Miracle-dz/UniFAR-FTUs huggingface dataset:
Studies of the Antihemophilic Factor (AHF, Factor VIII) Produced in von Willebrand's Disease.∗

In 1959 one of us pointed out that the apparent dependence of plasma antihemophilic factor (AHF, F. VIII) level on the activity of 2 genetic loci must have significance with respect to AHF synthesis(l). At that time only structural genes were generally considered operative in protein synthesis. This coupled with the widespread acceptance of the “one gene—one enzyme” concept impeded consideration of models for AHF synthesis involving 2 loci. Since then it has become apparent that both structural and regulatory genes function in bacteria(2) and that 2, non-allelic structural genes are sometimes involved in protein synthesis in mammals, e.g., hemoglobin in humans. It has been found experimentally that the 2 mutant phenotypes showing reduced plasma AHF levels, hemophilia A and von Willebrand's disease (v.W.d.), do not “complement” in vitro as do hemophilias A and B, for example. This is in fact the basis for the assertion that AHF is “deficient” in v.W. d. A paradoxical type of “complementation” does occur in vivo, however. Plasma from persons with autosomal von Willebrand's disease is ineffective when transfused into persons having X-linked hemophilia A(3), but the reciprocal transfusion has a striking effect. When plasma from a person with X-linked hemophilia A and low AHF is transfused into a person with v.W. d. with low plasma AHF, large amounts of AHF appear in the blood (4). The quantity of AHF appearing is so large that it has been interpreted as new synthesis, and the observation has been confirmed in several laboratories (5,6). This finding has led to a considerable elaboration of the possible models of AHF synthesis. The locus on the X-chromosome has been suggested as a structural locus coding for the peptide sequence of AHF, while the locus on the (as yet unidentified) autosomal chromosome has been suggested as regulatory(7,8).
Management and outcomes of a first recurrence of Clostridium difficile-associated disease in Quebec, Canada.

BACKGROUND During an epidemic of Clostridium difficile-associated disease (CDAD) caused by a strain that is a hyper-producer of toxins A and B, the frequency of a first recurrence after metronidazole treatment of the initial episode doubled in 2003-2004, compared with 1991-2002. METHODS To examine whether administration of metronidazole as treatment for a first recurrence of CDAD remained appropriate, we reviewed data for patients with CDAD diagnosed in a hospital in Quebec, Canada, during 1991-2005, who experienced a first recurrence. The frequency of a second recurrence within 60 days after the first one was measured using Kaplan-Meier analysis. Cox regression was used for multivariate analysis. RESULTS A total of 463 patients had a first recurrence of CDAD, of whom 154 (33.3%) experienced a second recurrence. Independent predictors of a second recurrence were age and duration of hospitalization after the first recurrence; this latter finding suggested that many such episodes were reinfections rather than relapses. Neither choice of treatment drug (metronidazole or vancomycin) nor use of the same drug for treatment of first recurrence, as had been used during the initial episode, was associated with increased risk of a second recurrence. However, 51 patients (11.0%) developed at least 1 complication (i.e., shock, need for colectomy, megacolon, perforation, or death within 30 days) during the first recurrence. Older age, a high leukocyte count, and renal failure at first recurrence were strongly associated with a complicated CDAD. CONCLUSIONS Metronidazole is not inferior to vancomycin for treatment of patients with a first recurrence of CDAD, but the risk of complications with any treatment of CDAD may be higher than has previously been documented.
Radiation Hardness Assurance Methodology of Radiation Tolerant Power Converter Controls for Large Hadron Collider

This work describes in detail the Radiation Hardness Assurance methodology applied to the development of new radiation tolerant power converter control system for the Large Hadron Collider at the European Organization for Nuclear Research (CERN). This system is based primarily on commercial-off-the-shelf components for which the extensive testing scheme is applied to decrease the probability of radiation failures during the operation of the LHC machine in the high-luminosity operating conditions era.
The identification possibilities of the measured parameters of an aircraft model and pilot behavior model on the flight simulator

This paper describes obtained mathematical identification results of an aircraft model and parameter analysis of mathematical models of human behavior while flying an aircraft. The part of this paper deals with a model parameters measurement possibility using flight simulator at the University of Defence. The paper also presents options and importance of using the flight simulator for pilot training and utilising the identified simulator parameters, described by transfer functions. These transfer functions are then applied for calculating mathematical models of mechatronic system pilot - flight simulator. This method provides monitoring of quality and progress of the pilot's training.
Sea surface slope recovery through passive polarimetric imaging

The goal of this project is to develop a technique to recover sea surface slopes and orientations over a distributed area using a non-intrusive imaging polarimeter. The technique relies upon the incidence angle dependence of the Fresnel coefficients at the air/water interface. Different incidence angles will lead to different polarization states in the reflected light. This is an ill-posed problem, whose challenges include multi-valued slope solutions using the polarization measurements, resolving the impact of the sky incident radiance (and polarization of the radiance), treating upwelling light and unresolved waves. In this paper, we will discuss the optimization algorithm appropriate for this type of inverse problem, describe the sensor, and show high-resolution data from a field experiment.
Are old males still good males and can females tell the difference?

Sperm function generally declines with male age. Paradoxically, females of many species still choose to mate with old males rather than young males. Females choosing old mates may suffer reduced fertilization rates and an increased incidence of birth defects in offspring, lowering fitness which may in turn lead to conflict between the sexes. This apparent paradox has generated much interest from theorists, but whether this paradox presents in nature remains equivocal. Empirical studies have found mixed support for both a decline in fertility with male age and age‐based female mate preference. Here, we examine recent evidence for this paradox, identify confounding variables, highlight areas that deserve further investigation, and suggest avenues for future research.
Efficient support for data-intensive scientific workflows on geo-distributed clouds. (Support pour l'exécution efficace des workflows scientifiques à traitement intensif de données sur les cloud géo-distribués)

By 2020, the digital universe is expected to reach 44 zettabytes, as it is doubling every two years. Data come in the most diverse shapes and from the most geographically dispersed sources ever. The data explosion calls for applications capable of highlyscalable, distributed computation, and for infrastructures with massive storage and processing power to support them. These large-scale applications are often expressed as workflows that help defining data dependencies between their different components. More and more scientific workflows are executed on clouds, for they are a cost-effective alternative for intensive computing. Sometimes, workflows must be executed across multiple geodistributed cloud datacenters. It is either because these workflows exceed a single site capacity due to their huge storage and computation requirements, or because the data they process is scattered in different locations. Multisite workflow execution brings about several issues, for which little support has been developed: there is no common ile system for data transfer, inter-site latencies are high, and centralized management becomes a bottleneck. This thesis consists of three contributions towards bridging the gap between single- and multisite workflow execution. First, we present several design strategies to eficiently support the execution of workflow engines across multisite clouds, by reducing the cost of metadata operations. Then, we take one step further and explain how selective handling of metadata, classified by frequency of access, improves workflows performance in a multisite environment. Finally, we look into a different approach to optimize cloud workflow execution by studying some parameters to model and steer elastic scaling.
Veterans Affairs diabetes trial--corrections.

To the Editor: The Supplementary Appendix in which we provided additional information related to our article (Jan. 8 issue)1 contains some errors. In both Appendix 1 and Appendix 2, the heading “6 Year Event Rate” should have read “6-Year Event-free Rate. ” We thank the reader who detected this error. In addition, on further examination of the data on albuminuria from the Veterans Affairs Diabetes Trial, we found that the data set that we used to evaluate the progression of disease was constructed improperly. As a result, the rates of progression to microalbuminuria and macroalbuminuria were reported in Table 3 of .
Genotype-environment interaction and correlation in the analysis of human behavior.

Two issues that complicate behavioral genetic analyses are the interaction and correlation between genetic and environmental influences. In this report, the effects of genotype-envi ronment interaction and correlation on behavioral genetic studies (twin and adoption studies) are examined. The analysis suggests that genotype-environment interaction may bias twin study estimates of genetic and environmental influence, but need not affect adoption studies. On the other hand, genotype-environment correlation may affect both twin and adoption study estimates of genetic and environmental influence, the direction of the effect depending on the sign of the correlation. Finally, new tests of genotypeenvironment interaction and correlation, using adoption data, are proposed.
The one-dimensional organic–inorganic hybrid: catena-poly[bis[1-(3-ammoniopropyl)-1H-imidazolium] [[iodidoplumbate(II)]-tri-μ-iodido-plumbate(II)-tri-μ-iodido-[iodidoplumbate(II)]-di-μ-iodido]]

The organic–inorganic hybrid, {(C6H13N3)2[Pb3I10]}n, was obtained by the reaction of 1-(3-ammoniopropyl)imidazolium triiodide and PbI2 at room temperature. The structure contains one-dimensional {[Pb3I10]4−}n polymeric anions spreading parallel to [001], resulting from face–face–edge association of PbI6 distorted octahedra. One of the PbII cations is imposed at an inversion centre, whereas the second occupies a general position. N—H⋯I hydrogen bonds connect the organic cations and inorganic anions.
Lithium trapping in alloy forming electrodes and current collectors for lithium based batteries

Significant capacity losses are generally seen for batteries containing high-capacity lithium alloy forming anode materials such as silicon, tin and aluminium. These losses are generally ascribed to a combination of volume expansion effects and irreversible electrolyte reduction reactions. Here, it is shown, based on e.g. elemental analyses of cycled electrodes, that the capacity losses for tin nanorod and silicon composite electrodes in fact involve diffusion controlled trapping of lithium in the electrodes. While an analogous effect is also demonstrated for copper, nickel and titanium current collectors, boron-doped diamond is shown to function as an effective lithium diffusion barrier. The present findings indicate that the durability of lithium based batteries can be improved significantly via proper electrode design or regeneration of the used electrodes.
Endogenous Bank Credit and its Link to Housing in OECD Countries

The relevant economic literature frequently focuses on the impact of credit shocks on housing prices. The doctrine of the "New Consensus Macroeconomics" completely ignores bank credit. The "Great Recession," however, has highlighted the significance of bank credit. The purpose of this contribution is to revisit this important macroeconomic variable. We propose to endogenize the volume of bank credit by paying special attention to those variables that are related to the real estate market, which can be considered key to the evolution of bank credit. Our theoretical hypothesis is tested by means of a sample of 15 Organisation for Economic Co-operation and Development (OECD) economies from 1970 to 2011. We apply the cointegration technique for the latter purpose, which permits the modeling of the long-run equilibrium relationship and the dynamics of the short run, along with an error-correction term.
Type 2 histone deacetylases play a major role in the control of elicitor-induced cell death in tobacco

The cell death which characterizes the onset of the Hypersensitive Response (HR) is a very important weapon evolved by plants to block pathogen development. By the use of numerous plant/avirulent pathogen or plant/elicitor models, we have now obtained detailed signalling pathways allowing, after pathogen or elicitor perception, the control of the expression of specific sets of genes that contribute to cell death. However, our knowledge of the molecular actors involved in this process still remains limited. This is particularly true when regarding what happen in the nucleus. We recently reported that nuclear post-translational protein modifications are major processes that control cell death. Using the tobacco / cryptogein model, we showed that type 2 histone deacetylase activities, which act as negative regulators of cell death, depend on their phosphorylation status. In the present paper, we integrated all these results to propose a model depicting the putative nuclear signalling pathways controlling the establishment of cell death in tobacco in response to the cryptogein elicitor. This model highlights the role of the nuclear protein acetylation and phosphorylation in the establishment of plant defences.
The model of set theory generated by countably many generic reals

Abstract Adjoin, to a countable standard model M of Zermelo-Fraenkel set theory (ZF), a countable set A of independent Cohen generic reals. If one attempts to construct the model generated over M by these reals (not necessarily containing A as an element) as the intersection of all standard models that include M ∪ A, the resulting model fails to satisfy the power set axiom, although it does satisfy all the other ZF axioms. Thus, there is no smallest ZF model including M ∪ A, but there are minimal such models. These are classified by their sets of reals, and there is one minimal model whose set of reals is the smallest possible. We give several characterizations of this model, we determine which weak axioms of choice it satisfies, and we show that some better known models are forcing extensions of it.
Opposing Phenotypes in Dravet Syndrome Patient-Derived Induced Pluripotent Stem Cell Neurons: Can Everyone be Right?

Commentary Dravet syndrome (DS) is a catastrophic pediatric epileptic encephalopathy with cognitive, behavioral, and motor impairments, as well as a high risk of sudden unexpected death in epilepsy (SUDEP). The majority of DS patients have de novo mutations in SCN1A, the gene encoding the α subunit of the voltage-gated sodium channel NaV1.1, that result in haploinsufficiency (1). While the field has made key advances toward the goal of understanding how reduced expression of a major brain sodium channel gene results in hyperexcitability and network synchrony, critical gaps remain. Importantly, what is the best experimental model of DS and how do we interpret seemingly conflicting results between and within model systems? The first model of DS was developed by the Catterall group (2). Scn1a deletion in mice resulted in a severe seizure phenotype in heterozygous animals and was the first in vivo confirmation that Scn1a haploinsufficiency resulted in seizures. In this study, postnatal day (P) 14–16 Scn1a bipolar neurons—but not pyramidal neurons—had significantly reduced sodium current density, resulting in reduced interneuron firing frequency. Further work showed that Dlx1/2 Cremediated inactivation of Scn1a in parvalbumin-positive (PV+) interneurons phenocopied the severe seizures and SUDEP (3). Based on these results, it was hypothesized that pathophysiology in DS could be explained by disinhibition, or dysfunctional inhibitory circuits, that involve PV+ fast-spiking interneurons. Kearney and George extended this work to demonstrate, first, that genetic background is key to the severity of pathophysiology in DS mouse models, and, second, that changes in sodium current density are dependent on the stage of brain development in mice. They reported that sodium current density was elevated in Scn1a pyramidal neurons, but not GABAergic neurons, at P21–24. Moreover, pyramidal neurons isolated from Scn1a mice on the more phenotypically severe background strain displayed a larger increase in sodium current density than pyramidal neurons from the less severe strain. Scn1a P21–24 pyramidal neurons from either strain exhibited spontaneous firing and hyperexcitability. To add further complexity, more recent results demonstrated that, while interneuron hypoexcitability is observed in Scn1a mouse brain slices, it is not observed in Scn1a DS mice during spontaneous activities in vivo, suggesting that alternative mechanisms must be considered (4). Clearly, transgenic mouse models are critical to understanding the mechanisms of neurological disease. However, mice are not small humans, and there are important differences between mouse and human brain development (5, 6). To understand the mechanism of DS in human brain, a number of A Deleterious Nav1.1 Mutation Selectively Impairs Telencephalic Inhibitory Neurons Derived From Dravet Syndrome Patients.
Continuously Wavelength-Tunable Light Source With Constant-Power Output for Elimination of Residual Amplitude Modulation

A continuously wavelength-tunable light source with constant-power output is presented. The unwanted optical power modulation companying with the wavelength modulation of the distributed feedback laser diode by the injection current is successfully offset by the external amplitude modulation of electro-optic modulator. The optical power stability has been experimentally demonstrated better than 1.2% in a wavelength-tunable range of 0.876 cm-1. The background baseline appearing in the direct absorption spectrum can be removed with the new light source. Not only that, the spectral distortion of the second harmonic signal caused by residual-amplitude modulation can also be largely eliminated. Experimental results have shown that the new light source is more suitable to be served as a pure wavelength-tuning reference source for different modes of spectroscopic measurement.
Reconstruction-based Unsupervised Feature Selection: An Embedded Approach

Feature selection has been proven to be effective and efficient in preparing high-dimensional data for data mining and machine learning problems. Since real-world data is usually unlabeled, unsupervised feature selection has received increasing attention in recent years. Without label information, unsupervised feature selection needs alternative criteria to define feature relevance. Recently, data reconstruction error emerged as a new criterion for unsupervised feature selection, which defines feature relevance as the capability of features to approximate original data via a reconstruction function. Most existing algorithms in this family assume predefined, linear reconstruction functions. However, the reconstruction function should be data dependent and may not always be linear especially when the original data is high-dimensional. In this paper, we investigate how to learn the reconstruction function from the data automatically for unsupervised feature selection, and propose a novel reconstruction-based unsupervised feature selection framework REFS, which embeds the reconstruction function learning process into feature selection. Experiments on various types of real-world datasets demonstrate the effectiveness of the proposed framework REFS.
PDN characteristics of 3D-SiP with a wide-bus structure under 4k-IO operations

The 4096 bits wide-bus three-dimensional integration device using through-silicon-vias (TSVs) has been designed and fabricated as a demonstrator for power integrity such as power distribution network (PDN) impedance and simultaneous switching output (SSO) noise characteristics. Anti-resonance peak of total PDN impedance was extracted at around 80 MHz. This result was well coincident with maximum SSO noise frequency at around 75 MHz. Further, SSO noise reduction clocking named phase-shift clock has also been implemented to demonstrate the effectiveness as measurement basis.
Objective Ethics for Managing InformationTechnology

Businessmen have faced ethical dilemmas throughout history in many varying contexts. Today, chief information officers (CIOs) and information technology (IT) managers, in particular, face many ethical dilemmas from not only traditional business dilemmas, but also in managing IT. Traditional ethical issues of business, such as receiving gifts and promotional items from vendors, affect any manager in charge of purchases. In addition, IT managers must also make decisions with regards to technological issues such as information privacy, security, and accountability. IT facilitates action, both good and bad. This means that individuals can act good with far more efficiency and act bad with more malevolence. Actions that may not have been possible without IT now become issues because people have the means to do them. Managers need not only to adopt a moral code for themselves, but to encourage their employees to adopt a moral code or guard against and appropriately deal with behavior that violates that code. Identifying and understanding an objective ethical framework is critical for the rapidly changing nature of information technology. Without an appropriate ethical standard to guide choices and actions in ever more complex and subtle situations, IT managers and professionals will find choosing morally acceptable solutions ever more difficult, leading to dangers in the long-term success of the organization. CIOs need to cut through the haze of conflicting demands to make decisions for the benefit of the organization. CIOs also need to be confident that their employees will act in appropriate, non-arbitrary manners when making decisions. The need to research ethics with regard to IT has been demonstrated numerous times (Davison, 2000; Rose, 2006; Stewart & Segars, 2002), yet none has provided an objective standard for making decisions. While several research efforts have explored normative and applied ethical theories as applied to IT (Davison, 2000; Walsham, 1996; Wood-Harper, Corder, Wood, & Watson, 1996), none has questioned the underlying assumptions nor provided a compelling case for a nonarbitrary, objective moral code. Reviewing existing ethical theories should lead us to a standard that is applicable and beneficial to IT managers.