Assessment of Cognitive Function in Children With Beta-Thalassemia Major

Abstract

Multiple risk factors contribute to cognitive impairment in children with β-thalassemia major. For a more refined understanding of this issue, we attempted to evaluate cognitive function in β-thalassemia major patients and identify the relationship between possible cognitive dysfunction and the following: demography, transfusion and chelation characteristics, iron overload, and disease complications. We studied 100 β-thalassemia major children and 100 healthy controls who matched well in terms of age, sex, and socioeconomic status. All participants underwent psychometric assessment using Wechsler Intelligence Scale for Children-Third Edition, Arabic version. The mean Full-Scale IQ and Performance IQ of patients were significantly lower than those of controls, whereas no significant difference was found for Verbal IQ. No significant relationship existed between IQ and any of the assessed parameters. We concluded that Performance IQ, not Verbal IQ, was significantly affected in β-thalassemia major patients, but there was no clear association between IQ and any of the parameters.

Executive function and neural activation in adults with β‐thalassemia major: an event‐related potentials study

“…A few studies have evaluated neurocognitive function in β‐TM patients as compared with healthy controls, but these have yielded controversial results. Moreover, almost all of those studies were conducted on children and adolescents …”

Serum ferritin, serum nitric oxide, and cognitive function in pediatric thalassemia major

“…Similarly, Raafat et al reported that 4 (0.04%) patients with ß-thalassemia major had superior IQ scores and 20 (0.2%) patients had high average IQ scores. 15 We observed a significant correlation between serum nitric oxide levels and performance IQ, but no significant correlation with verbal or full scale IQ ( Table 4). The pathology of thalassemia disease includes accelerated destruction of nitric oxide and limited compensation processes to increase nitric oxide production.…”

“…19 Similarly, Raafat et al showed no correlation between serum ferritin level and full-scale IQ, verbal IQ, or performance IQ. 15 However, Retnani found a correlation between serum ferritin level and performance IQ, but no significant correlation between serum ferritin level and verbal or full scale IQ. 21 The limitations of this study were the lack of comparative assessment of serum nitric oxide levels in healthy subjects, and that the high levels of serum ferritin cannot be used to evaluate iron deposition in tissue, especially in the brain.…”

Hubungan Kadar Feritin Serum dengan Fungsi Kognitif Berdasarkan Pemeriksaan Status Mini-Mental (MMSE) pada Penyandang Thalassemia Anak

“…4 Besi bebas atau non transferrin bound iron (NTBI) bersifat toksik terhadap organ tubuh melalui reaksi fenton menghasilkan radikal bebas yang dapat menyebabkan stres oksidatif dan mempercepat degenerasi sel. [5][6][7] Penyandang thalassemia memiliki risiko tiga kali lipat untuk memiliki gangguan fungsi kognitif dan intelektual sehingga sering mengalami masalah dalam lingkungan sosial, pekerjaan dan komunikasi. 8 Fungsi kognitif dipengaruhi oleh faktor biologis dan lingkungan.…”

“…12,13 Pemeriksaan fungsi kognitif pada penyandang thalassemia seharusnya dilakukan secara rutin sehingga apabila dicurigai atau didapatkan adanya gangguan kognitif, dapat segera diberikan intervensi. 7 Penapisan fungsi kognitif dengan pemeriksaan status mini mental (MMSE) dapat digunakan klinik. Tes MMSE memiliki senisitivitas dan spesifisitas yang baik dalam menapis gangguan fungsi kognitif.…”