Miracle-dz/UniFAR-FTUs huggingface dataset:
A family with Wagner syndrome with uveitis and a new versican mutation

Purpose To report the clinical and molecular findings of a kindred with Wagner syndrome (WS) revealed by intraocular inflammatory features. Methods Eight available family members underwent complete ophthalmologic examination, including laser flare cell meter measurements. Collagen, type II, alpha 1, versican (VCAN), frizzled family receptor 4, low density lipoprotein receptor-related protein 5, tetraspanin 12, and Norrie disease (pseudoglioma) genes were screened with direct sequencing. Results The index case was initially referred for unexplained severe and chronic postoperative bilateral uveitis following a standard cataract surgery procedure. Clinical examination of the proband revealed an optically empty vitreous with avascular vitreous strands and veils, features highly suggestive of WS. The systematic familial ophthalmologic examination identified three additional unsuspected affected family members who also presented with the WS phenotype, including uveitis for one of them. We identified a novel c.4004–6T>A nucleotide substitution at the acceptor splice site of intron 7 of the VCAN gene that segregated with the disease phenotype. Conclusions We present a family with WS with typical WS features and intraocular inflammatory manifestations associated with a novel splice site VCAN mutation. Beyond the structural role in the retinal-vitreous architecture, versican is also emerging as a pivotal mediator of the inflammatory response, supporting uveitis predisposition as a clinical manifestation of WS.
Defining fundamentals and meeting expectations: Trends in LIS education in Australia

The current state of Australian library and information studies (LIS) education is characterised by unresolved tensions, only some of which have been accommodated. Our objective in this paper is to ascertain current trends in LIS education in Australia, rather than to compile an inventory of current programs and courses. We focus on a framework of tensions whose accommodation by the Australian LIS community form the trends. Australia has no central data source for LIS education comparable to the statistics compiled by ALISE (the Association of Library and Information Science Educators) about LIS schools in Canada and the United States. Consequently our evidence comes from a range of sources, with varying degrees of validity. Some of it is based on our experience of LIS education in Australia, while other evidence is taken from material produced by the Australian Library and Information Association (ALIA) and other sources.
Structural Semigraphoids 1

The new approach to mathematical description of structures of stochastic conditional independence from Studen y, 1993a-c] is related to the classical approach by means of the notion of structural semigraphoid. It is shown how to realize the corresponding deduc-tive mechanism to infer probabilistically valid consequences of input information about CI-structure, called facial implication. In the case of 4 attributes (random variables), structural semigraphoids are characterized in terms of inference rules.
An On-Line Calibration Monitoring Technique Using Support Vector Regression and Principal Component Analysis

In a nuclear power plant (NPP), periodic sensor calibrations are required to assure sensors are operating correctly. However, only a few faulty sensors are found to be calibrated. For the safe operation of an NPP and the reduction of unnecessary calibration, on-line calibration monitoring is needed. In this study, principal component-based auto-associative support vector regression (AASVR) is proposed for the sensor signal validation of the NPP. This paper describes the design of an AASVR-based sensor validation system for a power generation system. Response surface methodology (RSM) is employed to efficiently determine the optimal values of SVR hyperparameters. The proposed PCSVR model was confirmed with actual plant data of Kori Nuclear Power Plant Unit 3 and compared with the AANN model. The results show that the accuracy and sensitivity of the model were very competitive. Hence, this model can be used to monitor sensor performance.
Monetary and Fiscal Policy with Flexible Exchange Rates

If price decisions are taken neither continuously nor in perfect synchronization, the process of adjustment of all prices to a new nominal level will imply temporary movements in relative prices. It might then well be that, to avoid these movements in relative prices, each price setter will want to move his own price slowly compared to others. The result will be a slow movement of all prices to their new nominal level, and substantial inertia of the price level. This paper formalizes this intuitive argument and reaches four main conclusions: (1) Even small departures from perfect synchronization can generate substantial price level inertia. (2) If price decisions are desynchronized, even anticipated movements in money will usually have an effect on economic activity. It is however possible to find paths of money deceleration which reduce inflation at no cost in output. (3) Price desynchronization has implications for relative price movements as well as for the price level. Goods early in the chain of production have more price and profit variability than goods further down the chain. (4) Price inertia, if it is due to price desynchronization, may be difficult to remove. It may well be that, given the timing decisions of others, no agent has an incentive to change his own timing decision: the time structure of price desynchronization may be stable.
The Improved Pagerank in Web Crawler

Pagerank is an algorithm for rating web pages. It introduces the relationship of citation in academic papers to evaluate the web page's authority. It gives the same weight to all edges and ignores the relevancy of web pages to the topic, resulting in a problem of topic-drift. On the analysis of several pagerank algorithms, an improved pagerank based upon thematic segments is proposed. In this algorithm, a web page is divided into several blocks by Html document's structure and the most weight is given to linkages in the block that is most relevant to given topic. Moreover, the visited outlinks are regarded as feedback to modify blocks' relevancy The experiment on Web crawler shows that the new algorithm has some effect on resolving the problem of topic-drift.
Exports, growth and causality: new evidence on Italy, 1863-2004

This paper investigates the causal relationship between real export and real GDP in Italy from 1863 to 2004 by using cointegration analysis and causality tests. The outcome suggests that in the period prior to WW1 the growth of the Italian economy led that of exports, while in the post-WW2 period the causal relationship was reversed with the expansion of exports that determined the growth of the Italian economy.
A Publication Aggregation System Using Semantic Blogging

Researchers need to share information about their publications. They also desire to share opinions and comments about each other’s publications. The paper describes a system which demonstrates how semantic blogging can be used for the purpose. The SWRC ontology has been incorporated in the blogging system for entering the metadata of publications. The semantic blogging system provides means to annotate publications. The system is a decentralized publication aggregation system. It utilizes the RSS technology to aggregate posts. The publication metadata is embedded in the RSS to produce BuRST feed. The RSS aggregator has been extended to handle the BuRST feeds. The system uses the FOAF links of the authors and friends to explore the social network of the research community for aggregation of relevant information.
The Complete Genome Sequence of Mycoplasma bovis Strain Hubei-1

Infection by Mycoplasma bovis (M. bovis) can induce diseases, such as pneumonia and otitis media in young calves and mastitis and arthritis in older animals. Here, we report the finished and annotated genome sequence of M. bovis strain Hubei-1, a strain isolated in 2008 that caused calf pneumonia on a Chinese farm. The genome of M. bovis strain Hubei-1 contains a single circular chromosome of 953,114 bp with a 29.37% GC content. We identified 803 open reading frames (ORFs) that occupy 89.5% of the genome. While 34 ORFs were Hubei-1 specific, 662 ORFs had orthologs in the M. bovis type strain PG45 genome. Genome analysis validated lateral gene transfer between M. bovis and the Mycoplasma mycoides subspecies mycoides, while phylogenetic analysis found that the closest M. bovis neighbor is Mycoplasma agalactiae. Glycerol may be the main carbon and energy source of M. bovis, and most of the biosynthesis pathways were incomplete. We report that 47 lipoproteins, 12 extracellular proteins and 18 transmembrane proteins are phase-variable and may help M. bovis escape the immune response. Besides lipoproteins and phase-variable proteins, genomic analysis found two possible pathogenicity islands, which consist of four genes and 11 genes each, and several other virulence factors including hemolysin, lipoate protein ligase, dihydrolipoamide dehydrogenase, extracellular cysteine protease and 5′-nucleotidase.
Network Intrusion Detection System Based On Machine Learning Algorithms

Network and system security is of paramount importance in the present data communication environment. Hackers and intruders can create many successful attempts to cause the crash of the networks and web services by unauthorized intrusion. New threats and associated solutions to prevent these threats are emerging together with the secured system evolution. Intrusion Detection Systems (IDS) are one of these solutions. The main function of Intrusion Detection System is to protect the resources from threats. It analyzes and predicts the behaviours of users, and then these behaviours will be considered an attack or a normal behaviour. We use Rough Set Theory (RST) and Support Vector Machine (SVM) to detect network intrusions. First, packets are captured from the network, RST is used to pre-process the data and reduce the dimensions. The features selected by RST will be sent to SVM model to learn and test respectively. The method is effective to decrease the space density of data. The experiments compare the results with Principal Component Analysis (PCA) and show RST and SVM schema could reduce the false positive rate and increase the accuracy.
Extending Scientific Literature Search by Including the Author's Writing Style

Our work is motivated by the idea to extend the retrieval of related scientific literature to cases, where the relatedness also incorporates the writing style of individual scientific authors. Therefore we conducted a pilot study to answer the question whether humans can identity authorship once the topological clues have been removed. As first result, we found out that this task is challenging, even for humans. We also found some agreement between the annotators. To gain a better understanding how humans tackle such a problem, we conducted an exploratory data analysis. Here, we compared the decisions against a number of topological and stylometric features. The outcome of our work should help to improve automatic authorship identification algorithms and to shape potential follow-up studies.
Evaluating squat performance with a single inertial measurement unit

Inertial measurement units (IMUs) may be used during exercise performance to assess form and technique. To maximise practicality and minimise cost a single-sensor system is most desirable. This study sought to investigate whether a single lumbar-worn IMU is capable of identifying seven commonly observed squatting deviations. Twenty-two volunteers (18 males, 4 females, age: 26.09±3.98 years, height: 1.75±0.14m, body mass: 75.2±14.2 kg) performed the squat exercise correctly and with 7 induced deviations. IMU signal features were extracted for each condition. Statistical analysis and leave one subject out classifier evaluation were used to assess the ability of a single sensor to evaluate performance. Binary level classification was able to distinguish between correct and incorrect squatting performance with a sensitivity of 64.41%, specificity of 88.01% and accuracy of 80.45%. Multi-label classification was able to distinguish between specific squat deviations with a sensitivity of 59.65%, specificity of 94.84% and accuracy of 56.55%. These results indicate that a single IMU can successfully discriminate between squatting deviations. A larger data set must be collected and more complex classification techniques developed in order to create a more robust exercise analysis IMU-based system.
Territorial Politics and formal structures of governance in spatial planning: insights from the Dublin city-region (NIRSA) Working Paper Series 64

This paper presents the argument that the contents of spatial plans and the discursive framing of processes of spatial strategy-making are structured by the political geography of the formal institutions of governance through which spatial plans derive their legitimacy. At the same time spatial planning processes and strategies themselves may be active in the social construction of particular spatial (territorial and scalar) configurations. Spatial planning strategies for cities and regions increasingly seek to transcend political and administrative boundaries and embrace functional spaces and fuzzy boundaries. It is argued here however, that the spatial reach of such strategies in practice may be constrained by the formal structures of governance through which they are produced. Recent studies of the contents of spatial planning strategies have found that spatial plans in practice continue to employ territorial rather than relational concepts of space and place. This paper moves towards explaining the territorial character of planning content through an engagement with recent literature in critical regional studies and critiques of state-centric thinking in political geography and related disciplines. The arguments presented are supported by a case study of the Dublin-city-region, drawing on official planning policy documents and qualitative interviews with political decision-makers and planning officials.
Comparison of respiratory mechanics between sevoflurane and propofol-remifentanil anesthesia for laparoscopic colectomy

Background The creation of pneumoperitoneum and Trendelenburg positioning during laparoscopic surgery are associated with respiratory changes. We aimed to compare respiratory mechanics while using intravenous propofol and remifentanil vs. sevoflurane during laparoscopic colectomy. Methods Sixty patients undergoing laparoscopic colectomy were randomly allocated to one of the two groups: group PR (propofol-remifentanil group; n = 30), and group S (sevoflurane group; n = 30). Peak inspiratory pressure (PIP), dynamic lung compliance (Cdyn), and respiratory resistance (Rrs) values at five different time points: 5 minutes after induction of anesthesia (supine position, T1), 3 minutes after pneumoperitoneum (lithotomy position, T2), 3 minutes after pneumoperitoneum while in the lithotomy-Trendelenburg position (T3), 30 minutes after pneumoperitoneum (T4), and 3 minutes after deflation of pneumoperitoneum (T5). Results In both groups, there were significant increases in PIP and Rrs while Cdyn decreased at times T2, T3, and T4 compared to T1 (P < 0.001). The Rrs of group PR for T2, T3, and T4 were significantly higher than those measured in group S for the corresponding time points (P < 0.05). Conclusions Respiratory mechanics can be adversely affected during laparoscopic colectomy. Respiratory resistance was significantly higher during propofol-remifentanil anesthesia than sevoflurane anesthesia.
Using SAS® Enterprise BI and SAS® Enterprise MinerTM to Reduce Student Attrition

The true supremacy of the SAS® Enterprise Business Intelligence Server is the ability to utilize the power of SAS® Analytics to deliver real-time information to end users, who usually do not understand statistics, but have the ability to make a difference if they have easy access to the analyzed data. This paper describes the process of using SAS® Enterprise Miner to develop a model to score university students based on their risk of attrition and deliver easy-to-understand results to university personnel using SAS® EBI.
INTELLIGENT CO-OPERATIVE PROCESSOR-IN-MEMORY ARCHITECTURES

Advances in VLSI technology are enabling the processor-memory integration to bridge the processor-memory performance gap. It is also a key driver in the innovation of a new concept called Processor-In-Memory (PIM). The work described in this paper capitalises on the extensive work carried out on PIMs in general and develops a road map for an intelligent revision of a PIM architecture referred to as Co-operative Intelligent Memory (CIM). The journey made to reach the goal of achieving a CIM is taken via the route of developing a Cooperative Pseudo Intelligent Memory (CPIM), as proof of concept and mid point in the ratification of the intelligence needed for a full CIM implementation. Both architectures use a hierarchical two level CPU structure referred to as major and minor CPUs. By partitioning computation through dividing workload between major and minor CPUs in an intelligent manner and without any pre-processor compilation or kernel task scheduling, the PIM system can be made more efficient and co-operative for class of tasks, which are heavily reliant on memory-to-memory iterative processes. The proposed architectures exploit the key feature in the iterative process by using vectors that characterize the iteration. The process of identifying intelligently these vectors is described in this paper. In addition, the performance of the proposed architectures has been evaluated
The Impact of Ocean Acidification on Reproduction, Early Development and Settlement of Marine Organisms

Predicting the impact of warming and acidifying on oceans on the early development life history stages of invertebrates although difficult, is essential in order to anticipate the severity and consequences of future climate change. This review summarises the current literature and meta-analyses on the early life-history stages of invertebrates including fertilisation, larval development and the implications for dispersal and settlement of populations. Although fertilisation appears robust to near future predictions of ocean acidification, larval development is much more vulnerable and across invertebrate groups, evidence indicates that the impacts may be severe. This is especially for those many marine organisms which start to calcify in their larval and/or juvenile stages. Species-specificity and variability in responses and current gaps in the literature are highlighted, including the need for studies to investigate the total effects of climate change including the synergistic impact of temperature, and the need for long-term multigenerational experiments to determine whether vulnerable invertebrate species have the capacity to adapt to elevations in atmospheric CO2 over the next century.
Attachment Representations in Adults with Congenital Blindness: Association with Maternal Interactive Behaviors during Childhood

This study explores how maternal interactive behaviors experienced during the childhood of adults with congenital blindness are associated with their subsequent development and personality. Many researchers have found a high frequency of maternal directiveness and overprotection in sighted mother-congenitally blind child relationships. One open question is whether these behaviors may have negative effects on congenitally blind children's subsequent development, or whether they may have a functionally adaptive-strategic role. The purpose of this study was to discriminate between the two hypotheses. This objective was pursued by adopting the theory of attachment and administering the Adult Attachment Interview to 15 participants with congenital blindness. Results suggested that directive and overprotective maternal behaviors are experienced by the persons with congenital blindness as encouraging and functional as long as they are accompanied by an affective, loving, and supportive attitude. Results did not support the hypothesis that directive and overprotective maternal interactive behaviors have a necessarily negative effect on the development of persons with congenital blindness.
Spatio-temporal development of the bacterial community in a tubular longitudinal microbial fuel cell

The spatio-temporal development of a bacterial community in an exoelectrogenic biofilm was investigated in sucrose-fed longitudinal tubular microbial fuel cell reactors, consisting of two serially connected modules. The proportional changes in the microbial community composition were assessed by PCR-DGGE and DNA sequencing in order to relate them to the performance and stability of the bioelectrochemical system. The reproducibility of duplicated reactors, evaluated by cluster analysis and Jaccard’s coefficient, shows 80-90% similarity in species composition. Biofilm development through fed-batch start-up and subsequent stable continuous operation results in a population shift from (-Proteobacteriaand Bacteroidetesto Firmicutesdominated communities, with other diverse species present at much lower relative proportions. DGGE patterns were analyzed by Range-weighted richness (Rr) and Pareto-Lorenz evenness distribution curves to investigate the evolution of the bacterial community. The first modules shifted from dominance by species closely related to B. graminisolvens, R. ornithinolytica and Klebsiella sp. BM21 at the start of continuous-mode operation, to a community dominated by P. propionicigenes, Lactococcus sp. , P. agglomerans and K. oxytoca related species with stable power generation (6.0 W/m3) at day 97. Operational strategies that consider the dynamics of the population will provide useful parameters for evaluating system performance in the practical application of MFCs.
Short-term health effects of particulate and photochemical air pollution in asthmatic children

In a previous panel study in Paris, France, detrimental effects of moderately high levels of winter air pollution on the symptoms and lung function of asthmatic children were demonstrated. A new study was conducted, with the aim of assessing the short-term effects of photo-oxidant and particulate air pollution on childhood asthma during spring and early summer in Paris. Eighty-two medically diagnosed asthmatic children were followed up for 3 months. Outcomes included the incidence and prevalence of asthma attacks, nocturnal cough, supplementary use of β2-agonists, symptoms of airway irritation, and peak expiratory flow (PEF) value and its variability. The statistical methods controlled for the lack of independence between daily health outcomes, temporal trends and pollen and weather conditions. Black smoke and nitrogen dioxide (NO2) were associated with increases in the occurrence of nocturnal cough and respiratory infections. Ozone (O3) was associated with an increase in the occurrence of asthma attacks and respiratory infections and with changes in lung function, as shown by an increase in PEF variability and a decrease in PEF. Statistically significant interactions were demonstrated between O3 and temperature and between O3 and pollen count for asthma attacks. O3 levels had a greater effect on additional bronchodilator use and on irritations of the eyes, nose and throat on days on which no steroids were used. Particulate matter was associated with eye irritation only. This study showed that, although within international air quality standards, the prevailing levels of photo-oxidant and particulate pollution in spring and early summer had measurable short-term effects on children with mild-to-moderate asthma.