Textbook of Hemophilia by Christine A. Lee, Erik E. Berntorp, W. Keith Hoots

By Christine A. Lee, Erik E. Berntorp, W. Keith Hoots

The Textbook of Hemophilia has develop into a definitive source for all these coping with hemophilia sufferers. It covers the entire universal and infrequent bleeding problems, either when it comes to medical administration in addition to the genetic, laboratory, monetary and mental features.

This moment version covers the entire most recent advancements within the box of hemophilia, with new chapters on:

  • the genetic and molecular foundation of inherited blood disorders
  • how to regulate adolescent and older patients
  • emergency drugs and inherited blood disorders
  • national hemophilia databases

Drawing at the significant event of the authors, the purpose of this textbook continues to be a similar - to enhance the care of sufferers being affected by hemophilia. The publication is filled with exact suggestions and suggestion on daily scientific questions making it valuable to all trainee and working towards hematologists.

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Sample text

Science 1964; 145: 1310–12. 4 Esmon CT. The protein C pathway. Chest 2003; 124: 26S–32S. 5 Stenflo J. Contributions of Gla and EGF-like domains to the function of vitamin K-dependent coagulation factors. Crit Rev Eukaryot Gene Expr 1999; 9: 59–88. 6 Nesheim ME, Taswell JB, Mann KG. The contribution of bovine Factor V and Factor Va to the activity of prothrombinase. J Biol Chem 1979; 254: 10952–62. 7 van Dieijen G, Tans G, Rosing J, Hemker HC. The role of phospholipid and factor VIIIa in the activation of bovine factor X.

Biochemistry 1992; 31(13): 3315–25. 45 Higuchi M, Wong C, Kochhan L, et al. Characterization of mutations in the factor VIII gene by direct sequencing of amplified genomic DNA. Genomics 1990; 6(1): 65–71. 46 Rand MD, Kalafatis M, Mann KG. Platelet coagulation factor Va: the major secretory platelet phosphoprotein. Blood 1994; 83(8): 2180–90. 47 Kalafatis M, Rand MD, Jenny RJ, Ehrlich YH, Mann KG. Phosphorylation of factor Va and factor VIIIa by activated platelets. Blood 1993; 81(3): 704–19. 48 Ganz PR, Tackaberry ES, Palmer DS, Rock G.

Recently however, a mutation altering a furin cleavage site in canine FVIII was shown to result in increased FVIII activity that was attributed to increased stability, possibly a consequence of reducing dissociation of the heavy and light chains [49]. These observations have contributed to interest in developing single chain FVIII protein for therapeutic use in hemophilia [50]. Summary Eukaryotic cells contain an extensive machinery to modify polypeptides that transit the secretory compartment.

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