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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.

Medrxiv : the Preprint Server for Health Sciences
|November 28, 2023
PubMed

Insights

Children with sickle cell anemia (SCA) older than age 4 show impaired neurocognitive function compared to siblings. Prior stroke and disease severity are key risk factors for cognitive deficits in SCA.

Area of Science:

  • Pediatric Neurology
  • Hematology
  • Neuroscience

Background:

  • Sickle cell anemia (SCA) is a genetic blood disorder that can affect neurocognitive development in children.
  • Identifying risk factors for cognitive impairment in children with SCA is crucial for timely intervention.

Approach:

  • A cross-sectional study compared neurocognitive function in 242 Ugandan children with SCA to 127 non-SCA siblings using standardized cognitive, executive function, and attention tests.
  • Neurocognitive scores were converted to age-normalized z-scores, and SCA patients underwent stroke assessment and transcranial Doppler ultrasound.

Key Points:

  • Children with SCA demonstrated significantly lower overall cognitive and executive function z-scores compared to their 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 in children with SCA.

Conclusions:

  • Children with SCA, particularly those over age 4, exhibit poorer neurocognitive functioning.
  • The findings highlight the need for clinical trials to evaluate disease-modifying therapies for improving neurocognitive outcomes in pediatric SCA.
Abstract

Keywords:
NeurocognitionNeurocognitive impairmentPediatric sickle cellSickle cell anemiaSub-Saharan Africa

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