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目的研究PKU患儿治疗期间血控制浓度与智商的关系。方法 PKU患儿治疗期间通过荧光法定期检测phe血浓度,调节奶粉口服量。智商检测采用CDCC智能量表与动作量表测定,同时在患儿1周岁时测定其智商(IQ)。结果 IQ<70组,其phe控制浓度为(8.00+1.83)mg/dl。IQ70-90组其phe控制浓度为(5.20+1.64)mg/dl。IQ﹥90组其phe控制浓度为(3.26+0.64)mg/dl。PKU患儿治疗过程中phe控制浓度与IQ呈负相关,r=-0.795。结论严格控制phe血浓度是提高智商的关键因素。
Objective To study the relationship between blood concentration and IQ in children with PKU during treatment. Methods During the treatment of PKU, the concentration of phe in blood was measured regularly by fluorometry to adjust the oral dose of milk powder. IQ test using the CDCC intelligent scale and the action scale determination, at the same time in children with 1 year old measured IQ (IQ). Results IQ <70 group, the phe concentration of (8.00 + 1.83) mg / dl. The concentration of phe in IQ70-90 group was (5.20 + 1.64) mg / dl. IQ> 90 group phe control concentration (3.26 + 0.64) mg / dl. PKU treatment of children with phe concentration and IQ was negatively correlated (r = -0.795). Conclusions Strict control of phe concentration is the key factor to improve IQ.