Journal
JOURNAL OF PEDIATRICS
Volume 150, Issue 5, Pages 479-484Publisher
MOSBY-ELSEVIER
DOI: 10.1016/j.jpeds.2006.12.011
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Objective To investigate effects of long-chain omega-3 polyunsaturated fatty acids (LC-PUFA) on motor skills in patients with phenylketonuria (PKU). Study design Thirty-six patients with PKU (1-11 years of age, good metabolic control: plasma phenylalanine <= 360 mu mol/L for >= 6 months). We determined plasma phospholipid fatty acids, and in patients > 4 years of age (N = 24) the motometric Rostock-Oseretzky Scale (ROS), before and after supplementation with fish oil for 3 months (15 mg docosahexaenoic acid [DHA]/kg body weight daily). ROS was also assessd in 22 age-matched controls. Results Patients had low n-3 LC-PUFA in plasma phospholipids (DHA, 2.37 +/- 0.10%; eicosapentaenoic acid [EPA], 0.4 +/- 0.03%) and poorer ROS performance than controls (motor development index [MQ] 107 +/- 3 vs 117 +/- 3, P = .010). Supplementation increased phospholipid n-3 LC-PUFA (DHA 7.05 +/- 0.24%; EPA 3.31 +/- 0.19%; P < .001), decreased n-6 LC-PUFA (arachidonic acid, 9.26 +/- 0.23% vs 6.76 +/- 0.16%; P < .001) and improved RIDS (MQ 115 +/- 3.54, P = .011, paired t test). ROS was unchanged in 11 retested controls (MQ 115 +/- 5.16, P = NS, paired it test multivariate analysis of variance [MANOVA] for time by group, P = .027). Patients tolerated fish oil well. Plasma phenylalanine remained unchanged. Conclusion in patients with PKU, fish oil supplementation enhances n-3 LC-PUFA levels and improves motor skills.
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