320 research outputs found

    Quality of life in children and adolescents with Osteogenesis Imperfecta: a qualitative interview based study

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    BACKGROUND: Osteogenesis Imperfecta (OI) is a disease with varying severity affecting physical, social and emotional well-being of the child and their family. There is no existing evidence on how the OI population regard their quality of life (QoL). The main aim of this study was to determine how OI impacts on the quality of life and well-being of children and their family. It is the first stage of a larger project to develop a disease specific quality of life measure for children with OI. METHODS: Purposive sampling was used to cover the diversity of the OI population. Twenty-five qualitative interviews were undertaken with children (n = 10), parents (n = 10) and health professionals (n = 5). Interviews were digitally recorded and transcribed verbatim. Significant themes were identified, extracted and organised, undergoing framework analysis. RESULTS: Six main themes were identified; being safe and careful, reduced function, pain, fear, isolation, independence. There was a large amount of agreement between the three groups of interviewees, although discrepancies did occur between parents and children, with regard to the themes independence and fear. CONCLUSIONS: This data presents the first step in developing items for a disease specific QoL measure for children with OI. Several of the themes uncovered showed similarity to other QoL measures, but the addition of being safe and careful, particularly in relation to fractures, demonstrated the need for a disease specific measure for children with OI

    A test of trophic cascade theory: fish and benthic assemblages across a predator density gradient on coral reefs

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    Removal of predators is often hypothesized to alter community structure through trophic cascades. However, despite recent advances in our understanding of trophic cascades, evidence is often circumstantial on coral reefs because fishing pressure frequently co-varies with other anthropogenic effects, such as fishing for herbivorous fishes and changes in water quality due to pollution. Australia’s outer Great Barrier Reef (GBR) has experienced fishing-induced declines of apex predators and mesopredators, but pollution and targeting of herbivorous fishes are minimal. Here, we quantify fish and benthic assemblages across a fishing-induced predator density gradient on the outer GBR, including apex predators and mesopredators to herbivores and benthic assemblages, to test for evidence of trophic cascades and alternative hypotheses to trophic cascade theory. Using structural equation models, we found no cascading effects from apex predators to lower trophic levels: a loss of apex predators did not lead to higher levels of mesopredators, and this did not suppress mobile herbivores and drive algal proliferation. Likewise, we found no effects of mesopredators on lower trophic levels: a decline of mesopredators was not associated with higher abundances of algae-farming damselfishes and algae-dominated reefs. These findings indicate that top-down forces on coral reefs are weak, at least on the outer GBR. We conclude that predator-mediated trophic cascades are probably the exception rather than the rule in complex ecosystems such as the outer GBR

    Quantified Aeolian dune changes on Mars derived from repeat context camera images

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    Aeolian systems are active across much of the surface of Mars and quantifying the activity of bedforms is important for understanding the modern and recent Martian environment. Recently, the migration rates and sand fluxes of dunes and ripples have been precisely measured using repeat High Resolution Imaging Science Experiment (HiRISE) images. However, the limited areal extent of HiRISE coverage means that only a small area can be targeted for repeat coverage. Context Camera (CTX) images, although lower in spatial resolution, have wider spatial coverage, meaning that dune migration can potentially be monitored over larger areas. We used time series, coregistered CTX images and digital elevation models to measure dune migration rates and sand fluxes at six sites: Nili Patera, Meroe Patera, two sites at Herschel crater, McLaughlin crater, and Hellespontus Montes. We observed dune displacement in the CTX images over long-term baselines (7.5–11 Earth years; 4–6 Mars years). Bedform activity has previously been measured at all these sites using HiRISE, which we used to validate our results. Our dune migration rates (0.2–1.1 m/EY) and sand fluxes (2.4–11.6 m3 m−1 EY−1) compare well to measurements made with HiRISE. The use of CTX in monitoring dune migration has advantages (wider spatial coverage, faster processing time) and disadvantages (ripples not resolved, digital elevation model dune heights may be underestimates); the future combined use of HiRISE and CTX is likely to be beneficial

    Measurement of the Charged Multiplicities in b, c and Light Quark Events from Z0 Decays

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    Average charged multiplicities have been measured separately in bb, cc and light quark (u,d,su,d,s) events from Z0Z^0 decays measured in the SLD experiment. Impact parameters of charged tracks were used to select enriched samples of bb and light quark events, and reconstructed charmed mesons were used to select cc quark events. We measured the charged multiplicities: nˉuds=20.21±0.10(stat.)±0.22(syst.)\bar{n}_{uds} = 20.21 \pm 0.10 (\rm{stat.})\pm 0.22(\rm{syst.}), nˉc=21.28±0.46(stat.)−0.36+0.41(syst.)\bar{n}_{c} = 21.28 \pm 0.46(\rm{stat.}) ^{+0.41}_{-0.36}(\rm{syst.}) nˉb=23.14±0.10(stat.)−0.37+0.38(syst.)\bar{n}_{b} = 23.14 \pm 0.10(\rm{stat.}) ^{+0.38}_{-0.37}(\rm{syst.}), from which we derived the differences between the total average charged multiplicities of cc or bb quark events and light quark events: Δnˉc=1.07±0.47(stat.)−0.30+0.36(syst.)\Delta \bar{n}_c = 1.07 \pm 0.47(\rm{stat.})^{+0.36}_{-0.30}(\rm{syst.}) and Δnˉb=2.93±0.14(stat.)−0.29+0.30(syst.)\Delta \bar{n}_b = 2.93 \pm 0.14(\rm{stat.})^{+0.30}_{-0.29}(\rm{syst.}). We compared these measurements with those at lower center-of-mass energies and with perturbative QCD predictions. These combined results are in agreement with the QCD expectations and disfavor the hypothesis of flavor-independent fragmentation.Comment: 19 pages LaTex, 4 EPS figures, to appear in Physics Letters

    Measurement of the B0-anti-B0-Oscillation Frequency with Inclusive Dilepton Events

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    The B0B^0-Bˉ0\bar B^0 oscillation frequency has been measured with a sample of 23 million \B\bar B pairs collected with the BABAR detector at the PEP-II asymmetric B Factory at SLAC. In this sample, we select events in which both B mesons decay semileptonically and use the charge of the leptons to identify the flavor of each B meson. A simultaneous fit to the decay time difference distributions for opposite- and same-sign dilepton events gives Δmd=0.493±0.012(stat)±0.009(syst)\Delta m_d = 0.493 \pm 0.012{(stat)}\pm 0.009{(syst)} ps−1^{-1}.Comment: 7 pages, 1 figure, submitted to Physical Review Letter

    Cross-ancestry genome-wide association analysis of corneal thickness strengthens link between complex and Mendelian eye diseases

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    Central corneal thickness (CCT) is a highly heritable trait associated with complex eye diseases such as keratoconus and glaucoma. We perform a genome-wide association meta-analysis of CCT and identify 19 novel regions. In addition to adding support for known connective tissue-related pathways, pathway analyses uncover previously unreported gene sets. Remarkably, >20% of the CCT-loci are near or within Mendelian disorder genes. These included FBN1, ADAMTS2 and TGFB2 which associate with connective tissue disorders (Marfan, Ehlers-Danlos and Loeys-Dietz syndromes), and the LUM-DCN-KERA gene complex involved in myopia, corneal dystrophies and cornea plana. Using index CCT-increasing variants, we find a significant inverse correlation in effect sizes between CCT and keratoconus (r =-0.62, P = 5.30 × 10-5) but not between CCT and primary open-angle glaucoma (r =-0.17, P = 0.2). Our findings provide evidence for shared genetic influences between CCT and keratoconus, and implicate candidate genes acting in collagen and extracellular matrix regulation

    Representational predicaments for employees: Their impact on perceptions of supervisors\u27 individualized consideration and on employee job satisfaction

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    A representational predicament for a subordinate vis-à-vis his or her immediate superior involves perceptual incongruence with the superior about the subordinate\u27s work or work context, with unfavourable implications for the employee. An instrument to measure the incidence of two types of representational predicament, being neglected and negative slanting, was developed and then validated through an initial survey of 327 employees. A subsequent substantive survey with a fresh sample of 330 employees largely supported a conceptual model linking being neglected and negative slanting to perceptions of low individualized consideration by superiors and to low overall job satisfaction. The respondents in both surveys were all Hong Kong Chinese. Two case examples drawn from qualitative interviews illustrate and support the conceptual model. Based on the research findings, we recommend some practical exercises to use in training interventions with leaders and subordinates. © 2013 Copyright Taylor and Francis Group, LLC

    CSF1R inhibitor JNJ-40346527 attenuates microglial proliferation and neurodegeneration in P301S mice

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    Neuroinflammation and microglial activation are significant processes in Alzheimer's disease pathology. Recent genome-wide association studies have highlighted multiple immune-related genes in association with Alzheimer's disease, and experimental data have demonstrated microglial proliferation as a significant component of the neuropathology. In this study, we tested the efficacy of the selective CSF1R inhibitor JNJ-40346527 (JNJ-527) in the P301S mouse tauopathy model. We first demonstrated the anti-proliferative effects of JNJ-527 on microglia in the ME7 prion model, and its impact on the inflammatory profile, and provided potential CNS biomarkers for clinical investigation with the compound, including pharmacokinetic/pharmacodynamics and efficacy assessment by TSPO autoradiography and CSF proteomics. Then, we showed for the first time that blockade of microglial proliferation and modification of microglial phenotype leads to an attenuation of tau-induced neurodegeneration and results in functional improvement in P301S mice. Overall, this work strongly supports the potential for inhibition of CSF1R as a target for the treatment of Alzheimer's disease and other tau-mediated neurodegenerative diseases
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