Periodic fever, aphthous stomatitis, pharyngitis and adenitis
Other Names for this Disease
- Marshall Syndrome
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 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; although it has been reported in children up to 13 years. The syndrome is sporadic and non-hereditary. The course of PFAPA can be persistent for years before spontaneous, full resolution.Periodic fever, aphthous stomatitis, pharyngitis, cervical adenitis (PFAPA) is a periodic disease, which is a heterogeneous group of disorders characterized by short episodes of illness that regularly recur for several years alternated with healthy periods.
Last updated: 10/7/2013
- Berlucchi M, Nicolai P. Marshall’s syndrome or PFAPA (periodic fever, aphthous stomatitis, pharyngitis, cervical adenitis) syndrome. Orphanet encyclopedia. 2004; http://www.orpha.net/data/patho/GB/uk-PFAPA.pdf. Accessed 10/7/2013.
- Ovetchkine P, Bry ML, Reinert P. [Marshall syndrome: results of a retrospective national survey].. Arch Pediatr. 2000; http://www.ncbi.nlm.nih.gov/pubmed/10941483. Accessed 10/7/2013.
- Kurtaran H, Karadað A, Catal F, Aktaþ D. PFAPA syndrome: a rare cause of periodic fever. Turk J Pediatr. 2004; http://www.ncbi.nlm.nih.gov/pubmed/15641271. Accessed 10/7/2013.
- Long S.. Tonsillectomy as treatment of PFAPA syndrome. Journal of Pediatrics. 2007;
- The Cleveland Clinic provides an overview of the different types of periodic fever syndromes.
- The Merck Maual provides information on periodic fever syndromes in general. The Merck Manuals are a series of healthcare books for medical professionals and consumers.
- TheDoctorsDoctor.com web site provides additional information on PFAPA syndrome. Click on the link above to view this information page.
- Orphanet is a European reference portal for information on rare diseases and orphan drugs. Access to this database is free of charge.
- PubMed is a searchable database of medical literature and lists journal articles that discuss Periodic fever, aphthous stomatitis, pharyngitis and adenitis. Click on the link to view a sample search on this topic.
- Licameli G, Jeffrey J, Luz J, Jones D, Kenna M. Effect of adenotonsillectomy in PFAPA syndrome. Arch Otolaryngol Head Neck Surg. 2008 Feb;134(2):136-40.
- Padeh S, Stoffman N, Berkun Y. Periodic fever accompanied by aphthous stomatitis, pharyngitis and cervical adenitis syndrome (PFAPA syndrome) in adults. Isr Med Assoc J. 2008 May;10(5):358-60.
- Wong KK, Finlay JC, Moxham JP. Role of Tonsillectomy in PFAPA Syndrome. Otolaryngol Head Neck Surg. 2008 Jan;134(1):16-9.