Transcranial ultrasonography can identify the children with sickle cell disease who are at highest risk for cerebral infarction. Periodic ultrasound examinations and the selective use of transfusion therapy could make the primary prevention of stroke an achievable goal.

Stroke is an important complication of sickle cell disease. Stroke prediction is clinically important because it offers the possibility of primary prevention. In 1992, transcranial Doppler (TCD) evidence of elevated intracranial internal carotid or middle cerebral artery velocity was demonstrated to be associated strongly with an increased risk of ischemic stroke. This study extends the original study and includes 125 more children, longer follow-up, and intracranial hemorrhage in the stroke-risk model. Elevated time averaged mean maximum blood flow velocity, especially when velocity is 200 cm/sec or greater by TCD, was associated strongly with stroke risk. The cases not predicted by TCD point to the need for more information on the optimal timing of TCD surveillance for stroke risk.

No abstract

In an effort to identify possible risk factors for stroke in Sickle Cell Anemia (Hb SS), we analyzed the distribution of alpha-globin gene deletions in a group of Hb SS patients with and without stroke. The group with stroke consisted of 44 patients, (27 male, 17 female) with a mean of 7.5 years at time of stroke. The control group (non-stroke) had 256 Hb SS patients (126 male, 130 female) with a mean age of 7.7 years. There were 9 patients with heterozygous alpha-thalassemia in the stroke group (20.5%). In the control group, there were 93 patients with heterozygous alpha-thalassemia and 5 with homozygous alpha-thalassemia. The incidence of alpha-thalassemia in Hb SS patients without stroke (38%) was comparable to that reported for the African-American population in general. The incidence in the stroke population (20.5%) was significantly lower (P = 0.02) These results indicate that alpha-thalassemia is associated with a lower risk of stroke in Hb SS. This observation should be confirmed in studies involving larger numbers of patients. Possible protective effects of alpha-thalassemia are unknown but may be related to decreased hemolysis and more favorable rheologic properties of red blood cells.

Measurements using the Acuson system are modestly lower than those obtained with dedicated Doppler using the Nicolet TCD.