Preliminary support for the oral administration of valine, isoleucine and leucine for phenylketonuria / Mary Kay JORDAN in Developmental Medicine & Child Neurology, 27-1 (February 1985)

Public ISBD [article]  inDevelopmental Medicine & Child Neurology > 27-1 (February 1985) . - p.33-39 Titre : Preliminary support for the oral administration of valine, isoleucine and leucine for phenylketonuria Type de document : Texte imprimé et/ou numérique Auteurs : Mary Kay JORDAN, Auteur ; Helen K. BERRY, Auteur ; Robert L. BRUNNER, Auteur ; Melanie M. HUNT, Auteur Année de publication : 1985 Article en page(s) : p.33-39 Langues : Anglais (eng) Index. décimale : PER Périodiques Résumé : 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. Permalink : https://www.cra-rhone-alpes.org/cid/opac_css/index.php?lvl=notice_display&id=586 [article] Preliminary support for the oral administration of valine, isoleucine and leucine for phenylketonuria [Texte imprimé et/ou numérique] / Mary Kay JORDAN, Auteur ; Helen K. BERRY, Auteur ; Robert L. BRUNNER, Auteur ; Melanie M. HUNT, Auteur . - 1985 . - p.33-39. Langues : Anglais (eng) in Developmental Medicine & Child Neurology > 27-1 (February 1985) . - p.33-39 Index. décimale : PER Périodiques Résumé : 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. Permalink : https://www.cra-rhone-alpes.org/cid/opac_css/index.php?lvl=notice_display&id=586

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