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A randomized trial of long‐chain polyunsaturated fatty acid supplementation in infants with phenylketonuria
Author(s) -
Agostoni Carlo,
Harvie Ann,
McCulloch Daphne L,
Demellweek Colin,
Cockburn Forrester,
Giovannini Marcello,
Murray Gordon,
Harkness R Angus,
Riva Enrica
Publication year - 2006
Publication title -
developmental medicine and child neurology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.658
H-Index - 143
eISSN - 1469-8749
pISSN - 0012-1622
DOI - 10.1017/s0012162206000442
Subject(s) - polyunsaturated fatty acid , docosahexaenoic acid , arachidonic acid , long chain , randomized controlled trial , phenylalanine , medicine , infant formula , endocrinology , fatty acid , chemistry , biochemistry , pediatrics , amino acid , polymer science , enzyme
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, C 22:6 n ‐3), and arachidonic acid (AA, C 20:4 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.

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