125 research outputs found

    Dissecting the knee - Air shower measurements with KASCADE

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    Recent results of the KASCADE air shower experiment are presented in order to shed some light on the astrophysics of cosmic rays in the region of the knee in the energy spectrum. The results include investigations of high-energy interactions in the atmosphere, the analysis of the arrival directions of cosmic rays, the determination of the mean logarithmic mass, and the unfolding of energy spectra for elemental groups

    Evidence of mild founder LMOD3 mutations causing nemaline myopathy 10 in Germany and Austria

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    Objective To expand the clinical and genetic spectrum of nemaline myopathy 10 by a series of Austrian and German patients with a milder disease course and missense mutations in LMOD3. Methods We characterized the clinical features and the genetic status of 4 unrelated adolescent or adult patients with nemaline myopathy. Results The 4 patients showed a relatively mild disease course. They all have survived into adulthood, 3 of 4 have remained ambulatory, and all showed marked facial weakness. Muscle biopsy specimens gave evidence of nemaline bodies. All patients were unrelated but originated from Austria (Tyrol and Upper Austria) and Southern Germany (Bavaria). All patients carried the missense variant c.1648C>T, p.(Leu550Phe) in the LMOD3 gene, either on both alleles or in trans with another missense variant (c.1004A>G, p.Gln335Arg). Both variants were not reported previously. Conclusions In 2014, a severe form of congenital nemaline myopathy caused by disrupting mutations in LMOD3 was identified and denoted as NEM10. Unlike the previously reported patients, who had a severe clinical picture with a substantial risk of early death, our patients showed a relatively mild disease course. As the missense variant c.1648C>T is located further downstream compared to all previously published LMOD3 mutations, it might be associated with higher protein expression compared to the reported loss-of-function mutations. The apparent clusters of 2 mild mutations in Germany and Austria in 4 unrelated families may be explained by a founder effect

    Cosmic Ray Energy Spectra and Mass Composition at the Knee - Recent Results from KASCADE -

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    Recent results from the KASCADE experiment on measurements of cosmic rays in the energy range of the knee are presented. Emphasis is placed on energy spectra of individual mass groups as obtained from an two-dimensional unfolding applied to the reconstructed electron and truncated muon numbers of each individual EAS. The data show a knee-like structure in the energy spectra of light primaries (p, He, C) and an increasing dominance of heavy ones (A > 20) towards higher energies. This basic result is robust against uncertainties of the applied interaction models QGSJET and SIBYLL which are used in the shower simulations to analyse the data. Slight differences observed between experimental data and EAS simulations provide important clues for further improvements of the interaction models. The data are complemented by new limits on global anisotropies in the arrival directions of CRs and by upper limits on point sources. Astrophysical implications for discriminating models of maximum acceleration energy vs galactic diffusion/drift models of the knee are discussed based on this data.Comment: 8 pages, 7 figures, to appear in Nuclear Physics B, Proceedings Supplements, as part of the volume for the CRIS 2004, Cosmic Ray International Seminar: GZK and Surrounding

    KASCADE: Astrophysical results and tests of hadronic interaction models

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    KASCADE is a multi-detector setup to get redundant information on single air shower basis. The information is used to perform multiparameter analyses to solve the threefold problem of the reconstruction of (i)the unknown primary energy, (ii) the primary mass, and (iii) to quantify the characteristics of the hadronic interactions in the air-shower development. In this talk recent results of the KASCADE data analyses are summarized concerning cosmic ray anisotropy studies, determination of flux spectra for different primary mass groups, and approaches to test hadronic interaction models. Neither large scale anisotropies nor point sources were found in the KASCADE data set. The energy spectra of the light element groups result in a knee-like bending and a steepening above the knee. The topology of the individual knee positions shows a dependency on the primary particle. Though no hadronic interaction model is fully able to describe the multi-parameter data of KASCADE consistently, the more recent models or improved versions of older models reproduce the data better than few years ago.Comment: to appear in Nucl. Phys. B (Proc. Suppl.), Proc. of the XIII ISVHECRI, Pylos 2004 - with a better quality of the figure

    Primary Proton Spectrum of Cosmic Rays measured with Single Hadrons

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    The flux of cosmic-ray induced single hadrons near sea level has been measured with the large hadron calorimeter of the KASCADE experiment. The measurement corroborates former results obtained with detectors of smaller size if the enlarged veto of the 304 m^2 calorimeter surface is encounted for. The program CORSIKA/QGSJET is used to compute the cosmic-ray flux above the atmosphere. Between E_0=300 GeV and 1 PeV the primary proton spectrum can be described with a power law parametrized as dJ/dE_0=(0.15+-0.03)*E_0^{-2.78+-0.03} m^-2 s^-1 sr^-1 TeV^-1. In the TeV region the proton flux compares well with the results from recent measurements of direct experiments.Comment: 13 pages, accepted by Astrophysical Journa

    Large scale cosmic-ray anisotropy with KASCADE

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    The results of an analysis of the large scale anisotropy of cosmic rays in the PeV range are presented. The Rayleigh formalism is applied to the right ascension distribution of extensive air showers measured by the KASCADE experiment.The data set contains about 10^8 extensive air showers in the energy range from 0.7 to 6 PeV. No hints for anisotropy are visible in the right ascension distributions in this energy range. This accounts for all showers as well as for subsets containing showers induced by predominantly light respectively heavy primary particles. Upper flux limits for Rayleigh amplitudes are determined to be between 10^-3 at 0.7 PeV and 10^-2 at 6 PeV primary energy.Comment: accepted by The Astrophysical Journa

    Metabolic biomarker signature to differentiate pancreatic ductal adenocarcinoma from chronic pancreatitis

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    Objective Current non-invasive diagnostic tests can distinguish between pancreatic cancer (pancreatic ductal adenocarcinoma (PDAC)) and chronic pancreatitis (CP) in only about two thirds of patients. We have searched for blood-derived metabolite biomarkers for this diagnostic purpose. Design For a case-control study in three tertiary referral centres, 914 subjects were prospectively recruited with PDAC (n=271), CP (n=282), liver cirrhosis (n=100) or healthy as well as non-pancreatic disease controls (n=261) in three consecutive studies. Metabolomic profiles of plasma and serum samples were generated from 477 metabolites identified by gas chromatography-mass spectrometry and liquid chromatography-tandem mass spectrometry. Results A biomarker signature (nine metabolites and additionally CA19-9) was identified for the differential diagnosis between PDAC and CP. The biomarker signature distinguished PDAC from CP in the training set with an area under the curve (AUC) of 0.96 (95% CI 0.93-0.98). The biomarker signature cut-off of 0.384 at 85% fixed specificity showed a sensitivity of 94.9% (95% CI 87.0%-97.0%). In the test set, an AUC of 0.94 (95% CI 0.91-0.97) and, using the same cut-off, a sensitivity of 89.9% (95% CI 81.0%-95.5%) and a specificity of 91.3% (95% CI 82.8%-96.4%) were achieved, successfully validating the biomarker signature. Conclusions In patients with CP with an increased risk for pancreatic cancer (cumulative incidence 1.95%), the performance of this biomarker signature results in a negative predictive value of 99.9% (95% CI 99.7%-99.9%) (training set) and 99.8% (95% CI 99.6%-99.9%) (test set). In one third of our patients, the clinical use of this biomarker signature would have improved diagnosis and treatment stratification in comparison to CA19-9

    First results of the air shower experiment KASCADE

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    The main goals of the KASCADE (KArlsruhe Shower Core and Array DEtector) experiment are the determination of the energy spectrum and elemental composition of the charged cosmic rays in the energy range around the knee at ca. 5 PeV. Due to the large number of measured observables per single shower a variety of different approaches are applied to the data, preferably on an event-by-event basis. First results are presented and the influence of the high-energy interaction models underlying the analyses is discussed.Comment: 3 pages, 3 figures included, to appear in the TAUP 99 Proceedings, Nucl. Phys. B (Proc. Suppl.), ed. by M. Froissart, J. Dumarchez and D. Vignau

    Electron, Muon, and Hadron Lateral Distributions Measured in Air-Showers by the KASCADE Experiment

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    Measurements of electron, muon, and hadron lateral distributions of extensive air showers as recorded by the KASCADE experiment are presented. The data cover the energy range from about 5x10^14 eV up to almost 10^17 eV and extend from the inner core region to distances of 200 m. The electron and muon distributions are corrected for mutual contaminations by taking into account the detector properties in the experiment. All distributions are well described by NKG-functions. The scale radii describing the electron and hadron data best are approx. 30 m and 10 m, respectively. We discuss the correlation between scale radii and `age' parameter as well as their dependence on shower size, zenith angle, and particle energy threshold.Comment: 28 pages, 14 figures, Accepted for publication in Astroparticle Physic

    Energy Spectra of Elemental Groups of Cosmic Rays: Update on the KASCADE Unfolding Analysis

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    The KASCADE experiment measures extensive air showers induced by cosmic rays in the energy range around the so-called knee. The data of KASCADE have been used in a composition analysis showing the knee at 3-5 PeV to be caused by a steepening in the light-element spectra. Since the applied unfolding analysis depends crucially on simulations of air showers, different high energy hadronic interaction models (QGSJet and SIBYLL) were used. The results have shown a strong dependence of the relative abundance of the individual mass groups on the underlying model. In this update of the analysis we apply the unfolding method with a different low energy interaction model (FLUKA instead of GHEISHA) in the simulations. While the resulting individual mass group spectra do not change significantly, the overall description of the measured data improves by using the FLUKA model. In addition data in a larger range of zenith angle are analysed. The new results are completely consistent, i.e. there is no hint to any severe problem in applying the unfolding analysis method to KASCADE data.Comment: accepted for publication in Astroparticle Physic
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