• White-matter abnormalities and cognitive dysfunction are linked to astrocyte activation in sickle mice
    by Rimi Hazra on May 22, 2023 at 10:00 am

    White-matter injury in sickle-cell disease (SCD) includes silent cerebral infarction diagnosed by diffusion tensor imaging (DTI), a complication associated with cognitive dysfunction in children with SCD. The link between white-matter injury and cognitive dysfunction has not been fully elucidated. The goal of this study was to define whether cerebrovascular lesions and cognitive function in SCD are linked to neuroaxonal damage and astrocyte activation in humanized Townes' SCD mice homozygous for...

  • Tailoring a Digital Mental Health Program for Patients With Sickle Cell Disease: Qualitative Study
    by Cara Nikolajski on April 6, 2023 at 10:00 am

    CONCLUSIONS: Enhancing the user experience by making digital CBT tools relevant to patient populations is critical for optimizing program engagement and its uptake. Our findings highlight potential strategies to modify and design digital CBT tools for users with SCD and may also be applicable to patients with other chronic conditions.

  • Metabolic correlates to critical speed in murine models of sickle cell disease
    by Francesca I Cendali on April 3, 2023 at 10:00 am

    Introduction: Exercise intolerance is a common clinical manifestation in patients with sickle cell disease (SCD), though the mechanisms are incompletely understood. Methods: Here we leverage a murine mouse model of sickle cell disease, the Berkeley mouse, to characterize response to exercise via determination of critical speed (CS), a functional measurement of mouse running speed upon exerting to exhaustion. Results: Upon observing a wide distribution in critical speed phenotypes, we...

  • Insulin-like Growth Factor-1 Prevents Hypoxia/Reoxygenation-Induced White Matter Injury in Sickle Cell Mice
    by Rimi Hazra on March 29, 2023 at 10:00 am

    Occlusion of cerebral blood vessels causes acute cerebral hypoxia-an important trigger of ischemic white matter injury and stroke in sickle cell disease (SCD). While chronic hypoxia triggers compensatory neuroprotection via insulin-like growth factor-1 (IGF-1) and hypoxia inducible factor-1α (HIF-1α), severe bouts of acute hypoxia and subsequent restoration of blood flow (hypoxia/reoxygenation, H/R) overwhelm compensatory mechanisms and cause neuroaxonal damage-identified as white matter...

  • In vivo evaluation of the effect of sickle cell hemoglobin S, C and therapeutic transfusion on erythrocyte metabolism and cardiorenal dysfunction
    by Angelo D'Alessandro on March 27, 2023 at 10:00 am

    Despite a wealth of exploratory plasma metabolomics studies in sickle cell disease (SCD), no study to date has evaluate a large and well phenotyped cohort to compare the primary erythrocyte metabolome of hemoglobin SS, SC and transfused AA red blood cells (RBCs) in vivo. The current study evaluates the RBC metabolome of 587 subjects with sickle cell sickle cell disease (SCD) from the WALK-PHaSST clinical cohort. The set includes hemoglobin SS, hemoglobin SC SCD patients, with variable levels of...