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目的探讨重型β地中海贫血(β-TM)患儿心脏、肝脏铁过载状况,以及与临床监测指标之间的关系。方法 2010年6月,根据自愿的原则,从规律输血治疗的80例≥7岁的β-TM患儿中,选择51例,进行心脏磁共振T2*(心脏T2*)及肝脏磁共振T2*(肝脏T2*)检测。检测结果与年龄、血清铁蛋白(SF)、心脏左室射血分数(LVEF)、输血年限、去铁年限、输血前血红蛋白(Hb)进行比较。结果 51例患儿中11例(21.6%)发生心肌铁过载,其中轻度3例,中度3例,重度5例;肝脏铁过载43例(84.3%),其中轻度14例,中度17例,重度12例。心脏T2*与SF、LVEF、肝脏T2*之间无相关性,SF与肝脏T2*呈正相关(r=0.558,P<0.01)。心肌铁过载患儿输血年限大于心肌铁正常患儿(P<0.05),而肝脏铁过载发生率差异无统计学意义(P>0.05)。11例心肌铁过载患儿中,2例LVEF降低。结论β-TM患儿SF值可反映机体肝脏铁过载的情况,但不能预测心肌铁过载;随着输血年限的增加,心肌铁过载风险亦加大;心肌铁过载与肝脏铁过载之间存在着不一致性,肝脏铁过载不能成为提示心肌铁过载的依据。心肌铁过载患儿LVEF可以在正常范围,LVEF不能作为预测心肌铁过载的可靠指标。
Objective To investigate the relationship between iron overload in the heart and liver of children with β-thalassemia major (β-TM) and its clinical significance. Methods Totally 51 out of 80 80-year-old children with β-TM who underwent regular blood transfusion were enrolled in this study. According to the voluntary principle, T2 * (T2 *) and T2 * (Liver T2 *) test. The test results were compared with age, serum ferritin (SF), left ventricular ejection fraction (LVEF), duration of blood transfusion, number of years of deferment, hemoglobin before transfusion (Hb). Results Myocardial iron overload occurred in 11 of 51 (21.6%) patients, of which 3 were mild, 3 moderate, and 5 severe. Iron overload was found in 43 (84.3%) of the patients, of which 14 17 cases, severe in 12 cases. There was no correlation between T2 * and SF, LVEF and liver T2 *, SF and liver T2 * (r = 0.558, P <0.01). The duration of blood transfusion in patients with myocardial iron overload was greater than that in patients with normal myocardial iron (P <0.05), while there was no significant difference in the incidence of liver iron overload (P> 0.05). Eleven patients with myocardial iron overload, 2 cases of LVEF decreased. Conclusion The SF value of β-TM children can reflect the iron overload in liver, but can not predict myocardial iron overload. With the increase of blood transfusion, the risk of myocardial iron overload also increases. There is a relationship between myocardial iron overload and liver iron overload Inconsistency, liver iron overload can not be the basis for suggesting myocardial iron overload. LVEF in patients with myocardial iron overload can be in the normal range, LVEF can not be used as a reliable indicator of myocardial iron overload.