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This study evaluated dietary patterns and physical activity levels in children with different PKU phenotypes compared to healthy controls. Eighty\u2010two children were recruited (22 classic PKU [cPKU], 21 BH\n                    <jats:sub>4<\/jats:sub>\n                    \u2010responsive PKU, 19 hyperphenylalaninemia, and 20 controls). Anthropometric data, dietary intake, biochemical markers, and physical activity were assessed. Classic PKU (cPKU) subjects exhibited higher carbohydrate and sugar intake than other PKU phenotypes and controls. Notably, 42% of carbohydrate and 17% of sugar intake was from special low\u2010protein foods, and 20% of carbohydrate and 29% of sugar intake was from protein substitutes. Compared to controls, the cPKU group was less physically active and reported a higher frequency of sweet consumption. Ninety percent of PKU had good metabolic control and carbohydrate intake was significantly correlated with HOMA\u2010IR; however, after adjusting for age, only a trend remained (\n                    <jats:italic>p<\/jats:italic>\n                    \u2009=\u20090.08). Participants in the PKU group following a low natural protein diet consumed more carbohydrate and sugars than those on a normal\u2010protein diet. Multivariate regression analysis showed that the low natural protein diet group was significantly associated with higher levels of vitamin B\n                    <jats:sub>12<\/jats:sub>\n                    , linoleic acid, \u03b1\u2010linolenic acid, eicosapentaenoic acid, and docosahexaenoic acid, and with lower levels of total cholesterol and HDL\u2010C compared to the normal\u2010protein diet group. In conclusion, children with PKU, particularly those with classical PKU following low\u2010protein diets, showed higher carbohydrate intake and distinct micronutrient and lipid profiles compared with healthy controls.\n                  <\/jats:p>","DOI":"10.1002\/jimd.70196","type":"journal-article","created":{"date-parts":[[2026,5,4]],"date-time":"2026-05-04T02:01:33Z","timestamp":1777860093000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":1,"title":["Comparative Analysis of Dietary Patterns in Children With Phenylketonuria Phenotypes and Controls: Implications for Nutritional Status"],"prefix":"10.1002","volume":"49","author":[{"given":"Dolores","family":"Garcia\u2010Arenas","sequence":"first","affiliation":[{"name":"Nutrition, Food Science and Gastronomy Department, Faculty of Pharmacy and Food Science Food Science and Nutrition Torribera Campus, University of Barcelona  Barcelona Spain"},{"name":"Inborn Errors of 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Department, Faculty of Pharmacy and Food Science Food Science and Nutrition Torribera Campus, University of Barcelona  Barcelona Spain"},{"name":"Institute for Research on Nutrition and Food Safety (INSA\u2010UB), Universitat de Barcelona  Barcelona Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Arnau","family":"Gonzalez\u2010Rodriguez","sequence":"additional","affiliation":[{"name":"Nutrition, Food Science and Gastronomy Department, Faculty of Pharmacy and Food Science Food Science and Nutrition Torribera Campus, University of Barcelona  Barcelona Spain"},{"name":"Institute for Research on Nutrition and Food Safety (INSA\u2010UB), Universitat de Barcelona  Barcelona Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Alba","family":"Tor\u2010Roca","sequence":"additional","affiliation":[{"name":"Nutrition, Food Science and Gastronomy Department, Faculty of Pharmacy and Food Science Food Science and Nutrition Torribera Campus, University of Barcelona  Barcelona Spain"},{"name":"Institute for Research on Nutrition and Food Safety (INSA\u2010UB), Universitat de Barcelona  Barcelona Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Rafael","family":"Llorach","sequence":"additional","affiliation":[{"name":"Nutrition, Food Science and Gastronomy Department, Faculty of Pharmacy and Food Science Food Science and Nutrition Torribera Campus, University of Barcelona  Barcelona Spain"},{"name":"Institute for Research on Nutrition and Food Safety (INSA\u2010UB), Universitat de Barcelona  Barcelona Spain"},{"name":"Centro de Investigaci\u00f3n Biom\u00e9dica en Red Fragilidad y Envejecimiento Saludable (CIBERFES), Instituto de Salud Carlos III  Madrid Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jaume","family":"Campistol\u2010Plana","sequence":"additional","affiliation":[{"name":"Inborn Errors of Metabolism Unit, Sant Joan de D\u00e9u Hospital  Barcelona Spain"},{"name":"Institut de Recerca Sant Joan de D\u00e9u  Barcelona Spain"},{"name":"Metabolic Unit, Neuropaediatrics Department Sant Joan de D\u00e9u Hospital  Barcelona Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-4064-5175","authenticated-orcid":false,"given":"Mireia","family":"Urpi\u2010Sarda","sequence":"additional","affiliation":[{"name":"Nutrition, Food Science and Gastronomy Department, Faculty of Pharmacy and Food Science Food Science and Nutrition Torribera Campus, University of Barcelona  Barcelona Spain"},{"name":"Centro de Investigaci\u00f3n Biom\u00e9dica en Red de Enfermedades Raras (CIBERER), Instituto de Salud Carlos III  Madrid Spain"},{"name":"Institute for Research on Nutrition and Food Safety (INSA\u2010UB), Universitat de Barcelona  Barcelona Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"name":"Consortium 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