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Updated: Aug 26, 2025

A Precision Medicine Tool for Measurement and Monitoring of Hemoglobin S in Sickle Cell Disease Patients Receiving Transfusion Therapy
Assessment of cerebrovascular function in patients with sickle cell disease using transfer function analysis
Ece Su Sayin1,2, Olivia Sobczyk1,2,3, Julien Poublanc3
1Department of Physiology, University of Toronto, Toronto, Canada.
Patients with sickle cell disease (SCD) show impaired cerebrovascular reactivity (CVR) and slower responses to carbon dioxide (CO2) challenges compared to healthy individuals. This indicates reduced brain blood flow regulation in SCD.
Area of Science:
- Neurology
- Vascular Biology
- Medical Imaging
Background:
- Sickle cell disease (SCD) compromises brain oxygen delivery due to anemia, abnormal blood flow, and vascular disease.
- Effective compensation relies on increased cerebral blood flow (CBF).
Purpose of the Study:
- To assess cerebrovascular reactivity (CVR) in SCD patients using magnetic resonance imaging (MRI).
- To evaluate the dynamic response of cerebral arteries to carbon dioxide (CO2) stimulation.
Main Methods:
- MRI was used to measure the blood oxygen level dependent (BOLD) signal response to hypercapnia (increased CO2).
- Cerebrovascular reactivity (CVR) was assessed using time constant (tau) and transfer function analysis (TFA) measuring gain, phase, and coherence.
- 34 SCD patients were compared to 24 healthy controls.
Main Results:
- SCD patients exhibited significantly reduced CVR and coherence in both gray and white matter compared to controls.
- Reduced gain was observed in gray matter of SCD patients.
- The speed of response, indicated by tau and TFA phase, was significantly slower in SCD patients.
Conclusions:
- Patients with SCD have demonstrably impaired and slowed cerebrovascular responsiveness to CO2.
- These findings highlight altered brain blood flow regulation in SCD, potentially impacting neurological outcomes.
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