (AQLQ). Methods We conducted a two-year prospective cohort study of 208 adult patients (108 asthma, 100 COPD). Baseline sociodemographic, clinical, functional and psychological variables were assessed. All patients completed the AQ20 and the SGRQ. COPD patients also completed the CCQ and the CRQ, while asthmatic patients completed the AQLQ. We registered all exacerbations that required ED or hospitalizations in the follow-up period. Differences between groups (zero ED visits or hospitalizations versus ≥ 1 ED visits or hospitalizations) were tested with Pearson´s X2 or Fisher´s exact test for categorical variables, ANOVA for normally distributed continuous variables, and Mann–Whitney U test for non-normally distributed variables. Logistic regression analyses were performed to estimate the predictive ability of each HRQoL questionnaire. Results In the first year of follow-up, the AQ20 scores predicted both ED visits (OR: 1.19; p = .004; AUC 0.723) and hospitalizations (OR: 1.21; p = .04; AUC 0.759) for asthma patients, and the CCQ emerged as independent predictor of ED visits in COPD patients (OR: 1.06; p = .036; AUC 0.651), after adjusting for sociodemographic, clinical, and psychological variables. Among the longer disease-specific questionnaires, only the AQLQ emerged as predictor of ED visits in asthma patients (OR: 0.9; p = .002; AUC 0.727). In the second year of follow-up, none of HRQoL questionnaires predicted exacerbations. Conclusions AQ20 predicts exacerbations in asthma and CCQ predicts ED visits in COPD in the first year of follow-up. Their predictive ability is similar to or even higher than that of longer disease-specific questionnaires SI