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Table of Contents
Vol. 68, No. 2, 2010
Issue release date: June 2010
Section title: Paper
Free Access
Ann Nestlé [Engl] 2010;68:58–69

Nutritional Management of Phenylketonuria

MacLeod E.L. · Ney D.M.
Department of Nutritional Sciences, University of Wisconsin, Madison, Wisc., USA
email Corresponding Author

Denise M. Ney

Department of Nutritional Sciences

University of Wisconsin

1415 Linden Drive, Madison, WI 53706 (USA)

Tel. +1 608 262 4386, Fax +1 608 262 5860, E-Mail ney@nutrisci.wisc.edu

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Phenylketonuria (PKU) is caused by deficient activity of the enzyme phenylalanine hydroxylase, needed to convert the essential amino acid (AA) phenylalanine (phe) to tyrosine. In order to prevent neurological damage, lifelong adherence to a low-phe diet that is restricted in natural foods and requires ingestion of a phe-free AA formula to meet protein needs is required. The goal of nutritional management for those with PKU is to maintain plasma phe concentrations that support optimal growth, development, and mental functioning while providing a nutritionally complete diet. This paper reviews developing a lifelong dietary prescription for those with PKU, outcomes of nutritional management, compliance with the low-phe diet across the life cycle, and new options for nutritional management. An individualized dietary prescription is needed to meet nutrient requirements, and the adequacy of phe intake is monitored with assessment of blood phe levels. Elevated phe concentrations may occur due to illness, excessive or inadequate phe intake, or inadequate intake of AA formula. Although normal growth and development occurs with adherence to the low-phe diet, it is important to monitor vitamin, mineral and essential fatty acid status, especially in those who do not consume sufficient AA formula. Given the growing population of adults with PKU, further research is needed to understand the risks for developing osteoporosis and cardiovascular disease. There are promising new options to liberalize the diet and improve metabolic control such as tetrahydrobiopterin therapy or supplementation with large neutral AAs. Moreover, foods made with glycomacropeptide, an intact protein that contains minimal phe, improves the PKU diet by offering a palatable alternative to AA formula. In summary, continued efforts are needed to overcome the biggest challenge to living with PKU – lifelong adherence to the low-phe diet.

© 2010 Nestec Ltd., Vevey/S. Karger AG, Basel

Article / Publication Details

First-Page Preview
Abstract of Paper

Published online: June 22, 2010
Issue release date: June 2010

Number of Print Pages: 12
Number of Figures: 2
Number of Tables: 2

ISSN: 0517-8606 (Print)
eISSN: 1661-4011 (Online)

For additional information: http://www.karger.com/ANE

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