El-Alameey Inas R, Alzaree Fatma, Shehata Manal A, Shady Mones M Abu, Atti Mohamed Abdel, El-Khonezy Mohamed I
National Research Centre, Child Health Department Dokki, Giza, Cairo, Egypt.
Open Access Maced J Med Sci. 2019 Jan 16;7(3):322-328. doi: 10.3889/oamjms.2019.077. eCollection 2019 Feb 15.
Repeated blood transfusions and hemolysis in β-Thalassemia major children lead to iron overload in various organs, including the brain which may cause neurodegeneration.
To evaluate intelligence quotient in children with β-thalassemia major and healthy counterparts and to assess risk factors that cause cognitive problems.
This case-control study was performed on 50 children aged 6-16 years old with β-thalassemia major as patients group and compared with 50 healthy children as a control group of matched age, sex, and social class. Cognitive functions were evaluated by using the Wechsler Intelligence Scale for Children. Serum ferritin and iron were measured by ELISA.
There were significantly lower mean performance and full-scale IQ scores of patients group in comparison with controls, whereas no significant differences between both groups as regards to a verbal IQ score. In thalassemic children, block design, comprehension and arithmetic were negatively correlated with age of disease onset, duration of illness and onset of chelation therapy. Serum iron and ferritin were negatively correlated with similarities and digit span. Serum iron levels were negatively correlated with performance IQ score.
Children with β-thalassemia major need to receive more academic attention and cognitive assessment to improve their IQ.
重型β地中海贫血患儿反复输血和溶血会导致包括大脑在内的多个器官铁过载,这可能会引起神经退行性变。
评估重型β地中海贫血患儿及健康儿童的智商,并评估导致认知问题的危险因素。
本病例对照研究选取了50名6至16岁的重型β地中海贫血患儿作为患者组,并与50名年龄、性别和社会阶层相匹配的健康儿童作为对照组。采用韦氏儿童智力量表评估认知功能。通过酶联免疫吸附测定法检测血清铁蛋白和铁。
与对照组相比,患者组的平均操作智商和全量表智商得分显著更低,而两组在言语智商得分方面无显著差异。在重型地中海贫血患儿中,积木图案、领悟和算术与疾病发病年龄、病程及螯合治疗开始时间呈负相关。血清铁和铁蛋白与相似性和数字广度呈负相关。血清铁水平与操作智商得分呈负相关。
重型β地中海贫血患儿需要得到更多学业关注和认知评估以提高其智商。