Sievers E, Oldigs H D, Dörner K, Schaub J
Department of Paediatrics, University of Kiel, FRG.
J Inherit Metab Dis. 1990;13(6):897-905. doi: 10.1007/BF01800217.
Knowledge of trace element requirements of infants with phenylketonuria (PKU) fed a semisynthetic diet is limited. Three infants with PKU detected early were studied longitudinally in classical balance studies for 72 h, under domestic conditions, at the ages of 2, 5, 8, 12 and 16 weeks. Iron, copper and manganese concentrations in the diet and faeces were determined by atomic absorption spectroscopy. The median concentrations in the diet (4.8 mg Fe/L, 1.7 mg Cu/L, 0.43 mg Mn/L) exceed those in human milk. This is mainly due to supplementation of the amino acid preparation used. The increased intake led to a significantly higher daily retention of Cu and Mn from the PKU-diet fed, with a median of 0.17 mg Cu/kg and 6.4 micrograms Mn/kg body weight; the median retention of Fe was 0.24 mg Fe/kg. Our results confirmed the doubts about the suitability of the present trace element supplementation in formula for infants with PKU during the first four months of life.
对于采用半合成饮食喂养的苯丙酮尿症(PKU)婴儿的微量元素需求了解有限。对三名早期确诊的PKU婴儿在国内条件下进行了纵向经典平衡研究,研究时长72小时,研究对象年龄分别为2、5、8、12和16周。通过原子吸收光谱法测定饮食和粪便中的铁、铜和锰浓度。饮食中的中位浓度(4.8毫克铁/升、1.7毫克铜/升、0.43毫克锰/升)超过了母乳中的浓度。这主要是由于所使用的氨基酸制剂进行了补充。摄入量的增加导致从PKU饮食中摄入的铜和锰的每日保留量显著更高,铜的中位保留量为0.17毫克/千克,锰为6.4微克/千克体重;铁的中位保留量为0.24毫克/千克。我们的结果证实了对目前用于PKU婴儿的配方奶粉中微量元素补充剂在生命前四个月适用性的质疑。