Connective Tissue and Its Heritable Disorders: Molecular, Genetic, and Medical Aspects by Peter M. Royce and Beat Steinmann Sales Rank: 1718474 List Price: $435.00 $461.50 At Amazon on 7-21-2008.

Hardcover: 1201 pages Publisher: Wiley-Liss; 2 edition May 15, 2002 Language: English ISBN-10: 0471251852 ISBN-13: 978-0471251859 Product Dimensions: 11.1 x 8.7 x 2.5 inches Shipping Weight: 8.8 pounds From the New England Journal of Medicine, May 29, 2003 I was genuinely thrilled at the prospect of reviewing the second edition of this multiauthored work covering the biology and pathology of connective tissues. The second edition, despite being 450 pages longer than the first edition, is a real pleasure for anyone with an interest in the skeleton and associated tissues. In the past 20 years, we have witnessed a remarkable change in our understanding of the intricate processes at work in the connective tissues, both in health and in disease. The view of the skeleton as a somewhat inert structure supporting the other tissues, whose physiology is more interesting, is long gone. Throughout this book, the editors have presented fascinating examples of how our knowledge of these tissues has been enhanced by the application of genetics, biochemistry, and structural biology. The editors have drawn widely from experts in their field, and there is a strong sense of personal involvement throughout. In a book of this sort, it is unusual to achieve such comprehensive coverage of a wide range of topics. Although not a small book at 1200 pages, it is, nonetheless, physically manageable and logically arranged. The indexing is meticulous and accurate, which facilitates its routine use as a source of reference. In the first section of the book, the biology of the connective tissues and the extracellular matrix is described in considerable detail. Separate sections detail the different tissues, including the skin, tendons, bones, cardiovascular system, and eyes; thereafter, the important components of the extracellular matrix, such as collagen, elastin, and adhesive glycoproteins, are described in meticulous detail. The descriptions of elastin and the associated microfibrils suffer somewhat by comparison with the two-part chapter on collagens, but only because the latter is simply superb. Any doctoral student will be more than satisfied with the detail and breadth of these accounts. In the second section, the range of heritable disorders affecting these tissues is described comprehensively. It is hard to fault the clinical, radiographic, or metabolic descriptions that accompany each section. The overall structure of this book is similar to that of the first edition, but useful additions include separate chapters on the eye and cardiovascular system. The section on chondrodysplasias has been substantially expanded to keep pace with the rapid developments in this exciting field. Disorders of sulphate metabolism, fibroblast growth-factor genes, and skeletal morphogenesis now receive separate consideration. There are also small, discrete chapters dealing with a number of miscellaneous disorders, such as the osteoporosis-pseudoglioma syndrome, regarding which important advances have been made. Because of its size, this book is not one to read at a single sitting, but each chapter is self-contained and comprehensively referenced. A useful feature is the "Recent Developments" summary at the end of most chapters. Perhaps the most obvious omission from an otherwise outstanding book is that of an overview chapter on medical and molecular genetics in the first section. Since genetics is a rapidly developing field that has played an enormous part in enhancing our knowledge of the connective-tissue disorders, this is a somewhat surprising omission. Paul Wordsworth, M.B., F.R.C.P. Copyright © 2003 Massachusetts Medical Society. All rights reserved. The New England Journal of Medicine is a registered trademark of the MMS. Product Review "an excellent book that provides an invaluable resource on connective tissue and associated disorders. It should be available to all with a scientific or medical interest in this field." (Doodys Health) "The second editionhas been vastly expandeda welcome addition to the library of the medical geneticist and is essential for workers in the connective tissue field" (The American Journal of Human Genetics, February 2003) "a real pleasure for anyone with an interest in the skeleton and associated tissuesoutstanding book" (New England Journal of Medicine, May 29, 2003) Customer Reviews & Comments "The second edition...has been vastly expanded...a welcome addition to the library of the medical geneticist and is essential for workers in the connective tissue field..." (The American Journal of Human Genetics, February 2003) Comment | Permalink | (Report this) Connective Tissue and Its Heritable Disorders: Molecular, Genetic, and Medical Aspects Available from Amazon Price: $461.50 Updated on 7-21-2008.

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