Sorcinelli R, Sitzia A, Figus A, Lai M E
University of Cagliari, Institute of Ophthalmology, Italy.
Metab Pediatr Syst Ophthalmol (1985). 1990;13(1):23-5.
Eye examination was performed in a series of 53 patients whose mean age was 19.5 years (range from 11 to 25 years), affected from Cooley's disease, in treatment with transfusions and desferrioxamine in subcutaneous infusion. The most frequent ocular change was fundus mottling appearance like "leopard skin" (15%). We found also lens opacity (11%), drusen (7%), retinal venous tortuosity (5%), without impairment of visual acuity. The pathogenic factors of the ocular change are related to abnormality of iron metabolism. These results suggest that the involvement of desferrioxamine to remove iron from the eyeball is relatively small.
对53例患有库利氏病的患者进行了眼部检查，这些患者的平均年龄为19.5岁（年龄范围为11至25岁），正在接受输血治疗和皮下输注去铁胺治疗。最常见的眼部变化是眼底呈“豹皮”样斑纹外观（15%）。我们还发现了晶状体混浊（11%）、玻璃膜疣（7%）、视网膜静脉迂曲（5%），但视力未受损害。眼部变化的致病因素与铁代谢异常有关。这些结果表明，去铁胺从眼球中去除铁的作用相对较小。
