McCabe L, Ernest A E, Neifert M R, Yannicelli S, Nord A M, Garry P J, McCabe E R
Department of Pediatrics, University of Colorado Health Sciences Center, Denver.
J Inherit Metab Dis. 1989;12(4):467-74. doi: 10.1007/BF01802044.
Treatment for phenylketonuria (PKU) involves using low phenylalanine-free or phenylalanine-free formulas and supplementation with sufficient phenylalanine for normal growth and development. Eighteen infants with phenylketonuria who received breast milk as their primary phenylalanine source were compared with ten other infants with PKU who received their phenylalanine primarily from infant formulas. There were no significant differences between breast-fed and formula-fed infants for serum phenylalanine, serum tyrosine, length, weight, head circumference, haematocrit, haemoglobin, serum iron, total iron binding capacity, percentage iron saturation, ferritin, plasma zinc and total calorie intake. Breast-fed infants did show lower mean corpuscular volume at 3 months and 6 months of age. Breast-fed infants had lower phenylalanine intake at 2, 4, 5 and 6 months of age. Breast-fed infants at 1, 2, 3, 4, 5 and 6 months of age had lower protein intake. Breast feeding may be continued in the newly diagnosed phenylketonuric infant without any apparent adverse nutritional consequences.
苯丙酮尿症（PKU）的治疗包括使用低苯丙氨酸或无苯丙氨酸配方奶粉，并补充足够的苯丙氨酸以促进正常生长发育。将18名以母乳作为主要苯丙氨酸来源的苯丙酮尿症婴儿与另外10名主要从婴儿配方奶粉中获取苯丙氨酸的苯丙酮尿症婴儿进行了比较。母乳喂养和配方奶喂养的婴儿在血清苯丙氨酸、血清酪氨酸、身长、体重、头围、血细胞比容、血红蛋白、血清铁、总铁结合力、铁饱和度百分比、铁蛋白、血浆锌和总热量摄入方面没有显著差异。母乳喂养的婴儿在3个月和6个月大时平均红细胞体积确实较低。母乳喂养的婴儿在2、4、5和6个月大时苯丙氨酸摄入量较低。母乳喂养的婴儿在1、2、3、4、5和6个月大时蛋白质摄入量较低。新诊断出的苯丙酮尿症婴儿可以继续母乳喂养，而不会有任何明显的不良营养后果。
