Neuropsychological effects of subsequent exposure to phenylalanine in adolescents and young adults with early-treated phenylketonuria.

Saved in:
Bibliographic Details
Title: Neuropsychological effects of subsequent exposure to phenylalanine in adolescents and young adults with early-treated phenylketonuria.
Authors: Griffiths, P.1, Paterson, L.1, Harvie, A.2
Source: Journal of Intellectual Disability Research. Oct1995, Vol. 39 Issue 5, p365-372. 8p.
Subject Terms: *Intellectual disabilities, *Mental health services, *Neuropsychology, Phenylketonuria, Amino acid metabolism disorders, Mental health of people with intellectual disabilities, Phenylalanine, Pathological psychology
Abstract: Severe mental handicap in phenytketonuria (PKU) can be prevented if dietary treatment is implemented at birth. Controversy remains about the optimum age for terminating treatment. A group of adolescents and young adults with PKU from the West of Scotland Register was identified which had received early treatment, been well-controlled on diet, ceased treatment at 10 years old and subsequently were hyperphenylalaninaemic for 3 years or more. They were given a battery of neuropsychological tests and their results were compared with those of on-diet subjects with PKU and normal controls. The findings generally supported the view that dietary cessation at age 10 is sufficient to prevent a substantial reduction of cognitive and motor ability, and that the central nervous system is probably mature enough to withstand the toxic effects of high blood phenylalanine by then. However, there were minor indications, in keeping with Welsh et al.'s hypothesis [M.C.Welsh. B.F. Pennington. S. Ozonoff, B. Rouse & E.R.B. McCabe (1990) Neuropsychology of early-treated phenylketonuria: specific executive function deficits. Child Development 61,1697–713], that subtle intellectual deficiencies might arise both during and after treatment, possibly in the realm of frontal-executive functions. [ABSTRACT FROM AUTHOR]
Copyright of Journal of Intellectual Disability Research is the property of Wiley-Blackwell and its content may not be copied or emailed to multiple sites without the copyright holder's express written permission. Additionally, content may not be used with any artificial intelligence tools or machine learning technologies. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)
Database: Education Research Complete
FullText Links:
  – Type: pdflink
Text:
  Availability: 0
Header DbId: ehh
DbLabel: Education Research Complete
An: 16270273
AccessLevel: 6
PubType: Academic Journal
PubTypeId: academicJournal
PreciseRelevancyScore: 0
IllustrationInfo
Items – Name: Title
  Label: Title
  Group: Ti
  Data: Neuropsychological effects of subsequent exposure to phenylalanine in adolescents and young adults with early-treated phenylketonuria.
– Name: Author
  Label: Authors
  Group: Au
  Data: <searchLink fieldCode="AR" term="%22Griffiths%2C+P%2E%22">Griffiths, P.</searchLink><relatesTo>1</relatesTo><br /><searchLink fieldCode="AR" term="%22Paterson%2C+L%2E%22">Paterson, L.</searchLink><relatesTo>1</relatesTo><br /><searchLink fieldCode="AR" term="%22Harvie%2C+A%2E%22">Harvie, A.</searchLink><relatesTo>2</relatesTo>
– Name: TitleSource
  Label: Source
  Group: Src
  Data: <searchLink fieldCode="JN" term="%22Journal+of+Intellectual+Disability+Research%22">Journal of Intellectual Disability Research</searchLink>. Oct1995, Vol. 39 Issue 5, p365-372. 8p.
– Name: Subject
  Label: Subject Terms
  Group: Su
  Data: *<searchLink fieldCode="DE" term="%22Intellectual+disabilities%22">Intellectual disabilities</searchLink><br />*<searchLink fieldCode="DE" term="%22Mental+health+services%22">Mental health services</searchLink><br />*<searchLink fieldCode="DE" term="%22Neuropsychology%22">Neuropsychology</searchLink><br /><searchLink fieldCode="DE" term="%22Phenylketonuria%22">Phenylketonuria</searchLink><br /><searchLink fieldCode="DE" term="%22Amino+acid+metabolism+disorders%22">Amino acid metabolism disorders</searchLink><br /><searchLink fieldCode="DE" term="%22Mental+health+of+people+with+intellectual+disabilities%22">Mental health of people with intellectual disabilities</searchLink><br /><searchLink fieldCode="DE" term="%22Phenylalanine%22">Phenylalanine</searchLink><br /><searchLink fieldCode="DE" term="%22Pathological+psychology%22">Pathological psychology</searchLink>
– Name: Abstract
  Label: Abstract
  Group: Ab
  Data: Severe mental handicap in phenytketonuria (PKU) can be prevented if dietary treatment is implemented at birth. Controversy remains about the optimum age for terminating treatment. A group of adolescents and young adults with PKU from the West of Scotland Register was identified which had received early treatment, been well-controlled on diet, ceased treatment at 10 years old and subsequently were hyperphenylalaninaemic for 3 years or more. They were given a battery of neuropsychological tests and their results were compared with those of on-diet subjects with PKU and normal controls. The findings generally supported the view that dietary cessation at age 10 is sufficient to prevent a substantial reduction of cognitive and motor ability, and that the central nervous system is probably mature enough to withstand the toxic effects of high blood phenylalanine by then. However, there were minor indications, in keeping with Welsh et al.'s hypothesis [M.C.Welsh. B.F. Pennington. S. Ozonoff, B. Rouse & E.R.B. McCabe (1990) Neuropsychology of early-treated phenylketonuria: specific executive function deficits. Child Development 61,1697–713], that subtle intellectual deficiencies might arise both during and after treatment, possibly in the realm of frontal-executive functions. [ABSTRACT FROM AUTHOR]
– Name: AbstractSuppliedCopyright
  Label:
  Group: Ab
  Data: <i>Copyright of Journal of Intellectual Disability Research is the property of Wiley-Blackwell and its content may not be copied or emailed to multiple sites without the copyright holder's express written permission. Additionally, content may not be used with any artificial intelligence tools or machine learning technologies. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract.</i> (Copyright applies to all Abstracts.)
PLink https://search.ebscohost.com/login.aspx?direct=true&site=eds-live&db=ehh&AN=16270273
RecordInfo BibRecord:
  BibEntity:
    Identifiers:
      – Type: doi
        Value: 10.1111/j.1365-2788.1995.tb00540.x
    Languages:
      – Code: eng
        Text: English
    PhysicalDescription:
      Pagination:
        PageCount: 8
        StartPage: 365
    Subjects:
      – SubjectFull: Intellectual disabilities
        Type: general
      – SubjectFull: Mental health services
        Type: general
      – SubjectFull: Neuropsychology
        Type: general
      – SubjectFull: Phenylketonuria
        Type: general
      – SubjectFull: Amino acid metabolism disorders
        Type: general
      – SubjectFull: Mental health of people with intellectual disabilities
        Type: general
      – SubjectFull: Phenylalanine
        Type: general
      – SubjectFull: Pathological psychology
        Type: general
    Titles:
      – TitleFull: Neuropsychological effects of subsequent exposure to phenylalanine in adolescents and young adults with early-treated phenylketonuria.
        Type: main
  BibRelationships:
    HasContributorRelationships:
      – PersonEntity:
          Name:
            NameFull: Griffiths, P.
      – PersonEntity:
          Name:
            NameFull: Paterson, L.
      – PersonEntity:
          Name:
            NameFull: Harvie, A.
    IsPartOfRelationships:
      – BibEntity:
          Dates:
            – D: 01
              M: 10
              Text: Oct1995
              Type: published
              Y: 1995
          Identifiers:
            – Type: issn-print
              Value: 09642633
          Numbering:
            – Type: volume
              Value: 39
            – Type: issue
              Value: 5
          Titles:
            – TitleFull: Journal of Intellectual Disability Research
              Type: main
ResultId 1