C1 inhibitor deficiency: 2014 United Kingdom consensus document

C1 inhibitor deficiency is a rare disorder manifesting with recurrent attacks of disabling and potentially life-threatening angioedema. Here we present an updated 2014 United Kingdom consensus document for the management of C1 inhibitor-deficient patients, representing a joint venture between the United Kingdom Primary Immunodeficiency Network and Hereditary Angioedema UK. To develop the consensus, we assembled a multi-disciplinary steering group of clinicians, nurses and a patient representative. This steering group first met in 2012, developing a total of 48 recommendations across 11 themes. The statements were distributed to relevant clinicians and a representative group of patients to be scored for agreement on a Likert scale. All 48 statements achieved a high degree of consensus, indicating strong alignment of opinion. The recommendations have evolved significantly since the 2005 document, with particularly notable developments including an improved evidence base to guide dosing and indications for acute treatment, greater emphasis on home therapy for acute attacks and a strong focus on service organisation. This article is protected by copyright. All rights reserved

A new grapevine yellows phytoplasma from the Buckland Valley of Victoria, Australia

A new phytoplasma detected in grapevines with grapevine yellows disease from the Buckland Valley of Victoria, Australia was characterized. Buckland Valley grapevine yellows phytoplasma (BVGYp) could not be amplified by PCR using primers specific for the stolbur (STOL, 16SrXII) group of phytoplasmas indicating that it was unlikely to be a STOL group phytoplasma. BVGYp was amplified by PCR using primers specific for both the aster yellows (AV, 16Sr I) and STOL phytoplasma groups, indicating that it may be more closely related to the AY group phytoplasmas. Sequence analysis of 16SrRNA gene sequences showed that BVGYp clustered with AY and STOL groups of phytoplasmas. Sequence similarities were determined by pairwise comparisons of the 16S rDNA sequence of BVGYp WAY and STOL group phytoplasmas and BVGYp was more closely related to the AY group phytoplasmas. Although the data indicate BVGYp may form a newAY subgroup, the similarity coefficients between BVGYp and phytoplasmas from the AY, STOL and Mexican periwinkle virescence groups, derived from putative RFLP patterns, were less than 90%, so BVGYp may actually form a new phytoplasma group.

The First Very Long Baseline Interferometric SETI Experiment

The first Search for Extra-Terrestrial Intelligence (SETI) conducted with Very Long Baseline Interferometry (VLBI) is presented. By consideration of the basic principles of interferometry, we show that VLBI is efficient at discriminating between SETI signals and human generated radio frequency interference (RFI). The target for this study was the star Gliese 581, thought to have two planets within its habitable zone. On 2007 June 19, Gliese 581 was observed for 8 hours at 1230-1544 with the Australian Long Baseline Array. The dataset was searched for signals appearing on all interferometer baselines above five times the noise limit. A total of 222 potential SETI signals were detected and by using automated data analysis techniques, were ruled out as originating from the Gliese 581 system. From our results we place an upper limit of 7 MW/Hz on the power output of any isotropic emitter located in the Gliese 581 system, within this frequency range. This study shows that VLBI is ideal for targeted SETI, including follow-up observations. The techniques presented are equally applicable to next-generation interferometers, such as the long baselines of the Square Kilometre Array (SKA).Comment: 34 pages, 6 figures, 2 tables. Accepted on 25/05/2012 for publication in The Astronomical Journa

Metal-Insulator oscillations in a Two-dimensional Electron-Hole system

The electrical transport properties of a bipolar InAs/GaSb system have been studied in magnetic field. The resistivity oscillates between insulating and metallic behaviour while the quantum Hall effect shows a digital character oscillating from 0 to 1 conducatance quantum e^2/h. The insulating behaviour is attributed to the formation of a total energy gap in the system. A novel looped edge state picture is proposed associated with the appearance of a voltage between Hall probes which is symmetric on magnetic field reversal.Comment: 4 pages, 5 Postscript figures: revised versio

Semapimod Sensitizes Glioblastoma Tumors to Ionizing Radiation by Targeting Microglia

Glioblastoma is the most malignant and lethal form of astrocytoma, with patients having a median survival time of approximately 15 months with current therapeutic modalities. It is therefore important to identify novel therapeutics. There is mounting evidence that microglia (specialized brain-resident macrophages) play a significant role in the development and progression of glioblastoma tumors. In this paper we show that microglia, in addition to stimulating glioblastoma cell invasion, also promote glioblastoma cell proliferation and resistance to ionizing radiation in vitro. We found that semapimod, a drug that selectively interferes with the function of macrophages and microglia, potently inhibits microglia-stimulated GL261 invasion, without affecting serum-stimulated glioblastoma cell invasion. Semapimod also inhibits microglia-stimulated resistance of glioblastoma cells to radiation, but has no significant effect on microglia-stimulated glioblastoma cell proliferation. We also found that intracranially administered semapimod strongly increases the survival of GL261 tumor-bearing animals in combination with radiation, but has no significant benefit in the absence of radiation. In conclusion, our observations indicate that semapimod sensitizes glioblastoma tumors to ionizing radiation by targeting microglia and/or infiltrating macrophages

α1-Adrenergic responsiveness in human skeletal muscle feed arteries: the impact of reducing extracellular pH

What is the central question of this study? In human arteries involved in the regulation of muscle blood flow, there is a lack of data about whether acidosis alters vascular sensitivity to vasoactive agents, as well as altering endothelium dependent vasorelaxation. Little is known about the interaction of metabolites and vascular function in human skeletal muscle feed arteries. • What is the main finding and its importance? Increasing acidosis attenuated the response and sensitivity of the arteries to phenylephrine; this effect was selective to the receptor over smooth muscle. Acidosis did not alter endothelium dependent vasorelaxation. Impaired vasoconstriction coupled with intact vasorelaxation, promotes decreased vascular tone with exposure to acidosis, and may contribute to sympatholysis during exercise

Geographically touring the eastern bloc: British geography, travel cultures and the Cold War

This paper considers the role of travel in the generation of geographical knowledge of the eastern bloc by British geographers. Based on oral history and surveys of published work, the paper examines the roles of three kinds of travel experience: individual private travels, tours via state tourist agencies, and tours by academic delegations. Examples are drawn from across the eastern bloc, including the USSR, Poland, Romania, East Germany and Albania. The relationship between travel and publication is addressed, notably within textbooks, and in the Geographical Magazine. The study argues for the extension of accounts of cultures of geographical travel, and seeks to supplement the existing historiography of Cold War geography

Muonium as a hydrogen analogue in silicon and germanium; quantum effects and hyperfine parameters

We report a first-principles theoretical study of hyperfine interactions, zero-point effects and defect energetics of muonium and hydrogen impurities in silicon and germanium. The spin-polarized density functional method is used, with the crystalline orbitals expanded in all-electron Gaussian basis sets. The behaviour of hydrogen and muonium impurities at both the tetrahedral and bond-centred sites is investigated within a supercell approximation. To describe the zero-point motion of the impurities, a double adiabatic approximation is employed in which the electron, muon/proton and host lattice degrees of freedom are decoupled. Within this approximation the relaxation of the atoms of the host lattice may differ for the muon and proton, although in practice the difference is found to be slight. With the inclusion of zero-point motion the tetrahedral site is energetically preferred over the bond-centred site in both silicon and germanium. The hyperfine and superhyperfine parameters, calculated as averages over the motion of the muon, agree reasonably well with the available data from muon spin resonance experiments.Comment: 20 pages, including 9 figures. To appear in Phys. Rev.

Radial Flow in Au+Au Collisions at E=0.25-1.15 A GeV

A systematic study of energy spectra for light particles emitted at midrapidity from Au+Au collisions at E=0.25-1.15 A GeV reveals a significant non-thermal component consistent with a collective radial flow. This component is evaluated as a function of bombarding energy and event centrality. Comparisons to Quantum Molecular Dynamics (QMD) and Boltzmann-Uehling-Uhlenbeck (BUU) models are made for different equations of state.Comment: 10 pages of text and 4 figures (all ps files in a uuencoded package)

The energy dependence of flow in Ni induced collisions from 400 to 1970A MeV

We study the energy dependence of collective (hydrodynamic-like) nuclear matter flow in 400-1970 A MeV Ni+Au and 1000-1970 A MeV Ni+Cu reactions. The flow increases with energy, reaches a maximum, and then gradually decreases at higher energies. A way of comparing the energy dependence of flow values for different projectile-target mass combinations is introduced, which demonstrates a common scaling behaviour among flow values from different systems.Comment: 12 pages, 3 figures. Submitted to Physical Review Letter