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

摘要

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.