Genetic and Rare Diseases Information Center (GARD)

Periodic fever, aphthous stomatitis, pharyngitis and adenitis
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Periodic fever, aphthous stomatitis, pharyngitis, cervical adenitis (PFAPA) is a periodic disease. 'Periodic disease' refer to a heterogeneous group of disorders characterized by short episodes of illness that regularly recur for several years alternated with healthy periods.[4] 

PFAPA is characterized by high fevers lasting three to six days and recurring every 21 to 28 days, accompanied by some or all of the signs noted in its name, namely mouth sores (aphthous stomatitis), sore throat (pharyngitis), and enlarged lymph nodes (cervical adenitis). The syndrome usually occurs in children younger than five years[3] although it has been reported in children up to 13 years[2]. The syndrome is sporadic and nonhereditary.[1] The course of PFAPA can be persistent for years before spontaneous, full resolution.[5]



References
  1. Kurtaran H, Karadað A, Catal F, Aktaþ D. PFAPA syndrome: a rare cause of periodic fever. Turk J Pediatr. 2004;:. Available at: http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=Abstract&list_uids=15641271&query_hl=33. March 19, 2009.
  2. Ovetchkine P, Bry ML, Reinert P. . Arch Pediatr. 2000;:. Available at: http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=Abstract&list_uids=10941483&query_hl=35. March 19, 2009.
  3. Sadovsky R. . American Family Physician. 2003;:. Available at: http://www.aafp.org/afp/20030215/tips/25.html. March 19, 2009.
  4. Berlucchi M, Nicolai P. . Orphanet encyclopedia. 2004;:. Available at: http://www.orpha.net/data/patho/GB/uk-PFAPA.pdf. March 19, 2009.
  5. Long S.. Tonsillectomy as treatment of PFAPA syndrome. Journal of Pediatrics. 2007.

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