Complications of the Low Phenylalanine Diet for Patients with Phenylketonuria and the Benefits of Increased Natural Protein

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...

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Autores principales: McWhorter, Nicole, Ndugga-Kabuye, Mesaki K., Puurunen, Marja, Ernst, Sharon L.
Formato: Online Artículo Texto
Lenguaje:English
Publicado: MDPI 2022
Materias:
Review
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9740314/
https://www.ncbi.nlm.nih.gov/pubmed/36500989
http://dx.doi.org/10.3390/nu14234960
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author McWhorter, Nicole
Ndugga-Kabuye, Mesaki K.
Puurunen, Marja
Ernst, Sharon L.
author_facet McWhorter, Nicole
Ndugga-Kabuye, Mesaki K.
Puurunen, Marja
Ernst, Sharon L.
author_sort McWhorter, Nicole
collection PubMed
description 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.
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spelling pubmed-97403142022-12-11 Complications of the Low Phenylalanine Diet for Patients with Phenylketonuria and the Benefits of Increased Natural Protein McWhorter, Nicole Ndugga-Kabuye, Mesaki K. Puurunen, Marja Ernst, Sharon L. Nutrients Review 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. MDPI 2022-11-23 /pmc/articles/PMC9740314/ /pubmed/36500989 http://dx.doi.org/10.3390/nu14234960 Text en © 2022 by the authors. https://creativecommons.org/licenses/by/4.0/Licensee MDPI, Basel, Switzerland. This article is an open access article distributed under the terms and conditions of the Creative Commons Attribution (CC BY) license (https://creativecommons.org/licenses/by/4.0/).
spellingShingle Review
McWhorter, Nicole
Ndugga-Kabuye, Mesaki K.
Puurunen, Marja
Ernst, Sharon L.
Complications of the Low Phenylalanine Diet for Patients with Phenylketonuria and the Benefits of Increased Natural Protein
title Complications of the Low Phenylalanine Diet for Patients with Phenylketonuria and the Benefits of Increased Natural Protein
title_full Complications of the Low Phenylalanine Diet for Patients with Phenylketonuria and the Benefits of Increased Natural Protein
title_fullStr Complications of the Low Phenylalanine Diet for Patients with Phenylketonuria and the Benefits of Increased Natural Protein
title_full_unstemmed Complications of the Low Phenylalanine Diet for Patients with Phenylketonuria and the Benefits of Increased Natural Protein
title_short Complications of the Low Phenylalanine Diet for Patients with Phenylketonuria and the Benefits of Increased Natural Protein
title_sort complications of the low phenylalanine diet for patients with phenylketonuria and the benefits of increased natural protein
topic Review
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9740314/
https://www.ncbi.nlm.nih.gov/pubmed/36500989
http://dx.doi.org/10.3390/nu14234960
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