The Huntington's Disease health-related Quality of Life questionnaire (HDQoL): a disease-specific measure of health-related quality of life

Hocaoglu MB, Gaffan EA, Ho AK. The Huntington's disease health-related quality of life questionnaire: a disease-specific measure of health-related quality of life. Huntington's disease (HD) is a genetic neurodegenerative disorder characterized by motor, cognitive and psychiatric disturbances, and yet there is no disease-specific patient-reported health-related quality of life outcome measure for patients. Our aim was to develop and validate such an instrument, i.e. the Huntington's Disease health-related Quality of Life questionnaire (HDQoL), to capture the true impact of living with this disease. Semi-structured interviews were conducted with the full spectrum of people living with HD, to form a pool of items, which were then examined in a larger sample prior to data-driven item reduction. We provide the statistical basis for the extraction of three different sets of scales from the HDQoL, and present validation and psychometric data on these scales using a sample of 152 participants living with HD. These new patient-derived scales provide promising patient-reported outcome measures for HD.

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.

School effectiveness: new managerialism, quality and the Japanisation of education

School effectiveness is a microtechnology of change. It is a relay device, which transfers macro policy into everyday processes and priorities in schools. It is part of the growing apparatus of performance evaluation. Change is brought about by a focus on the school as a site-based system to be managed. There has been corporate restructuring in response to the changing political economy of education. There are now new work regimes and radical changes in organizational cultures. Education, like other public services, is now characterized by a range of structural realignments, new relationships between purchasers and providers and new coalitions between management and politics. In this article, we will argue that the school effectiveness movement is an example of new managerialism in education. It is part of an ideological and technological process to industrialize educational productivity. That is to say, the emphasis on standards and standardization is evocative of production regimes drawn from industry. There is a belief that education, like other public services can be managed to ensure optimal outputs and zero defects in the educational product.

Does short termism affect the quality of urban design in the UK?

This paper follows the report on the “Quality of Urban Design: Study of the Influence of Private Property Decision Maker in Urban Design” (RICS 1996). It focuses on one of the findings in the report, namely that decisions made in development, investment and occupation seemed overly influenced by short term considerations. In this paper, the authors review the Report and examine the concept of short termism as it affects urban design decisions. The paper concludes that although it is difficult to establish whether or not short termism exists in many decisions, there are grounds for believing that a priori short termism might particularly influence property orientated decisions. The paper ends with some implications for policy both at the economy and local level.

The implications of the 2003 Common Agricultural Policy reforms for land use and landscape quality in England

The assessment of the potential landscape impacts of the latest Common Agricultural Policy reforms constitutes a challenge for policy makers and it requires the development of models that can reliably project the likely spatial distribution of land uses. The aim of this study is to investigate the impact of 2003 CAP reforms to land uses and rural landscapes across England. For this purpose we modified an existing economic model of agriculture, the Land-Use Allocation Model (LUAM) to provide outputs at a scale appropriate for informing a semi-quantitative landscape assessment at the level of ‘Joint Character Areas’ (JCAs). Overall a decline in the cereal and oilseed production area is projected but intensive arable production will persist in specific locations (East of England, East Midlands and South East), having ongoing negative effects on the character of many JCAs. The impacts of de-coupling will be far more profound on the livestock sector; extensification of production will occur in traditional mixed farming regions (e.g. the South West), a partial displacement of cattle by sheep in the upland regions and an increase in the sheep numbers is expected in the lowlands (South East, Eastern and East Midlands). This extensification process will affect positively those JCAs of mixed farming conditions, but it will have negative impacts on the JCAs of historically low intensity farming (e.g. the uplands of north-west) because they will suffer from under-management and land idling. Our analysis shows that the territorialisation between intensively and extensively agricultural landscapes will continue.

Pressing technique and its effect on the quality of Dhaka cheese

Dhaka cheese is a semihard artisanal variety made mainly from bovine milk, using very simple pressing methods. Experimental cheeses were pressed at gauge pressures up to 31 kPa for 12 h at 24 °C and 70% RH. These cheeses were subsequently examined for their compositional, textural and rheological properties plus their microstructures investigated by confocal laser microscopy. The cheese pressed at 15.6 kPa was found to have the best compositional and structural properties.

The monitoring of ecological quality and the classification of standing waters in temperate regions: a review and proposal based on a worked scheme for British waters

This paper reviews the ways that quality can be assessed in standing waters, a subject that has hitherto attracted little attention but which is now a legal requirement in Europe. It describes a scheme for the assessment and monitoring of water and ecological quality in standing waters greater than about I ha in area in England & Wales although it is generally relevant to North-west Europe. Thirteen hydrological, chemical and biological variables are used to characterise the standing water body in any current sampling. These are lake volume, maximum depth, onductivity, Secchi disc transparency, pH, total alkalinity, calcium ion concentration, total N concentration,winter total oxidised inorganic nitrogen (effectively nitrate) concentration, total P concentration, potential maximum chlorophyll a concentration, a score based on the nature of the submerged and emergent plant community, and the presence or absence of a fish community. Inter alia these variables are key indicators of the state of eutrophication, acidification, salinisation and infilling of a water body.

Regulation of surface water quality in a Cretaceous chalk catchment, UK: an assessment of the relative importance of instream and wetland processes

To investigate the relative importance of instream nutrient spiralling and wetland transformation processes on surface water quality, total nitrogen (TN) and total phosphorus (TP) concentrations in a 200 m reach of the River Lambourn in the south-east of England were monitored over a 2-year period. In addition, the soil pore water nutrient dynamics in a riparian ecosystem adjacent to the river were investigated. Analysis of variance indicated that TN, TP and suspended sediment concentrations recorded upstream of the wetland were statistically significantly higher (P<0.05) than those downstream of the site. Such results suggest that the wetland was performing a nutrient retention function. Indeed, analysis of soil pore waters within the site show that up to 85% of TN and 70% of TP was removed from water flowing through the wetland during baseflow conditions, thus supporting the theory that the wetland played an important role in the regulation of surface water quality at the site. However, the small variations observed (0.034 mg TN l-1 and 0.031 mg P l-1) are consistent with the theory of nutrient spiralling suggesting that both instream and wetland retention processes have a causal effect on surface water quality.

The quality of referendum debate: the UK's electoral system referendum in the print media

This paper analyses the quality of debate surrounding the UK’s 2011 electoral reform referendum as represented in the print media. It first considers how debate quality in the context of a referendum campaign may best be conceptualized. It then uses content analysis of media coverage to investigate three aspects of that debate: its quantity; the balance between Yes and No arguments, and the quality of reason-giving. It finds that the quantity of debate was comparable to other recent electoral reform referendums. Coverage was predominantly, but not overwhelmingly, hostile to change. The different indicators of the quality of reason-giving present a mixed picture. The paper concludes by considering how the analysis could be extended through further comparison with other cases.

Estimating regional benefits of reducing targeted pollutants: An application to agricultural effects on water quality and the value of recreational fishing

The general focus of this paper is the regional estimation of marginal benefits of targeted water pollution abatement to instream uses. Benefit estimates are derived from actual consumer choices of recreational fishing activities and the implied expenditures for various levels of water quality. The methodology is applied to measuring the benefits accruing to recreational anglers in Indiana from the abatement of pollutants that are by-products of agricultural crop production.

Precision placement of fertiliser for optimising the early nutrition of vegetable crops: a review of the implications for the yield and quality of crops, and their nutrient use efficiency

The research outlined in this paper highlights the importance of the early nutrition of vegetable crops, and its long-term effects on their subsequent growth and development. Results are also presented to demonstrate how the nutrient supply during the establishment stages of young seedlings and transplants can be enhanced by targeting fertiliser to a zone close to their developing roots. Three different precision fertiliser placement techniques are compared for this purpose: starter, band or side-injected fertiliser. The use of each of these methods consistently produced the same (or greater) yields at lower application rates than those from conventional broadcast applications, increasing the apparent recovery of N, P and K, and the overall efficiency of nutrient use, while reducing the levels of residual nutrients in the soil. Starter fertilisers also advanced the maturity of some crops, and enhanced produce quality by increasing the proportions of the larger and/or more desirable marketable grades. The benefits of the different placement techniques are illustrated with selected examples from research at Warwick HRI using different vegetable crops, including lettuce, onion and carrot.

Quality control, validation and user feedback of the European Flood Alert System (EFAS)

The quality control, validation and verification of the European Flood Alert System (EFAS) are described. EFAS is designed as a flood early warning system at pan-European scale, to complement national systems and provide flood warnings more than 2 days before a flood. On average 20–30 alerts per year are sent out to the EFAS partner network which consists of 24 National hydrological authorities responsible for transnational river basins. Quality control of the system includes the evaluation of the hits, misses and false alarms, showing that EFAS has more than 50% of the time hits. Furthermore, the skills of both the meteorological as well as the hydrological forecasts are evaluated, and are included here for a 10-year period. Next, end-user needs and feedback are systematically analysed. Suggested improvements, such as real-time river discharge updating, are currently implemented.