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Sickle cell disease and stroke

Sickle cell disease and stroke
Sickle cell disease and stroke

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.

0887-8994
Hirtz, Deborah
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Kirkham, Fenella J.
1dfbc0d5-aebe-4439-9fb2-dac6503bcd58
Hirtz, Deborah
d6909b95-95ea-4183-80a1-9a97f91cc0bf
Kirkham, Fenella J.
1dfbc0d5-aebe-4439-9fb2-dac6503bcd58

Hirtz, Deborah and Kirkham, Fenella J. (2019) Sickle cell disease and stroke. Pediatric Neurology. (doi:10.1016/j.pediatrneurol.2019.02.018).

Record type: Review

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.

Text
revision SCA AND STROKE jan 30 fjkdh refman - Accepted Manuscript
Restricted to Repository staff only until 27 February 2020.
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More information

Accepted/In Press date: 22 February 2019
e-pub ahead of print date: 27 February 2019
Additional Information: Copyright © 2019. Published by Elsevier Inc.

Identifiers

Local EPrints ID: 430126
URI: https://eprints.soton.ac.uk/id/eprint/430126
ISSN: 0887-8994
PURE UUID: f3741211-5662-4b88-a796-58e446f80259
ORCID for Fenella J. Kirkham: ORCID iD orcid.org/0000-0002-2443-7958

Catalogue record

Date deposited: 12 Apr 2019 16:30
Last modified: 20 Jul 2019 01:05

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Contributors

Author: Deborah Hirtz
Author: Fenella J. Kirkham ORCID iD

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