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Periodic fever, aphthous stomatitis, pharyngitis, and adenitis (PFAPA) syndrome, first described by Marshall1 in 1987, is the commonest type of periodic fever among children. Episodes of PFAPA are characterized by periodic high fever lasting three to six days and recurring every three to eight weeks, often accompanied by aphthous stomatitis, pharyngitis, and adenitis. Treatment with antipyretics and antibiotics has been ineffective.2 A single high dose of corticosteroid (prednisone 1–2 mg/kg) frequently leads to rapid resolution of the fever3 but does not prevent subsequent episodes. The only therapies reported to reduce recurrence of episodes are continuous cimetidine therapy4 and tonsillectomy,5 but no controlled studies of either have been published.
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