Health-related quality of life in hereditary transthyretin amyloidosis polyneuropathy: a prospective, observational study

Background Hereditary Transthyretin Amyloidosis Polyneuropathy is a rare life-threatening neurologic disease that imposes considerable mortality and it is associated with progressive related disabilities. In this study, we aimed to assess the effect of the disease across health-related quality of life dimensions, in both carriers of the mutation and patients, to compare health-related quality of life with general population, as well as to explore health-related quality of life prognostic factors among patients, including disease progression and treatment. Methods This study was a multi-institutional, longitudinal, prospective, observational study of hereditary Transthyretin Amyloidosis Polyneuropathy Portuguese adult subjects (621 asymptomatic carriers and 733 symptomatic patients) enrolled in the Transthyretin Amyloidosis Outcomes Survey. Health-related quality of life was captured with the preference-based instrument EQ-5D-3 L. For general population the dataset included all subjects enrolled in a representative national study (n = 1500). Different econometric models were specified; multivariate probit, generalized linear model and generalized estimating equations model; including demographic and clinical covariates. Results Hereditary Transthyretin Amyloidosis Polyneuropathy patients have their health status severely impaired in all quality of life dimensions and more anxiety/depression problems were found among asymptomatic carriers. No differences on utility were found between carriers and general population (p = 0.209). Among patients, the utility value is estimated to be 0.51 (0.021), a decrement of 0.27 as compared with general population utility. Higher disease duration, advanced disease stage and not receiving treatment are associated with impaired health-related quality of life. No differences were found between genders (p = 0.910) or between late (≥50 years) and early-onset patients (p = 0.254). The utility estimate ranged from 0.63 (0.009) in stage I to 0.01 (0.005) in stage IV. Conclusions Hereditary Transthyretin Amyloidosis Polyneuropathy symptoms and progressive associated disabilities substantially decrease patient’s health-related quality of life. Clinical strategies focused on health-related quality of life preservation such as close follow-up of asymptomatic carriers, prompt diagnosis and adequate, early treatment would benefit patient’s long-term outcomes, slowing the progressive decline in health-related quality of life.info:eu-repo/semantics/publishedVersio

Arquitectura eficiente. Edificio El Rondo de Markus Pernthaler

[EN] Alejandro Zaera, by making a reference to Naomi Klein s book Capitalism vs. Climate , said in one of his many conferences that whoever would solve that equation, would control the world. He said this in front of an audience that was mainly composed of architecture students. And with good reason, since the energy and cost of constructing and maintaining a building is a matter of big rather than small numbers; its environmental impact can reach a high degree of importance, which is why the architect is a key player within this delicate game of the future of the planet. It is within this unknown of Klein that lie the past, present and particularly the future of architecture: within the equilibrium between quality and need, art and construction, an engineer s square and an architect s icosahedron. In the present paper, this struggle is brought to light by a specific building: El Rondo. Made by the architect Markus Pernthaler and located in Graz (Austria), it is a construction whose way of dealing with the energy and space make of it an example for others to follow. This is why this paper focuses on the study and analysis of the building s energetic efficiency, as well as on the techniques and constructive solutions used for the making of the different habitable and working spaces that make of this building an icon of its city. Finally, there is a conclusion with a thought about the importance of the sustainable and intelligent design within the framework of the 21st Century s architecture and the role that it could play in the future of the profession.[CA] Alejandro Zaera decía en una de sus muchas conferencias, haciendo referencia al libro de Naomi Klein Capitalismo vs. Clima , que quien resolviese esa ecuación, controlaría el mundo. Y lo decía ante un público formado esencialmente por estudiantes de arquitectura. Y es que, no en vano, la energía y el gasto que supone levantar y mantener un edificio, no se mide en pequeñas sino en grandes cifras; su impacto medioambiental alcanza elevadas cuotas y por ello, el arquitecto es una pieza clave en el delicado juego del futuro del planeta. En la incógnita de Klein reside el pasado, el presente y especialmente el futuro de la arquitectura: en el equilibrio entre la calidad y la necesidad, entre el arte y la construcción, entre el cuadrado de un ingeniero y el icosaedro de un arquitecto. En este trabajo, esta lucha se pone de manifiesto en un edificio concreto: El Rondo. Se trata de una construcción del arquitecto Markus Pernthaler ubicada en Graz (Austria), cuyo tratamiento de la energía y del espacio la convierten en un ejemplo a seguir. Es por ello por lo que el trabajo se centra en el estudio y análisis de la eficiencia energética del edificio, así como las técnicas y soluciones constructivas empleadas en la realización de los diferentes espacios habitables y de trabajo, que hacen de este edificio un icono de su ciudad. Por último, se concluye con una reflexión sobre la importancia del diseño sostenible e inteligente en el marco de la arquitectura del siglo XXI y el papel que puede llegar a desempeñar en el futuro de la profesión.Lara Sánchez De Puerta, T. (2018). Arquitectura eficiente. Edificio El Rondo de Markus Pernthaler. http://hdl.handle.net/10251/115335TFG

COVID-19 : Up to 82% critically ill patients had low Vitamin C values

Funding: Funding Support was provided solely from institutional and/or departmental sources. No funding was provided from any of the following organizations: National Institutes of Health (NIH), Wellcome Trust, Howard Hughes Medical Institute (HHMI).There are limited proven therapeutic options for the prevention and treatment of COVID-19. We underwent an observational study with the aim of measure plasma vitamin C levels in a population of critically ill COVID-19 adult patients who met ARDS criteria according to the Berlin definition. This epidemiological study brings to light that up to 82% had low Vitamin C values. Notwithstanding the limitation that this is a single-center study, it nevertheless shows an important issue. Given the potential role of vitamin C in sepsis and ARDS, there is gathering interest of whether supplementation could be beneficial in COVID-19

Growth rate of technician adolescent athletes of swimming, water polo, diving and synchronized swimming

El objetivo de este estudio longitudinal fue hacer un análisis de la evolución del crecimiento de deportistas de ambos sexos, de 11 a 18 años de edad, en la especialización de natación, waterpolo, saltos y natación sincronizada, pertenecientes a un Centro de Tecnificación y evaluados en un Centro de Medicina Deportiva. A lo largo de dos años fueron recogidas las medidas antropométricas de estatura (cm) y peso (kg), la respectiva fecha de medición y la fecha de nacimiento de los deportistas. Fueron calculadas en el programa estadístico SPSS 17.0 para análisis estadístico descriptivo las medias de estatura y peso; la velocidad de crecimiento (cm/año) y las edades medias cronológicas que tenían los deportistas entre las recogidas de datos. Los gráficos fueron realizados en el programa de computador OriginPro7. Se utilizó la Evaluación Criterial para evaluar y ubicar los deportistas según las medias presentadas. En general la velocidad de crecimiento de los deportistas estuvo por encima del percentil 50 de las curvas y tablas para la población española general por lo que la variable especialización deportiva influye significativamente en esta diferencia con la población general. Palabras claves. Velocidad de crecimiento, natación, waterpolo, saltos, natación sincronizadaThe aim of this longitudinal study is to analyze the evolution of growth of athletes, boys and girls, 11 to 18 years old, specializing in swimming, water polo, diving and synchronized swimming, belonging to a Technical Center and evaluated in a Medical Sports Center. Over two years were collected anthropometric measurements of height and weight, the respective measurement date and the date of birth of athletes. They were calculated in SPSS 17.0 statistical software for statistical analysis descriptive the means of their height and weight; the rate of increase growth and the chronological middle ages that had athletes between one data collection and other. The graphics were made in the computer program OriginPro7. The «Evaluación Criterial» was used to evaluate and locate the athletes according to averages presented. Overall growth rate of athletes were above the 50th percentile of the curves and tables for the general Spanish populatio

Prácticas tutoriales en la facultad de ciencias de la comunicación de la UANL.

Fue realizado un estudio relacionado con las prácticas de tutoría por los profesores asignados, en la Facultad de Ciencias de la Comunicación de la UANL, cuyo objetivo general fue conocer la opinión personal de los estudiantes sobre la realización de las prácticas de tutoría y el desempeño de sus profesores, a través de las entrevistas grupales e individuales

Propuesta de la licenciatura en periodismo multimedia

El Objetivo general consiste en dar a conocer el diseño y el documento en extenso del Programa Educativo de la Licenciatura en Periodismo Multimedia. Los objetivos específicos son dar a conocer la fundamentación de este PE y sus 4 ejes, el programa educativo completo, la malla curricular, el perfil de ingreso y egreso, las opiniones de los encuestados y entrevistados, pues estas últimas sustentan la propuesta para su aceptación, autorización e implementación, por las autoridades universitarias correspondientes

Antioxidant system of ginseng under stress by cadmium

Toxic levels of Cd can cause protein denaturation and oxidative stress, which result in membrane damage, enzimatic activity changes and other metabolic damage. Some plants may show alteration in the activity of their antioxidant enzymes as a heavy metal tolerance mechanism. This study aims at evaluating the role of enzymes of the antioxidant system in adaptive responses of the accumulator P. glomerata species to levels of cadmium (Cd). Plants were cultivated in nutrient solutions containing concentrations of Cd in the form of CdSO4 (0, 45 and 90 µmol L-1), for 20 d. Cd concentrations and yields of root and shoot dry matter were determined at the end of the experiment. Malondialdehyde (MDA) production and the activities of antioxidant enzymes were determined after days 1, 12 and 20. Higher Cd concentrations in tissues of P. glomerata were found to reduce biomass production in both roots and shoots. The lipid peroxidation rates in leaves and roots were smaller at the start of the experiment for all Cd levels. Superoxide dismutase (SOD) activity increased in leaves on day 1 and in roots on day 20 as Cd levels increased. Cd stress induced an increase in the activity of APX in leaves, whereas in roots ascorbate peroxidase (APX) activity was reduced at high concentration of Cd. At the end of the experiment, catalase (CAT) activity in leaves was reduced as Cd concentration increased. Nevertheless, the glutathione reductase (GR) and guaiacol peroxidase (GPX) activities increased. In roots, GR activity was reduced on days 1 and 20

Relative fascicle excursion effects on dynamic strength generation during gait in children with cerebral palsy

Evaluation of muscle structure gives us a better understanding of how muscles contribute to force generation which is significantly altered in children with cerebral palsy (CP). While most muscle structure parameters have shown to be significantly correlated to different expressions of strength development in children with CP and typically developing (TD) children, conflicting results are found for muscle fascicle length. Muscle fascicle length determines muscle excursion and velocity, and contrary to what might be expected, correlations of fascicle length to rate of force development have not been found for children with CP. The lack of correlation between muscle fascicle length and rate of force development in children with CP could be due, on the one hand, to the non-optimal joint position adopted for force generation on the isometric strength tests as compared to the position of TD children. On the other hand, the lack of correlation could be due to the erroneous assumption that muscle fascicle length is representative of sarcomere length. Thus, the relationship between muscle architecture parameters reflecting sarcomere length, such as relative fascicle excursions and dynamic power generation, should be assessed. Understanding of the underlying mechanisms of weakness in children with CP is key for individualized prescription and assessment of muscle-targeted interventions. Findings could imply the detection of children operating on the descending limb of the sarcomere length–tension curve, which in turn might be at greater risk of developing crouch gait. Furthermore, relative muscle fascicle excursions could be used as a predictive variable of outcomes related to crouch gait prevention treatments such as strength training

El inicio de las Relaciones Públicas como herramienta de calidad en Tutoría en el año 2015. Estudio de Caso: Universidad Autónoma de Nuevo León - Facultad de Ciencias de la Comunicación.

La Facultad de Ciencias de la Comunicación contempla en su Plan de Desarrollo los lineamientos generales que la UANL sustenta en su Visión 2020, de crear las mejores prácticas en la gestión administrativa y docente que realizan las dependencias guiadas por los indicadores de calidad que cada una de ellas aplica, de acuerdo a su área de Conocimiento por los organismos especializados, que evalúan sus procesos de gestión

Evaluación de Relaciones Públicas con Tutoría y la calidad en el servicio en el año 2016. Estudio de Caso: Universidad Autónoma de Nuevo León-Facultad de Ciencias de la Comunicación.

En la Coordinación de Tutoría de la FCC de la UANL se ha considerado la implementación de las Relaciones Públicas como una herramienta para vincular los profesores tutores y los estudiantes para cumplir con los objetivos estipulados primeramente en el Plan de Desarrollo Institucional de nuestra máxima casa de estudios en la Visión 2020, (Visión 2020) y la Dirección de Formación Integral al Estudiante (DEFIE), cuyo objetivo es administrar la gestión de Tutoría en las 26 facultades de la UANL, las preparatorias y planteles que la conforman