Textbook of Hemophilia, Second Edition

✍ Scribed by Christine A. Lee, Erik E. Berntorp, W. Keith Hoots

• Publisher: Wiley-Blackwell
• Year: 2010
• Tongue: English
• Leaves: 482
• Edition: 2
• Category: Library

✦ Synopsis

The Textbook of Hemophilia has become a definitive resource for all those managing hemophilia patients. It covers all the common and rare bleeding disorders, both in terms of clinical management as well as the genetic, laboratory, financial and psychological aspects.This second edition covers all the latest developments in the field of hemophilia, with new chapters on:the genetic and molecular basis of inherited blood disordershow to manage adolescent and older patientsemergency medicine and inherited blood disordersnational hemophilia databasesDrawing on the vast experience of the authors, the aim of this textbook remains the same - to improve the care of patients suffering from hemophilia. The book is full of detailed guidance and advice on everyday clinical questions making it invaluable to all trainee and practicing hematologists.

✦ Table of Contents

Textbook of Hemophilia, Second Edition......Page 5
Contents......Page 7
Contributors......Page 9
Historical introduction......Page 15
1: Overview of hemostasis......Page 21
2: Cellular processing of factor VIII and factor IX......Page 27
3: Molecular basis of hemophilia A......Page 38
4: Phenotypic–genotypic relationship......Page 47
5: Prophylaxis......Page 52
6: Continuous infusion of coagulation products in hemophilia......Page 58
7: Inhibitors to factor VIII—immunology......Page 66
8: Genetic and environmental risk factors for inhibitor development......Page 71
9: Inhibitors to factor VIII: mild and moderate hemophilia......Page 76
10: Inhibitors to factor VIII/IX: immune tolerance......Page 80
11: Inhibitors to factor VIII: treatment of acute bleeds......Page 89
12: Acquired inhibitors to factor VIII......Page 95
13: Hemophilia B—molecular basis......Page 102
14: Factor IX inhibitors in hemophilia B......Page 108
15: Treatment of inhibitors in hemophilia B......Page 111
16: Pharmacokinetics......Page 118
17: The neonate with hemophilia......Page 125
18: Work-up of a bleeding child......Page 132
19: Care of the child with hemophilia......Page 141
20: Adolescence......Page 147
21: Hemophilia and medicine in old age......Page 152
22: Products used to treat hemophilia: recombinant products......Page 160
23: Plasma-derived coagulation factor concentrates......Page 171
24: Products used to treat hemophilia: recombinant factor VIIa......Page 177
25: Products used to treat hemophilia: dosing......Page 183
26: Products used to treat hemophilia: regulation......Page 188
27: Joint replacement......Page 194
28: Synoviorthesis in hemophilia......Page 200
29: Pseudotumors in patients with hemophilia......Page 205
30: Imaging modalities for assessment of hemophilic arthropathy......Page 209
31: Physiotherapy in the management of hemophilia......Page 218
32: Clinimetric instruments in hemophilia......Page 225
33: Hepatitis and hemophilia......Page 233
34: Transfusion-transmitted disease: emerging infections......Page 240
35: Hemophilia gene therapy: an overview......Page 244
36: Gene therapy trials in hemophilia A and B......Page 249
37: Gene therapy: molecular engineering of factor VIII and factor IX......Page 257
38: Laboratory assays in hemophilia......Page 265
39: Standardization of assays in hemophilia......Page 272
40: Global laboratory assays in hemophilia......Page 281
41: Obstetrics and gynecology: hemophilia......Page 287
42: von Willebrand disease: molecular aspects......Page 296
43: von Willebrand disease: epidemiology......Page 304
44: von Willebrand disease: biologic diagnosis......Page 312
45: Classification and clinical aspects of von Willebrand disease......Page 320
46: Women and von Willebrand disease......Page 327
47: Treatment of von Willebrand disease: desmopressin......Page 334
48: Treatment of von Willebrand disease: therapeutic concentrates......Page 338
49: Factor II......Page 346
50: Factor V and combined factor V and VIII deficiencies......Page 350
51: Congenital factor VII deficiency......Page 359
52: Factor X and factor X deficiency......Page 366
53: Factor XI deficiency......Page 373
54: Factor XIII deficiency......Page 380
55: Fibrinogen......Page 387
56: Miscellaneous bleeding disorders......Page 394
57: Emergency management of hemophilia......Page 412
58: Quality of life in hemophilia......Page 419
59: The economics of hemophilia treatment......Page 427
60: National hemophilia databases......Page 433
61: Comprehensive care and delivery of care: the developed world......Page 438
62: Comprehensive care and delivery of care in hemophilia: the developing world......Page 447
63: Comprehensive care and delivery of care: the global perspective......Page 453
Index......Page 461
colour plates......Page 163