{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,9,25]],"date-time":"2025-09-25T16:20:24Z","timestamp":1758817224467},"reference-count":33,"publisher":"Wiley","issue":"8","license":[{"start":{"date-parts":[[2015,2,6]],"date-time":"2015-02-06T00:00:00Z","timestamp":1423180800000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"funder":[{"name":"RBWH Research Foundation"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["J Paediatrics Child Health"],"published-print":{"date-parts":[[2015,8]]},"abstract":"<jats:sec><jats:title>Aim<\/jats:title><jats:p>Ataxia\u2010telangiectasia (<jats:styled-content style=\"fixed-case\">A<\/jats:styled-content>\u2010<jats:styled-content style=\"fixed-case\">T<\/jats:styled-content>) is a rare genomic syndrome resulting in severe disability. Chronic childhood disorders can profoundly influence growth and development. Nutrition\u2010related issues in <jats:styled-content style=\"fixed-case\">A<\/jats:styled-content>\u2010<jats:styled-content style=\"fixed-case\">T<\/jats:styled-content> are not well described, and there are no nutritional guidelines. This study investigated the nutrition\u2010related characteristics and behaviours of <jats:styled-content style=\"fixed-case\">A<\/jats:styled-content>ustralian <jats:styled-content style=\"fixed-case\">A<\/jats:styled-content>\u2010<jats:styled-content style=\"fixed-case\">T<\/jats:styled-content> patients attending a national clinic.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>A cross\u2010sectional analysis of 13 <jats:styled-content style=\"fixed-case\">A<\/jats:styled-content>\u2010<jats:styled-content style=\"fixed-case\">T<\/jats:styled-content> patients (nine females; aged: 4\u201323 years): nutritional status was assessed by anthropometric and body cell mass (<jats:styled-content style=\"fixed-case\">BCM<\/jats:styled-content>) calculations. Parents reported their child's diet history and physical and behavioural factors that affect nutrition including fatigue and need for assistance.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>Ten (77%) had short stature (height for age <jats:italic>z<\/jats:italic> scores &lt;\u22121), and seven (54%) were underweight for height (weight\/height <jats:italic>z<\/jats:italic> scores &lt;\u22121). Significant malnutrition (BCM <jats:italic>z<\/jats:italic> scores &lt;\u22122) was detected in nine (69%) including the one adult who was severely malnourished. Malnutrition increased significantly with age (BCM for height <jats:italic>z<\/jats:italic> scores and age, <jats:italic>r<\/jats:italic> = \u22120.937, <jats:italic>P<\/jats:italic> &lt; 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.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusions<\/jats:title><jats:p>This study confirms profound malnutrition in <jats:styled-content style=\"fixed-case\">A<\/jats:styled-content>ustralian <jats:styled-content style=\"fixed-case\">A<\/jats:styled-content>\u2010<jats:styled-content style=\"fixed-case\">T<\/jats:styled-content> 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 <jats:styled-content style=\"fixed-case\">A<\/jats:styled-content>\u2010<jats:styled-content style=\"fixed-case\">T<\/jats:styled-content>.<\/jats:p><\/jats:sec>","DOI":"10.1111\/jpc.12828","type":"journal-article","created":{"date-parts":[[2015,2,6]],"date-time":"2015-02-06T08:17:55Z","timestamp":1423210675000},"page":"802-807","update-policy":"http:\/\/dx.doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":33,"title":["Nutritional status of patients with ataxia\u2010telangiectasia: A case for early and ongoing nutrition support and intervention"],"prefix":"10.1111","volume":"51","author":[{"given":"Lynda J","family":"Ross","sequence":"first","affiliation":[{"name":"Department of Nutrition and Dietetics The Royal Brisbane &amp; Women's Hospital  Brisbane Queensland Australia"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Sandra","family":"Capra","sequence":"additional","affiliation":[{"name":"School of Human Movement and Nutrition Sciences The University of Queensland  Brisbane Queensland Australia"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Brenton","family":"Baguley","sequence":"additional","affiliation":[{"name":"School of Human Movement and Nutrition Sciences The University of Queensland  Brisbane Queensland Australia"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Kate","family":"Sinclair","sequence":"additional","affiliation":[{"name":"Department of Neurology The Royal Children's Hospital Brisbane  Brisbane Queensland Australia"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Kate","family":"Munro","sequence":"additional","affiliation":[{"name":"Department of Neurology The Royal Children's Hospital Brisbane  Brisbane Queensland Australia"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Peter","family":"Lewindon","sequence":"additional","affiliation":[{"name":"School of Human Movement and Nutrition Sciences The University of Queensland  Brisbane Queensland Australia"},{"name":"Department of Neurology The Royal Children's Hospital Brisbane  Brisbane Queensland Australia"},{"name":"Department of Gastroenterology The Royal Children's Hospital  Brisbane Queensland Australia"},{"name":"School of Medicine The University of Queensland  Brisbane Queensland Australia"},{"name":"Queensland Institute of Medical Research (QIMR)  Brisbane Queensland Australia"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Martin","family":"Lavin","sequence":"additional","affiliation":[{"name":"The University of Queensland Centre for Clinical Research  Brisbane Queensland Australia"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2015,2,6]]},"reference":[{"key":"e_1_2_8_2_1","doi-asserted-by":"publisher","DOI":"10.1038\/nrm2514"},{"key":"e_1_2_8_3_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jpeds.2009.12.007"},{"key":"e_1_2_8_4_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jaci.2011.03.052"},{"key":"e_1_2_8_5_1","doi-asserted-by":"publisher","DOI":"10.1136\/adc.2006.094268"},{"key":"e_1_2_8_6_1","doi-asserted-by":"publisher","DOI":"10.1136\/bmj.2.5912.189"},{"key":"e_1_2_8_7_1","doi-asserted-by":"publisher","DOI":"10.1136\/gut.34.7.939"},{"key":"e_1_2_8_8_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-2222.1982.tb02541.x"},{"key":"e_1_2_8_9_1","doi-asserted-by":"publisher","DOI":"10.1136\/thorax.56.10.746"},{"key":"e_1_2_8_10_1","first-page":"169","article-title":"Ataxia telangiectasia in the British Isles: the clinical and laboratory features of 70 affected individuals","volume":"82","author":"Woods CG","year":"1992","journal-title":"Q. 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