Koletzko Berthold, Beblo Skadi, Demmelmair Hans, Müller-Felber Wolfgang, Hanebutt Fabienne L
Division of Metabolic Diseases and Nutritional Medicine, Dr. von Hauner Children's Hospital, University of Munich Medical Centre, Lindwurmstr. 4, 80337 Munich, Germany.
Prostaglandins Leukot Essent Fatty Acids. 2009 Aug-Sep;81(2-3):159-64. doi: 10.1016/j.plefa.2009.06.006. Epub 2009 Jul 16.
Children with phenylketonuria (PKU) have a restricted protein intake and thus low dietary intakes of long-chain polyunsaturated fatty acids (LC-PUFA), which may cause subtle neurological deficits. We measured plasma phospholipid fatty acids and visual evoked potential (VEP) in 36 children with well-controlled PKU (6.3+/-0.6 years, 19 girls), before and after 3 months of supplementing fish oil capsules providing 15 mg docosahexaenoic acid (DHA)/kg daily. The motometric Rostock-Oseretzky Scale (ROS) was performed before and after supplementation in the 24 PKU children aged >4 years. VEP latencies and ROS were also assessed in omnivorous, age-matched controls without fish oil supply at baseline and after 3 months. Fish oil supply increased plasma phospholipid eicosapentaenoic acid (EPA) (0.40+/-0.03 vs 3.31+/-0.19%, p<0.001) and DHA (2.37+/-0.10 vs 7.05+/-0.24%, p<0.001), but decreased arachidonic acid (AA) (9.26+/-0.23 vs 6.76+/-0.16%, p<0.001). Plasma phenylalanine was unchanged. VEP latencies and ROS results significantly improved after fish oil in PKU children, but remained unchanged in controls. The improvements of VEP latencies, fine motor and coordination skills indicate that preformed n-3 LC-PUFA are needed for neural normalcy in PKU children. The optimal type and dose of supply still needs to be determined. Since PKU children are generally healthy and have normal energy and fatty acid metabolism, these data lead us to conclude that childhood populations in general require preformed n-3 LC-PUFA to achieve optimal neurological function.
患有苯丙酮尿症（PKU）的儿童蛋白质摄入量受限，因此膳食中长链多不饱和脂肪酸（LC-PUFA）的摄入量较低，这可能会导致轻微的神经功能缺陷。我们对36名苯丙酮尿症控制良好的儿童（6.3±0.6岁，19名女孩）进行了血浆磷脂脂肪酸和视觉诱发电位（VEP）测量，这些儿童在每日补充提供15毫克二十二碳六烯酸（DHA）/千克的鱼油胶囊3个月前后各进行一次测量。对24名年龄大于4岁的苯丙酮尿症儿童在补充前后进行了运动功能的罗斯托克-奥塞雷茨基量表（ROS）测试。在基线和3个月后，还对不补充鱼油的杂食性、年龄匹配的对照组进行了VEP潜伏期和ROS评估。补充鱼油后，血浆磷脂中的二十碳五烯酸（EPA）增加（0.40±0.03%对3.31±0.19%，p<0.001），DHA增加（2.37±0.10%对7.05±0.24%，p<0.001），但花生四烯酸（AA）减少（9.26±0.23%对6.76±0.16%，p<0.001）。血浆苯丙氨酸未发生变化。苯丙酮尿症儿童补充鱼油后，VEP潜伏期和ROS结果显著改善，但对照组保持不变。VEP潜伏期、精细运动和协调技能的改善表明，苯丙酮尿症儿童的神经正常发育需要预先形成的n-3 LC-PUFA。最佳的供应类型和剂量仍有待确定。由于苯丙酮尿症儿童一般健康，能量和脂肪酸代谢正常，这些数据使我们得出结论，一般儿童群体需要预先形成的n-3 LC-PUFA以实现最佳的神经功能。
