By H. Krebs, W. Schramm (auth.), Professor Dr.med. Inge Scharrer, Professor Dr.med. Wolfgang Schramm (eds.)
This ebook comprises the contribution to the thirty sixth Hemophilia Symposium, Hamburg 2005. the most subject matters are epidemiolgy, hemophilia treatment, orthopedic remedy in hemophiliacs, hemostaseologic analysis and pediatric hemostaseology. the amount is rounded off by way of quite a few loose papers and posters on hemophilia, inhibitors in hemophilia and diagnostics.
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Extra info for 36th Hemophilia Symposium Hamburg 2005
Results These data demonstrate the deficiencies in diagnosis and treatment of hemophilia patients in Romania. Because our goal is to optimize life expectancy and quality of life, we consider that the NHR is an important instrument for improving the quality of care of hemophiliacs. It allows the identification of people with hemophilia, and monitoring of their health. It is an important tool for policy makers, care givers and patient organizations in planning health-care resources for the treatment of hemophilia, and it is one of the first steps in establishing an effective hemophilia program.
Ligand-receptor interactions of the low density lipoprotein receptor-related protein, a multi-ligand endocytic receptor. Fibrinolysis and Proteolysis 1998; 12: 219–40. 13. Sarafanov AG, Ananyeva NM, Shima M, Saenko EL. Cell surface heparan sulfate proteoglycans participate in factor VIII catabolism mediated by low density lipoprotein receptorrelated protein. J Biol Chem 2001; 276: 11970–9. 14. Bovenschen N, Herz J, Grimbergen JM, Lenting PJ, Havekes LM, Mertens K, van Vlijmen BJ. Elevated plasma factor VIII in a mouse model of low-density lipoprotein receptor-related protein deficiency.
Mice with combined LRP and low-density lipoprotein receptor (LDLR) deficiency show a further increase of FVIII level and more impressive, ~5-fold, prolongation of FVIII residence time in the circulation. Receptor-mediated clearance of FVIII is facilitated by heparan sulfate proteoglycans of extracellular matrix, which provide the initial binding of FVIII to the cell surface. We discuss the mapping of the major high-affinity LRP-binding sites to the regions 484-509 and 1811-1818 of A2 and A3 domains of FVIII, respectively; LDLR-binding sites are yet to be identified.