title: A randomized, controlled trial of tonsillectomy in periodic fever, aphthous stomatitis, pharyngitis, and adenitis syndrome.
abstract: OBJECTIVE
  We carried out a prospective, randomized, controlled trial to clarify the effect of tonsillectomy on the clinical course of periodic fever, aphthous stomatitis, pharyngitis, and adenitis (PFAPA) syndrome.
  
  
  METHODS
  Twenty-six consecutive children (mean age 4.1 years) with at least 5 PFAPA attacks were recruited from 3 tertiary care pediatric hospitals during 1999-2003 and randomly allocated to tonsillectomy or follow-up alone. They were all followed up with symptom diaries for 12 months. Tonsillectomy was allowed after 6 months in the control group if the attacks recurred.
  
  
  RESULTS
  Six months after randomization all 14 children in the tonsillectomy group and 6/12 children in the control group (50%) were free of symptoms (difference 50%, 95% confidence interval 23% to 75%, P < .001). Tonsillectomy was performed on 5/6 of the patients in the control group who still had symptoms after 6 months. The remaining unoperated child in the control group had recurrences of the fever episodes throughout the follow-up, but the symptoms became less severe, and the parents did not choose tonsillectomy.
  
  
  CONCLUSIONS
  Tonsillectomy appeared to be effective for treating PFAPA syndrome. The fever episodes ceased without any intervention in half of the control subjects. We conclude that although the mechanisms behind this syndrome are unknown, tonsillectomy can be offered as an effective intervention for children with PFAPA.
authors.affiliation: Department of Pediatrics, University of Oulu, Finland. marjo.renko@oulu.fi
authors.forename: M
authors.initials: M
authors.lastname: Renko
authors.forename: E
authors.initials: E
authors.lastname: Salo
authors.forename: A
authors.initials: A
authors.lastname: Putto-Laurila
authors.forename: H
authors.initials: H
authors.lastname: Saxen
authors.forename: P S
authors.initials: PS
authors.lastname: Mattila
authors.forename: J
authors.initials: J
authors.lastname: Luotonen
authors.forename: O
authors.initials: O
authors.lastname: Ruuskanen
authors.forename: M
authors.initials: M
authors.lastname: Uhari
mesh_terms: D002675:Child, Preschool; D010505:Familial Mediterranean Fever / Q000150:complications / Q000601:surgery*; D005260:Female; D006801:Humans; D008199:Lymphadenitis / Q000150:complications / Q000601:surgery*; D008297:Male; D010612:Pharyngitis / Q000150:complications / Q000601:surgery*; D011446:Prospective Studies; D012008:Recurrence; D013281:Stomatitis, Aphthous / Q000150:complications / Q000601:surgery*; D013577:Syndrome; D014068:Tonsillectomy