{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,17]],"date-time":"2026-10-17T02:00:06Z","timestamp":1792202406643,"version":"4.4.0"},"reference-count":227,"publisher":"MDPI AG","issue":"18","license":[{"start":{"date-parts":[[2021,9,18]],"date-time":"2021-09-18T00:00:00Z","timestamp":1631923200000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["JCM"],"abstract":"<jats:p>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 &gt;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.<\/jats:p>","DOI":"10.3390\/jcm10184232","type":"journal-article","created":{"date-parts":[[2021,9,21]],"date-time":"2021-09-21T08:04:23Z","timestamp":1632211463000},"page":"4232","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":75,"title":["Epidemiology of Stroke in Sickle Cell Disease"],"prefix":"10.3390","volume":"10","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-2443-7958","authenticated-orcid":false,"given":"Fenella Jane","family":"Kirkham","sequence":"first","affiliation":[{"name":"Developmental Neurosciences, UCL Great Ormond Street Institute of Child Health, Faculty of Population Health Sciences, 30 Guilford Street, London WC1N 1EH, UK"},{"name":"Child Health, Clinical and Experimental Sciences, Faculty of Medicine, University of Southampton and University Hospital Southampton, Southampton SO16 6YD, UK"},{"name":"Paediatric Neurosciences, King\u2019s College Hospital, London SE5 9RS, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ikeoluwa A.","family":"Lagunju","sequence":"additional","affiliation":[{"name":"Department of Paediatrics, College of Medicine, University of Ibadan, Ibadan PMB 3017, Nigeria"},{"name":"Department of Paediatrics, University College Hospital, Ibadan PMB 5116, Nigeria"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1968","published-online":{"date-parts":[[2021,9,18]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"1","DOI":"10.1038\/ncomms1104","article-title":"Global distribution of the sickle cell gene and geographical confirmation of the malaria hypothesis","volume":"1","author":"Piel","year":"2010","journal-title":"Nat. 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