859 resultados para Nijmegen-breakage-syndrome


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The abdominal compartment syndrome (ACS) is the result of various physiological alterations produced by an abnormal increase of the intra-abdominal pressure. Some of these patients will undergo a surgical procedure for its management. Methods: This is a retrospective case series of 28 patients with ACS who required surgical treatment at the Hospital Occidente de Kennedy between 1999 and 2003. We assessed retrospectively the behavior of McNelis’s equation for prediction of the development of the ACS. Results: The leading cause of ACS in our study was intraabadominal infection (n=6 21,4%). Time elapsed between diagnosis and surgical decompression was less than 4 hours in 75% (n=21) of the cases. The variables that improved significantly after the surgical decompression were CVP (T: 4,0 p: 0,0001), PIM (T: 2,7; p: 0,004), PIA (T1,8; p:0,034) and Urine Output (T:-2,4; p:0,02). The values of BUN, Creatinine and the cardiovascular instability did not show improvement. The ICU and hospital length of stay were 11 days (SD: 9) and 18 days (SD13) respectively. Global mortality was 67,9% (n=19) and mortality directly attributable to the syndrome was 30% (n=8). The behavior of the McNelis’s equation was erratic. Conclusions: The demographic characteristics as well as disease processes associated with ACS are consistent with the literature. The association between physiological variables and ACS is heterogeneous between patients. Mortality rates attributable to ACS in our institution are within the range described world-wide. The behavior of the McNelis’s equation seems to depend greatly upon fluid balance.

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Recurso de bolsillo que ayuda a los profesores y al personal de apoyo a entender las dificultades de los alumnos con síndrome de Asperger y les ofrece estrategias para superar los retos que plantean en el aula. El primer capítulo explica exactamente lo que es, y lo que no es, el citado síndrome, enumera sus características y destaca las complicaciones y los placeres de tener un alumno con estas dificultades en clase. Los capítulos siguientes hacen frente a desafíos específicos: deterioro social; intereses obsesivos; problemas con el cambio; la etapa de la rabia, la etapa de recuperación. Tiene ejemplos ilustrativos y soluciones prácticas. Hace referencia a recursos adicionales, programas de enseñanza, sitios web.

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Recurso que ofrece enfoques y estrategias que los profesores pueden utilizar para ayudar a sus estudiantes con síndrome de Asperger y autismo en el camino hacia el éxito. Analiza los problemas que pueden surgir en el aula de inclusión y cómo los educadores pueden hacer adaptaciones para atender a sus alumnos con autismo sin interferir en las rutinas del aula estándar. Incluye información sobre lo que puede causar ansiedad en el estudiante con estas discapacidades, los posibles incrementos en los problemas de comportamiento, y lo que el profesor puede hacer para ayudar. Cuenta con diez estrategias acompañadas de ejemplos y razones por las que son importantes usarlas.

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Entrevista realizada a Andreu van Hooft, profesor titular de la Sección de Español del Departamento de Ciencias de la Comunicación y la Información en la Facultad de letras de la Universidad Radboud de Nijmegen (Países Bajos) y miembro del Comité científico del Congreso Internacional de Español para Fines Específicos (CIEFE). Responde a preguntas sobre cómo ha ido evolucionado el Congreso en cuanto a temática, a las investigaciones y a los campos de interés en sus diferentes ediciones, el estudio del español en los Paises Bajos y su promoción.

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A dança é uma actividade de grande exigência atlética, que pode conduzir a um elevado número de lesões, particularmente na região do tornozelo, possivelmente devido à amplitude extrema do movimento articular de flexão plantar do mesmo, que os bailarinos, especialmente do sexo feminino possuem, para realizar a ponta e meia ponta tão características do ballet clássico (Kadel, 2006; Motta-Valencia, 2006; Russel, Kruse, Koutedakis, McEwan, Wyon, 2010). Estas posições de flexão plantar extrema produzem força excessiva na região posterior do tornozelo, o que muitas vezes pode resultar em conflito, dor e incapacidade, representando na maioria das vezes um desafio de diagnóstico. O síndrome do conflito posterior do tornozelo refere-se a um grupo de entidades patológicas que resultam da flexão plantar forçada do tornozelo, de forma repetitiva ou traumática, causando um conflito das estruturas ósseas e/ou de tecidos moles (Hamilton, Geppert, Thompson, 1996; Hamilton, 2008) . Os objectivos deste projecto são compreender os quais os factores de risco, mecânicos e funcionais que contribuem para a mecânica patológica da lesão descritos na literatura, e proceder a uma avaliação biomecânica do movimento de flexão plantar do tornozelo. Método. Realizar uma revisão sistemática de literatura dirigida á mecânica patológica do síndrome do conflito posterior do tornozelo em bailarinas e conduzir um estudo caso-controlo, cujo objectivo é avaliar, comparar e descrever o movimento da flexão plantar do tornozelo realizado ao efectuar os movimentos de ponta e meia-ponta, em bailarinas pré-profissionais com e sem lesão recorrente resultante do conflito posterior do tornozelo. Resultados. Não foram encontrados estudos relacionados especificamente com a mecânica patológica do tornozelo, no entanto vários estudos foram encontrados considerando as características clínicas e anatómicas assim como os procedimentos de tratamento, indicando que os principais factores de risco relacionados com a lesão se dividem em factores mecânicos e funcionais que quando combinados entre si e associados ao sobre-uso podem resultar no conflito posterior do tornozelo. Na avaliação do movimento foram observadas diferenças na actividade muscular entre os sujeitos com lesão e controlos, tendo sido possível a observação de um padrão na sequência de activação para um dos movimentos testados. Na oscilação postural e na rigidez do tornozelo foram também observadas diferenças entre os sujeitos bem como entre as posições realizadas. Conclusão. Concluiu-se que não sendo possível alterar a anatomia do bailarino, por vezes é possível intervir a nível funcional melhorando a capacidade técnica de forma obter um melhor desempenho e a actuar preventivamente em relação às lesões, uma vez que estas podem apresentar padrões cinéticos próprios, relacionados com a função muscular, a estabilidade postural e a rigidez articular.

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This paper reviews a study to determine the maximum rate the acoustic reflex can follow pulsed stimuli in normal hearing subjects and in subjects with Meniere's Syndrome.

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This paper reviews a study to determine the maximum rate the acoustic reflex can follow pulsed stimuli in normal hearing subjects and in subjects with Meniere's Syndrome.

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This study investigated the development of three aspects of linguistic prosody in a group of children with Williams syndrome compared to typically developing children. The prosodic abilities investigated were: (1) the ability to understand and use prosody to make specific words or syllables stand out in an utterance (focus); (2) the ability to understand and use prosody to disambiguate complex noun phrases (chunking); (3) the ability to understand and use prosody to regulate conversational behaviour (turn-end). The data were analysed using a cross-sectional developmental trajectory approach. The results showed that, relative to chronological age, there was a delayed onset in the development of the ability of children with WS to use prosody to signal the most important word in an utterance (the focus function). Delayed rate of development was found for all the other aspects of expressive and receptive prosody under investigation. However, when non-verbal mental age was taken into consideration, there were no differences between the children with WS and the controls neither with the onset nor with the rate of development for any of the prosodic skills under investigation apart from the ability to use prosody in order to regulate conversational behaviour. We conclude that prosody is not a ‘preserved’ cognitive skill in WS. The genetic factors, development in other cognitive domains and environmental influences affect developmental pathways and as a result, development proceeds along an atypical trajectory.

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This study looks at idiom comprehension by French-speaking people with Williams’ syndrome (WS) and metapragmatic knowledge is examined. Idiomatic expressions are a nonliteral form of language where there is a considerable difference between what is said (literal interpretation) and what is meant (idiomatic interpretation). WS is characterized by a relatively preserved formal language, social interest and poor conversational skills. Using this framework, the present study aims to explore the comprehension of idiomatic expressions by 20 participants with WS. Participants performed a story completion task (comprehension task), and a task of metapragmatic knowledge to justify their chosen answers. WS performances were compared to typically developing children with the same verbal mental age. The main results can be summarized as follows: (1) People with WS have difficulties to understand idioms; (3) WS group seems to perform partly as typically developing children for the acquisition of metapragmatic knowledge of linguistic convention: there is a progressive increase in metapragmatic knowledge of linguistic convention as age increased. Our results indicate a delay of acquisition in idiom comprehension in Williams’ syndrome.

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Many time series are measured monthly, either as averages or totals, and such data often exhibit seasonal variability-the values of the series are consistently larger for some months of the year than for others. A typical series of this type is the number of deaths each month attributed to SIDS (Sudden Infant Death Syndrome). Seasonality can be modelled in a number of ways. This paper describes and discusses various methods for modelling seasonality in SIDS data, though much of the discussion is relevant to other seasonally varying data. There are two main approaches, either fitting a circular probability distribution to the data, or using regression-based techniques to model the mean seasonal behaviour. Both are discussed in this paper.

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Two studies examine the experience of “earworms”, unwanted catchy tunes that repeat. Survey data show that the experience is widespread but earworms are not generally considered problematic, although those who consider music to be important to them report earworms as longer, and harder to control, than those who consider music as less important. The tunes which produce these experiences vary considerably between individuals but are always familiar to those who experience them. A diary study confirms these findings and also indicates that, although earworm recurrence is relatively uncommon and unlikely to persist for longer than 24 hours, the length of both the earworm and the earworm experience frequently exceed standard estimates of auditory memory capacity. Active attempts to block or eliminate the earworm are less successful than passive acceptance, consistent with Wegner’s (1994) theory of ironic mental control.