A randomized trial of long-chain polyunsaturated fatty acid supplementation in infants with phenylketonuria

Carlo Agostoni, Ann Harvie, Daphne L McCulloch, Colin Demellweek, Forrester Cockburn, Marcello Giovannini, Gordon Murray, R Angus Harkness, Enrica Riva

Research output: Contribution to journalArticlepeer-review

Abstract

Forty-two infants (20 males, 22 females) with classical phenylketonuria (PKU) entered a prospective, double-blind, randomized study to investigate the effects on biochemical and physiological outcomes of a phenylalanine-free infant formula containing a fat blend supplemented with the long-chain polyunsaturated fatty acids (LC-PUFA), docosahexaenoic acid (DHA, C22:6 n-3), and arachidonic acid (AA, C20:4 n-6). Between entry and 20 weeks (entry and 1y) of age, median DHA levels in erythrocyte membrane phospholipids decreased by 15% (22%) in the LC-PUFA supplemented group (n=21) and by 61% (64%) in the control group (p<0.001; n=18). A dietary supply of LC-PUFA in infants with PKU prevents the decline in DHA levels associated with a diet supplying minimal sources of LC-PUFA. DHA status in turn, independent of diet, may influence the maturation of the visual system in infants with PKU.

Original languageEnglish
Pages (from-to)207-12
Number of pages6
JournalDevelopmental medicine and child neurology
Volume48
Issue number3
DOIs
Publication statusPublished - Mar 2006

Keywords

  • Developmental Disabilities
  • Double-Blind Method
  • Evoked Potentials, Visual
  • Fatty Acids, Unsaturated
  • Female
  • Humans
  • Infant
  • Male
  • Phenylalanine
  • Phenylketonurias
  • Prospective Studies

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