桂林市75例重型地中海贫血治疗现状

Current treatment status of severe thalassemia in 75 cases in Guilin City

目的了解桂林市重型地中海贫血治疗状况。方法搜集桂林市区7家医院以及所辖12个县人民医院定期输血的重型地中海贫血患者128例中能规范输血且开始去铁治疗的75例患者作为研究对象．对比不同去铁方式患者的左心室射血分数（LVEF）和血清铁蛋白（SF）检测值及不同年龄组患者的SF值。结果本组75例患者规范治疗30例，占40％。其中，足疗程联合组15例，足疗程单用地拉罗司（DFX）组15例。足疗程联合组和足疗程单用DFX组降低SF浓度的去铁效果明显优于不足疗程联合组（P〈0．05）。三组LVEF值比较，差异无统计学意义（P〉0．05）。2～〈6岁组SF浓度为（2678±783）ng／mL，6～〈13岁组SF浓度为（3478±1074）ng／mL，13～28岁组SF浓度为（4391±1165）ng／mL，对各年龄组SF浓度进行分析，结果显示，随着年龄增大，SF值增大，差异有统计学意义（P〈0．05）。结论桂林市重型地中海贫血患者随着年龄增长，输血量增多，铁过载情况逐渐加重，这些患者采用规范、足疗程去铁治疗能明显降低铁过载。而昂贵的医疗费用使得规范治疗受到一定制约。需政府加强对其的补助。

Objective To investigate the treatment status of severe thalassemia in Guilin City. Methods Among 128 patients with thalassemia who treated with regular blood transfusion in 7 hospitals in downtown of Guilin City and 12 People＇s Hospitals in counties, 75 patients who received standard blood transfusion and had started de-iron therapy were selected as the study objects. The LVEF and SF detection values among patients with different de-iron treatment ways and the SF values among different age groups were compared. Results Among 75 patients, 30 cases received standard treatment, accounting for 40%, of which 15 patients belonged to the full treatment course combination group and 15 patients belonged to the full treatment course single DFX group. The full treatment course combination group and the full treatment course single DFX group were significantly superior to the insufficient treatment course combi- nation group in the de-iron effects of reducing SF concentration （P 〈 0.05）. The LVEF values in the three groups had no significant differences （P 〉 0.05）. The SF concentration was （2678＋783） ng/mL in the 2-〈6 years old group, （3478＋ 1074） ng/mL in the 6-〈13 years old group and （4391＋1165） ng/mL in the 13-28 years old group, then analysis of the SF concentration showed that the average concentration of SF increased with the age, the difference was statistically significant （P 〈 0.05）. Conclusion In Guilin City, along with the growth of the age, the blood transfusion amount increased and the iron overload worsened in thalassemia patients. Standard and full treatment course of de-iron therapy can sig- nificantly reduce the iron overload, but large medical expenses restrain the standard treatment, and thus the govern- ment should increase its subsidy.