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[ES] El objetivo de este trabajo se centra en desarrollar una identidad de marca y una estrategia de comunicación para un negocio familiar ya existente en el sector agrícola. El proyecto parte del análisis de la utilización de la marca y la comunicación en el sector agrícola, tanto por parte de grandes empresas como PYMES o negocios familiares. A continuación, se procede a aplicar las conclusiones de este análisis al desarrollo de una marca gráfica y un conjunto de acciones de comunicación que permitan a este negocio familiar transformarse en un negocio más elaborado y rentable., [EN] The goal of this project is focused on developing a brand identity and a communication strategy for an existing family business in the agricultural sector. The project starts analysing the use of the brand and communication in the agricultural sector, both by large companies such as SMEs, as well as by other family businesses too. Next, the conclusions of the analysis are applied to the development of a graphic brand and a set of communication actions that allow this family business to transform itself into a more elaborate and profitable business.

Background This study examines how dogs observe images of natural scenes containing living creatures (wild animals, dogs and humans) recorded with eye gaze tracking. Because dogs have had limited exposure to wild animals in their lives, we also consider the natural novelty of the wild animal images for the dogs. Methods The eye gaze of dogs was recorded while they viewed natural images containing dogs, humans, and wild animals. Three categories of images were used: naturalistic landscape images containing single humans or animals, full body images containing a single human or an animal, and full body images containing a pair of humans or animals. The gazing behavior of two dog populations, family and kennel dogs, were compared. Results As a main effect, dogs gazed at living creatures (object areas) longer than the background areas of the images; heads longer than bodies; heads longer than background areas; and bodies longer than background areas. Dogs gazed less at the object areas vs. the background in landscape images than in the other image categories. Both dog groups also gazed wild animal heads longer than human or dog heads in the images. When viewing single animal and human images, family dogs focused their gaze very prominently on the head areas, but in images containing a pair of animals or humans, they gazed more at the body than the head areas. In kennel dogs, the difference in gazing times of the head and body areas within single or paired images failed to reach significance. Discussion Dogs focused their gaze on living creatures in all image categories, also detecting them in the natural landscape images. Generally, they also gazed at the biologically informative areas of the images, such as the head, which supports the importance of the head/face area for dogs in obtaining social information. The natural novelty of the species represented in the images as well as the image category affected the gazing behavior of dogs. Furthermore, differences in the gazing strategy between family and kennel dogs was obtained, suggesting an influence of different social living environments and life experiences.

Protocolos de práctica asistencial [Resumen] Características generales. Las miocardiopatías genéticas engloban un grupo heterogéneo de enfermedades. Algunas afectan de forma aislada al músculo cardíaco, en otros casos forman parte del espectro de una enfermedad sistémica. Con frecuencia comienzan en la juventud, constituyendo una causa importante de morbimortalidad en los pacientes afectados. Diagnóstico. Existen manifestaciones clínicas, alteraciones electrocardiográficas y pruebas de imagen que permiten el diagnóstico de la afectación estructural cardíaca. Los avances en el campo de la genética han permitido el conocimiento de un amplio porcentaje de mutaciones causales de estas enfermedades. Una vez realizado el diagnóstico del paciente afectado, es necesario ampliar el estudio a la familia, para así identificar a individuos asintomáticos y realizar una estratificación pronóstica [Abstract] General characteristics. Genetic cardiomyopathies encompass a heterogeneous group of diseases. Some affect the heart muscle in isolation, while in other cases they form part of the spectrum of a systemic disease. These diseases frequently start in youth and represent a significant cause of morbidity and mortality in those affected by the disease. Diagnosis. There are clinical manifestations, electrocardiographic abnormalities and imaging tests that enable the diagnosis of cardiac structural involvement. Advances in the field of genetics have revealed a high rate of causal mutations of these diseases. Once the affected patient has been diagnosed, the study needs to be extended to the family to thereby identify asymptomatic individuals and to perform a prognostic stratification.

The objective of the study is to classify electroencephalogram signals recorded in a familiar and unfamiliar face recognition experiment. Frontal views of familiar and unfamiliar face images were shown to 10 volunteers in different sessions. In contrast to previous studies, no marker button was used during the experiment. Participants had to decide whether the displayed face was familiar or unfamiliar at the instant of stimulus presentation. The signals were analyzed in the preprocessing, channel selection, feature extraction, and classification stages. The novel two-feature extraction and eight-channel selection methods were applied to the analyses. Sixteen classification results were compared and the best performance was investigated. Consequently, the highest average classification accuracy was obtained at 72.67% when piecewise constant modeling feature extraction and relative entropy channel selection methods were used. The objective of the study is to classify electroencephalogram signals recorded in a familiar and unfamiliar face recognition experiment. Frontal views of familiar and unfamiliar face images were shown to 10 volunteers in different sessions. In contrast to previous studies, no marker button was used during the experiment. Participants had to decide whether the displayed face was familiar or unfamiliar at the instant of stimulus presentation. The signals were analyzed in the preprocessing, channel selection, feature extraction, and classification stages. The novel two-feature extraction and eight-channel selection methods were applied to the analyses. Sixteen classification results were compared and the best performance was investigated. Consequently, the highest average classification accuracy was obtained at 72.67% when piecewise constant modeling feature extraction and relative entropy channel selection methods were used.

The aim of this study was to investigate the appropriateness, attractiveness, use-intention and (un)desirable sensory properties of meat substitutes in different dishes based only on visual information. A web-based survey was developed to let consumers assess the use of meat substitutes in different dishes. The survey consisted of 38 key questions with subdivisions and was completed by 251 respondents. Six different dishes (spaghetti, rice, wrap, pizza, pasta salad, and soup) were rated for their appropriateness for the use of meat substitutes. Subsequently, appropriateness, attractiveness, and use-intention were rated based on photographs of the six dishes prepared with meat substitutes that differed in shape and appearance. Respondents also had to indicate (un)desirable sensory properties of meat substitutes for every dish. Spaghetti, rice and wrap were more appropriate for the use of meat substitutes than the other dishes. The most appropriate meat substitute–meal combinations were those that are similar to common Dutch meal combinations (e.g. spaghetti with mince and rice with pieces). Attractiveness and intention scores were in line with the appropriateness scores. Furthermore, we found that current users of meat substitutes and younger respondents gave higher appropriateness ratings. This study demonstrates that appropriateness of meat substitutes in a dish is related to attractiveness and use-intention and that meal context should be taken into account in the development of new meat substitutes.

Background Alexander disease (AxD) is a rare neurological disease, especially in adults. It shows variable clinical and radiological features. Case presentation We diagnosed a female with AxD presenting with paroxysmal numbness of the limbs at the onset age of 28-year-old, progressing gradually to spastic paraparesis at age 30. One year later, she had ataxia, bulbar paralysis, bowel and bladder urgency. Her mother had a similar neurological symptoms and died within 2 years after onset (at the age of 47), and her maternal aunt also had similar but mild symptoms at the onset age of 54-year-old. Her brain magnetic resonance imaging (MRI) showed abnormal signals in periventricular white matter with severe atrophy in the medulla oblongata and thoracic spinal cord, and mild atrophy in cervical spinal cord, which is unusual in the adult form of AxD. She and her daughter’s glial fibrillary acidic protein (GFAP) gene analysis revealed the same heterozygous missense mutation, c.1246C > T, p.R416W, despite of no neurological symptoms in her daughter. Conclusions Our case report enriches the understanding of the familial adult AxD. Genetic analysis is necessary when patients have the above mentioned symptoms and signs, MRI findings, especially with family history.

International audience; Feature modeling is a popular Software Product Line Engineering (SPLE) technique used to describe variability in a product family. A usable feature modeling tool environment should enable SPLE practitioners to produce good quality models, in particular, models that effectively communicate modeled information. FAMILIAR is a text-based environment for manipulating and composing Feature Models (FMs). In this paper we present extensions we made to FAMILIAR to enhance its usability. The extensions include a visualization of FMs, or more precisely , a feature diagram rendering mechanism that supports the use of a combination of text and graphics to describe FMs, their configurations, and the results of FM analyses. We also present the results of a preliminary evaluation of the environment's usability. The evaluation involves comparing the use of the extended environment with the previous text-based console-driven version. The preliminary experiment provides some evidence that use of the new environment results in increased cognitive effectiveness of novice users and improved quality of new FMs.

La presente recopilación, muestra la importancia de la empresa familiar, un tipo de empresa que genera empleo y que es uno de los sectores más productivos del país, el manejo administrativo y financiero no es el más adecuado, por lo tanto son empresas que deben entrar en un proceso de modernización o actualización, porque de lo contrario tienden a desaparecer. La guía de manejo para hacer que una empresa familiar sea exitosa brinda las herramientas necesarias, que de ser aplicadas en debida forma garantizan empresas familiares duraderas y de proyección de generación en generación This collection, shows the importance of the family business, a type of company which creates jobs and which is one of the most productive sectors of the country, the administrative and financial management is not the most appropriate, therefore they are companies that should enter into a process of modernization or update, because otherwise they tend to disappear. Management Guide to make a family business to be successful provides the necessary tools to be applied properly ensure family business and long-term projection from generation to generation

A key question in cognitive neuroscience is how the brain combines low-level features processed in remote sensory cortices to represent meaningful multisensory objects in our everyday environment. Models of visual object processing typically assume a feedforward cascade through the hierarchically organized ventral stream. We contrasted this feedforward view with an alternate hypothesis in which object processing is viewed as an interactive, feedforward and feedback process. We found that higher-order regions in anterior temporal (AT) and inferior prefrontal cortex (IPC) performed audio-visual (AV) integration 100 ms earlier than a sensory-driven region in the posterior occipital (pO) cortex, and were modulated by semantic variables (congruency), from as early as 50-100 ms. We propose that the brain represents familiar and complex multisensory objects through early interactivity between higher-order and sensory-driven regions. This interactivity may underpin the enhanced behavioral performance reported for semantically congruent AV objects.

La Colestasia Intrahepática Familiar Progresiva (CIFP) comprende un grupo heterogéneo de alteraciones autosómicas recesiva caracterizadas por una colestasia hepatocelular secundaria a una interrupción del proceso normal de síntesis de la bilis. Objetivo: Describir la presentación de CIFP en 3 de 5 hijos de una familia estudiada. Caso clínico: Paciente de 5 años de edad (caso 1), que presenta una hepatoesplenomegalia, aumento de enzimas hepáticas y de ácidos biliares en suero. La ecotomografía abdominal describe alteraciones compatibles con fibrosis hepática. La biopsia reveló fibrosis en puente, proliferación ductular y colestasia crónica mínima. Estos hallazgos fueron compatibles con el fenotipo de una CIFP-3 con niveles elevados de Gamaglutamiltransferasa (GGT). Una mutación del gen MDR3 es responsable de la ausencia de fosfolípidos en la bilis, lo que permite la acción detergente de los ácidos biliares sobre el epitelio de los conductos desencadenando una colangitis, fibrosis y luego cirrosis. De los cuatro hermanos del caso 1 se detectó la enfermedad en 2 hermanas gemelas (casos 2 y 3). Estos tres niños afectados fueron tratados con ácido ursodeoxicólico 30 mg/kilo/peso, logrando excelentes resultados en las gemelas pero no en el caso 1. Conclusión: Se presenta a 3 hermanos con el fenotipo de CIFP. La expresión clínica de esta enfermedad puede ser variable y de manifestación tardía, la elevación de las aminotransferasas debe considerar esta patología en el diagnóstico diferencial de las numerosas causas que dan origen a un aumento de estas enzimas. Sólo el diagnóstico y tratamiento precoz puede evitar la evolución a un daño hepático irreversible como es la cirrosis.

OBJECTIVE: To analyze seizure outcome in individuals with familial mesial temporal lobe epilepsy (FMTLE). METHOD: We followed prospectively 64 individuals with FMTLE and 37 asymptomatic individuals belonging to 28 families. RESULTS: Patients with FMTLE had a mean follow up was 93.4 ± 15.8 months. At baseline they were divided in benign (n = 29), remission (n = 28) and refractory (n = 7). At last follow up visit 41.4% patients with benign FMTLE remained classified as benign, 20.7% became refractory and 37.9% were in remission. In the subgroup of FMTLE in remission 21 75% remained without seizures; 21.4% were classified as benign FMTLE, and one died (3.6%) from cause unrelated to epilepsy. All refractory patients remained refractory. From the asymptomatic group, 10.8% became symptomatic (FMTLE). The mean follow up was 76.0 ± 21.2 months. CONCLUSION: Prospective follow up of more than 7 years in patients with FMTLE revealed that it is unlikely to achieve seizure control in those with refractory seizures. Patients with diagnose of more benign forms of FMTLE for more than one year are likely to either remit or remain under well controlled seizures. The majority of patients who had achieved seizure remission remained seizure-free and none became refractory. Asymptomatic individuals had a greater probability to have seizures compared to the general population in a 6 year period of follow up. OBJETIVOS: Analisar a evolução de famílias com epilepsia de lobo temporal mesial familiar (ELTMF). METODOLOGIA: Seguimento prospectivo de 64 pacientes com ELTMF e 37 membros assintomáticos pertencente a 28 famílias. RESULTADOS: A média de seguimento dos pacientes com ELTMF foi de 93,4 ± 15,8 meses. Na avaliação inicial os pacientes foram divididos em benignos (n = 29), remissão (n = 28) e refratários (n = 7). Na última visita disponível, 41,4% dos pacientes com ELTMF benigna permaneceram classificados como benignos, 20,7% tornaram-se refratários e 37,9% entraram em remissão. No grupo em remissão, 75% permaneceram livres de crise, 21,4% foram classificados como benignos e um faleceu (3,6%) de causa não relacionada à epilepsia. Todos pacientes refratários permaneceram refratários. Em relação aos assintomáticos 10,8% evoluíram com crises. A média de seguimento dos assintomáticos foi de 76,0 ± 21,2 meses. CONCLUSÃO: O seguimento prospectivo de mais de 7 anos de pacientes com ELTMF revelou que é improvável ocorrer controle de crises no grupo refratário. No grupo benigno é muito provável que estes indivíduos entrem em remissão ou permaneçam com evolução benigna. A maioria dos pacientes do grupo em remissão permaneceu em remissão e nenhum se tornou refratário. Em relação aos assintomáticos a probabilidade de apresentar uma crise no decorrer de aproximadamente 6 anos foi maior que o observado na população geral.

La distrofia simpático refleja (DSR) puede estar relacionada con diferentes factores desencadenantes o predisponentes. No obstante, en la literatura médica no hay muchas publicaciones referentes a un terreno genético o familiar en la DSR. Por ello, presentamos el caso de 2 hermanos con similar HLA y con DSR de extremidades inferiores, politópica y recurrente.