A Case Control Study to Evaluate the Cognitive and Brain Function of β-thalassemia Patients.

Study Overview
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Brief Summary
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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.

Official Title
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A Case Control Study to Evaluate the Cognitive and Brain Function of β-thalassemia Patients in EMEK MEDICAL CENTER Compare to Healthy Controls.

Conditions
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Thalassemia, Brain Function (Measured by ERP Level)

Participation Criteria
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Eligibility Criteria
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Inclusion Criteria: Thalassemia major and intermedia patients older than 6 years treated at Emek Medical Center Afula Israel. healthy controls matched for age and ethnicity. Exclusion Criteria: patients suffering from acute diseases.

Ages Eligible for Study
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Minimum Age: 6 Years
Maximum Age: 70 Years

Sexes Eligible for Study
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All

Accepts Healthy Volunteers
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Accepts Healthy Volunteers

Study Plan
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How is the study designed?
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What is the study measuring?
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Primary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| Brain function assessment evaluated by Event-related potentials (ERPs of the information stream in the brain of Thalassemia patients. |  | one year | 

Secondary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| correlation between brain function evaluated by ERP measurements and Hemosiderosis parameters like ferritin levels, Iron transferrin and Saturation. |  | one year | 

 Terms related to the study
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Keywords Provided by Centre Hospitalier Valida
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Blood transfusion, Hemosiderosis, ERP