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Tyrosine monitoring in children with early and continuously treated phenylketonuria: results of an international practice survey.
- Source :
-
Journal of inherited metabolic disease [J Inherit Metab Dis] 2010 Dec; Vol. 33 Suppl 3, pp. S417-20. Date of Electronic Publication: 2010 Sep 30. - Publication Year :
- 2010
-
Abstract
- 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.
- Subjects :
- Adolescent
Australasia
Biomarkers metabolism
Child
Child, Preschool
Cognition
Europe
Executive Function
Health Care Surveys
Humans
Infant
Infant, Newborn
Intelligence Tests
Neuropsychological Tests
North America
Phenylalanine blood
Phenylketonurias diagnosis
Phenylketonurias metabolism
Phenylketonurias psychology
Predictive Value of Tests
Surveys and Questionnaires
Time Factors
Treatment Outcome
Tyrosine adverse effects
Dietary Supplements adverse effects
Phenylketonurias blood
Phenylketonurias therapy
Practice Patterns, Physicians'
Tyrosine blood
Tyrosine therapeutic use
Subjects
Details
- Language :
- English
- ISSN :
- 1573-2665
- Volume :
- 33 Suppl 3
- Database :
- MEDLINE
- Journal :
- Journal of inherited metabolic disease
- Publication Type :
- Academic Journal
- Accession number :
- 20882350
- Full Text :
- https://doi.org/10.1007/s10545-010-9211-6