title: Executive function and psychosocial adjustment in children with early treated phenylketonuria: correlation with historical and concurrent phenylalanine levels.
abstract: The issue of what level of phenylalanine (phe) constitutes a safe upper limit for the therapeutic range in dietary treatment of phenylketonuria (PKU) remains unsettled. It has been proposed that the previous guideline figure of 600 mumol l-1 may result in specific impairment of executive functions such as attention, planning and set maintenance. The executive dysfunction theory was investigated by correlation historical and concurrent phe with executive, non-executive and personality tests in a group of early and continuously treated children, aged 10-13 years, with classical PKU, whose average phe levels of 355 mumol l-1 (SD = +/-144) for the pre-school period and 480 mumol l-1 (SD +/-193) for the primary school period corresponded to the upper limits presently recommended in the UK of 360 and 480 mumol l-1 for these age ranges. No clear associations were found between historical or concurrent phe levels and any of the neuropsychological or personality measures, thereby weakening the case for the emergence of executive deficits, at least when average phe levels remain close to the upper limits now considered safe. Furthermore no significant differences were found on psychological variables where comparison with population norms was possible.
authors.affiliation: Department of Psychology, University of Stirling, Scotland.
authors.forename: P
authors.initials: P
authors.lastname: Griffiths
authors.forename: M
authors.initials: M
authors.lastname: Tarrini
authors.forename: P
authors.initials: P
authors.lastname: Robinson
mesh_terms: D000275:Adjustment Disorders / Q000150:complications* / Q000175:diagnosis; D002648:Child; D002675:Child, Preschool; D003072:Cognition Disorders / Q000150:complications / Q000175:diagnosis; D005260:Female; D006801:Humans; D007223:Infant; D008297:Male; D009483:Neuropsychological Tests; D010649:Phenylalanine / Q000097:blood*; D010661:Phenylketonurias / Q000150:complications* / Q000523:psychology*
chemical_list: D010649:Phenylalanine