292 research outputs found

    Does Devolution Make a Difference? Legislative Output and Policy Divergence in Scotland

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    Devolution provides large scope for Scotland to make its own policy. Primary legislation is one measure of this. Scottish legislation before devolution tended to replicate measures for the rest of the United Kingdom, with differences of style. Scottish legislation in the first four-year term of the Parliament shows a big increase in output. There is an autonomous sphere, in which Scotland has gone its own way without reference to the rest of the UK. In other areas, there is evidence of joint or parallel policy-making, with Scottish legislation meeting the same goals by different means. Finally there is a sphere in which Scottish legislation is essentially the same as that in England and Wales. Sewel motions have not been used to impose policy uniformity on Scotland. There is evidence that devolution has shifted influence both vertically, between the UK and Scottish levels, and horizontally, within a Scottish legislative system that has been opened up

    When do autistic children exhibit a shape bias? : Investigating the impact of methodology on novel noun generalisation in autism and typical development

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    From ~24 months old, typically developing (TD) children often generalise names for solid objects to novel examples that are the same shape. This ‘shape bias’ can facilitate word learning by providing an attentional short-cut, allowing children to accurately generalise novel nouns. Autistic children often experience delays in language acquisition, and difficulty exploiting the shape bias may be a contributing factor. However, extant research findings are highly inconsistent, with autistic children exhibiting a shape bias in certain tasks but not others. Thus, the current research investigated whether variability in autistic children’s shape bias can be explained by methodological differences. Autistic children (aged 4 to 9 years) and TD children matched on receptive vocabulary (aged 30 months to 4 years) participated in five experiments measuring shape bias in both ‘forced-choice’ and ‘yes or no’ variants of a novel noun generalisation task (NNG). Each task included an ‘online’ condition, where children were asked to generalise a label from a visible standard item to a target, and an ‘offline’ condition where the standard was absent at test, and generalisation had to be completed from memory. Experiment 1 investigated whether the visibility of the standard affected autistic children’s shape bias in a forced-choice task with high contrast stimuli. We found that both autistic and TD groups generalised by shape regardless of standard visibility. Experiment 2 investigated whether shape was still preferred in a yes/no task, where children had the freedom to include any of the stimuli in the category rather than just the best example. In this task, autistic children were more likely to accept the differently shaped distractors than the TD group. Experiments 3 and 4 used the same tasks with low contrast stimuli to investigate whether the requirement to remember the standard had an impact when object shapes were more similar. Both groups again exhibited a shape bias in the forced-choice task, however in the yes/no version only the TD group generalised by shape. Finally, Experiment 5 investigated whether an attentional preference for small details could account for shape bias differences in autism in a yes/no task. We found that both autistic and TD children generalised based on global shape rather than a salient local feature. Overall, our results suggest that methodological variations can explain discrepancies in previous findings regarding shape bias in autism. Autistic children exhibited a strong shape bias in forced-choice tasks, whereas the bias appeared reduced or absent in yes/no tasks requiring children to categorise items individually. This suggests that differences may lie in autistic children’s use of the shape bias as a tool for category exclusion decisions, rather than inclusion decisions, raising questions about the role of the shape bias in word and category learning for all children. There may be multiple routes through which attention to shape can contribute to learning and, by identifying which routes are most accessible for autistic children, we can inform teaching methods that work in harmony with their strengths

    The interaction of silver(II) complexes with biological macromolecules and antioxidants

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    Silver is widely used for its antimicrobial properties, but microbial resistance to heavy metals is increasing. Silver(II) compounds are more oxidizing and therefore have the potential to overcome resistance via extensive attack on cellular components, but have traditionally been hard to stabilize for biological applications. Here, the high oxidation state cation was stabilised using pyridinecarboxylate ligands, of which the 2,6-dicarboxypyridine Ag(II) complex (Ag2,6P) was found to have the best tractability. This complex was found to be more stable in phosphate buffer than DMSO, allowing studies of its interaction with water soluble antioxidants and biological macromolecules, with the aim of demonstrating its potential to oxidize them, as well as determining the reaction products. Spectrophotometric analysis showed that Ag2,6P was rapidly reduced by the antioxidants glutathione, ascorbic acid and vitamin E; the unsaturated lipids arachidonic and linoleic acids, model carbohydrate ÎČ-cyclodextrin, and protein cytochrome c also reacted readily. Analysis of the reaction with glutathione by NMR and electrospray mass spectrometry confirmed that the glutathione was oxidized to the disulfide form. Mass spectrometry also clearly showed the addition of multiple oxygen atoms to the unsaturated fatty acids, suggesting a radical mechanism, and cross-linking of linoleic acid was observed. The seven hydroxyl groups of ÎČ-cyclodextrin were found to be completely oxidized to the corresponding carboxylates. Treatment of cytochrome c with Ag2,6P led to protein aggregation and fragmentation, and dose-dependent oxidative damage was demonstrated by oxyblotting. Thus Ag2,6P was found to be highly oxidizing to a wide variety of polar and nonpolar biological molecules

    Oncologists' perspectives on post-cancer treatment communication and care coordination with primary care physicians

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    Post-treatment cancer care is often fragmented and of suboptimal quality. We explored factors that may affect cancer survivors' post-treatment care coordination, including oncologists' use of electronic technologies such as e-mail and integrated electronic health records (EHRs) to communicate with primary care physicians (PCPs). We used data from a survey (357 respondents; participation rate 52.9%) conducted in 2012-2013 among medical oncologists caring for patients in a large US study of cancer care delivery and outcomes. Oncologists reported their frequency and mode of communication with PCPs, and role in providing post-treatment care. Seventy-five per cent said that they directly communicated with PCPs about post-treatment status and care recommendations for all/most patients. Among those directly communicating with PCPs, 70% always/usually used written correspondence, while 36% always/usually used integrated EHRs; telephone and e-mail were less used. Eighty per cent reported co-managing with PCPs at least one post-treatment general medical care need. In multivariate-adjusted analyses, neither communication mode nor intensity were associated with co-managing survivors' care. Oncologists' reliance on written correspondence to communicate with PCPs may be a barrier to care coordination. We discuss new research directions for enhancing communication and care coordination between oncologists and PCPs, and to better meet the needs of cancer survivors post-treatment

    A NGS-Targeted Autism/ID Panel Reveals Compound Heterozygous GNB5 Variants in a Novel Patient

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    Homozygous and compound heterozygous pathogenic variants in GNB5 have been recently associated with a spectrum of clinical presentations varying from a severe multisystem form of the disorder including intellectual disability, early infantile developmental and epileptic encephalopathy, retinal abnormalities and cardiac arrhythmias (IDDCA) to a milder form with language delay, attention-deficit/hyperactivity disorder, cognitive impairment, with or without cardiac arrhythmia (LADCI). Approximately twenty patients have been described so far; here we report a novel case of a 2.5-year-old female who is a compound heterozygote for a frameshift and a missense variant in the GNB5 gene. Her clinical presentation is consistent with a moderate phenotype, corroborating the direct correlation between the type and pathogenic mechanism of the GNB5 genetic variant and the severity of related phenotype

    Detection of a novel RYR1 mutation in four malignant hyperthermia pedigrees

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    Malignant hyperthermia (MH) is a potentially fatal autosomal dominant disorder of skeletal muscle and is triggered in susceptible people by all commonly used inhalational anaesthetics and depolarizing muscle relaxants. To date, six mutations in the skeletal muscle ryanodine receptor gene (RYR1) have been identified in malignant hyperthermia susceptible (MHS) and central core disease (CCD) cases. Using SSCP analysis, we have screened the RYR1 gene in affected individuals for novel MHS mutations and have identified a G to A transition mutation which results in the replacement of a conserved Gly at position 2433 with an Arg. The Gly2433Arg mutation was present in four of 104 unrelated MHS individuals investigated and was not detected in a normal population sample. This mutation is adjacent to the previously identified Arg2434His mutation reported in a CCD/MH family and indicates that there may be a second region in the RYR1 gene where MHS/CCD mutations cluste

    Age-Gender Influence on the Rate-Corrected QT Interval and the QT-Heart Rate Relation in Families With Genotypically Characterized Long QT Syndrome

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    AbstractObjectives. We sought to analyze age-gender differences in the rate-corrected QT (QTc) interval in the presence of a QT-prolonging gene.Background. Compared with men, women exhibit a longer QTc interval and an increased propensity toward torsade de pointes. In normal subjects, the QTc gender difference reflects QTc interval shortening in men during adolescence.Methods. QTc intervals were analyzed according to age (<16 or ≄16 years) and gender in 460 genotyped blood relatives from families with long QT syndrome linked to chromosome 11p (KVLQT1; n = 199), 7q (HERG; n = 208) or 3p (SCN5A; n = 53).Results. The mean QTc interval in genotype-negative blood relatives (n = 240) was shortest in men, but similar among women, boys and girls. For genotype-positive blood relatives, men exhibited the shortest mean QTc interval in chromosome 7q- and 11p-linked blood relatives (n = 194), but not in the smaller 3p-linked group (n = 26). Among pooled 7q- and 11p-linked blood relatives, multiple regression analysis identified both genotype (p < 0.001) and age-gender group (men vs. women/children; p < 0.001) as significant predictors of the QTc interval; and heart rate (p < 0.001), genotype (p < 0.001) and age-gender group (p = 0.01) as significant predictors of the absolute QT interval. A shorter mean QT interval in men was most evident for heart rates <60 beats/min.Conclusions. In familial long QT syndrome linked to either chromosome 7q or 11p, men exhibit shorter mean QTc values than both women and children, for both genotype-positive and -negative blood relatives. Thus, adult gender differences in propensity toward torsade de pointes may reflect the relatively greater presence in men of a factor that blunts QT prolongation responses, especially at slow heart rates.(J Am Coll Cardiol 1997;29:93–9)
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