314 research outputs found
Impact of lung function and baseline clinical characteristics on patient-reported outcome measures in systemic sclerosis-associated interstitial lung disease.
OBJECTIVE: The SENSCIS® trial demonstrated a significant reduction of lung function decline in patients with systemic sclerosis (SSc)-associated interstitial lung disease (SSc-ILD) treated with nintedanib, but no significant effect on health-related quality of life (HRQoL). To assess whether SSc/SSc-ILD severity and large changes in lung function correlate with HRQoL, a post-hoc analysis of SENSCIS®, aggregating treatment arms, was undertaken. METHODS: Patient-reported outcome (PRO) measures (St. George's Respiratory Questionnaire [SGRQ], Functional Assessment of Chronic Illness Therapy [FACIT]-Dyspnoea, and Health Assessment Questionnaire-Disability Index [HAQ-DI], incorporating the Scleroderma Health Assessment Questionnaire visual analogue scale [SHAQ VAS]) at baseline and week 52 were assessed for associations to SSc-ILD severity. RESULTS: At baseline and at week 52, forced vital capacity (FVC) 30% fibrosis on high-resolution computed tomography at baseline demonstrated worse PRO measure scores at week 52. After 1 year, patients with a major (>10%) improvement/worsening in FVC demonstrated corresponding improvement/worsening in SGRQ and other PRO measures, significant for the SGRQ symptom domain (p< 0.001). CONCLUSION: Severe SSc-ILD and major deteriorations in lung function have important impacts on HRQoL. Treatments that slow lung function decline and prevent severe SSc-ILD are important to preserve HRQoL. TRIAL REGISTRATION: clinicaltrials.gov, www.clinicaltrials.gov, NCT02597933
Island dynamics and anisotropy during vapor phase epitaxy of m-plane GaN
Using in situ grazing-incidence x-ray scattering, we have measured the diffuse scattering from islands that form during layer-by-layer growth of GaN by metal-organic vapor phase epitaxy on the (101⎯⎯0)(101¯0)(101¯0) m-plane surface. The diffuse scattering is extended in the (0001)(0001)(0001) in-plane direction in reciprocal space, indicating a strong anisotropy with islands elongated along [12⎯⎯10][12¯10] [12¯10] and closely spaced along [0001][0001][0001]. This is confirmed by atomic force microscopy of a quenched sample. Islands were characterized as a function of growth rate F and temperature. The island spacing along [0001][0001][0001] observed during the growth of the first monolayer obeys a power-law dependence on growth rate F−nF−nF−n, with an exponent n=0.25±0.02n=0.25±0.02n=0.25±0.02. The results are in agreement with recent kinetic Monte Carlo simulations, indicating that elongated islands result from the dominant anisotropy in step edge energy and not from surface diffusion anisotropy. The observed power-law exponent can be explained using a simple steady-state model, which gives n = 1/4
Search for the Weak Decay of an H Dibaryon
We have searched for a neutral dibaryon decaying via and
. Our search has yielded two candidate events from which we set
an upper limit on the production cross section. Normalizing to the
inclusive production cross section, we find at 90% C.L., for an of mass
2.15 GeV/.Comment: 11 pages, 6 postscript figures, epsfig, aps, preprint, revte
Neonatal diabetes, gallbladder agenesis, duodenal atresia, and intestinal malrotation caused by a novel homozygous mutation in RFX6
Recently, bi-allelic mutations in the transcription factor RFX6 were described as the cause of a rare condition characterized by neonatal diabetes with pancreatic and biliary hypoplasia and duodenal/jejunal atresia. A male infant developed severe hyperglycemia (446mg/dL) within 24h of birth. Acute abdominal concerns by day five necessitated exploratory surgery that revealed duodenal atresia, gallbladder agenesis, annular pancreas and intestinal malrotation. He also exhibited chronic diarrhea and feeding intolerance, cholestatic jaundice, and subsequent liver failure. He died of sepsis at four months old while awaiting liver transplantation. The phenotype of neonatal diabetes with intestinal atresia and biliary agenesis clearly pointed to RFX6 as the causative gene; indeed, whole exome sequencing revealed a novel homozygous RFX6 mutation c.779A>C; p.Lys260Thr (K260T). This missense mutation also changes the consensus 5′ splice donor site before intron 7 and is thus predicted to cause disruption in splicing. Both parents, who were not known to be related, were heterozygous carriers. Targeted genetic testing based on consideration of phenotypic features may reveal a cause among the many genes now associated with heterogeneous forms of monogenic neonatal diabetes. Our study demonstrates the feasibility of using modern sequencing technology to identify one such rare cause. Continued research is needed to determine the possible cost-effectiveness of this approach, especially when clear phenotypic clues are absent. Further study of patients with RFX6 mutations should clarify its role in pancreatic, intestinal and enteroendocrine cellular development and explain features such as the diarrhea exhibited in our case
Ultrafast terahertz-field-driven ionic response in ferroelectric BaTiO3
The dynamical processes associated with electric field manipulation of the polarization in a ferroelectric remain largely unknown but fundamentally determine the speed and functionality of ferroelectric materials and devices. Here we apply subpicosecond duration, single-cycle terahertz pulses as an ultrafast electric field bias to prototypical BaTiO[subscript 3] ferroelectric thin films with the atomic-scale response probed by femtosecond x-ray-scattering techniques. We show that electric fields applied perpendicular to the ferroelectric polarization drive large-amplitude displacements of the titanium atoms along the ferroelectric polarization axis, comparable to that of the built-in displacements associated with the intrinsic polarization and incoherent across unit cells. This effect is associated with a dynamic rotation of the ferroelectric polarization switching on and then off on picosecond time scales. These transient polarization modulations are followed by long-lived vibrational heating effects driven by resonant excitation of the ferroelectric soft mode, as reflected in changes in the c-axis tetragonality. The ultrafast structural characterization described here enables a direct comparison with first-principles-based molecular-dynamics simulations, with good agreement obtained
The CERES/NA45 Radial Drift Time Projection Chamber
The design, calibration, and performance of the first radial drift Time
Projection Chamber (TPC) are presented. The TPC was built and installed at the
CERES/NA45 experiment at the CERN SPS in the late nineties, with the objective
to improve the momentum resolution of the spectrometer. The upgraded experiment
took data twice, in 1999 and in 2000. After a detailed study of residual
distortions a spatial resolution of 340 um in the azimuthal and 640 um in the
radial direction was achieved, corresponding to a momentum resolution of Dp/p =
sqrt{(1% * p/GeV)^2 + (2%)^2}.Comment: 57 pages, 59 figure
Theoretical methods for the calculation of Bragg curves and 3D distributions of proton beams
The well-known Bragg-Kleeman rule RCSDA = A dot E0p has become a pioneer work
in radiation physics of charged particles and is still a useful tool to
estimate the range RCSDA of approximately monoenergetic protons with initial
energy E0 in a homogeneous medium. The rule is based on the
continuous-slowing-down-approximation (CSDA). It results from a generalized
(nonrelativistic) Langevin equation and a modification of the phenomenological
friction term. The complete integration of this equation provides information
about the residual energy E(z) and dE(z)/dz at each position z (0 <= z <=
RCSDA). A relativistic extension of the generalized Langevin equation yields
the formula RCSDA = A dot (E0 +E02/2M dot c2)p. The initial energy of
therapeutic protons satisfies E0 << 2M dot c2 (M dot c2 = 938.276 MeV), which
enables us to consider the relativistic contributions as correction terms.
Besides this phenomenological starting-point, a complete integration of the
Bethe-Bloch equation (BBE) is developed, which also provides the determination
of RCSDA, E(z) and dE(z)/dz and uses only those parameters given by the BBE
itself (i.e., without further empirical parameters like modification of
friction). The results obtained in the context of the aforementioned methods
are compared with Monte-Carlo calculations (GEANT4); this Monte-Carlo code is
also used with regard to further topics such as lateral scatter, nuclear
interactions, and buildup effects. In the framework of the CSDA, the energy
transfer from protons to environmental atomic electrons does not account for
local fluctuations.Comment: 97 pages review pape
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