Nutrient intake, body composition, and blood phenylalanine control in children with phenylketonuria compared to healthy controls.

ABSTRACT:

Background

Phenylketonuria (PKU) treatment consists of life-long protein restriction and Phe-free medical foods for adequate nutritional intake and growth. A relationship between body composition and blood phenylalanine (Phe) concentrations in subjects with PKU has been proposed but this has not been consistently reported.

Methods

Dietary intake, lean body mass (LBM) and fat mass (FM) were measured in 30 pediatric subjects with PKU compared to 30 age, and sex matched controls. The relationship between body composition and blood Phe was analyzed within the PKU cohort from clinically collected dried blood spot Phe concentrations.

Results

Male subjects with PKU had less LBM% and more FM% than controls (p = .024). There was no difference in LBM% and FM% among femal

SUBMITTER: Sailer M 

PROVIDER: S-EPMC7218298 | biostudies-literature | 2020 Jun

REPOSITORIES: biostudies-literature