Dorsal and ventral stream mediated visual processing in genetic subtypes of Prader-Willi syndrome

Previous work has suggested that there are specific deficits in dorsal stream processing in a variety of developmental disorders. Prader-Willi syndrome (PWS) is associated with two main genetic subtypes, deletion and disomy. Relative strengths in visual processing are shown in PWS, although these strengths may be specific to the deletion subtype. We investigated visual processing in PWS using an adapted Simon task which contrasted location (dorsal stream) and shape identity (ventral stream) tasks. Compared to a group of typically developing children, children with PWS deletion showed a greater degree of impairment in the dorsal stream task than in the ventral stream task, a pattern similar to that shown in a group of boys with Fragile-X syndrome. When matched on a measure of non-verbal ability, children with PWS disomy showed the opposite pattern with better performance in the location compared to the shape task, although these task performance asymmetries may have been linked to executive control processes. It is proposed that children with PWS deletion show a relative strength in visual processing in the ventral stream along with a specific deficit in dorsal stream processing. In contrast, children with PWS disomy show neither effect. (C) 2009 Published by Elsevier Ltd.

Associations between repetitive questioning, resistance to change, temper outbursts and anxiety in Prader-Willi and Fragile-X syndromes

The behavioural phenotypes of Prader-Willi (PWS) and Fragile-X (FraX) syndromes both comprise repetitive behaviours with differences between the profiles. In this study we investigated the context and antecedents to the repetitive behaviours and the association with other behavioural phenotypic characteristics in order to generate testable hypotheses regarding the cause of the behaviours.

The parents or carers of 46 children with PWS (mean age 14.1 years; 20 girls), and 33 boys with FraX (mean age 13.11 years) were interviewed about their children's repetitive behaviour in a semi-structured format.

Children showed negative emotional behaviour (PWS: 87.0%; FraX: 79.4%) and repetitive questions (PWS: 78.3%; FraX: 73.5%) following changes in routine or expectations. Significantly more temper outbursts were reported to follow changes in children with PWS (89.1%) compared with boys with FraX (41.2%) (chi(2) = 20.93; P <0.001). Anxiety that was frequently associated with repetitive and self-injurious behaviours in boys with FraX, followed changes in significantly more boys with FraX (76.5%) compared with children with PWS (6.5%) (chi(2) = 43.19, P <0.001).

On the basis of these reports and existing literature, we hypothesise that decreases in predictability are aversive to children with PWS and FraX. We also hypothesise that these children have a propensity to show a syndrome-related pattern of behaviour (temper outbursts in PWS and displays of anxiety in FraX) when an event in the environment has this aversive property. We hypothesise that questions may be reinforcing to children in their own right by increasing the predictability of the environment. We outline how a specific cognitive deficit in the endophenotypes associated with both PWS and FraX could be investigated as a potential explanation for the hypothesised aversive properties of decreased predictability.

Task-switching deficits and repetitive behaviour in genetic neurodevelopmental disorders: Data from children with Prader-Willi syndrome chromosome 15 q11-q13 deletion and boys with Fragile X syndrome

Prader-Willi syndrome (PWS) and Fragile X syndrome (FraX) are associated with distinctive cognitive and behavioural profiles. We examined whether repetitive behaviours in the two syndromes were associated with deficits in specific executive functions. PWS, FraX, and typically developing (TD) children were assessed for executive functioning using the Test of Everyday Attention for Children and an adapted Simon spatial interference task. Relative to the TD children, children with PWS and FraX showed greater costs of attention switching on the Simon task, but after controlling for intellectual ability, these switching deficits were only significant in the PWS group. Children with PWS and FraX also showed significantly increased preference for routine and differing profiles of other specific types of repetitive behaviours. A measure of switch cost from the Simon task was positively correlated to scores on preference for routine questionnaire items and was strongly associated with scores on other items relating to a preference for predictability. It is proposed that a deficit in attention switching is a component of the endophenotypes of both PWS and FraX and is associated with specific behaviours. This proposal is discussed in the context of neurocognitive pathways between genes and behaviour.

Further refinement of the nature of the communication impairment in Cornelia de Lange syndrome

Purpose – Research into the communication skills of individuals with Cornelia de Lange syndrome (CdLS) is extremely limited. This paper aims to evaluate the nature of these skills and impairments in CdLS using a detailed informant assessment of pre-verbal communication skills.
Design/methodology/approach – The study used the Pre-verbal Communication Schedule to evaluate communication skills in individuals with CdLS (n ¼ 14), aged five to14 years. The group was compared with a contrast group of individuals with Cri du Chat syndrome (CdCS; n ¼ 14) who were matched for age and intellectual ability.
Findings – A significant difference was identified in understanding non-vocal communication (p , 0.005), with the CdLS group showing a greater deficit. These findings indicate the presence of a syndrome-specific deficit in understanding non-verbal communication in individuals with CdLS and suggest that there may be a dissociation between the processing of verbal and non-verbal communication.
Originality/value – The findings indicate that, in many ways, these two syndrome groups are not dissimilar in terms of their communication skills. However, individuals with CdLS show a syndrome-specific deficit in understanding non-vocal communication relative to the CdCS group.

Trends in Economic Stress and the Great Recession in Ireland: An Analysis of the CSO Survey in Income and Living Conditions (SILC)

Since 2008, Ireland has experienced the most severe economic and labour market crisis since the foundation of the State. These economic and labour market changes have had a stark impact on the standard of living across the Irish population. The rapid deterioration in the labour market, the rising level of household indebtedness and stringent austerity measures to plug the public finance deficit have had a widespread impact yet there is debate about where the heaviest burden has fallen and where the economic stress has been felt most. The paper analyses data from the Survey of Income and Living Conditions for the period 2004 to 2011. The aim of the paper is to develop and test a measure of economic stress, which will capture some of the aspects of the rapid change in economic fortunes on Irish households that are not picked up by income alone. This includes tapping into features of the recession such as debt problems, unsustainable housing costs, and other difficulties associated with managing on reduced household income in a period of uncertainty. In testing such a measure we examine trends over time from boom to bust in the Irish economy and consider how economic stress is distributed across different socio-economic groups. The paper explores the distribution and level of economic stress across income class groups, social classes and the life-course and tests the thesis of ‘middle class squeeze’.

'Super-Chandrasekhar' Type Ia Supernovae at nebular epochs

We present a first systematic comparison of superluminous Type Ia supernovae (SNe Ia) at late epochs, including previously unpublished photometric and spectroscopic observations of SN 2007if, SN 2009dc and SNF20080723-012. Photometrically, the objects of our sample show a diverse late-time behaviour, some of them fading quite rapidly after a light-curve break at ∼ 150-200 d. The latter is likely the result of flux redistribution into the infrared, possibly caused by dust formation, rather than a true bolometric effect. Nebular spectra of superluminous SNe Ia are characterized by weak or absent [Fe III] emission, pointing at a low ejecta ionization state as a result of high densities. To constrain the ejecta and Ni masses of superluminous SNe Ia, we compare the observed bolometric light curve of SN 2009dc with synthetic model light curves, focusing on the radioactive tail after ∼60 d. Models with enough Ni to explain the light-curve peak by radioactive decay, and at the same time sufficient mass to keep the ejecta velocities low, fail to reproduce the observed light-curve tail of SN 2009dc because of too much γ -ray trapping.We instead propose a model with ∼1M of Ni and ∼2 M of ejecta, which may be interpreted as the explosion of a Chandrasekhar-mass white dwarf (WD) enshrouded by 0.6-0.7 M of C/O-rich material, as it could result from a merger of two massive C/O WDs. This model reproduces the late light curve of SN 2009dc well. A flux deficit at peak may be compensated by light from the interaction of the ejecta with the surrounding material.

Challenging the SD benefits attributed to sport Mega-Events hosting

Sport Mega-event hosting faces opposition that is manifested with different intensity during the different phases of the event, from its inception as an idea to its delivery and legacy. Some Social Movements Organisations (SMOs) have acted as indefatigable monitors of the Sustainable Development (SD) dimension of sporting events in general and, in some of the most recent sport mega-events, in particular the Olympics, they have served as important advisors and facilitators. Nevertheless, in many cases we see enthusiastic supporters turning to vehemently challenging whatever positives have been associated with hosting the event. In addition, there is opposition to sport Mega-events in their entirety. That type of opposition tends to employ a holistic prism that manages to identify multiple interconnected negative aspects of hosting a sport mega-event and incorporate them into an anti-systemic discourse. It is important to bear in mind that irrespective of many proclamations to the opposite as far as megas are concerned (projects and/or events), a number of studies have demonstrated that citizen participation and democratic accountability in decision-making have been notoriously absent. After all, the idea of citizen participation in the planning of sport mega-event is essentially the public response to a plan conceived by others. There were, of course, some notable cases of democratic consultation at the early stages of bidding to host a sport mega-event but these more democratic approaches resulted in the failure of the bid (for e.g. Toronto 1996). The knowledge of this by the groups that initiated the hosting idea and the bidding process has led to discouraging in depth public consultation that may fit perfectly to the democratic process but not to the tight schedules of associated projects completion. That produces ‘autocracy against which opposition may arise’ (Hiller, 2000, p. 198). It is this democratic deficit that has led to important instances of social contestation and protest mobilizations by citizen groups as well as the more regular corps of social activists. From a perspective borrowed from the sociology of protest and social movements, sport mega-events hosting can operate as an issue that stimulates protest activities by an existing protest milieu and new actors as well as an important mobilizing resource. In fact, some scholars have also argued that the Olympic Games were an important frame for the transnational activism that was marked by anti-globalization protest in Seattle in 1999 (Cottrell & Nelson, 201; Lenskyj, 2008). In addition, it’s important not to lose sight of other acts dissent that take place in relatively close proximity, about a year before the event when most infrastructural and societal changes brought by hosting the event and impact start to become apparent by the host communities, like the rioting of August 2011 in the London Olympic Boroughs and the 2012 riots of June 2013 in Sao Paulo and other Brazilian cities. This paper starts by outlining the SD claims made in the bidding to host the summer Olympic Games by five prospective hosts (Sydney; Athens; Beijing; London and Rio) proceeds towards examining the opposition and challenges that was manifested in relation to these claims. In Particular it provides an assessment of protest-events over the aforementioned different phases of sport mega-events hosting. A different picture emerges for each of the host nation that is partly explained by local, national and global configuration of protest politics. Whereas the post-event legacy of the first two hosts of the Games can be assessed and that way see the validity of claims made by challengers in the other phases, in the other three cases, the implementation of Olympic Games Impact (OGI) studies offers the tool for discussing the post-event phase for Beijing and London and engage in a speculative exercise for the case of Rio. Judging by available findings, the paper concludes that the SD aspiration made in the bid documents are unlikely to be met and social contestation based on the same issues is likely to increase due to the current global economic crisis and BRICS, like China and Brazil, having entered the process of becoming global economic hegemons.

Towards a Critical Ecology of Child Development: Aligning the Theories of Bronfenbrenner and Bourdieu

Bronfenbrenner’s model of bio-ecological development has been utilized widely within the social sciences, in the field of human development, and in social work. Yet, while championing the rights of marginalised families and communities, Bronfenbrenner had under-theorized the role of power, agency and structure in shaping the ‘person-context’ interrelationship, life opportunities and social well-being. To respond to this deficit, this paper firstly outlines Bronfenbrenner’s ‘person, process, context, time’ model. Secondly, it then seeks to loosely align aspects of Bronfenbrenner’s model with Bourdieu’s analytical categories of habitus, field and capital. It is argued that these latter categories enable social workers to develop a critical ecology of child development, taking account of power and the interplay between agency and structure. The implications of the alignment for child and family social work are considered in the final section.

General practitioners’ perceptions of the barriers and solutions to good quality palliative care in dementia

The general practitioner (GP) is in a pivotal position to initiate and adapt care for their patients living with dementia. This study aimed to elicit GPs' perceptions of the potential barriers and solutions to the provision of good-quality palliative care in dementia in their practices. A postal survey of GPs across Northern Ireland was conducted with open-ended items soliciting for barriers in their practices and possible solutions; 40.6% (138/340) were returned completed. Barriers to palliative care in dementia were perceived to be a dementia knowledge deficit for healthcare staff and the public, a resource shortfall within the GP practice and community, poor team coordination alongside inappropriate dementia care provision, and disagreements from and within families. These findings have significant implications for educators and clinicians as enhanced dementia education and training were highlighted as a strong agenda for GPs with the suggestions of dementia awareness programmes for the public.

Evaluating environmental enrichment options for commercial broiler chickens

Implications Provision of environmental enrichment in line with that required by welfare-based quality assurance schemesdoes not always appear to lead to clear improvements in broiler chicken welfare. This research perhaps serves to highlightthe deficit in information regarding the ‘real world’ implications of enrichment with perches, string and straw bales.

Introduction Earlier work showed that provision of natural light and straw bales improved leg health in commercial broilerchickens (Bailie et al., 2013). This research aimed to determine if additional welfare benefits were shown in windowedhouses by increasing straw bale provision (Study 1), or by providing perches and string in addition to straw bales (Study 2).

Material and methods Commercial windowed houses in Northern Ireland containing ~23,000 broiler chickens (placed inhouses as hatched) were used in this research which took place in 2011. In Study 1 two houses on a single farm wereassigned to one of two treatments: (1) 30 straw bales per house (1 bale/44m2), or (2) 45 straw bales per house (1bale/29m2). Bales of wheat straw, each measuring 80cm x 40cm x 40cm were provided from day 10 of the rearing cycle,as in Bailie et al. (2013). Treatments were replicated over 6 production cycles (using 276,000 Ross 308 and Cobb birds),and were swapped between houses in each replicate. In Study 2, four houses on a single farm were assigned to 1 of 4treatments in a 2 x 2 factorial design. Treatments involved 2 levels of access to perches (present (24/house), or absent), and2 levels of access to string (present (24/house), or absent), and both types of enrichment were provided from the start of thecycle. Each perch consisted of a horizontal, wooden beam (300 cm x 5 cm x 5cm) with a rounded upper edge resting on 2supports (15 cm high). In the string treatment, 6 pieces of white nylon string (60 cm x 10 mm) were tied at their mid-pointto the wire above each of 4 feeder lines. Thirty straw bales were also provided per house from day 10. This study wasreplicated over 4 production cycles using 368,000 Ross 308 birds. In both studies behaviour was observed between 0900and 1800 hours in weeks 3-5 of the cycle. In Study 1, 8 focal birds were selected in each house each week, and generalactivity, exploratory and social behaviours recorded directly for 10 minutes. In Study 2, 10 minute video recordings weremade of 6 different areas (that did not contain enrichment) of each house each week. The percentage of birds engaged inlocomotion or standing was determined through scan sampling these recordings at 120 second intervals. Four perches andfour pieces of string were filmed for 25 mins in each house that contained these enrichments on one day per week. The totalnumber of times the perch or string was used was recorded, along with the duration of each bout. In both studies, gaitscores (0 (perfect) to 5 (unable to walk)) and latency to lie (measured in seconds from when a bird had been encouraged tostand) were recorded in 25 birds in each house each week. Farm and abattoir records were also used in both studies todetermine the number of birds culled for leg and other problems, mortality levels, slaughter weights, and levels of pododermatitis and hock burn. Data were analysed using SPSS (version 20.0) and treatment and age effects on behaviouralparameters were determined in normally distributed data using ANOVA (‘Straw bale density*week’, or‘string*perches*week’ as appropriate), and in non-normally distributed data using Kuskall-Wallace tests (P<0.05 forsignificance) . Treatment (but not age) effects on performance and health data were determined using the same testsdepending on normality of data.

Results Average slaughter weight, and levels of mortality, culling, hock burn and pododermatitis were not affected bytreatment in either study (P<0.05). In Study 1 straw bale (SB) density had no significant effect on the frequency orduration of behaviours including standing, walking, ground pecking, dust bathing, pecking at bales or aggression, or onaverage gait score (P>0.05). However, the average latency to lie was greater when fewer SB were provided (30SB 23.38s,45SB 18.62s, P<0.01). In Study 2 there was an interaction between perches (Pe) and age in lying behaviour, with higherpercentages of birds observed lying in the Pe treatment during weeks 4 and 5 (week 3 +Pe 77.0 -Pe 80.9, week 4 +Pe 79.5 -Pe 75.2, week 5 +Pe 78.4 -Pe 76.2, P<0.02). There was also a significant interaction between string (S) and age inlocomotory behaviour, with higher percentages of birds observed in locomotion in the string treatment during week 3 butnot weeks 4 and 5 (week 3 +S 4.9 -S 3.9, week 4 +S 3.3 -S 3.7, week 5 +S 2.6 -S 2.8, P<0.04). There was also aninteraction between S and age in average gait scores, with lower gait scores in the string treatment in weeks 3 and 5 (week3: +S 0.7, -S 0.9, week 4: +S 1.5, -S 1.4, week 5: +S 1.9, -S 2.0, P<0.05). On average per 25 min observation there were15.1 (±13.6) bouts of perching and 19.2 (±14.08) bouts of string pecking, lasting 117.4 (±92.7) and 4.2 (±2.0) s for perchesand string, respectively.

Conclusion Increasing straw bale levels from 1 bale/44m2 to 1 bale/29m2 floor space does not appear to lead to significantimprovements in the welfare of broilers in windowed houses. The frequent use of perches and string suggests that thesestimuli have the potential to improve welfare. Provision of string also appeared to positively influence walking ability.However, this effect was numerically small, was only shown in certain weeks and was not reflected in the latency to lie.Further research on optimum design and level of provision of enrichment items for broiler chickens is warranted. Thisshould include measures of overall levels of activity (both in the vicinity of, and away from, enrichment items).

Observational Constraints on the Progenitors of Core-Collapse Supernovae: The Case for Missing High-Mass Stars

Over the last 15 years, the supernova community has endeavoured to directly identify progenitor stars for core-collapse supernovae discovered in nearby galaxies. These precursors are often visible as resolved stars in high-resolution images from space-and ground-based telescopes. The discovery rate of progenitor stars is limited by the local supernova rate and the availability and depth of archive images of galaxies, with 18 detections of precursor objects and 27 upper limits. This review compiles these results (from 1999 to 2013) in a distance-limited sample and discusses the implications of the findings. The vast majority of the detections of progenitor stars are of type II-P, II-L, or IIb with one type Ib progenitor system detected and many more upper limits for progenitors of Ibc supernovae (14 in all). The data for these 45 supernovae progenitors illustrate a remarkable deficit of high-luminosity stars above an apparent limit of log L/L-circle dot similar or equal to 5.1 dex. For a typical Salpeter initial mass function, one would expect to have found 13 high-luminosity and high-mass progenitors by now. There is, possibly, only one object in this time-and volume-limited sample that is unambiguously high-mass (the progenitor of SN2009ip) although the nature of that supernovae is still debated. The possible biases due to the influence of circumstellar dust, the luminosity analysis, and sample selection methods are reviewed. It does not appear likely that these can explain the missing high-mass progenitor stars. This review concludes that the community's work to date shows that the observed populations of supernovae in the local Universe are not, on the whole, produced by high-mass (M greater than or similar to 18 M-circle dot) stars. Theoretical explosions of model stars also predict that black hole formation and failed supernovae tend to occur above an initial mass of M similar or equal to 18 M-circle dot. The models also suggest there is no simple single mass division for neutron star or black-hole formation and that there are islands of explodability for stars in the 8-120 M-circle dot range. The observational constraints are quite consistent with the bulk of stars above M similar or equal to 18 M-circle dot collapsing to form black holes with no visible supernovae.

Looking again at troubled families: parents' perspectives on multiple adversities

The 'Troubled Families' policy and intervention agenda is based on a deficit approach that tends to ignore the role of structural disadvantage in the lives of the families it targets. In an effort to support this rhetoric, both quantitative and qualitative data have been used, and misused, to create a representation of these families, which emphasizes risk and individual blame and minimizes societal factors. This current paper presents findings from an in-depth qualitative study using a biographical narrative approach to explore parents' experiences of multiple adversities at different times over the life-course. Key themes relating to the pattern and nature of adversities experienced by participants provide a more nuanced understanding of the lives of families experiencing multiple and complex problems, highlighting how multiple interpretations are often possible within the context of professional intervention. The findings support the increasing call to move away from procedurally driven, risk averse child protection practice towards more relationally based practice, which addresses not only the needs of all family members but recognizes parents as individuals in their own right.

Vision and deaf awareness training is it app.ropriate?

Title
Visual and deaf awareness training is it app.ropriate
Purpose
Some of our most vulnerable patients have a sensory deficit. An app which focused on patients with a vision and/or hearing loss was developed for healthcare students. The intent was to embed the core values necessary for students to provide appropriate care for patients with a sensory deficit.
Setting
Queen’s University Belfast, School of Nursing and Midwifery.
Methods
Stage 1
A review of current sensory awareness training in the United Kingdom
Stage 2
Application for funding
Stage 3
Development of a teaching tool template with the essential aspects required for sensory awareness training
Stage 4
Collaboration with others: Royal National Institute for the Blind, Action on Hearing Loss, Computer technician.
Stage 5
Production and transfer of multimedia outputs onto a software application system.
Stage 6
App Piloted with a sample of lecturers (n=5), undergraduate nursing students (n=20), service users (n=5)
Stage 7
Editing
Stage 8
App made available to all undergraduate nursing students
Stage 9
App evaluation (n=300)



Results
Overall nursing students positively evaluated the app, 100% of students rated the app between good and excellent. Qualitative evidence from service users and practice partnerships was extremely positive:
"At last I feel listened too in respect to my hearing loss and empowered. I don't feel like I am complaining I am actually helping to create something which should benefit staff and all of us with a hearing or vision loss". Patient
“Very insightful into the lives of those with a disability will be so useful in practice as an aid to jog my memory". 1st year nursing student
Conclusion
It is hoped that further evaluation and implementation of the app will show an improved quality to the care delivered to those with a sensory deficit. We believe that by working in partnership with service users we have helped to create an innovative tool that benefits both staff and patients.
Financial disclosure Yes
Funding of £2700 was awarded in 2014 through the Martha McMenamin Memorial Northern Ireland Scholarship.

Impacts of invasive browsers on demographic rates and forest structure in New Zealand

Background/Question/Methods

Assessing the large scale impact of deer populations on forest structure and composition is important because of their increasing abundance in many temperate forests. Deer are invasive animals and sometimes thought to be responsible for immense damage to New Zealand’s forests. We report demographic changes taking place among 40 widespread indigenous tree species over 20 years, following a period of record deer numbers in the 1950s and a period of extensive hunting and depletion of deer populations during the 1960s and 1970s.

Results/Conclusions

Across a network of 578 plots there was an overall 13% reduction in sapling density of our study species with most remaining constant and a few declining dramatically. The effect of suppressed recruitment when deer populations were high was evident in the small tree size class (30 – 80 mm dbh). Stem density decreased by 15% and species with the greatest annual decreases in small tree density were those which have the highest rates of sapling recovery in exclosures indicating that deer were responsible. Densities of large canopy trees have remained relatively stable. There were imbalances between mortality and recruitment rates for 23 of the 40 species, 7 increasing and 16 in decline. These changes were again linked with sapling recovery in exclosures; species which recovered most rapidly following deer exclusion had the greatest net recruitment deficit across the wider landscape, indicating recruitment suppression by deer as opposed to mortality induced by disturbance and other herbivores. Species are not declining uniformly across all populations and no species are in decline across their entire range. Therefore we predict that with continued deer presence some forests will undergo compositional changes but that none of the species tested will become nationally extinct.

Impacts of invasive browsers on demographic rates and forest structure in New Zealand. Available from: http://www.researchgate.net/publication/267285500_Impacts_of_invasive_browsers_on_demographic_rates_and_forest_structure_in_New_Zealand [accessed Oct 9, 2015].

A large scale model experimental study of a tidal turbine in uniform steady flow

An experimental study measuring the performance and wake characteristics of a 1:10th scale horizontal axis turbine in steady uniform flow conditions is presented in this paper.
Large scale towing tests conducted in a lake were devised to model the performance of the tidal turbine and measure the wake produced. As a simplification of the marine environment, towing the turbine in a lake provides approximately steady, uniform inflow conditions. A 16m long x 6m wide catamaran was constructed for the test programme. This doubled as a towing rig and flow measurement platform, providing a fixed frame of reference for measurements in the wake of a horizontal axis tidal turbine. Velocity mapping was conducted using Acoustic Doppler Velocimeters.
The results indicate varying the inflow speed yielded little difference in the efficiency of the turbine or the wake velocity deficit characteristics provided the same tip speed ratio is used. Increasing the inflow velocity from 0.9 m/s to 1.2 m/s influenced the turbulent wake characteristics more markedly. The results also demonstrate that the flow field in the wake of a horizontal axis tidal turbine is strongly affected by the turbine support structure