Miracle-dz/UniFAR-FTUs huggingface dataset:
Natural History, Outcome, and Treatment Efficacy in Children and Adults with Glutaryl-CoA Dehydrogenase Deficiency

Glutaryl-CoA dehydrogenase (GCDH) deficiency is a rare inborn disorder of l-lysine, l-hydroxylysine, and l-tryptophan metabolism complicated by striatal damage during acute encephalopathic crises. Three decades after its description, the natural history and how to treat this disorder are still incompletely understood. To study which variables influenced the outcome, we conducted an international cross-sectional study in 35 metabolic centers. Our main outcome measures were onset and neurologic sequelae of acute encephalopathic crises. A total of 279 patients (160 male, 119 female) were included who were diagnosed clinically after clinical presentation (n = 218) or presymptomatically by neonatal screening (n = 23), high-risk screening (n = 24), or macrocephaly (n = 14). Most symptomatic patients (n = 185) had encephalopathic crises, characteristically resulting in bilateral striatal damage and dystonia, secondary complications, and reduced life expectancy. First crises usually occurred during infancy (95% by age 2 y); the oldest age at which a repeat crisis was reported was 70 mo. In a few patients, neurologic disease developed without a reported crisis. Differences in the diagnostic criteria and therapeutic protocols for patients with GCDH deficiency resulted in a huge variability in the outcome worldwide. Recursive partitioning demonstrated that timely diagnosis in neurologically asymptomatic patients followed by treatment with l-carnitine and a lysine-restricted diet was the best predictor of good outcome, whereas treatment efficacy was low in patients diagnosed after the onset of neurologic disease. Notably, the biochemical phenotype did not predict the clinical phenotype. Our study proves GCDH deficiency to be a treatable disorder and a good candidate for neonatal screening.
Copyright 2009 by Human Factors and Ergonomics Society, Inc. All rights reserved. 10.1518/107118109X12524444080675

Decrement of visual attention to repeatedly exposed warning labels and signs has been discussed in the warning literature without much empirical research support. The present research examined whether habituation, dishabituation, and recovery of habituation occur to visually presented warnings. Participants (N=72) were presented to a long sequence of repeated visual warnings (40 warnings presented 8 times = 320 initial trials) in a particular format (ANSI or OSHA type formats), followed by 5 warnings manipulated either in the same or different format. Five more warnings in same format as in the initial trials were presented to end the sequence. Measured were participants’ ratings of perceived alertness to each warning. Findings showed a decrease in ratings from beginning to end of the initial 320 trials, indicative of habituation. Dishabituation was observed with higher ratings when the warning format changed. Evidence of habituation recovery of lowered ratings was observed upon return to the previously habituated (initial trials) format after a set of different formatted warnings. Implications for formalized standards and guidelines, which recommend an unchanging, relatively constant format, are discussed.
In vivo cardiac role of migfilin during experimental pressure overload.

AIMS Increased myocardial wall strain triggers the cardiac hypertrophic response by increasing cardiomyocyte size, reprogramming gene expression, and enhancing contractile protein synthesis. The LIM protein, migfilin, is a cytoskeleton-associated protein that was found to translocate in vitro into the nucleus in a Ca(2+)-dependent manner, where it co-activates the pivotal cardiac transcription factor Csx/Nkx2.5. However, the in vivo role of migfilin in cardiac function and stress response is unclear. METHODS AND RESULTS To define the role of migfilin in cardiac hypertrophy, we induced hypertension by transverse aortic constriction (TAC) and compared cardiac morphology and function of migfilin knockout (KO) with wild-type (WT) hearts. Heart size and myocardial contractility were comparable in untreated migfilin KO and WT hearts, but migfilin-null hearts presented a reduced extent of hypertrophic remodelling in response to chronic hypertensile stress. Migfilin KO mice maintained their cardiac function for a longer time period compared with WT mice, which presented extensive fibrosis and death due to heart failure. Migfilin translocated into the nucleus of TAC-treated cardiomyocytes, and migfilin KO hearts showed reduced Akt activation during the early response to pressure overload. CONCLUSIONS Our findings indicate an important role of migfilin in the regulation of cardiac hypertrophy upon experimental TAC.
Submillimeter Mapping and Analysis of Cold Dust Condensations in the Orion M42 Star-forming Complex

We present here the continuum submillimeter maps of the molecular cloud around the M42 Nebula in the Orion region. These have been obtained in four wavelength bands (200, 260, 360, and 580 μm) with the Programme National d'Observations Submillimétriques 2 m balloon-borne telescope. The area covered is 7 pc wide (50' at a distance of 470 pc) with a spatial resolution of about 0.4 pc. Thanks to the high sensitivity to faint surface brightness gradients, we have found several cold condensations, with temperatures ranging from 12 to 17 K, within 3 pc of the dense ridge. The statistical analysis of the temperature and spectral index spatial distribution shows an evidence of an inverse correlation between these two parameters. Being invisible in the IRAS 100 μm survey, some cold clouds are likely to be the seeds for future star formation activity going on in the complex. We estimate their masses, and we show that two of them have masses higher than their Jeans masses and may be gravitationally unstable.
The Transcriptional Activator LdtR from ‘Candidatus Liberibacter asiaticus’ Mediates Osmotic Stress Tolerance

The causal agent of Huanglongbing disease, ‘Candidatus Liberibacter asiaticus’, is a non-culturable, gram negative, phloem-limited α-proteobacterium. Current methods to control the spread of this disease are still limited to the removal and destruction of infected trees. In this study, we identified and characterized a regulon from ‘Ca. L. asiaticus’ involved in cell wall remodeling, that contains a member of the MarR family of transcriptional regulators (ldtR), and a predicted L,D-transpeptidase (ldtP). In Sinorhizobium meliloti, mutation of ldtR resulted in morphological changes (shortened rod-type phenotype) and reduced tolerance to osmotic stress. A biochemical approach was taken to identify small molecules that modulate LdtR activity. The LdtR ligands identified by thermal shift assays were validated using DNA binding methods. The biological impact of LdtR inactivation by the small molecules was then examined in Sinorhizobium meliloti and Liberibacter crescens, where a shortened-rod phenotype was induced by growth in presence of the ligands. A new method was also developed to examine the effects of small molecules on the viability of ‘Ca. Liberibacter asiaticus’, using shoots from HLB-infected orange trees. Decreased expression of ldtRLas and ldtPLas was observed in samples taken from HLB-infected shoots after 6 h of incubation with the LdtR ligands. These results provide strong proof of concept for the use of small molecules that target LdtR, as a potential treatment option for Huanglongbing disease.
DieHard: Reliable Scheduling to Survive Correlated Failures in Cloud Data Centers

In large scale data centers, a single fault can lead to correlated failures of several physical machines and the tasks running on them, simultaneously. Such correlated failures can severely damage the reliability of a service or a job. This paper models the impact of stochastic and correlated failures on job reliability in a data center. We focus on correlated failures caused by power outages or failures of network components, on jobs running multiple replicas of identical tasks. We present a statistical reliability model and an approximation technique for computing a job's reliability in the presence of correlated failures. In addition, we address the problem of scheduling a job with reliability constraints. We formulate the scheduling problem as an optimization problem, with the aim being to achieve the desired reliability with the minimum number of extra tasks. We present a scheduling algorithm that approximates the minimum number of required tasks and a placement to achieve a desired job reliability. We study the efficiency of our algorithm using an analytical approach and by simulating a cluster with different failure sources and reliabilities. The results show that the algorithm can effectively approximate the minimum number of extra tasks required to achieve the job's reliability.
An Audio-Based Sports Video Segmentation and Event Detection Algorithm

In this paper, we present an audio-based event detection algorithm shown to be effective when applied to Soccer video. The main benefit of this approach is the ability to recognise patterns that display high levels of crowd response correlated to key events. The soundtrack from a Soccer sequence is first parameterised using Mel-frequency Cepstral coefficients. It is then segmented into homogenous components using a windowing algorithm with a decision process based on Bayesian model selection. This decision process eliminated the need for defining a heuristic set of rules for segmentation. Each audio segment is then labelled using a series of Hidden Markov model (HMM) classifiers, each a representation of one of 6 predefined semantic content classes found in Soccer video. Exciting events are identified as those segments belonging to a crowd cheering class. Experimentation indicated that the algorithm was more effective for classifying crowd response when compared to traditional model-based segmentation and classification techniques.
Scalable clustering of news search results

In this paper, we present a system for clustering the search results of a news search engine. The news search interface includes the relevant news articles to a given query organized in terms of related news stories. Here each cluster corresponds to a news story and the news articles are clustered into stories. We present a system that clusters the search results of a news search system in a fast and scalable manner. The clustering system is organized into three components including offline clustering, incremental clustering and realtime clustering. We propose novel techniques for clustering the search results in realtime. The experimental results with large collections of news documents reveal that our system is both scalable and also achieves good accuracy in clustering the news search results.
Research on Pattern Representation Method in Semi-supervised Semantic Relation Extraction Based on Bootstrapping

Semantic relation extraction is an important part of information extraction, it has application value in the automatic question answering system, retrieval system, ontology learning, semantic web annotation, and many other areas. Pattern representation method is context pattern in previous semi-Supervised semantic relation extraction based on bootstrapping, but it did not consider the role of the keywords in the semantic relation. This paper presents an improved context pattern, which has a stronger semantic expressiveness, which is used to extract semantic relations and makes the semantic relation extraction more accurate. First of all, the sentence context pattern is obtained by lexical analysis. Then, the syntax tree model is obtained by syntactic analysis, calculate words weight using the syntax tree pattern. Finally, extract semantic relations using semi-Supervised machine learning method based on bootstrapping. The experimental results show that this method can effectively extract the semantic relations.
The insulin resistance syndrome: physiological considerations

The insulin resistance syndrome, also referred to as the ‘metabolic syndrome’ or ‘syndrome X’, is associated with a primary cellular defect in insulin action (insulin resistance) and a compensatory increase in insulin secretion. The combination of insulin resistance and subsequent hyperinsulinaemia causes a number of metabolic and cardiovascular changes that result in a syndrome typically characterised by type 2 diabetes, obesity, dyslipidaemia, coronary artery disease and hypertension. Moreover, disturbances in sleep (sleep apnoea) and ovarian dysfunction are also characterised by insulin resistance. The pathophysiological basis for these disturbances reflects the impact of variable genetic and environmental influences. At a molecular level, insulin resistance involves defects of insulin signalling such as reduced insulin receptor tyrosine kinase activity and reduced post-receptor phosphorylation steps that impinge on metabolic and vascular effects of insulin.
Learning and Extracting Initial Mealy Automata with a Modular Neural Network Model

A hybrid recurrent neural network is shown to learn small initial mealy machines (that can be thought of as translation machines translating input strings to corresponding output strings, as opposed to recognition automata that classify strings as either grammatical or nongrammatical) from positive training samples. A well-trained neural net is then presented once again with the training set and a Kohonen self-organizing map with the "star" topology of neurons is used to quantize recurrent network state space into distinct regions representing corresponding states of a mealy machine being learned. This enables us to extract the learned mealy machine from the trained recurrent network. One neural network (Kohonen self-organizing map) is used to extract meaningful information from another network (recurrent neural network).
Decay in Two-Dimensional String Theory

We consider unstable D0-branes of two dimensional string theory, described by the boundary state of Zamolodchikov and Zamolodchikov [hep-th/0101152] multiplied by the Neumann boundary state for the time coordinate t. In the dual description in terms of the c = 1 matrix model, this D0-brane is described by a matrix eigenvalue on top of the upside down harmonic oscillator potential. As suggested by McGreevy and Verlinde [hepth/0304224], an eigenvalue rolling down the potential describes D-brane decay. As the eigenvalue moves down the potential to the asymptotic region it can be described as a free relativistic fermion. Bosonizing this fermion we get a description of the state in terms of a coherent state of the tachyon field in the asymptotic region, up to a non-local linear field redefinition by an energy-dependent phase. This coherent state agrees with the exponential of the closed string one-point function on a disk with Sen’s marginal boundary interaction for t which describes D0-brane decay.
Different approaches to corporate reporting regulation: How jurisdictions differ and why

Abstract This paper discusses differences in countries’ approaches to reporting regulation and explores the reasons why they exist in the first place as well as why they are likely to persist. I first delineate various regulatory choices and discuss the trade‐offs associated with these choices. I also provide a framework that can explain differences in corporate reporting regulation. Next, I present descriptive and stylised evidence on regulatory and institutional differences across countries. There are robust institutional clusters around the world. I discuss that these clusters are likely to persist given the complementarities among countries’ institutions. An important implication of this finding is that reporting practices are unlikely to converge globally, despite efforts to harmonise reporting standards. Convergence of reporting practices is also unlikely due to persistent enforcement differences around the world. Given an ostensibly strong demand for convergence in reporting practices for globally operating firms, I propose a different way forward that does not require convergence of reporting regulation and enforcement across countries. The idea is to create a ‘global player segment’, in which member firms play by the same reporting rules and face the same enforcement. Such a segment could be created and administered by a supra‐national body like IOSCO.
Exploring the efficiency of parallel bacteria foraging metaheuristics for job shop scheduling problem optimization

This paper suggest a model of parallel computation based on Bacteria Foraging metaheuristics for Job Shop Scheduling optimization problem. The parallel model is verified experimentally by Hybrid (MPI + OpenMP) programming implementations, run on heterogeneous computer cluster. The experimental study comprises parallel performance evaluation, parallel algorithm scalability analysis, profiling and evaluation of solution quality.
Compressed Sensing for Wireless Communications: Useful Tips and Tricks

As a paradigm to recover the sparse signal from a small set of linear measurements, compressed sensing (CS) has stimulated a great deal of interest in recent years. In order to apply the CS techniques to wireless communication systems, there are a number of things to know and also several issues to be considered. However, it is not easy to grasp simple and easy answers to the issues raised while carrying out research on CS. The main purpose of this paper is to provide essential knowledge and useful tips and tricks that wireless communication researchers need to know when designing CS-based wireless systems. First, we present an overview of the CS technique, including basic setup, sparse recovery algorithm, and performance guarantee. Then, we describe three distinct subproblems of CS, viz., sparse estimation, support identification, and sparse detection, with various wireless communication applications. We also address main issues encountered in the design of CS-based wireless communication systems. These include potentials and limitations of CS techniques, useful tips that one should be aware of, subtle points that one should pay attention to, and some prior knowledge to achieve better performance. Our hope is that this paper will be a useful guide for wireless communication researchers and even non-experts to get the gist of CS techniques.
DWDM performance of a packaged reconfigurable optical add-drop multiplexer subsystem supporting modular systems growth

We describe dense wavelength-division-multiplexing (DWDM) operation of a reconfigurable optical add-drop multiplexer subsystem module employing silica waveguide technology that is suitable for use with both fixed-wavelength and wavelength-selectable add-drop transceivers. We illustrate its use in providing modular growth in high channel-count DWDM systems.
Relationship between Diversity and Perfomance of Multiple Classifiers for Decision Support

The paper presents the investigation and implementation of the relationship between diversity and the performance of multiple classifiers on classification accuracy. The study is critical as to build classifiers that are strong and can generalize better. The parameters of the neural network within the committee were varied to induce diversity; hence structural diversity is the focus for this study. The hidden nodes and the activation function are the parameters that were varied. The diversity measures that were adopted from ecology such as Shannon and Simpson were used to quantify diversity. Genetic algorithm is used to find the optimal ensemble by using the accuracy as the cost function. The results observed shows that there is a relationship between structural diversity and accuracy. It is observed that the classification accuracy of an ensemble increases as the diversity increases. There was an increase of 3%-6% in the classification accuracy.
Acute Meteorite Dust Exposure and Pulmonary Inflammation - Implications for Human Space Exploration

FOR HUMAN SPACE EXPLORATION. A.D. Harrington, F.M. McCubbin, J. Kaur, A. Smirnov, K. Galdanes, M.A.A. Schoonen, L.C. Chen, S.E. Tsirka, and T. Gordon. NASA Johnson Space Center, Mail Code XI2, Houston, TX 77058 (Andrea.D.Harrington@NASA.gov). Deptartment of Environmental Medicine, New York University School of Medicine, Tuxedo, NY 10987. Deptartment of Geosciences, Stony Brook University, Stony Brook, NY 11794. Geology Department, Lone Star College, Kingwood, TX 77339. Environmental Sciences Department, Brookhaven National Laboratory, Upton, NY 11973. Pharmacological Sciences, Stony Brook University, Stony Brook, NY 11794.
RP-HPLC METHOD FOR SIMULTANEOUS ESTIMATION OF LOSARTAN, HYDROCHLOROTHIAZIDE AND AMLODIPINE IN TABLET DOSAGE FORM

Objective: The present study was designed with an objective of a simple, fast, precise, selective and accurate RP-HPLC method was developed and validated for the simultaneous determination of Losartan, Hydrochlorothiazide and Amlodipine from bulk and formulations. Methods: Reverse phase high performance liquid chromatography (RP-HPLC) method was developed and validated for rapid analysis of simultaneous determination of Losartan, Hydrochlorothiazide and Amlodipine. The separation of these three drugs was achieved on a Hypersil Gold column (250 mm X 4.6 mm, 5 µ) as stationary phase with a mobile phase consisting of methanol: water in the ratio of 95:5percent v/v at a flow rate of 0.8 mL/min and UV detection at 230 nm. Results: The retention times were observed to be 2.850, 3.875 and 7.333 minutes for Losartan, Hydrochlorothiazide and Amlodipine, respectively. The method was statistically validated for linearity, recovery, limit of detection, limit of quantification, accuracy and precision. Conclusion: The method was successfully applied for analysis of combined dose tablet formulation containing Losartan, Hydrochlorothiazide and Amlodipine. Keyword s: Amlodipine, hydrochlorothiazide, losartan, reverse phase high performance liquid chromatography.
Summary of the recommendations on sexual dysfunctions in women.

INTRODUCTION Women's sexual dysfunctions include persistent or recurrent disorders of sexual interest/desire, disorders of subjective and genital arousal, orgasm disorder, pain and difficulty with attempted or completed intercourse. There are limited recommendation/guideline documents pertaining to the diagnosis and treatment of women's sexual dysfunctions. AIM To provide recommendations/guidelines concerning state-of-the-art knowledge for the clinical management of women's sexual dysfunctions. METHODS An International Consultation in collaboration with the major sexual medicine associations assembled over 200 multidisciplinary experts from 60 countries into 17 committees. Committee members established specific objectives and scopes for various sexual medicine topics. The recommendations concerning state-of-the-art knowledge in the respective sexual medicine topic represent the opinion of experts from five continents developed in a process over a 2-year period. Ten experts from four countries compiled the Recommendations on Sexual Dysfunctions in Women. MAIN OUTCOME MEASURE Expert opinion was based on grading of evidence-based medical literature, widespread internal committee discussion, public presentation and debate. RESULTS Recommendations and guidelines concerning the various sexual dysfunctions were updated. A comprehensive assessment of medical, sexual and psychosocial history is recommended for diagnosis and management planning. Indications for general and focused pelvic genital examination and laboratory testing are included. Recently revised definitions for sexual desire/interest disorder, arousal disorders (genital, subjective, combined and persistent), orgasm disorder, dyspareunia and vaginismus are presented. An evidence-based approach to management is provided. CONCLUSIONS There is a need for more research and scientific reporting, re-assessment, and management of women's sexual dysfunction including long-term outcome studies.