G. Krauch et al., COMPARISON OF THE PROTEIN-QUALITY OF DIETETICALLY TREATED PHENYLKETONURIA PATIENTS WITH THE RECOMMENDATIONS OF THE WHO EXPERT CONSULTATION, European journal of pediatrics, 155, 1996, pp. 153-157
The protein quality of the diets of phenylketonuria (PKU) children of
different ages (3 months, 10 months, 3 years, 8 years, 12 years, 16 ye
ars) with low or high phenylalanine (Phe) tolerance was assessed accor
ding to the recommendations of the FAO/WHO consultation group [13]. Th
e amount of each essential amino acid (AA) per gram dietary protein wa
s calculated and compared to the reference. The resultant amino acid s
core (AAS) indicated a limited to inadequate biological protein qualit
y of the diets in 3-month-old infants (2.2 g protein/kg body weight/da
y) and 10-month-old infants (2.0 g protein/kg body weight/day) with a
''high'' Phe tolerance. In all other age groups the AAS was > 100%. Ho
wever remarkable imbalances in the AA pattern were apparent. Beginning
with the age of 3 years (1.7 g protein/kg body weight/day) the intake
of the AA lysine and isoleucine was three or two times higher than re
commended. At the age of 8 years (1.4 g protein/kg body weight/day) th
e intake of three AA (valine, isoleucine, lysine) was - related to the
WHO recommendations - 217%, 229% and 291%. Similar results could be f
ound in the age groups of 12 years (1.1 g protein/kg body weight/day)
and 16 years (0.9 g protein/kg body weight/day), respectively. These c
alculations might help to reconsider the composition of the AA mixture
s used in the dietetic treatment of PKU patients.