Miracle-dz/UniFAR-FTUs huggingface dataset:
Selected disorders of carbohydrate metabolism

Chapter 10. Myopathies affecting fuel and energy metabolism: Selected disorders of carbohydrate metabolism Definitions of entities The breakdown of glucose (from blood) or intracellular glycogen yields pyruvate that can then enter the TCA cycle (via acetyl CoA in aerobic conditions) or be converted to lactate under ischaemic conditions. Failure in any step in glycogenolysis or glycolysis eliminates or diminishes carbon flux, leading to a rapid decline in high energy phosphates for muscular work, and causing rapid muscle fatigue, cramping, and sometimes rhabdomyolysis. In those conditions where there is a muscle-specific gene product, the symptoms may be restricted to muscle but in other conditions, where there is a single gene encoding the enzyme activity, the effects can be more widespread. Inherited deficiencies in glyogenolytic/glycolytic enzymes are rare, and are usually autosomal recessive. They generally present as exercise intolerance, elevated serum creatine kinase, with rhabdomyolysis and exertion-induced myoglobinuria (3). One of the less well-understood features of this group of conditions relates to the marked variability in the severity of symptoms, even between individuals carrying the same mutations. This suggests that these enzyme deficiencies must be projected onto additional factors influencing muscle capabilities, whether genetic or environmental. In this respect, the anecdotal evidence of greater prevalence/severity in males may be relevant (24). In this chapter, we will consider three of the more common of these conditions: McArdle’s disease, Tarui’s disease, and debrancher deficiency. Some indication of the relative frequencies of these conditions may be gleaned from a survey conducted some years ago as part of the CARMEN programme, (http://carmen. liv.ac.uk). Whilst not intended to be exhaustive, these figures (Figure 1) report the total cases from twenty different muscle clinics within the European community, although these data will reflect local interests.
Testing Time Goal-Driven Requirements with Model Checking Techniques

In this paper we present the testing of time goal requirements by using model checking as an engine for test generation. The goal model that we use to capture the time requirements is an extension of KAOS. This goal model specifies the properties that the system must satisfy and how they are tested by using the model checker engine, UPPAAL. As an illustration of this proposal we take a particular study case based on GSM cell technology
W^{1,p} estimates for solutions to the Ginzburg-Landau equation with boundary data in H^{1/2}.

We consider complex-valued solutions uε of the Ginzburg–Landau on a smooth bounded simply connected domain Ω of R , N 2 (hereε is a parameter between 0 and1). We assume that uε = gε on∂Ω, where|gε|= 1 andgε is uniformly bounded inH(∂Ω). We also assume that the Ginzburg–Landau energy Eε(uε) is bounded byM0| log ε|, whereM0 is some given constant. We establish, for every 1 p < N/(N − 1), uniformW bounds for uε (independent of ε). These types of estimates play a central role in the asymptotic analysis ofuε asε → 0.  2001 Académie des sciences/Éditions scientifiques et médicales Elsevier SAS Estimées dans W1,p des solutions de l’équation de Ginzburg–Landau Résumé. Nous considérons des solutions à valeurs complexes uε de l’équation de Ginzburg–Landau sur un domaine borné régulier simplement connexe Ω de R , N 2 (ici ε désigne un paramètre strictement compris entre 0 et 1). Nous faisons l’hypothèse uε = gε sur ∂Ω, où gε est une application donnée telle que |gε| = 1 et gε est uniformement bornée dans H(∂Ω). Nous supposons de plus que l’énergie de Ginzburg–Landau Eε( ε) est bornée par M0| log ε|, oùM0 est une constante donnée. Nous montrons que pour tout p inférieur strictement àN/(N − 1), uε est bornée dansW, uniformément enε. Ce type de majoration joue un rôle crucial dans l’étude asymptotique de uε, lorsqueε → 0.  2001 Académie des sciences/Éditions scientifiques et médicales Elsevier SAS Version française abrégée SoientN un entier supérieur à deux, et Ω un domaine régulier, borné et simplement connexe de R . Nous considérons, pour un petit paramètre 0 < ε< 1, les applicationsuε : Ω →C solutions de l’équation de Note présentée par Haïm BREZIS. S0764-4442(01)02191-7/FLA  2001 Académie des sciences/Éditions scientifiques et médicales Elsevier SAS. Tous droits réservés 1069
RNN Based Uyghur Text Line Recognition and Its Training Strategy

Uyghur language is written in a modified Arabic script. Due to its cursive nature and the lack of enough labeled training samples, Uyghur document recognition is still a challenging problem. In this paper, we propose a new Recurrent Neural Network (RNN) based Uyghur text line recognition method combining Gated Recurrent Unit (GRU) and Restricted Boltzmann Machine (RBM) with pretraining mechanism. We also present a novel curriculum learning technique guided by sample distribution information. Experimental results on practical Uyghur printed document image dataset show that the proposed network architecture and training strategy not only achieve better recognition accuracy compared with traditional methods, but can accelerate the training speed as well.
Exploring Interactions and Perceptions of Kinetic Wearables

Jewelry and accessories have long been objects for decorating the human body; however they remain static and non-interactive. This work explores opportunities for accessory-like kinetic wearables and their association with individual style. We developed Kino, a kinetic accessory system which enables both aesthetic and functional clothing-specific design possibilities. We engaged both fashion designers and every-day users to unpack envisioned use cases and perceptions of the system. Participants viewed the devices not as gadgets but as companions due to their close proximity to the body. They envisioned a wide range of usage scenarios, highlighting the complexity of mobility in relation to personal style. We observe how mobility offers opportunities for fluid representations of self, which is unachievable though static clothing and accessories. We also outline how personalized aesthetics is important for the meaning making of novel on-body devices.
Understanding the Social Dynamics of Energy Regions—The Importance of Discourse Analysis

Regional initiatives pursuing self-sufficiency through the use of renewable energy sources (RESS-initiatives) aim at contributing to broader transitions towards more sustainable energy systems. As such, they have raised high expectations among local activists and are increasingly supported by diverse funding schemes such as national programs. How can the social dynamics entangled in these initiatives be understood and assessed? A discourse analytical perspective, such as the Argumentative Discourse Analysis developed by Hajer, can bring valuable insights in this regard. This approach highlights the formation of discourse coalitions and processes of discourse structuration and institutionalization. In order to illustrate my conceptual and methodological considerations, I present an analysis of discursive dynamics observed in the alpine district of Murau, Austria, where the vision of reaching ‘energy autarky’ by the year 2015 has influenced regional development plans since 2003. The chosen discourse analytical approach has been very helpful in guiding the analysis of this case. Specific local conditions can explain why certain visions gained discursive hegemony.
Parenting Behavior Dimensions and Child Psychopathology: Specificity, Task Dependency, and Interactive Relations

This study examined the specificity of relations between parent or caregiver behaviors and childhood internalizing and externalizing problems in a sample of 70 fourth-grade children (64% boys, M age = 9.7 years). Specificity was assessed via (a) unique effects, (b) differential effects, and (c) interactive effects. When measured as unique and differential effects, specificity was not found for warmth or psychological control but was found for caregiver's use of behavior control. Higher levels of behavior control were uniquely related to lower levels of externalizing problems and higher levels of internalizing problems; differential effects analyses indicated that higher levels of behavior control were related to decreases in the within-child difference in relative levels of level of internalizing versus externalizing problems. Interactive relations among the 3 parenting behavior dimensions also were identified. Although caregivers emphasized different parenting behavior dimensions across 2 separate caregiver-child interaction tasks, relations between parenting behavior dimensions and child psychopathology did not vary as a function of task. These findings indicate the importance of assessing and simultaneously analyzing multiple parenting behavior dimensions and multiple child psychopathology domains.
Coordinated intercellular calcium waves induced by noradrenaline in rat hepatocytes: dual control by gap junction permeability and agonist

Calcium‐mobilizing agonists induce intracellular Ca2+ concentration ([Ca2+]i) changes thought to trigger cellular responses. In connected cells, rises in [Ca2+]i can propagate from cell to cell as intercellular Ca2+ waves, the mechanisms of which are not elucidated. Using fura2‐loaded rat hepatocytes, we studied the mechanisms controlling coordination and intercellular propagation of noradrenaline‐induced Ca2+ signals. Gap junction blockade with 18 α‐glycyrrhetinic acid resulted in a loss of coordination between connected cells. We found that second messengers and [Ca2+]i rises in one hepatocyte cannot trigger Ca2+ responses in connected cells, suggesting that diffusion across gap junctions, while required for coordination, is not sufficient by itself for the propagation of intercellular Ca2+ waves. In addition, our experiments revealed functional differences between noradrenaline‐induced Ca2+ signals in connected hepatocytes. These results demonstrate that intercellular Ca2+ signals in multicellular systems of rat hepatocytes are propagated and highly organized through complex mechanisms involving at least three factors. First, gap junction coupling ensures coordination of [Ca2+]i oscillations between the different cells; second, the presence of hormone at each hepatocyte is required for cell‐cell Ca2+ signal propagation; and third, functional differences between adjacent connected hepatocytes could allow a ‘pacemaker‐like’ intercellular spread of Ca2+ waves.
Technical and Human Issues in Computer-Based Systems Security

Computer systems and internet are becoming pervasive in our everyday life. Being online brings the consequence that such systems are prone to malicious attack. This vulnerability, along with our reliance on these systems, implies that it is important for us to do our best in securing them to ensure their proper functioning. In this paper, we are trying to tackle the security issues from both technical and human perspectives. From this dual standpoint, we hope to obtain a better understanding on how computer attacks are performed, including how to gain illicit access, the types of attacks, as well as the potential damage that they can cause. We also uncover sociological and psychological traits of the attackers, including their community, taxonomy, motives and work ethics. This survey paper will not provide a concrete solution on how to secure computer systems, but it highlights the socio-technical approach that we must take in order to obtain that goal.
Linear quadratic Gaussian control with quantized feedback

This paper studies a generalized linear quadratic Gaussian (LQG) control problem subject to the constraint that the feedback signal is quantized with a fixed bit rate. We show that state feedback control, state estimation and quantization can not be fully separated in general. Only a weak separation principle holds which converts the quantized LQG control problem into a quantized state estimation problem. We show that under high resolution quantization and the assumption that the output dimension does not exceed the input dimension, full separation of state feedback control, state estimation and quantization can be achieved.
Ocean surface current measurement using pencil-beam rotating scatterometer

In order to measure the ocean surface current vector in a direct way, an interferometric method using pencil-beam scatterometer is proposed and the parameters of the traditional scatterometer are modified. What's more, the measurement principle and correlation model are introduced in this paper. The simulation results show that the Std of ocean surface current speed in both along-track and cross-track direction can be smaller than 10cm/s when the wind speed is larger than 4m/s. And the effective swath is greater than 445km when the wind speed is larger than 7m/s. The Kpc of the modified scatterometer is better than that of the tradition scatterometer.
Neuropathy: The Crystal Ball for Cardiovascular Disease?

Cardiovascular disease (CVD) is a major cause of death in patients with type 2 diabetes. Unclear, however, is the effect of intensive therapy in reducing the development of cardiovascular complications. The UK Prospective Diabetes Study (UKPDS), involving patients with newly diagnosed disease, showed a nonsignificant trend in the reduction of rates for myocardial infarction (MI) (1). However, data 10 years after cessation of the trial showed a 15% reduction in the risk of MI for those in the original intensive therapy group (2). This benefit from early intensive therapy persisted despite the fact that the within-trial differences for A1C between the intensive and conventional therapy groups were lost within 1 year of completion of the trial. The sustained benefit from early aggressive treatment is referred to as the legacy effect or metabolic memory. The question of more intensive therapy and reduction in cardiovascular complications was addressed for people with type 2 diabetes of long duration in three other studies (i.e., the Action to Control Cardiovascular Risk in Diabetes [ACCORD] [3], the Action in Diabetes and Vascular Disease: Preterax and Diamicron Modified Release Controlled Evaluation [ADVANCE] [4], the Veterans Affairs Diabetes Trial [VADT] [5]). Although the three studies utilized different patient cohorts, with varying durations of diabetes (ACCORD, 10 years; VADT, 11.5 years; and ADVANCE, 8 years) and had different treatment regimens, the results of these trials indicated that intensive glucose control did not reduce CVD events. In fact, the ACCORD trial was terminated early because an increased rate of mortality was found to be associated with intensive control of hyperglycemia (6). A higher rate of mortality for those on intensive treatment was not, however, found in ADVANCE and VADT. Recent articles from the ACCORD trial, by Pop-Busui et al. (7) and Calles Escandon et …
Comparing and contrasting adaptive middleware support in wide-area and embedded distributed object applications

The Quality Objects (QuO) middleware is a set of extensions to standard distributed object computing middleware that is used to control and adapt the quality of service in a number of distributed application environments, from wide-area to embedded distributed applications. This paper compares and contrasts the characteristics of key use cases and the variations in QuO implementations that have emerged to support them. We present these variations in the context of several actual applications being developed using the QuO middleware.
Percutaneous ablation therapy for hepatocellular carcinomas.

HCC is one of the 10 most common forms of cancer in the world. Men are affected more frequently than women (3-8:1 ratio). HCC is frequent in Southeast Asia and sub-Saharan Africa (30 cases per 100,000 men per year) and common in both Europe and North America (two cases per 100,000 men per year) [2]. The survival of patients with asymptomatic HCC varies. However, the median survival ranges from 3-6 months after the onset of symptoms. It is estimated that HCC causes 1 ,250,000 deaths per year worldwide [3]. Most patients who develop HCC have chronic liver disease caused by hepatitis B or hepatitis C or have ingested hepatotoxic substances (e.g., ethanol and aflatoxin) for a long time. Screening of these highrisk patients every 6 months to 1 year with sonographic examination of the liver and measurement of the serum a-fetoprotein levels (70% sensitivity for HCC) is recommended. Two thirds of the carcinomas detected in this fashion are unifocal and less than 3 cm in diameter. The sensitivity of imaging studies, including sonography, CT, and angiography, to detect small HCCs (23 cm) is 80-85% [3]. CT-arterioportography, MR imaging, and positron emission tomography scanning may improve HCC detection. Overall, the staging of a lesion suspected of being an HCC in a cirrhotic liver may be imprecise. On gross examination, HCC may be unifocal (50%), multifocal (25%), or diffuse (25%). On microscopic examination, common histologic forms of HCC must be distinguished from fibrolamellar carcinoma. The fibrolamellar type develops in young patients with normal livers and has a better prognosis. Staging of HCC includes evaluation of the lesion(s) and liver function. The size, number, and location of lesion(s) in the liver, vascular and biliary tumor ingrowth, regional lymphatic involvement, and distant metastases are the parameters considered. Child’s classification is used to categorize liver function in HCC patients [4].
Extra-motor abnormalities in amyotrophic lateral sclerosis: another layer of heterogeneity

ABSTRACT Introduction: Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disease defined by the presence of muscle weakness. The motor features of disease are heterogeneous in site of onset and progression. There are also extra-motor features in some patients. The genetic basis for extra-motor features is uncertain. The heterogeneity of ALS is an issue for clinical trials. Areas covered: This paper reviews the range and prevalence of extra-motor features associated with ALS, and highlights the current information about genetic associations with extra-motor features. Expert commentary: There are extra-motor features of ALS, but these are not found in all patients. The most common is cognitive abnormality. More data is required to ascertain whether extra-motor features arise with progression of disease. Extra-motor features are reported in patients with a range of causative genetic mutations, but are not found in all patients with these mutations. Further studies are required of the heterogeneity of ALS, and genotype/phenotype correlations are required, taking note of extra-motor features.
The Subjective Sensation of Synchrony: An Experimental Study

People performing actions together have a natural tendency to synchronize their behavior. Consistently, people doing a task together build internal representations not only of their actions and goals, but also of the other people performing the task. However, little is known about which are the behavioral mechanisms and the psychological factors affecting the subjective sensation of synchrony, or “connecting” with someone else. In this work, we sought to find which factors induce the subjective sensation of synchrony, combining motion capture data and psychological measures. Our results show that the subjective sensation of synchrony is affected by performance quality together with task category, and time. Psychological factors such as empathy and negative subjective affects also correlate with the subjective sensation of synchrony. However, when people estimate synchrony as seen from a third person perspective, their psychological factors do not affect the accuracy of the estimation. We suggest that to feel this sensation it is necessary to, first, have a good joint performance and, second, to assume the existence of an attention monitoring mechanism that reports that the attention of both participants (self and other) is focused on the task.
Mathematical corrections for bacterial loss in pharmacodynamic in vitro dilution models

In vitro dilution models are used to simulate in vivo drug concentration-time profiles and thus to study the effects of various antibiotic concentrations on the bacteria investigated. The major disadvantage of these models is permanent dilution of the bacterial culture, which falsifies the resulting kill curves. Known equations, which usually correct bacterial loss by simple first-order kinetics, do not take into account special test conditions, such as variable elimination rate constants, exceptionally long periods of investigation, or formation of biofilms. In the present investigation, we examined the validity of these equations with regard to the test conditions mentioned. We simulated the concentration-time curves resulting from continuous infusion of 1,000 mg of meropenem with steady-state levels of 2.5, 5.0, and 7.5 micrograms/ml in an in vitro dilution model. The resulting kill curves were compared with the kill curves obtained from incubation of bacteria in an undiluted system with meropenem at constant concentrations corresponding to the above-mentioned steady-state levels. Comparison of the matching kill curves showed that the common corrections, which do not consider the formation of biofilms in the compartments, partly overestimated the effect of bacterial dilution. We defined a factor, f, as an extension to the known equations which compensates for the effect of biofilms. Another extension was developed to allow the investigation of variable elimination rate constants. With the help of these extended mathematical corrections, we were able to fit the kill curves resulting from the in vitro dilution model exactly to the kill curves given by an undiluted system.
Clinical and radiologic evidence of the epidural plica mediana dorsalis.

The presence of a connective tissue band that divides the epidural space at the dorsal midline is widely accepted (1-5). Its clinical relevance is related to an increase in the incidence of dural puncture and the possibility of unilateral analgesia, as well as to greater difficulty in introducing an epidural catheter. In the present paper we report a case in which dural puncture and unilateral analgesia, together with radiologic imaging, strongly suggested the presence of this midline band.