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Regional cerebral blood flow, blood volume, fractional oxygen extraction and oxygen consumption were measured by positron emission tomography in six patients with sickle cell disease to see how oxygen delivery to the brain is maintained in the presence of both anemia and a low oxygen affinity hemoglobin. Both regional cerebral blood flow and blood volume were found to be markedly increased compared to values obtained from 14 normal subjects in the same age range. The mean fractional oxygen extraction was not significantly different in the two groups. Mean oxygen consumption in the two groups was also not significantly different but low values in individual patients with sickle cell disease and the presence of atrophy on the CT-scans of three of them were suggestive of some neuronal loss in patients without any history of nervous system involvement. In view of the known high values of cerebral blood flow and metabolism in childhood, it is suggested that when compounded by anemia and abnormal red cells, a hypercirculatory state may make patients in this age-group particularly prone to ischemic infarction.

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Measurement of regional cerebral blood flow, blood volume and oxygen metabolism in patients with sickle cell disease using positron emission tomography.