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

• Published in: Developmental medicine and child neurology Vol. 48; no. 3; pp. 207 - 212
• Main Authors: Agostoni, Carlo, Harvie, Ann, McCulloch, Daphne L, Demellweek, Colin, Cockburn, Forrester, Giovannini, Marcello, Murray, Gordon, Harkness, R Angus, Riva, Enrica
• Format: Journal Article
• Language: English
• Published: Cambridge, UK Cambridge University Press 01.03.2006; Blackwell Publishing Ltd; Mac Keith Press
• Subjects: Age Differences; Anatomy; Computation; Control Groups; Developmental Disabilities - diagnosis; Developmental Disabilities - epidemiology; Double-Blind Method; Evoked Potentials, Visual - physiology; Fatty Acids, Unsaturated - therapeutic use; Female; Humans; Infant; Male; Original Articles; Outcome Measures; Phenylalanine - blood; Phenylketonurias - blood; Phenylketonurias - drug therapy; Prospective Studies; Sample Size; Statistical Analysis; Young Children
• ISSN: 0012-1622; 1469-8749
• DOI: 10.1017/S0012162206000442

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, C22:6n–3), and arachidonic acid (AA, C20:4n–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.