Kindt E, Lunde H A, Gjessing L R, Halvorsen S, Lie S O
Pediatric Research Institute, National Hospital of Norway, University of Oslo, Norway.
Acta Paediatr Scand. 1988 Jan;77(1):60-6. doi: 10.1111/j.1651-2227.1988.tb10598.x.
Two groups of children with phenylketonuria were treated with different amounts of protein. One group (RDA group) received protein as recommended by the U.S. Food and Nutrition Board, the other (FAO group) the amount recommended by the FAO/WHO 1973 ad hoc Expert Committee. Two of the children in the FAO group showed a decrease in length/growth velocity which raised the question of the adequacy of the protein content in the diet. Since deviations from normal plasma amino acids have been observed in protein deficient, energy-adequate diets, we examined these parameters in the PKU children. In the two children with retardation in length/growth, very high glycine values as well as very high alanine values were observed at the end of the study. These observations strengthened our suspicion that the FAO/WHO 1973 recommendations are marginal.
两组苯丙酮尿症儿童接受了不同量蛋白质的治疗。一组（RDA组）接受的蛋白质摄入量为美国食品与营养委员会推荐的量，另一组（粮农组织组）接受的是粮农组织/世界卫生组织1973年特设专家委员会推荐的量。粮农组织组的两名儿童身长/生长速度出现下降，这引发了饮食中蛋白质含量是否充足的问题。由于在蛋白质缺乏但能量充足的饮食中已观察到血浆氨基酸偏离正常情况，我们对苯丙酮尿症儿童的这些参数进行了检查。在两名身长/生长发育迟缓的儿童中，研究结束时观察到甘氨酸值和丙氨酸值都非常高。这些观察结果强化了我们的怀疑，即粮农组织/世界卫生组织1973年的建议是临界的。
