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PEDIATRICS Vol. 112 No. 6 December 2003, pp. 1575-1579

SUPPLEMENT ARTICLE

Brain Phenylalanine Concentrations in Phenylketonuria: Research and Treatment of Adults

Rex A. Moats, PhD*, Kathryn D. Moseley, MS, RD{ddagger}, Richard Koch, MD{ddagger} and Marvin Nelson, Jr, MD*

* Department of Radiology
{ddagger} Division of Medical Genetics, Department of Pediatrics, University of California/Keck School of Medicine at Childrens Hospital Los Angeles, Los Angeles, California

Objective. To assess the effects of 2 pharmacologic interventions (amino acid supplements) on the brain levels of phenylalanine (Phe) in adults with phenylketonuria (PKU).

Methods. A prospective study was conducted in an outpatient treatment and follow-up setting. The volunteers who were recruited for the first intervention included 4 subjects with classic PKU. The second intervention included 3 adults with classic PKU. The first intervention consisted of dietary supplementation during 1 day with Phlexy 10. Two individuals were given a dose of 0.5 g/kg/d, and 2 were given 1.0 g/kg/d. The second intervention consisted of dietary supplementation with PreKUnil at 0.4 g kg/d over a period of 6 months. Brain Phe was measured by magnetic resonance spectroscopy. The number of the patients involved precluded analysis for significance.

Results. The first, shorter intervention resulted in a decrease in brain Phe. The second intervention resulted in a 20% decrease in brain Phe, which was maintained after 6 months of treatment.

Conclusion. Dietary supplementation of large neutral amino acids seems to lower the brain Phe in adults who have PKU and have difficulty following their diet.

Key Words: magnetic resonance spectroscopy • blood/brain barrier • large neutral amino acids

Abbreviations: PKU, phenylketonuria • Phe, phenylalanine • MRS, magnetic resonance spectroscopy • LNAA, large neutral amino acid • MRI, magnetic resonance imaging


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