Thalassemia Clinical Trial
Official title:
A Case Control Study to Evaluate the Cognitive and Brain Function of β-thalassemia Patients in EMEK MEDICAL CENTER Compare to Healthy Controls.
β-thalassemia syndromes are a group of hereditary disorders characterized by a genetic
deficiency in the synthesis of beta-globin chains.
In recent studies done in β-thalassemia major patients abnormal iron deposition was evident
using MRI in brain structures, cortex, putamen, and caudate nucleus . In most of the cases
the neurological involvement is subclinical. Cognitive functioning was evaluated in beta
thalassemia major, compared with healthy controls, using a neuropsychological battery
including tests of abstract reasoning, attention, executive functions, language,
constructional/visuospatial skills, and memory. Patients with beta thalassemia major, in
particular those showing signs of hemosiderosis, had significantly impaired function in all
neuropsychological tests. There was no relationship between cognitive performances and signs
of deferoxamine toxicity, deferoxamine dosage, and levels of hemoglobin and ferritin.
Event-related potentials (ERPs) are one of the most informative and dynamic methods of
monitoring the information stream in the living brain. ERPs are linked in time with a
physical or mental event, and are typically extracted from the scalp-recorded
electroencephalogram (EEG) by means of signal averaging.
ERPs have been used in the assessment of cognitive function in several disorders, including
anemia and iron deficiency anemia. However, literature regarding cognitive function and ERP
activity in thalassemia patients is extremely limited, especially in adults.
The purpose of this study is to evaluate the cognitive and brain function in a group of 60
thalassemia patients and compare the results to healthy controls.
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