Autologous haematopoietic stem cell transplantation for refractory stiff-person syndrome: the UK experience

Stiff Person Syndrome (SPS) is a rare immune-mediated disabling neurological disorder characterised by muscle spasms and high GAD antibodies. There are only a few case reports of autologous haematopoietic stem cell transplantation (auto-HSCT) as a treatment for SPS. Objective To describe the UK experience of treating refractory SPS with auto-HSCT. Methods Between 2015 and 2019, 10 patients with SPS were referred to our institution for consideration of auto-HSCT. Eight patients were deemed suitable for autograft and four were treated. Of the treated patients, three had classical SPS and one had the progressive encephalomyelitis with rigidity and myoclonus variant. All patients were significantly disabled and had failed conventional immunosuppressive therapy. Patients were mobilised with Cyclophosphamide (Cy) 2 g/m2 + G-CSF and conditioned with Cy 200 mg/kg + ATG followed by auto-HSCT. Results Despite their significantly reduced performance status, all patients tolerated the procedure with no unexpected toxicities. Following autograft, all patients improved symptomatically and stopped all forms of immunosuppressive therapies. Two patients were able to ambulate independently from being wheelchair dependent. One patient’s walking distance improved from 300 meters to 5 miles and one patient’s ambulation improved from being confined to a wheelchair to be able to walk with a frame. Two patients became seronegative for anti-GAD antibodies and normalised their neurophysiological abnormalities. Conclusions Auto-HSCT is an intensive but well tolerated and effective treatment option for patients with SPS refractory to conventional immunotherapy. Further work is warranted to optimise patient selection and establish the efficacy, long-term safety, and cost-effectiveness of this treatment

Topological aspects of geometrical signatures of phase transitions

Certain geometric properties of submanifolds of configuration space are numerically investigated for classical lattice phi^4 models in one and two dimensions. Peculiar behaviors of the computed geometric quantities are found only in the two-dimensional case, when a phase transition is present. The observed phenomenology strongly supports, though in an indirect way, a recently proposed topological conjecture about a topology change of the configuration space submanifolds as counterpart of a phase transition.Comment: REVTEX file, 4 pages, 5 figure

Dog ownership and physical activity: A review of the evidence

Background: Dog walking is a strategy for increasing population levels of physical activity (PA). Numerous cross-sectional studies of the relationship between dog ownership and PA have been conducted. The purpose was to review studies comparing PA of dog owners (DO) to nondog owners (NDO), summarize the prevalence of dog walking, and provide recommendations for research. Methods: A review of published studies (1990-2010) examining DO and NDO PA and the prevalence of dog walking was conducted (N = 29). Studies estimating the relationship between dog ownership and PA were grouped to create a pointestimate using meta-analysis. Results: Most studies were conducted in the last 5 years, were cross-sectional, and sampled adults from Australia or the United States. Approximately 60% of DO walked their dog, with a median duration and frequency of 160 minutes/week and 4 walks/week, respectively. Meta-analysis showed DO engage in more walking and PA than NDO and the effect sizes are small to moderate (d = 0.26 and d = 0.16, respectively). Three studies provided evidence of a directional relationship between dog ownership and walking. Conclusions: Longitudinal and interventional studies would provide stronger causal evidence for the relationship between dog ownership and PA. Improved knowledge of factors associated with dog walking will guide intervention research

Comparison between light scattering and gravimetric samplers for PM10 mass concentration in poultry and pig houses

The objective of this study was to compare co-located real-time light scattering devices and equivalent gravimetric samplers in poultry and pig houses for PM10 mass concentration, and to develop animal-specific calibration factors for light scattering samplers. These results will contribute to evaluate the comparability of different sampling instruments for PM10, concentrations. Paired DustTrak light scattering device (DustTrak aerosol monitor, TSI, U.S.) and PM10 gravimetric cyclone sampler were used for measuring PM10 mass concentrations during 24 h periods (from noon to noon) inside animal houses. Sampling was conducted in 32 animal houses in the Netherlands, including broilers, broiler breeders, layers in floor and in aviary system, turkeys, piglets, growing-finishing pigs in traditional and low emission housing with dry and liquid feed, and sows in individual and group housing. A total of 119 pairs of 24 h measurements (55 for poultry and 64 for pigs) were recorded and analyzed using linear regression analysis. Deviations between samplers were calculated and discussed. In poultry, cyclone sampler and DustTrak data fitted well to a linear regression, with a regression coefficient equal to 0.41, an intercept of 0.16 mg m(-3) and a correlation coefficient of 0.91 (excluding turkeys). Results in turkeys showed a regression coefficient equal to 1.1 (P = 0.49), an intercept of 0.06 mg m(-3) (P < 0.0001) and a correlation coefficient of 0.98. In pigs, we found a regression coefficient equal to 0.61, an intercept of 0.05 mg m(-3) and a correlation coefficient of 0.84. Measured PM10 concentrations using DustTraks were clearly underestimated (approx. by a factor 2) in both poultry and pig housing systems compared with cyclone pre-separators. Absolute, relative, and random deviations increased with concentration. DustTrak light scattering devices should be self-calibrated to investigate PM10 mass concentrations accurately in animal houses. We recommend linear regression equations as animal-specific calibration factors for DustTraks instead of manufacturer calibration factors, especially in heavily dusty environments such as animal houses. (C) 2015 Elsevier Ltd. All rights reserved.The study was financed by the Dutch Ministry of Economic affairs, Agriculture and Innovation. Authors wish to thank the Campus de Excelencia Internacional of the Universitat Politecnica de Valencia (Spain) for funding Dr. Cambra-Lopez's postdoc contract.Cambra López, M.; Winkel, A.; Mosquera, J.; Ogink, NW.; Aarnink, AJA. (2015). Comparison between light scattering and gravimetric samplers for PM10 mass concentration in poultry and pig houses. Atmospheric Environment. 111:20-27. doi:10.1016/j.atmosenv.2015.03.051S202711

Sub-femto-g free fall for space-based gravitational wave observatories: LISA pathfinder results

We report the first results of the LISA Pathfinder in-flight experiment. The results demonstrate that two free-falling reference test masses, such as those needed for a space-based gravitational wave observatory like LISA, can be put in free fall with a relative acceleration noise with a square root of the power spectral density of 5.2 ± 0.1 fm s−2/√Hz or (0.54 ± 0.01) × 10−15 g/√Hz, with g the standard gravity, for frequencies between 0.7 and 20 mHz. This value is lower than the LISA Pathfinder requirement by more than a factor 5 and within a factor 1.25 of the requirement for the LISA mission, and is compatible with Brownian noise from viscous damping due to the residual gas surrounding the test masses. Above 60 mHz the acceleration noise is dominated by interferometer displacement readout noise at a level of (34.8 ± 0.3) fm/√Hz, about 2 orders of magnitude better than requirements. At f ≤ 0.5 mHz we observe a low-frequency tail that stays below 12 fm s−2/√Hz down to 0.1 mHz. This performance would allow for a space-based gravitational wave observatory with a sensitivity close to what was originally foreseen for LISA

PHYTO-THREATS: addressing threats to UK forests and woodlands from Phytophthora; identifying risks of spread in trade and methods for mitigation

The multidisciplinary ‘Phyto-threats’ project was initiated in 2016 to address the increasing risks to UK forest and woodland ecosystems from trade-disseminated Phytophthora. A major component of this project was to examine the risk of Phytophthora spread through nursery and trade practices. Close to 4000 water and root samples were collected from plant nurseries located across the UK over a three-year period. Approximately half of the samples tested positive for Phytophthora DNA using a metabarcoding approach with 63 Phytophthora species identified across nurseries, including quarantine-regulated pathogens and species not previously reported in the UK. Phytophthora diversity within nurseries was linked to high-risk management practices such as use of open rather than closed water sources. Analyses of global Phytophthora risks identified biological traits and trade pathways that explained global spread and host range, and which may be of value for horizon-scanning. Phytophthoras having a higher oospore wall index and faster growth rates had wider host ranges, whereas cold-tolerant species had broader geographic and latitudinal ranges. Annual workshops revealed how stakeholder and sector ‘appetite’ for nursery accreditation increased over three years, although an exploratory cost-benefit analysis indicated that the predicted benefits of introducing best practice expected by nurseries outweigh their costs only when a wider range of pests and diseases (for example, Xylella) is considered. However, scenario analyses demonstrated the significant potential carbon costs to society from the introduction and spread of a new tree-infecting Phytophthora: Thus, the overall net benefit to society from nurseries adopting best practice could be substantial

Combination of searches for Higgs boson pairs in pp collisions at \sqrts = 13 TeV with the ATLAS detector

This letter presents a combination of searches for Higgs boson pair production using up to 36.1 fb(-1) of proton-proton collision data at a centre-of-mass energy root s = 13 TeV recorded with the ATLAS detector at the LHC. The combination is performed using six analyses searching for Higgs boson pairs decaying into the b (b) over barb (b) over bar, b (b) over barW(+)W(-), b (b) over bar tau(+)tau(-), W+W-W+W-, b (b) over bar gamma gamma and W+W-gamma gamma final states. Results are presented for non-resonant and resonant Higgs boson pair production modes. No statistically significant excess in data above the Standard Model predictions is found. The combined observed (expected) limit at 95% confidence level on the non-resonant Higgs boson pair production cross-section is 6.9 (10) times the predicted Standard Model cross-section. Limits are also set on the ratio (kappa(lambda)) of the Higgs boson self-coupling to its Standard Model value. This ratio is constrained at 95% confidence level in observation (expectation) to -5.0 &lt; kappa(lambda) &lt; 12.0 (-5.8 &lt; kappa(lambda) &lt; 12.0). In addition, limits are set on the production of narrow scalar resonances and spin-2 Kaluza-Klein Randall-Sundrum gravitons. Exclusion regions are also provided in the parameter space of the habemus Minimal Supersymmetric Standard Model and the Electroweak Singlet Model. For complete list of authors see http://dx.doi.org/10.1016/j.physletb.2019.135103</p

Searches for lepton-flavour-violating decays of the Higgs boson in s=13\sqrt{s}=13 TeV pp\mathit{pp} collisions with the ATLAS detector

This Letter presents direct searches for lepton flavour violation in Higgs boson decays, H → eτ and H → μτ , performed with the ATLAS detector at the LHC. The searches are based on a data sample of proton–proton collisions at a centre-of-mass energy √s = 13 TeV, corresponding to an integrated luminosity of 36.1 fb−1. No significant excess is observed above the expected background from Standard Model processes. The observed (median expected) 95% confidence-level upper limits on the leptonflavour-violating branching ratios are 0.47% (0.34+0.13−0.10%) and 0.28% (0.37+0.14−0.10%) for H → eτ and H → μτ , respectively.publishedVersio