Title Congenital bleeding disorders : diagnosis and management / Akbar Dorgalaleh, editor

Published Cham, Switzerland : Springer, 2018

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Description 1 online resource : color illustrations Contents 880-01 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 880-01/(S 3.2.5 Intracellular Storage and Secretion of VWF3.2.6 Biological Activities of von Willebrand Factor; 3.2.7 Stabilization and Transport of Coagulation Factor VIII; 3.3 Platelet Adhesion; 3.3.1 Interaction of von Willebrand Factor with Extracellular Protein; 3.3.2 Interaction of von Willebrand Factor with Glycoprotein Ibα; 3.3.3 Interaction of von Willebrand Factor with Integrin αIIbβ3 (GPIIb/IIIa); 3.4 von Willebrand Disease; 3.4.1 Type 1 von Willebrand Disease; 3.4.2 Type 2 von Willebrand Disease; 3.4.2.1 Type 2A von Willebrand Disease; 3.4.2.2 Type 2B von Willebrand Disease 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 Bibliography Includes bibliographical references Notes Online resource; title from PDF title page (EBSCO, viewed July 31, 2018) Subject Blood coagulation disorders. Blood -- Diseases. Hematologic Diseases Blood Coagulation Disorders Paediatric medicine. Haematology. HEALTH & FITNESS -- Diseases -- General. MEDICAL -- Clinical Medicine. MEDICAL -- Diseases. MEDICAL -- Evidence-Based Medicine. MEDICAL -- Internal Medicine. Blood coagulation disorders Blood -- Diseases Form Electronic book Author Dorgalaleh, Akbar, editor ISBN 9783319767239 3319767232

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