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Cerebral Infarcts and Vasculopathy in Tanzanian Children With Sickle Cell Anemia
Mboka Jacob1, Dawn E Saunders2, Raphael Z Sangeda3
1Department of Radiology & Imaging, Muhimbili University of Health and Allied Sciences, Dar Es Salaam, Tanzania.
Insights
Silent cerebral infarcts (SCI) and vasculopathy are common in African children with sickle cell anemia (SCA). Vasculopathy increases SCI risk, with anemia and hemolysis as key risk factors.
Area of Science:
- Neurology
- Pediatrics
- Hematology
Background:
- Cerebral infarcts and vasculopathy in children with sickle cell anemia (SCA) are understudied in African settings.
- Silent cerebral infarcts (SCI) and vasculopathy can occur in neurologically asymptomatic children with SCA.
Purpose of the Study:
- To determine the prevalence of SCI and vasculopathy in children with SCA in Africa.
- To investigate associations between SCI/vasculopathy and chronic hemolysis, anemia, and hypoxia.
Main Methods:
- Prospective study of 224 children with SCA.
- Utilized transcranial Doppler (TCD), MRI, and MRA for assessment.
- Performed regression analyses with hemoglobin, reticulocyte count, oxygen content, and bilirubin levels.
Main Results:
- SCI prevalence was 27%; vasculopathy (stenosis/occlusion) was present in 17%.
- SCI was associated with vasculopathy (OR 2.68) and elevated indirect bilirubin.
- Vasculopathy correlated with prior hemoglobin, oxygen content, reticulocytes, and indirect bilirubin levels.
Conclusions:
- SCI and vasculopathy are prevalent in African children with SCA, even with normal TCD.
- Children with vasculopathy face a higher risk of SCI.
- Chronic anemia, hypoxia, and hemolysis are identified as risk factors for vasculopathy.
Background:
Cerebral infarcts and vasculopathy in neurologically asymptomatic children with sickle cell anemia (SCA) have received little attention in African settings. This study aimed to establish the prevalence of silent cerebral infarcts (SCI) and vasculopathy and determine associations with exposure to chronic hemolysis, anemia, and hypoxia.
Methods:
We prospectively studied 224 children with SCA with transcranial Doppler (TCD), and magnetic resonance imaging (MRI) and magnetic resonance angiography (MRA). Regressions were undertaken with contemporaneous hemoglobin, reticulocyte count, mean prior hemoglobin, oxygen content, reticulocyte count, and indirect bilirubin.
Results:
Prevalence of SCI was 27% (61 of 224); cerebral blood flow velocity was abnormal (>200 cm/s) in three and conditional (>170<200 cm/s) in one. Vasculopathy grades 2 (stenosis) and 3 (occlusion) occurred in 16 (7%) and two (1%), respectively; none had grade 4 (moyamoya). SCI was associated with vasculopathy on MRA (odds ratio 2.68; 95% confidence intervals [95% CI] 1.32 to 5.46; P = 0.007) and mean prior indirect bilirubin (odds ratio 1.02, 95% CI 1.00 to 1.03, P = 0.024; n = 83) but not age, sex, non-normal TCD, or contemporaneous hemoglobin. Vasculopathy was associated with mean prior values for hemoglobin (odds ratio 0.33, 95% CI 0.16 to 0.69, P = 0.003; n = 87), oxygen content (odds ratio 0.43, 95% CI 0.25 to 0.74, P = 0.003), reticulocytes (odds ratio 1.20, 95% CI 1.01-1.42, P = 0.041; n = 77), and indirect bilirubin (odds ratio 1.02, 95% CI 1.01 to 1.04, P = 0.009).
Conclusions:
SCI and vasculopathy on MRA are common in neurologically asymptomatic children with SCA living in Africa, even when TCD is normal. Children with vasculopathy on MRA are at increased risk of SCI. Longitudinal exposure to anemia, hypoxia, and hemolysis appear to be risk factors for vasculopathy.
