Cognitive-behavioural phenotype in a group of girls from 1.2 to 12 years old with the Incontinentia Pigmenti syndrome:recommendations for clinical management

Incontinentia Pigmenti (IP, OMIM#308300) is a rare X-linked genomic disorder (about 1,400 cases) that affects the neuroectodermal tissue and Central Nervous System (CNS). The objective of this study was to describe the cognitive-behavioural profile in children in order to plan a clinical intervention to improve their quality of life. A total of 14 girls (age range: from 1 year and 2 months to 12 years and 10 months) with IP and the IKBKG/NEMO gene deletion were submitted to a cognitive assessment including intelligence scales, language and visuo-spatial competence tests, learning ability tests, and a behavioural assessment. Five girls had severe to mild intellectual deficiencies and the remaining nine had a normal neurodevelopment. Four girls were of school age and two of these showed no intellectual disability, but had specific disabilities in calculation and arithmetic reasoning. This is the first description of the cognitive-behavioural profile in relation to developmental age. We stress the importance of an early assessment of learning abilities in individuals with IP without intellectual deficiencies to prevent the onset of any such deficit.

A vállalati likviditásmenedzsment számviteli támogatása (Accounting support for company’s liquidity management)

A cikk kiindulópontja, hogy a kettős könyvvitelt vezető vállalkozások által kötelezően elkészítendő éves pénzügyi kimutatások (számviteli beszámolók) olyan információbázist jelentenek, amelyek segítséget nyújthatnak egyrészről a vállalati likviditásmenedzsment támogatásához, másrészről a vállalkozások likviditási helyzetének megítéléséhez. A cikk első fele felvázolja a vállalkozások pénzügyi helyzetét bemutató adatok számviteli kereteit, bemutatja a likviditás fogalmának egyes értelmezéseit, majd részletesen kitér arra, hogy a likviditás utólagos vizsgálatához, illetve előrejelzéséhez milyen korrekciókat kell (lehet) végezni a számviteli beszámoló adatain. A cikk második fele az elemzés lehetséges módjait és eszközeit veszi számba, kitérve a statikus és dinamikus elemzés közötti különbségek bemutatására, az egyes mutatók számításának lehetséges módjaira és értelmezésükre, mindvégig szem előtt tartva az alkalmazás korlátait. ________ The study examines how information provided by accounting information systems could support companies’ liquidity management. The starting point is that compulsory financial statements prepared by economic entities embody an adequate information basis, which could help liquidity management as well as the judgement of liquidity. The first part of the study introduces the various interpretations of liquidity and gives a detailed description of the adjustments that need to be made on accounting data for an a posteriori examination of liquidity and to be able to forecast liquidity. The second part discusses the possible means and tools of analysis, including the differences between static and dynamic methods and the calculation and interpretation of the various ratios widely used by professionals. The conclusion is that there is no best-practice method or ratio, but rather a wide range of tools is to be used when one is willing to gain a complex and comprehensive insight about an entity’s liquidity.

Design principles of semantic binary database management systems

The Semantic Binary Data Model (SBM) is a viable alternative to the now-dominant relational data model. SBM would be especially advantageous for applications dealing with complex interrelated networks of objects provided that a robust efficient implementation can be achieved. This dissertation presents an implementation design method for SBM, algorithms, and their analytical and empirical evaluation. Our method allows building a robust and flexible database engine with a wider applicability range and improved performance. ^ Extensions to SBM are introduced and an implementation of these extensions is proposed that allows the database engine to efficiently support applications with a predefined set of queries. A New Record data structure is proposed. Trade-offs of employing Fact, Record and Bitmap Data structures for storing information in a semantic database are analyzed. ^ A clustering ID distribution algorithm and an efficient algorithm for object ID encoding are proposed. Mapping to an XML data model is analyzed and a new XML-based XSDL language facilitating interoperability of the system is defined. Solutions to issues associated with making the database engine multi-platform are presented. An improvement to the atomic update algorithm suitable for certain scenarios of database recovery is proposed. ^ Specific guidelines are devised for implementing a robust and well-performing database engine based on the extended Semantic Data Model. ^

Data partitioning and replication management in distributed geographic information system (GIS) database

This research presents several components encompassing the scope of the objective of Data Partitioning and Replication Management in Distributed GIS Database. Modern Geographic Information Systems (GIS) databases are often large and complicated. Therefore data partitioning and replication management problems need to be addresses in development of an efficient and scalable solution. ^ Part of the research is to study the patterns of geographical raster data processing and to propose the algorithms to improve availability of such data. These algorithms and approaches are targeting granularity of geographic data objects as well as data partitioning in geographic databases to achieve high data availability and Quality of Service(QoS) considering distributed data delivery and processing. To achieve this goal a dynamic, real-time approach for mosaicking digital images of different temporal and spatial characteristics into tiles is proposed. This dynamic approach reuses digital images upon demand and generates mosaicked tiles only for the required region according to user's requirements such as resolution, temporal range, and target bands to reduce redundancy in storage and to utilize available computing and storage resources more efficiently. ^ Another part of the research pursued methods for efficient acquiring of GIS data from external heterogeneous databases and Web services as well as end-user GIS data delivery enhancements, automation and 3D virtual reality presentation. ^ There are vast numbers of computing, network, and storage resources idling or not fully utilized available on the Internet. Proposed "Crawling Distributed Operating System "(CDOS) approach employs such resources and creates benefits for the hosts that lend their CPU, network, and storage resources to be used in GIS database context. ^ The results of this dissertation demonstrate effective ways to develop a highly scalable GIS database. The approach developed in this dissertation has resulted in creation of TerraFly GIS database that is used by US government, researchers, and general public to facilitate Web access to remotely-sensed imagery and GIS vector information. ^

Medical advice, diabetes self-management, and health outcomes of a multi-ethnic population from the National Health and Nutrition Examination Survey 2007--2008

Diabetes self-management, an essential component of diabetes care, includes weight control practices and requires guidance from providers. Minorities are likely to have less access to quality health care than White non-Hispanics (WNH) (American College of Physicians-American Society of Internal Medicine, 2000). Medical advice received and understood may differ by race/ethnicity as a consequence of the patient-provider communication process; and, may affect diabetes self-management. ^ This study examined the relationships among participants’ report of: (1) medical advice given; (2) diabetes self-management, and; (3) health outcomes for Mexican-Americans (MA) and Black non-Hispanics (BNH) as compared to WNH (reference group) using data available through the National Health and Nutrition Examination Survey (NHANES) for the years 2007–2008. This study was a secondary, single point analysis. Approximately 30 datasets were merged; and, the quality and integrity was assured by analysis of frequency, range and quartiles. The subjects were extracted based on the following inclusion criteria: belonging to either the MA, BNH or WNH categories; 21 years or older; responded yes to being diagnosed with diabetes. A final sample size of 654 adults [MA (131); BNH (223); WNH (300)] was used for the analyses. The findings revealed significant statistical differences in medical advice reported given. BNH [OR = 1.83 (1.16, 2.88), p = 0.013] were more likely than WNH to report being told to reduce fat or calories. Similarly, BNH [OR = 2.84 (1.45, 5.59), p = 0.005] were more likely than WNH to report that they were told to increase their physical activity. Mexican-Americans were less likely to self-monitor their blood glucose than WNH [OR = 2.70 (1.66, 4.38), p<0.001]. There were differences among ethnicities for reporting receiving recent diabetes education. Black, non-Hispanics were twice as likely to report receiving diabetes education than WNH [OR = 2.29 (1.36, 3.85), p = 0.004]. Medical advice reported given and ethnicity/race, together, predicted several health outcomes. Having recent diabetes education increased the likelihood of performing several diabetes self-management behaviors, independent of race. ^ These findings indicate a need for patient-provider communication and care to be assessed for effectiveness and, the importance of ongoing diabetes education for persons with diabetes.^

Not getting by: Poverty management and homelessness in Miami

Urban inequality has emerged as one of the dominant themes of modern life and globalization. More than three million people experienced homelessness in the United States last year; in Miami-Dade, more than 15,000 individuals were homeless. Surviving extreme poverty, and exiting or avoiding homelessness, involves negotiating a complex mix of public and private assistance. However, a range of factors influence what types of help are available and how they can be accessed. Frequently, larger social structures determine which resource are available, leaving many choices entirely out of the individual's control. For single men, who are ineligible for many benefits, homelessness can be difficult to avoid and even harder to exit. My study seeks to better understand how adult, minority men living in extreme poverty in Miami-Dade negotiate their daily survival. Specific research questions address: Do black and Hispanic men who are homeless or at risk of homelessness have different personal characteristics and different experiences in avoiding or exiting homelessness? How does Miami's response to extreme poverty/homelessness, including availability of public benefits and public and private service organizations, either maximize or constrain the choices available to this population? And, what is the actual experience of single, adult men who are homeless or at risk of homelessness, in negotiating their daily survival? A mixed methods approach combines quantitative survey data from 7,605 homeless men, with qualitative data from 54 semi-structured interviews incorporating the visual ethnography techniques of Photo Elicitation Interviewing. Results show the differences experienced by black and Hispanic men who are poor and homeless in Miami. Findings also highlight how the community's official and unofficial responses to homelessness intersect with the actual experiences of the persons targeted by the policies and programs, challenging preconceived notions regarding the lives of persons living in extreme poverty. It adds to the existing body of literature by focusing on the urban Miami context, emphasizing disparities amongst racial and ethnic groups. Findings are intended to provide an empirically grounded thesis that humanizes the subjects and illuminates their personal experiences, helping to inform public policy around the needs of extremely poor populations.

Reference Points: Hotel Management Training In The United States and Europe

Different cultures and historical precedents produce a broad range of influences on the training of hotel managers in Europe and the United States. The author isolates a certain number of facts the nature of which clarify an understanding of two attitudes which complement each other to the benefit of their common objective - efficient professional training.

Range Queries on an encrypted outsourced database

This project is about retrieving data in range without allowing the server to read it, when the database is stored in the server. Basically, our goal is to build a database that allows the client to maintain the confidentiality of the data stored, despite all the data is stored in a different location from the client's hard disk. This means that all the information written on the hard disk can be easily read by another person who can do anything with it. Given that, we need to encrypt that data from eavesdroppers or other people. This is because they could sell it or log into accounts and use them for stealing money or identities. In order to achieve this, we need to encrypt the data stored in the hard drive, so that only the possessor of the key can easily read the information stored, while all the others are going to read only encrypted data. Obviously, according to that, all the data management must be done by the client, otherwise any malicious person can easily retrieve it and use it for any malicious intention. All the methods analysed here relies on encrypting data in transit. In the end of this project we analyse 2 theoretical and practical methods for the creation of the above databases and then we tests them with 3 datasets and with 10, 100 and 1000 queries. The scope of this work is to retrieve a trend that can be useful for future works based on this project.

The paradoxes of risk management in the banking sector

This paper uses empirical evidence to examine the operational dynamics and paradoxical nature of risk management systems in the banking sector. It demonstrates how a core paradox of market versus regulatory demands and an accompanying variety of performance, learning and belonging paradoxes underlie evident tensions in the interaction between front and back office staff in banks. Organisational responses to such paradoxes are found to range from passive to proactive, reflecting differing organisational, departmental and individual risk culture(s), and performance management systems. Nonetheless, a common feature of regulatory initiatives designed to secure a more structurally independent risk management function is that they have failed to rectify a critical imbalance of power - with the back office control functions continuing to be dominated by front office trading and investment functions. Ultimately, viewing the 'core' of risk management systems as a series of connected paradoxes rather than a set of assured, robust practices, requires a fundamental switch in emphasis away from a normative, standards-based approach to risk management to one which gives greater recognition to its behavioural dimensions.

Non-Pharmacological Approaches for Pain Management in Sickle Cell Disease: Development of a Mindfulness-Based Intervention

Background: Sickle Cell Disease (SCD) is a genetic hematological disorder that affects more than 7 million people globally (NHLBI, 2009). It is estimated that 50% of adults with SCD experience pain on most days, with 1/3 experiencing chronic pain daily (Smith et al., 2008). Persons with SCD also experience higher levels of pain catastrophizing (feelings of helplessness, pain rumination and magnification) than other chronic pain conditions, which is associated with increases in pain intensity, pain behavior, analgesic consumption, frequency and duration of hospital visits, and with reduced daily activities (Sullivan, Bishop, & Pivik, 1995; Keefe et al., 2000; Gil et al., 1992 & 1993). Therefore effective interventions are needed that can successfully be used manage pain and pain-related outcomes (e.g., pain catastrophizing) in persons with SCD. A review of the literature demonstrated limited information regarding the feasibility and efficacy of non-pharmacological approaches for pain in persons with SCD, finding an average effect size of .33 on pain reduction across measurable non-pharmacological studies. Second, a prospective study on persons with SCD that received care for a vaso-occlusive crisis (VOC; N = 95) found: (1) high levels of patient reported depression (29%) and anxiety (34%), and (2) that unemployment was significantly associated with increased frequency of acute care encounters and hospital admissions per person. Research suggests that one promising category of non-pharmacological interventions for managing both physical and affective components of pain are Mindfulness-based Interventions (MBIs; Thompson et al., 2010; Cox et al., 2013). The primary goal of this dissertation was thus to develop and test the feasibility, acceptability, and efficacy of a telephonic MBI for pain catastrophizing in persons with SCD and chronic pain.

Methods: First, a telephonic MBI was developed through an informal process that involved iterative feedback from patients, clinical experts in SCD and pain management, social workers, psychologists, and mindfulness clinicians. Through this process, relevant topics and skills were selected to adapt in each MBI session. Second, a pilot randomized controlled trial was conducted to test the feasibility, acceptability, and efficacy of the telephonic MBI for pain catastrophizing in persons with SCD and chronic pain. Acceptability and feasibility were determined by assessment of recruitment, attrition, dropout, and refusal rates (including refusal reasons), along with semi-structured interviews with nine randomly selected patients at the end of study. Participants completed assessments at baseline, Week 1, 3, and 6 to assess efficacy of the intervention on decreasing pain catastrophizing and other pain-related outcomes.

Results: A telephonic MBI is feasible and acceptable for persons with SCD and chronic pain. Seventy-eight patients with SCD and chronic pain were approached, and 76% (N = 60) were enrolled and randomized. The MBI attendance rate, approximately 57% of participants completing at least four mindfulness sessions, was deemed acceptable, and participants that received the telephonic MBI described it as acceptable, easy to access, and consume in post-intervention interviews. The amount of missing data was undesirable (MBI condition, 40%; control condition, 25%), but fell within the range of expected missing outcome data for a RCT with multiple follow-up assessments. Efficacy of the MBI on pain catastrophizing could not be determined due to small sample size and degree of missing data, but trajectory analyses conducted for the MBI condition only trended in the right direction and pain catastrophizing approached statistically significance.

Conclusion: Overall results showed that at telephonic group-based MBI is acceptable and feasible for persons with SCD and chronic pain. Though the study was not able to determine treatment efficacy nor powered to detect a statistically significant difference between conditions, participants (1) described the intervention as acceptable, and (2) the observed effect sizes for the MBI condition demonstrated large effects of the MBI on pain catastrophizing, mental health, and physical health. Replication of this MBI study with a larger sample size, active control group, and additional assessments at the end of each week (e.g., Week 1 through Week 6) is needed to determine treatment efficacy. Many lessons were learned that will guide the development of future studies including which MBI strategies were most helpful, methods to encourage continued participation, and how to improve data capture.

En Route Speed Reduction Concept for Absorbing Air Traffic Flow Management Delays

This paper proposes an en route speed reduction to complement current ground delay practices in air traffic flow management. Given a nominal cruise speed, there exists a bounded range of speeds that allows aircraft to fly slower with the same or lower fuel consumption than the nominal flight. Therefore, flight times are increased and delay can be partially performed in the air, at no extra fuel cost for the operator. This concept has been analyzed in an initial feasibility study, computing the maximum amount of delay that can be performed in the air in some representative flights. The impact on fuel consumption has been analyzed, and two scenarios are proposed: the flight fuel remains the same as in the nominal flight, and some extra fuel allowance is permitted in order to face uncertainties. Results show significant values of airborne delay that may be useful in many situations, with the exception of short hauls where airborne delay may be too short. If cruise altitude is changed, the amount of airborne delay increases, since changes in cruise speed modify the optimal flight altitudes. From the analyzed flights, a linear dependency is found relating the airborne delay with the amount of extra fuel allowance.

Service Quality in Arts Events: Operations Management Strategies for Effective Delivery

Recent debate on the quality of arts events has concentrated on the requirement to deliver against a complex range of political, social, and cultural criteria with an emphasis on the external partnerships that are forged. Yet those aspects of quality over which event organizers have more direct control have been accorded minor examination. The authors believe that operational effectiveness is key to service quality in the cultural context, and seek to demonstrate that a balanced consideration of both process and product is vital to fully deliver quality arts events. This article identifies areas of emergent research and practice and focuses on issues in the front-of-house environment where the breakdown of service quality is a real concern, using the experience of one UK not-for-profit arts organization as a case study to illustrate potential management responses.

Projections of change in key ecosystem indicators for planning and management of marine protected areas: An example study for European seas

Marine Protected Areas (MPAs) are widely used as tools to maintain biodiversity, protect habitats and ensure that development is sustainable. If MPAs are to maintain their role into the future it is important for managers to understand how conditions at these sites may change as a result of climate change and other drivers, and this understanding needs to extend beyond temperature to a range of key ecosystem indicators. This case study demonstrates how spatially-aggregated model results for multiple variables can provide useful projections for MPA planners and managers. Conditions in European MPAs have been projected for the 2040s using unmitigated and globally managed scenarios of climate change and river management, and hence high and low emissions of greenhouse gases and riverborne nutrients. The results highlight the vulnerability of potential refuge sites in the north-west Mediterranean and the need for careful monitoring at MPAs to the north and west of the British Isles, which may be affected by changes in Atlantic circulation patterns. The projections also support the need for more MPAs in the eastern Mediterranean and Adriatic Sea, and can inform the selection of sites.

Projections of change in key ecosystem indicators for planning and management of marine protected areas: An example study for European seas

Marine Protected Areas (MPAs) are widely used as tools to maintain biodiversity, protect habitats and ensure that development is sustainable. If MPAs are to maintain their role into the future it is important for managers to understand how conditions at these sites may change as a result of climate change and other drivers, and this understanding needs to extend beyond temperature to a range of key ecosystem indicators. This case study demonstrates how spatially-aggregated model results for multiple variables can provide useful projections for MPA planners and managers. Conditions in European MPAs have been projected for the 2040s using unmitigated and globally managed scenarios of climate change and river management, and hence high and low emissions of greenhouse gases and riverborne nutrients. The results highlight the vulnerability of potential refuge sites in the north-west Mediterranean and the need for careful monitoring at MPAs to the north and west of the British Isles, which may be affected by changes in Atlantic circulation patterns. The projections also support the need for more MPAs in the eastern Mediterranean and Adriatic Sea, and can inform the selection of sites.

Ocean sprawl: challenges and opportunities for biodiversity management in a changing world.

The last few decades have seen rapid proliferation of hard artificial structures (e.g., energy infra-structure, aquaculture, coastal defences) in the marine environment: ocean sprawl. The replacement of natural, often sedimentary, substrata with hard substrata has altered the distribution of species, particularly non-indigenous species, and can facilitate the assisted migration of native species at risk from climate change. This has been likened to urbanization as a driver of global biotic homogenization in the marine environment—the process by which species invasions and extinctions increase the genetic, taxonomic, or functional similarity of communities at local, regional, and global scales. Ecological engineering research showed that small-scale engineering interventions can have a significant positive effect on the biodiversity of artificial structures, promoting more diverse and resilient communities on local scales. This knowledge can be applied to the design of multifunctional structures that provide a range of ecosystem services. In coastal regions, hybrid designs can work with nature to combine hard and soft approaches to coastal defence in a more environmentally sensitive manner. The challenge now is to manage ocean sprawl with the dual goal of supporting human populations and activities, simultaneously strengthening ecosystem resilience using an ecosystem- based approach.