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Determinants of Plasma Docosahexaenoic Acid Levels and Their Relationship to Neurological and Cognitive Functions in PKU Patients: A Double Blind Randomized Supplementation Study

Determinants of Plasma Docosahexaenoic Acid Levels and Their Relationship to Neurological and Cognitive Functions in PKU Patients: A Double Blind Randomized Supplementation Study

Authors :
S. Paci
Melike Ersoy
Hans Demmelmair
Behiye Alyanak
Eva Reischl
Gülden Gökçay
Wolfgang Müller-Felber
Elvira Verduci
Fabienne L Faber
Berthold Koletzko
Uschi Handel
Anita MacDonald
Urania Kotzaeridou
Peter Burgard
Domingo González-Lamuño
Universidad de Cantabria
Source :
Nutrients. 2018 Dec 7;10(12). pii: E1944, Nutrients 10:1944 (2018), Nutrients, Nutrients, Vol 10, Iss 12, p 1944 (2018), UCrea Repositorio Abierto de la Universidad de Cantabria, Universidad de Cantabria (UC), Volume 10, Issue 12
Publication Year :
2018
Publisher :
MDPI AG, 2018.

Abstract

Children with phenylketonuria (PKU) follow a protein restricted diet with negligible amounts of docosahexaenoic acid (DHA). Low DHA intakes might explain subtle neurological deficits in PKU. We studied whether a DHA supply modified plasma DHA and neurological and intellectual functioning in PKU. In a double-blind multicentric trial, 109 PKU patients were randomized to DHA doses from 0 to 7 mg/kg&amp<br />day for six months. Before and after supplementation, we determined plasma fatty acid concentrations, latencies of visually evoked potentials, fine and gross motor behavior, and IQ. Fatty acid desaturase genotypes were also determined. DHA supplementation increased plasma glycerophospholipid DHA proportional to dose by 0.4% DHA per 1 mg intake/kg bodyweight. Functional outcomes were not associated with DHA status before and after intervention and remained unchanged by supplementation. Genotypes were associated with plasma arachidonic acid levels and, if considered together with the levels of the precursor alpha-linolenic acid, also with DHA. Functional outcomes and supplementation effects were not significantly associated with genotype. DHA intakes up to 7 mg/kg did not improve neurological functions in PKU children. Nervous tissues may be less prone to low DHA levels after infancy, or higher doses might be required to impact neurological functions. In situations of minimal dietary DHA, endogenous synthesis of DHA from alpha-linolenic acid could relevantly contribute to DHA status.

Details

ISSN :
20726643
Volume :
10
Database :
OpenAIRE
Journal :
Nutrients
Accession number :
edsair.doi.dedup.....0b0e92c1a16e3d624780d845272215c5
Full Text :
https://doi.org/10.3390/nu10121944