AI Article Synopsis
- Smith-Magenis syndrome (SMS) is primarily linked to a deletion on chromosome 17, and the study focused on the behaviors and emotional problems of individuals with SMS using the Developmental Behavior Checklist (DBC).
- The study included 28 participants represented by their parents, revealing that while total DBC scores decreased with age, they remained above the typical cut-off of 46, indicating ongoing behavioral and emotional challenges.
- Significant differences in behavioral problems were noted between younger (<9 years) and older age groups (9-17 and >18 years), but no link was found between adaptive behavior, communication, and emotional issues.
Article Abstract
Smith-Magenis syndrome (SMS) is a genetic syndrome most often caused by a deletion on chromosome 17 or more rarely by a mutation in the retinoic acid-induced 1 gene. The aim of this study was to investigate the Developmental Behavior Checklist (DBC) profile of persons with SMS and the associations between behavioural and emotional problems, age, gender, adaptive behaviour and autism symptomatology. Twenty-eight persons with SMS were represented by their parents in this study. DBC Total scores are reduced with age, but they still show a mean that is clearly above the cut-off of 46. The differences between the age groups <9 years and 9-17 years ( = 0.024) and between the age groups <9 years and >18 years ( = 0.007) are significant. We found a significant decrease in behavioural and emotional problems with age in SMS. We did not find a relationship between adapted behaviour and communication and behavioural and emotional problems.
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Source |
|---|---|
| http://www.ncbi.nlm.nih.gov/pmc/articles/PMC8649468 | PMC |
| http://dx.doi.org/10.1177/1744629519901056 | DOI Listing |
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