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Improved Eating Behaviour and Nutrient Intake in Noncompliant Patients with Phenylketonuria after Reintroducing a Protein Substitute: Observations from a Multicentre Study

Noncompliance is widespread in adults with PKU and is associated with adverse metabolic, nutritional and cognitive abnormalities. Returning to the PKU diet is important for this at-risk population, yet for many this is challenging to achieve. Strategies that ease the return to the PKU diet, while of...

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Autores principales: Green, Benjamin, Rahman, Yusof, Firman, Sarah, Adam, Sarah, Jenkinson, Fiona, Nicol, Claire, Adams, Sandra, Dawson, Charlotte, Robertson, Louise, Dunlop, Carolyn, Cozens, Alison, Hubbard, Gary, Stratton, Rebecca
Formato: Online Artículo Texto
Lenguaje:English
Publicado: MDPI 2019
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6770397/
https://www.ncbi.nlm.nih.gov/pubmed/31480383
http://dx.doi.org/10.3390/nu11092035
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author Green, Benjamin
Rahman, Yusof
Firman, Sarah
Adam, Sarah
Jenkinson, Fiona
Nicol, Claire
Adams, Sandra
Dawson, Charlotte
Robertson, Louise
Dunlop, Carolyn
Cozens, Alison
Hubbard, Gary
Stratton, Rebecca
author_facet Green, Benjamin
Rahman, Yusof
Firman, Sarah
Adam, Sarah
Jenkinson, Fiona
Nicol, Claire
Adams, Sandra
Dawson, Charlotte
Robertson, Louise
Dunlop, Carolyn
Cozens, Alison
Hubbard, Gary
Stratton, Rebecca
author_sort Green, Benjamin
collection PubMed
description Noncompliance is widespread in adults with PKU and is associated with adverse metabolic, nutritional and cognitive abnormalities. Returning to the PKU diet is important for this at-risk population, yet for many this is challenging to achieve. Strategies that ease the return to the PKU diet, while offering nutritional and cognitive advantages, are needed. Twelve PKU adults (33.7 ± 2.6 years), who had been noncompliant for 4.5 years (range: 1 to 11 years), took 33 g of a low-volume, nutrient-enriched, protein substitute daily for 28 days. Outcomes of eating behaviour, nutrient intake and mood were assessed at entry (baseline, days 1–3) and after the intervention period (days 29–31). At baseline, intakes of natural protein and estimated phenylalanine were high (66.4 g and 3318.5 mg, respectively) and intakes of calcium, magnesium, iron, zinc, iodine and vitamin D were below country-specific recommendations. With use of the experimental protein substitute, natural protein and estimated phenylalanine intake declined (p = 0.043 for both). Fat and saturated fat intakes also decreased (p = 0.019 and p = 0.041, respectively), while energy and carbohydrate intake remained unchanged. Micronutrient intake increased (p ≤ 0.05 for all aforementioned) to levels well within reference nutrient intake recommendations. Blood vitamin B(12) and vitamin D increased by 19.8% and 10.4%, respectively. Reductions in anxiety and confusion were also observed during the course of the study yet should be handled as preliminary data. This study demonstrates that reintroducing a low-volume, nutrient-enriched protein substitute delivers favourable nutritional and possible mood benefits in noncompliant PKU patients, yet longer-term studies are needed to further confirm this. This preliminary knowledge should be used in the design of new strategies to better facilitate patients’ return to the PKU diet, with the approach described here as a foundation.
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spelling pubmed-67703972019-10-30 Improved Eating Behaviour and Nutrient Intake in Noncompliant Patients with Phenylketonuria after Reintroducing a Protein Substitute: Observations from a Multicentre Study Green, Benjamin Rahman, Yusof Firman, Sarah Adam, Sarah Jenkinson, Fiona Nicol, Claire Adams, Sandra Dawson, Charlotte Robertson, Louise Dunlop, Carolyn Cozens, Alison Hubbard, Gary Stratton, Rebecca Nutrients Article Noncompliance is widespread in adults with PKU and is associated with adverse metabolic, nutritional and cognitive abnormalities. Returning to the PKU diet is important for this at-risk population, yet for many this is challenging to achieve. Strategies that ease the return to the PKU diet, while offering nutritional and cognitive advantages, are needed. Twelve PKU adults (33.7 ± 2.6 years), who had been noncompliant for 4.5 years (range: 1 to 11 years), took 33 g of a low-volume, nutrient-enriched, protein substitute daily for 28 days. Outcomes of eating behaviour, nutrient intake and mood were assessed at entry (baseline, days 1–3) and after the intervention period (days 29–31). At baseline, intakes of natural protein and estimated phenylalanine were high (66.4 g and 3318.5 mg, respectively) and intakes of calcium, magnesium, iron, zinc, iodine and vitamin D were below country-specific recommendations. With use of the experimental protein substitute, natural protein and estimated phenylalanine intake declined (p = 0.043 for both). Fat and saturated fat intakes also decreased (p = 0.019 and p = 0.041, respectively), while energy and carbohydrate intake remained unchanged. Micronutrient intake increased (p ≤ 0.05 for all aforementioned) to levels well within reference nutrient intake recommendations. Blood vitamin B(12) and vitamin D increased by 19.8% and 10.4%, respectively. Reductions in anxiety and confusion were also observed during the course of the study yet should be handled as preliminary data. This study demonstrates that reintroducing a low-volume, nutrient-enriched protein substitute delivers favourable nutritional and possible mood benefits in noncompliant PKU patients, yet longer-term studies are needed to further confirm this. This preliminary knowledge should be used in the design of new strategies to better facilitate patients’ return to the PKU diet, with the approach described here as a foundation. MDPI 2019-08-30 /pmc/articles/PMC6770397/ /pubmed/31480383 http://dx.doi.org/10.3390/nu11092035 Text en © 2019 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 Article
Green, Benjamin
Rahman, Yusof
Firman, Sarah
Adam, Sarah
Jenkinson, Fiona
Nicol, Claire
Adams, Sandra
Dawson, Charlotte
Robertson, Louise
Dunlop, Carolyn
Cozens, Alison
Hubbard, Gary
Stratton, Rebecca
Improved Eating Behaviour and Nutrient Intake in Noncompliant Patients with Phenylketonuria after Reintroducing a Protein Substitute: Observations from a Multicentre Study
title Improved Eating Behaviour and Nutrient Intake in Noncompliant Patients with Phenylketonuria after Reintroducing a Protein Substitute: Observations from a Multicentre Study
title_full Improved Eating Behaviour and Nutrient Intake in Noncompliant Patients with Phenylketonuria after Reintroducing a Protein Substitute: Observations from a Multicentre Study
title_fullStr Improved Eating Behaviour and Nutrient Intake in Noncompliant Patients with Phenylketonuria after Reintroducing a Protein Substitute: Observations from a Multicentre Study
title_full_unstemmed Improved Eating Behaviour and Nutrient Intake in Noncompliant Patients with Phenylketonuria after Reintroducing a Protein Substitute: Observations from a Multicentre Study
title_short Improved Eating Behaviour and Nutrient Intake in Noncompliant Patients with Phenylketonuria after Reintroducing a Protein Substitute: Observations from a Multicentre Study
title_sort improved eating behaviour and nutrient intake in noncompliant patients with phenylketonuria after reintroducing a protein substitute: observations from a multicentre study
topic Article
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6770397/
https://www.ncbi.nlm.nih.gov/pubmed/31480383
http://dx.doi.org/10.3390/nu11092035
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