Sickle cell disease: primary stroke prevention.
Stroke is an important and common complication of sickle cell disease (SCD), affecting children as well as adults. Clinically evident stroke, usually brain infarction, is usually associated with stenosis or occlusion of the intracranial arteries of the Circle of Willis, sometimes with formation of m...
| Published in: | CNS Spectrums: The International Journal of Neuropsychiatric Medicine Vol. 9; no. 6; pp. 445 - 453 |
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| Main Authors: | , |
| Format: | CEU exam questions tables/charts Journal Article |
| Published: |
Cambridge University Press
2004 Jun
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| Online Access: | View this record in EBSCOhost |
| fields | @attributes: recordID: 1 pdfLink: plink: https://search.ebscohost.com/login.aspx?direct=true&db=ccm&AN=106683865&site=ehost-live header: @attributes: shortDbName: ccm uiTerm: 106683865 longDbName: CINAHL Complete uiTag: AN controlInfo: bkinfo: dissinfo: jinfo: jid: 10928529 1XG0 jtl: CNS Spectrums: The International Journal of Neuropsychiatric Medicine issn: 10928529 maglogo: N pubinfo: dt: 2004 Jun vid: 9 iid: 6 pid: 15979 pub: Cambridge University Press artinfo: ui: 106683865 106683865 2004168910 NLM15162093 106683865 ppf: 445 ppct: 8 formats: tig: atl: Sickle cell disease: primary stroke prevention. aug: au: Gebreyohanns M Adams RJ affil: Cerebrovascular Fellow, Dept of Neurology, Medical College of Georgia, Augusta, GA sug: subj: Anemia, Sickle Cell Complications Stroke Prevention and Control Adolescence Blood Transfusion Child Child, Preschool Education, Continuing (Credit) Ultrasonography, Doppler, Transcranial Adolescent: 13-18 years Child: 6-12 years Child, Preschool: 2-5 years ab: Stroke is an important and common complication of sickle cell disease (SCD), affecting children as well as adults. Clinically evident stroke, usually brain infarction, is usually associated with stenosis or occlusion of the intracranial arteries of the Circle of Willis, sometimes with formation of moyamoya (a Japanese word for 'hazy' or 'like a puff of smoke' that describes the appearance of a abnormal microvasculature on angiography believed secondary to internal carotid artery stenosis or occlusion and the resultant extensive collateralization). Several types of intracranial hemorrhage are observed but usually in older children and adults. Cerebrovascular diseases restricted to small vessels may go unrecognized but is associated with cognitive and learning problems. Prevention of recurrent stroke has been accomplished with chronic blood transfusion. A primary prevention strategy for clinical stroke, based on the Stroke Prevention in Sickle Cell Anemia Trial, has been tested in a randomized clinical trial. Over 2,000 young children with SCD were screened with transcranial Doppler ultrasound (TCD) to detect elevated blood flow velocity indicative of vessel disease and high risk of future stroke. Those randomized to standard care (no transfusion) had a 10%/year risk of stroke, which was reduced >90% with chronic transfusion. This approach is the only primary stroke prevention strategy so far tested in SCD in a randomized controlled trial. Silent lesions on magnetic resonance imaging are associated with an approximately 1.5%/year risk of clinical stroke and a trial is now starting in children with these lesions who do not meet Stroke Prevention in Sickle Cell Anemia Trial criteria for transfusion based on TCD. A controlled trial, based on intervention for nocturnal hypoxemia, is also underway. Hydroxyurea, bone marrow transplantation, antiplatelet, and antithrombotic agents may work but have not been tested in primary prevention in a systematic way. If early and repeated, TCD screening of children, as recommended by National Heart Lung and Blood Institute and the American Stroke Association, were implemented broadly the incidence o f new strokes could be greatly reduced in these children. pubtype: Academic Journal doctype: CEU exam questions tables/charts Journal Article ougenre: Article language: English refInfo: holdings: @attributes: islocal: N |
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