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The Stroke Prevention Trial in Sickle Cell Anemia (STOP) was a randomized trial to evaluate whether chronic transfusion could prevent initial stroke in children with sickle-cell anemia at high risk as determined by transcranial Doppler (TCD). The trial demonstrated a large benefit of transfusion and was halted early. After termination of the trial, patients(More)
Brain infarction is a well-known but poorly understood complication of sickle cell disease. Seventy-three sickle cell disease patients underwent neurological examinations and high-field, heavily T2-weighted axial cranial magnetic resonance image (MRI) scanning. Eighteen of the 73 had a history of stroke, defined as an acute, focal neurological sign lasting(More)
To investigate cerebral hemodynamics in sickle cell disease (SCD), we used the 133Xenon inhalation technique of quantifying cerebral blood flow (CBF) in 67 patients. Clinical examinations and cerebral magnetic resonance imaging also were performed in all patients. Compared with age-matched healthy controls, CBF was elevated by 68% in patients, and inversely(More)
To understand better the relationship between blood velocity measured by transcranial Doppler and cerebral blood flow measured by the 133Xe inhalation method, we examined 23 patients undergoing evaluation in the Comprehensive Sickle Cell Center at Columbia University. Blood velocity in the middle cerebral artery was directly related to cerebral flow (r =(More)
We evaluated the effects of hematocrit on blood velocity in the middle cerebral artery measured by transcranial Doppler ultrasonography in 45 patients. Hematocrits ranged from 16% to 46%, and systolic blood velocities ranged from 70 to 190 (mean 40 to 140) cm/sec. Decreases in hematocrit correlated with increases in blood velocity in the middle cerebral(More)
BACKGROUND AND PURPOSE Sickle cell disease is associated with cerebral hyperemia, which is therapeutically reduced by transfusion; however, the process of transfusion-induced cerebral perfusion changes has heretofore not been observed. METHODS We document the acute changes of intracranial arterial velocity in 10 patients (7 with strokes, 3 without)(More)
BACKGROUND The standard treatment of stroke in sickle cell disease is chronic transfusion to maintain the fraction of abnormal hemoglobin (hemoglobin S [HbS]) below 20%. Risks associated with such transfusion can be reduced by allowing higher HbS levels, but the physiological consequences of this modification are unknown. Cerebral blood flow is elevated in(More)
Exposure to toxic chemicals may result in alterations of red cell function. In certain cases, the toxic effect requires a genetic predisposition and thus affects only a restricted number of individuals; in other instances, the toxic effect is exerted on the hematopoietic system of every person. Glucose-6-phosphate dehydrogenase deficiency is probably the(More)
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