Hemophilia and Von Willebrand Disease: Factor VIII and Von Willebrand Factor (Academic Press)

__Hemophilia and Von Willebrand Disease: Factor VIII and Von Willebrand Factor__ serves as a must-have reference on the important role these essential blood-clotting proteins play in research and clinical medicine. Clinicians and researchers face the daily challenge of staying current on the vast amounts of research that is now generated. The reference to Janus in the title refers to the two roles of the Factor VIII/Von Willebrand Factor Complex: initiation of coagulation and propagation of clot formation. The complex prevents bleeding in hemophilia and Von Willebrand disease but also augments arterial and venous thrombosis. * Presents one source of information on Hemophilia and Von Willebrand Disease, as well as Factor VIII and Von Willebrand Factor, eliminating the search through hundreds of journal articles * Combines the multi-disciplinary research that is generated from Factor VIII/Von Willebrand Factor – hematology, drug discovery, genetics, cell biology, and oncology * Delves into unanswered questions and future directions of this important blood-clotting complex Book Copyright_2018_Hemophilia-and-Von-Willebrand-Disease Copyright Acknowledgments_2018_Hemophilia-and-Von-Willebrand-Disease Acknowledgments Author-Biography_2018_Hemophilia-and-Von-Willebrand-Disease Author Biography Front-Matter_2018_Hemophilia-and-Von-Willebrand-Disease Title Page Introduction_2018_Hemophilia-and-Von-Willebrand-Disease Introduction FVIII/VWF: The Janus of Hemostasis Preface_2018_Hemophilia-and-Von-Willebrand-Disease Preface References Chapter-1---Historical-Background-of-Blood_2018_Hemophilia-and-Von-Willebran Chapter 1: Historical Background of Blood Coagulation Factor VIII and Hemophilia A Von Willebrand Factor and Von Willebrand Disease Summary References Recommended Reading Chapter-2---Hemostasis-and-FVIII_2018_Hemophilia-and-Von-Willebrand-Disease Chapter 2: Hemostasis and FVIII Normal Hemostasis Vasoconstriction Phase Initiation Phase Amplification Phase Propagation Phase Alternative Pathway Termination Phase Fibrinolysis Hemostasis in the Absence of FVIII Laboratory Assessment of Hemostasis Summary References Recommended Reading Chapter-3---FVIII-Anatomy-and-Physiol_2018_Hemophilia-and-Von-Willebrand-Dis Chapter 3: FVIII Anatomy and Physiology Isolation and Structure Relationship to the Von Willebrand Factor Cell of Origin, Secretion, and Clearance Activation and Inactivation Summary References Recommended Reading Chapter-4---FVIII-Genetics_2018_Hemophilia-and-Von-Willebrand-Disease Chapter 4: FVIII Genetics The FVIII Gene FVIII Mutations Missense/Nonsense Mutations Complex Rearrangements (Intron-22 Inversions) Miscellaneous Mutations Retrotransposons Functional Classification of Mutations FVIII Mutations in Women Application of Genetic Analyses Carrier Detection and Prenatal Diagnosis Summary References Recommended Reading Chapter-5---Congenital-Deficiency-of-Factor-_2018_Hemophilia-and-Von-Willebr Chapter 5: Congenital Deficiency of Factor VIII: Hemophilia A Historical Aspects Epidemiology Clinical Manifestations of Hemophilia Hemarthroses Muscle Hemorrhages Abdominal Hemorrhages and Pseudotumors Central Nervous System Bleeding Bleeding in Carriers of Hemophilia Disorders Other Than Bleeding Diagnosis of Hemophilia Differential Diagnosis Summary References Recommended Reading Chapter-6---FVIII-Concentrates_2018_Hemophilia-and-Von-Willebrand-Disease Chapter 6: FVIII Concentrates The First FVIII Concentrates Cryoprecipitate Plasma-Derived Concentrates Recombinant Concentrates Concentrates Free of Extraneous Human and Animal Proteins Longer-Acting Concentrates A Concentrate Prepared From a Human Cell Line Adverse Events With Concentrates Improving Concentrates Summary References Chapter-7---General-Management-of-Hemop_2018_Hemophilia-and-Von-Willebrand-D Chapter 7: General Management of Hemophilia Management of Acute Hemorrhages On-Demand Treatment Perioperative Management Prophylaxis Lifestyle Measures Future Approaches Novel Bypassing Agents Gene Therapy Summary References Recommended Reading Chapter-8---Antibodies-to-FVIII_2018_Hemophilia-and-Von-Willebrand-Disease Chapter 8: Antibodies to FVIII Alloantibodies Risk Factors for Alloantibody Development (Table 8.1) Management of Hemophiliacs Developing Alloantibodies Autoantibodies References Chapter-9---The-Von-Willebrand-Fact_2018_Hemophilia-and-Von-Willebrand-Disea Chapter 9: The Von Willebrand Factor The VWF Gene The VWF Protein Clearance of VWF Functions of VWF Summary References Recommended Reading Chapter-10---Von-Willebrand-Disease--Classifi_2018_Hemophilia-and-Von-Willeb Chapter 10: Von Willebrand Disease: Classification and Diagnosis Epidemiology Classification Type 1 Type 2 Type 3 Clinical and Laboratory Evaluation Diagnosis Diagnostic Features of VWD Subtypes Gene Mutations in VWD Subtypes Differential Diagnosis Summary References Recommended Reading Chapter-11---Treatment-of-Von-Willebrand_2018_Hemophilia-and-Von-Willebrand- Chapter 11: Treatment of Von Willebrand Disease Desmopressin VWF Concentrates Adjunctive Therapies Management of Bleeding by VWD Type Low VWD and Type 1 VWD Type 2 VWD Type 3 VWD Prophylaxis Other Management Issues Gastrointestinal Bleeding Heavy Menstrual Bleeding Pregnancy and Postpartum Future Approaches: Gene Therapy Summary References Recommended Reading Chapter-12---Acquired-Von-Willebrand-Sy_2018_Hemophilia-and-Von-Willebrand-D Chapter 12: Acquired Von Willebrand Syndrome Immunologically Mediated Loss of VWF Cardiovascular Disease Myeloproliferative Disorders Glycogen Storage Disease Miscellaneous Disorders Summary References Recommended Reading Chapter-13---Factor-VIII-and-Thrombo_2018_Hemophilia-and-Von-Willebrand-Dise Chapter 13: Factor VIII and Thrombosis Venous Thrombosis Arterial Thrombosis Treatment of Thrombosis in Patients With FVIII Deficiency Summary References Recommended Reading Chapter-14---Von-Willebrand-Factor-and-T_2018_Hemophilia-and-Von-Willebrand- Chapter 14: Von Willebrand Factor and Thrombosis Atherothrombosis Epidemiologic Studies Venous Thrombosis Atrial Fibrillation/Embolic Stroke Modification of VWF Summary References Recommended Reading Chapter-15---Factor-VIII-Von-Willebrand-Factor-_2018_Hemophilia-and-Von-Will Chapter 15: Factor VIII/Von Willebrand Factor: The Janus of Coagulation Origin and Clearance of the FVIII/VWF Complex Participation in Hemostasis Role in Bleeding Disorders Role in Thrombotic Disorders The FVIII/VWF Complex in the Treatment of Bleeding Disorders Areas for Future Investigation Summary References Recommended Reading Index_2018_Hemophilia-and-Von-Willebrand-Disease Index A B C D E F G H I J K L M N O P R S T U V W X