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Duchenne muscular dystrophy is associated with progressive cardiorespiratory failure, including left ventricular dysfunction.
Methods and Results:
Males with probable or definite diagnosis of Duchenne muscular dystrophy, diagnosed between 1 January, 1982 and 31 December, 2011, were identified from the Muscular Dystrophy Surveillance Tracking and Research Network database. Two non-mutually exclusive groups were created: patients with ≥2 echocardiograms and non-invasive positive pressure ventilation-compliant patients with ≥1 recorded ejection fraction. Quantitative left ventricular dysfunction was defined as an ejection fraction <55%. Qualitative dysfunction was defined as mild, moderate, or severe. Progression of quantitative left ventricular dysfunction was modelled as a continuous time-varying outcome. Change in qualitative left ventricle function was assessed by the percentage of patients within each category at each age. Forty-one percent (n = 403) had ≥2 ejection fractions containing 998 qualitative assessments with a mean age at first echo of 10.8 ± 4.6 years, with an average first ejection fraction of 63.1 ± 12.6%. Mean age at first echo with an ejection fraction <55 was 15.2 ± 3.9 years. Thirty-five percent (140/403) were non-invasive positive pressure ventilation-compliant and had ejection fraction information. The estimated rate of decline in ejection fraction from first ejection fraction was 1.6% per year and initiation of non-invasive positive pressure ventilation did not change this rate.
In our cohort, we observed that left ventricle function in patients with Duchenne muscular dystrophy declined over time, independent of non-invasive positive pressure ventilation use. Future studies are needed to examine the impact of respiratory support on cardiac function.
Bioenergy has a significant greenhouse gas (GHG) mitigation potential, provided that the resources are developed sustainably and that efficient bioenergy systems are used. Certain current systems and key future options including perennial cropping systems, use of biomass residues and wastes and advanced conversion systems are able to deliver 80 to 90% emission reductions compared to the fossil energy baseline. However, land use conversion and forest management that lead to a loss of carbon stocks (direct) in addition to indirect land use change (d+iLUC) effects can lessen, and in some cases more than neutralize, the net positive GHG mitigation impacts. Impacts of climate change through temperature increases, rainfall pattern changes and increased frequency of extreme events will influence and interact with biomass resource potential. This interaction is still poorly understood, but it is likely to exhibit strong regional differences. Climate change impacts on biomass feedstock production exist but if global temperature rise is limited to less than 2°C compared with the pre-industrial record, it may pose few constraints. Combining adaptation measures with biomass resource production can offer more sustainable opportunities for bioenergy and perennial cropping systems.
Biomass is a primary source of food, fodder and fibre and as a renewable energy (RE) source provided about 10.2% (50.3 EJ) of global total primary energy supply (TPES) in 2008. Traditional use of wood, straws, charcoal, dung and other manures for cooking, space heating and lighting by generally poorer populations in developing countries accounts for about 30.7 EJ, and another 20 to 40% occurs in unaccounted informal sectors including charcoal production and distribution.
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