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Central nervous system complications and management in sickle cell disease

Central nervous system complications and management in sickle cell disease
Central nervous system complications and management in sickle cell disease
With advances in brain imaging and completion of randomized clinical trials (RCTs) for primary and secondary stroke prevention, the natural history of central nervous system (CNS) complications in sickle cell disease (SCD) is evolving. In order of current prevalence, the primary CNS complications include silent cerebral infarcts (39% by 18 years), headache (both acute and chronic: 36% in children with sickle cell anemia [SCA]), ischemic stroke (as low as 1% in children with SCA with effective screening and prophylaxis, but ?11% in children with SCA without screening), and hemorrhagic stroke in children and adults with SCA (3% and 10%, respectively). In high-income countries, RCTs (Stroke Prevention in Sickle Cell Anemia [STOP], STOP II) have demonstrated that regular blood transfusion therapy (typically monthly) achieves primary stroke prevention in children with SCA and high transcranial Doppler (TCD) velocities; after at least a year, hydroxycarbamide may be substituted (TCD With Transfusions Changing to Hydroxyurea [TWiTCH]). Also in high-income countries, RCTs have demonstrated that regular blood transfusion is the optimal current therapy for secondary prevention of infarcts for children with SCA and strokes (Stroke With Transfusions Changing to Hydroxyurea [SWiTCH]) or silent cerebral infarcts (Silent Infarct Transfusion [SIT] Trial). For adults with SCD, CNS complications continue to be a major cause of morbidity and mortality, with no evidence-based strategy for prevention.
0006-4971
829-838
DeBaun, Michael R.
76559153-80c6-4642-bdf8-672a75570dfe
Kirkham, Fenella J.
1dfbc0d5-aebe-4439-9fb2-dac6503bcd58
DeBaun, Michael R.
76559153-80c6-4642-bdf8-672a75570dfe
Kirkham, Fenella J.
1dfbc0d5-aebe-4439-9fb2-dac6503bcd58

DeBaun, Michael R. and Kirkham, Fenella J. (2016) Central nervous system complications and management in sickle cell disease. Blood, 127 (7), 829-838. (doi:10.1182/blood-2015-09-618579). (PMID:26758917)

Record type: Article

Abstract

With advances in brain imaging and completion of randomized clinical trials (RCTs) for primary and secondary stroke prevention, the natural history of central nervous system (CNS) complications in sickle cell disease (SCD) is evolving. In order of current prevalence, the primary CNS complications include silent cerebral infarcts (39% by 18 years), headache (both acute and chronic: 36% in children with sickle cell anemia [SCA]), ischemic stroke (as low as 1% in children with SCA with effective screening and prophylaxis, but ?11% in children with SCA without screening), and hemorrhagic stroke in children and adults with SCA (3% and 10%, respectively). In high-income countries, RCTs (Stroke Prevention in Sickle Cell Anemia [STOP], STOP II) have demonstrated that regular blood transfusion therapy (typically monthly) achieves primary stroke prevention in children with SCA and high transcranial Doppler (TCD) velocities; after at least a year, hydroxycarbamide may be substituted (TCD With Transfusions Changing to Hydroxyurea [TWiTCH]). Also in high-income countries, RCTs have demonstrated that regular blood transfusion is the optimal current therapy for secondary prevention of infarcts for children with SCA and strokes (Stroke With Transfusions Changing to Hydroxyurea [SWiTCH]) or silent cerebral infarcts (Silent Infarct Transfusion [SIT] Trial). For adults with SCD, CNS complications continue to be a major cause of morbidity and mortality, with no evidence-based strategy for prevention.

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More information

Accepted/In Press date: 25 December 2015
e-pub ahead of print date: 12 January 2016
Published date: 18 February 2016
Organisations: Clinical & Experimental Sciences

Identifiers

Local EPrints ID: 390098
URI: https://eprints.soton.ac.uk/id/eprint/390098
ISSN: 0006-4971
PURE UUID: 6ff7edab-3217-4b7d-9fa6-e7e945c82913
ORCID for Fenella J. Kirkham: ORCID iD orcid.org/0000-0002-2443-7958

Catalogue record

Date deposited: 18 Mar 2016 12:36
Last modified: 10 Dec 2019 01:48

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