Risk of inadequate protein and micronutrient intakes in patients with PKU with an increased phe-tolerance: Impact of a micronutrient-dense protein substitute.

ABSTRACT:

Background

Patients with phenylketonuria (PKU) with higher phenylalanine (phe)-tolerance may have inadequate diets due to high-quality protein restriction and reduced intakes of nutrient-fortified phe-free, tyrosine (tyr)-enriched protein substitutes (PS).

Methods

Open-label, 24-week, multi-centre intervention assessed the impact of a once-daily, micronutrient-dense PS on nutrient intakes in patients with an increased phe-tolerance. Subjects >12 years, consuming 0-25 g protein/day from a regular PS, were recruited. Diet records and fasting blood samples were collected at baseline and study end.

Results

11/13 subjects, 15-49y (5 males), with 6/11 taking tetrahydrobiopterin dihydrochloride (BH4) and/or a regular PS, completed the study. At baseline, protein and some ess

SUBMITTER: Rohde C 

PROVIDER: S-EPMC12528915 | biostudies-literature | 2025 Dec

REPOSITORIES: biostudies-literature