苯酮酸尿病是由于肝中缺乏苯丙氨酸羟化酶,致使体内苯丙氨酸不能变为酪氨酸而引起苯丙氨酸代谢紊乱。其主要表现是血清中苯丙氨酸和血清及尿中苯丙酮酸升高,有时伴随着精神障碍。给苯酮酸尿病人以高亮氨酸而蛋白质平衡的玉米膳(每天每公斤体重供给0.55克蛋白质,27毫克苯丙氨酸,32卡热量),水解酪蛋白膳(每天每公斤体重供给1克蛋白质,21毫克苯丙氨酸,35卡热量)。 Phenylketonuria is due to the lack of phenylalanine hydroxylase in the liver, resulting in phenylalanine can not become tyrosine and cause phenylalanine metabolism disorders. Its main manifestations are phenylalanine serum and urine phenylpropylate acid increased, sometimes accompanied by mental disorders. To phenylketonuria patients with leucine and protein balance corn meal (0.55 grams of protein per kilogram of body weight per day, 27 mg of phenylalanine, 32 calories), hydrolyzed casein diet (daily supply of 1 kg body weight G protein, 21 mg phenylalanine, 35 calories).