Objective The management of arterial pathology in individuals with vascular Ehlers-Danlos syndrome (vEDS) remains a challenge. Here we describe the correlation between COL3A1 gene mutation type and ...the clinical phenotype in individuals with vEDS. Methods Individuals with confirmed molecular diagnoses of vEDS were enrolled in a multi-institutional natural history study. Data collected included demographics, clinical and family histories, arterial pathology (aneurysm, dissection, and rupture), operative details, and autopsy reports. Individuals were classified into two cohorts by the type of COL3A1 mutations and their effect on the amount of normal collagen produced: those with mutations that lead to minimal (MIN) production (10%-15%) of normal type III collagen and those with haploinsufficiency (HI) mutations that lead to production of 50% of the normal type III collagen. Results A cohort of 68 individuals (72%) from 56 families had arterial pathology (44% male) with 13% HI. The HI group was older at the time of their first vascular event (mean, 42 range, 26-58 years vs 33 range, 8-62 years; P = .016) and had a higher incidence of aortic pathology than the MIN group (56% vs 21%; P = .025). Visceral arterial pathology was seen in 43 arteries in 23 individuals in the MIN group vs only one artery in five individuals in the HI group. Emergency surgical procedures were more likely to be undertaken when vEDS diagnosis was not known (81% vs 41%; P = .005), and 81% of these procedures were open surgical repair compared with 19% endovascular repairs ( P = .019). Open and endovascular repairs were equally used in the elective setting. Postoperative complications were highest when the diagnosis of vEDS was not known (62% vs 14%; P < .001) and when procedures were undertaken in an emergency setting (5% vs 55% P < .001). Mortality due to arterial complications was 0% in the HI cohort and 21% in the MIN cohort ( P = .132). Conclusions Arterial pathology in vEDS individuals is related to the underlying COL3A1 mutation type. The arterial pathology in individuals with HI mutations occurs at later ages with a higher incidence of aortic disease compared with other COL3A1 mutation types. Molecular diagnosis is recommended because diagnosis confirmation, appropriate surveillance, and prophylactic interventions in an elective setting improve surgical outcomes.
We study the chemical evolution of H2O:CO:NH3 ice mixtures irradiated with soft X-rays, in the range of 250-1250 eV. We identify many nitrogen-bearing molecules such as, e.g., OCN−, , HNCO, CH3CN, ...HCONH2, and NH2COCONH2. Several infrared features are compatible with glycine or its isomers. During the irradiation, we detected through mass spectroscopy many species desorbing the ice. Such findings support the infrared identifications and reveal less abundant species without clear infrared features. Among them, m/z = 57 has been ascribed to methyl isocyanate (CH3NCO), a molecule of prebiotic relevance, recently detected in protostellar environments. During the warm-up after the irradiation, several infrared features including the 2168 cm−1 band of OCN−, the 1690 cm−1 band of formamide, and the 1590 cm−1 band associated with three different species, HCOO−, CH3NH2, and survive up to room temperature. Interestingly, many high masses have also been detected. Possible candidates are methylformate, (m/z = 60, HCOOCH3), ethanediamide (m/z = 88, NH2COCONH2), and N-acetyl-L-aspartic acid (m/z = 175). The latter species is compatible with the presence of the m/z = 43, 70, and 80 fragments. Photodesorption of organics is relevant for the detection of such species in the gas phase of cold environments, where organic synthesis in ice mantles should dominate. We estimate the gas-phase enrichment of some selected species in the light of a protoplanetary disk model around young solar-type stars.
We report on the search for spectral irregularities induced by oscillations between photons and axion-like particles (ALPs) in the gamma-ray spectrum of NGC 1275, the central galaxy of the Perseus ...cluster. Using 6 years of Fermi Large Area Telescope data, we find no evidence for ALPs and exclude couplings above 5 times 10 (sup -12) per gigaelectronvolt for ALP masses less than or approximately equal to 0.5 apparent magnitude (m (sub a)) less than or approximately equal to 5 nanoelectronvolts at 95 percent confidence. The limits are competitive withthe sensitivity of planned laboratory experiments, and, together with other bounds, strongly constrain thepossibility that ALPs can reduce the gamma-ray opacity of the Universe.
Thoracic aortic aneurysms involving the root and/or the ascending aorta enlarge over time until an acute tear in the intimal layer leads to a highly fatal condition, an acute aortic dissection (AAD). ...These Stanford type A AADs, in which the tear occurs above the sinotubular junction, leading to the formation of a false lumen in the aortic wall that may extend to the arch and thoracoabdominal aorta. Type B AADs originate in the descending thoracic aorta just distal to the left subclavian artery. Genetic variants and various environmental conditions that disrupt the aortic wall integrity have been identified that increase the risk for thoracic aortic aneurysms and dissections (TAD). In this review, we discuss the predominant TAD-associated risk factors, focusing primarily on the non-genetic factors, and discuss the underlying mechanisms leading to TAD.
The Fermi Large Area Telescope (LAT) has opened the way for comparative studies of cosmic rays (CRs) and high-energy objects in the Milky Way (MW) and in other, external, star-forming galaxies. Using ...2 yr of observations with the Fermi LAT, Local Group galaxy M31 was detected as a marginally extended gamma-ray source, while only an upper limit has been derived for the other nearby galaxy M33. We revisited the gamma-ray emission in the direction of M31 and M33 using more than 7 yr of LAT Pass 8 data in the energy range 0.1 - 100 GeV , presenting detailed morphological and spectral analyses. M33 remains undetected, and we computed an upper limit of 2.0 × 10 − 12 erg cm − 2 s − 1 on the 0.1 - 100 GeV energy flux (95% confidence level). This revised upper limit remains consistent with the observed correlation between gamma-ray luminosity and star formation rate tracers and implies an average CR density in M33 that is at most half of that of the MW. M31 is detected with a significance of nearly 10 . Its spectrum is consistent with a power law with photon index Γ = 2.4 0.1 stat + syst and a 0.1 - 100 GeV energy flux of ( 5.6 0.6 stat + syst ) × 10 − 12 erg cm − 2 s − 1 . M31 is detected to be extended with a 4 significance. The spatial distribution of the emission is consistent with a uniform-brightness disk with a radius of 0 4 and no offset from the center of the galaxy, but nonuniform intensity distributions cannot be excluded. The flux from M31 appears confined to the inner regions of the galaxy and does not fill the disk of the galaxy or extend far from it. The gamma-ray signal is not correlated with regions rich in gas or star formation activity, which suggests that the emission is not interstellar in origin, unless the energetic particles radiating in gamma rays do not originate in recent star formation. Alternative and nonexclusive interpretations are that the emission results from a population of millisecond pulsars dispersed in the bulge and disk of M31 by disrupted globular clusters or from the decay or annihilation of dark matter particles, similar to what has been proposed to account for the so-called Galactic center excess found in Fermi-LAT observations of the MW.
First measurements of beam backgrounds at SuperKEKB Lewis, P.M.; Jaegle, I.; Nakayama, H. ...
Nuclear instruments & methods in physics research. Section A, Accelerators, spectrometers, detectors and associated equipment,
01/2019, Letnik:
914
Journal Article
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The high design luminosity of the SuperKEKB electron–positron collider is expected to result in challenging levels of beam-induced backgrounds in the interaction region. Properly simulating and ...mitigating these backgrounds is critical to the success of the Belle II experiment. We report on measurements performed with a suite of dedicated beam background detectors, collectively known as BEAST II, during the so-called Phase 1 commissioning run of SuperKEKB in 2016, which involved operation of both the high energy ring (HER) of 7 GeV electrons as well as the low energy ring (LER) of 4 GeV positrons. We describe the BEAST II detector systems, the simulation of beam backgrounds, and the measurements performed. The measurements include standard ones of dose rates versus accelerator conditions, and more novel investigations, such as bunch-by-bunch measurements of injection backgrounds and measurements sensitive to the energy spectrum and angular distribution of fast neutrons. We observe beam–gas, Touschek, beam–dust, and injection backgrounds. As there is no final focus of the beams in Phase 1, we do not observe significant synchrotron radiation, as expected. Measured LER beam–gas backgrounds and Touschek backgrounds in both rings are slightly elevated, on average three times larger than the levels predicted by simulation. HER beam–gas backgrounds are on average two orders of magnitude larger than predicted. Systematic uncertainties and channel-to-channel variations are large, so that these excesses constitute only 1–2 sigma level effects. Neutron background rates are higher than predicted and should be studied further. We will measure the remaining beam background processes, due to colliding beams, in the imminent commissioning Phase 2. These backgrounds are expected to be the most critical for Belle II, to the point of necessitating replacement of detector components during the Phase 3 (full-luminosity) operation of SuperKEB.
We report on the Fermi-LAT detection of high-energy emission from the behind-the-limb (BTL) solar flares that occurred on 2013 October 11, and 2014 January 6 and September 1. The Fermi-LAT ...observations are associated with flares from active regions originating behind both the eastern and western limbs, as determined by STEREO. All three flares are associated with very fast coronal mass ejections (CMEs) and strong solar energetic particle events. We present updated localizations of the >100 MeV photon emission, hard X-ray (HXR) and EUV images, and broadband spectra from 10 keV to 10 GeV, as well as microwave spectra. We also provide a comparison of the BTL flares detected by Fermi-LAT with three on-disk flares and present a study of some of the significant quantities of these flares as an attempt to better understand the acceleration mechanisms at work during these occulted flares. We interpret the HXR emission to be due to electron bremsstrahlung from a coronal thin-target loop top with the accelerated electron spectra steepening at semirelativistic energies. The >100 MeV gamma-rays are best described by a pion-decay model resulting from the interaction of protons (and other ions) in a thick-target photospheric source. The protons are believed to have been accelerated (to energies >10 GeV) in the CME environment and precipitate down to the photosphere from the downstream side of the CME shock and landed on the front side of the Sun, away from the original flare site and the HXR emission.
Pure methanol ices have been irradiated with monochromatic soft X-rays of 300 and 550eV close to the 1s resonance edges of C and O, respectively, and with a broadband spectrum (250-1200 eV). The ...infrared (IR) spectra of the irradiated ices show several new products of astrophysical interest such as CH sub(2)OH, H sub(2)CO, CH sub(4), HCOOH, HCOCH sub(2)OH, CH sub(3)COOH, CH sub(3)OCH sub(3), HCOOCH sub(3), and (CH sub(2)OH) sub(2), as well as HCO, CO, and CO sub(2). The effect of X-rays is the result of the combined interactions of photons and electrons with the ice. A significant contribution to the formation and growth of new species in the CH sub(3)OH ice irradiated with X-rays is given by secondary electrons, whose energy distribution depends on the energy of X-ray photons. Within a single experiment, the abundances of the new products increase with the absorbed energy. Monochromatic experiments show that product abundances also increase with the photon energy. However, the abundances per unit energy of newly formed species show a marked decrease in the broadband experiment as compared to irradiations with monochromatic photons, suggesting a possible regulatory role of the energy deposition rate. The number of new molecules produced per absorbed eV in the X-ray experiments has been compared to those obtained with electron and ultraviolet (UV) irradiation experiments.
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