van Meurs J C
Oogziekenhuis, Vitreoretinale Afdeling, Rotterdam.
Ned Tijdschr Geneeskd. 1990 Sep 15;134(37):1800-2.
Prospectively we performed an ophthalmological examination in 81 Hb SS and 97 Hb SC patients on the island of Curaçao, about half the estimated number of patients, based on gene frequency. These patients had not been selected on ophthalmological symptoms. Proliferative retinopathy was present in 2% of the Hb SS patients, and in 50% of the Hb SC patients (71% among those over 18 years old). The frequency of severe visual loss (counting fingers or less) in one eye due to sickle cell disease in Hb SS patients was 1% (one patient, both eyes), and in Hb SC patients 6% (6 patients, 6 eyes). Bilateral blindness was rare: one Hb SS patient had cortical blindness. Visual loss in Hb SC patients (of which four eyes with loss of light perception) was caused by proliferative retinopathy, with its complications like vitreous haemorrhage and retinal detachment. Although the effectiveness of prophylactic laser treatment remains to be established, it would appear prudent to have an eye examination performed regularly in Hb SC patients older than 20 years.
我们前瞻性地对库拉索岛的81例血红蛋白SS型患者和97例血红蛋白SC型患者进行了眼科检查，根据基因频率估算，这些患者约占患者总数的一半。这些患者并非基于眼科症状挑选出来的。增殖性视网膜病变在血红蛋白SS型患者中的发生率为2%，在血红蛋白SC型患者中的发生率为50%（18岁以上患者中为71%）。血红蛋白SS型患者因镰状细胞病导致一只眼睛严重视力丧失（仅能数指或更差）的发生率为1%（1例患者，双眼），血红蛋白SC型患者为6%（6例患者，6只眼）。双眼失明很罕见：1例血红蛋白SS型患者患有皮质盲。血红蛋白SC型患者的视力丧失（其中4只眼无光感）是由增殖性视网膜病变及其并发症如玻璃体积血和视网膜脱离引起的。尽管预防性激光治疗的效果仍有待确定，但对20岁以上的血红蛋白SC型患者定期进行眼部检查似乎是明智的。
