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Neurocognitive impairment in Ugandan children with sickle cell anemia compared to sibling controls: a cross-sectional
Paul Bangirana1,2, Amelia K Boehme3, Annet Birabwa4
1Department of Psychiatry, Makerere University College of Health Sciences, Kampala, Uganda.
Insights
Children with sickle cell anemia (SCA) show impaired neurocognitive function, particularly those over age 4. Prior stroke and disease severity are key risk factors, highlighting the need for interventions.
Area of Science:
- Pediatric Neurology
- Hematology
- Neuroscience
Background:
- Sickle cell anemia (SCA) is a genetic blood disorder affecting red blood cells.
- Neurocognitive impairments are a known complication of SCA, impacting children's development.
- Understanding risk factors is crucial for early intervention and improved outcomes.
Purpose of the Study:
- To compare neurocognitive functions in Ugandan children with SCA to their healthy siblings.
- To identify risk factors associated with neurocognitive impairment in children with SCA.
Main Methods:
- A cross-sectional study involving 242 children with SCA and 127 non-SCA siblings.
- Standardized cognitive, executive function, and attention tests were administered.
- Stroke risk was assessed using transcranial Doppler ultrasound and stroke examinations.
Main Results:
- Children with SCA exhibited significantly lower overall cognitive and executive function z-scores compared to siblings.
- Neurocognitive deficits in SCA were more pronounced in children older than 4 years.
- Prior stroke, disease severity, and age were identified as the strongest predictors of poor neurocognitive outcomes.
Conclusions:
- Children with SCA, especially those over age 4, experience significant neurocognitive impairments.
- Disease-modifying treatments should be investigated to mitigate these effects.
- Early identification of risk factors like stroke is vital for managing SCA-related neurocognitive deficits.
Introduction:
The neurocognitive functions in Ugandan children aged 1-12 years with sickle cell anemia (SCA) were compared to their non-SCA siblings to identify risk factors for disease-associated impairment.
Methods:
This cross-sectional study of the neurocognitive functions in children with SCA (N = 242) and non-SCA siblings (N = 127) used age- and linguistically appropriate standardized tests of cognition, executive function, and attention for children ages 1-4 and 5-12. Test scores were converted to locally derived age-normalized z-scores. The SCA group underwent a standardized stroke examination for prior stroke and transcranial Doppler ultrasound to determine stroke risk by arterial flow velocity.
Results:
The SCA group was younger than their siblings (mean ages 5.46 ± 3.0 vs. 7.11 ± 3.51 years, respectively; p < 0.001), with a lower hemoglobin concentration (7.32 ± 1.02 vs. 12.06 ± 1.42, p < 0.001). The overall cognitive SCA z-scores were lower, -0.73 ± 0.98, vs. siblings, -0.25 ± 1.12 (p < 0.001), with comparable findings for executive function of -1.09 ± 0.94 vs. -0.84 ± 1.26 (p = 0.045), respectively. The attention z-scores for ages 5-12 for the SCA group and control group were similar: -0.37 ± 1.4 vs. -0.11 ± 0.17 (p = 0.09). The overall differences in SCA status were largely driven by the older age group, as the z-scores in the younger subsample did not differ from controls. Analyses revealed the strongest predictors of poor neurocognitive outcomes among the SCA sample to be the disease, age, and prior stroke (each p < 0.001). The impacts of anemia and SCA were indistinguishable.
Discussion:
Neurocognitive testing in children with SCA compared to non-SCA siblings revealed poorer SCA-associated functioning in children older than age 4. The results indicate the need for trials assessing the impact of disease modification on children with SCA.
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