{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,28]],"date-time":"2026-09-28T19:48:44Z","timestamp":1790624924478,"version":"4.1.0"},"reference-count":71,"publisher":"Wiley","issue":"6","license":[{"start":{"date-parts":[[2016,1,5]],"date-time":"2016-01-05T00:00:00Z","timestamp":1451952000000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Br J Haematol"],"published-print":{"date-parts":[[2016,3]]},"abstract":"Summary<\/jats:title>\n \n Children with sickle cell disease (\n SCD<\/jats:styled-content>\n ) have a significant vascular morbidity, especially cerebral macrovasculopathy (\n CV<\/jats:styled-content>\n ), detectable by transcranial Doppler. This study aimed to identify risk factors for\n CV<\/jats:styled-content>\n using longitudinal biological and clinical data in a\n SCD<\/jats:styled-content>\n newborn cohort followed at the Robert Debre Reference centre (\n n<\/jats:italic>\n \u00a0=\u00a0375\n SS<\/jats:styled-content>\n \/S\u03b2\n 0<\/jats:sup>\n ). Median follow\u2010up was 6\u00b78\u00a0years (2677 patient\u2010years). Among the 59 children presenting with\n CV<\/jats:styled-content>\n , seven had a stroke. Overall, the incidence of\n CV<\/jats:styled-content>\n was 2\u00b720\/100 patient\u2010years [95% confidence interval (\n 95%<\/jats:sub>\n CI<\/jats:styled-content>\n ): 1\u00b764\u20132\u00b776] and the incidence of stroke was 0\u00b726\/100 patient\u2010years (\n 95%<\/jats:sub>\n CI<\/jats:styled-content>\n : 0\u00b707\u20130\u00b746). The cumulative risk of\n CV<\/jats:styled-content>\n by age 14\u00a0years was 26\u00b70% (\n 95%<\/jats:sub>\n CI<\/jats:styled-content>\n : 20\u00b70\u201033\u00b73%). Risk factors for\n CV<\/jats:styled-content>\n were assessed by a Cox model encompassing linear multivariate modelling of longitudinal quantitative variables. Years per upper\u2010airway obstruction [Hazard ratio (\n HR<\/jats:styled-content>\n )\u00a0=\u00a01\u00b747;\n 95%<\/jats:sub>\n CI<\/jats:styled-content>\n : 1\u00b705\u20132\u00b706] or bronchial obstruction (\n HR<\/jats:styled-content>\n \u00a0=\u00a01\u00b776; 95%\n CI<\/jats:styled-content>\n : 1\u00b749\u20132\u00b708) and reticulocyte count (\n HR<\/jats:styled-content>\n \u00a0=\u00a01\u00b782 per 50\u00a0\u00d7\u00a010\n 9<\/jats:sup>\n \/l increase;\n 95%<\/jats:sub>\n CI<\/jats:styled-content>\n : 1\u00b710\u20133\u00b701) were independent risk factors whereas fetal haemoglobin level (\n HR<\/jats:styled-content>\n \u00a0=\u00a00\u00b768 per 5% increase;\n 95%<\/jats:sub>\n CI<\/jats:styled-content>\n : 0\u00b748\u20130\u00b796) was protective. Alpha\u2010thalassaemia was not protective in multivariate analysis (ancillary analysis\n n<\/jats:italic>\n \u00a0=\u00a0209). Specific treatment for upper or lower\u2010airway obstruction and indirect targeting of fetal haemoglobin and reticulocyte count by hydroxycarbamide could potentially reduce the risk of\n CV<\/jats:styled-content>\n .\n <\/jats:p>","DOI":"10.1111\/bjh.13916","type":"journal-article","created":{"date-parts":[[2016,1,5]],"date-time":"2016-01-05T09:14:32Z","timestamp":1451985272000},"page":"966-977","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":36,"title":["Clinical and haematological risk factors for cerebral macrovasculopathy in a sickle cell disease newborn cohort: a prospective study"],"prefix":"10.1111","volume":"172","author":[{"given":"Julie","family":"Sommet","sequence":"first","affiliation":[{"name":"U 1123 ECEVE H\u00f4pital Robert\u2010Debr\u00e9 INSERM Paris France"},{"name":"UMR\u2010S 1123 ECEVE Sorbonne Paris Cit\u00e9 Univ Paris Diderot Paris France"},{"name":"H\u00f4pital Robert\u2010Debr\u00e9 Unit\u00e9 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