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Nutrient intake and growth of infants with phenylketonuria undergoing therapy
P B Acosta1, S Yannicelli, B Marriage
1Medical Department, Ross Products Division/Abbott Laboratories, Columbus, Ohio 43215, USA.
Insights
Phenex-1, an amino acid-based medical food, supports normal growth in infants with phenylketonuria when provided in adequate amounts. This study confirms its efficacy in supporting healthy development in these patients.
Area of Science:
- Biochemistry
- Pediatric Nutrition
- Metabolic Disorders
Background:
- Phenylketonuria (PKU) is a genetic disorder requiring dietary management.
- Previous reports suggested potential growth issues with certain medical foods.
Purpose of the Study:
- To evaluate the growth of infants with classic phenylketonuria fed Phenex-1 as their primary protein source.
- To assess the nutritional adequacy of Phenex-1 for supporting infant growth.
Main Methods:
- A 6-month longitudinal study involving 35 infants diagnosed with classic PKU.
- Monthly collection of diet diaries and anthropometric measurements (length, head circumference, weight).
Main Results:
- Infants demonstrated normal growth percentiles for length, head circumference, and weight at 6 months.
- Adequate daily intake of protein, energy, and tyrosine was observed.
- Plasma phenylalanine and tyrosine levels were within acceptable ranges and did not correlate with growth outcomes.
Conclusions:
- Phenex-1 effectively supports normal growth in infants with PKU when administered in sufficient quantities.
- The findings align with recommendations for amino acid intake in PKU management.
Background:
Because of reports of poor growth, a study was conducted for 6 months in 35 infants with classic phenylketonuria diagnosed during the neonatal period who were fed Phenex-1 Amino Acid Modified Medical Food With Iron (Ross Products Division, Columbus, OH, U.S.A.).as their primary protein source.
Methods:
Diet diaries and anthropometric measures were obtained monthly as part of a larger study in which nutrition status was evaluated.
Results:
In 6-month-old infants, mean percentiles for crown-heel length (59.14+/-4.31 SEM), head circumference (63.88+/-4.50) and weight (71.51+/-4.25) were normal. Mean (+/- SEM) daily intake of medical food was 79+/-4 g; protein and energy intakes were 17.3+/-0.6 g and 2772+/-75.6 kJ (660+/-18 kcal). Mean daily phenylalanine and tyrosine intakes per kilogram of body weight were 40+/-1 mg and 219+/-9 mg. Intakes of protein, energy, and tyrosine were positively correlated with crown-heel length, head circumference, and weight at 3 months of study. Overall plasma phenylalanine and tyrosine concentrations during the 6-month study were 297+/-41 micromol/l and 58+/-5 micromol/l, respectively. Neither plasma phenylalanine nor tyrosine concentration was correlated with growth.
Conclusion:
Phenex-1 supports normal growth when fed in adequate amounts. These data support those of the Medical Research Council Working Party on Phenylketonuria for 3 g/kg per day of amino acids from medical food.