13 research outputs found

    Impact of interstitial lung disease on the survival of systemic sclerosis with pulmonary arterial hypertension

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    To assess severity markers and outcomes of patients with systemic sclerosis (SSc) with or without pulmonary arterial hypertension (PAH-SSc/non-PAH-SSc), and the impact of interstitial lung disease (ILD) on PAH-SSc. Non-PAH-SSc patients from the Spanish SSc registry and PAH-SSc patients from the Spanish PAH registry were included. A total of 364 PAH-SSc and 1589 non-PAH-SSc patients were included. PAH-SSc patients had worse NYHA-functional class (NYHA-FC), worse forced vital capacity (FVC) (81.2 ± 20.6% vs 93.6 ± 20.6%, P < 0.001), worse tricuspid annular plane systolic excursion (TAPSE) (17.4 ± 5.2 mm vs 19.9 ± 6.7 mm, P < 0.001), higher incidence of pericardial effusion (30% vs 5.2%, P < 0.001) and similar prevalence of ILD (41.8% vs. 44.9%). In individuals with PAH-SSc, ILD was associated with worse hemodynamics and pulmonary function tests (PFT). Up-front combination therapy was used in 59.8% and 61.7% of patients with and without ILD, respectively. Five-year transplant-free survival rate was 41.1% in PAH-SSc patients and 93.9% in non-PAH-SSc patients (P < 0.001). Global survival of PAH-SSc patients was not affected by ILD regardless its severity. The multivariate survival analysis in PAH-SSc patients confirmed age at diagnosis, worse NYHA-FC, increased PVR, reduced DLCO, and lower management with up-front combination therapy as major risk factors. In conclusion, in PAH-SSc cohort risk of death was greatly increased by clinical, PFT, and hemodynamic factors, whereas it was decreased by up-front combination therapy. Concomitant ILD worsened hemodynamics and PFT in PAH-SSc but not survival regardless of FVC impairment

    Impact of interstitial lung disease on the survival of systemic sclerosis with pulmonary arterial hypertension

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    To assess severity markers and outcomes of patients with systemic sclerosis (SSc) with or without pulmonary arterial hypertension (PAH-SSc/non-PAH-SSc), and the impact of interstitial lung disease (ILD) on PAH-SSc. Non-PAH-SSc patients from the Spanish SSc registry and PAH-SSc patients from the Spanish PAH registry were included. A total of 364 PAH-SSc and 1589 non-PAH-SSc patients were included. PAH-SSc patients had worse NYHA-functional class (NYHA-FC), worse forced vital capacity (FVC) (81.2 +/- 20.6% vs 93.6 +/- 20.6%, P < 0.001), worse tricuspid annular plane systolic excursion (TAPSE) (17.4 +/- 5.2 mm vs 19.9 +/- 6.7 mm, P < 0.001), higher incidence of pericardial effusion (30% vs 5.2%, P < 0.001) and similar prevalence of ILD (41.8% vs. 44.9%). In individuals with PAH-SSc, ILD was associated with worse hemodynamics and pulmonary function tests (PFT). Up-front combination therapy was used in 59.8% and 61.7% of patients with and without ILD, respectively. Five-year transplant-free survival rate was 41.1% in PAH-SSc patients and 93.9% in non-PAH-SSc patients (P < 0.001). Global survival of PAH-SSc patients was not affected by ILD regardless its severity. The multivariate survival analysis in PAH-SSc patients confirmed age at diagnosis, worse NYHA-FC, increased PVR, reduced DLCO, and lower management with up-front combination therapy as major risk factors. In conclusion, in PAH-SSc cohort risk of death was greatly increased by clinical, PFT, and hemodynamic factors, whereas it was decreased by up-front combination therapy. Concomitant ILD worsened hemodynamics and PFT in PAH-SSc but not survival regardless of FVC impairment

    CARB-ES-19 Multicenter Study of Carbapenemase-Producing Klebsiella pneumoniae and Escherichia coli From All Spanish Provinces Reveals Interregional Spread of High-Risk Clones Such as ST307/OXA-48 and ST512/KPC-3

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    ObjectivesCARB-ES-19 is a comprehensive, multicenter, nationwide study integrating whole-genome sequencing (WGS) in the surveillance of carbapenemase-producing K. pneumoniae (CP-Kpn) and E. coli (CP-Eco) to determine their incidence, geographical distribution, phylogeny, and resistance mechanisms in Spain.MethodsIn total, 71 hospitals, representing all 50 Spanish provinces, collected the first 10 isolates per hospital (February to May 2019); CPE isolates were first identified according to EUCAST (meropenem MIC &gt; 0.12 mg/L with immunochromatography, colorimetric tests, carbapenem inactivation, or carbapenem hydrolysis with MALDI-TOF). Prevalence and incidence were calculated according to population denominators. Antibiotic susceptibility testing was performed using the microdilution method (EUCAST). All 403 isolates collected were sequenced for high-resolution single-nucleotide polymorphism (SNP) typing, core genome multilocus sequence typing (cgMLST), and resistome analysis.ResultsIn total, 377 (93.5%) CP-Kpn and 26 (6.5%) CP-Eco isolates were collected from 62 (87.3%) hospitals in 46 (92%) provinces. CP-Kpn was more prevalent in the blood (5.8%, 50/853) than in the urine (1.4%, 201/14,464). The cumulative incidence for both CP-Kpn and CP-Eco was 0.05 per 100 admitted patients. The main carbapenemase genes identified in CP-Kpn were blaOXA–48 (263/377), blaKPC–3 (62/377), blaVIM–1 (28/377), and blaNDM–1 (12/377). All isolates were susceptible to at least two antibiotics. Interregional dissemination of eight high-risk CP-Kpn clones was detected, mainly ST307/OXA-48 (16.4%), ST11/OXA-48 (16.4%), and ST512-ST258/KPC (13.8%). ST512/KPC and ST15/OXA-48 were the most frequent bacteremia-causative clones. The average number of acquired resistance genes was higher in CP-Kpn (7.9) than in CP-Eco (5.5).ConclusionThis study serves as a first step toward WGS integration in the surveillance of carbapenemase-producing Enterobacterales in Spain. We detected important epidemiological changes, including increased CP-Kpn and CP-Eco prevalence and incidence compared to previous studies, wide interregional dissemination, and increased dissemination of high-risk clones, such as ST307/OXA-48 and ST512/KPC-3

    Late-onset episodic ataxia type 2 associated with a novel loss-of-function mutation in the CACNA1A gene

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    We report a patient with typical features of episodic ataxia type 2 (EA2) but with onset in the sixth decade and associated interictal hand dystonia. He was found to bear the novel heterozygous missense mutation p.Gly638Asp (c.1913G > A) in the CACNA1A gene. Functional analysis of the mutation on P/Q channels expressed in HEK 293 cells revealed a reduction of Ca2+ current densities, a left-shift in the apparent reversal potential, the slowing of inactivation kinetics and the increase in the rate of current recovery from inactivation. These results are consistent with a decrease in Ca2+ permeability through mutant P/Q channels. To our knowledge, this is just the second patient with late onset EA2 linked to a CACNA1A mutation and the first to carry a loss-of-function missense mutation.This study was supported by the Spanish Ministry of Education and Science (SAF2003-04704, SAF2006-13893-C02-01, SAF2006-13893-C02-02, SAF2006-4973), Fondo de Investigación Sanitaria (red HERACLES), Fundació La Marató de TV3 (061330, 061331) and Agència de Gestió d'Ajuts Universitaris i de Recerca-AGAUR (2005SGR00848 and 2005SGR00266). RC was funded by the Institut de Recerca Vall d'Hebron, MR and EC-L are recipients of a Juan de la Cierva and a Marató de TV3 contracts, respectively, and SAS is a recipient of a FPI scholarship of the Spanish Ministry of Education and Science. JMF-F is a Ramón y Cajal Fellow. MAV is an ICREA Academia Fellow

    La contaminación debida a actividades humanas y su remediación : concienciación y la responsabilidad ambiental orientadas para alumnos de Primaria

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    Se presenta un proyecto que pretende introducir a los alumnos en la problemática medioambiental y la contaminación de las aguas y atmosférica. Se incide principalmente en el problema del cambio climático debido a las emisiones de CO2, y su remediación. También, las posibles soluciones a estas situaciones, incidiendo en que el conocimiento científico y tecnológico y la concienciación son las mejores armas para disminuir y prevenir estos problemas. Se utilizan las herramientas tecnológicas (TIC) adecuadas para la profundización, producción y difusión de las experiencias que se desarrollan en seminarios teóricos y actividades prácticas tales como un taller de concienciación, reciclado de papel, aplicaciones informáticas en la Web 2.0, lecturas relacionadas con el tema medioambiental o visitas al medio como fuente potencial de experiencias vividas y desarrollo de las interpretaciones científicas; además de otras actividades complementarias que sirven para fijar los conceptos trabajados durante el proyecto: planificar y realizar actividades de experimentación del medio y en el medio para interpretar el entorno y los fenómenos que en él acontecen; conocer y utilizar adecuadamente los elementos constituyentes de un laboratorio escolar, así como el material empleado; conocer normas básicas de seguridad e higiene en el laboratorio escolar; integrar los contenidos del proyecto en otros planes y actuaciones que se desarrollan en el centro: plan de lectura, lengua extranjera o incorporación de las TIC en las actuaciones docentes. Los alumnos que participan son en su mayoría de quinto y sexto de curso de Primaria, así como el profesorado directamente implicado en estos cursos. La investigación se adapta a la realidad del entorno del Centro y a las necesidades en relación a los objetivos. La flexibilidad en los procedimientos permite conectar directamente con los intereses de los alumnos y conjugarlos a la vez con las metas propuestas. Tanto el profesorado como el alumnado implicado en el proyecto toman conciencia de la problemática ambiental analizada, tomando decisiones en cuanto a actuaciones prácticas en el Centro y en la vida fuera de él: ahorro energético, reciclaje, concienciación en el entorno familiar. Además se consigue un mayor conocimiento sobre los aspectos tecnológicos y los procesos de producción de energía.Castilla La ManchaConsejería de Educación, Ciencia y Cultura. Viceconsejería de Educación y Cultura. Servicio de Documentación; Bulevar del Río Alberche, s. n. - 1 Planta; 45071 Toledo; +34925286045; +34925247410; [email protected]

    Investigación educativa en las aulas de primaria

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    Reúne trabajos derivados de la experiencias de diversos docentes en educación primaria en los siguientes temas: Tecnología de Información y Comunicación, educación inclusiva, enseñanza de la música, educación física, enseñanza de la historia, acoso escolar, auto-evaluación, métodos de enseñanza, inteligencia emocional, percepción del alumno, marco cognitivo en comprensión lectora y comunicación escuela-familia

    Todos muy capaces

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    El trabajo obtuvo un Premio Tomás García Verdejo a las buenas prácticas educativas en la Comunidad Autónoma de Extremadura para el curso académico 2015/2016. Modalidad BSe describe un proyecto llevado a cabo con alumnos de 4º de diversificación y 2º de Formación Profesional Básica del IES Bioclimático de Badajoz, que consistió en realizar diversas actividades con la asociación Aspaceba (Asociación de Parálisis Cerebral de Badajoz) y que tuvo como objetivos principales que los alumnos se informase sobre la discapacidad en general y sobre la parálisis cerebral en particular, que se acercaran al mundo de la discapacidad superarando las barreras físicas y psicológicas, que conociesen los aspectos positivos y las necesidades de apoyo y recursos de las personas con discapacidad, que trabajasen la empatía y el respeto como mecanismo para entender sentimientos y vivencias de los demás y que rechazaran situciones de injusticia hacia personas con alguna discapacidadAndalucíaES

    Spanish Catheter Ablation Registry. 18th Official Report of the Spanish Society of Cardiology Working Group on Electrophysiology and Arrhythmias (2018)

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