Sporadic late-onset nemaline myopathy with MGUS: long-term follow-up after melphalan and SCT.

OBJECTIVE Sporadic late-onset nemaline myopathy (SLONM) is a rare, late-onset myopathy that progresses subacutely. If associated with a monoclonal gammopathy of unknown significance (MGUS), the outcome is unfavorable: the majority of these patients die within 1 to 5 years of respiratory failure. This study aims to qualitatively assess the long-term treatment effect of high-dose melphalan (HDM) followed by autologous stem cell transplantation (SCT) in a series of 8 patients with SLONM-MGUS. METHODS We performed a retrospective case series study (n = 8) on the long-term (1-8 years) treatment effect of HDM followed by autologous SCT (HDM-SCT) on survival, muscle strength, and functional capacities. RESULTS Seven patients showed a lasting moderate-good clinical response, 2 of them after the second HDM-SCT. All of them had a complete, a very good partial, or a partial hematologic response. One patient showed no clinical or hematologic response and died. CONCLUSIONS This case series shows the positive effect of HDM-SCT in this rare disorder. Factors that may portend an unfavorable outcome are a long disease course before the hematologic treatment and a poor hematologic response. Age at onset, level and type of M protein (κ vs λ), and severity of muscle weakness were not associated with a specific outcome. CLASSIFICATION OF EVIDENCE This study provides Class IV evidence that for patients with SLONM-MGUS, HDM-SCT increases the probability of survival and functional improvement.

The rotation state of 67P/Churyumov-Gerasimenko from approach observations with the OSIRIS cameras on Rosetta

Aims. Approach observations with the Optical, Spectroscopic, and Infrared Remote Imaging System (OSIRIS) experiment onboard Rosetta are used to determine the rotation period, the direction of the spin axis, and the state of rotation of comet 67P’s nucleus. Methods. Photometric time series of 67P have been acquired by OSIRIS since the post wake-up commissioning of the payload in March 2014. Fourier analysis and convex shape inversion methods have been applied to the Rosetta data as well to the available ground-based observations. Results. Evidence is found that the rotation rate of 67P has significantly changed near the time of its 2009 perihelion passage, probably due to sublimation-induced torque. We find that the sidereal rotation periods P1 = 12.76129 ± 0.00005 h and P2 = 12.4043 ± 0.0007 h for the apparitions before and after the 2009 perihelion, respectively, provide the best fit to the observations. No signs of multiple periodicity are found in the light curves down to the noise level, which implies that the comet is presently in a simple rotation state around its axis of largest moment of inertia. We derive a prograde rotation model with spin vector J2000 ecliptic coordinates λ = 65° ± 15°, β = + 59° ± 15°, corresponding to equatorial coordinates RA = 22°, Dec = + 76°. However, we find that the mirror solution, also prograde, at λ = 275° ± 15°, β = + 50° ± 15° (or RA = 274°, Dec = + 27°), is also possible at the same confidence level, due to the intrinsic ambiguity of the photometric problem for observations performed close to the ecliptic plane.

Lung Nodule Detection by Microdose CT Versus Chest Radiography (Standard and Dual-Energy Subtracted).

OBJECTIVE The purpose of this study was to investigate the feasibility of microdose CT using a comparable dose as for conventional chest radiographs in two planes including dual-energy subtraction for lung nodule assessment. MATERIALS AND METHODS We investigated 65 chest phantoms with 141 lung nodules, using an anthropomorphic chest phantom with artificial lung nodules. Microdose CT parameters were 80 kV and 6 mAs, with pitch of 2.2. Iterative reconstruction algorithms and an integrated circuit detector system (Stellar, Siemens Healthcare) were applied for maximum dose reduction. Maximum intensity projections (MIPs) were reconstructed. Chest radiographs were acquired in two projections with bone suppression. Four blinded radiologists interpreted the images in random order. RESULTS A soft-tissue CT kernel (I30f) delivered better sensitivities in a pilot study than a hard kernel (I70f), with respective mean (SD) sensitivities of 91.1% ± 2.2% versus 85.6% ± 5.6% (p = 0.041). Nodule size was measured accurately for all kernels. Mean clustered nodule sensitivity with chest radiography was 45.7% ± 8.1% (with bone suppression, 46.1% ± 8%; p = 0.94); for microdose CT, nodule sensitivity was 83.6% ± 9% without MIP (with additional MIP, 92.5% ± 6%; p < 10(-3)). Individual sensitivities of microdose CT for readers 1, 2, 3, and 4 were 84.3%, 90.7%, 68.6%, and 45.0%, respectively. Sensitivities with chest radiography for readers 1, 2, 3, and 4 were 42.9%, 58.6%, 36.4%, and 90.7%, respectively. In the per-phantom analysis, respective sensitivities of microdose CT versus chest radiography were 96.2% and 75% (p < 10(-6)). The effective dose for chest radiography including dual-energy subtraction was 0.242 mSv; for microdose CT, the applied dose was 0.1323 mSv. CONCLUSION Microdose CT is better than the combination of chest radiography and dual-energy subtraction for the detection of solid nodules between 5 and 12 mm at a lower dose level of 0.13 mSv. Soft-tissue kernels allow better sensitivities. These preliminary results indicate that microdose CT has the potential to replace conventional chest radiography for lung nodule detection.

Adapting agricultural water governance to climate change: Experiences from Germany, Spain and California

This study describes and discusses initiatives taken by public (water) agencies in the state of Brandenburg in Germany, the state of California in the USA and the Ebro River Basin in Spain in response to the challenges which climate change poses for the agricultural water sector. The drivers and actors and the process of changing agricultural water governance are its particular focus. The assumptions discussed are: (i) the degree of planned and anticipatory top-down implementation processes decreases if actions are more decentralized and are introduced at the regional and local level; (ii) the degree of autonomous and responsive adaptation approaches seems to grow with actions at a lower administrative level. Looking at processes of institutional change, a variety of drivers and actors are at work such as changing perceptions of predicted climate impacts; international obligations which force politicians to take action; socio-economic concerns such as the cost of not taking action; the economic interests of the private sector. Drivers are manifold and often interact and, in many cases, reforms in the sector are driven by and associated with larger reform agendas. The results of the study may serve as a starting point in assisting water agencies in developing countries with the elaboration of coping strategies for tackling climate change-induced risks related to agricultural water management.

Search for the lepton flavor violating decay Z→eμ in pp collisions at √s =8 TeV with the ATLAS detector

The ATLAS detector at the Large Hadron Collider is used to search for the lepton flavor violating process Z→eμ in pp collisions using 20.3  fb −1 of data collected at s √ =8  TeV . An enhancement in the eμ invariant mass spectrum is searched for at the Z -boson mass. The number of Z bosons produced in the data sample is estimated using events of similar topology, Z→ee and μμ , significantly reducing the systematic uncertainty in the measurement. There is no evidence of an enhancement at the Z -boson mass, resulting in an upper limit on the branching fraction, B(Z→eμ)<7.5×10 −7 at the 95% confidence level.

Search for new resonances in Wѵ and ℤѵ final states in pp collisions at √s=8 TeV with the ATLAS detector

This Letter presents a search for new resonances decaying to final states with a vector boson produced in association with a high transverse momentum photon, Vγ, with V=W(→ℓν)or Z(→ℓ+ℓ−), where ℓ =e or μ. The measurements use 20.3fb−1 of proton–proton collision data at a center-of-mass energy of √s=8TeV recorded with the ATLAS detector. No deviations from the Standard Model expectations are found, and production cross section limits are set at 95% confidence level. Masses of the hypothetical aT and ωT states of a benchmark Low Scale Technicolor model are excluded in the ranges [275, 960] GeVand [200, 700] ∪[750, 890] GeV, respectively. Limits at 95% confidence level on the production cross section of a singlet scalar resonance decaying to Zγ final states have also been obtained for masses below 1180 GeV.

Search for new particles in events with one lepton and missing transverse momentum in pp collisions at √s = 8 TeV with the ATLAS detector

This paper presents a search for new particles in events with one lepton (electron or muon) and missing transverse momentum using 20.3 fb−1 of proton-proton collision data at TeX = 8 TeV recorded by the ATLAS experiment at the Large Hadron Collider. No significant excess beyond Standard Model expectations is observed. A W ′ with Sequential Standard Model couplings is excluded at the 95% confidence level for masses up to 3.24 TeV. Excited chiral bosons (W *) with equivalent coupling strengths are excluded for masses up to 3.21 TeV. In the framework of an effective field theory limits are also set on the dark matter-nucleon scattering cross-section as well as the mass scale M * of the unknown mediating interaction for dark matter pair production in association with a leptonically decaying W.

Search for Scalar Diphoton Resonances in the Mass Range 65-600 GeV with the ATLAS Detector in pp Collision Data at √s = 8 TeV

A search for scalar particles decaying via narrow resonances into two photons in the mass range 65–600 GeV is performed using 20.3  fb −1 of s √ =8  TeV pp collision data collected with the ATLAS detector at the Large Hadron Collider. The recently discovered Higgs boson is treated as a background. No significant evidence for an additional signal is observed. The results are presented as limits at the 95% confidence level on the production cross section of a scalar boson times branching ratio into two photons, in a fiducial volume where the reconstruction efficiency is approximately independent of the event topology. The upper limits set extend over a considerably wider mass range than previous searches.

Search for pair-produced third-generation squarks decaying via charm quarks or in compressed supersymmetric scenarios in pp collisions at √s=8 TeV with the ATLAS detector

Results of a search for supersymmetry via direct production of third-generation squarks are reported, using 20.3  fb −1 of proton-proton collision data at √s =8  TeV recorded by the ATLAS experiment at the LHC in 2012. Two different analysis strategies based on monojetlike and c -tagged event selections are carried out to optimize the sensitivity for direct top squark-pair production in the decay channel to a charm quark and the lightest neutralino (t 1 →c+χ ˜ 0 1 ) across the top squark–neutralino mass parameter space. No excess above the Standard Model background expectation is observed. The results are interpreted in the context of direct pair production of top squarks and presented in terms of exclusion limits in the m ˜t 1, m ˜ X0 1 ) parameter space. A top squark of mass up to about 240 GeV is excluded at 95% confidence level for arbitrary neutralino masses, within the kinematic boundaries. Top squark masses up to 270 GeV are excluded for a neutralino mass of 200 GeV. In a scenario where the top squark and the lightest neutralino are nearly degenerate in mass, top squark masses up to 260 GeV are excluded. The results from the monojetlike analysis are also interpreted in terms of compressed scenarios for top squark-pair production in the decay channel t ˜ 1 →b+ff ′ +χ ˜ 0 1 and sbottom pair production with b ˜ 1 →b+χ ˜ 0 1 , leading to a similar exclusion for nearly mass-degenerate third-generation squarks and the lightest neutralino. The results in this paper significantly extend previous results at colliders.

Search for supersymmetry in events with large missing transverse momentum, jets, and at least one tau lepton in 20 fb⁻¹ of √s= 8 TeV proton-proton collision data with the ATLAS detector

A search for supersymmetry (SUSY) in events with large missing transverse momentum, jets, at least one hadronically decaying tau lepton and zero or one additional light leptons (electron/muon), has been performed using 20.3 fb−1 of proton-proton collision data at √s = 8TeV recorded with the ATLAS detector at the Large Hadron Collider. No excess above the Standard Model background expectation is observed in the various signal regions and 95% confidence level upper limits on the visible cross section for new phenomena are set. The results of the analysis are interpreted in several SUSY scenarios, significantly extending previous limits obtained in the same final states. In the framework of minimal gauge-mediated SUSY breaking models, values of the SUSY breaking scale ʌ below 63TeV are excluded, independently of tan β. Exclusion limits are also derived for an mSUGRA/CMSSM model, in both the R-parity-conserving and R-parity-violating case. A further interpretation is presented in a framework of natural gauge mediation, in which the gluino is assumed to be the only light coloured sparticle and gluino masses below 1090GeV are excluded.

Search for squarks and gluinos with the ATLAS detector in final states with jets and missing transverse momentum using √s=8 TeV proton--proton collision data

A search for squarks and gluinos in final states containing high-pT jets, missing transverse momentum and no electrons or muons is presented. The data were recorded in 2012 by the ATLAS experiment in √s = 8TeV proton-proton collisions at the Large Hadron Collider, with a total integrated luminosity of 20.3 fb−1. Results are interpreted in a variety of simplified and specific supersymmetry-breaking models assuming that R-parity is conserved and that the lightest neutralino is the lightest supersymmetric particle. An exclusion limit at the 95% confidence level on the mass of the gluino is set at 1330GeV for a simplified model incorporating only a gluino and the lightest neutralino. For a simplified model involving the strong production of first- and second-generation squarks, squark masses below 850GeV (440GeV) are excluded for a massless lightest neutralino, assuming mass degenerate (single light-flavour) squarks. In mSUGRA/CMSSM models with tan β = 30, A0 = −2m0 and μ > 0, squarks and gluinos of equal mass are excluded for masses below 1700GeV. Additional limits are set for non-universal Higgs mass models with gaugino mediation and for simplified models involving the pair production of gluinos, each decaying to a top squark and a top quark, with the top squark decaying to a charm quark and a neutralino. These limits extend the region of supersymmetric parameter space excluded by previous searches with the ATLAS detector.

Evidence for Electroweak Production of W±W±jj in pp Collisions at √s=8 TeV with the ATLAS Detector

This Letter presents the first study of W ± W ± jj , same-electric-charge diboson production in association with two jets, using 20.3  fb −1 of proton-proton collision data at s √ =8  TeV recorded by the ATLAS detector at the Large Hadron Collider. Events with two reconstructed same-charge leptons (e ± e ± , e ± μ ± , and μ ± μ ± ) and two or more jets are analyzed. Production cross sections are measured in two fiducial regions, with different sensitivities to the electroweak and strong production mechanisms. First evidence for W ± W ± jj production and electroweak-only W ± W ± jj production is observed with a significance of 4.5 and 3.6 standard deviations, respectively. The measured production cross sections are in agreement with standard model predictions. Limits at 95% confidence level are set on anomalous quartic gauge couplings

Search for supersymmetry in events with four or more leptons in √s = 8 TeV pp collisions with the ATLAS detector

Results from a search for supersymmetry in events with four or more leptons including electrons, muons and taus are presented. The analysis uses a data sample corresponding to 20.3  fb −1 of proton-proton collisions delivered by the Large Hadron Collider at s √ =8  TeV and recorded by the ATLAS detector. Signal regions are designed to target supersymmetric scenarios that can be either enriched in or depleted of events involving the production of a Z boson. No significant deviations are observed in data from standard model predictions and results are used to set upper limits on the event yields from processes beyond the standard model. Exclusion limits at the 95% confidence level on the masses of relevant supersymmetric particles are obtained. In R -parity-violating simplified models with decays of the lightest supersymmetric particle to electrons and muons, limits of 1350 and 750 GeV are placed on gluino and chargino masses, respectively. In R -parity-conserving simplified models with heavy neutralinos decaying to a massless lightest supersymmetric particle, heavy neutralino masses up to 620 GeV are excluded. Limits are also placed on other supersymmetric scenarios.

Search for microscopic black holes and string balls in final states with leptons and jets with the ATLAS detector at √s = 8 TeV

A search for an excess of events with multiple high transverse momentum objects including charged leptons and jets is presented, using 20.3 fb−1 of proton-proton collision data recorded by the ATLAS detector at the Large Hadron Collider in 2012 at a centre-of-mass energy of √s = 8TeV. No excess of events beyond Standard Model expectations is observed. Using extra-dimensional models for black hole and string ball production and decay, exclusion contours are determined as a function of the mass threshold for production and the fundamental gravity scale for two, four and six extra dimensions. For six extra dimensions, mass thresholds of 4.8–6.2TeV are excluded at 95% confidence level, depending on the fundamental gravity scale and model assumptions. Upper limits on the fiducial cross-sections for non-Standard Model production of these final states are set.

Search for high-mass dilepton resonances in pp collisions at √s=8 TeV with the ATLAS detector

The ATLAS detector at the Large Hadron Collider is used to search for high-mass resonances decaying to dielectron or dimuon final states. Results are presented from an analysis of proton-proton (pp ) collisions at a center-of-mass energy of 8 TeV corresponding to an integrated luminosity of 20.3  fb −1 in the dimuon channel. A narrow resonance with Standard Model Z couplings to fermions is excluded at 95% confidence level for masses less than 2.79 TeV in the dielectron channel, 2.53 TeV in the dimuon channel, and 2.90 TeV in the two channels combined. Limits on other model interpretations are also presented, including a grand-unification model based on the E 6 gauge group, Z ∗ bosons, minimal Z' models, a spin-2 graviton excitation from Randall-Sundrum models, quantum black holes, and a minimal walking technicolor model with a composite Higgs boson.