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Background: When measuring young Duchenne Muscular Dystrophy (DMD) patients’ health-related quality of life (HRQoL), parent-proxy reports are heavily relied on. Therefore, it is imperative that the relationship between parent-proxy and child self-report HRQoL is understood. This study examined the level of agreement between children and their parent-proxy rating of the child’s HRQoL. Methods: We used FOR-DMD clinical trial baseline data. HRQoL, measured using the PedsQL inventory, was reported by 178 parent and child (ages 4 to 7 years) dyads. Intracorrelation coefficients (ICC) measured absolute agreement while paired t-tests determined differences in the average HRQoL ratings between groups. Results: The level of agreement between child and parent-proxy ratings of HRQoL was poor for the generic PedsQL scale (ICC: 0.29) and its subscales; and, similarly low for the neuromuscular disease module (ICC:0.16). On average, parents rated their child’s HRQoL as poorer than the children rated themselves in all scales except for psychosocial and school functioning. Conclusions: Child and parent-proxy HRQoL ratings are discordant in this study sample, as occurs in other chronic pediatric diseases. This should be taken into account when interpreting clinical and research HRQoL findings in this population. Future studies should examine reasons for parents’ perception of poorer HRQoL than that reported by their children.
The Glasgow area has a combination of highly variable superficial deposits and a legacy of heavy industry, quarrying and mining. These factors create complex foundation and hydrological conditions, influencing the movement of contaminants through the subsurface and giving rise locally to unstable ground conditions. Digital geological three-dimensional models developed by the British Geological Survey are helping to resolve the complex geology underlying Glasgow, providing a key tool for planning and environmental management. The models, covering an area of 3200km2 to a depth of 1.2km, include glacial and post-glacial deposits and the underlying, faulted Carboniferous igneous and sedimentary rocks. Control data, including 95,000 boreholes, digital mine plans and published geological maps, were used in model development. Digital outputs from the models include maps of depth to key horizons, such as rockhead or depth to mine workings. The models have formed the basis for the development of site-scale high-resolution geological models and provide input data for a wide range of other applications from groundwater modelling to stochastic lithological modelling.
Fontan survivors have depressed cardiac index that worsens over time. Serum biomarker measurement is minimally invasive, rapid, widely available, and may be useful for serial monitoring. The purpose of this study was to identify biomarkers that correlate with lower cardiac index in Fontan patients.
Methods and results
This study was a multi-centre case series assessing the correlations between biomarkers and cardiac magnetic resonance-derived cardiac index in Fontan patients ⩾6 years of age with biochemical and haematopoietic biomarkers obtained ±12 months from cardiac magnetic resonance. Medical history and biomarker values were obtained by chart review. Spearman’s Rank correlation assessed associations between biomarker z-scores and cardiac index. Biomarkers with significant correlations had receiver operating characteristic curves and area under the curve estimated. In total, 97 cardiac magnetic resonances in 87 patients met inclusion criteria: median age at cardiac magnetic resonance was 15 (6–33) years. Significant correlations were found between cardiac index and total alkaline phosphatase (−0.26, p=0.04), estimated creatinine clearance (0.26, p=0.02), and mean corpuscular volume (−0.32, p<0.01). Area under the curve for the three individual biomarkers was 0.63–0.69. Area under the curve for the three-biomarker panel was 0.75. Comparison of cardiac index above and below the receiver operating characteristic curve-identified cut-off points revealed significant differences for each biomarker (p<0.01) and for the composite panel [median cardiac index for higher-risk group=2.17 L/minute/m2 versus lower-risk group=2.96 L/minute/m2, (p<0.01)].
Higher total alkaline phosphatase and mean corpuscular volume as well as lower estimated creatinine clearance identify Fontan patients with lower cardiac index. Using biomarkers to monitor haemodynamics and organ-specific effects warrants prospective investigation.
During 1990 we surveyed the southern sky using a multi-beam receiver at frequencies of 4850 and 843 MHz. The half-power beamwidths were 4 and 25 arcmin respectively. The finished surveys cover the declination range between +10 and −90 degrees declination, essentially complete in right ascension, an area of 7.30 steradians. Preliminary analysis of the 4850 MHz data indicates that we will achieve a five sigma flux density limit of about 30 mJy. We estimate that we will find between 80 000 and 90 000 new sources above this limit. This is a revised version of the paper presented at the Regional Meeting by the first four authors; the surveys now have been completed.
This Summary for Policymakers presents key findings from the Special Report on Managing the Risks of Extreme Events and Disasters to Advance Climate Change Adaptation (SREX). The SREX approaches the topic by assessing the scientific literature on issues that range from the relationship between climate change and extreme weather and climate events (‘climate extremes’) to the implications of these events for society and sustainable development. The assessment concerns the interaction of climatic, environmental, and human factors that can lead to impacts and disasters, options for managing the risks posed by impacts and disasters, and the important role that non-climatic factors play in determining impacts. Box SPM.1 defines concepts central to the SREX.
The character and severity of impacts from climate extremes depend not only on the extremes themselves but also on exposure and vulnerability. In this report, adverse impacts are considered disasters when they produce widespread damage and cause severe alterations in the normal functioning of communities or societies. Climate extremes, exposure, and vulnerability are influenced by a wide range of factors, including anthropogenic climate change, natural climate variability, and socioeconomic development (Figure SPM.1). Disaster risk management and adaptation to climate change focus on reducing exposure and vulnerability and increasing resilience to the potential adverse impacts of climate extremes, even though risks cannot fully be eliminated (Figure SPM.2). Although mitigation of climate change is not the focus of this report, adaptation and mitigation can complement each other and together can significantly reduce the risks of climate change. [SYR AR4, 5.3]
Selective Chemical Vapor Deposition of Crystalline Ge-Sb-Te alloys initiating at the bottom metal contact of vias of various sizes has been accomplished. The method is based on selecting Sb and Te precursors which do not decompose on dielectric surfaces in the utilized temperature range.
We investigated the electrical characteristics of the MOSCAP structures with W/WNx/poly Si1−xGex gates stack using C-V and I-V. The low frequency C-V measurements demonstrated that the flat band voltage of the W/WNx /poly Si0.4Ge0.6 stack was lower than that of W/ WNx /poly Si0.2Ge0.8 stack by 0.3V, and showed less gate-poly-depletion-effect than that of W/ WNx /poly- Si0.2Ge0.8 gates due to the increase of dopant activation rate with the increase of Ge content in the poly Si1−xGex films. As Ge content in poly Si1−xGex increased, the leakage current level increased a little due to the increase of direct tunneling and QBD became higher due to the lower boron penetration.
A simple and novel salicidation process applying pulsed laser annealing as the first annealing step was used to induce TiSi2 formation. Both Raman spectroscopy and transmission electron microscope results confirm the formation of a new phase of Ti disilicide, the pure C40 TiSi2 after laser irradiation. Direct C54 phase growth on the basis of C40 template bypassing the C49 phase is accomplished at the second annealing temperature as low as 600°C. Line width independent formation of the C54 phase was observed on patterned wafers using this salicidation process and “fine line effect” is thus eliminated.
Classical plasticity theories generally assume that the stress at a point is a function of strain at that point only. However, when gradients in strain become significant, this localization assumption is no longer valid. These conventional models fail to display a ‘size effect’. This effect is seen experimentally when the scale of the phenomenon of interest is on the order of several microns. Under these conditions, strain gradients are of a significant magnitude as compared to the overall strain and must be considered for models to accurately capture observed phenomena.
The mechanics community has been actively involved in the development of strain gradient theories for many years. Recently, interest in this area has been rekindled and several new approaches have appeared in the literature. Two different approaches are currently being evaluated. One approach considers strain gradients as internal variables that do not introduce work conjugate higher order stresses. Another approach considers the strain gradients as internal degrees of freedom that requires work conjugate higher order stresses. Experiments are being performed to determine which approach models material behavior accurately with the least amount of complexity. A key difference between the two models considered here is the nature of the assumed boundary conditions at material interfaces. Therefore, we are investigating the deformation behavior of aluminum/sapphire interfaces loaded under simple shear. Samples are fabricated using ultra-high vacuum diffusion bonding. To determine the lattice rotations near the boundary, we are examining the samples with both electron backscatter diffraction methods (EBSD) in the scanning electron microscope and with a variety of diffraction techniques in the transmission electron microscope. The experimentally found boundary conditions shall be subsequently used to determine whether the simpler internal variable model is adequately descriptive or if the greater complexity associated with the internal degree of freedom approach is warranted.
PKS 1934–638 is an archetypal GPS source, peaking at 1.4 GHz and exhibits almost no flux density variability. VLBI images at frequencies of .843, 2.3, 4.8, & 8.4 were made with the southern hemisphere VLBI array and they reveal that the source is a 42 mas compact double. There is no detectable change in separation over the last 20 years, yielding an upper limit of ~ 0.03c ± 0.2c on any expansion velocity. The spectral shapes of the two components are remarkably similar, despite indications of finer structure on longer baselines. Magnetic field calculations indicate fields of a few mGauss and the results are consistent with equipartition.
The Southern Hemisphere VLBI Experiment (SHEVE) program is aimed at producing high-resolution images of southern radio sources. The radio telescopes of the present SHEVE array are described below and some recent results presented.
Attempts at understanding evolutionary relationships among Paleozoic Dipnoi (lungfish) using cladistic methodology have proved totally unsatisfactory (Miles 1977; Marshall 1987). We attempt to reconstruct the relationships between the better known genera using a method that involves the recognition of lineages based on evolving functional complexes, particularly those involved with food reduction and respiration. Within these broadly defined lineages, we have defined sub-lineages based on evolutionary patterns shown by structures that have been stratigraphically dated; such patterns are found inter alia in the roofing bones and the external dermal bones of the mandible. A number of new suborders and families are recognised; genera for which further morphological data are required before they can be assigned to a higher taxon are indicated; two generic synonyms are recognised.
In appendices, short descriptions are given of two new genera—Pillararhynchus from the Gogo Formation (Upper Devonian) of Western Australia, and Sorbitorhynchus from the Emsian of Guangxi, China.
A number of workers have accepted the proposition that phylogenetic relations between extant organisms can be determined only by reference to the characters of those organisms. Palaeontological data, it is said, have not been useful for developing or refuting such hypotheses. This view may be tested by reference to the respiratory mechanisms of dipnoans (lungfishes) and amphibians. The structure of the heart, lungs, and circulatory systems has been used by various authors to support the view that these are sister groups. Geological data derived from stratigraphy, palaeoecology, taphonomy, geochemistry and functional morphology of primitive dipnoans is used herein to show that these organisms did not engage in aerial respiration. The first unequivocal evidence that dipnoans had become air breathers is from aestivation burrows in Early Permian rocks of the U.S.A. The Dipnoi must have evolved this capacity at some time between the Early Devonian, when the group became well established, and the Early Permian. Similarities between the respiratory systems of extant dipnoans and amphibians must be the result of convergence, or of the derivation of the Amphibia directly from the Dipnoi. The latter view is not currently accepted by any workers in the field. Thus data from the fossil record are used to demonstrate the invalid use of some neontological data for the development of a phylogenetic hypothesis. Classifications of extant organisms depending on putative shared–derived morphological or physiological characters that cannot be adequately tested for convergence by reference to fossils, must be regarded with due caution.
Recent studies of the resonance effect between the wavelength of planar channeled ions and the periodicity of strained-layer superlattices are summarized for MeV 4He ions backscattering from GaAsxP1-X/GaP superlattices. When the incident beam energy is adjusted so the half-wavelength matches the thickness of the layers, a catastrophic dechanneling effect is observed. A simple phase rotation analysis has been developed to calculate the dechanneling in each layer and good agreement is obtained with the observed dechanneling per interface. This resonance effect is used to measure the amount of strain in the GaAsxP1-x/GaP strained-layer superlattice.
Axial ion channeling and backscattering with 2 MeV 4He are used to probe the strain in GaAs. 16P.84/GaP strained-layer superlattices (SLS) by two methods. The tetragonal distortions in the growth direction of SLS's give rise to alternating tilts in inclined crystal directions at each interface. In the first method the large increase in dechanneling along <110> directions containing the angular tilts is measured relative to the negligible increase in dechanneling along the <100> growth direction. This method provides a sensitive depth-dependent measure of strain but requires computer simulation for quantitative analysis. In the second method, channeling angular scans are used to directly measure the lattice strain by the shift in the <110> crystal direction in the top layer relative to the average <110> direction. These results demonstrate the validity of the strain accommodation model for the interpretation of the dechanneling and indicate that GaAsxP1-x/GaP SLS structures of high crystalline quality can be obtained with strains somewhat lower than that predicted theoretically from bulk lattice properties.