Abstract
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 (n = 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.
Keywords
paediatric - trauma - injury - coagulopathy - ADAMTS13 - von Willebrand Factor - human
neutrophil peptide
