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The Benefit of Large Neutral Amino Acid Supplementation to a Liberalized Phenylalanine-Restricted Diet in Adult Phenylketonuria Patients: Evidence from Adult Pah-Enu2 Mice
Open AccessArticle

Nutritional and Metabolic Characteristics of UK Adult Phenylketonuria Patients with Varying Dietary Adherence

1
Medical Affairs, Nutricia Advanced Medical Nutrition, Wiltshire BA14 0XQ, UK
2
Guy’s and St Thomas’ NHS Foundation Trust, London SE1 9RT, UK
3
Sheffield Teaching Hospitals NHS Foundation Trust, Sheffield S10 2JF, UK
4
University College London Hospitals NHS Foundation Trust, London WC1N 3BG, UK
5
Royal Hospital for Children, Glasgow G51 4TF, UK
6
Great Ormond Street Hospital for Children NHS Foundation Trust, London WC1N 3JH, UK
7
Royal Victoria Infirmary, Newcastle upon Tyne NE1 4LP, UK
8
Cambridge University Hospitals NHS Foundation Trust, Cambridge CB2 0QQ, UK
9
Queen Elizabeth Hospital, Birmingham B15 2TH, UK
10
Royal Hospital for Sick Children, Edinburgh EH9 1LF, UK
11
Bristol University Hospitals NHS Foundation Trust, Bristol BS1 3NU, UK
12
Faculty of Medicine, University of Southampton, Southampton SO16 6YD, UK
*
Author to whom correspondence should be addressed.
Nutrients 2019, 11(10), 2459; https://doi.org/10.3390/nu11102459
Received: 12 September 2019 / Revised: 30 September 2019 / Accepted: 11 October 2019 / Published: 14 October 2019
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 ± 30.7 vs. 18.3 ± 7.7 g/day; q < 0.001). In contrast, the contribution of protein substitutes to total protein intake was lower in nonadherent compared to adherent patients (3.9 ± 9.2 g/day vs. 58.6 ± 10.2 g/day; q < 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 ± 348 vs. 464 ± 196 µmol/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. View Full-Text
Keywords: phenylketonuria; PKU; adherence; nonadherence; nutrient intake; phenylalanine phenylketonuria; PKU; adherence; nonadherence; nutrient intake; phenylalanine
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Green, B.; Browne, R.; Firman, S.; Hill, M.; Rahman, Y.; Kaalund Hansen, K.; Adam, S.; Skeath, R.; Hallam, P.; Herlihy, I.; Jenkinson, F.; Nicol, C.; Adams, S.; Gaff, L.; Donald, S.; Dawson, C.; Robertson, L.; Fitzachary, C.; Chan, H.; Slabbert, A.; Dunlop, C.; Cozens, A.; Newby, C.; Bittle, V.; Hubbard, G.; Stratton, R. Nutritional and Metabolic Characteristics of UK Adult Phenylketonuria Patients with Varying Dietary Adherence. Nutrients 2019, 11, 2459.

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