中国实用儿科杂志

中国实用儿科杂志 ›› 2012, Vol. 27 ›› Issue (2): 120-123.

• 论著 • 上一篇    下一篇

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

陈光福，陈娟娟，高红英，龙    琦，富国华   

  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
  • 出版日期:2012-02-06 发布日期:2012-04-12

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

  • Online:2012-02-06 Published:2012-04-12

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。

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

Abstract:

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， ultrasonograph 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）， myocardial， liver， pancreas and pituitary MR imaging T2* were performed on 51 patients of them. The51 patients were divided 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 myocardial 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 51cases （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 dose DFO with DFP treatment， and the DFX treatment can effectively reduce SF，compared with that of insufficient DFO with DFP treatment. All iron chelation therapy can alleviate myocardial iron overload.

Key words: Beta-thlassemia/Major, blood transfusion, iron chelating agents, iron overload