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Practical hemostasis and thrombosis / / edited by Nigel S. Key, Michael Makris, David Lillicrap
| Practical hemostasis and thrombosis / / edited by Nigel S. Key, Michael Makris, David Lillicrap |
| Edizione | [Third edition.] |
| Pubbl/distr/stampa | Chichester, [England] : , : Wiley-Blackwell, , 2017 |
| Descrizione fisica | 1 online resource (486 pages) : illustrations, tables |
| Disciplina | 616.1/57 |
| Soggetto topico | Hemostasis - Physiology |
| Soggetto genere / forma | Electronic books. |
| ISBN |
1-118-34475-8
1-118-34474-X 1-118-34472-3 |
| Formato | Materiale a stampa  |
| Livello bibliografico | Monografia |
| Lingua di pubblicazione | eng |
| Record Nr. | UNINA-9910154927503321 |
| Chichester, [England] : , : Wiley-Blackwell, , 2017 | ||
| Materiale a stampa | ||
| Lo trovi qui: Univ. Federico II | ||
| ||
Practical hemostasis and thrombosis / / edited by Nigel S. Key, Michael Makris, David Lillicrap
| Practical hemostasis and thrombosis / / edited by Nigel S. Key, Michael Makris, David Lillicrap |
| Edizione | [Third edition.] |
| Pubbl/distr/stampa | Chichester, [England] : , : Wiley-Blackwell, , 2017 |
| Descrizione fisica | 1 online resource (486 pages) : illustrations, tables |
| Disciplina | 616.1/57 |
| Soggetto topico | Hemostasis - Physiology |
| ISBN |
1-118-34475-8
1-118-34474-X 1-118-34472-3 |
| Formato | Materiale a stampa |
| Livello bibliografico | Monografia |
| Lingua di pubblicazione | eng |
| Record Nr. | UNINA-9910830365703321 |
| Chichester, [England] : , : Wiley-Blackwell, , 2017 | ||
| Materiale a stampa | ||
| Lo trovi qui: Univ. Federico II | ||
| ||
Practical hemostasis and thrombosis [[electronic resource] /] / edited by Denise O'Shaughnessy, Michael Makris, David Lillicrap
| Practical hemostasis and thrombosis [[electronic resource] /] / edited by Denise O'Shaughnessy, Michael Makris, David Lillicrap |
| Pubbl/distr/stampa | Malden, Mass., : Blackwell Pub., c2005 |
| Descrizione fisica | vii, 224 p. : ill |
| Disciplina | 616.1/57 |
| Altri autori (Persone) |
O'ShaughnessyDenise
MakrisMichael LillicrapDavid |
| Soggetto topico |
Blood coagulation disorders
Thrombosis Hemostasis |
| ISBN |
1-280-28634-2
9786610286348 0-470-98863-0 1-4051-5199-4 |
| Formato | Materiale a stampa |
| Livello bibliografico | Monografia |
| Lingua di pubblicazione | eng |
| Nota di contenuto | Basic principles underlying the coagulation system / O'Connell -- Laboratory tests: routine and specialised / Kitchen and Makris -- Molecular diagnostic approaches to hemostasis / James and Lillicrap -- Tests of platelet function / Harrison -- Hemophilia A and B / Maclean and Makris -- Von Willebrand disease / Castaman, Tosetto and Rodeghiero -- The rarer inherited coagulation disorders / Bolton-Maggs and Wilde -- Quantitative platelet disorders / Kahr and Blanchette -- Qualitative platelet disorders / Cattanneo -- Disseminated intravascular coagulation and other microangiopathies / Kasthuri and Key -- Venous thromboembolism / Linkins and Kearon -- Arterial thrombosis / Lowe and Tait -- Anticoagulation / Palareti and Cosmi -- Antiphospholipid syndrome / Watson -- Obstetrics / Walker -- Paediatrics / Bauman and Massicotte -- Intensive/critical care / Hunt and Stuart-Smith -- Cardiothoracic surgery / O'Shaughnessy and von Kier -- Hepatology / Patel and Arya -- Oncology / Falanga and Marchetti -- Transfusion / Copplestone. |
| Record Nr. | UNISA-996205351103316 |
| Malden, Mass., : Blackwell Pub., c2005 | ||
| Materiale a stampa | ||
| Lo trovi qui: Univ. di Salerno | ||
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Textbook of Von Willebrand Disease : Basic and Clinical Aspects
| Textbook of Von Willebrand Disease : Basic and Clinical Aspects |
| Autore | Federici Augusto B |
| Edizione | [2nd ed.] |
| Pubbl/distr/stampa | Newark : , : John Wiley & Sons, Incorporated, , 2024 |
| Descrizione fisica | 1 online resource (306 pages) |
| Disciplina | 616.1/57 |
| Altri autori (Persone) |
BerntorpErik E
LillicrapDavid MontgomeryRobert R |
| Soggetto topico | Von Willebrand Diseases |
| ISBN |
1-119-41952-2
1-119-41947-6 1-119-41949-2 |
| Formato | Materiale a stampa |
| Livello bibliografico | Monografia |
| Lingua di pubblicazione | eng |
| Nota di contenuto |
Cover -- Title Page -- Copyright Page -- Contents -- List of Contributors -- Foreword -- Preface -- Chapter 1 Historical perspective on von Willebrand disease -- Introduction -- The scientist of the disease -- First description of the disease: the Åland family -- Other early clinical reports -- The search for a new factor-the bleeding time factor -- The end of the beginning -- Recent scientific visits to Åland Islands -- References -- Chapter 2 Biosynthesis and organization of von Willebrand factor -- Introduction -- Terminology -- Molecular biology of VWF -- The VWF gene -- VWF domain structure -- VWF promoter -- Cell biology of VWF -- VWF processing and dimerization in the endoplasmic reticulum -- VWF processing in the Golgi -- VWF multimerization -- Regulated storage of VWF in Weibel-Palade bodies and -granules -- Weibel-Palade body biogenesis -- Regulated release of VWF -- Postsecretion modification of VWF -- References -- Chapter 3 Von Willebrand factor structure and function -- Introduction -- VWF function -- Structure of VWF -- VWF domain structure -- Functional biochemistry of VWF -- Interaction of VWF with FVIII -- Two sites of interaction between VWF and platelets -- Proteolysis of VWF -- Carbohydrate modifications of VWF -- Clearance of VWF through autoantibodies and other mechanisms -- Increased clearance as a cause of type 1C VWD -- Critical disulfide structure of VWF -- Shear and its effect on VWF -- Structure and function of the VWF propeptide -- Summary -- Acknowledgement -- References -- Chapter 4 Regulation of von Willebrand factor expression -- Introduction -- VWF gene structure and chromosomal location -- VWF expressing cells -- VWF promoter structure -- Transcriptional activators -- Transcriptional suppressors -- NFY, a dual-purpose transcription factor -- Influence of regulatory sequence variants on VWF expression.
Regulatory variants in the proximal regulatory region: the VWF promoter -- Regulatory variants in the distal regulatory region: VWF enhancers -- Epigenetic regulation of VWF expression -- Posttranscriptional regulation of VWF expression -- Splicing events and differential VWF expression in endothelial cells -- MicroRNAs regulate VWF biosynthesis, maturation, and secretion -- Physiological, pathological, and environmental factors affecting VWF expression -- Hemodynamic shear stress regulates VWF expression -- VWF is an acute-phase reactant -- Hormones raise blood VWF levels -- Conclusion -- References -- Chapter 5 Modulation of VWF by ADAMTS13 -- Introduction -- VWF structure, synthesis, and function -- ADAMTS13 structure -- ADAMTS family proteases -- Prodomain -- Metalloprotease (MP) domain -- Disintegrin-like (Dis) domain -- Thrombospondin type 1 repeats (TSP) -- Cysteine-rich (Cys-rich) domain -- Spacer domain -- CUB domains -- ADAMTS13 conformation/latency -- Global latency -- Local latency -- ADAMTS13 function -- Physiology and pathophysiology of the VWF-ADAMTS13 axis -- Summary -- References -- Chapter 6 Assessment of VWF clearance -- Introduction -- Macrophage-mediated VWF clearance -- Low-density lipoprotein receptor-related protein 1 (LRP1) -- Scavenger receptor class A member I (SR-A1) -- Macrophage galactose lectin (MGL) -- Siglec-5 -- Endothelial cell contribution to VWF clearance -- C-type lectin domain family 4 member M (CLEC4M) -- Stabilin-2 -- Scavenger Receptor Class A Member 5 (SCARA5) -- Conclusion -- References -- Chapter 7 Classification of VWD -- Classification of VWD -- von Willebrand factor -- Historical classification -- Type 1 VWD -- Type 1C VWD -- Type 1 versus low VWF -- Type 2 VWD -- Type 3 VWD -- Clinical classification of VWD -- Genetic classification of VWD -- Conclusion -- Acknowledgement -- Conflict of Interest. References -- Chapter 8 The epidemiology of von Willebrand disease -- Introduction -- Historical studies on the prevalence of VWD -- Prevalence of bleeding patients in the general population -- Bleeding score: A new diagnostic tool to assess clinically relevant VWD -- The problem of diagnosing mild VWD -- Prevalence of intermediate VWD -- Prevalence of severe VWD -- Prevalence of a mutant VWF gene -- Conclusions -- References -- Chapter 9 Clinical aspects of von Willebrand disease: bleeding history -- Introduction -- Bleeding history in VWD -- Bleeding symptoms in VWD -- Cutaneous symptoms -- Epistaxis -- Gynecologic bleeding -- Oral cavity bleeding -- Postoperative bleeding -- Gastrointestinal bleeding -- Other bleeding symptoms -- Specific situations -- Women-the value of gynecologic assessment -- Bleeding assessment tools -- Conclusion -- References -- Chapter 10 Laboratory diagnosis of von Willebrand disease: the phenotype -- Screening diagnostic tests -- Bleeding time -- PFA-100/200 and other global shear stress test systems -- Activated partial thromboplastin time -- Platelet adhesion -- Platelet count -- Extended diagnostic tests -- Assay of the FVIII/VWF complex -- Ristocetin-induced agglutination in platelet-rich plasma (RIPA) -- Botrocetin-induced aggregation in platelet-rich plasma (BIPA) -- Binding studies with washed platelets -- VWF propeptide (VWFpp or VWF:Ag II) -- Qualitative changes in VWF -- VWF multimers -- VWF fragments -- FVIII-binding capacity of VWF (VWF:FVIIIB) -- VWF antibodies/inhibitors -- Diagnosis in neonates and young children -- Diagnosis in pregnancy -- Desmopressin trials as an aid to the diagnosis and functional characterization of VWD -- An algorithmic approach to diagnosis of VWD -- Future perspectives -- References -- Chapter 11 Molecular diagnosis of von Willebrand disease: the genotype. Introduction and role of genetic testing in VWD -- Techniques used in molecular analysis of VWD -- Direct Sanger sequencing -- Next generation sequencing -- Copy number variant analysis -- Assessment of variant pathogenicity -- Molecular spectrum of VWD -- Type 1 -- Type 2 -- Type 3 -- Challenges and future of VWD genotyping -- Acknowledgments -- References -- Chapter 12 Clinical, laboratory, and molecular markers of type 1 von Willebrand disease and low von Willebrand factor -- Introduction -- The epidemiology of type 1 von Willebrand disease -- Clinical features of type 1 von Willebrand disease -- The laboratory diagnosis of type 1 von Willebrand disease -- The genetics of type 1 von Willebrand disease -- The role of ABO blood group and type 1 von Willebrand disease -- von Willebrand factor gene mutations and type 1 von Willebrand disease -- Recurrent type I von Willebrand disease candidate mutations -- Non-coding sequence variants in type 1 von Willebrand disease -- Type 1 von Willebrand disease and accelerated clearance of von Willebrand factor -- Future priorities in type 1 von Willebrand disease characterization -- References -- Chapter 13 Clinical and molecular markers of type 1C VWD -- Introduction -- Epidemiology -- Clinical features -- Laboratory diagnosis and studies on increased clearance in type 1 VWD -- Genetics and the specific mutations of type 1C VWD -- The role of glycans in increased VWF clearance -- Summary -- References -- Chapter 14 Clinical and molecular markers of VWD2A -- Introduction -- Pathophysiology -- Clinical manifestations -- Laboratory diagnosis -- Molecular markers -- Treatment -- References -- Chapter 15 Clinical and molecular markers of VWD2B -- Introduction -- Pathophysiology -- Clinical manifestations -- Laboratory diagnosis -- Molecular markers -- Treatment -- Conclusions -- References. Chapter 16 Clinical and molecular markers of type 2M VWD -- Introduction -- Clinical features of type 2M VWD -- Laboratory classification of type 2M VWD -- Problems with ristocetin in VWF assays -- Genetic variants found in type 2M VWD -- Treatment of type 2M VWD -- Summary -- Acknowledgements -- Conflicts of Interest -- References -- Chapter 17 Clinical and molecular markers of VWD2N -- The VWF-FVIII interaction -- Laboratory diagnosis -- Molecular analysis -- Proposed algorithm for the diagnosis of type 2N -- Clinical symptoms -- Therapeutic options -- Conclusions -- References -- Chapter 18 Clinical, laboratory, and molecular markers of type 3 von Willebrand disease -- General definition, history, and epidemiology -- General definitions -- History -- Epidemiology -- Clinical markers of VWD3 -- Laboratory markers of VWD3 -- Molecular markers of VWD3 -- Inheritance pattern -- Large gene deletions -- Nonsense mutations -- Small deletions and insertions -- Splice site mutation8 -- Missense mutation -- Additional VWF molecular markers found in 3WINTERS-IPS patients -- Prenatal and molecular diagnosis of VWD3 -- Treatment and prevention of bleeding in VWD3 -- Treatment of patients with alloantibodies to VWF -- Secondary long-term prophylaxis -- Future perspectives -- Acknowledgments -- References -- Chapter 19 Pediatric aspects of von Willebrand disease -- Introduction -- Diagnosis of VWD in childhood -- Clinical presentation and screening tests -- Diagnosis of type 1 VWD versus low VWF levels as a risk factor for bleeding -- VWD in neonates -- Acquired VWS in childhood -- VWD in adolescents -- Treatment strategies in children -- General principles -- DDAVP -- Replacement therapy -- Conclusions -- References -- Chapter 20 Women with von Willebrand Disease -- Introduction -- Gynecology -- Approach to screening and diagnosis of VWD in women with HMB. Gynecological problems in women with VWD. |
| Record Nr. | UNINA-9910877191103321 |
Federici Augusto B
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| Newark : , : John Wiley & Sons, Incorporated, , 2024 | ||
| Materiale a stampa | ||
| Lo trovi qui: Univ. Federico II | ||
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