Outcomes in mild hyperphenylalaninemia: a comparison with PKU and healthy controls across cognition, behaviour, and quality of life.

AIMS: Considering the cognitive, behavioural and quality of life (QoL) consequences of high phenylalanine levels in early treated phenylketonuria (PKU), this study examined whether monitoring and active management of individuals with the mild form of the condition hyperphenylalaninemia (HPA) would be advisable. METHOD: Six individuals (aged 6 to 15) with untreated HPA were compared with six age and gender matches with PKU, and six healthy controls on the Wechsler Intelligence Scale for Children, 5th edition; Wechsler Individual Achievement Test, 2nd edition; Trail-Making test; Contingency Naming Test; and Oral Fluency test. Self- and parent-report rating scales administered included the Conners Comprehensive Behavior Rating Scales; Behavior Rating Inventory of Executive Function, 2nd edition; the Pediatric Quality of Life Inventory, and the Phenylketonuria Quality of Life (PKU group only) questionnaires. RESULTS: Early treated PKU participants demonstrated normal intelligence, pointing to the efficacy of dietary management. Quality of life and behavioural difficulties were observed including more severe externalising problems. HPA participants showed normal ability, including executive ability. Power was limited by the small sample. CONCLUSION: This was the first study of the New Zealand population with HPA. While there was insufficient evidence to warrant treatment, there was also insufficient evidence to safely exclude the presence of cognitive impairment.

Driving With ADHD: Performance Effects and Environment Demand in Traffic.

Objective: This research investigated the on-road driving performance of individuals with ADHD across a range of road and traffic conditions to determine whether errors were linked to situational complexity and attentional demands. Method: The everyday driving performance of medicated drivers with ADHD, unmedicated drivers with ADHD, and controls was tested in urban, residential, rural, and highway environments using driver license testing procedures. Results: Unmedicated drivers with ADHD displayed fewer safe driving skills and committed more inattentive and impatient driving errors, particularly in low demand highway and rural driving conditions. Medicated drivers' performance was not reliably different than controls. Participants in both ADHD groups were more likely than controls to report risky driving and involvement in crashes. Conclusion: The results demonstrate that situations with low attentional demand are particularly risky for unmedicated ADHD drivers and suggest that focus on these situations may be useful in improving driving outcomes for this population.