title: Comparison of the protein quality of dietetically treated phenylketonuria patients with the recommendations of the WHO Expert Consultation.
abstract: The protein quality of the diets of phenylketonuria (PKU) children of different ages (3 months, 10 months, 3 years, 8 years, 12 years, 16 years) with low or high phenylalanine (Phe) tolerance was assessed according to the recommendations of the FAO/WHO consultation group [13]. The amount of each essential amino acid (AA) per gram dietary protein was calculated and compared to the reference. The resultant amino acid score (AAS) indicated a limited to inadequate biological protein quality of the diets in 3-month-old infants (2.2 g protein/kg body weight/day) 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%. However 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 recommended. At the age of 8 years (1.4 g protein/kg body weight/day) the intake of three AA (valine, isoleucine, lysine) was-related to the WHO recommendations-217%, 229% and 291%. Similar results could be found 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 calculations might help to reconsider the composition of the AA mixtures used in the dietetic treatment of PKU patients.
authors.affiliation: Universitäts-Kinderklinik, Heidelberg, Germany.
authors.forename: G
authors.initials: G
authors.lastname: Krauch
authors.forename: E
authors.initials: E
authors.lastname: Müller
authors.forename: A
authors.initials: A
authors.lastname: Anninos
authors.forename: H J
authors.initials: HJ
authors.lastname: Bremer
mesh_terms: D000293:Adolescent; D000601:Amino Acids, Essential / Q000008:administration & dosage*; D002648:Child; D002675:Child, Preschool; D018753:Diet, Protein-Restricted; D004044:Dietary Proteins / Q000032:analysis*; D006801:Humans; D007223:Infant; D009751:Nutritional Requirements; D010661:Phenylketonurias / Q000178:diet therapy*; D014944:World Health Organization
chemical_list: D000601:Amino Acids, Essential; D004044:Dietary Proteins