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InicioE-BooksE-Books Médicos Familial Mediterranean Fever (Rare Diseases of the Immune System Book 3)
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Familial Mediterranean Fever (Rare Diseases of the Immune System Book 3)

Product details

  • File Size: 1417 KB
  • Print Length: 170 pages
  • Page Numbers Source ISBN: 3319146149
  • Publisher: Springer; 2015 edition (March 19, 2015)
  • Publication Date: March 19, 2015
  • Sold by: Amazon.com Services LLC
  • Language: English
  • ASIN: B00UYF3A30
  • Text-to-Speech: Enabled
  • X-Ray: Not Enabled
  • Word Wise: Not Enabled
  • Lending: Not Enabled
  • Enhanced Typesetting: Enabled

 

This book, written by very well known opinion leaders in the field, covers all aspects of familial Mediterranean fever, the most common monogenic autoinflammatory disease. The opening chapters explain the genetic basis of the disease and provide insights into the pathogenesis derived from recent experimental studies. A large part of the book is then devoted to a detailed description of the typical and atypical clinical presentations, the disease course, and potential complications in both pediatric and adult patients. Guidance is provided on the measurement of disease severity and the management of patients in daily practice. The advice regarding treatment is based on the best currently available evidence and attention is also paid to important emerging treatments.

The book is part of Springer’s series Rare Diseases of the Immune System, which presents recently acquired knowledge on pathogenesis, diagnosis, and therapy with the aim of promoting a more holistic approach to these conditions. Monogenic autoinflammatory diseases are hereditary disorders that are caused by single-gene defects in innate immune regulatory pathways and are characterized by a clinical and biological inflammatory syndrome in which there is limited, if any, evidence of autoimmunity. Familial Mediterranean fever itself is due to a mutation in the MEFV gene, which codes for the protein pyrin; it is characterized by periodic fever and episodes of painful inflammation in the abdomen, chest, and joints. Familial Mediterranean Fever will be an invaluable source of up-to-date information for all practitioners involved in the care of patients with the disease.

Categoría: E-Books Médicos
  • Descripción
  • Por qué utilizar archivos PDF
Descripción

When my friend and colleague Lorenzo Emmi asked me to act as Editor for a book on Familial Mediterranean Fever in the context of a trilogy dedicated to autoinfl ammatory syndromes, I was really honoured but also a bit nervous. Indeed, the challenging idea to collect in a single book all the enormous amount of knowledge related to the “Big Mother” of all autoinfl ammatory diseases was really stimulating to me. On the other hand, I immediately realized that a relevant number of my colleagues, practically all the people involved in the present book and many others, had the right to act as the Editor of such a challenge much more than me. Indeed most of them literally wrote the history of this disease and in a sense of the whole expanding spectrum of autoinfl ammatory diseases.

Isabelle Touitou and Dan Kastner are the true pioneers in the identifi cation of genes involved in the rheumatic conditions transmitted in a Mendelian fashion. Indeed their identifi cation of MEFV gene (or marenostrin) in 1997 was the seminal step of the fascinating history of autoinfl ammatory disease. In her chapter, Isabelle Touitou and Guillaume Sarrabay describe all the secrets concerning the genetic aspects of this condition, whereas Dan Kaster, with the precious help of JJ Chae, gives us a brilliant and updated overview of all the advancements and the still unsolved questions related to the pathogenesis of the disease. Avi Livneh with his co-workers, Eldad Ben-Chetrit and Helen Lachmann share with us their outstanding clinical experience in the diagnosis and management of FMF from their perspective of adult rheumatologists and experts in amyloidosis. For the same reason, I am in debt with my dear colleagues, pediatric rheumatologists Seza Ozen, Tilo Kallinich and Huri Özdoğan, for their fantastic contribution in the description of the condition in children and in the illustration of the novel therapeutic approaches with biologics.

Finally, I am really grateful to Raffaele Manna and Erkan Demirkaya for their excellent contribution on epidemiology of FMF around the world and the description of available tools for the practical monitoring of disease activity and severity in everyday clinical practice. I am in debt to all these outstanding colleagues for their generous and extremely competent availability to be part of this project. This book defi nite is a tribute to their work on FMF during all these years.

Por qué utilizar archivos PDF

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