Developmental trajectories in syndromes with intellectual disability, with a focus on wolf-hirschhorn and its cognitive-behavioral profile

Gene S. Fisch, Nancy Carpenter, Patricia N. Howard-Peebles, Jeanette J A Holden, Jack Tarleton, Richard Simensen, Agatino Battaglia

Research output: Contribution to journalArticle

15 Citations (Scopus)

Abstract

Few studies exist of developmental trajectories in children with intellectual disability, and none for those with subtelomeric deletions. We compared developmental trajectories of children with Wolf-Hirschhorn syndrome to other genetic disorders. We recruited 106 children diagnosed with fragile X, Williams-Beuren syndrome, or Wolf-Hirschhorn syndrome, assessing their intellectual and adaptive behavior abilities. We retested 61 children 2 years later. We compared Time 1 and Time 2 difference scores related to genetic disorder, age, initial IQ, or adaptive behavior composite. Results show genetic disorder and initial IQ score were significant factors for IQ differences, but only genetic disorder affected adaptive behavior differences. Results suggest different gene-brain-behavior pathways likely exist for these genetic disorders. Different developmental trajectories will influence the type and intensity of intervention implemented by caregivers.

Original languageEnglish
Pages (from-to)167-179
Number of pages13
JournalAmerican Journal on Intellectual and Developmental Disabilities
Volume117
Issue number2
DOIs
Publication statusPublished - Mar 2012

Fingerprint

Inborn Genetic Diseases
Intellectual Disability
Psychological Adaptation
Wolf-Hirschhorn Syndrome
Williams Syndrome
Aptitude
Disabled Children
Caregivers
Syndrome
Trajectory
Brain
Genes

Keywords

  • Adaptive behavior
  • Developmental quotient
  • FMR1 mutation
  • Fragile X syndrome
  • Intellectual disability (ID)
  • IQ
  • Williams-Beuren syndrome
  • Wolf-Hirschhorn syndrome

ASJC Scopus subject areas

  • Clinical Neurology
  • Pediatrics, Perinatology, and Child Health
  • Psychiatry and Mental health
  • Developmental and Educational Psychology
  • Neuropsychology and Physiological Psychology
  • Arts and Humanities (miscellaneous)

Cite this

Developmental trajectories in syndromes with intellectual disability, with a focus on wolf-hirschhorn and its cognitive-behavioral profile. / Fisch, Gene S.; Carpenter, Nancy; Howard-Peebles, Patricia N.; Holden, Jeanette J A; Tarleton, Jack; Simensen, Richard; Battaglia, Agatino.

In: American Journal on Intellectual and Developmental Disabilities, Vol. 117, No. 2, 03.2012, p. 167-179.

Research output: Contribution to journalArticle

Fisch, Gene S. ; Carpenter, Nancy ; Howard-Peebles, Patricia N. ; Holden, Jeanette J A ; Tarleton, Jack ; Simensen, Richard ; Battaglia, Agatino. / Developmental trajectories in syndromes with intellectual disability, with a focus on wolf-hirschhorn and its cognitive-behavioral profile. In: American Journal on Intellectual and Developmental Disabilities. 2012 ; Vol. 117, No. 2. pp. 167-179.
@article{eab701c9f5e94c44bbd5c9a95fd2cadc,
title = "Developmental trajectories in syndromes with intellectual disability, with a focus on wolf-hirschhorn and its cognitive-behavioral profile",
abstract = "Few studies exist of developmental trajectories in children with intellectual disability, and none for those with subtelomeric deletions. We compared developmental trajectories of children with Wolf-Hirschhorn syndrome to other genetic disorders. We recruited 106 children diagnosed with fragile X, Williams-Beuren syndrome, or Wolf-Hirschhorn syndrome, assessing their intellectual and adaptive behavior abilities. We retested 61 children 2 years later. We compared Time 1 and Time 2 difference scores related to genetic disorder, age, initial IQ, or adaptive behavior composite. Results show genetic disorder and initial IQ score were significant factors for IQ differences, but only genetic disorder affected adaptive behavior differences. Results suggest different gene-brain-behavior pathways likely exist for these genetic disorders. Different developmental trajectories will influence the type and intensity of intervention implemented by caregivers.",
keywords = "Adaptive behavior, Developmental quotient, FMR1 mutation, Fragile X syndrome, Intellectual disability (ID), IQ, Williams-Beuren syndrome, Wolf-Hirschhorn syndrome",
author = "Fisch, {Gene S.} and Nancy Carpenter and Howard-Peebles, {Patricia N.} and Holden, {Jeanette J A} and Jack Tarleton and Richard Simensen and Agatino Battaglia",
year = "2012",
month = "3",
doi = "10.1352/1944-7558-117.2.167",
language = "English",
volume = "117",
pages = "167--179",
journal = "American Journal on Intellectual and Developmental Disabilities",
issn = "1944-7558",
publisher = "American Association on Intellectual and Developmental Disabilities",
number = "2",

}

TY - JOUR

T1 - Developmental trajectories in syndromes with intellectual disability, with a focus on wolf-hirschhorn and its cognitive-behavioral profile

AU - Fisch, Gene S.

AU - Carpenter, Nancy

AU - Howard-Peebles, Patricia N.

AU - Holden, Jeanette J A

AU - Tarleton, Jack

AU - Simensen, Richard

AU - Battaglia, Agatino

PY - 2012/3

Y1 - 2012/3

N2 - Few studies exist of developmental trajectories in children with intellectual disability, and none for those with subtelomeric deletions. We compared developmental trajectories of children with Wolf-Hirschhorn syndrome to other genetic disorders. We recruited 106 children diagnosed with fragile X, Williams-Beuren syndrome, or Wolf-Hirschhorn syndrome, assessing their intellectual and adaptive behavior abilities. We retested 61 children 2 years later. We compared Time 1 and Time 2 difference scores related to genetic disorder, age, initial IQ, or adaptive behavior composite. Results show genetic disorder and initial IQ score were significant factors for IQ differences, but only genetic disorder affected adaptive behavior differences. Results suggest different gene-brain-behavior pathways likely exist for these genetic disorders. Different developmental trajectories will influence the type and intensity of intervention implemented by caregivers.

AB - Few studies exist of developmental trajectories in children with intellectual disability, and none for those with subtelomeric deletions. We compared developmental trajectories of children with Wolf-Hirschhorn syndrome to other genetic disorders. We recruited 106 children diagnosed with fragile X, Williams-Beuren syndrome, or Wolf-Hirschhorn syndrome, assessing their intellectual and adaptive behavior abilities. We retested 61 children 2 years later. We compared Time 1 and Time 2 difference scores related to genetic disorder, age, initial IQ, or adaptive behavior composite. Results show genetic disorder and initial IQ score were significant factors for IQ differences, but only genetic disorder affected adaptive behavior differences. Results suggest different gene-brain-behavior pathways likely exist for these genetic disorders. Different developmental trajectories will influence the type and intensity of intervention implemented by caregivers.

KW - Adaptive behavior

KW - Developmental quotient

KW - FMR1 mutation

KW - Fragile X syndrome

KW - Intellectual disability (ID)

KW - IQ

KW - Williams-Beuren syndrome

KW - Wolf-Hirschhorn syndrome

UR - http://www.scopus.com/inward/record.url?scp=84860237793&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=84860237793&partnerID=8YFLogxK

U2 - 10.1352/1944-7558-117.2.167

DO - 10.1352/1944-7558-117.2.167

M3 - Article

VL - 117

SP - 167

EP - 179

JO - American Journal on Intellectual and Developmental Disabilities

JF - American Journal on Intellectual and Developmental Disabilities

SN - 1944-7558

IS - 2

ER -