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在小儿痴呆病中,尤以“苯丙酮酸尿症”引起的智力发育不全最为多见。该病主要是先天性肝内缺乏苯丙氨酸羟化酶,使苯丙氨酸代谢异常所致。因先天缺乏这种苯丙氨酸羟化酶,致使苯丙酸不能转变成酪氨酸,从而造成苯丙氨酸大量堆积, 浓度可高达正常的几倍到几十倍。过量的苯丙酸在其它酶的作用下,转变成苯丙酮酸和苯乳酸、苯乙酸等从尿中排出。如果在尿中加进12滴10%的三氯化铁溶液,立即显示出深蓝绿色,医生便可诊断为“苯丙酮酸尿症”性小儿痴呆
In children with dementia, especially “phenylketonuria ” caused by mental retardation most common. The disease is mainly congenital liver phenylalanine hydroxylase deficiency, phenylalanine metabolism caused by abnormal. Because of the inherent lack of this phenylalanine hydroxylase, resulting in benzene propionate can not be converted into tyrosine, resulting in a large accumulation of phenylalanine, the concentration can be up to several times the normal several times. Excess phenylalanine under the action of other enzymes, into phenylpyruvate and benzene lactic acid, phenylacetic acid, etc. discharged from the urine. If you add 12 drops of 10% ferric chloride solution in your urine, it will immediately show a dark blue-green color and the doctor can diagnose it as “phenylketonuria” or “dementia”