Thalassemia is a commonly occurring genetic hemoglobinopathy worldwide. Periodic and routine blood transfusions, iron chelation therapy and splenectomy procedures are all required for the treatment of thalassemia. Numerous organs and bodily systems could be impacted by thalassemia, particularly the nervous system, which could impede cognitive performance. The study aimed to assess cognitive abilities of pediatric patients diagnosed with beta thalassemia major and intermedia. A total of 168 participants [54 β-thalassemic major children, 51 with β-thalassemia intermedia and 63 age-matched healthy controls from both genders (85 girls and 83 boys)] with age ranging from 8 to 16 years were participated in a cross-sectional study. Cognitive function was evaluated for all children by using the Wechsler Intelligence Scale For Children 4th edition. Compared with control group, a significant decline was found in all Wechsler Intelligence Scale subtests as well as in verbal comprehension index, perceptual reasoning index, working memory index, processing speed index and full scale index scores of thalassemia major and intermedia patients (p < 0.001). When compared to children with thalassemia intermedia, the beta thalassemic major children showed a significant decrease in all Wechsler Scale subtests as well as in all its five index scores (p < 0.001). The study concluded that thalassemia negatively affects and impairs cognition and intellectual capacities.

中文翻译：

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重型和中型β地中海贫血儿科患者的认知障碍：一项横断面研究

地中海贫血是世界范围内常见的遗传性血红蛋白病。地中海贫血的治疗需要定期和常规输血、铁螯合疗法和脾切除手术。许多器官和身体系统可能会受到地中海贫血的影响，特别是神经系统，这可能会妨碍认知能力。该研究旨在评估被诊断为重型和中间型β地中海贫血的儿科患者的认知能力。共有 168 名参与者（54 名重型 β 地中海贫血儿童、51 名中间型 β 地中海贫血患者和 63 名年龄匹配的男女健康对照者（85 名女孩和 83 名男孩））参加，年龄范围为 8 至 16 岁。 -截面研究。使用韦克斯勒儿童智力量表第四版评估所有儿童的认知功能。与对照组相比，重度和中度地中海贫血患者的所有韦氏智力量表分项测试以及语言理解指数、知觉推理指数、工作记忆指数、处理速度指数和全量表指数评分均显着下降（p < 0.001） ）。与中间型地中海贫血儿童相比，重型β地中海贫血儿童的所有韦氏量表子测试及其所有五个指数得分均显着下降（p < 0.001）。该研究得出的结论是，地中海贫血会对认知和智力产生负面影响和损害。