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Objective. Severe acute pancreatitis (SAP) frequently progresses to pancreatitis-associated multiorgan failure (MOF) with high mortality. Decreased plasma ADAMTS13 activity (ADAMTS13:AC) results in the accumulation of unusually large von Willebrand factor multimers (UL-VWFM) and the formation of platelet thrombi, ultimately leading to MOF. The purpose of the study was to investigate the potential role of ADAMTS13:AC in the severity of SAP. Material and methods. Plasma ADAMTS13:AC and its related parameters were sequentially determined in 13 SAP patients. ADAMTS13:AC was determined by the chromogenic act-ELISA. Results. Within 1 or 2 days after admission, ADAMTS13:AC was lower in SAP patients (mean 28%) than in healthy controls (99%), and gradually recovered in the 11 survivors but further decreased in the 2 non-survivors. Patients with higher sepsis-related organ failure assessment (SOFA) scores showed lower ADAMTS13:AC than those without these scores. The inhibitor against ADAMTS13 was undetectable. On day 1, von Willebrand factor antigen (VWF:Ag) was higher (402%, p<0.001) in SAP patients than in controls (100%). VWF:Ag gradually decreased in the survivors, except in the 3 patients needing a necrosectomy, but remained high in the non-survivors. ADAMTS13:AC was inversely correlated with the APACHE II score (r=-0.750, p<0.005), and increased plasma concentrations of interleukin 6 (IL-6) and IL-8 at admission. UL-VWFM-positive patients had lower ADAMTS13:AC and decreased serum calcium concentrations, but higher VWF:Ag and IL-8 concentrations than UL-VWFM-negative patients. Conclusions. Plasma ADAMTS13:AC was closely related to the APACHE II score. This intimate relationship may serve as an early prognostic indicator for SAP patients. The imbalance between decreased ADAMTS13:AC and increased UL-VWFM could contribute to SAP pathogenesis through enhanced thrombogenesis. [ABSTRACT FROM AUTHOR]