This volume is a reference handbook focusing on diseases like Marfan syndrome, Ehlers-Danlos syndrome, Loeys-Dietz syndrome and other heritable soft connective tissue diseases. The book presents detailed information for both basic scientists and for clinicians seeing patients. It is also a stepping stone for new investigations and studies that goes beyond the facts about the composition and biochemistry of the connective tissue and extracellular matrix, as the authors connect individual components to specific aspects of various soft tissue disorders and to the actual or potential treatment of them. Progress in Heritable Soft Connective Tissue Diseases features very prominent physicians and scientists as contributors who bring their most recent discoveries to the benefit of readers. Their expertise will help clinicians with proper diagnosis of sometimes elusive and uncommon heritable diseases of soft connective tissues. This book also offers an update on the pathophysiology of these diseases, including an emphasis on unifying aspects such as connections between embryonic development of the different types of connective tissues and systems, and the role of TGF-beta in development and physiology of soft tissues. This new set of data explains, at least in part, why many of these disorders are interconnected, though the primary pathophysiological events, such as gene mutations, may be different for each disorder.

Synthesizes a wealth of new information in the areas of biochemistry, genetics and molecular biology. Contains clear,well-organized material regarding advances in the study of heritable disorders of connective tissue, describing breakthroughs in the last ten years which, until now, have not been published.

This volume provides reviews covering the latest advances in particular areas of connective tissue research. This comprehensive work also includes areas of the medical field in which the basic aspects could be applied. It explains that both cells and matrix are altered in disease states because of the strong interactions established between cells and the extracellular matrix. The aim of this book is to close the existing gap between basic scientists and clinical investigators. This reference is an absolute must for all biological chemists, clinical investigators, and pathologists. Students of these professions will find this reading both informative and useful as well.

Ehlers-Danlos Syndrom.

Learning from Disease in Pets: A ‘One Health’ Model for Discovery is the first encompassing reference guide for veterinarians, researchers and physicians on conducting studies using spontaneous models of disease in animals. The study of naturally occurring disease in (pet) animals can help model our understanding of the biology, prevention and therapy of human and animal diseases. Studies of pet dogs, for instance, can aid treatment of complex medical problems such as cancer, orthopedic, cardiopulmonary, and neuro-inflammatory diseases, and zoonotic infections. Each chapter within this novel cross-species approach is contributed by a leader, or leaders, in their field of research. Using clinical trials to learn how pets with real diseases respond to therapy can lead to breakthroughs in human medicine, as well as benefiting pets suffering from otherwise debilitating illness. Despite similarities of diseases across species, there are very few spontaneous models of disease used in research compared with models where disease is induced in healthy laboratory animals. Many medical researchers and veterinarians have a multitude of questions regarding how to use naturally occurring diseases in pets for the discovery of treatments and diagnostics: this book will demonstrate how to safely make this happen. This book encourages veterinarians to build on and disseminate existing findings for the wider benefit of pets and humans. Many pets suffering from incurable illnesses may benefit from clinical trials; the book includes a section on the imperative communication styles necessary within the research environment and with clients, a compelling discussion on the ethics of using pets in veterinary clinical research, comprehensive tables of diseases that spontaneously occur in animals and humans, the regulatory requirements necessary to move therapy from benchside research to patient bedside, as well as intricate details on how to design a robust clinical study.

Generalized hypermobility has been known since ancient times, and a clinical description of Ehlers-Danlos syndrome (EDS) is said to have first been recorded by Hippocrates in 400 BC. Hypermobility syndromes occur frequently, but the wide spectrum of possible symptoms, coupled with a relative lack of awareness and recognition, are the reason that they are frequently not recognized, or remain undiagnosed. This book is an international, multidisciplinary guide to hypermobility syndromes, and EDS in particular. It aims to create better awareness of hypermobility syndromes among health professionals, including medical specialists, and to be a guide to the management of such syndromes for patients and practitioners. It is intended for use in daily clinical practice rather than as a reference book for research or the latest developments, and has been written to be understandable for any healthcare worker or educated patient without compromise to the scientific content. The book is organized as follows: chapters on classifications and genetics are followed by chapters on individual types, organ (system) manifestations and complications, and finally ethics and therapeutic strategies, with an appendix on surgery and the precautions which should attend it. A special effort has been made to take account of the perspective of the patient; two of the editors have EDS. The book will be of interest to patients with hypermobility syndromes and their families, as well as to all those healthcare practitioners who may encounter such syndromes in the course of their work.

For more than 30 years, the highly regarded Secrets Series® has provided students and practitioners in all areas of health care with concise, focused, and engaging resources for quick reference and exam review. Rheumatology Secrets, 4th Edition, features the Secrets’ popular question-and-answer format that also includes lists, tables, pearls, memory aids, and an easy-to-read style – making inquiry, reference, and review quick, easy, and enjoyable. The proven Secrets Series® format gives you the most return for your time – succinct, easy to read, engaging, and highly effective. Fully revised and updated throughout, including protocols and guidelines that are continuously evolving and that increasingly dictate best practices. Practical coverage of basic immunology and pathophysiology, important disease manifestations, and clinical management issues related to common and uncommon rheumatic disorders. Top 100 Secrets and Key Points boxes provide a fast overview of the secrets you must know for success in practice and on exams. Features bulleted lists, mnemonics, practical tips from leaders in the field – all providing a concise overview of important board-relevant content. Keeps you up to date with new techniques and technologies, as well as changing treatment options and drug information. Portable size makes it easy to carry with you for quick reference or review anywhere, anytime.