Glycomacropeptide for nutritional management of phenylketonuria: a randomized, controlled, crossover trial.

ABSTRACT:

Background

To prevent cognitive impairment, phenylketonuria requires lifelong management of blood phenylalanine (Phe) concentration with a low-Phe diet. The diet restricts intake of Phe from natural proteins in combination with traditional amino acid medical foods (AA-MFs) or glycomacropeptide medical foods (GMP-MFs) that contain primarily intact protein and a small amount of Phe.

Objective

We investigated the efficacy and safety of a low-Phe diet combined with GMP-MFs or AA-MFs providing the same quantity of protein equivalents in free-living subjects with phenylketonuria.

Design

This 2-stage, randomized crossover trial included 30 early-treated phenylketonuria subjects (aged 15-49 y), 20 with classical and 10 with variant phenylketonuria. Subjects consumed, in rando

SUBMITTER: Ney DM 

PROVIDER: S-EPMC4962165 | biostudies-literature | 2016 Aug

REPOSITORIES: biostudies-literature