Neuropsychological development in children with early and continuously treated phenylketonuria : association with biochemical markers

PKU is a genetically inherited inborn error of metabolism caused by a deficiency of the enzyme phenylalanine hydroxylase. The failure of this enzyme causes incomplete metabolism of protein ingested in the diet, specifically the conversion of one amino acid, phenylalanine, to tyrosine, which is a precursor to the neurotransmitter dopamine. Rising levels of phenylalanine is toxic to the developing brain, disrupting the formation of white matter tracts. The impact of tyrosine deficiency is not as well understood, but is hypothesized to lead to a low dopamine environment for the developing brain. Detection in the newborn period and continuous treatment (a low protein phe-restricted diet supplemented with phenylalanine-free protein formulas) has resulted in children with early and continuously treated PKU now developing normal I.Q. However, deficits in executive function (EF) are common, leading to a rate of Attention Deficit Hyperactivity Disorder (ADHD) up to five times the norm. EF worsens with exposure to higher phenylalanine levels, however recent research has demonstrated that a high phenylalanine to tyrosine ratio (phenylalanine:tyrosine ratio), which is hypothesised to lead to poorer dopamine function, has a more negative impact on EF than phenylalanine levels alone. Research and treatment of PKU is currently phenylalanine-focused, with little investigation of the impact of tyrosine on neuropsychological development. There is no current consensus as to the veracity of tyrosine monitoring or treatment in this population. Further, the research agenda in this population has demonstrated a primary focus on EF impairment alone, even though there may be additional neuropsychological skills compromised (e.g., mood, visuospatial deficits). The aim of this PhD research was to identify residual neuropsychological deficits in a cohort of children with early and continuously treated phenylketonuria, at two time points in development (early childhood and early adolescence), separated by eight years. In addition, this research sought to determine which biochemical markers were associated with neuropsychological impairments. A clinical practice survey was also undertaken to ascertain the current level of monitoring/treatment of tyrosine in this population. Thirteen children with early and continuously treated PKU were tested at mean age 5.9 years and again at mean age 13.95 years on several neuropsychological measures. Four children with hyperphenylalaninemia (a milder version of PKU) were also tested at both time points and provide a comparison group in analyses. Associations between neuropsychological function and biochemical markers were analysed. A between groups analysis in adolescence was also conducted (children with PKU compared to their siblings) on parent report measures of EF and mood. Minor EF impairments were evident in the PKU group by age 6 years and these persisted into adolescence. Life-long exposure to high phenylalanine:tyrosine ratio and/or low tyrosine independent of phenylalanine were significantly associated with EF impairments at both time points. Over half the children with PKU showed severe impairment on a visuospatial task, and this was associated only with concurrent levels of tyrosine in adolescence. Children with PKU also showed a statistically significant decline in a language comprehension task from 6 years to adolescence (going from normal to subnormal), this deficit was associated with lifetime levels of phenylalanine. In comparison, the four children with hyperphenylalaninemia demonstrated normal function at both time points, across all measures. No statistically significant differences were detected between children with PKU and their siblings on the parent report of EF and mood. However, depressive symptoms were significantly correlated with: EF; long term high phe:tyr exposure; and low tyrosine levels independent of phenylalanine. The practice survey of metabolic clinics from 12 countries indicated a high level of variability in terms of monitoring/treatment of tyrosine in this population. Whilst over 80% of clinics surveyed routinely monitored tyrosine levels in their child patients, 25% reported treatment strategies to increase tyrosine (and thereby lower the phenylalanine:tyrosine ratio) under a variety of patient presentation conditions. Overall, these studies have shown that EF impairments associated with PKU provide support for the dopamine-deficiency model. A language comprehension task showed a different trajectory, serving a timely reminder that non-EF functions also remain vulnerable in this population; and that normal function in childhood does not guarantee normal function by adolescence. Mood impairments were associated with EF impairments as well as long term measures of phenylalanine:tyrosine and/or tyrosine. The implications of this research for enhanced clinical guidelines are discussed given varied current practice.

Augmenting mobile computing with video game elements to support, motivate and engage young people in educational, real-world events

Video games have shown great potential as tools that both engage and motivate players to achieve tasks and build communities in fantasy worlds. We propose that the application of game elements to real world activities can aid in delivering contextual information in interesting ways and help young people to engage in everyday events. Our research will explore how we can unite utility and fun to enhance information delivery, encourage participation, build communities and engage users with utilitarian events situated in the real world. This research aims to identify key game elements that work effectively to engage young digital natives, and provide guidelines to influence the design of interactions and interfaces for event applications in the future. This research will primarily contribute to areas of user experience and pervasive gaming.

A geographical analysis of the role of water supply and sanitation in the risk of helminth infections of children in West Africa

Background We investigated the geographical variation of water supply and sanitation indicators (WS&S) and their role to the risk of schistosomiasis and hookworm infection in school age children in West Africa. The aim was to predict large-scale geographical variation in WS&S, quantify the attributable risk of S. haematobium, S. mansoni and hookworm infections due to WS&S and identify communities where sustainable transmission control could be targeted across the region. Methods National cross-sectional household-based demographic health surveys were conducted in 24,542 households in Burkina Faso, Ghana and Mali, in 2003–2006. We generated spatially-explicit predictions of areas without piped water, toilet facilities and finished floors in West Africa, adjusting for household covariates. Using recently published helminth prevalence data we developed Bayesian geostatistical models (MGB) of S. haematobium, S. mansoni and hookworm infection in West Africa including environmental and the mapped outputs for WS&S. Using these models we estimated the effect of WS&S on parasite risk, quantified their attributable fraction of infection, and mapped the risk of infection in West Africa. Findings Our maps show that most areas in West Africa are very poorly served by water supply except in major urban centers. There is a better geographical coverage for toilet availability and improved household flooring. We estimated smaller attributable risks for water supply in S. mansoni (47%) compared to S. haematobium (71%), and 5% of hookworm cases could be averted by improving sanitation. Greater levels of inadequate sanitation increased the risk of schistosomiasis, and increased levels of unsafe water supply increased the risk of hookworm. The role of floor type for S. haematobium infection (21%) was comparable to that of S. mansoni (16%), but was significantly higher for hookworm infection (86%). S. haematobium and hookworm maps accounting for WS&S show small clusters of maximal prevalence areas in areas bordering Burkina Faso and Mali smaller. The map of S. mansoni shows that this parasite is much more wide spread across the north of the Niger River basin than previously predicted. Interpretation Our maps identify areas where the Millennium Development Goal for water and sanitation is lagging behind. Our results show that WS&S are important contributors to the burden of major helminth infections of children in West Africa. Including information about WS&S as well as the “traditional” environmental risk factors in spatial models of helminth risk yielded a substantial gain both in model fit and at explaining the proportion of spatial variance in helminth risk. Mapping the distribution of infection risk adjusted for WS&S allowed the identification of communities in West Africa where integrative preventive chemotherapy and engineering interventions will yield the greatest public health benefits.

Data modeling in elementary and middle school classes : a shared experience

This paper argues for a renewed focus on statistical reasoning in the elementary school years, with opportunities for children to engage in data modeling. Data modeling involves investigations of meaningful phenomena, deciding what is worthy of attention, and then progressing to organizing, structuring, visualizing, and representing data. Reported here are some findings from a two-part activity (Baxter Brown’s Picnic and Planning a Picnic) implemented at the end of the second year of a current three-year longitudinal study (grade levels 1-3). Planning a Picnic was also implemented in a grade 7 class to provide an opportunity for the different age groups to share their products. Addressed here are the grade 2 children’s predictions for missing data in Baxter Brown’s Picnic, the questions posed and representations created by both grade levels in Planning a Picnic, and the metarepresentational competence displayed in the grade levels’ sharing of their products for Planning a Picnic.

The politicization of youth crime in Scotland and the rise of the 'Burberry Court'

In 2003, the youth justice system in Scotland entered a new phase with the introduction of a pilot youth court. The processing of persistent 16 and 17 year old (and serious 15 year olds) represented a stark deviation from a ‘child centred’ and needs-oriented state apparatus for dealing with young offenders to one based on deeds and individual responsibility. This article, based on an evaluation funded by the Scottish Executive, is the first to provide a critical appraisal of this youth justice reform. It examines the views of the judiciary and young offenders and reveals that the pilot youth court in Scotland represents a punitive excursion that poses serious concerns for due process, human rights and net widening.

Stability and change in prosocial and antisocial behavior across the transition to school : Teacher and peer perspectives

Research Findings: The transition to school is a major developmental milestone, and behavior tendencies already evident at the point of school entry can impact upon a child's subsequent social and academic adjustment. The current study aimed to investigate stability and change in the social behavior of girls and boys across the transition from day care to 1st grade. Teacher ratings and peer nominations for prosocial and antisocial behavior were obtained for 248 children belonging to 2 cohorts: school transitioning (n = 118) and day care remaining (n = 130). Data were gathered again from all children 1 year later, following the older group's entry into school. Teacher ratings of prosocial and antisocial behavior significantly predicted teacher ratings of the same behavior at Time 2 for both cohorts. Peer reports of antisocial behavior also showed significant stability, whereas stability of peer-reported prosocial behavior varied as a function of behavior type. Practice or Policy: The results contribute to understanding of trends in early childhood social behavior that potentially influence long-term developmental trajectories. Identification of some behaviors as more stable in early childhood than others, regardless of school entry, provides useful information for both the type and timing of early interventions. © 2010 Taylor & Francis Group, LLC.

The effect of housing characteristics and occupant activities on the respiratory health of women and children in Lao PDR

The paper presents the results of a study conducted into the relationship between dwelling characteristics and occupant activities with the respiratory health of resident women and children in Lao People’s Democratic Republic (PDR). Lao is one of the least developed countries in south-east Asia with poor life expectancies and mortality rates. The study, commissioned by the World Health Organisation, included questionnaires delivered to residents of 356 dwellings in nine districts in Lao PDR over a five month period (December 2005-April 2006), with the aim of identifying the association between respiratory health and indoor air pollution, in particular exposures related to indoor biomass burning. Adjusted odds ratios were calculated for each health outcome separately using binary logistic regression. After adjusting for age, a wide range of symptoms of respiratory illness in women and children aged 1-4 years were positively associated with a range of indoor exposures related to indoor cooking, including exposure to a fire and location of the cooking place. Among women, “dust always inside the house” and smoking were also identified as strong risk factors for respiratory illness. Other strong risk factors for children, after adjusting for age and gender, included dust and drying clothes inside. This analysis confirms the role of indoor air pollution in the burden of disease among women and children in Lao PDR.

A case study examination of the influence of cultural diversity in the multicultural nursing workforce on the quality of care and patient safety in a Saudi Arabian hospital

The purpose of the study: The purpose of this study is to investigate the influence of cultural diversity, in a multicultural nursing workforce, on the quality and safety of patient care and the work environment at King Abdul-Aziz Medical City, Riyadh region. Study background: Due to global migration and workforce mobility, to varying degrees, cultural diversity exists in most health services around the world, particularly occurring where the health care workforce is multicultural or where the domestic population comprises minority groups from different cultures speaking different languages. Further complexities occur when countries have a multicultural workforce which is different from the population for whom they care, in addition to the workers being from culturally diverse countries and with different languages. In Saudi Arabia the health system is mainly staffed by expatriate nurses who comprise 67.7% of the total number of nurses. Study design: This research utilised a case study design which incorporated multiple methods including survey, qualitative interviews and document review. Methods: The participant nurses were selected for the survey via a population sampling strategy; 319 nurses returned their completed Safety Climate Survey questionnaires. Descriptive and inferential statistics (Kruskal–Wallis test) were used to analyse survey data. For the qualitative component of the study, a purposive sampling strategy was used; 24 nurses were interviewed using a semi-structured interview technique. The documentary review included KAMC-R policy documents that met the inclusion criteria using a predetermined data abstraction instrument. Content analysis was used to analyse the policy documents data. Results: The data revealed the nurses‘ perceptions of the clinical climate in this multicultural environment is that it was unsafe, with a mean score of 3.9 out of 5. No significant difference was detected between the age groups or years of experience of the nurses and the perception of safety climate in this context; the study did reveal a statistically significant difference between the cultural background categories and the perception of safety climate. The qualitative phase indicated that the nurses within this environment were struggling to achieve cultural competence; consequently, they were having difficulties in meeting the patients‘ cultural and spiritual needs as well as maintaining a high standard of care. The results also indicated that nurses were disempowered in this context. Importantly, there was inadequate support by the organisation to manage the cultural diversity issue and to protect patients from any associated risks, as demonstrated by the policy documents and supported by the nurses‘ experiences. The study also illustrated the limitations of the conceptual framework of cultural competence when tested in this multicultural workforce context. Therefore, this study generated amendments to the model that is suitable to be used in the context of a multicultural nursing workforce. Conclusion: The multicultural nature of this nursing work environment is inherently risky due to the conflicts that arise from the different cultural norms, beliefs, behaviours and languages. Further, there was uncertainty within the multicultural nursing workforce about the clinical and cultural safety of the patient care environment and about the cultural safety of the nursing workforce. The findings of the study contribute important new knowledge to the area of patient and nurse safety in a multicultural environment and contribute theoretical development to the field of cultural competence. Specifically, the findings will inform policy and practice related to patient care in the context of cultural diversity.

Rethinking Youth Prostitution: National Provision at the Margins of Child Protection and Youth Justice

In March 2000, the Department of Health and the Home Office issued guidance fundamentally altering policy and practice with regard to young people in prostitution. 1 Instead of being arrested and punished for prostitution-related offences, those under 18 years old were to be thought of as children ‘in need’ and offered welfare-based interventions. The practice that has developed in the last three years has offered interventions that are located within both child protection and youth justice work. This article examines these changes in order to generate insights about the changing nature of youth justice. In particular, it is argued that the drive to manage the risks posed by young people in prostitution to specific organisations, takes precedence over either the desire to care for, or the demand to punish them. Through an analysis of how practitioners and policy makers responsible for implementing this new approach to youth prostitution talk about ‘risk’ and ‘responsibility’, ‘liability’, ‘protection’ and ‘punishment’, the article argues that the contradiction between care and control has been re-interpreted, such that there is noticeable blurring of the boundaries between welfare and punishment at the margins of youth justice work.

Secular changes in BMI and obesity risk in Japanese children: Considerations from a morphologic perspective

The increasing prevalence of childhood obesity is a global health issue. Past studies in Japan have reported an increase in both body mass index (BMI) and risk of obesity among children and adolescents. However, changes in body size and proportion in this population over time have also influenced BMI. To date, no study of secular changes in childhood obesity has considered the impact of changes in morphological factors. The current study explored the secular changes in BMI and childhood obesity risk among Japanese children from 1950 to 2000 with consideration of changes in body size and the proportions using The Statistical Report of the School Health Survey (SHS). The age of peak velocity (PV) occurred approximately two years earlier in both genders across this period. While the increments in height, sitting height and sub-ischial leg length relative to height levelled off by 1980, weight gain continued in boys. Between 1980 and 2000, the rate of the upper body weight gain in boys and girls were 0.7-1.3 kg/decade and 0.2-1.0 kg/decade, respectively. After considering body proportions, increments in body weight were small. It could be suggested that the increments in weight and BMI across the 50-year period may be due to a combination of changes including the tempo of growth and body size due to lifestyle factors.

School-community engagement : a critical approach to involving young people in an urban planning project

This study examines the participation of a group of high school students in designing a Heritage Journey as part of an urban redevelopment project in their community. School-community engagement offers young people an opportunity to engage in community life and influence decisions that affect them. Forging links between community and school is becoming more important for teachers as they attempt to create new authentic learning opportunities for young people within a changing world. Increasingly, researchers and urban planners are including children and young people as active decision makers and participants in community engagement projects. However, models of participation tend to be adult-focussed, conceive participation in terms of low to high graduated levels and lack a clearly articulated theoretical basis. The research problem in this study focuses on investigating whether the inclusion of young people in school-community engagement results in value adding to urban planning and is an example of genuine participation. The aim of the study is to provide a theoretically informed, empirically rich understanding of the inclusion of young people in a community engagement strategy for an urban planning project. Theories of space developed by Henri Lefebvre and Edward Soja are drawn upon for understanding how space is understood, used, and redeveloped by the students and other stakeholders. The study also draws on David Harvey’s notion of utopia and space to consider the imaginative possibilities of the students’ designs and ideas. The study uses a participatory research approach and documents the opportunities and challenges of this methodology. The thesis argues that school-community engagement within a "Thirdspace" offers many new opportunities for the emergence of authentic learning situations. Key findings from the study show young people’s participation in an urban planning project can achieve successful results when young people are given opportunities for full participation in decision-making processes; multiple pathways for active engagement are incorporated into the research design; opportunities for mentoring are provided; realistic timelines are communicated to all stakeholders and the needs and social practices of the local community are acknowledged. A new spatial model of community engagement is proposed as an outcome of the study. Unlike previous models of participation, this model demonstrates how exclusion and inclusion can be conceived visually, and may prove effective for conceptualising future community engagement projects that involve young people.

Heterosexual and nonheterosexual young university students’ involvement in traditional and cyber forms of bullying

Research has consistently found that school students who do not identify as self-declared completely heterosexual are at increased risk of victimization by bullying from peers. This study examined heterosexual and nonheterosexual university students’ involvement in both traditional and cyber forms of bullying, as either bullies or victims. Five hundred twenty-eight first-year university students (M= 19.52 years old) were surveyed about their sexual orientation and their bullying experiences over the previous 12 months. The results showed that nonheterosexual young people reported higher levels of involvement in traditional bullying, both as victims and perpetrators, in comparison to heterosexual students. In contrast, cyberbullying trends were generally found to be similar for heterosexual and nonheterosexual young people. Gender differences were also found. The implications of these results are discussed in terms of intervention and prevention of the victimization of nonheterosexual university students.