Nutrient Status and Intakes of Adults with Phenylketonuria.
amino acid
cognitive wellbeing
micronutrient
nutritional status
phenylalanine hydroxylase deficiency
Journal
Nutrients
ISSN: 2072-6643
Titre abrégé: Nutrients
Pays: Switzerland
ID NLM: 101521595
Informations de publication
Date de publication:
15 Aug 2024
15 Aug 2024
Historique:
received:
26
07
2024
revised:
12
08
2024
accepted:
14
08
2024
medline:
1
9
2024
pubmed:
31
8
2024
entrez:
29
8
2024
Statut:
epublish
Résumé
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 ± SD, age: 28 ± 9 years, phenylalanine concentration: 847 ± 285 µmol/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 (
Identifiants
pubmed: 39203860
pii: nu16162724
doi: 10.3390/nu16162724
pii:
doi:
Substances chimiques
Phenylalanine
47E5O17Y3R
Amino Acids
0
Vitamins
0
Micronutrients
0
Types de publication
Journal Article
Langues
eng
Sous-ensembles de citation
IM
Subventions
Organisme : Danone Research & Innovation
ID : N/A