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Epidemiology of Stroke in Sickle Cell Disease

Epidemiology of Stroke in Sickle Cell Disease
Epidemiology of Stroke in Sickle Cell Disease
Sickle cell disease is the most common cause of stroke in childhood, both ischaemic and haemorrhagic, and it also affects adults with the condition. Without any screening or preventative treatment, the incidence appears to fall within the range 0.5 to 0.9 per 100 patient years of observation. Newborn screening with Penicillin prophylaxis and vaccination leading to reduced bacterial infection may have reduced the incidence, alongside increasing hydroxyurea prescription. Transcranial Doppler screening and prophylactic chronic transfusion for at least an initial year has reduced the incidence of stroke by up to 10-fold in children with time averaged mean of the maximum velocity >200 cm/s. Hydroxyurea also appears to reduce the incidence of first stroke to a similar extent in the same group but the optimal dose remains controversial. The prevention of haemorrhagic stroke at all ages and ischaemic stroke in adults has not yet received the same degree of attention. Although there are fewer studies, silent cerebral infarction on magnetic resonance imaging (MRI), and other neurological conditions, including headache, epilepsy and cognitive dysfunction, are also more prevalent in sickle cell disease compared with age matched controls. Clinical, neuropsychological and quantitative MRI screening may prove useful for understanding epidemiology and aetiology.
anemia, sickle cell, cerebrovascular disorders, incidence, prevalence, stroke, intracranial hemorrhage, seizures, epilepsy, headache, cognition, paraplegia, neuropathy, myopathy
2077-0383
Kirkham, Fenella Jane
1dfbc0d5-aebe-4439-9fb2-dac6503bcd58
Lagunju, Ikeoluwa A.
e0513823-b942-4309-bc8e-95a6365e39e2
Kirkham, Fenella Jane
1dfbc0d5-aebe-4439-9fb2-dac6503bcd58
Lagunju, Ikeoluwa A.
e0513823-b942-4309-bc8e-95a6365e39e2

Kirkham, Fenella Jane and Lagunju, Ikeoluwa A. (2021) Epidemiology of Stroke in Sickle Cell Disease. Journal of Clinical Medicine, 10 (18). (doi:10.3390/jcm10184232).

Record type: Review

Abstract

Sickle cell disease is the most common cause of stroke in childhood, both ischaemic and haemorrhagic, and it also affects adults with the condition. Without any screening or preventative treatment, the incidence appears to fall within the range 0.5 to 0.9 per 100 patient years of observation. Newborn screening with Penicillin prophylaxis and vaccination leading to reduced bacterial infection may have reduced the incidence, alongside increasing hydroxyurea prescription. Transcranial Doppler screening and prophylactic chronic transfusion for at least an initial year has reduced the incidence of stroke by up to 10-fold in children with time averaged mean of the maximum velocity >200 cm/s. Hydroxyurea also appears to reduce the incidence of first stroke to a similar extent in the same group but the optimal dose remains controversial. The prevention of haemorrhagic stroke at all ages and ischaemic stroke in adults has not yet received the same degree of attention. Although there are fewer studies, silent cerebral infarction on magnetic resonance imaging (MRI), and other neurological conditions, including headache, epilepsy and cognitive dysfunction, are also more prevalent in sickle cell disease compared with age matched controls. Clinical, neuropsychological and quantitative MRI screening may prove useful for understanding epidemiology and aetiology.

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jcm-10-04232 - Version of Record
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More information

Accepted/In Press date: 26 July 2021
e-pub ahead of print date: 18 September 2021
Keywords: anemia, sickle cell, cerebrovascular disorders, incidence, prevalence, stroke, intracranial hemorrhage, seizures, epilepsy, headache, cognition, paraplegia, neuropathy, myopathy

Identifiers

Local EPrints ID: 470162
URI: http://eprints.soton.ac.uk/id/eprint/470162
ISSN: 2077-0383
PURE UUID: a659dc36-69ce-43f5-ab92-a783147c3dce
ORCID for Fenella Jane Kirkham: ORCID iD orcid.org/0000-0002-2443-7958

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Date deposited: 04 Oct 2022 16:38
Last modified: 17 Mar 2024 02:53

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Author: Ikeoluwa A. Lagunju

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