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Down Syndrome Research and Practice 11(2)

Sensory impairments and health concerns related to the degree of intellectual disability in people with Down syndrome

Tuomo Määttä, Markus Kaski, Anja Taanila, Sirkka Keinänen-Kiukaanniemi, and Matti Iivanainen

The relationship between poor health and cognitive impairment is not fully understood yet. People with Down syndrome are prone to a number of health problems, including congenital heart defect, visual impairment, hearing loss, autoimmune diseases, epilepsy, early-onset Alzheimer's disease and intellectual disability. Our aim was to assess the impact of impaired health on cognitive performance in people with Down syndrome. A series of people with Down syndrome (n=129) were studied for their intellectual disability, sensory impairments and health concerns. The medical and psychological records of all persons with Down syndrome in the Intellectual Disability Service Register of Kainuu from 1970 to 2004 were analysed. The detected health issues were related to the individuals' cognitive levels. Visual impairment, poor dental health and acute neurological disease were associated with the severity of intellectual disability and ageing.

Määttä, T, Kaski, M, Taanila, A, Keinänen-Kiukaanniemi, S, and Iivanainen, M. (2006) Sensory impairments and health concerns related to the degree of intellectual disability in people with Down syndrome. Down Syndrome Research and Practice, 11(2), 78-83.

Parental strategies for assisting children to wait

Monica Cuskelly, Anne Jobling, Linda Gilmore, and Sheila Glenn

This study examined parents' behaviours as they waited with their child. Children were presented with an attractively wrapped gift and then asked not to touch it until the experimenter returned from finishing some work in another room. Three parent groups and their children participated in the study - parents of children with Down syndrome, parents of children with intellectual disability from another cause, and parents of children who were developing typically. There were no significant differences between children in how long they were able to wait before touching the gift. The data from the first two groups were combined for all analyses after it was established that there were no significant differences between them. There were few significant differences between parents of a child with intellectual disability and comparison parents. The former group were more likely to be classified as Authoritarian than were comparison parents, however with one exception, parenting style was unrelated to the strategies parents used in the waiting situation. Very few parents in either group used the opportunity to teach or explicitly praise effective waiting strategies in their children.

Cuskelly, M, Jobling, A, Gilmore, L, and Glenn, S. (2006) Parental strategies for assisting children to wait. Down Syndrome Research and Practice, 11(2), 55-63.

The passive in adolescents with Down syndrome: a case study

Maraci Rubin

This paper shows that some individuals with Down syndrome are capable of producing, imitating (repeating) and comprehending passive sentences, even though group studies indicate that this is not the norm. Experimental tests of elicited production, repetition and comprehension of passive and active sentences applied in ten adolescents with Down syndrome, speakers of Portuguese, showed that out of the ten adolescents, one, Fa, is able to produce, imitate and comprehend passive sentences. It is hypothesised that, when there is no comprehension, or when the comprehension of reversible passives is unstable, the passive is understood as active, because the first noun of the passive sentence is interpreted as agent/causer of the action/non-action. This hypothesis is strong inasmuch as it assumes that both active and passive have very similar initial derivations. There is not, however, strong evidence that the nine adolescents interpret the passive as active. But if it is assumed that their chance results in comprehension of the passive is due to the fact that they are beginning to understand it, then it could be said that, for around 50% of the time, the adolescents with Down syndrome interpret the passive as active. Fa, on the other hand, does not interpret the passive as active, as she produces, imitates and comprehends the passive structure very well.

Rubin, M. (2006) The passive in adolescents with Down syndrome: a case study. Down Syndrome Research and Practice, 11(2), 88-96.

Addressing challenging behaviour in children with Down syndrome: The use of applied behaviour analysis for assessment and intervention

Kathleen Feeley, and Emily Jones

Children with Down syndrome are at an increased risk for engaging in challenging behaviour that may be part of a behavioural phenotype characteristic of Down syndrome. The methodology of applied behaviour analysis has been demonstrated effective with a wide range of challenging behaviours, across various disabilities. Applications to children with Down syndrome and the examination of behaviourally based strategies to specifically address the unique characteristics of children with Down syndrome are limited. However, there are several studies in which a subset of the participants did have Down syndrome. A handful of these studies are reviewed within the context of functional behaviour assessment and Positive Behavioural Supports. Drawing from these studies and the behavioural literature, as well as the authors' clinical experience and research, suggestions regarding early intervention for challenging behaviour with children with Down syndrome are provided.

Feeley, K, and Jones, E. (2006) Addressing challenging behaviour in children with Down syndrome: The use of applied behaviour analysis for assessment and intervention. Down Syndrome Research and Practice, 11(2), 64-77.

Glucose-6-phosphate-dehydrogenase is also increased in erythrocytes from adolescents with Down syndrome

Francisco Ordonez, Manuel Rosety-Plaza, and Manuel Rosety-Rodriguez

For some time it has been claimed that trisomic cells are more sensitive to oxidative stress since there is an imbalance in hydrogen peroxide metabolism due to an increase in superoxide dismutase (SOD) catalytic activity. We designed the present study to assess activity levels of antioxidant enzymes [superoxide dismutase (SOD), glutathione peroxidase (GPx), catalase (CAT) and glucose-6-phosphate-dehydrogenase (G6PDH)] in erythrocytes in 31 male adolescents with Down syndrome (mean age 16.3 ± 1.1). An increase of 35.2%, 15.3% and 14.9% in the catalytic activity of SOD, GPx and G6PDH respectively was observed in male adolescents with Down syndrome compared to age-matched controls. For CAT, a slight increase of 6.0% was also found. It is concluded that our data are consistent with previous evidence of the existence of oxidative stress in individuals with Down syndrome as revealed by significantly enhanced activities of SOD and GPx. The most striking feature was that G6PDH, in contrast to CAT, presented a similar behaviour. Further studies are required to identify other antioxidant enzymes in red blood cells as well as in white blood cells in order to increase the range of potential bioindicators of oxidative stress.

Ordonez, F, Rosety-Plaza, M, and Rosety-Rodriguez, M. (2006) Glucose-6-phosphate-dehydrogenase is also increased in erythrocytes from adolescents with Down syndrome. Down Syndrome Research and Practice, 11(2), 84-87.