Kreuz W D, Schneider W, Nowak-Göttl U
Department of Paediatrics, University Hospital Frankfurt/Main, Germany.
Eur J Pediatr. 1999 Dec;158 Suppl 3:S187-91. doi: 10.1007/pl00014353.
Consumption coagulopathy is a serious problem in childhood. In addition to treatment of the underlying disease, consumption coagulopathy was previously treated with heparin. Nowadays it is treated by substitution of coagulation factors, especially antithrombin (AT) concentrate, alone or in combination with heparin. In this pilot study we administered AT concentrate (dosage 80 U/kgbw/d), without additional heparin treatment, to 29 children beyond infancy with acquired AT deficiency. Antithrombin, platelet count, fibrinogen, PT, and APTT were assayed before and during the course of AT substitution. These coagulation parameters returned to normal 48 hours after normalisation of the plasma AT level. AT levels normalised within 24 h of initial substitution in all children. Lethal outcome due to the underlying disease was observed in only two children.
Data of this pilot study suggest that, concomitantly with the treatment of the underlying disease, consumption coagulopathy in childhood can be managed successfully with early substitution of AT concentrate.
消耗性凝血病是儿童期的一个严重问题。除了治疗基础疾病外,消耗性凝血病以前用肝素治疗。如今,它通过单独或与肝素联合使用凝血因子替代治疗,尤其是抗凝血酶(AT)浓缩物。在这项前瞻性研究中,我们对29名婴儿期后的获得性AT缺乏儿童给予AT浓缩物(剂量80 U/kgbw/d),不进行额外的肝素治疗。在AT替代治疗前及治疗过程中检测抗凝血酶、血小板计数、纤维蛋白原、PT和APTT。这些凝血参数在血浆AT水平恢复正常48小时后恢复正常。所有儿童在首次替代治疗后24小时内AT水平恢复正常。仅两名儿童因基础疾病导致死亡。
这项前瞻性研究的数据表明,在治疗基础疾病的同时,早期使用AT浓缩物替代治疗可成功管理儿童消耗性凝血病。