Summary
Background
Several new genes and clinical subtypes have been identified since the publication in 2014 of the report of the last International Consensus Meeting on Epidermolysis Bullosa (EB).
...Objectives
We sought to reclassify disorders with skin fragility, with a focus on EB, based on new clinical and molecular data.
Methods
This was a consensus expert review.
Results
In this latest consensus report, we introduce the concept of genetic disorders with skin fragility, of which classical EB represents the prototype. Other disorders with skin fragility, where blisters are a minor part of the clinical picture or are not seen because skin cleavage is very superficial, are classified as separate categories. These include peeling skin disorders, erosive disorders, hyperkeratotic disorders, and connective tissue disorders with skin fragility. Because of the common manifestation of skin fragility, these ‘EB‐related’ disorders should be considered under the EB umbrella in terms of medical and socioeconomic provision of care.
Conclusions
The proposed classification scheme should be of value both to clinicians and researchers, emphasizing both clinical and genetic features of EB.
What is already known about this topic?
Epidermolysis bullosa (EB) is a group of genetic disorders with skin blistering.
The last updated recommendations on diagnosis and classification were published in 2014.
What does this study add?
We introduce the concept of genetic disorders with skin fragility, of which classical EB represents the prototype.
Clinical and genetic aspects, genotype–phenotype correlations, disease‐modifying factors and natural history of EB are reviewed.
Other disorders with skin fragility, e.g. peeling skin disorders, erosive disorders, hyperkeratotic disorders, and connective tissue disorders with skin fragility are classified as separate categories; these ‘EB‐related’ disorders should be considered under the EB umbrella in terms of medical and socioeconomic provision of care.
Linked Comment: Pope. Br J Dermatol 2020; 183:603.
Linked Comment: Pope. Br J Dermatol 2020; 183:603.
Plain language summary available online
Malnutrition occurs frequently in patients with cancer of the gastrointestinal (GI) or head and neck area and can lead to negative outcomes. The aim of this study is to determine the impact of early ...and intensive nutrition intervention (NI) on body weight, body composition, nutritional status, global quality of life (QoL) and physical function compared to usual practice in oncology outpatients receiving radiotherapy to the GI or head and neck area. Outpatients commencing at least 20 fractions of radiotherapy to the GI or head and neck area were randomised to receive intensive, individualised nutrition counselling by a dietitian using a standard protocol and oral supplements if required, or the usual practice of the centre (general advice and nutrition booklet). Outcome parameters were measured at baseline and 4, 8 and 12 weeks after commencing radiotherapy using valid and reliable tools. A total of 60 patients (51 M : 9 F; mean age 61.9+/-14.0 years) were randomised to receive either NI (n=29) or usual care (UC) (n=31). The NI group had statistically smaller deteriorations in weight (P<0.001), nutritional status (P=0.020) and global QoL (P=0.009) compared with those receiving UC. Clinically, but not statistically significant differences in fat-free mass were observed between the groups (P=0.195). Early and intensive NI appears beneficial in terms of minimising weight loss, deterioration in nutritional status, global QoL and physical function in oncology outpatients receiving radiotherapy to the GI or head and neck area. Weight maintenance in this population leads to beneficial outcomes and suggests that this, rather than weight gain, may be a more appropriate aim of NI.
ABSTRACT We present measurements of the evolution of normal-galaxy X-ray emission from 0-7 using local galaxies and galaxy samples in the 6 Ms Chandra Deep Field-South (CDF-S) survey. The majority of ...the CDF-S galaxies are observed at rest-frame energies above 2 keV, where the emission is expected to be dominated by X-ray binary (XRB) populations; however, hot gas is expected to provide small contributions to the observed-frame 1 keV emission at z 1. We show that a single scaling relation between X-ray luminosity ( ) and star-formation rate (SFR) literature, is insufficient for characterizing the average X-ray emission at all redshifts. We establish that scaling relations involving not only SFR, but also stellar mass ( ) and redshift, provide significantly improved characterizations of the average X-ray emission from normal galaxy populations at 0-7. We further provide the first empirical constraints on the redshift evolution of X-ray emission from both low-mass XRB (LMXB) and high-mass XRB (HMXB) populations and their scalings with and SFR, respectively. We find (LMXB)/ and (HMXB)/SFR , and show that these relations are consistent with XRB population-synthesis model predictions, which attribute the increase in LMXB and HMXB scaling relations with redshift as being due to declining host galaxy stellar ages and metallicities, respectively. We discuss how emission from XRBs could provide an important source of heating to the intergalactic medium in the early universe, exceeding that of active galactic nuclei.
Abstract
Vera C. Rubin Observatory is a ground-based astronomical facility under construction, a joint project of the National Science Foundation and the U.S. Department of Energy, designed to ...conduct a multipurpose 10 yr optical survey of the Southern Hemisphere sky: the Legacy Survey of Space and Time. Significant flexibility in survey strategy remains within the constraints imposed by the core science goals of probing dark energy and dark matter, cataloging the solar system, exploring the transient optical sky, and mapping the Milky Way. The survey’s massive data throughput will be transformational for many other astrophysics domains and Rubin’s data access policy sets the stage for a huge community of potential users. To ensure that the survey science potential is maximized while serving as broad a community as possible, Rubin Observatory has involved the scientific community at large in the process of setting and refining the details of the observing strategy. The motivation, history, and decision-making process of this strategy optimization are detailed in this paper, giving context to the science-driven proposals and recommendations for the survey strategy included in this Focus Issue.
Abstract
Electrons at the border of localization generate exotic states of matter across all classes of strongly correlated electron materials and many other quantum materials with emergent ...functionality. Heavy electron metals are a model example, in which magnetic interactions arise from the opposing limits of localized and itinerant electrons. This remarkable duality is intimately related to the emergence of a plethora of novel quantum matter states such as unconventional superconductivity, electronic-nematic states, hidden order and most recently topological states of matter such as topological Kondo insulators and Kondo semimetals and putative chiral superconductors. The outstanding challenge is that the archetypal Kondo lattice model that captures the underlying electronic dichotomy is notoriously difficult to solve for real materials. Here we show, using the prototypical strongly-correlated antiferromagnet CeIn
3
, that a multi-orbital periodic Anderson model embedded with input from ab initio bandstructure calculations can be reduced to a simple Kondo-Heisenberg model, which captures the magnetic interactions quantitatively. We validate this tractable Hamiltonian via high-resolution neutron spectroscopy that reproduces accurately the magnetic soft modes in CeIn
3
, which are believed to mediate unconventional superconductivity. Our study paves the way for a quantitative understanding of metallic quantum states such as unconventional superconductivity.
The SAMI Galaxy Survey will observe 3400 galaxies with the Sydney-AAO Multi-object Integral-field spectrograph (SAMI) on the Anglo-Australian Telescope in a 3-yr survey which began in 2013. We ...present the throughput of the SAMI system, the science basis and specifications for the target selection, the survey observation plan and the combined properties of the selected galaxies. The survey includes four volume-limited galaxy samples based on cuts in a proxy for stellar mass, along with low-stellar-mass dwarf galaxies all selected from the Galaxy And Mass Assembly (GAMA) survey. The GAMA regions were selected because of the vast array of ancillary data available, including ultraviolet through to radio bands. These fields are on the celestial equator at 9, 12 and 14.5 h, and cover a total of 144 deg2 (in GAMA-I). Higher density environments are also included with the addition of eight clusters. The clusters have spectroscopy from 2-degree Field Galaxy Redshift Survey (2dFGRS) and Sloan Digital Sky Survey (SDSS) and photometry in regions covered by the SDSS and/or VLT Survey Telescope/ATLAS. The aim is to cover a broad range in stellar mass and environment, and therefore the primary survey targets cover redshifts 0.004 ... 0.095, magnitudes ... 19.4, stellar masses ..., and environments from isolated field galaxies through groups to clusters of ... (ProQuest: ... denotes formulae/symbols omitted.)
Superconductivity in diamond Sidorov, V. A; Ekimov, E. A; Bauer, E. D ...
Nature,
04/2004, Letnik:
428, Številka:
6982
Journal Article
Recenzirano
Diamond is an electrical insulator well known for its exceptional hardness. It also conducts heat even more effectively than copper, and can withstand very high electric fields. With these physical ...properties, diamond is attractive for electronic applications, particularly when charge carriers are introduced (by chemical doping) into the system. Boron has one less electron than carbon and, because of its small atomic radius, boron is relatively easily incorporated into diamond; as boron acts as a charge acceptor, the resulting diamond is effectively hole-doped. Here we report the discovery of superconductivity in boron-doped diamond synthesized at high pressure (nearly 100,000 atmospheres) and temperature (2,500-2,800 K). Electrical resistivity, magnetic susceptibility, specific heat and field-dependent resistance measurements show that boron-doped diamond is a bulk, type-II superconductor below the superconducting transition temperature Tc 4 K; superconductivity survives in a magnetic field up to Hc2(0) ≥ 3.5 T. The discovery of superconductivity in diamond-structured carbon suggests that Si and Ge, which also form in the diamond structure, may similarly exhibit superconductivity under the appropriate conditions.
The main challenge for light-emitting diodes is to increase the efficiency in the green part of the spectrum. Gallium phosphide (GaP) with the normal cubic crystal structure has an indirect band gap, ...which severely limits the green emission efficiency. Band structure calculations have predicted a direct band gap for wurtzite GaP. Here, we report the fabrication of GaP nanowires with pure hexagonal crystal structure and demonstrate the direct nature of the band gap. We observe strong photoluminescence at a wavelength of 594 nm with short lifetime, typical for a direct band gap. Furthermore, by incorporation of aluminum or arsenic in the GaP nanowires, the emitted wavelength is tuned across an important range of the visible light spectrum (555–690 nm). This approach of crystal structure engineering enables new pathways to tailor materials properties enhancing the functionality.
ABSTRACT We present X-ray source catalogs for the 7 Ms exposure of the Chandra Deep Field-South (CDF-S), which covers a total area of 484.2 arcmin2. Utilizing wavdetect for initial source detection ...and ACIS Extract for photometric extraction and significance assessment, we create a main source catalog containing 1008 sources that are detected in up to three X-ray bands: 0.5-7.0 keV, 0.5-2.0 keV, and 2-7 keV. A supplementary source catalog is also provided, including 47 lower-significance sources that have bright ( ) near-infrared counterparts. We identify multiwavelength counterparts for 992 (98.4%) of the main-catalog sources, and we collect redshifts for 986 of these sources, including 653 spectroscopic redshifts and 333 photometric redshifts. Based on the X-ray and multiwavelength properties, we identify 711 active galactic nuclei (AGNs) from the main-catalog sources. Compared to the previous 4 Ms CDF-S catalogs, 291 of the main-catalog sources are new detections. We have achieved unprecedented X-ray sensitivity with average flux limits over the central 1 arcmin2 region of 1.9 × 10−17, 6.4 × 10−18, and 2.7 × 10−17 erg cm−2 s−1 in the three X-ray bands, respectively. We provide cumulative number-count measurements observing, for the first time, that normal galaxies start to dominate the X-ray source population at the faintest 0.5-2.0 keV flux levels. The highest X-ray source density reaches 50,500 deg−2, and 47% 4% of these sources are AGNs ( 23,900 deg−2).
We present the Lambda Adaptive Multi-Band Deblending Algorithm in R (lambdar), a novel code for calculating matched aperture photometry across images that are neither pixel- nor PSF-matched, using ...prior aperture definitions derived from high-resolution optical imaging. The development of this program is motivated by the desire for consistent photometry and uncertainties across large ranges of photometric imaging, for use in calculating spectral energy distributions. We describe the program, specifically key features required for robust determination of panchromatic photometry: propagation of apertures to images with arbitrary resolution, local background estimation, aperture normalization, uncertainty determination and propagation, and object deblending. Using simulated images, we demonstrate that the program is able to recover accurate photometric measurements in both high-resolution, low-confusion, and low-resolution, high-confusion, regimes. We apply the program to the 21-band photometric data set from the Galaxy And Mass Assembly (GAMA) Panchromatic Data Release (PDR; Driver et al. 2016), which contains imaging spanning the far-UV to the far-IR. We compare photometry derived from lambdar with that presented in Driver et al. (2016), finding broad agreement between the data sets. None the less, we demonstrate that the photometry from lambdar is superior to that from the GAMA PDR, as determined by a reduction in the outlier rate and intrinsic scatter of colours in the lambdar data set. We similarly find a decrease in the outlier rate of stellar masses and star formation rates using lambdar photometry. Finally, we note an exceptional increase in the number of UV and mid-IR sources able to be constrained, which is accompanied by a significant increase in the mid-IR colour–colour parameter-space able to be explored.
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