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Published on: January 4, 2013
Silent infarcts in children with sickle cell anemia and abnormal cerebral artery velocity
C H Pegelow1, W Wang, S Granger
1Department of Pediatrics (R-131), University of Miami School of Medicine, PO Box 016960, Miami, FL 33101, USA. cpegelow@miami.edu
Insights
Transfusion therapy significantly reduces the risk of silent cerebral infarcts and stroke in children with sickle cell disease and abnormal findings on transcranial Doppler (TCD) and MRI scans. Early detection and treatment are crucial for preventing neurological damage.
Area of Science:
- Pediatric Neurology
- Vascular Neurology
- Medical Imaging
Background:
- A significant number of children with sickle cell disease (SCD) exhibit silent cerebral infarcts on MRI, despite being neurologically normal.
- These infarcts represent a risk for future stroke events.
Purpose of the Study:
- To investigate the impact of transfusion therapy on the development of silent infarcts in children with SCD.
- To assess the utility of Magnetic Resonance Imaging (MRI) in predicting stroke risk, in conjunction with Transcranial Doppler (TCD) ultrasonography.
Main Methods:
- Children with elevated TCD velocities were randomized to either long-term transfusion therapy or standard care.
- Brain MRI was performed at baseline, annually, and upon neurological events.
- The incidence of new silent lesions or stroke was compared between treatment groups.
Main Results:
- Among children with silent infarcts at baseline (37%), those receiving standard care had a higher likelihood of developing new lesions or stroke compared to those on transfusion therapy.
- In the standard care group, pre-existing lesions on MRI significantly increased the risk of subsequent stroke or new silent lesions.
Conclusions:
- Transfusion therapy effectively reduces the risk of new silent infarcts or stroke in children with SCD who have both abnormal TCD velocities and silent infarcts.
- Children with both abnormal TCD and silent infarcts who do not receive transfusions face a higher risk of stroke.
- Screening with TCD and MRI is essential for identifying at-risk children, guiding the consideration of transfusion therapy to prevent neurological complications.
Background:
A substantial minority of neurologically normal children with sickle cell disease have lesions consistent with cerebral infarction as seen on magnetic resonance imaging (MRI).
Objectives:
To determine if transfusion therapy affects the rate at which silent infarcts develop and to evaluate the contribution of MRI of the brain to stroke prediction by transcranial Doppler (TCD) ultrasonography.
Study Design:
Children with elevated TCD ultrasonographic velocity were randomized to receive long-term transfusion therapy or standard care. Magnetic resonance imaging of the brain was obtained at randomization, annually, and with clinical neurologic events. The risk for new silent lesions and/or stroke was compared for each treatment arm.
Results:
Among the 37% of subjects with silent infarcts, those receiving standard care were significantly more likely to develop new silent lesions or stroke than were those who received transfusion therapy. For subjects receiving standard care, those with lesions at baseline were significantly more likely to develop stroke or new silent lesions than those whose MRI studies showed no abnormality.
Conclusions:
Transfusion therapy lowers the risk for new silent infarct or stroke for children having both abnormal TCD ultrasonographic velocity and silent infarct. However, those with both abnormalities who are not provided transfusion therapy are at higher risk for developing a new silent infarct or stroke than are those whose initial MRI showed no abnormality. The finding of a silent infarct reinforces the need for TCD ultrasonographic screening and consideration of transfusion therapy if the abnormalities are seen. Similarly, elevated TCD ultrasonographic velocity warrants MRI of the brain because children with both abnormalities seem to be at increased risk for developing new silent infarct or stroke.
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