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10.1155/2015/563876

http://scihub22266oqcxt.onion/10.1155/2015/563876
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suck abstract from ncbi


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pmid26457006
      Mediators+Inflamm 2015 ; 2015 (ä): 563876
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  • The Pathogenesis of Periodic Fever, Aphthous Stomatitis, Pharyngitis, and Cervical Adenitis Syndrome: A Review of Current Research #MMPMID26457006
  • Kraszewska-G?omba B ; Matkowska-Kocjan A ; Szenborn L
  • Mediators Inflamm 2015[]; 2015 (ä): 563876 PMID26457006 show ga
  • BACKGROUND: PFAPA syndrome is a chronic disease that is characterized by recurrent episodes of high fever, aphthous stomatitis, pharyngitis, and cervical adenitis. Knowledge regarding the etiology of PFAPA is limited. OBJECTIVES: To provide up-to-date information considering etiology of PFAPA syndrome, by summarizing what has been explored and established in this area so far. MATERIALS AND METHODS: PubMed, Web of Science, and Scopus databases were searched for pertinent reports. Eventually 19 articles were selected. The results were classified into categories regarding three areas of interest: familial occurrence, genetic basis, and immunological mechanisms of PFAPA. RESULTS: Recent findings suggest that there is a familial tendency to PFAPA but the level of evidence does not warrant definite conclusions. The absence of a clear monogenic trait indicates a heterogenous, polygenic, or complex inheritance of PFAPA syndrome. As two mutations with a possible functional effect on the inflammasomes (MEFV E148Q and NLRP3 Q703K) have been found in several PFAPA cohorts, the role of inflammasome-related genes in PFAPA pathogenesis cannot be excluded. Immunological mechanisms of PFAPA involve an abnormal, IL-1? dependent innate immune response to an environmental trigger, which leads to Th1-driven inflammation expressed by recruitment of T-cells to the periphery.
  • |Animals [MESH]
  • |Fever/genetics/*immunology/pathology [MESH]
  • |Humans [MESH]
  • |Inflammation/genetics/immunology/pathology [MESH]
  • |Lymphadenitis/genetics/*immunology/pathology [MESH]
  • |Pharyngitis/genetics/*immunology/pathology [MESH]


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