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Protection from sickle cell retinopathy is associated with elevated HbF levels and hydroxycarbamide use in children
Author(s) -
Estepp Jeremie H.,
Smeltzer Matthew P.,
Wang Winfred C.,
Hoehn Mary E.,
Hankins Jane S.,
Aygun Banu
Publication year - 2013
Publication title -
british journal of haematology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.907
H-Index - 186
eISSN - 1365-2141
pISSN - 0007-1048
DOI - 10.1111/bjh.12238
Subject(s) - hydroxycarbamide , retinopathy , medicine , fetal hemoglobin , hemoglobinopathy , odds ratio , pediatrics , hemolytic anemia , endocrinology , chemotherapy , pregnancy , fetus , biology , genetics , diabetes mellitus
Summary 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 H b SS , 10·6% of whom developed retinopathy. Independent of hydroxycarbamide, children with a H b F <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.