School of Life and Health Sciences, Aston University, Birmingham B4 7ET, UK.
Department of Human Neuroscience-Unit of Child Neurology and Psychiatry, Sapienza University of Rome, 00185 Rome, Italy.
Nutrients. 2020 Oct 3;12(10):3033. doi: 10.3390/nu12103033.
We aimed to assess if the same cognitive batteries can be used cross-nationally to monitor the effect of Phenylketonuria (PKU). We assessed whether a battery, previously used with English adults with PKU (AwPKU), was also sensitive to impairments in Italian AwPKU. From our original battery, we selected a number of tasks that comprehensively assessed visual attention, visuo-motor coordination, executive functions (particularly, reasoning, planning, and monitoring), sustained attention, and verbal and visual memory and learning. When verbal stimuli/or responses were involved, stimuli were closely matched between the two languages for psycholinguistic variables. We administered the tasks to 19 Italian AwPKU and 19 Italian matched controls and compared results from with 19 English AwPKU and 19 English matched controls selected from a previously tested cohort. Participant election was blind to cognitive performance and metabolic control, but participants were closely matched for age and education. The Italian AwPKU group had slightly worse metabolic control but showed levels of performance and patterns of impairment similar to the English AwPKU group. The Italian results also showed extensive correlations between adult cognitive measures and metabolic measures across the life span, both in terms of Phenylalanine (Phe) levels and Phe fluctuations, replicating previous results in English. These results suggest that batteries with the same and/or matched tasks can be used to assess cognitive outcomes across countries allowing results to be compared and accrued. Future studies should explore potential differences in metabolic control across countries to understand what variables make metabolic control easier to achieve.
我们旨在评估是否可以使用相同的认知测试在跨国范围内监测苯丙酮尿症 (PKU) 的治疗效果。我们评估了一套先前用于英国成年 PKU 患者（AwPKU）的测试是否也能敏感地反映意大利 AwPKU 患者的认知障碍。我们从原始测试中选择了一些能全面评估视觉注意力、视动协调、执行功能（特别是推理、规划和监控）、持续注意力以及言语和视觉记忆和学习的任务。当涉及言语刺激/反应时，两种语言的刺激在心理语言学变量上是匹配的。我们对 19 名意大利 AwPKU 患者和 19 名意大利对照组进行了测试，并将结果与之前测试过的 19 名英国 AwPKU 患者和 19 名英国对照组进行了比较。参与者的选择是盲法的，不考虑认知表现和代谢控制情况，但参与者在年龄和教育程度上是匹配的。意大利 AwPKU 组的代谢控制稍差，但表现水平和损伤模式与英国 AwPKU 组相似。意大利的结果还表明，在整个生命周期中，成人认知测量与代谢测量之间存在广泛的相关性，无论是在苯丙氨酸（Phe）水平还是 Phe 波动方面，这与之前在英语中的研究结果一致。这些结果表明，使用相同和/或匹配任务的测试可以用于评估跨国认知结果，从而可以比较和累积结果。未来的研究应探讨不同国家代谢控制的潜在差异，以了解哪些变量使代谢控制更容易实现。
