Effect of fish oil supplementation on fatty acid status, coordination, and fine motor skills in children with phenylketonuria

被引:65
作者
Beblo, Skadi [1 ]
Reinhardt, Hannes [1 ]
Demmelmair, Hans [1 ]
Muntau, Ania C. [1 ]
Koletzko, Berthold [1 ]
机构
[1] Univ Munich, Dr Von Haunerschen Kinderspital, Div Metab Dis & Nutr, D-80337 Munich, Germany
关键词
D O I
10.1016/j.jpeds.2006.12.011
中图分类号
R72 [儿科学];
学科分类号
100202 ;
摘要
Objective To investigate effects of long-chain omega-3 polyunsaturated fatty acids (LC-PUFA) on motor skills in patients with phenylketonuria (PKU). Study design Thirty-six patients with PKU (1-11 years of age, good metabolic control: plasma phenylalanine <= 360 mu mol/L for >= 6 months). We determined plasma phospholipid fatty acids, and in patients > 4 years of age (N = 24) the motometric Rostock-Oseretzky Scale (ROS), before and after supplementation with fish oil for 3 months (15 mg docosahexaenoic acid [DHA]/kg body weight daily). ROS was also assessd in 22 age-matched controls. Results Patients had low n-3 LC-PUFA in plasma phospholipids (DHA, 2.37 +/- 0.10%; eicosapentaenoic acid [EPA], 0.4 +/- 0.03%) and poorer ROS performance than controls (motor development index [MQ] 107 +/- 3 vs 117 +/- 3, P = .010). Supplementation increased phospholipid n-3 LC-PUFA (DHA 7.05 +/- 0.24%; EPA 3.31 +/- 0.19%; P < .001), decreased n-6 LC-PUFA (arachidonic acid, 9.26 +/- 0.23% vs 6.76 +/- 0.16%; P < .001) and improved RIDS (MQ 115 +/- 3.54, P = .011, paired t test). ROS was unchanged in 11 retested controls (MQ 115 +/- 5.16, P = NS, paired it test multivariate analysis of variance [MANOVA] for time by group, P = .027). Patients tolerated fish oil well. Plasma phenylalanine remained unchanged. Conclusion in patients with PKU, fish oil supplementation enhances n-3 LC-PUFA levels and improves motor skills.
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收藏
页码:479 / 484
页数:6
相关论文
共 42 条
[1]   ZINC STATUS AND GROWTH OF CHILDREN UNDERGOING TREATMENT FOR PHENYLKETONURIA [J].
ACOSTA, PB ;
FERNHOFF, PM ;
WARSHAW, HS ;
ELSAS, LJ ;
HAMBIDGE, KM ;
ERNEST, A ;
MCCABE, ERB .
JOURNAL OF INHERITED METABOLIC DISEASE, 1982, 5 (02) :107-110
[2]   Effects of long-chain polyunsaturated fatty acid supplementation on fatty acid status and visual function in treated children with hyperphenylalaninemia [J].
Agostoni, C ;
Massetto, N ;
Biasucci, G ;
Rottoli, A ;
Bonvissuto, M ;
Bruzzese, AG ;
Giovannini, M ;
Riva, E .
JOURNAL OF PEDIATRICS, 2000, 137 (04) :504-509
[3]  
[Anonymous], METABOLIC MOL BASIS
[4]   Factors affecting cognitive, motor, behavioral and executive functioning in children with phenylketonuria [J].
Arnold, GL ;
Kramer, BM ;
Kirby, RS ;
Plumeau, PB ;
Blakely, EM ;
Cregan, LSS ;
Davidson, PW .
ACTA PAEDIATRICA, 1998, 87 (05) :565-570
[5]  
Artuch R, 1999, AM J CLIN NUTR, V70, P892
[6]   Fish oil supplementation improves visual evoked potentials in children with phenylketonuria [J].
Beblo, S ;
Reinhardt, H ;
Muntau, AC ;
Mueller-Felber, W ;
Roscher, AA ;
Koletzko, B .
NEUROLOGY, 2001, 57 (08) :1488-1491
[7]   DISTURBED MYELINATION IN PATIENTS WITH TREATED HYPERPHENYLALANINEMIA - EVALUATION WITH MAGNETIC-RESONANCE-IMAGING [J].
BICK, U ;
FAHRENDORF, G ;
LUDOLPH, AC ;
VASSALLO, P ;
WEGLAGE, J ;
ULLRICH, K .
EUROPEAN JOURNAL OF PEDIATRICS, 1991, 150 (03) :185-189
[8]   WHITE-MATTER ABNORMALITIES IN PATIENTS WITH TREATED HYPERPHENYLALANINEMIA - MAGNETIC-RESONANCE RELAXOMETRY AND PROTON SPECTROSCOPY FINDINGS [J].
BICK, U ;
ULLRICH, K ;
STOBER, U ;
MOLLER, H ;
SCHUIERER, G ;
LUDOLPH, AC ;
OBERWITTLER, C ;
WEGLAGE, J ;
WENDEL, U .
EUROPEAN JOURNAL OF PEDIATRICS, 1993, 152 (12) :1012-1020
[9]   A randomized controlled trial of early dietary supply of long-chain polyunsaturated fatty acids and mental development in term infants [J].
Birch, EE ;
Garfield, S ;
Hoffman, DR ;
Uauy, R ;
Birch, DG .
DEVELOPMENTAL MEDICINE AND CHILD NEUROLOGY, 2000, 42 (03) :174-181
[10]   Visual acuity and the essentiality of docosahexaenoic acid and arachidonic acid in the diet of term infants [J].
Birch, EE ;
Hoffman, DR ;
Uauy, R ;
Birch, DG ;
Prestidge, C .
PEDIATRIC RESEARCH, 1998, 44 (02) :201-209