Health-related quality of life in Huntington's disease: a comparison of two generic instruments, SF-36 and SIP

Whereas several clinical endpoints in monitoring the response to treatment in patients with Huntington's disease (HD) have been explored, there has been a paucity of research in the quality of life in such patients. The aim of this study was to validate the use of two generic health-related quality of life instruments (the Short Form 36 health survey questionnaire [SF-36] and the Sickness Impact Profile [SIP]) and to evaluate their psychometric properties. We found that both instruments demonstrated acceptable convergent validity and reliability for patients and carers. However, there was an advantage in using the SF-36 because of its more robust construct validity and test-retest reliability; furthermore, motor symptoms appeared to influence some strictly nonmotor dimensions of the SIP. On a pragmatic level, the SF-36 is shorter and quicker to administer and, therefore, easier for patients at various stages of the disease to complete. Thus, the SF-36 would appear to be the recommended instrument of choice for patients with HD and their carers, although further work needs to be done to investigate the sensitivity of this instrument longitudinally. (C) 2004 Movement Disorder Society.

Modelling of the natural chlorine cycling in a coniferous stand: implications for chlorine-36 behaviour in a contaminated forest environment

Considered as one of the most available radionuclide in soileplant system, 36Cl is of potential concern for long-term management of radioactive wastes, due to its high mobility and its long half-life. To evaluate the risk of dispersion and accumulation of 36Cl in the biosphere as a consequence of a potential contamination, there is a need for an appropriate understanding of the chlorine cycling dynamics in the ecosystems. To date, a small number of studies have investigated the chlorine transfer in the ecosystem including the transformation of chloride to organic chlorine but, to our knowledge, none have modelled this cycle. In this study, a model involving inorganic as well as organic pools in soils has been developed and parameterised to describe the biogeochemical fate of chlorine in a pine forest. The model has been evaluated for stable chlorine by performing a range of sensitivity analyses and by comparing the simulated to the observed values. Finally a range of contamination scenarios, which differ in terms of external supply, exposure time and source, has been simulated to estimate the possible accumulation of 36Cl within the different compartments of the coniferous stand. The sensitivity study supports the relevancy of the model and its compartments, and has highlighted the chlorine transfers affecting the most the residence time of chlorine in the stand. Compared to observations, the model simulates realistic values for the chlorine content within the different forest compartments. For both atmospheric and underground contamination scenarios most of the chlorine can be found in its organic form in the soil. However, in case of an underground source, about two times less chlorine accumulates in the system and proportionally more chlorine leaves the system through drainage than through volatilisation.

The impacts of climate change on river flow regimes at the global scale

This paper presents an assessment of the impacts of climate change on a series of indicators of hydrological regimes across the global domain, using a global hydrological model run with climate scenarios constructed using pattern-scaling from 21 CMIP3 (Coupled Model Intercomparison Project Phase 3) climate models. Changes are compared with natural variability, with a significant change being defined as greater than the standard deviation of the hydrological indicator in the absence of climate change. Under an SRES (Special Report on Emissions Scenarios) A1b emissions scenario, substantial proportions of the land surface (excluding Greenland and Antarctica) would experience significant changes in hydrological behaviour by 2050; under one climate model scenario (Hadley Centre HadCM3), average annual runoff increases significantly over 47% of the land surface and decreases over 36%; only 17% therefore sees no significant change. There is considerable variability between regions, depending largely on projected changes in precipitation. Uncertainty in projected river flow regimes is dominated by variation in the spatial patterns of climate change between climate models (hydrological model uncertainty is not included). There is, however, a strong degree of consistency in the overall magnitude and direction of change. More than two-thirds of climate models project a significant increase in average annual runoff across almost a quarter of the land surface, and a significant decrease over 14%, with considerably higher degrees of consistency in some regions. Most climate models project increases in runoff in Canada and high-latitude eastern Europe and Siberia, and decreases in runoff in central Europe, around the Mediterranean, the Mashriq, central America and Brasil. There is some evidence that projecte change in runoff at the regional scale is not linear with change in global average temperature change. The effects of uncertainty in the rate of future emissions is relatively small