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Abstract #0738

Impaired blood-brain barrier function in pediatric sickle cell disease

Zixuan Lin1,2, Eboni Lance3, Yang Li1, Pan Su1, Peiying Liu1, Alicia Cannon3, James F. Casella4, and Hanzhang Lu1,2

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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