Journal
CHILDREN-BASEL
Volume 10, Issue 3, Pages -Publisher
MDPI
DOI: 10.3390/children10030532
Keywords
anemia; sickle cell; neuropsychology; intelligence quotient; transcranial Doppler; magnetic resonance imaging; connectome
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This systematic review investigates the association between neuroimaging findings and cognition in children with sickle cell disease (SCD). The results suggest that overt or silent strokes, elevated blood flow velocities, abnormal functional connectivity, and decreased fMRI activation are associated with neuropsychological deficits in children with SCD compared to controls. Early diagnosis and treatment of SCD are critical to address the disease manifestations.
Sickle cell disease (SCD) is the most common inherited single-gene disease. Complications include chronic anaemia, reduced oxygen-carrying capability, and cerebral vasculopathy, resulting in silent cerebral infarction, stroke, and cognitive dysfunction with impairments in measures of executive function, attention, reasoning, language, memory, and IQ. This systematic review aims to investigate the association between neuroimaging findings and cognition in children with SCD. Searches of PubMed and Embase were conducted in March 2022. Studies were included if participants were <18 years, if original data were published in English between 1960 and 2022, if any genotype of SCD was included, and if the relationship between cognition and neuroimaging was examined. Exclusion criteria included case studies, editorials, and reviews. Quality was assessed using the Critical Appraisal Skills Programme Case Control Checklist. A total of 303 articles were retrieved; 33 met the eligibility criteria. The presence of overt or silent strokes, elevated blood flow velocities, abnormal functional connectivity, and decreased fMRI activation were associated with neuropsychological deficits in children with SCD when compared to controls. There is a critical need to address the disease manifestations of SCD early, as damage appears to begin at a young age. Most studies were cross-sectional, restricting the interpretation of the directionality of relationships. Future research employing longitudinal neuroimaging and neuropsychological assessments could improve our understanding of the cumulative consequences of SCD on the developing brain.
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