Gartaganis S, Ismiridis K, Papageorgiou O, Beratis N G, Papanastasiou D
Department of Ophthalmology, University of Patras Medical School, Greece.
Am J Ophthalmol. 1989 Dec 15;108(6):699-703. doi: 10.1016/0002-9394(89)90864-7.
We examined 29 patients with homozygous beta thalassemia. The mean age of the patients was 15.6 +/- 8.9 years. Twelve patients (mean age, 02.0 +/- 10.4 years) had one or more ocular abnormalities. Five patients had degeneration of the retinal pigment epithelium, one had lens opacities, two had lens opacities and degeneration of the retinal pigment epithelium, one had vascular abnormalities and degeneration of the retinal pigment epithelium, one had angioid streaks, lens opacities, and degeneration of the retinal pigment epithelium, and two had angioid streaks and degeneration of the retinal pigment epithelium. These abnormalities were observed in patients with both forms of beta thalassemia, major and intermedia. The frequency of the ocular abnormalities increased with age. The youngest patient with an ocular abnormality was 6 1/2 years old. There was no correlation between the abnormalities observed and the serum ferritin level, the mean hematocrit value, and the dose of deferoxamine given to the patients.
我们检查了29例纯合子β地中海贫血患者。患者的平均年龄为15.6±8.9岁。12例患者(平均年龄为12.0±10.4岁)有一项或多项眼部异常。5例有视网膜色素上皮变性,1例有晶状体混浊,2例有晶状体混浊和视网膜色素上皮变性,1例有血管异常和视网膜色素上皮变性,1例有血管样条纹、晶状体混浊和视网膜色素上皮变性,2例有血管样条纹和视网膜色素上皮变性。在重型和中间型两种β地中海贫血患者中均观察到这些异常。眼部异常的发生率随年龄增加。最年轻的有眼部异常的患者为6岁半。观察到的异常与患者的血清铁蛋白水平、平均血细胞比容值以及去铁胺剂量之间无相关性。