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Total choline intake and working memory performance in adults with phenylketonuria

BACKGROUND: Despite early diagnosis and compliance with phenylalanine (Phe)-restricted diets, many individuals with phenylketonuria (PKU) still exhibit neurological changes and experience deficits in working memory and other executive functions. Suboptimal choline intake may contribute to these impa...

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Autores principales: Schoen, Meriah S., Boland, Kelly M., Christ, Shawn E., Cui, Xiangqin, Ramakrishnan, Usha, Ziegler, Thomas R., Alvarez, Jessica A., Singh, Rani H.
Formato: Online Artículo Texto
Lenguaje:English
Publicado: BioMed Central 2023
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10386684/
https://www.ncbi.nlm.nih.gov/pubmed/37516884
http://dx.doi.org/10.1186/s13023-023-02842-y
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author Schoen, Meriah S.
Boland, Kelly M.
Christ, Shawn E.
Cui, Xiangqin
Ramakrishnan, Usha
Ziegler, Thomas R.
Alvarez, Jessica A.
Singh, Rani H.
author_facet Schoen, Meriah S.
Boland, Kelly M.
Christ, Shawn E.
Cui, Xiangqin
Ramakrishnan, Usha
Ziegler, Thomas R.
Alvarez, Jessica A.
Singh, Rani H.
author_sort Schoen, Meriah S.
collection PubMed
description BACKGROUND: Despite early diagnosis and compliance with phenylalanine (Phe)-restricted diets, many individuals with phenylketonuria (PKU) still exhibit neurological changes and experience deficits in working memory and other executive functions. Suboptimal choline intake may contribute to these impairments, but this relationship has not been previously investigated in PKU. The objective of this study was to determine if choline intake is correlated with working memory performance, and if this relationship is modified by diagnosis and metabolic control. METHODS: This was a cross-sectional study that included 40 adults with PKU and 40 demographically matched healthy adults. Web-based neurocognitive tests were used to assess working memory performance and 3-day dietary records were collected to evaluate nutrient intake. Recent and historical blood Phe concentrations were collected as measures of metabolic control. RESULTS: Working memory performance was 0.32 z-scores (95% CI 0.06, 0.58) lower, on average, in participants with PKU compared to participants without PKU, and this difference was not modified by total choline intake (F[1,75] = 0.85, p = 0.36). However, in a subgroup with complete historical blood Phe data, increased total choline intake was related to improved working memory outcomes among participants with well controlled PKU (Phe = 360 µmol/L) after adjusting for intellectual ability and mid-childhood Phe concentrations (average change in working memory per 100 mg change in choline = 0.11; 95% CI 0.02, 0.20; p = 0.02). There also was a trend, albeit nonsignificant (p = 0.10), for this association to be attenuated with increased Phe concentrations. CONCLUSIONS: Clinical monitoring of choline intake is essential for all individuals with PKU but may have important implications for working memory functioning among patients with good metabolic control. Results from this study should be confirmed in a larger controlled trial in people living with PKU.
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spelling pubmed-103866842023-07-30 Total choline intake and working memory performance in adults with phenylketonuria Schoen, Meriah S. Boland, Kelly M. Christ, Shawn E. Cui, Xiangqin Ramakrishnan, Usha Ziegler, Thomas R. Alvarez, Jessica A. Singh, Rani H. Orphanet J Rare Dis Research BACKGROUND: Despite early diagnosis and compliance with phenylalanine (Phe)-restricted diets, many individuals with phenylketonuria (PKU) still exhibit neurological changes and experience deficits in working memory and other executive functions. Suboptimal choline intake may contribute to these impairments, but this relationship has not been previously investigated in PKU. The objective of this study was to determine if choline intake is correlated with working memory performance, and if this relationship is modified by diagnosis and metabolic control. METHODS: This was a cross-sectional study that included 40 adults with PKU and 40 demographically matched healthy adults. Web-based neurocognitive tests were used to assess working memory performance and 3-day dietary records were collected to evaluate nutrient intake. Recent and historical blood Phe concentrations were collected as measures of metabolic control. RESULTS: Working memory performance was 0.32 z-scores (95% CI 0.06, 0.58) lower, on average, in participants with PKU compared to participants without PKU, and this difference was not modified by total choline intake (F[1,75] = 0.85, p = 0.36). However, in a subgroup with complete historical blood Phe data, increased total choline intake was related to improved working memory outcomes among participants with well controlled PKU (Phe = 360 µmol/L) after adjusting for intellectual ability and mid-childhood Phe concentrations (average change in working memory per 100 mg change in choline = 0.11; 95% CI 0.02, 0.20; p = 0.02). There also was a trend, albeit nonsignificant (p = 0.10), for this association to be attenuated with increased Phe concentrations. CONCLUSIONS: Clinical monitoring of choline intake is essential for all individuals with PKU but may have important implications for working memory functioning among patients with good metabolic control. Results from this study should be confirmed in a larger controlled trial in people living with PKU. BioMed Central 2023-07-29 /pmc/articles/PMC10386684/ /pubmed/37516884 http://dx.doi.org/10.1186/s13023-023-02842-y Text en © The Author(s) 2023 https://creativecommons.org/licenses/by/4.0/Open Access This 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/ (https://creativecommons.org/licenses/by/4.0/) . The Creative Commons Public Domain Dedication waiver (http://creativecommons.org/publicdomain/zero/1.0/ (https://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
Schoen, Meriah S.
Boland, Kelly M.
Christ, Shawn E.
Cui, Xiangqin
Ramakrishnan, Usha
Ziegler, Thomas R.
Alvarez, Jessica A.
Singh, Rani H.
Total choline intake and working memory performance in adults with phenylketonuria
title Total choline intake and working memory performance in adults with phenylketonuria
title_full Total choline intake and working memory performance in adults with phenylketonuria
title_fullStr Total choline intake and working memory performance in adults with phenylketonuria
title_full_unstemmed Total choline intake and working memory performance in adults with phenylketonuria
title_short Total choline intake and working memory performance in adults with phenylketonuria
title_sort total choline intake and working memory performance in adults with phenylketonuria
topic Research
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10386684/
https://www.ncbi.nlm.nih.gov/pubmed/37516884
http://dx.doi.org/10.1186/s13023-023-02842-y
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