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Phenylketonuria and some aspects of emotional development

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Abstract

Early dietary treatment of phenylketonuria (PKU) prevents intellectual retardation and gross neurological impairment although not all neuropsychological problems. This study investigates to what extent the illness and its treatment imposes a burden on emotional development of early-treated PKU patients and on rearing practices of their parents. It is concluded that in early-treated PKU it is particularly difficult to find constructive ways to adapt to the situation for children as well as for parents. As coping with PKU and its treatment is so complicated, paediatric control should be combined with psychocounselling.

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Abbreviations

CBCL-p :

child behaviour check list, parents form

DM :

diabetes mellitus

DQC :

depression questionnaire for children

EvIl :

evaluation of illness

HYP −p :

hyperactivity score on CBCL-p

IMP :

impulsivity score on MFFT

IN :

inadequacy score on DPQ-C (selfesteem)

PKU :

phenylketonuria

Restr :

restrictiveness score on CRPR

SI :

social inadequacy score on DPQ-C

References

  1. Achenbach TM, Edelbrock C (1983) Manual for the child behavior check list and revised behavior profile. University of Vermont, Burlington

    Google Scholar 

  2. Awiszus D, Unger I (1990) Coping with PKU: results of narrative interviews with parents. Eur J Pediatr 149 [Suppl 1]:45–51

    Google Scholar 

  3. Block JH, Block J (1980) The role of ego-control and ego-resilience in the organization of behavior. In: WA Collins (Ed) The Minnesota Symposia on Child Psychology 13: 39–101

  4. Dekovic M (1989) Measuring dimensions and patterns of childrearing: dutch version of the block child rearing practices report (CRPR). Catholic University Nijmegen

  5. Fishler K, Azen CG, Henderson R, Friedman EG, Koch R (1987) Psychoedu- cational findings among children treated for phenylketonuria. Am J Ment Defic 92: 65–73

    PubMed  Google Scholar 

  6. Huisman J, Schot LWA van der, Hendrixk, MMT, Kalverboer AF, Slijper FME (1992) The interlectual development of patients with phenylketonuria; 15 years screening in the Netherlands (in Dutch). Ned Tijdschr Geneeskd 136, 46: 2277–2281

    PubMed  Google Scholar 

  7. Kagan J, Rosman BL, Day D, Albert J, Philips W (1964) Information processing in the child: Significance of analytic and reflective attitudes. Psychol Monogr; 78

  8. Koch R, Azen C, Friedman EG, Williamson ML (1984) Paired comparisons between early-treated PKU children and their matched sibling controls on intelligence and school achievement test results at eight years of age. J Inherited Metab Dis 7: 86–90

    Article  PubMed  Google Scholar 

  9. Koff E, Boyle P, Sigfried M, Pueschel SM (1977) Perceptual-motor functioning in children with phenylketonuria. Am J Dis Child 131: 1084–1087

    PubMed  Google Scholar 

  10. Luteijn F, Dijk H van, Ploeg FAE van der (1989) of the Dutch personality questionnaire for children (in Dutch). Swets and Zeitlinger, Lisse, the Netherlands

    Google Scholar 

  11. Primrose DA (1983) Phenylketonuria with normal intelligence. J Ment Defic Res 27: 239–246

    PubMed  Google Scholar 

  12. Realmuto GM, Garfinkel BD, Tuchman M, Tsai MY, Chang PN, Fisch RO, Shapiro S (1986) Psychiatric diagnosis and behavioral characteristics of phenylketonuric children. J Nerv Ment Dis 174(9): 536–540

    PubMed  Google Scholar 

  13. Shulman S, Fisch RO, Zempel CE, Gadish O, Chang PN (1991) Children with phenylketonuria: the interface of family and child functioning. Dev Behav Pediatr 12(5): 315–321

    Google Scholar 

  14. Smith I, Beasley MG, Wolff OH, Ades AE (1988) Behavior disturbance in 8 year old children with early treated phenylketonuria. J Pediatr 112(3): 403–408

    PubMed  Google Scholar 

  15. Sonneville LMJ de, Schmidt E, Michel U, Batzler U (1990) Preliminary neuropsychological test results. Eur J Pediatr 149: S39–44

    Google Scholar 

  16. Stevenson JE, Hawcroft J, Lobascher M, Smith I, Wolff O, Graham PJ (1979) Behavioral deviance in children with early treated phenylketonu-ria. Arch Dis Child 54: 14–18

    PubMed  Google Scholar 

  17. Taylor EA (1986) The overactive child. Clinics in developmental medicine 97. Blackwell, Oxford

    Google Scholar 

  18. Verhulst FC, Akkerhuis GW, Althaus M (1985) Mental health in Dutch children: (I) a cross-cultural comparison. Acta Psychiatr Scand 72 [Suppl 323]

  19. Waisbren SE, Mahon BE, Schnell RR, Levy HL (1987) Predictors of intelligence quotient and intelligence quotient change in persons treated for phenylketonuria early in life. Pediatrics 79(3): 351–355

    PubMed  Google Scholar 

  20. Welsh MC, Pennington BF, Ozonoff S, Rouse B, McCabe ERB (1990) Neuropsychology of early-treated phenylketonuria: specific executive function deficits. Child Dev 61: 1697–1713

    PubMed  Google Scholar 

  21. Wit CAM de (1987) Depression questionnaire for children (in Dutch) (DVK). Acco, Amersfoort

    Google Scholar 

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Authors and Affiliations

  1. Department of Child and Adolescent Psychiatry, Academic Medical Centre, Tafelbergweg 25, NL-1105 BC, Amsterdam, The Netherlands

    M. M. T. Hendrikx

  2. Department of Medical Psychology, Academic Hospital, Nijmegen, The Netherlands

    L. W. A. van der Schot

  3. Department of Child and Adolescent Psychiatry, Sophia Children Hospital, Rotterdam, The Netherlands

    F. M. E. Slijper

  4. Department of Medical Psychology, Free University Hospital, Amsterdam, The Netherlands

    J. Huisman

  5. Laboratory for Experimental Clinical Psychology, State University Groningen, The Netherlands

    A. F. Kalverboer

Authors
  1. M. M. T. Hendrikx
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  2. L. W. A. van der Schot
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  3. F. M. E. Slijper
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  4. J. Huisman
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  5. A. F. Kalverboer
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Hendrikx, M.M.T., van der Schot, L.W.A., Slijper, F.M.E. et al. Phenylketonuria and some aspects of emotional development. Eur J Pediatr 153, 832–835 (1994). https://doi.org/10.1007/BF01972893

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  • DOI: https://doi.org/10.1007/BF01972893

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