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目的探讨采用不同剂量铁螯合剂治疗高量输血重型β珠蛋白生成障碍贫血(地贫)患儿1年后的血清铁蛋白(SF)的变化。方法以美国Beckman-Access化学发光仪分别于2005年4月和2006年3月每月定期检测68例高量输血维持血红蛋白100 g/L以上的重型β地贫患儿血SF水平。男41例,女27例;年龄4~15岁。每月使用铁螯合剂(去铁胺或去铁酮)≥20 d 6例(A组),10~15 d 13例(B组),5~9 d 24例(C组),≤3 d 25例(D组),健康对照组(E组)20例。男女各10例。结果2005年4月和2006年3月检测血SF水平分别为A组(2205.02±707.82)μg/L和(2285.29±693.14)μg/L,B组(4028.24±752.11)μg/L和(4736.69±841.50)μg/L,C组(2462.81±937.16)μg/L和(3356.79±994.85)μg/L,D组(2390.42±730.94)μg/L和(3286.12±641.12)μg/L,E组(213.10±42.88)μg/L和(217.25±57.42)μg/L。2006年3月的SF均高于2005年4月,除A组及E组外差异均有统计学意义(P均<0.01)。结论高量输血重型β地贫患儿应长期坚持足量应用铁螯合剂,每月按体质量计算足量使用20 d以上才能使SF增高不明显,SF已明显增高才开始用铁螯合剂患儿应增加每日用量。
Objective To investigate the changes of serum ferritin (SF) after 1 year in children with high blood transfusion of severe β-thalassemia with thalidomide using different doses of iron chelators. Methods The serum SF levels in 68 children with severe β thalassemia who maintained hemoglobin above 100 g / L were detected by Beckman-Access chemiluminescence instrument in America in April 2005 and March 2006 respectively. 41 males and 27 females; aged 4 to 15 years. The use of iron chelators (deferoxamine or deferiprone) ≥20 days in 6 cases (group A), 10-15 days in 13 cases (group B), 5-9 days in 24 cases (group C), ≤3 days 25 cases (group D), healthy control group (group E) 20 cases. 10 men and women in each. Results The blood levels of SF in April 2005 and March 2006 were respectively A (2205.02 ± 707.82) μg / L and (2285.29 ± 693.14) μg / L, B group (4028.24 ± 752.11) μg / L and (4736.69 ± 841.50 μg / L, 2462.81 ± 937.16 μg / L and 3356.79 ± 994.85 μg / L in group C, 2390.42 ± 730.94 μg / L in group D and 3286.12 ± 641.12 μg / ± 42.88) μg / L and (217.25 ± 57.42) μg / L. The SF of March 2006 was higher than that of April 2005, except for the difference between groups A and E (all P <0.01). Conclusion High blood transfusion in children with severe β-thalassemia should be long-term adherence to the full use of iron chelating agent, monthly body mass calculated sufficient use of more than 20 d in order to make SF increased significantly SF has increased significantly before the start with iron chelator Children should increase the daily dosage.