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Psychological and social findings in adolescents with phenylketonuria

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Abstract

In a retrospective study, 34 early treated, normally intelligent adolescents with phenylketonuria (PKU) and their parents were tested with several psychometric personality inventories and self-developed questionaires concerning their psychosocial situation and their disease-and diet-specific knowledge. Results show that the patients are characterized by less autonomy, a more negative evaluation of their scholastic ability, less achievement motivation, less extraversion and impulsiveness, a feeling of not being quite healthy, more grave and a higher level of dependency from their families. The patients saw their whole social situation as being destinctly restricted. Their knowledge concerning disease and diet was alarmingly poor and the majority had great difficulties in managing the diet satisfactorily without parental help. Up to the age of 15 years the serum phenylalanine levels were persistently above the desired range.

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Abbreviations

IQ:

intelligence quotient

Phe:

phenylalanine

PKU:

phenylketonuria

References

  1. Acosta PB, Trahms C, Wellmann NS, Williamson N (1983) Phenylalanine intakes of 1-to-6-years old children with phenylketonuria undergoing therapy. Am J Clin Nutr 38:694–700

    PubMed  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. Blascovis ME, Scheffler GE, Hack S (1974) Phenylalaninemia. Differential diagnosis. Arch Dis Child 49:835–843

    PubMed  Google Scholar 

  4. Brunner R, Berry H (1987) Phenylketonuria and sustained attention: The continous performance test. Int J Clin Neuropsych 9:68–70

    Google Scholar 

  5. Dobson JC, Williamson ML, Azen C, Koch R (1977) Intellectual assessment of 111 four year old children with phenylketonuria. J Pediatr 5:53–56

    Google Scholar 

  6. Fahrenberg J, Hampel R, Selg H (1983) Das Freiburger Persönlichkeitsinventar (FPI-R), Verlag für Psychologie Der CH Hogrefe, Göttingen

    Google Scholar 

  7. German Paediatric Society (1990) Limits in serum phenylalanine concentrations of different ages. Convention of the “Deutsche Arbeitsgemeinschaft für Pädiatrische Stoffwechselerkrankungen”. Monatschr Kinderheilk 138:636

    Google Scholar 

  8. Holtzman N, Richard M, Kornmal A (1986) Effect of age at loss of dietary control on intellectual performance and behavior of children with phenylketonuria. N Engl J Med 314:593–598

    PubMed  Google Scholar 

  9. Jäger R, Berbig E, Geisel B, Gosslar H, Hagen J, Liebich W, Schafheutle R (1973) Mannheimer Biographisches Inventar (MBI), Verlag für Psychologie, Dr. H.J. Hogrefe, Göttingen

    Google Scholar 

  10. Petermann F, Noeker M, Bode U (1987) Psychologie chronischer Krankheiten im Kindes- und Jugendalter. Psychologie Verlags-Union, München-Weinheim

    Google Scholar 

  11. Realmuto G, Garfinkel BD, Tuchmann M, Tsai MT, Chang PN, Fisch RO, Sharpio S (1986) Psychiatric diagnosis and behavioral characteristics of phenylketonuric children. J Nerv Ment Dis 174:536–540

    PubMed  Google Scholar 

  12. Reber M, Kazak AE, Himmelberg P (1987) Phenylalanine control and family functoning in early treated phenylketonuria. Dev Behav Pediatr 8:311–317

    Google Scholar 

  13. Schmidt H, Mahle M, Michel U, Pietz J (1987) Continuation vs discontinuation of low-phenylalanine diet in PKU adolescents. Eur J Pediatr 146 [Suppl 1]:A12-A16

    Article  PubMed  Google Scholar 

  14. Seitz W, Rausche A (1976) Persönlichkeitsfragebogen für Kinder (PFK 9-14). Westermann-Verlag Braunschweig

    Google Scholar 

  15. Smith I, Beasly MG, Wolff OH, Ades AE (1988) Behaviour disturbances in 8-year-old children with early treated phenylketonuria. J Pediatr 112:408

    Google Scholar 

  16. Sonneville LMJ de, Schmidt E, Michel U, Batzler U (1990) Preliminary neuropsychological test results. Eur J Pediatr 149 [Suppl 1]:A39-A44

    Google Scholar 

  17. Tanner JM (1962) Wachstum und Reifung des Menschen. Thieme-Verlag, Stuttgart

    Google Scholar 

  18. Weiß RH (1987) Grundintelligenztest Skala 2 CFT 20. Verlag für Psychologie, DR CJ Hogrefe Göttingen

    Google Scholar 

  19. Wendel U, Ullrich K, Schmidt H, Batzler U (1990) Six-year follow up of phenylalanine intakes and plasma phenylalanine concentrations. Eur J Pediatr 149 [Suppl 1]:13–16

    Google Scholar 

  20. Zwiauer K, Widhalm S, Scheibenreiter S, Knoll E (1990) Longterm intellectual development in 86 children with phenylketonuria (PKU) Pediatr Res 28:74

    Google Scholar 

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Weglage, J., Fünders, B., Wilken, B. et al. Psychological and social findings in adolescents with phenylketonuria. Eur J Pediatr 151, 522–525 (1992). https://doi.org/10.1007/BF01957759

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  • DOI: https://doi.org/10.1007/BF01957759

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