Increased excretion of o-hydroxymandelic acid in phenylketonuria.
o-Hydroxymandelic acid has been found in large amounts in the urine of patients with phenylketonuria by gas chromatography mass spectrometry selected ion monitoring. Using deuterated o-hydroxymandelic acid as an internal standard, five patients were found to excrete 150-340 ng mg-1 creatinine. This may be compared with the excretion of 4-16 ng mg-1 creatine by normal subjects. Neither o-octopamine nor o-synephrine were found in urine under conditions by which 1 ng mg-1 creatinine would have been detected.
D008401:Gas Chromatography-Mass Spectrometry; D006801:Humans; D006880:Hydroxy Acids / Q000652:urine; D008333:Mandelic Acids / Q000652:urine; D009655:Octopamine / Q000652:urine; D010661:Phenylketonurias / Q000652:urine*; D013578:Synephrine / Q000652:urine
D006880:Hydroxy Acids; D008333:Mandelic Acids; D009655:Octopamine; C024038:2-hydroxymandelic acid; C028434:2-synephrine; C028435:2-octopamine; D013578:Synephrine