Details

Inherited Bleeding Disorders in Women


Inherited Bleeding Disorders in Women


2. Aufl.

von: Rezan A. Kadir, Paula D. James, Christine A. Lee

179,99 €

Verlag: Wiley-Blackwell
Format: EPUB
Veröffentl.: 06.11.2018
ISBN/EAN: 9781119426066
Sprache: englisch
Anzahl Seiten: 264

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Beschreibungen

<p><b>The essential guide for understanding and treating women with inherited bleeding disorders, revised and updated</b></p> <p>Now in its second edition,<i> Inherited Bleeding Disorders in Women</i> includes the most recent developments and research in the field. This important guide offers the most current information available for the effective management of these complex and difficult to diagnose disorders. Treating women with inherited bleeding disorders can be challenging and requires close collaboration among practitioners in different specialties.</p> <p>This important guide is written by a team of international experts who offer advice and practical suggestions for treating women with inherited bleeding disorders. <i>Inherited Bleeding Disorders in Women</i> comprehensively covers obstetric and gynecological issues for carriers of hemophilia, women with von Willebrand disease, rare bleeding disorders and inherited platelet disorders. This important resource:</p> <ul> <li>Offers an updated guide for hematologists, obstetricans and gynecologists and other clinicians treating women with inherited bleeding disorders</li> <li>Includes information for treating both common and rare bleeding disorders</li> <li>Contains the most recent developments and advances in the field for the treatment and management of inherited bleeding disorders in women</li> <li>Presents information from noted experts in the field</li> <li>Offers a multidisciplinary approach to the topic</li> </ul> <p>Written for hematologists, obstetricians and gynecologists and other clinicians working with women, <i>Inherited Bleeding Disorders in Women</i> has been fully revised and updated and continues to serve as a trusted guide for the management and treatment of women with inherited bleeding disorders. </p>
<p>Preface to Second Edition ix</p> <p>Preface to First Edition xi</p> <p>List of Contributors xiii</p> <p><b>1 Hematological Assessment of a Patient with an Inherited Bleeding Disorder 1 <br /></b><i>Sue Pavord and Henna Wong</i></p> <p>1.1 Introduction 1</p> <p>1.2 Normal Hemostasis 1</p> <p>1.3 Defects of Hemostasis 5</p> <p>1.4 Clinical Presentation of Bleeding 5</p> <p>1.5 Diagnosis 6</p> <p>1.6 Approach to a Female with a Bleeding History 6</p> <p>1.7 Summary 10</p> <p>References 11</p> <p><b>2 Bleeding Assessment Tools 13<br /></b><i>Sarah H. O’Brien and Paula D. James</i></p> <p>2.1 Introduction 13</p> <p>2.2 Evolution of Vicenza‐Based Bleeding Assessment Tools 13</p> <p>2.3 Women’s Studies Using Vincenza‐Based Bleeding Tools 22</p> <p>2.4 Tools to Evaluate Menstrual Blood Loss 23</p> <p>2.5 Young Women’s Hematology and Bleeding Assessment Tools 25</p> <p>2.6 Clinical Utility of Bleeding Assessment Tools 25</p> <p>2.7 Conclusion 26</p> <p>References 27</p> <p><b>3 Physiology of Menstruation 29<br /></b><i>Jane J. Reavey, Jacqueline A. Maybin, and Hilary O.D. Critchley</i></p> <p>3.1 Introduction 29</p> <p>3.2 Normal Menstruation 29</p> <p>3.3 Neuroendocrine Hormones 30</p> <p>3.4 Menarche 31</p> <p>3.5 Ovarian Follicle Development and Endocrine Function 31</p> <p>3.6 Endometrium 33</p> <p>3.7 Secretory Phase 34</p> <p>3.8 Menstruation 35</p> <p>3.9 Control of Bleeding 37</p> <p>3.10 Endometrial Repair 38</p> <p>3.11 Proliferative Phase 40</p> <p>3.12 Summary 40</p> <p>References 41</p> <p><b>4 Gynecology 45<br /></b><i>Joanna S. Davies and Rezan A. Kadir</i></p> <p>4.1 Introduction 45</p> <p>4.2 Heavy Menstrual Bleeding 48</p> <p>4.3 Management of Acute Episodes of Heavy Menstrual Bleeding 58</p> <p>4.4 Ovulation Bleeding 59</p> <p>4.5 Endometriosis 59</p> <p>4.6 Other Gynecological Conditions 60</p> <p>4.7 Conclusion 61</p> <p>References 61</p> <p><b>5 Carriers of Hemophilia A and Hemophilia B 65<br /></b><i>Roseline d’Oiron</i></p> <p>5.1 Inheritance 65</p> <p>5.2 Screening for the Genetic Status of Carriers of Hemophilia 65</p> <p>5.3 Confusion Between Genetic and Coagulation Testing 66</p> <p>5.4 When to Perform Genetic Testing 67</p> <p>5.5 What Reasons might Contribute to Delayed Genetic Diagnosis of Carriership? 68</p> <p>5.6 Bleeding Disorders in Carriers of Hemophilia 68</p> <p>5.7 Quality of Life of Carriers of Hemophilia 73</p> <p>5.8 Carriers of Hemophilia A and B and Pregnancy 73</p> <p>5.9 How to Improve Care for Carriers of Hemophilia 77</p> <p>References 79</p> <p><b>6 Von Willebrand Disease 83<br /></b><i>Carolyn M. Millar</i></p> <p>6.1 Introduction 83</p> <p>6.2 Structure and Function of Von Willebrand Factor 83</p> <p>6.3 Von Willebrand Factor Levels and Prevalence of Von Willebrand Disease 84</p> <p>6.4 VWF Levels, the Menstrual Cycle and Pregnancy 84</p> <p>6.5 Von Willebrand Disease Classification and Inheritance 85</p> <p>6.6 Clinical Presentation 87</p> <p>6.7 Menorrhagia and Postpartum Hemorrhage 87</p> <p>6.8 Diagnosis and Laboratory Testing 88</p> <p>6.9 Management of Von Willebrand Disease 90</p> <p>6.10 Management of Gynecological Bleeding 91</p> <p>6.11 Obstetric Management of Von Willebrand Disease 92</p> <p>6.12 Neonatal Management 94</p> <p>6.13 Cases 94</p> <p>References 96</p> <p><b>7 Factor XI Deficiency 101<br /></b><i>Bethan Myers and Rezan A. Kadir</i></p> <p>7.1 Factor XI Structure and Function in Coagulation 101</p> <p>7.2 Incidence and Inheritance of Factor XI Deficiency 101</p> <p>7.3 Bleeding Manifestations and Diagnosis of Factor XI Deficiency 103</p> <p>7.4 Factor XI Deficiency and Gynecological Issues 103</p> <p>7.5 Factor XI Deficiency and Pregnancy 105</p> <p>7.6 Neuroaxial Analgesia and Anesthesia 108</p> <p>7.7 Management of Postpartum Period 108</p> <p>7.8 Treatment Options 109</p> <p>7.9 Neonatal Bleeding 111</p> <p>7.10 Conclusions and Recommendations 111</p> <p>References 112</p> <p><b>8 Rare Bleeding Disorders 117<br /></b><i>Danijela Mikovic, Marzia Menegatti, and Flora Peyvandi</i></p> <p>8.1 Introduction 117</p> <p>8.2 Clinical Symptoms 118</p> <p>8.3 Gynecological and Obstetrical Manifestations in Women with Rare Bleeding Disorders 119</p> <p>8.4 Laboratory Diagnosis 122</p> <p>8.5 Differential Diagnosis in Women with Menorrhagia 123</p> <p>8.6 Treatment 124</p> <p>8.7 Conclusion 128</p> <p>References 129</p> <p><b>9 Inherited Platelet Defects 133<br /></b><i>Mike Makris and Clare Samuelson</i></p> <p>9.1 Introduction 133</p> <p>9.2 Normal Platelet Function 133</p> <p>9.3 Presentation 136</p> <p>9.4 Investigation 137</p> <p>9.5 Syndromic and Non‐Syndromic Inherited Platelet Disorders with Recognized Causative Genes 143</p> <p>9.6 Prenatal Diagnosis 143</p> <p>9.7 Management 144</p> <p>9.8 Future Directions 147</p> <p>9.9 Case Histories 147</p> <p>9.10 Conclusion 148</p> <p>References 149</p> <p><b>10 Genetic and Laboratory Diagnosis 153<br /></b><i>Anne C. Goodeve</i></p> <p>10.1 Introduction 153</p> <p>10.2 Phenotypic Analysis of Hemophilia A 154</p> <p>10.3 Phenotypic Analysis of Hemophilia B 156</p> <p>10.4 Phenotypic Analysis of von Willebrand Disease 157</p> <p>10.5 Phenotypic Analysis of Inherited Bleeding Disorders 157</p> <p>10.6 Genetic Analysis of Hemophilia A 160</p> <p>10.7 Genetic Analysis of Hemophilia B 162</p> <p>10.8 Genetic Analysis of von Willebrand Disease 163</p> <p>10.9 Guidelines 164</p> <p>10.10 Summary 164</p> <p>References 164</p> <p><b>11 Antenatal Diagnosis 167<br /></b><i>Rezan A. Kadir, Irena Hudecova, and Claudia Chi</i></p> <p>11.1 Introduction 167</p> <p>11.2 Genetic Counseling 167</p> <p>11.3 Prenatal Diagnosis 170</p> <p>11.4 Prenatal Diagnosis of Hemophilia 177</p> <p>11.5 Prenatal Diagnosis of von Willebrand Disease 180</p> <p>11.6 Prenatal Diagnosis of Rare Bleeding Disorders 180</p> <p>11.7 Preimplantation Genetic Diagnosis 181</p> <p>11.8 Views about and Experiences of Prenatal Diagnosis of Women in Families Affected with Inherited Bleeding Disorders 183</p> <p>11.9 Termination of Pregnancy 185</p> <p>References 186</p> <p><b>12 Analgesia and Anesthesia for Pregnant Women with Inherited Bleeding Disorders 191<br /></b><i>Anne‐Sophie Bouthors, Adrian England, and Rezan A. Kadir</i></p> <p>12.1 Introduction 191</p> <p>12.2 Non‐Pharmacological Methods 192</p> <p>12.3 Pharmacological Methods 192</p> <p>12.4 Conclusion 201</p> <p>References 202</p> <p><b>13 The Newborn 205<br /></b><i>Manuel Carcao and Vanessa Bouskill</i></p> <p>13.1 Introduction 205</p> <p>13.2 Developmental Hemostasis 205</p> <p>13.3 Laboratory Hemostatic Evaluation of the Neonate 208</p> <p>13.4 When to Suspect a Congenital Bleeding Disorder in a Newborn 209</p> <p>13.5 Congenital Bleeding Disorders and Their Presentation in Newborns 213</p> <p>13.6 Management of Bleeding in Neonates with Congenital Bleeding Disorders 218</p> <p>13.7 Conclusion 221</p> <p>References 222</p> <p><b>14 Women with Inherited Bleeding Disorders in Different Cultural Settings 225<br /></b><i>Tahira Zafar, Jameela Sathar, Ali T. Taher, Fadi G. Mirza, and Christine A. Lee</i></p> <p>14.1 Introduction 225</p> <p>14.2 Pakistan 225</p> <p>14.3 Malaysia 227</p> <p>14.4 Lebanon 229</p> <p>14.5 Discussion 231</p> <p>References 232</p> <p>Index 235</p>
<p><b>Rezan A. Kadir,</b> Professor in the Department of Obstetrics and Gynaecology and Katharine Dormandy Haemophilia and Thrombosis Centre, The Royal Free Foundation Hospital and Institute for Women's Health, University College London, London, UK. <p><b>Paula D. James,</b> Professor in the Department of Medicine, Queen's University, Kingston, Canada. <p><b>Christine A. Lee,</b> Emeritus Professor of Hemophilia, University College London, London, UK.
<p><b>The Essential Guide for Understanding and Treating Women with Inherited Bleeding Disorders, Revised and Updated</b></p> <p>Treating women with inherited bleeding disorders can be challenging and requires close collaboration among practitioners in different specialties. Now in its second edition, <i>Inherited Bleeding Disorders in Women</i> includes the most recent developments and research in the field, and offers the most current information available for the effective management of these complex and difficult-to-diagnose disorders.</p> <p>Written by a team of international experts who offer advice and practical suggestions for treating women with inherited bleeding disorders. <i>Inherited Bleeding Disorders in Women </i>comprehensively covers obstetric and gynecological issues for carriers of hemophilia, women with von Willebrand disease, rare bleeding disorders, and inherited platelet disorders. This important resource:</p> <ul> <li>Offers an updated guide for hematologists, obstetricans and gynecologists, and other clinicians treating women with inherited bleeding disorders,</li> <li>Includes information for treating both common and rare bleeding disorders,</li> <li>Contains the most recent developments and advances in the field for the management of inherited bleeding disorders in women,</li> <li>Presents information from noted experts in the field, and</li> <li>Offers a multidisciplinary approach to the topic.</li> </ul> <p>Essential reading for hematologists, obstetricians and gynecologists, and other clinicians working with women, <i>Inherited Bleeding Disorders in Women</i> has been fully revised and updated and continues to serve as a trusted guide for the management of women with inherited bleeding disorders.</p>

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