Una herramienta de soporte al proceso de desarrollo dirigido por modelos (MDD)

The use of models for building different types of software systems is at the moment one of the keys for developing new technologies. The fundamental idea of this “Model Driven Development” (MDD) it is to substitute, as main artifact in the process of software development, the code in a programming language by models. Such models are then considered as first class entities, allowing new ways of creating, analyzing and manipulating systems through different languages and tools. In this context that involves graphic modeling languages, precise specification of models, model transformation tools and formal models specification languages, among other, arises the need of developing PAMPA: a visual environment tool developed on .Net platform that allows building models starting from UML diagrams, providing also formal support with OCL expressions, based on a metamodel specification. We’ll show step by step the concerns, functionalities and application environment of the tool, which is essentially educational, in order to promote the use of the new paradigm MDD.El uso de modelos para construir distintos tipos de sistemas de software es actualmente una de las claves para desarrollar nuevas tecnologías. La idea fundamental de este “desarrollo dirigido por modelos” (MDD) es sustituir, como artefacto principal en el proceso de desarrollo de software, al código de lenguajes de programación por modelos. De este modo, tales modelos son considerados como entidades de primera clase, permitiendo nuevas posibilidades de crear, analizar y manipular sistemas a través de diversos lenguajes y herramientas. Dentro de este contexto que involucra lenguajes gráficos de modelado, la especificación de modelos precisos, herramientas de transformación de modelos y lenguajes para especificar modelos formales, entre otros, yace la necesidad de crear PAMPA: una herramienta de entorno visual desarrollada sobre plataforma .NET que permite construir modelos a partir de diagramas UML, proveyendo soporte formal con expresiones OCL, basados en un metamodelo de especificación. Mostraremos paso a paso las incumbencias, funcionalidades y ámbito de aplicación de la herramienta, la cual se orienta esencialmente, con fines educativos, a fomentar el uso del nuevo paradigma MDD.III Workshop de Ingeniería de Software y Bases de Datos (WISBD

Neurodegenerative disorder risk in idiopathic REM sleep behavior disorder: study in 174 Patients.

Objective To estimate the risk for developing a defined neurodegenerative syndrome in a large cohort of idiopathic REM sleep behavior disorder (IRBD) patients with long follow-up. Methods Using the Kaplan-Meier method, we estimated the disease-free survival rate from defined neurodegenerative syndromes in all the consecutive IRBD patients diagnosed and followed-up in our tertiary referal sleep center between November 1991 and July 2013. Results The cohort comprises 174 patients with a median age at diagnosis of IRBD of 69 years and a median follow-up of four years. The risk of a defined neurodegenerative syndrome from the time of IRBD diagnosis was 33.1% at five years, 75.7% at ten years, and 90.9% at 14 years. The median conversion time was 7.5 years. Emerging diagnoses (37.4%) were dementia with Lewy bodies (DLB) in 29 subjects, Parkinson disease (PD) in 22, multiple system atrophy (MSA) in two, and mild cognitive impairment (MCI) in 12. In six cases, in whom postmortem was performed, neuropathological examination disclosed neuronal loss and widespread Lewy-type pathology in the brain in each case. Conclusions In a large IRBD cohort diagnosed in a tertiary referal sleep center, prolonged follow-up indicated that the majority of patients are eventually diagnosed with the synucleinopathies PD, DLB and less frequently MSA. IRBD represented the prodromal period of these conditions. Our findings in IRBD have important implications in clinical practice, in the investigation of the early pathological events occurring in the synucleinopathies, and for the design of interventions with potential disease-modifying agents

Clinical presentations and antibody mechanisms in anti-IgLON5 disease

Anti-IgLON5 disease is a rare neurological disease, identified just ten years ago, where autoimmunity and neurodegeneration converge. The heterogeneity of symptoms, sometimes mimicking pure neurodegenerative diseases or motor neuron diseases, in addition to lack of awareness, represents a diagnostic challenge. Biomarkers of neuronal damage in combination with in vivo visualization of tau deposition using positron emission tomography (PET) scanning could represent a major advance in monitoring disease progression. Recent studies with more autopsies available have helped refine the knowledge of the pathological features of the disease and strengthen the autoimmune hypothesis of the disease. Although the pathogenesis of anti-IgLON5 disease remains unclear, the irreversible antibody-mediated decrease of IgLON5 clusters from the cell surface and alterations produced in the cytoskeleton, as well as the behavioural abnormalities and signs of neuroinflammation and neurodegeneration observed in the brains of animals infused with antibodies from patients by passive transfer, which have recently been published, support the autoimmune hypothesis of the disease. This review aims to summarize these important aspects and recent advances in the pathophysiology of anti-IgLON5 disease

Temporal distribution of sleep onset REM periods and N3 sleep in the MSLT and night polysomnogram of narcolepsy type 1 and other hypersomnias

The presence of $>\geq$2 sleep onset REM periods (SOREMP) in the Multiple Sleep Latency Test (MSLT) and the previous night polysomnogram (PSG) is part of the diagnostic criteria of narcolepsy, with every SOREMP having the same diagnostic value, despite evidence suggesting that time of SOREMP appearance and their preceding sleep stage might be relevant. We studied the temporal distribution of SOREMPs and associated sleep stages in the MSLT of patients with narcolepsy type 1 (NT1) and other hypersomnias (OH).We reviewed consecutive five-nap MSLTs and their preceding PSG from 83 untreated adult patients with hypersomnolence and ?1 SOREMPs. Wake/N1(W/N1)-SOREMPs, N2-SOREMPs, and N3 sleep presence and time of appearance were analyzed.Thirty-nine patients had NT1 and 44 OH. There were 183 (78%) SOREMPs in patients with NT1 and 83 (31%) in OH. Sixty-seven percent of SOREMPs in NT1 were from W/N1, and 20% -none from wake-in OH (p < 0.001). Most patients (94%) with ?2 W/N1-SOREMPs had NT1 (specificity 95%, sensitivity 82%). In patients with NT1 but not in OH, W/N1-SOREMPs decreased throughout the day (from 79% in the 1st nap to 33% in the preceding night, p < 0.001), whereas N2-SOREMPs did not change. N3 sleep frequency in the 5th nap was higher in NT1 than in OH (28% vs. 7%, p:0.009). Nocturnal-SOREMP plus ?4 daytime SOREMPs, Wake-REM transitions, and REM followed by N3 were only seen in NT1.Measuring the sleep stage sequence and temporal distribution of SOREMP helps to identify patients with narcolepsy in the MSLT.Copyright © 2022 Elsevier B.V. All rights reserved

Evolució del sistema d’emmagatzematge d’energia d’un vehicle de la Formula Student

En els darrers anys, la Formula Student ha rebut un gran suport per part de les empreses que ha permès fer un pas gegant en quant a nivell de tecnologia utilitzada en aquesta competició. Tot i tractar-se d’una competició a nivell universitari, gairebé tots els equips intenten diferenciar-se de la resta utilitzant les últimes evolucions tecnològiques. Sobretot queda palès amb la categoria de monoplaces elèctrics, que han passat de ser minoria a la competició a ser tendència. Cada any augmenta el nombre d’universitats que es decanten per aquesta categoria, i ara ja superen per prestacions als vehicles de combustió interna. Des de l’any 2012 a l’equip de Formula Student de l’Escola, ETSEIB Motorsport, s’ha optat per a dissenyar un monoplaça que competeixi a la categoria de propulsió elèctrica. Per tal de seguir evolucionant i millorar les prestacions globals del monoplaça, l’any 2014 es va decidir que després de dues edicions de la competició l’objectiu més important seria millorar les bateries. L’objectiu d’aquest projecte arrela amb la necessitat de fer aquest canvi, sobretot per reduir pes al monoplaça i alhora de disposar de més energia per finalitzar l’Endurance, la prova de resistència de la competició. Per això ha estat necessari un estudi de tecnologies i tipus de bateries disponibles al mercat, un dimensionament elèctric per saber les necessitats energètiques i prestacions que ha de poder subministrar la bateria. També s’ha elaborat una validació experimental del model de cel·la escollit. Aquesta tria de bateries es va realitzar per a dos anys vista, ja que seria una inversió econòmica important i de cara a 2015 la intenció era renovar la resta de la secció de tren de potència, motors i inversors. Per tant, un factor important a tenir en compte era que fossin molt versàtils, de manera que es poguessin adaptar a diferents sistemes de propulsió. A més, la bateria està monitoritzada per un Battery Management System, BMS, que s’encarrega de controlar la tensió de cada cel·la i la seva temperatura. Aquest projecte també engloba l’elecció d’aquest controlador de la bateria i la seva implementació durant dues edicions de la competició. Finalment, també ha estat necessària l’adaptació del carregador a la nova bateria, tant a nivell de seguretat com per millorar les seves prestacions

Doctrina Parot. Cobertura mediática en periódicos españoles

Treballs Finals de Grau de Criminologia, Facultat de Dret, Universitat de Barcelona, Curs: 2013-2014, Tutor: Dr. Santiago Redondo IllescasEsta investigación se centra en el tratamiento mediático que se ha producido como consecuencia de la derogación de la Doctrina Parot. Se han analizado veinte noticias de los dos periódicos más leídos en España: diez de El Mundo y diez de El País, ambos en su formato digital. El objetivo de este estudio es observar si asocian el fenómeno a valores positivos, negativos o si, en cambio, exponen la información de forma objetiva. También se ha analizado el posicionamiento ideológico acerca de la derogación. Los resultados indican un dominio de información objetiva por parte de ambos periódicos. No obstante, en los casos en que no es así, El País asocia el fenómeno a valores positivos mientras que El Mundo lo hace a valores negativos. Por último, El País se posiciona a favor de la derogación de la Doctrina Parot mientras que El Mundo lo hace en contra

Serum metabolic biomarkers for synucleinopathy conversion in isolated REM sleep behavior disorder

Neurodegeneració; Malaltia de Parkinson; Marcadors predictiusNeurodegeneración; Enfermedad de Parkinson; Marcadores predictivosNeurodegeneration; Parkinson's disease; Predictive markersIsolated rapid eye movement (REM) sleep behavior disorder (iRBD) is a prodromal stage of Lewy-type synucleinopathies (LTS), which can present either with an initial predominant parkinsonism (Parkinson’s disease (PD)) or dementia (dementia with Lewy bodies (DLB)). To provide insights into the underlying pathogenic mechanisms, the lipoprotein and protein glycosylation profile of 82 iRBD patients, collected before and/or after their conversion to an overt LTS, and 29 matched control serum samples were assessed by nuclear magnetic resonance (NMR) spectroscopy. Data were statistically analyzed to identify altered metabolites and construct predictive models. Univariant analysis detected no differences between iRBD patients with an LTS compared to controls. However, significant differences were found when the analysis distinguished between iRBD patients that manifested initially predominant parkinsonism (pre-PD) or dementia (pre-DLB). Significant differences were also found in the analysis of paired iRBD samples pre- and post-LTS diagnosis. Predictive models were built and distinguished between controls and pre-DLB patients, and between pre-DLB and pre-PD patients. This allowed a prediction of the possible future clinical outcome of iRBD patients. We provide evidence of altered lipoprotein and glycosylation profiles in subgroups of iRBD patients. Our results indicate that metabolic alterations and inflammation are involved in iRBD pathophysiology, and suggest biological differences underlying the progression of LTS in iRBD patients. Our data also indicate that profiling of serum samples by NMR may be a useful tool for identifying short-term high-risk iRBD patients for conversion to parkinsonism or dementia.The study was funded by the Fondo de Investigación Sanitaria-Instituto de Salud Carlos III (FIS-ISCIII, Spain)-European Regional Development Fund (FEDER, E.U.) (PI13/01897 to M.V.), Ministerio de Economía y Competitividad (MINECO, Spain) (SAF2015-73997-JIN to A.L. and SAF2016-77541-R to M.V.), Fundació Bancària La Caixa (Junior Leader Fellowship LCF/BQ/PR19/11700005 to A.L. and Health Research Project HR17-00513 to M.V.) and CIBERNED (to M.V. and E.T.). A.L. was the recipient of a postdoctoral contract SAF2015-73997-JIN from MINECO (Spain) with co-funding from FEDER (E.U.) and is currently funded by the Junior Leader Program from Fundació Bancària La Caixa (grant LCF/BQ/PR19/11700005). H.X. is the recipient of a Radboud University Personal Ph.D. Grant

Angioedema severity and impact on quality of life: Chronic histaminergic angioedema versus chronic spontaneous urticaria

Chronic histaminergic angioedema; Urticaria; Quality of lifeAngioedema histaminérgico crónico; Urticaria; Calidad de vidaAngioedema histaminèrgic crònic; Urticària; Qualitat de vidaThis work was supported by Grants PI16/01304 and #PI20/01536 from the Instituto de Salud Carlos III and the Thematic Networks for Co-operative Research Centers: Reacciones Adversas y Alérgicas network (RD16/0006/0031) from Instituto de Salud Carlos III, cofounded by Fondo Europeo de Desarrollo Regional. The manuscript was edited for English language by American Journal Experts

Cervical spinal cord injury by a low-impact trauma as an unnoticed cause of cardiorespiratory arrest

Background: Cardiorespiratory arrest (CA) secondary to traumatic cervical spinal cord injury can occur in minor accidents with low-impact trauma and may be overlooked as the cause of CA in patients admitted in the coronary care unit. Case summary: We present two patients admitted to the coronary care unit because of suspected CA of cardiac origin. Both patients were found in CA with asystole, one after collapsing in a shopping mall and falling down a few steps and the other in the street next to his bicycle. They underwent early pharmacologically induced coma and hypothermia precluding neurological examination. Both patients remained in coma after rewarming, with preserved brainstem reflexes but absent motor response to pain. One patient had post-anoxic myoclonus in the face without limb involvement. In both patients, median nerve somatosensory evoked potentials demonstrated bilateral absence of thalamocortical N19 responses and abnormal cervicomedullary junction potentials (N13 wave). Extensive diagnostic work-up did not find a cardiac cause of the CA, pulmonary thromboembolism, or intracranial haemorrhage. In both patients, cervical spinal cord injury was diagnosed incidentally 5 and 6 days after CA, when a brain magnetic resonance imaging performed to assess post-anoxic brain injuries detected spinal cord hyperintensities with fracture and luxation of the odontoid. Both patients died 11 and 8 days after CA. Discussion: Low-impact traumatic cervical spinal cord injury should be considered in the diagnostic work-up of patients with CA of unknown cause