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Sickle Cell Disease and Stroke

Hirtz, D; Kirkham, FJ; (2019) Sickle Cell Disease and Stroke. Pediatric Neurology , 95 pp. 34-41. 10.1016/j.pediatrneurol.2019.02.018. Green open access

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Abstract

Cerebral infarction is a common complication of sickle cell disease and may manifest as overt stroke or cognitive impairment associated with “silent” cerebral infarction on magnetic resonance imaging. Vasculopathy may be diagnosed on transcranial Doppler or magnetic resonance angiography. The risk factors in sickle cell disease for cognitive impairment, overt ischemic stroke, silent cerebral infarction, overt hemorrhagic stroke, and vasculopathy defined by transcranial Doppler or magnetic resonance angiography overlap, with severe acute and chronic anemia, acute chest crisis, reticulocytosis, and low oxygen saturation reported with the majority. However, there are differences reported in different cohorts, which may reflect age, geographic location, or neuroimaging techniques, for example, magnetic resonance imaging field strength. Regular blood transfusion reduces, but does not abolish, the risk of neurological complications in children with sickle cell disease and either previous overt stroke or silent cerebral infarction or abnormal transcranial Doppler. There are relatively few data on the use of hydroxyurea or other management strategies. Early assessment of the risk of neurocognitive complications is likely to become increasingly important in the management of sickle cell disease.

Type: Article
Title: Sickle Cell Disease and Stroke
Location: United States
Open access status: An open access version is available from UCL Discovery
DOI: 10.1016/j.pediatrneurol.2019.02.018
Publisher version: https://doi.org/10.1016/j.pediatrneurol.2019.02.01...
Language: English
Additional information: This version is the author accepted manuscript. For information on re-use, please refer to the publisher’s terms and conditions.
Keywords: Cerebral blood flow, Hemorrhage, Hydroxyurea, Silent infarction, Transcranial Doppler, Transfusion therapy
UCL classification: UCL
UCL > Provost and Vice Provost Offices
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Population Health Sciences
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Population Health Sciences > UCL GOS Institute of Child Health
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Population Health Sciences > UCL GOS Institute of Child Health > Developmental Neurosciences Dept
URI: https://discovery.ucl.ac.uk/id/eprint/10073826
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