Sickle cell anemia: reference values of cerebral blood flow determined by continuous arterial spin labeling MRI
M Arkuszewski1, J Krejza, R Chen
1Department of Radiology, Division of Neuroradiology, University of Pennsylvania, Philadelphia, PA, USA. michal.arkuszewski@gmail.com
Insights
Establishing normal cerebral blood flow (CBF) limits in children with sickle cell anemia (SCA) is crucial for predicting cerebrovascular accident (CVA) risk. This study defines reference CBF values using non-invasive MRI in low-risk pediatric SCA patients.
Area of Science:
- Neurology
- Pediatrics
- Medical Imaging
Background:
- Sickle cell anemia (SCA) significantly impacts quality of life and carries a high risk of cerebrovascular accidents (CVA).
- Understanding "normal" cerebral blood flow (CBF) thresholds is vital for assessing CVA risk in asymptomatic children with SCA.
- Current understanding of CBF reference ranges in pediatric SCA is limited, hindering accurate risk stratification.
Purpose of the Study:
- To establish reference tolerance limits for global and regional cerebral blood flow (CBF) in children with sickle cell anemia (SCA).
- To utilize advanced non-invasive imaging techniques for accurate CBF quantification in a pediatric SCA cohort.
- To provide a basis for estimating CVA risk in asymptomatic children with SCA who are at low risk based on other clinical markers.
Main Methods:
- A cohort of 42 children with SCA, carefully selected for low stroke risk, underwent Continuous Arterial Spin Labeling (CASL) MR perfusion imaging.
- Examinations were performed using a 3-Tesla MR scanner to non-invasively quantify global and regional CBF.
- Inclusion criteria ensured participants had no history of stroke, transient ischemic attack, or significant vascular abnormalities.
Main Results:
- The study successfully quantified global and regional CBF in a cohort of pediatric SCA patients using CASL MR.
- Reference tolerance limits for CBF were established for this specific low-risk pediatric SCA population.
- These findings provide crucial data for understanding CBF dynamics in SCA.
Conclusions:
- The established reference CBF values can aid in the risk assessment for cerebrovascular accidents (CVA) in children with sickle cell anemia (SCA).
- Non-invasive CASL MR perfusion is an effective tool for quantifying CBF in pediatric SCA patients.
- Further research can build upon these reference limits to refine CVA risk prediction models in SCA.
Abstract:
Sickle cell anemia (SCA) is a chronic illness associated with progressive deterioration in patients' quality of life. The major complications of SCA are cerebrovascular accidents (CVA) such as asymptomatic cerebral infarct or overt stroke. The risk of CVA may be related to chronic disturbances in cerebral blood flow (CBF), but the thresholds of "normal" steady-state CBF are not well established. The reference tolerance limits of CBF can be useful to estimate the risk of CVA in asymptomatic children with SCA, who are negative for hyperemia or evidence of arterial narrowing. Continuous arterial spin labeling (CASL) MR perfusion allows for non-invasive quantification of global and regional CBF. To establish such reference tolerance limits we performed CASL MR examinations on a 3-Tesla MR scanner in a carefully selected cohort of 42 children with SCA (mean age, 8.1±3.3 years; range limits, 2.3-14.4 years; 24 females), who were not on chronic transfusion therapy, had no history of overt stroke or transient ischemic attack, were free of signs and symptoms of focal vascular territory ischemic brain injury, did not have intracranial arterial narrowing on MR angiography and were at low risk for stroke as determined by transcranial Doppler ultrasonography.
