Correlation between plasma activity of ADAMTS-13 and coagulopathy, and prognosis in disseminated intravascular coagulation

Hyun, Jungwon; Kim, Hyun Kyung; Kim, Jieun; Lim, Mingyu; Jung, Jae Seol; Park, Wan Beom; Cho, Han‐Ik

doi:10.1016/j.thromres.2008.11.020

Cited by 43 publications

(41 citation statements)

References 22 publications

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“…In some cases, the potential addition of platelet/VWF thrombi due to an acquired ADAMTS13 functional deficiency and leading to so-called TMA-like lesions is highly suspected in clinical arguments [41-43] but still remains debated in terms of prognosis and pathophysiology. Furthermore, in most studies published so far, the subtle combination of DIC and TMA-like lesions has been difficult to establish because of the great heterogeneity of the sepsis cohorts tested, involving mixed cases within the categories of systemic inflammation, sepsis, septic shock, and sepsis-related multiple organ failure [20-26], some of them complicated by DIC and others not. In the current study, we focused on a cohort of 72 patients with septic shock prospectively enrolled from a single ICU in order to respond to two objectives: first, to analyze the interaction between DIC and ADAMTS13 and its impact on prognosis, and second, to elucidate the pathophysiological mechanisms for ADAMTS13 deficiency.…”

Section: Discussionmentioning

confidence: 99%

“…Some cases of ADAMTS13 deficiency have been reported recently in adult patients with sepsis-induced DIC and this ADAMTS13 deficiency was associated with acute renal failure [20]. Later clinical studies, also in adults and focusing on sepsis without DIC [21-25] or isolated DIC not specifically related to sepsis [26], reported decreased ADAMTS13 activity. Interestingly, some of these studies [20,21,25,26] showed that partially decreased ADAMTS13 activity was associated with outcome.…”

Section: Introductionmentioning

confidence: 99%

“…Later clinical studies, also in adults and focusing on sepsis without DIC [21-25] or isolated DIC not specifically related to sepsis [26], reported decreased ADAMTS13 activity. Interestingly, some of these studies [20,21,25,26] showed that partially decreased ADAMTS13 activity was associated with outcome. In pediatric patients with severe sepsis, ADAMTS13 deficiencies have also been reported [27-29].…”

Section: Introductionmentioning

confidence: 99%

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The prognostic value of ADAMTS13 (a disintegrin and metalloprotease with thrombospondin type 1 repeats, member 13) deficiency in septic shock patients involves interleukin-6 and is not dependent on disseminated intravascular coagulation

et al. 2013

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IntroductionADAMTS13 (a disintegrin and metalloprotease with thrombospondin type 1 repeats, member 13) deficiency has been reported in patients with sepsis but its clinical relevance and pathophysiology remain unclear. Our objectives were to assess the clinical significance, prognostic value and pathophysiology of ADAMTS13 deficiency in patients with septic shock with and without disseminated intravascular coagulation (DIC).MethodsThis was a prospective monocenter cohort study of patients with septic shock. Von Willebrand Factor, ADAMTS13-related parameters and plasma IL-6 concentration were measured at inclusion to the study. Patients were categorized into three groups according to the presence of ADAMT13 deficiency (<30%) or DIC.ResultsThis study included 72 patients with a median age of 59 years (interquartile range (IQR) 50 to 71). Each of the included patients received vasopressors; 55 (76%) were under mechanical ventilation and 22 (33%) underwent renal replacement therapy. Overall, 19 patients (26%) had DIC, and 36 patients had ADMTS13 deficiency (50%). Patients with DIC, ADAMTS13 deficiency or both were more severe at ICU admission. Mortality was higher in septic shock patients from group one. By multivariate analysis, Simplified Acute Physiology Score 2 (SAPS2) score (odds ratio (OR) 1.11/point; 95% CI 1.01 to 1.24) and ADAMTS13 activity <30% (OR 11.86; 95% CI 1.36 to 103.52) were independently associated with hospital mortality. There was no correlation between ADAMTS13 activity and the International Society for Thrombosis and Haemostasis (ISTH) score (rs = -0.97, P = 0.41) suggesting that ADAMTS13 functional deficiency and DIC were independent parameters. IL-6 level was higher in patients with ADAMTS13 activity <30% [895 (IQR 330 to 1843) pg/mL versus 83 (IQR 43 to 118), P = 0.0003).ConclusionsSeptic shock was associated with a functional deficiency of ADAMTS13, independently of DIC. ADAMTS13 functional deficiency is then a prognostic factor for mortality in septic shock patients, independently of DIC.

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Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

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The prognostic value of ADAMTS13 (a disintegrin and metalloprotease with thrombospondin type 1 repeats, member 13) deficiency in septic shock patients involves interleukin-6 and is not dependent on disseminated intravascular coagulation

et al. 2013

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“…Elevated plasma vWF antigen (vWF: Ag) and/or decreased ADAMTS 13 activity are associated with negative outcomes of several disorders [11]. An imbalance between the circulating levels of vWF and ADAMTS 13 has been reported in a number of acquired diseases in adults such as coronary artery disease and myocardial infarction, peripheral arterial disease, ischemic stroke, preeclampsia, inflammatory bowel disease, and liver cirrhosis [12,13] and these findings have been reported also in different diseases of pediatric age such as type 1 diabetes mellitus [14] and ESRD [15].…”

Section: Discussionmentioning

confidence: 99%

Study of A Disintegrin and Metalloproteinase with ThromboSpondin Type 1 Repeats 13 (ADAMTS 13) in Children with Idiopathic Nephrotic Syndrome

Gamasy¹,

Abdel-Hafez²,

Abdelmageed³

et al. 2017

Arch Med

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IntroductionMany predisposing factors for TE were reported in nephrotic patients , abnormalities in platelet activation and aggregation, activation of prothrombotic factors of coagulation system e.g. factors V,VII, VIII, X, von Willebrand factor (vWF), fibrinogen, and α 2-macroglobulin, decreased activity of fibrinolytic system such as plasminogen [1] and decreased endogenous anticoagulants, antithrombin III, protein C, protein S and tissue factor pathway inhibitor resulting in local activation of the glomerular hemostasis system [2]. vWF mediates platelet adhesion and aggregation at sites of vascular injury [3]. It is released from the stimulated endothelium as unusually large (UL) multimer [4]. ULvWF favor platelet aggregation and formation of microvascular thrombi [5]. A disintegrin and metalloprotease with thrombospondin type-1 motif 13 (ADAMTS 13) can cleaves and thus converses ULvWF into a less active form [6]. Reduced ADAMTS 13 activity due to gene mutation or presence of autoimmune IgM and IgG inhibitors [7] results in deficient proteolysis of ULvWF with formation of disseminated platelet-rich thrombi in the microcirculation seen in thrombotic microangiopathies (TMA) [8,9]. Study of A Disintegrin and

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“…DIC: Decreased ADAMTS13 and elevated VWFpp have been reported in patients with DIC [57] and TMA [13], suggesting that ADAMTS13 is also consumed through the continuing cleavage of VWF or from the low production of ADAMTS13 in DIC patients. Although DIC and TMA are different diseases, they display similar hemostatic abnormalities to patients with LDLT.…”

Section: Activation and Consumption Of Platelets Due To Thrombosismentioning

confidence: 99%

Platelets Activation and Liver Transplantation

Usui¹,

Wada²,

Mizuno³

et al. 2017

J Liver

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Transient thrombocytopenia is a common phenomenon after living donor liver transplantation (LDLT), and severe thrombocytopenia after LDLT is associated with graft loss and poor patient outcomes. The various causes of thrombocytopenia include bone marrow hematopoiesis failure due to decreased thrombopoietin (TPO) production in the injured liver, platelet destruction associated with splenomegaly, and the activation and consumption of platelets due to various forms of thrombosis, including disseminated intravascular coagulation (DIC), thrombotic microangiopathy (TMA), and venous thromboembolism (VTE).The observation of biomarkers such as soluble platelet glycoprotein VI (sGPVI), TPO, von Willebrand factor (VWF), VWF propeptide (VWFpp), and disintegrin-like and metalloproteinase with thrombospondin type-1 motifs member 13 (ADAMTS13) is useful in the evaluation of the mechanisms of thrombocytopenia in patients who undergo LDLT. The presence of these biomarkers, including sGPVI, ADAMTS13, VWF and VWFpp, suggests that platelet activation occurs in the early phase of LDLT and that vascular endothelial cell injury occurs on postoperative days 7-14.

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Correlation between plasma activity of ADAMTS-13 and coagulopathy, and prognosis in disseminated intravascular coagulation

Cited by 43 publications

References 22 publications

The prognostic value of ADAMTS13 (a disintegrin and metalloprotease with thrombospondin type 1 repeats, member 13) deficiency in septic shock patients involves interleukin-6 and is not dependent on disseminated intravascular coagulation

The prognostic value of ADAMTS13 (a disintegrin and metalloprotease with thrombospondin type 1 repeats, member 13) deficiency in septic shock patients involves interleukin-6 and is not dependent on disseminated intravascular coagulation

Study of A Disintegrin and Metalloproteinase with ThromboSpondin Type 1 Repeats 13 (ADAMTS 13) in Children with Idiopathic Nephrotic Syndrome

Platelets Activation and Liver Transplantation

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