{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,30]],"date-time":"2026-09-30T04:59:57Z","timestamp":1790744397442,"version":"4.1.0"},"reference-count":37,"publisher":"Wiley","issue":"5","license":[{"start":{"date-parts":[[2013,10,10]],"date-time":"2013-10-10T00:00:00Z","timestamp":1381363200000},"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":[[2013,12]]},"abstract":"Summary<\/jats:title>In patients with sickle cell anaemia (SCA<\/jats:styled-content>), concomitant glucose\u20106\u2010phosphate dehydrogenase (G<\/jats:styled-content>6PD<\/jats:styled-content>) deficiency is usually described as having no effect and only occasionally as increasing severity. We analysed sequential clinical and biological data for the first 42\u00a0months of life in SCA<\/jats:styled-content> patients diagnosed by neonatal screening, including 27 G<\/jats:styled-content>6PD<\/jats:styled-content>\u2010deficient patients, who were matched on sex, age and parents' geographic origin to 81 randomly selected patients with normal G<\/jats:styled-content>6PD<\/jats:styled-content> activity. In the G<\/jats:styled-content>6PD<\/jats:styled-content>\u2010deficient group, steady\u2010state haemoglobin was lower (\u22126\u00b72\u00a0g\/l, 95% confidence interval (CI<\/jats:styled-content>), [\u221210\u00b71;\u00a0\u22122\u00b73]) and reticulocyte count higher (247\u00a0\u00d7\u00a0109<\/jats:sup>\/l, 95%CI<\/jats:styled-content>, [97; 397]). The acute anaemic event rate was 3 times higher in the G<\/jats:styled-content>6PD<\/jats:styled-content>\u2010deficient group (P<\/jats:styled-content>\u00a0<\/jats:italic><\u00a010\u22123<\/jats:sup>). A higher proportion of G6PD<\/jats:styled-content>\u2010deficient patients required blood transfusion (20\/27 [74%] vs. 37\/81 [46%], P<\/jats:styled-content>\u00a0<\/jats:italic><\u00a010\u22123<\/jats:sup>), for acute anaemic events, and also vaso\u2010occlusive and infectious events. No significant between\u2010group differences were found regarding the rates of vaso\u2010occlusive, infectious, or cerebrovascular events. G<\/jats:styled-content>6PD<\/jats:styled-content> deficiency in babies with SCA<\/jats:styled-content> worsens anaemia and increases blood transfusion requirements in the first years of life. These effects decrease after 2\u00a0years of age, presumably as the decline in fetal haemoglobin levels leads to increased sickle cell haemolysis and younger red blood cells with higher G<\/jats:styled-content>6PD<\/jats:styled-content> activity.<\/jats:p>","DOI":"10.1111\/bjh.12590","type":"journal-article","created":{"date-parts":[[2013,10,12]],"date-time":"2013-10-12T02:45:13Z","timestamp":1381545913000},"page":"646-654","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":34,"title":["Impact of glucose\u20106\u2010phosphate dehydrogenase deficiency on sickle cell anaemia expression in infancy and early childhood: a prospective study"],"prefix":"10.1111","volume":"163","author":[{"given":"Malika","family":"Benkerrou","sequence":"first","affiliation":[{"name":"AP\u2010HP H\u00f4pital Robert Debr\u00e9 Service d'H\u00e9matologie P\u00e9diatrique Paris France"},{"name":"Centre de R\u00e9f\u00e9rence de la Dr\u00e9panocytose Paris France"},{"name":"Inserm U763 Paris France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Corinne","family":"Alberti","sequence":"additional","affiliation":[{"name":"AP\u2010HP H\u00f4pital Robert Debr\u00e9 Unit\u00e9 d'Epid\u00e9miologie Clinique Paris France"},{"name":"Univ Paris Diderot Sorbonne Paris Cit\u00e9 Paris France"},{"name":"Inserm CIE 5 Paris France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Nathalie","family":"Couque","sequence":"additional","affiliation":[{"name":"AP\u2010HP H\u00f4pital Robert Debr\u00e9 G\u00e9n\u00e9tique Mol\u00e9culaire et Biochimie Paris France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Zinedine","family":"Haouari","sequence":"additional","affiliation":[{"name":"AP\u2010HP H\u00f4pital Robert Debr\u00e9 Service d'H\u00e9matologie P\u00e9diatrique Paris France"},{"name":"Centre de R\u00e9f\u00e9rence de la Dr\u00e9panocytose Paris France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Aissatou","family":"Ba","sequence":"additional","affiliation":[{"name":"AP\u2010HP H\u00f4pital Robert Debr\u00e9 Service d'H\u00e9matologie P\u00e9diatrique Paris France"},{"name":"Centre de R\u00e9f\u00e9rence de la Dr\u00e9panocytose Paris France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Florence","family":"Missud","sequence":"additional","affiliation":[{"name":"AP\u2010HP H\u00f4pital Robert Debr\u00e9 Service d'H\u00e9matologie P\u00e9diatrique Paris France"},{"name":"Centre de R\u00e9f\u00e9rence de la Dr\u00e9panocytose Paris France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Priscilla","family":"Boizeau","sequence":"additional","affiliation":[{"name":"AP\u2010HP H\u00f4pital Robert Debr\u00e9 Unit\u00e9 d'Epid\u00e9miologie Clinique Paris France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Laurent","family":"Holvoet","sequence":"additional","affiliation":[{"name":"AP\u2010HP H\u00f4pital Robert Debr\u00e9 Service d'H\u00e9matologie P\u00e9diatrique Paris France"},{"name":"Centre de R\u00e9f\u00e9rence de la Dr\u00e9panocytose Paris France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ghislaine","family":"Ithier","sequence":"additional","affiliation":[{"name":"AP\u2010HP H\u00f4pital Robert Debr\u00e9 Service d'H\u00e9matologie P\u00e9diatrique Paris France"},{"name":"Centre de R\u00e9f\u00e9rence de la Dr\u00e9panocytose Paris France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jacques","family":"Elion","sequence":"additional","affiliation":[{"name":"Inserm U763 Paris France"},{"name":"Univ Paris Diderot Sorbonne Paris Cit\u00e9 Paris France"},{"name":"AP\u2010HP H\u00f4pital Robert Debr\u00e9 G\u00e9n\u00e9tique Mol\u00e9culaire et Biochimie Paris France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Andr\u00e9","family":"Baruchel","sequence":"additional","affiliation":[{"name":"AP\u2010HP H\u00f4pital Robert Debr\u00e9 Service d'H\u00e9matologie P\u00e9diatrique Paris France"},{"name":"Univ Paris Diderot Sorbonne Paris Cit\u00e9 Paris France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Rolande","family":"Ducrocq","sequence":"additional","affiliation":[{"name":"Inserm U763 Paris France"},{"name":"Univ Paris Diderot Sorbonne Paris Cit\u00e9 Paris France"},{"name":"AP\u2010HP H\u00f4pital Robert Debr\u00e9 G\u00e9n\u00e9tique Mol\u00e9culaire et Biochimie Paris France"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2013,10,10]]},"reference":[{"key":"e_1_2_8_2_1","doi-asserted-by":"publisher","DOI":"10.1056\/NEJM199807023390102"},{"key":"e_1_2_8_3_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2011-03-325258"},{"key":"e_1_2_8_4_1","unstructured":"ANSM(2008)M\u00e9dicaments et d\u00e9ficit en G6PD. 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