A synopsis of the unconjugated acidic transamination metabolites of phenylalanine in phenylketonuria
- 10 May 1991
- journal article
- Published by Wiley in Journal of Inherited Metabolic Disease
- Vol. 15 (1) , 136-144
- https://doi.org/10.1007/bf01800355
Abstract
We present blood and urine levels of unconjugatedo-hydroxyphenylacetic, phenyllactic and phenylpyruvic acids in 61 children (2 years of age and above) and juveniles with phenylketonuria on or partially off diet. The samples were obtained during 185 scheduled outpatient visits and have been analysed with gas chromatographic methods. The compiled data define reference ranges of phenylalanine transamination capacity and of renal transport of metabolites which may be of value in further studies on the pathogenesis of phenylketonuria.Keywords
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