Effects of management objectives and rules on marine conservation outcomes.

Understanding the relative effectiveness and enabling conditions of different area-based management tools is essential for supporting efforts that achieve positive biodiversity outcomes as area-based conservation coverage increases to meet newly set international targets. We used data from a coastal social-ecological monitoring program in 6 Indo-Pacific countries to analyze whether social, ecological, and economic objectives and specific management rules (temporal closures, fishing gear-specific, species-specific restrictions) were associated with coral reef fish biomass above sustainable yield levels across different types of area-based management tools (i.e., comparing those designated as marine protected areas [MPAs] with other types of area-based management). All categories of objectives, multiple combinations of rules, and all types of area-based management had some sites that were able to sustain high levels of reef fish biomass-a key measure for coral reef functioning-compared with reference sites with no area-based management. Yet, the same management types also had sites with low biomass. As governments advance their commitments to the Kunming-Montreal Global Biodiversity Framework and the target to conserve 30% of the planet's land and oceans by 2030, we found that although different types of management can be effective, most of the managed areas in our study regions did not meet criteria for effectiveness. These findings underscore the importance of strong management and governance of managed areas and the need to measure the ecological impact of area-based management rather than counting areas because of their designation.

Efectos de las reglas y objetivos de manejo sobre los resultados de conservación marina Resumen Es esencial entender la efectividad relativa y las condiciones habilitantes de las diferentes herramientas de manejo basadas en el área para respaldar los esfuerzos que brindan resultados positivos para la biodiversidad conforme aumenta la cobertura de la conservación basada en el área para alcanzar los objetivos internacionales recién establecidos. Usamos los datos de un programa de monitoreo socioeconómico costero en seis países del Indo-Pacífico para analizar si los objetivos sociales, ecológicos y económicos y las reglas específicas de manejo (cierres temporales, restricciones de equipo de pesca, vedas de especies) se asociaban con la biomasa de los peces de arrecife de coral por encima de los niveles de producción sustentable en diferentes tipos de herramientas de manejo basadas en el área (es decir, comparar aquellas designadas como áreas marinas protegidas[AMP] con otros tipos de manejo basado en el área). Todas las categorías de objetivos, las múltiples combinaciones de reglas y todos los tipos de manejo basado en el área tuvieron algunos sitios capaces de mantener los niveles altos de biomasa de peces de arrecife-una medida importante para el funcionamiento de los arrecifes-en comparación con los sitios de referencia sin manejo basado en el área. Sin embargo, los mismos tipos de manejo también tuvieron sitios con baja biomasa. Conforme los gobiernos avanzan en sus compromisos con el Marco Global de Biodiversidad de Kunming-Montreal y hacia el objetivo de conservar el 30% del suelo y los océanos del planeta para el 2030, descubrimos que, aunque diferentes tipos de manejo pueden ser efectivos, la mayoría de las áreas manejadas en nuestras regiones de estudio no cumplieron con los criterios de efectividad. Este descubrimiento enfatiza la importancia de una gestión y un gobierno sólidos de las áreas manejadas y la necesidad de medir el impacto ecológico del manejo basado en el área en lugar de contar las áreas por su designación.

Local practices and production confer resilience to rural Pacific food systems during the COVID-19 pandemic.

Resilience of food systems is key to ensuring food security through crisis. The COVID-19 pandemic presents an unprecedented shock that reveals varying levels of resilience of increasingly interconnected food systems across the globe. We contribute to the ongoing debate about whether increased connectivity reduces or enhances resilience in the context of rural Pacific food systems, while examining how communities have adapted to the global shocks associated with the pandemic to ensure food security. We conducted 609 interviews across 199 coastal villages from May to October 2020 in Federated States of Micronesia, Fiji, Palau, Papua New Guinea, Solomon Islands, Tonga, and Tuvalu to understand community-level impacts and adaptations during the first 5-10 months of the COVID-19 crisis. We found that local food production practices and food sharing conferred resilience, and that imported foods could aid or inhibit resilience. Communities in countries more reliant on imports were almost twice as likely to report food insecurity compared to those least reliant. However, in places dealing with a concurrent cyclone, local food systems were impaired, and imported foods proved critical. Our findings suggest that policy in the Pacific should bolster sustainable local food production and practices. Pacific states should avoid becoming overly reliant on food imports, while having measures in place to support food security after disasters, supplementing locally produced and preserved foods with imported foods when necessary. Developing policies that promote resilient food systems can help prepare communities for future shocks, including those anticipated with climate change.

Self-report of pain in young people and adults with spastic cerebral palsy: interrater reliability of the revised Face, Legs, Activity, Cry, and Consolability (r-FLACC) scale ratings.

AIM: People with cerebral palsy (CP) are often unable to express pain owing to cognitive or speech impairments. Reports that rely on observation can be inaccurate, because behaviours such as grimacing, common in people with spastic CP, resemble pain expressions. We examined preliminary validity and reliability of the revised Face, Legs, Activity, Cry, and Consolability (r-FLACC) scale in people with spastic CP. METHOD: Forty-eight young people and adults (35 females, 13 males; mean [SD] age 29y 2mo [13y]) were video-recorded during a standard examination, rating their pain (0-10) afterwards. Two raters completed the r-FLACC using the video recordings. Interrater reliability was assessed with an unconditional cross-classified random-effects model and item response theory approach; Pearson correlations measured agreement between raters and participants. RESULTS: Mean (SD) participant (n=48) pain scores were 2.48 (2.5) and mean (SD) r-FLACC scores were 1.46 (1.68). There was moderate agreement between raters (intraclass coefficient 0.41 and 0.57 respectively) but low agreement between participants and raters (r=0.26). There were no significant effects for raters (lay observers, nurses, physicians, and inexperienced raters). INTERPRETATION: Results provide mixed support for the interrater reliability of the r-FLACC in people with spastic CP. WHAT THIS PAPER ADDS: The revised Face, Legs, Activity, Cry, and Consolability (r-FLACC) scale can be reliably used by experts and lay raters for people with spastic cerebral palsy (CP). Support is mixed for interrater reliability of the r-FLACC scale used with people with spastic CP.

Understanding the dementia diagnosis gap in Norfolk and Suffolk: a survey of general practitioners.

BACKGROUND: The National Health Service (NHS) has announced its new target to increase the 'shockingly low dementia diagnosis rate' in England from the current level of 45% to 66% by end of March 2015. Clinical commissioning groups (CCGs) in England have committed to meeting this target. The Norfolk and Suffolk dementia diagnosis rate (DDR) is below the rate for England in some areas; across the CCGs included in this study, the average DDR was 39.9% with a standard deviation of 5.3. AIMS: This study aimed to explore and understand the low DDR in Norfolk and Suffolk and to learn what might be needed to support general practitioners (GPs) to meet the targets set by the UK Department of Health. METHODS: An online survey was developed including questions from the National GP Audit 2009. The link to the online survey was sent via email to all GPs in four participating CCGs in Norfolk and Suffolk. SPSS was used for descriptive analysis. Chi-square tests were conducted to identify significant differences in response rates between groups of GPs. RESULTS: The survey was completed by 28% (N = 113) of 400 GPs in 108 practices across three CCGs receiving the survey link. There was a significant difference in response rates from GPs in each CCG, but there were no significant differences in terms of their answers to the questions in the survey. GP respondents expressed confidence in their ability to identify cases of dementia for onward referral to memory services. Participating GPs also acknowledged the benefits to patients and their carers of a timely dementia diagnosis at an early stage of the disease. However, they reported concerns about the quality and availability of post-diagnostic support services for people with dementia and their carers. In this survey, GPs' attitudes were more positive about diagnosing dementia than those responding to the National Audit 2009. CONCLUSIONS: Despite GPs' attitudes being more positive than in 2009 about diagnosing dementia, the Norfolk and Suffolk DDR remains low. This may reflect lack of GP confidence in the quality and availability of post-diagnostic support services. This study has identified a need to map the existing post-diagnostic support services for people with dementia and to identify gaps in services. This could lead to the development of a resource which might enable GPs to provide relevant advice to newly diagnosed patients and their carers, facilitate signposting to support services, and give GPs confidence to increase the DDR in their area.

Health sector costs of self-reported food allergy in Europe: a patient-based cost of illness study.

INTRODUCTION: Food allergy is a recognized health problem, but little has been reported on its cost for health services. The EuroPrevall project was a European study investigating the patterns, prevalence and socio-economic cost of food allergy. AIMS: To investigate the health service cost for food-allergic Europeans and the relationship between severity and cost of illness. METHODS: Participants recruited through EuroPrevall studies in a case-control study in four countries, and cases only in five countries, completed a validated economics questionnaire. Individuals with possible food allergy were identified by clinical history, and those with food-specific immunoglobulin E were defined as having probable allergy. Data on resource use were used to estimate total health care costs of illness. Mean costs were compared in the case-control cohorts. Regression analysis was conducted on cases from all 9 countries to assess impact of country, severity and age group. RESULTS: Food-allergic individuals had higher health care costs than controls. The mean annual cost of health care was international dollars (I$)2016 for food-allergic adults and I$1089 for controls, a difference of I$927 (95% confidence interval I$324-I$1530). A similar result was found for adults in each country, and for children, and was not sensitive to baseline demographic differences. Cost was significantly related to severity of illness in cases in nine countries. CONCLUSIONS: Food allergy is associated with higher health care costs. Severity of allergic symptoms is a key explanatory factor.

Weight changes in children receiving intrathecal baclofen for the treatment of spasticity.

OBJECTIVE: To identify changes in weight for children receiving intrathecal baclofen (ITB) therapy for the treatment of spasticity. DESIGN: Retrospective medical chart review. Weights were obtained before and after ITB pump implantation. SETTING: Tertiary care children's hospital. PARTICIPANTS: 52 children < 20 years old at the time of data collection receiving ITB therapy for the treatment of cerebral origin spasticity for > 1 year. MEASURES: Paired t-test compared pre and post-implantation weights. Analyses of covariance were run to compare differences in overall weight change as defined by variables of gender, diet, diagnosis, and ethnicity. RESULTS: A significant change in pre-implantation weight (M=26.77, SD=11.29) from post-implantation weight (M=32.11, SD=12.62) was noted. No significant weight change was noted among variables. CONCLUSION: Study participants gained an average of 5.43 kg (SD=4.34) after receiving 1 year of ITB therapy.

Social and economic costs of food allergies in Europe: development of a questionnaire to measure costs and health utility.

OBJECTIVES: To develop a questionnaire to measure the additional social costs of food allergies (FAs). DATA SOURCE AND STUDY SETTING: People with FAs and sampled members of the general population (with and without FAs) in the Netherlands and the United Kingdom in 2006. STUDY DESIGN: (1) Literature review. (2) Focus group to identify key costs of FAs and seek views on the questionnaires. (3) Pilot survey to test the questionnaires in cases and controls. DATA COLLECTION: Twenty-eight participants in the United Kingdom and the Netherlands with clinically or self-diagnosed FAs took part in one of five focus groups. A case-control postal survey was conducted in the United Kingdom and the Netherlands (with 125 FA cases and 62 controls). PRINCIPAL FINDINGS: Methods exist to measure social costs in chronic illness, but not FAs. Focus groups found features of FAs likely to impact costs of living. Pilot results suggest higher costs of living and health care costs, and well-being in FAs. CONCLUSION: The questionnaire is proposed for use in wider European and other comparative studies of FAs.

Weight gain in children with hypertonia of cerebral origin receiving intrathecal baclofen therapy.

OBJECTIVE: To identify the pattern of weight change in children receiving intrathecal baclofen (ITB) therapy. DESIGN: A retrospective medical chart review was conducted to identify weight status before and after ITB pump implantation. SETTING: Tertiary care children's hospital and academic medical center. PARTICIPANTS: All children and adolescents with hypertonia of cerebral origin who were younger than 19 years of age at the time of pump placement and followed in our pediatric baclofen pump program. INTERVENTIONS: Not applicable. MAIN OUTCOME MEASURES: A linear mixed model was used to examine the rate of change in weight (weight-gain velocity) before and after surgery. Weight change was adjusted for age, sex, functional level determined by the Gross Motor Functional Classification System, tube feeding, dystonia, and other comorbidities. RESULTS: The average weight-gain velocity was 2.32 kg/y presurgery and 2.93 kg/y postsurgery, adjusted for potential confounders. The 0.61 kg/y increase in weight-gain velocity attained statistical significance (P = .028). CONCLUSIONS: Although excessive weight gain is not a common problem in children with cerebral origin spasticity, increased weight-gain velocity is prevalent in children receiving ITB therapy. Health care providers may anticipate a welcome weight gain in the underweight child. This consequence should be considered when managing children receiving ITB therapy, and health care providers must appropriately intervene to prevent excessive weight gain. Further studies exploring the reasons for the increased weight-gain velocity are warranted.

Gene structure and tissue expression of human selenoprotein W, SEPW1, and identification of a retroprocessed pseudogene, SEPW1P.

We have determined that the human SEPW1 (selenoprotein W) gene maps to chromosome 19q13.3, spans approximately 6.3 kb and comprises six exons, in contrast to the previously published five exons. The gene lacks canonical TATA and CAAT boxes, but has numerous Sp1 consensus binding sites upstream of multiple transcription start sites. SEPW1 is expressed in all of the 22 tissues assayed, and shows highest expression in skeletal muscle and heart. Additionally, we have also identified a retroprocessed SEPW1 pseudogene, SEPW1P, which maps to chromosome 1p34-35.

Nonsense-mediated RNA decay in the TSC1 gene suggests a useful tool pre- and post-positional cloning.

Many mRNAs carrying mutations that are predicted to encode a truncated protein are subject to a mechanism known as nonsense-mediated mRNA decay (NMD), which results in reduced levels of mutant transcript. Tuberous sclerosis (TSC), an autosomal dominant neurocutaneous disorder with mutations in either of two genes, TSC1 or TSC2, requires comprehensive screening of both genes for molecular diagnosis. Virtually all TSC1 mutations are predicted to truncate the protein product. Coding and newly identified 3' untranslated region polymorphisms in TSC1 were used to develop a transcript imbalance assay to investigate TSC1 transcript levels in patients. This approach allowed the correct identification of six out of seven TSC1 patients tested blind from a panel of TSC1 and TSC2 patients, with no false positives. The extent of NMD in TSC1 was found to correlate with each individual mutation regardless of intra-familial variation in clinical features and with no strong evidence for positional bias. NMD in TSC1 was more pronounced in cultured cells than in RNA prepared directly from peripheral lymphocytes (in which novel splicing of exon 5 was observed). The advent of a dense SNP map of transcribed regions of the genome may allow a similar transcript imbalance assay in the assessment of candidate genes for diseases whose causes are still unknown.