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Hereditary periodic fever syndromes
Joint Authors
Shawqi, Rabah M.
Gaboon, Najwa E. A.
Source
The Egyptian Journal of Medical Human Genetics
Issue
Vol. 12, Issue 2 (31 Dec. 2011), pp.117-125, 9 p.
Publisher
Egyptian Society of Human Genetics
Publication Date
2011-12-31
Country of Publication
Egypt
No. of Pages
9
Main Subjects
Topics
Abstract EN
Hereditary periodic fever syndromes, comprise a group of hereditary disorders with similar clinical features of recurrent short episodes of fever associated with inflammatory manifestations.
These are usually self-limited in nature and occur in the absence of infection or autoimmune reaction.
Between attacks, patients feel well and regain their normal daily functions until the next episode occurs.
The episodes are usually associated with elevated serum levels of acute-phase reactants (e.g., fibrinogen, serum amyloid A [SAA]), an elevated erythrocyte sedimentation rate (ESR), and leukocytosis.
These illnesses represent inborn errors in the regulation of innate immunity thus substantiating the distinction from autoimmune disorders, which more directly affect the adaptive immune system.
Each of these disorders has a distinct genetic defect.
Most of these proteins are members of the Death Domain Superfamily and are involved in inflammation and apoptosis.
These proteins mediate the regulation of nuclear factor-kB (NF-kB), cell apoptosis, and interleukin 1b (IL-1b) secretion through cross-regulated and common signaling pathways.
Six periodic fever syndromes have been characterized.
Genetic defects, pathogenesis, epidemiology and management of these fevers will be discussed.
American Psychological Association (APA)
Shawqi, Rabah M.& Gaboon, Najwa E. A.. 2011. Hereditary periodic fever syndromes. The Egyptian Journal of Medical Human Genetics،Vol. 12, no. 2, pp.117-125.
https://search.emarefa.net/detail/BIM-380318
Modern Language Association (MLA)
Shawqi, Rabah M.& Gaboon, Najwa E. A.. Hereditary periodic fever syndromes. The Egyptian Journal of Medical Human Genetics Vol. 12, no. 2 (2011), pp.117-125.
https://search.emarefa.net/detail/BIM-380318
American Medical Association (AMA)
Shawqi, Rabah M.& Gaboon, Najwa E. A.. Hereditary periodic fever syndromes. The Egyptian Journal of Medical Human Genetics. 2011. Vol. 12, no. 2, pp.117-125.
https://search.emarefa.net/detail/BIM-380318
Data Type
Journal Articles
Language
English
Notes
Includes bibliographical references : p. 124-125
Record ID
BIM-380318