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Blood, 15 January 2006, Vol. 107, No. 2, pp. 528-534. Prepublished online as a Blood First Edition Paper on September 27, 2005; DOI 10.1182/blood-2005-03-1087. This Article Full Text Full Text (PDF) All Versions of this Article: 2005-03-1087v1 107/2/528    most recent Alert me when this article is cited Alert me if a correction is posted Citation Map Services Email this article to a friend Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Download to citation manager Rights and Permissions Citing Articles Citing Articles via HighWire Citing Articles via CrossRef Citing Articles via Google Scholar Google Scholar Articles by Ono, T. Articles by Sakata, Y. Search for Related Content PubMed PubMed Citation Articles by Ono, T. Articles by Sakata, Y. Related Collections Hemostasis, Thrombosis, and Vascular Biology Clinical Trials and Observations Social Bookmarking                   What's this?  Previous Article  |  Table of Contents  |  Next Article  HEMOSTASIS, THROMBOSIS, AND VASCULAR BIOLOGY 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 Tomoko Ono, Jun Mimuro, Seiji Madoiwa, Kenji Soejima, Yuji Kashiwakura, Akira Ishiwata, Katsuhiro Takano, Tsukasa Ohmori, and Yoichi Sakata From the Research Division of Cell and Molecular Medicine, Center for Molecular Medicine, Jichi Medical School, Tochigi-ken, Japan; the Research and Development Department, Mitsubishi Kagaku Iatron, Chiba-ken, Japan; and the Chemo-Sero Therapeutic Research Institute, Kumamoto-ken, Japan. 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 of acute renal failure and serum creatinine levels in patients with ADAMTS13 activity levels lower than 20% (incidence, 41.2%; creatinine, 160 ± 150 µM [1.81 ± 1.70 mg/dL]) (P < .05) were significantly higher than they were in patients with ADAMTS13 activity levels higher than 20% (incidence, 15.4%; creatinine, 84 ± 67 µM [0.95 ± 0.76 mg/dL]) (P < .01). Additionally, unusually large von Willebrand factor multimers were detected in 26 (51.0%) of 51 patients with ADAMTS13 activity levels lower than 20%. Lower molecular weight forms of ADAMTS13 were found in the plasma of patients with sepsis-induced DIC, suggesting that the deficiency of ADAMTS13 was partially caused by its cleavage by proteases in addition to decreased synthesis in the liver. These data suggested that severe secondary ADAMTS13 deficiency can be associated with sepsis-induced DIC and may contribute to the development of renal failure. CiteULike    Connotea    Del.icio.us    Digg    Reddit    Technorati    What's this? This article has been cited by other articles: T. J. Raife, W. Cao, B. S. Atkinson, B. Bedell, R. R. Montgomery, S. R. Lentz, G. F. Johnson, and X. L. Zheng Leukocyte proteases cleave von Willebrand factor at or near the ADAMTS13 cleavage site Blood, August 20, 2009; 114(8): 1666 - 1674. [Abstract] [Full Text] [PDF] Q. de Mast, E. Groot, P. B. Asih, D. Syafruddin, M. Oosting, S. Sebastian, B. Ferwerda, M. G. Netea, P. G. de Groot, A. J. A. M. van der Ven, et al. ADAMTS13 Deficiency with Elevated Levels of Ultra-Large and Active von Willebrand Factor in P. falciparum and P. vivax Malaria Am J Trop Med Hyg, March 1, 2009; 80(3): 492 - 498. [Abstract] [Full Text] [PDF] K. N. Patel, S. H. Soubra, R. V. Bellera, J.-F. Dong, C. A. McMullen, A. R. Burns, and R. E. Rumbaut Differential Role of von Willebrand Factor and P-Selectin on Microvascular Thrombosis in Endotoxemia Arterioscler Thromb Vasc Biol, December 1, 2008; 28(12): 2225 - 2230. [Abstract] [Full Text] [PDF] A. K. Chauhan, J. Kisucka, A. Brill, M. T. Walsh, F. Scheiflinger, and D. D. Wagner ADAMTS13: a new link between thrombosis and inflammation J. Exp. Med., September 1, 2008; 205(9): 2065 - 2074. [Abstract] [Full Text] [PDF] M.-C. Chung, T. G. Popova, S. C. Jorgensen, L. Dong, V. Chandhoke, C. L. Bailey, and S. G. Popov Degradation of Circulating von Willebrand Factor and Its Regulator ADAMTS13 Implicates Secreted Bacillus anthracis Metalloproteases in Anthrax Consumptive Coagulopathy J. Biol. Chem., April 11, 2008; 283(15): 9531 - 9542. [Abstract] [Full Text] [PDF] C. V. Denis Pas de deux between VWF and ADAMTS13 Blood, April 1, 2008; 111(7): 3306 - 3307. [Full Text] [PDF] A. K. Chauhan, M. T. Walsh, G. Zhu, D. Ginsburg, D. D. Wagner, and D. G. Motto The combined roles of ADAMTS13 and VWF in murine models of TTP, endotoxemia, and thrombosis Blood, April 1, 2008; 111(7): 3452 - 3457. [Abstract] [Full Text] [PDF] B.-J. H. van den Born, N. V. van der Hoeven, E. Groot, P. J. Lenting, J. C.M. Meijers, M. Levi, and G. A. van Montfrans Association Between Thrombotic Microangiopathy and Reduced ADAMTS13 Activity in Malignant Hypertension Hypertension, April 1, 2008; 51(4): 862 - 866. [Abstract] [Full Text] [PDF] B. Lammle, J. A. Kremer Hovinga, and J. N. George Acquired thrombotic thrombocytopenic purpura: ADAMTS13 activity, anti-ADAMTS13 autoantibodies and risk of recurrent disease Haematologica, February 1, 2008; 93(2): 172 - 177. [Full Text] [PDF] J. Song, K. A Lee, T. S. Park, R. Park, and J. R. Choi Linear Relationship between ADAMTS13 Activity and Platelet Dynamics Even Before Severe Thrombocytopenia Ann. Clin. Lab. Sci., January 1, 2008; 38(4): 368 - 375. [Abstract] [Full Text] [PDF] C. L. Bockmeyer, R. A. Claus, U. Budde, K. Kentouche, R. Schneppenheim, W. Losche, K. Reinhart, and F. M. Brunkhorst Inflammation-associated ADAMTS13 deficiency promotes formation of ultra-large von Willebrand factor Haematologica, January 1, 2008; 93(1): 137 - 140. [Abstract] [Full Text] [PDF] P. Zhang, W. Pan, A. H. Rux, B. S. Sachais, and X. L. Zheng The cooperative activity between the carboxyl-terminal TSP1 repeats and the CUB domains of ADAMTS13 is crucial for recognition of von Willebrand factor under flow Blood, September 15, 2007; 110(6): 1887 - 1894. [Abstract] [Full Text] [PDF] J. N. George Evaluation and Management of Patients With Thrombotic Thrombocytopenic Purpura J Intensive Care Med, March 1, 2007; 22(2): 82 - 91. [Abstract] [PDF] D. Shang, X. W. Zheng, M. Niiya, and X. L. Zheng 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 Blood, October 1, 2006; 108(7): 2207 - 2215. [Abstract] [Full Text] [PDF] J. N. George Clinical practice. Thrombotic thrombocytopenic purpura. N. Engl. J. Med., May 4, 2006; 354(18): 1927 - 1935. [Full Text] [PDF] P. S.M., M. T., S. A., Y. H.T., C. D., K. S.A., S. V.P., W. B., L. P.S., T. S.M., et al. Leaking Capillaries and White Lung in Sepsis--Is Angiopoietin 2 the Culprit?: Excess Circulating Angiopoietin-2 May Contribute to Pulmonary Vascular Leak in Sepsis in Humans. PLoS Medicine 3: e46, 2006 J. Am. Soc. Nephrol., May 1, 2006; 17(5): 1207 - 1217. [Full Text] [PDF] J. E. Sadler Thrombotic Thrombocytopenic Purpura: A Moving Target Hematology, January 1, 2006; 2006(1): 415 - 420. [Abstract] [Full Text] [PDF]

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