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Contribution of sickle cell disease to the occurrence of developmental disabilities: a population-based study
A Ashley-Koch1, C C Murphy, M J Khoury
1Office of Genetics and Disease Prevention, Centers for Disease Control and Prevention, Atlanta, Georgia, USA.
Insights
Children with sickle cell disease (SCD) face a significantly higher risk of developmental disabilities (DD) linked to stroke. Early intervention is crucial to prevent stroke and mitigate associated neurological damage in affected children.
Area of Science:
- Pediatric Neurology
- Hematology
- Developmental Pediatrics
Background:
- Sickle cell disease (SCD) is a genetic blood disorder that can lead to serious complications, including stroke.
- Stroke in childhood can result in significant neurological deficits and developmental disabilities (DD).
Purpose of the Study:
- To investigate the association between stroke-related neurological damage and developmental disabilities in children with sickle cell disease.
- To quantify the risk of DD in children with SCD, specifically those affected by stroke.
Main Methods:
- Population-based surveillance of children aged 3-10 years in metropolitan Atlanta.
- Annual review of school and medical records to identify eligible children with SCD.
- Calculation of observed-to-expected ratios and population attributable fractions to assess risk.
Main Results:
- Children with SCD exhibited a significantly increased risk for developmental disabilities (O/E = 3.2, P < 0.0001).
- This elevated risk was primarily associated with stroke-related neurological damage, particularly for cerebral palsy (O/E = 10.8, P < 0.0001).
- The risk for DD without stroke was not statistically significant (O/E = 1.3, P = 0.23).
Conclusions:
- Children with sickle cell disease have a substantially higher risk of developmental disabilities directly linked to stroke.
- Aggressive preventative strategies targeting stroke are essential to reduce the incidence of associated developmental disabilities in children with SCD.
Purpose:
Population-based surveillance of children aged 3-10 years from metropolitan Atlanta was used to determine if stroke-related neurological damage in children with sickle cell disease (SCD) is associated with developmental disabilities (DD).
Methods:
School and medical records were reviewed annually to identify eligible children. Observed-to-expected ratios, P values, and population attributable fractions were calculated.
Results:
Children with SCD had increased risk for DD (O/E = 3.2, P < 0.0001), particularly mental retardation (O/E = 2.7, P = 0.0005) and cerebral palsy (O/E = 10.8, P < 0.0001). This risk was confined to DD associated with stroke (O/E = 130, P < 0.0001; for DD without stroke: O/E = 1.3, P = 0.23).
Conclusions:
Children with SCD have increased risk for DD associated with stroke; thus, aggressive interventions are needed to prevent stroke in these children.