Textbook of Hemophilia

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

• ISBN13: 9781405169141
• ISBN10: 1405169141
• Edition: 2nd
• Format: Hardcover
• Copyright: 2010-07-13
• Publisher: Wiley-Blackwell

Summary

The Textbook of Hemophilia has become the definitive resource on all aspects of hemophilia, including diagnosis, management and treatment. 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. Complications of hemophilia care are well covered in chapters on inhibitors, musculoskeletal problems and transfusion-transmitted disease. This new edition includes all the latest developments in the field of hemophilia, including the molecular basis of the disease, the role of factors in coagulation, epidemiology, pharmacokinetics, treatment inhibitors and musculoskeletal aspects of the disease. It contains a new chapter on chronic pain management and coverage of new developments such as gene therapy, and highlights controversial issues and provides advice for everyday clinical questions.

Author Biography

Christine A. Lee MA, MD, DSc, FRCP, FRCPath
Emeritus Professor of Haemophilia, University of London;
Honorary Consultant Haematologist, Oxford Haemophilia & Thrombosis Centre, Oxford, UK

Erik E. Berntorp MD, PhD
Professor of Haemophilia, Lund University,  Malmö Centre for Thrombosis and Haemostasis, Skåne University Hospital, Malmö, Sweden

W. Keith Hoots MD
Director, Division of Blood Diseases and Resources, National Heart, Lung and Blood Institute, National Institutes of Health, Bethesda, MD;
Professor of Pediatrics and Internal Medicine, University of Texas Medical School at Houston,
Houston, TX, USA

Table of Contents

List of Contributors (Historical introduction)

Introduction

Overview of Hemostasis

Cellular processing of Factor VIII and Factor IX

Hemophilia A

Molecular Basis of Hemophilia A

Phenotypic/genotypic relationship

Prophylaxis

Continuous Infusion of Coagulation Products in Hemophilia

Inhibitors to Factor VIII

Inhibitors to Factor VIII - Immunology

Genetic and environmental risk factors for FVIII inhibitor development

Inhibitors to Factor VIII - Mild and Moderate Hemophilia

Inhibitors to Factor VIII/IX: Immune Tolerance

Inhibitors to factor VIII: treatment of acute bleeds

Acquired Hemophilia

Acquired Inhibitors to Factor VIII

Hemophilia B

Hemophilia B - Molecular Basis

Factor IX Inhibitors in Hemophilia B

Treatment of Inhibitors in Hemophilia B

Pharmacokinetics of Factors VIII & IX

Pharmacokinetics

Hemophilia - birth to old age

The Neonate with Hemophilia

Work-up of a Bleeding Child

Care of the Child with Hemophilia

Adolescence

Old age medicine and hemophilia

Products used to Treat Hemophilia

Products Used to Treat Hemophilia: Recombinant Products

Products Used to Treat Hemophilia: Plasma-Derived Coagulation Factor Concentrates

Products Used to Treat Hemophilia: Recombinant Factor VIIa

Products Used to Treat Hemophilia: Dosing

Products Used to Treat Hemophilia: Regulation

Musculoskeletal

Joint Replacement

Synovioarthesis in Hemophilia

Pseudotumours in patients with Hemophilia

Imaging modalities for assessment of hemophilic arthropathy

Physiotherapy in the Management of Hemophilia

Clinimetric Instruments in Haemophilia

Transfusion-Transmitted Disease

Hepatitis C Virus Infection and Liver Transplantation

Emerging Infections

Gene Therapy

Hemophilia Gene Therapy: An overview

Gene Therapy Trials

Gene Therapy: Molecular Engineering of Factor VIII and Factor IX

Laboratory

Laboratory Assays in Hemophilia

Standardization of Assays

Global laboratory assays in Haemophilia

Obstetrics & Gynaecology

Obstetrics and Gynecology: Hemophilia

Von Willebrand Disease

von Willebrand Disease: Molecular Aspects

von Willebrand Disease: Epidemiology

von Willebrand Disease: Biological Diagnosis

Classification and Clinical Aspects of von Willebrand Disease

Treatment of Von Willebrand Disease

Treatment of von Willebrand Disease: Desmopressin

Treatment of von Willebrand Disease: Therapeutic Concentrates

Women & Von Willebrand Disease

Rare Bleeding Disorders

Factor II

Factor V and combined Factor V and VIII Deficiencies

Congenital Factor VII Deficiency

Factor X Deficiency

Factor XI Deficiency

Factor XIII Deficiency

Fibrinogen Deficiency

Miscellaneous Rare Bleeding Disorders

Emergency medicine

Emergency Medicine and Inherited Blood Disorders

Quality of Life

Quality of Life in Hemophilia

Economics

The Economics of Hemophilia Treatments

Comprehensive Care and Delivery of Care

National Haemophilia Databases

Comprehensive Care and the Delivery of Care: The Developed World

Comprehensive care and delivery of care in haemophilia: The Developing World

Comprehensive Care and Delivery of Care: The Global Perspective

Index

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