Cognitive Status In Thalassemia

Wahida Akter, Dhananjoy Das, Gouri Saha, M. Chowdhury, Rehana Ahmed, M. Chowdhury
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Abstract

Background: Thalassemia is one of the most common chronic and genetic hematological disorder. This chronic disease challenges the individual at the physical, emotional, cognitive level and disrupts the quality of life because of persistent anemia and hypoxia. The aim of study is to assess the cognitive status in thalassemia. Material and methods: This is a cross-sectional observational type of study that was performed in Autism and Child Development Center of Chattagram Maa Shishu–O–General Hospital. Children aged 6 to 16 years with thalassemia of different catagories were enrolled as study subjects. Study period was six months. Cognitive status of this study subject was assessed by WICS-IV equipment. Results: Among the 50 study subjects, Hb E b thalassemia was the most common type of thalassemia 39(78%). 2(4%) patient took oral chelating agent, 18 (36%) were under injectable, 20(40%) were found taking both oral and injectable and 10(20%) were observed not under any iron chelation therapy. Regular iron chelation therapy was found in 22(44%) cases.10(20%) cases had normal intelligence,19(38%) cases had mild mental retardation and 21 (42%) had moderate mental retardation. Among the 10 patients who do not use any chelation therapy,8(80%) patients had moderate retardation and 2(20%) had mild mental retardation. On the other hand, among the 40 patients who were having iron chelation therapy, 10(25%) patients showed normal level of intelligence, 17(42.5%) had mild mental retardation and 13(32.5%) had moderate mental retardation. In different score of WISC-IV, it was found that working memory and processing speed were poorer than verbal comprehension and perceptual reasoning in different type of thalassemia. This study also showed poor cognition is more prominent in thalassemia major and Hb E beta thalassemia. Conclusion: Cognitive status of the study subjects who take regular iron chelation therapy is much better than those who do not take iron chelation therapy. Hence, iron chelation therapy is proved to have better cognitive outcome for thalassemia patients with iron overload. Chatt Maa Shi Hosp Med Coll J; Vol.22 (1); January 2023; Page 57-61
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地中海贫血患者的认知状况
背景:地中海贫血是最常见的慢性遗传性血液病之一。这种慢性疾病在身体、情感和认知水平上对个体构成挑战,并因持续贫血和缺氧而破坏生活质量。本研究的目的是评估地中海贫血患者的认知状况。材料与方法:这是一项横断面观察型研究,在马石蜀总医院自闭症与儿童发展中心进行。6至16岁不同类型的地中海贫血儿童被纳入研究对象。研究期为6个月。本研究对象的认知状态采用WICS-IV设备进行评估。结果:在50名研究对象中,Hb E b地中海贫血是最常见的地中海贫血类型39(78%)。口服铁螯合剂2例(4%),注射铁螯合剂18例(36%),口服和注射铁螯合剂20例(40%),未服用铁螯合剂10例(20%)。常规铁螯合治疗22例(44%),智力正常10例(20%),轻度智力迟钝19例(38%),中度智力迟钝21例(42%)。在未使用任何螯合治疗的10例患者中,8例(80%)为中度智力迟钝,2例(20%)为轻度智力迟钝。另一方面,在接受铁螯合治疗的40例患者中,智力正常10例(25%),轻度智力迟钝17例(42.5%),中度智力迟钝13例(32.5%)。在不同的WISC-IV评分中,不同类型的地中海贫血患者的工作记忆和加工速度均低于言语理解和知觉推理。本研究还显示认知障碍在重度地中海贫血和Hb E β地中海贫血中更为突出。结论:定期接受铁螯合治疗的受试者认知状况明显优于未接受铁螯合治疗的受试者。因此,铁螯合疗法被证明对铁超载的地中海贫血患者有更好的认知结果。上海医科大学医学院;工程系(1);2023年1月;57 - 61页
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