Janka G E, Möhring P, Helmig M, Haas R J, Betke K
Eur J Pediatr. 1981 Nov;137(3):285-90. doi: 10.1007/BF00443259.
Ten children with transfusion dependent anemias (thalassemia, sideroblastic anemia, congenital pure red cell aplasia) received either intravenous desferrioxamine (DF) in increasing doses up to 450 mg/kg at the time of transfusion or daily subcutaneous DF up to 110 mg/kg on an outpatient basis. No patient on intravenous DF reached a negative iron balance. All children with a subcutaneous DF dose of more than 60 mg/kg obtained a negative iron balance with a net iron excretion (transfusion iron already substracted) between 206 to 810 mg (mean 496 mg) monthly. The effectiveness of regular subcutaneous DF on liver storage iron could be confirmed in 4 patients by liver biopsy, showing a decrease between 40-60% iron after 12-14 months of chelation therapy. So far the daily iron excretion has remained constant with a given dose of DF over a period up to 15 months. Even if poor compliance in some patients is taken into account, it is possible with this method of treatment to prevent further accumulation of iron in chronically transfused children.
10名依赖输血的贫血患儿（地中海贫血、铁粒幼细胞贫血、先天性纯红细胞再生障碍性贫血）在输血时接受了静脉注射去铁胺（DF），剂量逐渐增加至450mg/kg，或者在门诊接受每日皮下注射DF，剂量高达110mg/kg。接受静脉注射DF的患者均未达到负铁平衡。所有皮下注射DF剂量超过60mg/kg的儿童均实现了负铁平衡，每月净铁排泄量（已减去输血铁）在206至810mg之间（平均496mg）。通过肝活检在4名患者中证实了定期皮下注射DF对肝脏储存铁的有效性，显示螯合治疗12至14个月后铁含量降低了40 - 60%。到目前为止，在长达15个月的时间内，给定剂量的DF每日铁排泄量保持恒定。即使考虑到部分患者依从性较差，采用这种治疗方法也能够防止长期输血儿童体内铁的进一步蓄积。
