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Periodic fever may be a challenging diagnostic problem and cause much anxiety. A relatively benign but extremely persistent form was described in Tennessee in 1987 and later given the acronym PFAPA (periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis). Now two reports in theJournal of Pediatrics have described 94 cases in America and 28 in Israel (Kenneth T Thomas and colleagues, 1999;135:15–21. Shai Padeh and colleagues,Ibid: 98–101. See also editorial by Sarah S Long, Ibid: 1–5). The condition usually affects preschool children with a slight preponderance of boys. A striking feature is regular periodicity with episodes lasting three to six days and recurring every four or five weeks for years. In the two series some 30–40% of the children followed up had stopped having attacks and the mean total duration of the illness in those children was 4.5 years in America and 8 years in Israel. Aphthous stomatitis, pharyngitis, and cervical lymphadenopathy are each present in some 70–90% of cases. Another characteristic feature is that the children are usually relatively well during episodes and completely well between them, with normal growth and development and no long term sequelae. Apart from mild leucocytosis and mildly raised erythrocyte sedimentation rates during episodes there are no characteristic laboratory findings. Fever usually resolved rapidly after one or two doses of prednisone or prednisolone but this was followed by increased attack frequency in some. In the American series 11 patients had tonsillectomy and the episodes stopped after operation in seven of them. The cause is unknown. The pros and cons of infection and “immune dysregulation” are discussed in the editorial.
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