{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,14]],"date-time":"2026-09-14T12:41:00Z","timestamp":1789389660400,"version":"build-2803163510"},"reference-count":43,"publisher":"Wiley","issue":"2","license":[{"start":{"date-parts":[[2016,9,8]],"date-time":"2016-09-08T00:00:00Z","timestamp":1473292800000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"funder":[{"name":"ALSAC (American Lebanese Syrian Associated Charities)"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Br J Haematol"],"published-print":{"date-parts":[[2016,10]]},"abstract":"<jats:title>Summary<\/jats:title><jats:p>Silent cerebral infarction (<jats:styled-content style=\"fixed-case\">SCI<\/jats:styled-content>) is the most common neurological abnormality among children with sickle cell anaemia (<jats:styled-content style=\"fixed-case\">SCA<\/jats:styled-content>). The effect of hydroxycarbamide (also termed hydroxyurea) on the development and progression of <jats:styled-content style=\"fixed-case\">SCI<\/jats:styled-content> is unclear. We evaluated brain magnetic resonance imaging\/angiography (<jats:styled-content style=\"fixed-case\">MRI<\/jats:styled-content>\/<jats:styled-content style=\"fixed-case\">MRA<\/jats:styled-content>) in children with <jats:styled-content style=\"fixed-case\">SCA<\/jats:styled-content> receiving long\u2010term hydroxycarbamide therapy. Fifty participants (median 9\u00b74\u00a0years, range 1\u00b71\u201317\u00b73) enrolled in the Hydroxyurea Study of Long\u2010Term Effects (<jats:styled-content style=\"fixed-case\">HUSTLE<\/jats:styled-content>;<jats:styled-content style=\"fixed-case\"> NCT<\/jats:styled-content>00305175) underwent brain <jats:styled-content style=\"fixed-case\">MRI<\/jats:styled-content>\/<jats:styled-content style=\"fixed-case\">MRA<\/jats:styled-content> and laboratory evaluations before hydroxycarbamide initiation and after 3 and 6\u00a0years of treatment to maximum tolerated dose. <jats:styled-content style=\"fixed-case\">SCI<\/jats:styled-content> and vascular stenosis were evaluated. At baseline, 3 and 6\u00a0years, <jats:styled-content style=\"fixed-case\">SCI<\/jats:styled-content> were present in 19\/50 (38%), 20\/49 (41%), and 7\/17 (41%), respectively. At 3\u00a0years, one child developed a <jats:styled-content style=\"fixed-case\">SCI<\/jats:styled-content> lesion, and another progressed (single lesion to multiple). Lower haemoglobin (Hb) (80\u00a0g\/l vs. 86\u00a0g\/l, <jats:italic>P<\/jats:italic>\u00a0=\u00a00\u00b7049), fetal Hb (5\u00b70% vs. 10\u00b74%, <jats:italic>P<\/jats:italic>\u00a0&lt;\u00a00\u00b7001) and oxygen saturation (97% vs. 98%, <jats:italic>P<\/jats:italic>\u00a0=\u00a00\u00b7027) before hydroxycarbamide initiation were associated with <jats:styled-content style=\"fixed-case\">SCI<\/jats:styled-content>. No patients had vascular stenosis identified on <jats:styled-content style=\"fixed-case\">MRA<\/jats:styled-content>, transient ischaemic attack or stroke. Our data indicate that children receiving hydroxycarbamide over a 3\u2010 to 6\u2010year period have a low rate of new or worsening cerebrovascular disease. Further studies are needed to confirm that hydroxycarbamide can prevent the onset and progression of <jats:styled-content style=\"fixed-case\">SCI<\/jats:styled-content>.<\/jats:p>","DOI":"10.1111\/bjh.14235","type":"journal-article","created":{"date-parts":[[2016,9,8]],"date-time":"2016-09-08T06:17:13Z","timestamp":1473315433000},"page":"331-338","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":36,"title":["Hydroxycarbamide treatment and brain MRI\/MRA findings in children with sickle cell anaemia"],"prefix":"10.1111","volume":"175","author":[{"given":"Kerri A.","family":"Nottage","sequence":"first","affiliation":[{"name":"Department of Hematology St. Jude Children's Research Hospital Memphis TN USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Russell E.","family":"Ware","sequence":"additional","affiliation":[{"name":"Division of Hematology Cincinnati Children's Hospital Medical Center Cincinnati OH 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