[Book reviews] HONDA Hiroyuki: Bunkaku kara "Kaikakukaiho"ki ni okeru Chugokuchosenzoku no Nihongokyoiku no Kenkyu

En este artículo analizamos los datos de visibilidad y uso de las dos revistas de temática americanista editadas por el CSIC, Revista de Indias y Anuario de Estudios Americanos. La edición electrónica de ambas data de 2007, y su contenido en artículos accesibles es comparable; ambas se publican en Acceso Abierto (OA) con un embargo de acceso de 6 meses. También analizamos cuestiones metodológicas relativas al análisis de descargas, en el sentido de la necesidad de una normalización de los datos que permita determinar las descargas reales, asimilables a una lectura del documento. Durante los años 2008 y 2009, ambas revistas han recibido cerca de 500.000 descargas normalizadas, que provienen en su mayoría de Latinoamérica y Estados Unidos (70%). Los datos muestran el elevado número de descargas repetidas de documentos en periodos cortos de tiempo por el mismo usuario (25% de las totales registradas) y de descargas de indización (25% de las descargas normalizadas), y también la falta de correlación entre visibilidad de las revistas (visitas a páginas web) y uso de los contenidos (descarga de documentos). La restricción de acceso tiene un efecto perjudicial, evidenciado por el bajo nivel de descarga de los documentos sometidos a embargo.In this article we analyze the online visibility and content usage of two Americanist Journals published by CSIC, Revista de Indias and Anuario de Estudios Americanos. The online edition dates back to 2007; contents are comparable in terms of accessible documents, and both Journals are published in Open Access, with a 6-months embargo. We also discuss some methodological issues in terms of the need of a data normalization allowing the measurement of genuine downloads assimilable to document reading. Both Journals registered close to 500,000 normalized downloads along 2008 and 2009, most coming from Latin America and the United States (70%). Data show a high degree of repeated downloads of the same document from the same user (25% of total downloads) and indexation downloads (25% of normalized downloads), and also the lack of correlation between Journal`s visibility (webpage visits) and content usage (document downloads). Access restriction has a negative effect, as evidenced by the low downloading of embargoed documents

Late-life depression accelerates cognitive impairment and tau-associated pathology in an Alzheimer´s disease model.

Clinical studies suggest that depression could be considered an important risk factor for the future development of cognitive impairment and Alzheimer's disease (AD). In fact, there is a strong association between late-life depression and AD. The age of AD onset has been shown to be accelerated in patients with mild cognitive impairment (MCI) with a history of depression, and women appear to be particularly more vulnerable to this condition. In addition, individuals with MCI who present depressive symptoms have an elevated burden of amyloid-beta (Aβ), the main toxic protein associated with Alzheimer's pathology, and a higher risk of developing AD compared to non-depressed MCI patients. Although it has been described that some transgenic models of AD can develop signs similar to depression in advanced stages, the induction of Alzheimer's pathology due to a depressive process has not been studied under experimental conditions to emulate late-life depression as a risk factor for AD. In this study, we induced chronic unpredictable mild stress (CUMS) in P301S tau transgenic mice to determine whether depression is a cause, rather than a consequence, of the development of AD pathology. Our results suggest that transgenic tau mice subjected to CUMS seem to develop a depressive state. This animals display enhanced cognitive impairment compared to controls. In addition, histological studies show increased tau deposition, suggesting that late-life depression could worse AD progression by accelerating tau aggregation and worsening clinical signs. The findings generated in this project could provide evidence of depression as a risk factor for AD, providing new insights on molecular mechanisms involved in AD onset and progression.Universidad de Málaga. Campus de Excelencia Internacional Andalucía Tech

La Enseñanza Musical de adultos en el Conservatorio Profesional de Música de Ceuta

La enseñanza musical reglada no se contempla generalmente para adultos en los Conservatorios; el límite de plazas y la normativa que establece la prioridad a los niños a partir de 8 años, son los motivos que provocan la imposibilidad de cursarlas a los adultos. En el Conservatorio de Ceuta, debido a la baja demanda infantil durante algunos años, las plazas ofertadas quedaron vacantes y el conservatorio abrió la matrícula a adultos con inquietudes musicales. Esto ha posibilitado la transmisión de estas enseñanzas durante cinco años a adultos, obteniendo una respuesta exitosa y muy satisfactoria tanto para  alumnos como profesores. En este trabajo pretendemos mostrar este caso excepcional que tiene lugar en el Conservatorio Profesional de Música de Ceuta, analizando las causas y los efectos que este tipo de enseñanza produce en toda la comunidad educativa y de esta manera demostrar los beneficios de expansión cultural y dar continuidad a una enseñanza tan específica como la música al igual que ocurre en otros niveles educativos

Creación del aula musical de adultos en el Conservatorio Profesional de Música Ángel García Ruiz de Ceuta

Durante el curso 2014-2015, el Conservatorio de Ceuta ha llevado a cabo un nuevo proyecto que ha consistido en la creación de un Aula Musical para adultos, con el objetivo de difundir la cultura musical y dar a conocer nuestra institución a un mayor número de personas. La idea de este proyecto surge a raíz de los siguientes hechos: En el Conservatorio de Ceuta, debido a la baja demanda infantil durante algunos años, muchas de las plazas ofertadas quedaron vacantes y el conservatorio abrió la matrícula a adultos con inquietudes musicales. Esta circunstancia posibilitó la transmisión de estas enseñanzas a grupos de adultos. El problema aparecía una vez que estos alumnos se encontraban terminando las enseñanzas elementales, tenían que decidir si continuar con las enseñanzas profesionales, que requerían muchas horas de dedicación, o abandonar el proceso de aprendizaje emprendido y las relaciones interpersonales establecidas con sus compañeros y profesores. En la mayoría de los casos ninguna de las opciones les convencía. En este trabajo pretendemos relatar en qué ha consistido el proyecto del Aula Musical, ya que creemos que ha sido una experiencia muy positiva para toda la comunidad educativa y para la expansión de la cultura musical

CIBERER : Spanish national network for research on rare diseases: A highly productive collaborative initiative

Altres ajuts: Instituto de Salud Carlos III (ISCIII); Ministerio de Ciencia e Innovación.CIBER (Center for Biomedical Network Research; Centro de Investigación Biomédica En Red) is a public national consortium created in 2006 under the umbrella of the Spanish National Institute of Health Carlos III (ISCIII). This innovative research structure comprises 11 different specific areas dedicated to the main public health priorities in the National Health System. CIBERER, the thematic area of CIBER focused on rare diseases (RDs) currently consists of 75 research groups belonging to universities, research centers, and hospitals of the entire country. CIBERER's mission is to be a center prioritizing and favoring collaboration and cooperation between biomedical and clinical research groups, with special emphasis on the aspects of genetic, molecular, biochemical, and cellular research of RDs. This research is the basis for providing new tools for the diagnosis and therapy of low-prevalence diseases, in line with the International Rare Diseases Research Consortium (IRDiRC) objectives, thus favoring translational research between the scientific environment of the laboratory and the clinical setting of health centers. In this article, we intend to review CIBERER's 15-year journey and summarize the main results obtained in terms of internationalization, scientific production, contributions toward the discovery of new therapies and novel genes associated to diseases, cooperation with patients' associations and many other topics related to RD research

Primary and Secondary Immunodeficiency Diseases in Oncohaematology: Warning Signs, Diagnosis, and Management

Background: Immunodeficiencies (ID), in particular primary immunodeficiencies (PID), are often associated with haematological manifestations, such as peripheral cytopenias or lymphoproliferative syndromes. Early diagnosis and management have significant prognostic implications. Secondary immunodeficiencies (SID) may also be induced by oncohaematological diseases and their treatments. Haematologists and oncologists must therefore be aware of the association between blood disorders and cancer and ID, and be prepared to offer their patients appropriate treatment without delay. Our aim was to define the warning signs of primary and secondary IDs in paediatric and adult patients with oncohaematological manifestations.Methods: A multidisciplinary group of six experts (2 haematologists, 2 immunologists, and 2 paediatricians specializing in ID) conducted a literature review and prepared a document based on agreements reached an in-person meeting. An external group of 44 IDs specialists from all over Spain assessed the document and were consulted regarding their level of agreement.Results: This document identifies the haematological and extra-haematological diseases that should prompt a suspicion of PIDs in adults and children, in both primary care and haematology and oncology departments. Cytopenia and certain lymphoproliferative disorders are key diagnostic pointers. The diagnosis must be based on a detailed clinical history, physical exploration, complete blood count and standard laboratory tests. The immunological and haematological tests included in the diagnostic process will depend on the care level. Patients who are candidates for immunoglobulin replacement therapy must be carefully selected, and treatment should be offered as soon as possible to avoid the development of complications. Finally, this document recommends procedures for monitoring these patients.Conclusions: This document combines scientific evidence with the opinion of a broad panel of experts, and emphasizes the importance of an early diagnosis and treatment to avoid complications. The resulting document is a useful tool for primary care physicians and specialists who see both adult and paediatric patients with oncohaematological diseases

Minimal Symptom Expression' in Patients With Acetylcholine Receptor Antibody-Positive Refractory Generalized Myasthenia Gravis Treated With Eculizumab

The efficacy and tolerability of eculizumab were assessed in REGAIN, a 26-week, phase 3, randomized, double-blind, placebo-controlled study in anti-acetylcholine receptor antibody-positive (AChR+) refractory generalized myasthenia gravis (gMG), and its open-label extension

Post-intervention Status in Patients With Refractory Myasthenia Gravis Treated With Eculizumab During REGAIN and Its Open-Label Extension

OBJECTIVE: To evaluate whether eculizumab helps patients with anti-acetylcholine receptor-positive (AChR+) refractory generalized myasthenia gravis (gMG) achieve the Myasthenia Gravis Foundation of America (MGFA) post-intervention status of minimal manifestations (MM), we assessed patients' status throughout REGAIN (Safety and Efficacy of Eculizumab in AChR+ Refractory Generalized Myasthenia Gravis) and its open-label extension. METHODS: Patients who completed the REGAIN randomized controlled trial and continued into the open-label extension were included in this tertiary endpoint analysis. Patients were assessed for the MGFA post-intervention status of improved, unchanged, worse, MM, and pharmacologic remission at defined time points during REGAIN and through week 130 of the open-label study. RESULTS: A total of 117 patients completed REGAIN and continued into the open-label study (eculizumab/eculizumab: 56; placebo/eculizumab: 61). At week 26 of REGAIN, more eculizumab-treated patients than placebo-treated patients achieved a status of improved (60.7% vs 41.7%) or MM (25.0% vs 13.3%; common OR: 2.3; 95% CI: 1.1-4.5). After 130 weeks of eculizumab treatment, 88.0% of patients achieved improved status and 57.3% of patients achieved MM status. The safety profile of eculizumab was consistent with its known profile and no new safety signals were detected. CONCLUSION: Eculizumab led to rapid and sustained achievement of MM in patients with AChR+ refractory gMG. These findings support the use of eculizumab in this previously difficult-to-treat patient population. CLINICALTRIALSGOV IDENTIFIER: REGAIN, NCT01997229; REGAIN open-label extension, NCT02301624. CLASSIFICATION OF EVIDENCE: This study provides Class II evidence that, after 26 weeks of eculizumab treatment, 25.0% of adults with AChR+ refractory gMG achieved MM, compared with 13.3% who received placebo

Search for top squarks in the four-body decay mode with single lepton final states in proton-proton collisions at s \sqrt{s} = 13 TeV

A search for the pair production of the lightest supersymmetric partner of the top quark, the top squark (t∼1), is presented. The search targets the four-body decay of the t∼1, which is preferred when the mass difference between the top squark and the lightest supersymmetric particle is smaller than the mass of the W boson. This decay mode consists of a bottom quark, two other fermions, and the lightest neutralino (χ∼01), which is assumed to be the lightest supersymmetric particle. The data correspond to an integrated luminosity of 138 fb−1 of proton-proton collisions at a center-of-mass energy of 13 TeV collected by the CMS experiment at the CERN LHC. Events are selected using the presence of a high-momentum jet, an electron or muon with low transverse momentum, and a significant missing transverse momentum. The signal is selected based on a multivariate approach that is optimized for the difference between m(t∼1) and m(χ∼01). The contribution from leading background processes is estimated from data. No significant excess is observed above the expectation from standard model processes. The results of this search exclude top squarks at 95% confidence level for masses up to 480 and 700 GeV for m(t∼1) − m(χ∼01) = 10 and 80 GeV, respectively