Executive function and psychosocial adjustment in children with early treated phenylketonuria: correlation with historical and concurrent phenylalanine levels

Author(s): Tarrini, M., Robinson, P.

Journal/Book: J Intellect Disabil Res. 1997; 41: Osney Mead, Oxford, Oxon, England OX2 0EL. Blackwell Science Ltd. 317-323.

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 mu mol l(-1) may result in specific impairment of executive functions such as attention, planning and set maintenance. The executive dysfunction theory was investigated by correlating 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 mu mol l(-1) (SD = +/-144) for the pre-school period and 480 mu mol l(-1) (SD +/- 193) for the primary school period corresponded to the upper limits presently recommended in the UK of 360 and 480 mu mol 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.

Note: Article Griffiths P, Univ Stirling, Dept Psychol, Stirling FK9 4LA, SCOTLAND

Keyword(s): phenylketonuria; cognition; psychosocial adjustment; DESIGN FLUENCY; YOUNG-ADULTS; DEFICITS

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