Reliability of functional outcome measures in adults with neurofibromatosis 1

Objectives: To determine intra-rater and inter-rater reliability of functional outcome measures in adults with neurofibromatosis 1 (NF1) and to ascertain how closely objective and subjective measures align. Methods: Forty-nine ambulant adults with NF1 aged 16 years and over were included in this observational study. Median age 31 years (range 16-66), 29 females, 20 males. Participants were video-recorded or photographed performing four functional outcome measures. Four raters from the Neurofibromatosis centre multi-disciplinary team independently scored the measures to determine inter-rater reliability. One rater scored the measures a second time on a separate occasion to determine intra-rater reliability. The measures evaluated were the functional reach, timed up and go, ten metre walk and a modified nine-hole peg tests. Participants also completed a disease specific quality of life questionnaire (INF1-QOL). Results: Inter-rater reliability and intra-rater reliability scores (intra-class coefficient, ICC) were similar for each outcome measure. Excellent rater agreement (ICC r ≥ 0.9) was found for the functional reach, timed up and go and the 10 metre walk tests. Rater agreement was good for the modified 9-hole peg test; ICC r= 0.75 for intra-rater reliability and 0.76 for inter-rater reliability. The timed up and go and the 10 metre walk tests correlated highly with perceived mobility challenges in the quality of life questionnaire (INF1-QOL). Conclusions: The functional reach, timed up and go and 10 metre walk tests are potentially useful outcome measures for monitoring NF1 treatment and will be assessed for validity and reliability in future multi-centre studies

From Monochrome to Technicolor: Simple Generic Approaches to Multicomponent Protein Nanopatterning Using Siloxanes with Photoremovable Protein-Resistant Protecting Groups.

We show that sequential protein deposition is possible by photodeprotection of films formed from a tetraethylene-glycol functionalized nitrophenylethoxycarbonyl-protected aminopropyltriethoxysilane (NPEOC-APTES). Exposure to near-UV irradiation removes the protein-resistant protecting group, and allows protein adsorption onto the resulting aminated surface. The protein resistance was tested using proteins with fluorescent labels and microspectroscopy of two-component structures formed by micro- and nanopatterning and deposition of yellow and green fluorescent proteins (YFP/GFP). Nonspecific adsorption onto regions where the protecting group remained intact was negligible. Multiple component patterns were also formed by near-field methods. Because reading and writing can be decoupled in a near-field microscope, it is possible to carry out sequential patterning steps at a single location involving different proteins. Up to four different proteins were formed into geometric patterns using near-field lithography. Interferometric lithography facilitates the organization of proteins over square cm areas. Two-component patterns consisting of 150 nm streptavidin dots formed within an orthogonal grid of bars of GFP at a period of ca. 500 nm could just be resolved by fluorescence microscopy

The dynamics of vortex generation in superfluid 3He-B

A profound change occurs in the stability of quantized vortices in externally applied flow of superfluid 3He-B at temperatures ~ 0.6 Tc, owing to the rapidly decreasing damping in vortex motion with decreasing temperature. At low damping an evolving vortex may become unstable and generate a new independent vortex loop. This single-vortex instability is the generic precursor to turbulence. We investigate the instability with non-invasive NMR measurements on a rotating cylindrical sample in the intermediate temperature regime (0.3 - 0.6) Tc. From comparisons with numerical calculations we interpret that the instability occurs at the container wall, when the vortex end moves along the wall in applied flow.Comment: revised & extended version. Journal of Low Temperature Physics, accepted (2008

Pension reform, the stock market, capital formation and economic growth: a critical commentary on the World Bank's proposals

Abstract Proposing far-reaching reforms to the pension systems, the World Bank has recently suggested that the existing pay-as-you-go pension systems in many rich as well as poor countries, should be replaced by fully funded, mandatory, preferably private pensions, as the main pillars of the new system. It argues that these reforms will not only benefit the pensioners, but also enhance savings, promote capital formation and economic development. This paper provides a critical examination of the Bank's theses and concludes that it has adopted a one-sided view of the relationships between the key critical variables. The proposed reform may therefore neither protect the old nor achieve faster economic growth

Combination of searches for heavy spin-1 resonances using 139 fb−1 of proton-proton collision data at √s = 13 TeV with the ATLAS detector

A combination of searches for new heavy spin-1 resonances decaying into diferent pairings of W, Z, or Higgs bosons, as well as directly into leptons or quarks, is presented. The data sample used corresponds to 139 fb−1 of proton-proton collisions at √s = 13 TeV collected during 2015–2018 with the ATLAS detector at the CERN Large Hadron Collider. Analyses selecting quark pairs (qq, bb, tt¯, and tb) or third-generation leptons (τν and τ τ ) are included in this kind of combination for the frst time. A simplifed model predicting a spin-1 heavy vector-boson triplet is used. Cross-section limits are set at the 95% confdence level and are compared with predictions for the benchmark model. These limits are also expressed in terms of constraints on couplings of the heavy vector-boson triplet to quarks, leptons, and the Higgs boson. The complementarity of the various analyses increases the sensitivity to new physics, and the resulting constraints are stronger than those from any individual analysis considered. The data exclude a heavy vector-boson triplet with mass below 5.8 TeV in a weakly coupled scenario, below 4.4 TeV in a strongly coupled scenario, and up to 1.5 TeV in the case of production via vector-boson fusion

Measurement of vector boson production cross sections and their ratios using pp collisions at √s = 13.6 TeV with the ATLAS detector

Abstract available from publisher's website

Search for the Zγ decay mode of new high-mass resonances in pp collisions at √s = 13 TeV with the ATLAS detector

This letter presents a search for narrow, high-mass resonances in the Zγ final state with the Z boson decaying into a pair of electrons or muons. The √s = 13 TeV pp collision data were recorded by the ATLAS detector at the CERN Large Hadron Collider and have an integrated luminosity of 140 fb−1. The data are found to be in agreement with the Standard Model background expectation. Upper limits are set on the resonance production cross section times the decay branching ratio into Zγ. For spin-0 resonances produced via gluon–gluon fusion, the observed limits at 95% confidence level vary between 65.5 fb and 0.6 fb, while for spin-2 resonances produced via gluon–gluon fusion (or quark–antiquark initial states) limits vary between 77.4 (76.1) fb and 0.6 (0.5) fb, for the mass range from 220 GeV to 3400 GeV