{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,2]],"date-time":"2026-10-02T11:13:39Z","timestamp":1790939619233,"version":"4.1.0"},"reference-count":35,"publisher":"MDPI AG","issue":"10","license":[{"start":{"date-parts":[[2019,10,14]],"date-time":"2019-10-14T00:00:00Z","timestamp":1571011200000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"name":"Nutricia Ltd","award":["n\/a"],"award-info":[{"award-number":["n\/a"]}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Nutrients"],"abstract":"<jats:p>The nutritional and metabolic characteristics of adult phenylketonuria (PKU) patients in the UK with varying dietary adherence is unknown. In other countries, nutritional and metabolic abnormalities have been reported in nonadherent patients compared to adherent counterparts. A pooled analysis of primary baseline data from two UK multi-centre studies was therefore performed to establish whether this is true from a UK perspective. Adult PKU patients who had provided 3-day food records and amino acid blood samples were included and grouped according to dietary adherence (adherent; n = 16 vs. nonadherent; n = 14). Nonadherent patients consumed greater amounts of natural protein compared to adherent patients (61.6 \u00b1 30.7 vs. 18.3 \u00b1 7.7 g\/day; q &lt; 0.001). In contrast, the contribution of protein substitutes to total protein intake was lower in nonadherent compared to adherent patients (3.9 \u00b1 9.2 g\/day vs. 58.6 \u00b1 10.2 g\/day; q &lt; 0.001). Intakes of iron, zinc, vitamin D3, magnesium, calcium, selenium, iodine, vitamin C, vitamin A and copper were significantly lower in nonadherent compared to adherent patients and were below UK Reference Nutrient Intakes. Similarly, intakes of thiamin, riboflavin, niacin, vitamin B6 and phosphorus were significantly lower in nonadherent compared to adherent patients but met the UK Reference Nutrient Intakes. Phenylalanine concentrations in nonadherent patients were significantly higher than adherent patients (861 \u00b1 348 vs. 464 \u00b1 196 \u00b5mol\/L; q = 0.040) and fell outside of European treatment target ranges. This study shows the nutritional and metabolic consequences of deviation from phenylalanine restriction and intake of PKU protein substitutes in nonadherent adult PKU patients. Collectively, these data further underlie the importance of life-long adherence to the PKU diet.<\/jats:p>","DOI":"10.3390\/nu11102459","type":"journal-article","created":{"date-parts":[[2019,10,14]],"date-time":"2019-10-14T12:14:05Z","timestamp":1571055245000},"page":"2459","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":30,"title":["Nutritional and Metabolic Characteristics of UK Adult Phenylketonuria Patients with Varying Dietary Adherence"],"prefix":"10.3390","volume":"11","author":[{"given":"Benjamin","family":"Green","sequence":"first","affiliation":[{"name":"Medical Affairs, Nutricia Advanced Medical Nutrition, Wiltshire BA14 0XQ, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Robert","family":"Browne","sequence":"additional","affiliation":[{"name":"Medical Affairs, Nutricia Advanced Medical Nutrition, Wiltshire BA14 0XQ, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sarah","family":"Firman","sequence":"additional","affiliation":[{"name":"Guy\u2019s and St Thomas\u2019 NHS Foundation Trust, London SE1 9RT, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Melanie","family":"Hill","sequence":"additional","affiliation":[{"name":"Sheffield Teaching Hospitals NHS Foundation Trust, Sheffield S10 2JF, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yusof","family":"Rahman","sequence":"additional","affiliation":[{"name":"Guy\u2019s and St Thomas\u2019 NHS Foundation Trust, London SE1 9RT, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kit","family":"Kaalund Hansen","sequence":"additional","affiliation":[{"name":"University College London Hospitals NHS Foundation Trust, London WC1N 3BG, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sarah","family":"Adam","sequence":"additional","affiliation":[{"name":"Royal Hospital for Children, Glasgow G51 4TF, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Rachel","family":"Skeath","sequence":"additional","affiliation":[{"name":"Great Ormond Street Hospital for Children NHS Foundation Trust, London WC1N 3JH, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Paula","family":"Hallam","sequence":"additional","affiliation":[{"name":"Great Ormond Street Hospital for Children NHS Foundation Trust, London WC1N 3JH, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ide","family":"Herlihy","sequence":"additional","affiliation":[{"name":"Great Ormond Street Hospital for Children NHS Foundation Trust, London WC1N 3JH, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Fiona","family":"Jenkinson","sequence":"additional","affiliation":[{"name":"Royal Victoria Infirmary, Newcastle upon Tyne NE1 4LP, 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2TH, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-5127-8454","authenticated-orcid":false,"given":"Louise","family":"Robertson","sequence":"additional","affiliation":[{"name":"Queen Elizabeth Hospital, Birmingham B15 2TH, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Carla","family":"Fitzachary","sequence":"additional","affiliation":[{"name":"Guy\u2019s and St Thomas\u2019 NHS Foundation Trust, London SE1 9RT, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Heidi","family":"Chan","sequence":"additional","affiliation":[{"name":"Medical Affairs, Nutricia Advanced Medical Nutrition, Wiltshire BA14 0XQ, UK"},{"name":"Guy\u2019s and St Thomas\u2019 NHS Foundation Trust, London SE1 9RT, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Arlene","family":"Slabbert","sequence":"additional","affiliation":[{"name":"Guy\u2019s and St Thomas\u2019 NHS Foundation Trust, London SE1 9RT, 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