The Eurofever Project: towards better care for autoinflammatory diseases.
Summary of "The Eurofever Project: towards better care for autoinflammatory diseases."
Autoinflammatory diseases are a group of diseases characterized by inflammatory attacks. The monogenic forms of these diseases are also classified as the hereditary periodic fever syndromes. All are characterized by attacks of fever along with certain clinical features and high acute phase reactants. Most of these monogenic diseases are associated with hereditary disorders of the interleukin-1 pathway. The most common autoinflammatory disease is familial Mediterranean fever. The other rather common monogenic diseases are the tumor necrosis factor receptor-associated periodic syndrome, hyperimmunoglobulinemia D with periodic fever syndrome, and cryopyrin-associated periodic fever syndromes (CAPS). However, a number of multifactorial diseases such as Behçet disease are now also categorized under the topic of autoinflammatory diseases. The main features and management of these diseases will be reviewed. Finally, we introduce the "Eurofever" project, aimed to increase awareness and education for the aforementioned diseases. We conclude that the pediatrician should be aware of the features and management of autoinflammatory diseases since all present with fever-the most common symptom of pediatric practice.
Affiliation
Department of Pediatrics, Faculty of Medicine, Hacettepe University, 06100, Ankara, Turkey, sezaozen@hacettepe.edu.tr.
Journal Details
This article was published in the following journal.
Name: European journal of pediatrics
ISSN: 1432-1076
Pages:
Links
- PubMed Source: http://www.ncbi.nlm.nih.gov/pubmed/21360011
- DOI: http://dx.doi.org/10.1007/s00431-011-1411-z
Medical and Biotech [MESH] Definitions
Hereditary Autoinflammatory Diseases
Hereditary inflammation conditions, characterized by recurrent episodes of systemic inflammation. Common symptoms include recurrent fever, rash, arthritis, fatigue, and secondary AMYLOIDOSIS. Hereditary autoinflammatory diseases are associated with mutations in genes involved in regulation of normal inflammatory process and are not caused by AUTOANTIBODIES, or antigen specific T-LYMPHOCYTES.
Dental Care For Chronically Ill
Dental care for patients with chronic diseases. These diseases include chronic cardiovascular, endocrinologic, hematologic, immunologic, neoplastic, and renal diseases. The concept does not include dental care for the mentally or physically disabled which is DENTAL CARE FOR DISABLED.
Progressive Patient Care
Organization of medical and nursing care according to the degree of illness and care requirements in the hospital. The elements are intensive care, intermediate care, self-care, long-term care, and organized home care.
Disease Notification
Notification or reporting by a physician or other health care provider of the occurrence of specified contagious diseases such as tuberculosis and HIV infections to designated public health agencies. The United States system of reporting notifiable diseases evolved from the Quarantine Act of 1878, which authorized the US Public Health Service to collect morbidity data on cholera, smallpox, and yellow fever; each state in the US has its own list of notifiable diseases and depends largely on reporting by the individual health care provider. (From Segen, Dictionary of Modern Medicine, 1992)
After-hours Care
Medical care provided after the regular practice schedule of the physicians. Usually it is designed to deliver 24-hour-a-day and 365-day-a-year patient care coverage for emergencies, triage, pediatric care, or hospice care.
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