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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\u2009:\u2009AC) 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\u2009:\u2009AC and its IgG inhibitor may reflect conditions similar to thrombotic thrombocytopenic purpura (TTP) or may reflect \u201csubclinical TTP.\u201d Hence, cirrhotic patients with severe to moderate deficiency of ADAMTS13\u2009:\u2009AC 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.<\/jats:p>","DOI":"10.4061\/2011\/759047","type":"journal-article","created":{"date-parts":[[2012,4,12]],"date-time":"2012-04-12T12:20:55Z","timestamp":1334233255000},"page":"1-12","source":"Crossref","is-referenced-by-count":28,"title":["Determination of ADAMTS13 and Its Clinical Significance for ADAMTS13 Supplementation Therapy to Improve the Survival of Patients with Decompensated Liver Cirrhosis"],"prefix":"10.4061","volume":"2011","author":[{"given":"Masahito","family":"Uemura","sequence":"first","affiliation":[{"name":"Third Department of Internal Medicine, Nara Medical University, 840 Shijo-cho, Kashihara, Nara 634-8522, Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yoshihiro","family":"Fujimura","sequence":"additional","affiliation":[{"name":"Department of Blood Transfusion Medicine, Nara Medical University, Kashihara, Nara 634-8522, 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