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Systemic autoinflammatory diseases (SAIDs) are a growing group of rare disorders caused by dysregulation of the innate immune system leading to episodes of organ-specific and systemic inflammation. Autoinflammation as a distinct disease category was first reported in 1999 as a group of monogenic disorders with resultant activation of the inflammasome and cytokine excess, presenting as periodic fever and inflammation in serous membranes. Since this original description, the focus has shifted considerably to the inclusion of complex multifactorial conditions, and more than 30 associated genes have been identified. Disease severity varies from mild to life threatening. Advances in the understanding of the pathogenetic role of interleukin-1, have opened new promising horizons in the treatment of these patients, and significantly altered disease outcome.
Over the course of the previous seven editions, Pediatric Nephrology has become the standard reference text for students, trainees, practicing physicians (pediatricians, nephrologists, internists, and urologists), subspecialists, and allied health professionals seeking information about children’s kidney diseases. It is global in perspective, reflecting the fact that the international group of editors are all acknowledged world experts. The latest edition of this text is no different, providing a comprehensive, state-of-the-art overview on pediatric nephrology. Much like the previous edition, the latest edition reviews the most critical aspects of the field. Topics covered include developm...
Many of the systemic autoimmune diseases seen in children are different from those seen in adults making them a special problem for physicians and scientists who care for the affected children and study their diseases. Benefiting both pediatric and adult rheumatologists, as well as physicians from other specialties, this volume covers the latest advances in pathogenesis and clinical management of common conditions seen in pediatric rheumatology practices.
This book, written by very well-known opinion leaders in the field, covers all aspects of periodic and non –periodic fevers, and related disorders. The expression refers to several different auto-inflammatory diseases, showing similar symptoms–the primary symptom being a recurrent fever for an infectious cause cannot be found. The opening chapters give some historical hints, explain the genetic basis of the disease and provide insights into the pathogenesis derived from recent experimental studies and guides the reader through classification and nomenclature. A large part of the book is then devoted to a detailed description of the specific related diseases and their clinical presentatio...
This book on rheumatology in children is intended to be a handy tool for all professionals interested in this field. It is divided into five sections: General considerations, arthritic problems, connective tissue diseases, systemic vasculitides and finally a section that covers miscellaneous issues such as Immunodeficiencies, bone heath and uveitis. The book has an easy format with each chapter having one or more clinicalvignettes at the start of the chapter. In addition each chapter outlines the learning objectives and key take home messages. At the end of each section a collection of cases to highlight important issues in the clinic are included. The colorful charts, pictures and tables make reading this book an easy task. The authors are leading experts in their fields working at best practices centers globally to provide the reader with state-of-the-art information.
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