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NORMAL FATTY ACID CONCENTRATIONS IN YOUNG CHILDREN WITH PHENYLKETONURIA (PKU)

Stacey M Lavoie1, Cary O Harding, Melanie B Gillingham

  • 1Department of Pediatrics, Oregon Health & Science University, Portland, Oregon 97239, USA.

Topics in Clinical Nutrition
|December 17, 2009
PubMed

Insights

Children with phenylketonuria (PKU) on a restricted diet show lower erythrocyte fatty acids. However, these levels remain adequate, indicating no essential fatty acid deficiency despite dietary changes.

Area of Science:

  • Biochemistry
  • Pediatrics
  • Nutrition Science

Background:

  • Phenylketonuria (PKU) requires a phenylalanine-restricted diet.
  • Dietary interventions may impact lipid metabolism and fatty acid profiles.

Purpose of the Study:

  • To investigate erythrocyte fatty acid concentrations in children with PKU on a phenylalanine-restricted diet.
  • To compare these concentrations with those in healthy control children.

Main Methods:

  • Measured dietary intake and erythrocyte lipid fatty acid concentrations.
  • Included twenty-one children with PKU (

Main Results:

  • PKU subjects had lower protein and higher polyunsaturated fat intake.
  • PKU subjects exhibited lower concentrations of omega-3, omega-6, saturated, and polyunsaturated fatty acids in erythrocyte lipids.
  • No PKU subjects showed signs of essential fatty acid deficiency.

Conclusions:

  • Despite lower concentrations, erythrocyte fatty acid levels in children with PKU on this diet are adequate.
  • The phenylalanine-restricted diet therapy for PKU does not appear to cause essential fatty acid deficiency in this cohort.

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