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Monoclonal antibodies (mABs) have become available to treat more efficiently patients with severe uncontrolled asthma (SUA). However, the use of mABs is lower than expected given the prevalence of SUA, with significant disparities in the use of these treatments.To evaluate the proportion of patients with SUA treated with mABs in Spain, and to analyze some of the factors that could determine these prescription patterns.An analysis was performed on the data provided from the Hospitals National Health System (NHS) 2018 catalogue where Chest Diseases Department and a Hospital Pharmacy were available. Random sampling was performed to determine the sample size, stratifying proportionally by geographic area and hospital level. Characteristics of the participating sites, as well as the prescribing of mABs were collected, which included geographic area, hospital levels, prescribing medical specialities, types of clinics, and mABs prescribed.Data from 90 hospitals were analyzed (Response rate 64.3%). Level 4 hospitals, the Canary Islands geographical area, and the presence of a high complexity Asthma Healthcare Unit (ACU) were associated with a higher probability that the SUA was treated with mABs.The map of the prescribing of mABs for SUA in Spain shows a significant variation by geographic area, hospital level, type of clinic, and the accreditation level of the ACUs. At the current time, there appears to be significant under-prescribing of these treatments.

OBJECTIVE: To visualise spatial data on chronic obstructive pulmonary disease (COPD) prevalence in Africa, Asia and Australasia using a Geographic Information System (GIS) inverse distance weighted (IDW) interpolation technique.DESIGN: Prevalence rates from population surveys on individuals aged ≥40, with spirometry-confirmed COPD, were searched systematically. The prevalence observed in 59 selected surveys and the geographic coordinates of the places where they were conducted informed a GIS computer programme. The prevalence was represented by an ascending chromatic scale (blue-green-yellow-orange-brown-red) in the GIS maps.RESULTS: IDW-interpolation GIS maps were obtained of all the geographic areas investigated, and even from regions lacking data. Areas of high/very high prevalence were found in: Southern Africa and in most of the Central and Eastern Africa regions; in practically all of Central Asia; in the western regions of Southern Asia; in the southern regions of the East European Plain and the West Siberian Plain of Northern Asia; and in the Malay Archipelago. Intermediate prevalence predominated in Oceania and in most of the other regions of Africa and Asia.CONCLUSION: Despite some biases inherent to the interpolation method used in the present study, our approach provided an understandable visual perspective of the COPD prevalence distribution in these geographic regions.

Background: Patients with mild asthma represent a substantial proportion of the population with asthma, yet there are limited data on their true burden of disease. We aimed to describe the clinical and healthcare resource utilisation (HCRU) burden of physician-assessed mild asthma. Methods: Patients with mild asthma were included from the NOVEL observational longiTudinal studY (NOVELTY; NCT02760329), a global, 3-year, real-world prospective study of patients with asthma and/or chronic obstructive pulmonary disease from community practice (specialised and primary care). Diagnosis and severity were based on physician discretion. Clinical burden included physician-reported exacerbations and patient-reported measures. HCRU included inpatient and outpatient visits. Results: Overall, 2004 patients with mild asthma were included; 22.8% experienced >= 1 exacerbation in the previous 12 months, of whom 72.3% experienced >= 1 severe exacerbation. Of 625 exacerbations reported, 48.0% lasted >1 week, 27.7% were preceded by symptomatic worsening lasting >3 days, and 50.1% required oral corticosteroid treatment. Health status was moderately impacted (St George's Respiratory Questionnaire score: 23.5 [standard deviation +/- 17.9]). At baseline, 29.7% of patients had asthma symptoms that were not well controlled or very poorly controlled (Asthma Control Test score = 2 exacerbations in the previous year. In terms of HCRU, at least one unscheduled ambulatory visit for exacerbations was required by 9.5% of patients, including 9.2% requiring >= 1 emergency department visit and 1.1% requiring >= 1 hospital admission. Conclusions: In this global sample representing community practice, a significant proportion of patients with physician-assessed mild asthma had considerable clinical burden and HCRU.

Background Omalizumab is a fully humanized monoclonal antibody indicated as add-on therapy to improve asthma control in patients with severe persistent allergic asthma. Aims The aim of this study was to evaluate social, healthcare expenditure and clinical outcomes changes after incorporating omalizumab into standard treatment in the control of severe asthma. Methods In this multicentre retrospective study, a total of 220 patients were included from 15 respiratory medicine departments in the regions of Andalusia and Extremadura (Spain). Effectiveness was calculated as a 3-point increase in the Asthma Control Test (ACT) and a reduction in the annual number of exacerbations. The economic evaluation included both direct and indirect costs. Incremental cost-effectiveness ratio (ICER) was calculated. Results from the year before and the year after incorporation of omalizumab were compared. Results After adding omalizumab, improvement of lung function, asthma and rhinitis according to patient perception, as well as the number of exacerbations and asthma control measured by the ACT score were observed. Globally, both healthcare resources and pharmacological costs decreased after omalizumab treatment, excluding omalizumab cost. When only direct costs were considered, the ICER was €1712 (95% CI 1487–1995) per avoided exacerbation and €3859 (95% CI 3327–4418) for every 3-point increase in the ACT score. When both direct and indirect costs were considered, the ICER was €1607 (95% CI 1385–1885) for every avoided exacerbation and €3555 (95% CI 3012–4125) for every 3-point increase. Conclusions Omalizumab was shown to be an effective add-on therapy for patients with persistent severe asthma and allowed reducing key drivers of asthma-related costs.

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Alpha-1 antitrypsin deficiency (AATD) is a rare and underdiagnosed disease that is associated with the development of liver disease in adults and children and pulmonary emphysema in adults. Several studies have shown that there is limited knowledge about the disease and its diagnosis among health care providers, and there is an important inequity in the access to specialized care and appropriate treatment across Europe. The European Commision and the European Respiratory Society (ERS) recommend that the care of patients with AATD must be organized in reference centers at national or regional levels. These reference centers must provide optimal clinical care in terms of adequate diagnostic techniques, such as phenotyping and genotyping, and ensure access to treatment according to guidelines. Reference centers should also provide continuous medical education for health care professionals, genetic counseling, collaboration with patient associations and promote collaborative research and clinical trials with new and existing treatments for the disease. These centers must have a registry of their activity and collaborate with large, international, multicenter registries, such as the European Alpha-1 antitrypsin Deficiency Research Collaboration (EARCO) international registry, which is endorsed by the ERS, and aims to recruit up to 3,000 patients over a period of three years and prospectively follow them to better understand the natural history of the disease and the impact of different treatments on outcomes in a real life setting. International collaboration and standardized collection of high-quality prospective data will provide new insights into the clinical manifestations and prognosis of AATD., Alexa Núñez is the recipient of a Rio Hortega contract in the 2019 Strategic Action Health Call from the Instituto de Salud Carlos III for the years 2020-2022. Miriam Barrecheguren is the recipient of a Rio Hortega contract in the 2017 Strategic Action Health Call from the Instituto de Salud Carlos III for the years 2018-2019. The Spanish centers for Alpha-1 antitrypsin Deficiency are funded by unrestricted research grants from Grifols.

[EN]: [Introduction]: The objective of this analysis was the evaluation of a new national circuit used for diagnosing alpha1 antitrypsin deficiency (AATD) based on multiplex technology using online registration and mail posted samples from dried blood spots (DBS) and buccal swabs. [Methods]: This is an observational, ongoing study conducted in Spain since March 2018. Samples are coded on a web platform and sent by postal mail to the central laboratory. Allele-specific genotyping for the 14 most common mutations was done with the Luminex 200 Instrument System. Gene sequencing was done if none of the mutations were found and the AAT serum level was, [ES]: [Introducción]: El objetivo de este análisis fue la evaluación de un nuevo circuito nacional utilizado para diagnosticar la deficiencia de alfa-1 antitripsina (DAAT) basado en tecnología multiplex con muestras de manchas de sangre seca (DBS, por sus siglas en inglés) y frotis bucales enviados por correo postal tras un registro previo en línea. [Métodos]: Este es un estudio observacional en curso que se está llevando a cabo en España desde marzo de 2018. Las muestras se codifican en una plataforma web y se envían por correo postal al laboratorio central. El genotipado de un alelo específico buscando las 14 mutaciones más comunes se realizó con el sistema Luminex® 200. Se realizó secuenciación génica si no se encontraba ninguna de las mutaciones y el nivel sérico de AAT era < 60 mg/dl, o por solicitud del médico responsable. [Resultados]: En el momento del presente informe se habían procesado 5.803 (92,9%) muestras, 4.984 (85,9%) de frotis bucal y 819 (14,1%) de DBS. La prevalencia de las combinaciones frecuentes de alelos fue: MS 19,0%, MZ 14,4%, SS 2,9%, SZ 3,7% y ZZ 1,4%. Globalmente, los portadores de Z representaron el 20,0% y los portadores de S el 26,6% de esta población, observándose diferencias entre las regiones. Se identificaron 209 (3,6%) portadores de alelos raros, 12 (0,2%) portadores de alelos nulos y se describieron 14 (0,3%) nuevas mutaciones. Otras enfermedades respiratorias que no eran EPOC, incluyendo el asma mal controlado o las bronquiectasias, también presentaron mutaciones DAAT. [Conclusiones]: La disponibilidad de un sistema de diagnóstico con registro web basado en el análisis simultáneo de 14 variantes genéticas de frotis bucales o DBS enviados por correo postal ha demostrado ser útil, y el sistema puede mejorar el diagnóstico temprano de DAAT.

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.

The percentage of α1-antitrypsin protease inhibitor ZZ (PiZZ) genotypes in patients with COPD is controversial, with large differences among various studies. We aimed to estimate the prevalence of PiZZ in COPD patients from 20 European countries with available data, according to the number of PiZZ and COPD individuals in each country.A systematic review was conducted to select European countries with reliable data on the prevalence of PiZZ and COPD. We created a database with the following data: 1) total population and population aged ≥40 years according to the Eurostat database; 2) number and 95% CI of PiZZ patients aged ≥40 years; 3) application of a conversion factor of genetic penetrance of 60%; 4) number of COPD individuals, with 95% CI, aged ≥40 years; and 5) calculation of the PiZZ/COPD ratio. Finally, results were presented using an Inverse Distance Weighted Interpolation map.We found 36 298 (95% CI 23 643–56 594) PiZZ individuals at high risk and 30 849 709 (95% CI 21 411 293–40 344 496) COPD patients, with a PiZZ/COPD ratio of 0.12% (range 0.08–0.24%), and a prevalence of 1 out of 408 in Northern, 1 out of 944 in Western, 1 out of 1051 in Central, 1 out of 711 in Southern, and 1 out of 1274 in Eastern Europe.These data may be useful to plan strategies for future research and diagnosis, and to rationalise the available therapeutic resources.

The global prevalence of COPD is 13.1%, ranging from 11.6% in Oceania to 13.9% in Africa. With the aid of GIS technology, a global map of prevalence of COPD has been obtained including areas without real data.http://bit.ly/2HRWfK7

Managing adequately pressure drawdown should be a key technical reservoir management driver due to its major impact on cash flow, acceleration and final recovery factor for operating hydraulically fracture shale gas condensate producers. Permeability should be regarded as a key dynamic property for ultra-low permeability shale reservoirs that influences shale hydrocarbon recovery. It is paramount to develop a pressure depletion plan that captures the pressure drawdown strategy and the changes in flow capacity associated to the interaction of the nano-Darcy rock and hydraulic fractures with stress dependent permeability effects. Defining the adequate drawdown strategy would aid maximizing the economic recovery. Considering the variability of permeability with pressure drawdown should be part of the reservoir management lifecycle for unconventional shale reservoirs. This study focus on evaluating the impact of pressure drawdown strategy on initial rates and recovery for a Duvernay Gas condensate producer with an initial condensate yield of 100-150 stb/mmscf. A sector compositional reservoir simulation model was built for a horizontal multistage hydraulically fracture Duvernay shale gas condensate producer. A full assessment of variability of permeability in the nano-Darcy rock and in the propped hydraulic fracture stages near the wellbore region was accomplished. Aggressive, moderate and conservative pressure drawdown strategies were evaluated, considering multiple operational pressure drawdown incremental ranges from 14.5 to 95 psia per day. Results clearly indicate that implementing daily pressure drawdown increments of 22 to 29 psia per day would provide a similar recovery factor than imposing daily pressure drawdowns of 44 to 95 psia per day. However, there is a golden operating window opportunity to accelerate recovery by imposing maximum drawdown from the early days of production and bringing significant benefits of accelerating recovery with an associate increase in revenue but the benefits of this acceleration vanished in less than one year due to substantial changes in hydraulic fracture conductivity and also in the nano-Darcy rock permeability in the near wellbore region. The reduction of nano-Darcy permeability is a function of pressure, time and distance from the hydraulic fractures. According to our results, the best reservoir management practice for operating lean/medium Gas Condensate unconventional shale producers should be maximizing pressure drawdown at the early stage of the life cycle and deferring the installation of production string to maximize inflow-outflow.

Resumen Introduccion La asociacion entre la enfermedad pulmonar obstructiva cronica (EPOC) y la ansiedad o la depresion no se conoce adecuadamente, y puede haber diferencias entre distintos paises. Investigamos un modelo predictivo para esta asociacion en una poblacion espanola. Pacientes y metodo Estudio prospectivo descriptivo transversal incluyendo 204 pacientes con EPOC estable. Se diagnostica la presencia de ansiedad o depresion mediante valoracion psiquiatrica, aplicando los criterios diagnosticos de la 10.a revision del International Statistical Classification of Diseases and Related Health Problems (ICD-10). Se analizan variables sociodemograficas, clinicas y de funcion pulmonar. Resultados Un 36% de pacientes con EPOC estable tienen comorbilidad psiquiatrica, pero en el 76% de los casos se desconocia dicho diagnostico. Presentan un trastorno de ansiedad pura el 19%, depresion aislada el 9,8% y un trastorno mixto de ansiedad y depresion el 7,3% de los pacientes. En el analisis multivariante las variables predictoras son: edad mas joven, mayor nivel de estudios, falta de apoyo domiciliario, mayor indice de BODE y mayor numero de agudizaciones. La curva ROC del modelo muestra un AUC de 0,765 (p Conclusiones En la EPOC, una mayoria de pacientes con comorbilidad psiquiatrica no son identificados. Los trastornos de ansiedad son mas frecuentes que la depresion, en base a un diagnostico mediante entrevista estructurada. Los pacientes mas jovenes y con mayor nivel de estudios tienen mas riesgo de de padecer ansiedad o depresion. Otros factores predictivos son: un mayor indice BODE, mas agudizaciones y la falta de apoyo domiciliario.

Surveys estimating chronic obstructive pulmonary disease (COPD) prevalence are unevenly distributed in the Americas, which make it difficult to estimate accurately its geographical distribution. The geographic information system inverse distance weighted (IDW) interpolation technique has proved to be an effective tool in spatial distribution estimation of epidemiological variables, even when real data are few or widely spread. We aimed to represent cartographically the COPD prevalence in the Americas by means of a blue to red scale representation of the prevalence data, where different values are represented as different colours, and a population density filtered IDW interpolation mapping, where areas with a population density

Cristina Esquinas,1,2,* Sonia Serreri,3,* Miriam Barrecheguren,1 Esther Rodriguez,1 Alexa Nuñez,1 Francisco Casas-Maldonado,4 Ignacio Blanco,5 Pietro Pirina,3 Beatriz Lara,6 Marc Miravitlles1,7 1Pneumology Department, University Hospital Vall d’Hebron, Barcelona, Spain; 2Public Health, Mental, Maternal and Child Health Nursing Department, Faculty of Medicine and Health Sciences,University of Barcelona, Barcelona, Spain; 3Università di Sassari, Sassari, Italy; 4Pneumology Department, University Hospital San Cecilio, Granada, Spain; 5Alpha-1 Antitrypsin Deficiency Spanish Registry (REDAAT), Spanish Society of Pneumology (SEPAR), Barcelona, Spain; 6Coventry and Warwickshire University Hospital, Coventry, UK; 7CIBER de Enfermedades Respiratorias (CIBERES), Spain *These authors contributed equally tothis work Background: The clinical course of alpha-1 antitrypsin deficiency (AATD) is very heterogeneous. It is estimated that 60% of individuals with severe AATD (Pi*ZZ) develop emphysema. The main objective of this study was to describe the outcomes of long-term lung function in individuals with AATD-associated emphysema after at least 8 years of follow-up. Materials and methods: We performed a retrospective analysis of longitudinal follow-up data of AATD PiZZ patients from the Spanish registry (AATD Spanish Registry [REDAAT]). The main follow-up outcome was the annual rate of decline in forced expiratory volume in 1 second (FEV1) calculated using the FEV1 values at baseline and in the last post-bronchodilator spirometry available. Results: One hundred and twenty-two AATD PiZZ patients were analyzed. The median follow-up was 11years (interquartile range =9–14). The mean FEV1 decline was 28 mL/year (SD=54), with a median of 33mL/year. Tobacco consumption (β=19.8, p

Objectives: Comparison of adherence to nebulized sodium colistimethate (Promixin) and agreement between the multidimensional FACED, E-FACED and BSI scales in a cohort of patients with non-cystic fibrosis BQ and chronic bronchial infection (CBI) by PsA. Patients and Methods: A multicenter, observational, longitudinal prospective study of a cohort of adults with non-CF BQ, all with PsA CBI and treated with Promixin. FACED, EFACED and BSI scales in the previous year, their modification after a year of treatment with the nebulized therapy and the concordance between them are collected. Results: 99 patients were started average age 63±14.7 years. In the year prior to treatment FEV1 was 55±19%, exacerbations 3.8±2.6 and 0.5±0.7 hospital admissions/year. At the end FEV1 was 59±22%, exacerbations/year 1.4±1.2 and hospital admissions/year 0.26±0.6. In the pretreatment assessment there is a low concordance between the EFACED and BSI scales (R=0,248; p Conclusions: In our cohort there is good adherence to nebulized treatment with Promixin® and good nebulizer management, regardless of the severity of BQ. These results have their clinical impact in terms of functional improvement, reduction of exacerbations and changes in the level of severity prior to treatment. Given the concordance between the prognostic scales, both could be applicable to assess sensitivity to change in a therapeutic intervention with nebulized antibiotics.

Objective: To assess the clinical and economic impact, in terms of reduction of costs associated with exacerbations, in a cohort of patients with non-cystic fibrosis (non-CF) BQ and PsA CBI treated with Promixin. Patients and Methods: A multicenter, observational, longitudinal prospective study of a cohort of adults with non-CF BQ and PsA CBI treated with Promixin. Data were collected in the year before and after nebulized treatment (pulmonary function, exacerbations without hospital admission, mean per pat/y and total number of admissions and per pat/y). Results: 86 pat, 45 males, 62±15 y. Results are shown in the following data table. Conclusions: Adherence to nebulized treatment is excellent in our patients. In clinical terms, this situation has allowed an improvement in lung function and a significant reduction in the number and severity of exacerbations and hospital admissions. Regarding this issue, the average cost per patient and year of treatment has been reduced by more than €1500 after onset of nebulized therapy.

Ignacio Blanco,1 Patricia Bueno,2 Isidro Diego,3 Sergio Pérez-Holanda,4 Beatriz Lara,5 Francisco Casas-Maldonado,6 Cristina Esquinas,7 Marc Miravitlles7,8 1Alpha1-Antitrypsin Deficiency Spanish Registry (REDAAT), Lung Foundation Breathe, Spanish Society of Pneumology (SEPAR), Barcelona, Spain; 2Internal Medicine Department, County Hospital of Jarrio, Principality of Asturias, Spain; 3Materials and Energy Department, School of Mining Engineering, Oviedo University, Principality of Asturias, Spain; 4Surgical Department, University Central Hospital of Asturias, Oviedo, Spain; 5Respiratory Medicine Department, Coventry and Warwickshire University Hospital, Coventry, UK; 6Pneumology Department, University Hospital San Cecilio, Granada, Spain; 7Pneumology Department, Hospital Universitari Vall d’Hebron, Barcelona, Spain; 8CIBER de Enfermedades Respiratorias (CIBERES), Barcelona, Spain Abstract: The alpha-1 antitrypsin (AAT) haplotype Pi*S, when inherited along with the Pi*Z haplotype to form a Pi*SZ genotype, can be associated with pulmonary emphysema in regular smokers, and less frequently with liver disease, panniculitis, and systemic vasculitis in a small percentage of people, but this connection is less well established. Since the detection of cases can allow the application of preventive measures in patients and relatives with this congenital disorder, the objective of this study was to update the prevalence of the SZ genotype to achieve accurate estimates of the number of Pi*SZ subjects worldwide, based on studies performed according to the following criteria: 1) samples representative of the general population, 2) AAT phenotyping characterized by adequate methods, and 3) selection of studies with reliable results assessed with a coefficient of variation calculated from the sample size and 95% confidence intervals. Studies fulfilling these criteria were used to develop tables and maps with an inverse distance-weighted (IDW) interpolation method, to provide numerical and geographical information of the Pi*SZ distribution worldwide. A total of 262 cohorts from 71 countries were included in the analysis. With the data provided by these cohorts, a total of 1,490,816 Pi*SZ were estimated: 708,792 in Europe; 582,984 in America and Caribbean; 85,925 in Africa; 77,940 in Asia; and 35,176 in Australia and New Zealand. Remarkably, the IDW interpolation maps predicted the Pi*SZ prevalence throughout the entire world even in areas lacking real data. These results may be useful to plan strategies for future research, diagnosis, and management of affected individuals. Keywords: SERPINA1, alpha-1 antitrypsin deficiency, protease inhibitor, genetic epidemiology, SZ genotype, inverse distance-weighted interpolation, geographic information system

Jose Luis López-Campos,1,2 Maria Abad Arranz,1 Carmen Calero-Acuña,1,2 Fernando Romero-Valero,3 Ruth Ayerbe-García,4 Antonio Hidalgo-Molina,3 Ricardo I Aguilar-Pérez-Grovas,4 Francisco García-Gil,5 Francisco Casas-Maldonado,6 Laura Caballero-Ballesteros,5 María Sánchez-Palop,6 Dolores Pérez-Tejero,7 Alejandro Segado Soriano,7 Jose Calvo-Bonachera,8 Bárbara Hernández-Sierra,8 Adolfo Doménech,9 Macarena Arroyo-Varela,9 Francisco González-Vargas,10 Juan J Cruz-Rueda10 1Unidad Médico-Quirúrgica de Enfermedades Respiratorias, Instituto de Biomedicina de Sevilla (IBiS), Hospital Universitario Virgen del Rocío/Universidad de Sevilla, Seville, 2CIBER de Enfermedades Respiratorias (CIBERES), Instituto de Salud Carlos III, Madrid, 3Sección de Neumología, Hospital Puerta del Mar, Cádiz, 4Servicio de Neumología, Hospital Juan Ramón Jiménez, Huelva, 5Servicio de Neumología, Hospital Universitario Reina Sofía, Córdoba, 6Servicio de Neumología, Hospital Universitario San Cecilio, Granada, 7Sección de Neumología, Hospital Infanta Margarita, Cabra, Córdoba, 8Servicio de Neumología, Hospital Torrecárdenas, Almería, 9Servicio de Neumología, Hospital Regional Universitario de Málaga, Málaga, 10Servicio de Neumología, Hospital Universitario Virgen de las Nieves, Granada,Spain Objectives: Clinical practice in chronic obstructive pulmonary disease (COPD) can be influenced by weather variability throughout the year. To explore the hypothesis of seasonal variability in clinical practice, the present study analyzes the results of the 2013–2014 Andalusian COPD audit with regard to changes in clinical practice according to the different seasons.Methods: The Andalusian COPD audit was a pilot clinical project conducted from October 2013 to September 2014 in outpatient respiratory clinics of hospitals in Andalusia, Spain (8provinces with more than 8 million inhabitants) with retrospective data gathering. For the present analysis, astronomical seasons in the Northern Hemisphere were used as reference. Bivariate associations between the different COPD guidelines and the clinical practice changes over the seasons were explored by using binomial multivariate logistic regression analysis with age, sex, Charlson comorbidity index, type of hospital, and COPD severity by forced expiratory volume in 1 second as covariates, and were expressed as odds ratio (OR) with 95% confidence intervals (CIs).Results: The Andalusian COPD audit included 621 clinical records from 9 hospitals. After adjusting for covariates, only inhaler device satisfaction evaluation was found to significantly differ according to the seasons with an increase in winter (OR, 3.460; 95% CI, 1.469–8.151), spring (OR, 4.215; 95% CI, 1.814–9.793), and summer (OR, 3.371; 95% CI, 1.391–8.169) compared to that in autumn. The rest of the observed differences were not significant after adjusting for covariates. However, compliance with evaluating inhaler satisfaction was low.Conclusion: The various aspects of clinical practice for COPD care were found to be quite homogeneous throughout the year for the variables evaluated. Inhaler satisfaction evaluation, however, presented some significant variation during the year. Inhaler device satisfaction should be evaluated during all clinical visits throughout the year for improved COPD management. Keywords: COPD, seasons, clinical practice, quality of care

Ignacio Blanco,1 Patricia Bueno,2 Isidro Diego,3 Sergio Pérez-Holanda,4 Francisco Casas-Maldonado,5 Cristina Esquinas,6 Marc Miravitlles6,7 1Alpha1-Antitrypsin Deficiency Spanish Registry (REDAAT), Fundación Respira, Spanish Society of Pneumology and Thoracic Surgery (SEPAR), Barcelona, 2Internal Medicine Department, County Hospital of Jarrio, 3Materials and Energy Department, School of Mining Engineering, Oviedo University, 4Surgical Department, University Central Hospital of Asturias (HUCA), Oviedo, Principality of Asturias, 5Pneumology Department, Complejo Hospitalario Universitario de Granada, Granada, 6Pneumology Department, Hospital Universitari Vall d’Hebron, 7CIBER de Enfermedades Respiratorias (CIBERES), Barcelona, Spain Abstract: In alpha-1 antitrypsin deficiency (AATD), the Z allele is present in 98% of cases with severe disease, and knowledge of the frequency of this allele is essential from a public health perspective. However, there is a remarkable lack of epidemiological data on AATD worldwide, and many of the data currently used are outdated. Therefore, the objective of this study was to update the knowledge of the frequency of the Z allele to achieve accurate estimates of the prevalence and number of Pi*ZZ genotypes worldwide based on studies performed according to the following criteria: 1) samples representative of the general population, 2) AAT phenotyping characterized by adequate methods, and 3) measurements performed using a coefficient of variation calculated from the sample size and 95% confidence intervals. Studies fulfilling these criteria were used to develop maps with an inverse distance weighted (IDW)-interpolation method, providing numerical and graphical information of Pi*Z distribution worldwide. A total of 224 cohorts from 65 countries were included in the study. With the data provided by these cohorts, a total of 253,404 Pi*ZZ were estimated worldwide: 119,594 in Europe, 91,490 in America and Caribbean, 3,824 in Africa, 32,154 in Asia, 4,126 in Australia, and 2,216 in New Zealand. In addition, the IDW-interpolation maps predicted Pi*Z frequencies throughout the world even in some areas that lack real data. In conclusion, the inclusion of new well-designed studies and the exclusion of the low-quality ones have significantly improved the reliability of results, which may be useful to plan strategies for future research and diagnosis and to rationalize the therapeutic resources available. Keywords: SERPINA1, alpha-1 antitrypsin deficiency, protease inhibitor, genetic epidemiology, inverse distance weighted interpolation, geographic information system

Objective: to assess the role of comorbidities with all cause mortality in patients with stable COPD Material and Methods: prospective, multicenter, longitudinal study of patients with stable COPD. We recorded demographic characteristics, respiratory functional tests, medication received, comorbidities, Charlson comorbidity index and hospital anxiety and depression scale. Patients were followed up for 3 years. In case of death it was investigated to determine the real cause of death Results: 138 patients were studied with a mean age of 66.3±10.3 years and mean FEV1 of 51.3±16.9%. The mean Charlson index was 4.66 ± 1.57. 17.2% had depression and 12.7% anxiety. Thirteen (9.5%) patients died, 5 of lung cancer, 5 COPD exacerbation, 1 colon cancer, another for acute myocardial infarction (AMI) and another one for congestive heart failure (CHF). The bivariate analysis showed statistical differences in Charlson index, number of comorbidities, hypertension, AMI, CHF, treatment with noninvasive mechanical ventilation, antidepressants and anxiolytics. In the multivariate analysis the number of comorbidities (HR 1.926; 95%IC: 1.384-2.680) and anxiolytic treatment (HR 4.072; IC95%: 1.106-14.987) showed relationship with mortality. Kaplan-Meier survival plots showed that patients with 2 o more comorbilities, in addition to COPD, have higher mortality than patients with 1 or no comorbidity (35.52±0.2 vs 33±1.3 months, p=0,039). Conclusions: The prevalence of comorbidities in patients with stable COPD was high. Mortality in these patients is related to the number of comorbidities and anxiolytic treatment. Mortality was higher in patients with 2 o more comorbidities. Funded by NEUMOSUR.

[Objectives] Previous clinical audits of COPD have provided relevant information about medical intervention in exacerbation admissions. The present study aims to evaluate adherence to current guidelines in COPD through a clinical audit., [Methods] This is a pilot clinical audit performed in hospital outpatient respiratory clinics in Andalusia, Spain (eight provinces with more than 8 million inhabitants), including 9 centers (20% of the public centers in the area) between 2013 and 2014. Cases with an established diagnosis of COPD based on risk factors, clinical symptoms, and a post-bronchodilator FEV1/FVC ratio of less than 0.70 were deemed eligible. The performance of the outpatient clinics was benchmarked against three guidance documents available at the time of the audit. The appropriateness of the performance was categorized as excellent (>80%), good (60−80%), adequate (40−59%), inadequate (20−39%), and highly inadequate (, [Results] During the audit, 621 clinical records were audited. Adherence to the different guidelines presented a considerable variability among the different participating hospitals, with an excellent or good adherence for symptom recording, MRC or CAT use, smoking status evaluation, spirometry, or bronchodilation therapy. The most outstanding areas for improvement were the use of the BODE index, the monitoring of treatments, the determination of alpha1-antitrypsin, the performance of exercise testing, and vaccination recommendations., [Conclusions] The present study reflects the situation of clinical care for COPD patients in specialized secondary care outpatient clinics. Adherence to clinical guidelines shows considerable variability in outpatient clinics managing COPD patients, and some aspects of the clinical care can clearly be improved., This study was financially supported by an unrestricted grant from Laboratorios Menarini, SA (Barcelona, Spain).

Objectives: At the moment no studies have evaluated a clinical audit of clinical practice in chronic obstructive pulmonary disease (COPD) in the outpatient visits so far. This work is a first pilot project that aims to assess the variability of clinical practice for COPD in this care setting through a clinical audit. Methods: Pilot medical record audit conducted in specialised outpatient Pneumology clinic from 9 public hospitals in Andalusia (Spain) between October 2013 and September 2014. The project goal was to audit 80 cases per centre distributed over the year. The information was collected by standardized questionnaire with 182 variables. The data are described in means and inter-hospital ranges to evaluate between centres variability. Results: During study analysed 621 stories. Patients were mostly men, in the seventh decade of life, with a percentage of current smokers of 26.2%, a considerable number of comorbidities and mostly the urban area. The data indicate that health care is generally correct as average but indicating areas for improvement in some points and considerable variability between centres with significant differences (p Conclusions: Health care COPD patients in outpatient Pneumology clinics shows considerable variability that probably cannot be explained only by the patient9s clinical condition. Future studies should elucidate which factors influence this variability.

Introduction The association between chronic obstructive pulmonary disease (COPD) and anxiety and depression is not yet completely characterized, and differences between countries may exist. We used a predictive model to assess this association in a Spanish population. Patients and method Prospective transversal descriptive study of 204 patients with stable COPD. Concomitant anxiety or depression was diagnosed by psychiatric assessment, using the diagnostic criteria of the 10th revision of the International Statistical Classification of Diseases and Related Health Problems (ICD-10). Sociodemographic, clinical and lung function parameters were analyzed. Results In total, 36% of stable COPD patients had psychiatric comorbidities, but 76% were unaware of their diagnosis. Nineteen percent had a pure anxiety disorder, 9.8% had isolated depression, and 7.3% had a mixed anxiety-depression disorder. Predictive variables in the multivariate analysis were younger age, higher educational level, lack of home support, higher BODE index, and greater number of exacerbations. The ROC curve of the model had an AUC of 0.765 ( P Conclusions In COPD, concomitant psychiatric disorders are significantly associated with sociodemographic factors. Anxiety disorders are more common than depression. Patients with more severe COPD, according to BODE, younger patients and those with a higher educational level have a greater risk of being diagnosed with anxiety or depression in a structured psychiatric interview. In our population, most patients with psychiatric comorbidities remain unidentified.

Jose Luis López-Campos,1,2 María Abad Arranz,1 Carmen Calero Acuña,1,2 Fernando Romero Valero,3 Ruth Ayerbe García,4 Antonio Hidalgo Molina,3 Ricardo I Aguilar Perez-Grovas,4 Francisco García Gil,5 Francisco Casas Maldonado,6 Laura Caballero Ballesteros,5 María Sánchez Palop,6 Dolores Pérez-Tejero,7 Alejandro Segado,7 Jose Calvo Bonachera,8 Bárbara Hernández Sierra,8 Adolfo Doménech,9 Macarena Arroyo Varela,9 Francisco González Vargas,10 Juan J Cruz Rueda10 1Unidad Médico-Quirúrgica de Enfermedades Respiratorias, Instituto de Biomedicina de Sevilla (IBiS), Hospital Universitario Virgen del Rocío/Universidad de Sevilla, Seville, 2Centro de Investigación Biomédica en Red de Enfermedades Respiratorias (CIBERES), Instituto de Salud Carlos III, Madrid, 3Hospital Universitario Puerta del Mar, Cádiz, 4Hospital Juan Ramón Jiménez, Huelva, 5Hospital Universitario Reina Sofía, Córdoba, 6Hospital Universitario San Cecilio, Granada, 7Hospital Infanta Margarita, Cabra, Córdoba, 8Hospital Torrecárdenas, Almería, 9Hospital Regional Universitario de Málaga, 10Hospital Universitario Virgen de las Nieves, Granada, Spain Introduction: This study is an analysis of a pilot COPD clinical audit that evaluated adherence to guidelines for patients with COPD in a stable disease phase during a routine visit in specialized secondary care outpatient clinics in order to identify the variables associated with the decision to step-up or step-down pharmacological treatment.Methods: This study was a pilot clinical audit performed at hospital outpatient respiratory clinics in the region of Andalusia, Spain (eight provinces with over eight million inhabitants), in which 20% of centers in the area (catchment population 3,143,086 inhabitants) were invited to participate. Treatment changes were evaluated in terms of the number of prescribed medications and were classified as step-up, step-down, or no change. Three backward stepwise binominal multivariate logistic regression analyses were conducted to evaluate variables associated with stepping up, stepping down, and inhaled corticosteroids discontinuation.Results: The present analysis evaluated 565 clinical records (91%) of the complete audit. Of those records, 366 (64.8%) cases saw no change in pharmacological treatment, while 99patients (17.5%) had an increase in the number of drugs, 55 (9.7%) had a decrease in the number of drugs, and 45 (8.0%) noted a change to other medication for a similar therapeutic scheme. Exacerbations were the main factor in stepping up treatment, as were the symptoms themselves. In contrast, rather than symptoms, doctors used forced expiratory volume in 1second and previous treatment with long-term antibiotics or inhaled corticosteroids as the key determinants to stepping down treatment.Conclusion: The majority of doctors did not change the prescription. When changes were made, a number of related factors were noted. Future trials must evaluate whether these therapeutic changes impact clinically relevant outcomes at follow-up. Keywords: quality of care, outpatient care, treatment strategies, follow-up, respiratory diseases, airway diseases

Clinical manifestations of cystic fibrosis (CF) are variable. Genetic and environmental factors that determine whether an individual will develop associated complications are still under investigation. The present study reports the genetic analysis of a family with different clinical forms of CF and addresses the difficulty of CF diagnosis in an individual with mutant alleles G542X and R117H because of the variable phenotype associated with R117H mutation. Both children in this family were heterozygous for G542X/R117H with the same thymine sequence (7T/9T) in intron 8 of CF transmembrane conductance regulator. The girl was diagnosed with CF, whereas the boy was diagnosed with azoospermia as the sole clinical manifestation. The possible implication of the hemochromatosis gene as a CF modifier locus was analyzed because the 2 children had the same genotype. No genetic differences were detected between brother and sister that explained the different clinical manifestations of CF.

Objectives: Previous clinical audits for chronic obstructive pulmonary disease (COPD) have provided valuable information on the clinical care delivered to patients admitted to medical wards because of COPD exacerbations. However, clinical audits of COPD in an outpatient setting are scarce and no methodological guidelines are currently available. Based on our previous experience, herein we describe a clinical audit for COPD patients in specialized outpatient clinics with the overall goal of establishing a potential methodological workflow., Methods: A pilot clinical audit of COPD patients referred to respiratory outpatient clinics in the region of Andalusia, Spain (over 8 million inhabitants), was performed. The audit took place between October 2013 and September 2014, and 10 centers (20% of all public hospitals) were invited to participate. Cases with an established diagnosis of COPD based on risk factors, clinical symptoms, and a post-bronchodilator FEV1/FVC ratio of less than 0.70 were deemed eligible. The usefulness of formally scheduled regular follow-up visits was assessed. Two different databases (resources and clinical database) were constructed. Assessments were planned over a year divided by 4 three-month periods, with the goal of determining seasonal-related changes. Exacerbations and survival served as the main endpoints., Conclusions: This paper describes a methodological framework for conducting a clinical audit of COPD patients in an outpatient setting. Results from such audits can guide health information systems development and implementation in real-world settings., This study was financially supported by an unrestricted grant from Laboratorios Menarini, SA (Barcelona, Spain).

Various studies have focused on understanding how the continuous positive air pressure (CPAP) treatment improves the emotional state of obstructive sleep apnea patients, as well as factors that determine improvement. Yet the results are contradictory. The objective of the present study was to evaluate the changes in depression levels, anxiety state and anxiety trait levels of patients with obstructive sleep apnea after 1 month and again after 3 months of CPAP treatment. The sample consisted of 51 obstructive sleep apnea patients. The Beck Depression Inventory (BDI) standardized spanish version was used to assess depressive symptoms. The State-Trait Anxiety Inventory (STAI) was used to assess two elements of the response to anxiety: anxiety-state and anxiety-trait. The results indicate that there exist statistically significant differences in depression levels after 1 month and after 3 months of treatment (P < 0.05). In specific, a drop in depression symptomatology was observed in both experimental conditions. With respect to anxiety state-trait levels, the obtained results show statistically significant differences in anxiety-trait levels after 1 month and after 3 months of treatment (P < 0.05) and in anxiety state after 3 months of treatment (P < 0.01). Both variables experienced a decrease after CPAP treatment.