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

Comparison of Bond Character in Hydrocarbons and Fullerenes

We present a comparison of the bond polarizabilities for carbon-carbon bonds in hydrocarbons and fullerenes, using two different models for the fullerene Raman spectrum and the results of Raman measurements on ethane and ethylene. We find that the polarizabilities for single bonds in fullerenes and hydrocarbons compare well, while the double bonds in fullerenes have greater polarizability than in ethylene.Comment: 7 pages, no figures, uses RevTeX. (To appear in Phys. Rev. B.

Quasi one dimensional 4^4He inside carbon nanotubes

We report results of diffusion Monte Carlo calculations for both $^4$He absorbed in a narrow single walled carbon nanotube (R = 3.42 \AA) and strictly one dimensional $^4$He. Inside the tube, the binding energy of liquid $^4$He is approximately three times larger than on planar graphite. At low linear densities, $^4$He in a nanotube is an experimental realization of a one-dimensional quantum fluid. However, when the density increases the structural and energetic properties of both systems differ. At high density, a quasi-continuous liquid-solid phase transition is observed in both cases.Comment: 11 pages, 3ps figures, to appear in Phys. Rev. B (RC

Pressure dependence of the thermoelectric power of single-walled carbon nanotubes

We have measured the thermoelectric power (S) of high purity single-walled carbon nanotube mats as a function of temperature at various hydrostatic pressures up to 2.0 GPa. The thermoelectric power is positive, and it increases in a monotonic way with increasing temperature for all pressures. The low temperature (T < 40 K) linear thermoelectric power is pressure independent and is characteristic for metallic nanotubes. At higher temperatures it is enhanced and though S(T) is linear again above about 100 K it has a nonzero intercept. This enhancement is strongly pressure dependent and is related to the change of the phonon population with hydrostatic pressure.Comment: 4 pages, 3 figure

Midinfrared Conductivity in Orientationally Disordered Doped Fullerides

The coupling between the intramolecular vibrational modes and the doped conduction electrons in $M_3C_{60}$ is studied by a calculation of the electronic contributions to the phonon self energies. The calculations are carried out for an orientationally ordered reference solid with symmetry $Fm \bar{3} m$ and for a model with quenched orientational disorder on the fullerene sites. In both cases, the dispersion and symmetry of the renormalized modes is governed by the electronic contributions. The current current correlation functions and frequency dependent conductivity through the midinfrared are calculated for both models. In the disordered structures, the renormalized modes derived from even parity intramolecular phonons are resonant with the dipole excited single particle spectrum, and modulate the predicted midinfrared conductivity. The spectra for this coupled system are calculated for several recently proposed microscopic models for the electron phonon coupling, and a comparison is made with recent experimental data which demonstrate this effect.Comment: 32 pages + 9 postscript figures (on request), REVTeX 3.

Size Effects in Carbon Nanotubes

The inter-shell spacing of multi-walled carbon nanotubes was determined by analyzing the high resolution transmission electron microscopy images of these nanotubes. For the nanotubes that were studied, the inter-shell spacing ${\hat{d}_{002}}$ is found to range from 0.34 to 0.39 nm, increasing with decreasing tube diameter. A model based on the results from real space image analysis is used to explain the variation in inter-shell spacings obtained from reciprocal space periodicity analysis. The increase in inter-shell spacing with decreased nanotube diameter is attributed to the high curvature, resulting in an increased repulsive force, associated with the decreased diameter of the nanotube shells.Comment: 4 pages. RevTeX. 4 figure

Bundling up carbon nanotubes through Wigner defects

We show, using ab initio total energy density functional theory, that the so-called Wigner defects, an interstitial carbon atom right besides a vacancy, which are present in irradiated graphite can also exist in bundles of carbon nanotubes. Due to the geometrical structure of a nanotube, however, this defect has a rather low formation energy, lower than the vacancy itself, suggesting that it may be one of the most important defects that are created after electron or ion irradiation. Moreover, they form a strong link between the nanotubes in bundles, increasing their shear modulus by a sizeable amount, clearly indicating its importance for the mechanical properties of nanotube bundles.Comment: 5 pages and 4 figure

Field-effect transistors assembled from functionalized carbon nanotubes

We have fabricated field effect transistors from carbon nanotubes using a novel selective placement scheme. We use carbon nanotubes that are covalently bound to molecules containing hydroxamic acid functionality. The functionalized nanotubes bind strongly to basic metal oxide surfaces, but not to silicon dioxide. Upon annealing, the functionalization is removed, restoring the electronic properties of the nanotubes. The devices we have fabricated show excellent electrical characteristics.Comment: 5 pages, 6 figure

Vibrational spectra of C60C8H8 and C70C8H8 in the rotor-stator and polymer phases

C60-C8H8 and C70-C8H8 are prototypes of rotor-stator cocrystals. We present infrared and Raman spectra of these materials and show how the rotor-stator nature is reflected in their vibrational properties. We measured the vibrational spectra of the polymer phases poly(C60C8H8) and poly(C70C8H8) resulting from a solid state reaction occurring on heating. Based on the spectra we propose a connection pattern for the fullerene in poly(C60C8H8), where the symmetry of the C60 is D2h. On illuminating the C60-C8H8 cocrystal with green or blue light a photochemical reaction was observed leading to a similar product to that of the thermal polymerization.Comment: 26 pages, 8 figures, to appear in Journal of Physical Chemistry B 2nd version: minor changes in wording, accepted version by journa

Electronic transport, structure, and energetics of endohedral Gd@C82 metallofullerenes

Electronic structure and transport properties of the fullerene C$_{82}$ and the metallofullerene Gd@C$_{82}$ are investigated with density functional theory and the Landauer-Buttiker formalism. The ground state structure of Gd@C$_{82}$ is found to have the Gd atom below the C-C bond on the C$_2$ molecular axis of C$_{82}$. Insertion of Gd into C$_{82}$ deforms the carbon chain in the vicinity of the Gd atoms. Significant overlap of the electron distribution is found between Gd and the C$_{82}$ cage, with the transferred Gd electron density localized mainly on the nearest carbon atoms. This charge localization reduces some of the conducting channels for the transport, causing a reduction in the conductivity of the Gd@C$_{82}$ species relative to the empty C$_{82}$ molecule. The electron transport across the metallofullerene is found to be insensitive to the spin state of the Gd atom.Comment: 13 pages, 7 figures, submitted Nano Let