Frontal lobe-dependent functions in treated phenylketonuria: Blood phenylalanine concentrations and long-term deficits in adolescents young adults
Frontal lobe-dependent functions in treated phenylketonuria: Blood phenylalanine concentrations and long-term deficits in adolescents young adults
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DOI：
10.1007/s10545-005-0445-7
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发表时间：
2005-08-01
影响因子：
4.2
通讯作者：
Weglage, J
中科院分区：
文献类型：
--
作者：
Feldmann, R;Denecke, J;Weglage, J
即使是早期治疗的苯丙酮尿症患者也可能患有苯丙氨酸相关的缺陷。苯丙氨酸浓度升高会干扰CNS的发育和功能。儿童期以后的结局尚未得到广泛研究。这项长期研究旨在确定与糖尿病患者相比，青少年和年轻人PKU是否表现出额叶依赖性缺陷。比较研究包括35例PKU患者，年龄13-21岁（平均17.8岁），以及35例性别、年龄和社会经济地位相匹配的糖尿病患者。对患者进行智商（文化公平智力测验）、信息处理（连线测验）和选择性和持续性注意力（Stroop任务，测验d-2）评估。在3年随访期内重复评估。PKU患者在随访时血液苯丙氨酸浓度没有增加。在两个评估时间，他们的测试结果都明显低于糖尿病患者。然而，在测试中，这是由于表现速度降低，而不是特定额叶依赖功能的缺陷。苯丙氨酸浓度升高似乎会产生整体效应，减缓运动速度。这种影响在青春期和成年早期是持久的。
Even early-treated phenylketonurics may suffer from phenylalanine-related deficits. Elevated phenylalanine concentrations can interfere with the development and function of the CNS. Outcome beyond childhood has not been extensively investigated. This long-term study was performed to determine whether adolescents and young adults with PKU show frontal lobe-dependent deficits when compared to diabetic patients. The comparative study covered 35 PKU patients, 13-21 years of age (mean 17.8 years), and 35 diabetic patients matched for sex, age and socioeconomic status. Patients were assessed for IQ (Culture Fair Intelligence Test), information processing (Trail Making Test), and selective and sustained attention (Stroop Task, Test d-2). Assessments were repeated within a 3-year follow-up. PKU patients showed no increase in blood phenylalanine concentrations at follow-up. They had significantly poorer test results than the diabetic patients at both assessment times. Within the tests, however, this was due to reduced performance speed but not to deficits in specific frontal lobe-dependent functions. Elevated phenylalanine concentrations seem to exert a global effect slowing performance speed. This effect is enduring in adolescence and early adulthood.