Research from Lund University, Department of Pediatrics in the area of thrombocytopenic purpura in children published
2009 JUN 1 - (NewsRx.com) -- Data detailed in 'Molecular basis of ADAMTS13 dysfunction in thrombotic thrombocytopenic purpura' have been presented. "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," scientists writing in the journal Pediatric Nephrology report. "Von Willebrand factor is a large glycoprotein that circulates in plasma as a series of multimers, and it plays a major role in primary hemostasis by inducing the formation of platelet plugs at sites of vascular injury and high-shear stress. Its activity is dependent on the sizes of the multimers, with ultra-large (UL) VWF multimers being biologically very potent. The ULVWF multimers are rapidly degraded upon their secretion from endothelial cells in normal individuals but not in the circulation of TTP patients, causing the formation of disseminated thrombi in the latter. The defective breakdown of VWF is attributed to a severely deficient activity of the VWF-cleaving protease ADAMTS13, a plasma metalloprotease synthesized in the liver, kidneys, and endothelium. This protease rapidly degrades VWF-platelet strings under flow by proteolytic cleavage of the VWF subunit, thereby regulating the size of the platelet thrombus," wrote M. Manea and colleagues, Lund University, Department of Pediatrics. The researchers concluded: "Congenital TTP occurs due to ADAMTS13 mutations, with the usual debut occurring during the first years of life, while acquired TTP is associated with auto-antibodies against ADAMTS13." Manea and colleagues published their study in Pediatric Nephrology (Molecular basis of ADAMTS13 dysfunction in thrombotic thrombocytopenic purpura. Pediatric Nephrology, 2009;24(3):447-58). Additional information can be obtained by contacting M. Manea, Clinical Sciences Lund, Dept. of Pediatrics, Lund University, 22185, Lund, Sweden. The publisher of the journal Pediatric Nephrology can be contacted at: Springer, 233 Spring Street, New York, NY 10013, USA. Keywords: Sweden, Lund, Angiology, Hematology, Hemolytic Anemia, Kidney, Microcirculation, Nephrology, Pediatric, Thrombocytopenia, Thrombocytopenic Purpura, Von Willebrand Disease. This article was prepared by Gastroenterology Week editors from staff and other reports. Copyright 2009, Gastroenterology Week via NewsRx.com.
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