Preliminary support for the oral administration of valine, isoleucine and leucine for phenylketonuria.

Abstract:

:Recent behavioral data have demonstrated the importance of maintaining low phenylalanine concentrations beyond early childhood in patients with phenylketonuria, which can be a difficult task, particularly during adolescence. Administration of certain large neutral amino-acids (valine, isoleucine, leucine--VIL) appears to reduce phenylalanine concentrations in the cerebrospinal fluid of humans and in the brain of rats. The present study compared neuropsychological test-performance of six patients with phenylketonuria during periods of VIL administration and periods when this supplement was not given. Although individual responses to VIL were variable, there was an over-all improvement of about 1 1/2 SD in neuropsychological test performance during VIL treatment. Abstract reasoning and tactile motor problem-solving increased more than pure motor performance.

journal_name

Dev Med Child Neurol

authors

Jordan MK,Brunner RL,Hunt MM,Berry HK

doi

10.1111/j.1469-8749.1985.tb04522.x

subject

Has Abstract

pub_date

1985-02-01 00:00:00

pages

33-9

issue

1

eissn

0012-1622

issn

1469-8749

journal_volume

27

pub_type

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