[Phenylalanine-free amino acid mixture: metabolic effects in relation to a single dose]

In the dietetic treatment of phenylketonuric patients phenylalanine free amino acid mixtures are given to meet the protein requirement of the patient. In four healthy probands we tested the metabolic effects of four different PKU-preparations given in variable amounts ingested with carbohydrate and fat containing meals. We determined glucose, insulin, amino acids, and urea in blood. Following the amino acid load we saw an increased insulin output with a hypoglycemic reaction or, with smaller amounts a lack of the normal postprandial blood glucose increase. Hyperaminoacidemias depended on the amount of amino acids ingested. The increase of blood urea found suggests that part of the amino acids were degraded. To compare the results we studied a PKU-patient. He showed corresponding but milder effects.