PDF(Pubmed)
Abstract：
Background and Objectives: congenital heart disease (CHD), cyanotic and, to a lesser degree, acyanotic, often are accompanied by coagulation abnormalities, impacting substantially morbidity and mortality. Until now, no consistent hemostatic patterns have been demonstrated in neonates and children with CHD because they represent a variable and heterogenous population. The aim of the present study is to investigate the hemostatic profile, as well as the role of ADAMTS-13 (a disintegrin and metalloprotease with thrombospondin type-1 motives), the cleaving protein of von Willebrand factor (VWF) in neonates with CHD and compare them to healthy age-matched controls. Materials and Methods: twenty neonates with a mean gestational age of 37.1 ± 2.5 weeks were included in the CHD group, and 18 healthy neonates with a mean gestational age of 38.2 ± 1.5 weeks were in the control group. Results: prothrombin time was significantly prolonged, and accordingly, factor VII (FVII) levels were significantly decreased in the CHD group in comparison to controls. Factor VIII (FVIII), VWF, and ristocetin cofactor activity (Rcof) levels were significantly higher in the study vs. control group. Concentrations of ADAMTS-13 were decreased in the CHD vs. control group, but the difference was not statistically significant. Our results, in combination, indicate a balanced hemostatic mechanism, although with greater variability in neonates with CHD, while developmental aspects of coagulation are evident in the specific patient population. Conclusions: the coagulation profile is moderately impaired early in the course of CHD, though increased thrombogenicity is already present and should not be ignored.
摘要：
背景和目的:先天性心脏病(CHD),紫癜和，在较小程度上，杂种,常伴有凝血异常，影响发病率和死亡率。直到现在,在CHD新生儿和儿童中没有发现一致的止血模式,因为它们代表了可变和异质的人群.本研究的目的是调查止血情况，以及ADAMTS-13（一种具有血小板反应蛋白1型动机的整合素和金属蛋白酶）的作用，新生儿冠心病vonWillebrand因子（VWF）裂解蛋白，并将其与健康年龄匹配的对照组进行比较。材料与方法:CHD组20例,平均胎龄37.1±2.5周,对照组18例健康新生儿，平均胎龄为38.2±1.5周。结果：凝血酶原时间明显延长，因此，与对照组相比,CHD组的因子VII(FVII)水平显著降低.因子VIII（FVIII），VWF,和瑞斯托霉素辅因子活性（Rcof）水平在研究中明显高于对照组。ADAMTS-13的浓度在CHD与对照组,但差异无统计学意义。我们的结果,结合起来，表明平衡的止血机制，尽管新生儿冠心病的变异性更大，而凝血的发育方面在特定患者人群中很明显。结论:冠心病早期凝血功能中度受损,虽然增加的血栓形成已经存在，不应忽视。
