Phenylketonuria: The Diet Basics

Abstract

Core Messages • The goal of nutrition management of phenylketonuria (PKU) is to maintain blood phenylalanine concentrations between 120 and 360 μmol/L. • The diet for PKU includes medical foods low in or devoid of phenylalanine and limited quantities of phenylalanine from intact protein sources. • Frequent monitoring of blood phenylal- anine concentrations is key to successful diet management. • Frequent adjustments in the diet are needed to achieve desired blood phenyl- alanine concentrations as well as to pro- mote normal growth and development. • A variety of PKU medical foods and modifi ed low- protein foods are avail- able to accommodate different nutrient needs and taste preferences throughout the life span. • Maintaining the diet is challenging for many patients with PKU; alterna- tive therapies are available, but most still require some degree of diet modification.