233 resultados para DOWN-SYNDROME


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This paper explores the scope to bridge top-down and bottom-up perspectives on spatial planning by drawing on EU-funded action research in relation to rural settlement planning in Northern Ireland. The empirical work is located within a review of planning theory that exposes a long running tension between the technocratic stances of government planners and the aspirations of engaged citizens. It demonstrates the operation of a large group planning methodology that delivers community preference with environmental responsibility as a participatory input into planning policy formulation. Transferable insights into the dynamics of spatial planning are identified.

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Since the publication of Hobsbawm and Rudé's Captain Swing our understanding of the role(s) of covert protests in Hanoverian rural England has advanced considerably. Whilst we now know much about the dramatic practices of incendiarism and animal maiming and the voices of resistance in seemingly straightforward acquisitive acts, one major gap remains. Despite the fact that almost thirty years have passed since E. P. Thompson brought to our attention that under the notorious ‘Black Act’ the malicious cutting of trees was a capital offence, no subsequent research has been published. This paper seeks to address this major lacuna by systematically analysing the practices and patterns of malicious attacks on plants (‘plant maiming’) in the context of late eighteenth- and early nineteenth-century southern England. It is shown that not only did plant maiming take many different forms, attacking every conceivable type of flora, but also that it was universally understood and practised. In some communities plant maiming was the protestors' weapon of choice. As a social practice it therefore embodied wider community beliefs regarding the defence of plebeian livelihoods and identities.

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The acute myeloid leukaemia (AML)14 trial addressed four therapeutic questions in patients predominantly aged over 60 years with AML and High Risk Myelodysplastic Syndrome: (i) Daunorubicin 50 mg/m(2) vs. 35 mg/m(2); (ii) Cytarabine 200 mg/m(2) vs. 400 mg/m(2) in two courses of DA induction; (iii) for part of the trial, patients allocated Daunorubicin 35 mg/m(2) were also randomized to receive, or not, the multidrug resistance modulator PSC-833 in a 1:1:1 randomization; and (iv) a total of three versus four courses of treatment. A total of 1273 patients were recruited. The response rate was 62% (complete remission 54%, complete remission without platelet/neutrophil recovery 8%); 5-year survival was 12%. No benefits were observed in either dose escalation randomization, or from a fourth course of treatment. There was a trend for inferior response in the PSC-833 arm due to deaths in induction. Multivariable analysis identified cytogenetics, presenting white blood count, age and secondary disease as the main predictors of outcome. Although patients with high Pgp expression and function had worse response and survival, this was not an independent prognostic factor, and was not modified by PSC-833. In conclusion, these four interventions have not improved outcomes in older patients. New agents need to be explored and novel trial designs are required to maximise prospects of achieving timely progress.

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Williams syndrome is a genetic disorder that, it has been claimed, results in an unusual pattern of linguistic strengths and weaknesses. The current study investigated the hypothesis that there is a reduced influence of lexical knowledge on phonological short-term memory in Williams syndrome. Fourteen children with Williams syndrome and 2 vocabulary la matched control groups, 20 typically developing children and 13 children with learning difficulties, were tested on 2 probed serial-recall tasks. On the basis of previous findings, it was predicted that children with Williams syndrome would demonstrate (a) a reduced effect of lexicality on the recall of list items, (b) relatively poorer recall of list items compared with recall of serial order, and (c) a reduced tendency to produce lexicalization errors in the recall of nonwords. in fact, none of these predictions were supported. Alternative explanations for previous findings and implications for accounts of language development in Williams syndrome are discussed.