{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,8]],"date-time":"2026-09-08T16:57:43Z","timestamp":1788886663339,"version":"build-2803163510"},"reference-count":29,"publisher":"Wiley","issue":"4","license":[{"start":{"date-parts":[[2023,4,11]],"date-time":"2023-04-11T00:00:00Z","timestamp":1681171200000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc-nd\/4.0\/"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["J Human Nutrition Diet"],"published-print":{"date-parts":[[2023,8]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:sec>\n                    <jats:title>Background<\/jats:title>\n                    <jats:p>Adrenoleukodystrophy (ALD) is a rare X\u2010linked metabolic disorder that causes the accumulation of very\u2010long\u2010chain fatty acids (VLCFAs) (C26:0) and the subsequent variety of clinical and neurological symptoms. Little is known about nutritional status and dietary habits of children affected by ALD, and so the present study aimed to assess nutritional status and food intake in children with ALD, also exploring the relationship between food intake and the consumption of disease\u2010specific dietary supplements to reduce blood C26:0 concentrations and increase monounsaturated fatty acids (C26:1).<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods<\/jats:title>\n                    <jats:p>All patients underwent a clinical and neurological evaluation and a comprehensive nutritional assessment. The association of VLCFA concentrations with dietary lipids was assessed.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>\n                      Nine boys (11.49\u2009\u00b1\u20093.61 years) were enrolled in a cross\u2010sectional study. All patients were normal weight, with normal resting energy expenditure. Only six of nine patients followed the low\u2010fat diet and dietary supplements. An inverse association was found between the food intake of polyunsaturated lipids and C26:0; conversely, the C26:0 was positively associated with the dietary saturated lipids. When consumed, dietary supplement consumption correlated positively with C26:1 (\n                      <jats:italic>\u03c1<\/jats:italic>\n                      \u2009=\u20090.917,\n                      <jats:italic>p<\/jats:italic>\n                      \u2009=\u20090.029) and no correlation was found with C26:0 (\n                      <jats:italic>\u03c1<\/jats:italic>\n                      \u2009=\u20090.410,\n                      <jats:italic>p<\/jats:italic>\n                      \u2009=\u20090,493).\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusions<\/jats:title>\n                    <jats:p>No children were found to be malnourished or overweight or obese; however, half of the children reported excessive body fat, probably as a result of the pharmacotherapies. A low\u2010fat diet could be adjuvant in the management of the accumulation of VLCFAs, but poor dietary compliance to disease\u2010specific nutritional guidelines appears to be a major problem of this condition and underlines the need for a structured and personalised nutritional management in ALD disease.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1111\/jhn.13173","type":"journal-article","created":{"date-parts":[[2023,3,30]],"date-time":"2023-03-30T00:45:02Z","timestamp":1680137102000},"page":"1316-1326","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":3,"title":["Nutritional status of children affected by X\u2010linked adrenoleukodystrophy"],"prefix":"10.1111","volume":"36","author":[{"given":"Isabella","family":"Moroni","sequence":"first","affiliation":[{"name":"Department of Pediatric Neuroscience Fondazione IRCCS Istituto Neurologico Besta Milan Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-0403-5465","authenticated-orcid":false,"given":"Ramona","family":"De Amicis","sequence":"additional","affiliation":[{"name":"Department of Food Environmental and Nutritional Sciences (DeFENS), International Center for the Assessment of Nutritional Status (ICANS) University of Milan Milan Italy"},{"name":"Laboratory of Nutrition and Obesity Research, Department of Endocrine and Metabolic Diseases IRCCS Istituto Auxologico Italiano Milan Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Anna","family":"Ardissone","sequence":"additional","affiliation":[{"name":"Department of Pediatric Neuroscience Fondazione IRCCS Istituto Neurologico Besta Milan Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Simone","family":"Ravella","sequence":"additional","affiliation":[{"name":"Department of Food Environmental and Nutritional Sciences (DeFENS), International Center for the Assessment of Nutritional Status (ICANS) University of Milan Milan Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Simona","family":"Bertoli","sequence":"additional","affiliation":[{"name":"Department of Food Environmental and Nutritional Sciences (DeFENS), International Center for the Assessment of Nutritional Status (ICANS) University of Milan Milan Italy"},{"name":"Laboratory of Nutrition and Obesity Research, Department of Endocrine and Metabolic Diseases IRCCS Istituto Auxologico Italiano Milan Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2023,4,11]]},"reference":[{"key":"e_1_2_9_2_1","doi-asserted-by":"publisher","DOI":"10.1002\/jdn.10003"},{"key":"e_1_2_9_3_1","doi-asserted-by":"publisher","DOI":"10.1007\/s10354-016-0534-2"},{"key":"e_1_2_9_4_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.atherosclerosis.2008.05.030"},{"key":"e_1_2_9_5_1","doi-asserted-by":"publisher","DOI":"10.1016\/0006-291x(80)91182-1"},{"key":"e_1_2_9_6_1","doi-asserted-by":"publisher","DOI":"10.1093\/ajcn\/40.2.277"},{"key":"e_1_2_9_7_1","doi-asserted-by":"publisher","DOI":"10.3177\/jnsv.34.633"},{"key":"e_1_2_9_8_1","doi-asserted-by":"publisher","DOI":"10.1212\/wnl.36.3.357"},{"key":"e_1_2_9_9_1","doi-asserted-by":"publisher","DOI":"10.1007\/s10545-011-9432-3"},{"key":"e_1_2_9_10_1","doi-asserted-by":"publisher","DOI":"10.1002\/ana.22363"},{"key":"e_1_2_9_11_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.biochi.2013.09.012"},{"key":"e_1_2_9_12_1","doi-asserted-by":"publisher","DOI":"10.1001\/archneur.62.7.1073"},{"key":"e_1_2_9_13_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.bbalip.2007.12.003"},{"key":"e_1_2_9_14_1","first-page":"1761","article-title":"Adrenoleukodystrophy: a scoring method for brain MR observations","volume":"15","author":"Loes DJ","year":"1994","journal-title":"AJNR Am J Neuroradiol"},{"key":"e_1_2_9_15_1","doi-asserted-by":"publisher","DOI":"10.1023\/A:1005612214179"},{"key":"e_1_2_9_16_1","volume-title":"Anthropometric standardization reference manual","author":"Lohman TG","year":"1988"},{"key":"e_1_2_9_17_1","first-page":"1","article-title":"2000 CDC Growth Charts for the United States: methods and development","volume":"11","author":"Kuczmarski RJ","year":"2002","journal-title":"Vital Health Stat 11"},{"key":"e_1_2_9_18_1","doi-asserted-by":"publisher","DOI":"10.1038\/sj.ejcn.1601240"},{"key":"e_1_2_9_19_1","doi-asserted-by":"publisher","DOI":"10.1093\/gerona\/57.10.B359"},{"key":"e_1_2_9_20_1","doi-asserted-by":"crossref","unstructured":"FrisanchoA. 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