gebhart/patrepeval huggingface dataset:
Burden of Illness in Adults With Hypophosphatasia: Data From the Global Hypophosphatasia Patient Registry (2020)

ABSTRACT Hypophosphatasia (HPP) is a rare, inherited, metabolic disease caused by deficient tissue non-specific alkaline phosphatase activity. This study aims to assess patient-reported pain, disability and health-related quality of life (HRQoL) in a real-world cohort of adults with HPP who were not receiving asfotase alfa during the analysis. Adults (≥18 years old) with HPP (confirmed by ALPL gene mutation and/or low serum alkaline phosphatase activity for age/sex) were identified from the Global HPP Registry (NCT02306720). Demographics, clinical characteristics, and data on patient-reported pain, disability, and HRQoL (assessed by Brief Pain Inventory Short Form [BPI-SF], Health Assessment Questionnaire Disability Index [HAQ-DI], and 36-Item Short-Form Health Survey version 2 [SF-36v2], respectively) were stratified by pediatric- and adult-onset HPP and summarized descriptively. Of the 304 adults included (median [min, max] age 48.6 [18.8, 79.8] years; 74% women), 45% had adult-onset HPP and 33% had pediatric-onset HPP (unknown age of onset, 22%). Of those with data, 38% had experienced ≥5 HPP manifestations and 62% had a history of ≥1 fracture/pseudofracture. Median (Q1, Q3) BPI-SF scores were 3.5 (1.5, 5.3) for pain severity and 3.3 (0.9, 6.2) for pain interference. Median (Q1, Q3) disability on the HAQ-DI was 0.3 (0.0, 0.7). Median (Q1, Q3) physical and mental component summary scores on the SF-36v2 were 42.4 (32.7, 49.9) and 45.3 (36.3, 54.8), respectively. Greater numbers of HPP manifestations experienced/body systems affected correlated significantly with poorer scores on the BPI-SF, HAQ-DI, and SF-36v2 (all p &amp;lt; 0.05). No significant differences between adults with pediatric- and adult-onset HPP were observed for patient-reported outcomes, except for disability and the BPI-SF question “pain at its worst,” which were significantly higher among adults with pediatric- versus adult-onset HPP (p = 0.03 and 0.04, respectively). These data from the Global HPP Registry show that adults with HPP have a substantial burden of illness that is associated with reduced patient-reported HRQoL, regardless of age of disease onset. © 2020 The Authors. Journal of Bone and Mineral Research published by Wiley Periodicals LLC on behalf of American Society for Bone and Mineral Research (ASBMR).
An empirical study of privacy-violating information flows in JavaScript web applications (2010)

The dynamic nature of JavaScript web applications has given rise to the possibility of privacy violating information flows. We present an empirical study of the prevalence of such flows on a large number of popular websites. We have (1) designed an expressive, fine-grained information flow policy language that allows us to specify and detect different kinds of privacy-violating flows in JavaScript code,(2) implemented a new rewriting-based JavaScript information flow engine within the Chrome browser, and (3) used the enhanced browser to conduct a large-scale empirical study over the Alexa global top 50,000 websites of four privacy-violating flows: cookie stealing, location hijacking, history sniffing, and behavior tracking. Our survey shows that several popular sites, including Alexa global top-100 sites, use privacy-violating flows to exfiltrate information about users' browsing behavior. Our findings show that steps must be taken to mitigate the privacy threat from covert flows in browsers.
The Diagnostic and Prognostic Accuracy of Five Markers of Serious Bacterial Infection in Malawian Children with Signs of Severe Infection (2009)

Admission PCT and CRP are useful markers of invasive bacterial infection in severely ill African children. The study of these markers using rapid tests in a less selected cohort would be important in this setting.
Immunology of disorders of neuromuscular transmission (2006)

The neuromuscular junction (NMJ) is a prototype synapse and myasthenia gravis is the prototypic antibody-mediated disorder. There are now three other disorders of neuromuscular transmission caused by antibodies to other essential components of the NMJ. Antibodies to the muscle-specific kinase, MuSK, are defining a new form of myasthenia that can be associated with muscle atrophy. Antibodies to voltage-gated calcium channels are responsible for muscle weakness and autonomic dysfunction in the Lambert Eaton myasthenic syndrome. Antibodies to voltage-gated potassium channels are found in patients with a range of disorders affecting the NMJ, the autonomic system or the central nervous system. The pathogenic mechanisms probably depend on the IgG subclass of the antibodies and are only partly shared between the diseases.
Accelerated proximity queries between convex polyhedra by multi-level Voronoi marching (2002)

We present an accelerated proximity query algorithm between moving convex polyhedra. The algorithm combines Voronoi-based feature tracking with a multi-level-of-detail representation, in order to adapt to the variation in levels of coherence and speed up the computation. It provides a progressive refinement framework for collision detection and distance queries. We have implemented our algorithm and have observed significant performance improvements in our experiments, especially on scenarios where the coherence is low.
Surface Grafting of Vinyl-Functionalized Poly(fluorene)s via Thiol–Ene Click Chemistry (2014)

Thiol-ene chemistry is used for the surface grafting of vinyl-functionalized poly(fluorene) derivatives onto substrates containing free surface thiol groups. The grafting reaction proceeds in a matter of minutes under UV irradiation without photoinitiator, and the resulting surface-bound, solvent-impervious conjugated polymers retain their characteristic optoelectronic properties. End-chain grafted poly(fluorene)s reach greater surface densities than their side-chain grafted counterparts and show less blue-shifting of photoluminescence upon grafting, suggesting that chain end-grafted conjugated polymers experience less disruption of their extended conjugation and adopt a more brush-like surface conformation. Surface grafted poly(fluorene)s showed facile photopatterning, and thin film transistors with semiconducting polymers directly grafted to the dielectric layer showed performances directly comparable to conventional self-assembled layers of performance-improving alkylsilanes.
Effects of metal ion complexation on the spiropyran–merocyanine interconversion: development of a thermally stable photo-switch (1998)

Spectrophotometric absorption and fluorescence measurements of spiropyrans 2 and 3 modified with chelating functionalities, in the presence of Ca2+ and Zn2+, provide evidence of a thermally stable spiropyran–merocyanine photoswitch that is modulated by the metal cations.
Scalable molecular dynamics with NAMD (2005)

NAMD is a parallel molecular dynamics code designed for high-performance simulation of large biomolecular systems. NAMD scales to hundreds of processors on high-end parallel platforms, as well as tens of processors on low-cost commodity clusters, and also runs on individual desktop and laptop computers. NAMD works with AMBER and CHARMM potential functions, parameters, and file formats. This article, directed to novices as well as experts, first introduces concepts and methods used in the NAMD program, describing the classical molecular dynamics force field, equations of motion, and integration methods along with the efficient electrostatics evaluation algorithms employed and temperature and pressure controls used. Features for steering the simulation across barriers and for calculating both alchemical and conformational free energy differences are presented. The motivations for and a roadmap to the internal design of NAMD, implemented in C++ and based on Charm++ parallel objects, are outlined. The factors affecting the serial and parallel performance of a simulation are discussed. Finally, typical NAMD use is illustrated with representative applications to a small, a medium, and a large biomolecular system, highlighting particular features of NAMD, for example, the Tcl scripting language. The article also provides a list of the key features of NAMD and discusses the benefits of combining NAMD with the molecular graphics/sequence analysis software VMD and the grid computing/collaboratory software BioCoRE. NAMD is distributed free of charge with source code at www.ks.uiuc.edu.
Diarrhée motrice. (1986)

The gut is a long convoluted tube, in which food is processed and nutrients, salt and water are absorbed. The degree of absorption depends to a large extent on the degree of contact between the luminal contents and the absorptive epithelium. Motor activity can influence the degree of absorption because it regulates the degree of contact with the epithelium and it may also induce secretion by a reflex mechanism. Many factors that induce diarrhoea are associated with 'abnormal' and highly propagative forms of motor activity that can clear material through the gut, allowing insufficient epithelial contact for absorption. These propulsive motor patterns may be provoked by distension of the gut with fluid, but they can also occur in response to diarrhoeogenic factors when there is minimal distension. Patients who complain of increased frequency, urgency and incontinence but pass normal stool volumes often have an abnormality in the motor activity of the anorectum. Thus, the generation of abnormal or propagated forms of motor activity must be regarded as an important component of the pathogenesis of all types of diarrhoea and an increased stool volume can be regarded as the end result of a vicious spiral (Figure 12) that may start with a primary abnormality in either motor activity or epithelial transport.
Consequences of aberrant ornithine decarboxylase regulation in rat hepatoma cells (1994)

DH23A cells, an alpha-difluoromethylornithine (DFMO)-resistant variant of rat hepatoma tissue culture cells (HTC), contain high levels of very stable ornithine decarboxylase (ODC). In the absence of DFMO, the high ODC activity results in a large accumulation of endogenous putrescine. Concomitant with the putrescine increase is a period of cytostasis and a subsequent loss of viable cells. In contrast, HTC cells with a moderate polyamine content can be maintained in exponential growth. This suggests that a moderate polyamine concentration is necessary for both optimal cell growth and survival. The cytotoxicity observed in the DH23A cells is apparently not due to byproducts of polyamine oxidation or alterations in steady state intracellular pH or free [Ca2+]. It is possible to mimic the effects of high levels of stable ODC by treatment of cells with exogenous putrescine in the presence of DFMO. This suggests that overaccumulation of putrescine is the causative agent in the observed cytotoxicity, although the mechanism is unclear. These data support the hypothesis that downregulation of ODC may be necessary to prevent accumulation of cytotoxic concentrations of the polyamines.
Isotopic compositions of the elements 1997 (Technical Report) (1998)

Abstract
THERMAL INSTABILITY OF A NANOFLUID SATURATING A ROTATING ANISOTROPIC POROUS MEDIUM (2011)

The present work aims at studying the thermal instability in a rotating anisotropic porous layer saturated by a nanofluid. The model used for nanofluid combines the effect of Brownian motion along with thermophoresis, while for a porous medium the Darcy model has been used. Using linear stability analysis the expression for the critical Rayleigh number has been obtained in terms of various nondimensional parameters. The effect of various parameters on the onset of instability has been presented graphically and discussed in detail. Bottom-heavy and top-heavy arrangements of nanoparticles are shown to prefer oscillatory and stationary modes of convection, respectively. Several results are obtained as limiting cases of the present study.
The viability of fatty tissues within adipose aspirates after conventional liposuction: a comprehensive study. (2005)

This study was conducted to evaluate the viability of fatty tissues within adipose aspirates after conventional liposuction and to examine their potential role as a source of donor material for possible future autogenous fat grafting. Samples of adipose aspirates (group I, n = 8) were obtained from adult female patients who underwent a conventional liposuction of the abdomen. Samples of fresh fatty tissues obtained from adult female patients who underwent an abdominoplasty (group II, n = 8) were cut into small pieces and served as a control. All samples were spun at 50 x g for 10 minutes; fatty tissues were then collected from the middle layer after centrifugation for the following studies: trypan blue vital staining for viable fatty cell counts, glycerol-3-phophatase dehydrogenase (G3PDH) assay for functional evaluation of fatty tissues, and routine pathology for histology of fatty tissues. There was no significant difference of viable fatty cell counts in group I compared with group II (2.57 +/- 0.56 versus 2.74 +/- 0.59 x 10/mL, P = 0.56). G3PGH assay showed a marked decrease of the enzyme activity in group I compared with group II (0.34 +/- 0.13 versus 0.76 +/- 0.13 micro/mL, P < 0.0001). Histologically, the normal structure of fatty tissues was found primarily in both groups. Our results indicate that although fatty tissues within adipose aspirates after conventional liposuction maintain normal structure with near the same number of viable fatty cells compared with fresh ones, they have a less-than-optimal level of cellular function and may not survive well after they are transplanted.
Diabetes complications and their potential prevention: Aldose reductase inhibition and other approaches (1999)

Despite recent advances both in the chemistry and molecular pharmacology of antidiabetic drugs, diabetes still remains a life-threatening disease, which tends to spread all over the world. The clinical profile of diabetic subjects is often worsened by the presence of several long-term complications, namely neuropathy, nephropathy, retinopathy, and cataract. Several attempts have been made to prevent or at least to delay them. The most relevant are reported in this review, including the development of compounds acting as aldose reductase inhibitors, anti-advanced glycation end-product drugs, free radical scavengers, vasoactive agents, essential fatty acid supplementation, and neurotropic growth factors.
Rates of Bronchopulmonary Dysplasia in Very Preterm Neonates in Europe: Results from the MOSAIC Cohort (2010)

A wide variability of BPD between European regions may be explained by different local practices; the strongest association however was with degree of immaturity.
Protein phosphorylation and regulation of adaptive responses in bacteria. (1989)

Bacteria continuously adapt to changes in their environment. Responses are largely controlled by signal transduction systems that contain two central enzymatic components, a protein kinase that uses adenosine triphosphate to phosphorylate itself at a histidine residue and a response regulator that accepts phosphoryl groups from the kinase. This conserved phosphotransfer chemistry is found in a wide range of bacterial species and operates in diverse systems to provide different regulatory outputs. The histidine kinases are frequently membrane receptor proteins that respond to environmental signals and phosphorylate response regulators that control transcription. Four specific regulatory systems are discussed in detail: chemotaxis in response to attractant and repellent stimuli (Che), regulation of gene expression in response to nitrogen deprivation (Ntr), control of the expression of enzymes and transport systems that assimilate phosphorus (Pho), and regulation of outer membrane porin expression in response to osmolarity and other culture conditions (Omp). Several additional systems are also examined, including systems that control complex developmental processes such as sporulation and fruiting-body formation, systems required for virulent infections of plant or animal host tissues, and systems that regulate transport and metabolism. Finally, an attempt is made to understand how cross-talk between parallel phosphotransfer pathways can provide a global regulatory curcuitry.
Determinants of catalytic activity and stability of carbonic anhydrase II as revealed by random mutagenesis. (1993)

The functional importance of a conserved hydrophobic face in human carbonic anhydrase II (CAII), including amino acid residues 190-210, was investigated by random mutagenesis. The catalytic activity, inhibitor binding, and level of CAII expression in Escherichia coli of 57 single amino acid variants were measured revealing that the function of amino acids correlates with their secondary structure placement. Side chains of amino acids in beta-sheet structure are required for the formation of folded, stable protein while those in the turn region determine catalytic efficiency and inhibitor specificity. The CAII active site is extremely plastic, accommodating amino acid substitutions of varied size, charge, and hydrophobicity with little effect on catalysis; only substitutions at Leu198 and Thr199 decrease the rates of CO2 hydration and ester hydrolysis more than 5-fold. These results pinpoint the hydrogen bond network, including the zinc-solvent molecule and Thr199, as crucial for high catalytic efficiency and also suggest that Leu198 forms a portion of a CO2 association site. Increased activity is observed for substitutions at Thr200 (esterase) and Leu203 (hydrase). In addition, the pKa of the zinc-bound water molecule varies upon substitution of amino acids which alter the overall charge of the active site. Three residues interact with sulfonamide inhibitors; substitutions at Thr199 decrease binding (up to 10(3)-fold) while mutations at Thr200 and Cys206 increase binding of dansylamide (up to 80-fold). Mutations in the beta-sheet structure (Asp190-Ser197 and Val207-Ile-210) decrease the protein expression of CAII in E. coli, causing the formation of insoluble protein aggregates in many cases. This may suggest an important role for these residues in the folding process. In addition, mutations in Trp192, cis-Pro202, and Trp209 increase thermal lability (up to 5000-fold).
Requirement of IL-4 and liver NK1+ T cells for concanavalin A-induced hepatic injury in mice (1997)

Con A-induced hepatic injury of mice accompanied by elevated transaminase was inhibited after in vivo depletion of liver NK cells and NK1+ T cells with intermediate TCR by anti-NK1 Ab or anti-IL-2Rbeta Ab. However, depletion of liver NK cells alone by anti-asialo-GM1 Ab did not inhibit hepatic injury. Although depletion of NK1+ T cells inhibited Con A-induced IL-2R expression of CD4+ high TCR (TCRhigh) cells and IL-4 mRNA expression of hepatic mononuclear cells, exogenous IL-4 engendered Con A-induced hepatic injury and endowed the expression of IL-2R of CD4+ TCRhigh cells. It was also found that in vivo treatment with anti-IL-4 Ab before Con A administration inhibited Con A-induced hepatic injury. In addition, although Con A did not induce hepatic injury in MHC class I-deficient mice, exogenous IL-4 again engendered severe hepatic injury in these mice. Further, while serum TNF-alpha levels induced by Con A were greatly decreased in NK1+ T cell-depleted mice and class I-deficient mice, TNF-alpha levels were recovered by exogenous IL-4. These findings reveal that although CD4+ TCRhigh cells in the liver and their production of TNF-alpha are the direct effectors of Con A-induced hepatic injury, liver NK1+ T cells also play an important role in this hepatitis model. Con A hepatitis may serve as an experimental model for human autoimmune hepatitis.
Suramin potently inhibits the enzymatic activity of PSM (2000)

This is one of the most potent activities described for suramin to date and may represent a portion of its pharmacologic and/or toxicological mechanism of action.
Integration of face-to-face and video-mediated meetings (1997)

Article Integration of face-to-face and video-mediated meetings: HERMES Share on Authors: Tomoo Inoue Department of Information and Computer Science, Keio University, 3-14-1, Hiyoshi, Kohoku-ku, Yokohama 223 Japan Department of Information and Computer Science, Keio University, 3-14-1, Hiyoshi, Kohoku-ku, Yokohama 223 JapanView Profile , Ken-ichi Okada Department of Information and Computer Science, Keio University, 3-14-1, Hiyoshi, Kohoku-ku, Yokohama 223 Japan Department of Information and Computer Science, Keio University, 3-14-1, Hiyoshi, Kohoku-ku, Yokohama 223 JapanView Profile , Yutaka Matsushita Department of Information and Computer Science, Keio University, 3-14-1, Hiyoshi, Kohoku-ku, Yokohama 223 Japan Department of Information and Computer Science, Keio University, 3-14-1, Hiyoshi, Kohoku-ku, Yokohama 223 JapanView Profile Authors Info & Claims GROUP '97: Proceedings of the international ACM SIGGROUP conference on Supporting group work: the integration challengeNovember 1997 Pages 405–414https://doi.org/10.1145/266838.267361Online:16 November 1997Publication History 10citation465DownloadsMetricsTotal Citations10Total Downloads465Last 12 Months5Last 6 weeks1 Get Citation AlertsNew Citation Alert added!This alert has been successfully added and will be sent to:You will be notified whenever a record that you have chosen has been cited.To manage your alert preferences, click on the button below.Manage my AlertsNew Citation Alert!Please log in to your account Save to BinderSave to BinderCreate a New BinderNameCancelCreateExport CitationPublisher SiteGet Access
A frequency warping approach to speaker normalization (1998)

In an effort to reduce the degradation in speech recognition performance caused by variation in vocal tract shape among speakers, a frequency warping approach to speaker normalization is investigated. A set of low complexity, maximum likelihood based frequency warping procedures have been applied to speaker normalization for a telephone based connected digit recognition task. This paper presents an efficient means for estimating a linear frequency warping factor and a simple mechanism for implementing frequency warping by modifying the filterbank in mel-frequency cepstrum feature analysis. An experimental study comparing these techniques to other well-known techniques for reducing variability is described. The results have shown that frequency warping is consistently able to reduce word error rate by 20% even for very short utterances.