Nutrient intake and growth of infants with phenylketonuria undergoing therapy.
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.


CONCLUSIONS
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.
Acosta
P B
PB
Medical Department, Ross Products Division/Abbott Laboratories, Columbus, Ohio 43215, USA.
Yannicelli
S
S
Marriage
B
B
Mantia
C
C
Gaffield
B
B
Porterfield
M
M
Hunt
M
M
McMaster
N
N
Bernstein
L
L
Parton
P
P
Kuehn
M
M
Lewis
V
V
D001827:Body Height; D001835:Body Weight; D004032:Diet; D004044:Dietary Proteins / Q000008:administration & dosage; D002149:Energy Intake; D005260:Female; D006128:Growth; D006257:Head / Q000033:anatomy & histology; D006801:Humans; D007223:Infant; D007227:Infant Nutritional Physiological Phenomena; D008297:Male; D009752:Nutritional Status; D010649:Phenylalanine / Q000008:administration & dosage / Q000097:blood; D010661:Phenylketonurias / Q000503:physiopathology* / Q000628:therapy*; D014443:Tyrosine / Q000008:administration & dosage / Q000097:blood
D004044:Dietary Proteins; D014443:Tyrosine; D010649:Phenylalanine