Wide Clinical Variability in Cat Eye Syndrome Patients: Four Non-Related Patients and Three Patients from the Same Family

A small supernumerary marker chromosome (sSMC) derived from chromosome 22 is a relatively common cytogenetic finding. This sSMC typically results in tetrasomy for a chromosomal region that spans the chromosome 22p arm and the proximal 2 Mb of 22q11.21. Using classical cytogenetics, fluorescence in situ hybridization, multiplex ligation-dependent probe amplification, and array techniques, 7 patients with sSMCs derived from chromosome 22 were studied: 4 non-related and 3 from the same family (mother, daughter, and son). The sSMCs in all patients were dicentric and bisatellited chromosomes with breakpoints in the chromosome 22 low-copy repeat A region, resulting in cat eye syndrome (CES) due to chromosome 22 partial tetrasomy 22pter -> q11.2 including the cat eye chromosome region. Although all subjects presented the same chromosomal abnormality, they showed a wide range of phenotypic differences, even in the 3 patients from the same family. There are no previous reports of CES occurring within 3 patients in the same family. Thus, the clinical and follow-up data presented here contribute to a better delineation of the phenotypes and outcomes of CES patients and will be useful for genetic counseling. Copyright (C) 2012 S. Karger AG, Basel

Implementation of a VLSI amplifier interfaced with biomedical sensors for ultra wide band data transmission

This thesis presents a CMOS Amplifier with High Common Mode rejection designed in UMC 130nm technology. The goal is to achieve a high amplification factor for a wide range of biological signals (with frequencies in the range of 10Hz-1KHz) and to reject the common-mode noise signal. It is here presented a Data Acquisition System, composed of a Delta-Sigma-like Modulator and an antenna, that is the core of a portable low-complexity radio system; the amplifier is designed in order to interface the data acquisition system with a sensor that acquires the electrical signal. The Modulator asynchronously acquires and samples human muscle activity, by sending a Quasi-Digital pattern that encodes the acquired signal. There is only a minor loss of information translating the muscle activity using this pattern, compared to an encoding technique which uses astandard digital signal via Impulse-Radio Ultra-Wide Band (IR-UWB). The biological signals, needed for Electromyographic analysis, have an amplitude of 10-100μV and need to be highly amplified and separated from the overwhelming 50mV common mode noise signal. Various tests of the firmness of the concept are presented, as well the proof that the design works even with different sensors, such as Radiation measurement for Dosimetry studies.

Depth-based face recognition using local quantized patterns adapted for range data

A depth-based face recognition algorithm specially adapted to high range resolution data acquired by the new Microsoft Kinect 2 sensor is presented. A novel descriptor called Depth Local Quantized Pattern descriptor has been designed to make use of the extended range resolution of the new sensor. This descriptor is a substantial modification of the popular Local Binary Pattern algorithm. One of the main contributions is the introduction of a quantification step, increasing its capacity to distinguish different depth patterns. The proposed descriptor has been used to train and test a Support Vector Machine classifier, which has proven to be able to accurately recognize different people faces from a wide range of poses. In addition, a new depth-based face database acquired by the new Kinect 2 sensor have been created and made public to evaluate the proposed face recognition system.

Analysis of extended range variable gain hybrid Raman-EDFAs in systems using Nyquist-WDM 100/200G PM-QPSK/16QAM

We use the GN-model to assess Nyquist-WDM 100/200Gbit/s PM-QPSK/16QAM signal reach on low loss, large core area fibre using extended range, variable gain hybrid Raman-EDFAs. 5000/1500km transmission is possible over a wide range of amplifier spans. © OSA 2014.

Insights into the influence of solvent polarity on the crystallization of poly(ethylene oxide) spin-coated thin films via in situ grazing incidence wide-angle X-ray scattering

Controlling polymer thin-film morphology and crystallinity is crucial for a wide range of applications, particularly in thin-film organic electronic devices. In this work, the crystallization behavior of a model polymer, poly(ethylene oxide) (PEO), during spin-coating is studied. PEO films were spun-cast from solvents possessing different polarities (chloroform, THF, and methanol) and probed via in situ grazing incidence wide-angle X-ray scattering. The crystallization behavior was found to follow the solvent polarity order (where chloroform < THF < methanol) rather than the solubility order (where THF > chloroform > methanol). When spun-cast from nonpolar chloroform, crystallization largely followed Avrami kinetics, resulting in the formation of morphologies comprising large spherulites. PEO solutions cast from more polar solvents (THF and methanol) do not form well-defined highly crystalline morphologies and are largely amorphous with the presence of small crystalline regions. The difference in morphological development of PEO spun-cast from polar solvents is attributed to clustering phenomena that inhibit polymer crystallization. This work highlights the importance of considering individual components of polymer solubility, rather than simple total solubility, when designing processing routes for the generation of morphologies with optimum crystallinities or morphologies.

A study of the perceived effectiveness of existing recruitment and general institution -wide practices used to enhance women and minority full -time faculty representation at Florida's public universities

As a federal contractor, the State University System of Florida (SUSF) has instituted a wide range of affirmative action practices to hire and promote women and minorities. Should affirmative action be abolished, universities valuing a diverse faculty will have to rely on voluntary practices to attract members of these groups. I explored the present use and perceived effectiveness of recruitment and institution-wide practices used to promote a diverse workforce and identified those practices considered very effective by informed respondents at the nine participating universities. ^ Two questionnaires were used for data collection. Selected recruitment and general institution-wide best practices found in previous studies were used as benchmarks for comparison with existing practices. The questionnaires also included an open-ended question to identify indigenous practices. A follow-up semi-structured interview was conducted to gather information regarding the background of identified practices. ^ Two overall themes emerged from the study. The first was the perception among respondents that women have made substantial gains in faculty representation. This perception is substantiated by actual percentage of women tenure-earning faculty. The second theme was that many of the practices considered very effective are affirmative action-driven, providing women and minorities considerations not afforded White males. These practices, because they single out members of one group over another based on gender and race/ethnicity may become illegal should affirmative action mandates be abolished. ^ Analysis of the data revealed that universities with the highest percentage of practices considered effective and universities located in the most urban areas of the state were the universities with the highest percentage of minority tenure-earning faculty. There appears to be no similar relationship between universities in urban areas and those with the highest percentage of practices considered effective and women tenure-earning faculty representation. The most frequently identified recruitment practice was the development of a receptive institutional image for women and minorities. The most frequently identified practice in promoting a receptive institutional climate was the use of conflict resolution processes and grievance procedures. Five themes also emerged from the 22 barriers in recruiting women and minority full-time faculty identified by the respondents. The most commonly identified barriers were related to a lack of financial resources to support effective practices. ^

Accelerating wide-angle converging waves in the near field

We show that a wide-angle converging wave may be transformed into a shape-preserving accelerating beam having a beam-width near the diffraction limit. For that purpose, we followed a strategy that is particularly conceived for the acceleration of nonparaxial laser beams, in contrast to the well-known method by Siviloglou et al (2007 Phys. Rev. Lett. 99 213901). The concept of optical near-field shaping is applied to the design of non-flat ultra-narrow diffractive optical elements. The engineered curvilinear caustic can be set up by the beam emerging from a dynamic assembly of elementary gratings, the latter enabling to modify the effective refractive index of the metamaterial as it is arranged in controlled orientations. This light shaping process, besides being of theoretical interest, is expected to open up a wide range of broadband application possibilities.

Identifying the knowledge management structures in enterprise systems projects

Enterprise System (ES) implementation and management are knowledge intensive tasks that inevitably draw upon the experience of a wide range of people with diverse knowledge capabilities. Knowledge Management (KM) has been identified as a critical success factor in ES projects. Despite the recognized importance of managing knowledge for ES benefits realization, systematic attempts to conceptualize KM-structures have been few. Where the adequacy of KM-structures is assessed, the process and measures are typically idiosyncratic and lack credibility. Using the ‘KM-process’, itself based in sociology of knowledge, this paper conceptualizes four main constructs to measure the adequacy of KM-structures. The SEM model is tested using 310 responses gathered from 27 ES installations that had implemented SAP R/3. The findings reveal six constructs for KM-structure. Furthermore, the paper demonstrates the application of KM-structures in the context of ES using the Adaptive Structuration Theory. The results demonstrate that having adequate KM-structures in place, while necessary, is not sufficient. These rules and resources must be appropriated to have greater positive influence on the Enterprise System. Furthermore, the study provides empirical support for knowledge-based theory by illustrating the importance of knowledge use/re-use (vs. knowledge creation) as the most important driver in the process of KM.

Re-booting regional planning in South East Queensland

This Issues Paper presents the outcomes of a recent seminar which addressed the theme of 'Re-Booting Regional Planning in South East Queensland'. The seminar was held at the Queensland University of Technology, Brisbane, in June 2004. The seminar brought together a wide range of state and local government, community, private sector and scholarly participants, including members of the Urban Policy Program. This report provides an overview of the context for the seminar, the problems faced by the South East Queensland region which the seminar seeks to address and indentifies outcomes and proposals for future directions arising from the seminar dicussions.

The science of assessment : identifying and predicting environmental impacts

The historical challenge of environmental impact assessment (EIA) has been to predict project-based impacts accurately. Both EIA legislation and the practice of EIA have evolved over the last three decades in Canada, and the development of the discipline and science of environmental assessment has improved how we apply environmental assessment to complex projects. The practice of environmental assessment integrates the social and natural sciences and relies on an eclectic knowledge base from a wide range of sources. EIA methods and tools provide a means to structure and integrate knowledge in order to evaluate and predict environmental impacts.----- This Chapter will provide a brief overview of how impacts are identified and predicted. How do we determine what aspect of the natural and social environment will be affected when a mine is excavated? How does the practitioner determine the range of potential impacts, assess whether they are significant, and predict the consequences? There are no standard answers to these questions, but there are established methods to provide a foundation for scoping and predicting the potential impacts of a project.----- Of course, the community and publics play an important role in this process, and this will be discussed in subsequent chapters. In the first part of this chapter, we will deal with impact identification, which involves appplying scoping to critical issues and determining impact significance, baseline ecosystem evaluation techniques, and how to communicate environmental impacts. In the second part of the chapter, we discuss the prediction of impacts in relation to the complexity of the environment, ecological risk assessment, and modelling.

Increasing relevance and interest : teaching environmental and planning law to built environment students

A new approach was taken to delivering a challenging "stewarship of land" unit to over 350 predominantly first year built environment students stewardship. The new approach involved incorporating environmental and planning law into the syllabus, exposing students to a wide range of statutes, selecting legal cases according to a et of criteria and revisiting the material using different modes of delivery and teaching resources. To evaluate the effectiveness of the new approach, the students were surveyed to elicit their learning experience and preferences. The survey found that most students perceived learning about environmental and planning law, including legal cases, worthwhile.----- Areas identified by the surcey for improvement included the perception by some students that: environmenatl and planning law is irrelevant to their discipline and future caree; studying law is dull and sometimes daunting; and the prescribed reading could be omitted.----- To address student perceptions, it is proposed to reorder the topics commencing with local, charismatic topics, while explanding international content and cases, to enlarge and enhance the repertoire of video clips to include sites of legal cawses and development projects, and to reformat the online weekly quizzes to promote reading of primary material.----- Overall, the approach to teaching environmental and planning law to built environment students, including the criteria for selecting legal cases, described in this paper, was found to be effective.

The guide to the project management body of knowledge (PMBOK Guide) : applicability and contribution to project success in Australia

The PMBOK Guide is one of the most influential publications concerning the knowledge of the project manangement. Moreover, the pervasion of this guide seems to be set to increase as the basis of accreditation - in conjunction with the increasing global trend toward obtaining project management professional status. However, despite the influence and strengthening profile of this guide, reports continue to be published that detail numerous project failures in a wide range of different industries. The PMBOK Guide comprises mainly declarative (know-what) and procedural (know-how) information. In this sense, the guide is largely normative and provides a very good example of the limitations of this approach as highlighted by proponents of a move to the genuine application of positibe theory in project management.----- The aim of this paper is to determine the applicability of the guide in Australia and to determine the extent to which project success can be attributed to the guide. Project Managers from a variety of organisations were surveyed. This postal survey yielded 48 replies. Descriptive statistics was used to assess the incidence and effectivieness of all the processes in the guide. The results indicate that there were no processes that could be considered as peripheral or as a candidate for elimination from the guide. More specifically, all the processes were identified as either a key routine process or a key selective process and positively related to the level of project success. However, the results also indicated that other major factors pertaining to causal knowledge (know-why) are, at least, equally important determinants of project success. It is concluded that declarative, procedural and causal knowledge are all valuable, and given the preponderance of the first two types of knowledge, there seems to be an urgent need to now ensure an equal quest for causal knowledge. In terms of developing causal knowledge, a good starting point would appear to be both positive theory from production and economics.

Space frame walls : facilitating positive development

This paper reports on progress in developing new design and measurement concepts, and translating these concepts into practical applications. This research addresses gaps in ‘best practice’ green building, and is aimed ultimately at replacing green buildings with sustainable urban environments. Building on the author’s previously articulated concepts of Design for Eco-services and Positive Development, this research will demonstrate how to eco-retrofit cities so that they reverse the negative impacts of past design and generate net positive ecological impacts, at no extra cost. In contrast to ‘restorative’ design,this means increasing ecological carrying capacity and natural and social capital through built environment design. Some exemplars for facilitating Positive development will be presented in this talk,such as Green Scaffolding for retrofits, and Green Space Walls for new construction. These structures have been designed to grow and change over time, be easily deconstructed, and entail little waste. The frames support mini-ecospheres that provide a wide range of ecosystem services and biodiversity habitats, as well as heating, cooling and ventilating. In combination, the modules serve to improve human and environmental health. Current work is focused on developing a range of such space frame walls, optimised through an innovative marriage of eco-logical design and virtual modelling.

Same but different: perceptions of IMC amongst marketing communication partners in Australia

This paper examines whether two key partners in the marketing communication process, advertising and public relations’ practitioners perceive IMC in the same way. It compares perceptions across a wide range of implementation, organizational and strategic issues in IMC to test if perceptions have moved past Stage 1 of IMC development (Schultz and Kitchen 2000). Although both advertising and PR practitioners concur with each other and the literature on a wide range of perceptions of IMC, they still believe that advertising and public relations practitioners have dissimilar views about IMC. PR practitioners position themselves as a separate breed of marketing communicator, requiring divergent skills from advertising practitioners and thinking differently about IMC.

Research Monograph: The role of community and lifestyle in the making of a knowledge city

Our brief is to investigate the role of community and lifestyle in the making of a globally successful knowledge city region. Our approach is essentially pragmatic. We start by broadly examining knowledge-based urban development from a number of different perspectives. The first view is historical. In this context knowledge work and knowledge workers are seen as vital parts of a new emergent mode of production reliant on the continual production of abstract knowledge. We briefly develop this perspective to encompass the work of Richard Florida who has, notedly, claimed: “Wherever talent goes, innovation, creativity, and economic growth are sure to follow.” Our next perspective examines concepts of knowledge and modes of its production to discover knowledge is not an unchanging object but a human activity that changes in form and content through history. The suggestion emerges that not only is the production of contemporary ‘knowledge’ organised in a specific (and new) manner but also the output of this networked production is a particular type of knowledge (i.e. techné). The third perspective locates knowledge production and its workers in the contemporary urban context. As such, it co-ordinates the knowledge city in the increasingly global structure of cities and develops a typology of different groups of knowledge workers in their preferred urban environment(s). We see emerging here a distinctive geography of knowledge production. It is an urban phenomenon. There is, in short, something about the nature of cities that knowledge workers find particularly attractive. In the next, essentially anthropological, perspective we start to explore the needs and desires of the individual knowledge worker. Beyond the needs basic to any modern human household an attempt is made to deduce, from a base understanding of knowledge work as mental labour, the compensatory cultural needs of the knowledge worker when not at work - and the expression of these needs in the urban fabric. Our final perspective consists of two case studies. In a review of the experiences of Austin, Texas and Singapore’s one-north precinct we collect empirical data on, respectively, a knowledge city that has sustained itself for over 50 years and an urban precinct newly launched into the global market for knowledge work and knowledge workers. Interwoven The Role of Community and Lifestyle in the Making of a Knowledge City Urban Research Program 8 through all perspectives, in the form of apposite citation, is that of ‘expert opinion’ gathered in a rudimentary poll of academic and industry sources. This opinion appears in text boxes while details of the survey can be found in Appendix A. In the conclusion of the report we interpret the wide range of evidence gathered above in a policy frame. It is our hope this report will leave the reader with a clearer picture of the decisive organisational, infrastructural, aesthetic and social dimensions of a knowledge precinct.