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Prevalence of stimulant use for attentional dysfunction in children with phenylketonuria

G L Arnold1, C J Vladutiu, C C Orlowski

  • 1Department of Pediatrics, University of Rochester School of Medicine and Dentistry, Rochester, NY 14642, USA. Georgianne_Arnold@urmc.rochester.edu

Journal of Inherited Metabolic Disease
|May 26, 2004
PubMed

Insights

Children with phenylketonuria (PKU) often experience attentional dysfunction, with over a quarter using stimulant medication. Higher phenylalanine levels correlate with increased attention problems and stimulant use in PKU patients.

Area of Science:

  • Neuroscience
  • Pediatrics
  • Metabolic Disorders

Background:

  • Phenylketonuria (PKU) is a metabolic disorder requiring dietary management.
  • Poor metabolic control in PKU may be linked to attentional deficits.
  • Limited formal research exists on attentional dysfunction in PKU.

Purpose of the Study:

  • To determine the prevalence of stimulant use for attentional dysfunction in children with PKU.
  • To investigate the relationship between metabolic control (phenylalanine levels) and attentional symptoms in PKU.
  • To compare stimulant use in PKU patients to control groups.

Main Methods:

  • Retrospective review of medical records for 38 school-aged children with early and continuously treated PKU.
  • Comparison of stimulant use prevalence with age- and sex-matched controls (type I diabetes mellitus).
  • Analysis of the correlation between plasma phenylalanine concentrations and stimulant use/attentional symptoms.

Main Results:

  • 26% of PKU children used stimulant medication for attentional dysfunction, significantly higher than controls (6.5%) and population norms (5%).
  • A significant relationship was found between higher phenylalanine levels and stimulant use (792 vs. 486 micromol/L).
  • Higher phenylalanine levels were also associated with reported attentional symptoms (702 vs. 462 micromol/L).

Conclusions:

  • Attentional dysfunction and stimulant use are common in children with PKU.
  • Elevated phenylalanine levels are strongly associated with attentional problems in PKU.
  • Stimulant medications are perceived as effective by parents for treating attentional symptoms in PKU.

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