Abstract #0738

Impaired blood-brain barrier function in pediatric sickle cell disease

Zixuan Lin^1,2, Eboni Lance³, Yang Li¹, Pan Su¹, Peiying Liu¹, Alicia Cannon³, James F. Casella⁴, and Hanzhang Lu^1,2

¹Department of Radiology, Johns Hopkins University, Baltimore, MD, United States, ²Department of Biomedical Engineering, Johns Hopkins University, Baltimore, MD, United States, ³Kennedy Krieger Institute, Baltimore, MD, United States, ⁴Department of Pediatric Hematology, Johns Hopkins University, Baltimore, MD, United States

Sickle cell disease (SCD) is associated with endothelium dysfunction, but the role of the blood-brain barrier (BBB) in SCD has not been evaluated. We evaluated BBB function in pediatric SCD using a novel non-invasive method, water-extraction-with-phase-contrast-arterial-spin-tagging (WEPCAST) MRI. Children with SCD showed lower water extraction compared with healthy individuals. Higher permeability-surface-area-product (PS), i.e. leaky BBB, was associated with a number of known abnormalities in SCD, including lower hematocrit, lower hemoglobin, higher HbS fraction, impaired cognition, and a higher risk for silent cerebral infarction. Collectively, these findings support a potential role for BBB dysfunction in SCD pathogenesis.

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