Intellectual development and academic achievement of children treated early for phenylketonuria / Helen K. BERRY in Developmental Medicine & Child Neurology, 21-3 (June 1979)

Public ISBD [article]  inDevelopmental Medicine & Child Neurology > 21-3 (June 1979) . - p.311-320 Titre : Intellectual development and academic achievement of children treated early for phenylketonuria Type de document : Texte imprimé et/ou numérique Auteurs : Helen K. BERRY, Auteur ; Donald J. O'GRADY, Auteur ; Lorraine J. PERLMUTTER, Auteur ; Mary K. BOFINGER, Auteur Année de publication : 1979 Article en page(s) : p.311-320 Langues : Anglais (eng) Index. décimale : PER Périodiques Résumé : Twenty early-treated children with classical phenylketonuria (PKU), five early-treated children with variant PKU and seven untreated children with hyperphenylalinemia were compared with non-PKU family members in terms of intellectual development, and 14 school-age PKU children were also compared for academic achievement. For the early-treated children with classical PKU, mean IQ (98) was within the normal range, but nine of these 20 children had IQ scores more than 1SD below those of family members. There was a significant negative correlation between phenylalanine concentrations at one to four years of age and later measured intelligence in these early-treated children, but this was probably a consequence of poor dietary control in the early years. The early-treated children with variant PKU and those with hyperphenylalaninemia had IQ scores consistent with those of unaffected family members, but untreated children with variant PKU had scores significantly lower than their own early-treated siblings. Achievement scores of the early-treated PKU children were consistent with their intellectual ability: they and their non-PKU siblings had similar standard scores for reading and spelling, but arithmetic scores were significantly lower for the PKU children. Early-treated children whose diet had been discontinued had achievement scores in all subjects below those predicted from their IQS. Permalink : https://www.cra-rhone-alpes.org/cid/opac_css/index.php?lvl=notice_display&id=496 [article] Intellectual development and academic achievement of children treated early for phenylketonuria [Texte imprimé et/ou numérique] / Helen K. BERRY, Auteur ; Donald J. O'GRADY, Auteur ; Lorraine J. PERLMUTTER, Auteur ; Mary K. BOFINGER, Auteur . - 1979 . - p.311-320. Langues : Anglais (eng) in Developmental Medicine & Child Neurology > 21-3 (June 1979) . - p.311-320 Index. décimale : PER Périodiques Résumé : Twenty early-treated children with classical phenylketonuria (PKU), five early-treated children with variant PKU and seven untreated children with hyperphenylalinemia were compared with non-PKU family members in terms of intellectual development, and 14 school-age PKU children were also compared for academic achievement. For the early-treated children with classical PKU, mean IQ (98) was within the normal range, but nine of these 20 children had IQ scores more than 1SD below those of family members. There was a significant negative correlation between phenylalanine concentrations at one to four years of age and later measured intelligence in these early-treated children, but this was probably a consequence of poor dietary control in the early years. The early-treated children with variant PKU and those with hyperphenylalaninemia had IQ scores consistent with those of unaffected family members, but untreated children with variant PKU had scores significantly lower than their own early-treated siblings. Achievement scores of the early-treated PKU children were consistent with their intellectual ability: they and their non-PKU siblings had similar standard scores for reading and spelling, but arithmetic scores were significantly lower for the PKU children. Early-treated children whose diet had been discontinued had achievement scores in all subjects below those predicted from their IQS. Permalink : https://www.cra-rhone-alpes.org/cid/opac_css/index.php?lvl=notice_display&id=496

Phenylketonuria and complex spatial visualization: an analysis of information processing / Robert L. BRUNNER in Developmental Medicine & Child Neurology, 29-4 (August 1987)

Public ISBD [article]  inDevelopmental Medicine & Child Neurology > 29-4 (August 1987) . - p.460-468 Titre : Phenylketonuria and complex spatial visualization: an analysis of information processing Type de document : Texte imprimé et/ou numérique Auteurs : Robert L. BRUNNER, Auteur ; Helen K. BERRY, Auteur ; Daniel B. BERCH, Auteur Année de publication : 1987 Article en page(s) : p.460-468 Langues : Anglais (eng) Index. décimale : PER Périodiques Résumé : Recent neuropsychological studies have suggested that patients with early-treated phenylketonuria (PKU) and normal intelligence have a specific deficiency in solving complex spatial problems. In the present study a task involving the assembly of various shapes was used to compare the performance of 16 PKU patients and 11 sibling controls. Error rates generally were higher and response times slower among the PKU patients, but greater complexity did not produce differential changes in accuracy or speed in the PKU group compared to the controls. Correlations between task performance and IQ measures were significant for the PKU patients, but when IQ was controlled for the group differences vanished. The results suggest that choice of problem-solving strategy, attention span and accuracy of mental representation may be affected in PKU patients, despite efforts to maintain well-controlled phenylalanine concentrations in the blood. Permalink : https://www.cra-rhone-alpes.org/cid/opac_css/index.php?lvl=notice_display&id=626 [article] Phenylketonuria and complex spatial visualization: an analysis of information processing [Texte imprimé et/ou numérique] / Robert L. BRUNNER, Auteur ; Helen K. BERRY, Auteur ; Daniel B. BERCH, Auteur . - 1987 . - p.460-468. Langues : Anglais (eng) in Developmental Medicine & Child Neurology > 29-4 (August 1987) . - p.460-468 Index. décimale : PER Périodiques Résumé : Recent neuropsychological studies have suggested that patients with early-treated phenylketonuria (PKU) and normal intelligence have a specific deficiency in solving complex spatial problems. In the present study a task involving the assembly of various shapes was used to compare the performance of 16 PKU patients and 11 sibling controls. Error rates generally were higher and response times slower among the PKU patients, but greater complexity did not produce differential changes in accuracy or speed in the PKU group compared to the controls. Correlations between task performance and IQ measures were significant for the PKU patients, but when IQ was controlled for the group differences vanished. The results suggest that choice of problem-solving strategy, attention span and accuracy of mental representation may be affected in PKU patients, despite efforts to maintain well-controlled phenylalanine concentrations in the blood. Permalink : https://www.cra-rhone-alpes.org/cid/opac_css/index.php?lvl=notice_display&id=626

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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