von Willebrand disease and von Willebrand factor: current aspects of diagnosis and treatment
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| Main Authors: | , |
|---|---|
| Format: | Book |
| Language: | English |
| Published: |
Bremen [u.a.]
UNI-MED-Verl.
2008
|
| Edition: | 1. Aufl. |
| Series: | UNI-MED science
|
| Online Access: | Inhaltsverzeichnis |
| Physical Description: | 93 S. Ill., zahlr. graph. Darst. |
| ISBN: | 9783837410099 9781848151314 |
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Record in the Search Index
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| adam_text | Titel: Von Willebrand Disease and von Willebrand Factor
Autor: Schneppenheim, Reinhard
Jahr: 2008
Contents
11 History 12
HI Clinical Symptoms and Genetics 16
2.1. Clinical symptoms of von Willebrand disease (VWD) 16
2.2. Clinical genetics of VWD 18
^ von Willebrand Factor (VWF) 22
3.1. Biosynthesis 22
3.2. Structure-function relationships 23
3.3. Molecular genetics 25
3.4. Role in hemostasis 27
3.4.1. Primary hemostasis 27
3.4.2. Secondary hemostasis 30
If Classification and Pathogenesis 32
4.1. Introduction 32
4.2. Critical appraisal of the current classification 33
4.2.1. Type 1 VWD (VWD 1) 33
4.2.1.1. Pathogenesis 34
4.2.2. Type 3 VWD (VWD 3) 34
4.2.3. Type 2 VWD (VWD 2) 35
4.2.3.1. Pathogenesis 35
4.2.3.2. Type 2A VWD 36
4.2.3.3. Type 2B VWD (VWD 2B) 37
4.2.3.4. Type 2M VWD (VWD 2M) 37
4.2.3.5. VWD type Normandy (VWD 2N) 37
m Diagnosis 40
5.1. Biochemical parameters 40
5.1.1. Investigation procedure 40
5.1.2. Screening diagnostic tests 41
5.1.2.1. Bleeding time 41
5.1.2.2. Filter methods with high shear stress 42
5.1.2.3. Activated partial thromboplastin time (aPTT) 42
5.1.2.4. Adhesion/retention 43
5.1.2.5. Platelet count 43
5.1.3. Extended diagnostic tests 43
5.1.3.1. Assay of the FVIII/VWF complex 43
5.1.3.2. Factor VIII (FVIII) 43
5.1.3.3. VWF Antigen (VWF:Ag) 43
5.1.3.4. Ristocetin cofactor activity (VWF:RCo) 44
5.1.4. Special diagnostic tests 44
5.1.4.1. Collagen binding capacity (VWF:CB) 44
5.1.4.2. Ristocetin-induced aggregation in platelet-rich plasma (RIPA) 45
5.1.4.3. Epitope-specific VWF:Ag ELISA 46
5.1.4.4. Botrocetin-induced aggregation in platelet-rich plasma (BIPA) 46
5.1.4.5. Binding studies with isolated platelets 46
5.1.4.6. VWF in platelets 46
8 Contents
5.1.4.7. VW:Ag II (propeptide) 47
5.1.4.8. Qualitative changes in VWF 47
5.1.5. Diagnosis in neonates and small children 49
5.1.6. Diagnosis in pregnancy 50
5.2. Molecular genetic diagnosis 50
5.3. Phenotype-genotype correlation 51
5.3.1. Defects of dimerization 51
5.3.2. Defects of multimerization 52
5.3.3. Increased proteolysis 52
5.3.4. Increased affinity for GP Ib 53
5.3.5. FVIII binding defect 53
5.3.6. Other variants 53
m Acquired von Willebrand Syndrome (VWS) 56
6.1. Pathophysiological mechanisms 57
6.1.1. Lymphoproliferative diseases 57
6.1.2. Thrombocythemia 59
6.1.3. Reactive thrombocytosis (RT) 60
6.1.4. Neoplasms 60
6.1.4.1. Wilms tumor (nephroblastoma) 60
6.1.4.2. Carcinomas and solid tumors 60
6.1.5. Immunological diseases 60
6.1.6. Cardiovascular diseases 60
6.2. Clinical situations where patients with cardiovascular diseases are at special risk 61
6.2.1. Unexpected bleeding complications during surgical procedures in patients with advanced
arteriosclerosis and aortic stenosis 61
6.2.2. Bleeding complications in patients with endocarditis 62
6.2.3. Bleeding complications in patients with arteriosclerosis, pulmonary hypertension or aortic
stenosis during treatment with oral anticoagulants 62
6.3. Acquired von Willebrand Syndrome in patients with different diseases 63
WM Treatment of von Willebrand Disease 68
7.1. Desmopressin (DDAVP) 68
7.2. Plasma concentrates 70
7.3. Treatment of acquired von Willebrand Syndrome 70
H Thrombotic Thrombocytopenic Purpura (TTP) 76
8.1. Conventional methods of diagnosis 76
8.1.1. Detection of supranormal multimers 76
8.1.2. Assay of the activity of ADAMTS13 based on Furlan et al 77
8.1.3. Assay of the activity of ADAMTS13 based on Tsai et al 78
8.1.4. Assay of the activity of ADAMTS13 using residual VWF:CB and VWF:RCo 78
8.1.5. Assay of the activity of ADAMTS13 using fragment-specific monoclonal antibodies 78
8.1.6. Rapid method by incubation of patient plasma in denaturing buffer 79
8.1.7. FRETS assay 79
8.1.8. Method under conditions of a specific shear stress and in an endothelial cell-based system 80
8.1.9. Detection of non-neutralizing antibodies by an ELISA test 80
8.2. Molecular genetics 80
Contents 9
8.3. Treatment 82
8.3.1. Hereditary TTP 82
8.3.2. Acquired TTP 82
8.3.2.1. Plasma exchange 82
8.3.2.2. Immunosuppression/immunomodulation 82
8.3.2.3. Splenectomy 83
8.3.2.4. Antibodies to B cells 83
8.3.2.5. Treatment with antiplatelet agents 83
8.3.2.6. TTP after bone marrow transplantation 83
8.3.2.7. TTP in malignant diseases 83
8.3.2.8. Drug-induced TTP 83
^| References 86
f/l Index 92
|
| any_adam_object | 1 |
| author | Schneppenheim, Reinhard 1950- Budde, Ulrich |
| author_GND | (DE-588)109434900 (DE-588)108324354 |
| author_facet | Schneppenheim, Reinhard 1950- Budde, Ulrich |
| author_role | aut aut |
| author_sort | Schneppenheim, Reinhard 1950- |
| author_variant | r s rs u b ub |
| building | Verbundindex |
| bvnumber | BV026672577 |
| ctrlnum | (OCoLC)318215244 (DE-599)GBV559788320 |
| dewey-full | 616.157 |
| dewey-hundreds | 600 - Technology (Applied sciences) |
| dewey-ones | 616 - Diseases |
| dewey-raw | 616.157 |
| dewey-search | 616.157 |
| dewey-sort | 3616.157 |
| dewey-tens | 610 - Medicine and health |
| discipline | Medizin |
| edition | 1. Aufl. |
| format | Book |
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| indexdate | 2024-07-09T23:16:56Z |
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| isbn | 9783837410099 9781848151314 |
| language | English |
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| spelling | Schneppenheim, Reinhard 1950- Verfasser (DE-588)109434900 aut von Willebrand disease and von Willebrand factor current aspects of diagnosis and treatment [Reinhard Schneppenheim ; Ulrich Budde] 1. Aufl. Bremen [u.a.] UNI-MED-Verl. 2008 93 S. Ill., zahlr. graph. Darst. txt rdacontent n rdamedia nc rdacarrier UNI-MED science Budde, Ulrich Verfasser (DE-588)108324354 aut HBZ Datenaustausch application/pdf http://bvbr.bib-bvb.de:8991/F?func=service&doc_library=BVB01&local_base=BVB01&doc_number=022218286&sequence=000004&line_number=0001&func_code=DB_RECORDS&service_type=MEDIA Inhaltsverzeichnis |
| spellingShingle | Schneppenheim, Reinhard 1950- Budde, Ulrich von Willebrand disease and von Willebrand factor current aspects of diagnosis and treatment |
| title | von Willebrand disease and von Willebrand factor current aspects of diagnosis and treatment |
| title_auth | von Willebrand disease and von Willebrand factor current aspects of diagnosis and treatment |
| title_exact_search | von Willebrand disease and von Willebrand factor current aspects of diagnosis and treatment |
| title_full | von Willebrand disease and von Willebrand factor current aspects of diagnosis and treatment [Reinhard Schneppenheim ; Ulrich Budde] |
| title_fullStr | von Willebrand disease and von Willebrand factor current aspects of diagnosis and treatment [Reinhard Schneppenheim ; Ulrich Budde] |
| title_full_unstemmed | von Willebrand disease and von Willebrand factor current aspects of diagnosis and treatment [Reinhard Schneppenheim ; Ulrich Budde] |
| title_short | von Willebrand disease and von Willebrand factor |
| title_sort | von willebrand disease and von willebrand factor current aspects of diagnosis and treatment |
| title_sub | current aspects of diagnosis and treatment |
| url | http://bvbr.bib-bvb.de:8991/F?func=service&doc_library=BVB01&local_base=BVB01&doc_number=022218286&sequence=000004&line_number=0001&func_code=DB_RECORDS&service_type=MEDIA |
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