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Long term effects of long chain polyunsaturated fats in hyperphenylalaninemic children
C Agostoni1, E Verduci, N Massetto
1Department of Pediatrics, San Paolo Hospital, University of Milan, Milan, Italy. agostoc@tin.it
Insights
Supplementing children with hyperphenylalaninemia (HPA) with long-chain polyunsaturated fatty acids (LCPUFA) temporarily improved blood docosahexaenoic acid (DHA) levels and visual function. However, these benefits did not persist three years after treatment cessation.
Area of Science:
- Biochemistry
- Neuroscience
- Pediatrics
Background:
- Hyperphenylalaninemia (HPA) is a metabolic disorder affecting phenylalanine metabolism.
- Visual function deficits are observed in some children with HPA.
- Long-chain polyunsaturated fatty acids (LCPUFA), such as docosahexaenoic acid (DHA), are crucial for visual development.
Purpose of the Study:
- To investigate the long-term effects of LCPUFA supplementation on blood fatty acid status and visual function in children with HPA.
- To determine if improvements observed during LCPUFA treatment are sustained after cessation.
Main Methods:
- A randomized controlled trial involving 20 children with HPA.
- Participants were supplemented with either LCPUFA (including DHA) or placebo for 12 months.
- Blood fatty acid levels and visual evoked potentials (P100 wave latency) were assessed.
- Follow-up assessments were conducted three years after the treatment period.
Main Results:
- At the end of supplementation, the LCPUFA group showed improved blood DHA levels and reduced P100 wave latency compared to placebo.
- Three years after treatment cessation, blood DHA levels in the LCPUFA group returned to baseline.
- Similarly, P100 wave latency in the LCPUFA group also reverted to baseline levels.
Conclusions:
- Short-term LCPUFA supplementation can transiently improve biochemical markers and visual function in children with HPA.
- The observed benefits on blood DHA levels and visual function are not sustained long-term after discontinuation of supplementation.
- Further research is needed to explore strategies for maintaining long-term benefits in HPA management.
Abstract:
Blood fatty acid status and visual function of 20 treated hyperphenylalaninemic (HPA) children, randomly allocated into two groups to receive supplementation of either long chain polyunsaturated fatty acids (LCPUFA), including docosahexaenoic acid (DHA), or placebo for 12 months, have been investigated three years after the end of the treatment. Although in the LCPUFA group blood DHA levels and P100 wave latency improved at the end of supplementation, they had returned to baseline after three years.