Department of Surgery, The University of Alabama at Birmingham, Birmingham, Alabama.
Division of Laboratory Medicine, Department of Pathology, The University of Alabama at Birmingham, Birmingham, Alabama.
Thromb Haemost. 2018 Apr;118(4):676-687. doi: 10.1055/s-0038-1636528. Epub 2018 Apr 4.
Decrease of plasma activity of ADAMTS13, a metalloenzyme that cleaves von Willebrand factor (VWF) and prevents adhesion and aggregation of platelets, has been reported early after onset of systemic inflammation resulting from infections and after severe trauma. Here, we determined whether trauma-induced systemic (sterile) inflammation would be associated with a reduction of plasma ADAMTS13 activity in paediatric patients and its association with disease severity and outcome. Paediatric patients ( = 106) with severe trauma at a level 1 paediatric trauma centre between 2014 and 2016 were prospectively enrolled. Blood samples were collected upon arrival and at 24 hours and analysed for plasma levels of ADAMTS13 activity, VWF antigen, collagen binding activity, human neutrophil peptides (HNP) 1-3, coagulation abnormalities, endothelial glycocalyx damage and clinical outcome. Plasma samples were also collected for similar measurements from 52 healthy paediatric controls who underwent elective minor surgery. The median age of patients was 9 years with 81% sustaining blunt trauma. The median injury severity score was 22 and the mortality rate was 11%. Plasma levels of ADAMTS13 activity were significantly lower and plasma levels of VWF antigen and HNP 1-3 proteins were significantly higher for paediatric trauma patients on admission and at 24 hours when compared with controls. Finally, the lowest plasma ADAMTS13 activity was found in patients who died from their injuries. We conclude that relative plasma deficiency of ADAMTS13 activity may be associated with more severe traumatic injury, significant endothelial glycocalyx damage, coagulation abnormalities and mortality after severe trauma in paediatric patients.
ADAMTS13 的血浆活性下降，ADAMTS13 是一种金属酶，可切割血管性血友病因子 (VWF)，防止血小板黏附和聚集，在感染和严重创伤后全身炎症发作的早期就有报道。在这里，我们确定创伤引起的全身（无菌）炎症是否与儿科患者血浆 ADAMTS13 活性降低相关，以及其与疾病严重程度和结局的关系。2014 年至 2016 年，在一家儿科创伤中心，对严重创伤的儿科患者（ = 106）进行前瞻性登记。入院时和 24 小时采集血样，分析血浆 ADAMTS13 活性、VWF 抗原、胶原结合活性、人中性粒细胞肽 (HNP) 1-3、凝血异常、内皮糖萼损伤和临床结局。还从 52 名接受择期小手术的健康儿科对照者采集了类似的血浆样本。患者的中位年龄为 9 岁，81%为钝性创伤。损伤严重程度评分中位数为 22，死亡率为 11%。与对照组相比，入院时和 24 小时，儿科创伤患者的 ADAMTS13 活性明显降低，VWF 抗原和 HNP 1-3 蛋白水平明显升高。最后，在因受伤而死亡的患者中发现 ADAMTS13 活性最低。我们的结论是，ADAMTS13 活性的相对血浆缺乏可能与更严重的创伤、明显的内皮糖萼损伤、凝血异常和儿科患者严重创伤后的死亡率相关。
