QUT ePrints

Tyrosine monitoring in children with early and continuously treated phenylketonuria: Results of an international practice survey

Sharman, Rachael R., Sullivan, Karen A., Young, Ross McD., & McGill, James J. (2010) Tyrosine monitoring in children with early and continuously treated phenylketonuria: Results of an international practice survey. Journal of Inherited Metabolic Disease.

[img] Paper (PDF 49kB)
Accepted Version.
    [img] Figure (PDF 35kB)
    Accepted Version.

      View at publisher


      Investigations into the biochemical markers associated with executive function (EF) impairment in children with early and continuously treated phenylketonuria (ECT-PKU) remain largely phenylalanine-only focused, despite experimental data showing that a high phenylalanine:tyrosine (phe:tyr) ratio is more strongly associated with EF deficit than phe alone. A high phe:tyr ratio is hypothesized to lead to a reduction in dopamine synthesis within the brain, which in turn results in the development of EF impairment. This paper provides a snapshot of current practice in the monitoring and/or treatment of tyrosine levels in children with PKU, across 12 countries from Australasia, North America and Europe. Tyrosine monitoring in this population has increased over the last 5 years, with over 80% of clinics surveyed reporting routine monitoring of tyrosine levels in infancy alongside phe levels. Twenty-five percent of clinics surveyed reported actively treating/managing tyrosine levels (with supplemental tyrosine above that contained in PKU formulas) to ensure tyrosine levels remain within normal ranges. Anecdotally, supplemental tyrosine has been reported to ameliorate symptoms of both attention deficit hyperactivity disorder and depression in this population. EF assessment of children with ECT-PKU was likewise highly variable, with 50% of clinics surveyed reporting routine assessments of intellectual function. However when function was assessed, test instruments chosen tended towards global measures of IQ prior to school entry, rather than specific assessment of EF development. Further investigation of the role of tyrosine and its relationship with phe and EF development is needed to establish whether routine tyrosine monitoring and increased supplementation is recommended.

      Impact and interest:

      6 citations in Scopus
      Search Google Scholar™
      1 citations in Web of Science®

      Citation countsare sourced monthly from Scopus and Web of Science® citation databases.

      These databases contain citations from different subsets of available publications and different time periods and thus the citation count from each is usually different. Some works are not in either database and no count is displayed. Scopus includes citations from articles published in 1996 onwards, and Web of Science® generally from 1980 onwards.

      Citations counts from the Google Scholar™ indexing service can be viewed at the linked Google Scholar™ search.

      Full-text downloads:

      338 since deposited on 20 Jan 2011
      120 in the past twelve months

      Full-text downloadsdisplays the total number of times this work’s files (e.g., a PDF) have been downloaded from QUT ePrints as well as the number of downloads in the previous 365 days. The count includes downloads for all files if a work has more than one.

      ID Code: 39598
      Item Type: Journal Article
      Keywords: Phenylketonuria, Executive Function, Phenylalanine, Tyrosine, Dopamine
      DOI: 10.1007/s10545-010-9211-6
      ISSN: 0141-8955
      Subjects: Australian and New Zealand Standard Research Classification > MEDICAL AND HEALTH SCIENCES (110000) > CLINICAL SCIENCES (110300)
      Australian and New Zealand Standard Research Classification > PSYCHOLOGY AND COGNITIVE SCIENCES (170000) > PSYCHOLOGY (170100) > Biological Psychology (Neuropsychology Psychopharmacology Physiological Psychology) (170101)
      Divisions: Current > QUT Faculties and Divisions > Faculty of Health
      Current > Institutes > Institute of Health and Biomedical Innovation
      Current > Schools > School of Psychology & Counselling
      Copyright Owner: Copyright 2010 SSIEM and Springer
      Copyright Statement: The final publication is available at www.springerlink.com
      Deposited On: 21 Jan 2011 09:59
      Last Modified: 01 Mar 2012 00:21

      Export: EndNote | Dublin Core | BibTeX

      Repository Staff Only: item control page