Department of Hematology, St. Jude Children's Research Hospital, Memphis, TN, USA.
Br J Haematol. 2013 May;161(3):402-5. doi: 10.1111/bjh.12238. Epub 2013 Feb 6.
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.
胎儿血红蛋白 (HbF) 水平升高可预防镰状细胞贫血的某些表现，但对视网膜病变的影响尚不清楚。我们报告了 123 名 HbSS 患儿，其中 10.6%发生了视网膜病变。独立于羟基脲，HbF<15%的儿童发生视网膜病变的几率高 7.1 倍（95%置信区间，1.5-33.6）。在接受羟基脲治疗的儿童中，有视网膜病变的儿童的 HbF 水平低于无视网膜病变的儿童（9%对 16%；P=0.005）。我们报告了 HbF 升高对视网膜病变的保护作用，我们的数据表明，用羟基脲诱导 HbF 可能预防儿童的视网膜病变。
