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Adaptive behavior in children with CHARGE syndrome
Nancy Salem-Hartshorne1, Susan Jacob
1Department of Psychology, Central Michigan University, Mount Pleasant, Michigan 48858, USA. nancy.hartshorne@cmich.edu
American Journal of Medical Genetics. Part A
|January 11, 2005
Summary
This study on CHARGE syndrome found that adaptive behavior scores declined over four years, but many individuals maintained abilities above average. Age at walking was the strongest predictor of adaptive behavior.
Area of Science:
- Developmental Pediatrics
- Clinical Genetics
- Behavioral Neuroscience
Background:
- Historically, CHARGE syndrome's "R" signified "Retardation of Growth and Development," but prior assessments lacked rigor.
- Previous literature has not fully captured the spectrum of developmental abilities in individuals with CHARGE syndrome.
Purpose of the Study:
- To investigate the range of developmental abilities in CHARGE syndrome using the Adaptive Behavior Evaluation Scale (ABES).
- To examine changes in adaptive behavior over a 4-year period and identify predictors of these changes.
Main Methods:
- Parents of individuals with CHARGE syndrome completed the ABES twice over 4 years (N=100, then N=85).
- Correlational, regression, and analysis of variance were used to analyze adaptive behavior scores and related variables.
- Key variables included age at walking, hearing and visual impairment severity, and medical involvement.
Main Results:
- A significant decline in ABES scores was observed over the 4-year period.
- Half of the children achieved standard scores above 70 at the second assessment.
- Age at walking was the strongest negative predictor of adaptive behavior scores; hearing impairment, visual impairment, and medical involvement also showed negative correlations.
Conclusions:
- Adaptive behavior in CHARGE syndrome is more varied and higher than previously reported.
- Age at walking and medical involvement predict changes in adaptive behavior over time.
- Early mobility milestones and reduced medical complexity may be associated with better adaptive outcomes.