Cargando…
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...
Autores principales: | , , , , , , , |
---|---|
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 |
_version_ | 1785081728396689408 |
---|---|
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. |
format | Online Article Text |
id | pubmed-10386684 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2023 |
publisher | BioMed Central |
record_format | MEDLINE/PubMed |
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 |
work_keys_str_mv | AT schoenmeriahs totalcholineintakeandworkingmemoryperformanceinadultswithphenylketonuria AT bolandkellym totalcholineintakeandworkingmemoryperformanceinadultswithphenylketonuria AT christshawne totalcholineintakeandworkingmemoryperformanceinadultswithphenylketonuria AT cuixiangqin totalcholineintakeandworkingmemoryperformanceinadultswithphenylketonuria AT ramakrishnanusha totalcholineintakeandworkingmemoryperformanceinadultswithphenylketonuria AT zieglerthomasr totalcholineintakeandworkingmemoryperformanceinadultswithphenylketonuria AT alvarezjessicaa totalcholineintakeandworkingmemoryperformanceinadultswithphenylketonuria AT singhranih totalcholineintakeandworkingmemoryperformanceinadultswithphenylketonuria |