Intelligence quotient in paediatric sickle cell disease: a systematic review and meta-analysis

Dev Med Child Neurol. 2016 Jul;58(7):672-9. doi: 10.1111/dmcn.13113. Epub 2016 Mar 31.


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.

Publication types

  • Meta-Analysis
  • Review
  • Systematic Review

MeSH terms

  • Anemia, Sickle Cell / complications*
  • Anemia, Sickle Cell / diagnostic imaging
  • Developmental Disabilities / diagnostic imaging
  • Developmental Disabilities / etiology
  • Humans
  • Intellectual Disability / diagnostic imaging
  • Intellectual Disability / etiology*
  • Intelligence Tests*
  • Magnetic Resonance Imaging
  • PubMed / statistics & numerical data