Actualización clínica y molecular de la telangiectasia hemorrágica hereditaria en Salamanca

[ES] Aproximación de la situación de una enfermedad minoritaria como es la Telangiectasia Hemorrágica Hereditaria a nivel del área de Salud de Salamanca. Descripción a nivel epidemiológico de la enfermedad, conocimiento de sus manifestaciones clínicas y realización de un estudio molecular de todas las familias afectadas. Una vez conocidas las mutaciones de las distintas familias se realiza una valoración de la relación del genotipo-fenotipo en los pacientes. Ante una patología hemorrágica, con mal control de sangrados y la necesidad de fármacos que actúen en la hemostasia, se completa el trabajo con un análisis de las distintas fases de la hemostasia, incluidos estudios de la función plaquetaria, donde se encuentra una disfunción en algunos de sus puntos

Efectos de las diferentes ondas de flujo y de la pausa teleinspiratoria sobre la oxigenación y ventilación en modelo animal

Clásicamente, la elección de modo ventilatorio en el neonato ha sido el flujo decelerado (DF) o ventilación mecánica por presión (VCP). Se ha sugerido que la onda de flujo decelerada mejora el intercambio de gases y la ventilación perfusión al compararla con la onda de flujo cuadrada (SF) o ventilación controlada por volumen (VCV). Aunque la superioridad de la DF comparada con la SF no ha sido todavía demostrada durante la ventilación de los neonatos. En el presente trabajo se han estudiado por primera vez los efectos sobre la oxigenación y ventilación de ambos flujos, decelerado y cuadrado, con y sin pausa tele- inspiratoria (EIP). Así como el efecto sobre la mecánica respiratoria y la hemodinámica, en modelo animal, sobre pulmón sano. Material y métodos: 12 cerdos recién nacidos fueron ventilados con los cuatro modos ventilatorios a estudio, SF,DF,SF-EIP y DF-EIP. Durante todos los modos ventilatrorios se mantuvieron constantes el volumen tidal (VT), la relación inspiración y espiración (I/E), la frecuencia respiratoria (FR) y la fracción inspirada de oxígeno (FiO2). Para asegurar un pulmón homogéneo y abierto durante el estudio, para evitar el colapso alveolar, aplicamos una presión positiva espiratoria (PEEP) de 6 cmH2O tras realizar maniobras de reclutamiento alveolar. Se recogieron parámetros de intercambio gaseoso, ventilatorios , de mecánica respiratoria y hemodinámicos. Resultados: La forma de la onda de flujo inspiratorio no tuvo efecto significativo sobre la oxigenación (PaO2) (276vs 278 mmHg, p=0.77), ni sobre la relación espacio muerto alveolar/volumen tidal alveolar (VDalv/VTalv) (0.21 vs 0.19 ml, p=0.33), ni en la presión media en vía aérea (Pawm) (13.1 vs 14.0, cm H2O, p=0.69) ni sobre la compliancia del sitema respiratorio (Csr) (3.5 vs 3.5 cmH2O, p=0.73) cuando comparamos SF y DF. Al añadir una EIP (10%) en ambos modos ventiltorios no se produjeron cambios en los resultados. Conclusión: El presente estudio muestra que no hay diferencias entre SF, DF, SF-EIP y DF-EIP en la oxigenación, ventilación, mecánica ventilatoria o hemodinámica en modelo de pulmón sano de cerdo recien nacido.Background: Historically, the elective ventilatory flow pattern for neonates has been decelerating flow (DF). Decelerating flow waveform has been suggested to improve gas exchange in the neonate when compared with square flow (SF) waveform by improving the ventilation perfusion. However, the superiority of DF compared with SF has not yet been demonstrated during ventilation in small infants. The aim of this study was to compare SF vs. DF, with or without end-inspiratory pause (EIP), in terms of oxygenation and ventilation in an experimental model of newborn piglets. Methods: The lungs of 12 newborn Landrace/LargeWhite crossbred piglets were ventilated with SF, DF, SF-EIP and DF-EIP. Tidal volume (VT), inspiratory to expiratory ratio (I/E), respiratory rate (RR), and FiO2 were keep constant during the study. In order to assure an open lung during the study while preventing alveolar collapse, a positive end-expiratory pressure (PEEP) of 6 cmH2O was applied after a single recruitment maneuver. Gas exchange, lung mechanics and hemodynamics were measured. Results: The inspiratory flow waveform had no effect on arterial oxygenation pressure (PaO2) (276 vs. 278 mmHg, p = 0.77), alveolar dead space to alveolar tidal volume (VDalv/VTalv) (0.21 vs. 0.19 ml, p = 0.33), mean airway pressure (Pawm) (13.1 vs. 14.0 cmH2O, p = 0.69) and compliance (Crs) (3.5 vs. 3.5 ml cmH2O−1, p = 0.73) when comparing SF and DF. A short EIP (10%) did not produce changes in the results. Conclusion: The present study showed that there are no differences between SF, DF, SF-EIP and DF-EIP in oxygenation, ventilation, lung mechanics, or hemodynamics in this experimental model of newborn piglets with healthy lungs. Keywords: Mechanical Ventilation, Pediatrics, Flow waveform, Oxygenation, Ventilation

Assessment of the Thermal Properties of Buildings in Eastern Almería (Spain) during the Summer in a Mediterranean Climate

Within a context in which temperatures are increasing due to global warming, it is important to assess the capacity of buildings, old and modern, to respond to this new situation. Recent studies have highlighted the importance of understanding more about the thermal properties of traditional constructions. This study quantifies the impact of the high summer temperatures typical of the Mediterranean climate on traditional farmhouses in Eastern Almería (Spain). The study group of farmhouses was divided into three models representative of the different types of Eastern Almería vernacular architecture. Energy consumption in the three models was simulated using EnergyPlus. The three models were assessed in free-floating conditions. The window-to-wall ratio and U-factor values were studied in order to evaluate potential benefits in terms of energy efficiency. Outdoor and indoor temperatures were compared. Finally, an adaptive thermal comfort analysis was performed according to ASHRAE 55. Results highlight the ability of Eastern Almería farmhouses to mitigate extreme temperatures.University of Granada and the Vicerrectorado de Investigación y Transferencia, with the project PP2022.PP.27 belonging to the Research and Transfer Pla

Phytoplankton photosynthesis-light relationship in the Pas estuary, Cantabria, Spain

ABSTRACT. The relationship between the photosynthesis-irradiance (P-I) curves of natural phytoplankton assemblages and environmental conditions was investigated in a partially mixed estuary, the Pas. During an annual cycle, photosynthesis was measured both by in situ and laboratory (light-saturation curve) incubation methods. The initial slope (aB) and the maximum photosynthetic rate (PDrnax) of P-I curves changed significantly over the year. The parameter (a" ranged from 0.001 to 0.04 mgC(mgCh1a) l h-' (FE m-' S l) and PBrnax ranged from 0.6 to 15 mgC(mgCh1a)-' h-'. Maxima for both aB and PBrnax occurred when the community of phytoplankton was dominated by small cell (small diatoms, Cryptornonas sp.) and the minima were obtained when the extinction coefficient was highest. In field (in situ) incubations, the production normalized to chlorophyll a (PDi) correlated positively with salinity and negatively with the nutrient concentration

Foreign accent strength and intelligibility at the segmental level

The relationship between strength of foreign accent and intelligibility is not straightforward. This relationship resists a simple characterisation due in part to the multiplicity of cues that carry accent in the word- and sentence-level materials typically used in the study of accent. One of the principal conveyors of accent is the phonetic segment. The current study attempts to isolate this segmental contribution to foreign accent and consequently measure the relationship between segmental accent and intelligibility for listeners with differing linguistic correspondence to the target and accented language. English, Spanish and Czech listeners identified English words in which the initial consonant was either intact, or had been replaced by a Spanish-accented counterpart; in a second task, they rated the accent strength of the same tokens. All speech material was produced by an English-Spanish bilingual talker. Overall, Spanish listeners displayed a smaller loss of intelligibility due to the accented segment than native English listeners, while the Czech cohort experienced the largest intelligibility loss. However, the relationship between accent strength and intelligibility loss was not linear, varying with phoneme identity and its role in a listener’s first language. These findings suggest that how accented and intelligible a sound is depends strongly on the interactions between the phonological systems of speakers and listener

Is segmental foreign accent perceived categorically?

The second language learning process involves acquisition of sounds that differ to varying degrees from the sounds of a learner’s native language. Learners’ productions are strongly influenced by their native language par- ticularly for sounds which are similar but non-identical in the two languages. However, foreign accent is typically investigated at the level of utterances and as a consequence the segmental basis of foreign accent and its role in communication remain unclear. The principal issues addressed by the current study are whether accentedness at the segmental level is judged categorically by native listeners, and whether consonantal and vocalic segments are treated similarly. British English listeners judged as native or non-native a series of words in which a single vowel or consonant had been replaced by its Spanish-accented counterpart. The degree of segment accent was varied in equal amounts along a 21-step continuum using a blending technique based on native and non-native segments excised from words spoken by a balanced bilingual talker. Listeners assessed 24 distinct consonant and vowel continua. Averaged across all vowel or consonant continua, listeners’ nativeness judgements varied with segment nativeness in a non-categorical fashion. However, most individual consonant continua, as well as those vowel continua that involved durational changes, were perceived categorically. These results suggest that while overall segment-level foreign accent might be considered to vary in degree, in reality at the level of individ- ual segments –where second language learners’ pronunciation awareness and control has to be focussed–small acoustic changes can convey a foreign accent.This work was funded by the Spanish MINECO project DIACEX (FFI2012-31597)

Trend analysis of CO2 and CH4 recorded at a semi-natural site in the northern plateau of the Iberian Peninsula

CO2 and CH4 were recorded from October 2010 to February 2016 with a Picarro G1301 analyser at the centre of the upper plateau of the Iberian Peninsula. Large CO2 values were observed during the vegetation growing season, and were reinforced by the stable boundary layer during the night. Annual CH4 evolution may be explained by ecosystem activity and by the dispersion linked with the evolution of the boundary layer. Their trends were studied using an equation that considers one polynomial and one harmonic part. The polynomial part revealed an increasing trend from 0.8 to 2.3 ppm year 1 for CO2 and from 0.004 to 0.011 ppm year 1 for CH4. The harmonic part considered four harmonics whose amplitudes were noticeable for the first and second harmonics for CO2 and for the first harmonic for CH4. Long-term evolution was similar with alternative equations. Finally, seasonal study indicated summer minima for both gases, which may be explained by the lack of vegetation in this season. Harmonic analysis showed two maxima for CO2, one in spring linked with vegetation growth, which decreased with time, and another in autumn related with the onset of plant activity after the summer, which increased with time. CH4 presented only one maximum in winter and a short time with steady concentration in spring where the evolution of the boundary layer may play a noticeable role. The harmonic equation, which takes into account all the observations, revealed opposite behaviour between CO2, whose minima decreased, and CH4, whose maxima increased.Ministry of Economy and Competitiveness and ERDF funds (projects CGL2009-11979 and CGL2014-53948-P)

Features of the annual evolution of CO2 and CH4 in the atmosphere of a Mediterranean climate site studied using a nonparametric and a harmonic function

Concentrations of CO2 and CH4 measured over 3 years at a rural site in the Spanish northern plateau were investigated together with vegetation and meteorological variables. Two procedures were implemented to study the annual evolution. Kernel estimation provided a detailed time description, and the harmonic model may be fitted easily. The site was characterised by grass from autumn to spring. However, vigorous growth was observed during the latter season due to the biological cycle of plants under favourable meteorological conditions. A CO2 peak was observed a fortnight before the time of maximum NDVI, and was attributed to the prevalence of respiration over photosynthesis. A pronounced trough was apparent in summer and was explained by the death of vegetation and active dispersion in a highly developed boundary layer. CH4 evolution was characterised by a deficit period from May to October, indicating that meteorological evolution played a key role. The harmonic model showed that annual and half-annual cycles evidenced a similar contribution for CO2, whereas said weight for the half-annual cycle was considerably smaller for CH4.Ministry of Economy and Competitiveness and ERDF funds (grant numbers CGL2009-11979 and CGL2014-53948-P

Influence of wind speed on CO2 and CH4 concentrations at a rural site

Producción CientíficaMeteorological variables have a noticeable impact on pollutant concentrations. Among these variables, wind speed is typically measured, although research into how pollutants respond to it can be improved. This study considers nine years of hourly CO2 and CH4 measurements at a rural site, where wind speed values were calculated by the METEX model. Nine wind speed intervals are proposed where concentrations, distribution functions, and daily as well as annual cycles are calculated. Contrasts between local and transported concentrations are around 5 and 0.03 ppm for CO2 and CH4, respectively. Seven skewed distributions are applied, and five efficiency criteria are considered to test the goodness of fit, with the modified Nash–Sutcliffe efficiency proving to be the most sensitive statistic. The Gumbel distribution is seen to be the most suitable for CO2, whereas the Weibull distribution is chosen for CH4, with the exponential function being the worst. Finally, daily and annual cycles are analysed, where a gradual decrease in amplitude is observed, particularly for the daily cycle. Parametric and nonparametric procedures are used to fit both cycles. The latter gave the best fits, with the agreement being higher for the daily cycle, where evolution is smoother than for the annual cycle.Ministerio de Economía, Industria y Competitividad y Fondo Europeo de Desarrollo Regional (FEDER) - (Projects CGL-2009-11979 and CGL2014-53948-P)Junta de Castilla y León - (Project VA027G19