Craniometaphyseal and craniodiaphyseal dysplasia, head and neck manifestations and management

Craniometaphyseal and craniodiaphyseal dysplasia are rare genetic disorders of bone due to modelling errors of long bones and skull bones. These syndromes present with multiple ENT symptomatology from an early age. The diagnostic distinction can now be made radiologically by serial skeletal survey which is important for prognosis. We review the clinical, radiological, computed tomography (CT) scan, otological, audiological and histopathological findings in two cases with craniodiaphyseal, and two cases with craniometaphyseal dysplasia, and report our experiences of medical and surgical treatment to date. In the craniodiaphyseal dysplasia, the hearing abnormality progressed from an initial conductive to a mixed loss on serial audiometric follow up. Temporal bone CT scans showed narrowing of the middle ear cavity, internal auditory meatus, and facial nerve canal at the geniculate ganglion. Benefits from choanal stenosis surgery, craniofacial remodelling and dacrocystorhinostomy were shortlived. Calcitriol therapy with a low calcium diet did not alter the clinical course of progression in our cases. The underlying defect, causing net bone formation in these phenotypically similar syndromes, appears to be different when based on the differing biochemical responses to calcitriol and bone biopsy findings. Increased numbers of osteoblasts were found in bone biopsies from both cases with craniodiaphyseal dysplasia. Early recognition is crucial in these conditions as therapy directed at the underlying bony defect has the best chance of success if initiated in infancy (Cole et al., 1988; Fanconi et al., 1988; Key et al., 1988)

“I want to do it all day!” – Students’ experiences of classroom movement integration

Whole-of-school physical activity programming is being advocated internationally, with movement integration in the classroom considered an effective method to increase physical activity throughout the school day. Students (N = 135) from two primary schools in the West of Ireland participated in this study. Data were collected using draw-and-write (N = 135) and focus group interviews (N = 24). Three themes were evident across data types and revealed that students thought the Moving to Learn Ireland active lessons were fun, that they learned academic content better, and the lessons contributed to their fitness development. In conclusion, children who participated in this study positively perceived movement integration lessons in their classrooms. The results of this study can inform future movement integration interventions

Viewpoint: Designing Research in Environmental Education Curriculum Policy Construction, Conceptualisation and Implementation as Exemplified by Southern African Examples

There is increasing dissatisfaction at many levels with existing environmental education curricula in southern Africa. The resulting change and innovation is opening up possibilities for innovative research into the construction, conceptualisation and implementation of the curriculum. However, researching the curriculum offers a range of challenges to those engaged in critically examining processes and practices quite different from those faced in the past. This paper examines a series of key issues and dilemmas in the field of curriculum research in environmental education using cases contributed by active researchers in the area. In the light of the researchers’ experiences we posit a series of propositions that might reduce barriers and constraining forces faced by academics working in the area

Arterial distensibility in children and teenagers: Normal evolution and the effect of childhood vasculitis

Background: Polyarteritis nodosa is a necrotising vasculitis of the medium sized and small muscular arteries. The inflammatory and subsequent reparative processes may alter the arterial mechanical properties. The effect of vasculitic damage on arterial distensibility has never been explored however. Aim: To determine the normal values and the effect of childhood vasculitis on arterial distensibility in children and teenagers. Methods: Distensibility of the brachioradial arterial segment was studied using pulse wave velocity (PWV ∝1/√distensibility), in 13 children with polyarteritis nodosa at a median age of 11.8 (range 4.9-16) years. As a control group, 155 healthy schoolchildren (6-18 years, 81 boys) were studied. PWV was assessed using a photoplethysmographic technique; blood pressure was measured by an automatic sphygmomanometer (Dinamap). Data from patients were expressed as z scores adjusted for age and compared to a population mean of 0 by a single sample t test. Determinants of PWV in normal children were assessed by univariate and multivariate linear regression analyses. Results: Age, height, weight, and systolic blood pressure correlated individually with the brachioradial PWV. Multivariate analysis identified age as the only independent determinant. Ten of the patients were in clinical remission, while three had evidence of disease activity at the time of study. The PWV in the patient group as a whole was significantly greater than those in healthy children (mean z score +0.99). Raised C reactive protein concentration (>2 mg/dl) in the three patients with active disease was associated with a higher PWV when compared to those in remission (z score +2.78 v +0.45). The diastolic blood pressure of the patients was higher than those of the controls (z score + 1.04) while the systolic pressure was similar (z score -0.36). Conclusions: PWV in the brachioradial arterial segment increases gradually during childhood independent of body weight, height, mass, and blood pressure. Increased PWV, and hence decreased distensibility, in this peripheral arterial segment occurs in polyarteritis nodosa and is amplified during acute inflammatory exacerbation.published_or_final_versio

Smartphone-based optical assays in the food safety field.

Smartphone based devices (SBDs) have the potential to revolutionize food safety control by empowering citizens to perform screening tests. To achieve this, it is of paramount importance to understand current research efforts and identify key technology gaps. Therefore, a systematic review of optical SBDs in the food safety sector was performed. An overview of reviewed SBDs is given focusing on performance characteristics as well as image analysis procedures. The state-of-the-art on commercially available SBDs is also provided. This analysis revealed several important technology gaps, the most prominent of which are: (i) the need to reach a consensus regarding optimal image analysis, (ii) the need to assess the effect of measurement variation caused by using different smartphones and (iii) the need to standardize validation procedures to obtain robust data. Addressing these issues will drive the development of SBDs and potentially unlock their massive potential for citizen-based food control

LEARNING TO TEACH ADVENTURE EDUCATION: PRE-SERVICE TEACHERS’ PERSPECTIVES AND EXPERIENCES

Feiman-Nemser (2008) noted the importance of teacher education on the development of teachers, and “how teachers acquire, generate and learn to use knowledge in teaching” (p. 698). The purpose of this investigation was to examine aspects of adventure education for which pre-service teachers’ (PTs) planned, and subsequently taught during a teaching practice (TP) placement. We also sought to gain an understanding of the challenges these PTs faced when teaching this content as part of a physical education curriculum. Data were collected through schemes of work (SoW) and lesson plans (LPs), researcher field notes, a focus group interview, and post lesson discussion. Analysis of data sources revealed little alignment between what PTs planned and what they taught. Attempts were made to incorporate debriefing into lessons but no adventure principles were integrated (Full Value Contract (FVC), Experiential Learning Cycle (ELC) or Challenge with/by Choice (CbC). Challenges that impacted the delivery of lessons included lack of resources and facilities, and perceived student resistance. Findings suggested PTs misunderstood the adventure principles and struggled with adapting and applying knowledge during teaching practice. PTs often focussed on ‘filling time’ as opposed to teaching toward student learning. Further study is needed to examine how teacher education is delivered and what adventure experiences PTs enter teacher education with, to understand why and what PTs plan for and teach and what influences their decisions