Behavioral profile of adults with Prader-Willi syndrome : correlations with individual and environmental variables

Background: Maladaptive behavior has been reported as a phenotypical feature in Prader–Willi syndrome (PWS). It severely limits social adaptation and the quality of life of children and adults with the syndrome. Different factors have been linked with the intensity and form of these behavioral disturbances but there is no consensus about the cause. Consequently, there is still controversy regarding management strategies and there is a need for new data. Methods: The behavior of 100 adults with PWS attending a dedicated center was assessed using the Developmental Behavior Checklist for Adults (DBC-A) and the PWS-specific Hyperphagia Questionnaire. The DBC-A was completed separately by trained caregivers at the center and relatives or caregivers in a natural setting. Genotype, gender, age, degree of obesity and cognitive impairment were analyzed as variables with a hypothetical influence on behavioral features. Results: Patients showed a relatively high rate of behavioral disturbances other than hyperphagia. Disruptive and social relating were the highest scoring DBC-A subscales whereas anxiety/antisocial and self-absorbed were the lowest. When hospital caregiver and natural caregiver scores were compared, scores for the latter were higher for all subscales except for disruptive and anxiety/antisocial. These effects of institutional management were underlined. In the DBC-A, 22 items have descriptive indications of PWS behavior and were used for further comparisons and correlation analysis. In contrast to previous reports, rates of disturbed behavior were lower in patients with a deletion genotype. However, the behavioral profile was similar for both genotypes. No differences were found in any measurement when comparing type I and type II deletions. The other analyzed variables showed little relevance. Conclusions: Significant rates of behavioral disorders were highlighted and their typology described in a large cohort of adults with PWS. The deletion genotype was related to a lower severity of symptoms. Some major behavioral problems, such as hyperphagia, may be well controlled if living circumstances are adapted to the specific requirements of individuals with PWS.

Social policy implications relating to road trauma in a rapidly motorising world : the example of China

The World Health Organization (WHO) identifies road trauma as a major public health issue in all countries, though most notably among low-to-middle income countries and particularly those experiencing rapid motorisation, such as China. As China transitions from a nation of bicycle riders and pedestrians to one where car ownership is increasingly desired, there is need to address the accompanying social policy challenges. With this increased motorisation has come an increased road trauma burden, shouldered disproportionately among the population. Vulnerable road users (i.e., pedestrians, cyclists, motorcyclists) are of primary concern because they are most frequently killed in road crashes, representing approximately 70% of all Chinese road-related fatalities. The aim of this paper is to summarise the scale of the road trauma burden, highlight the disparity of this burden across the Chinese population, and discuss the related social policy implications in dealing with the impact of deaths and of otherwise healthy lives diminished by injury and disability. Future research priorities are also discussed and include the need to strive to provide detailed information on the level of inequity of the road trauma burden across the population and identify appropriate social supports and healthcare services required, both preventative and post-crash, so these can be developed and implemented throughout China.

Review of Legislation and Policy Guidance relating to Adult Social Care in Northern Ireland

Introduction and Background

This research was undertaken by an international team of academics from Queen’s University, Belfast, Leeds University and Penn State University (USA) who have examined models of adult social care provision across thirteen jurisdictions. The aim of this research is to present the Commissioner for Older People in Northern Ireland (COPNI) with possible options for legal reform to adult social care provision for older people in Northern Ireland.

Project Objectives

The agreed objectives of this research were to provide:
• Identification of gaps and issues surrounding the current legislative framework including policy provision for adult social care in Northern Ireland.
• Comparison of Northern Ireland with best practice in other jurisdictions to include (but not be limited to): England and Wales, Republic of Ireland, Scotland and at least two other international examples; Recommendations, based on the above, as to whether there is a need for legislative reform – provision of suggestions other than legislative change (if applicable).
• Recommendations or options based on the above, on how to best change the current framework in Northern Ireland to provide better support outcomes for older people.
• Stakeholder engagement via roundtable event to discuss outcomes/ recommendations.

Structure of Report

The findings from this research are based on an international review of adult social care in the local, national and international contexts. The report will, therefore, firstly present the key recommendations for Northern Ireland which have emerged from a systematic examination and review of adult social care in diverse jurisdictions. Each jurisdiction is then examined in the context of legislative and policy provision and examples of best practice are provided. The final section of the report then compares Northern Ireland to best practice from each of these aforementioned jurisdictions and the discussion entails the background to the report’s final Recommendations. The recommendations in this report are thus directly linked in with the evidence we have gathered across different countries with contrasting systems of welfare.

Relating Fragile States to Social and Human Fragilities

Fragile States is a way of naming this particular category of states that have weak performance, insufficient service delivery, weak administrative and government power, and lack of legal rules. Little consideration is usually made to the fact that their own societies may also be fragile and easily jeopardised by inappropriate economic measures or external events. Poverty traps and social exclusion, unjust inequalities with lack of equity, feelings of insecurity and vulnerability, usually undermine the social fabric. Moreover, the people bear their own internal fragilities, which are based on the lack of capabilities and recognition, and interfere in the relationships between the groups that constitute the society. Therefore, dealing with the issue of fragility requires to consider various decision levels, from the personal one to the State level. Such an approach could allow fragile states to conceive preventive policies that would avoid the surge of a political crisis resulting from the combination of social conflict and individual failure.