{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,30]],"date-time":"2026-09-30T19:20:52Z","timestamp":1790796052492,"version":"4.1.0"},"reference-count":23,"publisher":"Wiley","issue":"4","license":[{"start":{"date-parts":[[2015,6,30]],"date-time":"2015-06-30T00:00:00Z","timestamp":1435622400000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["European J of Haematology"],"published-print":{"date-parts":[[2016,4]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>The aim of this study was to test the association between hematological\/genetic factors and cerebral vasculopathy in children with sickle cell anemia (<jats:styled-content style=\"fixed-case\">SCA<\/jats:styled-content>). A group with cerebral vasculopathy (<jats:styled-content style=\"fixed-case\">VASC<\/jats:styled-content>) was composed of children who had stroke (<jats:italic>n<\/jats:italic>\u00a0=\u00a06), silent infarct (<jats:italic>n<\/jats:italic>\u00a0=\u00a011), or an abnormal transcranial <jats:styled-content style=\"fixed-case\">D<\/jats:styled-content>oppler (<jats:italic>n<\/jats:italic>\u00a0=\u00a05). Eighty\u2010four patients had neither positive history of stroke or silent infarct, nor abnormal transcranial <jats:styled-content style=\"fixed-case\">D<\/jats:styled-content>oppler (<jats:styled-content style=\"fixed-case\">NORM<\/jats:styled-content> group). An intermediate group (<jats:styled-content style=\"fixed-case\">COND<\/jats:styled-content>;<jats:italic> n<\/jats:italic>\u00a0=\u00a015) was composed of <jats:styled-content style=\"fixed-case\">SCA<\/jats:styled-content> children with a conditional transcranial <jats:styled-content style=\"fixed-case\">D<\/jats:styled-content>oppler. Biological analyses were performed on samples obtained at steady state and before the beginning of any chronic treatment. The comparisons of the three groups demonstrated a protective effect of \u03b1\u2010thalassemia against cerebral vasculopathy through its effects on hemoglobin and reticulocyte levels. Moreover, we observed higher frequency of <jats:styled-content style=\"fixed-case\">G<\/jats:styled-content>6<jats:styled-content style=\"fixed-case\">PD<\/jats:styled-content> deficiency in the <jats:styled-content style=\"fixed-case\">VASC<\/jats:styled-content> group compared with the other groups. Our study confirms the key role of \u03b1\u2010thalassemia and <jats:styled-content style=\"fixed-case\">G<\/jats:styled-content>6<jats:styled-content style=\"fixed-case\">PD<\/jats:styled-content> status in the pathophysiology of cerebral vasculopathy in <jats:styled-content style=\"fixed-case\">SCA<\/jats:styled-content> children.<\/jats:p>","DOI":"10.1111\/ejh.12607","type":"journal-article","created":{"date-parts":[[2015,6,13]],"date-time":"2015-06-13T07:01:20Z","timestamp":1434178880000},"page":"404-408","source":"Crossref","is-referenced-by-count":45,"title":["<scp>G<\/scp>6<scp>PD<\/scp> deficiency and absence of \u03b1\u2010thalassemia increase the risk for cerebral vasculopathy in children with sickle cell anemia"],"prefix":"10.1111","volume":"96","author":[{"given":"Philippe","family":"Joly","sequence":"first","affiliation":[{"name":"Unit\u00e9 de Pathologie Mol\u00e9culaire du Globule Rouge Laboratoire de Biochimie et de Biologie Mol\u00e9culaire H\u00f4pital Edouard Herriot Hospices Civils de Lyon Lyon France"},{"name":"Laboratoire CRIS EA 647 Section\u00a0\u201cVascular Biology and Red Blood Cell\u201d Universit\u00e9 Claude Bernard Lyon 1 Lyon France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Nathalie","family":"Garnier","sequence":"additional","affiliation":[{"name":"Institut d'H\u00e9matologie et d'Oncologie P\u00e9diatrique Hospices Civils de Lyon Lyon France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kamila","family":"Kebaili","sequence":"additional","affiliation":[{"name":"Institut d'H\u00e9matologie et d'Oncologie P\u00e9diatrique Hospices Civils de Lyon Lyon France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"C\u00e9line","family":"Renoux","sequence":"additional","affiliation":[{"name":"Unit\u00e9 de Pathologie Mol\u00e9culaire du Globule Rouge Laboratoire de Biochimie et de Biologie Mol\u00e9culaire H\u00f4pital Edouard Herriot Hospices Civils de Lyon Lyon 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