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Disease on EC 3.4.24.87 - ADAMTS13 endopeptidase

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DISEASE
TITLE OF PUBLICATION
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Abortion, Spontaneous
Pregnancy complications in acquired thrombotic thrombocytopenic purpura: a case¿control study.
Acquired Immunodeficiency Syndrome
von Willebrand factor-cleaving protease inhibitor in a patient with human immunodeficiency syndrome-associated thrombotic thrombocytopenic purpura.
Acute Chest Syndrome
Dynamics of von Willebrand factor reactivity in sickle cell disease during vaso-occlusive crisis and steady state.
Acute Coronary Syndrome
Change in plasma a disintegrin and metalloprotease with thrombospondin type-1 repeats-13 and von Willebrand factor levels in venous thromboembolic patients.
VWF, ADAMTS13, and acute coronary syndromes.
Acute Kidney Injury
Absolute immature platelet count helps differentiate thrombotic thrombocytopenic purpura from hypertension-induced thrombotic microangiopathy.
Acute renal failure is prevalent in patients with thrombotic thrombocytopenic purpura associated with low plasma ADAMTS13 activity.
Acute renal failure is prevalent in patients with thrombotic thrombocytopenic purpura associated with low plasma ADAMTS13 activity: comment.
Acute renal failure is prevalent in patients with thrombotic thrombocytopenic purpura associated with low plasma ADAMTS13 activity: reply.
ADAMTS13 activity to von Willebrand factor antigen ratio predicts acute kidney injury in patients with COVID-19: Evidence of SARS-CoV-2 induced secondary thrombotic microangiopathy.
ADAMTS13 inhibits oxidative stress and ameliorates progressive chronic kidney disease following ischaemia/reperfusion injury.
Decreased a disintegrin-like and metalloprotease with thrombospondin (ADAMTS)-13 is associated with a poor prognosis in sepsis-induced organ failure*
Decreased ADAMTS-13 (A disintegrin-like and metalloprotease with thrombospondin type 1 repeats) is associated with a poor prognosis in sepsis-induced organ failure.
Hereditary ADAMTS 13 deficiency presenting as recurrent acute kidney injury.
High Frequency of Acquired ADAMTS13 Deficiency After Hemolysis in Hemiscorpius Lepturus (Scorpion) Stung Children.
Nonsteroidal Anti-inflammatory Drug Induced Thrombotic Thrombocytopenic Purpura.
Novel compound heterozygote mutations (H234Q/R1206X) of the ADAMTS13 gene in an adult patient with Upshaw-Schulman syndrome showing predominant episodes of repeated acute renal failure.
Renal thrombotic microangiopathy and pulmonary arterial hypertension in a patient with late-onset cobalamin C deficiency.
Severe secondary deficiency of von Willebrand factor-cleaving protease (ADAMTS13) in patients with sepsis-induced disseminated intravascular coagulation: its correlation with development of renal failure.
Thrombotic microangiopathy as first manifestation of acute human immunodeficiency virus infection: a case report and review of the literature.
Acute Lung Injury
ADAMTS-13 regulates neutrophil recruitment in a mouse model of invasive pulmonary aspergillosis.
[Post-transfusion acute lung injury (Trali) after plasma infusion in a patient having a constitutional thrombotic microangiopathy]
adamts13 endopeptidase deficiency
A case for consideration by apheresis practitioners: Melanoma and PD-1 inhibitor treatment in a patient with multiple relapses of immune thrombotic thrombocytopenic purpura.
A case of severe ADAMTS13 deficiency presenting as thrombotic thrombocytopenic purpura in pregnancy.
A common origin of the 4143insA ADAMTS13 mutation.
A first case of congenital TTP on the African continent due to a new homozygous mutation in the catalytic domain of ADAMTS13.
A mechanistic approach to the diagnosis and management of atypical hemolytic uremic syndrome.
A modified PLASMIC score including the lactate dehydrogenase/the upper limit of normal ratio more accurately identifies Chinese thrombotic thrombocytopenic purpura patients than the original PLASMIC score.
A new mouse model mimicking thrombotic thrombocytopenic purpura: correction of symptoms by recombinant human ADAMTS13.
A novel association of acquired ADAMTS13 inhibitor and acute dengue virus infection.
A novel homozygous missense ADAMTS13 mutation Y658C in a patient with recurrent thrombotic thrombocytopenic purpura.
A novel nanobody that detects the gain-of-function phenotype of von Willebrand factor in ADAMTS13 deficiency and von Willebrand disease type 2B.
A perspective on the measurement of ADAMTS13 in thrombotic thrombocytopaenic purpura.
A Rare Cause of Thrombotic Thrombocytopenia Purpura- (TTP-) Like Syndrome, Vitamin B12 Deficiency: Interpretation of Significant Pathological Findings.
A recombinant human ADAMTS-13: first-in-human study evaluating pharmacokinetics, safety and tolerability in cTTP patients.
A relative ADAMTS13 deficiency supports the presence of a secondary microangiopathy in COVID 19.
A role for tissue plasminogen activator in thrombotic thrombocytopenic purpura.
Acquired ADAMTS-13 deficiency in pediatric patients with severe sepsis.
Acquired intracoronary ADAMTS13 deficiency and VWF retention at sites of critical coronary stenosis in patients with STEMI.
Acquired thrombotic thrombocytopenia purpura associated with severe ADAMTS13 deficiency in a 3-year-old boy: a case report and review of the literature.
Acquired thrombotic thrombocytopenic purpura due to antibody-mediated ADAMTS13 deficiency precipitated by a localized Castleman's disease: A case report.
Acquired thrombotic thrombocytopenic purpura with isolated CFHR3/1 deletion-rapid remission following complement blockade.
Acquired von Willebrand factor deficiency caused by LVAD is ADAMTS-13 and platelet dependent.
Acquired, noncongenital thrombotic thrombocytopenic purpura in children and adolescents: clinical management and the use of ADAMTS 13 assays.
ADAMTS 13 deficiency is associated with abnormal distribution of von Willebrand factor multimers in patients with COVID-19.
ADAMTS-13 activity and autoantibodies classes and subclasses as prognostic predictors in acquired thrombotic thrombocytopenic purpura.
ADAMTS-13 activity in patients with brain and prostate tumors is mildly reduced, but not correlated to stage of malignancy and metastasis.
ADAMTS-13 deficiency following Hemiscorpius lepturus scorpion sting.
ADAMTS-13 deficiency: can it cause chronic renal failure?
ADAMTS-13 level in children with severe diarrhea-associated hemolytic uremic syndrome: Unmasking new association.
ADAMTS13 activity and genetic mutations in Japan.
ADAMTS13 activity and the presence of acquired inhibitors in human immunodeficiency virus-related thrombotic thrombocytopenic purpura.
ADAMTS13 activity in sickle cell disease.
ADAMTS13 activity in thrombotic thrombocytopenic purpura-hemolytic uremic syndrome: relation to presenting features and clinical outcomes in a prospective cohort of 142 patients.
ADAMTS13 activity is decreased in a septic porcine model. Significance for glomerular thrombus deposition.
ADAMTS13 and anti-ADAMTS13 antibodies as markers for recurrence of acquired thrombotic thrombocytopenic purpura during remission.
ADAMTS13 and its variants promote angiogenesis via upregulation of VEGF and VEGFR2.
ADAMTS13 and von Willebrand factor in thrombotic thrombocytopenic purpura.
ADAMTS13 assays and ADAMTS13-deficient mice.
ADAMTS13 autoantibodies in patients with thrombotic microangiopathies and other immunomediated diseases.
ADAMTS13 controls vascular remodeling by modifying VWF reactivity during stroke recovery.
ADAMTS13 deficiency and immunological abnormalities in patients with systemic sclerosis.
ADAMTS13 Deficiency and Thrombotic Thrombocytopenic Purpura Associated with Trimethoprim-Sulfamethoxazole.
ADAMTS13 deficiency exacerbates VWF-dependent acute myocardial ischemia/reperfusion injury in mice.
ADAMTS13 deficiency in mice does not affect adipose tissue development.
ADAMTS13 deficiency in severe postpartum HELLP syndrome.
ADAMTS13 deficiency promotes microthrombosis in a murine model of diet-induced liver steatosis.
ADAMTS13 Deficiency Shortens the Life Span of Mice With Experimental Diabetes.
ADAMTS13 deficiency with elevated levels of ultra-large and active von Willebrand factor in P. falciparum and P. vivax malaria.
ADAMTS13 Deficiency Worsens Colitis and Exogenous ADAMTS13 Administration Decreases Colitis Severity in Mice.
ADAMTS13 deficiency, an important cause of thrombocytopenia during pregnancy.
ADAMTS13 deficiency, despite well-compensated liver functions in patients with noncirrhotic portal hypertension.
ADAMTS13 gene defects in two brothers with constitutional thrombotic thrombocytopenic purpura and normalization of von Willebrand factor-cleaving protease activity by recombinant human ADAMTS13.
ADAMTS13 gene deletion enhances plasma high-mobility group box1 elevation and neuroinflammation in brain ischemia-reperfusion injury.
ADAMTS13 Gene Mutations Influence ADAMTS13 Conformation and Disease Age-Onset in the French Cohort of Upshaw-Schulman Syndrome.
ADAMTS13 maintains cerebrovascular integrity to ameliorate Alzheimer-like pathology.
ADAMTS13 missense variants associated with defective activity and secretion of ADAMTS13 in a patient with non-cirrhotic portal hypertension.
ADAMTS13 mutations and polymorphisms in congenital thrombotic thrombocytopenic purpura.
ADAMTS13 promotes angiogenesis and modulates VEGF-induced angiogenesis.
ADAMTS13 reduces VWF-mediated acute inflammation following focal cerebral ischemia in mice.
ADAMTS13 Retards Progression of Diabetic Nephropathy by Inhibiting Intrarenal Thrombosis in Mice.
ADAMTS13 test and/or PLASMIC clinical score in management of acquired thrombotic thrombocytopenic purpura: a cost-effective analysis.
ADAMTS13 turns 3.
ADAMTS13 unbound to larger von Willebrand factor multimers in cryosupernatant: implications for selection of plasma preparations for thrombotic thrombocytopenic purpura treatment.
ADAMTS13-specific circulating immune complexes as potential predictors of relapse in patients with acquired thrombotic thrombocytopenic purpura.
ADAMTS13: a new link between thrombosis and inflammation.
ADAMTS13: origins, applications, and prospects.
Adenoviral-mediated gene transfer restores plasma ADAMTS13 antigen and activity in ADAMTS13 knockout mice.
Allosteric activation of ADAMTS13 by von Willebrand factor.
Amplified endogenous plasmin activity resolves acute thrombotic thrombocytopenic purpura in mice.
An update on pathogenesis and diagnosis of thrombotic thrombocytopenic purpura.
Analysis of clinical and laboratory characteristics in 42 patients with thrombotic thrombocytopenic purpura from a single center in China.
Animal models for thrombotic thrombocytopenic purpura.
Annual incidence and severity of acute episodes in hereditary thrombotic thrombocytopenic purpura.
Anti-ADAMTS13 Autoantibodies against Cryptic Epitopes in Immune-Mediated Thrombotic Thrombocytopenic Purpura.
Anti-ADAMTS13 autoantibody profiling in patients with immune-mediated thrombotic thrombocytopenic purpura.
Antibody-mediated ADAMTS13 deficiency workup is commonly missed.
Antigen and substrate withdrawal in the management of autoimmune thrombotic disorders.
Application of PLASMIC score in prediction of ADAMTS13 deficiency in a pediatric case of acquired thrombotic thrombocytopenic purpura.
Assessing thrombocytopenia in the intensive care unit: the past, present, and future.
Assessment of ADAMTS-13 Level in Hospitalized Children with Serious Bacterial Infections as a Possible Prognostic Marker.
Autoimmune thrombotic microangiopathy: advances in pathogenesis, diagnosis, and management.
Autosomal recessive inheritance of von Willebrand factor-cleaving protease deficiency.
Best practices and recommendations for drug regimens and plasma exchange for immune thrombotic thrombocytopenic purpura.
Beyond plasma exchange: novel therapies for thrombotic thrombocytopenic purpura.
Bilateral serous retinal detachment as a complication of acquired peripartum thrombotic thrombocytopenic purpura bout.
Blood group O and black race are independent risk factors for thrombotic thrombocytopenic purpura associated with severe ADAMTS13 deficiency.
Breast Cancer-Associated Thrombotic Microangiopathy.
Cancer-associated Microangiopathic Hemolytic Anemia with Thrombocytopenia: an important diagnostic consideration.
Cancer-related thrombotic microangiopathy secondary to Von Willebrand factor-cleaving protease deficiency.
Change in plasma a disintegrin and metalloprotease with thrombospondin type-1 repeats-13 and von Willebrand factor levels in venous thromboembolic patients.
Characteristics and Outcomes of Patients with Systemic Lupus Erythematosus-associated Thrombotic Microangiopathy, and Their Acquired ADAMTS13 Inhibitor Profiles.
Child-onset and adolescent-onset acquired thrombotic thrombocytopenic purpura with severe ADAMTS13 deficiency: a cohort study of the French national registry for thrombotic microangiopathy.
Child-onset thrombotic thrombocytopenic purpura caused by p.R498C and p.G259PfsX133 mutations in ADAMTS13.
Children and adults with thrombotic thrombocytopenic purpura associated with severe, acquired Adamts13 deficiency: comparison of incidence, demographic and clinical features.
Circulating DNA and myeloperoxidase indicate disease activity in patients with thrombotic microangiopathies.
Cleavage of von Willebrand factor requires the spacer domain of the metalloprotease ADAMTS13.
Clinical and laboratory diagnosis of TTP: an integrated approach.
Clinical characteristics and outcomes of thrombotic microangiopathy in Malaysia.
Clinical features and outcomes in patients with thrombotic microangiopathy not associated with severe ADAMTS13 deficiency.
Clinical features and prognostic factors of thrombotic thrombocytopenic purpura associated with systemic lupus erythematosus: a literature review of 105 cases from 1999 to 2011.
Clinical features of severe acquired ADAMTS13 deficiency in thrombotic thrombocytopenic purpura: the Korean TTP registry experience.
Clinical insights from observations on ADAMTS13 deficiency in liver cirrhosis.
Clinical utility of ADAMTS-13 testing in suspected thrombotic microangiopathy: an audit of ADAMTS-13 activity assay requests in routine practice from a tertiary hospital.
Comparison of Clinical Scoring Systems in the Management of Patients with Microangiopathic Hemolytic Anemia and Thrombocytopenia
Complement Activation Associated with ADAMTS13 Deficiency in Human and Murine Thrombotic Microangiopathy.
Complement activation associated with ADAMTS13 deficiency may contribute to the characteristic glomerular manifestations in Upshaw-Schulman syndrome.
Complement activation, inflammation and relative ADAMTS13 deficiency in secondary thrombotic microangiopathies.
Complement activation: the missing link between ADAMTS-13 deficiency and microvascular thrombosis of thrombotic microangiopathies.
Complement factor H mutation in familial thrombotic thrombocytopenic purpura with ADAMTS13 deficiency and renal involvement.
Complete ADAMTS13 remission in a patient with refractory autoimmune-mediated thrombotic thrombocytopenic purpura after infliximab.
Complete deficiency in ADAMTS13 is prothrombotic, but it alone is not sufficient to cause thrombotic thrombocytopenic purpura.
Congenital ADAMTS-13 deficiency presenting as life-threatening thrombosis during pregnancy.
Congenital ADAMTS13 Deficiency: A Rare Mimicker of Immune Thrombocytopenic Purpura.
Congenital and acquired ADAMTS13 deficiency: Two mechanisms, one patient.
Congenital thrombotic thrombocytopenic purpura caused by new compound heterozygous mutations of the ADAMTS13 gene.
Correction of ADAMTS13 deficiency by in utero gene transfer of lentiviral vector encoding ADAMTS13 genes.
Correction of murine ADAMTS13 deficiency by hematopoietic progenitor cell-mediated gene therapy.
Corticosteroids and rituximab as adjunctive treatments for thrombotic thrombocytopenic purpura.
Current concepts in thrombotic thrombocytopenic purpura.
Current insight into thrombotic thrombocytopenic purpura.
Current insights into thrombotic microangiopathies: Thrombotic thrombocytopenic purpura and pregnancy.
Current management and therapeutical perspectives in thrombotic thrombocytopenic purpura.
Current management of thrombotic thrombocytopenic purpura.
Cyclosporin A impairs the secretion and activity of ADAMTS13 (a disintegrin and metalloprotease with thrombospondin type 1 repeat).
Cyclosporin A therapy on idiopathic thrombotic thrombocytopenic purpura in the relapse setting: two case reports and a review of the literature.
De Novo Mutation of the ADAMTS13 Gene with Mesenteric Ischemia in an Infant with Congenital Thrombotic Thrombocytopenic Purpura.
Decreased a disintegrin-like and metalloprotease with thrombospondin (ADAMTS)-13 is associated with a poor prognosis in sepsis-induced organ failure*
Decreased ADAMTS-13 (A disintegrin-like and metalloprotease with thrombospondin type 1 repeats) is associated with a poor prognosis in sepsis-induced organ failure.
Deficiency of ADAMTS-13 in pediatric patients with severe sepsis and impact on in-hospital mortality.
Derivation and external validation of the PLASMIC score for rapid assessment of adults with thrombotic microangiopathies: a cohort study.
Development and validation of a multivariable prediction rule for detecting a severe acquired ADAMTS13 activity deficiency in patients with thrombotic microangiopathies.
Development and validation of a predictive model for death in acquired severe ADAMTS13 deficiency-associated idiopathic thrombotic thrombocytopenic purpura: the French TMA Reference Center experience.
Development of a severe von Willebrand factor/ADAMTS13 dysbalance during orthotopic liver transplantation.
Diagnostic and prognostic values of ADAMTS13 activity measured during daily plasma exchange therapy in patients with acquired thrombotic thrombocytopenic purpura.
Diagnostic and therapeutic challenges in the thrombotic thrombocytopenic purpura and hemolytic uremic syndromes.
Different disparities of gender and race among the thrombotic thrombocytopenic purpura and hemolytic-uremic syndromes.
Differentiating malignant hypertension-induced thrombotic microangiopathy from thrombotic thrombocytopenic purpura.
Difficulties in diagnosing congenital thrombotic thrombocytopenic purpura.
Disseminated intravascular coagulation: is it fact or fancy?
Does corticosteroid treatment cause prolonged recovery and increased total bilirubin level in severe ADAMTS-13-deficient TTP patient?
Does severe ADAMTS13 deficiency in thrombotic microangiopathy rule out complement-mediated atypical hemolytic uremic syndrome.
Dynamic changes in absolute immature platelet count suggest the presence of a coexisting immune process in the setting of thrombotic thrombocytopenic purpura.
Dynamics of von Willebrand factor reactivity in sickle cell disease during vaso-occlusive crisis and steady state.
Early response to caplacizumab and rituximab after anaphylaxis to Octaplas plasma in a patient with thrombotic thrombocytopenic purpura.
Effects of naturally occurring mutations in CUB-1 domain on synthesis, stability, and activity of ADAMTS-13.
Efficacy and Safety Profile of Solvent/Detergent Plasma in the Treatment of Acute Thrombotic Thrombocytopenic Purpura: A Single-Center Experience.
Efficacy of rituximab in acute refractory or chronic relapsing non-familial idiopathic thrombotic thrombocytopenic purpura: a systematic review with pooled data analysis.
Epidemiology and pathophysiology of adulthood-onset thrombotic microangiopathy with severe ADAMTS13 deficiency (thrombotic thrombocytopenic purpura): a cross-sectional analysis of the French national registry for thrombotic microangiopathy.
Epitope mapping of ADAMTS13 autoantibodies in acquired thrombotic thrombocytopenic purpura.
Evaluating the impact of the ABO blood group on the clinical outcome of thrombotic thrombocytopenic purpura associated with severe ADAMTS13 deficiency.
Evaluation and clinical application of a new method for measuring activity of von Willebrand factor-cleaving metalloprotease (ADAMTS13).
Evaluation of von Willebrand factor and ADAMTS-13 antigen and activity levels in sickle cell disease patients in Kuwait.
Evidence for a role of anti-ADAMTS13 autoantibodies despite normal ADAMTS13 activity in recurrent thrombotic thrombocytopenic purpura.
Expression and characterization of recombinant human ADAMTS-13.
External validation of the PLASMIC score: a clinical prediction tool for thrombotic thrombocytopenic purpura diagnosis and treatment.
Familial acquired thrombotic thrombocytopenic purpura: ADAMTS13 inhibitory autoantibodies in identical twins.
Fatal case of chikungunya and concomitant thrombotic thrombocytopenic purpura in French Guiana during air flight medical evacuation.
Fatal congenital thrombotic thrombocytopenic purpura with apparent ADAMTS13 inhibitor: in vitro inhibition of ADAMTS13 activity by hemoglobin.
FRETS-VWF73 rather than CBA assay reflects ADAMTS13 proteolytic activity in acquired thrombotic thrombocytopenic purpura patients.
FRETS-VWF73, a first fluorogenic substrate for ADAMTS13 assay.
From humoral rejection to generalized thrombotic microangiopathy--role of acquired ADAMTS13 deficiency in a renal allograft recipient.
Generation of Anti-Murine ADAMTS13 Antibodies and Their Application in a Mouse Model for Acquired Thrombotic Thrombocytopenic Purpura.
Haemoglobin blocks von Willebrand factor proteolysis by ADAMTS-13: a mechanism associated with sickle cell disease.
Hematocrit and C-reactive protein predict treatment response times in ADAMTS13-deficient thrombotic microangiopathy.
Hemolytic uremic syndrome with Mycoplasma pneumoniae infection and membrane cofactor protein mutation – case report.
Heparins that block VEGF-A-mediated von Willebrand factor fiber generation are potent inhibitors of hematogenous but not lymphatic metastasis.
Hereditary ADAMTS 13 deficiency presenting as recurrent acute kidney injury.
High Frequency of Acquired ADAMTS13 Deficiency After Hemolysis in Hemiscorpius Lepturus (Scorpion) Stung Children.
Highly elevated plasma level of von Willebrand factor accelerates the formation of platelet thrombus under high shear stress in plasma with deficient ADAMTS13 activity.
How I treat patients with thrombotic thrombocytopenic purpura - 2010.
Human immunodeficiency virus-associated thrombotic microangiopathies: clinical characteristics and outcome according to ADAMTS13 activity.
Human neutrophil peptides and complement factor Bb in pathogenesis of acquired thrombotic thrombocytopenic purpura.
Idiopathic Noncirrhotic Intrahepatic Portal Hypertension is Associated with Sustained ADAMTS13 Deficiency.
Immature platelet dynamics correlate with ADAMTS13 deficiency and predict therapy response in immune-mediated thrombotic thrombocytopenic purpura.
Impact of a multidisciplinary team for the management of thrombotic microangiopathy.
Impact of severe ADAMTS13 deficiency on clinical presentation and outcomes in patients with thrombotic microangiopathies: the experience of the Harvard TMA Research Collaborative.
Implementation of a rapid assay of ADAMTS13 activity was associated with improved 30-day survival rate in patients with acquired primary thrombotic thrombocytopenic purpura who received platelet transfusions.
In-vitro and in-vivo consequences of mutations in the von Willebrand factor cleaving protease ADAMTS13 in thrombotic thrombocytopenic purpura.
Inactivation of ADAMTS13 by plasmin as a potential cause of thrombotic thrombocytopenic purpura.
Increased urinary albumin excretion following recovery from thrombotic thrombocytopenic purpura due to acquired ADAMTS13 deficiency.
Increased Von Willebrand factor, decreased ADAMTS13 and thrombocytopenia in melioidosis.
Increased VWF and Decreased ADAMTS-13 in COVID-19: Creating a Milieu for (Micro)Thrombosis.
Indicators Differentiating Thrombotic Thrombocytopenic Purpura From Other Thrombotic Microangiopathies in a Canadian Apheresis Referral Center.
Inflammation-associated ADAMTS13 deficiency promotes formation of ultra-large von Willebrand factor.
Influence of ADAMTS13 deficiency on venous thrombosis in mice.
Inherited ADAMTS13 deficiency (Upshaw-Schulman syndrome): a short review.
Inherited ADAMTS13 deficiency: unique presentation and treatment.
Inherited ADAMTS13 deficiency: Unique presentation and treatment.
Inherited Thrombotic Thrombocytopenic Purpura (Upshaw Schulman Syndrome) as Differential Diagnosis to Neonatal Septicaemia with Disseminated Intravascular Coagulation - a Case Series.
Inherited thrombotic thrombocytopenic purpura in pregnancy.
Inhibitory anti ADAMTS13 antibodies with a new rapid fully automated CLiA assay.
Inhibitory autoantibodies against ADAMTS-13 in patients with thrombotic thrombocytopenic purpura bind ADAMTS-13 protease and may accelerate its clearance in vivo.
Insights into 3D Structure of ADAMTS13: A Stepping Stone towards Novel Therapeutic Treatment of Thrombotic Thrombocytopenic Purpura.
Interaction between Multimeric von Willebrand Factor and Complement: A Fresh Look to the Pathophysiology of Microvascular Thrombosis.
Interferon-? is not elevated in idiopathic thrombotic thrombocytopenic purpura patients.
Invited commentary to: ADAMTS13 deficiency is associated with abnormal distribution of von Willebrand factor multimers in patients with COVID-19 by Tiffany Pascreau et al. Letter to the Editors-in-Chief, Thrombosis Research.
Is ADAMTS-13 deficiency specific for thrombotic thrombocytopenic purpura? No.
Is factor V Leiden a risk factor for thrombotic microangiopathies without severe ADAMTS 13 deficiency?
Keeping von Willebrand Factor under Control: Alternatives for ADAMTS13.
Lessons learned from the Oklahoma thrombotic thrombocytopenic purpura-hemolytic uremic syndrome registry.
Letter by Hugenholtz and Lisman regarding article, "plasmin cleavage of von Willebrand factor as an emergency bypass for ADAMTS13 deficiency in thrombotic microangiopathy".
Limited renal prophylaxis in regular plasmatherapy for heritable ADAMTS13 deficiency.
Link between von Willebrand factor multimers, relapses and coronary microcirculation in patients with thrombotic thrombocytopenic purpura in remission.
Long-term deficits in health-related quality of life after recovery from thrombotic thrombocytopenic purpura.
Macrovascular thrombosis in critically ill patients with thrombotic micro-angiopathies.
Malignant Hypertension with Thrombotic Microangiopathy.
Management of thrombotic thrombocytopenic purpura.
Measurement of ADAMTS13 activity and inhibitors.
Measurement of von Willebrand factor-cleaving protease (ADAMTS-13) activity in plasma: a multicenter comparison of different assay methods.
Mechanisms of the interaction between two ADAMTS13 gene mutations leading to severe deficiency of enzymatic activity.
Mild to moderate reduction of a von Willebrand factor cleaving protease (ADAMTS-13) in pregnant women with HELLP microangiopathic syndrome.
Modifier genes for disorders of thrombosis and hemostasis.
Modulation of ADAMTS13 secretion and specific activity by a combination of common amino acid polymorphisms and a missense mutation.
Mutation analysis and clinical implications of von Willebrand factor-cleaving protease deficiency.
Myocardial infarction is a complication of factor H-associated atypical HUS.
Neutrophil Protease Cleavage of Von Willebrand Factor in Glomeruli - An Anti-thrombotic Mechanism in the Kidney.
Non-O blood group thrombotic thrombocytopenic purpura patients take longer to recover as measured by number of therapeutic plasma exchanges needed for platelet recovery.
Nonsense-mediated mRNA decay in the ADAMTS13 gene caused by a 29-nucleotide deletion.
Novel ADAMTS13 mutations in an obstetric patient with upshaw-schulman syndrome.
Novel, semi-automated, 60-min-assay to determine von Willebrand factor cleaving activity of ADAMTS-13.
Orthotopic liver transplantation (OLTx) in non-cirrhotic portal hypertension secondary to ADAMTS13 deficiency.
Pancreatitis preceding acute episodes of thrombotic thrombocytopenic purpura-hemolytic uremic syndrome: report of five patients with a systematic review of published reports.
Paradigm shift of childhood thrombotic thrombocytopenic purpura with severe ADAMTS13 deficiency.
Partial ADAMTS13 deficiency in atypical hemolytic uremic syndrome.
Pathogenesis of thrombotic thrombocytopenic purpura.
Pathogenesis of thrombotic thrombocytopenic purpura: ADAMTS13 deficiency and beyond.
Pathogenicity of Anti-ADAMTS13 Autoantibodies in Acquired Thrombotic Thrombocytopenic Purpura.
Pathophysiology of thrombotic thrombocytopenic purpura and hemolytic uremic syndrome.
Pathophysiology of thrombotic thrombocytopenic purpura.
Patients with localized and disseminated tumors have reduced but measurable levels of ADAMTS-13 (von Willebrand factor cleaving protease).
Pediatric thrombotic thrombocytopenic purpura.
Performance of a clinical prediction score for thrombotic thrombocytopenic purpura in an independent cohort.
Plasma therapy in thrombotic thrombocytopenic purpura: review of the literature and the Bern experience in a subgroup of patients with severe acquired ADAMTS-13 deficiency.
Plasmic score applicability for the diagnosis of thrombotic microangiopathy associated with ADAMTS13-acquired deficiency in a developing country.
Plasmin Cleavage of von Willebrand Factor as an Emergency Bypass for ADAMTS13 Deficiency in Thrombotic Microangiopathy.
Platelet Reactive Conformation and Multimeric Pattern of von Willebrand Factor in Acquired Thrombotic Thrombocytopenic Purpura During Acute Disease and Remission.
Platelet rescue by macrophage depletion in obese ADAMTS-13-deficient mice at risk of thrombotic thrombocytopenic purpura.
Platelets: thrombotic thrombocytopenic purpura.
Porto-pulmonary hypertension exacerbated by platelet transfusion in a patient with ADAMTS13 deficiency.
Post-partum management in a patient affected by thrombotic thrombocytopenic purpura: case report and review of literature.
Posttransplantation thrombotic thrombocytopenic purpura: a single-center experience and a contemporary review.
Predictive features of severe acquired ADAMTS13 deficiency in idiopathic thrombotic microangiopathies: the French TMA reference center experience.
Predictive Value of Schistocytes in Recurrence of Acquired Thrombotic Thrombocytopenic Purpura With Severe ADAMTS13 Deficiency at Discontinuation of Daily Therapeutic Plasma Exchange.
Preemptive rituximab infusions after remission efficiently prevent relapses in acquired thrombotic thrombocytopenic purpura.
Preemptive rituximab prevents long-term relapses in immune-mediated thrombotic thrombocytopenic purpura.
Pregnancy outcomes following recovery from acquired thrombotic thrombocytopenic purpura.
Presence of ADAMTS13 activity in a patient with metastatic cancer and thrombotic microangiopathy.
Presence of anti-ADAMTS13 antibodies in obesity.
Preserved Expression of mRNA Coding von Willebrand Factor-Cleaving Protease ADAMTS13 by Selenite and Activated Protein C.
Prevention of relapse in patients with acquired thrombotic thrombocytopenic purpura undergoing elective surgery: a case series.
Proteolytic inactivation of ADAMTS13 by plasmin in human plasma: risk of thrombotic thrombocytopenic purpura.
Recent advances in thrombotic thrombocytopenic purpura.
Reduced von Willebrand factor-cleaving protease levels in secondary thrombotic microangiopathies and other diseases.
Refractory thrombotic thrombocytopenic purpura associated with oral contraceptives and factor V Leiden: a case report.
Refractory thrombotic thrombocytopenic purpura following influenza vaccination.
Relapsing or refractory idiopathic thrombotic thrombocytopenic purpura-hemolytic uremic syndrome: the role of rituximab.
Remission of thrombotic thrombocytopenic purpura in a patient with compound heterozygous deficiency of von Willebrand factor-cleaving protease by infusion of solvent/detergent plasma.
Response to letter regarding article, "plasmin cleavage of von willebrand factor as an emergency bypass for ADAMTS13 deficiency in thrombotic microangiopathy".
Ribosomal and Immune Transcripts Associate with Relapse in Acquired ADAMTS13-Deficient Thrombotic Thrombocytopenic Purpura.
Rituximab for acute plasma-refractory thrombotic thrombocytopenic purpura. A case report and concise review of the literature.
Rituximab for refractory and or relapsing thrombotic thrombocytopenic purpura related to immune-mediated severe ADAMTS13-deficiency: a report of four cases and a systematic review of the literature.
Rituximab in a child with autoimmune thrombotic thrombocytopenic purpura refractory to plasma exchange.
Rituximab prolongs the time to relapse in patients with immune thrombotic thrombocytopenic purpura: analysis of off-label use in Japan.
Rituximab-refractory thrombotic thrombocytopenic purpura responsive to intravenous but not subcutaneous bortezomib.
Role of ADAMTS13 in diet-induced liver steatosis.
Role of ADAMTS13 in the management of thrombotic microangiopathies including thrombotic thrombocytopenic purpura (TTP).
Role of ADAMTS13 in the pathogenesis, diagnosis, and treatment of thrombotic thrombocytopenic purpura.
Role of CD40 and ADAMTS13 in von Willebrand factor-mediated endothelial cell-platelet-monocyte interaction.
Schistocytic anaemia, severe thrombocytopenia, and renal dysfunction: thrombotic microangiopathy due to severe acquired ADAMTS-13 deficiency. Case 2.
Secondary thrombotic microangiopathy with severely reduced ADAMTS13 activity in a patient with Capnocytophaga canimorsus sepsis: a case report.
Sepsis-Induced Disseminated Intravascular Coagulation With Features of Thrombotic Thrombocytopenic Purpura: a Fatal Fulminant Syndrome.
Severe ADAMTS-13 deficiency in childhood.
Severe ADAMTS13 deficiency in adult idiopathic thrombotic microangiopathies defines a subset of patients characterized by various autoimmune manifestations, lower platelet count, and mild renal involvement.
Severe deficiency of the specific von Willebrand factor-cleaving protease (ADAMTS 13) activity in a subgroup of children with atypical hemolytic uremic syndrome.
Severe deficiency of VWF-cleaving protease (ADAMTS13) activity defines a distinct population of thrombotic microangiopathy patients.
Severe Hemolysis and Pulmonary Hypertension in a Neonate With Upshaw-Schulman Syndrome.
Severe secondary deficiency of von Willebrand factor-cleaving protease (ADAMTS13) in patients with sepsis-induced disseminated intravascular coagulation: its correlation with development of renal failure.
Severe transient ADAMTS13 deficiency in pneumococcal-associated hemolytic uremic syndrome.
Shigatoxin triggers thrombotic thrombocytopenic purpura in genetically susceptible ADAMTS13-deficient mice.
Short- and long-term effects of rituximab for the treatment of thrombotic thrombocytopenic purpura: four case reports.
Silent Thrombotic Thrombocytopenic Purpura: PLASMIC, Lessons Learned, and Current Management Overview.
Specific von Willebrand factor-cleaving protease in thrombotic microangiopathies: a study of 111 cases.
Splenectomy and/or cyclophosphamide as salvage therapies in thrombotic thrombocytopenic purpura: the French TMA Reference Center experience.
Splenectomy in relapsing and plasma-refractory acquired thrombotic thrombocytopenic purpura.
Spontaneous recovery in a patient with acquired thrombotic thrombocytopenic purpura (TTP): observation of a 'subclinical' TTP state.
STEC-HUS, atypical HUS and TTP are all diseases of complement activation.
Structural and functional correlation of ADAMTS13.
Successful treatment of neonatal atypical hemolytic uremic syndrome with C5 monoclonal antibody.
Successful treatment with rituximab for acute refractory thrombotic thrombocytopenic purpura related to acquired ADAMTS13 deficiency: A pediatric report and literature review.
Survival and relapse in patients with thrombotic thrombocytopenic purpura.
Synergistic effects of ADAMTS13 deficiency and complement activation in pathogenesis of thrombotic microangiopathy.
Synergistic interactions between interferon-gamma and TRAIL modulate c-FLIP in endothelial cells, mediating their lineage-specific sensitivity to thrombotic thrombocytopenic purpura plasma-associated apoptosis.
Systemic antithrombotic effects of ADAMTS13.
Systemic infections mimicking thrombotic thrombocytopenic purpura.
Taking Empiricism out of Immune Thrombotic Thrombocytopenic Purpura: Current and Future Treatment Strategies.
Ten patient stories illustrating the extraordinarily diverse clinical features of patients with thrombotic thrombocytopenic purpura and severe ADAMTS13 deficiency.
The active conformation of von Willebrand factor in patients with thrombotic thrombocytopenic purpura in remission.
The balance between von-Willebrand factor and its cleaving protease ADAMTS13: biomarker in systemic inflammation and development of organ failure?
The combined roles of ADAMTS13 and VWF in murine models of TTP, endotoxemia, and thrombosis.
The course of ADAMTS-13 activity and inhibitor titre in the treatment of thrombotic thrombocytopenic purpura with plasma exchange and vincristine.
The first deletion mutation in the TSP1-6 repeat domain of ADAMTS13 in a family with inherited thrombotic thrombocytopenic purpura.
The function of ADAMTS13 in thrombogenesis in vivo: insights from mutant mice.
The incidence of thrombotic thrombocytopenic purpura-hemolytic uremic syndrome: all patients, idiopathic patients, and patients with severe ADAMTS-13 deficiency.
The Oklahoma Thrombotic Thrombocytopenic Purpura-Hemolytic Uremic Syndrome (TTP-HUS) Registry: a community perspective of patients with clinically diagnosed TTP-HUS.
The Oklahoma Thrombotic Thrombocytopenic Purpura-Hemolytic Uremic Syndrome Registry: the Swiss connection.
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 remarkable diversity of thrombotic thrombocytopenic purpura: a perspective.
The role of ADAMTS-13 in the coagulopathy of sepsis.
The role of ADAMTS13 in the pathogenesis of thrombotic thrombocytopenic purpura-hemolytic uremic syndrome.
The splenic autoimmune response to ADAMTS13 in thrombotic thrombocytopenic purpura contains recurrent antigen-binding CDR3 motifs.
The thrombotic microangiopathies.
The thrombotic thrombocytopenic purpura and hemolytic uremic syndromes: evaluation, management, and long-term outcomes experience of the Oklahoma TTP-HUS Registry, 1989-2007.
The thrombotic thrombocytopenic purpura and hemolytic uremic syndromes: overview of pathogenesis (Experience of The Oklahoma TTP-HUS Registry, 1989-2007).
The utility of patient characteristics in predicting severe ADAMTS13 deficiency and response to plasma exchange.
Thrombocytopenia and severe hyperbilirubinemia in the neonatal period secondary to congenital thrombotic thrombocytopenic purpura and ADAMTS13 deficiency.
Thrombocytopenia in hospitalized patients: approach to the patient with thrombotic microangiopathy.
Thrombogenesis and thrombotic disorders based on 'two-path unifying theory of hemostasis': philosophical, physiological, and phenotypical interpretation.
Thrombospondin-1 and ADAMTS13 competitively bind to VWF A2 and A3 domains in vitro.
Thrombotic microangiopathies: towards a pathophysiology-based classification.
Thrombotic microangiopathy due to acquired ADAMTS13 deficiency in a patient receiving interferon-beta treatment for multiple sclerosis.
Thrombotic microangiopathy with acquired deficiency in ADAMTS 13 activity in lung transplant recipients.
Thrombotic thrombocytopenic purpura and acquired immunodeficiency syndrome diagnosed in pregnancy: Case report.
Thrombotic Thrombocytopenic Purpura and Anti-Thrombotic Therapy Targeted to Von Willebrand Factor.
Thrombotic thrombocytopenic purpura developed during the conservative treatment of anti-phospholipase A2 receptor antibody-positive idiopathic membranous nephropathy: a case report.
Thrombotic thrombocytopenic purpura in a patient with rapidly progressive glomerulonephritis with both anti-glomerular basement membrane antibodies and myeloperoxidase anti-neutrophil cytoplasmic antibodies.
Thrombotic thrombocytopenic purpura in humans and mice.
Thrombotic thrombocytopenic purpura related to ADAMTS13 deficiency, and successful treatment in a chimpanzee (Pan troglodytes verus).
Thrombotic thrombocytopenic purpura related to severe ADAMTS13 deficiency in children.
Thrombotic Thrombocytopenic Purpura with Severe ADAMTS-13 Deficiency in a Patient with Antiphospholipid Antibodies and Charcot-Marie-Tooth Disease.
Thrombotic thrombocytopenic purpura with severe ADAMTS-13 deficiency in two patients with primary antiphospholipid syndrome.
Thrombotic thrombocytopenic purpura.
Thrombotic thrombocytopenic purpura: a moving target.
Thrombotic thrombocytopenic purpura: A rare complication of acute pancreatitis.
Thrombotic thrombocytopenic purpura: a thrombotic disorder caused by ADAMTS13 deficiency.
Thrombotic thrombocytopenic purpura: basic pathophysiology and therapeutic strategies.
Thrombotic thrombocytopenic purpura: from diagnosis to therapy.
Thrombotic Thrombocytopenic Purpura: Pathophysiology, Diagnosis, and Management.
Thrombotic thrombocytopenic purpura: proposal of a new pathogenic mechanism involving Helicobacter pylori infection.
Thrombotic thromboytopenic purpura: another example of immunomediated thrombosis.
Treatment of autoimmune thrombotic thrombocytopenic purpura in the more severe forms.
Treatment of thrombotic thrombocytopenic purpura beyond therapeutic plasma exchange.
Treatment with or without plasma exchange for patients with acquired thrombotic microangiopathy not associated with severe ADAMTS13 deficiency: a propensity score-matched study.
TTP and ADAMTS13: When Is Testing Appropriate?
TTP-like syndrome: novel concept and molecular pathogenesis of endotheliopathy-associated vascular microthrombotic disease.
TTP/HUS and prognosis: the syndrome and the disease(s).
Twice-daily therapeutical plasma exchange-based salvage therapy in severe autoimmune thrombotic thrombocytopenic purpura: the French TMA Reference Center experience.
Two cases of refractory thrombotic thrombocytopenic purpura associated with collagen vascular disease were significantly improved by rituximab treatment.
Two generations with familial thrombotic thrombocytopenic purpura.
Two novel ADAMTS13 gene mutations in thrombotic thrombocytopenic purpura/hemolytic-uremic syndrome (TTP/HUS).
Ultralarge von Willebrand factor multimers and normal ADAMTS13 activity in the umbilical cord blood.
Unbalanced expression of ADAMTS13 and von Willebrand factor in mouse endotoxinemia.
Unexpected frequency of Upshaw-Schulman syndrome in pregnancy-onset thrombotic thrombocytopenic purpura.
Unique case of autoantibody mediated inactivation of ADAMTS13 in an Indian TTP patient.
Unresponsive Thrombotic Thrombocytopenic Purpura (TTP): Challenges and Solutions.
Validation of PLASMIC score and follow-up data in a cohort of patients with suspected microangiopathies from Southern Italy.
Validation of PLASMIC score: an academic medical center case series (2012-present).
Validation of the PLASMIC score for predicting ADAMTS13 activity <10% in patients with suspected thrombotic thrombocytopenic purpura in Alberta, Canada.
Validation of the PLASMIC score, a clinical prediction tool for thrombotic thrombocytopenic purpura diagnosis, in Chinese patients.
Von Willebrand factor and ADAMTS13 impact on the outcome of Staphylococcus aureus sepsis.
von Willebrand factor and thrombotic thrombocytopenic purpura.
von Willebrand factor cleaving protease (ADAMTS-13) and ADAMTS-13 neutralizing autoantibodies in 100 patients with thrombotic thrombocytopenic purpura.
Von Willebrand factor cleaving protease (ADAMTS-13) in 123 patients with connective tissue diseases (systemic lupus erythematosus and systemic sclerosis).
von Willebrand factor cleaving protease (ADAMTS13) is deficient in recurrent and familial thrombotic thrombocytopenic purpura and hemolytic uremic syndrome.
Von Willebrand factor, ADAMTS13, and thrombotic thrombocytopenic purpura.
von Willebrand factor, von Willebrand factor-cleaving protease, and shear stress.
von Willebrand factor-cleaving protease (ADAMTS-13) activity determination in the diagnosis of thrombotic microangiopathies: the Swiss experience.
Von Willebrand factor-cleaving protease (ADAMTS-13) activity in thrombotic microangiopathies: diagnostic experience 2001/2002 of a single research laboratory.
von Willebrand factor-cleaving protease (ADAMTS13) in the course of stem cell transplantation.
Von Willebrand factor-cleaving protease (ADAMTS13) in thrombocytopenic disorders: a severely deficient activity is specific for thrombotic thrombocytopenic purpura.
von Willebrand factor-cleaving protease ADAMTS13 reduces ischemic brain injury in experimental stroke.
von Willebrand factor-cleaving protease in thrombotic thrombocytopenic purpura and the hemolytic-uremic syndrome.
VWF excess and ADAMTS13 deficiency: a unifying pathomechanism linking inflammation to thrombosis in DIC, malaria, and TTP.
What's new in the diagnosis and pathophysiology of thrombotic thrombocytopenic purpura.
[A child with relapsing haemolytic anemia and thrombocytopenia]
[Acquired idiopathic thrombotic thrombocytopenic purpura: arguments for an autoimmune disease]
[Acute renal failure and thrombotic microangiopathy (TM)]
[Atypical hemolytic and uremic syndrome associated with von Willebrand factor-cleaving protease (ADAMTS 13) deficiency in children].
[Determination of the ADAMTS13 antigen and its activity in TTP patients and carriers]
[Diagnosis of thrombotic thrombocytopenic purpura].
[Efficacy of eculizumab in a case of pregnancy-associated aHUS].
[Frontline clinical practice for thrombotic thrombocytopenic purpura].
[Genetic polymorphism of von Willebrand factor (VWF)-cleaving protease, ADAMTS13]
[Hemolytic and uremic syndrome in the child]
[Platelet--vessel wall interactions]
[Post-transfusion acute lung injury (Trali) after plasma infusion in a patient having a constitutional thrombotic microangiopathy]
[Thrombotic thrombocytopenic purpura: Do not ignore cardiac involvement.]
[Transplantation related ADAMTS13 deficiency in thrombotic thrombocytopenic purpura: a case report and literature review].
[Treatment of immune-mediated thrombotic thrombocytopenic purpura: A decisive turning point].
[Von Willebrand factor and ADAMTS13 balancing primary haemostasis.]
Albuminuria
Therapeutic application of recombinant human ADAMTS-13 improves shock reversal and coagulation status in a trauma hemorrhage and transfusion rat model.
Anemia
A Case of Thrombotic Thrombocytopenia Purpura Associated with Systemic Lupus Erythematosus: Diagnostic Utility of ADAMTS-13 Activity.
A journey from sickle cell anemia to ADAMTS13.
Autoimmune Thrombotic Thrombocytopenic Purpura: Two Rare Cases Associated with Juvenile Idiopathic Arthritis and Multiple Sclerosis.
C3 Glomerulopathy and Atypical Hemolytic Uremic Syndrome: Two Important Manifestations of Complement System Dysfunction.
Clinical application of a rapid method using agarose gel electrophoresis and Western blotting to evaluate von Willebrand factor protease activity.
Early response to caplacizumab and rituximab after anaphylaxis to Octaplas plasma in a patient with thrombotic thrombocytopenic purpura.
Hereditary Thrombotic Thrombocytopenic Purpura in a 9-Month Old: Diagnosing and Managing an Ultra-rare Disorder.
Microangiopathic Hemolytic Anemia Due to ADAMTS-13 Loss in Idiopathic Systemic Capillary Leak Syndrome.
Mutation of the H-bond acceptor S119 in the ADAMTS13 metalloprotease domain reduces secretion and substrate turnover in a patient with congenital thrombotic thrombocytopenic purpura.
Prasugrel and Acquired Thrombotic Thrombocytopenic Purpura Associated with ADAMTS13 Activity Deficiency.
Presumed complement-mediated, checkpoint inhibitor-induced, thrombotic microangiopathy in a patient with metastatic melanoma.
Rituximab as pre-emptive treatment in patients with thrombotic thrombocytopenic purpura and evidence of anti-ADAMTS13 autoantibodies.
Schistocytic anaemia, severe thrombocytopenia, and renal dysfunction: thrombotic microangiopathy due to severe acquired ADAMTS-13 deficiency. Case 2.
Thrombotic thrombocytopenic purpura and other thrombotic microangiopathic hemolytic anemias: Diagnosis and classification.
[A CASE REPORT OF THROMBOTIC THROMBOCYTOPENIC PURPURA (TTP) IN A BREAST CANCER PATIENT].
Anemia, Hemolytic
A case of pregnancy-induced thrombotic thrombocytopenic purpura with a kidney allograft recipient.
ADAMTS-13 activity, microangiopathic haemolytic anaemia and thrombocytopenia following snake bite envenomation.
ADAMTS13 mutations and polymorphisms in congenital thrombotic thrombocytopenic purpura.
An Imperfect Marker: SLE and TTP-Like MAHA Without Low ADAMTS13.
Assessment and Monitoring of Patients with Immune-Mediated Thrombotic Thrombocytopenic Purpura (iTTP): Strategies to Improve Outcomes.
Caplacizumab Treatment for Acquired Thrombotic Thrombocytopenic Purpura.
Case Report: Microangiopathic Hemolytic Anemia With Normal ADAMTS13 Activity.
Complete deficiency in ADAMTS13 is prothrombotic, but it alone is not sufficient to cause thrombotic thrombocytopenic purpura.
Congenital ADAMTS13 Deficiency: A Rare Mimicker of Immune Thrombocytopenic Purpura.
Congenital microangiopathic hemolytic anemia and thrombocytopenia with unusually large von Willebrand factor multimers and von Willebrand factor-cleaving protease.
Congenital Thrombotic Thrombocytopenic Purpura: Atypical Presentation and New ADAMTS 13 Mutation in a Tunisian Child.
Current management and therapeutical perspectives in thrombotic thrombocytopenic purpura.
EB virus reactivation triggers thrombotic thrombocytopenic purpura in a healthy adult.
Impact of a multidisciplinary team for the management of thrombotic microangiopathy.
Infection as Trigger for Congenital Thrombotic Thrombocytopenic Purpura in an Adult Patient.
Inherited thrombotic thrombocytopenic purpura mimicking immune thrombocytopenic purpura during pregnancy: a case report.
Is therapeutic plasma exchange indicated for patients with gemcitabine-induced hemolytic uremic syndrome?
Long-term response to rituximab in patients with relapsing thrombotic thrombocytopenic purpura.
Microangiopathic haemolytic anaemia in metastasizing malignant tumours is not associated with a severe deficiency of the von Willebrand factor-cleaving protease.
Microangiopathic haemolytic anaemia resembling thrombotic thrombocytopenic purpura in systemic lupus erythematosus: the role of ADAMTS13.
Microangiopathic Hemolytic Anemia Due to ADAMTS-13 Loss in Idiopathic Systemic Capillary Leak Syndrome.
Microangiopathic hemolytic anemia due to ADAMTS-13 loss in idiopathic systemic capillary leak syndrome: comment.
Microangiopathic hemolytic anemia due to ADAMTS-13 loss in idiopathic systemic capillary leak syndrome: reply.
Nivolumab-Induced Thrombotic Thrombocytopenic Purpura in a Patient with Anal Squamous Cell Carcinoma: A Lesson on Hematologic Toxicity from Immunotherapy.
Novel therapies in thrombotic thrombocytopenic purpura.
Pathophysiology of thrombotic thrombocytopenic purpura.
Postoperative hemolytic uremic syndrome with renal cortical necrosis following laparoscopic hemicolectomy.
Predictive Value of Schistocytes in Recurrence of Acquired Thrombotic Thrombocytopenic Purpura With Severe ADAMTS13 Deficiency at Discontinuation of Daily Therapeutic Plasma Exchange.
Pregnancy-associated thrombotic thrombocytopenic purpura with anti-centromere antibody-positive Raynaud's Syndrome.
Rapid ADAMTS13 availability impacts treatment for microangiopathic hemolytic anemia and thrombocytopenia.
Recombinant thrombomodulin for secondary thrombotic thrombocytopenic purpura.
Recommendations for the diagnosis and treatment of patients with thrombotic thrombocytopenic purpura.
Rituximab for chronic recurring thrombotic thrombocytopenic purpura: a case report and review of the literature.
Siblings with congenital thrombotic thrombocytopenic purpura.
Spontaneous recovery in a patient with acquired thrombotic thrombocytopenic purpura (TTP): observation of a 'subclinical' TTP state.
The D173G mutation in ADAMTS-13 causes a severe form of congenital thrombotic thrombocytopenic purpura. A clinical, biochemical andin silico study.
The features of acquired thrombotic thrombocytopenic purpura occurring at advanced age.
Therapeutic plasma exchange in thrombotic thrombocytopenic purpura.
Thrombotic microangiopathic hemolytic anemia with reduction of ADAMTS13 activity: initial manifestation of childhood-onset systemic lupus erythematosus.
Thrombotic microangiopathy as first manifestation of acute human immunodeficiency virus infection: a case report and review of the literature.
Thrombotic thrombocytopenic purpura associated to dual checkpoint inhibitor therapy for metastatic melanoma.
Thrombotic thrombocytopenic purpura directly linked with ADAMTS13 inhibition in the baboon (Papio ursinus).
Thrombotic thrombocytopenic purpura: diagnostic criteria, clinical features, and long-term outcomes from 1995 through 2015.
Two novel mutations in ADAMTS13 in a Chinese boy with congenital thrombocytopenic purpura: a case report.
Utilizing a PLASMIC score-based approach in the management of suspected immune thrombotic thrombocytopenic purpura: a cost minimization analysis within the Harvard TMA Research Collaborative.
Von Willebrand factor-cleaving protease (ADAMTS-13) activity in thrombotic microangiopathies: diagnostic experience 2001/2002 of a single research laboratory.
What's new in the diagnosis and pathophysiology of thrombotic thrombocytopenic purpura.
[Activity loss of Von Willebrand factor cleaving protein (ADAMTS-13) is diagnostic for primary and pregnancy-related thrombotic thrombocytopenic purpura]
[ADAMTS13, von Willebrand factor specific cleaving protease].
[Analysis of five children with acquired thrombotic thrombocytopenic purpura].
[Clinical Analysis of 12 cases of Systemic Lupus Erythematosus Associated with Thrombotic Thrombocytopenic Purpura].
[Clopidogrel-associated thrombotic thrombocytopenic purpura].
[Diagnosis and treatment of thrombotic thrombocytopenic purpura].
[Exacerbation of cranial nerurological symptoms by platelet transfusion before the diagnosis of thrombotic thrombocytopenic purpura].
[Frontline clinical practice for thrombotic thrombocytopenic purpura].
[Thrombotic thrombocytopenic purpura after circumcision].
[Thrombotic thrombocytopenic purpura during pregnancy refractory to plasma exchange and rituximab].
[Variety of thrombotic thrombocytopenic purpura clinical course in Polish family members with ADAMTS 13 gene mutation].
Anemia, Macrocytic
ADAMTS-13 deficiency: can it cause chronic renal failure?
Anemia, Pernicious
Association of acquired thrombotic thrombocytopaenic purpura in a patient with pernicious anaemia.
Anemia, Sickle Cell
A journey from sickle cell anemia to ADAMTS13.
Absence of exaggerated pharmacology by recombinant ADAMTS13 in the rat and monkey.
ADAMTS13 activity in sickle cell disease.
ADAMTS13 and von Willebrand factor assessment in steady state and acute vaso-occlusive crisis of sickle cell disease.
Evaluation of von Willebrand factor and ADAMTS-13 antigen and activity levels in sickle cell disease patients in Kuwait.
Haemoglobin blocks von Willebrand factor proteolysis by ADAMTS-13: a mechanism associated with sickle cell disease.
Relationship of Thrombospondin 1 to von Willebrand Factor and ADAMTS-13 in Sickle Cell Disease Patients of Arab Ethnicity.
Thrombospondin-1 inhibits ADAMTS13 activity in sickle cell disease.
Aneurysm
Expression and significance of vWF, GMP-140 and ADAMTS13 in patients with aneurysmal subarachnoid hemorrhage.
Angina, Unstable
Association of the von Willebrand Factor-ADAMTS13 Ratio With Incident Cardiovascular Events in Patients With Peripheral Arterial Disease.
Changes in plasma von Willebrand factor-cleaving protease (ADAMTS13) levels in patients with unstable angina.
Prothrombotic response to coronary angioplasty in patients with unstable angina and raised C-reactive protein.
Anthrax
Degradation of Circulating von Willebrand Factor and Its Regulator ADAMTS13 Implicates Secreted Bacillus anthracis Metalloproteases in Anthrax Consumptive Coagulopathy.
Anti-Glomerular Basement Membrane Disease
Goodpasture's syndrome associated with thrombotic thrombocytopenic purpura secondary to an ADAMTS-13 deficit.
Antiphospholipid Syndrome
ADAMTS-13 gene expression in antiphospholipid syndrome.
ADAMTS-13 metalloprotease abnormalities in systemic lupus erythematosus: is there a correlation with disease status?
Early ADAMTS13 testing associates with pre-eclampsia occurrence in antiphospholipid syndrome.
Management of thrombotic microangiopathy in pregnancy and postpartum: report from an international working group.
Plasma ADAMTS13, von Willebrand Factor (VWF), and VWF Propeptide Profiles in Patients With Connective Tissue Diseases and Antiphospholipid Syndrome.
The VWF/ADAMTS13 axis in the antiphospholipid syndrome: ADAMTS13 antibodies and ADAMTS13 dysfunction.
Thrombotic thrombocytopenic purpura with severe ADAMTS-13 deficiency in two patients with primary antiphospholipid syndrome.
Aortic Valve Stenosis
Changes in von Willebrand factor-cleaving protease (ADAMTS-13) in patients with aortic stenosis undergoing valve replacement or balloon valvuloplasty.
Shear stress and von Willebrand factor in health and disease.
Arthritis
Severe ADAMTS13 deficiency in adult idiopathic thrombotic microangiopathies defines a subset of patients characterized by various autoimmune manifestations, lower platelet count, and mild renal involvement.
Arthritis, Rheumatoid
Heterogeneous pathogenic processes of thrombotic microangiopathies in patients with connective tissue diseases.
Astrocytoma
IL-1? Down-Regulates ADAMTS-13 mRNA Expression in Cells of the Central Nervous System.
Atherosclerosis
ADAMTS13 modulates atherosclerotic plaque progression in mice via a VWF-dependent mechanism.
ADAMTS13 reduces vascular inflammation and the development of early atherosclerosis in mice.
Association of a disintegrin and metalloproteinase with a thrombospondin type 1 motif member 13 polymorphisms with severity of coronary stenosis in type 2 diabetes mellitus.
Genetic ablation of Adamts13 gene dramatically accelerates the formation of early atherosclerosis in a murine model.
Inverse correlations between serum ADAMTS13 levels and systolic blood pressure, pulse pressure, and serum C-reactive protein levels observed at a general health examination in a Japanese population: A cross-sectional study.
Inverse Regulation of Confluence-Dependent ADAMTS13 and von Willebrand Factor Expression in Human Endothelial Cells.
Role of CD40 and ADAMTS13 in von Willebrand factor-mediated endothelial cell-platelet-monocyte interaction.
Simvastatin Increases ADAMTS13 Expression in Podocytes.
Atrial Fibrillation
ADAMTS13--more than just TMA and TTP.
Changes in plasma von Willebrand factor and ADAMTS13 levels associated with left atrial remodeling in atrial fibrillation.
Correlation analysis between ADAMTS-13 gene polymorphism and hypertension-induced atrial fibrillation.
Levels of von Willebrand factor and ADAMTS13 determine clinical outcome after cardioversion for atrial fibrillation.
Prognostic value of plasma von Willebrand factor and its cleaving protease ADAMTS13 in patients with atrial fibrillation.
The Value of ADAMTS13 in Predicting Clinical Outcomes in Patients With Acute Ischemic Stroke Receiving Thrombolysis.
Verification of the Role of ADAMTS13 in the Cardiovascular Disease Using Two-Sample Mendelian Randomization.
Von Willebrand Factor and ADAMTS13 as Predictors of Adverse Outcomes in Patients With Nonvalvular Atrial Fibrillation.
Von Willebrand factor and ADAMTS13 plasma in older patients with high CHA2DS2-VASc Score with and without atrial fibrillation.
Von Willebrand Factor: Multimeric Structure and Functional Activity in Patients With Atrial Fibrillation With and Without Oral Anticoagulation.
Atypical Hemolytic Uremic Syndrome
A novel quantitative hemolytic assay coupled with restriction fragment length polymorphisms analysis enabled early diagnosis of atypical hemolytic uremic syndrome and identified unique predisposing mutations in Japan.
Atypical hemolytic uremic syndrome in first trimester pregnancy successfully treated with eculizumab.
Atypical hemolytic uremic syndrome.
Defining the genetics of thrombotic microangiopathies.
Diabetic ketoacidosis presenting with atypical hemolytic uremic syndrome associated with a variant of complement factor B in an adult: a case report.
Does severe ADAMTS13 deficiency in thrombotic microangiopathy rule out complement-mediated atypical hemolytic uremic syndrome.
Evaluation of a New, Rapid, Fully Automated Assay for the Measurement of ADAMTS13 Activity.
Indications of underdiagnosis of Atypical Hemolytic Uremic Syndrome in a cohort referred to the Coagulation Unit in Malmo, Sweden, for analysis of ADAMTS13 2007-2012.
Management of thrombotic microangiopathy in pregnancy and postpartum: report from an international working group.
Partial ADAMTS13 deficiency in atypical hemolytic uremic syndrome.
Plasma ADAMTS13, von Willebrand Factor (VWF) and VWF Propeptide Profiles in Patients with DIC and Related Diseases.
Postoperative thrombotic thrombocytopenic purpura in an infant: case report and literature review.
Prognostic utility of ADAMTS13 activity for the atypical hemolytic uremic syndrome (aHUS) and comparison of complement serology between aHUS and thrombotic thrombocytopenic purpura.
Severe deficiency of the specific von Willebrand factor-cleaving protease (ADAMTS 13) activity in a subgroup of children with atypical hemolytic uremic syndrome.
Synergistic effects of ADAMTS13 deficiency and complement activation in pathogenesis of thrombotic microangiopathy.
Thrombocytopenia in pregnancy.
Thrombosis, Microangiopathies, and Inflammation.
Thrombotic microangiopathies and the linkage between von Willebrand factor and the alternative complement pathway.
Thrombotic thrombocytopenic purpura-what is new?
Thrombotic thrombocytopenic purpura: pathogenesis, diagnosis and potential novel therapeutics.
Autoimmune Diseases
An open conformation of ADAMTS-13 is a hallmark of acute acquired thrombotic thrombocytopenic purpura.
Autoimmune Thrombotic Thrombocytopenic Purpura: Two Rare Cases Associated with Juvenile Idiopathic Arthritis and Multiple Sclerosis.
Concentration and Subclass Distribution of Anti-ADAMTS13 IgG Autoantibodies in Different Stages of Acquired Idiopathic Thrombotic Thrombocytopenic Purpura.
Epidemiology and pathophysiology of adulthood-onset thrombotic microangiopathy with severe ADAMTS13 deficiency (thrombotic thrombocytopenic purpura): a cross-sectional analysis of the French national registry for thrombotic microangiopathy.
Quantification of von Willebrand factor and ADAMTS-13 after traumatic injury: a pilot study.
Treatment of Concurrent Thrombotic Thrombocytopenic Purpura and Graves' Disease: A Report on Two Cases.
TTP-like syndrome: novel concept and molecular pathogenesis of endotheliopathy-associated vascular microthrombotic disease.
Von Willebrand factor cleaving protease (ADAMTS-13) in 123 patients with connective tissue diseases (systemic lupus erythematosus and systemic sclerosis).
Bacteremia
Evaluation of a disintegrin-like and metalloprotease with thrombospondin type 1 repeat motifs 13 (ADAMTS13) activity enzyme-linked immunosorbent assay for measuring plasma ADAMTS13 activity in dogs.
Von Willebrand factor and ADAMTS13 impact on the outcome of Staphylococcus aureus sepsis.
Bacterial Infections
Assessment of ADAMTS-13 Level in Hospitalized Children with Serious Bacterial Infections as a Possible Prognostic Marker.
Imbalance of von Willebrand factor and its cleaving protease ADAMTS13 during systemic inflammation superimposed on advanced cirrhosis.
Oxidation of Met1606 in von Willebrand factor is a risk factor for thrombotic and septic complications in chronic renal failure.
Biliary Atresia
Relevance of ADAMTS13 to liver transplantation and surgery.
Border Disease
Increased expressions of ADAMTS-13, neuronal nitric oxide synthase, and neurofilament correlate with severity of neuropathology in Border disease virus-infected small ruminants.
Brain Diseases
An unbalance between von Willebrand factor and ADAMTS13 in acute liver failure: Implications for hemostasis and clinical outcome.
Reversible posterior leukoencephalopathy syndrome in a patient with thrombotic thrombocytopenic purpura.
Brain Infarction
[A study on the significance of plasma thrombospondin1 in thrombotic thrombocytopenic purpura and the relationship between thrombospondin1 and von Willebrand factor cleaving protease (ADAMTS13)]
Brain Injuries
ADAMTS13 gene deletion enhances plasma high-mobility group box1 elevation and neuroinflammation in brain ischemia-reperfusion injury.
ADAMTS13 reduces VWF-mediated acute inflammation following focal cerebral ischemia in mice.
Delayed treatment with ADAMTS13 ameliorates cerebral ischemic injury without hemorrhagic complication.
Effect of recombinant ADAMTS13 on microthrombosis and brain injury after experimental subarachnoid hemorrhage.
miR-525-5p inhibits ADAMTS13 and is correlated with Ischemia/reperfusion injury-induced neuronal cell death.
Recombinant ADAMTS 13 Attenuates Brain Injury After Intracerebral Hemorrhage.
Role of von Willebrand factor and ADAMTS-13 in early brain injury after experimental subarachnoid hemorrhage.
von Willebrand factor-cleaving protease ADAMTS13 reduces ischemic brain injury in experimental stroke.
Brain Ischemia
ADAMTS13 gene deletion enhances plasma high-mobility group box1 elevation and neuroinflammation in brain ischemia-reperfusion injury.
ADAMTS13 reduces VWF-mediated acute inflammation following focal cerebral ischemia in mice.
miR-525-5p inhibits ADAMTS13 and is correlated with Ischemia/reperfusion injury-induced neuronal cell death.
Reduced ADAMTS13 activity in delayed cerebral ischemia after aneurysmal subarachnoid hemorrhage.
Brain Neoplasms
ADAMTS-13 activity in patients with brain and prostate tumors is mildly reduced, but not correlated to stage of malignancy and metastasis.
Brucellosis
Microangiopathic Anemia of Acute Brucellosis - is it a True TTP?
Capillary Leak Syndrome
Microangiopathic Hemolytic Anemia Due to ADAMTS-13 Loss in Idiopathic Systemic Capillary Leak Syndrome.
Microangiopathic hemolytic anemia due to ADAMTS-13 loss in idiopathic systemic capillary leak syndrome: comment.
Microangiopathic hemolytic anemia due to ADAMTS-13 loss in idiopathic systemic capillary leak syndrome: reply.
Carcinogenesis
Novel protein and immune response markers of human serous tubal intraepithelial carcinoma of the ovary.
Carcinoma, Hepatocellular
A splice variant of ADAMTS13 is expressed in human hepatic stellate cells and cancerous tissues.
ADAMTS13 and von Willebrand factor are useful biomarkers for sorafenib treatment efficiency in patients with hepatocellular carcinoma.
Association between ADAMTS13 activity-VWF antigen imbalance and the therapeutic effect of HAIC in patients with hepatocellular carcinoma.
Prediction of hepatocellular carcinoma development by plasma ADAMTS13 in chronic hepatitis B and C.
VWF/ADAMTS13 ratio as a potential biomarker for early detection of hepatocellular carcinoma.
Carcinoma, Renal Cell
Biologically active ADAMTS13 is expressed in renal tubular epithelial cells.
Cardiomyopathies
Thrombocytopenic purpura and cardiomyopathy in pregnancy reversed by combined plasma exchange and infusion.
Cardiovascular Diseases
ADAMTS-13 and von Willebrand factor: a dynamic duo.
ADAMTS13 gene variants and function in women with preeclampsia: a population- based nested case- control study from the HUNT Study.
Conformational quiescence of ADAMTS13 prevents proteolytic promiscuity.
Hypercoagulability and cardiovascular disease in diabetic nephropathy.
Levels of von Willebrand factor antigen and von Willebrand factor cleaving protease (ADAMTS13) activity predict clinical events in chronic heart failure.
Lower levels of ADAMTS13 are associated with cardiovascular disease in young patients.
Shear Stress-Induced Activation of von Willebrand Factor and Cardiovascular Pathology.
SNPs in ADAMTS13.
The role of ADAMTS13 testing in the diagnosis and management of thrombotic microangiopathies and thrombosis.
Verification of the Role of ADAMTS13 in the Cardiovascular Disease Using Two-Sample Mendelian Randomization.
Von Willebrand factor and ADAMTS13 activity in relation to risk of dementia: a population-based study.
Von Willebrand factor and ADAMTS13 in arterial thrombosis: a systematic review and meta-analysis.
Von Willebrand Factor, ADAMTS13, and the Risk of Mortality: The Rotterdam Study.
Carotid Stenosis
Association between fibrinogen and fibrinogen ?' and atherosclerotic plaque morphology and composition in symptomatic carotid artery stenosis: Plaque-At-RISK study.
von Willebrand Factor Antigen, von Willebrand Factor Propeptide, and ADAMTS13 in Carotid Stenosis and Their Relationship with Cerebral Microemboli.
Castleman Disease
Acquired thrombotic thrombocytopenic purpura due to antibody-mediated ADAMTS13 deficiency precipitated by a localized Castleman's disease: A case report.
Cerebral Amyloid Angiopathy
ADAMTS13 maintains cerebrovascular integrity to ameliorate Alzheimer-like pathology.
Cerebral Hemorrhage
Delayed treatment with ADAMTS13 ameliorates cerebral ischemic injury without hemorrhagic complication.
Recombinant ADAMTS 13 Attenuates Brain Injury After Intracerebral Hemorrhage.
von Willebrand factor-cleaving protease ADAMTS13 reduces ischemic brain injury in experimental stroke.
Cerebral Infarction
ADAMTS13-mediated thrombolysis of t-PA resistant occlusions in ischemic stroke in mice.
Assessment of the Diagnostic Value of Plasma Levels, Activities, and Their Ratios of von Willebrand Factor and ADAMTS13 in Patients with Cerebral Infarction.
Genetic ablation of Adamts13 gene dramatically accelerates the formation of early atherosclerosis in a murine model.
Prognostic value of plasma von Willebrand factor-cleaving protease (ADAMTS13) antigen levels in patients with coronary artery disease.
Structure-function and regulation of ADAMTS-13 protease.
Von Willebrand factor and ADAMTS13 in arterial thrombosis: a systematic review and meta-analysis.
[Clinical application of a method for evaluating von Willebrand factor cleaving protease activity]
Cerebrovascular Disorders
Increased expression of ADAMTS13 mRNA correlates with ischemic cerebrovascular disease in systemic lupus erythematosus patients.
Inverse Regulation of Confluence-Dependent ADAMTS13 and von Willebrand Factor Expression in Human Endothelial Cells.
Reduced ADAMTS13 levels in patients with acute and chronic cerebrovascular disease.
Charcot-Marie-Tooth Disease
Thrombotic Thrombocytopenic Purpura with Severe ADAMTS-13 Deficiency in a Patient with Antiphospholipid Antibodies and Charcot-Marie-Tooth Disease.
Cholangitis
Platelet hyperaggregability is associated with decreased ADAMTS13 activity and enhanced endotoxemia in patients with acute cholangitis.
Cholestasis
Increased production of ADAMTS13 in hepatic stellate cells contributes to enhanced plasma ADAMTS13 activity in rat models of cholestasis and steatohepatitis.
Coinfection
Impact of HIV infection on the haemostatic response during sepsis and malaria.
Colitis
ADAMTS13 Deficiency Worsens Colitis and Exogenous ADAMTS13 Administration Decreases Colitis Severity in Mice.
Colitis, Ulcerative
P265. Decreased plasma ADAMTS13 antigen and ADAMTS13 activity as a risk factor for hypercoagulability in patients with ulcerative colitis.
Collagen Diseases
A second national questionnaire survey of TMA.
Quetiapine-induced thrombotic microangiopathy in a patient on maintenance dialysis.
[Von Willebrand factor-cleaving protease activity in patients of collagen disease with antiphospholipid antibodies]
Colonic Neoplasms
Deficiency of von Willebrand factor-cleaving protease activity in the plasma of malignant patients.
Colorectal Neoplasms
Platelet Count, ADAMTS13 Activity, von Willebrand Factor Level and Survival in Patients with Colorectal Cancer: 5-Year Follow-up Study.
Coma
Systemic infections mimicking thrombotic thrombocytopenic purpura.
Communicable Diseases
Biomarkers of endothelial activation/dysfunction in infectious diseases.
Plasma ADAMTS13, von Willebrand Factor (VWF) and VWF Propeptide Profiles in Patients with DIC and Related Diseases.
Confusion
Measurement of ADAMTS13.
The D173G mutation in ADAMTS-13 causes a severe form of congenital thrombotic thrombocytopenic purpura. A clinical, biochemical andin silico study.
Congenital Abnormalities
ADAMTS proteins in human disorders.
Pathogenesis of thrombotic thrombocytopenic purpura.
Connective Tissue Diseases
A 35-year-old woman with influenza A-associated thrombotic thrombocytopenic purpura.
Analyses of ADAMTS13 activity and its inhibitor in patients with thrombotic thrombocytopenic purpura secondary to connective tissue diseases: Observations in a single hospital.
Heterogeneous pathogenic processes of thrombotic microangiopathies in patients with connective tissue diseases.
Plasma ADAMTS13, von Willebrand Factor (VWF), and VWF Propeptide Profiles in Patients With Connective Tissue Diseases and Antiphospholipid Syndrome.
Von Willebrand factor cleaving protease (ADAMTS-13) in 123 patients with connective tissue diseases (systemic lupus erythematosus and systemic sclerosis).
Coronary Artery Disease
ADAMTS-13 activity in the presence of elevated von Willebrand factor levels as a novel mechanism of residual platelet reactivity in high risk coronary patients on antiplatelet treatment.
Association between ADAMTS13 polymorphisms and risk of cardiovascular events in chronic coronary disease.
Genetic Basis of Common Human Disease: Insight into the Role of Missense SNPs from Genome-Wide Association Studies.
Prognostic value of plasma von Willebrand factor-cleaving protease (ADAMTS13) antigen levels in patients with coronary artery disease.
Role of CD40 and ADAMTS13 in von Willebrand factor-mediated endothelial cell-platelet-monocyte interaction.
The role of ADAMTS13 testing in the diagnosis and management of thrombotic microangiopathies and thrombosis.
Coronary Disease
Are increased levels of von Willebrand factor in chronic coronary heart disease caused by decrease in von Willebrand factor cleaving protease activity? A study by an immunoassay with antibody against intact bond 842Tyr-843Met of the von Willebrand factor protein.
Association between ADAMTS13 polymorphisms and risk of cardiovascular events in chronic coronary disease.
Decreased levels of von Willebrand factor-cleaving protease in coronary heart disease and thrombotic thrombocytopenic purpura: study of a simplified method for assaying the enzyme activity based on ristocetin-induced platelet aggregation.
Low ADAMTS-13 activity and the risk of coronary heart disease - a prospective cohort study: the Rotterdam Study.
Verification of the Role of ADAMTS13 in the Cardiovascular Disease Using Two-Sample Mendelian Randomization.
Coronary Stenosis
Acquired intracoronary ADAMTS13 deficiency and VWF retention at sites of critical coronary stenosis in patients with STEMI.
Association of a disintegrin and metalloproteinase with a thrombospondin type 1 motif member 13 polymorphisms with severity of coronary stenosis in type 2 diabetes mellitus.
COVID-19
A Case of COVID-19 Induced Thrombotic Thrombocytopenic Purpura.
A mild deficiency of ADAMTS13 is associated with severity in COVID-19: comparison of the coagulation profile in critically and noncritically ill patients.
A relative ADAMTS13 deficiency supports the presence of a secondary microangiopathy in COVID 19.
ADAMTS 13 deficiency is associated with abnormal distribution of von Willebrand factor multimers in patients with COVID-19.
ADAMTS13 activity to von Willebrand factor antigen ratio predicts acute kidney injury in patients with COVID-19: Evidence of SARS-CoV-2 induced secondary thrombotic microangiopathy.
ADAMTS13 activity, von Willebrand factor, factor VIII and D-dimers in COVID-19 inpatients.
ADAMTS13 regulation of VWF multimer distribution in severe COVID-19.
COVID 19 infection associated with thrombotic thrombocytopenic purpura.
Distinctive Biomarker Features in The Endotheliopathy of COVID-19 and Septic Syndromes.
Early response to caplacizumab and rituximab after anaphylaxis to Octaplas plasma in a patient with thrombotic thrombocytopenic purpura.
Imbalance of von Willebrand factor and ADAMTS13 axis is rather a biomarker of strong inflammation and endothelial damage than a cause of thrombotic process in critically ill COVID-19 patients.
In vitro hypercoagulability and ongoing in vivo activation of coagulation and fibrinolysis in COVID-19 patients on anticoagulation.
Increased von Willebrand factor antigen and low ADAMTS13 activity are related to poor prognosis in covid-19 patients.
Increased VWF and Decreased ADAMTS-13 in COVID-19: Creating a Milieu for (Micro)Thrombosis.
Insights Into Immunothrombosis: The Interplay Among Neutrophil Extracellular Trap, von Willebrand Factor, and ADAMTS13.
Invited commentary to: ADAMTS13 deficiency is associated with abnormal distribution of von Willebrand factor multimers in patients with COVID-19 by Tiffany Pascreau et al. Letter to the Editors-in-Chief, Thrombosis Research.
Laboratory testing for ADAMTS13: Utility for TTP diagnosis/exclusion and beyond.
Life-threatening COVID-19 presenting as stroke with antiphospholipid antibodies and low ADAMTS-13 activity, and the role of therapeutic plasma exchange: A case series.
Low ADAMTS 13 plasma levels are predictors of mortality in COVID-19 patients.
Low ADAMTS13 Activity Correlates with Increased Mortality in COVID-19 Patients.
Prothrombotic changes in patients with COVID-19 are associated with disease severity and mortality.
Prothrombotic hemostasis disturbances in patients with severe COVID-19: Individual daily data.
Recombinant ADAMTS13 reduces abnormally up-regulated von Willebrand factor in plasma from patients with severe COVID-19.
Role of von Willebrand Factor and ADAMTS-13 in the Pathogenesis of Thrombi in SARS-CoV-2 Infection: Time to Rethink.
Role of von Willebrand Factor in COVID-19 Associated Coagulopathy.
Severe COVID-19 is associated with endothelial activation and abnormal glycosylation of von Willebrand factor in patients undergoing hemodialysis.
The ADAMTS13-von Willebrand factor axis in COVID-19 patients.
Von Willebrand factor and ADAMTS13 activity as clinical severity markers in patients with COVID-19.
Von Willebrand factor collagen-binding capacity predicts in-hospital mortality in COVID-19 patients: insight from VWF/ADAMTS13 ratio imbalance.
von Willebrand Factor Multimer Formation Contributes to Immunothrombosis in Coronavirus Disease 2019.
Dementia
Von Willebrand factor and ADAMTS13 activity in relation to risk of dementia: a population-based study.
Dementia, Vascular
Localization of blood proteins thrombospondin1 and ADAMTS13 to cerebral corpora amylacea.
Demyelinating Diseases
ADAMTS13 ameliorates inflammatory responses in experimental autoimmune encephalomyelitis.
Dengue
A novel association of acquired ADAMTS13 inhibitor and acute dengue virus infection.
Biomarkers of endothelial activation/dysfunction in infectious diseases.
Successful treatment of thrombotic microangiopathy associated with dengue infection: A case report and literature review.
Diabetes Complications
ADAMTS13 Predicts Renal and Cardiovascular Events in Type 2 Diabetic Patients and Response to Therapy.
Diabetes Mellitus
ADAMTS13 activity as a novel risk factor for incident type 2 diabetes mellitus: a population-based cohort study.
ADAMTS13 and von Willebrand factor concentrations in patients with diabetes mellitus.
Association of a disintegrin and metalloproteinase with a thrombospondin type 1 motif member 13 polymorphisms with severity of coronary stenosis in type 2 diabetes mellitus.
Liver up-regulation of ADAMTS13 gene expression and its correlation with renal markers in mice with type 1 diabetes mellitus and nephropathy.
Plasma Peptidylarginine Deiminase IV Promotes VWF-Platelet String Formation and Accelerates Thrombosis After Vessel Injury.
Von Willebrand Factor, ADAMTS13 and D-Dimer Are Correlated with Different Levels of Nephropathy in Type 1 Diabetes Mellitus.
Diabetes Mellitus, Type 1
Liver up-regulation of ADAMTS13 gene expression and its correlation with renal markers in mice with type 1 diabetes mellitus and nephropathy.
Von Willebrand Factor, ADAMTS13 and D-Dimer Are Correlated with Different Levels of Nephropathy in Type 1 Diabetes Mellitus.
Diabetes Mellitus, Type 2
ADAMTS13 activity as a novel risk factor for incident type 2 diabetes mellitus: a population-based cohort study.
Diabetic Angiopathies
ADAMTS13--more than just TMA and TTP.
Diabetic Nephropathies
ADAMTS13 Retards Progression of Diabetic Nephropathy by Inhibiting Intrarenal Thrombosis in Mice.
Association between reduced ADAMTS13 and diabetic nephropathy.
Diabetic ketoacidosis presenting with atypical hemolytic uremic syndrome associated with a variant of complement factor B in an adult: a case report.
Hypercoagulability and cardiovascular disease in diabetic nephropathy.
Significance of plasma von Willebrand factor level and von Willebrand factor-cleaving protease activity in patients with chronic renal diseases.
Diabetic Retinopathy
Association between Von Willebrand factor, disintegrin and metalloproteinase with thrombospondin type 1 motif member 13, d-Dimer and cystatin C levels with retinopathy in type 1 diabetes mellitus.
Disseminated Intravascular Coagulation
ADAMTS-13 activity can predict the outcome of disseminated intravascular coagulation in hematologic malignancies treated with recombinant human soluble thrombomodulin.
Correlation between plasma activity of ADAMTS-13 and coagulopathy, and prognosis in disseminated intravascular coagulation.
Decreased a disintegrin-like and metalloprotease with thrombospondin (ADAMTS)-13 is associated with a poor prognosis in sepsis-induced organ failure*
Decreased ADAMTS 13 Activity is Associated With Disease Severity and Outcome in Pediatric Severe Sepsis.
Decreased ADAMTS-13 (A disintegrin-like and metalloprotease with thrombospondin type 1 repeats) is associated with a poor prognosis in sepsis-induced organ failure.
Low ADAMTS-13 activity during hemorrhagic events with disseminated intravascular coagulation.
Measurement of ADAMTS13 activity and inhibitors.
Plasma ADAMTS13, von Willebrand Factor (VWF) and VWF Propeptide Profiles in Patients with DIC and Related Diseases.
Plasmodium falciparum Malaria Complicated by Symmetrical Peripheral Gangrene, Bowel Ischemia, Repeated Candidemia, and Bacteraemia.
Renal thrombotic microangiopathy in a patient with septic disseminated intravascular coagulation.
Sepsis-Induced Disseminated Intravascular Coagulation With Features of Thrombotic Thrombocytopenic Purpura: a Fatal Fulminant Syndrome.
Severe secondary deficiency of von Willebrand factor-cleaving protease (ADAMTS13) in patients with sepsis-induced disseminated intravascular coagulation: its correlation with development of renal failure.
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.
Von Willebrand factor and ADAMTS13 impact on the outcome of Staphylococcus aureus sepsis.
VWF excess and ADAMTS13 deficiency: a unifying pathomechanism linking inflammation to thrombosis in DIC, malaria, and TTP.
[Changes of ADAMTS13 Activity and vWF Antigen Level in Patients with Acute Myelogenous Leukemia and Their Significance].
Drug-Related Side Effects and Adverse Reactions
[Thrombotic microangiopathy after kidney transplantation]
Encephalitis
ADAMTS13 gene deletion enhances plasma high-mobility group box1 elevation and neuroinflammation in brain ischemia-reperfusion injury.
Increased expressions of ADAMTS-13 and apoptosis contribute to neuropathology during Toxoplasma gondii encephalitis in mice.
Role of ADAMTS-13 and nNOS expression in neuropathogenesis of listeric encephalitis of small ruminants.
Encephalomyelitis
ADAMTS13 ameliorates inflammatory responses in experimental autoimmune encephalomyelitis.
Encephalomyelitis, Autoimmune, Experimental
ADAMTS13 ameliorates inflammatory responses in experimental autoimmune encephalomyelitis.
endopeptidase la deficiency
Assays of von Willebrand factor-cleaving protease: a test for diagnosis of familial and acquired thrombotic thrombocytopenic purpura.
Autosomal recessive inheritance of von Willebrand factor-cleaving protease deficiency.
Cancer-related thrombotic microangiopathy secondary to Von Willebrand factor-cleaving protease deficiency.
Mutation analysis and clinical implications of von Willebrand factor-cleaving protease deficiency.
Prospective study on the behaviour of the metalloprotease ADAMTS13 and of von Willebrand factor after bone marrow transplantation.
Thrombotic thrombocytopenic purpura--the role of ADAMTS13 assay in clinical practice.
Two generations with familial thrombotic thrombocytopenic purpura.
von Willebrand factor-cleaving protease in thrombotic thrombocytopenic purpura and the hemolytic-uremic syndrome.
[Hemolytic and uremic syndrome in the child]
Endotoxemia
Decreased activity of plasma ADAMTS13 are related to enhanced cytokinemia and endotoxemia in patients with acute liver failure.
Platelet hyperaggregability is associated with decreased ADAMTS13 activity and enhanced endotoxemia in patients with acute cholangitis.
Potential Role of Enhanced Cytokinemia and Plasma Inhibitor on the Decreased Activity of Plasma ADAMTS13 in Patients With Alcoholic Hepatitis: Relationship to Endotoxemia.
The combined roles of ADAMTS13 and VWF in murine models of TTP, endotoxemia, and thrombosis.
Erythema
Significance of plasma von Willebrand factor level and von Willebrand factor-cleaving protease activity in patients with chronic renal diseases.
Escherichia coli Infections
Clinical Characteristics and Outcome of Canadian Patients Diagnosed With Atypical Hemolytic Uremic Syndrome.
Exanthema
Severe ADAMTS13 deficiency in adult idiopathic thrombotic microangiopathies defines a subset of patients characterized by various autoimmune manifestations, lower platelet count, and mild renal involvement.
Familial Primary Pulmonary Hypertension
The ADAMTS13-VWF axis is dysregulated in chronic thromboembolic pulmonary hypertension.
Fatty Liver
ADAMTS13 deficiency promotes microthrombosis in a murine model of diet-induced liver steatosis.
Increased production of ADAMTS13 in hepatic stellate cells contributes to enhanced plasma ADAMTS13 activity in rat models of cholestasis and steatohepatitis.
Role of ADAMTS13 in diet-induced liver steatosis.
Genetic Diseases, Inborn
Multiple in silico tools predict phenotypic manifestations in congenital thrombotic thrombocytopenic purpura.
The ADAMTS(L) family and human genetic disorders.
Glomerulonephritis
Disseminated cerebral aspergillosis complicated by thrombotic microangiopathy.
Severe ADAMTS13 deficiency in adult idiopathic thrombotic microangiopathies defines a subset of patients characterized by various autoimmune manifestations, lower platelet count, and mild renal involvement.
Significance of plasma von Willebrand factor level and von Willebrand factor-cleaving protease activity in patients with chronic renal diseases.
Glomerulonephritis, IGA
Significance of plasma von Willebrand factor level and von Willebrand factor-cleaving protease activity in patients with chronic renal diseases.
Glomerulonephritis, Membranous
Significance of plasma von Willebrand factor level and von Willebrand factor-cleaving protease activity in patients with chronic renal diseases.
Glycogen Storage Disease Type VI
Dissociation between the level of von Willebrand factor-cleaving protease activity and disease in a patient with congenital thrombotic thrombocytopenic purpura.
Graft vs Host Disease
[Influence of HSCT Preconditioning on ADAMTS-13 Activity and vWF level and Its Clinical Significance].
Graves Disease
Thrombotic thrombocytopenic purpura precipitated by thyrotoxicosis.
Treatment of Concurrent Thrombotic Thrombocytopenic Purpura and Graves' Disease: A Report on Two Cases.
Hantavirus Infections
Platelet ligands and ADAMTS13 during Puumala hantavirus infection and associated thrombocytopenia.
Heart Defects, Congenital
Compounding variants rescue the effect of a deleterious ADAMTS13 mutation in a child with severe congenital heart disease.
Decreased plasma ADAMTS-13 activity as a predictor of postoperative bleeding in cyanotic congenital heart disease.
In silico features of ADAMTS13 contributing to plasmatic ADAMTS13 levels in neonates with congenital heart disease.
Plasmatic ADAMTS-13 metalloprotease and von Willebrand factor in children with cyanotic congenital heart disease.
Heart Diseases
Diagnosis and Management of Acquired von Willebrand Disease in Heart Disease: A Review of the Literature.
Successful Perioperative Management of Orthotopic Cardiac Transplantation in a Pediatric Patient With Concurrent Congenital von Willebrand Disease and Acquired von Willebrand Syndrome Using Recombinant von Willebrand Factor.
Heart Failure
Levels of von Willebrand factor antigen and von Willebrand factor cleaving protease (ADAMTS13) activity predict clinical events in chronic heart failure.
Plasma Proteomics of COVID-19 Associated Cardiovascular Complications: Implications for Pathophysiology and Therapeutics.
Update on ADAMTS13 and VWF in cardiovascular and hematological disorders.
Verification of the Role of ADAMTS13 in the Cardiovascular Disease Using Two-Sample Mendelian Randomization.
HELLP Syndrome
Acute activation of the endothelium results in increased levels of active von Willebrand factor in hemolysis, elevated liver enzymes and low platelets (HELLP) syndrome.
ADAMTS13 deficiency in severe postpartum HELLP syndrome.
Hemolysis, Elevated Liver Enzymes, and Low Platelet Count Syndrome With Severe Thrombocytopenia and Severe ADAMTS13 Activity Deficiency.
Increased plasma von Willebrand factor antigen levels but normal von Willebrand factor cleaving protease (ADAMTS13) activity in preeclampsia.
Mild to moderate reduction of a von Willebrand factor cleaving protease (ADAMTS-13) in pregnant women with HELLP microangiopathic syndrome.
Severe reduction of free-form ADAMTS13, unbound to von Willebrand factor, in plasma of patients with HELLP syndrome.
Hematologic Diseases
ADAMTS13 turns 3.
Hematologic Neoplasms
ADAMTS-13 activity can predict the outcome of disseminated intravascular coagulation in hematologic malignancies treated with recombinant human soluble thrombomodulin.
Decreased ADAMTS-13 level is related to inflammation factors and risk stratification of acute lymphoblastic leukemia patients.
Recipient ADAMTS13 Single-Nucleotide Polymorphism Predicts Relapse after Unrelated Bone Marrow Transplantation for Hematologic Malignancy.
Reduced ADAMTS-13 level negatively correlates with inflammation factors in plasma of acute myeloid leukemia patients.
[Changes of ADAMTS13 Activity and TSP1 Level in Patients with Hematologic Malignancies].
Hemoglobinopathies
Refractory acquired thrombotic thrombocytopenic purpura in a patient with sickle cell trait successfully treated with caplacizumab.
Hemoglobinuria
Thrombotic microangiopathies and the linkage between von Willebrand factor and the alternative complement pathway.
Hemoglobinuria, Paroxysmal
Thrombotic microangiopathies and the linkage between von Willebrand factor and the alternative complement pathway.
Hemolytic-Uremic Syndrome
ADAMTS-13 level in children with severe diarrhea-associated hemolytic uremic syndrome: Unmasking new association.
ADAMTS13 and von Willebrand factor in thrombotic thrombocytopenic purpura.
ADAMTS13 Gene mutations in children with hemolytic uremic syndrome.
ADAMTS13, thrombotic thrombocytopenic purpura, and hemolytic uremic syndrome.
An open conformation of ADAMTS-13 is a hallmark of acute acquired thrombotic thrombocytopenic purpura.
Assays of von Willebrand factor-cleaving protease: a test for diagnosis of familial and acquired thrombotic thrombocytopenic purpura.
Atypical reduction of plasma ADAMTS13 activity by a non-IgG-type inhibitor in a patient with hemolytic uremic syndrome caused by Shiga toxin-producing Escherichia coli.
Clinical experience in treatment of thrombotic thrombocytopenic purpura--hemolytic uremic syndrome with 28 patients.
Deficiency of complement factor H-related proteins and autoantibody-positive hemolytic uremic syndrome in an infant with combined partial deficiencies and autoantibodies to complement factor H and ADAMTS13.
Diagnostic relevance of ADAMTS13 activity: evaluation of 28 patients with thrombotic thrombocytopenic purpura - hemolytic uremic syndrome clinical diagnosis.
Erratum: Deficiency of complement factor H-related proteins and autoantibody-positive hemolytic uremic syndrome in an infant with combined partial deficiencies and autoantibodies to complement factor H and ADAMTS13.
Hemolytic uremic syndrome associated with Escherichia coli O157:H7 infection in older adults: a case report and review of the literature.
Immature platelet fraction can help adjust therapy in refractory thrombotic microangiopathic hemolytic anemia cases.
Intravenous gamma globulin for thrombotic microangiopathy of unknown etiology.
Is factor V Leiden a risk factor for thrombotic microangiopathies without severe ADAMTS 13 deficiency?
Natural history of thrombotic thrombocytopenic purpura and hemolytic uremic syndrome.
Pathogenesis of thrombotic thrombocytopenic purpura: ADAMTS13 deficiency and beyond.
Severe deficiency of the specific von Willebrand factor-cleaving protease (ADAMTS 13) activity in a subgroup of children with atypical hemolytic uremic syndrome.
Severe transient ADAMTS13 deficiency in pneumococcal-associated hemolytic uremic syndrome.
Shear stress and von Willebrand factor in health and disease.
Shiga Toxin as a Potential Trigger of CFHR1 Deletion-Associated Thrombotic Microangiopathy.
Successful treatment of thrombotic microangiopathy associated with dengue infection: A case report and literature review.
Successful treatment with rituximab for acute refractory thrombotic thrombocytopenic purpura related to acquired ADAMTS13 deficiency: A pediatric report and literature review.
Thrombotic microangiopathy (TTP and HUS): advances in differentiation and diagnosis.
Thrombotic thrombocytopenic purpura and the hemolytic uremic syndrome.
Thrombotic thrombocytopenic purpura--a syndrome caused by multiple pathogenetic mechanisms.
Thrombotic thrombocytopenic purpura: from platelet aggregates to plasma.
Total deficiency of specific von Willebrand factor-cleaving protease and recovery following plasma therapy in one patient with hemolytic-uremic syndrome.
von Willebrand factor and von Willebrand factor-cleaving metalloprotease activity in Escherichia coli O157:H7-associated hemolytic uremic syndrome.
von Willebrand factor cleaving protease (ADAMTS13) is deficient in recurrent and familial thrombotic thrombocytopenic purpura and hemolytic uremic syndrome.
Von Willebrand factor-cleaving protease (ADAMTS13) in thrombocytopenic disorders: a severely deficient activity is specific for thrombotic thrombocytopenic purpura.
von Willebrand factor-cleaving protease in thrombotic thrombocytopenic purpura and hemolytic-uremic syndrome.
von Willebrand factor-cleaving protease in thrombotic thrombocytopenic purpura and the hemolytic-uremic syndrome.
[Hemolytic uremic syndrome and thrombotic thrombocytopenic purpura. Current perspectives on EHEC, complement mutations and ADAMTS13]
Hemophilia A
Factor VIII concentrate infusion in patients with haemophilia results in decreased von Willebrand factor and ADAMTS-13 activity.
Hemophilia B
Development of novel treatment options for patients with haemophilia.
Hemorrhagic Stroke
von Willebrand factor-cleaving protease ADAMTS13 reduces ischemic brain injury in experimental stroke.
Hepatic Encephalopathy
Determination of ADAMTS13 and Its Clinical Significance for ADAMTS13 Supplementation Therapy to Improve the Survival of Patients with Decompensated Liver Cirrhosis.
Hepatic Veno-Occlusive Disease
Impaired activity of plasma von Willebrand factor-cleaving protease may predict the occurrence of hepatic veno-occlusive disease after stem cell transplantation.
Hepatitis
Decreased activity of plasma ADAMTS13 are related to enhanced cytokinemia and endotoxemia in patients with acute liver failure.
Hepatitis B, Chronic
Increasing plasma ADAMTS13 activity is associated with HBeAg seroconversion in chronic hepatitis B patients during 5 years of entecavir treatment.
Plasma Level of ADAMTS13 or IL-12 as an Indicator of HBeAg Seroconversion in Chronic Hepatitis B Patients Undergoing m-ETV Treatment.
Prediction of hepatocellular carcinoma development by plasma ADAMTS13 in chronic hepatitis B and C.
Hepatitis C
Active Hepatitis C Leading to Refractory Thrombotic Thrombocytopenic Purpura - A Dubious Association and The Challenges Faced in Management.
ADAMTS13 Levels in Young Patients With ?-Thalassemia Major: Relation to Hepatitis C Virus Infection, Liver Cirrhosis, and Iron Overload.
Development of ADAMTS13 inhibitor in a patient with hepatitis C virus-related liver cirrhosis causes thrombotic thrombocytopenic purpura.
Opana ER (Oxymorphone)-Induced Thrombotic Microangiopathy: An Atypical Presentation in a Patient With Hepatitis C.
Hepatitis C, Chronic
Interferon induced thrombotic microangiopathy (TMA): Analysis and concise review.
Thrombotic thrombocytopenic purpura associated with pegylated-interferon alpha-2a by an ADAMTS13 inhibitor in a patient with chronic hepatitis C.
Hepatitis, Alcoholic
Decreased activity of plasma ADAMTS13 may contribute to the development of liver disturbance and multiorgan failure in patients with alcoholic hepatitis.
Increased von Willebrand Factor Over Decreased ADAMTS13 Activity May Contribute to the Development of Liver Disturbance and Multiorgan Failure in Patients With Alcoholic Hepatitis.
Potential role of ADAMTS13 in the progression of alcoholic hepatitis.
Potential Role of Enhanced Cytokinemia and Plasma Inhibitor on the Decreased Activity of Plasma ADAMTS13 in Patients With Alcoholic Hepatitis: Relationship to Endotoxemia.
Hepatitis, Autoimmune
Altered von Willebrand Factor and ADAMTS13 Levels in Children With Cirrhosis and Extrahepatic Portal Hypertension.
The significance of ADAMTS13 in a patient with thrombotic thrombocytopenic purpura complicated autoimmune hepatitis.
Hepatorenal Syndrome
Determination of ADAMTS13 and Its Clinical Significance for ADAMTS13 Supplementation Therapy to Improve the Survival of Patients with Decompensated Liver Cirrhosis.
HIV Infections
Von Willebrand Factor Adhesive Activity and ADAMTS13 Protease Activity in HIV-1-Infected Men.
Hyperbilirubinemia, Neonatal
Molecular characterization of ADAMTS13 gene mutations in Japanese patients with Upshaw-Schulman syndrome.
Hypercholesterolemia
Gender-dependent up-regulation of ADAMTS-13 in mice with obesity and hypercholesterolemia.
Hyperferritinemia
Secretion of von Willebrand Factor and Suppression of ADAMTS-13 Activity by Markedly High Concentration of Ferritin.
Hyperlipidemias
Change in plasma a disintegrin and metalloprotease with thrombospondin type-1 repeats-13 and von Willebrand factor levels in venous thromboembolic patients.
Hypersensitivity
Idiopathic Relapsing Thrombotic Thrombocytopenic Purpura with Persistent ADAMTS13 Inhibitor Activity Treated Sequentially with Plasmapheresis, Rituximab, Cyclophosphamide and Splenectomy.
Hypertension
Absolute immature platelet count helps differentiate thrombotic thrombocytopenic purpura from hypertension-induced thrombotic microangiopathy.
Association between thrombotic microangiopathy and reduced ADAMTS13 activity in malignant hypertension.
Circulating miR-3656 induces human umbilical vein endothelial cell injury by targeting eNOS and ADAMTS13: a novel biomarker for hypertension.
Correlation analysis between ADAMTS-13 gene polymorphism and hypertension-induced atrial fibrillation.
Differentiating malignant hypertension-induced thrombotic microangiopathy from thrombotic thrombocytopenic purpura.
Is therapeutic plasma exchange indicated for patients with gemcitabine-induced hemolytic uremic syndrome?
Porto-pulmonary hypertension exacerbated by platelet transfusion in a patient with ADAMTS13 deficiency.
The ADAMTS13-VWF axis is dysregulated in chronic thromboembolic pulmonary hypertension.
The kidney in thrombotic thrombocytopenic purpura.
Thrombotic microangiopathy in malignant hypertension and hemolytic uremic syndrome (HUS)/ thrombotic thrombocytopenic purpura (TTP): can we differentiate one from the other?
Update on ADAMTS13 and VWF in cardiovascular and hematological disorders.
Hypertension, Malignant
Association between thrombotic microangiopathy and reduced ADAMTS13 activity in malignant hypertension.
Catastrophic APS in the context of other thrombotic microangiopathies.
Thrombotic microangiopathy due to malignant hypertension complicated with late-onset bleeding after renal biopsy.
Hypertension, Portal
ADAMTS13 deficiency, despite well-compensated liver functions in patients with noncirrhotic portal hypertension.
ADAMTS13 missense variants associated with defective activity and secretion of ADAMTS13 in a patient with non-cirrhotic portal hypertension.
Altered von Willebrand Factor and ADAMTS13 Levels in Children With Cirrhosis and Extrahepatic Portal Hypertension.
Idiopathic Noncirrhotic Intrahepatic Portal Hypertension is Associated with Sustained ADAMTS13 Deficiency.
Imbalance of von Willebrand factor and its cleaving protease ADAMTS13 during systemic inflammation superimposed on advanced cirrhosis.
Orthotopic liver transplantation (OLTx) in non-cirrhotic portal hypertension secondary to ADAMTS13 deficiency.
Plasma ADAMTS-13 protein is not associated with portal hypertension or hemodynamic changes in patients with cirrhosis.
Hypertension, Pregnancy-Induced
Changes of plasma Von Willebrand factor and Von Willebrand factor-cleaving protease levels in pregnancy-induced hypertension.
Hypertension, Pulmonary
Porto-pulmonary hypertension exacerbated by platelet transfusion in a patient with ADAMTS13 deficiency.
The ADAMTS13-VWF axis is dysregulated in chronic thromboembolic pulmonary hypertension.
Hypertensive Retinopathy
[Clinical reasoning and decision making in practice. A 39-year-old woman with somnolence, hypertension and haemolysis]
Immune System Diseases
Treatment of refractory thrombotic thrombocytopenic purpura using multimodality therapy including splenectomy and cyclosporine.
Immunoglobulin G4-Related Disease
Thrombotic thrombocytopenic purpura in IgG4-related disease with severe deficiency of ADAMTS-13 activity and IgG4 autoantibody against ADAMTS-13.
Immunoglobulin Light-chain Amyloidosis
Clinical and prognostic significance of serum levels of von Willebrand factor and ADAMTS-13 antigens in AL amyloidosis.
Infarction, Middle Cerebral Artery
ADAMTS13 gene deletion aggravates ischemic brain damage: a possible neuroprotective role of ADAMTS13 by ameliorating postischemic hypoperfusion.
ADAMTS13 gene deletion enhances plasma high-mobility group box1 elevation and neuroinflammation in brain ischemia-reperfusion injury.
Delayed treatment with ADAMTS13 ameliorates cerebral ischemic injury without hemorrhagic complication.
Infections
A 35-year-old woman with influenza A-associated thrombotic thrombocytopenic purpura.
A novel association of acquired ADAMTS13 inhibitor and acute dengue virus infection.
A novel quantitative hemolytic assay coupled with restriction fragment length polymorphisms analysis enabled early diagnosis of atypical hemolytic uremic syndrome and identified unique predisposing mutations in Japan.
A novel role for von Willebrand factor in the pathogenesis of experimental cerebral malaria.
Acquired thrombotic thrombocytopenic purpura due to antibody-mediated ADAMTS13 deficiency precipitated by a localized Castleman's disease: A case report.
ADAMTS-13 activity reduction in plasma of acute myeloid leukemia predicts poor prognosis after bone marrow transplantation.
ADAMTS13 and von Willebrand factor in thrombotic thrombocytopenic purpura.
ADAMTS13 Levels in Young Patients With ?-Thalassemia Major: Relation to Hepatitis C Virus Infection, Liver Cirrhosis, and Iron Overload.
ADAMTS13 regulation of VWF multimer distribution in severe COVID-19.
Atypical hemolytic uremic syndrome: from diagnosis to treatment.
Biomarkers of endothelial activation/dysfunction in infectious diseases.
Catastrophic APS in the context of other thrombotic microangiopathies.
Clinical Characteristics and Outcome of Canadian Patients Diagnosed With Atypical Hemolytic Uremic Syndrome.
Coagulopathy in malaria.
COVID 19 infection associated with thrombotic thrombocytopenic purpura.
Decreased ADAMTS-13 level is related to inflammation factors and risk stratification of acute lymphoblastic leukemia patients.
Degradation of Circulating von Willebrand Factor and Its Regulator ADAMTS13 Implicates Secreted Bacillus anthracis Metalloproteases in Anthrax Consumptive Coagulopathy.
Idiopathic TTP in the Middle East: Epidemiology and clinical outcomes in infection associated episodes.
Low Plasma ADAMTS13 Activity Is Associated with Coagulopathy, Endothelial Cell Damage and Mortality after Severe Paediatric Trauma.
Neonatal Atypical Hemolytic Uremic Syndrome in the Eculizumab Era.
Platelet ligands and ADAMTS13 during Puumala hantavirus infection and associated thrombocytopenia.
Reduced ADAMTS-13 level negatively correlates with inflammation factors in plasma of acute myeloid leukemia patients.
Renal thrombotic microangiopathy in a patient with septic disseminated intravascular coagulation.
Ribosomal and Immune Transcripts Associate with Relapse in Acquired ADAMTS13-Deficient Thrombotic Thrombocytopenic Purpura.
Role of von Willebrand Factor in COVID-19 Associated Coagulopathy.
Severe Plasmodium falciparum malaria is associated with circulating ultra-large von Willebrand multimers and ADAMTS13 inhibition.
Shiga Toxin as a Potential Trigger of CFHR1 Deletion-Associated Thrombotic Microangiopathy.
Short Course of Eculizumab May Be Effective in Dialysis-Dependent Transplantation-Associated Thrombotic Microangiopathy After Hematopoietic Stem Cell Transplantation: A Case Report.
Successful treatment of thrombotic microangiopathy associated with dengue infection: A case report and literature review.
Systemic infections mimicking thrombotic thrombocytopenic purpura.
Ten patient stories illustrating the extraordinarily diverse clinical features of patients with thrombotic thrombocytopenic purpura and severe ADAMTS13 deficiency.
Von Willebrand Factor Adhesive Activity and ADAMTS13 Protease Activity in HIV-1-Infected Men.
Von Willebrand factor and ADAMTS13 impact on the outcome of Staphylococcus aureus sepsis.
von Willebrand factor and von Willebrand factor-cleaving metalloprotease activity in Escherichia coli O157:H7-associated hemolytic uremic syndrome.
von Willebrand factor increases in experimental cerebral malaria but is not essential for late-stage pathogenesis in mice.
von Willebrand factor-cleaving protease in childhood diarrhoea-associated haemolytic uraemic syndrome.
VWF excess and ADAMTS13 deficiency: a unifying pathomechanism linking inflammation to thrombosis in DIC, malaria, and TTP.
[Changes of ADAMTS13 Activity and TSP1 Level in Patients with Hematologic Malignancies].
[Changes of ADAMTS13 Activity and vWF Antigen Level in Patients with Acute Myelogenous Leukemia and Their Significance].
Inflammatory Bowel Diseases
ADAMTS13 activity to antigen ratio in physiological and pathological conditions associated with an increased risk of thrombosis.
ADAMTS13 in health and disease.
Levels of TAFI, TFPI and ADAMTS-13 in inflammatory bowel disease.
Influenza, Human
A 35-year-old woman with influenza A-associated thrombotic thrombocytopenic purpura.
Acquired Thrombotic Thrombocytopenic Purpura Without Anti-ADAMTS13 Antibody Caused by Influenza A (H1N1) Virus Successfully Treated by Plasma Exchange: A Case Report.
H1N1 Influenza (Swine Flu)-Associated Thrombotic Microangiopathy with a Markedly High Plasma Ratio of von Willebrand Factor to ADAMTS13.
Invasive Pulmonary Aspergillosis
ADAMTS-13 regulates neutrophil recruitment in a mouse model of invasive pulmonary aspergillosis.
Author Correction: ADAMTS-13 regulates neutrophil recruitment in a mouse model of invasive pulmonary aspergillosis.
Iron Overload
ADAMTS13 Levels in Young Patients With ?-Thalassemia Major: Relation to Hepatitis C Virus Infection, Liver Cirrhosis, and Iron Overload.
Ischemic Attack, Transient
Association between fibrinogen and fibrinogen ?' and atherosclerotic plaque morphology and composition in symptomatic carotid artery stenosis: Plaque-At-RISK study.
Increased expression of ADAMTS13 mRNA correlates with ischemic cerebrovascular disease in systemic lupus erythematosus patients.
von Willebrand factor/ADAMTS13 ratio at presentation of acute ischemic brain injury is predictive of outcome.
Ischemic Stroke
ADAMTS-13 Activity Predicts Outcome in Acute Ischaemic Stroke Patients Undergoing Endovascular Treatment.
ADAMTS13 activity is associated with early neurological improvement in acute ischemic stroke patients treated with intravenous thrombolysis.
ADAMTS13 reduces VWF-mediated acute inflammation following focal cerebral ischemia in mice.
ADAMTS13-mediated thrombolysis of t-PA resistant occlusions in ischemic stroke in mice.
ADAMTS13: An Emerging Target in Stroke Therapy.
Association between fibrinogen and fibrinogen ?' and atherosclerotic plaque morphology and composition in symptomatic carotid artery stenosis: Plaque-At-RISK study.
Association between genetic variation at the ADAMTS13 locus and ischemic stroke.
Author response: Usefulness of ADAMTS13 to predict response to recanalization therapies in acute ischemic stroke.
Characterization and treatment of congenital thrombotic thrombocytopenic purpura.
Delayed treatment with ADAMTS13 ameliorates cerebral ischemic injury without hemorrhagic complication.
Editors' note: Usefulness of ADAMTS13 to predict response to recanalization therapies in acute ischemic stroke.
Enhanced activity of an ADAMTS-13 variant (R568K/F592Y/R660K/Y661F/Y665F) against platelet agglutination in vitro and in a murine model of acute ischemic stroke.
High von Willebrand factor levels increase the risk of first ischemic stroke: influence of ADAMTS13, inflammation, and genetic variability.
High VWF, low ADAMTS13 puts women at risk.
High VWF, low ADAMTS13, and oral contraceptives increase the risk of ischemic stroke and myocardial infarction in young women.
Immune-Thrombotic Thrombocytopenic Purpura is a Rare Cause of Ischemic Stroke in Young Adults: Case Reports and Literature Review.
Insights Into Immunothrombosis: The Interplay Among Neutrophil Extracellular Trap, von Willebrand Factor, and ADAMTS13.
Low ADAMTS-13 activity and the risk of coronary heart disease - a prospective cohort study: the Rotterdam Study.
Low ADAMTS13 activity is associated with an increased risk of ischemic stroke.
Plasma ADAMTS-13 levels and the risk of myocardial infarction: an individual patient data meta-analysis.
Reader response: Usefulness of ADAMTS13 to predict response to recanalization therapies in acute ischemic stroke.
Reduced ADAMTS13 levels in patients with acute and chronic cerebrovascular disease.
Relationship between ADAMTS13 activity, von Willebrand factor antigen levels and platelet function in the early and late phases after TIA or ischaemic stroke.
Role of reduced ADAMTS13 in arterial ischemic stroke: a pediatric cohort study.
The role of ADAMTS13 testing in the diagnosis and management of thrombotic microangiopathies and thrombosis.
The Role of von Willebrand Factor, ADAMTS13, and Cerebral Artery Thrombus Composition in Patient Outcome Following Mechanical Thrombectomy for Acute Ischemic Stroke.
The Value of ADAMTS13 in Predicting Clinical Outcomes in Patients With Acute Ischemic Stroke Receiving Thrombolysis.
Update on ADAMTS13 and VWF in cardiovascular and hematological disorders.
Usefulness of ADAMTS13 to predict response to recanalization therapies in acute ischemic stroke.
Von Willebrand factor and ADAMTS13 activity in relation to risk of dementia: a population-based study.
von Willebrand factor/ADAMTS13 ratio at presentation of acute ischemic brain injury is predictive of outcome.
[Determination of ADAMTS13 antigen and activity levels in patients with acute myocardial infarction and acute ischemic stroke]
[Genetic polymorphism of von Willebrand factor (VWF)-cleaving protease, ADAMTS13]
[Study of C1423T polymorphism of the von Willebrand factor-cleaving protease gene in Chinese Han population]
Kidney Diseases
ADAMTS13 and Von Willebrand factor in patients undergoing hemodialysis.
Kidney Failure, Chronic
Predictive value of ADAMTS-13 on concealed chronic renal failure in COPD patients.
Severe ADAMTS13 deficiency in adult idiopathic thrombotic microangiopathies defines a subset of patients characterized by various autoimmune manifestations, lower platelet count, and mild renal involvement.
Larva Migrans, Visceral
Atypical presentation of hepatic visceral larva migrans mimicking cancer and associated with ADAMTS13 deficiency-mediated thrombotic microangiopathy: A first report from Reunion Island.
Leukemia
Decreased von Willebrand factor protease activity associated with thrombocytopenic disorders.
Interferon induced thrombotic microangiopathy (TMA): Analysis and concise review.
Thrombogenesis and thrombotic disorders based on 'two-path unifying theory of hemostasis': philosophical, physiological, and phenotypical interpretation.
[ADAMTS13 Level in Prothrombotic Status and Its Related Factor Analysis].
Leukemia, Hairy Cell
Interferon induced thrombotic microangiopathy (TMA): Analysis and concise review.
Leukemia, Myelogenous, Chronic, BCR-ABL Positive
Interferon induced thrombotic microangiopathy (TMA): Analysis and concise review.
Leukemia, Myeloid, Acute
ADAMTS-13 activity reduction in plasma of acute myeloid leukemia predicts poor prognosis after bone marrow transplantation.
Reduced ADAMTS-13 level negatively correlates with inflammation factors in plasma of acute myeloid leukemia patients.
[Changes of ADAMTS13 Activity and vWF Antigen Level in Patients with Acute Myelogenous Leukemia and Their Significance].
Leukemia, Promyelocytic, Acute
Thrombogenesis and thrombotic disorders based on 'two-path unifying theory of hemostasis': philosophical, physiological, and phenotypical interpretation.
[ADAMTS13 Level in Prothrombotic Status and Its Related Factor Analysis].
Liver Cirrhosis
ADAMTS13 activity may predict the cumulative survival of patients with liver cirrhosis in comparison with the Child-Turcotte-Pugh score and the Model for End-Stage Liver Disease score.
ADAMTS13 activity to antigen ratio in physiological and pathological conditions associated with an increased risk of thrombosis.
ADAMTS13 in health and disease.
ADAMTS13 Levels in Young Patients With ?-Thalassemia Major: Relation to Hepatitis C Virus Infection, Liver Cirrhosis, and Iron Overload.
Clinical insights from observations on ADAMTS13 deficiency in liver cirrhosis.
Comprehensive analysis of ADAMTS13 in patients with liver cirrhosis.
Determination of ADAMTS13 and Its Clinical Significance for ADAMTS13 Supplementation Therapy to Improve the Survival of Patients with Decompensated Liver Cirrhosis.
Development of ADAMTS13 inhibitor in a patient with hepatitis C virus-related liver cirrhosis causes thrombotic thrombocytopenic purpura.
Increased ADAMTS-13 proteolytic activity in rat hepatic stellate cells upon activation in vitro and in vivo.
Increased production of ADAMTS13 in hepatic stellate cells contributes to enhanced plasma ADAMTS13 activity in rat models of cholestasis and steatohepatitis.
Plasma ADAMTS-13 protein is not associated with portal hypertension or hemodynamic changes in patients with cirrhosis.
Presence of portal vein thrombosis in liver cirrhosis is strongly associated with low levels of ADAMTS-13: a pilot study.
Serum ADAMTS-13 Levels as an Indicator of Portal Vein Thrombosis.
Liver Cirrhosis, Alcoholic
Decreased activity of plasma ADAMTS13 may contribute to the development of liver disturbance and multiorgan failure in patients with alcoholic hepatitis.
Increased von Willebrand Factor Over Decreased ADAMTS13 Activity May Contribute to the Development of Liver Disturbance and Multiorgan Failure in Patients With Alcoholic Hepatitis.
Liver Cirrhosis, Biliary
Relevance of ADAMTS13 to liver transplantation and surgery.
Liver Diseases
ADAMTS13 activity decreases after hepatectomy, reflecting a postoperative liver dysfunction.
ADAMTS13 activity may predict the cumulative survival of patients with liver cirrhosis in comparison with the Child-Turcotte-Pugh score and the Model for End-Stage Liver Disease score.
ADAMTS13 deficiency promotes microthrombosis in a murine model of diet-induced liver steatosis.
Change in plasma a disintegrin and metalloprotease with thrombospondin type-1 repeats-13 and von Willebrand factor levels in venous thromboembolic patients.
Localization of ADAMTS13 to the stellate cells of human liver.
Pivotal role of ADAMTS13 function in liver diseases.
Role of ADAMTS13 in diet-induced liver steatosis.
Liver Failure
ADAMTS13 activity decreases after hepatectomy, reflecting a postoperative liver dysfunction.
An unbalance between von Willebrand factor and ADAMTS13 in acute liver failure: Implications for hemostasis and clinical outcome.
Levels of von Willebrand factor antigen and von Willebrand factor cleaving protease (ADAMTS13) activity predict clinical events in chronic heart failure.
Liver Failure, Acute
An unbalance between von Willebrand factor and ADAMTS13 in acute liver failure: Implications for hemostasis and clinical outcome.
Decreased activity of plasma ADAMTS13 are related to enhanced cytokinemia and endotoxemia in patients with acute liver failure.
Lung Neoplasms
Association of ABO blood groups with von Willebrand factor, factor VIII and ADAMTS-13 in patients with lung cancer.
Increased von Willebrand factor over decreased ADAMTS-13 activity is associated with poor prognosis in patients with advanced non-small-cell lung cancer.
Lupus Erythematosus, Systemic
A Case of Thrombotic Thrombocytopenia Purpura Associated with Systemic Lupus Erythematosus: Diagnostic Utility of ADAMTS-13 Activity.
A patient with SLE-associated thrombotic microangiopathy and non-neutralizing antibodies against ADAMTS13.
Acquired deficiency of von Willebrand factor-cleaving protease in a patient suffering from acute systemic lupus erythematosus.
ADAMTS-13 metalloprotease abnormalities in systemic lupus erythematosus: is there a correlation with disease status?
Alteration of ADAMTS13 antigen levels in patients with idiopathic thrombotic thrombocytopenic purpura, idiopathic thrombocytopenic purpura and systemic lupus erythematosus.
Characteristics and Outcomes of Patients with Systemic Lupus Erythematosus-associated Thrombotic Microangiopathy, and Their Acquired ADAMTS13 Inhibitor Profiles.
Decreased von Willebrand factor protease activity associated with thrombocytopenic disorders.
Heterogeneous pathogenic processes of thrombotic microangiopathies in patients with connective tissue diseases.
Increased expression of ADAMTS13 mRNA correlates with ischemic cerebrovascular disease in systemic lupus erythematosus patients.
Microangiopathic haemolytic anaemia resembling thrombotic thrombocytopenic purpura in systemic lupus erythematosus: the role of ADAMTS13.
Occurrence of thrombotic thrombocytopenic purpura in a systemic lupus erythematosus patient with antiphospholipid antibodies in association with a decreased activity of von Willebrand factor-cleaving protease.
Reduced ADAMTS13 activity is associated with thrombotic risk in systemic lupus erythematosus.
Ribosomal and Immune Transcripts Associate with Relapse in Acquired ADAMTS13-Deficient Thrombotic Thrombocytopenic Purpura.
Successful treatment of refractory thrombotic thrombocytopenic purpura with cyclosporine and corticosteroids in a patient with systemic lupus erythematosus and antibodies to ADAMTS13.
Systemic lupus erythematosus with ADAMTS13 inhibitor-negative thrombotic microangiopathy treated with combination of mycophenolate mofetil, plasma exchange and steroid.
Thrombotic microangiopathic hemolytic anemia with reduction of ADAMTS13 activity: initial manifestation of childhood-onset systemic lupus erythematosus.
Thrombotic thrombocytopenic purpura as an initial presentation of systemic lupus erythematosus with acquired ADAMTS 13 antibody.
Von Willebrand factor cleaving protease (ADAMTS-13) in 123 patients with connective tissue diseases (systemic lupus erythematosus and systemic sclerosis).
[The association of von Willebrand factor and von Willebrand factor-cleaving protease in systemic lupus erythematosus]
Lupus Nephritis
Lupus nephritis combined with renal injury due to thrombotic thrombocytopaenic purpura-haemolytic uraemic syndrome.
Microangiopathic haemolytic anaemia secondary to lupus nephritis: an important differential diagnosis of thrombotic thrombocytopenic purpura.
Plasma ADAMTS-13 activity in proliferative lupus nephritis: a large cohort study from China.
Significance of plasma von Willebrand factor level and von Willebrand factor-cleaving protease activity in patients with chronic renal diseases.
Lymphoma
Chemotherapy-resistant intravascular lymphoma accompanied by ADAMTS13 inhibitor successfully treated with rituximab.
[Thrombotic microangiopathy]
Lymphopenia
Life-threatening COVID-19 presenting as stroke with antiphospholipid antibodies and low ADAMTS-13 activity, and the role of therapeutic plasma exchange: A case series.
Thrombotic microangiopathy as first manifestation of acute human immunodeficiency virus infection: a case report and review of the literature.
Malaria
ADAMTS13 deficiency with elevated levels of ultra-large and active von Willebrand factor in P. falciparum and P. vivax malaria.
Biomarkers of endothelial activation/dysfunction in infectious diseases.
Impact of HIV infection on the haemostatic response during sepsis and malaria.
Severe malaria is associated with a deficiency of von Willebrand factor cleaving protease, ADAMTS13.
Severe Plasmodium falciparum malaria is associated with circulating ultra-large von Willebrand multimers and ADAMTS13 inhibition.
Structure-function and regulation of ADAMTS-13 protease.
von Willebrand factor in experimental malaria-associated acute respiratory distress syndrome.
VWF excess and ADAMTS13 deficiency: a unifying pathomechanism linking inflammation to thrombosis in DIC, malaria, and TTP.
Malaria, Cerebral
ADAMTS13 and von Willebrand factor in thrombotic thrombocytopenic purpura.
Association of ADAMTS13 polymorphism with cerebral malaria.
Malaria, Falciparum
Severe Plasmodium falciparum malaria is associated with circulating ultra-large von Willebrand multimers and ADAMTS13 inhibition.
Malaria, Vivax
ADAMTS13 deficiency with elevated levels of ultra-large and active von Willebrand factor in P. falciparum and P. vivax malaria.
Melanoma
High Frequency of Mutations in Genes Encoding Factor VIII, von Willebrand Factor, and ADAMTS13 in Skin Cutaneous Melanoma.
Thrombotic thrombocytopenic purpura associated to dual checkpoint inhibitor therapy for metastatic melanoma.
Melioidosis
Correction: Increased Von Willebrand factor, decreased ADAMTS13 and thrombocytopenia in melioidosis.
Increased Von Willebrand factor, decreased ADAMTS13 and thrombocytopenia in melioidosis.
Mesenteric Ischemia
De Novo Mutation of the ADAMTS13 Gene with Mesenteric Ischemia in an Infant with Congenital Thrombotic Thrombocytopenic Purpura.
Monoclonal Gammopathy of Undetermined Significance
Antibodies against the CUB1-2 domains of ADAMTS13 in a patient with benign monoclonal gammopathy: no causal relationship.
Mucocutaneous Lymph Node Syndrome
Involvement of the ADAMTS13-VWF axis in acute Kawasaki disease and effects of intravenous immunoglobulin.
Multiple Sclerosis
ADAMTS13 ameliorates inflammatory responses in experimental autoimmune encephalomyelitis.
Interferon induced thrombotic microangiopathy (TMA): Analysis and concise review.
Thrombotic microangiopathy due to acquired ADAMTS13 deficiency in a patient receiving interferon-beta treatment for multiple sclerosis.
Myelodysplastic Syndromes
Does Outcome/Survival of Patients With Myelodysplastic Syndromes Should Be Predicted by Reduced Levels of ADAMTS-13? Results From a Pilot Study.
Myocardial Infarction
Acute myocardial infarction as a systemic prothrombotic condition evidenced by increased von Willebrand factor protein over ADAMTS13 activity in coronary and systemic circulation.
ADAMTS-13 attenuates thrombus formation on type I collagen surface and disrupted plaques under flow conditions.
ADAMTS13 and von Willebrand factor and the risk of myocardial infarction in men.
ADAMTS13 and von Willebrand factor in thrombotic thrombocytopenic purpura.
ADAMTS13 deficiency exacerbates VWF-dependent acute myocardial ischemia/reperfusion injury in mice.
ADAMTS13 safeguards the myocardium in a mouse model of acute myocardial infarction.
ADAMTS13--more than just TMA and TTP.
Association of the von Willebrand Factor-ADAMTS13 Ratio With Incident Cardiovascular Events in Patients With Peripheral Arterial Disease.
Effect of Acute Myocardial Infarction on a Disintegrin and Metalloprotease with Thrombospondin Motif 13 and Von Willebrand Factor and Their Relationship with Markers of Inflammation.
Evaluation and clinical application of a new method for detecting ADAMTS13 activity.
Evidence that high von Willebrand factor and low ADAMTS-13 levels independently increase the risk of a non-fatal heart attack.
High VWF, low ADAMTS13 puts women at risk.
High VWF, low ADAMTS13, and oral contraceptives increase the risk of ischemic stroke and myocardial infarction in young women.
Inverse Regulation of Confluence-Dependent ADAMTS13 and von Willebrand Factor Expression in Human Endothelial Cells.
Link between von Willebrand factor multimers, relapses and coronary microcirculation in patients with thrombotic thrombocytopenic purpura in remission.
Measurement of ADAMTS13 activity and inhibitors.
Molecular Imaging of VWF (von Willebrand Factor) and Platelet Adhesion in Postischemic Impaired Microvascular Reflow.
Plasma ADAMTS-13 levels and the risk of myocardial infarction: an individual patient data meta-analysis.
Plasma levels of active Von Willebrand factor are increased in patients with first ST-segment elevation myocardial infarction: A multicenter and multiethnic study.
Protective anti-inflammatory effect of ADAMTS13 on myocardial ischemia/reperfusion injury in mice.
Reduced von Willebrand factor-cleaving protease (ADAMTS13) activity in acute myocardial infarction.
Serial changes in von Willebrand factor-cleaving protease (ADAMTS13) and prognosis after acute myocardial infarction.
The role of ADAMTS13 in acute myocardial infarction: cause or consequence?
The Role of von Willebrand Factor in Vascular Inflammation: From Pathogenesis to Targeted Therapy.
Update on ADAMTS13 and VWF in cardiovascular and hematological disorders.
Verification of the Role of ADAMTS13 in the Cardiovascular Disease Using Two-Sample Mendelian Randomization.
Von Willebrand factor antigen predicts response to double dose of aspirin and clopidogrel by PFA-100 in patients undergoing primary angioplasty for ST elevation myocardial infarction.
[A study on the significance of plasma thrombospondin1 in thrombotic thrombocytopenic purpura and the relationship between thrombospondin1 and von Willebrand factor cleaving protease (ADAMTS13)]
[Determination of ADAMTS13 antigen and activity levels in patients with acute myocardial infarction and acute ischemic stroke]
[Genetic polymorphism of von Willebrand factor (VWF)-cleaving protease, ADAMTS13]
[Relationship between post-stenting coronary thrombolysis in myocardial infarction flow and plasma von Willebrand factor and ADAMTS-13 levels in patients with ST segment elevation myocardial infarction].
[Study of C1423T polymorphism of the von Willebrand factor-cleaving protease gene in Chinese Han population]
Myocardial Ischemia
ADAMTS-13 activity and ischemic heart disease: a Mendelian randomization study.
Mutations and Common Polymorphisms in ADAMTS13 and vWF Genes Responsible for Increasing Risk of Thrombosis.
Neoplasm Metastasis
ADAMTS-13 activity in patients with brain and prostate tumors is mildly reduced, but not correlated to stage of malignancy and metastasis.
Association of ABO blood groups with von Willebrand factor, factor VIII and ADAMTS-13 in patients with lung cancer.
Neoplasms
Acquired thrombotic thrombocytopenic purpura due to antibody-mediated ADAMTS13 deficiency precipitated by a localized Castleman's disease: A case report.
ADAMTS-13 activity in patients with brain and prostate tumors is mildly reduced, but not correlated to stage of malignancy and metastasis.
ADAMTS-13 and von Willebrand factor predict venous thromboembolism in patients with cancer.
ADAMTS13 and von Willebrand factor are useful biomarkers for sorafenib treatment efficiency in patients with hepatocellular carcinoma.
ADAMTS13 and von Willebrand factor in thrombotic thrombocytopenic purpura.
All-trans retinoic acid modulates the balance of ADAMTS13 and VWF in human microvascular endothelial cells.
Association between ADAMTS13 activity-VWF antigen imbalance and the therapeutic effect of HAIC in patients with hepatocellular carcinoma.
Atypical presentation of hepatic visceral larva migrans mimicking cancer and associated with ADAMTS13 deficiency-mediated thrombotic microangiopathy: A first report from Reunion Island.
Blood biomarkers associated with neurological deterioration in patients with acute penetrating artery territory infarction: A multicenter prospective observational study.
Cancer-related thrombotic microangiopathy secondary to Von Willebrand factor-cleaving protease deficiency.
Change in plasma a disintegrin and metalloprotease with thrombospondin type-1 repeats-13 and von Willebrand factor levels in venous thromboembolic patients.
Circular RNA profiling identifies circADAMTS13 as a miR-484 sponge which suppresses cell proliferation in hepatocellular carcinoma.
Decreased ADAMTS-13 level is related to inflammation factors and risk stratification of acute lymphoblastic leukemia patients.
Deficiency of von Willebrand factor-cleaving protease activity in the plasma of malignant patients.
Deficient activity of von Willebrand's factor-cleaving protease in patients with disseminated malignancies.
Detection of a secreted metalloprotease within the nuclei of liver cells.
Does Outcome/Survival of Patients With Myelodysplastic Syndromes Should Be Predicted by Reduced Levels of ADAMTS-13? Results From a Pilot Study.
Effect of plasma exchange on plasma ADAMTS13 metalloprotease activity, inhibitor level, and clinical outcome in patients with idiopathic and nonidiopathic thrombotic thrombocytopenic purpura.
Evaluation of von Willebrand factor-cleaving protease activity in patients with thrombotic thrombocytopenic purpura.
Impact of severe ADAMTS13 deficiency on clinical presentation and outcomes in patients with thrombotic microangiopathies: the experience of the Harvard TMA Research Collaborative.
Inverse Regulation of Confluence-Dependent ADAMTS13 and von Willebrand Factor Expression in Human Endothelial Cells.
Long-lasting prothrombotic state implied by changes of plasma von Willebrand factor parameters after radical prostatectomy for prostate malignancy.
Microangiopathic haemolytic anaemia in metastasizing malignant tumours is not associated with a severe deficiency of the von Willebrand factor-cleaving protease.
Microangiopathic Hemolytic Anemia and Thrombocytopenia in Patients With Cancer.
Patients with localized and disseminated tumors have reduced but measurable levels of ADAMTS-13 (von Willebrand factor cleaving protease).
Potential Role of Enhanced Cytokinemia and Plasma Inhibitor on the Decreased Activity of Plasma ADAMTS13 in Patients With Alcoholic Hepatitis: Relationship to Endotoxemia.
Presence of ADAMTS13 activity in a patient with metastatic cancer and thrombotic microangiopathy.
Prognostic value of anti-ADAMTS 13 antibody features (Ig isotype, titer, and inhibitory effect) in a cohort of 35 adult French patients undergoing a first episode of thrombotic microangiopathy with undetectable ADAMTS 13 activity.
Proteasome inhibitor associated thrombotic microangiopathy.
Quetiapine-induced thrombotic microangiopathy in a patient on maintenance dialysis.
Reduced ADAMTS-13 level negatively correlates with inflammation factors in plasma of acute myeloid leukemia patients.
The role of ADAMTS-13 and von Willebrand factor in cancer patients: Results from the Vienna Cancer and Thrombosis Study.
Validation of PLASMIC score: an academic medical center case series (2012-present).
von Willebrand factor fibers promote cancer-associated platelet aggregation in malignant melanoma of mice and humans.
Von Willebrand factor, ADAMTS13 activity, TNF-? and their relationships in patients with chronic kidney disease.
Von Willebrand factor:antigen and ADAMTS-13 level, but not soluble P-selectin, are risk factors for the first asymptomatic deep vein thrombosis in cancer patients undergoing chemotherapy.
[ADAMTS13 Level in Prothrombotic Status and Its Related Factor Analysis].
[Changes of ADAMTS13 Activity and TSP1 Level in Patients with Hematologic Malignancies].
[Residual collagen binding assay for von Willebrand factor-cleaving protease activity and its clinical application]
Nephrosclerosis
ADAMTS13--more than just TMA and TTP.
Nephrosis, Lipoid
Significance of plasma von Willebrand factor level and von Willebrand factor-cleaving protease activity in patients with chronic renal diseases.
Neuroblastoma
IL-1? Down-Regulates ADAMTS-13 mRNA Expression in Cells of the Central Nervous System.
Neurodegenerative Diseases
Increased expressions of ADAMTS-13, neuronal nitric oxide synthase, and neurofilament correlate with severity of neuropathology in Border disease virus-infected small ruminants.
Neuroinflammatory Diseases
ADAMTS13 gene deletion enhances plasma high-mobility group box1 elevation and neuroinflammation in brain ischemia-reperfusion injury.
Neurologic Manifestations
A case of pregnancy-induced thrombotic thrombocytopenic purpura with a kidney allograft recipient.
Acquired idiopathic ADAMTS13 activity deficient thrombotic thrombocytopenic purpura in a population from Japan.
Atypical presentations of thrombotic thrombocytopenic purpura: a diagnostic role for ADAMTS13.
Long-term response to rituximab in patients with relapsing thrombotic thrombocytopenic purpura.
Severe ADAMTS13 deficiency in adult idiopathic thrombotic microangiopathies defines a subset of patients characterized by various autoimmune manifestations, lower platelet count, and mild renal involvement.
Thrombotic microangiopathy without renal involvement: two novel mutations in complement-regulator genes.
[Allogeneic hematopoietic stem cell transplantation associated thrombotic microangiopathy: 16 cases report and literature review].
Non-alcoholic Fatty Liver Disease
Preserved hemostatic status in patients with non-alcoholic fatty liver disease.
Obesity
ADAMTS13 deficiency in mice does not affect adipose tissue development.
ADAMTS13 deficiency promotes microthrombosis in a murine model of diet-induced liver steatosis.
Dissecting the pathophysiology of immune thrombotic thrombocytopenic purpura: interplay between genes and environmental triggers.
Gender-dependent up-regulation of ADAMTS-13 in mice with obesity and hypercholesterolemia.
Identification of Novel Candidate Markers of Type 2 Diabetes and Obesity in Russia by Exome Sequencing with a Limited Sample Size.
Non-O blood group thrombotic thrombocytopenic purpura patients take longer to recover as measured by number of therapeutic plasma exchanges needed for platelet recovery.
Platelet rescue by macrophage depletion in obese ADAMTS-13-deficient mice at risk of thrombotic thrombocytopenic purpura.
Presence of anti-ADAMTS13 antibodies in obesity.
Weight loss reduces anti-ADAMTS13 autoantibodies and improves inflammatory and coagulative parameters in obese patients.
Oliguria
The kidney in thrombotic thrombocytopenic purpura.
Pancreatitis
A case of acute pancreatitis-induced microangiopathic hemolytic anemia with thrombocytopenia.
A Rare Case of Acquired Thrombotic Thrombocytopenic Purpura Triggered by Acute Pancreatitis.
A rare case of thrombotic microangiopathy triggered by acute pancreatitis.
Acute pancreatitis-induced thrombotic thrombocytopenic purpura with recurrent acute pancreatitis.
Lessons from acute pancreatitis-induced thrombotic thrombocytopenic purpura.
Pancreatitis preceding acute episodes of thrombotic thrombocytopenic purpura-hemolytic uremic syndrome: report of five patients with a systematic review of published reports.
Plasma ADAMTS13 activity parallels the APACHE II score, reflecting an early prognostic indicator for patients with severe acute pancreatitis.
Thrombotic thrombocytopenic purpura associated with severe acute pancreatitis in a context of decreased ADAMTS13 activity: a case report.
Persistent Infection
Increased expressions of ADAMTS-13 and apoptosis contribute to neuropathology during Toxoplasma gondii encephalitis in mice.
Placenta Diseases
Early ADAMTS13 testing associates with pre-eclampsia occurrence in antiphospholipid syndrome.
Pneumococcal Infections
Severe transient ADAMTS13 deficiency in pneumococcal-associated hemolytic uremic syndrome.
The thrombotic microangiopathies.
Pneumonia
ADAMTS-13 regulates neutrophil recruitment in a mouse model of invasive pulmonary aspergillosis.
Marked von Willebrand factor and factor VIII elevations in severe acute respiratory syndrome coronavirus-2-positive, but not severe acute respiratory syndrome coronavirus-2-negative, pneumonia: a case-control study.
Polycythemia Vera
Interferon induced thrombotic microangiopathy (TMA): Analysis and concise review.
Post-Cardiac Arrest Syndrome
Decreased a disintegrin-like and metalloprotease with thrombospondin type 1 motif 13 activity and neurologic outcome in patients with successful resuscitation of out-of-hospital cardiac arrest: A prospective observational study.
Pre-Eclampsia
Acute activation of the endothelium results in increased levels of active von Willebrand factor in hemolysis, elevated liver enzymes and low platelets (HELLP) syndrome.
ADAMTS 1, 4, 12, and 13 levels in maternal blood, cord blood, and placenta in preeclampsia.
ADAMTS13 and von Willebrand factor in thrombotic thrombocytopenic purpura.
ADAMTS13 gene variants and function in women with preeclampsia: a population- based nested case- control study from the HUNT Study.
ADAMTS13, FVIII, von Willebrand factor, ABO blood group assessment in preeclampsia.
Early ADAMTS13 testing associates with pre-eclampsia occurrence in antiphospholipid syndrome.
Expression of ADAMTS13 in Normal and Abnormal Placentae and Its Potential Role in Angiogenesis and Placenta Development.
Increased plasma von Willebrand factor antigen levels but normal von Willebrand factor cleaving protease (ADAMTS13) activity in preeclampsia.
Increased VWF antigen levels and decreased ADAMTS13 activity in preeclampsia.
Management of Thrombotic Thrombocytopenic Purpura with Autoantibodies to ADAMTS-13 and Concurrent Preeclampsia in Pregnancy: Multidisciplinary Team Approach.
Structure-function and regulation of ADAMTS-13 protease.
The association of pregnancy with thrombotic thrombocytopenic purpura-hemolytic uremic syndrome.
Thrombocytopenia in pregnancy.
Von Willebrand factor and ADAMTS13: a candidate couple for preeclampsia pathophysiology.
Von Willebrand factor antigen and ADAMTS13 activity assay in pregnant women and severe preeclamptic patients.
Precursor Cell Lymphoblastic Leukemia-Lymphoma
Decreased ADAMTS-13 level is related to inflammation factors and risk stratification of acute lymphoblastic leukemia patients.
Prediabetic State
ADAMTS13 activity as a novel risk factor for incident type 2 diabetes mellitus: a population-based cohort study.
Pregnancy Complications
ADAMTS-13 gene expression in antiphospholipid syndrome.
Proteinuria
ADAMTS13 inhibits oxidative stress and ameliorates progressive chronic kidney disease following ischaemia/reperfusion injury.
Characteristics and Outcomes of Patients with Systemic Lupus Erythematosus-associated Thrombotic Microangiopathy, and Their Acquired ADAMTS13 Inhibitor Profiles.
Microangiopathic haemolytic anaemia secondary to lupus nephritis: an important differential diagnosis of thrombotic thrombocytopenic purpura.
Plasma ADAMTS-13 activity in proliferative lupus nephritis: a large cohort study from China.
Successful treatment of refractory thrombotic thrombocytopenic purpura associated with systemic lupus erythematosus with combination of plasma exchange and low-dose rituximab.
Pulmonary Disease, Chronic Obstructive
Predictive value of ADAMTS-13 on concealed chronic renal failure in COPD patients.
Pulmonary Embolism
Changes in von Willebrand factor and ADAMTS-13 in patients following arthroplasty.
Therapeutic plasma exchange in patients with life-threatening COVID-19: a randomised controlled clinical trial.
Purpura
A Case of Thrombotic Thrombocytopenia Purpura Associated with Systemic Lupus Erythematosus: Diagnostic Utility of ADAMTS-13 Activity.
A patient with SLE-associated thrombotic microangiopathy and non-neutralizing antibodies against ADAMTS13.
A perspective on the measurement of ADAMTS13 in thrombotic thrombocytopaenic purpura.
A Severe Case of Congenital Thrombotic Thrombocytopenia Purpura Resulting From Compound Heterozygosity Involving a Novel ADAMTS13 Pathogenic Variant.
Acquired thrombotic thrombocytopenia purpura associated with severe ADAMTS13 deficiency in a 3-year-old boy: a case report and review of the literature.
ADAMTS 13 in non-thrombotic thrombocytopaenic purpura conditions.
ADAMTS-13 regulates platelet adhesion under flow. A new method for differentiation between inherited and acquired thrombotic thrombocytopenic purpura.
ADAMTS13 autoantibodies cloned from patients with acquired thrombotic thrombocytopenic purpura: 1. Structural and functional characterization in vitro.
Effects of naturally occurring mutations in CUB-1 domain on synthesis, stability, and activity of ADAMTS-13.
Emerging roles of adjunct therapies in acquired thrombotic thrombocytopenia purpura.
Establishment of the WHO 1st International Standard ADAMTS13, plasma (12/252): communication from the SSC of the ISTH.
Evaluation of ADAMTS-13 activity in plasma using recombinant von Willebrand Factor A2 domain polypeptide as substrate.
External validation of the PLASMIC score: a clinical prediction tool for thrombotic thrombocytopenic purpura diagnosis and treatment.
Fatal relapse of thrombotic thrombocytopaenic purpura after cardiac surgery in patient with congenital absence of vWF-cleaving protease activity.
Malignant hypertension as a rare cause of thrombotic microangiopathy.
Myocardial infarction is a complication of factor H-associated atypical HUS.
Prospective evaluation of ADAMTS-13 and von Willebrand factor multimers in cardiac surgery.
Refractory thrombotic thrombocytopenic purpura associated with oral contraceptives and factor V Leiden: a case report.
The role of ADAMTS-13 in the coagulopathy of sepsis.
Thrombotic thrombocytopaenic purpura in HIV-infected patients.
Thrombotic thrombocytopenic purpura relapse induced by acute hepatitis E transmitted by cryosupernatant plasma and successfully controlled with ribavirin.
[Hemolytic and uremic syndrome and related thrombotic microangiopathies: Epidemiology, pathophysiology and clinics].
Purpura, Thrombocytopenic
A successfully treated case of an acute presentation of congenital thrombotic thrombocytopenic purpura (Upshaw-Schulman syndrome) with decreased ADAMTS13 during late stage of pregnancy.
Acquired von Willebrand factor deficiency caused by LVAD is ADAMTS-13 and platelet dependent.
ADAMTS-13 regulates platelet adhesion under flow. A new method for differentiation between inherited and acquired thrombotic thrombocytopenic purpura.
ADAMTS13 turns 3.
ADAMTS13--more than just TMA and TTP.
Deficiency of ADAMTS-13 in thrombotic and thrombocytopenic purpura.
Diarrhea-associated Hemolytic Uremic Syndrome in Adults: Two Case Reports and Review of the Literature.
How do we reduce plasma transfusion in Rhode Island?
Interrelationship between ADAMTS13 activity, von Willebrand factor, and complement activation in remission from immune-mediated trhrombotic thrombocytopenic purpura.
Shiga Toxin as a Potential Trigger of CFHR1 Deletion-Associated Thrombotic Microangiopathy.
Two novel mutations in ADAMTS13 in a Chinese boy with congenital thrombocytopenic purpura: a case report.
[Identification of two novel mutations in ADAMTS13 gene in a patient with hereditary thrombotic thrombocytopenic purpura]
[Thrombotic Thrombocytopenic Purpura --Pathophysiology and Assays of ADAMTS13 Activity].
Purpura, Thrombocytopenic, Idiopathic
A successfully treated case of an acute presentation of congenital thrombotic thrombocytopenic purpura (Upshaw-Schulman syndrome) with decreased ADAMTS13 during late stage of pregnancy.
Alteration of ADAMTS13 antigen levels in patients with idiopathic thrombotic thrombocytopenic purpura, idiopathic thrombocytopenic purpura and systemic lupus erythematosus.
An Imperfect Marker: SLE and TTP-Like MAHA Without Low ADAMTS13.
Congenital ADAMTS13 Deficiency: A Rare Mimicker of Immune Thrombocytopenic Purpura.
Decreased von Willebrand factor protease activity associated with thrombocytopenic disorders.
Evaluation and clinical application of a new method for detecting ADAMTS13 activity.
Purpura, Thrombotic Thrombocytopenic
A 9-month-old infant with acquired idiopathic thrombotic thrombocytopenic purpura caused by inhibitory IgG-autoantibody to ADAMTS13.
A case for consideration by apheresis practitioners: Melanoma and PD-1 inhibitor treatment in a patient with multiple relapses of immune thrombotic thrombocytopenic purpura.
A case of congenital TTP presenting with microganiopathy in adulthood.
A case of severe ADAMTS13 deficiency presenting as thrombotic thrombocytopenic purpura in pregnancy.
A case of thrombotic thrombocytopenic purpura induced by acute pancreatitis.
A common origin of the 4143insA ADAMTS13 mutation.
A critical evaluation of caplacizumab for the treatment of acquired thrombotic thrombocytopenic purpura.
A Difficult Diagnosis Case of Prolonged Thrombocytopenia With Sepsis and Disseminated Intravascular Coagulation.
A human monoclonal antibody against the distal carboxyl terminus of ADAMTS-13 modulates its susceptibility to an inhibitor in thrombotic thrombocytopenic purpura.
A life-threatening case of pregnancy-related atypical Haemolytic uremic syndrome and successful treatment with Eculizumab.
A multicenter laboratory assessment of a new automated chemiluminescent assay for ADAMTS13 activity.
A new ADAMTS13 missense mutation (D1362V) in thrombotic thrombocytopenic purpura diagnosed during pregnancy.
A new mouse model mimicking thrombotic thrombocytopenic purpura: correction of symptoms by recombinant human ADAMTS13.
A Novel Case of Leflunomide-Induced Thrombotic Thrombocytopenic Purpura.
A novel CD46 mutation in a patient with microangiopathy clinically resembling thrombotic thrombocytopenic purpura and normal ADAMTS13 activity.
A novel homozygous frameshift mutation in Exon 7 of the ADAMTS13 gene in a patient with congenital thrombotic thrombocytopenic purpura from India: a case report.
A novel homozygous missense ADAMTS13 mutation Y658C in a patient with recurrent thrombotic thrombocytopenic purpura.
A novel mutation in ADAMTS13 of a child with Upshaw-Schulman Syndrome.
A novel quantitative hemolytic assay coupled with restriction fragment length polymorphisms analysis enabled early diagnosis of atypical hemolytic uremic syndrome and identified unique predisposing mutations in Japan.
A phenotype-genotype correlation of ADAMTS13 mutations in congenital thrombotic thrombocytopenic purpura patients treated in the United Kingdom.
A rapid enzyme-linked assay for ADAMTS-13.
A Severe Case of Congenital Thrombotic Thrombocytopenia Purpura Resulting From Compound Heterozygosity Involving a Novel ADAMTS13 Pathogenic Variant.
A shear-based assay for assessing plasma ADAMTS13 activity and inhibitors in patients with thrombotic thrombocytopenic purpura.
A successfully treated case of an acute presentation of congenital thrombotic thrombocytopenic purpura (Upshaw-Schulman syndrome) with decreased ADAMTS13 during late stage of pregnancy.
A Unique Case Involving a Female Patient with Upshaw-Schulman Syndrome: Low Titers of Antibodies against ADAMTS13 prior to Pregnancy Disappeared after Successful Delivery.
AAV-mediated expression of an ADAMTS13 variant prevents shigatoxin-induced thrombotic thrombocytopenic purpura.
Absence of exaggerated pharmacology by recombinant ADAMTS13 in the rat and monkey.
Absolute immature platelet count dynamics of thrombotic thrombocytopenic purpura patients with high ADAMTS13 inhibitor.
Absolute immature platelet count helps differentiate thrombotic thrombocytopenic purpura from hypertension-induced thrombotic microangiopathy.
Acquired deficiency of von Willebrand factor-cleaving protease in a patient with thrombotic thrombocytopenic purpura.
Acquired idiopathic ADAMTS13 activity deficient thrombotic thrombocytopenic purpura in a population from Japan.
Acquired thrombotic thrombocytopenic purpura due to antibody-mediated ADAMTS13 deficiency precipitated by a localized Castleman's disease: A case report.
Acquired thrombotic thrombocytopenic purpura with a smoldering clinical course.
Acquired thrombotic thrombocytopenic purpura with isolated CFHR3/1 deletion-rapid remission following complement blockade.
Acquired Thrombotic Thrombocytopenic Purpura Without Anti-ADAMTS13 Antibody Caused by Influenza A (H1N1) Virus Successfully Treated by Plasma Exchange: A Case Report.
Acquired thrombotic thrombocytopenic purpura: ADAMTS13 activity, anti-ADAMTS13 autoantibodies and risk of recurrent disease.
Acquired TTP: ADAMTS13 meets the immune system.
Acquired, noncongenital thrombotic thrombocytopenic purpura in children and adolescents: clinical management and the use of ADAMTS 13 assays.
Acute renal failure is prevalent in patients with thrombotic thrombocytopenic purpura associated with low plasma ADAMTS13 activity.
Acute renal failure is prevalent in patients with thrombotic thrombocytopenic purpura associated with low plasma ADAMTS13 activity: comment.
Acute renal failure is prevalent in patients with thrombotic thrombocytopenic purpura associated with low plasma ADAMTS13 activity: reply.
Acute thrombotic thrombocytopenic purpura in Louisiana: Seasonal distribution and evaluation of an ADAMTS13 order screening protocol.
ADAMTS proteases in cardiovascular physiology and disease.
ADAMTS proteins in human disorders.
ADAMTS-13 activity and autoantibodies classes and subclasses as prognostic predictors in acquired thrombotic thrombocytopenic purpura.
ADAMTS-13 activity and ischemic heart disease: a Mendelian randomization study.
ADAMTS-13 activity and von Willebrand factor levels in methylene-blue photo-inactivated plasma processed by either the Springe method or an 'in house' system.
ADAMTS-13 activity in plasma is rapidly measured by a new ELISA method that uses recombinant VWF-A2 domain as substrate.
ADAMTS-13 and Von Willebrand factor in relation to platelet response during plasma exchange in thrombotic thrombocytopenic purpura: a clue for disease mechanism?
ADAMTS-13 antigen and activity levels in thrombocytopenic disorders including thrombotic thrombocytopenic purpura in Kuwait.
ADAMTS-13 assays in thrombotic thrombocytopenic purpura.
ADAMTS-13 cysteine-rich/spacer domains are functionally essential for von Willebrand factor cleavage.
ADAMTS-13 gene expression in antiphospholipid syndrome.
ADAMTS-13 level in children with severe diarrhea-associated hemolytic uremic syndrome: Unmasking new association.
ADAMTS-13 levels in fresh, stored, and solvent detergent treated plasma.
ADAMTS-13 may not predict disease or outcome in patients with Thrombotic Thrombocytopenic Purpura.
ADAMTS-13 metalloprotease interacts with the endothelial cell-derived ultra-large von Willebrand factor.
ADAMTS-13 plasma level determination uncovers antigen absence in acquired thrombotic thrombocytopenic purpura and ethnic differences.
ADAMTS-13 regulates platelet adhesion under flow. A new method for differentiation between inherited and acquired thrombotic thrombocytopenic purpura.
ADAMTS-13, von Willebrand factor and related parameters in severe sepsis and septic shock.
ADAMTS13 activity and antigen during therapy and follow-up of patients with idiopathic thrombotic thrombocytopenic purpura: correlation with clinical outcome.
ADAMTS13 activity and genetic mutations in Japan.
ADAMTS13 activity and inhibitor.
ADAMTS13 activity and the presence of acquired inhibitors in human immunodeficiency virus-related thrombotic thrombocytopenic purpura.
ADAMTS13 activity and the risk of thrombotic thrombocytopenic purpura relapse in pregnancy.
ADAMTS13 and anti-ADAMTS13 antibodies as markers for recurrence of acquired thrombotic thrombocytopenic purpura during remission.
ADAMTS13 and anti-ADAMTS13 autoantibodies in thrombotic thrombocytopenic purpura - current perspectives and new treatment strategies.
ADAMTS13 and its variants promote angiogenesis via upregulation of VEGF and VEGFR2.
ADAMTS13 and microvascular thrombosis.
ADAMTS13 and thrombotic thrombocytopenic purpura: where we are and where we need to go.
ADAMTS13 and von Willebrand factor in thrombotic thrombocytopenic purpura.
ADAMTS13 and von Willebrand factor interactions.
ADAMTS13 antibody depletion by bortezomib in thrombotic thrombocytopenic purpura.
ADAMTS13 assays and ADAMTS13-deficient mice.
ADAMTS13 autoantibodies cloned from patients with acquired thrombotic thrombocytopenic purpura: 1. Structural and functional characterization in vitro.
ADAMTS13 autoantibodies cloned from patients with acquired thrombotic thrombocytopenic purpura: 2. Pathogenicity in an animal model.
ADAMTS13 autoantibodies in patients with thrombotic microangiopathies and other immunomediated diseases.
ADAMTS13 binds to CD36: a potential mechanism for platelet and endothelial localization of ADAMTS13.
ADAMTS13 content in plasma-derived factor VIII/von Willebrand factor concentrates.
ADAMTS13 Deficiency and Thrombotic Thrombocytopenic Purpura Associated with Trimethoprim-Sulfamethoxazole.
ADAMTS13 deficiency in mice does not affect adipose tissue development.
ADAMTS13 deficiency, an important cause of thrombocytopenia during pregnancy.
ADAMTS13 gene defects in two brothers with constitutional thrombotic thrombocytopenic purpura and normalization of von Willebrand factor-cleaving protease activity by recombinant human ADAMTS13.
ADAMTS13 gene mutation in congenital thrombotic thrombocytopenic purpura with previously reported normal VWF cleaving protease activity.
ADAMTS13 Gene Mutations Influence ADAMTS13 Conformation and Disease Age-Onset in the French Cohort of Upshaw-Schulman Syndrome.
ADAMTS13 is expressed in hepatic stellate cells.
ADAMTS13 kinetics after therapeutic plasma exchange and plasma infusion in patients with Upshaw-Schulman syndrome.
ADAMTS13 mutations and polymorphisms in congenital thrombotic thrombocytopenic purpura.
ADAMTS13 phenotype in plasma from normal individuals and patients with thrombotic thrombocytopenic purpura.
ADAMTS13 promotes angiogenesis and modulates VEGF-induced angiogenesis.
ADAMTS13 related markers and von Willebrand factor in plasma from patients with thrombotic microangiopathy (TMA).
ADAMTS13 Secretion and Residual Activity among Patients with Congenital Thrombotic Thrombocytopenic Purpura with and without Renal Impairment.
ADAMTS13 test and/or PLASMIC clinical score in management of acquired thrombotic thrombocytopenic purpura: a cost-effective analysis.
ADAMTS13 Testing Methodologies and Thrombotic Thrombocytopenic Purpura (TTP): Conflicting Results Can Pose a Clinical Dilemma.
ADAMTS13 testing update: Focus on laboratory aspects of difficult thrombotic thrombocytopenic purpura diagnoses and effects of new therapies.
ADAMTS13 unbound to larger von Willebrand factor multimers in cryosupernatant: implications for selection of plasma preparations for thrombotic thrombocytopenic purpura treatment.
ADAMTS13, thrombotic thrombocytopenic purpura, and hemolytic uremic syndrome.
ADAMTS13-binding IgG are present in patients with thrombotic thrombocytopenic purpura.
ADAMTS13-specific circulating immune complexes as potential predictors of relapse in patients with acquired thrombotic thrombocytopenic purpura.
Adenoviral-mediated gene transfer restores plasma ADAMTS13 antigen and activity in ADAMTS13 knockout mice.
Adult-onset congenital thrombotic thrombocytopenic purpura caused by a novel compound heterozygous mutation of the ADAMTS13 gene.
Advantages and limits of ADAMTS13 testing in the prognostic assessment of thrombotic thrombocytopenic purpura.
Advantages and limits of ADAMTS13 testing in thrombotic thrombocytopenic purpura.
Aetiology and pathogenesis of thrombotic thrombocytopenic purpura and haemolytic uraemic syndrome: the role of von Willebrand factor-cleaving protease.
AFM Imaging Reveals Multiple Conformational States of ADAMTS13.
Alteration of ADAMTS13 antigen levels in patients with idiopathic thrombotic thrombocytopenic purpura, idiopathic thrombocytopenic purpura and systemic lupus erythematosus.
Amino acid regions 572-579 and 657-666 of the spacer domain of ADAMTS13 provide a common antigenic core required for binding of antibodies in patients with acquired TTP.
Amino acid residues Arg(659), Arg(660), and Tyr(661) in the spacer domain of ADAMTS13 are critical for cleavage of von Willebrand factor.
Amplified endogenous plasmin activity resolves acute thrombotic thrombocytopenic purpura in mice.
An autoantibody epitope comprising residues R660, Y661, and Y665 in the ADAMTS13 spacer domain identifies a binding site for the A2 domain of VWF.
An enzyme immunoassay of ADAMTS13 distinguishes patients with thrombotic thrombocytopenic purpura from normal individuals and carriers of ADAMTS13 mutations.
An IAP retrotransposon in the mouse ADAMTS13 gene creates ADAMTS13 variant proteins that are less effective in cleaving von Willebrand factor multimers.
An inquiry into the relationship between ABO blood group and thrombotic thrombocytopenic purpura.
An open conformation of ADAMTS-13 is a hallmark of acute acquired thrombotic thrombocytopenic purpura.
An optimized fluorogenic ADAMTS13 assay with increased sensitivity for the investigation of patients with thrombotic thrombocytopenic purpura.
An unusual case of rapidly progressive hyperbilirubinemia.
An unusual presentation of primary pulmonary extranodal marginal zone lymphoma of mucosal associated lymphoid tissue: An autopsy case report.
An update on the pathogenesis and management of acquired thrombotic thrombocytopenic purpura.
Analyses of ADAMTS13 activity and its inhibitor in patients with thrombotic thrombocytopenic purpura secondary to connective tissue diseases: Observations in a single hospital.
Analysis on the Molecular Species and Concentration of Circulating ADAMTS13 in Blood.
Annual incidence and severity of acute episodes in hereditary thrombotic thrombocytopenic purpura.
Anti-ADAMTS13 Autoantibodies against Cryptic Epitopes in Immune-Mediated Thrombotic Thrombocytopenic Purpura.
Anti-ADAMTS13 autoantibodies in immune-mediated thrombotic thrombocytopenic purpura do not hamper ELISA-based quantification of ADAMTS13 antigen.
Anti-ADAMTS13 autoantibody profiling in patients with immune-mediated thrombotic thrombocytopenic purpura.
Antibodies to von Willebrand factor-cleaving protease in acute thrombotic thrombocytopenic purpura.
Apical sorting of ADAMTS13 in vascular endothelial cells and Madin-Darby canine kidney cells depends on the CUB domains and their association with lipid rafts.
Application of PLASMIC score in prediction of ADAMTS13 deficiency in a pediatric case of acquired thrombotic thrombocytopenic purpura.
Assays of ADAMTS-13 activity.
Assays of von Willebrand factor-cleaving protease: a test for diagnosis of familial and acquired thrombotic thrombocytopenic purpura.
Association between thrombotic microangiopathy and reduced ADAMTS13 activity in malignant hypertension.
Atypical Hemolytic Uremic Syndrome Secondary to Lupus Nephritis, Responsive to Eculizumab.
Atypical hemolytic uremic syndrome.
Atypical presentations of thrombotic thrombocytopenic purpura: a diagnostic role for ADAMTS13.
Autoimmune Thrombotic Thrombocytopenic Purpura: Two Rare Cases Associated with Juvenile Idiopathic Arthritis and Multiple Sclerosis.
Autopsy case of sudden maternal death from thrombotic thrombocytopenic purpura.
Bilateral serous retinal detachment as a complication of acquired peripartum thrombotic thrombocytopenic purpura bout.
Biological variations of ADAMTS13 and von Willebrand factor in human adults.
Blood group O and black race are independent risk factors for thrombotic thrombocytopenic purpura associated with severe ADAMTS13 deficiency.
Bortezomib for chronic relapsing thrombotic thrombocytopenic purpura: a case report.
Bortezomib in the treatment of refractory thrombotic thrombocytopenic purpura.
Bortezomib induces clinical remission and reduction of ADAMTS13 inhibitory antibodies in relapsed refractory idiopathic thrombotic thrombocytopenic purpura.
Cancer-associated Microangiopathic Hemolytic Anemia with Thrombocytopenia: an important diagnostic consideration.
Candidate gene analysis using genomic quantitative PCR: identification of ADAMTS13 large deletions in two patients with Upshaw-Schulman syndrome.
Caplacizumab in adult patients with acquired thrombotic thrombocytopenic purpura.
Caplacizumab Treatment for Acquired Thrombotic Thrombocytopenic Purpura.
Caplacizumab: a change in the paradigm of thrombotic thrombocytopenic purpura treatment.
Cardiac involvement in acute thrombotic thrombocytopenic purpura: association with troponin T and IgG antibodies to ADAMTS 13.
Case of maternal and fetal deaths due to severe congenital thrombotic thrombocytopenic purpura (Upshaw-Schulman syndrome) during pregnancy.
Case Report: Microangiopathic Hemolytic Anemia With Normal ADAMTS13 Activity.
CD4+ T cells from patients with acquired thrombotic thrombocytopenic purpura recognize CUB2 domain-derived peptides.
Cerebral MRI findings predict the risk of cognitive impairment in thrombotic thrombocytopenic purpura.
Change in plasma a disintegrin and metalloprotease with thrombospondin type-1 repeats-13 and von Willebrand factor levels in venous thromboembolic patients.
Changes in plasma von Willebrand factor and von Willebrand factor cleaving protease in thrombotic thrombocytopenic purpura: A case report.
Characterization and treatment of congenital thrombotic thrombocytopenic purpura.
Characterization of conformation-sensitive antibodies to ADAMTS13, the von Willebrand cleavage protease.
Chikungunya Fever Presenting as Life Threatening Thrombotic Thrombocytopenic Purpura.
Child-onset and adolescent-onset acquired thrombotic thrombocytopenic purpura with severe ADAMTS13 deficiency: a cohort study of the French national registry for thrombotic microangiopathy.
Child-onset thrombotic thrombocytopenic purpura caused by p.R498C and p.G259PfsX133 mutations in ADAMTS13.
Children and adults with thrombotic thrombocytopenic purpura associated with severe, acquired Adamts13 deficiency: comparison of incidence, demographic and clinical features.
Chronic relapsing thrombotic thrombocytopenic purpura due to a deficiency of von Willebrand factor-cleaving protease activity.
Ciclosporin and plasma exchange in thrombotic thrombocytopenic purpura: long-term follow-up with serial analysis of ADAMTS13 activity.
Circulating DNA and myeloperoxidase indicate disease activity in patients with thrombotic microangiopathies.
Cleavage of ultra-large von Willebrand factor by ADAMTS-13 under flow conditions.
Cleavage of von Willebrand factor by ADAMTS-13 on endothelial cells.
Cleavage of von Willebrand factor by granzyme M destroys its factor VIII binding capacity.
Cleavage of von Willebrand factor requires the spacer domain of the metalloprotease ADAMTS13.
Clinical and Laboratory Features of Patients with Acquired Thrombotic Thrombocytopenic Purpura: Fourteen Years of the Milan TTP Registry.
Clinical application of a rapid method using agarose gel electrophoresis and Western blotting to evaluate von Willebrand factor protease activity.
Clinical Characteristics and Outcome of Canadian Patients Diagnosed With Atypical Hemolytic Uremic Syndrome.
Clinical experience in treatment of thrombotic thrombocytopenic purpura--hemolytic uremic syndrome with 28 patients.
Clinical features of severe acquired ADAMTS13 deficiency in thrombotic thrombocytopenic purpura: the Korean TTP registry experience.
Clinical importance of ADAMTS13 activity during remission in patients with acquired thrombotic thrombocytopenic purpura.
Clinical study on five cases of thrombotic thrombocytopenic purpura complicating pregnancy.
Clinical use of a rapid collagen binding assay for von Willebrand factor cleaving protease in patients with thrombotic thrombocytopenic purpura.
Clinical usefulness of a functional assay for the von Willebrand factor cleaving protease (ADAMTS 13) and its inhibitor in a patient with thrombotic thrombocytopenic purpura.
Cloning, expression and functional characterization of the full-length murine ADAMTS13.
Cocaine-induced microangiopathic hemolytic anemia mimicking idiopathic thrombotic thrombocytopenic purpura: A case report and review of the literature.
Comparison and stability of ADAMTS13 activity in therapeutic plasma products.
Comparison of ADAMTS13 and Von Willebrand factor levels and activities, and plasminogen levels, in plasma products currently available for the treatment of thrombotic thrombocytopenic purpura in South Africa.
Comparison of Rituximab originator (MabThera) to biosimilar (Truxima) in patients with immune-mediated thrombotic thrombocytopenic purpura.
Comparison of von Willebrand factor antigen, von Willebrand factor-cleaving protease and protein S in blood components used for treatment of thrombotic thrombocytopenic purpura.
Complement Activation Associated with ADAMTS13 Deficiency in Human and Murine Thrombotic Microangiopathy.
Complement activation associated with ADAMTS13 deficiency may contribute to the characteristic glomerular manifestations in Upshaw-Schulman syndrome.
Complement activation in diseases presenting with thrombotic microangiopathy.
Complement factor H mutation in familial thrombotic thrombocytopenic purpura with ADAMTS13 deficiency and renal involvement.
Complete ADAMTS13 remission in a patient with refractory autoimmune-mediated thrombotic thrombocytopenic purpura after infliximab.
Complete deficiency in ADAMTS13 is prothrombotic, but it alone is not sufficient to cause thrombotic thrombocytopenic purpura.
Concentration and Subclass Distribution of Anti-ADAMTS13 IgG Autoantibodies in Different Stages of Acquired Idiopathic Thrombotic Thrombocytopenic Purpura.
Conformational plasticity of ADAMTS13 in hemostasis and autoimmunity.
Congenital ADAMTS13 Deficiency: A Rare Mimicker of Immune Thrombocytopenic Purpura.
Congenital microangiopathic hemolytic anemia and thrombocytopenia with unusually large von Willebrand factor multimers and von Willebrand factor-cleaving protease.
Congenital thrombotic thrombocytopenic purpura (Upshaw-Schulman syndrome) caused by novel ADAMTS13 mutations.
Congenital Thrombotic Thrombocytopenic Purpura Associated With Moyamoya Syndrome in a 3-Year-Old Girl: A Case Report.
Congenital thrombotic thrombocytopenic purpura associated with unilateral moyamoya disease.
Congenital thrombotic thrombocytopenic purpura caused by new compound heterozygous mutations of the ADAMTS13 gene.
Congenital thrombotic thrombocytopenic purpura in a large cohort of patients carrying a novel mutation in ADAMTS13 gene.
Congenital thrombotic thrombocytopenic purpura in association with a mutation in the second CUB domain of ADAMTS13.
Congenital Thrombotic Thrombocytopenic Purpura related to a Novel Mutation in ADAMTS13 Gene and Management during Pregnancy.
Congenital Thrombotic Thrombocytopenic Purpura With a Novel ADAMTS13 Gene Mutation.
Congenital thrombotic thrombocytopenic purpura with novel mutations in three unrelated Turkish children.
Congenital Thrombotic Thrombocytopenic Purpura: Atypical Presentation and New ADAMTS 13 Mutation in a Tunisian Child.
Coombs Positive Thrombotic Thrombocytopenic Purpura in a Male Pediatric Patient: An Urgent Diagnostic Challenge.
Correction of murine ADAMTS13 deficiency by hematopoietic progenitor cell-mediated gene therapy.
Correction to: Long-Term Prevention of Congenital Thrombotic Thrombocytopenic Purpura in ADAMTS13 Knockout Mice by Sleeping Beauty Transposon-Mediated Gene Therapy.
Correction to: Transfusion of Platelets Loaded With Recombinant ADAMTS13 (A Disintegrin and Metalloprotease With Thrombospondin Type 1 Repeats-13) Is Efficacious for Inhibiting Arterial Thrombosis Associated With Thrombotic Thrombocytopenic Purpura.
Cost-effectiveness of thrombotic thrombocytopenic purpura diagnosis: a retrospective analysis in the University Hospital Center of Lyon (France).
COVID 19 infection associated with thrombotic thrombocytopenic purpura.
COVID-19 as a Potential Trigger for Immune Thrombotic Thrombocytopenic Purpura and Reason for an Unusual Treatment: A Case Report.
Crystal structures of the noncatalytic domains of ADAMTS13 reveal multiple discontinuous exosites for von Willebrand factor.
Current and Future Perspectives on ADAMTS13 and Thrombotic Thrombocytopenic Purpura.
Current concepts in thrombotic thrombocytopenic purpura.
Current management and therapeutical perspectives in thrombotic thrombocytopenic purpura.
Current management of thrombotic thrombocytopenic purpura.
Current prophylactic plasma infusion protocols do not adequately prevent long-term cumulative organ damage in the Japanese congenital thrombotic thrombocytopenic purpura cohort.
Cyclosporin A impairs the secretion and activity of ADAMTS13 (a disintegrin and metalloprotease with thrombospondin type 1 repeat).
Cyclosporine or steroids as an adjunct to plasma exchange in the treatment of immune-mediated thrombotic thrombocytopenic purpura.
De Novo Mutation of the ADAMTS13 Gene with Mesenteric Ischemia in an Infant with Congenital Thrombotic Thrombocytopenic Purpura.
Decreased ADAMTS13 activity in plasma from patients with thrombotic thrombocytopenic purpura.
Decreased levels of von Willebrand factor-cleaving protease in coronary heart disease and thrombotic thrombocytopenic purpura: study of a simplified method for assaying the enzyme activity based on ristocetin-induced platelet aggregation.
Defective ADAMTS13 synthesis as a possible consequence of NASH in an obese patient with recurrent thrombotic thrombocytopenic purpura.
Deficiency of ADAMTS-13 in pediatric patients with severe sepsis and impact on in-hospital mortality.
Deficiency of ADAMTS13 and thrombotic thrombocytopenic purpura.
Deficiency of ADAMTS13 in thrombotic thrombocytopenic purpura.
Deficiency of von Willebrand factor-cleaving protease in familial and acquired thrombotic thrombocytopenic purpura.
Deficient activity of von Willebrand factor-cleaving protease in chronic relapsing thrombotic thrombocytopenic purpura.
Deficient activity of von Willebrand factor-cleaving protease in patients with Upshaw-Schulman syndrome.
Deficient activity of von Willebrand factor-cleaving protease in thrombotic thrombocytopenic purpura in the setting of adult-onset Still's disease.
Deficient activity of von Willebrand factor-cleaving protease in thrombotic thrombocytopenic purpura.
Defining the genetics of thrombotic microangiopathies.
Degradation of two novel congenital TTP ADAMTS13 mutants by the cell proteasome prevents ADAMTS13 secretion.
Demographic and ADAMTS13 biomarker data as predictors of early recurrences of idiopathic thrombotic thrombocytopenic purpura.
Derivation and external validation of the PLASMIC score for rapid assessment of adults with thrombotic microangiopathies: a cohort study.
Detection of intracellular ADAMTS13, a secreted zinc-metalloprotease, via flow cytometry.
Deterioration in Clinical Status Is Not Enough to Suspend Eculizumab: A Genetic Complement-Mediated Atypical Hemolytic Uremic Syndrome Case Report.
Determination of ADAMTS13 and Its Clinical Significance for ADAMTS13 Supplementation Therapy to Improve the Survival of Patients with Decompensated Liver Cirrhosis.
Development and validation of a multivariable prediction rule for detecting a severe acquired ADAMTS13 activity deficiency in patients with thrombotic microangiopathies.
Development and validation of a predictive model for death in acquired severe ADAMTS13 deficiency-associated idiopathic thrombotic thrombocytopenic purpura: the French TMA Reference Center experience.
Development of a clinically significant ADAMTS13 inhibitor in a patient with hereditary thrombotic thrombocytopenic purpura.
Development of ADAMTS13 inhibitor in a patient with hepatitis C virus-related liver cirrhosis causes thrombotic thrombocytopenic purpura.
Developments in the use of plasma exchange and adjunctive therapies to treat immune-mediated thrombotic thrombocytopenic purpura.
Diagnosis and follow-up of thrombotic thrombocytopenic purpura with an automated chemiluminescent ADAMTS13 activity immunoassay.
Diagnosis of complement alternative pathway disorders.
Diagnosis of Hereditary TTP Caused by Homozygosity for a Rare Complex ADAMTS13 Allele After Salmonella Infection in a 43-Year-Old Asylum Seeker.
Diagnosis of thrombotic thrombocytopenic purpura among patients with ADAMTS13 Activity 10%-20.
Diagnosis of thrombotic thrombocytopenic purpura with normal ADAMTS13 activity and absence of its inhibitor (anti-ADAMTS13 antibodies).
Diagnostic and prognostic values of ADAMTS13 activity measured during daily plasma exchange therapy in patients with acquired thrombotic thrombocytopenic purpura.
Diagnostic relevance of ADAMTS13 activity: evaluation of 28 patients with thrombotic thrombocytopenic purpura - hemolytic uremic syndrome clinical diagnosis.
Differentiation between severe HELLP syndrome and thrombotic microangiopathy, thrombotic thrombocytopenic purpura and other imitators.
Difficulties in diagnosing congenital thrombotic thrombocytopenic purpura.
Discrepant activity levels of von Willebrand factor-cleaving protease (ADAMTS-13) in congenital thrombotic thrombocytopenic purpura.
Disseminated intravascular coagulation: is it fact or fancy?
Dissociation between the level of von Willebrand factor-cleaving protease activity and disease in a patient with congenital thrombotic thrombocytopenic purpura.
Drop of residual plasmatic activity of ADAMTS13 to undetectable levels during acute disease in a patient with adult-onset congenital thrombotic thrombocytopenic purpura.
EB virus reactivation triggers thrombotic thrombocytopenic purpura in a healthy adult.
Effect of ADAMTS13 activity turnaround time on plasma utilization for suspected thrombotic thrombocytopenic purpura.
Effect of blood group on idiopathic thrombotic thrombocytopenic purpura.
Effect of plasma exchange on plasma ADAMTS13 metalloprotease activity, inhibitor level, and clinical outcome in patients with idiopathic and nonidiopathic thrombotic thrombocytopenic purpura.
Effect of prophylactic cyclosporine therapy on ADAMTS13 biomarkers in patients with idiopathic thrombotic thrombocytopenic purpura.
Effect of rituximab on B cell phenotype and serum B cell-activating factor levels in patients with thrombotic thrombocytopenic purpura.
Effects of naturally occurring mutations in CUB-1 domain on synthesis, stability, and activity of ADAMTS-13.
Efficacy and Safety of Rituximab for Refractory and Relapsing Thrombotic Thrombocytopenic Purpura: A Cohort of 10 Cases.
Efficacy of rituximab in acute refractory or chronic relapsing non-familial idiopathic thrombotic thrombocytopenic purpura: a systematic review with pooled data analysis.
Elevated plasma levels of syndecan-1 and soluble thrombomodulin predict adverse outcomes in thrombotic thrombocytopenic purpura.
Enzymatically active ADAMTS13 variants are not inhibited by anti-ADAMTS13 autoantibodies: a novel therapeutic strategy?
Epidemiology and pathophysiology of adulthood-onset thrombotic microangiopathy with severe ADAMTS13 deficiency (thrombotic thrombocytopenic purpura): a cross-sectional analysis of the French national registry for thrombotic microangiopathy.
Epitope analysis of autoantibodies to ADAMTS13 in patients with acquired thrombotic thrombocytopenic purpura.
Epitope mapping of ADAMTS13 autoantibodies in acquired thrombotic thrombocytopenic purpura.
Evaluating the impact of the ABO blood group on the clinical outcome of thrombotic thrombocytopenic purpura associated with severe ADAMTS13 deficiency.
Evaluation and clinical application of a new method for detecting ADAMTS13 activity.
Evaluation and clinical application of a new method for measuring activity of von Willebrand factor-cleaving metalloprotease (ADAMTS13).
Evaluation of a chromogenic commercial assay using VWF-73 peptide for ADAMTS13 activity measurement.
Evaluation of a rapid turn-over, fully-automated ADAMTS13 activity assay: a method comparison study.
Evaluation of complement regulatory components in patients with atypical hemolytic uremic syndrome.
Evaluation of von Willebrand factor-cleaving protease activity in patients with thrombotic thrombocytopenic purpura.
Evidence based therapeutic apheresis in autoimmune and other hemolytic anemias.
Evidence for a role of anti-ADAMTS13 autoantibodies despite normal ADAMTS13 activity in recurrent thrombotic thrombocytopenic purpura.
Evolution of ADAMTS13 antibodies in a fatal case of thrombotic thrombocytopenic purpura.
Exome Sequencing Identifies Abnormalities in Glycosylation and ANKRD36C in Patients with Immune-Mediated Thrombotic Thrombocytopenic Purpura.
Expression and characterization of recombinant human ADAMTS-13.
External validation of the PLASMIC score: a clinical prediction tool for thrombotic thrombocytopenic purpura diagnosis and treatment.
Factor XI/ADAMTS13 complexes are quantitatively insignificant in human plasma.
Familial acquired thrombotic thrombocytopenic purpura: ADAMTS13 inhibitory autoantibodies in identical twins.
Fatal case of chikungunya and concomitant thrombotic thrombocytopenic purpura in French Guiana during air flight medical evacuation.
Fatal congenital thrombotic thrombocytopenic purpura with apparent ADAMTS13 inhibitor: in vitro inhibition of ADAMTS13 activity by hemoglobin.
First reported case of congenital thrombotic thrombocytopenic purpura in Taiwan with novel mutation of ADAMTS13 gene.
FRETS-VWF73 rather than CBA assay reflects ADAMTS13 proteolytic activity in acquired thrombotic thrombocytopenic purpura patients.
FRETS-VWF73, a first fluorogenic substrate for ADAMTS13 assay.
Gain-of-function ADAMTS13 variants that are resistant to autoantibodies against ADAMTS13 in patients with acquired thrombotic thrombocytopenic purpura.
Generation and validation of small ADAMTS13 fragments for epitope mapping of anti-ADAMTS13 autoantibodies in immune-mediated thrombotic thrombocytopenic purpura.
Generation of Anti-Murine ADAMTS13 Antibodies and Their Application in a Mouse Model for Acquired Thrombotic Thrombocytopenic Purpura.
Genetic and Functional Characterization of ADAMTS13 Variants in a Patient Cohort with Upshaw-Schulman Syndrome Investigated in Germany.
Genetic screening in haemolytic uraemic syndrome.
Genetic variations in complement factors in patients with congenital thrombotic thrombocytopenic purpura with renal insufficiency.
Gold nanoparticle enhanced multiplexed biosensing on a fiber optic surface plasmon resonance probe.
Goodpasture's syndrome associated with thrombotic thrombocytopenic purpura secondary to an ADAMTS-13 deficit.
Hematopoietic cell transplantation-associated thrombotic microangiopathy: a review of pathophysiology, diagnosis, and treatment.
Heparin cofactor II as a predictor of thrombotic microangiopathy after bone marrow transplantation.
Heparin-induced thrombocytopenia antibody and the pathogenesis of thrombotic microangiopathy after stem cell transplantation.
Heparin-induced thrombocytopenia as a cause of prolonged low platelet count in a patient with thrombotic thrombocytopenic purpura treated with plasmapheresis.
Hepatic stellate cell damage may lead to decreased plasma ADAMTS13 activity in rats.
Hereditary thrombotic thrombocytopenic purpura and the hereditary TTP registry.
Hereditary Thrombotic Thrombocytopenic Purpura in a 9-Month Old: Diagnosing and Managing an Ultra-rare Disorder.
Hereditary Thrombotic Thrombocytopenic Purpura in a Chinese Boy With a Novel Compound Heterozygous Mutation of the ADAMTS13 Gene.
Heterogeneous pathogenic processes of thrombotic microangiopathies in patients with connective tissue diseases.
High prevalence of hereditary thrombotic thrombocytopenic purpura in central Norway: from clinical observation to evidence.
High-resolution epitope mapping by HX MS reveals the pathogenic mechanism and a possible therapy for autoimmune TTP syndrome.
Highly elevated plasma level of von Willebrand factor accelerates the formation of platelet thrombus under high shear stress in plasma with deficient ADAMTS13 activity.
Histone-induced thrombotic thrombocytopenic purpura in adamts13-/- zebrafish depends on von Willebrand factor.
HLA-DRB1*11: a strong risk factor for acquired severe ADAMTS13 deficiency-related idiopathic thrombotic thrombocytopenic purpura in Caucasians.
How I treat thrombotic thrombocytopenic purpura in pregnancy.
Human leukocyte antigen association in idiopathic thrombotic thrombocytopenic purpura: evidence for an immunogenetic link.
Human Neutrophil Peptides (HNPs) Inhibit Proteolytic Cleavage of von Willebrand Factor by ADAMTS13: A Potential Link between Inflammation and Thrombotic Thrombocytopenic Purpura.
Human neutrophil peptides and complement factor Bb in pathogenesis of acquired thrombotic thrombocytopenic purpura.
Humoral immune response to ADAMTS13 in acquired thrombotic thrombocytopenic purpura.
Hypersensitivity to plasma exchange in a patient with thrombotic thrombocytopenic purpura.
Identification of glycans on plasma-derived ADAMTS13.
Identification of N-linked glycosylation and putative O-fucosylation, C-mannosylation sites in plasma derived ADAMTS13.
Identification of novel mutations in ADAMTS13 in an adult patient with congenital thrombotic thrombocytopenic purpura.
Idiopathic Noncirrhotic Intrahepatic Portal Hypertension is Associated with Sustained ADAMTS13 Deficiency.
Idiopathic Relapsing Thrombotic Thrombocytopenic Purpura with Persistent ADAMTS13 Inhibitor Activity Treated Sequentially with Plasmapheresis, Rituximab, Cyclophosphamide and Splenectomy.
Immature platelet dynamics correlate with ADAMTS13 deficiency and predict therapy response in immune-mediated thrombotic thrombocytopenic purpura.
Immature platelet fraction can help adjust therapy in refractory thrombotic microangiopathic hemolytic anemia cases.
Immune-Mediated Thrombotic Thrombocytopenic Purpura: A Narrative Review of Diagnosis and Treatment in Adults.
Immune-Thrombotic Thrombocytopenic Purpura is a Rare Cause of Ischemic Stroke in Young Adults: Case Reports and Literature Review.
Immunoadsorption for the treatment of a patient with severe thrombotic thrombocytopenic purpura resistant to plasma exchange: kinetics of an inhibitor of ADAMTS13.
Immunochip analysis identifies novel susceptibility loci in the human leukocyte antigen region for acquired thrombotic thrombocytopenic purpura.
Immunogenic hotspots in the spacer domain of ADAMTS13 in immune-mediated thrombotic thrombocytopenic purpura.
Immunotherapy for thrombotic thrombocytopenic purpura.
Implementation of a rapid assay of ADAMTS13 activity was associated with improved 30-day survival rate in patients with acquired primary thrombotic thrombocytopenic purpura who received platelet transfusions.
In-vitro and in-vivo consequences of mutations in the von Willebrand factor cleaving protease ADAMTS13 in thrombotic thrombocytopenic purpura.
Inactivation of ADAMTS13 by plasmin as a potential cause of thrombotic thrombocytopenic purpura.
Incidence of obstetrical thrombotic thrombocytopenic purpura in a retrospective study within thrombocytopenic pregnant women. A difficult diagnosis and a treatable disease.
Increased urinary albumin excretion following recovery from thrombotic thrombocytopenic purpura due to acquired ADAMTS13 deficiency.
Increased VWF and Decreased ADAMTS-13 in COVID-19: Creating a Milieu for (Micro)Thrombosis.
Indicators Differentiating Thrombotic Thrombocytopenic Purpura From Other Thrombotic Microangiopathies in a Canadian Apheresis Referral Center.
Infection as Trigger for Congenital Thrombotic Thrombocytopenic Purpura in an Adult Patient.
Influence of Personality, Resilience and Life Conditions on Depression and Anxiety in 104 Patients Having Survived Acute Autoimmune Thrombotic Thrombocytopenic Purpura.
Inherited ADAMTS13 deficiency (Upshaw-Schulman syndrome): a short review.
Inherited ADMATS13 deficiency: When to evoke the in the newborn?
Inherited and de novo mutations of ADAMTS13 in a patient with Upshaw-Schulman syndrome.
Inherited Thrombotic Thrombocytopenic Purpura (Upshaw Schulman Syndrome) as Differential Diagnosis to Neonatal Septicaemia with Disseminated Intravascular Coagulation - a Case Series.
Inherited thrombotic thrombocytopenic purpura in children.
Inherited thrombotic thrombocytopenic purpura in pregnancy.
Inherited thrombotic thrombocytopenic purpura mimicking immune thrombocytopenic purpura during pregnancy: a case report.
Inherited Thrombotic Thrombocytopenic Purpura Revealed by Recurrent Strokes in a Male Adult: Case Report and Literature Review.
Inhibition of von Willebrand factor-platelet glycoprotein Ib interaction prevents and reverses symptoms of acute acquired thrombotic thrombocytopenic purpura in baboons.
Inhibitors of ADAMTS13: a potential factor in the cause of thrombotic microangiopathy in a renal allograft recipient.
Inhibitors of von Willebrand factor-cleaving protease in thrombotic thrombocytopenic purpura.
Inhibitory anti ADAMTS13 antibodies with a new rapid fully automated CLiA assay.
Inhibitory autoantibodies against ADAMTS-13 in patients with thrombotic thrombocytopenic purpura bind ADAMTS-13 protease and may accelerate its clearance in vivo.
Insights into 3D Structure of ADAMTS13: A Stepping Stone towards Novel Therapeutic Treatment of Thrombotic Thrombocytopenic Purpura.
Insights into ADAMTS13 structure: impact on thrombotic thrombocytopenic purpura diagnosis and management.
Interaction between Multimeric von Willebrand Factor and Complement: A Fresh Look to the Pathophysiology of Microvascular Thrombosis.
Interactions of von Willebrand factor and ADAMTS13 in von Willebrand disease and thrombotic thrombocytopenic purpura.
Interferon-? is not elevated in idiopathic thrombotic thrombocytopenic purpura patients.
International Council for Standardization in Haematology (ICSH) recommendations for laboratory measurement of ADAMTS13.
Intravenous gamma globulin for thrombotic microangiopathy of unknown etiology.
Inverse correlations between serum ADAMTS13 levels and systolic blood pressure, pulse pressure, and serum C-reactive protein levels observed at a general health examination in a Japanese population: A cross-sectional study.
Is factor V Leiden a risk factor for thrombotic microangiopathies without severe ADAMTS 13 deficiency?
Leukocyte proteases cleave von Willebrand factor at or near the ADAMTS13 cleavage site.
Limitations of ADAMTS-13 Activity Level in Diagnosing Thrombotic Thrombocytopenic Purpura in Pregnancy.
Linear relationship between ADAMTS13 activity and platelet dynamics even before severe thrombocytopenia.
Link between von Willebrand factor multimers, relapses and coronary microcirculation in patients with thrombotic thrombocytopenic purpura in remission.
Long Term Management of Acquired Thrombotic Thrombocytopenic Purpura Using Serial Plasma ADAMTS13 Measurements.
Long-Term Prevention of Congenital Thrombotic Thrombocytopenic Purpura in ADAMTS13 Knockout Mice by Sleeping Beauty Transposon-Mediated Gene Therapy.
Long-term response to rituximab in patients with relapsing thrombotic thrombocytopenic purpura.
Longitudinal assessments of plasma ADAMTS13 biomarkers predict recurrence of immune thrombotic thrombocytopenic purpura.
Loss of von Willebrand factor high-molecular-weight multimers at acute phase is associated with detectable anti-ADAMTS13 IgG and neurological symptoms in acquired thrombotic thrombocytopenic purpura.
Low activity of von Willebrand factor-cleaving protease is not restricted to patients suffering from thrombotic thrombocytopenic purpura.
Low ADAMTS-13 in plavix induced thrombotic thrombocytopenic purpura.
Low Incidence of ADAMTS13 Missense Mutation R1060W in Adult Egyptian Patients with Thrombotic Thrombocytopenic Purpura.
Low levels of ADAMTS-13 with high anti-ADAMTS-13 antibodies during remission of immune-mediated thrombotic thrombocytopenic purpura highly predict for disease relapse: A multi-institutional study.
Making the Correct Diagnosis in Thrombotic Microangiopathy: A Narrative Review.
Management of cancer-associated thrombotic microangiopathy: what is the right approach?
Management of thrombotic microangiopathy in pregnancy and postpartum: report from an international working group.
Management of Thrombotic Thrombocytopenic Purpura with Autoantibodies to ADAMTS-13 and Concurrent Preeclampsia in Pregnancy: Multidisciplinary Team Approach.
Mass spectrometry-assisted identification of ADAMTS13-derived peptides presented on HLA-DR and HLA-DQ.
Measurement of ADAMTS13 activity and inhibitors.
Measurement of von Willebrand factor-cleaving protease (ADAMTS-13) activity in plasma: a multicenter comparison of different assay methods.
Measuring ADAMTS13 activity in patients with suspected thrombotic thrombocytopenic purpura: when, how, and why?
Mechanisms of microvascular thrombosis in thrombotic thrombocytopenic purpura.
Mechanisms of the interaction between two ADAMTS13 gene mutations leading to severe deficiency of enzymatic activity.
Methodologies and clinical utility of ADAMTS-13 activity testing.
Microangiopathic haemolytic anaemia in metastasizing malignant tumours is not associated with a severe deficiency of the von Willebrand factor-cleaving protease.
Microangiopathic haemolytic anaemia resembling thrombotic thrombocytopenic purpura in systemic lupus erythematosus: the role of ADAMTS13.
Microangiopathic haemolytic anaemia with thrombocytopenia induced by vitamin B12 deficiency long term after gastrectomy.
Minor stroke as singular manifestation of hereditary thrombotic thrombocytopenic purpura in a young man.
Modifying ADAMTS13 to modulate binding of pathogenic autoantibodies of patients with acquired thrombotic thrombocytopenic purpura.
Modulation of ADAMTS13 secretion and specific activity by a combination of common amino acid polymorphisms and a missense mutation.
Molecular basis of ADAMTS13 dysfunction in thrombotic thrombocytopenic purpura.
Molecular biology of ADAMTS13 and diagnostic utility of ADAMTS13 proteolytic activity and inhibitor assays.
Molecular characterization of ADAMTS13 gene mutations in Japanese patients with Upshaw-Schulman syndrome.
Molecular characterization of four ADAMTS13 mutations responsible for congenital thrombotic thrombocytopenic purpura (Upshaw-Schulman syndrome).
Molecular cloning, in vitro expression and functional characterization of canine ADAMTS13.
More about low-dose rituximab and plasma exchange as front-line therapy for patients with thrombotic thrombocytopenic purpura.
mRNA treatment produces sustained expression of enzymatically active human ADAMTS13 in mice.
Multicentric evaluation of the new HemosIL Acustar® chemiluminescence ADAMTS13 activity assay.
Multiple B-cell clones producing antibodies directed to the spacer and disintegrin/thrombospondin type-1 repeat 1 (TSP1) of ADAMTS13 in a patient with acquired thrombotic thrombocytopenic purpura.
Multiple domains of ADAMTS13 are targeted by autoantibodies against ADAMTS13 in patients with acquired idiopathic thrombotic thrombocytopenic purpura.
Multiple in silico tools predict phenotypic manifestations in congenital thrombotic thrombocytopenic purpura.
Mutation of the H-bond acceptor S119 in the ADAMTS13 metalloprotease domain reduces secretion and substrate turnover in a patient with congenital thrombotic thrombocytopenic purpura.
N-glycan-mediated shielding of ADAMTS13 prevents binding of pathogenic autoantibodies in immune-mediated TTP.
N-Glycans of ADAMTS13 modulate its secretion and von Willebrand factor cleaving activity.
Natural history of thrombotic thrombocytopenic purpura and hemolytic uremic syndrome.
Natural history of Upshaw-Schulman syndrome based on ADAMTS13 gene analysis in Japan.
Neonate with Congenital Thrombotic Thrombocytopenic Purpura: a Case Report of a de novo Compound Heterozygote Mutation in ADAMTS13 Gene and Review of Literature.
Neutralization of inhibitory antibodies and restoration of therapeutic ADAMTS-13 activity levels in inhibitor-treated rats by the use of defined doses of recombinant ADAMTS-13.
New strategies in diagnosis and treatment of thrombotic thrombocytopenic purpura: case report and review.
Nonneutralizing IgM and IgG antibodies to von Willebrand factor-cleaving protease (ADAMTS-13) in a patient with thrombotic thrombocytopenic purpura.
Nonsteroidal Anti-inflammatory Drug Induced Thrombotic Thrombocytopenic Purpura.
Normal levels of ADAMTS13 and factor H are present in the pharmaceutically licensed plasma for transfusion (Octaplas) and in the universally applicable plasma (Uniplas) in development.
Novel ADAMTS-13 mutations in an adult with delayed onset thrombotic thrombocytopenic purpura.
Novel ADAMTS13 mutations in an obstetric patient with upshaw-schulman syndrome.
Novel compound heterozygote mutations (H234Q/R1206X) of the ADAMTS13 gene in an adult patient with Upshaw-Schulman syndrome showing predominant episodes of repeated acute renal failure.
Novel frameshift mutations in ADAMTS13 in two families with hereditary thrombotic thrombocytopenic purpura.
Novel Heterozygous Mutations of Congenital Thrombotic Thrombocytopenic Purpura: A Rare Case Report.
Novel monoclonal antibody-based enzyme immunoassay for determining plasma levels of ADAMTS13 activity.
Novel mutations in ADAMTS13 CUB domains cause abnormal pre-mRNA splicing and defective secretion of ADAMTS13.
Novel recombinant glycosylphosphatidylinositol (GPI)-anchored ADAMTS13 and variants for assessment of anti-ADAMTS13 autoantibodies in patients with thrombotic thrombocytopenic purpura.
Novel therapeutic approaches for thrombotic thrombocytopenic purpura.
Novel therapies in thrombotic thrombocytopenic purpura.
O-fucosylation is required for ADAMTS13 secretion.
Occurrence of thrombotic thrombocytopenic purpura in a systemic lupus erythematosus patient with antiphospholipid antibodies in association with a decreased activity of von Willebrand factor-cleaving protease.
Ofatumumab for acute treatment and prophylaxis of a patient with multiple relapses of acquired thrombotic thrombocytopenic purpura.
Open ADAMTS13, induced by antibodies, is a biomarker for subclinical immune-mediated thrombotic thrombocytopenic purpura.
Optimization of the detection of inhibitory autoantibodies against the VWF-cleaving protease ADAMTS13 with an automated chemiluminescent ADAMTS13 activity immunoassay.
Overweight individuals are at increased risk for thrombotic thrombocytopenic purpura.
Paradigm shift of childhood thrombotic thrombocytopenic purpura with severe ADAMTS13 deficiency.
Partial ADAMTS13 deficiency in atypical hemolytic uremic syndrome.
Pathogenesis of thrombotic thrombocytopenic purpura: ADAMTS13 deficiency and beyond.
Pharmacokinetics of plasma infusion in congenital thrombotic thrombocytopenic purpura.
Plasma ADAMTS13, von Willebrand Factor (VWF) and VWF Propeptide Profiles in Patients with DIC and Related Diseases.
Plasma levels of ADAMTS13 antigen determined with an enzyme immunoassay using a neutralizing monoclonal antibody parallel ADAMTS13 activity LevEls.
Plasma Levels of Big Endothelin-1 Are Associated with Renal Insufficiency and In-Hospital Mortality of Immune Thrombotic Thrombocytopenic Purpura.
Plasma levels of S100A8/A9, histone/DNA complexes, and cell-free DNA predict adverse outcomes of immune thrombotic thrombocytopenic purpura.
Plasma of patients with Upshaw-Schulman syndrome, a congenital deficiency of von Willebrand factor-cleaving protease activity, enhances the aggregation of normal platelets under high shear stress.
Plasma therapy in thrombotic thrombocytopenic purpura: review of the literature and the Bern experience in a subgroup of patients with severe acquired ADAMTS-13 deficiency.
Plasmin Cleavage of von Willebrand Factor as an Emergency Bypass for ADAMTS13 Deficiency in Thrombotic Microangiopathy.
Plasmin Cleaves Von Willebrand Factor at K1491-R1492 in the A1-A2 Linker Region in a Shear- and Glycan-Dependent Manner In Vitro.
Platelet activation and the formation of the platelet plug: deficiency of ADAMTS13 causes thrombotic thrombocytopenic purpura.
Platelet activation in hemolytic uremic syndrome.
Platelet rescue by macrophage depletion in obese ADAMTS-13-deficient mice at risk of thrombotic thrombocytopenic purpura.
Platelet-delivered ADAMTS13 inhibits arterial thrombosis and prevents thrombotic thrombocytopenic purpura in murine models.
Platelet-free shear flow assay facilitates analysis of shear-dependent functions of VWF and ADAMTS13.
Podocytes express ADAMTS13 in normal renal cortex and in patients with thrombotic thrombocytopenic purpura.
Polymorphisms and mutations of ADAMTS13 in the Japanese population and estimation of the number of patients with Upshaw-Schulman syndrome.
Poor responder to plasma exchange therapy in acquired thrombotic thrombocytopenic purpura is associated with ADAMTS13 inhibitor boosting: visualization of an ADAMTS13 inhibitor complex and its proteolytic clearance from plasma.
Positive Response and Increase in ADAMTS13 with Scheduled Rituximab in a Patient with Relapsing Thrombotic Thrombocytopenic Purpura.
Postoperative thrombotic thrombocytopenic purpura in an infant: case report and literature review.
Postpartum plasma exchange in a woman with suspected thrombotic thrombocytopenic purpura (TTP) vs. hemolysis, elevated liver enzymes, and low platelet syndrome (HELLP): a case study.
Posttransplantation thrombotic thrombocytopenic purpura: a single-center experience and a contemporary review.
Potential for Recombinant ADAMTS13 as an Effective Therapy for Acquired Thrombotic Thrombocytopenic Purpura.
Potential impact of a delayed ADAMTS13 result in the treatment of thrombotic microangiopathy: an economic analysis.
Potential role of ADAMTS13 in the progression of alcoholic hepatitis.
Practical issues in ADAMTS13 testing and emerging therapies in thrombotic thrombocytopenic purpura.
Prasugrel and Acquired Thrombotic Thrombocytopenic Purpura Associated with ADAMTS13 Activity Deficiency.
Preclinical assessment of a new recombinant ADAMTS-13 drug product (BAX930) for the treatment of thrombotic thrombocytopenic purpura.
Predicting response to plasma exchange in patients with thrombotic thrombocytopenic purpura with measurement of vWF-cleaving protease activity.
Prediction of spacer-?6 complex: a novel insight into binding of ADAMTS13 with A2 domain of von Willebrand factor under forces.
Predictive Value of Schistocytes in Recurrence of Acquired Thrombotic Thrombocytopenic Purpura With Severe ADAMTS13 Deficiency at Discontinuation of Daily Therapeutic Plasma Exchange.
Preemptive rituximab infusions after remission efficiently prevent relapses in acquired thrombotic thrombocytopenic purpura.
Preemptive rituximab prevents long-term relapses in immune-mediated thrombotic thrombocytopenic purpura.
Preferential HLA-DRB1*11 dependent presentation of CUB2 derived peptides by ADAMTS13 pulsed dendritic cells.
Pregnancy in Upshaw-Schulman syndrome.
Pregnancy-induced thrombocytopenia and TTP, and the risk of fetal death, in Upshaw-Schulman syndrome: a series of 15 pregnancies in 9 genotyped patients.
Presence of anti-ADAMTS13 antibodies in obesity.
Presenting ADAMTS13 antibody and antigen levels predict prognosis in immune-mediated thrombotic thrombocytopenic purpura.
Prevalence of the ADAMTS-13 missense mutation R1060W in late onset adult thrombotic thrombocytopenic purpura.
Prevention of relapse in patients with acquired thrombotic thrombocytopenic purpura undergoing elective surgery: a case series.
Prognostic utility of ADAMTS13 activity for the atypical hemolytic uremic syndrome (aHUS) and comparison of complement serology between aHUS and thrombotic thrombocytopenic purpura.
Prolonged inhibition of von Willebrand factor-cleaving protease after splenectomy in a 22-year-old patient with acute and plasma refractory thrombotic thrombocytopenic purpura.
Proteolytic fragmentation and sugar chains of plasma ADAMTS13 purified by a conformation-dependent monoclonal antibody.
Proteolytic inactivation of ADAMTS13 by plasmin in human plasma: risk of thrombotic thrombocytopenic purpura.
Purification of human von Willebrand factor-cleaving protease and its identification as a new member of the metalloproteinase family.
Quantitative Western blot analysis of plasma ADAMTS13 antigen in patients with Upshaw-Schulman syndrome.
Rapid quantitative assay of ADAMTS13 activity on an automated coagulation analyzer: clinical applications and comparison with immunoblot method.
Real-world experience with caplacizumab in the management of acute TTP.
Recombinant ADAMTS 13 in thrombotic thrombocytopenic purpura.
Recombinant ADAMTS13 normalizes von Willebrand factor-cleaving activity in plasma of acquired TTP patients by overriding inhibitory antibodies.
Recombinant thrombomodulin for secondary thrombotic thrombocytopenic purpura.
Recommendations for the diagnosis and treatment of patients with thrombotic thrombocytopenic purpura.
Recovery and half-life of von Willebrand factor-cleaving protease after plasma therapy in patients with thrombotic thrombocytopenic purpura.
Redefining outcomes in immune TTP: an international working group consensus report.
Reduced ADAMTS-13 level negatively correlates with inflammation factors in plasma of acute myeloid leukemia patients.
Reduced ADAMTS13 activity in delayed cerebral ischemia after aneurysmal subarachnoid hemorrhage.
Reduced ADAMTS13 activity is associated with thrombotic risk in systemic lupus erythematosus.
Relapsing thrombotic thrombocytopenic purpura with low ADAMTS13 antigen levels: An indication for splenectomy?
Relationship between ADAMTS13 activity, von Willebrand factor antigen levels and platelet function in the early and late phases after TIA or ischaemic stroke.
Relevance of ADAMTS13 to liver transplantation and surgery.
Remission in acute refractory and relapsing thrombotic thrombocytopenic purpura following rituximab is associated with a reduction in IgG antibodies to ADAMTS-13.
Remission of chronic thrombotic thrombocytopenic purpura after treatment with cyclophosphamide and rituximab.
Remission of thrombotic thrombocytopenic purpura in a patient with compound heterozygous deficiency of von Willebrand factor-cleaving protease by infusion of solvent/detergent plasma.
Renal thrombotic microangiopathy and pulmonary arterial hypertension in a patient with late-onset cobalamin C deficiency.
Residual plasmatic activity of ADAMTS13 is correlated with phenotype severity in congenital thrombotic thrombocytopenic purpura.
Residues Arg568 and Phe592 contribute to an antigenic surface for anti-ADAMTS13 antibodies in the spacer domain.
Retinal involvement in acute thrombotic thrombocytopenic purpura: a case report.
Retrospective analysis of bleeding events after central venous catheter placement in thrombotic thrombocytopenic purpura.
Ribosomal and Immune Transcripts Associate with Relapse in Acquired ADAMTS13-Deficient Thrombotic Thrombocytopenic Purpura.
Rituximab and intermediate-purity plasma-derived factor VIII concentrate (Koate®) as adjuncts to therapeutic plasma exchange for thrombotic thrombocytopenic purpura in patients with an ADAMTS13 inhibitor.
Rituximab as pre-emptive treatment in patients with thrombotic thrombocytopenic purpura and evidence of anti-ADAMTS13 autoantibodies.
Rituximab as prophylaxis in chronic relapsing thrombotic thrombocytopenic purpura: a case report and review of the literature.
Rituximab can be combined with daily plasma exchange to achieve effective B-cell depletion and clinical improvement in acute autoimmune TTP.
Rituximab for chronic recurring thrombotic thrombocytopenic purpura: a case report and review of the literature.
Rituximab for refractory and or relapsing thrombotic thrombocytopenic purpura related to immune-mediated severe ADAMTS13-deficiency: a report of four cases and a systematic review of the literature.
Rituximab induces remission of cerebral ischemia caused by thrombotic thrombocytopenic purpura.
Rituximab prevents recurrence of thrombotic thrombocytopenic purpura: a case report.
Rituximab prolongs the time to relapse in patients with immune thrombotic thrombocytopenic purpura: analysis of off-label use in Japan.
Rituximab therapy for thrombotic thrombocytopenic purpura: A proposed study of the Transfusion Medicine/Hemostasis Clinical Trials Network with a systematic review of rituximab therapy for immune-mediated disorders.
Role of ADAMTS13 in the management of thrombotic microangiopathies including thrombotic thrombocytopenic purpura (TTP).
Role of ADAMTS13 in the pathogenesis, diagnosis, and treatment of thrombotic thrombocytopenic purpura.
Safety and efficacy of cryoprecipitate-poor plasma as a replacement fluid for therapeutic plasma exchange in thrombotic thrombocytopenic purpura: A single center retrospective evaluation.
Schistocytic anaemia, severe thrombocytopenia, and renal dysfunction: thrombotic microangiopathy due to severe acquired ADAMTS-13 deficiency. Case 2.
Seasonal distribution of severe ADAMTS13 deficient idiopathic thrombotic thrombocytopenic purpura.
Sepsis-Induced Disseminated Intravascular Coagulation With Features of Thrombotic Thrombocytopenic Purpura: a Fatal Fulminant Syndrome.
Serial ADAMTS13 measurements during initial plasma exchange therapy guide decisions for management of unresponsive thrombotic thrombocytopenic purpura.
Severe ADAMTS13 deficiency in adult idiopathic thrombotic microangiopathies defines a subset of patients characterized by various autoimmune manifestations, lower platelet count, and mild renal involvement.
Severe deficiency of the specific von Willebrand factor-cleaving protease (ADAMTS 13) activity in a subgroup of children with atypical hemolytic uremic syndrome.
Severe HELLP syndrome masquerading as thrombocytopenic thrombotic purpura: a case report.
Severe malaria is associated with a deficiency of von Willebrand factor cleaving protease, ADAMTS13.
Severe secondary deficiency of von Willebrand factor-cleaving protease (ADAMTS13) in patients with sepsis-induced disseminated intravascular coagulation: its correlation with development of renal failure.
Severe vitamin B-12 deficiency in a child mimicking thrombotic thrombocytopenic purpura.
Shear stress and von Willebrand factor in health and disease.
Shigatoxin triggers thrombotic thrombocytopenic purpura in genetically susceptible ADAMTS13-deficient mice.
Siblings with congenital thrombotic thrombocytopenic purpura.
Simple plasma exchange reduced autoantibody to von Willebrand factor-cleaving protease in a Japanese man with ticlopidine-associated thrombotic thrombocytopenic purpura.
Simvastatin Increases ADAMTS13 Expression in Podocytes.
Single-nucleotide variations defining previously unreported ADAMTS13 haplotypes are associated with differential expression and activity of the VWF-cleaving protease in a Salvadoran congenital thrombotic thrombocytopenic purpura family.
SNPs in ADAMTS13.
Splenectomy in relapsing and plasma-refractory acquired thrombotic thrombocytopenic purpura.
Structural and functional correlation of ADAMTS13.
Structure of von Willebrand factor-cleaving protease (ADAMTS13), a metalloprotease involved in thrombotic thrombocytopenic purpura.
Structure-function and regulation of ADAMTS-13 protease.
Successful management of pregnancy-associated thrombotic thrombocytopenic purpura by monitoring ADAMTS13 activity.
Successful management of three patients with autoimmune thrombotic thrombocytopenic purpura with paradigm-changing therapy: Caplacizumab, steroids, plasma exchange, rituximab, and intravenous immunoglobulins (CASPERI).
Successful treatment of a young infant who developed high-titer inhibitors against VWF-cleaving protease (ADAMTS-13): important discrimination from Upshaw-Schulman syndrome.
Successful treatment of an elderly frail patient with acquired idiopathic thrombotic thrombocytopenic purpura under close monitoring of ADAMTS13 activity and anti-ADAMTS13 antibody titers.
Successful treatment of congenital thrombotic thrombocytopenic purpura using the intermediate purity factor VIII concentrate BPL 8Y.
Successful treatment of refractory thrombotic thrombocytopenic purpura with cyclosporine and corticosteroids in a patient with systemic lupus erythematosus and antibodies to ADAMTS13.
Successful treatment of thrombotic microangiopathy associated with dengue infection: A case report and literature review.
Successful treatment with rituximab for acute refractory thrombotic thrombocytopenic purpura related to acquired ADAMTS13 deficiency: A pediatric report and literature review.
Sustained response with rituximab in patients with thrombotic thrombocytopenic purpura: a report of 13 cases and review of the literature.
Synergistic effects of ADAMTS13 deficiency and complement activation in pathogenesis of thrombotic microangiopathy.
Synergistic interactions between interferon-gamma and TRAIL modulate c-FLIP in endothelial cells, mediating their lineage-specific sensitivity to thrombotic thrombocytopenic purpura plasma-associated apoptosis.
Systemic antithrombotic effects of ADAMTS13.
Systemic Lupus Erythematosus Presenting as Refractory Thrombotic Thrombocytopenic Purpura: A Diagnostic and Management Challenge. A Case Report and Concise Review of the Literature.
Targeting the inhibitor of ADAMTS13 in thrombotic thrombocytopenic purpura.
Ten candidate ADAMTS13 mutations in six French families with congenital thrombotic thrombocytopenic purpura (Upshaw-Schulman syndrome).
Ten patient stories illustrating the extraordinarily diverse clinical features of patients with thrombotic thrombocytopenic purpura and severe ADAMTS13 deficiency.
Ten years of prophylactic treatment with fresh-frozen plasma in a child with chronic relapsing thrombotic thrombocytopenic purpura as a result of a congenital deficiency of von Willebrand factor-cleaving protease.
The active conformation of von Willebrand factor in patients with thrombotic thrombocytopenic purpura in remission.
The ADAMTS(L) family and human genetic disorders.
The Alternative Pathway of Complement and the Evolving Clinical-Pathophysiological Spectrum of Atypical Hemolytic Uremic Syndrome.
The balance between von-Willebrand factor and its cleaving protease ADAMTS13: biomarker in systemic inflammation and development of organ failure?
The clinical utility of ADAMTS13 activity, antigen and autoantibody assays in thrombotic thrombocytopenic purpura.
The course of ADAMTS-13 activity and inhibitor titre in the treatment of thrombotic thrombocytopenic purpura with plasma exchange and vincristine.
The D173G mutation in ADAMTS-13 causes a severe form of congenital thrombotic thrombocytopenic purpura. A clinical, biochemical andin silico study.
The emerging concept of residual ADAMTS13 activity in ADAMTS13-deficient thrombotic thrombocytopenic purpura.
The first deletion mutation in the TSP1-6 repeat domain of ADAMTS13 in a family with inherited thrombotic thrombocytopenic purpura.
The function of ADAMTS13 in thrombogenesis in vivo: insights from mutant mice.
The HLA Variant rs6903608 Is Associated with Disease Onset and Relapse of Immune-Mediated Thrombotic Thrombocytopenic Purpura in Caucasians.
The homozygous p.C1024R- ADAMTS13 gene mutation links to a late-onset phenotype of Upshaw-Schulman syndrome in Japan.
The International Hereditary Thrombotic Thrombocytopenic Purpura Registry: Key findings at enrolment until 2017.
The macrophage mannose receptor promotes uptake of ADAMTS13 by dendritic cells.
The novel ADAMTS13-p.D187H mutation impairs ADAMTS13 activity and secretion and contributes to thrombotic thrombocytopenic purpura in mice.
The potential therapeutic benefit of targeting ADAMTS13 activity.
The role of ADAMT-13 in platelet adhesion in flow: methods for diagnosis of thrombotic thrombocytopenic purpura.
The role of ADAMTS-13 activity and complement mutational analysis in differentiating acute thrombotic microangiopathies.
The role of ADAMTS13 in the pathogenesis of thrombotic thrombocytopenic purpura-hemolytic uremic syndrome.
The role of von Willebrand factor in hemorrhagic and thrombotic disorders.
The significance of ADAMTS13 in a patient with thrombotic thrombocytopenic purpura complicated autoimmune hepatitis.
The spacer domain of ADAMTS13 contains a major binding site for antibodies in patients with thrombotic thrombocytopenic purpura.
The splenic autoimmune response to ADAMTS13 in thrombotic thrombocytopenic purpura contains recurrent antigen-binding CDR3 motifs.
The use of 50% albumin/plasma replacement fluid in therapeutic plasma exchange for thrombotic thrombocytopenic purpura.
The use of ADAMTS13 activity, platelet count, and serum creatinine to differentiate acquired thrombotic thrombocytopenic purpura from other thrombotic microangiopathies.
The utility of a fast turnaround ADAMTS13 activity in the diagnosis and exclusion of thrombotic thrombocytopenic purpura.
The utility of ADAMTS13 in differentiating TTP from other acute thrombotic microangiopathies: results from the UK TTP Registry.
The utility of patient characteristics in predicting severe ADAMTS13 deficiency and response to plasma exchange.
The von Willebrand factor-cleaving protease (ADAMTS-13) and the diagnosis of thrombotic thrombocytopenic purpura (TTP).
The VWF/ADAMTS13 axis in the antiphospholipid syndrome: ADAMTS13 antibodies and ADAMTS13 dysfunction.
Therapeutic efficacy of the platelet glycoprotein Ib antagonist anfibatide in murine models of thrombotic thrombocytopenic purpura.
Thrombocytopenia and severe hyperbilirubinemia in the neonatal period secondary to congenital thrombotic thrombocytopenic purpura and ADAMTS13 deficiency.
Thrombocytopenia in pregnancy.
Thrombocytopenia-Associated Multiple Organ Failure.
Thrombogenesis and thrombotic disorders based on 'two-path unifying theory of hemostasis': philosophical, physiological, and phenotypical interpretation.
Thrombosis, Microangiopathies, and Inflammation.
Thrombospondin-1 and ADAMTS13 competitively bind to VWF A2 and A3 domains in vitro.
Thrombotic microangiopathies and the linkage between von Willebrand factor and the alternative complement pathway.
Thrombotic microangiopathies assessment: mind the complement.
Thrombotic microangiopathies, thrombotic thrombocytopenic purpura, and ADAMTS-13.
Thrombotic microangiopathies: towards a pathophysiology-based classification.
Thrombotic microangiopathy (TTP and HUS): advances in differentiation and diagnosis.
Thrombotic microangiopathy associated with Valproic acid toxicity.
Thrombotic microangiopathy caused by interferon ?-1b for multiple sclerosis: a case report.
Thrombotic Microangiopathy in the Setting of HIV Infection: A Case Report and Review of the Differential Diagnosis and Therapy.
Thrombotic Thrombocytopenic Purpura and Anti-Thrombotic Therapy Targeted to Von Willebrand Factor.
Thrombotic thrombocytopenic purpura as an initial presentation of systemic lupus erythematosus with acquired ADAMTS 13 antibody.
Thrombotic thrombocytopenic purpura associated with pegylated-interferon alpha-2a by an ADAMTS13 inhibitor in a patient with chronic hepatitis C.
Thrombotic thrombocytopenic purpura associated with severe acute pancreatitis in a context of decreased ADAMTS13 activity: a case report.
Thrombotic thrombocytopenic purpura associated with von Willebrand factor-cleaving protease (ADAMTS13) deficiency in children.
Thrombotic thrombocytopenic purpura attributable to von Willebrand factor-cleaving protease inhibitor in an 8-year-old boy.
Thrombotic Thrombocytopenic Purpura Cases and Consumption of Large Quantities of Energy Drink: Related or Coincidence?
Thrombotic thrombocytopenic purpura directly linked with ADAMTS13 inhibition in the baboon (Papio ursinus).
Thrombotic thrombocytopenic purpura during pregnancy versus imitator of preeclampsia.
Thrombotic Thrombocytopenic Purpura in a Child Treated for Acute Lymphoblastic Leukemia: Case Report and Review of Literature.
Thrombotic thrombocytopenic purpura in a patient with rapidly progressive glomerulonephritis with both anti-glomerular basement membrane antibodies and myeloperoxidase anti-neutrophil cytoplasmic antibodies.
Thrombotic thrombocytopenic purpura in children.
Thrombotic thrombocytopenic purpura in IgG4-related disease with severe deficiency of ADAMTS-13 activity and IgG4 autoantibody against ADAMTS-13.
Thrombotic thrombocytopenic purpura presenting with pathologic fracture: a case report.
Thrombotic thrombocytopenic purpura related to ADAMTS13 deficiency, and successful treatment in a chimpanzee (Pan troglodytes verus).
Thrombotic thrombocytopenic purpura related to severe ADAMTS13 deficiency in children.
Thrombotic Thrombocytopenic Purpura Treated with Rituximab Associated with Primary Sjögren's Syndrome and Primary Hypothyroidism.
Thrombotic thrombocytopenic purpura with an autoantibody to ADAMTS13 complicating Sjögren's syndrome: two cases and a literature review.
Thrombotic Thrombocytopenic Purpura with Severe ADAMTS-13 Deficiency in a Patient with Antiphospholipid Antibodies and Charcot-Marie-Tooth Disease.
Thrombotic thrombocytopenic purpura with severe ADAMTS-13 deficiency in two patients with primary antiphospholipid syndrome.
Thrombotic thrombocytopenic purpura with unusual 33 recurrences: a case report.
Thrombotic thrombocytopenic purpura-what is new?
Thrombotic thrombocytopenic purpura.
Thrombotic thrombocytopenic purpura: a diagnostic and therapeutic challenge.
Thrombotic thrombocytopenic purpura: a moving target.
Thrombotic thrombocytopenic purpura: a thrombotic disorder caused by ADAMTS13 deficiency.
Thrombotic thrombocytopenic purpura: basic pathophysiology and therapeutic strategies.
Thrombotic Thrombocytopenic Purpura: Beyond Empiricism and Plasma Exchange.
Thrombotic thrombocytopenic purpura: pathogenesis, diagnosis and potential novel therapeutics.
Thrombotic thrombocytopenic purpura: The role of ADAMTS13.
Thrombotic thrombocytopenic purpura: Toward targeted therapy and precision medicine.
Ticlopidine-associated ADAMTS13 activity deficient thrombotic thrombocytopenic purpura in 22 persons in Japan: a report from the Southern Network on Adverse Reactions (SONAR).
Ticlopidine-Associated thrombotic thrombocytopenic purpura with an IgG-type inhibitor to von Willebrand factor-cleaving protease activity.
Transcriptional regulation of ADAMTS13.
Transfer of ADAMTS13 antibody-mediated thrombotic thrombocytopenic purpura via kidney transplantation.
Transfusion of Platelets Loaded With Recombinant ADAMTS13 (A Disintegrin and Metalloprotease With Thrombospondin Type 1 Repeats-13) Is Efficacious for Inhibiting Arterial Thrombosis Associated With Thrombotic Thrombocytopenic Purpura.
Treatment of Concurrent Thrombotic Thrombocytopenic Purpura and Graves' Disease: A Report on Two Cases.
Treatment of Congenital Thrombotic Thrombocytopenic Purpura With Eculizumab.
Treatment of refractory thrombotic thrombocytopenic purpura using multimodality therapy including splenectomy and cyclosporine.
Treatment with or without plasma exchange for patients with acquired thrombotic microangiopathy not associated with severe ADAMTS13 deficiency: a propensity score-matched study.
Two approaches to the clinical dilemma of treating TTP with therapeutic plasma exchange in patients with a history of anaphylactic reactions to plasma.
Two newborn-onset patients of Upshaw-Schulman syndrome with distinct subsequent clinical courses.
Two novel heterozygote missense mutations of the ADAMTS13 gene in a child with recurrent thrombotic thrombocytopenic purpura.
Two novel mutations in ADAMTS13 in a Chinese boy with congenital thrombocytopenic purpura: a case report.
Ultralarge von Willebrand factor multimers and normal ADAMTS13 activity in the umbilical cord blood.
Unwinding the von Willebrand factor strings puzzle.
Update on ADAMTS13 and VWF in cardiovascular and hematological disorders.
Upshaw-Schulman syndrome revisited: a concept of congenital thrombotic thrombocytopenic purpura.
Upshaw-Schulman Syndrome With c.2728C>T Mutation in ADAMTS13 Gene.
Upshaw-Schulman syndrome-associated ADAMTS13 variants possess proteolytic activity at the surface of endothelial cells and in simulated circulation.
Use of the ADAMTS13 Activity Assay Improved the Accuracy and Efficiency of the Diagnosis and Treatment of Suspected Acquired Thrombotic Thrombocytopenic Purpura.
Utility of ADAMTS13 assays in diagnosing thrombotic thrombocytopenic purpura.
Utility of ADAMTS13 assays in diagnosing thrombotic thrombocytopenic purpura. In reply.
Validation of a panel of ADAMTS13 assays for diagnosis of thrombotic thrombocytopenic purpura: activity, functional inhibitor, and autoantibody test.
Validation of PLASMIC score and follow-up data in a cohort of patients with suspected microangiopathies from Southern Italy.
Validation of PLASMIC score: an academic medical center case series (2012-present).
Validation of the PLASMIC score at a University Medical Center.
Validation of the PLASMIC score for predicting ADAMTS13 activity <10% in patients with suspected thrombotic thrombocytopenic purpura in Alberta, Canada.
Value of ADAMTS13 activity and inhibitor in the postmortem diagnosis of thrombotic thrombocytopenic purpura.
VH1-69 germline encoded antibodies directed towards ADAMTS13 in patients with acquired thrombotic thrombocytopenic purpura.
von Willebrand factor and its cleaving protease ADAMTS13 balance in coronary artery vessels: Lessons learned from thrombotic thrombocytopenic purpura. A narrative review.
Von Willebrand factor and thrombosis.
von Willebrand factor and thrombotic thrombocytopenic purpura.
von Willebrand factor cleaving protease (ADAMTS-13) and ADAMTS-13 neutralizing autoantibodies in 100 patients with thrombotic thrombocytopenic purpura.
Von Willebrand factor cleaving protease (ADAMTS-13) in 123 patients with connective tissue diseases (systemic lupus erythematosus and systemic sclerosis).
von Willebrand factor cleaving protease (ADAMTS13) is deficient in recurrent and familial thrombotic thrombocytopenic purpura and hemolytic uremic syndrome.
von Willebrand factor cleaving protease and ADAMTS13 mutations in childhood TTP.
von Willebrand Factor Multimer Formation Contributes to Immunothrombosis in Coronavirus Disease 2019.
von Willebrand factor, ADAMTS-13, and thrombotic thrombocytopenic purpura.
Von Willebrand factor, ADAMTS-13, and thrombotic thrombocytopenic purpura.
Von Willebrand factor, ADAMTS13, and thrombotic thrombocytopenic purpura.
von Willebrand factor, von Willebrand factor-cleaving protease, and shear stress.
Von Willebrand factor-cleaving protease (ADAMTS-13) activity in thrombotic microangiopathies: diagnostic experience 2001/2002 of a single research laboratory.
von Willebrand factor-cleaving protease (ADAMTS13) activity in normal non-pregnant women, pregnant and post-delivery women.
Von Willebrand factor-cleaving protease (ADAMTS13) in thrombocytopenic disorders: a severely deficient activity is specific for thrombotic thrombocytopenic purpura.
Von Willebrand factor-cleaving protease activity remains at the intermediate level in thrombotic thrombocytopenic purpura.
Von Willebrand factor-cleaving protease and Upshaw-Schulman syndrome.
von Willebrand factor-cleaving protease in childhood diarrhoea-associated haemolytic uraemic syndrome.
von Willebrand factor-cleaving protease in thrombotic thrombocytopenic purpura and hemolytic-uremic syndrome.
von Willebrand factor-cleaving protease in thrombotic thrombocytopenic purpura and the hemolytic-uremic syndrome.
Von Willebrand factor-cleaving protease in thrombotic thrombocytopenic purpura.
von Willebrand factor-cleaving protease inhibitor in a patient with human immunodeficiency syndrome-associated thrombotic thrombocytopenic purpura.
von Willebrand factor/ADAMTS-13 interactions at birth: implications for thrombosis in the neonatal period.
Von Willebrand factor: another janus-faced hemostasis protein.
von Willebrand factor: two sides of a coin.
VWF excess and ADAMTS13 deficiency: a unifying pathomechanism linking inflammation to thrombosis in DIC, malaria, and TTP.
VWF-cleaving protease (ADAMTS13) in premature infants.
What's new in the diagnosis and pathophysiology of thrombotic thrombocytopenic purpura.
[A child with relapsing haemolytic anemia and thrombocytopenia]
[A clinical study of congenital thrombotic thrombocytopenic purpura]
[A study on the significance of plasma thrombospondin1 in thrombotic thrombocytopenic purpura and the relationship between thrombospondin1 and von Willebrand factor cleaving protease (ADAMTS13)]
[Acquired thrombotic thrombocytopenic purpura after vascular prosthesis implantation for impending rupture of an abdominal aortic aneurysm].
[Activity loss of Von Willebrand factor cleaving protein (ADAMTS-13) is diagnostic for primary and pregnancy-related thrombotic thrombocytopenic purpura]
[ADAMTS13, von Willebrand factor specific cleaving protease].
[Biology of von Willebrand factor]
[Changes of ADAMTS13 activity and endothelial cell markers in TMA cases]
[Clinical application of a method for evaluating von Willebrand factor cleaving protease activity]
[Clinical observation of a patient with thrombotic thrombocytopenic purpura with renal and intestinal lesions].
[Determination of the ADAMTS13 antigen and its activity in TTP patients and carriers]
[Development and application of a new ADAMTS13 assay for TTP diagnosis]
[Diagnosis and treatment of thrombotic thrombocytopenic purpura].
[Diagnosis of thrombosis by hemostatic markers].
[Effect of Recombinant Protein in the Spacer Domain on ADAMTS13 Activity].
[Exacerbation of cranial nerurological symptoms by platelet transfusion before the diagnosis of thrombotic thrombocytopenic purpura].
[From gene to disease; congenital thrombotic thrombocytopenic purpura due to mutations in the ADAMTS13 gene]
[Frontline clinical practice for thrombotic thrombocytopenic purpura].
[Genetic polymorphism of von Willebrand factor (VWF)-cleaving protease, ADAMTS13]
[Hemolytic uremic syndrome and thrombotic thrombocytopenic purpura. Current perspectives on EHEC, complement mutations and ADAMTS13]
[Hemolytic-uremic syndrome]
[Hereditary form of thrombotic thrombocytopenic purpura]
[Human immunodeficiency virus-associated thrombotic microangiopathies.]
[Identification of two novel mutations in ADAMTS13 gene in a patient with hereditary thrombotic thrombocytopenic purpura]
[Latent malignant lymphoma diagnosed at autopsy in a patient with cold agglutinin disease coexisting thrombotic thrombocytopenic purpura].
[New marker in thrombotic thrombocytopenic purpura]
[Platelet--vessel wall interactions]
[Role of plasmapheresis and immunoadsorption in salvage therapy of rheumatological diseases].
[Structure and function of ADAMTS13 protease and its relation with diagnosis and treatment of TTP].
[Successful treatment with rituximab of a patient with coincident acquired hemophilia A and thrombotic thrombocytopenic purpura].
[The value of von Willebrand factor pro-peptide and ADAMTS13 in diagnosis of thrombotic thrombocytopenic purpura].
[Thrombotic microangiopathies].
[Thrombotic microangiopathy under an effective treatment with gemcitabine]
[Thrombotic thrombocytopenic purpura during pregnancy refractory to plasma exchange and rituximab].
[Thrombotic thrombocytopenic purpura in a child with low ADAMTS13 enzyme level.]
[Thrombotic thrombocytopenic purpura in childhood]
[Thrombotic thrombocytopenic purpura induced by antibody to von Willebrand factor-cleaving protease]
[Thrombotic thrombocytopenic purpura. Reduced activity of von Willebrand factor cleaving protease]
[Thrombotic thrombocytopenic purpura: Do not ignore cardiac involvement.]
[Transplantation related ADAMTS13 deficiency in thrombotic thrombocytopenic purpura: a case report and literature review].
[Treatment of immune-mediated thrombotic thrombocytopenic purpura: A decisive turning point].
[Treatment strategy for patients with thrombotic thrombocytopenic purpura].
[Variety of thrombotic thrombocytopenic purpura clinical course in Polish family members with ADAMTS 13 gene mutation].
[Von Willebrand factor and ADAMTS13 balancing primary haemostasis.]
Raynaud Disease
Severe ADAMTS13 deficiency in adult idiopathic thrombotic microangiopathies defines a subset of patients characterized by various autoimmune manifestations, lower platelet count, and mild renal involvement.
Renal Insufficiency
Change in plasma a disintegrin and metalloprotease with thrombospondin type-1 repeats-13 and von Willebrand factor levels in venous thromboembolic patients.
Is therapeutic plasma exchange indicated for patients with gemcitabine-induced hemolytic uremic syndrome?
Neutrophil Protease Cleavage of Von Willebrand Factor in Glomeruli - An Anti-thrombotic Mechanism in the Kidney.
Novel compound heterozygote mutations (H234Q/R1206X) of the ADAMTS13 gene in an adult patient with Upshaw-Schulman syndrome showing predominant episodes of repeated acute renal failure.
Prasugrel and Acquired Thrombotic Thrombocytopenic Purpura Associated with ADAMTS13 Activity Deficiency.
Severe ADAMTS13 deficiency in adult idiopathic thrombotic microangiopathies defines a subset of patients characterized by various autoimmune manifestations, lower platelet count, and mild renal involvement.
Severe secondary deficiency of von Willebrand factor-cleaving protease (ADAMTS13) in patients with sepsis-induced disseminated intravascular coagulation: its correlation with development of renal failure.
Sporadic bloody diarrhoea-associated thrombotic thrombocytopenic purpura-haemolytic uraemic syndrome: an adult and paediatric comparison.
Systemic infections mimicking thrombotic thrombocytopenic purpura.
Thrombotic microangiopathy due to acquired ADAMTS13 deficiency in a patient receiving interferon-beta treatment for multiple sclerosis.
Thrombotic Thrombocytopenic Purpura in a Child with Diabetic Ketoacidosis.
Ticlopidine- and clopidogrel-associated thrombotic thrombocytopenic purpura (TTP): review of clinical, laboratory, epidemiological, and pharmacovigilance findings (1989-2008).
Von Willebrand factor-cleaving protease (ADAMTS-13) activity in thrombotic microangiopathies: diagnostic experience 2001/2002 of a single research laboratory.
What's new in the diagnosis and pathophysiology of thrombotic thrombocytopenic purpura.
Renal Insufficiency, Chronic
ADAMTS-13 deficiency: can it cause chronic renal failure?
ADAMTS13 inhibits oxidative stress and ameliorates progressive chronic kidney disease following ischaemia/reperfusion injury.
Interferon induced thrombotic microangiopathy (TMA): Analysis and concise review.
Significance of plasma von Willebrand factor level and von Willebrand factor-cleaving protease activity in patients with chronic renal diseases.
Von Willebrand factor, ADAMTS13 activity, TNF-? and their relationships in patients with chronic kidney disease.
Reperfusion Injury
ADAMTS13 gene deletion aggravates ischemic brain damage: a possible neuroprotective role of ADAMTS13 by ameliorating postischemic hypoperfusion.
ADAMTS13 gene deletion enhances plasma high-mobility group box1 elevation and neuroinflammation in brain ischemia-reperfusion injury.
ADAMTS13 protects mice against renal ischemia-reperfusion injury by reducing inflammation and improving endothelial function.
Plasma ADAMTS13 activity may predict early adverse events in living donor liver transplantation: observations in 3 cases.
Relevance of ADAMTS13 to liver transplantation and surgery.
Von Willebrand Factor Aggravates Hepatic Ischemia-Reperfusion Injury by Promoting Neutrophil Recruitment in Mice.
Respiratory Distress Syndrome
Oxidative modification of von Willebrand factor by neutrophil oxidants inhibits its cleavage by ADAMTS13.
Retinal Detachment
Bilateral serous retinal detachment as a complication of acquired peripartum thrombotic thrombocytopenic purpura bout.
Rocky Mountain Spotted Fever
Twice-daily plasma exchange for patients with refractory thrombotic thrombocytopenic purpura: the experience of the Oklahoma Registry, 1989 through 2006.
Salmonella Infections
Diagnosis of Hereditary TTP Caused by Homozygosity for a Rare Complex ADAMTS13 Allele After Salmonella Infection in a 43-Year-Old Asylum Seeker.
Scleroderma, Systemic
ADAMTS13 deficiency and immunological abnormalities in patients with systemic sclerosis.
Heterogeneous pathogenic processes of thrombotic microangiopathies in patients with connective tissue diseases.
Pregnancy-associated thrombotic thrombocytopenic purpura with anti-centromere antibody-positive Raynaud's Syndrome.
The Plasma Levels of ADAMTS-13, von Willebrand Factor, VWFpp, and Fibrin-Related Markers in Patients With Systemic Sclerosis Having Thrombosis.
Von Willebrand factor cleaving protease (ADAMTS-13) in 123 patients with connective tissue diseases (systemic lupus erythematosus and systemic sclerosis).
Sepsis
Acid sphingomyelinase promotes endothelial stress response in systemic inflammation and sepsis.
Acquired ADAMTS-13 deficiency in pediatric patients with severe sepsis.
ADAMTS-13 (A disintegrin-like and metalloprotease with thrombospondin) and endothelial dysfunction in sepsis: marker or culprit?
ADAMTS-13 in Critically Ill Patients With Septic Syndromes and Non-Infectious Systemic Inflammatory Response Syndrome.
ADAMTS-13, von Willebrand factor and related parameters in severe sepsis and septic shock.
ADAMTS13 activity is decreased in a septic porcine model. Significance for glomerular thrombus deposition.
An open conformation of ADAMTS-13 is a hallmark of acute acquired thrombotic thrombocytopenic purpura.
Assessment of ADAMTS-13 Level in Hospitalized Children with Serious Bacterial Infections as a Possible Prognostic Marker.
Biomarkers of endothelial activation/dysfunction in infectious diseases.
Characterization of ADAMTS13 and von Willebrand factor levels in septic and non-septic ICU patients.
Clinical features and outcomes in patients with thrombotic microangiopathy not associated with severe ADAMTS13 deficiency.
Decreased a disintegrin-like and metalloprotease with thrombospondin (ADAMTS)-13 is associated with a poor prognosis in sepsis-induced organ failure*
Decreased ADAMTS 13 Activity is Associated With Disease Severity and Outcome in Pediatric Severe Sepsis.
Decreased ADAMTS-13 (A disintegrin-like and metalloprotease with thrombospondin type 1 repeats) is associated with a poor prognosis in sepsis-induced organ failure.
Deficiency of ADAMTS-13 in pediatric patients with severe sepsis and impact on in-hospital mortality.
Impact of HIV infection on the haemostatic response during sepsis and malaria.
Inflammation-associated ADAMTS13 deficiency promotes formation of ultra-large von Willebrand factor.
Inverse correlations between serum ADAMTS13 levels and systolic blood pressure, pulse pressure, and serum C-reactive protein levels observed at a general health examination in a Japanese population: A cross-sectional study.
Oxidation of Met1606 in von Willebrand factor is a risk factor for thrombotic and septic complications in chronic renal failure.
Oxidative modification of von Willebrand factor by neutrophil oxidants inhibits its cleavage by ADAMTS13.
Plasma Peptidylarginine Deiminase IV Promotes VWF-Platelet String Formation and Accelerates Thrombosis After Vessel Injury.
Preserved Expression of mRNA Coding von Willebrand Factor-Cleaving Protease ADAMTS13 by Selenite and Activated Protein C.
Prognostic value of ADAMTS13 in patients with severe sepsis and septic shock.
Prognostic value of anti-ADAMTS 13 antibody features (Ig isotype, titer, and inhibitory effect) in a cohort of 35 adult French patients undergoing a first episode of thrombotic microangiopathy with undetectable ADAMTS 13 activity.
Ratio of von Willebrand Factor Propeptide to ADAMTS13 is Associated with Severity of Sepsis.
Reduced ADAMTS13 in children with severe meningococcal sepsis is associated with severity and outcome.
Risk stratification and prognostic evaluation of endothelial cell-specific molecule1, von Willebrand factor, and a disintegrin-like and metalloprotease with thrombospondin type 1 motif for sepsis in the emergency department: An observational study.
Secondary thrombotic microangiopathy with severely reduced ADAMTS13 activity in a patient with Capnocytophaga canimorsus sepsis: a case report.
Severe transient ADAMTS13 deficiency in pneumococcal-associated hemolytic uremic syndrome.
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 role of ADAMTS-13 in the coagulopathy of sepsis.
The Role of von Willebrand Factor in Vascular Inflammation: From Pathogenesis to Targeted Therapy.
Variation of endothelium-related hemostatic factors during sepsis.
Variations in the ratio between von Willebrand factor and its cleaving protease during systemic inflammation and association with severity and prognosis of organ failure.
Von Willebrand factor and ADAMTS13 impact on the outcome of Staphylococcus aureus sepsis.
von Willebrand factor is a major determinant of ADAMTS-13 decrease during mouse sepsis induced by cecum ligation and puncture.
VWF excess and ADAMTS13 deficiency: a unifying pathomechanism linking inflammation to thrombosis in DIC, malaria, and TTP.
[ADAMTS13 Level in Prothrombotic Status and Its Related Factor Analysis].
Severe Dengue
Severe dengue is associated with consumption of von Willebrand factor and its cleaving enzyme ADAMTS-13.
Shock, Septic
ADAMTS-13, von Willebrand factor and related parameters in severe sepsis and septic shock.
Decreased ADAMTS 13 Activity is Associated With Disease Severity and Outcome in Pediatric Severe Sepsis.
Prognostic value of ADAMTS13 in patients with severe sepsis and septic shock.
Ratio of von Willebrand Factor Propeptide to ADAMTS13 is Associated with Severity of Sepsis.
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.
Spinal Cord Injuries
ADAMTS-13 is produced by glial cells and upregulated after spinal cord injury.
Spotted Fever Group Rickettsiosis
Twice-daily plasma exchange for patients with refractory thrombotic thrombocytopenic purpura: the experience of the Oklahoma Registry, 1989 through 2006.
ST Elevation Myocardial Infarction
Acquired intracoronary ADAMTS13 deficiency and VWF retention at sites of critical coronary stenosis in patients with STEMI.
Clinical significance of dynamic changes in hs-CRP and ADAMTS13 levels in the blood serum of patients with no-reflow after PCI operation.
The role of ADAMTS13 in acute myocardial infarction: cause or consequence?
Still's Disease, Adult-Onset
Deficient activity of von Willebrand factor-cleaving protease in thrombotic thrombocytopenic purpura in the setting of adult-onset Still's disease.
Stroke
ADAMTS proteases in cardiovascular physiology and disease.
ADAMTS13 and von Willebrand factor in thrombotic thrombocytopenic purpura.
ADAMTS13 controls vascular remodeling by modifying VWF reactivity during stroke recovery.
ADAMTS13 gene deletion aggravates ischemic brain damage: a possible neuroprotective role of ADAMTS13 by ameliorating postischemic hypoperfusion.
ADAMTS13-mediated thrombolysis of t-PA resistant occlusions in ischemic stroke in mice.
ADAMTS13: An Emerging Target in Stroke Therapy.
Association of the von Willebrand Factor-ADAMTS13 Ratio With Incident Cardiovascular Events in Patients With Peripheral Arterial Disease.
Biomarkers of Unfavorable Outcome in Acute Ischemic Stroke Patients with Successful Recanalization by Endovascular Thrombectomy.
Endothelial Cell-Derived von Willebrand Factor Is the Major Determinant That Mediates von Willebrand Factor-Dependent Acute Ischemic Stroke by Promoting Postischemic Thrombo-Inflammation.
Enhanced activity of an ADAMTS-13 variant (R568K/F592Y/R660K/Y661F/Y665F) against platelet agglutination in vitro and in a murine model of acute ischemic stroke.
High levels of von Willebrand factor and low levels of its cleaving protease, ADAMTS13, are associated with stroke in young HIV-infected patients.
High von Willebrand factor levels increase the risk of first ischemic stroke: influence of ADAMTS13, inflammation, and genetic variability.
In silico features of ADAMTS13 contributing to plasmatic ADAMTS13 levels in neonates with congenital heart disease.
Increased expression of ADAMTS13 mRNA correlates with ischemic cerebrovascular disease in systemic lupus erythematosus patients.
Life-threatening COVID-19 presenting as stroke with antiphospholipid antibodies and low ADAMTS-13 activity, and the role of therapeutic plasma exchange: A case series.
Measurement of ADAMTS13 activity and inhibitors.
Minor stroke as singular manifestation of hereditary thrombotic thrombocytopenic purpura in a young man.
Rare Variants in the ADAMTS13 Von Willebrand Factor-Binding Domain Contribute to Pediatric Stroke.
Recombinant ADAMTS13 reduces tissue plasminogen activator-induced hemorrhage after stroke in mice.
Reduced ADAMTS13 activity during TTP remission is associated with stroke in TTP survivors.
Reduced ADAMTS13 levels in patients with acute and chronic cerebrovascular disease.
Role of reduced ADAMTS13 in arterial ischemic stroke: a pediatric cohort study.
Serum and cerebrospinal fluid host proteins indicate stroke in children with tuberculous meningitis.
Targeting von Willebrand Factor in Ischaemic Stroke: Focus on Clinical Evidence.
The role of ADAMTS13 testing in the diagnosis and management of thrombotic microangiopathies and thrombosis.
The Role of von Willebrand Factor in Vascular Inflammation: From Pathogenesis to Targeted Therapy.
The Role of von Willebrand Factor, ADAMTS13, and Cerebral Artery Thrombus Composition in Patient Outcome Following Mechanical Thrombectomy for Acute Ischemic Stroke.
Tissue Plasminogen Activator Neurotoxicity is Neutralized by Recombinant ADAMTS 13.
von Willebrand Factor Antigen, von Willebrand Factor Propeptide, and ADAMTS13 in Carotid Stenosis and Their Relationship with Cerebral Microemboli.
von Willebrand factor-cleaving protease ADAMTS13 reduces ischemic brain injury in experimental stroke.
[Exacerbation of cranial nerurological symptoms by platelet transfusion before the diagnosis of thrombotic thrombocytopenic purpura].
Subarachnoid Hemorrhage
Association between variation in ADAMTS13 and aneurysmal subarachnoid hemorrhage.
Effect of ADAMTS-13 on cerebrovascular microthrombosis and neuronal injury after experimental subarachnoid hemorrhage.
Effect of recombinant ADAMTS13 on microthrombosis and brain injury after experimental subarachnoid hemorrhage.
Expression and significance of vWF, GMP-140 and ADAMTS13 in patients with aneurysmal subarachnoid hemorrhage.
Reduced ADAMTS13 activity in delayed cerebral ischemia after aneurysmal subarachnoid hemorrhage.
Role of von Willebrand factor and ADAMTS-13 in early brain injury after experimental subarachnoid hemorrhage.
The changes of von willebrand factor/a disintegrin-like and metalloprotease with thrombospondin type I repeats-13 balance in aneurysmal subarachnoid hemorrhage.
Thalassemia
ADAMTS13 Levels in Young Patients With ?-Thalassemia Major: Relation to Hepatitis C Virus Infection, Liver Cirrhosis, and Iron Overload.
Thrombocytopenia
A 9-month-old infant with acquired idiopathic thrombotic thrombocytopenic purpura caused by inhibitory IgG-autoantibody to ADAMTS13.
A case of pregnancy-induced thrombotic thrombocytopenic purpura with a kidney allograft recipient.
A Case of Thrombotic Thrombocytopenia Purpura Associated with Systemic Lupus Erythematosus: Diagnostic Utility of ADAMTS-13 Activity.
A Difficult Diagnosis Case of Prolonged Thrombocytopenia With Sepsis and Disseminated Intravascular Coagulation.
A rare combination of thrombotic thrombocytopenic purpura and antiphospholipid syndrome.
A role for tissue plasminogen activator in thrombotic thrombocytopenic purpura.
A Severe Case of Congenital Thrombotic Thrombocytopenia Purpura Resulting From Compound Heterozygosity Involving a Novel ADAMTS13 Pathogenic Variant.
Absolute immature platelet count helps differentiate thrombotic thrombocytopenic purpura from hypertension-induced thrombotic microangiopathy.
Acquired deficiency of von Willebrand factor-cleaving protease in a patient with thrombotic thrombocytopenic purpura.
Acquired thrombotic thrombocytopenia purpura associated with severe ADAMTS13 deficiency in a 3-year-old boy: a case report and review of the literature.
Acquired thrombotic thrombocytopenic purpura with a smoldering clinical course.
ADAMTS-13 activity, microangiopathic haemolytic anaemia and thrombocytopenia following snake bite envenomation.
ADAMTS-13 gene expression in antiphospholipid syndrome.
ADAMTS13 activity decreases after hepatectomy, reflecting a postoperative liver dysfunction.
ADAMTS13 autoantibodies cloned from patients with acquired thrombotic thrombocytopenic purpura: 1. Structural and functional characterization in vitro.
ADAMTS13 deficiency with elevated levels of ultra-large and active von Willebrand factor in P. falciparum and P. vivax malaria.
ADAMTS13 deficiency, an important cause of thrombocytopenia during pregnancy.
ADAMTS13 mutations and polymorphisms in congenital thrombotic thrombocytopenic purpura.
Assessment and Monitoring of Patients with Immune-Mediated Thrombotic Thrombocytopenic Purpura (iTTP): Strategies to Improve Outcomes.
Autoimmune Thrombotic Thrombocytopenic Purpura: Two Rare Cases Associated with Juvenile Idiopathic Arthritis and Multiple Sclerosis.
C3 Glomerulopathy and Atypical Hemolytic Uremic Syndrome: Two Important Manifestations of Complement System Dysfunction.
Caplacizumab Treatment for Acquired Thrombotic Thrombocytopenic Purpura.
Characteristics and Outcomes of Patients with Systemic Lupus Erythematosus-associated Thrombotic Microangiopathy, and Their Acquired ADAMTS13 Inhibitor Profiles.
Clinical application of a rapid method using agarose gel electrophoresis and Western blotting to evaluate von Willebrand factor protease activity.
Clinical usefulness of a functional assay for the von Willebrand factor cleaving protease (ADAMTS 13) and its inhibitor in a patient with thrombotic thrombocytopenic purpura.
Complete deficiency in ADAMTS13 is prothrombotic, but it alone is not sufficient to cause thrombotic thrombocytopenic purpura.
Congenital microangiopathic hemolytic anemia and thrombocytopenia with unusually large von Willebrand factor multimers and von Willebrand factor-cleaving protease.
Congenital Thrombotic Thrombocytopenic Purpura With a Novel ADAMTS13 Gene Mutation.
Correction: Increased Von Willebrand factor, decreased ADAMTS13 and thrombocytopenia in melioidosis.
Current management and therapeutical perspectives in thrombotic thrombocytopenic purpura.
Decreased von Willebrand factor protease activity associated with thrombocytopenic disorders.
Deficiency of ADAMTS-13 in pediatric patients with severe sepsis and impact on in-hospital mortality.
Differentiating malignant hypertension-induced thrombotic microangiopathy from thrombotic thrombocytopenic purpura.
Early response to caplacizumab and rituximab after anaphylaxis to Octaplas plasma in a patient with thrombotic thrombocytopenic purpura.
EB virus reactivation triggers thrombotic thrombocytopenic purpura in a healthy adult.
Effects of naturally occurring mutations in CUB-1 domain on synthesis, stability, and activity of ADAMTS-13.
Emerging roles of adjunct therapies in acquired thrombotic thrombocytopenia purpura.
Erratum to: Prolonged thrombocytopenia after living donor liver transplantation is a strong prognostic predictor irrespective of history of splenectomy: the significance of ADAMTS13 and graft function.
Evaluation of ADAMTS-13 activity in plasma using recombinant von Willebrand Factor A2 domain polypeptide as substrate.
External validation of the PLASMIC score: a clinical prediction tool for thrombotic thrombocytopenic purpura diagnosis and treatment.
Hemolysis, Elevated Liver Enzymes, and Low Platelet Count Syndrome With Severe Thrombocytopenia and Severe ADAMTS13 Activity Deficiency.
Hereditary Thrombotic Thrombocytopenic Purpura in a 9-Month Old: Diagnosing and Managing an Ultra-rare Disorder.
Hereditary Thrombotic Thrombocytopenic Purpura in a Chinese Boy With a Novel Compound Heterozygous Mutation of the ADAMTS13 Gene.
Immature platelet fraction can help adjust therapy in refractory thrombotic microangiopathic hemolytic anemia cases.
Immune-Mediated Thrombotic Thrombocytopenic Purpura: A Narrative Review of Diagnosis and Treatment in Adults.
Impact of a multidisciplinary team for the management of thrombotic microangiopathy.
Incidence of obstetrical thrombotic thrombocytopenic purpura in a retrospective study within thrombocytopenic pregnant women. A difficult diagnosis and a treatable disease.
Increased Von Willebrand factor, decreased ADAMTS13 and thrombocytopenia in melioidosis.
Infection as Trigger for Congenital Thrombotic Thrombocytopenic Purpura in an Adult Patient.
Inherited thrombotic thrombocytopenic purpura mimicking immune thrombocytopenic purpura during pregnancy: a case report.
Inherited Thrombotic Thrombocytopenic Purpura Revealed by Recurrent Strokes in a Male Adult: Case Report and Literature Review.
Intensive plasma exchange increases a disintegrin and metalloprotease with thrombospondin motifs-13 activity and reverses organ dysfunction in children with thrombocytopenia-associated multiple organ failure.
Intensive plasma exchange increases ADAMTS-13 activity and reverses organ dysfunction in children with thrombocytopenia-associated multiple organ failure.
Interferon induced thrombotic microangiopathy (TMA): Analysis and concise review.
Is it quinine TTP/HUS or quinine TMA? ADAMTS13 levels and implications for therapy.
Is therapeutic plasma exchange indicated for patients with gemcitabine-induced hemolytic uremic syndrome?
Linear relationship between ADAMTS13 activity and platelet dynamics even before severe thrombocytopenia.
Long-term response to rituximab in patients with relapsing thrombotic thrombocytopenic purpura.
Mechanisms of microvascular thrombosis in thrombotic thrombocytopenic purpura.
Mutation of the H-bond acceptor S119 in the ADAMTS13 metalloprotease domain reduces secretion and substrate turnover in a patient with congenital thrombotic thrombocytopenic purpura.
Nivolumab-Induced Thrombotic Thrombocytopenic Purpura in a Patient with Anal Squamous Cell Carcinoma: A Lesson on Hematologic Toxicity from Immunotherapy.
Nonsteroidal Anti-inflammatory Drug Induced Thrombotic Thrombocytopenic Purpura.
Novel therapies in thrombotic thrombocytopenic purpura.
Parasite biomass-related inflammation, endothelial activation, microvascular dysfunction and disease severity in vivax malaria.
Pathophysiology of thrombotic thrombocytopenic purpura.
Plasma ADAMTS13 activity may predict early adverse events in living donor liver transplantation: observations in 3 cases.
Platelet ligands and ADAMTS13 during Puumala hantavirus infection and associated thrombocytopenia.
Platelet rescue by macrophage depletion in obese ADAMTS-13-deficient mice at risk of thrombotic thrombocytopenic purpura.
Platelets: thrombotic thrombocytopenic purpura.
Postoperative hemolytic uremic syndrome with renal cortical necrosis following laparoscopic hemicolectomy.
Prasugrel and Acquired Thrombotic Thrombocytopenic Purpura Associated with ADAMTS13 Activity Deficiency.
Predictive Value of Schistocytes in Recurrence of Acquired Thrombotic Thrombocytopenic Purpura With Severe ADAMTS13 Deficiency at Discontinuation of Daily Therapeutic Plasma Exchange.
Pregnancy-associated thrombotic thrombocytopenic purpura with anti-centromere antibody-positive Raynaud's Syndrome.
Pregnancy-induced thrombocytopenia and TTP, and the risk of fetal death, in Upshaw-Schulman syndrome: a series of 15 pregnancies in 9 genotyped patients.
Presumed complement-mediated, checkpoint inhibitor-induced, thrombotic microangiopathy in a patient with metastatic melanoma.
Prolonged thrombocytopenia after living donor liver transplantation is a strong prognostic predictor irrespective of history of splenectomy: the significance of ADAMTS13 and graft function.
Rapid ADAMTS13 availability impacts treatment for microangiopathic hemolytic anemia and thrombocytopenia.
Recombinant thrombomodulin for secondary thrombotic thrombocytopenic purpura.
Recommendations for the diagnosis and treatment of patients with thrombotic thrombocytopenic purpura.
Relapse Rate in Survivors of Acute Autoimmune Thrombotic Thrombocytopenic Purpura Treated with or without Rituximab.
Remission of thrombotic thrombocytopenic purpura in a patient with compound heterozygous deficiency of von Willebrand factor-cleaving protease by infusion of solvent/detergent plasma.
Rituximab as pre-emptive treatment in patients with thrombotic thrombocytopenic purpura and evidence of anti-ADAMTS13 autoantibodies.
Rituximab induces remission of cerebral ischemia caused by thrombotic thrombocytopenic purpura.
Schistocytic anaemia, severe thrombocytopenia, and renal dysfunction: thrombotic microangiopathy due to severe acquired ADAMTS-13 deficiency. Case 2.
Serial ADAMTS13 measurements during initial plasma exchange therapy guide decisions for management of unresponsive thrombotic thrombocytopenic purpura.
Severe ADAMTS13 deficiency in adult idiopathic thrombotic microangiopathies defines a subset of patients characterized by various autoimmune manifestations, lower platelet count, and mild renal involvement.
Severe vitamin B-12 deficiency in a child mimicking thrombotic thrombocytopenic purpura.
Siblings with congenital thrombotic thrombocytopenic purpura.
Silent Thrombotic Thrombocytopenic Purpura: PLASMIC, Lessons Learned, and Current Management Overview.
Spontaneous recovery in a patient with acquired thrombotic thrombocytopenic purpura (TTP): observation of a 'subclinical' TTP state.
The Association Between Human Leukocyte Antigens and ITP, TTP, and HIT.
The combined roles of ADAMTS13 and VWF in murine models of TTP, endotoxemia, and thrombosis.
The D173G mutation in ADAMTS-13 causes a severe form of congenital thrombotic thrombocytopenic purpura. A clinical, biochemical andin silico study.
The features of acquired thrombotic thrombocytopenic purpura occurring at advanced age.
The Importance of the Full Blood Count in Cerebral Ischemia: A Review of 609 Consecutive Young Patients with Stroke and Transient Ischemic Attacks.
The role of ADAMTS-13 in the coagulopathy of sepsis.
Therapeutic plasma exchange in thrombotic thrombocytopenic purpura.
Thrombocytopenia and severe hyperbilirubinemia in the neonatal period secondary to congenital thrombotic thrombocytopenic purpura and ADAMTS13 deficiency.
Thrombotic microangiopathy in malignant hypertension and hemolytic uremic syndrome (HUS)/ thrombotic thrombocytopenic purpura (TTP): can we differentiate one from the other?
Thrombotic thrombocytopenic purpura and other thrombotic microangiopathic hemolytic anemias: Diagnosis and classification.
Thrombotic thrombocytopenic purpura associated to dual checkpoint inhibitor therapy for metastatic melanoma.
Thrombotic thrombocytopenic purpura directly linked with ADAMTS13 inhibition in the baboon (Papio ursinus).
Thrombotic thrombocytopenic purpura in a newborn.
Thrombotic thrombocytopenic purpura relapse induced by acute hepatitis E transmitted by cryosupernatant plasma and successfully controlled with ribavirin.
Thrombotic Thrombocytopenic Purpura with Severe ADAMTS-13 Deficiency in a Patient with Antiphospholipid Antibodies and Charcot-Marie-Tooth Disease.
Thrombotic thrombocytopenic purpura: a moving target.
Thrombotic thrombocytopenic purpura: diagnostic criteria, clinical features, and long-term outcomes from 1995 through 2015.
Ticlopidine- and clopidogrel-associated thrombotic thrombocytopenic purpura (TTP): review of clinical, laboratory, epidemiological, and pharmacovigilance findings (1989-2008).
Two novel mutations in ADAMTS13 in a Chinese boy with congenital thrombocytopenic purpura: a case report.
Von Willebrand factor, ADAMTS13, and thrombotic thrombocytopenic purpura.
Von Willebrand factor-cleaving protease (ADAMTS-13) activity in thrombotic microangiopathies: diagnostic experience 2001/2002 of a single research laboratory.
Von Willebrand factor-cleaving protease (ADAMTS13) in thrombocytopenic disorders: a severely deficient activity is specific for thrombotic thrombocytopenic purpura.
What's new in the diagnosis and pathophysiology of thrombotic thrombocytopenic purpura.
[A CASE REPORT OF THROMBOTIC THROMBOCYTOPENIC PURPURA (TTP) IN A BREAST CANCER PATIENT].
[Activity loss of Von Willebrand factor cleaving protein (ADAMTS-13) is diagnostic for primary and pregnancy-related thrombotic thrombocytopenic purpura]
[ADAMTS13, von Willebrand factor specific cleaving protease].
[Analysis of five children with acquired thrombotic thrombocytopenic purpura].
[Clinical Analysis of 12 cases of Systemic Lupus Erythematosus Associated with Thrombotic Thrombocytopenic Purpura].
[Clopidogrel-associated thrombotic thrombocytopenic purpura].
[Diagnosis and treatment of thrombotic thrombocytopenic purpura].
[Exacerbation of cranial nerurological symptoms by platelet transfusion before the diagnosis of thrombotic thrombocytopenic purpura].
[Frontline clinical practice for thrombotic thrombocytopenic purpura].
[Measurement of plasma von Willebrand factor cleaving protease in patients with varied thrombotic microangiopathy]
[Thrombotic thrombocytopenic purpura during pregnancy refractory to plasma exchange and rituximab].
Thromboinflammation
Insights Into Immunothrombosis: The Interplay Among Neutrophil Extracellular Trap, von Willebrand Factor, and ADAMTS13.
The Role of von Willebrand Factor in Vascular Inflammation: From Pathogenesis to Targeted Therapy.
Thrombophilia
ADAMTS13 and Von Willebrand factor in patients undergoing hemodialysis.
Association between ADAMTS13 activity-VWF antigen imbalance and the therapeutic effect of HAIC in patients with hepatocellular carcinoma.
Association between Von Willebrand factor, disintegrin and metalloproteinase with thrombospondin type 1 motif member 13, d-Dimer and cystatin C levels with retinopathy in type 1 diabetes mellitus.
Hypercoagulability and cardiovascular disease in diabetic nephropathy.
Increased von Willebrand factor over decreased ADAMTS-13 activity is associated with poor prognosis in patients with advanced non-small-cell lung cancer.
P265. Decreased plasma ADAMTS13 antigen and ADAMTS13 activity as a risk factor for hypercoagulability in patients with ulcerative colitis.
Thrombotic microangiopathy in patients with phosphatidylserine dependent antiprothrombin antibodies and antiphospholipid syndrome.
von Willebrand factor increases in experimental cerebral malaria but is not essential for late-stage pathogenesis in mice.
Von Willebrand Factor, ADAMTS13 and D-Dimer Are Correlated with Different Levels of Nephropathy in Type 1 Diabetes Mellitus.
[THE EFFECT OF VITAMIN D ON THE EXPRESSION OF ADAMTS13 IN CULTURED ENDOTHELIAL CELLS EXPOSED TO A DIABETIC-LIKE ENVIRONMENT].
Thrombosis
A mechanistic approach to the diagnosis and management of atypical hemolytic uremic syndrome.
A model for single-substrate trimolecular enzymatic kinetics.
A new name in thrombosis, ADAMTS13.
A rapid test for the diagnosis of thrombotic thrombocytopenic purpura using surface enhanced laser desorption/ionization time-of-flight (SELDI-TOF)-mass spectrometry.
ADAMTS-13 and von Willebrand factor: a dynamic duo.
ADAMTS-13 attenuates thrombus formation on type I collagen surface and disrupted plaques under flow conditions.
ADAMTS-13 in the Diagnosis and Management of Thrombotic Microangiopathies.
ADAMTS-13, von Willebrand factor and related parameters in severe sepsis and septic shock.
ADAMTS13 activity is decreased in a septic porcine model. Significance for glomerular thrombus deposition.
ADAMTS13 activity to antigen ratio in physiological and pathological conditions associated with an increased risk of thrombosis.
ADAMTS13 activity, high VWF and FVIII levels in the pathogenesis of deep vein thrombosis.
ADAMTS13 ameliorates inflammatory responses in experimental autoimmune encephalomyelitis.
ADAMTS13 and microvascular thrombosis.
ADAMTS13 exerts a thrombolytic effect in microcirculation.
ADAMTS13 gene deletion aggravates ischemic brain damage: a possible neuroprotective role of ADAMTS13 by ameliorating postischemic hypoperfusion.
ADAMTS13 gene variants and function in women with preeclampsia: a population- based nested case- control study from the HUNT Study.
ADAMTS13 reduces vascular inflammation and the development of early atherosclerosis in mice.
ADAMTS13 regulation of VWF multimer distribution in severe COVID-19.
ADAMTS13 Retards Progression of Diabetic Nephropathy by Inhibiting Intrarenal Thrombosis in Mice.
ADAMTS13: a new link between thrombosis and inflammation.
ADAMTS13: An Emerging Target in Stroke Therapy.
ADAMTS13: more than a regulator of thrombosis.
AFM Imaging Reveals Multiple Conformational States of ADAMTS13.
Allosteric activation of ADAMTS13 by von Willebrand factor.
Amino acid residues Arg(659), Arg(660), and Tyr(661) in the spacer domain of ADAMTS13 are critical for cleavage of von Willebrand factor.
anti-ADAMTS13 autoantibodies in patients with thrombotic thrombocytopenic purpura.
Association of a disintegrin and metalloproteinase with a thrombospondin type 1 motif member 13 polymorphisms with severity of coronary stenosis in type 2 diabetes mellitus.
Association of the von Willebrand Factor-ADAMTS13 Ratio With Incident Cardiovascular Events in Patients With Peripheral Arterial Disease.
Bench-to-bedside review: thrombocytopenia-associated multiple organ failure--a newly appreciated syndrome in the critically ill.
Binding of platelet glycoprotein Ibalpha to von Willebrand factor domain A1 stimulates the cleavage of the adjacent domain A2 by ADAMTS13.
Binding of von Willebrand factor cleaving protease ADAMTS13 to Lys-plasmin(ogen).
Carboxyl terminus of ADAMTS13 directly inhibits platelet aggregation and ultra large von Willebrand factor string formation under flow in a free-thiol-dependent manner.
Changes in plasma von Willebrand factor and ADAMTS13 levels associated with left atrial remodeling in atrial fibrillation.
Changes in von Willebrand factor and ADAMTS-13 in patients following arthroplasty.
Characterization of the interactions of ADAMTS13 CUB1 domain to WT- and GOF-Spacer domain by molecular dynamics simulation.
Cilostazol down-regulates the height of mural platelet thrombi formed under a high-shear rate flow in the absence of ADAMTS13 activity.
Clinical application of a rapid method using agarose gel electrophoresis and Western blotting to evaluate von Willebrand factor protease activity.
Complement activation: the missing link between ADAMTS-13 deficiency and microvascular thrombosis of thrombotic microangiopathies.
Conformational plasticity of ADAMTS13 in hemostasis and autoimmunity.
Conformational quiescence of ADAMTS13 prevents proteolytic promiscuity.
Congenital ADAMTS-13 deficiency presenting as life-threatening thrombosis during pregnancy.
Correction of murine ADAMTS13 deficiency by hematopoietic progenitor cell-mediated gene therapy.
Correction to: Transfusion of Platelets Loaded With Recombinant ADAMTS13 (A Disintegrin and Metalloprotease With Thrombospondin Type 1 Repeats-13) Is Efficacious for Inhibiting Arterial Thrombosis Associated With Thrombotic Thrombocytopenic Purpura.
Corticosteroids and rituximab as adjunctive treatments for thrombotic thrombocytopenic purpura.
Crystal structures of the noncatalytic domains of ADAMTS13 reveal multiple discontinuous exosites for von Willebrand factor.
Current concepts in thrombotic thrombocytopenic purpura.
Degradation of Circulating von Willebrand Factor and Its Regulator ADAMTS13 Implicates Secreted Bacillus anthracis Metalloproteases in Anthrax Consumptive Coagulopathy.
Destabilization of the von Willebrand factor A2 domain under oxidizing conditions investigated by molecular dynamics simulations.
Detection of von Willebrand factor-cleaving protease (ADAMTS-13) in human platelets.
Development of a Hyperactive Primary Hemostatic System During Off-Pump Lung Transplantation Resulting From an Unbalance Between von Willebrand Factor and Its Cleaving Protease ADAMTS13.
Effect of ADAMTS-13 on cerebrovascular microthrombosis and neuronal injury after experimental subarachnoid hemorrhage.
Effect of recombinant ADAMTS13 on microthrombosis and brain injury after experimental subarachnoid hemorrhage.
Effects of naturally occurring mutations in CUB-1 domain on synthesis, stability, and activity of ADAMTS-13.
Efficacy of rituximab in acute refractory or chronic relapsing non-familial idiopathic thrombotic thrombocytopenic purpura: a systematic review with pooled data analysis.
Elevated preoperative von Willebrand factor is associated with perioperative thrombosis in infants and neonates with congenital heart disease.
Endothelial CD40 Mediates Microvascular von Willebrand Factor-Dependent Platelet Adhesion Inducing Inflammatory Venothrombosis in ADAMTS13 Knockout Mice.
Enhanced activity of an ADAMTS-13 variant (R568K/F592Y/R660K/Y661F/Y665F) against platelet agglutination in vitro and in a murine model of acute ischemic stroke.
Escherichia coli-derived von Willebrand factor-A2 domain fluorescence/Förster resonance energy transfer proteins that quantify ADAMTS13 activity.
Essential Domains of ADAMTS13 Metalloprotease Required for Modulation of Arterial Thrombosis.
Establishment of the WHO 1st International Standard ADAMTS13, plasma (12/252): communication from the SSC of the ISTH.
Exosite interactions contribute to tension-induced cleavage of von Willebrand factor by the antithrombotic ADAMTS13 metalloprotease.
Formation and Resolution of Pial Microvascular Thrombosis in a Mouse Model of Thrombotic Thrombocytopenic Purpura.
Functional imaging of shear-dependent activity of ADAMTS13 in regulating mural thrombus growth under whole blood flow conditions.
Genetic ablation of Adamts13 gene dramatically accelerates the formation of early atherosclerosis in a murine model.
High levels of von Willebrand factor and low levels of its cleaving protease, ADAMTS13, are associated with stroke in young HIV-infected patients.
High titer of ADAMTS13 inhibitor associated with thrombotic microangiopathy of the gut and skeletal muscle after allogeneic hematopoietic stem cell transplantation.
Highly elevated plasma level of von Willebrand factor accelerates the formation of platelet thrombus under high shear stress in plasma with deficient ADAMTS13 activity.
Identification of ADAMTS13 peptide sequences binding to von Willebrand factor.
In silico features of ADAMTS13 contributing to plasmatic ADAMTS13 levels in neonates with congenital heart disease.
In-vitro and in-vivo consequences of mutations in the von Willebrand factor cleaving protease ADAMTS13 in thrombotic thrombocytopenic purpura.
Increased ADAMTS13 activity in patients with venous thromboembolism.
Increased dipeptidyl peptidase-4 accelerates chronic stress-related thrombosis in a mouse carotid artery model.
Increased VWF and Decreased ADAMTS-13 in COVID-19: Creating a Milieu for (Micro)Thrombosis.
Inflammation, von Willebrand factor, and ADAMTS13.
Influence of Personality, Resilience and Life Conditions on Depression and Anxiety in 104 Patients Having Survived Acute Autoimmune Thrombotic Thrombocytopenic Purpura.
Intensive plasma exchange increases a disintegrin and metalloprotease with thrombospondin motifs-13 activity and reverses organ dysfunction in children with thrombocytopenia-associated multiple organ failure.
Intensive plasma exchange increases ADAMTS-13 activity and reverses organ dysfunction in children with thrombocytopenia-associated multiple organ failure.
Interaction between Multimeric von Willebrand Factor and Complement: A Fresh Look to the Pathophysiology of Microvascular Thrombosis.
Interplay between ADAMTS13 and von Willebrand factor in inherited and acquired thrombotic microangiopathies.
Inverse correlations between serum ADAMTS13 levels and systolic blood pressure, pulse pressure, and serum C-reactive protein levels observed at a general health examination in a Japanese population: A cross-sectional study.
Inverse Regulation of Confluence-Dependent ADAMTS13 and von Willebrand Factor Expression in Human Endothelial Cells.
Invited commentary to: ADAMTS13 deficiency is associated with abnormal distribution of von Willebrand factor multimers in patients with COVID-19 by Tiffany Pascreau et al. Letter to the Editors-in-Chief, Thrombosis Research.
Involvement of the ADAMTS13-VWF axis in acute Kawasaki disease and effects of intravenous immunoglobulin.
Laboratory testing for ADAMTS13: Utility for TTP diagnosis/exclusion and beyond.
Levels and activities of von Willebrand factor and metalloproteinase with thrombospondin type-1 motif, number 13 in inflammatory bowel diseases.
Lower levels of ADAMTS13 are associated with cardiovascular disease in young patients.
Macrovascular thrombosis in critically ill patients with thrombotic micro-angiopathies.
Mechanisms of microvascular thrombosis in thrombotic thrombocytopenic purpura.
Modifier genes for disorders of thrombosis and hemostasis.
Molecular biology of ADAMTS13 and diagnostic utility of ADAMTS13 proteolytic activity and inhibitor assays.
Molecular mechanisms in thrombotic thrombocytopenic purpura.
Multi-step binding of ADAMTS13 to VWF.
Mutations and Common Polymorphisms in ADAMTS13 and vWF Genes Responsible for Increasing Risk of Thrombosis.
Neutrophil Protease Cleavage of Von Willebrand Factor in Glomeruli - An Anti-thrombotic Mechanism in the Kidney.
Next-Generation Sequencing and In Vitro Expression Study of ADAMTS13 Single Nucleotide Variants in Deep Vein Thrombosis.
Next-generation sequencing study finds an excess of rare, coding single-nucleotide variants of ADAMTS13 in patients with deep vein thrombosis.
Novel mutations in ADAMTS13 CUB domains cause abnormal pre-mRNA splicing and defective secretion of ADAMTS13.
Pathogenesis of thrombotic thrombocytopenic purpura.
Pathophysiology of thrombotic thrombocytopenic purpura.
Phylogenetic and functional analysis of ADAMTS13 identifies highly conserved domains essential for allosteric regulation.
Plasma ADAMTS13 activity and von Willebrand factor antigen and activity in patients with subarachnoid haemorrhage.
Plasma ADAMTS13 activity may predict early adverse events in living donor liver transplantation: observations in 3 cases.
Plasma ADAMTS13, von Willebrand Factor (VWF), and VWF Propeptide Profiles in Patients With Connective Tissue Diseases and Antiphospholipid Syndrome.
Plasma Proteomics of COVID-19 Associated Cardiovascular Complications: Implications for Pathophysiology and Therapeutics.
Platelet factor 4 inhibits ADAMTS13 activity and regulates the multimeric distribution of von Willebrand factor.
Platelet-delivered ADAMTS13 inhibits arterial thrombosis and prevents thrombotic thrombocytopenic purpura in murine models.
Predicting pathological von Willebrand factor unraveling in elongational flow.
Presence of portal vein thrombosis in liver cirrhosis is strongly associated with low levels of ADAMTS-13: a pilot study.
Rapid Restoration of Thrombus Formation and High-Molecular-Weight von Willebrand Factor Multimers in Patients with Severe Aortic Stenosis After Valve Replacement.
Rare Variants in the ADAMTS13 Von Willebrand Factor-Binding Domain Contribute to Pediatric Stroke.
Recent advances in thrombotic thrombocytopenic purpura.
Recombinant thrombomodulin for secondary thrombotic thrombocytopenic purpura.
Reduced ADAMTS13 levels in patients with acute and chronic cerebrovascular disease.
Reference range for ADAMTS13 antigen, activity and anti-ADAMTS13 antibody in the healthy adult Singapore population.
Relative Deficiency of Plasma A Disintegrin and Metalloprotease with Thrombospondin Type 1 Repeats 13 Activity and Elevation of Human Neutrophil Peptides in Patients with Traumatic Brain Injury.
Secretion of von Willebrand Factor and Suppression of ADAMTS-13 Activity by Markedly High Concentration of Ferritin.
Serial changes in von Willebrand factor-cleaving protease (ADAMTS13) and prognosis after acute myocardial infarction.
Serum ADAMTS-13 Levels as an Indicator of Portal Vein Thrombosis.
Shear stress and platelet-induced tensile forces regulate ADAMTS13-localization within the platelet thrombus.
Shear stress and von Willebrand factor in health and disease.
Shear-Induced Unfolding and Enzymatic Cleavage of Full-Length VWF Multimers.
Significance of plasma von Willebrand factor level and von Willebrand factor-cleaving protease activity in patients with chronic renal diseases.
Size regulation of von Willebrand factor-mediated platelet thrombi by ADAMTS13 in flowing blood.
SNPs in ADAMTS13.
Structure-function and regulation of ADAMTS-13 protease.
Systemic antithrombotic effects of ADAMTS13.
Ten candidate ADAMTS13 mutations in six French families with congenital thrombotic thrombocytopenic purpura (Upshaw-Schulman syndrome).
The combined roles of ADAMTS13 and VWF in murine models of TTP, endotoxemia, and thrombosis.
The distal carboxyl-terminal domains of ADAMTS13 are required for regulation of in vivo thrombus formation.
The expression of ADAMTS13 in human microvascular endothelial cells.
The function of ADAMTS13 in thrombogenesis in vivo: insights from mutant mice.
The kidney in thrombotic thrombocytopenic purpura.
The Plasma Levels of ADAMTS-13, von Willebrand Factor, VWFpp, and Fibrin-Related Markers in Patients With Systemic Sclerosis Having Thrombosis.
The proximal carboxyl-terminal domains of ADAMTS13 determine substrate specificity and are all required for cleavage of von Willebrand factor.
The role of ADAMTS-13 and von Willebrand factor in cancer patients: Results from the Vienna Cancer and Thrombosis Study.
The role of ADAMTS13 testing in the diagnosis and management of thrombotic microangiopathies and thrombosis.
The Role of von Willebrand Factor, ADAMTS13, and Cerebral Artery Thrombus Composition in Patient Outcome Following Mechanical Thrombectomy for Acute Ischemic Stroke.
Therapeutic efficacy of the platelet glycoprotein Ib antagonist anfibatide in murine models of thrombotic thrombocytopenic purpura.
Thrombospondin-1 controls vascular platelet recruitment and thrombus adherence in mice by protecting (sub)endothelial VWF from cleavage by ADAMTS13.
Thrombotic microangiopathies and the linkage between von Willebrand factor and the alternative complement pathway.
Thrombotic Thrombocytopenic Purpura and Anti-Thrombotic Therapy Targeted to Von Willebrand Factor.
Thrombotic thrombocytopenic purpura presenting with pathologic fracture: a case report.
Thrombotic thrombocytopenic purpura with unusual 33 recurrences: a case report.
Thrombotic thrombocytopenic purpura: a moving target.
Transfusion of Platelets Loaded With Recombinant ADAMTS13 (A Disintegrin and Metalloprotease With Thrombospondin Type 1 Repeats-13) Is Efficacious for Inhibiting Arterial Thrombosis Associated With Thrombotic Thrombocytopenic Purpura.
Update on ADAMTS13 and VWF in cardiovascular and hematological disorders.
Update on thrombotic thrombocytopenic purpura.
Use of a mouse model to elucidate the phenotypic effects of the von willebrand factor cleavage mutants, y1605a/m1606a and r1597w.
Validation of PLASMIC score and follow-up data in a cohort of patients with suspected microangiopathies from Southern Italy.
Variations among normal individuals in the cleavage of endothelial-derived ultra-large von Willebrand factor under flow.
Von Willebrand Factor Adhesive Activity and ADAMTS13 Protease Activity in HIV-1-Infected Men.
Von Willebrand factor and ADAMTS13 in arterial thrombosis: a systematic review and meta-analysis.
Von Willebrand factor and thrombosis.
von Willebrand Factor, ADAMTS13 Activity, and Decline in Kidney Function: A Population-Based Cohort Study.
von Willebrand Factor, Shear Stress, and ADAMTS13 in Hemostasis and Thrombosis.
von Willebrand factor, von Willebrand factor-cleaving protease, and shear stress.
von Willebrand factor-cleaving protease ADAMTS13 reduces ischemic brain injury in experimental stroke.
Von Willebrand factor: molecular size and functional activity.
Von Willebrand factor:antigen and ADAMTS-13 level, but not soluble P-selectin, are risk factors for the first asymptomatic deep vein thrombosis in cancer patients undergoing chemotherapy.
VWF excess and ADAMTS13 deficiency: a unifying pathomechanism linking inflammation to thrombosis in DIC, malaria, and TTP.
Whole-exome sequencing detects mutations in pediatric patients with atypical hemolytic uremic syndrome in Taiwan.
[Changes of ADAMTS13 Activity and TSP1 Level in Patients with Hematologic Malignancies].
[Influence of HSCT Preconditioning on ADAMTS-13 Activity and vWF level and Its Clinical Significance].
[Stable expression and characterization of the von Willebrand factor cleaving protease]
[Thrombosis and ADAMTS13 exclusively produced in the liver]
[Thrombotic microangiopathy]
[Thrombotic Thrombocytopenic Purpura --Pathophysiology and Assays of ADAMTS13 Activity].
Thrombotic Microangiopathies
A Case of Recurrent Thrombotic Microangiopathy Caused by Hypertensive Urgency.
A Case of Thrombotic Thrombocytopenia Purpura Associated with Systemic Lupus Erythematosus: Diagnostic Utility of ADAMTS-13 Activity.
A critical evaluation of caplacizumab for the treatment of acquired thrombotic thrombocytopenic purpura.
A human monoclonal antibody against the distal carboxyl terminus of ADAMTS-13 modulates its susceptibility to an inhibitor in thrombotic thrombocytopenic purpura.
A mechanistic approach to the diagnosis and management of atypical hemolytic uremic syndrome.
A mild deficiency of ADAMTS13 is associated with severity in COVID-19: comparison of the coagulation profile in critically and noncritically ill patients.
A multi-center evaluation of TECHNOSCREEN® ADAMTS-13 activity assay as a screening tool for detecting deficiency of ADAMTS-13.
A new mouse model mimicking thrombotic thrombocytopenic purpura: correction of symptoms by recombinant human ADAMTS13.
A novel association of acquired ADAMTS13 inhibitor and acute dengue virus infection.
A Novel Case of Leflunomide-Induced Thrombotic Thrombocytopenic Purpura.
A novel quantitative hemolytic assay coupled with restriction fragment length polymorphisms analysis enabled early diagnosis of atypical hemolytic uremic syndrome and identified unique predisposing mutations in Japan.
A patient with SLE-associated thrombotic microangiopathy and non-neutralizing antibodies against ADAMTS13.
Acquired thrombotic thrombocytopenic purpura due to antibody-mediated ADAMTS13 deficiency precipitated by a localized Castleman's disease: A case report.
ADAMTS-13 deficiency: can it cause chronic renal failure?
ADAMTS-13 in the Diagnosis and Management of Thrombotic Microangiopathies.
ADAMTS-13 metalloprotease abnormalities in systemic lupus erythematosus: is there a correlation with disease status?
ADAMTS13 activity levels in patients with human immunodeficiency virus-associated thrombotic microangiopathy and profound CD4 deficiency.
ADAMTS13 activity to von Willebrand factor antigen ratio predicts acute kidney injury in patients with COVID-19: Evidence of SARS-CoV-2 induced secondary thrombotic microangiopathy.
ADAMTS13 and TTP.
ADAMTS13 and von Willebrand factor in thrombotic thrombocytopenic purpura.
ADAMTS13 autoantibodies in patients with thrombotic microangiopathies and other immunomediated diseases.
ADAMTS13 Endopeptidase Protects against Vascular Endothelial Growth Factor Inhibitor-Induced Thrombotic Microangiopathy.
ADAMTS13 missense variants associated with defective activity and secretion of ADAMTS13 in a patient with non-cirrhotic portal hypertension.
ADAMTS13 related markers and von Willebrand factor in plasma from patients with thrombotic microangiopathy (TMA).
ADAMTS13 test and/or PLASMIC clinical score in management of acquired thrombotic thrombocytopenic purpura: a cost-effective analysis.
ADAMTS13 Testing Methodologies and Thrombotic Thrombocytopenic Purpura (TTP): Conflicting Results Can Pose a Clinical Dilemma.
ADAMTS13--more than just TMA and TTP.
ADAMTS13-specific circulating immune complexes as potential predictors of relapse in patients with acquired thrombotic thrombocytopenic purpura.
Advances in the pathogenesis, diagnosis, and treatment of thrombotic thrombocytopenic purpura.
Annual incidence and severity of acute episodes in hereditary thrombotic thrombocytopenic purpura.
ASFA Category IV becomes Category I: Idiopathic thrombotic thrombocytopenic purpura in a patient with presumed gemcitabine-induced thrombotic microangiopathy.
Assembly and activation of alternative complement components on endothelial cell-anchored ultra-large von Willebrand factor links complement and hemostasis-thrombosis.
Assessment and Monitoring of Patients with Immune-Mediated Thrombotic Thrombocytopenic Purpura (iTTP): Strategies to Improve Outcomes.
Association between thrombotic microangiopathy and reduced ADAMTS13 activity in malignant hypertension.
Attending rounds: microangiopathic hemolytic anemia with renal insufficiency.
Atypical Hemolytic Uremic Syndrome Presenting as Acute Heart Failure-A Rare Presentation: Diagnosis Supported by Skin Biopsy.
Atypical presentation of hepatic visceral larva migrans mimicking cancer and associated with ADAMTS13 deficiency-mediated thrombotic microangiopathy: A first report from Reunion Island.
Autoimmune thrombotic microangiopathy: advances in pathogenesis, diagnosis, and management.
Availability of an ADAMTS13 assay with rapid turnaround time may avoid interhospital transfer in patients with thrombotic microangiopathy.
Behavior of ADAMTS13 and Von Willebrand factor levels in patients after living donor liver transplantation.
Cancer-related thrombotic microangiopathy secondary to Von Willebrand factor-cleaving protease deficiency.
Characteristics and Outcomes of Patients with Systemic Lupus Erythematosus-associated Thrombotic Microangiopathy, and Their Acquired ADAMTS13 Inhibitor Profiles.
Child-onset and adolescent-onset acquired thrombotic thrombocytopenic purpura with severe ADAMTS13 deficiency: a cohort study of the French national registry for thrombotic microangiopathy.
Clinical and laboratory diagnosis of TTP: an integrated approach.
Clinical and Laboratory Features of Patients with Acquired Thrombotic Thrombocytopenic Purpura: Fourteen Years of the Milan TTP Registry.
Clinical characteristics and outcomes of thrombotic microangiopathy in Malaysia.
Clinical features and outcomes in patients with thrombotic microangiopathy not associated with severe ADAMTS13 deficiency.
Clinical usefulness of a functional assay for the von Willebrand factor cleaving protease (ADAMTS 13) and its inhibitor in a patient with thrombotic thrombocytopenic purpura.
Clinical utility of ADAMTS-13 testing in suspected thrombotic microangiopathy: an audit of ADAMTS-13 activity assay requests in routine practice from a tertiary hospital.
Combined study of ADAMTS13 and complement genes in the diagnosis of thrombotic microangiopathies using next-generation sequencing.
Comparison of Clinical Scoring Systems in the Management of Patients with Microangiopathic Hemolytic Anemia and Thrombocytopenia
Comparison of Rituximab originator (MabThera) to biosimilar (Truxima) in patients with immune-mediated thrombotic thrombocytopenic purpura.
Complement Activation Associated with ADAMTS13 Deficiency in Human and Murine Thrombotic Microangiopathy.
Complement activation, inflammation and relative ADAMTS13 deficiency in secondary thrombotic microangiopathies.
Complement activation: the missing link between ADAMTS-13 deficiency and microvascular thrombosis of thrombotic microangiopathies.
Complete defect in vWF-cleaving protease activity associated with increased shear-induced platelet aggregation in thrombotic microangiopathy.
Congenital thrombotic thrombocytopenic purpura associated with unilateral moyamoya disease.
Congenital thrombotic thrombocytopenic purpura caused by new compound heterozygous mutations of the ADAMTS13 gene.
Coombs Positive Thrombotic Thrombocytopenic Purpura in a Male Pediatric Patient: An Urgent Diagnostic Challenge.
Correlation between ADAMTS13 activity and neurological impairment in acute thrombotic microangiopathy patients.
COVID 19 infection associated with thrombotic thrombocytopenic purpura.
Current management and therapeutical perspectives in thrombotic thrombocytopenic purpura.
Decreased ADAMTS13 Levels in Patients after Living Donor Liver Transplantation.
Deficient activity of von Willebrand factor-cleaving protease in thrombotic thrombocytopenic purpura.
Derivation and external validation of the PLASMIC score for rapid assessment of adults with thrombotic microangiopathies: a cohort study.
Development and validation of a multivariable prediction rule for detecting a severe acquired ADAMTS13 activity deficiency in patients with thrombotic microangiopathies.
Diabetic ketoacidosis presenting with atypical hemolytic uremic syndrome associated with a variant of complement factor B in an adult: a case report.
Diagnosis and follow-up of thrombotic thrombocytopenic purpura with an automated chemiluminescent ADAMTS13 activity immunoassay.
Diagnosis of complement alternative pathway disorders.
Discrepancies between ADAMTS13 activity assays in patients with thrombotic microangiopathies.
Disseminated cerebral aspergillosis complicated by thrombotic microangiopathy.
Does Outcome/Survival of Patients With Myelodysplastic Syndromes Should Be Predicted by Reduced Levels of ADAMTS-13? Results From a Pilot Study.
Does severe ADAMTS13 deficiency in thrombotic microangiopathy rule out complement-mediated atypical hemolytic uremic syndrome.
Drug-induced thrombotic microangiopathy.
Early response to caplacizumab and rituximab after anaphylaxis to Octaplas plasma in a patient with thrombotic thrombocytopenic purpura.
Elevated plasma levels of soluble platelet glycoprotein VI (GPVI) in patients with thrombotic microangiopathy.
Epidemiology and pathophysiology of adulthood-onset thrombotic microangiopathy with severe ADAMTS13 deficiency (thrombotic thrombocytopenic purpura): a cross-sectional analysis of the French national registry for thrombotic microangiopathy.
Evaluation of a chromogenic commercial assay using VWF-73 peptide for ADAMTS13 activity measurement.
Evaluation of a rapid turn-over, fully-automated ADAMTS13 activity assay: a method comparison study.
Evaluation of assay methods to measure plasma ADAMTS13 activity in thrombotic microangiopathies.
External validation of the PLASMIC score: a clinical prediction tool for thrombotic thrombocytopenic purpura diagnosis and treatment.
Factor V Leiden: a genetic risk factor for thrombotic microangiopathy in patients with normal von Willebrand factor-cleaving protease activity.
Fatal case of chikungunya and concomitant thrombotic thrombocytopenic purpura in French Guiana during air flight medical evacuation.
FRETS-VWF73, a first fluorogenic substrate for ADAMTS13 assay.
Generation of Anti-Murine ADAMTS13 Antibodies and Their Application in a Mouse Model for Acquired Thrombotic Thrombocytopenic Purpura.
H1N1 Influenza (Swine Flu)-Associated Thrombotic Microangiopathy with a Markedly High Plasma Ratio of von Willebrand Factor to ADAMTS13.
Hematopoietic cell transplantation-associated thrombotic microangiopathy: a review of pathophysiology, diagnosis, and treatment.
Heparin cofactor II as a predictor of thrombotic microangiopathy after bone marrow transplantation.
Heparin-induced thrombocytopenia antibody and the pathogenesis of thrombotic microangiopathy after stem cell transplantation.
Heparin-induced thrombocytopenia as a cause of prolonged low platelet count in a patient with thrombotic thrombocytopenic purpura treated with plasmapheresis.
Heterogeneous pathogenic processes of thrombotic microangiopathies in patients with connective tissue diseases.
High titer of ADAMTS13 inhibitor associated with thrombotic microangiopathy of the gut and skeletal muscle after allogeneic hematopoietic stem cell transplantation.
How I treat thrombotic thrombocytopenic purpura in pregnancy.
Human immunodeficiency virus-associated thrombotic microangiopathies: clinical characteristics and outcome according to ADAMTS13 activity.
Hyperbilirubinemia interferes with ADAMTS-13 activity measurement by FRETS-VWF73 assay: diagnostic relevance in patients suffering from acute thrombotic microangiopathies.
IL-1? Down-Regulates ADAMTS-13 mRNA Expression in Cells of the Central Nervous System.
Immunochip analysis identifies novel susceptibility loci in the human leukocyte antigen region for acquired thrombotic thrombocytopenic purpura.
Impact of severe ADAMTS13 deficiency on clinical presentation and outcomes in patients with thrombotic microangiopathies: the experience of the Harvard TMA Research Collaborative.
Increased VWF and Decreased ADAMTS-13 in COVID-19: Creating a Milieu for (Micro)Thrombosis.
Indicators Differentiating Thrombotic Thrombocytopenic Purpura From Other Thrombotic Microangiopathies in a Canadian Apheresis Referral Center.
Inherited thrombotic thrombocytopenic purpura mimicking immune thrombocytopenic purpura during pregnancy: a case report.
Inherited Thrombotic Thrombocytopenic Purpura Revealed by Recurrent Strokes in a Male Adult: Case Report and Literature Review.
Inhibitors of ADAMTS13: a potential factor in the cause of thrombotic microangiopathy in a renal allograft recipient.
Insights Into Immunothrombosis: The Interplay Among Neutrophil Extracellular Trap, von Willebrand Factor, and ADAMTS13.
Intensive plasma exchange increases a disintegrin and metalloprotease with thrombospondin motifs-13 activity and reverses organ dysfunction in children with thrombocytopenia-associated multiple organ failure.
Intensive plasma exchange increases ADAMTS-13 activity and reverses organ dysfunction in children with thrombocytopenia-associated multiple organ failure.
International Council for Standardization in Haematology (ICSH) recommendations for laboratory measurement of ADAMTS13.
Interplay between ADAMTS13 and von Willebrand factor in inherited and acquired thrombotic microangiopathies.
Is factor V Leiden a risk factor for thrombotic microangiopathies without severe ADAMTS 13 deficiency?
Is it quinine TTP/HUS or quinine TMA? ADAMTS13 levels and implications for therapy.
Loss of von Willebrand factor high-molecular-weight multimers at acute phase is associated with detectable anti-ADAMTS13 IgG and neurological symptoms in acquired thrombotic thrombocytopenic purpura.
MED-TMA: A clinical decision support tool for differential diagnosis of TMA with enhanced accuracy using an ensemble method.
Methodologies and clinical utility of ADAMTS-13 activity testing.
Microangiopathic Anemia of Acute Brucellosis - is it a True TTP?
Mild to moderate reduction of a von Willebrand factor cleaving protease (ADAMTS-13) in pregnant women with HELLP microangiopathic syndrome.
Multicentric evaluation of the new HemosIL Acustar® chemiluminescence ADAMTS13 activity assay.
Multiple centre evaluation study of ADAMTS13 activity and inhibitor assays.
Natural history of thrombotic thrombocytopenic purpura and hemolytic uremic syndrome.
Neutrophil Protease Cleavage of Von Willebrand Factor in Glomeruli - An Anti-thrombotic Mechanism in the Kidney.
Novel compound heterozygote mutations (H234Q/R1206X) of the ADAMTS13 gene in an adult patient with Upshaw-Schulman syndrome showing predominant episodes of repeated acute renal failure.
Novel monoclonal antibody-based enzyme immunoassay for determining plasma levels of ADAMTS13 activity.
Novel therapeutic approaches for thrombotic thrombocytopenic purpura.
Ofatumumab for acute treatment and prophylaxis of a patient with multiple relapses of acquired thrombotic thrombocytopenic purpura.
Pathogenesis of Thrombotic Microangiopathies.
Pathogenesis of thrombotic thrombocytopenic purpura.
Patients with localized and disseminated tumors have reduced but measurable levels of ADAMTS-13 (von Willebrand factor cleaving protease).
Performance of Diagnostic Scores in Thrombotic Microangiopathy Patients in the Intensive Care Unit: A Monocentric Study.
Plasmapheresis in Sepsis-induced Thrombotic Microangiopathy: A Case Series.
Plasmin Cleavage of von Willebrand Factor as an Emergency Bypass for ADAMTS13 Deficiency in Thrombotic Microangiopathy.
Platelet-activating immune complexes identified in critically ill COVID-19 patients suspected of heparin-induced thrombocytopenia.
Post-traumatic thrombotic microangiopathy following pelvic fracture treated with transcatheter arterial embolization: a case report.
Potential impact of a delayed ADAMTS13 result in the treatment of thrombotic microangiopathy: an economic analysis.
Predictive features of severe acquired ADAMTS13 deficiency in idiopathic thrombotic microangiopathies: the French TMA reference center experience.
Presence of ADAMTS13 activity in a patient with metastatic cancer and thrombotic microangiopathy.
Prognostic and Long-term Survival of Immune Thrombotic Thrombocytopenic Purpura in older patients.
Prognostic value of anti-ADAMTS 13 antibody features (Ig isotype, titer, and inhibitory effect) in a cohort of 35 adult French patients undergoing a first episode of thrombotic microangiopathy with undetectable ADAMTS 13 activity.
Prospective evaluation of ADAMTS-13 and von Willebrand factor multimers in cardiac surgery.
Rapid quantitative assay of ADAMTS13 activity on an automated coagulation analyzer: clinical applications and comparison with immunoblot method.
Recent advances in thrombotic thrombocytopenic purpura.
Recommendations for the diagnosis and treatment of patients with thrombotic thrombocytopenic purpura.
Redefining outcomes in immune TTP: an international working group consensus report.
Reduced von Willebrand factor-cleaving protease levels in secondary thrombotic microangiopathies and other diseases.
Reference range for ADAMTS13 antigen, activity and anti-ADAMTS13 antibody in the healthy adult Singapore population.
Refractory thrombotic thrombocytopenic purpura associated with oral contraceptives and factor V Leiden: a case report.
Relation between ADAMTS13 activity and ADAMTS13 antigen levels in healthy donors and patients with thrombotic microangiopathies (TMA).
Relevance of ADAMTS13 to liver transplantation and surgery.
Renal thrombotic microangiopathy and pulmonary arterial hypertension in a patient with late-onset cobalamin C deficiency.
Rituximab in a child with autoimmune thrombotic thrombocytopenic purpura refractory to plasma exchange.
Role of ADAMTS13 in the management of thrombotic microangiopathies including thrombotic thrombocytopenic purpura (TTP).
Safety and efficacy of cryoprecipitate-poor plasma as a replacement fluid for therapeutic plasma exchange in thrombotic thrombocytopenic purpura: A single center retrospective evaluation.
Schistocytic anaemia, severe thrombocytopenia, and renal dysfunction: thrombotic microangiopathy due to severe acquired ADAMTS-13 deficiency. Case 2.
Seasonal distribution of severe ADAMTS13 deficient idiopathic thrombotic thrombocytopenic purpura.
Second international collaborative study evaluating performance characteristics of methods measuring the von Willebrand factor cleaving protease (ADAMTS-13).
Secondary thrombotic microangiopathy with severely reduced ADAMTS13 activity in a patient with Capnocytophaga canimorsus sepsis: a case report.
Severe ADAMTS13 deficiency in adult idiopathic thrombotic microangiopathies defines a subset of patients characterized by various autoimmune manifestations, lower platelet count, and mild renal involvement.
Severe deficiency of VWF-cleaving protease (ADAMTS13) activity defines a distinct population of thrombotic microangiopathy patients.
Specific von Willebrand factor-cleaving protease in thrombotic microangiopathies: a study of 111 cases.
Structure and Proteolytic Properties of ADAMTS13, A Metalloprotease Involved in the Pathogenesis of Thrombotic Microangiopathies.
Successful treatment of TAFRO syndrome, a variant type of multicentric Castleman disease with thrombotic microangiopathy, with anti-IL-6 receptor antibody and steroids.
Synergistic effects of ADAMTS13 deficiency and complement activation in pathogenesis of thrombotic microangiopathy.
Systemic lupus erythematosus presenting as thrombotic thrombocytopaenic purpura in a child: a diagnostic challenge.
Systemic lupus erythematosus with ADAMTS13 inhibitor-negative thrombotic microangiopathy treated with combination of mycophenolate mofetil, plasma exchange and steroid.
The "cutting" edge: von Willebrand factor-cleaving protease activity in thrombotic microangiopathies.
The activity of the von Willebrand factor cleaving protease ADAMTS-13 in newborn infants.
The D173G mutation in ADAMTS-13 causes a severe form of congenital thrombotic thrombocytopenic purpura. A clinical, biochemical andin silico study.
The Differential Diagnosis and Treatment of Thrombotic Microangiopathies.
The HLA Variant rs6903608 Is Associated with Disease Onset and Relapse of Immune-Mediated Thrombotic Thrombocytopenic Purpura in Caucasians.
The potential therapeutic benefit of targeting ADAMTS13 activity.
The role of ADAMTS-13 activity and complement mutational analysis in differentiating acute thrombotic microangiopathies.
The role of ADAMTS-13 in the coagulopathy of sepsis.
The role of ADAMTS13 testing in the diagnosis and management of thrombotic microangiopathies and thrombosis.
The use of ADAMTS13 activity, platelet count, and serum creatinine to differentiate acquired thrombotic thrombocytopenic purpura from other thrombotic microangiopathies.
The utility of ADAMTS13 in differentiating TTP from other acute thrombotic microangiopathies: results from the UK TTP Registry.
The utility of patient characteristics in predicting severe ADAMTS13 deficiency and response to plasma exchange.
Thrombocytopenia in pregnancy.
Thrombotic microangiopathic hemolytic anemia with reduction of ADAMTS13 activity: initial manifestation of childhood-onset systemic lupus erythematosus.
Thrombotic microangiopathies and the linkage between von Willebrand factor and the alternative complement pathway.
Thrombotic microangiopathies, thrombotic thrombocytopenic purpura, and ADAMTS-13.
Thrombotic microangiopathies: role of ADAMTS-13.
Thrombotic microangiopathy (TTP and HUS): advances in differentiation and diagnosis.
Thrombotic microangiopathy due to acquired ADAMTS13 deficiency in a patient receiving interferon-beta treatment for multiple sclerosis.
Thrombotic microangiopathy due to malignant hypertension complicated with late-onset bleeding after renal biopsy.
Thrombotic microangiopathy in a 17-year-old patient: TTP, HUS or a bit of both?
Thrombotic microangiopathy in malignant hypertension and hemolytic uremic syndrome (HUS)/ thrombotic thrombocytopenic purpura (TTP): can we differentiate one from the other?
Thrombotic microangiopathy with acquired deficiency in ADAMTS 13 activity in lung transplant recipients.
Thrombotic Microangiopathy: Current Knowledge and Outcomes With Plasma Exchange.
Thrombotic thrombocytopenic purpura as a rare cause of anemia with thrombocytopenia in childhood: report of 2 cases.
Thrombotic Thrombocytopenic Purpura in a Child Treated for Acute Lymphoblastic Leukemia: Case Report and Review of Literature.
Thrombotic Thrombocytopenic Purpura in a ChildWithSystemic Lupus Erythematosus.
Thrombotic thrombocytopenic purpura--a syndrome caused by multiple pathogenetic mechanisms.
Thrombotic thrombocytopenic purpura-what is new?
Thrombotic thrombocytopenic purpura.
Thrombotic thrombocytopenic purpura: Toward targeted therapy and precision medicine.
Treatment of thrombotic thrombocytopenic purpura.
Treatment with or without plasma exchange for patients with acquired thrombotic microangiopathy not associated with severe ADAMTS13 deficiency: a propensity score-matched study.
Twice-daily therapeutical plasma exchange-based salvage therapy in severe autoimmune thrombotic thrombocytopenic purpura: the French TMA Reference Center experience.
Update on ADAMTS13 and VWF in cardiovascular and hematological disorders.
Validation of PLASMIC score and follow-up data in a cohort of patients with suspected microangiopathies from Southern Italy.
Validation of the PLASMIC score, a clinical prediction tool for thrombotic thrombocytopenic purpura diagnosis, in Chinese patients.
Variations in the ratio between von Willebrand factor and its cleaving protease during systemic inflammation and association with severity and prognosis of organ failure.
von Willebrand factor and thrombotic thrombocytopenic purpura.
von Willebrand factor-cleaving protease (ADAMTS-13) activity determination in the diagnosis of thrombotic microangiopathies: the Swiss experience.
Von Willebrand factor-cleaving protease (ADAMTS-13) activity in thrombotic microangiopathies: diagnostic experience 2001/2002 of a single research laboratory.
von Willebrand factor-cleaving protease (ADAMTS13) in the course of stem cell transplantation.
Von Willebrand factor-cleaving protease (ADAMTS13) in thrombocytopenic disorders: a severely deficient activity is specific for thrombotic thrombocytopenic purpura.
Von Willebrand factor-cleaving protease activity and proteolysis of von Willebrand factor in bone marrow transplant-associated thrombotic microangiopathy.
Von Willebrand factor-cleaving protease activity in thrombotic microangiopathy: first report from iran.
VWF excess and ADAMTS13 deficiency: a unifying pathomechanism linking inflammation to thrombosis in DIC, malaria, and TTP.
What's new in the diagnosis and pathophysiology of thrombotic thrombocytopenic purpura.
[ADAMTS13, von Willebrand factor specific cleaving protease].
[Clinical observation of a patient with thrombotic thrombocytopenic purpura with renal and intestinal lesions].
[Development of a novel ADAMTS13 activity assay for diagnosis of TMA and criteria for judgment of platelet transfusion]
[Diagnosis and treatment of thrombotic thrombocytopenic purpura].
[Expression of the metalloproteinase domain of von Willebrand factor-cleaving protease and preparation of its McAb]
[Frontline clinical practice for thrombotic thrombocytopenic purpura].
[Measurement of plasma von Willebrand factor cleaving protease in patients with varied thrombotic microangiopathy]
[Post-transfusion acute lung injury (Trali) after plasma infusion in a patient having a constitutional thrombotic microangiopathy]
[Thrombotic microangiopathy after extracorporeal circulation : Important differential diagnosis.]
[Thrombotic microangiopathy: thrombocytopenia and hemolytic anemia]
[Thrombotic thrombocytopenic purpura: Do not ignore cardiac involvement.]
[Treatment of immune-mediated thrombotic thrombocytopenic purpura: A decisive turning point].
Thyrotoxicosis
Thrombotic thrombocytopenic purpura precipitated by thyrotoxicosis.
Toxoplasmosis
Increased expressions of ADAMTS-13 and apoptosis contribute to neuropathology during Toxoplasma gondii encephalitis in mice.
Transfusion-Related Acute Lung Injury
[Post-transfusion acute lung injury (Trali) after plasma infusion in a patient having a constitutional thrombotic microangiopathy]
Unconsciousness
Acquired thrombotic thrombocytopenic purpura with a smoldering clinical course.
Vascular Diseases
Inverse correlations between serum ADAMTS13 levels and systolic blood pressure, pulse pressure, and serum C-reactive protein levels observed at a general health examination in a Japanese population: A cross-sectional study.
Prognostic value of plasma von Willebrand factor and its cleaving protease ADAMTS13 in patients with atrial fibrillation.
Quantitative and qualitative changes of von Willebrand factor and their impact on mortality in patients with end-stage kidney disease.
Vascular System Injuries
ADAMTS13 and von Willebrand factor in thrombotic thrombocytopenic purpura.
ADAMTS13--more than just TMA and TTP.
Altered glycosylation of platelet-derived von Willebrand factor confers resistance to ADAMTS13 proteolysis.
Binding of von Willebrand factor cleaving protease ADAMTS13 to Lys-plasmin(ogen).
Characterization of the interactions of ADAMTS13 CUB1 domain to WT- and GOF-Spacer domain by molecular dynamics simulation.
Complement activation associated with ADAMTS13 deficiency may contribute to the characteristic glomerular manifestations in Upshaw-Schulman syndrome.
Conformational plasticity of ADAMTS13 in hemostasis and autoimmunity.
Detection of von Willebrand factor-cleaving protease (ADAMTS-13) in human platelets.
Exosite interactions contribute to tension-induced cleavage of von Willebrand factor by the antithrombotic ADAMTS13 metalloprotease.
Pathogenesis of thrombotic thrombocytopenic purpura: ADAMTS13 deficiency and beyond.
Platelet-delivered ADAMTS13 inhibits arterial thrombosis and prevents thrombotic thrombocytopenic purpura in murine models.
Proteolytic inactivation of ADAMTS13 by thrombin and plasmin.
Thrombogenesis and thrombotic disorders based on 'two-path unifying theory of hemostasis': philosophical, physiological, and phenotypical interpretation.
Venous Thromboembolism
ADAMTS-13 and von Willebrand factor predict venous thromboembolism in patients with cancer.
Increased ADAMTS13 activity in patients with venous thromboembolism.
Verification of the Role of ADAMTS13 in the Cardiovascular Disease Using Two-Sample Mendelian Randomization.
Venous Thrombosis
ADAMTS-13-VWF axis in sickle cell disease patients.
ADAMTS13 activity, high VWF and FVIII levels in the pathogenesis of deep vein thrombosis.
Changes in von Willebrand factor and ADAMTS-13 in patients following arthroplasty.
Influence of ADAMTS13 deficiency on venous thrombosis in mice.
Low ADAMTS13 levels are associated with venous thrombosis risk in women.
Macrovascular thrombosis in critically ill patients with thrombotic micro-angiopathies.
Next-Generation Sequencing and In Vitro Expression Study of ADAMTS13 Single Nucleotide Variants in Deep Vein Thrombosis.
Next-generation sequencing study finds an excess of rare, coding single-nucleotide variants of ADAMTS13 in patients with deep vein thrombosis.
Polymorphisms and Mutations in vWF and ADAMTS13 Genes and Their Correlation With Plasma Levels of FVIII and vWF in Patients With Deep Venous Thrombosis.
The role of von Willebrand factor in hemorrhagic and thrombotic disorders.
Von Willebrand factor:antigen and ADAMTS-13 level, but not soluble P-selectin, are risk factors for the first asymptomatic deep vein thrombosis in cancer patients undergoing chemotherapy.
Virus Diseases
A novel association of acquired ADAMTS13 inhibitor and acute dengue virus infection.
ADAMTS13 Levels in Young Patients With ?-Thalassemia Major: Relation to Hepatitis C Virus Infection, Liver Cirrhosis, and Iron Overload.
Biomarkers of endothelial activation/dysfunction in infectious diseases.
Interferon induced thrombotic microangiopathy (TMA): Analysis and concise review.
von Willebrand Disease, Type 1
An amino acid polymorphism in von Willebrand factor correlates with increased susceptibility to proteolysis by ADAMTS13.
Changes in von Willebrand factor-cleaving protease (ADAMTS13) activity after infusion of desmopressin.
The prevalence of the cysteine1584 variant of von Willebrand factor is increased in type 1 von Willebrand disease: co-segregation with increased susceptibility to ADAMTS13 proteolysis but not clinical phenotype.
von Willebrand Disease, Type 2
A common mechanism by which type 2A von Willebrand disease mutations enhance ADAMTS13 proteolysis revealed with a von Willebrand factor A2 domain FRET construct.
Simultaneous Exposure of Sites in von Willebrand Factor for Glycoprotein Ib Binding and ADAMTS13 Cleavage: Studies With Ristocetin.
The effect of exercise on von Willebrand factor and ADAMTS-13 in individuals with type 1 and type 2B von Willebrand disease.
von Willebrand Disease, Type 3
ADAMTS-13 and bleeding phenotype in von Willebrand disease.
Plasma levels of von Willebrand factor regulate ADAMTS-13, its major cleaving protease.
von Willebrand Diseases
A cluster of mutations in the D3 domain of von Willebrand factor correlates with a distinct subgroup of von Willebrand disease: type 2A/IIE.
A common mechanism by which type 2A von Willebrand disease mutations enhance ADAMTS13 proteolysis revealed with a von Willebrand factor A2 domain FRET construct.
A novel nanobody that detects the gain-of-function phenotype of von Willebrand factor in ADAMTS13 deficiency and von Willebrand disease type 2B.
ADAMTS-13 activity in von Willebrand disease.
ADAMTS-13 and bleeding phenotype in von Willebrand disease.
ADAMTS13 unbound to larger von Willebrand factor multimers in cryosupernatant: implications for selection of plasma preparations for thrombotic thrombocytopenic purpura treatment.
An amino acid polymorphism in von Willebrand factor correlates with increased susceptibility to proteolysis by ADAMTS13.
Are increased levels of von Willebrand factor in chronic coronary heart disease caused by decrease in von Willebrand factor cleaving protease activity? A study by an immunoassay with antibody against intact bond 842Tyr-843Met of the von Willebrand factor protein.
C2362F mutation gives rise to an ADAMTS13-resistant von Willebrand factor.
Changes in von Willebrand factor-cleaving protease (ADAMTS13) activity after infusion of desmopressin.
Effect of von Willebrand disease type 2B and type 2M mutations on the susceptibility of von Willebrand factor to ADAMTS-13.
Higher and lower active circulating VWF levels: different facets of von Willebrand disease.
Interactions of von Willebrand factor and ADAMTS13 in von Willebrand disease and thrombotic thrombocytopenic purpura.
Plasma levels of von Willebrand factor regulate ADAMTS-13, its major cleaving protease.
Platelet-free shear flow assay facilitates analysis of shear-dependent functions of VWF and ADAMTS13.
Shear stress and von Willebrand factor in health and disease.
Simultaneous Exposure of Sites in von Willebrand Factor for Glycoprotein Ib Binding and ADAMTS13 Cleavage: Studies With Ristocetin.
The effect of exercise on von Willebrand factor and ADAMTS-13 in individuals with type 1 and type 2B von Willebrand disease.
The prevalence of the cysteine1584 variant of von Willebrand factor is increased in type 1 von Willebrand disease: co-segregation with increased susceptibility to ADAMTS13 proteolysis but not clinical phenotype.
Type 1 von Willebrand disease: a possible novel mechanism.
Type 2 A (group II) von Willebrand disease mutations increase the susceptibility of VWF to ADAMTS-13.
Update on von Willebrand factor multimers: focus on high-molecular-weight multimers and their role in hemostasis.
von Willebrand factor and von Willebrand disease.
[A2 domain of human von Willebrand factor expressed in e. Coli and its biological activity.]
[Increased susceptibility of recombinant type 2A von Willebrand factor mutant A1500E to proteolysis by ADAMTS13].