Acosta P B
Ross Products Division, Abbott Laboratories, Columbus, OH 43215, USA.
Eur J Pediatr. 1996 Jul;155 Suppl 1:S136-9. doi: 10.1007/pl00014231.
Chemically defined or elemental medical foods provide the majority of protein equivalent in the diets of children treated for phenylketonuria (PKU). Because of the restricted intake of high biologic value protein, children with PKU often have lower than normal plasma concentrations of ferritin and zinc. Few reported studies are available on vitamin status of children with PKU undergoing treatment. This report addresses intakes of iron, zinc and vitamin A and their plasma concentrations in children ingesting either a low phenyl-alanine (Phe) casein hydrolysate or a Phe-free L-amino acid mix. With significantly greater than recommended mean intakes of iron and low to recommended mean intakes of zinc, individual plasma ferritin concentrations were often in the deficient (< 12 ng/ml) or marginal (12 < 20 ng/ml) range; plasma zinc concentrations were usually normal when patients received an L-amino acid mix. When L-amino acids were the source of protein equivalent for infants, 48% of plasma retinol concentrations were in the marginal (20 < 30 micrograms/dl) or deficient range (< 20 micrograms/dl) in spite of most vitamin A intakes being greater than Recommended Dietary Allowance for age. Some hypothetical reasons for low concentrations of plasma ferritin and retinol are discussed.
化学定义的或元素性医用食品在接受苯丙酮尿症（PKU）治疗的儿童饮食中提供了大部分蛋白质当量。由于高生物价值蛋白质的摄入量受限，PKU患儿的血浆铁蛋白和锌浓度往往低于正常水平。关于接受治疗的PKU患儿维生素状况的报道研究很少。本报告探讨了摄入低苯丙氨酸（Phe）酪蛋白水解物或无Phe的L-氨基酸混合物的儿童的铁、锌和维生素A摄入量及其血浆浓度。铁的平均摄入量显著高于推荐值，锌的平均摄入量低至推荐值，个体血浆铁蛋白浓度往往处于缺乏（<12 ng/ml）或边缘（12<20 ng/ml）范围；当患者接受L-氨基酸混合物时，血浆锌浓度通常正常。当L-氨基酸作为婴儿蛋白质当量的来源时，尽管大多数维生素A摄入量高于年龄对应的推荐膳食摄入量，但48%的血浆视黄醇浓度处于边缘（20<30微克/分升）或缺乏范围（<20微克/分升）。讨论了血浆铁蛋白和视黄醇浓度低的一些假设原因。
