The major metabolites of phenylalanine present in phenylketonuric (PKU) urine, phenylpyruvic, phenyllactic, orthohydroxyphenylacetic, and phenylacetic acids, can be identified by gas chromatography as the trimethylsilyl derivatives, following pretreatment of the urine with hydroxylamine. Extraction from acidified urine with ethyl acetate and ether is followed by reaction with hexamethyldisilazane and chlorotrimethylsilane in pyridine. In addition, phenylacetylglutamine can be measured by the change in the phenylacetic level following acid hydrolysis. Quantitative data from 15 PKU patients are presented. Urinary patterns in PKU are distinct and easily distinguished from the normal.
|Original language||English (US)|
|Number of pages||9|
|Journal||The Journal of Laboratory and Clinical Medicine|
|State||Published - Jan 1971|
ASJC Scopus subject areas
- Pathology and Forensic Medicine