|
Purpura, Thrombotic Thrombocytopenic
|
0.100 |
Biomarker
|
disease |
BEFREE |
This study enrolled any patient who was diagnosed with TMA excluding Shiga toxin-producing Escherichia coli-HUS or thrombotic thrombocytopenic purpura based on Japanese clinical guide published in 2013 as inclusion criteria and treated with eculizumab.
|
29959568 |
2019 |
|
Purpura, Thrombotic Thrombocytopenic
|
0.100 |
Biomarker
|
disease |
BEFREE |
In 2013, eculizumab was approved for treatment of the atypical hemolytic-uremic syndrome (aHUS) in Japan, which was defined as a thrombotic microangiopathy (TMA) excluding Shiga toxin-producing Escherichia coli-HUS and thrombotic thrombocytopenic purpura.
|
30039480 |
2019 |
|
Purpura, Thrombotic Thrombocytopenic
|
0.100 |
Biomarker
|
disease |
BEFREE |
TTP and CM HUS are rare disorders, and their diagnosis may be missed, no less because features at presentation may be misdiagnosed as a pregnancy-related TMA, such as hypertension, proteinuria, fetal growth restriction, or in utero fetal death.
|
30177429 |
2018 |
|
Purpura, Thrombotic Thrombocytopenic
|
0.100 |
Biomarker
|
disease |
BEFREE |
If TTP and STEC-HUS have been excluded, a diagnosis of aHUS should be considered, for which treatment is with the monoclonal complement C5 inhibitor, eculizumab.
|
29419916 |
2018 |
|
Purpura, Thrombotic Thrombocytopenic
|
0.100 |
Biomarker
|
disease |
BEFREE |
If TTP and STEC-HUS have been excluded, a diagnosis of aHUS should be considered, for which treatment is with the monoclonal complement C5 inhibitor, eculizumab.
|
29582550 |
2018 |
|
Purpura, Thrombotic Thrombocytopenic
|
0.100 |
Biomarker
|
disease |
BEFREE |
TTP is confirmed by severe ADAMTS13 deficiency (which can be undertaken in real time) and CM-HUS by an abnormality in complement regulators, confirmed by mutational analysis (in 60% to 70% of cases) or the presence of Factor H antibodies (which may not be available for weeks or months).
|
29222317 |
2017 |
|
Purpura, Thrombotic Thrombocytopenic
|
0.100 |
AlteredExpression
|
disease |
BEFREE |
For thrombotic microangiopathies (TMAs), the diagnosis of atypical hemolytic uremic syndrome (aHUS) is made by ruling out Shiga toxin-producing Escherichia coli (STEC)-associated HUS and ADAMTS13 activity-deficient thrombotic thrombocytopenic purpura (TTP), often using the exclusion criteria for secondary TMAs.
|
25951460 |
2015 |
|
Purpura, Thrombotic Thrombocytopenic
|
0.100 |
Biomarker
|
disease |
BEFREE |
Until recently, atypical hemolytic uremic syndrome (aHUS), conventionally defined in the pediatric literature as a syndrome of the triad of renal failure, microangiopathic hemolytic anemia, and thrombocytopenia without a prodrome of hemorrhagic diarrhea, has received little attention in adult practice because the patients are commonly given the diagnosis of thrombotic thrombocytopenic purpura (TTP) or TTP/HUS and treated as TTP with plasma exchange, augmented in refractory cases with rituximab and sometimes even splenectomy.
|
25280590 |
2014 |
|
Purpura, Thrombotic Thrombocytopenic
|
0.100 |
Biomarker
|
disease |
BEFREE |
Complement-mediated aHUS is distinct from bacterial shiga-toxin (produced e.g. by E. coli O:157 or O:104 serotypes) induced "typical" HUS, thrombotic thrombocytopenic purpura (TTP) associated with ADAMTS13 (an adamalysin enzyme) dysfunction and from a recently described disease related to mutations in intracellular diacylglycerol kinase ε (DGKE).
|
23743117 |
2013 |
|
Purpura, Thrombotic Thrombocytopenic
|
0.100 |
Biomarker
|
disease |
BEFREE |
In addition autoimmune forms, with acquired, de-novo generated inhibitors in the form of autoantibodies exist for both disorders, affecting ADAMTS13 in TTP or the central complement inhibitor factor H in HUS.
|
20539230 |
2010 |
|
Purpura, Thrombotic Thrombocytopenic
|
0.100 |
Biomarker
|
disease |
BEFREE |
Recent studies demonstrate that Stx also induces von Willebrand factor (VWF) secretion by human endothelial cells and causes thrombotic thrombocytopenic purpura, a disease with similarities to D+HUS, in Adamts13(-/-) mice.
|
20644116 |
2010 |
|
Purpura, Thrombotic Thrombocytopenic
|
0.100 |
Biomarker
|
disease |
BEFREE |
In TTP thrombi formation occurs subsequently to the release of multimers of von Willebrand Factor (vWF) and in HUS (both typical and atypical) to endothelial cell damage (via toxins or complement dysregulation).
|
19640589 |
2009 |
|
Purpura, Thrombotic Thrombocytopenic
|
0.100 |
Biomarker
|
disease |
BEFREE |
Renal failure is a predominant complication of both Stx-HUS and atypical HUS, whereas neurological complications are more prominent in TTP.
|
16760911 |
2006 |
|
Purpura, Thrombotic Thrombocytopenic
|
0.100 |
GeneticVariation
|
disease |
BEFREE |
Thirteen mutations were located in exons XXII and XXIII.No TTP patient carried HF1 mutations.
|
14583443 |
2003 |
|
Purpura, Thrombotic Thrombocytopenic
|
0.100 |
Biomarker
|
disease |
BEFREE |
A child with chronic relapsing thrombotic thrombocytopenic purpura (TTP/HUS) had recurrent thrombocytopenia, microangiopathic hemolytic anemia with fragmented erythrocytes, microthrombi in the lung vessels, and renal dysfunction.
|
10955922 |
2000 |
|
Purpura, Thrombotic Thrombocytopenic
|
0.100 |
Biomarker
|
disease |
BEFREE |
Reduced C3 clusters in familial HUS and TTP is likely related to a genetically determined deficiency in factor H and may predispose to the disease.
|
10215327 |
1999 |
|
Purpura, Thrombotic Thrombocytopenic
|
0.100 |
GeneticVariation
|
disease |
BEFREE |
Two brothers and one sister had three variants of thrombotic thrombocytopenic purpura and haemolytic uraemic syndrome (the 'TTP-HUS' complex).
|
3389894 |
1988 |