A randomized trial of long-chain polyunsaturated fatty acid supplementation in infants with phenylketonuria.
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.
Department of Pediatrics, University of Milan Medical School, Milan, Italy. carlo.agostoni@unimi.it
Carlo
C
Agostoni
Ann
A
Harvie
Daphne L
DL
McCulloch
Colin
C
Demellweek
Forrester
F
Cockburn
Marcello
M
Giovannini
Gordon
G
Murray
R Angus
RA
Harkness
Enrica
E
Riva
D002658:Developmental Disabilities / Q000175:diagnosis / Q000453:epidemiology; D004311:Double-Blind Method; D005074:Evoked Potentials, Visual / Q000502:physiology; D005231:Fatty Acids, Unsaturated / Q000627:therapeutic use*; D005260:Female; D006801:Humans; D007223:Infant; D008297:Male; D010649:Phenylalanine / Q000097:blood; D010661:Phenylketonurias / Q000097:blood / Q000188:drug therapy*; D011446:Prospective Studies
D005231:Fatty Acids, Unsaturated; D010649:Phenylalanine