Von Willebrand Disease: Basic and Clinical Aspects provides an insight into all aspects of the condition. Since its discovery, von Willebrand disease has been extensively studied and the causative factor deficiency, the understanding of the condition and its treatment has greatly improved. This book summarizes recent research and will help to optimize the management of patients with von Willebrand disease. This valuable book describes the important and complex role of von Willebrand factor in hemostasis and thrombosis. In addition to the current understanding of its molecular biology, this book gives particular focus to the association between genetic variants of von Willebrand factor and different von Willebrand disease phenotypes. It also reviews the important area of the obstetric and gynecological manifestations of von Willebrand disease, as well as the treatment of acute bleeding.
Written by an international team of contributors it describes the progress in, and difficulties of diagnosing phenotypes and genotypes. Molecular diagnosis of type 1, type 2 and its subgroups, and type 3 von Willebrand disease are presented as well as discussion of the potential future role of gene therapy.
Von Willebrand Disease: Basic and Clinical Aspects is a valuable resource for hematologists in practice and in training, and specialists in thrombosis and hemostasis.
Arvustused
"This book certainly helped to enhance my knowledge about von Willebrand disease and even prompted me to present a review of the disease to my colleagues. It is excellently written, appeals to a broad category of readers, and exceeds its stated objectives. I would definitely recommend the book to colleagues." (Doody's, 23 September 2011)
| Contributors |
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vii | |
| Foreword |
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xi | |
| Preface |
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xiii | |
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1 Historical perspective on von Willebrand disease |
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1 | (6) |
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2 Biosynthesis and organization of von Willebrand factor |
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7 | (23) |
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3 Von Willebrand factor structure and function |
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30 | (19) |
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4 Modulation of von Willebrand factor by ADAMTS13 |
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49 | (14) |
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5 Animal models in von Willebrand disease |
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63 | (11) |
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6 Classification of von Willebrand disease |
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74 | (12) |
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Maria Fernanda Lopez-Fernandez |
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7 The epidemiology of von Willebrand disease |
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86 | (5) |
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8 Clinical aspects of von Willebrand disease: bleeding history |
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91 | (9) |
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9 Laboratory diagnosis of von Willebrand disease: the phenotype |
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100 | (14) |
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10 Molecular diagnosis of von Willebrand disease: the genotype |
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114 | (13) |
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11 Clinical, laboratory, and molecular markers of type 1 von Willebrand disease |
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127 | (10) |
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12 Clinical, laboratory, and molecular markers of type 2 von Willebrand disease |
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137 | (11) |
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13 Clinical, laboratory, and molecular markers of type 3 von Willebrand disease |
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148 | (18) |
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14 Pediatric aspects of von Willebrand disease |
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166 | (8) |
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15 Women with von Willebrand disease |
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174 | (12) |
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16 On the use of desmopressin in von Willebrand disease |
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186 | (14) |
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17 The use of plasma-derived concentrates |
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200 | (7) |
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18 Prophylaxis in von Willebrand disease |
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207 | (7) |
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19 Pathophysiology, epidemiology, diagnosis, and treatment of acquired von Willebrand syndrome |
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214 | (17) |
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20 Gene therapy for von Willebrand disease |
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231 | (14) |
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| Index |
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245 | |
Augusto B. Federici, University of Milan, Milan, Italy Christine A. Lee, Oxford Haemophilia & Thrombosis Centre, Oxford, UK
Erik E. Berntorp, Lund University, Malmö, Sweden
David Lillicrap, Queens University, Kingston, ON, Canada
Robert R. Montgomery, Medical College of Wisconsin; Milwaukee, WI, USA
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