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PHENYLKETONURIA: REDUCTION OF SERUM LEVELS OF PHENYLALANINE FOLLOWING ORAL ADMINISTRATION OF B‐2 THIENYLALANINE
Author(s) -
KRIPS CAROL,
LINES D. R.
Publication year - 1972
Publication title -
journal of paediatrics and child health
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.631
H-Index - 76
eISSN - 1440-1754
pISSN - 1034-4810
DOI - 10.1111/j.1440-1754.1972.tb01845.x
Subject(s) - phenylalanine , medicine , oral administration , mentally retarded , phenylketonurias , endocrinology , biochemistry , amino acid , developmental psychology , chemistry , psychology
SYNOPSIS 8 mentally retarded but otherwise healthy phenylketonuric individuals were given two oral loads of phenylalanine (100 mgm/Kg). One of these loads was accompanied by β‐2‐thienylalanine (20 mgm/Kg). Blood levels of phenylalanine in the 6 hours after the two phenylalanine loads were compared. β‐2‐thienylalanine reduced the levels of phenylalanine in every case. This suggests that if the phenylketonuric child were to take an appropriate amount of β‐thienylalanine with meals, he might be able to eat more phenylalanine, and this would allow a considerably more liberal diet. As diet has been shown to be a dominant factor in the emotional climate of families of phenylketonuric children, this stress would be considerably reduced. Thus, the administration of β‐thienylalanine might lead to a more normal social development of the patient.