title: Incidence, clinical characteristics and outcome in Norwegian children with periodic fever, aphthous stomatitis, pharyngitis and cervical adenitis syndrome; a population-based study.
abstract: OBJECTIVE
  To describe the incidence, epidemiology, clinical presentation and clinical outcome of children with the syndrome of periodic fever, aphthous stomatitis, pharyngitis and cervical adenitis (PFAPA) in a population-based study.
  
  
  METHODS
  In a prospective population-based study, all children in South Rogaland, Norway, diagnosed with PFAPA during 2004-2010 were evaluated clinically, and parents were interviewed systematically. A follow-up interview was performed for all patients.
  
  
  RESULTS
  A total of 46 children (32 boys; p = 0.011) were diagnosed with PFAPA. We calculated an incidence of 2.3 per 10 000 children up to 5 years of age. The median age of onset was 11.0 months (quartiles: 5.0, 14.8). Nearly 37 children were followed until resolution. In 17 of these, a tonsillectomy was performed with prompt resolution of PFAPA in all. The median age of spontaneous resolution was 60.2 months (range 24-120) and in children with tonsillectomy 50.9 months (range 15-128).
  
  
  CONCLUSIONS
  The incidence of PFAPA was 2.3 per 10 000 children up to 5 years of age. In the majority of cases, onset of symptoms may be during the first year of life.
authors.affiliation: Department of Pediatrics, Stavanger University Hospital, Stavanger, Norway. fojo@sus.no
authors.forename: Jostein
authors.initials: J
authors.lastname: Førsvoll
authors.forename: Einar Klaeboe
authors.initials: EK
authors.lastname: Kristoffersen
authors.forename: Knut
authors.initials: K
authors.lastname: Øymar
mesh_terms: D017668:Age of Onset; D002675:Child, Preschool; D005260:Female; D005334:Fever / Q000175:diagnosis / Q000453:epidemiology / Q000601:surgery; D005500:Follow-Up Studies; D006801:Humans; D015994:Incidence; D007223:Infant; D008199:Lymphadenitis / Q000175:diagnosis / Q000453:epidemiology / Q000601:surgery; D008297:Male; D009333:Neck; D009664:Norway / Q000453:epidemiology; D010612:Pharyngitis / Q000175:diagnosis / Q000453:epidemiology / Q000601:surgery; D011446:Prospective Studies; D013281:Stomatitis, Aphthous / Q000175:diagnosis / Q000453:epidemiology / Q000601:surgery; D013577:Syndrome; D014068:Tonsillectomy; D016896:Treatment Outcome