Textbook of Hemophilia
http://www.100md.com
《新英格兰医药杂志》
Hemophilia is an unusual sex-linked inherited hemorrhagic disorder, but it has contributed to the advancement of biomedical knowledge and medical practice at a level that far exceeds its relatively low incidence. Transfusion medicine, cell biology, and translational medicine, to name just a few areas, have all benefited from research into this disease. It has been more than 20 years since the publication of a textbook that focused on hemophilia and, consequently, this book seeks to and successfully fills an obvious void.
Textbook of Hemophilia does more than meet the objective of providing a compendium of facts on the disease; it is a mosaic of 63 relatively short chapters that are supported by figures, tables, and photographs, and with bibliographies that range in number from 10 to 62 sources. These chapters are organized into 19 sections that cover both major and less critical topics related to current information on hemophilia and its management. The book also covers von Willebrand's disease and the rare inherited deficiencies of coagulation proteins. The book seeks to be multidimensional and comprehensive, and in this it is broadly successful. It covers current scientific knowledge, for example, of the structure of the factor VIII gene; the chemistry of factor VIII protein; and intracellular trafficking of factor VIII, factor IX, and von Willebrand factor; and also the pathophysiology, natural history, diagnostic problems, and treatment of these inherited defects. There is a considerable emphasis on the management of complications.
A number of particularly strong chapters enhance the book. The opening chapter, "Overview of Hemostasis," is a masterly distillation of the process and succeeds in presenting this topic in a comprehensible manner. Equally illuminating are the chapters on the intracytoplasmic processing associated with post-translational trafficking of coagulation proteins , such as "Cellular Processing of Factors VIII and IX," and the chapter on hepatitis C virus infection. The discussion of hemophilic joint disease takes up six chapters, and although the editors skimp on the pathophysiology of this important complication, there is an excellent chapter on assessment of the outcome of hemarthrosis by magnetic resonance imaging. There is also a chapter on regulatory aspects of the manufacture of therapeutic products for hemophilia, a seldom-discussed topic. The excellent reviews in this book will make it useful to a wide audience.
Edward D. Gomperts, M.D.
Childrens Hospital Los Angeles
Los Angeles, CA 90027(Edited by Christine A. Le)
Textbook of Hemophilia does more than meet the objective of providing a compendium of facts on the disease; it is a mosaic of 63 relatively short chapters that are supported by figures, tables, and photographs, and with bibliographies that range in number from 10 to 62 sources. These chapters are organized into 19 sections that cover both major and less critical topics related to current information on hemophilia and its management. The book also covers von Willebrand's disease and the rare inherited deficiencies of coagulation proteins. The book seeks to be multidimensional and comprehensive, and in this it is broadly successful. It covers current scientific knowledge, for example, of the structure of the factor VIII gene; the chemistry of factor VIII protein; and intracellular trafficking of factor VIII, factor IX, and von Willebrand factor; and also the pathophysiology, natural history, diagnostic problems, and treatment of these inherited defects. There is a considerable emphasis on the management of complications.
A number of particularly strong chapters enhance the book. The opening chapter, "Overview of Hemostasis," is a masterly distillation of the process and succeeds in presenting this topic in a comprehensible manner. Equally illuminating are the chapters on the intracytoplasmic processing associated with post-translational trafficking of coagulation proteins , such as "Cellular Processing of Factors VIII and IX," and the chapter on hepatitis C virus infection. The discussion of hemophilic joint disease takes up six chapters, and although the editors skimp on the pathophysiology of this important complication, there is an excellent chapter on assessment of the outcome of hemarthrosis by magnetic resonance imaging. There is also a chapter on regulatory aspects of the manufacture of therapeutic products for hemophilia, a seldom-discussed topic. The excellent reviews in this book will make it useful to a wide audience.
Edward D. Gomperts, M.D.
Childrens Hospital Los Angeles
Los Angeles, CA 90027(Edited by Christine A. Le)