Nutrition Management of Phenylketonuria

Sandy van Calcar

doi:10.1007/978-3-030-94510-7_10

Nutrition Management of Phenylketonuria

Sandy van Calcar

Molecular and Medical Genetics

Research output: Chapter in Book/Report/Conference proceeding › Chapter

1 Scopus citations

Abstract

The goal of nutrition management of phenylketonuria (PKU) is to maintain blood phenylalanine concentrations between 120 and 360 µmol/L (2-6 mg/dL). 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 phenylalanine concentrations is key to successful diet management. Frequent diet adjustments are needed to achieve desired blood phenylalanine concentrations, as well as to promote normal growth and feeding development. A variety of PKU medical foods and modified low protein foods are available to accommodate different nutrient needs and taste preferences throughout the lifespan. Maintaining the diet is challenging for many patients with PKU; alternative therapies are available that may increase phenylalanine tolerance and diet sustainability.

Original language	English (US)
Title of host publication	Nutrition Management of Inherited Metabolic Diseases
Subtitle of host publication	Lessons from Metabolic University: Second Edition
Publisher	Springer International Publishing
Pages	127-151
Number of pages	25
ISBN (Electronic)	9783030945107
ISBN (Print)	9783030945091
DOIs	https://doi.org/10.1007/978-3-030-94510-7_10
State	Published - Jan 1 2022

ASJC Scopus subject areas

General Medicine
General Engineering

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10.1007/978-3-030-94510-7_10

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abstract = "The goal of nutrition management of phenylketonuria (PKU) is to maintain blood phenylalanine concentrations between 120 and 360 µmol/L (2-6 mg/dL). 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 phenylalanine concentrations is key to successful diet management. Frequent diet adjustments are needed to achieve desired blood phenylalanine concentrations, as well as to promote normal growth and feeding development. A variety of PKU medical foods and modified low protein foods are available to accommodate different nutrient needs and taste preferences throughout the lifespan. Maintaining the diet is challenging for many patients with PKU; alternative therapies are available that may increase phenylalanine tolerance and diet sustainability.",

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Nutrition Management of Phenylketonuria

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