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Intelligence quotient in paediatric sickle cell disease: a systematic review and meta-analysis
Jamie M Kawadler1, Jonathan D Clayden1, Chris A Clark1
1Developmental Neurosciences, Institute of Child Health, University College London, London, UK.
Insights
Children with sickle cell disease (SCD) and no visible brain abnormalities on MRI still show lower IQ scores compared to healthy children. This suggests other factors significantly impact cognitive function in SCD.
Area of Science:
- Pediatric Neurology
- Neuroscience
- Hematology
Background:
- Sickle cell disease (SCD) is a leading cause of stroke in children globally.
- Magnetic resonance imaging (MRI) is used to identify silent cerebral infarction (SCI) in SCD patients.
- Lower IQ scores are observed in children with SCI and stroke.
Purpose of the Study:
- To assess the impact of infarction on IQ in children with SCD.
- To determine if children with SCI but no apparent MRI abnormalities have lower IQ compared to healthy controls.
Main Methods:
- A systematic review and meta-analysis of 19 articles.
- Included studies focused on pediatric SCD populations with MRI data and Wechsler IQ scores.
- Compared IQ across three groups: stroke vs. SCI, SCI vs. no SCI, and no SCI vs. healthy controls.
Main Results:
- Stroke patients had a 10-point lower IQ than SCI patients.
- SCI patients had a 6-point lower IQ than those with no SCI.
- Children with SCD and no SCI had a 7-point lower IQ than healthy controls.
Conclusions:
- Children with SCD and no apparent MRI abnormalities exhibit significantly lower IQ than healthy peers.
- Biological, socioeconomic, and environmental factors likely play a crucial role in cognitive function for children with SCD.
Aim:
Sickle cell disease (SCD) is the commonest cause of childhood stroke worldwide. Magnetic resonance imaging (MRI) is routinely used to detect additional silent cerebral infarction (SCI), as IQ is lower in SCI as well as stroke. This review assesses the effect of infarction on IQ, and specifically whether, compared to healthy controls, IQ differences are seen in children with SCI with no apparent MRI abnormality.
Method:
A systematic review was conducted to include articles with an SCD paediatric population, MRI information, and Wechsler IQ. A meta-analysis of 19 articles was performed to compare IQ in three groups: stroke vs SCI; SCI vs no SCI; and no SCI vs healthy controls.
Results:
Mean differences in IQ between all three groups were significant: stroke patients had lower IQ than patients with SCI by 10 points (six studies); patients with SCI had lower IQ than no patients with SCI by 6 points (17 studies); and no patients with SCI had lower IQ than healthy controls by 7 points (seven studies).
Interpretation:
Children with SCD and no apparent MRI abnormality have significantly lower IQ than healthy controls. In this chronic condition, other biological, socioeconomic, and environmental factors must play a significant role in cognition.
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