Rituximab responsive immune thrombocytopenic purpura in an adult with underlying autoimmune lymphoproliferative syndrome due to a splice-site mutation (IVS7+2 T>C) affecting the Fas gene

A 36 yr-old man of Israeli descent with a history of childhood splenectomy for severe thrombocytopenia and a family history of autoimmune lymphoproliferative syndrome (ALPS), presented with severe immune thrombocytopenic purpura refractory to standard therapy. He was found to possess a heterozygous mutation in the Fas gene (also termed TNFRSF6, CD95, Apo-1) affecting the donor splice site of intron 7 (IVS7+2 T>C). This frameshift mutation truncates the cytoplasmic domain of the Fas death receptor, resulting in circulating CD4/8 double negative T lymphocytes, lymphadenopathy and autoimmune complications typical of ALPS. Administration of Rituximab in this patient was associated with a durable hematologic response (currently more than 12 months). This report highlights the need to consider rare inherited causes of thrombocytopenia in adults with a family history of immune cytopenia(s) and the effective use of anti-CD20 monoclonal antibody in patients unresponsive to immunosuppression and splenectomy

Minimum wage estimates and adjustments in Australia since 1983

This document provides estimates of national minimum wage rates for Australia since 1983. Providing estimates is not always a straight forward matter. We have provided a brief account of each wage decision providing: an estimated weekly minimum wage; an estimated hourly minimum wage based on the relevant standard working week; the relevant dollar increase; and the relevant percentage increase. We have also provided a summary of relevant events and submission from each decision in an endeavour to provide some of the social and political context for each decision

Numerical Optimisation of Building Thermal and Energy Performance in Hospitals

This thesis details the development and testing of a metamodel-based building optimisation methodology dubbed thermal building optimisation tool (T-BOT), designed as an information gathering framework and decision support tool rather than a design automator. Initial samples of building simulations are used to train moving least squares regression (MLSR) meta-models of the design space. A genetic algorithm (GA) is then used to optimise with the dual objectives of minimising time-averaged thermal discomfort and energy use. The optimum trade-off is presented as a Pareto front. Adaptive coupling functionality of the building simulation program ESP-r is used to augment the dynamic thermal model (DTM) with computational fluid dynamics (CFD), allowing local evaluation of thermal comfort within rooms. Furthermore, the disconnect between simulation and optimisation induced by the metamodeling is exploited to lend flexibility to the data gathered in the initial samples. Optimisations can hence be performed for any combination of location, time period, thermal comfort criteria and design variables, from a single set of sample simulations; this was termed a “one sample many optimisations” or OSMO approach. This can present substantial time savings over a comparable direct search optimisation technique. To the author’s knowledge the OSMO approach and adaptive coupling of DTM and CFD are unique among building thermal optimisation (BTO) models. Development and testing was focussed on hospital environments, though the method is potentially applicable to other environments. The program was tested by application to two models, one a theoretical test case and one a case study based on a real hospital building. It was found that variation in spatial location, time period and thermal comfort criteria can result in different optimum conditions, though seasonal variation had a large effect on this. Also the sample size and selection of design variables and their ranges were found to be critical to meta-model fidelity

Predicting the effect of changes to the urban environment on future electrical demand using building simulation and archetype models

Future urban electrical loads are of interest to a range of stakeholders from utilities to network planners. In this paper, a pragmatic approach to the modelling of urban electrical demands using archetype models and simulated building demand profiles is described. The profiles can be scaled, transformed and combined to produce time-series electrical loads for multiple buildings connected to a substation in a distribution network. The modelling approach has been verified against measured demand data. Possible changes in future peak urban electrical demand were quantified for a sample of substations in Glasgow, UK, using four future demand scenarios. The picture emerging was complex, with peak demand increasing in some cases where electric vehicles and electrified heating combine. However, there were many situations where a combination of improved energy efficiency and microgeneration lead to reduced peak demand

Quantitative magnetic resonance imaging in traumatic brain injury

PhD ThesisMild traumatic brain injury (TBI) may be complicated by long term cognitive and affective symptoms. Conventional imaging findings often do not correlate with the clinical picture in these patients, and frequently underestimate the extent of damage. Quantitative MR imaging techniques are sensitive to microstructural damage in brain grey matter (GM) and white matter (WM) which appear uninjured on conventional MRI. Previous work has predominantly evaluated their use in acute TBI in moderate and severely injured patients, or in chronic TBI across the severity spectrum. This thesis explored the application of quantitative T1 (qT1) and quantitative T2 (qT2) relaxometry and diffusion tensor imaging (DTI) in the acute evaluation of 44 mild and 9 moderate TBI patients in whom neuropsychological assessment had been performed, and compared the results to those of 30 matched control subjects. By combining the scan data with results from the cognitive testing, this work sought to identify correlations between regions of detectable microstructural damage and the neurocognitive functions related to them. Differences between groups were observed in whole brain normal appearing GM in qT1, and in frontal lobe normal appearing GM and WM in qT1 and DTI measures. Differences were also observed in memory performance and executive function between patients and control subjects which correlated with injury severity. Significant negative correlations were revealed between whole brain WM qT1 time and executive function and negative correlations were shown between frontal and left temporal GM qT1 time and both memory performance and phonemic fluency. Also demonstrated were a positive correlation between frontal GM MD and phonemic fluency, and a negative correlation between frontal GM FA and both memory and executive function. Lastly, increases in WM FA in the corpus callosum, corona radiata, superior longitudinal fasciculus and cingulum were shown to negatively correlate with all components of verbal fluency. This work has demonstrated, using quantitative MR imaging, acute differences at a microstructural level between TBI patients and matched control subjects, in tissue appearing normal on conventional imaging. Furthermore, it has shown that these changes correlate with post-concussive cognitive deficits. It is likely that these changes represent damage as a result of traumatic brain injury in the regions responsible for the cognitive functions found to be impaired.The Sir Jules Thorn Charitable Trus

Policies, programs, and practices: Exploring the complex dynamics of assessment education in teacher education across four countries

There has been a global trend toward increased accountability and assessment in schools over the past several decades. Across policy and professional standards, teachers have been repeatedly called to integrate assessment throughout their practice to identify, monitor, support, evaluate, and report on student learning. This professional capacity to integrate and utilize assessment to effectively facilitate student learning has long been characterized as teachers' “assessment literacy,” or more recently “assessment competency,” and “assessment capability”. Concerningly, research indicates that teachers generally maintain low levels of assessment knowledge and skills, with beginning teachers particularly underprepared for assessment in schools. This persistent finding is unsurprising as researchers argue that assessment has historically been a neglected area of study in teacher education programs. However, with the rise of accountability mandates, assessment is beginning to occupy a more prominent and necessary role in pre-service preparatory programs. However, analyzing and situating assessment education in relation to broader conceptions of assessment literacy remains necessary in order to effectively promote the assessment capability of beginning teachers. Likewise, understanding how assessment education and assessment literacy are shaped by the complex dynamics and larger teacher education frameworks and how they contribute to teachers' developing professional identities is essential in constructing a more comprehensive view of teacher preparation within and for accountability-driven systems of education. This paper analyzes teacher education policies, programs, and practices aimed at supporting initial teacher learning in assessment across four country contexts: Australia, Canada, England, and New Zealand. Bernstein's (1999) codes of classification and framing provide an analytic discourse for examining the vertical and horizontal messages about assessment that shape teacher capability in this key area of professional practice. In drawing on policy and teacher education documents and qualitative data (i.e., interview and teacher reflections) from across each country context, the paper concludes with five consistent and interconnected findings about the complex landscape for teacher preparation in assessment

The role of hand size in body representation:a developmental investigation

Knowledge of one’s own body size is a crucial facet of body representation, both for acting on the environment and perhaps also for constraining body ownership. However, representations of body size may be somewhat plastic, particularly to allow for physical growth in childhood. Here we report a developmental investigation into the role of hand size in body representation (the sense of body ownership, perception of hand position, and perception of own-hand size). Using the rubber hand illusion paradigm, this study used different fake hand sizes (60%, 80%, 100%, 120% or 140% of typical size) in three age groups (6- to 7-year-olds, 12- to 13-year-olds, and adults; N = 229). We found no evidence that hand size constrains ownership or position: participants embodied hands which were both larger and smaller than their own, and indeed judged their own hands to have changed size following the illusion. Children and adolescents embodied the fake hands more than adults, with a greater tendency to feel their own hand had changed size. Adolescents were particularly sensitive to multisensory information. In sum, we found substantial plasticity in the representation of own-body size, with partial support for the hypothesis that children have looser representations than adults.</p

Local pathways to low-carbon domestic heat : exploring the options in the UK

Currently, natural gas is the predominant source of domestic heat provision. Take-up of heat pumps and district heating remains at a minimal penetration of around 0.5%. In total, only around 2.5% of heat comes from low carbon sources, compared with more than 45% of electricity. As heat accounts for around 40% of UK energy consumption and 20% of GHG emissions, the decarbonisation of the heat sector is seen as vital for the UK to reach UK emission reduction targets. Different trajectories in heat provision using parallel energy vectors (electricity, gas, alternative gases, heat networks) imply a range of infrastructure impacts. In order to explore the form of different local energy systems under decarbonisation scenarios, this work seeks to: - Capture the broad forms of ’last-mile’ network: Urban, Suburban, Rural (on/off gas grid ) seen as exemplar of the UK energy system; - Downscale whole system-derived technology mixes and construct demonstrative local energy systems representing key use cases; - Using multi-carrier optimisation, determine the impacts of heat decarbonisation on current and future system actors

Simulation and implementation of heat load shifting in a low carbon building

A predictive load shifting control system for a heat pump has been developed and installed in a low carbon test house located at the BRE Innovation Park, Motherwell, near Glasgow. The house features an exhaust-air source heat pump supplying an under floor heating system. The controller predicted the day-ahead space heating requirements for the house, based on forecast air temperatures and solar radiation levels and then automatically set the heat pump’s start and stop times for the following day. The heat pump’s operation was restricted where possible to off-peak electricity tariff periods (00:00-07:00). The controller’s operating parameters were pre-set using a calibrated building simulation model. After installation, the controller’s performance was monitored during September 2015 and analysis of test data showed that the predictive control maintained indoor air temperatures between 18-23oC for around 87% of notional occupied hours between 07:00-22:00; this was better than predicted by simulation. However, the energy performance of the heat pump was extremely poor as it did not function well under intermittent load-shifting operation, with the majority of the heat was delivered primarily by an auxiliary immersion coil rather than the heat pump itself. The paper concludes with suggestions for refinements to the controller and further work