Longitudinal observational study investigating outcome measures for clinical trials in inclusion body myositis.

OBJECTIVE: To describe decline in muscle strength and physical function in patients with sporadic inclusion body myositis (IBM). METHODS: Manual muscle testing (MMT), quantitative muscle testing (QMT) and disability scoring using the IBM Functional Rating Scale (IBMFRS) were undertaken for 181 patients for up to 7.3 years. The relationship between MMT, QMT and IBMFRS composite scores and time from onset were examined using linear mixed effects models adjusted for gender and age of disease onset. Adaptive LASSO regression analysis was used to identify muscle groups that best predicted the time elapsed from onset. Cox proportional hazards regression was used to evaluate time to use of a mobility aid. RESULTS: Multilevel modelling of change in percentage MMT, QMT and IBMFRS score over time yielded an average decline of 3.7% (95% CI 3.1% to 4.3%), 3.8% (95% CI 2.7% to 4.9%) and 6.3% (95% CI 5.5% to 7.2%) per year, respectively. The decline, however, was not linear, with steeper decline in the initial years. Older age of onset was associated with a more rapid IBMFRS decline (p=0.007), but did not influence the rate of MMT/QMT decline. Combination of selected muscle groups allowed for generation of single measures of patient progress (MMT and QMT factors). Median (IQR) time to using a mobility aid was 5.4 (3.6-9.2) years, significantly affected by greater age of onset (HR 1.06, 95% CI 1.04 to 1.09, p<0.001). CONCLUSION: This prospective observational study represents the largest IBM cohort to date. Measures of patient progress evaluated in this study accurately predict disease progression in a reliable and useful way to be used in trial design

Comprehension as social and intellectual practice: Rebuilding curriculum in low socioeconomic and cultural minority schools

This article reframes the concept of comprehension as a social and intellectual practice. It reviews current approaches to reading instruction for linguistically and culturally diverse and low socioeconomic students, noting an emphasis on comprehension as autonomous skills. The Four Resources model (Freebody &amp; Luke, 1990) is used to make the case for the integration of comprehension instruction with an emphasis on student cultural and community knowledge, and substantive intellectual and sociocultural content in elementary school curricula. Illustrations are drawn from research underway on the teaching of literacy in primary schools in low SES communities

Literacy practices of primary education children in Andalusia (Spain): a family-based perspective

Primary school children develop literacy practices in various domains and situations in everyday life. This study focused on the analysis of literacy practices of children aged 8–12 years from the perspec- tive of their families. 1,843 families participated in the non-experimental explanatory study. The children in these families speak Spanish as a first language and are schooled in this language. The instrument used was a self-report questionnaire about children’s home-literacy practices. The data obtained were analysed using categorical principal components analysis (CATPCA) and analysis of variance (ANOVA). The results show the complex relationship between literacy practices developed by children in the domains of home and school and the limited development of a literacy-promoting ‘third space’. In conclusion, the families in our study had limited awareness of their role as literacy- promoting agents and thought of literacy learning as restricted to formal or academic spaces

Adult North Star Network (ANSN): Consensus Document for Therapists Working with Adults with Duchenne Muscular Dystrophy (DMD) - Therapy Guidelines

BACKGROUND The survival of people with Duchenne Muscular Dystrophy (DMD) significantly increased due to improvements in standards of care (SOC) [1]. Consequently, DMD has evolved from a paediatric disease to a severe, chronic, multisystem, adult condition. The published international standards of care advocate specialist multidisciplinary health monitoring through proactive, anticipatory approaches to slow down the effects of the disease and allow advanced, informed decision-making [1–3]. Therapy starts as soon as the diagnosis is made and plays a vital role in symptom management in individuals to improve function, participation and effective quality of life. Therapy interventions for management, differ depending on the setting in which the care is being provided, specifically in terms of the expertise within the teams and resources available within these settings. People with DMD find that when they transition to adult services there is a dearth of expertise and limited access to therapy services. The survey conducted in the UK highlighted substantial differences between the care received by adults and children with the condition [2]. A large proportion of adults with DMD reported increased difficulties with access to professional physiotherapy, particularly at transition from childhood to adulthood. Additionally, having their functional abilities assessed regularly or receiving professional physiotherapy in general were both significantly more difficult to achieve within adult services in the UK. Furthermore, some of the major problems expressed by adults with DMD were mobility and transportation as well as, getting involved in leisure activities and work [3]. Therefore, while pediatric services are predominantly family-centred, after transition the paradigm of patient care changes towards individual-centred with focus on different therapy goals. Those become more tailored to the individuals’ needs, balancing quality of life and management options.This document is aimed at providing guidelines for physiotherapy, occupational therapy and speech and language considerations. The ‘Adult North Star Network’ (ANSN) was founded in 2015 to advance care of adults with DMD living in the UK and to develop a prospective natural history database. There are currently 28 adult centres within the network, caring for at least 700 DMD patients. Transition age is varied depending on services and is generally between the ages of 16 to 18. There is a wide range of severity affecting people with DMD transitioned to adult services, those who are steroid naive will have been permanent wheelchair users for many years and have profound muscle weakness. On the other hand, steroid treated patients will most commonly have good upper limb function, and some maybe ambulant at the time of transition. Additionally the specific type of genetic mutation, compliance to therapy and environmental factors may play a role in disease progression and presentation at transition. The aim of these guidelines is to support therapists working with adults with DMD with little or no experience to assist their clinical practice. Whilst the recommendations can be adopted by other health care systems in the world, we appreciate it will depend on resource availability

Pre-service teachers linking their metalinguistic knowledge to their practice: A functional approach

Existing work in Anglophone countries has raised concerns regarding teachers’ knowledge about language (KAL); this may well be an issue in other countries also, with notable exceptions such as Finland. In Australia, with the introduction of the new Australian Curriculum, the question of teacher KAL has become crucial. Teachers, both practising and pre-service, generally have some knowledge about language as an object, usually including the text structures of particular school genres and information about sentence structure and word classes. This knowledge may be based on traditional grammar and may not be well applied above the sentence level. Teachers may also have an intuitive knowledge of discourse structures and are beginning to reflect on their own discourse using understandings of dialogic teaching. This paper provides an example of how first-year pre-service teachers (PSTs) were introduced to KAL at both the grammatical and the discourse levels, as part of an introductory unit on spoken language. A range of approaches was used, including a functional view of discourse. The PSTs then applied their KAL by putting it into a context that was meaningful for them: discussing their own practice. The paper gives an illustration of some of the work they produced that demonstrates their emerging understandings

The rise of non-dissertation track master’s programmes: an academic literacies approach

Although dissertations have traditionally been part of master’s programmes, non-dissertation track master’s are on the rise across the world. This paper reports faculty views on MA TESOL dissertations (Teaching English to Speakers of Other Languages) in five Anglophone countries. Drawing on the academic literacies tradition and informed by Bourdieusian concepts of ‘field’, ‘habitus’, and ‘symbolic violence’, the results suggest that the need for more student intake has affected the presence or absence of the dissertation component in MA TESOL programmes

Am I just not good enough? The creation, development and questioning of a high performance coaching identity

While the career experiences and trajectories of various sports workers have received increased scholarly attention, those of professional coaches have, in comparison, received scant consideration. This paper focuses on the career experiences of Maeve (a pseudonym), a high performance coach, and the critical incidents related to the creation, development, and, ultimately, questioning of her professional identity. Data were collected through a series of narrative-biographical interviews and were subject to a process of iterative data analysis. The results indicated that her significant investment into her coaching self, combined with the vagaries and uncertain nature of work in high performance coaching, led her to experience a biographical disruption that interrupted the narrative coherence of her coaching life. The findings add further credence to recent critiques of only understanding and representing coaching careers in a linear and chronically staged fashion

Stochastic phase portraits of a damped bistable oscillator driven by colored noise.

We present measurements of the stationary, joint statistical densities of velocity and displacement in a three-dimensional system: the damped oscillator with a bistable potential driven by additive, colored noise. Approximate Fokker-Planck theories of such "higher-dimensional" systems are assessed and compared to the measurements. Because of limitations inherent in all present theories and generally available numerical techniques, analog simulators appear to offer the only effective, high-resolution means for obtaining the joint probability densities of d = 3 systems

Coaches as sport workers: professional agency within the employment context of elite European basketball

Increased activity of multiple stakeholders (e.g. agents and owners) have created new challenges for some coaches working in professional sports clubs. The purpose of this project was to draw attention to the normative or accepted practices inherent in sport work, some of the day-to-day realities of some coaches working in this context, and to understand how coaches' perceptions of other stakeholders come to bear on their individual circumstances, career expectations/objectives and professional agency. Data were generated from semi-structured interviews with seven professional basketball coaches who worked in top-level European clubs. The analysis reveals the coach's relationships between some owners and agents differed with respect to exercising professional agency, and, coach's decisions and actions were tied to their professional ideals as well as understandings of what they need to undertake their work effectively and negotiation and/or adjustment strategies. Occasionally coach's work practices could be viewed as antithetical to employment security, however, the presence of insecurity was at times embraced and used strategically to affect workers' career decisions. Amid contemporary regional geo-political shifts, this work aids examinations of global sport settings, structures and issues that may contour sporting professionals' live

Reading with drama: relations between texts, readers and experiences

This article explores the intersections of drama and reading, specifically focusing on approaches that are situated within “drama in education.” Supported with a retrospective analysis, this article portrays the research, related practice and possible futures in drama education in relation to literacy and in particular to reading fiction as meaning making practice. This study is situated in a reassertion of the value of relational literacies through imaginative practices that dramatic modes generate and support. The article disrupts common misconceptions about the purposes and effects of drama in reading and establishes prominent research discourses and definitions across the history of drama and reading practices. By locating paradigmatic and practical opportunities in our analysis of contemporary research, we bring visibility to the intricacies of drama in education as a generative pedagogy in reading as relational meaning making work