Neuropsychological development in children with early and continuously treated phenylketonuria : association with biochemical markers

PKU is a genetically inherited inborn error of metabolism caused by a deficiency of the enzyme phenylalanine hydroxylase. The failure of this enzyme causes incomplete metabolism of protein ingested in the diet, specifically the conversion of one amino acid, phenylalanine, to tyrosine, which is a precursor to the neurotransmitter dopamine. Rising levels of phenylalanine is toxic to the developing brain, disrupting the formation of white matter tracts. The impact of tyrosine deficiency is not as well understood, but is hypothesized to lead to a low dopamine environment for the developing brain. Detection in the newborn period and continuous treatment (a low protein phe-restricted diet supplemented with phenylalanine-free protein formulas) has resulted in children with early and continuously treated PKU now developing normal I.Q. However, deficits in executive function (EF) are common, leading to a rate of Attention Deficit Hyperactivity Disorder (ADHD) up to five times the norm. EF worsens with exposure to higher phenylalanine levels, however recent research has demonstrated that a high phenylalanine to tyrosine ratio (phenylalanine:tyrosine ratio), which is hypothesised to lead to poorer dopamine function, has a more negative impact on EF than phenylalanine levels alone. Research and treatment of PKU is currently phenylalanine-focused, with little investigation of the impact of tyrosine on neuropsychological development. There is no current consensus as to the veracity of tyrosine monitoring or treatment in this population. Further, the research agenda in this population has demonstrated a primary focus on EF impairment alone, even though there may be additional neuropsychological skills compromised (e.g., mood, visuospatial deficits). The aim of this PhD research was to identify residual neuropsychological deficits in a cohort of children with early and continuously treated phenylketonuria, at two time points in development (early childhood and early adolescence), separated by eight years. In addition, this research sought to determine which biochemical markers were associated with neuropsychological impairments. A clinical practice survey was also undertaken to ascertain the current level of monitoring/treatment of tyrosine in this population. Thirteen children with early and continuously treated PKU were tested at mean age 5.9 years and again at mean age 13.95 years on several neuropsychological measures. Four children with hyperphenylalaninemia (a milder version of PKU) were also tested at both time points and provide a comparison group in analyses. Associations between neuropsychological function and biochemical markers were analysed. A between groups analysis in adolescence was also conducted (children with PKU compared to their siblings) on parent report measures of EF and mood. Minor EF impairments were evident in the PKU group by age 6 years and these persisted into adolescence. Life-long exposure to high phenylalanine:tyrosine ratio and/or low tyrosine independent of phenylalanine were significantly associated with EF impairments at both time points. Over half the children with PKU showed severe impairment on a visuospatial task, and this was associated only with concurrent levels of tyrosine in adolescence. Children with PKU also showed a statistically significant decline in a language comprehension task from 6 years to adolescence (going from normal to subnormal), this deficit was associated with lifetime levels of phenylalanine. In comparison, the four children with hyperphenylalaninemia demonstrated normal function at both time points, across all measures. No statistically significant differences were detected between children with PKU and their siblings on the parent report of EF and mood. However, depressive symptoms were significantly correlated with: EF; long term high phe:tyr exposure; and low tyrosine levels independent of phenylalanine. The practice survey of metabolic clinics from 12 countries indicated a high level of variability in terms of monitoring/treatment of tyrosine in this population. Whilst over 80% of clinics surveyed routinely monitored tyrosine levels in their child patients, 25% reported treatment strategies to increase tyrosine (and thereby lower the phenylalanine:tyrosine ratio) under a variety of patient presentation conditions. Overall, these studies have shown that EF impairments associated with PKU provide support for the dopamine-deficiency model. A language comprehension task showed a different trajectory, serving a timely reminder that non-EF functions also remain vulnerable in this population; and that normal function in childhood does not guarantee normal function by adolescence. Mood impairments were associated with EF impairments as well as long term measures of phenylalanine:tyrosine and/or tyrosine. The implications of this research for enhanced clinical guidelines are discussed given varied current practice.

Re-conceptualising and re-positioning Australian library and information science education for the 21st century [Final Report 2011]

How can Australian library and information science (LIS) education produce, in a sustainable manner, the diverse supply of graduates with the appropriate attributes to develop and maintain high quality professional practice in the rapidly changing 21st century? This report presents the key findings of a project that has examined this question through research into future directions for LIS education in Australia. Titled Re-conceptualising and re-positioning Australian library and information science education for the twenty-first century, the purpose of the project was to establish a consolidated and holistic picture of the Australian LIS profession, and identify how its future education and training can be mediated in a cohesive and sustainable manner. The project was undertaken with a team of 12 university and vocational LIS educators from 11 institutions around Australia between November 2009 and December 2010. Collectively, these eleven institutions represented the broad spectrum and diversity of LIS education in Australia, and enabled the project to examine education for the information profession in a holistic and synergistic manner. Participating institutions in the project included Queensland University of Technology (Project Leader), Charles Sturt University, Curtin University of Technology, Edith Cowan University, Monash University, RMIT University, University of Canberra, University of South Australia, University of Tasmania, University of Technology Sydney and Victoria University. The inception and need for the project was motivated by a range of factors. From a broad perspective several of these factors relate to concerns raised at national and international levels regarding problems with education for LIS. In addition, the motivation and need for the project also related to some unique challenges that LIS education faces in the Australian tertiary education landscape. Over recent years a range of responses to explore the various issues confronting LIS education in Australia have emerged at local and national levels however this project represented the first significant investment of funding for national research in this area. In this way, the inception of the project offered a unique opportunity and powerful mechanism through which to bring together key stakeholders and inspire discourse concerning future education for the profession. Therefore as the first national project of its kind, its intent has been to provide foundation research that will inform and guide future directions for LIS education and training in Australia. The primary objective of the project was to develop a Framework for the Education of the Information Professions in Australia. The purpose of this framework was to provide evidence based strategic recommendations that would guide Australia’s future education for the information professions. Recognising the three major and equal players in the education process the project was framed around three areas of consideration: LIS students, the LIS workforce and LIS educators. Each area of consideration aligned to a research substudy in the project. The three research substudies were titled Student Considerations, Workforce Planning Considerations and Tertiary Education Considerations. The Students substudy provided a profile of LIS students and an analysis of their choices, experiences and expectations in regard to LIS education and their graduate destinations. The Workforce substudy provided an overview and analysis of the nature of the current LIS workforce, including a focus on employer expectations and employment opportunities and comment on the core and elective skill, knowledge and attitudes of current and future LIS professionals. Finally the Tertiary Education substudy provided a profile of LIS educators and an analysis of their characteristics and experiences including the key issues and challenges. In addition it also explored current national and international trends and priorities impacting on LIS education. The project utilised a Community Based Participatory Research (CBPR) approach. This approach involves all members of the community in all aspects of the project. It recognised the unique strengths and perspectives that community members bring to the process. For this project ‘community’ comprised of all individuals who have a role in, or a vested interest in, LIS education and included LIS educators, professionals, employers, students and professional associations. Individuals from these sub-groups were invited to participate in a range of aspects of the project from design through to implementation and evaluation. A range of research methodologies were used to consider the many different perspectives of LIS education, including employers and recruiters, professional associations, students, graduates and LIS teaching staff. Data collection involved a mixed method approach of questionnaires, focus groups, semi-structured interviews and environmental scans. An array of approaches was selected to ensure that broadest possible access to different facets of the information profession would be achieved. The main findings and observations from each substudy have highlighted a range of challenges for LIS education that need to be addressed. These findings and observations have grounded the development of the Framework for the Education of the Information Professions in Australia. The framework presents eleven recommendations to progress the national approach to LIS education and guide Australia’s future education for the information professions. The framework will be used by the LIS profession, most notably its educators, as strategic directions for the future of LIS education in Australia. Framework for the Education of the Information Professions in Australia: Recommendation 1: It is recommended that a broader and more inclusive vocabulary be adopted that both recognises and celebrates the expanding landscape of the field, for example ‘information profession’, ‘information sector’, ‘information discipline’ and ‘information education’. Recommendation 2: It is recommended that a self-directed body composed of information educators be established to promote, support and lead excellence in teaching and research within the information discipline. Recommendation 3: It is recommended that Australia’s information discipline continue to develop excellence in information research that will raise the discipline’s profile and contribute to its prominence within the national and international arena. Recommendation 4: It is recommended that further research examining the nature and context of Australia’s information education programs be undertaken to ensure a sustainable and relevant future for the discipline. Recommendation 5: It is recommended that further research examining the pathways and qualifications available for entry into the Australian information sector be undertaken to ensure relevance, attractiveness, accessibility and transparency. Recommendation 6: It is recommended that strategies are developed and implemented to ensure the sustainability of the workforce of information educators. Recommendation 7: It is recommended that a national approach to promoting and marketing the information profession and thereby attracting more students to the field is developed. Recommendation 8: It is recommended that Australia’s information discipline continues to support a culture of quality teaching and learning, especially given the need to accommodate a focus on the broader information landscape and more flexible delivery options. Recommendation 9: It is recommended that strategies are developed that will support and encourage collaboration between information education within the higher education and VET sectors. Recommendation 10: It is recommended that strategies and forums are developed that will support the information sector working together to conceptualise and articulate their professional identity and educational needs. Recommendation 11: It is recommended that a research agenda be established that will identify and prioritise areas in which further development or work is needed to continue advancing information education in Australia. The key findings from this project confirm that a number of pressing issues are confronting LIS education in Australia. Left unaddressed these issues will have significant implications for the future of LIS education as well as the broader LIS profession. Consequently creating a sustainable and cohesive future can only be realised through cooperation and collaboration among all stakeholders including those with the capacity to enact radical change in university and vocational institutions. Indeed the impending adoption and implementation of the project’s recommendations will fundamentally determine whether Australian LIS education is assured both for the present day and into the future.

Tracking the defining moments of crisis process and practice

Crisis holds the potential for profound change in organizations and industries. The past 50 years of crisis management highlight key shifts in crisis practice, creating opportunities for multiple theories and research tracks. Defining crises such as Tylenol, Exxon Valdez, and September 11 terrorist attacks have influenced or challenged the principles of best practice of crisis communication in public relations. This study traces the development of crisis process and practice by identifying shifts in crisis research and models and mapping these against key management theories and practices. The findings define three crisis domains: crisis planning, building and testing predictive models, and mapping and measuring external environmental influences. These crisis domains mirror but lag the evolution of management theory, suggesting challenges for researchers to reshape the research agenda to close the gap and lead the next stage of development in the field of crisis communication for effective organizational outcomes.

Nonprofit organisations in Australia : what do we know nine years later and what should we find out now?

I welcomed this chance to look back over the past nine years of third sector research in Australia and the opportunity to participate in this gathering to chart another nine years. There will be two parts to this paper. Part one will review what I said in 1991 (Lyons, 1991) and summarise what has happened since to see if my hopes were realised. Part two will attempt to sketch a framework for a research agenda, informed by the same motivation as my 1991 paper. It will conclude with some reflections about how to build a stronger institutional framework to sustain third sector research...

Managing Work, Hedonism and 'the Borderline' Between the Legal and Illegal Markets: Two Case Studies of Recreational Heavy Drug Users

In the context of cultural and/or differential ‘normalisation’ of certain forms of drug use, this article describes two case-studies of heavy recreational drug users. The daily lives of these users blur the line between the legal and the illegal; their drug trading is generally as a consumer and ‘friend of a friend’ small dealer in the low-level market. In the first case, problems with management of employment, time and financial budgeting are described; in the second case, such management is accomplished. Discussion refers to: differences between the two in relation to resources and vulnerability to risks, and to leisure/pleasure cultures of hedonism. The research agenda should pay more attention to users who seek to maintain a legitimate lifestyle but who develop problems managing work and their drug-related leisure. Understanding the consumer demand and dealing activity of such users is important in trying to develop a fuller understanding of drug markets.

A left realist approach to antifeminist fathers’ rights groups

Despite earlier critiques of left realists’ failure to adequately address feminist concerns, recent left realist theorizing and empirical research have made valuable contributions to the understanding of woman abuse and other forms of gendered violence. Left realism has further potential to contribute to the criminological understanding of woman abuse and its contributing socioeconomic and cultural contexts. This article describes left realists’ early efforts to include gender in analyses of crime. It then summarizes feminist critiques of left realism and reviews the work that has responded to them. Drawing upon two prominent strands of feminist left realist theorizing about violence and gender, the paper proposes a preliminary left realist theory of antifeminist fathers’ rights group activism. It then outlines a provisional research agenda on antifeminist fathers’ rights groups, and proposes short and long term policies and practices to enhance the safety of abused mothers and their children following divorce or separation.

Managing intravenous medications in the non-hospital setting: An ethnographic investigation

Little published information exists about the issues involved in conducting complex intravenous medication therapy in patients' homes. An ethnographic study of a local hospital-in-the-home program in the Australian Capital Territory explored this phenomenon to identify those factors that had an impact on the use of medicine in the home environment. This article focuses on one of the three themes identified in the study-Clinical Practice. Within this theme, topics related to the organization and management of intravenous medications, geography and diversity of patient caseload, and communication in the practice setting are discussed. These findings have important implications for policy development and establishment of a research agenda for hospital-in-the-home services.

Design and Anthropology

Design and Anthropology challenges conventional thinking regarding the nature of design and creativity, in a way that acknowledges the improvisatory skills and perceptual acuity of people. Combining theoretical investigations and documentation of practice based experiments, it addresses methodological questions concerning the re-conceptualisation of the relation between design and use from both theoretical and practice-based positions. Concerned with what it means to draw 'users' into processes of designing and producing this book emphasises the creativity of design and the emergence of objects in social situations and collaborative endeavours. Organised around the themes of perception and the user-producer, skilled practices of designing and using, and the relation between people and things, the book contains the latest work of researchers from academia and industry, to enhance our understanding of ethnographic practice and develop a research agenda for the emergent field of design anthropology. Drawing together work from anthropologists, philosophers, designers, engineers, scholars of innovation and theatre practitioners, Design and Anthropology will appeal to anthropologists and to those working in the fields of design and innovation, and the philosophy of technology and engineering.

A methodological approach to modelling design led innovation across secondary education : an Australian case study

Incorporating design thinking as a generic capability at a school level is needed to ensure future generations are empowered for business innovation and active citizenship. This paper describes the methodology of an investigation into modelling design led innovation approaches from the business sector to secondary education, as part of a larger study. It builds on a previously discussed research agenda by outlining the scope, significance and limitations of currently available research in this area, examining an action research methodology utilising an Australian design immersion program case study, and discussing implications and future work. It employs a triangulated approach encompassing thematic analysis of qualitative data collection from student focus groups, semi-structured convergent interviews with teachers and facilitators, and student journals. Eventual outcomes will be reviewed and analysed within the framework of a proposed innovation matrix model for educational growth, synthesising principles responding to 21st century student outcomes. It is anticipated this research will inform a successful design led secondary education innovation model, facilitating new engagement frameworks between tertiary and secondary education sectors, as well as providing new insight into the suitability of action research in prototyping social innovation in Australia.

Design minds : an online design education platform for non-designers to enact cultural change

Education in the 21st century demands a model for understanding a new culture of learning in the face of rapid change, open access data and geographical diversity. Teachers no longer need to provide the latest information because students themselves are taking an active role in peer collectives to help create it. This paper examines, through an Australian case study entitled ‘Design Minds’, the development of an online design education platform as a key initiative to enact a government priority for state-wide cultural change through design-based curriculum. Utilising digital technology to create a supportive community, ‘Design Minds’ recognises that interdisciplinary learning fostered through engagement will empower future citizens to think, innovate, and discover. This paper details the participatory design process undertaken with multiple stakeholders to create the platform. It also outlines a proposed research agenda for future measurement of its value in creating a new learning culture, supporting regional and remote communities, and revitalising frontline services. It is anticipated this research will inform ongoing development of the online platform, and future design education and research programs in K-12 schools in Australia.

Micro-credit is not sufficient for the success of micro-enterprises at the bottom of the pyramid

This study examines the factors affecting the successful provision of micro-credit to people at the bottom of the pyramid and discusses the activities required to support entrepreneurial activities in a peri-urban African setting. The findings enable us to better understand why micro-credit, though useful, is only part of the solution, in a setting characterized by extreme resource constraints with an institutional fabric lacking the infrastructure that assists market development. We depict the crafting of new entrepreneurial activity as an ongoing process and present an emerging research agenda for future developments.

We still don’t know how much BPMN is enough – but we are getting closer

Process models expressed in BPMN typically rely on a small subset of all available symbols. In our 2008 study, we examined the composition of these subsets, and found that the distribution of BPMN symbols in practice closely resembles the frequency distribution of words in natural language. We offered some suggestions based on our findings, how to make the use of BPMN more manageable and also outlined ideas for further development of BPMN. Since this paper was published it has provoked spirited debate in the BPM practitioner community, prompted the definition of a modeling standard in US government, and helped shape the next generation of the BPMN standard.

ICT shared services in the higher education sector : foundations, benefits, success factors and issues

Universities are more and more challenged by the emerging global higher education market, facilitated by advances in Information and Communication Technologies (ICT). This requires them to reconsider their mission and direction in order to function effectively and efficiently, and to be responsive to changes in their environment. In the face of increasing demands and competitive pressures, Universities like other companies, seek to continuously innovate and improve their performance. Universities are considering co-operating or sharing, both internally and externally, in a wide range of areas to achieve cost effectiveness and improvements in performance. Shared services are an effective model for re-organizing to reduce costs, increase quality and create new capabilities. Shared services are not limited to the Higher Education (HE) sector. Organizations across different sectors are adopting shared services, in particular for support functions such as Finance, Accounting, Human Resources and Information Technology. While shared services has been around for more than three decades, commencing in the 1970’s in the banking sector and then been adopted by other sectors, it is an under researched domain, with little consensus on the most fundamental issues even as basic as defining what shared services is. Moreover, the interest in shared services within Higher Education is a global phenomenon. This study on shared services is situated within the Higher Education Sector of Malaysia, and originated as an outcome resulting from a national project (2005 – 2007) conducted by the Ministry of Higher Education (MOHE) entitled "Knowledge, Information Communication Technology Strategic Plan (KICTSP) for Malaysian Public Higher Education"- where progress towards more collaborations via shared services was a key recommendation. The study’s primary objective was to understand the nature and potential for ICT shared services, in particular in the Malaysian HE sector; by laying a foundation in terms of definition, typologies and research agenda and deriving theoretically based conceptualisations of the potential benefits of shared services, success factors and issues of pursuing shared services. The study embarked on this objective with a literature review and pilot case study as a means to further define the context of the study, given the current under-researched status of ICT shared services and of shared services in Higher Education. This context definition phase illustrated a range of unaddressed issues; including a lack of common understanding of what shared services are, how they are formed, what objectives they full fill, who is involved etc. The study thus embarked on a further investigation of a more foundational nature with an exploratory phase that aimed to address these gaps, where a detailed archival analysis of shared services literature within the IS context was conducted to better understand shared services from an IS perspective. The IS literature on shared services was analysed in depth to report on the current status of shared services research in the IS domain; in particular definitions, objectives, stakeholders, the notion of sharing, theories used, and research methods applied were analysed, which provided a firmer base to this study’s design. The study also conducted a detailed content analysis of 36 cases (globally) of shared services implementations in the HE sector to better understand how shared services are structured within the HE sector and what is been shared. The results of the context definition phase and exploratory phase formed a firm basis in the multiple case studies phase which was designed to address the primary goals of this study (as presented above). Three case sites within the Malaysian HE sector was included in this analysis, resulting in empirically supported theoretical conceptualizations of shared services success factors, issues and benefits. A range of contributions are made through this study. First, the detailed archival analysis of shared services in Information Systems (IS) demonstrated the dearth of research on shared services within Information Systems. While the existing literature was synthesised to contribute towards an improved understanding of shared services in the IS domain, the areas that are yet under-developed and requires further exploration is identified and presented as a proposed research agenda for the field. This study also provides theoretical considerations and methodological guidelines to support the research agenda; to conduct better empirical research in this domain. A number of literatures based a priori frameworks (i.e. on the forms of sharing and shared services stakeholders etc) are derived in this phase, contributing to practice and research with early conceptualisations of critical aspects of shared services. Furthermore, the comprehensive archival analysis design presented and executed here is an exemplary approach of a systematic, pre-defined and tool-supported method to extract, analyse and report literature, and is documented as guidelines that can be applied for other similar literature analysis, with particular attention to supporting novice researchers. Second, the content analysis of 36 shared services initiatives in the Higher Education sector presented eight different types of structural arrangements for shared services, as observed in practice, and the salient dimensions along which those types can be usefully differentiated. Each of the eight structural arrangement types are defined and demonstrated through case examples, with further descriptive details and insights to what is shared and how the sharing occurs. This typology, grounded on secondary empirical evidence, can serve as a useful analytical tool for researchers investigating the shared services phenomenon further, and for practitioners considering the introduction or further development of shared services. Finally, the multiple case studies conducted in the Malaysian Higher Education sector, provided further empirical basis to instantiate the conceptual frameworks and typology derived from the prior phases and develops an empirically supported: (i) framework of issues and challenges, (ii) a preliminary theory of shared services success, and (iii) a benefits framework, for shared services in the Higher Education sector.

Augmenting analytical CRM strategies with social BI

Large communities built around social media on the Internet offer an opportunity to augment analytical customer relationship management (CRM) strategies. The purpose of this paper is to provide direction to advance the conceptual design of business intelligence (BI) systems for implementing CRM strategies. After introducing social CRM and social BI as emerging fields of research, the authors match CRM strategies with a re-engineered conceptual data model of Facebook in order to illustrate the strategic value of these data. Subsequently, the authors design a multi-dimensional data model for social BI and demonstrate its applicability by designing management reports in a retail scenario. Building on the service blueprinting framework, the authors propose a structured research agenda for the emerging field of social BI.

The challenges of managing complexity in projects : an Australian perspective

Project Management (PM) as an academic field is relatively new in Australian universities. Moreover, the field is distributed across four main areas: business (management), built environment and construction, engineering and more recently ICT (information systems). At an institutional level, with notable exceptions, there is little engagement between researchers working in those individual areas. Consequently, an initiative was launched in 2009 to create a network of PM researchers to build a disciplinary base for PM in Australia. The initiative took the form of a bi-annual forum. The first forum established the constituency and spread of PM research in Australia (Sense et al., 2011). This special issue of IJPM arose out of the second forum, held in 2012, that explored the notion of an Australian perspective on PM. At the forum, researchers were invited to collaborate to explore issues, methodological approaches, and theoretical positions underpinning their research and to answer the question: is there a distinctly Australian research agenda which responds to the current challenges of large and complex projects in our region? From a research point of view, it was abundantly clear at the forum that many of the issues facing Australian researchers are shared around the world. However, what emerged from the forum as the Australian perspective was a set of themes and research issues that dominate the Australia research agenda.