一项针对苯丙酮尿症婴儿补充长链多不饱和脂肪酸的随机试验。

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

作者信息

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

机构信息

Department of Pediatrics, University of Milan Medical School, Milan, Italy.

出版信息

Dev Med Child Neurol. 2006 Mar;48(3):207-12. doi: 10.1017/S0012162206000442.

DOI:10.1017/S0012162206000442

PMID:16483397

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.

摘要

42名患有典型苯丙酮尿症（PKU）的婴儿（20名男性，22名女性）参与了一项前瞻性、双盲、随机研究，以调查一种不含苯丙氨酸的婴儿配方奶粉对生化和生理指标的影响。该配方奶粉含有一种脂肪混合物，并添加了长链多不饱和脂肪酸（LC-PUFA）、二十二碳六烯酸（DHA，C22:6 n-3）和花生四烯酸（AA，C20:4 n-6）。在婴儿从入组到20周龄（入组到1岁）期间，补充LC-PUFA组（n=21）红细胞膜磷脂中的DHA水平中位数下降了15%（22%），而对照组（n=18）下降了61%（64%）（p<0.001）。给患有PKU的婴儿提供LC-PUFA饮食可防止与提供极少LC-PUFA来源的饮食相关的DHA水平下降。反过来，独立于饮食的DHA状态可能会影响患有PKU的婴儿视觉系统的成熟。