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Nutrient intake of treated infants with phenylketonuria
Insights
Growth in infants with phenylketonuria (PKU) was normal, regardless of treatment group. While phenylalanine intake varied, other nutrient intakes normalized when adjusted for energy. Casein hydrolysate supplementation supported growth.
Area of Science:
- Pediatric Nutrition
- Metabolic Disorders
- Growth and Development
Background:
- Phenylketonuria (PKU) is a genetic disorder requiring dietary management.
- Nutrient intake and growth are critical parameters in PKU treatment.
- Longitudinal studies are essential to evaluate long-term treatment efficacy.
Purpose of the Study:
- To evaluate growth, energy, and nutrient intake in infants treated for PKU.
- To compare outcomes between different treatment groups and normative data.
- To assess the adequacy of nutrient intake based on Recommended Dietary Allowances (RDA).
Main Methods:
- Longitudinal evaluation of 88 infants in the Collaborative Study of Children Treated for PKU.
- Comparison of growth parameters (height, weight) with normative data and treatment groups.
- Analysis of nutrient intake, including phenylalanine, energy, protein, vitamins, and minerals.
Main Results:
- Growth parameters did not differ significantly between treatment groups or from normative data.
- Infants in treatment group 2 had higher phenylalanine intake than group 1.
- Nutrient intake differences (excluding phenylalanine) normalized when adjusted for energy or body weight.
- Most infants met RDA for protein, iron, vitamin A, calcium, phosphorus, thiamin, riboflavin, and ascorbic acid.
- 48-80% of subjects had low preformed niacin intake; 0-10% had low energy intake.
Conclusions:
- Dietary management in PKU infants supported normal growth.
- Casein hydrolysate supplementation appears adequate for essential amino acids and nitrogen.
- Monitoring niacin intake may be important in PKU management.
- Nutrient intake should be assessed relative to energy and body weight for accurate comparisons.
Abstract:
Growth, energy, and nutrient intake of 88 treated infants in the Collaborative Study of Children Treated for Phenylketonuria were evaluated longitudinally and compared to normative data and by treatment group. Growth parameters (height and weight) did not differ according to treatment group assignment, nor did they differ from normative data. Subjects in treatment group 2 had a significantly higher intake of phenylalanine than did subjects in treatment group 1. Differences in intake of other nutrients disappeared when intake was compared on an energy or body weight basis. Differences in intake by males and females also disappeared when compared on an energy and a body weight basis. From none to 10% of the subjects had energy intakes below two-thirds of the 1968 Recommended Dietary Allowances (RDA). Low individual energy intakes were more commonly found in the first and fourth quarters, and only during the first quarter of infancy did mean energy intake meet the RDA. From 48 to 80% of subjects had intakes of preformed niacin below two-thirds of the 1968 RDA. Few subjects had low intakes of protein, iron, or vitamin A, and intakes of all subjects were greater than 67% of the RDA for calcium, phosphorus, thiamin, riboflavin, and ascorbic acid. It is suggested from the data presented that a supplemental casein hydrolysate supplies adequate essential amino acids and nitrogen to support normal growth in infants when protein and energy intakes are fed at the levels described.