Nowak-Göttl U, Funk M, Mosch G, Wegerich B, Kornhuber B, Breddin H K
Dept. Haematology and Oncology, University Hospital Frankfurt/Main.
Klin Padiatr. 1994 Nov-Dec;206(6):437-9. doi: 10.1055/s-2008-1046646.
To avoid misclassification of lowered or enhanced coagulation proteins in childhood the purpose of this study was to establish functional normal ranges for healthy children aged 6 months to 16 years. PT, aPTT, fibrinogen, antithrombin III, protein C, protein S, plasminogen and alpha 2-antiplasmin were tested using the new Automated Coagulation Laboratory (ACL) method. Values for PT, aPTT, fibrinogen, antithrombin III, plasminogen and alpha 2-antiplasmin were closely comparable in all children although we found a minimum range of 61% in children aged 8 to 16 years in plasma concentrations of alpha 2-antiplasmin. Children < 2.5 years showed reduced lower boundaries encompassing 95% of the population for protein C and protein S activity, although medians for protein S activity were similar in all children. The rapid and automatic determination of functional coagulation proteins using chromogenic substrates on the ACL 300 (25-50 microliters citrated plasma/test) renders the possibility to realize a screening program for inherited thrombotic syndromes in a routine laboratory.
为避免儿童期凝血蛋白降低或升高的错误分类，本研究的目的是建立6个月至16岁健康儿童的功能正常范围。使用新型自动凝血实验室（ACL）方法检测凝血酶原时间（PT）、活化部分凝血活酶时间（aPTT）、纤维蛋白原、抗凝血酶III、蛋白C、蛋白S、纤溶酶原和α2-抗纤溶酶。尽管我们发现8至16岁儿童血浆α2-抗纤溶酶浓度的最低范围为61%，但所有儿童的PT、aPTT、纤维蛋白原、抗凝血酶III、纤溶酶原和α2-抗纤溶酶的值具有高度可比性。2.5岁以下儿童的蛋白C和蛋白S活性的下限降低，涵盖了95%的人群，尽管所有儿童的蛋白S活性中位数相似。使用ACL 300（25 - 50微升枸橼酸盐血浆/检测）上的发色底物快速自动测定功能性凝血蛋白，使得在常规实验室中实现遗传性血栓形成综合征筛查项目成为可能。
