rdf-syntax-grammar
mods:
  schema location: http://www.loc.gov/mods/v3 http://www.loc.gov/standards/mods/v3/mods-3-2.xsd
  title info:
    title: Familial Mediterranean Fever
  name:
    name part: Gattorno, Marco.
    role:
      role term: editor.
  name:
    name part: SpringerLink (Online service)
  type of resource: text
  origin info:
    place:
      place term: gw
    date issued: 2015
    issuance: monographic
  language:
    language term: eng
  physical description:
    form: electronic
    form: electronic resource
    reformatting quality: access
    form: remote
    extent: VII, 162 p. 17 illus., 12 illus. in color. online resource.
  abstract: 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.
  table of contents: Preface -- 1 Genetics.- 2 Pathogenesis.- 3 Clinical Picture in Childhood -- 4 Clinical Picture in Adulthood and Unusual Clinical Features.- 5 FMF in Western Countries.- 6 Long Term Complications in FMF -- 7 Recent advances in quantitative assessment of FMF -- 8 How to Manage FMF Patients in Daily Practice -- 9 New Emerging Treatments.- Subject Index.
  note: edited by Marco Gattorno.
  subject:
    topic: Medicine
  subject:
    topic: Human genetics
  subject:
    topic: Immunology
  subject:
    topic: Internal medicine
  subject:
    topic: Rheumatology
  subject:
    topic: Medicine & Public Health
  subject:
    topic: Rheumatology
  subject:
    topic: Immunology
  subject:
    topic: Internal Medicine
  subject:
    topic: Human Genetics
  classification: RC927-927.5
  classification: 616.723
  related item:
    title info:
      title: Springer eBooks
  related item:
    title info:
      title: Rare Diseases of the Immune System, 3
  identifier: 9783319146157
  identifier: http://dx.doi.org/10.1007/978-3-319-14615-7
  record info:
    record creation date: 150319
    record change date: 20150519185138.0
    record identifier: 978-3-319-14615-7