The measurement of subjective wellbeing in people with intellectual disability in Australia

The importance of measuring quality of life, and most particularly the personal wellbeing of people with intellectual disabilities (ID), is now recognized. The measurement of wellbeing is an important component of program evaluation and can assist in the identification and planning of individualized support needs. There remains, however, a need for further research in this area. This paper describes a new scale, the Personal Wellbeing Index Intellectual Disability Scales (PWI-ID), which has been shown to be valid and reliable. Data is presented regarding its use in the measurement of wellbeing in people with ID and the focus of discussion is on its advantages and limitations.<br /

Mitigation and screening for environmental assessment

This article considers how, as a matter of law and policy, mitigation measures should be taken into account in determining whether a project will have significant environmental effects and therefore be subject to assessment under the EU Environmental Impact Assessment (EIA) Directive. This is not straightforward: it is problematic to distinguish clearly between an activity and the measures proposed to minimise or mitigate for the adverse consequences of the activity. The issue is a salient one in impact assessment law, but under-explored in the literature and handled with some difficulty by the courts. I argue that there is an unnecessarily and undesirably narrow approach currently taken under the EIA Directive, which could be improved upon by taking a more adaptive approach; alternatively a heightened standard of review of ‘significance’, and within this of the scope for mitigation measures to bring projects beneath the significance threshold, may also be desirable

What you know can influence what you are going to know (especially for older adults)

Stimuli related to an individual's knowledge/experience are often more memorable than abstract stimuli, particularly for older adults. This has been found when material that is congruent with knowledge is contrasted with material that is incongruent with knowledge, but there is little research on a possible graded effect of congruency. The present study manipulated the degree of congruency of study material with participants’ knowledge. Young and older participants associated two famous names to nonfamous faces, where the similarity between the nonfamous faces and the real famous individuals varied. These associations were incrementally easier to remember as the name-face combinations became more congruent with prior knowledge, demonstrating a graded congruency effect, as opposed to an effect based simply on the presence or absence of associations to prior knowledge. Older adults tended to show greater susceptibility to the effect than young adults, with a significant age difference for extreme stimuli, in line with previous literature showing that schematic support in memory tasks particularly benefits older adults

X-Linked lissencephaly with absent corpus callosum and abnormal genitalia: an evolving multisystem syndrome with severe congenital intestinal diarrhea disease

X-linked lissencephaly with abnormal genitalia is a rare and devastating syndrome. The authors present an infant with a multisystem phenotype where the intestinal manifestations were as life limiting as the central nervous system features. Severe chronic diarrhea resulted in failure to thrive, dehydration, electrolyte derangements, long-term hospitalization, and prompted transition to palliative care. Other multisystem manifestations included megacolon, colitis, pancreatic insufficiency hypothalamic dysfunction, hypothyroidism, and hypophosphatasia. A novel aristaless-related homeobox gene mutation, c.1136G>T/p.R379L, was identified. This case contributes to the clinical, histological, and molecular understanding of the multisystem nature of this disorder, especially the role of ARX in the development of the enteroendocrine system.David Coman, Tom Fullston, Cheryl Shoubridge, Richard Leventer, Flora Wong, Simon Nazaretian, Ian Simpson, Josef Gecz, and George McGillivra