Pediatric Neurology
Volume 33, Issue 4 , Pages 267-271 , October 2005

School Performance in Early and Continuously Treated Phenylketonuria

  • Rosa Gassió, MD

      Affiliations

    • PKU Follow-up Unit: Neuropediatrics Department, Hospital Sant Joan de Déu, Universitat de Barcelona, Barcelona, Spain.
  • ,
  • Eugenia Fusté, MSC

      Affiliations

    • PKU Follow-up Unit: Psychology Department, Hospital Sant Joan de Déu, Universitat de Barcelona, Barcelona, Spain.
  • ,
  • Anna López-Sala, MSC

      Affiliations

    • PKU Follow-up Unit: Neuropediatrics Department, Hospital Sant Joan de Déu, Universitat de Barcelona, Barcelona, Spain.
  • ,
  • Rafael Artuch, MD, PhD

      Affiliations

    • PKU Follow-up Unit: Biochemistry Department, Hospital Sant Joan de Déu, Universitat de Barcelona, Barcelona, Spain.
  • ,
  • María Antonia Vilaseca, PhD

      Affiliations

    • PKU Follow-up Unit: Biochemistry Department, Hospital Sant Joan de Déu, Universitat de Barcelona, Barcelona, Spain.
  • ,
  • Jaume Campistol, MD, PhD

      Affiliations

    • Corresponding Author InformationCommunications should be addressed to: Dr. Campistol; Neuropediatrics Department; Hospital Sant Joan de Déu; Passeig Sant Joan de Déu no. 2; 08950-Esplugues de Llobregat; Barcelona, Spain.
    • PKU Follow-up Unit: Neuropediatrics Department, Hospital Sant Joan de Déu, Universitat de Barcelona, Barcelona, Spain.

Received 19 September 2004 ,Accepted 9 May 2005.

References 

  1. Scriver CR , Kaufman S . The hyperphenylalaninemias . In:  Scriver CR ,  Beaudet AL ,  Sly WS ,  Valle D editor. The metabolic and molecular bases of inherited disease . 8th ed.. New York: McGraw Hill; 2001;p. 1667–1724
  2. Thompson AJ , Smith I , Brenton D , et al.   Neurological deterioration in young adults with phenylketonuria . Lancet . 1990;336:602–605
  3. Levy HL , Waisbren SE . PKU in adolescents (Rationale and psychosocial factors in diet continuation) . Acta Paediatr . 1994;407:92–97
  4. Smith I , Beasley MG , Ades AE . Effect on intelligence of relaxing the low phenylalanine diet in phenylketonuria . Arch Dis Child . 1991;66:311–316
  5. Diamond A , Prevor MB , Callender G , Druin D . Prefrontal cortex cognitive deficits in children treated early and continuously for PKU . Monogr Soc Res Child Dev . 1997;62: 4, serial no. 252
  6. Dyer CA . Pathophysiology of phenylketonuria . MRDD Research Reviews . 1999;5:104–112
  7. Weglage J , Fünders B , Wilken B , Schubert D , Ullrich K . School performance and intellectual outcome in adolescents with phenylketonuria . Acta Paediatr . 1993;81:582–586
  8. Rey F , Abadie V , Plainguet F , Rey J . Long-term follow up of patients with classical phenylketonuria after diet relaxation at 5 years of age. The Paris Study . Eur J Pediatr . 1996;155(Suppl. 1):S39–S44
  9. Zeman J , Pijackova A , Behulova J , Urge O , Saligova D , Hyanek J . Intellectual and school performance in adolescents with phenylketonuria according to their dietary compliance . Eur J Pediatr . 1996;155(Suppl. 1):S56–S58
  10. Schuler A , Somogyi C , Toros I , et al.   A longitudinal study of phenylketonuria based on the data of the Budapest Screening Center . Eur J Pediatr . 1996;155(Suppl. 1):S50–S52
  11. Chang PN , Gray RM , O’Brien LL . Patterns of academic achievement among patients treated early with phenylketonuria . Eur J Pediatr . 2000;159(Suppl. 2):S96–S99
  12. Tiefenthaler M . Comments on patterns of academic achievement among patients treated early with phenylketonuria . Eur J Pediatr . 2000;159(Suppl. 2):S100–S101
  13. Stemerdink BA , Kalverboer AF , Van der Meere JJ , et al.   Behaviour and school achievement in patients with early and continuously treated phenylketonuria . J Inherit Metab Dis . 2000;23:548–562
  14. Friedman EG , Azen CG , Koch R  Writing Committee for the PKU Collaborative Study . Effects of dietary treatment on children with classical phenylketonuria (PKU): The United States PKU Collaborative Study, 1967-1984. Consensus Development Conference on Phenylketonuria (PKU): Screening and Management . Bethesda, Maryland: National Institutes of Health; 2000; October 16-18
  15. Kaufman AS , Kaufman NL . K-ABC. Batería de evaluación de Kaufman para niños . Madrid: TEA Ediciones; 1997;
  16. Wechsler D . WISC-R. Escala de Inteligencia de Wechsler para niños–Revisada . 9a ed.. Madrid: TEA Ediciones; 2001;
  17. Wechsler D . WAIS-III. Escala de Inteligencia de Wechsler para adultos - III . Madrid: TEA Ediciones; 1999;
  18. Rey A . Test de Copia de una Figura Compleja . 5a ed.. Madrid: TEA Ediciones; 1987;
  19. Talley JL . CAVLT-2. Children’s Auditory Verbal Learning Test 2 . Odessa: PAR; 1993;
  20. Spreen O , Strauss E . Rey Auditory–Verbal Learning Test . In:  Spreen O ,  Strauss E editor. A compendium of neuropsychological tests. Administration, norms, and commentary . New York: Oxford University Press; 1991;p. 149–157
  21. Spreen O , Strauss E . Purdue Pegboard Test . In:  Spreen O ,  Strauss E editor. A compendium of neuropsychological tests. Administration, norms, and commentary . New York: Oxford University Press; 1991;p. 375–383
  22. Spreen O , Strauss E . Finger Tapping Test . In:  Spreen O ,  Strauss E editor. A compendium of neuropsychological tests. Administration, norms, and commentary . New York: Oxford University Press; 1991;p. 360–365
  23. Conners CK . CPT. Conners’ Continuous Performance Test . Toronto, Ontario, Canada: Multi-Health Systems Inc; 1995;
  24. Golden CHJ . STROOP. Test de Colores y Palabras . Madrid: TEA Ediciones; 1994;
  25. Heaton RK , Chelune GJ , Talley JL , Kay GG , Curtis G . WCST. Test de Clasificación de Tarjetas de Wisconsin . Madrid: TEA Ediciones; 1997;
  26. Spreen O , Strauss E . Trail Making Test . In:  Spreen O ,  Strauss E editor. A compendium of neuropsychological tests. Administration, norms, and commentary . New York: Oxford University Press; 1991;p. 321–329
  27. Vilaseca MA , Campistol J , Cambra FJ , Lambruschini N . Index of dietary control of PKU patients . Química Clínica . 1995;14:271
  28. Feldman R , Denecke J , Pietsch M , Grenzebach M , Weglage J . Phenylketonuria (No specific frontal lobe-dependent neuropsychological deficits of early-treated patients in comparison with diabetics) . Pediatr Res . 2002;51:761–765
  29. Huijbregts SCJ , De Sonneville LMJ , Van Spronsen FJ , et al.   Motor function under lower and higher controlled processing demands in early and continuously treated phenylketonuria . Neuropsychology . 2003;17:369–379
  30. Anderson PJ , Wood SJ , Francis DE , et al.   Neuropsychological functioning in children with early-treated phenylketonuria (Impact of white matter abnormalities) . Dev Med Child Neurol . 2004;46:230–238
  31. Gassio R , Campistol J , Vilaseca MA , Lambruschini N , Cambra FJ , Fuste E . Do adult patients with phenylketonuria improve their quality of life after introduction/resumption of a phenylalanine-restricted diet? . Acta Paediatr . 2003;92:1474–1478

PII: S0887-8994(05)00255-9

doi: 10.1016/j.pediatrneurol.2005.05.003

Pediatric Neurology
Volume 33, Issue 4 , Pages 267-271 , October 2005