Heads of alternative provision: committed to realising young peoples’ potential in an unregulated market

Alternative provision (AP) caters for pupils marginalised and excluded from mainstream schooling. In England, it is conceptualised in policy as providing education to support behavioural improvements (pupils are directed off-site to improve behaviour). There is limited research on the experiences of those who work in AP settings. That which does exist tends to report the commitment of these professionals to the young people with whom they work. Young people who attend these schools frequently talk positively about the relationships they experience there. As such, there is a need to better understand the motivations of those working with these young people if we are to understand the key relationships that make AP work. This article fills a gap by focusing on the experiences of those managing AP settings across a geographical area. The findings are based on 3 interviews and 20 surveys and develop significantly our understanding of the motivations of those working in and managing AP settings. Interesting divergences in practice are highlighted and findings show managers both see and work to realise the potential of young people in AP. These findings suggest staff commitment should be conceptualised as belief in the potential of the young people who attend AP

Quantitative sensory testing in a large cohort of neuropathy patients: QST in Neuropathy

Background: Quantitative sensory testing (QST) is a subjective but reliable and quantifiable method to detect patient thresholds to different sensory stimuli. QST is used to measure small- and large-fiber nerve function and can be used in conjunction with other diagnostic modalities in the evaluation of peripheral neuropathy (PN). The utility of QST to distinguish among different types of PN, however, has not been explored. The objective of the study was to evaluate if different patterns of QST abnormalities could distinguish between PN types. Methods: This single-center retrospective cohort study evaluated the frequency of QST abnormalities to vibratory, cold and heat detection thresholds in a large population of PN cases evaluated at the University of Texas Southwestern Medical Center peripheral neuropathy clinic between 1995-2000. PN was categorized by etiology. Results: A total of 559 QST studies were performed in this study. The average age of patients (n=557) was 60 years with a male-to-female ratio of 1:1. The most common diagnosis was cryptogenic sensory polyneuropathy (CSPN, n=294), followed by Charcot–Marie–Tooth disease (n=84)). Meta-regression of vibration and cold indicate that the expected proportion of abnormal responses is less for the vibration test (p = 0.0002), relative to the cold test. However, no differences were observed between diagnoses. Conclusions: Though abnormal QST thresholds were seen in most patients with PN, patterns of QST abnormalities do not distinguish between different types of PN. The routine clinical utility of QST is likely limited

Gender in Tourism Research: Perspectives from Latin America

Purpose This paper aims to examine the knowledge production on tourism gender research in Latin America and to reflect on the main challenges faced by this subfield. Design/methodology/approach The study conducts a bibliometric analysis of the journal articles on tourism gender research in the largest scientific databases in Latin America: Redalyc, Scielo and Latindex. The paper examines variables such as year of publication, journal, authors, affiliation, types of articles, research topics, methodologies and geographical location of fieldwork. Findings The study identified 153 gender aware papers from 70 journals for the period 2001-2015. The leading countries in the subfield are Brazil, Mexico and Argentina. The majority of papers are empirical and have a local scope. The main theoretical approaches derive from sociological and anthropological perspectives with a predominance of qualitative methodologies. There is a need to strengthen the theoretical and epistemological frameworks and increase international collaboration for knowledge exchange among tourism gender scholars. Research limitations/implications The bibliometric analysis was limited to indexed journals with online access. It focused on academic articles and excluded research notes, book reviews and conference proceedings. Originality/value As the main working languages of scientific production in Latin America are Spanish and Portuguese, this is the first attempt to make tourism gender research from this region visible for the predominantly Anglophone tourism academy, with the intention of identifying common challenges

Parallel processes:Getting it write?

This paper offers a critical reflection on the processes surrounding the writing of a book aimed at foster carers and residential workers. By utilising the concept of parallel process as well as the four modes of reflection identified by Ruch (2000), we explore the ways in which the wider context of both direct works with children and reflective practice have been impacted by the tensions between relationally based, child-centred practice and wider managerialist imperatives. The paper draws parallels between these practice tensions and those currently in play within the academy. By employing a dialogical and reflective analysis of the process and interactions surrounding the writing of a practitioner-targeted book, the paper demonstrates the ways in which critical and process reflection post-event took place, considering the heretofore unexplored parallel processes between writing for practice, and practice. In so doing, it identifies the ways in which the authors mirrored practitioners in relation to the management of anxiety, a sense of constrained autonomy and confidence, and an avoidance of recognising and challenging structural and political context. Implications for the creation of practice literature and for the academy are considered.Output Status: Forthcoming/Available Onlin

Database Evaluation for Muscle and Nerve Diseases - DEMAND: An academic neuromuscular coding system

Background: A database which documents the diagnosis of neuromuscular patients is useful for determining the types of patients referred to academic centers and for identifying participants for clinical trials and other studies. The ICD-9 or ICD-10 numeric systems are insufficiently detailed for this purpose. Objective: To develop a database for neuromuscular diagnoses Methods: We developed a detailed diagnostic coding system for neuromuscular diseases called DEMAND: Database Evaluation for Muscle and Nerve Diseases that has been adopted by neuromuscular clinics at University of Texas Health Science Center San Antonio (UTHSCSA), Ohio State University (OSU), University of Kansas Medical Center (KUMC), and University of Texas Southwestern (UTSW). At the initial visit, patients are assigned a diagnostic code which can be revised later if appropriate. Fields include patient’s name, date of birth, and diagnostic code. The neuromuscular database consisted of 457 codes. Each code has a prefix (MUS or PNS) followed by a three-digit number. Depending on whether muscle or nerve is primarily involved, there are eight broad groups: motor neuron disease (MUS codes 100-139); neuromuscular junction disorders (MUS 200-217); acquired and hereditary myopathies (MUS 300-600s); acquired and hereditary polyneuropathies (PNS 100-400); mononeuropathies (PNS 500s); plexopathies (PNS 600s); radiculopathies (PNS 700s); and mononeuritis multiplex (PNS 800s). Results: During a period of 10 years, 17,163 of patients were entered (1,752 at UTHSCSA, 1,840 at OSU, 3,699 at KUMC, 9,872 at UTSW). The number of patients in several broad categories are: 3,080 motor neuron disease; 1,575 neuromuscular junction disease; 1,851 muscular dystrophies; 633 inflammatory myopathies; 1,090 hereditary neuropathies; 1,001 immune-mediated polyneuropathies; 620 metabolic/toxic polyneuropathies; 535 mononeuropathies; 296 plexopathies; and 769 radiculopathies. Conclusion: A detailed diagnostic neuromuscular database can be utilized at multiple academic centers. The database should be simple without too many fields to complete, to ensure compliance during busy clinic operations. This database has been very useful in identifying groups of patients for retrospective, observational studies and for prospective treatment studies including trials for Amyotrophic Lateral Sclerosis (ALS), Muscular Dystrophies (MD), Myasthenia Gravis (MG), and retrospective studies of Primary Lateral Sclerosis (PLS), chronic inflammatory demyelinating neuropathy (CIDP), etc

Copy Number Variants in Extended Autism Spectrum Disorder Families Reveal Candidates Potentially Involved in Autism Risk

Copy number variations (CNVs) are a major cause of genetic disruption in the human genome with far more nucleotides being altered by duplications and deletions than by single nucleotide polymorphisms (SNPs). In the multifaceted etiology of autism spectrum disorders (ASDs), CNVs appear to contribute significantly to our understanding of the pathogenesis of this complex disease. A unique resource of 42 extended ASD families was genotyped for over 1 million SNPs to detect CNVs that may contribute to ASD susceptibility. Each family has at least one avuncular or cousin pair with ASD. Families were then evaluated for co-segregation of CNVs in ASD patients. We identified a total of five deletions and seven duplications in eleven families that co-segregated with ASD. Two of the CNVs overlap with regions on 7p21.3 and 15q24.1 that have been previously reported in ASD individuals and two additional CNVs on 3p26.3 and 12q24.32 occur near regions associated with schizophrenia. These findings provide further evidence for the involvement of ICA1 and NXPH1 on 7p21.3 in ASD susceptibility and highlight novel ASD candidates, including CHL1, FGFBP3 and POUF41. These studies highlight the power of using extended families for gene discovery in traits with a complex etiology

Evidence of novel finescale structural variation at autism spectrum disorder candidate loci

Background: Autism spectrum disorders (ASD) represent a group of neurodevelopmental disorders characterized by a core set of social-communicative and behavioral impairments. Gamma-aminobutyric acid (GABA) is the major inhibitory neurotransmitter in the brain, acting primarily via the GABA receptors (GABR). Multiple lines of evidence, including altered GABA and GABA receptor expression in autistic patients, indicate that the GABAergic system may be involved in the etiology of autism. Methods: As copy number variations (CNVs), particularly rare and de novo CNVs, have now been implicated in ASD risk, we examined the GABA receptors and genes in related pathways for structural variation that may be associated with autism. We further extended our candidate gene set to include 19 genes and regions that had either been directly implicated in the autism literature or were directly related (via function or ancestry) to these primary candidates. For the high resolution CNV screen we employed custom-designed 244 k comparative genomic hybridization (CGH) arrays. Collectively, our probes spanned a total of 11 Mb of GABA-related and additional candidate regions with a density of approximately one probe every 200 nucleotides, allowing a theoretical resolution for detection of CNVs of approximately 1 kb or greater on average. One hundred and sixty-eight autism cases and 149 control individuals were screened for structural variants. Prioritized CNV events were confirmed using quantitative PCR, and confirmed loci were evaluated on an additional set of 170 cases and 170 control individuals that were not included in the original discovery set. Loci that remained interesting were subsequently screened via quantitative PCR on an additional set of 755 cases and 1,809 unaffected family members. Results: Results include rare deletions in autistic individuals at JAKMIP1, NRXN1, Neuroligin4Y, OXTR, and ABAT. Common insertion/deletion polymorphisms were detected at several loci, including GABBR2 and NRXN3. Overall, statistically significant enrichment in affected vs. unaffected individuals was observed for NRXN1 deletions. Conclusions: These results provide additional support for the role of rare structural variation in ASD

Within- and between-person and group variance in behavior and beliefs in cross-cultural longitudinal data

This study grapples with what it means to be part of a cultural group, from a statistical modeling perspective. The method we present compares within- and between-cultural group variability, in behaviors in families. We demonstrate the method using a cross-cultural study of adolescent development and parenting, involving three biennial waves of longitudinal data from 1296 eight-year-olds and their parents (multiple cultures in nine countries). Family members completed surveys about parental negativity and positivity, child academic and social-emotional adjustment, and attitudes about parenting and adolescent behavior. Variance estimates were computed at the cultural group, person, and within-person level using multilevel models. Of the longitudinally consistent variance, most was within and not between cultural groups—although there was a wide range of between-group differences. This approach to quantifying cultural group variability may prove valuable when applied to quantitative studies of acculturation

Growth patterns in early childhood: Better trajectories in Afro-Ecuadorians independent of sex and socioeconomic factors.

The first years of life are the most dynamic period for childhood growth. There are limited data available on growth patterns of infants and children living in rural Latin America. The aim of this study was to describe the growth patterns from birth to 5years in children living in a rural District of tropical coastal Ecuador using data from a birth cohort of 2404 neonates. We hypothesize that there would be growth differences according to ethnicity and sex. Evaluations were conducted at birth or until 2weeks of age and at 7, 13, 24, 36 and 60months during clinic and home visits. Individual growth trajectories for weight-for-age, height-for-age and weight/height-for-age Z-scores were estimated using multilevel models. Girls were lighter and shorter than boys at birth. However, Afro-Ecuadorian children (versus mestizo or indigenous) were longer/taller and heavier throughout the first 5years of life and had greater mean trajectories for HAZ and WAZ independent of sex and socioeconomic factors. Our data indicate that ethnicity is a determinant of growth trajectories during the first 5years of life independent of socioeconomic factors in a birth cohort conducted in a rural region of Latin America

A randomized controlled trial of methotrexate for patients with generalized myasthenia gravis

OBJECTIVE: To determine the steroid-sparing effect of methotrexate (MTX) in patients with symptomatic generalized myasthenia gravis (MG). METHODS: We performed a 12-month multicenter, randomized, double-blind, placebo-controlled trial of MTX 20 mg orally every week vs placebo in 50 acetylcholine receptor antibody-positive patients with MG between April 2009 and August 2014. The primary outcome measure was the prednisone area under the dose-time curve (AUDTC) from months 4 to 12. Secondary outcome measures included 12-month changes of the Quantitative Myasthenia Gravis Score, the Myasthenia Gravis Composite Score, Manual Muscle Testing, the Myasthenia Gravis Quality of Life, and the Myasthenia Gravis Activities of Daily Living. RESULTS: Fifty-eight patients were screened and 50 enrolled. MTX did not reduce the month 4-12 prednisone AUDTC when compared to placebo (difference MTX - placebo: -488.0 mg, 95% confidence interval -2,443.4 to 1,467.3, p = 0.26); however, the average daily prednisone dose decreased in both groups. MTX did not improve secondary measures of MG compared to placebo over 12 months. Eight participants withdrew during the course of the study (1 MTX, 7 placebo). There were no serious MTX-related adverse events. The most common adverse event was nonspecific pain (19%). CONCLUSIONS: We found no steroid-sparing benefit of MTX in MG over 12 months of treatment, despite being well-tolerated. This study demonstrates the challenges of conducting clinical trials in MG, including difficulties with recruitment, participants improving on prednisone alone, and the need for a better understanding of outcome measure variability for future clinical trials. CLASSIFICATION OF EVIDENCE: This study provides Class I evidence that for patients with generalized MG MTX does not significantly reduce the prednisone AUDTC over 12 months of therapy