Next Article in Journal
Genetic Permissiveness and Dietary Glycemic Load Interact to Predict Type-II Diabetes in the Nile rat (Arvicanthis niloticus)
Previous Article in Journal
Modulation of Milk Allergenicity by Baking Milk in Foods: A Proteomic Investigation
Previous Article in Special Issue
Over Restriction of Dietary Protein Allowance: The Importance of Ongoing Reassessment of Natural Protein Tolerance in Phenylketonuria
Article Menu

Export Article

Open AccessReview

Effects of LC-PUFA Supplementation in Patients with Phenylketonuria: A Systematic Review of Controlled Trials

1
Department of Pediatrics, University Clinical Hospital of Santiago de Compostela, 15706 Santiago de Compostela, Spain
2
IDIS-Health Research Institute of Santiago de Compostela, 15706 Santiago de Compostela, Spain
3
CIBERER, Pabellón 11, 28029 Madrid, Spain
4
Universidade de Santiago de Compostela, 15704 Santiago de Compostela, Spain
5
Department of Pediatrics, Pediatric Metabolism and Research Unit, Reina Sofia University Hospital, IMIBIC, 14004 Cordoba, Spain
*
Author to whom correspondence should be addressed.
Nutrients 2019, 11(7), 1537; https://doi.org/10.3390/nu11071537
Received: 4 June 2019 / Revised: 1 July 2019 / Accepted: 2 July 2019 / Published: 6 July 2019
  |  
PDF [516 KB, uploaded 10 July 2019]
  |  

Abstract

Evidence suggests a role of long chain polyunsaturated fatty acids (LC-PUFA), in which animal foods are especially rich, in optimal neural development. The LC-PUFAs docosahexaenoic acid (DHA) and arachidonic acid, found in high concentrations in the brain and retina, have potential beneficial effects on cognition, and motor and visual functions. Phenylketonuria (PKU) is the most common inborn error of amino acid metabolism. The treatment of PKU consists of a phenylalanine-free diet, which limits the intake of natural proteins of high biological value. In this systematic review, we summarize the available evidence supporting a role for LC-PUFA supplementation as an effective means of increasing LC-PUFA levels and improving visual and neurocognitive functions in PKU patients. Data from controlled trials of children and adults (up to 47 years of age) were obtained by searching the MEDLINE and SCOPUS databases following Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines. For each selected study, the risk of bias was assessed applying the methodology of the Cochrane Collaboration. The findings indicate that DHA supplementation in PKU patients from 2 weeks to 47 years of age improves DHA status and decreases visual evoked potential P100 wave latency in PKU children from 1 to 11 years old. Neurocognitive data are inconclusive. View Full-Text
Keywords: arachidonic acid; cognitive function; docosahexaenoic acid; long-chain polyunsaturated fatty acids; phenylketonuria; visual function arachidonic acid; cognitive function; docosahexaenoic acid; long-chain polyunsaturated fatty acids; phenylketonuria; visual function
Figures

Figure 1

This is an open access article distributed under the Creative Commons Attribution License which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited (CC BY 4.0).

Supplementary material

SciFeed

Share & Cite This Article

MDPI and ACS Style

Couce, M.L.; de Castro, M.J.; de Lamas, C.; Leis, R. Effects of LC-PUFA Supplementation in Patients with Phenylketonuria: A Systematic Review of Controlled Trials. Nutrients 2019, 11, 1537.

Show more citation formats Show less citations formats

Note that from the first issue of 2016, MDPI journals use article numbers instead of page numbers. See further details here.

Related Articles

Article Metrics

Article Access Statistics

1

Comments

[Return to top]
Nutrients EISSN 2072-6643 Published by MDPI AG, Basel, Switzerland RSS E-Mail Table of Contents Alert
Back to Top