A preliminary investigation of the role of the Phenylalynine:Tyrosine ratio in children with early and continuously treated Phenylketonuria: Toward identification of "Safe" levels

Developmental Neuropsychology

Volumn 35, Issue 1, 2009, Pages 57-65

  Sharman, Rachael ^a   Sullivan, Karen ^a   Young, Ross ^a   Mcgill, Jim ^b  

^a QUEENSLAND UNIVERSITY OF TECHNOLOGY   (Australia)

^b ROYAL CHILDREN'S HOSPITAL   (Australia)

Author keywords

[No Author keywords available]

Indexed keywords

EID: 77951045995     PISSN: 87565641     EISSN: None     Source Type: Journal    
DOI: 10.1080/87565640903325725     Document Type: Article

References (18)

1. Antshel, K. M.,&Waisbren, S. E. (2003). Timing is everything: Executive functions in children exposed to elevated levels of phenylalanine. Neuropsychology, 17(3), 458-468.
2. Arnold, G. L., Vladutiu, C. J., Orlowski, C. C., Blakely, E. M., & DeLuca, J. (2004.) Prevalence of stimulant use for attentional dysfunction in children with phenylketonuria. Journal of Inherited Metabolic Disease, 27(2), 137-143.
3. Australian Society for Inborn Errors of Metabolism. (2005). The PKU handbook. Alexandra, Victoria: Human Genetic Society of Australia.
4. DeRoche, K., & Welsh, M. (2008). Twenty-five years of research on neurocognitive outcomes in early-treated phenylketonuria: Intelligence and executive function. Developmental Neuropsychology, 33(4), 474-504.
5. Diamond, A., Prevor, M. B., Callender, G., & Druin, D. P. (1997). Prefrontal cortex cognitive deficits in children treated early and continuously for PKU. Monographs of the Society for Research in Child Development, 62(4), i-iv.
6. Gioia, G. A., Isquith, P. K., Guy, S. C., & Kenworthy, L. (2000). Behaviour rating inventory of executive function, professional manual. Lutz, FL: Psychological Assessment Resources, Inc.
7. Huijbregts, S. C., de Sonneville L. M., van Spronsen, F. J., Licht, R., & Sergeant, J. A. (2002). The neuropsychological profile of early and continuously treated phenylketonuria: Orienting, vigilance, and maintenance versus manipulation-functions of working memory. Neuroscience and Biobehavioural Reviews, 26(6), 697-712.
8. Huijbregts, S. C., de Sonneville, L. M., Licht, R., van Spronsen, F. J., Verkerk, P. H., & Sergeant, J. (2002). Sustained attention and inhibition of cognitive interference in treated phenylketonuria: Associations with concurrent and lifetime phenylalanine concentrations. Neuropsychologia, 40(1), 7-15.
9. Koch, R., Moats, R., Guttler, F., Guldberg, P., & Nelson, M., Jr. (2000). Blood brain phenylalanine relationships in persons with phenylketonuria. Pediatrics, 106(5), 1093-1096.
10. Landvogt, C., Mengel, E., Bartenstein, P., Buchholz, H. G., Schrekenberger, M., Siessmeier, T., et al. (2007). Reduced cerebral fluoro-L-dopamine uptake in adult patients suffering from phenylketonuria. Journal of Cerebral Blood Flow and Metabolism, 28(4), 824-831.
11. Luciana, M., Sullivan, J.,&Nelson, C. A. (2001). Associations between phenylalanine-to-tyrosine ratios and performance on tests of neuropsychological function in adolescents treated early and continuously for phenylketonuria. Child Development, 72(6), 1637-1653.
12. Ormazabal, A., Artuch, R., Vilaseca, M. A., Garcia-Cazorla, A., & Campistol, J. (2004). Pathogenetic mechanisms in phenylketonuria: Disorders affecting the metabolism of neurotransmitters and the antioxidant system. Revista de Neurologia, 39(10), 956-961.
13. Pitt, D., & Danks, D. (1991). The natural history of untreated phenylketonuria over 20 years. Journal of Pediatric Child Health, 27(3), 189-190.
14. Prince, J. (2008). Catecholamine dysfunction in attention-deficit/ hyperactivity disorder: An update. Journal of Clinical Psychopharmacology, 28, S39-45.
15. Scriver, C. R., & Sly, W. S. (eds.) (2000). The metabolic and molecular bases of metabolic disease, eighth ed. New York: McGraw-Hill Professional.
16. Sharman, R., Sullivan, K., Young, R.,&McGill, J. (2009). Biochemical markers associated with executive function in adolescents with early and continuously treated phenylketonuria. Clinical Genetics, 75(2), 169-174.
17. Stallar, J. A., & Faraone, S. V. (2007). Targeting the dopamine system in the treatment of attention-deficit/hyperactivity disorder. Expert Review of Neurotherapuetics, 7(4), 351-362.
18. Waisbren, S. E., Noel, K., Fahrbach, K., Cella, C., Frame, D., Dorenbaum, A., et al. (2007). Phenylalanine blood levels and clinical outcomes in phenylketonuria: A systematic literature review and meta-analysis. Molecular Genetics and Metabolism, 92, 63-70.