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Dietary long-chain polyunsaturated fatty acid supplementation in infants with phenylketonuria: a randomized

B Koletzko1, T Sauerwald, H Demmelmair

  • 1Department of Paediatrics, University of Munich, Munich, Germany. Berthold.Koletzko@med.uni-muenchen.de

Journal of Inherited Metabolic Disease
|April 14, 2007
PubMed

Insights

Phenylketonuria (PKU) diets deplete long-chain polyunsaturated fatty acids (LCPUFA) in infants. Supplementation with LCPUFA effectively restores levels, comparable to healthy breastfed infants.

Area of Science:

  • Nutritional science
  • Pediatric nutrition
  • Biochemistry

Background:

  • Long-chain polyunsaturated fatty acids (LCPUFA) are crucial for infant development.
  • Phenylketonuria (PKU) diets are low in preformed LCPUFA.
  • Infants with PKU may have suboptimal LCPUFA status.

Purpose of the Study:

  • To assess LCPUFA status in infants with PKU.
  • To evaluate the impact of LCPUFA supplementation in PKU diets.
  • To compare LCPUFA levels in supplemented vs. unsupplemented PKU infants.

Main Methods:

  • Prospective study of infants with PKU from diagnosis.
  • Randomized double-blind trial comparing LCPUFA-supplemented vs. unsupplemented amino acid formulas.
  • Measurement of plasma phospholipid fatty acids at multiple time points during the first year.
  • Calculation of dietary LCPUFA intake from dietary records.

Main Results:

  • Unsupplemented PKU infants showed significant LCPUFA depletion.
  • Supplemented infants maintained higher and stable arachidonic acid (AA) and docosahexaenoic acid (DHA) levels.
  • Plasma LCPUFA levels correlated with dietary intake, indicating limited endogenous synthesis.

Conclusions:

  • PKU diets lead to marked depletion of AA and DHA in infants.
  • Endogenous synthesis of LCPUFA is insufficient to compensate for low dietary intake.
  • LCPUFA supplementation is effective in improving LCPUFA status in infants with PKU.
Abstract

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