gebhart/patrepeval huggingface dataset:
Case Report: Novel CSF1R Variant in a Patient With Behavioral Variant Frontotemporal Dementia Syndrome With Prodromal Repetitive Scratching Behavior (2022)

<i>CSF1R</i>-related leukoencephalopathy is an autosomal dominant neurodegenerative disease caused by mutations in the tyrosine kinase domain of the colony stimulating factor 1 receptor (CSF1R). Several studies have found that hematogenic stem cell transplantation is an effective disease modifying therapy however the literature regarding prodromal and early symptoms <i>CSF1R</i>-related leukoencephalopathy is limited. We describe a 63-year-old patient with 4 years of repetitive scratching and skin picking behavior followed by 10 years of progressive behavioral, cognitive, and motor decline in a pattern suggesting behavioral variant of frontotemporal dementia. Brain MRI demonstrated prominent frontal and parietal atrophy accompanied by underlying bilateral patchy white matter hyperintensities sparing the U fibers and cavum septum pellucidum. Whole-exome sequencing revealed a novel, predicted deleterious missense variant in a highly conserved amino acid in the tyrosine kinase domain of <i>CSF1R</i> (p.Gly872Arg). Given this evidence and the characteristic clinical and radiological findings this novel variant was classified as likely pathogenic according to the American College of Medical Genetics standard guidelines. Detailed description of the prodromal scratching and skin picking behavior and possible underlying mechanisms in this case furthers knowledge about early manifestations of <i>CSF1R</i>-related leukoencephalopathy with the hope that early detection and timely administration of disease modifying therapies becomes possible.
Performance of GSM and GSM-SM over $\alpha -\mu$ Fading Channel Model (2019)

Generalized spatial modulation (GSM) is a modification to conventional spatial modulation scheme which provide the possibility to use any antenna count for transmitter. In GSM, the same information symbol from more than one active antenna is transmitted simultaneously at any instant. GSM combining with spatial multiplexing (GSM-SM) results in the increase in spectral efficiency while increase in the multiple RF chain requirements cost degrade in BER performance. In this paper, the GSM as well as GSM-SM system have been simulated considering channel as uncorrelated and flat fading with generalized α-μ distributed. The maximum likelihood (ML) method at the receiver is used for detection. The mapping of group data bits to resultant antenna combinations and transmitted symbol vectors for GSM and GSM-SM have been presented with the help of examples. The average bit error rate of the GSM, GSM-SM with different number of active antennas have been evaluated. It is also found that the error rate of GSM-SM is degraded as compared to GSM only.
Transient Evaluation of a GEN IV LFR Demonstration Plant througha Lumped Parameters Analysis of Coupled Kinetics and Thermal-Hydraulics (2010)

A preliminary approach to the simulation of a Generation IV Lead-cooled Fast Reactor (LFR) demonstrator (DEMO) core dynamics has been developed to provide a helpful tool in this early phase of the reactor pre-design -in which all the system specifications are still considered to be open design parameters-, allowing a relatively quick, qualitative analysis of dynamics and stability aspects that cannot be left aside when refining or even finalizing the system configuration.
Novel phosphorus containing polymers (1962)

Abstract Tris‐hydroxymethylphosphine oxide (THPO) derived from commercially available tetrakishydroxy‐methylphosphonium chloride (THPC), was studied for its flame retardant properties in reactions with carboxylic acids, anhydrides, diisocyanates, urea, phenol, acyl and aroyl halides. Then the monobenzylated derivative of THPO referred to as BHPO was studied, which is more stable and more reactive with difunctional molecules.
Internalization of Leishmania mexicana Complex Amastigotes via the Fc Receptor Is Required to Sustain Infection in Murine Cutaneous Leishmaniasis (2000)

We show here that maintenance of Leishmania infections with Leishmania mexicana complex parasites (Leishmania amazonensis and Leishmania pifanoi) is impaired in the absence of circulating antibody. In these studies, we used mice genetically altered to contain no circulating antibody, with and without functional B cells. This experimental design allowed us to rule out a critical role for B cell antigen presentation in Leishmania pathogenesis. In addition, we show that mice lacking the common gamma chain of Fc receptors (FcgammaRI, FcepsilonRI, and FcgammaRIII) are similarly refractory to infection with these parasites. These observations establish a critical role for antibody in the pathogenesis associated with infection by members of the L. mexicana complex.
Optimal resource-aware deployment planning for component-based distributed applications (2004)

Component-based approaches are becoming increasingly popular in the areas of adaptive distributed systems, Web services, and grid computing. In each case, the underlying infrastructure needs to address a deployment problem involving the placement of application components onto computational, data, and network resources across a wide-area environment subject to a variety of qualitative and quantitative constraints. In general, the deployment needs to also introduce auxiliary components (e.g., to compress/decompress data, or invoke GridFTP sessions to make data available at a remote site), and reuse preexisting components and data. To provide the flexibility required in the latter case, recently proposed systems such as Sekitei and Pegasus have proposed solutions that rely upon Al planning-based techniques. Although promising, the inherent complexity of Al planning and the fact that constraints governing component deployment often involve nonlinear and nonreversible functions have prevented such solutions from generating deployments in resource-constrained situations and achieving optimality in terms of overall resource usage or other cost metrics. We address both of these shortcomings in the context of the Sekitei system. Our extension relies upon information supplied by a domain expert, which classifies component behavior into a discrete set of levels. This discretization, often justified in practice, permits the planner to identify cost-optimal plans (whose quality improves with the level definitions) without restricting the form of the constraint functions. We describe the modified Sekitei algorithm, and characterize, using a media stream delivery application, its scaling behavior when generating optimal deployments for various network configurations.
Isolation,Purification and Analysis of Polysaccharide from Aloe (2007)

Aloe polysaccharide has many potential applications in medicine,food,cosmetology health care and other fields,because it has remarkable pharmacological effects such as disinfection,anti-irradiation damage,anti-tumor,decreasing blood sugar and blood lipid,etc.Recent progress in the study on Aloe polysaccharide home and abroad are reviewed in this paper,including its chemical constituents and the methods for its extraction,purification and analysis,which can provide a theoretical foundation for further research and the development of new products.
Calibrating image roughness by estimating Lipschitz exponents, with application to image restoration (2007)

Most images f (x, y) are not smoothly differentiable functions of x and y, but display edges, localized singularities, and other significant fine scale roughness or texture. Correct characterization and calibration of image roughness is vital in many image processing tasks. The L 1 Lipschitz exponent , where 0 < 1, measures fine scale image roughness provided the image is relatively noise free. This paper describes a recently developed mathematical technique for estimating . The method is based on successively blurring the image by convolution with increasingly narrower Gaussians, using commonly available Fast Fourier Transform algorithms.
Magnetic Resonance for Nonrotating Fields (1940)

A treatment of the magnetic resonance is given for a particle with spin \textonehalf{} in a constant field ${H}_{0}$ and under the action of an arbitrary alternating field with circular frequency $\ensuremath{\omega}$ perpendicular to ${H}_{0}$. A method of finding a solution, valid at any time, is given which converges the better the smaller the deviations from a rotating field or the larger ${H}_{0}$. It is shown that in the lowest order correction the shape of the resonance curve is unchanged but that it is shifted by a percentage amount $\frac{{{H}_{1}}^{2}}{16 {{H}_{0}}^{2}}$ where ${H}_{1}$ is the effective amplitude of the oscillating field. This also involves a correction in the values of the magnetic moments thus obtained towards smaller values which however in all practical cases is negligibly small.
Modelization of thickness shear integrated filters (2002)

In order to optimize the design of bulk acoustic wave filters, a model based on the equations governing the thickness modes is established. Structures in which two electroded regions are separated by a "coupling" region are considered. The electroded and the coupling regions are surrounded by an external unelectroded region have a higher cutoff frequency to ensure that the energy can be trapped in the device. Depending on the distance between the electrodes and some other parameters, the resonators can be from strongly (monolithic filters) to negligibly acoustically coupled. A semi-algebraical method of solution is used, making use of series of exact solutions of the equations and of a discretization of the continuity conditions between the different regions. This makes it possible to obtain the eigenfrequencies and the eigenmodes. The forced modes of the structures are then computed as linear combinations of the eigenmodes. Using the forced modes, the terms of the admittance matrix of the four-port structure are calculated in order to obtain the elements of the lattice equivalent circuit.< <ETX xmlns:mml="http://www.w3.org/1998/Math/MathML" xmlns:xlink="http://www.w3.org/1999/xlink">&gt;</ETX>
Laser-Ablation Electrospray Ionization Mass Spectrometry (LAESI®-MS): Ambient Ionization Technology for 2D and 3D Molecular Imaging (2014)

Laser ablation electrospray ionization mass spectrometry is an ambient ionization technique applicable to plant and animal tissue imaging, live-cell imaging (bacterial and fungal colonies), and most recently to cell-by-cell imaging. This ambient pressure technique uses a mid-infrared (mid-IR) laser with a wavelength (2.94 µm) that is strongly absorbed by water to ablate samples. The resultant ablation plume contains a population of neutral molecules from the sample. Ionization occurs via coalescence of the sample molecules with an electrospray plume above the sample, and the sample ions pass into a mass spectrometer for detection. This direct analysis of the tissues alleviates the need for sample preparation, such as rinse steps, the application of a surface coating or matrix, or solvent extraction, all of which adds time to the analysis and may result in sample contamination or loss. The use of the natural water content of tissue enables both 2D and 3D imaging of plant and animal tissue sections, cell colonies on agar plates, and contact lenses. This chapter discusses the advancements in LAESI-MS technology for imaging applications, and describes the Protea LAESI DP-1000 Direct Ionization System, the first integrated commercial instrument system using LAESI technology for imaging.
Control of augmented reality information volume by glabellar fader (2010)

In this paper, we propose a device for controlling the volume of augmented reality information by the glabellar movement. Our purpose is to avoid increasing the sum of the amount of information during the perception of "Real Space +Augmented Reality" by an intuitive and seamless control. For this, we focused on the movement of the glabella (between the eyebrows) when the user stare at objects as a trigger of information presentation. The system detects the movement of the eyebrows by the amount of the light reflected by a photo-reflector, and controlling information volume or the transparency of objects in augmented reality space.
Mobility and satisfaction with a microprocessor-controlled knee in moderately active amputees: A multi-centric randomized crossover trial (2018)

This study enhances the level of evidence to argue equal opportunity for all individuals with transfemoral amputation or knee disarticulation, regardless of their mobility grade, to be provided with appropriate prostheses.
Endogenous Interferon in the Cerebrospinal Fluid of Herpes Encephalitis Patients (1981)

Low titers of antiviral activity were found in the cerebrospinal fluids (CSF) of herpes encephalitis patients when using a sensitive interferon assay. With this dye-uptake assay on human fibroblasts, trisomic for chromosome 21, 1 IU of human fibroblast or leukocyte interferon per milliliter can unequivocally be determined. Eight CSF samples taken during the first 10 days of illness were antivirally active whereas only 1 of 11 taken later proved positive. With the exception of one serum no interferon was found in the serum samples taken simultaneously with the CSF samples. By antibody neutralization using specific antisera against human fibroblast and leukocyte interferon it was found that the antivirally active substance is identical with or closely related to human leukocyte interferon.
SU‐E‐T‐515: Investigating the Linear Energy Transfer Dependency of Different PRESAGE Formulations in a Proton Beam (2015)

Purpose Previous studies have reported an under‐response of PRESAGE in a proton beam as a Result of the extremely high LET in the distal end of the spread out Bragg peak (SOBP). This work is a preliminary investigation to quantify the effect of the formulation, specifically the concentration of halocarbon radical initiator relative to leuco dye, on radical recombination resulting in LET dependence. Methods The traditional PRESAGE formulation developed by Heuris Pharma was altered to constitute radical initiator concentrations of 5, 15, and 30% (low, medium, and high) by weight with all other components balanced to maintain proportionality. Chloroform was specifically examined in this study and all dosimeters were made in‐house. Cylindrical PRESAGE dosimeters (3.5cm diameter and 6cm length) were made for each formulation and irradiated by a 200‐MeV proton beam to 500 cGy across a 2cm SOBP. Dosimeters were read out using the DMOS optical‐CT scanner. The dose distributions were analyzed and dose profiles were used to compare the relative dose response to find the stability across the high‐LET region of the SOBP. LET dependence was measured by the variation to ion chamber measurements for the final 25% of the SOBP (∼0.5cm) prior to the distal‐90 of each profile. Results Relative to ion chamber data, all PRESAGE dosimeters showed an under‐response at the distal end of the SOBP. The medium concentration formulation matched most closely with an average 8.3% under‐response closely followed by the low concentration at 12.2% and then the high concentration at 22.8%. In all three cases, the highest points of discrepancy were in the distal most regions. Conclusion The radical initiator concentration in PRESAGE can be tailored to reduce the LET dependence in a proton beam. This warrants further study to quantify comprehensively the effect of concentration of different halocarbon radical initiators on LET dependency. Grant number 5RO1CA100835
Security and Collaboration Protocols for Mobile and Sensor Networks. (2012)

Research in network and computer system architecture is evolving beyond its traditional focus as mobile devices become ubiquitous and mobile computing triggers dramatic change in the computing world. Mobile devices can form heterogeneous mobile networks that provide distributed services and information access in real time from anywhere in the world. Coincident with this change, the assurance of network and system security and availability becomes an important problem. This problem is challenging because it requires the system to be easy to manage and operate, but also requires reliability and security. For the purpose of securing a network, we usually require authentication, authorization, and accounting. Authentication requires users to prove their identity. Accounting requires intrusion detection or forensic analysis to find attacks, if any, in the system. Finally, authorization requires access control to ensure data privacy. This dissertation aims to design security and collaboration protocols to create a comprehensive trust framework to protect mobile and sensor networks by applying cryptographic algorithms. It makes three primary contributions. First, we propose and implement a distributed authentication protocol called DAPP in wireless sensor networks to allow sensors to authenticate servers without requiring a commonly-used trusted authentication server. DAPP maintains the distributed nature of sensor networks, has low computation and communication overhead, and is resilient to node compromises. Second, to attain security for nodes in mobile ad hoc networks, we present an intrusion detection system (IDS) architecture at the application layer to help detect malicious nodes in the network. We describe the design of this architecture and the use of mobile agents to augment each node's IDS. Finally, we design a trusted group-based information sharing protocol called TGIS for mobile devices to establish a trust relationship with collaborators and enforce data access control between collaborators with different privileges. TGIS is built upon existing trust infrastructures in individual organizations to enable trust management for group collaboration. The security and collaboration protocols presented in this dissertation together achieve secure distributed authentication, authorization, and accounting in mobile and sensor networks.
<title>Switched grating devices for phased-array applications</title> (1996)

Free space optical systems are described that use switchable grating technology and nonlinear materials to form digital time shifters. A cascade of n independently controlled gratings provides 2<SUP>n</SUP> evenly spaced time delay paths. Featured characteristics including potential for excellent switch isolation, spurious beam and crosstalk suppression, reduction in complexity, and low insertion loss are discussed in the context of phased array applications. Varied configurations are discussed including hybrid free- space/guided wave configurations for long time delays; transmission and double-pass digital optical time shifters; and entire array drivers that replace many single-channel time shifters with a single configuration. Using free space micro-optics, many independent time shifter configurations can be compactly stacked. A novel noise suppression device is discussed that enhances the channel isolation and signal purity of the systems.
Neurosurgeon (2017)

The computation for today's intelligent personal assistants such as Apple Siri, Google Now, and Microsoft Cortana, is performed in the cloud. This cloud-only approach requires significant amounts of data to be sent to the cloud over the wireless network and puts significant computational pressure on the datacenter. However, as the computational resources in mobile devices become more powerful and energy efficient, questions arise as to whether this cloud-only processing is desirable moving forward, and what are the implications of pushing some or all of this compute to the mobile devices on the edge.
Flexible C2-Symmetric Bis-Sulfoxides as Ligands in Enantioselective 1,4-Addition of Boronic Acids to Electron-Deficient Alkenes (2013)

The application of acyclic C2-symmetric chelating bis-sulfoxide ligands in the Rh(I)-catalyzed enantioselective 1,4-addition of boronic acids to electron-deficient alkenes is reported. Among the acyclic ethane-bridged bis-sulfoxides tested, the ligand Ferbisox (11), bearing ferrocenyl moieties as substituents at the sulfinyl sulfurs, has exhibited the best results in terms of chemical yield (up to 96%) and enantioselectivity (up to 97% ee). The conjugate addition takes place smoothly in toluene at room temperature in short reaction times (typically 2 h). The reaction scope, including the use of different boronic acids, five-, six-, and seven-membered cyclic enones, an unsaturated lactone, and the most challenging acyclic ketones, is reported. An X-ray diffraction study of the [Ferbisox·RhCl]2 precatalyst clearly exhibits a dimeric structure with an S coordination of the sulfoxide to rhodium. On the basis of the X-ray data and on structural studies conducted in solution by (1)H NMR, a model explaining the high enantioselection observed is proposed.
TheHubble Space Telescope's High‐Speed Photometer (1999)

We present an overview of the performance of the High Speed Photometer (HSP), one of the five original instruments on the Hubble Space Telescope (HST). As its name implies, the HSP was designed for precise high time resolution photometry from visual to ultraviolet wavelengths; sample times as short as 10.7 μs were possible. Three image dissectors along with 23 different broad‐ and narrowband filters spanning the spectral range from 1200 to 7500 Å were used for photometry of stars and galaxies. A fourth image dissector with associated polarization analyzers allowed the measurement of broadband linear polarization at near‐ultraviolet wavelengths. Simultaneous observations in the blue and red could be made using one of the dissectors and a photomultiplier. Except for an instability in the sensitivity of one of the image dissectors and a reduced throughput in a specialized observational mode, the instrument met or exceeded operational specifications until it was removed from HST in 1993 December. The ability of HSP to carry out its primary program, however, was nearly totally compromised by the poor performance of the telescope, i.e., the spherical aberration of the primary mirror that greatly amplified the effects of large spacecraft pointing jitter and telescope "breathing." As a consequence, the real possibility of HST/HSP opening a new era of astronomical photometry was lost. HSP data affected by various telescope problems, as well as data in which these complications were relatively small, are shown. The excellent condition of the HSP after its return to Earth is described briefly.
Nonaqueous Production of Nanostructured Anatase with High-Energy Facets (2008)

Although solution-based synthesis is the most powerful and economic method to create nanostructured anatase TiO(2), under those synthesis conditions the {101} facets are the most thermodynamically stable, making it difficult to create anatase nanomaterials with a large percentage of high-energy {001} or {010} facets exposed. Here, we report a facile nonaqueous synthetic route to prepare anatase nanosheets with exposed {001} facets and high-quality rhombic-shaped anatase nanocrystals with a large percentage of exposed {010} facets. Including adscititious water in the nonaqueous synthesis and eliminating the use of carboxylic acid type capping agents are the two keys to integrating the structural diversity from aqueous systems into large-quantity synthesis in nonaqueous systems. The nanostructured TiO(2) that we prepared exhibits conspicuous activity in the photocatalytic degradation of organic contaminants.