【作者】 张媛；

【导师】 陈光福；

【作者基本信息】 深圳大学 ， 儿科（专业学位）， 2020， 硕士

【摘要】 目的:铁过载导致器官功能障碍是重型β-地中海贫血(β-TM)患者常见的临床问题。本研究应用MRI T2*技术评估β-TM患者脑组织(尾状核头部、背侧丘脑、豆状核、半卵圆中心)铁含量,同时对符合年龄要求的患儿进行神经心理行为检查,探讨β-TM患儿脑组织铁含量与神经心理行为的关系。方法:选取深圳市第二人民医院儿科收治住院的长期规律输血的β-TM患者共42例,收集年龄、性别、血清铁蛋白、首次输血年龄、祛铁方式等临床资料,于2019年1月至2020年1月应用MRI T2*检测患者双侧尾状核头部、背侧丘脑、豆状核、半卵圆中心等脑组织T2*值,并对16岁以下患儿进行儿童韦氏智力测验(WISC)、Conners问卷(父母版)、儿童数字划消注意力测试(DCT)、儿童自我意识测试等神经心理行为评估,分析β-TM患儿脑组织铁含量对神经心理行为的影响。结果:1.β-TM患者脑组织中不同部位T2*值比较不同部位脑组织双侧均值T2*值由低到高依次为半卵圆中心、豆状核、背侧丘脑、尾状核头部,除半卵圆中心与豆状核T2*值无统计学差异,其余脑组织之间T2*值差异均有统计学意义;单因素分析时年龄与半卵圆中心、背侧丘脑、豆状核T2*值呈负相关(r=-0.432、-0.468、-0.517,P<0.05),与尾状核T2*值的相关性无统计学意义;多因素分析时年龄、性别、血清铁蛋白、首次输血年龄、祛铁方式等与脑组织中不同部位T2*值的相关性均无统计学意义(P>0.05)。2.β-TM患儿神经心理行为测试结果患儿总智商95±7.93分,其中言语商(9.77±2.97)高于操作商(6.70±2.07)(P<0.05);DCT平均58.5±10.6分,失误率0.50±0.23;自我意识测评正常4例,异常18例,异常占81.82%,平均分54.78±11.7分;Conners评分均正常。3.β-TM患儿不同部位脑组织T2*值与神经心理行为的相关性β-TM患儿自我意识与半卵圆中心T2*值有关,自我意识异常组的T2*值低于正常组的T2*值(t=4.10,P<0.05),与其余脑组织T2*值的相关性无统计学意义;智商、注意力失误率与各部位脑组织T2*值的相关性均无统计学意义。4.β-TM患儿神经心理行为多因素分析年龄、性别、SF、首次输血年龄、祛铁方式等与智商、注意力失误率、自我意识测试等多因素分析,注意力失误率与年龄呈负相关(r=-5.849;P<0.001),其余均无统计学意义。结论:1.本研究结果显示β-TM患者脑组织铁含量由高到低依次为半卵圆中心>豆状核>背侧丘脑>尾状核头部,提示β-TM患者深部白质铁含量明显高于深部核团。2.β-TM患儿存在神经心理行为异常,表现为注意力欠佳、自我意识异常。3.β-TM患儿半卵圆中心T2*与自我意识结果有关,提示β-TM患儿半卵圆中心铁含量过多使其功能受损,从而影响患儿情感,出现自我意识异常。更多还原

【Abstract】 Objective:Organ dysfunction caused by iron overload is a common clinical problem in patients withβ-thalassemia major(β-TM).In this study,the MRI T2*technique was used to assess the iron content of the brain tissue(caudate nucleus,dorsal thalamus,lenticular nucleus,and centrum ovale)of patients withβ-TM.At the same time,neuropsychological behaviortests were performed on children who met the age To explore the relationship between iron content in brain tissue and neuropsychological behavior of children withβ-TM.Method:42 cases of patients with long-term regular blood transfusions ofβ-TM patients admitted to the Pediatrics Department of the Second People’s Hospital of Shenzhen wereselected to collect clinical data such as age,gender,serum ferritin(SF),age of first blood transfusion,and the way of iron chelation therapy,from January 2019 to 2020 In January,MRI T2*was used to detect the T2*value of the brain tissue of the patient’s bilateralcaudate nucleus,dorsal thalamus,lenticular nucleus,and centrum ovale,while the patients who under 16 years of age underwent Weschler intelligence scale for children(WISC),Conners questionnaire(parental version),children’s digit cancellation text(DCT)andchildren’s self-concept scale,and neuropsychological behavior assessments,to analyze the effect of iron content in brain tissue on neuropsychological behavior in children withβ-TM.Results:1.Comparison of T2*values in different parts of brain tissue in patients withβ-TM.The average T2*values of the brain tissues in different parts from low to high are the centrum ovale,lenticular nucleus,dorsal thalamus,and caudate nucleus head.There were significant difference in the T2*value between the remaining brain tissue,except for the T2*value of the centrum ovale and the lenticular nucleus.In the single factor analysis,age was negatively correlated with the T2*values of the centrum ovale,dorsal thalamus,andlenticular nucleus(r=-0.432,-0.468,-0.517,P<0.05),the correlation with caudate nucleus T2*is not statistically significant;While multi-factor analysis,age,gender,serum ferritin,age of first blood transfusion,and the way of iron chelation therapy was not significant correlation values of T2*in different parts of brain tissue(P>0.05).2.Neuropsychological behavior test results of children withβ-TMThe mean scores of the patients’total IQ was 95±7.93,of which the verbal IQ(9.77±2.97)was higher than the performance IQ(6.70±2.07)(P<0.05).The mean score of DCT was 58.5±10.6,and the error rate of it was 0.50±0.23.Self-awareness scale was normal in 4cases and abnormal in 18 cases,with abnormality accounting for 81.82%,with an average score of 54.78±11.7.Conners scores were normal.3.Correlation between T2*values of different parts of brain tissue and neuropsycho-logical behavior in children withβ-TMT2*in the centrum ovale was related to the results of self-awareness of children withβ-TM,and the value of T2*in the abnormal group was lower than that in the normal group(t=4.10,P<0.05),but no statistically significant compare to T2*values in other brain tissue.There was no statistically significant in the correlation between IQ,DCT’s error rate and T2~*values in different parts brain tissues.4.Multivariate analysis of neuropsychological behavior in children withβ-TMMultiple factors,such as age,gender,SF,age of the first blood transfusion,the way of iron chelation therapy,with IQ,DCT’s error rate and self-awareness scale were analyzed showed that there was negatively correlated between DCT’s error rate and age(r=-5.849,P<0.001).Conclusions:1.The iron content in the brain tissue from high to low was centrum ovale>lentiform nucleus>dorsal thalamic>cauda nucleus.The results indicated that the iron content of deep white matter in patients withβ-TM was significantly higher than that of deep nuclei.2.Neuropsychological behavior abnormalities were found in children withβ-TM,such as abnormal self-awareness and poor attention.3.T2*in the centrum ovaler of children withβ-TM was related to the results ofself-awareness,suggesting that the excessive iron content in the centrum ovaler of children withβ-TM impaired its function,thereby affecting the emotion and abnormal self-awareness of children.更多还原