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Background: In-hospital mortality in patients with coronavirus disease 2019 (COVID-19) is high. Simple prognostic indices are needed to identify patients at high-risk of COVID-19 health outcomes. We aimed to determine the usefulness of the CONtrolling NUTritional status (CONUT) index as a potential prognostic indicator of mortality in COVID-19 patients upon hospital admission. Methods: Our study design is of a retrospective observational study in a large cohort of COVID-19 patients. In addition to descriptive statistics, a Kaplan-Meier mortality analysis and a Cox regression were performed, as well as receiver operating curve (ROC). Results: From February 5, 2020 to January 21, 2021, there was a total of 2969 admissions for COVID-19 at our hospital, corresponding to 2844 patients. Overall, baseline (within 4 days of admission) CONUT index could be scored for 1627 (57.2%) patients. Patients' age was 67.3 ± 16.5 years and 44.9% were women. The CONUT severity distribution was: 194 (11.9%) normal (0-1); 769 (47.2%) light (2-4); 585 (35.9%) moderate (5-8); and 79 (4.9%) severe (9-12). Mortality of 30 days after admission was 3.1% in patients with normal risk CONUT, 9.0% light, 22.7% moderate, and 40.5% in those with severe CONUT (P < 0.05). An increased risk of death associated with a greater baseline CONUT stage was sustained in a multivariable Cox regression model (P < 0.05). An increasing baseline CONUT stage was associated with a longer duration of admission, a greater requirement for the use of non-invasive and invasive mechanical ventilation, and other clinical outcomes (all P < 0.05). The ROC of CONUT for mortality had an area under the curve (AUC) and 95% confidence interval of 0.711 (0.676-0746). Conclusion: The CONUT index upon admission is potentially a reliable and independent prognostic indicator of mortality and length of hospitalization in COVID-19 patients, The work is supported by a grant from the European Union’s Horizon 2020 Research and Innovation Programme under Grant Agreement (No 101016216)

The objective of the study was to analyze the factors associated with chronic bronchial infection (CBI) due to methicillin-susceptibleA prospective, longitudinal, multicenter study was conducted with patients included in the RIBRON registry between January 2015 and October 2020. The inclusion criteria were an age of 18 years or older and an initial diagnosis of bronchiectasis. Patients recorded in the registry had a situation of clinical stability in the absence of an exacerbation in the four weeks before their inclusion. All patients were encouraged to provide a sputum sample at each visit for microbiological culture. Annual pulmonary function tests were performed according to the national spirometry guidelines.A total of 426 patients were ultimately included in the study: 77 patients (18%) with CBI due to SA and 349 (82%) who did not present any isolation of PPMs in sputum. The mean age was 66.9 years (16.2), and patients 297 (69.7%) were female, with an average BMI of 25.1 (4.7) kg/mPatients with non-CF bronchiectasis with CBI due to SA were younger, with lower FEV1% values, more significant extension of bronchiectasis, and a higher number of exacerbations of mild to moderate symptoms than those with no PPM isolation in respiratory secretions. The reduction in FEV1% was -1.19% (95% CI: -2.09, -0.69) (

Both systemic inflammation and exacerbations have been associated with greater severity of bronchiectasis. Our objective was to analyze the prognostic value of the peripheral concentration of C-reactive protein (CRP) for the number and severity of exacerbations in patients with bronchiectasis.Patients from the Spanish Bronchiectasis Registry (RIBRON) with valid data on their CRP value (in a clinically stable phase) and valid data on exacerbations during the first year of follow-up were included. A logistic regression analysis was used to evaluate the prognostic value of the CRP concentration (divided into tertiles) with the presence of at least one severe exacerbation or at least two mild-moderate exacerbations during the first year of follow-up.802 patients (mean age: 68.1 [11.1 years], 65% female) were included. Of these, 33.8% and 13%, respectively, presented ≥2 mild-moderate exacerbations or at least one severe exacerbation during the first year of follow-up. The mean value of the CRP was 6.5 (17.6mg/L). Patients with a CRP value between 0.4 and 2.7mg/L (second tertile) and ≥2.7mg/L (third tertile) presented a 2.9 (95%CI: 1.4-5.9) and 4.2 (95%CI: 2.2-8.2) times greater probability, respectively, of experiencing a severe exacerbation than those with0.4mg/L (control group), regardless of bronchiectasis severity or a history of previous exacerbations. However, the CRP value did not present any prognostic value for the number of mild-moderate exacerbations.The CRP value was associated with a greater risk of future severe exacerbations but not with mild or moderate exacerbations in patients with steady-state bronchiectasis.

Resumen Introduccion El Registro Espanol de Bronquiectasias de la SEPAR (RIBRON) comenzo como una plataforma longitudinal de recogida de datos de pacientes con esta enfermedad. El objetivo del presente estudio es describir tanto su funcionamiento, como analizar las caracteristicas de los pacientes con bronquiectasias segun el sexo. Metodos Entre febrero de 2015 y 2019, fueron incluidos 1.912 pacientes adultos diagnosticados de bronquiectasias procedentes de 43 centros. Todos los pacientes presentaron al menos datos completos de 79 variables basicas necesarias y controladas mediante una auditoria externa. Resultados Edad media fue de 67,6 anos (15,2), el 63,9% mujeres. El sintoma mas comun fue la tos productiva en el 78,3%, que fue mucopurulenta-purulenta en el 45,9%. La etiologia mas frecuente fue la postinfecciosa en el 40,4%, siendo idiopaticas en el 18,5%. Pseudomonas aeruginosa fue el microorganismo mas frecuentemente aislado con el 40,4%, el 25,6% en forma de infeccion cronica. El numero anual de agudizaciones leve-moderadas/graves fue de 1,62 (1,9)/0,59 (1,3). El 50% de los pacientes presentaron obstruccion al flujo aereo (el 17% grave). La localizacion mas frecuente fueron los lobulos inferiores. El valor medio del FACED /E-FACED/BSI fue de 2,06 (1,7)/2,67 (2,2)/7,8 (4,5), respectivamente. El 66,7% de los pacientes tomaban corticoides inhalados, el 19,2% macrolidos y el 19,5% antibioticos inhalados. Las mujeres presentaron un perfil de menor gravedad que los varones en terminos clinico-funcionales y etiologicos, pero semejante perfil infeccioso, radiologico y terapeutico. Conclusiones RIBRON representa un excelente mapa de las caracteristicas de las bronquiectasias en nuestro pais. Dos tercios de los pacientes son mujeres que presentaron unas caracteristicas propias, de menor gravedad de la enfermedad.

Resumen A pesar de que es conocido que la presencia cronica de microorganismos en las vias aereas de pacientes con enfermedad pulmonar obstructiva cronica (EPOC) en fase de estabilidad conlleva una evolucion desfavorable, ninguna guia de manejo de la enfermedad establece pautas sobre como diagnosticar y tratar este tipo de casos. Con la intencion de orientar a los profesionales, desde la Sociedad Espanola de Neumologia y Cirugia Toracica (SEPAR) se ha elaborado un documento que pretende aportar respuestas clinicas sobre el manejo de pacientes con EPOC en los que se aislan microorganismos de forma puntual o persistente. Dado que la heterogeneidad de las evidencias cientificas disponibles no permite crear una Guia de Practica Clinica, se ha elaborado un documento basado en la literatura cientifica existente y/o en la propia experiencia clinica que aborda tanto la definicion de las diferentes situaciones clinicas como su diagnostico y manejo. El texto ha sido consensuado entre un amplio numero de neumologos con gran experiencia clinica y cientifica en este ambito. Este documento cuenta con el aval del Comite Cientifico de SEPAR.

Resumen Introduccion El papel de las micobacterias no tuberculosas (MNT) en los pacientes con fibrosis quistica (FQ) esta, en ocasiones, en controversia. El objetivo del trabajo es evaluar la prevalencia y las caracteristicas clinicas/microbiologicas de pacientes adultos con FQ colonizados con MNT, destacando Mycobacterium abscessus (M. abscessus). Metodos Se ha realizado un estudio retrospectivo en 92 pacientes adultos con FQ en el que se diferencio: grupo control, 64 pacientes no colonizados por MNT, y grupo a estudio, 28 pacientes colonizados por MNT. Se han analizado variables como la edad, mutacion F508del, funcion pulmonar, afectacion pancreatica, tincion de auramina y recolonizaciones entre ambos grupos. Resultados La prevalencia de MNT encontrada ha sido 30,4%. La MNT mas prevalente fue Mycobacterium avium complex seguida por M. abscessus. Para M. abscessus, en el estudio comparativo con pacientes colonizados por otras MNT, se obtuvieron diferencias estadisticamente significativas en las variables de edad. Discusion Hemos encontrado alta prevalencia de MNT en pacientes adultos con FQ y relacionamos la aparicion de M. asbcessus con edades inferiores a 30 anos y F508del. Con el fin de conocer mejor el papel patogeno de las MNT, especialmente de M. asbcessus, se requieren estudios multicentricos en poblacion con FQ.

Introduction The role of non-tuberculous mycobacteria (NTM) among cystic fibrosis (CF) patients, on occasion, remains unknown. The aim of our study is to evaluate the prevalence and clinical/microbiological characteristics of CF adult patients colonized by NTM, highlighting Mycobacterium abscessus (M. abscessus). Methods A retrospective study was conducted with 92 CF adult patients: including a control group of 64 patients, not colonized by NTM, and a study group of 28 patients, colonized by NTM. We have analyzed variables such as age, F508del mutation, lung function, pancreatic involvement, auramine staining and co-colonizations between both groups. Results The prevalence of NTM found was 30.4%. The most prevalent was Mycobacterium avium complex followed by M. abscessus. For M. abscessus, in the comparative study with patients colonized by other NTM, significant results were obtained for variables age. Discussion We have found a high prevalence of NTM among adult patients with CF, and we associated the presence of M. asbcessus with ages less than 30 years and F508del. Due to the pathogenic role of NTM, especially M. asbcessus, multicenter studies are required within the population suffering from CF.

Resumen: Las bronquiectasias (BQ) son una enfermedad tanto heterogénea en su presentación como compleja en su naturaleza. Una variable única no es capaz de captar el impacto total que las BQ producen sobre el individuo. Algunos autores han observado la presencia de algunos fenotipos clínicos más homogéneos en su clínica, pronóstico o respuesta al tratamiento, como el paciente con infección bronquial crónica por P. aeruginosa, el paciente «exacerbador» o el overlap (BQ + enfermedad pulmonar obstructiva crónica [EPOC]). Otros autores han desarrollado escalas multidimensionales que tratan de clasificar la gravedad o el pronóstico, teniendo en cuenta diferentes aspectos de la enfermedad como la clínica, la función pulmonar, la extensión radiológica o el perfil microbiológico entre otros. Finalmente, se están desarrollando herramientas clínicas que van un paso más allá y valoran las BQ como una enfermedad compleja, teniendo en cuenta no solo su gravedad, sino también su actividad biológica y la forma que el paciente tiene de vivir la enfermedad (impacto). Para ello, además de las puntuaciones multidimensionales de gravedad, se han venido desarrollando diferentes cuestionarios de calidad de vida específicos o se han validado para esta enfermedad aquellos que eran utilizados en otras patologías respiratorias, fundamentalmente EPOC. Por otro lado, se siguen investigando biomarcadores biológicos válidos que se relacionen con outcomes de importancia en BQ. Abstract: Bronchiectasis is a disease that is both heterogeneous in form and complex in nature. The overall impact of bronchiectasis on the individual cannot be captured by a single variable. Some authors have observed the presence of some more homogeneous clinical phenotypes in terms of symptoms, prognosis or response to treatment in, for example, patients with chronic P. aeruginosa bronchial infection, exacerbators or patients with bronchiectasis and COPD overlap. Other authors have developed multidimensional scales that attempt to classify severity or prognosis, taking into account different aspects of the disease, such as symptoms, lung function, radiological extension, and microbiological profile, among others. Finally, clinical tools are being developed that go a step further and consider bronchiectasis as a complex disease, taking into account not only its severity, but also its biological activity and impact on the patient's way of life. To this end, multidimensional severity scores and different specific quality of life questionnaires have been developed, and those used in other respiratory pathologies, mainly COPD, have been validated for this disease. On another front, the search continues for valid biological biomarkers associated with outcomes of importance in bronchiectasis.

Resumen En la mayoria de los ambitos de la neumologia se sigue utilizando un principio osleriano (basado en los sintomas y signos) en los que la enfermedad es el centro de toda actividad, pero este paradigma esta cambiando. Actualmente, gracias al reconocimiento de la heterogeneidad y complejidad de las enfermedades pulmonares, la tendencia es a realizar una medicina mas personalizada, de precision, o centrada en el paciente. En la presente revision se intentara establecer la situacion actual sobre el conocimiento de las bronquiectasias, o mejor, del sindrome bronquiectasico, como una enfermedad multidimensional, sistemica, heterogenea y compleja, los pasos que ya se han dado en este sentido, y sobre todo, en los muchos que quedan por dar. Asimismo, se propondran algunas herramientas que podrian facilitar la traslacion de estos conceptos a la practica clinica, y con ellos seguir avanzando hacia una imagen mas holistica de esta enfermedad.

Introduction: Bronchiectasis is a very heterogeneous disease but some homogeneous groups with similar clinical characteristics and prognosis have been identified. Exacerbations have been shown to have a negative impact on the natural history of bronchiectasis. The objective of this study was to identify the definition and characteristics of the "frequent exacerbator patient" with the best prognostic value and its relationship with the severity of bronchiectasis. Methods: A historical cohort of 651 patients diagnosed with bronchiectasis was included. They had all received 5 years of follow-up since their radiological diagnosis. Exacerbation was defined as a worsening of the symptoms derived from bronchiectasis that required antibiotic treatment. The main outcome was all-cause mortality at the end of follow-up. Results: The mean age was 48.2 (16) years (32.9% males). 39.8% had chronic infection by Pseudomonas aeruginosa. Mean BSI, FACED, and E-FACED were 7 (4.12), 2.36 (1.68), and 2.89 (2.03), respectively. There were 95 deaths during follow-up. The definition of the "frequent exacerbator patient" that presented the greatest predictive power for mortality was based on at least two exacerbations/year or one hospitalization/year (23.3% of patients; AUC-ROC: 0.75 [95% CI: 0.69-0.81]). Its predictive power was independent of the patient's initial severity. The clinical characteristics of the frequent exacerbator patient according to this definition varied according to the initial severity of bronchiectasis, presence of systemic inflammation, and treatment. Conclusions: The combination of two exacerbations or one hospitalization per year is the definition of frequent exacerbator patient that has the best predictive value of mortality independent of the initial severity of bronchiectasis. (C) 2018 SEPAR. Published by Elsevier Espana, S.L.U. All rights reserved.

Background There are important advances in the management of bacterial infection in patients with cystic fibrosis (CF), but there are many gaps in the field of fungal infections. Aims The aim of this study was to analyse whether chronic respiratory filamentous fungal colonization had clinical impact and whether antifungal treatment can change the disease. Methods The prospective, bicentric and descriptive study was carried out within a 3-year follow-up period, with four-month periodicity medical controls. Adult patients from two CF units of tertiary hospitals were included. Clinical, microbiological, analytical and spirometric variables were collected. Quality of life was evaluated in a subgroup, using the Spanish version of the Revised Cystic Fibrosis Quality of Life Questionnaire (CFQ-R). To statistically analyze the evolution of forced expiratory along time (volume of air blown out in 1 second -FEV1-) and the forced vital capacity (FVC), mixed linear models were carried out. Results From the ninety-eight patients under study, 40 suffered chronic filamentous fungal colonization. The presence of filamentous fungi in airway was associated to an annual fall of FEV1 and FVC of 0.029 and 0.017 litres, respectively (p Conclusions Chronic filamentous fungal colonization in patients with CF is associated with a significant annual decline of lung function that persists over time. Chronic antifungal therapy slows down this progression, mainly in the patient with more advanced disease.

Pseudomonas aeruginosa (P. aeruginosa) is a ubiquitous and opportunistic microorganism and is considered one of the most significant pathogens that produce chronic colonization and infection of the lower respiratory tract, especially in people with chronic inflammatory airway diseases such as asthma, chronic obstructive pulmonary disease (COPD), cystic fibrosis (CF), and bronchiectasis. From a microbiological viewpoint, the presence and persistence of P. aeruginosa over time are characterized by adaptation within the host that precludes any rapid, devastating injury to the host. Moreover, this microorganism usually develops antibiotic resistance, which is accelerated in chronic infections especially in those situations where the frequent use of antimicrobials facilitates the selection of “hypermutator P. aeruginosa strain”. This phenomenon has been observed in people with bronchiectasis, CF, and the “exacerbator” COPD phenotype. From a clinical point of view, a chronic bronchial infection of P. aeruginosa has been related to more severity and poor prognosis in people with CF, bronchiectasis, and probably in COPD, but little is known on the effect of this microorganism infection in people with asthma. The relationship between the impact and treatment of P. aeruginosa infection in people with airway diseases emerges as an important future challenge and it is the most important objective of this review.

Background: Nebulised antibiotics are habitually used in patients with bronchiectasis, but the use of dry powder inhaled antibiotics (DPIA) in these patients is extremely limited. This study seeks to analyse the efficacy and safety of DPIA in bronchiectasis patients. Material and methods: Multi-centre study of historic cohorts. All the hospital centres in Spain were contacted in order to collect data on patients with a diagnosis of bronchiectasis who had taken at least one dose of DPIA. Its efficacy was analysed in clinical, functional and microbiological terms by comparing the year before and the year after the prescription of DPIA. Adverse effects and variables associated with these effects, or any need to withdraw the drug, were also analysed. Results: 164 patients from 33 Spanish centres were included, 86% and 14% of these were treated with dry powder colistin and tobramycin, respectively. Chronic bronchial infection by Pseudomonas aeruginosa was present in 86% of these patients, and DPIA significantly reduced the number of exacerbations, the quantity and purulence of sputum and the isolation of pathogenic microorganisms. The most common adverse effect was cough (40%), particularly in cases of Chronic Obstructive Pulmonary Disease (COPD) and a previous cough and in those patients who had difficulties in handling the device. These factors were associated with a higher level of withdrawal of the treatment. There were no serious adverse effects. Conclusions: Our study suggests that DPIA are clinically efficacious and safe for treating bronchiectasis patients. Cough was shown to be the most common side-effect and reason for withdrawal of the treatment.

Resumen En 2008, la Sociedad Espanola de Neumologia y Cirugia Toracica (SEPAR) publico las primeras normativas del mundo sobre el diagnostico y tratamiento de las bronquiectasias. Tras casi una decada, muchos han sido los avances cientificos en esta enfermedad, tanto en sus aspectos terapeuticos como en su valoracion y diagnostico. Por ello estas nuevas normativas sobre el tratamiento de las bronquiectasias en el adulto tratan de ofrecer al lector una actualizacion del conocimiento cientifico sobre las posibilidades terapeuticas en bronquiectasias, basandose en un estricto procedimiento metodologico que asegura la calidad del contenido de la misma, y en una amplia cantidad de informacion online que incluye abundante bibliografia. En estas normativas se ha enfocado el tratamiento de las bronquiectasias desde un punto de vista tanto multidisciplinar, que implica las especialidades y escalones asistenciales involucrados, como multidimensional que incluye todos y cada uno de los aspectos que definen a la enfermedad. Asi, se establecen recomendaciones basadas en una exhaustiva revision de la evidencia sobre los tratamientos de la etiologia, de la infeccion bronquial en sus diferentes formas de presentacion y con las diferentes terapias existentes, de la inflamacion bronquial y de la obstruccion al flujo aereo. Se revisan los aspectos nutricionales, el manejo de las secreciones, el entrenamiento muscular, el manejo de las complicaciones y comorbilidades, la profilaxis de la infeccion, los aspectos educacionales, el manejo del paciente en el domicilio, el tratamiento quirurgico, las agudizaciones y el seguimiento de los pacientes.

In 2008, the Spanish Society of Pulmonology (SEPAR) published the first guidelines in the world on the diagnosis and treatment of bronchiectasis. Almost 10 years later, considerable scientific advances have been made in both the treatment and the evaluation and diagnosis of this disease, and the original guidelines have been updated to include the latest scientific knowledge on bronchiectasis. These new recommendations have been drafted following a strict methodological process designed to ensure the quality of content, and are linked to a large amount of online information that includes a wealth of references. These guidelines cover aspects ranging from a consensual definition of bronchiectasis to an evaluation of the natural course and prognosis of the disease. The topics of greatest interest and some new areas are addressed, including epidemiology and economic costs of bronchiectasis, pathophysiological aspects, the causes (placing particular emphasis on the relationship with other airway diseases such as chronic obstructive pulmonary disease and asthma), clinical and functional aspects, measurement of quality of life, radiological diagnosis and assessment, diagnostic algorithms, microbiological aspects (including the definition of key concepts, such as bacterial eradication or chronic bronchial infection), and the evaluation of severity and disease prognosis using recently published multidimensional tools.

Whether high blood eosinophil counts may define a better phenotype in bronchiectasis patients, as shown in chronic obstructive pulmonary disease (COPD), remains to be investigated. Differential phenotypic characteristics according to eosinophil counts were assessed using a biostatistical approach in a large cohort study from the Spanish Online Bronchiectasis Registry (RIBRON). The 906 patients who met the inclusion criteria were clustered into two groups on the basis of their eosinophil levels. The potential differences according to the bronchiectasis severity index (BSI) score between two groups (Mann-Whitney U test and eosinophil count threshold: 100 cells/µL) showed the most balanced cluster sizes: above-threshold and below-threshold groups. Patients above the threshold exhibited significantly better clinical outcomes, lung function, and nutritional status, while showing lower systemic inflammation levels. The proportion of patients with mild disease was higher in the above-threshold group, while the below-threshold patients were more severe. Two distinct clinical phenotypes of stable patients with non-cystic fibrosis (CF) bronchiectasis of a wide range of disease severity were established on the basis of blood eosinophil counts using a biostatistical approach. Patients classified within the above-threshold cluster were those exhibiting a mild disease, significantly better clinical outcomes, lung function, and nutritional status while showing lower systemic inflammatory levels. These results will contribute to better characterizing bronchiectasis patients into phenotypic profiles with their clinical implications. This study was supported by FIS 18/00075, FIS 21/00215 (FEDER, ISC-III) & CIBERES (ISC-III), SEPAR 2020, and ZAMBON-PHARMA (Spain). JGO was supported by the Spanish Ministry of Science and Innovation and FEDER (project PGC 2018-101251-B-I00 and Maria de Maeztu Programme for Units of Excellence in R&D, grant CEX2018-000792-M) and by the Generalitat de Catalunya (ICREA Academia programme).

Objetivo: Determinar el patrón alimentario que caracteriza el estado nutricional de los estudiantes de la Institución Educativa Inicial N° 547, Huaral. Material y Métodos: Estudio descriptivo, transversal y cuantitativo que evaluó a 54 preescolares y sus madres; se aplicó un cuestionario sobre características sociodemográficas y alimentación del preescolar por duplicado. Se midió peso y talla para determinar el estado nutricional según indicadores P/E, T/E y P/T, comparándolas con las tablas del CENAN. Resultados: Las principales preparaciones consumidas por los preescolares en el desayuno fueron: las bebidas a base de cereales c/s maca (52,2%) acompañado de pan sin frituras (39,8%); en el almuerzo las preparaciones no fritas con pollo (48,1%) acompañada de bebidas naturales (64,9%) y la cena las preparaciones sobrantes del almuerzo (50%), los alimentos de mayor consumo durante las colaciones o refrigerios fueron las frutas. Los cereales (26,5%) fueron el principal grupo de alimentos consumidos. El 72% de los preescolares consume sus alimentos principalmente en el hogar. Hubo mayor prevalencia de niños con estado nutricional normal (66,7%). Conclusiones: El número de comidas consumidas al día por los preescolares fueron 3; desayuno compuesta por bebidas calientes a base de cereales y pan; el almuerzo por preparaciones no fritas y fritas con pollo; la cena por preparaciones sobrantes del almuerzo o bebidas calientes con pan; las colaciones por frutas y bebidas naturales, siendo el hogar el principal lugar de consumo de estas preparaciones. El estado nutricional normal fue el de mayor prevalencia para el 66%.Palabras clave: Patrón alimentario, Estado nutricional, Preescolares

The SEPAR Spanish Bronchiectasis Registry (RIBRON) began as a platform for the collection of longitudinal data on patients with this disease. The objective of this study is to describe its operation and to analyze the characteristics of bronchiectasis patients according to sex.A total of 1912 adult patients diagnosed with bronchiectasis in 43 centers were included between February 2015 and 2019. All patients had complete data consisting of at least 79 basic required variables, controlled by an external audit.Mean age was 67.6 (15.2) years; 63.9% were women. The most common symptom was productive cough (78.3%) which was mucopurulent-purulent in 45.9% of cases. The most common etiology was post-infectious (40.4%), while 18.5% were idiopathic. Pseudomonas aeruginosa was the most frequently isolated microorganism (40.4%), of which 25.6% were associated with chronic infection. The annual number of mild-to-moderate/severe exacerbations was 1.62 (1.9)/0.59 (1.3). Half of the patients (50%) presented with airflow obstruction (17% severe). The most frequent radiological localization was lower lobes. The average FACED/E-FACED/BSI values were 2.06 (1.7)/2.67 (2.2)/7.8 (4.5), respectively. Overall, 66.7% of patients were taking inhaled corticosteroids, 19.2% macrolides, and 19.5% inhaled antibiotics. Women presented a less severe profile than men in clinical and functional terms, and a similar infectious, radiological and therapeutic profile.RIBRON represents an excellent map of the characteristics of bronchiectasis in our country. Two thirds of patients are women who presented lower disease severity as a specific characteristic.

Bronchiectasis; Quality of life; Respiratory disease Bronquiectasia; Calidad de vida; Enfermedad respiratoria Bronquiectàsia; Qualitat de vida; Malaltia respiratòria Cough is a main symptom in cystic fibrosis (CF). We aim to validate a Spanish version of the Leicester Cough Questionnaire (LCQ-Sp) to measure the impact of cough in CF bronchiectasis. A prospective longitudinal multicentre study was performed. Internal consistency and score changes over a 15-day period in stable state were assessed to analyse reliability. Concurrent validity was analysed by correlation with Saint George’s Respiratory Questionnaire (SGRQ) and convergent validity by assessing the association with clinical variables. Changes in scores between stable state and the first exacerbation were assessed to analyse responsiveness. 132 patients (29.73 ± 10.52 years) were enrolled in four hospitals. Internal consistency was high for the total score and good for the three domains (Cronbach’s α 0.81–0.93). The test–retest reliability showed an intraclass correlation coefficient of 0.86 for the total score. The correlation between LCQ-Sp and SGRQ scores was −0.74. The LCQ-Sp score negatively correlated with sputum volume, and the mean score decreased at the beginning of exacerbations (16.04±3.81 vs 13.91±4.29) with a large effect size. The LCQ-Sp is a reliable, repeatable and responsive instrument to assess the impact of cough in CF bronchiectasis and is responsive to change in the event of exacerbations. The author(s) received no financial support for the research, authorship, and/or publication of this article.

Background: The excessive retention of sputum in the airways, leading to pulmonary infections, is a common consequence of bronchiectasis. Although inhalation of 7% hypertonic saline (HS) has proven an effective method to help remove the mucus, many patients are intolerant of this treatment. The addition of 0.1% hyaluronic acid to HS (HS+HA) could increase tolerance to HS in these patients. The main objective of this study was to evaluate the tolerability of HS+HA in bronchiectasis patients who are intolerant to HS. Methods: This prospective, observational, open-label study analysed the outcomes of two groups of bronchiectasis patients previously scheduled to start HS therapy. Patients were assessed for tolerance to HS by a questionnaire, spirometry and clinical evaluation. Patients who were intolerant were evaluated for tolerance to HS+HA approximately one week later. All patients were evaluated for their tolerance to HS or HS+HA 4 weeks after the start of their treatment. Patients were also assessed with quality-of-life and adherence questionnaires, and all adverse events were registered. Results: A total of 137 bronchiectasis patients were enrolled in the study (age = 63.0 ± 14.7 years; 63.5% women). Of these, 92 patients (67.1%) were tolerant and 45 patients (32.9%) were intolerant to HS. Of the 45 patients intolerant to HS, 31 patients (68.9%) were tolerant and 14 patients (31.1%) intolerant to HS+HA. Of these 31 tolerant patients, 26 (83.9%) could complete the 4-week treatment with HS+HA. Conclusions: Two-thirds of bronchiectasis patients that presented intolerance to inhaled HS alone are tolerant to inhaled HS+HA, suggesting that HA improves tolerance to HS therapy.

Background Glycosaminoglycans (GAGs) are essential in many infections, including recurrent bacterial respiratory infections, the main cause of mortality in cystic fibrosis (CF) patients. Methods Using a cellular model of healthy and CF lung epithelium, a comparative transcriptomic study of GAG encoding genes was performed using qRT-PCR, and their differential involvement in the adhesion of bacterial pathogens analyzed by enzymatic degradation and binding competition experiments. Results Various alterations in gene expression in CF cells were found which affect GAG structures and seem to influence bacterial adherence to lung epithelium cells. Heparan sulfate appears to be the most important GAG species involved in bacterial binding. Conclusions Adherence to lung epithelial cells of some of the main pathogens involved in CF is dependent on GAGs, and the expression of these polysaccharides is altered in CF cells, suggesting it could play an essential role in the development of infectious pathology.

Objectives: Despite the clinical relevance of exacerbations in bronchiectasis (BE), little is known about the microbiology and outcomes of pneumonic (CAP) vs. non-pneumonic (NOCAP) exacerbations. Methods: This study compares clinical and microbiological characteristics of CAP vs. NOCAP in adults with BE. We performed a multicenter prospective observational study of consecutive cases of NOCAP and CAP from four Spanish hospitals (2011-2015). Results: We recruited 144 patients, 47 of them CAP (33%) cases. CAP patients were older, with a larger representation of males, more comorbidities, higher arterial hypertension and COPD but less chronic bronchial infection and previous history of exacerbations. Clinical presentation was similar, excepting creatinine, C-reactive protein (C-RP), glucose and leukocytes which were higher in CAP. C-RP of 8.38 mg/dL showed a significant predictive discrimination for CAP. Streptococcus pneumoniae and Pseudomonas aeruginosa were the first causes of CAP and NOCAP, respectively. The rate of microbiological concordance with previous chronic bronchial infection was variable. Main clinical outcomes (mortality, length of stay, etc.) were similar in the two groups. Chronic bronchial infection and history of frequent exacerbations (>= 2/year) were associated with a reduced risk of CAP. Conclusions: CAP and NOCAP in BE had similar clinical presentation with the exception of fever, leukocytosis, and C-RP. Microbiology also differed. A cut-off value of C-RP >= 8.83 mg/dL can predict CAP in bronchiectasis. (C) 2018 The British Infection Association. Published by Elsevier Ltd. All rights reserved.

Resumen Pseudomonas aeruginosa es el patogeno mas importante en la infeccion broncopulmonar en fibrosis quistica (FQ). Solo se erradica en la infeccion inicial, mientras que la reduccion de su carga bacteriana es el objetivo terapeutico en la infeccion cronica y exacerbaciones. El cribado neonatal y la farmacocinetica/farmacodinamica han cambiado el manejo del paciente con FQ. Se debe realizar un seguimiento microbiologico en los pacientes sin infeccion por P. aeruginosa. En la infeccion inicial se recomienda tratamiento inhalado (28 dias) con colistina (0,5-2 MU/8 h), tobramicina (300 mg/12 h) o aztreonam (75 mg/8 h) con o sin ciprofloxacino oral (15-20 mg/kg/12 h, 2-3 semanas). En la infeccion cronica se recomienda solo via inhalada en tratamiento continuo con colistina, o en ciclos on-off de 28 dias con tobramicina o aztreonam. Durante las exacerbaciones leves-moderadas se recomienda tratamiento oral (ciprofloxacino, 2-3 semanas) y en las graves tratamiento intravenoso (β-lactamico asociado a un aminoglicosido o una fluoroquinolona). Estudios futuros sustentaran la rotacion y nuevas combinaciones de antimicrobianos. Se deben establecer tambien medidas epidemiologicas que eviten nuevas infecciones y la transmision cruzada de P. aeruginosa.

Pseudomonas aeruginosa is the main pathogen in bronchopulmonary infections in cystic fibrosis (CF) patients. It can only be eradicated at early infection stages while reduction of its bacterial load is the therapeutic goal during chronic infection or exacerbations. Neonatal screening and pharmacokinetic/pharmacodynamic knowledge has modified the management of CF-patients. A culture based microbiological follow-up should be performed in patients with no infection with P. aeruginosa. At initial infection, inhaled colistin (0,5-2 MU/tid), tobramycin (300 mg/bid) or aztreonam (75 mg/tid) with or without oral ciprofloxacin (15-20 mg/kg/bid, 2-3 weeks) are recommended. In chronic infections, treatment is based on continuous administration of colistin or with a 28-day on-off regimen with tobramycin or aztreonam. During mild-moderate exacerbations oral ciprofloxacin (2-3 weeks) can be administered while serious exacerbations must be treated with intravenous combination therapy (beta-lactam with an aminoglycoside or a fluoroquinolone). Future studies will support antibiotic rotation and/or new combination therapies. Epidemiological measures are also recommended to avoid new P. aeruginosa infections and "patient-to-patient transmission" of this pathogen. (C) 2014 SEPAR. Published by Elsevier Espana, S.L.U. All rights reserved.

Background: Information on the role of fungi in non-cystic fibrosis (CF) bronchiectasis is lacking. Objectives: Our aim was to determine the prevalence of and factors associated with the isolation and persistence of fungi from sputum in these patients. Methods: We performed a multicenter observational study comprising adult patients with non-CF bronchiectasis. Persistence of Aspergillus spp. and Candida albicans was defined as the presence of ≥2 positive sputum cultures taken at least 6 months apart within a period of 5 years. Results: A total of 252 patients (62.7% women with a mean ± SD age of 55.3 ± 16.7 years) were included in the study. All patients had at least 1 sputum sample cultured for fungi, with a mean ± SD of 7 ± 6 cultures per patient. Eighteen (8.7%) and 71 (34.5%) patients had persistent positive cultures for Aspergillus spp. and C. albicans, respectively. Patients with persistence of Aspergillus spp. and C. albicans were older and had more daily purulent sputum. In addition, patients with persistent C. albicans had worse postbronchodilator forced expiratory volume in the first second (FEV1), more frequent cystic bronchiectasis, and more hospital-treated exacerbations. They were also more frequently treated with long-term antibiotics. Multivariate analysis showed that daily purulent sputum (OR = 3.75, p = 0.045) and long-term antibiotics (OR = 2.37, p = 0.005) were independently associated with persistence of Aspergillus spp. and C. albicans, respectively. Conclusions: Isolation and persistence of Aspergillus spp. and C. albicans are frequent in patients with non-CF bronchiectasis. Daily purulent sputum and chronic antibiotic treatment were associated with persistence of Aspergillus spp. and C. albicans, respectively.

In 2008, the Spanish Society of Pulmonology (SEPAR) published the first guidelines in the world on the diagnosis and treatment of bronchiectasis. Almost 10 years later, considerable scientific advances have been made in both the treatment and the evaluation and diagnosis of this disease, and the original guidelines have been updated to include the latest therapies available for bronchiectasis. These new recommendations have been drafted following a strict methodological process designed to ensure quality of content, and are linked to a large amount of online information that includes a wealth of references. The guidelines are focused on the treatment of bronchiectasis from both a multidisciplinary perspective, including specialty areas and the different healthcare levels involved, and a multidimensional perspective, including a comprehensive overview of the specific aspects of the disease. A series of recommendations have been drawn up, based on an in-depth review of the evidence for treatment of the underlying etiology, the bronchial infection in its different forms of presentation using existing therapies, bronchial inflammation, and airflow obstruction. Nutritional aspects, management of secretions, muscle training, management of complications and comorbidities, infection prophylaxis, patient education, home care, surgery, exacerbations, and patient follow-up are addressed. (C) 2017 SEPAR. Published by Elsevier Espana, S.LU. All rights reserved.

Information about antibiotic prescription patterns for cystic fibrosis (CF) patients and, specifically, about inhaled treatment strategies for their management is lacking in Spain due to the absence of a national patient registry. In this study we present data about antibiotic prescription in the Spanish CF context that were obtained in a multicenter study, being inhaled treatment strategies the special focus of this work.Twenty-four specialized CF units (12 adult, 12 pediatric) from 17 tertiary-care hospitals covering all Spanish Autonomous Communities provided sputa and clinical data from 15 consecutive patients. Data about antibiotic and non-antibiotic therapies prescribed to these patients during the year prior inclusion (2013) were retrospectively collected.The multicenter study included 341 CF patients from all age groups and clinical status. The prevalence of oral, inhaled and intravenous therapies was 89% (n = 302), 80% (n = 273) and 31% (n = 105), respectively. The most prevalent oral agents were ciprofloxacin (n = 177, 59%), cotrimoxazole (n = 109, 36%) and amoxicillin-clavulanate (n = 99, 33%), whereas ceftazidime (n = 53, 50%), tobramycin (n = 43, 41%) and meropenem (n = 41, 49%) were the most prevalent intravenous ones. Two or more different inhaled antibiotics were administered to 67 patients (24%), 51 of them receiving 2 drugs continuously in alternating schemes. Nebulization of intravenous specific antibiotics was common (n = 39) and, in some cases, was used for maintenance purposes.These results show that the treatment of CF patients is evolving more rapidly than clinical consensus guidelines. Clinical trials evaluating new specific inhaled combinations and new alternative treatment regimes of the existing ones are needed.Objetivos: Existen actualmente pocos datos acerca de las pautas de tratamiento antimicrobiano administradas a los pacientes con fibrosis quística (FQ) en España, sobre todo en lo que se refiere a la antibioterapia inhalada. Esta escasez de conocimiento se debe principalmente a la ausencia de un registro nacional de datos de pacientes. En 2013 se llevó a cabo el primer estudio multicéntrico español focalizado en la microbiología de la FQ. En este trabajo presentamos los patrones de prescripción de antimicrobianos administrados durante un año a los pacientes incluidos en dicho estudio.Métodos: Se contó con la participación de 24 unidades de FQ (12 de adultos y 12 de pediatría) procedentes de 17 hospitales españoles. Cada unidad reclutó a 15 pacientes de manera consecutiva, que aportaron muestras respiratorias y datos clínicos. Se recogieron de manera retrospectiva los tratamientos antibióticos y no antibióticos administrados a estos pacientes durante el año previo a su inclusión en el estudio.Resultados: Se incluyeron 341 pacientes con FQ de todos los rangos de edad y de gravedad clínica. La prevalencia de antibioterapia oral, inhalada e intravenosa fue del 89% (n = 302), 80% (n = 273) y 31% (n = 105), respectivamente. Los fármacos administrados con mayor frecuencia por vía oral fueron ciprofloxacino (n = 177, 59%), cotrimoxazol (n = 109, 36%) y amoxicilina-clavulánico (n = 99, 33%), siendo ceftazidima (n = 53, 50%), tobramicina (n = 43, 41%) y meropenem (n = 41, 49%) los más frecuentes por vía intravenosa. Se administraron dos o más antibióticos por vía inhalada a 67 pacientes (24%), habiendo recibido 51 de ellos 2 antibióticos simultáneamente de manera rotatoria. La nebulización de antibióticos con formulación intravenosa fue una práctica común (n = 39) y, en algunos casos, se utilizó durante un tiempo prolongado como terapia de mantenimiento.Conclusiones: Los esquemas de tratamiento observados en este estudio demuestran que la terapia antibiótica de la FQ evoluciona más rápidamente que las recomendaciones reflejadas en las guías clínicas. Es necesario evaluar estos nuevos esquemas con estudios clínicos, así como con otros fármacos inhalados de reciente aparición y su papel en los esquemas existentes.

Background The FACED score is an easy-to-use multidimensional grading system that has demonstrated an excellent prognostic value for mortality in patients with bronchiectasis. A Spanish group developed the score but no multicenter international validation has yet been published. Methods Retrospective and multicenter study conducted in six historical cohorts of patients from Latin America including 651 patients with bronchiectasis. Clinical, microbiological, functional, and radiological variables were collected, following the same criteria used in the original FACED score study. The vital status of all patients was determined in the fifth year of follow-up. The area under ROC curve (AUC-ROC) was used to calculate the predictive power of the FACED score for all-cause and respiratory deaths and both number and severity of exacerbations. The discriminatory power to divide patients into three groups of increasing severity was also analyzed. Results Mean (SD) age of 48.2 (16), 32.9% of males. The mean FACED score was 2.35 (1.68). During the follow up, 95 patients (14.6%) died (66% from respiratory causes). The AUC ROC to predict all-cause and respiratory mortality were 0.81 (95% CI: 0.77 to 0.85) 0.84 (95% CI: 0.80 to 0.88) respectively, and 0.82 (95% CI: 078–0.87) for at least one hospitalization per year. The division into three score groups separated bronchiectasis into distinct mortality groups (mild: 3.7%; moderate: 20.7% and severe: 48.5% mortality; p