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PEDIATRICS Vol. 112 No. 6 December 2003, pp. 1580-1583

SUPPLEMENT ARTICLE

Brain Imaging and Proton Magnetic Resonance Spectroscopy in Patients With Phenylketonuria

Harald E. Möller, PhD*, Josef Weglage, MD{ddagger}, Ulrich Bick, MD§, Dirk Wiedermann, PhD||, Reinhold Feldmann, MD{ddagger} and Kurt Ullrich, MD

* Max Planck Institute of Cognitive Neuroscience, Leipzig, Germany
{ddagger} Department of Pediatrics, University of Münster, Münster, Germany
§ Department of Radiology, University of Chicago, Chicago, Illinois
|| Department of Pediatrics, University of Hamburg, Hamburg, Germany
Max Planck Institute of Neurological Research, Köln, Germany

Magnetic resonance imaging studies in patients with phenylketonuria (PKU) revealed white matter alterations that correlated to most recent blood phenylalanine (Phe) concentrations as well as to brain Phe concentrations measured by magnetic resonance spectroscopy. The clinical significance of these changes is unknown. Magnetic resonance imaging data thus have no impact on therapeutic recommendations for adolescents and adults with PKU. Kinetic investigations of patients by magnetic resonance spectroscopy showed differences in brain Phe concentrations despite similar blood Phe levels. These were influenced by interindividual variations of blood-brain barrier Phe transport constants and by variations of the individual brain Phe consumption rate. Blood-brain barrier Phe transport characteristics as well as brain Phe consumption rates thus seem to be causative factors for the individual outcome in PKU.

Key Words: phenylketonuria • brain imaging • proton magnetic resonance spectroscopy

Abbreviations: Phe, phenylalanine, PKU, phenylketonuria • MRI, magnetic resonance imaging • Kt, Michaelis-Menten transport constant • Tmax, maximal transport velocity • Vmet, consumption velocity


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