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URINARY PHENYLALANINE EXCRETION IN HYPERPHENYL‐ALANINEMIC CHILDREN
Author(s) -
GUTTLER F.,
ROSLEFF F.
Publication year - 1973
Publication title -
acta pædiatrica
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.772
H-Index - 115
eISSN - 1651-2227
pISSN - 0803-5253
DOI - 10.1111/j.1651-2227.1973.tb08116.x
Subject(s) - phenylalanine , hyperphenylalaninemia , excretion , medicine , urinary system , endocrinology , amino acid , biochemistry , chemistry
Summary 24‐hour phenylalanine loading tests were done in 5 children with persistent hyperphenyl‐alaninemia off diet. The urinary excretion of phenylalanine were compared to the excretion after loading of phenylketonuric children on diet. Serum phenylalanine of children with persistent hyperphenylalaninemia returned to preloading levels (285 μmol/l) within 24 hours. These children, however, excreted phenylalanine during phenylalanine loading in lesser quantities than patients with phenylketonuria on diet. Serum phenylalanine of phenylketonuric children on diet attained preloading levels (300 μmol/l) approximately 14 days after loading. Thus, the present data do not support the hypothesis that hyperphenylalaninemic patients are phenylketonurics protected by increased urinary excretion of phenylalanine.