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

Published online by Cambridge University Press:  17 February 2006

Carlo Agostoni ,
Ann Harvie ,
Daphne L McCulloch ,
Colin Demellweek ,
Forrester Cockburn ,
Marcello Giovannini ,
Gordon Murray ,
R Angus Harkness  and
Enrica Riva
Carlo Agostoni
Affiliation:
Department of Pediatrics, University of Milan Medical School, Milan, Italy.
Ann Harvie
Affiliation:
Department of Child Health, Royal Hospital for Sick Children, Glasgow, UK.
Daphne L McCulloch
Affiliation:
Department of Vision Sciences, Glasgow Caledonian University, Glasgow, UK.
Colin Demellweek
Affiliation:
Roald Dahl EEG unit, Alder Hey Children's Hospital, Liverpool, UK.
Forrester Cockburn
Affiliation:
Department of Child Health, Royal Hospital for Sick Children, Glasgow, UK.
Marcello Giovannini
Affiliation:
Department of Pediatrics, University of Milan Medical School, Milan, Italy.
Gordon Murray
Affiliation:
Public Health Sciences, UK.
R Angus Harkness
Affiliation:
Department of Child Life and Health, University of Edinburgh, Edinburgh, UK.
Enrica Riva
Affiliation:
Department of Pediatrics, University of Milan Medical School, Milan, Italy.
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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, C22:6n–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.

Type
Original Articles
Information
Developmental Medicine and Child Neurology , Volume 48 , Issue 3 , March 2006 , pp. 207 - 212
Copyright
2006 Mac Keith Press

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