Hermann M E, Mönch E, Reinbacher M, Kohlen W, Brösicke H G
Kaiserin Auguste Victoria Haus, Universitäts-Kinderklinik, Freie Universität Berlin.
Monatsschr Kinderheilkd. 1991 Oct;139(10):670-5.
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.
在苯丙酮尿症患者的饮食治疗中，给予无苯丙氨酸的氨基酸混合物以满足患者的蛋白质需求。我们对四名健康受试者进行了测试，给予他们四种不同的苯丙酮尿症制剂，以不同的量与含碳水化合物和脂肪的餐食一起摄入。我们测定了血液中的葡萄糖、胰岛素、氨基酸和尿素。在摄入氨基酸后，我们观察到胰岛素分泌增加并伴有低血糖反应，或者在摄入量较小时，餐后血糖没有正常升高。高氨基酸血症取决于摄入的氨基酸量。血液尿素的增加表明部分氨基酸被分解代谢。为了比较结果，我们研究了一名苯丙酮尿症患者。他表现出相应但较轻的影响。
