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      Dietary aspects related to health and obesity in Williams syndrome, Down syndrome, and Prader–Willi syndrome

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          Abstract

          Background

          Dietary aspects that might contribute to development of obesity and secondary conditions are not well documented in genetic subgroups associated with intellectual disability.

          Objective

          To describe the intake frequencies of selected foods in participants with Prader–Willi syndrome (PWS), Down syndrome (DS), and Williams syndrome (WS), and investigate the association with body mass index (BMI). To explore food-related autonomy and intake frequencies among persons with DS in different living arrangements.

          Methods

          Self-reported intake frequencies and measurement of plasma carotenoids and erythrocyte content of omega-3 fatty acids (FAs) were investigated in persons aged 16–42 years, with WS ( n=21), DS ( n=40), and PWS ( n=20).

          Results

          A larger proportion of participants with PWS showed high-frequency intake of fruits ( p=0.012) and vegetables ( p=0.004), and had higher plasma carotenoids ( p<0.001) compared to participants with DS and WS. Furthermore, a larger proportion of participants with WS were low-frequency consumers of fish ( p=0.005), less likely to use omega-3 FA supplements ( p=0.023), and had reduced erythrocyte concentrations of long-chain omega-3 FAs ( p<0.001), compared to participants with PWS and DS. In DS, BMI was negatively associated with plasma carotenoids. Increased proportions of participants living in communities showed high-frequency intake of precooked meals ( p=0.030), and a tendency toward high-frequency consumption of soft drinks ( p=0.079), when compared to peers living with relatives. Participants in community residences were also more likely to participate frequently in food-related decisions and preparations.

          Conclusions

          Persons with WS had a less-favorable dietary pattern when compared to persons with PWS. A larger proportion of persons living in communities frequently consumed precooked meals and showed a tendency of high-frequency soft drink consumption. Otherwise, their intake frequencies of the investigated foods were similar to those living with relatives, but they participated more frequently in decisions and preparations of foods.

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          Most cited references55

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          Fruit and vegetable consumption and risk of coronary heart disease: a meta-analysis of cohort studies.

          The consumption of fruit and vegetables is associated with a reduced rate of coronary heart disease (CHD) in observational cohorts. The purpose of this study was to assess the strength of this association in a meta-analysis. Cohort studies were selected if they reported relative risks (RRs) and 95% CI for coronary heart disease or mortality and if they presented a quantitative assessment of fruit and vegetable intake. The pooled RRs were calculated for each additional portion of fruit and/or vegetables consumed per day, and the linearity of the associations were examined. Nine studies were eligible for inclusion in the meta-analysis that consisted of 91,379 men, 129,701 women, and 5,007 CHD events. The risk of CHD was decreased by 4% [RR (95% CI): 0.96 (0.93-0.99), P = 0.0027] for each additional portion per day of fruit and vegetable intake and by 7% [0.93 (0.89-0.96), P < 0.0001] for fruit intake. The association between vegetable intake and CHD risk was heterogeneous (P = 0.0043), more marked for cardiovascular mortality [0.74 (0.75-0.84), P < 0.0001] than for fatal and nonfatal myocardial infarction [0.95 (0.92-0.99), P = 0.0058]. Visual inspection of the funnel plot suggested a publication bias, although not statistically significant. Therefore, the reported RRs are probably overestimated. This meta-analysis of cohort studies shows that fruit and vegetable consumption is inversely associated with the risk of CHD. The causal mechanism of this association, however, remains to be demonstrated.
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            The DNA sequence of human chromosome 21.

            Chromosome 21 is the smallest human autosome. An extra copy of chromosome 21 causes Down syndrome, the most frequent genetic cause of significant mental retardation, which affects up to 1 in 700 live births. Several anonymous loci for monogenic disorders and predispositions for common complex disorders have also been mapped to this chromosome, and loss of heterozygosity has been observed in regions associated with solid tumours. Here we report the sequence and gene catalogue of the long arm of chromosome 21. We have sequenced 33,546,361 base pairs (bp) of DNA with very high accuracy, the largest contig being 25,491,867 bp. Only three small clone gaps and seven sequencing gaps remain, comprising about 100 kilobases. Thus, we achieved 99.7% coverage of 21q. We also sequenced 281,116 bp from the short arm. The structural features identified include duplications that are probably involved in chromosomal abnormalities and repeat structures in the telomeric and pericentromeric regions. Analysis of the chromosome revealed 127 known genes, 98 predicted genes and 59 pseudogenes.
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              Comparison between plasma and erythrocyte fatty acid content as biomarkers of fatty acid intake in US women.

              Erythrocyte fatty acids may be superior to plasma fatty acids for reflecting long-term fatty acid intake because of less sensitivity to recent intake and a slower turnover rate. The objective was to compare the fatty acid content of erythrocytes with that of plasma with respect to their abilities to reflect usual fatty acid intake. Fatty acids in plasma and erythrocytes were measured by capillary gas-liquid chromatography in 306 US women aged 43-69 y. Fatty acid intake was assessed with a food-frequency questionnaire, which was validated for measuring intakes of various fatty acids. Docosahexaenoic acid (DHA, 22:6n-3) in erythrocytes and plasma provided the strongest correlations with its intake, but erythrocyte DHA concentrations [Spearman's partial correlation coefficient (r(s))=0.56] were better than plasma DHA concentrations (r(s)=0.48) as a biomarker. Total trans fatty acids (r(s)=0.43) and total 18:1 trans isomers (r(s)=0.42) in erythrocytes were also more strongly correlated with intake than were those in plasma (r(s)=0.30 and r(s)=0.29, respectively). Moderate correlations were observed for linoleic acid (18:2n-6; erythrocytes, r(s)=0.24; plasma, r(s)=0.25), alpha-linolenic acid (18:3n-3; erythrocytes, r(s)=0.18; plasma, r(s)=0.23), and eicosapentaenoic acid (20:5 n-3; erythrocytes, r(s)=0.38; plasma, r(s)=0.21). For polyunsaturated and trans fatty acids, correlations between intakes and biomarkers improved moderately when average intakes over previous years were used. Erythrocyte n-3 fatty acids of marine origin and trans fatty acid content are suitable biomarkers for long-term intake.
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                Author and article information

                Journal
                Food Nutr Res
                Food Nutr Res
                FNR
                Food & Nutrition Research
                Co-Action Publishing
                1654-6628
                1654-661X
                03 February 2015
                2015
                : 59
                : 10.3402/fnr.v59.25487
                Affiliations
                [1 ]Frambu Resource Centre for Rare Disorders, Siggerud, Norway
                [2 ]Department of Nutrition, Institute of Basic Medical Sciences, University of Oslo, Oslo, Norway
                [3 ]Institute of Clinical Medicine, University of Oslo, Oslo, Norway
                [4 ]Department of Medical Genetics, Oslo University Hospital, Oslo, Norway
                Author notes
                [* ] Marianne Nordstrøm, Frambu Resource Centre for Rare Disorders, Sandbakkveien 18, NO-1404 Siggerud, Norway. Email: mal@ 123456frambu.no

                Responsible Editor: Anna Ólafsdóttir, University of Iceland, Iceland.

                Article
                25487
                10.3402/fnr.v59.25487
                4317472
                25653019
                33f1fbf9-b8cd-411d-83bd-9b5b58d1be19
                © 2015 Marianne Nordstrøm et al.

                This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

                History
                : 18 July 2014
                : 05 November 2014
                : 22 December 2014
                Categories
                Original Article

                Nutrition & Dietetics
                diet,carotenoids,omega-3 fatty acids,obesity,intellectual disability,autonomy,developmental disability,living arrangements

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