title: Acute silent cerebral ischemic events in children with sickle cell anemia.
abstract: BACKGROUND
  Irregular, sporadic episodes of ischemic brain injury are known to occur in sickle cell anemia (SCA), resulting in overt stroke and silent cerebral infarction. Ongoing ischemia in other organs is common in SCA but has never been documented in the brain.
  
  
  OBJECTIVE
  To test the hypothesis that acute silent cerebral ischemic events (ASCIEs) are frequent and potentially transient.
  
  
  METHODS
  Cross-sectional and cohort study of children with SCA screened by magnetic resonance imaging (MRI) of the brain for a randomized clinical trial.
  
  
  METHODS
  Clinical trial setting in tertiary care centers.
  
  
  METHODS
  Asymptomatic children with SCA without known stroke, neurologic injury, or epilepsy not receiving treatment with transfusions or hydroxyurea.
  
  
  METHODS
  Incidence of ASCIEs calculated using single diffusion-weighted MRI scans (acute ischemic events that occurred within 10 days of the MRI).
  
  
  RESULTS
  Acute silent cerebral ischemic events were detected on 1.3% of MRIs (10 of 771) in 652 children (mean age, 10.0 years), with an incidence of 47.3 events per 100 patient-years (95% CI, 22.7-87.2). Two of 10 children with ASCIEs had follow-up MRIs of the brain; only 1 had silent cerebral infarction in the same location as the previously detected ASCIE.
  
  
  CONCLUSIONS
  Children with SCA experience ongoing (chronic, intermittent) cerebral ischemia, sometimes reversible, far more frequently than previously recognized. The brain in SCA is at constant threat of ischemia.
authors.affiliation: Department of Hematology, Cincinnati Children’s Hospital Medical Center, Cincinnati, USA.
authors.forename: Charles T
authors.initials: CT
authors.lastname: Quinn
authors.forename: Robert C
authors.initials: RC
authors.lastname: McKinstry
authors.forename: Michael M
authors.initials: MM
authors.lastname: Dowling
authors.forename: William S
authors.initials: WS
authors.lastname: Ball
authors.forename: Michael A
authors.initials: MA
authors.lastname: Kraut
authors.forename: James F
authors.initials: JF
authors.lastname: Casella
authors.forename: Nomazulu
authors.initials: N
authors.lastname: Dlamini
authors.forename: Rebecca N
authors.initials: RN
authors.lastname: Ichord
authors.forename: Lori C
authors.initials: LC
authors.lastname: Jordan
authors.forename: Fenella J
authors.initials: FJ
authors.lastname: Kirkham
authors.forename: Michael J
authors.initials: MJ
authors.lastname: Noetzel
authors.forename: E Steve
authors.initials: ES
authors.lastname: Roach
authors.forename: John J
authors.initials: JJ
authors.lastname: Strouse
authors.forename: Janet L
authors.initials: JL
authors.lastname: Kwiatkowski
authors.forename: Deborah
authors.initials: D
authors.lastname: Hirtz
authors.forename: Michael R
authors.initials: MR
authors.lastname: DeBaun
mesh_terms: D000208:Acute Disease; D000755:Anemia, Sickle Cell / Q000150:complications* / Q000453:epidemiology; D002545:Brain Ischemia / Q000175:diagnosis* / Q000453:epidemiology / Q000209:etiology; D002544:Cerebral Infarction / Q000175:diagnosis / Q000453:epidemiology / Q000209:etiology; D002648:Child; D002908:Chronic Disease; D015331:Cohort Studies; D003430:Cross-Sectional Studies; D038524:Diffusion Magnetic Resonance Imaging; D005260:Female; D006801:Humans; D015994:Incidence; D008279:Magnetic Resonance Imaging; D008297:Male; D016032:Randomized Controlled Trials as Topic; D020521:Stroke / Q000175:diagnosis / Q000453:epidemiology / Q000209:etiology*; D063128:Tertiary Health Care; D013997:Time Factors