642 research outputs found
Evaluation of real-life outcome data of patients with spinal muscular atrophy treated with nusinersen in Switzerland
Spinal muscular atrophy (SMA) is an autosomal recessive disorder causing progressive proximal muscular, respiratory, and bulbar weakness. We present outcome data on motor function, ventilation, nutrition, and language development of SMA patients treated with nusinersen in Switzerland. This multicenter, observational study included 44 patients. At treatment initiation, after 2 months and then every 4 months we assessed motor function with the Children's Hospital of Philadelphia Infant Test of Neuromuscular Disorders (CHOP-INTEND), Hammersmith Functional Motor Scale expanded (HFMSE) and 6-Minute Walk Test (6MWT). At treatment initiation, patients were 0.1-44.6 years old, treatment duration ranged from 6 to 41 months. All 11 SMA type 1 children achieved higher CHOP-INTEND scores at the last assessment compared to treatment initiation, 4 acquired stable sitting. Six type 1 children were <18 months-old at treatment initiation. Two of them did not need ventilation or nutritional support at the last assessment; three had delayed language development and 3 articulation difficulties. 5/21 SMA type 2 patients achieved higher HFMSE scores. All ambulant type 3 patients showed a gain in the 6MWT. Nusinersen is an effective treatment, with gains in motor function occurring particularly in children and SMA type 1, but also in type 2 and 3, adolescents and adults
Evaluation of real-life outcome data of patients with spinal muscular atrophy treated with nusinersen in Switzerland.
Spinal muscular atrophy (SMA) is an autosomal recessive disorder causing progressive proximal muscular, respiratory, and bulbar weakness. We present outcome data on motor function, ventilation, nutrition, and language development of SMA patients treated with nusinersen in Switzerland. This multicenter, observational study included 44 patients. At treatment initiation, after 2 months and then every 4 months we assessed motor function with the Children's Hospital of Philadelphia Infant Test of Neuromuscular Disorders (CHOP-INTEND), Hammersmith Functional Motor Scale expanded (HFMSE) and 6-Minute Walk Test (6MWT). At treatment initiation, patients were 0.1-44.6 years old, treatment duration ranged from 6 to 41 months. All 11 SMA type 1 children achieved higher CHOP-INTEND scores at the last assessment compared to treatment initiation, 4 acquired stable sitting. Six type 1 children were <18 months-old at treatment initiation. Two of them did not need ventilation or nutritional support at the last assessment; three had delayed language development and 3 articulation difficulties. 5/21 SMA type 2 patients achieved higher HFMSE scores. All ambulant type 3 patients showed a gain in the 6MWT. Nusinersen is an effective treatment, with gains in motor function occurring particularly in children and SMA type 1, but also in type 2 and 3, adolescents and adults
Unemployment insurance and physical activity
Unemployment insurance (UI) reduces the opportunity cost of leisure, but it is unknown whether this additional leisure time is physically active. To obtain unbiased estimates of the effect of UI on physically active leisure participation, I exploit changes in UI program legislation across US states and time. Using nationally representative monthly data between 2003 and 2010 from the Behavioral Risk Factor Surveillance System (BRFSS) and the American Time Use Survey (ATUS), I find evidence that both state UI eligibility expansions and increases in maximum allowable state UI benefits coincide with greater probability of physical activity among the recently unemployed. Based on point estimates, state UI eligibility expansions increased the probability of physical activity participation by 8â10 percentage points among the unemployed with less than a high school education, while a 10% increase in the maximum allowable state UI benefit increased the probability of physical activity by 0.3 to 0.6 percentage points among the unemployed who have completed high school or some college
The IceCube Neutrino Observatory: Instrumentation and Online Systems
The IceCube Neutrino Observatory is a cubic-kilometer-scale high-energy
neutrino detector built into the ice at the South Pole. Construction of
IceCube, the largest neutrino detector built to date, was completed in 2011 and
enabled the discovery of high-energy astrophysical neutrinos. We describe here
the design, production, and calibration of the IceCube digital optical module
(DOM), the cable systems, computing hardware, and our methodology for drilling
and deployment. We also describe the online triggering and data filtering
systems that select candidate neutrino and cosmic ray events for analysis. Due
to a rigorous pre-deployment protocol, 98.4% of the DOMs in the deep ice are
operating and collecting data. IceCube routinely achieves a detector uptime of
99% by emphasizing software stability and monitoring. Detector operations have
been stable since construction was completed, and the detector is expected to
operate at least until the end of the next decade.Comment: 83 pages, 50 figures; updated with minor changes from journal review
and proofin
Neutrinos and Cosmic Rays Observed by IceCube
The core mission of the IceCube Neutrino observatory is to study the origin
and propagation of cosmic rays. IceCube, with its surface component IceTop,
observes multiple signatures to accomplish this mission. Most important are the
astrophysical neutrinos that are produced in interactions of cosmic rays, close
to their sources and in interstellar space. IceCube is the first instrument
that measures the properties of this astrophysical neutrino flux, and
constrains its origin. In addition, the spectrum, composition and anisotropy of
the local cosmic-ray flux are obtained from measurements of atmospheric muons
and showers. Here we provide an overview of recent findings from the analysis
of IceCube data, and their implications on our understanding of cosmic rays.Comment: Review article, to appear in Advances in Space Research, special
issue "Origins of Cosmic Rays
Measurement of the multi-TeV neutrino cross section with IceCube using Earth absorption
Neutrinos interact only very weakly, so they are extremely penetrating.
However, the theoretical neutrino-nucleon interaction cross section rises with
energy such that, at energies above 40 TeV, neutrinos are expected to be
absorbed as they pass through the Earth. Experimentally, the cross section has
been measured only at the relatively low energies (below 400 GeV) available at
neutrino beams from accelerators \cite{Agashe:2014kda, Formaggio:2013kya}. Here
we report the first measurement of neutrino absorption in the Earth, using a
sample of 10,784 energetic upward-going neutrino-induced muons observed with
the IceCube Neutrino Observatory. The flux of high-energy neutrinos transiting
long paths through the Earth is attenuated compared to a reference sample that
follows shorter trajectories through the Earth. Using a fit to the
two-dimensional distribution of muon energy and zenith angle, we determine the
cross section for neutrino energies between 6.3 TeV and 980 TeV, more than an
order of magnitude higher in energy than previous measurements. The measured
cross section is (stat.) (syst.)
times the prediction of the Standard Model \cite{CooperSarkar:2011pa},
consistent with the expectation for charged and neutral current interactions.
We do not observe a dramatic increase in the cross section, expected in some
speculative models, including those invoking new compact dimensions
\cite{AlvarezMuniz:2002ga} or the production of leptoquarks
\cite{Romero:2009vu}.Comment: Preprint version of Nature paper 10.1038/nature2445
Search for astrophysical sources of neutrinos using cascade events in IceCube
The IceCube neutrino observatory has established the existence of a flux of
high-energy astrophysical neutrinos inconsistent with the expectation from
atmospheric backgrounds at a significance greater than . This flux has
been observed in analyses of both track events from muon neutrino interactions
and cascade events from interactions of all neutrino flavors. Searches for
astrophysical neutrino sources have focused on track events due to the
significantly better angular resolution of track reconstructions. To date, no
such sources have been confirmed. Here we present the first search for
astrophysical neutrino sources using cascades interacting in IceCube with
deposited energies as small as 1 TeV. No significant clustering was observed in
a selection of 263 cascades collected from May 2010 to May 2012. We show that
compared to the classic approach using tracks, this statistically-independent
search offers improved sensitivity to sources in the southern sky, especially
if the emission is spatially extended or follows a soft energy spectrum. This
enhancement is due to the low background from atmospheric neutrinos forming
cascade events and the additional veto of atmospheric neutrinos at declinations
.Comment: 14 pages, 9 figures, 1 tabl
- âŠ