Surgical fusion of early onset severe scoliosis increases survival in Rett syndrome: A cohort study

Aim Scoliosis is a common co-morbidity in Rett syndrome and spinal fusion may be recommended if severe. We investigated the impact of spinal fusion on survival and risk of severe lower respiratory tract infection in Rett syndrome. Method Data were ascertained from hospital medical records, the Australian Rett Syndrome Database, a longitudinal and population-based registry, and from the Australian Institute of Health and Welfare National Death Index database. Cox regression and generalized estimating equation models were used to estimate the effects of spinal surgery on survival and severe respiratory infection respectively in 140 females who developed severe scoliosis (Cobb angle ≥45°) before adulthood. Results After adjusting for mutation type and age of scoliosis onset, the rate of death was lower in the surgery group (hazard ratio [HR] 0.30, 95% confidence interval [CI] 0.12–0.74; p=0.009) compared to those without surgery. Rate of death was particularly reduced for those with early onset scoliosis (HR 0.17, 95% CI 0.06–0.52; p=0.002). There was some evidence to suggest that spinal fusion was associated with a reduction in risk of severe respiratory infection among those with early onset scoliosis (risk ratio 0.41, 95% CI 0.16–1.03; p=0.06). Interpretation With appropriate cautions, spinal fusion confers an advantage to life expectancy in Rett syndrome.

School policies on bullying and cyberbullying: Perspectives across three Australian states

Background Despite decades of research, bullying in all its forms is still a significant problem within schools in Australia, as it is internationally. Anti-bullying policies and guidelines are thought to be one strategy as part of a whole school approach to reduce bullying. However, although Australian schools are required to have these policies, their effectiveness is not clear. As policies and guidelines about bullying and cyberbullying are developed within education departments, this paper explores the perspectives of those who are involved in their construction. Purpose This study examined the perspectives of professionals involved in policy construction, across three different Australian states. The aim was to determine how their relative jurisdictions define bullying and cyberbullying, the processes for developing policy, the bullying prevention and intervention recommendations given to schools and the content considered essential in current policies. Sample Eleven key stakeholders from three Australian states with similar education systems were invited to participate. The sample selection criteria included professionals with experience and training in education, cyber-safety and the responsibility to contribute to or make decisions which inform policy in this area for schools in their state. Design and methods Participants were interviewed about the definitions of bullying they used in their state policy frameworks; the extent to which cyberbullying was included; and the content they considered essential for schools to include in anti-bullying policies. Data were collected through in-depth, semi-structured interviews and analysed thematically. Findings Seven themes were identified in the data: - (1) Definition of bullying and cyberbullying; - (2) Existence of a policy template; - (3) Policy location; - (4) Adding cyberbullying; - (5) Distinguishing between bullying and cyberbullying; - (6) Effective policy, and; - (7) Policy as a prevention or intervention tool. The results were similar both across state boundaries and also across different disciplines. Conclusion Analysis of the data suggested that, across the themes, there was some lack of information about bullying and cyberbullying. This limitation could affect the subsequent development, dissemination and sustainability of school anti-bullying policies, which have implications for the translation of research to inform better student outcomes.

PolyPatEx : an R package for paternity exclusion in autopolyploids

Microsatellite markers have demonstrated their value for performing paternity exclusion and hence exploring mating patterns in plants and animals. Methodology is well established for diploid species, and several software packages exist for elucidating paternity in diploids; however, these issues are not so readily addressed in polyploids due to the increased complexity of the exclusion problem and a lack of available software. We introduce polypatex, an r package for paternity exclusion analysis using microsatellite data in autopolyploid, monoecious or dioecious/bisexual species with a ploidy of 4n, 6n or 8n. Given marker data for a set of offspring, their mothers and a set of candidate fathers, polypatex uses allele matching to exclude candidates whose marker alleles are incompatible with the alleles in each offspring–mother pair. polypatex can analyse marker data sets in which allele copy numbers are known (genotype data) or unknown (allelic phenotype data) – for data sets in which allele copy numbers are unknown, comparisons are made taking into account all possible genotypes that could arise from the compared allele sets. polypatex is a software tool that provides population geneticists with the ability to investigate the mating patterns of autopolyploids using paternity exclusion analysis on data from codominant markers having multiple alleles per locus.

HealthMap: A cluster randomised trial of interactive health plans and self-management support to prevent coronary heart disease in people with HIV

Background The leading causes of morbidity and mortality for people in high-income countries living with HIV are now non-AIDS malignancies, cardiovascular disease and other non-communicable diseases associated with ageing. This protocol describes the trial of HealthMap, a model of care for people with HIV (PWHIV) that includes use of an interactive shared health record and self-management support. The aims of the HealthMap trial are to evaluate engagement of PWHIV and healthcare providers with the model, and its effectiveness for reducing coronary heart disease risk, enhancing self-management, and improving mental health and quality of life of PWHIV. Methods/Design The study is a two-arm cluster randomised trial involving HIV clinical sites in several states in Australia. Doctors will be randomised to the HealthMap model (immediate arm) or to proceed with usual care (deferred arm). People with HIV whose doctors are randomised to the immediate arm receive 1) new opportunities to discuss their health status and goals with their HIV doctor using a HealthMap shared health record; 2) access to their own health record from home; 3) access to health coaching delivered by telephone and online; and 4) access to a peer moderated online group chat programme. Data will be collected from participating PWHIV (n = 710) at baseline, 6 months, and 12 months and from participating doctors (n = 60) at baseline and 12 months. The control arm will be offered the HealthMap intervention at the end of the trial. The primary study outcomes, measured at 12 months, are 1) 10-year risk of non-fatal acute myocardial infarction or coronary heart disease death as estimated by a Framingham Heart Study risk equation; and 2) Positive and Active Engagement in Life Scale from the Health Education Impact Questionnaire (heiQ). Discussion The study will determine the viability and utility of a novel technology-supported model of care for maintaining the health and wellbeing of people with HIV. If shown to be effective, the HealthMap model may provide a generalisable, scalable and sustainable system for supporting the care needs of people with HIV, addressing issues of equity of access. Trial registration Universal Trial Number (UTN) U111111506489; ClinicalTrial.gov Id NCT02178930 submitted 29 June 2014

In-training assessment - Its potential in enhancing clinical teaching

Context In-training assessment (ITA) has established its place alongside formative and summative assessment at both the undergraduate and postgraduate level. In this paper the authors aimed to identify those characteristics of ITA that could enhance clinical teaching. Methods A literature review and discussions by an expert working group at the Ninth Cambridge Conference identified the aspects of ITA that could enhance clinical teaching. Results The features of ITA identified included defining the specific benefits to the learner, teacher and institution, and highlighting the patient as the context for ITA and clinical teaching. The ‘mapping’ of a learner’s progress towards the clinical teaching objectives by using multiple assessments over time, by multiple observers in both a systematic and opportunistic way correlates with the incremental nature of reaching clinical competence. Conclusions The importance of ITA based on both direct and indirect evidence of what the learner actually does in the real clinical setting is emphasized. Particular attention is given to addressing concerns in the more controversial areas of assessor training, ratings and documentation for ITA. Areas for future research are also identified.