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Published on: November 29, 2013
Effects of long-chain polyunsaturated fatty acid supplementation on fatty acid status and visual function in treated
C Agostoni1, N Massetto, G Biasucci
1Department of Pediatrics, San Paolo Hospital, University of Milan, Milan, Italy.
Insights
Children with phenylketonuria (PKU) have low docosahexaenoic acid (DHA) levels. Supplementing with long-chain polyunsaturated fatty acids (LCPUFA) improved DHA status and visual function in these children.
Area of Science:
- Nutritional Science
- Pediatric Medicine
- Biochemistry
Background:
- Children with phenylketonuria (PKU) require a low-phenylalanine diet, limiting intake of essential long-chain polyunsaturated fatty acids (LCPUFA).
- This dietary restriction leads to suboptimal levels of docosahexaenoic acid (DHA), crucial for visual and neural development.
- PKU patients often exhibit poorer DHA status compared to healthy peers.
Purpose of the Study:
- To evaluate the impact of 12-month LCPUFA supplementation on DHA status and visual function in children with PKU.
- To conduct a double-blind, placebo-controlled trial to assess the efficacy of LCPUFA supplementation.
Main Methods:
- Twenty children with well-controlled PKU were randomized to receive either an LCPUFA-rich fat supplement or a placebo for 12 months.
- Erythrocyte lipid fatty acid composition and visual evoked potentials (VEPs) were measured at baseline and after supplementation.
- Reference data from age-matched healthy children were used for comparison.
Main Results:
- Baseline assessment revealed lower DHA levels and prolonged P100 wave latencies in children with PKU compared to controls.
- The LCPUFA supplementation group demonstrated a significant increase in erythrocyte DHA levels.
- A decrease in P100 wave latency was observed in the LCPUFA group, correlating negatively with DHA level changes.
Conclusions:
- Dietary supplementation with LCPUFA in children with PKU effectively enhances DHA status.
- LCPUFA supplementation is associated with improved visual function in children with phenylketonuria.
- This intervention offers a strategy to mitigate nutritional deficiencies and support neurodevelopment in PKU patients.
Background:
Children with phenylalanine-hydroxylase deficiency (type-I hyperphenylalaninemia, HPA) follow a low-phenylalanine diet, severely restricted in animal foods and long-chain polyunsaturated fatty acids (LCPUFA). Consequently, they have a poor LCPUFA status, particularly for docosahexaenoic acid (DHA). DHA is relevant to visual and neural development.
Objective:
To investigate the effects of a 12-month supplementation with LCPUFA in a double-blind, placebo-controlled trial in treated children with HPA.
Study Design:
Twenty children with well-controlled HPA were randomly allocated to receive either a fat supplement (supplying 26% as fatty acids including DHA, 8%) or a placebo. The fatty acid composition of erythrocyte lipids and the visual evoked potentials were measured at baseline and after 12 months of supplementation. Reference data were obtained from healthy children of comparable age.
Results:
At baseline children with HPA had a poorer DHA status and prolonged P100 wave latencies than the reference group. At the end of the trial the LCPUFA group showed a significant increase in DHA levels of erythrocyte lipids. In the LCPUFA group P100 wave latency decreased and was negatively associated with the DHA changes.
Conclusions:
A balanced dietary supplementation with LCPUFA in children with HPA is associated with an increase of the DHA pool and improved visual function.
