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经过早期严格低苯丙氨酸饮食控制的苯丙酮尿症儿童 ,血苯丙氨酸水平控制在 36 0～ 40 0 μmol L之间 ,智力水平可接近正常 ,但仍可能出现智力发育的不平衡 ;PKU儿童的认知损伤目前多认为是额叶功能特异性损伤 ,如记忆、注意力及执行功能的损伤 ;在行为问题上多表现为内向型行为问题 ,但男女可能存在有差异 ;脑磁共振成像(MRI)异常表现为白质病变 ,MRI的异常表现与血Phe水平及脑Phe水平可能存在关系 ,但仍有争议。
In children with phenylketonuria, who were controlled by the early strict hypo-phenylalanine diet, the level of para-phenylalanine was controlled between 36 0 and 40 0 μmol L and the intelligence level was close to normal. However, imbalances in intelligence development may still occur At present, cognitive impairment in PKU children is mostly considered as prefrontal-specific injury, such as impairment of memory, attention and executive function. In behavioral problems, it manifests as introverted behavioral problems, but there may be differences between men and women. Abnormal resonance imaging (MRI) manifests as white matter lesion. The abnormal MRI findings may be related to blood Phe level and brain Phe level, but there is still controversy.