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Continuous use of glycomacropeptide in the nutritional management of patients with phenylketonuria: a clinical perspective

BACKGROUND: In phenylketonuria (PKU), modified casein glycomacropeptide supplements (CGMP-AA) are used as an alternative to the traditional phenylalanine (Phe)-free L-amino acid supplements (L-AA). However, studies focusing on the long-term nutritional status of CGMP-AA are lacking. This retrospecti...

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Autores principales: Pena, Maria João, Pinto, Alex, de Almeida, Manuela Ferreira, de Sousa Barbosa, Catarina, Ramos, Paula Cristina, Rocha, Sara, Guimas, Arlindo, Ribeiro, Rosa, Martins, Esmeralda, Bandeira, Anabela, Dias, Cláudia Camila, MacDonald, Anita, Borges, Nuno, Rocha, Júlio César
Formato: Online Artículo Texto
Lenguaje:English
Publicado: BioMed Central 2021
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7881530/
https://www.ncbi.nlm.nih.gov/pubmed/33581730
http://dx.doi.org/10.1186/s13023-021-01721-8
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author Pena, Maria João
Pinto, Alex
de Almeida, Manuela Ferreira
de Sousa Barbosa, Catarina
Ramos, Paula Cristina
Rocha, Sara
Guimas, Arlindo
Ribeiro, Rosa
Martins, Esmeralda
Bandeira, Anabela
Dias, Cláudia Camila
MacDonald, Anita
Borges, Nuno
Rocha, Júlio César
author_facet Pena, Maria João
Pinto, Alex
de Almeida, Manuela Ferreira
de Sousa Barbosa, Catarina
Ramos, Paula Cristina
Rocha, Sara
Guimas, Arlindo
Ribeiro, Rosa
Martins, Esmeralda
Bandeira, Anabela
Dias, Cláudia Camila
MacDonald, Anita
Borges, Nuno
Rocha, Júlio César
author_sort Pena, Maria João
collection PubMed
description BACKGROUND: In phenylketonuria (PKU), modified casein glycomacropeptide supplements (CGMP-AA) are used as an alternative to the traditional phenylalanine (Phe)-free L-amino acid supplements (L-AA). However, studies focusing on the long-term nutritional status of CGMP-AA are lacking. This retrospective study evaluated the long-term impact of CGMP-AA over a mean of 29 months in 11 patients with a mean age at CGMP-AA onset of 28 years (range 15–43) [8 females; 2 hyperphenylalaninaemia (HPA), 3 mild PKU, 3 classical PKU and 3 late-diagnosed]. Outcome measures included metabolic control, anthropometry, body composition and biochemical parameters. RESULTS: CGMP-AA, providing 66% of protein equivalent intake from protein substitute, was associated with no significant change in blood Phe with CGMP-AA compared with baseline (562 ± 289 µmol/L vs 628 ± 317 µmol/L; p = 0.065). In contrast, blood tyrosine significantly increased on CGMP-AA (52.0 ± 19.2 μmol/L vs 61.4 ± 23.8 μmol/L; p = 0.027). CONCLUSIONS: Biochemical nutritional markers remained unchanged which is an encouraging finding in adults with PKU, many of whom are unable to maintain full adherence with nutritionally fortified protein substitutes. Longitudinal, prospective studies with larger sample sizes are necessary to fully understand the metabolic impact of using CGMP-AA in PKU.
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spelling pubmed-78815302021-02-17 Continuous use of glycomacropeptide in the nutritional management of patients with phenylketonuria: a clinical perspective Pena, Maria João Pinto, Alex de Almeida, Manuela Ferreira de Sousa Barbosa, Catarina Ramos, Paula Cristina Rocha, Sara Guimas, Arlindo Ribeiro, Rosa Martins, Esmeralda Bandeira, Anabela Dias, Cláudia Camila MacDonald, Anita Borges, Nuno Rocha, Júlio César Orphanet J Rare Dis Research BACKGROUND: In phenylketonuria (PKU), modified casein glycomacropeptide supplements (CGMP-AA) are used as an alternative to the traditional phenylalanine (Phe)-free L-amino acid supplements (L-AA). However, studies focusing on the long-term nutritional status of CGMP-AA are lacking. This retrospective study evaluated the long-term impact of CGMP-AA over a mean of 29 months in 11 patients with a mean age at CGMP-AA onset of 28 years (range 15–43) [8 females; 2 hyperphenylalaninaemia (HPA), 3 mild PKU, 3 classical PKU and 3 late-diagnosed]. Outcome measures included metabolic control, anthropometry, body composition and biochemical parameters. RESULTS: CGMP-AA, providing 66% of protein equivalent intake from protein substitute, was associated with no significant change in blood Phe with CGMP-AA compared with baseline (562 ± 289 µmol/L vs 628 ± 317 µmol/L; p = 0.065). In contrast, blood tyrosine significantly increased on CGMP-AA (52.0 ± 19.2 μmol/L vs 61.4 ± 23.8 μmol/L; p = 0.027). CONCLUSIONS: Biochemical nutritional markers remained unchanged which is an encouraging finding in adults with PKU, many of whom are unable to maintain full adherence with nutritionally fortified protein substitutes. Longitudinal, prospective studies with larger sample sizes are necessary to fully understand the metabolic impact of using CGMP-AA in PKU. BioMed Central 2021-02-13 /pmc/articles/PMC7881530/ /pubmed/33581730 http://dx.doi.org/10.1186/s13023-021-01721-8 Text en © The Author(s) 2021 Open AccessThis article is licensed under a Creative Commons Attribution 4.0 International License, which permits use, sharing, adaptation, distribution and reproduction in any medium or format, as long as you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons licence, and indicate if changes were made. The images or other third party material in this article are included in the article's Creative Commons licence, unless indicated otherwise in a credit line to the material. If material is not included in the article's Creative Commons licence and your intended use is not permitted by statutory regulation or exceeds the permitted use, you will need to obtain permission directly from the copyright holder. To view a copy of this licence, visit http://creativecommons.org/licenses/by/4.0/. The Creative Commons Public Domain Dedication waiver (http://creativecommons.org/publicdomain/zero/1.0/) applies to the data made available in this article, unless otherwise stated in a credit line to the data.
spellingShingle Research
Pena, Maria João
Pinto, Alex
de Almeida, Manuela Ferreira
de Sousa Barbosa, Catarina
Ramos, Paula Cristina
Rocha, Sara
Guimas, Arlindo
Ribeiro, Rosa
Martins, Esmeralda
Bandeira, Anabela
Dias, Cláudia Camila
MacDonald, Anita
Borges, Nuno
Rocha, Júlio César
Continuous use of glycomacropeptide in the nutritional management of patients with phenylketonuria: a clinical perspective
title Continuous use of glycomacropeptide in the nutritional management of patients with phenylketonuria: a clinical perspective
title_full Continuous use of glycomacropeptide in the nutritional management of patients with phenylketonuria: a clinical perspective
title_fullStr Continuous use of glycomacropeptide in the nutritional management of patients with phenylketonuria: a clinical perspective
title_full_unstemmed Continuous use of glycomacropeptide in the nutritional management of patients with phenylketonuria: a clinical perspective
title_short Continuous use of glycomacropeptide in the nutritional management of patients with phenylketonuria: a clinical perspective
title_sort continuous use of glycomacropeptide in the nutritional management of patients with phenylketonuria: a clinical perspective
topic Research
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7881530/
https://www.ncbi.nlm.nih.gov/pubmed/33581730
http://dx.doi.org/10.1186/s13023-021-01721-8
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