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Executive function and psychosocial adjustment in children with early treated phenylketonuria: correlation with historical and concurrent phenylalanine levels
Author(s) -
Griffiths P.,
Tarrini M.,
Robinson P.
Publication year - 1997
Publication title -
journal of intellectual disability research
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.941
H-Index - 104
eISSN - 1365-2788
pISSN - 0964-2633
DOI - 10.1111/j.1365-2788.1997.tb00715.x
Subject(s) - psychosocial , psychology , executive functions , neuropsychology , executive dysfunction , population , pediatrics , phenylketonurias , phenylalanine , personality , clinical psychology , developmental psychology , psychiatry , cognition , medicine , social psychology , biochemistry , chemistry , environmental health , amino acid
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 μmol 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, nonexecutive 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 μmol 1 − (SD =± 144) for the pre‐school period and 480 μmol 1 − (SD ± 193) for the primary school period corresponded to the upper limits presently recommended in the UK of 360 and 480 μmoll − 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.