51例重型β珠蛋白生成障碍性贫血患儿长期输血去铁治疗与铁过载的关系研究  

Relationship of long-term blood transfusion, iron chelation therapy with iron overload in 51 children with beta-thalassaemia major

作　　者：陈光福[1] 陈娟娟[1] 高红英[1] 龙琦[1] 富国华[1] 

机构地区：[1]深圳市第二人民医院深圳大学第一附属医院儿科,广东深圳518035

出　　处：《中国实用儿科杂志》2012年第2期120-123,共4页Chinese Journal of Practical Pediatrics

摘　　要：目的探讨重型β珠蛋白生成障碍性贫血(beta-thalassaemia,简称β-TM)患儿长期输血、去铁治疗与铁过载的关系。方法深圳市第二人民医院2001年成立"地贫之友"与"地贫服务队",对β-TM患儿进行规范性的长期输血和去铁治疗。每3个月监测血清铁蛋白浓度(SF)、肝肾功能、心肌酶谱、心功能、心脏和肝脾B超、血糖和尿糖。2001年2月至2010年6月对其中51例患儿进行核磁共振检测心脏T2*、左心室射血分数(LVEF)、肝脏T2*、胰腺T2*和垂体T2*。根据治疗方法分为足疗程去铁胺+去铁酮(DFO+DFP)联合去铁治疗组(足疗程联合组)10例、不足疗程DFO+DFP联合去铁治疗组(不足疗程联合组)31例、单用足疗程地拉罗司(DFX)去铁治疗组(单用DFX组)10例。根据SF质量浓度分为SF≤2000μg/L组(A组)12例、SF^3000μg/L组(B组)17例、SF>3000μg/L组(C组)22例。结果各组LVEF、心脏T2*、垂体T2*值差异无统计学意义(P>0.05);足疗程联合组肝脏T2*高于不足疗程联合组(P<0.05),单用DFX组肝脏T2*、胰腺T2*、垂体T2*均高于足疗程联合组和不足疗程联合组(P<0.05)。足疗程联合组SF低于不足疗程联合组,单用DFX组SF低于足疗程联合组和不足疗程联合组,差异均具有统计学意义(P<0.05)。C组肝脏T2*和胰腺T2*明显低于A组和B组,差异具有统计学意义(P<0.05)。心肌铁过载11例(21.6%),肝脏铁过载43例(84.3%)。SF与心脏T2*无相关性(r=0.254,P>0.05),与肝脏T2*呈中度负相关(r=0.558,P<0.01)。结论足疗程DFO+DFP联合去铁治疗和单用足疗程DFX去铁治疗均能有效降低血清铁蛋白浓度,动员肝脏组织铁,效果优于不足疗程DFO+DFP联合去铁治疗。不同去铁方式均能减轻心脏铁过载。Objective To explore the relationship of long-term blood transfusion, iron chelation therapy with iron overload in the patients with beta-thalassaemia major. Methods The serum ferritin （SF） , liver function, renal function, myocardial enzyme, uhrasonograph of liver and spleen, blood glucose and urine glucose were detected in the patients with beta-thalassaemia major in ＂Friends of thalassemia＂ and ＂Service team for thalassemia in Shenzhen＂ with regular long-term blood transfusion and iron chelation therapy in three months. Left ventricular ejection fraction （LVEF）, myo- cardial, liver, pancreas and pituitary MR imaging T2＊ were performed on 51 patients of them. The51 patients were divid- ed into 3 groups. 10 cases with sufficient dose DFO and sufficient dose DFP iron chelation treatment group （sufficient dose joint group） ; 31 cases with insufficient DFO and DFP iron chelation treatment group （insufficient joint group） ; 10 cases with sufficient dose DFX iron chelation treatment group （DFX group）. Results There was no difference in myo- cardial T2＊, pituitary T2＊, LVEF in every group. （P 〉 0.05 ）. Liver T2＊ in sufficient dose joint group was higher than that in insufficient joint group （P 〈 0.05）. Liver T2＊, pancreas T2＊, and pituitary T2＊in DFX group was significantly higher than that in the other two groups （P 〈 0.05）. SF in sufficient dose joint group was significantly lower than that in insufficient joint group （P 〈 0.05） , SF in DFX group was significantly lower than that in the other two groups （P 〈 0.05）. Liver T2＊ and pancreas T2＊in C group were significantly lower than those in A and B groups （P 〈 0.05 ）.There was myocardial iron overload in 11 of 51 cases （21.6% ）, and there was liver iron overload in 43 of 51 cases （84.3%）. SF had no correlation with myocardial T2＊ （r = 0.254, P 〉 0.05 ）, and there was moderate negative correlation with liver T2＊ （r = 0.558, P 〈 0.01）. Conclusion The sufficient dos

关 键 词：Β珠蛋白生成障碍性贫血 重型 输血 铁螯合剂 铁过载 

分 类 号：R72[医药卫生—儿科]