الفهرس 
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Abstract
S
UMMARY
-thalassemia is an inherited blood disorder resulting from reduction or total lack of β-globin chains that requires lifelong transfusion with inevitable iron overload. Neurological complications in β-thalassemia patients remain subclinical and detected only during neuropsychological, neurophysiological, or neuroimaging evaluation. Cognitive impairment, abnormal findings on evoked related potentials, complications due to extramedullary hematopoiesis, cerebrovascular disease, and peripheral neuropathy comprise the broad spectrum of neurological involvement.
Therefore, this study assessed association between central iron overload and neurocognitive function in transfusion-dependent β-thalassemia patients.
This study included 30 transfusion-dependent β-thalassemia patients (20 males and 10 females) recruited from the regular attendants of the Pediatric Hematology Clinic, Pediatric Hospital, Ain Shams University. Patients were compared with 30 age- and sex-matched healthy subjects (17 males and 13 females) enrolled as controls. The mean age of thalassemia patients was 15.56 ± 1.95 while that of controls was 15.13 ± 1.63 years. All included patients were subjected to detailed medical history and thorough clinical examination with special emphasis on anthropometric measures, disease duration,
β
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history of splenectomy, transfusion history and chelation therapy.
Liver and cardiac magnetic resonance imaging (MRI) were performed to assess LIC and cardiac T2*, respectively. Brain MRI was done and brain R2* values of both caudate and thalamic regions were calculated. Neurocognitive functions were assessed using Stanford-Binet Test. Psychological evaluation was done using Taylor Manifest Anxiety and Deck Depression Inventory.
Laboratory investigations included; CBC, liver and kidney function tests as well as analysis of LDH and indirect bilirubin as markers of hemolysis. Serum ferritin level was measured at study with calculation of the mean value of the last year prior to the study in order to know the ferritin trend.
In this study, comparison between transfusion-dependent β-thalassemia patients and healthy controls showed that 17 (56.7%) β-thalassemia had under-threshold IQ, 36.7% had anxiety and 63.3% had moderate to severe depression. The total IQ was significantly lower among β-thalassemia patients compared with healthy controls; however, no significant difference was found as regards memory IQ. Psychological assessment showed no significant difference as regards anxiety or depression between groups.
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Brain MRI showed that right and left thalamus R2* as well as right and left caudate R2* were significantly higher in β-thalassemia patients while T2* values were decreased compared with controls.
β-thalassemia patients with borderline and low average IQ had higher right and left thalamus R2* as well as right and left caudate R2* values.
Brain MRI R2* of right and left thalamus was significantly higher among thalassemia patients with delayed puberty and those with moderate and severe anxiety. Patients with moderate and severe depression had higher right thalamus R2* compared with minimal or mild depression. No significant difference was found as regards R2* of right and left caudate in relation to any of the studied clinical data and psychiatric assessment of β-thalassemia patients.
Correlation studies showed that cardiac T2* was negatively correlated to Taylor Manifest Anxiety values. Right and left thalamus R2* as well as right and left caudate R2* were negatively correlated to total IQ. Moreover, right and left caudate R2* were positively correlated to transfusion index, duration of chelation and LIC.
Of note, no significant relation was found between total IQ and clinical and laboratory data, MRI results of liver and heart or psychiatric assessment of β-thalassemia patients