Krauch G, Müller E, Anninos A, Bremer H J
Universitäts-Kinderklinik, Heidelberg, Germany.
Eur J Pediatr. 1996 Jul;155 Suppl 1:S153-7. doi: 10.1007/pl00014235.
The protein quality of the diets of phenylketonuria (PKU) children of different ages (3 months, 10 months, 3 years, 8 years, 12 years, 16 years) with low or high phenylalanine (Phe) tolerance was assessed according to the recommendations of the FAO/WHO consultation group [13]. The amount of each essential amino acid (AA) per gram dietary protein was calculated and compared to the reference. The resultant amino acid score (AAS) indicated a limited to inadequate biological protein quality of the diets in 3-month-old infants (2.2 g protein/kg body weight/day) and 10-month-old infants (2.0 g protein/kg body weight/day) with a "high" Phe tolerance. In all other age groups the AAS was > 100%. However remarkable imbalances in the AA pattern were apparent. Beginning with the age of 3 years (1.7 g protein/kg body weight/day) the intake of the AA lysine and isoleucine was three or two times higher than recommended. At the age of 8 years (1.4 g protein/kg body weight/day) the intake of three AA (valine, isoleucine, lysine) was-related to the WHO recommendations-217%, 229% and 291%. Similar results could be found in the age groups of 12 years (1.1 g protein/kg body weight/day) and 16 years (0.9 g protein/kg body weight/day), respectively. These calculations might help to reconsider the composition of the AA mixtures used in the dietetic treatment of PKU patients.
根据粮农组织/世卫组织咨询小组的建议[13]，对不同年龄（3个月、10个月、3岁、8岁、12岁、16岁）、苯丙氨酸（Phe）耐受性低或高的苯丙酮尿症（PKU）儿童饮食中的蛋白质质量进行了评估。计算了每克膳食蛋白质中每种必需氨基酸（AA）的含量，并与参考值进行比较。所得氨基酸评分（AAS）表明，苯丙氨酸耐受性“高”的3个月大婴儿（2.2克蛋白质/千克体重/天）和10个月大婴儿（2.0克蛋白质/千克体重/天）的饮食中，生物蛋白质质量有限至不足。在所有其他年龄组中，氨基酸评分均>100%。然而，氨基酸模式存在明显失衡。从3岁（1.7克蛋白质/千克体重/天）开始，氨基酸赖氨酸和异亮氨酸的摄入量比推荐量高三倍或两倍。在8岁（1.4克蛋白质/千克体重/天）时，三种氨基酸（缬氨酸、异亮氨酸、赖氨酸）的摄入量与世界卫生组织的建议相比分别为217%、229%和291%。在12岁（1.1克蛋白质/千克体重/天）和16岁（0.9克蛋白质/千克体重/天）年龄组中也分别发现了类似结果。这些计算可能有助于重新考虑用于苯丙酮尿症患者饮食治疗的氨基酸混合物的组成。
