Ocular Abnormalities in Patients with β Thalassemia

1989 
We examined 29 patients with homozygous β 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 β thalassemia, major and intermedia. The frequency of the ocular abnormalities increased with age. The youngest patient with an ocular abnormality was 6 ½ 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.
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