Bentovim A, Clayton B E, Francis D E, Shepherd J, Wolff O H
Arch Dis Child. 1970 Oct;45(243):640-50. doi: 10.1136/adc.45.243.640.
Twelve children with phenylketonuria diagnosed and treated from the first few weeks of life were grouped into pairs. Before the trial all of them were receiving a commercial preparation containing a protein hydrolysate low in phenylalanine (Cymogran, Allen and Hanburys Ltd.) as a substitute for natural protein. One of each pair was given an amino acid mixture instead of Cymogran for about 6 months. Use of the mixture involved considerable modification of the diet, and in particular the inclusion of greater amounts of phenylalanine-free foods. All six accepted the new mixture without difficulty, food problems were greatly reduced, parents welcomed the new preparation, and the quality of family life improved. Normal growth was maintained and with a mixture of l amino acids the plasma and urinary amino acid levels were normal. Further studies are needed before the mixture can be recommended for children under 20 months of age.
12名从出生后最初几周就被诊断并接受治疗的苯丙酮尿症患儿被分成了几组。在试验前，他们都在服用一种含有低苯丙氨酸蛋白水解物的商业制剂（Cymogran，艾伦和汉伯里有限公司）来替代天然蛋白质。每组中的一名患儿服用氨基酸混合物替代Cymogran，持续约6个月。使用该混合物需要对饮食进行相当大的调整，特别是要增加无苯丙氨酸食物的摄入量。所有6名患儿都顺利接受了新的混合物，食物问题大幅减少，家长对新制剂表示欢迎，家庭生活质量得到改善。患儿保持了正常生长，使用L-氨基酸混合物时血浆和尿液中的氨基酸水平正常。在该混合物可被推荐用于20个月以下儿童之前，还需要进一步研究。
