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.

Understanding Density in an Uneven City, Santiago de Chile: Implications for Social and Environmental Sustainability

Efforts to promote infill development and to raise densities are growing in many cities around the world as a way to encourage urban sustainability. However, in cities polarized along socio-economic lines, the benefits of densification are not so evident. The aim of this paper is to discuss some of the contradictions of densification in Santiago de Chile, a city characterized by socio-spatial disparities. To that end, we first use regression analysis to explain differences in density rates within the city. The regression analysis shows that dwelling density depends on the distance from the city center, socioeconomic conditions, and the availability of urban attributes in the area. After understanding the density profile, we discuss the implications for travel and the distribution of social infrastructures and the environmental services provided by green areas. While, at the metropolitan scale, densification may favor a more sustainable travel pattern, it should be achieved by balancing density rates and addressing spatial differences in the provision of social services and environmental amenities. We believe a metropolitan approach is essential to correct these spatial imbalances and to promote a more sustainable and socially cohesive growth pattern.