La protección de los derechos al honor, a la intimidad y a la propia imagen de los menores de edad frente a los medios de comunicación

Este trabajo tiene como objetivo analizar la protección reforzada que existe en el ordenamiento jurídico español sobre los derechos al honor, a la intimidad y a la propia imagen de los menores de edad frente a las intromisiones producidas por los medios de comunicación, además de dar a conocer el importante papel que en este ámbito desempeña el Ministerio Fiscal para proteger dichos derechos, así como averiguar, y mostrar, a través de senencias dictadas por el TS, qué situaciones se producen en la práctica y llegan a la vía judicial, y la respuesta de la jurisprudencia frente a ellas

Integració d'eines de simulació de xarxes en un entorn virtual

L'objecte d'estudi d'aquest projecte és la integració d'eines de simulació en entorns virtuals. Per a dur a terme aquest estudi i al llarg del present treball, en primer lloc, es farà una breu introducció als fonaments teòrics generals de sistemes, models i simulació de sistemes. Tot seguit, es realitzarà l'estat de l'art de diferents simuladors, valorant-ne les diferents alternatives proposades, tot indicant-ne els avantatges i inconvenients detectats. D'aquesta manera es definirà i contextualitzarà el marc de treball. Finalment, s'aprofundirà en la simulació d'un conjunt d'escenaris proposats. Mitjançant Opnet IT Guru Academic Edition 9.1 es realitzarà l'estudi sobre l'efecte que poden tenir els diferents planificadors, cues FIFO, amb prioritat i equitatives (FQ, WFQ), en la transmissió de paquets per a diferents serveis, en els escenaris anteriorment esmentats. De la mateixa manera s'abordarà l'estudi de l'efecte de l'ús del Protocol de Reserva de Recursos (RSVP) com a part de l'enfocament dels serveis integrats IntServ per a proporcionar qualitat de servei (QoS). Així, s'analitzarà la manera en com el protocol RSVP pot contribuir en el rendiment de les aplicacions que l'utilitzen.El objeto de estudio de este proyecto es la integración de herramientas de simulación en entornos virtuales. Para llevar a cabo este estudio y a lo largo del presente trabajo, en primer lugar, se hará una breve introducción a los fundamentos teóricos generales de sistemas, modelos y simulación de sistemas. A continuación, se realizará el estado del arte de diferentes simuladores, valorando las diferentes alternativas propuestas, indicando las ventajas e inconvenientes detectados. De este modo se definirá y contextualizará el marco de trabajo. Finalmente, se profundizará en la simulación de un conjunto de escenarios propuestos. Mediante Opnet IT Guru Academic Edition 9.1 se realizará el estudio sobre el efecto que pueden tener los diferentes planificadores, colas FIFO, con prioridad y equitativas (FQ, WFQ), en la transmisión de paquetes para diferentes servicios, en los escenarios anteriormente mencionados. Del mismo modo se abordará el estudio del efecto del uso del Protocolo de Reserva de Recursos (RSVP) como parte del enfoque de los servicios integrados IntServ para proporcionar calidad de servicio (QoS). Así, se analizará la manera en como el protocolo RSVP puede contribuir en el rendimiento de las aplicaciones que lo utilizan.This project deals with the integration of simulation tools in virtual environments. To carry out this study and throughout this paper, first there will be drafted a brief introduction to general theoretical systems, models and simulation. Then, there will be state of the art of different simulators, evaluating different alternatives, giving the advantages and disadvantages identified. This will define and contextualize the framework. Finally, we look into the simulation of a proposed set of scenarios. By Opnet IT Guru Academic Edition 9.1 will be conducted the study on the effect of using different planners, FIFO queues, priority and fair (FQ, WFQ), packet switching for different services. Equally we will study the effect of using Resource Reservation Protocol (RSVP) as part of an integrated services approach IntServ to provide quality of service (QoS). Thus, we will analyze the way in which the RSVP protocol may contribute to the performance of applications which use it

Development and fine-tuning of an artificial organoleptic analysis method for virgin olive oil using an electronic olfactory system

Motivation: The catalogation of olive oil by organoleptic parameters has a high grade of subjection because of human factor. A new system of electronic tasting is used as a reliable, fast and cheap method. The use of an electronic olfatory system (EOS), which consist of an array of electonic sensors and a mechanism for pattern recognition, requires of a previous training process like a human olfatory system. The training is one of the most relevant and delicated phase, as this process involves the creation of a complete database that the instrument uses as a reference for the subsequent sample recognition.Methods: A set of samples of different olive oils were analysed by three official panel tests before using the EOS. The instrument is composed of a chamber with five metal oxide semiconductor based sensors. During the analysis, each sensor is maintained at a specific temperature showing a different response for a certain sample, which implies a reduction in the sensors resistance. For the measurement, volatile compounds were directed to the sensor chamber by the carrier gas (dry air). Principal component analyses to interpret the datasets from the EOS is employed in order to assess the classification of the measurements.A database was created through the analysis of a set of samples which should be representative of differents aromas to be recognized.Conclusions: The main conclussion is the high variability between the official panel tests which could be reduced by the EOS. From the database that was created we can analyse an olive oil with unknown catalogation and classify into Extra Virgin, virgin or lampante.On-line detection of the different categories, during the oil industrial process, could have a notable economical impact

Exact exchange-correlation potential for a time-dependent two electron system

We obtain an exact solution of the time-dependent Schroedinger equation for a two-electron system confined to a plane by an isotropic parabolic potential whose curvature is periodically modulated in time. From this solution we compute the exact time-dependent exchange correlation potential v_xc which enters the Kohn-Sham equation of time-dependent density functional theory. Our exact result provides a benchmark against which various approximate forms for v_xc can be compared. Finally v_xc is separated in an adiabatic and a pure dynamical part and it is shown that, for the particular system studied, the dynamical part is negligible.Comment: 23 pages, 6 figure

Different Approaches to Analyze Muscle Fat Replacement With Dixon MRI in Pompe Disease

Altres ajuts: Asociación Española de Enfermos de Glucogenosis (AEEG)Quantitative MRI is an increasingly used method to monitor disease progression in muscular disorders due to its ability to measure changes in muscle fat content (reported as fat fraction) over a short period. Being able to objectively measure such changes is crucial for the development of new treatments in clinical trials. However, the analysis of the images involved continues to be a daunting task because of the time needed. Whether a more specific analysis selecting individual muscles or a global one analyzing the whole thigh or compartments could be a suitable alternative has only been marginally studied. In our study we compare three methods of analysis of 2-point-dixon images in a cohort of 34 patients with late onset Pompe disease followed over a period of one year. We measured fat fraction on MRIs obtained at baseline and at year 1, and we calculated the increment of fat fraction. We correlated the results obtained with the results of muscle function tests to investigate whether the three methods of analysis were equivalent or not. We observed significant differences between the three methods in the estimation of the fat fraction at both baseline and year 1, but no difference was found in the increment in fat fraction between baseline and year 1. When we correlated the fat fraction obtained with each method and the muscle function tests, we found a significant correlation with most tests in all three methods, although in most comparisons the highest correlation coefficient was found with the analysis of individual muscles. We conclude that the fastest strategy of analysis assessing compartments or the whole thigh could be reliable for certain cohorts of patients where the variable to study is the fat increment. In other sorts of studies, an individual muscle approach seems the most reliable technique

Correlation Between Respiratory Accessory Muscles and Diaphragm Pillars MRI and Pulmonary Function Test in Late-Onset Pompe Disease Patients

Objectives: Pompe disease is a rare genetic disease produced by mutations in the GAA gene leading to progressive skeletal and respiratory muscle weakness. T1-weighted magnetic resonance imaging is useful to identify fatty replacement in skeletal muscles of late-onset Pompe disease (LOPD) patients. Previous studies have shown that replacement by fat correlates with worse results of muscle function tests. Our aim was to investigate if fat replacement of muscles involved in the ventilation process correlated with results of the spirometry and predicted respiratory muscle impairment in LOPD patients over time. Materials and Methods: We studied a cohort of 36 LOPD patients followed up annually in our center for a period of 4 years. We quantified muscle fat replacement using Mercuri score of the thoracic paraspinal and abdominal muscles and the pillars of the diaphragm. We correlated the combined Mercuri scores of these areas with spirometry results and the need of respiratory support. Results: We found a statistically significant correlation (Spearman test, p 0.6) between forced vital capacity seated and lying and fat fraction score of all muscle groups studied. The group of patients who needed respiratory support had higher fat fraction scores than patients not requiring ventilatory support. Higher fat replacement in these areas correlated with worse progression in spirometry values over time. Conclusions: Fat replacement of paraspinal, abdominal, and trunk muscles correlates with results of spirometry and is able to predict worsening in respiratory muscle function tests that could lead to an emerging ventilatory dysfunction. Therefore, the identification of fat replacement in these muscle groups should lead to a closer monitorization of patients. Radiologic evaluation of diaphragm pillars in T1-weighted imaging axial sequences could also be helpful to predict respiratory insufficiency

Quantitative muscle MRI to follow up late onset Pompe patients : a prospective study

Late onset Pompe disease (LOPD) is a slow, progressive disorder characterized by skeletal and respiratory muscle weakness. Enzyme replacement therapy (ERT) slows down the progression of muscle symptoms. Reliable biomarkers are needed to follow up ERT-treated and asymptomatic LOPD patients in clinical practice. In this study, 32 LOPD patients (22 symptomatic and 10 asymptomatic) underwent muscle MRI using 3-point Dixon and were evaluated at the time of the MRI with several motor function tests and patient-reported outcome measures, and again after one year. Muscle MRI showed a significant increase of 1.7% in the fat content of the thigh muscles in symptomatic LOPD patients. In contrast, there were no noteworthy differences between muscle function tests in the same period of time. We did not observe any significant changes either in muscle MRI or in muscle function tests in asymptomatic patients over the year. We conclude that 3-point Dixon muscle MRI is a useful tool for detecting changes in muscle structure in symptomatic LOPD patients and could become part of the current follow-up protocol in daily clinics

Follow-up of late-onset Pompe disease patients with muscle magnetic resonance imaging reveals increase in fat replacement in skeletal muscles

Altres ajuts: This investigation was sponsored by the following grants, one from Sanofi Genzyme and another from the Spanish Ministry of Health, Fondos FEDER-ISCIII. Isabel Illa has received speaker honorarium from Grifols and Sanofi-Genzyme. Jordi Díaz-Manera has received speaker honorarium from PTC Therapeutics and Sanofi-Genzyme. The authors of this manuscript certify that they comply with the ethical guidelines for authorship and publishing in the Journal of Cachexia, Sarcopenia, and Muscle.42Late-onset Pompe disease (LOPD) is a genetic disorder characterized by progressive degeneration of the skeletal muscles produced by a deficiency of the enzyme acid alpha-glucosidase. Enzymatic replacement therapy with recombinant human alpha-glucosidase seems to reduce the progression of the disease; although at the moment, it is not completely clear to what extent. Quantitative muscle magnetic resonance imaging (qMRI) is a good biomarker for the follow-up of fat replacement in neuromuscular disorders. The aim of this study was to describe the changes observed in fat replacement in skeletal muscles using qMRI in a cohort of LOPD patients followed prospectively. A total of 36 LOPD patients were seen once every year for 4 years. qMRI, several muscle function tests, spirometry, activities of daily living scales, and quality-of-life scales were performed on each visit. Muscle MRI consisted of two-point Dixon studies of the trunk and thigh muscles. Computer analysis of the images provided the percentage of muscle degenerated and replaced by fat in every muscle (known as fat fraction). Longitudinal analysis of the measures was performed using linear mixed models applying the Greenhouse-Geisser test. We detected a statistically significant and continuous increase in mean thigh fat fraction both in treated (+5.8% in 3 years) and in pre-symptomatic patients (+2.6% in 3years) (Greenhouse-Geisser p < 0.05). As an average, fat fraction increased by 1.9% per year in treated patients, compared with 0.8% in pre-symptomatic patients. Fat fraction significantly increased in every muscle of the thighs. We observed a significant correlation between changes observed in fat fraction in qMRI and changes observed in the results of the muscle function tests performed. Moreover, we identified that muscle performance and mean thigh fat fraction at baseline visit were independent parameters influencing fat fraction progression over 4 years (analysis of covariance, p < 0.05). Our study identifies that skeletal muscle fat fraction continues to increase in patients with LOPD despite the treatment with enzymatic replacement therapy. These results suggest that the process of muscle degeneration is not stopped by the treatment and could impact muscle function over the years. Hereby, we show that fat fraction along with muscle function tests can be considered a good outcome measures for clinical trials in LOPD patients

Identification of serum microRNAs as potential biomarkers in Pompe disease

Altres ajuts: This study was supported by a grant from Sanofi-Genzyme (GZ-2015-11342) to Dr. Gallardo and has been registered in Clinicaltrials.gov (identifier NCT03045042).This study was supported by a grant from Sanofi-Genzyme (GZ-2015-11342) to Dr. Gallardo and has been registered in Clinicaltrials.gov (identifier NCT03045042).To analyze the microRNA profile in serum of patients with Adult Onset Pompe disease (AOPD). We analyzed the expression of 185 microRNAs in serum of 15 AOPD patients and five controls using microRNA PCR Panels. The expression levels of microRNAs that were deregulated were further studied in 35 AOPD patients and 10 controls using Real-Time PCR. Additionally, the skeletal muscle expression of microRNAs which showed significant increase levels in serum samples was also studied. Correlations between microRNA serum levels and muscle function test, spirometry, and quantitative muscle MRI were performed (these data correspond to the study NCT01914536 at ClinicalTrials.gov). We identified 14 microRNAs that showed different expression levels in serum samples of AOPD patients compared to controls. We validated these results in a larger cohort of patients and we found increased levels of three microRNAs, the so called dystromirs: miR-1-3p, miR-133a-3p, and miR-206. These microRNAs are involved in muscle regeneration and the expression of these was increased in patients' muscle biopsies. Significant correlations between microRNA levels and muscle function test were found. Serum expression levels of dystromirs may represent additional biomarkers for the follow-up of AOPD patients

Platelet Derived Growth Factor-AA Correlates With Muscle Function Tests and Quantitative Muscle Magnetic Resonance in Dystrophinopathies

Introduction: Duchenne (DMD) and Becker (BMD) muscular dystrophy are X-linked muscular disorders produced by mutations in the DMD gene which encodes the protein dystrophin. Both diseases are characterized by progressive involvement of skeletal, cardiac, and respiratory muscles. As new treatment strategies become available, reliable biomarkers and outcome measures that can monitor disease progression are needed for clinical trials. Methods: We collected clinical and functional data and blood samples from 19 DMD patients, 13 BMD patients, and 66 healthy controls (8 pediatric and 58 adult controls), and blood samples from 15 patients with dysferlinopathy (DYSF) and studied the serum concentration of 4 growth factors involved in the process of muscle fibrosis. We correlated the serum concentration of these growth factors with several muscle function tests, spirometry results and fat fraction identified by quantitative Dixon muscle MRI. Results: We found significant differences in the serum concentration of Platelet Derived Growth Factor-AA (PDGF-AA) between DMD patients and pediatric controls, in Connective Tissue Growth Factor (CTGF) between BMD patients and adult controls, and in and Transforming Growth Factor- β1 (TGF-β1) between BMD and DYSF patients. PDGF-AA showed a good correlation with several muscle function tests for both DMD and BMD patients and with thigh fat fraction in BMD patients. Moreover, PDGF-AA levels were increased in muscle biopsies of patients with DMD and BMD as was demonstrated by immunohistochemistry and Real-Time PCR studies. Conclusion: Our study suggests that PDGF-AA should be further investigated in a larger cohort of DMD and BMD patients because it might be a good biomarker candidate to monitor the progression of these diseases