Visual function and CFH/ARMS2 risk genotypes in macular dystrophy due to maternally inherited diabetes and deafness

Maternally inherited diabetes and deafness (MIDD) is an autosomal dominant inherited syndrome caused by the mitochondrial DNA (mtDNA) nucleotide mutation A3243G. It affects various organs including the eye with external ophthalmoparesis, ptosis, and bilateral macular pattern dystrophy.1, 2 The prevalence of retinal involvement in MIDD is high, with 50% to 85% of patients exhibiting some macular changes.1 Those changes, however, can vary between patients and within families dramatically based on the percentage of retinal mtDNA mutations, making it difficult to give predictions on an individual’s visual prognosis...

Exploring the experiences of embedding Indigenous knowledge and perspectives on teaching practicum through interpretive phenomenology

In this paper, we discuss interpretive/hermeneutic phenomenology as a theoretical approach to explore the experiences of three stakeholder groups in embedding Indigenous knowledge and perspectives on teaching practicum, a project sponsored by ALTC. We begin by asking the phenomenological question ‘what is your experience of practice teaching?’ An open, explorative, phenomenological framework seeks the meanings of experiences, not truths, from the participants’ words themselves. Interpretive phenomenology is particularly suitable to explore educational experiences (Grumet, 1992; M. van Manen, 1990), as it provides rich ground for listening to the stakeholders’ lived experience and documenting it for interpretation. In an interpretive process, perspectives on lifeworlds, worldview and lenses get highlighted (Cunningham & Stanley, 2003). We establish how through various project stages, interpretive phenomenology gets to the essence of practice teaching experience creating a pedagogical ‘understanding’ of the essential nature of shared experience as lived by the participants (M van Manen, 2002). Thereby, it foregrounds voices of agency, dissent, acceptance and resistance. We consider how our research study focuses on the pedagogic voice of Indigenous pre-service teachers and the recognition of complex pedagogic fields in Indigenous education. We explain how this study seeks insights into their evaluation of pedagogic relations with two other education stakeholders – their practicum supervising teachers at schools and university staff involved practicum experience. As such, our study aims to support and develop long term, future-oriented opportunities for Indigenous pre-service teachers to embed Indigenous knowledge in the curricula. We conclude with some projections into the discourse on how Indigenous knowledge (IK) and perspectives might be diversely exemplified in pre-service teachers’ professional works (particularly E-portfolios). We speculate how this change could in turn maximise opportunities for Indigenous pre-service teachers, their supervising teachers and university staff to demonstrate leadership in their field through the creation of future tangible products such as units of work, resources, assessment and reflection tools. The processes contextualising the cultural interface of competing knowledge systems (Nakata, 2007) provide important analytical tools for understanding issues affecting student-teacher-mentor relationships occurring on practicum.

Antibiotics for bronchiectasis exacerbations in children: rationale and study protocol for a randomised placebo-controlled trial

Background Despite bronchiectasis being increasingly recognised as an important cause of chronic respiratory morbidity in both indigenous and non-indigenous settings globally, high quality evidence to inform management is scarce. It is assumed that antibiotics are efficacious for all bronchiectasis exacerbations, but not all practitioners agree. Inadequately treated exacerbations may risk lung function deterioration. Our study tests the hypothesis that both oral azithromycin and amoxicillin-clavulanic acid are superior to placebo at improving resolution rates of respiratory exacerbations by day 14 in children with bronchiectasis unrelated to cystic fibrosis. Methods We are conducting a bronchiectasis exacerbation study (BEST), which is a multicentre, randomised, double-blind, double-dummy, placebo-controlled, parallel group trial, in five centres (Brisbane, Perth, Darwin, Melbourne, Auckland). In the component of BEST presented here, 189 children fulfilling inclusion criteria are randomised (allocation-concealed) to receive amoxicillin-clavulanic acid (22.5 mg/kg twice daily) with placebo-azithromycin; azithromycin (5 mg/kg daily) with placebo-amoxicillin-clavulanic acid; or placebo-azithromycin with placebo-amoxicillin-clavulanic acid for 14 days. Clinical data and a paediatric cough-specific quality of life score are obtained at baseline, at the start and resolution of exacerbations, and at day 14. In most children, blood and deep nasal swabs are also collected at the same time points. The primary outcome is the proportion of children whose exacerbations have resolved at day 14. The main secondary outcome is the paediatric cough-specific quality of life score. Other outcomes are time to next exacerbation; requirement for hospitalisation; duration of exacerbation; and spirometry data. Descriptive viral and bacteriological data from nasal samples and blood markers will also be reported. Discussion Effective, evidence-based management of exacerbations in people with bronchiectasis is clinically important. Yet, there are few randomised controlled trials (RCTs) in the neglected area of non-cystic fibrosis bronchiectasis. Indeed, no published RCTs addressing the treatment of bronchiectasis exacerbations in children exist. Our multicentre, double-blind RCT is designed to determine if azithromycin and amoxicillin-clavulanic acid, compared with placebo, improve symptom resolution on day 14 in children with acute respiratory exacerbations. Our planned assessment of the predictors of antibiotic response, the role of antibiotic-resistant respiratory pathogens, and whether early treatment with antibiotics affects duration and time to the next exacerbation, are also all novel.

Bronchiectasis exacerbation study on azithromycin and amoxycillin-clavulanate for respiratory exacerbations in children (BEST-2) : study protocol for a randomized controlled trial

Background Bronchiectasis unrelated to cystic fibrosis (CF) is being increasingly recognized in children and adults globally, both in resource-poor and in affluent countries. However, high-quality evidence to inform management is scarce. Oral amoxycillin-clavulanate is often the first antibiotic chosen for non-severe respiratory exacerbations, because of the antibiotic-susceptibility patterns detected in the respiratory pathogens commonly associated with bronchiectasis. Azithromycin has a prolonged half-life, and with its unique anti-bacterial, immunomodulatory, and anti-inflammatory properties, presents an attractive alternative. Our proposed study will test the hypothesis that oral azithromycin is non-inferior (within a 20% margin) to amoxycillin-clavulanate at achieving resolution of non-severe respiratory exacerbations by day 21 of treatment in children with non-CF bronchiectasis. Methods This will be a multicenter, randomized, double-blind, double-dummy, placebo-controlled, parallel group trial involving six Australian and New Zealand centers. In total, 170 eligible children will be stratified by site and bronchiectasis etiology, and randomized (allocation concealed) to receive: 1) azithromycin (5 mg/kg daily) with placebo amoxycillin-clavulanate or 2) amoxycillin-clavulanate (22.5 mg/kg twice daily) with placebo azithromycin for 21 days as treatment for non-severe respiratory exacerbations. Clinical data and a parent-proxy cough-specific quality of life (PC-QOL) score will be obtained at baseline, at the start and resolution of exacerbations, and on day 21. In most children, blood and deep-nasal swabs will also be collected at the same time points. The primary outcome is the proportion of children whose exacerbations have resolved at day 21. The main secondary outcome is the PC-QOL score. Other outcomes are: time to next exacerbation; requirement for hospitalization; duration of exacerbation, and spirometry data. Descriptive viral and bacteriological data from nasal samples and blood inflammatory markers will be reported where available. Discussion Currently, there are no published randomized controlled trials (RCT) to underpin effective, evidence-based management of acute respiratory exacerbations in children with non-CF bronchiectasis. To help address this information gap, we are conducting two RCTs. The first (bronchiectasis exacerbation study; BEST-1) evaluates the efficacy of azithromycin and amoxycillin-clavulanate compared with placebo, and the second RCT (BEST-2), described here, is designed to determine if azithromycin is non-inferior to amoxycillin-clavulanate in achieving symptom resolution by day 21 of treatment in children with acute respiratory exacerbations. Trial registration Australia and New Zealand Clinical Trials Register (ANZCTR) number ACTRN12612000010897. http://www.anzctr.org.au/trial_view.aspx?id=347879

Does a 10-valent pneumococcal-Haemophilus influenzae protein D conjugate vaccine prevent respiratory exacerbations in children with recurrent protracted bacterial bronchitis, chronic suppurative lung disease and bronchiectasis : protocol for a randomised controlled trial

Background Recurrent protracted bacterial bronchitis (PBB), chronic suppurative lung disease (CSLD) and bronchiectasis are characterised by a chronic wet cough and are important causes of childhood respiratory morbidity globally. Haemophilus influenzae and Streptococcus pneumoniae are the most commonly associated pathogens. As respiratory exacerbations impair quality of life and may be associated with disease progression, we will determine if the novel 10-valent pneumococcal-Haemophilus influenzae protein D conjugate vaccine (PHiD-CV) reduces exacerbations in these children. Methods A multi-centre, parallel group, double-blind, randomised controlled trial in tertiary paediatric centres from three Australian cities is planned. Two hundred six children aged 18 months to 14 years with recurrent PBB, CSLD or bronchiectasis will be randomised to receive either two doses of PHiD-CV or control meningococcal (ACYW(135)) conjugate vaccine 2 months apart and followed for 12 months after the second vaccine dose. Randomisation will be stratified by site, age (<6 years and >= 6 years) and aetiology (recurrent PBB or CSLD/bronchiectasis). Clinical histories, respiratory status (including spirometry in children aged >= 6 years), nasopharyngeal and saliva swabs, and serum will be collected at baseline and at 2, 3, 8 and 14 months post-enrolment. Local and systemic reactions will be recorded on daily diaries for 7 and 30 days, respectively, following each vaccine dose and serious adverse events monitored throughout the trial. Fortnightly, parental contact will help record respiratory exacerbations. The primary outcome is the incidence of respiratory exacerbations in the 12 months following the second vaccine dose. Secondary outcomes include: nasopharyngeal carriage of H. influenzae and S. pneumoniae vaccine and vaccine-related serotypes; systemic and mucosal immune responses to H. influenzae proteins and S. pneumoniae vaccine and vaccine-related serotypes; impact upon lung function in children aged >= 6 years; and vaccine safety. Discussion As H. influenzae is the most common bacterial pathogen associated with these chronic respiratory diseases in children, a novel pneumococcal conjugate vaccine that also impacts upon H. influenzae and helps prevent respiratory exacerbations would assist clinical management with potential short- and long-term health benefits. Our study will be the first to assess vaccine efficacy targeting H. influenzae in children with recurrent PBB, CSLD and bronchiectasis.

Rates of radiologically confirmed pneumonia as defined by the World Health Organization in Northern Territory Indigenous children

Objective To determine the burden of hospitalised, radiologically confirmed pneumonia (World Health Organization protocol) in Northern Territory Indigenous children. Design, setting and participants Historical, observational study of all hospital admissions for any diagnosis of NT resident Indigenous children, aged between >= 29 days and < 5 years, 1 April 1997 to 31 March 2005. Intervention All chest radiographs taken during these admissions, regardless of diagnosis, were assessed for pneumonia in accordance with the WHO protocol. Main outcome measure The primary outcome was endpoint consolidation (dense fluffy consolidation [alveolar infiltrate] of a portion of a lobe or the entire lung) present on a chest radiograph within 3 days of hospitalisation. Results We analysed data on 24 115 hospitalised episodes of care for 9492 children and 13 683 chest radiographs. The average annual cumulative incidence of endpoint consolidation was 26.6 per 1000 population per year (95% Cl, 25.3-27.9); 57.5 per 1000 per year in infants aged 1-11 months, 38.3 per 1000 per year in those aged 12-23 months, and 13.3 per 1000 per year in those aged 24-59 months. In all age groups, rates of endpoint consolidation in children in the arid southern region of NT were about twice that of children in the tropical northern region. Conclusion The rates of severe pneumonia in hospitalised NT Indigenous children are among the highest reported in the world. Reducing this unacceptable burden of disease should be a national health priority.

A snapshot of 40 years in early childhood education and care through oral histories

The year 2012 marked 40 years since the introduction of the Child Care Act 1972 and the federal government introduced financial support for the provision of child care services in Australia. Significant changes have occurred in social, political and theoretical contexts of early childhood education and care (ECEC) during this time. Bringing these to life, this paper investigates archival data of key changes in ECEC in association with oral histories of staff, parents and children associated with The Gowrie Qld during the years 1972‒2012. With narrative analysis considered alongside historical information, two dominant issues emerge as integral to ECEC in the past, now and the future. These are: 1) what constitutes effective teaching and learning in the educational program and 2) professional expectations in ECEC. Building an historical picture, this paper provides for critical reflection on the past to inform current and future practices.

Reconciling dilemmas of social justice in literacy lessons : a case study of preservice primary teachers

Literacy is promoted as one factor in overcoming disadvantage. In this paper, we employ Fraser’s (1997 & 2008) framing of social justice in order to analyse the disparate agendas of literacy education for improved outcomes in national policy. We do this to better understand the dilemmas confronting preservice teachers as they prepare to become teachers in complex education contexts. We then examine what 20 preservice primary teachers say about social justice in interview responses to a scripted scenario. Our findings demonstrate that most preservice teachers are trying to demonstrate that they have a well-placed commitment to teaching for social justice, however, most of our respondents are yet to frame productive practices that might work in providing socially just education for the students they will teach. These outcomes raise possibilities for future iterations of preservice teacher courses at the case study site and beyond.

Talk in activity during young children’s use of digital technologies at home

Internet-connected tablets and smart phones are being used increasingly by young children. Little is known, however, about their social interactions with family members when engaged with these technologies. This article examines video-recorded interactions between a father and his two young children, one aged 18 months using an iPhone and one aged three years accessing an iPad. Drawing on Ethnomethodology and Conversation Analysis, analysis establishes ways the family members engage and disengage in talk so as to manage their individual activity with mobile devices and accomplish interaction with each other. Findings are relevant for understanding children’s everyday practices with mobile technologies.

Work integrated learning: Exposure to professional practice : expectations and challenges

Prior to graduation engineering students are expected to provide evidence of relevant experience in the workplace. This experience is expected to provide opportunities for exposure to the profession and to help students develop confidence, skills and capabilities as emerging professionals. This investigation considers the expectations and challenges in implementing WIL programs in different contexts. While this will inform the next iteration of engineering course development at QUT the issues and interventions described provide useful insights into options available and engineering curriculum design more broadly. This comparative analysis across three phases highlights expectations and challenges including stakeholder responsibilities, expectations, and assessment. The study draws on the findings of a 2005 investigation into the purpose and provision of WIL and findings of a 2012 Faculty review of the current WIL model. The enhancement of WIL through a series of developmental phases highlights strengths and weaknesses of various models. It is anticipated that this investigation will inform course development decisions on a whole-of-course approach to WIL that improves student engagement and learning experience. The importance of WIL is not disputed. However with industry expectations, increasing student numbers and cohort diversity the ways in which students and industry currently engage in WIL are not sustainable and more creative, flexible and engaging approaches are needed.

Creating efficiencies in teaching : where are the right resources when you need them?

Early career engineering academics are encouraged to join and contribute to established research groups at the leading edge of their discipline. This is often facilitated by various staff development and support programs. Given that academics are often appointed primarily on the basis of their research skills and outputs, such an approach is justified and is likely to result in advancing the individual academic’s career. It also enhances their capacity to attract competitive research funding, while contributing to the overall research performance of their institution, with further potential for an increased share of government funding. In contrast, there is much less clarity of direction or availability of support mechanisms for those academics in their role as teachers. Following a general induction to teaching and learning at their institution, they would commonly think about preparing some lecture materials, whether for delivery in a face-to-face or on-line modality. Typically they would look for new references and textbooks to act as a guide for preparing the content. They would probably find out how the course has been taught before, and what laboratory facilities and experiments have been used. In all of these and other related tasks, the majority of newly appointed academics are guided strongly by their own experiences as students, rather than any firm knowledge of pedagogical principles. At a time of increased demands on academics’ time, and high expectations of performance and productivity in both research and teaching, it is essential to examine possible actions to support academics in enhancing their teaching performance in effective and efficient ways. Many resources have been produced over the years in engineering schools around the world, with very high intellectual and monetary costs. In Australia, the last few years have seen a surge in the number of ALTC/OLT projects and fellowships addressing a range of engineering education issues and providing many resources. There are concerns however regarding the extent to which these resources are being effectively utilised. Why are academics still re-inventing the wheel and creating their own version of teaching resources and pedagogical practice? Why do they spend so much of their precious time in such an inefficient way? A symposium examining the above issues was conducted at the AAEE2012 conference, and some pointers to possible responses to the above questions were obtained. These are explored in this paper and supplemented by the responses to a survey of a group of engineering education leaders on some of the aspects of these research questions. The outcomes of the workshop and survey results have been analysed in view of the literature and the ALTC/OLT sponsored learning and teaching projects and resources. Other factors are discussed, including how such resources can be found, how their quality might be evaluated, and how assessment may be appropriately incorporated, again using readily available resources. This study found a strong resonance between resources reuse with work on technology acceptance (Davis, 1989), suggesting that technology adoption models could be used to encourage resource sharing. Efficient use of outstanding learning materials is an enabling approach. The paper provides some insights on the factors affecting the re-use of available resources, and makes some recommendations and suggestions on how the issue of resources re-use might be incorporated in the process of applying and completing engineering education projects.

Inside the pleasure dome : cultural tourism on Australia’s Gold Coast

In 2018 the City of the Gold Coast in south-east Queensland, Australia, will host the next Commonwealth Games. The City is made up a 57 km stretch of coastline and hinterland divided by a major highway. The famous surfing beaches are framed by high-rise development while the hinterland is marketed as a green, unspoilt environment. The winning bid for the Games, and discussion about future infrastructure and marketing of the region’s attributes, has focussed attention on the way City residents and policy makers think about their region in broad terms. Whereas in the past tourism marketing has been directed towards the pleasures of sun and surf by day and bright lights by night, various regional tourist stakeholders are beginning to reorient their programs. This paper considers some of the competing aims of the various stakeholders in this region and the interaction of existing ‘cultures’ with new technology and the demands of permanent residents, using data from a case study of e-literary trails developed in Brisbane, the capital city of Queensland. The importance of tourist imaginaries as a basis for using rich accounts of the past for future planning is emphasized.