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Title: Acquired ADAMTS-13 Deficiency in Pediatric Patients with Severe Sepsis
Authors: Nguyen, Trung C.;Liu, Anne;Liu, Li;Ball, Chalmette;Choi, Hiuwan;May, William S.;Aboulfatova, Khatira;Bergeron, Angela L.;Dong, Jing-fei
Contributors: 化學系
Keywords: Sepsis;ADAMTS-13;ULWF;Thrombotic microangiopathy
Date: 2007
Issue Date: 2012-07-03T03:29:46Z
Publisher: Ferrata Storti Foundation
Abstract: We studied the state of ultralarge von Willebrand factor (ULVWF) proteolysis in 21
pediatric patients with severe sepsis and found that the overall group of patients
had moderately reduced ADAMTS-13 activity, but 31% had severe enzymatic deficiency.
The severe deficiency correlated with greater adhesion activity of von
Willebrand factor, severity of thrombocytopenia and plasma levels of interleukin-6.
It also correlated clinically with severity of illness and organ dysfunction. These
results suggest that ULVWF proteolysis is insufficient in septic patients and severely
deficient in a subgroup of patients. The deficiency may contribute to the development
of thrombocytopenia and ischemic organ failure associated with sepsis.
Relation: Haematologica, 92(1): 121-124
Appears in Collections:[Department of Chemistry] Periodical Articles

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