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The Use of Glycomacropeptide in Patients with Phenylketonuria: A Systematic Review and Meta-Analysis

In phenylketonuria (PKU), synthetic protein derived from L-amino acids (AAs) is essential in a low-phenylalanine (Phe) diet. Glycomacropeptide (GMP), an intact protein, is very low in Phe in its native form. It has been modified and adapted for PKU to provide an alternative protein source through su...

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Autores principales: Pena, Maria João, Pinto, Alex, Daly, Anne, MacDonald, Anita, Azevedo, Luís, Rocha, Júlio César, Borges, Nuno
Formato: Online Artículo Texto
Lenguaje:English
Publicado: MDPI 2018
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6266274/
https://www.ncbi.nlm.nih.gov/pubmed/30453665
http://dx.doi.org/10.3390/nu10111794
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author Pena, Maria João
Pinto, Alex
Daly, Anne
MacDonald, Anita
Azevedo, Luís
Rocha, Júlio César
Borges, Nuno
author_facet Pena, Maria João
Pinto, Alex
Daly, Anne
MacDonald, Anita
Azevedo, Luís
Rocha, Júlio César
Borges, Nuno
author_sort Pena, Maria João
collection PubMed
description In phenylketonuria (PKU), synthetic protein derived from L-amino acids (AAs) is essential in a low-phenylalanine (Phe) diet. Glycomacropeptide (GMP), an intact protein, is very low in Phe in its native form. It has been modified and adapted for PKU to provide an alternative protein source through supplementation with rate-limiting amino acids (GMP-AAs), although it still contains residual Phe. This review aims to systematically evaluate published intervention studies on the use of GMP-AAs in PKU by considering its impact on blood Phe control (primary aim) and changes in tyrosine control, nutritional biomarkers, and patient acceptability or palatability (secondary aims). Four electronic databases were searched for articles published from 2007 to June 2018. Of the 274 studies identified, only eight were included. Bias risk was assessed and a quality appraisal of the body of evidence was completed. A meta-analysis was performed with two studies with adequate comparable methodology which showed no differences between GMP-AAs and AAs for any of the interventions analysed. This work underlines the scarcity and nature of studies with GMP-AAs interventions. All were short-term with small sample sizes. There is a need for better-designed studies to provide the best evidence-based recommendations.
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spelling pubmed-62662742018-12-06 The Use of Glycomacropeptide in Patients with Phenylketonuria: A Systematic Review and Meta-Analysis Pena, Maria João Pinto, Alex Daly, Anne MacDonald, Anita Azevedo, Luís Rocha, Júlio César Borges, Nuno Nutrients Review In phenylketonuria (PKU), synthetic protein derived from L-amino acids (AAs) is essential in a low-phenylalanine (Phe) diet. Glycomacropeptide (GMP), an intact protein, is very low in Phe in its native form. It has been modified and adapted for PKU to provide an alternative protein source through supplementation with rate-limiting amino acids (GMP-AAs), although it still contains residual Phe. This review aims to systematically evaluate published intervention studies on the use of GMP-AAs in PKU by considering its impact on blood Phe control (primary aim) and changes in tyrosine control, nutritional biomarkers, and patient acceptability or palatability (secondary aims). Four electronic databases were searched for articles published from 2007 to June 2018. Of the 274 studies identified, only eight were included. Bias risk was assessed and a quality appraisal of the body of evidence was completed. A meta-analysis was performed with two studies with adequate comparable methodology which showed no differences between GMP-AAs and AAs for any of the interventions analysed. This work underlines the scarcity and nature of studies with GMP-AAs interventions. All were short-term with small sample sizes. There is a need for better-designed studies to provide the best evidence-based recommendations. MDPI 2018-11-18 /pmc/articles/PMC6266274/ /pubmed/30453665 http://dx.doi.org/10.3390/nu10111794 Text en © 2018 by the authors. 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 (http://creativecommons.org/licenses/by/4.0/).
spellingShingle Review
Pena, Maria João
Pinto, Alex
Daly, Anne
MacDonald, Anita
Azevedo, Luís
Rocha, Júlio César
Borges, Nuno
The Use of Glycomacropeptide in Patients with Phenylketonuria: A Systematic Review and Meta-Analysis
title The Use of Glycomacropeptide in Patients with Phenylketonuria: A Systematic Review and Meta-Analysis
title_full The Use of Glycomacropeptide in Patients with Phenylketonuria: A Systematic Review and Meta-Analysis
title_fullStr The Use of Glycomacropeptide in Patients with Phenylketonuria: A Systematic Review and Meta-Analysis
title_full_unstemmed The Use of Glycomacropeptide in Patients with Phenylketonuria: A Systematic Review and Meta-Analysis
title_short The Use of Glycomacropeptide in Patients with Phenylketonuria: A Systematic Review and Meta-Analysis
title_sort use of glycomacropeptide in patients with phenylketonuria: a systematic review and meta-analysis
topic Review
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6266274/
https://www.ncbi.nlm.nih.gov/pubmed/30453665
http://dx.doi.org/10.3390/nu10111794
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