Nutrient intake and growth of infants with phenylketonuria undergoing therapy.
J Pediatr Gastroenterol Nutr
; 27(3): 287-91, 1998 Sep.
Article
in En
| MEDLINE
| ID: mdl-9740198
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.
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Collection:
01-internacional
Database:
MEDLINE
Main subject:
Phenylketonurias
/
Growth
/
Infant Nutritional Physiological Phenomena
Limits:
Female
/
Humans
/
Infant
/
Male
Language:
En
Journal:
J Pediatr Gastroenterol Nutr
Year:
1998
Document type:
Article
Affiliation country:
United States