Influence of the phenylalanine dose on the aromatic acid excretion in phenylketonuric heterozygotes and controls after oral loading

Research paper by G. Dahmann, K. Olek, A. Pravitz, P. Wardenbach

Indexed on: 01 Sep '78Published on: 01 Sep '78Published in: European Journal of Pediatrics


Heterozygotes for phenylketonuria and controls were given oral loads of 100 mg and 200 mg L-phenylalanine per kilogram body weight. The concentrations of urinary aromatic acids were determined by gas-chromatography after isolation by ion-exchange chromatography and ethylacetate extraction. On an intake of 100 mg L-phenylalanine per kilogram, controls and carriers of classical phenylketonuria excreted nearly the same amounts of aromatic acids (P>0.05). However on an intake of 200 mg per kilogram L-phenylalanine they could be distinguished from one another (P<0.001).