Long-Term Follow-Up of Cognition and Mental Health in Adult Phenylketonuria: A PKU-COBESO Study
SourceBehavior Genetics, 47, 5, (2017), pp. 486-497
Article / Letter to editor
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Paediatrics - OUD tm 2017
SubjectRadboudumc 6: Metabolic Disorders RIMLS: Radboud Institute for Molecular Life Sciences
Cognitive and mental health problems in individuals with the inherited metabolic disorder phenylketonuria (PKU) have often been associated with metabolic control and its history. For the present study executive functioning (EF) was assessed in 21 PKU patients during childhood (T1, mean age 10.4 years, SD = 2.0) and again in adulthood (T2, mean age 25.8 years, SD = 2.3). At T2 additional assessments of EF in daily life and mental health were performed. Childhood (i.e. 0-12 years) blood phenylalanine was significantly related to cognitive flexibility, executive motor control, EF in daily life and mental health in adulthood (i.e. at T2). Patients with a greater increase in phenylalanine levels after the age of 12 performed more poorly on EF-tasks at T2. Group-based analyses showed that patients with phenylalanine <360 micromol/L in childhood and phenylalanine >/=360 micromol/L from age 13 onwards (n = 11) had better cognitive flexibility and executive motor control than those who had phenylalanine >/=360 micromol/L throughout life (n = 7), supporting the notion that phenylalanine should be below the recommended upper treatment target of 360 micromol/L during childhood for better outcome in adulthood. Despite some results indicating additional influence of phenylalanine levels between 13 and 17 years of age, evidence for a continued influence of phenylalanine levels after childhood on adult outcomes was largely lacking. This may be explained by the fact that the patients in the present study had relatively low phenylalanine levels during childhood (mean: 330 micromol/L, range: 219-581 micromol/L) and thereafter (mean Index of Dietary Control at T2: 464 micromol/L, range: 276-743 micromol/L), which may have buffered against transitory periods of poor metabolic control during adolescence and early adulthood.
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