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.

Stratospheric climate and variability from a general circulation model and observations

The climate and natural variability of the large-scale stratospheric circulation simulated by a newly developed general circulation model are evaluated against available global observations. The simulation consisted of a 30-year annual cycle integration performed with a comprehensive model of the troposphere and stratosphere. The observations consisted of a 15-year dataset from global operational analyses of the troposphere and stratosphere. The model evaluation concentrates on the simulation of the evolution of the extratropical stratospheric circulation in both hemispheres. The December–February climatology of the observed zonal mean winter circulation is found to be reasonably well captured by the model, although in the Northern Hemisphere upper stratosphere the simulated westerly winds are systematically stronger and a cold bias is apparent in the polar stratosphere. This Northern Hemisphere stratospheric cold bias virtually disappears during spring (March–May), consistent with a realistic simulation of the spring weakening of the mean westerly winds in the model. A considerable amount of monthly interannual variability is also found in the simulation in the Northern Hemisphere in late winter and early spring. The simulated interannual variability is predominantly caused by polar warmings of the stratosphere, in agreement with observations. The breakdown of the Northern Hemisphere stratospheric polar vortex appears therefore to occur in a realistic way in the model. However, in early winter the model severely underestimates the interannual variability, especially in the upper troposphere. The Southern Hemisphere winter (June–August) zonal mean temperature is systematically colder in the model, and the simulated winds are somewhat too strong in the upper stratosphere. Contrary to the results for the Northern Hemisphere spring, this model cold bias worsens during the Southern Hemisphere spring (September–November). Significant discrepancies between the model results and the observations are therefore found during the breakdown of the Southern Hemisphere polar vortex. For instance, the simulated Southern Hemisphere stratosphere westerly jet continuously decreases in intensity more or less in situ from June to November, while the observed stratospheric jet moves downward and poleward.

Climatology and variability in the ECHO coupled GCM

ECHO is a new global coupled ocean-atmosphere general circulation model (GCM), consisting of the Hamburg version of the European Centre atmospheric GCM (ECHAM) and the Hamburg Primitive Equation ocean GCM (HOPE). We performed a 20-year integration with ECHO. Climate drift is significant, but typical annual mean errors in sea surface temperature (SST) do not exceed 2° in the open oceans. Near the boundaries, however, SST errors are considerably larger. The coupled model simulates an irregular ENSO cycle in the tropical Pacific, with spatial patterns similar to those observed. The variability, however, is somewhat weaker relative to observations. ECHO also simulates significant interannual variability in mid-latitudes. Consistent with observations, variability over the North Pacific can be partly attributed to remote forcing from the tropics. In contrast, the interannual variability over the North Atlantic appears to be generated locally.

Seasonal cycles and variability of O3 and H2O in the UT/LMS during SPURT

Airborne high resolution in situ measurements of a large set of trace gases including ozone (O3) and total water (H2O) in the upper troposphere and the lowermost stratosphere (UT/LMS) have been performed above Europe within the SPURT project. SPURT provides an extensive data coverage of the UT/LMS in each season within the time period between November 2001 and July 2003. In the LMS a distinct spring maximum and autumn minimum is observed in O3, whereas its annual cycle in the UT is shifted by 2–3 months later towards the end of the year. The more variable H2O measurements reveal a maximum during summer and a minimum during autumn/winter with no phase shift between the two atmospheric compartments. For a comprehensive insight into trace gas composition and variability in the UT/LMS several statistical methods are applied using chemical, thermal and dynamical vertical coordinates. In particular, 2-dimensional probability distribution functions serve as a tool to transform localised aircraft data to a more comprehensive view of the probed atmospheric region. It appears that both trace gases, O3 and H2O, reveal the most compact arrangement and are best correlated in the view of potential vorticity (PV) and distance to the local tropopause, indicating an advanced mixing state on these surfaces. Thus, strong gradients of PV seem to act as a transport barrier both in the vertical and the horizontal direction. The alignment of trace gas isopleths reflects the existence of a year-round extra-tropical tropopause transition layer. The SPURT measurements reveal that this layer is mainly affected by stratospheric air during winter/spring and by tropospheric air during autumn/summer. Normalised mixing entropy values for O3 and H2O in the LMS appear to be maximal during spring and summer, respectively, indicating highest variability of these trace gases during the respective seasons.

Improving assessment and modelling of climate change impacts on global terrestrial biodiversity.

Understanding how species and ecosystems respond to climate change has become a major focus of ecology and conservation biology. Modelling approaches provide important tools for making future projections, but current models of the climate-biosphere interface remain overly simplistic, undermining the credibility of projections. We identify five ways in which substantial advances could be made in the next few years: (i) improving the accessibility and efficiency of biodiversity monitoring data, (ii) quantifying the main determinants of the sensitivity of species to climate change, (iii) incorporating community dynamics into projections of biodiversity responses, (iv) accounting for the influence of evolutionary processes on the response of species to climate change, and (v) improving the biophysical rule sets that define functional groupings of species in global models.

Comparing the fit between BREEAM assessment and design processes

This paper explores the mapping of the environmental assessment process onto design and construction processes. A comparative case study method is used to identify and account for variations in the ‘fit’ between these two processes. The analysis compares eight BREEAM projects (although relevant to LEED, GreenStar, etc.) and distinguishes project-level characteristics and dynamics. Drawing on insights from literature on sustainable construction and assessment methods, an analytic framework is developed to examine the effect of clusters of project and assessment level elements on different types of fit (tight, punctual and bolt-on). Key elements distinguishing between types include: prior working experience with project team members, individual commitment to sustainable construction, experience with sustainable construction, project continuity, project-level ownership of the assessment process, and the nature and continuity of assessor involvement. Professionals with ‘sustainable’ experience used BREEAM judiciously to support their designs (along with other frameworks), but less committed professionals tended to treat it purely as an assessment method. More attention needs to be paid to individual levels of engagement with, and understanding of, sustainability in general (rather than knowledge of technical solutions to individual credits), to ownership of the assessment process and to the potential effect of discontinuities at the project level on sustainable design.

Assessment and management of anxiety disorders in children and adolescents

Anxiety disorders in childhood and adolescence are extremely common and are often associated with lifelong psychiatric disturbance. Consistent with DSM-5 and the extant literature, this review concerns the assessment and treatment of specific phobias, separation anxiety disorder, generalised anxiety disorder, social anxiety disorder, panic disorder and agoraphobia. Evidence-based psychological treatments (cognitive behaviour therapy; CBT) for these disorders have been developed and investigated, and in recent years promising low-intensity versions of CBT interventions have been proposed that offer a means to increase access to evidence-based treatments. There is some evidence of effectiveness of pharmacological treatments for anxiety disorders in children and young people, however, routine prescription is not recommended due to concerns about potential harm.

On the relationship between decadal buoyancy anomalies and variability of the Atlantic meridional overturning circulation

Owing to the role of the Atlantic meridional overturning circulation (AMOC) in ocean heat transport, AMOC variability is thought to play a role in climate variability on a wide range of time scales. This paper focuses on the potential role of the AMOC in climate variability on decadal time scales. Coupled and ocean-only general circulation models run in idealized geometries are utilized to study the relationships between decadal AMOC and buoyancy variability and determine whether the AMOC plays an active role in setting sea surface temperature on decadal time scales.DecadalAMOC variability is related to changes in the buoyancy field along the western boundary according to the thermal wind relation. Buoyancy anomalies originate in the upper ocean of the subpolar gyre and travel westward as baroclinic Rossby waves. When the buoyancy anomalies strike the western boundary, they are advected southward by the deep western boundary current, leading to latitudinally coherent AMOC variability. The AMOC is observed to respond passively to decadal buoyancy anomalies: although variability of the AMOC leads to meridional ocean heat transport anomalies, these transports are not responsible for creating the buoyancy anomalies in the subpolar gyre that drive AMOC variability.

Mean climate and variability of the atmosphere and ocean on an aquaplanet

Numerical experiments are described that pertain to the climate of a coupled atmosphere–ocean–ice system in the absence of land, driven by modern-day orbital and CO2 forcing. Millennial time-scale simulations yield a mean state in which ice caps reach down to 55° of latitude and both the atmosphere and ocean comprise eastward- and westward-flowing zonal jets, whose structure is set by their respective baroclinic instabilities. Despite the zonality of the ocean, it is remarkably efficient at transporting heat meridionally through the agency of Ekman transport and eddy-driven subduction. Indeed the partition of heat transport between the atmosphere and ocean is much the same as the present climate, with the ocean dominating in the Tropics and the atmosphere in the mid–high latitudes. Variability of the system is dominated by the coupling of annular modes in the atmosphere and ocean. Stochastic variability inherent to the atmospheric jets drives variability in the ocean. Zonal flows in the ocean exhibit decadal variability, which, remarkably, feeds back to the atmosphere, coloring the spectrum of annular variability. A simple stochastic model can capture the essence of the process. Finally, it is briefly reviewed how the aquaplanet can provide information about the processes that set the partition of heat transport and the climate of Earth.