Elevated fetal haemoglobin levels are associated with decreased incidence of retinopathy in adults with sickle cell disease
Elevated fetal haemoglobin levels are associated with decreased incidence of retinopathy in adults with sickle cell disease
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DOI：
10.1111/bjh.15617
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发表时间：
2018-12-01
影响因子：
6.5
通讯作者：
Minniti, Caterina P.
中科院分区：
文献类型：
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作者：
Mian, Umar K.;Tang, Joyce;Minniti, Caterina P.
在镰状细胞病（SCD）的众多血管并发症中，视网膜病变最为常见，是导致失明的主要原因。羟基脲疗法可改善SCD的许多症状，并且高胎儿血红蛋白（HbF）水平已被证明可预防HbSS型儿童视网膜病变的发展。其对患视网膜病变风险高得多的成人SCD患者的影响尚未得到研究。我们旨在研究羟基脲的使用和HbF水平对成人镰状细胞视网膜病变发展的影响。我们进行了一项回顾性横断面研究，收集了2012年10月至2017年11月这5年期间在蒙特菲奥里医疗中心亨金德眼科研究所接受检查的300名成人镰状细胞患者的人口统计学资料、合并症以及眼部和血液学数据。该队列主要由18 - 71岁的所有SCD基因型的黑人和西班牙裔受试者组成。结果显示，在接受羟基脲治疗的HbSS患者中，患有视网膜病变的患者与无视网膜病变的患者相比，HbF水平显著较低（P = 0.018）。我们的研究确定了预防视网膜病变的HbF最佳临界值为14.87%。因此，HbF水平达到15%似乎是一个阈值，在此阈值之上，SS患者患视网膜病变的几率降低50%。
Among the many vascular complications of sickle cell disease (SCD), retinopathy is the most prevalent and represents a leading cause of blindness. Hydroxycarbamide therapy ameliorates many symptoms of SCD, and high fetal haemoglobin (HbF) levels have been shown to protect against the development of retinopathy in children with HbSS. Its effect on adults with SCD, who are at a much higher risk of developing retinopathy, has not been studied. We aimed to investigate the effect of hydroxycarbamide use and HbF level on sickle cell retinopathy development in adults. We performed a retrospective cross-sectional study and collected demographics, comorbidities, and ocular and haematological data from 300 adult sickle cell subjects examined at the Henkind Eye Institute at Montefiore Medical Center during a 5-year period, from October 2012 to November 2017. The cohort was comprised mainly of Black and Hispanic subjects with all SCD genotypes, aged 18-71 years. Results show that in HbSS patients treated with hydroxycarbamide, those with retinopathy had significantly lower HbF levels compared to patients without retinopathy (P = 0.018). Our study identified the optimal HbF cut-off point for retinopathy protection to be 14.87%. Thus, a HbF level of 15% appears to be the threshold above which the odds for developing retinopathy in SS patients are reduced by 50%.