Truncation mutations of the receptor cytoplasmic domain for colony-stimulating factor 3 (CSF3R) are frequently seen in severe congenital neutropenia, whereas activating missense mutations affecting ...the extracellular domain (exon 14) have been described in hereditary neutrophilia and chronic neutrophilic leukemia (CNL). In order to clarify mutational frequency, specificity and phenotypic associations, we sequenced CSF3R exons 14-17 in 54 clinically suspected cases of CNL (n=35) or atypical chronic myeloid leukemia (aCML; n=19). Central review of these cases confirmed WHO-defined CNL in 12 patients, monoclonal gammopathy (MG)-associated CNL in 5 and WHO-defined aCML in 9. A total of 14 CSF3R mutations were detected in 13 patients, including 10 with CSF3RT618I (exon 14 mutation, sometimes annotated as CSF3R T595I). CSF3RT618I occurred exclusively in WHO-defined CNL with a mutational frequency of 83% (10 of 12 cases). CSF3R mutations were not seen in aCML or MG-associated CNL. CSF3RT618I was also absent among 170 patients with primary myelofibrosis (PMF; n=76) or chronic myelomonocytic leukemia (CMML; n=94). SETBP1 mutational frequencies in WHO-defined CNL, aCML, CMML and PMF were 33, 0, 7 and 3%, respectively. Four CSF3RT618I-mutated cases co-expressed SETBP1 mutations. We conclude that CSF3RT618I is a highly sensitive and specific molecular marker for CNL and should be incorporated into current diagnostic criteria.
Terahertz (THz) radiation promises breakthrough advances in compact advanced accelerators due to the gigavolts-per-meter fields achievable, but the challenge of maintaining overlap and synchronism ...between beams and short laser-generated THz pulses has so far limited interactions to the few-millimeter scale. We implement a novel scheme for simultaneous group and phase velocity matching of nearly single-cycle THz radiation with a relativistic electron beam for meter-scale inverse free-electron laser interaction in a magnetic undulator, resulting in energy modulations of up to 150 keV using modest THz pulse energies (≤1 μJ). Using this scheme, we demonstrate for the first time the use of a laser-based THz source for bunch-length compression and time-stamping of a relativistic electron beam.
As Lost Cause statues come down across the country, communities are forced to reckon with monumental absences. While the rhetorical significance of monuments is well-established in scholarly ...literature, the rhetoric of monumental absence is not as thoroughly covered. To better understand the role that monumental absence plays in both public space and civic life, this essay theorizes residual memory as the remaining rhetorical potency that clings to a commemorative site after the focal object or structure of memorialization is removed. To demonstrate the theoretical utility of residual memory and contribute to ongoing public debates about Lost Cause monuments, this essay uses rhetorical field methods to conduct a rhetorical analysis of Take 'Em Down NOLA's "Second Line to Bury White Supremacy." The secretive night time removal of the Liberty Place Monument in New Orleans on April 24, 2017 and the activist celebration that followed two weeks later provide an exemplary case for exploring how residual memories can be used to resist and revise regressive practices of public memory.
The properties of photoemission electron sources determine the ultimate performance of a wide class of electron accelerators and photon detectors. To date, all high-efficiency visible-light ...photocathode materials are either polycrystalline or exhibit intrinsic surface disorder, both of which limit emitted electron beam brightness. In this Letter, we demonstrate the synthesis of epitaxial thin films of Cs_{3}Sb on 3C-SiC (001) using molecular-beam epitaxy. Films as thin as 4 nm have quantum efficiencies exceeding 2% at 532 nm. We also find that epitaxial films have an order of magnitude larger quantum efficiency at 650 nm than comparable polycrystalline films on Si. Additionally, these films permit angle-resolved photoemission spectroscopy measurements of the electronic structure, which are found to be in good agreement with theory. Epitaxial films open the door to dramatic brightness enhancements via increased efficiency near threshold, reduced surface disorder, and the possibility of engineering new photoemission functionality at the level of single atomic layers.
Using an 800 nm, 45 fs pulse-front-tilted laser we demonstrate a record 315 keV energy gain in a dual grating dielectric laser accelerator (DLA) and average accelerating gradients of 560 MV/m over ...0.5 mm. These results open a new regime in DLA characterized by significant evolution of the beam distribution in the longitudinal phase space, corresponding to > 1/4 of a synchrotron oscillation. By tilting the laser wavefront we control the resonant velocity of the DLA and observe a net energy gain, indicating that a tapered optical phase could be used to achieve very high energy gain.
•Exergy transfer characteristics of turbulent heat transfer enhancement were analyzed in surfactant solution flow.•Energy-saving effect of surfactant additives was evaluated in terms of the flow ...velocity and fluid transport distance.•Static or dynamic mixers were installed for turbulent heat transfer enhancement.•Proposed flow performance curve provided the exergy transfer efficiency for an arbitrary mean exergy transfer Nusselt number.
This paper presents an exergy transfer analysis for turbulent drag reducing surfactant solution flow with and without heat transfer enhancement devices which employ static or dynamic mixers. Heat transfer and pressure drop measurements were performed at an inlet temperature of 297.7–298.2 K in pipe flow with a concentric tube heat exchanger. The environmental temperature in the analysis was set to be 288.2 K in assumption of a cooling system. The experimental range of the Reynolds number was 1.0×104 to 4.2×104. Adding surfactant to a water flow decreases the exergy transfer Nusselt number, Nue, owing to the heat transfer reduction and increases the exergy transfer efficiency, ηeff, by a maximum of 9.3% at high Reynolds numbers at moderate fluid transport distance owing to the drag reduction. Also the secondary flow caused by the enhancement devices increases Nue. The proposed flow performance curve which provides ηeff for an arbitrary Nue shows that ηeff in viscoelastic fluid flow compared to Newtonian fluid flow is small independently of the fluid transport distance, and that the installation of heat transfer enhancement devices has a positive effect on energy-saving in certain ranges of Reynolds numbers and fluid transport distances.
Summary
Our study investigates 25-hydroxyvitamin D levels and fracture risk using population-level data. 25-Hydroxyvitamin D values < 12, 12–19, and > 50 ng/mL were not associated with increased risk ...of fractures overall compared with values 20–50 ng/mL. Severely low levels may be associated with increased risk of osteoporotic fracture, particularly of the wrist.
Introduction
Studies of the relationship between serum 25-hydroxyvitamin D (25(OH)D) levels and fracture risk have been inconsistent. We hypothesized that high 25(OH)D concentrations (> 50 ng/mL) would be associated with increased risk of fracture.
Methods
We identified all adult patients living in Olmsted County, Minnesota, between January 1, 2005 and December 31, 2011, who had at least one 25(OH)D measurement. Fracture outcomes were retrieved starting 30 days after 25(OH)D measurement and until patients’ final clinical visit as an Olmsted County resident, December 31, 2014, or death. Data were analyzed using Cox proportional hazard regression.
Results
Of 11,002 individuals with a 25(OH)D measurement, 5.8% had a 25(OH)D value ˂ 12 ng/mL, and 5.1% had a value > 50 ng/mL. Compared with subjects with 25(OH)D values 20–50 ng/mL (reference group), values < 12, 12–19, and > 50 ng/mL displayed no association with overall fracture risk. After adjusting for a prior diagnosis of osteoporosis/osteopenia, only individuals with values ˂ 12 ng/mL had increased risk of any osteoporotic fracture (aHR = 1.41; 95% CI 1.05–1.89) and wrist fracture (aHR = 2.11; 95% CI 1.27–3.48) compared with the reference group. Compared with the reference group, values ˂ 12 ng/mL were associated with increased risk of any fracture (aHR = 1.35; 95% CI 1.01–1.80), osteoporotic fracture (aHR = 2.18; 95% CI 1.44–3.31), and wrist fracture (aHR = 2.39; 95% CI 1.19–4.81) in subjects without a prior diagnosis of osteoporosis/osteopenia, but not in those with a prior diagnosis of osteoporosis/osteopenia.
Conclusion
Severely low 25(OH)D levels may be associated with increased risk of osteoporotic fracture, particularly of the wrist, but 25(OH)D values > 50 ng/mL were not associated with increased fracture risk.
In the field of beam physics, two frontier topics have taken center stage due to their potential to enable new approaches to discovery in a wide swath of science. These areas are: advanced, high ...gradient acceleration techniques, and x-ray free electron lasers (XFELs). Further, there is intense interest in the marriage of these two fields, with the goal of producing a very compact XFEL. In this context, recent advances in high gradient radio-frequency cryogenic copper structure research have opened the door to the use of surface electric fields between 250 and 500 MV m−1. Such an approach is foreseen to enable a new generation of photoinjectors with six-dimensional beam brightness beyond the current state-of-the-art by well over an order of magnitude. This advance is an essential ingredient enabling an ultra-compact XFEL (UC-XFEL). In addition, one may accelerate these bright beams to GeV scale in less than 10 m. Such an injector, when combined with inverse free electron laser-based bunching techniques can produce multi-kA beams with unprecedented beam quality, quantified by 50 nm-rad normalized emittances. The emittance, we note, is the effective area in transverse phase space (x, p x /m e c) or (y, p y /m e c) occupied by the beam distribution, and it is relevant to achievable beam sizes as well as setting a limit on FEL wavelength. These beams, when injected into innovative, short-period (1-10 mm) undulators uniquely enable UC-XFELs having footprints consistent with university-scale laboratories. We describe the architecture and predicted performance of this novel light source, which promises photon production per pulse of a few percent of existing XFEL sources. We review implementation issues including collective beam effects, compact x-ray optics systems, and other relevant technical challenges. To illustrate the potential of such a light source to fundamentally change the current paradigm of XFELs with their limited access, we examine possible applications in biology, chemistry, materials, atomic physics, industry, and medicine-including the imaging of virus particles-which may profit from this new model of performing XFEL science.
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