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Psychosocial Difficulties in Preschool-Age Children with Beckwith-Wiedemann Syndrome: An Exploratory Study
Niccolò Butti1,2, Annalisa Castagna1, Rosario Montirosso1
10-3 Center for the at-Risk Infant, Scientific Institute, IRCCS Eugenio Medea, Bosisio Parini, 23842 Lecco, Italy.
Insights
Beckwith-Wiedemann syndrome (BWS) may impact psychosocial development. While most children with BWS show average behavioral scores, nearly half exhibit social development risks, with older children facing more challenges.
Area of Science:
- Pediatric endocrinology
- Developmental psychology
- Childhood chronic illness
Background:
- Beckwith-Wiedemann syndrome (BWS) is a rare genetic overgrowth disorder.
- While typically not associated with intellectual delay, BWS may influence emotional-behavioral and psychosocial development.
- Chronic illness in childhood necessitates understanding its broader impact on development.
Purpose of the Study:
- To investigate the emotional-behavioral functioning and psychosocial development in young children with Beckwith-Wiedemann syndrome.
- To identify potential risk factors and developmental challenges associated with BWS in early childhood.
Main Methods:
- Parents of 30 children diagnosed with BWS (ages 1.5-6 years) completed standardized questionnaires.
- Assessments focused on emotional-behavioral problems and psychomotor development.
- Data analysis examined group mean scores and identified children exceeding risk thresholds.
Main Results:
- Group mean scores for behavioral problems were within the average range.
- However, 23% of children scored above the risk threshold for social withdrawal.
- Social development scores were lower compared to other developmental domains, and gross-motor skills lagged behind fine-motor skills. Nearly half of the children were at risk for social development issues.
- Older age correlated with increased emotional-behavioral and developmental difficulties; other variables did not show significant associations.
Conclusions:
- Young children with Beckwith-Wiedemann syndrome may experience specific psychosocial and developmental challenges, particularly in social domains.
- Early detection of social withdrawal and developmental delays is crucial for children with BWS.
- Health and educational professionals should integrate psychosocial assessments into routine care for children with BWS to facilitate timely interventions.
Abstract:
Beckwith-Wiedemann syndrome (BWS) is a rare overgrowth disease and is not usually associated with intellectual delay. Living with a chronic illness condition such as BWS, however, might affect emotional-behavioral functioning and psychosocial development. To investigate this issue, parents of 30 children with BWS between 1.5 and 6 years old compiled standardized questionnaires assessing the presence of emotional-behavioral and developmental problems. The group mean scores in each scale of behavioral problems fell within the average range. Nevertheless, 23% of the sample presented scores beyond the risk threshold for social withdrawal. As regards psychomotor development, a lower mean score was reliable in the social domain compared to other developmental scales, and in the gross-motor compared to fine-motor functions. Moreover, scores in the at-risk band were reliable in almost half of the children for social development. Notably, older age was overall associated with higher emotional-behavioral and developmental difficulties, while no other socio-demographic or clinical variables accounted for the scores obtained in the questionnaires. These findings ask for a wider consideration by health and educational professionals of the psychosocial functioning of children with BWS, so as to early detect at-risk conditions and eventually promote adequate interventions.
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