Nutritional and Metabolic Characteristics of UK Adult Phenylketonuria Patients with Varying Dietary Adherence
| Autor: | Melanie Hill, Charlotte Dawson, Camille Newby, Victoria Bittle, Fiona Jenkinson, Kit Kaalund Hansen, Claire Nicol, Louise Robertson, Alison Cozens, Rachel Skeath, Sarah Firman, Arlene Slabbert, Rebecca J. Stratton, Heidi Chan, Sarah Adam, Lisa Gaff, Carolyn Dunlop, Sandra Adams, Robert Browne, S. Donald, Ide Herlihy, Carla Fitzachary, Yusof Rahman, Paula Hallam, Gary P. Hubbard, Benjamin Green |
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| Rok vydání: | 2019 |
| Předmět: |
Adult
Male 0301 basic medicine Vitamin congenital hereditary and neonatal diseases and abnormalities Adolescent phenylalanine phenylketonuria Nutritional Status chemistry.chemical_element Physiology lcsh:TX341-641 Phenylalanine Riboflavin 030105 genetics & heredity Iodine Article Young Adult 03 medical and health sciences chemistry.chemical_compound 0302 clinical medicine Phenylketonurias Humans Medicine Micronutrients adherence Amino Acids Young adult Nutrition and Dietetics nonadherence nutrient intake Vitamin C business.industry nutritional and metabolic diseases United Kingdom Diet Pooled analysis chemistry PKU Dietary Supplements Patient Compliance Female Energy Intake business lcsh:Nutrition. Foods and food supply 030217 neurology & neurosurgery Niacin Food Science |
| Zdroj: | Nutrients Nutrients, Vol 11, Iss 10, p 2459 (2019) Volume 11 Issue 10 |
| ISSN: | 2072-6643 |
| DOI: | 10.3390/nu11102459 |
| Popis: | 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 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 µ mol/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. |
| Databáze: | OpenAIRE |
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