Study of Beta-Thalassemia Biomarkers and Their Relationship to Cognition Among Children

Abstract

The current study is to detect some biomarkers of beta-thalassemia (ferritin, serum transferrin receptors, and nitric oxide levels) and to examine the relation between these markers and cognition in children with beta-thalassemia. Thirty children with beta-thalassemia were selected from the Pediatric Department at Assiut University hospital. Another 40 healthy children of the same age, sex, years of schooling, body mass index (BMI), and social scale were chosen as the control group. Assessment of clinical, laboratory, cognitive functions, and event related potential was done for patients and control groups. Significantly higher levels of ferritin and serum transferrin receptors with decreased nitric oxide were detected among children with beta-thalassemia. There were significant correlations between serum transferrin receptors and nitric oxide levels with event related potential latencies and with some cognitive function tests and P300-N2 amplitude. Frequent blood transfusion was associated with increased serum transferrin receptors and decreased nitric oxide levels.

Brain functional impairment in beta‐thalassaemia: the cognitive profile in Italian neurologically asymptomatic adult patients in comparison to the reported literature

“…Adult TDT patients were investigated in 2/15 studies (Monastero et al , ; Raz et al , ) and the four above mentioned studies also included a minimal proportion of adult patients (Orsini et al , ; Logothetis et al , ; Aydin et al , ; Karimi et al , ). All studies but one (Teli et al , ) recruited TDT patients (in two studies the recruited population was probably mixed as the transfusion rate ranged from 1 to 12 per year; Shehata et al , ,). The sample size ranged widely, from 12 (Orsini et al , ) to 294 (Karimi et al , ) (median: 30 patients) The cognitive evaluation was also heterogeneous: Wechsler Intelligence Scale for Children (WISC) and WAIS (or their subtests) were applied in 10/15 studies.…”

HbE/β-Thalassemia and Oxidative Stress: The Key to Pathophysiological Mechanisms and Novel Therapeutics

“…Lower blood levels of the endogenous vasodilator NO are reported in thalassemia (162,180). Plasma circulating cellfree hemoglobin decreases NO bioavailability from endothelial cells by oxidizing NO to nitrate.…”

Cognitive functions in adults with β‐thalassemia major: before and after blood transfusion and comparison with healthy controls

“…Shehata et al . reported a significant decline in cognitive function, as measured on the Stanford–Binet scale, in β‐TM children aged 4–17 years and 8–17 years . Poorer performance was found on several memory‐related tasks, including a bead memory test, a memory‐for‐sentences test, and total short‐term memory.…”

“…In that study, there was no clear association between IQ and demography, transfusion or chelation characteristics, iron overload, or disease complications. Shehata et al . reported a significant decline in cognitive function, as measured on the Stanford–Binet scale, in β‐TM children aged 4–17 years and 8–17 years .…”