Amyloid Diseases

Neurodegenerative diseases are severe, rapidly developing, and currently incurable conditions that result in progressive degeneration and the death of neurons. This causes dementia, movement problems, and essentially loss of personal identity. Amyloids attempts to answer the following questions: (1) why do we develop these severe neurodegenerative diseases? (2) what histological and physiological changes are observed upon development and progression of these diseases? and (3) how can we treat amyloid-associated diseases?

The second edition of this text presents an overview of the most recent developments in this area including clinical presentation, etiology, pathogenesis, and differential diagnosis. The rationale for various therapies, including transplantation, is discussed and tissue diagnosis (its pitfalls and strategies for avoiding them) and laboratory support are included. The involvement of all major organ systems including renal/genitourinary, cardiac, gastrointestinal, pulmonary, peripheral nerve/central nervous system, soft tissue, skin, lymph node/spleen and bone marrow pathology is also covered. Amyloid and Related Disorders, Second Edition will be invaluable to specialized and general pathologists as well as cytopathologists. Other medical professionals may also benefit from this concise update on the systemic amyloidoses.

This book is a gift from the international community of amyloid friends, presented to Professor Dr. Enno Mandema on the occasion of his retirement from the University of Groningen, the Netherlands. It is the "precipitation" of up to date knowledge of amyloidosis, as presented at the International Course on Amyloidosis in Groningen, on the 10th and 11th of October 1986. Twenty years ago, Professor Mandema invited a group of scientists, who were studying the various aspects of amyloidosis from different points of view, to discuss their mutual interest in the subject. This "First International Symposium" was held for five days in September 1967. It was a wonderful experience for the participants, as most of them had until then only read each others work in the literature. The proceedings of that symposium, which contained the "lively" dis cussions, became a text-book for the following years. Research continued, and while the book was still in preparation, the revolutionary method of "water-soluble amyloid" was published. In the following years, different amyloid proteins were discovered and the mo lecular basis of the different amyloid syndromes was elucidated. The increase in knowledge parallelled the availability of modern, ingenious and also rapid methods in the biomedical sciences.

Amyloid protein aggregates are involved in ''protein-misfolding diseases'' of enormous social and economic impact, still with no effective therapies. The most prevalent amyloid pathologies are related to neurodegenerative diseases, but amyloidosis also affects other organs. The majority of the studies includes serious health connotations on amyloids. However, not all amyloid fibers play a detrimental role in host. An increasing number of studies shows an important beneficial role as ''functional amyloids''. This book opens an exciting door to provide up-to-date information about the function and the mechanisms of the amyloid formation process from the structural, biophysical, biomedical, and nanotechnological perspective, combining the new findings on toxic and functional amyloids studies using theoretical and experimental approaches to fight against amyloid-based diseases.

Edited by a stellar team of scientists compiling more than 120 papers into a single volume, the XIth Annual International Symposium on Amyloidosis represents the most important review of the state-of-the-science and future directions of this rapidly growing field. An unparalleled cast of pioneering researchers, including keynote speaker and Nobel Laureate Dr. Stanley Prusiner, present groundbreaking research in systemic amyloidosis including the mechanisms of disease and cellular toxicity, AA amyloidosis, familial amyloidosis, AL amyloidosis, clinical trials, and emerging Divided into six sections, the book begins with a study of the mechanisms of fibril formation and cellular response. It investigates possible horizontal transmission, charge differences of deposits in AL and NALCDD, and novel assays to detect amyloid induced apoptosis. The second section considers AA and localized amyloidosis and presents advances in non-invasive imaging, amyloid typing, and the translocation of systemic amyloidosis. The section on familial and TTR amyloidosis features an original report on hereditary gelsolin amyloidosis in an Iranian family, as well as other novel and rare mutations. An extensive section on AL amyloidosis introduces ALBASE as a platform analysis tool, AL amyloidosis in the elderly, and a study of the therapeutic potential of naturally occurring antibodies. Section five focuses on new and emerging diagnostic and translational approaches including characterization and identification through biopsy, mass spectrometry, proteomics, and the use of an amyloid "chip". The book concludes with clinical trials of diflunisal and high dose melphalan-dexamethasone and autologous stem cell transplantation. translational approaches. Focusing on new basic and translational medicine approaches in systemic amyloidosis, XIth Annual International Symposium on Amyloidosis provides clinicians and researchers with an invaluable single source reference to the most up-to-date research in the field.

Amyloidoses are a heterogeneous group of diverse etiology diseases. They are characterized by an endogenous production of abnormal proteins called amyloid proteins, which are not hydrosoluble, form depots in various organs and tissue of animals and humans and cause dysfunctions. Despite many decades of research, the origin of the pathogenesis and the molecular determinants involved in amyloid diseases has remained elusive. At present, there is not an effective treatment to prevent protein misfolding in these amyloid diseases. The aim of this book is to present an overview of different aspects of amyloidoses from basic mechanisms and diagnosis to latest advancements in treatment.

An up-to-date reference on this fascinating set of complex disorders, this book features the most comprehensive strategies for diagnosing, classifying, imaging, treating, and managing amyloidosis in multiple organ systems. Beneficial to the spectrum of practitioners from residents to sub-specialists, this book is a succinct authoritative text written by leaders in the field. The authors provide instruction on all forms of amyloidosis - including primary amyloidosis (AL), secondary amyloidosis (AA), and familial amyloidosis. With essential treatment algorithms, Amyloidosis: Diagnosis and Treatment is the gold-standard for all hematologists, oncologists, and internists caring for patients with this complex disease.