{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,2,5]],"date-time":"2026-02-05T05:35:36Z","timestamp":1770269736011,"version":"3.49.0"},"reference-count":50,"publisher":"MDPI AG","issue":"16","license":[{"start":{"date-parts":[[2024,8,15]],"date-time":"2024-08-15T00:00:00Z","timestamp":1723680000000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"name":"Danone Research &amp; Innovation"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Nutrients"],"abstract":"<jats:p>A phenylalanine-restricted diet, supplemented with protein substitutes (PSs), remains the cornerstone of phenylketonuria (PKU) management. However, adherence is challenging in adulthood, and data on the nutritional status of early and continuously treated adults with PKU (ETAwPKU) are scarce. A total of 34 ETAwPKU (16 females; mean \u00b1 SD, age: 28 \u00b1 9 years, phenylalanine concentration: 847 \u00b1 285 \u00b5mol\/L) and 34 age- and sex-matched control subjects were compared regarding their blood nutrient status, self-reported dietary intake, and cognitive wellbeing. Though diet adherence varied, all ETAwPKU were taking a PS. No significant differences were found for blood DHA, calcium, ferritin, transferrin, and zinc concentrations. However, selenium and ubiquinone concentrations were 16% and 29% lower in ETAwPKU, respectively (p &lt; 0.01 and &lt;0.0001). Vitamin concentrations (D, B12, B6, and folic acid) were significantly higher in ETAwPKU except for alpha-tocopherol. Amino acid (AA) concentrations differed between ETAwPKU and controls: they were significantly lower for 12 AAs and higher for phenylalanine and glycine. ETAwPKU had a significantly higher intake of most minerals and vitamins, except for niacin and phosphorus (no difference). Depending on the nutrient, PSs represented 52\u2013100% of patients\u2019 daily intake and 19% of total daily energy intake. Compared with controls, ETAwPKU scored significantly lower in three of the four subscales of the cognitive wellbeing questionnaire. Overall, the blood DHA and micronutrient status of ETAwPKU was adequate, except for selenium, with higher intakes than controls for most micronutrients. Patients relied heavily on PSs to meet the recommended intakes for protein, DHA, and micronutrients. The potential clinical impact of differences found in AA status should be further studied.<\/jats:p>","DOI":"10.3390\/nu16162724","type":"journal-article","created":{"date-parts":[[2024,8,16]],"date-time":"2024-08-16T04:29:57Z","timestamp":1723782597000},"page":"2724","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":6,"title":["Nutrient Status and Intakes of Adults with Phenylketonuria"],"prefix":"10.3390","volume":"16","author":[{"given":"Eva","family":"Venegas","sequence":"first","affiliation":[{"name":"Endocrinology and Nutrition Department, Hospital Universitario Virgen del Roc\u00edo, 41013 Sevilla, Spain"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-7604-3013","authenticated-orcid":false,"given":"Simone","family":"Langeveld","sequence":"additional","affiliation":[{"name":"Danone Research & Innovation, 3584 CT Utrecht, The Netherlands"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Kirsten","family":"Ahring","sequence":"additional","affiliation":[{"name":"Department of Phenylketonuria, Copenhagen University Hospital, 2100 Copenhagen, Denmark"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Rosa","family":"Benitez","sequence":"additional","affiliation":[{"name":"Endocrinology and Nutrition Department, Hospital Universitario Virgen del Roc\u00edo, 41013 Sevilla, Spain"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"An","family":"Desloovere","sequence":"additional","affiliation":[{"name":"Department of Pediatric Neurology, Center for Inherited Metabolic Disorders, University Hospital Ghent, European Reference Network for Hereditary Metabolic Disorders (MetabERN), 9000 Ghent, Belgium"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Elena","family":"Dios","sequence":"additional","affiliation":[{"name":"Endocrinology and Nutrition Department, Hospital Universitario Virgen del Roc\u00edo, 41013 Sevilla, Spain"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Eva","family":"G\u00f3mez","sequence":"additional","affiliation":[{"name":"Unit for Diagnosis and Treatment of Congenital Metabolic Disorders, University Clinical Hospital of Santiago de Compostela, Health Research Institute of Santiago de Compostela (IDIS), European Reference Network for Hereditary Metabolic Disorders (MetabERN), 15706 Santiago de Compostela, Spain"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-3757-262X","authenticated-orcid":false,"given":"Alvaro","family":"Hermida","sequence":"additional","affiliation":[{"name":"Unit for Diagnosis and Treatment of Congenital Metabolic Disorders, University Clinical Hospital of Santiago de Compostela, Health Research Institute of Santiago de Compostela (IDIS), European Reference Network for Hereditary Metabolic Disorders (MetabERN), 15706 Santiago de Compostela, Spain"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-4725-6359","authenticated-orcid":false,"given":"Cyril","family":"Marsaux","sequence":"additional","affiliation":[{"name":"Danone Research & Innovation, 3584 CT Utrecht, The Netherlands"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-2077-0275","authenticated-orcid":false,"given":"Patrick","family":"Verloo","sequence":"additional","affiliation":[{"name":"Department of Pediatric Neurology, Center for Inherited Metabolic Disorders, University Hospital Ghent, European Reference Network for Hereditary Metabolic Disorders (MetabERN), 9000 Ghent, Belgium"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-4861-9905","authenticated-orcid":false,"given":"Maria-Luz","family":"Couce","sequence":"additional","affiliation":[{"name":"Unit for Diagnosis and Treatment of Congenital Metabolic Disorders, University Clinical Hospital of Santiago de Compostela, Health Research Institute of Santiago de Compostela (IDIS), European Reference Network for Hereditary Metabolic Disorders (MetabERN), 15706 Santiago de Compostela, Spain"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"1968","published-online":{"date-parts":[[2024,8,15]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"1417","DOI":"10.1016\/S0140-6736(10)60961-0","article-title":"Phenylketonuria","volume":"376","author":"Blau","year":"2010","journal-title":"Lancet"},{"key":"ref_2","doi-asserted-by":"crossref","first-page":"531","DOI":"10.1007\/s00415-019-09608-2","article-title":"Neurological manifestations in adults with phenylketonuria: New cases and review of the literature","volume":"267","author":"Jaulent","year":"2020","journal-title":"J. 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