1043-3155 Pediatr Neurol Briefs pedneurbriefs Pediatr Neurol Briefs Pediatric Neurology Briefs Pediatr Neurol Briefs 2166-6482 1043-3155 2166-3155 Pediatric Neurology Briefs Publishers Chicago, IL, USA PNB-4-34-a 10.15844/pedneurbriefs-4-5-2 Metabolic Disorders Neurology Pediatrics Nervous System Diseases Child Development Brain Diseases Neurosurgery Child Infant New Treatment for Phenylketonuria http://orcid.org/0000-0002-0173-7931 Millichap J. Gordon MD 1 2 * Division of Neurology, Children's Memorial Hospital, Chicago, IL Departments of Pediatrics and Neurology, Northwestern University Feinberg School of Medicine, Chicago, IL Correspondence: Dr. J. Gordon Millichap, E-mail: jgmillichap@northwestern.edu 05 1990 01 08 2016 4 5 34 34 Copyright: © 1990 The Author(s) 1990 This work is licensed under the Creative Commons Attribution 4.0 International License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited. Valine, isoleucine, and leucine. A new treatment for phenylketonuria

Sixteen adolescents and young adults with phenylketonuria were treated with a mixture of valine, isoleucine, and leucine for four 3-month periods, and biochemical and neuropsychological tests were carried out before and after treatment at the Metabolic Disease Center, Children's Hospital Medical Center, University of Cincinnati College of Medicine.

 Phenylketonuria Biochemical and Neuropsychological Tests Significant Improvements

Sixteen adolescents and young adults with phenylketonuria were treated with a mixture of valine, isoleucine, and leucine for four 3-month periods, and biochemical and neuropsychological tests were carried out before and after treatment at the Metabolic Disease Center, Children's Hospital Medical Center, University of Cincinnati College of Medicine. The performance of timed attentional tests and a continuous performance test was improved during the valine, isoleucine, and leucine periods compared to the control mixture periods. The attention diagnostic method, a test with strong attentional components, showed significant improvements. These results were consistent with earlier reports of improvement in specific cognitive processes with valine, isoleucine, and leucine treatment in patients who were unable to maintain low serum phenylalanine levels. [1]

COMMENT. Phenylalanine and other large neutral amino acids share common receptocytes on a blood brain barrier transport system. The administration of other large neutral amino acids to patients with elevated plasma phenylalanine may reduce the amount of phenylalanine reaching the brain and prevent further deficits in cognition. The amino acid mixture consisted of 150 mg/kg Valine, 150 mg/kg Isoleucine, and 200 mg/kg Leucine. This mixture was prescribed as a supplement to the low phenylalanine formula.

 Berry HK Brunner RL Hunt MM White PP Valine, isoleucine, and leucine. A new treatment for phenylketonuria Am J Dis Child 1990 May 144 5 539 543 10.1001/archpedi.1990.02150290033020 2184659