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We assessed patient outcomes, medical care and compliance with the national guidelines published in December 2005. Data from 1033 patients (742 <jats:styled-content style=\"fixed-case\">SS<\/jats:styled-content>\/S\u03b2\u00b0\u2010thalassaemia) with 6776 patient\u2010years of follow\u2010up were analysed (mean age 7\u00b71\u00a0\u00b1\u00a03\u00b79\u00a0years). <jats:styled-content style=\"fixed-case\">SCD<\/jats:styled-content>\u2010related deaths (<jats:italic>n<\/jats:italic>\u00a0=\u00a013) occurred only in <jats:styled-content style=\"fixed-case\">SS<\/jats:styled-content>\u2010genotype patients at a median age of 23\u00b71\u00a0months, mainly due to acute anaemia (<jats:italic>n<\/jats:italic>\u00a0=\u00a05, including 2 acute splenic sequestrations) and infection (<jats:italic>n<\/jats:italic>\u00a0=\u00a03). Treatment non\u2010compliance was associated with a 10\u2010fold higher risk of <jats:styled-content style=\"fixed-case\">SCD<\/jats:styled-content>\u2010related death (<jats:italic>P\u00a0<\/jats:italic>=<jats:italic>\u00a0<\/jats:italic>0\u00b701). Therapeutic intensification was provided for all stroke patients (<jats:italic>n<\/jats:italic>\u00a0=\u00a012), almost all patients with abnormal transcranial Doppler (TCD) (<jats:italic>n<\/jats:italic>\u00a0=\u00a076) or with &gt;1\u00a0acute chest syndrome\/lifetime (<jats:italic>n<\/jats:italic>\u00a0=\u00a064) and\/or \u22653 severe vaso\u2010occlusive crises\/year (<jats:italic>n<\/jats:italic>\u00a0=\u00a0100). Only 2\/3 of patients with baseline haemoglobin &lt;70\u00a0g\/l received intensification, mainly for other severity criteria. Overall, hydroxycarbamide was under\u2010prescribed, given to 2\/3 of severe vaso\u2010occlusive patients and 1\/3 of severely anaemic patients. Nevertheless, introduction of the on\u2010line guidelines was concomitant with an improvement in medical care in the 2006\u20132009 cohort with a trend towards increased survival at 5\u00a0years, from 98\u00b73% to 99\u00b72%, significantly increased TCD coverage (<jats:italic>P\u00a0<\/jats:italic>=<jats:italic>\u00a0<\/jats:italic>0\u00b7004) and earlier initiation of intensification of therapy (<jats:italic>P\u00a0<\/jats:italic>\u2264<jats:italic>\u00a0<\/jats:italic>0\u00b701).<\/jats:p>","DOI":"10.1111\/bjh.14015","type":"journal-article","created":{"date-parts":[[2016,4,10]],"date-time":"2016-04-10T17:38:45Z","timestamp":1460309925000},"page":"927-937","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":74,"title":["Improvement of medical care in a cohort of newborns with sickle\u2010cell disease in North Paris: impact of national 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