Premium
Incidence, clinical characteristics and outcome in N orwegian children with periodic fever, aphthous stomatitis, pharyngitis and cervical adenitis syndrome; a population‐based study
Author(s) -
Førsvoll Jostein,
Kristoffersen Einar Klæboe,
Øymar Knut
Publication year - 2013
Publication title -
acta paediatrica
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.772
H-Index - 115
eISSN - 1651-2227
pISSN - 0803-5253
DOI - 10.1111/apa.12069
Subject(s) - medicine , pharyngitis , incidence (geometry) , tonsillectomy , pediatrics , adenitis , population , stomatitis , epidemiology , prospective cohort study , tonsillitis , surgery , dermatology , physics , environmental health , optics
Aim 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 S outh R ogaland, N orway, 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). Conclusion 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.