Article
Molecular basis of ADAMTS13 dysfunction in thrombotic thrombocytopenic purpura.
Pediatric nephrology (Berlin, Germany) - 1 Mar 2009
Manea Minola, Karpman Diana
Abstract excerpt
Thrombotic thrombocytopenic purpura (TTP) is a thrombotic microangiopathic disorder characterized by thrombocytopenia, hemolytic anemia, neurological and renal manifestations, and fever. It is associated with dysfunctional von Willebrand factor (VWF) proteolysis and the occurrence of VWF- and platelet-rich thrombi in the microcirculation of multiple organs, including the kidneys. Von Willebrand factor is a large...
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