Beta-Thalassemia Clinical Trial
Official title:
Evaluation of Choroidal Thickness in Patients Suffering From Beta-thalassemia
The purpose of this study is to examine choroidal thickness in beta-thalassemia patients and
compare it to the one of healthy controls.
An equal number of transfusion dependent beta-thalassemic patients and age and sex matched
healthy volunteers will undergo spectral-domain optical coherence tomography utilizing the
enhanced depth imaging application to visualize and measure the choroid.
Beta thalassemia is an inherited hemoglobinopathy, associated with defective production of
beta-chain globin resulting in problematic HbA production. It is classified according to the
severity as minor, intermedia and major. Patients suffering from beta-thalassemia intermedia
and beta-thalassemia major require regular blood transfusions. Transfusion related
hemosiderosis in these patients necessitates the use of chelating agents to prevent iron
overload in vital organs such as the liver and heart.
A number of ocular abnormalities can present in beta-thalassemia patients. Those are
categorized as pseudoxanthoma elasticum (PXE)-like changes that include angioid streaks, peau
d'orange like fundus and optic nerve head drusen and non-PXE-like changes such as increased
venous tortuosity.
Furthermore, it is well established that prolonged treatment with some of the chelating
agents such as deferoxamine are associated with ocular toxicity, namely nyctalopia, colour
perception anomalies, visual field disturbances, cataract formation, optic neuropathy and
pigmentary retinopathy.
The pathophysiology of those ocular manifestations has not been fully clarified. The role of
the choroid has not been determined since this particular tissue was previously unaccessible
to imaging. Enhanced depth imaging optical coherence tomography is able to image the choroid
providing reasonable clarity compared to spectral domain optical coherence tomography.
The aim of this study is to evaluate possible alterations of choroidal thickness in beta
thalassemia as compared to healthy controls.
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