50 research outputs found

    Changes in ventilation distribution in children with neuromuscular disease using the insufflator/exsufflator technique: an observational study.

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    Patients with neuromuscular disease often suffer from weak and ineffective cough resulting in mucus retention and increased risk for chest infections. Different airway clearance techniques have been proposed, one of them being the insufflator/exsufflator technique. So far, the immediate physiological effects of the insufflator/exsufflator technique on ventilation distribution and lung volumes are not known. We aimed to describe the immediate effects of the insufflator/exsufflator technique on different lung volumes, forced flows and ventilation distribution. Eight subjects (age 5.8-15.2 years) performed lung function tests including spirometry, multiple breath washout and electrical impedance tomography before and after a regular a chest physiotherapy session with an insufflator/exsufflator device. Forced lung volumes and flows as well as parameters of ventilation distribution derived from multiple breath washout and electrical impedance tomography were compared to assess the short-term effect of the therapy. In this small group of stable paediatric subjects with neuromuscular disease we could not demonstrate any short-term effects of insufflation/exsufflation manoeuvres on lung volumes, expiratory flows and ventilation distribution. With the currently used protocol of the insufflation/exsufflation manoeuvre, we cannot demonstrate any immediate changes in lung function

    Association of lung clearance index with survival in individuals with cystic fibrosis.

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    BACKGROUND The lung clearance index (LCI) assesses global ventilation inhomogeneity and is a sensitive biomarker of airway function in cystic fibrosis (CF) lung disease. OBJECTIVES We examined the association of LCI with the risk of death or lung transplantation (LTX) in individuals with CF. METHODS We performed a retrospective analysis in a cohort of individuals with CF aged≥5 years with LCI and FEV1 measurements performed between 1980 and 2006. The outcome was time until death or LTX. We used the earliest available LCI and FEV1 values in a Cox proportional hazard regression adjusted for demographic and clinical variables. For sensitivity analyses, we used the mean of the first three LCI and FEV1 measurements, stratified the cohort based on age, and investigated individuals with normal FEV1. RESULTS In total, 237 individuals with CF with a mean (range) age of 13.9 (5.6-41.0) years were included. The time-to-event analysis accrued 3813 person-years and 94 (40%) individuals died or received LTX. Crude hazard ratios [95% CI] were 1.04 [1.01-1.06] per one z-score increase in LCI and 1.25 [1.11-1.41] per one z-score decrease in FEV1. After adjusting LCI and FEV1 mutually in addition to sex, age, BMI and the number of hospitalisations, hazard ratios were 1.04 [1.01-1.07] for LCI, and 1.12 [0.95-1.33] for FEV1. Sensitivity analyses yielded similar results and using the mean LCI strengthened the associations. CONCLUSIONS Increased ventilation inhomogeneity is associated with greater risk of death or LTX. Our data support LCI as novel surrogate of survival in individuals with CF

    Índice de progreso social del distrito de San Luis

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    En las últimas décadas han sido varios los esfuerzos que se han realizado para medir el progreso de las naciones, desarrollando indicadores que aseguren una comparabilidad entre ellas. Uno de los indicadores más utilizados ha sido el Producto Bruto Interno (PBI), orientado principalmente a medir el desempeño económico. En el 2012 se fundó el Social Progress Imperative, con la misión de desarrollar el Índice de Progreso Social (IPS). A partir del análisis de tres dimensiones: Necesidades Humanas Básicas, Fundamentos del Bienestar y Oportunidades; y con énfasis en el Progreso Social, busca ser un indicador viable que mida los resultados de las políticas y de las acciones gubernamentales y privadas, antes que el volumen de las inversiones; y que sirva como herramienta de gestión de políticas públicas que aseguren una mejor calidad de vida de los habitantes de sus localidades. La medición realizada en el distrito de San Luis dio como resultado un IPS de 61.87 ubicándolo en un nivel de progreso social medio bajo. El análisis permitió identificar que el distrito ha logrado satisfacer las Necesidades Humanas Básicas de sus ciudadanos como acceso a agua, saneamiento y vivienda; mientras que tiene un desempeño sobresaliente en la cobertura de los Fundamentos de Bienestar, como es el caso de la sostenibilidad ambiental. Sin embargo, ello contrasta con la deficiente gestión de las Oportunidades necesarias para que los ciudadanos logren potenciar el bienestar. La gestión municipal del distrito de San Luis ha emprendido un plan de desarrollo local que incluye ocho variables estratégicas las cuales contemplan el manejo de cuatro de las cinco brechas identificadas en el presente estudio. Dicho plan define acciones e indicadores específicos para el manejo de los componentes de Seguridad Personal, Tolerancia e Inclusión, Derechos Personales y Vivienda. No obstante, no contempla acciones para la mejora del Acceso a Educación Superior, componente que se agrava por la existencia de grupos vulnerables desatendidos en la localidadIn the last few decades, many efforts have been made in order to measure nation's progress, developing indices that enable comparisons between them. The most widely used indicator over the years has been the Gross Domestic Product (GDP), which mainly measures the economic growth. The Social Progress Imperative was founded in 2012, with the primary goal to create the Social Progress Index. It has three dimensions: basic human needs, foundations of wellbeing and opportunity; and with emphasis in social progress seeks to create a viable index that measures public policies and public/private efforts results rather than investment, and to serve as a tool for public management to ensure a better quality of life of their community. The survey performed on San Luis district resulted in a SPI of 61.87, which ranked San Luis as a middle lower location. The analysis identified that San Luis achieved to satisfy Basic Human Needs, like water and sanitation and shelter; and an outstanding performance in the Foundations of Wellbeing, like Ecosystem Sustainability. However, it contrasts with a poor management in the Opportunity dimension, necessarily for their population to enhance their wellbeing. The municipal management of the San Luis district has established a local development plan, which include eight strategic variables that consider four of the five gaps identified in this study. The plan defines actions and specific indices to address the Personal Safety, Tolerance and Inclusion, Personal Rights and Shelter. Nevertheless, it doesn´t include actions to improve Access to Advanced Education, component exacerbated by the existence of neglected and vulnerable groups in the communityTesi

    Afiliaci?n y no afiliaci?n sindical

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    Esta investigaci?n, tiene como objetivo principal entender y conocer cu?les son los principales factores de motivaci?n (intr?nsecos) e higiene (extr?nsecos) que influyen en la afiliaci?n o no afiliaci?n sindical. As? tambi?n tiene como objetivos espec?ficos conocer el detalle de cada una de estas motivaciones y diferenciarlas considerando cada una de las muestras, es decir, el p?blico de afiliados y el p?blico de no afiliados. Es as? que esta investigaci?n demuestra que, para ambos p?blicos, las motivaciones de afiliaci?n y no afiliaci?n al sindicato est?n relacionadas en su mayor?a con factores de higiene (extr?nsecos). Cabe mencionar que los criterios m?s representativos para afiliarse al sindicato son beneficios laborales del sindicato, l?deres sindicales no influyentes, motivaci?n familiar ? Sindicato y retribuci?n econ?mica no conforme; y los criterios extr?nsecos m?s representativos para no afiliarte al sindicato son injusticia (Sindicato solo apoya a sindicato), l?deres sindicales no influyentes, retribuci?n econ?mica conforme y sindicato no da estabilidad laboral. Finalmente, se puede concluir que los factores extr?nsecos son los que generan mayor motivaci?n en ambos p?blicos, por lo que gestionar estos aspectos desde recursos humanos permitir?a lograr un mejor entendimiento de las motivaciones de sus trabajadores y tomar las acciones para una adecuada gesti?n

    Data accuracy, consistency and completeness of the national Swiss cystic fibrosis patient registry: Lessons from an ECFSPR data quality project.

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    BACKGROUND Good data quality is essential when rare disease registries are used as a data source for pharmacovigilance studies. This study investigated data quality of the Swiss cystic fibrosis (CF) registry in the frame of a European Cystic Fibrosis Society Patient Registry (ECFSPR) project aiming to implement measures to increase data reliability for registry-based research. METHODS All 20 pediatric and adult Swiss CF centers participated in a data quality audit between 2018 and 2020, and in a re-audit in 2022. Accuracy, consistency and completeness of variables and definitions were evaluated, and missing source data and informed consents (ICs) were assessed. RESULTS The first audit included 601 out of 997 Swiss people with CF (60.3 %). Data quality, as defined by data correctness ≥95 %, was high for most of the variables. Inconsistencies of specific variables were observed because of an incorrect application of the variable definition. The proportion of missing data was low with 5 % of missing documents). After providing feedback to the centers, availability of genetic source data and ICs improved. CONCLUSIONS Data audits demonstrated an overall good data quality in the Swiss CF registry. Specific measures such as support of the participating sites, training of data managers and centralized data collection should be implemented in rare disease registries to optimize data quality and provide robust data for registry-based scientific research
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