Cognitive dysfunction in β-thalassemia major and intermedia patients and its clinical correlates


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Abstract

BackgroundIn Egypt, thalassemia is considered one of the most common genetically determined hemolytic diseases. Its high prevalence causes a significant burden on health resources. Few studies conducted on thalassemia patients have shown a heightened risk for cognitive function affection, and hence affects the patient’s quality of life.ObjectiveThe aim of this study was to assess the cognitive functions and its clinical correlates in β-thalassemia major and β-thalassemia intermedia patients in comparison with healthy controls.Patients and methodsThis study included 40 β-thalassemia patients who were divided into two groups: 20 β-thalassemia major patients and 20 β-thalassemia intermedia patients. 20 controls matched for age and sex participated in the study. Cases were collected from the Hematology Outpatient Clinic, Internal Medicine Department of Ain Shams University. All participants were subjected to a full neuropsychological battery.ResultsCompared with controls, both β-thalassemia patient groups were equally and significantly impaired on most of the neuropsychological battery domains. Serum hemoglobin, serum iron, heart abnormalities, and liver functions were nonsignificant correlates, and hepatitis C virus infection and vaccination history were significant correlates.ConclusionOur findings suggest that both thalassemia types are equally affected as regards multiple cognitive domains, with no correlation with blood transfusion or serum iron levels, questioning any role for hemosiderosis as a cause for cognitive impairment.

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