Complications of the Low Phenylalanine Diet for Patients with Phenylketonuria and the Benefits of Increased Natural Protein
Nicole McWhorter, Mesaki Kenneth Ndugga-Kabuye, Marja Puurunen, Sharon L. Ernst
Abstract
Phenylketonuria (PKU) is an inherited disorder in which phenylalanine (Phe) is not correctly metabolized leading to an abnormally high plasma Phe concentration that causes profound neurologic damage if left untreated. The mainstay of treatment for PKU has centered around limiting natural protein in the diet while supplementing with medical foods in order to prevent neurologic injury while promoting growth. This review discusses several deleterious effects of the low Phe diet along with benefits that have been reported for patients with increased natural protein intake while maintaining plasma Phe levels within treatment guidelines.
Topics & Concepts
PhenylalanineLimitingMedicinePhenylketonuriasEndocrinologyInternal medicineNatural historyLow-protein dietChemistryAmino acidBiochemistryEngineeringMechanical engineeringMetabolism and Genetic DisordersDiet and metabolism studiesAmino Acid Enzymes and Metabolism