Protein intake affects phenylalanine requirements and growth of infants with phenylketonuria

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Abstract
Growth and metabolic status of 25 infants with PKU were evaluated based on protain intake. Food A‐fed infants received a medical food containing 3.12g protein equivalent per 100 kcal and Food B‐fed infants received a medical food containing 2.74 g protein equivalent per 100 kcal. Growth percentiles of infants in the Food A group were significantly greater than those for infants in the Food B group at 6 months of age (Food A percentiles: crown‐heel length 55, head circumference 60, weight 73. Food B percentiles: crown‐heel length 28; head circumference 29, weight 39). At study entrance, only crown‐heel length of the two groups differed; Food B infants had a significantly greater mean crown‐heel length percentile (p < 0.05). Mean phenylalanine (PHE) intake was 38% greater by Food A‐fed infants than by Food B‐fed infants. Plasma PHE concentrations and mean energy intakes of the two groups did not differ. Mean protein intake of Food A‐fed infants was greater during the first three months of life and significantly greater (p < 0.05) during the second three months of life than by Food B‐fed infants. Mean protein intake 24% greater than Recommended Dietary Allowances (RDA) was associated with better PHE tolerance and growth than was found when mean protein intake was 9% greater than RDA.