Department of Metabolic Disease, Wilhelmina Children's Hospital Utrecht, University Medical Center, Utrecht, The Netherlands.
Mol Genet Metab. 2013 Jun;109(2):121-4. doi: 10.1016/j.ymgme.2013.03.006. Epub 2013 Mar 21.
Patients with phenylketonuria (PKU) have a poor LC-PUFA status and require supplementation. The objective of this study was to evaluate the LC-PUFA status of PKU patients supplemented with fish oil or the fatty acid supplement KeyOmega. Plasma and erythrocyte docosahexaenoic acid (DHA) and arachidonic acid (AA) levels were determined in 54 patients (1-18.5years of age) with confirmed PKU. The influence of supplementation with fish oil versus KeyOmega, a powdered blend of DHA and AA, on LC-PUFA status was investigated and compared to the status in samples obtained from unsupplemented patients. Differential effects on LC-PUFA status were observed upon suppletion with fish oil versus KeyOmega. Whereas supplementation with fish oil increased the level of DHA, the AA concentration did not increase to normal values in these patients. In contrast, both DHA and AA levels increased and reached reference values upon supplementation with KeyOmega.
these results indicate that KeyOmega offers additional benefit over fish oil since both AA and DHA status are normalized in PKU patients supplemented with KeyOmega.
患有苯丙酮尿症（PKU）的患者 LC-PUFA 状态较差，需要补充。本研究的目的是评估补充鱼油或脂肪酸补充剂 KeyOmega 的 PKU 患者的 LC-PUFA 状态。在 54 名确诊 PKU 患者（1-18.5 岁）中测定了血浆和红细胞二十二碳六烯酸（DHA）和花生四烯酸（AA）水平。研究了补充鱼油与 KeyOmega（DHA 和 AA 的粉末混合物）对 LC-PUFA 状态的影响，并将其与未补充患者的样本状态进行了比较。补充鱼油与 KeyOmega 的效果不同。尽管补充鱼油可增加 DHA 水平，但 AA 浓度并未在这些患者中增加至正常水平。相比之下，补充 KeyOmega 可使 DHA 和 AA 水平均增加并达到参考值。
这些结果表明，KeyOmega 比鱼油具有额外的益处，因为补充 KeyOmega 后 PKU 患者的 AA 和 DHA 状态均正常化。
