Hereditary ataxias and paediatric neurology: new movers and shakers enter the field

Over 25 autosomal dominant and autosomal recessive spinocerebellar ataxias have been isolated over the last decade. The recognition of paediatric ataxia phenotypes and, in addition, other movement disorders including hereditary choreiform and parkinsonian syndromes, has improved our knowledge of these diseases. Advances in molecular genetics has allowed fuller delineation and better recognition of these diseases. (C) 2003 European Paediatric Neurology Society. Published by Elsevier Ltd. All rights reserved.

Diálogo entre tradição e contemporaneidade na música erudita do século XX: a obra sinfónica do compositor chinês Zhu Jian-ER

O presente trabalho tem como objectivo estudar o processo dialógico que se manifesta entre a tradição e a contemporaneidade, nas culturas Oriental e Ocidental, em particular na nova música erudita chinesa do século XX. Evocando uma análise diversificada, e um estudo sobre a “individualidade do diálogo”, aprofundamos o nosso conhecimento sobre o compositor Zhu Jian-Er e a sua obra, enquanto manifesto individual das características e da determinação de um novo fazer artístico. Nesse estudo, trata-se, por um lado, de estudar de que forma a obra de Zhu espelha, e expressa, o espaço dialógico, examinando como a observação e a percepção individual se redimensionam nas diferentes vivências sócio-históricas e políticas do autor, e estas, na história da cultura chinesa do século XX. Procuramos ainda perceber como o auto-conhecimento e a personalidade de Zhu se declaram na sua última fase criativa, e a maneira como as diversas construções técnicoestéticas se edificam e interactuam num ponto de encontro único, e, por sua vez, se influenciam reciprocamente no processo dialógico. A estruturação do presente trabalho contém três partes, abrangendo a narração do contexto sócio-histórico da produção musical de Zhu Jian-Er, a pesquisa sobre a vida e obra do autor e, a análise detalhada da sua Décima Sinfonia. Desvendaremos ainda de que forma integra e interage com os diferentes elementos técnico, estilísticos e estéticos que obtém do contacto com diversas manifestações artísticas, não só da música erudita ocidental, como da música erudita oriental, em particular da tradição musical chinesa, determinando a forma como engloba os elementos que obtém na produção de arte. A maneira como constrói o diálogo entre as duas culturas, e que revela, posteriormente, na produção da sua Décima Sinfonia, constituirá, assim, o âmago deste trabalho.

Using Workload Measurement Tools in Diverse Care Contexts: the Experience of Staff in Mental Health and Learning Disability Inpatient Settings

Introduction The critical challenge of determining the correct level and skill-mix of nursing staff required to deliver safe and effective healthcare has become an international concern. It is recommended that evidence-based staffing decisions are central to the development of future workforce plans. Workforce planning in mental health and learning disability nursing is largely under-researched with few tools available to aid the development of evidence-based staffing levels in these environments. Aim It was the aim of this study to explore the experience of staff using the Safer Nursing Care Tool (SNCT) and the Mental Health and Learning Disability Workload Tool (MHLDWT) in mental health and learning disability environments. Method Following a 4-week trial period of both tools a survey was distributed via Qualtrics on-line survey software to staff members who used the tools during this time. Results The results of the survey revealed that the tools were considered a useful resource to aid staffing decisions; however specific criticisms were highlighted regarding their suitability to psychiatric intensive care units (PICU) and learning disability wards. Discussion This study highlights that further development of workload measurement tools is required to support the implementation of effective workforce planning strategies within mental health and learning disability services. Implications for Practice With increasing fiscal pressures the need to provide cost-effective care is paramount within NHS services. Evidence-based workforce planning is therefore necessary to ensure that appropriate levels of staff are determined. This is of particular importance within mental health and learning disability services due to the reduction in the number of available beds and an increasing focus on purposeful admission and discharge.

What is universal and what differs in language development?

Goldin-Meadow (2015) presents an exceptional synthesis of work from studies of children acquiring language under variable circumstances of input or processing abilities. Deaf children who acquire homesign without any well- formed model from which to learn language represent a powerful example. Goldin-Meadow argues that the resilient properties of language that nevertheless emerge include simple syntactic structures, hierarchical organisa- tion, markers modulating the meaning of sentences, and social-communicative functions. Among the fragile or input-dependent properties are the orders that the language follows, the parts into which words are decomposed, and the features that distinguish nominals from predicates. Separation of these two types of properties poses questions concerning the innate constraints on language acquisition (perhaps these equate to the resilient properties) and con‐ cerning the specificity of processes to language (e.g., whether properties such as hierarchical organisation are specific to language or originate in the structure of thought). The study of the resilient properties of human language in the face of adversity and the relation of these properties to the information that is encoded in the human genome represent a research strategy that draws inferences about species universals (properties that all humans share) from data about individual differences (IDs; factors that make humans different from one another). In the following, we suggest three reasons to be cautious about this approach.

Prática de ensino supervisionado no 1.º e 2.º ciclo do ensino básico: desenvolver competências de recolha e tratamento da informação na aprendizagem da História e Geografia no 1.º e 2.º CEB

Relatório de investigação apresentado à Escola Superior de Educação de Lisboa para obtenção de grau de mestre no Mestrado em Ensino do 1.º e 2.º Ciclo do Ensino Básico

L'Écho de Paris

1894/10/20 (Numéro 3798).

L'Oye Royalle, tirée devant leurs Maiestez. Prosopopée : ou, l'Oye qui parle

[Mazarinade. 1649]

Resoluciones Cámara de Comercio de Bogotá

Doctrina

Appleton's northern and eastern Traveller's guide

Full Title: Appleton's northern and eastern Traveller's guide : with new and authentic maps, illustrating those divisions of the country; forming likewise a complete guide to the Middle States, Canada, New Brunswick, and Nova Scotia, the White Mountains, Catskill Mountains, Niagara and Trenton Falls, Saratoga and Virginia Springs andc.; with the fashionable and healthful resort, and full and accurate descriptions of the principal cities, towns, villages; with distances, fares, andc.; illustrated with numerous maps and plans of cities, engraved on steel, and several wood engravings

Modelo de comercialización basado en comercio electrónico

Tesis (Maestría en Administración de Empresas con Especialidad en Mercadotecnia) U.A.N.L.

Étude empirique de distributions associées à la Fonction de Pénalité Escomptée

On présente une nouvelle approche de simulation pour la fonction de densité conjointe du surplus avant la ruine et du déficit au moment de la ruine, pour des modèles de risque déterminés par des subordinateurs de Lévy. Cette approche s'inspire de la décomposition "Ladder height" pour la probabilité de ruine dans le Modèle Classique. Ce modèle, déterminé par un processus de Poisson composé, est un cas particulier du modèle plus général déterminé par un subordinateur, pour lequel la décomposition "Ladder height" de la probabilité de ruine s'applique aussi. La Fonction de Pénalité Escomptée, encore appelée Fonction Gerber-Shiu (Fonction GS), a apporté une approche unificatrice dans l'étude des quantités liées à l'événement de la ruine été introduite. La probabilité de ruine et la fonction de densité conjointe du surplus avant la ruine et du déficit au moment de la ruine sont des cas particuliers de la Fonction GS. On retrouve, dans la littérature, des expressions pour exprimer ces deux quantités, mais elles sont difficilement exploitables de par leurs formes de séries infinies de convolutions sans formes analytiques fermées. Cependant, puisqu'elles sont dérivées de la Fonction GS, les expressions pour les deux quantités partagent une certaine ressemblance qui nous permet de nous inspirer de la décomposition "Ladder height" de la probabilité de ruine pour dériver une approche de simulation pour cette fonction de densité conjointe. On présente une introduction détaillée des modèles de risque que nous étudions dans ce mémoire et pour lesquels il est possible de réaliser la simulation. Afin de motiver ce travail, on introduit brièvement le vaste domaine des mesures de risque, afin d'en calculer quelques unes pour ces modèles de risque. Ce travail contribue à une meilleure compréhension du comportement des modèles de risques déterminés par des subordinateurs face à l'éventualité de la ruine, puisqu'il apporte un point de vue numérique absent de la littérature.

Spatial and temporal characteristics of rain intensity in the peninsular Malaysia using TRMM rain rate

The present study is focused on the intensity distribution of rainfall in different classes and their contribution to the total seasonal rainfall. In addition, we studied the spatial and diurnal variation of the rainfall in the study areas. For the present study, we retrieved data from TRMM (Tropical Rain Measuring Mission) rain rate available in every 3 h temporal and 25 km spatial resolutions. Moreover, station rainfall data is used to validate the TRMM rain rate and found significant correlation between them (linear correlation coefficients are 0.96, 0.85, 0.75 and 0.63 for the stations Kota Bharu, Senai, Cameron highlands and KLIA, respectively). We selected four areas in the Peninsular Malaysia and they are south coastal, east coastal, west coastal and highland regions. Diurnal variation of frequency of rain occurrence is different for different locations. We noticed bimodal variation in the coastal areas in most of the seasons and unimodal variation in the highland/inland area. During the southwest monsoon period in the west coastal stations, there is no distinct diurnal variation. The distribution of different intensity classes during different seasons are explained in detail in the results

Matejuegos. Grupo CIMAT.

Colección de diversas propuestas didácticas a través de juegos matemáticos planteados en estrecha correspondencia con los contenidos curriculares. Los juegos se dirigen especialmente a los alumnos que comienzan la enseñanza secundaria y pretenden la consolidación de conceptos matemáticos y la automatización de algoritmos de cálculo. Presentan, en la mayoría de los casos, un carácter cooperativo dentro del equipo, con lo que se potencia el aprendizaje entre iguales, la tolerancia y la colaboración. Cada uno de los juegos propuestos cuenta con información precisa para su aplicación y con plantillas para que puedan ser reproducidos fácilmente.

Unidad interdisciplinar : Los Sitios de Zaragoza.

Seleccionado en la convocatoria: Ayudas a la innovación e investigación educativa en centros docentes de niveles no universitarios, Gobierno de Aragón 2008-09