Nutrient intake and growth of infants with phenylketonuria undergoing therapy.

Abstract

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.

• Authors: P B Acosta, S Yannicelli, B Marriage, C Mantia, B Gaffield, M Porterfield, M Hunt, N McMaster, L Bernstein, P Parton, M Kuehn, V Lewis
• Journal: Journal of pediatric gastroenterology and nutrition (J Pediatr Gastroenterol Nutr) Vol. 27 Issue 3 Pg. 287-91 (Sep 1998) ISSN: 0277-2116 [Print] United States
• PMID: 9740198 (Publication Type: Journal Article)

Chemical References

• Dietary Proteins
• Tyrosine
• Phenylalanine

Topics

• Body Height
• Body Weight
• Diet
• Dietary Proteins (administration & dosage)
• Energy Intake
• Female
• Growth
• Head (anatomy & histology)
• Humans
• Infant
• Infant Nutritional Physiological Phenomena
• Male
• Nutritional Status
• Phenylalanine (administration & dosage, blood)
• Phenylketonurias (physiopathology, therapy)
• Tyrosine (administration & dosage, blood)