thrombotic microangiopathies/protease

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The "cutting" edge: von Willebrand factor-cleaving protease activity in thrombotic microangiopathies.

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Identifikohuni Regjistrohu

The thrombotic microangiopathies (TMAs), including thrombotic thrombocytopenic purpura (TTP) and hemolytic uremic syndrome (HUS), are classically defined by constellations of clinical findings. In the late 1990's, several groups reported that a single test might be able to diagnose TTP and

Factor V Leiden: a genetic risk factor for thrombotic microangiopathy in patients with normal von Willebrand factor-cleaving protease activity.

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Identifikohuni Regjistrohu

Thrombotic microangiopathy (TM) is associated with abnormalities of von Willebrand factor-cleaving protease (VWCP) and other hemostatic factors. This study hypothesized that TM patients might have genetically determined thrombotic risk factors that predispose them to aberrant microvascular

Von Willebrand factor-cleaving protease activity and proteolysis of von Willebrand factor in bone marrow transplant-associated thrombotic microangiopathy.

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Identifikohuni Regjistrohu

BACKGROUND Thrombotic microangiopathy (TM) of the fulminant type occurring in patients following bone marrow transplant (BMT) has clinical manifestations that are similar to thrombotic thrombocytopenic purpura (TTP) and hemolytic uremic syndrome, but the outcome is generally fatal despite

[Measurement of plasma von Willebrand factor cleaving protease in patients with varied thrombotic microangiopathy].

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Identifikohuni Regjistrohu

Thrombotic thrombocytopenic purpura(TTP) is a multisystem disorders characterized by thrombocytopenia, microangiopathic hemolytic anemia associated with red cell fragmentation, and neurological and renal symptoms. Plasma of patients with TTP has been shown to contain unusually large von Willebrand

Von Willebrand factor--cleaving protease activity in thrombotic microangiopathy after living donor liver transplantation: a case report.

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Identifikohuni Regjistrohu

Defective plasma activity of Von Willebrand factor (VWF)-cleaving protease (CP) and/or the inhibitors against this protease has been shown to have a pathological role in several forms of thrombotic microangiopathy (TMA). This report describes a patient for whom a diagnosis of TMA was made

Von Willebrand factor-cleaving protease activity in thrombotic microangiopathy: first report from iran.

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Identifikohuni Regjistrohu

BACKGROUND Thrombotic microangiopathy (TMA) is a rare but devastating small vessels disorder that is characterized by intravascular platelet thrombi, thrombocytopenia, and various degrees of organ ischemia and anemia, which is due to erythrocyte fragmentation in microcirculation. OBJECTIVE The Aim

Complete defect in vWF-cleaving protease activity associated with increased shear-induced platelet aggregation in thrombotic microangiopathy.

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Identifikohuni Regjistrohu

Von Willebrand factor (vWF) cleaving metalloprotease activity represents an important factor in understanding the pathophysiology of thrombotic microangiopathies (TMA). Thrombotic events, leading to dramatic complications, occur preferentially in the microvasculature. Our aim was to determine the

von Willebrand factor-cleaving protease (ADAMTS-13) activity determination in the diagnosis of thrombotic microangiopathies: the Swiss experience.

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Identifikohuni Regjistrohu

Severe deficiency of von Willebrand factor (VWF)-cleaving protease (ADAMTS-13) activity (<5% of normal) is a specific finding for acute idiopathic thrombotic thrombocytopenic purpura (TTP), a disorder that presents as thrombocytopenia, microangiopathic hemolytic anemia, and often organ dysfunction

Cancer-related thrombotic microangiopathy secondary to Von Willebrand factor-cleaving protease deficiency.

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Identifikohuni Regjistrohu

Cancer-related thrombotic microangiopathy (TM) is a serious complication with a short-term life-threatening prognosis. This complication shares certain similarities with thrombotic thrombocytopenic purpura and hemolytic uremic syndrome, both characterized by circulating platelet aggregates

Specific von Willebrand factor-cleaving protease in thrombotic microangiopathies: a study of 111 cases.

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Identifikohuni Regjistrohu

Retrospective studies of patients with thrombotic microangiopathies (TMAs) have shown that a deficient activity of von Willebrand factor (vWF)-cleaving protease is involved in thrombotic thrombocytopenic purpura (TTP) but not in the hemolytic-uremic syndrome (HUS). To further analyze the relevance

Reduced von Willebrand factor-cleaving protease levels in secondary thrombotic microangiopathies and other diseases.

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Identifikohuni Regjistrohu

Thrombotic thrombocytopenic purpura (TTP) is a severe multisystem thrombotic microangiopathy (TMA). Significant advances have been made in understanding the pathogenesis of TTP since the discovery of ADAMTS-13 ( A Disintegrin And Metalloproteinase with Thrombo Spondin-1-like domains), the enzyme

Severe deficiency of VWF-cleaving protease (ADAMTS13) activity defines a distinct population of thrombotic microangiopathy patients.

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Identifikohuni Regjistrohu

BACKGROUND Severe deficiency of ADAMTS13 activity is a biologic risk factor for thrombotic microangiopathy (TMA). It was hypothesized that severe ADAMTS13 deficiency is associated with a distinct TMA subpopulation. METHODS ADAMTS13 activity before treatment was determined retrospectively in 107

Von Willebrand factor-cleaving protease (ADAMTS-13) activity in thrombotic microangiopathies: diagnostic experience 2001/2002 of a single research laboratory.

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Identifikohuni Regjistrohu

BACKGROUND Severe deficiency of von Willebrand factor-cleaving protease (ADAMTS-13) activity (<5% of normal) is specific for classical thrombotic thrombocytopenic purpura (TTP), a disorder presenting with thrombocytopenia, microangiopathic haemolytic anaemia and often with organ dysfunction such as

Thrombotic microangiopathies, thrombotic thrombocytopenic purpura, and ADAMTS-13.

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Identifikohuni Regjistrohu

Thrombotic microangiopathy (TMA) is a term used to describe a group of disorders characterized by hemolytic anemia (with prominent red blood cell fragmentation), thrombocytopenia, and thrombosis in the microvasculature. It may be used when describing patients with thrombotic thrombocytopenic purpura

Proteolytic processing of von Willebrand factor by adamts13 and leukocyte proteases.

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Identifikohuni Regjistrohu

ADAMTS13 is a 190 kDa zinc protease encoded by a gene located on chromosome 9q34. This protease specifically hydrolyzes von Willebrand factor (VWF) multimers, thus causing VWF size reduction. ADAMTS13 belongs to the A Disintegrin And Metalloprotease with ThromboSpondin type 1 repeats (ADAMTS)

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