Decreased plasma ADAMTS-13 activity as a predictor of postoperative bleeding in cyanotic congenital heart disease

Soares, R.P.S.; Bydlowski, Sérgio Paulo; Jatene, Marcelo Biscegli; Hironaka, Janete Ferreira; Lopes, Antônio Augusto

doi:10.6061/clinics/2013(04)15

Cited by 7 publications

(3 citation statements)

References 24 publications

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“…44,45 Furthermore, low plasma ADAMTS13 activity has been found to be a predictor of postoperative bleeding in paediatric patients with cyanotic congenital heart disease. 46 The mechanism for this correlation is not fully understood. Our results also show a low ADAMTS13 activity to VWF antigen ratio, suggesting a potential for accrual of large VWF antigen multimers at the surface of the vascular endothelium causing platelet clumping and formation of thrombin.…”

Section: Discussionmentioning

confidence: 99%

Low Plasma ADAMTS13 Activity Is Associated with Coagulopathy, Endothelial Cell Damage and Mortality after Severe Paediatric Trauma

et al. 2018

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Summary 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 pediatric patients and its association with disease severity and outcome. Pediatric patients (n=106) with severe trauma at a level 1 pediatric trauma center between 2014 and 2016 were prospectively enrolled. Blood samples were collected upon arrival and at 24 hours and analyzed 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 pediatric controls patients who underwent elective minor surgery. The median patient age 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 HNP1-3 proteins were significantly higher for pediatric trauma patients on admission and at 24 hours when compared to 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 pediatric patients.

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Section: Discussionmentioning

confidence: 99%

Low Plasma ADAMTS13 Activity Is Associated with Coagulopathy, Endothelial Cell Damage and Mortality after Severe Paediatric Trauma

et al. 2018

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“…A decrease in ADAMTS13act in association with an increase in the VWF:Ag/ADAMTS13act coefficient may be a risk factor for thrombosis and coagulopathy[ 41 ]. Claus et al[ 21 ] reported that significant disturbances in the balance between VWF:Ag and ADAMTS13act can result in the formation of UL-VWF multimers.…”

Section: Discussionmentioning

confidence: 99%

Levels and activities of von Willebrand factor and metalloproteinase with thrombospondin type-1 motif, number 13 in inflammatory bowel diseases

Cibor¹,

Owczarek²,

Butenas³

et al. 2017

WJG

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AIMTo evaluate the levels of von Willebrand factor (VWF) and metalloproteinase with thrombospondin type-1 motif, number 13 (ADAMTS13) in inflammatory bowel disease (IBD) and correlate them with the disease activity.METHODSConsecutive patients with IBD aged 18 years or older were enrolled in the study. Forty-seven patients with ulcerative colitis (UC), 38 with Crohn’s disease (CD), and 50 healthy controls were included. The white blood cell count, haematocrit, platelet count, fibrinogen, partial activated thromboplastin time, C-reactive protein, albumin, VWF antigen level (VWF:Ag), VWF ristocetin cofactor activity (VWF:RCo), VWF collagen-binding activity (VWF:CB), and ADAMTS13 antigen level (ADAMTS13:Ag) and activity (ADAMTS13act) were measured. The following ratios were assessed: VWF:RCo/VWF:Ag, VWF:CB/VWF:Ag, VWF:Ag/ADAMTS13act, and ADAMTS13act/ADAMTS13:Ag.RESULTSCompared to controls, the odds ratio (OR) of an elevated VWF: Ag > 150% was 8.7 (95%CI: 2.7-28.1) in the UC group and 16.2 (95%CI: 4.8-54.0) in the CD group. VWF:CB was lower in UC patients, and active CD was associated with a higher VWF: RCo (+38%). The ORs of VWF:CB/VWF:Ag < 0.7 (a marker of acquired von Willebrand syndrome) in the UC and CD groups were 11.9 (95%CI: 4.4-32.4) and 13.3 (95%CI: 4.6-38.1), respectively. Active UC was associated with lower ADAMTS13:Ag (-23%) and ADAMTS13act (-20%) compared to UC in remission. Patients with active CD had a 15% lower ADAMTS13act than controls. The activity of UC, but not that of CD, was inversely correlated with ADAMTS13:Ag (r = -0.76) and ADAMTS13act (r = -0.81).CONCLUSIONComplex VWF-ADAMTS13-mediated mechanisms disturb haemostasis in IBD. A reduced WVF:CB is a risk factor for bleeding, while a lower ADAMTS13 level combined with an elevated VWF:Ag could predispose one to thrombosis.

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“…I helped to establish and characterize a novel VWF:CB assay for the measurement of VWF activity. 223 Soares et al 224 225 employed a VWF:CB to help characterize ADAMTS13 and VWF level and activity in children with cyanotic congenital heart disease. Hugenholtz et al 226 included a VWF:CB to highlight an unbalance between VWF and ADAMTS13 in acute liver failure; in these patients, VWF:Ag was raised, but VWF:CB and VWF:RCo/Ag ratio was reduced, as was ADAMTS13 and the proportion of HMWM.…”

Section: A History Of the Von Willebrand Factor Collagen-binding Assaymentioning

confidence: 99%

The Role of the von Willebrand Factor Collagen-Binding Assay (VWF:CB) in the Diagnosis and Treatment of von Willebrand Disease (VWD) and Way Beyond: A Comprehensive 36-Year History

Favaloro

2023

Semin Thromb Hemost

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The von Willebrand factor (VWF) collagen binding (VWF:CB) assay was first reported for use in von Willebrand diagnostics in 1986, by Brown and Bosak. Since then, the VWF:CB has continued to be used to help diagnose von Willebrand disease (VWD) (correctly) and also to help assign the correct subtype, as well as to assist in the monitoring of VWD therapy, especially desmopressin (DDAVP). However, it is important to recognize that the specific value of any VWF:CB is predicated on the use of an optimized VWF:CB, and that not all VWF:CB assays are so optimized. There are some good commercial assays available, but there are also some “not-so-good” commercial assays available, and these may continue to give the VWF:CB “a bad reputation.” In addition to VWD diagnosis and management, the VWF:CB found purpose in a variety of other applications, from assessing ADAMTS13 activity, to investigation into acquired von Willebrand syndrome (especially as associated with use of mechanical circulatory support or cardiac assist devices), to assessment of VWF activity in disease states in where an excess of high-molecular-weight VWF may accumulate, and lead to increased (micro)thrombosis risk (e.g., coronavirus disease 2019, thrombotic thrombocytopenic purpura). The VWF:CB turns 37 in 2023. This review is a celebration of the utility of the VWF:CB over this nearly 40-year history.

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Decreased plasma ADAMTS-13 activity as a predictor of postoperative bleeding in cyanotic congenital heart disease

Cited by 7 publications

References 24 publications

Low Plasma ADAMTS13 Activity Is Associated with Coagulopathy, Endothelial Cell Damage and Mortality after Severe Paediatric Trauma

Low Plasma ADAMTS13 Activity Is Associated with Coagulopathy, Endothelial Cell Damage and Mortality after Severe Paediatric Trauma

Levels and activities of von Willebrand factor and metalloproteinase with thrombospondin type-1 motif, number 13 in inflammatory bowel diseases

The Role of the von Willebrand Factor Collagen-Binding Assay (VWF:CB) in the Diagnosis and Treatment of von Willebrand Disease (VWD) and Way Beyond: A Comprehensive 36-Year History

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