title: Protection from sickle cell retinopathy is associated with elevated HbF levels and hydroxycarbamide use in children.
abstract: Elevated foetal haemoglobin (HbF) levels are protective against some manifestations of sickle cell anaemia but the impact on retinopathy is unknown. We report on 123 children with HbSS, 10.6% of whom developed retinopathy. Independent of hydroxycarbamide, children with a HbF <15% had 7.1-fold (95% confidence interval, 1.5-33.6) higher odds of developing retinopathy. In children treated with hydroxycarbamide, those with retinopathy had lower HbF levels compared to children without retinopathy (9% vs. 16%; P = 0.005). We report a protective benefit of elevated HbF regarding retinopathy, and our data suggests induction of HbF with hydroxycarbamide may prevent retinopathy in children.
authors: Department of Hematology, St. Jude Children's Research Hospital, Memphis, TN, USA. jeremie.estepp@stjude.org
authors: Jeremie H
authors: JH
authors: Estepp
authors: Matthew P
authors: MP
authors: Smeltzer
authors: Winfred C
authors: WC
authors: Wang
authors: Mary E
authors: ME
authors: Hoehn
authors: Jane S
authors: JS
authors: Hankins
authors: Banu
authors: B
authors: Aygun
mesh_terms: D000293:Adolescent; D000755:Anemia, Sickle Cell / Q000150:complications* / Q000188:drug therapy; D000986:Antisickling Agents / Q000627:therapeutic use*; D002648:Child; D004341:Drug Evaluation; D005260:Female; D005319:Fetal Hemoglobin / Q000032:analysis* / Q000096:biosynthesis / Q000235:genetics; D005500:Follow-Up Studies; D005786:Gene Expression Regulation / Q000187:drug effects; D006801:Humans; D006918:Hydroxyurea / Q000627:therapeutic use*; D015994:Incidence; D008297:Male; D012164:Retinal Diseases / Q000097:blood / Q000453:epidemiology / Q000209:etiology / Q000517:prevention & control*; D012189:Retrospective Studies; D012306:Risk; D018630:Vitreoretinopathy, Proliferative / Q000097:blood / Q000453:epidemiology / Q000209:etiology / Q000517:prevention & control
chemical_list: D000986:Antisickling Agents; D005319:Fetal Hemoglobin; D006918:Hydroxyurea