PLASMA & ERYTHROCYTE FATTY ACIDS IN TREATED CHILDREN W/ PKU

本研究旨在验证以下假设：1)不同药膳治疗的PKU患儿与正常儿童的必需脂肪酸（EFAS）摄取量无差异；2)不同药膳治疗的PKU患儿与正常儿童的血浆和红细胞脂肪酸摄取量无差异。研究人员将比较服用洛芬那酸、Phenex、无苯基或XP Maximaid的PKU治疗儿童与正常兄弟姐妹的EFA摄入量。将服用洛芬那酸、Phenex、无苯基或XP Maxamaid治疗的PKU患儿的血浆和红细胞脂肪酸谱与正常兄弟姐妹的脂肪酸谱进行比较。

This proposal is designed to test the hypotheses that: 1) there are no differences in the intake of essential fatty acids (EFAS) among groups of children with PKU fed different medical foods and normal children, and 2) there are no differences in the plasma and erythrocyte fatty acids among groups of children undergoing therapy for PKU with different medical foods and normal children. The investigators will compare EFA intake of children undergoing therapy for PKU who are ingesting Lofenalac, Phenex, Phenyl-free or XP Maximaid to intakes of their normal siblings. Plasma and erythrocyte fatty acid profiles of children undergoing therapy for PKU who are ingesting Lofenalac, Phenex, Phenyl-free, or XP Maxamaid will be compared to the fatty acid profiles of their normal siblings.