Health-related quality of life in Huntington’s Disease patients: a comparison of proxy assessment and patient self-rating using the disease-specific Huntington’s Disease health-related quality of life questionnaire (HDQoL)

Huntington’s disease (HD) is a fatal, neurodegenerative disease for which there is no known cure. Proxy evaluation is relevant for HD as its manifestation might limit the ability of persons to report their health-related quality of life (HrQoL). This study explored patient–proxy ratings of HrQoL of persons at different stages of HD, and examined factors that may affect proxy ratings. A total of 105 patient–proxy pairs completed the Huntington’s disease health-related quality of life questionnaire (HDQoL) and other established HrQoL measures (EQ-5D and SF-12v2). Proxy–patient agreement was assessed in terms of absolute level (mean ratings) and intraclass correlation. Proxies’ ratings were at a similar level to patients’ self-ratings on an overall Summary Score and on most of the six Specific Scales of the HDQoL. On the Specific Hopes and Worries Scale, proxies on average rated HrQoL as better than patients’ self-ratings, while on both the Specific Cognitive Scale and Specific Physical and Functional Scale proxies tended to rate HrQoL more poorly than patients themselves. The patient’s disease stage and mental wellbeing (SF-12 Mental Component scale) were the two factors that primarily affected proxy assessment. Proxy scores were strongly correlated with patients’ self-ratings of HrQoL, on the Summary Scale and all Specific Scales. The patient–proxy correlation was lower for patients at moderate stages of HD compared to patients at early and advanced stages. The proxy report version of the HDQoL is a useful complementary tool to self-assessment, and a promising alternative when individual patients with advanced HD are unable to self-report.

A STUDY OF THE RELATIONSHIP BETWEEN SELF-ASSESSMENT AND CLINICAL EVALUATION OF HEARING STATUS

The relationship was explored between a subjective measure of hearing status, derived from a functional self-assessment expressed in terms of ability to hear and understand spoken words, and a comparable objective measure of hearing status, obtained from a speech reception test. The Augmentation Survey of the Health and Nutrition Examination Survey of the National Center for Health Statistics provided the necessary data for a sample of 3059 adults. Using chi-square tests for the subsample with the highest level of objectively assessed hearing status, favorable subjective assessments were found to be significantly associated with higher income, lower age group, higher level of educational attainment, greater psychological adjustment, fewer symptoms of depression, and higher self-ratings of overall health. In a linear regression with self-assessment of hearing status as the dependent variable, less than one-quarter of the variation could be explained by objective status and the six explanatory variables.^

Sex offender risk assessment and management in Ireland

This research examined sex offender risk assessment and management in Ireland. It focused on the statutory agencies with primary responsibility (Garda Síochána and the Probation Service). The goal was to document the historical, contextual and current systems, in addition to identifying areas of concern/improvements. The research was a mixed-methods approach. Eight studies were conducted. This incorporated documentary reviews of four Commission to Inquire Reports, qualitative interviews/focus groups with Garda staff, Probation Service staff, statutory agencies, community stakeholders, various Non-Governmental Organisations (NGOs) and sex offenders. Quantitative questionnaires were also administered to Garda staff. In all over 70 interviews were conducted and questionnaires were forwarded to 270 Garda members. The overall findings are: •Sex offender management in Ireland has become formal only since 2001. Knowledge, skills and expertise is in its infancy and is still evolving. •Mixed reviews and questions regarding fitness for purpose of currently used risk assessments tools were noted. •The Sex Offender Act 2001 requires additional elements to ensure safe sex offender monitoring and public protection. A judicial review of the Sex Offender Act 2001 was recommended by many respondents. •Interagency working under SORAM was hugely welcomed. The sharing of information has been welcomed by managing agencies as the key benefit to improving sex offender management. •Respondents reported that in practice, sex offender management in Ireland is fragmented and unevenly implemented. The research concluded that an independent National Sex Offender Authority should be established as an oversight and regulatory body for policy, strategy and direction in sex offender management. Further areas of research were also highlighted: ongoing evaluation and audits of the joint agency process and systems in place; recidivism studies tracking the risk assessment ratings and subsequent offending; and an evaluation of the current status of sex offender housing in Ireland.

Formative and summative assessment and the notion of constructive alignment

This paper discusses the different perceptions of first year accounting students about their tutorial activities and their engagements in assessment. As the literature suggests, unless participation in learning activities forms part of graded assessment, it is often difficult to engage students in these activities. Using an action research model, this paper reports the study of first year accounting students' responses to action-oriented learning tasks in tutorials. The paper focuses on the importance of aligning curriculum objectives, learning and teaching activities and assessment, i.e. the notion of constructive alignment. However, as the research findings indicate, without support at institutional level, applying constructive alignment to facilitate quality student learning outcomes is a difficult task. Thus, the impacts of policy constraints on curriculum issues are also discussed, focusing on the limitations faced by tutors and their lack of involvement in curriculum development.

Formative and Summative Assessment and the Notion of Constructive Allignment

This paper discusses perceptions of first year accounting students about their tutorial activities and their engagements in assessment. As the literature suggests, unless participation in learning activities forms part of graded assessment it is often difficult to engage students in these activities. Using an action research model, this paper reports the study of first year accounting students' responses to action-orientated learning tasks in tutorials. The paper focuses on the importance of aligning curriculum objectives, learning and teaching activities and assessment,i.e. the notion of constructive alignment. However, as the research findings indicate, without support at institutional level, applying constructive alignment to facilitate quality student learning outcomes is a difficult task. Thus, the impacts of policy constraints on curriculum issues are also discussed, focusing on the limitations faced by tutors and their lack of involvement in curriculum development.

Assessment and curriculum reform in China : the college English test and tertiary English as a foreign language education.

Educational assessment was a worldwide commonplace practice in the last century. With the theoretical underpinnings of education shifting from behaviourism and social efficiency to constructivism and cognitive theories in the past two decades, the assessment theories and practices show a widespread changing movement. The emergent assessment paradigm, with a futurist perspective, indicates a deviation away from the prevailing large scale high-stakes standardised testing and an inclination towards classroom-based formative assessment. Innovations and reforms initiated in attempts to achieve better education outcomes for a sustainable future via more developed learning and assessment theories have included the 2007 College English Reform Program (CERP) in Chinese higher education context. This paper focuses on the College English Test (CET) - the national English as a Foreign Language (EFL) testing system for non-English majors at tertiary level in China. It seeks to explore the roles that the CET played in the past two College English curriculum reforms, and the new role that testing and assessment assumed in the newly launched reform. The paper holds that the CET was operationalised to uplift the standards. However, the extended use of this standardised testing system brings constraints as well as negative washback effects on the tertiary EFL education. Therefore in the newly launched reform -CERP, a new assessment model which combines summative and formative assessment approaches is proposed. The testing and assessment, assumed a new role - to engender desirable education outcomes. The question asked is: will the mixed approach to formative and summative assessment provide the intended cure to the agony that tertiary EFL education in China has long been suffering - spending much time, yet achieving little effects? The paper reports the progresses and challenges as informed by the available research literature, yet asserts a lot needs to be explored on the potential of the assessment mix in this examination tradition deep-rooted and examination-obsessed society.

Harnessing assessment and feedback in the first year to support learning success, engagement and retention

It has been argued that intentional first year curriculum design has a critical role to play in enhancing first year student engagement, success and retention (Kift, 2008). A fundamental first year curriculum objective should be to assist students to make the successful transition to assessment in higher education. Scott (2006) has identified that ‘relevant, consistent and integrated assessment … [with] prompt and constructive feedback’ are particularly relevant to student retention generally; while Nicol (2007) suggests that ‘lack of clarity regarding expectations in the first year, low levels of teacher feedback and poor motivation’ are key issues in the first year. At the very minimum, if we expect first year students to become independent and self-managing learners, they need to be supported in their early development and acquisition of tertiary assessment literacies (Orrell, 2005). Critical to this attainment is the necessity to alleviate early anxieties around assessment information, instructions, guidance, and performance. This includes, for example:  inducting students thoroughly into the academic languages and assessment genres they will encounter as the vehicles for evidencing learning success; and  making expectations about the quality of this evidence clear. Most importantly, students should receive regular formative feedback of their work early in their program of study to aid their learning and to provide information to both students and teachers on progress and achievement. Leveraging research conducted under an ALTC Senior Fellowship that has sought to articulate a research-based 'transition pedagogy' (Kift & Nelson, 2005) – a guiding philosophy for intentional first year curriculum design and support that carefully scaffolds and mediates the first year learning experience for contemporary heterogeneous cohorts – this paper will discuss theoretical and practical strategies and examples that should be of assistance in implementing good assessment and feedback practices across a range of disciplines in the first year.

Simulated cataract and low contrast stimuli impair cognitive performance in older adults : implications for neuropsychological assessment and everyday function

Objective: To investigate how age-related declines in vision (particularly contrast sensitivity), simulated using cataract-goggles and low-contrast stimuli, influence the accuracy and speed of cognitive test performance in older adults. An additional aim was to investigate whether declines in vision differentially affect secondary more than primary memory. Method: Using a fully within-subjects design, 50 older drivers aged 66-87 years completed two tests of cognitive performance - letter matching (perceptual speed) and symbol recall (short-term memory) - under different viewing conditions that degraded visual input (low-contrast stimuli, cataract-goggles, and low-contrast stimuli combined with cataract-goggles, compared with normal viewing). However, presentation time was also manipulated for letter matching. Visual function, as measured using standard charts, was taken into account in statistical analyses. Results: Accuracy and speed for cognitive tasks were significantly impaired when visual input was degraded. Furthermore, cognitive performance was positively associated with contrast sensitivity. Presentation time did not influence cognitive performance, and visual gradation did not differentially influence primary and secondary memory. Conclusion: Age-related declines in visual function can impact on the accuracy and speed of cognitive performance, and therefore the cognitive abilities of older adults may be underestimated in neuropsychological testing. It is thus critical that visual function be assessed prior to testing, and that stimuli be adapted to older adults' sensory capabilities (e.g., by maximising stimuli contrast).

Observational assessment and maternal reports of motivation in children and adolescents with Down Syndrome

Despite a lack of consistent empirical evidence, there has been an ongoing assumption that intellectual disability is associated with reduced levels of motivation. The participants in this study were 33 children with Down syndrome ages 10–15 years and 33 typically developing 3–8-year-old children. Motivation was measured through observational assessments of curiosity, preference for challenge, and persistence, as well as maternal reports. There were no significant group differences on motivation tasks, but mothers of children with Down syndrome rated their children significantly lower on motivation than did parents of typically developing children. There were some intriguing group differences in the pattern of correlations among observations and parent reports. The findings challenge long-held views that individuals with intellectual disability are invariably deficient in motivation.

Cellulosic ethanol from sugarcane bagasse in Australia : exploring industry feasibility through systems analysis, techno-economic assessment and pilot plant development

Overcoming many of the constraints to early stage investment in biofuels production from sugarcane bagasse in Australia requires an understanding of the complex technical, economic and systemic challenges associated with the transition of established sugar industry structures from single product agri-businesses to new diversified multi-product biorefineries. While positive investment decisions in new infrastructure requires technically feasible solutions and the attainment of project economic investment thresholds, many other systemic factors will influence the investment decision. These factors include the interrelationships between feedstock availability and energy use, competing product alternatives, technology acceptance and perceptions of project uncertainty and risk. This thesis explores the feasibility of a new cellulosic ethanol industry in Australia based on the large sugarcane fibre (bagasse) resource available. The research explores industry feasibility from multiple angles including the challenges of integrating ethanol production into an established sugarcane processing system, scoping the economic drivers and key variables relating to bioethanol projects and considering the impact of emerging technologies in improving industry feasibility. The opportunities available from pilot scale technology demonstration are also addressed. Systems analysis techniques are used to explore the interrelationships between the existing sugarcane industry and the developing cellulosic biofuels industry. This analysis has resulted in the development of a conceptual framework for a bagassebased cellulosic ethanol industry in Australia and uses this framework to assess the uncertainty in key project factors and investment risk. The analysis showed that the fundamental issue affecting investment in a cellulosic ethanol industry from sugarcane in Australia is the uncertainty in the future price of ethanol and government support that reduces the risks associated with early stage investment is likely to be necessary to promote commercialisation of this novel technology. Comprehensive techno-economic models have been developed and used to assess the potential quantum of ethanol production from sugarcane in Australia, to assess the feasibility of a soda-based biorefinery at the Racecourse Sugar Mill in Mackay, Queensland and to assess the feasibility of reducing the cost of production of fermentable sugars from the in-planta expression of cellulases in sugarcane in Australia. These assessments show that ethanol from sugarcane in Australia has the potential to make a significant contribution to reducing Australia’s transportation fuel requirements from fossil fuels and that economically viable projects exist depending upon assumptions relating to product price, ethanol taxation arrangements and greenhouse gas emission reduction incentives. The conceptual design and development of a novel pilot scale cellulosic ethanol research and development facility is also reported in this thesis. The establishment of this facility enables the technical and economic feasibility of new technologies to be assessed in a multi-partner, collaborative environment. As a key outcome of this work, this study has delivered a facility that will enable novel cellulosic ethanol technologies to be assessed in a low investment risk environment, reducing the potential risks associated with early stage investment in commercial projects and hence promoting more rapid technology uptake. While the study has focussed on an exploration of the feasibility of a commercial cellulosic ethanol industry from sugarcane in Australia, many of the same key issues will be of relevance to other sugarcane industries throughout the world seeking diversification of revenue through the implementation of novel cellulosic ethanol technologies.