scholarly journals Magnetic resonance angiography-defined intracranial vasculopathy is associated with silent cerebral infarcts and glucose-6-phosphate dehydrogenase mutation in children with sickle cell anaemia

2012 ◽  
Vol 159 (3) ◽  
pp. 352-359 ◽  
Author(s):  
Mathula Thangarajh ◽  
Genyan Yang ◽  
Dana Fuchs ◽  
Maria R. Ponisio ◽  
Robert C. McKinstry ◽  
...  
Keyword(s):  
Magnetic Resonance ◽  
Magnetic Resonance Angiography ◽  
Sickle Cell ◽  
Sickle Cell Anaemia ◽  
Cerebral Infarcts ◽  
Glucose 6 Phosphate Dehydrogenase

scholarly journals Intracranial vasculopathy and infarct recurrence in children with sickle cell anaemia, silent cerebral infarcts and normal transcranial Doppler velocities

2017 ◽  
Vol 183 (2) ◽  
pp. 324-326 ◽  
Author(s):  
Natasha A. Choudhury ◽  
Michael R. DeBaun ◽  
Maria R. Ponisio ◽  
Lori C. Jordan ◽  
Mark Rodeghier ◽  
...  
Keyword(s):  
Sickle Cell ◽  
Transcranial Doppler ◽  
Sickle Cell Anaemia ◽  
Cerebral Infarcts

scholarly journals Silent cerebral infarcts occur despite regular blood transfusion therapy after first strokes in children with sickle cell disease

Blood ◽  
2011 ◽  
Vol 117 (3) ◽  
pp. 772-779 ◽  
Author(s):  
Monica L. Hulbert ◽  
Robert C. McKinstry ◽  
JoAnne L. Lacey ◽  
Christopher J. Moran ◽  
Julie A. Panepinto ◽  
...  
Keyword(s):  
Magnetic Resonance ◽  
Sickle Cell Disease ◽  
Blood Transfusion ◽  
Sickle Cell ◽  
Stroke Prevention ◽  
Secondary Stroke Prevention ◽  
Cell Disease ◽  
Cerebral Infarcts ◽  
Eligibility Criteria ◽  
Transfusion Therapy

Abstract Children with sickle cell disease (SCD) and strokes receive blood transfusion therapy for secondary stroke prevention; despite this, approximately 20% experience second overt strokes. Given this rate of second overt strokes and the clinical significance of silent cerebral infarcts, we tested the hypothesis that silent cerebral infarcts occur among children with SCD being transfused for secondary stroke prevention. A prospective cohort enrolled children with SCD and overt strokes at 7 academic centers. Magnetic resonance imaging and magnetic resonance angiography of the brain were scheduled approximately every 1 to 2 years; studies were reviewed by a panel of neuroradiologists. Eligibility criteria included regularly scheduled blood transfusion therapy. Forty children were included; mean pretransfusion hemoglobin S concentration was 29%. Progressive cerebral infarcts occurred in 45% (18 of 40 children) while receiving chronic blood transfusion therapy; 7 had second overt strokes and 11 had new silent cerebral infarcts. Worsening cerebral vasculopathy was associated with new cerebral infarction (overt or silent; relative risk = 12.7; 95% confidence interval, 2.65-60.5, P = .001). Children with SCD and overt strokes receiving regular blood transfusion therapy experience silent cerebral infarcts at a higher rate than previously recognized. Additional therapies are needed for secondary stroke prevention in children with SCD.

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