title: Cerebral hyperemia, stroke, and transfusion in sickle cell disease.
abstract: 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 related to hematocrit. An experimental index of cerebral blood volume, pr4, was also elevated in the patients in a similar manner. Cerebral blood volume was positively correlated to CBF in SCD patients but not in controls. History of stroke and current neurologic symptoms were associated with lower flow and higher cerebral blood volume. Transfusion therapy reduced the hyperemia, the reduction being greater than expected by hematocrit elevation alone. These findings document a vasodilatory hyperemia in SCD. This dilatation may be a risk factor for ischemic distal-field infarctions, as visualized by MRI, due to a limitation of cerebrovascular reserve capacity.
authors.affiliation: Department of Psychiatry, College of Physicians and Surgeons, Columbia University, New York, NY.
authors.forename: I
authors.initials: I
authors.lastname: Prohovnik
authors.forename: S G
authors.initials: SG
authors.lastname: Pavlakis
authors.forename: S
authors.initials: S
authors.lastname: Piomelli
authors.forename: J
authors.initials: J
authors.lastname: Bello
authors.forename: J P
authors.initials: JP
authors.lastname: Mohr
authors.forename: S
authors.initials: S
authors.lastname: Hilal
authors.forename: D C
authors.initials: DC
authors.lastname: De Vivo
mesh_terms: D000293:Adolescent; D000328:Adult; D000755:Anemia, Sickle Cell / Q000150:complications / Q000503:physiopathology* / Q000628:therapy; D001803:Blood Transfusion; D002560:Cerebrovascular Circulation; D002561:Cerebrovascular Disorders / Q000150:complications / Q000503:physiopathology*; D002648:Child; D005260:Female; D006400:Hematocrit; D006801:Humans; D006940:Hyperemia / Q000150:complications / Q000503:physiopathology*; D008297:Male; D008875:Middle Aged