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PFAPA (periodic fever, aphthous stomatitis, pharyngitis, cervical adenitis) is a recurrent syndrome with an unknown etiology. Multiple therapies have been tried with little success. Preventive treatment with daily cimetidine is associated with a low success rate, and intermittent corticosteroids usually abort episodes but might increase their frequency. Some case reports and case series in children have documented symptom resolution after tonsillectomy. Investigators conducted the first randomized controlled trial of surgical intervention in children who met strict diagnostic criteria for PFAPA syndrome.
From 2003 to 2006, 39 children (mean age, 5 years) who presented with PFAPA syndrome to a single hospital in Italy were randomized to adeno…