I need assistance in my Annotated Bibliography. Please review the PDFs attached for all the information related to the assignment. My main topic for the AB is Trisomy 21 and the effect it has on peopl

This is particularly evident in people with trisomy21 and Prader–Willi syndrome (PWS). Although metabolic factors are known to contribute to obesity in trisomy21and hyperphagia plays a primary role in PWS, hyperphagia has not yet been investigated as a possible contributing factor to obesity in trisomy21.

MethodsParticipants comprised three diagnostic groups: trisomy21(T21group), PWS (PWS group) and lifestyle-related obesity (LRO group). They were re- quired to be aged6–18years and have a body mass index over the85th percentile for age and gender. A parent of each participant completed the Hyperphagia Question- naire and the Children’s Leisure Activity Study Survey.

Mean scores for each domain and across all domains of the Hyperphagia Questionnaire and the Children’s Leisure Activity Study Survey were compared between diagnostic groups using linear regression analysis.

ResultsThe study group consisted of52young people (23men and29women) aged6–18years (mean12.5years; T21groupn=17, PWS groupn=16and LRO groupn=19). As hypothesised, the PWS group had the highest mean scores across all domains of the Hyperphagia Questionnaire, and the LRO group had the lowest. Food-seeking behaviour was more pronounced in the PWS group than the T21 group (mean score13.2vs.8.6,p=0.008). The LRO group spent more hours per week engaged in physical activity (14.7) in comparison with the other groups (9.6 and9.7), whereas between the groups, differences in time spent in sedentary activities were less pronounced.

ConclusionsPreoccupation with food and low levels of physical activity may contribute to the development of overweight and obesity in some individuals with trisomy21. These factors warrant consideration in the clinical context.

KeywordsDown syndrome, hyperphagia, physical activity, Prader–Willi syndrome, trisomy 21, weight Background Globally,10% of school-aged children are classified as overweight or obese (Lobsteinet al.2004), and the combined rate of overweight and obesity for Australian children has been reported to be as high as 19%to24% (Boothet al.2001;O’Dea2003).

Children and adolescents with intellectual disability are at increased risk of overweight and obesity 856 Correspondence: Mrs Tess Foerste, Developmental Disability and Rehabilitation Research, Murdoch Childrens Research Institute,50 Flemington Road, Parkville, Victoria3052, Australia (e-mail: tess.

[email protected]).

Journal of Intellectual Disability Research doi: 10.1111/jir.12259 VOLUME 60 PART 9pp856–864S EPTEMBER 2016 ©2016MENCAP and International Association of the Scientific Study of Intellectual and Developmental Disabilities and John Wiley & Sons Ltdbs_bs_banner (Deet al.2008), with reports of the frequency of overweight and obesity for individuals with intellectual disability varying from 31%(Liontiet al.2013) to almost 40% (Deet al.2008; Rimmeret al.2010). Syndromes associated with intellectual disability, where the predisposition to weight gain is well known, include Prader–Willi syndrome (Holland & Wong 1999) and trisomy 21 (Down syndrome) (Rubinet al.1998; Hawn et al.2009; Van Gameren-Oosteromet al.2012).

Trisomy21has a birth prevalence of1:1100live births in Australia (Riley & Halliday2008). Reports of the prevalence of overweight and obesity in individuals with trisomy21have varied internationally. In a Dutch study of1596individuals with trisomy21aged0–26years, the prevalence of overweight and obesity was reported as25.5% and 4.2% for men and32% and5.1% for women, respectively (Van Gameren-Oosteromet al.2012). In the USA, Rimmeret al.(2010) reported a prevalence of55% overweight and31.2% obese, for a sample of 81adolescents (aged12–18years) with trisomy21.A retrospective study from the USA of283adolescents and adults with trisomy21reported a56% prevalence of overweight for women and45% for men. This study demonstrated an environmental influence on weight, showing that those who lived in the family home had a tendency towards overweight in comparison with those living in group homes (Rubinet al.1998).

There are several interrelated factors, common in individuals with Trisomy21, which are known to contribute to the development of obesity within this group. Metabolic factors are well known such as hypothyroidism, decreasedbasal metabolic rate and increased leptin levels (Murray & Ryan-Krause 2010). In addition to this metabolic predisposition to weight gain, children and adolescents with trisomy21have been shown to participate in less high-intensity physical activity than their siblings (Whitt-Gloveret al.2006) and do not meet the recommended daily requirements of moderate to vigorous physical activity (Espositoet al.2012; Nordstromet al.2013). Although there are valid medical reasons for the lower level of exercise, such as the risk of atlantoaxial subluxation or dislocation (Semineet al.1978;Au-Yonget al.2008), unfortunately, environmental factors may also play a role in preventing regular participation in physical activity for individuals with intellectual disability.

Helleret al.(2002) reported that many adults withtrisomy21are unaware of opportunities for engagement in team sports or gym programmes within the community or may have difficulty with independent exercise and require the support, or participation, of family and friends.

In addition to environmental and metabolic factors, abnormally increased appetite, or hyperphagia, plays a role in the development of obesity in some conditions associated with intellectual disability, such as Prader–Willi syndrome (Hollandet al.1995). Prader–Willi syndrome is a genetic condition with a prevalence of approximately1:15 000(Liontiet al.2015)to1:

25 000lives births in Australia (Smithet al.2003).

Features of Prader–Willi syndrome include low muscle tone, characteristic facial features, short stature, autistic traits, global developmental delay and intellectual disability (Cassidyet al.2012).

Hyperphagia is a prominent characteristic of this condition, beginning in early childhood and persisting throughout the adult years. The presence and severity of hyperphagia in Prader–Willi syndrome have been the focus of many research studies (Fieldstoneet al.1998; Dykens et al.2007).

Although the mechanisms that cause hyperphagia in this population are not yetcompletely understood, abnormal changes in hunger and satiety signals in the brain are evident (Shapiraet al.2005;Hinton et al.2006;Holsenet al.2012), as are metabolic abnormalities (Hollandet al.1993;Haqqet al.

2007). People with Prader–Willi syndrome are subsequently at risk of morbid obesity and early death (Einfeldet al.2006;Liontiet al.2012), if the hyperphagia is not controlled by calorie restriction, meticulous food supervision and regular physical activity. The propensity towards obesity in Prader– Willi syndrome is further complicated by a low muscle mass, which means that persons with this condition expend less energy when they exercise (Butleret al.2007). Like individuals with trisomy 21, they have also been shown to do minimal regular vigorous exercise (Nordstromet al.2013).

Although the presence of eating disorders in intellectual disability is acknowledged in the literature (Gravestock2000), there is little in the published literature that casts light on the contribution of hyperphagia to the development of obesity in this population, and specifically about hyperphagia in people with trisomy21. 857 Journal of Intellectual Disability Research VOLUME 60 PART 9S EPTEMBER 2016 T. Foersteet al.Understanding obesity in children with trisomy 21 ©2016MENCAP and International Association of the Scientific Study of Intellectual and Developmental Disabilities and John Wiley & Sons Ltd Aim To further our understanding of pathways to obesity in individuals with trisomy21, we aimed to investigate the presence of hyperphagia and levels of physical and sedentary activity in children and adolescents with trisomy21compared with individuals with lifestyle- related obesity and individuals with Prader–Willi syndrome.

Based on the known association between hyperphagia and Prader–Willi syndrome, we hypothesised that the children and adolescents with trisomy21would have less hyperphagia than those with Prader–Willi syndrome but more than children and adolescents with lifestyle-related obesity.

Furthermore, based on the recognised barriers to physical activity in individuals with intellectual disability, we hypothesised that individuals with lifestyle-related obesity would have higher levels of physical activity than individuals with either trisomy 21or Prader–Willi syndrome.

Methods Setting, participants and recruitment procedure This study was undertaken at The Royal Children’s Hospital, Melbourne, Australia. Ethics approval was obtained from the host institution’s Human Research Ethics Committee.

Inclusion criteria required participants to have a body mass index (BMI) denoting the presence of overweight or obesity, as defined by internationally published cut-points (Coleet al.2000). Each individual’s BMI standard deviation score (BMI SDS) was also calculated. Adjusted for age and gender, overweight was defined as a BMI SDS between the85th and95th percentile, and obesity was defined as a BMI SDS over the95th percentile. All individuals with a BMI SDS under the85th percentile were excluded from the study, as were any individuals with physical disabilities that limited their ability to ambulate and exercise.

There were three groups of overweight and obese participants in this study. A group of young people with lifestyle-related obesity and no known physical or intellectual disabilities (LRO group) was recruited from the Weight Management Clinic at The Royal Children’s Hospital, a group of individuals with trisomy21(T21group) was recruited from the DownSyndrome Association of Victoria and the third group comprised children and adolescents with Prader– Willi syndrome (PWS group) who were recruited from both the Prader–Willi Syndrome Clinic at The Royal Children’s Hospital and the Victorian Prader– Willi Syndrome Register.

At both the Weight Management and Prader-Willi Syndrome clinics, the treating clinician approached eligible participants at the time of their consultation to introduce the study. If the family agreed to participate, weight and height were collected in order to calculate BMI and BMI SDS.

Families who had consented to be contacted for research by the Victorian Prader–Willi Syndrome Register were approached by the Register Manager either at the Prader–Willi Syndrome Clinic or by telephone if they did not have an appointment. If consent was obtained via telephone, the parent/carer was asked to collect the young person’s weight and height in the family home.

The Down Syndrome Association of Victoria contacted, by mail, families of individuals with trisomy21who were in the correct age bracket. The letter included a reply slip (to be returned to the research assistant) to indicate whether they would like to participate. If so, the research assistant telephoned the family to further explain the project and offer participation.

In all cases where consent was obtained, a research assistant provided the family with a questionnaire booklet, comprising two parent-report questionnaires about hyperphagia and engagement in both physical and sedentary activities, as well as demographic questions and questions on food restriction and supervision in the family home. Outcome measures The primary outcome measure was the Hyperphagia Questionnaire (Dykenset al.2007). This13-item multiple choice questionnaire was designed for carers of people with Prader–Willi syndrome to measure the presence of hyperphagia. The questions are divided into three domains that assess the range and severity of food-related issues: hyperphagic behaviour, drive and severity. Although developed specifically for Prader–Willi syndrome, the authors have also used the Hyperphagia Questionnaire for persons with trisomy21or undiagnosed intellectual disabilities 858 Journal of Intellectual Disability Research VOLUME 60 PART 9S EPTEMBER 2016 T. Foersteet al.Understanding obesity in children with trisomy 21 ©2016MENCAP and International Association of the Scientific Study of Intellectual and Developmental Disabilities and John Wiley & Sons Ltd who show unusual food seeking behaviours (State et al.1999).

The Children’s Leisure Activities Study Survey (CLASS) was used as a secondary measure to determine how much time engaged in both physical and sedentary activities in a typical week, including during school hours, each participant spent engaged in both physical and sedentary activities in a typical week, including during school hours (Salmonet al.

2004). Physical and sedentary activities were analysed and reported individually, including separate scores for team sports and other physical activities, and separate scores for television viewing, computer time, and other sedentary activities.

Statistical analysis Sample size calculations were performed using published means and standard deviations (SD) for each domain on the Hyperphagia Questionnaire in a Prader– Willi syndrome sample (Dykenset al.2007). Using a power of0.8, and a two-sided level of statistical significance of0.05, a sample size of16per group was calculated to be sufficient tofind a1SD difference in each of the domains of the Hyperphagia Questionnaire.

In the absence of any relevant data, this difference was deemed to represent a clinically meaningful difference.

Univariable linear regression analysis and chi- squared tests were used, for interval and categorical variables respectively, to compare demographic and clinical variables across the three diagnostic groups.

Linear regression was also performed to compare mean total and domain scores from the Hyperphagia Questionnaire and the CLASS between diagnostic groups, with and without adjustment for potential confounders. For all analyses, aP-value of<0.05was used to denote statistical evidence of difference, representing no more than a5% likelihood of the difference being attributable to chance alone. Analysis was performed in Stata13.1(StataCorp2013, College Station, TX, USA). Results Characteristics of the study group (Table 1) The study group consisted of52children and adolescents (23men and29women) aged6–18years (mean12.5years). It included17individuals with trisomy21(T21group),19with lifestyle-relatedobesity (LRO group) and16with Prader–Willi syndrome (PWS group); all participants were living in the family home. Participants with Prader–Willi syndrome all had molecular confirmation of the diagnosis. Across the entire study group,42of the52 individuals (81%) were classified as obese and10of 52(19%) as overweight. The mean BMI SDS for the entire group was2.1(range1.1–3.4).

There were some relevant sampling differences between the three diagnostic groups. The LRO group included a similar proportion of men and women, whereas the T21and PWS groups comprised relatively more women (65% and56%, respectively).

The LRO group had the highest mean BMI SDS (2.4 vs.2.1and1.7for the PWS and T21groups, respectively) and the highest proportion of persons classified as obese (95% vs.81% and65% for the PWS and T 21groups, respectively). Indicative of differences in the frequency of intellectual disability, members of the LRO group were all educated at mainstream schools, whereas the majority of participants with non-missing data from the other two groups attended special schools.Compared with both the T21 and LRO groups, parents of the PWS group were more likely to lock fridges and cupboards in the family home for additional food restriction and were more likely to require children to ask for permission before having a snack. Despite differences between the groups in food availability, a similar proportion of individuals in each group were permitted to get their own snacks. Potential confounding We found no strong statistical evidence of association between total hyperphagia scores, total physical activity scores or total sedentary activity scores and age, gender or BMI SDS in this cohort (allp>0.05).

However, this did not rule them out as potential confounders in the association between diagnostic groups and study outcomes. Type of schooling was not considered as a potential confounder as it is indicative of intellectual status, a defining characteristic of the diagnostic groups.

Hyperphagia (Table 2) The mean hyperphagia scores for the T21group fell between the mean scores for the LRO and PWS groups for each domain of the Hyperphagia Questionnaire and for total hyperphagia scores. 859 Journal of Intellectual Disability Research VOLUME 60 PART 9S EPTEMBER 2016 T. Foersteet al.Understanding obesity in children with trisomy 21 ©2016MENCAP and International Association of the Scientific Study of Intellectual and Developmental Disabilities and John Wiley & Sons Ltd When the mean total and domain scores for the T21and LRO groups were compared statistically, we observed no differences between the two groups that could not be attributable to chance.

The results were similar when statistical adjustment was made for age, BMI SDS and gender.When the unadjusted mean scores for the T21and PWS groups were compared, there were no differences between the groups in hyperphagic drive and severity that could not be attributable to chance.

There was strong evidence, however, that hyperphagic behaviours such as food foraging and stealing were more prevalent in the PWS group. After 860 Table 1Characteristics of the study group T21 group N=17LRO group N=19PWS group N=16Total group N=52P-value for group difference Gender0.578 Male,n(%) 6 (35.3) 10 (52.6) 7 (43.7) 23 (44.3) Female,n(%) 11 (64.7) 9 (47.4) 9 (56.3) 29 (55.7) Age (years), mean (SD) 13.8 (2.6) 11.7 (2.9) 12.1 (4.1) 12.5 (3.3) 0.119 BMI SDS, mean (SD) 1.74 (0.34) 2.44 (0.4) 2.09 (0.5) 2.10 (0.5)<0.001 BMI category0.074 Overweight,n(%) 6 (35.3) 1 (5.3) 3 (18.7) 10 (19.3) Obese,n(%) 11 (64.7) 18 (94.7) 13 (81.3) 42 (80.7) School type<0.001 Mainstream,n(%) 0 (0.0) 19 (100.0) 3 (33.3) 22 (64.7) Special,n(%) 6 (100.0) 0 (0.0) 6 (66.7) 12 (35.3) Locks on fridge<0.001 Yes,n(%) 2 (11.7) 1 (5.3) 9 (56.3) 12 (23.8) No,n(%) 15 (88.3) 18 (94.7) 7 (43.7) 41 (76.9) Permission to eat0.121 Yes,n(%) 10 (58.8) 9 (47.4) 13 (81.3) 32 (61.5) No,n(%) 7 (41.2) 10 (52.6) 3 (18.7) 20 (38.5) Gets own snacks0.875 Yes,n(%) 11 (64.7) 12 (63.2) 9 (56.3) 32 (61.5) No,n(%) 6 (35.3) 7 (36.8) 7 (43.7) 20 (38.5) BMI, body mass index; SD, standard deviation; CI, confidence interval; T21, trisomy21; PWS, Prader–Willi syndrome; LRO, lifestyle-related obesity.

Table 2Comparison of mean domain and total scores on the Hyperphagia Questionnaire between diagnostic groups Hyperphagia scoreLRO group N=19T21 group N=17PWS group N=16T21 vs. LRO groupT21 vs. PWS group Mean (SD) Mean (SD) Mean (SD) Unadjusted P-valueAdjusted* P-valueUnadjusted P-valueAdjusted* P-value Behaviour 7.0 (4.1) 8.6 (2.7) 13.2 (6.2) 0.186 0.466 0.008 0.010 Drive 7.7 (3.4) 9.9 (3.3) 11.1 (4.3) 0.057 0.318 0.378 0.144 Severity 2.7 (1.5) 3.3 (1.3) 4.1 (2.3) 0.257 0.410 0.239 0.005 Total score 17.5 (8.5) 21.8 (6.1) 28.4 (11.6) 0.090 0.365 0.050 0.020 *Adjusted for age, body mass index standard deviation score and gender.

SD, standard deviation; T21, trisomy 21; PWS, Prader–Willi syndrome; LRO, lifestyle-related obesity.

Journal of Intellectual Disability Research VOLUME 60 PART 9S EPTEMBER 2016 T. Foersteet al.Understanding obesity in children with trisomy 21 ©2016MENCAP and International Association of the Scientific Study of Intellectual and Developmental Disabilities and John Wiley & Sons Ltd statistical adjustment was made for age, BMI SDS and gender, there was strong evidence for a real difference between the two groups in hyperphagic behaviours, severity of hyperphagia and mean total scores.

Physical and sedentary activities (Table 3) Data on physical and sedentary activities were available for48individuals (four parents of individuals with Prader–Willi syndrome did not complete the CLASS questionnaire).

On average, the T21group spent fewer total hours each week engaged in physical activity compared with the LRO group (10vs.15h) but a comparable amount of time to the PWS group (10h). This trend was seen for participation in team sports as well as for engagement in other forms of physical activity. We found no strong statistical evidence in support of a difference either between the T21and LRO groups or the T21and PWS groups in mean hours spent engaged in physical activities on either unadjusted or adjusted analysis.

With respect to sedentary activities, variation was seen between diagnostic groups according to the type of activity. Of the three groups, the LRO group spent the most overall time engaged in sedentary activity (mean44vs.35and31h/week for the T21and PWS groups) and the most time playing computer games orsurfing the Internet (mean11vs.2and4h/week for the T21and PWS groups). On the other hand, the T21 group spent the most time watching television (mean 13vs.12and11h/week for the LRO and PWS groups).

There was statistical evidence of difference between the T21and LRO groups in their mean computer times (2vs.11h/week;p=0.03), but we observed a slightly higher likelihood that this difference could be attributable to chance when adjustment was made for gender, age and BMI SDS (p=0.09).

Although there was no evidence of difference between the T21and PWS groups for any of the sedentary behaviour scores on unadjusted analysis (all p>0.05), after adjustment for gender, age and BMI SDS, we found a higher likelihood beyond chance that the T21group spent more hours each week watching television (p <0.01) and engaging in other non-computer-related sedentary activities (p<0.01). Discussion Overweight and obesity are significant problems not only in the general Australian population but also place significant burden on individuals with intellectual disability, where opportunities for a physically active lifestyle may be limited. There are many factors that contribute to the development of obesity in this population, both metabolic and environmental. Hyperphagia is a well-known 861 Table 3Comparison of mean CLASS scores by activity type between diagnostic groups LRO group N=19T21 group N=17PWS group N=16T21 vs. LRO groupT21 vs. PWS group Mean (SD) Mean (SD) Mean (SD)Unadjusted P-valueAdjusted* P-valueUnadjusted P-valueAdjusted* P-value Physical Team sports 2.8 (3.1) 1.7 (1.3) 1.7 (3.0) 0.176 0.230 0.980 0.296 Other 11.9 (6.9) 8.0 (6.0) 8.2 (5.6) 0.079 0.318 0.935 0.270 Total score 14.8 (8.4) 9.7 (6.1) 9.6 (5.3) 0.050 0.223 0.948 0.308 Sedentary Television 11.8 (9.4) 12.8 (7.6) 10.6 (10.3) 0.710 0.743 0.518 0.002 Computer 11.3 (16.7) 2.2 (3.5) 3.9 (4.7) 0.034 0.093 0.279 0.653 Other 21.0 (20.1) 21.3 (11.6) 16.6 (6.4) 0.970 0.308 0.215<0.001 Total score 44.3 (32.8) 35.2 (11.3) 30.6 (12.9) 0.284 0.488 0.325 0.098 *Adjusted for age, body mass index standard deviation score and gender.

CLASS, Children’s Leisure Activity Study Survey; SD, standard deviation; T21, trisomy 21; PWS, Prader–Willi syndrome; LRO, lifestyle- related obesity.

Journal of Intellectual Disability Research VOLUME 60 PART 9S EPTEMBER 2016 T. Foersteet al.Understanding obesity in children with trisomy 21 ©2016MENCAP and International Association of the Scientific Study of Intellectual and Developmental Disabilities and John Wiley & Sons Ltd characteristic of Prader–Willi syndrome that has been extensively researched for its contribution to life- threatening obesity if left uncontrolled. To our knowledge; this is thefirst research paper to investigate hyperphagia in individuals with trisomy21.

Consistent with oura priorihypotheses, our research shows that individuals with trisomy21who are overweight or obese may display some characteristics of hyperphagia that are often present in individuals with Prader–Willi syndrome, although to a lesser degree. For all domains of the Hyperphagia Questionnaire, the mean scores for the T21group fell between the mean scores for the LRO and PWS groups. Statistical evidence of difference in food- seeking behaviours between the T21and PWS groups was evident, supporting the idea that the T21group was less likely than the PWS group to practise pathological food-seeking behaviours.

Lack of physical activity continues to be a major contributor to the development of obesity within the Australian population. Ten percent of Australian children aged5–14years reportedly play video games 20h or more per fortnight or2h per day (Australian Bureau of Statistics2006). In our study, all groups exhibited high levels of sedentary activity, with an average of6.3h per week spent playing video games or surfing the Internet.

Despite the high levels of sedentary activity, the Australian Bureau of Statistics has also reported that Australian children aged5–14years participate in organised team sports for an average of3h per week (Australian Bureau of Statistics2006). In our study, the average time spent participating in team sports for the LRO group (2.8h per week) was similar to the general population. This was not the case for the PWS and T21groups where, on average, only1.7h per week was spent participating in team sports.

This may be an indication of the barriers faced by persons with intellectual disability in trying to access organised team sports or gym programmes, particularly once they reach adolescence (Heller et al.2002). It is possible that group differences may reflect variation in educational settings, in particular, there may be differences in physical education programmes between primary and secondary school settings and between special and mainstream schools.

The authors acknowledge that this study had some limitations. Firstly, the measure used for the investigation of hyperphagia was developed for people with Prader–Willi syndrome and not for trisomy21or lifestyle-related obesity. Despite this, the authors of the questionnaire report that they have found the measure useful in other populations such as trisomy 21(Stateet al.1999). Secondly, although we met our sample size requirements forfinding a1-SD difference between groups in hyperphagia scores, larger numbers may have produced more generalisable results. Thirdly, the CLASS relies upon parent recollection of the physical and sedentary activities that their child engages in each week. This may have resulted in some measurement error around the amount of time engaged in each type activity, particularly for activities during school hours and in other settings outside the home. Furthermore, parents may not be aware of the time spent in particular kinds of activity at school or in other settings outside the home.

In conclusion, this research suggests that a preoccupation with food may play a role in the development of obesity in some individuals with trisomy21; however, differences between this group and the LRO group were not definitive. In the clinical setting, it may be worthwhile for families of individuals with intellectual disability to be provided with more information aboutthe risks of over-eating for their child and to be linked in with local dieticians, exercise programmes and support networks.

Acknowledgements The authors gratefully acknowledge the members of the multidisciplinary PWS clinic, the Victorian Prader–Willi Syndrome Association and the Down Syndrome Association of Victoria for their invaluable assistance with recruitment.

We are also appreciative of thefinancial support provided by the Ultimate Challenge, the Doo Bees and Trailblazers Auxiliaries. The third author’s(S.R.) research is supported by an Early Career Fellowship (2014–2017) from the National Health and Medical Research Council of Australia. This study was also supported by the Victorian Government’s Operational Infrastructure Support Program.

Conflict of Interest None of the authors have conflicts of interest to declare. 862 Journal of Intellectual Disability Research VOLUME 60 PART 9S EPTEMBER 2016 T. Foersteet al.Understanding obesity in children with trisomy 21 ©2016MENCAP and International Association of the Scientific Study of Intellectual and Developmental Disabilities and John Wiley & Sons Ltd References Au-Yong I., Boszczyk B., Mehdian H. & Kerslake R. (2008) Spontaneous rotatory atlantoaxial dislocation without neurological compromise in a child with Down syndrome:

a case report.European Spine Journal17,308–11.

Australian Bureau of Statistics (2006)4901.0Children’s Participation in Culture and Leisure Activities. Canberra Booth M. L., Wake M., Armstrong T., Chey T., Hesketh K.

&MathurS.(2001) The epidemiology of overweight and obesity among Australian children and adolescents, 1995–97.Australian and New Zealand Journal of Public Health25,162–9.

Butler M. G., Theodoro M. F., Bittel D. C. & Donnelly J. E.

(2007) Energy expenditure and physical activity in Prader–Willi syndrome: comparison with obese subjects.

American Journal of Medical Genetics. Part A143A,449–59.

Cassidy S. B., Schwartz S., Miller J. L. & Driscoll D. J.

(2012)Prader–Willi syndrome.Genetics in Medicine14, 10–26.

Cole T. J., Bellizi M. C., Flegal K. M. & Dietz W. H. (2000) Establishing a standard definition for child overweight and obesity worldwide: international survey.British Medical Journal320,1240–5.

De S., Small J. & Baur L. A. (2008) Overweight and obesity among children with developmental disabilities.Journal of Intellectual & Developmental Disability33,43–7.

Dykens E. M., Maxwell M. A., Pantino E., Kossler R. & Roof E. (2007) Assessment of hyperphagia in Prader–Willi syndrome. Obesity15,1816–26.

Einfeld S. L., Kavanagh S. J., Smith A., Evans E. J., Tonge B. J. & Taffe J. (2006) Mortality in Prader–Willi syndrome.American Journal of Mental Retardation111, 193–8.

Esposito P. E., Macdonald M., Hornyak J. E. & Ulrich D. A.

(2012) Physical activity patterns of youth with Down syndrome.Intellectual and Developmental Disabilities50, 109–19.

Fieldstone A., Zipf W. B., Sarter M. F. & Bernston G. G.

(1998) Food intake in Prader–Willi syndrome and controls with obesity after administration of a benzodiazepine receptor agonist.Obesity Research6,29–33.

Gravestock S. (2000) Eating disorders in adults with intellectual disability.Journal of Intellectual Disability Research44,625–37.

Haqq A. M., Muehlbauer M., Svetkey L. P., Newgard C. B., Purnell J. Q., Grambow S. C.et al.(2007) Altered distribution of adiponectin isoforms in children with Prader–Willi syndrome (PWS): association with insulin sensitivity and circulating satiety peptide hormones.

Clinical Endocrinology67,944–51.

Hawn J., Rice C., Nichols H. & McDermott S. (2009) Overweight and obesity among children with Down syndrome: a descriptive study of children attending a Down syndrome clinic in South Carolina.Journal - South Carolina Medical Association105,64–8.Heller T., Hseih K. & Rimmer J. (2002) Barriers and supports for exercise participation among adults with down syndrome.Journal of Gerontological Social Work30, 161 –77.

Hinton E. C., Holland A. J., Gellatly M. S. N., Soni S., Patterson M., Ghatei M. A.et al.(2006) Neural representations of hunger and satiety in Prader–Willi syndrome.International Journal of Obesity30,313–21.

Holland A. J., Treasure J., Coskeran P. & Dallow J. (1995) Characteristics of the eating disorder in Prader–Willi syndrome: implications for treatment.Journal of Intellectual Disability Research39,373–81.

Holland A. J., Treasure J., Coskeran P., Dallow J., Milton N.

& Hillhouse E. (1993) Measurement of excessive appetite and metabolic changes in Prader–Willi syndrome.

International Journal of Obesity and Related Metabolic Disorders: Journal of the International Association for the Study of Obesity17,527–32.

Holland A. J. & Wong J. (1999) Genetically determined obesity in Prader–Willi syndrome: the ethics and legality of treatment.Journal of Medical Ethics25, 230–6.

Holsen L. M., Savage C. R., Martin L. E., Bruce A. S., Lepping R. J., Ko E.et al.(2012) Importance of reward and prefrontal circuitry in hunger and satiety: Prader– Willi syndrome vs simple obesity.International Journal of Obesity36,638–47.

Lionti T., Reid S. M., Reddihough D. S. & Sabin M. (2013) Monitoring height and weight:findings from a developmental paediatric service.Journal of Paediatrics and Child Health49,1063–8.

Lionti T., Reid S. M. & Rowell M. M. (2012) Prader–Willi syndrome in Victoria: mortality and causes of death.

Journal of Paediatrics and Child Health48,506–11.

Lionti T., Reid S. M., White S. M. & Rowell M. M. (2015) A population-based profile of160Australians with Prader–Willi syndrome: trends in diagnosis, birth prevalence and birth characteristics.American Journal of Medical Genetics. Part A167,371–8.

Lobstein T., Baur L., Uauy R. & IASO International Obesity Task Force (2004) Obesity in children and young people:

a crisis in public health.Obesity Reviews5,4–85.

Murray J. & Ryan-Krause P. (2010) Obesity in children with Down syndrome: background and recommendations for management.Pediatric Nursing36,314–9.

Nordstrom M., Hansen B. H., Paus B. & Kolset S. O. (2013) Accelerometer-determined physical activity and walking capacity in persons with Down syndrome, Williams syndrome and Prader–Willi syndrome.Research in Developmental Disabilities34,4395–403.

O’Dea J. A. (2003) Differences in overweight and obesity among Australian school children of low and middle/high socioeconomic status.Medical Journal of Australia179,63.

Riley M. & Halliday J. (2008)Birth Defects in Victoria 2005–2006. Victorian Perinatal Data Collection Unit, Melbourne. 863 Journal of Intellectual Disability Research VOLUME 60 PART 9S EPTEMBER 2016 T. Foersteet al.Understanding obesity in children with trisomy 21 ©2016MENCAP and International Association of the Scientific Study of Intellectual and Developmental Disabilities and John Wiley & Sons Ltd Rimmer J. H., Yamaki K., Lowry B. M. D., Wang E. & Vogel L. C. (2010) Obesity and obesity-related secondary conditions in adolescents with intellectual/developmental disabilities.Journal of Intellectual Disability Research54, 787–94.

Rubin S. S., Rimmer J. H., Chicoine B., Braddock D. & McGuire D. E. (1998) Overweight prevalence in persons with Down syndrome.Mental Retardation36,175–81.

Salmon J., Telford A. & Crawford D. (2004)Children’s Leisure Activities Study Survey–Summary Report. Deakin University, Burwood.

Semine A. A., Ertel A. N., Goldberg M. J. & Bull M. J.

(1978) Cervical-spine instability in children with Down syndrome (trisomy21).Journal of Bone and Joint Surgery 60,649–52.

Shapira N. A., Lessig M. C., He A. G., James G. A., Driscoll D. J. & Liu Y. (2005) Satiety dysfunction in Prader–Willi syndrome demonstrated by fMRI.Journal of Neurology, Neurosurgery, and Psychiatry76,260–2.Smith A., Egan J., Ridley G., Haan E., Montgomery P., Williams K.et al.(2003) Birth prevalence of Prader–Willi syndrome in Australia.Archives of Disease in Childhood88, 262–4.

State M., Dykens E., Rosner B., Martin A. & King B.

(1999) Obsessive-compulsive symptoms in Prader– Willi and“Prader-Willi-like”patients.Journal of the American Academy of Child and Adolescent Psychiatry38, 329–34.

Van Gameren-Oosterom H. B. M., Van Dommelen P., Schonbeck Y., Oudesluys-Murphy A. M., Van Wouwe J. P. & Buitendijk S. E. (2012) Prevalence of overweight in Dutch children with Down syndrome.Pediatrics130, e1520–6.

Whitt-Glover M. C., O’Neill K. L. & Stettler N. (2006) Physical activity patterns in children with and without Down syndrome.Pediatric Rehabilitation9,158–64.

Accepted20January2016 864 Journal of Intellectual Disability Research VOLUME 60 PART 9S EPTEMBER 2016 T. Foersteet al.Understanding obesity in children with trisomy 21 ©2016MENCAP and International Association of the Scientific Study of Intellectual and Developmental Disabilities and John Wiley & Sons Ltd This document isascanned copyofaprinted document. Nowarranty isgiven aboutthe accuracy ofthe copy. Users should refertothe original published versionofthe material.