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AAP Grand Rounds 2:39-40 (1999) Periodic Fever: A Not-So-Rare Pediatric Syndrome?Source: (1) Thomas KT, Feder HM, Lawton AR, Edwards KM. Periodic fever syndrome in children. J Pediatr. 1999(1);135:1521. (2) Padeh S, Brezniak N, Zemer D, et al. Periodic fever, aphthous stomatitis, pharyngitis, and adenopathy syndrome: clinical characteristics and outcome. J Pediatr.1999;135(1):98101.
Thomas et al summarize the clinical features and provide follow-up data for 94 patients from 22 states and 3 foreign countries with PFAPA (Period Fever, Aphthous stomatitis, Pharyngitis, cervical Adenitis) syndrome referred to one center over a 10-year period. Children experienced episodes that lasted 4.8 days (95% CI 4.4, 5.1) and recurred every 28 days (26, 30) with maximum temperatures of 40.5(C (40.4, 40.6). Males predominated (52 vs 42) and the mean age at onset was 2.8 years (2.4, 3.3). The most common features of the attacks were chills (80%), cervical adenopathy (77%), aphthous stomatitis (67%), headache (65%), and pharyngitis (65%). Of 83 children for whom follow-up was successful, 34 had not had an episode for one or more years, while episodes tended to
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