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E-raamat: Inherited Bleeding Disorders in Women

Edited by , Edited by , Edited by (Professor in Obstetrics and Gynaecology, The Royal Free Foundation Hospital, Department of Obstetrics and Gynaecology and Katharine Dormandy Haemophilia and Thrombosis Centre, London, UK; and; Institute for Women's Health, University Col)
  • Formaat: EPUB+DRM
  • Ilmumisaeg: 06-Nov-2018
  • Kirjastus: Wiley-Blackwell
  • Keel: eng
  • ISBN-13: 9781119426066
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  • Formaat: EPUB+DRM
  • Ilmumisaeg: 06-Nov-2018
  • Kirjastus: Wiley-Blackwell
  • Keel: eng
  • ISBN-13: 9781119426066
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"This practical guide for clinicians is written by multi-disciplinary team of haematologists, obstetrician--gynaecologists, an anaesthetist and those involved in patient advocacy. It covers the gynaecological and obstetric issues for carriers of haemophilia, women with von Willebrand's disease, rare bleeding disorders and inherited platelet disorders. Inherited bleeding disorders have an adverse effect on the quality of life of afflicted women. Management of these women and their conditions is challenging and close collaboration between professionals in different specialities is needed. Written by international experts and leaders of clinical research Inherited Bleeding Disorders in Women covers; - Considerations for female carriers of haemophilia - Specific issues for females with von Willebrand's disease, the most common inherited bleeding disorder - Patients with rare bleeding disorders - Inherited platelet disorders This book includes recent advances in the science and genetics, novel and establishedtreatment strategies, and explores areas for future research. The women's perspective and the efforts of advocacy groups are also discussed in the final chapter. These features make this book a unique, comprehensive and multi-disciplinary resource. Primary: haematologists working in the fields of haemophilia, thrombosis and haemostasis Secondary: obstetricians, gynaecologists and neonatologists"--Provided by publisher.

The essential guide for understanding and treating women with inherited bleeding disorders, revised and updated

Now in its second edition, Inherited Bleeding Disorders in Women 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.

This important guide is written by a team of international experts who offer advice and practical suggestions for treating women with inherited bleeding disorders. Inherited Bleeding Disorders in Women 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:

  • Offers an updated guide for hematologists, obstetricans and gynecologists and other clinicians treating women with inherited bleeding disorders
  • Includes information for treating both common and rare bleeding disorders
  • Contains the most recent developments and advances in the field for the treatment and management of inherited bleeding disorders in women
  • Presents information from noted experts in the field
  • Offers a multidisciplinary approach to the topic

Written for hematologists, obstetricians and gynecologists and other clinicians working with women, Inherited Bleeding Disorders in Women 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. 

Preface to Second Edition ix
Preface to First Edition xi
List of Contributors xiii
1 Hematological Assessment of a Patient with an Inherited Bleeding Disorder 1(12)
Sue Pavord
Henna Wong
1.1 Introduction
1(1)
1.2 Normal Hemostasis
1(4)
1.3 Defects of Hemostasis
5(1)
1.4 Clinical Presentation of Bleeding
5(1)
1.5 Diagnosis
6(1)
1.6 Approach to a Female with a Bleeding History
6(4)
1.7 Summary
10(1)
References
11(2)
2 Bleeding Assessment Tools 13(16)
Sarah H. O'Brien
Paula D. James
2.1 Introduction
13(1)
2.2 Evolution of Vicenza-Based Bleeding Assessment Tools
13(9)
2.3 Women's Studies Using Vincenza-Based Bleeding Tools
22(1)
2.4 Tools to Evaluate Menstrual Blood Loss
23(2)
2.5 Young Women's Hematology and Bleeding Assessment Tools
25(1)
2.6 Clinical Utility of Bleeding Assessment Tools
25(1)
2.7 Conclusion
26(1)
References
27(2)
3 Physiology of Menstruation 29(16)
Jane J. Reavey
Jacqueline A. Maybin
Hilary O.D. Critchley
3.1 Introduction
29(1)
3.2 Normal Menstruation
29(1)
3.3 Neuroendocrine Hormones
30(1)
3.4 Menarche
31(1)
3.5 Ovarian Follicle Development and Endocrine Function
31(2)
3.6 Endometrium
33(1)
3.7 Secretory Phase
34(1)
3.8 Menstruation
35(2)
3.9 Control of Bleeding
37(1)
3.10 Endometrial Repair
38(2)
3.11 Proliferative Phase
40(1)
3.12 Summary
40(1)
References
41(4)
4 Gynecology 45(20)
Joanna S. Davies
Rezan A. Kadir
4.1 Introduction
45(3)
4.2 Heavy Menstrual Bleeding
48(10)
4.3 Management of Acute Episodes of Heavy Menstrual Bleeding
58(1)
4.4 Ovulation Bleeding
59(1)
4.5 Endometriosis
59(1)
4.6 Other Gynecological Conditions
60(1)
4.7 Conclusion
61(1)
References
61(4)
5 Carriers of Hemophilia A and Hemophilia B 65(18)
Roseline d'Oiron
5.1 Inheritance
65(1)
5.2 Screening for the Genetic Status of Carriers of Hemophilia
65(1)
5.3 Confusion Between Genetic and Coagulation Testing
66(1)
5.4 When to Perform Genetic Testing
67(1)
5.5 What Reasons might Contribute to Delayed Genetic Diagnosis of Carriership?
68(1)
5.6 Bleeding Disorders in Carriers of Hemophilia
68(5)
5.7 Quality of Life of Carriers of Hemophilia
73(1)
5.8 Carriers of Hemophilia A and B and Pregnancy
73(4)
5.9 How to Improve Care for Carriers of Hemophilia
77(2)
References
79(4)
6 Von Willebrand Disease 83(18)
Carolyn M. Millar
6.1 Introduction
83(1)
6.2 Structure and Function of Von Willebrand Factor
83(1)
6.3 Von Willebrand Factor Levels and Prevalence of Von Willebrand Disease
84(1)
6.4 VWF Levels, the Menstrual Cycle and Pregnancy
84(1)
6.5 Von Willebrand Disease Classification and Inheritance
85(2)
6.6 Clinical Presentation
87(1)
6.7 Menorrhagia and Postpartum Hemorrhage
87(1)
6.8 Diagnosis and Laboratory Testing
88(2)
6.9 Management of Von Willebrand Disease
90(1)
6.10 Management of Gynecological Bleeding
91(1)
6.11 Obstetric Management of Von Willebrand Disease
92(2)
6.12 Neonatal Management
94(1)
6.13 Cases
94(2)
References
96(5)
7 Factor XI Deficiency 101(16)
Bethan Myers
Rezan A. Kadir
7.1 Factor XI Structure and Function in Coagulation
101(1)
7.2 Incidence and Inheritance of Factor XI Deficiency
101(2)
7.3 Bleeding Manifestations and Diagnosis of Factor XI Deficiency
103(1)
7.4 Factor XI Deficiency and Gynecological Issues
103(2)
7.5 Factor XI Deficiency and Pregnancy
105(3)
7.6 Neuroaxial Analgesia and Anesthesia
108(1)
7.7 Management of Postpartum Period
108(1)
7.8 Treatment Options
109(2)
7.9 Neonatal Bleeding
111(1)
7.10 Conclusions and Recommendations
111(1)
References
112(5)
8 Rare Bleeding Disorders 117(16)
Danijela Mikovic
Marzia Menegatti
Flora Peyvandi
8.1 Introduction
117(1)
8.2 Clinical Symptoms
118(1)
8.3 Gynecological and Obstetrical Manifestations in Women with Rare Bleeding Disorders
119(3)
8.4 Laboratory Diagnosis
122(1)
8.5 Differential Diagnosis in Women with Menorrhagia
123(1)
8.6 Treatment
124(4)
8.7 Conclusion
128(1)
References
129(4)
9 Inherited Platelet Defects 133(20)
Mike Makris
Clare Samuelson
9.1 Introduction
133(1)
9.2 Normal Platelet Function
133(3)
9.3 Presentation
136(1)
9.4 Investigation
137(6)
9.5 Syndromic and Non-Syndromic Inherited Platelet Disorders with Recognized Causative Genes
143(1)
9.6 Prenatal Diagnosis
143(1)
9.7 Management
144(3)
9.8 Future Directions
147(1)
9.9 Case Histories
147(1)
9.10 Conclusion
148(1)
References
149(4)
10 Genetic and Laboratory Diagnosis 153(14)
Anne C. Goodeve
10.1 Introduction
153(1)
10.2 Phenotypic Analysis of Hemophilia A
154(2)
10.3 Phenotypic Analysis of Hemophilia B
156(1)
10.4 Phenotypic Analysis of von Willebrand Disease
157(1)
10.5 Phenotypic Analysis of Inherited Bleeding Disorders
157(3)
10.6 Genetic Analysis of Hemophilia A
160(2)
10.7 Genetic Analysis of Hemophilia B
162(1)
10.8 Genetic Analysis of von Willebrand Disease
163(1)
10.9 Guidelines
164(1)
10.10 Summary
164(1)
References
164(3)
11 Antenatal Diagnosis 167(24)
Rezan A. Kadir
Irena Hudecova
Claudia Chi
11.1 Introduction
167(1)
11.2 Genetic Counseling
167(3)
11.3 Prenatal Diagnosis
170(7)
11.4 Prenatal Diagnosis of Hemophilia
177(3)
11.5 Prenatal Diagnosis of von Willebrand Disease
180(1)
11.6 Prenatal Diagnosis of Rare Bleeding Disorders
180(1)
11.7 Preimplantation Genetic Diagnosis
181(2)
11.8 Views about and Experiences of Prenatal Diagnosis of Women in Families Affected with Inherited Bleeding Disorders
183(2)
11.9 Termination of Pregnancy
185(1)
References
186(5)
12 Analgesia and Anesthesia for Pregnant Women with Inherited Bleeding Disorders 191(14)
Anne-Sophie Bouthors
Adrian England
Rezan A. Kadir
12.1 Introduction
191(1)
12.2 Non-Pharmacological Methods
192(1)
12.3 Pharmacological Methods
192(9)
12.4 Conclusion
201(1)
References
202(3)
13 The Newborn 205(20)
Manuel Carcao
Vanessa Bouskill
13.1 Introduction
205(1)
13.2 Developmental Hemostasis
205(3)
13.3 Laboratory Hemostatic Evaluation of the Neonate
208(1)
13.4 When to Suspect a Congenital Bleeding Disorder in a Newborn
209(4)
13.5 Congenital Bleeding Disorders and Their Presentation in Newborns
213(5)
13.6 Management of Bleeding in Neonates with Congenital Bleeding Disorders
218(3)
13.7 Conclusion
221(1)
References
222(3)
14 Women with Inherited Bleeding Disorders in Different Cultural Settings 225(10)
Tahira Zafar
Jameela Sathar
Ali T. Taher
Fadi G. Mirza
Christine A. Lee
14.1 Introduction
225(1)
14.2 Pakistan
225(2)
14.3 Malaysia
227(2)
14.4 Lebanon
229(2)
14.5 Discussion
231(1)
References
232(3)
Index 235
Rezan A. Kadir, 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.

Paula D. James, Professor in the Department of Medicine, Queen's University, Kingston, Canada.

Christine A. Lee, Emeritus Professor of Hemophilia, University College London, London, UK.