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首页> 美国卫生研究院文献>JIMD Reports >The Use of d2 and Benton Tests for Assessment of Attention Deficits and Visual Memory in Teenagers with Phenylketonuria
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The Use of d2 and Benton Tests for Assessment of Attention Deficits and Visual Memory in Teenagers with Phenylketonuria

机译：d2和Benton检验在评估青少年苯丙酮尿症的注意缺陷和视觉记忆中的应用

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Hyperphenylalaninemia-related, subtle deficits of attention and of working memory are often reported in adolescents with phenylketonuria. Focused neuropsychological tests can be used to detect such deficits and to confirm the presence of poor metabolic control in the periods between routine blood phenylalanine tests, which are rarely performed in many patients from this age group due to their low treatment adherence.We assessed the practical value of the d2 test of attention and of the Benton visual retention test for identification of teenagers, who have a high risk of brain dysfunction due to hyperphenylalaninemia. We analyzed the correlation between neuropsychological test scores achieved by 30 patients and their blood phenylalanine profiles since the neonatal period.We observed strong correlation between the Concentration Performance scores on the d2 test and the quality of metabolic control within last month prior to the follow-up visit in the outpatient clinic (r = −0.72; p = 0.0003). The mean z-score was significantly higher in patients with good metabolic control than in those with poorly controlled hyperphenylalaninemia (0.44 vs. −1.12; p = 0.00002). On contrary, the results of the Benton visual retention test did not correlate significantly with the individual blood phenylalanine profiles.We believe that neuropsychological assessment should be used in adolescents with phenylketonuria on a regular basis in order to increase the self-awareness in these patients and, consequently, to increase their treatment adherence and safety. The d2 test can be effectively used for detection of attention deficits and seems to be a valuable supplementary procedure for routine follow-up.
机译：在患有苯丙酮尿症的青少年中，经常会报告与高苯丙氨酸血症相关的注意力和工作记忆的细微缺陷。集中的神经心理学测试可用于检测此类缺陷并确认常规血液苯丙氨酸检测之间存在代谢控制不佳的情况，该年龄段的许多患者由于治疗依从性差而很少进行此类检测。 d2注意测试和Benton视觉保持测试对识别因高苯丙氨酸血症而导致脑功能异常的高风险青少年的价值。我们分析了自新生儿期以来30例患者获得的神经心理学测验得分与其血液苯丙氨酸谱之间的相关性。我们观察到，在随访之前的最后一个月内，d2测验中的注意力成绩得分与代谢控制质量之间存在密切相关性在门诊就诊（r = −0.72; p = 0.0003）。代谢控制良好的患者的平均z得分显着高于控制不佳的高苯丙氨酸血症的患者（0.44 vs.-1.12; p = 0.00002）。相反，Benton视觉保留测试的结果与个体血液中的苯丙氨酸谱没有显着相关性。我们认为，应定期对患有苯丙酮尿症的青少年进行神经心理学评估，以提高这些患者的自我意识和因此，以增加其治疗依从性和安全性。 d2检验可以有效地用于检测注意缺陷，并且似乎是常规随访的有价值的补充程序。

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