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Osmotic Drug Delivery to Ischemic Hindlimbs and Perfusion of Vasculature with Microfil for Micro-Computed Tomography Imaging
Published on: June 29, 2013
Sickle cell disease, vasculopathy, and therapeutics
Adetola A Kassim1, Michael R DeBaun
1Department of Medicine, Hematology/Stem Cell Transplant, Vanderbilt and Meharry Center for Excellence in Sickle Cell Disease, Vanderbilt University Medical Center, Nashville, Tennessee 37232, USA. adetola.kassim@vanderbilt.edu
Annual Review of Medicine
|November 30, 2012
Summary
Sickle cell disease (SCD) involves gene mutation causing red blood cell issues. Vasculopathy in SCD often signals future organ damage, suggesting targeted therapies could prevent severe complications.
Area of Science:
- Hematology
- Vascular Biology
- Genetics
Background:
- Sickle cell disease (SCD) stems from beta globin gene mutations, leading to chronic hemolysis, vaso-occlusion, and multiorgan damage.
- Pathogenesis involves HbS polymerization and endothelial dysfunction, influenced by hemolysis, nitric oxide defects, oxidative stress, and inflammation.
Purpose of the Study:
- To review evidence linking sickle cell disease vasculopathy to subsequent organ dysfunction.
- To explore the potential of targeted antivasculopathy therapies for SCD management.
Main Methods:
- Literature review of studies on SCD pathogenesis and vasculopathy.
- Analysis of organ-specific vasculopathies and their predictive value for dysfunction.
- Evaluation of therapeutic strategies targeting vascular abnormalities in SCD.
Main Results:
- SCD-related vasculopathies, including moyamoya, retinopathy, pulmonary hypertension, and renal disease, often precede overt organ dysfunction.
- These vascular changes are critical indicators of disease progression and potential therapeutic targets.
Conclusions:
- Sickle cell disease vasculopathy serves as an early warning sign for organ damage.
- Targeted antivasculopathy treatments hold promise for mitigating SCD-related morbidity and mortality.
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