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Adaptive behaviour deficits in individuals with 3q29 deletion syndrome.

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Title: Adaptive behaviour deficits in individuals with 3q29 deletion syndrome.
Authors: Pollak, R. M. (AUTHOR), Burrell, T. L. (AUTHOR), Cubells, J. F. (AUTHOR), Klaiman, C. (AUTHOR), Murphy, M. M. (AUTHOR), Saulnier, C. A. (AUTHOR), Walker, E. F. (AUTHOR), White, S. P. (AUTHOR), Mulle, J. G. (AUTHOR)
Source: Journal of Intellectual Disability Research. Feb2024, Vol. 68 Issue 2, p113-127. 15p.
Subjects: Executive function, Cognition, Behavior disorders, Risk assessment, Severity of illness index, Chromosome abnormalities, Child psychopathology, Genomics, Autism, Research funding, Psychological adaptation, Social disabilities, Intellectual disabilities, Disease risk factors, Disease complications
Abstract: Background: 3q29 deletion syndrome (3q29del) is associated with a significantly increased risk for neurodevelopmental and neuropsychiatric phenotypes. Mild to moderate intellectual disability (ID) is common in this population, and previous work by our team identified substantial deficits in adaptive behaviour. However, the full profile of adaptive function in 3q29del has not been described nor has it been compared with other genomic syndromes associated with elevated risk for neurodevelopmental and neuropsychiatric phenotypes. Methods: Individuals with 3q29del (n = 32, 62.5% male) were evaluated using the Vineland Adaptive Behaviour Scales, Third Edition, Comprehensive Parent/Caregiver Form (Vineland‐3). We explored the relationship between adaptive behaviour and cognitive function, executive function, and neurodevelopmental and neuropsychiatric comorbidities in our 3q29del study sample, and we compared subjects with 3q29del with published data on fragile X syndrome, 22q11.2 deletion syndrome and 16p11.2 deletion and duplication syndromes. Results: Individuals with 3q29del had global deficits in adaptive behaviour that were not driven by specific weaknesses in any given domain. Individual neurodevelopmental and neuropsychiatric diagnoses had a small effect on adaptive behaviour, and the cumulative number of comorbid diagnoses was significantly negatively associated with Vineland‐3 performance. Both cognitive ability and executive function were significantly associated with adaptive behaviour, and executive function was a better predictor of Vineland‐3 performance than cognitive ability. Finally, the severity of adaptive behaviour deficits in 3q29del was distinct from previously published data on comparable genomic disorders. Conclusions: Individuals with 3q29del have significant deficits in adaptive behaviour, affecting all domains assessed by the Vineland‐3. Executive function is a better predictor of adaptive behaviour than cognitive ability in this population and suggests that interventions targeting executive function may be an effective therapeutic strategy. [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.)
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  Data: Adaptive behaviour deficits in individuals with 3q29 deletion syndrome.
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  Data: <searchLink fieldCode="AR" term="%22Pollak%2C+R%2E+M%2E%22">Pollak, R. M.</searchLink> (AUTHOR)<br /><searchLink fieldCode="AR" term="%22Burrell%2C+T%2E+L%2E%22">Burrell, T. L.</searchLink> (AUTHOR)<br /><searchLink fieldCode="AR" term="%22Cubells%2C+J%2E+F%2E%22">Cubells, J. F.</searchLink> (AUTHOR)<br /><searchLink fieldCode="AR" term="%22Klaiman%2C+C%2E%22">Klaiman, C.</searchLink> (AUTHOR)<br /><searchLink fieldCode="AR" term="%22Murphy%2C+M%2E+M%2E%22">Murphy, M. M.</searchLink> (AUTHOR)<br /><searchLink fieldCode="AR" term="%22Saulnier%2C+C%2E+A%2E%22">Saulnier, C. A.</searchLink> (AUTHOR)<br /><searchLink fieldCode="AR" term="%22Walker%2C+E%2E+F%2E%22">Walker, E. F.</searchLink> (AUTHOR)<br /><searchLink fieldCode="AR" term="%22White%2C+S%2E+P%2E%22">White, S. P.</searchLink> (AUTHOR)<br /><searchLink fieldCode="AR" term="%22Mulle%2C+J%2E+G%2E%22">Mulle, J. G.</searchLink> (AUTHOR)
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  Data: <searchLink fieldCode="JN" term="%22Journal+of+Intellectual+Disability+Research%22">Journal of Intellectual Disability Research</searchLink>. Feb2024, Vol. 68 Issue 2, p113-127. 15p.
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  Data: <searchLink fieldCode="DE" term="%22Executive+function%22">Executive function</searchLink><br /><searchLink fieldCode="DE" term="%22Cognition%22">Cognition</searchLink><br /><searchLink fieldCode="DE" term="%22Behavior+disorders%22">Behavior disorders</searchLink><br /><searchLink fieldCode="DE" term="%22Risk+assessment%22">Risk assessment</searchLink><br /><searchLink fieldCode="DE" term="%22Severity+of+illness+index%22">Severity of illness index</searchLink><br /><searchLink fieldCode="DE" term="%22Chromosome+abnormalities%22">Chromosome abnormalities</searchLink><br /><searchLink fieldCode="DE" term="%22Child+psychopathology%22">Child psychopathology</searchLink><br /><searchLink fieldCode="DE" term="%22Genomics%22">Genomics</searchLink><br /><searchLink fieldCode="DE" term="%22Autism%22">Autism</searchLink><br /><searchLink fieldCode="DE" term="%22Research+funding%22">Research funding</searchLink><br /><searchLink fieldCode="DE" term="%22Psychological+adaptation%22">Psychological adaptation</searchLink><br /><searchLink fieldCode="DE" term="%22Social+disabilities%22">Social disabilities</searchLink><br /><searchLink fieldCode="DE" term="%22Intellectual+disabilities%22">Intellectual disabilities</searchLink><br /><searchLink fieldCode="DE" term="%22Disease+risk+factors%22">Disease risk factors</searchLink><br /><searchLink fieldCode="DE" term="%22Disease+complications%22">Disease complications</searchLink>
– Name: Abstract
  Label: Abstract
  Group: Ab
  Data: Background: 3q29 deletion syndrome (3q29del) is associated with a significantly increased risk for neurodevelopmental and neuropsychiatric phenotypes. Mild to moderate intellectual disability (ID) is common in this population, and previous work by our team identified substantial deficits in adaptive behaviour. However, the full profile of adaptive function in 3q29del has not been described nor has it been compared with other genomic syndromes associated with elevated risk for neurodevelopmental and neuropsychiatric phenotypes. Methods: Individuals with 3q29del (n = 32, 62.5% male) were evaluated using the Vineland Adaptive Behaviour Scales, Third Edition, Comprehensive Parent/Caregiver Form (Vineland‐3). We explored the relationship between adaptive behaviour and cognitive function, executive function, and neurodevelopmental and neuropsychiatric comorbidities in our 3q29del study sample, and we compared subjects with 3q29del with published data on fragile X syndrome, 22q11.2 deletion syndrome and 16p11.2 deletion and duplication syndromes. Results: Individuals with 3q29del had global deficits in adaptive behaviour that were not driven by specific weaknesses in any given domain. Individual neurodevelopmental and neuropsychiatric diagnoses had a small effect on adaptive behaviour, and the cumulative number of comorbid diagnoses was significantly negatively associated with Vineland‐3 performance. Both cognitive ability and executive function were significantly associated with adaptive behaviour, and executive function was a better predictor of Vineland‐3 performance than cognitive ability. Finally, the severity of adaptive behaviour deficits in 3q29del was distinct from previously published data on comparable genomic disorders. Conclusions: Individuals with 3q29del have significant deficits in adaptive behaviour, affecting all domains assessed by the Vineland‐3. Executive function is a better predictor of adaptive behaviour than cognitive ability in this population and suggests that interventions targeting executive function may be an effective therapeutic strategy. [ABSTRACT FROM AUTHOR]
– Name: AbstractSuppliedCopyright
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  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.)
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        Value: 10.1111/jir.13094
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      – Code: eng
        Text: English
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        PageCount: 15
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      – SubjectFull: Executive function
        Type: general
      – SubjectFull: Cognition
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      – SubjectFull: Behavior disorders
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      – SubjectFull: Risk assessment
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      – SubjectFull: Severity of illness index
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      – TitleFull: Adaptive behaviour deficits in individuals with 3q29 deletion syndrome.
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              Text: Feb2024
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