Burden of neurological and neurocognitive impairment in pediatric sickle cell anemia in Uganda (BRAIN SAFE): a cross-sectional study

Nancy S. Green, Deogratias Munube, Paul Bangirana, Linda Rosset Buluma, Bridget Kebirungi, Robert Opoka, Ezekiel Mupere, Philip Kasirye, Sarah Kiguli, Annet Birabwa, Michael S. Kawooya, Samson K. Lubowa, Rogers Sekibira, Edwards Kayongo, Heather Hume, Mitchell Elkind, Weixin Peng, Gen Li, Caterina Rosano, Philip LaRussaFrank J. Minja, Amelia Boehme, Richard Idro

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Children with sickle cell anemia (SCA) are highly susceptible to stroke and other manifestations of pediatric cerebral vasculopathy. Detailed evaluations in sub-Saharan Africa are limited.


We aimed to establish the frequency and types of pediatric brain injury in a cross-sectional study at a large SCA clinic in Kampala, Uganda in a randomly selected sample of 265 patients with HbSS ages 1–12 years. Brain injury was defined as one or more abnormality on standardized testing: neurocognitive impairment using an age-appropriate test battery, prior stroke by examination or transcranial Doppler (TCD) velocities associated with stroke risk in children with SCA (cerebral arterial time averaged mean maximum velocity ≥ 170 cm/second).


Mean age was 5.5 ± 2.9 years; 52.3% were male. Mean hemoglobin was 7.3 ± 1.01 g/dl; 76.4% had hemoglobin


The high frequency of neurocognitive impairment or other abnormal results describes a large burden of pediatric SCA brain disease in Uganda. Evaluation by any single modality would have underestimated the impact of SCA. Testing the impact of hydroxyurea or other available disease-modifying interventions for reducing or preventing SCA brain effects is warranted.

Original languageUndefined/Unknown
JournalPaediatrics and Child Health, East Africa
Publication statusPublished - 1 Oct 2019

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