Outcome of Tonsillectomy in Selected Patients With PFAPA Syndrome

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Abstract
The medical syndrome clinically characterized by the association of periodic fever, aphthous stomatitis, pharyngitis, and cervical lymphadenopathy was first described in 1987 by Marshall et al1 and was subsequently called fever, aphthous stomatitis, pharyngitis, and cervical lymphadenopathy (FAPA) syndrome by Feder and Bialecki in 1989.2 In the same year, Marshall et al3 renamed it PFAPA syndrome to underline the distinctiveness of the clockwork periodicity of events and thus include it in the differential diagnosis of periodic fever syndromes in children. The diagnostic criteria for typical PFAPA syndrome according to Marshall et al3 include the following: (1) onset of disease before 5 years of age; (2) regularly recurring, abrupt episodes of fever lasting about 5 days with constitutional symptoms associated with both (a) aphthous stomatitis and/or pharyngitis (with or without cervical adenitis) without other signs of respiratory tract infection and (b) acute inflammatory markers (leukocytosis or elevated erythrocyte sedimentation rate); (3) completely asymptomatic interval period, normal growth parameters, benign long-term course, and no sequelae; (4) exclusion of cyclic neutropenia; (5) exclusion of other periodic fevers; and (6) exclusion of immunodeficiency, autoimmune diseases, or chronic infections.