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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.

Published on Oct 1, 2008in Critical Care Medicine6.971
· DOI :10.1097/CCM.0b013e318186aa49
Trung C. Nguyen15
Estimated H-index: 15
,
Yong Y. Han16
Estimated H-index: 16
+ 6 AuthorsJoseph A. Carcillo51
Estimated H-index: 51
Abstract
Background:Thrombocytopenia-associated multiple organ failure (TAMOF) is a poorly understood syndrome in critically ill children. A disintegrin and metalloprotease with thrombospondin motifs (ADAMTS-13), formerly known as von Willebrand factor (VWF) cleaving protease, is decreased in adults with VWF
  • References (43)
  • Citations (110)
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References43
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#1Michael DarmonH-Index: 44
#2Elie AzoulayH-Index: 84
Last. Benoît SchlemmerH-Index: 53
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Introduction: Few studies have investigated adults with thrombotic microangiopathy (TMA) requiring intensive care unit (ICU) admission, and the treatment remains controversial. Objective: To describe causes, outcomes, prognostic factors, and daily organ-failure score changes in adults with TMA requiring ICU admission. Design: A 3-yr single-center cohort study. Patients: The patients were 36 adults with TMA admitted to a teaching-hospital medical ICU between January 2000 and June 2003. Results: O...
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#1N Turner (Rice University)H-Index: 23
#2Leticia Nolasco (BCM: Baylor College of Medicine)H-Index: 25
Last. Joel L. Moake (Rice University)H-Index: 50
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Summary. Hepatic stellate cells have been considered to be a primary source for human plasma ADAMTS-13, the von Willebrand factor (VWF)-cleaving metalloprotease. In this study, ADAMTS-13 antigen was detected by immunofluorescence in both venous (HUVECs) and arterial endothelial cells (HUAECs) using both polyclonal antibodies made against peptides found in various domains of human ADAMTS-13, as well as by a monoclonal antibody against the ADAMTS-13 metalloprotease domain. ADAMTS-13 antigen had an...
139 CitationsSource
#1Anil K. Chauhan (Harvard University)H-Index: 27
#2David G. Motto (UM: University of Michigan)H-Index: 29
Last. Denisa D. Wagner (Harvard University)H-Index: 103
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The metalloprotease ADAMTS13 (a disintegrin-like and metalloprotease with thrombospondin type I repeats 13) cleaves highly adhesive large von Willebrand factor (VWF) multimers after their release from the endothelium. ADAMTS13 deficiency is linked to a life-threatening disorder, thrombotic thrombocytopenic purpura (TTP), characterized by platelet-rich thrombi in the microvasculature. Here, we show spontaneous thrombus formation in activated microvenules of Adamts13−/− mice by intravital microsco...
178 CitationsSource
#1Tomoko OnoH-Index: 4
#2Jun MimuroH-Index: 26
Last. Yoichi SakataH-Index: 27
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Deficiency of ADAMTS13 is found in patients with thrombotic thrombocytopenic purpura (TTP), and the genetic defects in the ADAMTS13 gene or the autoantibody against ADAMTS13 is thought to be responsible for the development of TTP. The clinical correlation and mechanisms of secondary ADAMTS13 deficiency in other disease states were investigated. In addition to TTP, ADAMTS13 levels were severely decreased in patients with sepsis-induced disseminated intravascular coagulation (DIC). The incidence o...
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#1David G. Motto (UM: University of Michigan)H-Index: 29
#2Anil K. Chauhan (Harvard University)H-Index: 27
Last. David GinsburgH-Index: 73
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Thrombotic thrombocytopenic purpura (TTP) is a life-threatening illness caused by deficiency of the vWF-cleaving protease ADAMTS13. Here we show that ADAMTS13-deficient mice are viable and exhibit normal survival, although vWF-mediated platelet-endothelial interactions are significantly prolonged. Introduction of the genetic background CASA/Rk (a mouse strain with elevated plasma vWF) resulted in the appearance of spontaneous thrombocytopenia in a subset of ADAMTS13-deficient mice and significan...
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#2Mari Inada (Albert Einstein College of Medicine)H-Index: 5
Last. Han-Mou Tsai (Albert Einstein College of Medicine)H-Index: 36
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ADAMTS13 is a circulating zinc metalloprotease that cleaves the hemostatic glycoprotein von Willebrand factor (VWF) in a shear-dependent manner. Deficiency in ADAMTS13, owing to genetic mutations or autoimmune inhibitors, causes thrombotic thrombocytopenic purpura (TPP). Northern blot analysis has shown that ADAMTS13 is expressed primarily in the liver. By using real-time RT-PCR, we confirmed that in mice the liver had the highest level of the ADAMTS13 transcript. To identify the liver cell-type...
138 CitationsSource
#1Allen P. Burke (AFIP: Armed Forces Institute of Pathology)H-Index: 77
#2Erik K. Mont (AFIP: Armed Forces Institute of Pathology)H-Index: 13
Last. Renu Virmani (AFIP: Armed Forces Institute of Pathology)H-Index: 141
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Abstract Background Thrombotic thrombocytopenic purpura (TTP) shares histologic features with disseminated intravascular coagulation (DIC) but is clinically distinct. TTP may result in myocardial hemorrhages and rapid death. We compared rapidly fatal TTP with heart involvement and DIC cases to determine if the differential diagnosis of TTP and DIC could be aided by immunohistochemical techniques. Design We examined 11 hearts from seven women and four men dying with TTP (aged 34±10 years) and 8 h...
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#1Jan-Dirk StudtH-Index: 13
Last. Bernhard LämmleH-Index: 49
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Severe ADAMTS13 deficiency in thrombotic thrombocytopenic purpura (TTP) is either constitutional and caused by ADAMTS13 mutations, or acquired and most often due to ADAMTS13 inhibitory autoantibodies. In strongly hemolytic serum of a pediatric patient, diagnosed with TTP postmortem, ADAMTS13 activity was less than 3%. Both parents had an ADAMTS13 activity of approximately 50%. Sequencing of the ADAMTS13 gene revealed an intronic 687-2A>G substitution affecting exon 7, homozygous in the propositu...
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#1Jing Fei Dong (BCM: Baylor College of Medicine)H-Index: 51
#2Jody L. Whitelock (BCM: Baylor College of Medicine)H-Index: 4
Last. Miguel Cruz (BCM: Baylor College of Medicine)H-Index: 39
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Summary. von Willebrand factor (VWF) freshly released from endothelial cells is normally cleaved by the ADAMTS-13 metalloprotease to prevent the direct release of these ultra-large (UL) and hyper-reactive multimers into plasma. The balance of ULVWF proteolysis may be regulated by the amount of ULVWF released and the processing capacity of ADAMTS-13. The former associates with the size of ULVWF storage pool, sensitivity of vascular endothelial cells to stimulation, and the type of agonists, where...
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#1Aubrey Bernardo (BCM: Baylor College of Medicine)H-Index: 12
#2Chalmette Ball (BCM: Baylor College of Medicine)H-Index: 8
Last. Jing Fei Dong (BCM: Baylor College of Medicine)H-Index: 51
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ADAMTS13 cleaves ultralarge and hyperreactive von Willebrand factor (ULVWF) freshly released from activated endothelial cells to smaller and less active forms. This process may be affected by the amount of ULVWF released and the processing capacity of ADAMTS13, contributing to the development of thrombotic diseases. We examined the effects of inflammatory cytokines on the release and cleavage of ULVWF to evaluate potential links between inflammation and thrombosis. Human umbilical vein endotheli...
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