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Determination of ADAMTS13 and Its Clinical Significance for ADAMTS13 Supplementation Therapy to Improve the Survival of Patients with Decompensated Liver Cirrhosis
Joint Authors
Nakajima, Yoshiyuki
Uemura, Masahito
Matsumoto, Masanori
Ko, Saiho
Fukui, Hiroshi
Fujimura, Yoshihiro
Source
International Journal of Hepatology
Issue
Vol. 2011, Issue 2011 (31 Dec. 2011), pp.1-12, 12 p.
Publisher
Hindawi Publishing Corporation
Publication Date
2011-07-18
Country of Publication
Egypt
No. of Pages
12
Main Subjects
Abstract EN
The liver plays a central role in hemostasis by synthesizing clotting factors, coagulation inhibitors, and fibrinolytic proteins.
Liver cirrhosis (LC), therefore, impacts on both primary and secondary hemostatic mechanisms.
ADAMTS13 is a metalloproteinase, produced exclusively in hepatic stellate cells, and specifically cleaves unusually large von Willebrand factor multimers (UL-VWFM).
Deficiency of ADAMTS13 results in accumulation of UL-VWFM, which induces platelet clumping or thrombi under high shear stress, followed by sinusoidal microcirculatory disturbances and subsequent progression of liver injuries, eventually leading to multiorgan failure.
The marked imbalance between decreased ADAMTS13 activity (ADAMTS13 : AC) and increased production of UL-VWFM indicating a high-risk state of platelet microthrombi formation was closely related to functional liver capacity, hepatic encephalopathy, hepatorenal syndrome, and intractable ascites in advanced LC.
Some end-stage LC patients with extremely low ADAMTS13 : AC and its IgG inhibitor may reflect conditions similar to thrombotic thrombocytopenic purpura (TTP) or may reflect “subclinical TTP.” Hence, cirrhotic patients with severe to moderate deficiency of ADAMTS13 : AC may be candidates for FFP infusion as a source of ADAMTS13 or for recombinant ADAMTS13 supplementation.
Such treatments may improve the survival of patients with decompensated LC.
American Psychological Association (APA)
Uemura, Masahito& Fujimura, Yoshihiro& Ko, Saiho& Matsumoto, Masanori& Nakajima, Yoshiyuki& Fukui, Hiroshi. 2011. Determination of ADAMTS13 and Its Clinical Significance for ADAMTS13 Supplementation Therapy to Improve the Survival of Patients with Decompensated Liver Cirrhosis. International Journal of Hepatology،Vol. 2011, no. 2011, pp.1-12.
https://search.emarefa.net/detail/BIM-496481
Modern Language Association (MLA)
Uemura, Masahito…[et al.]. Determination of ADAMTS13 and Its Clinical Significance for ADAMTS13 Supplementation Therapy to Improve the Survival of Patients with Decompensated Liver Cirrhosis. International Journal of Hepatology No. 2011 (2011), pp.1-12.
https://search.emarefa.net/detail/BIM-496481
American Medical Association (AMA)
Uemura, Masahito& Fujimura, Yoshihiro& Ko, Saiho& Matsumoto, Masanori& Nakajima, Yoshiyuki& Fukui, Hiroshi. Determination of ADAMTS13 and Its Clinical Significance for ADAMTS13 Supplementation Therapy to Improve the Survival of Patients with Decompensated Liver Cirrhosis. International Journal of Hepatology. 2011. Vol. 2011, no. 2011, pp.1-12.
https://search.emarefa.net/detail/BIM-496481
Data Type
Journal Articles
Language
English
Notes
Includes bibliographical references
Record ID
BIM-496481