Stroke Risk Reduction in Sickle Cell Anemia
Juliana Mariani
Howard J. Weinstein
Prevention of a First Stroke by Transfusions in Children With Sickle Cell Anemia and Abnormal Results on Transcranial Doppler Ultrasonography
Adams RJ, Mckie VC, Hsu L, et al. N Engl J Med. 1998;339(1):5–11
Background
Stroke occurs in 11% of patients with sickle cell anemia (SCA) by age 20.1 Children at high risk for stroke have been identified using transcranial Doppler ultrasonography (TCD) screening. Although the use of blood transfusions to prevent recurrent stroke in SCA was established, the role in prevention of first stroke had not been investigated until this study.
Objectives
To assess the impact of blood transfusions on prevention of first stroke in children with SCA.
Methods
Randomized controlled study at 14 US centers from 1995 to 1996.
Patients
130 children ages 2 to 16 years with SCA or sickle β0-thalassemia with abnormal TCD results (mean blood flow velocity of internal carotid or middle cerebral artery >200 cm/s on 2 ultrasounds). Select exclusion criteria: prior stroke, contraindication to chronic transfusion, conditions or treatments that increase stroke risk, ferritin >500 ng/mL.
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