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  Congenital bleeding disorders : diagnosis and management

  Akbar Dorgalaleh, editor.

  Summary: This book describes in detail the clinical presentation, diagnosis, and management of a wide range of congenital bleeding disorders. It will assist readers in overcoming the significant challenges involved in clinical and laboratory diagnosis and in providing effective clinical care that makes optimal use of new products, including recombinant factor concentrate. The coverage ranges from hemophilia A and B and von Willebrand disease to rare bleeding disorders such as congenital factor V, factor X, factor XI, and factor XIII deficiency and inherited platelet function disorders. The exceptional attention to rarer conditions is of particular importance given the considerable risk of overlooking them during diagnosis, with potential consequences for disease-related morbidity and mortality. The authors are acknowledged specialists in the field from across the world who have particular expertise in the disorder that they discuss. The book will be of value to hematologists, oncologists, pediatricians, laboratory specialists and technicians, general physicians, and trainees. .
  
  Contents:
  Part I: Common bleeding disorders
  1. Von Willebrand disease
  2. Hemophilia A (congenital factor VIII deficiency)
  3. Hemophilia B (congenital factor IX deficiency) Part II: Rare bleeding disorders
  4. Congenital factor I (fibrinogen) disorders
  5. Congenital factor II deficiency
  6. Congenital factor V deficiency
  7. Combined coagulation factor deficiencies
  8. Congenital factor VII deficiency
  9. Congenital factor X deficiency
  10. Congenital factor XI deficiency
  11. Congenital factor XIII deficiency Part III: Inherited platelet function disorders
  12. Glanzmann thrombasthenia
  13. Bernard–Soulier syndrome
  14. Gray platelet syndrome
  15. Quebec platelet disorder. .

  Digital Access Springer 2018
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• Article

  Biosynthesis of lipid A. Enzymatic incorporation of 3-deoxy-D-mannooctulosonate into a precursor of lipid A in Salmonella typhimurium.

  Munson RS, Rasmussen NS, Osborn MJ.

  J Biol Chem. 1978 Mar 10;253(5):1503-11.

  The cell envelope fraction of Salmonella typhimurium contains an enzyme system which catalyzes transfer of 3-deoxyoctulosonate (KDO) from CMP-KDO to an incomplete, KDO-deficient precursor of lipid A. The enzyme system is firmly membrane-bound, but has been solubilized by treatment with nonionic detergent at alkaline pH and partially purified. Both the particulate and partially purified fractions catalyzed formation of a single reaction product containing 2 residues of KDO. Periodate oxidation of the purified product permitted tentative identification of the KDO disaccharide structure as KDO2-4KDO.

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  PubMed