Fissured tongue: Debut of Melkersson-Rosenthal syndrome.

[ES] Introducción: De las múltiples causas de parálisis faciales, uni o bilaterales, la gran dominadora es la parálisis de Bell o idiopática (40-75%). Melkersson (1928) y Rosenthal (1930) describieron este síndrome, todavía de etiopatogenia desconocida; algunos autores defienden la hipótesis autoinmune (mejora con corticoides), la infecciosa, o la genética (alteraciones en el brazo corto del cromosoma 9). Se estima una prevalencia entre la población general del 0,08%, sin objetivarse diferencias significativas entre sexo o etnia. Este síndrome puede llevar consigo en el 25-40% de las veces, el edema orofacial, la lengua plicata y la parálisis facial uni o bilateral, siendo la forma oligosintomática la más frecuente. Material y métodos: Paciente de 68 años con, visto en varias ocasiones en consultas de urgencias por presentar edema lingual, hiposmia y episodios de vómitos en los últimos meses. El paciente finalmente ingresa en planta de ORL por presentar parálisis facial unilateral y edema facial de 36 horas de evolución. A la exploración se objetiva la presencia de surcos fibrosos linguales, edema labial superior e inferior, así como una parálisis facial periférica izquierda (en la clasificación de House-Brackmann (5/6). Resultados: Se le solicitó una analítica y radiografía de tórax como primeras pruebas de orientación diagnóstica. De los datos analíticos cabe destacar una ligera eosinofilia con niveles de complemento y radiografía de tórax normales (sin adenopatías hiliares). Se tomaron biopsias linguales para confirmar el diagnóstico mediante anatomía patológica (granulomas no caseificantes). Se le administraron corticoides endovenosos y medidas de oclusión ocular (pomada epitelizante y taponamiento ocular). Se realizaron estudios posteriores, como la ENG y la EMG para comprobar el estado del Nervio facial. Discusión: Esta clase de cuadro puede ir asociado a síntomas más generales que nos pueden despistar y dar una mala orientación diagnóstica. El edema facial es el signo de la triada más frecuente (65%) y con el que suele debutar, de ahí lo inusual de este caso. Pueden presentar recidivas de su sintomatología en múltiples ocasiones. El diagnóstico es clínico y la confirmación se obtiene a través de la anatomía patológica (granulomas no caseificantes).Destacan estudios con talidomida, con buenos resultados pero en fase de aceptación. [EN] Introduction: the many causes of facial paralysis, unilateral or bilateral, the great master is Bell's palsy or idiopathic (40-75%). Melkersson (1928) and Rosenthal (1930) described this syndrome, still of unknown etiology, some authors support the autoimmune hypothesis (improvement with corticosteroids), the infectious, or genetic (changes in the short arm of chromosome 9). The estimated general population prevalence of 0.08%, no differences were significant between sex or ethnicity. This syndrome can bring in 25-40% of the time, orofacial edema, tongue and facial paralysis plicata unilateral or bilateral, being the most frequent oligosymptomatic. Methods: Patient 68 years, seen repeatedly in emergency visits for lingual edema, hyposmia and vomiting episodes in recent months. The patient eventually admitted to ENT for filing plant facial palsy and facial edema 36 hours of evolution. Upon examination revealed the presence of fibrous lingual grooves, upper and lower lip edema and left peripheral facial paralysis (in the House-Brackmann classification (5 / 6). Results: Analytical and requested a chest radiograph as first tests of diagnostic orientation. Analytical data include a slight eosinophilia in complement levels and normal chest radiograph (without hilar lymphadenopathy). Tongue biopsies were taken to confirm the diagnosis by pathology (noncaseating granulomas). Intravenous corticosteroids were administered and measures of ocular occlusion (blockage epitelizant cream and eye). Subsequent studies were performed, such as ENG and EMG to check the status of the facial nerve. Discussion: This kind of picture may be associated with more general symptoms that may mislead and give us a bad diagnostic orientation. Facial edema is a sign of the triad most frequent (65%) and that tends to appear, hence the unusual case. Can relapses of their symptoms on multiple occasions. The diagnosis is clinical and confirmation is obtained through the pathology (noncaseating granulomas). Outstanding scholar with thalidomide, with good results but in the process of acceptance

Conflict transformation in indigenous' peoples territories: doing environmental justice with a 'decolonial turn'

One of the distinctive features of environmental justice theory in Latin America is its influence by decolonial thought, which explains social and environmental injustices as arising from the project of modernity and the ongoing expansion of a European cultural imaginary. The decolonization of knowledge and social relations is highlighted as one of the key challenges for overcoming the history of violent oppression and marginalization in development and conservation practice in the region. In this paper we discuss how conflict transformation theory and practice has a role to play in this process. In doing so, we draw on the Socio-environmental Conflict Transformation (SCT) framework elaborated by Grupo Confluencias, which puts a focus on building community capacity to impact different spheres of power: people and networks, structures and cultural power. We discuss this framework and its practical use in the light of ongoing experiences with indigenous peoples in Latin America. We propose that by strengthening the power of agency of indigenous peoples to impact each of these spheres it is possible to build constructive intra and intercultural relations that can help increase social and environmental justice in their territories and thus contribute to decolonizing structures, relations and ways of being

Innovative approach for self management and social welfare of children with cystic fibrosis in Europe : development validation and implementation of an mHealth tool (MyCyFAPP)

Introduction For the optimal management of children with cystic fibrosis, there are currently no efficient tools for the precise adjustment of pancreatic enzyme replacement therapy, either for advice on appropriate dietary intake or for achieving an optimal nutrition status. Therefore, we aim to develop a mobile application that ensures a successful nutritional therapy in children with cystic fibrosis. Methods and analysis A multidisciplinary team of 12 partners coordinate their efforts in 9 work packages that cover the entire so-called \u2018from laboratory to market\u2019 approach by means of an original and innovative co-design process. A cohort of 200 patients with cystic fibrosis aged 1\u201317\u2005years are enrolled. We will develop an innovative, clinically tested mobile health application for patients and health professionals involved in cystic fibrosis management. The mobile application integrates the research knowledge and innovative tools for maximising self-management with the aim of leading to a better nutritional status, quality of life and disease prognosis. Bringing together different and complementary areas of knowledge is fundamental for tackling complex challenges in disease treatment, such as optimal nutrition and pancreatic enzyme replacement therapy in cystic fibrosis. Patients are expected to benefit the most from the outcomes of this innovative project. Ethics and dissemination The project is approved by the Ethics Committee of the coordinating organisation, Hospital Universitari La Fe (Ref: 2014/0484). Scientific findings will be disseminated via journals and conferences addressed to clinicians, food scientists, information and communications technology experts and patients. The specific dissemination working group within the project will address the wide audience communication through the website (http://www.mycyfapp.eu), the social networks and the newsletter

A perspective on radical transformations to sustainability: resistances, movements and alternatives

A transformation to sustainability calls for radical and systemic societal shifts. Yet what this entails in practice and who the agents of this radical transformation are require further elaboration. This article recenters the role of environmental justice movements in transformations, arguing that the systemic, multi-dimensional and intersectional approach inherent in EJ activism is uniquely placed to contribute to the realization of equitable sustainable futures. Based on a perspective of conflict as productive, and a “conflict transformation” approach that can address the root issues of ecological conflicts and promote the emergence of alternatives, we lay out a conceptual framework for understanding transformations through a power analysis that aims to confront and subvert hegemonic power relations; that is, multi-dimensional and intersectional; balancing ecological concerns with social, economic, cultural and democratic spheres; and is multi-scalar, and mindful of impacts across place and space. Such a framework can help analyze and recognize the contribution of grassroots EJ movements to societal transformations to sustainability and support and aid radical transformation processes. While transitions literature tends to focus on artifacts and technologies, we suggest that a resistance-centred perspective focuses on the creation of new subjectivities, power relations, values and institutions. This recenters the agency of those who are engaged in the creation and recuperation of ecological and new ways of being in the world in the needed transformation

Clinical validation of an evidence-based method to adjust Pancreatic Enzyme Replacement Therapy through a prospective interventional study in paediatric patients with Cystic Fibrosis

Background A method to adjust Pancreatic Enzyme Replacement Therapy in Cystic Fibrosis is not currently available. Objectives To assess the in vivo efficacy of a method to adjust the dose of enzymatic supplement in CF extrapolated from previous in vitro digestion studies (theoretical optimal dose, TOD). Secondly, to assess how individual patient characteristics influence the expected coefficient of fat absorption (CFA) and thus to identify an individual correction factor to improve TOD. Methods A prospective interventional study in 43 paediatric patients with CF from 5 European centres. They followed a 24h fixed diet with the theoretical optimal dose for each meal. Faecal collection was carried out between colorimetric markers in order to include all the faeces corresponding to the fixed diet. Beta regression models were applied to assess the associations of individual patient characteristics with the CFA. Results Median CFA was 90% (84, 94% 1st, 3rd Q.) with no significant differences among centres. Intestinal transit time was positively associated with CFA (p = 0.007), but no statistical associations were found with and age, gender, phenotype or BMI. Regression model showed no improvement of the in vitro predicted theoretical optimal dose when taking individual patient characteristics into account. Conclusion Strict adherence to the theoretical optimal dose of enzymatic supplement for a prescribed meal, led to median CFA levels at the clinical target of 90% with a low variability between patients. The proposed method can be considered as a first approach for an evidencebased method in PERT dosing based on food characteristics. Results have to be confirmed in free dietary settings

Low exposure long-baseline neutrino oscillation sensitivity of the DUNE experiment

The Deep Underground Neutrino Experiment (DUNE) will produce world-leading neutrino oscillation measurements over the lifetime of the experiment. In this work, we explore DUNE's sensitivity to observe charge-parity violation (CPV) in the neutrino sector, and to resolve the mass ordering, for exposures of up to 100 kiloton-megawatt-years (kt-MW-yr). The analysis includes detailed uncertainties on the flux prediction, the neutrino interaction model, and detector effects. We demonstrate that DUNE will be able to unambiguously resolve the neutrino mass ordering at a 3$\sigma$ (5$\sigma$) level, with a 66 (100) kt-MW-yr far detector exposure, and has the ability to make strong statements at significantly shorter exposures depending on the true value of other oscillation parameters. We also show that DUNE has the potential to make a robust measurement of CPV at a 3$\sigma$ level with a 100 kt-MW-yr exposure for the maximally CP-violating values \delta_{\rm CP}} = \pm\pi/2. Additionally, the dependence of DUNE's sensitivity on the exposure taken in neutrino-enhanced and antineutrino-enhanced running is discussed. An equal fraction of exposure taken in each beam mode is found to be close to optimal when considered over the entire space of interest

Identification and reconstruction of low-energy electrons in the ProtoDUNE-SP detector

Measurements of electrons from $\nu_e$ interactions are crucial for the Deep Underground Neutrino Experiment (DUNE) neutrino oscillation program, as well as searches for physics beyond the standard model, supernova neutrino detection, and solar neutrino measurements. This article describes the selection and reconstruction of low-energy (Michel) electrons in the ProtoDUNE-SP detector. ProtoDUNE-SP is one of the prototypes for the DUNE far detector, built and operated at CERN as a charged particle test beam experiment. A sample of low-energy electrons produced by the decay of cosmic muons is selected with a purity of 95%. This sample is used to calibrate the low-energy electron energy scale with two techniques. An electron energy calibration based on a cosmic ray muon sample uses calibration constants derived from measured and simulated cosmic ray muon events. Another calibration technique makes use of the theoretically well-understood Michel electron energy spectrum to convert reconstructed charge to electron energy. In addition, the effects of detector response to low-energy electron energy scale and its resolution including readout electronics threshold effects are quantified. Finally, the relation between the theoretical and reconstructed low-energy electron energy spectrum is derived and the energy resolution is characterized. The low-energy electron selection presented here accounts for about 75% of the total electron deposited energy. After the addition of lost energy using a Monte Carlo simulation, the energy resolution improves from about 40% to 25% at 50~MeV. These results are used to validate the expected capabilities of the DUNE far detector to reconstruct low-energy electrons.Comment: 19 pages, 10 figure

Snowmass Neutrino Frontier: DUNE Physics Summary

The Deep Underground Neutrino Experiment (DUNE) is a next-generation long-baseline neutrino oscillation experiment with a primary physics goal of observing neutrino and antineutrino oscillation patterns to precisely measure the parameters governing long-baseline neutrino oscillation in a single experiment, and to test the three-flavor paradigm. DUNE's design has been developed by a large, international collaboration of scientists and engineers to have unique capability to measure neutrino oscillation as a function of energy in a broadband beam, to resolve degeneracy among oscillation parameters, and to control systematic uncertainty using the exquisite imaging capability of massive LArTPC far detector modules and an argon-based near detector. DUNE's neutrino oscillation measurements will unambiguously resolve the neutrino mass ordering and provide the sensitivity to discover CP violation in neutrinos for a wide range of possible values of δCP. DUNE is also uniquely sensitive to electron neutrinos from a galactic supernova burst, and to a broad range of physics beyond the Standard Model (BSM), including nucleon decays. DUNE is anticipated to begin collecting physics data with Phase I, an initial experiment configuration consisting of two far detector modules and a minimal suite of near detector components, with a 1.2 MW proton beam. To realize its extensive, world-leading physics potential requires the full scope of DUNE be completed in Phase II. The three Phase II upgrades are all necessary to achieve DUNE's physics goals: (1) addition of far detector modules three and four for a total FD fiducial mass of at least 40 kt, (2) upgrade of the proton beam power from 1.2 MW to 2.4 MW, and (3) replacement of the near detector's temporary muon spectrometer with a magnetized, high-pressure gaseous argon TPC and calorimeter

A Gaseous Argon-Based Near Detector to Enhance the Physics Capabilities of DUNE

This document presents the concept and physics case for a magnetized gaseous argon-based detector system (ND-GAr) for the Deep Underground Neutrino Experiment (DUNE) Near Detector. This detector system is required in order for DUNE to reach its full physics potential in the measurement of CP violation and in delivering precision measurements of oscillation parameters. In addition to its critical role in the long-baseline oscillation program, ND-GAr will extend the overall physics program of DUNE. The LBNF high-intensity proton beam will provide a large flux of neutrinos that is sampled by ND-GAr, enabling DUNE to discover new particles and search for new interactions and symmetries beyond those predicted in the Standard Model