Social behaviour and task orientation in early‐treated PKU

Abstract

Kalverboer AF, van der Schot LWA, Hendrikx MMH, Huisman J, Slijper FME, Stemerdink BA. Social behaviour and task orientation in early-treated PKU. Acta Pzdiatr 1994;(Suppl 407): 104-5. Stockholm. ISSN 0803-5326In the context of the Dutch Collaborative Study on the Psychological Development of Children, early treated for Phenylketonuria (PKU), data were obtained on children's behaviour as perceived by parents (n = 53) and teachers (n = 57), using standardized behavioural questionnaires. Results are compared with a group representative of Dutch school-aged children (n = 1381) with a matched control group (n = 38), and with a group, to be considered as "Attention Deficit Hyperactivity Disorder" (ADHD, n = 38). Especially the clusters "negative task orientation" and "extraversion" distinguish between PKU children and controls. In parents' descriptions as well as in teachers' descriptions PKU children score higher in both clusters. Intriguing differences are found between PKU and ADHD. In parents' but not in teachers' descriptions PKU children show signs of negative task orientation almost to a similar degree as children with ADHD, but not of the signs of "negative social behaviour", characteristic for ADHD children. Data are discussed in terms of the effectiveness of early treatment and guidance.

Prefrontal Dysfunction in Early and Continuously Treated Phenylketonuria

“…Although generally within the normal range (Brunner, Berch, & Berry, 1987;Huisman, Slijper, Hendrikx, Kalverboer, & van der Schot, 1985;Pennington, van Doorninck, McCabe, & McCabe, 1985), their intelligence scores are often significantly lower than the means of their family members and the mean expected in the general population (Medical Research Council Working Party on Phenylketonuria, 1993). In addition, an increased prevalence of behavior problems (Kalverboer et al, 1994;I. Smith, Beasley, Wolff, & Ades, 1988), learning disabilities (Koch, Azen, Hurst, Gross Friedman, & Fishler, 1987;Saudubray et al, 1987), attention problems (Anderson, Siegel, Fisch, & Wirt, 1969;Clarke, 30 STEMERDINK ET AL. Gates, Hogan, Barrett, & MacDonald, 1987;Craft, Gourovitch, Dowton, Swanson, & Bonforte, 1992;Lou, Lykkelund, Gerdes, Udesen, & Bruhn, 1987; de Sonneville, Schmidt, & Michel, 1989; de Sonneville, Schmidt, Michel, & Batzler, 1990), and cognitive deficits (Brunner et al, 1987;Brunner, Jordan, & Berry, 1983;Clarke et al, 1987;Seashore, Friedman, Novelly, & Bapat, 1985) was noted in these patients.…”

Prevalence of stimulant use for attentional dysfunction in children with phenylketonuria

“…Parents of the stimulant-using children felt that the stimulants were e⁄cacious in treating their child's attentional symptoms. Stimulant use and parent reports of attentional dysfunction are quite common in our PKU patients and appear to be strongly related to higher phenylalanine concentrations.Preliminary evidence suggests that children with phenylketonuria (PKU), McKusick 261600) may have an increased prevalence of attentional dysfunction (Kalverboer et al 1994;Smith and Knowles 2000). In addition, numerous studies have identi¢ed de¢cits in 'sustained attention' (working memory) in PKU-a¡ected individuals, particularly when phenylalanine levels are outside of the suggested 'treatment' range (Diamond et al…”

Retrospective neuropsychological evaluation of 18 Romanian subjects with hyperphenylalaninaemia

“…The general intelligence (IQ) score ranges and interpretation were calculated according to the classi cation of intellectual level by Swaiman et al (3). The results of this study are presented in Table 1 and suggest that: (a) a very precocious and correctly maintained treatment (< 400 m mol/l Phe) is essential for a normal neuropsychological development of PKU subjects, as has already been shown (4,5), despite the genotype severity with one exception-the genotyped non-PKU HPA patients, who do not require treatment; (b) less severe genotype could positively in uence intellectual performance under conditions of low compliance with the Phe regimen; (c) a condition for the success of this treatment, at least during early childhood, is the very good family cooperation. The society and the multidisciplinary team advising the family are also determinant factors.…”