title: Does regular blood transfusion prevent progression of cerebrovascular lesions in children with sickle cell disease?
abstract: A retrospective study was conducted to assess changes in cerebrovascular lesions, as assessed by magnetic resonance (MR) imaging and angiography in 18 children with sickle cell disease (SCD) receiving optimised chronic transfusions for primary stroke prevention (abnormal transcranial Doppler flow, nine patients, median follow-up 14.3 months (range, 7.9-48.9)) or secondary stroke prevention (nine patients, median follow-up 59.6 months (range, 11.0-127.9)). An experienced neuroradiologist blinded to patient data reviewed the 41 MR scans (median/patient, three (2-4)). Standard scores were used to evaluate parenchymal and vascular abnormalities at baseline and last follow-up. Within-patient score changes evaluated using Wilcoxon's paired rank test indicated lesion progression in the secondary-prevention group (p = 0.027). Optimised transfusion therapy does not prevent progression of cerebral vasculopathy in SCD children with a history of stroke.
authors.affiliation: Reference Centre for Haemoglobinopathies, Hôpital Necker, APHP, Paris, France. valentine.brousse@nck.aphp.fr
authors.forename: Valentine
authors.initials: V
authors.lastname: Brousse
authors.forename: Lucie
authors.initials: L
authors.lastname: Hertz-Pannier
authors.forename: Yann
authors.initials: Y
authors.lastname: Consigny
authors.forename: Jean-Louis
authors.initials: JL
authors.lastname: Bresson
authors.forename: Robert
authors.initials: R
authors.lastname: Girot
authors.forename: Elsa
authors.initials: E
authors.lastname: Mirre
authors.forename: Gérard
authors.initials: G
authors.lastname: Lenoir
authors.forename: Mariane
authors.initials: M
authors.lastname: de Montalembert
mesh_terms: D000755:Anemia, Sickle Cell / Q000150:complications*; D000792:Angiography; D001803:Blood Transfusion / Q000379:methods*; D002561:Cerebrovascular Disorders / Q000209:etiology / Q000517:prevention & control*; D002648:Child; D002675:Child, Preschool; D018450:Disease Progression; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male; D012189:Retrospective Studies; D020521:Stroke / Q000209:etiology / Q000517:prevention & control