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dc.contributor.authorRoss, Lynda J
dc.contributor.authorCapra, Sandra
dc.contributor.authorBaguley, Brenton
dc.contributor.authorSinclair, Kate
dc.contributor.authorMunro, Kate
dc.contributor.authorLewindon, Peter
dc.contributor.authorLavin, Martin
dc.date.accessioned2019-03-29T03:20:43Z
dc.date.available2019-03-29T03:20:43Z
dc.date.issued2015
dc.identifier.issn1034-4810
dc.identifier.doi10.1111/jpc.12828
dc.identifier.urihttp://hdl.handle.net/10072/171697
dc.description.abstractAim: Ataxia‐telangiectasia (A‐T) is a rare genomic syndrome resulting in severe disability. Chronic childhood disorders can profoundly influence growth and development. Nutrition‐related issues in A‐T are not well described, and there are no nutritional guidelines. This study investigated the nutrition‐related characteristics and behaviours of Australian A‐T patients attending a national clinic. Methods: A cross‐sectional analysis of 13 A‐T patients (nine females; aged: 4–23 years): nutritional status was assessed by anthropometric and body cell mass (BCM) calculations. Parents reported their child's diet history and physical and behavioural factors that affect nutrition including fatigue and need for assistance. Results: Ten (77%) had short stature (height for age z scores <−1), and seven (54%) were underweight for height (weight/height z scores <−1). Significant malnutrition (BCM z scores <−2) was detected in nine (69%) including the one adult who was severely malnourished. Malnutrition increased significantly with age (BCM for height z scores and age, r = −0.937, P < 0.001). Eight (62%) patients ate poorly compared with estimated energy requirement for weight. Poor diet quality was characterised by high fat and sugar choices. Parents reported significant nutritional barriers as chronic tiredness and the need for care giver assistance with meals. Conclusions: This study confirms profound malnutrition in Australian A‐T patients. Poor intakes and diet quality suggest the need for early nutrition intervention. Ongoing support for families and early discussions on tube feeding are required to address changing needs in childhood and likely nutritional decline into adulthood. A prospective study is required to assess feasibility and effectiveness of nutrition interventions in young people with A‐T.
dc.description.peerreviewedYes
dc.languageEnglish
dc.publisherWiley-Blackwell Publishing
dc.relation.ispartofpagefrom802
dc.relation.ispartofpageto807
dc.relation.ispartofissue8
dc.relation.ispartofjournalJournal of Paediatrics and Child Health
dc.relation.ispartofvolume51
dc.subject.fieldofresearchNutrition and Dietetics not elsewhere classified
dc.subject.fieldofresearchPaediatrics and Reproductive Medicine
dc.subject.fieldofresearchcode111199
dc.subject.fieldofresearchcode1114
dc.titleNutritional status of patients with Ataxia-Telangiectasia: A case for early and ongoing nutrition support and intervention
dc.typeJournal article
dc.type.descriptionC1 - Articles
dc.type.codeC - Journal Articles
dc.description.versionPost-print
gro.rights.copyright© 2015 Paediatrics and Child Health Division (Royal Australasian College of Physicians). This is the peer reviewed version of the following article: Nutritional status of patients with ataxia‐telangiectasia: A case for early and ongoing nutrition support and intervention, Journal of Paediatrics and Child Health, Volume51, Issue8, August 2015, Pages 802-807, which has been published in final form at https://doi.org/10.1111/jpc.12828. This article may be used for non-commercial purposes in accordance with Wiley Terms and Conditions for Self-Archiving (http://olabout.wiley.com/WileyCDA/Section/id-828039.html)
gro.hasfulltextFull Text
gro.griffith.authorRoss, Lynda J.


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