{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,19]],"date-time":"2026-09-19T13:10:01Z","timestamp":1789823401965,"version":"4.0.1"},"reference-count":32,"publisher":"Wiley","issue":"11","license":[{"start":{"date-parts":[[2018,9,21]],"date-time":"2018-09-21T00:00:00Z","timestamp":1537488000000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"funder":[{"name":"\u201cInvestissements d'avenir\u201d of the French National Research Agency","award":["ANR-11-IDEX-0005-02"],"award-info":[{"award-number":["ANR-11-IDEX-0005-02"]}]},{"name":"Laboratory of Excellence GR-Ex","award":["ANR-11-LABX-0051"],"award-info":[{"award-number":["ANR-11-LABX-0051"]}]},{"name":"D\u00e9partement de la Recherche Clinique et du D\u00e9veloppement de l'Assistance Publique-H\u00f4pitaux de Paris"},{"name":"French Ministry of Health","award":["P071228"],"award-info":[{"award-number":["P071228"]}]}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["American J Hematol"],"published-print":{"date-parts":[[2018,11]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>In order to identify very early prognostic factors that can provide insights into subsequent clinical complications, we performed a comprehensive longitudinal multi\u2010center cohort study on 57 infants with sickle cell anemia (55 SS; 2 S\u03b2\u00b0) during the first 2 years of life (<jats:ext-link xmlns:xlink=\"http:\/\/www.w3.org\/1999\/xlink\" xlink:href=\"http:\/\/clinicaltrials.gov\">ClinicalTrials.gov<\/jats:ext-link>: NCT01207037). Time to first occurrence of a severe clinical event\u2014acute splenic sequestration (ASS), vaso\u2010occlusive (VOC) event requiring hospitalization, transfusion requirement, conditional\/ abnormal cerebral velocities, or death\u2014was used as a composite endpoint. Infants were recruited at a mean age of 4.4\u2009\u00b11 months. Median follow\u2010up was 19.4 months.<\/jats:p><jats:p>During the study period, 38.6% of infants experienced \u22651 severe event: 14% ASS, 22.8%\u2009\u2265\u20091 VOC (median age: 13.4 and 12.8 months, respectively) and 33.3% required transfusion. Of note, 77% of the cohort was hospitalized, with febrile illness being the leading cause for admission. Univariate analysis of various biomarkers measured at enrollment showed that fetal hemoglobin (HbF) was the strongest prognostic factor of subsequent severe outcome. Other biomarkers measured at enrolment including absolute neutrophil or reticulocyte counts, expression of erythroid adhesion markers, % of dense red cells, cellular deformability or \u03d2\u2010globin genetic variants, failed to be associated with severe clinical outcome. Multivariate analysis demonstrated that higher Hb concentration and HbF level are two independent protective factors (adjusted HRs (95% CI) 0.27 (0.11\u20100.73) and 0.16 (0.06\u20100.43), respectively). These findings imply that early measurement of HbF and Hb levels can identify infants at high risk for subsequent severe complications, who might maximally benefit from early disease modifying treatments.<\/jats:p>","DOI":"10.1002\/ajh.25260","type":"journal-article","created":{"date-parts":[[2018,8,22]],"date-time":"2018-08-22T15:57:29Z","timestamp":1534953449000},"page":"1411-1419","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":23,"title":["Prognostic factors of disease severity in infants with sickle cell anemia: A comprehensive longitudinal cohort study"],"prefix":"10.1002","volume":"93","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-4984-754X","authenticated-orcid":false,"given":"Valentine","family":"Brousse","sequence":"first","affiliation":[{"name":"Service de P\u00e9diatrie et Maladies Infectieuses H\u00f4pital Universitaire Necker\u2010Enfants Malades  Paris France"},{"name":"UMR_S 1134 Biologie 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