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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...
Autores principales: | , , , , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
MDPI
2018
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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. |
format | Online Article Text |
id | pubmed-6266274 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2018 |
publisher | MDPI |
record_format | MEDLINE/PubMed |
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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