核磁共振功能成像T2~*对重型β-地中海贫血患儿脑组织铁过载的评价

Evaluation of nuclear magnetic resonance imaging T2~* for iron overload in the brain tissue in patients with severe beta thalassemia

目的分析核磁共振功能成像T2~*在重型β-地中海贫血患儿脑组织铁过载的评价中的应用价值。方法 2009年1月至2016年9月深圳市第二人民医院儿科收治住院的长期高量输血的重型β-地中海贫血患儿100例,为观察组,同期选取进行核磁共振成像T2~*检查的健康体检者25例为对照组。根据血清铁蛋白水平将观察组分为两个亚组:轻度组(1.0~2.5g/L)57例,重度组(>2.5g/L)43例。观察组及对照组均行核磁共振功能成像T2~*检查,比较两组脑组织不同部位T2~*值。比较轻度组与重度组脑组织T2~*及脑脊液铁代谢蛋白水平并进行相关性分析。结果观察组脑组织不同部位T2~*值均显著低于对照组,差异有统计学意义(P<0.05),且观察组脑组织不同部位T2~*值从低到高依次为苍白球<红核<黑质<壳核<齿状核<尾状核<灰质<白质<小脑。重度组脑组织T2~*值、转铁蛋白、亚铁转运膜蛋白1、血红素氧化酶1显著低于轻度组,铁蛋白、铜蓝蛋白、铁调素显著高于轻度组,差异有统计学意义(P<0.05)。转铁蛋白、亚铁转运膜蛋白1、血红素氧化酶1与T2~*值呈正相关(r=0.309、0.617、0.326,P<0.05),与铁蛋白、铜蓝蛋白、铁调素呈负相关(r=-0.512、-0.474、-0.639,P<0.05)。结论核磁共振成像T2~*技术有助于评价β-地中海贫血患儿脑组织铁过载及评估疾病程度,值得临床上推广应用。

Objective To analyze the application values of nuclear magnetic resonance functional imaging T2^＊ in evaluation of iron overload in brain tissue in patients with severe beta thalassemia. Methods Totally 100 cases of patients with severe beta thalassemia receiving long-term high-volume transfusion who accepted treatments and were hospitalized in the Second People＇s Hospital of Shenzhen City of Department of Pediat- rics from January 2009 to September 2016 were included as the observation group, and another 25 cases of healthy subjects who underwent magnetic resonance imaging （T2^＊ ） examination were selected as control group. According to serum ferritin level, the observation group were divided into two subgroups： mild group （1.0 to 2.5 g/L） of 57 cases, severe group （〉2.5 g/L） of 43 cases. Then, the observation group and the control group underwent magnetic resonance imaging T2^＊ examination, and the T2^＊ values of dif- ferent parts of brain tissue were compared between the two groups; the levels of T2^＊ and CSF iron metabo- lism protein in brain tissue of mild group and severe group were compared and analyzed. Results The T2^＊ values of different parts of brain tissue in the observation group were significantly lower than those of the control group, the difference being statistically significant （P〈0.05）. T2^＊ values of different parts of brain tissue in the observation group were （from low to high） ：globus pallidus〈corpora rubrum〈lack substance〈dorsal caudate putamen〈corpora dentatum〈caudate nucleus〈gray matter〈leukoencephalopathy〈cerebel-la. The T2^＊ value of brain tissue, transferrin, ferrous transporter membrane protein 1 and heme oxidase-1 in the severe group were significantly lower those in the mild group（P〈0.05） ,and the ferritin, eeruloplas- rain and hepeidin were significantly higher than the mild group（P〈0.05）. The transferrin, ferrous transporter membrane protein 1, heme oxidase-1 and T2^＊ values showed positive correlation （r = 0. 309,0. 617,0. 326, P 0. 05）, while the ferritin, ceruloplasmin and hepcidin showed negative correlation with T2^＊ （r=-0. 512, - 0.474, -0. 639 ,P〈0.05）. Conehlsion The nuclear magnetic resonance imaging T2^＊ technology is helpful to eval- uate the iron overload in brain tissue and assess the degree of disease in patients with beta-TM, thus it is worthy of being clinieally promoted and applied.