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Fat intakes of children with PKU on low phenylalanine diets

H J Rose1, F White, A Macdonald

  • 1SHS International Ltd, Liverpool, UK. hrose@shsint.co.uk

Journal of Human Nutrition and Dietetics : the Official Journal of the British Dietetic Association
|September 10, 2005
PubMed

Insights

Children with phenylketonuria (PKU) often have inadequate fat and essential fatty acid (EFA) intake. Supplementing their diet improved EFA intake, especially for young children, highlighting the need for careful dietary fat consideration.

Area of Science:

  • Nutritional Science
  • Pediatric Nutrition
  • Metabolic Disorders

Background:

  • Phenylketonuria (PKU) management requires a phenylalanine-free diet.
  • Assessing dietary fat and essential fatty acid (EFA) adequacy is crucial for children with PKU.
  • Fat-supplemented protein substitutes may impact fatty acid status.

Purpose of the Study:

  • To evaluate the adequacy of fat and EFA intake in children with PKU.
  • To assess the effect of a fat-supplemented phenylalanine-free protein substitute on dietary fat and EFA intake.
  • To compare outcomes between a test group receiving EFA-supplemented substitute and a control group.

Main Methods:

  • A 20-week study involving children aged 1-10 years with PKU.
  • Randomization of subjects into a test group (EFA-supplemented substitute) and a control group (fat-free substitute).
  • Collection of 3-day semi-weighed food intake records at the study's conclusion.

Main Results:

  • The control group exhibited poor total fat and alpha-linolenic acid (alpha-LA) intake.
  • The test group showed significantly higher fat and EFA intakes, closer to population norms.
  • Younger children (<5 years) in the control group were particularly vulnerable to poor fat intake due to limited diet diversity.

Conclusions:

  • Dietary fat quantity and quality require careful consideration for children with PKU.
  • Optimizing the linoleic acid to alpha-LA ratio is important for children with PKU.
  • Ensuring adequate fat and alpha-LA intake is essential for the nutritional well-being of children with PKU.
Abstract

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