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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.

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