Three-year-old child with meroacrania - Neurological signs

Neurological findings in a three-year-old child with meroacrania provide new insights into how the nervous system develops and functions in the absence of superior levels of control from the time of origin. The girl is the first child of a non-consanguineous white Brazilian couple, born at term, weighing 2650 g and measuring 44 cm in length. Upon examination at 43 months, she had quadriplegia, global hypotonia with occasional body hypertonia in a decorticate posture, hyperreflexia, ankle clonus, and extensor plantar response. This case allowed us to verify that, in the absence of upper structures and subcortical nuclei, there are clear signs that suggest corticospinal primacy in motor functions without a substitute pathway. Sound orientation responses suggest the independence of the vestibular-acoustic-ocular system, and manifestations of responsiveness to the environment raise questions about consciousness. (C) 2010 The Japanese Society of Child Neurology. Published by Elsevier B.V. All rights reserved.

Postural adjustment as a sign of attention in 7-month-old infants

Adjustments during postural control have been recognized in the process of the integration of movement and cognition. The objective of the present work was to describe postural changes and to verify if there is a correlation between postural adjustment and attention span in 7-month-old infants during 1 min of viewing an animated puppet. Method: Twenty-nine healthy infants (14 males) born from 31 to 39 weeks (median 36) were placed in a prone position and filmed watching a puppet during I min. The analysis of the images allowed us to catalogue the changes in position, the frequency of these changes, and the attention span. The following items were quantified: total number of infant positions, positions with weight transfer, changes in support, axis, decubitus, trunk and cervical movements, timing and pauses in visual attention. Results: Twenty-one infants stayed in the prone position during most of the recording, and eight chose sitting position before starting the session. Two groups were studied according to the main position throughout the filming, one in prone and the other in sitting positions, although they could rolling or crawling. For prone group the attention span was positively correlated with the number of positions with weight transfer (r = 0.53, p = 0.04), negatively correlated with trunk movements (r = -0.63, p = 0.01), and not correlated with birth or current weight. This work suggests that changes in the trunk movements and weight transfer are different traits related to the attention in 7-month-old infants. (C) 2008 Elsevier B.V.. All rights reserved.

Functional deficiencies of sulfite oxidase: Differential diagnoses in neonates presenting with intractable seizures and cystic encephalomalacia

Sulfite oxidase is a mitochondrial enzyme encoded by the SUOX gene and essential for the detoxification of sulfite which results mainly from the catabolism of sulfur-containing amino acids. Decreased activity of this enzyme can either be due to mutations in the SUOX gene or secondary to defects in the synthesis of its cofactor, the molybdenum cofactor. Defects in the synthesis of the molybdenum cofactor are caused by mutations in one of the genes MOCS1, MOCS2, MOCS3 and GEPH and result in combined deficiencies of the enzymes sulfite oxidase, xanthine dehydrogenase and aldehyde oxidase. Although present in many ethnic groups, isolated sulfite oxidase deficiency and molybdenum cofactor deficiency are rare inborn errors of metabolism, which makes awareness of key clinical and laboratory features of affected individuals crucial for early diagnosis. We report clinical, radiologic, biochemical and genetic data on a Brazilian and on a Turkish child with sulfite oxidase deficiency due to the isolated defect and impaired synthesis of the molybdenum cofactor, respectively. Both patients presented with early onset seizures and neurological deterioration. They showed no sulfite oxidase activity in fibroblasts and were homozygous for the mutations c.1136A>G in the SUOX gene and c.667insCGA in the MOCS1 gene, respectively. Widely available routine laboratory tests such as assessment of total homocysteine and uric acid are indicated in children with a clinical presentation resembling that of hypoxic ischemic encephalopathy and may help in obtaining a tentative diagnosis locally, which requires confirmation by specialized laboratories. (C) 2009 Published by Elsevier B.V.

Pain and distress reactivity and recovery as early predictors of temperament in toddlers born preterm

Background: Pain reactivity may reflect underlying mechanisms of constitutional aspects of temperament. Aim: To examine whether the neonatal biobehavioral reactivity and recovery responses from pain and distress, as well as the gestational age, the illness severity and the amount of painful procedures undergone the Neonatal Intensive Care Unit (NICU) stay, predict temperament later in toddlerhood, in vulnerable children born preterm. Study design: Prospective-longitudinal study. Subjects: Twenty-six preterm and very low birth weight infants followed from birth to toddlerhood. Outcome measures: Illness severity was assessed with the Clinical Risk Index for Babies (CRIB) score. The medical charts were reviewed prospectively for obtaining the amount of pain exposure in NICU. For assessing the behavioral and cardiac reactivity and recovery from pain and distress, the neonates were evaluated during routine blood collection in the NICU in the first 10 days of life. Pain and distress reactivity and recovery was measured using the Neonatal Facial Coding System score, the duration of crying. and the magnitude of average heart rate. At toddlerhood, mothers answered the Early Childhood Behavior Questionnaire. Results: Higher biobehavioral reactivity to pain and distress predicted higher temperamental Negative Affect, above and beyond gestational age, illness severity and amount of pain exposure in NICU. However, we did not find a predictive relation between gestational age, CRIB score and number of painful procedures undergone NICU and toddler`s temperament. Conclusions: The findings highlight the relevance of the neonatal individual characteristics of reactivity for identifying more vulnerable infants for future problems in biobehavioral regulation. (C) 2009 Elsevier Ireland Ltd. All rights reserved.

Short-term urea feeding decreases in vitro hatching of bovine blastocysts

Cows fed high-protein diets may have impaired reproductive performance. Although the pathogenesis has not been completely elucidated, it appears that not only the uterus, but also the follicle and oocyte, are affected by excessive plasma urea nitrogen (PUN) concentrations. Thus, the objective was to determine the effects of short-term urea feeding on the competence of bovine oocytes. Forty crossbred heifers (Bos indicus vs Bos taurus) were allocated to two groups, namely CONTROL (maintenance diet) and UREA (maintenance diet supplemented with 75 g of urea/day), following a cross-over design. Heifers received their respective diets for 6 d (without adaptation). On the sixth day, blood samples were harvested both before and 3 h after feeding, and cumulus oocyte complexes (COCs) were collected by ovum pick-up. Although PUN concentrations were higher in UREA than CONTROL heifers (31.31 mg/dL +/- 1.13 vs 22.12 mg/dL +/- 0.86; mean +/- SEM), neither the number of COCs recovered (8.8 +/- 1.0 vs 9.2 +/- 0.8, UREA vs CONTROL, respectively) nor their quality (based on morphology) differed significantly between groups. Next, oocytes were fertilized and cultured in vitro to assess developmental rates. There was an absence of significant differences between groups for rates of cleavage (Day 3) or blastocyst formation (Days 6, 7 and 9), but the hatched blastocyst rate on Day 11 after fertilization was lower (P < 0.05) in the UREA than the CONTROL groups (64.3 vs 83.5%). Therefore, we inferred that the effects of urea were only manifest later in development. In conclusion, high PUN concentrations decreased oocyte competence in heifers, reinforcing the hypothesis that poor reproductive performance in cows with high PUN was due, at least in part, to a deleterious effect on oocytes. (C) 2011 Elsevier Inc. All rights reserved.

Characterization of language and reading skills in familial polymicrogyria

Polymicrogyria (PMG) is a malformation of cortical development characterized by an excessive number of small gyri and abnormal cortical lamination, giving the cortical surface an irregular and gross appearance. The severity of clinical manifestations correlates with the extent of cortical involvement. The objective of the present study was to describe three families with linguistic features of developmental language disorder and reading impairment, and to establish a neuroanatomic correlation through neuroimaging. Subjects have been submitted to a comprehensive protocol including psychological assessment, language evaluation, neurological examination, and neuroimaging investigation. In our families, children usually had the diagnosis of developmental language disorder while adults had the diagnosis of reading impairment. MRI showed perisylvian polymicrogyria in several subjects of each family. Our data support the idea that there is a co-occurrence of developmental language disorder and reading impairment and both conditions may be associated with polymicrogyria. (c) 2007 Elsevier B.V. All rights reserved.

A sociolinguistic approach to applied epistemology: Examining technocratic values in global 'knowledge' policy

This special issue presents an excellent opportunity to study applied epistemology in public policy. This is an important task because the arena of public policy is the social domain in which macro conditions for ‘knowledge work’ and ‘knowledge industries’ are defined and created. We argue that knowledge-related public policy has become overly concerned with creating the politico-economic parameters for the commodification of knowledge. Our policy scope is broader than that of Fuller (1988), who emphasizes the need for a social epistemology of science policy. We extend our focus to a range of policy documents that include communications, science, education and innovation policy (collectively called knowledge-related public policy in acknowledgement of the fact that there is no defined policy silo called ‘knowledge policy’), all of which are central to policy concerned with the ‘knowledge economy’ (Rooney and Mandeville, 1998). However, what we will show here is that, as Fuller (1995) argues, ‘knowledge societies’ are not industrial societies permeated by knowledge, but that knowledge societies are permeated by industrial values. Our analysis is informed by an autopoietic perspective. Methodologically, we approach it from a sociolinguistic position that acknowledges the centrality of language to human societies (Graham, 2000). Here, what we call ‘knowledge’ is posited as a social and cognitive relationship between persons operating on and within multiple social and non-social (or, crudely, ‘physical’) environments. Moreover, knowing, we argue, is a sociolinguistically constituted process. Further, we emphasize that the evaluative dimension of language is most salient for analysing contemporary policy discourses about the commercialization of epistemology (Graham, in press). Finally, we provide a discourse analysis of a sample of exemplary texts drawn from a 1.3 million-word corpus of knowledge-related public policy documents that we compiled from local, state, national and supranational legislatures throughout the industrialized world. Our analysis exemplifies a propensity in policy for resorting to technocratic, instrumentalist and anti-intellectual views of knowledge in policy. We argue that what underpins these patterns is a commodity-based conceptualization of knowledge, which is underpinned by an axiology of narrowly economic imperatives at odds with the very nature of knowledge. The commodity view of knowledge, therefore, is flawed in its ignorance of the social systemic properties of ��knowing’.

Improving the public management career: report for the ministry of planning, budgeting e management, federal government of Brazil

This report sets out the findings and recommendations of a review of the Public Management Career (EPPGG) undertaken between 5 and 9 January 2009. The purpose of the review was to clarify EPPGG???s role, functions and profile, and to develop proposals for improving its effectiveness and management, taking account of the context within which such improvements would have to be implemented. Specific objectives were to examine a number of human resources management (HRM) aspects of the career in the light of international practice, including recruitment; initial education; professional development; performance evaluation; deployment; mobility and networking.

Programa de Técnica de Dança Clássica : do pré-primário ao 2ª ciclo de escolaridade do Ensino Não Vocacional

Relatório Final de Projecto Pedagógico apresentado à Escola Superior de Dança, com vista à obtenção do grau de Mestre em Metodologias do Ensino da Dança

A inclusão de alunos surdos em agrupamentos sem escolas de referência para a educação bilingue

Este estudo, de natureza qualitativa e de tipo exploratório e descritivo, visa conhecer como se organizam os Agrupamentos sem Escolas de Referência para Educação Bilingue, de forma a dar resposta às necessidades educativas especiais (NEE) dos alunos surdos do 1.º Ciclo do Ensino Básico. Utilizámos a entrevista semi-directiva, de forma a: i) conhecer as percepções dos vários agentes educativos sobre a inclusão dos alunos surdos em turmas regulares do 1º CEB; ii) identificar as formas de adequação curricular mais utilizada para alunos surdos em turmas inclusivas; iii) caracterizar as necessidades dos Agrupamentos sem Escolas de Referência para Educação Bilingue para dar resposta às Necessidades Educativas Especiais dos alunos surdos. Foram entrevistados 10 docentes com diferentes funções no ensino: 2 directores de agrupamento, 3 professores de educação especial, 2 coordenadores de grupo de educação especial, 2 professores titulares e 1 coordenador de conselho de docentes. O cruzamento dos dados resultantes das entrevistas aos professores com diferentes funções nos Agrupamentos sem Escolas de Referência permitiu-nos conhecer as concepções dos profissionais sobre a inclusão em termos gerais e das crianças surdas em particular. Foi-nos possível concluir que os agentes educativos revelam alguma ansiedade profissional relativamente à inclusão dos alunos surdos em turmas regulares, demonstrando uma particular atenção à necessidade de obterem formação específica na área. Relacionam grande parte das limitações sentidas na adequação de respostas para alunos surdos com a precariedade de recursos, quer humanos, quer materiais. Os profissionais equacionam ainda o problema do atendimento a alunos surdos em regiões em que estes são em número reduzido, não justificando a criação de uma Escola de Referência para a Educação Bilingue e reflectem sobre as condições necessárias para que os Agrupamentos que não possuem essas Escolas possam dar uma resposta educativa satisfatória aos alunos surdos

O currículo e as necessidades educativas especiais : práticas de adequação curricular no terceiro ciclo

A responsabilidade da construção de uma escola para todos deve ser o grande objectivo de todo e qualquer professor, criando espaços de reflexão conjunta no sentido de serem traçados projectos curriculares diferenciados que dêem resposta a todos os alunos na sua diversidade plural. Assim, a concepção e construção da escola inclusiva implicam mudanças, quer nas atitudes e práticas dos agentes educativos, quer nas estruturas do sistema de ensino ao nível organizacional e da gestão curricular. Neste âmbito, a adequação do currículo às necessidades educativas especiais (NEE) dos alunos torna-se essencial na (re)construção e operacionalização dos diferentes processos curriculares. O trabalho que agora se apresenta foi desenvolvido através de um estudo de caso. Constitui uma tentativa de compreensão das percepções dos professores e das práticas pedagógicas que desenvolvem para efectuar adequações curriculares para alunos com NEE. Centrámo-nos nos professores de um conselho de turma do sétimo ano do terceiro ciclo do ensino básico (3º CEB) que inclui uma aluna com NEE, procurando saber o que pensam sobre o processo de adequação curricular, como o concebem e como o implementam. Como metodologia de recolha dos dados utilizamos as técnicas da entrevista, da análise documental e da observação naturalista. Concluímos que, apesar das dificuldades que expressam e demonstram, os professores assumem a necessidade de implementar adequações curriculares face às NEE dos alunos, como resposta às diferenças e particularidades de cada um, tendo em vista o desenvolvimento de competências essenciais e a conclusão da escolaridade obrigatória. As Adequações Curriculares são percepcionadas como muito vantajosas do ponto de vista da promoção do sucesso educativo para todos os alunos e em particular para os alunos com NEE, proporcionando a estes, acompanhar o currículo mais próximo do que é normal, sentirem-se como “iguais” aos seus colegas, melhorar a sua auto-estima, auto-confiança e motivação ABSTRACT The responsibility of building a school for all must be the ultimate goal of any teacher, creating spaces for joint reflection in order to draw differentiated curricular projects which respond to all students in their plural diversity. Thus, the design and construction of the inclusive school involve changes both in attitudes and practices of the educational agents, and in the structures of the education system regarding the organization and the curriculum management. In this context, the adequacy of the curriculum to students’ special educational needs (SEN) becomes essential in the (re) construction and operationalization of the different curriculum processes. The work now presented was developed through a case study. It attempts to understand the perceptions of teachers and the pedagogical practices they develop to make curricular adjustments for students with SEN. We focused on the teachers of a seventh year class of the third cycle of basic education (3rd CBE) which includes a pupil, a girl, with SEN, trying to find out what they think about the process of adapting the curriculum, how they conceive and implement it. As a methodology of data collection we used different techniques: the interview, the documentary analysis and the naturalistic observation. We concluded that, despite the difficulties that teachers express and demonstrate they assume the need to implement curricular adjustments in relation to pupils with SEN in response to the differences and particularities of each one in order to develop core competencies and to complete compulsory schooling. The Curriculum Adequacies are perceived as very advantageous from the standpoint of promoting educational success for all students and particularly for pupils with SEN, providing the latter the possibility of following the curriculum closer than usual, feeling as "equal" to their schoolmates, improving their self-esteem, self- confidence and motivation.

O programa de formação dos mentores: conceção e planeamento

Este texto define e enquadra teoricamente as principais finalidades e orientações metodológicas do programa de formação dos mentores que acompanharam os professores em período probatório. O referencial de formação dos mentores construiu-se em torno das diferentes dimensões do ato de ensinar. A metodologia de formação organizou-se a partir do conceito de desenvolvimento profissional, considerando o mentor como objeto e sujeito da formação e procurando articular o processo formativo e o trabalho de supervisão desenvolvido pelos mentores, no terreno. As sessões de formação foram planeadas estrategicamente a partir de questões orientadoras às quais se procurou dar resposta através de um conjunto de atividades e materiais. Durante a implementação do período probatório foi produzido pelos intervenientes um conjunto considerável de documentos que foram trabalhados durante a formação. - Abstract This article defines and frames theoretically the main purposes and the methodological guidelines of the training program of mentors who have accompanied teachers on the probationary period. Those guidelines were constructed around the different dimensions of the act of teaching. The training methodology was organized from the concept of professional development, considering the mentor as object and subject of training and seeking to articulate the formative process and supervision work developed by mentors on the field. Training sessions have been strategically planned from guiding questions which sought to respond through a range of activities and materials. During the implementation of the probationary period, the participants delivered a considerable set of documents, which were worked on during training.- Resumen Este texto define y enfoca teóricamente las principales finalidades y orientaciones metodológicas del programa de formación de los mentores que acompañaron a los profesores en el periodo probatorio. La referencia formativa de los mentores se construyó en torno de las distintas dimensiones del hecho de enseñar. La metodología formativa se organizó a partir del concepto de desarrollo profesional, considerando al mentor como objeto y sujeto de la formación y buscando articular el proceso formativo y el trabajo de supervisión desarrollado por los mentores, en el terreno. Las sesiones de formación se proyectaron estratégicamente a partir de planteos orientadores, a los cuales se les dio respuesta a través de un conjunto de actividades y materiales. Durante la implementación del periodo probatorio, los participantes produjeron un considerable conjunto de documentos que se elaboraron durante la formación.

O desenvolvimento profissional docente: a função supervisiva do coordenador de ano do 1º ciclo

Dissertação apresentada à Escola Superior de Educação de Lisboa para obtenção de grau de mestre em Ciências da Educação, especialidade de Supervisão em Educação

Traduzir o universo: tradução e legendagem de vídeos de divulgação científica

Trabalho de Projeto apresentado ao Instituto de Contabilidade e Administração do Porto para a obtenção do grau de Mestre em Tradução e Interpretação Especializadas, sob orientação da Mestre Graça Chorão