重型β地中海贫血患儿心脏及肝脏铁沉积状态的多中心研究  

Liver and heart iron deposition status n patients with β thalassemia major： A multicenter study

作　　者：李长钢[1] 刘四喜[1] 王缨[1] 文飞球[1] 高红英[2] 陈光福[2] 李春富[3] 吴学东[3] 方建培[4] 郝文革 刘日阳[6] 张新华[7] 朱昭颖[8] 區永仁 

机构地区：[1]深圳市儿童医院血液肿瘤科,518026 [2]深圳市第二人民医院儿科 [3]南方医科大学附属南方医院儿科,广州 [4]中山大学孙逸仙纪念医院儿科,广州 [5]广州市妇女儿童医疗中心血液科 [6]惠州市中心人民医院儿科 [7]广西南宁解放军第三○三医院血液科 [8]香港中文大学威尔斯亲王医院放射影像科 [9]香港大学玛丽医院内科

出　　处：《中华儿科杂志》2014年第2期90-93,共4页Chinese Journal of Pediatrics

基　　金：深圳市科技计划项目（201001019）

摘　　要：目的 观察重型β地中海贫血患儿体内铁沉积状况,以制定相应的治疗对策.方法 对135例重型β地中海贫血患儿的输血、排铁的情况进行分析,检测患者血清铁蛋白（SF）水平,并运用MRI T2＊技术检测心脏及肝脏铁沉积状况.结果 本组135例,男66例,女69例,年龄6岁4个月～17岁11个月,中位年龄12.1岁.111例患儿SF为1 086.8～15 011.5 μg/L.SF＜2 000 μg/L者17例（15.3％）,SF 2 000 ～4 000 μg/L者40例（36.0％）,SF＞4 000 μg/L者54例（48.7％）.肝脏MRI T2＊检测结果：肝铁正常者8例（5.9％）,肝铁轻度沉积19例（14.1％）,中度沉积34例（25.2％）,重度沉积74例（54.8％）.重度肝铁沉积患儿中年龄最小为6岁4个月.心脏MRI T2＊检测结果：心铁正常89例（65.9％）,轻度铁沉积19例（14.1％）,重度沉积27例（20.0％）.重度心铁沉积患儿中年龄最小为9岁3个月.本组患儿SF水平与肝铁（R=-0.284,P=0.003）和心铁（R=-0.374,P=0.000）（MRI T2＊值）呈明显相关性.108例患儿因家庭经济原因未能进行规律输血及排铁治疗,且开始排铁时间较晚.结论 重型β地中海贫血患儿生存状况令人担忧,大多数患儿未能接受规范的输血及排铁治疗,肝脏及心脏重度铁沉积发生早且发生率高.Objective To observe the status of iron deposition in patient with β thalassemia major,and to formulate appropriate treatment strategies.Method The data of status of transfusion and chelation in 135 patients aged from 6 years and 4 months to 17 years and 11 months with β thalassemia major were collected and analyzed.Serum ferritin levels were determined and cardiac and hepatic iron deposition was determined using MRI T2＊ technology.Result Of the 135 cases studied,66 were male,and 69 were female,their average age was 12.1 years.Serum ferritin （SF） was determined for 111 cases,it varied from 1 086.8 μg/L to 15 011.5 μg/L.Among them,16 cases had SF level 〈 2 000 μg/L（14.5%）,in 41cases SF were between 2 000 and 4 000 μg/L （36.0%） ;in 54 cases SF 〉4 000 μg/L（48.7%）.Liver MRI T2 ＊ results showed that in only 8 cases （5.9%） iron content in the liver was in normal range,19 cases （14.9%） showed mild liver iron deposition;34（25.2%） moderate and 74 （54.8%,the youngest one was only 6 years and 4 months of age） had severe iron deposition respectively.Cardiac MRI T2 ＊ showed that in 89 cases（65.9%） iron content in the heart was in normal range; 19 cases（14.1%） had mild cardiac iron deposition and 27 （20.0%） presented severe iron deposition （the youngest one was only 9 years and 3months of age）.SF level was obviously related to liver and cardiac iron deposition （MRI T2 ＊） r and P value were-0.284,0.003 and-O.374,0.000 respectively.In 108 cases regular transfusion and chelation were delayed due to financial problem.The late and insufficient dosage administered and irregular chelation caused the higher SF level and the severe iron deposition.Conclusion The survival status of β thalassemia major in China is worrisome.Majority of them had not received regular transfusion and chelation.Liver and cardiac iron deposition occur early and had a high incidence.

关 键 词：地中海贫血 铁蛋白质类 铁沉积 

分 类 号：R725.5[医药卫生—儿科]