Cognitive impairment in children with β-thalassemia major: influence of clinical and biochemical factors

S.İ. Açıcı, E.G. Kazancı, D. Güven, G.R. Aksoy, Ö.F. Kızılsoy, B.Z. Kral

Department of Developmental Pediatrics, University of Health Sciences, Bursa City Hospital, Bursa, Turkey. deniz.guven06@hotmail.com

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• Pediatrics

OBJECTIVE: Children with β-thalassemia major are at risk for neurocognitive impairment due to chronic anemia, iron overload, and possible neurotoxicity from chelation therapy. Despite improved transfusion and chelation protocols, the impact on cognitive function remains unclear. This study evaluated cognitive performance in children with β-thalassemia major and explored selected clinical and biochemical factors associated with cognitive scores.

MATERIALS AND METHODS: Seventeen patients aged 6-16 years with β-thalassemia major receiving regular transfusions and 17 healthy age- and sex-comparable controls were included. Demographic, anthropometric, and laboratory data (hemoglobin, ferritin, vitamin B12, and vitamin D) were collected. Cognitive performance was evaluated using the Wechsler Intelligence Scale for Children-Revised (WISC-R). Associations between cognitive scores and selected clinical and biochemical variables were evaluated as exploratory analyses, and key associations involving BMI z-score and transfusion duration were additionally adjusted for age.

RESULTS: Patients showed significantly lower verbal IQ (81.9 ± 21.9) and full-scale IQ (83.7 ± 21.0) scores compared with controls (109.1 ± 20.1 and 104.6 ± 19.6; p<0.05). All verbal and performance subtests, including picture arrangement and block design, were reduced. In exploratory analyses, age, BMI z-score, and transfusion duration were associated with selected cognitive scores; however, several of these associations were attenuated after adjustment for age. Vitamin D showed positive associations with selected verbal cognitive scores, while ferritin was not significantly associated with cognitive scores in the revised analysis.

CONCLUSIONS: Children with β-thalassemia major exhibit lower cognitive performance, especially in verbal domains. Associations with clinical and biochemical variables should be interpreted cautiously. Routine cognitive evaluation and metabolic follow-up are recommended for early detection and multidisciplinary care.

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