|
ADAMTS13
|
Purpura, Thrombotic Thrombocytopenic
|
0.700 |
Biomarker |
LHGDN |
Persistence of high titers of anti-ADAMTS13 autoantibodies predisposes to relapsing TTP.
|
19190804 |
2009 |
|
ADAMTS13
|
Purpura, Thrombotic Thrombocytopenic
|
0.700 |
GeneticVariation |
LHGDN |
Functional deficiency of ADAMTS13 caused by genetic mutation and inhibitory autoantibodies leads to the accumulation of unusually large VWF multimers in the plasma and results in a thrombotic disease known as thrombotic thrombocytopenic purpura (TTP).
|
19069166 |
2008 |
|
ADAMTS13
|
Purpura, Thrombotic Thrombocytopenic
|
0.700 |
Biomarker |
LHGDN |
The short-term prognostic usefulness of ADAMTS13 testing during acute TTP warrants further investigation because of limited prospective studies (Ferrari S et al., Blood 2007;109:2815-2822; Peyvandi et al., Haematologica 2008;93:232-239).
|
18756543 |
2008 |
|
ADAMTS13
|
Purpura, Thrombotic Thrombocytopenic
|
0.700 |
Biomarker |
LHGDN |
[Thrombosis and ADAMTS13 exclusively produced in the liver].
|
18833927 |
2008 |
|
ADAMTS13
|
Purpura, Thrombotic Thrombocytopenic
|
0.700 |
Biomarker |
LHGDN |
We detected a missense mutation (C3178T) in exon 24 of ADAMTS-13 in 6/53 (11.3%) adult onset TTP patients, but no normal controls (n = 100).
|
18031293 |
2008 |
|
ADAMTS13
|
Purpura, Thrombotic Thrombocytopenic
|
0.700 |
AlteredExpression |
LHGDN |
Survivors of an acute episode of acquired thrombotic thrombocytopenic purpura with severely reduced levels of ADAMTS13 and/or with anti-ADAMTS13 antibodies during remission have an approximately three-fold greater likelihood of developing another episode of thrombotic thrombocytopenic purpura than patients with higher protease activity and no antibody.
|
18223285 |
2008 |
|
ADAMTS13
|
Purpura, Thrombotic Thrombocytopenic
|
0.700 |
GeneticVariation |
LHGDN |
By use of a collagen-binding assay, ADAMTS13 activity was analyzed at presentation in 20 patients with HIV-related TTP.
|
17725738 |
2007 |
|
ADAMTS13
|
Purpura, Thrombotic Thrombocytopenic
|
0.700 |
GeneticVariation |
LHGDN |
ADAMTS13 mutations S203P, R268P, R507Q and A596V were previously identified in French patients with hereditary thrombotic thrombocytopenic purpura (TTP) (Upshaw-Schulman syndrome).
|
17849048 |
2007 |
|
ADAMTS13
|
Purpura, Thrombotic Thrombocytopenic
|
0.700 |
AlteredExpression |
LHGDN |
These findings were compatible with TTP and the decreased activity of ADAMTS13 was considered to be associated with the disease activity of TTP.
|
16391894 |
2007 |
|
ADAMTS13
|
Purpura, Thrombotic Thrombocytopenic
|
0.700 |
Biomarker |
LHGDN |
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.
|
16953270 |
2006 |
|
ADAMTS13
|
Purpura, Thrombotic Thrombocytopenic
|
0.700 |
Biomarker |
LHGDN |
Development of ADAMTS13 inhibitor in a patient with hepatitis C virus-related liver cirrhosis causes thrombotic thrombocytopenic purpura.
|
15710227 |
2005 |
|
ADAMTS13
|
Purpura, Thrombotic Thrombocytopenic
|
0.700 |
Biomarker |
LHGDN |
ADAMTS13, thrombotic thrombocytopenic purpura, and hemolytic uremic syndrome.
|
15865866 |
2005 |
|
ADAMTS13
|
Purpura, Thrombotic Thrombocytopenic
|
0.700 |
GeneticVariation |
LHGDN |
No cases have been found without mutations in the ADAMTS13 gene, suggesting that genetic defects in ADAMTS13 are the dominant cause of hereditary TTP.
|
14727257 |
2004 |
|
ADAMTS13
|
Purpura, Thrombotic Thrombocytopenic
|
0.700 |
Biomarker |
LHGDN |
Identification of novel mutations in ADAMTS13 in an adult patient with congenital thrombotic thrombocytopenic purpura.
|
15126318 |
2004 |
|
ADAMTS13
|
Purpura, Thrombotic Thrombocytopenic
|
0.700 |
Biomarker |
LHGDN |
Severe deficiency of the von Willebrand Factor (VWF)-cleaving proteinase, ADAMTS13, is associated with the development of thrombotic thrombocytopenic purpura (TTP).
|
14512317 |
2004 |
|
ADAMTS13
|
Purpura, Thrombotic Thrombocytopenic
|
0.700 |
GeneticVariation |
LHGDN |
ADAMTS13 severe enzymatic deficiency and mutations have been described in the congenital thrombotic thrombocytopenic purpura (TTP or Upshaw-Schulman syndrome), a rare and severe disease related to multivisceral microvascular thrombosis.
|
15009458 |
2004 |
|
ADAMTS13
|
Purpura, Thrombotic Thrombocytopenic
|
0.700 |
Biomarker |
LHGDN |
Acquired idiopathic TTP, in contrast, is the result in many patients of the production of autoantibodies that inhibit the function of ADAMTS-13.
|
14727254 |
2004 |
|
ADAMTS13
|
Purpura, Thrombotic Thrombocytopenic
|
0.700 |
Biomarker |
LHGDN |
Blood for DNA analysis was collected from six unrelated TTP families, consisting of nine patients from four different countries, and was screened for mutations in the ADAMTS13 gene.
|
12753286 |
2003 |
|
ADAMTS13
|
Purpura, Thrombotic Thrombocytopenic
|
0.700 |
Biomarker |
LHGDN |
This article reviews the role of this cleavage in regulating vWF-platelet interaction and proposes a scheme for understanding how a deficiency of ADAMTS13 results in the development of microthrombi in patients with thrombotic thrombocytopenic purpura.
|
12615692 |
2003 |
|
ADAMTS13
|
Purpura, Thrombotic Thrombocytopenic
|
0.700 |
AlteredExpression |
LHGDN |
We report data on a patient with confirmed TTP who had severely reduced ADAMTS-13 activity but showed no ADAMTS-13 inhibition in a widely used fluid phase activity assay.
|
12855569 |
2003 |
|
ADAMTS13
|
Purpura, Thrombotic Thrombocytopenic
|
0.700 |
AlteredExpression |
LHGDN |
A severe deficiency of ADAMTS13 (< 5%), identified in more than 120 patients during 1996 to 2001 in our laboratory, is specific for a thrombotic microangiopathy commonly labeled TTP.
|
12091372 |
2002 |
|
ADAMTS13
|
Purpura, Thrombotic Thrombocytopenic
|
0.700 |
Biomarker |
LHGDN |
Von Willebrand factor, ADAMTS13, and thrombotic thrombocytopenic purpura.
|
12395148 |
2002 |
|
ADAMTS13
|
Purpura, Thrombotic Thrombocytopenic
|
0.700 |
AlteredExpression |
LHGDN |
In patients with TTP and HUS there was indirect evidence of increased VWF fragmentation, and this occurred also in patients with ADAMTS13 deficiency.
|
12130486 |
2002 |
|
ADAMTS13
|
Purpura, Thrombotic Thrombocytopenic
|
0.700 |
GeneticVariation |
LHGDN |
We show that deficiency of ADAMTS13 is the molecular mechanism responsible for TTP, and suggest that physiologic proteolysis of VWF and/or other ADAMTS13 substrates is required for normal vascular homeostasis.
|
11586351 |
2001 |
|
ADAMTS13
|
Purpura, Thrombotic Thrombocytopenic
|
0.700 |
Biomarker |
LHGDN |
Human genetics. To clot or not.
|
11586344 |
2001 |