139 research outputs found

    Design and construction of the MicroBooNE detector

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    This paper describes the design and construction of the MicroBooNE liquid argon time projection chamber and associated systems. MicroBooNE is the first phase of the Short Baseline Neutrino program, located at Fermilab, and will utilize the capabilities of liquid argon detectors to examine a rich assortment of physics topics. In this document details of design specifications, assembly procedures, and acceptance tests are reported

    Risk profiles and one-year outcomes of patients with newly diagnosed atrial fibrillation in India: Insights from the GARFIELD-AF Registry.

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    BACKGROUND: The Global Anticoagulant Registry in the FIELD-Atrial Fibrillation (GARFIELD-AF) is an ongoing prospective noninterventional registry, which is providing important information on the baseline characteristics, treatment patterns, and 1-year outcomes in patients with newly diagnosed non-valvular atrial fibrillation (NVAF). This report describes data from Indian patients recruited in this registry. METHODS AND RESULTS: A total of 52,014 patients with newly diagnosed AF were enrolled globally; of these, 1388 patients were recruited from 26 sites within India (2012-2016). In India, the mean age was 65.8 years at diagnosis of NVAF. Hypertension was the most prevalent risk factor for AF, present in 68.5% of patients from India and in 76.3% of patients globally (P < 0.001). Diabetes and coronary artery disease (CAD) were prevalent in 36.2% and 28.1% of patients as compared with global prevalence of 22.2% and 21.6%, respectively (P < 0.001 for both). Antiplatelet therapy was the most common antithrombotic treatment in India. With increasing stroke risk, however, patients were more likely to receive oral anticoagulant therapy [mainly vitamin K antagonist (VKA)], but average international normalized ratio (INR) was lower among Indian patients [median INR value 1.6 (interquartile range {IQR}: 1.3-2.3) versus 2.3 (IQR 1.8-2.8) (P < 0.001)]. Compared with other countries, patients from India had markedly higher rates of all-cause mortality [7.68 per 100 person-years (95% confidence interval 6.32-9.35) vs 4.34 (4.16-4.53), P < 0.0001], while rates of stroke/systemic embolism and major bleeding were lower after 1 year of follow-up. CONCLUSION: Compared to previously published registries from India, the GARFIELD-AF registry describes clinical profiles and outcomes in Indian patients with AF of a different etiology. The registry data show that compared to the rest of the world, Indian AF patients are younger in age and have more diabetes and CAD. Patients with a higher stroke risk are more likely to receive anticoagulation therapy with VKA but are underdosed compared with the global average in the GARFIELD-AF. CLINICAL TRIAL REGISTRATION-URL: http://www.clinicaltrials.gov. Unique identifier: NCT01090362

    Bi-allelic Loss-of-Function CACNA1B Mutations in Progressive Epilepsy-Dyskinesia.

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    The occurrence of non-epileptic hyperkinetic movements in the context of developmental epileptic encephalopathies is an increasingly recognized phenomenon. Identification of causative mutations provides an important insight into common pathogenic mechanisms that cause both seizures and abnormal motor control. We report bi-allelic loss-of-function CACNA1B variants in six children from three unrelated families whose affected members present with a complex and progressive neurological syndrome. All affected individuals presented with epileptic encephalopathy, severe neurodevelopmental delay (often with regression), and a hyperkinetic movement disorder. Additional neurological features included postnatal microcephaly and hypotonia. Five children died in childhood or adolescence (mean age of death: 9 years), mainly as a result of secondary respiratory complications. CACNA1B encodes the pore-forming subunit of the pre-synaptic neuronal voltage-gated calcium channel Cav2.2/N-type, crucial for SNARE-mediated neurotransmission, particularly in the early postnatal period. Bi-allelic loss-of-function variants in CACNA1B are predicted to cause disruption of Ca2+ influx, leading to impaired synaptic neurotransmission. The resultant effect on neuronal function is likely to be important in the development of involuntary movements and epilepsy. Overall, our findings provide further evidence for the key role of Cav2.2 in normal human neurodevelopment.MAK is funded by an NIHR Research Professorship and receives funding from the Wellcome Trust, Great Ormond Street Children's Hospital Charity, and Rosetrees Trust. E.M. received funding from the Rosetrees Trust (CD-A53) and Great Ormond Street Hospital Children's Charity. K.G. received funding from Temple Street Foundation. A.M. is funded by Great Ormond Street Hospital, the National Institute for Health Research (NIHR), and Biomedical Research Centre. F.L.R. and D.G. are funded by Cambridge Biomedical Research Centre. K.C. and A.S.J. are funded by NIHR Bioresource for Rare Diseases. The DDD Study presents independent research commissioned by the Health Innovation Challenge Fund (grant number HICF-1009-003), a parallel funding partnership between the Wellcome Trust and the Department of Health, and the Wellcome Trust Sanger Institute (grant number WT098051). We acknowledge support from the UK Department of Health via the NIHR comprehensive Biomedical Research Centre award to Guy's and St. Thomas' National Health Service (NHS) Foundation Trust in partnership with King's College London. This research was also supported by the NIHR Great Ormond Street Hospital Biomedical Research Centre. J.H.C. is in receipt of an NIHR Senior Investigator Award. The research team acknowledges the support of the NIHR through the Comprehensive Clinical Research Network. The views expressed are those of the author(s) and not necessarily those of the NHS, the NIHR, Department of Health, or Wellcome Trust. E.R.M. acknowledges support from NIHR Cambridge Biomedical Research Centre, an NIHR Senior Investigator Award, and the University of Cambridge has received salary support in respect of E.R.M. from the NHS in the East of England through the Clinical Academic Reserve. I.E.S. is supported by the National Health and Medical Research Council of Australia (Program Grant and Practitioner Fellowship)

    Komatiites as Mercury Surface Analogues: Spectral Measurements at PEL

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    X-ray fluorescence and gamma ray measurements by the NASA MESSENGER spacecraft orbiting Mercury show a surface composition strongly different from other terrestrial planets [1, 2]. The X-Ray Spectrometer (XRS) measures elemental composi-tion for the topmost 0.1 mm of Mercury’s regolith, while the Gamma Ray and Neutron Spectrometer (GRNS) is sensitive to depths of tens of centimeters. High Mg/Si and low Al/Si and Ca/Si ratios exclude a feldspathic surface composition like that of the lunar highlands. Mercury's surface mineralogy is likely dom-inated by magnesian orthopyroxene, sodium-rich pla-gioclase feldspar, and lesser amounts of Ca, Mg, and/or Fe sulfides. Enstatite chondrite melts and cer-tain enstatite achondrites provide a good compositional and mineralogical match for much of the surface of Mercury [3]. Elemental results from the GRNS [4] are consistent with those previously obtained by the X-Ray Spectrometer, including the high sulfur and low iron abundances. The elemental results from gamma-ray and X-ray spectrometry are most consistent with petro-logic models suggesting that Mercury's surface is dom-inated by magnesian silicates [5]. Mercury’s magne-sian silicates and high Mg/Si ratio (in excess of the Mg/Si ratio of terrestrial and lunar basalts [1]) invites comparison to the terrestrial ultramafic lavas known as komatiites. To assess komatiites as analogs for the surface of Mercury, at the Planetary Emissivity Laboratory (PEL) at the German Aerospace Center (DLR) we have measured visible and infrared spectra for a suite of komatiite samples under a range of environmental conditions

    Komatiites as Mercury surface analogues: Spectral measurements at PEL

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    The elemental composition of Mercury's surface, which has been recently measured by the NASA MErcury Surface, Space ENvironment, GEochemistry, and Ranging (MESSENGER) spacecraft, suggests a mineralogy dominated by magnesium-rich orthopyroxene and feldspar. The most magnesium-rich and aluminium-poor regions of Mercury's surface (which are presumably orthopyroxene-rich) have compositions, and possibly mineralogies, analogous to terrestrial boninites and basaltic komatiites. Unfortunately, little is known about the spectral properties of komatiites, especially at the high surface temperatures of Mercury. We therefore have collected three terrestrial komatiites with different compositions plus a synthetic komatiitic sample, and measured their reflectances in the visible and thermal infrared spectral ranges. Samples divided into four grain size ranges (when enough material was available) were measured fresh and after thermal processing in vacuum (10 Pa) at 500 °C, comparable to Mercury peak surface temperatures. Our measurements show that spectral changes between fresh and thermally processed samples occur in both spectral channels, but are stronger in the visible range, with reddening affecting all the samples, while darkening is more selective. It is important to note that darkening and reddening after thermally processing the samples are independent of the komatiites ferrous iron content. In fact the synthetic sample which is nearly iron-free is most strongly affected. From our study it turns out that thermally processing the samples in vacuum at Mercury surface temperature produces the removal of samples' colour centres. The results of our study show also that the Mercury Atmospheric and Surface Composition Spectrometer (MASCS) instrument on MESSENGER orbiting Mercury currently cannot distinguish between different compositions of komatiites, while the future MErcury Radiometer and Thermal infrared Imaging Spectrometer (MERTIS) on the upcoming ESA BepiColombo mission will resolve their differences in the 7-14 Όm spectral range
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